WorldWideScience

Sample records for clinical characteristics pathophysiology

  1. Pathophysiology and Japanese clinical characteristics in Marfan syndrome.

    Science.gov (United States)

    Fujita, Daishi; Takeda, Norifumi; Imai, Yasushi; Inuzuka, Ryo; Komuro, Issei; Hirata, Yasunobu

    2014-08-01

    Marfan syndrome is an autosomal dominant heritable disorder of the connective tissue, caused by mutations of the gene FBN1, which encodes fibrillin-1, a major component of the microfibrils of the extracellular matrix. Fibrillin-1 interacts with transforming growth factor-β (TGF-β), and dysregulated TGF-β signaling plays a major role in the development of connective tissue disease and familial aortic aneurysm and dissection, including Marfan syndrome. Losartan, an angiotensin II blocker, has the potential to reduce TGF-β signaling and is expected to be an additional therapeutic option. Clinical diagnosis is made using the Ghent nosology, which requires comprehensive patient assessment and has been proven to work well, but evaluation of some of the diagnostic criteria by a single physician is difficult and time-consuming. A Marfan clinic was established at the University of Tokyo Hospital in 2005, together with cardiologists, cardiac surgeons, pediatricians, orthopedists, and ophthalmologists in one place, for the purpose of speedy and accurate evaluation and diagnosis of Marfan syndrome. In this review, we discuss the recent progress in diagnosis and treatment of Marfan syndrome, and the characteristics of Japanese patients with Marfan syndrome.

  2. A prospective study of the pathophysiology and clinical characteristics of pain in a palliative medicine population.

    Science.gov (United States)

    Gutgsell, Terence; Walsh, Declan; Zhukovsky, Donna S; Gonzales, Francisco; Lagman, Ruth

    2003-01-01

    Comprehensive pain evaluation is requisite for optimal management. Few studies have evaluated pain syndromes and adequacy of associated analgesic regimens in one population. Available studies in cancer populations have focused on ambulatory patients or hospice-type inpatients. This study was designed to evaluate multiple characteristics of pain and adequacy of therapy in a broad spectrum of patients with advanced cancer presenting to a palliative medicine service. One hundred pain patients (95 with cancer) underwent a comprehensive pain evaluation consisting of history, physical examination, review of available diagnostics, and a pain assessment tool designed for routine clinical use. Seventy-one percent of 141 evaluable patients reported pain in the month before referral. In these 100 patients, 158 distinct sites of pain were reported, with 88 percent reporting a maximum of 2. Pain due to tumor was the most common cause (68 percent), and the most common pathophysiologic mechanism, somatic (52 percent). Pain was almost equally divided between continuous (48 percent) and intermittent (52 percent). Breakthrough pain occurred in 75 percent of continuous pains. Of these, 30 percent were exclusively incidental, 26percent nonincidental, and 16 percent due to end-of-dose failure. The remainder was of mixed etiology, but almost always with an incidental component. Of intermittent pain syndromes, 61 percent were incidental. On referral, analgesic dosing was inadequate and was compounded by use of regimens that typically did not meet peer-reviewed guidelines. Comprehensive studies rigorously evaluating characteristics of pain and response to treatment are a necessary first step toward more effective treatments for difficult pain syndromes.

  3. Multifocal motor neuropathy: update on clinical characteristics, pathophysiological concepts and therapeutic options.

    Science.gov (United States)

    Meuth, Sven G; Kleinschnitz, Christoph

    2010-01-01

    Multifocal motor neuropathy (MMN) is an acquired immune-mediated neuropathy characterized by chronic or stepwise progressive asymmetrical limb weakness without sensory deficits. The upper extremities are more often affected than the lower extremities with distal paresis dominating over proximal paresis. Important diagnostic features are persistent multifocal partial conduction blocks (CBs) and the presence of high-titer anti-GM1 serum antibodies. Motor neuron disease, other chronic dysimmune neuropathies, such as chronic inflammatory demyelinating polyneuropathy and the Lewis-Sumner syndrome (MADSAM neuropathy), are important differential diagnoses. While corticosteroids and plasma exchange are largely ineffective, high-dose intravenous immunoglobulins are regarded as first-line treatment. In spite of significant success in elucidating the underlying disease mechanisms in MMN during the past few years, important pathophysiological issues and the optimum long-term therapy remain to be clarified. The present review summarizes the clinical picture and current pathophysiological concepts of MMN with a special focus on the molecular and electrophysiological basis of CBs and highlights established therapies as well as possible novel treatment options.

  4. Clinical characteristics and pathophysiological mechanisms of focal and diffuse traumatic brain injury.

    NARCIS (Netherlands)

    Andriessen, T.M.J.C.; Jacobs, B.; Vos, P.E.

    2010-01-01

    Traumatic brain injury (TBI) is a frequent and clinically highly heterogeneous neurological disorder with large socioeconomic consequences. TBI severity classification, based on the hospital admission Glasgow Coma Scale (GCS) score, ranges from mild (GCS 13-15) and moderate (GCS 9-12) to severe (GCS

  5. Clinical characteristics and pathophysiological mechanisms of focal and diffuse traumatic brain injury

    NARCIS (Netherlands)

    Andriessen, Teuntje M J C; Jacobs, Bram; Vos, Pieter E

    2010-01-01

    Traumatic brain injury (TBI) is a frequent and clinically highly heterogeneous neurological disorder with large socioeconomic consequences. TBI severity classification, based on the hospital admission Glasgow Coma Scale (GCS) score, ranges from mild (GCS 13-15) and moderate (GCS 9-12) to severe (GCS

  6. Major Pathophysiological Correlations of Rosacea: A Complete Clinical Appraisal

    OpenAIRE

    Vemuri, Ravi Chandra; Gundamaraju, Rohit; Sekaran, Shamala Devi; Manikam, Rishya

    2015-01-01

    Background: Rosacea is a characteristic cutaneous disorder with a diverse clinical manifestations ranging from facial vascular hyper-reactivity to sebaceous gland hyperplasia. Many theories on pathophysiology of rosacea were proposed over the past decade, however the pathogenicity is poorly understood. Aim: To review the evidence on different pathophysiological correlations of rosacea. Methods: A literature search was conducted for studies published between 1990 to March 2014. The inclusion c...

  7. Eyelid aging: pathophysiology and clinical management

    Directory of Open Access Journals (Sweden)

    Renato Wendell Damasceno

    2015-10-01

    Full Text Available ABSTRACTLife expectancy is increasing in most countries. With increasing age, many individuals may develop involutional ophthalmic diseases, such as eyelid aging. Dermatochalasis, ptosis, ectropion, and entropion are common disorders in middle-aged and older adults. This review outlines the pathophysiology and clinical management of these involutional eyelid disorders. Recently, a decrease in elastic fibers with ultrastructural abnormalities and an overexpression of elastin-degrading enzymes have been demonstrated in involutional ectropion and entropion. This may be the consequence of local ischemia, inflammation, and/or chronic mechanical stress. Eyelid aging with progressive loss of tone and laxity may affect the ocular surface and adnexal tissues, resulting in different clinical symptoms and signs. Surgical management depends on the appropriate correction of the underlying anatomical defect.

  8. Pathophysiology, Clinical, and Therapeutic Aspects of Narcolepsy

    Directory of Open Access Journals (Sweden)

    Pinar Guzel Ozdemir

    2014-09-01

    Full Text Available Narcolepsy is a lifelong sleep disorder characterized by excessive daytime sleepiness, cataplexy, hypnagogic hallucination, and sleep paralysis. The exact cause remains unknown, but there is significant evidence that hypocretin deficiency plays an integral role. There have been advances in the understanding of the pathogenesis of narcolepsy. It has a negative effect on the quality of life and can restrict the patients from certain careers and activities. Diagnosis relies on patient history and objective data gathered from polysomnography and multiple sleep latency testing. Treatment focuses on symptom relief through medication, education, and behavioral modification. Both classic pharmacological treatments as well as newer options have significant problems, especially because of side effects and abuse potential. Some novel modalities are being examined to expand options for treatment. In this review, the pathophysiological, clinical, and pharmacotherapeutic aspects of narcolepsy are discussed. [Psikiyatride Guncel Yaklasimlar - Current Approaches in Psychiatry 2014; 6(3.000: 271-283

  9. Hemorrhoids: From basic pathophysiology to clinical management

    Institute of Scientific and Technical Information of China (English)

    Varut Lohsiriwat

    2012-01-01

    This review discusses the pathophysiology,epidemiology,risk factors,classification,clinical evaluation,and current non-operative and operative treatment of hemorrhoids.Hemorrhoids are defined as the symptomatic enlargement and distal displacement of the normal anal cushions.The most common symptom of hemorrhoids is rectal bleeding associated with bowel movement.The abnormal dilatation and distortion of the vascular channel,together with destructive changes in the supporting connective tissue within the anal cushion,is a paramount finding of hemorrhoids.It appears that the dysregulation of the vascular tone and vascular hyperplasia might play an important role in hemorrhoidal development,and could be a potential target for medical treatment.In most instances,hemorrhoids are treated conservatively,using many methods such as lifestyle modification,fiber supplement,suppositorydelivered anti-inflammatory drugs,and administration of venotonic drugs.Non-operative approaches include sclerotherapy and,preferably,rubber band ligation.An operation is indicated when non-operative approaches have failed or complications have occurred.Several surgical approaches for treating hemorrhoids have been introduced including hemorrhoidectomy and stapled hemorrhoidopexy,but postoperative pain is invariable.Some of the surgical treatments potentially cause appreciable morbidity such as anal stricture and incontinence.The applications and outcomes of each treatment are thoroughly discussed.

  10. [Pulmonary rehabilitation: pathophysiology, indications, and clinical efficacy].

    Science.gov (United States)

    Riario Sforza, G G; Incorvaia, C

    2010-01-01

    Pulmonary rehabilitation (PR) is a non pharmacologic treatment of demonstrated efficacy indicated for symptomatic patients with chronic lung diseases. In the pulmonary rehabilitation programs (PRPs), exercise training is a cornerstone component, recommended for improving muscle function. Its aim is to address the disability, intended as the reduction in functional performance and quality of life, derived from muscle deconditioning caused by physical inactivity due to chronic breathlessness, fatigue, and impairment of daily activities claimed by patients with chronic pulmonary diseases. Other components of PRPs are educational, psychosocial and nutritional interventions. Moreover, any PRP should include an outcome assessment, needed for an objective evaluation of program effectiveness, and of patient progress through the time. Although its pivotal role is now accepted worldwide, this was not the prevailing thought during the 1980's and the 1990's, when the pathophysiologic rationale of PR was still not demonstrated, condemning it to be an ancillary treatment to add to standard COPD treatment. The following ascent of PR was mainly due to the number of studies providing its effectiveness, overcoming skepticism and convincing physicians and institutions about its efficacy. Today PR clearly improves exercise tolerance, dyspnea, and quality of life, but despite such demonstration, it is mostly overlooked by health professionals, and only about 2% of patients with COPD undergo to PRPs. A proper consideration of the capability of PR is warranted to ensure optimal management of COPD when the disease causes symptoms and a decrease in physical capacity.

  11. Pathophysiology and clinical presentations of salt-losing tubulopathies.

    Science.gov (United States)

    Seyberth, Hannsjörg W

    2016-03-01

    At least three renal tubular segments are involved in the pathophysiology of salt-losing tubulopathies (SLTs). Whether the pathogenesis starts either in the thick ascending limb of the loop of Henle (TAL) or in the distal convoluted tubule (DCT), it is the function of the downstream-localized aldosterone sensitive distal tubule (ASDT) to contribute to the adaptation process. In isolated TAL defects (loop disorders) ASDT adaptation is supported by upregulation of DCT, whereas in DCT disorders the ASDT is complemented by upregulation of TAL function. This upregulation has a major impact on the clinical presentation of SLT patients. Taking into account both the symptoms and signs of primary tubular defect and of the secondary reactions of adaptation, a clinical diagnosis can be made that eventually leads to an appropriate therapy. In addition to salt wasting, as occurs in all SLTs, characteristic features of loop disorders are hypo- or isosthenuric polyuria and hypercalciuria, whereas characteristics of DCT disorders are hypokalemia and (symptomatic) hypomagnesemia. In both SLT categories, replacement of urinary losses is the primary goal of treatment. In loop disorders COX inhibitors are also recommended to mitigate polyuria, and in DCT disorders magnesium supplementation is essential for effective treatment. Of note, the combination of a salt- and potassium-rich diet together with an adequate fluid intake is always the basis of long-term treatment in all SLTs.

  12. Palmar hyperhidrosis: clinical, pathophysiological, diagnostic and therapeutic aspects*

    Science.gov (United States)

    Romero, Flávio Ramalho; Haddad, Gabriela Roncada; Miot, Hélio Amante; Cataneo, Daniele Cristina

    2016-01-01

    Abstract Palmar hyperhidrosis affects up to 3% of the population and inflict significant impact on quality of life. It is characterized by chronic excessive sweating, not related to the necessity of heat loss. It evolves from a localized hyperactivity of the sympathetic autonomic system and can be triggered by stressful events. In this study, the authors discuss clinical findings, pathophysiological, diagnostic and therapeutic issues (clinical and surgical) related to palmar hyperhidrosis. PMID:28099590

  13. Scrub typhus:pathophysiology, clinical manifestations and prognosis

    Institute of Scientific and Technical Information of China (English)

    Senaka Rajapakse; Chaturaka Rodrigo; Deepika Fernando

    2012-01-01

    ABSTRACT Scrub typhus is a zoonosis caused by the pathogenOrientia tsutsugamushi (O. tsutsugamushi). The disease has significant prevalence in eastern and Southeast Asia. Usually presenting as an acute febrile illness, the diagnosis is often missed because of similarities with other tropical febrile infections. Many unusual manifestations are present, and these are described in this review, together with an outline of current knowledge of pathophysiology. Awareness of these unusual clinical manifestations will help the clinician to arrive at an early diagnosis, resulting in early administration of appropriate antibiotics. Prognostic indicators for severe disease have not yet been clearly established.

  14. Thromboembolic complications in the nephrotic syndrome: pathophysiology and clinical management.

    Science.gov (United States)

    Singhal, Rajni; Brimble, K Scott

    2006-01-01

    Patients with the nephrotic syndrome are at increased risk of developing venous and arterial thromboembolism, the most common of which is renal vein thrombosis. There are several unanswered or controversial issues relating to the nephrotic syndrome and thromboembolism, which include the mechanism of thromboembolism, and optimal diagnostic and anticoagulant management strategies. This review will discuss several of these issues: the epidemiology and clinical spectrum of thromboembolic disease occurring in patients with the nephrotic syndrome; the pathophysiology of the hypercoagulable state associated with the nephrotic syndrome; the diagnosis of renal vein thrombosis in the nephrotic syndrome; and the evidence for prophylactic and therapeutic anticoagulation strategies in such patients.

  15. Transient stress cardiomyopathies in the elderly: Clinical & Pathophysiologic considerations

    Institute of Scientific and Technical Information of China (English)

    Michael A Chen

    2012-01-01

    Transient stress-induced cardiomyopathies have been increasingly recognized and while rare,they tend to affect elderly women more than other demographic groups.One type,often called tako-tsubo cardiomyopathy (TTC),is typically triggered by significant emotional or physical stress and is associated with chest pain,electrocardiogram (ECG) changes and abnormal cardiac enzymes.Significant left ventricular regional wall motion abnormalities usually include an akinetic "ballooning" apex with normal or hyperdynamic function of the base.A second type,often called neurogenic stunned myocardium,typically associated with subarachnoid hemorrhage,also usually presents with ECG changes and positive enzymes,but the typical wall motion abnormalities seen include normal basal and apical left ventricular contraction with akinesis of the mid-cavity in a circumferential fashion.The pathophysiology,clinical care and typical courses,are reviewed.

  16. Genetics of liver disease: From pathophysiology to clinical practice.

    Science.gov (United States)

    Karlsen, Tom H; Lammert, Frank; Thompson, Richard J

    2015-04-01

    Paralleling the first 30 years of the Journal of Hepatology we have witnessed huge advances in our understanding of liver disease and physiology. Genetic advances have played no small part in that. Initial studies in the 1970s and 1980s identified the strong major histocompatibility complex associations in autoimmune liver diseases. During the 1990 s, developments in genomic technologies drove the identification of genes responsible for Mendelian liver diseases. Over the last decade, genome-wide association studies have allowed for the dissection of the genetic susceptibility to complex liver disorders, in which also environmental co-factors play important roles. Findings have allowed the identification and elaboration of pathophysiological processes, have indicated the need for reclassification of liver diseases and have already pointed to new disease treatments. In the immediate future genetics will allow further stratification of liver diseases and contribute to personalized medicine. Challenges exist with regard to clinical implementation of rapidly developing technologies and interpretation of the wealth of accumulating genetic data. The historical perspective of genetics in liver diseases illustrates the opportunities for future research and clinical care of our patients.

  17. [Etiology, pathophysiology and clinical significance of hereditary fructose intolerance].

    Science.gov (United States)

    Fauth, U; Halmágyi, M

    1991-10-01

    Due to repeatedly described incidents in patients with undiscovered hereditary fructose intolerance, the application of fructose and sorbit-containing parenteral solutions is a topic vehemently discussed. This paper presents a survey of the literature dealing with the inborn defect of fructose-1-phosphate aldolase. The physiology and pathophysiology of fructose metabolism are described as well as the clinical appearance and diagnostic possibilities. The acute course of a fructose incompatibility is determined by a threatening decrease in the blood glucose level, which is attributed to the inhibition of several enzymes of glycolysis and gluconeogenesis by an intracellular accumulation of fructose-1-phosphate. Within hours a global functional breakdown of organs, which normally have the enzyme, occurs. The impairment of the liver function finds expression in a severe coagulopathy, the damage of the kidney leads to anuria. In chronic oral fructose supply, damage of the liver and small intestinal mucosa with corresponding gastrointestinal symptoms determine the clinical course. Concerning diagnosis, contrary to the liver biopsy and the fructose tolerance test, the mucosal biopsy with determination of fructose-1-phosphate aldolase activity has the advantage of greater specificity and is better tolerated by the patient. A total abstinence to fructose and sorbitol-containing solutions is not considered to be necessary when the rarity of the illness is taken into account and certain precautions are taken. These include a specific anamnesis of nutrition as well as a total abstinence from fructose and sorbitol in infants and in the unconscious patient. For clinical routine a simple fructose tolerance test is suggested.

  18. GAGs and GAGs diseases: when pathophysiology supports the clinic.

    Science.gov (United States)

    Costantini, Elisabetta; Lazzeri, Massimo; Porena, Massimo

    2013-01-01

    The urinary epithelium has been the subject of considerable interest and much research in recent years. What has radically changed in the last decade is the concept of what the bladder epithelium really is. It is currently no longer considered just a simple barrier and a non-specific defence against infections, and it has been recognized as a specialized tissue regulating complex bladder functions and playing a fundamental and active role in the pathogenesis of cystitis. Researchers have been focussing on the receptors and mediators that are active in the sub-epithelial layer, with the hope that understanding the role of the urothelium defect will offer the opportunity for new therapeutic strategies. On the surface of the urothelial umbrella-cells there is a thick layer of glycoproteins and proteoglycans, which together are called Glycosaminoglycans (GAGs). They constitute a hydrophilic mucosal coating and act as a barrier against solutes found in urine. In recent years they have received special attention because injury to Gags, due to different noxae, has been identified as the first step in the genesis of chronic inflammatory bladder diseases, such as recurrent urinary tract infections, chemical or radiation cystitis, interstitial cystitis and/or Bladder Pain Syndrome. Aim of this study is to define the importance of the urothelium starting from the anatomy and physiology of the bladder wall. Furthermore, we will underline the role of glycosaminoglycans, focusing both on their pathophysiological role in the principal bladder diseases and on the therapeutic aspects from the clinical point of view.

  19. Translation of clinical problems in osteoarthritis into pathophysiological research goals.

    Science.gov (United States)

    van der Kraan, Peter M; Berenbaum, Francis; Blanco, Francisco J; Cosimo, de Bari; Lafeber, Floris; Hauge, Ellen; Higginbottom, Adele; Ioan-Facsinay, Andreea; Loughlin, John; Meulenbelt, Ingrid; Moilanen, Eeva; Pitsillidou, Irene; Tsezou, Aspasia; van Meurs, Joyce; Vincent, Tonia; Wittoek, Ruth; Lories, Rik

    2016-01-01

    Osteoarthritis (OA) accounts for more disability among the elderly than any other disease and is associated with an increased mortality rate. The prevalence in Europe will rise in the future since this continent has a strongly ageing population and an obesity epidemic; obesity and age both being major risk factors for OA. No adequate therapeutic options, besides joint replacement, are available, although they are greatly needed and should be acquired by adequate research investments. However, the perspective on OA from a researcher's point of view is not always aligned with the perspective of a patient with OA. Researchers base their views on OA mainly on abnormalities in structure and function while patients consider OA as a collection of symptoms. In this viewpoint paper, we discuss the possibility of translating the most important clinical problems into pathophysiological research goals to facilitate the translation from bench to bedside and vice versa. This viewpoint is the outcome of a dialogue within the 'European League Against Rheumatism study group on OA' and People with Arthritis/Rheumatism across Europe (PARE) representatives.

  20. Sleep: General Characteristics. Physiological and Pathophysiological Patterns in Adolescence

    Directory of Open Access Journals (Sweden)

    Claudia Talero Gutiérrez

    2013-09-01

    Full Text Available Adolescence is a critical development period, related to multiple changes in physiological and psychological functions. Sleep is one of these important functions. At this stage, the childhood sleep pattern changes to the adult sleep pattern. Sleep is a complex phenomenon, resulting from the interaction between the neuroendocrine system function, the biological clock and biochemical processes, and environmental, social and cultural elements of special significance at this stage in life. The homeostasis of these systems ensures the adolescent’s growth and development within the expected physiological parameters for this stage. The restorative function of sleep and the consequences of its alteration on cognition, daytime performance and health in general, ought to be studied. At this stage of life, it is necessary to detect sleep problems or disorders and to explore possible causes. Early therapeutic intervention is needed in order to avoid the consequences of poor sleep quality. This article summarizes the results found in the current scientific literature on sleep characteristics in adolescence and their common problems.

  1. The role of anti-Müllerian hormone in the pathogenesis and pathophysiological characteristics of polycystic ovary syndrome.

    Science.gov (United States)

    Qi, Xinyu; Pang, Yanli; Qiao, Jie

    2016-04-01

    Polycystic ovarian syndrome (PCOS) is one of the major causes of anovulatory infertility. High levels of anti-Müllerian hormone (AMH) in the serum of PCOS patients participate in the major steps of the anovulation, and are related to pathogenesis and pathophysiological characteristic of PCOS, including the interactions of AMH with intra/extra ovarian factors like FSH, LH, androgen, and estrogen, as well as the role of AMH in folliculogenesis of PCOS. AMH promotes follicular atresia which may participate in the follicle pattern in PCOS patients. Recent years, the abnormally increased AMH in serum and follicle fluid of PCOS patients have attracted many scholars' attention. In this review, we summarized the role of AMH played in PCOS patients. It is of great significance for clarifying the role of AMH in the diagnosis and treatment of PCOS patients because AMH has the potential to increase our understanding of ovarian pathophysiology and to guide the clinical management of a broader range of conditions.

  2. Primary headache pathophysiology in children: the contribution of clinical neurophysiology.

    Science.gov (United States)

    Pro, S; Tarantino, S; Capuano, A; Vigevano, F; Valeriani, M

    2014-01-01

    Although primary headaches are very prevalent also in pediatric age, most neurophysiologic studies in these diseases concerned only the adulthood. The neurophysiologic investigation of the pathophysiological mechanisms subtending migraine and tension-type headache in children and adolescents could be particularly interesting, since during the developmental age the migrainous phenotype is scarcely influenced by many environmental factors that can typically act on adult headache patients. The neurophysiologic abnormality most frequently found in adult migraineurs, that is the reduced habituation of evoked potentials, was confirmed also in migraine children, although it was shown to involve also children with tension-type headache. Some studies showed abnormalities in the maturation of brain functions in migraine children and adolescents. While the visual system maturation seems slowed in young migraineurs, the psychophysiological mechanisms subtending somatosensory spatial attention in migraine children are more similar to those of healthy adults than to those of age-matched controls. There are some still unexplored fields that will have to be subjects of future studies. The nociceptive modality, which has been investigated in adult patients with primary headaches, should be studied also in pediatric migraine. Moreover, the technique of transcranial magnetic stimulation, not yet used in young migraineurs, will possibly provide further elements about brain excitability in migraine children.

  3. [Clinical picture and pathophysiology of elevated intracranial pressure].

    Science.gov (United States)

    Jacobi, G; Klinter, D; Weiermann, G

    1988-01-01

    In 48 children who had raised intracranial pressure (icp) this was monitored continuously by an epidural route. Icp-values were correlated with other important parameters like the mean arterial pressure (map) and the early components of brainstem acoustic evoked responses (BAER). The results were as follows: 1. focal and multifocal brain lesions resulting in vasogenic brain edema have better prognosis than those morbid conditions causing cytotoxic edema. In the letter group the whole brain's metabolism is impaired, brainstem, cerebellum and spinal cord included. 2. by monitoring map and icp we are enabled to assess the cerebral perfusion pressure. If this value falls short of 20 Torr the duration of unconsciousness and the frequency of brainstem related symptoms increases as well as impairment of cranial nerves and cerebellar-extrapyramidal symptoms and mental handicaps in the long term course. 3. cerebral dysfunction and organic brain syndromes in general are not related to icp-increase. They resemble local brain damage, e.g. after contusions. 4. serial registration of BAER is a good aid in iatrogenic induced deep phenobarbital coma. If there are critical values of the perfusion pressure mainly the waves III and V of BAER have increased peak latencies and go flattened. This trend takes place slowly within hours or even days. Loss of the components III and V is prompted by an irreversible damage of the caudal brainstem. This finding after cytotoxic brain edema is even more relevant than after vasogenic edema. If the component loss III-V is bilateral dissociated brain death has to be anticipated. Some important issues of icp-pathophysiology are discussed according to the literature.

  4. [Lactose intolerance: pathophysiology, clinical symptoms, diagnosis and treatment].

    Science.gov (United States)

    Hutyra, Tomasz; Iwańczak, Barbara

    2009-02-01

    Lactose malabsorption and milk products intolerance symptoms are the most common alimentary tract disorders. Lactose intolerance is a result of lactase deficiency or lack of lactase and lactose malabsorption. Three types of lactase deficiency were distinguished: congenital, late-onset lactase deficiency and secondary lactase deficiency. Lactose intolerance means the appearance of clinical gastrointestinal symptoms after ingestion of lactose. To the clinical symptoms of lactose intolerance belongs: nausea, vomiting, abdominal distension, cramps, flatulence, flatus, diarrhea and abdominal pain. The diagnosis of lactose intolerance is based on the breath hydrogen test and analysis of lactase activity in the small intestine mucosa. Dietary treatment eliminates clinical symptoms.

  5. Pathophysiology of Acute Exercise-Induced Muscular Injury: Clinical Implications

    OpenAIRE

    1995-01-01

    Acute muscular injury is the most common injury affecting athletes and those participating in exercise. Nearly everyone has experienced soreness after unaccustomed or intense exercise. Clinically, acute strains and delayed-onset muscle soreness are very similar. The purpose of this paper is to review the predisposing factors, mechanisms of injury, structural changes, and biochemical changes associated with these injuries. Laboratory and clinical findings are discussed to help athletic trainer...

  6. Asthma and coagulation: A clinical and pathophysiological evaluation

    NARCIS (Netherlands)

    C.J. Majoor

    2016-01-01

    In clinical practice pulmonologists have suspected that pulmonary embolisms occur more often in patients with asthma. To confirm this suspicion, a study was initiated to investigate the incidence of venous thromboembolic events in patients with asthma compared to the general population. In addition,

  7. Incidence, pathophysiology, and clinical manifestations of antiphospholipid syndrome.

    Science.gov (United States)

    Brock, Clifton O'neill; Brohl, Andrew Scott; Običan, Sarah Gloria

    2015-09-01

    Antiphospholipid syndrome (APLS) is a complex systemic disease with a wide variety of clinical manifestations. In the obstetric population, recurrent early pregnancy loss, fetal loss, and thrombosis are hallmarks of the disease. Patients with APLS have developed one or more pathogenic auto-antibodies directed against plasma and cell surface proteins. These antibodies are characterized by their affinity for anionic phospholipids. Interactions between APLS antibodies and their protein targets influence a wide variety of biological systems and signaling pathways, including monocytes, platelets, the complement system, and endothelial cells. While much research is currently directed at understanding the mechanisms involved in this autoimmune disease, the key clinical presentation is the hypercoagulable state resulting in thrombosis occurring in essentially any arterial or venous location, as well as numerous obstetrical complications. Treatment of APLS is generally directed at preventing thrombosis and poor pregnancy outcomes by ameliorating the hypercoagulable state.

  8. [Opioid-induced hyperalgesia. Pathophysiology and clinical relevance].

    Science.gov (United States)

    Koppert, W

    2004-05-01

    Opioids are the drugs of choice for the treatment of moderate to severe acute and chronic pain. However, clinical evidence suggests that opioids can elicit increased sensitivity to noxious stimuli suggesting that administration of opioids can activate both pain inhibitory and pain facilitatory systems. Acute receptor desensitization via uncoupling of the receptor from G-proteins, up-regulation of the cAMP pathway, activation of the N-methyl-D-aspartate (NMDA) receptor system, as well as descending facilitation, have been proposed as potential mechanisms underlying opioid-induced hyperalgesia. Numerous reports exist demonstrating that opioid-induced hyperalgesia is observed both in animal and human experimental models. Brief exposures to micro-receptor agonists induce long-lasting hyperalgesic effects for days, which might by reflected by clinical observations that large doses of intraoperative micro-receptor agonists increased postoperative pain and morphine consumption. Furthermore, the prolonged use of opioids in patients often requires increasing doses and may be accompanied by the development of abnormal pain. Successful strategies that may decrease or prevent opioid-induced hyperalgesia include the concomitant administration of drugs like NMDA-antagonists, alpha(2)-agonists, or non-steroidal anti-inflammatory drugs (NSAIDs), opioid rotation or combinations of opioids with different receptor selectivity.

  9. Metabolic syndrome in rheumatic diseases: epidemiology, pathophysiology, and clinical implications.

    Science.gov (United States)

    Sidiropoulos, Prodromos I; Karvounaris, Stylianos A; Boumpas, Dimitrios T

    2008-01-01

    Subjects with metabolic syndrome--a constellation of cardiovascular risk factors of which central obesity and insulin resistance are the most characteristic--are at increased risk for developing diabetes mellitus and cardiovascular disease. In these subjects, abdominal adipose tissue is a source of inflammatory cytokines such as tumor necrosis factor-alpha, known to promote insulin resistance. The presence of inflammatory cytokines together with the well-documented increased risk for cardiovascular diseases in patients with inflammatory arthritides and systemic lupus erythematosus has prompted studies to examine the prevalence of the metabolic syndrome in an effort to identify subjects at risk in addition to that conferred by traditional cardiovascular risk factors. These studies have documented a high prevalence of metabolic syndrome which correlates with disease activity and markers of atherosclerosis. The correlation of inflammatory disease activity with metabolic syndrome provides additional evidence for a link between inflammation and metabolic disturbances/vascular morbidity.

  10. X-linked creatine transporter deficiency: clinical aspects and pathophysiology.

    Science.gov (United States)

    van de Kamp, Jiddeke M; Mancini, Grazia M; Salomons, Gajja S

    2014-09-01

    Creatine transporter deficiency was discovered in 2001 as an X-linked cause of intellectual disability characterized by cerebral creatine deficiency. This review describes the current knowledge regarding creatine metabolism, the creatine transporter and the clinical aspects of creatine transporter deficiency. The condition mainly affects the brain while other creatine requiring organs, such as the muscles, are relatively spared. Recent studies have provided strong evidence that creatine synthesis also occurs in the brain, leading to the intriguing question of why cerebral creatine is deficient in creatine transporter deficiency. The possible mechanisms explaining the cerebral creatine deficiency are discussed. The creatine transporter knockout mouse provides a good model to study the disease. Over the past years several treatment options have been explored but no treatment has been proven effective. Understanding the pathogenesis of creatine transporter deficiency is of paramount importance in the development of an effective treatment.

  11. Obesity and heart failure: epidemiology, pathophysiology, clinical manifestations, and management.

    Science.gov (United States)

    Alpert, Martin A; Lavie, Carl J; Agrawal, Harsh; Aggarwal, Kul B; Kumar, Senthil A

    2014-10-01

    Obesity is a risk factor for heart failure (HF) in both men and women. The mortality risk of overweight and class I and II obese adults with HF is lower than that of normal weight or underweight adults with HF of comparable severity, a phenomenon referred to as the obesity paradox. Severe obesity produces hemodynamic alterations that predispose to changes in cardiac morphology and ventricular function, which may lead to the development of HF. The presence of systemic hypertension, sleep apnea, and hypoventilation, comorbidities that occur commonly with severe obesity, may contribute to HF in such patients. The resultant syndrome is known as obesity cardiomyopathy. Substantial weight loss in severely obese persons is capable of reversing most obesity-related abnormalities of cardiac performance and morphology and improving the clinical manifestations of obesity cardiomyopathy.

  12. Obstructive Sleep Apnea and Coronary Artery Disease: From Pathophysiology to Clinical Implications

    Directory of Open Access Journals (Sweden)

    Fernando De Torres-Alba

    2013-01-01

    Full Text Available Coronary artery disease (CAD and obstructive sleep apnea (OSA are both complex and significant clinical problems. The pathophysiological mechanisms that link OSA with CAD are complex and can influence the broad spectrum of conditions caused by CAD, from subclinical atherosclerosis to myocardial infarction. OSA remains a significant clinical problem among patients with CAD, and evidence suggesting its role as a risk factor for CAD is growing. Furthermore, increasing data support that CAD prognosis may be influenced by OSA and its treatment by continuous positive airway pressure (CPAP therapy. However, stronger evidence is needed to definitely answer these questions. This paper focuses on the relationship between OSA and CAD from the pathophysiological effects of OSA in CAD, to the clinical implications of OSA and its treatment in CAD patients.

  13. Left bundle-branch block--pathophysiology, prognosis, and clinical management.

    Science.gov (United States)

    Francia, Pietro; Balla, Cristina; Paneni, Francesco; Volpe, Massimo

    2007-03-01

    Given its broad use as a screening tool, the electrocardiogram (ECG) has largely become one of the most common diagnostic tests performed in routine clinical practice. As a result, the finding of left bundle-branch block (LBBB) in the absence of a well-defined clinical setting has become relatively frequent and raises questions and often concerns. While in the absence of clinically detectable heart disease LBBB does not necessarily imply poor outcomes, physicians should be aware of the role of LBBB in stratifying risk of cardiovascular events and death in subjects with both ischemic and nonischemic heart disease. This paper reviews historical landmarks, pathophysiologic features, prognostic implications, and clinical management of LBBB in apparently healthy subjects and those with heart disease.

  14. Carbon dioxide production during cardiopulmonary bypass: pathophysiology, measure and clinical relevance.

    Science.gov (United States)

    Ranucci, Marco; Carboni, Giovanni; Cotza, Mauro; de Somer, Filip

    2017-01-01

    Carbon dioxide production during cardiopulmonary bypass derives from both the aerobic metabolism and the buffering of lactic acid produced by tissues under anaerobic conditions. Therefore, carbon dioxide removal monitoring is an important measure of the adequacy of perfusion and oxygen delivery. However, routine monitoring of carbon dioxide removal is not widely applied. The present article reviews the main physiological and pathophysiological sources of carbon dioxide, the available techniques to assess carbon dioxide production and removal and the clinically relevant applications of carbon dioxide-related variables as markers of the adequacy of perfusion during cardiopulmonary bypass.

  15. Sexual Dysfunctions of HIV-Positive Men: Associated Factors, Pathophysiology Issues, and Clinical Management

    Directory of Open Access Journals (Sweden)

    Marco de Tubino Scanavino

    2011-01-01

    Full Text Available Sexual dysfunctions in HIV-positive men are associated with an increase in risky sexual behavior and decreased adherence to antiretroviral drug regimens. Because of these important public health issues, we reviewed the literature on the pathophysiology, associated factors and clinical management of sexual dysfunction in HIV-positive men. The goal was to investigate the current research on these issues. Literature searches were performed in June 2011 on PubMed, Web of Science, and PsycInfo databases with the keywords “AIDS” and “sexual dysfunction” and “HIV” and “sexual dysfunction”, resulting in 54 papers. Several researchers have investigated the factors associated with sexual dysfunction in HIV-positive men. The association between sexual dysfunction and antiretroviral drugs, particularly protease inhibitors, has been reported in many studies. The lack of standardized measures in many studies and the varying study designs are the main reasons that explain the controversial results. Despite some important findings, the pathophysiology of sexual dysfunction in the HAART era still not completely understood. Clinical trials of testosterone replacement therapy have shown the treatment to be beneficial to the improvement of sexual dysfunctions related to hypogonadism. However, there are not enough psychological intervention studies to make conclusions regarding the therapeutic effects of psychotherapy.

  16. Takotsubo cardiomyopathy systematic review: Pathophysiologic process, clinical presentation and diagnostic approach to Takotsubo cardiomyopathy.

    Science.gov (United States)

    Ono, Ryohei; Falcão, L Menezes

    2016-04-15

    Takotsubo cardiomyopathy (TTC) is characterized by transient left ventricular apical ballooning with the absence of coronary occlusion, which typically occurs in older women after emotional or physical stress. The pathophysiology of TTC is not well established, though several possible causes such as catecholamine cardiotoxicity, metabolic disturbance, coronary microvascular impairment and multivessel epicardial coronary artery spasm have been proposed. A number of diagnostic criteria have been suggested in the world and not unified as single, but the most common accepted one is Mayo Clinic proposed criteria. Since the clinical presentation of TTC is usually similar to acute coronary syndrome, differential diagnosis is essential to exclude other diseases and also for its treatment. Imaging modality including echocardiogram, angio CT and cardiac MRI, and lab tests for catecholamine, troponin T, creatine kinase MB and B-type natriuretic peptide can be useful to differentiate TTC from other diseases. Prognosis is generally favorable and in-hospital mortality is from 0% to within 10%.

  17. The inflammatory response in cardiac surgery: an overview of the pathophysiology and clinical implications.

    Science.gov (United States)

    Corral-Velez, Vicente; Lopez-Delgado, Juan C; Betancur-Zambrano, Nelson L; Lopez-Suñe, Neus; Rojas-Lora, Mariel; Torrado, Herminia; Ballus, Josep

    2015-01-01

    During cardiac surgery different factors, such as the aortic clamp, the extracorporeal circulation and the surgical injury itself, produce complex inflammatory responses which can lead to varying degrees of ischemia-reperfusion injury and/or systemic inflammatory response. This may have clinical implications due to hemodynamic changes related with an enlarged vasodilatory response. Thus, maintaining adequate levels of blood pressure during and after cardiac surgery represents a challenge for physicians when inflammatory response appears. The use of noradrenaline to raise arterial pressure is the most current pharmacological approach in the operating room and ICU. However, it is not always effective and other drugs, such as methylene blue, have to be used among others in specific cases as rescue therapy. The aim of our research is to review briefly the pathophysiology and clinical implications in the treatment of the inflammatory response in cardiac surgery, together with the mechanisms involved in those treatments.

  18. Ultraviolet Keratitis: From the Pathophysiological Basis to Prevention and Clinical Management.

    Science.gov (United States)

    Willmann, Gabriel

    2015-12-01

    Ultraviolet keratitis is caused by the toxic effects of acute high-dose ultraviolet radiation (UVR) reflecting the sensitivity of the ocular surface to photochemical injury. The clinical syndrome presents with ocular pain, tearing, conjunctival chemosis, blepharospasm, and deterioration of vision typically several hours after exposure, lasting up to 3 days. Mountaineers, skiers, and beach recreationalists are particularly at risk to suffer from ultraviolet (UV) keratitis as the reflectivity of UVR in these environments is extremely high. The aim of this review is to raise awareness about the potential of UV damage on the eye with an emphasis on UV keratitis, to highlight the pathophysiological basis of corneal phototoxicity, and to provide practical guidance for the prevention and clinical management of UV keratitis commonly known as snow blindness.

  19. The clinical spectrum and pathophysiology of skeletal complications in lysosomal storage disorders.

    Science.gov (United States)

    Clarke, Lorne A; Hollak, Carla E M

    2015-03-01

    Lysosomal storage disorders affect multiple organs including the skeleton. Disorders with prominent skeletal symptoms are type 1 and 3 Gaucher disease, the mucopolysaccharidoses, the glycoproteinoses and pycnodysostosis. Clinical manifestations range from asymptomatic radiographical evidence of bone pathology to overt bone crises (Gaucher), short stature with typical imaging features known as dysostosis multiplex (MPS), with spine and joint deformities (mucopolysaccharidoses, mucolipidosis), or osteopetrosis with pathological fractures (pynodysostosis). The pathophysiology of skeletal disease is only partially understood and involves direct substrate storage, inflammation and other complex alterations of cartilage and bone metabolism. Current treatments are enzyme replacement therapy, substrate reduction therapy and hematopoietic stem cell transplantation. However, effects of these interventions on skeletal disease manifestations are less well established and outcomes are highly dependent on disease burden at treatment initiation. It is now clear that adjunctive treatments that target skeletal disease are needed and should be part of future research agenda.

  20. Adrenal Disorders and the Paediatric Brain: Pathophysiological Considerations and Clinical Implications

    Directory of Open Access Journals (Sweden)

    Vincenzo Salpietro

    2014-01-01

    Full Text Available Various neurological and psychiatric manifestations have been recorded in children with adrenal disorders. Based on literature review and on personal case-studies and case-series we focused on the pathophysiological and clinical implications of glucocorticoid-related, mineralcorticoid-related, and catecholamine-related paediatric nervous system involvement. Childhood Cushing syndrome can be associated with long-lasting cognitive deficits and abnormal behaviour, even after resolution of the hypercortisolism. Exposure to excessive replacement of exogenous glucocorticoids in the paediatric age group (e.g., during treatments for adrenal insufficiency has been reported with neurological and magnetic resonance imaging (MRI abnormalities (e.g., delayed myelination and brain atrophy due to potential corticosteroid-related myelin damage in the developing brain and the possible impairment of limbic system ontogenesis. Idiopathic intracranial hypertension (IIH, a disorder of unclear pathophysiology characterised by increased cerebrospinal fluid (CSF pressure, has been described in children with hypercortisolism, adrenal insufficiency, and hyperaldosteronism, reflecting the potential underlying involvement of the adrenal-brain axis in the regulation of CSF pressure homeostasis. Arterial hypertension caused by paediatric adenomas or tumours of the adrenal cortex or medulla has been associated with various hypertension-related neurological manifestations. The development and maturation of the central nervous system (CNS through childhood is tightly regulated by intrinsic, paracrine, endocrine, and external modulators, and perturbations in any of these factors, including those related to adrenal hormone imbalance, could result in consequences that affect the structure and function of the paediatric brain. Animal experiments and clinical studies demonstrated that the developing (i.e., paediatric CNS seems to be particularly vulnerable to alterations induced by

  1. Clinical characteristics of cervicogenic-related dizziness and vertigo.

    Science.gov (United States)

    Yacovino, Dario A; Hain, Timothy C

    2013-07-01

    Cervical vertigo has long been a controversial entity and its very existence as a medical entity has advocates and opponents. Supporters of cervical vertigo claim that its actual prevalence is underestimated due to the overestimation of other diagnostic categories in clinics. Furthermore, different pathophysiological mechanisms have been attributed to cervical vertigo. Here the authors discuss the clinical characteristics of rotational vertebral artery vertigo, postwhiplash vertigo, proprioceptive cervical vertigo, and cervicogenic vertigo of old age. A clinical entity named subclinical vertebrobasilar insufficiency appears in the context of cervical osteoarticular changes. Migraine-associated vertigo may explain why some patients suffering from cervical pain have vertigo while others do not.

  2. Solitary rectal ulcer syndrome: clinical features, pathophysiology, diagnosis and treatment strategies.

    Science.gov (United States)

    Zhu, Qing-Chao; Shen, Rong-Rong; Qin, Huan-Long; Wang, Yu

    2014-01-21

    Solitary rectal ulcer syndrome (SRUS) is an uncommon benign disease, characterized by a combination of symptoms, clinical findings and histological abnormalities. Ulcers are only found in 40% of the patients; 20% of the patients have a solitary ulcer, and the rest of the lesions vary in shape and size, from hyperemic mucosa to broad-based polypoid. Men and women are affected equally, with a small predominance in women. SRUS has also been described in children and in the geriatric population. Clinical features include rectal bleeding, copious mucus discharge, prolonged excessive straining, perineal and abdominal pain, feeling of incomplete defecation, constipation, and rarely, rectal prolapse. This disease has well-described histopathological features such as obliteration of the lamina propria by fibrosis and smooth muscle fibers extending from a thickened muscularis mucosa to the lumen. Diffuse collage deposition in the lamina propria and abnormal smooth muscle fiber extensions are sensitive markers for differentiating SRUS from other conditions. However, the etiology remains obscure, and the condition is frequently associated with pelvic floor disorders. SRUS is difficult to treat, and various treatment strategies have been advocated, ranging from conservative management to a variety of surgical procedures. The aim of the present review is to summarize the clinical features, pathophysiology, diagnostic methods and treatment strategies associated with SRUS.

  3. The dark side of the QT interval. The Short QT Syndrome: pathophysiology, clinical presentation and management

    Directory of Open Access Journals (Sweden)

    I. Comelli

    2012-12-01

    Full Text Available A large number of studies has been carried out to investigate the pathophysiology and the clinical implications of QT interval prolongation in the ECG over recent years (1, 2, 3, 4, 5, 6. It was only in the last decade, however, that the scientists have focused on the specular aspects of the long QT syndrome (LQTS, and it is now well established that the abnormal shortening of the QT interval is associated with meaningful clinical consequences and adverse outcomes. The aim of the present article is to summarize knowledge and existing evidence about the Short QT Syndrome (SQTS. SQTS is a rare, albeit largely underdiagnosed, genetically determined disease, which is characterized by a high tendency to develop life-threatening arrhythmias. The two clinical landmarks of SQTS are the presence of a short QT interval (i.e., less than 320 ms in a structurally normal heart. The disease is now classified as a “channellopathy”, and is principally caused by a defective functioning of both potassium and calcium ion channels. The underlying genetic anomalies cause an abnormal ripolarization and a reduced refractoriness of myocardiocites. Pharmacologic treatments are mainly tailored to slow the conduction and to prolong the refractory period of myocardiocites. The implantable cardioverter and defibrillator (ICD is currently considered the therapeutic gold standard (7.

  4. The neurobiology of depression in later-life: clinical, neuropsychological, neuroimaging and pathophysiological features.

    Science.gov (United States)

    Naismith, Sharon L; Norrie, Louisa M; Mowszowski, Loren; Hickie, Ian B

    2012-07-01

    As the population ages, the economic and societal impacts of neurodegenerative and neuropsychiatric disorders are expected to rise sharply. Like dementia, late-life depressive disorders are common and are linked to increased disability, high healthcare utilisation, cognitive decline and premature mortality. Considerable heterogeneity in the clinical presentation of major depression across the life cycle may reflect unique pathophysiological pathways to illness; differentiating those with earlier onset who have grown older (early-onset depression), from those with illness onset after the age of 50 or 60 years (late-onset depression). The last two decades have witnessed significant advances in our understanding of the neurobiology of early- and late-onset depression, and has shown that disturbances of fronto-subcortical functioning are implicated. New biomedical models extend well beyond perturbations of traditional monoamine systems to include altered neurotrophins, endocrinologic and immunologic system dysfunction, inflammatory processes and gene expression alterations. This more recent research has highlighted that a range of illness-specific, neurodegenerative and vascular factors appear to contribute to the various phenotypic presentations. This review highlights the major features of late-life depression, with specific reference to its associated aetiological, clinical, cognitive, neuroimaging, neuropathological, inflammatory and genetic correlates. Data examining the efficacy of pharmacological, non-pharmacological and novel treatments for depression are discussed. Ultimately, future research must aim to evaluate whether basic biomedical knowledge can be successfully translated into enhanced health outcomes via the implementation of early intervention paradigms.

  5. Hypopigmented mycosis fungoides: a review of its clinical features and pathophysiology*

    OpenAIRE

    Furlan, Fabricio Cecanho; Sanches, José Antonio

    2013-01-01

    Several distinct clinical forms of mycosis fungoides have been described. Hypopigmented mycosis fungoides should be regarded as a subtype of mycosis fungoides, insofar as it presents some peculiar characteristics that contrast with the clinical features of the classical form. Most patients with hypopigmented mycosis fungoides are younger than patients typically diagnosed with classical mycosis fungoides. In addition to typical dark-skinned individuals impairment, hypopigmented mycosis fungoid...

  6. Clinical Characteristics of Labyrinthine Concussion

    OpenAIRE

    Choi, Mi Suk; Shin, See-Ok; Yeon, Je Yeob; Choi, Young Seok; Kim, Jisung; Park, Soo Kyoung

    2013-01-01

    Background and Objectives Inner ear symptoms like hearing loss, dizziness or tinnitus are often developed after head trauma, even in cases without inner ear destruction. This is also known as labyrinthine concussion. The purpose of this study is to determine the clinical manifestations, characteristics of audiometry and prognostic factors of these patients. Materials and Methods We reviewed the medical records of the 40 patients that had been diagnosed as labyrinthine concussion from 1996 to ...

  7. Dystonia in complex regional pain syndrome : clinical, pathophysiological and therapeutic aspects

    NARCIS (Netherlands)

    Rijn, Monica Adriana van

    2010-01-01

    The clinical characteristics of Complex Regional Pain Syndrome (CRPS) are defined by pain and various combinations of sensory disturbances, autonomic features, and sudomotor and trophic changes. Furthermore, patients with CRPS may suffer from movement disorders, of which dystonia is the most prevale

  8. Rheumatoid arthritis associated pulmonary hypertension: Clinical challenges reflecting the diversity of pathophysiology

    Directory of Open Access Journals (Sweden)

    Evangelia Panagiotidou

    2017-01-01

    Full Text Available The present article reports three clinical cases in order to elucidate the diversity of the pathophysiological mechanisms that underlie rheumatoid arthritis associated pulmonary hypertension. The condition's three major causes are: interstitial lung disease, vasculitis, and chronic thromboembolic disease, but it should be noted that the multiple pulmonary manifestations of rheumatoid arthritis, can all contribute to chronic lung disease or hypoxia. The first patient in this report suffered from moderate restriction due to fibrosis and was diagnosed with pulmonary hypertension during an episode of life threatening hypoxia. Early upfront combination therapy prevented intubation and reversed hypoxia to adequate levels. The second presented patient was a case of isolated pulmonary hypertension attributable to vasculopathy. The patient maintained normal lung volumes but low diffusion capacity and echocardiography dictated the need for right heart catheterization. Finally, the third patient presented severe functional limitation due to several manifestations of rheumatoid arthritis, but a past episode of acute pulmonary embolism was also reported although it had never been evaluated. Chronic thromboembolic disease was eventually proved to be one major cause of the patient's pulmonary hypertension. The importance of early identification of pulmonary hypertension in patients with rheumatoid arthritis is therefore emphasized, especially since multiple treatment options are available, symptoms can be treated, and right heart failure can be avoided.

  9. Rheumatoid arthritis associated pulmonary hypertension: Clinical challenges reflecting the diversity of pathophysiology.

    Science.gov (United States)

    Panagiotidou, Evangelia; Sourla, Evdokia; Kotoulas, Serafim Xrisovalantis; Akritidou, Sofia; Bikos, Vasileios; Bagalas, Vasileios; Stanopoulos, Ioannis; Pitsiou, Georgia

    2017-01-01

    The present article reports three clinical cases in order to elucidate the diversity of the pathophysiological mechanisms that underlie rheumatoid arthritis associated pulmonary hypertension. The condition's three major causes are: interstitial lung disease, vasculitis, and chronic thromboembolic disease, but it should be noted that the multiple pulmonary manifestations of rheumatoid arthritis, can all contribute to chronic lung disease or hypoxia. The first patient in this report suffered from moderate restriction due to fibrosis and was diagnosed with pulmonary hypertension during an episode of life threatening hypoxia. Early upfront combination therapy prevented intubation and reversed hypoxia to adequate levels. The second presented patient was a case of isolated pulmonary hypertension attributable to vasculopathy. The patient maintained normal lung volumes but low diffusion capacity and echocardiography dictated the need for right heart catheterization. Finally, the third patient presented severe functional limitation due to several manifestations of rheumatoid arthritis, but a past episode of acute pulmonary embolism was also reported although it had never been evaluated. Chronic thromboembolic disease was eventually proved to be one major cause of the patient's pulmonary hypertension. The importance of early identification of pulmonary hypertension in patients with rheumatoid arthritis is therefore emphasized, especially since multiple treatment options are available, symptoms can be treated, and right heart failure can be avoided.

  10. Pathophysiological mechanisms of acute pancreatitis define inflammatory markers of clinical prognosis.

    Science.gov (United States)

    Minkov, Georgi A; Halacheva, Krasimira S; Yovtchev, Yovcho P; Gulubova, Maya V

    2015-07-01

    Development of acute pancreatitis illustrates the need to understand the basic mechanisms of disease progression to drive the exploration of therapeutic options. Cytokines play a major role in the pathogenesis of acute pancreatitis as underlying systemic inflammatory response, tissue damage, and organ dysfunction. However, little is known about circulating concentrations of these inflammatory markers and their real impact on clinical practice. Experimental studies have suggested that the prognosis for acute pancreatitis depends on the degree of pancreatic necrosis and the intensity of multisystem organ failure generated by the systemic inflammatory response. This suggests an intricate balance between localized tissue damage with proinflammatory cytokine production and a systemic anti-inflammatory response that restricts the inappropriate movement of proinflammatory agents into the circulation. Implication of such mediators suggests that interruption or blunting of an inappropriate immune response has the potential to improve outcome. A detailed understanding of pathophysiological processes and immunological aspects in patients with acute pancreatitis is the basis for the development of therapeutic strategies that will provide significant reductions in morbidity and mortality.

  11. Male sexual function and its disorders: physiology, pathophysiology, clinical investigation, and treatment.

    Science.gov (United States)

    Kandeel, F R; Koussa, V K; Swerdloff, R S

    2001-06-01

    This review is designed to help the reproductive endocrinologist integrate his or her professional activity with those of other disciplines including urology, radiology, neurology, and psychology in order to successfully manage all of the inseparable aspects of male sexual and reproductive functioning. Significant advances in the field of male sexual physiology and pathophysiology and new methods of investigation and treatment of male sexual disorders are outlined. The review synthesizes available data on the following: norms of sexual organs, aging and sexuality, role of central and peripheral neurochemicals in each stage of the sexual cycle, role of corporeal smooth muscles in the hemodynamic control of erection and detumescence, influence of psychological factors, drugs, and disease on all aspects of sexual functioning, and use of nocturnal penile tumescence monitoring, imaging investigations, and neurophysiologic studies in the diagnostic workup of males with sexual dysfunction. Clinical algorithms are presented where appropriate. Extensive discussions on newly developed strategies in psychological and behavioral counseling, drug therapy, tissue engineering, nonsurgical devices, and surgical treatments for all forms of sexual disorders are also provided. Lastly, the effect of sexual dysfunction and its treatment on quality of life in affected men is addressed, along with recommendations for future research endeavors.

  12. Liver cirrhosis and diabetes: Risk factors, pathophysiology, clinical implications and management

    Institute of Scientific and Technical Information of China (English)

    Diego Garcia-Compean; Joel Omar Jaquez-Quintana; Jose Alberto Gonzalez-Gonzalez; Hector Maldonado-Garza

    2009-01-01

    About 30% of patients with cirrhosis have diabetes mellitus (DM). Nowadays, it is a matter for debate whether type 2 DM in the absence of obesity and hypertriglyceridemia may be a risk factor for chronic liver disease. DM,which develops as a complication of cirrhosis, is known as "hepatogenous diabetes". Insulin resistance in muscular and adipose tissues and hyperinsulinemia seem to be the pathophysiologic bases of diabetes in liver disease. An impaired response of the islet β-cells of the pancreas and hepatic insulin resistance are also contributory factors. Non-alcoholic fatty liver disease, alcoholic cirrhosis, chronic hepatitis C (CHC) and hemochromatosis are more frequently associated with DM. Insulin resistance increases the failure of the response to treatment in patients with CHC and enhances progression of fibrosis. DM in cirrhotic patients may be subclinical.Hepatogenous diabetes is clinically different from that of type 2 DM, since it is less frequently associated with microangiopathy and patients more frequently suffer complications of cirrhosis. DM increases the mortality of cirrhotic patients. Treatment of the diabetes is complex due to liver damage and hepatotoxicity of oral hypoglycemic drugs. This manuscript will review evidence that exists in relation to: type 2 DM alone or as part of the metabolic syndrome in the development of liver disease;factors involved in the genesis of hepatogenous diabetes;the impact of DM on the clinical outcome of liver disease; the management of DM in cirrhotic patients and the role of DM as a risk factor for the occurrence and exacerbation of hepatocellular carcinoma.

  13. Gait disorders and balance disturbances in Parkinson's disease: clinical update and pathophysiology.

    NARCIS (Netherlands)

    Boonstra, T.A.; Kooij, H. van der; Munneke, M.; Bloem, B.R.

    2008-01-01

    PURPOSE OF REVIEW: Gait disorders and balance impairments are one of the most incapacitating symptoms of Parkinson's disease. Here, we discuss the latest findings regarding epidemiology, assessment, pathophysiology and treatment of gait and balance impairments in Parkinson's disease. RECENT FINDINGS

  14. [Multiaxial evaluation of the pathophysiology of mood disorder and therapeutic mechanisms of clinical drugs by neuronal plasticity and neuronal load].

    Science.gov (United States)

    Omata, Naoto; Mizuno, Tomoyuki; Mitsuya, Hironori; Wada, Yuji

    2013-11-01

    Impairment of neuronal plasticity is important in the pathophysiology of mood disorder. Both zinc deficiency and social isolation impair neuronal plasticity. Both cause a depressive state. However, in experiments using animals, their combined loading induced manic-like behavior. Therefore, it was inferred that moderate impairment of neuronal plasticity induces a depressive state, and that further impairment of neuronal plasticity induces a manic state. However, some kind of load toward neuronal function through neural transmission can influence mood disorder symptoms without direct effects on neuronal plasticity. Our hypothesis is that mania is an aggravation of depression from the perspective of neuronal plasticity, and that multiaxial evaluation by neuronal plasticity and neuronal load through neural transmission is useful for understanding the pathophysiology of mood disorder. There are many clinical aspects that have been difficult to interpret in mood disorder: Why is a mood stabilizer or electric convulsive therapy useful for both mania and depression? What is the pathophysiology of the mixed state? Why does manic switching by an antidepressant occur or not? Our hypothesis is useful to understand these aspects, and using this hypothesis, it is expected that the pathophysiology of mood disorder and clinical mechanism of mood stabilizers and antidepressants can now be understood as an integrated story.

  15. Clinical characteristics of Caroli's syndrome

    Institute of Scientific and Technical Information of China (English)

    Ozlem Yonem; Yusuf Bayraktar

    2007-01-01

    Caroli's syndrome is characterized by multiple segmental cystic or saccular dilatations of intrahepatic bile ducts associated with congenital hepatic fibrosis. The clinical features of this syndrome reflect both the characteristics of congenital hepatic fibrosis such as portal hypertension and that of Caroli's disease named as recurrent cholangitis and cholelithiasis. The diagnosis depends on both histology and imaging methods which can show the communication between the sacculi and the bile ducts. Treatment consists of symptomatic treatment of cholangitis attacks by antibiotics, some endoscopic,radiological and surgical drainage procedures and surgery. Liver transplantation seems the ultimate treatment for this disease. Prognosis is fairly good unless recurrent cholangitis and renal failure develops.

  16. Clinical Consequences of New Insights in the Pathophysiology of Disorders of Iron and Heme Metabolism.

    Science.gov (United States)

    Brittenham, Gary M.; Weiss, Günter; Brissot, Pierre; Lainé, Fabrice; Guillygomarc'h, Anne; Guyader, Dominique; Moirand, Romain; Deugnier, Yves

    2000-01-01

    This review examines the clinical consequences for the practicing hematologist of remarkable new insights into the pathophysiology of disorders of iron and heme metabolism. The familiar proteins of iron transport and storage-transferrin, transferrin receptor, and ferritin-have recently been joined by a host of newly identified proteins that play critical roles in the molecular management of iron homeostasis. These include the iron-regulatory proteins (IRP-1 and -2), HFE (the product of the HFE gene that is mutated in most patients with hereditary hemochromatosis), the divalent metal transporter (DMT1), transferrin receptor 2, ceruloplasmin, hephaestin, the "Stimulator of Fe Transport" (SFT), frataxin, ferroportin 1 and others. The growing appreciation of the roles of these newly identified proteins has fundamental implications for the clinical understanding and laboratory evaluation of iron metabolism and its alterations with iron deficiency, iron overload, infection, and inflammation. In Section I, Dr. Brittenham summarizes current concepts of body and cellular iron supply and storage and reviews new means of evaluating the full range of body iron stores including genetic testing for mutations in the HFE gene, measurement of serum ferritin iron, transferrin receptor, reticulocyte hemoglobin content and measurement of tissue iron by computed tomography, magnetic resonance imaging and magnetic susceptometry using superconducting quantum interference device (SQUID) instrumentation. In Section II, Dr. Weiss discusses the improved understanding of the molecular mechanisms underlying alterations in iron metabolism due to chronic inflammatory disorders. The anemia of chronic disorders remains the most common form of anemia found in hospitalized patients. The network of interactions that link iron metabolism with cellular immune effector functions involving pro- and anti-inflammatory cytokines, acute phase proteins and oxidative stress is described, with an emphasis on

  17. Cutaneous approach towards clinical and pathophysiological aspects of hyperglycemia by ATR FTIR spectroscopy

    Science.gov (United States)

    Eikje, Natalja Skrebova; Sota, Takayuki; Aizawa, Katsuo

    2007-07-01

    Attempts were made to non-invasively detect glucose-specific spectral signals in the skin by ATR-FTIR spectroscopy. In vivo spectra were collected from the inner wrists of healthy, prediabetes and diabetes subjects in the 750-4000 cm -1 region, with a closer assessment of the glucose-related region between 1000 and 1180 cm -1. Spectra in vivo showed glucose-specific peaks at 1030, 1080, 1118 and 1151 cm -1, as a variety of glucose solutions are found in vitro. Based on the differences of intensities at 1030 and 1118 cm -1 two spectral patterns were seen: I 1118 > I 1030 for a diabetes and I 1030> I 1118 for non-diabetes subjects. The peak at 1030 cm -1 was used to assess glucose concentrations in the skin due to its good correlation with glucose concentrations in vitro. Calculated mean values of the peak at 1030 cm -1 showed evidence of correlation with blood glucose levels when grouped as = 200 mg/dL, though there was no constant correlation between them when compared before/after OGTT or at the fasting/postprandial states. Absorbances at 1030 cm -1 were not only increased in a dose-dependent manner in a diabetes patient, but were also generally higher than in non-diabetes subjects at 30 min OGTT assessment. Also we could monitor absorbances at 1030 cm -1 and determine their changes in the skin tissue at different times of OGTT. We assume that our approach to in vivo measurement and monitoring of glucose concentrations at 1030 cm -1 may be one of the indicators to assess glucose activity level and its changes in the skin tissue, and has further implications in the study of clinical and pathophysiological aspects of hyperglycemia in diabetes and non-diabetes subjects by ATR-FTIR spectroscopy.

  18. Pathophysiological Characteristics of Diabetic Ocular Complications in Spontaneously Diabetic Torii Rat

    Directory of Open Access Journals (Sweden)

    Tomohiko Sasase

    2010-01-01

    Full Text Available The Spontaneously Diabetic Torii (SDT rat, a nonobese type 2 diabetes model, develops severe diabetic retinopathy as result of chronic severe hyperglycemia. Although existing diabetes animal models also develop ocular complications, severe retinal lesions frequently observed in human diabetes patients such as preretinal neovascularization or retinal detachment are not found. Distinctive features in SDT rat are hypermature cataract, tractional retinal detachment with fibrous proliferation, and massive hemorrhaging in the anterior chamber. These pathophysiological changes are caused by sustained hyperglycemic condition and subsequent increased expression of vascular endothelial growth factor (VEGF in retina, iris, and ciliary body. Although some differences in diabetic retinopathy exist between SDT rats and humans (e.g., a low incidence of neovascular formation and poor development of nonperfused area are found in this animal, SDT rat will be a useful model in studies of the pathogenesis and treatment of diabetic retinopathy.

  19. [Clinical and pathophysiological patterns of otitis externa and overview of problematic cases].

    Science.gov (United States)

    Dzepina, Davor; Ajduk, Jakov; Zurak, Kreso

    2011-01-01

    Otitis externa can sometimes present itself as a difficult clinical problem, in cases such as unsuccessful outcome of usual therapy, prolonged duration of symptoms or frequent recurrencies with local or systemic complications. In these cases more attention should be focused on possible errors made in establishing the right diagnosis, incorrect sequence of applying therapeutical measures, or presence of unrecognized problems, including individual patient factors, characteristics of the pathogen, or other. In this article we present a case report of a 84-year-old patient with complicated otitis externa, together with a short review of physiology, patophysiology, diagnostic and therapeutical measures in external otitis, and suggest a possible clinical approach in the management of problematic cases.

  20. Pathophysiological role and clinical significance of lipoprotein-associated phospholipase A₂ (Lp-PLA₂) bound to LDL and HDL.

    Science.gov (United States)

    Tellis, Constantinos C; Tselepis, Alexandros D

    2014-01-01

    Lipoprotein-associated phospholipase A2 (Lp-PLA2), also named as platelet-activating factor (PAF)-acetylhydrolase, exhibits a Ca2+-independent phospholipase A2 activity and catalyzes the hydrolysis of the ester bond at the sn-2 position of PAF and oxidized phospholipids (oxPL). These phospholipids are formed under oxidative and inflammatory conditions, and may play important roles in atherogenesis. The vast majority of plasma Lp-PLA2 mass binds to low-density lipoprotein (LDL) while a smaller amount is associated with high-density lipoprotein (HDL). Lp-PLA2 is also bound to lipoprotein (a) [Lp(a)], very low-density lipoprotein (VLDL) and remnant lipoproteins. Several lines of evidence suggest that the role of plasma Lp-PLA2 in atherosclerosis may depend on the type of lipoprotein particle with which this enzyme is associated. Data from large Caucasian population studies have supported plasma Lp-PLA2 (primarily LDL-associated Lp-PLA2) as a cardiovascular risk marker independent of, and additive to, traditional risk factors. On the contrary, the HDL-associated Lp-PLA2 may express antiatherogenic activities and is also independently associated with lower risk for cardiac death. The present review presents data on the biochemical and enzymatic properties of Lp-PLA2 as well as its structural characteristics that determine the association with LDL and HDL. We also critically discuss the possible pathophysiological and clinical significance of the Lp- PLA2 distribution between LDL and HDL in human plasma, in view of the results of prospective epidemiologic studies on the association of Lp-PLA2 with future cardiovascular events as well the recent studies that evaluate the possible effectiveness of specific Lp-PLA2 inhibitors in reducing residual cardiovascular risk.

  1. PEEP Role in ICU and Operating Room: From Pathophysiology to Clinical Practice

    Directory of Open Access Journals (Sweden)

    M. Vargas

    2014-01-01

    Full Text Available Positive end expiratory pressure (PEEP may prevent cyclic opening and collapsing alveoli in acute respiratory distress syndrome (ARDS patients, but it may play a role also in general anesthesia. This review is organized in two sections. The first one reports the pathophysiological effect of PEEP on thoracic pressure and hemodynamic and cerebral perfusion pressure. The second section summarizes the knowledge and evidence of the use of PEEP in general anesthesia and intensive care. More specifically, for intensive care this review refers to ARDS and traumatic brain injured patients.

  2. Radiation-induced blood-brain barrier changes: pathophysiological mechanisms and clinical implications.

    Science.gov (United States)

    d'Avella, D; Cicciarello, R; Angileri, F F; Lucerna, S; La Torre, D; Tomasello, F

    1998-01-01

    The pathophysiology of whole-brain radiation (WBR) toxicity remains incompletely understood. The possibility of a primary change in blood-brain barrier (BBB) associated with microvascular damage was investigated. Rats were exposed to conventional fractionation in radiation (200 +/- cGy/d, 5d/wk; total dose, 4,000 cGy). BBB changes were assessed by means of the quantitative 14C-alpha-aminoisobutyric acid (AIB) technique coupled with standard electron microscopy (EM) and morphometric techniques as well as studies of the transcapillary passage of horseradish peroxidase (HRP). At 15 days after WBR, AIB transport across BBB increased significantly in cerebral cortex. EM disclosed vesicular transport of HRP across the intact endothelium without opening of the tight junctions. Ninety days after WBR, well-defined alterations of the microvasculature were observed. The main feature of cortical microvessels was their collapsed aspect, associated with perivascular edema containing cell debris. Data suggest a possible association between damage of the microvascular/glial unit of tissue injury and development of radiation-induced brain cerebral dysfunction. We hypothesize the following sequence of pathophysiological events: WBR causes an early increase in BBB permeability, which produces perivascular edema and microvascular collapse. The interference with microcirculation affects blood flow and energy supply to the tissue, resulting in structural damage on an ischemic/dysmetabolic basis.

  3. Gender differences in Parkinson's disease: clinical characteristics and cognition.

    Science.gov (United States)

    Miller, Ivy N; Cronin-Golomb, Alice

    2010-12-15

    More men than women are diagnosed with Parkinson's disease (PD), and a number of gender differences have been documented in this disorder. Examples of clinical characteristics that appear in men more often than women include rigidity and rapid eye movement behavior disorder, whereas more women than men exhibit dyskinesias and depression. Differences between men and women in cognition have not been extensively examined, though there are reports of deficits in men in aspects of cognition that contribute to activities of daily living, in verbal fluency, and in the recognition of facial emotion, and deficits in women in visuospatial cognition. Side of disease onset may interact with gender to affect cognitive abilities. One possible source of male-female differences in the clinical and cognitive characteristics of PD is the effect of estrogen on dopaminergic neurons and pathways in the brain. This effect is not yet understood, as insight into how the fluctuation of estrogen over the lifetime affects the brain is currently limited. Further attention to this area of research will be important for accurate assessment and better management of PD. Attention should also be directed to multiple covariates that may affect clinical characteristics and cognition. Knowledge about differences in the presentation of PD symptoms in men and women and about the pathophysiology underlying those differences may enhance the accuracy and effectiveness of clinical assessment and treatment of the disease.

  4. Clinical characteristics and management of melanoma families

    NARCIS (Netherlands)

    Rhee, Jasper Immanuel van der

    2013-01-01

    Being a member of a melanoma family is a major risk factor for cutaneous malignant melanoma. In this thesis clinical characteristics and management of melanoma families are discussed. In the first part of the thesis clinical and histological characteristics of melanoma (patients) from families with

  5. Diabetes and vascular disease: pathophysiology, clinical consequences, and medical therapy: part I.

    Science.gov (United States)

    Paneni, Francesco; Beckman, Joshua A; Creager, Mark A; Cosentino, Francesco

    2013-08-01

    Hyperglycemia and insulin resistance are key players in the development of atherosclerosis and its complications. A large body of evidence suggest that metabolic abnormalities cause overproduction of reactive oxygen species (ROS). In turn, ROS, via endothelial dysfunction and inflammation, play a major role in precipitating diabetic vascular disease. A better understanding of ROS-generating pathways may provide the basis to develop novel therapeutic strategies against vascular complications in this setting. Part I of this review will focus on the most current advances in the pathophysiological mechanisms of vascular disease: (i) emerging role of endothelium in obesity-induced insulin resistance; (ii) hyperglycemia-dependent microRNAs deregulation and impairment of vascular repair capacities; (iii) alterations of coagulation, platelet reactivity, and microparticle release; (iv) epigenetic-driven transcription of ROS-generating and proinflammatory genes. Taken together these novel insights point to the development of mechanism-based therapeutic strategies as a promising option to prevent cardiovascular complications in diabetes.

  6. The pathophysiologic aspects and clinical implications of electrocardiographic parameters of ventricular conduction delay in repaired tetralogy of Fallot.

    Science.gov (United States)

    Udink ten Cate, Floris E A; Sreeram, Narayanswami; Brockmeier, Konrad

    2014-01-01

    The 12-lead surface electrocardiogram is a valuable and feasible clinical tool in the management of patients following tetralogy of Fallot (TOF) repair. The importance of QRS duration in TOF patients has long been acknowledged. A prolonged QRS complex has been associated with increased risk for subsequent life-threatening ventricular arrhythmia and sudden cardiac death. Our current ability to risk-stratify TOF patients for malignant arrhythmogenic events primarily on the basis of QRS duration is rather limited. Nevertheless, increasing evidence suggests that QRS morphology and duration may be useful as surrogate markers of infundibular and regional right ventricular myocardial disease. The aim of this review is to provide a critical appraisal of the clinical implications of established and new electrocardiographic markers of ventricular conduction delay in TOF patients following surgical correction with a particular focus on QRS duration, lengthening, and fragmentation. In addition, the pathophysiological background of these parameters is addressed.

  7. Restless legs syndrome in Parkinson's disease: clinical characteristics and biochemical correlations

    Directory of Open Access Journals (Sweden)

    Tiago Machado Guerreiro

    2010-12-01

    Full Text Available Restless legs syndrome (RLS is a neurological disorder that responds to dopaminergic drugs, indicating a common pathophysiology with Parkinson's disease (PD. The prevalence of RLS was estimated in a group of PD patients and its clinical and biochemical characteristics were analysed. Forty-eight patients with PD were evaluated into two groups, with and without RLS. Clinical characteristics assessed in both groups were age, gender, duration of PD, Hoehn and Yahr, and Schwab and England scales. Laboratory variables such as hemoglobin, s-iron, s-ferritin and creatinine were obtained. The prevalence of RLS was 18.75%. No significant differences regarding clinical variables and biochemical parameters were observed. The high prevalence of RLS found in PD patients suggests the concept of a common etiological link and it seems that secondary causes did not play a central role in the pathophysiology of RLS in this group of parkinsonian patients.

  8. Morphology, Biochemistry, and Pathophysiology of MENX-Related Pheochromocytoma Recapitulate the Clinical Features.

    Science.gov (United States)

    Wiedemann, Tobias; Peitzsch, Mirko; Qin, Nan; Neff, Frauke; Ehrhart-Bornstein, Monika; Eisenhofer, Graeme; Pellegata, Natalia S

    2016-08-01

    Pheochromocytomas (PCCs) are tumors arising from neural crest-derived chromaffin cells. There are currently few animal models of PCC that recapitulate the key features of human tumors. Because such models may be useful for investigations of molecular pathomechanisms and development of novel therapeutic interventions, we characterized a spontaneous animal model (multiple endocrine neoplasia [MENX] rats) that develops endogenous PCCs with complete penetrance. Urine was longitudinally collected from wild-type (wt) and MENX-affected (mutant) rats and outputs of catecholamines and their O-methylated metabolites determined by mass spectrometry. Adrenal catecholamine contents, cellular ultrastructure, and expression of phenylethanolamine N-methyltransferase, which converts norepinephrine to epinephrine, were also determined in wt and mutant rats. Blood pressure was longitudinally measured and end-organ pathology assessed. Compared with wt rats, mutant animals showed age-dependent increases in urinary outputs of norepinephrine (P = .0079) and normetanephrine (P = .0014) that correlated in time with development of tumor nodules, increases in blood pressure, and development of hypertension-related end-organ pathology. Development of tumor nodules, which lacked expression of N-methyltransferase, occurred on a background of adrenal medullary morphological and biochemical changes occurring as early as 1 month of age and involving increased adrenal medullary concentrations of dense cored vesicles, tissue contents of both norepinephrine and epinephrine, and urinary outputs of metanephrine, the metabolite of epinephrine. Taken together, MENX-affected rats share several biochemical and pathophysiological features with PCC patients. This model thus provides a suitable platform to study the pathogenesis of PCC for preclinical translational studies aimed at the development of novel therapies for aggressive forms of human tumors.

