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Sample records for syndrome requiring intensive

  1. [Intensive care of delirium syndromes].

    Science.gov (United States)

    Zielmann, S; Petrow, H; Walther, P; Henze, Th

    2003-01-01

    Delirium is mental dysfunctions occurring as impaired attentional and memory systems with disturbances of consciousness, affectivity, psychomotor activity and sleep patterns. Numerous factors and underlying diseases may be responsible for these non-specific symptoms. Therefore, a thorough evaluation of preadmission history and current clinical status, supplemented by laboratory and extended technical diagnostic procedures, are always required. If delirium occurs in connection with emergency admission to hospital, an organic disease can most regularly be found. Due to its rapid time of onset and minor side-effects, the intravenous injection of 2.0 g gamma-hydroxybutyric acid is preferred for sedation of extremely agitated patients. Neuroleptic drugs are indicated in psychiatric patients. A central anticholinergic syndrome in the early postoperative period causative of the symptoms of delirium may respond to intravenous injection of physostigmine. Most of the time, however, these acute disturbances of brain function are best treated by correction of homeostatic imbalances, restoration of cardiovascular and respiratory stability and alleviation of pain. Postoperative delirium occurring two or more days later is frequently due to respiratory distress, followed by sepsis, alcohol withdrawal and many other causes including heart failure, exsiccosis and side-effects of drugs. In intensive care patients, delirium may be caused, for example, by withdrawal (alcohol, opioids, benzodiazepines), the onset of sepsis (often venous catheter related), side-effects of drugs, problems of communication, sleep deprivation and others. Treatment should focus on finding the right approach. Personal care should be intensified and include help from family members. Most problems arise from agitated, non-cooperative patients. Treatment with clonidine, gamma-hydroxybutyric acid or neuroleptic drugs like perazin and haloperidol may be required to reduce agitation and the activation of

  2. Severe ovarian hyperstimulation syndrome: Intensive care management of two cases

    Directory of Open Access Journals (Sweden)

    Praveen Talawar

    2011-01-01

    Full Text Available Severe ovarian hyperstimulation syndrome (OHSS is characterized by increased capillary permeability and fluid retention in the third space. It is generally a complication of assisted reproduction therapy (ART with exogenous gonadotropins, but cases with natural onset of OHSS have been reported. The massive extravascular exudation can cause tense ascites, pleural and pericardial effusion, hypovolemic shock, oliguria, electrolyte imbalance (hyponatremia and hyperkalemia, and hemoconcentration, with a tendency for hypercoagulability and risk of life-threatening thromboembolic complications. The patient can rarely develop multi-organ failure (adult respiratory distress syndrome, renal failure and death. With increasing use of ART, this syndrome may be seen more frequently in the intensive care unit (ICU, requiring multidisciplinary care. We report the management of two cases of severe OHSS, which required admission to the ICU in our hospital.

  3. [Burnout syndrome in different intensive care units].

    Science.gov (United States)

    Frade Mera, M J; Vinagre Gaspar, R; Zaragoza García, I; Viñas Sánchez, S; Antúnez Melero, E; Alvarez González, S; Malpartida Martín, P

    2009-01-01

    To determine the prevalence of the professional burnout syndrome in health care personnel of different Intensive Care Units (ICUs). To know the association between burnout, its dimensions and sociodemographic-laboral variables. To compare the dimensions of burnout, characteristics of the personnel and of the patients of the different ICUs. Analytic, comparative, cross-sectional study performed in the ICU of a tertiary hospital in November 2006 performed in a sample of 289 professionals. The Maslach Burnout Inventory questionnaire and sociodemographic-laboral variables were provided. The following were evaluated in the ICUs: Therapeutic Intervention Scoring System (TISS), Nine Equivalents of Nursing Manpower Use Score (NEMS), mortality, stay, isolations and travel of third parties. The chi2 test, Fischer test, Kruskall-Wallis test and multivariate logistic regression analysis were used. A total of 73% of the workers answered. Ages ranged from 37 +/- 9 and 81% were women. The prevalence of burnout was 14%, this affecting 16% of the nurses, 14% of residents, 13% physicians and 10% auxiliary workers. Burnout was associated to low professional satisfactions, relationship with regular colleagues, low work recognition and time worked and experience in the ICU to high emotional tiredness, with a p burnout syndrome 17%, elevated emotional tiredness 49%, elevated depersonalization 63% and low professional performance 44%. The prevalence of the burnout syndrome in our sample was 14%, those being affected most being the nursing professionals. We detected elevated levels of depersonalization and middle levels of emotional tiredness and professional performance. The variables related with professional burnout syndrome were low professional satisfaction, relationship with regular colleagues, low work recognition, and elevated emotional tiredness in the more expert personnel. The ICU with the greatest prevalence of burnout during the month studied attended patients with greater

  4. Radiation doses to neonates requiring intensive care

    International Nuclear Information System (INIS)

    Robinson, A.; Dellagrammaticas, H.D.

    1983-01-01

    Radiological investigations have become accepted as an important part of the range of facilities required to support severely ill newborn babies. Since the infants are so small, many of the examinations are virtually ''whole-body'' irradiations and it was thought that the total doses received might be appreciable. A group of such babies admitted to the Neonatal Intensive Care Unit in Sheffield over a six-month period have been studied. X-ray exposure factors used for each examination have been noted and total skin, gonad and bone marrow doses calculated, supplemented by measurements on phantoms. It is concluded that in most cases doses received are of the same order as those received over the same period from natural background radiation and probably less than those received from prenatal obstetric radiography, so that the additional risks from the diagnostic exposure are small. The highest doses are received in CT scans and barium examinations and it is recommended that the need for these should be carefully considered. (author)

  5. Shoulder impingement syndrome in relation to shoulder intensive work.

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    Frost, P; Andersen, J H

    1999-07-01

    To analyse the risk of shoulder impingement syndrome relative to shoulder intensive work. A cross sectional study of a historical cohort of 1591 workers employed between 1986 and 1993 at a slaughterhouse or a chemical factory. Workers not doing tasks in slaughtering or meat processing constituted the reference group. Intensity of shoulder work in meat processing tasks was assessed by video based observations. Information on shoulder disorders was collected by questionnaire and by physical examinations. Impingement syndrome was diagnosed when shoulder symptoms had been present for at least 3 months during the past year and there were signs of subacromial impingement in the corresponding shoulder at physical examination. Shoulder function was assessed at the same occasion with the Constant scoring technique. Prevalence of shoulder impingement syndrome was analysed according to job title and cumulative exposure. Prevalence ratio for shoulder impingement syndrome was 5.27 (95% confidence interval (95% CI), 2.09 to 12.26) among currently working and 7.90 (95% CI, 2.94 to 21.18) among former slaughterhouse workers. Transformed model based prevalence ratios according to years in slaughterhouse work showed an overall association between cumulative exposure and risk for shoulder impingement syndrome. This study supports the hypothesis that shoulder intensive work is a risk factor for impingement syndrome of the shoulder. Despite the historical cohort design healthy worker selection may have influenced the exposure-response relation found.

  6. Nursing staff requirements for neonatal intensive care.

    OpenAIRE

    Williams, S; Whelan, A; Weindling, A M; Cooke, R W

    1993-01-01

    A study to estimate the number of nursing staff required for neonatal nursing was undertaken. Certain nursing tasks, such as transporting any infant, caring for the dying infant, and looking after the very unstable infant required continuous attention by one nurse (5.5 whole time equivalent (wte) nurses for each cot). The stable ventilated infant required 10.5 nursing hours each day-that is, 2.4 wte/cot. Infants with intravenous infusions, but not ventilated, required only slightly less nursi...

  7. Shoulder impingement syndrome in relation to shoulder intensive work

    OpenAIRE

    Frost, P.; Andersen, J. H.

    1999-01-01

    OBJECTIVES: To analyse the risk of shoulder impingement syndrome relative to shoulder intensive work. METHODS: A cross sectional study of a historical cohort of 1591 workers employed between 1986 and 1993 at a slaughterhouse or a chemical factory. Workers not doing tasks in slaughtering or meat processing constituted the reference group. Intensity of shoulder work in meat processing tasks was assessed by video based observations. Information on shoulder disorders was collected by quest...

  8. Intensive Nutrition Management in a Patient with Short Bowel Syndrome Who Underwent Bariatric Surgery

    OpenAIRE

    Kweon, MeeRa; Ju, Dal Lae; Park, Misun; Choe, JiHyeong; Suh, Yun-Suhk; Seol, Eun-Mi; Lee, Hyuk-Joon

    2017-01-01

    Many individuals with short bowel syndrome (SBS) require long-term parenteral nutrition (PN) to maintain adequate nutritional status. Herein, we report a successful intestinal adaptation of a patient with SBS through 13 times intensive nutritional support team (NST) managements. A thirty-five-year-old woman who could not eat due to intestinal discontinuity visited Seoul National University Hospital for reconstruction of the bowel. She received laparoscopic Roux-en-Y gastric bypass (RYGB) due ...

  9. Intense neutron source requirements for fusion reactor materials development

    International Nuclear Information System (INIS)

    Ishino, Shiori

    1989-01-01

    Materials research should precede machine construction by at least ten years because considerable time is required for the materials development. When the next generation machine is under discussion, materials scientists and engineers should consider next-next generation device as DEMO for establishing the materials database in time. In this sense, development of an intense high energy neutron source is an urgent problem. Characteristic features of radiation effects with 14 MeV neutrons will be briefly reviewed. Then, the reasons why we need intense source will be discussed. These discussions will lead to identify requirements for the intense neutron sources. There are both near term and long term materials issues which can be studied with such intense neutron sources depending on their capacity. One should also recognize that development of such an intense source will require considerable time and maximum use of existing intense fission reactor neutrons will be one of the practical options for the moment. In other words, the intense neutron sources under discussion should be superior for the study of fusion radiation effects than the existing fission reactors. Items are listed for the evaluation of the sources and some critical comments will be made on several kinds of sources currently being proposed. (author)

  10. Does Horner's syndrome in infancy require investigation?

    Science.gov (United States)

    George, N; Gonzalez, G; Hoyt, C

    1998-01-01

    AIMS—To evaluate whether isolated Horner's syndrome presenting in the first year of life warrants investigation.
METHOD—Retrospective review of 23 children presenting with Horner's syndrome in the first year of life.
RESULTS—In 16 patients (70%) no cause was identified. Birth trauma was the most common identifiable cause (four patients). Twenty one children (91%) had urinary vanillylmandelic acid (VMA) measured and 13 patients (57%) underwent either computed tomography or magnetic resonance imaging of the chest and neck. These investigations revealed previously undisclosed pathology in only two—one ganglioneuroma of the left pulmonary apex and one cervical neuroblastoma. A further patient was known to have abdominal neuroblastoma before presenting with Horner's syndrome. There were no cases of Horner's syndrome occurring after cardiothoracic surgery. Long term follow up of the patients (mean 9.3 years) has not revealed further pathology.
CONCLUSIONS—Routine diagnostic imaging of isolated Horner's syndrome in infancy is unnecessary. Infants should be examined for cervical or abdominal masses and involvement of other cranial nerves. If the Horner's syndrome is truly isolated then urinary VMA levels and follow up in conjunction with a paediatrician should detect any cases associated with neuroblastoma. Further investigation is warranted if the Horner's syndrome is acquired or associated with other signs such as increasing heterochromia, a cervical mass, or cranial nerve palsies.

 Keywords: Horner's syndrome; neuroblastoma PMID:9536881

  11. Intensive treatment of dysarthria in two adults with Down syndrome.

    Science.gov (United States)

    Mahler, Leslie A; Jones, Harrison N

    2012-01-01

    This study investigated the impact of an established behavioural dysarthria treatment on acoustic and perceptual measures of speech in two adults with Down syndrome (DS) and dysarthria to obtain preliminary measures of treatment effect, effect size and treatment feasibility. A single-subject A-B-A experimental design was used to measure the effects of the Lee Silverman Voice treatment (LSVT®) on speech in two adults with DS and dysarthria. Dependent measures included vocal sound pressure level (dB SPL), phonatory stability and listener intelligibility scores. Statistically significant improvements (p dysarthria can respond positively to intensive speech treatment such as LSVT. Further investigations are needed to develop speech treatments specific to DS.

  12. Exercise Intensity and Incidence of Metabolic Syndrome: The SUN Project.

    Science.gov (United States)

    Hidalgo-Santamaria, María; Fernandez-Montero, Alejandro; Martinez-Gonzalez, Miguel A; Moreno-Galarraga, Laura; Sanchez-Villegas, Almudena; Barrio-Lopez, María T; Bes-Rastrollo, Maira

    2017-04-01

    Emerging evidence suggests that vigorous physical activity may be associated with higher cardioprotective benefits than moderate physical activity. This study aimed to assess the long-term relationship between the intensity of leisure time physical activity (LTPA) and the risk of developing metabolic syndrome (MS) in a prospective cohort study. The Seguimiento Universidad de Navarra (SUN) Project comprises Spanish university graduates. Participants (n=10,145) initially free of MS were followed for a minimum of 6 years (2008-2014). Analysis was conducted in 2015. Physical activity was assessed though a validated questionnaire. The intensity of each physical activity was measured in METs. The intensity of LTPA was estimated by the ratio between total METs/week and total hours of LTPA/week, obtaining the mean METs/hour of LTPA. MS was defined according to the harmonizing definition. The association between the intensity of LTPA (METs/hour) and MS was assessed with logistic regression models adjusting for potential confounders. Among 10,145 participants initially free of any MS criteria, 412 new MS cases were observed. Vigorous LTPA was associated with a 37% relatively lower risk (AOR=0.63, 95% CI=0.44, 0.89) compared with light LTPA. For a given total energy expenditure, independent of the time spent on it, participants who performed vigorous LTPA exhibited a higher reduction in the risk of MS than those who performed light to moderate LTPA. Vigorous LTPA was significantly associated with lower risk of developing MS after a 6-year follow-up period. Copyright © 2016 American Journal of Preventive Medicine. Published by Elsevier Inc. All rights reserved.

  13. The intense world syndrome - an alternative hypothesis for autism

    Directory of Open Access Journals (Sweden)

    Henry Markram

    2007-10-01

    Full Text Available Autism is a devastating neurodevelopmental disorder with a polygenetic predisposition that seems to be triggered by multiple environmental factors during embryonic and/or early postnatal life. While significant advances have been made in identifying the neuronal structures and cells affected, a unifying theory that could explain the manifold autistic symptoms has still not emerged. Based on recent synaptic, cellular, molecular, microcircuit, and behavioral results obtained with the valproic acid (VPA rat model of autism, we propose here a unifying hypothesis where the core pathology of the autistic brain is hyper-reactivity and hyper-plasticity of local neuronal circuits. Such excessive neuronal processing in circumscribed circuits is suggested to lead to hyper-perception, hyper-attention, and hyper-memory, which may lie at the heart of most autistic symptoms. In this view, the autistic spectrum are disorders of hyper-functionality, which turns debilitating, as opposed to disorders of hypo-functionality, as is often assumed. We discuss how excessive neuronal processing may render the world painfully intense when the neocortex is affected and even aversive when the amygdala is affected, leading to social and environmental withdrawal. Excessive neuronal learning is also hypothesized to rapidly lock down the individual into a small repertoire of secure behavioral routines that are obsessively repeated. We further discuss the key autistic neuropathologies and several of the main theories of autism and re-interpret them in the light of the hypothesized Intense World Syndrome.

  14. Post-Operative Intensive Care Unit Requirements Following Elective Craniotomy

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    HANAK, BRIAN W.; WALCOTT, BRIAN P.; NAHED, BRIAN V.; MUZIKANSKY, ALONA; MIAN, MATTHEW K.; KIMBERLY, WILLIAM T.; CURRY, WILLIAM T.

    2012-01-01

    Objective Commonly, patients undergoing craniotomy are admitted to an intensive care setting post-operatively to allow for close monitoring. We aim to determine the frequency with which patients who have undergone elective craniotomies require intensive care unit level interventions or experience significant complications during the post-operative period to identify a subset of patients for whom an alternative to ICU level care may be appropriate. Methods Following Institutional Review Board approval, a prospective, consecutive cohort of adult patients undergoing elective craniotomy was established at the Massachusetts General Hospital between the dates of April 2010 and March 2011. Inclusion criteria were intradural operations requiring craniotomy performed on adults (18 years of age or greater). Exclusion criteria were cases of an urgent or emergent nature, patients who remained intubated post-operatively, and patients who had a ventriculostomy drain in place at the conclusion of the case. Results 400 patients were analyzed. Univariate analysis revealed that diabetics (p = 0.00047), patients who required intra-operative blood product administration (p = 0.032), older patients (p craniotomy. Properly selected patients may not require post-craniotomy ICU monitoring. Further study of resource utilization is necessary to validate these preliminary findings, particularly in different hospital types. PMID:23182731

  15. Cauda equina syndrome: A rare complication in intensive care

    Directory of Open Access Journals (Sweden)

    Jagatsinh Yogendrasinh

    2009-01-01

    Full Text Available A 73-year-old married retired woman with a history of myocardial infarction and primary biliary cirrhosis was admitted to intensive care unit with complaints of chest pain. She was suspected to have pulmonary embolism (PE and was treated with low-molecular-weight heparin (LMWH and aspirin. She had computerized tomographic pulmonary angiography on next day, which ruled out any evidence of PE, until she was continued on LMWH. Three days later, she developed progressive right leg weakness and loss of sphincter control and patchy loss of sensation from T10 and below. She was seen by neurologist and had an MRI scan, which showed extensive subdural clot compressing the conus and lower half of the thoracic cord. She underwent T9-L1, L3, L5-S1 laminectomies, and evacuation and decompression of the clot. She showed very slight recovery following the surgery and left with residual paraparesis. This case is reported to raise awareness among intensivists to be cautious in establishing the diagnosis before prescribing the LMWH and be vigilant to diagnose cauda equina syndrome and treat promptly to avoid residual neurological problems.

  16. Experience with Guillain-Barré syndrome in a neurological Intensive Care Unit.

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    González, P; García, X; Guerra, A; Arango, J C; Delgado, H; Uribe, C S; Sará, J; López de Mesa, J C; Hernández, O

    2016-01-01

    Guillain-Barré syndrome, an acute polyradiculoneuropathy that presents with weakness and areflexia, is the most common cause of acute flaccid paralysis. In certain patients, respiratory failure is secondary to this disorder, eventually causing patients to require mechanical ventilation and experience additional complications due to diminished respiratory support and related mobility limitations. Prognoses for most of these cases are positive; treatment consists of basic support combined with plasmapheresis or administration of immunoglobulins. This study sought to describe the socio-demographic, clinical, laboratory and neurophysiological characteristics of patients with Guillain-Barré syndrome who were hospitalised in the Intensive Care Unit of the Neurological Institute of Colombia between 2006 and 2012. This study presents a case series. We surveyed 25 patients (32% female and 68% male) with Guillain-Barré syndrome and an average age of 54 years. Sixty per cent of these patients were admitted between days 3 and 7 after symptom onset; 64% had a history of respiratory infection and 20% had a history of intestinal infection. In addition, 84% of the patients presented with albuminocytological dissociation. We observed the following clinical subtypes of Guillain-Barré syndrome: inflammatory demyelinating polyneuropathy in 32%, acute motor-sensory axonal neuropathy in 28%, acute motor axonal neuropathy in 28%, and Miller Fisher syndrome in 12%. In this descriptive study of a group of critical care patients with GBS, results depended on patients' clinical severity at time of admission. Our findings are similar to results published in the international literature. Copyright © 2014 Sociedad Española de Neurología. Publicado por Elsevier España, S.L.U. All rights reserved.

  17. Recognition of Facial Expressions and Prosodic Cues with Graded Emotional Intensities in Adults with Asperger Syndrome

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    Doi, Hirokazu; Fujisawa, Takashi X.; Kanai, Chieko; Ohta, Haruhisa; Yokoi, Hideki; Iwanami, Akira; Kato, Nobumasa; Shinohara, Kazuyuki

    2013-01-01

    This study investigated the ability of adults with Asperger syndrome to recognize emotional categories of facial expressions and emotional prosodies with graded emotional intensities. The individuals with Asperger syndrome showed poorer recognition performance for angry and sad expressions from both facial and vocal information. The group…

  18. Myasthenic crisis patients who require intensive care unit management.

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    Sakaguchi, Hideya; Yamashita, Satoshi; Hirano, Teruyuki; Nakajima, Makoto; Kimura, En; Maeda, Yasushi; Uchino, Makoto

    2012-09-01

    The purpose of this report was to investigate predictive factors that necessitate intensive care in myasthenic crisis (MC). We retrospectively reviewed MC patients at our institution and compared ICU and ward management groups. Higher MG-ADL scale scores, non-ocular initial symptoms, infection-triggered findings, and higher MGFA classification were observed more frequently in the ICU group. In patients with these prognostic factors, better outcomes may be obtained with early institution of intensive care. Copyright © 2012 Wiley Periodicals, Inc.

  19. Intensive Care Physiotherapy during Extracorporeal Membrane Oxygenation for Acute Respiratory Distress Syndrome.

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    Munshi, Laveena; Kobayashi, Tadahiro; DeBacker, Julian; Doobay, Ravi; Telesnicki, Teagan; Lo, Vincent; Cote, Nathalie; Cypel, Marcelo; Keshavjee, Shaf; Ferguson, Niall D; Fan, Eddy

    2017-02-01

    There are limited data on physiotherapy during extracorporeal membrane oxygenation (ECMO) for acute respiratory distress syndrome (ARDS). We sought to characterize physiotherapy delivered to patients with ARDS supported with ECMO, as well as to evaluate the association of this therapeutic modality with mortality. We conducted a retrospective cohort study of all adult patients with ARDS supported with ECMO at our institution between 2010 and 2015. The highest level of daily activity while on ECMO was coded using the ICU Mobility Scale. Through multivariable logistic regression, we evaluated the association between intensive care unit (ICU) physiotherapy and ICU mortality. In an exploratory univariate analysis, we also evaluated factors associated with a higher intensity of ICU rehabilitation while on ECMO. Of 107 patients who underwent ECMO, 61 (57%) had ARDS requiring venovenous ECMO. The ICU physiotherapy team was consulted for 82% (n = 50) of patients. Thirty-nine percent (n = 18) of these patients achieved an activity level of 2 or higher (active exercises in bed), and 17% (n = 8) achieved an activity level 4 or higher (actively sitting over the side of the bed). In an exploratory analysis, consultation with the ICU physiotherapy team was associated with decreased ICU mortality (odds ratio, 0.19; 95% confidence interval, 0.04-0.98). In univariate analysis, severity-of-illness factors differentiated higher-intensity and lower-intensity physiotherapy. Physiotherapy during ECMO is feasible and safe when performed by an experienced team and executed in stages. Although our study suggests an association with improved ICU mortality, future research is needed to identify potential barriers, optimal timing, dosage, and safety profile.

  20. [Professional Burnout Syndrome of intensive care physicians from Salvador, Bahia, Brazil].

    Science.gov (United States)

    Tironi, Márcia Oliveira Staffa; Nascimento Sobrinho, Carlito Lopes; Barros, Dalton de Souza; Reis, Eduardo José Farias Borges; Marques Filho, Edson Silva; Almeida, Alessandro; Bitencourt, Almir; Feitosa, Ana Isabela Ramos; Neves, Flávia Serra; Mota, Igor Carlos Cunha; França, Juliana; Borges, Lorena Guimarães; Lordão, Manuela Barreto de Jesus; Trindade, Maria Valverde; Teles, Marcelo Santos; Almeida, Mônica Bastos T; Souza, Ygor Gomes de

    2009-01-01

    Describe prevalence of the Burnout syndrome in intensive care physicians of Salvador, associated to demographic data and aspects of the work environment (psychological demand and job control). This cross sectional study has investigated the association between work conditions and Burnout Syndrome in a population of 297 Intensive Care Physicians from Salvador, Bahia, Brazil. An individual, self-report questionnaire evaluated the physicians' psychological aspects of work, using the demand-control model (Job Content Questionnaire) and their mental health, using the Maslash Burnout Inventory (MBI). The study found work overload,a high proportion of on duty physicians and low income for the hours worked. Prevalence of the Burnout Syndrome was 7.4% and it was more closely associated with aspects of the job's psychological demand than with its control. Physicians under great stress (high demand and low control) presented prevalence of the Bornout Syndrome 10.2 times higher than those under low stress (low demand and high control) jobs.

  1. Lemierre's Syndrome - A rare cause of disseminated sepsis requiring multi-organ support.

    Science.gov (United States)

    Rae, John; Misselbrook, Katie

    2017-11-01

    Lemierre's syndrome is a rare complication of acute pharyngitis characterised by septicaemia with infective thrombophlebitis of the internal jugular vein, most commonly due to Fusobacterium necrophorum . It characteristically affects healthy young adults causing persistent pyrexia and systemic sepsis presenting several days after an initial pharyngitis. Septic emboli seed via the bloodstream to distant sites including the lung, joints, skin, liver, spleen and brain. Prolonged antimicrobial therapy is required and admission to intensive care common. This once rare condition is increasing in incidence but awareness amongst clinicians is low. We present a classic case in a young man who developed multi-organ failure requiring intensive care support and describe the epidemiology, pathophysiology, microbiology, clinical features and management of the disease.

  2. A gap between Need and Reality: Neonatal Nursing Staff Requirements on a German Intensive Care Unit

    OpenAIRE

    Patry, Christian; Schindler, Monika; Reinhard, Julia; Hien, Steffen; Demirakca, Süha; Böhler, Thomas; Schaible, Thomas

    2014-01-01

    Recently, new staffing rules for neonatal nurses in intensive care units (ICU) were issued in Germany, using categories of care of the British Association of Perinatal Medicine as blueprint. Neonates on intensive care require a nurse-to-patient ratio of 1:1, on intensive surveillance (high dependency care) of 1:2. No requirements exist for special care, transitional care, and pediatric ICU patients. Using these rules, nursing staff requirement was calculated over a period of 31 consecutive da...

  3. Design Requirements for Communication-Intensive Interactive Applications

    Science.gov (United States)

    Bolchini, Davide; Garzotto, Franca; Paolini, Paolo

    Online interactive applications call for new requirements paradigms to capture the growing complexity of computer-mediated communication. Crafting successful interactive applications (such as websites and multimedia) involves modeling the requirements for the user experience, including those leading to content design, usable information architecture and interaction, in profound coordination with the communication goals of all stakeholders involved, ranging from persuasion to social engagement, to call for action. To face this grand challenge, we propose a methodology for modeling communication requirements and provide a set of operational conceptual tools to be used in complex projects with multiple stakeholders. Through examples from real-life projects and lessons-learned from direct experience, we draw on the concepts of brand, value, communication goals, information and persuasion requirements to systematically guide analysts to master the multifaceted connections of these elements as drivers to inform successful communication designs.

  4. Therapeutic Plasma Exchange in Critically Ill Children Requiring Intensive Care.

    Science.gov (United States)

    Cortina, Gerard; McRae, Rosemary; Chiletti, Roberto; Butt, Warwick

    2018-02-01

    To characterize the clinical indications, procedural safety, and outcome of critically ill children requiring therapeutic plasma exchange. Retrospective observational study based on a prospective registry. Tertiary and quaternary referral 30-bed PICU. Forty-eight critically ill children who received therapeutic plasma exchange during an 8-year period (2007-2014) were included in the study. Therapeutic plasma exchange. A total of 48 patients underwent 244 therapeutic plasma exchange sessions. Of those, therapeutic plasma exchange was performed as sole procedure in 193 (79%), in combination with continuous renal replacement therapy in 40 (16.4%) and additional extracorporeal membrane oxygenation in 11 (4.6%) sessions. The most common admission diagnoses were hematologic disorders (30%), solid organ transplantation (20%), neurologic disorders (20%), and rheumatologic disorders (15%). Complications associated with the procedure occurred in 50 (21.2%) therapeutic plasma exchange sessions. Overall, patient survival from ICU was 82%. Although patients requiring therapeutic plasma exchange alone (n = 31; 64%) had a survival rate of 97%, those with additional continuous renal replacement therapy (n = 13; 27%) and extracorporeal membrane oxygenation (n = 4; 8%) had survival rates of 69% and 50%, respectively. Factors associated with increased mortality were lower Pediatric Index of Mortality 2 score, need for mechanical ventilation, higher number of failed organs, and longer ICU stay. Our results indicate that, in specialized centers, therapeutic plasma exchange can be performed relatively safely in critically ill children, alone or in combination with continuous renal replacement therapy and extracorporeal membrane oxygenation. Outcome in children requiring therapeutic plasma exchange alone is excellent. However, survival decreases with the number of failed organs and the need for continuous renal replacement therapy and extracorporeal membrane oxygenation.

  5. The incidence of intensive care unit-acquired weakness syndromes: A systematic review.

    Science.gov (United States)

    Appleton, Richard Td; Kinsella, John; Quasim, Tara

    2015-05-01

    We conducted a literature review of the intensive care unit-acquired weakness syndromes (critical illness polyneuropathy, critical illness myopathy and critical illness neuromyopathy) with the primary objective of determining their incidence as a combined group. Studies were identified through MEDLINE, Embase, Cochrane Database and article reference list searches and were included if they evaluated the incidence of one or more of these conditions in an adult intensive care unit population. The incidence of an intensive care unit-acquired weakness syndrome in the included studies was 40% (1080/2686 patients, 95% confidence interval 38-42%). The intensive care unit populations included were heterogeneous though largely included patients receiving mechanical ventilation for seven or more days. Additional prespecified outcomes identified that the incidence of intensive care unit-acquired weakness varied with the diagnostic technique used, being lower with clinical (413/1276, 32%, 95% CI 30-35%) compared to electrophysiological techniques (749/1591, 47%, 95% CI 45-50%). Approximately a quarter of patients were not able to comply with clinical evaluation and this may be responsible for potential underreporting of this condition.

  6. High intensity aerobic interval training improves peak oxygen consumption in patients with metabolic syndrome: CAT

    Directory of Open Access Journals (Sweden)

    Alexis Espinoza Salinas

    2014-06-01

    Full Text Available Introduction A number of cardiovascular risk factors characterizes the metabolic syndrome: insulin resistance (IR, low HDL cholesterol and high triglycerides. The aforementioned risk factors lead to elevated levels of abdominal adipose tissue, resulting in oxygen consumption deficiency. Purpose To verify the validity and applicability of using high intensity interval training (HIIT in subjects with metabolic syndrome and to answer the following question: Can HIIT improve peak oxygen consumption? Method The systematic review "Effects of aerobic interval training on exercise capacity and metabolic risk factors in individuals with cardiometabolic disorders" was analyzed. Results Data suggests high intensity aerobic interval training increases peak oxygen consumption by a standardized mean difference of 3.60 mL/kg-1/min-1 (95% confidence interval, 0.28-4.91. Conclusion In spite of the methodological shortcomings of the primary studies included in the systematic review, we reasonably conclude that implementation of high intensity aerobic interval training in subjects with metabolic syndrome, leads to increases in peak oxygen consumption.

  7. Persistent inflammation and immunosuppression: a common syndrome and new horizon for surgical intensive care.

    Science.gov (United States)

    Gentile, Lori F; Cuenca, Alex G; Efron, Philip A; Ang, Darwin; Bihorac, Azra; McKinley, Bruce A; Moldawer, Lyle L; Moore, Frederick A

    2012-06-01

    Surgical intensive care unit (ICU) stay of longer than 10 days is often described by the experienced intensivist as a "complicated clinical course" and is frequently attributed to persistent immune dysfunction. "Systemic inflammatory response syndrome" (SIRS) followed by "compensatory anti-inflammatory response syndrome" (CARS) is a conceptual framework to explain the immunologic trajectory that ICU patients with severe sepsis, trauma, or emergency surgery for abdominal infection often traverse, but the causes, mechanisms, and reasons for persistent immune dysfunction remain unexplained. Often involving multiple-organ failure (MOF) and death, improvements in surgical intensive care have altered its incidence, phenotype, and frequency and have increased the number of patients who survive initial sepsis or surgical events and progress to a persistent inflammation, immunosuppression, and catabolism syndrome (PICS). Often observed, but rarely reversible, these patients may survive to transfer to a long-term care facility only to return to the ICU, but rarely to self-sufficiency. We propose that PICS is the dominant pathophysiology and phenotype that has replaced late MOF and prolongs surgical ICU stay, usually with poor outcome. This review details the evolving epidemiology of MOF, the clinical presentation of PICS, and our understanding of how persistent inflammation and immunosuppression define the pathobiology of prolonged intensive care. Therapy for PICS will involve innovative interventions for immune system rebalance and nutritional support to regain physical function and well-being. Copyright © 2012 by Lippincott Williams & Wilkins.

  8. Clinical Characteristics and Outcomes of Patients With Cellulitis Requiring Intensive Care

    NARCIS (Netherlands)

    Cranendonk, Duncan R.; van Vught, Lonneke A.; Wiewel, Maryse A.; Cremer, Olaf L.; Horn, Janneke; Bonten, Marc J.; Schultz, Marcus J.; van der Poll, Tom; Wiersinga, W. Joost

    2017-01-01

    Cellulitis is a commonly occurring skin and soft tissue infection and one of the most frequently seen dermatological diseases in the intensive care unit (ICU). However, clinical characteristics of patients with cellulitis requiring intensive care treatment are poorly defined. Necrotizing fasciitis

  9. Clinical Characteristics and Outcomes of Patients With Cellulitis Requiring Intensive Care

    NARCIS (Netherlands)

    Cranendonk, Duncan R; van Vught, Lonneke A; Wiewel, Maryse A; Cremer, Olaf L; Horn, Janneke; Bonten, Marc J; Schultz, Marcus J; van der Poll, Tom; Wiersinga, W Joost

    Importance: Cellulitis is a commonly occurring skin and soft tissue infection and one of the most frequently seen dermatological diseases in the intensive care unit (ICU). However, clinical characteristics of patients with cellulitis requiring intensive care treatment are poorly defined. Necrotizing

  10. Epidemiology, Patterns of Care, and Mortality for Patients With Acute Respiratory Distress Syndrome in Intensive Care Units in 50 Countries

    NARCIS (Netherlands)

    Bellani, Giacomo; Laffey, John G.; Pham, Tài; Fan, Eddy; Brochard, Laurent; Esteban, Andres; Gattinoni, Luciano; van Haren, Frank; Larsson, Anders; McAuley, Daniel F.; Ranieri, Marco; Rubenfeld, Gordon; Thompson, B. Taylor; Wrigge, Hermann; Slutsky, Arthur S.; Pesenti, Antonio; Francois, Guy M.; Rabboni, Francesca; Madotto, Fabiana; Conti, Sara; Sula, Hektor; Nunci, Lordian; Cani, Alma; Zazu, Alan; Dellera, Christian; Insaurralde, Carolina S.; Alejandro, Risso V.; Daldin, Julio; Vinzio, Mauricio; Fernandez, Ruben O.; Cardonnet, Luis P.; Bettini, Lisandro R.; Bisso, Mariano Carboni; Osman, Emilio M.; Setten, Mariano G.; Lovazzano, Pablo; Alvarez, Javier; Villar, Veronica; Pozo, Norberto C.; Grubissich, Nicolas; Plotnikow, Gustavo A.; Vasquez, Daniela N.; Ilutovich, Santiago; Tiribelli, Norberto; Chena, Ariel; Pellegrini, Carlos A.; Saenz, María G.; Estenssoro, Elisa; Brizuela, Matias; Gianinetto, Hernan; Gomez, Pablo E.; Cerrato, Valeria I.; Bezzi, Marco G.; Borello, Silvina A.; Loiacono, Flavia A.; Fernandez, Adriana M.; Knowles, Serena; Reynolds, Claire; Inskip, Deborah M.; Miller, Jennene J.; Kong, Jing; Whitehead, Christina; Bihari, Shailesh; Seven, Aylin; Krstevski, Amanda; Rodgers, Helen J.; Millar, Rebecca T.; Mckenna, Toni E.; Bailey, Irene M.; Hanlon, Gabrielle C.; Aneman, Anders; Lynch, Joan M.; Azad, Raman; Neal, John; Woods, Paul W.; Roberts, Brigit L.; Kol, Mark R.; Wong, Helen S.; Riss, Katharina C.; Staudinger, Thomas; Wittebole, Xavier; Berghe, Caroline; Bulpa, Pierre A.; Dive, Alain M.; Verstraete, Rik; Lebbinck, Herve; Depuydt, Pieter; Vermassen, Joris; Meersseman, Philippe; Ceunen, Helga; Rosa, Jonas I.; Beraldo, Daniel O.; Piras, Claudio; Rampinelli, Adenilton M.; Nassar, Antonio P.; Mataloun, Sergio; Moock, Marcelo; Thompson, Marlus M.; Gonçalves, Claudio H.; Antônio, Ana Carolina P.; Ascoli, Aline; Biondi, Rodrigo S.; Fontenele, Danielle C.; Nobrega, Danielle; Sales, Vanessa M.; Shindhe, Suresh; Ismail, Dk Maizatul Aiman B. Pg Hj; Laffey, John; Beloncle, Francois; Davies, Kyle G.; Cirone, Rob; Manoharan, Venika; Ismail, Mehvish; Goligher, Ewan C.; Jassal, Mandeep; Ferguson, Niall D.; Nishikawa, Erin; Javeed, Areej; Curley, Gerard; Rittayamai, Nuttapol; Parotto, Matteo; Mehta, Sangeeta; Knoll, Jenny; Pronovost, Antoine; Canestrini, Sergio; Bruhn, Alejandro R.; Garcia, Patricio H.; Aliaga, Felipe A.; Farías, Pamela A.; Yumha, Jacob S.; Ortiz, Claudia A.; Salas, Javier E.; Saez, Alejandro A.; Vega, Luis D.; Labarca, Eduardo F.; Martinez, Felipe T.; Carreño, Nicolás G.; Lora, Pilar; Liu, Haitao; Qiu, Haibo; Liu, Ling; Tang, Rui; Luo, Xiaoming; An, Youzhong; Zhao, Huiying; Gao, Yan; Zhai, Zhe; Ye, Zheng L.; Wang, Wei; Li, Wenwen; Li, Qingdong; Zheng, Ruiqiang; Yu, Wenkui; Shen, Juanhong; Li, Xinyu; Yu, Tao; Lu, Weihua; Wu, Ya Q.; Huang, Xiao B.; He, Zhenyang; Lu, Yuanhua; Han, Hui; Zhang, Fan; Sun, Renhua; Wang, Hua X.; Qin, Shu H.; Zhu, Bao H.; Zhao, Jun; Liu, Jian; Li, Bin; Liu, Jing L.; Zhou, Fa C.; Li, Qiong J.; Zhang, Xing Y.; Li-Xin, Zhou; Xin-Hua, Qiang; Jiang, Liangyan; Gao, Yuan N.; Zhao, Xian Y.; Li, Yuan Y.; Li, Xiao L.; Wang, Chunting; Yao, Qingchun; Yu, Rongguo; Chen, Kai; Shao, Huanzhang; Qin, Bingyu; Huang, Qing Q.; Zhu, Wei H.; Hang, Ai Y.; Hua, Ma X.; Li, Yimin; Xu, Yonghao; Di, Yu D.; Ling, Long L.; Qin, Tie H.; Wang, Shou H.; Qin, Junping; Han, Yi; Zhou, Suming; Vargas, Monica P.; Jimenez, Juan I. Silesky; Rojas, Manuel A. González; Solis-Quesada, Jaime E.; Ramirez-Alfaro, Christian M.; Máca, Jan; Sklienka, Peter; Gjedsted, Jakob; Christiansen, Aage; Nielsen, Jonas; Villamagua, Boris G.; Llano, Miguel; Burtin, Philippe; Buzancais, Gautier; Beuret, Pascal; Pelletier, Nicolas; Mortaza, Satar; Mercat, Alain; Chelly, Jonathan; Jochmans, Sébastien; Terzi, Nicolas; Daubin, Cédric; Carteaux, Guillaume; de Prost, Nicolas; Chiche, Jean-Daniel; Daviaud, Fabrice; Fartoukh, Muriel; Barberet, Guillaume; Biehler, Jerome; Dellamonica, Jean; Doyen, Denis; Arnal, Jean-Michel; Briquet, Anais; Klasen, Fanny; Papazian, Laurent; Follin, Arnaud; Roux, Damien; Messika, Jonathan; Kalaitzis, Evangelos; Dangers, Laurence; Combes, Alain; Au, Siu-Ming; Béduneau, Gaetan; Carpentier, Dorothée; Zogheib, Elie H.; Dupont, Herve; Ricome, Sylvie; Santoli, Francesco L.; Besset, Sebastien L.; Michel, Philippe; Gelée, Bruno; Danin, Pierre-Eric; Goubaux, Bernard; Crova, Philippe J.; Phan, Nga T.; Berkelmans, Frantz; Badie, Julio C.; Tapponnier, Romain; Gally, Josette; Khebbeb, Samy; Herbrecht, Jean-Etienne; Schneider, Francis; Declercq, Pierre-Louis M.; Rigaud, Jean-Philippe; Duranteau, Jacques; Harrois, Anatole; Chabanne, Russell; Marin, Julien; Constantin, Jean-Michel; Thibault, Sandrine; Ghazi, Mohammed; Boukhazna, Messabi; Zein, Salem Ould; Richecoeur, Jack R.; Combaux, Daniele M.; Grelon, Fabien; Le Moal, Charlene; Sauvadet, Elise P.; Robine, Adrien; Lemiale, Virginie; Reuter, Danielle; Dres, Martin; Demoule, Alexandre; Goldgran-Toledano, Dany; Baboi, Loredana; Guérin, Claude; Lohner, Ralph; Kraßler, Jens; Schäfer, Susanne; Zacharowski, Kai D.; Meybohm, Patrick; Reske, Andreas W.; Simon, Philipp; Hopf, Hans-Bernd F.; Schuetz, Michael; Baltus, Thomas; Papanikolaou, Metaxia N.; Papavasilopoulou, Theonymfi G.; Zacharas, Giannis A.; Ourailogloy, Vasilis; Mouloudi, Eleni K.; Massa, Eleni V.; Nagy, Eva O.; Stamou, Electra E.; Kiourtzieva, Ellada V.; Oikonomou, Marina A.; Avila, Luis E.; Cortez, Cesar A.; Citalán, Johanna E.; Jog, Sameer A.; Sable, Safal D.; Shah, Bhagyesh; Gurjar, Mohan; Baronia, Arvind K.; Memon, Mohammedfaruk; Muthuchellappan, Radhakrishnan; Ramesh, Venkatapura J.; Shenoy, Anitha; Unnikrishnan, Ramesh; Dixit, Subhal B.; Rhayakar, Rachana V.; Ramakrishnan, Nagarajan; Bhardwaj, Vallish K.; Mahto, Heera L.; Sagar, Sudha V.; Palaniswamy, Vijayanand; Ganesan, Deeban; Hashemian, Seyed Mohammadreza; Jamaati, Hamidreza; Heidari, Farshad; Meaney, Edel A.; Nichol, Alistair; Knapman, Karl M.; O'Croinin, Donall; Dunne, Eimhin S.; Breen, Dorothy M.; Clarkson, Kevin P.; Jaafar, Rola F.; Dwyer, Rory; Amir, Fahd; Ajetunmobi, Olaitan O.; O'Muircheartaigh, Aogan C.; Black, Colin S.; Treanor, Nuala; Collins, Daniel V.; Altaf, Wahid; Zani, Gianluca; Fusari, Maurizio; Spadaro, Savino; Volta, Carlo A.; Graziani, Romano; Brunettini, Barbara; Palmese, Salvatore; Formenti, Paolo; Umbrello, Michele; Lombardo, Andrea; Pecci, Elisabetta; Botteri, Marco; Savioli, Monica; Protti, Alessandro; Mattei, Alessia; Schiavoni, Lorenzo; Tinnirello, Andrea; Todeschini, Manuel; Giarratano, Antonino; Cortegiani, Andrea; Sher, Sara; Rossi, Anna; Antonelli, Massimo M.; Montini, Luca M.; Casalena, Paolo; Scafetti, Sergio; Panarello, Giovanna; Occhipinti, Giovanna; Patroniti, Nicolò; Pozzi, Matteo; Biscione, Roberto R.; Poli, Michela M.; Raimondi, Ferdinando; Albiero, Daniela; Crapelli, Giulia; Beck, Eduardo; Pota, Vincenzo; Schiavone, Vincenzo; Molin, Alexandre; Tarantino, Fabio; Monti, Giacomo; Frati, Elena; Mirabella, Lucia; Cinnella, Gilda; Fossali, Tommaso; Colombo, Riccardo; Pattarino, Pierpaolo Terragni Ilaria; Mojoli, Francesco; Braschi, Antonio; Borotto, Erika E.; Cracchiolo, Andrea N.; Palma, Daniela M.; Raponi, Francesco; Foti, Giuseppe; Vascotto, Ettore R.; Coppadoro, Andrea; Brazzi, Luca; Floris, Leda; Iotti, Giorgio A.; Venti, Aaron; Yamaguchi, Osamu; Takagi, Shunsuke; Maeyama, Hiroki N.; Watanabe, Eizo; Yamaji, Yoshihiro; Shimizu, Kazuyoshi; Shiozaki, Kyoko; Futami, Satoru; Ryosuke, Sekine; Saito, Koji; Kameyama, Yoshinobu; Ueno, Keiko; Izawa, Masayo; Okuda, Nao; Suzuki, Hiroyuki; Harasawa, Tomofumi; Nasu, Michitaka; Takada, Tadaaki; Ito, Fumihito; Nunomiya, Shin; Koyama, Kansuke; Abe, Toshikazu; Andoh, Kohkichi; Kusumoto, Kohei; Hirata, Akira; Takaba, Akihiro; Kimura, Hiroyasu; Matsumoto, Shuhei; Higashijima, Ushio; Honda, Hiroyuki; Aoki, Nobumasa; Imai, Hiroshi; Ogino, Yasuaki; Mizuguchi, Ichiko; Ichikado, Kazuya; Nitta, Kenichi; Mochizuki, Katsunori; Hashida, Tomoaki; Tanaka, Hiroyuki; Nakamura, Tomoyuki; Niimi, Daisuke; Ueda, Takeshi; Kashiwa, Yozo; Uchiyama, Akinori; Sabelnikovs, Olegs; Oss, Peteris; Haddad, Youssef; Liew, Kong Y.; Ñamendys-Silva, Silvio A.; Jarquin-Badiola, Yves D.; Sanchez-Hurtado, Luis A.; Gomez-Flores, Saira S.; Marin, Maria C.; Villagomez, Asisclo J.; Lemus, Jordana S.; Fierro, Jonathan M.; Cervantes, Mavy Ramirez; Mejia, Francisco Javier Flores; Dector, Dulce; Dector, Dulce M.; Gonzalez, Daniel R.; Estrella, Claudia R.; Sanchez-Medina, Jorge R.; Ramirez-Gutierrez, Alvaro; George, Fernando G.; Aguirre, Janet S.; Buensuseso, Juan A.; Poblano, Manuel; Dendane, Tarek; Zeggwagh, Amine Ali; Balkhi, Hicham; Elkhayari, Mina; Samkaoui, Nacer; Ezzouine, Hanane; Benslama, Abdellatif; Amor, Mourad; Maazouzi, Wajdi; Cimic, Nedim; Beck, Oliver; Bruns, Monique M.; Schouten, Jeroen A.; Rinia, Myra; Raaijmakers, Monique; Heunks, Leo M.; van Wezel, Hellen M.; Heines, Serge J.; Strauch, Ulrich; Buise, Marc P.; Simonis, Fabienne D.; Schultz, Marcus J.; Goodson, Jennifer C.; Browne, Troy S.; Navarra, Leanlove; Hunt, Anna; Hutchison, Robyn A.; Bailey, Mathew B.; Newby, Lynette; Mcarthur, Colin; Kalkoff, Michael; Mcleod, Alex; Casement, Jonathan; Hacking, Danielle J.; Andersen, Finn H.; Dolva, Merete S.; Laake, Jon H.; Barratt-Due, Andreas; Noremark, Kim Andre L.; Søreide, Eldar; Sjøbø, Brit Å; Guttormsen, Anne B.; Yoshido, Hector H. Leon; Aguilar, Ronald Zumaran; Oscanoa, Fredy A. Montes; Alisasis, Alain U.; Robles, Joanne B.; Pasanting-Lim, Rossini Abbie B.; Tan, Beatriz C.; Andruszkiewicz, Pawel; Jakubowska, Karina; Coxo, Cristina M.; Alvarez, António M.; Oliveira, Bruno S.; Montanha, Gustavo M.; Barros, Nelson C.; Pereira, Carlos S.; Messias, António M.; Monteiro, Jorge M.; Araujo, Ana M.; Catorze, Nuno T.; Marum, Susan M.; Bouw, Maria J.; Gomes, Rui M.; Brito, Vania A.; Castro, Silvia; Estilita, Joana M.; Barros, Filipa M.; Serra, Isabel M.; Martinho, Aurelia M.; Tomescu, Dana R.; Marcu, Alexandra; Bedreag, Ovidiu H.; Papurica, Marius; Corneci, Dan E.; Negoita, Silvius Ioan; Grigoriev, Evgeny; Gritsan, Alexey I.; Gazenkampf, Andrey A.; Almekhlafi, Ghaleb; Albarrak, Mohamad M.; Mustafa, Ghanem M.; Maghrabi, Khalid A.; Salahuddin, Nawal; Aisa, Tharwat M.; Al Jabbary, Ahmed S.; Tabhan, Edgardo; Arabi, Yaseen M.; Trinidad, Olivia A.; Al Dorzi, Hasan M.; Tabhan, Edgardo E.; Bolon, Stefan; Smith, Oliver; Mancebo, Jordi; Aguirre-Bermeo, Hernan; Lopez-Delgado, Juan C.; Esteve, Francisco; Rialp, Gemma; Forteza, Catalina; de Haro, Candelaria; Artigas, Antonio; Albaiceta, Guillermo M.; de Cima-Iglesias, Sara; Seoane-Quiroga, Leticia; Ceniceros-Barros, Alexandra; Ruiz-Aguilar, Antonio L.; Claraco-Vega, Luis M.; Soler, Juan Alfonso; Lorente, Maria del Carmen; Hermosa, Cecilia; Gordo, Federico; Prieto-González, Miryam; López-Messa, Juan B.; Perez, Manuel P.; Perez, Cesar P.; Allue, Raquel Montoiro; Roche-Campo, Ferran; Ibañez-Santacruz, Marcos; Temprano, Susana; Pintado, Maria C.; de Pablo, Raul; Gómez, Pilar Ricart Aroa; Ruiz, Silvia Rodriguez; Moles, Silvia Iglesias; Jurado, Ma Teresa; Arizmendi, Alfons; Piacentini, Enrique A.; Franco, Nieves; Honrubia, Teresa; Cheng, Meisy Perez; Losada, Elena Perez; Blanco, Javier; Yuste, Luis J.; Carbayo-Gorriz, Cecilia; Cazorla-Barranquero, Francisca G.; Alonso, Javier G.; Alda, Rosa S.; Algaba, Ángela; Navarro, Gonzalo; Cereijo, Enrique; Diaz-Rodriguez, Esther; Marcos, Diego Pastor; Montero, Laura Alvarez; Para, Luis Herrera; Sanchez, Roberto Jimenez; Navalpotro, Miguel Angel Blasco; Abad, Ricardo Diaz; González, Raquel Montiel; Toribio, Dácil Parrilla; Castro, Alejandro G.; Artiga, Maria Jose D.; Penuelas, Oscar; Roser, Tomas P.; Olga, Moreno F.; Curto, Elena Gallego; Sánchez, Rocío Manzano; Imma, Vallverdu P.; Elisabet, Garcia M.; Claverias, Laura; Magret, Monica; Pellicer, Ana M.; Rodriguez, Lucia L.; Sánchez-Ballesteros, Jesús; González-Salamanca, Ángela; Jimenez, Antonio G.; Huerta, Francisco P.; Diaz, Juan Carlos J. Sotillo; Lopez, Esther Bermejo; Moya, David D. Llinares; Alfonso, Alec A. Tallet; Luis, Palazon Sanchez Eugenio; Cesar, Palazon Sanchez; Rafael, Sánchez I.; Virgilio, Corcoles G.; Recio, Noelia N.; Adamsson, Richard O.; Rylander, Christian C.; Holzgraefe, Bernhard; Broman, Lars M.; Wessbergh, Joanna; Persson, Linnea; Schiöler, Fredrik; Kedelv, Hans; Tibblin, Anna Oscarsson; Appelberg, Henrik; Hedlund, Lars; Helleberg, Johan; Eriksson, Karin E.; Glietsch, Rita; Larsson, Niklas; Nygren, Ingela; Nunes, Silvia L.; Morin, Anna-Karin; Kander, Thomas; Adolfsson, Anne; Piquilloud, Lise; Zender, Hervé O.; Leemann-Refondini, Corinne; Elatrous, Souheil; Bouchoucha, Slaheddine; Chouchene, Imed; Ouanes, Islem; Ben Souissi, Asma; Kamoun, Salma; Demirkiran, Oktay; Aker, Mustafa; Erbabacan, Emre; Ceylan, Ilkay; Girgin, Nermin Kelebek; Ozcelik, Menekse; Ünal, Necmettin; Meco, Basak Ceyda; Akyol, Onat O.; Derman, Suleyman S.; Kennedy, Barry; Parhar, Ken; Srinivasa, Latha; McNamee, Lia; McAuley, Danny; Steinberg, Jack; Hopkins, Phil; Mellis, Clare; Kakar, Vivek; Hadfield, Dan; Vercueil, Andre; Bhowmick, Kaushik; Humphreys, Sally K.; Ferguson, Andrew; Mckee, Raymond; Raj, Ashok S.; Fawkes, Danielle A.; Watt, Philip; Twohey, Linda; Jha, Rajeev R.; Thomas, Matthew; Morton, Alex; Kadaba, Varsha; Smith, Mark J.; Hormis, Anil P.; Kannan, Santhana G.; Namih, Miriam; Reschreiter, Henrik; Camsooksai, Julie; Kumar, Alek; Rugonfalvi, Szabolcs; Nutt, Christopher; Oneill, Orla; Seasman, Colette; Dempsey, Ged; Scott, Christopher J.; Ellis, Helen E.; McKechnie, Stuart; Hutton, Paula J.; Di Tomasso, Nora N.; Vitale, Michela N.; Griffin, Ruth O.; Dean, Michael N.; Cranshaw, Julius H.; Willett, Emma L.; Ioannou, Nicholas; Gillis, Sarah; Csabi, Peter; Macfadyen, Rosaleen; Dawson, Heidi; Preez, Pieter D.; Williams, Alexandra J.; Boyd, Owen; de Gordoa, Laura Ortiz-Ruiz; Bramall, Jon; Symmonds, Sophie; Chau, Simon K.; Wenham, Tim; Szakmany, Tamas; Toth-Tarsoly, Piroska; Mccalman, Katie H.; Alexander, Peter; Stephenson, Lorraine; Collyer, Thomas; Chapman, Rhiannon; Cooper, Raphael; Allan, Russell M.; Sim, Malcolm; Wrathall, David W.; Irvine, Donald A.; Zantua, Kim S.; Adams, John C.; Burtenshaw, Andrew J.; Sellors, Gareth P.; Welters, Ingeborg D.; Williams, Karen E.; Hessell, Robert J.; Oldroyd, Matthew G.; Battle, Ceri E.; Pillai, Suresh; Kajtor, Istvan; Sivashanmugavel, Mageswaran; Okane, Sinead C.; Donnelly, Adrian; Frigyik, Aniko D.; Careless, Jon P.; May, Martin M.; Stewart, Richard; Trinder, T. John; Hagan, Samantha J.; Wise, Matt P.; Cole, Jade M.; MacFie, Caroline C.; Dowling, Anna T.; Hurtado, Javier; Nin, Nicolás; Nuñez, Edgardo; Pittini, Gustavo; Rodriguez, Ruben; Imperio, María C.; Santos, Cristina; França, Ana G.; Ebeid, Alejandro; Deicas, Alberto; Serra, Carolina; Uppalapati, Aditya; Kamel, Ghassan; Banner-Goodspeed, Valerie M.; Beitler, Jeremy R.; Mukkera, Satyanarayana Reddy; Kulkarni, Shreedhar; Lee, Jarone; Mesar, Tomaz; Shinn, John O.; Gomaa, Dina; Tainter, Christopher; Yeatts, Dale J.; Warren, Jessica; Lanspa, Michael J.; Miller, Russel R.; Grissom, Colin K.; Brown, Samuel M.; Bauer, Philippe R.; Gosselin, Ryan J.; Kitch, Barrett T.; Cohen, Jason E.; Beegle, Scott H.; Stoger, John H.; Gueret, Renaud M.; Tulaimat, Aiman; Choudry, Shazia; Stigler, William; Batra, Hitesh; Huff, Nidhi G.; Lamb, Keith D.; Oetting, Trevor W.; Mohr, Nicholas M.; Judy, Claine; Saito, Shigeki; Kheir, Fayez M.; Kheir, Fayez; Schlichting, Adam B.; Delsing, Angela; Crouch, Daniel R.; Elmasri, Mary; Ismail, Dina; Dreyer, Kyle R.; Blakeman, Thomas C.; Baron, Rebecca M.; Grijalba, Carolina Quintana; Hou, Peter C.; Seethala, Raghu; Aisiku, Imo; Henderson, Galen; Frendl, Gyorgy; Hou, Sen-Kuang; Owens, Robert L.; Schomer, Ashley; Bumbasirevic, Vesna; Jovanovic, Bojan; Surbatovic, Maja; Veljovic, Milic

    2016-01-01

    IMPORTANCE Limited information exists about the epidemiology, recognition, management, and outcomes of patients with the acute respiratory distress syndrome (ARDS). OBJECTIVES To evaluate intensive care unit (ICU) incidence and outcome of ARDS and to assess clinician recognition, ventilation

  11. INTENSIVE THERAPY OF THE ENDOGENOUS INTOXICATION SYNDROME IN PATIENTS WITH SEVERE BURNS

    Directory of Open Access Journals (Sweden)

    N. M. Shulayeva

    2013-01-01

    Full Text Available BACKGROUND. Severe burns are accompanied by the development of endogenous intoxication syndrome. Endogenous intoxication syndrome (EIS is thus specified as a complex of severe clinical aspects with detoxification systems failure — in other words, the syndrome of multi-organ failure (MOFS or inefficiency, depending on severity of EIS (compensation, subcompensation, decompensation [1, 2].PURPOSE. To improve the results of EIS and MOFS treatment in the patients with severe burns by including into medication succinic acid-based anti-oxidants combined with hepatoprotectors of the ademetionine group.MATERIAL AND METHODS. We carried out analysis of complex examination and treatment of 108 patients aged 18–50 with severe thermal injury, total burn area (S ≥40%, and severity index ≥90.RESULTS. The use of succinic acid-based anti-oxidants within the framework of antishock and disintoxication therapy, later combined with hepatoprotectors of the ademetionine group, decreases the intensity of endogenous intoxication and multi-organ failure syndrome.CONCLUSION. To treat endotoxemia and multiple organ dysfunction caused by oxidative stress and hypoxia in patients with severe and extremely severe burns, it is appropriate to prescribe succinic acid-based anti-oxidants. To prevent albumen synthesis and detoxication hepar dysfunctions, decrease the manifestations of endotoxemia, and complement the cytoprotective action of succinic acid-based antioxidants, it is appropriate to prescribe hepatoprotectors. 

  12. Outcomes for patients with Guillain-Barré syndrome requiring mechanical ventilation: a literature review.

    Science.gov (United States)

    de Boisanger, L

    2016-02-01

    This is a literature review of outcomes for patients with Guillain-Barré Syndrome (GBS) who require admission to the intensive care unit for mechanical ventilation. Respiratory distress is the leading cause of death in the acute phase, and occurs in about 25 % of patients. The aim of this review is to compile, analyse, and summarise the most relevant literature looking at outcomes for Guillain-Barré (GB) patients requiring admission to the intensive care unit and mechanical ventilation. A PubMed and Google-Scholar literature search was performed using the key words 'Guillain-Barré, Outcomes, Mechanical Ventilation, Prognosis, Mortality, ICU. All 7 papers from the years 2000-2014 which assessed outcomes for GBS patients requiring mechanical ventilation were included, and critically analysed. The parameters recorded by these studies looked at mortality, disability, length of hospitalisation, and complications. The mortality of GB patients requiring mechanical ventilation varied from 8.3 to 20 %, Disability was primarily measured by the GBS disability scale. One study deemed that a score of 0-1 was a positive outcome, and found that slightly over half 53.8 % of the patients fulfilled that criteria. Over half of the mechanically ventilated patients were required to be admitted for over 3 weeks. Complications during ICU admission are common, with bed-sores (40 %), pneumonia (30.2 %) and sepsis (17.4) being the most frequently encountered in one study. Accurate data are limited by the fact that these studies are retrospective, often covering long periods in the past. Larger, more recent, prospective, multi-centre studies will be required.

  13. Implementing a Mobility Program to Minimize Post-Intensive Care Syndrome.

    Science.gov (United States)

    Hopkins, Ramona O; Mitchell, Lorie; Thomsen, George E; Schafer, Michele; Link, Maggie; Brown, Samuel M

    2016-01-01

    Immobility in the intensive care unit (ICU) is associated with neuromuscular weakness, post-intensive care syndrome, functional limitations, and high costs. Early mobility-based rehabilitation in the ICU is feasible and safe. Mobility-based rehabilitation varied widely across 5 ICUs in 1 health care system, suggesting a need for continuous training and evaluation to maintain a strong mobility-based rehabilitation program. Early mobility-based rehabilitation shortens ICU and hospital stays, reduces delirium, and increases muscle strength and the ability to ambulate. Long-term effects include increased ability for self-care, faster return to independent functioning, improved physical function, and reduced hospital readmission and death. Factors that influence early mobility-based rehabilitation include having an interdisciplinary team; strong unit leadership; access to physical, occupational, and respiratory therapists; a culture focused on patient safety and quality improvement; a champion of early mobility; and a focus on measuring performance and outcomes.

  14. Long-term outcome in patients with Guillain-Barré syndrome requiring mechanical ventilation.

    Science.gov (United States)

    Witsch, J; Galldiks, N; Bender, A; Kollmar, R; Bösel, J; Hobohm, C; Günther, A; Schirotzek, I; Fuchs, K; Jüttler, E

    2013-05-01

    We aimed to determine long-term disability and quality of life in patients with Guillain-Barré syndrome (GBS) who required mechanical ventilation (MV) in the acute phase. Our retrospective cohort study included 110 GBS patients admitted to an intensive care unit and requiring MV (01/1999-08/2010) in nine German tertiary academic medical centers. Outcome was determined 1 year or longer after hospital admission using the GBS disability scale, Barthel index (BI), EuroQuol-5D (EQ-5D) and Fatigue Severity Scale. Linear/multivariate regression analysis was used to analyze predicting factors for outcome. Mean time to follow up was 52.6 months. Hospital mortality was 5.5 % and long-term mortality 13.6 %. Overall 53.8 % had a favorable outcome (GBS disability score 0-1) and 73.7 % of survivors had no or mild disability (BI 90-100). In the five dimensions of the EQ-5D "mobility", "self-care", "usual activities", "pain" and "anxiety/depression" no impairments were stated by 50.6, 58.4, 36.4, 36.4 and 50.6 % of patients, respectively. A severe fatigue syndrome was present in 30.4 % of patients. Outcome was statistically significantly correlated with age, type of therapy and number of immunoglobulin courses. In GBS-patients requiring MV in the acute phase in-hospital, and long-term mortality are lower than that in previous studies, while long-term quality of life is compromised in a large fraction of patients, foremost by immobility and chronic pain. Efforts towards improved treatment approaches should address autonomic dysfunction to further reduce hospital mortality while improved rehabilitation concepts might ameliorate long-term disability.