  9. Huntington's disease: clinical characteristics, pathogenesis and therapies.

    Science.gov (United States)

    Nakamura, Ken; Aminoff, Michael J

    2007-02-01

    Huntington's disease is a devastating disorder with no known cure. The disease results from an expanded sequence of CAG repeats in the huntingtin gene and leads to a movement disorder with associated cognitive and systemic deficits. Huntington's disease is diagnosed by genetic testing and disease progression can be followed with a variety of imaging modalities. The accumulation of aggregated huntingtin with associated striatal degeneration is evident at autopsy. The pathophysiology of Huntington's disease remains unknown, although protein aggregation, excitotoxicity, deficits in energy metabolism, transcriptional dysregulation and apoptosis may all be involved. Current pharmacologic therapy for Huntington's disease is limited and exclusively symptomatic. However, the disease is being heavily researched, and a wide range of disease-modifying therapies is currently under development. The efficacy of these therapies is being evaluated in transgenic models of Huntington's disease and in preliminary clinical trials.

  10. [Pathophysiology of hormonal, immune, metabolic changes in acute and chronic pancreatitis. Experimental and clinical studies].

    Science.gov (United States)

    Trubitsyna, I E; Chikunova, B Z; Tkachenko, E V; Tsaregorodtseva, T M; Vinokurova, L V; Varvanina, G G

    2008-01-01

    There is literature review of the acute and chronic pancreatitis experimental models. Patogenetic necrosis mechanisms with fibrosis progress in pancreas were revealed. The stimulation of the proteolytic enzymes synthesis and secretion, that was examined in experiments were compared with clinical examinations. The patients with chronic pancreatitis were investigated in the Central Research Institute of Gastroenterology.

  11. Drug Insight: the role of leptin in human physiology and pathophysiology--emerging clinical applications.

    Science.gov (United States)

    Brennan, Aoife M; Mantzoros, Christos S

    2006-06-01

    Leptin is an adipocyte-secreted hormone with a key role in energy homeostasis. Studies in animal models, in humans with congenital complete leptin deficiency, and observational and interventional studies in humans with relative leptin deficiency (lower than normal leptin levels) have all indicated that leptin regulates multiple physiological functions, primarily in states of energy deficiency. This information led to proof-of-concept clinical trials involving leptin administration to individuals with relative or complete leptin deficiency. These conditions include congenital complete leptin deficiency, due to mutations in the leptin gene, and states of relative leptin deficiency including lipoatrophy and some forms of hypothalamic amenorrhea. Leptin, in replacement doses, normalizes neuroendocrine, metabolic and immune function in patients with these conditions, but further clinical studies are required to determine its long-term efficacy and safety. Management of leptin-deficient states with replacement doses of leptin holds promise as a therapeutic option. In addition, elucidation of the mechanisms underlying leptin resistance, which characterizes hyperleptinemic states such as human obesity and diabetes, might provide novel therapeutic targets for these prevalent clinical problems.

  12. Pathophysiology, clinical features and radiological findings of differentiation syndrome/all-trans-retinoic acid syndrome

    Institute of Scientific and Technical Information of China (English)

    Luciano; Cardinale; Francesco; Asteggiano; Federica; Moretti; Federico; Torre; Stefano; Ulisciani; Carmen; Fava; Giovanna; Rege-Cambrin

    2014-01-01

    In acute promyelocytic leukemia, differentiation thera-py based on all-trans-retinoic acid can be complicated by the development of a differentiation syndrome(DS). DS is a life-threatening complication, characterized by respiratory distress, unexplained fever, weight gain, interstitial lung infiltrates, pleural or pericardial effusions, hypotension and acute renal failure. The diagnosis of DS is made on clinical grounds and has proven to be difficult, because none of the symptoms is pathognomonic for the syndrome without any definitive diagnostic criteria. As DS can have subtle signs and symptoms at presentation but progress rapidly, end-stage DS clinical picture resembles the acute respiratory distress syndrome with extremely poor prognosis; so it is of absolute importance to be conscious of these complications and initiate therapy as soon as it was suspected. The radiologic appearance resembles the typical features of cardiogenic pulmonary edema. Diagnosis of DS remains a great skill for radiologists and haematologist but it is of an utmost importance the cooperation in suspect DS, detect the early signs of DS, examine the patients’ behaviour and rapidly detect the complications.

  13. [Pathophysiological and clinical aspects of using L-arginine in cardiology and angiology].

    Science.gov (United States)

    Valeev, V V; Trashkov, A P; Kovalenko, A L; Vasil'ev, A G

    Presented herein is a review of scientific publications dedicated to studying the pharmacodynamics of L-arginine and possibilities of its clinical application. Interest to L-arginine is associated, first of all, with its role as a precursor in endogenous synthesis of nitric oxide (NO), playing an important role in regulation of the functional state of the vascular wall. According to numerous studies, oral and parenteral administration of L-arginine restores endothelial production of NO in such diseases as atherosclerosis, hypertension, type 2 diabetes mellitus, obliterating diseases of arteries of lower extremities. The NO-mediated effect of L-arginine manifests itself in increasing the capability of vessels to dilatation, decreasing blood platelet aggregation, and inhibiting proliferation of smooth muscle cells of vessels. The effect is most pronounced in patients presenting with hypercholesterolaemia and initially decreased reactivity of the blood channel. The mostly pronounced NO-mediated effect of L-arginine is observed in parenteral route of its administration. Prolonged administration of L-arginine slows down progression of atherosclerosis.

  14. Hyporeninemic hypoaldosteronism and diabetes mellitus:Pathophysiology assumptions,clinical aspects and implications for management

    Institute of Scientific and Technical Information of China (English)

    André; Gustavo; P; Sousa; Jo?o; Victor; de; Sousa; Cabral; William; Batah; El-Feghaly; Luísa; Silva; de; Sousa; Adriana; Bezerra; Nunes

    2016-01-01

    Patients with diabetes mellitus(DM) frequently develop electrolyte disorders,including hyperkalemia.The most important causal factor of chronic hyperkalemia in patients with diabetes is the syndrome of hyporeninemic hypoaldosteronism(HH),but other conditions may also contribute.Moreover,as hyperkalemia is related to the blockage of the renin-angiotensin-aldosterone system(RAAS) and HH is most common among patients with mild to moderate renal insufficiency due to diabetic nephropathy(DN),the proper evaluation and management of these patients is quite complex.Despite its obvious relationship with diabetic nephropathy,HH is also related to other microvascular complications,such as DN,particularly the autonomic type.To confirm the diagnosis,plasma aldosterone concentration and the levels of renin and cortisol are measured when the RAAS is activated.In addition,synthetic mineralocorticoid and/or diuretics are used for the treatment of this syndrome.However,few studies on the implications of HH in the treatment of patients with DM have been conducted in recent years,and therefore little,if any,progress has been made.This comprehensive review highlights the findings regarding the epidemiology,diagnosis,and management recommendations for HH in patients with DM to clarify the diagnosis of this clinical condition,which is often neglected,and to assist in the improvement of patient care.

  15. Sitosterolemia: a review and update of pathophysiology, clinical spectrum, diagnosis, and management.

    Science.gov (United States)

    Yoo, Eun-Gyong

    2016-03-01

    Sitosterolemia is an autosomal recessive disorder characterized by increased plant sterol levels, xanthomas, and accelerated atherosclerosis. Although it was originally reported in patients with normolipemic xanthomas, severe hypercholesterolemia have been reported in patients with sitosterolemia, especially in children. Sitosterolemia is caused by increased intestinal absorption and decreased biliary excretion of sterols resulting from biallelic mutations in either ABCG5 or ABCG8, which encode the sterol efflux transporter ABCG5 and ABCG8. Patients with sitosterolemia show extreme phenotypic heterogeneity, ranging from almost asymptomatic individuals to those with severe hypercholesterolemia leading to accelerated atherosclerosis and premature cardiac death. Hematologic manifestations include hemolytic anemia with stomatocytosis, macrothrombocytopenia, splenomegaly, and abnormal bleeding. The mainstay of therapy includes dietary restriction of both cholesterol and plant sterols and the sterol absorption inhibitor, ezetimibe. Foods rich in plant sterols include vegetable oils, wheat germs, nuts, seeds, avocado, shortening, margarine and chocolate. Hypercholesterolemia in patients with sitosterolemia is dramatically responsive to low cholesterol diet and bile acid sequestrants. Plant sterol assay should be performed in patients with normocholesterolemic xanthomas, hypercholesterolemia with unexpectedly good response to dietary modifications or to cholesterol absorption inhibitors, or hypercholesterolemia with poor response to statins, or those with unexplained hemolytic anemia and macrothrombocytopenia. Because prognosis can be improved by proper management, it is important to find these patients out and diagnose correctly. This review article aimed to summarize recent publications on sitosterolemia, and to suggest clinical indications for plant sterol assay.

  16. Canine and feline obesity: a review of pathophysiology, epidemiology, and clinical management

    Directory of Open Access Journals (Sweden)

    Loftus JP

    2014-12-01

    Full Text Available John P Loftus, Joseph J Wakshlag Cornell University College of Veterinary Medicine, Veterinary Medical Center, Ithaca, NY, USAAbstract: Canine and feline obesity rates have reached pandemic proportions and are similar to those in humans, with approximately 30%–40% of dogs and cats being overweight to obese. Obesity has been associated with other health problems, including osteoarthritis, renal disease, skin disease, insulin resistance, and neoplasia in dogs, while in cats obesity is associated with dermatological issues, diabetes mellitus, neoplasia, and urolithiasis. The health issues appear to be slightly different across the two species, which may be due to some inherent differences in the hormonal milieu involved in obesity that differs between the dog and the cat. In this review, we discuss the complicated nature of the pathogenesis of obesity, the hormonal stimulus for orexigenic and anorexigenic behavior, adipose tissue as an endocrine organ, and most importantly, clinical management of the number one disease in canine and feline medicine.Keywords: obesity, canine, feline, veterinary

  17. Rare hereditary red blood cell enzymopathies associated with hemolytic anemia - pathophysiology, clinical aspects, and laboratory diagnosis.

    Science.gov (United States)

    Koralkova, P; van Solinge, W W; van Wijk, R

    2014-06-01

    Hereditary red blood cell enzymopathies are genetic disorders affecting genes encoding red blood cell enzymes. They cause a specific type of anemia designated hereditary nonspherocytic hemolytic anemia (HNSHA). Enzymopathies affect cellular metabolism, which, in the red cell, mainly consists of anaerobic glycolysis, the hexose monophosphate shunt, glutathione metabolism, and nucleotide metabolism. Enzymopathies are commonly associated with normocytic normochromic hemolytic anemia. In contrast to other hereditary red cell disorders such as membrane disorders or hemoglobinopathies, the morphology of the red blood cell shows no specific abnormalities. Diagnosis is based on detection of reduced specific enzyme activity and molecular characterization of the defect on the DNA level. The most common enzyme disorders are deficiencies of glucose-6-phosphate dehydrogenase (G6PD) and pyruvate kinase (PK). However, there are a number of other enzyme disorders, often much less known, causing HNSHA. These disorders are rare and often underdiagnosed, and the purpose of this review. In this brief review, we provide an overview of clinically relevant enzymes, their function in red cell metabolism, and key aspects of laboratory diagnosis.

  18. Hyporeninemic hypoaldosteronism and diabetes mellitus: Pathophysiology assumptions, clinical aspects and implications for management.

    Science.gov (United States)

    Sousa, André Gustavo P; Cabral, João Victor de Sousa; El-Feghaly, William Batah; de Sousa, Luísa Silva; Nunes, Adriana Bezerra

    2016-03-10

    Patients with diabetes mellitus (DM) frequently develop electrolyte disorders, including hyperkalemia. The most important causal factor of chronic hyperkalemia in patients with diabetes is the syndrome of hyporeninemic hypoaldosteronism (HH), but other conditions may also contribute. Moreover, as hyperkalemia is related to the blockage of the renin-angiotensin-aldosterone system (RAAS) and HH is most common among patients with mild to moderate renal insufficiency due to diabetic nephropathy (DN), the proper evaluation and management of these patients is quite complex. Despite its obvious relationship with diabetic nephropathy, HH is also related to other microvascular complications, such as DN, particularly the autonomic type. To confirm the diagnosis, plasma aldosterone concentration and the levels of renin and cortisol are measured when the RAAS is activated. In addition, synthetic mineralocorticoid and/or diuretics are used for the treatment of this syndrome. However, few studies on the implications of HH in the treatment of patients with DM have been conducted in recent years, and therefore little, if any, progress has been made. This comprehensive review highlights the findings regarding the epidemiology, diagnosis, and management recommendations for HH in patients with DM to clarify the diagnosis of this clinical condition, which is often neglected, and to assist in the improvement of patient care.

  19. Variant angina and coronary artery spasm: the clinical spectrum, pathophysiology, and management.

    Science.gov (United States)

    Kusama, Yoshiki; Kodani, Eitaro; Nakagomi, Akihiro; Otsuka, Toshiaki; Atarashi, Hirotsugu; Kishida, Hiroshi; Mizuno, Kyoichi

    2011-01-01

    Variant angina is a form of angina pectoris that shows transient ST-segment elevation on electrocardiogram during an attack of chest pain. Ischemic episodes of variant angina show circadian variation and often occur at rest from midnight to early morning. Ischemic episodes also occur during mild exercise in the early morning. However, they are not usually induced by strenuous exercise in the afternoon. Other important clinical features of variant angina include the high frequency of asymptomatic ischemic episodes and the syncope that sometimes occur during the ischemic episodes. Syncope is due to severe arrhythmias, including ventricular tachycardia, ventricular fibrillation, and high-degree atrioventricular block. Coronary artery spasm is the mechanism of ischemic episodes in variant angina. The incidence of coronary artery spasm shows a racial difference and is higher in Japanese than in Caucasians. Coronary arteriograms are normal or near-normal in most Japanese patients with variant angina. Deficient basal release of nitric oxide (NO) due to endothelial dysfunction, and enhanced vascular smooth muscle contractility with the involvement of the Rho/Rho-kinase pathway are reported to play important roles in the pathogenesis of coronary artery spasm. Other precipitating factors of coronary artery spasm include imbalance in autonomic nervous activity, increased oxidative stress, chronic low-grade inflammation, magnesium deficiency, and genetic susceptibility. The genetic risk factors associated with coronary artery spasm include gene polymorphisms of endothelial NO synthase (NOS), paraoxonase, and other genes. Calcium channel blockers are extremely effective in preventing coronary spasm. The long-acting nitrate, nicorandil, and Rho-kinase inhibitor are also useful for inhibiting coronary artery spasm. Because variant angina can lead to acute myocardial infarction, fatal arrhythmias, and sudden death, early treatment is important. The prognosis of patients with

  20. Intracranial Infections: Clinical and Imaging Characteristics

    Energy Technology Data Exchange (ETDEWEB)

    Foerster, B.R.; Thurnher, M.M.; Malani, P.N.; Petrou, M.; Carets-Zumelzu, F.; Sundgren, P.C. [Dept. of Radiology, and Divisions of Infectious Diseases and G eriatric Medicine, Dept. of Internal Medicine, Univ. of Michigan Medical Center, Ann Arbor, MI (United States)

    2007-10-15

    The radiologist plays a crucial role in identifying and narrowing the differential diagnosis of intracranial infections. A thorough understanding of the intracranial compartment anatomy and characteristic imaging findings of specific pathogens, as well incorporation of the clinical information, is essential to establish correct diagnosis. Specific types of infections have certain propensities for different anatomical regions within the brain. In addition, the imaging findings must be placed in the context of the clinical setting, particularly in immunocompromised and human immunodeficiency virus (HIV)-positive patients. This paper describes and depicts infections within the different compartments of the brain. Pathology-proven infectious cases are presented in both immunocompetent and immunocompromised patients, with a discussion of the characteristic findings of each pathogen. Magnetic resonance spectroscopy (MRS) characteristics for several infections are also discussed.

  1. Clinical characteristics of fulminant hepatitis in pregnancy

    Institute of Scientific and Technical Information of China (English)

    Xiao-Mao Li; Lin Ma; Yue-Bo Yang; Zhong-Jie Shi; Shui-Sheng Zhou

    2005-01-01

    AIM: To investigate the clinical characteristics of fulminant hepatitis in pregnancy.METHODS: We compared and analyzed the etiology,clinical characteristics, and laboratory examinations of 25 cases of fulminant hepatitis in pregnancy and 30 cases of fulminant hepatitis not in pregnancy.RESULTS: HBV infection and chronic fulminant hepatitis were most common both in the pregnant and in the non-pregnant groups. Jaundice, digestive tract symptoms,increase of bilirubin and thrombinogen activity were the main manifestations. The incidence of hepatic encephalopathy (HE) and hepato-renal syndrome (HRS) was significantly different between the two groups. The incidence of preterm labor, dead fetus and neonatal asphyxia was high.CONCLUSION: Fulminant hepatitis is likely to occur in late pregnancy with more severe complications, which significantly influences maternity, perinatal fetus, and newborn.

  2. Reform in teaching preclinical pathophysiology.

    Science.gov (United States)

    Li, Yong-Yu; Li, Kun; Yao, Hong; Xu, Xiao-Juan; Cai, Qiao-Lin

    2015-12-01

    Pathophysiology is a scientific discipline that studies the onset and progression of pathological conditions and diseases, and pathophysiology is one of the core courses in most preclinical medical curricula. In China, most medical schools house a Department of Pathophysiology, in contrast to medical schools in many developed countries. The staff in Chinese Departments of Pathophysiology generally consists of full-time instructors or lecturers who teach medical students. These lecturers are sometimes lacking in clinic knowledge and experiences. To overcome this, in recent years, we have been trying to bring new trends in teaching pathophysiology into our curriculum. Our purpose in writing this article was to share our experiences with our colleagues and peers worldwide in the hope that the insights we have gained in pathophysiology teaching will be of some value to educators who advocate teaching reform in medical schools.

  3. [New antiepileptic drugs: characteristics and clinical applications].

    Science.gov (United States)

    Ohtsuka, Yoko

    2014-05-01

    New antiepileptic drugs (AEDs) that have been used in many other countries for more than 10 years have only recently became available for use in Japan. Gabapentin, topiramate, lamotrigine and levetiracetam were licensed for use in Japan between 2006 and 2010. Stiripentol for Dravet syndrome and rufinamide for Lennox-Gastaut syndrome were also approved in 2012 and 2013 as orphan drugs. Clinical trials of other new AEDs such as oxcarbazepine, vigabatrin, lacosamide, and perampanel are in progress. In this review, the general characteristics of the new AEDs are discussed with regards to their effectiveness, tolerability, drug interaction, safety and mechanisms of action. The effectiveness, of the new AEDs compared with established AEDs is also discussed. Clinical applications of the new AEDs, focusing on gabapentin, topiramate, lamotrigine and levetiracetam are also discussed based on our domestic experience as well as overseas reports.

  4. Pituitary gigantism: Causes and clinical characteristics.

    Science.gov (United States)

    Rostomyan, Liliya; Daly, Adrian F; Beckers, Albert

    2015-12-01

    Acromegaly and pituitary gigantism are very rare conditions resulting from excessive secretion of growth hormone (GH), usually by a pituitary adenoma. Pituitary gigantism occurs when GH excess overlaps with the period of rapid linear growth during childhood and adolescence. Until recently, its etiology and clinical characteristics have been poorly understood. Genetic and genomic causes have been identified in recent years that explain about half of cases of pituitary gigantism. We describe these recent discoveries and focus on some important settings in which gigantism can occur, including familial isolated pituitary adenomas (FIPA) and the newly described X-linked acrogigantism (X-LAG) syndrome.

  5. Clinic Characteristics of Varicella Zoster Myocarditis

    Institute of Scientific and Technical Information of China (English)

    2001-01-01

    Objective To analyze the clinic characteristics of varicella zoster myocarditis and to determine the diagnostic value of serum cardiac troponin I (cTnI ) on the disease. Methods Information of 58 hospitalized patients with varicella zoster was collected, and the incidence of varicella zoster myocarditis and clinic characteristics (ECG, cTnI, age and sex distribution, etc. ) were analyzed respectively. Results It was found that 36.2% of the patients developed myocarditis. The old and female persons were much more susceptible to varicella zoster myocarditis. All patients had responsive ECG manifestations, and the abnormal ST-T changes were more common than other ECG abnormalities. CTnI remained higher than normal and had significant diagnostic value. Most of the patients had good prognosis, only a few patients lasted a long time and even progressed into cardiomyopathy. Nearly all the patients came to see dermatologists when they felt ill initially. That would lead to more misdiagnosis. Conclusion Infection of varicella zoster can complicate myocarditis, we must pay more attention to the patients who suffer from varicella zoster especially in the aged and female; cTnI is an important and effective index for diagnosis of varicella zoster myocarditis.

  6. Trauma-associated tinnitus: audiological, demographic and clinical characteristics.

    Directory of Open Access Journals (Sweden)

    Peter M Kreuzer

    Full Text Available BACKGROUND: Tinnitus can result from different etiologies. Frequently, patients report the development of tinnitus after traumatic injuries. However, to which extent this specific etiologic factor plays a role for the phenomenology of tinnitus is still incompletely understood. Additionally, it remains a matter of debate whether the etiology of tinnitus constitutes a relevant criterion for defining tinnitus subtypes. OBJECTIVE: By investigating a worldwide sample of tinnitus patients derived from the Tinnitus Research Initiative (TRI Database, we aimed to identify differences in demographic, clinical and audiological characteristics between tinnitus patients with and without preceding trauma. MATERIALS: A total of 1,604 patients were investigated. Assessment included demographic data, tinnitus related clinical data, audiological data, the Tinnitus Handicap Inventory, the Tinnitus Questionnaire, the Beck Depression Inventory, various numeric tinnitus rating scales, and the World Health Organisation Quality of Life Scale (WHOQoL. RESULTS: Our data clearly indicate differences between tinnitus patients with and without trauma at tinnitus onset. Patients suffering from trauma-associated tinnitus suffer from a higher mental burden than tinnitus patients presenting with phantom perceptions based on other or unknown etiologic factors. This is especially the case for patients with whiplash and head trauma. Patients with posttraumatic noise-related tinnitus experience more frequently hyperacousis, were younger, had longer tinnitus duration, and were more frequently of male gender. CONCLUSIONS: Trauma before tinnitus onset seems to represent a relevant criterion for subtypization of tinnitus. Patients with posttraumatic tinnitus may require specific diagnostic and therapeutic management. A more systematic and - at best - standardized assessment for hearing related sequelae of trauma is needed for a better understanding of the underlying pathophysiology and

  7. Sydenham's chorea: clinical and evolutive characteristics

    Directory of Open Access Journals (Sweden)

    Maria Teresa Ramos Ascensão Terreri

    2002-01-01

    Full Text Available CONTEXT: During the last 12 years we have observed an increase in the frequency of Sydenham's chorea in our country. We have observed that some of our patients have presented recurrence of the chorea despite regular treatment with benzathine penicillin. OBJECTIVE: The aim of our study was to evaluate clinical and evolutive characteristics of Sydenham's chorea in a group of patients followed in our Pediatric Rheumatology Unit. TYPE OF STUDY: Retrospective study. SETTING: Section of Pediatric Rheumatology - Discipline of Allergy, Clinical Immunology and Rheumatology - Department of Pediatrics - UNIFESP - EPM. PARTICIPANTS: Two hundred and ninety patients with rheumatic fever followed between 1986 and 1999. METHODS: We reviewed the records of 290 patients with rheumatic fever followed between 1986 and 1999. All patients were diagnosed according to the revised Jones criteria (1992. We included 86 patients that presented Sydenham's chorea as one of the major criteria (one or more attacks and evaluated their clinical and evolutive characteristics as well the treatment. RESULTS: Fifty-five patients were girls and 31 were boys. The mean age at onset was 9.7 years and mean follow-up period was 3.6 years. The 86 Sydenham's chorea patients presented 110 attacks of chorea. We observed isolated chorea in 35% of the patients, and 25 (29% presented one or more recurrences. We included only 17 of the 25 patients for further analysis, with a total of 22 recurrences of which 14 were attacks of chorea, because it was not possible to precisely detect the interval between attacks in the other patients. The approximate interval between the attacks ranged from 4 to 96 months. In 71% of the patients there was no failure in the secondary prophylaxis with benzathine penicillin, which was performed every 3 weeks. CONCLUSION: Despite the regular use of secondary benzathine penicillin prophylaxis, children with rheumatic fever have a high risk of Sydenham's chorea

  8. Pathophysiology of cancer cachexia

    OpenAIRE

    Younes Riad N.; Noguchi Yoshikazu

    2000-01-01

    Cancer cachexia is a frequent complication observed in patients with malignant tumors. Although several decades have passed since the first focus on the metabolic dysfunction's associated with cancer, few effective therapeutic interventions have been successfully introduced into the medical armamentarium. The present study thoroughly reviews the basic pathophysiology of cancer cachexia and the treatment options already investigated in that field. Experimental and clinical studies were evaluat...

  9. Clinical Characteristics of Fuchs’ Uveitis Syndrome

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    Pınar Çakar Özdal

    2016-04-01

    Full Text Available Objectives: To evaluate the clinical and demographic properties of Fuchs’ uveitis syndrome (FUS in Turkish patients. Materials and Methods: The medical records of 161 patients with FUS followed in the Uveitis Division of Ulucanlar Eye Hospital between 1996 and 2014 were respectively reviewed. The mean age at diagnosis, sex, the number of affected eyes, follow-up period, clinical findings at presentation, complications during the follow-up period, medical and surgical treatments, and best corrected visual acuity at the initial and final visits were recorded. Results: The study included 171 eyes of 161 patients diagnosed with FUS. Of the patients, 94 (58.4% were female and 67 (41.6% were male. The mean age at presentation was 35.2±11.0 (11-65 years. The mean follow-up period was 23.5±32.8 (2-216 months. Ten (6.2% patients had bilateral involvement. The most common symptoms at presentation were decreased visual acuity or blurred vision in 63 (39.1% and floaters in 19 (11.8% patients. Clinical findings at presentation included diffuse small, round, white keratic precipitates in 128 (74.8% eyes, anterior chamber reaction in 82 (47.9%, vitreous cells in 122 (71.3%, heterochromia in 47 (27.4% and iris nodules in 32 (18.7% eyes. During the follow-up period, elevated intraocular pressure occured in 31 (18.1% eyes and the most common complication was cataract development (89 eyes, 52.0%. Conclusion: Heterochromia was observed in 27.4% of patients in our study. However, the diffuse small, round keratic precipitates, low-grade anterior chamber reaction and varying degrees of vitreous reaction are more common clinical characteristics that are helpful in making the diagnosis.

  10. Clinical Characteristics of Fuchs’ Uveitis Syndrome

    Science.gov (United States)

    Nalçacıoğlu, Pınar; Çakar Özdal, Pınar; Şimşek, Mert

    2016-01-01

    Objectives: To evaluate the clinical and demographic properties of Fuchs’ uveitis syndrome (FUS) in Turkish patients. Materials and Methods: The medical records of 161 patients with FUS followed in the Uveitis Division of Ulucanlar Eye Hospital between 1996 and 2014 were respectively reviewed. The mean age at diagnosis, sex, the number of affected eyes, follow-up period, clinical findings at presentation, complications during the follow-up period, medical and surgical treatments, and best corrected visual acuity at the initial and final visits were recorded. Results: The study included 171 eyes of 161 patients diagnosed with FUS. Of the patients, 94 (58.4%) were female and 67 (41.6%) were male. The mean age at presentation was 35.2±11.0 (11-65) years. The mean follow-up period was 23.5±32.8 (2-216) months. Ten (6.2%) patients had bilateral involvement. The most common symptoms at presentation were decreased visual acuity or blurred vision in 63 (39.1%) and floaters in 19 (11.8%) patients. Clinical findings at presentation included diffuse small, round, white keratic precipitates in 128 (74.8%) eyes, anterior chamber reaction in 82 (47.9%), vitreous cells in 122 (71.3%), heterochromia in 47 (27.4%) and iris nodules in 32 (18.7%) eyes. During the follow-up period, elevated intraocular pressure occured in 31 (18.1%) eyes and the most common complication was cataract development (89 eyes, 52.0%). Conclusion: Heterochromia was observed in 27.4% of patients in our study. However, the diffuse small, round keratic precipitates, low-grade anterior chamber reaction and varying degrees of vitreous reaction are more common clinical characteristics that are helpful in making the diagnosis. PMID:27800260

  11. Clinical Characteristics of Marfan Syndrome in Korea

    Science.gov (United States)

    Lim, A Young; Song, Ju Sun; Kim, Eun Kyoung; Jang, Shin Yi; Chung, Tae-Young; Choi, Seung-Hyuk; Sung, Kiick; Huh, June; Kang, I-Seok; Choe, Yeon Hyeon; Ki, Chang-Seok

    2016-01-01

    Background and Objectives Marfan syndrome (MFS) is a connective tissue disorder with autosomal dominant inheritance and a highly variable clinical spectrum. However, there are limited data available on the clinical features of Korean patients with MFS. The aim of the present study was to describe the clinical characteristics and outcomes of Korean patients with MFS. Subjects and Methods We included all patients who were diagnosed with MFS between January 1995 and May 2015 at a single tertiary medical center. Patients with an MFS-related disorder including MASS phenotype (myopia, mitral valve prolapse, borderline and non-progressive aortic root dilatation, skeletal findings, and striae), mitral valve prolapse syndrome, and ectopia lentis syndrome were excluded. A total of 343 Korean patients aged ≥15 years who satisfied the revised Ghent nosology were included. Results The mean patient age at diagnosis was 35.9±12.6 years and 172 (50.1%) patients were male. Median follow-up duration was 52.8 months. A total of 303 patients (88.6%) had aortic root dilatation with Z score ≥2 or aortic root dissection. Ectopia lentis was relatively less common (163 patients, 55.1%) and systemic score ≥7 was found in 217 patients (73.8%). Among 219 probands, a family history of MFS was present in 97 patients (44.5%) and sporadic cases in 121 patients (55.5%). Among the 157 probands who underwent genetic analysis, 141 (89.8%) had an FBN1 mutation associated with aortic root aneurysm/dissection. Aortic dissection (AD) or intramural hematoma (IMH) was identified in 110 patients (32.1%). Among the 221 patients without AD or IMH, descending aortic aneurysms were identified in 19 patients (8.6%). Two hundred thirteen patients (62%) underwent cardiovascular surgery of any type. Eight patients died during follow-up. Conclusion We described the clinical characteristics and outcomes of Korean MFS patients. Cardiovascular manifestations were commonly detected and FBN1 mutation was present

  12. Psychogenic tics: clinical characteristics and prevalence

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    Janik, Piotr

    2014-08-01

    Full Text Available Aim. Clinical characteristics and the prevalence of psychogenic tics (PT Methods. 268 consecutively examined patients aged 4 to 54 years (221 men, 47 females; 134 children, 134 adults with tic phenotype: Gilles de la Tourette syndrome (GTS, n = 255, chronic motor tics (n = 6, chronic vocal tics (n = 1, transient tics (n = 1, tics unclassified (n = 2, PT (n = 5 were analyzed. The diagnosis of tic disorders was made on the DSM-IV-TR criteria and mental disorders by psychiatrists. Results. PT were found in 5 patients (1.9%, aged 17 to 51 years, four men and one woman. The phenotype included vocalizations and complex movements. In none of the patients simple motor facial tics, inability to tic suppress, unchanging clinical pattern, peak severity from the beginning of the disease, lack of concern about the disease were present. The absence of premonitory urges, regression in unexpected positions, and the presence of atypical for GTS mental disorders were found in two persons. PT occurred in three persons in whom organic tics were present in childhood. Pharmacological treatment and psychotherapy were unsuccessful. In two persons spontaneous resolution occurred, in two patients the tics persist, in one person the course of PT is unknown. Conclusions. PT are rare and may occur in patients with organic tics. The most typical features of PT are: early onset in adulthood, lack of simple motor tics, inability to tic suppress. The diagnosis is established if a few atypical symptoms for organic tics occur.

  13. The Relationship of Proper Skin Cleansing to Pathophysiology, Clinical Benefits, and the Concomitant Use of Prescription Topical Therapies in Patients with Acne Vulgaris.

    Science.gov (United States)

    Levin, Jacquelyn

    2016-04-01

    Patients often perceive the cause of their acne to be related to a lack of proper cleansing, therefore many patients attempt to treat their acne either alone or with prescription therapy by frequent aggressive cleansing with harsh cleansing agents. Altered epidermal barrier function, inflammation, and Propionibacterium acnes are related to acne vulgaris (AV) pathophysiology; proper cleansing can favorably modulate the development of AV. The available clinical studies support gentle cleansing in AV by showing the ability to contribute to improving AV lesion counts and severity and minimizing the irritation seen with topical AV therapies such as retinoids and BP.

  14. Clinical and morphological characteristics of cutaneous melanoma.

    Science.gov (United States)

    Balaban, Jagoda; Ninković Baroš, Djuka; Grujić, Dragana; Starović, Dragana; Ćelić, Milanka

    2014-01-01

    The incidence of cutaneous melanoma has increased significantly worldwide over the last several decades. The aim of this study is to determine clinical and morphology characteristics of primary melanoma, since some of them are important prognostic factors. This retrospective study included 172 patients. The data were collected by the Consulting team for malignant skin tumors in the Banja Luka Clinical Centre from 2009 to 2011. We did not use dermoscopy as a diagnostic tool in our investigation. We determined that melanoma occurs equally commonly in both sexes, in women in the sixth decade and the seventh in men. The most common sub-type was nodular melanoma (59.5%, P<0.05), followed by superficial spreading (27.8%) and acral lentiginous melanoma (11.4%). The most common localization was on the back in men (34.3%) and on the legs in women (P<0.05). More than half of our patients (55.8%) had melanoma thickness from 1.0 to 4.0 mm, and 38% had a melanoma thicker than 4.0 mm. The average Breslow thickness is 4.6 mm. More women than men had melanoma thicker than 4 mm (P<0.05). Spread of the primary tumor localization was found in 31.4% of patients, more frequently in men than in women (P<0.05). In most cases it was abstraction of lymph nodes (P<0.05). The average thickness of the melanoma in our patients is much higher than the average in the world and the countries of Europe. The results of this study indicate a need for better unique regional registry in this part of Bosnia and Herzegovina and improvement of preventive measures in the early diagnosis of melanoma.

  15. Clinical characteristics of patients with conjunctivochalasis

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    Balci O

    2014-08-01

    Full Text Available Ozlem Balci Ophthalmology Department, Kolan Hospitalium Group, Istanbul, Turkey Purpose: To evaluate the clinical characteristics of patients with conjunctivochalasis (CCh. Methods and materials: This retrospective study enrolled 30 subjects diagnosed with conjunctivochalasis. Complete ophthalmic examination, including visual acuity assessment, slit-lamp examination, applanation tonometry, dilated funduscopy, tear break-up time, Schirmer 1 test, and fluorescein staining were performed in all patients. Age, sex, laterality, ocular history, symptoms, and clinical findings were recorded. Results: The study included 50 eyes from 30 cases. Ages ranged from 45 to 80 years, with a mean age of 65±10 years. CChs grading were as follows: 30 (60% eyes with grade 1 CCh; 15 (30% eyes with grade 2 CCh; and five (10% eyes with grade 3 CCh. CCh was located in the inferior bulbar conjunctiva in 45 (90% eyes, and in the remaining five (10% CCh was located in the superior bulbar conjunctiva. Ten (33.3% patients had no symptoms. Dryness, eye pain, redness, blurry vision, tired eye feeling, and epiphora were the symptoms encountered in the remaining twenty (63.6% patients. Altered tear meniscus was noted in all cases. The mean tear break-up time was 7.6 seconds. The mean Schirmer 1 test score was 7 mm. Pinguecula was found in ten patients. Conclusion: Dryness, eye pain, redness, blurry vision, and epiphora were the main symptoms in patients with CCh. Dryness, eye pain, and blurry vision were worsened during downgaze and blinking. So CCh should be taken into consideration in the differential diagnosis of chronic ocular irritation and epiphora. Keywords: ocular irritation, epiphora, dryness, eye pain, blurry vision

  16. [Clinical characteristics of pancreatitis after cardiovascular surgery].

    Science.gov (United States)

    Ikegami, H; Sumiyoshi, T; Ishizuka, N; Ueda, M; Inaba, T; Hosoda, S; Aomi, S; Endo, M; Hashimoto, A; Koyanagi, H

    1995-10-01

    Increases in pancreatic enzyme levels after cardiovascular surgery were studied, and their clinical characteristics evaluated. The subjects were 128 patients who had undergone cardiovascular surgery (65 patients after valve replacement, 32 after coronary bypass surgery and 31 after aortic artificial graft replacement). The pancreatic enzyme (serum amylase and lypase) levels were monitored serially before and after operation, and amylase fractions were measured at their peaks. The relationships of the peak lypase level with underlying cardiac diseases, background factors, factors related to surgery, factors related to the extracorporeal circulation, presence or absence of symptoms, and treatments were examined. The amylase level exhibited biphasic changes consisting of a peak in which salivary glands amylase (S type) was dominant and a peak in which pancreatic amylase (P type) was dominant. The second peak coincided with the peak lypase and occurred mostly 3 to 10 days after operation. The peak lypase level exceeded the normal range in 78% of all the patients. It exceeded 564 U/l, 4 times the normal value in 28% of the patients, many of whom were symptomatic. So, we recommended that these cases should be treated as "postoperative pancreatitis". A high peak lypase level showed a significant correlation with the history of gallbladder and pancreatic diseases and diabetes mellitus among the background factors and emergency operation and the use of IABP among the surgery-related factors.(ABSTRACT TRUNCATED AT 250 WORDS)

  17. Mesenchymal stem cells: characteristics and clinical applications.

    Directory of Open Access Journals (Sweden)

    Sylwia Bobis

    2007-01-01

    Full Text Available Mesenchymal stem cells (MSCs are bone marrow populating cells, different from hematopoietic stem cells, which possess an extensive proliferative potential and ability to differentiate into various cell types, including: osteocytes, adipocytes, chondrocytes, myocytes, cardiomyocytes and neurons. MSCs play a key role in the maintenance of bone marrow homeostasis and regulate the maturation of both hematopoietic and non-hematopoietic cells. The cells are characterized by the expression of numerous surface antigens, but none of them appears to be exclusively expressed on MSCs. Apart from bone marrow, MSCs are located in other tissues, like: adipose tissue, peripheral blood, cord blood, liver and fetal tissues. MSCs have been shown to be powerful tools in gene therapies, and can be effectively transduced with viral vectors containing a therapeutic gene, as well as with cDNA for specific proteins, expression of which is desired in a patient. Due to such characteristics, the number of clinical trials based on the use of MSCs increase. These cells have been successfully employed in graft versus host disease (GvHD treatment, heart regeneration after infarct, cartilage and bone repair, skin wounds healing, neuronal regeneration and many others. Of special importance is their use in the treatment of osteogenesis imperfecta (OI, which appeared to be the only reasonable therapeutic strategy. MSCs seem to represent a future powerful tool in regenerative medicine, therefore they are particularly important in medical research.

  18. Clinical characteristics of high grade foveal hypoplasia.

    Science.gov (United States)

    Park, Kyung-Ah; Oh, Sei Yeul

    2013-02-01

    To report clinical characteristics of high grade foveal hypoplasia. Patients with foveal hypoplasia of grade 3 or 4 on spectral domain optical coherence tomography according to a previously published scheme were enrolled. All patients underwent a full ophthalmologic assessment including visual acuity testing, slit lamp biomicroscopy, fundus examination, and evaluation of ocular alignment. The underlying causes of foveal hypoplasia were identified as albinism in five patients and aniridia in six patients. The mean logMAR visual acuity was 0.57 ± 0.24 (range 0.22-1.00) in the right eyes and 0.58 ± 0.21 (range 0.30-1.00) in the left eyes. On fundus examination in patients with albinism, two patients showed marked transparency, one patient showed moderate transparency, and two patients showed minimal transparency. Among six patients with aniridia, five patients showed normal macular pigmentation without macular reflex and one patient showed decreased macular pigmentation with no macular reflex. Patients with high grade macular hypoplasia tended to have poor visual acuities; however, the range of visual acuity was quite variable. Other factors associated with underlying disease could be the reason of this variability. Therefore, careful consideration should be given when assessing visual prognosis in foveal hypoplasia using optical coherence tomography.

  19. Herbal Hepatotoxicity: Clinical Characteristics and Listing Compilation.

    Science.gov (United States)

    Frenzel, Christian; Teschke, Rolf

    2016-04-27

    Herb induced liver injury (HILI) and drug induced liver injury (DILI) share the common characteristic of chemical compounds as their causative agents, which were either produced by the plant or synthetic processes. Both, natural and synthetic chemicals are foreign products to the body and need metabolic degradation to be eliminated. During this process, hepatotoxic metabolites may be generated causing liver injury in susceptible patients. There is uncertainty, whether risk factors such as high lipophilicity or high daily and cumulative doses play a pathogenetic role for HILI, as these are under discussion for DILI. It is also often unclear, whether a HILI case has an idiosyncratic or an intrinsic background. Treatment with herbs of Western medicine or traditional Chinese medicine (TCM) rarely causes elevated liver tests (LT). However, HILI can develop to acute liver failure requiring liver transplantation in single cases. HILI is a diagnosis of exclusion, because clinical features of HILI are not specific as they are also found in many other liver diseases unrelated to herbal use. In strikingly increased liver tests signifying severe liver injury, herbal use has to be stopped. To establish HILI as the cause of liver damage, RUCAM (Roussel Uclaf Causality Assessment Method) is a useful tool. Diagnostic problems may emerge when alternative causes were not carefully excluded and the correct therapy is withheld. Future strategies should focus on RUCAM based causality assessment in suspected HILI cases and more regulatory efforts to provide all herbal medicines and herbal dietary supplements used as medicine with strict regulatory surveillance, considering them as herbal drugs and ascertaining an appropriate risk benefit balance.

  20. Herbal Hepatotoxicity: Clinical Characteristics and Listing Compilation

    Directory of Open Access Journals (Sweden)

    Christian Frenzel

    2016-04-01

    Full Text Available Herb induced liver injury (HILI and drug induced liver injury (DILI share the common characteristic of chemical compounds as their causative agents, which were either produced by the plant or synthetic processes. Both, natural and synthetic chemicals are foreign products to the body and need metabolic degradation to be eliminated. During this process, hepatotoxic metabolites may be generated causing liver injury in susceptible patients. There is uncertainty, whether risk factors such as high lipophilicity or high daily and cumulative doses play a pathogenetic role for HILI, as these are under discussion for DILI. It is also often unclear, whether a HILI case has an idiosyncratic or an intrinsic background. Treatment with herbs of Western medicine or traditional Chinese medicine (TCM rarely causes elevated liver tests (LT. However, HILI can develop to acute liver failure requiring liver transplantation in single cases. HILI is a diagnosis of exclusion, because clinical features of HILI are not specific as they are also found in many other liver diseases unrelated to herbal use. In strikingly increased liver tests signifying severe liver injury, herbal use has to be stopped. To establish HILI as the cause of liver damage, RUCAM (Roussel Uclaf Causality Assessment Method is a useful tool. Diagnostic problems may emerge when alternative causes were not carefully excluded and the correct therapy is withheld. Future strategies should focus on RUCAM based causality assessment in suspected HILI cases and more regulatory efforts to provide all herbal medicines and herbal dietary supplements used as medicine with strict regulatory surveillance, considering them as herbal drugs and ascertaining an appropriate risk benefit balance.

  1. Clinical Characteristics and Treatment of Cardiomyopathies in Children.

    Science.gov (United States)

    Price, Jack F; Jeewa, Aamir; Denfield, Susan W

    2016-01-01

    Cardiomyopathies are diseases of the heart muscle, a term introduced in 1957 to identify a group of myocardial diseases not attributable to coronary artery disease. The definition has since been modified to refer to structural and or functional abnormalities of the myocardium where other known causes of myocardial dysfunction, such as systemic hypertension, valvular disease and ischemic heart disease, have been excluded. In this review, we discuss the pathophysiology, clinical assessment and therapeutic strategies for hypertrophic, dilated and hypertrophic cardiomyopathies, with a particular focus on aspects unique to children.

  2. ENT and speech disorders in children with Down's syndrome: an overview of pathophysiology, clinical features, treatments, and current management.

    Science.gov (United States)

    Venail, Frédéric; Gardiner, Quentin; Mondain, Michel

    2004-01-01

    Down's syndrome is the most commonly occurring genetic abnormality, involving about 1 in 600 births. The increasing life expectancy of individuals with Down's syndrome has revealed the presence of several unexpected pathological processes. Among these, ENT disorders hold an important place because of their high incidence and severity. Accurate knowledge of the pathophysiology underlying ENT disorders (facial dysmorphism, ear abnormalities, upper airway abnormalities, and immunodeficiency) allow an understanding of the reasons for the development of the upper airway obstruction, obstructive sleep apnea syndrome, subglottic stenosis, deafness, speech delay, and ENT infections that occur frequently in these children. Early screening and specific treatment may allow some of the long-term sequelae to be avoided, or at least their prognosis to be improved. In order to help health care professionals in their daily practice, this review makes a series of recommendations to allow them to develop a master plan for the ENT management of children with Down's syndrome. In children with Down's syndrome, ENT disorders occur frequently and are often severe. They develop owing to craniofacial, functional, and immune system abnormalities. Early screening and treatment allow improvements in long-term outcomes.

  3. Maladaptive reward-learning and impulse control disorders in patients with Parkinson's disease: a clinical overview and pathophysiology update.

    Science.gov (United States)

    Lee, Jee-Young; Jeon, Beom Seok

    2014-10-01

    Impulse control disorders (ICD) in Parkinson's disease (PD) are a disabling non-motor symptom with frequencies of 13-35% among patients receiving dopamine replacement therapy. ICD in PD is strongly associated with dopaminergic drug use, especially non-ergot dopamine agonists (DA). However, individual susceptibility and disease-related neural changes are also important contributors to the development of ICD. Discrepancies between nigrostriatal and mesolimbic dopaminergic degeneration and non-physiological administration of dopaminergic drugs may induce abnormal 'hyperstimulation' of the mesolimbic system, which alters reward-learning behaviors in PD patients. In addition, DA can make patients more impulsive during decision-making and seek risk-taking behaviors. DA intake is also related to the biased representation of rewards. Ultimately, loss of negative feedback control due to dysfunctional frontostriatal connections is necessary for the establishment of ICD in PD. The subsequent behavioral and neural changes are affected by PD treatment and disease progression; thus, proper treatment guidelines for physicians are needed to prevent the development of ICD. Future studies aimed at producing novel therapeutics to control the risk factors for ICD or treat ICD behaviors in PD are warranted. This review summarizes recent advances from epidemiological and pathophysiological studies on ICD in PD. Management principles and limitations of current therapeutics are briefly discussed.

  4. Clinical Characteristics and Treatment of Malignant Granuloma

    Institute of Scientific and Technical Information of China (English)

    Chongjin Feng; Junbing Guo; Yong Chen

    2005-01-01

    OBJECTIVE To analyse the clinical characteristics of malignant granuloma(MG) and evaluate its treatment.METHODS From March 1985 to May 1998, 101 cases of malignant granuloma were treated with radiation (RT) alone or radiotherapy followed by chemotherapy or chemotherapy followed by radiotherapy. For chemotherapy, a CCNU, COMP (CTX, VCR, MTX, PDN) or CHOP (CTX,ADM, VCR, PDN) regimen was given. Radiation was directed through the anterior field of the nose mainly for nasal and paranasal sinus malignant granuloma, and through the faciocervical field for malignant granuloma of Walderyer's ring or for patients with cervical lymphadenectasis. Total dose was 45-65 Gy over 5-6 weeks.RESULTS The overall 3-year, 5-year and 10-year survival rates were 78.2%, 56.1% and 39.7% respectively. The 5-year survival rate was: RT group 60.3%, RT + CHOP or CHOP + RT group 64.7% (P >0.05), RT +CCNU group 40%, and RT + COMP group 33.3%. The 5-year survival rates of patients with one involved focus and more than 2 involved foci were 75.6%(34/45) and 39.3% (22/56) (P<0.001). The 5-year survival rates of patients with or without body symptoms were 39.6% (18/48) and 67.9%(36/53) (P<0.05). The 5-year survival rate of the 50-60 Gy group and the <50Gy group were 60.1% (40/66) and 20% (1/5)(P <0.05). The local and regional recurrence rate was 20.8 % (21/101 ).CONCLUSION Radiotherapy alone should be the treatment of choice for patients with one site involvement and without body symptoms. Radiation fields should be large enough to include the potentially involved sites. The recommended dose is 50-60 Gy over 5-6 weeks. It is suggested that patients with more than 2 foci involvement and those with body symptoms should receive the combination therapy.

  5. Characteristics and Roles of Literacy Clinic Directors

    Science.gov (United States)

    Ortlieb, Evan; Pearce, Daniel L.

    2013-01-01

    A literacy clinic is an ideal setting where research and exploration often lead to breakthroughs in reading remediation; that information can then be transferred to classroom instruction (Morris, 2003). Although it is clear that literacy clinics should be structured around what works for their student populations, there remains ambiguity…

  6. Clinical Characteristics of Fuchs’ Uveitis Syndrome

    OpenAIRE

    2016-01-01

    Objectives: To evaluate the clinical and demographic properties of Fuchs’ uveitis syndrome (FUS) in Turkish patients. Materials and Methods: The medical records of 161 patients with FUS followed in the Uveitis Division of Ulucanlar Eye Hospital between 1996 and 2014 were respectively reviewed. The mean age at diagnosis, sex, the number of affected eyes, follow-up period, clinical findings at presentation, complications during the follow-up period, medical and surgical treatments, and best cor...

  7. Clinical Characteristics of Fuchs’ Uveitis Syndrome

    OpenAIRE

    2016-01-01

    Objectives: To evaluate the clinical and demographic properties of Fuchs’ uveitis syndrome (FUS) in Turkish patients. Materials and Methods: The medical records of 161 patients with FUS followed in the Uveitis Division of Ulucanlar Eye Hospital between 1996 and 2014 were respectively reviewed. The mean age at diagnosis, sex, the number of affected eyes, follow-up period, clinical findings at presentation, complications during the follow-up period, medical and surgical treatments, and...

  8. Clinical characteristics of idiopathic portal hypertension

    Institute of Scientific and Technical Information of China (English)

    Ozgur Harmanci; Yusuf Bayraktar

    2007-01-01

    Idiopathic portal hypertension is one of the interesting causes of portal hypertension. Even in very developed medical centers, this disorder is still one of the most important misdiagnoses of clinical practice. To inexperienced physicians, presenting esophageal varices and upper gastrointestinal bleeding usually prompt an unfortunate diagnosis of cirrhosis. A heterogenous clinical presentation and progression of this disorder should be recognized by physicians, and management should be directed towards some specific problems confined to this disorder. Although a genetic basis and other factors are implicated in its pathogenesis, exact underlying mechanism(s) is (are) unknown. In this review, we discuss the heterogeneity of idiopathic portal hypertension, its etiopathogenesis, clinical presentation and management issues. With the expectation of an excellent prognosis, a practicing gastroenterologist should be aware that "not all varices mean cirrhosis".

  9. An international survey on the interpretation of pigmentation using the C class of the Clinical, Etiological, Anatomical, Pathophysiological Classification

    Directory of Open Access Journals (Sweden)

    Christopher R. Lattimer

    2013-08-01

    Full Text Available Skin changes over the gaiter area like pigmentation, lipodermatosclerosis and eczema are a clinical sign of advanced chronic venous disorder. This is documented as C4 in the Clinical, Etiological, Anatomical, Pathophysio logical (CEAP classification. The hypothesis was that there is great variability whether skin changes are recorded as early or advanced disease. The aim was to evaluate different patterns of skin changes by delegates at 3 international venous conferences. Seven high-definition, A4-sized, color photographs were taken of untreated legs with skin changes from patients attending a public hospital venous clinic. They all had venous disease confirmed on duplex with deep or superficial vein reflux >0.5 s. The photographs were displayed and a questionnaire was provided. Delegates familiar with CEAP were asked to choose from 3 C class options for each photograph. The responses were summarized by grouping them into mild (C0-3 and severe (C4-6. A total of 117 delegates completed the questionnaire from 30 countries. A percentage of 60 had practiced phlebology >10 years. The percentages of responders scoring mild (C0-3 and severe disease (C4-6 were: mild/severe=3/96 (photo 1, 65/33 (photo 2, 31/67 (photo 3, 56/34 (photo 4, 74/21 (photo 5, 89/10 (photo 6 and 37/59 (photo 7. The median percentage measure of agreement was 36.8 [95% confidence interval (CI: 24.8- 48.9]. The range was 23.2 (95% CI: 10.5-36.0 to 94.8 (95% CI: 90.7-98.9, P<0.001/image, Fisher exact test. This indicates a significant difference of opinion between the appearances of mild and severe clinical disease. Clinical decisions using the C class as a sign of advanced disease may be unreliable if used alone for recording severity, grouping patients or rationing treatment.

  10. Clinical presentation, pathophysiology, diagnosis, and treatment of acquired and hereditary angioedema: Exploring state-of-the-art therapies in RI.

    Science.gov (United States)

    Guo, Canting; Settipane, Russell A

    2016-06-01

    Hereditary and acquired angioedema are potentially life-threatening diseases characterized by spontaneous episodes of subcutaneous and submucosal swelling of face, lips, oral cavity, larynx, and GI tract. Hereditary angioedema (HAE) usually presents within the first and second decades of life, whereas acquired angioedema presents in adults after 40 years of age. These clinical symptoms together with reduced C1 inhibitor levels and/or activity can usually confirm the diagnosis. In recent years, multiple novel therapies for treating hereditary angioedema have emerged including C1 inhibitor concentrates, ecallantide/kallikrein inhibitor, and icatibant/bradykinin receptor antagonist. This article reviews the clinical presentation, diagnosis, treatment, and prophylaxis of HAE. Lastly, this article takes into consideration that, in reality, acute care treatment can often be limited by each hospital's formulary, included is a review of HAE treatments available at the nine major hospitals in Rhode Island. [Full article available at http://rimed.org/rimedicaljournal-2016-06.asp, free with no login].

  11. Pathophysiology of dilatation of pupils due to scorpion and snake envenomation and its therapeutic value: Clinical observations

    Science.gov (United States)

    Bawaskar, Himmatrao S; Bawaskar, Parag H; Bawaskar, Pramodini H

    2017-01-01

    Dilated nonreacting pupils are routinely taken as a sign of irreversible brain damage. Alpha-receptor stimulation (scorpion sting) and presynaptic acetylcholine receptor blocker (krait bite) may result in dilation of pupils without involvement of the brain. This study was aimed to clinically evaluate the response of pupils in scorpion sting and krait bite. Victims of scorpion sting and krait bite were chosen from Raigad district. Scorpion sting and krait bite cases were admitted to hospital and were clinically evaluated in detail regarding neurological manifestations. Both cases had nonreacting dilation of pupils, complete neurological recovery accompanied with reverse of pupillary size and its response to light. In scorpion sting and krait bite poisoning, dilated nonreacting pupils are not the signs of irreversible brain damage. PMID:28300747

  12. Clinical characteristics and current treatment of glaucoma.

    Science.gov (United States)

    Cohen, Laura P; Pasquale, Louis R

    2014-06-02

    Glaucoma is a neurodegenerative disorder in which degenerating retinal ganglion cells (RGC) produce significant visual disability. Clinically, glaucoma refers to an array of conditions associated with variably elevated intraocular pressure (IOP) that contributes to RGC loss via mechanical stress, vascular abnormalities, and other mechanisms, such as immune phenomena. The clinical diagnosis of glaucoma requires assessment of the ocular anterior segment with slit lamp biomicroscopy, which allows the clinician to recognize signs of conditions that can produce elevated IOP. After measurement of IOP, a specialized prismatic lens called a gonioscope is used to determine whether the angle is physically open or closed. The structural manifestation of RGC loss is optic nerve head atrophy and excavation of the neuroretinal rim tissue. Treatment is guided by addressing secondary causes for elevated IOP (such as inflammation, infection, and ischemia) whenever possible. Subsequently, a variety of medical, laser, and surgical options are used to achieve a target IOP.

  13. [Pathophysiological and clinical correlations between endocrine and cardiovascular systems. An inter-systemic model of internal medicine].

    Science.gov (United States)

    Andreoli, Mario

    2006-12-01

    This review focuses on the mechanisms by which thyroid hormones affect the regulation of the cardiovascular system and the thermogenic and hemodynamic variation induced by thyroid disfunction. It is also stressed the hormonal role of the cardiac myocytes realising natriuretic peptides, involved in plasma volume homeostasis and cardiovascular remodelling; its rapid measurement is a useful clinical tool, in the diagnostic and prognostic of left ventricular dysfunction, correlating with the degree of the clinical symptoms. The endothelial layer is a receptor-effector endocrine organ that produces substances that maintain vasomotor balance and vascular-tissue homeostasis. Cardiovascular risk factors causes oxidative stress that alter endothelial function and leads to endothelial dysfunction. On the basis of the present body of evidence there is no doubt that endothelial dysfunction contributes to the initiation, and progression, of atherosclerotic disease and that it could be considered an independent vascular risk factor for the micro- and macrovascular damages in the diabetes disease. In several extrathyroidal pathological condition, as well as in heart failure, the main alteration of the thyroid function is referred to as "low T3 syndrome". This syndrome is due to an adaptative reaction of the metabolic pathway of thyroxine, producing an increased amount of rT3, metabolically inactive, thus decreasing the detrimental metabolic effects of T3, in conditions of critically impaired hemodynamic and metabolic efficiency. Preliminary clinical trials, in heart failure, suggest the prognostic value of the level of circulating T3, as well as usefulness of T3, or of thyromimetic derivatives (DITPA), in chronic treatment of the heart ventricular dysfunction.

  14. Neonatal peripheral hypotonia: clinical and electromyographic characteristics.

    Science.gov (United States)

    Parano, E; Lovelace, R E

    1993-06-01

    Hypotonia is a common occurrence in pediatrics, especially in the neonatal period. The hypotonic neonate represents a diagnostic challenge for the general pediatrician because hypotonia may be caused by a lesion at any level in the neuraxis: (1) central nervous system (CNS), (2) peripheral nerves (PN), (3) neuromuscular junction, or (4) muscles. Distinguishing among these pathologies is a particularly arduous task. This review will discuss the clinical approach to neonatal hypotonia with emphasis on disorders of the peripheral nervous system and muscle, and the importance of the electrophysiological study as a diagnostic test.

  15. Candidiasis: pathogenesis, clinical characteristics, and treatment.

    Science.gov (United States)

    Appleton, S S

    2000-12-01

    Candida organisms live on the skin and mucous membranes of up to 75 percent of the population. They can live commensally without causing harm or can change to an aggressive form and invade tissue, causing both acute and chronic disease in the host. Oropharyngeal candidiasis manifests clinically as acute pseudomembranous, acute atrophic, chronic atrophic, chronic hypertrophic/hyperplastic, and angular cheilitis. Systemic infection leading to candidemia can be devastating and cause up to a 60 percent mortality rate in medical or post-surgical intensive care wards. Oral nystatin, clotrimazole, and fluconazole usually provide appropriate therapy; although resistance to medications is increasing, particularly in immunocompromised hosts.

  16. Hydatidiform mole clinical and pathological characteristics

    OpenAIRE

    Pacheco D., José; Instituto de Patología, UNMSM; Instituto de Medicina Legal; Alegre, Jaime; Hospital Nacional Dos de Mayo, Lima, Perú; Paniagua, Gissela; Instituto de Patología de la UNMSM

    2013-01-01

    Objective: To determine clinical and pathological findings in hydatidiform mole. Design: Descriptive retrospective study. Material and methods: All cases de molar pregnancy attended at Dos de Mayo National Hospital (HNDM) between 1998 and 2001 were reviewed. Results: Twenty-four cases of hydatidiform mole were attended; mean age was 26,4 years ± 6,4 and gestational age 13,1 weeks ± 3,3. Past history only showed 1 and 3 cases of hyperemesis gravidarum and previous abortions, respectively; 18 p...

  17. Acute demyelinating encephalomyelitis: Clinical characteristics and outcome

    Directory of Open Access Journals (Sweden)

    Ahmed Farag Elhassanien

    2013-01-01

    Full Text Available Background: ADEM, although relatively uncommon, is probably under-recognized. Objectives: To spotlight the clinical profile and therapeutic outcome of children with ADEM. Materials and Methods: This is a prospective study of patients with ADEM who were admitted to the Pediatric Departments in Aladan and Alfarawanya Hospitals in Kuwait, from January 2009 to January 2011. Clinical, microbiological and radiological data were analyzed. Results: Of 48 patients presented with acute neurological symptoms and signs, 21 patients fulfilled criteria for ADEM. 80.95% of cases were presenting in winter and spring, 57% of patients had a history of upper respiratory tract illness. The commonest presentations were motor deficits, convulsions and altered consciousness. CSF virology studies showed herpes simplex virus (HSV and Epstein-Barr virus (EBV (3 patients whereas nasal and nasopharyngeal swab showed evidence of influenza H1N1 virus (1 patient. Brain MRI was performed in all patients and revealed multiple hyperintense supratentorial brain lesions on T2/FLAIR images. 85.7% of patients had cortical and/or subcortical white matter lesions which were bilateral and asymmetric in location and size. Conclusion: ADEM although rare must be considered in children with acute onset of neurological signs and symptoms and must be distinguished from any acute neurological insult.

  18. Near Fatal Asthma: Clinical and Airway Biopsy Characteristics

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    Richard G. Barbers

    2012-01-01

    Full Text Available Background. Inflammation and remodeling are integral parts of asthma pathophysiology. We sought to describe the clinical and pathologic features of near fatal asthma exacerbation (NFE. Methods. Bronchial biopsies were collected prospectively from NFE I subjects. Another NFE II group and a moderate severity exacerbation control group (ME II were retrospectively identified—no biopsies obtained. Results. All NFE II (=9 subjects exhibited remodeling and significant inflammation (eosinophilic, neutrophilic. NFE II group (=37 had a significant history of prior intubation and inhaled corticosteroids usage compared to ME II group (=41. They also exhibited leukocytosis, eosinophilia, and longer hospitalization days. Conclusions. Remodeling, eosinophilic, and neutrophilic inflammation were observed in NFE. NFE is associated with prior intubation and inhaled corticosteroids usage.

  19. Workshop report: Schistosomiasis vaccine clinical development and product characteristics.

    Science.gov (United States)

    Mo, Annie X; Colley, Daniel G

    2016-02-17

    A schistosomiasis vaccine meeting was organized to evaluate the utility of a vaccine in public health programs, to discuss clinical development paths, and to define basic product characteristics for desirable vaccines to be used in the context of schistosomiasis control and elimination programs. It was concluded that clinical evaluation of a schistosomiasis vaccine is feasible with appropriate trial design and tools. Some basic Preferred Product Characteristics (PPC) for a human schistosomiasis vaccine and for a veterinary vaccine for bovine use were also proposed.

  20. Clinical characteristics of 297 newly diagnosed Chinese HIV / AIDS patients

    Institute of Scientific and Technical Information of China (English)

    曹玮

    2014-01-01

    Objective To determine the clinical characteristics of HIV infected patients in China in order to improve early recognition and diagnosis of AIDS.Methods A total of297 newly diagnosed HIV/AIDS patients were enrolled in Peking Union Medical College Hospital(PUMCH)from January 2001 to December 2012,including 19 patients of primary phase,115 of asymptomatic phase and 163 of AIDS phase.Clinical characteristics of these patients were retrospectively analyzed.Results Two hundred and

  1. Clinical characteristics of roxatidine acetate: a review.

    Science.gov (United States)

    Dammann, H G; de Looze, S M; Bender, W; Labs, R

    1988-01-01

    Pharmacodynamic studies revealed that 150 mg of roxatidine acetate were optimal in suppressing gastric acid secretion, and that a single bedtime dose of 150 mg was more effective than a dose of 75 mg twice daily in terms of inhibiting nocturnal acid secretion. When administered orally as a capsule containing a granule formulation, the drug displayed modified-release properties, which led to a sustained suppression of gastric acid secretion. Clinical trials revealed that roxatidine acetate, 75 mg twice daily and 150 mg at night, was highly effective in healing duodenal and gastric ulcers and in reducing ulcer pain, over 4, 6, and 8 weeks of therapy. A steady reduction in diameter was observed in those ulcers not completely healed during therapy. The single bedtime dose regimen, while producing the same degree of healing as the divided daily dose during controlled clinical trials, may be of greater value in therapeutic use owing to improved patient compliance. In all efficacy criteria (cure, reduction in ulcer size, and pain relief) there was no significant difference between roxatidine acetate in a total daily dose of 150 mg, ranitidine in a total daily dose of 300 mg, and cimetidine in a total daily dose of 800 mg. Prevention of gastric and duodenal ulcer relapse was achieved by roxatidine acetate, 75 mg at night for 6 months, in about 70% of patients, as determined in open, pilot studies--a rate comparable to those reported for cimetidine and ranitidine. Roxatidine acetate shares with ranitidine an improved safety profile when compared with cimetidine. Human pharmacology studies and short-term and long-term clinical trials have all shown that roxatidine acetate is an exceptionally well tolerated compound, without the antiandrogenic activity and interference with hepatic drug metabolism which have characterized cimetidine treatment. A reason for the improved safety profile of roxatidine acetate may be its greater potency than cimetidine (six times less potent) and

  2. Neurovascular pathophysiology in cerebral ischemia, dementia and the ageing brain – current trends in basic, translational and clinical research

    Directory of Open Access Journals (Sweden)

    Boltze Johannes

    2012-08-01

    Full Text Available Abstract The 7th International Symposium on Neuroprotection and Neurorepair was held from May 2nd to May 5th, 2012 in Potsdam, Germany. The symposium, which directly continues the successful Magdeburg meeting series, attracted over 330 colleagues from 29 countries to discuss recent findings and advances in the field. The focus of the 2012 symposium was widened from stroke and traumatic brain injury to neurodegenerative diseases, notably dementia, and more generally the ageing brain. Thereby, emphasis was given on neurovascular aspects of neurodegeneration and stroke including the blood–brain barrier, recent findings regarding the pathomechanism of Alzheimer’s disease, and brain imaging approaches. In addition, neurobiochemical aspects of neuroprotection, the role of astrogliosis, the clinical progress of cell-based approaches as well as translational hurdles and opportunities were discussed in-depth. This review summarizes some of the most stimulating discussions and reports from the meeting.

  3. Clinical characteristics of eosinophilic asthma exacerbations

    DEFF Research Database (Denmark)

    Bjerregaard, Asger; Laing, Ingrid A; Backer, Vibeke;

    2016-01-01

    BACKGROUND AND OBJECTIVE: Airway eosinophilia is associated with an increased risk of asthma exacerbations; however, the impact on the severity of exacerbations is largely unknown. We describe the sputum inflammatory phenotype during asthma exacerbation and correlate it with severity and treatment...... response. METHODS: Patients presenting to hospital with an asthma exacerbation were recruited during a 12-month period and followed up after 4 weeks. Induced sputum was collected at both visits. Patients underwent spirometry, arterial blood gas analysis, fractional exhaled nitric oxide analysis, white...... with a sensitivity of 86% and a specificity of 70%. CONCLUSION: Our findings suggest that eosinophilic asthma exacerbations may be clinically more severe than NEEs, supporting the identification of these higher risk patients for specific interventions....

  4. Ovarian fibroma--clinical and histopathological characteristics.

    Science.gov (United States)

    Sivanesaratnam, V; Dutta, R; Jayalakshmi, P

    1990-11-01

    Twenty-three cases of ovarian fibroma, comprising 3% of all benign tumors seen over a 20-year period, were analyzed. It was unilateral in all cases affecting more commonly the left ovary (70%). Whilst a majority of cases (77%) were encountered in the reproductive age group, the tumor was rare before the second decade. Only in 13% of cases was ascitis clinically detectable. This was not influenced by the size and weight (average of 9.3 x 10.8 x 11.1 cm and 959 g, respectively) of the tumors; a smooth-surfaced tumor was, however, associated with a greater amount of peritoneal fluid. Varying degrees of calcification in some tumors are detectable on ultrasonography and occasionally on abdominal radiography. The classical Meig's Syndrome was seldom encountered. The histopathological features, diagnostic problems and management are discussed.

  5. [Clinical and echocardiographic characteristics of scimitar syndrome].

    Science.gov (United States)

    Espinola-Zavaleta, Nilda; Játiva-Chávez, Silvio; Muñoz-Castellanos, Luis; Zamora-González, Carlos

    2006-03-01

    Seven patients with scimitar syndrome underwent retrospective clinical and echocardiographic examination. The findings were: respiratory infection with dyspnea on moderate exercise in 90%, scimitar sign in four (57%), dextrocardia in five (71%), and interatrial septal defect in five (71%), one of whom had patent ductus arteriosus. Overall, two patients had patent ductus arteriosus: one also had aortic coarctation and the other, a bicuspid aortic valve. Dilation of the right cavities was found in five (71%) and blunt edge in five (71%). In two patients, anomalous drainage was into the right atrium; in another two, into the inferior vena cava; and in three, towards the junction of the right atrium and the inferior vena cava. In three patients, drainage was obstructed. Six patients with cardiac abnormalities proceeded to surgery. Scimitar syndrome is a rare entity. In the large majority of patients (86%), diagnosis and follow-up can be performed echocardiographically.

  6. Clinical characteristics and treatment outcomes of severe ulcerative colitis

    Institute of Scientific and Technical Information of China (English)

    詹翔

    2014-01-01

    Objective To explore the clinical characteristics and medicine treatment of patients with severe ulcerative colitis(UC),the efficacy of rescue treatment in patients with glucocorticoid(GCS)resistant severe UC,and the clinical risk factors in patients with GCS-refractory severe UC.Methods From January 2001 to December 2012,

  7. Radiation-induced cataract-genesis: pathophysiologic, radiobiological and clinical aspects; Catarate radio-induite: aspects physiopathologiques, radiobiologiques et cliniques

    Energy Technology Data Exchange (ETDEWEB)

    Belkacemi, Y.; Touboul, E.; Meric, J.B. [Hopital Tenon, Service d' Oncologie-Radiotherapie, 75 - Paris (France); Rat, P.; Warnet, J.M. [Paris-5 Univ., Lab. de Toxicologie, UFR Pharmacie, Unite de Pharmacotoxicologie Cellulaire, Service Pharmacie, 75 (France)

    2001-08-01

    Cataract-genesis is a widely reported late effect of irradiated crystalline lens. In this review the authors discussed the different aspects of radiation cataract pathogenesis, and the different mechanisms involved in the lens opacification, particularly the epithelium modifications such as epithelial cell death. The authors also reported the influence of radiation exposure on cataract formation following total body irradiation (TBI) and autologous or allogeneic bone marrow transplantation for hematologic malignancies. Moreover, the radiobiological parameters are not studied for the crystalline lens of human. We applied for the first time the linear quadratic (LQ) and biological effective dose (BED) concept to TBI data. The calculated value of {alpha}/{beta} of 1 Gy is in the range of the values reported for the other late responding tissues. The other risk factors for cataract development after TBI such as age, gender, central nervous system boost, long-term steroid therapy and heparin administration are discussed. In terms of cataract or sicca syndrome prevention, numerous compounds have been successfully tested in experimental models or used for the prevention of radiation-induced xerostomia in patients treated for head and neck cancer. However, none of them has been clinically evaluated for ocular radiation late effects prevention. In this report the authors discussed some of the radioprotectors potentially interesting for radiation-induced cataract or sicca syndrome prevention. (author)

  8. Clinical characteristics of patients with persistent postural-perceptual dizziness

    OpenAIRE

    Roseli Saraiva Moreira Bittar; Eliane Maria Dias von Söhsten Lins

    2015-01-01

    INTRODUCTION: Persistent postural-perceptual dizziness is the dizziness that lasts for over three months with no clinical explanation for its persistence. The patient's motor response pattern presents changes and most patients manifest significant anxiety. OBJECTIVE: To evaluate the clinical characteristics of patients with persistent postural and perceptual dizziness. METHODS: statistical analysis of clinical aspects of patients with persistent postural-perceptual dizziness. RESULTS: 81 pati...

  9. Ketosis-prone type 2 diabetes in patients of sub-Saharan African origin: clinical pathophysiology and natural history of beta-cell dysfunction and insulin resistance.

    Science.gov (United States)

    Mauvais-Jarvis, Franck; Sobngwi, Eugène; Porcher, Raphaël; Riveline, Jean-Pierre; Kevorkian, Jean-Philippe; Vaisse, Christian; Charpentier, Guillaume; Guillausseau, Pierre-Jean; Vexiau, Patrick; Gautier, Jean-François

    2004-03-01

    Nonautoimmune ketosis-prone diabetic syndromes are increasingly frequent in nonwhite populations. We have characterized a cohort of patients of sub-Saharan African origin who had ketosis-prone type 2 diabetes (n = 111), type 1 diabetes (n = 21), and type 2 diabetes (n = 88) and were admitted to a hospital for management of uncontrolled diabetes. We compared epidemiological, clinical, and metabolic features at diabetes onset and measured insulin secretion (glucagon-stimulated C-peptide) and insulin action (short intravenous insulin tolerance test) during a 10-year follow-up. Ketosis-prone type 2 diabetes shows a strong male predominance, stronger family history, higher age and BMI, and more severe metabolic decompensation than type 1 diabetes. In ketosis-prone type 2 diabetes, discontinuation of insulin therapy with development of remission of insulin dependence is achieved in 76% of patients (non-insulin dependent), whereas only 24% of patients remain insulin dependent. During evolution, ketosis-prone type 2 diabetes exhibit specific beta-cell dysfunction features that distinguish it from type 1 and type 2 diabetes. The clinical course of non-insulin-dependent ketosis-prone type 2 diabetes is characterized by ketotic relapses followed or not by a new remission. Progressive hyperglycemia precedes and is a strong risk factor for ketotic relapses (hazard ratio 38). The probability for non-insulin-dependent ketosis-prone type 2 diabetes to relapse is 90% within 10 years, of whom approximately 50% will become definitively insulin dependent. Insulin sensitivity is decreased in equal proportion in both ketosis-prone type 2 diabetes and type 2 diabetes, but improves significantly in non-insulin-dependent ketosis-prone type 2 diabetes, only after correction of hyperglycemia. In conclusion, ketosis-prone type 2 diabetes can be distinguished from type 1 diabetes and classical type 2 diabetes by specific features of clinical pathophysiology and also by the natural history of

  10. Clinical and behavioral characteristics in FG syndrome.

    Science.gov (United States)

    Graham, J M; Superneau, D; Rogers, R C; Corning, K; Schwartz, C E; Dykens, E M

    1999-08-27

    FG syndrome is a rare X-linked recessive form of mental retardation, first described by Opitz and Kaveggia in 1974. Based on over 50 reported cases, FG syndrome is associated with agenesis of the corpus callosum, minor facial anomalies (high, broad forehead with frontal cowlick, ocular hypertelorism, down-slanted palpebral fissures, and small cupped auricles), relative macrocephaly, broad thumbs and halluces, and prominent fetal fingertip pads. Affected individuals manifest neonatal hypotonia and severe constipation, which usually resolves during mid-childhood. The hypotonia with joint hyperlaxity evolves into spasticity with joint contractures in later life. Affability, hyperactivity, and excessive talkativeness are noted frequently in patients with FG syndrome. Recently, we described three additional families (six additional patients) with FG syndrome who support the localization of a gene for the FG syndrome in chromosome region Xq12-q21 [Graham JM Jr, Tackels D, Dibbern K, Superneau D, Rodgers C, Corning K, Schwartz CE. 1998. Am J Med Genet 80:145-156.]. Using these same families and one additional sporadic case of FG syndrome, we compared behavioral and personality characteristics of 6 FG boys with other boys with syndromic and nonsyndromic mental retardation: eight with Down syndrome, seven with Prader-Willi syndrome, eight with nonspecific mental retardation, and 13 with Williams syndrome. Using the Vineland Adaptive Behavior Scales, the Reiss Personality Profiles, and the Achenbach Child Behavior Checklist, parents were asked to characterize the behavior and personality of their boys from ages 4 to 10 years. When compared with Williams syndrome, the FG boys had fewer internalizing behaviors and were significantly less anxious and withdrawn but had similar socially oriented, attention-seeking behaviors. On the Reiss Profile, FG boys were also quite similar to Williams syndrome boys. On the Vineland Scales, FG boys demonstrated significant relative strengths

  11. Pneumonias virais: aspectos epidemiológicos, clínicos, fisiopatológicos e tratamento Viral pneumonia: epidemiological, clinical, pathophysiological and therapeutic aspects

    Directory of Open Access Journals (Sweden)

    Luiz Tadeu Moraes Figueiredo

    2009-09-01

    Full Text Available As infecções do trato respiratório são as formas de infecção mais comuns que afetam o homem e, dentre essas, predominam as de causa viral. Os vírus também podem acometer o trato respiratório baixo, causando bronquiolite, bronquite e pneumonia. Neste artigo de revisão, objetivamos mostrar aspectos epidemiológicos, fisiopatológicos, clínicos e do tratamento das pneumonias comunitárias por vírus. Essas pneumonias costumam ser causadas por vírus influenza A e B; parainfluenza 1, 2 e 3; vírus respiratório sincicial; e adenovírus. Também são apresentados aqui os hantavírus, metapneumovírus e rinovírus causando pneumonia.In humans, the most common types of infection are respiratory tract infections, among which viral infections predominate. Viruses can also infect the low respiratory tract, causing bronchiolitis, bronchitis and pneumonia. The objective of this review article was to show epidemiological, pathophysiological, clinical and therapeutic aspects of viral community-acquired pneumonia. These types of pneumonia are commonly caused by influenza A and B; parainfluenza 1, 2 and 3; respiratory syncytial virus; or adenovirus. We also address the types of pneumonia caused by hantaviruses, metapneumoviruses and rhinoviruses.

  12. Clinic Characteristics of Varicella Zoster Myocarditis

    Institute of Scientific and Technical Information of China (English)

    FANG; Wu-wang

    2001-01-01

    [1]Kokstein Z, Balatka J, Horacek J. ECG changes in children with acute myocarditis[J]. Sb Ved Pr Lek Fak Karlovy Univerzity Hradci Kralove Suppl, 1990, 33(4): 373-379.[2]Rich R, McErlean M. Complete heart block in a child with varicella[J]. Am J Emerg M, 1993, 11(6): 602-605.[3]Sobrino MJM, Lopez PF, Cisneros JM, et al. Thoracic pain as early clinical manifestation of myocarditis caused by varicella [J]. Med Clin Barc,1992, 99(6): 236-237.[4]Teravanichpong S, Chuangsuwanich T. Fatal varicella in a healthy girl[J]. J Med Assoc Thai, 1990, 73(11): 648-651.[5]Amral FT, Bestetti RB, Araujo RC, et al. Transient atroventricular conduction disordor: a potential cause of sudden death in myocarditis due to varicella[J]. Arq Bras Cardiol, 1989, 53(2): 129-131.[6]Lorber A, Zonis Z, Maisuls E, et al. The scale of myocardial involvement in varicella myocarditis[J]. Int J Cardiol,1988, 20(2): 257-262.[7]Ettedgui JA, Ladusans E, Bamford M. Complete heart block as a complication of varicella[J]. Int J Cardiol, 1987, 14(3): 362-365.[8]Waagner DC, Murphy TV. Varicella myocarditis[J]. Pediatr Infect Dis J, 1990, 9(5): 360-363.[9]Rivera CF, Omar M, Aliaga ML, et al. Varicella complicated by pericarditis and pneumonia [J]. An Med Interna, 1996, 13(3): 130-132.[10]Civico RF, Omar M, Martinez LA, et al. Varicella complicated by pericarditis and pneumonia [J]. An Med Interna, 1996, 13(3): 130-132.[11]Bachli E, Kagi MK, Krause M. Visceral and neurological complications in varicella infections of adults [J]. Schweiz Med Wochenschr, 1996, 126(11): 440-446.[12]Senellart F, Bozio A, Sassolas F, et al. Varicella myocarditis and junctional ectopic tachycardia [J]. Pediatrie Bucur, 1991, 46(3): 267-270.[13]Straus S, Ostrove J, Inchauspe G, et al. Varicella- zoster virus infections[J]. Ann Intern Med,1988, 108: 221-237.[14]Tsintsof A, Delprado WJ, Keogh AM. Varicella zoster myocarditis progressing to cardiomyopathy and cardiac

  13. Characteristics of student preparedness for clinical learning: clinical educator perspectives using the Delphi approach

    Directory of Open Access Journals (Sweden)

    Chipchase Lucinda S

    2012-11-01

    Full Text Available Abstract Background During clinical placements, clinical educators facilitate student learning. Previous research has defined the skills, attitudes and practices that pertain to an ideal clinical educator. However, less attention has been paid to the role of student readiness in terms of foundational knowledge and attitudes at the commencement of practice education. Therefore, the aim of this study was to ascertain clinical educators’ views on the characteristics that they perceive demonstrate that a student is well prepared for clinical learning. Methods A two round on-line Delphi study was conducted. The first questionnaire was emailed to a total of 636 expert clinical educators from the disciplines of occupational therapy, physiotherapy and speech pathology. Expert clinical educators were asked to describe the key characteristics that indicate a student is prepared for a clinical placement and ready to learn. Open-ended responses received from the first round were subject to a thematic analysis and resulted in six themes with 62 characteristics. In the second round, participants were asked to rate each characteristic on a 7 point Likert Scale. Results A total of 258 (40.56% responded to the first round of the Delphi survey while 161 clinical educators completed the second (62.40% retention rate. Consensus was reached on 57 characteristics (six themes using a cut off of greater than 70% positive respondents and an interquartile deviation IQD of equal or less than 1. Conclusions This study identified 57 characteristics (six themes perceived by clinical educators as indicators of a student who is prepared and ready for clinical learning. A list of characteristics relating to behaviours has been compiled and could be provided to students to aid their preparation for clinical learning and to universities to incorporate within curricula. In addition, the list provides a platform for discussions by professional bodies about the role of placement

  14. Pathophysiology of Post Amputation Pain

    Science.gov (United States)

    2014-12-01

    nociception and pain : analysis through imaging. Proc Natl Acad Sci U S A 1999;96:7668-74. 44. Casey KL, Minoshima S, Morrow TJ, Koeppe RA. Comparison of...trials. Eur J Pain 2006;10:77-88. 95. Dotson RM. Clinical neurophysiology laboratory tests to assess the nociceptive system in humans. J Clin...Award Number: W81XWH-11-1-0815 TITLE: Pathophysiology of Post Amputation Pain PRINCIPAL INVESTIGATOR: Dr. R. Norman Harden CONTRACTING

  15. Demographic, clinical and radiological characteristics of seronegative spondyloarthritis Egyptian patients: A rheumatology clinic experience in Mansoura

    Directory of Open Access Journals (Sweden)

    Adel Abdelsalam

    2017-04-01

    Conclusion: The demographic, clinical and radiological characteristics of Egyptian SpA patients are comparable to those from other countries except for the lower prevalence of extra-articular manifestations.

  16. Sex differences in clinical characteristics and outcomes after myocardial infarction

    DEFF Research Database (Denmark)

    Lam, Carolyn S P; McEntegart, Margaret; Claggett, Brian;

    2015-01-01

    BACKGROUND: We examined the association of sex with clinical characteristics and outcomes in patients following myocardial infarction (MI) in the Valsartan in Acute Myocardial Infarction Trial (VALIANT). METHODS AND RESULTS: A total of 4570 women and 10 133 men with heart failure (HF), left ventr...

  17. Characteristics and Clinical Practices of Rural Marriage and Family Therapists

    Science.gov (United States)

    Morris, James

    2007-01-01

    This report presents a subset of data collected from the American Association for Marriage and Family Therapy (AAMFT) Practice Research Network project conducted in 2002. A sample of 47 clinical members of AAMFT who indicated they practiced in a rural community provided descriptive information on demographic characteristics, training, clinical…

  18. [Hereditary optic neuropathies: clinical and molecular genetic characteristics].

    Science.gov (United States)

    Khanakova, N A; Sheremet, N L; Loginova, A N; Chukhrova, A L; Poliakov, A V

    2013-01-01

    The article presents a review of literature on hereditary optic neuropathies: Leber mitochondrial hereditary optic neuropathy, autosomal dominant and autosomal recessive optic neuropathies, X-linked optic atrophy. Clinical and molecular genetic characteristics are covered. Isolated optic neuropathies, as well as hereditary optic disorders, being a part of a complex syndromic disease are described.

  19. [Sickle cell pathophysiology].

    Science.gov (United States)

    Renaudier, P

    2014-11-01

    Sickle cell disease is associated with the inversion of one base pair (A = T → A = T). The sixth codon of the beta globin chain [GAA] becomes [GTA]. Accordingly, the sixth amino acid (glutamic acid, negatively charged) is replaced by valine, hydrophobic. A hydrophobic site is present on the outside of the HbS β chain. This incurs a hydrophobic bond with the phenylalanine in position 85 and leucine in position 88, in which outsource deoxy haemoglobin. Therefore, it creates a HbS polymer that deforms the red blood cell and causes vaso-occlusive crisis in the capillary venous pole. In this conventional design, the roles are added to the nitrogen monoxide and vascular tone, the increase in adhesion of red blood cells to the endothelium damage caused by red blood cells HbS: dehydration, senescence, formation of microvesicles. If these advances in our understanding of the pathophysiology have not yet had a clinical application, they will happen one day. It is therefore particularly important to pursue in France the network structure of sickle cell disease with a view to set up multicenter trials when the day comes.

  20. Clinical characteristics and current therapies for inherited retinal degenerations.

    Science.gov (United States)

    Sahel, José-Alain; Marazova, Katia; Audo, Isabelle

    2014-10-16

    Inherited retinal degenerations (IRDs) encompass a large group of clinically and genetically heterogeneous diseases that affect approximately 1 in 3000 people (>2 million people worldwide) (Bessant DA, Ali RR, Bhattacharya SS. 2001. Molecular genetics and prospects for therapy of the inherited retinal dystrophies. Curr Opin Genet Dev 11: 307-316.). IRDs may be inherited as Mendelian traits or through mitochondrial DNA, and may affect the entire retina (e.g., rod-cone dystrophy, also known as retinitis pigmentosa, cone dystrophy, cone-rod dystrophy, choroideremia, Usher syndrome, and Bardet-Bidel syndrome) or be restricted to the macula (e.g., Stargardt disease, Best disease, and Sorsby fundus dystrophy), ultimately leading to blindness. IRDs are a major cause of severe vision loss, with profound impact on patients and society. Although IRDs remain untreatable today, significant progress toward therapeutic strategies for IRDs has marked the past two decades. This progress has been based on better understanding of the pathophysiological pathways of these diseases and on technological advances.

  1. Clinical and demographic characteristics of 165 patients with lichen planus

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    Bilge Bülbül Şen

    2014-03-01

    Full Text Available Objective: Lichen planus (LP, is a papulosquamous inflammatory disease, which involves the skin, mucous membranes, nails and scalp. The incidence varies according to geographical regions. In this study, it was aimed to detect the clinical and demographic characteristics of the patients with LP who have been under follow-up at our clinic. Methods: One hundred sixty five patients, who were diagnosed as LP in our clinic between 2010 and 2013, were enrolled to the study. The age, gender, disease duration, time of onset of disease, characteristics of involvement, associated systemic diseases, laboratory findings and treatment were recorded retrospectively. Results: In our study, 0.6% of all patients admitted to our clinic were diagnosed as LP. A total of 165 patients included in the study, 92 women (56% and 73 men (44%, respectively. Patients’ ages ranged 8-78 (mean 44.7±16.7. Disease duration ranged from 1 month to 20 years (mean 15.6±29.7. One hundred thirty four patients (81.2% had skin involvement, 51 (31% had oral mucosal involvement, and 15 (9% had genital involvement. Five (4.5% of 111 patients with viral hepatitis tests were positive for hepatitis C virus. Hepatitis B virus positivity was seen in 4 (4% patients. Conclusion: There is a need for further studies with more patients to better understand the epidemiological, clinical and pathological characteristics of LP. We believe that our study will contribute to the determination of our country’s data.

  2. [Pathophysiology of urticaria].

    Science.gov (United States)

    Nosbaum, A; Augey, F; Nicolas, J-F; Bérard, F

    2014-11-01

    Urticaria is a dermal edema resulting from vascular dilatation and leakage of fluid into the skin in response to molecules released from mast cells. The major mediator responsible for urticaria is histamine. However, the clinical spectrum and pattern of lesions indicate that other molecules, including prostaglandins, leukotrienes, cytokines, and chemokines, produced at different times after mast cell activation contribute to the polymorphism of this symptom and the variable evolution of this disease. It is a common practice to distinguish immunological and nonimmunological urticaria. Immunological urticaria is a hypersensitivity reaction mediated by antibodies and/or T-cells that results in mast cell activation. Although immunoglobulin (Ig) E-mediated type I hypersensitivity (HS) was long postulated to be the major immunological pathway associated with mast cell activation, interaction between IgEbound mast cells and allergens is unlikely to be the mechanism by which urticaria develops in most patients. It is now well established that urticaria may result from the binding of IgG auto-antibodies to IgE and/or to the receptor for IgE molecules on mast cells, thus corresponding to a type II HS reaction. These auto-immune urticarias represent up to 50 % of patients with chronic urticaria. Mast cell activation can also result from type III HS through the binding of circulating immune complexes to mast cell-expressing Fc receptors for IgG and IgM. Finally, under certain circumstances, T-cells can induce activation of mast cells, as well as histamine release (type IV HS). Nonimmunological urticarias result from mast cell activation through membrane receptors involved in innate immunity (e.g., complement, Toll-like, cytokine/chemokine, opioid) or by direct toxicity of xenobiotics (haptens, drugs). In conclusion, urticaria may result from different pathophysiological mechanisms that explain the great heterogeneity of clinical symptoms and the variable responses to treatment.

  3. Genetic characteristics of Japanese clinical Listeria monocytogenes isolates.

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    Satoko Miya

    Full Text Available Listeria monocytogenes causes foodborne illnesses through consumption of ready-to-eat foods. Although 135-201annual listeriosis cases have been estimated in Japan, the details regarding the clinical isolates such as infection source, virulence level, and other genetic characteristics, are not known. In order to uncover the trends of listeriosis in Japan and use the knowledge for prevention measures to be taken, the genetic characteristics of the past human clinical isolates needs to be elucidated. For this purpose, multilocus tandem-repeat sequence analysis (MLTSA and multi-virulence-locus sequence typing (MVLST were used in this study. The clinical isolates showed a variety of genetically distant genotypes, indicating they were from sporadic cases. However, the MVLST profiles of 7 clinical isolates were identical to those of epidemic clone (EC I isolates, which have caused several serious outbreaks in other countries, suggesting the possibility that they have strong virulence potential and originated from a single outbreak. Moreover, 6 Japanese food isolates shared their genotypes with ECI isolates, indicating that there may be risks for listeriosis outbreak in Japan. This is the first investigational study on genetic characteristics of Japanese listeriosis isolates. The listeriosis cases happened in the past are presumably sporadic, but it is still possible that some isolates with strong virulence potential have caused listeriosis outbreaks, and future listeriosis risks also exist.

  4. [A new possible strategy for prevention and preventive treatment of age-related macular degeneration resting on recent clinical and pathophysiological observations].

    Science.gov (United States)

    Fischer, Tamás

    2009-03-15

    The beneficial effect achieved by the treatment of endothelial dysfunction in chronic cardiovascular diseases is already an evidence belonging to the basic treatment of the disease. Given the fact that the vascular system is uniform and consubstantial both physiologically, pathophysiologically and in terms of therapy, and that it plays a key role in age-related macular degeneration (AMD)--a disease leading to tragic loss of vision with its etiology and therapy being unknown--endothelial dysfunction should be treated. The pleiotropic effects of ACE-inhibitors, AR-blockers and statins and third generation beta blockers help to restitute the balance between vasodilators and vasoconstrictors in endothelial dysfunction caused by oxidative stress, the balance of growth factors and their inhibitors, pro- and anti-inflammatory substances and prothrombotic and fibrinolytic factors, inhibit the formation of oxidative stress and its harmful effects; while aspirin with its pleiotropic effects acting as an antiaggregation substance on platelets helps to set the endothelial layer back to its normal balance regarding its vasodilating, antithrombotic, antiadhesive and anti-inflammatory functions; trimetazidine as an adjuvant agent helps to normalize, to restore the disturbed metabolism of the retinal tissue functioning insufficiently, in the end. The angiotensin II receptor blocker telmisartan with its peroxisome proliferator-activated receptor-gamma (PPAR-gamma) agonist effect inhibits the development of choroidal neovascularisation (CNV) and improves it clinically favourably. The third generation beta adrenergic receptor blocker carvedilol and nebivolol as well as the peroxisome proliferator-activated receptor-gamma agonist pioglitazone elicit their antioxidant vascular protective effects mitochondrially. For the above reasons it is suggested that, as a part of long term primary and/or secondary prevention, the following groups of patients with AMD receive--taking into

  5. Clinical characteristics associated with illness perception in psoriasis.

    Science.gov (United States)

    Wahl, Astrid K; Robinson, Hilde S; Langeland, Eva; Larsen, Marie H; Krogstad, Anne-Lene; Moum, Torbjørn

    2014-05-01

    Knowledge of illness perception may aid the identification of groups of patients with a higher risk of coping poorly with the demands of their illness. This study aims to investigate associations between illness perception, clinical characteristics, patient knowledge, quality of life and subjective health in persons with psoriasis. The present study was based on cross-sectional data from patients awaiting climate therapy in Gran Canaria. We included 254 eligible patients (74%) who completed a questionnaire including the revised Illness Perception Questionnaire, the Psoriasis Knowledge Questionnaire, and the Dermatological Life Quality Index. Disease severity was measured using the Psoriasis Area and Severity Index. Several statistically significant associations between clinical characteristics, knowledge and various illness perception dimensions were found. Illness perception was also significantly related to disease-specific quality of life and subjective health. These findings contradict previous findings, which suggested that objective disease factors are not relevant to illness perception in psoriasis.

  6. GENDER DIFFERENCES IN PARKINSON'S DISEASE: CLINICAL CHARACTERISTICS AND COGNITION

    OpenAIRE

    Miller, Ivy N.; Cronin-Golomb, Alice

    2010-01-01

    More men than women are diagnosed with Parkinson's disease (PD), and a number of gender differences have been documented in this disorder. Examples of clinical characteristics that appear in men more often than women include rigidity and rapid eye movement behavior disorder, whereas more women than men exhibit dyskinesias and depression. Differences between men and women in cognition have not been extensively examined, though there are reports of deficits in men in aspects of cognition that c...

  7. Clinical and laboratory characteristics of Achromobacter xylosoxidans infection.

    OpenAIRE

    1980-01-01

    Achromobacter xylosoxidans was isolated from six patients. The organism causes opportunistic infections in patients who are compromised. A. xylosoxidans is a catalase- and oxidase-positive, motile, gram-negative rod that oxidizes xylose and glucose. The organism exists in a water environment and may be confused with Pseudomonas species. Unlike pseudomonas, achromobacter has peritrichous flagella. The clinical and laboratory characteristics of A. xylosoxidans are presented.

  8. Pathophysiology of cancer cachexia

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    Riad N. Younes

    2000-10-01

    Full Text Available Cancer cachexia is a frequent complication observed in patients with malignant tumors. Although several decades have passed since the first focus on the metabolic dysfunction's associated with cancer, few effective therapeutic interventions have been successfully introduced into the medical armamentarium. The present study thoroughly reviews the basic pathophysiology of cancer cachexia and the treatment options already investigated in that field. Experimental and clinical studies were evaluated individually in order to clarify the intricate alterations observed in tumor-bearing patients. The difficulties in introducing sound and effective nutritional support or metabolic manipulation to reverse cancer cachexia are outlined in this review.A caquexia é uma complicação freqüentemente observada em pacientes portadores de tumores malignos. Apesar de várias décadas transcorrerem desde a descrição inicial das disfunções metabólicas associadas ao câncer, poucas medidas terapêuticas foram induzidas com sucesso na prática médica. O presente estudo apresenta uma revisão detalhada da fisiopatologia básica da caquexia em câncer, e as opções terapêuticas desenvolvidas nesta área. Estudos experimentais, assim como clínicos, são avaliados individualmente para esclarecer as alterações complexas observadas em pacientes portadores de tumores. As dificuldades encontradas para introduzir manipulações metabólicas e terapias de suporte nutricional eficientes são discutidas nesta revisão.

  9. Pathophysiology of hemolytic transfusion reactions.

    Science.gov (United States)

    Davenport, Robertson D

    2005-07-01

    Hemolytic transfusion reactions (HTR) are systemic reactions provoked by immunologic red blood cell (RBC) incompatibility. Clinical and experimental observations of such reactions indicate that they proceed through phases of humoral immune reaction, activation of phagocytes, productions of cytokine mediators, and wide-ranging cellular responses. HTR have many features in common with the systemic inflammatory response syndrome (SIRS). Knowledge of the pathophysiologic mechanisms in HTR suggest that newer biological agents that target complement intermediates or proinflammatory cytokines may be effective agents in the treatment of severe HTRs.

  10. Inner Ear Disease and Benign Paroxysmal Positional Vertigo: A Critical Review of Incidence, Clinical Characteristics, and Management

    Directory of Open Access Journals (Sweden)

    M. Riga

    2011-01-01

    Full Text Available Background. This study is a review of the incidence, clinical characteristics, and management of secondary BPPV. The different subtypes of secondary BPPV are compared to each other, as well as idiopathic BPPV. Furthermore, the study highlights the coexistence of BPPV with other inner ear pathologies. Methods. A comprehensive search for articles including in the abstract information on incidence, clinical characteristics, and management of secondary BPPV was conducted within the PubMed library. Results. Different referral patterns, different diagnostic criteria used for inner ear diseases, and different patient populations have led to greatly variable incidence results. The differences regarding clinical characteristics and treatment outcomes may support the hypothesis that idiopathic BPPV and the various subtypes of secondary BPPV do not share the exact same pathophysiological mechanisms. Conclusions. Secondary BPPV is often under-diagnosed, because dizziness may be atypical and attributed to the primary inner ear pathology. Reversely, a limited number of BPPV patients may not be subjected to a full examination and characterized as idiopathic, while other inner ear diseases are underdiagnosed. A higher suspicion index for the coexistence of BPPV with other inner ear pathologies, may lead to a more integrated diagnosis and consequently to a more efficient treatment of these patients.

  11. Characteristics of liver cancer stem cells and clinical correlations.

    Science.gov (United States)

    Cheng, Zhuo; Li, Xiaofeng; Ding, Jin

    2016-09-01

    Liver cancer is an aggressive malignant disease with a poor prognosis. Patients with liver cancer are usually diagnosed at an advanced stage and thus miss the opportunity for surgical resection. Chemotherapy and radiofrequency ablation, which target tumor bulk, have exhibited limited therapeutic efficacy to date. Liver cancer stem cells (CSCs) are a small subset of undifferentiated cells existed in liver cancer, which are considered to be responsible for liver cancer initiation, metastasis, relapse and chemoresistance. Elucidating liver CSC characteristics and disclosing their regulatory mechanism might not only deepen our understanding of the pathogenesis of liver cancer but also facilitate the development of diagnostic, prognostic and therapeutic approaches to improve the clinical management of liver cancer. In this review, we will summarize the recent advances in liver CSC research in terms of the origin, identification, regulation and clinical correlation.

  12. Pathophysiology of migraine

    Directory of Open Access Journals (Sweden)

    Peter J Goadsby

    2012-01-01

    Full Text Available Migraine is a common disabling brain disorder whose pathophysiology is now being better understood. The study of anatomy and physiology of pain producing structures in the cranium and the central nervous system modulation of the input have led to the conclusion that migraine involves alterations in the sub-cortical aminergic sensory modulatory systems that influence the brain widely.

  13. [Pathophysiology of secondary hyperparathyroidism.

    Science.gov (United States)

    Kawarazaki, Hiroo

    2017-01-01

    Secondary hyperparathyroidism(SHPT)is the result of a compensatory response of the calcium phosphate homeostatic mechanism. Vitamin D deficiency and chronic kidney disease, both representative pathophysiological causes of SHPT, have been related not only to skeletal disorders but also cardiovascular diseases, ADL and QOL. This relates the importance of SHPT as a pathological cause or marker of such states.

  14. Clinical and Molecular Characteristics of Post-Colonoscopy Colorectal Cancer

    DEFF Research Database (Denmark)

    Stoffel, Elena M; Erichsen, Rune; Frøslev, Trine;

    2016-01-01

    BACKGROUND AND AIMS: Colonoscopy provides incomplete protection from colorectal cancer (CRC), but determinants of post-colonoscopy CRC are not well understood. We compared clinical features and molecular characteristics of CRCs diagnosed at different time intervals after a previous colonoscopy....... METHODS: We performed a population-based, cross-sectional study of incident CRC cases in Denmark (2007-2011), categorized as post-colonoscopy or detected during diagnostic colonoscopy (in patients with no prior colonoscopy). We compared prevalence of proximal location and DNA mismatch repair deficiency (d...

  15. Clinical characteristics of patients with persistent postural-perceptual dizziness

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    Roseli Saraiva Moreira Bittar

    2015-06-01

    Full Text Available INTRODUCTION: Persistent postural-perceptual dizziness is the dizziness that lasts for over three months with no clinical explanation for its persistence. The patient's motor response pattern presents changes and most patients manifest significant anxiety. OBJECTIVE: To evaluate the clinical characteristics of patients with persistent postural and perceptual dizziness. METHODS: statistical analysis of clinical aspects of patients with persistent postural-perceptual dizziness. RESULTS: 81 patients, average age: 50.06 ± 12.16 years; female/male ratio: 5.7/1; main reasons for dizziness: visual stimuli (74%, body movements (52%, and sleep deprivation (38%. The most prevalent comorbidities were hypercholesterolemia (31%, migraine headaches (26%, carbohydrate metabolism disorders (22% and cervical syndrome (21%. DHI, State-Trait Anxiety Inventory - Trait, Beck Depression Inventory, and Hospital Anxiety and Depression Scale questionnaires were statistically different (p < 0.05 when compared to controls. 68% demonstrated clinical improvement after treatment with serotonin reuptake inhibitors. CONCLUSION: Persistent postural-perceptual dizziness affects more women than men, with a high associated prevalence of metabolic disorders and migraine. Questionnaires help to identify the predisposition to persistent postural-perceptual dizziness. The prognosis is good with adequate treatment.

  16. Clinical characteristics of early- and late-onset gout

    Science.gov (United States)

    Zhang, Bingqing; Fang, Weigang; Zeng, Xuejun; Zhang, Yun; Ma, Ya; Sheng, Feng; Zhang, Xinlei

    2016-01-01

    Abstract A retrospective cross-sectional study using data from an outpatient clinic in China was conducted to investigate the clinical features of early-onset gout patients. All patients diagnosed with gout were asked about clinical characteristics of their gout and comorbid diseases. Patients presenting with acute flares were asked about common triggers before the flare. “Early-onset” gout was defined as onset of gout before 40 years and “late-onset” as onset ≥40 years. Major joint involvement, flare frequency before presentation, the cumulative number of involved joints, proportions of tophi complications at presentation, flare triggers, as well as any metabolic, cardiovascular, cerebrovascular, and renal comorbidities, were compared between the 2 groups. A total of 778 gout patients were enrolled in this study, including 449 (57.7%) in the early-onset group and 329 (42.3%) in the late-onset group. Compared with the late-onset gout patients, the early-onset gout patients had a higher proportion of ankle/mid-foot involvement (62.8% vs 48.2%, P gout patients had fewer metabolic, cardiovascular, cerebrovascular, or renal complications. Early- and late-onset gout patients had different clinical features. Early-onset seems to be influenced more by lifestyle, while late-onset patients have more complications because of comorbidities. PMID:27893683

  17. Sociodemographic and clinical characteristics of patients with recurrent aphthous stomatitis

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    Anıl Gülsel Bahalı

    2014-12-01

    Full Text Available Background and Design: The purpose of this study was to obtain data that may provide an insight into the etiopathogenesis of recurrent aphtous stomatitis (RAS by the way of analysing the sociodemographic and clinical characteristics of patients who had been diagnosed with RAS. Materials and Metods: The patients, who were diagnosed with RAS in the dermatology outpatient clinic, between May 2007 and May 2010, were evaluated retrospectively. The data including sociodemografic and clinical characteristics, and treatment options were recorded. Results: A hundred patients (68 women, 32 men were included in this study. The average age was 40±13.6 years. RAS was more common in patients with middle-income and low education. The most common type of RAS was minor aphtous ulcers (88%. The lesions were most frequently seen on the lateral side of the tongue (34% and cheek (34%. Sixty percent of patients had a positive family history. Some factors such as biting (12%, tooth brushing (18%, dental disease presence (82%, food (39%, menstruation (10.3%, stress (76%, iron deficiency (16.7%, vitamin B12 deficiency (22.4%, low serum ferritin levels (18%, and seasonal variability (32% showed positive correlation with RAS. A negative correlation was found between RAS and smoking. Forty-nine percent of patients had used alternative therapies in addition to drug therapy. The most frequently used alternative method was consumption of sumac (26.5%. Conlucions: In contrast to the literature, our study found that RAS is started in the third decade of life and, approximately 50% of patients prefered alternative treatment methods, particularly sumac. Nowadays, discussions about the etiopathogenesis of RAS continue. In this study, we found that different sociodemographic and clinical factors may be associated with the etiopathogenesis of the disease. Our study will be followed by further studies using prospective design to identify the the etiopathogenesis of RAS.

  18. A combined clinical and biomarker approach to predict diuretic response in acute heart failure

    NARCIS (Netherlands)

    Ter Maaten, Jozine M; Valente, Mattia A E; Metra, Marco; Bruno, Noemi; O'Connor, Christopher M; Ponikowski, Piotr; Teerlink, John R; Cotter, Gad; Davison, Beth; Cleland, John G; Givertz, Michael M; Bloomfield, Daniel M; Dittrich, Howard C; van Veldhuisen, Dirk J; Hillege, Hans L; Damman, Kevin; Voors, Adriaan A

    2015-01-01

    BACKGROUND: Poor diuretic response in acute heart failure is related to poor clinical outcome. The underlying mechanisms and pathophysiology behind diuretic resistance are incompletely understood. We evaluated a combined approach using clinical characteristics and biomarkers to predict diuretic resp

  19. Kikuchi-Fujimoto disease: Clinical and laboratory characteristics and outcome

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    P S Rakesh

    2014-01-01

    Full Text Available Introduction: Kikuchi-Fujimoto disease is an uncommon disorder with worldwide distribution, characterized by fever and benign enlargement of the lymph nodes, primarily affecting young adults. Awareness about this disorder may help prevent misdiagnosis and inappropriate investigations and treatment. The objective of the study was to evaluate the clinical and laboratory characteristics of histopathologically confirmed cases of Kikuchi′s disease from a tertiary care center in southern India. Materials and Methods: Retrospective analysis of all adult patients with histopathologically confirmed Kikuchi′s disease from January 2007 to December 2011 in a 2700-bed teaching hospital in South India was done. The clinical and laboratory characteristics and outcome were analyzed. Results: There were 22 histopathologically confirmed cases of Kikuchi′s disease over the 5-year period of this study. The mean age of the subjects′ was 29.7 years (SD 8.11 and majority were women (Male: female- 1:3.4. Apart from enlarged cervical lymph nodes, prolonged fever was the most common presenting complaint (77.3%. The major laboratory features included anemia (54.5%, increased erythrocyte sedimentation rate (31.8%, elevated alanine aminotransferase (27.2% and elevated lactate dehydrogenase (LDH (31.8%. Conclusion: Even though rare, Kikuchi′s disease should be considered in the differential diagnosis of young individuals, especially women, presenting with lymphadenopathy and prolonged fever. Establishing the diagnosis histopathologically is essential to avoid inappropriate investigations and therapy.

  20. Clinical Characteristics of Cases with Spinal Muscular Atrophy

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    Mehmet Canpolat

    2016-04-01

    Full Text Available Introduction: The aim of this study is was to evaluate the clinical features of cases with diagnosis of spinal muscular atrophy (SMA. Materials and Methods: Thirty-eight pediatric patients were evaluated retrospectively. All patients were followed in the Pediatric Neurology Department of Erciyes University Faculty of Medicine. The diagnosis of patients had been confirmed by genetic analysis of homozygous deletions of survival motor neuron 1 gene. Detailed history, newborn symptoms, nutritional characteristics, initial complaints, physical examination, concomitant pathologies, genetic characteristics, and treatment modalities were investigated in all patients. Results: The study population consisted of 19 boys (50% and 19 girls (50%. The mean age of patients was 26.9±25.7 months (range: 3-96 months. The mean follow-up period was 12.2±13.3 months (range: 2-48 months. According to SMA classification, 22 patients (57.8% were type 1, 8 patients (21.1% were type 2, and 8 patients were (21.1% type 3. Neonatal respiratory distress, age at early diagnosis, nutritional problems, and recurrent lung diseases were detected as poor prognostic factors. Conclusions: SMA is a neuromuscular disease that requires multidisciplinary approach to medical care. There is a wide range of clinical severity. Identification of poor prognostic factors will help in terms of guiding close monitoring and timely treatments of children with SMA.

  1. Clinical characteristics affecting motor recovery and ambulation in stroke patients

    Science.gov (United States)

    Yetisgin, Alparslan

    2017-01-01

    [Purpose] To describe the clinical characteristics affecting motor recovery and ambulation in stroke patients. [Subjects and Methods] Demographic and clinical characteristics of 53 stroke patients (31 M, 22 F), such as age, gender, etiology, hemiplegic side, Brunnstrom stage, functional ambulation scale scores, history of rehabilitation, and presence of shoulder pain and complex regional pain syndrome were evaluated. [Results] The etiology was ischemic in 79.2% of patients and hemorrhagic in 20.8%. Brunnstrom hand and upper extremity values in females were lower than in males. Complex regional pain syndrome was observed at a level of 18.9% in all patients (more common in females). Brunnstrom hand stage was lower in complex regional pain syndrome patients than in those without the syndrome. Shoulder pain was present in 44.4% of patients. Brunnstrom lower extremity values and functional ambulation scale scores were higher in rehabilitated than in non-rehabilitated cases. [Conclusion] Brunnstrom stages of hand and upper extremity were lower and complex regional pain syndrome was more common in female stroke patients. Shoulder pain and lower Brunnstrom hand stages were related to the presence of complex regional pain syndrome. PMID:28265142

  2. Clinical characteristics of Takotsubo cardiomyopathy in North America

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    Saeed Ahmed

    2013-01-01

    Full Text Available Background: Takotsubo cardiomyopathy (TC or transient left ventricular apical ballooning syndrome is an acute cardiac syndrome characterized by transient wall motion abnormalities extending beyond a single epicardial vessel in the absence of significant obstructive coronary artery disease. Aim: This study was to describe the clinical characteristics of TC in North America. Materials and Methods: We identified 10 patients who met the Mayo Clinic criteria for TC using our Electronic Medical Records. We also conducted a systematic review of case series of TC that were done in North America by searching the MEDLINE database. We identified 11 case series that met our eligibility criteria. Results: Our systematic review included 620 patients. Chest pain and ST segment elevation were the cardinal features of this syndrome, but the prevalence was lower than in the European and Asian cohort (50% and 39% as compared with 80% and 70%, respectively. Classic precipitating emotional or physical stress was described in > 80% of patients. Cardiac biomarkers were found to be elevated in >90% of our patients. Conclusions : TC is a worldwide problem and clinical presentation appears to be similar in North American, European, and Asian countries. However, fewer patients in our cohort presented with typical chest pain and electrocardiography (ECG changes, which might suggest ethnic variations in the syndrome or perhaps a more aggressive diagnostic approach in North American countries.

  3. Obesity: Pathophysiology and Intervention

    Directory of Open Access Journals (Sweden)

    Yi Zhang

    2014-11-01

    Full Text Available Obesity presents a major health hazard of the 21st century. It promotes co-morbid diseases such as heart disease, type 2 diabetes, obstructive sleep apnea, certain types of cancer, and osteoarthritis. Excessive energy intake, physical inactivity, and genetic susceptibility are main causal factors for obesity, while gene mutations, endocrine disorders, medication, or psychiatric illnesses may be underlying causes in some cases. The development and maintenance of obesity may involve central pathophysiological mechanisms such as impaired brain circuit regulation and neuroendocrine hormone dysfunction. Dieting and physical exercise offer the mainstays of obesity treatment, and anti-obesity drugs may be taken in conjunction to reduce appetite or fat absorption. Bariatric surgeries may be performed in overtly obese patients to lessen stomach volume and nutrient absorption, and induce faster satiety. This review provides a summary of literature on the pathophysiological studies of obesity and discusses relevant therapeutic strategies for managing obesity.

  4. Pathophysiology of Equine Neonatal Septicemia

    Directory of Open Access Journals (Sweden)

    Juan Carlos Ospina Chirivi

    2014-07-01

    Full Text Available Neonatal septicemia is a major cause of mortality and morbidity in horses in their first seven days of life and within their pathophysiology. It is important to consider the extrinsic and intrinsic predisposing factors which make foals susceptible to agents of primarily bacterial etiology. However, other types of infectious etiology (viruses and fungi should be considered too, as well as noninfectious etiologies. The paper mentions a wide variety of mechanisms that produce different injuries that must be addressed with measures of critical neonatal care, so it is imperative for the veterinarian to know the pathogenic mechanisms of the disease, its clinical presentation and anatomo-pathological lesions. Thus, systemic inflammatory response syndrome (SIRS, multiple organ dysfunction syndrome (MODS, and peripheral circulatory collapse or shock are some of the elements defined as the pillars of the pathophysiology of neonatal septicemia, extensively studied in equine medicine. This paper presents a short review of the triggering mechanisms of neonatal septicemia highlighting the importance of epidemiological investigations in Colombia. It shows the need for retrospective and prospective studies and for divulgation of some of the preventive measures of the disease in horses.

  5. [Functional pathophysiology of consciousness].

    Science.gov (United States)

    Jellinger, Kurt A

    2009-01-01

    from important somatic and sensory pathways and acts as a control system of neuronal activities of the cerebral cortex. The principal function of the ARAS is to focus our alertness on specific stimuli or internal processes, which run via complex neuronal cell groups and numerous neurotransmitters that influence various aspects of consciousness and wakefulness. Stimulation of the ARAS produces an arousal reaction as the electric correlate of consciousness; its destruction causes coma and related states. The highest level are cortical (prefrontal and association) networks for recognition, motor activity, longterm memory and attention, the left hemisphere being considered as the dominant one. Different levels of consciousness are distinguished: 1. hyperalertness, 2. alertness (normal state of wakefulness), 3. somnolence or lethargy, 4. obtundation with tendency to fall asleep, 5. stupor, 6. coma and its subtypes, like akinetic mutism, apallic syndrome or persistent vegative state, locked-in syndrome, delirium, and catatonia. They are caused by damages in various functional levels of the brain, by psychogenic factors or experimentally, and are accompanied by characteristic neurological and psychiatric disorders. The relevant morphological lesions can be detected by electrophysiological and imaging studies. The bases of functional anatomy and pathophysiology of consciousness, its cognitive aspects and its major disorders, their causes and functional substrates with reference to sleep and both spontaneous and iatrogenic disorders of consciousness are critically summarized.

  6. Clinical manifestation, imageological and pathological characteristics of Wernicke encephalopathy

    Institute of Scientific and Technical Information of China (English)

    Shunchang Han; Chuanqiang Pu; Qiuping Gui; Xusheng Huang; Senyang Lang; Weiping Wu; Peifu Wang

    2006-01-01

    BACKGROUND: The clinical manifestations of Wernicke encephalopathy(WE) are atypical and short of effective auxiliary examination means. The effects of magnetic resonance imaging (MRI) in the diagnosis of WE have been reported suecessively. But its imageological detection needs to be further investigated.OBJECTIVE: To analyze the eharacteristics of clinical manifestations, skull MRI examination and pathological results in patients with WE.DESTGN: Retrospective analysis.SETTTNG: The General Hospital of Chinese PLA.PARTTCTPANTS: Ten patients of WE admitted to the Department of Neurology, General Hospital of Chinese PLA were recruited. Among them, five patients were diagnosed pathologically after death. Their pathological changes accorded with the pathological characteristics of WE. The other 5 patients were diagnosed clinically before death. Their pathological changes accorded with clinical and imageological manifestations and had definite reaction to the treatment of thiamine. Ten patients, 7 males and 3 females, were aged (47±13) years ranging from 33 to 73 years. Their disease courses averaged 6 weeks ranging from 3 to 10 weeks. They all were non-alcoholics. Four patients developed WE after acute pancreatitis, two patients after the recurrence of gastric cancer, two patients after cholecystectomy, one patient after hepatitis medicamentosa, one patient after Alzheimer disease. Informed consents were obtained from all the patients and their relatives.METHODS: After admission, clinical manifestations of patients were observed and recorded. Five patients underwent skull MRI examination and their detected results were recorded. Five dead patients underwent autopsy and brain pathological examinations. Neuropathological examination involved cerebrum, cerebellum and brain stem.MATN OUTCOME MEASURES: Clinical manifestations, MRI examination results, pathological analysis results and prognosis of all the patients.RESULTS: Ten patients with WE were involved in the final

  7. Community-acquired Acinetobacter baumannii: clinical characteristics, epidemiology and pathogenesis.

    Science.gov (United States)

    Dexter, Carina; Murray, Gerald L; Paulsen, Ian T; Peleg, Anton Y

    2015-05-01

    Community-acquired Acinetobacter baumannii (CA-Ab) is a rare but serious cause of community-acquired pneumonia in tropical regions of the world. CA-Ab infections predominantly affect individuals with risk factors, which include excess alcohol consumption, diabetes mellitus, smoking and chronic lung disease. CA-Ab pneumonia presents as a surprisingly fulminant course and is characterized by a rapid onset of fever, severe respiratory symptoms and multi-organ dysfunction, with a mortality rate reported as high as 64%. It is unclear whether the distinct clinical syndrome caused by CA-Ab is because of host predisposing factors or unique bacterial characteristics, or a combination of both. Deepening our understanding of the drivers of overwhelming CA-Ab infection will provide important insights into preventative and therapeutic strategies.

  8. Isolation Frequency Characteristics of Candida Species from Clinical Specimens.

    Science.gov (United States)

    Kim, Ga-Yeon; Jeon, Jae-Sik; Kim, Jae Kyung

    2016-06-01

    Candida spp. is an invasive infectious fungus, a major risk factor that can increase morbidity and mortality in hospitalized patients. In this study, 2,508 Candida spp. were isolated from various clinical specimens collected from university hospitals from July 2011 to October 2014. They were identified in order to determine isolation frequencies and characteristics by specimen, gender, age group, year, season, and month. The strain-specific isolation rate of Candida spp. is in the order of Candida albicans (1,218 strains, 48.56%), Candida glabrata (416 strains, 16.59%), Candida utilis (305 strains, 12.16%), Candida tropicalis (304 strains, 12.12%), and Candida parapsilosis (116 strains, 4.63%) and these five species accounted for more than 94% of the total strains. Of the specimens, Candida spp. were most frequently isolated from urine-catheter, followed by urine-voided, blood, sputum, other, open pus, vaginal discharge, Tip, ear discharge, bronchial aspiration and bile, in that order. Looking at the age distribution, the detection rate of patients in their 60s and older was significantly higher at 75.8% (1,900/2,508). The detection rate of patients in their 20s and younger was shown to be very low at 2.55% (64/2,508). By year, the detection rate of non-albicans Candida spp. showed a tendency to gradually increase each year compared with C. albicans. As isolation of Candida spp. from clinical samples at the specie level can vary depending on characteristics of the patient, sample, season, etc., continual studies are required.

  9. Clinical characteristics of 41 patients with intractable asthma

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    Li BAI

    2011-09-01

    Full Text Available Objective To explore the clinical characteristics of intractable asthma,and to provide new knowledge for diagnosis and treatment of the disease.Methods Forty one patients with intractable asthma,admitted to the Institute of Respiratory Disease,Xinqiao Hospital of Third Military Medical University from Jan.2009 to Dec.2010,were included in present study.Spirometry tests were performed for all the 41 patients.Cell classification and counting were done in the induced sputum of 37 patients,and 34 patients underwent high-resolution chest computed tomography(HRCT.Results Incomplete reversibility of airflow obstruction(FEV1/FVC 0.03 of the total cells,13(35.1% and increased neutrophils( > 0.61 of total cells,6(16.2% showed increased both eosinophils and neutrophils,and only that of one patient showed normal percentage of the eosinophils and neutrophils.Chest HRCT of 34 patients showed thickening of bronchial wall in visible segment in 28 cases(82.3%,and in 22 cases(64.7% thickening of bronchial wall in secondary segments was accompanied with narrowed bronchus lumen,cylindrical bronchiectasis was predominant in 7 patients,and centrilobular emphysema was seen in 5 patients.Conclusion Airway remodeling,incomplete reversibility of airflow obstruction,airway inflammation appear to be the major clinical characteristics of intractable asthma.Combined use of chest HRCT,spirometry test,and cellular analysis of induced sputum may be helpful for identifying intractable asthma,and they provide the basis for individualized strategies to manage the disease.

  10. Clinical characteristics of 4355 patients with anterior cruciate ligament injury

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    MEI Yu; AO Ying-fang; WANG Jian-quan; MA Yong; ZHANG Xin; WANG Jia-ning; ZHU Jing-xian

    2013-01-01

    Background Clinical features of anterior cruciate ligament (ACL) injury are important for its prevention,diagnosis and treatment.However,few studies have reported such data,especially in China.The purpose of this study was to describe the clinical characteristics of ACL injury on a large cohort.Methods Between 1993 and 2007,a total of 4355 ACL deficient inpatients (612 athletes and 3743 non-athletes) were registered.Data were collected using a special database system.And the distributions of characteristics in different groups were compared and analyzed statistically.Results All subjects were confirmed with ACL tear during surgery.Statistical analysis revealed that the percentage of females in Athlete Group was significantly higher than that in Non-athlete Group (56.05% vs.24.95%,P<0.001).This study also found that sports trauma was the main cause of ACL tears.Soccer,basketball,judo,wrestling and track and field were the five most responsible activities for athletes.The average injury time for athletes was significantly shorter than that for non-athletes (413.3 days vs.717.5 days,P<0.001).Three thousand nine hundred and eight cases were ordered ACL reconstruction (76.04% single-bundle,18.30% double-bundle).Three hundred and forty-five patients (7.92%)were combined with other ligaments injuries,2667 (61.24%) were found with various grades of cartilage lesions,and 3377 (77.54%) were found with meniscal injury.Conclusions Sports trauma was the main cause of ACL tears in China,and reconstruction had become the principal surgical choice.In order to restore knee joint stability and reduce the incidence of cartilage and meniscal injury,patienttailored ACL reconstruction should be suggested at the right moment.

  11. Clinical characteristics of children and young adults with co-occurring autism spectrum disorder and epilepsy.

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    El Achkar, Christelle M; Spence, Sarah J

    2015-06-01

    The association between autism spectrum disorder (ASD) and epilepsy has been described for decades, and yet we still lack the full understanding of this relationship both clinically and at the pathophysiologic level. This review evaluates the available data in the literature pertaining to the clinical characteristics of patients with autism spectrum disorder who develop epilepsy and, conversely, patients with epilepsy who develop autism spectrum disorder. Many studies demonstrate an increased risk of epilepsy in individuals with ASD, but rates vary widely. This variability is likely secondary to the different study methods employed, including the study population and definitions of the disorders. Established risk factors for an increased risk of epilepsy in patients with ASD include intellectual disability and female gender. There is some evidence of an increased risk of epilepsy associated with other factors such as ASD etiology (syndromic), severity of autistic features, developmental regression, and family history. No one epilepsy syndrome or seizure type has been associated, although focal or localization-related seizures are often reported. The age at seizure onset can vary from infancy to adulthood with some evidence of a bimodal age distribution. The severity and intractability of epilepsy in populations with ASD have not been well studied, and there is very little investigation of the role that epilepsy plays in the autism behavioral phenotype. There is evidence of abnormal EEGs (especially epileptiform abnormalities) in children with ASD even in the absence of clinical seizures, but very little is known about this phenomenon and what it means. The development of autism spectrum disorder in patients with epilepsy is less well studied, but there is evidence that the ASD risk is greater in those with epilepsy than in the general population. One of the risk factors is intellectual disability, and there is some evidence that the presence of a particular seizure

  12. Clinical and epidemiological characteristics of infectious keratitis in Paraguay.

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    Nentwich, Martin M; Bordón, M; di Martino, D Sánchez; Campuzano, A Ruiz; Torres, W Martínez; Laspina, F; Lichi, S; Samudio, M; Farina, N; Sanabria, Rosa R; de Kaspar, Herminia Mino

    2015-06-01

    To describe the clinical and epidemiological characteristics of patients with severe infectious keratitis in Asunción, Paraguay between April 2009 and September 2011. All patients with the clinical diagnosis of severe keratitis (ulcer ≥2 mm in size and/or central location) were included. Empiric treatment consisted of topical antibiotics and antimycotics; in cases of advanced keratitis, fortified antibiotics were used. After microbiological analysis, treatment was changed if indicated. In total 48 patients (62.5 % males, 25 % farmers) were included in the analysis. A central ulcer was found in 81.3 % (n = 39). The median delay between onset of symptoms and time of first presentation at our institution was 7 days (range 1-30 days). Fungal keratitis was diagnosed in 64.5 % (n = 31) of patients, of which Fusarium sp. (n = 17) was the most common. Twenty-one patients (43.8 %) reported previous trauma to the eye. The globe could be preserved in all cases. While topical therapy only was sufficient in most patients, a conjunctival flap was necessary in six patients suffering from fungal keratitis. The high rate of fungal keratitis in this series is remarkable, and microbiological analysis provided valuable information for the appropriate treatment. In this setting, one has to be highly suspicious of fungal causes of infectious keratitis.

  13. Clinical and laboratory characteristics of children with Kawasaki disease

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    Fatih Akın

    2015-03-01

    Full Text Available Objective: In this study, we aimed to evaluate clinical and laboratory characteristics of patients with Kawasaki disease (KD in Konya region of Turkey. Methods: The hospital records of patients who were hospitalized with the diagnosis of KD in the Pediatrics Clinics of Konya Training and Research Hospital between May 2010 and June 2012 were reviewed retrospectively. Results: Seven cases were found to have the diagnosis of KD, two of whom were incomplete KD. Oropharynx changes were the most common (100% feature in our patients. Five (71% patients had bulbar conjunctivitis. Three (43% patients had erythema at the site of BCG inoculation. Adenopathy was present in all of our patients with the classical form. A desquamation was observed in one case at the seventh day of fever. No cardiac manifestation was seen. Elevated erythrocyte sedimentation rate and thrombocytosis were present in all patients. All of the patients were received intravenous immunoglobulin in the first ten days of the fever. Conclusion: KD should be considered as a possible diagnosis in any child presenting with prolonged fever. BCG reaction can be attributed as a diagnostic criterion for incomplete form of the disease especially in countries where BCG vaccination is routinely performed. Early treatment is essential to prevent cardiovascular complications.

  14. The demographic and clinical characteristics of leprosy in Saudi Arabia.

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    Alotaibi, Mohammad H; Bahammam, Salman A; Ur Rahman, Saeed; Bahnassy, Ahmed A; Hassan, Imad S; Alothman, Adel F; Alkayal, Abdulkareem M

    2016-01-01

    Leprosy is a chronic disease caused by Mycobacterium leprae. Although the occurrence of leprosy has declined in Saudi Arabia, it has not yet been eradicated. To our knowledge, this descriptive retrospective study is the first to assess the clinical presentation of leprosy at the time of diagnosis in Saudi Arabia. All study subjects were leprosy patients admitted to Ibn Sina hospital, the only referral hospital for leprosy in Saudi Arabia, between January 2000 and May 2012. A total of 164 subjects, the majority of whom (65%) were between 21 and 50 years of age, were included, and the male-to-female ratio was 2.8:1. Of these 164 patients, 63% were Saudis, and 77% of all admitted patients were from the western region. Lepromatous leprosy was observed most frequently (33%), and 31% of cases had a positive history of close contact with leprosy. At the time of diagnosis, 84% of all subjects presented with skin manifestation. The prevalence of neurological deficit at the time of diagnosis was 87%. Erythema nodosum leprosum (E.N.L.) developed in only 10% of all subjects. Further studies are needed to determine the clinical characteristics pertaining to each type of leprosy in the region, and training courses in caring for and diagnosing patients with leprosy should be organized for health workers.

  15. Clinical trial participant characteristics and saliva and DNA metrics

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    Richards Julie

    2009-10-01

    Full Text Available Abstract Background Clinical trial and epidemiological studies need high quality biospecimens from a representative sample of participants to investigate genetic influences on treatment response and disease. Obtaining blood biospecimens presents logistical and financial challenges. As a result, saliva biospecimen collection is becoming more frequent because of the ease of collection and lower cost. This article describes an assessment of saliva biospecimen samples collected through the mail, trial participant demographic and behavioral characteristics, and their association with saliva and DNA quantity and quality. Methods Saliva biospecimens were collected using the Oragene® DNA Self-Collection Kits from participants in a National Cancer Institute funded smoking cessation trial. Saliva biospecimens from 565 individuals were visually inspected for clarity prior to and after DNA extraction. DNA samples were then quantified by UV absorbance, PicoGreen®, and qPCR. Genotyping was performed on 11 SNPs using TaqMan® SNP assays and two VNTR assays. Univariate, correlation, and analysis of variance analyses were conducted to observe the relationship between saliva sample and participant characteristics. Results The biospecimen kit return rate was 58.5% among those invited to participate (n = 967 and 47.1% among all possible COMPASS participants (n = 1202. Significant gender differences were observed with males providing larger saliva volume (4.7 vs. 4.5 ml, p = 0.019, samples that were more likely to be judged as cloudy (39.5% vs. 24.9%, p 0.21, P Conclusion Findings from this study show that demographic and behavioral characteristics of smoking cessation trial participants have significant associations with saliva and DNA metrics, but not with the performance of TaqMan® SNP or VNTR genotyping assays. Trial registration COMPASS; registered as NCT00301145 at clinicaltrials.gov.

  16. Clinical characteristics and prognostic factors of severe acute pancreatitis

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    Lei Kong; Nn Santiago; Tian-Quan Han; Sheng-Dao Zhang

    2004-01-01

    AIM: To investigate the clinical characteristics and prognostic factors of a consecutive series of patients with severe acute pancreatitis (SAP).METHODS: Clinical data of SAP patients admitted to our hospital from January 2003 to January 2004 were retrospectively reviewed. Collected data included the age, gender, etiology,length of hospitalization, APACHE Ⅱ score at admission,local and organ/systemic complications of the patients.RESULTS: Of the 268 acute pancreatitis patients, 94 developed SAP. The mean age of SAP patients was 52 years, the commonest etiology was cholelithiasis (45.7%), the mean length of hospitalization was 70 d, the mean score of APACHE Ⅱ was 7.7. Fifty-four percent of the patients developed necrosis, 25% abscess, 58% organ/systemic failure. A total of 23.4% (22/94) of the SAP patients died. Respiratory failure was the most common organ clysfunction (90.9%) in deceased SAP patients, followed by cardiovascular failure (86.4%),renal failure (50.0%). In the SAP patients, 90.9% (20/22)developed multiple organ/systemic failures. There were significant differences in age, length of hospitalization,APACHE Ⅱ score and incidences of respiratory failure, renal failure, cardiovascular failure and hematological failure between deceased SAP patients and survived SAP patients.By multivariate logistic regression analysis, independent prognostic factors for mortality were respiratory failure,cardiovascular failure and renal failure.CONCLUSION: SAP patients are characterized by advanced age, high APACHE Ⅱ score, organ failure and their death is mainly due to multiple organ/systemic failures. In patients with SAP, respiratory, cardiovascular and renal failures can predict the fatal outcome and more attention should be paid to their clinical evaluation.

  17. [Current insights into the pathophysiology of rosacea].

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    Schauber, J; Homey, B; Steinhoff, M

    2013-07-01

    Rosacea is a chronic inflammatory skin disease mainly affecting the face. Four major clinical subtypes of rosacea can be identified: erythemato-telangiectatic, papulopustular, phymatous and ocular rosacea. Still, it is currently unclear whether these subtypes develop consecutively or if any subtypes may occur individually as part of a syndrome. Rosacea is characterized by facial flushing, erythema, chronic inflammation, edema and fibrosis. Several trigger factors can worsen the disease or cause recurring episodes of inflammation. Although some aspects in the pathophysiology of rosacea have been characterized in more detail during the past years, the precise interplay of the various dysregulated systems is still poorly understood. In early disease manifestations and milder stages, dysfunction of neurovascular regulation and the innate immune system seem to be driving forces in rosacea pathophysiology. A disturbed chemokine and cytokine network further contributes to disease progression. This current review highlights some of the recent findings in rosacea pathophysiology and points out novel targets for therapeutic intervention.

  18. Claudin-Low Breast Cancer; Clinical & Pathological Characteristics

    Science.gov (United States)

    Dias, Kay; Dvorkin-Gheva, Anna; Hallett, Robin M.; Wu, Ying; Hassell, John; Pond, Gregory R.; Levine, Mark; Whelan, Tim; Bane, Anita L.

    2017-01-01

    Claudin-low breast cancer is a molecular type of breast cancer originally identified by gene expression profiling and reportedly associated with poor survival. Claudin-low tumors have been recognised to preferentially display a triple-negative phenotype, however only a minority of triple-negative breast cancers are claudin-low. We sought to identify an immunohistochemical profile for claudin-low tumors that could facilitate their identification in formalin fixed paraffin embedded tumor material. First, an in silico collection of ~1600 human breast cancer expression profiles was assembled and all claudin-low tumors identified. Second, genes differentially expressed between claudin-low tumors and all other molecular subtypes of breast cancer were identified. Third, a number of these top differentially expressed genes were tested using immunohistochemistry for expression in a diverse panel of breast cancer cell lines to determine their specificity for claudin-low tumors. Finally, the immunohistochemical panel found to be most characteristic of claudin-low tumors was examined in a cohort of 942 formalin fixed paraffin embedded human breast cancers with >10 years clinical follow-up to evaluate the clinico-pathologic and survival characteristics of this tumor subtype. Using this approach we determined that claudin-low breast cancer is typically negative for ER, PR, HER2, claudin 3, claudin 4, claudin 7 and E-cadherin. Claudin-low tumors identified with this immunohistochemical panel, were associated with young age of onset, higher tumor grade, larger tumor size, extensive lymphocytic infiltrate and a circumscribed tumor margin. Patients with claudin-low tumors had a worse overall survival when compared to patients with luminal A type breast cancer. Interestingly, claudin-low tumors were associated with a low local recurrence rate following breast conserving therapy. In conclusion, a limited panel of antibodies can facilitate the identification of claudin-low tumors

  19. Sepsis in AIDS patients: clinical, etiological and inflammatory characteristics

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    João Manoel Silva

    2013-01-01

    Full Text Available Introduction: Intensive care mortality of HIV-positive patients has progressively decreased. However, critically ill HIV-positive patients with sepsis present a worse prognosis. To better understand this condition, we propose a study comparing clinical, etiological and inflammatory data, and the hospital course of HIV-positive and HIV-negative patients with severe sepsis or septic shock. Methods: A prospective observational study enrolling patients with severe sepsis or septic shock associated or not with HIV infection, and admitted to intensive care unit (ICU. Clinical, microbiological and inflammatory parameters were assessed, including C-reactive protein (CRP, procalcitonin (PCT, interleukin-6, interleukin-10 and TNF-α. Outcome measures were in-hospital and six-month mortality. Results: The study included 58 patients with severe sepsis/septic shock admitted to ICU, 36 HIV-positive and 22 HIV-negative. All HIV-positive patients met the criteria for AIDS (CDC/2008. The main foci of infection in HIV-positive patients were pulmonary and abdominal (p=0.001. Fungi and mycobacteria were identified in 44.4% and 16.7% of HIV-positive patients, respectively. In contrast, the main etiologies for sepsis in HIV-negative patients were Gram-negative bacilli (36.4% and Gram-positive cocci (36.4% (p=0.001. CRP and PCT admission concentrations were lower in HIV-positive patients (130 vs. 168 mg/dL p=0.005, and 1.19 vs. 4.06 ng/mL p=0.04, respectively, with a progressive decrease in surviving patients. Initial IL-10 concentrations were higher in HIV-positive patients (4.4 pg/mL vs. 1.0 pg/mL, p=0.005, with moderate accuracy for predicting death (area under receiver-operating characteristic curve =0.74. In-hospital and six-month mortality were higher in HIV-positive patients (55.6 vs. 27.3% p=0.03, and 58.3 vs. 27.3% p=0.02, respectively. Conclusions: The course of sepsis was more severe in HIV-positive patients, with distinct clinical, etiological and

  20. Clinical and psychosocial characteristics of children with nonepileptic seizures

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    Chinta Sri

    2008-01-01

    Full Text Available Objective: The aim of this study is to present a comprehensive profile of clinical and psychosocial characteristics of children with psychogenic nonepileptic seizures and to assess the short-term outcome of these patients. Materials and Methods: The subjects were consecutive cases of children with a diagnosis of nonepileptic seizures (N=17, mean age = 10.7 years, S.D. = 1.26 and two groups of control groups matched on age and sex: true seizure group and healthy controls. All the children were recruited from the out-patient services of the Department of Pediatrics of a tertiary care teaching hospital in North India. Detailed history taking and clinical examination was done in the case of every child. A standard 18 channel EEG was done in all the children and a video EEG was done in 12 cases of children with nonepileptic seizures. The Childhood Psychopathology Measurement Schedule (CPMS and Life Events Scale for Indian Children (LESIC were used to measure the children′s emotional and behavioral functioning at home, and the number of life events and the stress associated with these events in the preceding year and the year before that. Short-term outcome was examined three to six months after the diagnosis of nonepileptic seizures was made. Results: Unresponsiveness without marked motor manifestations was the most common "ictal" characteristic of the nonepileptic seizures. Pelvic thrusting, upper and lower limb movements, head movements, and vocalization were observed in less than one-third of the patients. Increased psychosocial stress and significantly higher number of life events in the preceding year were found to characterize children with nonepileptic seizures, as compared to the two control groups. The nonepileptic seizures and true seizures groups had a higher proportion of children with psychopathology scores in the clinically significant maladjustment range, as compared to those in the healthy control group. A majority of the patients

  1. Pathophysiological Study of Sensitive Skin.

    Science.gov (United States)

    Buhé, Virginie; Vié, Katell; Guéré, Christelle; Natalizio, Audrey; Lhéritier, Céline; Le Gall-Ianotto, Christelle; Huet, Flavien; Talagas, Matthieu; Lebonvallet, Nicolas; Marcorelles, Pascale; Carré, Jean-Luc; Misery, Laurent

    2016-03-01

    Sensitive skin is a clinical syndrome characterized by the occurrence of unpleasant sensations, such as pruritus, burning or pain, in response to various factors, including skincare products, water, cold, heat, or other physical and/or chemical factors. Although these symptoms suggest inflammation and the activation of peripheral innervation, the pathophysiogeny of sensitive skin remains unknown. We systematically analysed cutaneous biopsies from 50 healthy women with non-sensitive or sensitive skin and demonstrated that the intraepidermal nerve fibre density, especially that of peptidergic C-fibres, was lower in the sensitive skin group. These fibres are involved in pain, itching and temperature perception, and their degeneration may promote allodynia and similar symptoms. These results suggest that the pathophysiology of skin sensitivity resembles that of neuropathic pruritus within the context of small fibre neuropathy, and that environmental factors may alter skin innervation.

  2. Fisiopatologia da enxaqueca Migraine pathophysiology

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    MAURICE B. VINCENT

    1998-12-01

    Full Text Available A fisiopatologia da enxaqueca ainda não foi completamente elucidada. As principais estruturas envolvidas parecem ser o sistema nervoso central (córtex e tronco cerebral, o sistema trigeminovascular e os vasos correspondentes, outras fibras autonômicas que inervam estes vasos, e os vários agentes vasoativos locais, como a SP, CGRP, NO, VIP, NPY, ACh, NA, NKA, entre outros. A depressão alastrante é o fenômeno neurológico que provavelmente justifica achados experimenais e clínicos na enxaqueca. Ela tem velocidade de propagação semelhante à aura, ativa o núcleo espinhal do trigêmeo e está relacionada à liberação de CGRP e NO. Alterações circulatórias detectadas por métodos complementares reforçam o papel da depressão alastrante. A identificação de anormalidades em pelo menos três loci (cromossomas 19 e 1 na enxaqueca hemiplégica familiar ocorreu recentemente. Elas estão relacionadas a anormalidades nos canais de cálcio voltagem dependentes tipo P/Q, específicos do sistema nervoso central, que regulam a liberação de vários neurotransmissores, incluindo possivelmente a serotonina. A exemplo de outras anormalidades neurológicas paroxísticas que resultam da hiperexcitabilidade da membrana plasmática, é possível que a enxaqueca ocorra devido a uma desordem de canais iônicos.The pathophysiology of migraine is not yet fully understood. The most important structures involved seem to be the central nervous system (cortex and brain stem, the trigeminovascular system and related cranial arteries, other autonomic fibres innervating such vessels, and various local vasoactive agents, including SP, CGRP, NO, VIP, NPY, ACh, NA, NKA, among others. The spreading depression phenomenon may explain clinical as well experimental findings in migraine. Its propagation velocity mirrors what is found in clinical aura, it may activate the spinal trigeminal nucleus and may induce CGRP and NO release. Circulatory changes detected with

  3. Clinical characteristics of adult patients with inborn errors of metabolism in Spain: A review of 500 cases from university hospitals

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    J. Pérez-López

    2017-03-01

    Full Text Available Patients with inborn errors of metabolism (IEMs have become an emerging and challenging group in the adult healthcare system whose needs should be known in order to implement appropriate policies and to adapt adult clinical departments. We aimed to analyze the clinical characteristics of adult patients with IEMs who attend the most important Spanish hospitals caring for these conditions. A cohort study was conducted in 500 patients, categorized by metabolic subtype according to pathophysiological classification. The most prevalent group of IEMs was amino acid disorders, with 108 (21.6% patients diagnosed with phenylketonuria. Lysosomal storage disorders were the second group, in which 32 (6.4% and 25 (5% patients had Fabry disease and Gaucher disease respectively. The great clinical heterogeneity, the significant delay in diagnosis after symptom onset, the existence of some degree of physical dependence in a great number of patients, the need for a multidisciplinary and coordinated approach, and the lack of specific drug treatment are common features in this group of conditions.

  4. Maladaptive Reward-Learning and Impulse Control Disorders in Patients with Parkinson’s Disease: A Clinical Overview and Pathophysiology Update

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    Jee-Young Lee

    2014-10-01

    Full Text Available Impulse control disorders (ICD in Parkinson’s disease (PD are a disabling non-motor symptom with frequencies of 13–35% among patients receiving dopamine replacement therapy. ICD in PD is strongly associated with dopaminergic drug use, especially non-ergot dopamine agonists (DA. However, individual susceptibility and disease-related neural changes are also important contributors to the development of ICD. Discrepancies between nigrostriatal and mesolimbic dopaminergic degeneration and non-physiological administration of dopaminergic drugs may induce abnormal ’hyperstimulation’ of the mesolimbic system, which alters reward-learning behaviors in PD patients. In addition, DA can make patients more impulsive during decision-making and seek risk-taking behaviors. DA intake is also related to the biased representation of rewards. Ultimately, loss of negative feedback control due to dysfunctional frontostriatal connections is necessary for the establishment of ICD in PD. The subsequent behavioral and neural changes are affected by PD treatment and disease progression; thus, proper treatment guidelines for physicians are needed to prevent the development of ICD. Future studies aimed at producing novel therapeutics to control the risk factors for ICD or treat ICD behaviors in PD are warranted. This review summarizes recent advances from epidemiological and pathophysiological studies on ICD in PD. Management principles and limitations of current therapeutics are briefly discussed.

  5. Clinical characteristics and perinatal outcome of fetal hydrops

    Science.gov (United States)

    Yeom, Wonkyung; Paik, E Sun; An, Jung-Joo; Oh, Soo-young; Roh, Cheong-Rae; Kim, Jong-Hwa

    2015-01-01

    Objective To investigate the clinical characteristics of fetal hydrops and to find the antenatal ultrasound findings predictive of adverse perinatal outcome. Methods This is a retrospective study of 42 women with fetal hydrops who delivered in a tertiary-referral center from 2005 to 2013. Fetal hydrops was defined as the presence of fluid collection in ≥2 body cavities: ascites, pleural effusion, pericardial effusion, and skin edema. Predictor variables recorded included: maternal characteristics, gestational age at diagnosis, ultrasound findings, and identifiable causes. Primary outcome variables analyzed were fetal death and neonatal death. Results The mean gestational age at diagnosis was 29.3±5.4 weeks (range, 18 to 39 weeks). The most common identifiable causes were cardiac abnormality (10), followed by syndrome (4), aneuploidy (3), congenital infection (3), twin-to-twin transfusion syndrome (3), non-cardiac anormaly (2), chorioangioma (2), inborn errors of metabolism (1), and immune hydrops by anti-E antibody isoimmunization (1). Thirteen cases had no definite identifiable causes. Three women elected termination of pregnancy. Fetal death occurred in 4 cases. Among the 35 live-born babies, only 16 survived (54.0% neonatal mortality rate). Fetal death and neonatal mortality rate was not significantly associated with Doppler velocimetry indices or location of fluid collection, but increasing numbers of fluid collection site was significantly associated with a higher risk of neonatal death. Conclusion The incidence of fetal hydrops in our retrospective study was 24.4 per 10,000 deliveries and the perinatal mortality rate was 61.9% (26/42). The number of fluid collection sites was the significant antenatal risk factor to predict neonatal death. PMID:25798421

  6. Clinical characteristics of intermediate uveitis in adult Turkish patients

    Institute of Scientific and Technical Information of China (English)

    Esra; Kardes; Betul; Ilkay; Sezgin; Akcay; Kansu; Bozkurt; Cihan; Unlu; Gurkan; Erdogan; Gulunay; Akcali

    2015-01-01

    ·AIM: To describe the clinical characteristics of Turkish patients with intermediate uveitis(IU) and to investigate the effect of clinical findings and complications on final visual acuity(VA).·METHODS: We retrospectively analyzed the medical records of patients with IU who had at least 6mo of follow-up and were older than 16 y.· RESULTS: A total of 78 eyes of 45 patients were included in the study and the mean follow-up period was19.4mo. The mean age at the time of presentation was42.9s. Systemic disease associations were found in17.7% of cases; sarcoidosis(8.8%) and multiple sclerosis(6.6%) were the most common diseases. Recurrence rate(odds ratio=45.53; 95%CI: 2.181-950.58), vitritis equals to or more than 3+ cells(odds ratio =57.456; 95%CI: 4.154-794.79) and presenting with VA less than 20/40(odds ratio =43.81; 95% CI: 2.184-878.71) were also found as high risk factors for poor final VA. At the last follow-up examination, 67.9% of eyes had VA of 20/40 or better.·CONCLUSION: IU is frequently seen at the beginning of the fourth decade of life. The disease is most commonly idiopathic in adult Turkish patients. Patients with severe vitritis at presentation and patients with frequent recurrences are at high risk for poor visual outcome.

  7. Holoprosencephaly: Epidemiologic and clinical characteristics of a California population

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    Croen, L.A.; Shaw, G.M. [California Birth Defects Monitoring Program, Emeryville, CA (United States); Lammer, E.J. [Children`s Hospital, Oakland, CA (United States)

    1996-08-23

    Holoprosencephaly is a brain defect resulting from incomplete cleavage of the embryonic forebrain. It involves forebrain and facial malformations that can range from mild to severe. The epidemiology of holoprosencephaly is largely unknown. Published prevalence estimates have been derived from clinic-based case series, and suggested risk factors for holoprosencephaly have been identified in case reports, without confirmation from systematically conducted population-based studies. Using data from a population-based birth defects registry in California, we described the epidemiologic and clinical characteristics of cytogenetically and phenotypically distinct types of holoprosencephaly. A total of 121 cases was identified among a cohort of 1,035,386 live births and fetal deaths. The prevalence of holoprosencephaly was 1.2 per 10,000 births (95% confidence interval 1.0-1.4 per 10,000). Of all cases, 41% (50/121) had a chromosomal abnormality, most commonly Trisomy 13. Among the 71 cytogenetically apparently normal cases, 18 had recognizable syndromes and the remaining 53 were of unknown cause. Among the cytogenetically abnormal cases, females had a greater risk than males (odds ratio = 2.3, 95% confidence interval [1.2, 4.4]). Among the cytogenetically normal cases, increased risks were observed among Hispanic whites (OR = 1.8 [0.9, 3.6]) and cases whose mother was born in Mexico (OR = 2.2 [1.0, 4.5]). Approximately 46% of all cases had alobar holoprosencephaly, the most severe form of the forebrain malformation. The facial phenotype did not strongly predict the severity of the brain defect; however, severity was inversely correlated with length of survival. This study is the first to present findings based on such a large population-based series of infants/fetuses affected by holoprosencephaly, and demonstrates the importance of investigating the component subgroups of this rare phenotype. 47 refs., 7 tabs.

  8. Acute Viral Hepatitis A – Clinical, Laboratory and Epidemiological Characteristics

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    Melinda HORVAT

    2013-06-01

    Full Text Available Background and Aims: Infection with hepatitis A virus is still one of the most common causes of hepatitis worldwide. The clinical manifestation of acute hepatitis A (AHA in adults can vary greatly, ranging from asymptomatic infection to severe and fulminant hepatitis. The aim of this study was to describe the demographic, clinical characteristics, laboratory features and hospital outcome of adult patients with AHA over a consecutive period of 4 years within an area from Eastern European country. Methods: Two hundred and two adult patients diagnosed with AHA were retrospective, observational and analytic analized over a period of 4 years. Based on prothrombin time less than 50, the study group was stratified in medium (79.2% and severe forms (20.8%. We investigated the clinical, laboratory and epidemiological features. Statistical analysis were applied to compare the medium and severe forms of AHA. Results: Most patients (72.7% were younger than 40 years. The main symptoms included: dyspepsia (72.07%, jaundice (86.63%, asteno-adynamia (86.72%, and flu-like symptoms (53.46%. The hemorrhagic cutaneous-mucous manifestations (6.93% associated with the severe forms of AHA (OR =12.19, 95%CI -3.59 - 41.3, p =0.001. We found statistically significant differences for PT (p <0.001, INR (p <0.001, TQ (p <0.001, ALAT (p <0.001, ASAT (p <0.001, ALP (p <0.001 and platelets (p =0.009 between severe and medium AHA forms. We found that TQ, INR, ALAT and ASAT have the highest diagnostic values, statistically significant (p <0.05 for severe AHA forms with AUC (0.99, 0.99, 0.72, 0.70 at values of sensitivity (95%, 90.5%, 89%, 95% and specificity (98%, 99%, 88%,94%. Conclusions Medium severity AHA forms were found in most of the study group patients (79.2%. The severe AHA forms were associated with hemorrhagic cutaneous-mucous manifestations (OR =12.19, p =0.001. The univariate analysis proved a negatively statistically significant correlation between IP and ALAT

  9. Clinical characteristics of zinc phosphide poisoning in Thailand

    Science.gov (United States)

    Trakulsrichai, Satariya; Kosanyawat, Natcha; Atiksawedparit, Pongsakorn; Sriapha, Charuwan; Tongpoo, Achara; Udomsubpayakul, Umaporn; Rittilert, Panee; Wananukul, Winai

    2017-01-01

    Objective The objectives of this study were to describe the clinical characteristics and outcomes of poisoning by zinc phosphide, a common rodenticide in Thailand, and to evaluate whether these outcomes can be prognosticated by the clinical presentation. Materials and methods A 3-year retrospective cohort study was performed using data from the Ramathibodi Poison Center Toxic Exposure Surveillance System. Results In total, 455 poisonings were identified. Most were males (60.5%) and from the central region of Thailand (71.0%). The mean age was 39.91±19.15 years. The most common route of exposure was oral (99.3%). Most patients showed normal vital signs, oxygen saturation, and consciousness at the first presentation. The three most common clinical presentations were gastrointestinal (GI; 68.8%), cardiovascular (22.0%), and respiratory (13.8%) signs and symptoms. Most patients had normal blood chemistry laboratory results and chest X-ray findings at presentation. The median hospital stay was 2 days, and the mortality rate was 7%. Approximately 70% of patients underwent GI decontamination, including gastric lavage and a single dose of activated charcoal. In all, 31 patients were intubated and required ventilator support. Inotropic drugs were given to 4.2% of patients. Four moribund patients also received hyperinsulinemia–euglycemia therapy and intravenous hydrocortisone; however, all died. Patients who survived and died showed significant differences in age, duration from taking zinc phosphide to hospital presentation, abnormal vital signs at presentation (tachycardia, low blood pressure, and tachypnea), acidosis, hypernatremia, hyperkalemia, in-hospital acute kidney injury, in-hospital hypoglycemia, endotracheal tube intubation, and inotropic requirement during hospitalization (P<0.05). Conclusion Zinc phosphide poisoning causes fatalities. Most patients have mild symptoms, and GI symptoms are the most common. Patients who present with abnormal vital signs or

  10. Clinical Characteristics of Adults with Asperger's Syndrome Assessed with Self-Report Questionnaires

    Science.gov (United States)

    Kanai, Chieko; Iwanami, Akira; Ota, Haruhisa; Yamasue, Hidenori; Matsushima, Eisuke; Yokoi, Hideki; Shinohara, Kazuyuki; Kato, Nobumasa

    2011-01-01

    Diagnosis of Asperger's Syndrome (AS) in adults is difficult, and clinical sample-based studies that systematically illustrate the clinical characteristics of adult AS patients are needed so that appropriate treatment can be provided. Here we examined the clinical characteristics of AS in 112 adults (median age, 28.0 years [range, 18-52]; 71 men…

  11. Clinical Characteristics and Genetic Variability of Human Rhinovirus in Mexico

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    Hilda Montero

    2012-01-01

    Full Text Available Human rhinovirus (HRV is a leading cause of acute respiratory infection (ARI in young children and infants worldwide and has a high impact on morbidity and mortality in this population. Initially, HRV was classified into two species: HRV-A and HRV-B. Recently, a species called HRV-C and possibly another species, HRV-D, were identified. In Mexico, there is little information about the role of HRV as a cause of ARI, and the presence and importance of species such as HRV-C are not known. The aim of this study was to determine the clinical characteristics and genetic variability of HRV in Mexican children. Genetic characterization was carried out by phylogenetic analysis of the 5′-nontranslated region (5′-NTR of the HRV genome. The results show that the newly identified HRV-C is circulating in Mexican children more frequently than HRV-B but not as frequently as HRV-A, which was the most frequent species. Most of the cases of the three species of HRV were in children under 2 years of age, and all species were associated with very mild and moderate ARI.