  15. [Equivalent continuous noise level in neonatal intensive care unit associated to burnout syndrome].

    Science.gov (United States)

    Garrido Galindo, A P; Camargo Caicedo, Y; Vélez-Pereira, A M

    2015-01-01

    Noise levels in neonatal intensive care units allow the appearance of symptoms associated with burnout such as stress, irritability, fatigue and emotional instability on health care personnel. The aim of this study was to evaluate the equivalent continuous noise levels in the neonatal intensive care unit and compare the results with noise levels associated with the occurrence of burnout syndrome on the care team. Continuous sampling was conducted for 20 days using a type I sound level meter on the unit. The maximum, the ninetieth percentile and the equivalent continuous noise level (Leq) values were recorded. Noise level is reported in the range of 51.4-77.6 decibels A (dBA) with an average of 64 dBA, 100.6 dBA maximum, and average background noise from 57.9 dBA. Noise levels exceed the standards suggested for neonatal intensive care units, are close to maximum values referred for noise exposure in the occupational standards and to noise levels associated with the onset of burnout; thus allowing to infer the probability of occurrence of high levels of noise present in the unit on the development of burnout in caregivers. Copyright © 2013 Elsevier España, S.L.U. y SEEIUC. All rights reserved.

  16. Angiotensin-converting Enzyme Inhibitor Angioedema Requiring Admission to an Intensive Care Unit.

    Science.gov (United States)

    Soo Hoo, Guy W; Lin, Henry K; Junaid, Imran; Klaustermeyer, William B

    2015-07-01

    The purpose of this study was to review consecutive cases of angiotensin-converting enzyme (ACE) inhibitor angioedema admitted to an intensive care unit. Fifty subjects with ACE-inhibitor angioedema admitted from 1998-2011 were reviewed. All 50 subjects were men, 62.8 ± 8.4 years of age, 76% African Americans. Fifteen (30%) required ventilatory support and 2 (4%) required tracheostomy. Over half (56%) had taken ACE inhibitors for over a year. Logistic regression identified dyspnea and tongue involvement with the need for ventilatory support (P angioedema. Angioedema can occur even after extended use. Dyspnea and tongue involvement identified patients requiring ventilatory support. Published by Elsevier Inc.

  17. Mutual influence of intensity of pain syndrome and borderline mental disorders in patients with coxarthrosis

    Directory of Open Access Journals (Sweden)

    I. D. Spirina

    2017-02-01

    Full Text Available The objective of this study is to evaluate the mutual influence of pain syndrome and borderline psychiatric disorders depending on its intensity and tolerability in patients with coxarthrosis who need endoprosthetics. 76 patients with coxarthrosis aged from 25 to 68 who were hospitalized in the Department of Endoprosthetics at Mechnikov Regional Clinical Hospital in Dnipro City in the period from November 2015 to September 2016 were observed. For diagnosis of psychopathological disorders, and for evaluation of the effectiveness of therapeutic interventions, the following methods were used in our research: clinical and psychopathological (technique SCL-90-R, Tаylor anxiety scale, study of the type of attitude to the disease (LOBI, Dembo-Rubinstein self-esteem scale, Leonhard-Schmieschek questionnaire for assessment of accentuation of personality traits, the Luscher 8-colour test and the Toronto alexithymia scale (TAS. Severity of pain syndrome was assessed using a visual analogue scale of pain (VAS. Forms of borderline mental disorders were diagnosed in 51 patients with coxarthrosis, such as depressive disorder (F 32 – 19 (24.8%, neurasthenia (F 48 – 12 (16.2, anxiety and phobic disorders (F 40–41 – 14 (18.1%, and personality disorders (F 60.5, F 60.6, F 60.7 – 6 (7.6%. In 25 (33.3% patients clinically-defined forms of mental disorders were identified. Leading syndromes in these disorders were depression – 19 (24.8% patients, anxiety and phobic – 15 (20.0%, asthenic – 10 (12.4%, hypochondriacal – 7 (9.5% patients. According to the results of the correlation analysis, a close correlation between the severity of pain syndrome and borderline mental disorders (r = 0.779 was established for patients in the preoperative stage. The average level of pain syndrome on the VAS scale in patients with borderline mental disorders was twice as high as in patients without these disorders (63.4 vs. 32.4 points, but it does not depend on the

  18. The impact of the patient post-intensive care syndrome components upon caregiver burden.

    Science.gov (United States)

    Torres, J; Carvalho, D; Molinos, E; Vales, C; Ferreira, A; Dias, C C; Araújo, R; Gomes, E

    2017-11-01

    To evaluate patient post-intensive care syndrome (PICS-P) and caregiver burden 3 months after discharge from the Intensive Care Unit (ICU) and determine the impact of different components of PICS-P upon caregiver burden. A prospective observational study was conducted over 26 months (January 2013-February 2015). Medical-surgical ICU and follow-up consultation in Portugal. Patients discharged after a minimum of 2 days in the ICU. Caregiver inclusion criteria: not paid, written and spoken Portuguese, and agreement to participate in the study. In ICU: Patient gender, age, severity of illness (SAPS II) and length of ICU stay. At 3 months caregiver burden, physical (reduced mobility, weakness acquired in the ICU) and psychological components of PICS (anxiety, depression, post-traumatic stress disorder). A total of 168 caregivers completed the survey (response rate of 69%). A low degree of overburden was reported by 34.5% of caregivers, while 15.5% showed moderate to high levels of overburden. Patient anxiety and depression 3 months after ICU discharge significantly influenced the presence of caregiver burden (p=0.030 vs p=0.008). When physical components of PICS-P were evaluated, no influence on caregiver burden was observed. Patient demographics, severity of illness and length of stay also failed to influence caregiver burden. The presence of psychological components of PICS-P 3 months after ICU seems to have a negative impact upon caregiver burden. On the other hand, physical problems showed no important impact upon caregiver overburden. Copyright © 2017 Elsevier España, S.L.U. y SEMICYUC. All rights reserved.

  19. Emergency management of children with acute severe asthma requiring transfer to intensive care.

    Science.gov (United States)

    Dehò, Anna; Lutman, Daniel; Montgomery, Mary; Petros, Andy; Ramnarayan, Padmanabhan

    2010-11-01

    Children presenting to emergency departments (ED) with acute severe asthma unresponsive to initial medical therapy may require endotracheal intubation and mechanical ventilation. There is little data on complications during the acute management of children with life-threatening asthma, particularly at hospitals where specialist paediatric staff are lacking. It was hypothesised that a better understanding of complications, particularly associated with intubation and mechanical ventilation, would improve acute management in ED, aid quality improvement initiatives at district general hospitals (DGH) and form the basis for educational interventions from regional paediatric critical care units. A retrospective case note review was performed for all children referred to a regional intensive care retrieval service with status asthmaticus over a 2-year period. Initial treatment, patient-related factors, indication for endotracheal intubation and the type and occurrence of adverse events during acute management at the DGH were studied. Bivariate and multivariate analyses were undertaken to identify factors associated with the occurrence of complications. 51 (85%) of the 60 children transferred to a paediatric intensive care unit for acute severe asthma required intubation. 36 (70.5%) experienced one or more complications during intubation and in the early phase of mechanical ventilation. The most common complications were hypotension (requiring fluid resuscitation and/or inotropic support) and severe bronchospasm with acute hypercarbia. The indication for intubation significantly affected the chances of a complication occurring during stabilisation. There is considerable morbidity in asthmatic children who are referred to paediatric intensive care. The majority of complications may be anticipated and prevented resulting in improved management at DGH.

  20. Incidence and preventability of adverse events requiring intensive care admission: a systematic review.

    Science.gov (United States)

    Vlayen, Annemie; Verelst, Sandra; Bekkering, Geertruida E; Schrooten, Ward; Hellings, Johan; Claes, Neree

    2012-04-01

    Adverse events are unintended patient injuries or complications that arise from health care management resulting in death, disability or prolonged hospital stay. Adverse events that require critical care are a considerable financial burden to the health care system, but also their global impact on patients and society is probably underestimated. The objectives of this systematic review were to synthesize the best available evidence regarding the estimates of the incidence and preventability of adverse events that necessitate intensive care admission, to determine the type and consequences [mortality, length of intensive care unit (ICU) stay and costs] of these adverse events. MEDLINE (from 1966 to present), EMBASE (from 1974 to present) and CENTRAL (version 1-2010) were searched for studies reporting on unplanned admissions on ICUs. Several other sources were searched for additional studies. Only quantitative studies that used chart review for the detection of adverse events requiring intensive care admission were considered for eligibility. For the purposes of this systematic review, ICUs were defined as specialized hospital facilities which provide continuous monitoring and intensive care for acutely ill patients. Studies that were published in the English, Dutch, German, French or Spanish language were eligible for inclusion. Two reviewers independently extracted data and assessed the methodological quality of the included studies. A total of 27 studies were reviewed. Meta-analysis of the data was not appropriate because of methodological and statistical heterogeneity between studies; therefore, results are presented in a descriptive way. The percentage of surgical and medical adverse events that required ICU admission ranged from 1.1% to 37.2%. ICU readmissions varied from 0% to 18.3%. Preventability of the adverse events varied from 17% to 76.5%. Preventable adverse events are further synthesized by type of event. Consequences of the adverse events included a

  1. Does nonstationarity in rainfall require nonstationary intensity-duration-frequency curves?

    Science.gov (United States)

    Ganguli, Poulomi; Coulibaly, Paulin

    2017-12-01

    In Canada, risk of flooding due to heavy rainfall has risen in recent decades; the most notable recent examples include the July 2013 storm in the Greater Toronto region and the May 2017 flood of the Toronto Islands. We investigate nonstationarity and trends in the short-duration precipitation extremes in selected urbanized locations in Southern Ontario, Canada, and evaluate the potential of nonstationary intensity-duration-frequency (IDF) curves, which form an input to civil infrastructural design. Despite apparent signals of nonstationarity in precipitation extremes in all locations, the stationary vs. nonstationary models do not exhibit any significant differences in the design storm intensity, especially for short recurrence intervals (up to 10 years). The signatures of nonstationarity in rainfall extremes do not necessarily imply the use of nonstationary IDFs for design considerations. When comparing the proposed IDFs with current design standards, for return periods (10 years or less) typical for urban drainage design, current design standards require an update of up to 7 %, whereas for longer recurrence intervals (50-100 years), ideal for critical civil infrastructural design, updates ranging between ˜ 2 and 44 % are suggested. We further emphasize that the above findings need re-evaluation in the light of climate change projections since the intensity and frequency of extreme precipitation are expected to intensify due to global warming.

  2. Lesions requiring wound management in a central tertiary neonatal intensive care unit.

    Science.gov (United States)

    Meszes, Angéla; Tálosi, Gyula; Máder, Krisztina; Orvos, Hajnalka; Kemény, Lajos; Csoma, Zsanett Renáta

    2017-04-01

    Most of the skin disorders that occur in neonatal intensive care units are due in part to the immaturity and vulnerability of the neonatal skin. Various iatrogenic diagnostic and therapeutic procedures are also conducive to iatrogenic damage. This study was to review the neonates admitted to our neonatal intensive care unit who needed wound management, and to assess the most common skin injuries and wounds, and their aetiology. Data were extracted from medical records of neonates who needed wound management in our Neonatal Intensive Care Unit between January 31, 2012 and January 31, 2013. Information about gestational age, sex, birth weight, area of involvement, wound aetiology, and therapy were collected. Among the 211 neonates observed, wound management was required in 10 cases of diaper dermatitis, 7 epidermal stripping, 6 extravasation injuries, 5 pressure ulcers, 1 surgical wound and infection, 1 thermal burn, and 5 other lesions. International guidelines in neonatal wound care practice are not available, and further research concerns are clearly needed. Dressings and antiseptic agents should be chosen with great care for application to neonates, with particular attention to the prevention of adverse events in this sensitive population. Team work among dermatologists, neonatologists and nurses is crucial for the successful treatment of neonates.

  3. Financial impact of nursing professionals staff required in an Intensive Care Unit 1

    Science.gov (United States)

    de Araújo, Thamiris Ricci; Menegueti, Mayra Gonçalves; Auxiliadora-Martins, Maria; Castilho, Valéria; Chaves, Lucieli Dias Pedreschi; Laus, Ana Maria

    2016-01-01

    ABSTRACT Objective: to calculate the cost of the average time of nursing care spent and required by patients in the Intensive Care Unit (ICU) and the financial expense for the right dimension of staff of nursing professionals. Method: a descriptive, quantitative research, using the case study method, developed in adult ICU patients. We used the workload index - Nursing Activities Score; the average care time spent and required and the amount of professionals required were calculated using equations and from these data, and from the salary composition of professionals and contractual monthly time values, calculated the cost of direct labor of nursing. Results: the monthly cost of the average quantity of available professionals was US$ 35,763.12, corresponding to 29.6 professionals, and the required staff for 24 hours of care is 42.2 nurses, with a monthly cost of US$ 50,995.44. Conclusion: the numerical gap of nursing professionals was 30% and the monthly financial expense for adaptation of the structure is US$ 15,232.32, which corresponds to an increase of 42.59% in the amounts currently paid by the institution. PMID:27878219

  4. Competence requirements in intensive and critical care nursing--still in need of definition? A Delphi study.

    Science.gov (United States)

    Lakanmaa, Riitta-Liisa; Suominen, Tarja; Perttilä, Juha; Puukka, Pauli; Leino-Kilpi, Helena

    2012-12-01

    Empirical studies in competence are lacking in the field of intensive and critical care nursing. To identify competence requirements, by soliciting the views of intensive care unit nurses and physicians. Two rounds of the Delphi method were used in 2006 in Finland. Data were analysed by content analysis and with descriptive statistics. Competence requirements in intensive and critical care nursing can be divided into five main domains: knowledge base, skill base, attitude and value base, nursing experience base and personal base of the nurse. Four of these domains can be found in the existing requirements and one new domain - personal base of the nurse - was identified. Competence requirements are multidimensional. Earlier descriptions of competence are not sufficient; more comprehensive and cohesive descriptions are needed. The personal base of a nurse should also be included in the competence requirements in intensive and critical care nursing. Copyright © 2012 Elsevier Ltd. All rights reserved.

  5. Survival advantage with decitabine versus intensive chemotherapy in patients with higher risk myelodysplastic syndrome: comparison with historical experience.

    Science.gov (United States)

    Kantarjian, Hagop M; O'Brien, Susan; Huang, Xuelin; Garcia-Manero, Guillermo; Ravandi, Farhad; Cortes, Jorge; Shan, Jianqin; Davisson, Jan; Bueso-Ramos, Carlos E; Issa, Jean-Pierre

    2007-03-15

    Decitabine, a hypomethylating agent, is active and has been approved for the treatment of myelodysplastic syndrome (MDS) and chronic myelomonocytic leukemia. Intensive chemotherapy is an accepted form of therapy for patients with higher risk MDS. The comparative efficacy of these 2 forms of treatment in MDS is unknown. The objective of the current study was to compare the efficacy and toxicity profiles of decitabine and intensive chemotherapy in MDS. The authors compared lower intensity decitabine therapy (n = 115 patients) with intensive chemotherapy (as it is used in acute myeloid leukemia [AML]) in patients with higher risk MDS. Two comparisons were made with a cohort of 376 historic patients (from 1995 to 2005): The first comparison included a subcohort of 115 patients (Group A) who matched the 115 decitabine study patients according to age, International Prognostic Scoring System, and cytogenetics; and the second comparison included the whole cohort of 376 patients without matching (Group B). A multivariate analysis was performed for outcome. The complete remission (CR) rate according to AML criteria was 43% with decitabine, 46% with intensive chemotherapy in Group A, and 52% with intensive chemotherapy in Group B. Compared with Group A, mortality at 6 weeks was 3% with decitabine versus 13% with intensive chemotherapy (P = .006) and, at 3 months, 7% with decitabine versus 23% with intensive chemotherapy (P = .001). Survival was better with decitabine versus intensive chemotherapy in Group A (median survival: 22 months vs 12 months; P accounting for the independent prognostic effect of pretreatment factors. In this analysis, decitabine was associated with a survival advantage compared with intensive chemotherapy in patients with higher risk MDS. Future studies should evaluate prospectively the results of decitabine versus intensive chemotherapy in this setting.

  6. Drug-induced long QT syndrome and fatal arrhythmias in the intensive care unit.

    Science.gov (United States)

    Beitland, S; Platou, E S; Sunde, K

    2014-03-01

    Long QT syndrome (LQTS) is a genetic or acquired condition characterised by a prolonged QT interval on the surface electrocardiogram (ECG) and is associated with a high risk of sudden cardiac death because of polymorph ventricular tachyarrhythmia called Torsade de Pointes arrhythmia. Drug-induced LQTS can occur as a side effect of commonly used cardiac and non-cardiac drugs in predisposed patients, often with baseline QT prolongation lengthened by medication and/or electrolyte disturbances. Hospitalised patients often have several risk factors for proarrhythmic response, such as advanced age and structural heart disease. Patients in the intensive care unit (ICU) are particularly prone to develop drug induced LQTS because they receive several different intravenous medications. Additionally, they might have impaired drug elimination because of reduced kidney and/or liver function, and also drug-drug-interactions. The clinical symptoms and signs of LQTS range from asymptomatic patients to sudden death because of malignant arrhythmias, and it is therefore important to recognise the clinical characteristics and typical ECG changes. Treatment of acquired LQTS is mainly awareness, identification and discontinuation of QT prolonging drugs, in addition to eventually supplement of magnesium and potassium. Overdrive cardiac pacing is highly effective in preventing recurrences, and antiarrhythmic drugs should be avoided. Recent data suggest that QT prolongation is quite common in ICU patients and adversely affects patient mortality. Thus, high-risk patients should be sufficiently monitored, and the use of medications known to cause drug-induced LQTS might have to be restricted. © 2014 The Acta Anaesthesiologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.

  7. [Surveillance of Supervised Flat-Sharing Communities Requiring Intensive Home Care: Results and Conclusions].

    Science.gov (United States)

    Horvath, Leila; Böhm, Doris; Gleich, Sabine

    2018-01-23

    Patients with intensive care and long-term mechanical ventilation needs are increasingly cared for in supervised flat-sharing communities. The municipal public health and environment department of Munich audited nursing services between April 2015 and August 2016. The structural analysis of the nursing services was conducted using standardised checklists, and statistical analysis was performed. In agreement with the residents and providers of the nursing service, flats were inspected. 20 of the 43 supervised flat-sharing communities in Munich were designed for intensive care patients. Nine nursing services took care of them. Monitoring of organizational structures and hygiene management were found to be positive. There was room for improvement in practical implementation of hygiene standards. Requirements for personal qualifications and for emergencies such as electrical power outages have to be regulated. It was shown that regular consulting, instructions and auditing by the municipal public health and environment department have a positive effect on hygiene and emergency management. National and binding agreements still need to be worked out. © Georg Thieme Verlag KG Stuttgart · New York.

  8. [Severe Reye syndrome: report of 14 cases managed in a pediatric intensive care unit over 11 years].

    Science.gov (United States)

    Thabet, F; Durand, P; Chevret, L; Fabre, M; Debray, D; Brivet, M; Devictor, D

    2002-06-01

    Idiopathic Reye syndrome is a rare disease revealed by unexplained encephalopathy and microvesicular liver steatosis. Some clinical and epidemiological studies mainly performed in English speaking countries questioned the reality of Reye syndrome because numerous know inherited metabolic diseases, and some of them unrecognized, could mimick this disorder. We focused in our study on severe forms of Reye syndrome admitted to a pediatric intensive care unit. Retrospective study over the last eleven years (1991-2001) included all the pediatric patients admitted to our tertiary referral center with the classical American Reye syndrome criteria (e.g. CDC). Extensive metabolic screening was performed in all cases, except for the ultimately dead patients. Fourteen patients (mean age 52 months) were included. Fever always occurred before their admission and aspirin (n = 12) or acetaminophen (n = 7) was prescribed. Median Glasgow scale was 7 on admission. Mean amoniac plasma level was 320 mumol/L and alanine-aminotransferase peak plasma level 1475 +/- 1387 IU/L. Mechanical ventilation was started in ten children and six of them underwent continuous venovenous hemofiltration. Three patients ultimately died and 11 survived with a mean five years follow-up without relapses or neurological impairment. Any of them demonstrated inherited metabolic disease except for one infant with hereditary fructose intolerance. Unlike widespread opinion, severe Reye syndrome without identified metabolic disorders seems to not disappear in our country. Reye syndrome remains a potentially life threatening disease and raises for aggressive treatment of brain edema. If aspirin and Reye syndrome association are not formally documented in France, cautiousness must be kept in mind and all the aspirin adverse effects notifications should be addressed to the public drugs survey network.

  9. Input-output analysis of energy requirements for short rotation, intensive culture, woody biomass

    International Nuclear Information System (INIS)

    Strauss, C.H.; Grado, S.C.

    1992-01-01

    A production model for short rotation, intensive culture (SRIC) plantations was developed to determine the energy and financial cost of woody biomass. The model was based on hybrid poplars planted on good quality agricultural sites at a density of 2100 cuttings ha -1 , with average annual growth forecast at 16 metric tonne, oven dry (mg(OD)). Energy and financial analyses showed preharvest cost 4381 megajoules (MJ) Mg -1 (OD) and $16 (US) Mg -1 (OD). Harvesting and transportation requirements increased the total costs 6130 MJ Mg -1 (OD) and $39 Mg -1 (OD) for the delivered material. On an energy cost basis, the principal input was land, whereas on a financial basis, costs were more uniformly distributed among equipment, land, labor, and materials and fuel

  10. Envelope protein requirements for the assembly of infectious virions of porcine reproductive and respiratory syndrome virus

    NARCIS (Netherlands)

    Wissink, E.H.J.; Kroese, M.V.; Wijk, van H.A.; Rijsewijk, F.A.M.; Meulenberg, J.J.; Rottier, P.J.M.

    2005-01-01

    Virions of porcine reproductive and respiratory syndrome virus (PRRSV) contain six membrane proteins: the major proteins GP5 and M and the minor proteins GP2a, E, GP3, and GP4. Here, we studied the envelope protein requirements for PRRSV particle formation and infectivity using full-length cDNA

  11. Emotion Recognition in Children with Down Syndrome: Influence of Emotion Label and Expression Intensity

    Science.gov (United States)

    Cebula, Katie R.; Wishart, Jennifer G.; Willis, Diane S.; Pitcairn, Tom K.

    2017-01-01

    Some children with Down syndrome may experience difficulties in recognizing facial emotions, particularly fear, but it is not clear why, nor how such skills can best be facilitated. Using a photo-matching task, emotion recognition was tested in children with Down syndrome, children with nonspecific intellectual disability and cognitively matched,…

  12. Variable-Intensity Simulated Team-Sport Exercise Increases Daily Protein Requirements in Active Males.

    Science.gov (United States)

    Packer, Jeffrey E; Wooding, Denise J; Kato, Hiroyuki; Courtney-Martin, Glenda; Pencharz, Paul B; Moore, Daniel R

    2017-01-01

    Protein requirements are generally increased in strength and endurance trained athletes relative to their sedentary peers. However, less is known about the daily requirement for this important macronutrient in individuals performing variable intensity, stop-and-go type exercise that is typical for team sport athletes. The objective of the present study was to determine protein requirements in active, trained adult males performing a simulated soccer match using the minimally invasive indicator amino acid oxidation (IAAO) method. After 2 days of controlled diet (1.2 g⋅kg -1 ⋅day -1 protein), seven trained males (23 ± 1 years; 177.5 ± 6.7 cm; 82.3 ± 6.1 kg; 13.5% ± 4.7% body fat; 52.3 ± 5.9 ml O 2 ⋅kg -1 ⋅min -1 ; mean ± SD) performed an acute bout of variable intensity exercise in the form of a modified Loughborough Intermittent Shuttle Test (4 × 15 min of exercise over 75 min). Immediately after exercise, hourly meals were consumed providing a variable amount of protein (0.2-2.6 g⋅kg -1 ⋅day -1 ) and sufficient energy and carbohydrate (6 g⋅kg -1 ⋅day -1 ). Protein was provided as a crystalline amino acids modeled after egg protein with the exception of phenylalanine and tyrosine, which were provided in excess to ensure the metabolic partitioning of the indicator amino acid (i.e., [1- 13 C]phenylalanine included within the phenylalanine intake) was directed toward oxidation when protein intake was limiting. Whole body phenylalanine flux and 13 CO 2 excretion (F 13 CO 2 ) were determined at metabolic and isotopic steady state from urine and breath samples, respectively. Biphasic linear regression analysis was performed on F 13 CO 2 to determine the estimated average requirement (EAR) for protein with a safe intake defined as the upper 95% confidence interval. Phenylalanine flux was not impacted by protein intake ( P  = 0.45). Bi-phase linear regression ( R 2  = 0.64) of F 13 CO 2 resulted

  13. High- and moderate-intensity aerobic exercise and excess post-exercise oxygen consumption in men with metabolic syndrome.

    Science.gov (United States)

    Larsen, I; Welde, B; Martins, C; Tjønna, A E

    2014-06-01

    Physical activity is central in prevention and treatment of metabolic syndrome. High-intensity aerobic exercise can induce larger energy expenditure per unit of time compared with moderate-intensity exercise. Furthermore, it may induce larger energy expenditure at post-exercise recovery. The aim of this study is to compare the excess post-exercise oxygen consumption (EPOC) in three different aerobic exercise sessions in men with metabolic syndrome. Seven men (age: 56.7 ± 10.8) with metabolic syndrome participated in this crossover study. The sessions consisted of one aerobic interval (1-AIT), four aerobic intervals (4-AIT), and 47-min continuous moderate exercise (CME) on separate days, with at least 48 h between each test day. Resting metabolic rate (RMR) was measured pre-exercise and used as baseline value. EPOC was measured until baseline metabolic rate was re-established. An increase in O2 uptake lasting for 70.4 ± 24.8 min (4-AIT), 35.9 ± 17.3 min (1-AIT), and 45.6 ± 17.3 min (CME) was observed. EPOC were 2.9 ± 1.7 L O2 (4-AIT), 1.3 ±  .1 L O2 (1-AIT), and 1.4 ± 1.1 L O2 (CME). There were significant differences (P EPOC was highest after 4-AIT. These data suggest that exercise intensity has a significant positive effect on EPOC in men with metabolic syndrome. © 2013 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  14. Surgery for subacromial impingement syndrome in relation to intensities of occupational mechanical exposures across 10-year exposure time windows

    DEFF Research Database (Denmark)

    Dalbøge, Annett; Frost, Poul; Andersen, Johan Hviid

    2018-01-01

    OBJECTIVES: We aimed to identify intensities of occupational mechanical exposures (force, arm elevation and repetition) that do not entail an increased risk of surgery for subacromial impingement syndrome (SIS) even after prolonged durations of exposure. Additionally, we wanted to evaluate...... if exposure to hand-arm vibration (HAV) is an independent risk factor. METHODS: We used data from a register-based cohort study of the entire Danish working population (n=2 374 403). During follow-up (2003-2008), 14 118 first-time events of surgery for SIS occurred. For each person, we linked register...... of exposure at specific intensities above minimal (low, medium and high). We used a logistic regression technique equivalent to discrete survival analysis adjusting for cumulative effects of other mechanical exposures. RESULTS: We found indications of safe exposure intensities for repetition (median angular...

  15. Research Thinking of Low-intensity laser For the Treatment of Menopausal Syndrome

    International Nuclear Information System (INIS)

    Chen, G Z; Wang, X Y; Xu, Y X; Li, L J; Liu, S H

    2011-01-01

    Female climacteric syndrome is a clinical syndrome due to autonomic nerve dysfunction occurring in women during climacteric period, which may affect their physical and mental health. Therefore, how to pass climacteric period for women without any problems, avoid or reduce the occurrence of climacteric syndrome, prevent geriatric diseases and improve life quality is a key issue now for great attention. Looking for a convenient, effective, and safer method without toxic-side effects to control the disease is a modern medical problem. By analyzing the relationship between laser technology and traditional acupuncture and moxibustion, the advantage and the existing problems on acupuncture and moxibustion for the treatment of menopausal syndrome, the application of laser methods for the mechanism research on TCM diagnosis and treatment of menopausal syndrome was discussed. It's pointed out that the laser acupuncture is safe and effective to treat menopausal syndrome. Breakthrough will be achieved from the research of the selection of the acupoint prescription and mechanism of Acupuncture and Moxibustion for the treatment of menopausal syndrome by utilizing the advantage of interdisciplinary intersection. Laser technology will make the development of acupuncture and moxibustion science possess an unprecedented field.

  16. Research Thinking of Low-intensity laser For the Treatment of Menopausal Syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Chen, G Z; Wang, X Y [Second Clinical Medical College, Guangzhou University of Chinese Medicine, Guangzhou, 510405 (China); Xu, Y X; Li, L J [Acupuncture and Massage College, Guangzhou University of Chinese Medicine, Guangzhou, 510405 (China); Liu, S H, E-mail: xuyx1968@163.com [South China Normal University, Guangzhou, 510631 (China)

    2011-02-01

    Female climacteric syndrome is a clinical syndrome due to autonomic nerve dysfunction occurring in women during climacteric period, which may affect their physical and mental health. Therefore, how to pass climacteric period for women without any problems, avoid or reduce the occurrence of climacteric syndrome, prevent geriatric diseases and improve life quality is a key issue now for great attention. Looking for a convenient, effective, and safer method without toxic-side effects to control the disease is a modern medical problem. By analyzing the relationship between laser technology and traditional acupuncture and moxibustion, the advantage and the existing problems on acupuncture and moxibustion for the treatment of menopausal syndrome, the application of laser methods for the mechanism research on TCM diagnosis and treatment of menopausal syndrome was discussed. It's pointed out that the laser acupuncture is safe and effective to treat menopausal syndrome. Breakthrough will be achieved from the research of the selection of the acupoint prescription and mechanism of Acupuncture and Moxibustion for the treatment of menopausal syndrome by utilizing the advantage of interdisciplinary intersection. Laser technology will make the development of acupuncture and moxibustion science possess an unprecedented field.

  17. Research Thinking of Low-intensity laser For the Treatment of Menopausal Syndrome

    Science.gov (United States)

    Chen, G. Z.; Xu, Y. X.; Wang, X. Y.; Liu, S. H.; Li, L. J.

    2011-02-01

    Female climacteric syndrome is a clinical syndrome due to autonomic nerve dysfunction occurring in women during climacteric period, which may affect their physical and mental health. Therefore, how to pass climacteric period for women without any problems, avoid or reduce the occurrence of climacteric syndrome, prevent geriatric diseases and improve life quality is a key issue now for great attention. Looking for a convenient, effective, and safer method without toxic-side effects to control the disease is a modern medical problem. By analyzing the relationship between laser technology and traditional acupuncture and moxibustion, the advantage and the existing problems on acupuncture and moxibustion for the treatment of menopausal syndrome, the application of laser methods for the mechanism research on TCM diagnosis and treatment of menopausal syndrome was discussed. It's pointed out that the laser acupuncture is safe and effective to treat menopausal syndrome. Breakthrough will be achieved from the research of the selection of the acupoint prescription and mechanism of Acupuncture and Moxibustion for the treatment of menopausal syndrome by utilizing the advantage of interdisciplinary intersection. Laser technology will make the development of acupuncture and moxibustion science possess an unprecedented field.

  18. Nasogastric Tube Syndrome: Why Is It Important in the Intensive Care Unit?

    Directory of Open Access Journals (Sweden)

    Taehyun Kim

    2015-08-01

    Full Text Available Although the nasogastric tube (NGT is widely used in critically ill patients, most intensivists do not give much thought to it or its possible complications. NGT syndrome is a rare but fatal complication characterized by throat pain and vocal cord paralysis in the presence of NGT. Recently, we experienced a case of NGT syndrome developed in an 86-year-old female twelve days after NGT insertion. We immediately removed the NGT and secured the airway by tracheostomy. She was treated successfully with an intravenous antibiotic, steroid and proton pump inhibitor and the syndrome did not recur after reinsertion of the NGT.

  19. Anti-N-Methyl-d-Aspartate Receptor Encephalitis in Adult Patients Requiring Intensive Care.

    Science.gov (United States)

    de Montmollin, Etienne; Demeret, Sophie; Brulé, Noëlle; Conrad, Marie; Dailler, Frédéric; Lerolle, Nicolas; Navellou, Jean-Christophe; Schwebel, Carole; Alves, Mikaël; Cour, Martin; Engrand, Nicolas; Tonnelier, Jean-Marie; Maury, Eric; Ruckly, Stéphane; Picard, Géraldine; Rogemond, Véronique; Magalhaes, Éric; Sharshar, Tarek; Timsit, Jean-François; Honnorat, Jérôme; Sonneville, Romain

    2017-02-15

    Encephalitis caused by anti-N-methyl-d-aspartate receptor (NMDAR) antibodies is the leading cause of immune-mediated encephalitis. There are limited data on intensive care unit (ICU) management of these patients. To identify prognostic factors of good neurologic outcome in patients admitted to an ICU with anti-NMDAR encephalitis. This was an observational multicenter study of all consecutive adult patients diagnosed with anti-NMDAR encephalitis at the French National Reference Centre, admitted to an ICU between 2008 and 2014. The primary outcome was a good neurologic outcome at 6 months after ICU admission, defined by a modified Rankin Scale score of 0-2. Seventy-seven patients were included from 52 ICUs. First-line immunotherapy consisted of steroids (n = 61/74; 82%), intravenous immunoglobulins (n = 71/74; 96%), and plasmapheresis (n = 17/74; 23%). Forty-five (61%) patients received second-line immunotherapy (cyclophosphamide, rituximab, or both). At 6 months, 57% of patients had a good neurologic outcome. Independent factors of good neurologic outcome were early (≤8 d after ICU admission) immunotherapy (odds ratio, 16.16; 95% confidence interval, 3.32-78.64; for combined first-line immunotherapy with steroids and intravenous immunoglobulins vs. late immunotherapy), and a low white blood cell count on the first cerebrospinal examination (odds ratio, 9.83 for 50 cells/mm 3 ; 95% confidence interval, 1.07-90.65). Presence of nonneurologic organ failures at ICU admission and occurrence of status epilepticus during ICU stay were not associated with neurologic outcome. The prognosis of adult patients with anti-NMDAR encephalitis requiring intensive care is good, especially when immunotherapy is initiated early, advocating for prompt diagnosis and early aggressive treatment.

  20. Emotion recognition in children with down syndrome: influence of emotion label and expression intensity.

    OpenAIRE

    Cebula, Katie R.; Wishart, Jennifer G.; Willis, Diane S.; Pitcairn, Tom K.

    2017-01-01

    Some children with Down syndrome may experience difficulties in recognising facial emotions, particularly fear, but it is not clear why, nor how such skills can best be facilitated. Using a photo-matching task, emotion recognition was tested in children with Down syndrome, children with non-specific intellectual disabilities and cognitively-matched typically-developing children (all groups N = 21) under four conditions: veridical vs exaggerated emotions and emotion-labelling vs generic task i...

  1. Surgery for subacromial impingement syndrome in relation to intensities of occupational mechanical exposures across 10-year exposure time windows.

    Science.gov (United States)

    Dalbøge, Annett; Frost, Poul; Andersen, Johan Hviid; Svendsen, Susanne Wulff

    2018-03-01

    We aimed to identify intensities of occupational mechanical exposures (force, arm elevation and repetition) that do not entail an increased risk of surgery for subacromial impingement syndrome (SIS) even after prolonged durations of exposure. Additionally, we wanted to evaluate if exposure to hand-arm vibration (HAV) is an independent risk factor. We used data from a register-based cohort study of the entire Danish working population (n=2 374 403). During follow-up (2003-2008), 14 118 first-time events of surgery for SIS occurred. For each person, we linked register-based occupational codes (1993-2007) to a general population job exposure matrix to obtain year-by-year exposure intensities on measurement scales for force, upper arm elevation >90° and repetition and expert rated intensities of exposure to HAV. For 10-year exposure time windows, we calculated the duration of exposure at specific intensities above minimal (low, medium and high). We used a logistic regression technique equivalent to discrete survival analysis adjusting for cumulative effects of other mechanical exposures. We found indications of safe exposure intensities for repetition (median angular velocity 90° >2 min/day implied an increased risk reaching ORs of 1.7 and 1.5 after 10 years at low intensities. No associations were found for HAV. We found indications of safe exposure intensities for repetition. Any intensities of force and upper arm elevation >90° above minimal implied an increased risk across 10-year exposure time windows. No independent associations were found for HAV. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  2. Facial emotion perception by intensity in children and adolescents with 22q11.2 deletion syndrome.

    Science.gov (United States)

    Leleu, Arnaud; Saucourt, Guillaume; Rigard, Caroline; Chesnoy, Gabrielle; Baudouin, Jean-Yves; Rossi, Massimiliano; Edery, Patrick; Franck, Nicolas; Demily, Caroline

    2016-03-01

    Difficulties in the recognition of emotions in expressive faces have been reported in people with 22q11.2 deletion syndrome (22q11.2DS). However, while low-intensity expressive faces are frequent in everyday life, nothing is known about their ability to perceive facial emotions depending on the intensity of expression. Through a visual matching task, children and adolescents with 22q11.2DS as well as gender- and age-matched healthy participants were asked to categorise the emotion of a target face among six possible expressions. Static pictures of morphs between neutrality and expressions were used to parametrically manipulate the intensity of the target face. In comparison to healthy controls, results showed higher perception thresholds (i.e. a more intense expression is needed to perceive the emotion) and lower accuracy for the most expressive faces indicating reduced categorisation abilities in the 22q11.2DS group. The number of intrusions (i.e. each time an emotion is perceived as another one) and a more gradual perception performance indicated smooth boundaries between emotional categories. Correlational analyses with neuropsychological and clinical measures suggested that reduced visual skills may be associated with impaired categorisation of facial emotions. Overall, the present study indicates greater difficulties for children and adolescents with 22q11.2DS to perceive an emotion in low-intensity expressive faces. This disability is subtended by emotional categories that are not sharply organised. It also suggests that these difficulties may be associated with impaired visual cognition, a hallmark of the cognitive deficits observed in the syndrome. These data yield promising tracks for future experimental and clinical investigations.

  3. A case of Lemierre's syndrome with septic shock and complicated parapneumonic effusions requiring intrapleural fibrinolysis

    Directory of Open Access Journals (Sweden)

    Daniel P. Croft

    2015-01-01

    Full Text Available Lemierre's syndrome is a septic thrombophlebitis of the internal jugular vein, which can lead to severe systemic illness. We report a case of an otherwise healthy 26-year-old man who suffered from pharyngitis followed by septic shock requiring intubation and vasopressor support from Fusobacterium necrophorum bacteremia. The septic emboli to his lungs caused complicated bilateral parapneumonic effusions, which recurred after initial drainage. He required bilateral chest tubes and intrapleural tPA to successfully drain his effusions. His fever curve and overall condition improved with the resolution of his effusions and after a 33-day hospitalization, he recovered without significant disability. The severity of his illness and difficult to manage complicated parapneumonic effusions were the unique facets of this case. Using an evidence-based approach of tPA and DNase for complicated parapneumonic effusions in Lemierre's syndrome can be safe and effective.

  4. Emotion Recognition in Children With Down Syndrome: Influence of Emotion Label and Expression Intensity.

    Science.gov (United States)

    Cebula, Katie R; Wishart, Jennifer G; Willis, Diane S; Pitcairn, Tom K

    2017-03-01

    Some children with Down syndrome may experience difficulties in recognizing facial emotions, particularly fear, but it is not clear why, nor how such skills can best be facilitated. Using a photo-matching task, emotion recognition was tested in children with Down syndrome, children with nonspecific intellectual disability and cognitively matched, typically developing children (all groups N = 21) under four conditions: veridical vs. exaggerated emotions and emotion-labelling vs. generic task instructions. In all groups, exaggerating emotions facilitated recognition accuracy and speed, with emotion labelling facilitating recognition accuracy. Overall accuracy and speed did not differ in the children with Down syndrome, although recognition of fear was poorer than in the typically developing children and unrelated to emotion label use. Implications for interventions are considered.

  5. Comparison of the effects of Matricaria chamomila (Chamomile) extract and mefenamic acid on the intensity of premenstrual syndrome.

    Science.gov (United States)

    Sharifi, Farangis; Simbar, Masoumeh; Mojab, Faraz; Majd, Hamid Alavi

    2014-02-01

    The study aimed to compare the effects of Chamomile Extract and Mefenamic acid (MA) on the intensity of Premenstrual syndrome (PMS) symptoms. This study was a clinical randomized double blind trial, carried out with 90 students living in the dorms of Iran. The participants filled the daily forms about the intensity of PMS for two consecutive months. Once the definitive diagnosis of PMS was made, the participants were divided into two groups, each receiving either Chamomile capsule 100 mg or MA 250 mg three times a day. Intensity reduction of emotional symptoms was significantly higher among Chamomile Extract-users (30.1 ± 26.6 and 33.4 ± 25.3 percent) than that among MA-users (11.6 ± 25.7 and 10.7 ± 26.8 percent) after two cycles intervention (p  0.05) among groups. Consumption of Chamomile seems to be more effective than MA in relieving the intensity of PMS associated symptomatic psychological pains. Copyright © 2013 Elsevier Ltd. All rights reserved.

  6. Unplanned transfer from the telemetry unit to the intensive care unit in hospitalized patients with suspected acute coronary syndrome.

    Science.gov (United States)

    Pelter, Michele M; Loranger, Denise; Kozik, Teri M; Fidler, Richard; Hu, Xiao; Carey, Mary G

    Most patients presenting with suspected acute coronary syndrome (ACS) are admitted to telemetry units. While telemetry is an appropriate level of care, acute complications requiring a higher level of care in the intensive care unit (ICU) occur. Among patients admitted to telemetry for suspected ACS, we determine the frequency of unplanned ICU transfer, and examine whether ECG changes indicative of myocardial ischemia, and/or symptoms preceded unplanned transfer. This was a secondary analysis from a study assessing occurrence rates for transient myocardial ischemia (TMI) using a 12-lead Holter. Clinicians were blinded to Holter data as it was used in the context research; off-line analysis was performed post discharge. Hospital telemetry monitoring was maintained as per hospital protocol. TMI was defined as >1mm ST-segment ↑ or ↓, in >1 ECG lead, >1minute. Symptoms were assessed by chart review. In 409 patients (64±13years), most were men (60%), Caucasian (93%), and had a history of coronary artery disease (47%). Unplanned transfer to the ICU occurred in 9 (2.2%), was equivalent by gender, and age (no transfer 64±13years vs transfer 67±11years). Four patients were transferred following unsuccessful percutaneous coronary intervention (PCI) attempt, four due to recurrent angina, and one due to renal and hepatic failure. Mean time from admission to transfer was 13±6hours, mean time to ECG detected ischemia was 6±5hours, and 8.8±5hours for symptoms prompting transfer. In two patients ECG detected ischemia and acute symptoms prompting transfer were simultaneous. In five patients, ECG detected ischemia was clinically silent. All patients eventually had symptoms that prompted transfer to the ICU. In all nine patients, there was no documentation or nursing notes regarding bedside ECG monitor changes prior to unplanned transfer. Hospital length of stay was longer in the unplanned transfer group (2days ± 2 versus 6days ± 4; p=0.018). In patients with suspected ACS

  7. Financial impact of nursing professionals staff required in an Intensive Care Unit.

    Science.gov (United States)

    Araújo, Thamiris Ricci de; Menegueti, Mayra Gonçalves; Auxiliadora-Martins, Maria; Castilho, Valéria; Chaves, Lucieli Dias Pedreschi; Laus, Ana Maria

    2016-11-21

    to calculate the cost of the average time of nursing care spent and required by patients in the Intensive Care Unit (ICU) and the financial expense for the right dimension of staff of nursing professionals. a descriptive, quantitative research, using the case study method, developed in adult ICU patients. We used the workload index - Nursing Activities Score; the average care time spent and required and the amount of professionals required were calculated using equations and from these data, and from the salary composition of professionals and contractual monthly time values, calculated the cost of direct labor of nursing. the monthly cost of the average quantity of available professionals was US$ 35,763.12, corresponding to 29.6 professionals, and the required staff for 24 hours of care is 42.2 nurses, with a monthly cost of US$ 50,995.44. the numerical gap of nursing professionals was 30% and the monthly financial expense for adaptation of the structure is US$ 15,232.32, which corresponds to an increase of 42.59% in the amounts currently paid by the institution. calcular o custo do tempo médio de assistência de enfermagem despendido e requerido pelos pacientes internados em Unidade de Terapia Intensiva (UTI) e o dispêndio financeiro para adequação do quadro de profissionais de enfermagem. pesquisa descritiva, quantitativa, na modalidade de estudo de caso, desenvolvida na UTI de pacientes adultos. Utilizou-se o índice de carga de trabalho - Nursing Activities Score; o tempo médio de assistência despendido, requerido e o quantitativo de profissionais requerido foram calculados por meio de equações e, a partir desses dados, e de valores da composição salarial dos profissionais e tempo mensal contratual, calculou-se o custo da mão de obra direta de enfermagem. o custo mensal do quantitativo médio de profissionais disponível foi de US$ 35.763,12, correspondendo a 29,6 profissionais, e o requerido para 24 horas de cuidado é de 42,2 profissionais de

  8. Nursing diagnoses, outcomes and interventions as measures of patient complexity and nursing care requirement in Intensive Care Unit.

    Science.gov (United States)

    Castellan, Cristiana; Sluga, Silvia; Spina, Eleonora; Sanson, Gianfranco

    2016-06-01

    To describe the nursing diagnoses, outcomes and interventions for patients admitted to intensive care units and to assess their possible relation with classical outcomes like length of stay and mortality. The analysis of nursing diagnosis frequencies may help to estimate the patients' complexity and the need for nursing interventions and can predict hospital outcomes. Nonetheless, few studies were conducted on critical patients. Prospective cohort observational study. Between 15 July-31 October 2013 we collected the above-described nursing parameters of 100 subjects throughout their stay in intensive care. We classified the parameters according to established taxonomies. The independent association between the number of nursing diagnoses and length of stay/mortality was investigated with multiple regressions. We found an average of 19 diagnoses, 24 outcomes and 60 interventions per patient. Most frequently, the plans of care involved support for self-care deficits or interrupted family processes. They also included strategies to prevent infection, disuse syndrome and impairment of skin integrity. Nineteen nursing diagnoses were significantly related with mortality or length of stay in bivariate analyses. In regression models, the number of such diagnoses explained 29·7% of the variance in length of stay and was an independent predictor of mortality. In critically ill patients, the analysis of nursing diagnoses, outcomes and interventions confirmed an intense activity in response to a broad spectrum of patient needs. The number of nursing diagnoses allowed to predict patient outcomes. © 2016 John Wiley & Sons Ltd.

  9. SHAKEN BABY SYNDROME – 10 YEARS EXPERIENCE IN A PAEDIATRIC INTENSIVE CARE SERVICE

    Directory of Open Access Journals (Sweden)

    Lara Lourenço

    2017-01-01

    Conclusion: In our study Shaken Baby Syndrome resulted in high morbidity with long-term sequelae in five patients (62,5%, consistent with data published in other series. The mortality rate was lower than in previous studies (12.5%. The presence of traumatic history was mentioned only in one case and, although all of them presented subdural haematomas, 25% of cases had no retinal haemorrhages. The clinical presentation was very severe and in most cases a high degree of suspicion is necessary in the diagnosis.

  10. INTENSITY, DURATION AND TYPE OF PHYSICAL ACTIVITY REQUIRED TO IMPROVE FUNCTION IN KNEE OSTEOARTHRITIS

    Science.gov (United States)

    KIRIHARA, RICARDO AKIHIRO; CATELAN, FELLIPE BRAVIM; FARIAS, FABIANE ELIZE SABINO DE; SILVA, CLEIDNÉIA APARECIDA CLEMENTE DA; CERNIGOY, CLAUDIA HELENA DE AZEVEDO; REZENDE, MÁRCIA UCHOA DE

    2017-01-01

    ABSTRACT Objective: To evaluate the effects of physical activity intensity, type and duration in patients with knee osteoarthritis (KOA). Methods: A retrospective study of 195 KOA patients who were followed for two years after receiving educational material about KOA with or without attending classes. The patients were evaluated at baseline and 24 months. At the evaluations, the patients answered questionnaires pertaining to pain and function (WOMAC, Lequesne, VAS and SF-36); reported the intensity, duration and type of exercise performed per week; and performed the Timed Up & Go (TUG) and Five Times Sit-to-Stand (FTSST) tests. Results: Increased age affected improvements in the TUG results (p=0.017). The type, intensity and duration of physical activity did not correlate with pain, function or quality of life improvements (p>0.05), but the TUG results were on average 4 seconds faster among the patients who practiced intense physical activity and/or exercised for more than 180 minutes per week and/or performed isolated weight training or swam compared with those who remained sedentary after 2 years (p=0.01; pbodybuilding) for relevant pain reduction and functional improvement.Level of Evidence II, Retrospective Study. PMID:28642646

  11. Burnout syndrome--assessment of a stressful job among intensive care staff.

    Science.gov (United States)

    Cubrilo-Turek, Mirjana; Urek, Roman; Turek, Stjepan

    2006-03-01

    The purpose of the study was to investigate the degree of burnout experienced by intensive care staff particularly, in Medical (MICU) and Surgical Intensive Care Units (SICU) General Hospital "Sveti Duh", Zagreb. A sample group of 41 emergency physicians and nurses from MICU and 30 from SICU was tested. The survey included demographic data and Maslach Burnout Inventory (MBI) scoring test identified by the three main components associated with burnout: emotional exhaustion (MBI-EE), depersonalization (MBI-DEP), and personal accomplishment (MBI-PA) were assessed using 22-item questionnaire. The degrees of burnout were stratified into low, moderate, and high range. Mean total MBI (X +/- SD) were high in both groups: higher for the MICU (65.5 +/- 6.7) than for SICU staff (55.7 +/- 3.8, p burnout represented in a moderate degree. The presence of burnout is a serious phenomenon, because it can lead to psychosomatic complaints, work-associated withdrawal behaviour, and a lower quality of care at intensive care units. Early recognition of burnout phenomenon as a result of prolonged stress and frustration among intensive care staff, contributes to better professional behavior, organizational structure changes in the work environment and better health care quality for critically ill patients.

  12. Remifentanil in the intensive care unit: tolerance and acute withdrawal syndrome after prolonged

    NARCIS (Netherlands)

    Delvaux, B.; Ryckwaert, Y.; Boven, van R.M.; Kock, M.; Capdevila, X.

    2005-01-01

    SEDATION in the intensive care unit should be minimized to reduce the duration of mechanical ventilation and its related complications.1 The drug regimen would ideally allow rapid awakening, to perform neurologic and respiratory evaluation on a daily basis.2,3 In this context, remifentanil, with its

  13. Electron beam requirements for a three-dimensional Smith-Purcell backward-wave oscillator for intense terahertz radiation

    Directory of Open Access Journals (Sweden)

    Kwang-Je Kim

    2007-08-01

    Full Text Available A Smith-Purcell device can operate as a backward-wave oscillator for intense, narrow-bandwidth, continuous wave radiation at terahertz wavelengths. We determine the requirements on electron beam current and emittance for the system to oscillate based on a three-dimensional extension of our previous two-dimensional analysis. It is found that specially designed electron beams are required with a current that exceeds a certain threshold value and a flat transverse profile that allows the beam to travel very close to the grating surface. Two methods for producing electron beams with the required characteristics are discussed.

  14. Severe alcohol withdrawal syndrome: Evolution of care and impact of adjunctive therapy on course and complications of 171 intensive care unit patients.

    Science.gov (United States)

    Puscas, Mircea; Hasoon, Mohammed; Eechevarria, Carlos; Cooper, Tracy; Tamura, Leslie; Chebbo, Ahmad; W Carlson, Richard

    2016-01-01

    This single site retrospective observational study assessed the evolution of sedation therapy for severe alcohol withdrawal syndrome in the intensive care unit. Patient records for 2 intervals were reviewed: Interval 1, which included 87 intensive care unit patients admitted January 2005 through September 2007, for whom benzodiazedpine monotherapy was utilized; and Interval 2, January 2010 through December 2010, for whom 54 of 84 (64.3%) intensive care unit patients, including all those intubated, received adjunctive agents, including dexmedetomidine or propofol. Clinical management was similar for both intervals, as well as prevalence of alcohol withdrawal syndrome versus total adult hospital admissions and comorbid conditions. Overall, respiratory failure (53 versus 39%), seizures (36 versus 18%), and pneumonia (51 versus 38%) were less frequent during Interval 2 (all p intensive care unit admission are excluded, the prevalence of these complications was similar (p = ns) for Interval 1 and Interval 2. Intensive care unit and hospital length of stay were not altered by adjunctive therapy, which was typically employed for more severely affected patients. High intensity sedation with adjunctive drugs led to few cardiovascular adverse events and may have facilitated management, but did not alter intensive care unit course of severe alcohol withdrawal syndrome.

  15. CD3+CD56+natural killer T cells in fibromyalgia syndrome patients: association with the intensity of depression.

    Science.gov (United States)

    Nugraha, Boya; Korallus, Christoph; Kielstein, Heike; Gutenbrunner, Christoph

    2013-01-01

    Fibromyalgia syndrome (FMS) patients have multiple symptoms, including mental symptoms such as depression. Natural killer T (NKT) cells have shown to be correlated with depression. However, up to now there is no information regarding the role of CD3+CD56+NKT cells in FMS patients, especially in the intensity of mental symptoms. The present study aimed to observe the role of CD3+CD56+NKT cells level in FMS patients in relation to the intensity of depression. Ninety-six female patients who matched definition of FMS were divided into subgroups of depressions according to Hospital Anxiety and Depression Scale (HADS) score (HADS-Depression 10). CD3+CD56+NKT cells from peripheral blood were measured by fluorescence-activated cell sorting methods. ANOVA test in the subgroup of depression of FMS patient showed significant differences. Additionally, the differences were observed in HADSD 10. The use of antidepressant significantly altered the level of CD3+CD56+NKT cells. A blocking variable ANCOVA with antidepressant as covariate showed main effects in the subgroup of depression, however, the interaction of antidepressant and the subgroup of depression did not influence the level of CD3+CD56+NKT cells. These results suggest that CD3+CD56+NKT cells could play a role as a mediator in mental symptom such as depression in FMS patients. It seems the role of antidepressant in the mood intensity is not mediated by CD3+CD56+NKT cells in FMS patients. Additionally, subgrouping FMS patients based on the intensity of mental symptoms may help to optimise the treatments.

  16. An intense and short-lasting burst of neutrophil activation differentiates early acute myocardial infarction from systemic inflammatory syndromes.

    Directory of Open Access Journals (Sweden)

    Norma Maugeri

    Full Text Available BACKGROUND: Neutrophils are involved in thrombus formation. We investigated whether specific features of neutrophil activation characterize patients with acute coronary syndromes (ACS compared to stable angina and to systemic inflammatory diseases. METHODS AND FINDINGS: The myeloperoxidase (MPO content of circulating neutrophils was determined by flow cytometry in 330 subjects: 69 consecutive patients with acute coronary syndromes (ACS, 69 with chronic stable angina (CSA, 50 with inflammation due to either non-infectious (acute bone fracture, infectious (sepsis or autoimmune diseases (small and large vessel systemic vasculitis, rheumatoid arthritis. Four patients have also been studied before and after sterile acute injury of the myocardium (septal alcoholization. One hundred thirty-eight healthy donors were studied in parallel. Neutrophils with normal MPO content were 96% in controls, >92% in patients undergoing septal alcoholization, 91% in CSA patients, but only 35 and 30% in unstable angina and AMI (STEMI and NSTEMI patients, compared to 80%, 75% and 2% of patients with giant cell arteritis, acute bone fracture and severe sepsis. In addition, in 32/33 STEMI and 9/21 NSTEMI patients respectively, 20% and 12% of neutrophils had complete MPO depletion during the first 4 hours after the onset of symptoms, a feature not observed in any other group of patients. MPO depletion was associated with platelet activation, indicated by P-selectin expression, activation and transactivation of leukocyte β2-integrins and formation of platelet neutrophil and -monocyte aggregates. The injection of activated platelets in mice produced transient, P-selectin dependent, complete MPO depletion in about 50% of neutrophils. CONCLUSIONS: ACS are characterized by intense neutrophil activation, like other systemic inflammatory syndromes. In the very early phase of acute myocardial infarction only a subpopulation of neutrophils is massively activated, possibly via

  17. Coffee consumption and the occurrence and intensity of metabolic syndrome: a cross-sectional study.

    Science.gov (United States)

    Suliga, Edyta; Kozieł, Dorota; Cieśla, Elżbieta; Rębak, Dorota; Głuszek, Stanisław

    2017-06-01

    The objective of the study was to investigate associations between coffee consumption and the occurrence of metabolic syndrome (MetS) and its components in individuals with a normal BMI, as well in those who are overweight and obese. The analysis was based on the data of 10,367 participants. The studies included a questionnaire interview, anthropometric measurements, blood pressure measurements and analyses of collected fasting-blood samples. In the overweight and obese participants, lower coffee consumption, compared with higher consumption was correlated with a significantly higher risk of abdominal obesity, hypertension, an abnormal glucose concentration, HDL cholesterol, triglycerides and MetS (p coffee consumption was related to the abdominal obesity, HDL cholesterol and MetS (p coffee, compared with those with excessive body mass.

  18. [Malignant hyperthermia syndrome in the intensive care unit : Differential diagnosis and acute measures].

    Science.gov (United States)

    Grander, W

    2016-06-01

    Malignant hyperthermia is a life-threatening disease caused by derangement of the autonomic nerve system and hypermetabolism of the peripheral musculature. Commonly body core temperatures of more than 40 °C will be found in this disease which is caused mostly by psychopharmacological drugs like antidepressants, neuroleptics but also antibiotics, pain killers, anti-Parkinson drugs, and volatile anesthetics. The inducers of malignant hyperthermia interact with postsynaptic receptors (serotonin, anticholinergics) or muscular intracellular structures responsible for calcium utilization (volatile anesthetics, succinylcholine). Rarely malignant hyperthermia is a consequence of mental stress or vigorous exercise and or heat. Malignant hyperthermic syndromes lead to a severe dysbalance of the autonomic nerve system accompanied by rhabdomyolysis, disseminated intravascular coagulopathy, and finally multi-organ failure. Accordingly, medical management is primarily directed to stabilize vital functions, withdrawal of the causing drug, and if possible antagonizing toxic substances. The leading symptom hyperthermia needs to be treated physically with available cooling systems.

  19. Applying the ICF to identify requirements for students with Asperger syndrome in higher education.

    Science.gov (United States)

    Adolfsson, Margareta; Simmeborn Fleischer, Ann

    2015-06-01

    Higher education requires more than academic skills and everyday student-life can be stressful. Students with Asperger syndrome (AS) may need support to manage their education due to difficulties in social functioning. As preparation for the development of a structured tool to guide student and coordinator dialogues at Swedish universities, this study aimed to identify ICF categories that reflect requirements in everyday student-life for students with AS. Using descriptive qualitative approach, information in documents reflecting the perspectives of university students, international classifications, user/health organisations and education authorities were linked to ICF codes. In total, 114 ICF categories were identified, most of which related to learning, tasks and demands, communication and interactions. Students with AS need varying accommodations to be successful in higher education. In the future, ICF-based code sets, including demands on student roles, can be used as checklists to describe functioning and needs for support.

  20. Effect of short-term intensive atorvastatin treatment on interventional treatment effect and cardiac function of patients with acute coronary syndrome

    Directory of Open Access Journals (Sweden)

    Yu Lei

    2016-06-01

    Full Text Available Objective: To study the effect of short-term intensive atorvastatin treatment on interventional treatment effect and cardiac function of patients with acute coronary syndrome. Methods: A total of 104 cases of patients with acute coronary syndrome who received PCI treatment in Emergency Department of our hospital from May 2014 to November 2015 were retrospectively analyzed and divided into intensive group and routine group according to different atorvastatin treatment methods, and then biochemical indexes, cardiac ultrasound indicators and inflammatory indexes of two groups were compared. Results: Serum TG, TC, LDL-C, hs- CRP, LDH, α-HBDH, CK and CK-MB content of intensive group were significantly lower than those of routine group while HDL-C content was higher than that of routine group; E/ A ratio and LVEF of intensive group were higher than those of routine group while Tei index, systolic index and diastolic index were lower than those of routine group; TLR4 and NF-kB expression levels in peripheral blood as well as TNF-α and IL-6 content in serum of intensive group were significantly lower than those of routine group. Conclusion: Short-term intensive atorvastatin treatment improves the interventional treatment effect of patients with acute coronary syndrome, and can reduce myocardial injury, improve cardiac diastolic and systolic function and inhibit the inflammation mediated by TLR4/NF-kB.

  1. Patients with oral allergic syndrome to apple have intense proliferative response to BET V 1.

    Science.gov (United States)

    Ciprandi, G; Fenoglio, G; Kalli, F; De Amici, M; Leonardi, S; Miraglia Del Giudice, M; Salpietro, C; La Rosa, M; Caimmi, S; Marseglia, G L

    2012-01-01

    Patients with pollen allergy may frequently present an additional food-related allergy (Oral Allergic Syndrome, OAS), as consequence of cross-reactivity between pollen allergens (mainly birch, hazelnut, alder, mugwort) and vegetable allergens. The aim of this study was to evaluate the effect on Bet v 1-induced T cell proliferation exerted by the presence of OAS in birch patients. Fourteen allergic patients were evaluated (6 males, mean age 35.8 years). All of them were monosensitized to birch and suffered from allergic rhinitis: 4 had also OAS to apple. Proliferation of peripheral mononuclear cells was evaluated using Bet v 1 and non-specific stimuli. OAS had higher proliferation than non-OAS patients. In addition, there were significant relationships between immunological and clinical parameters in OAS patients. This study evidences that OAS characterizes a more severe form of birch allergy: as OAS patients had higher SI, circulating eosinophils, and IgE levels. Thus, this study confirms the previous report and underlines the relevance of measuring recombinant birch allergen as higher values may suggest a reliable prediction of OAS.

  2. [Surgical treatment of Marfan syndrome; analysis of the patients required multiple surgical interventions].

    Science.gov (United States)

    Yamazaki, F; Shimamoto, M; Fujita, S; Nakai, M; Aoyama, A; Chen, F; Nakata, T; Yamada, T

    2002-07-01

    Without treatment, the life expectancy of patients with Marfan syndrome is reduced by the associated cardiovascular abnormalities. In this study, we reviewed our experience of the patients with Marfan syndrome who required multiple surgical interventions to identify the optimal treatment for these patients. Between January 1986 and December 2000, 44 patients with Marfan syndrome were operated on at Shizuoka City Hospital (SCH). Among them, 10 patients (22.7%) underwent multiple surgical interventions. There were 5 male and 5 female patients with a mean age of 40.6 +/- 16.1 years at the initial surgery. Only one patient was operated on at another hospital for his first, second, and third operations. His fourth operation was carried out at SCH. The remaining 9 patients underwent a total of 14 additional surgical procedures at SCH. Computed tomography (CT) scans were taken every 6 months postoperatively, and aortic diameter greater than 60 mm was considered as the indication for the additional surgery. There were no early death and one late death. The causes of additional surgery were enlargement of true aneurysm in 6, enlargement of residual dissection in 4, new dissection in 4, false aneurysm at the coronary anastomosis of Bentall procedure in 1. In 9 patients, both ascending and descending aorta were replaced. Among these 9 patients, only 3 patients underwent total arch replacement, and remaining 6 patients had their arch left in place with or without dissection. Our current strategy of the treatment of Marfan patients with acute type A dissection is total arch replacement with an elephant trunk at the initial emergent surgery.