  12. Characteristics of Veterans Health Administration chiropractors and chiropractic clinics.

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    Lisi, Anthony J; Goertz, Christine; Lawrence, Dana J; Satyanarayana, Preeti

    2009-01-01

    Chiropractic services have been delivered on station at select Veterans Health Administration (VHA) medical facilities since late 2004. No published data describing the characteristics of VHA chiropractic physicians (chiropractors) and chiropractic clinics exist at a national level. This study was designed to examine elements of the structures of chiropractic services in VHA settings. Web-based survey methods were used to question all chiropractors in VHA facilities (N = 36). Data were obtained from 33 providers, yielding a 91.6% response rate. Most respondents were full-time VHA employees, while others were part-time employees or contractors. Differences were found in prior training, integrated practice, and academic or research experience. Of the respondents, 88% ranked low back pain as the most common patient complaint seen in practice and 79% ranked cervical pain the second most common complaint. Of the new patient consultations, 67.6% originated from primary care, 9.4% from pain management, and 6.2% from physiatry. Most respondents were similar in their reported use of diagnostic and therapeutic procedures, but their reported rates of participation in various facility activities were different. Further work is needed for researchers and policy makers to more fully understand the integration and delivery of chiropractic services in VHA settings.

  13. Epidemiological, clinical, and immunological characteristics of neuromyelitis optica: A review.

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    Pereira, Wildéa Lice de Carvalho Jennings; Reiche, Edna Maria Vissoci; Kallaur, Ana Paula; Kaimen-Maciel, Damacio Ramón

    2015-08-15

    The aim of this study was to review the epidemiological and clinical characteristics of neuromyelitis optica (NMO) and the immunopathological mechanisms involved in the neuronal damage. NMO is an inflammatory demyelinating autoimmune disease of the central nervous system that most commonly affects the optic nerves and spinal cord. NMO is thought to be more prevalent among non-Caucasians and where multiple sclerosis (MS) prevalence is low. NMO follows a relapsing course in more than 80-90% of cases, which is more commonly in women. It is a complex disease with an interaction between host genetic and environmental factors and the main immunological feature is the presence of anti-aquaporin 4 (AQP4) antibodies in a subset of patients. NMO is frequently associated with multiple other autoantibodies and there is a strong association between NMO with other systemic autoimmune diseases. AQP4-IgG can cause antibody-dependent cellular cytotoxicity (ADCC) when effector cells are present and complement-dependent cytotoxicity (CDC) when complement is present. Acute therapies, including corticosteroids and plasma exchange, are designed to minimize injury and accelerate recovery. Several aspects of NMO pathogenesis remain unclear. More advances in the understanding of NMO disease mechanisms are needed in order to identify more specific biomarkers to NMO diagnosis.

  14. Spinal epidural angiolipomas: Clinical characteristics, management and outcomes

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    Bouali, Sofiene; Maatar, Nidhal; Bouhoula, Asma; Abderrahmen, Khansa; Said, Imed Ben; Boubaker, Adnen; Kallel, Jalel; Jemel, Hafedh

    2016-01-01

    Purpose: The spinal epidural angiolipomas are rare expansive processes made of mature lipomatous and angiomatous elements. They often have a benign character. Their etiology, pathogenesis remains uncertain, and it is a cause of spinal cord compression. The magnetic resonance imaging is the most important neuroradiological examination. Histological examination is the only examination to confirm the diagnosis. Surgery is the treatment of choice. Methods: A retrospective study of all patients operated on for a spinal epidural angiolipoma at the Department of Neurosurgery at the National Institute of Neurology of Tunis between January 2000 and December 2014 (15 years) was performed. The aim of this study is to describe the clinical, radiological, histological characteristics and the treatment of this tumor. Results: A total of nine patients were operated from January 01, 2000 to November 30, 2014. The average age of our patients was 51 years with ages that ranged from 29 to 65 with a male predominance. The period between onset of symptoms and diagnosis ranged from 24 months with an average 12 months. Posterior localization of the tumor was seen in all patients. Surgical resection was performed for all cases. The postoperative course has been satisfactory, with a complete recovery of neurological functions in all patients. Conclusions: The spinal epidural angiolipomas is rare expansive process causing spinal cord compression. Treatment is exclusively surgical resection. The functional outcome of spinal epidural angiolipomas is particularly favorable with a complete neurological recovery is if the patient was quickly operated. PMID:27695535

  15. Investigation of Tinnitus Patients in Italy: Clinical and Audiological Characteristics

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    Francesco Martines

    2010-01-01

    Full Text Available Objective. 312 tinnitus sufferers were studied in order to analyze: the clinical characteristics of tinnitus; the presence of tinnitus-age correlation and tinnitus-hearing loss correlation; the impact of tinnitus on subjects' life and where possible the etiological/predisposing factors of tinnitus. Results. There is a slight predominance of males. The highest percentage of tinnitus results in the decades 61–70. Of the tinnitus sufferers, 197 (63.14% have a hearing deficit (light hearing loss in 37.18% of cases. The hearing impairment results of sensorineural type in 74.62% and limited to the high frequencies in 58.50%. The tinnitus is referred as unilateral in 59.93%, a pure tone in 66.99% and 10 dB above the hearing threshold in 37.7%. It is limited to high frequencies in 72.10% of the patients with sensorineural hearing loss (SNHL while the 88.37% of the patients with high-frequency SNHL have a high-pitched tinnitus (2=66.26;<.005. Conclusion. Hearing status and age represent the principal tinnitus related factors; there is a statistically significant association between high-pitched tinnitus and high-frequency SNHL. There is no significant correlation between tinnitus severity and tinnitus loudness confirming the possibility that neural connection involved in evoking tinnitus-related negative reactions are governed by conditioned reflexes.

  16. Clinical characteristics and risk factors of ocular candidiasis.

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    Nagao, Miki; Saito, Takashi; Doi, Shoichi; Hotta, Gou; Yamamoto, Masaki; Matsumura, Yasufumi; Matsushima, Aki; Ito, Yutaka; Takakura, Shunji; Ichiyama, Satoshi

    2012-06-01

    Ocular candidiasis is a major complication of Candida bloodstream infection (BSI). This study was performed to reveal the clinical characteristics of ocular candidiasis. Of the 220 patients with Candida BSI, 204 cases received ophthalmology consultations between January 2005 and December 2011 at 2 teaching hospitals. Fifty-four (26.5%) cases had findings consistent with the diagnosis of ocular candidiasis. Of these 54 cases, 43 (79.6%) were diagnosed within 7 days after a positive blood culture. Among ocular candidiasis cases, more cases were due to Candida albicans (P =0.034 odds ratio [OR]; 3.68 95% confidence interval [CI] 1.11-12.2) and had higher β-d-glucan values (P = 0.001 OR; 9.99 95% CI 2.60-21.3). We need to consider fundoscopic examination to be performed within the first 7 days of therapy, especially for those patients who have C. albicans BSIs and higher β-d-glucan values. Additionally, follow-up fundoscopic examination should be considered before stopping therapy for high-risk patients.

  17. Clinical characteristics and progression of liver abscess caused by toxocara

    Institute of Scientific and Technical Information of China (English)

    Kyung; Ho; Ha; Jung; Eun; Song; Byung; Seok; Kim; Chang; Hyeong; Lee

    2016-01-01

    AIM: To evaluate the clinical characteristics and progression of liver abscess caused by toxocara.METHODS: We retrospectively reviewed the medical records of patients with serum Ig G antibody to Toxocara canis and liver abscess diagnosed using abdominal computed tomography between February 2010 and February 2015. Among 84 patients exhibiting serum Ig G antibody to Toxocara canis, 34 patients were diagnosed with liver asbscess and treated with albendazole. A follow-up period of 1 year was conducted. RESULTS: Mean patient age was 53(34-79) years, with 26(76.5%) patients being male. Twenty-one(61.7%) patients were moderate or heavy drinkers, 23(67.6%) patients had a history of eating raw meat or liver and 6(17.6%) patients owned pet dogs or cats. Main patient symptoms consisted of right upper quadrant pain, fever, and fatigue; 18(52.9%) patients, however, presented with no symptoms. Lung involvement was detected in 444(11.7%) patients. The eosinophil count increased in 29(85.3%) patients at initial diagnosis, and decreased in most patients after albendazole treatment. The initial serum Ig E level increased in 25(73.5%) patients, but exhibited various response levels after albendazole treatment. Liver abscess formation improved in all patients.CONCLUSION: The liver abscess was improved with albendazole treatment.

  18. Clinical characteristics of patients with gender identity disorder at a Japanese gender identity disorder clinic.

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    Okabe, Nobuyuki; Sato, Toshiki; Matsumoto, Yosuke; Ido, Yumiko; Terada, Seishi; Kuroda, Shigetoshi

    2008-01-15

    The aim of this study was to examine the clinical characteristics of patients with gender identity disorder (GID) at a GID clinic in Japan. A total of 603 consecutive patients were evaluated at the GID clinic using clinical information and results of physical and neurological examinations. Using DSM-IV criteria, 579 patients (96.0%) were diagnosed with GID. Four patients were excluded for transvestic fetishism, eight for homosexuality, five for schizophrenia, three for personality disorders, and four for other psychiatric disorders. Among the GID patients, 349 (60.3%) were the female-to-male (FTM) type, and 230 (39.7%) were the male-to-female (MTF) type. Almost all FTM-type GID patients started to feel discomfort with their sex before puberty and were sexually attracted to females. The proportion of FTM patients who had experienced marriage as a female was very low, and very few had children. Therefore, FTM-type GID patients seem to be highly homogeneous. On the other hand, various patterns of age at onset and sexual attraction existed among MTF patients. Among the MTF-type GID patients, 28.3% had married as males and 18.7% had sired children. Thus, MTF-type GID patients seem to be more heterogeneous.

  19. Clinical characteristics of foot ulceration in people with chronic gout.

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    Rome, Keith; Erikson, Kathryn; Otene, Cynthia; Sahid, Hazra; Sangster, Karyn; Gow, Peter

    2016-04-01

    Gout is the most common form of inflammatory arthritis and it has an affliction to the foot. Foot involvement in gout has been linked to foot pain, impairment and disability. There has been limited research on the effect of ulceration on foot pain, impairment, disability and health-related quality of life in patients already living with gout. The aim of the study was to describe the wound characteristics and the effect on foot pain, disability and health-related quality of life in patients with foot ulceration associated with gout. Participants were recruited from rheumatology clinics in Auckland, New Zealand. All the current foot ulceration sites and wound characteristics were recorded using the TIME wound assessment tool. The outcome measures included general pain, patient global assessment scale, foot pain, disability and impairment. Participants completed the Cardiff Wound Impact Schedule to assess the effect of ulcers on health-related quality of life. Sensory loss, vibrational thresholds and ankle brachial pressure index were collated to assess for lower limb arterial disease. Six participants were predominantly older men with a long duration of gout, high rates of obesity and co-morbidities such as hypertension, hyperlipidaemia, diabetes and cardiovascular disease. The mean (SD) duration of the foot ulcers was 4 (2) months. The majority of foot ulcers observed were 0·5 cm(2) or smaller superficial thickness with surrounding callus. Partial thickness and full-thickness ulcers were also observed. Two patients presented with ulcers on multiple sites. There was only one case of infection. Gouty tophi were evident in most of the wounds. The dorsal aspect of the third toe was found to ulcerate in most cases. Moderate scores of foot pain, disability, impairment and health-related quality of life were observed. Most participants wore shoes deemed as poor. Foot ulceration in gout is chronic and multiple ulcers can occur with the potential of leading to delayed

  20. Visual height intolerance and acrophobia: clinical characteristics and comorbidity patterns.

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    Kapfhammer, Hans-Peter; Huppert, Doreen; Grill, Eva; Fitz, Werner; Brandt, Thomas

    2015-08-01

    The purpose of this study was to estimate the general population lifetime and point prevalence of visual height intolerance and acrophobia, to define their clinical characteristics, and to determine their anxious and depressive comorbidities. A case-control study was conducted within a German population-based cross-sectional telephone survey. A representative sample of 2,012 individuals aged 14 and above was selected. Defined neurological conditions (migraine, Menière's disease, motion sickness), symptom pattern, age of first manifestation, precipitating height stimuli, course of illness, psychosocial impairment, and comorbidity patterns (anxiety conditions, depressive disorders according to DSM-IV-TR) for vHI and acrophobia were assessed. The lifetime prevalence of vHI was 28.5% (women 32.4%, men 24.5%). Initial attacks occurred predominantly (36%) in the second decade. A rapid generalization to other height stimuli and a chronic course of illness with at least moderate impairment were observed. A total of 22.5% of individuals with vHI experienced the intensity of panic attacks. The lifetime prevalence of acrophobia was 6.4% (women 8.6%, men 4.1%), and point prevalence was 2.0% (women 2.8%; men 1.1%). VHI and even more acrophobia were associated with high rates of comorbid anxious and depressive conditions. Migraine was both a significant predictor of later acrophobia and a significant consequence of previous acrophobia. VHI affects nearly a third of the general population; in more than 20% of these persons, vHI occasionally develops into panic attacks and in 6.4%, it escalates to acrophobia. Symptoms and degree of social impairment form a continuum of mild to seriously distressing conditions in susceptible subjects.

  1. Lobular breast cancer: Clinical, molecular and morphological characteristics.

    Science.gov (United States)

    Christgen, Matthias; Steinemann, Doris; Kühnle, Elna; Länger, Florian; Gluz, Oleg; Harbeck, Nadia; Kreipe, Hans

    2016-07-01

    Infiltrating lobular breast cancer (ILBC) is the most common special breast cancer subtype. This review provides a comprehensive description of ILBC characteristics, including epidemiology, clinical features, molecular genetics and histomorphology. Twenty detailed supplemental data tables guide through primary data of more than 200 original studies. Meta-analyses indicate that ILBC is at least twice as common in the Western world as it is in other geographic regions. ILBC is over-represented in so-called interval carcinomas and in primary metastatic breast cancer. ILBC is also associated higher age, higher pT stage and hormone receptor (ER/PR) positivity. Pathological complete response rates after neoadjuvant chemotherapy are low, ranging between 0% and 11%. Positive resection margins after breast-conserving surgery are comparatively frequent and 17% to 65% of patients undergo a second surgical intervention. Depending on the morphological stringency in the diagnosis of ILBC, lack of E-cadherin expression is observed in 55% to 100% of cases. CDH1/E-cadherin mutation detection rates vary between 12% and 83%. Various additional molecular factors, including PIK3CA, TP53, FOXA1, FGFR1, ZNF703 and BCAR4, have been implicated in ILBC or progression of lobular carcinoma in situ (LCIS) to invasive cancer and are discussed in detail. Eight instructive figure plates recapitulate the histomorphology of ILBC and its variants. Furthermore, we draw attention to rarely addressed histological details, such as two-sided nuclear compression and fat-avoiding growth at the invasion front. Last but not least, we discuss future translational research directions and emphasize the concept of synthetic lethality, which promises new options for targeted ILBC therapy.

  2. Severe acute pancreatitis in the elderly: Etiology and clinical characteristics

    Institute of Scientific and Technical Information of China (English)

    Ming-Jun Xin; Hong Chen; Bin Luo; Jia-Bang Sun

    2008-01-01

    AIM: To investigate the etiology and clinical characteristics of severe acute pancreatitis (SAP) in elderly patients (≥60 years of age).METHODS: We reviewed retrospectively all the SAP cases treated in Xuanwu Hospital in Beijing between 2000 and 2007.RESULTS: In 169 patients with SAP, 94 were elderly and 16 died.Biliary and idiopathic etiologies were the first two causes that accounted for over 90% of SAP in the elderly.Biliary, hyperlipemic and alcoholic etiologies were the first three causes in the young.The proportion of comorbidity of cholelithiasis, biliary infection, hypertension and coronary heart disease in the aged was significantly higher than that in their young partners.The scores of APACHE Ⅱ and Ranson were also significantly higher in the elderly except the CT score.Organ failures were more common in the elderly, but the local pancreatic complications were not different between the two groups.Mortality of the aged was correlated with the severity of SAP, multiple co-morbidity and incidence of multiple organ dysfunction syndrome (NODS).NODS was the main cause of death.CONCLUSION: The etiology of SAP in the elderly is quite different from that in the young.Biliary and unknown factors are main causes in the aged.The elderly are subject to major organ failures but there is no difference in the occurrence of local pancreatic complications between the elderly and the young.It is crucial to monitor and improve the functions of major organs so as to prevent MODS in the aged with SAP.

  3. The clinical characteristics of 169 cases of severe fever with thrombocytopenia syndrome

    Institute of Scientific and Technical Information of China (English)

    崔宁

    2012-01-01

    Objective To analyze the clinical characteristics of severe fever with thrombocytopenia syndrome(SFTS) so as to improve the recognition of the emerging infectious disease. Methods A retrospective analysis was performed upon clinical manifestations,laboratory test results and

  4. Interhemispheric functional connectivity and its relationships with clinical characteristics in major depressive disorder: a resting state fMRI study.

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    Li Wang

    Full Text Available BACKGROUND: Abnormalities in large-scale, structural and functional brain connectivity have been increasingly reported in patients with major depressive disorder (MDD. However, MDD-related alterations in functional interaction between the cerebral hemispheres are still not well understood. Resting state fMRI, which reveals spontaneous neural fluctuations in blood oxygen level dependent signals, provides a means to detect interhemispheric functional coherence. We examined the resting state functional connectivity (RSFC between the two hemispheres and its relationships with clinical characteristics in MDD patients using a recently proposed measurement named "voxel-mirrored homotopic connectivity (VMHC". METHODOLOGY/PRINCIPAL FINDINGS: We compared the interhemispheric RSFC, computed using the VMHC approach, of seventeen first-episode drug-naive patients with MDD and seventeen healthy controls. Compared to the controls, MDD patients showed significant VMHC decreases in the medial orbitofrontal gyrus, parahippocampal gyrus, fusiform gyrus, and occipital regions including the middle occipital gyrus and cuneus. In MDD patients, a negative correlation was found between VMHC of the fusiform gyrus and illness duration. Moreover, there were several regions whose VMHC showed significant negative correlations with the severity of cognitive disturbance, including the prefrontal regions, such as middle and inferior frontal gyri, and two regions in the cereballar crus. CONCLUSIONS/SIGNIFICANCE: These findings suggest that the functional coordination between homotopic brain regions is impaired in MDD patients, thereby providing new evidence supporting the interhemispheric connectivity deficits of MDD. The significant correlations between the VMHC and clinical characteristics in MDD patients suggest potential clinical implication of VMHC measures for MDD. Interhemispheric RSFC may serve as a useful screening method for evaluating MDD where neural connectivity is

  5. Pathophysiology of nasal congestion

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    Robert M Naclerio

    2010-02-01

    Full Text Available Robert M Naclerio1, Claus Bachert2, James N Baraniuk31University of Chicago, Department of Surgery, Section of Otolaryngology – Head and Neck Surgery, Chicago, Illinois, USA; 2University of Ghent, Ghent, Belgium; 3Georgetown University, Washington, DC, USAAbstract: Nasal congestion is a common symptom in rhinitis (both allergic and nonallergic, rhinosinusitis and nasal polyposis. Congestion can also be caused by physical obstruction of nasal passages and/or modulation of sensory perception. Mucosal inflammation underlies many of the specific and interrelated factors that contribute to nasal congestion, as well as other symptoms of both allergic rhinitis and rhinosinusitis. A wide range of biologically active agents (eg, histamine, tumor necrosis factor-α, interleukins, cell adhesion molecules and cell types contribute to inflammation, which can manifest as venous engorgement, increased nasal secretions and tissue swelling/edema, ultimately leading to impaired airflow and the sensation of nasal congestion. Inflammation-induced changes in the properties of sensory afferents (eg, expression of peptides and receptors that innervate the nose can also contribute to altered sensory perception, which may result in a subjective feeling of congestion. Increased understanding of the mechanisms underlying inflammation can facilitate improved treatment selection and the development of new therapies for congestion.Keywords: allergic rhinitis, congestion, obstruction, pathophysiology, rhinosinusitis

  6. Genital ulcers in women: clinical, microbiologic and histopathologic characteristics.

    Science.gov (United States)

    Gomes, Christiane Maria Moreira; Giraldo, Paulo César; Gomes, Francis de Assis Moraes; Amaral, Rose; Passos, Mauro Romero Leal; Gonçalves, Ana Katherine da Silveira

    2007-04-01

    Female genital ulcer is a disease that affects a large number of women, and its etiologic diagnosis can be difficult. The disease may increase the risk of acquiring HIV. Genital ulcer may be present in sexually transmitted diseases (STD)--syphilis, chancroid, genital herpes, donovanosis, lymphogranuloma venereum; and other non-STD disorders (NSTD)--Behçet's syndrome, pemphigus, Crohn's disease, erosive lichen planus and others. This study evaluated the clinical-histopathologic-microbiologic characteristics of female genital ulcers. A cross-sectional descriptive prospective study was conducted during a six-month period to investigate the first 53 women without a definitive diagnosis, seeking medical care for genital ulcers at a genital infections outpatient facility in a university hospital. A detailed and specific history was taken, followed by a dermatologic and gynecologic examination. In addition to collecting material from the lesions for microbiologic study, a biopsy of the ulcer was performed for histopathologic investigation. The average age of the patients was 32.7 years, 56.6% had junior high school education and higher education. The most frequent etiology was herpetic lesion, followed by auto-immune ulcers. At the time of their first consultation, around 60% of the women were using inadequate medication that was inconsistent with the final diagnosis. Histologic diagnosis was conclusive in only 26.4% of the patients (14/53). Cure was obtained in 99% of the cases after proper therapy. The female genital ulcers studied were equally distributed between sexually transmitted and non-sexually transmitted causes. Herpes was the most frequent type of genital ulcer, affecting women indiscriminately, mostly between the ages of 20 and 40 years. The etiologic diagnosis of herpetic ulcers is difficult to make even when various diagnostic methods are applied. It is imperative that NSTD should be included in the differential diagnoses of female genital ulcers. The

  7. Genital ulcers in women: clinical, microbiologic and histopathologic characteristics

    Directory of Open Access Journals (Sweden)

    Christiane Maria Moreira Gomes

    2007-04-01

    Full Text Available Female genital ulcer is a disease that affects a large number of women, and its etiologic diagnosis can be difficult. The disease may increase the risk of acquiring HIV. Genital ulcer may be present in sexually transmitted diseases (STD - syphilis, chancroid, genital herpes, donovanosis, lymphogranuloma venereum and other non-STD disorders (NSTD - Behçet's syndrome, pemphigus, Crohn's disease, erosive lichen planus and others. This study evaluated the clinical-histopathologic-microbiologic characteristics of female genital ulcers. A cross-sectional descriptive prospective study was conducted during a six-month period to investigate the first 53 women without a definitive diagnosis, seeking medical care for genital ulcers at a genital infections outpatient facility in a university hospital. A detailed and specific history was taken, followed by a dermatologic and gynecologic examination. In addition to collecting material from the lesions for microbiologic study, a biopsy of the ulcer was performed for histopathologic investigation. The average age of the patients was 32.7 years, 56.6% had junior high school education and higher education. The most frequent etiology was herpetic lesion, followed by auto-immune ulcers. At the time of their first consultation, around 60% of the women were using inadequate medication that was inconsistent with the final diagnosis. Histologic diagnosis was conclusive in only 26.4% of the patients (14/53. Cure was obtained in 99% of the cases after proper therapy. The female genital ulcers studied were equally distributed between sexually transmitted and non-sexually transmitted causes. Herpes was the most frequent type of genital ulcer, affecting women indiscriminately, mostly between the ages of 20 and 40 years. The etiologic diagnosis of herpetic ulcers is difficult to make even when various diagnostic methods are applied. It is imperative that NSTD should be included in the differential diagnoses of female

  8. Clinical characteristics of 42 patients with cardiac amyloidosis

    Institute of Scientific and Technical Information of China (English)

    黄雨晴

    2014-01-01

    Objective To characterize the clinical features of patients with cardiac amyloidosis(CA).Methods Totally42 patients with CA admitted to Guangdong General Hospital since 2008 were included and retrospectively analyzed in the present study.CA was confirmed by abdomen and endocardium biopsy examination.Clinical manifestations,electrocardiogram and echocardiography were collected for the evaluation.Results Several clinic features are common in CA.In the present study,37 cases

  9. Clinical characteristics,prognosis and indicators of glucose metabolism of liver cirrhosis complicated with diabetes

    Institute of Scientific and Technical Information of China (English)

    吴娟

    2013-01-01

    Objective To investigate clinical characteristics and outcome of patients with liver cirrhosis complicated with diabetes,and to explore the differences of clinical characteristics and prognosis between hepatogenous diabetes (HD) and type 2 diabetes mellitus (T2DM) .Methods From November 2010 to April 2012,246 patients with liver cirrhosis were collected and divided into liver cir-

  10. Trigeminal neuralgia--a prospective systematic study of clinical characteristics in 158 patients

    DEFF Research Database (Denmark)

    Maarbjerg, Stine; Gozalov, Aydin; Olesen, Jes

    2014-01-01

    OBJECTIVE: To prospectively describe the clinical characteristics of classical trigeminal neuralgia (TN) in a standardized manner. BACKGROUND: TN is a rare disease and most clinicians only see a few patients. There is a lack of prospective systematic studies of the clinical characteristics of TN...

  11. Pathophysiology of Resistant Hypertension: The Role of Sympathetic Nervous System

    Directory of Open Access Journals (Sweden)

    Costas Tsioufis

    2011-01-01

    Full Text Available Resistant hypertension (RH is a powerful risk factor for cardiovascular morbidity and mortality. Among the characteristics of patients with RH, obesity, obstructive sleep apnea, and aldosterone excess are covering a great area of the mosaic of RH phenotype. Increased sympathetic nervous system (SNS activity is present in all these underlying conditions, supporting its crucial role in the pathophysiology of antihypertensive treatment resistance. Current clinical and experimental knowledge points towards an impact of several factors on SNS activation, namely, insulin resistance, adipokines, endothelial dysfunction, cyclic intermittent hypoxaemia, aldosterone effects on central nervous system, chemoreceptors, and baroreceptors dysregulation. The further investigation and understanding of the mechanisms leading to SNS activation could reveal novel therapeutic targets and expand our treatment options in the challenging management of RH.

  12. Developing clinically successful biomedical devices by understanding the pathophysiology of the target tissue: insights from over 25 years at the microscope

    Science.gov (United States)

    Thomsen, Sharon L.; Coad, James E.

    2007-02-01

    Volumetric conductive-convective heat sources, microwave and radiofrequency energy sources, high intensity focused ultrasound (HIFU), laser irradiation and other non-ionizing irradiation sources can be used to generate hyperthermic tissue injury in a variety of clinical settings with therapeutic temperature gradients ranging from 40 to over 90°C. On the opposite side, cryotherapy can be used to freeze tissues with negative therapeutic temperature gradients. The development of a successful thermal therapy using any one of these devices requires a precise understanding of the desired clinical end point in terms of 1) diagnosis vs. therapy, 2) cure vs. palliative intent, 3) dysfunctional vs. malignant tissue and 4) long-term monitoring issues. The effects of a specific thermal exposure depend on the architecture of the heat source and overall thermal history. During initial treatment before heat generation or cooling becomes dominant, tissue interactions with the delivered treatment may affect the geometry of the treatment effect and body's healing response. These two parameters are also affected by tissue anatomy, blood supply and protein vs. lipid content. The thermal lesion and final clinical outcome represent the sum of direct primary and secondary short and long term delayed injury. The latter occurs primarily from host responses producing ischemia, inflammation and wound healing followed by possible regeneration and/or scar formation. Once the thermal insult has been deployed, the resulting lesions can be broadly divided into two major zones: 1) a complete tissue ablation with lethal tissue injury closer to the device and 2) a peripheral transition zone of partial injury. Hyperthermic complete ablation zones can have two sub-regions: 1) thermal fixation from direct denaturation of cellular and tissue components and 2) coagulative necrosis due to direct injury and delayed secondary host responses. With a variety of special techniques, direct cellular injury can

  13. Clinical Characteristics of the Causes of Plantar Heel Pain

    OpenAIRE

    Yi, Tae Im; Lee, Ga Eun; Seo, In Seok; Huh, Won Seok; Yoon, Tae Hee; Kim, Bo Ra

    2011-01-01

    Objective The objectives of this study were to investigate the causes of plantar heel pain and find differences in the clinical features of plantar fasciitis (PF) and fat pad atrophy (FPA), which are common causes of plantar heel pain, for use in differential diagnosis. Method This retrospective study analyzed the medical records of 250 patients with plantar heel pain at the Foot Clinic of Rehabilitation Medicine at Bundang Jesaeng General Hospital from January to September, 2008. Results The...

  14. Analysis of clinical characteristics of 950 cases of cervical cancer

    Directory of Open Access Journals (Sweden)

    Shu-li ZHU

    2015-04-01

    Full Text Available Objective To discuss the clinical features of the patients suffering from cervical cancer who visited Daping Hospital affiliated to Third Military Medical University in recent 10 years. Methods The clinical data of the patients who were pathologically diagnosed as invasive cervical cancer in Daping Hospital of TMMU from Jan. 2004 to Dec. 2013 were retrospectively analyzed. They were divided into different age groups and analyzed according to age, clinical features, pathological type, and surgical approach. Results Clinical data of 950 patients with invasive cervical cancer were reviewed in this study. The mean age of the patients was 46.9 years. The clinical features, pathological type, and surgical approaches were different in different age groups. Analysis of the age structure of the patients, the onset age of cervical cancer seemed to increase year by year. Conclusion The clinical features of cervical cancer are diversity in different age, and the strategy for controlling its development should be varied according to age. DOI: 10.11855/j.issn.0577-7402.2015.03.09

  15. Comparing School and Clinical Psychology Internship Applicant Characteristics

    Science.gov (United States)

    Mahoney, Emery B.; Perfect, Michelle M.; Edwinson, Roxanne M.

    2015-01-01

    The ratio of internship applicants to internship positions listed in the online directory of the Association of Psychology Postdoctoral and Internship Centers (APPIC) is estimated at 1.23:1. In 2014a, approximately 14% of all students who participated in the match were not placed. Although the internship crisis impacts students in clinical,…

  16. Characteristics of leadership that influence clinical learning: a narrative review.

    Science.gov (United States)

    Walker, Rachel; Cooke, Marie; Henderson, Amanda; Creedy, Debra K

    2011-11-01

    Leadership has been consistently implied in fostering clinical learning. However there is a lack of clarity about the form leadership should take. Limited quantitative research indicated a narrative approach to review literature from a broad perspective. A framework to guide the synthesis was developed to ensure a rigorous review process. Preliminary reading and review of papers using search terms nursing and leadership and clinical learning and learning culture narrowed the inclusion criteria to 245 papers published between 2000 and 2010. Given the diversity of the papers' focus, aim and context, a refined screening process justified the inclusion of twenty-six papers in the review. A critical appraisal of these peer-reviewed quantitative, qualitative and commentary papers identified factors/elements integral to effective leadership. Across the literature leadership was discussed in relation to two broad themes: influence of leadership on organisational learning and development and; influence of leadership on undergraduate clinical education. The factors central to leadership emerged as transformative principles, the role of the nurse unit/ward manager, collaboration and relationship building and role-modelling. The review has raised some suggestions for future research aimed at examining the impact of a leadership capacity building intervention that supports clinical learning.

  17. Clinical characteristics and outcome of intracerebral hemorrhage in young adults

    NARCIS (Netherlands)

    Rutten-Jacobs, L.C.A.; Maaijwee, N.A.M.M.; Arntz, R.M.; Schoonderwaldt, H.C.; Dorresteijn, L.D.A.; Dijk, E.J. van; Leeuw, F.E. de

    2014-01-01

    Data on determinants of prognosis after intracerebral hemorrhage (ICH) in young adults are scarce. Our aim was to identify clinical determinants of prognosis after ICH in adults aged 18-50. We investigated 98 consecutive patients with an ICH, aged 18-50 years, admitted to our hospital between 1980 a

  18. Hemifacial spasm: clinical characteristics of 321 Indian patients.

    Science.gov (United States)

    Batla, Amit; Goyal, Chanchal; Shukla, Garima; Goyal, Vinay; Srivastava, Achal; Behari, Madhuri

    2012-08-01

    Hemifacial spasm (HFS) is a common neurological disorder characterized by involuntary tonic and clonic contractions of the muscles innervated by the facial nerve. We aimed to describe clinical features, common antecedents, triggers and relieving factors in patients with hemifacial spasm to study the correlation of hypertension and HFS, and to compare clinical features of primary and secondary cases of HFS. The data for the study were collected prospectively on a predesigned and pre-tested format at the first attendance in all consecutive HFS patients attending the movement disorders clinic of a tertiary teaching hospital in India. The demographic profile, HFS symptoms, antecedent illnesses and neurological examination were recorded and analyzed. Muscle power in individual muscles innervated by the facial nerve was tested carefully before botulinum toxin injection. Hemifacial spasm occurred in 7.14% (n = 582) of 8,151 cases registered at the movement disorders clinic from 1993 to 2010. Data of 321 patients were complete and were included in the study. Females constituted 49.22% (n = 158). The mean age the patients was 46.02 ± 11.82 years; ipsilateral ear clicking was observed in 22.74% cases. The most common aggravating factor was stress (44.86%), while the most common relieving factor was sleep (44.24%). Two hundred fifty-two patients (78.5%) had primary HFS. The severity of spasm correlated significantly with disease duration (p muscles (p < 0.001). We did not observe any correlation between HFS on the left side and hypertension, as has been reported earlier. This is one of the largest studies of HFS patients and the only one that prospectively assesses patients with HFS clinically on their first visit. Interesting observations of this study are lack of female preponderance, presence of clicking in the ipsilateral ear and facial weakness even prior to botulinum toxin injection.

  19. HIV-associated opportunistic CNS infections: pathophysiology, diagnosis and treatment.

    Science.gov (United States)

    Bowen, Lauren N; Smith, Bryan; Reich, Daniel; Quezado, Martha; Nath, Avindra

    2016-10-27

    Nearly 30 years after the advent of antiretroviral therapy (ART), CNS opportunistic infections remain a major cause of morbidity and mortality in HIV-positive individuals. Unknown HIV-positive disease status, antiretroviral drug resistance, poor drug compliance, and recreational drug abuse are factors that continue to influence the morbidity and mortality of infections. The clinical and radiographic pattern of CNS opportunistic infections is unique in the setting of HIV infection: opportunistic infections in HIV-positive patients often have characteristic clinical and radiological presentations that can differ from the presentation of opportunistic infections in immunocompetent patients and are often sufficient to establish the diagnosis. ART in the setting of these opportunistic infections can lead to a paradoxical worsening caused by an immune reconstitution inflammatory syndrome (IRIS). In this Review, we discuss several of the most common CNS opportunistic infections: cerebral toxoplasmosis, progressive multifocal leukoencephalopathy (PML), tuberculous meningitis, cryptococcal meningitis and cytomegalovirus infection, with an emphasis on clinical pearls, pathological findings, MRI findings and treatment. Moreover, we discuss the risk factors, pathophysiology and management of IRIS. We also summarize the challenges that remain in management of CNS opportunistic infections, which includes the lack of phase II and III clinical trials, absence of antimicrobials for infections such as PML, and controversy regarding the use of corticosteroids for treatment of IRIS.

  20. Quantitative Clinical Chemistry Proteomics (qCCP) using mass spectrometry: general characteristics and application.

    Science.gov (United States)

    Lehmann, Sylvain; Hoofnagle, Andrew; Hochstrasser, Denis; Brede, Cato; Glueckmann, Matthias; Cocho, José A; Ceglarek, Uta; Lenz, Christof; Vialaret, Jérôme; Scherl, Alexander; Hirtz, Christophe

    2013-05-01

    Proteomics studies typically aim to exhaustively detect peptides/proteins in a given biological sample. Over the past decade, the number of publications using proteomics methodologies has exploded. This was made possible due to the availability of high-quality genomic data and many technological advances in the fields of microfluidics and mass spectrometry. Proteomics in biomedical research was initially used in 'functional' studies for the identification of proteins involved in pathophysiological processes, complexes and networks. Improved sensitivity of instrumentation facilitated the analysis of even more complex sample types, including human biological fluids. It is at that point the field of clinical proteomics was born, and its fundamental aim was the discovery and (ideally) validation of biomarkers for the diagnosis, prognosis, or therapeutic monitoring of disease. Eventually, it was recognized that the technologies used in clinical proteomics studies [particularly liquid chromatography-tandem mass spectrometry (LC-MS/MS)] could represent an alternative to classical immunochemical assays. Prior to deploying MS in the measurement of peptides/proteins in the clinical laboratory, it seems likely that traditional proteomics workflows and data management systems will need to adapt to the clinical environment and meet in vitro diagnostic (IVD) regulatory constraints. This defines a new field, as reviewed in this article, that we have termed quantitative Clinical Chemistry Proteomics (qCCP).

  1. Trichomonas vaginalis: Diagnosis and Clinical Characteristics in Pregnancy

    OpenAIRE

    R. Phillip Heine; James A. McGregor; Elisa Patterson; Deborah Draper; Janice French; Ward Jones

    1994-01-01

    Objective: The objectives of this study were to 1) determine the prevalance and characterize the symptomatology of Trichomonas vaginalis (TV) infection in pregnant women on entry into prenatal care in an inner-city population; 2) compare conventional microscopic methods vs. culture techniques in diagnosing TV in both symptomatic and asymptomatic pregnant patients; and 3) correlate wet mount microscopic and microbiologic characteristics of varying manifestations of trichomoniasis. Methods: One...

  2. Requirements for the formal representation of pathophysiology mechanisms by clinicians.

    Science.gov (United States)

    de Bono, B; Helvensteijn, M; Kokash, N; Martorelli, I; Sarwar, D; Islam, S; Grenon, P; Hunter, P

    2016-04-01

    Knowledge of multiscale mechanisms in pathophysiology is the bedrock of clinical practice. If quantitative methods, predicting patient-specific behaviour of these pathophysiology mechanisms, are to be brought to bear on clinical decision-making, the Human Physiome community and Clinical community must share a common computational blueprint for pathophysiology mechanisms. A number of obstacles stand in the way of this sharing-not least the technical and operational challenges that must be overcome to ensure that (i) the explicit biological meanings of the Physiome's quantitative methods to represent mechanisms are open to articulation, verification and study by clinicians, and that (ii) clinicians are given the tools and training to explicitly express disease manifestations in direct contribution to modelling. To this end, the Physiome and Clinical communities must co-develop a common computational toolkit, based on this blueprint, to bridge the representation of knowledge of pathophysiology mechanisms (a) that is implicitly depicted in electronic health records and the literature, with (b) that found in mathematical models explicitly describing mechanisms. In particular, this paper makes use of a step-wise description of a specific disease mechanism as a means to elicit the requirements of representing pathophysiological meaning explicitly. The computational blueprint developed from these requirements addresses the Clinical community goals to (i) organize and manage healthcare resources in terms of relevant disease-related knowledge of mechanisms and (ii) train the next generation of physicians in the application of quantitative methods relevant to their research and practice.

  3. Abnormal echocardiography in patients with type 2 diabetes and relation to symptoms and clinical characteristics

    DEFF Research Database (Denmark)

    Jørgensen, Peter Godsk; Jensen, Magnus T; Mogelvang, Rasmus;

    2016-01-01

    OBJECTIVES: We aimed to determine the prevalence of echocardiographic abnormalities and their relation to clinical characteristics and cardiac symptoms in a large, contemporary cohort of patients with type 2 diabetes. RESULTS: A total of 1030 patients with type 2 diabetes participated...... with abnormal echocardiography along with dyspnoea and characteristic chest pain (p characteristics had sufficient sensitivity and specificity to accurately identify patients with abnormal echocardiography. CONCLUSION: Echocardiographic abnormalities...... are very common in outpatients with type 2 diabetes, but neither cardiac symptoms nor clinical characteristics are effective to identify patients with echocardiographic abnormalities....

  4. The clinical characteristics and MRI findings of acquired hepatocerebral degeneration

    Directory of Open Access Journals (Sweden)

    Xiao-yu DONG

    2015-03-01

    Full Text Available Acquired hepatocerebral degeneration (AHD is a rare chronic encephalopathy, and its etiology is usually correlated with cirrhosis and portal hypertension. In this study, clinical and radiological data of 326 patients with cirrhosis of the liver were retrospectively analyzed, among whom 11 patients were diagnosed as AHD, with the incidence of 3.37% . The most common neurological symptoms were static tremor (9 cases and ataxia (7 cases. Cerebral MRI showed T1WI hyperintensity in bilateral globus pallidus of all 11 patients. The lesions were also involved in bilateral thalamus (one case, tegment of midbrain (one case, bilateral posterior crus of internal capsule (one case and bilateral putamen (one case. Anti-Parkinson's disease drugs, such as levodopa and benserazide, had not presented satisfactory clinical effect. DOI: 10.3969/j.issn.1672-6731.2015.02.014

  5. [Minimal hepatic encephalopathy: characteristics, diagnosis and clinical implications].

    Science.gov (United States)

    Torre Delgadillo, Aldo; Guerrero-Hernández, Ignacio; Uribe, Misael

    2006-01-01

    The term minimal hepatic encephalopathy (MHE) refers to the subtle changes in cognitive function, electrophysiological parameters, cerebral neurochemical/neurotransmitter homeostasis, cerebral blood flow, metabolism, and fluid homeostasis that can be observed in patients with cirrhosis who have no clinical evidence of hepatic encephalopathy; the prevalence is as high as 84% in patients with hepatic cirrhosis. This cirrhosis complication is generally not perceived by physician, and diagnosis can only be made by neuropsychological tests and other especial measurements like evoked potentials and image studies like positron emission tomography. Diagnosis of minimal hepatic encephalopathy may have prognostic and therapeutic implications in cirrhotic patients. The present review pretends to explore the clinic, therapeutic, diagnosis and prognostic aspects of this complication.

  6. CLINICAL CHARACTERISTICS OF PATIENTS WITH CHRONIC ACQUIRED DEMYELINATING POLYNEUROPATHY

    Directory of Open Access Journals (Sweden)

    M. Ghabace

    2005-09-01

    Full Text Available Chronic acquired demyelinating neuropathy (CADP is heterogeneous ill both clinical and laboratory features. This study was performed to define the clinical. clccuodiagnostic and histological findings. course and response 10 therapy in patients with CADI'. Thirty patients (20 men and 10 women with CADI' were studied. Diagnostic criteria were based on clinical presentation. clcctrophysiolcgical studies. cerebrospinal fluid (CSF protein level and sural nerve biopsy findings. Response 10 treatment was assessed by changes in average muscle score (A:vlS. Twenty-one patients conformed to the diagnostic criteria of chronic inflammatory demyelinating polyneuropathy (Cf Dl" and 9 to distal acquired demyelinating symmetric neuropathy (DADS. The course was monophasic in Cl (23~/~-, relapsing in I0 (40(~/;1 and chronic progressive in 8 (30':••;,: 4( 13°•'( had ucutc presentation with subsequent progression or relapsing course. Motor nerve conduction velocity (i"--INCV of less than 70°,-( and greater than 70'~;(, of normal were seen in 18 (60'~'; and 12 (40{~-;1 patients. respectively. Conduction block was observed in 14 (47(~/o and CSF protein levels WCl"C elevaled in 19 patients (66':--;. Demyelination was reported in 61(;--( and 58% of the biopsies performed in patients with MNCV <: 70'~";l and> 70'}'( of normal. respectively. The association between "•lNCV and histologic findings was no! significant. Twenty-one patients were treated with intravenous immunoglobulin (lVlg. Fifteen patients  83(;-{1 with ClDP had significant improvement in AfvlS following the iuitial fVlg treatment (P n.ol. This study highlights the heterogeneity of clinical and laboratory findings in C:"IP and the importance of early treatment.

  7. Clinical characteristics of pulmonary embolism with concomitant pneumonia.

    Science.gov (United States)

    Cha, Seung-Ick; Choi, Keum-Ju; Shin, Kyung-Min; Lim, Jae-Kwang; Yoo, Seung-Soo; Lee, Jaehee; Lee, Shin-Yup; Kim, Chang-Ho; Park, Jae-Yong

    2016-04-01

    Although pneumonia is associated with an increased risk of venous thromboembolism, patients with pulmonary embolism and concomitant pneumonia are uncommon. The aim of the present study was to investigate the clinical features of pulmonary embolism with coexisting pneumonia. We retrospectively compared clinical, radiologic and laboratory parameters between patients with pulmonary embolism and concomitant pneumonia (pneumonia group) and those with unprovoked pulmonary embolism (unprovoked group), and then between the pneumonia group and those with pulmonary infarction (infarction group). Of 794 patients with pulmonary embolism, 36 (5%) had coexisting pneumonia and six (1%) had no provoking factor other than pneumonia. Stroke was significantly more common in the pneumonia group, than either the unprovoked group or the infarction group. In the pneumonia group, fever was significantly more common and serum C-reactive protein levels were significantly higher. By contrast, central pulmonary embolism and right ventricular dilation on computed tomography were significantly less frequent in the pneumonia group. In addition, an adverse outcome due to pulmonary embolism was less common in the pneumonia group than in either of the other two groups. The coexistence of pulmonary embolism and pneumonia is rarely encountered in clinical practice, especially without the presence of other factors that could provoke venous thromboembolism and is commonly associated with stroke. It is characterized by lower incidences of central pulmonary embolism and right ventricular dilation and by a lower rate of adverse outcomes due to pulmonary embolism itself.

  8. Clinical characteristics and presence of prolactinoma in women with hyperprolactinemia

    Directory of Open Access Journals (Sweden)

    López-Clavijo, Carlos Alberto

    2016-07-01

    Full Text Available Objectives: To clinically characterize women with hyperprolactinemia at InSer Pereira (Colombia and to determine the prevalence of prolactinoma. Methods: Retrospective description of 45 patients with hyperprolactinemia, and contrasted pituitary magnetic resonance (MR, between January 2008 and July 2013. Clinical manifestations, etiology, serum prolactin level, and MR findings were included. Results: The most frequent clinical manifestations were: infertility, galactorrhea, oligomenorrhea. There were 26 cases of prolactinoma (57.8 %, and 12 of idiopathic hyperprolactinemia (26.6 %. Out of the 26 prolactinomas, 23 were microadenomas (average size 5.1 mm; SD ± 3.2 mm. Average serum prolactin level was 74.05 ng/mL (SD ± 13.33 ng/mL. 78 % of patients with prolactinoma had serum prolactin level under 70.0 ng/mL. No significant association was found between serum prolactin level and the presence of prolactinoma. Conclusion: Prolactinomas are the main cause of hyperprolactinemia and they are found mostly with slight rise of serum prolactin. Pituitary MRI is recommended in patients with hyperprolactinemia, regardless of their prolactin level.

  9. Clinical and diagnostic characteristics of hyperprolactinemia in childhood and adolescence.

    Science.gov (United States)

    Catli, Gonul; Abaci, Ayhan; Bober, Ece; Büyükgebiz, Atilla

    2013-01-01

    Pituitary adenoma is the most common cause of hyperprolactinemia, which is a rare endocrine disorder encountered in pediatric patient care. Epidemiological and clinical information about hyperprolactinemia in childhood and adolescence is limited. Clinical signs of hyperprolactinemia are very heterogeneous. In girls, disturbances in menstrual function and galactorrhea may be seen, whereas in boys, headache, visual disturbances, delayed pubertal development and hypogonadism are often present. Owing to the ease of ordering a serum prolactin measurement, an evidence-based, cost-effective approach to the management of this endocrine disorder is required. Before a diagnosis of hyperprolactinemia is made, drug use, renal insufficiency, hypothyroidism, and parasellar tumors should be excluded. The main objectives of treatment are normalization of prolactin level, adenoma shrinkage, and recovery from clinical signs related to hyperprolactinemia. In patients with microadenoma, invasive or non-invasive macroadenoma, and even in patients with visual field defects, dopamine agonists are the first-line treatment. Surgical treatment is indicated in patients who are unresponsive or intolerant to medical treatment or who have persistent neurological signs. Radiotherapy should be considered as a supportive treatment for patients in whom surgery fails or medical response is not achieved.

  10. Paranasal sinuses malignancies: A 12-year review of clinical characteristics

    Science.gov (United States)

    Sarafraz, Alireza; Chamani, Mojtaba; Derakhshandeh, Hossein

    2016-01-01

    Background Inadequate epidemiologic investigations of the paranasal sinuses malignancies prompted this retrospective study with special emphasis on a major group of 111 tumors. Material and Methods Clinical records of 111 patients with histologically confirmed malignant tumors of the paranasal sinuses were investigated retrospectively from April 2000 to January 2012. Collection of data included demographic information, clinical manifestations, treatment plans, and histopathology of the tumor. Results There were 69 (62.16%) male and 42 (37.83%) female patients (male-to-female ratio of 1.6:1), with a median age of 49±12.2 years (range 21 to 88 years). A high level of occurrence was noticed in the fifth (26.3%) decade of life. The most frequent histological types were squamous cell carcinoma (43.5%) and adenoid cystic carcinoma (19%). Among clinical manifestations, nasal obstruction was the most frequent followed by diplopia, and facial swelling. Fifty three patients (47.74%) were treated with combined approach of surgery and radiation therapy. Conclusions Paranasal sinuses malignancies are rare conditions with nonspecific symptoms which make early diagnosis of the lesions more challenging. The optimal therapeutic protocol for patients suffering from these tumors is still a somewhat controversial entity and requires further studies. Key words:Paranasal sinuses, malignancy, surgery,radiotherapy. PMID:27475693

  11. [Zhu Lian's characteristics and experiences in clinical practice of acupuncture and moxibustion].

    Science.gov (United States)

    Wei, Li fu; Pan, Xiaoria; Liu, Bing; Yue, Jin; Zhang, Lijian

    2015-01-01

    This paper aims at discussing the clinical characteristics and experiences of ZHU Lian, the renowned contemporary acupuncture master from the following three aspects: "characteristics of clinical manipulations and techniques", "thoughts on diagnosis and treatment" and "examples of clinical cases". The study has shown that ZHU Lian invented the slow insertion technique by rotating needle and the embedding needle technique, improved moxibustion technique with moxa roll and proposed the three keys on the treatment with acupuncture and moxibustion, as well as discovered new acupoints for treatment. The pioneering and distinguished achievements she con tributed play the great demonstrating and driving role in the development of clinical study and practice of acupuncture and moxibustion.

  12. Site Characteristics Influencing the Translation of Clinical Research Into Clinical Practice

    DEFF Research Database (Denmark)

    Smed, Marie; Getz, Kenneth A.

    2014-01-01

    Investigative sites participating in clinical trials play an instrumental role in aiding market adoption. Site experiences in clinical research help physician investigators and research professionals gain familiarity with and exposure to investigational treatments. This knowledge may be passed on...

  13. Clinical disease presentation and ECG characteristics of LMNA mutation carriers

    Science.gov (United States)

    Ollila, Laura; Nikus, Kjell; Holmström, Miia; Jalanko, Mikko; Jurkko, Raija; Kaartinen, Maija; Koskenvuo, Juha; Kuusisto, Johanna; Kärkkäinen, Satu; Palojoki, Eeva; Reissell, Eeva; Piirilä, Päivi; Heliö, Tiina

    2017-01-01

    Objective Mutations in the LMNA gene encoding lamins A and C of the nuclear lamina are a frequent cause of cardiomyopathy accounting for 5–8% of familial dilated cardiomyopathy (DCM). Our aim was to study disease onset, presentation and progression among LMNA mutation carriers. Methods Clinical follow-up data from 27 LMNA mutation carriers and 78 patients with idiopathic DCM without an LMNA mutation were collected. In addition, ECG data were collected and analysed systematically from 20 healthy controls. Results Kaplan-Meier analysis revealed no difference in event-free survival (death, heart transplant, resuscitation and appropriate implantable cardioverter-defibrillator therapy included as events) between LMNA mutation carriers and DCM controls (p=0.5). LMNA mutation carriers presented with atrial fibrillation at a younger age than the DCM controls (47 vs 57 years, p=0.003). Male LMNA mutation carriers presented with clinical manifestations roughly a decade earlier than females. In close follow-up non-sustained ventricular tachycardia was detected in 78% of LMNA mutation carriers. ECG signs of septal remodelling were present in 81% of the LMNA mutation carriers, 21% of the DCM controls and none of the healthy controls giving a high sensitivity and specificity for the standard ECG in distinguishing LMNA mutation carriers from patients with DCM and healthy controls. Conclusions Male LMNA mutation carriers present clinical manifestations at a younger age than females. ECG septal remodelling appears to distinguish LMNA mutation carriers from healthy controls and patients with DCM without LMNA mutations. PMID:28123761

  14. Clinical characteristics and treatment of neurofibroma of the choroid

    Institute of Scientific and Technical Information of China (English)

    WEI Wen-bin; JIE Ying; MO Jing; LI Bin

    2012-01-01

    Background Neurofibroma is a kind of benign neoplasm that derives from nervous tissues.Though this tumor is the most common types in the peripheral nervous system,it is rarely seen in the choroid and easy to be misdiagnosed of choroidal melanoma.The aim of this study was to review the clinical features of neurofibroma of the choroid in the Chinese race.Methods A retrospective case series design was used.Two male and one female patients diagnosed with choroidal neurofibroma in Beijing Tongren Eye Center were included in this study.The clinical histories were abstracted from the patients' medical records.Routine eye examinations including visual acuity,intraocular pressure,slit lamp and ophthalmoscope were performed.Auxiliary examinations included fluorescein fundus angiography (FFA),AB-ultrasound scan,color doppler imaging (CDI),and magnetic resonance imaging (MRI).Local resection of the tumors was performed and the specimens underwent pathological examinations.Results The tumors were of yellow-pink color with pigmentation on the surface.CDI showed arterial blood signals in the tumor and MRI showed high-intensity in the T1-weighted image and a slightly increased intensity in the T2-weighted image.FFA and indocyaninegreen angiography demonstrated the tumors were of hypofluorescence at early stage and hyperfluorescence with prominent leakage at late stage.The pathological examination confirmed the tumors were choroidal neurofibroma.After 5-10-year follow-up,there were no recurrences of the tumors and the retinas were well attached.Conclusions Choroidal neurofibroma is difficult to be diagnosed clinically and pathological confirmation is important.These tumors can be managed conservatively by local resection.

  15. Association between neurovascular contact and clinical characteristics in classical trigeminal neuralgia

    DEFF Research Database (Denmark)

    Maarbjerg, Stine; Wolfram, Frauke; Gozalov, Aydin

    2015-01-01

    BACKGROUND: Previous studies demonstrated that a severe neurovascular contact (NVC) causing displacement or atrophy of the trigeminal nerve is highly associated with classical trigeminal neuralgia (TN). There are no studies describing the association between the clinical characteristics of TN...

  16. Ideological and political education for clinical graduates on the basis of medical characteristics

    Directory of Open Access Journals (Sweden)

    Yun-chuan JING

    2014-09-01

    Full Text Available Along with the reform of medical system, medical education in China is also undergoing great changes. Due to the special characteristics of medical education, it differs from other educational characteristics. It carries with the characteristics of clinical practice on the basis distributed learning, physical and mental development along with ages, enrollment expansion and medical requirement, and standardization training for resident doctors. So, ideological and political education of clinical graduates showed many new characteristics. First, medical ethics education is the basic step, combined with the related disciplines of medical humanity connotation. Second, flexible and diversified form of ideological and political education on the basis of medical work is necessary. Third, establish a system of ideological and political education for clinical graduates, to build up new education concept, and to develop ideological and political education activities for clinical graduates in depth.

  17. Clinical characteristics of patients with gastroesophageal reflux disease in several centers of Northwest China

    Institute of Scientific and Technical Information of China (English)

    高麦仓

    2013-01-01

    Objective To investigate the clinical characteristics of gastroesophageal reflux disease (GERD) in several endoscopy centers of Northwest China.Methods From September 2008 to September 2009,a questionnaire survey was carried out in the endoscopy centers of four hospitals

  18. Clinical characteristics and genetic analysis of three pediatric patients with idiopathic restrictive cardiomyopathy

    Institute of Scientific and Technical Information of China (English)

    杨世伟

    2013-01-01

    Objective Restrictive cardiomyopathy(RCM) is rare in children,and little is known about the molecular basis of RCM.The aim of this study was to investigate the clinical and myopathological characteristics and to detect

  19. Zoonotic bacterial meningitis in adults: clinical characteristics, etiology, treatment and outcome

    NARCIS (Netherlands)

    van Samkar, A.

    2016-01-01

    In this thesis, we describe the clinical characteristics, etiology, treatment and outcome of zoonotic bacterial meningitis. Each chapter describes meningitis patients infected by a specific zoonotic pathogen, such as Streptococcus equi, Streptococcuis suis, Capnocytophaga canimorsus, Campylobacter f

  20. Clinical management of behavioral characteristics of Prader–Willi syndrome

    Directory of Open Access Journals (Sweden)

    Alan Y Ho

    2010-04-01

    Full Text Available Alan Y Ho, Anastasia DimitropoulosDepartment of Psychology, Case Western Reserve University, Cleveland, OH, USAAbstract: Prader–Willi syndrome (PWS is a complex neurodevelopmental disorder caused by an abnormality on the long arm of chromosome 15 (q11–q13 that results in a host of phenotypic characteristics, dominated primarily by hyperphagia and insatiable appetite. Characteristic behavioral disturbances in PWS include excessive interest in food, skin picking, difficulty with a change in routine, temper tantrums, obsessive and compulsive behaviors, and mood fluctuations. Individuals with PWS typically have intellectual disabilities (borderline to mild/moderate mental retardation and exhibit a higher overall behavior disturbance compared to individuals with similar intellectual disability. Due to its multisystem disorder, family members, caregivers, physicians, dieticians, and speech-language pathologists all play an important role in the management and treatment of symptoms in an individual with PWS. This article reviews current research on behavior and cognition in PWS and discusses management guidelines for this disorder.Keywords: Prader–Willi syndrome; neurodevelopment, hyperphagia, disability

  1. Clinical characteristics of polycystic ovary syndrome in Indian women

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    Sunita J Ramanand

    2013-01-01

    Full Text Available Background: Polycystic ovary syndrome (PCOS is common diagnosis in women presenting with infertility. All the dimensions of PCOS have not been completely explored. Many studies have tried to characterize the exact presentation of the disease. In this study we studied clinical features of PCOS in Indian women to characterize different phenotypes of this syndrome. Prevalence of acanthosis nigricans (AN as surrogate marker of insulin resistance, obesity, hirsutism and hypothyroidism in PCOS women have been simultaneously studied. Materials and Methods: Present work is a non comparative cross-sectional open label study carried out over a period of 18 months in an endocrinology hospital in western Maharashtra, India. Results and Conclusion: Authors conclude that PCOS occurs both in obese and non-obese women; AN and hirsutism occur in equal proportion of patients. AN is correlated with obesity. Hormonal dysfunctions in PCOS manifested together or independently. PCOS women can be sub grouped based on clinical features suggestive of endocrinological malfunctions and can be investigated accordingly for selection of appropriate treatment modalities.

  2. Clinical and diagnostic characteristics of patients with suspected polyneuropathy

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    Mikhailova Е.V.

    2013-09-01

    Full Text Available Objective: to study the causes and clinical manifestations of disease in children referred for hospitalization in children infectious diseases hospital in Saratov with a diagnosis of «acute flaccid paralysis». Material and methods: 157 children with the diagnosis on admission of the guide «acute flaccid paralysis». Conducted clinical examination and laboratory tests included a general analysis of cerebrospinal fluid, urine, virological examination of nasal swabs and faeces, with the definition of a serological ELISA method and RPHA immunoglobulins to influenza, rubella, and enterovirus, immunological study of blood, cerebrospinal fluid PCR, electromyography of the affected limbs. Results. 77 patients (49% with the disease associated with the violation of the musculoskeletal system were registered. In the other cases revealed polyneuropathy was not of poliovirus etiology. Etiological nature of the disease could be explained by 54% of patients. In 37 (46% patients the diagnosis was formulated in accordance with the severity of paralysis. One child was diagnosed with a vaccine-associated poliomyelitis. Conclusion. The diagnosis of «acute flaccid paralysis» used as administered requires a detailed interpretation in a hospital.

  3. Clinical and laboratory characteristics of women with uterine leiomiyoma

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    Özgür ÖZKUL

    2009-06-01

    Full Text Available The aim of this study was to compare clinical and laboratory findings of women with or without uterine leiomyoma.Study group consisted of 82 women with uterine leiomyoma and the control group comprised 42 healthy women. Women’s age, gravity, parity, blood groups, pattern of menstrual cycles, complaints at presentation, fertility, ultrasonographical findings, surgical operations and thyroid function tests were evaluated.There were no significant differences in blood group distribution, gravity, parity and thyroid function test results between the patients and the control subjects (P>0.05. A significant difference was found in the complaints at presentation between two groups (P<0.001. Mentrual cycles irregularity was more frequently found in the patients compared with the controls (57.3% vs. 42.9%, respectively, P=0.009. Although no infertile woman was found in the control group, 8.5% of patients were found to have infertility. The sensitivity of ultrasonography was found to be 97.6%. Except for the existence of higher infertility rate and the menstrual cycles irregularities, no significant difference was found in the clinical and laboratory findings between women with or without uterine leiomyoma. Therefore, physical examination and imaging methods are remained as the most important diagnostic tools for uterine leiomyoma.

  4. [Clinical-electroneuromyographical characteristics of facial nerve paralysis in children].

    Science.gov (United States)

    Gribova, N P; Galitskaia, O S

    2009-01-01

    A clinical-electroneuromyographical study of 40 children (32 (80%) of them aged from 12 to 17 years, mean age 13,9+/-1,8 years, and 8 (20%) - from 1 to 8 years, mean age 4,4+/-2,1 years) were studied in the acute period of facial nerve paralysis (FNP). Six (15%) children had FNP in the anamnesis. Among precipitating factors were the cold exposure the day before disease onset (20 (50%) patients), symptoms of flu (13 (32,5%) patients) and psycho-emotional tension (3 (7,5%) patients). No precipitation was noted in 4 (10%) children. The degree of muscle paresis was 81,9+/-7% that corresponded to clinical stages III-IV according to K. Rosler. An electroneuromyographical analysis of motor ortho- and antidromic response to the facial nerve stimulation on the side of paresis and on the contralateral side in patients and controls revealed the presence of proximal axon- and myelinopathy of facial nerve with the involvement of its own motorneurons and brain stem interneurons. The maintenance of wink reflex and F-wave blocks in the period over 3 weeks are prognostically unfavorable factors for restoration of mimic muscle function in the early stage of disease.

  5. Clinical characteristics and antimicrobial susceptibility of Bacillus cereus blood stream infections

    OpenAIRE

    Ikeda, Mahoko; Yagihara,Yuka; Tatsuno, Keita; Okazaki, Mitsuhiro; Okugawa, Shu; Moriya, Kyoji

    2015-01-01

    Background Bacillus cereus is one of the pathogens causing nosocomial bloodstream infections (BSIs). However, few reports have documented the antimicrobial susceptibility and clinical characteristics of Bacillus cereus BSI and the importance of empirical therapy. The aim of this study was to investigate the clinical characteristics and antimicrobial susceptibility of B. cereus isolates from patients with BSI and to analyze the impact of appropriate empirical therapy on the outcome of patients...

  6. Monosomal karyotype among adult acute myeloid leukemia: clinical characteristic and prognostic analysis

    Institute of Scientific and Technical Information of China (English)

    冯茹

    2014-01-01

    Objective To explore the clinical characteristics and prognostic value of monosomal karyotype(MK)patients in adult acute myeloid leukemia(AML).Methods We retrospectively studied 45 patients of MK+in newly-diagnosed adult AML in our center from Oct 2000 to Dec2012.Clinical characteristics,cytogenetic data and prognostic features were analyzed in the cohort of MK+patients.Results MK was found in 45 patients(19.0%)

  7. Clinical, Molecular, and Genetic Characteristics of PAPA Syndrome: A Review.

    Science.gov (United States)

    Smith, Elisabeth J; Allantaz, Florence; Bennett, Lynda; Zhang, Dongping; Gao, Xiaochong; Wood, Geryl; Kastner, Daniel L; Punaro, Marilynn; Aksentijevich, Ivona; Pascual, Virginia; Wise, Carol A

    2010-11-01

    PAPA syndrome (Pyogenic Arthritis, Pyoderma gangrenosum, and Acne) is an autosomal dominant, hereditary auto-inflammatory disease arising from mutations in the PSTPIP1/CD2BP1 gene on chromosome 15q. These mutations produce a hyper-phosphorylated PSTPIP1 protein and alter its participation in activation of the "inflammasome" involved in interleukin-1 (IL-1β) production. Overproduction of IL-1β is a clear molecular feature of PAPA syndrome. Ongoing research is implicating other biochemical pathways that may be relevant to the distinct pyogenic inflammation of the skin and joints characteristic of this disease. This review summarizes the recent and rapidly accumulating knowledge on these molecular aspects of PAPA syndrome and related disorders.

  8. Arterial stiffness: pathophysiology and clinical impact.

    Science.gov (United States)

    London, Gérard M; Marchais, Sylvain J; Guerin, Alain P; Pannier, Bruno

    2004-01-01

    The ill effects of hypertension are usually attributed to a reduction in the caliber or the number of arterioles, resulting in an increase in total peripheral resistance (TPR). This definition does not take into account the fact that BP is a cyclic phenomenon with systolic and diastolic BP being the limits of these oscillations. The appropriate term to define the arterial factor(s) opposing LV ejection is aortic input impedance which depends on TPR, arterial distensibility (D), and wave reflections (WR). D defines the capacitive properties of arterial stiffness, whose role is to dampen pressure and flow oscillations and to transform pulsatile flow and pressure in arteries into a steady flow and pressure in peripheral tissues. Stiffness is the reciprocal value of D. These parameters are BP dependent, and arteries become stiffer at high pressure. In to D which provides information about the elasticity> of artery as a hollow structure, the elastic incremental modulus (Einc) characterizes the properties of the arterial wall biomaterials, independently of vessel geometry. As an alternative, arterial D can be evaluated by measuring the pulse wave velocity (PWV) which increases with the stiffening of arteries. Arterial stiffening increases left ventricular (LV) afterload and alters the coronary perfusion. With increased PWV, the WR impacts on the aorta during systole, increasing systolic pressures and myocardial oxygen consumption, and decreasing diastolic BP and coronary flow. The arterial stiffness is altered primarily in association with increased collagen content and alterations of extracellular matrix (arteriosclerosis) as classically observed during aging or in arterial hypertension. The arterial stiffening estimated by changes in aortic PWV and intensity of WR are independent predictors of survival in end stage renal disease (ESRD) and general population. Improvement of arterial stiffening could be obtained by antihypertensive treatmen as observed with the calcium-channel blocker and ACE inhibitors. ACE inhibitors increased AC and reduced WR, and it has been shown that reversibility of aortic stiffening and use of ACE inhbitors had favorable independent effect on survival in hypertensive patients with advanced renal disease.

  9. Pathophysiology of osteoporosis: new mechanistic insights.

    Science.gov (United States)

    Armas, Laura A G; Recker, Robert R

    2012-09-01

    Understanding of the pathophysiology of osteoporosis has evolved to include compromised bone strength and skeletal fragility caused by several factors: (1) defects in microarchitecture of trabeculae, (2) defective intrinsic material properties of bone tissue, (3) defective repair of microdamage from normal daily activities, and (4) excessive bone remodeling rates. These factors occur in the context of age-related bone loss. Clinical studies of estrogen deprivation, antiresorptives, mechanical loading, and disuse have helped further knowledge of the factors affecting bone quality and the mechanisms that underlie them. This progress has led to several new drug targets in the treatment of osteoporosis.

  10. Fatal tumors: prenatal ultrasonographic findings and clinical characteristics

    Energy Technology Data Exchange (ETDEWEB)

    Cho, Jeong Yeon [Dept. of Radiology, Seoul National University College of Medicine, Seoul (Korea, Republic of); Lee, Young Ho [Dept. of Radiology, Cheil General Hospital and Women' s Healthcare Center, Kwandong University College of Medicine, Seoul (Korea, Republic of)

    2014-12-15

    The incidence of fetal tumors has been increased due to generalization of prenatal evaluation and improvement of imaging techniques. The early detection of a fetal tumor and understanding of its imaging features are very important for fetal, maternal, and neonatal care. Ultrasonography is usually used for the detection and differential diagnosis of fetal tumors, and magnetic resonance imaging is increasingly being used as a complementary study. Many fetal tumors have different clinical and imaging features compared with pediatric tumors. Although several fetal tumors may mimic other common anomalies, some specific imaging features may carry early accurate diagnosis of fetal tumors, which may alter the prenatal management of a pregnancy and the mode of delivery, and facilitate immediate postnatal treatment.

  11. Causes and clinical characteristics of spontaneous intracerebral hemorrhage in children

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    Yan-ju MENG

    2014-01-01

    Full Text Available In this study, clinical data of 31 patients in childhood with spontaneous intracerebral hemorrhage (SICH were retrospectively analyzed. According to various imaging examinations (CT, MRI, CTA, MRA and DSA, 22 cases (70.97% had definite causes, including 9 cases (29.03% with intracranial arteriovenous malformation, 6 cases (19.35% with cavernous hemangioma, 3 cases (9.68% with hematopathy, 2 cases (6.45% with tumor apoplexy, one case (3.23% with intracranial aneurysm and one case (3.23% with moyamoya disease; 9 cases (29.03% had unclear causes. All cases were timely diagnosed and treated. Among all the patients, 23 cases (74.19% were cured with good prognosis, 6 cases (19.35% improved, and the other 2 cases (6.45% died. Therefore, primary diseases should be timely treated as hematoma was removed.

  12. Clinical characteristics and diagnostic imaging of cranial osteoblastoma.

    Science.gov (United States)

    Pelargos, Panayiotis E; Nagasawa, Daniel T; Ung, Nolan; Chung, Lawrance K; Thill, Kimberly; Tenn, Stephen; Gopen, Quinton; Yang, Isaac

    2015-03-01

    Benign osteoblastoma is a rare, vascular, osteoid-forming bone tumor that occurs even less frequently in the cranial bones. Benign osteoblastoma of the cranium affects women slightly more often than men and typically presents in the first three decades of life. Although clinical presentation can vary depending on location, cranial osteoblastoma usually presents as a painful, non-mobile, subcutaneous mass or swelling. On CT scan, it generally presents as a well-demarcated, mixed lytic and sclerotic lesion, with enlarged diploe, thinning outer and/or inner tables, and varying degrees of calcification. It is hypo to isointense on T1-weighted MRI and has variable presentation on T2-weighted MRI. Gross total resection is the definitive treatment, while subtotal resection is utilized when it is necessary to preserve critical adjacent neurovascular structures.

  13. Case of clinical Reye syndrome presenting characteristic CT changes

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    Hino, Tamaki; Sai, Hoshun; Morikawa, Yuji; Mizuta, Ryuzo (Kyoto Second Red Cross Hospital (Japan)); Okuno, Takehiko

    1984-05-01

    A 9-month-old male infant was admitted to our hospital on the second day of cold like syndrome because of high fever, convulsion, coma, and decerebrate rigidity. Serum GOT, GPT, LDH, and CPK were markedly elevated. Serum ammonia was slightly increased, and hypoglycemia was present. The cerebrospinal fluid showed no pleocytosis, normal sugar content, but increased protein. Thus we made a diagnosis of clinical Reye syndrome according to the criteria by Yamashita, et al. A CT on the day of admission showed symmetrical low-density areas in the posterior fossa and the regions of thalamus. Ringed enhancements were seen around the areas of low density in the thalamus on the twenty-second hospital day. We consider that these lesions may represent the infarction due to obstruction of the thalamoperforant arteries caused by cerebral edema in the early stage of the disease.

  14. Clinical Characteristics and Prognosis of End-stage Hypertrophic Cardiomyopathy

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    Yan Xiao

    2015-01-01

    Full Text Available Background: End-stage hypertrophic cardiomyopathy (HCM is complicated by substantial adverse events. However, few studies have focused on electrocardiographic features and their prognostic values in HCM. This study aimed to evaluate the clinical manifestations and prognostic value of electrocardiography in patients with end-stage HCM. Methods: End-stage HCM patients were enrolled from a total of 1844 consecutive HCM patients from April 2002 to November 2013 at Fuwai Hospital. Clinical data, including medical history, electrocardiography, and echocardiography, were analyzed. Cox hazards regression analysis was used to assess the risk factors for cardiovascular mortality. Results: End-stage HCM was identified in 99 (5.4% patients, averaged at 52 ± 16 years old at entry. Atrial fibrillation was observed in 53 patients and mural thrombus in 19 patients. During 3.9 ± 3.0 years of follow-up, embolic stroke, refractory heart failure, and death or transplantation were observed in 20, 39, and 51 patients, respectively. The incidence of annual mortality was 13.2%. Multivariate Cox hazards regression analysis identified New York Heart Association Class (NYHA III/IV at entry (hazard ratio [HR]: 1.99; 95% confidence interval [CI]: 1.05-3.80; P = 0.036, left bundle branch block (LBBB (HR: 2.80; 95% CI: 1.47-5.31; P = 0.002, and an abnormal Q wave (HR: 2.21; 95% CI: 1.16-4.23; P = 0.016 as independent predictors of cardiovascular death, in accordance with all-cause death and heart failure-related death. Conclusions: LBBB and an abnormal Q wave are risk factors of cardiovascular mortality in end-stage HCM and provide new evidence for early intervention. Susceptibility of end-stage HCM patients to mural thrombus and embolic events warrants further attention.

  15. CLINICAL AND BIOCHEMICAL CHARACTERISTICS AT THE CIRRHOSIS OF VARIOUS GENESIS

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    A. R. Bilalova

    2016-01-01

    Full Text Available Purpose of the study. To carry out a comparative description of the clinical and biochemical parameters at patients with cirrhosis of an alcoholic genesis (CP-HGA and cirrhosis of mixed etiologies — CP-HGM (HСV+ alcohol.Materials and methods. The study involved 62 patients with cirrhosis of different etiologies, who carries out clinical, immunogenetic and biochemical studies.Results. Patients with the 3d genotype and low viral load were registered with cirrhosis of mixed etiologies (HСV+ alcohol. At the cirrhosis Class B for Child-Pugh basic data biochemical parameters were similar in patients with CP-HGA and CP-HGM, but ALT and AST activity, which are significantly higher than observed in patients with CP-HGM. At dismissal, ALT and GGT activities were detected significantly higher in patients with CP-HGM than the CP-HGA. At the cirrhosis Class C for Child-Pugh the baseline, reflecting cholestasis — is total bilirubin, GGT and alkaline phosphatase and were detected significantly higher in the CP-HGA, than with CP-HGM significantly reduced, and thore is no differences between the groups to be discharged from the hospital, in addition to the activity of GGT, which it remained significantly higher in the CP-HGA, than with CP-HGM. Cytolytic activity of enzymes (ALT, AST during the entire period of the disease was observed significantly higher normal values and did not depend on the CP etiology.Conclusion decision. The maximum rate of change of the basic biochemical parameters is observed in patients with cirrhosis of mixed etiologies Class B for Child-Pugh and at the cirrhosis of an alcoholic genesis — in patients with cirrhosis Class C.