  3. Tidal Volume Requirement in Mechanically Ventilated Infants with Meconium Aspiration Syndrome.

    Science.gov (United States)

    Sharma, Saumya; Clark, Shane; Abubakar, Kabir; Keszler, Martin

    2015-08-01

    The aim of the study is to test the hypothesis that increased physiologic dead space and functional residual capacity seen in meconium aspiration syndrome (MAS) results in higher tidal volume (VT) requirement to achieve adequate ventilation. Retrospective review of infants with MAS admitted to our hospital from 2000 to 2010 managed with conventional ventilation. Demographics, ventilator settings, VT, respiratory rate (RR), and blood gas values were recorded. Minute ventilation (MV) was calculated as RR × VT. Only VT values with corresponding partial pressure of carbon dioxide (Paco 2) between 35 and 60 mm Hg were included. Mean VT/kg and MV/kg were calculated for each patient. Forty infants ventilated for lung disease other than MAS or pulmonary hypoplasia served as controls. Birth weights of the 28 MAS patients and 40 control infants were similar (3,330 ± 500 g and 3,300 ± 640 g). Two patients in each group required extracorporeal membrane oxygenation. Infants with MAS required 26% higher VT and 42% higher MV compared with controls to maintain equal Paco 2. Infants with MAS require larger VT and higher total MV to achieve similar alveolar ventilation, consistent with pathophysiology of MAS. Our findings provide the first reference data to guide selection of VT in infants with MAS. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

  4. The Energy Required to Produce Materials: Constraints on Energy Intensity Improvements, Parameters of Demand

    NARCIS (Netherlands)

    Gutowski, T.G.; Sahni, S.; Allwood, J.M.; Ashby, M.F.; Worrell, E.|info:eu-repo/dai/nl/106856715

    2013-01-01

    In this paper, we review the energy requirements to make materials on a global scale by focusing on the five construction materials that dominate energy used in material production: steel, cement, paper, plastics and aluminium. We then estimate the possibility of reducing absolute material

  5. The energy requirements of Eurasian perch (Perca fluviatilis L.) in intensive culture

    DEFF Research Database (Denmark)

    Strand, A.; Overton, Julia Lynne; Alanara, A.

    2011-01-01

    Fish feed constitutes one of the largest costs in aquaculture, therefore inefficient feed management will have a negative impact on fish farm economics. Eurasian perch (Perca fluviatilis L.) is a relatively new candidate for freshwater aquaculture, however little is known about the energy require...

  6. CHARGE syndrome

    Directory of Open Access Journals (Sweden)

    Prasad Chitra

    2006-09-01

    Full Text Available Abstract CHARGE syndrome was initially defined as a non-random association of anomalies (Coloboma, Heart defect, Atresia choanae, Retarded growth and development, Genital hypoplasia, Ear anomalies/deafness. In 1998, an expert group defined the major (the classical 4C's: Choanal atresia, Coloboma, Characteristic ears and Cranial nerve anomalies and minor criteria of CHARGE syndrome. Individuals with all four major characteristics or three major and three minor characteristics are highly likely to have CHARGE syndrome. However, there have been individuals genetically identified with CHARGE syndrome without the classical choanal atresia and coloboma. The reported incidence of CHARGE syndrome ranges from 0.1–1.2/10,000 and depends on professional recognition. Coloboma mainly affects the retina. Major and minor congenital heart defects (the commonest cyanotic heart defect is tetralogy of Fallot occur in 75–80% of patients. Choanal atresia may be membranous or bony; bilateral or unilateral. Mental retardation is variable with intelligence quotients (IQ ranging from normal to profound retardation. Under-development of the external genitalia is a common finding in males but it is less apparent in females. Ear abnormalities include a classical finding of unusually shaped ears and hearing loss (conductive and/or nerve deafness that ranges from mild to severe deafness. Multiple cranial nerve dysfunctions are common. A behavioral phenotype for CHARGE syndrome is emerging. Mutations in the CHD7 gene (member of the chromodomain helicase DNA protein family are detected in over 75% of patients with CHARGE syndrome. Children with CHARGE syndrome require intensive medical management as well as numerous surgical interventions. They also need multidisciplinary follow up. Some of the hidden issues of CHARGE syndrome are often forgotten, one being the feeding adaptation of these children, which needs an early aggressive approach from a feeding team. As the child

  7. Droplet size characteristics and energy input requirements of emulsions formed using high-intensity-pulsed electric fields

    International Nuclear Information System (INIS)

    Scott, T.C.; Sisson, W.G.

    1987-01-01

    Experimental methods have been developed to measure droplet size characteristics and energy inputs associated with the rupture of aqueous droplets by high-intensity-pulsed electric fields. The combination of in situ microscope optics and high-speed video cameras allows reliable observation of liquid droplets down to 0.5 μm in size. Videotapes of electric-field-created emulsions reveal that average droplet sizes of less than 5 μm are easily obtained in such systems. Analysis of the energy inputs into the fluids indicates that the electric field method requires less than 1% of the energy required from mechanical agitation to create comparable droplet sizes. 11 refs., 3 figs., 2 tabs

  8. Effects of High Intensity Interval Training and Strength Training on Metabolic, Cardiovascular and Hormonal Outcomes in Women with Polycystic Ovary Syndrome: A Pilot Study.

    Directory of Open Access Journals (Sweden)

    Ida Almenning

    Full Text Available Polycystic ovary syndrome is a common endocrinopathy in reproductive-age women, and associates with insulin resistance. Exercise is advocated in this disorder, but little knowledge exists on the optimal exercise regimes. We assessed the effects of high intensity interval training and strength training on metabolic, cardiovascular, and hormonal outcomes in women with polycystic ovary syndrome.Three-arm parallel randomized controlled trial. Thirty-one women with polycystic ovary syndrome (age 27.2 ± 5.5 years; body mass index 26.7 ± 6.0 kg/m2 were randomly assigned to high intensity interval training, strength training, or a control group. The exercise groups exercised three times weekly for 10 weeks.The main outcome measure was change in homeostatic assessment of insulin resistance (HOMA-IR. HOMA-IR improved significantly only after high intensity interval training, by -0.83 (95% confidence interval [CI], -1.45, -0.20, equal to 17%, with between-group difference (p = 0.014. After high intensity interval training, high-density lipoprotein cholesterol increased by 0.2 (95% CI, 0.02, 0.5 mmol/L, with between group difference (p = 0.04. Endothelial function, measured as flow-mediated dilatation of the brachial artery, increased significantly after high intensity interval training, by 2.0 (95% CI, 0.1, 4.0 %, between-group difference (p = 0.08. Fat percentage decreased significantly after both exercise regimes, without changes in body weight. After strength training, anti-Müllarian hormone was significantly reduced, by -14.8 (95% CI, -21.2, -8.4 pmol/L, between-group difference (p = 0.04. There were no significant changes in high-sensitivity C-reactive protein, adiponectin or leptin in any group.High intensity interval training for ten weeks improved insulin resistance, without weight loss, in women with polycystic ovary syndrome. Body composition improved significantly after both strength training and high intensity interval training. This pilot

  9. Effects of High Intensity Interval Training and Strength Training on Metabolic, Cardiovascular and Hormonal Outcomes in Women with Polycystic Ovary Syndrome: A Pilot Study.

    Science.gov (United States)

    Almenning, Ida; Rieber-Mohn, Astrid; Lundgren, Kari Margrethe; Shetelig Løvvik, Tone; Garnæs, Kirsti Krohn; Moholdt, Trine

    2015-01-01

    Polycystic ovary syndrome is a common endocrinopathy in reproductive-age women, and associates with insulin resistance. Exercise is advocated in this disorder, but little knowledge exists on the optimal exercise regimes. We assessed the effects of high intensity interval training and strength training on metabolic, cardiovascular, and hormonal outcomes in women with polycystic ovary syndrome. Three-arm parallel randomized controlled trial. Thirty-one women with polycystic ovary syndrome (age 27.2 ± 5.5 years; body mass index 26.7 ± 6.0 kg/m2) were randomly assigned to high intensity interval training, strength training, or a control group. The exercise groups exercised three times weekly for 10 weeks. The main outcome measure was change in homeostatic assessment of insulin resistance (HOMA-IR). HOMA-IR improved significantly only after high intensity interval training, by -0.83 (95% confidence interval [CI], -1.45, -0.20), equal to 17%, with between-group difference (p = 0.014). After high intensity interval training, high-density lipoprotein cholesterol increased by 0.2 (95% CI, 0.02, 0.5) mmol/L, with between group difference (p = 0.04). Endothelial function, measured as flow-mediated dilatation of the brachial artery, increased significantly after high intensity interval training, by 2.0 (95% CI, 0.1, 4.0) %, between-group difference (p = 0.08). Fat percentage decreased significantly after both exercise regimes, without changes in body weight. After strength training, anti-Müllarian hormone was significantly reduced, by -14.8 (95% CI, -21.2, -8.4) pmol/L, between-group difference (p = 0.04). There were no significant changes in high-sensitivity C-reactive protein, adiponectin or leptin in any group. High intensity interval training for ten weeks improved insulin resistance, without weight loss, in women with polycystic ovary syndrome. Body composition improved significantly after both strength training and high intensity interval training. This pilot study

  10. Obstetric Patients Requiring Intensive Care: A One Year Retrospective Study in a Tertiary Care Institute in India

    Directory of Open Access Journals (Sweden)

    Niyaz Ashraf

    2014-01-01

    Full Text Available Background and Objectives. Critically ill obstetric patients are a particularly unique cohort for the intensivist. The objective of this study was to review the indications for admission, demographics, clinical characteristics, and outcomes of obstetric patients admitted to intensive care unit of a medical college hospital in southern India and to identify conditions associated with maternal mortality. Design. Retrospective analysis of pregnant/postpartum (up to 6 weeks admissions over a 1-year result. We studied 55 patients constituting 11.6% of mixed ICU admissions during the study period. Results. The mean APACHE (acute physiology and chronic health evaluation II score of patients at admission was 11.8. Most of the patients (76% were admitted in the antepartum period. The commonest indications for ICU admission were obstetric haemorrhage (51% and hypertensive disorders of pregnancy (18%. 85% of patients required mechanical ventilation and 78% required inotropic support. Conclusions. Maternal mortality was 13%, and the majority of the deaths were due to disseminated intravascular coagulation and multiorgan failure, following an obstetric haemorrhage. A dedicated obstetric ICU in tertiary hospitals can ensure that there is no delay in patient management and intensive care can be instituted at the earliest.

  11. The role of burnout syndrome as a mediator for the effect of psychosocial risk factors on the intensity of musculoskeletal disorders: a structural equation modeling approach.

    Science.gov (United States)

    Gholami, Tahereh; Pahlavian, Ahmad Heidari; Akbarzadeh, Mahdi; Motamedzade, Majid; Moghaddam, Rashid Heidari

    2016-01-01

    This study examined the hypothesis that burnout syndrome mediates effects of psychosocial risk factors and intensity of musculoskeletal disorders (MSDs) among hospital nurses. The sample was composed of 415 nurses from various wards across five hospitals of Iran's Hamedan University of Medical Sciences. Data were collected through three questionnaires: job content questionnaire, Maslach burnout inventory and visual analogue scale. Results of structural equation modeling with a mediating effect showed that psychosocial risk factors were significantly related to changes in burnout, which in turn affects intensity of MSDs.

  12. Impact of GnRH agonist triggering and intensive luteal steroid support on live-birth rates and ovarian hyperstimulation syndrome

    DEFF Research Database (Denmark)

    Iliodromiti, Stamatina; Lan, Vuong Thi Ngoc; Tuong, Ho Manh

    2013-01-01

    Conventional luteal support packages are inadequate to facilitate a fresh transfer after GnRH agonist (GnRHa) trigger in patients at high risk of developing ovarian hyperstimulation syndrome (OHSS). By providing intensive luteal-phase support with oestradiol and progesterone satisfactory implanta...... implantation rates can be sustained. The objective of this study was to assess the live-birth rate and incidence of OHSS after GnRHa trigger and intensive luteal steroid support compared to traditional hCG trigger and conventional luteal support in OHSS high risk Asian patients....

  13. [Frequency and maternal complications of the criteria of hemolysis in preeclamptic patients with HELLP syndrome treated in an intensive care unit].

    Science.gov (United States)

    Vázquez-Rodríguez, J G; Ríos-Gutiérrez, C D; Paredes-Lozano, E P; García-Flores, A

    2016-01-01

    HELLP syndrome is an aggressive form of preeclampsia related with hemolysis and its complications. To determine the frequency of the appearance of criteria of hemolysis and maternal complications in preeclamptic patients with HELLP syndrome treated in an intensive care unit. We carried out a cross-sectional study in 50 preeclamptic women with HELLP syndrome admitted to intensive care unit to determine the presence of the following criteria of hemolysis: peripheral blood schistocytes, anemia (hemoglobin ≤ 10 g/dL), lactate dehydrogenase ≥ 600 U/L, indirect bilirubin ≥ 0.6 mg/dL and hemoglobinuria. We also studied maternal complications in patients with lactate dehydrogenase ≥ 600 U/L and positive for schistocytes. Descriptive (mean, median, range, standard deviation) and inferential (Student t test) statistics were used. Lactate dehydrogenase ≥ 600 U/L was found in 36%, indirect bilirubin 0.6 mg/dL in 20%, positive schistocytes in 16%, hemoglobinuria in 4% and anemia in 0%. Patients with lactate dehydrogenase ≥ 600 U/L had more clinical and laboratory deterioration as well as prolonged intensive care unit stay (p = 0.0025). Patients positive for schistocytes did not demonstrate adverse effects. Biochemical criteria of hemolysis were more frequent than schistocytes, hemoglobinuria and anemia. Patients with lactate dehydrogenase ≥ 600 U/L was alterations more serious than patients with positive schistocytes so its usefulness as a biomarker may be higher.

  14. Timing of low tidal volume ventilation and intensive care unit mortality in acute respiratory distress syndrome. A prospective cohort study.

    Science.gov (United States)

    Needham, Dale M; Yang, Ting; Dinglas, Victor D; Mendez-Tellez, Pedro A; Shanholtz, Carl; Sevransky, Jonathan E; Brower, Roy G; Pronovost, Peter J; Colantuoni, Elizabeth

    2015-01-15

    Reducing tidal volume decreases mortality in acute respiratory distress syndrome (ARDS). However, the effect of the timing of low tidal volume ventilation is not well understood. To evaluate the association of intensive care unit (ICU) mortality with initial tidal volume and with tidal volume change over time. Multivariable, time-varying Cox regression analysis of a multisite, prospective study of 482 patients with ARDS with 11,558 twice-daily tidal volume assessments (evaluated in milliliter per kilogram of predicted body weight [PBW]) and daily assessment of other mortality predictors. An increase of 1 ml/kg PBW in initial tidal volume was associated with a 23% increase in ICU mortality risk (adjusted hazard ratio, 1.23; 95% confidence interval [CI], 1.06-1.44; P = 0.008). Moreover, a 1 ml/kg PBW increase in subsequent tidal volumes compared with the initial tidal volume was associated with a 15% increase in mortality risk (adjusted hazard ratio, 1.15; 95% CI, 1.02-1.29; P = 0.019). Compared with a prototypical patient receiving 8 days with a tidal volume of 6 ml/kg PBW, the absolute increase in ICU mortality (95% CI) of receiving 10 and 8 ml/kg PBW, respectively, across all 8 days was 7.2% (3.0-13.0%) and 2.7% (1.2-4.6%). In scenarios with variation in tidal volume over the 8-day period, mortality was higher when a larger volume was used earlier. Higher tidal volumes shortly after ARDS onset were associated with a greater risk of ICU mortality compared with subsequent tidal volumes. Timely recognition of ARDS and adherence to low tidal volume ventilation is important for reducing mortality. Clinical trial registered with www.clinicaltrials.gov (NCT 00300248).

  15. Effect of Means of Problem-Oriented Physical Rehabilitation on Pain and Tactile Sensitivity and Pain Syndrome Intensity in Women with Post-Mastectomy Syndrome

    Directory of Open Access Journals (Sweden)

    Т. Є. Одинець

    2015-03-01

    Full Text Available The objective of the paper is to determine the effectiveness of problem-oriented physical rehabilitation of women with post-mastectomy syndrome in terms of normalization of their sensitivity and lessening of the pain syndrome. Materials and methods. The paper provides a review of the related literary sources and empirical data analyzed and summarized, offers definitions of pain by the Visual Analogue Scale, McGill Pain Questionnaire and the Verbal Rating Scale, evaluates tactile and pain sensitivity, and uses the methods of mathematical statistics. The participants in the study were 50 women with diagnosed with the post-mastectomy syndrome and at the stage of residential treatment. Results: The developed problem-oriented physical rehabilitation experimentally proved effective by showing improvements in tactile and pain sensitivity, and pain lessening by the sensory, affective and rating scales in women with post-mastectomy syndrome at the stage of residential treatment.

  16. Transcription is required to establish maternal imprinting at the Prader-Willi syndrome and Angelman syndrome locus.

    Directory of Open Access Journals (Sweden)

    Emily Y Smith

    2011-12-01

    Full Text Available The Prader-Willi syndrome (PWS [MIM 17620] and Angelman syndrome (AS [MIM 105830] locus is controlled by a bipartite imprinting center (IC consisting of the PWS-IC and the AS-IC. The most widely accepted model of IC function proposes that the PWS-IC activates gene expression from the paternal allele, while the AS-IC acts to epigenetically inactivate the PWS-IC on the maternal allele, thus silencing the paternally expressed genes. Gene order and imprinting patterns at the PWS/AS locus are well conserved from human to mouse; however, a murine AS-IC has yet to be identified. We investigated a potential regulatory role for transcription from the Snrpn alternative upstream exons in silencing the maternal allele using a murine transgene containing Snrpn and three upstream exons. This transgene displayed appropriate imprinted expression and epigenetic marks, demonstrating the presence of a functional AS-IC. Transcription of the upstream exons from the endogenous locus correlates with imprint establishment in oocytes, and this upstream exon expression pattern was conserved on the transgene. A transgene bearing targeted deletions of each of the three upstream exons exhibited loss of imprinting upon maternal transmission. These results support a model in which transcription from the Snrpn upstream exons directs the maternal imprint at the PWS-IC.

  17. Acute renal failure requiring renal replacement therapy in the intensive care unit: impact on prognostic assessment for shared decision making.

    Science.gov (United States)

    Johnson, Robert F; Gustin, Jillian

    2011-07-01

    A 69-year-old female was receiving renal replacement therapy (RRT) for acute renal failure (ARF) in an intensive care unit (ICU). Consultation was requested from the palliative medicine service to facilitate a shared decision-making process regarding goals of care. Clinician responsibility in shared decision making includes the formulation and expression of a prognostic assessment providing the necessary perspective for a spokesperson to match patient values with treatment options. For this patient, ARF requiring RRT in the ICU was used as a focal point for preparing a prognostic assessment. A prognostic assessment should include the outcomes of most importance to a discussion of goals of care: mortality risk and survivor functional status, in this case including renal recovery. A systematic review of the literature was conducted to document published data regarding these outcomes for adult patients receiving RRT for ARF in the ICU. Forty-one studies met the inclusion criteria. The combined mean values for short-term mortality, long-term mortality, renal-function recovery of short-term survivors, and renal-function recovery of long-term survivors were 51.7%, 68.6%, 82.0%, and 88.4%, respectively. This case example illustrates a process for formulating and expressing a prognostic assessment for an ICU patient requiring RRT for ARF. Data from the literature review provide baseline information that requires adjustment to reflect specific patient circumstances. The nature of the acute primary process, comorbidities, and severity of illness are key modifiers. Finally, the prognostic assessment is expressed during a family meeting using recommended principles of communication.

  18. Multidisciplinary care in the intensive care unit for a patient with Prader-Willi syndrome: a dental approach.

    Science.gov (United States)

    Setti, Juliana Santiago; Pinto, Sérgio Felix; Gaetti-Jardim, Ellen Cristina; Manrique, Gustavo Rodrigues; Mendonça, José Carlos Garcia de

    2012-03-01

    Prader-Willi syndrome is a genetic neurobehavioral disease affecting children's development and resulting in obesity, reduced height, hypotonia, endocrine disorders and cognitive deficits, which may impair oral integrity. This study aims to report on a case involving a white male 15-year-old patient with Prader-Willi syndrome whose oral examination revealed bacterial plaque, gingivitis, poor occlusion, viscous salivation and multiple lip, jugal mucosa, inserted gum and tongue ulcerations. An excision biopsy revealed oral ulcerations typical of herpes, which were considered to be likely to correlate with herpes encephalitis. This result demonstrates that a large portion of the deleterious effects of Prader-Willi syndrome can be attenuated by appropriate diagnosis and early therapeutic intervention, highlighting the role of an integrated multidisciplinary team in the development of therapeutic protocols for Prader-Willi syndrome patients.

  19. The impact of architectural design upon the environmental sound and light exposure of neonates who require intensive care: an evaluation of the Boekelheide Neonatal Intensive Care Nursery.

    Science.gov (United States)

    Stevens, D C; Akram Khan, M; Munson, D P; Reid, E J; Helseth, C C; Buggy, J

    2007-12-01

    To evaluate the differences in environmental sound, illumination and physiological parameters in the Boekelheide Neonatal Intensive Care Unit (BNICU), which was designed to comply with current recommendations and standards, as compared with a conventional neonatal intensive care unit (CNICU). Prospectively designed observational study. Median sound levels in the unoccupied BNICU (37.6 dBA) were lower than the CNICU (42.1 dBA, P<0.001). Median levels of minimum (6.4 vs 48.4 lux, P<0.05) and maximum illumination (357 vs 402 lux, P<0.05) were lower in the BNICU. A group of six neonates delivered at 32 weeks gestation showed significantly less periodic breathing (14 vs 21%) and awake time (17.6 vs 29.3%) in the BNICU as compared to the CNICU. Light and sound were both significantly reduced in the BNICU. Care in the BNICU was associated with improved physiological parameters.

  20. Liver transplantation in patients with end-stage liver disease requiring intensive care unit admission and intubation.

    Science.gov (United States)

    Knaak, Jan; McVey, Mark; Bazerbachi, Fateh; Goldaracena, Nicolás; Spetzler, Vinzent; Selzner, Nazia; Cattral, Mark; Greig, Paul; Lilly, Les; McGilvray, Ian; Levy, Gary; Ghanekar, Anand; Renner, Eberhard; Grant, David; Hawryluck, Laura; Selzner, Markus

    2015-06-01

    Data regarding transplantation outcomes in ventilated intensive care unit (ICU)-dependent patients with end-stage liver disease (ESLD) are conflicting. This single-center cohort study investigated the outcomes of patients with ESLD who were intubated with mechanical support before liver transplantation (LT). The ICU plus intubation group consisted of 42 patients with decompensated cirrhosis and mechanical ventilation before transplantation. LT was considered for intubated ICU patients if the fraction of inspired oxygen was ≤40% with a positive end-expiratory pressure ≤ 10, low pressor requirements, and the absence of an active infection. Intubated ICU patients were compared to 80 patients requiring ICU admission before transplantation without intubation and to 126 matched non-ICU-bound patients. Patients requiring ICU care with intubation and ICU care alone had more severe postoperative complications than non-ICU-bound patients. Intubation before transplantation was associated with more postoperative pneumonias (15% in intubated ICU transplant candidates, 5% in ICU-bound but not intubated patients, and 3% in control group patients; P = 0.02). Parameters of reperfusion injury and renal function on postoperative day (POD) 2 and POD 7 were similar in all groups. Bilirubin levels were higher in the ICU plus intubation group at POD 2 and POD 7 after transplantation but were normalized in all groups within 3 months. The ICU plus intubation group versus the ICU-only group and the non-ICU group had decreased 1-, 3-, and 5-year graft survival (81% versus 84% versus 92%, 76% versus 78% versus 87%, and 71% versus 77% versus 84%, respectively; P = 0.19), but statistical significance was not reached. A Glasgow coma scale score of 7 before transplantation was associated with high postoperative mortality in ICU-bound patients requiring intubation (38% versus 23%; P = 0.01). In conclusion, ICU admission and mechanical ventilation should not be considered

  1. Epidemiology and risk factors for severe respiratory syncytial virus infections requiring pediatric intensive care admission in Hong Kong children.

    Science.gov (United States)

    Leung, T F; Lam, D S Y; Miu, T Y; Hon, K L; Chau, C S K; Ku, S W; Lee, R S Y; Chow, P Y; Chiu, W K; Ng, D K K

    2014-04-01

    Respiratory syncytial virus (RSV) is a common cause of lower respiratory tract infection in young children. However, there are limited data on severe RSV infection requiring pediatric intensive care unit (PICU) admission. This retrospective study described features of RSV-associated PICU admissions in Hong Kong and investigated factors for mortality and duration of PICU stay. Children with laboratory-confirmed RSV infection and admitted to the PICUs of all eight government hospitals in Hong Kong between January 2009 and June 2011 were identified from computerized auditing systems and PICU databases. RSV in respiratory samples was detected by direct immunofluorescence and/or viral culture. The relationships between mortality and PICU duration and demographic and clinical factors were analyzed. A total of 118 (2.4 %) PICU admissions were identified among 4,912 RSV-positive pediatric cases in all hospitals. Sixty-five (55.6 %) patients were infants. PICU admissions were higher between October and March. Eight (6.8 %) patients died, but only two were infants. RSV-associated mortality was related to prior sick contact, presence of older siblings, neurodevelopmental conditions, chromosomal and genetic diseases, and bacterial co-infections, but none was significant following logistic regression analyses (odds ratio 9.36, 95 % confidence interval 0.91-96.03 for prior sick contact, p = 0.060). Chronic lung disease was the only risk factor for the duration of PICU admission (β = 0.218, p = 0.017). The majority of RSV-infected children do not require PICU support. There is winter seasonality for RSV-associated PICU admission in Hong Kong. Prior sick contact is the only risk factor for RSV-associated mortality, whereas the presence of chronic lung disease is associated with longer PICU stay. The current risk-based approach of RSV prophylaxis may not be effective in reducing severe RSV infections.

  2. Acute kidney injury in bariatric surgery patients requiring intensive care admission: a state-wide, multicenter, cohort study.

    Science.gov (United States)

    Morgan, David J R; Ho, Kwok M

    2015-01-01

    A multidisciplinary bariatric surgical approach is currently the most effective treatment for obesity. However, little is known about how the physiologic impact of weight reduction surgery superimposed on premorbid obesity-related co-morbidities may adversely influence perioperative renal function. This observational, multicenter study investigated all bariatric surgery patients (n = 590) admitted to any intensive care unit (ICU) in Western Australia between 2007 and 2011. Using Acute Kidney Injury Network (AKIN) criteria, we ascertained the incidence and contributing risk factors for acute kidney injury (AKI). Acute kidney injury (AKI) occurred in 103 patients, accounting for 17.5% of all ICU admissions after bariatric surgery with 76.8% of the AKI episodes limited to AKIN stage 1. In a multivariate analysis, male gender, premorbid hypertension, higher admission APACHE II scores, and blood transfusions were all associated with AKI, while preexisting chronic kidney disease and body mass index (BMI) appeared not to influence renal decline. Both ICU (6.7 versus 2.5 d, Ppatients required hemodialysis while both ICU mortality (2.9 versus 0%, P = .005) and long-term mortality (18.2 versus 4.7 deaths per 1000 bariatric patient-yr, P = .01) were greater in patients experiencing AKI. AKI is common in bariatric patients requiring critical care support leading to increased healthcare utilization, prolonged hospitalization, and is associated with a higher mortality. BMI, a previously described risk factor, was not predictive of AKI in this cohort. Copyright © 2015 American Society for Bariatric Surgery. Published by Elsevier Inc. All rights reserved.

  3. Immediate postoperative pain in orthopedic patients is more intense and requires more analgesia than in post-laparotomy patients.

    Science.gov (United States)

    Ekstein, Margaret P; Weinbroum, Avi A

    2011-02-01

    To compare the immediate postoperative pain intensity between orthopedic and general surgery patients and evaluate the extent of severe pain in each group. Observational, open-label study. Post-anesthesia care unit (PACU) in a tertiary, university-affiliated hospital. Patients undergoing orthopedic surgery or laparotomy under general anesthesia over a one-year period. Follow-up of patient self-rated pain visual analog scale (VAS, 0-10), and observation of the efficacy of the routine analgesic protocol of morphine, ketamine, and diclofenac administration in the PACU. We followed pain scores and sorted patients according to morphine requirements during the PACU immediate postoperative stay. Patients whose pain was controlled with ≤120 µg/kg intravenous morphine were considered pain-controllable. Where this amount was insufficient to control pain (VAS ≥5/10), patients were categorized as suffering from severe pain. They were further treated with repeated doses of 1 mg morphine plus 350 µg/kg ketamine (M+K) and eventually diclofenac. PACU follow-up lasted 3 hours. The overall rate of immediate severe postoperative pain within the entire cohort (3,460 patients) was 9.4%: 123 (6.6%) of laparotomy patients and 202 (12.7%) of orthopedic patients. Pain in the laparotomy patients identified as suffering from severe pain was controlled with 1.21±0.45 doses of M+K compared with 1.37±0.62 (Ppostoperative pain. They required more analgesia than that dictated by existing PACU analgesia protocols. Ketamine and morphine co-administration proved effective in controlling severe postoperative pain after each type of surgery. Wiley Periodicals, Inc.

  4. Routine Admission to Intensive Care Unit After Cytoreductive Surgery and Heated Intraperitoneal Chemotherapy: Not Always a Requirement.

    Science.gov (United States)

    Mogal, Harveshp D; Levine, Edward A; Fino, Nora F; Obiora, Chukwuemeka; Shen, Perry; Stewart, John H; Votanopoulos, Konstantinos I

    2016-05-01

    Routine postoperative intensive care unit (ICU) observation of patients undergoing cytoreductive surgery (CRS) and heated intraperitoneal chemotherapy (HIPEC) is driven by historically reported morbidity and mortality data. The validity of this practice and the criteria for ICU admission have not been elucidated. A prospectively maintained database of 1146 CRS/HIPEC procedures performed from December 1991 to 2014 was retrospectively analyzed. Patients with routine postoperative ICU admission were compared with patients sent directly to the surgical floor. To test the safety of non-ICU care practice, patients with less than 48 h ICU admission were compared with patients directly admitted to the floor. Demographics, primary tumor site, comorbidities, estimated blood loss (EBL), extent of CRS, Eastern Cooperative Oncology Group (ECOG) status, and overall survival were analyzed. Complete data were available for 1064 CRS/HIPEC procedures, of which 244 cases (22.93 %) did not require ICU admission. Multivariate logistic regression identified age [odds ratio (OR) 1.024; p = 0.02], EBL (OR 1.002; p  2 (OR 6.387; p = 0.003) as predictive variables of postoperative ICU admission. The cohort directly admitted to the floor demonstrated less minor grade I/II morbidity (29 vs. 47 %; p observation is not routinely required for all patients treated with CRS/HIPEC. Selective ICU admission based on ECOG status, nutritional status, age, EBL, and CRS extent is safe, with potential implications for hospitalization cost for these complex cases.

  5. Antenatal consultation for parents whose child may require admission to neonatal intensive care: a focus group study for media design.

    Science.gov (United States)

    von Hauff, Patrick; Long, Karen; Taylor, Barbara; van Manen, Michael A

    2016-05-14

    For parents whose child may require admission to a neonatal intensive care unit (NICU), the antenatal consultation is often their first point of contact with the child's medical team. Consultation challenges health professionals, as parents may be anxious, overwhelmed, or even exhausted by what is and what might occur. Despite consultation being a common practice, there is a paucity of research on how to support practitioners and parents. The purpose of this study was to gain insights into important relational aspects of antenatal consultation that may be used to spur the development of media to support consultation. Focus group, as a data collection method, was employed to gather insights about antenatal consultation from a total of 50 hospital staff and 17 NICU parents from a large urban NICU program in western Canada. Qualitative content analysis was applied to the obtained materials to explicate themes that may serve as necessary understandings for media design. Participating hospital staff and parents expressed their desire for a good antenatal consultation with comments grouped under the following themes: supporting the building of a caring relation; sharing information in conversation; and, preparing for what is to come. To support the emerging relations of baby, parent, and hospital staff, a good antenatal consultation needs to convey care, understanding, and empathy; create possibilities for open and genuine conversations; and, foster the buildings of respect, confidence, and trust.

  6. The effect of atorvastatin on pancreatic beta cell requirement in women with polycystic ovary syndrome

    Directory of Open Access Journals (Sweden)

    Thozhukat Sathyapalan

    2017-11-01

    Full Text Available Background: There is an increased risk of developing T2DM in women with polycystic ovary syndrome (PCOS, and there is evidence that statins improve metabolic parameters in these patients. However, there are some data to show that statins increase the risk of incipient diabetes. Materials and methods: We have previously shown that 12 weeks of atorvastatin improves insulin resistance when measured using HOMA-IR. This post hoc analysis was designed to look at the effect of atorvastatin on pancreatic β cell function using HOMA-β in the same study. In this randomised, double-blind placebo controlled study, 40 medication-naïve patients with PCOS were randomised to either atorvastatin 20 mg daily or placebo for 3 months. A 3-month extension study for both groups of patients was undertaken with metformin 1500 mg daily after completing initial 3 months of atorvastatin or placebo. Results: There was a significant reduction in HOMA-β (240 ± 3.2 vs 177 ± 2.3; P value <0.01 after 12 weeks of atorvastatin treatment, which was maintained by metformin in the subsequent 12 weeks. There were no changes in HOMA-β after the placebo or after subsequent metformin treatment. There was no linear correlation between reduction in HOMA-β with improvement of free androgen index (FAI (r2 = 0.02; P = 0.72, testosterone (r2 = 0.13; P = 0.49, SHBG (r2 = 0.22; P = 0.48, hsCRP (r2 = 0.19; P = 0.64, triglycerides (r2 = 0.09; P = 0.12, total cholesterol (r2 = 0.11; P = 0.32 or LDL-C (r2 = 0.19; P = 0.38. Conclusion: Treatment with atorvastatin for 12 weeks in women with PCOS significantly reduced HOMA-β. This could be potentially due to fall in β-cell requirement with improvement of insulin resistance rather than a reduction of β-cell function.

  7. Papillon-Lefevre syndrome patient reveals species-dependent requirements for neutrophil defenses

    DEFF Research Database (Denmark)

    Sorensen, O. E.; Clemmensen, S. N.; Dahl, S. L.

    2014-01-01

    Papillon-Lefevre syndrome (PLS) results from mutations that inactivate cysteine protease cathepsin C (CTSC), which processes a variety of serine proteases considered essential for antimicrobial defense. Despite serine protease-deficient immune cell populations, PLS patients do not exhibit marked ...

  8. Prevalence of augmented renal clearance and performance of glomerular filtration estimates in Indigenous Australian patients requiring intensive care admission.

    Science.gov (United States)

    Tsai, D; Udy, A A; Stewart, P C; Gourley, S; Morick, N M; Lipman, J; Roberts, J A

    2018-01-01

    Augmented renal clearance (ARC) refers to the enhanced renal excretion of circulating solute commonly demonstrated in numerous critically ill subgroups. This study aimed to describe the prevalence of ARC in critically ill Indigenous Australian patients and explore the accuracy of commonly employed mathematical estimates of glomerular filtration. We completed a single-centre, prospective, observational study in the intensive care unit (ICU), Alice Springs Hospital, Central Australia. Participants were critically ill adult Indigenous and non-Indigenous Australian patients with a urinary catheter in situ. Exclusion criteria were anuria, pregnancy or the requirement for renal replacement therapy. Daily eight-hour measured creatinine clearances (CrCLm) were collected throughout the ICU stay. ARC was defined by a CrCLm ≥130 ml/min/1.73 m2. The Cockcroft-Gault and Chronic Kidney Disease Epidemiology Collaboration equations were also used to calculate mathematical estimates for comparison. In total, 131 patients were recruited (97 Indigenous, 34 non-Indigenous) and 445 samples were collected. The median (range) CrCLm was 93.0 (5.14 to 205.2) and 90.4 (18.7 to 206.8) ml/min/1.73 m2 in Indigenous and non-Indigenous patients, respectively. Thirty-one of 97 (32%) Indigenous patients manifested ARC, compared to 7 of 34 (21%) non-Indigenous patients (P=0.21). Younger age, major surgery, higher baseline renal function and an absence of diabetes were all associated with ARC. Both mathematical estimates manifest limited accuracy. ARC was prevalent in critically ill Indigenous patients, which places them at significant risk of underdosing with renally excreted drugs. CrCLm should be obtained wherever possible to ensure accurate dosing.

  9. Emotion Perception in Asperger's Syndrome and High-Functioning Autism: The Importance of Diagnostic Criteria and Cue Intensity

    Science.gov (United States)

    Mazefsky, Carla A.; Oswald, Donald P.

    2007-01-01

    This study compared emotion perception accuracy between children with Asperger's syndrome (AS) and high-functioning autism (HFA). Thirty children were diagnosed with AS or HFA based on empirically supported diagnostic criteria and administered an emotion perception test consisting of facial expressions and tone of voice cues that varied in…

  10. Measuring Physical Activity Intensity

    Medline Plus

    Full Text Available ... activity: relative intensity and absolute intensity. Relative Intensity The level of effort required by a person to do an activity. When using relative intensity, people pay attention to how physical activity affects their heart rate ...

  11. Comparison of the effects of knee and hip and single knee muscles strengthening/ stretching exercises on pain intensity and function in athletes with patellofemoral pain syndrome

    Directory of Open Access Journals (Sweden)

    Vahid Mazloum

    2016-08-01

    Full Text Available Background: Patellofemoral pain syndrome (PFPS is a common musculoskeletal condition among athletes. The evidence emphasizes on the importance of hip musculature strengthening exercises for such patients. Objective: To investigate the effects of strengthening-stretching knee muscles exercises and hip posterolateral musculature exercises in athletes with PFPS. Methods: In this clinical trial, 28 athletes with age average of 22.7±2.4 years with PFPS were allocated into conventional knee muscles exercises (CKME (n=14 and posterolateral hip muscles exercises (PHME (n=14. The subjects of both groups performed the supervised exercise protocols in 12 sessions. The Visual Analogue Scale and 6-minute walking tests were administrated respectively to evaluate pain intensity and function. The data were analyzed using Shapiro-wilk test, Independent-sample t test, and Repeated Measure ANOVA test. Findings: Demographic, pain intensity, and physical function data were similar between groups at baseline. Both groups significantly improved in pain intensity and function following a 4-week exercise program. Additionally, the athletes in PHME group had higher level of decreased pain intensity and improved function in follow-up assessment than the subjects in CKME group. Conclusion: Using hip posterolateral musculature exercises in addition to the knee conventional exercises is more effective for athletes with PFPS.

  12. The Effectiveness of Cognitive Behavioral Therapy on Symptoms Intensity, Quality of Life, and Mental Health in Patients with Irritable Bowel Syndrome

    Directory of Open Access Journals (Sweden)

    Amrollah Ebrahimi

    2015-12-01

    Full Text Available Background: Irritable bowel syndrome (IBS is a functional gastrointestinal disorder with chronic abdominal pain, bowel habit variations, and lack of structural causes. Symptom intensity has a statistical relation with patients' quality of life (QOL and mental health. The first objective of the present study was to develop and provide a therapeutic plan based on cognitive behavioral therapy (CBT for IBS that was operated for the very first time in Iran. The second objective was to determine the effectiveness of these treatments on IBS symptoms intensity, health-related QOL, and psychological health among patients with IBS.Methods: The participants were 15 women with IBS. The participants were diagnosed on the basis of ROME-III diagnosis criteria. The data collection tools consisted of IBS Symptom Severity Scale (IBS-SSS, the Irritable Bowel Syndrome Quality of Life (IBS-QOL questionnaire, and the Symptom Checklist-90-Revised (SCL-90-R used to evaluate mental health. Data were collected during the weeks of 0, 4, 12, and 24, during the treatment process. The extracted data was examined statistically via repeated measures MANOVA in SPSS software.Results: CBT has a significant effect on IBS symptoms reduction, QOL improvement, and mental health promotion of the patients. The effect of the therapeutic plan persisted until the follow-up stage.Conclusion: According to the results, applied CBT can be specifically implemented as an effective treatment for IBS. Therefore, the use of this treatment is advised.

  13. Delirium the under-recognised syndrome: survey of healthcare professionals' awareness and practice in the intensive care units.

    Science.gov (United States)

    Selim, Abeer A; Wesley Ely, E

    2017-03-01

    To survey intensive care unit healthcare professionals' awareness and practice related to delirium. Despite the current evidence revealing the risks linked to delirium and advances in practice guidelines promoting delirium assessment, healthcare professionals show little sensitivity towards delirium and evident training needs. The study had a cross-sectional survey design. A sample of 168 intensive care unit healthcare professionals including nurses and physicians completed a semistructured questionnaire to survey their awareness, screening and management of delirium in intensive care units. The survey took place at 11 intensive care units from academic (university) and nonacademic (nonuniversity) governmental hospitals in Mansoura, Egypt. The mean score of delirium awareness was 64·4 ± 14·0 among intensive care unit healthcare professionals. Awareness of delirium was significantly lower when definition of delirium was not provided, among diploma nurses compared to bachelor degree nurses and physicians, among those who did not attend any workshop/lecture or read an article related to delirium and lastly, those who work in an intensive care unit when delirium. The survey found that only 26·8% of the healthcare professionals screen for delirium on a routine basis, and 14·3% reported attending workshops or lectures or reading an article related to delirium in the last year. In screening delirium, healthcare professionals did not use any tools, nor did they follow adopted protocols or guidelines to manage delirium. To manage delirium, 52·4% of the participants reported using sedatives, 36·9% used no drugs, and 10·7% reported using antipsychotics (primarily haloperidol). Intensive care unit healthcare professionals do not have adequate training or routine screening of delirium. There is an evident absence of using standardised tools or adapting protocols to monitor and manage delirium. This study has the potentials to shed some lights on the variables that

  14. Multiple syncope mechanisms coexisting in a Brugada syndrome patient requiring a single therapeutic approach.

    Science.gov (United States)

    Vouliotis, A I; Gatzoulis, K A; Dilaveris, P; Stefanadis, C

    2013-05-01

    We report the case of a Brugada syndrome patient with a history of syncopal and presyncopal episodes and evidence of sinus node and atrioventricular (AV) conduction abnormalities. The patient developed sinus bradycardia, sinoatrial conduction abnormalities, prolonged HV interval, early appearance of AV block, AV nodal reentrant tachycardia and polymorphic ventricular tachycardia in the electrophysiological study. He was treated with a dual-chamber pacemaker defibrillator. At the 9-year follow-up, the patient remained asymptomatic with several episodes of 1:1 AV-relationship tachycardia, interrupted with antitachycardia pacing, while the predominant pacing states of the device were AP-VS and AS-VP for most of the time.

  15. Objectively measured light-intensity lifestyle activity and sedentary time are independently associated with metabolic syndrome: a cross-sectional study of Japanese adults.

    Science.gov (United States)

    Kim, Junghoon; Tanabe, Kai; Yokoyama, Noriko; Zempo, Hirofumi; Kuno, Shinya

    2013-03-04

    Reducing sedentary time and increasing lifestyle activities, including light-intensity activity, may be an option to help prevent metabolic syndrome (MetS). The purpose of the present study was to examine whether objectively measured light-intensity lifestyle activity and sedentary time is associated with MetS, independent of moderate-vigorous intensity physical activity (MVPA). The participants in this cross-sectional study were 483 middle-aged Japanese adults, aged 30-64 years. The participants were divided into those with or without MetS according to the Japanese criteria for MetS. A triaxial accelerometer was used to measure light-intensity lifestyle activity [1.6-2.9 metabolic equivalents (METs)] and sedentary time (≤1.5 METs). Logistic regression was used to predict MetS from the levels of light-intensity lifestyle activity and sedentary time with age, sex, smoking, calorie intake, accelerometer wear time, and MVPA as covariates. The odds ratios (OR) for MetS in the highest and middle tertiles of light-intensity lifestyle activity were 0.44 [95% confidence interval (CI): 0.24 to 0.81] and 0.51 (95% CI: 0.29 to 0.89) relative to the lowest tertile, after adjustment for age, sex, smoking, calorie intake, accelerometer wear time and MVPA (Ptrend = 0.012). Sedentary time was also associated with the risk of MetS (Ptrend = 0.018). Among participants in the highest tertile of sedentary time, the risk of MetS was 2.27-times greater than that in the lowest tertile (95% CI: 1.25 to 4.11). The risk of MetS was not significantly increased in subjects in the middle tertile of sedentary time. We found that light-intensity lifestyle activity and sedentary time were significantly associated with the risk of MetS, independent of MVPA. The results of our study suggest that public health messages and guidelines should be refined to include increases in light-intensity lifestyle activity and/or decreases in sedentary time, alongside promoting MVPA, to prevent MetS.

  16. Effect of Atorvastatin intensive therapy on the serum inflammatory factors, platelet activity and fibrinolytic activity in patients with acute coronary syndrome

    Directory of Open Access Journals (Sweden)

    Xiao-Li Zhu

    2016-05-01

    Full Text Available Objective: To observe the effect of Atorvastatin intensive therapy on the serum inflammatory factors, platelet activity and fibrinolytic activity in patients with acute coronary syndrome (ACS. Methods: A total of 92 patients with ACS were randomly divided into observation group (47 cases and control group (45 cases. The control group was given Atorvastatin (10mg/d based on the conventional therapy, while the observation group was given Atorvastatin at an intensive dose (40 mg/d based on the conventional therapy. Half a month later, the changes of IL-6, IL-8, hs-CRP, TNF-α, TXB2, GMP-140, PAI-1 and t-PA were observed and compared between the two groups. Results: After treatment, the inflammatory factors (IL-6, IL-8, hs-CRP and TNF-α and the indicators of platelet activity (TXB2, GMP-140 and PAI-1 were obviously decreased, while the indicator of fibrinolytic activity (t-PA was apparently increased in the two groups. Besides, the amplitudes of change referring to these indicators in the observation group were bigger than those in the control group after treatment, and the differences were statistically significant. Conclusion: The intensive therapy with the administration of Atorvastatin at a dose of 40 mg/d was better than the conventional therapy (Atorvastatin: 10 mg/d in aspects of reducing inflammatory factors, inhibiting platelet activity and correcting the high coagulation state of fibrinolytic system.

  17. Determining Nutrient Requirements For Intensively Managed Loblolly Pine Stands Using the SSAND (Soil Supply and Nutrient Demand) Model

    Science.gov (United States)

    Hector G. Adegbidi; Nicholas B. Comerford; Hua Li; Eric J. Jokela; Nairam F. Barros

    2002-01-01

    Nutrient management represents a central component of intensive silvicultural systems that are designed to increase forest productivity in southern pine stands. Forest soils throughout the South are generally infertile, and fertilizers may be applied one or more times over the course of a rotation. Diagnostic techniques, such as foliar analysis and soil testing are...

  18. Astrocyte glycogen metabolism is required for neural activity during aglycemia or intense stimulation in mouse white matter

    DEFF Research Database (Denmark)

    Brown, Angus M; Sickmann, Helle M; Fosgerau, Keld

    2005-01-01

    We tested the hypothesis that inhibiting glycogen degradation accelerates compound action potential (CAP) failure in mouse optic nerve (MON) during aglycemia or high-intensity stimulation. Axon function was assessed as the evoked CAP, and glycogen content was measured biochemically. Isofagomine, ...

  19. Meeting the International Health Regulations (2005) surveillance core capacity requirements at the subnational level in Europe: the added value of syndromic surveillance.

    Science.gov (United States)

    Ziemann, Alexandra; Rosenkötter, Nicole; Riesgo, Luis Garcia-Castrillo; Fischer, Matthias; Krämer, Alexander; Lippert, Freddy K; Vergeiner, Gernot; Brand, Helmut; Krafft, Thomas

    2015-02-07

    The revised World Health Organization's International Health Regulations (2005) request a timely and all-hazard approach towards surveillance, especially at the subnational level. We discuss three questions of syndromic surveillance application in the European context for assessing public health emergencies of international concern: (i) can syndromic surveillance support countries, especially the subnational level, to meet the International Health Regulations (2005) core surveillance capacity requirements, (ii) are European syndromic surveillance systems comparable to enable cross-border surveillance, and (iii) at which administrative level should syndromic surveillance best be applied? Despite the ongoing criticism on the usefulness of syndromic surveillance which is related to its clinically nonspecific output, we demonstrate that it was a suitable supplement for timely assessment of the impact of three different public health emergencies affecting Europe. Subnational syndromic surveillance analysis in some cases proved to be of advantage for detecting an event earlier compared to national level analysis. However, in many cases, syndromic surveillance did not detect local events with only a small number of cases. The European Commission envisions comparability of surveillance output to enable cross-border surveillance. Evaluated against European infectious disease case definitions, syndromic surveillance can contribute to identify cases that might fulfil the clinical case definition but the approach is too unspecific to comply to complete clinical definitions. Syndromic surveillance results still seem feasible for comparable cross-border surveillance as similarly defined syndromes are analysed. We suggest a new model of implementing syndromic surveillance at the subnational level. In this model, syndromic surveillance systems are fine-tuned to their local context and integrated into the existing subnational surveillance and reporting structure. By enhancing

  20. The Vastus Medialis Oblique: Vastus Lateralis Electromyographic Intensity Ratio During Squat with Hip Adduction in Athletes with and Without Patellofemoral Pain Syndrome

    Directory of Open Access Journals (Sweden)

    Farhad Reza-zadeh

    2012-07-01

    Full Text Available Objective: This study was designed to compare vastus medialis oblique (VMO: vastus lateralis longus (VLL electromyographic intensity ratio during squat with hip adduction in athletes with and without patellofemoral pain syndrome (PFPS. Materials & Methods: In this non-experimental and case-control study, 16 male athletes with PFPS were selected purposefully and 16 healthy male athletes aged 18-30 years from national teams (Volleyball, Handball and Taekwondo were matched based on variables such as weight, height, age, dominancy. All subjects selected based on inclusion and exclusion criteria. EMG activity of VMO and VLL muscles was recorded by surface electrodes with Telemetric EMG System at 15, 30 and 45 degrees of squat and VMO: VLL ratio was calculated. One way ANOVA was used to compare these muscles ratio between two groups. Results: The ratio of VMO: VLL in both groups with and without PFPS in almost all angles were lower than one. However, healthy athletes had lower ratios. Also, there were no significant differences in VMO: VLL ratio at various angles. Conclusion: It seems that sports activities prevent VMO weakening in athletes. However, VMO: VLL ratio in athletes with and without patellofemoral pain does not influence by this syndrome.

  1. Skin rash in the intensive care unit: Stevens-Johnson syndrome, toxic epidermal necrolysis, or a rare manifestation of a hidden cutaneous malignancy: A case report.

    Science.gov (United States)

    Al-Saffar, Farah; Ibrahim, Saif; Patel, Pujan; Jacob, Rafik; Palacio, Carlos; Cury, James

    2016-03-01

    Skin rashes are infrequently encountered in the intensive care units, either as a result or as a cause of admission. The entities of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) form a spectrum of desquamating skin diseases that have multiple etiologies, the most common being drug-related reactions; very rarely, the cause may be cutaneous malignancies. We herein present a unique case of a 54-year-old male patient with psoriasis treated with methotrexate, who presented with a cellulitis-like clinical picture, then developed a severe progressive systemic inflammatory response syndrome, and progressed clinically to SJS, then TEN even after discontinuing the antibiotics and methotrexate. A skin biopsy demonstrated an aggressive and rapidly-progressing T-cell lymphoma. The present case highlights the necessity of skin biopsy when encountering SJS and TEN in the ICU in order to identify potentially treatable/controllable causes. Although it appeared reasonable to correlate TEN solely to medications, the skin biopsies clearly demonstrated an aggressive T-cell skin lymphoma. In a patient with a better general condition it may have been helpful to treat this malignancy. TEN is a life-threatening condition and skin biopsy is the cornerstone of diagnosis, despite the presence of multiple risk factors and the typical physical findings of a drug-induced reaction.

  2. Molecular typing of toxic shock syndrome toxin-1- and Enterotoxin A-producing methicillin-sensitive Staphylococcus aureus isolates from an outbreak in a neonatal intensive care unit.

    Science.gov (United States)

    Layer, Franziska; Sanchini, Andrea; Strommenger, Birgit; Cuny, Christiane; Breier, Ann-Christin; Proquitté, Hans; Bührer, Christoph; Schenkel, Karl; Bätzing-Feigenbaum, Jörg; Greutelaers, Benedikt; Nübel, Ulrich; Gastmeier, Petra; Eckmanns, Tim; Werner, Guido

    2015-10-01

    Outbreaks of Staphylococcus aureus are common in neonatal intensive care units (NICUs). Usually they are documented for methicillin-resistant strains, while reports involving methicillin-susceptible S. aureus (MSSA) strains are rare. In this study we report the epidemiological and molecular investigation of an MSSA outbreak in a NICU among preterm neonates. Infection control measures and interventions were commissioned by the Local Public Health Authority and supported by the Robert Koch Institute. To support epidemiological investigations molecular typing was done by spa-typing and Multilocus sequence typing; the relatedness of collected isolates was further elucidated by DNA SmaI-macrorestriction, microarray analysis and bacterial whole genome sequencing. A total of 213 neonates, 123 healthcare workers and 205 neonate parents were analyzed in the period November 2011 to November 2012. The outbreak strain was characterized as a MSSA spa-type t021, able to produce toxic shock syndrome toxin-1 and Enterotoxin A. We identified seventeen neonates (of which two died from toxic shock syndrome), four healthcare workers and three parents putatively involved in the outbreak. Whole-genome sequencing permitted to exclude unrelated cases from the outbreak and to discuss the role of healthcare workers as a reservoir of S. aureus on the NICU. Genome comparisons also indicated the presence of the respective clone on the ward months before the first colonized/infected neonates were detected. Copyright © 2015 Elsevier GmbH. All rights reserved.

  3. The Effectiveness of an Intensive Interdisciplinary Pain Rehabilitation Program in the Treatment of Post-Laminectomy Syndrome in Patients Who Have Failed Spinal Cord Stimulation.

    Science.gov (United States)

    Bailey, Joshua C; Kurklinsky, Svetlana; Sletten, Christopher D; Osborne, Michael D

    2018-02-01

    Post-laminectomy syndrome (PLS) patients who have previously undergone spinal cord stimulation and failed to have significant improvement create a unique challenge for ongoing pain management. We hypothesize that, following successful completion of intensive, interdisciplinary pain rehabilitation (IPR), this patient population can achieve a significant reduction in pain, improvement in mood, functional levels, and self-efficacy. A retrospective chart review was conducted comparing the following for patients prior to enrollment in the IPR program and upon completion: numeric rating scale (NRS) pain scores; functional status via the six-minute walk test; mood via the Center for Epidemiologic Studies Depression Scale (CES-D), Multidimensional Pain Inventory (MPI) Life control scores and MPI Interference, and the Pain Catastrophizing Scale (PCS); and self-efficacy via the Pain Self-Efficacy Questionnaire (PSEQ). Forty-three patients met inclusion criteria, with 17 males and 26 females and a mean age of 64 years. Patients demonstrated a statistically significant increase in six-minute walk test distance of 104 m, a decrease in average NRS pain score of 1.4 points, an increase in average MPI life control by 8.3 points, a decrease average MPI interference by 5.3 points, an increase in average Short Form-36 by 6.5 points, an increase in average PCS by 4.4 points, and an increase in average PSEQ score of 18.1. Their average mood via CES-D improved by 4.2 points. Intensive, interdisciplinary pain rehabilitation provides an effective therapeutic modality for patients with post-laminectomy syndrome who have failed spinal cord stimulation by decreasing pain levels and by increasing functional status and self-efficacy. © 2017 American Academy of Pain Medicine. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com

  4. Autosomal recessive mutations in THOC6 cause intellectual disability: syndrome delineation requiring forward and reverse phenotyping.

    Science.gov (United States)

    Amos, J S; Huang, L; Thevenon, J; Kariminedjad, A; Beaulieu, C L; Masurel-Paulet, A; Najmabadi, H; Fattahi, Z; Beheshtian, M; Tonekaboni, S H; Tang, S; Helbig, K L; Alcaraz, W; Rivière, J-B; Faivre, L; Innes, A M; Lebel, R R; Boycott, K M

    2017-01-01

    THOC6 is a part of the THO complex, which is involved in coordinating mRNA processing with export. The THO complex interacts with additional components to form the larger TREX complex (transcription export complex). Previously, a homozygous missense mutation in THOC6 in the Hutterite population was reported in association with syndromic intellectual disability. Using exome sequencing, we identified three unrelated patients with bi-allelic mutations in THOC6 associated with intellectual disability and additional clinical features. Two of the patients were compound heterozygous for a stop and a missense mutation, and the third was homozygous for a missense mutation; the missense mutations were predicted to be pathogenic by in silico analysis and modeling. Clinical features of the three newly identified patients and those previously reported are reviewed; intellectual disability is moderate to severe, and malformations are variable including renal and heart defects, cleft palate, microcephaly, and corpus callosum dysgenesis. Facial features are variable and include tall forehead, short upslanting palpebral fissures +/- deep set eyes, and a long nose with overhanging columella. These subtle facial features render the diagnosis difficult to make in isolation with certainty. Our results expand the mutational and clinical spectrum of this rare disease, confirm that THOC6 is an intellectual disability causing gene, while providing insight into the importance of the THO complex in neurodevelopment. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  5. Evaluation of spinal cord stimulation on the symptoms of anxiety and depression and pain intensity in patients with failed back surgery syndrome.

    Science.gov (United States)

    Robb, L P; Cooney, J M; McCrory, C R

    2017-08-01

    Spinal cord stimulation (SCS) is now established as the primary treatment for failed back surgery syndrome (FBSS). Commonly, patients with chronic pain and FBSS often report symptoms of anxiety and depression resulting from this condition. These factors can modulate and amplify the pain experience, therefore, further challenging treatment success. This study examined the efficacy of SCS on alleviating the symptoms of anxiety and depression associated with chronic pain as well as pain intensity in a group of patients with FBSS. A convenience sample (n = 26) was selected for participation. Questionnaires [Hospital Anxiety and Depression Scale (HADS) and Brief Pain Inventory Short Form (BPI-SF)] were completed and examined pre and post spinal cord implant. Analysis of the data 1 year following SCS indicates that there was a statistical significant improvement in the symptoms of depression and anxiety reported as well as pain intensity in all participants (p anxiety and depression scores on the HADS were significantly lower compared to baseline (p anxiety and depression with an associated reduction in opioid consumption.

  6. Early Exposure to Recommended Calorie Delivery in the Intensive Care Unit Is Associated With Increased Mortality in Patients With Acute Respiratory Distress Syndrome.

    Science.gov (United States)

    Peterson, Sarah J; Lateef, Omar B; Freels, Sally; McKeever, Liam; Fantuzzi, Giamila; Braunschweig, Carol A

    2017-06-01

    The Intensive Nutrition in Acute Lung Injury: Clinical Trial (INTACT), designed to evaluate outcomes of calorie delivery from acute respiratory distress syndrome (ARDS) diagnosis through hospital discharge, was stopped due to higher mortality in the intervention group. Post hoc analysis found timing and dose of calorie delivery influenced mortality. The objective of this retrospective cohort study was to determine if early vs late calorie exposure changed the hazard of death among a larger sample of patients with ARDS. Adult patients who met the eligibility criteria for INTACT but did not participate were included. Daily calorie delivery was collected from the date INTACT eligibility was determined to extubation or death. Cox proportional hazards regression was used to model the relationship between hazard of hospital death with average calorie exposure received over increasing study days and after day 7. A total of 298 patients were included; overall mortality was 33%. Among patients who remained intubated at 1 week (n = 202), higher kcal/kg received from intensive care unit (ICU) days 1-6 increased hazards of subsequent death on days 7+ (hazard ratio [HR], 1.04; 95% confidence interval [CI], 1.01-1.06); kcal/kg received after ICU day 7 decreased the hazards of death on day 7+ (HR, 0.53; 95% CI, 0.33-0.84). Higher calorie exposure between ICU days 1 and 7 was associated with higher subsequent hazard of mortality, and provision of high-calorie exposure after day 8 decreased the hazards of death.

  7. Astrocyte glycogen metabolism is required for neural activity during aglycemia or intense stimulation in mouse white matter.

    Science.gov (United States)

    Brown, Angus M; Sickmann, Helle M; Fosgerau, Keld; Lund, Trine M; Schousboe, Arne; Waagepetersen, Helle S; Ransom, Bruce R

    We tested the hypothesis that inhibiting glycogen degradation accelerates compound action potential (CAP) failure in mouse optic nerve (MON) during aglycemia or high-intensity stimulation. Axon function was assessed as the evoked CAP, and glycogen content was measured biochemically. Isofagomine, a novel inhibitor of central nervous system (CNS) glycogen phosphorylase, significantly increased glycogen content under normoglycemic conditions. When MONs were bathed in artificial cerebrospinal fluid (aCSF) containing 10 mM glucose, the CAP failed 16 min after exposure to glucose-free aCSF. MONs bathed in aCSF plus isofagomine displayed accelerated CAP failure on glucose removal. Similar results were obtained in MONs bathed in 30 mM glucose, which increased baseline glycogen concentration. The ability of isofagomine to increase glycogen content thus was not translated into delayed CAP failure. This is likely due to the inability of the tissue to metabolize glycogen in the presence of isofagomine, highlighting the importance of glycogen in sustaining neural function during aglycemia. The hypothesis that glycogen breakdown supports intense neural activity was tested by blocking glycogen breakdown during periods of high-frequency stimulation. The CAP area declined more rapidly when glycogen metabolism was inhibited by isofagomine, explicitly showing an important physiological role for glycogen metabolism during neural activity. (c) 2004 Wiley-Liss, Inc.

  8. Long-term follow-up of a retrospective comparison of reduced-intensity conditioning and conventional high-dose conditioning for allogeneic transplantation from matched related donors in myelodysplastic syndromes

    NARCIS (Netherlands)

    Martino, R.; Henseler, A.; Lint, M. van; Schaap, N.P.; Finke, J.; Beelen, D.; Vigouroux, S.; Alessandrino, E.P.; Mufti, G.J.; Veelken, J.H.; Bruno, B.; Yakoub-Agha, I.; Volin, L.; Maertens, J.; Or, R.; Leblond, V.; Rovira, M.; Kalhs, P.; Alvarez, A.F.; Vitek, A.; Sierra, J.; Wagner, E.; Robin, M.; Witte, T.J. de; Kroger, N.