  16. Prevalence, clinical characteristics and management of atrial fibrillation in patients with Brugada syndrome

    NARCIS (Netherlands)

    Rodriguez-Manero, M.; Namdar, M.; Sarkozy, A.; Casado-Arroyo, R.; Ricciardi, D.; Asmundis, C. de; Chierchia, G.B.; Wauters, K.; Rao, J.Y.; Bayrak, F.; Malderen, S. Van; Brugada, P.

    2013-01-01

    Atrial fibrillation (AF) can be the first manifestation of latent Brugada syndrome (BS). The aim of our study was to assess the prevalence of AF as the first clinical diagnosis in patients with BS and their demographic and clinical characteristics and diagnosis management in a large cohort of patien

  17. The clinical characteristics of 625 patients with malignant small bowel tumors

    Institute of Scientific and Technical Information of China (English)

    俞利结

    2012-01-01

    Objective To investigate the clinical characteristics of MSBT(malignant small bowel tumors) .Methods Six hundred and twenty-five cases of MSBT were recruited in this study and their clinical records and information including age,gender,blood types,family history,personal

  18. Clinical Application and Characteristics of KONG's Scalp Acupuncture and Daoyin Technique

    Institute of Scientific and Technical Information of China (English)

    JIANG Ling-zhen; XIAO Yuan-chun

    2004-01-01

    @@ Scalp Acupuncture and Daoyin Technique, which are summarized by Dr. KONG in the light of his long-term acupuncture practice, has been considered as one of the clinical diagnostic and treatment programs in Chinese medicine by the State Administration of Traditional Chinese Medicine. In this paper, the characteristics and clinical application of Scalp Acupuncture and Daoyin Technique are presented as follows.

  19. The characteristics and clinical manifestation of subjects with non-specific pattern of pulmonary function tests

    Institute of Scientific and Technical Information of China (English)

    周德训

    2014-01-01

    Objective To analyze the characteristics of pulmonary function and the clinical significance of non-specific pattern(NSP).Methods A total of 1 933 pulmonary function tests of adult patients were analyzed,and those with NSP were selected.The pulmonary function test results,clinical diagnosis and radiological manifestations were

  20. [The clinical and molecular genetic characteristics of phenylketonuria patients in the Republic of Crimea].

    Science.gov (United States)

    Afanas'eva, N A; Bychkova, A M; Livshits, L A; Bariliak, I R

    1998-01-01

    The clinical and genetical characteristics of patients with phenylketonuria in the Crimean population is done in the present work. The comparison of clinical peculiarities of 28 patients, revealed by means of neonatal screening and that of 24 patients, the treatment of which was started late is presented. The prenatal diagnostics of 4 families with high phenylketonuria risk is conducted.

  1. Characteristics of Placebo Responders in Pediatric Clinical Trials of Attention-Deficit/Hyperactivity Disorder

    Science.gov (United States)

    Newcorn, Jeffrey H.; Sutton, Virginia K.; Zhang, Shuyu; Wilens, Timothy; Kratochvil, Christopher; Emslie, Graham J.; D'Souza, Deborah N.; Schuh, Leslie M.; Allen, Albert J.

    2009-01-01

    Objective: Understanding placebo response is a prerequisite to improving clinical trial methodology. Data from placebo-controlled trials of atomoxetine in the treatment of children and adolescents with attention-deficit/hyperactivity disorder (ADHD) were analyzed to identify demographic and clinical characteristics that might predict placebo…

  2. CLINICAL-IMMUNOLOGIC CHARACTERISTICS OF ACUTE BRUCELLOSIS IN CHILDREN

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    D. R. Atakhodjayeva

    2013-12-01

    Full Text Available Background: Brucellosis is acute zoonotic, multi-systemic infection caused by Brucella bacteria kind. Brucellosis is met everywhere on all continents of the world, especially in the countries where livestock sector is developed. Nowadays in spite of significant success in the struggle against brucellosis this infection is a social problem. Brucellosis has specific clinical manifestations during various age periods. Problems interrelated with the study of the pathogenesis of brucellosis infection, particularly immune genesis, defining the progress and the outcome of the disease have great importance. Object of the research: to study peculiarities of the progress of acute brucellosis in children taking into account clinical-immunologic data. Materials and methods of the research: the research was based on the results of examinations of 23 children from 3 to 14 years old with brucellosis mostly living in endemic foci of the Republic of Uzbekistan. The diagnosis was defined based on epidemiologic anamnesis, clinical symptoms and laboratory data. All examined children got the analysis of detailed blood immunogramm. The corresponding data of 20 healthy children served to be controlling ones. Results and discussion: The analysis of epidemiologic anamnesis showed, that 78.3% of the examined patients with brucellosis were villagers keeping sheep, goats and cattle. In 73.9% cases source of infection was sheep and goats, 8.7% - cattle, and in 17.4% cases we could observe mixed type of infection. 78% of patients applied to hospital during prodromal period. Main complaints were weakness, frustration, headache, fatigue, bad appetite. These symptoms lasted not more than 3-5 days, after which there were symptoms of intoxication of organism with the rise of cardinal symptoms such as fever with chill (100%, arthalgia (69%, slight sweating (70%, hepato-lienal syndrome (68%. 32.9% of patients had tachycardia. Objective visual examination showed enlargement of

  3. Clinical characteristics of patients with benign nonlesional temporal lobe epilepsy

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    Kim J

    2016-07-01

    Full Text Available Jiyeon Kim,1 Seong Hoon Kim,2 Sung Chul Lim,2 Woojun Kim,2 Young-Min Shon3 1Department of Neurology, Korea University Ansan Hospital, College of Medicine, Korea University, Ansan, 2Department of Neurology, Catholic Neuroscience Institute, College of Medicine, The Catholic University of Korea, Seocho-gu, 3Department of Neurology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea Purpose: To evaluate the evolution of nonlesional temporal lobe epilepsy (TLE-NL in patients treated exclusively with antiepileptic drugs and to elucidate clinical phenotypes related to the prognosis of these patients.Methods: Clinical, radiological, and electroencephalographic (EEG findings in 84 patients with TLE-NL were reviewed. A good response group (GRG and a poor response group (PRG were defined if the duration of their seizure-free period was >1 year, or <1 year, respectively.Results: There were 46 (54.8% patients in the GRG and 38 (45.2% patients in the PRG. The number of antiepileptic drugs administered was significantly lower in the GRG than that in the PRG (1.3±0.8 vs 2.8±1.0, respectively; P<0.05. The GRG had a significantly older age of onset than the PRG and a lower occurrence of initial precipitating events, such as febrile seizures, central nervous system infection, and head trauma (P<0.05. The prevalence of EEG abnormality, presence of aura, generalized seizures, and automatism was less frequently observed in the GRG (P<0.05. Multivariate analysis showed that the presence of automatism and initial precipitating events were significantly associated with a poor prognosis (P<0.05.Conclusion: In contrast to the commonly assumed intractability of TLE, we found that more than 54% of patients with TLE-NL achieved a long seizure-free period. Older age at onset of TLE-NL was associated with a better prognosis. However, the presence of automatism and initial precipitating events were related to a poor prognosis. Future prospective

  4. Clinical Characteristics of Spinal Levobupivacaine: Hyperbaric Compared with Isobaric Solution

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    Vimolluck Sanansilp

    2012-01-01

    Full Text Available We performed a prospective, double-blinded study in 20 patients undergoing gynecologic surgery with lower abdominal incision, to investigate characteristics of intrathecal hyperbaric levobupivacaine compared with isobaric levobupivacaine. We randomly assigned them to receive 3 mL of either isobaric or hyperbaric 0.42% levobupivacaine intrathecally. We found that hyperbaric levobupivacaine, compared with isobaric levobupivacaine, spread faster to T10 level (2.8 ± 1.1 versus 6.6 ± 4.7 minutes, =0.039, reached higher sensory block levels at 5 and 15 minutes after injection (T8 versus L1, =0.011, and T4 versus T7, =0.027, resp., and had a higher peak level (T4 versus T8, =0.040. Isobaric levobupivacaine caused a wider range of peak levels (L1 to C8 compared with hyperbaric form (T7 to T2. The level of T4 or higher reached 90% in the hyperbaric group compared with 20% in the isobaric group (=0.005. Our results suggest that hyperbaric levobupivacaine was more predictable for sensory block level and more effective for surgical procedures with lower abdominal approach. Hyperbaric levobupivacaine seems to be suitable, but the optimal dosage needs further investigation.

  5. [Pathophysiology of rheumatoid arthritis].

    Science.gov (United States)

    Lequerré, Thierry; Richez, Christophe

    2012-10-01

    These last years were especially marked by the best understanding of the physiopathological mechanisms at the onset of rheumatoid arthritis (RA) and in the processes of joint inflammation and joint destruction. RA is more and more considered as a syndrome with at least two clinical entities with different phenotype and profiles: seronegative RA and seropositive RA. In RA with ACPA, it is the process of immunization, that is the immunological reaction against citrullinated peptides, that leads to the disease. The peptide citrullination is directly favored by environmental factors such as tobacco, infection to Porphyromonas gingivalis and alcohol. The immunization supposes a genetic predisposition including approximately 22 genetic factors including the molecules of the major histocompatibility complex (MHC) and PTPN22. Finally, joint damage result at the same time from an excess of destruction (RANK/RANKL, TNFalpha) and from a defect of bone reparation by the way Wnt/Frizzled. It is thanks to the best understanding of RA physiopathology that leads to development of targeted treatments and specially processing for this disease.

  6. [Pathophysiology of NREM parasomnias].

    Science.gov (United States)

    2009-01-01

    Parasomnias are physical, behavioral and experiental phenomena ocurring during entry or along the sleep or on arousal/awakening. The behavior includes movements, emotional, perceptual or dreaming experience, frequently containing manifestations of autonomic nervous system. Parasomnias are devided into primary (ocurring etiher in NREM or REM sleep) and secondary (following organic system disease manifested during sleep). Primary parasomnias are further devided into those that appear during NREM, REM or states of consciousnes that do not respect boundaries between wake and sleep. Parasomnias represent an example of "dissocitaion of sleep stages" with the overlaping of wakefulness and NREM sleep (confusional arousals, somnambulism and night terrors) or wake and REM sleep (REM sleep behavior disorder parasomnia). NREM parasomnias are a significant clinical problem that appears with functional reorganization of the brain as it transits throuh different states of consciousness. Aside from the above dissociation there are other physiological phenomena that render behavior more complex during sleep such as 1) activation of locomotor centers during sleep, 2) sleep intertia (confusion and desorientation during transition from sleep to wakefulness) and 3) instability of sleep stages (rapid oscilation(s) between seleep and wake).

  7. Pathophysiology of merkel cell

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    Prashant Balasaheb Munde

    2013-01-01

    Full Text Available The objective of this review is to introduce Merkel cells (MCs, to provide a basic overview on the theoretical background of function, development and clinical importance of MCs. The origin of human MCs have been controversial. Some investigators believe that it is a neural crest derivate, whereas others have proposed that it is a differentiation product of the fetal epidermal keratinocytes. MCs are cells primarily localized in the epidermal basal layer of vertebrates and concentrated in touch-sensitive areas in glabrous, hairy skin and in some mucosa. In routine light microscopy, human MCs can hardly be identified. Cytokeratin 20 (CK20 is a reliable marker with highest degree of specificity. MCs can be also distinguished by electron microscopy. MC carcinoma (MCC is an uncommon and often aggressive malignancy and found mainly in elderly patients. It occurs most frequently in the head and neck region. Diagnosis is based on typical histological presentation on hematoxylin and eosin (H and E stained slides together with the results of immunohistochemistry. Histologically, MCC has been classified into three distinct subtypes: Trabecular, intermediate and small cell type

  8. Clinical disease characteristics according to karyotype in Turner syndrome

    Directory of Open Access Journals (Sweden)

    Chae Young Yeo

    2010-02-01

    Full Text Available Purpose : Turner syndrome (TS is a disorder in which various anomalies can be accompanied, especially cardiovascular, renal, thyroid and auditory problems. The aim of this study is to identify the incidence of these disorders in patients with TS according to karyotype. Methods : We reviewed medical records of 90 patients with TS diagnosed by chromosomal analysis in 4 hospitals from Jan 1998 to Dec 2007. We evaluated these cases by prepared protocol of 4 medical problems. Results : The distribution of karyotype was 45,X (47.8%, mosaic pattern (34.4% and structural aberration group (17.8 %. Renal anomalies, cardiovascular anomalies, thyroid disorders and auditory problems are accompanied in 4.4%, 10.0 %, 11.1% and 5.6%, respectively. 45,X group had renal anomalies (7.0%, cardiovascular anomalies (18.6%, thyroid disorders (9.3% and auditory problems (11.6%. Mosaic group had renal anomalies (3.2%, thyroid disorders (12.9%, no cardiovascular anomalies and auditory problems. Structural aberration group had cardiovascular anomalies (6.3%, thyroid disorders (12.5% and no other 2 problems. Patients with 45,X group had a significant higher incidence of cardiovascular anomalies (P=0.025. Conclusion : Our results indicate that there are differences clinically according to karyotype of TS, especially in incidence of cardiovascular anomalies.

  9. Histamine H1 antagonists and clinical characteristics of febrile seizures

    Directory of Open Access Journals (Sweden)

    Zolaly MA

    2012-03-01

    Full Text Available Mohammed A ZolalyDepartment of Pediatrics, College of Medicine, Taibah University, Al-Madinah Al-Munawarah, Kingdom of Saudi ArabiaBackground: The purpose of this study was to determine whether seizure susceptibility due to antihistamines is provoked in patients with febrile seizures.Methods: The current descriptive study was carried out from April 2009 to February 2011 in 250 infants and children who visited the Madinah Maternity and Children's Hospital as a result of febrile convulsions. They were divided into two groups according to administration of antihistamines at the onset of fever.Results: Detailed clinical manifestations were compared between patients with and without administration of antihistamines. The time from fever detection to seizure onset was significantly shorter in the antihistamine group than that in the nonantihistamine group, and the duration of seizures was significantly longer in the antihistamine group than in the nonantihistamine group. No significant difference was found in time from fever detection to seizure onset or seizure duration between patients who received a first-generation antihistamine and those who received a second-generation antihistamine.Conclusion: Due to their central nervous system effects, H1 antagonists should not be administered to patients with febrile seizures and epilepsy. Caution should be exercised regarding the use of histamine H1 antagonists in young infants, because these drugs could potentially disturb the anticonvulsive central histaminergic system.Keywords: antihistamine, nonantihistamine, histamine H1 antagonist, febrile seizures

  10. Clinical and electrophysiological characteristics of neuropathy associated with Tangier disease.

    Science.gov (United States)

    Zyss, Julie; Béhin, Anthony; Couvert, Philippe; Bouhour, Françoise; Sassolas, Agnès; Kolev, Ivan; Denys, Violaine; Vial, Christophe; Lacour, A; Carrié, Alain; Stojkovic, Tanya

    2012-06-01

    Tangier disease (TD) (OMIM#205400) is a rare autosomal recessive disorder resulting from mutations in the ABCA1 gene, leading to decreased levels of plasma high-density lipoproteins (HDL). Peripheral neuropathy is a common finding in this disease, and may present as relapsing/remitting mono/polyneuropathies or as syringomyelia-like neuropathy. We retrospectively analyzed four patients, and report here their clinical, biological, electrophysiological, imaging, and genetic findings. Three patients had a typical pseudosyringomyelic neuropathy including facial diplegia, but asymmetrical onset was observed in one patient who had first been misdiagnosed with Lewis-Sumner syndrome. Electrophysiological pattern was heterogeneous, showing both signs of demyelination and axonal degeneration. Truncating mutations of the ABCA1 gene, including two previously undescribed mutations, were constantly found. Atypical symptom onset and demyelinating features on electrophysiological examination can be misleading in case of pseudosyringomyelic neuropathy. These reports illustrate two different neurological phenotypes in TD, namely the pseudosyringomyelic type and the Lewis-Sumner-like type, and advocate for a systematic assessment of lipid profile including HDL cholesterol in demyelinating neuropathies.

  11. Epidemiology and clinical characteristics of traumatic brain injury in Lebanon

    Science.gov (United States)

    Abou-Abbass, Hussein; Bahmad, Hisham; Ghandour, Hiba; Fares, Jawad; Wazzi-Mkahal, Rayyan; Yacoub, Basel; Darwish, Hala; Mondello, Stefania; Harati, Hayat; El Sayed, Mazen J.; Tamim, Hani; Kobeissy, Firas

    2016-01-01

    , epidemiological, clinical and translational research in this field in the future. PMID:27893670

  12. On renal pathophysiology in preeclampsia

    NARCIS (Netherlands)

    Penning, Maria Elisabeth (Marlies)

    2014-01-01

    Preeclampsia is a complication of pregnancy which can suddenly change from a relatively mild phenotype into a life-threatening situation. One of the organs that is always involved during preeclampsia is the kidney. The placenta plays an important role in the renal pathophysiology of preeclampsia. Th

  13. Pathophysiological mechanisms of insulin resistance

    NARCIS (Netherlands)

    Brands, M.

    2013-01-01

    In this thesis we studied pathophysiological mechanisms of insulin resistance in different conditions in humans, i.e. in obesity, during lipid infusions, after hypercaloric feeding, and glucocorticoid treatment. We focused on 3 important hypotheses that are suggested to be implicated in the pathophy

  14. Pathophysiology and management of multivalvular disease

    Science.gov (United States)

    Unger, Philippe; Clavel, Marie-Annick; Lindman, Brian R.; Mathieu, Patrick; Pibarot, Philippe

    2016-01-01

    Multivalvular disease (MVD) is a common condition with a complex pathophysiology, dependent on the specific combination of valve lesions. Diagnosis is challenging as several echocardiographic methods commonly used for the assessment of stenosis or regurgitation have been validated only in patients with single valve disease. Decisions about the timing and type of treatment should be made by a multidisciplinary heart valve team, on a case-by-case basis. Several factors should be considered, including the severity and consequences of the MVD, the patient’s life expectancy and comorbidities, the surgical risk associated with combined valve procedures, the long-term risk of morbidity and mortality associated with multiple valve prostheses, and the likelihood and risk of reoperation. The introduction of transcatheter valve therapies into clinical practice has provided new treatment options for patients with MVD, and decision-making algorithms on how to combine surgical and percutaneous treatment options are evolving rapidly. In this Review, we discuss the pathophysiology, diagnosis, and treatment of MVD, focussing on the combination of valve pathologies that are most often encountered in clinical practice. PMID:27121305

  15. Inflammation and Arterial Hypertension: From Pathophysiological Links to Risk Prediction.

    Science.gov (United States)

    Pietri, Panagiota; Vlachopoulos, Charalambos; Tousoulis, Dimitris

    2015-01-01

    Over the last years, ample data have demonstrated the pivotal role of low-grade inflammation in the pathophysiology of atherosclerosis and cardiovascular disease. It is well established that inflammatory activation, serving either as a substrate, in the chronic phase of atherosclerotic disease, or as a trigger, in the acute phase, increases cardiovascular events. Considering hypertension, the inflammatory process is implicated in its pathophysiology through a bidirectional relationship since arterial hypertension may enhance inflammation and vice versa. Inflammatory biomarkers such as high-sensitivity C-reactive protein, have shown predictive value for both the incidence of hypertension and the clinical outcomes in hypertensive patients. In the present review, data on the association between arterial hypertension and low-grade inflammation will be reported and potential pathophysiological pathways and clinical implications underlying this association will be discussed.

  16. An overview of the Charcot foot pathophysiology.

    Science.gov (United States)

    Kaynak, Gökhan; Birsel, Olgar; Güven, Mehmet Fatih; Oğüt, Tahir

    2013-01-01

    Charcot arthropathy of the foot is a rare but devastating complication of diabetes that remains to be a challenging issue for the foot and ankle surgeons. Charcot foot fails to be an obvious diagnostic option that comes to mind, even in a pathognomonic clinical appearance. The rarity of the disorder, more common pathologies that mimic the condition, and the self-limiting prognosis deviate the clinician from the right diagnosis. The clinical challenges in the diagnosis of Charcot foot require in-depth investigations of its enigmatic nature to establish useful guidelines. Yet, this goal seems to be beyond reach, without a holistic view of the immense literature concerning the pathophysiology of the disorder. The primary objective of this article is to put together and review the recent advancements about the etiology and intrinsic mechanisms of diabetic Charcot foot.

  17. An overview of the Charcot foot pathophysiology

    Directory of Open Access Journals (Sweden)

    Gökhan Kaynak

    2013-08-01

    Full Text Available Charcot arthropathy of the foot is a rare but devastating complication of diabetes that remains to be a challenging issue for the foot and ankle surgeons. Charcot foot fails to be an obvious diagnostic option that comes to mind, even in a pathognomonic clinical appearance. The rarity of the disorder, more common pathologies that mimic the condition, and the self-limiting prognosis deviate the clinician from the right diagnosis. The clinical challenges in the diagnosis of Charcot foot require in-depth investigations of its enigmatic nature to establish useful guidelines. Yet, this goal seems to be beyond reach, without a holistic view of the immense literature concerning the pathophysiology of the disorder. The primary objective of this article is to put together and review the recent advancements about the etiology and intrinsic mechanisms of diabetic Charcot foot.

  18. The pathophysiology of lifelong premature ejaculation

    Science.gov (United States)

    2016-01-01

    For many decades it has been thought that lifelong premature ejaculation (PE) is only characterized by persistent early ejaculations. Despite enormous progress of in vivo animal research, and neurobiological, genetic and pharmacological research in men with lifelong PE, our current understanding of the mechanisms behind early ejaculations is far from complete. The new classification of PE into four PE subtypes has shown that the symptomatology of lifelong PE strongly differs from acquired PE, subjective PE and variable PE. The phenotype of lifelong PE and therefore also the pathophysiology of lifelong PE is much more complex. A substantial number of men with lifelong PE not only have PE, but also premature erection and premature penile detumescence as part of an acute hypertonic or hypererotic state when engaged in an erotic situation or when making love. As both erectio praecox, ejaculatio praecox, detumescentia praecox, and the hypererotic state are part of the phenotype lifelong PE, it is argued that lifelong PE is not only a disturbance of the timing of ejaculation but also a disturbance of the timing of erection, detumescence and arousal. Since 1998, the pathophysiology of lifelong PE was thought to be mainly mediated by the central serotonergic system in line with genetic polymorphisms of specific serotonergic genes. However, by accepting that lifelong PE is characterized by the reversible hypertonic state the hypothesis of mainly serotonergic dysfunction is no longer tenable. Instead, it has been postulated that the pathophysiology of lifelong PE is mediated by a very complex interplay of central and peripheral serotonergic, dopaminergic, oxytocinergic, endocrinological, genetic and probably also epigenetic factors. Progress in research of lifelong PE can only be accomplished when a stopwatch is used to measure the IELT and the cut-off point of 1 minute for the definition of lifelong PE is maintained. Current use of validated questionnaires, neglect of

  19. The pathophysiology of lifelong premature ejaculation.

    Science.gov (United States)

    Waldinger, Marcel D

    2016-08-01

    For many decades it has been thought that lifelong premature ejaculation (PE) is only characterized by persistent early ejaculations. Despite enormous progress of in vivo animal research, and neurobiological, genetic and pharmacological research in men with lifelong PE, our current understanding of the mechanisms behind early ejaculations is far from complete. The new classification of PE into four PE subtypes has shown that the symptomatology of lifelong PE strongly differs from acquired PE, subjective PE and variable PE. The phenotype of lifelong PE and therefore also the pathophysiology of lifelong PE is much more complex. A substantial number of men with lifelong PE not only have PE, but also premature erection and premature penile detumescence as part of an acute hypertonic or hypererotic state when engaged in an erotic situation or when making love. As both erectio praecox, ejaculatio praecox, detumescentia praecox, and the hypererotic state are part of the phenotype lifelong PE, it is argued that lifelong PE is not only a disturbance of the timing of ejaculation but also a disturbance of the timing of erection, detumescence and arousal. Since 1998, the pathophysiology of lifelong PE was thought to be mainly mediated by the central serotonergic system in line with genetic polymorphisms of specific serotonergic genes. However, by accepting that lifelong PE is characterized by the reversible hypertonic state the hypothesis of mainly serotonergic dysfunction is no longer tenable. Instead, it has been postulated that the pathophysiology of lifelong PE is mediated by a very complex interplay of central and peripheral serotonergic, dopaminergic, oxytocinergic, endocrinological, genetic and probably also epigenetic factors. Progress in research of lifelong PE can only be accomplished when a stopwatch is used to measure the IELT and the cut-off point of 1 minute for the definition of lifelong PE is maintained. Current use of validated questionnaires, neglect of

  20. Functional capacity of Brazilian patients with Parkinson's disease (PD): relationship between clinical characteristics and disease severity.

    Science.gov (United States)

    Barbieri, Fabio A; Rinaldi, Natalia M; Santos, Paulo Cezar R; Lirani-Silva, Ellen; Vitório, Rodrigo; Teixeira-Arroyo, Cláudia; Stella, Florindo; Gobbi, Lilian Teresa B

    2012-01-01

    The present study had three objectives: (a) to characterize the functional capacity of patients with PD, (b) to assess the relationship between the physical fitness components of functional capacity with clinical characteristics and disease severity, and (c) to compare the physical fitness components of functional capacity with clinical characteristics according to disease severity. The study included 54 patients with idiopathic PD who were distributed into two groups according to PD severity: unilateral group (n=35); and bilateral group (n=19). All patients underwent psychiatric assessment by means of the Hoehn and Yahr (HY) staging of PD, the Unified Parkinson's Disease Rating Scale (UPDRS), the Hospital Anxiety and Depression Scale (HADS-A and HADS-D, respectively), and The Mini-Mental State Examination (MMSE). The physical fitness components of functional capacity were evaluated over a 2-day period, using recommendations by the American Alliance for Health, Physical Education, Recreation and Dance, and the Berg Balance Scale (BBS). Pearson correlation coefficients and multiple regressions were calculated to test the correlation between functional capacity and clinical characteristics, and to predict clinical scores from physical performance, respectively. Clinical variables and physical component data were compared between groups using analysis of variance to determine the effects of disease severity. Patients with advanced disease showed low levels of functional capacity. Interestingly, patients with good functional capacity in one of the physical fitness components also showed good capacities in the other components. Disease severity is a major factor affecting functional capacity and clinical characteristics. Medical providers should take disease severity into consideration when prescribing physical activity for PD patients, since the relationship between functional capacity and clinical characteristics is dependent on disease severity.

  1. Clinical and Pathologic Characteristics of Myocarditis as a Cause of Sudden Death

    Science.gov (United States)

    2008-01-01

    Clinical and Pathologic Characteristics of  Myocarditis  as a Cause of Sudden Death Lena Avedissian, Jennifer A. McNear, David A. Appel, Laudino M...00-00-2008 to 00-00-2008 4. TITLE AND SUBTITLE Clinical and Pathologic Characteristics of Myocarditis as a Cause of Sudden Death 5a. CONTRACT...unclassified b. ABSTRACT unclassified c. THIS PAGE unclassified Standard Form 298 (Rev. 8-98) Prescribed by ANSI Std Z39-18  Myocarditis  as a

  2. On renal pathophysiology in preeclampsia

    OpenAIRE

    2014-01-01

    Preeclampsia is a complication of pregnancy which can suddenly change from a relatively mild phenotype into a life-threatening situation. One of the organs that is always involved during preeclampsia is the kidney. The placenta plays an important role in the renal pathophysiology of preeclampsia. The placenta produces excessive amounts of anti-angiogenic factors which are associated with systemic endothelial dysfunction. Although the underlying mechanisms of renal injury during preeclampsia r...

  3. Pathophysiology of cutaneous lupus erythematosus

    OpenAIRE

    Achtman, Jordan C; Werth, Victoria P.

    2015-01-01

    The pathophysiology of cutaneous lupus erythematosus (CLE) encompasses the complex interactions between genetics, the environment, and cells and their products. Recent data have provided enhanced understanding of these interactions and the mechanism by which they cause disease. A number of candidate genes have been identified which increase the risk of developing CLE. Ultraviolet radiation, the predominant environmental exposure associated with CLE, appears to initiate CLE lesion formation by...

  4. Virologic and clinical characteristics of HBV genotypes/subgenotypes in 487 Chinese pediatric patients with CHB

    OpenAIRE

    Gu Mei-Lei; Li Xiao-Dong; Xing Xiao-Yan; Dong Yi; Duan Zhong-Ping; Song Hong-Bin; Li Jin; Zhong Yan-Wei; Han Yu-Kun; Zhu Shi-Shu; Zhang Hong-Fei

    2011-01-01

    Abstract Background The association of hepatitis B virus (HBV) genotypes/subgenotypes with clinical characteristics is increasingly recognized. However, the virologic and clinical features of HBV genotypes/subgenotypes in pediatric patients remain largely unknown. Methods Four hundred and eighty-seven pediatric inpatients with CHB were investigated, including 217 nucleos(t)ide analog-experienced patients. HBV genotypes/subgenotypes and reverse transcriptase (RT) mutations were determined by d...

  5. Eosinophilic esophagitis: From pathophysiology to treatment.

    Science.gov (United States)

    D'Alessandro, Alessandra; Esposito, Dario; Pesce, Marcella; Cuomo, Rosario; De Palma, Giovanni Domenico; Sarnelli, Giovanni

    2015-11-15

    Eosinophilic esophagitis (EoE) is a chronic immune disease, characterized by a dense eosinophilic infiltrate in the esophagus, leading to bolus impaction and reflux-like symptoms. Traditionally considered a pediatric disease, the number of adult patients with EoE is continuously increasing, with a relatively higher incidence in western countries. Dysphagia and food impaction represent the main symptoms complained by patients, but gastroesophageal reflux-like symptoms may also be present. Esophageal biopsies are mandatory for the diagnosis of EoE, though clinical manifestations and proton pump inhibitors responsiveness must be taken into consideration. The higher prevalence of EoE in patients suffering from atopic diseases suggests a common background with allergy, however both the etiology and pathophysiology are not completely understood. Elimination diets are considered the first-line therapy in children, but this approach appears less effective in adults patients, who often require steroids; despite medical treatments, EoE is complicated in some cases by esophageal stricture and stenosis, that require additional endoscopic treatments. This review summarizes the evidence on EoE pathophysiology and illustrates the safety and efficacy of the most recent medical and endoscopic treatments.

  6. Craniosynostosis in Growing Children : Pathophysiological Changes and Neurosurgical Problems

    Science.gov (United States)

    Choi, Jung Won; Lim, So Young

    2016-01-01

    Craniosynostosis is defined as the premature fusion of one or more cranial sutures resulting in skull deformity. Characteristically, this disorder can cause diverse neurosurgical problems, as well as abnormal skull shape. Intracranial hypertension, hydrocephalus, Chiari malformation and neuropsychological dysfunction are the major neurosurgical concerns in children with craniosynostosis. In this review article, we investigate pathophysiology, characteristics and proper neurosurgical management of these neurosurgical issues, respectively. PMID:27226849

  7. Clinical microbiology in the intensive care unit: Strategic and operational characteristics

    Directory of Open Access Journals (Sweden)

    Bhattacharya S

    2010-01-01

    Full Text Available Infection is a major cause of morbidity and mortality among patients admitted in intensive care units (ICUs. The application of the principles and the practice of Clinical Microbiology for ICU patients can significantly improve clinical outcome. The present article is aimed at summarising the strategic and operational characteristics of this unique field where medical microbiology attempts to venture into the domain of direct clinical care of critically ill patients. The close and strategic partnership between clinical microbiologists and intensive care specialists, which is essential for this model of patient care have been emphasized. The article includes discussions on a variety of common clinical-microbiological problems faced in the ICUs such as ventilator-associated pneumonia, blood stream infections, skin and soft tissue infection, UTI, infection control, besides antibiotic management.

  8. Semantic characteristics of NLP-extracted concepts in clinical notes vs. biomedical literature.

    Science.gov (United States)

    Wu, Stephen; Liu, Hongfang

    2011-01-01

    Natural language processing (NLP) has become crucial in unlocking information stored in free text, from both clinical notes and biomedical literature. Clinical notes convey clinical information related to individual patient health care, while biomedical literature communicates scientific findings. This work focuses on semantic characterization of texts at an enterprise scale, comparing and contrasting the two domains and their NLP approaches. We analyzed the empirical distributional characteristics of NLP-discovered named entities in Mayo Clinic clinical notes from 2001-2010, and in the 2011 MetaMapped Medline Baseline. We give qualitative and quantitative measures of domain similarity and point to the feasibility of transferring resources and techniques. An important by-product for this study is the development of a weighted ontology for each domain, which gives distributional semantic information that may be used to improve NLP applications.

  9. The clinical characteristics,treatment and outcome of macrolide-resistant Mycoplasma pneumoniae pneumonia in children

    Institute of Scientific and Technical Information of China (English)

    鲍芳

    2013-01-01

    Objective To investigate the drug resistance of My-coplasma pneumoniae among children with community-acquired pneumonia (CAP) ,and to explore the clinical and radiological characteristics of and the role of azithromycin in the treatment of of macrolide-resistant (MR) Mycoplasma pneumoniae pneumonia.Methods Cases of CAP in children (n=179) were prospectively enrolled in

  10. Cerebellar Ataxia with Bilateral Vestibulopathy: Description of a Syndrome and Its Characteristic Clinical Sign

    Science.gov (United States)

    Migliaccio, Americo A.; Halmagyi, G. Michael; McGarvie, Leigh A.; Cremer, Phillip D.

    2004-01-01

    We report four patients with the syndrome of cerebellar ataxia with bilateral vestibulopathy (CABV) and, using search coil oculography, we validate its characteristic clinical sign, namely impairment of the visually enhanced vestibulo-ocular reflex (VVOR) or doll's head reflex. In our four patients, CABV began in the sixth decade of life; they are…

  11. Clinical characteristics and heterogeneity in patients with ketosis-prone diabetes

    Institute of Scientific and Technical Information of China (English)

    谭惠文

    2014-01-01

    Objective To investigate the clinical characteristics,peripheral insulin sensitivity,andβ-cell function in patients with ketosis-prone diabetes(KPD).Methods Thirty-one patients with newly diagnosed ketosis-prone diabetes were admitted to West China Hospital from January2004 to December 2009.They were divided into 2 groups according to

  12. Clinical characteristic of pulse wave velocity and arterial compliance in elderly patients with diabetes mellitus

    Institute of Scientific and Technical Information of China (English)

    张红

    2013-01-01

    Objective To explore the clinical characteristics of pulse wave velocity,arterial compliance and cardiovascular risk factors in elderly patients with type 2 diabetes mellitus.Methods A total of 363 patients were selected and divided into 4 groups:diabetic group,diabetic

  13. Clinical patterns and characteristics of uveitis in a tertiary center for uveitis in China

    NARCIS (Netherlands)

    Yang, P.; Zhang, Z.; Zhou, H.; Li, B.; Huang, X.; Gao, Y.; Zhu, L.; Ren, Y.; Klooster, J.; Kijlstra, A.

    2005-01-01

    Purpose: To address the clinical pattern and characteristics of uveitis in a tertiary center for uveitis in China and compare the similarity and difference in the distribution of uveitis entities between China and other countries. Methods: A retrospective study was performed on the patients with uve

  14. The concept and characteristics of clinical practice ability in Master Degree of Nursing (specialty)

    Institute of Scientific and Technical Information of China (English)

    Jing Zeng; Jing-Ci Zhu; Xiao-Yu Zhao

    2016-01-01

    The concepts of ability, practice ability, and professional practice ability were analyzed using a theoretical research method. Based on the results of the analysis, the concept of clinical practice ability for Master Degree of Nursing (specialty) students was defined, and the characteristics were interpreted to provide references for future in-depth studies.

  15. Enterococcus hirae-associated endocarditis outbreaks in broiler flocks : clinical and pathological characteristics and molecular epidemiology

    NARCIS (Netherlands)

    Velkers, F C; van de Graaf-Bloois, L; Wagenaar, J A; Westendorp, S T; van Bergen, M A P; Dwars, R M; Landman, W J M; Wagenaar, Jaap

    2011-01-01

    BACKGROUND: Enterococcus hirae-associated endocarditis, characterized by a peak in mortality during the second week of the grow-out, and occasionally lameness, was diagnosed at Dutch broiler farms. OBJECTIVES: Field cases were studied to increase knowledge on clinical and pathological characteristic

  16. Enterococcus hirae-associated endocarditis outbreaks in broiler flocks: clinical and pathological characteristics and molecular epidemiology.

    NARCIS (Netherlands)

    Velkers, F.C.; Graaf-Blois, Van de L.; Wagenaar, J.A.; Westendorp, S.T.; Bergen, Van M.A.P.; Dwars, R.M.; Landman, W.J.M.

    2011-01-01

    Background: Enterococcus hirae-associated endocarditis, characterized by a peak in mortality during the second week of the grow-out, and occasionally lameness, was diagnosed at Dutch broiler farms. Objectives: Field cases were studied to increase knowledge on clinical and pathological characteristic

  17. Clinical characteristics of familial and sporadic age-related macular degeneration: differences and similarities

    NARCIS (Netherlands)

    Saksens, N.T.M.; Kersten, E.; Groenewoud, J.M.M.; Grinsven, M.J.J.P. van; Ven, J.P.H. van de; Sanchez, C.I.; Schick, T.; Fauser, S.; Hollander, A.I. den; Hoyng, C.B.; Boon, C.J.F.

    2014-01-01

    PURPOSE: We describe the differences and similarities in clinical characteristics and phenotype of familial and sporadic patients with age-related macular degeneration (AMD). METHODS: We evaluated data of 1828 AMD patients and 1715 controls enrolled in the European Genetic Database. All subjects und

  18. Incidence, diagnosis and pathophysiology of amniotic fluid embolism.

    Science.gov (United States)

    Ito, F; Akasaka, J; Koike, N; Uekuri, C; Shigemitsu, A; Kobayashi, H

    2014-10-01

    Amniotic fluid embolism (AFE) is a rare clinical entity, sometimes fatal. A review was conducted to describe the frequency, diagnosis and pathophysiology of AFE. The reported incidences ranged from 1.9 cases per 100,000 maternities (UK) to 6.1 per 100,000 maternities (Australia), which can vary considerably, depending on the period, region of study and the definition. Although the development of amniotic fluid-specific markers would have an impact on early diagnosis, definition of AFE based on these markers is not widely accepted. To date, immunological mechanisms, amniotic fluid-dependent anaphylactic reaction and complement activation, have been proposed as potential pathogenetic and pathophysiological mechanisms. Immune cell activation induced through complement activation may be associated with the mechanism that immediately initiates maternal death, only in susceptible individuals. This review will focus on advances in the field of AFE biology and discuss the prevalence, diagnosis and pathophysiology of AFE.

  19. Clinical and Biochemical Characteristics of Growth Hormone-Secreting Pituitary Tumors

    Institute of Scientific and Technical Information of China (English)

    2000-01-01

    To investigate the difference of biochemical characteristics on gsp-positive and gsp-negative growth hormone (GH)-secreting pituitary tumors, 18 GH-secreting pituitary tumors were examined for their clinical characteristics and gsp oncogenes. All patients received the pituitary function combinative stimulating test. It was found that there were no difference in the sex, age, tumor size, course of disease and plasma basal GH levels with gsp- positive and gsp-negative patients. The plasma levels of PRL were increased in most patients (11/18), and the plasma levels of TSH in gsp-positive patients were higher than those in gsp-negative patients (P<0.05). There was no significant difference in the responses to pituitary combinative stimulating test in gsp-positive and gsp-negative patients. It was concluded that there was little difference in the clinical biochemical characteristics of gsp-positive with gsp-negative GH-secreting pituitary tumors.

  20. Incidence, frequency and clinical characteristics of type 3 myocardial infarction in clinical practice

    DEFF Research Database (Denmark)

    Jangaard, Nikolaj; Sarkisian, Laura; Saaby, Lotte

    2017-01-01

    the Danish Register of Causes of Death, ambulance and hospital patient files. Adjudication of the diagnosis was done by two local experts and one external senior cardiologist. RESULTS: A total of 2766 of the 246.723 adult residents in the region had died. A type 3 myocardial infarction was diagnosed in 18......OBJECTIVES: Cardiac death in a patient with symptoms and electrocardiographic changes indicative of myocardial ischemia but without available measurements of cardiac biomarkers is designated a type 3 myocardial infarction. We wanted to investigate the incidence, the frequency...... and the characteristics of patients diagnosed as type 3 myocardial infarction. METHODS: The occurrence of deaths in a well-defined geographic region was retrieved from the Danish Civil Registration System during a one-year period from 2010 to 2011. Complementary data concerning causes of deaths were obtained from...

  1. The role of ADAMs in disease pathophysiology.

    LENUS (Irish Health Repository)

    Duffy, Michael J

    2012-02-01

    The ADAMs are a family of multidomain transmembrane and secreted proteins involved in both proteolysis and cell adhesion. Altered expression of specific ADAMs is implicated in the pathophysiology of several diseases including rheumatoid arthritis, Alzheimer\\'s disease, cardiac hypertrophy, asthma and cancer. Of these different diseases, it is in cancer where most research has been carried out. Multiple ADAMs, including ADAM-9, ADAM-10, ADAM-12, ADAM-15 and ADAM-17, have been shown to play a role in either cancer formation or progression. Consistent with these findings, increased expression of specific ADAMs in several cancer types was found to correlate with features of aggressive disease and poor prognosis. Currently, selective ADAM inhibitors against ADAM-10 and ADAM-17 are undergoing clinical trials for the treatment of cancer. Further work is required in order to establish a causative role for ADAMs in rheumatoid arthritis, Alzheimer\\'s disease, cardiac hypertrophy and asthma.

  2. Narcolepsy: Pathophysiology and Neuropsychological Changes

    Directory of Open Access Journals (Sweden)

    Angela Naumann

    2003-01-01

    Full Text Available Narcolepsy is now recognized as a distinctive disorder with specific pathophysiology and neurochemical abnormalities. Findings on the role of the neuropeptide hypocretin are opening new avenues of research and new strategies for therapy. Recently, neuropsychological and electrophysiological studies have provided evidence for reduced memory performance on standard memory tests in addition to subjective complaints of forgetfulness which may be related to changes in attentional processing. Further studies are, however, necessary to clarify the neuropsychological profile in narcolepsy. This review focuses on the recent advances in understanding narcolepsy.

  3. Molecular pathophysiology of cerebral edema.

    Science.gov (United States)

    Stokum, Jesse A; Gerzanich, Volodymyr; Simard, J Marc

    2016-03-01

    Advancements in molecular biology have led to a greater understanding of the individual proteins responsible for generating cerebral edema. In large part, the study of cerebral edema is the study of maladaptive ion transport. Following acute CNS injury, cells of the neurovascular unit, particularly brain endothelial cells and astrocytes, undergo a program of pre- and post-transcriptional changes in the activity of ion channels and transporters. These changes can result in maladaptive ion transport and the generation of abnormal osmotic forces that, ultimately, manifest as cerebral edema. This review discusses past models and current knowledge regarding the molecular and cellular pathophysiology of cerebral edema.

  4. Can Psychological, Social and Demographical Factors Predict Clinical Characteristics Symptomatology of Bipolar Affective Disorder and Schizophrenia?

    Science.gov (United States)

    Maciukiewicz, Malgorzata; Pawlak, Joanna; Kapelski, Pawel; Łabędzka, Magdalena; Skibinska, Maria; Zaremba, Dorota; Leszczynska-Rodziewicz, Anna; Dmitrzak-Weglarz, Monika; Hauser, Joanna

    2016-09-01

    Schizophrenia (SCH) is a complex, psychiatric disorder affecting 1 % of population. Its clinical phenotype is heterogeneous with delusions, hallucinations, depression, disorganized behaviour and negative symptoms. Bipolar affective disorder (BD) refers to periodic changes in mood and activity from depression to mania. It affects 0.5-1.5 % of population. Two types of disorder (type I and type II) are distinguished by severity of mania episodes. In our analysis, we aimed to check if clinical and demographical characteristics of the sample are predictors of symptom dimensions occurrence in BD and SCH cases. We included total sample of 443 bipolar and 439 schizophrenia patients. Diagnosis was based on DSM-IV criteria using Structured Clinical Interview for DSM-IV. We applied regression models to analyse associations between clinical and demographical traits from OPCRIT and symptom dimensions. We used previously computed dimensions of schizophrenia and bipolar affective disorder as quantitative traits for regression models. Male gender seemed protective factor for depression dimension in schizophrenia and bipolar disorder sample. Presence of definite psychosocial stressor prior disease seemed risk factor for depressive and suicidal domain in BD and SCH. OPCRIT items describing premorbid functioning seemed related with depression, positive and disorganised dimensions in schizophrenia and psychotic in BD. We proved clinical and demographical characteristics of the sample are predictors of symptom dimensions of schizophrenia and bipolar disorder. We also saw relation between clinical dimensions and course of disorder and impairment during disorder.

  5. Melancholic features in inpatients with major depressive disorder associate with differential clinical characteristics and treatment outcomes.

    Science.gov (United States)

    Lin, Ching-Hua; Huang, Chun-Jen; Liu, Shi-Kai

    2016-04-30

    To determine whether the presence of melancholic features in hospitalized patients with major depressive disorder (MDD) was associated with specific clinical characteristics and treatment outcomes, supporting melancholic depression as a distinct subtype within MDD. 126 acutely ill inpatients with MDD were enrolled in an open, 6-week trial with fixed-dose fluoxetine 20mg daily. Symptom severity was assessed regularly, using the 17-item Hamilton Depression Rating Scale (HAMD-17) and Clinical Global Impression of Severity (CGI-S). Melancholic features were defined according to the DSM-IV criteria. Clinical variables were compared between patients with and without melancholic features. Generalized estimating equations method was used to explore the differences in HAMD-17 and CGI-S scores between the 2 groups over time. Clinical response was defined as having a 50% or greater reduction in HAMD-17 scores. 96 (76.2%) of the 126 patients with at least one post-baseline assessment met the criteria for melancholic depression. Melancholic depression differed from non-melancholic depression in clinical characteristics and predicted a better response to fluoxetine treatment. The differentiation between melancholic and non-melancholic depression within MDD hence is clinically significant and valid.

  6. Pathophysiology of the Belgrade Rat

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    Tania eVeuthey

    2014-04-01

    Full Text Available The Belgrade rat is an animal model of Divalent Metal Transporter-1 (DMT1 deficiency. This strain originates from an X-irradiation experiment first reported in 1966. Since then, the Belgrade rat’s pathophysiology has helped to reveal the importance of iron balance and the role of DMT1. This review discusses our current understanding of iron transport homeostasis and summarizes molecular details of DMT1 function. We describe how studies of the Belgrade rat have revealed key roles for DMT1 in iron distribution to red blood cells as well as duodenal iron absorption. The Belgrade rat’s pathology has extended our knowledge of hepatic iron handling, pulmonary and olfactory iron transport as well as brain iron uptake and renal iron handling. For example, relationships between iron and manganese metabolism have been discerned since both are essential metals transported by DMT1. Pathophysiologic features of the Belgrade rat provide us with a unique and interesting animal model to understand iron homeostasis.

  7. Clinical characteristics of early- and late-onset gout: A cross-sectional observational study from a Chinese gout clinic.

    Science.gov (United States)

    Zhang, Bingqing; Fang, Weigang; Zeng, Xuejun; Zhang, Yun; Ma, Ya; Sheng, Feng; Zhang, Xinlei

    2016-11-01

    A retrospective cross-sectional study using data from an outpatient clinic in China was conducted to investigate the clinical features of early-onset gout patients.All patients diagnosed with gout were asked about clinical characteristics of their gout and comorbid diseases. Patients presenting with acute flares were asked about common triggers before the flare. "Early-onset" gout was defined as onset of gout before 40 years and "late-onset" as onset ≥40 years. Major joint involvement, flare frequency before presentation, the cumulative number of involved joints, proportions of tophi complications at presentation, flare triggers, as well as any metabolic, cardiovascular, cerebrovascular, and renal comorbidities, were compared between the 2 groups.A total of 778 gout patients were enrolled in this study, including 449 (57.7%) in the early-onset group and 329 (42.3%) in the late-onset group. Compared with the late-onset gout patients, the early-onset gout patients had a higher proportion of ankle/mid-foot involvement (62.8% vs 48.2%, P gout patients had fewer metabolic, cardiovascular, cerebrovascular, or renal complications.Early- and late-onset gout patients had different clinical features. Early-onset seems to be influenced more by lifestyle, while late-onset patients have more complications because of comorbidities.

  8. Classic Kaposi’s sarcoma: The clinical, demographic and teratment characteristics of seventy-four patients

    Directory of Open Access Journals (Sweden)

    Beril Gülüş Demirel

    2016-12-01

    Full Text Available Background and Design: Classic Kaposi's sarkoma (CKS is a rare disease, generally seen across Mediterranean and the Middle East region. It's an angioproliferative disorder associated with human herpes virus-8 infection. There is a few data on epidemiology and clinical characteristics among Turkish patients with CKS. This study aims to evaluate epidemiologic, clinical characteristics and treatment results in patients with the diagnosis of CKS in Zonguldak. Materials and Methods: We retrospectively evaluated the hospital records of patients with CKS who attended the dermatological and venereal diseases department between 2003 and 2014. Seventy-four patients were included in this study. Demographic and clinical characteristics, applied treatments and responses to treatments were evaluated. Results: During the eleven year examination period, 74 CKS patients have been diagnosed in the dermatology clinic. The prevalence of CKS among dermatologic patients was found to be 0.02%. Patient age at diagnosis ranged from 33 to 90 years (mean: 70.2±11.7. Fifty-two patients were male (70.3% and 22 patients were female (29.7%. Multiple nodules were the most frequently seen clinical forms and the distal lower extremity was the most common site of involvement (80.6%. According to the CKS staging system, it was observed that 47 patients (62.7% were at stage 1, 11 patients (15.49% at stage 2, eight patients (10.7% at stage 3, and six patients (8% were at stage 4. Treatment options were excision for 35.1% of patients (n=26, radiotherapy for 25.7% of patients (n=19, cryosurgery for 14.9% of patients (n=11, and chemotherapy for 10.8% of patients (n=8. Relapse was found to occur most commonly after excision (58.3%. Conclusion: Larger, multicenter studies are needed in order to determine the prevalence of CKS and characteristics of patients with CKS in our country.

  9. Hyperprolactinemia: pathophysiology and therapeutic approach.

    Science.gov (United States)

    Capozzi, Anna; Scambia, Giovanni; Pontecorvi, Alfredo; Lello, Stefano

    2015-07-01

    Prolactin (PRL) is a hormone, mainly secreted by lactotroph cells of the anterior pituitary gland. Recent studies have shown it may also be produced by many extrapituitary cells. Its well-recognized PRL plays an important role in lactation during pregnancy, but it is involved in other biological functions such as angiogenesis, immunoregulation and osmoregulation. Hyperprolactinemia is a typical condition producing reproductive dysfunction in both sexes, resulting in hypogonadism, infertility and galactorrhea. It may be also asymptomatic. Lactotroph adenomas (prolactinoma) is one of the most common cause of PRL excess, representing approximately 40% of all pituitary tumors. Several other conditions should be excluded before a clear diagnosis of hyperprolactinemia is made. Hyperprolactinemia may be secondary to pharmacological or pathological interruption of hypothalamic-pituitary dopaminergic pathways or idiopathic. Stress, renal failure or hypothyroidism are other frequent conditions to exclude in patients with hyperprolactinemia. We will review biochemical characteristics and physiological functions of that hormone. Clinical and pharmacological approach to hyperprolactinemia will also be discussed.

  10. Self-perceived health and clinical characteristics in young adult students from the brazilian northeast

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    Thereza Maria Magalhães Moreira

    2014-10-01

    Full Text Available Objective To analyze the association between socioeconomic situation, clinical characteristics referred and the family history of cardiovascular disease, with the Self-perceived health of young adults education and their implications for clinical characteristics observed. Method Analytical study conducted with 501 young adults who are students in countryside city in the Brazilian Northeast. We used binary logistic regression. Results The final model explained 83.3% of the self-perceived positive health, confirming the association of Self-perceived health with male, residence in the community, have excellent/very good lifestyle and does not have or do not know that there are cases of stroke in the family. Conclusion Health perception was often optimistic, being important to identify devices to be worked closer to their perception of their actual health condition, increasing the effectiveness of health promotion activities undertaken by professionals.

  11. The influence of job characteristics on job outcomes of pharmacists in hospital, clinic, and community pharmacies.

    Science.gov (United States)

    Lin, Blossom Yen-Ju; Yeh, Ying-Chen; Lin, Wen-Hung

    2007-06-01

    This study examines the relationship between job characteristics and job outcomes of pharmacists in hospital, clinic, and community pharmacies in Taiwan. The structured questionnaires covered the items of job characteristics, job outcomes, and demographics of pharmacists, and were distributed between Feb 2004 and April 2004. Two hundred and ninety-eight pharmacists responded. Data were analyzed descriptively, and univariate analyses, factor analysis, and multiple regression analyses were used. It found the more enriched the job, the greater the job satisfaction and less intention to leave. And community pharmacists reported greater job enrichment and job satisfaction and less intention to leave than did hospital and clinic pharmacists. It suggests pharmacy managers could recognize the needs of pharmacists to redesign and enrich their work arrangements.

  12. Etiology of aseptic meningitis and clinical characteristics in immune-competent adults.

    Science.gov (United States)

    Han, Su-Hyun; Choi, Hye-Yeon; Kim, Jeong-Min; Park, Kwang-Ryul; Youn, Young Chul; Shin, Hae-Won

    2016-01-01

    Viral meningitis is the most common cause of aseptic meningitis. Use of the polymerase chain reaction (PCR) has increased the ability to determine the etiology of viral meningitis. This study used PCR analysis to evaluate the etiology of aseptic meningitis in 177 previously healthy adults over a 5-year period, as well as analyzing the clinical characteristics, cerebrospinal fluid (CSF) findings, and prognosis according to each etiology. The most frequent cause of aseptic meningitis was enterovirus (EV), followed by varicella zoster virus (VZV). Patients with EV meningitis were significantly younger than those with VZV meningitis. The percentage of lymphocytes in white blood cell counts and protein concentrations in the CSF differed significantly among patients with EV, VZV and meningitis of undetermined etiology. Younger age and lower percentage of lymphocyte and protein level in CSF analysis may be suggestive of EV meningitis. Further prospective studies are warranted to identify the correlations between the clinical characteristics and the etiologies of meningitis.

  13. [Clinical characteristics of splenic marginal zone lymphoma with abnormal complete blood count].

    Science.gov (United States)

    Yang, Shen-Miao; Jiang, Qian; Jiang, Bin; Chen, Ding-Bao; Wang, Jing; Jiang, Hao; Lu, Jin; Lu, Xi-Jing; Bao, Li; Shi, Hong-Xia; Liu, Yan-Rong; Huang, Xiao-Jun

    2013-02-01

    The aim of this study was to investigate the clinical and laboratorial characteristics of splenic marginal zone lymphoma (SMZL) with an abnormal complete blood count (CBC). Data of 19 newly diagnosed SMZL patients with abnormal CBC were analyzed retrospectively. Seven patients were diagnosed by using splenic histology, 12 patients who did not undergo splenectomy were diagnosed on the basis of typical clinical presentation and cytologic, immunophenotypic and histologic characteristics of peripheral blood and bone marrow, according to SBLG guidelines. The results showed that leukocytosis (≥ 10.0×10(9)/L) was seen in 5 cases (26.3%); leukocytopenia (complete response was achieved in 9 out of 11 (81.8%) patients. It is concluded that SMZL with abnormal CBC has a higher incidence of cytopenia, bone marrow involvement and autoimmune phenomena. Therapeutic strategies consisting of Rituximab show a better efficacy.

  14. The Structure of Pathological Gambling among Korean Gamblers: A Cluster and Factor Analysis of Clinical and Demographic Characteristics

    Science.gov (United States)

    Lee, Tae Kyung; LaBrie, Richard A.; Grant, Jon E.; Kim, Suck Won; Shaffer, Howard J.

    2008-01-01

    This paper reports the underlying structure of the demographic and clinical characteristics of level 3 (i.e., pathological) Korean casino gamblers. The participants reported their gambling behavior and clinical characteristics known to be associated with gambling problems (e.g., alcohol use problems, eating disorders, depression, anxiety, and…

  15. The clinical characteristics and prognostic analysis of gastric mucosa-associated lymphoid tissue lymphoma of 103 cases

    Institute of Scientific and Technical Information of China (English)

    李晓武

    2012-01-01

    Objective To analyze the clinical characteristics and prognosis of the patients with gastric mucosa-associated lymphoid tissue(MALT) lymphoma. Methods The clinical characteristics and prognostic factors of 103 gastric MALT lymphoma patients admitted to our hospital from April 2001 to August 2011 were

  16. Erythrodermic psoriasis: pathophysiology and current treatment perspectives

    Directory of Open Access Journals (Sweden)

    Singh RK

    2016-07-01

    Full Text Available Rasnik K Singh,1 Kristina M Lee,2 Derya Ucmak,2 Merrick Brodsky,3 Zaza Atanelov,4 Benjamin Farahnik,5 Michael Abrouk,3 Mio Nakamura,2 Tian Hao Zhu,6 Wilson Liao2 1Department of Medicine, University of California – Los Angeles, David Geffen School of Medicine, Los Angeles, 2Department of Dermatology, University of California – San Francisco, San Francisco, 3Department of Medicine, University of California – Irvine, School of Medicine, Irvine, CA, 4Department of Medicine, New York Medical College, Valhalla, NY, 5Department of Medicine, University of Vermont College of Medicine, Burlington, VT, 6Department of Medicine, University of Southern California Keck School of Medicine, Los Angeles, CA, USA Abstract: Erythrodermic psoriasis (EP is a rare and severe variant of psoriasis vulgaris, with an estimated prevalence of 1%–2.25% among psoriatic patients. The condition presents with distinct histopathologic and clinical findings, which include a generalized inflammatory erythema involving at least 75% of the body surface area. The pathogenesis of EP is not well understood; however, several studies suggest that the disease is associated with a predominantly T helper 2 (Th2 phenotype. Given the morbidity and potential mortality associated with the condition, there is a need for a better understanding of its pathophysiology. The management of EP begins with a comprehensive assessment of the patient’s presentation and often requires multidisciplinary supportive measures. In 2010, the medical board of the US National Psoriasis Foundation published consensus guidelines advocating the use of cyclosporine or infliximab as first-line therapy in unstable cases, with acitretin and methotrexate reserved for more stable cases. Since the time of that publication, additional information regarding the efficacy of newer agents has emerged. We review the latest data with regard to the treatment of EP, which includes biologic therapies such as ustekinumab and

  17. Clinical characteristics and primary management of patients diagnosed with prostate cancer between 2007 and 2013

    DEFF Research Database (Denmark)

    Thomsen, Frederik B; Mikkelsen, Marta K; Hansen, Rikke B;

    2016-01-01

    BACKGROUND: The Danish Cancer Registry holds information on all prostate cancers (PCa) cases, including diagnostic TNM. However, stratification according to contemporary risk classification is not possible because histopathological grading and prostate-specific antigen (PSA) level...... December 2013, were reviewed. Clinical characteristics and primary treatment were recorded. The National Comprehensive Cancer Network risk group classification was used. RESULTS: A total of 1934 men with a median age of 69 years (interquartile range 65-75) were diagnosed with PCa in the study period...

  18. Demographic, clinical and antibody characteristics of patients with digital ulcers in systemic sclerosis

    DEFF Research Database (Denmark)

    Denton, Christopher P; Krieg, Thomas; Guillevin, Loic;

    2012-01-01

    OBJECTIVES: The Digital Ulcers Outcome (DUO) Registry was designed to describe the clinical and antibody characteristics, disease course and outcomes of patients with digital ulcers associated with systemic sclerosis (SSc). METHODS: The DUO Registry is a European, prospective, multicentre......, observational, registry of SSc patients with ongoing digital ulcer disease, irrespective of treatment regimen. Data collected included demographics, SSc duration, SSc subset, internal organ manifestations, autoantibodies, previous and ongoing interventions and complications related to digital ulcers. RESULTS...

  19. Clinical characteristics and epidermal barrier function of papulopustular rosacea: A comparison study with acne vulgaris

    OpenAIRE

    Zhou, Maosong; Xie, Hongfu; Cheng, Lin; Li, Ji

    2016-01-01

    Objective: To evaluate the clinical characteristics and epidermal barrier function of papulopustular rosacea by comparing with acne vulgaris. Methods: Four hundred and sixty-three papulopustular rosacea patients and four hundred and twelve acne vulgaris patients were selected for the study in Xiangya Hospital of Central South University from March 2015 to May 2016. They were analyzed for major facial lesions, self-conscious symptoms and epidermal barrier function. Results: Erythema, burning, ...

  20. The counseling african americans to control hypertension (caatch) trial: baseline demographic, clinical, psychosocial, and behavioral characteristics

    OpenAIRE

    Diaz-Gloster Marleny; Cassells Andrea; Tobin Jonathan N; Fernandez Senaida; Kalida Chamanara; Ogedegbe Gbenga

    2011-01-01

    Abstract Background Effectiveness of combined physician and patient-level interventions for blood pressure (BP) control in low-income, hypertensive African Americans with multiple co-morbid conditions remains largely untested in community-based primary care practices. Demographic, clinical, psychosocial, and behavioral characteristics of participants in the Counseling African American to Control Hypertension (CAATCH) Trial are described. CAATCH evaluates the effectiveness of a multi-level, mu...

  1. [Pruritus: considerable progress in pathophysiology].

    Science.gov (United States)

    Misery, Laurent

    2014-12-01

    Pruritus is defined as "an unpleasant sensation that causes the need to scratch". This is not a small pain. It seems that pruriceptors exist but their level of separation from nociceptive receptors is still debated. Pathways of pruritus were identified from the skin (around the dermo-epidermal junction) to the brain. Many mediators are involved in pruritus but there are at least a histaminergic and a non-histaminergic pathway (PAR-2dependent). Similarly to pain, gate control or peripheral and central sensitization mechanisms have been highlighted in pruritus. These pathophysiological advances are important and anticipate therapeutic advances, that will be very useful for the symptomatic treatment of pruritus (poorly efficient at present).

  2. Dystonia : emerging concepts in pathophysiology.

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    Madhusudanan M

    1999-10-01

    Full Text Available The essential pathophysiological feature of dystonia is co-contraction of antagonistic muscles. This may be due to derangement of the spinal cord or cortical mechanism. In the cord, there is disruption of the normal reciprocal inhibition of antagonists during agonist contraction. This decreased reciprocal inhibition is due to reduced presynaptic inhibition of muscle afferent input to the inhibitory interneuron. The reduced presynaptic inhibition may in turn be either due to defective suprasegmental control or to changes in the tonic afferent input to the interneuron from cutaneous and muscle afferents. Alternatively, genesis of dystonia may entirely be a cortical mechanism. Overactivity of the premotor cortices, which receive projections from basal ganglia via ventral thalamus, could result in dystonia by abnormal activation of cortical motor neurons. This may again be due to a dopaminergic dysfunction of basal ganglia.

  3. Understanding changes in cardiovascular pathophysiology.

    Science.gov (United States)

    Chummun, Harry

    Cardiovascular pathophysiological changes, such as hypertension and enlarged ventricles, reflect the altered functions of the heart and its circulation during ill-health. This article examines the normal and altered anatomy of the cardiac valves, the contractile elements and enzymes of the myocardium, the significance of the different factors associated with cardiac output, and the role of the autonomic nervous system in the heart beat. It also explores how certain diseases alter these functions and result in cardiac symptoms. Nurses can benefit from knowledge of these specific changes, for example, by being able to ask relevant questions in order to ascertain the nature of a patients condition, by being able to take an effective patient history and by being able to read diagnostic results, such as electrocardiograms and cardiac enzyme results. All this will help nurses to promote sound cardiac care based on a physiological rationale.

  4. Pathophysiologic mechanisms of biomedical nanomaterials.

    Science.gov (United States)

    Wang, Liming; Chen, Chunying

    2016-05-15

    Nanomaterials (NMs) have been widespread used in biomedical fields, daily consuming, and even food industry. It is crucial to understand the safety and biomedical efficacy of NMs. In this review, we summarized the recent progress about the physiological and pathological effects of NMs from several levels: protein-nano interface, NM-subcellular structures, and cell-cell interaction. We focused on the detailed information of nano-bio interaction, especially about protein adsorption, intracellular trafficking, biological barriers, and signaling pathways as well as the associated mechanism mediated by nanomaterials. We also introduced related analytical methods that are meaningful and helpful for biomedical effect studies in the future. We believe that knowledge about pathophysiologic effects of NMs is not only significant for rational design of medical NMs but also helps predict their safety and further improve their applications in the future.

  5. Intraductal Oncocytic Papillary Neoplasm Having Clinical Characteristics of Mucinous Cystic Neoplasm and a Benign Histology

    Directory of Open Access Journals (Sweden)

    Takatomi Oku

    2007-03-01

    Full Text Available Context An intraductal oncocytic papillary neoplasm is a rare pancreatic tumor which was first described by Adsay et al. in 1996. It has been defined as a new subgroup of IPMN. Case report We report the case of a 76-year-old woman who presented with nausea. Imaging studies revealed a cystic mass in the body of the pancreas. She underwent a successful distal pancreatectomy and splenectomy, and has subsequently remained well. Microscopically, the cyst was lined by columnar epithelium similar to pancreatic duct epithelium, and the nodular projection consisted of arborizing papillary structures, lined by plump cells with abundant eosinophilic cytoplasm. These eosinophilic cells were immunohistochemically positively stained with anti-mitochondrial antibody. The cellular atypism was mild and the proliferating index was low, compatible with adenoma of an intraductal oncocytic papillary neoplasm. Although no ovarian type stroma was identified, in our case, no communication to main pancreatic duct (located in the pancreatic body and rapid growth by intracystic hemorrhage were clinical characteristics of a mucinous cystic neoplasm, but not IPMN. Conclusion With only 17 cases reported to date, the clinical and pathological details of an intraductal oncocytic papillary neoplasm are still unclear. We herein add one case with different characteristics from those of the past reports. To our knowledge, this is the first case report of an intraductal oncocytic papillary neoplasm with the clinical characteristics of a mucinous cystic neoplasm.

  6. Gender difference of clinical characteristics in Chinese patients with spontaneous variant angina

    Institute of Scientific and Technical Information of China (English)

    ZHU Cheng-gang; GUO Yuan-lin; GAO Zhan; ZHENG Xin; LI Jian-jun; XU Yan-lu; YUAN Jin-qing; QIN Xue-wen; YANG Yue-jin; QIAO Shu-bin; CHEN Ji-lin; CHEN Zai-jia

    2010-01-01

    Background Spontaneous attack of variant angina (VA) is a unique component of coronary artery disease (CAD), and associated with severe cardiac events. However, no data are available regarding sex differences in Chinese patients with spontaneous attacks of VA. Accordingly, the present retrospective study was initiated to evaluate the Clinical characteristics of Chinese female patients with spontaneous attacks of VA.Methods From January 2003 to January 2008, a total of 209 patients were diagnosed to have had a spontaneous attack of VA at Fu Wai Hospital. Of them, 27 were female, and their clinical findings were collected and compared with male patients for aspects of risk factors, clinical features and angiographical findings.Results Spontaneous attacks of VA was relatively uncommon in female (12.9%) compared with male patients. The female patients were less likely to have a history of smoking (14.8% vs. 79.7%, P <0.001), more likely to have a family history of CAD (33.3% vs. 11.0%, P<0.01), and to have had a greater incidence of ventricular fibrillation during attack (11.1% vs. 2.2%, P<0.05). There were no significant differences in other characteristics between the two groups.Conclusion Chinese female patients who experienced a spontaneous attack of VA had the characteristics of less smoking history, more family history of CAD and higher occurrence of ventricular fibrillation than male patients.

  7. Reporting of research quality characteristics of studies published in 6 major clinical dental specialty journals.

    Science.gov (United States)

    Pandis, Nikolaos; Polychronopoulou, Argy; Madianos, Phoebus; Makou, Margarita; Eliades, Theodore

    2011-06-01

    The objective of this article was to record reporting characteristics related to study quality of research published in major specialty dental journals with the highest impact factor (Journal of Endodontics, Journal of Oral and Maxillofacial Surgery, American Journal of Orthodontics and Dentofacial Orthopedics; Pediatric Dentistry, Journal of Clinical Periodontology, and International Journal of Prosthetic Dentistry). The included articles were classified into the following 3 broad subject categories: (1) cross-sectional (snap-shot), (2) observational, and (3) interventional. Multinomial logistic regression was conducted for effect estimation using the journal as the response and randomization, sample calculation, confounding discussed, multivariate analysis, effect measurement, and confidence intervals as the explanatory variables. The results showed that cross-sectional studies were the dominant design (55%), whereas observational investigations accounted for 13%, and interventions/clinical trials for 32%. Reporting on quality characteristics was low for all variables: random allocation (15%), sample size calculation (7%), confounding issues/possible confounders (38%), effect measurements (16%), and multivariate analysis (21%). Eighty-four percent of the published articles reported a statistically significant main finding and only 13% presented confidence intervals. The Journal of Clinical Periodontology showed the highest probability of including quality characteristics in reporting results among all dental journals.

  8. PSA testing without clinical indication for prostate cancer in relation to socio-demographic and clinical characteristics in the Danish Diet, Cancer and Health Study

    DEFF Research Database (Denmark)

    Karlsen, Randi V; Larsen, Signe B; Christensen, Jane;

    2013-01-01

    associations between socio-demographic or clinical characteristics and PSA testing without clinical indication. Material and methods. In the Danish Diet, Cancer and Health Cohort, we identified 1051 men with PC diagnosed in 1993-2008. Diagnostic and clinical characteristics were obtained from medical records......, and socio-demographic information was retrieved from administrative registers. We used general logistic regression analysis to estimate odds ratios (ORs) and 95% confidence intervals (CIs) for associations between socio-demographic or clinical characteristics and PSA testing without clinical indication. Cox......Background. Social differences in prostate cancer (PC) incidence and mortality might be related to testing for prostate-specific antigen (PSA). Although routine PSA screening is not recommended in Denmark, testing without clinical indication increased during the past decade. We evaluated...

  9. Idiopathic Inflammatory Myopathies; Association with Overlap Myositis and Syndromes: Classification, Clinical Characteristics, and Associated Autoantibodies

    Directory of Open Access Journals (Sweden)

    Pari Basharat

    2016-07-01

    Full Text Available Idiopathic inflammatory myopathies (IIM are traditionally identified as a group of disorders that target skeletal muscle due to autoimmune dysfunction. The IIM can be divided into subtypes based on certain clinical characteristics, and several classification schemes have been proposed. The predominant diagnostic criteria for IIM is the Bohan and Peter criteria, which subdivides IIM into primary polymyositis (PM, primary dermatomyositis (DM, myositis with another connective tissue disease, and myositis associated with cancer. However, this measure has been criticised for several reasons including lack of specific criteria to help distinguish between muscle biopsy findings of PM, DM, and immune-mediated necrotising myopathy, as well as the lack of identification of cases of overlap myositis (OM. Because of this issue, other classification criteria for IIM have been proposed, which include utilising myositis-associated antibodies and myositis-specific antibodies, as well as overlap features such as Raynaud’s phenomenon, polyarthritis, oesophageal abnormalities, interstitial lung disease, small bowel abnormalities such as hypomotility and malabsorption, and renal crises, amongst others. Indeed, the identification of autoantibodies associated with certain clinical phenotypes of myositis, in particular connective tissue disease-myositis overlap, has further helped divide IIM into distinct clinical subsets, which include OM and overlap syndromes (OS. This paper reviews the concepts of OM and OS as they pertain to IIM, including definitions in the literature, clinical characteristics, and overlap autoantibodies.

  10. Incidence and clinical characteristics of fungal keratitis in a Danish population from 2000 to 2013

    DEFF Research Database (Denmark)

    Nielsen, Stine E; Nielsen, Esben; Julian, Hanne Olsen;

    2015-01-01

    PURPOSE: Fungal keratitis is a severe sight-threatening condition. The aim of this study was to investigate the incidence and clinical characteristics of fungal keratitis patients living in a temperate climate. METHODS: By reviewing medical records from 2000 to July 2013, patients with fungal...... keratitis were identified. Risk factors, clinical signs and outcome were registered. RESULTS: Twenty-five patients were identified: 52% with Candida, 20% with Fusarium, 16% with Aspergillus and 12% with mixed filamentous fungi. A minimum incidence of fungal keratitis of 0.6 cases per million per year...... with corneal transplantation. Patients with Candida infections had a significantly worse visual outcome. CONCLUSION: We found that patients with fungal keratitis had a poor visual outcome. However, knowledge of risk factors and clinical signs leading to early treatment can improve the prognosis....

  11. Asthma characteristics and biomarkers from the Airways Disease Endotyping for Personalized Therapeutics (ADEPT) longitudinal profiling study

    DEFF Research Database (Denmark)

    Silkoff, P E; Strambu, I; Laviolette, M;

    2015-01-01

    BACKGROUND: Asthma is a heterogeneous disease and development of novel therapeutics requires an understanding of pathophysiologic phenotypes. The purpose of the ADEPT study was to correlate clinical features and biomarkers with molecular characteristics, by profiling asthma (NCT01274507). This re......BACKGROUND: Asthma is a heterogeneous disease and development of novel therapeutics requires an understanding of pathophysiologic phenotypes. The purpose of the ADEPT study was to correlate clinical features and biomarkers with molecular characteristics, by profiling asthma (NCT01274507......, were stable over 12 months. Use of the ADEPT data should prove useful in defining biological phenotypes to facilitate personalized therapeutic approaches....

  12. Predictor characteristics necessary for building a clinically useful risk prediction model: a simulation study

    Directory of Open Access Journals (Sweden)

    Laura Schummers

    2016-09-01

    Full Text Available Abstract Background Compelled by the intuitive appeal of predicting each individual patient’s risk of an outcome, there is a growing interest in risk prediction models. While the statistical methods used to build prediction models are increasingly well understood, the literature offers little insight to researchers seeking to gauge a priori whether a prediction model is likely to perform well for their particular research question. The objective of this study was to inform the development of new risk prediction models by evaluating model performance under a wide range of predictor characteristics. Methods Data from all births to overweight or obese women in British Columbia, Canada from 2004 to 2012 (n = 75,225 were used to build a risk prediction model for preeclampsia. The data were then augmented with simulated predictors of the outcome with pre-set prevalence values and univariable odds ratios. We built 120 risk prediction models that included known demographic and clinical predictors, and one, three, or five of the simulated variables. Finally, we evaluated standard model performance criteria (discrimination, risk stratification capacity, calibration, and Nagelkerke’s r2 for each model. Results Findings from our models built with simulated predictors demonstrated the predictor characteristics required for a risk prediction model to adequately discriminate cases from non-cases and to adequately classify patients into clinically distinct risk groups. Several predictor characteristics can yield well performing risk prediction models; however, these characteristics are not typical of predictor-outcome relationships in many population-based or clinical data sets. Novel predictors must be both strongly associated with the outcome and prevalent in the population to be useful for clinical prediction modeling (e.g., one predictor with prevalence ≥20 % and odds ratio ≥8, or 3 predictors with prevalence ≥10 % and odds ratios ≥4. Area

  13. Tinnitus: Network pathophysiology-network pharmacology

    Directory of Open Access Journals (Sweden)

    Ana Belen eElgoyhen

    2012-01-01

    Full Text Available Tinnitus, the phantom perception of sound, is a prevalent disorder. One in 10 adults has clinically significant subjective tinnitus, and for 1 in 100, tinnitus severely affects their quality of life. Despite the significant unmet clinical need for a safe and effective drug targeting tinnitus relief, there is currently not a single FDA-approved drug on the market. The search for drugs that target tinnitus is hampered by the lack of a deep knowledge of the underlying neural substrates of this pathology. Recent studies are increasingly demonstrating that, as described for other central nervous system disorders, tinnitus is a pathology of brain networks. The application of graph theoretical analysis to brain networks has recently provided new information concerning their topology, their robustness and their vulnerability to attacks. Moreover, the philosophy behind drug design and pharmacotherapy in central nervous system pathologies is changing from that of magic bullets that target individual chemoreceptors or disease-causing genes into that of magic shotguns, promiscuous or dirty drugs that target disease-causing networks, also known as network pharmacology. In the present work we provide some insight into how this knowledge could be applied to tinnitus pathophysiology and pharmacotherapy.

  14. [Vestibular neuronitis: pathophysiology, diagnosis and treatment].