    2017-01-01

    This study shows the long-term updated outcomes of a multicenter retrospective study which analyzed 843 patients with myelodysplastic syndrome (MDS) who underwent transplantation with an HLA-identical sibling donor with either reduced-intensity conditioning (RIC) in 213 patients, or standard

  9. Epidemiological characteristics, practice of ventilation, and clinical outcome in patients at risk of acute respiratory distress syndrome in intensive care units from 16 countries (PRoVENT): an international, multicentre, prospective study

    NARCIS (Netherlands)

    Neto, Ary Serpa; Barbas, Carmen S. V.; Simonis, Fabienne D.; Artigas-Raventós, Antonio; Canet, Jaume; Determann, Rogier M.; Anstey, James; Hedenstierna, Goran; Hemmes, Sabrine N. T.; Hermans, Greet; Hiesmayr, Michael; Hollmann, Markus W.; Jaber, Samir; Martin-Loeches, Ignacio; Mills, Gary H.; Pearse, Rupert M.; Putensen, Christian; Schmid, Werner; Severgnini, Paolo; Smith, Roger; Treschan, Tanja A.; Tschernko, Edda M.; Melo, Marcos F. V.; Wrigge, Hermann; de Abreu, Marcelo Gama; Pelosi, Paolo; Schultz, Marcus J.; Bell, Adam; Gecaj-Gashi, Agreta; Dilek, Ahmet; Denker, Ahmet Sukru; Aytulun, Akut; Kienbaum, Peter; Rose, Alastair; Bacuzzi, Alessandro; Cavalcanti, Alexandre Biasi; Chan, Alexandre; Molin, Alexandre; Ghosh, Alison; Roy, Alistair; Cowton, Amanda; Skinner, Amanda; Whileman, Amanda; McInerney, Amy; Peçanha, Ana Carolina; Cortegiani, Andrea; Sribar, Andrej; Bentley, Andrew; Corner, Andrew; Pinder, Angela; Hormis, Anil; Walker, Anna; Dixon, Barry; Creagh-Brown, Ben; Volta, Carlo Alberto; Munhoz, Carlos; Brown, Carly; Scott, Carmen; Wreybrown, Caroline; Plowright, Catherine; Downes, Charlotte; Padilla-Harris, Cheryl; Hughes, Chloe; Frey, Christian; Schlegel, Christian; Boyd, Christine; Ryan, Christine; Muench, Christoph; Smalley, Christopher; Zincircioglu, Çiler; Harris, Clair; Kaloo, Claire; Matthews, Claire; Miller, Claire; Pegg, Claire; Bullock, Clare; Mellis, Clare; Piras, Claudio; Seasman, Colette; Santos, Cristina; Beraldo, Daniel; Collins, Daniel; Hadfield, Daniel; Hull, Daniel; Prado, Daniel; Pogson, David; Rogerson, David; Shaw, David; D'Antini, Davide; Griffin, Dawn Trodd Denise; Weller, Debbie; Smith, Deborah; Wilson, Deborah; Aydin, Demet; Donaldson, Denise; Mestria, Donatella; Lauro, Eduardo Di; Caser, Eliane Bernadete; Seghelini, Elisa; Cirstea, Emanuel; Young, Eoin; Alberts, Erna; Senturk, Evren; Brohi, Farooq; Ulger, Fatma; Kahveci, Feda; da Silva Ramos, Fernando José; van Haren, Frank; Turan, Güldem; Sales, Gabriele; Clifford, Gayle; Cinnella, Gilda; Mecatti, Giovana Colozza; Melchionda, Giuseppe; Eren, Gulay; Crowther, Hannah; Spencer, Hazel; Blaylock, Heather; Green, Helen; Robertson, Helen; Rodgers, Helen; Talbot, Helen; Wong, Helen; Barcraft-Barnes, Helena; Ceunen, Helga; Reschreiter, Henrik; Ulusoy, Hulya; Toman, Huseyin; McCullagh, Iain; White, Ian; Welters, Ingeborg; van den Hul, Ingrid; Gava, Isabela Ambrósio; Reed, Isabelle; Kose, Isil; Maia, Israel; Limb, James; Máca, Jan; Adderly, Jane; Hunt, Jane; Martin, Jane; Montgomery, Jane; Snell, Jane; Salgado, Jean; Ritzema, Jenny; Bewley, Jeremy; Howe, Joanne; Decruyenaere, Johan; Mouland, Johanna; Stickley, Johanna; Mellinghoff, Johannes; Criswell, John; Knighton, John; Cooper, Jonathan; Harrison, Jonathan; Paddle, Jonathan; Pellegrini, Jose Augusto Santos; Needleman, Joseph; Giles, Julian; Camsooksai, Julie; Furneval, Julie; Toms, Julie; Burt, Karen; Simeson, Karen; Williams, Karen; Blenk, Karl; Turner, Kate; Lynch, Katie; Sweet, Katie; Hugill, Keith; Matthews, Kelly; Ruas, Kessia; Clarkson, Kevin; Preller, Kobus; Joyce, Kristen; Ortiz-Ruiz, Laura; Youds, Laura; Tbaily, Lee; Barrell, Lisa; Grimmer, Lisa; Soyoral, Lokman; Peluso, Lorenzo; Murray, Lorna; Niska, Lotta; Tonks, Louise; Fasting, Lousie; DeCrop, Luc; Brazzi, Luca; Mirabella, Lucia; Cooper, Lucy; Falcão, Luis Fernando; Everett, Lynn; Watters, Malcolm; Carnahan, Mandy; Bourgeois, Marc; Abreu, Marcelo Gama de; Romano, Marcelo Luz Pereira; Botteri, Marco; Melo, Marcos F. Vidal; Faulkner, Maria; Krkusek, Marijana; Bahl, Marina; Holliday, Mark; Kol, Mark; Pulletz, Mark; Kozlowski, Marta; Dvorscak, Matea Bogdanovic; Jurjevic, Matija; Koopmans, Matty; Morales, Mauricio; Schaefer, Maximilian; Brazier, Melinda; Harris, Meredith; Devile, Michael; Kuiper, Michael; Parris, Michael; Sharman, Michael; Kratochvil, Milan; Ramali, Mohamed; Dos Santos, Moreno Calcagnotto; Bynorth, Natalie; Wilson, Natalie; Anquez, Nathalie; Huneke, Nathan; Dogan, Nazim; Karanovic, Nenad; Tarmey, Nicholas; Carreño, Nicolás; Fisher, Nicola; Lamb, Nicola; Venner, Nicola; Hollister, Nigel; Akgun, Nur; Ekinci, Osman; Boyd, Owen; Gill, Pardeep; Raimondo, Pasquale; Verrastro, Pasquale; Pulak, Paul; Fitzell, Pauline; Dark, Paulo; Alzugaray, Pedro; Özcan, Perihan Ergin; MacNaughton, Peter; Stourac, Petr; Hopkins, Phil; Tuinman, Pieter Roel; Pearson, Rachel; Walker, Rachel; Santos, Rafaella Souza Dos; Caione, Raffaele; Matsa, Ramprasad; Oliver, Rebecca; Jacob, Reni; Howard-Griffin, Richard; Wilde, Robert Bp de; Plant, Robert; Hollands, Robin; Biondi, Rodrigo; Jaafar, Rola; Avendaño, Rossana; Salt, Ruth; Humphries, Ryan; Pinto, Sérgio Felix; Pearson, Sallyane; Hendry, Sam; Lakhani, Sandeep; Beavis, Sarah; Moreton, Sarah; Prudden, Sarah; Thornthwaite, Sarah; Spadaro, Savino; Saylan, Sedat; Chenna, Shailaja; Gopal, Shammer; James, Shanaz; Suresh, Sheeba; Birch, Sian; Skilijic, Sonja; Aguirre, Stefania; Metherell, Stella; Bell, Stephanie; Janes, Stephanie; Wright, Stephen; Rose, Steve; Windebank, Steve; Glenn, Sue; Melbourne, Susan; Tyson, Susan; Leaver, Susannah; Patel, Tasmin; Simurina, Tatjana; Sewell, Terri-Ann; Macruz, Tiago; Hatton, Tom; Evans, Tracey; Goktas, Ugur; Poultney, Una; Buyukkocak, Unase; Linnett, Vanessa; Oliveira, Vanessa; Russotto, Vincenzo; Klaric, Vlasta; Orak, Yavuz; Demirtürk, Zerrin

    2016-01-01

    Scant information exists about the epidemiological characteristics and outcome of patients in the intensive care unit (ICU) at risk of acute respiratory distress syndrome (ARDS) and how ventilation is managed in these individuals. We aimed to establish the epidemiological characteristics of patients

  10. Planning intensive care unit design using computer simulation modeling: optimizing integration of clinical, operational, and architectural requirements.

    Science.gov (United States)

    OʼHara, Susan

    2014-01-01

    Nurses have increasingly been regarded as critical members of the planning team as architects recognize their knowledge and value. But the nurses' role as knowledge experts can be expanded to leading efforts to integrate the clinical, operational, and architectural expertise through simulation modeling. Simulation modeling allows for the optimal merge of multifactorial data to understand the current state of the intensive care unit and predict future states. Nurses can champion the simulation modeling process and reap the benefits of a cost-effective way to test new designs, processes, staffing models, and future programming trends prior to implementation. Simulation modeling is an evidence-based planning approach, a standard, for integrating the sciences with real client data, to offer solutions for improving patient care.

  11. Sunflower exposed to high-intensity microwave-frequency electromagnetic field: electrophysiological response requires a mechanical injury to initiate.

    Science.gov (United States)

    Roux, David; Catrain, Alexandre; Lallechere, Sébastien; Joly, Jean-Christophe

    2015-01-01

    We have monitored the electrical potential variations (EPV) of sunflower plants illuminated by a high-intensity microwave-frequency (2.5 GHz, 1.5 kV/m) electromagnetic field (EMF). We have designed an appropriate set-up that allows parallel temperature and EPV measurements while part of the plant is being exposed to the field. The results show that the considered EMF does not induce plant EPV directly. This electrophysiological response appears only when the EMF leads to a mechanical injury of the tissues via a thermal effect (dielectric heating). Once the plant inner temperature reached a threshold, we systematically observed burn-like lesions associated with the bending of the stem or leaf-stalks. Theses mechanical constraints were rapidly followed by EPVs, moving through the stem.

  12. Superior Semicircular Canal Dehiscence Syndrome without Vestibular Symptoms

    OpenAIRE

    Teixeira,Emidio Oliveira; Fonseca,Marconi Teixeira

    2014-01-01

    Introduction Superior semicircular canal dehiscence syndrome is mainly characterized by vestibular symptoms induced by intense sound stimuli or pressure changes, which occur because of dehiscence of the bony layer covering the superior semicircular canal. Case Report Here, we report a case of the syndrome with pulsatile tinnitus and ear fullness, in the absence of vestibular symptoms. Discussion Signs and symptoms of the syndrome are rarely obvious, leading to the requirement for a min...

  13. Antiatherosclerotic effects of long-term maximally intensive statin therapy after acute coronary syndrome: insights from Study of Coronary Atheroma by Intravascular Ultrasound: Effect of Rosuvastatin Versus Atorvastatin.

    Science.gov (United States)

    Puri, Rishi; Nissen, Steven E; Shao, Mingyuan; Ballantyne, Christie M; Barter, Philip J; Chapman, M John; Erbel, Raimund; Libby, Peter; Raichlen, Joel S; Uno, Kiyoko; Kataoka, Yu; Nicholls, Stephen J

    2014-11-01

    Patients with acute coronary syndromes (ACS) display diffuse coronary atheroma instability and heightened risk of early and late recurrent coronary events. We compared the long-term antiatherosclerotic efficacy of high-intensity statins in patients with ACS when compared with stable disease. Study of Coronary Atheroma by Intravascular Ultrasound: Effect of Rosuvastatin Versus Atorvastatin (SATURN) used serial intravascular ultrasound measures of coronary atheroma volume in patients treated with rosuvastatin 40 mg or atorvastatin 80 mg for 24 months. The overall effect of high-intensity statins on the change in coronary percent atheroma volume and major adverse cardiovascular events (death/nonfatal myocardial infarction/coronary revascularization) were evaluated in this post hoc analysis. When compared with non-ACS patients (n=678), patients with ACS (n=361) were younger, actively smoking, and have had a previous myocardial infarction (all P<0.001). At baseline, patients with ACS exhibited lower high-density lipoprotein cholesterol (43.5±11 versus 45.8±11 mg/dL; P=0.002), a higher apolipoprotein B: apolipoprotein A-1 ratio (0.90±0.24 versus 0.83±0.24; P<0.001) and greater percent atheroma volume (37.3±8.5% versus 35.9±8.1%; P=0.01) when compared with non-ACS patients. Despite similar achieved levels of lipid and inflammatory markers after high-intensity statin therapy, patients with ACS demonstrated greater percent atheroma volume regression than non-ACS patients (-1.46±0.14 versus -0.89±0.13; P=0.003). After propensity-weighted multivariable adjustment, baseline percent atheroma volume (P<0.001) and an ACS clinical presentation (P=0.02) independently associated with plaque regression. The 24-month major adverse cardiovascular events-free survival was similar between patients with ACS and non-ACS (90.6 versus 92.9%; P=0.25). Long-term high-intensity statin therapy caused greater plaque regression and comparable major adverse cardiovascular events rates in

  14. Fulminant hepatitis linked to dapsone hypersensitivity syndrome requiring urgent living donor liver transplantation: a case report.

    Science.gov (United States)

    Garcia, Angelica; Yi, Nam-Joon; Lee, Kyoung Bun; Lee, Jeong-Moo; Choi, Young Rok; Suh, Suk-Won; You, Tae; Lee, Kwang-Woong; Park, June Dong; Kang, Hyoung Jin; Kim, Joon Gon; Kang, Eun Kyeong; Hong, Geun; Suh, Kyung-Suk

    2014-11-01

    Dapsone is a sulfone-type drug used widely for different infectious, immune, and hypersensitivity disorders as an antibacterial treatment alone or in combination for leprosy and sometimes for infected skin lesions. DHS is a severe idiosyncratic adverse reaction with multi-organ involvement. However, acute necrotic hepatitis requiring an emergent LT is rare. Herein, we report a case of 12-yr-old girl who suffered from fulminant hepatitis and multi-organ failure due to DHS for PPD. She was saved by emergent LDLT. A high index of suspicion and rapid diagnosis are necessary not to miss this potentially lethal but rare disease. © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  15. The Role of Rotavirus Infection Requiring Treatment in a Hospital in Children with Diarrheal Syndrome

    Directory of Open Access Journals (Sweden)

    Yu.Yu. Stepanova

    2013-11-01

    Full Text Available The aim of this research was to investigate the prognostic and diagnostic value of some clinical and biological factors in the development of acute intestinal rotavirus infection in children. We observed 161 children aged 1 month to 6 years. 81 children were diagnosed with rotavirus infection, and 80 — with secretory diarrhea of unknown origin. Treatment of children was carried out in a hospital. Analysis of clinical and anamnestic data enabled to choose the most prognostically and diagnostically important predictors out of 117 studied risk factors. The authors investigated the association of clinical and biological factors with the severity of rotavirus infection, which would highlight the groups of children with high risk of rotavirus infection, which requires treatment in a hospital. The practical application of the results of this work should be the optimization of early diagnostics of rotavirus infections and timeliness of preventive measures.

  16. Clinical Efficacy of 1-Year Intensive Systematic Dietary Manipulation as Complementary and Alternative Medicine Therapies on Female Patients With Interstitial Cystitis/Bladder Pain Syndrome.

    Science.gov (United States)

    Oh-Oka, Hitoshi

    2017-08-01

    To evaluate the clinical efficacy of intensive systematic dietary manipulation (ISDM) for female patients with interstitial cystitis (IC)/bladder pain syndrome (BPS) in stable condition who were followed up in our hospital. In cooperation with the nutrition control team, we created a basic IC/BPS diet menu for 1 month. Data regarding daily food intake and food-related symptoms were collected by conducting a detailed interview of each patient, and we set meal menu to control IC/BPS symptoms and advised the patients to reduce the intake of specific food items to the maximum possible extent. The following food items were removed from or restricted in the diet of patients: tomatoes, tomato products, soybean, tofu product, spices, excessive potassium, citrus, high-acidity-inducing substances, etc. We evaluated the following factors 3 months and 1 year after the start of the intervention: O'Leary-Sant symptom index, O'Leary-Sant problem index, urgency visual analogue scale score, bladder or pelvic pain visual analogue scale score, and numerical patient-reported quality of life index. All evaluated factors improved statistically significantly when the intensive group was compared with the nonintensive group (baseline to 3 months and 3 months to 1 year ISDM, P <.05, respectively). ISDM was found to alleviate the symptoms of IC/BPS in almost 3 months and continued clinical efficacy for at least 1 year. ISDM as one of the conservative treatment modality for IC/BPS should be attempted more strictly because of its noninvasiveness, without alterations to the other treatments. Copyright © 2017 Elsevier Inc. All rights reserved.

  17. Long-term effect of pulsed high-intensity laser therapy in the treatment of post-mastectomy pain syndrome: a double blind, placebo-control, randomized study.

    Science.gov (United States)

    Ebid, Anwar Abdelgayed; El-Sodany, Ahmed Mohamed

    2015-08-01

    We assess the long-term effect of pulsed high-intensity laser therapy (HILT) in the treatment of the post-mastectomy pain syndrome (PMPS). A total of 61 women participated in this study (30 in the laser group and 31 in the placebo laser group), with a mean age of 53.56 ± 1.11 years. Patients who were randomly assigned to the laser group received HILT three times per week for 4 weeks, plus a routine physical therapy program (RPTP). The placebo laser group received placebo HILT plus RPTP. The outcomes measured were pain level by visual analog scale (VAS), shoulder range of motion (ROM), and quality of life (QOL). Statistical analysis was performed by ANOVA with repeated measures to compare the differences between baseline and post-treatment measurements and after 12 weeks of follow-up for both groups. The level of statistical significance was set at P placebo group. VAS results showed a significant decrease post-treatment in the laser group relative to the placebo group, and QOL results showed a significant improvement in the laser group compared with the placebo group and still improved after 12 weeks of follow-up. HILT combined with an RPTP appears to be more effective in patients with PMPS than a placebo laser procedure with RPTP.

  18. Positive end expiratory pressure for preterm infants requiring conventional mechanical ventilation for respiratory distress syndrome or bronchopulmonary dysplasia.

    Science.gov (United States)

    Bamat, Nicolas; Millar, David; Suh, Sanghee; Kirpalani, Haresh

    2012-01-18

    Conventional mechanical ventilation (CMV) of neonates has been used as a treatment of respiratory failure for over 30 years. While CMV facilitates gas exchange, it may simultaneously damage the lung. Positive end expiratory pressure (PEEP) has received less attention than other ventilation parameters when considering this balance of benefit and possible harm. While an appropriate level of PEEP may exert substantial benefits in ventilation, both inappropriately low or high levels may lead to harm. An appropriate level of PEEP for neonates may also be best achieved by an individualized approach. 1. To compare the effects of different levels of PEEP in preterm newborn infants requiring CMV for respiratory distress syndrome (RDS).2. To compare the effects of different levels of PEEP in preterm infants requiring CMV for bronchopulmonary dysplasia (BPD).3. To compare the effects of different methods for individualizing PEEP to an optimal level in preterm newborn infants requiring CMV for RDS. The search was performed in accordance with the standard search strategy for the Cochrane Neonatal Review Group. The Cochrane Central Register of Controlled Trials (CENTRAL) (The Cochrane Library), Ovid MEDLINE, EMBASE, study references and experts were utilized for study identification. All randomized and quasi-randomized controlled trials studying preterm infants (less than 37 weeks gestational age) requiring CMV with endotracheal intubation and undergoing randomization to either different PEEP levels (RDS or BPD) or two or more alternative methods for individualizing PEEP levels (RDS only) were included. Cross-over trials were included but we limited the findings to those in the first cross-over period. Data collection and analysis were performed in accordance with the recommendations of the Cochrane Neonatal Review Group. An initial evaluation identified 10 eligible articles. Ultimately, a single study met our inclusion criteria. The study addressed the effects of different

  19. Development of a subset of forelimb muscles and their attachment sites requires the ulnar-mammary syndrome gene Tbx3

    Directory of Open Access Journals (Sweden)

    Mary P. Colasanto

    2016-11-01

    Full Text Available In the vertebrate limb over 40 muscles are arranged in a precise pattern of attachment via muscle connective tissue and tendon to bone and provide an extensive range of motion. How the development of somite-derived muscle is coordinated with the development of lateral plate-derived muscle connective tissue, tendon and bone to assemble a functional limb musculoskeletal system is a long-standing question. Mutations in the T-box transcription factor, TBX3, have previously been identified as the genetic cause of ulnar-mammary syndrome (UMS, characterized by distinctive defects in posterior forelimb bones. Using conditional mutagenesis in mice, we now show that TBX3 has a broader role in limb musculoskeletal development. TBX3 is not only required for development of posterior forelimb bones (ulna and digits 4 and 5, but also for a subset of posterior muscles (lateral triceps and brachialis and their bone eminence attachment sites. TBX3 specification of origin and insertion sites appears to be tightly linked with whether these particular muscles develop and may represent a newly discovered mechanism for specification of anatomical muscles. Re-examination of an individual with UMS reveals similar previously unrecognized muscle and bone eminence defects and indicates a conserved role for TBX3 in regulating musculoskeletal development.

  20. Relationship between red blood cell transfusion requirements and severity of renal disease during the acute stage of hemolytic uremic syndrome.

    Science.gov (United States)

    Cobeñas, Carlos J; Bresso, Paula S; Lombardi, Laura L; Amoreo, Oscar R; Ruscasso, Javier D; Spizzirri, Ana P; Del C Suarez, Ângela; Zalba, Javier H; Rahman, Ricardo C; Risso, Paula

    2015-12-01

    We performed a retrospective evaluation of patients with diarrhea-associated hemolytic uremic syndrome (D + HUS) with the aims of: (1) determining the rate of red blood cell (RBC) transfusions; (2) establishing the relationship between need for RBC transfusion and severity of renal involvement; (3) determining whether precise measurements of lactic dehydrogenase (LDH) levels can predict the rate of hemolysis and severity of renal disease. A total of 288 patients with D + HUS were retrospectively divided into three groups based on dialysis treatment: group 1, no dialysis treatment (144 patients); group 2, dialysis for 1-10 days (67 patients); group 3, dialysis for ≥11 days (77 patients). Of the patients in groups 1, 2 and 3, 73.6, 86.5 and 83.1%, respectively, required at least one RBC transfusion. The number of RBC transfusions in groups 1, 2 and 3 was 163, 107 and 162, respectively. Comparison of the groups revealed that the number of RBC transfusions was significantly higher in patients in groups 2 and 3 than in those in group 1 (p = 0.0001). Most RBC transfusions (94.2%) occurred during the first 2 weeks of the disease. The median peak LDH level was 2091 U/l in 32 patients with no RBC transfusion (group A), 3900 U/l in 73 patients with one transfusion (group B) and 6378 U/l in 62 patients with two or more transfusions (group C). Patients who received two or more RBC transfusions had a significantly higher median peak LDH level than those who did not receive RBC transfusions or received only one transfusion. This difference was also observed between patients who received only one RBC transfusion and those who did not receive any transfusions (p 10 days of dialysis (group 3) had the highest LDH levels, followed by patients with 1-10 days of dialysis (group 2) and then by patients with no dialysis requirements (group 1) (p < 0.00001). The rate of RBC transfusion was higher in patients with the most severe renal injury, and most were performed during the

  1. Mechanical ventilation strategies for intensive care unit patients without acute lung injury or acute respiratory distress syndrome: a systematic review and network meta-analysis.

    Science.gov (United States)

    Guo, Lei; Wang, Weiwei; Zhao, Nana; Guo, Libo; Chi, Chunjie; Hou, Wei; Wu, Anqi; Tong, Hongshuang; Wang, Yue; Wang, Changsong; Li, Enyou

    2016-07-22

    It has been shown that the application of a lung-protective mechanical ventilation strategy can improve the prognosis of patients with acute lung injury (ALI) or acute respiratory distress syndrome (ARDS). However, the optimal mechanical ventilation strategy for intensive care unit (ICU) patients without ALI or ARDS is uncertain. Therefore, we performed a network meta-analysis to identify the optimal mechanical ventilation strategy for these patients. We searched the Cochrane Central Register of Controlled Trials (CENTRAL) in the Cochrane Library, EMBASE, MEDLINE, CINAHL, and Web of Science for studies published up to July 2015 in which pulmonary compliance or the partial pressure of arterial oxygen/fraction of inspired oxygen (PaO2/FIO2) ratio was assessed in ICU patients without ALI or ARDS, who received mechanical ventilation via different strategies. The data for study characteristics, methods, and outcomes were extracted. We assessed the studies for eligibility, extracted the data, pooled the data, and used a Bayesian fixed-effects model to combine direct comparisons with indirect evidence. Seventeen randomized controlled trials including a total of 575 patients who received one of six ventilation strategies were included for network meta-analysis. Among ICU patients without ALI or ARDS, strategy C (lower tidal volume (VT) + higher positive end-expiratory pressure (PEEP)) resulted in the highest PaO2/FIO2 ratio; strategy B (higher VT + lower PEEP) was associated with the highest pulmonary compliance; strategy A (lower VT + lower PEEP) was associated with a shorter length of ICU stay; and strategy D (lower VT + zero end-expiratory pressure (ZEEP)) was associated with the lowest PaO2/FiO2 ratio and pulmonary compliance. For ICU patients without ALI or ARDS, strategy C (lower VT + higher PEEP) was associated with the highest PaO2/FiO2 ratio. Strategy B (higher VT + lower PEEP) was superior to the other strategies in improving pulmonary

  2. An observational study on the association of nonalcoholic fatty liver disease and metabolic syndrome with gall stone disease requiring cholecystectomy

    Directory of Open Access Journals (Sweden)

    Farah Ahmed

    2017-05-01

    Conclusion: We found association of metabolic syndrome with gallstones and NAFLD. Non alcoholic fatty liver was highly prevalent in our study subjects. Huge percentage of first degree relatives of gall stone patients had gallstones and this relation was more pronounced patients who had associated NAFLD.

  3. The Effect of High-Intensity Interval Training Versus Strength Training to Improve Body Composition in Women With Polycystic Ovary Syndrome - A Randomized Controlled Trial

    OpenAIRE

    Almenning, Ida

    2014-01-01

    Background: Polycystic ovary syndrome (PCOS) is a common endocrine disorder affecting 6-20% of reproductive-age women. Women with PCOS tend to accumulate more upper body fat, and the syndrome is often present with abdominal obesity and visceral adiposity which is further associated with metabolic – and cardiovascular complications. Despite well-established benefits of exercise and its recommendation in PCOS management, few randomized controlled trials have evaluated the...

  4. Usher Syndrome

    Science.gov (United States)

    ... with age. Decline in hearing and vision varies. Children with type 3 Usher syndrome often develop hearing loss by adolescence, requiring hearing aids by mid-to-late adulthood. Night blindness also usually begins during adolescence. Blind spots appear ...

  5. FOXP3 mutations causing early-onset insulin-requiring diabetes but without other features of immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome.

    Science.gov (United States)

    Hwang, Jessica L; Park, Soo-Young; Ye, Honggang; Sanyoura, May; Pastore, Ashley N; Carmody, David; Del Gaudio, Daniela; Wilson, Janna F; Hanis, Craig L; Liu, Xiaoming; Atzmon, Gil; Glaser, Benjamin; Philipson, Louis H; Greeley, Siri Atma W

    2017-11-29

    Diabetes occurs in 1/90 000 to 1/160 000 births and when diagnosed under 6 months of age is very likely to have a primary genetic cause. FOXP3 encodes a transcription factor critical for T regulatory cell function and mutations are known to cause "immune dysregulation, polyendocrinopathy (including insulin-requiring diabetes), enteropathy, X-linked" (IPEX) syndrome. This condition is often fatal unless patients receive a bone-marrow transplant. Here we describe the phenotype of male neonates and infants who had insulin-requiring diabetes without other features of IPEX syndrome and were found to have mutations in FOXP3. Whole-exome or next generation sequencing of genes of interest was carried out in subjects with isolated neonatal diabetes without a known genetic cause. RT-PCR was carried out to investigate the effects on RNA splicing of a novel intronic splice-site variant. Four male subjects were found to have FOXP3 variants in the hemizygous state: p.Arg114Trp, p.Arg347His, p.Lys393Met, and c.1044+5G>A which was detected in 2 unrelated probands and in a brother diagnosed with diabetes at 2.1 years of age. Of these, p.Arg114Trp is likely a benign rare variant found in individuals of Ashkenazi Jewish ancestry and p.Arg347His has been previously described in patients with classic IPEX syndrome. The p.Lys393Met and c.1044+5G>A variants are novel to this study. RT-PCR studies of the c.1044+5G>A splice variant confirmed it affected RNA splicing by generating both a wild type and truncated transcript. We conclude that FOXP3 mutations can cause early-onset insulin-requiring diabetes with or without other features of IPEX syndrome. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  6. Effects of intensive glucose control on platelet reactivity in patients with acute coronary syndromes. Results of the CHIPS Study ("Control de Hiperglucemia y Actividad Plaquetaria en Pacientes con Sindrome Coronario Agudo").

    Science.gov (United States)

    Vivas, David; García-Rubira, Juan C; Bernardo, Esther; Angiolillo, Dominick J; Martín, Patricia; Calle-Pascual, Alfonso; Núñez-Gil, Iván; Macaya, Carlos; Fernández-Ortiz, Antonio

    2011-05-01

    Hyperglycaemia has been associated with increased platelet reactivity and impaired prognosis in patients with acute coronary syndrome (ACS). Whether platelet reactivity can be reduced by lowering glucose in this setting is unknown. The aim of this study was to assess the functional impact of intensive glucose control with insulin on platelet reactivity in patients admitted with ACS and hyperglycaemia. This is a prospective, randomised trial evaluating the effects of either intensive glucose control (target glucose 80-120 mg/dl) or conventional control (target glucose 180 mg/dl or less) with insulin on platelet reactivity in patients with ACS and hyperglycaemia. The primary endpoint was platelet aggregation following stimuli with 20 μM ADP at 24 h and at hospital discharge. Aggregation following collagen, epinephrine and thrombin receptor-activated peptide, as well as P2Y₁₂ reactivity index and surface expression of glycoprotein IIb/IIIa and P-selectin were also measured. Of the 115 patients who underwent random assignment, 59 were assigned to intensive and 56 to conventional glucose control. Baseline platelet functions and inhospital management were similar in both groups. Maximal aggregation after ADP stimulation at hospital discharge was lower in the intensive group (47.9 ± 13.2% vs 59.1 ± 17.3%; p=0.002), whereas no differences were found at 24 h. Similarly all other parameters of platelet reactivity measured at hospital discharge were significantly reduced in the intensive glucose control group. In this randomised trial, early intensive glucose control with insulin in patients with ACS presenting with hyperglycaemia was found to decrease platelet reactivity. Clinical Trial Registration Number http://www.controlledtrials.com/ISRCTN35708451/ISRCTN35708451.

  7. Graft intolerance syndrome requiring graft nephrectomy after late kidney graft failure: can it be predicted? A retrospective cohort study.

    Science.gov (United States)

    Bunthof, Kim L W; Verhoeks, Carmen M; van den Brand, Jan A J G; Hilbrands, Luuk B

    2018-02-01

    Graft nephrectomy is recommended in case of early graft failure. When the graft fails more than 3-6 months after transplantation, it is current practice to follow a wait-and-see policy. A common indication for graft removal is the graft intolerance syndrome. We aimed to create a risk prediction model for the occurrence of graft intolerance resulting in graft nephrectomy. We collected data of kidney transplantations performed in our center between 1980 and 2010 that failed at least 6 months after transplantation. We evaluated the association between baseline characteristics and the occurrence of graft nephrectomy because of graft intolerance using a competing risk regression model. Prognostic factors were included in a multivariate prediction model. In- and exclusion criteria were met in 288 cases. In 48 patients, the graft was removed because of graft intolerance. Donor age, the number of rejections, and shorter graft survival were predictive factors for graft nephrectomy because of the graft intolerance syndrome. These factors were included in a prediction rule. Using donor age, graft survival, and the number of rejections, clinicians can predict the need for graft nephrectomy with a reasonable accuracy. © 2017 Steunstichting ESOT.

  8. Hepatorenal Syndrome

    Directory of Open Access Journals (Sweden)

    Pınar Zeyneloğlu

    2012-04-01

    Full Text Available Renal failure is a common major complication in patients with advanced cirrhosis and generally indicates a poor prognosis when combined with liver failure. Hepatorenal syndrome (HRS is characterised by a combination of disturbances in circulatory and kidney function. Arterial pressure is decreased in the systemic circulation due to reduced total systemic vascular resistance. Kidney dysfunction is caused by reduction in renal blood flow. The diagnosis of HRS is based on exclusion of other disorders that cause acute kidney injury in cirrhosis as there are no specific tests. There are two types of HRS with different characteristics and prognostics. Liver transplantation is the treatment of choice for all patients without contraindication. The best approach to the pharmacologic management is the administration vasoconstrictor drugs based on the pathogenesis. Many vasoconstrictors including vasopressin analogues (terlipressin, ornipressin and vasopressin, somatostatin analogues (octreotide and alpha-adrenergic analogues (midodrine and norepinephrine have been studied. In most of the studies intravenous albumin therapy was coadministered with vasoconstrictor drugs and suggested that albumin should be considered as the component of pharmacologic intervention in patients with HRS. Renal replacement therapy in the form of hemodialysis or continuous venovenous hemofiltration has been used in the management of HRS patients awaiting transplantation or in those with acute potentially reversible conditions. The artificial hepatic support systems require further investigation. (Journal of the Turkish Society Intensive Care 2012; 10: 37-44

  9. Trabalho e síndrome da estafa profissional (Síndrome de Burnout em médicos intensivistas de Salvador Professional Burnout Syndrome among intensive care physicians in Salvador, Brazil

    Directory of Open Access Journals (Sweden)

    Márcia Oliveira Staffa Tironi

    2009-01-01

    Full Text Available OBJETIVO: Descrever a prevalência da Síndrome de Burnout em médicos intensivistas de Salvador, associando-a a dados demográficos e aspectos da situação de trabalho (demanda psicológica e controle sobre o trabalho. MÉTODOS: Um estudo de corte transversal investigou a associação entre aspectos psicossocias do trabalho e a síndrome da estafa profissional em uma população de 297 médicos intensivistas de Salvador, Bahia. Um questionário individual autoaplicável avaliou aspectos psicossociais do trabalho, utilizando o modelo demanda-controle (Job Content Questionnaire e a saúde mental dos médicos, usando Inventário de Burnout de Maslach (MBI. RESULTADOS: Constatou-se elevada sobrecarga de trabalho e de trabalho em regime de plantão. A prevalência da Síndrome da Estafa Profissional (Burnout foi de 7,4% e estava mais fortemente associada com aspectos da demanda psicológica do trabalho do que com o controle deste por parte dos médicos intensivistas. CONCLUSÃO: Médicos com trabalho de alta exigência (alta demanda e baixo controle apresentaram 10,2 vezes mais burnout que aqueles com trabalho de baixa exigência (baixa demanda e alto controle.OBJECTIVE: Describe prevalence of the Burnout syndrome in intensive care physicians of Salvador, associated to demographic data and aspects of the work environment (psychological demand and job control. METHODS: This cross sectional study has investigated the association between work conditions and Burnout Syndrome in a population of 297 Intensive Care Physicians from Salvador, Bahia, Brazil. An individual, self-report questionnaire evaluated the physicians' psychological aspects of work, using the demand-control model (Job Content Questionnaire and their mental health, using the Maslash Burnout Inventory (MBI. RESULTS: The study found work overload,a high proportion of on duty physicians and low income for the hours worked. Prevalence of the Burnout Syndrome was 7.4% and it was more closely

  10. Guillain-Barre Syndrome – rehabilitation outcome, residual deficits and requirement of lower limb orthosis for locomotion at 1 year follow-up.

    Science.gov (United States)

    Gupta, Anupam; Taly, Arun B; Srivastava, Abhishek; Murali, Thyloth

    2010-01-01

    To analyse long-term functional recovery, deficits and requirement of lower limb orthosis (LLO) for locomotion in patients with Guillain-Barre Syndrome (GBS). Prospective longitudinal follow-up study. Neurological Rehabilitation unit of university hospital. Sixty-nine patients of GBS admitted for inpatient rehabilitation. Thirty-five patients (M:F, 19:16) reporting after 1 year follow-up (50.72%) were included in study (between September 2005 and July 2009). Their residual deficits and requirement of LLO were recorded and analysed. Age ranged from 4 to 65 year (29.74 ± 15.75). Twenty-seven patients had typical GBS and eight patients had acute motor axonal neuropathy variant. Twenty-eight patients (80%) had neuropathic pain needing medication with 11 required more than one drug. Twenty-one patients (60%) had foot drop and advised ankle-foot orthosis-AFO (20 bilateral AFO). Thirty patients (85.71%) needed assistive devices also for locomotion at discharge. After 1 year, foot drop was still present in 12 patients (34.28%) using orthosis. Modified Barthel Index scores, Modified Rankin Scale and Hughes Disability Scale were used to assess functional disabilities. Significant recovery was observed at the time of discharge and after 1 year (p < 0.001 each). Patients with GBS continue to show significant functional recovery for long period. They have residual deficits even after 1 year with requirement of orthosis in large number of patients.

  11. SLC25A46 is required for mitochondrial lipid homeostasis and cristae maintenance and is responsible for Leigh syndrome.

    Science.gov (United States)

    Janer, Alexandre; Prudent, Julien; Paupe, Vincent; Fahiminiya, Somayyeh; Majewski, Jacek; Sgarioto, Nicolas; Des Rosiers, Christine; Forest, Anik; Lin, Zhen-Yuan; Gingras, Anne-Claude; Mitchell, Grant; McBride, Heidi M; Shoubridge, Eric A

    2016-09-01

    Mitochondria form a dynamic network that responds to physiological signals and metabolic stresses by altering the balance between fusion and fission. Mitochondrial fusion is orchestrated by conserved GTPases MFN1/2 and OPA1, a process coordinated in yeast by Ugo1, a mitochondrial metabolite carrier family protein. We uncovered a homozygous missense mutation in SLC25A46, the mammalian orthologue of Ugo1, in a subject with Leigh syndrome. SLC25A46 is an integral outer membrane protein that interacts with MFN2, OPA1, and the mitochondrial contact site and cristae organizing system (MICOS) complex. The subject mutation destabilizes the protein, leading to mitochondrial hyperfusion, alterations in endoplasmic reticulum (ER) morphology, impaired cellular respiration, and premature cellular senescence. The MICOS complex is disrupted in subject fibroblasts, resulting in strikingly abnormal mitochondrial architecture, with markedly shortened cristae. SLC25A46 also interacts with the ER membrane protein complex EMC, and phospholipid composition is altered in subject mitochondria. These results show that SLC25A46 plays a role in a mitochondrial/ER pathway that facilitates lipid transfer, and link altered mitochondrial dynamics to early-onset neurodegenerative disease and cell fate decisions. © 2016 The Authors. Published under the terms of the CC BY 4.0 license.

  12. Transfusion requirements and clinical outcome in intensive care patients receiving continuous renal replacement therapy: comparison of prostacyclin vs. heparin prefilter administration

    DEFF Research Database (Denmark)

    Windeløv, Nis Agerlin; Ostrowski, Sisse R; Perner, Anders

    2010-01-01

    patients at a general intensive care unit (ICU) receiving continuous RRT (CRRT) in a 14-month period. Patients were stratified according to the used anticoagulant, that is prefilter PGI(2) group (n=24) and prefilter heparin group (n=70). The ICU stay of the patients was divided into three time periods...

  13. Acute respiratory failure following ovarian hyperstimulation syndrome

    Directory of Open Access Journals (Sweden)

    Antonello Nicolini

    2013-03-01

    Full Text Available Ovarian hyperstimulation syndrome is a serious and potentially life-threatening physiological complication that may be encountered in patients who undergo controlled ovarian hyperstimulation cycles. The syndrome is typically associated with regimes of exogenous gonadotropins, but it can be seen, albeit rarely, when clomiphene is administered during the induction phase. Although this syndrome is widely described in scientific literature and is well known by obstetricians, the knowledge of this pathological and potentially life-threatening condition is generally less than satisfactory among physicians. The dramatic increase in therapeutic strategies to treat infertility has pushed this condition into the realm of acute care therapy. The potential complications of this syndrome, including pulmonary involvement, should be considered and identified so as to allow a more appropriate diagnosis and management. We describe a case of a woman with an extremely severe (Stage 6 ovarian hyperstimulation syndrome who presented ascites, bilateral pleural effusion and severe respiratory failure treated with non-invasive ventilation. The patient was admitted to the intensive care unit because of severe respiratory failure, ascites, and bilateral pleural effusion due to ovarian hyperstimulation syndrome. Treatment included non-invasive ventilation and three thoracentesis procedures, plus the administration of albumin, colloid solutions and high-dose furosemid. Severe form of ovarian hyperstimulation syndrome is observed in 0.5-5% of the women treated, and intensive care may be required for management of thromboembolic complications, renal failure and severe respiratory failure. Pulmonary intensive care may involve thoracentesis, oxygen supplementation and, in more severe cases, assisted ventilation. To our knowledge, there have been only two studies in English language medical literature that describe severe respiratory failure treated with non

  14. The Warsaw breakage syndrome-related protein DDX11 is required for ribosomal RNA synthesis and embryonic development.

    Science.gov (United States)

    Sun, Xinliang; Chen, Hongbo; Deng, Zaian; Hu, Bo; Luo, Hui; Zeng, Xiaobin; Han, Liqiao; Cai, Guoping; Ma, Lan

    2015-09-01

    DDX11 was recently identified as a cause of Warsaw breakage syndrome (WABS). However, the functional mechanism of DDX11 and the contribution of clinically described mutations to the pathogenesis of WABS are elusive. Here, we show that DDX11 is a novel nucleolar protein that preferentially binds to hypomethylated active ribosomal DNA (rDNA) gene loci, where it interacts with upstream binding factor (UBF) and the RNA polymerase I (Pol I). DDX11 knockdown changed the epigenetic state of rDNA loci from euchromatic structures to more heterochromatic structures, reduced the activity of UBF, decreased the recruitment of UBF and RPA194 (a subunit of Pol I) to rDNA promoter, suppressed rRNA transcription and thereby inhibited growth and proliferation of HeLa cells. Importantly, two indentified WABS-derived mutants, R263Q and K897del, and a Fe-S deletion construct demonstrated significantly reduced binding abilities to rDNA promoters and lowered DNA-dependent ATPase activities compared with wild-type DDX11. Knockdown of the zebrafish ortholog of human DDX11 by morpholinos resulted in growth retardation and vertebral and craniofacial malformations in zebrafish, concomitant with the changes in histone epigenetic modifications at rDNA loci, the reduction of Pol I recruitment to the rDNA promoter and a significant decrease in nascent pre-RNA levels. These growth disruptions in zebrafish in response to DDX11 reduction showed similarities to the clinically described developmental abnormalities found in WABS patients for the first time in any vertebrate. Thus, our results indicate that DDX11 functions as a positive regulator of rRNA transcription and provides a novel insight into the pathogenesis of WABS. © The Author 2015. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  15. Role and Place of omega-3 Fatty Acids in the Regulation of Systemic Inflammatory Response Syndrome in Patients Receiving Intensive Care

    OpenAIRE

    Matolinets, N.V.

    2017-01-01

    В статье проанализированы современные научные данные о роли ω-3 жирных кислот в комплексе нутритивной поддержки у больных в критическом состоянии, а также влияние на динамику синдрома общего ответа на воспаление (Systemic Inflammatory Response Syndrome, SIRS), синдрома противовоспалительного ответа (Сompensatory Anti-inflammatory Response Syndrome, CARS), синдрома смешанного антагонистического действия (Mixed Antagonistic Response Syndrome, MARS), развивающегося при взаимодействии разнонаправ...

  16. Measuring Physical Activity Intensity

    Medline Plus

    Full Text Available ... level of effort required by a person to do an activity. When using relative intensity, people pay ... State and Local Programs File Formats Help: How do I view different file formats (PDF, DOC, PPT, ...

  17. Serotonin syndrome

    Science.gov (United States)

    Hyperserotonemia; Serotonergic syndrome; Serotonin toxicity; SSRI - serotonin syndrome; MAO - serotonin syndrome ... brain area. For example, you can develop this syndrome if you take migraine medicines called triptans together ...

  18. The stress ulcer syndrome

    NARCIS (Netherlands)

    H.A. van Essen

    1986-01-01

    textabstractThe stress ulcer syndrome is described in this thesis. This syndrome is seen in patients admitted to intensive care departments or being treated in field hospitals, in disaster areas, or battle fields. Acute mucosal lesions associated with burns (Curling's ulcers) and central nervous

  19. Musculocontractural Ehlers–Danlos syndrome and neurocristopathies: dermatan sulfate is required for Xenopus neural crest cells to migrate and adhere to fibronectin

    Directory of Open Access Journals (Sweden)

    Nadège Gouignard

    2016-06-01

    Full Text Available Of all live births with congenital anomalies, approximately one-third exhibit deformities of the head and face. Most craniofacial disorders are associated with defects in a migratory stem and progenitor cell population, which is designated the neural crest (NC. Musculocontractural Ehlers–Danlos syndrome (MCEDS is a heritable connective tissue disorder with distinct craniofacial features; this syndrome comprises multiple congenital malformations that are caused by dysfunction of dermatan sulfate (DS biosynthetic enzymes, including DS epimerase-1 (DS-epi1; also known as DSE. Studies in mice have extended our understanding of DS-epi1 in connective tissue maintenance; however, its role in fetal development is not understood. We demonstrate that DS-epi1 is important for the generation of isolated iduronic acid residues in chondroitin sulfate (CS/DS proteoglycans in early Xenopus embryos. The knockdown of DS-epi1 does not affect the formation of early NC progenitors; however, it impairs the correct activation of transcription factors involved in the epithelial–mesenchymal transition (EMT and reduces the extent of NC cell migration, which leads to a decrease in NC-derived craniofacial skeleton, melanocytes and dorsal fin structures. Transplantation experiments demonstrate a tissue-autonomous role for DS-epi1 in cranial NC cell migration in vivo. Cranial NC explant and single-cell cultures indicate a requirement of DS-epi1 in cell adhesion, spreading and extension of polarized cell processes on fibronectin. Thus, our work indicates a functional link between DS and NC cell migration. We conclude that NC defects in the EMT and cell migration might account for the craniofacial anomalies and other congenital malformations in MCEDS, which might facilitate the diagnosis and development of therapies for this distressing condition. Moreover, the presented correlations between human DS-epi1 expression and gene sets of mesenchymal character, invasion and

  20. Low-dose decitabine versus best supportive care in elderly patients with intermediate- or high-risk myelodysplastic syndrome (MDS) ineligible for intensive chemotherapy: final results of the randomized phase III study of the European Organisation for Research and Treatment of Cancer Leukemia Group and the German MDS Study Group

    NARCIS (Netherlands)

    Lubbert, M.; Suciu, S.; Baila, L.; Ruter, B.H.; Platzbecker, U.; Giagounidis, A.; Selleslag, D.; Labar, B.; Germing, U.; Salih, H.R.; Beeldens, F.; Muus, P.; Pfluger, K.H.; Coens, C.; Hagemeijer, A.; Eckart Schaefer, H.; Ganser, A.; Aul, C.; Witte, T.J.M. de; Wijermans, P.W.

    2011-01-01

    PURPOSE: To compare low-dose decitabine to best supportive care (BSC) in higher-risk patients with myelodysplastic syndrome (MDS) age 60 years or older and ineligible for intensive chemotherapy. PATIENTS AND METHODS: Two-hundred thirty-three patients (median age, 70 years; range, 60 to 90 years)

  1. The effects of low-intensity narrow-band blue-light treatment compared to bright white-light treatment in sub-syndromal seasonal affective disorder.

    Science.gov (United States)

    Meesters, Ybe; Winthorst, Wim H; Duijzer, Wianne B; Hommes, Vanja

    2016-02-18

    The discovery of a novel photoreceptor in the retinal ganglion cells with a highest sensitivity of 470-490 nm blue light has led to research on the effects of short-wavelength light in humans. Several studies have explored the efficacy of monochromatic blue or blue-enriched light in the treatment of SAD. In this study, a comparison has been made between the effects of broad-wavelength light without ultraviolet (UV) wavelengths compared to narrow-band blue light in the treatment of sub-syndromal seasonal affective disorder (Sub-SAD). In a 15-day design, 48 participants suffering from Sub-SAD completed 20-minute sessions of light treatment on five consecutive days. 22 participants were given bright white-light treatment (BLT, broad-wavelength light without UV 10 000 lux, irradiance 31.7 Watt/m(2)) and 26 participants received narrow-band blue light (BLUE, 100 lux, irradiance 1.0 Watt/m(2)). All participants completed daily and weekly questionnaires concerning mood, activation, sleep quality, sleepiness and energy. Also, mood and energy levels were assessed by means of the SIGH-SAD, the primary outcome measure. On day 15, SIGH-SAD ratings were significantly lower than on day 1 (BLT 54.8 %, effect size 1.7 and BLUE 50.7 %, effect size 1.9). No statistically significant differences were found on the main outcome measures. Light treatment is an effective treatment for Sub-SAD. The use of narrow-band blue-light treatment is equally effective as bright white-light treatment. This study was registered in the Dutch Trial Register (Nederlands Trial Register TC =  4342 ) (20-12-2013).

  2. Fragile X syndrome associated with tic disorders.

    Science.gov (United States)

    Schneider, Susanne A; Robertson, Mary M; Rizzo, Renata; Turk, Jeremy; Bhatia, Kailash P; Orth, Michael

    2008-06-15

    Movement disorders other than late onset tremor-ataxia in association with fragile X syndrome, the most common identifiable cause of inherited mental retardation, seem to be rare. Here we describe five male patients from three unrelated families with fragile X syndrome that presented with motor and phonic tics. Clinically, 4 patients fulfilled diagnostic criteria for Gilles de la Tourette syndrome (GTS) while 1 patient would have been diagnosed with an adult onset tic disorder. However, in all patients onset of tics was considerably later than in typical GTS. Three patients had atypical tics and two patients reported waxing and waning of tic intensity over time. Four of the 5 patients showed clinical signs typical of fragile X syndrome, in particular dysmorphic features, learning difficulties and speech and language problems that required special treatment. All patients had co-morbidities common to both GTS and fragile X syndrome. We suggest considering fragile X syndrome in GTS complicated by co-morbidity with late onset of atypical tics, in particular when learning disability and dysmorphic features are present. (c) 2008 Movement Disorder Society

  3. Beals Syndrome

    Science.gov (United States)

    ... the syndrome. How does Beals syndrome compare with Marfan syndrome? People with Beals syndrome have many of the ... bone) and aortic enlargement problems as people with Marfan syndrome, and treatments for these problems are the same. ...

  4. Eagle's Syndrome

    Directory of Open Access Journals (Sweden)

    Pinheiro, Thaís Gonçalves

    2014-01-01

    Full Text Available Introduction: Eagle's syndrome is characterized by cervicopharyngeal signs and symptoms associated with elongation of the styloid apophysis. This elongation may occur through ossification of the stylohyoid ligament, or through growth of the apophysis due to osteogenesis triggered by a factor such as trauma. Elongation of the styloid apophysis may give rise to intense facial pain, headache, dysphagia, otalgia, buzzing sensations, and trismus. Precise diagnosis of the syndrome is difficult, and it is generally confounded by other manifestations of cervicopharyngeal pain. Objective: To describe a case of Eagle's syndrome. Case Report: A 53-year-old man reported lateral pain in his neck that had been present for 30 years. Computed tomography (CT of the neck showed elongation and ossification of the styloid processes of the temporal bone, which was compatible with Eagle's syndrome. Surgery was performed for bilateral resection of the stylohyoid ligament by using a transoral and endoscopic access route. The patient continued to present pain laterally in the neck, predominantly on his left side. CT was performed again, which showed elongation of the styloid processes. The patient then underwent lateral cervicotomy with resection of the stylohyoid process, which partially resolved his painful condition. Final Comments: Patients with Eagle's syndrome generally have a history of chronic pain. Appropriate knowledge of this disease is necessary for adequate treatment to be provided. The importance of diagnosing this uncommon and often unsuspected disease should be emphasized, given that correct clinical-surgical treatment is frequently delayed. The diagnosis of Eagle's syndrome is clinical and radiographic, and the definitive treatment in cases of difficult-to-control pain is surgical.

  5. Geriatric patients with known acute kidney injury and normal renal function at the time of admittance to the intensive care unit/assessment of RRT requirement and mortality: retrospective case-control study.

    Science.gov (United States)

    Karakose, Fatmanur; Akkoyunlu, Muhammet Emin; Erkoc, Reha; Kansu, Abdullah; Bayram, Mehmet; Sezer, Murat; Ozcelik, Hatice; Kart, Levent

    2015-04-01

    The objective of our study was to investigate the renal functions on admittance, chronic disease status, the diagnosis on admittance to the intensive care unit (ICU), need for mechanical ventilation and medication groups and their impact over mortality and renal replacement treatment (RRT) requirement in geriatric patients with and without known acute kidney injury (AKI) at the time of admittance. A total of 168 patients over 65 years of age having been monitored for more than 24 h in our Respiratory ICU and were assessed retrospectively. Factors influencing the RRT requirement and the mortality rates of patients with known AKI and normal renal function at the time of admittance were reviewed. Of 80 patients, 8 (10 %) without AKI at the time of admittance to the ICU required RRT during the follow-up, however, 72 of those (90 %) completed the follow-up without developing AKI. Of 88 patients, 29 (33 %) with AKI at the time of admittance to the ICU required RRT, however, 59 of those (67 %) completed the follow-up without any need for RRT. Presence of known AKI in the geriatric population at the time of their admittance to the respiratory ICU remarkably increased both the need for RRT and the mortality with respect to the geriatric population with normal renal functions. After having assessed the reasons justifying RRT and increasing the mortality during the intensive care, gastrointestinal bleeding and hypotension which necessitate the combined use of dopamine and noradrenaline were concluded to be prominent.

  6. Upfront allogeneic stem cell transplantation after reduced-intensity/nonmyeloablative conditioning for patients with myelodysplastic syndrome: a study by the Société Française de Greffe de Moelle et de Thérapie Cellulaire.

    Science.gov (United States)

    Damaj, Gandhi; Mohty, Mohammad; Robin, Marie; Michallet, Mauricette; Chevallier, Patrice; Beguin, Yves; Nguyen, Stephanie; Bories, Pierre; Blaise, Didier; Maillard, Natacha; Rubio, Marie Therese; Fegueux, Nathalie; Cornillon, Jerome; Clavert, Aline; Huynh, Anne; Adès, Lionel; Thiébaut-Bertrand, Anne; Hermine, Olivier; Vigouroux, Stephane; Fenaux, Pierre; Duhamel, Alain; Yakoub-Agha, Ibrahim

    2014-09-01

    Cytoreduction before allogeneic stem cell transplantation (allo-SCT) for patients with myelodysplastic syndromes remains a debatable issue. After excluding patients who had received preconditioning induction chemotherapy, we analyzed 128 consecutive patients with myelodysplastic syndrome who received reduced-intensity or nonmyeloablative conditioning (RIC/NMA) allo-SCT. Among them, 40 received azacitidine (AZA) before transplant (AZA group) and 88 were transplanted up front (best supportive care [BSC] group). At diagnosis, 55 patients had intermediate 2 or high-risk scores per the International Prognostic Scoring System and 33 had a high cytogenetic risk score. Progression to a more advanced disease before allo-SCT was recorded in 22 patients. Source of stem cells were blood (n = 112) or marrow (n = 16) from sibling (n = 78) or HLA-matched unrelated (n = 50) donors. With a median follow-up of 60 months, 3-year overall survival, relapse-free survival, cumulative incidence of relapse, and nonrelapse mortality were, respectively, 53% versus 53% (P = .69), 37% versus 42% (P = .78), 35% versus 36% (P = .99), and 20% versus 23% (P = .74), for the AZA group and BSC group, respectively. Multivariate analysis confirmed the absence of statistical differences in outcome between the AZA and BSC groups, after adjusting for potential confounders using the propensity score approach. The absence of cytoreduction before RIC/NMA allo-SCT did not seem to alter the outcome. However, our results emphasize the need to perform prospective protocols to delineate the role of debulking strategy and to identify subsets of patients who may benefit from this approach. Copyright © 2014 American Society for Blood and Marrow Transplantation. Published by Elsevier Inc. All rights reserved.

  7. [Personnel requirements of medical radiation physics in radiotherapy in comparison to the current guidelines "radiation protection in medicine" : Special consideration of intensity-modulated radiation therapy].

    Science.gov (United States)

    Leetz, H-K; Eipper, H H; Gfirtner, H; Schneider, P; Welker, K

    2014-08-01

    In 1994 and 1998 reports on staffing levels in medical radiation physics for radiation therapy were published by the "Deutsche Gesellschaft für Medizinische Physik" (DGMP, German Society for Medical Physics). Because of the technical and methodological progress, changes in recommended qualifications of staff and new governmental regulations, it was necessary to establish new staffing levels. The data were derived from a new survey in clinics. Some of the previously established results from the old reports were adapted to the new conditions by conversion.The staffing requirements were normalized to main components as in the earlier reports resulting in a simple method for calculation of staffing levels. The results were compared with the requirements in the "Richtlinie Strahlenschutz in der Medizin" (guidelines on radiation protection in medicine) and showed satisfactory agreement.

  8. Wiskott-Aldrich syndrome protein is required for NK cell cytotoxicity and colocalizes with actin to NK cell-activating immunologic synapses

    Science.gov (United States)

    Orange, Jordan S.; Ramesh, Narayanaswamy; Remold-O'Donnell, Eileen; Sasahara, Yoji; Koopman, Louise; Byrne, Michael; Bonilla, Francisco A.; Rosen, Fred S.; Geha, Raif S.; Strominger, Jack L.

    2002-08-01

    The Wiskott-Aldrich syndrome (WAS) is a primary immunodeficiency disorder caused by a mutation in WAS protein (WASp) that results in defective actin polymerization. Although the function of many hematopoietic cells requires WASp, the specific expression and function of this molecule in natural killer (NK) cells is unknown. Here, we report that WAS patients have increased percentages of peripheral blood NK cells and that fresh enriched NK cells from two patients with a WASp mutation have defective cytolytic function. In normal NK cells, WASp was expressed and localized to the activating immunologic synapse (IS) with filamentous actin (F-actin). Perforin also localized to the NK cell-activating IS but at a lesser frequency than F-actin and WASp. The accumulation of F-actin and WASp at the activating IS was decreased significantly in NK cells that had been treated with the inhibitor of actin polymerization, cytochalasin D. NK cells from WAS patients lacked expression of WASp and accumulated F-actin at the activating IS infrequently. Thus, WASp has an important function in NK cells. In patients with WASp mutations, the resulting NK cell defects are likely to contribute to their disease.

  9. Meeting the International Health Regulations (2005) surveillance core capacity requirements at the subnational level in Europe: the added value of syndromic surveillance

    OpenAIRE

    Ziemann, Alexandra; Rosenk?tter, Nicole; Riesgo, Luis Garcia-Castrillo; Fischer, Matthias; Kr?mer, Alexander; Lippert, Freddy K; Vergeiner, Gernot; Brand, Helmut; Krafft, Thomas

    2015-01-01

    BACKGROUND: The revised World Health Organization's International Health Regulations (2005) request a timely and all-hazard approach towards surveillance, especially at the subnational level. We discuss three questions of syndromic surveillance application in the European context for assessing public health emergencies of international concern: (i) can syndromic surveillance support countries, especially the subnational level, to meet the International Health Regulations (2005) core surveilla...

  10. Intensidad del síndrome climatérico y su relación con algunos factores socioambientales Climateric syndrome intensity and its relation to some socio-environmental factors

    Directory of Open Access Journals (Sweden)

    Margeris Yanes Calderón

    2009-12-01

    con la satisfacción de sus necesidades básicas, tienen una mayor incidencia de síndrome climatérico leve o asintomático.Introduction: Expressions like …I am in the menopause, I am old, and the problem is that I am in the climateric or it is the age change… are very common in the multidisciplinary consultation of climateric and menopause of "Ana Betancourt" University Polyclinic of Playa municipality. It was the reason to make this research, avoiding the biomedical approach with which the climateric period has been approached until nowadays moving forward to a medical-social approach. Objective: To determine how the socioenvironmental factors influence on the climateric syndrome intensity. Methods: A cross-sectional and descriptive study was conducted in 192 climateric women seen in three medical consulting rooms from "Ana Betancourt" University Polyclinic of the Playa municipality from Ciudad de La Habana from June, 2006 to May, 2007. Results: Slight climateric syndrome was present in the 42,28% of study women, and those with a high schooling level have a major incidence of the asymptomatic climateric syndrome or slight; there was a close relation among the women presenting with a moderate climateric syndrome and the dysfunctional familiar performance; the workers women have a trend to present a less intensive climateric syndrome stayed by more than 33% of them, thus demonstrating that more than 30% women with a proper satisfaction of its basic needs and a good resident hygienic environment, have the greater incidence of the asymptomatic climateric syndrome or slight closely related to sociocultural and economic features of study health areas. Conclusions: Women with a higher schooling level, with conjugal stability and working link being part also of functional or moderately functional families and with a good resident hygienic environment in correspondence with satisfaction of their basic needs, have a higher incidence of slight climateric syndrome or

  11. Musculocontractural Ehlers-Danlos syndrome and neurocristopathies: dermatan sulfate is required for Xenopus neural crest cells to migrate and adhere to fibronectin.

    Science.gov (United States)

    Gouignard, Nadège; Maccarana, Marco; Strate, Ina; von Stedingk, Kristoffer; Malmström, Anders; Pera, Edgar M

    2016-06-01

    Of all live births with congenital anomalies, approximately one-third exhibit deformities of the head and face. Most craniofacial disorders are associated with defects in a migratory stem and progenitor cell population, which is designated the neural crest (NC). Musculocontractural Ehlers-Danlos syndrome (MCEDS) is a heritable connective tissue disorder with distinct craniofacial features; this syndrome comprises multiple congenital malformations that are caused by dysfunction of dermatan sulfate (DS) biosynthetic enzymes, including DS epimerase-1 (DS-epi1; also known as DSE). Studies in mice have extended our understanding of DS-epi1 in connective tissue maintenance; however, its role in fetal development is not understood. We demonstrate that DS-epi1 is important for the generation of isolated iduronic acid residues in chondroitin sulfate (CS)/DS proteoglycans in early Xenopus embryos. The knockdown of DS-epi1 does not affect the formation of early NC progenitors; however, it impairs the correct activation of transcription factors involved in the epithelial-mesenchymal transition (EMT) and reduces the extent of NC cell migration, which leads to a decrease in NC-derived craniofacial skeleton, melanocytes and dorsal fin structures. Transplantation experiments demonstrate a tissue-autonomous role for DS-epi1 in cranial NC cell migration in vivo Cranial NC explant and single-cell cultures indicate a requirement of DS-epi1 in cell adhesion, spreading and extension of polarized cell processes on fibronectin. Thus, our work indicates a functional link between DS and NC cell migration. We conclude that NC defects in the EMT and cell migration might account for the craniofacial anomalies and other congenital malformations in MCEDS, which might facilitate the diagnosis and development of therapies for this distressing condition. Moreover, the presented correlations between human DS-epi1 expression and gene sets of mesenchymal character, invasion and metastasis in

  12. Sound intensity

    DEFF Research Database (Denmark)

    Crocker, Malcolm J.; Jacobsen, Finn

    1998-01-01

    This chapter is an overview, intended for readers with no special knowledge about this particular topic. The chapter deals with all aspects of sound intensity and its measurement from the fundamental theoretical background to practical applications of the measurement technique.......This chapter is an overview, intended for readers with no special knowledge about this particular topic. The chapter deals with all aspects of sound intensity and its measurement from the fundamental theoretical background to practical applications of the measurement technique....

  13. Sound Intensity

    DEFF Research Database (Denmark)

    Crocker, M.J.; Jacobsen, Finn

    1997-01-01

    This chapter is an overview, intended for readers with no special knowledge about this particular topic. The chapter deals with all aspects of sound intensity and its measurement from the fundamental theoretical background to practical applications of the measurement technique.......This chapter is an overview, intended for readers with no special knowledge about this particular topic. The chapter deals with all aspects of sound intensity and its measurement from the fundamental theoretical background to practical applications of the measurement technique....

  14. Efficacy of Various Scoring Systems for Predicting the 28-Day Survival Rate among Patients with Acute Exacerbation of Chronic Obstructive Pulmonary Disease Requiring Emergency Intensive Care

    Directory of Open Access Journals (Sweden)

    Zhihong Feng

    2017-01-01

    Full Text Available We aimed to investigate the efficacy of four severity-of-disease scoring systems in predicting the 28-day survival rate among patients with acute exacerbation of chronic obstructive pulmonary disease (AECOPD requiring emergency care. Clinical data of patients with AECOPD who required emergency care were recorded over 2 years. APACHE II, SAPS II, SOFA, and MEDS scores were calculated from severity-of-disease indicators recorded at admission and compared between patients who died within 28 days of admission (death group; 46 patients and those who did not (survival group; 336 patients. Compared to the survival group, the death group had a significantly higher GCS score, frequency of comorbidities including hypertension and heart failure, and age (P<0.05 for all. With all four systems, scores of age, gender, renal inadequacy, hypertension, coronary heart disease, heart failure, arrhythmia, anemia, fracture leading to bedridden status, tumor, and the GCS were significantly higher in the death group than the survival group. The prediction efficacy of the APACHE II and SAPS II scores was 88.4%. The survival rates did not differ significantly between APACHE II and SAPS II (P=1.519. Our results may guide triage for early identification of critically ill patients with AECOPD in the emergency department.

  15. [Serotonin syndrome and pain medication : What is relevant for practice?].

    Science.gov (United States)

    Schenk, M; Wirz, S

    2015-04-01

    Serotonin syndrome is a dangerous and rare complication of a pharmacotherapy and can lead to death. Caused by unwanted interactions of serotonergic drugs, it is characterised by a neuroexcitatory triad of mental changes, neuromuscular hyperactivity and autonomic instability. Opioids with serotonergic effects include the phenylpiperidine series opioids fentanyl, methadone, meperidine and tramadol and the morphine analogues oxycodone and codeine. In combination with certain serotonergic drugs, e.g. antidepressants, they can provoke serotonin syndrome. In patients with such combinations, special attention should be paid to clinical signs of serotonergic hyperactivity. Higher risk combinations (e.g. monoamine oxidase inhibitors with tramadol) must be avoided. Treatment with serotonergic agents must be stopped in moderate or severe serotonin syndrome. Patients with a severe serotonin syndrome require symptomatic intensive care and specifically a pharmacological antagonism with cyproheptadine or chlorpromazine.

  16. The beneficial effect of chronic graft-versus-host disease on the clinical outcome of transplantation with fludarabine/busulfan-based reduced-intensity conditioning for patients with de novo myelodysplastic syndrome.

    Science.gov (United States)

    Cho, Byung-Sik; Kim, Yoo-Jin; Cho, Seok-Goo; Kim, Sung-Yong; Eom, Ki-Seong; Kim, Hee-Je; Lee, Seok; Min, Chang-Ki; Kim, Dong-Wook; Lee, Jong-Wook; Min, Woo-Sung; Kim, Chun-Choo

    2007-06-01

    Allogeneic hematopoietic stem cell transplantation following reduced-intensity stem cell transplantation (RIST) has enabled the treatment of older or medically infirm patients with myeloid malignancies; however, determining the value of RIST outcomes for myelodysplastic syndrome (MDS) is difficult because of the heterogeneity of the diseases included in most trials. To define the role of RIST in MDS, we performed RIST for 22 consecutive patients who had de novo MDS as classified by World Health Organization (WHO) criteria and who received an allograft with fludarabine/busulfan (Busulfex) or fludarabine/Busulfex/antithymocyte globulin (ATG) conditioning. Nineteen patients (86.4%) achieved engraftment. At a median follow-up of 18.9 months (range, 13.1-24.8 months), the estimated 2-year rates of overall survival, event-free survival (EFS), transplantation-related mortality, and relapse were 78.7%, 67.7%, 12.6%, and 22.5%, respectively. Acute graft-versus-host disease (GVHD) greater than grade II developed in 3 patients (15.8%). Chronic GVHD developed in 10 patients (55.6%), none of whom received ATG as a conditioning regimen. Variables influencing EFS were chronic GVHD, marrow blasts before transplantation, and the WHO criteria. The present study clarifies the benefits of the fludarabine/Busulfex-based conditioning regimen for de novo MDS diagnosed according to the WHO criteria and shows that chronic GVHD appears to have a beneficial effect on survival rates, which are strongly associated with graft-versus-tumor effects.

  17. GnRH agonist trigger with intensive luteal phase support vs. human chorionic gonadotropin trigger in high responders: an observational study reporting pregnancy outcomes and incidence of ovarian hyperstimulation syndrome.

    Science.gov (United States)

    Christopoulos, Georgios; Vlismas, Antonios; Carby, Anna; Lavery, Stuart; Trew, Geoffrey

    2016-09-01

    A retrospective, cohort study of high-risk patients undergoing IVF treatment was performed to assess if there is a difference in clinical pregnancy rate, live birth rate and the incidence of ovarian hyperstimulation syndrome, when a GnRH agonist (GnRHa) trigger with intensive luteal support is compared to human chorionic gonadotropin (hCG) with standard luteal support. The control group consisted of 382 high-risk patients having a GnRH antagonist protocol with 194 receiving an hCG trigger. All patients had ≥18 follicles ≥11mm or serum oestradiol >18,000pmol/l on the day of trigger. Patients had a single or double embryo transfer at cleavage or blastocyst stage. Logistic regression was used to adjust for differences between the groups. An intention-to-treat analysis of all cycles was performed. No statistically significant differences were observed in terms of positive pregnancy test, clinical pregnancy rate and live birth rate. Only one patient (0.3%) was hospitalized with severe OHSS in the GnRHa group, compared to 26 patients (13%) in the hCG group. In conclusion, GnRHa trigger is associated with similar pregnancy rates with hCG trigger and a significant reduction in hospitalization for severe OHSS after an intention to treat analysis was performed.

  18. The effects of adjuvant immunoglobulin M-enriched immunoglobulin therapy on mortality rate and renal function in sepsis-induced multiple organ dysfunction syndrome: retrospective analysis of intensive care unit patients.

    Science.gov (United States)

    Yavuz, L; Aynali, G; Aynali, A; Alaca, A; Kutuk, S; Ceylan, B G

    2012-01-01

    To determine the effect of immunoglobulin (Ig)M-enriched Ig therapy on mortality rate and renal function in sepsis-induced multiple organ dysfunction syndrome (MODS), using the Acute Physiology and Chronic Health Evaluation II (APACHE II) score. Retrospective study of patients with sepsis-induced MODS treated with standard antibiotic plus supportive therapy (control group) or IgM-enriched Ig therapy adjuvant to control group therapy (IVIg group). Total length of stay in the intensive care unit (ICU), overall mortality rate and 28-day case fatality rate (CFR), as well as APACHE II scores and renal function parameters at day 1 and day 4 of therapy, were recorded. A total of 118 patients were included (control group, n = 62; IVIg group, n = 56). In both groups, day 4 APACHE II scores decreased significantly compared with day 1 scores; the effect of treatment on renal function was minimal. Length of ICU stay, overall mortality rate and 28-day CFR were significantly lower in the IVIg group compared with the control group. Adding IgM-enriched Ig therapy to standard therapy for MODS improved general clinical conditions and significantly reduced APACHE II scores, overall mortality rate and 28-day CFR, although effects on renal function were minimal.

  19. Chronic fatigue syndrome: aetiology, diagnosis and treatment

    Directory of Open Access Journals (Sweden)

    Palacín Delgado Cecilia

    2009-10-01

    Full Text Available Abstract Chronic fatigue syndrome is characterised by intense fatigue, with duration of over six months and associated to other related symptoms. The latter include asthenia and easily induced tiredness that is not recovered after a night's sleep. The fatigue becomes so severe that it forces a 50% reduction in daily activities. Given its unknown aetiology, different hypotheses have been considered to explain the origin of the condition (from immunological disorders to the presence of post-traumatic oxidative stress, although there are no conclusive diagnostic tests. Diagnosis is established through the exclusion of other diseases causing fatigue. This syndrome is rare in childhood and adolescence, although the fatigue symptom per se is quite common in paediatric patients. Currently, no curative treatment exists for patients with chronic fatigue syndrome. The therapeutic approach to this syndrome requires a combination of different therapeutic modalities. The specific characteristics of the symptomatology of patients with chronic fatigue require a rapid adaptation of the educational, healthcare and social systems to prevent the problems derived from current systems. Such patients require multidisciplinary management due to the multiple and different issues affecting them. This document was realized by one of the Interdisciplinary Work Groups from the Institute for Rare Diseases, and its aim is to point out the main social and care needs for people affected with Chronic Fatigue Syndrome. For this, it includes not only the view of representatives for different scientific societies, but also the patient associations view, because they know the true history of their social and sanitary needs. In an interdisciplinary approach, this work also reviews the principal scientific, medical, socio-sanitary and psychological aspects of Chronic Fatigue Syndrome.

  20. Fanconi syndrome

    Science.gov (United States)

    De Toni-Fanconi syndrome ... Fanconi syndrome can be caused by faulty genes, or it may result later in life due to kidney damage. Sometimes the cause of Fanconi syndrome is unknown. Common causes of Fanconi syndrome in ...

  1. Duane Syndrome

    Science.gov (United States)

    ... Frequently Asked Questions Español Condiciones Chinese Conditions Duane Syndrome En Español Read in Chinese What is Duane Syndrome? Duane syndrome, also called Duane retraction syndrome (DRS), ...

  2. Hunter Syndrome

    Science.gov (United States)

    ... in girls. There's no cure for Hunter syndrome. Treatment of Hunter syndrome involves management of symptoms and complications. Symptoms Hunter syndrome is one type of a group of inherited metabolic disorders called mucopolysaccharidoses (MPSs), and Hunter syndrome is ...

  3. Nuclear magnetic resonance therapy in lumbar disc herniation with lumbar radicular syndrome: effects of the intervention on pain intensity, health-related quality of life, disease-related disability, consumption of pain medication, duration of sick leave and MRI analysis.

    Science.gov (United States)

    Salfinger, H; Salomonowitz, G; Friedrich, K M; Hahne, J; Holzapfel, J; Friedrich, M

    2015-06-01

    The objective was to assess the effects of therapeutic nuclear magnetic resonance (tNMR) as a conservative treatment for lumbar radicular syndrome (LRS) in patients with lumbar disc herniation. The prospective, randomised, double-blind, placebo-controlled trial included 94 patients, aged 20-60 years (44.79 ± 8.83), with LRS caused by lumbar disc herniation confirmed by MRI scans and with clinical signs of a radicular lesion without indication for surgical intervention. Treatment group (TG) and control group (CG) received standard non-surgical therapy. Additionally, the TG had seven sessions with the tNMR device with a magnetic flux density of 2.3 mT and a frequency of 85 kHz; the CG received 7 sham treatments. Outcome parameters were the treatment effect on pain intensity (Visual Analogue Scale-VAS), health-related quality of life (36-item Short Form Health Survey-SF-36), disease-related disability (Roland Morris Disability Questionnaire-RMDQ), pain medication intake, duration of sick leave and morphological changes assessed by MRI scan analysis. VAS scores improved significantly in both groups (p Patients in the TG recorded significantly fewer days of sick leave in month 3 after treatment (p = 0.026). MRI scan summary scores improved significantly in both groups (L4/5 p treatment of lumbar disc herniation with LRS. The application of tNMR did not meet MCID criteria. It rendered few statistically significant differences between patient groups. The overall results of this trial make a clinical implementation of tNMR in the treatment of lumbar disc herniation with LRS appear premature. Further research is needed to better understand the mode of action of tNMR on compressed neural tissue and to elucidate the issue of the cost/benefit ratio.

  4. Hamartomatous polyposis syndromes

    DEFF Research Database (Denmark)

    Jelsig, Anne Marie; Qvist, Niels; Brusgaard, Klaus

    2014-01-01

    Hamartomatous Polyposis Syndromes (HPS) are genetic syndromes, which include Peutz-Jeghers syndrome, Juvenile polyposis syndrome, PTEN hamartoma tumour syndrome (Cowden Syndrom, Bannayan-Riley-Ruvalcaba and Proteus Syndrome) as well as hereditary mixed polyposis syndrome. Other syndromes such as ......Hamartomatous Polyposis Syndromes (HPS) are genetic syndromes, which include Peutz-Jeghers syndrome, Juvenile polyposis syndrome, PTEN hamartoma tumour syndrome (Cowden Syndrom, Bannayan-Riley-Ruvalcaba and Proteus Syndrome) as well as hereditary mixed polyposis syndrome. Other syndromes...

  5. Correlation between workplace and occupational burnout syndrome in nurses

    OpenAIRE

    Ahmadi, Omid; Azizkhani, Reza; Basravi, Monem

    2014-01-01

    Background: This study was conducted to determine the effect of nurses′ workplace on burnout syndrome among nurses working in Isfahan′s Alzahra Hospital as a reference and typical university affiliated hospital, in 2010. Materials and Methods: In this cross-sectional study, 100 nurses were randomly selected among those working in emergency, orthopedic, dialysis wards and intensive care unit (ICU). Required data on determination of occupational burnout rate among the nurses of these wards ...

  6. Capgras syndrome in adolescence.

    Science.gov (United States)

    Jackson, R S; Naylor, M W; Shain, B N; King, C A

    1992-09-01

    Capgras syndrome, the delusion of substitution, has rarely been reported in adolescents. The etiology is unknown, and intense controversy surrounds the debate over the relative importance of biological versus psychological factors. Presented here are two cases of Capgras syndrome in adolescents and a review of the relevant biological, neuropsychological, and psychodynamic literature. The authors suggest that the psychological processes underlying the Capgras delusion are mediated by neuroanatomical connections between various brain areas and hypothesize that the fundamental lesion in Capgras syndrome may be the patient's inability or failure to acknowledge the authenticity of a person they clearly recognize.

  7. Acute heart failure syndrome

    African Journals Online (AJOL)

    Heart failure can be defined as a clinical syndrome in which a structural or functional cardiac abnormality impairs the capacity of the ventricle to fill or eject enough blood for the requirements of the body. Acute heart failure syndrome represents a complex, heterogeneous set of clinical conditions, all with the common.

  8. Ehlers-Danlos syndrome type IV

    Science.gov (United States)

    Germain, Dominique P

    2007-01-01

    Ehlers-Danlos syndrome type IV, the vascular type of Ehlers-Danlos syndromes (EDS), is an inherited connective tissue disorder defined by characteristic facial features (acrogeria) in most patients, translucent skin with highly visible subcutaneous vessels on the trunk and lower back, easy bruising, and severe arterial, digestive and uterine complications, which are rarely, if at all, observed in the other forms of EDS. The estimated prevalence for all EDS varies between 1/10,000 and 1/25,000, EDS type IV representing approximately 5 to 10% of cases. The vascular complications may affect all anatomical areas, with a tendency toward arteries of large and medium diameter. Dissections of the vertebral arteries and the carotids in their extra- and intra-cranial segments (carotid-cavernous fistulae) are typical. There is a high risk of recurrent colonic perforations. Pregnancy increases the likelihood of a uterine or vascular rupture. EDS type IV is inherited as an autosomal dominant trait that is caused by mutations in the COL3A1 gene coding for type III procollagen. Diagnosis is based on clinical signs, non-invasive imaging, and the identification of a mutation of the COL3A1 gene. In childhood, coagulation disorders and Silverman's syndrome are the main differential diagnoses; in adulthood, the differential diagnosis includes other Ehlers-Danlos syndromes, Marfan syndrome and Loeys-Dietz syndrome. Prenatal diagnosis can be considered in families where the mutation is known. Choriocentesis or amniocentesis, however, may entail risk for the pregnant woman. In the absence of specific treatment for EDS type IV, medical intervention should be focused on symptomatic treatment and prophylactic measures. Arterial, digestive or uterine complications require immediate hospitalisation, observation in an intensive care unit. Invasive imaging techniques are contraindicated. Conservative approach is usually recommended when caring for a vascular complication in a patient suffering

  9. Ehlers-Danlos syndrome type IV

    Directory of Open Access Journals (Sweden)

    Germain Dominique P

    2007-07-01

    Full Text Available Abstract Ehlers-Danlos syndrome type IV, the vascular type of Ehlers-Danlos syndromes (EDS, is an inherited connective tissue disorder defined by characteristic facial features (acrogeria in most patients, translucent skin with highly visible subcutaneous vessels on the trunk and lower back, easy bruising, and severe arterial, digestive and uterine complications, which are rarely, if at all, observed in the other forms of EDS. The estimated prevalence for all EDS varies between 1/10,000 and 1/25,000, EDS type IV representing approximately 5 to 10% of cases. The vascular complications may affect all anatomical areas, with a tendency toward arteries of large and medium diameter. Dissections of the vertebral arteries and the carotids in their extra- and intra-cranial segments (carotid-cavernous fistulae are typical. There is a high risk of recurrent colonic perforations. Pregnancy increases the likelihood of a uterine or vascular rupture. EDS type IV is inherited as an autosomal dominant trait that is caused by mutations in the COL3A1 gene coding for type III procollagen. Diagnosis is based on clinical signs, non-invasive imaging, and the identification of a mutation of the COL3A1 gene. In childhood, coagulation disorders and Silverman's syndrome are the main differential diagnoses; in adulthood, the differential diagnosis includes other Ehlers-Danlos syndromes, Marfan syndrome and Loeys-Dietz syndrome. Prenatal diagnosis can be considered in families where the mutation is known. Choriocentesis or amniocentesis, however, may entail risk for the pregnant woman. In the absence of specific treatment for EDS type IV, medical intervention should be focused on symptomatic treatment and prophylactic measures. Arterial, digestive or uterine complications require immediate hospitalisation, observation in an intensive care unit. Invasive imaging techniques are contraindicated. Conservative approach is usually recommended when caring for a vascular

  10. Polyposis syndromes: pediatric implications.

    Science.gov (United States)

    Hyer, W

    2001-10-01

    The diagnosis of a polyposis syndrome, such as juvenile polyposis, Peutz-Jeghers syndrome, and familial adenomatous polyposis, requires knowledge of the site, number, and histologic type of the polyps and an appreciation of relevant family history. Children and adolescents with polyposis syndromes are faced with not only the immediate complications of the polyps, such as intussusception or bleeding, but also the extraintestinal manifestations and the long-term risk for malignancy. This article reviews the diagnosis, clinical management, surveillance, and surgical options for children with polyposis syndromes and discusses genetics and appropriate screening programs.

  11. Loss-of-Function KCNE2 Variants: True Monogenic Culprits of Long-QT Syndrome or Proarrhythmic Variants Requiring Secondary Provocation?

    NARCIS (Netherlands)

    Roberts, Jason D.; Krahn, Andrew D.; Ackerman, Michael J.; Rohatgi, Ram K.; Moss, Arthur J.; Nazer, Babak; Tadros, Rafik; Gerull, Brenda; Sanatani, Shubhayan; Wijeyeratne, Yanushi D.; Baruteau, Alban-Elouen; Muir, Alison R.; Pang, Benjamin; Cadrin-Tourigny, Julia; Talajic, Mario; Rivard, Lena; Tester, David J.; Liu, Taylor; Whitman, Isaac R.; Wojciak, Julianne; Conacher, Susan; Gula, Lorne J.; Leong-Sit, Peter; Manlucu, Jaimie; Green, Martin S.; Hamilton, Robert; Healey, Jeff S.; Lopes, Coeli M.; Behr, Elijah R.; Wilde, Arthur A.; Gollob, Michael H.; Scheinman, Melvin M.

    2017-01-01

    Insight into type 6 long-QT syndrome (LQT6), stemming from mutations in the KCNE2-encoded voltage-gated channel β-subunit, is limited. We sought to further characterize its clinical phenotype. Individuals with reported pathogenic KCNE2 mutations identified during arrhythmia evaluation were collected

  12. Prone position in patients with acute respiratory distress syndrome

    Science.gov (United States)

    Setten, Mariano; Plotnikow, Gustavo Adrián; Accoce, Matías

    2016-01-01

    Acute respiratory distress syndrome occupies a great deal of attention in intensive care units. Despite ample knowledge of the physiopathology of this syndrome, the focus in intensive care units consists mostly of life-supporting treatment and avoidance of the side effects of invasive treatments. Although great advances in mechanical ventilation have occurred in the past 20 years, with a significant impact on mortality, the incidence continues to be high. Patients with acute respiratory distress syndrome, especially the most severe cases, often present with refractory hypoxemia due to shunt, which can require additional treatments beyond mechanical ventilation, among which is mechanical ventilation in the prone position. This method, first recommended to improve oxygenation in 1974, can be easily implemented in any intensive care unit with trained personnel. Prone position has extremely robust bibliographic support. Various randomized clinical studies have demonstrated the effect of prone decubitus on the oxygenation of patients with acute respiratory distress syndrome measured in terms of the PaO2/FiO2 ratio, including its effects on increasing patient survival. The members of the Respiratory Therapists Committee of the Sociedad Argentina de Terapia Intensiva performed a narrative review with the objective of discovering the available evidence related to the implementation of prone position, changes produced in the respiratory system due to the application of this maneuver, and its impact on mortality. Finally, guidelines are suggested for decision-making. PMID:27925054

  13. Prone position in patients with acute respiratory distress syndrome.

    Science.gov (United States)

    Setten, Mariano; Plotnikow, Gustavo Adrián; Accoce, Matías

    2016-01-01

    Acute respiratory distress syndrome occupies a great deal of attention in intensive care units. Despite ample knowledge of the physiopathology of this syndrome, the focus in intensive care units consists mostly of life-supporting treatment and avoidance of the side effects of invasive treatments. Although great advances in mechanical ventilation have occurred in the past 20 years, with a significant impact on mortality, the incidence continues to be high. Patients with acute respiratory distress syndrome, especially the most severe cases, often present with refractory hypoxemia due to shunt, which can require additional treatments beyond mechanical ventilation, among which is mechanical ventilation in the prone position. This method, first recommended to improve oxygenation in 1974, can be easily implemented in any intensive care unit with trained personnel. Prone position has extremely robust bibliographic support. Various randomized clinical studies have demonstrated the effect of prone decubitus on the oxygenation of patients with acute respiratory distress syndrome measured in terms of the PaO2/FiO2 ratio, including its effects on increasing patient survival. The members of the Respiratory Therapists Committee of the Sociedad Argentina de Terapia Intensiva performed a narrative review with the objective of discovering the available evidence related to the implementation of prone position, changes produced in the respiratory system due to the application of this maneuver, and its impact on mortality. Finally, guidelines are suggested for decision-making.

  14. Reflex test reminders in required cancer synoptic templates decrease order entry error: An analysis of mismatch repair immunohistochemical orders to screen for Lynch syndrome

    Directory of Open Access Journals (Sweden)

    Mark R Kilgore

    2016-01-01

    Full Text Available Background: Endometrial carcinoma (EC is the most common extracolonic malignant neoplasm associated with Lynch syndrome (LS. LS is caused by autosomal dominant germline mutations in DNA mismatch repair (MMR genes. Screening for LS in EC is often evaluated by loss of immunohistochemical (IHC expression of DNA MMR enzymes MLH1, MSH2, MSH6, and PMS2 (MMR IHC. In July 2013, our clinicians asked that we screen all EC in patients ≤60 for loss of MMR IHC expression. Despite this policy, several cases were not screened or screening was delayed. We implemented an informatics-based approach to ensure that all women who met criteria would have timely screening. Subjects and Methods: Reports are created in PowerPath (Sunquest Information Systems, Tucson, AZ with custom synoptic templates. We implemented an algorithm on March 6, 2014 requiring pathologists to address MMR IHC in patients ≤60 with EC before sign out (S/O. Pathologists must answer these questions: is patient ≤60 (yes/no, if yes, follow-up questions (IHC done previously, ordered with addendum to follow, results included in report, N/A, or not ordered, if not ordered, one must explain. We analyzed cases from July 18, 2013 to August 31, 2016 preimplementation (PreImp and postimplementation (PostImp that met criteria. Data analysis was performed using the standard data package included with GraphPad Prism® 7.00 (GraphPad Software, Inc., La Jolla, CA, USA. Results: There were 147 patients who met criteria (29 PreImp and 118 PostImp. IHC was ordered in a more complete and timely fashion PostImp than PreImp. PreImp, 4/29 (13.8% cases did not get any IHC, but PostImp, only 4/118 (3.39% were missed (P = 0.0448. Of cases with IHC ordered, 60.0% (15/25 were ordered before or at S/O PreImp versus 91.2% (104/114 PostImp (P = 0.0004. Relative to day of S/O, the mean days of order delay were longer and more variable PreImp versus PostImp (12.9 ± 40.7 vs. -0.660 ± 1.15; P = 0.0227, with the average

  15. Metabolic syndrome increases dietary α-tocopherol requirements as assessed using urinary and plasma vitamin E catabolites: a double-blind, crossover clinical trial.

    Science.gov (United States)

    Traber, Maret G; Mah, Eunice; Leonard, Scott W; Bobe, Gerd; Bruno, Richard S

    2017-03-01

    Background: Vitamin E supplementation improves liver histology in patients with nonalcoholic steatohepatitis, which is a manifestation of the metabolic syndrome (MetS). We reported previously that α-tocopherol bioavailability in healthy adults is higher than in those with MetS, thereby suggesting that the latter group has increased requirements. Objective: We hypothesized that α-tocopherol catabolites α-carboxyethyl hydroxychromanol (α-CEHC) and α-carboxymethylbutyl hydroxychromanol (α-CMBHC) are useful biomarkers of α-tocopherol status. Design: Adults (healthy or with MetS; n = 10/group) completed a double-blind, crossover clinical trial with four 72-h interventions during which they co-ingested 15 mg hexadeuterium-labeled RRR -α-tocopherol (d 6 -α-T) with nonfat, reduced-fat, whole, or soy milk. During each intervention, we measured α-CEHC and α-CMBHC excretions in three 8-h urine collections (0-24 h) and plasma α-tocopherol, α-CEHC, and α-CMBHC concentrations at various times ≤72 h. Results: During the first 24 h, participants with MetS compared with healthy adults excreted 41% less α-CEHC (all values are least-squares means ± SEMs: 0.6 ± 0.1 compared with 1.0 ± 0.1 μmol/g creatinine, respectively; P = 0.002), 63% less hexadeuterium-labeled (d 6 )-α-CEHC (0.04 ± 0.02 compared with 0.13 ± 0.02 μmol/g creatinine, respectively; P = 0.002), and 58% less d 6 -α-CMBHC (0.017 ± 0.004 compared with 0.041 ± 0.004 μmol/g creatinine, respectively; P = 0.0009) and had 52% lower plasma d 6 -α-CEHC areas under the concentration curves [area under the curve from 0 to 24 h (AUC 0-24h ): 27.7 ± 7.9 compared with 58.4 ± 7.9 nmol/L × h, respectively; P = 0.01]. d 6 -α-CEHC peaked before d 6 -α-T in 77 of 80 paired plasma concentration curves. Urinary d 6 -α-CEHC 24-h concentrations were associated with the plasma AUC 0-24 h of d 6 -α-T ( r = 0.53, P = 0.02) and d 6 -α-CEHC ( r = 0.72, P = 0.0003), and with urinary d 6 -α-CMBHC ( r = 0.88, P

  16. Metabolic syndrome increases dietary α-tocopherol requirements as assessed using urinary and plasma vitamin E catabolites: a double-blind, crossover clinical trial123

    Science.gov (United States)

    Mah, Eunice; Leonard, Scott W; Bobe, Gerd; Bruno, Richard S

    2017-01-01

    Background: Vitamin E supplementation improves liver histology in patients with nonalcoholic steatohepatitis, which is a manifestation of the metabolic syndrome (MetS). We reported previously that α-tocopherol bioavailability in healthy adults is higher than in those with MetS, thereby suggesting that the latter group has increased requirements. Objective: We hypothesized that α-tocopherol catabolites α-carboxyethyl hydroxychromanol (α-CEHC) and α-carboxymethylbutyl hydroxychromanol (α-CMBHC) are useful biomarkers of α-tocopherol status. Design: Adults (healthy or with MetS; n = 10/group) completed a double-blind, crossover clinical trial with four 72-h interventions during which they co-ingested 15 mg hexadeuterium-labeled RRR-α-tocopherol (d6-α-T) with nonfat, reduced-fat, whole, or soy milk. During each intervention, we measured α-CEHC and α-CMBHC excretions in three 8-h urine collections (0–24 h) and plasma α-tocopherol, α-CEHC, and α-CMBHC concentrations at various times ≤72 h. Results: During the first 24 h, participants with MetS compared with healthy adults excreted 41% less α-CEHC (all values are least-squares means ± SEMs: 0.6 ± 0.1 compared with 1.0 ± 0.1 μmol/g creatinine, respectively; P = 0.002), 63% less hexadeuterium-labeled (d6)-α-CEHC (0.04 ± 0.02 compared with 0.13 ± 0.02 μmol/g creatinine, respectively; P = 0.002), and 58% less d6-α-CMBHC (0.017 ± 0.004 compared with 0.041 ± 0.004 μmol/g creatinine, respectively; P = 0.0009) and had 52% lower plasma d6-α-CEHC areas under the concentration curves [area under the curve from 0 to 24 h (AUC0–24h): 27.7 ± 7.9 compared with 58.4 ± 7.9 nmol/L × h, respectively; P = 0.01]. d6-α-CEHC peaked before d6-α-T in 77 of 80 paired plasma concentration curves. Urinary d6-α-CEHC 24-h concentrations were associated with the plasma AUC0–24 h of d6-α-T (r = 0.53, P = 0.02) and d6-α-CEHC (r = 0.72, P = 0.0003), and with urinary d6-α-CMBHC (r = 0.88, P < 0.0001), and

  17. Coagulation disorders in intensive care

    NARCIS (Netherlands)

    Levi, Marcel

    2009-01-01

    Coagulation disorders are common in intensive care patients and may range from isolated thrombocytopenia or prolonged clotting times to disseminated intravascular coagulation. There are many causes of disturbed coagulation in critically ill patients and each may require specific treatment

  18. Marfan Syndrome

    Science.gov (United States)

    Marfan syndrome is a disorder that affects connective tissue. Connective tissues are proteins that support skin, bones, blood ... fibrillin. A problem with the fibrillin gene causes Marfan syndrome. Marfan syndrome can be mild to severe, and ...

  19. WIEDEMANN SYNDROME

    African Journals Online (AJOL)

    hi-tech

    BILATERAL BENIGN HAEMORRHAGIC ADRENAL CYSTS IN BECKWITH - WIEDEMANN. SYNDROME: CASE REPORT. P. ANOOP and M. A. ANJAY. SUMMARY. Beckwith-Wiedemann syndrome is the most common overgrowth malformation syndrome. The classical features include macrosomia, macroglossia, ...

  20. Tourette syndrome

    Science.gov (United States)

    Gilles de la Tourette syndrome; Tic disorders - Tourette syndrome ... Tourette syndrome is named for Georges Gilles de la Tourette, who first described this disorder in 1885. The disorder is likely passed down through families. ...

  1. Williams syndrome

    Science.gov (United States)

    Williams-Beuren syndrome ... Williams syndrome is caused by not having a copy of several genes. It may be passed down in families. ... history of the condition. However, people with Williams syndrome have a 50% chance of passing the disorder ...

  2. Piriformis Syndrome

    Science.gov (United States)

    ... the National Library of Medicine’s MedlinePlus Piriformis Syndrome Metabolic Syndrome Show More Show Less Search Disorders SEARCH SEARCH Definition Treatment Prognosis Clinical Trials Organizations Publications Definition Piriformis syndrome ...

  3. Unusual Straatsma Syndrome - How dogmatic is a bad prognosis?

    Directory of Open Access Journals (Sweden)

    Ana Vide-Escada

    2017-12-01

    Conclusions and importance: Straatsma Syndrome can present with heterochromia iridum. When strabismus is present, early surgery should be withheld. Intensive treatment of Straatsma Syndrome can yield an unexpected good result, despite initial high degree anisometropia and low vision acuity.

  4. Major vault protein, a candidate gene in 16p11.2 microdeletion syndrome, is required for the homeostatic regulation of visual cortical plasticity.

    Science.gov (United States)

    Ip, Jacque P K; Nagakura, Ikue; Petravicz, Jeremy; Li, Keji; Wiemer, Erik A C; Sur, Mriganka

    2018-03-14

    Microdeletion of a region in chromosome 16p11.2 increases susceptibility to autism. Although this region contains exons of 29 genes, disrupting only a small segment of the region, which spans 5 genes, is sufficient to cause autistic traits. One candidate gene in this critical segment is MVP , which encodes for the major vault protein (MVP) that has been implicated in regulation of cellular transport mechanisms. MVP expression levels in MVP +/- mice closely phenocopy those of 16p11.2 mutant mice, suggesting that MVP +/- mice may serve as a model of MVP function in 16p11.2 microdeletion. Here we show that MVP regulates the homeostatic component of ocular dominance (OD) plasticity in primary visual cortex (V1). MVP +/- mice of both sexes show impairment in strengthening of open-eye responses after several days of monocular deprivation (MD) while closed-eye responses are weakened as normal, resulting in reduced overall OD plasticity. The frequency of mEPSCs in pyramidal neurons is decreased in MVP +/- mice after extended MD, suggesting a reduction of functional synapses. Correspondingly, upregulation of surface GluA1 AMPA receptors is reduced in MVP +/- mice after extended MD, and is accompanied by altered expression of STAT1 and phosphorylated ERK, which have been previously implicated in OD plasticity. Normalization of STAT1 levels by introducing STAT1 shRNA rescues surface GluA1 and open-eye responses, implicating STAT1 as a downstream effector of MVP. These findings demonstrate a specific role for MVP as a key molecule influencing the homeostatic component of activity-dependent synaptic plasticity, and potentially the corresponding phenotypes of 16p11.2 microdeletion syndrome. Significance Statement Major vault protein (MVP), a candidate gene in 16p11.2 microdeletion syndrome, has been implicated in the regulation of several cellular processes including transport mechanisms and scaffold signaling. However, its role in brain function and plasticity remains unknown

  5. Evidence based exercise - clinical benefits of high intensity interval training.

    Science.gov (United States)

    Shiraev, Tim; Barclay, Gabriella

    2012-12-01

    Aerobic exercise has a marked impact on cardiovascular disease risk. Benefits include improved serum lipid profiles, blood pressure and inflammatory markers as well as reduced risk of stroke, acute coronary syndrome and overall cardiovascular mortality. Most exercise programs prescribed for fat reduction involve continuous, moderate aerobic exercise, as per Australian Heart Foundation clinical guidelines. This article describes the benefits of exercise for patients with cardiovascular and metabolic disease and details the numerous benefits of high intensity interval training (HIIT) in particular. Aerobic exercise has numerous benefits for high-risk populations and such benefits, especially weight loss, are amplified with HIIT. High intensity interval training involves repeatedly exercising at a high intensity for 30 seconds to several minutes, separated by 1-5 minutes of recovery (either no or low intensity exercise). HIT is associated with increased patient compliance and improved cardiovascular and metabolic outcomes and is suitable for implementation in both healthy and 'at risk' populations. Importantly, as some types of exercise are contraindicated in certain patient populations and HIIT is a complex concept for those unfamiliar to exercise, some patients may require specific assessment or instruction before commencing a HIIT program.

  6. Intensive Exercise Training During Bed Rest Attenuates Deconditioning

    Science.gov (United States)

    Greenleaf, John E.

    1997-01-01

    Intensive exercise training during bed rest attenuates deconditioning. Med. Sci. Sports Exerc., Vol. 29, No. 2, pp. 207-215, 1997. A 30-d 6 deg head-down bed rest project was conducted to evaluate variable high-intensity, short-duration, isotonic cycle ergometer exercise (ITE) training and high-intensity intermittent resistive isokinetic exercise (IKE) training regimens designed to maintain peak VO2 and muscle mass, strength, and endurance at ambulatory control levels throughout prolonged bed rest. Other elements of the deconditioning (adaptive) syndrome, such as proprioception, psychological performance, hypovolemia, water balance, body composition, and orthostatic tolerance, were also measured. Major findings are summarized in this paper. Compared with response during bed rest of the no exercise (NOE) control group: the ITE training regimen (a) maintained work capacity (peak VO2), (b) maintained plasma and red cell volumes, (c) induced positive body water balance, (d) decreased quality of sleep and mental concentration, and (e) had no effect on the decrease in orthostatic tolerance; the IKE training regimen (f) attenuated the decrease in peak VO2 by 50%, (g) attenuated loss of red cell volume by 40% but had no effect on loss of plasma volume, (b) induced positive body water balance, (i) had no adverse effect on quality of sleep or concentration, and 0) had no effect on the decrease in orthostatic tolerance. These findings suggest that various elements of the deconditioning syndrome can be manipulated by duration and intensity of ITE or IKE training regimens and that several different training protocols will be required to maintain or restore physiological and psychological performance of individuals confined to prolonged bed rest.

  7. Measuring Physical Activity Intensity

    Medline Plus

    Full Text Available ... Compartir For more help with what counts as aerobic activity, watch this video: Windows Media Player, 4: ... ways to understand and measure the intensity of aerobic activity: relative intensity and absolute intensity. Relative Intensity ...

  8. Jacobsen syndrome

    Directory of Open Access Journals (Sweden)

    Grossfeld Paul

    2009-03-01

    Full Text Available Abstract Jacobsen syndrome is a MCA/MR contiguous gene syndrome caused by partial deletion of the long arm of chromosome 11. To date, over 200 cases have been reported. The prevalence has been estimated at 1/100,000 births, with a female/male ratio 2:1. The most common clinical features include pre- and postnatal physical growth retardation, psychomotor retardation, and characteristic facial dysmorphism (skull deformities, hypertelorism, ptosis, coloboma, downslanting palpebral fissures, epicanthal folds, broad nasal bridge, short nose, v-shaped mouth, small ears, low set posteriorly rotated ears. Abnormal platelet function, thrombocytopenia or pancytopenia are usually present at birth. Patients commonly have malformations of the heart, kidney, gastrointestinal tract, genitalia, central nervous system and skeleton. Ocular, hearing, immunological and hormonal problems may be also present. The deletion size ranges from ~7 to 20 Mb, with the proximal breakpoint within or telomeric to subband 11q23.3 and the deletion extending usually to the telomere. The deletion is de novo in 85% of reported cases, and in 15% of cases it results from an unbalanced segregation of a familial balanced translocation or from other chromosome rearrangements. In a minority of cases the breakpoint is at the FRA11B fragile site. Diagnosis is based on clinical findings (intellectual deficit, facial dysmorphic features and thrombocytopenia and confirmed by cytogenetics analysis. Differential diagnoses include Turner and Noonan syndromes, and acquired thrombocytopenia due to sepsis. Prenatal diagnosis of 11q deletion is possible by amniocentesis or chorionic villus sampling and cytogenetic analysis. Management is multi-disciplinary and requires evaluation by general pediatrician, pediatric cardiologist, neurologist, ophthalmologist. Auditory tests, blood tests, endocrine and immunological assessment and follow-up should be offered to all patients. Cardiac malformations can be

  9. Loss-of-Function KCNE2 Variants: True Monogenic Culprits of Long-QT Syndrome or Proarrhythmic Variants Requiring Secondary Provocation?

    Science.gov (United States)

    Roberts, Jason D; Krahn, Andrew D; Ackerman, Michael J; Rohatgi, Ram K; Moss, Arthur J; Nazer, Babak; Tadros, Rafik; Gerull, Brenda; Sanatani, Shubhayan; Wijeyeratne, Yanushi D; Baruteau, Alban-Elouen; Muir, Alison R; Pang, Benjamin; Cadrin-Tourigny, Julia; Talajic, Mario; Rivard, Lena; Tester, David J; Liu, Taylor; Whitman, Isaac R; Wojciak, Julianne; Conacher, Susan; Gula, Lorne J; Leong-Sit, Peter; Manlucu, Jaimie; Green, Martin S; Hamilton, Robert; Healey, Jeff S; Lopes, Coeli M; Behr, Elijah R; Wilde, Arthur A; Gollob, Michael H; Scheinman, Melvin M

    2017-08-01

    Insight into type 6 long-QT syndrome (LQT6), stemming from mutations in the KCNE2 -encoded voltage-gated channel β-subunit, is limited. We sought to further characterize its clinical phenotype. Individuals with reported pathogenic KCNE2 mutations identified during arrhythmia evaluation were collected from inherited arrhythmia clinics and the Rochester long-QT syndrome (LQTS) registry. Previously reported LQT6 cases were identified through a search of the MEDLINE database. Clinical features were assessed, while reported KCNE2 mutations were evaluated for genotype-phenotype segregation and classified according to the contemporary American College of Medical Genetics guidelines. Twenty-seven probands possessed reported pathogenic KCNE2 mutations, while a MEDLINE search identified 17 additional LQT6 cases providing clinical and genetic data. Sixteen probands had normal resting QTc values and only developed QT prolongation and malignant arrhythmias after exposure to QT-prolonging stressors, 10 had other LQTS pathogenic mutations, and 10 did not have an LQTS phenotype. Although the remaining 8 subjects had an LQTS phenotype, evidence suggested that the KCNE2 variant was not the underlying culprit. The collective frequency of KCNE2 variants implicated in LQT6 in the Exome Aggregation Consortium database was 1.4%, in comparison with a 0.0005% estimated clinical prevalence for LQT6. On the basis of clinical phenotype, the high allelic frequencies of LQT6 mutations in the Exome Aggregation Consortium database, and absence of previous documentation of genotype-phenotype segregation, our findings suggest that many KCNE2 variants, and perhaps all, have been erroneously designated as LQTS-causative mutations. Instead, KCNE2 variants may confer proarrhythmic susceptibility when provoked by additional environmental/acquired or genetic factors, or both. © 2017 American Heart Association, Inc.

  10. [Recommendations for analgesia and sedation in neonatal intensive care].

    Science.gov (United States)

    Rawicz, Marcin

    2008-01-01

    The purpose of the study was to present recommendations, relevant to the management of neonates and infants aged 0-1 years, treated in intensive care settings. They include general principles and recommendations for pain and sedation assessment, sedation and pain management and advice on the use of pharmacological strategies. The bolus (on demand) administration of sedative agents should be avoided because of increased risk of cardiovascular depression and/or neurological complications. Midazolam administration time should be limited to 72 hours because of tachyphylaxis, and the possibility of development of a withdrawal syndrome and neurological complications (grade A, LOE 1b). The level of sedation and pain should be regularly assessed and documented, using presented scales; the COMFORT scale is preferred. Opioids, given in continuous infusion, are the drugs of choice for neonatal sedation. To avoid withdrawal syndrome, the total doses and time of administration of sedative agents should be limited. Methadone is a drug of choice in the treatment of a withdrawal (Grade B, LOE 2). Intravenous ketamine is recommended, when short-term sedation/anaesthesia is required (Grade C, LOE 3) for painful and/or stressful intensive care procedures. (Grade C, LOE 2). Muscle relaxants should be used for endotracheal intubation and in the situations when mechanical ventilation is not possible due to maximal respiratory effort of the patient.

  11. Ectopic ACTH syndrome

    OpenAIRE

    Isidori, Andrea M.; Lenzi, Andrea

    2007-01-01

    Ectopic adrenocorticotropic secretion (EAS) is responsible for 12-17% of cases of Cushing's syndrome (CS) and covers a range of tumours, from undetectable benign lesions to widespread metastases. The syndrome is often associated with severe hypercortisolaemia, which aggravates the underlying condition. EAS requires a complete workup that includes the establishment of endogenous CS, diagnosis of adrenocorticotropic hormone (ACTH) dependency, localization of the source of ACTH secretion and rap...

  12. Pediatric Toxic Shock Syndrome

    OpenAIRE

    Yee, Jennifer; King, Andrew

    2017-01-01

    Audience: This scenario was developed to educate emergency medicine residents on the diagnosis and management of a pediatric patient with toxic shock syndrome. The case is also appropriate for teaching of medical students and advanced practice providers, as well as a review of the principles of crisis resource management, teamwork, and communication. Introduction: Toxic shock syndrome is a low-frequency, high-acuity scenario requiring timely identification and aggressive management....

  13. Asymptomatic giant coronary aneurysm in an adolescent with Behcet's syndrome

    Directory of Open Access Journals (Sweden)

    Kahn Philip J

    2012-01-01

    Full Text Available Abstract Objective Behcet's is an idiopathic multi-organ syndrome, which may have onset during childhood. Vascular involvement is uncommon, with rarely reported coronary aneurysm formation. We present a case report of a teenager girl who developed recalcitrant life-threatening Behcet's vasculitis, involving both small and large venous and arterial systems including a giant coronary aneurysm. Case report De-identified data were collected retrospectively in case report format. Although our sixteen year old female with Behcet's vasculitis had resolution of many arterial aneurysms, she had persistent venous thrombosis of large vessels, as well as persistent, giant arterial aneurysms requiring intra-arterial coiling of a lumbar artery and coronary bypass grafting despite intensive immunosuppression including glucocorticoids, cyclophosphamide, infliximab, methotrexate, azathioprine and intravenous immunoglobulin. Conclusions Vascular manifestations may be seen in Behcet's syndrome, including asymptomatic coronary aneurysm, which may be refractory to immunosuppression and ultimately require surgical intervention. Increased awareness is essential for prompt diagnosis and management.

  14. Pediatric Toxic Shock Syndrome

    Directory of Open Access Journals (Sweden)

    Jennifer Yee

    2017-09-01

    Full Text Available Audience: This scenario was developed to educate emergency medicine residents on the diagnosis and management of a pediatric patient with toxic shock syndrome. The case is also appropriate for teaching of medical students and advanced practice providers, as well as a review of the principles of crisis resource management, teamwork, and communication. Introduction: Toxic shock syndrome is a low-frequency, high-acuity scenario requiring timely identification and aggressive management. If patients suffering from this condition are managed incorrectly, they may progress into multi-organ dysfunction and potentially death. Toxic shock syndrome has been associated with Streptococcus and Staphylococcus aureus (Staph. Approximately half of Staph cases are associated with menstruation, which was first described in the 1970s-1980s and was associated with the use of absorbent tampons.1 Group A Streptococcus may cause complications such as necrotizing fasciitis and gangrenous myositis.2 Pediatric patients may present critically ill from toxic shock syndrome. Providers need to perform a thorough history and physical exam to discern the source of infection. Management requires aggressive care with antibiotics and IV fluids. Objectives: By the end of this simulation session, the learner will be able to: 1 Recognize toxic shock syndrome. 2 Review the importance of a thorough physical exam. 3 Discuss management of toxic shock syndrome, including supportive care and the difference in antibiotic choices for streptococcal and staphylococcal toxic shock syndrome. 4 Appropriately disposition a patient suffering from toxic shock syndrome. 5 Communicate effectively with team members and nursing staff during a resuscitation of a critically ill patient. Method: This session was conducted using high-fidelity simulation, followed by a debriefing session and lecture on toxic shock syndrome.

  15. Does it require to exclude cardiobiliary reflex in every acute coronary syndrome follow up patient with bedside ultrasound on emergency department

    Directory of Open Access Journals (Sweden)

    Mustafa Bolatkale

    2017-06-01

    Full Text Available In emergency department, physicians can diagnose pulseless electrical activity, asystole, pericardial effusions, ischemic heart disease, wall motion abnormalities, valvular cardiac disease volume status or global cardiac function evaluating with electrocardiographic findings or using bedside cardiac ultrasonography. But these two methods are not always sufficient to explain the underlying another pathologies such as pancreatitis and acute cholecystitis which can mimick acute cardiac events. Patients who are followed up with a preliminary diagnosis of acute coronary syndrome in the emergency department, might have underlying biliary or pancreatic pathologies, or even more, these might be the sole reason of the clinical picture. So bedside abdomen ultrasonography and liver enzymes may be requested in all patients with suspected cardiac pathology with a normal cardiac ultrasonography when a patient presented with acute chest or abdominal pain. Physicians must be aware for coexisting pathophysiologies and take into account the differential diagnosis of all life-threatening causes such as cardiac ischemia or acute abdominal situations. So the diagnostic tests for gallbladder pathology could be added to cardiac ultrasonography

  16. PP085. Hypertensive complication in pregnancy - HELLP syndrome - One year study (2012).

    Science.gov (United States)

    Miranda, Mariana; Ambrosio, Bruna; Gomes, Rui; Santos, Isabel; Matos, Teresa; Nazare, Antonia

    2013-04-01

    HELLP syndrome is characterized by hemolysis with a microangiopathic blood smear, elevated liver enzymes, and low platelet count. It develops in 10-20% of women with severe preeclampsia/eclampsia. The syndrome is associated with maternal morbidities - DIC, renal failure, pulmonary edema and hepatic hematoma/rupture. Some need transfusions and others require laparotomies for intraabdominal bleeding. Study the incidence and related risk factors of HELLP syndrome in Pregnancy, as well as the maternal/fetal outcome. A retrospective study of admissions for HELLP syndrome in 2012.The statistical analysis was based on Excel 2007. In 55 admissions for hypertensive complications in pregnancy, 3 women had HELLP syndrome - 2 were black race and 1 was caucasian. The average maternal age was 29. None had relevant medical history. 2 of the women developed HELLP syndrome after severe preeclampsia. The fetal/neonatal outcomes were prematurity in 2 cases, birth weight average was 1798g and none had apgar-index below 7. There was no fetal death. Premature delivery occurred in 2 cases and all were by cesarean. Maternal complications that determined Intensive Care Unit Admission was recorded in one case - laparotomy for internal bleeding and transfusions were needed. No maternal death occured. HELLP syndrome is associated with many morbidities which risk increases with severity of symptoms/lab results. We had no aggresive/fatal outcomes. Copyright © 2013. Published by Elsevier B.V.

  17. Restless legs syndrome.

    Science.gov (United States)

    Venkateshiah, Saiprakash B; Ioachimescu, Octavian C

    2015-07-01

    Restless legs syndrome is a common sensorimotor disorder characterized by an urge to move, and associated with uncomfortable sensations in the legs (limbs). Restless legs syndrome can lead to sleep-onset or sleep-maintenance insomnia, and occasionally excessive daytime sleepiness, all leading to significant morbidity. Brain iron deficiency and dopaminergic neurotransmission abnormalities play a central role in the pathogenesis of this disorder, along with other nondopaminergic systems, although the exact mechanisms are still. Intensive care unit patients are especially vulnerable to have unmasking or exacerbation of restless legs syndrome because of sleep deprivation, circadian rhythm disturbance, immobilization, iron deficiency, and use of multiple medications that can antagonize dopamine. Published by Elsevier Inc.

  18. Myelodysplastic Syndromes

    Science.gov (United States)

    ... blood cells, and the cells have a specific mutation in their DNA. Myelodysplastic syndrome with excess blasts — ... Chemicals linked to myelodysplastic syndromes include tobacco smoke, pesticides and industrial chemicals, such as benzene. Exposure to ...

  19. Moebius Syndrome

    Science.gov (United States)

    ... and supports a broad range of research on neurogenetic disorders, including Moebius syndrome. The goals of these ... and supports a broad range of research on neurogenetic disorders, including Moebius syndrome. The goals of these ...

  20. Pendred Syndrome

    Science.gov (United States)

    ... scan) to look for two characteristics of Pendred syndrome. One characteristic might be a cochlea with too few turns. ... Inner Ear Credit: NIH Medical Arts A second characteristic of Pendred syndrome is an enlarged vestibular aqueduct (see figure). The ...

  1. Rett Syndrome

    Science.gov (United States)

    Rett syndrome is a rare genetic disease that causes developmental and nervous system problems, mostly in girls. It's related to autism spectrum disorder. Babies with Rett syndrome seem to grow and ...

  2. Ohtahara Syndrome

    Science.gov (United States)

    ... but be profoundly handicapped. As they grow, some children will progress into other epileptic disorders such as West syndrome and Lennox-Gestaut syndrome. What research is being done? The NINDS conducts and supports an extensive research program on seizures ...

  3. Gardner's syndrome

    International Nuclear Information System (INIS)

    Sobrado Junior, C.W.; Bresser, A.; Cerri, G.G.; Habr-Gama, A.; Pinotti, H.W.; Magalhaes, A.

    1988-01-01

    A case of familiar poliposis of colon related to a right mandibular osteoma is reported (this association is usually called Gardner's syndrome). Radiologic pictures ae shown and some commentaries about this syndrome concerning the treatment are made. (author) [pt

  4. Turner Syndrome

    Science.gov (United States)

    Turner syndrome is a genetic disorder that affects a girl's development. The cause is a missing or incomplete X ... work properly. Other physical features typical of Turner syndrome are Short, "webbed" neck with folds of skin ...

  5. Metabolic Syndrome

    Science.gov (United States)

    Metabolic syndrome is a group of conditions that put you at risk for heart disease and diabetes. These conditions ... agree on the definition or cause of metabolic syndrome. The cause might be insulin resistance. Insulin is ...

  6. Felty syndrome

    Science.gov (United States)

    Seropositive rheumatoid arthritis (RA); Felty's syndrome ... The cause of Felty syndrome is unknown. It is more common in people who have had rheumatoid arthritis (RA) for a long time. People with ...

  7. Cushing's Syndrome

    Science.gov (United States)

    Cushing's syndrome is a hormonal disorder. The cause is long-term exposure to too much cortisol, a hormone that ... your body to make too much cortisol. Cushing's syndrome is rare. Some symptoms are Upper body obesity ...

  8. Usher Syndrome

    Science.gov (United States)

    Usher syndrome is an inherited disease that causes serious hearing loss and retinitis pigmentosa, an eye disorder that causes ... and vision. There are three types of Usher syndrome: People with type I are deaf from birth ...

  9. Piriformis syndrome

    Science.gov (United States)

    Pseudosciatica; Wallet sciatica; Hip socket neuropathy; Pelvic outlet syndrome; Low back pain - piriformis ... Sciatica is the main symptom of piriformis syndrome. Other symptoms include: Tenderness or a dull ache in ...

  10. Dressler's Syndrome

    Science.gov (United States)

    ... syndrome Overview Dressler's syndrome is a type of pericarditis — inflammation of the sac surrounding the heart (pericardium). ... reducing its ability to pump blood efficiently. Constrictive pericarditis. Recurring or chronic inflammation can cause the pericardium ...

  11. Down syndrome, RASopathies, and other rare syndromes.

    Science.gov (United States)

    Kratz, Christian P; Izraeli, Shai

    2017-04-01

    In this article we discuss the occurrence of myeloid neoplasms in patients with a range of syndromes that are due to germline defects of the RAS signaling pathway and in patients with trisomy 21. Both RAS mutations and trisomy 21 are common somatic events contributing to leukemogenis. Thus, the increased leukemia risk observed in children affected by these conditions is biologically highly plausible. Children with myeloid neoplasms in the context of these syndromes require different treatments than children with sporadic myeloid neoplasms and provide an opportunity to study the role of trisomy 21 and RAS signaling during leukemogenesis and development. Copyright © 2017 Elsevier Inc. All rights reserved.

  12. Requirement of fatty acid transport protein 4 for development, maturation, and function of sebaceous glands in a mouse model of ichthyosis prematurity syndrome.

    Science.gov (United States)

    Lin, Meei-Hua; Hsu, Fong-Fu; Miner, Jeffrey H

    2013-02-08

    Fatty acid transport protein 4 (FATP4) is one of a family of six transmembrane proteins that facilitate long- and very long-chain fatty acid uptake. FATP4 is expressed in several tissues, including skin. Mutations in human SLC27A4, which encodes FATP4, cause ichthyosis prematurity syndrome, characterized by a thick desquamating epidermis and premature birth. Mice lacking FATP4, which genetically model the human disease, are born with tight, thick skin and a defective skin barrier; they die neonatally due to dehydration and restricted movements. Both the skin phenotype and the lethality are rescued by transgene expression of FATP4 in suprabasal keratinocytes. Sebaceous glands in Fatp4 null skin grafted onto nude mice were found to be dystrophic and enwrapped by thick layers of epithelial cells. Consistent with these results, transgene-rescued Fatp4 null mice showed a subnormal level of FATP4 expression in sebocytes and exhibited abnormal development of both sebaceous glands and meibomian glands, specialized sebaceous glands of the eyelids. Sebum from these mice contained a reduced level of type II diester wax, a major mouse sebum lipid species, and showed perturbations in mass spectrometric profiles of diester wax and cholesteryl ester species. In addition, these mice showed an impaired ability to repel water and regulate body temperature after water immersion. Taken together, our results suggest that FATP4 plays crucial roles in the development and maturation of both sebaceous and meibomian glands, as well as in the formation and composition of sebum, likely by regulating the trafficking of fatty acids necessary for proper synthesis of sebum lipids.

  13. Anorexia‐cachexia syndrome in hepatoma tumour‐bearing rats requires the area postrema but not vagal afferents and is paralleled by increased MIC‐1/GDF15

    Science.gov (United States)

    Borner, Tito; Arnold, Myrtha; Ruud, Johan; Breit, Samuel N.; Langhans, Wolfgang; Lutz, Thomas A.; Blomqvist, Anders

    2016-01-01

    Abstract Background The cancer‐anorexia‐cachexia syndrome (CACS) negatively affects survival and therapy success in cancer patients. Inflammatory mediators and tumour‐derived factors are thought to play an important role in the aetiology of CACS. However, the central and peripheral mechanisms contributing to CACS are insufficiently understood. The area postrema (AP) and the nucleus tractus solitarii are two important brainstem centres for the control of eating during acute sickness conditions. Recently, the tumour‐derived macrophage inhibitory cytokine‐1 (MIC‐1) emerged as a possible mediator of cancer anorexia because lesions of these brainstem areas attenuated the anorectic effect of exogenous MIC‐1 in mice. Methods Using a rat hepatoma tumour model, we examined the roles of the AP and of vagal afferents in the mediation of CACS. Specifically, we investigated whether a lesion of the AP (APX) or subdiaphragmatic vagal deafferentation (SDA) attenuate anorexia, body weight, muscle, and fat loss. Moreover, we analysed MIC‐1 levels in this tumour model and their correlation with tumour size and the severity of the anorectic response. Results In tumour‐bearing sham‐operated animals mean daily food intake significantly decreased. The anorectic response was paralleled by a significant loss of body weight and muscle mass. APX rats were protected against anorexia, body weight loss, and muscle atrophy after tumour induction. In contrast, subdiaphragmatic vagal deafferentation did not attenuate cancer‐induced anorexia or body weight loss. Tumour‐bearing rats had substantially increased MIC‐1 levels, which positively correlated with tumour size and cancer progression and negatively correlated with food intake. Conclusions These findings demonstrate the importance of the AP in the mediation of cancer‐dependent anorexia and body weight loss and support a pathological role of MIC‐1 as a tumour‐derived factor mediating CACS, possibly via an AP

  14. Anorexia-cachexia syndrome in hepatoma tumour-bearing rats requires the area postrema but not vagal afferents and is paralleled by increased MIC-1/GDF15.

    Science.gov (United States)

    Borner, Tito; Arnold, Myrtha; Ruud, Johan; Breit, Samuel N; Langhans, Wolfgang; Lutz, Thomas A; Blomqvist, Anders; Riediger, Thomas

    2017-06-01

    The cancer-anorexia-cachexia syndrome (CACS) negatively affects survival and therapy success in cancer patients. Inflammatory mediators and tumour-derived factors are thought to play an important role in the aetiology of CACS. However, the central and peripheral mechanisms contributing to CACS are insufficiently understood. The area postrema (AP) and the nucleus tractus solitarii are two important brainstem centres for the control of eating during acute sickness conditions. Recently, the tumour-derived macrophage inhibitory cytokine-1 (MIC-1) emerged as a possible mediator of cancer anorexia because lesions of these brainstem areas attenuated the anorectic effect of exogenous MIC-1 in mice. Using a rat hepatoma tumour model, we examined the roles of the AP and of vagal afferents in the mediation of CACS. Specifically, we investigated whether a lesion of the AP (APX) or subdiaphragmatic vagal deafferentation (SDA) attenuate anorexia, body weight, muscle, and fat loss. Moreover, we analysed MIC-1 levels in this tumour model and their correlation with tumour size and the severity of the anorectic response. In tumour-bearing sham-operated animals mean daily food intake significantly decreased. The anorectic response was paralleled by a significant loss of body weight and muscle mass. APX rats were protected against anorexia, body weight loss, and muscle atrophy after tumour induction. In contrast, subdiaphragmatic vagal deafferentation did not attenuate cancer-induced anorexia or body weight loss. Tumour-bearing rats had substantially increased MIC-1 levels, which positively correlated with tumour size and cancer progression and negatively correlated with food intake. These findings demonstrate the importance of the AP in the mediation of cancer-dependent anorexia and body weight loss and support a pathological role of MIC-1 as a tumour-derived factor mediating CACS, possibly via an AP-dependent action. © 2016 The Authors. Journal of Cachexia, Sarcopenia and Muscle

  15. International Rett Syndrome Foundation

    Science.gov (United States)

    ... Newsletters & Reports About Rett Syndrome What is Rett Syndrome? Rett Syndrome Diagnosis Boys with MECP2 Clinics FAQs Glossary ... Newsletters & Reports About Rett Syndrome What is Rett Syndrome? Rett Syndrome Diagnosis Boys with MECP2 Clinics FAQs Glossary ...

  16. [Capgras syndrome].

    Science.gov (United States)

    Alcoverro Fortuny, O; Sierra Acín, A C

    2001-01-01

    The authors report a case of Capgras' syndrome in a 16-years-old child, who had been hospitalized for psychotic disorder. A review of the literature is performed. Most authors state that Capgras' syndrome would represent a symptom of underlying medical o functional disorders, although the term syndrome is used. The main etiopathogenic hypothesis of this syndrome are put forward (psychodynamic, disconnection, neuropsychological and medical).

  17. Impact of mobile intensive care unit use on total ischemic time and clinical outcomes in ST-elevation myocardial infarction patients - real-world data from the Acute Coronary Syndrome Israeli Survey.