    Science.gov (United States)

    Zaper, Dinka; Adamec, Ivan; Gabelić, Tereza; Krbot, Magdalena; Isgum, Velimir; Hajnsek, Sanja; Habek, Mario

    2012-01-01

    Vestibular neuritis (VN) is one of the most common causes of peripheral vertigo. Caloric testing has been the traditional gold standard for detecting a peripheral vestibular deficit, but some recently developed bedside tests (head thrust, head heave, head shake and vibration test) were evaluated as a good alternative with similar sensitivity and specificity. These tests have shown both diagnostic value in the short term and prognostic value in the long term, and have availability and ease of use as an advantage. As an addition to clinical examination, vestibular evoked myogenic potentials can differentiate between involvement of superior and inferior branch of the vestibular nerve, but also between peripheral and central lesions. Although glucocorticoids are currently widely used in the treatment of VN, there is a lack of evidence for the validity of their administration. There are a number of high quality clinical trials that suggest vestibular rehabilitation exercises, which are based on the mechanisms of vestibular compensation, in the managment of VN. This review will focus on the latest developments in the pathophysiology, diagnosis and treatment of patients with VN.

  15. Pathophysiology and imaging in inflammatory and blastomatous synovial diseases

    Energy Technology Data Exchange (ETDEWEB)

    Imhof, H.; Noebauer-Huhmann, I.-M.; Gahleitner, A.; Kainberger, F.; Krestan, C.; Trattnig, S. [Osteology, Universitaets Klinik fuer Radiodiagnostik, AKH Vienna (Austria); Sulzbacher, I. [Klinisches Institut fuer klinische Pathologie, AKH Vienna (Austria)

    2002-06-01

    Variable pathologies are subsumed under the term ''synovial disease'', including common pathologies such as rheumatoid arthritis. While formerly radiologists had to rely on conventional radiographs and bone scintigraphy with their inherent problems in visualizing soft tissue, noninvasive imaging of the synovium has recently improved substantially with the technical development of MRI and (Doppler) ultrasound. These imaging modalities allow differentiation of characteristic pathologic features based on a profound knowledge of normal anatomy and pathophysiology. (orig.)

  16. Clinical characteristics associated with Borrelia burgdorferi sensu lato skin culture results in patients with erythema migrans.

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    Franc Strle

    Full Text Available Clinical characteristics associated with isolation of Borrelia burgdorferi sensu lato from skin have not been fully evaluated. To gain insight into predictors for a positive EM skin culture, we compared basic demographic, epidemiologic, and clinical data in 608 culture-proven and 501 culture-negative adult patients with solitary EM. A positive Borrelia spp. skin culture was associated with older age, a time interval of >2 days between tick bite and onset of the skin lesion, EM ≥ 5 cm in diameter, and location of the lesion on the extremities, whereas several other characteristics used as clinical case definition criteria for the diagnosis of EM (such as tick bite at the site of later EM, information on expansion of the skin lesion, central clearing were not. A patient with a 15-cm EM lesion had almost 3-fold greater odds for a positive skin culture than patients with a 5-cm lesion. Patients with a free time interval between the tick bite and onset of EM had the same probability of a positive skin culture as those who did not recall a tick bite (OR=1.02; however, the two groups had >3-fold greater odds for EM positivity than patients who reported a tick bite with no interval between the bite and onset of the lesion. In conclusion, several yet not all clinical characteristics used in EM case definitions were associated with positive Borrelia spp. skin culture. The findings are limited to European patients with solitary EM caused predominantly by B. afzelii but may not be valid for other situations.

  17. Behçet’s disease: The clinical and demographic characteristics of 182 patients

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    Ercan Karabacak

    2014-09-01

    Full Text Available Background and Design: Behçet’s disease (BD is a multisystem inflammatory disorder that is an important cause of morbidity worldwide. In this study, we aimed to investigate the clinical and demographic characteristics of Turkish patients diagnosed with BD. Materials and Methods: We retrospectively evaluated the hospital records of patients with BD who attended the Dermatoveneorology Department at the GATA Haydarpaşa Teaching and Research Hospital between 2001 and 2012. One hundred eighty-two male patients who met the diagnostic criteria of the International Study Group for BD were included in the study. The clinical and demographic characteristics, including symptoms and age at onset, systemic symptoms, duration of the disease and initial signs were recorded. Results: The ages of the 182 male patients included in the study varied from 20 to 53. The mean age at disease onset was 20.59±4.55 years. The leading clinical features were aphthous ulcers (100% followed by genital ulcers (86.8% and papulopustular lesions (80.3%. The pathergy test was positive in 57.3% of the patients. Ocular involvement was present in 527%, vascular involvement in 22.5%, and neurological manifestations was found in 7.1% of the patients. Joint involvement was reported in 18.1% of the subjects. Only 0.5% had genitourinary involvement. There was no relationship of BD with the age at onset and disease duration. Conclusion: We assume that the present study will contribute to the data on demographic and clinical characteristics of especially male BD patients in Turkey

  18. Streptozotocin-Induced Diabetes Models: Pathophysiological Mechanisms and Fetal Outcomes

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    D. C. Damasceno

    2014-01-01

    Full Text Available Glucose homeostasis is controlled by endocrine pancreatic cells, and any pancreatic disturbance can result in diabetes. Because 8% to 12% of diabetic pregnant women present with malformed fetuses, there is great interest in understanding the etiology, pathophysiological mechanisms, and treatment of gestational diabetes. Hyperglycemia enhances the production of reactive oxygen species, leading to oxidative stress, which is involved in diabetic teratogenesis. It has also been suggested that maternal diabetes alters embryonic gene expression, which might cause malformations. Due to ethical issues involving human studies that sometimes have invasive aspects and the multiplicity of uncontrolled variables that can alter the uterine environment during clinical studies, it is necessary to use animal models to better understand diabetic pathophysiology. This review aimed to gather information about pathophysiological mechanisms and fetal outcomes in streptozotocin-induced diabetic rats. To understand the pathophysiological mechanisms and factors involved in diabetes, the use of pancreatic regeneration studies is increasing in an attempt to understand the behavior of pancreatic beta cells. In addition, these studies suggest a new preventive concept as a treatment basis for diabetes, introducing therapeutic efforts to minimize or prevent diabetes-induced oxidative stress, DNA damage, and teratogenesis.

  19. Chronic pain disorders in HIV primary care: clinical characteristics and association with healthcare utilization.

    Science.gov (United States)

    Jiao, Jocelyn M; So, Eric; Jebakumar, Jebakaran; George, Mary Catherine; Simpson, David M; Robinson-Papp, Jessica

    2016-04-01

    Chronic pain is common in HIV, but incompletely characterized, including its underlying etiologies, its effect on healthcare utilization, and the characteristics of affected patients in the HIV primary care setting. These data are needed to design and justify appropriate clinic-based pain management services. Using a clinical data warehouse, we analyzed one year of data from 638 patients receiving standard-of-care antiretroviral therapy in a large primary care HIV clinic, located in the Harlem neighborhood of New York City. We found that 40% of patients carried one or more chronic pain diagnoses. The most common diagnoses were degenerative musculoskeletal disorders (eg, degenerative spinal disease and osteoarthritis), followed by neuropathic pain and headache disorders. Many patients (16%) had multiple chronic pain diagnoses. Women, older patients, and patients with greater burdens of medical illness, and psychiatric and substance use comorbidities were disproportionately represented among those with chronic pain diagnoses. Controlling for overall health status, HIV patients with chronic pain had greater healthcare utilization including emergency department visits and radiology procedures. In summary, our study demonstrates the high prevalence of chronic pain disorders in the primary care HIV clinic. Colocated interventions for chronic pain in this setting should not only focus on musculoskeletal pain but also account for complex multifaceted pain syndromes, and address the unique biopsychosocial features of this population. Furthermore, because chronic pain is prevalent in HIV and associated with increased healthcare utilization, developing clinic-based pain management programs could be cost-effective.

  20. [Clinical characteristics of histoplamosis in 8 patients: case report and literature review].

    Science.gov (United States)

    Zhou, Li; Fan, Songqing; Liang, Qingchun; Peng, Yating; Zong, Dandan; Ouyang, Ruoyun

    2016-06-28

    To explore the clinical characteristics, imaging manifestation, diagnosis and treatment for histoplasmosis and to improve therapeutic level, we retrospectively analyzed the clinical data of 8 patients with biopsy-confirmed histoplasmosis from 2004 to 2014 in the Second Xiangya Hospital of Central South University and reviewed relevant literatures. The main clinical symptoms of histoplasmosis included fever, cough, expectoration, chest pain, blood-stained sputum, lymphadenectasis, etc. The major lung imaging features were mass, node or pneumonia-like performance. No case was diagnosed as histoplasimosis firstly. Four patients whose imaging manifestations were focal pulmonary lesion received lobectomy of lung lesions or wedge resection. Clinical and imaging manifestations in 3 patients, who treated with amphotericin B or its liposomal, itraconazole or fluconazole, were improved. The clinical symptoms and imaging findings of histoplasmosis are nonspecific. It is easy for the physicians to misdiagnose histoplasmosis as bacterial infection, lung cancer, tuberculosis lymphoma, etc. Therefore, it is significant and necessary to carry out multiple biopsies combined with multiple etiological examinations for patients with difficult diagnosis.

  1. Clinical characteristics, diagnosis and management of respiratory distress syndrome in full-term neonates

    Institute of Scientific and Technical Information of China (English)

    FENG Zhi-chun; SHI Yun; DONG Jian-ying; ZHENG Tian; LI Jing-ya; LU Li-li; LIU Jing-jing; LIANG Jing; ZHANG Hao

    2010-01-01

    Background Respiratory distress syndrome (RDS) is one of the most common causes of neonatal respiratory failure and neonatal death, however, its clinical characteristics are very different from premature RDS, and these characteristics have not been well documented as yet. This study was to investigate the pathogenesis, clinical characteristics and management strategies of RDS in full-term neonates, with the aim of developing a working protocol for improving the outcome in full-term neonates with RDS.Methods A total of 125 full-term infants with RDS were enrolled in this study. Their clinical and laboratory data were collected for analyzing the characteristics of full-term neonatal RDS.Results (1) The 125 cases included 94 male and 31 female infants, vaginal delivery occurred in 80 cases and cesarean section in 45 cases. (2) The onset time of RDS was (3.11±3.59) hours after birth. (3)The possible reasons included severe perinatal infections in 63 patients, elective cesarean section in 34 cases, severe birth asphyxia in 12 patients,meconium aspiration syndrome in 9 patients, pulmonary hemorrhage in 4 patients and matemal diabetes in 3 patients. (4)Complications included multiple organ system failure (MOSF) in 49 patients, persistent pulmonary hypertension of newborn (PPHN) in 25 patients, acute renal failure in 18 patients, severe hyperkalemia in 25 patients, severe metabolic acidosis in 6 cases, severe myocardial injury in 9 cases, pulmonary hemorrhage in 3 cases, disseminated intravascular coagulation in 14 patients and shock in 12 patients.(5) Four patients died, the mortality was therefore 3.2% with the main cause of septicemia complicating of MOSF, but their prognosis was improved while comprehensive treatment measures including early mechanical ventilation and broad spectrum antibiotics were taken into account.Conclusions RDS is not an uncommon disease in full-term infants and is associated with a higher mortality, its clinical characteristics are very

  2. Clinical Characteristics, Radiological Features and Gene Mutation in 10 Chinese Families with Spinocerebellar Ataxias

    Institute of Scientific and Technical Information of China (English)

    Jian-Wen Chen; Li Zhao; Feng Zhang; Lan Li; Yu-Hang Gu; Jing-Yuan Zhou; Hui Zhang

    2015-01-01

    Background:Spinocerebellar ataxias (SCAs) are a group ofneurodegenerative disorders that primarily cause the degeneration in the cerebellum,spinal cord,and brainstem.We study the clinical characteristics,radiological features and gene mutation in Chinese families with SCAs.Methods:In this study,we investigated 10 SCAs Chinese families with SCA1,SCA3/Machado-Joseph disease (MJD),SCA7,SCAB.There were 27 people who were genetically diagnosed as SCA,of which 21 people showed clinical symptoms,and 6 people had no clinical phenotype that we called them presymptomatic patients.In addition,3 people with cerebellar ataxia and cataracts were diagnosed according to the Harding diagnostic criteria but failed to be recognized as SCAs on genetic testing.Clinical characteristic analyses of each type of SCAs and radiological examinations were performed.Results:We found that SCA3/MJD was the most common subtype in Han population in China,and the ratio of the pontine tegmentum and the posterior fossa area was negatively correlated with the number of cytosine-adenine-guanine (CAG) repeats;the disease duration was positively correlated with the International Cooperative Ataxia Rating Scale score;and the CAG repeats number of abnormal alleles was negatively correlated with the age of onset.Conclusions:Collectively our study is a systematic research on SCAs in China,which may help for the clinical diagnosis and prenatal screening of this disease,and it may also aid toward better understanding of this disease.

  3. Clinical and Epidemiological Clinical and Epidemiological Characteristics of Colloid Goiter Patients Undergoing Reoperation for Recurrent Thyroid Disease

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    Gladys Iglesias Díaz

    2015-11-01

    Full Text Available Background: thyroid disease is one of the most prevalent medical conditions and thyroid cancer is the most common endocrine neoplasm. Thyroid reoperations are more common than it might seem.Objective: to establish the clinical and epidemiological characteristics of patients operated on for colloid goiter undergoing reoperation for recurrent thyroid disease.Methods: a case-series study was conducted at the León Cuervo Rubio Clinical-Surgical Hospital in Pinar del Rio. The sample included all patients (n = 29 who underwent thyroid operation and were reoperated on for recurrent thyroid disease in 2010, 2011 and 2012. The information was obtained from medical records and the register of histopathological diagnoses.Results: most patients (27.6% were in the 40-49 age group and females (79.3% were most affected. Recurrence was observed 5 to 9 years after surgery. Mixed nodules (55.2% followed by cysts (17.2% were the most frequent ultrasound diagnoses. Recurrent thyroid disease was malignant in 9% of patients.Conclusions: patients reoperated on for thyroid disease were mostly females in the fourth and fifth decade of life. The disease recurred in a period of 10 years after the first surgery in a greater number of cases. Follicular-patterned lesions, colloid goiter and cancer were the most common histologic diagnosis.

  4. Clinical characteristics and treatment outcomes in 132 patients with malignant mesothelioma

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    Abdurrahman Abakay

    2011-01-01

    Full Text Available Purpose: Our objective is to scrutinize clinical, laboratory, radiological characteristics, treatment regimens, and treatment outcomes of malignant mesothelioma (MM cases in our hospital. Materials and Methods: We investigated, retrospectively, the clinical characteristics and treatment outcomes of all 132 MM patients at Dicle University Hospital between January 2006 and April 2010. Results: A total of 82 (62.1% patients were male, and 50 (37.9% female. Median age was 56.0 years. Mean survival time was 9.6±6.9 months. Mean survival time of patients who had received best supportive care was 7.5 months, chemotherapy 10.4 months, and multimodality treatment regimen 12.6 months. Patients in the multimodality treatment group survived longer than did those in the other two groups (P=0.042. A total of 76 patients received chemotherapy, of whom 17 (22.3% were administered Cisplatin/Carboplatin and Gemcitabine, 58 (76.4% Cisplatin/Carboplatin and Pemetrexed, and one (1.3% Cisplatin + Docetaxel. Complete and partial response to treatment in patients receiving Cisplatin/Carboplatin and Gemcitabine was found 47.1% and Cisplatin/Carboplatin and Pemetrexed was found 50.0% (P>0.05. Conclusions: MM related to asbestos exposure is seen frequently in Turkey. Patients present with the typical clinical features of dyspnea, weight loss, and chest pain. Survival analysis shows that patients receiving multimodality treatment may be better.

  5. Clinical and pathological characteristics of septum pellucidum tumor and choice of surgical approaches for its resection

    Institute of Scientific and Technical Information of China (English)

    WANG Lei; ZHANG Mao-zhi; ZHANG Wei; ZHAO Shang-feng; ZHAO Ji-zong; JIA Jin-xiu

    2005-01-01

    Background Tumor involving the septum pellucidum is uncommon. Surgery as the main therapeutic procedure for this lesion is a challenge to neurosurgeons. We analyzed the clinical characteristics and pathological features of septum pellucidum tumor in 41 patients and compared the curative effects of frontal transcortical, trans-sulcal and interhemispheric transcallosal approaches. Methods Clinical characteristics and the pathological features of septum pellucidum tumor were investigated retrospectively in 41 patients. The differences in postoperative residual rates, extents of tumors and resection of normal brain tissues after use of the three approaches in these patients were analyzed statistically. Results Septum pellucidum tumor is more likely to attack young or middle-aged persons. The tumor mainly presents itself as a central neurocytoma or cerebral low-grade glioma in pathology and manifests as intracranial hypertension clinically. No difference was found in the extent of tumor resection but significant difference in the extent of normal brain tissue resection and in postoperative disability rate among the three approaches. The transcortical approach brought about the most serious injury to brain tissue and the highest disability rate, Whereas the frontal transcallosal approach the lightest injury and the lowest disability rate. The injury to brain tissue and the disability rate brought about by the front trans-sulcus approach were between the above two approaches. Conclusions Operation is still regarded the major treatment for septum pellucidum tumor. Transcallosal and trans-sulcus approaches are fit with the concept of minimally invasive surgery, and transcallosal approach is the first choice for septum pellucidum tumor.

  6. Patients with Helicobacter pylori positive and negative duodenal ulcers have distinct clinical characteristics

    Institute of Scientific and Technical Information of China (English)

    Kent-Man Chu; Ka-Fai Kwok; Simon Law; Kam-Ho Wong

    2005-01-01

    AIM: To assess the clinical characteristics of Helicobacterpylori(H pylori) negative duodenal ulcer.METHODS: Patients with an endoscopic diagnosis of duodenal ulcer between 1996 and 2002 were included in the present study. Patients were considered to be negative for Hpylori, if both histological examination and rapid urease test of biopsy specimens were negative. A comparison was made between patients with H pyloripositive and negative duodenal ulcers.RESULTS: A total of 1 343 patients were studied. Their mean age was 54.7±0.5 years. There was a male preponderance (M:F = 2.5:1). Three hundred and ninetyeight patients (29.6%) did not have H pylori infection. The annual proportion of patients with H pylori negative duodenal ulcers increased progressively from 1996 to2002. On multivariate analysis, patients with H pylorinegative duodenal ulcer were more likely to be older, have concomitant medical problem, pre-existing malignancy, recent surgery, underlying sepsis, or taken non-steroidal anti-inflammatory drugs. In terms of clinical presentations, patients with H pylori negative duodenal ulcer were more likely to present with bleeding, multiple ulcers and larger ulcers.CONCLUSION: The proportion of patients with H pylori negative duodenal ulcers is on the rise because of a continued drop in incidence of H pylori positive duodenalulcers in recent years. Such patients have distinct clinical characteristics and it is important to ascertain the H pylori status before starting eradication therapy.

  7. Microdosimetric Characteristics of the Clinical Proton Beams at the JINR Phasotron, Dubna

    CERN Document Server

    Vlcek, B; Spurny, F

    2002-01-01

    The contribution of the high LET particles to dosimetric and microdosimetric characteristics of 150 and 205 MeV clinical proton beams was experimentally studied using track etched detectors. Secondary heavy charged particles produced from nuclear interactions and degraded protons at the Bragg peak region are particles with high LET. The method of the LET spectra measurement with track etched detectors allows one to determine the contribution of high LET particles to dosimetric characteristics of clinical proton beams: absorbed dose, equivalent dose and the value of the Relative Biological Effectiveness (RBE). Track detectors were irradiated in the various depth of clinical proton beams with the primary energies of 150 and 205 MeV. The LET spectra between 10 and 700 keV/m were measured by means of CR-39 track etched detectors and the automatic optical image analyzer LUCIA-II. The relative contribution of the high LET particles to absorbed dose increases from several per cent at the beam entrance to several ten...

  8. Sociodemographic and Clinical Characteristics of Patients attending Psychotherapy in a Tertiary Care Hospital in Oman

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    Zena Al-Sharbati

    2012-02-01

    Full Text Available Objectives: There is significant evidence that psychotherapy is a pivotal treatment for persons diagnosed with Axis I clinical psychiatric conditions; however, a psychotherapy service has only recently been established in the Omani health care system. This study aimed to investigate the sociodemographic and clinical characteristics of attendees at a psychotherapy clinic at a tertiary care hospital. Methods: An analysis was carried out of 133 new referrals to the Psychotherapy Service at Sultan Qaboos University Hospital, a tertiary care hospital. Results: The majority of referrals were females (59%, aged 18–34 years, employed (38%, had ≤12 years of formal education (51%, and were single (54%. A total of 43% were treated for anxiety disorders (including obsessive compulsive disorder, while 22% were treated for depression. A total of 65% were prescribed psychotropic medications. The utilisation of the Psychotherapy Service and its user characteristics are discussed within the context of a culturally diverse Omani community which has unique personal belief systems such as in supernatural powers (Jinn, contemptuous envy (Hassad, evil eye (Ain and sorcery (Sihr which are often used to explain the aetiology of mental illness and influence personal decisions on utilising medical and psychological treatments. Conclusion: Despite the low number of referrals to the Psychotherapy Service, there is reason to believe that psychotherapy would be an essential tool to come to grips with the increasing number of mental disorders in Oman.

  9. Wound pruritus: pathophysiology and management

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    Paul JC

    2015-08-01

    Full Text Available Julia C PaulSchool of Nursing, Oakland University, Rochester, MI, USAPurpose: The objective of this article is to review literature on wound pruritus, with a focus on summarizing pathophysiology and management.Method: Literature related to the physiology of itch was reviewed. PubMed, MEDLINE, the Cumulative Index to Nursing and Allied Health Literature (CINAHL, and Embase were searched for all research studies written in English which include “wound” (injury/burn and “pruritus” (itch in the title or abstract. Articles were accepted if they involved wounds or acute burns. Literature related to options for management of wound pruritus was reviewed.Results: While all types of wounds can be the source of associated pruritus, most studies have been done concerning pruritus associated with burns. There are treatment options for pruritus which can be considered for management of wound pruritus. Conclusion: Further research is indicated to gain insights into the problem of wound pruritus. As more is learned about the physiology of wound pruritus, more effective management strategies can be developed and employed.Keywords: wound, chronic itch, C-fibers, spinothalamic tract, positron emission tomography, pruritogens

  10. Clinical and genetic characteristics of Chinese hereditary nonpolyposis colorectal cancer families

    Institute of Scientific and Technical Information of China (English)

    Xu-Lin Wang; Ying Yuan; Su-Zhan Zhang; Shan-Rong Cai; Yan-Qin Huang; Qiang Jiang; Shu Zheng

    2006-01-01

    AIM: To analyze the clinical characteristics of Chinese hereditary nonpolyposis colorectal cancer (HNPCC) families and to screen the germline mutations of human mismatch repair genes hMLH1 and hMSH2 in the probands.METHODS: Thirty-one independent Chinese HNPCC families were collected in Zhejiang Province. All of them met Chinese HNPCC criteria. Clinical data about patient gender, site of colorectal cancer, age of onset, history of multiple colorectal cancer, associated extracolonic cancer were recorded. PCR and denaturing high performance liquid chromatography (DHPLC) were employed to screen the mutations. Sequencing analysis was used to find out the exact mutation site and characteristics of the samples showing abnormal DHPLC profiles.RESULTS: One hundred and thirty-six malignant neoplasms were found in 107 patients including 14 multiple cancers. One hundred and six of the 136 neoplasms (77.9%) were diagnosed as colorectal cancer, with an average age of onset at 48.57 ± 29.00 years. Gastric cancer was the most common extracolonic cancer (10.3%) in these families. Twenty-three different sequence variations in hMLH1 and hMSH2 genes were detected in these 17 families. Fifteen sequence variations were located in the exons, including 5 SNPs, 3 silent mutations, 3 missense mutations, 2 nonsense mutations and 2 frameshift mutations. The latter seven mutations seemed to be pathogenic.CONCLUSION: Germline mutations of hMLH1 and hMSH2 genes are identified in about one-third HNPCC kindreds fulfilling Chinese HNPCC criteria. Chinese HNPCC families have some particular clinical characteristics, such as a left-sided predominance, less synchronous or metachronous colorectal cancer, and frequent occurrence of gastric cancer.

  11. Geographic differences in clinical characteristics and management of COPD: the EPOCA study

    Science.gov (United States)

    Miravitlles, Marc; Murio, Cristina; Tirado-Conde, Gema; Levy, Gur; Muellerova, Hana; Soriano, Joan B; Ramirez-Venegas, Alejandra; Ko, Fanny WS; Canelos-Estrella, Byron; Giugno, Eduardo; Bergna, Miguel; Chérrez, Ivan; Anzueto, Antonio

    2008-01-01

    Aims Data on differences in clinical characteristics and management of COPD in different countries and settings are limited. We aimed to characterize the profile of patients with COPD in a number of countries and their treatment in order to evaluate adherence to recommendations of international guidelines. Method This was an observational, international, cross-sectional study on patients with physician-diagnosed COPD. Demographic and clinical characteristics, risk factors, and treatment were collected by their physician via an internet web-based questionnaire developed for the study. Results A total of 77 investigators from 17 countries provided data on 833 patients. The countries with the highest number of patients included were: Argentina (128), Ecuador (134), Spain (162), and Hong Kong (153). Overall, 79.3% were men and 81% former smokers, with a mean FEV1 = 42.7%, ranging from 34.3% in Hong Kong to 58.8% in Ecuador. Patients reported a mean of 1.6 exacerbations the previous year, with this frequency being significantly and negatively correlated with FEV1(%) (r = −0.256; p < 0.0001). Treatment with short-acting bronchodilators and theophyllines was more frequent in Ecuador and Hong Kong compared with Spain and Argentina, and in patients belonging to lower socioeconomic levels (p < 0.0001 for all comparisons). Inadequacy of treatment with inhaled corticosteroids and theophyllines was high, with significant differences among countries. Conclusions Differences in the clinical characteristics and management of COPD were significant across countries. Adherence to international guidelines appears to be low. Efforts should be made to disseminate and adapt guidelines to the socioeconomic reality of different settings. PMID:19281096

  12. Observational study identifies non-attendance characteristics in two hospital outpatient clinics

    DEFF Research Database (Denmark)

    Blæhr, Emely; Søgaard, Rikke; Kristensen, Thomas;

    2016-01-01

    INTRODUCTION: Non-attended hospital appointments are receiving increasing attention in times when rapid access and efficient service delivery at public hospitals are on the agenda. The aim of this study was to investigate the extent of non-attendance in a Danish outpatient setting and its...... association with user-level and provider-level characteristics. METHODS: The study was based on appointments scheduled from June 2013 to March 2015 at an orthopaedic and a radiologic outpatient clinic. Data on outcomes of cancellation on the part of the user or the provider, and non-attendance without giving...... unattended without notice. The latter was significantly associated with male gender, younger age and longer time since referral. Other characteristics were identified as significant, but differed between departments. CONCLUSION: There seems to be a potential for a targeted effort aiming to reduce non-attendance...

  13. Clinical, epidemiological and social characteristics of long-lived persons of the Volga region industrial centre

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    Malinova L.I.

    2011-09-01

    Full Text Available Aim: In spite of recent achievements in public health, average lifetime in the Volga region as in whole Russia is rather short. Issues of longevity in the region produce scientific interest by clinical, epidemiological and social characteristics of long-lived persons. Material and methods: In total 167 long-lived persons were examined. Medical, social, psychic, emotional and epidemiological characteristics were assessed. Results: The typical long-lived person of the Volga region was brought out. The high quality of life was revealed. The negative influence of smoking and alcoholism on the longevity was shown. Most of the patients needed communication. Lack of complaints was revealed, patients mentioned only the general weakness. Conclusion: The study has explained new social and demographic features of the Volga region long-livers

  14. Beck Anxiety Inventory: psychometric characteristics in a sample from the clinical Spanish population.

    Science.gov (United States)

    Vázquez Morejón, Antonio J; Vázquez-Morejón Jiménez, Raquel; Zanin, Gloria Bellido

    2014-10-28

    Even though the Beck Anxiety Inventory (BAI) is one of the most popular instruments to assess anxiety today, only limited data is available about its psychometric characteristics and normative values in clinical Spanish populations. A study was conducted to test the psychometric characteristics of a Spanish adaptation of the Beck Anxiety Inventory (BAI) in a sample of 918 outpatients being treated at a community mental health center in Spain. Results confirmed the adaptation's high internal consistency (∝ = .91), substantial test-retest reliability at 8-10 weeks (r = .84, p Anxiety (r = .86, p Phobic Anxiety (r = .63, p < .01) dimensions of the SCL-90-R, and with the Anxious Thoughts Inventory (r = .57, p < .01). Gender differences in BAI scores did occur, so normative values appear separately for each gender.

  15. Acute HIV infection (AHI in a specialized clinical setting: case-finding, description of virological, epidemiological and clinical characteristics

    Directory of Open Access Journals (Sweden)

    Adriana Ammassari

    2014-11-01

    Full Text Available Introduction: Diagnosis of HIV infection during early stages is mandatory to catch up with the challenge of limiting HIV viral replication and reservoirs formation, as well as decreasing HIV transmissions by immediate cART initiation. Objectives: Aims were to describe (a virological characteristics of AHI identified, (b epidemiological and clinical factors associated with being diagnosed with AHI. Methods: Cross-sectional, retrospective study. All individuals diagnosed with AHI according to Fiebig's staging between Jan 2013 and Mar 2014 at the INMI “L. Spallanzani” were included. Serum samples reactive to a fourth generation HIV-1/2 assay (Architect HIV Ag/Ab Combo, Abbott were retested with another fourth generation assay (VIDAS DUO HIV Ultra, Biomérieux and underwent confirmation with HIV-1 WB (New Lav I Bio-Rad and/or with Geenius confirmatory assay (Bio-Rad. WHO criteria (two env products reactivity were used to establish positivity of confirmatory assays. In case of clinically suspected AHI, HIV-1 RNA (Real time, Abbott and p24 assay (VIDAS HIV P24 Bio-Rad were also performed. Avidity test was carried out, on confirmed positive samples lacking p31 reactivity, to discriminate between recent (true Fiebig V phase and late infections; to avoid possible misclassifications, clinical data were also used. Demographic, epidemiological, clinical and laboratory data are routinely, and anonymously recorded in the SENDIH and SIREA studies. Results: During the study period, we observed 483 newly HIV diagnosed individuals, of whom 40 were identified as AHI (8.3%. Fiebig classification showed: 7 stage II/III, 13 stage IV, 20 stage V. Demographic, epidemiological, and clinical characteristics of patients are shown in the Table. Overall, the study population had a median S/Co ratio at fourth generation EIA (Architect of 49.50 (IQR, 23.54–98.05: values were significantly lower in Fiebig II-IV than in Fiebig V (38.68 [IQR, 20.08–54.84] vs 75.72 [IQR

  16. Incidence, clinical characteristics and 30-day mortality of enterococcal bacteraemia in Denmark 2006-2009

    DEFF Research Database (Denmark)

    Pinholt, M; Ostergaard, C; Arpi, M;

    2014-01-01

    Enterococci currently account for approximately 10% of all bacteraemias, reflecting remarkable changes in their epidemiology. However, population-based data of enterococcal bacteraemia are scarce. A population-based cohort study comprised all patients with a first episode of Enterococcus faecalis...... or Enterococcus faecium bacteraemia in two Danish regions during 2006-2009. We used data collected prospectively during clinical microbiological counselling and hospital registry data. We determined the incidence of mono- and polymicrobial bacteraemia and assessed clinical and microbiological characteristics...... as predictors of 30-day mortality in monomicrobial bacteraemia by logistic regression analysis. We identified 1145 bacteraemic patients, 700 (61%) of whom had monomicrobial bacteraemia. The incidence was 19.6/100 000 person-years (13.0/100 000 person-years for E. faecalis and 6.6/100 000 person-years for E...

  17. Clinical Characteristics and Outcome of Cardiovascular Implantable Electronic Device Infections in Turkey.

    Science.gov (United States)

    Aydin, Mesut; Yildiz, Abdulkadir; Kaya, Zeynettin; Kaya, Zekeriya; Basarir, Ahmet Ozgur; Cakmak, Nazmiye; Donmez, Ibrahim; Morrad, Baktash; Avci, Ahmet; Demir, Kenan; Cagliyan, Emre Caglar; Yuksel, Murat; Elbey, Mehmet Ali; Kayan, Fethullah; Ozaydogdu, Necdet; Islamoglu, Yahya; Cayli, Murat; Alan, Said; Ulgen, Mehmet Siddik; Ozhan, Hakan

    2016-07-01

    Infection is one of the most devastating outcomes of cardiovascular implantable electronic device (CIED) implantation and is related to significant morbidity and mortality. In our country, there is no evaluation about CIED infection. Therefore, our aim was to investigate clinical characteristics and outcome of patients who had infection related to CIED implantation or replacement. The study included 144 consecutive patients with CIED infection treated at 11 major hospitals in Turkey from 2005 to 2014 retrospectively. We analyzed the medical files of all patients hospitalized with the diagnosis of CIED infection. Inclusion criteria were definite infection related to CIED implantation, replacement, or revision. Generator pocket infection, with or without bacteremia, was the most common clinical presentation, followed by CIED-related endocarditis. Coagulase-negative staphylococci and Staphylococcus aureus were the leading causative agents of CIED infection. Multivariate analysis showed that infective endocarditis and ejection fraction were the strongest predictors of in-hospital mortality.

  18. The clinical characteristics and therapy of syndrome of craniocerebral- cervical vertebral injury

    Institute of Scientific and Technical Information of China (English)

    LIU Sheng; LIU Yuan-xin; WANG Cheng

    2005-01-01

    Objective: To explore the clinical characteristics and new treatment for syndrome of craniocerebral-cervical vertebral injury. Methods: The clinical data of 52 patients with head injury accompanied by neck injury were analyzed retrospectively. Results: Craniocerebral injury could result in damage to cervical vertebrae, muscles, vessels and nerves, and even cause vertebral artery injury, which may lead to insufficient blood-supply of vertebral-basal artery. All patients were treated with cervical vertebral traction and the results were good. Conclusions: Acute craniocerebral injury with symptom of insufficient blood-supply of vertebral-basal artery, evident neurosis and atlas-axis half-dislocation in X-ray should be treated by cervical vertebral traction, which will yield better outcome.

  19. Clinical Characteristics and Prognostic Analysis of 107 Patients with Bronchioloalveolar Carcinoma

    Institute of Scientific and Technical Information of China (English)

    Dongsheng Yue; Changli Wang; Zhenfa Zhang; Zhongli Zhan

    2006-01-01

    OBJECTIVE To study the clinical characteristics and prognostic factors for bronchioloalveolar carcinoma.METHODS Clinical data from 107 inpatient cases at The Cancer Hospital of Tianjin Medical University, from 1990-2000, were retrospectively reviewed.RESULTS The overall 1, 3 and 5-year survival rates were 88.7, 64.8 and 48.6% respectively. The main prognostic factors were tumor diameter (P=0.022), bronchial stump (P=0.016), TNM stage (P=0.000), T stage (P=0.002), N stage (P=0.000) and postoperation radiotherapy (P=0.001). Coxregression analysis suggested that the TNM stage (P=0.000) and tumor diameter (P=0.015) are independent factors affecting the prognosis.CONCLUSION The overall survival rate of BAC patients was superior to those with other non-small cell lung cancer (NSCLC). The TNM stage and tumor diameter were independent factors affecting the prognosis for BAC.

  20. Clinical characteristics and prognostic impact of bacterial infection in hospitalized patients with alcoholic liver disease.

    Science.gov (United States)

    Park, Jin Kyoung; Lee, Chang Hun; Kim, In Hee; Kim, Seon Min; Jang, Ji Won; Kim, Seong Hun; Kim, Sang Wook; Lee, Seung Ok; Lee, Soo Teik; Kim, Dae-Ghon

    2015-05-01

    Bacterial infection is an important cause of death in patients with liver cirrhosis. The aim of this study was to investigate the clinical characteristics and prognostic impact of bacterial infection in hospitalized patients with alcoholic liver disease (ALD). We retrospectively analyzed data from 409 patients consecutively admitted to a tertiary referral center with ALD diagnosis. Of a total of 544 admissions, 133 (24.4%) cases presented with bacterial infection, of which 116 were community-acquired whereas 17 were hospital-acquired. The common types of infection were pneumonia (38%), biliary tract infection (17%), soft tissue infection (12%), and spontaneous bacterial peritonitis (9%). Diabetes, serum Na patients with ALD. Overall 30-day and 90-day mortalities in patients with bacterial infection were significantly (P patients with ALD. A thorough evaluation at admission or on clinical deterioration is required to detect possible infection with prompt management.

  1. Chronic Inflammatory Demyelinating Polyneuropathy in Children: A Review of Clinical Characteristics and Recommendations for Treatment

    Directory of Open Access Journals (Sweden)

    Narges Karimi

    2015-07-01

    Full Text Available Context: Chronic inflammatory demyelinating polyradiculopathy (CIDP is an acquired and autoimmune neuropathy, characterized by a chronic, rapidly progressive, symmetric weakness. In children, abnormal gait is as a first symptom of muscle weakness. Evidence Acquisition: The diagnosis of CIDP is on the basis of clinical characteristics, electrodiagnostic that shows the severity of the disease, lumbar puncture and spine magnetic resonance imaging (MRI. Results: The first-line treatments in childhood CIDP are intravenous immunoglobulin (IVIG, corticosteroids, and plasmapheresis. Response to first-line therapies is usually satisfactory; nevertheless, recommendations regarding the choice of second-line therapy can only be prepared on the basis of the existing practice described in some of the case reports. Conclusions: This review demonstrated the clinical presentation, diagnosis, and treatment of childhood CIDP.

  2. Clinical characteristics of late-onset severe pneumonia after allogeneic hematopoietic stem cell transplantation

    Institute of Scientific and Technical Information of China (English)

    刘代红

    2013-01-01

    Objective To analyze the clinical characteristics of the late-onset severe pneumonia after allogeneic hematopoietic stem cell transplantation (allo-HSCT) .Methods A retrospective study was conducted in patients diagnosed as late-onset severe pneumonia after allo-HSCT from March,2009 to January,2013 in People’s Hospital of Peking University.Results Of 1538 patients receiving allo-HSCT,20 developed late-onset severe pneumonia with an incidence rate of 1.3%.Among the 20 patients,17 (85%) had human leukocyte antigen (HLA) identical donors.The other 3 (15%) patients had received haploidentical transplantation.Severe pneumonia occurred at a

  3. Extranodal marginal zone lymphoma in the ocular region: clinical, immunophenotypical, and cytogenetical characteristics

    DEFF Research Database (Denmark)

    Sjö, Lene D; Heegaard, Steffen; Prause, Jan U;

    2008-01-01

    the period 1980 to 2005 were reviewed and reclassified according to the World Health Organization (WHO) classification. Cases reclassified as EMZL were selected and reviewed with respect to clinical characteristics and outcome. The presence of translocations involving IGH and/or MALT1 was investigated in 42...... were frequently found at extraocular sites. Five-year progression-free survival and overall survival (OS) rates were 71% and 75%, respectively. Translocations involving the IGH- or MALT1-gene loci were detected in 2 (5%) of 42 specimens. In Cox regression multivariate analysis, IGH...

  4. Mooren's ulcer in China: a study of clinical characteristics and treatment

    OpenAIRE

    Chen, J; Xie, H; Wang, Z.(Institute of High Energy Physics, Beijing, China); Yang, B.; Liu, Z.; Chen, L.; Gong, X.; Lin, Y.

    2000-01-01

    AIMS—To investigate the clinical characteristics and compare the effects of several methods of treatment of Mooren's corneal ulcer.
METHODS—550 consecutive cases of Mooren's corneal ulcer were analysed in patients, including age, sex, laterality of eye, ulcer location, perforative rate, cure rate of surgeries, recurrent rate, the effects of conjunctiva excision, lamellar keratoplasty (LKP), and LKP plus 1% cyclosporin A eye drops.
RESULTS—The average age of onset was 48.4 years of age. The ra...

  5. STUDY ON THE CLINICAL CHARACTERISTICS OF ADULT PATIENTS WITH KETOSISONSET DIABETES

    Institute of Scientific and Technical Information of China (English)

    叶新华; 成金罗

    2013-01-01

    Objective To explore the classification and analyze the clinical characteristics of adult diabetic patients with ketosis as initial manifestation.Methods 142 cases with initial onset of ketosis were retrospectively analyzed and the levels of blood lipid,random blood glucose,glycosylated hemoglobin,islet function and islet autoantibody[insulin autoantibody (IAA) ,glutamic acid decarboxylase antibody (GADA) ]were measured.The blood pressure and family history of diabetes were also documented.Based on the presence of islet autoantibodies (A+or

  6. Clinical characteristics of Raoultella ornithinolytica bacteremia: a case series and literature review.

    Science.gov (United States)

    Haruki, Yuto; Hagiya, Hideharu; Sakuma, Akiko; Murase, Tomoko; Sugiyama, Tetsuhiro; Kondo, Sachiyo

    2014-09-01

    Raoultella ornithinolytica is a rare pathogen in human infection and bacteremic cases had been scarcely reported. For further comprehension of the rare infection, we summarized clinical characteristics of 6 cases that were detected at our medical facility and 5 cases from previous literature. The most common infectious focus was biliary infection and elderly patients with a history of any biliary intervention or malignancy were considered to be at a great risk for the infection. The prognosis of the patients was quite satisfactory. Bacterial identification in this report was performed on the basis of biochemical tests alone, and further investigations by molecular analysis are required to confirm our findings.

  7. Pandemic and Avian Influenza A Viruses in Humans: Epidemiology, Virology, Clinical Characteristics, and Treatment Strategy.

    Science.gov (United States)

    Li, Hui; Cao, Bin

    2017-03-01

    The intermittent outbreak of pandemic influenza and emergence of novel avian influenza A virus is worldwide threat. Although most patients present with mild symptoms, some deteriorate to severe pneumonia and even death. Great progress in the understanding of the mechanism of disease pathogenesis and a series of vaccines has been promoted worldwide; however, incidence, morbidity, and mortality remains high. To step up vigilance and improve pandemic preparedness, this article elucidates the virology, epidemiology, pathogenesis, clinical characteristics, and treatment of human infections by influenza A viruses, with an emphasis on the influenza A(H1N1)pdm09, H5N1, and H7N9 subtypes.

  8. Impulse control disorders: updated review of clinical characteristics and pharmacological management

    Directory of Open Access Journals (Sweden)

    Jon E Grant

    2011-02-01

    Full Text Available Impulse control disorders (ICDs are characterized by urges and behaviors that are excessive and/or harmful to oneself or others and cause significant impairment in social and occupational functioning, as well as legal and financial difficulties. ICDs are relatively common psychiatric conditions, yet are poorly understood by the general public, clinicians, and individuals struggling with the disorder. Although ICD treatment research is limited, studies have shown ICDs may respond well to pharmacological treatment. This article presents a brief overview about the clinical characteristics of ICDs and pharmacological treatment options for individuals with ICDs.

  9. Clinical and molecular characteristics of East Asian patients with von Hippel-Lindau syndrome

    Institute of Scientific and Technical Information of China (English)

    Meihua Wong; YingHsia Chu; Hwei Ling Tan; Hideharu Bessho; Joanne Ngeow; Tiffany Tang; MinHan Tan

    2016-01-01

    Background: Von Hippel–Lindau (VHL) syndrome is a dominantly inherited multisystem cancer syndrome caused by a heterozygous mutation in the VHL tumor suppressor gene. Previous studies suggested that similar populations of Caucasian and Japanese patients have similar genotype or phenotype characteristics. In this comprehensive study of East Asian patients, we investigated the genetic and clinical characteristics of patients with VHL syndrome. Methods: To create a registry of clinical characteristics and mutations reported in East Asian patients with VHL syndrome, we conducted a comprehensive review of English language and non‑English language articles identi‑fied through a literature search. Publications in Japanese or Chinese language were read by native speakers of the language, who then performed the data extraction. Results: Of 237 East Asian patients with VHL syndrome, 154 unique kindreds were identified for analysis. Analyzed by kindred, missense mutations were the most common (40.9%, 63/154), followed by large/complete deletions (32.5%, 50/154) and nonsense mutations (11.7%, 18/154). Compared with a previously reported study of both East Asian and non‑East Asian patients, we found several key differences. First, missense and frameshift mutations in the VHL gene occurred less commonly in our population of East Asian patients (40.9% vs. 52.0%; P = 0.012 and 8.4% vs.13.0%; P < 0.001, respectively). Second, large/complete deletions were more common in our population of East Asian patients (32.5% vs. 10.5%; P < 0.001). Third, phenotypically, we observed that, in our population of East Asian patients with VHL syndrome, the incidence of retinal capillary hemangioblastoma was lower, whereas the incidence of renal cell carcinoma was higher. Conclusions: Evidence suggests that the genotypic and phenotypic characteristics of East Asian patients with VHL syndrome differ from other populations. This should be considered when making screening recommendations for

  10. Clinical and histopathological characteristics of cutaneous Leishmaniasis in Sanliurfa City of Turkey including Syrian refugees

    Directory of Open Access Journals (Sweden)

    Sezen Koçarslan

    2013-01-01

    Full Text Available Background: The aim of our study was to investigate the clinical and histopathological characteristics of cutaneous leishmaniasis (CL in the city of Sanliurfa in Turkey, where Syrian refugees also reside. Materials and Methods: At the Harran University Hospital outpatient clinics between 2012 and 2013, 54 CL cases, including 24 Syrian patients, underwent punch biopsy of the skin and/or a touch imprint. Patients in whom leishmania parasites were detected were included in the study. The clinical and histopathological data of the patients were obtained by a review of the patients′ medical records. All the slides of each patient were re-evaluated histopathologically. Results: Fifty-four cases (mean age; 17 ± 12 years, consisting of 32 males (59.3% and 22 females (40.7%, were examined. The most common site of involvement was the face (63%. The most common presentation was noduloulcerative lesions (57.4%. Histopathologically, the majority of the cases exhibited hyperkeratosis, follicular plugging of the epidermis, chronic inflammatory infiltration, leishmania amastigotes and non-caseating granulomatous inflammation in the dermis. Conclusion: CL presents with a wide spectrum of expression, both clinically and histologically, and may mimic other inflammatory and neoplastic diseases. The diagnosis of CL relies on the identification of leishmania amastigotes in either a direct smear of the lesion or in a tissue section.

  11. The characteristics of heterosexual STD clinic attendees who practice oral sex in Zhejiang Province, China.

    Directory of Open Access Journals (Sweden)

    Qiaoqin Ma

    Full Text Available BACKGROUND: The characteristics of heterosexual attendees who visit sexually transmitted disease (STD clinics and practice oral sex have not been revealed in China. This information is important for the development of targeted STD prevention programmes for this population. STUDY DESIGN: A self-administered questionnaire survey with a cross-sectional design was administered to consecutive attendees at four STD clinics in Zhejiang Province, China, between October and December in 2007. Demographic, psychosocial, and behavioural factors associated with oral sex over a lifetime were identified using univariate and multivariate analyses. RESULTS: Of the 872 attendees, 6.9% engaged in oral sex over their lifetimes. Of the oral-sex group, 96.6% also engaged in vaginal sex. The correlates for oral sex over a lifetime as determined by the multivariate analysis were high income (odds ratio [OR] = 2.53, 95% confidence interval [CI] 1.39-4.59, high human immunodeficiency virus (HIV-related knowledge (OR = 2.71, 95% CI 1.26-5.81, early sex initiation (OR = 2.42, 95% CI 1.37-4.27, multiple sexual partners (OR = 3.09, 95% CI 1.58-6.06, and sexually active in the previous 6 months (OR = 7.73, 95% CI 1.04-57.39. CONCLUSIONS: Though the prevalence of oral sex is low, the heterosexual STD clinic attendees practicing oral sex was found to have higher risks associated with STD/HIV transmission than those not. Behavioural and medical interventions conducted by clinicians in Chinese STD clinics should take into account the characteristics and related risks of those who practice oral sex.

  12. Pituitary stalk interruption syndrome in Chinese people: clinical characteristic analysis of 55 cases.

    Directory of Open Access Journals (Sweden)

    Qinghua Guo

    Full Text Available OBJECTIVE: Pituitary stalk interruption syndrome (PSIS is characterized by the absence of pituitary stalk, pituitary hypoplasia, and ectopic posterior pituitary. Due to the rarity of PSIS, clinical data are limited, especially in Chinese people. Herein, we analyzed the clinical characteristics of patients diagnosed with PSIS from our center over 10 years. PATIENTS AND METHODS: We retrospectively analyzed the clinical manifestations and laboratory and MRI findings in 55 patients with PSIS. RESULTS: Of the 55 patients with PSIS, 48 (87.3% were male. The average age was 19.7±6.7 years and there was no familial case. A history of breech delivery was documented in 40 of 45 patients (88.9% and 19 of 55 patients (34.5% had a history of dystocia. Short stature was found in 47 of 55 patients (85.5% and bone age delayed 7.26±5.37 years. Secondary sex characteristics were poor or undeveloped in most patients. The prevalence of deficiencies in growth hormone, gonadotropins, corticotropin, and thyrotropin were 100%, 95.8%, 81.8%, 76.3%, respectively. Hyperprolactinemia was found in 36.4% of patients. Three or more pituitary hormone deficiencies were found in 92.7% of the patients. All patients had normal posterior pituitary function and absent pituitary stalk on imaging. The average height of anterior pituitary was 28 mm, documented anterior pituitary hypoplasia. Midline abnormalities were presented in 9.1% of patients. CONCLUSIONS: The clinical features of our Chinese PSIS patients seem to be different from other reported patients in regarding to the higher degree of hypopituitarism and lower prevalence of midline defects. In addition, our patients were older at the time of case detection and the bone age was markedly delayed. We also had no cases of familial PSIS.

  13. Clinical And Paraclinical Characteristics Of Patients With Unstable Anyina Hospitalized At Ccu

    Directory of Open Access Journals (Sweden)

    Sadr Bafghi S M

    2003-09-01

    Full Text Available Unstable angina (UA is situated in a spectrum with myocardial infarction (MI at one end of it and stable angina at the other end. To determine the clinical and paraclinical characteristics of unstable angina this study was designed."nMaterials and Methods: Two hundred patients with definite UA who were hospitalized at cardiac care units (CCU in Yazd were enrolled in this cross- sectional study. Clinical and para-clinical characteristics including class of severity of angina, clinical circumstances, drugs, risk factors, changes in ECG, and patient's condition at the time of leaving hospital were evaluated."nResults& Conclusions: The average of age of patients was 61.85 years and 57% of them were female and 43% were male. The most prevalent risk factor among men was smoking (48.8% and among women was hypertension (62.8%. According to Braunwald suggestion, patients were divided into 3 classes based on the severity of their diseases. Frequency distribution of patients in classes I,H and III were 24.5%, 4% and 71.5%, respectively. Normal ECG, was observed in 24.5% of subjects at the time of hospitalization and in 67% of patients when leaving hospitals. Frequency distribution based on the risk stratification was 22.5%, 58.5% and 19% in the low, medium and high risk groups, respectively. During hospitalization, acute myocardial infarction (AMI was seen in 3.5% of individuals that majority of them belonged to class III of angina.

  14. The characteristic features of auditory verbal hallucinations in clinical and non-clinical groups: State-of-the-art overview and future directions.

    OpenAIRE

    2012-01-01

    Despite a growing interest in auditory verbal hallucinations (AVHs) in different clinical and nonclinical groups, the phenomenological characteristics of such experiences have not yet been reviewed and contrasted, limiting our understanding of these phenomena on multiple empirical, theoretical, and clinical levels. We look at some of the most prominent descriptive features of AVHs in schizophrenia (SZ). These are then examined in clinical conditions including substance abuse, Parkinson’s dise...

  15. Proposed Pathophysiologic Framework to Explain Some ...

    Science.gov (United States)

    The paper proposes a pathophysiologic framework to explain the well-established epidemiological association between exposure to ambient air particle pollution and premature cardiovascular mortality, and offers insights into public health solutions that extend beyond regularory environmental protections to actions that can be taken by individuals, public health officials, healthcare professionals, city and regional planners, local and state governmental officials and all those who possess the capacity to improve cardiovascular health within the popula­tion.The foundation of the framework rests on the contribution of traditional cardiovascular risk factors acting alone and in concert with long-term exposures to air pollutants to create a conditional susceptibility for clinical vascular events, such as myocardial ischemia and infarction; stroke and lethal ventricular arrhythmias. The conceprual framework focuses on the fact that short-term exposures to ambient air particulate matter (PM) are associated with vascular thrombosis (acute coronary syndrome. stroke, deep venous thrombosis. and pulmonary embolism ) and electrical dysfunction (ventricular arrhythmia); and that individuals having prevalent heart disease are at greatest risk. Moreover, exposure is concomitant with changes in autonomic nervous system balance, systemic in­flammation, and prothrombotic/anti-thrombotic and profibrinolytic-antifibrinolytic balance.Thus, a comprehensive solution to the problem o

  16. Orthostatic intolerance: potential pathophysiology and therapy.

    Science.gov (United States)

    Lu, Chih-Cherng; Tseng, Ching-Jiunn; Tang, Hung-Shang; Tung, Che-Se

    2004-09-30

    Orthostatic intolerance affects an estimated 1 in 500 persons and causes a wide range of disabilities. After essential hypertension, it is the most frequently encountered dysautonomia, accounting for the majority of patients referred to centers specializing in autonomic disorders. Patients are typically young females with symptoms such as dizziness, visual changes, head and neck discomfort, poor concentration, fatigue, palpitations, tremulousness, anxiety, and, in some cases, syncope. Syncope is the most hazardous symptom of orthostatic intolerance, presumably occurring because of impaired cerebral perfusion and in part to compensatory autonomic mechanisms. The etiology of this syndrome is still unclear but is heterogeneous. Orthostatic intolerance used to be characterized by an overall enhancement of noradrenergic tone at rest in some patients and by a patchy dysautonomia of postganglionic sympathetic fibers with a compensatory cardiac sympathetic activation in others. However, recent advances in molecular genetics are improving our understanding of orthostatic intolerance, such as several genetic diseases (such as Ehler-Danlos syndrome and norepinephrine transporter deficiency) presenting with symptoms typical of orthostatic intolerance. Future work will include investigation of genetic functional mutations underlying interindividual differences in autonomic cardiovascular control, body fluid regulation, and vascular regulation in orthostatic intolerance patients. The goal of this review article is to describe recent advances in understanding the pathophysiological mechanisms of orthostatic intolerance and their clinical significance.

  17. The Charcot foot: pathophysiology, diagnosis and classification.

    Science.gov (United States)

    Trieb, K

    2016-09-01

    Neuropathic changes in the foot are common with a prevalence of approximately 1%. The diagnosis of neuropathic arthropathy is often delayed in diabetic patients with harmful consequences including amputation. The appropriate diagnosis and treatment can avoid an extensive programme of treatment with significant morbidity for the patient, high costs and delayed surgery. The pathogenesis of a Charcot foot involves repetitive micro-trauma in a foot with impaired sensation and neurovascular changes caused by pathological innervation of the blood vessels. In most cases, changes are due to a combination of both pathophysiological factors. The Charcot foot is triggered by a combination of mechanical, vascular and biological factors which can lead to late diagnosis and incorrect treatment and eventually to destruction of the foot. This review aims to raise awareness of the diagnosis of the Charcot foot (diabetic neuropathic osteoarthropathy and the differential diagnosis, erysipelas, peripheral arterial occlusive disease) and describe the ways in which the diagnosis may be made. The clinical diagnostic pathways based on different classifications are presented. Cite this article: Bone Joint J 2016;98-B:1155-9.

  18. Pathophysiology of constipation in the older adult

    Institute of Scientific and Technical Information of China (English)

    G Lindsay McCrea; Christine Miaskowski; Nancy A Stotts; Liz Macera; Madhulika G Varma

    2008-01-01

    This review provides information on the definition of constipation,normal continence and defecation and a description of the pathophysiologic mechanisms of constipation.In addition,changes in the anatomy and physiology of the lower gastrointestinal tract associated with aging that may contribute to constipation are described.MEDLINE (1966-2007) and CINAHL (1980-2007) were searched.The following MeSH terms were used:constipation/etiology OR constipation/physiology OR constipation/physiopathology) AND (age factors OR aged OR older OR 80 and over OR middle age).Constipation is not well defined in the literature.While self-reported constipation increases with age,findings from a limited number of clinical studies that utilized objective measures do not support this association.Dysmotility and pelvic floor dysfunction are important mechanisms associated with constipation.Changes in GI function associated with aging appear to be relatively subtle based on a limited amount of conflicting data.Additional research is warranted on the effects of aging on GI function,as well as on the timing of these changes.

  19. Obesity-related hypertension: possible pathophysiological mechanisms.

    Science.gov (United States)

    Vaněčková, Ivana; Maletínská, Lenka; Behuliak, Michal; Nagelová, Veronika; Zicha, Josef; Kuneš, Jaroslav

    2014-12-01

    Hypertension is one of the major risk factors of cardiovascular diseases, but despite a century of clinical and basic research, the discrete etiology of this disease is still not fully understood. The same is true for obesity, which is recognized as a major global epidemic health problem nowadays. Obesity is associated with an increasing prevalence of the metabolic syndrome, a cluster of risk factors including hypertension, abdominal obesity, dyslipidemia, and hyperglycemia. Epidemiological studies have shown that excess weight gain predicts future development of hypertension, and the relationship between BMI and blood pressure (BP) appears to be almost linear in different populations. There is no doubt that obesity-related hypertension is a multifactorial and polygenic trait, and multiple potential pathogenetic mechanisms probably contribute to the development of higher BP in obese humans. These include hyperinsulinemia, activation of the renin-angiotensin-aldosterone system, sympathetic nervous system stimulation, abnormal levels of certain adipokines such as leptin, or cytokines acting at the vascular endothelial level. Moreover, some genetic and epigenetic mechanisms are also in play. Although the full manifestation of both hypertension and obesity occurs predominantly in adulthood, their roots can be traced back to early ontogeny. The detailed knowledge of alterations occurring in the organism of experimental animals during particular critical periods (developmental windows) could help to solve this phenomenon in humans and might facilitate the age-specific prevention of human obesity-related hypertension. In addition, better understanding of particular pathophysiological mechanisms might be useful in so-called personalized medicine.

  20. Analysis on clinical characteristics of intracranial Arachnoid Cysts in 488 pediatric cases.

    Science.gov (United States)

    Huang, Jian-Huang; Mei, Wen-Zhong; Chen, Yao; Chen, Jian-Wu; Lin, Zhi-Xiong

    2015-01-01

    To summarize the clinical characteristics of intracranial arachnoid cysts (IACs) in pediatric cases. A retrospective analysis was carried out on clinical characteristics of IACs in 488 pediatric cases who were treated at our hospital from January 2003 to September 2013. There were 342 males and 146 females (male-to-female ratio, 2.34:1), aged 5.61±3.25 years on average. 221 cases (45.29%) were diagnosed accidentally, 267 cases had clinical complaints (54.71%), among which relationships between clinical complaints and IACs were identified in 123 (46.07%). Simple IACs occurred in 364 cases (4.59%), and concurrent congenital abnormalities occurred in 124 cases (4.59%). In terms of location, 355 had IACs in middle cranial fossa (72.75%), 82 cases in posterior cranial fossa (16.80%), 20 cases in anterior cranial fossa (4.10%), 12 cases in dorsolateral surface (2.46%), 7 cases in suprasellar cistern (1.43%), 5 cases in cerebral ventricle (1.02%), 5 cases in quadrigeminal cistern (1.02%), and 2 cases in interhemispheric region (0.41%). There were 449 cases with single IAC (92.01%) and 39 cases with multiple IACs (7.99%). On MRI, the cysts produced tension in 127 cases (26.02%), but not in the remaining 361 cases (73.98%). Surgery was performed on 76 of 488 cases (15.57%), while conservative observation was accepted in 412 cases (84.43%). For the former, the symptoms and the cyst volume were improved to varying extent; for the latter, the follow-up lasting for 3-72 months (average 32.43±8.92 months) showed that the cyst volume remained stable in 407 cases (98.78%), enlarged with aggravated symptoms in 3 cases (0.73%), and shrank in 2 cases (0.49%). Clinical complaints of IACs varied in pediatric cases, and the relationships between clinical complaints and IACs were established only partially. Some pediatric cases were combined with other congenital abnormalities. The cyst volume largely remained stable during the disease course, and surgery was required for only a few

  1. Epidemiology, clinical characteristics and resource implications of pandemic (H1N1) 2009 in intensive care units in Ireland.

    LENUS (Irish Health Repository)

    Nicolay, Nathalie

    2010-12-01

    To describe the incidence, clinical characteristics and outcomes of critically ill patients in Ireland with pandemic (H1N1) 2009 infection, and to provide a dynamic assessment of the burden of such cases on Irish intensive care units.

  2. Clinical and biochemical characteristics of Cushing’s disease with different suppression rates by high-dose dexamethasone

    Institute of Scientific and Technical Information of China (English)

    唐志清

    2013-01-01

    Objective To analyze the clinical and biochemical characteristics of Cushing’s disease with different suppression rates by high-dose dexamethasone.Methods Two hundred and two consecutive patients with

  3. New insights into rosacea pathophysiology: a review of recent findings.

    Science.gov (United States)

    Steinhoff, Martin; Schauber, Jürgen; Leyden, James J

    2013-12-01

    Rosacea is a common, chronic inflammatory skin disease of poorly understood origin. Based on its clinical features (flushing, chronic inflammation, fibrosis) and trigger factors, a complex pathobiology involving different regulatory systems can be anticipated. Although a wealth of research has shed new light over recent years on its pathophysiology, the precise interplay of the various dysregulated systems (immune, vascular, nervous) is still poorly understood. Most authors agree on 4 major clinical subtypes of rosacea: erythematotelangiectatic rosacea, papulopustular rosacea, phymatous rosacea, and ocular rosacea. Still, it needs to be elucidated whether these subtypes develop in a consecutive serial fashion or if any subtypes may occur individually as part of a syndrome. Because rosacea often affects multiple family members, a genetic component is also suspected, but the genetic basis of rosacea remains unclear. During disease manifestation and early stage, the innate immune system and neurovascular dysregulation seem to be driving forces in rosacea pathophysiology. Dissection of major players for disease progression and in advanced stages is severely hampered by the complex activation of the innate and adaptive immune systems, enhanced neuroimmune communication, profound blood vessel and possibly lymphatic vessel changes, and activation of almost every resident cell in the skin. This review discusses some of the recent findings and aims to build unifying hypotheses for a modern understanding of rosacea pathophysiology.

  4. Mapping the genetic and clinical characteristics of Gaucher disease in the Iberian Peninsula

    Directory of Open Access Journals (Sweden)

    Giraldo Pilar

    2012-03-01

    Full Text Available Abstract Background Gaucher disease (GD is due to deficiency of the glucocerebrosidase enzyme. It is panethnic, but its presentation reveals ethnicity-specific characteristics. Methods We evaluated the distribution, and clinical and genetic characteristics of GD patients in the Iberian Peninsula (IP. We analysed geographical distribution, demographic, genetic and clinical data, age at diagnosis, type, and years of therapy in 436 GD patients from the IP. Results The prevalence of GD was 1/149,000 inhabitants; 88.3% were type 1, 6.7% type 2, and 5.0% type 3. The mean age at diagnosis in type 1 was 28.7 years. A total of 72.7% were classified as having mild forms, 25.5% moderate, and 1.7% severe. Anemia and thrombocytopenia were present in 56% and 55%, respectively. Bone disease and hepatomegaly were reported in 62% and 68%, respectively, and were more likely in asplenic than in non-splenectomized patients. Sixty-nine mutant alleles were identified, and five mutations accounted for 75% of the GBA alleles. Several patients described in our series had interesting phenotypes. A total of 58.7% of patients had received enzyme replacement therapy and 12.6% were treated with miglustat. Conclusions A broad spectrum of GBA mutations is present in the IP, with 98.2% of type 1 GD being mild and 23.0% never treated. These data highlight genetic and phenotypic heterogeneities among geographic populations.

  5. The Evaluation Clinical and Demographic Characteristics of 115 Patients Diagnosed with Herpes Zoster in Eeastern Turkey

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    Hatice Uce Özkol

    2013-12-01

    Full Text Available Objective: The aim of this study was to investigate the clinical and demographic characteristics of patients diagnosed with herpes zoster and to explore the similarities and differences with other epidemiological studies from Turkey and the world. Methods: We retrospectively reviewed the records of 115 patients diagnosed with herpes zoster in the Yuzuncu Yıl University Medical Faculty Dermatology Department between January 2007 and December 2010. Results: The mean age of the patients was 42.21±23.88 years. 115 patients, -47 female (40.9%, 68 male (59.1%- aged between 2 and 93 years were assessed. Pediatric age group, 20 (17.4%, adult age group, 95 (82.6% patients, respectively. The incidence of HZ was found to be 0.43%. HZ was observed winter rarely (13.04%. Is mostly seen in the months of March (17.39% The affected dermatome were thorasic (49 patient, 42.6%, servical (21 patient, 18.3%, ophtalmic (22 patient, 19.1%, lomber (16 patient, 13.9%, sacral (7 patient, 6.1% respectively. Complications developed in 13% of patients. Conclusion: We observed that our findings were more or less similar to the findings of the literature data. Cases of HZ in our study was very rare during the winter season. Multi-center studies are needed to the emergence of clinical and epidemiological characteristics of HZ in Turkey.

  6. Clinical characteristics of ruptured distal middle cerebral artery aneurysms: Review of the literature.

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    Tsutsumi, Keiji; Horiuchi, Tetsuyoshi; Nagm, Alhusain; Toba, Yasuyuki; Hongo, Kazuhiro

    2017-01-10

    Middle cerebral artery (MCA) aneurysms usually arise at the primary MCA bifurcation or trifurcation. Distal MCA aneurysms are rarely considered as sources of aneurysmal subarachnoid hemorrhage (SAH). It has been reported that ruptured distal MCA aneurysms are associated with head trauma, neoplastic emboli, arterial dissection, or bacterial infection. We experienced five cases of ruptured distal MCA aneurysms and evaluated their clinical characteristics. Retrospective analysis of aneurysmal SAH at Kobayashi Neurosurgical Neurological Hospital was performed from January, 2004 to December, 2014. Clinical characteristics of ruptured distal MCA aneurysms were analyzed using our database. Among 191 aneurysmal SAH patients, there were five ruptured distal MCA aneurysms. All patients did not have any specific medical problems such as infectious disease, head trauma, or cardiac disorders. The incidence of ruptured distal MCA aneurysm was higher than expected and was equivalent to 9.4% of the total ruptured MCA aneurysms. Strong male predominance (80%) and M2-3 junction aneurysm preponderance (80%) were observed. In addition, there were only two patients (40%) with intracerebral hematoma in our study. We reported five cases of ruptured distal MCA aneurysms. Although ruptured distal MCA aneurysms are thought to be rare as sources of aneurysmal SAH, the incidence of ruptured distal MCA aneurysm was 9.4% of all ruptured MCA aneurysms in our study. Ruptured distal MCA aneurysms should be considered as sources of aneurysmal SAH without intracerebral hematoma.

  7. Clinical Characteristics of Recurrent Nasopharyngeal Carcinoma in High-Incidence Area

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    Jia-Xin Li

    2012-01-01

    Full Text Available Background. To describe the clinical characteristics of the patients who suffered from relapse after conventional irradiation for nasopharyngeal carcinoma (NPC. Methods. Three hundred and fifty-one consecutive patients with first-time recurrent NPC between January 1999 and July 2005 were included. The patients’ clinical data were reviewed, including recurrent interval time, symptoms, signs, imaging characteristics, pathologic features, and restaging. Results. The median interval of relapse was 26.0 months. The most common symptoms in symptomatic patients were nasal bloody discharge (37.9% and headache (31.1%. Local recurrence alone accounted for 73.5%. Most patients were restaged as stage III (23.1% and stage IV (51.1%. Subgroup analysis suggested a significantly higher proportion of the long-latent relapses originated from early primary. A series of postreirradiation complications were more frequent in patients with longer latency at reception. Conclusions. Most recurrent nasopharyngeal carcinoma is advanced disease. Patients with different recurrent interval time show different nature behavior.

  8. Clinical and microbiologic characteristics of vulvovaginitis in Korean prepubertal girls, 2009–2014: a single center experience

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    Kim, Hounyoung; Chai, Sun Myung; Ahn, Eun Hee; Lee, Mee-Hwa

    2016-01-01

    Objective To update information on the clinical and microbiologic characteristics of pediatric vulvovaginitis in Korean prepubertal girls. Methods A total of 120 girls (aged 0 to 9 years) with culture-confirmed pediatric vulvovaginitis, diagnosed between 2009 and 2014, were enrolled in the study. The epidemiologic and microbiologic characteristics, and clinical outcomes were assessed. Patients with sexual precocity, as well as those who were referred for suspected sexual abuse, were excluded....

  9. Clinical characteristics and outcome of patients with neuroblastoma presenting genomic amplification of loci other than MYCN.

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    Anne Guimier

    Full Text Available BACKGROUND: Somatically acquired genomic alterations with MYCN amplification (MNA are key features of neuroblastoma (NB, the most common extra-cranial malignant tumour of childhood. Little is known about the frequency, clinical characteristics and outcome of NBs harbouring genomic amplification(s distinct from MYCN. METHODS: Genomic profiles of 1100 NBs from French centres studied by array-CGH were re-examined specifically to identify regional amplifications. Patients were included if amplifications distinct from the MYCN locus were seen. A subset of NBs treated at Institut Curie and harbouring MNA as determined by array-CGH without other amplification was also studied. Clinical and histology data were retrospectively collected. RESULTS: In total, 56 patients were included and categorised into 3 groups. Group 1 (n = 8 presented regional amplification(s without MNA. Locus 12q13-14 was a recurrent amplified region (4/8 cases. This group was heterogeneous in terms of INSS stages, primary localisations and histology, with atypical clinical features. Group 2 (n = 26 had MNA as well as other regional amplifications. These patients shared clinical features of those of a group of NBs MYCN amplified (Group 3, n = 22. Overall survival for group 1 was better than that of groups 2 and 3 (5 year OS: 87.5%±11% vs 34.9%±7%, log-rank p<0.05. CONCLUSION: NBs harbouring regional amplification(s without MNA are rare and seem to show atypical features in clinical presentation and genomic profile. Further high resolution genetic explorations are justified in this heterogeneous group, especially when considering these alterations as predictive markers for targeted therapy.

  10. Clinical characteristics and outcome of Penicillium marneffei infection among HIV-infected patients in northern Vietnam

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    Larsson Mattias

    2012-08-01

    Full Text Available Abstract Objective This study reports the clinical characteristics and outcome of HIV-associated Penicilliummarneffei infection in northern Vietnam. Methods We conducted a retrospective chart review of all patients with laboratory confirmed Penicilliummarneffei infection admitted to the National Hospital for Tropical Diseases in Hanoi, Vietnam, between July 2006 and September 2009. Results 127 patients with P. marneffei infection were identified. All were HIV-infected; median CD4+ T-cell count was 24 cells/μl (IQR:12-48; 76% were men. Common clinical features were fever (92.9%, skin lesions (82.6%, hepatomegaly (61.4%, lymphadenopathy (40.2%, weight loss (59.1% and cough (49.6%. Concurrent opportunistic infections were present in 22.0%; half of those had tuberculosis. Initial treatment regimens were: itraconazole or ketoconazole capsule (77.2%, amphotericin B (20.5%, and fluconazole (1.6%. In-hospital mortality was 12.6% and showed no significant difference in patients treated with itraconazole (or ketoconazole and amphotericin B (p = 0.43. Dyspnea, ascites, and increased LDH level were independent predictors of mortality. No seasonality was observed. Conclusion The clinical features, treatments and outcomes of HIV-associated P. marneffei infection in northern Vietnam are similar to those reported in other endemic regions. Dyspnea was an important predictor of mortality. More patients were treated with itraconazole than amphotericin B and no significant difference in treatment outcome was observed. It would be of clinical value to compare the efficacy of oral itraconazole and amphotericin B in a clinical trial.

  11. Different Pathophysiological Phenotypes among Newly Diagnosed Type 2 Diabetes Patients

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    Stidsen, Jacob

    2013-01-01

    Type 2 diabetes (T2D) can be considered a syndrome with several different pathophysiological mechanisms leading to hyperglycemia. Nonetheless, T2D is treated according to algorithms as if it was one disease entity. Methods: We investigated the prevalence of different pathophysiological phenotypes...... among newly diagnosed T2D patients in Denmark. Based on baseline data from a Danish national cohort study we investigated 1048 incident diagnosed T2D patients. The diagnosis T2D was made by general practitioners based on clinical judgement. Phenotypes were classified in the following groups: latent...... autoimmune diabetes (LADA) (GAD antibody titer >= 20 IE/ml and not T1D), secondary diabetes (recent history of pancreatitis, pancreatectomy or pancreas amylase > 65U/l, and GAD negativity), steroid-induced diabetes (oral glucocorticoid-treated subjects), insulinopenic (f-P-C-peptide

  12. Purple pigments: the pathophysiology of acute porphyric neuropathy.

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    Lin, Cindy S-Y; Lee, Ming-Jen; Park, Susanna B; Kiernan, Matthew C

    2011-12-01

    The porphyrias are inherited metabolic disorders arising from disturbance in the haem biosynthesis pathway. The neuropathy associated with acute intermittent porphyria (AIP) occurs due to mutation involving the enzyme porphobilinogen deaminase (PBGD) and is characterised by motor-predominant features. Definitive diagnosis often encompasses a combination of biochemical, enzyme analysis and genetic testing, with clinical neurophysiological findings of a predominantly motor axonal neuropathy. Symptomatic and supportive treatment are the mainstays during an acute attack. If administered early, intravenous haemin may prevent progression of neuropathy. While the pathophysiology of AIP neuropathy remains unclear, axonal dysfunction appears intrinsically linked to the effects of neural energy deficits acquired through haem deficiency coupled to the neurotoxic effects of porphyrin precursors. The present review will provide an overview of AIP neuropathy, including discussion of recent advances in understanding developed through neurophysiological approaches that have further delineated the pathophysiology of axonal degeneration.

  13. Facial Erythema of Rosacea - Aetiology, Different Pathophysiologies and Treatment Options.

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    Steinhoff, Martin; Schmelz, Martin; Schauber, Jürgen

    2016-06-15

    Rosacea is a common chronic skin condition that displays a broad diversity of clinical manifestations. Although the pathophysiological mechanisms of the four subtypes are not completely elucidated, the key elements often present are augmented immune responses of the innate and adaptive immune system, and neurovascular dysregulation. The most common primary feature of all cutaneous subtypes of rosacea is transient or persistent facial erythema. Perilesional erythema of papules or pustules is based on the sustained vasodilation and plasma extravasation induced by the inflammatory infiltrates. In contrast, transient erythema has rapid kinetics induced by trigger factors independent of papules or pustules. Amongst the current treatments for facial erythema of rosacea, only the selective α2-adrenergic receptor agonist brimonidine 0.33% topical gel (Mirvaso®) is approved. This review aims to discuss the potential causes, different pathophysiologies and current treatment options to address the unmet medical needs of patients with facial erythema of rosacea.

  14. The Relation between Diverse Phenotypes of PCOS with Clinical Manifestations, Anthropometric Indices and Metabolic Characteristics.

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    Shahrami, Seyedeh Hajar; Abbasi Ranjbar, Zahra; Milani, Forozan; Kezem-Nejad, Ehsan; Hassanzadeh Rad, Afagh; Dalil Heirat, Seyedeh Fatemeh

    2016-02-01

    Critical issue regarding to variation of findings based on different phenotypes led investigators to define whether they are distinct features or overlapping ones. Therefore, we aimed to investigate the association between diverse phenotypes of PCOS (Poly Cystic Ovary Syndrome) with clinical manifestations, anthropometric indices, and metabolic characteristics. This was a descriptive cross-sectional study conducted in 15-39 years old women with PCOS referred to infertility clinics in the north part of Iran, Rasht during 2010-2011. Data were gathered through an interview by a form consisted of demographic characteristics, laboratory findings, ovarian volume and anthropometric indices. A total of 214 patients consisted of 161 PCOS (cases) and 53 normal women (controls) participated in this study. The most prevalent phenotype in PCOS population was IM/PCO/HA (54%), followed by IM/HA (28%) and IM/PCO (13%). PCO/HA was present only in 6 PCOS patients (5%). PCOS patients were significantly younger than controls (P=0.07). Results showed that increased ovarian volume were higher in PCOS group in comparison with controls and IM/PCO/HA, and IM/PCO had respectively the largest ovarian volumes. Also, a significant relation was observed based on Cholesterol, 17OHP, LH, TG, 2hpp, and LH/FSH between patients with PCOS and control groups. There were significant differences in demographic, anthropometric, hormonal and ultrasound findings between PCOS and controls. Therefore, it seems that classification of the characteristics of each phenotype could offer an appropriate guide for screening risks of PCOS and may facilitate performing most favorable treatment for these complications.