    Science.gov (United States)

    Koifman, Edward; Beigel, Roy; Iakobishvili, Zaza; Shlomo, Nir; Biton, Yitschak; Sabbag, Avi; Asher, Elad; Atar, Shaul; Gottlieb, Shmuel; Alcalai, Ronny; Zahger, Doron; Segev, Amit; Goldenberg, Ilan; Strugo, Rafael; Matetzky, Shlomi

    2017-01-01

    Ischemic time has prognostic importance in ST-elevation myocardial infarction patients. Mobile intensive care unit use can reduce components of total ischemic time by appropriate triage of ST-elevation myocardial infarction patients. Data from the Acute Coronary Survey in Israel registry 2000-2010 were analyzed to evaluate factors associated with mobile intensive care unit use and its impact on total ischemic time and patient outcomes. The study comprised 5474 ST-elevation myocardial infarction patients enrolled in the Acute Coronary Survey in Israel registry, of whom 46% ( n=2538) arrived via mobile intensive care units. There was a significant increase in rates of mobile intensive care unit utilization from 36% in 2000 to over 50% in 2010 ( pintensive care unit use were Killip>1 (odds ratio=1.32, pintensive care units benefitted from increased rates of primary reperfusion therapy (odds ratio=1.58, pintensive care unit benefitted from shorter median total ischemic time compared with non-mobile intensive care unit patients (175 (interquartile range 120-262) vs 195 (interquartile range 130-333) min, respectively ( pintensive care unit use was the most important predictor in achieving door-to-balloon time intensive care unit group (odds ratio=0.79, 95% confidence interval (0.66-0.94), p=0.01). Among patients with ST-elevation myocardial infarction, the utilization of mobile intensive care units is associated with increased rates of primary reperfusion, a reduction in the time interval to reperfusion, and a reduction in one-year adjusted mortality.

  18. Iliopsoas Syndrome in Dancers.

    Science.gov (United States)

    Laible, Catherine; Swanson, David; Garofolo, Garret; Rose, Donald J

    2013-08-01

    Coxa saltans refers to a constellation of diagnoses that cause snapping of the hip and is a major cause of anterior hip pain in dancers. When the internal type is accompanied by weakness or pain, it is referred to as iliopsoas syndrome. Iliopsoas syndrome is the result of repetitive active hip flexion in abduction and can be confused with other hip pathology, most commonly of labral etiology. To report the incidence, clinical findings, treatment protocol, and results of treatment for iliopsoas syndrome in a population of dancers. Retrospective case series; Level of evidence, 4. A retrospective database review of 653 consecutive patients evaluated for musculoskeletal complaints over a 3-year period was completed. The diagnosis of iliopsoas syndrome was made based on anterior hip or groin pain, weakness with resisted hip flexion in abduction, or symptomatic clicking or snapping with a positive iliopsoas test. Patients identified with iliopsoas syndrome were further stratified according to age at time of onset, insidious versus acute onset, duration of symptoms, side of injury, presence of rest pain, pain with activities of daily living, and associated lower back pain. All patients diagnosed with iliopsoas syndrome underwent physical therapy, including hip flexor stretching and strengthening, pelvic mobilization, and modification of dance technique or exposure as required. A total of 49 dancers were diagnosed and treated for iliopsoas syndrome. Within this injured population of 653 patients, the incidence in female dancers was 9.2%, significantly higher than that in male dancers (3.2%). The mean age at the time of injury was 24.6 years. The incidence of iliopsoas syndrome in dancers younger than 18 years was 12.8%, compared with 7% in dancers older than 18 years. Student dancers had the highest incidence (14%), followed by amateur dancers (7.5%), while professional dancers had the lowest incidence (4.6%). All patients responded to conservative treatment, and no

  19. Velocardiofacial Syndrome

    Science.gov (United States)

    Gothelf, Doron; Frisch, Amos; Michaelovsky, Elena; Weizman, Abraham; Shprintzen, Robert J.

    2009-01-01

    Velocardiofacial syndrome (VCFS), also known as DiGeorge, conotruncal anomaly face, and Cayler syndromes, is caused by a microdeletion in the long arm of Chromosome 22. We review the history of the syndrome from the first clinical reports almost half a century ago to the current intriguing molecular findings associating genes from the…

  20. Cri-du-chat syndrome

    Directory of Open Access Journals (Sweden)

    Eka Agustia Rini

    2007-07-01

    there is no cure for cri-du-chat syndrome. The most successful approach in the management of children with CDCS requires a multidisciplinary team approach. 4 The case presented below will remind us how to reveal, suspect and diagnose Cri-Du-Chat syndrome, a rare case in pediatric.

  1. The alcohol withdrawal syndrome.

    LENUS (Irish Health Repository)

    McKeon, A

    2008-08-01

    The alcohol withdrawal syndrome (AWS) is a common management problem in hospital practice for neurologists, psychiatrists and general physicians alike. Although some patients have mild symptoms and may even be managed in the outpatient setting, others have more severe symptoms or a history of adverse outcomes that requires close inpatient supervision and benzodiazepine therapy. Many patients with AWS have multiple management issues (withdrawal symptoms, delirium tremens, the Wernicke-Korsakoff syndrome, seizures, depression, polysubstance abuse, electrolyte disturbances and liver disease), which requires a coordinated, multidisciplinary approach. Although AWS may be complex, careful evaluation and available treatments should ensure safe detoxification for most patients.

  2. Measuring Physical Activity Intensity

    Medline Plus

    Full Text Available ... videos Here are some ways to understand and measure the intensity of aerobic activity: relative intensity and ... The talk test is a simple way to measure relative intensity. In general, if you're doing ...

  3. Syndrome of pathologically changed lung image

    International Nuclear Information System (INIS)

    Ginzburg, M.A.; Kinoshenko, Yu.T.

    1987-01-01

    Syndrome of pathologically changed lung image unites radiological signs arising on the basis of vascular, stromal and bronchial lung structures. Intrasyndrome differential diagnosis of pathologically changed lung image is presented. Elements of the lung image are characterized from the view point of their localization and extension of changes, quantity, size and form of shadows, their intensity and configuration (contours). Dynamics of syndrome changes in different variants is taken account of. Methods of roentgenological study in case of different syndromes are discussed

  4. A multicenter study on Leigh syndrome

    DEFF Research Database (Denmark)

    Sofou, Kalliopi; De Coo, Irenaeus F M; Isohanni, Pirjo

    2014-01-01

    to thrive, brainstem lesions on neuroimaging and intensive care treatment were significantly associated with poorer survival. CONCLUSIONS: This is a multicenter study performed in a large cohort of patients with Leigh syndrome. Our data help define the natural history of Leigh syndrome and identify novel...

  5. Functional and structural vascular adaptations following 8 weeks of low volume high intensity interval training in lower leg of type 2 diabetes patients and individuals at high risk of metabolic syndrome

    DEFF Research Database (Denmark)

    Madsen, Søren Møller; Thorup, Anne Cathrine Sønderstgaard; Overgaard, Kristian

    2015-01-01

    We wished to investigate the effects of 8 weeks of low volume high intensity interval training (HIIT) on endothelial function of popliteal artery and circulating cell adhesion molecules in type 2 diabetes (T2D) patients and matched controls (CON). Methods: Over 8 weeks, non-active T2D patients...

  6. Noonan syndrome

    Directory of Open Access Journals (Sweden)

    van der Burgt Ineke

    2007-01-01

    Full Text Available Abstract Noonan Syndrome (NS is characterised by short stature, typical facial dysmorphology and congenital heart defects. The incidence of NS is estimated to be between 1:1000 and 1:2500 live births. The main facial features of NS are hypertelorism with down-slanting palpebral fissures, ptosis and low-set posteriorly rotated ears with a thickened helix. The cardiovascular defects most commonly associated with this condition are pulmonary stenosis and hypertrophic cardiomyopathy. Other associated features are webbed neck, chest deformity, mild intellectual deficit, cryptorchidism, poor feeding in infancy, bleeding tendency and lymphatic dysplasias. The syndrome is transmitted as an autosomal dominant trait. In approximately 50% of cases, the disease is caused by missense mutations in the PTPN11 gene on chromosome 12, resulting in a gain of function of the non-receptor protein tyrosine phosphatase SHP-2 protein. Recently, mutations in the KRAS gene have been identified in a small proportion of patients with NS. A DNA test for mutation analysis can be carried out on blood, chorionic villi and amniotic fluid samples. NS should be considered in all foetuses with polyhydramnion, pleural effusions, oedema and increased nuchal fluid with a normal karyotype. With special care and counselling, the majority of children with NS will grow up and function normally in the adult world. Management should address feeding problems in early childhood, evaluation of cardiac function and assessment of growth and motor development. Physiotherapy and/or speech therapy should be offered if indicated. A complete eye examination and hearing evaluation should be performed during the first few years of schooling. Preoperative coagulation studies are indicated. Signs and symptoms lessen with age and most adults with NS do not require special medical care.

  7. Complicaciones maternas y mortalidad perinatal en el Síndrome de Hellp: Registro multicéntrtico en unidades de cuidados intensivos del área Buenos Aires Maternal morbidity and perinatal mortality in HELLP syndrome. Multicentric studies in intensive care units in Buenos Aires area

    Directory of Open Access Journals (Sweden)

    Eduardo Malvino

    2005-03-01

    Full Text Available Se analizaron en forma retrospectiva las características clínicas, complicaciones, gravedad, y sobrevivencia materna y fetal, en un grupo de gestantes con síndrome HELLP ( Hemolysis , Elevated Liver enzyme levels, Low Platelet count que requirieron admisión en cuatro unidades de cuidados intensivos del área metropolitana Buenos Aires, Argentina. Durante el período comprendido entre marzo de 1997 y marzo de 2003 se evaluaron 62 pacientes en la segunda mitad del embarazo o el puerperio inmediato que cumplían criterios diagnósticos de hipertensión inducida por el embarazo, asociado a plaquetopenia 70 UI/l, láctico deshidrogenasa >600 UI/l, bilirrubina total >1.2 mg / dl , y/o frotis de sangre periférica con signos de hemólisis. La edad promedio fue 28 ± 8 años; número de gestas promedio 2.7 ± 2.3; edad gestacional media 33 ± 4 semanas. Según el grado de plaquetopenia, 23 casos pertenecieron a la clase 1, 29 a la clase 2 y el resto a la clase 3 de la clasificación de Martin . Hubo 16 formas eclámpticas. El recuento plaquetario promedio fue 67 604 ± 31 535/ mm3 ; TGO 271 ± 297 UI/l; TGP 209 ± 178 UI/l; LDH 1 444 ± 1 295 UI/l; creatininemia 1.1 ± 0.8 mg / dl. Cuarenta y una pacientes cursaron con diverso grado de deterioro del filtrado glomerular, con requerimiento de tratamiento hemodialítico y plasmaféresis en un caso. Se presentó insuficiencia respiratoria vinculada a síndrome de distrés respiratorio del adulto en cuatro enfermas. Todas las puérperas sobrevivieron y se comprobaron cuatro muertes perinatales. En la población estudiada, se observó baja prevalencia de complicaciones graves, óptima sobrevivencia materna y baja mortalidad perinatal.We analized the clinical characteristics, complications, severity, and maternal and fetal survival of patients suffering from HELLP syndrome ( Hemolysis , Elevated Liver enzymes level, Low Platelet count requiring admission to the intensive care unit in four hospitals from

  8. Mechanical ventilation in the acute respiratory distress syndrome.

    Science.gov (United States)

    Epelbaum, Oleg; Aronow, Wilbert S

    2017-08-01

    The management of the acute respiratory distress syndrome (ARDS) patient is fundamental to the field of intensive care medicine, and it presents unique challenges owing to the specialized mechanical ventilation techniques that such patients require. ARDS is a highly lethal disease, and there is compelling evidence that mechanical ventilation itself, if applied in an injurious fashion, can be a contributor to ARDS mortality. Therefore, it is imperative for any clinician central to the care of ARDS patients to understand the fundamental framework that underpins the approach to mechanical ventilation in this special scenario. The current review summarizes the major components of the mechanical ventilation strategy as it applies to ARDS.

  9. [Effect of posture on spinal cord stimulation in patients with chronic pain syndromes: analysis of energy requirements in different patient postures].

    Science.gov (United States)

    Abejón, D; Camacho, M; Pérez-Cajaraville, J; Ortego, R; del Pozo, C; del Saz, J

    2009-05-01

    Patients being treated with spinal cord stimulation for chronic pain complain of variable paresthesias, particularly in relation to changes in posture. Such changes affect the great majority of patients with implantable pulse generators, requiring them to use the external programmer for avoidance of painful paresthesias or even to disconnect the generator, leading to loss of pain relief. The aim of this study was to determine the relationship between the pulse charge needed for stimulation and the patient's different postures. Observational study of 70 patients treated with spinal cord stimulation in the following postures and situations: decubitus position, standing, seated, and walking. With the patients standing, we analyzed the thresholds of perception, pain, and pain relief, as well as the therapeutic range. Studies were performed in all patients. Later, data were analyzed by anatomical positioning of the stimulator (cervical, thoracic, sacral, occipital, or subcutaneous). In the analysis of the therapeutic range in the overall group we identified statistically significant differences between decubitus and standing positions and between decubitus position and walking. At the level of the thoracic spine differences were identified between all positions except between standing and walking and between seated and decubitus positions. At the level of the cervical spine, no significant differences were detected. Analysis of the pulse charge showed a significant difference in the decubitus position, in which less charge was needed to achieve satisfactory stimulation. When electrodes implanted at the cervical and thoracic levels were compared, differences were found between standing and seated positions (P=.04) but none between decubitus position or walking and the other positions. Stimulation systems are not currently designed to adapt to changes in distance between the electrodes and nerve fibers. Improvements are required in this respect.

  10. Against all odds. Conservative management of Boerhaave's syndrome.

    Science.gov (United States)

    Anwuzia-Iwegbu, Charles; Al Omran, Yasser; Heaford, Amelia

    2014-05-21

    Spontaneous oesophageal perforation or Boerhaave's syndrome is a life-threatening condition that usually requires early diagnosis and early surgical management. A 79-year-old man presented to the accident and emergency department with an ischaemic left big toe. He reported a 2-week history of worsening symptoms and a claudication distance in his left leg of 20-30 m. Three days post-revascularisation of the leg, the patient reported chest pain radiating to the back. CT angiography of the aorta indicated Boerhaave's syndrome. Following 35 days of conservative management in the intensive care unit and high dependency unit, the patient was stepped down to a surgical ward. A water-soluble contrast study demonstrated minimal leak through the perforated oesophagus. The patient was started on oral intake, which was well tolerated. This case highlights that conservative management may be appropriate. 2014 BMJ Publishing Group Ltd.

  11. Alport's Syndrome in Pregnancy

    Directory of Open Access Journals (Sweden)

    Suchita Mehta

    2013-01-01

    Full Text Available Background. Alport's syndrome is an X-linked hereditary disorder affecting the glomerular basement membrane associated with ocular and hearing defects. In women, the disease is much less severe compared to that in men. However, women with Alport's syndrome can have an accelerated form of their disease during pregnancy with worsening of kidney function and can also develop preeclampsia. There are only four described cases of Alport's syndrome in pregnancy. Case Presentation. 20-year-old woman with a history of Alport's syndrome, which during pregnancy worsened resulting in hypertension, proteinuria, and acute kidney injury. Fortunately, there was complete resolution of the proteinuria and kidney injury with delivery, and the patient did not require any renal replacement therapy. Conclusion. One of the four reported cases had an accelerated form of the disease during pregnancy with rapid progression of kidney injury and end-stage renal disease. There are no definite guidelines to monitor these patients during pregnancy. Further studies are required to understand the exact pathophysiology of kidney damage that occurs in pregnant women with Alport's syndrome. This may give us some insight into the prognostic predictors, so that we can monitor these women more thoroughly and prevent adverse outcomes.

  12. Next-generation sequencing for diagnosis of rare diseases in the neonatal intensive care unit

    Science.gov (United States)

    Daoud, Hussein; Luco, Stephanie M.; Li, Rui; Bareke, Eric; Beaulieu, Chandree; Jarinova, Olga; Carson, Nancy; Nikkel, Sarah M.; Graham, Gail E.; Richer, Julie; Armour, Christine; Bulman, Dennis E.; Chakraborty, Pranesh; Geraghty, Michael; Lines, Matthew A.; Lacaze-Masmonteil, Thierry; Majewski, Jacek; Boycott, Kym M.; Dyment, David A.

    2016-01-01

    Background: Rare diseases often present in the first days and weeks of life and may require complex management in the setting of a neonatal intensive care unit (NICU). Exhaustive consultations and traditional genetic or metabolic investigations are costly and often fail to arrive at a final diagnosis when no recognizable syndrome is suspected. For this pilot project, we assessed the feasibility of next-generation sequencing as a tool to improve the diagnosis of rare diseases in newborns in the NICU. Methods: We retrospectively identified and prospectively recruited newborns and infants admitted to the NICU of the Children’s Hospital of Eastern Ontario and the Ottawa Hospital, General Campus, who had been referred to the medical genetics or metabolics inpatient consult service and had features suggesting an underlying genetic or metabolic condition. DNA from the newborns and parents was enriched for a panel of clinically relevant genes and sequenced on a MiSeq sequencing platform (Illumina Inc.). The data were interpreted with a standard informatics pipeline and reported to care providers, who assessed the importance of genotype–phenotype correlations. Results: Of 20 newborns studied, 8 received a diagnosis on the basis of next-generation sequencing (diagnostic rate 40%). The diagnoses were renal tubular dysgenesis, SCN1A-related encephalopathy syndrome, myotubular myopathy, FTO deficiency syndrome, cranioectodermal dysplasia, congenital myasthenic syndrome, autosomal dominant intellectual disability syndrome type 7 and Denys–Drash syndrome. Interpretation: This pilot study highlighted the potential of next-generation sequencing to deliver molecular diagnoses rapidly with a high success rate. With broader use, this approach has the potential to alter health care delivery in the NICU. PMID:27241786

  13. Next-generation sequencing for diagnosis of rare diseases in the neonatal intensive care unit.

    Science.gov (United States)

    Daoud, Hussein; Luco, Stephanie M; Li, Rui; Bareke, Eric; Beaulieu, Chandree; Jarinova, Olga; Carson, Nancy; Nikkel, Sarah M; Graham, Gail E; Richer, Julie; Armour, Christine; Bulman, Dennis E; Chakraborty, Pranesh; Geraghty, Michael; Lines, Matthew A; Lacaze-Masmonteil, Thierry; Majewski, Jacek; Boycott, Kym M; Dyment, David A

    2016-08-09

    Rare diseases often present in the first days and weeks of life and may require complex management in the setting of a neonatal intensive care unit (NICU). Exhaustive consultations and traditional genetic or metabolic investigations are costly and often fail to arrive at a final diagnosis when no recognizable syndrome is suspected. For this pilot project, we assessed the feasibility of next-generation sequencing as a tool to improve the diagnosis of rare diseases in newborns in the NICU. We retrospectively identified and prospectively recruited newborns and infants admitted to the NICU of the Children's Hospital of Eastern Ontario and the Ottawa Hospital, General Campus, who had been referred to the medical genetics or metabolics inpatient consult service and had features suggesting an underlying genetic or metabolic condition. DNA from the newborns and parents was enriched for a panel of clinically relevant genes and sequenced on a MiSeq sequencing platform (Illumina Inc.). The data were interpreted with a standard informatics pipeline and reported to care providers, who assessed the importance of genotype-phenotype correlations. Of 20 newborns studied, 8 received a diagnosis on the basis of next-generation sequencing (diagnostic rate 40%). The diagnoses were renal tubular dysgenesis, SCN1A-related encephalopathy syndrome, myotubular myopathy, FTO deficiency syndrome, cranioectodermal dysplasia, congenital myasthenic syndrome, autosomal dominant intellectual disability syndrome type 7 and Denys-Drash syndrome. This pilot study highlighted the potential of next-generation sequencing to deliver molecular diagnoses rapidly with a high success rate. With broader use, this approach has the potential to alter health care delivery in the NICU. © 2016 Canadian Medical Association or its licensors.

  14. Revesz syndrome

    Directory of Open Access Journals (Sweden)

    Dayane Cristine Issaho

    2015-04-01

    Full Text Available Revesz syndrome is a rare variant of dyskeratosis congenita and is characterized by bilateral exudative retinopathy, alterations in the anterior ocular segment, intrauterine growth retardation, fine sparse hair, reticulate skin pigmentation, bone marrow failure, cerebral calcification, cerebellar hypoplasia and psychomotor retardation. Few patients with this syndrome have been reported, and significant clinical variations exist among patients. This report describes the first Brazilian case of Revesz syndrome and its ocular and clinical features.

  15. Gorlin syndrome

    Directory of Open Access Journals (Sweden)

    Basanti Devi

    2013-01-01

    Full Text Available Gorlin Syndrome, a rare genodermatosis, otherwise known as Nevoid basal cell carcinoma syndrome (NBCCS is a multisystem disease affecting skin, nervous system, eyes, endocrine glands, and bones. It is characterized by multiple basal cell carcinomas, palmoplantar pits, jaw cysts, and bony deformities like kyphoscoliosis and frontal bossing. We would like to report a case of Gorlin syndrome with classical features, as this is a rare genodermatosis.

  16. Urofacial syndrome

    Directory of Open Access Journals (Sweden)

    Kamal F Akl

    2012-01-01

    Full Text Available The urofacial syndrome is characterized by functional obstructive uropathy asso-ciated with an inverted smile. The importance of the subject is that it sheds light, not only on the muscles of facial expression, but also on the inheritance of voiding disorders and lower urinary tract malformations. We report a 10-year-old-male patient who had the urofacial syndrome. Early diagnosis of the urofacial syndrome is important to avoid upper urinary tract damage and renal failure.

  17. Joubert Syndrome

    Science.gov (United States)

    ... syndrome is inherited in an autosomal recessive manner (meaning both parents must have a copy of ... physical, occupational, and speech therapy may benefit some children. Infants with abnormal ...

  18. Reye's Syndrome

    Science.gov (United States)

    ... that contain aspirin. Some hospitals and medical facilities conduct newborn screenings for fatty acid oxidation disorders to determine which children are at greater risk of developing Reye's syndrome. ...

  19. Angelman Syndrome

    Science.gov (United States)

    ... See More About Research The NINDS supports and conducts research on neurogenetic disorders such as Angelman syndrome, to develop techniques to diagnose, treat, ... Publications Definition Angelman ...

  20. Médicos plantonistas de unidade de terapia intensiva: perfil sócio-demográfico, condições de trabalho e fatores associados à síndrome de burnout Intensive care unit physicians: socio-demographic profile, working conditions and factors associated with burnout syndrome

    Directory of Open Access Journals (Sweden)

    Dalton de Souza Barros

    2008-09-01

    of this study was to describe socio-demographic characteristics of intensive care unit physicians and evaluate factors associated to the presence of Burnout syndrome in this population. METHODS: A cross-sectional study was performed to evaluate physicians who have worked in intensive care units from the city of Salvador (Bahia - Brazil with a minimum weekly workload of 12-hour. An anonymous self-reported questionnaire was used and it was divided into two parts: socio-demographic characteristics and evaluation of Burnout syndrome through Maslach Burnout Inventory. RESULTS: We studied 297 physicians and most of them were male (70%. The mean age and time of graduation were, respectively, 34.2 and 9 years. High levels of emotional exhaustion, depersonalization, and reduced personal accomplishment were found in respectively, 47.5%, 24.6% and 28.3%. The prevalence of Burnout syndrome, considered as high level in at least one dimension, was of 63.3%. This prevalence was statistically lower in physicians specialized on intensive care, those with more than nine years of graduation and those who intend to continue working in intensive care units for more than 10 years. The prevalence was higher in the doctors with more than 24-hours of uninterrupted intensive care work per week. CONCLUSIONS: Burnout syndrome was common among intensive care physicians and it was more frequent in the youngest doctors, with higher workload and without specialization on intensive care.

  1. Infants Born with Down Syndrome: Burden of Disease in the Early Neonatal Period.

    Science.gov (United States)

    Martin, Therese; Smith, Aisling; Breatnach, Colm R; Kent, Etaoin; Shanahan, Ita; Boyle, Michael; Levy, Phillip T; Franklin, Orla; El-Khuffash, Afif

    2018-02-01

    To evaluate the incidence of direct admission of infants with Down syndrome to the postnatal ward (well newborn nursery) vs the neonatal intensive care unit (NICU), and to describe the incidence of congenital heart disease (CHD) and pulmonary hypertension (PH). This retrospective cohort study of Down syndrome used the maternal/infant database (2011-2016) at the Rotunda Hospital in Dublin, Ireland. Admission location, early neonatal morbidities, outcomes, and duration of stay were evaluated and regression analyses were conducted to identify risk factors associated with morbidity and mortality. Of the 121 infants with Down syndrome, 54 (45%) were initially admitted to the postnatal ward, but 38 (70%) were later admitted to the NICU. Low oxygen saturation profile was the most common cause for the initial and subsequent admission to the NICU. Sixty-six percent of the infants (80/121) had CHD, 34% (41/121) had PH, and 6% died. Risk factors independently associated with primary NICU admission included antenatal diagnosis of Down syndrome, presence of CHD, PH, and the need for ventilation. Infants with Down syndrome initially admitted to the postnatal ward have a high likelihood of requiring NICU admission. Overall, high rates of neonatal morbidity were noted, including rates of PH that were higher than previously reported. Proper screening of all infants with Down syndrome for CHD and PH is recommended to facilitate timely diagnoses and potentially shorten the duration of the hospital stay. Copyright © 2017 Elsevier Inc. All rights reserved.

  2. Predictive risk factors for systemic inflammatory response syndrome following ureteroscopic laser lithotripsy.

    Science.gov (United States)

    Uchida, Yusuke; Takazawa, Ryoji; Kitayama, Sachi; Tsujii, Toshihiko

    2017-07-10

    The objective of this study was to investigate risk factors for the development of systemic inflammatory response syndrome following ureteroscopic laser lithotripsy. We retrospectively collected data of 469 patients who underwent ureteroscopic laser lithotripsy at our single institution from February 2008 to June 2016. Details for the patient, the stone, and the surgical factors that potentially contributed to postoperative infection were extracted. Using a logistic regression model, we analyzed how the clinical factors affected the incidence of systemic inflammatory response syndrome. Twenty-seven patients (5.7%) were postoperatively diagnosed with systemic inflammatory response syndrome; of these, 25 patients were diagnosed within 24 h after ureteroscopy. One patient required intensive care unit admission, but no death was reported. A preoperative stent was significantly associated with postoperative systemic inflammatory response syndrome only on univariate analysis, and the reasons for stenting were varied. Multivariate analysis revealed that obstructive pyelonephritis, a positive preoperative bladder urine culture result, and female gender were significantly associated with postoperative systemic inflammatory response syndrome. Patients who experienced obstructive pyelonephritis preceding ureteroscopic laser lithotripsy or had a positive preoperative bladder urine culture result were at an increased risk of systemic inflammatory response syndrome despite receiving appropriate preoperative antibiotic therapy. Regarding the impact of a preoperative stent on postoperative infection, further investigation focusing on reasons for stenting is needed.

  3. Russell-Silver syndrome

    Science.gov (United States)

    Silver-Russell syndrome; Silver syndrome; RSS; Russell-Silver syndrome ... One in 10 children with this syndrome has a problem involving chromosome 7. In other people with the syndrome, it may affect chromosome 11. Most of the time, it ...

  4. Optimal management of subfertility in polycystic ovary syndrome

    Directory of Open Access Journals (Sweden)

    Berger JJ

    2014-06-01

    Full Text Available Joshua J Berger, G Wright Bates JrUniversity of Alabama at Birmingham, Department of Obstetrics and Gynecology, Division of Reproductive Endocrinology and Infertility, Birmingham, AL, USAAbstract: The purpose of this paper is to provide a stepwise approach to treating the infertility/subfertility associated with polycystic ovary syndrome. Defining polycystic ovary syndrome in a patient requires first investigating other possible causes for polycystic ovary morphology, acne, hirsutism, obesity, and the metabolic derangements that often accompany polycystic ovary syndrome. Beginning with lifestyle modification and use of metformin, the progressive inclusion of more intensive therapies for induction of ovulation is described. Second-line treatments are discussed and the new findings from a large multicenter trial are discussed in the context of evidence-based treatment strategies for first-line agents. Finally, monofollicular development as a treatment goal and in vitro fertilization are discussed for those with recalcitrant disease.Keywords: polycystic ovary syndrome, infertility, metformin, ovarian drilling, ovulation induction, subfertility

  5. Locomotive Syndrome: Definition and Management.

    Science.gov (United States)

    Nakamura, Kozo; Ogata, Toru

    Locomotive syndrome is a condition of reduced mobility due to impairment of locomotive organs. Since upright bipedal walking involves minutely controlled movement patterns, impairment of any aspect of the locomotive organs has the potential to adversely affect it. In addition to trauma, chronic diseases of the locomotive organs, which progress with repeated bouts of acute exacerbations, are common causes of the locomotive syndrome. In Japan's super-aging society, many people are likely to experience locomotive syndrome in the later part of their lives. Exercise intervention is effective in improving motor function, but because the subjects are elderly people with significant degenerative diseases of the locomotor organs, caution should be taken in choosing the type and intensity of exercise. The present review discusses the definition, current burden, diagnosis and interventions pertaining to the locomotive syndrome. The concept and measures are spreading throughout Japan as one of the national health policy targets.

  6. [Ballantyne syndrome or mirror syndrome].

    Science.gov (United States)

    Torres-Gómez, Luis Guillermo; Silva-González, María Eugenia; González-Hernández, Rigoberto

    2010-11-01

    Ballantyne syndrome or mirror syndrome is a triad consisting of the presence of fetal hydrops, generalized edema placentomegaly mother. May be related to any cause of fetal hydrops. The fetal prognosis is poor in untreated cases, the mother has reference to be the cause or the termination of pregnancy. Present the case of a 26-year-old who developed mirror syndrome secondary to non-immune fetal hydrops of unknown origin, accompanied by preeclampsia.

  7. Treacher Collins Syndrome

    Directory of Open Access Journals (Sweden)

    Alexander

    2010-01-01

    Full Text Available Treacher Collins syndrome is a rare autosomal dominant condition, predominantly affecting the orofacial structures. The incidence varies between 1 in 40,000 to 1 in 70,000 per live births. 40% of the cases have a hereditary factor while 60% are due to genetic mutations. The features include antimongloid slanting of the eyes, deformed or underdeveloped pinna of the ear, retrognathic mandible, microgenia, hypoplasia of the facial bones. In some patients the retrognathic mandible may cause difficulty in breathing and swallowing and may require surgical interventions. The present article describes the clinical features of Treacher Collins syndrome as seen in 3 cases.

  8. Neonatal opioid withdrawal syndrome.

    Science.gov (United States)

    Sutter, Mary Beth; Leeman, Lawrence; Hsi, Andrew

    2014-06-01

    Neonatal opioid withdrawal syndrome is common due to the current opioid addiction epidemic. Infants born to women covertly abusing prescription opioids may not be identified as at risk until withdrawal signs present. Buprenorphine is a newer treatment for maternal opioid addiction and appears to result in a milder withdrawal syndrome than methadone. Initial treatment is with nonpharmacological measures including decreasing stimuli, however pharmacological treatment is commonly required. Opioid monotherapy is preferred, with phenobarbital or clonidine uncommonly needed as adjunctive therapy. Rooming-in and breastfeeding may decease the severity of withdrawal. Limited evidence is available regarding long-term effects of perinatal opioid exposure. Copyright © 2014 Elsevier Inc. All rights reserved.

  9. [Pathomorphologic findings following intensive therapy].

    Science.gov (United States)

    Ruchti, C

    1986-05-24

    Based on autopsy findings in 301 adults who had died after intensive care, different patterns of single or multiple organ damage were identified. Signs of septicemia and/or exudative-to-fibrosing alveolitis (EFA) of the lungs, the prominent cause of the adult respiratory distress syndrome (ARDS), were recognized in 89 cases. Severe, progressive EFA, which appears to ensue mainly from continuing damage to alveolar walls, consequent fibroblast proliferation and so-called atelectatic induration (collapsed alveoli forming single thick septa), was registered only in individuals who had undergone long-term intensive care. The latter was necessitated in various severe conditions of which abdominal disease was the most important. Systemic disorders, such as hemorrhagic diathesis and multiple organ damage ("multiple organ failure"), showed a close correlation with septicemia. Infections with gram-negative bacteria appear to play a special role in such processes. Morphologically, these cases were characterized by multiple organ/system damage, e.g., in order of frequency, partial necrosis of renal proximal tubules, followed by signs of regeneration; jaundice; splenitis; lobular pneumonia; centrolobular-to-zonal liver necrosis, in part combined with cholestasis; petechial and/or extended hemorrhage; and others. This complex pattern contrasted strongly with findings in individuals who had been admitted to intensive care because of e.g. a cardiovascular incident: in this latter group (125 cases) intensive care was usually of short duration, progressive EFA was largely absent, signs of septicemia were exceptional, and multiple organ/system damage was rare. The group with polytraumatism (28 cases) exhibited a pattern of organ damage intermediate between (a) and (b).

  10. Antiphospholipid syndrome

    DEFF Research Database (Denmark)

    Cervera, Ricard; Piette, Jean-Charles; Font, Josep

    2002-01-01

    To analyze the clinical and immunologic manifestations of antiphospholipid syndrome (APS) in a large cohort of patients and to define patterns of disease expression.......To analyze the clinical and immunologic manifestations of antiphospholipid syndrome (APS) in a large cohort of patients and to define patterns of disease expression....

  11. Ascher syndrome

    Directory of Open Access Journals (Sweden)

    Zhifang Zhai

    2015-03-01

    Full Text Available Ascher syndrome is a rare, benign skin disorder characterized by a double upper lip, blepharochalasis, and nontoxic enlargement of the thyroid gland. The exact cause is unknown, but it is considered to be a hereditary disease with an autosomal dominant trait. We report here a case of forme fruste Ascher syndrome in a 29-year-old man.

  12. Passwell syndrome

    Directory of Open Access Journals (Sweden)

    Muhammed K

    2003-03-01

    Full Text Available There is an expanding list of syndromes that combine ichthyosis with neuroectodermal and mesodermal defects. We report a syndrome of congenital ichthyosis with atrophy, mental retardation, dwarfism, aminoaciduria, primary amenorrhoea and underdeveloped secondary sexual characters in a 38-year-old woman of non consanguinous parentage.

  13. Proteus syndrome

    Directory of Open Access Journals (Sweden)

    George Renu

    1993-01-01

    Full Text Available A case of proteus syndrome in a 20 year old male is repoted. Hemihypertrophy, asymmetric megalodactyly, linear epidermal naevus, naevus flammeus, angiokeratoma, lymphangioma circumscriptum, thickening of the palms and soles, scoliosis and varicose veins were present. There are only few reports of these cases in adults. The syndrome has not been reported from India.

  14. Lemierre's syndrome

    DEFF Research Database (Denmark)

    Johannesen, Katrine; Bødtger, Uffe; Heltberg, Ole

    2014-01-01

    Lemierre's syndrome is an often un-diagnosed disease seen in previously healthy young subjects, presenting with symptoms of pharyngitis, fever and elevated markers of inflammation. The syndrome is characterised by infectious thrombosis of the jugular vein due to infection with Fusobacteria, causing...

  15. Tourette Syndrome

    Science.gov (United States)

    If you have Tourette syndrome, you make unusual movements or sounds, called tics. You have little or no control over them. Common tics are throat- ... spin, or, rarely, blurt out swear words. Tourette syndrome is a disorder of the nervous system. It ...

  16. Fahr's Syndrome

    Science.gov (United States)

    ... or 50s, although it can occur at any time in childhood or adolescence. × Definition Fahr's Syndrome is a rare, genetically dominant, inherited ... or 50s, although it can occur at any time in childhood or adolescence. View Full Definition Treatment There is no cure for Fahr's Syndrome, ...

  17. Iowa Intensive Archaeological Survey

    Data.gov (United States)

    Iowa State University GIS Support and Research Facility — This shape file contains intensive level archaeological survey areas for the state of Iowa. All intensive Phase I surveys that are submitted to the State Historic...

  18. Measuring Physical Activity Intensity

    Medline Plus

    Full Text Available ... for a breath. Absolute Intensity The amount of energy used by the body per minute of activity. ... or vigorous-intensity based upon the amount of energy used by the body while doing the activity. ...

  19. Measuring Physical Activity Intensity

    Medline Plus

    Full Text Available ... using relative intensity, people pay attention to how physical activity affects their heart rate and breathing. The talk test is a simple way to measure relative intensity. ...

  20. Measuring Physical Activity Intensity

    Medline Plus

    Full Text Available ... Older Adults Overcoming Barriers Measuring Physical Activity Intensity Target Heart Rate & Estimated Maximum Heart Rate Perceived Exertion ( ... a heavy backpack Other Methods of Measuring Intensity Target Heart Rate and Estimated Maximum Heart Rate Perceived ...

  1. Stochastic conditional intensity processes

    DEFF Research Database (Denmark)

    Bauwens, Luc; Hautsch, Nikolaus

    2006-01-01

    In this article, we introduce the so-called stochastic conditional intensity (SCI) model by extending Russell’s (1999) autoregressive conditional intensity (ACI) model by a latent common dynamic factor that jointly drives the individual intensity components. We show by simulations that the propos...... for a joint latent factor and show that its inclusion allows for an improved and more parsimonious specification of the multivariate intensity process...

  2. Rainfed intensive crop systems

    DEFF Research Database (Denmark)

    Olesen, Jørgen E

    2014-01-01

    This chapter focuses on the importance of intensive cropping systems in contributing to the world supply of food and feed. The impact of climate change on intensive crop production systems is also discussed.......This chapter focuses on the importance of intensive cropping systems in contributing to the world supply of food and feed. The impact of climate change on intensive crop production systems is also discussed....

  3. TAFRO Syndrome.

    Science.gov (United States)

    Igawa, Takuro; Sato, Yasuharu

    2018-02-01

    TAFRO syndrome is a newly recognized variant of idiopathic multicentric Castleman disease (iMCD) that involves a constellation of syndromes: thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis (R), and organomegaly (O). Thrombocytopenia and severe anasarca accompanied by relatively low serum immunoglobulin levels are characteristic clinical findings of TAFRO syndrome that are not present in iMCD-not otherwise specified (iMCD-NOS). Lymph node biopsy is recommended to exclude other diseases and to diagnose TAFRO syndrome, which reveals characteristic histopathological findings similar to hyaline vascular-type CD. TAFRO syndrome follows a more aggressive course, compared with iMCD-NOS, and there is no standard treatment. Copyright © 2017 Elsevier Inc. All rights reserved.

  4. Marfan Syndrome: A Clinical Update.

    Science.gov (United States)

    Bitterman, Adam D; Sponseller, Paul D

    2017-09-01

    Marfan syndrome is a connective tissue disorder that can affect many organ systems. Affected patients present with orthopaedic manifestations of the syndrome during all phases of life. Pain caused by musculoskeletal abnormalities often requires definitive orthopaedic treatment. Orthopaedic surgeons must understand the phenotypes of Marfan syndrome so they can recognize when screening is warranted and can appropriately address the skeletal manifestations. Through medical advancements, patients with Marfan syndrome are living longer and more active lives. Knowledge of the latest diagnostic criteria for the disorder, as well as of advances in understanding the skeletal phenotype, clinical trials of medication therapy, and lifestyle considerations is important for orthopaedic surgeons who treat these patients because these clinicians often are the first to suspect Marfan syndrome and recommend screening.

  5. Pandemic influenza and pediatric intensive care

    NARCIS (Netherlands)

    Nap, Raoul E.; Andriessen, Maarten P. H. M.; Meessen, Nico E. L.; Albers, Marcel J. I. J.; van der Werf, Tjip S.

    Objective: To assess the adequacy of preparedness planning for an influenza pandemic by modeling the pediatric surge capacity of healthcare facility and pediatric intensive care unit (PICU) requirements over time. Governments and Public Health authorities have planned preparedness activities and

  6. Activity of processes of a lipoperoksidation at patients with a long postoperative pain syndrome against the combined application of application of low-intensive infrared laser therapy at impact on thymus area and pantovegin electrophoresis

    Directory of Open Access Journals (Sweden)

    Dugieva M.Z.

    2013-12-01

    Full Text Available The aim of the study was to optimize the treatment of postoperative gynecological patients using physiotherapy method. Material and methods. It was examined 220 patients in postoperative period. It was investigated antioxidant status and lipid peroxidation. Results. It is demonstrated that patients with prolonged postoperative pain disorders are more pronounced as compared with the control group. The article presents data on the effectiveness of the impact on the antioxidant system and lipid peroxidation in postoperative period of combined use of low-intensity infrared laser therapy when exposed area of the thymus and in pantovegin electrophoresis in patients sustained postoperative pain after gynecological laparotomy. Conclusion. It is shown that this method helps to reduce pain in the postoperative wound.

  7. Bouveret's syndrome

    International Nuclear Information System (INIS)

    Rehman, A.; Hasan, Z.; Saeed, A.; Abdullah, K.

    2008-01-01

    Gastric Outlet Obstruction (GOO) due to impaction of a gallstone in the duodenum after migration through a bilioduodenal fistula is known as Bouveret's syndrome. Its clinical symptoms are entirely vague and nonspecific. Because of its rarity, insidiousness and unpredictable symptomatology. Bouveret's syndrome is never thought of in the differential diagnosis as aetiology of gastric outlet obstruction. Recent advances in fiberoptics technology, advent of modern imaging modalities and minimally-invasive techniques like endoscopy and laparoscopy has brought a great revolution in the management of Bouveret's syndrome and have tremendously decreased morbidity and mortality associated with this rare clinical entity. (author)

  8. Neuroacanthocytosis Syndromes

    Directory of Open Access Journals (Sweden)

    Walker Ruth H

    2011-10-01

    Full Text Available Abstract Neuroacanthocytosis (NA syndromes are a group of genetically defined diseases characterized by the association of red blood cell acanthocytosis and progressive degeneration of the basal ganglia. NA syndromes are exceptionally rare with an estimated prevalence of less than 1 to 5 per 1'000'000 inhabitants for each disorder. The core NA syndromes include autosomal recessive chorea-acanthocytosis and X-linked McLeod syndrome which have a Huntington´s disease-like phenotype consisting of a choreatic movement disorder, psychiatric manifestations and cognitive decline, and additional multi-system features including myopathy and axonal neuropathy. In addition, cardiomyopathy may occur in McLeod syndrome. Acanthocytes are also found in a proportion of patients with autosomal dominant Huntington's disease-like 2, autosomal recessive pantothenate kinase-associated neurodegeneration and several inherited disorders of lipoprotein metabolism, namely abetalipoproteinemia (Bassen-Kornzweig syndrome and hypobetalipoproteinemia leading to vitamin E malabsorption. The latter disorders are characterized by a peripheral neuropathy and sensory ataxia due to dorsal column degeneration, but movement disorders and cognitive impairment are not present. NA syndromes are caused by disease-specific genetic mutations. The mechanism by which these mutations cause neurodegeneration is not known. The association of the acanthocytic membrane abnormality with selective degeneration of the basal ganglia, however, suggests a common pathogenetic pathway. Laboratory tests include blood smears to detect acanthocytosis and determination of serum creatine kinase. Cerebral magnetic resonance imaging may demonstrate striatal atrophy. Kell and Kx blood group antigens are reduced or absent in McLeod syndrome. Western blot for chorein demonstrates absence of this protein in red blood cells of chorea-acanthocytosis patients. Specific genetic testing is possible in all NA syndromes

  9. Cowden syndrome

    Directory of Open Access Journals (Sweden)

    Ravi Prakash S

    2010-01-01

    Full Text Available Cowden syndrome or multiple hamartoma syndrome is an autosomal dominant condition with variable expressions that result mainly from mutation in the PTEN gene on arm 10q. It is characterized by multiple hamartomatous neoplasms of the skin, oral mucosa, gastrointestinal tract, bones, CNS, eyes, and genitourinary tract. Mucocutaneous features include trichilemmomas, oral mucosal papillomatosis, acral keratosis, and palmoplantar keratosis. Here we present a case of Cowden syndrome in a 14-year-old female patient with the chief complaint of multiple oral papillomatous lesions.

  10. Premenstrual Syndrome (PMS) FAQ

    Science.gov (United States)

    ... Search FAQs Premenstrual Syndrome (PMS) Page Navigation ▼ ACOG Pregnancy Book Premenstrual Syndrome (PMS) Patient Education FAQs Premenstrual Syndrome (PMS) Patient Education Pamphlets - Spanish ...

  11. Intensity Frontier Instrumentation

    Energy Technology Data Exchange (ETDEWEB)

    Kettell S.; Rameika, R.; Tshirhart, B.

    2013-09-24

    The fundamental origin of flavor in the Standard Model (SM) remains a mystery. Despite the roughly eighty years since Rabi asked “Who ordered that?” upon learning of the discovery of the muon, we have not understood the reason that there are three generations or, more recently, why the quark and neutrino mixing matrices and masses are so different. The solution to the flavor problem would give profound insights into physics beyond the Standard Model (BSM) and tell us about the couplings and the mass scale at which the next level of insight can be found. The SM fails to explain all observed phenomena: new interactions and yet unseen particles must exist. They may manifest themselves by causing SM reactions to differ from often very precise predictions. The Intensity Frontier (1) explores these fundamental questions by searching for new physics in extremely rare processes or those forbidden in the SM. This often requires massive and/or extremely finely tuned detectors.

  12. A Nonfatal Case of Dobrava Hantavirus Hemorrhagic Fever with Renal Syndrome Combined with Hantavirus Cardiopulmonary Syndrome

    Directory of Open Access Journals (Sweden)

    Shemsedin Dreshaj

    2018-01-01

    Full Text Available Among hantaviruses (HTNV, 22 are known as pathogenic for humans. HTNV can cause two clinical entities: hemorrhagic fever with renal syndrome (HFRS and hantavirus pulmonary syndrome or hantavirus cardiopulmonary syndrome (HCPS. In most countries of Eastern Europe as well as in Kosovo, HTNV infection is presented mainly as HFRS. Here, we report a 20-year-old man with HFRS and HCPS caused by Dobrava hantavirus strain, successfully treated in Intensive Care Unit of Infectious Diseases Clinic, University Clinical Center of Kosovo. In HFRS endemic areas, patients with acute respiratory distress syndrome need to be evaluated for Dobrava hantavirus strain as a possible causative agent.

  13. Reye's Syndrome

    Science.gov (United States)

    ... vomiting Diarrhea Reye's syndrome Symptoms & causes Diagnosis & treatment Advertisement Mayo Clinic does not endorse companies or products. ... a Job Site Map About This Site Twitter Facebook Google YouTube Pinterest Mayo Clinic is a not- ...

  14. Turner Syndrome

    Science.gov (United States)

    ... girls and women with TS may have low self- esteem, anxiety, or depression. How is Turner syndrome diagnosed? ... to active partners in their health care. This fact sheet is also available in Spanish at www. ...

  15. Alport syndrome

    Science.gov (United States)

    ... have equally severe disease. Autosomal dominant Alport syndrome (ADAS) -- This is the rarest type. Males and females ... and girls have hearing loss during childhood. With ADAS, it occurs later in life. Hearing loss usually ...

  16. Sotos Syndrome

    Science.gov (United States)

    ... and social development; hypotonia (low muscle tone), and speech impairments. Clumsiness, an awkward gait, and unusual aggressiveness or irritability may also occur. Although most cases of Sotos syndrome occur sporadically (meaning they are not known to be inherited), familial ...

  17. Potter syndrome

    Science.gov (United States)

    Potter phenotype ... In Potter syndrome, the primary problem is kidney failure. The kidneys fail to develop properly as the baby is ... kidneys normally produce the amniotic fluid (as urine). Potter phenotype refers to a typical facial appearance that ...

  18. Alport Syndrome

    Science.gov (United States)

    ... signs and symptoms may differ, based on age, gender and inherited type of Alport syndrome. For example, ... prevention and treatment of kidney disease. The Better Business Bureau Wise Giving Alliance Charity Seal provides the ...

  19. Gilbert's Syndrome

    Science.gov (United States)

    ... doctor before taking new medications. Also, having certain types of Gilbert's syndrome may increase your risk of ... of Nondiscrimination Advertising Mayo Clinic is a not-for-profit organization ...

  20. Compartment syndrome

    Science.gov (United States)

    ... term (chronic) compartment syndrome can be caused by repetitive activities, such as running. The pressure in a compartment ... or loosened to relieve the pressure Stopping the repetitive activity or exercise, or changing the way it's done ...

  1. Aicardi Syndrome

    Science.gov (United States)

    ... See More About Research The NINDS supports and conducts research on neurogenetic disorders such as Aicardi syndrome. The goals of this research are to locate and understand ... Publications Definition Aicardi ...

  2. Zellweger Syndrome

    Science.gov (United States)

    ... swallow. Some babies will be born with glaucoma, retinal degeneration, and impaired hearing. Jaundice and gastrointestinal bleeding also may occur. Treatment There is no cure for Zellweger syndrome, nor ...

  3. Gerstmann's Syndrome

    Science.gov (United States)

    ... drawings. Frequently, there is also an impairment in reading. Children with a high level of intellectual functioning as well as those with brain damage may be affected with the disorder. × Definition Gerstmann's syndrome is a cognitive impairment that results ...

  4. Piriformis Syndrome

    Science.gov (United States)

    ... may order additional tests. Computerized tomography (CT) and magnetic resonance imaging (MRI) scans cannot diagnose piriformis syndrome, ... you are sitting, driving or standing. Don’t lift by bending over. Lift an object by bending ...

  5. Turner Syndrome

    Science.gov (United States)

    ... have an increased risk of an underactive thyroid (hypothyroidism) due to the autoimmune disorder Hashimoto's thyroiditis. They also have an increased risk of diabetes. Some women with Turner syndrome have gluten intolerance (celiac disease) or inflammatory bowel disease. Skeletal ...

  6. Reye Syndrome

    Science.gov (United States)

    ... Legacy Society Make Gifts of Stock Donate Your Car Personal Fundraising Partnership & Support Share Your Story Spread the Word Give While You Shop Contact Us Donate Now What Is Reye’s Syndrome? ...

  7. Alagille Syndrome

    Science.gov (United States)

    ... Legacy Society Make Gifts of Stock Donate Your Car Personal Fundraising Partnership & Support Share Your Story Spread the Word Give While You Shop Contact Us Donate Now Alagille Syndrome Back Alagille ...

  8. Cushing syndrome

    Science.gov (United States)

    ... mellitus High blood pressure (hypertension) Increased cholesterol and triglycerides (hyperlipidemia) Women with Cushing syndrome may have: Excess ... Wisse, MD, Associate Professor of Medicine, Division of Metabolism, Endocrinology & Nutrition, University of Washington School of Medicine, ...

  9. MR findings in iliotibial band syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Nishimura, G.; Yamato, M. [Department of Radiology, Dokkyo University School of Medicine, 880 Kitakobayashi, Mibu, Shimotsuga-gun, Tochigi-ken 321-02 (Japan); Tamai, K. [Department of Orthopedic Surgery, Dokkyo University School of Medicine, Tochigi-ken (Japan); Takahashi, J. [Department of Orthopedic Surgery, Shimizu Municipal Hospital, Shimizu (Japan); Uetani, M. [Department of Radiology, Nagasaki University School of Medicine, Nagasaki (Japan)

    1997-09-01

    Objective. To elucidate the MR findings in iliotibial band (ITB) syndrome. Design and patients. The subjects comprised four patients (five knees) with lateral knee pain: two athletes and two non-athletes. One non-athlete was engaged in work requiring repetitive knee movement, and the other suffered from Cushing syndrome and had bilateral abnormalities. All patients were suspected of having a lateral meniscal tear prior to MR examination, but physical examination following provisional MR diagnosis warranted the final diagnosis. MR studies included fast spin echo sagittal imaging, fat-saturated fast spin echo proton density coronal imaging, and T2* radial imaging. Twelve normal volunteers were examined. Results and conclusion. Fat-saturated coronal imaging demonstrated an ill-defined, high-intensity area deep to the ITB. T2* radial imaging showed an identical, but less conspicuous, abnormality. The MR finding suggested soft tissue inflammation and/or edema rather than focal fluid collection in the bursae. The signal alteration predominated in the region beneath the posterior fibers of the ITB, thus supporting the current opinion that the posterior fibers of the ITB are tighter against the lateral femoral epicondyle than the anterior fibers. The ITB itself did not show any signal alteration or increased thickness. (orig.). With 4 figs., 1 tab.

  10. Short-term antidiabetic treatment with insulin or metformin has a similar impact on the components of metabolic syndrome in women with gestational diabetes mellitus requiring antidiabetic agents: results of a prospective, randomised study.

    Science.gov (United States)

    Zawiejska, A; Wender-Ozegowska, E; Grewling-Szmit, K; Brazert, M; Brazert, J

    2016-04-01

    highest quartile at term. The risk of gestational weight gain and total cholesterol falling within the highest quartile at term was insignificantly reduced in the Metformin Group. In conclusion, short-term antidiabetic treatment with insulin or metformin has a similar impact on markers of metabolic syndrome in women with GDM requiring antidiabetic treatment. Secondly, treatment with metformin is associated with increased triglyceride levels and higher AIP in the third trimester in pregnant women with GDM.

  11. What is Metabolic Syndrome?

    Science.gov (United States)

    ... Research Home / Metabolic Syndrome Metabolic Syndrome What Is Metabolic syndrome is the name for a group of risk ... three metabolic risk factors to be diagnosed with metabolic syndrome. A large waistline. This also is called abdominal ...

  12. Obesity Hypoventilation Syndrome

    Science.gov (United States)

    ... Home / Obesity Hypoventilation Syndrome Obesity Hypoventilation Syndrome Also known as Pickwickian Syndrome What ... your neck is larger than normal. Complications of Obesity Hypoventilation Syndrome When left untreated, OHS can cause ...

  13. Learning about WAGR Syndrome

    Science.gov (United States)

    ... children who have WAGR syndrome may have normal intelligence. Other symptoms of WAGR syndrome may also include: ... mild. Some individuals with WAGR syndrome have normal intelligence. Children with WAGR syndrome should be referred for ...

  14. Toxic Shock Syndrome

    Science.gov (United States)

    ... may also be caused by toxins produced by group A streptococcus (strep) bacteria. Toxic shock syndrome has been associated ... syndrome. The syndrome can also be caused by group A streptococcus (strep) bacteria. Risk factors Toxic shock syndrome can ...

  15. Milk-alkali syndrome

    Science.gov (United States)

    Calcium-alkali syndrome; Cope syndrome; Burnett syndrome; Hypercalcemia; Calcium metabolism disorder ... Milk-alkali syndrome is almost always caused by taking too many calcium supplements, usually in the form of calcium ...

  16. What Causes Down Syndrome?

    Science.gov (United States)

    ... it? Share Facebook Twitter Pinterest Email Print What causes Down syndrome? Down syndrome is caused by a random error ... The Down Syndrome Registry . Chromosomal Changes That Can Cause Down Syndrome Research shows that three types of chromosomal changes ...

  17. Waardenburg syndrome: A report of three cases

    Directory of Open Access Journals (Sweden)

    Ghosh Sudip

    2010-01-01

    Full Text Available Waardenburg syndrome (WS is a rare autosomally inherited and genetically heterogeneous disorder of neural crest cell development with distinct cutaneous manifestations. Based on the clinical presentations, four subtypes of the disease are recognized. A careful clinical evaluation is required to differentiate various types of WS and other associated auditory-pigmentary syndromes. We describe a case series of WS to highlight the wide spectrum of manifestations of the syndrome including a rare association.

  18. [Ogilvie syndrome. 2 clinical case reports].

    Science.gov (United States)

    Terzi, A; Menghini, I; Manzi, A; Bonini, C A

    1991-05-31

    Acute pseudo-obstruction of the colon is an uncommon syndrome usually related to other diseases. Two patients with this syndrome are described. It is stressed that it is of paramount importance to recognize this syndrome as a high mortality is present in these patients if they are not treated in a right fashion. The treatment doesn't require surgery unless a complication, like cecum perforation, doesn't appear.

  19. Measuring Physical Activity Intensity

    Medline Plus

    Full Text Available ... or vigorous-intensity based upon the amount of energy used by the body while doing the activity. Top of Page Moderate Intensity Walking briskly (3 miles per hour or faster, but not race-walking) Water aerobics Bicycling slower than 10 miles per hour ...

  20. MECONIUM ASPIRATION SYNDROME : A CLINICAL STUDY

    OpenAIRE

    BEHERA, MK; KULKARNI, SD; GUPTA, RK

    1998-01-01

    Forty two babies with Meconium aspiration syndrome managed in neonatal intensive care unit of a service hospital were analysed. Incidence was 1.7 per cent. Twelve babies were delivered by caserean section and 30 babies by vaginal route. Seventeen deliveries were conducted by team of gynaecologists, pediatrician and nursing staff whereas 25 deliveries were conducted by nursing staff alone. Out of total babies with meconium aspiration syndrome, thin meconium detected in 9 and thick meconium det...

  1. Fournier gangrene associated with hyper IgE syndrome (Job syndrome).

    Science.gov (United States)

    Hori, Junichi; Yamaguchi, Satoshi; Watanabe, Masaki; Osanai, Hiroaki; Hori, Masako

    2008-04-01

    We report a case of a 32-year-old man with hyper IgE syndrome (Job syndrome) who developed Fournier gangrene due to infectious multiple atheromas of the scrotal skin that progressed to the right groin and thigh. The patient required surgical debridement and subsequent skin grafting. This is a rare case of Fournier gangrene associated with hyper IgE syndrome (Job syndrome). When a patient without diabetes mellitus has repeated infections and atopic-like dermatitis, Job syndrome should be considered.

  2. Management of neutropenic patients in the intensive care unit (NEWBORNS EXCLUDED) recommendations from an expert panel from the French Intensive Care Society (SRLF) with the French Group for Pediatric Intensive Care Emergencies (GFRUP), the French Society of Anesthesia and Intensive Care (SFAR), the French Society of Hematology (SFH), the French Society for Hospital Hygiene (SF2H), and the French Infectious Diseases Society (SPILF).

    Science.gov (United States)

    Schnell, David; Azoulay, Elie; Benoit, Dominique; Clouzeau, Benjamin; Demaret, Pierre; Ducassou, Stéphane; Frange, Pierre; Lafaurie, Matthieu; Legrand, Matthieu; Meert, Anne-Pascale; Mokart, Djamel; Naudin, Jérôme; Pene, Frédéric; Rabbat, Antoine; Raffoux, Emmanuel; Ribaud, Patricia; Richard, Jean-Christophe; Vincent, François; Zahar, Jean-Ralph; Darmon, Michael

    2016-12-01

    Neutropenia is defined by either an absolute or functional defect (acute myeloid leukemia or myelodysplastic syndrome) of polymorphonuclear neutrophils and is associated with high risk of specific complications that may require intensive care unit (ICU) admission. Specificities in the management of critically ill neutropenic patients prompted the establishment of guidelines dedicated to intensivists. These recommendations were drawn up by a panel of experts brought together by the French Intensive Care Society in collaboration with the French Group for Pediatric Intensive Care Emergencies, the French Society of Anesthesia and Intensive Care, the French Society of Hematology, the French Society for Hospital Hygiene, and the French Infectious Diseases Society. Literature review and formulation of recommendations were performed using the Grading of Recommendations Assessment, Development and Evaluation system. Each recommendation was then evaluated and rated by each expert using a methodology derived from the RAND/UCLA Appropriateness Method. Six fields are covered by the provided recommendations: (1) ICU admission and prognosis, (2) protective isolation and prophylaxis, (3) management of acute respiratory failure, (4) organ failure and organ support, (5) antibiotic management and source control, and (6) hematological management. Most of the provided recommendations are obtained from low levels of evidence, however, suggesting a need for additional studies. Seven recommendations were, however, associated with high level of evidences and are related to protective isolation, diagnostic workup of acute respiratory failure, medical management, and timing surgery in patients with typhlitis.

  3. Supramaximal Stimulus Intensity as a Diagnostic Tool in Chronic Demyelinating Neuropathy

    Directory of Open Access Journals (Sweden)

    Vivien Parker

    2016-01-01

    Full Text Available Objective. The ability to correctly identify chronic demyelinating neuropathy can have important therapeutic and prognostic significance. The stimulus intensity value required to obtain a supramaximal compound muscle action potential amplitude is a commonly acquired data point that has not been formally assessed as a diagnostic tool in routine nerve conduction studies to identify chronic neuropathies. We postulated that this value was significantly elevated in chronic demyelinating neuropathy. Methods. We retrospectively reviewed electrophysiology laboratory records to compare the stimulus intensity values recorded during median and ulnar motor nerve conduction studies. The groups studied included normal controls (n=42 and the following diagnostic categories: chronic inflammatory demyelinating neuropathy (CIDP (n=20, acquired inflammatory demyelinating neuropathy (AIDP (n=13, Charcot Marie Tooth (CMT type 1 or 4C (n=15, carpal tunnel syndrome (CTS (n=11, and amyotrophic lateral sclerosis (ALS (n=18. Results. Supramaximal intensities were significantly higher in patients with CMT (median nerve: 43.4 mA and CIDP (median nerve: 38.9 mA, whereas values similar to normal controls (median nerve: 25.3 mA were obtained in ALS, CTS, and AIDP. Conclusions. Supramaximal stimulus intensity may be used as an additional criterion to identify the pathophysiology of neuropathy. We postulate that endoneurial hypertrophic changes may increase electrical impedance and thus the threshold of excitation at nodes of Ranvier.

  4. Pfeiffer syndrome

    Directory of Open Access Journals (Sweden)

    Fryns Jean-Pierre

    2006-06-01

    Full Text Available Abstract Pfeiffer syndrome is a rare autosomal dominantly inherited disorder that associates craniosynostosis, broad and deviated thumbs and big toes, and partial syndactyly on hands and feet. Hydrocephaly may be found occasionally, along with severe ocular proptosis, ankylosed elbows, abnormal viscera, and slow development. Based on the severity of the phenotype, Pfeiffer syndrome is divided into three clinical subtypes. Type 1 "classic" Pfeiffer syndrome involves individuals with mild manifestations including brachycephaly, midface hypoplasia and finger and toe abnormalities; it is associated with normal intelligence and generally good outcome. Type 2 consists of cloverleaf skull, extreme proptosis, finger and toe abnormalities, elbow ankylosis or synostosis, developmental delay and neurological complications. Type 3 is similar to type 2 but without a cloverleaf skull. Clinical overlap between the three types may occur. Pfeiffer syndrome affects about 1 in 100,000 individuals. The disorder can be caused by mutations in the fibroblast growth factor receptor genes FGFR-1 or FGFR-2. Pfeiffer syndrome can be diagnosed prenatally by sonography showing craniosynostosis, hypertelorism with proptosis, and broad thumb, or molecularly if it concerns a recurrence and the causative mutation was found. Molecular genetic testing is important to confirm the diagnosis. Management includes multiple-staged surgery of craniosynostosis. Midfacial surgery is performed to reduce the exophthalmos and the midfacial hypoplasia.

  5. The Syndrome of Catatonia

    Directory of Open Access Journals (Sweden)

    James Allen Wilcox

    2015-12-01

    Full Text Available Catatonia is a psychomotor syndrome which has historically been associated with schizophrenia. Many clinicians have thought that the prevalence of this condition has been decreasing over the past few decades. This review reminds clinicians that catatonia is not exclusively associated with schizophrenia, and is still common in clinical practice. Many cases are related to affective disorders or are of an idiopathic nature. The illusion of reduced prevalence has been due to evolving diagnostic systems that failed to capture catatonic syndromes. This systemic error has remained unchallenged, and potentiated by the failure to perform adequate neurological evaluations and catatonia screening exams on psychiatric patients. We find that current data supports catatonic syndromes are still common, often severe and of modern clinical importance. Effective treatment is relatively easy and can greatly reduce organ failure associated with prolonged psychomotor symptoms. Prompt identification and treatment can produce a robust improvement in most cases. The ongoing prevalence of this syndrome requires that psychiatrists recognize catatonia and its presentations, the range of associated etiologies, and the import of timely treatment.

  6. The Syndrome of Catatonia

    Science.gov (United States)

    Wilcox, James Allen; Reid Duffy, Pam

    2015-01-01

    Catatonia is a psychomotor syndrome which has historically been associated with schizophrenia. Many clinicians have thought that the prevalence of this condition has been decreasing over the past few decades. This review reminds clinicians that catatonia is not exclusively associated with schizophrenia, and is still common in clinical practice. Many cases are related to affective disorders or are of an idiopathic nature. The illusion of reduced prevalence has been due to evolving diagnostic systems that failed to capture catatonic syndromes. This systemic error has remained unchallenged, and potentiated by the failure to perform adequate neurological evaluations and catatonia screening exams on psychiatric patients. We find that current data supports catatonic syndromes are still common, often severe and of modern clinical importance. Effective treatment is relatively easy and can greatly reduce organ failure associated with prolonged psychomotor symptoms. Prompt identification and treatment can produce a robust improvement in most cases. The ongoing prevalence of this syndrome requires that psychiatrists recognize catatonia and its presentations, the range of associated etiologies, and the import of timely treatment. PMID:26690229

  7. Nutritional armor for the injured warfighter: omega-3 fatty acids in surgery, trauma, and intensive care.

    Science.gov (United States)

    McCarthy, Mary S; Morgan, Brian B; Heineman, John T; Martindale, Robert G

    2014-11-01

    Nutrients have traditionally been viewed as a means to provide basic energy for cellular homeostasis and amino acids for protein synthesis in all humans. Young, healthy men and women in the military today are presumed to be well nourished and mentally and physically fit to perform their duties in austere environments. Exposure to high-intensity projectiles, blast injuries, and other wounds of war, however, is an everyday occurrence during deployment that potentially challenges all homeostatic mechanisms. After sustaining such devastating injuries, critically ill, surgical, and trauma patients are in a constant dynamic state between the systemic inflammatory response syndrome (and compensatory anti-inflammatory response syndrome. Compelling evidence supports both immune and metabolic response modulation by specific nutrients, including omega-3 fatty acids, primarily eicosapentaenoic acid (EPA), and docosahexaenoic acid (DHA). The concept of providing nutrients as therapeutic rather than supportive agents to meet the basic cellular caloric and metabolic demands requires a major paradigm shift. Although the exact route and dose of these metabolically active lipids has yet to be determined, data from large clinical studies of cellular ex-vivo experiments in patients support the liberal use of eicosapentaenoic acid and docosahexaenoic acid in the setting of trauma, surgery, and intensive care. Reprint & Copyright © 2014 Association of Military Surgeons of the U.S.

  8. Goodpasture's syndrome associated with pulmonary eosinophilic vasculitis.

    Science.gov (United States)

    Komadina, K H; Houk, R W; Vicks, S L; Desrosier, K F; Ridley, D J; Boswell, R N

    1988-08-01

    Lung hemorrhage and antiglomerular basement membrane (anti-GBM) antibody mediated nephritis define Goodpasture's syndrome. We present the case of a 19-year-old Caucasian woman with unique clinical findings of Goodpasture's syndrome. Our patient initially presented with leukocytoclastic vasculitis of the skin followed by the development of nephritis and lung hemorrhage. An open lung biopsy done prior to diagnosing anti-GBM antibody disease demonstrated an intense eosinophilic vasculitis. Skin vasculitis has only been rarely reported, and to our knowledge this is the first reported case of pulmonary eosinophilic vasculitis associated with Goodpasture's syndrome.

  9. Compartment syndromes

    Science.gov (United States)

    Mubarak, S. J.; Pedowitz, R. A.; Hargens, A. R.

    1989-01-01

    The compartment syndrome is defined as a condition in which high pressure within a closed fascial space (muscle compartment) reduces capillary blood perfusion below the level necessary for tissue viability'. This condition occurs in acute and chronic (exertional) forms, and may be secondary to a variety of causes. The end-result of an extended period of elevated intramuscular pressure may be the development of irreversible tissue injury and Volkmann's contracture. The goal of treatment of the compartment syndrome is the reduction of intracompartmental pressure thus facilitating reperfusion of ischaemic tissue and this goal may be achieved by decompressive fasciotomy. Controversy exists regarding the critical pressure-time thresholds for surgical decompression and the optimal diagnostic methods of measuring intracompartmental pressures. This paper will update and review some current knowledge regarding the pathophysiology, aetiology, diagnosis, and treatment of the acute compartment syndrome.

  10. Fraser syndrome

    DEFF Research Database (Denmark)

    Barisic, Ingeborg; Odak, Ljubica; Loane, Maria

    2013-01-01

    Fraser syndrome is a rare autosomal recessive disorder characterized by cryptophthalmos, cutaneous syndactyly, laryngeal, and urogenital malformations. We present a population-based epidemiological study using data provided by the European Surveillance of Congenital Anomalies (EUROCAT) network...... of birth defect registries. Between January 1990 and December 2008, we identified 26 cases of Fraser syndrome in the monitored population of 12,886,464 births (minimal estimated prevalence of 0.20 per 100,000 or 1:495,633 births). Most cases (18/26; 69%) were registered in the western part of Europe, where...... was particularly high (42%). Most cases of Fraser syndrome (85%) are suspected prenatally, often due to the presence of the association of renal agenesis and cryptophthalmos. In the European population, a high proportion (82%) of pregnancies is terminated, thus reducing the live birth prevalence to a third...

  11. Dressler Syndrome

    Directory of Open Access Journals (Sweden)

    Erkan Ceylan

    2016-02-01

    Full Text Available Dressler Syndrome (DS is a febrile illness secondary to an inflammatory reaction involving the pleura and pericardium. It is more common in patients who have undergone surgery that involves opening the pericardium. However, DS has also been described following myocardial infarction and as an unusual complication after percutaneous procedures such as coronary stent implantation, after implantation of epicardial pacemaker leads and transvenous pacemaker leads, and following blunt trauma, stab wounds, and heart puncture. Pericardial effusions often accompany the syndrome and may develop into early or late postoperative cardiac tamponade and even recurrent cardiac tamponade. The syndrome is also characterized by pericardial or pleuritic pain, pleural effusions, pneumonitis, and abnormal ECG and radiography findings.

  12. BRUGADA SYNDROME

    Directory of Open Access Journals (Sweden)

    Tomislav Kostić

    2015-06-01

    Full Text Available In 1992, Brugada syndrom was introduced as a new clinical entity linking typical but variable ST segment changes in the right precordial leads to an increased vulnerability for lethal ventricular arrhythmias. The diagnosis of Brugada syndrome is based on clinical and electrocardiographic features. Recent studies illustrate the dynamic character of these ECG patterns. Whenever a large number of baseline ECGs was available during a follow-up, the diagnostic pattern could be documented only in approximately 25% of the tracings. Because the presence of the spontaneous coved type I ECG pattern is thought to be a useful predictor of future arrhythmic events in asymptomatic patients, these findings are of great clinical importance. ICD implantation is an option for the patients with Brugada syndrome and ventricular tachycardia or fibrillation. Extensive research is ongoing to find alternative pharmacological options for these patients, especially for patients in whom ICD implantation is contraindicated for various reasons.

  13. Hispanic Inpatient Pain Intensity.

    Science.gov (United States)

    McDonald, Deborah Dillon; Ambrose, Margaret; Morey, Barbara

    2015-11-01

    Hispanic adults experience significant pain, but little is known about their pain during hospitalization. The purpose of this research was to describe Hispanic inpatients' pain intensity and compare their pain intensity with that of non-Hispanic patients. A post hoc descriptive design was used to examine 1,466 Hispanic inpatients' medical records (63.2% English speakers) and 12,977 non-Hispanic inpatients' medical records from one hospital for 2012. Mean documented pain intensity was mild for both Hispanic and non-Hispanic inpatients. Pain intensity was greater for English-speaking Hispanic patients than Spanish speakers. The odds of being documented with moderate or greater pain intensity decreased 30% for Spanish-speaking patients. Greater pain intensity documented for English-speaking Hispanic inpatients suggests underreporting of pain intensity by Spanish-speaking patients. Practitioners should use interpreter services when assessing and treating pain with patients who speak languages different from the practitioners' language(s). © The Author(s) 2014.

  14. Intensive therapy of severe complications of influenza

    Directory of Open Access Journals (Sweden)

    V. I. Kozhokaru

    2012-01-01

    Full Text Available Definition of risk factors and clinical characteristics of heavy complications of influenza for working out of criteria of polymodal intensive therapy and resuscitation was the work purpose. Results of linical supervision by 114 sick heavy form of influenza with pulmonary and extra pulmonary complications are presented. All patients suffered from virus pneumonia and sharp defeat of lungs /sharp respirator distress-syndrome (OPL/ORDS. Pulmonary complications included bacterial (85, 96 % and micotic (2, 63 % pneumonia, thrombosis of legoch arteries (19,29 %, the sharp respiratory insufficiency, caused by the polyneuropathy of a critical condition of the patient (18,42 %, the exssudative pleuritis (9,65 %, spontaneous pheumothorax (4,39 %, spontaneous pheumomediastinum (2,69 %. Extra pulmonary complications concerned the swell of brain at 100 % of patients, including, with development of a brain coma (26,32 %, sepsis (85,96 %, including, with development of septic shock (68,42 %, syndrome of disseminated intravenous coagulation (49,12 %, multyorgan insufficiency (56,14 %, sharp heavy miocarditis (22,81 %, sharp nephritic insufficiency (18,42 %, sharp hepatic insufficiency (10,52 %. Early development of pathophysiological processes (the expressed infringements of a gas exchange and an acid-base condition, a secondary immunodeficiency, heamodynamical insufficiency,m etc. leads to underestimation of severity of the patient’s condition and to failer of algorithm of polymodal intensive therapy. Development of severe complications(sepsis, septic shock, sharp respirator distress-syndrome demands expansion for epidemic of specialized medicalstructures on rendering timely intensive therapy and resuscitation.

  15. Anticholinergic syndrome following an unintentional overdose of scopolamine

    Directory of Open Access Journals (Sweden)

    Carmela E Corallo

    2009-09-01

    Full Text Available Carmela E Corallo1, Ann Whitfield2, Adeline Wu21Department of Pharmacy, The Alfred, Melbourne, Victoria, Australia; 2Intensive Care Unit, Box Hill Hospital, Melbourne, Victoria, AustraliaAbstract: Scopolamine hydrobromide (hyoscine is an antimuscarinic drug which is primarily used in the prophylaxis and treatment of motion sickness and as a premedication to dry bronchial and salivary secretions. In acute overdosage, the main clinical problem is central nervous system (CNS depression. In Australia, tablets containing scopolamine hydrobromide 0.3 mg are available over the counter in packs of ten. The recommended dose for adults is one to two tablets as a single dose, repeated four to six hours later, if required. The maximum dose stated on the pack is four tablets over a 24-hour period with a caution regarding drowsiness and blurred vision. We describe a patient who presented with symptoms of anticholinergic syndrome secondary to an unintentional overdose of scopolamine. Whilst at work, the patient noticed that he had forgotten his prescribed medication, domperidone, at home; a friend gave him some travel sickness medication which contained scopolamine for relief of nausea. On a previous occasion, he had experienced a similar, less severe reaction with another anticholinergic agent, loperamide. This report highlights the need to consider nonprescription products, ie, over the counter medications, herbal/nutritional supplements as causes of anticholinergic syndrome when a patient presents with symptoms suggestive of this diagnosis.Keywords: domperidone, scopolamine, nonprescription drugs, toxicity, anticholinergic syndrome

  16. Eagle syndrome

    International Nuclear Information System (INIS)

    Raina, Deepika; Gothi, Rajesh; Rajan, Sriram

    2009-01-01

    Eagle syndrome occurs due to elongation of the styloid process or calcification of the stylohyoid ligament, which then may produce a pain sensation due the pressure exerted on various structures in the head and neck. When suspected, imaging helps in identifying the abnormally elongated styloid process or the calcified ligament. In recent years, three-dimensional CT (3DCT) has proved to be valuable in these cases. We report the case of a 62-year-old man with this syndrome in whom imaging with 3DCT conclusively established the diagnosis

  17. Lemierre's syndrome.

    LENUS (Irish Health Repository)

    O'Dwyer, D N

    2012-02-01

    Lemierre\\'s syndrome is a rare disease that results in an oropharyngeal infection, which precipitates an internal jugular vein thrombosis and metastatic infection. Fusobacterium necrophorum is an anaerobic Gram-negative bacillus and has been identified as the causative agent. We describe the case of a young girl whose presentation and diagnosis were confounded by a history of valvular heart disease. Infection of heart valves can produce many of the signs and symptoms associated with Lemierre\\'s syndrome. We describe the diagnosis, investigation and optimal management of this rare disorder.

  18. Olmsted syndrome

    Directory of Open Access Journals (Sweden)

    Kumar Pramod

    2008-01-01

    Full Text Available Olmsted syndrome is a rare disorder characterized by the combination of periorificial, keratotic plaques and bilateral palmoplantar keratoderma. New associated features are being reported. Olmsted syndrome is particularly rare in a female patient, and we report such a case in a six year-old Indian girl, who presented with keratoderma of her soles since birth and on her palms since the age of two years along with perioral and perinasal hyperkeratosis. She had sparse, light brown, thin hair. Although the psychomotor development of the child was normal until 18 months of age, the keratoderma plaques had restricted the child′s mobility after that stage.

  19. Rapunzel syndrome

    International Nuclear Information System (INIS)

    Al-Wadan, Ali H.; Al-Saai, Azan S.; Abdoulgafour, Mohamed; Al-Absi, Mohamed

    2006-01-01

    An 18-year-old single female patient, presented with non specific gastrointestinal symptoms of anorexia, abdominal pain, and change in bowel habit. Clinically she was anemic, cachectic, and depressed. Abdominal examination revealed mobile epigastric mass. The scalp alopecia and endoscopy coupled by computed tomography scan, confirmed the diagnoses of trichobezoar, but it was not diagnosed as Rapunzel syndrome except after laparotomy, gastrotomy, and enterotomy. There are less than 16 cases of Rapunzel syndrome described worldwide, and this is the first case to be described in the middle east. (author)

  20. Fenton's syndrome

    International Nuclear Information System (INIS)

    Rimondi, E.; Albasini, V.

    1989-01-01

    The authors report two recent cases of Fenton's syndrome, a very rare carpal fracture-dislocation. After some anatomophysiopathological considerations and a review of the literature, a wider nosographic frame is proposed in which the entity of the dislocation of the head of capitate bone is not essential. According to both the literature and personal findings, the authors remark that this syndrome is always found in the presence of two morphological variants of the distal radioulnar joint. Finally, the authors stress the importance of a corect diagnosis of this lesion to avoid unnecessary attempts of reduction

  1. Waardenburg syndrome

    Directory of Open Access Journals (Sweden)

    Tagra Sunita

    2006-01-01

    Full Text Available Waardenburg syndrome is a rare inherited and genetically heterogenous disorder of neural crest cell development. Four distinct subtypes showing marked interfamilial and intrafamilial variability have been described. We report a girl showing constellation of congenital hearing impairment with 110 dB and 105 dB loss in right and left ear respectively, hypoplastic blue iridis, white forelock, dystopia canthorum and broad nasal root. Other affected relatives of the family, with variable features of the syndrome, have been depicted in the pedigree.

  2. Morbihan syndrome

    Directory of Open Access Journals (Sweden)

    Stefano Veraldi

    2013-01-01

    Full Text Available We report a case of severe Morbihan syndrome (chronic erythematous edema of the upper portion of the face in a 60-year-old man. The syndrome was characterized clinically by erythematous edema involving the forehead, glabella, and both eyelids, because of which the patient was not able to open completely his eyes. Furthermore, erythema and telangiectasiae were visible on the nose and cheeks. Laboratory and instrumental examinations were within normal ranges or negative. Histopathological examination showed dermal edema, perivascular and periadnexal lympho-histiocytic infiltrate, and sebaceous gland hyperplasia. Oral isotretinoin was ineffective despite the relatively long duration of the therapy (26 weeks.

  3. Joubert syndrome

    International Nuclear Information System (INIS)

    Villanua, J.A.; Lopez, J.M.; Recondo, J.A.; Garcia, J.M.; Gaztanaga, R.

    1998-01-01

    Joubert syndrome is a rare malformation of the posterior fossa, mainly affecting the cerebellar vermis, which generally appears as a dysplastic lesion. Other structures of the cervico medullary junction may be involved, with accompanying brainstem hypoplasia according to neuroimaging studies. The diagnosis is usually reached during, childhood, based on a constellation of changes in the child's neurological development that are supported by the results of imaging studied. Respiratory problems are the most common signs in newborns,leading to the suspicion of the presence of this syndrome. (Author) 11 refs

  4. Lemierre's syndrome

    International Nuclear Information System (INIS)

    Gong, J.; Garcia, J.

    1999-01-01

    Postanginal sepsis, or Lemierre's syndrome, is rare but with life-threatening potential involving mainly infants and adolescents. The morbidity or mortality is caused mainly by lack of knowledge of the syndrome. The 18-year-old boy described here developed a jugular thrombosis 7 days after an angina. Fusobacterium necrophorum was isolated from the culture of the excised jugular vein. Secondary embolism involved the lungs, associated with an iliac osteomyelitis and sacroiliitis. Computed tomography was used for diagnosis and follow-up. (orig.)

  5. Turner Syndrome

    Directory of Open Access Journals (Sweden)

    Ramachandran Sudarshan

    2012-08-01

    Full Text Available Turner syndrome is a genetic disorder that affects mostly females. Affected females have characteristic features such as short stature, premature ovarian failure, and several other features. Oral manifestations of this condition are not much discussed in the literature. But reported literature includes teeth, palate, periodontal and salivary changes. So the aim of this review is to illustrate the general manifestations, and especially the oral manifestations of Turner syndrome and evaluate their possible management. [Archives Medical Review Journal 2012; 21(4.000: 246-252

  6. Poland's syndrome associated with chronic granulocytic leukemia.

    Science.gov (United States)

    Costa, R; Afonso, E; Benedito, M; Maricato, L

    1991-10-01

    Poland's syndrome has been sporadically associated with haematological neoplasms, namely acute lymphoblastic and myeloblastic leukaemias and non-Hodgkin's lymphomas. The authors present the case of a child in whom this syndrome coexists with a Philadelphia negative, chronic granulocytic leukaemia, which has only required one course of treatment with busulphan in two and a half years of follow-up.

  7. Seamless Requirements

    OpenAIRE

    Naumchev, Alexandr; Meyer, Bertrand

    2017-01-01

    Popular notations for functional requirements specifications frequently ignore developers' needs, target specific development models, or require translation of requirements into tests for verification; the results can give out-of-sync or downright incompatible artifacts. Seamless Requirements, a new approach to specifying functional requirements, contributes to developers' understanding of requirements and to software quality regardless of the process, while the process itself becomes lighter...

  8. Anxiety in Asperger's Syndrome: Assessment in Real Time

    Science.gov (United States)

    Hare, Dougal J.; Wood, Christopher; Wastell, Sarah; Skirrow, Paul

    2015-01-01

    Anxiety is a major problem for many people with Asperger's syndrome who may have qualitatively different fears from a non-Asperger's syndrome population. Research has relied on measures developed for non-Asperger's syndrome populations that require reporting past experiences of anxiety, which may confound assessment in people with Asperger's…

  9. Chromosome 22q11.2 microdeletion syndrome.

    Science.gov (United States)

    Molesky, Marion G

    2011-01-01

    Chromosome 22q11.2 microdeletion syndrome is the most common microdeletion syndrome in humans. It involves the loss of genetic material on the short arm of one of the chromosome 22 alleles. Until advanced testing was available, this syndrome was known by various names including DiGeorge syndrome and velo-cardio-facial syndrome. This syndrome has a varied presentation with significant abnormalities including congenital heart disease, hypocalcemia, immunologic deficiencies, learning disabilities, and behavioral problems. A multidisciplinary approach is required to diagnose and manage the varied manifestations.

  10. Measuring Physical Activity Intensity

    Medline Plus

    Full Text Available ... Healthy Weight Breastfeeding Micronutrient Malnutrition State and Local Programs Measuring Physical Activity Intensity Recommend on Facebook Tweet ... Healthy Weight Breastfeeding Micronutrient Malnutrition State and Local Programs File Formats Help: How do I view different ...

  11. Measuring Physical Activity Intensity

    Medline Plus

    Full Text Available ... be able to say more than a few words without pausing for a breath. Absolute Intensity The ... site? Adobe PDF file Microsoft PowerPoint file Microsoft Word file Microsoft Excel file Audio/Video file Apple ...

  12. Measuring Physical Activity Intensity

    Medline Plus

    Full Text Available ... Intensity The amount of energy used by the body per minute of activity. The table below lists ... upon the amount of energy used by the body while doing the activity. Top of Page Moderate ...

  13. Measuring Physical Activity Intensity

    Medline Plus

    Full Text Available ... Adults Need More Physical Activity MMWR Data Highlights State Indicator Report on Physical Activity, 2014 Recommendations & Guidelines ... Activity Overweight & Obesity Healthy Weight Breastfeeding Micronutrient Malnutrition State and Local Programs Measuring Physical Activity Intensity Recommend ...

  14. Measuring Physical Activity Intensity

    Medline Plus

    Full Text Available ... an activity. When using relative intensity, people pay attention to how physical activity affects their heart rate ... Obesity , National Center for Chronic Disease Prevention and Health Promotion Email Recommend Tweet YouTube Instagram Listen Watch ...

  15. Measuring Physical Activity Intensity

    Medline Plus

    Full Text Available ... 3 miles per hour or faster, but not race-walking) Water aerobics Bicycling slower than 10 miles ... Tennis (doubles) Ballroom dancing General gardening Vigorous Intensity Race walking, jogging, or running Swimming laps Tennis (singles) ...

  16. Measuring Physical Activity Intensity

    Medline Plus

    Full Text Available ... miles per hour Tennis (doubles) Ballroom dancing General gardening Vigorous Intensity Race walking, jogging, or running Swimming ... miles per hour or faster Jumping rope Heavy gardening (continuous digging or hoeing) Hiking uphill or with ...

  17. Measuring Physical Activity Intensity

    Medline Plus

    Full Text Available ... When using relative intensity, people pay attention to how physical activity affects their heart rate and breathing. ... Malnutrition State and Local Programs File Formats Help: How do I view different file formats (PDF, DOC, ...

  18. Measuring Physical Activity Intensity

    Medline Plus

    Full Text Available ... Hiking uphill or with a heavy backpack Other Methods of Measuring Intensity Target Heart Rate and Estimated ... YouTube Instagram Listen Watch RSS ABOUT About CDC Jobs Funding LEGAL Policies Privacy FOIA No Fear Act ...

  19. Measuring Physical Activity Intensity

    Medline Plus

    Full Text Available ... Activities for Children Activities for Older Adults Overcoming Barriers Measuring Physical Activity Intensity Target Heart Rate & Estimated ... YouTube Instagram Listen Watch RSS ABOUT About CDC Jobs Funding LEGAL Policies Privacy FOIA No Fear Act ...

  20. Measuring Physical Activity Intensity

    Medline Plus

    Full Text Available ... Top of Page Moderate Intensity Walking briskly (3 miles per hour or faster, but not race-walking) Water aerobics Bicycling slower than 10 miles per hour Tennis (doubles) Ballroom dancing General gardening ...

  1. Measuring Physical Activity Intensity

    Medline Plus

    Full Text Available ... than 10 miles per hour Tennis (doubles) Ballroom dancing General gardening Vigorous Intensity Race walking, jogging, or running Swimming laps Tennis (singles) Aerobic dancing Bicycling 10 miles per hour or faster Jumping ...

  2. Hepatorenal syndrome: a review | Gadour | Sudan Journal of ...

    African Journals Online (AJOL)

    The hepatorenal syndrome [HRS]is a reversible functional acute renal failure secondary to intense renal cortical vasoconstriction in a patient with liver disease. It affects around 40% of patients with cirrhosis and ascites1. The exact cause of the syndrome is not well understood. The state of liver dysfunction [Child-Pugh ...

  3. Takotsubo Cardiomyopathy in Intensive Care Unit: Prevention, Diagnosis and Management

    Directory of Open Access Journals (Sweden)

    Hannah Masoud

    2016-01-01

    Full Text Available Accurate diagnosis of Takotsubo Cardiomyopathy has substantial prognostic implications in an intensive care unit, given its increased mortality risk and association with life-threatening complications. This report seeks to discuss diagnostic modalities that can be useful in accurately differentiating Takotsubo Cardiomyopathy from Acute Coronary Syndrome, and also briefly discuss prevention and management of this cardiomyopathy in an intensive care unit. For critically ill Takotsubo patients, intensive clinicians can consider establishment of diagnosis by specific electrocardiograph changes, distinctive marked release of cardiac enzymes, characteristic echocardiograph findings, as well as invasive coronary angiography or noninvasive cardiac magnetic imaging.

  4. Poland's syndrome revisited.

    Science.gov (United States)

    Fokin, Alexander A; Robicsek, Francis

    2002-12-01

    Poland's syndrome is a rare congenital anomaly characterized by unilateral chest wall hypoplasia and ipsilateral hand abnormalities. Literary data suggest its sporadic nature. The prevailing theory of its cause is hypoplasia of the subclavian artery or its branches, which may lead to a range of developmental changes. The incidence of Poland's syndrome varies between groups (male versus female patients, congenital versus familial cases, and so on) and ranges from 1 in 7,000 to 1 in 100,000 live births. Cases of Poland's syndrome associated with leukemia, carcinoma of the hypoplastic breast, and other conditions, confirm the relationship between developmental defects and tumors, and require oncologic awareness. Various manifestations, age, and gender require different surgical approaches. Our experience, which includes 27 patients (15 male, 12 female), 20 of whom (12 male, 8 female) underwent operation, suggests that the repair should be done in two stages in children and in a single stage in adults. Reconstruction and/or stabilization of the aplastic ribs may be achieved using bone grafts or prosthetic mesh. Muscle flaps and breast implants may be used to correct muscle deficiency and breast hypoplasia and to help achieve a complete cosmetic repair.

  5. Data-intensive science

    CERN Document Server

    Critchlow, Terence

    2013-01-01

    Data-intensive science has the potential to transform scientific research and quickly translate scientific progress into complete solutions, policies, and economic success. But this collaborative science is still lacking the effective access and exchange of knowledge among scientists, researchers, and policy makers across a range of disciplines. Bringing together leaders from multiple scientific disciplines, Data-Intensive Science shows how a comprehensive integration of various techniques and technological advances can effectively harness the vast amount of data being generated and significan

  6. Plasmas and intense laser light

    International Nuclear Information System (INIS)

    Kennedy, E.T.

    1984-01-01

    The present article begins with a description of the laser technology required to reach the high irradiances of interest and provides a brief outline of the more important diagnostic techniques used in investigating the plasmas. An introduction to plasma waves is given and the linear and nonlinear excitation of waves is discussed. The remainder of the article describes some of the experimental evidence supporting the interpretation of the plasma behaviour at high laser-light intensities in terms of the excitation of plasma waves and the subsequent heating of plasma by these waves. (author)

  7. Ageing in Rett syndrome.

    Science.gov (United States)

    Cianfaglione, R; Clarke, A; Kerr, M; Hastings, R P; Oliver, C; Felce, D

    2016-02-01

    The aim was to gain a UK national sample of people with Rett syndrome across the age range and (1) conduct a cross-sectional comparison of age groups and (2) undertake a longitudinal follow-up. From 308 potential participants approached to take part, a sample of 91 girls and women was achieved (29.5%). Their ages ranged from 4 to 47 years, and 71 were known to have a mutation in the methyl-CpG binding protein-2 (MECP2) gene. Seventy-two of the initial sample were followed up 16 months later, and 50 returned completed assessments (69.4%). Their ages ranged from 7 to 48 years, and 42 were MECP2 positive. Parental questionnaire measures of Rett syndrome specific characteristics, impulsivity, overactivity, mood, interest and pleasure, repetitive behaviour and self-injury were administered. Adaptive behaviour and behavioural characteristics of Rett syndrome were similar across age groups and, where assessed, stable over time, as were repetitive behaviours generally and self-injury. There was some suggestion of deterioration in health arising with ageing, principally contributed to by deteriorations in dental and gastro-intestinal problems both with moderate effect sizes. Indicators of mood, interest and pleasure differed significantly across age groups. The total scale score significantly deteriorated over time, with a moderate effect size. This study provides further evidence for the post-regression stability that characterises Rett syndrome. Emergent low mood in Rett syndrome requires further research. © 2015 MENCAP and International Association of the Scientific Study of Intellectual and Developmental Disabilities and John Wiley & Sons Ltd.

  8. Hypoplastic left heart syndrome

    Directory of Open Access Journals (Sweden)

    Thiagarajan Ravi

    2007-05-01

    Full Text Available Abstract Hypoplastic left heart syndrome(HLHS refers to the abnormal development of the left-sided cardiac structures, resulting in obstruction to blood flow from the left ventricular outflow tract. In addition, the syndrome includes underdevelopment of the left ventricle, aorta, and aortic arch, as well as mitral atresia or stenosis. HLHS has been reported to occur in approximately 0.016 to 0.036% of all live births. Newborn infants with the condition generally are born at full term and initially appear healthy. As the arterial duct closes, the systemic perfusion becomes decreased, resulting in hypoxemia, acidosis, and shock. Usually, no heart murmur, or a non-specific heart murmur, may be detected. The second heart sound is loud and single because of aortic atresia. Often the liver is enlarged secondary to congestive heart failure. The embryologic cause of the disease, as in the case of most congenital cardiac defects, is not fully known. The most useful diagnostic modality is the echocardiogram. The syndrome can be diagnosed by fetal echocardiography between 18 and 22 weeks of gestation. Differential diagnosis includes other left-sided obstructive lesions where the systemic circulation is dependent on ductal flow (critical aortic stenosis, coarctation of the aorta, interrupted aortic arch. Children with the syndrome require surgery as neonates, as they have duct-dependent systemic circulation. Currently, there are two major modalities, primary cardiac transplantation or a series of staged functionally univentricular palliations. The treatment chosen is dependent on the preference of the institution, its experience, and also preference. Although survival following initial surgical intervention has improved significantly over the last 20 years, significant mortality and morbidity are present for both surgical strategies. As a result pediatric cardiologists continue to be challenged by discussions with families regarding initial decision

  9. Titanium exposure and yellow nail syndrome

    Directory of Open Access Journals (Sweden)

    Ali Ataya

    2015-01-01

    Full Text Available Yellow nail syndrome is a rare disease of unclear etiology. We describe a patient who develops yellow nail syndrome, with primary nail and sinus manifestations, shortly after amalgam dental implants. A study of the patient's nail shedding showed elevated nail titanium levels. The patient had her dental implants removed and had complete resolution of her sinus symptoms with no change in her nail findings. Since the patient's nail findings did not resolve we do not believe titanium exposure is a cause of her yellow nail syndrome but perhaps a possible relationship exists between titanium exposure and yellow nail syndrome that requires further studies.

  10. Toxic shock syndrome responsive to steroids

    Directory of Open Access Journals (Sweden)

    Vergis Nikhil

    2007-02-01

    Full Text Available Abstract Background Toxic Shock Syndrome is a dangerous disease with clinical features mimicking bacterial sepsis. The best management of Toxic Shock Syndrome is not determined. Case presentation A 28 year-old woman presenting with high fever, tachycardia and widespread erythroderma is described. She failed to respond to intravenous antibiotics and required ITU admission. High dose corticosteroids dramatically improved her clinical condition. Conclusion Toxic Shock Syndrome should be considered in the differential diagnosis of unexplained fever, rash and features resembling septic shock. Corticosteroids should be considered in the treatment of Toxic Shock Syndrome.

  11. Towards higher intensities

    CERN Multimedia

    CERN Bulletin

    2010-01-01

    Over the past 2 weeks, commissioning of the machine protection system has advanced significantly, opening up the possibility of higher intensity collisions at 3.5 TeV. The intensity has been increased from 2 bunches of 1010 protons to 6 bunches of 2x1010 protons. Luminosities of 6x1028 cm-2s-1 have been achieved at the start of fills, a factor of 60 higher than those provided for the first collisions on 30 March.   The recent increase in LHC luminosity as recorded by the experiments. (Graph courtesy of the experiments and M. Ferro-Luzzi) To increase the luminosity further, the commissioning crews are now trying to push up the intensity of the individual proton bunches. After the successful injection of nominal intensity bunches containing 1.1x1011 protons, collisions were subsequently achieved at 450 GeV with these intensities. However, half-way through the first ramping of these nominal intensity bunches to 3.5 TeV on 15 May, a beam instability was observed, leading to partial beam loss...

  12. Marfan syndrome masked by Down syndrome?

    NARCIS (Netherlands)

    Vis, J.C.; Engelen, K. van; Timmermans, J.; Hamel, B.C.J.; Mulder, B.J.

    2009-01-01

    Down syndrome is the most common chromosomal abnormality. A simultaneous occurrence with Marfan syndrome is extremely rare. We present a case of a 28-year-old female with Down syndrome and a mutation in the fibrillin-1 gene. The patient showed strikingly few manifestations of Marfan syndrome.

  13. Nevoid basal cell carcinoma syndrome (Gorlin syndrome

    Directory of Open Access Journals (Sweden)

    Lo Muzio Lorenzo

    2008-11-01

    Full Text Available Abstract Nevoid basal cell carcinoma syndrome (NBCCS, also known as Gorlin syndrome, is a hereditary condition characterized by a wide range of developmental abnormalities and a predisposition to neoplasms. The estimated prevalence varies from 1/57,000 to 1/256,000, with a male-to-female ratio of 1:1. Main clinical manifestations include multiple basal cell carcinomas (BCCs, odontogenic keratocysts of the jaws, hyperkeratosis of palms and soles, skeletal abnormalities, intracranial ectopic calcifications, and facial dysmorphism (macrocephaly, cleft lip/palate and severe eye anomalies. Intellectual deficit is present in up to 5% of cases. BCCs (varying clinically from flesh-colored papules to ulcerating plaques and in diameter from 1 to 10 mm are most commonly located on the face, back and chest. The number of BBCs varies from a few to several thousand. Recurrent jaw cysts occur in 90% of patients. Skeletal abnormalities (affecting the shape of the ribs, vertebral column bones, and the skull are frequent. Ocular, genitourinary and cardiovascular disorders may occur. About 5–10% of NBCCS patients develop the brain malignancy medulloblastoma, which may be a potential cause of early death. NBCCS is caused by mutations in the PTCH1 gene and is transmitted as an autosomal dominant trait with complete penetrance and variable expressivity. Clinical diagnosis relies on specific criteria. Gene mutation analysis confirms the diagnosis. Genetic counseling is mandatory. Antenatal diagnosis is feasible by means of ultrasound scans and analysis of DNA extracted from fetal cells (obtained by amniocentesis or chorionic villus sampling. Main differential diagnoses include Bazex syndrome, trichoepithelioma papulosum multiplex and Torre's syndrome (Muir-Torre's syndrome. Management requires a multidisciplinary approach. Keratocysts are treated by surgical removal. Surgery for BBCs is indicated when the number of lesions is limited; other treatments include laser

  14. Gitelman syndrome.

    NARCIS (Netherlands)

    Knoers, N.V.A.M.; Levtchenko, E.N.

    2008-01-01

    Gitelman syndrome (GS), also referred to as familial hypokalemia-hypomagnesemia, is characterized by hypokalemic metabolic alkalosis in combination with significant hypomagnesemia and low urinary calcium excretion. The prevalence is estimated at approximately 1:40,000 and accordingly, the prevalence

  15. Dravet Syndrome

    Science.gov (United States)

    ... but can be reduced by anticonvulsant drugs. A ketogenic diet, high in fats and low in carbohydrates, also may be beneficial. View Full Treatment Information Definition Dravet syndrome, also called severe myoclonic epilepsy of infancy (SMEI), is a severe form of ...

  16. Kartagener's Syndrome.

    Science.gov (United States)

    Dhar, D K; Ganguly, K C; Alam, S; Hossain, A; Sarker, U K; Das, B K; Haque, M J

    2009-01-01

    Kartagener's Syndrome or Immotile Cilia Syndrome, a variant of Primary Ciliary Dyskinesia (PCD), is a rare autosomal recessive genetic disorder caused by defect in the tiny hair like structure, the cilia lining the respiratory tract (upper and lower), sinuses, eustachian tubes, middle ear and fallopian tubes. Here electron microscopy shows abnormal arrangement of ciliary tubules and patients with Kartagener's syndrome has an absence of dynein arms at the base of the cilia. The inability of cilia to move results in inadequate clearance of bacteria from the air passages, resulting in an increased risk of infection and causing bronchiectasis. Another result of ciliary immobility is infertility. A 60 years old lady was diagnosed as a case of Kartagener's syndrome. She had history of chronic cough for 20 years, irregular fever for 20 years and occasional shortness of breath for 5 years. Relevant investigations revealed dextrocardia, situs inversus, bilateral maxillary sinusitis with non pneumatised frontal sinus and bronchiectasis. She was treated with low concentration oxygen inhalation, antibiotic, bronchodilator, chest physiotherapy including postural drainage, vitamins and other supportive treatment.

  17. Barth Syndrome

    DEFF Research Database (Denmark)

    Saric, Ana; Andreau, Karine; Armand, Anne-Sophie

    2016-01-01

    , liver and skeletal muscle of patients have revealed mitochondrial malformations and dysfunctions. It is the purpose of this review to summarize recent results of studies on various animal or cell models of Barth syndrome, which have characterized biochemically the strong cellular defects associated...

  18. Proteus syndrome

    Directory of Open Access Journals (Sweden)

    Debi Basanti

    2005-01-01

    Full Text Available Proteus syndrome is a variable and complex disorder characterized by multifocal overgrowths affecting any tissue or structure of the body. We present a girl aged 3 years and 8 months with an epidermal nevus, port-wine stain, macrodactyly with gigantism of the feet, lymphohemagiomas and multiple lipomas.

  19. Sjogren syndrome

    NARCIS (Netherlands)

    Brito-Zeron, Pilar; Baldini, Chiara; Bootsma, Hendrika; Bowman, Simon J.; Jonsson, Roland; Mariette, Xavier; Sivils, Kathy; Theander, Elke; Tzioufas, Athanasios; Ramos-Casals, Manuel

    2016-01-01

    Sjogren syndrome (SjS) is a systemic autoimmune disease that primarily affects the exocrine glands (mainly the salivary and lacrimal glands) and results in the severe dryness of mucosal surfaces, principally in the mouth and eyes. This disease predominantly affects middle-aged women, but can also be

  20. Goldenhar syndrome

    African Journals Online (AJOL)

    operational on the derivatives of the first and second bran- chial arches and clefts before the end of the organogenetic period (7'h or 8'h week of embryonic life)? ..... Marshman WE, Schalit G, Jones RB, Lee JP, Mathews TD and McCabe S: Congenital anomalies in patients with Duane retraction syndrome and their relatives.

  1. Rett Syndrome.

    Science.gov (United States)

    Culbert, Linda A.

    This pamphlet reviews the historical process involved in initially recognizing Rett Syndrome as a specific disorder in girls. Its etiology is unknown, but studies have considered factors as hyperammonemia, a two-step mutation, a fragile X chromosome, metabolic disorder, environmental causation, dopamine deficiency, and an inactive X chromosome.…

  2. Nodding Syndrome

    Centers for Disease Control (CDC) Podcasts

    2013-12-19

    Dr. Scott Dowell, a CDC director, discusses the rare illness, nodding syndrome, in children in Africa.  Created: 12/19/2013 by National Center for Emerging and Zoonotic Infectious Diseases (NCEZID).   Date Released: 1/27/2014.

  3. Metabolic syndrome

    Science.gov (United States)

    ... gov/pubmed/26718656 . Ruderman NB, Shulman GI. Metabolic syndrome. In: Jameson JL, De Groot LJ, de Kretser DM, et al, eds. Endocrinology: Adult and Pediatric . 7th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 43. Review ... NIH MedlinePlus Magazine Read more Health ...

  4. Stickler Syndrome

    Science.gov (United States)

    ... tongue to drop back toward the throat. Blindness. Blindness can occur if retinal detachments aren't repaired promptly. Ear infections. Children with facial structure abnormalities associated with Stickler syndrome are more likely to develop ear infections than are children with normal facial ...

  5. Gorlin syndrome

    African Journals Online (AJOL)

    develop medulloblastomas and are treated with radiation, it is mandatory that they be monitored for the development of BCCs in the radiation field. Management of a patient or family with this syndrome should include genetic counselling and neurological evaluations to detect medulloblastoma until the age of 7, after which ...

  6. Kartagener's Syndrome

    African Journals Online (AJOL)

    GB

    presenting with recurrent upper and lower respiratory tract infections, sinusitis or bronchiectasis. Inability to diagnose this condition may subject the patient to unnecessary and repeated hospital admissions, investigations and treatment failure. KEY WORDS: Kartagener's syndrome, primary cilliary dyskinesia, situs inversus, ...

  7. Kounis syndrome

    African Journals Online (AJOL)

    Kounis syndrome is charac terised by a group of symp toms that manifest as unsta ble vasospastic or nonvaso spastic angina secondary to a hypersensitivity reaction.[1] It was first described by. Kounis and Zavras in 1991[2] as the concurrence of an allergic response with an anaphylactoid or anaphylactic reaction and ...

  8. Recent developments for beam intensity increase operation

    International Nuclear Information System (INIS)

    Andre, T.; Anger, P.; Baelde, J.L.; Baumgarten, R.; Doutressoulles, C.; Ducoudret, B.; Etaix, G.; Jamet, C.; Mariette, C.; Petit, E.; Tichoux, G.

    1999-01-01

    The aim of the beam intensity increase operation (THI project) is to multiply the present intensities for lightest ions by a factor of fifteen in order to reach maximum power of six kilowatts. The main objective is the production of large amounts of rare isotopes, either with SISSI (device intended for producing radioactive beams), or with SPIRAL (production and acceleration of radioactive ion beams). As part of this THI project, new developments have been required such as spiral scanners, for beam profile measurements, and safety system to protect equipments against beam loses. Other developments are being carried on to improve the high intensity beam operation. (authors)

  9. Polycystic ovarian syndrome

    Directory of Open Access Journals (Sweden)

    Nina Madnani

    2013-01-01

    Full Text Available Polycystic ovarian syndrome (PCOS is a "multispeciality" disorder suspected in patients with irregular menses and clinical signs of hyperandrogenism such as acne, seborrhoea, hirsutism, irregular menses, infertility, and alopecia. Recently, PCOS has been associated with the metabolic syndrome. Patients may develop obesity, insulin resistance, acanthosis nigricans, Type 2 diabetes, dyslipidemias, hypertension, non-alcoholic liver disease, and obstructive sleep apnoea. Good clinical examination with hematological and radiological investigations is required for clinical evaluation. Management is a combined effort involving a dermatologist, endocrinologist, gynecologist, and nutritionist. Morbidity in addition includes a low "self image" and poor quality of life. Long term medications and lifestyle changes are essential for a successful outcome. This article focuses on understanding the normal and abnormal endocrine functions involved in the pathogenesis of PCOS. Proper diagnosis and management of the patient is discussed.

  10. The metabolic syndrome.

    Science.gov (United States)

    Harris, Mark F

    2013-08-01

    The metabolic syndrome (MetSy) is increasingly common in Australia. It is associated with the rise in obesity and lifestyle risk behaviours. It is also controversial - its value in predicting cardiovascular disease and diabetes risk and in guiding therapy has been challenged. This article aims to provide advice on the diagnosis of the MetSy and the principles for its prevention and management in the context of primary care, taking into consideration aetiological factors and the complexity of managing its constituent risk factors. Diagnosis of the MetSy is useful in focusing attention on central adiposity and insulin resistance as risk factors both for the syndrome, and cardiovascular and diabetes morbidity and mortality. Its assessment requires measurement of waist circumference - a simple but seldom performed procedure in general practice. The most essential components for the prevention and management of the MetSy are measures to change diet and physical activity in order to achieve and sustain weight loss.

  11. Melting graft wound syndrome

    Directory of Open Access Journals (Sweden)

    Shiou-Mei Chen

    2017-09-01

    Full Text Available Melting graft wound syndrome is characterized by progressive epidermal loss from a previously well-taken skin graft, healed burn, or donor site. It may result in considerable morbidity and require prolonged treatment. We report a 23-year-old flame-burned patient with second- to third-degree burns involving more than 70% of the total body surface area, whose condition was complicated with septic shock. The patient presented with erosions and ulcers occurring on previously well-taken skin graft recipient sites over both legs and progressive epidermal loss on donor sites over the back. The patient's presentation was compatible with the diagnosis of melting graft wound syndrome, and we successfully treated the patient with debridement and supportive treatment.

  12. The Quality of Mainstreaming in Preschool: The Views of Parents of Children with Down Syndrome

    Science.gov (United States)

    Kayhan, Nilay; Özaydin, Latife

    2018-01-01

    Down syndrome (DS), one of the developmental (cognitive) deficits, is the most common syndrome that arises from genetic disorders. The mothers of children with DS who encounter the most intense emotional situations since the tendency to take responsibility the children's care and development usually belongs them. Among these intensive feelings,…

  13. Reversible posterior leukoencephalopathy syndrome

    International Nuclear Information System (INIS)

    Lee, Eun Ja; Yu, Won Jong; Ahn, Kook Jin; Jung, So Lyung; Lee, Yeon Soo; Kim, Ji Chang; Kang, Si Won; Song, Chang Joon; Song, Soon-Young; Koo, Ja Hong; Kim, Man Deuk

    2001-01-01

    To review reversible posterior leukoencephalopathy syndrome. We reviewed 22 patients (M:F=3:19; age, 17-46 years) with the characteristic clinical and imaging features of reversible posterior leukoencephalopathy syndrome. All underwent brain MRI, and in three cases both CT and MRI were performed. In one, MRA was obtained, and in eleven, follow-up MR images were obtained. We evaluated the causes of this syndrome, its clinical manifestations, and MR findings including the locations of lesions, the presence or absence of contrast enhancement, and the changes seen at follow-up MRI. Of the 22 patients, 13 had eclampsia (six during pregnancy and seven during puerperium). Four were receiving immunosuppressive therapy (three, cyclosporine ; one, FK 506). Four suffered renal failure and one had complicated migraine. The clinical manifestations included headache (n=12), visual disturbance (n=13), seizure (n=15), focal neurologic sign (n=3), and altered mental status (n=2). Fifteen patients had hypertension and the others normotension. MRI revealed that lesions were bilateral (n=20) or unilateral (n=2). In all patients the lesion was found in the cortical and subcortical areas of the parieto-occipital lobes ; other locations were the basal ganglia (n=9), posterior temporal lobe (n=8), frontal lobe (n=5), cerebellum (n=5), pons (n=2), and thalamus (n=1). All lesions were of high signal intensity on T2-weighted images, and of iso to low intensity on T1-weighted images. One was combined with acute hematoma in the left basal ganglia. In eight of 11 patients who underwent postcontrast T1-weighted MRI, there was no definite enhancement ; in one, enhancement was mild, and in tow, patchy. CT studies showed low attenuation, and MRA revealed mild vasospasm. The symptoms of all patients improved. Follow-up MRI in nine of 11 patients depicted complete resolution of the lesions ; in two, small infarctions remained but the extent of the lesions had decreased. Reversible posterior

  14. Reversible posterior leukoencephalopathy syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Eun Ja; Yu, Won Jong; Ahn, Kook Jin; Jung, So Lyung; Lee, Yeon Soo; Kim, Ji Chang; Kang, Si Won [The Catholic Univ. of Korea, Taejon (Korea, Republic of); Song, Chang Joon [Chungnam National Univ. School of Medicine, Cheonju (Korea, Republic of); Song, Soon-Young; Koo, Ja Hong [Kwandong Univ. College of Medicine, Myungji Hospital, Seoul (Korea, Republic of); Kim, Man Deuk [College of Medicine Pochon CHA Univ., Seoul (Korea, Republic of)

    2001-10-01

    To review reversible posterior leukoencephalopathy syndrome. We reviewed 22 patients (M:F=3:19; age, 17-46 years) with the characteristic clinical and imaging features of reversible posterior leukoencephalopathy syndrome. All underwent brain MRI, and in three cases both CT and MRI were performed. In one, MRA was obtained, and in eleven, follow-up MR images were obtained. We evaluated the causes of this syndrome, its clinical manifestations, and MR findings including the locations of lesions, the presence or absence of contrast enhancement, and the changes seen at follow-up MRI. Of the 22 patients, 13 had eclampsia (six during pregnancy and seven during puerperium). Four were receiving immunosuppressive therapy (three, cyclosporine ; one, FK 506). Four suffered renal failure and one had complicated migraine. The clinical manifestations included headache (n=12), visual disturbance (n=13), seizure (n=15), focal neurologic sign (n=3), and altered mental status (n=2). Fifteen patients had hypertension and the others normotension. MRI revealed that lesions were bilateral (n=20) or unilateral (n=2). In all patients the lesion was found in the cortical and subcortical areas of the parieto-occipital lobes ; other locations were the basal ganglia (n=9), posterior temporal lobe (n=8), frontal lobe (n=5), cerebellum (n=5), pons (n=2), and thalamus (n=1). All lesions were of high signal intensity on T2-weighted images, and of iso to low intensity on T1-weighted images. One was combined with acute hematoma in the left basal ganglia. In eight of 11 patients who underwent postcontrast T1-weighted MRI, there was no definite enhancement ; in one, enhancement was mild, and in tow, patchy. CT studies showed low attenuation, and MRA revealed mild vasospasm. The symptoms of all patients improved. Follow-up MRI in nine of 11 patients depicted complete resolution of the lesions ; in two, small infarctions remained but the extent of the lesions had decreased. Reversible posterior

  15. Hemolytic Uremic Syndrome in Pregnancy and Postpartum.

    Science.gov (United States)

    Bruel, Alexandra; Kavanagh, David; Noris, Marina; Delmas, Yahsou; Wong, Edwin K S; Bresin, Elena; Provôt, François; Brocklebank, Vicky; Mele, Caterina; Remuzzi, Giuseppe; Loirat, Chantal; Frémeaux-Bacchi, Véronique; Fakhouri, Fadi

    2017-08-07

    Pregnancy is associated with various forms of thrombotic microangiopathy, including hemolytic uremic syndrome. A previous small French study suggested that pregnancy-associated hemolytic uremic syndrome was to be included in the spectrum of atypical hemolytic uremic syndrome linked to complement alternative pathway dysregulation. We sought to retrospectively analyze the presentation, outcome, and frequency of complement alternative pathway gene variants in a larger international (France, United Kingdom, Italy) cohort of patients with pregnancy-associated hemolytic uremic syndrome. Eighty-seven patients with pregnancy-associated hemolytic uremic syndrome were included. Hemolytic uremic syndrome occurred mainly during the first pregnancy (58%) and in the postpartum period (76%). At diagnosis, 56 (71%) patients required dialysis. Fifty-six (78%) patients underwent plasma exchanges, 21 (41%) received plasma infusions, and four (5%) received eculizumab. During follow-up (mean duration of 7.2 years), 41 (53%) patients reached ESRD, 15 (19%) had CKD, and 18 (28%) patients experienced hemolytic uremic syndrome relapse. Twenty-four patients (27%) received a kidney transplant and a recurrence of hemolytic uremic syndrome occurred in 13 (54%) patients. Variants in complement genes were detected in 49 (56%) patients, mainly in the CFH (30%) and CFI genes (9%). Pregnancy-associated hemolytic uremic syndrome and atypical hemolytic uremic syndrome nonrelated to pregnancy have the same severity at onset and during follow-up and the same frequency of complement gene variants. Copyright © 2017 by the American Society of Nephrology.

  16. Genetics Home Reference: Coffin-Lowry syndrome

    Science.gov (United States)

    ... in cell signaling pathways that are required for learning, the formation of long-term memories, and the ... RC, Tolmie JL, McWilliam RC, Zuberi SM. The movement disorders of Coffin-Lowry syndrome. Brain Dev. 2005 Mar; ...

  17. Neurally adjusted ventilatory assist (NAVA) mode as an adjunct diagnostic tool in congenital central hypoventilation syndrome

    International Nuclear Information System (INIS)

    Rahmani, A.; Rehman, N.U.; Chedid, F.

    2013-01-01

    A full term female newborn was admitted to the neonatal intensive care unit (NICU) for continuous observation of apnea. Infant was noted to have apnea while asleep requiring intubation and mechanical ventilation. A video EEG was performed which demonstrated normal awake background without any seizure activity. Neurally adjusted ventilatory assist (NAVA) demonstrated the absence of electrical activity of the diaphragm (Edi) when the patient was in quiet phase of sleep. This finding on NAVA monitor raised the suspicion of central hypoventilation syndrome (CCHS) which was confirmed by genetic identification of the PHOX2B mutation. (author)

  18. Neurally adjusted ventilatory assist (NAVA) mode as an adjunct diagnostic tool in congenital central hypoventilation syndrome.

    Science.gov (United States)

    Rahmani, Aiman; Ur Rehman, Naveed; Chedid, Fares

    2013-02-01

    A full term female newborn was admitted to the neonatal intensive care unit (NICU) for continuous observation of apnea. Infant was noted to have apnea while asleep requiring intubation and mechanical ventilation. A video EEG was performed which demonstrated normal awake background without any seizure activity. Neurally adjusted ventilatory assist (NAVA) demonstrated the absence of electrical activity of the diaphragm (Edi) when the patient was in quiet phase of sleep. This finding on NAVA monitor raised the suspicion of central hypoventilation syndrome (CCHS) which was confirmed by genetic identification of the PHOX2B mutation.

  19. Intensity Conserving Spectral Fitting

    Science.gov (United States)

    Klimchuk, J. A.; Patsourakos, S.; Tripathi, D.

    2015-01-01

    The detailed shapes of spectral line profiles provide valuable information about the emitting plasma, especially when the plasma contains an unresolved mixture of velocities, temperatures, and densities. As a result of finite spectral resolution, the intensity measured by a spectrometer is the average intensity across a wavelength bin of non-zero size. It is assigned to the wavelength position at the center of the bin. However, the actual intensity at that discrete position will be different if the profile is curved, as it invariably is. Standard fitting routines (spline, Gaussian, etc.) do not account for this difference, and this can result in significant errors when making sensitive measurements. Detection of asymmetries in solar coronal emission lines is one example. Removal of line blends is another. We have developed an iterative procedure that corrects for this effect. It can be used with any fitting function, but we employ a cubic spline in a new analysis routine called Intensity Conserving Spline Interpolation (ICSI). As the name implies, it conserves the observed intensity within each wavelength bin, which ordinary fits do not. Given the rapid convergence, speed of computation, and ease of use, we suggest that ICSI be made a standard component of the processing pipeline for spectroscopic data.

  20. Analysis of slit-distored small-angle X-ray scattering intensities without desmearing

    Energy Technology Data Exchange (ETDEWEB)

    Goodisman, J.; Delaglio, F.; Brumberger, H.

    1986-08-01

    Experimental small-angle X-ray scattering intensities, generated from a primary beam of known intensity profile, are often ''desmeared'' to obtain point-collimated intensities. A much simpler way is shown of using the known beam intensity profile to derive, from the experimental scattering intensity, the quantities required for calculation of surface areas.

  1. Marfan Syndrome (For Teens)

    Science.gov (United States)

    ... Staying Safe Videos for Educators Search English Español Marfan Syndrome KidsHealth / For Teens / Marfan Syndrome What's in this ... a genetic disorder called Marfan syndrome. What Is Marfan Syndrome? Marfan syndrome is named after Antoine Marfan, the ...

  2. The Source for Syndromes.

    Science.gov (United States)

    Richard, Gail J.; Hoge, Debra Reichert

    Designed for practicing speech-language pathologists, this book discusses different syndrome disabilities, pertinent speech-language characteristics, and goals and strategies to begin intervention efforts at a preschool level. Chapters address: (1) Angelman syndrome; (2) Asperger syndrome; (3) Down syndrome; (4) fetal alcohol syndrome; (5) fetal…

  3. Neurologic manifestations of Angelman syndrome.

    Science.gov (United States)

    Thibert, Ronald L; Larson, Anna M; Hsieh, David T; Raby, Annabel R; Thiele, Elizabeth A

    2013-04-01

    Angelman syndrome is a neurogenetic disorder characterized by the loss or reduction of the ubiquitin-protein ligase E3A enzyme. Angelman syndrome results from a deletion or mutation of the maternally inherited 15q11.2-13.1 region, paternal uniparental disomy of chromosome 15, or an imprinting error. Epilepsy is common and may present with multiple seizure types, including nonconvulsive status epilepticus. Seizures are often intractable and typically require broad-spectrum antiepileptic medications. Dietary therapy has also proved successful in Angelman syndrome. Electroencephalographic patterns include notched δ and rhythmic θ activity and epileptiform discharges. Sleep disorders are also common, often characterized by abnormal sleep-wake cycles. Movement disorders are nearly universal in Angelman syndrome, most frequently presenting with ataxia and tremor. Neurocognitive impairment is always present to varying degrees, and expressive speech is typically severely affected. Individuals with Angelman syndrome often manifest psychiatric comorbidities including hyperactivity, anxiety, and challenging behaviors such as aggression and self-injury. We focus on a comprehensive whole-child approach to the diagnosis and long-term clinical care of individuals with Angelman syndrome. Copyright © 2013 Elsevier Inc. All rights reserved.

  4. The intense neutron generator

    International Nuclear Information System (INIS)

    Lewis, W.B.

    1966-01-01

    The presentation discusses both the economic and research contexts that would be served by producing neutrons in gram quantities at high intensities by electrical means without uranium-235. The revenue from producing radioisotopes is attractive. The array of techniques introduced by the multipurpose 65 megawatt Intense Neutron Generator project includes liquid metal cooling, superconducting magnets for beam bending and focussing, super-conductors for low-loss high-power radiofrequency systems, efficient devices for producing radiofrequency power, plasma physics developments for producing and accelerating hydrogen, ions at high intensity that are still far out from established practice, a multimegawatt high voltage D.C. generating machine that could have several applications. The research fields served relate principally to materials science through neutron-phonon and other quantum interactions as well as through neutron diffraction. Nuclear physics is served through μ-, π- and K-meson production. Isotope production enters many fields of applied research. (author)

  5. The intense world syndrome--an alternative hypothesis for autism

    DEFF Research Database (Denmark)

    Markram, Henry; Rinaldi, Tania; Markram, Kamila

    2007-01-01

    Autism is a devastating neurodevelopmental disorder with a polygenetic predisposition that seems to be triggered by multiple environmental factors during embryonic and/or early postnatal life. While significant advances have been made in identifying the neuronal structures and cells affected......, a unifying theory that could explain the manifold autistic symptoms has still not emerged. Based on recent synaptic, cellular, molecular, microcircuit, and behavioral results obtained with the valproic acid (VPA) rat model of autism, we propose here a unifying hypothesis where the core pathology...... of the autistic brain is hyper-reactivity and hyper-plasticity of local neuronal circuits. Such excessive neuronal processing in circumscribed circuits is suggested to lead to hyper-perception, hyper-attention, and hyper-memory, which may lie at the heart of most autistic symptoms. In this view, the autistic...

  6. Apert syndrome

    Directory of Open Access Journals (Sweden)

    Premalatha

    2010-01-01

    Full Text Available Apert syndrome (acrocephalosyndactyly is a rare developmental malformation characterized by craniosynostosis, mid-face hypoplasia, symmetrical syndactyly of hands and feet. The prodromal characteristics for the typical cranio-facial appearance are early craniosynostosis of the coronal suture, cranial base and agenesis of the sagittal suture. The purpose of this paper is to report a case of Apert syndrome with emphasis on craniofacial and oral features in an eighteen-month-old male child. The patient presented with several craniofacial deformities, including brachycephaly, midface hypoplasia, flat face, hypertelorism, ocular proptosis, downslanting palpebral fissures. Syndactylies with osseous fusion of the hands and feet were also observed. Intraoral findings included delayed eruption of teeth, high arched palate with pseudo cleft in the posterior one third.

  7. Hepatorenal Syndrome

    Directory of Open Access Journals (Sweden)

    Ebru Yilmaz

    2014-06-01

    Full Text Available Hepatorenal syndrome (HRS is functional renal failure that occurs with advanced liver failure. HRS is considered the most severe complication of cirrhosis. Type 1 HRS develops due to severe reduction of effective circulating volume results in hemodynamic dysfunction. Type 1 HRS is characterized by acute renal failure and rapid deterioration in the function of other organs. It can ocur spontaneously or in the setting of a precipitating event. Type 2 hepatorenal syndrome (HRS, which is characterized by slowly progressive renal failure and refractory ascites. Liver transplantation is the only definitive treatment for both type. The most suitable and ldquo;bridge treatments and rdquo; or treatment for patients ineligible for a liver transplant include terlipressin plus albumin. [J Contemp Med 2014; 4(2.000: 106-113

  8. Paraneoplastic syndromes

    Energy Technology Data Exchange (ETDEWEB)

    Weller, R.E.

    1994-03-01

    Paraneoplastic syndromes (PNS) comprise a diverse group of disorders that are associated with cancer but unrelated to the size, location, metastases, or physiologic activities of the mature tissue of origin. They are remote effects of tumors that may appear as signs, symptoms, or syndromes which can mimic other disease conditions encountered in veterinary medicine. Recognition of PNS is valuable for several reasons: the observed abnormalities may represent tumor cell markers and facilitate early diagnosis of the tumor; they may allow assessment of premalignant states; they may aid in the search metastases; they may help quantify and monitor response to therapy; and, they may provide insight into the study of malignant transformation and oncogene expression. This review will concentrate on the pathophysiology, diagnosis, and treatment of some of the common PNS encountered in veterinary medicine.

  9. Pearson syndrome.

    Science.gov (United States)

    Farruggia, Piero; Di Marco, Floriana; Dufour, Carlo

    2018-03-01

    Pearson syndrome (PS) is a sporadic and very rare syndrome classically associated with single large-scale deletions of mitochondrial DNA and characterized by refractory sideroblastic anemia during infancy. Areas covered: This review presents an analysis and interpretation of the published data that forms the basis for our understanding of PS. PubMed, Google Scholarand Thompson ISI Web of Knowledge were searched for relevant data. Expert commentary: PS is a very rare mitochodrial disease that involves different organs and systems. Clinical phenotype is extremely variable and may change over the course of disease itself with the possibility both of worsenings and improvements. Outcome is invariably lethal and at the moment no cure is available. Accurate supportive treatment and follow up program in centres with experience in mitochondrial diseases and marrow failure may positively influence quality and duration of life.

  10. Otodental syndrome

    Directory of Open Access Journals (Sweden)

    Bloch-Zupan Agnès

    2006-03-01

    Full Text Available Abstract The otodental syndrome also named otodental dysplasia, is characterised by a striking dental phenotype known as globodontia, associated with sensorineural high frequency hearing loss and eye coloboma. Globodontia occurs in both primary and permanent dentition, affecting canine and molar teeth (i.e. enlarged bulbous malformed posterior teeth with almost no discernable cusps or grooves. The condition appears to be inherited in an autosomal dominant mode, although sporadic cases have been reported. It is a rare disease, a few families have been described in the literature. In the British family, the locus for oculo-oto-dental syndrome was mapped to 20q13.1 within a 12-cM critical chromosomal region. Dental management is complex, interdisciplinary and will include regular follow up, scheduled teeth extraction and orthodontic treatment. Hearing checks and, if necessary, hearing aids are mandatory, as well as eye examination and ad hoc treatment if necessary.