  15. Early clinical characteristics according to developmental stage in children with definite moyamoya disease.

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    Kim, Young Ok; Joo, Sung-Pil; Seo, Bo-Ra; Rho, Young Il; Yoon, Woong; Woo, Young Jong

    2013-06-01

    The objective is to clarify the early clinical characteristics in childhood moyamoya disease (MD). Epidemiologic characteristics, symptoms and diagnostic rates were assessed in 64 children (0-18 years) with definite MD according to developmental stage: infancy (5; 0-1 years); toddlerhood/preschool age (22; 2-5 years); school age (29; 6-10 years); and adolescence (8; 11-18 years). The median ages at onset was 6.25 years and the female to male ratio was 1.9 (~2.5 in toddlerhood/preschool age and in adolescence, P=0.71). Previous headache was observed in 23% (14/64): frequently in school age (38%, P=0.02) and within 6 months before main symptoms (6/11). As an initial symptom, weakness was observed in 78% (50/64) mainly as transient ischemic attack (TIA, 61%) in limbs (90%) and unilaterally (82%). TIA was less frequent in infancy (40%, P=0.04). Seizure was observed in 27% (17/64): frequently in infancy (100%, Pchildren ~5 years (P<0.01). Severe headache associated with MD was observed in 14% (9/64). Provoking events were positive in 42% (27/64): in school age, frequently during eating (28%); and in toddlerhood/preschool age, during crying (27%). The diagnostic rates at 3 and 12 months from symptom-onset were 39% (80% during infancy vs. 28% in school age, P=0.14) and 67%, respectively. Symptomatic progression at diagnosis was observed in 38% (24/64). Initial clinical characteristics in childhood definite MD differed according to developmental stage and from at diagnosis.

  16. Analysis of clinical characteristics of 96 patients with acute superior mesenteric venous thrombosis

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    Wen-hui LIU

    2015-06-01

    Full Text Available Objective To investigate the clinical characteristics of patients suffering from acute superior mesenteric venous thrombosis (ASMVT. Methods Clinical data of 96 ASMVT patients admitted to the PLA General Hospital from January 2000 to December 2013 were retrospectively analyzed. Clinical characteristics and death-associated risk factors were studied, and the influence of treatment strategy and thrombosis location on patients' outcome were analyzed. Results The patients were divided into survival group (n=83 and death group (n=13 according to the outcome. The mean age was 46.9 years old, and the ratio of male/female was 3:1. Thirty-nine patients presented isolated superior mesenteric venous thrombosis (SMVT and fiftyseven patients presented combined SMVT. In the death group, higher incidence of severe acute pancreatitis and isolated SMVT were found than the survival group (P<0.01, P=0.004. The patients were again divided into laparotomy group, interventional thrombolysis group, and conservative treatment group according to treatment modality. The interval between symptom onset and treatment was shorter, the incidence of isolated SMVT and mortality rate were higher in the laparotomy group compared with those in interventional thrombolysis group and conservative treatment group. There was no death in the conservative treatment group. In comparison with the combined SMVT group, more patients in the isolated SMVT group presented peritoneal signs and less with history of splenectomy (P<0.001, P=0.002. The proportion of patients with laparotomy and bowel necrosis in the isolated SMVT group was higher than those in the combined SMVT group (P=0.023, P=0.012. Conclusions Patients with isolated SMVT are more likely to have peritoneal signs and bowel necrosis, surgical treatment is mandatory. Patients with combined SMVT often have a history of splenectomy. ASMVT patients with severe pancreatitis may present higher mortality rate. DOI: 10.11855/j

  17. [Clinical characteristics research of shenmai injection treating tumor based on hospital information system in real world].

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    Hu, Yuan-Chun; Xie, Yan-Ming; Yang, Wei; Wang, Yong-Yan; Wang, Lian-Xin; Tang, Hao; Zhuang, Yan

    2014-09-01

    The study was to research the clinical characteristics of Shenmai injection treating tumor based on hospital information system, including the characteristics of the age, the sex, the dosage, the course of the treatment and the combination drugs. The data of tumor patients injected with Shenmai injection was analyzed. The information was collected from the hospital information system (HIS) in twenty hospitals of grade III-A. The method of frequencies and association rules was used in this reaearch. The patients over 45 years old were up to 3 338, about 79.36% of the whole. The ratio of male and female was 1.73: 1. The hospitalization day between 15 and 28 was most. The complications of the hypertension and coronary heart disease happened most. The support was 5.939% and 5.099% respectively. Fifty-five patients had the traditional Chinese medicine (TCM) syndrome of Qi-Yin deficiency, about 14.78% of the whole. There were 8 491 patients treated with the single dose of 81 to 100 mL, about 48.70% of the whole. The main combination drugs were dexamethasone, tropisetron and maxolon. The confidence was 44.63%, 31.22% and 20.53% respectively. The information from HIS showed that tumor patients used Shenmai injection were most quinquagenarian with smooth condition. The dose of the Shenmai injection sometimes was higher than that of the drug use instructions in clinical. Shenmai injection was most often combined with glucocorticoid, antemetic and nutritional support medicine when treating tumor in clinical.

  18. Clinical Characteristics and Metabolic Features of Patients with Adrenal Incidentalomas with or without Subclinical Cushing's Syndrome

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    Bo-Yeon Kim

    2014-12-01

    Full Text Available BackgroundThe aim of this study was to examine the clinical characteristics of adrenal incidentalomas discovered by computed tomography (CT and to investigate metabolic features of subclinical Cushing's syndrome (SCS in patients with adrenal incidentalomas in a tertiary hospital in Korea.MethodsThis retrospective study examined the clinical aspects of 268 patients with adrenal incidentalomas discovered by CT at Soonchunhyang University Bucheon Hospital. Clinical data and endocrine function of the patients as well as histological findings were obtained from medical records, while anatomic characteristics were analyzed by reviewing imaging studies. Hormonal tests for pheochromocytoma, Cushing's syndrome, and aldosterone-secreting adenoma were performed.ResultsMost (n=218, 81.3% cases were nonfunctioning tumors. Of the 50 patients with functioning tumors (18.7%, 19 (7.1% were diagnosed with SCS, nine (3.4% with overt Cushing's syndrome, 12 (4.5% with primary aldosteronism, and 10 (3.7% with pheochromocytoma. Malignant tumors (both primary and metastatic were rare (n=2, 0.7%. Body mass index, fasting glucose, hemoglobin A1c, and total cholesterol were significantly higher in patients with SCS in comparison with those with nonfunctioning tumors. The prevalence of type 2 diabetes mellitus and hypertension were significantly higher in patients with SCS compared with those with nonfunctioning tumors.ConclusionFunctioning tumors, especially those with subclinical cortisol excess, are commonly found in patients with adrenal incidentalomas, although malignancy is rare. In addition, patients with SCS in adrenal incidentalomas have adverse metabolic and cardiovascular profiles.

  19. Clinical characteristics of myotonia congenita in China Literature analysis of the past 30 years

    Institute of Scientific and Technical Information of China (English)

    Yangwei Zhang; Shushan Zhang; Huifang Shang

    2008-01-01

    AIM:To understand the distinct,clinical features of myotonia congenita in China.METHODS:Case reports of myotonia congenita were retrieved from the Chinese Journal Full-text database,dating between 1980 and 2007,and analyzed for clinical characteristics of myotonia congenita. RESULTS:There were 35 published reports and 258 cases about myotonia congenita.Six reports(62 cases)were excluded due to lack of clinical data,imprecise diagnosis,or duplication.Finally,29 published reports and 196 cases(140 males and 56 females)were included in this analysis.About 78.6% of patients were diagnosed with myotonia congenita before the age of 20,and among these, 86.1% were classified as dominant inheritance.Lower and upper extremities were frequently affected with severe symptoms.Eyelids,mouth and lingual muscles,and trunk muscles and cervical muscles were less frequently involved.However,muscles for swallowing,sphincter muscles,and smoothmuscles were not involved.There were no reports of cataracts,cardiac conduction block,or dyscrinism. Myotonia congenita symptoms were induced or aggravated by cold temperatures in 71.9% of the patients and warming-up effect occurred in 95.6% of the patients.Muscle hypertrophy was observed in 69.6% and percussion of muscles in 76.5% of the patients.Myotonia potential or myotonia-likepotential was detected in all patients using electromyography.Muscle fiber swelling or hypertrophy was frequently detected through muscular biopsy.CONCLUSION:Myotonia congenita frequently occurs in males before the age of 20,in particular as the autosomal dominant form of myotonia congenita.Skeletal muscles throughout the body,especially the lower and upper extremities,are involved.However,muscles for swallowing,sphincter muscles, and smooth muscles are not involved.Warming-up effect is the main characteristic of myotonia congenita.

  20. A single hospital study on portal vein thrombosis in cirrhotic patients - clinical characteristics & risk factors

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    Huisong Chen

    2014-01-01

    Full Text Available Background & objectives: Discrepancies exist in the reported prevalence of portal vein thrombosis (PVT, and its clinical characteristics and sites of occurrence need to be elucidated. The risk factors for PVT are also poorly understood. This single centre study was undertaken to determine the clinical characteristics, sites of occurrence, and risk factors associated with PVT in patients with liver cirrhosis. Methods: Hospitalized cirrhotic patients (N = 162 were segregated into the PVT and non-PVT groups. Indices possibly associated with PVT were measured and PVT was detected by both Doppler ultrasonography and computed tomography portal angiography. The portal vein diameter and flow velocity and splenic thickness were measured by ultrasonography. Results: PVT was found in 40 patients (24.7%; in 34 PVT patients (85%, the liver cirrhosis resulted from hepatitis B virus infections. Most (90% patients were Child-Pugh classes B and C, with similar distribution between the groups. PVT was seen in 20 patients in the portal and superior mesenteric veins; ascites, abdominal pain, gastrointestinal bleeding, and jaundice were common findings in PVT patients. Haemoglobin levels and blood platelet counts (BPCs were significantly lower and splenic thickness was greater in PVT than in non-PVT patients (P<0.01. There was a significant positive correlation between BPCs and platelet aggregation rates (R = 0.533, P<0.01. Interpretation & conclusions: The occurrence of PVT was 24.7 per cent, primarily in post-hepatitis B liver cirrhosis patients. PVT occurred mainly in the portal vein trunk and superior mesenteric vein. Different PVT sites may account for the differing clinical presentations. The lower levels of haemoglobin and BPCs as well as splenic thickening were associated with PVT. Splenic thickening may be a risk factor for PVT.

  1. [Analysis of clinical characteristics in 45 cases of Diamond-Blackfan anemia].

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    Chen, Yu-Mei; Ruan, Min; Wang, Ya-Qin; Zou, Yao; Zhang, Li; Liu, Tian-Feng; Zhu, Xiao-Fan

    2012-06-01

    In order to explore the diagnosis and therapy of Diamond Blackfan anemia (DBA), the clinical data of 45 cases of DBA admitted in our hospital from February 1994 to July 2011 were analyzed retrospectively. The clinical characteristics, results of laboratory examination, treatment reaction and outcome of disease were investigated. The results indicated that out of 45 children diagnosed as DBA, 14 cases (31.1%) had short stature and physical malformation. All patients had anemia with reticulocytopenia. Thirty-four patients (75.6%) had mean corpuscular volume. Eleven patients (24.4%) had macrocytic anemia. Bone marrow examination showed a marked erythroid hypoplasia in all patients. Out of 29 cases tested for fetal hemoglobin (HbF), 13 cases (44.8%) had high level of HbF. Erythroid colony-forming unit of bone marrow was tested in 25 patients, among them 12 patients (48%) showed normal plasia, 13 (52%) showed hypoplasia. The erythropoietin (EPO) levels of 17 patients were elevated. Karyotypes were examined in 28 patients, and showed all normal. The treatment was based on corticosteroids and Cyclosporine A. Thirty patients had good response to corticosteroid therapy, and 10 of them obtained a sustained corticosteroid-induced remission. Twenty cases discontinued corticosteroid therapy after remission, as a result, 15 cases (75%) relapsed, moreover all the relapsed cases still had good response to corticosteroid. Two relapsed patients suffered from aplastic anemia, one of them died of therapy failure. Six patients were unresponsive to corticosteroid, 1 of which achieved remission with cyclosporine A and the others continued to receive regular transfusions. 3 patients received iron chelation therapy. It is concluded that the clinical characteristics, complete blood count, bone marrow smear, HbF level and EPO level are useful to make a diagnosis of DBA. Most patients have a good response to corticosteroid therapy, but relapse rate is high when drug was discontinued. Patients

  2. Clinical and demographic characteristics of patients with urinary tract hydatid disease.

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    Mou Huang

    Full Text Available BACKGROUND: Human cystic echinococcosis (CE is caused by flatworm larvae of Echinococcus granulosus and is endemic in many parts of the world. In humans, CE cysts primarily affect the liver and pulmonary system, but can also affect the renal system. However, the clinical manifestations of renal CE can be subtle, so healthcare professionals often overlook renal CE in differential diagnosis. In this study, we examined the clinical and demographic characteristics of patients with urinary tract CE and analyzed the diagnosis and treatment procedures for this disease. METHODS: The records of 19 consecutive renal CE patients who were admitted to the First Affiliated Hospital of Xinjiang Medical University from January 1983 to April 2011 were retrospectively reviewed. In all cases, CE of the urinary tract was confirmed by pathological examination and visual inspection during surgery. RESULTS: Fifteen patients were males and 4 were females. The most common symptoms were non-specific lower back pain and percussion tenderness on the kidney region. All patients were followed up for 9-180 months after surgery. None of the patients experienced a recurrence of renal CE, but 4 patients experienced non-renal recurrence of hydatid disease. CONCLUSIONS: Hydatid cysts from E. granulosus are structurally similar in the liver and urinary tract. Thus, the treatment regimen for liver CE developed by the World Health Organization/Informal Working Group on Echinococcosis (WHO/IWGE could also be used for urinary tract CE. In our patients, the use of ultrasound, computed tomography, serology, and clinical characteristics provided a diagnostic accuracy of 66.7% to 92.3%.

  3. Lactose intolerance in infants with gluten-sensitive enteropathy: Frequency and clinical characteristics

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    Radlović Nedeljko

    2009-01-01

    Full Text Available Introduction. Secondary lactose intolerance (SLI belongs to the rarer manifestations of gluten-sensitive enteropathy (GSE. It occurs in more severe forms of the disease and its presence contributes significantly to the degree of its expression. Objective. The goal of the study was to determine the frequency of SLI in infants with clinically classic form of GSE, as well as its relationship with the duration, severity and age at the diagnosis of the basic disease and the degree of small bowel mucosa damage. Methods. The study was based on a sample of 42 infants, 30 female and 12 male, aged 7-12 months (x=9.98±1.69, with a clinically classic form of GSE. The diagnosis of GSE was established based on the characteristic pathohistological appearance of small bowel mucosa and clinical improvement of patients on gluten-free diet, while SLI on pathological lactose or milk tolerance test. The assessment of basic disease severity was based on body mass divergence in relation to the standard value, as well as on Hb and serum iron levels, while the degree of small bowel mucosa damage was determined according to the modified Marsh criteria. Results. SLI was verified in 8/42 or 19.05% of patients. In addition to the symptoms and clinical signs of GSE, all the patients with SLI also featured the problems characteristic of lactose tolerance disorders, i.e. watery diarrhoea, borborygmus and meteorism occurring after milk meals. In addition, all had perianal erythema (6 with erosive changes, as well as destructive enteropathy (5 subtotal and 3 total. The difference in the duration of the basic disease, age at diagnosis, as well as in the degree of body mass deviation from the standard value between the lactose-tolerant and lactose-intolerant infants was not found. In addition, no difference in Hb and serum iron levels or in the degree of small bowel mucosa damage was found between the two groups. Conclusion. Our findings indicate that SLI presents a relatively

  4. Non-typhoidal Salmonella bacteraemia: Epidemiology, clinical characteristics and its' association with severe immunosuppression

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    Fatt Quek

    2009-05-01

    Full Text Available Abstract Background Non-typhoidal Salmonella (NTS is increasingly recognized as an important pathogen associated with bacteraemia especially in immunosuppressed patients. However, there is limited data specifically describing the clinical characteristics and outcome amongst the immunosuppressed patients. Methods A total of 56,707 blood culture samples and 5,450 stool samples were received by the microbiology laboratory at a tertiary referral hospital in Malaysia, during a 4-year study period. Out of these samples, 55 non-duplicate NTS isolates were identified from blood and 121 from stool. A retrospective analysis of the 55 patients with NTS bacteraemia was then conducted to determine the predominant NTS serovars causing bacteraemia and its' blood invasive potential, epidemiological data, clinical characteristics and antimicrobial susceptibility. Patients were then grouped as immunosuppressed and non-immunosuppressed to determine the association of severe immunosuppression on clinical features. Data was analyzed using the Statistical Package for Social Sciences (SPSS version 15.0 using the non-parametric Mann-Whitney test, Fisher's exact test or Chi-squared test. The odds ratio (OR and its 95% confidence intervals (CI were calculated. The P-value Results Out of 55 NTS bacteraemia cases identified, 81.8% (45/55 were community-acquired. Salmonella enterica serovar Enteritidis had the highest blood invasiveness. An extra-intestinal focus of infection was noted in 30.9% (17/55 of the patients, most commonly involving the lungs and soft tissue. 90.9% (50/55 of the patients had an underlying disease and 65.5% (36/55 of the patients had severe clinical immunosuppressive condition with malignancy and HIV being the most common. Immunosuppressed patients had higher mortality (P = 0.04, presented more commonly with primary bacteraemia (P = 0.023, leukopenia (P = 0.001 and opportunistic infections (P = 0.01. In contrast, atherosclerotic conditions (P = 0

  5. Childhood-onset pathologic skin picking: clinical characteristics and psychiatric comorbidity.

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    Odlaug, Brian L; Grant, Jon E

    2007-01-01

    There has been little research examining clinical correlates of childhood-onset pathologic skin picking in a sample of individuals with a primary diagnosis of pathologic skin picking. Using a sample of 40 consecutive subjects with current pathologic skin picking, we compared subjects with childhood-onset (before 10 years of age) pathologic skin picking to those with later onset on a variety of clinical measures. Symptom severity was examined by assessing time spent picking per day, intensity and frequency of thoughts and urges to pick, and social and occupational functioning. Of the 40 subjects, 19 (47.5%) reported onset of skin picking before 10 years of age. Subjects with childhood-onset had significantly longer durations of illness before receiving treatment and were more likely to pick unconsciously. Symptom severity, comorbidity, and social functioning did not differ between groups. These preliminary results suggest that although onset before 10 years of age is fairly common among people with pathologic skin picking, individuals developing this behavior earlier in life have similar clinical characteristics as those with later onset but may be less likely to seek treatment.

  6. Phenotypic Characteristics Associated with Virulence of Clinical Isolates from the Sporothrix Complex

    Science.gov (United States)

    Almeida-Paes, Rodrigo; de Oliveira, Luã Cardoso; Oliveira, Manoel Marques Evangelista; Gutierrez-Galhardo, Maria Clara; Nosanchuk, Joshua Daniel; Zancopé-Oliveira, Rosely Maria

    2015-01-01

    The Sporothrix complex members cause sporotrichosis, a subcutaneous mycosis with a wide spectrum of clinical manifestations. Several specific phenotypic characteristics are associated with virulence in many fungi, but studies in this field involving the Sporothrix complex species are scarce. Melanization, thermotolerance, and production of proteases, catalase, and urease were investigated in 61 S. brasiliensis, one S. globosa, and 10 S. schenckii strains. The S. brasiliensis strains showed a higher expression of melanin and urease compared with S. schenckii. These two species, however, presented similar thermotolerances. Our S. globosa strain had low expression of all studied virulence factors. The relationship between these phenotypes and clinical aspects of sporotrichosis was also evaluated. Strains isolated from patients with spontaneous regression of infection were heavily melanized and produced high urease levels. Melanin was also related to dissemination of internal organs and protease production was associated with HIV-coinfection. A murine sporotrichosis model showed that a S. brasiliensis strain with high expression of virulence factors was able to disseminate and yield a high fungal burden in comparison with a control S. schenckii strain. Our results show that virulence-related phenotypes are variably expressed within the Sporothrix complex species and might be involved in clinical aspects of sporotrichosis. PMID:25961005

  7. Clinical and immunological characteristics of hemorrhagic fever with renal syndrome in women of different age groups

    Directory of Open Access Journals (Sweden)

    Kutdusova A.M.

    2012-09-01

    Full Text Available Objective: To evaluate the clinical and immunological features of the hemorrhagic fever with renal syndrome in women of different age groups. Materials and methods: Clinical and laboratory characteristics of hemorrhagic fever with renal syndrome in 148 women aged 17 to 65 years old have been investigated. Patients have been divided into two groups: group I included 101 patients with normal menstrual rhythm, group II included 47 female patients with menopause. In 57 women (36 from group I, 21 — from group II the content of CD3+, CD4+, CD8+, CD16+, CD19+ — sub-populations of peripheral blood lymphocytes has been determined. Results: In compared groups significant differences in structure and frequency of complications of the disease have been revealed. Unidirectional tendency to increase significantly reduced absolute rates of investigated lymphocyte subpopulations in dynamics of the disease has been identified. It also has been stated that by the time of early convalescence in case of severe form of HFRS the indices did not reach the standard level. In an older group of women deeper damage and long-term recovery of immune system have been marked. Conclusion: According to the results of clinical and immunological studies the research work has revealed that in young women the response of the immune system to HFRS has developed faster and stronger than that in patients during the menopause period.

  8. Phenotypic characteristics associated with virulence of clinical isolates from the Sporothrix complex.

    Science.gov (United States)

    Almeida-Paes, Rodrigo; de Oliveira, Luã Cardoso; Oliveira, Manoel Marques Evangelista; Gutierrez-Galhardo, Maria Clara; Nosanchuk, Joshua Daniel; Zancopé-Oliveira, Rosely Maria

    2015-01-01

    The Sporothrix complex members cause sporotrichosis, a subcutaneous mycosis with a wide spectrum of clinical manifestations. Several specific phenotypic characteristics are associated with virulence in many fungi, but studies in this field involving the Sporothrix complex species are scarce. Melanization, thermotolerance, and production of proteases, catalase, and urease were investigated in 61 S. brasiliensis, one S. globosa, and 10 S. schenckii strains. The S. brasiliensis strains showed a higher expression of melanin and urease compared with S. schenckii. These two species, however, presented similar thermotolerances. Our S. globosa strain had low expression of all studied virulence factors. The relationship between these phenotypes and clinical aspects of sporotrichosis was also evaluated. Strains isolated from patients with spontaneous regression of infection were heavily melanized and produced high urease levels. Melanin was also related to dissemination of internal organs and protease production was associated with HIV-coinfection. A murine sporotrichosis model showed that a S. brasiliensis strain with high expression of virulence factors was able to disseminate and yield a high fungal burden in comparison with a control S. schenckii strain. Our results show that virulence-related phenotypes are variably expressed within the Sporothrix complex species and might be involved in clinical aspects of sporotrichosis.

  9. A Content Analysis of Preconception Health Education Materials: Characteristics, Strategies, and Clinical-Behavioral Components

    Science.gov (United States)

    Levis, Denise M.; Westbrook, Kyresa

    2015-01-01

    Purpose Many health organizations and practitioners in the United States promote preconception health (PCH) to consumers. However, summaries and evaluations of PCH promotional activities are limited. Design We conducted a content analysis of PCH health education materials collected from local-, state-, national-, and federal-level partners by using an existing database of partners, outreach to maternal and child health organizations, and a snowball sampling technique. Setting Not applicable. Participants Not applicable. Method Thirty-two materials were included for analysis, based on inclusion/exclusion criteria. A codebook guided coding of materials’ characteristics (type, authorship, language, cost), use of marketing and behavioral strategies to reach the target population (target audience, message framing, call to action), and inclusion of PCH subject matter (clinical-behavioral components). Results The self-assessment of PCH behaviors was the most common material (28%) to appear in the sample. Most materials broadly targeted women, and there was a near-equal distribution in targeting by pregnancy planning status segments (planners and nonplanners). “Practicing PCH benefits the baby’s health” was the most common message frame used. Materials contained a wide range of clinical-behavioral components. Conclusion Strategic targeting of subgroups of consumers is an important but overlooked strategy. More research is needed around PCH components, in terms of packaging and increasing motivation, which could guide use and placement of clinical-behavioral components within promotional materials. PMID:23286661

  10. Clinical characteristics of in-situ pulmonary artery thrombosis in Korea.

    Science.gov (United States)

    Cha, Seung-Ick; Choi, Keum-Ju; Shin, Kyung-Min; Lim, Jae-Kwang; Yoo, Seung-Soo; Lee, Jaehee; Lee, Shin-Yup; Kim, Chang-Ho; Park, Jae-Yong

    2015-12-01

    Little is known regarding the clinical features and course of in-situ pulmonary artery thrombosis (PAT). The aim of the present study was to investigate the clinical characteristics of PAT. Patients with PAT were retrospectively identified from a tertiary referral center in South Korea. A control group consisted of patients with pulmonary embolism in whom the right or left pulmonary artery was the largest pulmonary embolism-involved site. We compared various clinical parameters between the two groups. Of the 23 PAT patients, the most common underlying condition was tuberculosis-destroyed lung [11 (47.8%)], followed by pulmonary artery stump after lobectomy or pneumonectomy [7 (30.4%)]. In all patients except one, PAT was located in the right or left pulmonary artery. Computed tomography scans demonstrated that clots were completely or partially resolved less frequently in the PAT group than in the control group [4 (25%) versus 62 (90%); P PAT group, three of the seven patients (43%) who had undergone anticoagulation therapy exhibited improvement, and one of the nine patients (11%) who had not received anticoagulation therapy experienced improvement; however, the difference was not significant. Tuberculosis-destroyed lung was the most common underlying condition in Korean PAT patients, followed by pulmonary artery stump after lung resection. The clots in patients with PAT were mostly located in the right or left pulmonary artery, and clot resolution was less frequent in the PAT group compared to the pulmonary embolism group.

  11. The clinical characteristics and treatment for sudden sensorineural hearing loss with vestibular schwannoma.

    Science.gov (United States)

    Lin, Chang; Gong, Qilin; Zuo, Wenjing; Zhang, Rong; Zhou, Aidong

    2015-04-01

    The aim of this study is to analyze the clinical characteristics and treatment of sudden sensorineural hearing loss (SSNHL) patients with vestibular schwannoma (VS). The clinical features of the VS patients were explored by retrospectively analyzing the clinical data from 542 cases of SSNHL patients between January 2008 and March 2013. There were 10 cases (10 ears) diagnosed with VS in 542 cases of SSNHL patients (10 ears, 1.85 %), 3 males, 7 females, with a range of 28-57 years. Among all the cases, eight patients with abnormal ABR, ten with ear ipsilateral stapedius reflexes which were completely not elicited and seven patients with healthy ear contralateral stapedius reflexes which were completely not elicited. Neuromas were classified by Koos grades according to size (8 of grade I, 1 of grade II, 1 of grade IV). Eight small VS  patients were taken waiting and MRI therapy strategies. Meanwhile, we used glucocorticoid treatment and timely and short-term medication to improve the microcirculation of the inner ear for these patients. And four cases' hearing was improved. Some vestibular schwannomas have SSNHL as initial symptoms, especially the small ones in internal auditory canal. To prevent misdiagnosis or leak-diagnosis, MRI should be performed as a routine test for SSNHL, and ABR is sometimes necessary for SSNHL patients. It is also necessary to give appropriate treatment to protect hearing of the small vestibular schwannoma patients whose first symptoms are diagnosed as SSNHL in acute phase.

  12. Clinical and pathological characteristics of primary intraspinal hemangiopericytoma and choice of treatment

    Institute of Scientific and Technical Information of China (English)

    ZHAO Yan; ZHAO Ji-zong

    2007-01-01

    Background Primary intraspinal hemangiopericytoma is a rare malignant mesenchymal tumor with high rates of recurrence and metastasis. Surgery is the main therapeutic procedure for this lesion. This clinical research was undertaken to analyze the pathological characteristics, clinical course, and the choice of treatment for this lesion. Methods Twenty-three patients with primary intraspinal hemangiopericytomas were treated from 1987 to 2004. The clinical and imaging features, pathological findings, therapeutic procedures, and prognosis were analyzed retrospectively. Results Primary intraspinal hemangiopericytoma is more likely to attack middle-aged persons. The tumor mainly manifests as muscle weakness and sensor abnormalities. Microscopic examination showed slit-like vascular spaces and oral- or spindle-shaped cells with slightly acidic cytoplasm and oral nuclei. Tumors were subtotally resected in 11 patients, subtotally resected with postoperative radiotherapy in 4, totally resected in 5, and totally resected with postoperative radiotherapy in 3. Two patients were given spinal stabilization after total resection. Recurrence and metastatic rates were 50% and 0 in intradural patients. They were 73% and 27% in extradural patients, respectively. Conclusions The tumor should be resected en bloc with the neighboring dural mater to reduce recurrence and metastasis. Patients with subtotal resection need adjuvant radiotherapy. Patients with evident spinal involvement may benefit from spinal stabilization. The prognosis of the lesion arising from the dural mater is better.

  13. Phenotypic Characteristics Associated with Virulence of Clinical Isolates from the Sporothrix Complex

    Directory of Open Access Journals (Sweden)

    Rodrigo Almeida-Paes

    2015-01-01

    Full Text Available The Sporothrix complex members cause sporotrichosis, a subcutaneous mycosis with a wide spectrum of clinical manifestations. Several specific phenotypic characteristics are associated with virulence in many fungi, but studies in this field involving the Sporothrix complex species are scarce. Melanization, thermotolerance, and production of proteases, catalase, and urease were investigated in 61 S. brasiliensis, one S. globosa, and 10 S. schenckii strains. The S. brasiliensis strains showed a higher expression of melanin and urease compared with S. schenckii. These two species, however, presented similar thermotolerances. Our S. globosa strain had low expression of all studied virulence factors. The relationship between these phenotypes and clinical aspects of sporotrichosis was also evaluated. Strains isolated from patients with spontaneous regression of infection were heavily melanized and produced high urease levels. Melanin was also related to dissemination of internal organs and protease production was associated with HIV-coinfection. A murine sporotrichosis model showed that a S. brasiliensis strain with high expression of virulence factors was able to disseminate and yield a high fungal burden in comparison with a control S. schenckii strain. Our results show that virulence-related phenotypes are variably expressed within the Sporothrix complex species and might be involved in clinical aspects of sporotrichosis.

  14. Clindamycin-resistant methicillin-resistant Staphylococcus aureus: epidemiologic and molecular characteristics and associated clinical factors.

    Science.gov (United States)

    Cadena, Jose; Sreeramoju, Pranavi; Nair, Shalini; Henao-Martinez, Andres; Jorgensen, James; Patterson, Jan E

    2012-09-01

    In this prospective, observational study of 618 consecutive adult patients with skin and soft tissue infections (SSTI) caused by methicillin-resistant Staphylococcus aureus (MRSA), the clinical characteristics, molecular epidemiology, and outcome of patients with clindamycin-resistant MRSA (n = 64) and clindamycin-susceptible MRSA (n = 554) were compared (including factors predictive of clindamycin-resistant MRSA SSTI). Patients with clindamycin-resistant MRSA were more likely to have had antibiotic exposure within 3 months (37.5% versus 17%, P < 0.01), surgery (25% versus 8%, P < 0.01), MRSA infection/colonization within 12 months (23% versus 7%, P < 0.01), or intravascular catheters (5% versus 0.5%, P = 0.02). On multivariate analysis, previous surgery (adjusted odds ratio [AOR] 2.97; 95% confidence interval [CI] 1.5-6.0), history of MRSA (AOR 3.4; 95% CI 1.7-7.1), and exposure to clindamycin (AOR 8.5; 95% CI 2.3-32) and to macrolides (AOR 7.2, 95% CI 1.6-31.8) were independently associated with presence of clindamycin-resistant MRSA. Clinical resolution was similar between groups (77% versus 68%; P = 0.26). Clindamycin-resistant MRSA was less often USA-300 (82% versus 98%, P = 0.004). Clindamycin resistance did not affect MRSA-SSTI clinical outcomes.

  15. Clinical and Immunologic Characteristics of Patients With ANCA-Associated Glomerulonephritis Combined With Membranous Nephropathy

    Science.gov (United States)

    Zou, Rong; Liu, Gang; Cui, Zhao; Chen, Min; Zhao, Ming-Hui

    2015-01-01

    Abstract The concurrent antineutrophil cytoplasmic antibody-associated glomerulonephritis (ANCA-GN) and membranous nephropathy (MN) have been increasingly documented, mainly in case studies and case series; however, the differences of clinical and pathologic characteristics as well as outcomes between ANCA-GN patients with and without MN remain unclear. The current study investigated the clinical and immunologic features of patients with combined ANCA-GN and MN in a large cohort. Twenty-seven of 223 patients had combined ANCA-GN and MN; they had significantly higher levels of initial serum creatinine, higher Birmingham Vasculitis Activity Score and poorer renal outcome than ANCA-GN patients without MN (P PLA2R antibodies and glomerular PLA2R deposits was significantly lower in patients with combined ANCA-GN and MN than that in patients with idiopathic MN (P < 0.05). Compared with the idiopathic MN patients, the patients with combined ANCA-GN and MN had significantly higher recognition frequency of immunoglobulin (Ig) G2 and IgG3, and significantly lower recognition frequency of IgG4 (P < 0.05). Patients with combined ANCA-GN and MN had distinct clinical features and a different pathogenesis of MN. PMID:26376387

  16. PULMONARY PATHOPHYSIOLOGY AND LUNG MECHANICS IN ANESTHESIOLOGY: A CASE-BASED OVERVIEW

    OpenAIRE

    2012-01-01

    The induction and maintenance of anesthesia, surgical requirements, and patients’ unique pathophysiology all combine to create a setting in which our accumulated knowledge of respiratory physiology and lung mechanics take on immediate and central importance in patient management. In this review we will take a case-based approach to illustrate how the complex interactions between anesthesia, surgery, and patient disease impact patient care with respect to pulmonary pathophysiology and clinical...

  17. Demographic and Clinical Characteristics of People with Intellectual Disabilities with and without Substance Abuse Disorders in a Medicaid Population

    Science.gov (United States)

    Slayter, Elspeth Maclean

    2010-01-01

    Little is known about the demographic and clinical characteristics of people with intellectual disabilities and substance abuse problems. Drawing on health care billing claims for people with Medicaid coverage aged 12-99 years, the characteristics of people with intellectual disability and a history of substance abuse (N = 9,484) were explored and…

  18. Acute Heart Failure in the Elderly : Differences in Clinical Characteristics, Outcomes, and Prognostic Factors in the VERITAS Study

    NARCIS (Netherlands)

    Metra, Marco; Cotter, Gad; El-Khorazaty, Jill; Davison, Beth A.; Milo, Olga; Carubelli, Valentina; Bourge, Robert C.; Cleland, John G.; Jondeau, Guillaume; Krum, Henry; O'Connor, Christopher M.; Parker, John D.; Torre-Amione, Guillermo; van Veldhuisen, Dirk J.; Rainisio, Maurizio; Kobrin, Isaac; Mcmurray, John J.; Teerlink, John R.

    2015-01-01

    Background: Acute heart failure (HF) is common in the elderly, but the association of age with clinical outcomes and prognostic factors has not been examined thoroughly. Methods and Results: We analyzed the clinical and laboratory characteristics and the outcomes of 1,347 patients with acute HF enro

  19. Clinical characteristics of patients with myositis and autoantibodies to different fragments of the Mi-2 beta antigen.

    NARCIS (Netherlands)

    Hengstman, G.J.D.; Vree Egberts, W.T.M.; Seelig, H.P.; Lundberg, I.E.; Moutsopoulos, H.M.; Doria, A.; Mosca, M.; Vencovsky, J.; Venrooij, W.J.W. van; Engelen, B.G.M. van

    2006-01-01

    OBJECTIVES: To assess the clinical implications of autoantibodies directed against different parts of the Mi-2 beta autoantigen in patients with myositis. METHODS: A systematic assessment of the clinical, laboratory, and histological characteristics of 48 anti-Mi-2 positive patients from six Europea

  20. Observed inter-camera variability of clinically relevant performance characteristics for Siemens Symbia gamma cameras.

    Science.gov (United States)

    Kappadath, S Cheenu; Erwin, William D; Wendt, Richard E

    2006-11-28

    We conducted an evaluation of the intercamera (i.e., between cameras) variability in clinically relevant performance characteristics for Symbia gamma cameras (Siemens Medical Solutions, Malvern, PA) based on measurements made using nine separate systems. The significance of the observed intercamera variability was determined by comparing it to the intracamera (i.e., within a single camera) variability. Measurements of performance characteristics were based on the standards of the National Electrical Manufacturers Association and reports 6, 9, 22, and 52 from the American Association of Physicists in Medicine. All measurements were performed using 99mTc (except 57Co used for extrinsic resolution) and low-energy, high-resolution collimation. Of the nine cameras, four have crystals 3/8 in. thick and five have crystals 5/8 in. thick. We evaluated intrinsic energy resolution, intrinsic and extrinsic spatial resolution, intrinsic integral and differential flood uniformity over the useful field-of-view, count rate at 20% count loss, planar sensitivity, single-photon emission computed tomography (SPECT) resolution, and SPECT integral uniformity. The intracamera variability was estimated by repeated measurements of the performance characteristics on a single system. The significance of the observed intercamera variability was evaluated using the two-tailed F distribution. The planar sensitivity of the gamma cameras tested was found be variable at the 99.8% confidence level for both the 3/8-in. and 5/8-in. crystal systems. The integral uniformity and energy resolution were found to be variable only for the 5/8-in. crystal systems at the 98% and 90% confidence level, respectively. All other performance characteristics tested exhibited no significant variability between camera systems. The measured variability reported here could perhaps be used to define nominal performance values of Symbia gamma cameras for planar and SPECT imaging.

  1. MR imaging characteristics and clinical symptoms related to displaced meniscal flap tears

    Energy Technology Data Exchange (ETDEWEB)

    Lance, Valentin; Heilmeier, Ursula R.; Joseph, Gabby B.; Steinbach, Lynne; Link, Thomas M. [University of California, Department of Radiology and Biomedical Imaging, San Francisco, CA (United States); Ma, Benjamin [University of California, Department of Orthopedic Surgery and Sports Medicine, San Francisco (United States)

    2014-11-16

    The purpose of our study was (1) to analyze the flap tear location, direction of displacement and size on magnetic resonance (MR) imaging, (2) to describe associated knee abnormalities including presence of effusion, synovitis, bone marrow edema pattern or ligamentous tear, and (3) to assess clinical findings found with flap tears, including the pain score, and determine differences between operative and nonoperative groups. A retrospective radiology database search over the last 3 years identified 238 patients with flap tears, of which ultimately 58 with isolated flap tears were included after exclusion of patients with other significant knee internal derangement, severe degenerative change or prior surgery. MR studies of the knee were analyzed by two radiologists. Imaging characteristics were correlated with associated knee abnormalities and clinical findings. Statistical analysis employed linear and logistic regression models. Inter- and intrareader reliability was calculated. The medial meniscus was the most common site of flap tears (52/60, 87 %), with inferior displacement (47/60, 78 %). The degree of tibial cartilage loss had a positive correlation with the visual analog pain scale (p = 0.03). Patients who underwent arthroscopy were younger than those who did not (p = 0.01) and more likely to have a positive clinical McMurray test (p = 0.01). Medially and inferiorly displaced flap tears are the most common tear pattern. Those undergoing arthroscopy are more likely to have positive meniscal signs on clinical examination. A greater degree of cartilage loss involving the tibia on MR imaging was associated with increasing visual analog pain scores. (orig.)

  2. Clinical and laboratory characteristics of adolescents with platelet function disorders and heavy menstrual bleeding

    Directory of Open Access Journals (Sweden)

    Amesse Lawrence S

    2013-01-01

    Full Text Available Abstract Background Platelet function disorders (PFDs have emerged as an important etiology of heavy menstrual bleeding (HMB in adolescents. However, neither clinical nor laboratory data have been methodically analyzed in this population subset. The objective of this study was to evaluate these parameters in order to distinguish characteristics of the disorder that in turn will lead to earlier diagnosis and therapy initiation. Methods Retrospective review of medical records from postmenarcheal adolescents with documented PFDs referred to a hemophilia treatment center and university faculty practices for bleeding diatheses with their clinical and laboratory data evaluated. Results Of 63 teens with documented PFDs, HMB was the most common clinical manifestation of PFD (43; 68.3%. Of these, 37 (86% were diagnosed with PFD either at or after menarche with the diagnosis based on HMB symptoms alone. Only 6 (14% were diagnosed with a PFD prior to menarche, based on associated bleeding, i.e., epistaxis, ecchymosis, and all developed HMB after menstruation onset. Interestingly, 20 girls were diagnosed with a PFD prior to menarche and of these, only 6 (30% went on to develop HMB after pubertal transition, while the majority (14; 70% did not. The average age-at-PFD diagnosis was 14.5yrs, significantly differing from the 10.9yrs average age-at-PFD diagnosis in their counterparts that, after menarche, did not develop HMB (PP P Conclusions Adolescents with PFDs and HMB appear to be clinically distinct from their non-HMB counterparts. This group of girls is characterized by HMB the major bleeding symptom, significantly high incidences of blood group O and the δ-SPD with a PFD diagnosed well after menarche. High false negative standard platelet function study results indicate additional diagnostic strategies, particularly for δ-SPD, should be considered.

  3. Differential clinical characteristics and prognosis of intraventricular conduction defects in patients with chronic heart failure

    Science.gov (United States)

    Cinca, Juan; Mendez, Ana; Puig, Teresa; Ferrero, Andreu; Roig, Eulalia; Vazquez, Rafael; Gonzalez-Juanatey, Jose R.; Alonso-Pulpon, Luis; Delgado, Juan; Brugada, Josep; Pascual-Figal, Domingo; Brugada, J.; Batlle, M.; Berruezo, A.; Hevia, S.; Mont, L.; Pérez-Villa, F.; Cinca, J.; Roig, E.; Bayés de Luna, A.; Borrás, X.; Carreras, F.; Ferrero, A.; Guerra, J.M.; Hove-Madsen, L.; Jorge, E.; Martínez, R.; Padró, J.; Puig, T.; Ribas, N.; Viñolas, X.; Alvarez-Garcia, J.; González-Juanatey, J.R.; Bandín, M.; Eiras, S.; Fernández-Hernández, L.; García-Acuña, J.; Gómez-Otero, I.; Grigorian-Shamagian, L.; Lago, F.; Manzón, P.; Moure, M.; Otero-Raviña, F.; Otero-Santiago, F.; Rodino Janeiro, B.K.; Rubio, J.; Salgado, A.; Seoane, A.; Varela, A.; Lear, P.V.; Fernández-Cruz, A.; Alvarez de Arcaya Vicente, A.; Avila, M.; Bordiu, E.; Calle, L.; Fernández-Pinilla, C.; Gómez-Garre, D.; González-Rubio, L.; Marco, J.; Martell, N.; Muñoz-Pacheco, P.; Ortega, A.; Patiño, R.; Pedrajas, J.; Reinares, L.; Pérez-Villacastín, J.; Bover, R.; Cobos, M.; García-Quintanilla, J.; Moreno, J.; Pérez-Castellano, N.; Pérez-Serrano, M.; Vila, I.; Delgado, J.F.; Arribas, F.; Escribano, P.; Flox, A.; Jiménez López-Guarch, C.; Paradina, M.; Ruiz-Cano, J.; Sáenz de la Calzada, C.; Salguero, R.; Sánchez-Sánchez, V.; Tello de Meneses, R.; Vicente-Hernández, M.; Alonso-Pulpón, L.; Fernández -Lozano, I.; García-Pavía, P.; García-Touchard, A.; Gómez-Bueno, M.; Márquez, J.; Segovia, J.; Silva, L.; Vázquez-Mosquera, M.; Valdés, M.; García-Alberola, A.; Garrido, I.; Pascual-Figal, D. A.; Pastor-Pérez, F.J.; Sánchez-Más, J.; Tornel, P.; Rivera, M.; Almenar, L.; Cortés, R.; Martínez-Dolz, L.; Montero, J.; Portolés, M.; Roselló-Lleti, E.; Salvador, A.; Vila, V.; Vázquez, R.; Cubero, J.; Fernández-Palacín, A.; García-Medina, D.; García-Rey, S.; Laguna, E.; Leal del Ojo, J.; Miñano, F.; Pastor-Torres, L.; Pavón, R.; Pérez-Navarro, A.; Villagómez, D.; Vázquez, R.; Arana, R.; Bartolomé, D.; Cabeza, P.; Calle-Pérez, G.; Camacho, F.; Cano, L.; Carrillo, A.; Díaz-Retamino, E.; Escolar, V.; Fernández-Rivero, R.; Gamaza, S.; Giráldes, A.; Hernández-Vicente, N.; Lagares, M.; López-Benítez, J.; Marante, M.; Otero, E.; Pedregal, J.; Sancho-Jaldón, M.; Sevillano, R.; Zayas, R.; Verdú, J.M.; Aguilar, S.; Aizpurúa, M.; Alguacil, F.; Casacuberta, J.; Cerain, J.; Domingo, M.; García-Lareo, M.; Herrero-Melechón, J.; López-Pareja, N.; Mena, A.; Pérez-Orcero, A.; Rodríguez- Cristóbal, J.; Rozas, M.; Sorribes, J.; Torán, P.; Worner, F.; Barta, L.; Bravo, C.; Cabau, J.; Casanova, J.; Daga, B.; De la Puerta, I.; Hernández-Martín, I.; Piñol, E.; Pueo, E.; Torres, G.; Troncoso, A.; Viles, D.; Bardají, A.; Mercè, J.; Sanz-Girgas, E.; Valdovinos, P.; Aramburu, O.; Arias, J.; García-González, C.; Alonso, M.; Bischofberger, C.; Domínguez-De Pablos, G.; Jiménez-Cervantes, D.; Ureña, I.; Grau-Sepúlveda, A.; Fiol, C.; Pericas, P.; Villalonga, M.; Orosa, P.; Agüero, J.; Planas-Aymá, F.; Grau-Amoros, J.; Planas-Comes, F.; San Vicente, L.

    2013-01-01

    Aims Intraventricular conduction defects (IVCDs) can impair prognosis of heart failure (HF), but their specific impact is not well established. This study aimed to analyse the clinical profile and outcomes of HF patients with LBBB, right bundle branch block (RBBB), left anterior fascicular block (LAFB), and no IVCDs. Methods and results Clinical variables and outcomes after a median follow-up of 21 months were analysed in 1762 patients with chronic HF and LBBB (n = 532), RBBB (n = 134), LAFB (n = 154), and no IVCDs (n = 942). LBBB was associated with more marked LV dilation, depressed LVEF, and mitral valve regurgitation. Patients with RBBB presented overt signs of congestive HF and depressed right ventricular motion. The LAFB group presented intermediate clinical characteristics, and patients with no IVCDs were more often women with less enlarged left ventricles and less depressed LVEF. Death occurred in 332 patients (interannual mortality = 10.8%): cardiovascular in 257, extravascular in 61, and of unknown origin in 14 patients. Cardiac death occurred in 230 (pump failure in 171 and sudden death in 59). An adjusted Cox model showed higher risk of cardiac death and pump failure death in the LBBB and RBBB than in the LAFB and the no IVCD groups. Conclusion LBBB and RBBB are associated with different clinical profiles and both are independent predictors of increased risk of cardiac death in patients with HF. A more favourable prognosis was observed in patients with LAFB and in those free of IVCDs. Further research in HF patients with RBBB is warranted. PMID:23512097

  4. Clinical characteristics, diagnostic evaluation, and antibiotic prescribing patterns for skin infections in nursing homes

    Directory of Open Access Journals (Sweden)

    Norihiro Yogo

    2016-07-01

    Full Text Available Background: The epidemiology and management of skin infections in nursing homes has not been adequately described. We reviewed the characteristics, diagnosis and treatment of skin infections among residents of nursing homes to identify opportunities to improve antibiotic use. Methods: A retrospective study involving 12 nursing homes in the Denver metropolitan area. For residents at participating nursing homes diagnosed with a skin infection between July 1, 2013 and June 30, 2014, clinical and demographic information was collected through manual chart review.Results: Of 100 cases included in the study, the most common infections were non-purulent cellulitis (n=55, wound infection (n=27, infected ulcer (n=8, and cutaneous abscess (n=7. In 26 cases, previously published minimum clinical criteria for initiating antibiotics (Loeb criteria were not met. Most antibiotics (n=52 were initiated as a telephone order following a call from a nurse, and 41 patients were not evaluated by a provider within 48 hours after initiation of antibiotics. Nearly all patients (n=95 were treated with oral antibiotics alone. The median treatment duration was 7 days (interquartile range [IQR] 7-10; 43 patients received treatment courses of ≥ 10 days.Conclusions: Most newly diagnosed skin infections in nursing homes were non-purulent infections treated with oral antibiotics. Antibiotics were initiated by telephone in over half of cases, and lack of a clinical evaluation within 48 hours after starting antibiotics was common. Improved diagnosis through more timely clinical evaluations and decreasing length of therapy are important opportunities for antibiotic stewardship in nursing homes.

  5. Clinical Characteristics of Down Syndrome Children With Congenital Heart Disease in a Developing Country

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    Mottaghi Moghaddam

    2015-11-01

    Full Text Available Background Down syndrome (DS is the most common chromosomal abnormality in newborns and is associated with other congenital malformations and health problems. The features of Down syndrome differ according to ethnicity and geographic region. Objectives The main aim was to assess the clinical characteristics of DS patients in a referral pediatric cardiology department. Patients and Methods In this cross-sectional study, we assessed the clinical characteristics of children with Down syndrome and heart defects in an educational hospital over 11 years (from September 2001 to September 2012 in Iran. All data were collected according to a checklist created by the researchers, which included the clinical information, genetic characteristics, cardiac and non-cardiac co-existing diseases, and parental variables of the children. An independent t-test and a chi-square test were used to compare qualitative variables such as birth weight and age of diagnosis. P < 0.05 was considered statistically significant. Results 100 patients with Down syndrome and congenital heart disease were evaluated; 52 were female (52% and 48 were male (48%. The average birth weight of the subjects was 2745 ± 523 (mean ± SD grams. The mean age of the patients’ mothers was 32 ± 6 years, and the mean age of the patients’ fathers was 36 ± 6 years. Chromosomal analysis was performed for 61 patients, 60 of whom had free trisomy (98.4%, one of whom had translocation (1.6%, and none of whom had a mosaic pattern of chromosomal abnormality. The parents of 33 the patients in this study were consanguineous. All patients had cardiac disorders, but non-cardiac disorder also was recorded in 37 patients (37%. The most common non-cardiac disorder in patients was hypothyroidism, and the second most common was gastrointestinal problems. Conclusions Parents were blood relatives in 33 (33% of the patient cases, which is a very high rate. Therefore, non-random mating is an important issue in

  6. Clinical Characteristics of Bowel Obstruction in Southern Iran; Results of a Single Center Experience

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    Majid Akrami

    2015-01-01

    Full Text Available Objective: To determine the epidemiological, clinical, laboratory characteristics as well as outcome of 411 patients with bowel obstruction in Southern Iran. Methods: This was a cross-sectional study being performed in Shahid Faghihi hospital of Shiraz between 2006 and 2012. We reviewed the medical charts of the 411 patients with initial diagnosis of bowel obstruction who were admitted to our center during the study period. The patients’ demographic, clinical and laboratory findings as well as their management and outcome was recorded in data gathering forms. The data were then analyzed according to the outcome and clinical characteristics. Results: Among the 411 patients with initial diagnosis of bowel obstruction, 253 (61.5% were men and 158 (38.5% were women. The mean age of the patients was 48.2±19.7 years. Besides, 73.6% were observed and 26.4% were operated. Those who were operated had those who underwent operation had significantly lower frequency of obstipation (28.1% vs. 71.9%; p=0.045 and abdominal distention (32.3% vs. 67.7%; p=0.007. Intraoperative findings included adhesion band formation in 50 (48.1%, mass 18 (17.3%, and hernia 7 (6.7%. We found that the frequency of malignancy was significantly higher in those who were managed conservatively compared to those undergoing operation (64.3% vs. 35.7%; p=0.042. The mean hospital stay was significantly higher in those who underwent operation (8.1±7.5 vs. 2.6±2.2 days; p=0.035. Conclusion: The results of this study demonstrates although some signs and symptoms, such as abdominal pain, vomiting, abdominal tenderness, abdominal distention, and obstipation, were more common among the patients with bowel obstruction, they were not sensitive and specific enough for definite diagnosis. Due to the lack of positive predictive value of clinical signs and symptoms in diagnosis of bowel obstruction, a reasonable and logical modality is needed for bowel obstruction diagnosis with better

  7. Neuroelectrophysiological indexes and clinical characteristics of patients with peroneal muscular atrophy: Retrospective analysis of 24 cases

    Institute of Scientific and Technical Information of China (English)

    Changchun Su; Qinbao Qin

    2006-01-01

    BACKGROUND: Peroneal muscular atrophy (PMA) is characterized by insidious onset, gradually progressive course of disease, very mild disability degree and easily subjecting to missed diagnosis and misdiagnosis.Nerve conductive velocity is helpful in the diagnosis of atypical cases.OBJECTIVE: To retrospectively analyze the characteristics of clinical manifestation, electromyogram (EMG),motor and sensory nerve conduction velocity of patients with PMA.DESIGN: Retrospective case analysis.SETTING: Department of Neurology, Guangzhou First People's Hospital.PARTICIPANTS: Twenty-four patients with PMA, including 16 males and 8 females, aged 5-68 years old,admitted to Guangzhou First People's Hospital between March 1996 and January 2006 were recruited.Informed consents were obtained from all the patients.METHODS: All the patients subjected to EMG and detection of nerve conduction velocity at distal end of four extremities with a Keypoint evoked potential/ EMG instrument (Denmark). Sensory and motor conduction velocity, EMG changes of upper and lower extremities were observed, and relationship of neuroelectrophysiological characteristics and clinical symptoms was analyzed.MAIN OUTCOME MEASURES: Changes in sensory and motor conduction velocity, EMG and clinical manifestations of 24 patients.RESULTS: ① All the patients suffered from insidious onset and gradually progressive course of PMA.Muscular atrophy of lower extremity was found in 14 patients, and that of upper extremity in 5 patients. ② Routine nerve conduction study showed that sensory and motor conduction velocity were stepped down,especially in 16 patients with type Ⅰ PMA (demyelinating pattern, nerve conduction velocity below normal level 50%). Motor nerve conduction velocity of median nerve, ulnar nerve, common peroneal nerve and tibial nerve averaged 34.8 m/s, 37.2 m/s, 16.5 m/s and 17.4 m/s, respectively; Sensory nerve conduction velocity of median nerve, ulnar nerve and sural nerve averaged 27.9%, 24.6 m

  8. Characteristics of adult smokers presenting to a mind-body medicine clinic.

    Science.gov (United States)

    Luberto, Christina M; Chad-Friedman, Emma; Dossett, Michelle L; Perez, Giselle K; Park, Elyse R

    2016-09-29

    Mind-body interventions can improve vulnerabilities that underlie smoking behavior. The characteristics of smokers who use mind-body medicine have not been explored, preventing the development of targeted interventions. Patients (N = 593) presenting to a mind-body medicine clinic completed self-report measures. Patients were 67 percent never smokers, 27 percent former smokers, and 6 percent current smokers. Current smokers were younger; more likely to be single, unemployed, or on disability; and report greater depression symptoms, greater pain, and lower social support (ps mind-body medicine have unique psychosocial needs that should be targeted in mind-body smoking cessation interventions.

  9. Heterogeneity of breast cancer associations with five susceptibility loci by clinical and pathological characteristics

    DEFF Research Database (Denmark)

    Garcia-Closas, M.; Hall, P.; Nevanlinna, H.

    2008-01-01

    A three-stage genome-wide association study recently identified single nucleotide polymorphisms ( SNPs) in five loci ( fibroblast growth receptor 2 ( FGFR2), trinucleotide repeat containing 9 ( TNRC9), mitogen-activated protein kinase 3 K1 (MAP3K1), 8q24, and lymphocyte- specific protein 1 ( LSP1......)) associated with breast cancer risk. We investigated whether the associations between these SNPs and breast cancer risk varied by clinically important tumor characteristics in up to 23,039 invasive breast cancer cases and 26,273 controls from 20 studies. We also evaluated their influence on overall survival...... related to PR-positive, low grade and node positive tumors (P = 10(-5), 10(-8), 0.013, respectively). The association for rs13281615 in 8q24 was stronger for ER- positive, PR-positive, and low grade tumors (P = 0.001, 0.011 and 10(-4), respectively). The differences in the associations between SNPs...

  10. Hospital costs and clinical characteristics of continuous renal replacement therapy patients: a continuous ethical dilemma.

    Science.gov (United States)

    Coustasse, Alberto

    2008-01-01

    This study describes the clinical characteristics and examines hospital costs involved in the care of 117 patients undergoing Continuous Renal Replacement Therapy (CRRT) between January 1999 and August 2002. The majority (70.9%) of the patients undergoing CRRT expired in the hospital. Statistically significant differences were found with respect to the length of stay for discharge status and gender; and with respect to costs for surgery versus no surgery and gender. Significant differences were also found between discharge status and gender, age, and cardiovascular surgery. The results of this study raise economic and ethical questions related to the cost/benefit of CRRT and the futility of the treatment. Hospitals should ensure that they have utilization protocols in place for CRRT, promote cooperation between intensive care unit (ICU) physicians and nephrologists, and create multi-disciplinary CRRT teams in an effort to maximize the effectiveness of therapy and minimize costs.

  11. Clinical characteristics and treatment response to SSRI in a female pedophile.

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    Chow, Eva W C; Choy, Alberto L

    2002-04-01

    Although much investigation has been done with male sex offenders, there have been few studies on female sex offenders. Female sex offenders have been reported as having a high incidence of psychiatric disorders, but female paraphilics were rarely described. The literature on the treatment of female sex offenders is also limited and treatment with a selective serotonin reuptake inhibitor (SSRI) has not been reported. This paper presents the case of a woman with DSM-IV pedophilia. Her clinical characteristics, her offense history, and her positive response to treatment with sertraline (a SSRI) are described. This case adds to the limited literature on female pedophiles and suggests that SSRIs may be an effective treatment for paraphilic disorders in female sex offenders.

  12. Variations and mutations in the hepatitis B virus genomeand their associations with clinical characteristics

    Institute of Scientific and Technical Information of China (English)

    Yoshihiko Yano; Takeshi Azuma; Yoshitake Hayashi

    2015-01-01

    Hepatitis B virus (HBV) infection is major global issue,because chronic HBV infection is strongly associatedwith liver cancer. HBV spread worldwide with variousmutations and variations. This variability, called quasispecies,is derived from no proof-reading capacity ofviral reverse transcriptase. So far, thousands of studiesreported that the variety of genome is closely related tothe geographic distribution and clinical characteristics.Recent technological advances including capillarysequencer and next generation sequencer have made ineasier to analyze mutations. The variety of HBV genomeis related to not only antigenicity of HBs-antigen but alsoresistance to antiviral therapies. Understanding of thesevariations is important for the development of diagnostictools and the appropriate therapy for chronic hepatitisB. In this review, recent publications in relation to HBVmutations and variations are updated and summarized.

  13. Clinical characteristics and outcome of patients diagnosed with psychogenic nonepileptic seizures: a 5-year review.

    LENUS (Irish Health Repository)

    O'Sullivan, S S

    2012-02-03

    OBJECTIVE: The goal of this article was to describe the clinical characteristics and outcomes of patients diagnosed with psychogenic nonepileptic seizures (PNES). METHODS: We conducted a retrospective review of patients diagnosed with PNES in a 5-year period. RESULTS: Fifty patients with PNES were identified, giving an estimated incidence of 0.91\\/100,000 per annum. Thirty-eight were included for review, 15 of whom were male (39%). Eighteen patients had been diagnosed with epilepsy as well as PNES (47%). We demonstrated a gender difference in our patients, with males having higher seizure frequencies, more antiepileptic drug use, and a longer interval before diagnosis of PNES. Females were diagnosed with other conversion disorders more often than males. Impaired social function was observed in PNES, as was resistance to psychological interventions with a subsequent poor response to treatments. CONCLUSIONS: PNES remains a difficult condition to treat, and may affect males in proportions higher than those described in previous studies.

  14. Baroreflex Sensitivity in Relation to Clinical Characteristics in Subject Aged 40 to 80 Years

    DEFF Research Database (Denmark)

    Brinth, Louise Schouborg; Pors, Kirsten; Latif, Tabassam;

    2014-01-01

    in our population comprising one component reflecting the sympathetic activity, the parasympathetic system, and the integrated spontaneous BRS, respectively. The parasympathetic component was the only one correlating with clinical characteristics of declining age, smoking habits, systolic blood pressure...... on the Framingham score. BRS was found to decline with age, but this change disappeared when correcting for the age related increase in systolic blood pressure. We found that the VM-derived indices of sympathetic vascular control declined with age as did the vagally controlled heart rate changes in response to deep...... breathing and VM. We could not demonstrate any correlation between BRS, smoking status, and body size when adjusting for age and gender, whereas spontaneous BRS was reduced with increasing Framingham risk score. Principal component analysis revealed three component explaining 69% of the total variance...

  15. Epidemiological and Clinical Characteristics of Fall Injuries in East Azerbaijan, Iran; A Cross-Sectional Study

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    Saber Ghaffari-Fam

    2015-07-01

    Full Text Available Objective: To describe the epidemiological and clinical characteristics of fall injuries in East Azerbaijan, Iran. Methods: This cross-sectional study was based on Hospital Information System (HIS data for patients referred to the Imam Reza Hospital between 2008 and 2013. We recorded the demographic characteristics and epidemiological patterns of patients who were admitted to our center due to fall injuries. To standardize the reports the International Classification of Diseases (ICD, the International Classification of Diseases 9 Clinical Modification (ICD-9-CM was used. Equally, the hospitalization period and number, admission ward, and the final status of victims after discharge from the hospital were extracted from the HIS. Results: Overall we included a total number of 3397 patients with mean age of 39.2±22.7 years. There were 2501 (73.6% men among the patients. Long bone fracture (48.1% and intracranial injury (24.2% were the most frequent injuries among fall injury victims. Operations on spinal cord and spinal canal structures (12.0%, Operations on nose (11.6% were the most common operations being performed in these patients. The survival was significantly lower in patients with age more than 60 years when compared to other age groups ( p=0.001. The survival rate was significantly lower in age group of >60 years, compared to other age groups ( p=0.001. Conclusion: Given the high rate of fall injuries and death among the elderly that increases with age, appropriate measures must be taken to control and prevent injuries while prioritizing the elderly.

  16. Clinical Characteristics of Pediatric Esophagitis in Southern Iran; A Single-Center Experience

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    Mozhgan Zahmatkeshan

    2013-06-01

    Full Text Available Background: We sought to determine the clinical characteristics of pediatric esophagitis in southern Iran. Methods: This cross-sectional study was conducted over a 4-year period, from 2005 to 2009, in Nemazee Hospital, a tertiary healthcare center in Shiraz, southern Iran. We consecutively included all pediatric patients (<18 years who underwent endoscopy in our center and had pathology-confirmed diagnosis of esophagitis. Data regarding the patients’ demographic characteristics, comorbidities, and clinical findings were recorded using a questionnaire. All the patients underwent upper gastrointestinal endoscopy and biopsy of the esophagus, and the findings were recorded in the questionnaire. Results: We studied 125 children, comprising 61 (48.8% girls and 64 (51.2% boys at a mean age of 6.6±5.5 years. Repeated vomiting was the prominent symptom in our series, with it being reported by 75 (60% patients, followed by fever in 35 (28%. Erythema (33.6%, esophageal ulcer (11.2%, and whitish patch (8.0% were the most common endoscopic findings, while reflux esophagitis (32.8%, chronic (6.4% and acute esophagitis (5.6%, and candida esophagitis (5.6% were the most common histological diagnoses. Only one (0.8% patient was diagnosed as having eosinophilic esophagitis, aspergillosis, and graft-versus-host disease. Conclusion: Reflux was the most common cause of esophagitis in the pediatric population of southern Iran. Contrary to previous reports, the prevalence of eosinophilic esophagitis was far less than that estimated, while the prevalence of opportunistic infections was higher secondary to post-liver transplantation immunosuppression.

  17. Clinical characteristics, cytogenetic and molecular findings in patients with disorders of sex development.

    Science.gov (United States)

    Tian, Li; Chen, Ming; Peng, Jian-hong; Zhang, Jian-wu; Li, Li

    2014-02-01

    The clinical characteristics of patients with disorders of sex development (DSD), and the diagnostic values of classic cytogenetic and molecular genetic assays for DSD were investigated. In the enrolled 56 cases, there were 9 cases of 46,XY DSD, 6 cases of Turner syndrome (TS), one case of Super female syndrome, 25 cases of Klinefelter syndrome, 14 cases of 46,XX DSD, and one case of autosomal balanced rearrangements with hypospadias. The diagnosis of sex was made through physical examination, cytogenetic assay, ultrasonography, gonadal biopsy and hormonal analysis. PCR was used to detect SRY, ZFX, ZFY, DYZ3 and DYZ1 loci on Y and X chromosomes respectively. The DSD patients with the same category had similar clinical characteristics. The karyotypes in peripheral blood lymphocytes of all patients were identified. PCR-based analysis showed presence or absence of the X/Y-linked loci in several cases. Of the 9 cases of 46,XY DSD, 6 were positive for SRY, 9 for ZFX/ZFY, 9 for DYZ3 and 8 for DYZ1 loci. Of the 6 cases of TS, only 1 case with the karyotype of 45,X,/46,XX/46,XY was positive for all 5 loci. Of the 25 cases of Klinefelter syndrome, all were positive for all 5 loci. In one case of rare Klinefelter syndrome variants azoospermia factor (AZF) gene detection revealed the loss of the AZFa+AZFb region. In 14 cases of 46,XX DSD, 7 cases were positive for SRY, 14 for ZFX, 7 for ZFY, 7 for ZYZ3, and 5 for DYZ1. PCR can complement and also confirm cytogenetic studies in the diagnosis of sex in cases of DSD.

  18. Characteristics of Pseudoaneurysms in Southern India; Risk Analysis, Clinical Profile, Surgical Management and Outcome

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    Hafeezulla Lone

    2015-04-01

    Full Text Available Objective: To determine the risk factors, clinical characteristics, surgical management and outcome of pseudoaneurysm secondary to iatrogenic or traumatic vascular injury. Methods: This was a cross-sectional study being performed in department of cardiovascular and thoracic surgery skims soura during a 4-year period. We included all the patients referring to our center with primary diagnosis of pseudoaneurysm. The pseudoaneurysm was diagnosed with angiography and color Doppler sonography. The clinical and demographic characteristics were recorded and the risk factors were identified accordingly. Patients with small swelling (less than 5-cm and without any complication were managed conservatively. They were followed for progression and development of complications in relation to swelling. Others underwent surgical repair and excision. The outcome of the patients was also recorded. Results: Overall we included 20 patients with pseudoaneurysm. The mean age of the patients was 42.1±0.6 years. Among them there were 11 (55% men and 9 (45% women. Nine (45% patients with end stage renal disease developed pseudoaneurysm after inadvertent femoral artery puncture for hemodialysis; two patients after interventional cardiology procedure; one after femoral embolectomy; one developed after fire arm splinter injury and one formed femoral artery related pseudoaneurysm after drainage of right inguinal abscess. The most common site of pseudoaneurysm was femoral artery followed by brachial artery. Overall surgical intervention was performed in 17 (85% patients and 3 (15% were managed conservatively. Conclusion: End stage renal disease is a major risk factor for pseudoaneurysm formation. Coagulopathy, either therapeutic or pathological is also an important risk factor. Patients with these risk factors need cannulation of venous structures for hemodialysis under ultrasound guide to prevent inadvertent arterial injury. Patients with end stage renal disease who

  19. A case series to describe the clinical characteristics of foot ulceration in patients with rheumatoid arthritis.

    Science.gov (United States)

    Siddle, Heidi J; Firth, Jill; Waxman, Robin; Nelson, E Andrea; Helliwell, Philip S

    2012-03-01

    The aim of this study was to describe the clinical characteristics of foot ulceration in patients with rheumatoid arthritis (RA). Adults with RA and current foot ulceration but without diabetes were recruited. Clinical examination included assessment of RA disease activity, foot deformity, peripheral vascular disease, neuropathy and plantar pressures. Location, wound characteristics and time to healing were recorded for each ulcer. Participants completed the Health Assessment Questionnaire and Leeds Foot Impact Scale. Thirty-two cases with 52 current ulcers were recruited. Thirteen patients (41%) experienced more than one current ulcer: 5 (16%) had bilateral ulceration, 15 (47%) had previous ulceration at a current ulcer site. The majority (n = 33) of open ulcers were located over the dorsal aspect of the interphalangeal joints (n = 12), plantar aspect of the metatarsophalangeal joints (MTPJs) (n = 12) and medial aspect of first MTPJs (n = 9). In ulcerated limbs (n = 37), ankle brachial pressure index (ABPI) was plantar pressures were >6 kg/cm(2) in 6 (16%). Mean ulcer size was 4.84 by 3.29 mm. Most ulcers (n = 42, 81%) were superficial; five (9.6%) were infected. Time to healing was available for 41 ulcers: mean duration was 28 weeks. Three ulcers remained open. In conclusion, foot ulceration in RA is recurrent and multiple ulcers are common. Whilst ulcers are small and shallow, time to achieve healing is slow, posing infection risk. Reduced protective sensation is common in affected patients. The prevalence of arterial disease is low but may be under estimated due to high intolerance of ABPI.

  20. Chronic fatigue syndrome after Giardia enteritis: clinical characteristics, disability and long-term sickness absence

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    Naess Halvor

    2012-02-01

    Full Text Available Abstract Background A waterborne outbreak of Giardia lamblia gastroenteritis led to a high prevalance of long-lasting fatigue and abdominal symptoms. The aim was to describe the clinical characteristics, disability and employmentloss in a case series of patients with Chronic Fatigue Syndrome (CFS after the infection. Methods Patients who reported persistent fatigue, lowered functional capacity and sickness leave or delayed education after a large community outbreak of giardiasis enteritis in the city of Bergen, Norway were evaluated with the established Centers for Disease Control and Prevention criteria for CFS. Fatigue was self-rated by the Fatigue Severity Scale (FSS. Physical and mental health status and functional impairment was measured by the Medical Outcome Severity Scale-short Form-36 (SF-36. The Hospital Anxiety and Depression Scale (HADS was used to measure co-morbid anxiety and depression. Inability to work or study because of fatigue was determined by sickness absence certified by a doctor. Results A total of 58 (60% out of 96 patients with long-lasting post-infectious fatigue after laboratory confirmed giardiasis were diagnosed with CFS. In all, 1262 patients had laboratory confirmed giardiasis. At the time of referral (mean illness duration 2.7 years 16% reported improvement, 28% reported no change, and 57% reported progressive course with gradual worsening. Mean FSS score was 6.6. A distinctive pattern of impairment was documented with the SF-36. The physical functioning, vitality (energy/fatigue and social functioning were especially reduced. Long-term sickness absence from studies and work was noted in all patients. Conclusion After giardiasis enteritis at least 5% developed clinical characteristics and functional impairment comparable to previously described post-infectious fatigue syndrome.

  1. Deep brain stimulation for severe autism: from pathophysiology to procedure.

    Science.gov (United States)

    Sinha, Saurabh; McGovern, Robert A; Sheth, Sameer A

    2015-06-01

    Autism is a heterogeneous neurodevelopmental disorder characterized by early-onset impairment in social interaction and communication and by repetitive, restricted behaviors and interests. Because the degree of impairment may vary, a spectrum of clinical manifestations exists. Severe autism is characterized by complete lack of language development and potentially life-threatening self-injurious behavior, the latter of which may be refractory to medical therapy and devastating for affected individuals and their caretakers. New treatment strategies are therefore needed. Here, the authors propose deep brain stimulation (DBS) of the basolateral nucleus of the amygdala (BLA) as a therapeutic intervention to treat severe autism. The authors review recent developments in the understanding of the pathophysiology of autism. Specifically, they describe the genetic and environmental alterations that affect neurodevelopment. The authors also highlight the resultant microstructural, macrostructural, and functional abnormalities that emerge during brain development, which create a pattern of dysfunctional neural networks involved in socioemotional processing. They then discuss how these findings implicate the BLA as a key node in the pathophysiology of autism and review a reported case of BLA DBS for treatment of severe autism. Much progress has been made in recent years in understanding the pathophysiology of autism. The BLA represents a logical neurosurgical target for treating severe autism. Further study is needed that considers mechanistic and operative challenges.

  2. Clinical Characteristics of Pediatric-Onset and Adult-Onset Multiple Sclerosis in Hispanic Americans.

    Science.gov (United States)

    Langille, Megan M; Islam, Talat; Burnett, Margaret; Amezcua, Lilyana

    2016-07-01

    Multiple sclerosis can affect pediatric patients. Our aim was to compare characteristics between pediatric-onset multiple sclerosis and adult-onset multiple sclerosis in Hispanic Americans. This was a cross-sectional analysis of 363 Hispanic American multiple scleroses cases; demographic and clinical characteristics were analyzed. A total of 110 Hispanic patients presented with multiple sclerosis before age 18 and 253 as adult multiple sclerosis. The most common presenting symptoms for both was optic neuritis. Polyfocal symptoms, seizures, and cognitive symptoms at presentation were more prevalent in pediatric-onset multiple sclerosis (P ≤ .001). Transverse myelitis was more frequent in adult-onset multiple sclerosis (P ≤ .001). Using multivariable analysis, pediatric-onset multiple sclerosis (adjusted odds ratio, 0.3OR 95% confidence interval 0.16-0.71, P = .004) and being US born (adjusted odds ratio, 0.553, 95% confidence interval 0.3-1.03, P = .006) were less likely to have severe ambulatory disability. Results suggest that pediatric-onset multiple sclerosis and adult-onset multiple sclerosis in Hispanics have differences that could be important for treatment and prognosis.

  3. Early Period Results and Clinical Characteristics of Upper Gastrointestinal Endoscopy in Sivrihisar State Hospital

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    Ozgur Turk

    2014-12-01

    Full Text Available Aim: Our aim was to identify the characteristics of the patient that performed upper gastrointestinal endoscopy in a new established endoscopy unit of a state hospital. We want to present the spectrum of gastrointestinal diseases in our hospitals region. Material and Method: We analyzed patients upper endoscopy results according to age, sex, complaints, clinical characteristics, type of anesthesia, and the necessity of biopsy. We reviewed 256 patients data between 2013 December-2014 July. All endoscopies were performed by same surgeon. Results: The highest complaint was epigastric pain (n=112, 43, 8%. Other complaints were followed as dyspepsia (n=84, 32.8%, heartburn (n=42, 16.4%, nausea (n=4, 1.6%, vomiting (n=2, 0.8%, dysphagia (n=6, 2.3%. We determined 218 gastritis (85.2%, 64 hiatal hernia (25%, 120 esophagitis (46.9%, 76 duodenitis (29.7%, 4 gastric ulcer (1.6%, 18 duodenal ulcers (7%, 20 bile reflux (7.8%, 26 Gastro esophageal reflux disease (GERD in patients (10.2%. 10 patients reported as normal (3.9%. Biopsy was performed in 186 of the patients. Discussion: Endoscopy can become an early diagnostic examination by increasing the availability of endoscopy. Also alarm symptoms should not be ignored and endoscopy should perform immediately in symptomatic patients. As an early result of upper gastrointestinal endoscopies that performed in this study; gastritis, esophagitis, duodenitis and hiatal hernia are common gastrointestinal diseases in our region.

  4. Clinical characteristics of patients with Rutherford category IV, compared with V and VI

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    Taketsugu Tsuchiya

    2015-07-01

    Full Text Available Objective: Patients categorized Rutherford category IV might have different characteristics compared with Rutherford category V and VI. Our study aims were to estimate the clinical differences between Rutherford category IV and Rutherford category V and VI, for those underwent endovascular therapy for isolated infrapopliteal disease, and also to find risk factors for endovascular therapy in Rutherford category IV. Methods: Based on the Japanese multi-center registry data, 1091 patients with 1332 limbs (Rutherford category IV: 226 patients with 315 limbs, Rutherford category V and VI: 865 patients with 1017 limbs were analyzed retrospectively. Results: Patients’ backgrounds and lesions’ characteristics had significant differences. Both freedom rate from major adverse limb event with perioperative death and amputation-free survival rate at 1 year were better in Rutherford category IV than Rutherford category V and VI (93.6% vs 78.3%, 87.7% vs 66.7% and those maintained to 3 years (p  3.0 mg/dL, chronic obstructive pulmonary disease, and coronary artery disease in Rutherford category IV. Conclusion: From the present results, Rutherford category IV should be recognized to have quite different backgrounds and better outcome from Rutherford category V and VI.

  5. Leptin in human physiology and pathophysiology.

    Science.gov (United States)

    Mantzoros, Christos S; Magkos, Faidon; Brinkoetter, Mary; Sienkiewicz, Elizabeth; Dardeno, Tina A; Kim, Sang-Yong; Hamnvik, Ole-Petter R; Koniaris, Anastasia

    2011-10-01

    Leptin, discovered through positional cloning 15 years ago, is an adipocyte-secreted hormone with pleiotropic effects in the physiology and pathophysiology of energy homeostasis, endocrinology, and metabolism. Studies in vitro and in animal models highlight the potential for leptin to regulate a number of physiological functions. Available evidence from human studies indicates that leptin has a mainly permissive role, with leptin administration being effective in states of leptin deficiency, less effective in states of leptin adequacy, and largely ineffective in states of leptin excess. Results from interventional studies in humans demonstrate that leptin administration in subjects with congenital complete leptin deficiency or subjects with partial leptin deficiency (subjects with lipoatrophy, congenital or related to HIV infection, and women with hypothalamic amenorrhea) reverses the energy homeostasis and neuroendocrine and metabolic abnormalities associated with these conditions. More specifically, in women with hypothalamic amenorrhea, leptin helps restore abnormalities in hypothalamic-pituitary-peripheral axes including the gonadal, thyroid, growth hormone, and to a lesser extent adrenal axes. Furthermore, leptin results in resumption of menses in the majority of these subjects and, in the long term, may increase bone mineral content and density, especially at the lumbar spine. In patients with congenital or HIV-related lipoatrophy, leptin treatment is also associated with improvements in insulin sensitivity and lipid profile, concomitant with reduced visceral and ectopic fat deposition. In contrast, leptin's effects are largely absent in the obese hyperleptinemic state, probably due to leptin resistance or tolerance. Hence, another emerging area of research pertains to the discovery and/or usefulness of leptin sensitizers. Results from ongoing studies are expected to further increase our understanding of the role of leptin and the potential clinical

  6. Clinical and laboratory characteristics of systemic anaplastic large cell lymphoma in Chinese patients

    Directory of Open Access Journals (Sweden)

    Wang Yan-Fang

    2012-07-01

    Full Text Available Abstract Background Systemic anaplastic large cell lymphoma (S-ALCL is a rare disease with a highly variable prognosis and no standard chemotherapy regimen. Anaplastic lymphoma kinase (ALK has been reported as an important prognostic factor correlated with S-ALCL in many but not all studies. In our study, we retrospectively analyzed 92 patients with S-ALCL from the Peking University Lymphoma Center for clinical and molecular prognostic factors to make clear the role of ALK and other prognostic factors in Han Chinese S-ALCL. Results The majority of Chinese S-ALCL patients were young male patients (median age 26, male/female ratio 1.7 and the median age was younger than previous reports regardless of ALK expression status. The only statistically significant different clinical characteristic in S-ALCL between ALK positive (ALK+ and ALK negative (ALK- was age, with a younger median age of 22 for ALK+ compared with 30 for ALK-. However, when pediatric patients (≤18 were excluded, there was no age difference between ALK+ and ALK-. The groups did not differ in the proportion of males, those with clinical stage III/IV (49 vs 51% or those with extranodal disease (53 vs 59%. Of 73 evaluable patients, the 3-year and 5-year survival rates were 60% and 47%, respectively. Univariate analysis showed that three factors: advanced stage III/IV, lack of expression of ALK, and high Ki-67 expression, were associated with treatment failure in patients with S-ALCL. However, ALK expression correlated with improved survival only in patients younger than 14 years, while not in adult patients. In multivariate analysis, only clinical stage was an independent prognostic factor for survival. Expressions of Wilms tumor 1 (WT1 and B-cell lymphoma 2 protein (BCL-2 correlated with the expression of ALK, but they did not have prognostic significance. High Ki-67 expression was also a poor prognostic factor. Conclusions Our results show that ALK expression alone is not

  7. Clinical and electroencephalographic characteristics of benign occipital epilepsy of childhood in two tertiary Brazilian hospitals

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    Soniza Vieira Alves-Leon

    2011-08-01

    Full Text Available This study intended to investigate the clinical and electroencephalographic benign occipital epilepsy of childhood (BOEC characteristics in a population sample of patients from two tertiary Brazilian hospitals. We analyzed retrospectively 4912 electroencephalograms (EEGs records, and the included patients were submitted to a new clinical and EEG evaluation. Were included 12 (0.92% patients; 4 (33.3% with criteria for early BOEC; 6 (50% for late form and 2 (16.7% with superimposed early and late onset forms. After new investigation, 2 (16.7% had normal EEG; 4 (33.3% had paroxysms over the occipital region; 3 (25% over the temporal posterior regions and 3 (25% over the posterior regions. Sharp waves were the predominant change, occurring in 8 (66.6%; spike and slow wave complexes in 1 (8.3% and sharp and slow wave complexes in 1 (8.3%. Vomiting, headache and visual hallucinations were the most common ictal manifestations, presented in 100% of patients with superimposed forms. Vomiting were absent in the late form and headache was present in all forms of BOEC.

  8. Giant cell tumor of bone: current review of morphological, clinical, radiological, and therapeutic characteristics

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    Georgi P. Georgiev

    2014-09-01

    Full Text Available Giant cell tumor of bone accounts for about 5% of all primary bone tumors in adults and is still one of the most obscure and intensively examined tumors of bone. This largely results from the lack of uniform clinical, radiographic, histological or morphological aspects that allow prediction of recurrence. Classified by the World Health Organization as “an aggressive, potentially malignant lesion”, the giant cell tumor of bone could give lung metastases, could undergo malignant degeneration or could have multicentric localization. It usually develops in long bones but can also occur in unusual locations. The common presenting symptom is increasing pain at the tumor site. Standard treatment ranges from curettage to wide resection, with reports of varying oncological and functional results. The recurrence rate is high during the first 2-3 years after surgery regardless of pre-operative tumor stage. Herein, we discuss the morphological, clinical, radiological, and therapeutic characteristics of this pathologic entity as well as its differential diagnosis. J Clin Exp Invest 2014; 5 (3: 475-485

  9. Clinical characteristics and prognosis of cerebral venous thrombosis in Chinese women during pregnancy and puerperium

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    Liang, Zhu-Wei; Gao, Wan-Li; Feng, Li-Min

    2017-01-01

    Due to the specific physiology associated with pregnancy and puerperium, cerebral venous sinus thrombosis (CVT) may manifest different characteristics. This study aimed to identify the clinical manifestations and prognosis of pregnancy-associated CVT. A total of 43 pregnancy-associated CVT patients were enrolled. We analysed the clinical presentations of the disease and performed a multivariate logistic regression analysis to determine which variables were associated with prognosis. Our descriptive results showed the following: 1) the incidence was 202 per 100,000 deliveries, and the mortality rate was 11.63%; 2) the most frequent symptom was headache; 3) the most frequent abnormal laboratory findings were increased levels of fibrinogen and several serum lipoproteins (including triglyceride, cholesterol, high-density lipoprotein, low-density lipoprotein, apolipoprotein A1, and apolipoprotein B); and 4) the superior sagittal sinus and transverse sinus were the most frequently affected locations. Moreover, an increased modified Rankin Scale score was positively associated with infection, seizure, intracerebral haemorrhage (ICH) and hypertensive disorders of pregnancy (HDP). Comparably, the occurrence of death was positively and significantly associated with infection, seizure and ICH. Consequently, timely diagnosis and treatment of pregnancy-associated CVT patients with infection, seizure, ICH or HDP are needed. Patients with infection, seizure or ICH have a greater risk of death. PMID:28262755

  10. The reliability of lung crackle characteristics in cystic fibrosis and bronchiectasis patients in a clinical setting.

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    Marques, Alda; Bruton, Anne; Barney, Anna

    2009-09-01

    Lung sounds provide useful information for assessing and monitoring respiratory patients, but standard auscultation is subjective. Computer aided lung sound analysis (CALSA) enables the quantification and characterisation of added lung sounds (e.g. crackles). At present, little is known about the reliability of these sound characteristics. Therefore, the aim of this study was to explore the reliability of crackle initial deflection width (IDW) and two-cycle deflection (2CD) in a clinical population. Fifty-four subjects (37 bronchiectasis, 17 cystic fibrosis) were recruited from out-patient clinics. Three repeated lung sound recordings were taken at seven anatomical sites with a digital stethoscope connected to a laptop computer. The intra-subject reliability of crackle IDW and 2CD was found to be 'good' to 'excellent', estimated by the analysis of variance, intraclass correlation coefficient (IDW 0.76;0.85, 2CD 0.83;0.94), Bland and Altman 95% limits of agreement (IDW -0.50;0.47 ms, 2CD -2.12;1.87 ms) and smallest real difference (IDW 0.30;0.66 ms, 2CD 1.57;2.42 ms). Crackle 2CD was found to be more reliable than IDW. It is concluded that crackle IDW and 2CD characterized by CALSA have good test-retest reliability. This technique requires further evaluation since CALSA has potential to diagnose or monitor respiratory conditions, and provide an objective physiological measure for respiratory interventions.

  11. Clinical characteristics of patients with labyrinthine fistulae caused by middle ear cholesteatoma

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    YANG Juan-mei; CHI Fang-lu; HAN Zhao; HUANG Yi-bo; LI Yi-ke

    2013-01-01

    Background Labyrinthine fistula (LF) is a very common clinical complication mainly caused by middle ear cholesteatoma.Whether the presence of different degree LF caused by middle ear cholesteatoma aggravates neurosensory hearing loss (NSHL) and what is the degree of the hearing loss caused by LF were still under controversial.This study aimed to investigate whether the LF degree is correlative with the age distribution,disease duration and hearing loss degree for cholesteatomatous patients.Methods The files of 143 patients with middle ear cholesteatoma were selected and reviewed in a retrospective study.Seventy-eight patients with LF were divided into three types according to the degree of destruction of labyrinth.Sixty-five patients without LF were randomly chosen for control.Then,we compared the clinical characteristics of patients with or without labyrinthine fistulae caused by middle ear cholesteatoma.Results According to the study,cholesteatomatous patients with LF were older and suffered longer disease duration than those without LF.Hearing loss is severe with high frequencies both in patients with and without LF.Moreover,inner ear impairment is correlative with the degree of destruction in labyrinth,and more severe destruction in labyrinth follow the more severe symptoms correlative with inner ear impairment.Conclusion Surgical intervention should be performed as eady as possible for these cholesteatomatous patients.

  12. Clinical characteristics and prognostic factors of patients with Stenotrophomonas maltophilia infections

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    Batra, Priyam; Mathur, Purva; Misra, Mahesh C.

    2017-01-01

    INTRODUCTION: Stenotrophomonas maltophilia earlier had limited pathogenic potential, but now with growing degree of immunosuppression in general population, it is being recognized as an important nosocomial pathogen. METHODOLOGY: A retrospective 7 years study was carried out to determine the clinical characteristics of all patients with Stenotrophomonas infections, antibiotic resistance pattern, and risk factors associated with hospital mortality. All patients with Stenotrophomonas culture positivity were identified and their medical records were reviewed. Risk factor associated with hospital mortality was analyzed. RESULTS: A total of 123 samples obtained from 88 patients were culture positive. Most patients presented with bacteremia (45, 51%) followed by pneumonia (37, 42%) and skin and soft tissue infections (6, 7%). About 23 of 88 Stenotrophomonas infected patients had co-infection. Percentage resistance to cotrimoxazole; 8 (5.4%) was lower than that for levofloxacin; 18 (12%). Twenty-eight patients died during hospital stay. Intensive Care Unit admission (P = 0.0002), mechanical ventilation (P = 0.0004), central venous catheterization (P = 0.0227), urethral catheterization (P = 0.0484), and previous antibiotic intake (P = 0.0026) were independent risk factors associated with mortality. CONCLUSION: Our findings suggest that Stenotrophomonas can cause various infections irrespective of patient's immune status and irrespective of potential source. Thus, Stenotrophomonas should be thought of as potential pathogen and its isolation should be looked with clinical suspicion.

  13. Clinical Characteristics of C9ORF72-Linked Frontotemporal Lobar Degeneration

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    Anna-Lotta Kaivorinne

    2013-08-01

    Full Text Available Background: The most common genetic cause of frontotemporal lobar degeneration (FTLD and amyotrophic lateral sclerosis (ALS has been linked to a hexanucleotide repeat expansion in the C9ORF72 gene. The frequency of the C9ORF72 expansion in Finland is among the highest in the world. Methods: We assessed 73 Finnish patients with FTLD in order to examine the clinical characteristics associated with the expanded C9ORF72. Demographic and clinical features were evaluated. As a potential disease modifier, the apolipoprotein E (APOE genotype was also assessed. Neuropathological analysis was available on 2 expansion carriers and 1 non-carrier. Results: The C9ORF72 expansion was present in 20 of 70 (29% probands. Significant associations with the C9ORF72 expansion were observed for concomitant ALS and positive family history of dementia or ALS. Psychoses were detected in both carriers and non-carriers (21 vs. 10%, p = 0.25. The APOE ε4 allele did not cluster among expansion carriers. Numerous p62-positive neuronal inclusions were detected in the cerebellar cortex of the 2 expansion carriers. Conclusion: In line with the suggested C9ORF72 core phenotype, we also detected a high frequency of neuropsychiatric symptoms; however, these symptoms seem not be specific to C9ORF72-associated FTLD. FTLD should be considered in cases of middle-age-onset psychosis.

  14. Dysmorphic Facial Features and Other Clinical Characteristics in Two Patients with PEX1 Gene Mutations

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    Mehmet Gunduz

    2016-01-01

    Full Text Available Peroxisomal disorders are a group of genetically heterogeneous metabolic diseases related to dysfunction of peroxisomes. Dysmorphic features, neurological abnormalities, and hepatic dysfunction can be presenting signs of peroxisomal disorders. Here we presented dysmorphic facial features and other clinical characteristics in two patients with PEX1 gene mutation. Follow-up periods were 3.5 years and 1 year in the patients. Case I was one-year-old girl that presented with neurodevelopmental delay, hepatomegaly, bilateral hearing loss, and visual problems. Ophthalmologic examination suggested septooptic dysplasia. Cranial magnetic resonance imaging (MRI showed nonspecific gliosis at subcortical and periventricular deep white matter. Case II was 2.5-year-old girl referred for investigation of global developmental delay and elevated liver enzymes. Ophthalmologic examination findings were consistent with bilateral nystagmus and retinitis pigmentosa. Cranial MRI was normal. Dysmorphic facial features including broad nasal root, low set ears, downward slanting eyes, downward slanting eyebrows, and epichantal folds were common findings in two patients. Molecular genetic analysis indicated homozygous novel IVS1-2A>G mutation in Case I and homozygous p.G843D (c.2528G>A mutation in Case II in the PEX1 gene. Clinical findings and developmental prognosis vary in PEX1 gene mutation. Kabuki-like phenotype associated with liver pathology may indicate Zellweger spectrum disorders (ZSD.

  15. Preconception Counseling and Care in the Setting of HIV: Clinical Characteristics and Comorbidities

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    Rupsa C. Boelig

    2015-01-01

    Full Text Available Objective. To describe the demographic and clinical characteristics of HIV-infected individuals and HIV-affected couples who were referred for preconception counseling (PCC at a large urban US-based HIV clinic. Methods. Electronic medical records were reviewed for HIV-infected individuals and HIV-affected couples. Medical, reproductive, surgical, psychosocial, and family history data were abstracted. Univariate analyses were done. Results. There were 8 single HIV-infected women and 100 HIV-affected couples who underwent PCC. HIV-infected women were older (mean age 35 years versus 32 years, P=0.06, were more likely to smoke (23% versus 0%, P<0.01, and had more medical comorbidities (57% versus 33%, P=0.04 than HIV-uninfected women. The majority of couples were serodiscordant (77%, and of these couples, 32% had a detectable plasma viral load and 33% report inconsistent condom use. Conclusions. HIV-infected women have a number of medical and psychosocial issues, including those related to HIV that may increase the risk of adverse pregnancy outcomes and HIV perinatal and sexual transmission. PCC is an important intervention to optimize maternal management to improve perinatal outcomes and minimize transmission risks.

  16. Clinical characteristics of a group of adults with nodular lymphoid hyperplasia: A single center experience

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    Alberto Rubio-Tapia; Jorge Hernéndez-Calleros; Sagrario Trinidad-Hernández; Luis Uscanga

    2006-01-01

    AIM: To describe the clinical and histological characteristics of a group of adults with small-bowel nodular lymphoid hyperplasia (NLH).METHODS: Patients were searched for five years in pathology records of our institution. The biopsy material was reassessed using strict histopathological criteria.Clinical data were obtained from medical records.RESULTS: Small-bowel NLH was diagnosed in 18 cases.The female: male ratio was 2: 1. The most frequent symptoms were diarrhea (72%), involuntary weight loss (72%) and abdominal pain (61%). Nine patients (50%)had immunodeficiency. Small-bowel bacterial overgrowth was found in three (17%) cases. At small-bowel NLH diagnosis, three (17%) had associated lymphoma: two intestinal and one extra-intestinal lymphomas. In two patients with villous atrophy and anti-endomysial antibodies the diagnosis of celiac disease was established.Giardia lamblia infection was found in only one patientwith hypogammaglobulinemia (Herman's syndrome).CONCLUSIONS; NLH is uncommon in adult patients.Associated diseases are immunodeficiency and lymphoid tissue malignancies.

  17. [Heterogenous abnormality polymorphism of gene PDGFRB in myeloid neoplasms and its clinical characteristics].

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    Wang, Quan-Shun; Gao, Li; Jing, Yu; Zhu, Hai-Yan; Yang, Hua; Yu, Li

    2012-04-01

    Myeloid neoplasms with eosinophilia and abnormalities of PDGFRB gene are a new kind of myeloid disorders in the revised 2008 WHO classification. Out of detected 2000 cases of myeloid cell abnormalities in our hospital, 12 cases of myeloid neoplasms with eosinophilia and abnormalities of PDGFRB were found. This study was purposed to summarize and analyze the clinical and laboratorial characteristics of the 12 cases with PDGFRB gene abnormalities. The results indicated that among 12 cases of myeloid neoplasms with PDGFRB abnormalities, 5 cases with TEL/PDGFRB fusion gene, 2 cases with HEPI/PDGFRB, 1 case with PDGFRB mutation, 1 case with RABAPTIN-5/PDGFRB, 1 case with GIT2/PDGFRB, 1 case with TP53/PDGFRB, 1 case with WDR43/PDGFRB fusion gene were detected, showing the polymorphism of PDGFRB gene abnormalities. Among this kind of myeloid neoplasm patients, almost all patients manifested monocytosis and eosinophilia in different degree, the thrombocytosis mainly was observed in atypical myeloid neoplasms, acute leukemia, chromic myelo-monocytic leukemia patients. The treatment with imatinib mesylate for this kind of patients was effective in some cases. It is concluded that the myeloid neoplasms with PDGFRB gene abnormalities are a kind of heterogenetic myeloid neoplasms, their gene abnormal types and clinical manifestations show polymorphism too. The monocytosis and eosinophilia appear in this kind myeloid neoplasms which may be treated with tyrosine kinase inhibitors such as imatinib mesylate.

  18. Clinical characteristics of children and adolescents with severe therapy-resistant asthma in Brazil

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    Andrea Mendonça Rodrigues

    2015-08-01

    Full Text Available AbstractObjective: To describe the clinical characteristics, lung function, radiological findings, and the inflammatory cell profile in induced sputum in children and adolescents with severe therapy-resistant asthma (STRA treated at a referral center in southern Brazil.Methods: We retrospectively analyzed children and adolescents (3-18 years of age with uncontrolled STRA treated with high-dose inhaled corticosteroids and long-acting β2 agonists. We prospectively collected data on disease control, lung function, skin test reactivity to allergens, the inflammatory cell profile in induced sputum, chest CT findings, and esophageal pH monitoring results.Results: We analyzed 21 patients (mean age, 9.2 ± 2.98 years. Of those, 18 (86% were atopic. Most had uncontrolled asthma and near-normal baseline lung function. In 4 and 7, induced sputum was found to be eosinophilic and neutrophilic, respectively; the inflammatory cell profile in induced sputum having changed in 67% of those in whom induced sputum analysis was repeated. Of the 8 patients receiving treatment with omalizumab (an anti-IgE antibody, 7 (87.5% showed significant improvement in quality of life, as well as significant reductions in the numbers of exacerbations and hospitalizations.Conclusions: Children with STRA present with near-normal lung function and a variable airway inflammatory pattern during clinical follow-up, showing a significant clinical response to omalizumab. In children, STRA differs from that seen in adults, further studies being required in order to gain a better understanding of the disease mechanisms.

  19. Clinical characteristics and prognostic factors for relapse in patients with polymyalgia rheumatica (PMR).

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    Lee, Jung Hwa; Choi, Sang Tae; Kim, Jin Su; Yoon, Bo Young; Kwok, Seung-Ki; Kim, Hyun-Sook; Kim, Yun Sung; Song, Jung-Soo; Lee, Sang-Heon; Kim, Hae-Rim

    2013-06-01

    Polymyalgia rheumatica (PMR) is a common inflammatory disease of the elderly in western countries, but the prevalence is apparently different between races and countries. Until now, an epidemiologic study of PMR is limited in Korea. We retrospectively evaluated the clinical data of 78 patients with PMR who were treated in 5 tertiary hospitals, and analyzed initial laboratory data, symptoms, therapeutic responses, and prognostic factors for relapse 1 year after treatments. Sixty percent of patients had pain in both shoulder and hip girdles with 10.6 weeks of duration, 75.9 ± 32.7 mm/h of erythrocyte sedimentation rate (ESR), and 6.2 ± 6.4 mg/dl of C-reactive protein. The rate of relapse and remission at 1 year was 38.4 and 2.5 %, respectively. The rate of overall relapse was 46.1 %, and the relapse occurred mostly in a year, especially between 6 and 12 months after diagnosis. There were more female in relapse group (88.9 %, p = 0.037), and cumulative steroid dose of 1 year was significantly higher in relapse group (5.5 ± 2.7 vs. 4.4 ± 2.5 g, p = 0.018). Independent risk factors for relapse were initial CRP ≥ 2.5 mg/dl (OR 6.296, p = 0.047) and the use of hydroxychloroquine (OR 6.798, p = 0.035). Initial dosage or tapering speed of steroid did not influence on prognosis. In Korean patients with PMR, baseline clinical characteristics and relapse rate were similar to previous studies, but our patients accompanied no giant cell arteritis and showed lower remission rate as well as delayed therapeutic response and later occurrence of relapse. More aggressive management would be needed according to the clinical status of patients.

  20. Prevalence, Clinical Characteristics, and Predictors of Patients with Thromboembolic Events in Takotsubo Cardiomyopathy

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    El-Battrawy, Ibrahim; Behnes, Michael; Hillenbrand, Dennis; Haghi, Darius; Hoffmann, Ursula; Papavassiliu, Theano; Lang, Siegfried; Fastner, Christian; Becher, Tobias; Baumann, Stefan; Heggemann, Felix; Kuschyk, Jürgen; Borggrefe, Martin; Akin, Ibrahim

    2016-01-01

    BACKGROUND Several acute complications related to takotsubo cardiomyopathy (TTC) have been documented recently. However, the incidence and clinical significance of acute thromboembolic events in TTC is not well established. METHODS A detailed investigation of the clinical characteristics and in-hospital complications of 114 consecutive patients diagnosed with TTC between January 2003 and September 2015 was carried out. This study was initiated to reveal the predictors, clinical significance, and short-term and long-term outcomes of patients with TTC associated with acute thromboembolic events on index presentation. RESULTS The incidence of acute thromboembolic events related to TTC was around 12.2%, and these included ventricular thrombi, cerebrovascular events, retinal and brachial artery pathologies, renal, splenic, and aortic involvement. The most frequent complication on initial presentation was cardiogenic shock (20%) accompanied with pulmonary congestion (20%). Interestingly, patients experiencing thromboembolic events had higher C-reactive protein (CRP) levels as compared to the non-thromboembolic group (P = 0.02). Certain thromboembolic events were characterized by the presence of ST-segment elevation in electrocardiogram (P = 0.02). Chest pain was the primary symptom in these patients (P = 0.09). Furthermore, there was significant right ventricular involvement (as assessed by transthoracic echocardiography) in patients presenting with an acute thromboembolic event (P = 0.08). A Kaplan–Meier analysis indicated a significantly higher mortality rate over a mean follow-up of three years in the thromboembolic group than the non-thromboembolic group (log-rank, P = 0.02). CONCLUSIONS Our results confirmed the relative common occurrence of thromboembolic events in the setting of TTC. Inflammation might play an important role in the development of thromboembolic events, and a right ventricular involvement and ST-segment elevation could be positive predictors for

  1. Respiratory viral pathogens among Singapore military servicemen 2009 – 2012: epidemiology and clinical characteristics

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    2014-01-01

    Background Few studies have comprehensively described tropical respiratory disease surveillance in military populations. There is also a lack of studies comparing clinical characteristics of the non-influenza pathogens with influenza and amongst themselves. Methods From May 2009 through October 2012, 7733 consenting cases of febrile respiratory illness (FRI) (temperature [greater than or equal to]37.5degreesC with cough or sorethroat) and controls in the Singapore military had clinical data and nasal washes collected prospectively. Nasal washes underwent multiplex PCR, and the analysis was limited to viral mono-infections. Results 49% of cases tested positive for at least one virus, of whom 10% had multiple infections. 53% of the FRI cases fulfilled the definition of influenza-like illness (ILI), of whom 52% were positive for at least one virus. The most frequent etiologies for mono-infections among FRI cases were Influenza A(H1N1)pdm09 (13%), Influenza B (13%) and coxsackevirus (9%). The sensitivity, specificity, positive predictive value and negative predictive value of ILI for influenza among FRI cases were 72%, 48%, 40% and 69% respectively. On logistic regression, there were marked differences in the prevalence of different symptoms and signs between viruses with fever more prevalent amongst influenza and adenovirus infections than other viruses. Conclusion There are multiple viral etiologies for FRI and ILI with differing clinical symptoms in the Singapore military. Influenza and coxsackevirus were the most common etiology for FRI, while influenza and adenoviruses displayed the most febrile symptoms. Further studies should explore these differences and possible interventions. PMID:24735158

  2. Clinical Characteristics of patients with Diabetes Mellitus 2 of Family Health Center Chiguay.

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    Ignacio López H. Marco Mendoza E. Consuelo Rodríguez M. Lesly Álvarez C

    2009-07-01

    Full Text Available Introduction : Diabetes mellitus (DM has a national prevalence near 4.2% and the worldwide fluctuates between 2 and 5%. The complications are caused by poor metabolic control and hence also by the poor management of cardiovascular risk factors (CVRF, hence the importance of managing and reducing the CVRF, the main objective of control programs for DM. AIM: To characterize clinically to patients with DM2 who were cared for in the agenda of cardiovascular CESFAM Chiguay (commune of Chiguayante, region of Bio Bio during the first 9 months of 2008. PATIENT and method S: Observational descriptivestudy, non-probability sampling a retrospective study was observational and descriptive, reviewing the ballots of each tabcontrol chronic clinic patients enrolled in the program Cardiovascular(VCP from January to September 2008 in CESFAM “Chiguay.” Results : The study included 332 patients, of whom 67.77% were women and 32.23% men. Average age was 63.6 years. 47.59% were obese. 49.39% had a HbA1c levels at about 7%. 21.69% had an “optimal” control of LDL. 56.93% had blood pressure poorly controlled. Discussion : The most noticeable clinical and epidemiological characteristics of the diabetes type 2 found in our study were late aged dominance in females and obese. Almost 80% of the cases had higher levels of LDL and only around a half had good metabolic control, with HbA1c under7%. This could be explained because the studied subjects belongedmostly to a low socioeconomic status, which would lead to unbalanced diet, poor adherence to the treatment and poor understanding of their pathology.

  3. Carpal tunnel syndrome: Assessment of correlation between clinical, neurophysiological and ultrasound characteristics

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    B Hemeshwar Rao

    2012-01-01

    Full Text Available Objectives: To evaluate the relationship between symptoms, clinical severity, neurophysiological characteristics with median nerve cross-sectional area (CSA at the level of carpal tunnel inlet at ultrasonography (USG and its utility in diagnosis of carpal tunnel syndrome (CTS. Materials and Methods: Prospective study of 30 patients with symptoms of CTS, attending to the Neurology out patient department (OPD at University Teaching Hospital. A multidimensional assessment of CTS was done using historic and objective scale (Hi-Ob scale for clinical severity, Boston carpal tunnel questionnaire (BCTQ for patient-oriented measures, neuro physiologic studies of median nerve at wrist and USG to measure median nerve CSA at carpal tunnel inlet. Results: Thirty patients included in the study (22 women and 8 men. Mean of CSA was 12.69 mm 2 (SD2.67. Association between BCTQ score value and inlet values was assessed by Karl Pearson correlation coefficient ( r = 0.376, P = 0.04. There was positive association with BCTQ scores and CSA of median nerve at carpal tunnel inlet. To compare clinical severity scale (Hi-Ob and USG CSA, analysis of variance was performed (F value and Scheffe′s multiple comparison test was used to find group difference (grades 1 and 2 P < 0.001, grades1 and 2P P < 0.001, grade 2 differs with grade 2P P < 0.006. As the number of patients is less in minimal, mild and severe groups, the difference between neurophysiological groups and mean of CSA was not statistically significant. Conclusion: A positive correlation exists between USG findings and all the conventional measures of CTS severity.

  4. Clinical and demographic characteristics of patients with occupational contact dermatitis: A 3-year single center experience

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    Aslı Aytekin

    2015-12-01

    Full Text Available Background and Design: Occupational contact dermatitis (OCD is responsible for 80-90% of the occupational dermatoses. The aim of this work was to evaluate the clinical features of patients with OCD admitted to our hospital. Materials and Methods: The records of patients, who were admitted to our hospital with OCD between December 2009 and January 2013, were evaluated retrospectively. One hundred fifty-nine patients, who were diagnosed with OCD according to the Mathias criteria, were included in the study. Age, sex, location of the lesions, atopic status, glove use, occupational exposure time and total IgE levels of the patients were assessed. Patients with positive allergic reaction with "European Standard Series Skin Patch Test" were identified as allergic OCD and patients with negative test results as "irritant OCD". The clinical features and patch results of patients are evaluated. Results: One hundred fifty-nine patients with a mean age of 39±7.9 years consisted of 151 men and 8 women. The hands were the most common site of OCD; the palms were the most common affected areas of hand eczema. Eighty-one patients (50.1% were identified to have allergic OCD and 78 (49.9% as irritant OCD. Irritant OCD was most commonly seen in dental technicians, whereas allergic OCD was most commonly seen in tailors. The top 3 most frequent allergens were potassium dichromate (15.1%, nickel sulfate (9.11% and cobalt chloride (10.7%. Conclusion: In our country, there has been no comprehensive study presenting the clinical and descriptive characteristics of OCD. For preventing OCD and reducing sick leave we need to have data that belong to our country. Consequently, multicenter studies should be performed for establishing our own database on OCD.

  5. The clinical features, outcomes and genetic characteristics of hypertrophic cardiomyopathy patients with severe right ventricular hypertrophy

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    Guo, Xiying; Fan, Chaomei; Tian, Lei; Zhang, Xiuling; Zhao, Xing; Wang, Fengqi; Zhu, Hongguang; Lin, Aiqing; Wu, Xia; Li, Yishi

    2017-01-01

    Introduction Severe right ventricular hypertrophy (SRVH) is a rare phenotype in hypertrophic cardiomyopathy (HCM) for which limited information is available. This study was undertaken to investigate the clinical, prognostic and genetic characteristics of HCM patients with SRVH. Methods HCM with SRVH was defined as HCM with a maximum right ventricular wall thickness ≥10 mm. Whole-genome sequencing (WGS) was performed in HCM patients with SRVH. Multivariate Cox proportional hazards regression models were used to identify risk factors for cardiac death and events in HCM with SRVH. Patients with apical hypertrophic cardiomyopathy (ApHCM) were selected as a comparison group. The clinical features and outcomes of 34 HCM patients with SRVH and 273 ApHCM patients were compared. Results Compared with the ApHCM group, the HCM with SRVH group included younger patients and a higher proportion of female patients and also displayed higher cardiovascular morbidity and mortality. The multivariate Cox proportional hazards regression models identified 2 independent predictors of cardiovascular death in HCM patients with SRVH, a New York Heart Association class ≥III (hazard ratio [HR] = 8.7, 95% confidence interval (CI): 1.43-52.87, p = 0.019) and an age at the time of HCM diagnosis ≤18 (HR = 5.5, 95% CI: 1.24-28.36, p = 0.026). Among the 11 HCM patients with SRVH who underwent WGS, 10 (90.9%) were identified as carriers of at least one specific sarcomere gene mutation. MYH7 and TTN mutations were the most common sarcomere mutations noted in this study. Two or more HCM-related gene mutations were observed in 9 (82%) patients, and mutations in either other cardiomyopathy-related genes or ion-channel disease-related genes were found in 8 (73%) patients. Conclusions HCM patients with SRVH were characterized by poor clinical outcomes and the presentation of multiple gene mutations. PMID:28323875

  6. Clinical and histopathological characteristics of basal cell carcinoma in Chinese patients

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    CHANG Jian-min; GAO Xiao-man

    2013-01-01

    Background The clinical and histopathological characteristics of basal cell carcinoma (BCC) have been relatively well studied in Caucasian population.To characterize BCC in Chinese population,we analyzed the association of the histopathological subtypes with gender,age and anatomical location in this study.Methods The clinical and histopathological data of 243 BCC cases diagnosed at three hospitals in Beijing from January 2000 to April 2009 were reviewed retrospectively.Gender,age,location and histopathological subtype were analyzed.Results Among 243 patients enrolled,118 were males and 125 were females.The male/female ratio was 0.94∶1.The mean age was (65.16±12.62) years old.The head and neck were the most common sites of BCC (77.4%).Of the BCCs,53.9% were nodular,18.9% superficial and 18.5% infiltrative-morphoeic.The nodular,infiltrative-morphoeic and micronodular subtypes were predominant located on the head and neck,whereas the trunk was the most common location for the superficial subtype (P <0.05).The age at first presentation for females was lower than that for males (P<0.05).The age at first presentation for the superficial BCCs was younger than the non-superficial subtypes (P <0.05).Women with superficial BCC subtype visited hospital earlier than men (P <0.05).Conclusions Consistent with previous reports in Caucasian patient,our study find that different histopathological subtypes of BCC has distinct clinical features.It is speculated that the mechanisms underlining the pathogenesis of the superficial BCC may be different than those of non-superficial subtypes of BCC.

  7. Clinical and laboratory characteristics of acute community-acquired urinary tract infections in adult hospitalised patients.

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    Piljic, Dilista; Piljic, Dragan; Ahmetagic, Sead; Ljuca, Farid; Porobic Jahic, Humera

    2010-02-01

    Urinary tract infections (UTI) cause a great number of morbidity and mortality. These infections are serious complications in pregnancy, patients with diabetes, polycystic kidneys disease, sickle cell anaemia, kidney transplant and in patients with functional or structural anomalies of the urinary tract. The aim of this investigation was to determine a dominant causative agents of UTI and some of the clinical and laboratory characteristics of acute community-acquired UTI in adult hospitalised patients. We studied 200 adult patients with acute community-acquired UTI hospitalised in the Clinic for Infectious Diseases Tuzla from January 2006 to December 2007. The patients were divided into two groups: a group of patients with E. coli UTI (147) and a group of patients with non-E. coli UTI (53). In these two groups, the symptoms and signs of illness, blood test and urine analysis results were analysed. Our results have shown that the patients with E. coli UTI frequently had fever higher than 38,5 degrees C (p<0,0001), chills (p=0,0349), headache (p=0,0499), cloudy urine (p<0,0001), proteinuria (p=0,0011) and positive nitrite-test (p=0,0002). The patients with non-E. coli UTI frequently had fever lower than 38,5 degrees C (p<0,0001) and urine specific gravity <1015 (p=0,0012). There was no significant difference in blood test results between patients with E. coli and non-E. coli UTI. These clinical and laboratory findings can lead us to early etiological diagnosis of these UTI before urine culture detection of causative agents, which takes several days. Early etiological diagnosis of the E. coli and non-E. coli UTI is necessary for an urgent administration of appropriate empirical antibiotic treatment. This is very important in prevention of irreversible kidney damage, prolonged treatment, complications, as well as recidives and chronicity of the illness.

  8. Clinical and microbiologic characteristics of invasive Streptococcus pyogenes infections in north and south India.

    Science.gov (United States)

    Haggar, Axana; Nerlich, Andreas; Kumar, Rajesh; Abraham, Vinod J; Brahmadathan, Kootallur N; Ray, Pallab; Dhanda, Vanita; Joshua, John Melbin Jose; Mehra, Narinder; Bergmann, Rene; Chhatwal, G Singh; Norrby-Teglund, Anna

    2012-05-01

    The lack of epidemiologic data on invasive Streptococcus pyogenes infections in many developing countries is concerning, as S. pyogenes infections are commonly endemic in these areas. Here we present the results of the first prospective surveillance study of invasive Streptococcus pyogenes infections in India. Fifty-four patients with invasive S. pyogenes infections were prospectively enrolled at two study sites, one in the north and one in the south of India. Sterile-site isolates were collected, and clinical information was documented using a standardized questionnaire. Available acute-phase sera were tested for their ability to inhibit superantigens produced by the patient's own isolate using a cell-based neutralizing assay. The most common clinical presentations were bacteremia without focus (30%), pneumonia (28%), and cellulitis (17%). Only two cases of streptococcal toxic shock syndrome and no cases of necrotizing fasciitis were identified. Characterization of the isolates revealed great heterogeneity, with 32 different emm subtypes and 29 different superantigen gene profiles being represented among the 49 sterile-site isolates. Analyses of acute-phase sera showed that only 20% of the cases in the north cohort had superantigen-neutralizing activity in their sera, whereas 50% of the cases from the south site had neutralizing activity. The results demonstrate that there are important differences in both clinical presentation and strain characteristics between invasive S. pyogenes infections in India and invasive S. pyogenes infections in Western countries. The findings underscore the importance of epidemiologic studies on streptococcal infections in India and have direct implications for current vaccine developments.

  9. Association of upper gastrointestinal symptoms with functional and clinical characteristics in the elderly

    Institute of Scientific and Technical Information of China (English)

    Alberto Pilotto; Stefania Maggi; Marianna Noale; Marilisa Franceschi; Giancarlo Parisi; Gaetano Crepaldi

    2011-01-01

    AIM: To evaluate the prevalence of upper gastrointestinal symptoms and their association with clinical and functional characteristics in elderly outpatients. METHODS: The study involved 3238 outpatients ≥ 60 years consecutively enrolled by 107 general practitioners. Information on social, behavioral and demographic characteristics, function in the activities of daily living (ADL), co-morbidities and drug use were collected by a structured interview. Upper gastrointestinal symptom data were collected by the 15-items upper gastro-intestinal symptom questionnaire for the elderly, a validated diagnostic tool which includes the following five symptom clusters: (1) abdominal pain syndrome; (2) reflux syndrome; (3) indigestion syndrome; (4) bleeding; and (5) non-specific symptoms. Presence and severity of gastrointestinal symptoms were analyzed through a logistic regression model. RESULTS: 3100 subjects were included in the final analysis. The overall prevalence of upper gastrointestinal symptoms was 43.0%, I.e. Cluster (1) 13.9%, (2) 21.9%, (3) 30.2%, (4) 1.2%, and (5) 4.5%. Upper gastrointestinal symptoms were more frequently reported by females (P < 0.0001), with high number of co-morbidities (P < 0.0001), who were taking higher number of drugs (P < 0.0001) and needed assistance in the ADL. Logistic regression analysis demonstrated that female sex (OR = 1.39, 95% CI: 1.17-1.64), disability in the ADL (OR = 1.47, 95% CI: 1.12-1.93), smoking habit (OR = 1.29, 95% CI: 1.00-1.65), and body mass index (OR = 1.06, 95% CI: 1.04-1.08), as well as the presence of upper (OR = 3.01, 95% CI: 2.52-3.60) and lower gastroenterological diseases (OR = 2.25, 95%CI: 1.70-2.97), psychiatric (OR = 1.60, 95% CI: 1.28-2.01) and respiratory diseases (OR = 1.25, 95% CI: 1.01-1.54) were significantly associated with the presence of upper gastrointestinal symptoms. CONCLUSION: Functional and clinical characteristics are associated with upper gastrointestinal symptoms. A multidimensional

  10. Cardiorenal Syndromes: Pathophysiology to Prevention

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    Peter A. McCullough

    2011-01-01

    Full Text Available There is a strong association between both acute and chronic dysfunction of the heart and kidneys with respect to morbidity and mortality. The complex interrelationships of longitudinal changes in both organ systems have been difficult to describe and fully understand due to a lack of categorization of the common clinical scenarios where these phenomena are encountered. Thus, cardiorenal syndromes (CRSs have been subdivided into five syndromes which represent clinical vignettes in which both the heart and the kidney are involved in bidirectional injury and dysfunction via a final common pathway of cell-to-cell death and accelerated apoptosis mediated by oxidative stress. Types 1 and 2 involve acute and chronic cardiovascular disease (CVD scenarios leading to acute kidney injury (AKI or accelerated chronic kidney disease (CKD. Types 3 and 4 describe AKI and CKD, respectively, leading primarily to heart failure, although it is possible that acute coronary syndromes, stroke, and arrhythmias could be CVD outcomes in these forms of CRS. Finally, CRSs type 5 describe a systemic insult to both heart and the kidneys, such as sepsis, where both organs are injured simultaneously in persons with previously normal heart and kidney function at baseline. Both blood and urine biomarkers, including the assessment of catalytic iron, a critical element to the generation of oxygen-free radicals and oxidative stress, are reviewed in this paper.

  11. The rise of pathophysiologic research in the United States: the role of two Harvard hospitals.

    Science.gov (United States)

    Tishler, Peter V

    2013-01-01

    Pathophysiologic research, the major approach to understanding and treating disease, was created in the 20th century, and two Harvard-affiliated hospitals, the Peter Bent Brigham Hospital and Boston City Hospital, played a key role in its development. After the Flexner Report of 1910, medical students were assigned clinical clerkships in teaching hospitals. Rockefeller-trained Francis Weld Peabody, who was committed to investigative, pathophysiologic research, was a critical leader in these efforts. At the Brigham, Harvard medical students observed patients closely and asked provocative questions about their diseases. Additionally, physicians returned from World War I with questions concerning the pathophysiology of wartime injuries. At the Boston City Hospital's new Thorndike Memorial Laboratory, Peabody fostered investigative question-based research by physicians. These physicians expanded pathophysiologic investigation from the 1920s. Post-war, Watson and Crick's formulation of the structure of DNA led shortly to modern molecular biology and new research approaches that are being furthered at the Boston Hospitals.

  12. Clinical characteristics of triple negative breast cancer in Egyptian women: a hospital-based experience

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    Nivine Gado

    2016-06-01

    Full Text Available Purpose: Triple negative breast cancer (TNBC is an aggressive subtype of breast cancer with poor prognosis despite the high rates of response to chemotherapy. We aim to study the clinical features, factors influencing recurrence and survival outcomes of TNBC patients.Methods: We retrospectively studied the charts of patients with biopsy proven TNBC treated at The Clinical Oncology Department Ain-Shams University between 2009 and 2012.Results: One hundred and forty five patients fulfilled the eligibility criteria. The incidence of TNBC was 10.5% - 15% with a mean of 12% of all breast cancer patients. The follow-up duration ranged from six months to four years. The age range was 26 to 78 years. Infiltrating ductal carcinoma represented 93.1% of the pathologic types. 87% of patients were free of metastases (M0 at presentation. Clinical stages II and III represented 38 and 39.5% of the patients. 66% of patients had modified radical mastectomy. Following surgery, 77.5% of patients received adjuvant chemotherapy while 61% of the patients had adjuvant radiation therapy. Anthracyclines based chemotherapy was given to 52% of patients. Disease-free survival (DFS of the M0 patients at 20 and 30 months was 92% and 80% respectively. Relapse occurred in 23% of M0 patients. After a mean duration of DFS of 15.1 months, the most common sites of metastases for relapsed M0 patients were pulmonary (44.8%, bone (41.4%, and locoregional (13.8%. The median overall survival (ORS of patients was 18 months (1 - 45 months, whereas for the M1 group of patients the median ORS was 9 months (2 - 29 months.Conclusion: The incidence, pathological characteristics, and clinical behavior of TNBC were similar to what is mentioned in the literature. Adding taxanes to the chemotherapy protocols and using postoperative radiotherapy were both associated with a significant increase in the mean period of DFS, while did not significantly affect the ORS.

  13. Allergic contact dermatitis: pathophysiology applied to future therapy.

    Science.gov (United States)

    Li, Lily Y; Cruz, Ponciano D

    2004-01-01

    Contact dermatitis is a common reason for patient visits to primary-care clinics and represents up to 7% of all dermatologic consultations in the US. Substantial progress has been made in elucidating the pathophysiology of contact dermatitis, particularly the allergic form. A better understanding of pathologic mechanisms has led to improved management of cases and will continue to advance treatment modalities. The present paper reviews the pathogenesis and current treatment of allergic contact dermatitis and speculates on the prospects for improved future therapy.

  14. Differences in pathophysiology between rheumatoid arthritis and ankylosing spondylitis.

    Science.gov (United States)

    Lories, R J; Baeten, D L P

    2009-01-01

    Rheumatoid arthritis and ankylosing spondylitis are common and severe chronic inflammatory skeletal diseases. Recognizing the differences rather than emphasizing similarities is important for a better understanding of the disease processes, the identification of specific therapeutic targets and in the long-term better treatment options for the individual patients. We discuss a number of pathophysiological differences between rheumatoid arthritis and ankylosing spondylitis by looking at the anatomical characteristics, differences and similarities in the autoimmune and autoinflammatory reactions, association with other immune mediated inflammatory diseases, structural outcome, and their potential significance for further therapeutic developments. Further research into the differences between these diseases should focus on the specific nature of the immune/inflammatory components, the role of resident cells in the joint and joint-associated tissues, the types and mechanisms of tissue remodeling and the characteristics of the articular cartilage. Better insights into their individual characteristics may lead to better therapeutic strategies, specific targets and useful biomarkers.

  15. The pathophysiology of primary dystonia.

    Science.gov (United States)

    Berardelli, A; Rothwell, J C; Hallett, M; Thompson, P D; Manfredi, M; Marsden, C D

    1998-07-01

    Co-contraction and overflow of EMG activity of inappropriate muscles are typical features of all dystonic movements whether voluntary or involuntary. Voluntary movements are slow and more variable than normal, and there is particular difficulty switching between component movements of a complex task. Reduced spinal cord and brainstem inhibition is common to many reflex studies (long-latency reflexes, cranial reflexes and reciprocal inhibition). These reflex abnormalities may contribute to the difficulties in voluntary movements but cannot be causal as they can occur outside the clinically involved territory. Clinical and neurophysiological studies have emphasized the possible role of sensory feedback in the generation of dystonic movements. Abnormalities of cortical and basal ganglia function have been described in functional imaging and neurophysiological studies of patients with dystonia and in animal models of primary dystonia. Studies of cortical function have shown reduced preparatory activity in the EEG before the onset of voluntary movements, whilst magnetic brain stimulation has revealed changes in motor cortical excitability. Functional imaging of the brain in primary dystonia has suggested reduced pallidal inhibition of the thalamus with consequent overactivity of medial and prefrontal cortical areas and underactivity of the primary motor cortex during movements. These findings are supported by preliminary neuronal recordings from the globus pallidus and the thalamus at the time of stereotaxic surgery in patients with dystonia. All this evidence suggests that primary dystonia results from a functional disturbance of the basal ganglia, particularly in the striatal control of the globus pallidus (and substantia nigra pars reticulata). This causes altered thalamic control of cortical motor planning and executive areas, and abnormal regulation of brainstem and spinal cord inhibitory interneuronal mechanisms.

  16. Pathophysiological Mechanisms in Gaseous Therapies for Severe Malaria.

    Science.gov (United States)

    Kayano, Ana Carolina A V; Dos-Santos, João Conrado K; Bastos, Marcele F; Carvalho, Leonardo J; Aliberti, Júlio; Costa, Fabio T M

    2016-04-01

    Over 200 million people worldwide suffer from malaria every year, a disease that causes 584,000 deaths annually. In recent years, significant improvements have been achieved on the treatment of severe malaria, with intravenous artesunate proving superior to quinine. However, mortality remains high, at 8% in children and 15% in adults in clinical trials, and even worse in the case of cerebral malaria (18% and 30%, respectively). Moreover, some individuals who do not succumb to severe malaria present long-term cognitive deficits. These observations indicate that strategies focused only on parasite killing fail to prevent neurological complications and deaths associated with severe malaria, possibly because clinical complications are associated in part with a cerebrovascular dysfunction. Consequently, different adjunctive therapies aimed at modulating malaria pathophysiological processes are currently being tested. However, none of these therapies has shown unequivocal evidence in improving patient clinical status. Recently, key studies have shown that gaseous therapies based mainly on nitric oxide (NO), carbon monoxide (CO), and hyperbaric (pressurized) oxygen (HBO) alter vascular endothelium dysfunction and modulate the host immune response to infection. Considering gaseous administration as a promising adjunctive treatment against severe malaria cases, we review here the pathophysiological mechanisms and the immunological aspects of such therapies.

  17. Imaging Alzheimer's disease pathophysiology with PET

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    Lucas Porcello Schilling

    Full Text Available ABSTRACT Alzheimer's disease (AD has been reconceptualised as a dynamic pathophysiological process characterized by preclinical, mild cognitive impairment (MCI, and dementia stages. Positron emission tomography (PET associated with various molecular imaging agents reveals numerous aspects of dementia pathophysiology, such as brain amyloidosis, tau accumulation, neuroreceptor changes, metabolism abnormalities and neuroinflammation in dementia patients. In the context of a growing shift toward presymptomatic early diagnosis and disease-modifying interventions, PET molecular imaging agents provide an unprecedented means of quantifying the AD pathophysiological process, monitoring disease progression, ascertaining whether therapies engage their respective brain molecular targets, as well as quantifying pharmacological responses. In the present study, we highlight the most important contributions of PET in describing brain molecular abnormalities in AD.

  18. Current concepts in the pathophysiology of glaucoma

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    Agarwal Renu

    2009-01-01

    Full Text Available Glaucoma, the second leading cause of blindness, is characterized by changes in the optic disc and visual field defects. The elevated intraocular pressure was considered the prime factor responsible for the glaucomatous optic neuropathy involving death of retinal ganglion cells and their axons. Extensive investigations into the pathophysiology of glaucoma now reveal the role of multiple factors in the development of retinal ganglion cell death. A better understanding of the pathophysiological mechanisms involved in the onset and progression of glaucomatous optic neuropathy is crucial in the development of better therapeutic options. This review is an effort to summarize the current concepts in the pathophysiology of glaucoma so that newer therapeutic targets can be recognized. The literature available in the National Medical Library and online Pubmed search engine was used for literature review.

  19. Application of established pathophysiologic processes brings greater clarity to diagnosis and treatment of hyponatremia.

    Science.gov (United States)

    Maesaka, John K; Imbriano, Louis J; Miyawaki, Nobuyuki

    2017-03-06

    Hyponatremia, serum sodium water-restrict in SIAD and administer salt and water in RSW. Differentiating SIAD from RSW is extremely difficult because of identical clinical parameters that define both syndromes and the mindset that CSW occurs rarely. It is thus insufficient to make the diagnosis of SIAD simply because it meets the defined characteristics. We review the pathophysiology of SIAD and RSW, the evolution of an algorithm that is based on determinations of fractional excretion of urate and distinctive responses to saline infusions to differentiate SIAD from RSW. This algorithm also simplifies the diagnosis of hyponatremic patients due to Addison's disease, reset osmostat and prerenal states. It is a common perception that we cannot accurately assess the volume status of a patient by clinical criteria. Our algorithm eliminates the need to determine the volume status with the realization that too many factors affect plasma renin, aldosterone, atrial/brain natriuretic peptide or urine sodium concentration to be useful. Reports and increasing recognition of RSW occurring in patients without evidence of cerebral disease should thus elicit the need to consider RSW in a broader group of patients and to question any diagnosis of SIAD. Based on the accumulation of supporting data, we make the clinically important proposal to change CSW to RSW, to eliminate reset osmostat as type C SIAD and stress the need for a new definition of SIAD.

  20. Clinical and Molecular Characteristics of Non-Transfusion-Dependent Thalassemia in Kuwait.

    Science.gov (United States)

    Adekile, Adekunle D; Azab, Asma F; Al-Sharida, Sondus I; Al-Nafisi, Bahia A; Akbulut, Nagihan; Marouf, Rajaa A; Mustafa, Nada Y

    2015-01-01

    Although not regularly transfused, patients with non-transfusion-dependent thalassemia (NTDT) are prone to iron overload and its complications. Their molecular, phenotypical and laboratory characteristics vary in different populations and there is a need to document local prevailing patterns. We have reviewed the records of our patients with NTDT in Kuwait and documented their clinical and molecular characteristics in addition to iron status [serum ferritin and liver magnetic resonance imaging (MRI) T2*], management and complications. There were 41 patients, made up of 20 with β-thalassemia intermedia (β-TI), 18 with Hb H (β4) disease and three with Hb E (HBB: c.79G > A)-β-thalassemia (Hb E-β-thal); their ages ranged from 3 to 36 years (mean 12.5 ± 7.7). While 18 (43.9%) had been transfused at least once, only three (7.3%) had been transfused on multiple occasions. Three patients had serum ferritin >500 ng/mL; while four of 38 had mild or moderate liver iron overload. Seven (35.0%) of the β-TI patients were managed with hydroxyurea (HU) with good response. Other complications included five patients with gallstones and one each of hypothyroidism and moyamoya. The most common mutations among the β-TI patients were IVS-II-1 (G > A) and IVS-I-6 (T > C), while among the Hb H patients, the Saudi α2-globin gene polyadenylation (polyA) (AATAAA > AATAAG) mutation was responsible for all cases either as homozygotes (61.1%) or compound heterozygotes with the α-thal-2 (-α(3.7)) allele (33.3%). Although the pattern of NTDT in Kuwaiti patients is generally mild, there is a need to follow them to adulthood as the complications are cumulative and more prevalent in this group.

  1. NF1-mutated melanoma tumors harbor distinct clinical and biological characteristics.

    Science.gov (United States)

    Cirenajwis, Helena; Lauss, Martin; Ekedahl, Henrik; Törngren, Therese; Kvist, Anders; Saal, Lao H; Olsson, Håkan; Staaf, Johan; Carneiro, Ana; Ingvar, Christian; Harbst, Katja; Hayward, Nicholas K; Jönsson, Göran

    2017-03-07

    In general, melanoma can be considered as a UV-driven disease with an aggressive metastatic course and high mutational load, with only few tumors (acral, mucosal, and uveal melanomas) not induced by sunlight and possessing a lower mutational load. The most commonly activated pathway in melanoma is the mitogen-activated protein kinase (MAPK) pathway. However, the prognostic significance of mutational stratification is unclear and needs further investigation. Here, in silico we combined mutation data from 162 melanomas subjected to targeted deep sequencing with mutation data from three published studies. Tumors from 870 patients were grouped according to BRAF, RAS, NF1 mutation or triple-wild-type status and correlated with tumor and patient characteristics. We found that the NF1-mutated subtype had a higher mutational burden and strongest UV mutation signature. Searching for co-occurring mutated genes revealed the RASopathy genes PTPN11 and RASA2, as well as another RAS domain-containing gene RASSF2 enriched in the NF1 subtype after adjustment for mutational burden. We found that a larger proportion of the NF1-mutant tumors were from males and with older age at diagnosis. Importantly, we found an increased risk of death from melanoma (disease-specific survival, DSS; HR, 1.9; 95% CI, 1.21-3.10; P = 0.046) and poor overall survival (OS; HR, 2.0; 95% CI, 1.28-2.98; P = 0.01) in the NF1 subtype, which remained significant after adjustment for age, gender, and lesion type (DSS P = 0.03, OS P = 0.06, respectively). Melanoma genomic subtypes display different biological and clinical characteristics. The poor outcome observed in the NF1 subtype highlights the need for improved characterization of this group.

  2. Clinical Characteristics and Prognostic Analysis of 38 Patients 
with Pulmonary Sarcomatoid Carcinoma

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    Yuanyuan LI

    2015-09-01

    Full Text Available Background and objective Pulmonary sarcomatoid carcinoma is a rare histologic subtype of non-small cell lung cancer. The effective treatment for this disease has not well defined due to its extremely low morbidity. This study explores the clinicopathological characteristics and prognosis of 38 patients with PSC, so as to provide some clues for its diagnosis and treatment. Methods The study enrolled 38 patients with PSC that were diagnosed with histology or cytology in our hospital between January 2000 and December 2013. We retrospectively analyzed general clinical characteristics, smoking history, tumor size, TNM staging, pathology, immunohistochemistry, diagnostic method, treatment and prognosis. We used SPSS 19.0 statistical software and Kaplan-Meier method to analyze our data. Results Patients in this study were aged from 26 to 76 years old (the median age was 57.5 years old. Among all of them, the male to female ratio was 4:1, and 81.6% of patients had smoking history. Cough and hemoptysis were the most common primary symptoms. The median survival was 21 months, while one-year survival rate, three-year survival rate and five-year survival rate were 68.4%, 31.6% and 18.4% respectively. Tumor size, TNM staging, distant metastasis and surgery therapy were associated with the prognosis of patients. Conclusion Patients with PSC present with no special symptoms generally. According to our study, factors that affect patients’ prognosis include tumor size, TNM staging, distant metastasis and surgery. Complete resection is the key treatment for PSC patients, but comprehensive chemoradiotherapy needs further exploration in evidence-based medicine. Biological target therapy may give new insight into treatment for PSC.

  3. Chronic hepatitis virus infection in patients with multiple myeloma: clinical characteristics and outcomes

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    Chung-Jen Teng

    2011-01-01

    Full Text Available OBJECTIVES: Cytotoxic agents and steroids are used to treat lymphoid malignancies, but these compounds may exacerbate chronic viral hepatitis. For patients with multiple myeloma, the impact of preexisting hepatitis virus infection is unclear. The aim of this study is to explore the characteristics and outcomes of myeloma patients with chronic hepatitis virus infection. METHODS: From 2003 to 2008, 155 myeloma patients were examined to determine their chronic hepatitis virus infection statuses using serologic tests for the hepatitis B (HBV and C viruses (HCV. Clinical parameters and outcome variables were retrieved via a medical chart review. RESULTS: The estimated prevalences of chronic HBV and HCV infections were 11.0% (n = 17 and 9.0% (n = 14, respectively. The characteristics of patients who were hepatitis virus carriers and those who were not were similar. However, carrier patients had a higher prevalence of conventional cytogenetic abnormalities (64.3% vs. 25.0%. The cumulative incidences of grade 3-4 elevation of the level of alanine transaminase, 30.0% vs. 12.0%, and hyperbilirubinemia, 20.0% vs. 1.6%, were higher in carriers as well. In a Kaplan-Meier analysis, carrier patients had worse overall survival (median: 16.0 vs. 42.4 months. The prognostic value of carrier status was not statistically significant in the multivariate analysis, but an age of more than 65 years old, the presence of cytogenetic abnormalities, a beta-2-microglobulin level of more than 3.5 mg/L, and a serum creatinine level of more than 2 mg/ dL were independent factors associated with poor prognosis. CONCLUSION: Myeloma patients with chronic hepatitis virus infections might be a distinct subgroup, and close monitoring of hepatic adverse events should be mandatory.

  4. Falls Assessment Clinical Trial (FACT: design, interventions, recruitment strategies and participant characteristics

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    Lawton Beverley

    2007-07-01

    Full Text Available Abstract Background Guidelines recommend multifactorial intervention programmes to prevent falls in older adults but there are few randomised controlled trials in a real life health care setting. We describe the rationale, intervention, study design, recruitment strategies and baseline characteristics of participants in a randomised controlled trial of a multifactorial falls prevention programme in primary health care. Methods Participants are patients from 19 primary care practices in Hutt Valley, New Zealand aged 75 years and over who have fallen in the past year and live independently. Two recruitment strategies were used – waiting room screening and practice mail-out. Intervention participants receive a community based nurse assessment of falls and fracture risk factors, home hazards, referral to appropriate community interventions, and strength and balance exercise programme. Control participants receive usual care and social visits. Outcome measures include number of falls and injuries over 12 months, balance, strength, falls efficacy, activities of daily living, quality of life, and physical activity levels. Results 312 participants were recruited (69% women. Of those who had fallen, 58% of people screened in the practice waiting rooms and 40% when screened by practice letter were willing to participate. Characteristics of participants recruited using the two methods are similar (p > 0.05. Mean age of all participants was 81 years (SD 5. On average participants have 7 medical conditions, take 5.5 medications (29% on psychotropics with a median of 2 falls (interquartile range 1, 3 in the previous year. Conclusion The two recruitment strategies and the community based intervention delivery were feasible and successful, identifying a high risk group with multiple falls. Recruitment in the waiting room gave higher response rates but was less efficient than practice mail-out. Testing the effectiveness of an evidence based intervention in a

  5. PACAP and its receptors in migraine pathophysiology

    DEFF Research Database (Denmark)

    Edvinsson, Lars

    2014-01-01

    Pituitary adenylate cyclase-activating peptide (PACAP) and its receptors (PAC1 , VPAC1 and VPAC2 ) are present in sensory neurons and in vascular smooth muscle related to the trigeminovascular system, a key factor in migraine pain. Recent data point to an involvement of PACAP, and in particular...... the PAC1 receptor, in the pathophysiology of migraine. Available data are discussed in relation to a study by Walker in this issue of the Journal with the goal of identifying possibilities for the development of novel antagonists and to further define the role of PACAP in migraine pathophysiology...

  6. Persistent idiopathic facial pain - a prospective systematic study of clinical characteristics and neuroanatomical findings at 3.0 Tesla MRI

    DEFF Research Database (Denmark)

    Maarbjerg, Stine; Wolfram, Frauke; Heinskou, Tone Bruvik

    2017-01-01

    INTRODUCTION: Persistent idiopathic facial pain (PIFP) is a poorly understood chronic orofacial pain disorder and a differential diagnosis to trigeminal neuralgia. To address the lack of systematic studies in PIFP we here report clinical characteristics and neuroimaging findings in PIFP. METHODS.......565) and the odds ratio between neurovascular contact with displacement of the trigeminal nerve and the painful side was 0.2 (95% Cl 0.0-2.1, p = 0.195). CONCLUSION: PIFP is separated from trigeminal neuralgia both with respect to the clinical characteristics and neuroimaging findings, as NVC was not associated...

  7. Clinical characteristics and laboratory analyses of acute myeloid leukemia with t(16;21)(p11;q22)

    OpenAIRE

    Zhang, Zhifen; ZOU, JIANWEN; Li, Yuantang; Liu, Zhanfeng; Xu, Rui; TIAN, WENJUN; ZHAO, ZONGCHEN; Sun, Hui; Han, Jingying; Wang, Jia; Zhang, Bingchang; Ju, Ying

    2015-01-01

    The present study reviewed three patients with acute myeloid leukemia (AML) who had the specific genetic abnormality t(16;21)(p11;q22). To investigate the clinical and laboratory characteristics of AML with t(16;21)(p11;q22) translocation, the similarities and differences of clinical characteristics and laboratory examinations were compared, and a literature review was conducted. According to the French-American-British classification system, patient 1 was M4, patient 2 was M1 and patient 3 w...

  8. Rotavirus Gastroenteritis in a Neonatal Unit of a Greek Tertiary Hospital: Clinical Characteristics and Genotypes.

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    Dimitra Koukou

    Full Text Available Rotavirus (RV infection in neonatal age can be mild or even asymptomatic. Several studies have reported that RV is responsible for 31%-87% of pediatric nosocomial diarrhea and causes gastroenteritis outbreaks in pediatric and neonatal units.Study clinical characteristics, genotypes and risk factors of RV infection in neonatal age.A prospective study was conducted from April 2009 till April 2013 in the neonatal special care unit of the largest tertiary pediatric hospital of Greece. Fecal samples and epidemiological data were collected from each neonate with gastrointestinal symptoms. RV antigen was detected with a rapid immunochromatography test. RV positive samples were further genotyped with RT PCR and sequencing using specific VP7 and VP4 primers.Positive for RV were 126/415 samples (30.4%. Mean age of onset was 18 days. Seventy four cases (58% were hospital acquired. Seasonality of RV infection did not differ significantly throughout the year with the exception of 4 outbreaks. Genotypes found during the study period were G4P[8] (58.7%, G1P[8] (14.7%, G12P[8] (9.3%, G3P[8] (9.3%, G12P[6] (5.3%, G9P[8] (1.3% and G2P[4] (1.3%. RV cases presented with: diarrhea (81%, vomiting (26.2%, fever (34.9%, dehydration (28.6%, feeding intolerance (39.7%, weight loss (54%, whilst 19% of cases were asymptomatic. Comparing community with hospital acquired cases differences in clinical manifestations were found.Significant incidence of nosocomially transmitted RV infection in neonatal age including asymptomatic illness exists. Genotypes causing nosocomial outbreaks are not different from community strains. Circulating vaccines can be effective in prevention of nosocomial RV infection through herd immunity.

  9. Clinical characteristics and frequency of TLR4 polymorphisms in Brazilian patients with ankylosing spondylitis

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    Natalia Pereira Machado

    Full Text Available ABSTRACT Objectives: Innate immunity is involved in the physiopathology of ankylosing spondylitis (AS, with the participation of Gram-negative bacteria, modulation of human leukocyte antigen (HLA B27 and the involvement of pattern recognition receptors, such as Toll-like receptors (TLRs. The aim of this study was to investigate the clinical characteristics and frequency of TLR4 polymorphisms (Asp299Gly and Thr 399Ile in a cohort of Brazilian patients with AS. Methods: A cross-sectional study was carried out involving 200 patients with a diagnosis of AS and a healthy control group of 200 individuals. Disease activity, severity and functional capacity were measured. The study of TLR4 polymorphisms was performed using the restriction fragment length polymorphism method. HLA-B27 was analyzed by conventional polymerase chain reaction. The IBM SPSS Statistics 20 program was used for the statistical analysis, with p-values less than 0.05 considered significant. Results: Mean age and disease duration were 43.1 ± 12.7 and 16.6 ± 9.2 years, respectively. The sample was predominantly male (71% and non-Caucasian (52%. A total of 66% of the group of patients were positive for HLA-B27. The sample of patients was characterized by moderate functional impairment and a high degree of disease activity. No significant association was found between the two TLR4 polymorphisms and susceptibility to AS. Conclusions: TLR4 polymorphisms 399 and 299 were not more frequent in patients with AS in comparison to the health controls and none of the clinical variables were associated with these polymorphisms.

  10. Clinical Characteristic and Outcome of Acute Lower Respiratory Tract Infection in Children with Congenital Heart Disease

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    Krystle Gabriela

    2015-09-01

    Full Text Available Background: Acute Lower Respiratory Tract Infection (ALRTI is the leading cause of deaths in children under 5 years of age worldwide, and has high morbidity and mortality in children with Congenital Heart Disease (CHD. The objective of this study was to obtain the incidence, clinical characteristic, and outcome of ALRTI children with CHD. Methods: A retrospective hospital-based study was conducted from January 2007–December 2011 to medical record of child patients with ALRTI and CHD in the Department of Child Health of Dr. Hasan Sadikin General Hospital, Bandung. The diagnosis of CHD was determined by echocardiography. The collected data was analyzed and presented in percentage shown in tables. Results : From 3,897 children who had ALRTI, there were 149 children with CHD (3.8%, with 11.4% of whom founded with recurrent episodes. This happened often in girls than boys with quite similar ratio of 1.37: 1.The majority of children (80% was under 1 year old of age, 72.5% with malnutrition, and 24.8% with severe malnutrition. Clinical symptoms mostly found were difficulty of breathing (98%, fever (85.2%, cough (75.2%, and runny nose (63.1%. The most common types of CHD were Patent Ductus Arteriosus (47.6%, followed by Ventricular Septal Defect (47%. Bronchopneumonia (86.6% was the common type of ALRTI. The length of stay was mostly less than 10 days (70.5%. From all the children 43.7% had complications, and 6.7% died. Conclusions: The ALRTI in children with CHD is not common and has good outcome. The majority for CHD lesions are Patent Ductus Arteriosus and Ventricular Septal Defect while for ALRTI is Bronchopneumonia.

  11. Correlation between serum adiponectin and clinical characteristics, biochemical parameters in Indian women with polycystic ovary syndrome

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    Ramanand, Sunita J.; Ramanand, Jaiprakash B.; Ghongane, Balasaheb B.; Patwardhan, Milind H.; Patwardhan, Varsha M.; Ghanghas, Ravi; Halasawadekar, Nimish R.; Patil, Praveenkumar

    2014-01-01

    Background: Polycystic ovary syndrome (PCOS) is a common disorder. PCOS women are at a high risk for insulin resistance and metabolic syndrome (MS). Adiponectin is positively related to insulin sensitivity. It has a preventive role in atherogenesis and MS. The present work was conducted to study the correlation between serum adiponectin levels and clinical characteristics and biochemical parameters in PCOS patients. Materials and Methods: A prospective study in 49 newly diagnosed (as per Rotterdam criteria) Indian PCOS women was conducted. PCOS women were clinically examined and investigated for biochemical parameters. Results: The mean serum adiponectin was 12 ± 9.4 μg/mL (range 0.47-45). Hypoadiponectinemia (serum adiponectin <4 μg/mL) was present in 22% patients. Age and adiponectin correlated significantly and inversely (r = −0.42, P = 0.027). Overweight/obese patients had lower mean adiponectin levels than normal weight (11.62 ± 9.5 vs 13.58 ± 9.5, P = 0.56). It was significantly lower in patients with acanthosis nigricans (AN) as compared with those without AN (8.4 ± 5.9 vs 15 ± 11, P = 0.038). Hirsute patients showed lower mean adiponectin levels than nonhirsute (10 ± 7.3 vs 13 ± 10, P = 0.57). A positive, insignificant correlation was observed between serum adiponectin and cholesterol, low-density lipoprotein, follicle stimulating hormone (FSH), thyroid stimulating hormone, levels. A negative insignificant correlation existed between serum adiponectin and luteinizing hormone (LH), LH: FSH ratio, prolactin, dehydroepiandrosterone, testosterone, triglyceride, high-density lipoprotein, fasting blood glucose, fasting insulin, and Homeostasis Model Assessment. Conclusion: Hypoadiponectinemia is present in one-fifth of women with PCOS. Adiponectin levels decrease as age advances. Low levels of adiponectin possibly contributes to the development of dermal manifestation (AN) of insulin resistance. PMID:24741521

  12. Serum lipid profile and clinical characteristics of patients with xanthelasma palpebrarum*

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    Kavoussi, Hossein; Ebrahimi, Ali; Rezaei, Mansour; Ramezani, Mazaher; Najafi, Behnaz; Kavoussi, Reza

    2016-01-01

    Background Although many factors are involved in the etiology of xanthelasma palpebrum, lipid disorder is strongly associated with its induction. Xanthelasma palpebrum, the most common type of xanthoma, usually presents in middle-aged females and results in aesthetic problems. Objective To evaluate thelipid profile and important clinical aspects of xanthelasma palpebrum patients. Methods In this descriptive study, we enrolled 42xanthelasma palpebrumpatients, and 42 cases of non-inflammatory skin disorders as thecontrol group, matched for age and gender.The clinical characteristics of the patients and fasting serum lipid profile were recorded for both groups. The data obtained were analyzed using SPSS-16. Results Xanthelasma palpebrum was found more commonly in middle-aged females with disease onset of less than 1 year, and without significant familial history of xanthoma. Furthermore,xanthelasma lesionswere most often seen in the upper lid with mild extension and was rarely associated with systemic disease. There was no statistically significant difference between two groups regarding hypertriglyceridemia (p= 0.231) and hypercholesterolemia (p= 0.302). The mean serum levels of cholesterol (221.51±60.4 mg/dl), triglyceride (185.98±71.1 mg/dl) and VLDL (37.7±17.6 mg/dl) were significantly higher and themedian HDL (36.2 (31, 41) mg/dl) level was lower in thepatient group. Conclusion In our study, hypercholesterolemia and hypertriglyceridemia did not reveal a significant difference between thepatient and control groups; however, mean serum values for cholesterol, triglyceride, VLDL and HDL showed a significant difference between the two groups. Therefore, in addition to lipid abnormality, other factors could be involved in the pathogenesis of xanthelasma palpebrum. PMID:27579742

  13. Staphylococcus aureus Clinical Isolates: Antibiotic Susceptibility, Molecular Characteristics, and Ability to Form Biofilm

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    N. Indrawattana

    2013-01-01

    Full Text Available Periodic monitoring of Staphylococcus aureus characteristics in a locality is imperative as their drug-resistant variants cause treatment problem. In this study, antibiograms, prevalence of toxin genes (sea-see, seg-ser, seu, tsst-1, eta, etb, and etd, PFGE types, accessory gene regulator (agr groups, and ability to form biofilm of 92 S. aureus Thailand clinical isolates were investigated. They were classified into 10 drug groups: groups 1–7 (56 isolates were methicillin resistant (MRSA and 8–10 (36 isolates were methicillin sensitive (MSSA. One isolate did not have any toxin gene, 4 isolates carried one toxin gene (seq, and 87 isolates had two or more toxin genes. No isolate had see, etb, or tsst-1; six isolates had eta or etd. Combined seg-sei-sem-sen-seo of the highly prevalent egc locus was 26.1%. The seb, sec, sel, seu, and eta associated significantly with MSSA; sek was more in MRSA. The sek-seq association was 52.17% while combined sed-sej was not found. Twenty-three PFGE types were revealed, no association of toxin genes with PFGE types. All four agr groups were present; agr group 1 was predominant (58.70% but agr group 2 strains carried more toxin genes and were more frequent toxin producers. Biofilm formation was found in 72.83% of the isolates but there was no association with antibiograms. This study provides insight information on molecular and phenotypic markers of Thailand S. aureus clinical isolates which should be useful for future active surveillance that aimed to control a spread of existing antimicrobial resistant bacteria and early recognition of a newly emerged variant.

  14. Clinical characteristics of renal cancer in Malaysia : a ten year review.

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    Singam, Praveen; Ho, Christopher; Hong, Goh Eng; Mohd, Azrif; Tamil, Azmi Md; Cheok, Lee Boon; Zainuddin, Zulkifli

    2010-01-01

    Renal cancer is rare and its incidence is 1.9 per 100,000 in the Malaysian population, which consists of three major ethnic groups (Malay, Chinese and Indians). A retrospective study was her conducted to identify clinical characteristics and ethnic background influences on presentation. The study included all renal cancer patients from a single medical institution over ten years, with a total of 75 cases. Seventy-three patients underwent surgery while 2 received only radiotherapy or chemotherapy. The male to female ratio was 2.75:1. Incidence was equal among the Malay (49.3%) and Chinese ethnic groups (45.3%). Mean age of patients were 57.1 (18-93) years old. There were 26 (37.4%) patients with Stage I disease, 14 (18.7%) at Stage II, 23 (30.7%) at Stage III and 12 (16%) at Stage IV. The Chinese race presented at mean older age (p= 0.02) and later stage of disease (p= 0.046). Patients above 40 years old had more advanced stage disease (p= 0.023). Tumour histology were clear cell (72%), urothelial cell (13.3%), sarcomatoid cell and nephroblastoma each contributed 2.7%. The mean tumour size was 8.1 (2-20) cm. There was substantial agreement between the pre and post operative staging (kappa 0.691). In conclusion we observed significant influences of age and race in the clinical presentation of renal cancer in our institution based population. There was larger male to female ratio and mean tumour size as compared to previous epidemiology studies.