  11. Antiphospholipid syndrome.

    Science.gov (United States)

    Cervera, Ricard

    2017-03-01

    The antiphospholipid syndrome (APS) is defined by the development of venous and/or arterial thromboses, often multiple, and pregnancy morbidity (mainly, recurrent fetal losses), in the presence of antiphospholipid antibodies (aPL). Some estimates indicate that the incidence of the APS is around 5 new cases per 100,000 persons per year and the prevalence around 40-50 cases per 100,000 persons. The aPL are positive in approximately 13% of patients with stroke, 11% with myocardial infarction, 9.5% of patients with deep vein thrombosis and 6% of patients with pregnancy morbidity. Currently, there is consensus in treating APS patients with thrombosis with long-term oral anticoagulation and to prevent obstetric manifestations with the use of aspirin and heparin. This review summarizes the main knowledge on the clinical and therapeutic aspects of this syndrome. © 2017 Elsevier Ltd. All rights reserved.

  12. Dravet syndrome

    Directory of Open Access Journals (Sweden)

    Incorpora Gemma

    2009-09-01

    Full Text Available Abstract "Dravet syndrome" (DS previously named severe myoclonic epilepsy of infancy (SMEI, or epilepsy with polymorphic seizures, is a rare disorder characterized by an early, severe, generalized, epileptic encephalopathy. DS is characterized by febrile and afebrile seizures beginning in the 1st year of life followed by different types of seizures (either focal or generalized, which are typically resistant to antiepileptic drugs. A developmental delay from the 2nd to 3rd year of life becomes evident, together with motor disturbances and personality disorders. Beside the classic syndrome, there are milder cases which have been called severe myoclonic epilepsy borderline (SMEB. DS is caused by a mutation in the neuronal sodium channel gene, SCN1A , that is also mutated in generalized epilepsy with FS+ (GEFS+.

  13. Sinding-Larsen-Johansson syndrome

    African Journals Online (AJOL)

    dislocation, chondromalacia patellae and prepatellar bursitis.1. Imaging of Sinding-Larsen-Johansson syndrome may require a combination of radiographs, MRI and ultrasound. Lateral radiographs may reveal peripatellar soft-tissue swelling, patella-alta deformity and one or multiple tiny osseous fragments adjacent to.

  14. [Fibromyalgia syndrome].

    Science.gov (United States)

    Naranjo Hernández, A; Rodríguez Lozano, C; Ojeda Bruno, S

    1992-02-01

    The Fibromialgia Syndrome (FS) is a common clinical entity which may produce symtoms and signs related to multiple fields of Medicine. Typical clinical characteristics of FS include extensive pain, presence of sensitive points during exploration, morning stiffness, asthenia and non-refresing sleep. Frequently, associated rheumatologic diseases are observed, as rheumatoid arthritis, osteoarthrosis and vertebral disorders. In FS, complementary tests are usually normal. The most widely accepted hypothesis suggests that this is a disorder affecting modulation of pain sensitivity.

  15. Griscelli syndrome

    Directory of Open Access Journals (Sweden)

    Kumar T

    2006-01-01

    Full Text Available Partial albinism with immunodeficiency is a rare and fatal immunologic disorder characterized by pigmentary dilution and variable cellular immunodeficiency. It was initially described in 1978. Primary abnormalities included silvery grayish sheen to the hair, large pigment agglomerations in hair shafts and an abundance of mature melanosomes in melanocytes, with reduced pigmentation of adjacent keratinocytes. We describe a child with Griscelli syndrome who presented with hepatitis, pancytopenia and silvery hair. The diagnosis was confirmed by microscopic skin and hair examination.

  16. Asperger Syndrome

    OpenAIRE

    Friedlander, Robin

    2002-01-01

    Abstract Asperger syndrome (AS) is a chronic neurodevelopmental disorder of social interaction, communication, and a restricted range of behaviors or interests. Although not generally associated with intellectual disability, the severe social disability and, in many cases, associated mental health and other medical problems, result in disability throughout life. The diagnosis is often delayed, sometimes into adulthood, which is unfortunate because there are now a range...

  17. Caregiver strain and posttraumatic stress symptoms of informal caregivers of intensive care unit survivors

    NARCIS (Netherlands)

    van den Born-van Zanten, Sascha A.; Dongelmans, Dave A.; Dettling-Ihnenfeldt, Daniela; Vink, Roel; van der Schaaf, Marike

    BACKGROUND: Many intensive care unit survivors (ICU) are confronted with undesirable and long-lasting impairments in physical, cognitive, and mental health, but not only patients are at risk of developing this post-intensive care syndrome (PICS). Family members can experience symptoms of depression,

  18. Caregiver strain and posttraumatic stress symptoms of informal caregivers of intensive care unit survivors

    NARCIS (Netherlands)

    van den Born-van Zanten, Sascha A.; Dongelmans, Dave A.; Dettling-Ihnenfeldt, Daniela; Vink, Roel; van der Schaaf, Marike

    2016-01-01

    Many intensive care unit survivors (ICU) are confronted with undesirable and long-lasting impairments in physical, cognitive, and mental health, but not only patients are at risk of developing this post-intensive care syndrome (PICS). Family members can experience symptoms of depression, anxiety,

  19. Endometriosis, a modern syndrome

    Science.gov (United States)

    Brosens, Ivo; Benagiano, Giuseppe

    2011-01-01

    The identification of endometriosis has been a subject of intense debate over the last decade. There is, however, no doubt that Thomas Cullen was the first to describe endometriosis and adenomyosis as one disease characterized by the presence of endometrium-like tissue outside the uterine cavity. With the introduction of laparoscopy in the early 1960s three different clinical presentations of endometriosis were distinguished: peritoneal, deep adenomyotic and cystic ovarian. As soon as synthetic steroids became available, pioneer clinicians started utilizing these in an attempt to replace radical surgery by a medical treatment. While medical therapy may resort in relief, in most cases the current approach consists of a combination of medical and surgical therapy. While the pathogenesis of endometriosis is still enigmatic and complex, there is increasing evidence that endometriosis is part of a uterine reproductive dysfunction syndrome. For prevention of complications, it is very important that diagnosis is made as early as possible in a woman's life. PMID:21727656

  20. Acute myocardial infarction in young adults with Antiphospholipid syndrome: report of two cases and literature review

    OpenAIRE

    Abid, Leila; Frikha, Faten; Bahloul, Zouhir; Kammoun, Samir

    2011-01-01

    Abstract Acute myocardial infarction (AMI) is rarely associated with antiphospholipid syndrome. The treatment of these patients is a clinical challenge. We report the observations of 2 young adults (1 woman and 1 man), admitted in our acute care unit for acute myocardial infarction (AMI). A coagulopathy work-up concludes the existence of antiphospholipid syndrome (APS) in the 2 cases. APS syndrome was considered primary in 2 cases. All patients presented an intense inflammatory syndrome (high...

  1. Rett Syndrome: A Longitudinal Developmental Case Report.

    Science.gov (United States)

    Garber, Norman; Veydt, Nicole

    1990-01-01

    The clinical course of development of a 14-year-old girl with Rett Syndrome is outlined. Results indicated a general stagnation in gross and fine motor skills, self-help skills, communication, and cognition, beginning at approximately 15 months. No skills progressed beyond the 2-year level despite several years of intensive intervention.…

  2. What Is Marfan Syndrome?

    Science.gov (United States)

    ... Outreach Initiative Breadcrumb Home Health Topics English Español Marfan Syndrome Basics In-Depth Download Download EPUB Download PDF What is it? Points To Remember About Marfan Syndrome Marfan syndrome affects connective tissue, which is the “ ...

  3. Turner Syndrome: Other FAQs

    Science.gov (United States)

    ... celiac disease. 5 Is Turner syndrome considered a disability? Turner syndrome is not considered a disability, although ... girls with Turner syndrome have difficulty with arithmetic, visual memory, and visio-spatial skills (such as determining ...

  4. Basal cell nevus syndrome

    Science.gov (United States)

    ... nevus syndrome Basal cell nevus syndrome - face References Evans DG, Farndon PA. Nevoid basal cell carcinoma syndrome. ... A.D.A.M. follows rigorous standards of quality and accountability. A.D.A.M. is among ...

  5. What Causes Rett Syndrome?

    Science.gov (United States)

    ... it? Share Facebook Twitter Pinterest Email Print What causes Rett syndrome? Most cases of Rett syndrome are ... in the MECP2 gene represent the most prevalent causes of Rett syndrome. The development and severity of ...

  6. Toxic shock syndrome

    Science.gov (United States)

    Staphylococcal toxic shock syndrome; Toxic shock-like syndrome; TSLS ... Toxic shock syndrome is caused by a toxin produced by some types of staphylococcus bacteria. A similar problem, called toxic shock- ...

  7. Neonatal respiratory distress syndrome

    Science.gov (United States)

    Hyaline membrane disease (HMD); Infant respiratory distress syndrome; Respiratory distress syndrome in infants; RDS - infants ... after that. Some infants with severe respiratory distress syndrome will die. This most often occurs between days ...

  8. Nutrition support in the intensive care unit: an evolving science.

    Science.gov (United States)

    Blackburn, George L; Wollner, Samuel; Bistrian, Bruce R

    2010-06-01

    Recent investigations of nutrition support in the intensive care unit (ICU) have revived discussion of optimal strategies for tight glucose control and the administration of total parenteral nutrition. Mode, timing, and adequacy of nutritional support affect glycemic control and outcomes in critically ill patients. The delivery of correctly formulated and safely administered nutritional and metabolic support is a matter of life or death in surgical and critical care units. High-quality research, adequately powered to detect differences in clinically meaningful outcomes, is needed to inform the delivery of nutrition support and serve as the foundation for future clinical trials. These are issues that will need to be addressed in the months and years ahead. Today, the field is in the midst of challenges and change, and though much has been accomplished, much remains to be done. The last 30 years have seen radical changes in the rates of severe obesity, metabolic syndrome, and weight loss surgery. Obese patients heighten surgical risk and require extra caution by ICU nutrition support specialists. This commentary will address the direction of nutrition support services by covering the history, progress, and potential of the field. It will review parenteral nutrition from its inception to its current standing in ICU patient care and discuss the future role of parenteral nutrition in a rapidly changing and increasingly diverse population.

  9. 99mTc-HIDA cholescintigraphy in Dubin-Johnson and Rotor syndromes

    International Nuclear Information System (INIS)

    Bar-Meir, S.; Baron, J.; Seligson, U.; Gottesfeld, F.; Levy, R.; Gilat, T.

    1982-01-01

    99mTc-HIDA cholescintigraphy was performed in 6 patients with Dubin-Johnson syndrome and 1 patient with Rotor syndrome. In the patients with Dubin-Johnson syndrome, the cholescintigrams had a characteristic pattern of delayed visualization or nonvisualization of the gallbladder and bile ducts in the presence of intense, homogeneous, and prolonged visualization of the liver. In the patient with Rotor syndrome, the hepatobiliary system was not visualized at all. It is concluded that 99mTc-HIDA cholescintigraphy may be helpful in the diagnosis of Dubin-Johnson syndrome and Rotor syndrome and in the differential diagnosis between these two conditions

  10. /sup 99m/Tc-HIDA cholescintigraphy in Dubin-Johnson and Rotor syndromes

    International Nuclear Information System (INIS)

    Bar-Meir; Baron, J.; Seligson, U.; Gottesfeld, F.; Levy, R.; Gilat, T.

    1982-01-01

    /sup 99m/Tc-HIDA cholescintigraphy was performed in 6 patients with Dubin-Johnson syndrome and 1 patient with Rotor syndrome. In the patients with Dubin-Johnson syndrome, the cholescintigrams had a characteristic pattern of delayed visualization or nonvisualization of the gallbladder and bile ducts in the presence of intense, homogeneous, and prolonged visualization of the liver. In the patient with Rotor syndrome, the hepatobiliary system was not visualized at all. It is concluded that /sup 99m/Tc-HIDA cholescintigraphy may be helpful in the diagnosis of Dubin-Johnson syndrome and Rotor syndrome and in the differential diagnosis between these two conditions

  11. Congenital Short QT Syndrome

    Directory of Open Access Journals (Sweden)

    Charles Antzelevitch

    2004-04-01

    Full Text Available Long QT intervals in the ECG have long been associated with sudden cardiac death. The congenital long QT syndrome was first described in individuals with structurally normal hearts in 1957.1 Little was known about the significance of a short QT interval. In 1993, after analyzing 6693 consecutive Holter recordings Algra et al concluded that an increased risk of sudden death was present not only in patients with long QT interval, but also in patients with short QT interval (<400 ms.2 Because this was a retrospective analysis, further evaluation of the data was not possible. It was not until 2000 that a short-QT syndrome (SQTS was proposed as a new inherited clinical syndrome by Gussak et al.3 The initial report was of two siblings and their mother all of whom displayed persistently short QT interval. The youngest was a 17 year old female presenting with several episodes of paroxysmal atrial fibrillation requiring electrical cardioversion.3 Her QT interval measured 280 msec at a heart rate of 69. Her 21 year old brother displayed a QT interval of 272 msec at a heart rate of 58, whereas the 51 year old mother showed a QT of 260 msec at a heart rate of 74. The authors also noted similar ECG findings in another unrelated 37 year old patient associated with sudden cardiac death.

  12. Stochastic conditional intensity processes

    DEFF Research Database (Denmark)

    Bauwens, Luc; Hautsch, Nikolaus

    2006-01-01

    model allows for a wide range of (cross-)autocorrelation structures in multivariate point processes. The model is estimated by simulated maximum likelihood (SML) using the efficient importance sampling (EIS) technique. By modeling price intensities based on NYSE trading, we provide significant evidence...

  13. Intensity (a.u)

    Indian Academy of Sciences (India)

    Administrator

    Supplementary Information. Fig. S1. Powder X-ray diffraction pattern (Cu Kα) for AlPO-5: (a) as prepared, (b) calcined and (c) simulated [Ref. 27]. 10. 20. 30. 40. 50. Intensity (a.u) b c a. 2θ ...

  14. Measuring Physical Activity Intensity

    Medline Plus

    Full Text Available ... CDC’s Example StairWELL Stairwell Appearance Motivational Signs Installing Music Other Ideas to Consider Tracking Stair Usage Project ... an activity. When using relative intensity, people pay attention to how physical activity affects their heart rate ...

  15. Measuring Physical Activity Intensity

    Medline Plus

    Full Text Available ... Updates To receive email updates about this page, enter your email address: Enter Email Address What's this? Submit What's this? Submit ... Intensity Walking briskly (3 miles per hour or faster, but not race-walking) Water aerobics Bicycling slower ...

  16. Measuring Physical Activity Intensity

    Medline Plus

    Full Text Available ... upon the amount of energy used by the body while doing the activity. Top of Page Moderate Intensity Walking briskly (3 miles per hour or faster, but not race-walking) Water aerobics Bicycling slower than 10 miles per hour ...

  17. Measuring Physical Activity Intensity

    Medline Plus

    Full Text Available ... gov . Physical Activity Physical Activity Basics Needs for Adults Needs for Children What Counts Needs for Older Adults Needs for ... Adding Physical Activity to Your Life Activities for Children Activities for Older Adults Overcoming Barriers Measuring Physical Activity Intensity Target Heart ...

  18. Measuring Physical Activity Intensity

    Medline Plus

    Full Text Available ... Stair Usage Project Checklist CDC’s Example Related Resources Walking Step It Up! Surgeon General’s Call to Action ... doing the activity. Top of Page Moderate Intensity Walking briskly (3 miles per hour or faster, but ...

  19. Measuring Physical Activity Intensity

    Medline Plus

    Full Text Available ... Obesity Healthy Weight Breastfeeding Micronutrient Malnutrition State and Local Programs Measuring Physical Activity Intensity Recommend on Facebook Tweet Share Compartir For more help with what counts as aerobic activity, watch this video: Windows Media Player, 4:48 More videos Here are some ...

  20. Exercise training dose differentially alters muscle and heart capillary density and metabolic functions in an obese rat with metabolic syndrome.

    Science.gov (United States)

    Machado, Marcus Vinicius; Vieira, Aline Bomfim; da Conceição, Fabiana Gomes; Nascimento, Alessandro Rodrigues; da Nóbrega, Antonio Claudio Lucas; Tibirica, Eduardo

    2017-12-01

    What is the central question of this study? Regular exercise is recommended as a non-pharmacological approach for the prevention and treatment of metabolic syndrome. However, the impact of different combinations of intensity, duration and frequency of exercise on metabolic syndrome and microvascular density has not been reported. What is the main finding and its importance? We provide evidence on the impact of aerobic exercise dose on metabolic and microvascular alterations in an experimental model of metabolic syndrome induced by high-fat diet. We found that the exercise frequency and duration were the main factors affecting anthropometric and metabolic parameters and microvascular density in the skeletal muscle. Exercise intensity was related only to microvascular density in the heart. We evaluated the effect of the frequency, duration and intensity of exercise training on metabolic parameters and structural capillary density in obese rats with metabolic syndrome. Wistar-Kyoto rats were fed either a standard commercial diet (CON) or a high-fat diet (HFD). Animals that received the HFD were randomly separated into either a sedentary (SED) group or eight different exercise groups that varied according to the frequency, duration and intensity of training. After 12 weeks of aerobic exercise training, the body composition, aerobic capacity, haemodynamic variables, metabolic parameters and capillary density in the heart and skeletal muscle were evaluated. All the exercise training groups showed reduced resting systolic blood pressure and heart rate and normalized fasting glucose. The minimal amount of exercise (90 min per week) produced little effect on metabolic syndrome parameters. A moderate amount of exercise (150 min per week) was required to reduce body weight and improve capillary density. However, only the high amount of exercise (300 min per week) significantly reduced the amount of body fat depots. The three-way ANOVA showed a main effect of exercise

  1. Genetics Home Reference: antiphospholipid syndrome

    Science.gov (United States)

    ... Share: Email Facebook Twitter Home Health Conditions Antiphospholipid syndrome Antiphospholipid syndrome Printable PDF Open All Close All Enable ... area? Other Names for This Condition anti-phospholipid syndrome antiphospholipid antibody syndrome Hughes syndrome Related Information How are ...

  2. Tarsaltunnel syndrome - MRI diagnosis

    International Nuclear Information System (INIS)

    Trattnig, S.; Helbich, T.; Imhof, H.

    1995-01-01

    Clinical findings and symptoms of tarsal tunnel are commonly vague and diffuse and electrodiagnostic studies do not provide definitive diagnosis. MR imaging with its excellent soft tissue contrast can demonstrate clearly the anatomy of the tarsal tunnel and its contents. MRI is able to demonstrate a space-occypyinglesion and its relationship to the posterior tibial nerve and its branches. This information aids in surgical planning by determining the extent of the decompression required. MR imaging may also be used to follow up non-surgical causes of tarsal tunnel syndrome such as tenosynovitis. (orig.) [de

  3. Peutz–Jeghers Syndrome

    OpenAIRE

    Parray, Fazl Qadir; Syed, Abdul Wahid; Yatoo, Ghulam Nabi; Zargar, Showkat Ali; Malik, Rayees Ahmad

    2012-01-01

    Peutz-Jeghers Syndrome (PJS) is a rare condition that tends to run in families. Diagnosis of PJS is made if a person has polyps in the gastrointestinal (GI) tract and at least two of the following: polyps in the small bowel, melanin spots, and/or a family history of PJS. The typical clinical manifestation of the disease is associated with complications secondary to intestinal polyps often requiring surgical treatment. A young woman, 29 years old with PJS had been hospitalized in Cipto Mangunk...

  4. Tics and Tourette syndrome.

    Science.gov (United States)

    Shaw, Zoey A; Coffey, Barbara J

    2014-09-01

    Tourette syndrome is a childhood onset neurodevelopmental disorder characterized by multiple motor and vocal tics. Although many youth experience attenuation or even remission of tics in adolescence and young adulthood, some individuals experience persistent tics, which can be debilitating or disabling. Most patients also have 1 or more psychiatric comorbid disorders, such as attention-deficit/hyperactivity disorder or obsessive-compulsive disorder. Treatment is multimodal, including both pharmacotherapy and cognitive-behavioral treatment, and requires disentanglement of tics and the comorbid symptoms. Copyright © 2014 Elsevier Inc. All rights reserved.

  5. Griscelli syndrome type-3

    Directory of Open Access Journals (Sweden)

    Bela J Shah

    2016-01-01

    Full Text Available Griscelli syndrome (GS is a rare autosomal recessive multisystem disorder of pigmentary dilution of skin, silver gray hair, variable immunodeficiency, neurological impairment, and abnormal accumulation of melanosomes in melanocytes. GS type 3 is characterized by hypomelanosis with no immunological and neurological manifestation. Prognosis is very good in type 3 GS and usually require no active intervention, as opposed to type 1 and 2 where early diagnosis and treatment plays a crucial role in patient's survival. The characteristic phenotypic appearance, especially the pigment dilution of the patient's hair, is emphasized here.

  6. The cannabis withdrawal syndrome: current insights

    Directory of Open Access Journals (Sweden)

    Bonnet U

    2017-04-01

    Full Text Available Udo Bonnet,1,2 Ulrich W Preuss3,4 1Department of Psychiatry, Psychotherapy and Psychosomatic Medicine, Evangelisches Krankenhaus Castrop-Rauxel, Academic Teaching Hospital of the University of Duisburg-Essen, Castrop-Rauxel, 2Department of Psychiatry and Psychotherapy, Faculty of Medicine, LVR-Hospital Essen, University of Duisburg-Essen, Essen, 3Vitos-Klinik Psychiatrie und Psychotherapie Herborn, Herborn, 4Martin Luther University Halle-Wittenberg, Halle (Saale, Germany Abstract: The cannabis withdrawal syndrome (CWS is a criterion of cannabis use disorders (CUDs (Diagnostic and Statistical Manual of Mental Disorders – Fifth Edition and cannabis dependence (International Classification of Diseases [ICD]-10. Several lines of evidence from animal and human studies indicate that cessation from long-term and regular cannabis use precipitates a specific withdrawal syndrome with mainly mood and behavioral symptoms of light to moderate intensity, which can usually be treated in an outpatient setting. Regular cannabis intake is related to a desensitization and downregulation of human brain cannabinoid 1 (CB1 receptors. This starts to reverse within the first 2 days of abstinence and the receptors return to normal functioning within 4 weeks of abstinence, which could constitute a neurobiological time frame for the duration of CWS, not taking into account cellular and synaptic long-term neuroplasticity elicited by long-term cannabis use before cessation, for example, being possibly responsible for cannabis craving. The CWS severity is dependent on the amount of cannabis used pre-cessation, gender, and heritable and several environmental factors. Therefore, naturalistic severity of CWS highly varies. Women reported a stronger CWS than men including physical symptoms, such as nausea and stomach pain. Comorbidity with mental or somatic disorders, severe CUD, and low social functioning may require an inpatient treatment (preferably qualified detox and

  7. The cannabis withdrawal syndrome: current insights

    Science.gov (United States)

    Bonnet, Udo; Preuss, Ulrich W

    2017-01-01

    The cannabis withdrawal syndrome (CWS) is a criterion of cannabis use disorders (CUDs) (Diagnostic and Statistical Manual of Mental Disorders – Fifth Edition) and cannabis dependence (International Classification of Diseases [ICD]-10). Several lines of evidence from animal and human studies indicate that cessation from long-term and regular cannabis use precipitates a specific withdrawal syndrome with mainly mood and behavioral symptoms of light to moderate intensity, which can usually be treated in an outpatient setting. Regular cannabis intake is related to a desensitization and downregulation of human brain cannabinoid 1 (CB1) receptors. This starts to reverse within the first 2 days of abstinence and the receptors return to normal functioning within 4 weeks of abstinence, which could constitute a neurobiological time frame for the duration of CWS, not taking into account cellular and synaptic long-term neuroplasticity elicited by long-term cannabis use before cessation, for example, being possibly responsible for cannabis craving. The CWS severity is dependent on the amount of cannabis used pre-cessation, gender, and heritable and several environmental factors. Therefore, naturalistic severity of CWS highly varies. Women reported a stronger CWS than men including physical symptoms, such as nausea and stomach pain. Comorbidity with mental or somatic disorders, severe CUD, and low social functioning may require an inpatient treatment (preferably qualified detox) and post-acute rehabilitation. There are promising results with gabapentin and delta-9-tetrahydrocannabinol analogs in the treatment of CWS. Mirtazapine can be beneficial to treat CWS insomnia. According to small studies, venlafaxine can worsen the CWS, whereas other antidepressants, atomoxetine, lithium, buspirone, and divalproex had no relevant effect. Certainly, further research is required with respect to the impact of the CWS treatment setting on long-term CUD prognosis and with respect to

  8. The cannabis withdrawal syndrome: current insights.

    Science.gov (United States)

    Bonnet, Udo; Preuss, Ulrich W

    2017-01-01

    The cannabis withdrawal syndrome (CWS) is a criterion of cannabis use disorders (CUDs) ( Diagnostic and Statistical Manual of Mental Disorders - Fifth Edition ) and cannabis dependence (International Classification of Diseases [ICD]-10). Several lines of evidence from animal and human studies indicate that cessation from long-term and regular cannabis use precipitates a specific withdrawal syndrome with mainly mood and behavioral symptoms of light to moderate intensity, which can usually be treated in an outpatient setting. Regular cannabis intake is related to a desensitization and downregulation of human brain cannabinoid 1 (CB1) receptors. This starts to reverse within the first 2 days of abstinence and the receptors return to normal functioning within 4 weeks of abstinence, which could constitute a neurobiological time frame for the duration of CWS, not taking into account cellular and synaptic long-term neuroplasticity elicited by long-term cannabis use before cessation, for example, being possibly responsible for cannabis craving. The CWS severity is dependent on the amount of cannabis used pre-cessation, gender, and heritable and several environmental factors. Therefore, naturalistic severity of CWS highly varies. Women reported a stronger CWS than men including physical symptoms, such as nausea and stomach pain. Comorbidity with mental or somatic disorders, severe CUD, and low social functioning may require an inpatient treatment (preferably qualified detox) and post-acute rehabilitation. There are promising results with gabapentin and delta-9-tetrahydrocannabinol analogs in the treatment of CWS. Mirtazapine can be beneficial to treat CWS insomnia. According to small studies, venlafaxine can worsen the CWS, whereas other antidepressants, atomoxetine, lithium, buspirone, and divalproex had no relevant effect. Certainly, further research is required with respect to the impact of the CWS treatment setting on long-term CUD prognosis and with respect to

  9. [Quality assurance concepts in intensive care medicine].

    Science.gov (United States)

    Brinkmann, A; Braun, J P; Riessen, R; Dubb, R; Kaltwasser, A; Bingold, T M

    2015-11-01

    Intensive care medicine (ICM) is characterized by a high degree of complexity and requires intense communication and collaboration on interdisciplinary and multiprofessional levels. In order to achieve good quality of care in this environment and to prevent errors, a proactive quality and error management as well as a structured quality assurance system are essential. Since the early 1990s, German intensive care societies have developed concepts for quality management and assurance in ICM. In 2006, intensive care networks were founded in different states to support the implementation of evidence-based knowledge into clinical routine and to improve medical outcome, efficacy, and efficiency in ICM. Current instruments and concepts of quality assurance in German ICM include core intensive care data from the data registry DIVI REVERSI, quality indicators, peer review in intensive care, IQM peer review, and various certification processes. The first version of German ICM quality indicators was published in 2010 by an interdisciplinary and interprofessional expert commission. Key figures, indicators, and national benchmarks are intended to describe the quality of structures, processes, and outcomes in intensive care. Many of the quality assurance tools have proved to be useful in clinical practice, but nationwide implementation still can be improved.

  10. The trisomy 18 syndrome

    Directory of Open Access Journals (Sweden)

    Cereda Anna

    2012-10-01

    Full Text Available Abstract The trisomy 18 syndrome, also known as Edwards syndrome, is a common chromosomal disorder due to the presence of an extra chromosome 18, either full, mosaic trisomy, or partial trisomy 18q. The condition is the second most common autosomal trisomy syndrome after trisomy 21. The live born prevalence is estimated as 1/6,000-1/8,000, but the overall prevalence is higher (1/2500-1/2600 due to the high frequency of fetal loss and pregnancy termination after prenatal diagnosis. The prevalence of trisomy 18 rises with the increasing maternal age. The recurrence risk for a family with a child with full trisomy 18 is about 1%. Currently most cases of trisomy 18 are prenatally diagnosed, based on screening by maternal age, maternal serum marker screening, or detection of sonographic abnormalities (e.g., increased nuchal translucency thickness, growth retardation, choroid plexus cyst, overlapping of fingers, and congenital heart defects . The recognizable syndrome pattern consists of major and minor anomalies, prenatal and postnatal growth deficiency, an increased risk of neonatal and infant mortality, and marked psychomotor and cognitive disability. Typical minor anomalies include characteristic craniofacial features, clenched fist with overriding fingers, small fingernails, underdeveloped thumbs, and short sternum. The presence of major malformations is common, and the most frequent are heart and kidney anomalies. Feeding problems occur consistently and may require enteral nutrition. Despite the well known infant mortality, approximately 50% of babies with trisomy 18 live longer than 1 week and about 5-10% of children beyond the first year. The major causes of death include central apnea, cardiac failure due to cardiac malformations, respiratory insufficiency due to hypoventilation, aspiration, or upper airway obstruction and, likely, the combination of these and other factors (including decisions regarding aggressive care. Upper airway

  11. Angiosarcoma in previously irradiated breast in patient with Li-Fraumeni syndrome. A case report

    Directory of Open Access Journals (Sweden)

    Oséias Vargas Barbosa

    Full Text Available CONTEXT: Li-Fraumeni syndrome is a rare disease with an autosomal dominant inheritance pattern and high penetrance that defines a 50% chance of developing cancer before the age of 30 years, including cases of breast sarcoma. Patients with this syndrome who require radiotherapy have an increased risk of developing secondary malignancies including angiosarcomas. CASE REPORT: This was a case report on a female patient with Li-Fraumeni syndrome. In October 2005, she was diagnosed with invasive ductal carcinoma of the right breast and underwent sectorectomy. She then received chemotherapy and adjuvant radiotherapy. Trastuzumab and tamoxifen were also part of the treatment. She recently sought care at our hospital, complaining of hyperemia and nodulation in the right breast, and underwent surgical resection that revealed epithelioid angiosarcoma. CONCLUSIONS: When genetic predisposition due to Li-Fraumeni syndrome is documented, the therapy should be adapted so as to minimize the risk. Thus, conservative surgical treatments should be avoided and mastectomy without radiation should be prioritized. In cases in which use of radiotherapy is justified, patients should be followed up intensively.

  12. Postprandial hypoglycemic syndrome

    Directory of Open Access Journals (Sweden)

    Т.V. Chaychenko

    2017-03-01

    Full Text Available Postprandial hypoglycemic syndrome, or reactive hypoglycemia, — vegetative symptoms, such as weakness, fatigue, hunger, nausea, palpitations, anxiety, tremor, sweating occurring one to two hours after ingestion. The syndrome is poorly described in literature and most of the information is disparate. Laboratory criteria for the diagnosis of postprandial reactive hypoglycemia are quite controversial, but most authors tend to consider that it is a blood glucose level, which is below 3.9 mmol/l for two hours after meal. Hypoglycemia is an unbalance between glucose influx to the circulation (from endogenous glucose production or exogenous glucose delivery and glucose efflux. The balance between glucose intake and consumption is controlled by a complex balance of glycoregulatory hormones. Insulin, glucagon and adrenaline are effective for several minutes, but cortisol and growth hormone — for seve-ral hours. This explains the presence of immediate and delayed various effects: adrenergic, neuroglycopenic ones and gastroin-testinal discomfort. Postprandial syndrome mechanisms are similar to post-gastric bypass patients with morbid obesity. The most likely cause of reactive hypoglycemia is post-prandial hypersecretion of insulin under the influence of glucose and glucagon-like peptide-1 (GLP-1, which is a component of the enteroendocrine system and acts at the cephalic phase of satiety. Both post-gastric bypass and relatively healthy individuals have symptoms after the meal rich of simple carbohydrates. Symptoms could be effectively reduced by low glycemic index diet rich of dietary fibers. When the effect is insufficient, it is recommended to use acarbose as an α-glucosidase inhibitor, which is the main stimulation of GLP-1 secretion. Thus, obesity epidemics based on the inadequate nutritional habits in the children makes the postprandial syndrome feasible, and it requires further studies. At the same time, healthy diet can significantly improve

  13. Inherited ichthyosis: Syndromic forms.

    Science.gov (United States)

    Yoneda, Kozo

    2016-03-01

    Among diseases that cause ichthyosis as one of the symptoms, there are some diseases that induce abnormalities in organs other than the skin. Of these, diseases with characteristic signs are regarded as syndromes. Although these syndromes are very rare, Netherton syndrome, Sjögren-Larsson syndrome, Conradi-Hünermann-Happle syndrome, Dorfman-Chanarin syndrome, ichthyosis follicularis, atrichia and photophobia (IFAP) syndrome, and Refsum syndrome have been described in texts as representative ones. It is important to know the molecular genetics and pathomechanisms in order to establish an effective therapy and beneficial genetic counseling including a prenatal diagnosis. © 2016 Japanese Dermatological Association.

  14. Marfan's Syndrome: Detection and Management.

    Science.gov (United States)

    Cantwell, J D

    1986-07-01

    In brief: Marian's syndrome has gained increased attention and interest recently, more so since the death of volleyball star Flo Hyman last January. The disorder was first described by French pediatrician Antoine Bernard-Jean Marfan in 1896, although it may not have been the same syndrome as we know it today. Current thought suggests a defect in collagen-the fibrous material found in bones, joints, eyes, heart, and blood vessels. Positive diagnosis requires the presence of at least two of four major features: a positive family history and ocular, cardiac, and skeletal abnormalities. This article reviews the disease and discusses methods of detection and management.

  15. Cytomegalovirus infection can mimic genetic nephrotic syndrome: a case report.

    Science.gov (United States)

    Hogan, Julien; Fila, Marc; Baudouin, Véronique; Peuchmaur, Michel; Deschênes, Georges; Niel, Olivier

    2015-09-22

    Nephrotic syndrome is a relatively rare but serious condition in children. Infantile nephrotic syndrome often has a genetic origin; the treatment is then symptomatic, with a poor prognosis, and a rapid evolution to chronic kidney disease. However, non-genetic infantile nephrotic syndrome has been identified. Here we report for the first time in a child a nephrotic syndrome as the sole clinical expression of a cytomegalovirus infection. The patient was 5 months old when he presented with a nephrotic syndrome. An exhaustive genetic testing was conducted and came back negative. A viral work-up only showed a positive cytomegalovirus PCR. Antiviral treatment lead to a complete remission of the nephrotic syndrome, with no requirement for steroid therapy. Renal function remained normal throughout follow-up. Nephrotic syndrome should always be carefully investigated in children. This observation reinforces the connection between viral infections and pediatric nephrotic syndrome, sparking more controversy about an infectious origin to childhood nephrotic disease.

  16. Lemierre’s Syndrome: A Neglected Disease with Classical Features

    Directory of Open Access Journals (Sweden)

    Andreas V. Hadjinicolaou

    2015-01-01

    Full Text Available We report the case of a previously healthy, immunocompetent 23-year-old male who presented to the Emergency Department with general malaise, difficulty in breathing, fever, and chest pain. He reported a two-week history of progressively worsening sore throat that he presumed to be a viral infection and thus initially neglected. However, when his condition deteriorated, he was admitted to hospital acutely unwell and in respiratory distress. He quickly developed septic shock requiring intensive care admission for inotropic support. Ultrasound and CT imaging revealed internal jugular vein thrombosis with associated septic emboli reaching the lungs to form bilateral cavitations and consequently pleural effusions. Blood cultures were positive for Fusobacterium necrophorum. Based on these findings, a diagnosis of Lemierre’s syndrome was made. The patient was treated with appropriate antibiotics and anticoagulation and gradually recovered. He was discharged 20 days after admission with advice to complete a six-week course of antibiotics.

  17. A Writing-Intensive Statistics Course

    Science.gov (United States)

    Cline, Kelly S.

    2008-01-01

    We discuss an upper division applied statistics course that has been structured to satisfy our college's writing intensive requirements within the mathematics major. In this course, students complete two projects, performing both a statistical survey and a controlled experiment, and then write up their work on each project in a formal paper. After…

  18. AGS intensity record

    International Nuclear Information System (INIS)

    Bleser, Ed

    1994-01-01

    As flashed in the September issue, this summer the Brookhaven Alternating Gradient Synchrotron (AGS) reached a proton beam intensity of 4.05 x 10 13 protons per puise, claimed as the highest intensity ever achieved in a proton synchrotron. It is, however, only two-thirds of the way to its final goal of 6 x 10 13 . The achievement is the resuit of many years of effort. The Report of the AGS II Task Force, issued in February 1984, laid out a comprehensive programme largely based on a careful analysis of the PS experience at CERN. The AGS plan had two essential components: the construction of a new booster, and major upgrades to the AGS itself.

  19. Reducing Transport Intensity

    Energy Technology Data Exchange (ETDEWEB)

    Banister, D. [University College London, London (United Kingdom); Stead, D. [OTB Research Institute, Delft University of Technology, Delft (Netherlands)

    2002-07-01

    In this paper transport intensity concerns the economic or energy efficiency of transport. The focus of the paper is the measurement and use of indicators of transport intensity. Most research has concentrated on the volume and distance measures, which show continuous increases over time, normally at a rate that is higher than the growth in Gross Domestic Product (GDP). It is argued that an additional element needs to be included, namely transport efficiency, which relates to modes, technologies, organisational structures, the use of resources and prices. The measurement of GDP also needs to be extended. Measures of economic and transport energy efficiency are applied to EU countries and contrasted with similar measures for the USA and Canada. The empirical evidence is then placed in the wider context of globalisation and economic change, and the case for real decoupling is made for both the freight and passenger sectors.

  20. Intensities of Mobility

    DEFF Research Database (Denmark)

    Bissell, David; Vannini, Phillip; Jensen, Ole B.

    2017-01-01

    This paper explores the intensities of long-distance commuting journeys in order to understand how bodily sensibilities become attuned to the regular mobilities which they undertake. More people are travelling farther to and from work than ever before, owing to a variety of factors which relate...... to complex social and geographical dynamics of transport, housing, lifestyle, and employment. Yet, the experiential dimensions of long-distance commuting have not received the attention that they deserve within research on mobilities. Drawing from fieldwork conducted in Australia, Canada, and Denmark...... this paper aims to further develop our collective understanding of the experiential particulars of long-distance workers or ‘supercommuters’. Rather than focusing on the extensive dimensions of mobilities that are implicated in broad social patterns and trends, our paper turns to the intensive dimensions...

  1. Intense fusion neutron sources

    Science.gov (United States)

    Kuteev, B. V.; Goncharov, P. R.; Sergeev, V. Yu.; Khripunov, V. I.

    2010-04-01

    The review describes physical principles underlying efficient production of free neutrons, up-to-date possibilities and prospects of creating fission and fusion neutron sources with intensities of 1015-1021 neutrons/s, and schemes of production and application of neutrons in fusion-fission hybrid systems. The physical processes and parameters of high-temperature plasmas are considered at which optimal conditions for producing the largest number of fusion neutrons in systems with magnetic and inertial plasma confinement are achieved. The proposed plasma methods for neutron production are compared with other methods based on fusion reactions in nonplasma media, fission reactions, spallation, and muon catalysis. At present, intense neutron fluxes are mainly used in nanotechnology, biotechnology, material science, and military and fundamental research. In the near future (10-20 years), it will be possible to apply high-power neutron sources in fusion-fission hybrid systems for producing hydrogen, electric power, and technological heat, as well as for manufacturing synthetic nuclear fuel and closing the nuclear fuel cycle. Neutron sources with intensities approaching 1020 neutrons/s may radically change the structure of power industry and considerably influence the fundamental and applied science and innovation technologies. Along with utilizing the energy produced in fusion reactions, the achievement of such high neutron intensities may stimulate wide application of subcritical fast nuclear reactors controlled by neutron sources. Superpower neutron sources will allow one to solve many problems of neutron diagnostics, monitor nano-and biological objects, and carry out radiation testing and modification of volumetric properties of materials at the industrial level. Such sources will considerably (up to 100 times) improve the accuracy of neutron physics experiments and will provide a better understanding of the structure of matter, including that of the neutron itself.

  2. NEUTRON FLUX INTENSITY DETECTION

    Science.gov (United States)

    Russell, J.T.

    1964-04-21

    A method of measuring the instantaneous intensity of neutron flux in the core of a nuclear reactor is described. A target gas capable of being transmuted by neutron bombardment to a product having a resonance absorption line nt a particular microwave frequency is passed through the core of the reactor. Frequency-modulated microwave energy is passed through the target gas and the attenuation of the energy due to the formation of the transmuted product is measured. (AEC)

  3. Cockayne syndrome

    DEFF Research Database (Denmark)

    Karikkineth, Ajoy C; Scheibye-Knudsen, Morten; Fivenson, Elayne

    2017-01-01

    Cockayne syndrome (CS) is a disorder characterized by a variety of clinical features including cachectic dwarfism, severe neurological manifestations including microcephaly and cognitive deficits, pigmentary retinopathy, cataracts, sensorineural deafness, and ambulatory and feeding difficulties......, leading to death by 12 years of age on average. It is an autosomal recessive disorder, with a prevalence of approximately 2.5 per million. There are several phenotypes (1-3) and two complementation groups (CSA and CSB), and CS overlaps with xeroderma pigmentosum (XP). It has been considered a progeria...

  4. [Dependency syndrome].

    Science.gov (United States)

    Vuorisalo, Sailaritta

    2013-01-01

    The most common causes of lower limb edema include cardiac insufficiency, venous insufficiency, insufficiency of lymph flow, and side effects of drugs. It can also be due to dependency syndrome, in which the edema and skin changes can only be explained by a passive calf muscle pump and the resulting venous hypertension. Underlying the drop foot is always immobilization for one reason or other. The patient must be given an explanation about the situation, activated to move if possible, and in any case guided to the use of support stockings and postural therapy.

  5. Eagle Syndrome

    Directory of Open Access Journals (Sweden)

    Beytholahi JM

    1998-09-01

    Full Text Available Eagle's syndrome is characterized by an elongated styloid process and (or calcification of"nstylohyoid ligament besides clinical symptoms. The symptoms are those related to pain when"nswallowing or rotating the neck, headacke, earache, dizziness, intermittent glossitis, sensation of"nforeign body in pharynx and transient syncope. The case which is presented can be considered a very"nrare form of the disease in which complete calcification of the ligament and it's thickening has"noccured. Also there is little relationship between the severity of calcification and severity of symptoms."nA careful and thorough evaluation of each panoramic radiography is emphasized.

  6. HELLP syndrome

    Directory of Open Access Journals (Sweden)

    Dilek Acar

    2014-08-01

    Suggested treatment modality consists, stabilization of blood pressure and magnesium sulfate infusion. Then evaluation of fetal status and planning delivery method and time if maternal status remains unstable. If prognosis seems favorable without urgent delivery and fetus can benefit from it, a course of betamethasone can be given to fetuses between 24 and 34 weeks of gestational age. The only and definite treatment of HELLP syndrome is delivering the baby. Suggested benefits of steroid therapy and other experimental treatments are still to be proven effective by large randomized controlled trials. [Archives Medical Review Journal 2014; 23(4.000: 735-760

  7. Olmsted Syndrome

    Directory of Open Access Journals (Sweden)

    Sirka C

    1999-01-01

    Full Text Available A 20-year-old Sikh man had palmoplantar keratoderma, flexion deformity of digits, universal alopecia, keratotic plaques at the angles of mouth, gluteal cleft, knees and dorsal aspects of the metacarpophalangeal joints of the hand; features of Olmsted syndrome. He had normal nails, teeth, oral mucosa and normal joint movements. Treatment with acitretin, 25mg/day for three and a half months, followed by 25mg once daily alternating with 50mg once daily for 3 months resulted in significant improvement.

  8. Absence of association between angiotensin converting enzyme polymorphism and development of adult respiratory distress syndrome in patients with severe acute respiratory syndrome: a case control study

    Directory of Open Access Journals (Sweden)

    Chiu Rossa WK

    2005-04-01

    Full Text Available Abstract Background It has been postulated that genetic predisposition may influence the susceptibility to SARS-coronavirus infection and disease outcomes. A recent study has suggested that the deletion allele (D allele of the angiotensin converting enzyme (ACE gene is associated with hypoxemia in SARS patients. Moreover, the ACE D allele has been shown to be more prevalent in patients suffering from adult respiratory distress syndrome (ARDS in a previous study. Thus, we have investigated the association between ACE insertion/deletion (I/D polymorphism and the progression to ARDS or requirement of intensive care in SARS patients. Method One hundred and forty genetically unrelated Chinese SARS patients and 326 healthy volunteers were recruited. The ACE I/D genotypes were determined by polymerase chain reaction and agarose gel electrophoresis. Results There is no significant difference in the genotypic distributions and the allelic frequencies of the ACE I/D polymorphism between the SARS patients and the healthy control subjects. Moreover, there is also no evidence that ACE I/D polymorphism is associated with the progression to ARDS or the requirement of intensive care in the SARS patients. In multivariate logistic analysis, age is the only factor associated with the development of ARDS while age and male sex are independent factors associated with the requirement of intensive care. Conclusion The ACE I/D polymorphism is not directly related to increased susceptibility to SARS-coronavirus infection and is not associated with poor outcomes after SARS-coronavirus infection.

  9. Influence of traditional Chinese medicine syndrome groups on quality of life in women with metabolic syndrome.

    Science.gov (United States)

    Huang, Li-Wen; Chen, I-Ju; Hsu, Chung-Hua

    2016-10-01

    Traditional Chinese medicine (TCM; zhōng yī) syndrome groups are based on the symptoms of human diseases and guide the use of Chinese herbs. The aim of this study was to examine the effects of TCM syndrome groups on biochemical characteristics and quality of life (QOL) in women with metabolic syndrome (MS). Among the 1080 registered female patients screened at our outpatient clinic, a total of 322 women aged between 18 and 65 years and meeting the requirements of MS were enrolled. All the patients were asked to fill out a questionnaire on metabolic TCM syndrome groups and a questionnaire on the QOL, the Medical Outcomes Study (MOS) Short Form-12 (SF-12). Data of biochemical characteristics were collected at the same time. The present study showed MS women in TCM syndrome groups had significantly lower physical and mental component scores in SF-12 compared with those not in TCM syndrome groups. We also found MS patients in TCM syndrome groups, except Kidney Deficiency syndrome, showed higher body mass indexes, waist circumference, and hip circumference. However, there was almost no difference in most biochemical characteristics between TCM syndrome groups. The MS patients diagnosed as belonging to TCM syndrome groups had poor QOL.

  10. Juvenile fibromyalgia syndrome. Interdisciplinary treatment

    Directory of Open Access Journals (Sweden)

    Hanna Siuchnińska

    2014-11-01

    Full Text Available Fibromyalgia syndrome (FM belongs to soft tissue pain syndromes of unknown cause, also referred to as “soft tissue rheumatism”. It is characterized by chronic widespread pain as well as additional symptoms such as fatigue, sleep and mood disturbance and cognitive problems. There is more and more data showing that this condition may start at a young age or even in childhood, adversely affecting development processes and resulting in dysfunctional social and family relationships. Because of the multifaceted character of fibromyalgia the efficient treatment of this disorder can be difficult and requires comprehensive care. This work reviews most recommended procedures used in integrated treatment programmes for juvenile fibromyalgia syndrome (JFM.

  11. Gorlin Syndrome in Eleven Patients

    Directory of Open Access Journals (Sweden)

    Gülen Eda Utine

    2017-06-01

    Full Text Available Aim: Gorlin syndrome is an autosomal dominant disorder characterized by cutaneous basal cell carcinomas, odontogenic keratocysts and skeletal anomalies. Predisposition to certain types of cancers is among the main features of the disease. Chromosome instability was suspected as a mechanism for cancer predisposition. However, previous studies failed to prove the presence of chromosome instability. Materials and Methods: We present 11 patients with Gorlin syndrome. Results: Six of the patients were checked for increased sister chromatid exchange and were found normal. Two other patients had concurrent chromosome anomalies. Conclusion: Evidence for chromosome instability was not found in our patients. Occurrence of chromosome instability in a subgroup of patients and mechanisms underlying cancer predisposition requires further studies for full elucidation. Hairy patches and pigmentary skin lesions are among the recently defined common features of the syndrome.

  12. Burning mouth syndrome: an update.

    Science.gov (United States)

    López-Jornet, Pia; Camacho-Alonso, Fabio; Andujar-Mateos, Paz; Sánchez-Siles, Mariano; Gómez-Garcia, Francisco

    2010-07-01

    Burning mouth syndrome (BMS) refers to chronic orofacial pain, unaccompanied by mucosal lesions or other evident clinical signs. It is observed principally in middle-aged patients and postmenopausal women. BMS is characterized by an intense burning or stinging sensation, preferably on the tongue or in other areas of the oral mucosa. It can be accompanied by other sensory disorders such as dry mouth or taste alterations. Probably of multifactorial origin, and often idiopathic, with a still unknown etiopathogenesis in which local, systemic and psychological factors are implicated. Currently there is no consensus on the diagnosis and classification of BMS. This study reviews the literature on this syndrome, with special reference to the etiological factors that may be involved and the clinical aspects they present. The diagnostic criteria that should be followed and the therapeutic management are discussed with reference to the most recent studies.

  13. [Geriatric intensive care patients : Perspectives and limits of geriatric intensive care medicine].

    Science.gov (United States)

    Müller-Werdan, U; Heppner, H-J; Michels, G

    2018-04-18

    Critically ill geriatric patients are vitally endangered due to the aging processes of organs, the frequently existing multimorbidity with subsequent polypharmacy and the typical geriatric syndrome of functional impairments. Aging processes in organs lower the clinical threshold for organ dysfunction and organ failure. Physiological organ aging processes with practical consequences for intensive care medicine are atypical manifestion of sepsis in immunosenescence, altered pharmacokinetics, reduced tolerance to hypovolemia due to proportionally reduced water compartment of the body in old age, the frequently only apparently normal function of the kidneys and the continuous reduction in pulmonary function in old age. The main reasons for changes in therapeutic targets are the will of the patient and risk-benefit considerations. The guidelines of the ethics section of the German Interdisciplinary Association for Intensive Care and Emergency Medicine (DIVI) provide assistance and suggestions for a structured decision-making process.

  14. KBG syndrome

    Directory of Open Access Journals (Sweden)

    Brancati Francesco

    2006-12-01

    Full Text Available Abstract KBG syndrome is a rare condition characterised by a typical facial dysmorphism, macrodontia of the upper central incisors, skeletal (mainly costovertebral anomalies and developmental delay. To date, KBG syndrome has been reported in 45 patients. Clinical features observed in more than half of patients that may support the diagnosis are short stature, electroencephalogram (EEG anomalies (with or without seizures and abnormal hair implantation. Cutaneous syndactyly, webbed short neck, cryptorchidism, hearing loss, palatal defects, strabismus and congenital heart defects are less common findings. Autosomal dominant transmission has been observed in some families, and it is predominantly the mother, often showing a milder clinical picture, that transmits the disease. The diagnosis is currently based solely on clinical findings as the aetiology is unknown. The final diagnosis is generally achieved after the eruption of upper permanent central incisors at 7–8 years of age when the management of possible congenital anomalies should have been already planned. A full developmental assessment should be done at diagnosis and, if delays are noted, an infant stimulation program should be initiated. Subsequent management and follow-up should include an EEG, complete orthodontic evaluation, skeletal investigation with particular regard to spine curvatures and limb asymmetry, hearing testing and ophthalmologic assessment.

  15. Cardiorenal syndrome

    Directory of Open Access Journals (Sweden)

    Sabry Omar

    2013-01-01

    Full Text Available Cardiovascular disease is the leading cause of death in patients with chronic kidney disease.  Heart failure may lead to acute kidney injury and vice versa. Chronic kidney disease may affect the clinical outcomes in patients with cardiovascular disorders. Renal impairment with any degree of albuminuria has been increasingly recognized as an independent risk factor for cardiovascular events and heart failure hospitalizations, while chronic heart failure may cause chronic kidney disease. The bidirectional nature of these disorders contributes to the complexity and the composite definitions of cardiorenal syndromes. However, the most important clinical trials in heart failure tend to exclude patients with significant renal dysfunction. The mechanisms whereby renal insufficiency worsens the outcome in heart failure are not known, and several pathways could contribute to the ‘‘vicious heart/kidney circle.’’ Traditionally, renal impairment has been attributed to the renal hypoperfusion due to reduced cardiac output and decreased systemic pressure. The hypovolemia leads to sympathetic activity, increased renin-angiotensin aldosterone pathway, and arginine-vasopressin release. These mechanisms cause fluid and sodium retention, peripheral vasoconstriction, and volume overload. Therapy to improve renal dysfunction, reduce neurohormonal activation and ameliorate renal blood flow could lead to a reduction in mortality and hospitalization in patients with cardiorenal syndrome.

  16. Sotos syndrome

    Directory of Open Access Journals (Sweden)

    Cormier-Daire Valérie

    2007-09-01

    Full Text Available Abstract Sotos syndrome is an overgrowth condition characterized by cardinal features including excessive growth during childhood, macrocephaly, distinctive facial gestalt and various degrees of learning difficulty, and associated with variable minor features. The exact prevalence remains unknown but hundreds of cases have been reported. The diagnosis is usually suspected after birth because of excessive height and occipitofrontal circumference (OFC, advanced bone age, neonatal complications including hypotonia and feeding difficulties, and facial gestalt. Other inconstant clinical abnormalities include scoliosis, cardiac and genitourinary anomalies, seizures and brisk deep tendon reflexes. Variable delays in cognitive and motor development are also observed. The syndrome may also be associated with an increased risk of tumors. Mutations and deletions of the NSD1 gene (located at chromosome 5q35 and coding for a histone methyltransferase implicated in transcriptional regulation are responsible for more than 75% of cases. FISH analysis, MLPA or multiplex quantitative PCR allow the detection of total/partial NSD1 deletions, and direct sequencing allows detection of NSD1 mutations. The large majority of NSD1 abnormalities occur de novo and there are very few familial cases. Although most cases are sporadic, several reports of autosomal dominant inheritance have been described. Germline mosaicism has never been reported and the recurrence risk for normal parents is very low (

  17. Spontaneous Tumor Lysis Syndrome

    Directory of Open Access Journals (Sweden)

    Alicia C. Weeks MD

    2015-08-01

    Full Text Available Tumor lysis syndrome (TLS is a known complication of malignancy and its treatment. The incidence varies on malignancy type, but is most common with hematologic neoplasms during cytotoxic treatment. Spontaneous TLS is thought to be rare. This case study is of a 62-year-old female admitted with multisystem organ failure, with subsequent diagnosis of aggressive B cell lymphoma. On admission, laboratory abnormalities included renal failure, elevated uric acid (20.7 mg/dL, and 3+ amorphous urates on urinalysis. Oliguric renal failure persisted despite aggressive hydration and diuretic use, requiring initiation of hemodialysis prior to chemotherapy. Antihyperuricemic therapy and hemodialysis were used to resolve hyperuricemia. However, due to multisystem organ dysfunction syndrome with extremely poor prognosis, the patient ultimately expired in the setting of a terminal ventilator wean. Although our patient did not meet current TLS criteria, she required hemodialysis due to uric acid nephropathy, a complication of TLS. This poses the clinical question of whether adequate diagnostic criteria exist for spontaneous TLS and if the lack of currently accepted guidelines has resulted in the underestimation of its incidence. Allopurinol and rasburicase are commonly used for prevention and treatment of TLS. Although both drugs decrease uric acid levels, allopurinol mechanistically prevents formation of the substrate rasburicase acts to solubilize. These drugs were administered together in our patient, although no established guidelines recommend combined use. This raises the clinical question of whether combined therapy is truly beneficial or, conversely, detrimental to patient outcomes.

  18. Marfan Syndrome (For Parents)

    Science.gov (United States)

    ... Staying Safe Videos for Educators Search English Español Marfan Syndrome KidsHealth / For Parents / Marfan Syndrome Print en español Síndrome de Marfan What Is Marfan Syndrome? Marfan syndrome is a genetic disorder of the ...

  19. What Is Usher Syndrome?

    Science.gov (United States)

    ... Usher syndrome. However, the RP associated with Usher syndrome shares most of its characteristics with other forms of RP. Researchers expect that advances in understanding and treating other forms of RP will directly benefit people with Usher syndrome and vice versa. What is Usher Syndrome? Risk ...

  20. Burnout Syndrome of Teachers

    OpenAIRE

    Semrádová, Michaela

    2013-01-01

    The bachelor's thesis covers burnout syndrome of teachers. Defines burnout syndrome, describes its causes and symptoms. Describes teaching as helping profession and focousing on stressful situations at school. In the last chapter described different prevention strategies burnout syndrome. Key words: burnout syndrome, teaching, teacher, helping professions, beginning teacher, stress

  1. [Hyperglycaemia and Acute Coronary Syndrome].

    Science.gov (United States)

    Grembiale, A; Cloro, C; Iorio, F; Cufone, S; Succurro, E; Arturi, F

    2012-01-01

    Hyperglycaemia in patients with Acute Coronary Syndrome (ACS)is common, and is an independent predictor of mortality and morbidity in patients both with and without diabetes mellitus. Hyperglycaemia may be a marker of pre-existing diabetes or glucose intolerance or may also represent a transient stress response mediated through the autonomic nervous system with release of adrenal corticosteroids and catecholamines. Several evidences suggest that an intensive control of hyperglycaemia results in a significant improvement of the adverse outcomes in the short and long term. In fact, an intensive metabolic treatment can counteract the negative effects of hyperglycaemia. However, the main difficulty to intensive glucose control in patients with ACS remains hypoglycaemia that is associated with an increased risk of mortality and myocardial re-infarction. No definitive data are available about the beneficial effects of insulin intensive treatment. Therefore, randomized multicenter clinical trials will be needed to definitively establish whether intensive glucose control will reduce the associated increased mortality rate and higher rates of complications in hospitalized ACS patients with hyperglycaemia.

  2. [Childhood periodic syndromes].

    Science.gov (United States)

    Cuvellier, J-C; Lépine, A

    2010-01-01

    This review focuses on the so-called "periodic syndromes of childhood that are precursors to migraine", as included in the Second Edition of the International Classification of Headache Disorders. Three periodic syndromes of childhood are included in the Second Edition of the International Classification of Headache Disorders: abdominal migraine, cyclic vomiting syndrome and benign paroxysmal vertigo, and a fourth, benign paroxysmal torticollis is presented in the Appendix. The key clinical features of this group of disorders are the episodic pattern and intervals of complete health. Episodes of benign paroxysmal torticollis begin between 2 and 8 months of age. Attacks are characterized by an abnormal inclination and/or rotation of the head to one side, due to cervical dystonia. They usually resolve by 5 years. Benign paroxysmal vertigo presents as sudden attacks of vertigo, accompanied by inability to stand without support, and lasting seconds to minutes. Age at onset is between 2 and 4 years, and the symptoms disappear by the age of 5. Cyclic vomiting syndrome is characterized in young infants and children by repeated stereotyped episodes of pernicious vomiting, at times to the point of dehydration, and impacting quality of life. Mean age of onset is 5 years. Abdominal migraine remains a controversial issue and presents in childhood with repeated stereotyped episodes of unexplained abdominal pain, nausea and vomiting occurring in the absence of headache. Mean age of onset is 7 years. Both cyclic vomiting syndrome and abdominal migraine are noted for the absence of pathognomonic clinical features but also for the large number of other conditions to be considered in their differential diagnoses. Diagnostic criteria, such as those of the Second Edition of the International Classification of Headache Disorders and the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, have made diagnostic approach and management easier. Their diagnosis

  3. Digital disruption ?syndromes.

    Science.gov (United States)

    Sullivan, Clair; Staib, Andrew

    2017-05-18

    operational forms of digital disruption which lead us to propose some digital disruption 'syndromes'. The definition and management of these 'syndromes' are discussed in detail. What are the implications for practitioners? Minimising the temporary effects of digital disruption in hospitals requires an understanding that these digital 'syndromes' are to be expected and actively managed during large-scale transformation.

  4. Cosmic Dawn Intensity Mapper

    Science.gov (United States)

    Cooray, Asantha

    2018-01-01

    Cosmic Dawn Intensity Mapper (CDIM) is a 1.0m-class infrared telescope capable of three-dimensional spectro-imaging observations over the wavelength range of 0.75 to 7.5 microns, at a spectral resolving power at or better than 300. This will be achieved with linear variablefilters (LVFs) and a large field-of-view (FoV). The survey strategy using spacecraft operations following a shift and stare mode will result in more than 1300 independent narrow-band spectral images of the sky at a given location. Currently prioritized science programs, taking over three-years of a five-year mission, will be accomplished with a two-tiered wedding-cake survey with the shallowest spanning close to 300 sq. degrees and the deepest tier of about 25 sq. degrees.The remaining two-years could be used for additional survey programs (the wide tier can be expanded to 1000 sq. degrees) or for use by the astronomical community through a General Observing (GO) campaign. CDIM survey data will allow us to (i) establish the initial mass function of stars in galaxies present during reionization, (ii) definitively address AGN/quasar contribution to the reionization photon budget; (iii) establish the environmental dependence of star-formation during reionization through clustering and other environmental measurements; (iv) establish the metal abundance of first-light galaxies during reionization over two decades of stellar mass; (v) measure 3D intensity fluctuations during reionization in both Ly-alpha and H-alpha; and (vi) combine intensity fluctuations with 21-cm data to establish the topology of reionization bubbles.

  5. Superior Mesenteric Artery Syndrome or Wilkie Syndrome

    International Nuclear Information System (INIS)

    Castano Llano, Rodrigo; Chams Anturi, Abraham; Arango Vargas, Paula

    2009-01-01

    We described three cases of superior mesenteric artery (SMA) syndrome, also known as Wilkie's syndrome, chronic duodenal ileus, or cast syndrome. This syndrome occurs when the third portion of the duodenum is compressed between the SMA and the aorta. The major risk factors for development of SMA syndrome are rapid weight loss and surgical correction of spinal deformities. The clinical presentation of SMA syndrome is variable and nonspecific, including nausea, vomiting, abdominal pain, and weight loss. The diagnosis is based on endoscopic, radiographic and tomographic findings of duodenal compression by the SMA. The treatment of SMA syndrome is aimed at the precipitating factor, which usually is related to weight loss. Therefore, conservative therapy with nutritional supplementation is the initial approach, and surgery is reserved for those who do not respond to nutritional therapy.

  6. Requirements Engineering

    CERN Document Server

    Hull, Elizabeth; Dick, Jeremy

    2011-01-01

    Written for those who want to develop their knowledge of requirements engineering process, whether practitioners or students.Using the latest research and driven by practical experience from industry, Requirements Engineering gives useful hints to practitioners on how to write and structure requirements. It explains the importance of Systems Engineering and the creation of effective solutions to problems. It describes the underlying representations used in system modeling and introduces the UML2, and considers the relationship between requirements and modeling. Covering a generic multi-layer r

  7. Software requirements

    CERN Document Server

    Wiegers, Karl E

    2003-01-01

    Without formal, verifiable software requirements-and an effective system for managing them-the programs that developers think they've agreed to build often will not be the same products their customers are expecting. In SOFTWARE REQUIREMENTS, Second Edition, requirements engineering authority Karl Wiegers amplifies the best practices presented in his original award-winning text?now a mainstay for anyone participating in the software development process. In this book, you'll discover effective techniques for managing the requirements engineering process all the way through the development cy

  8. Silvery hair syndrome in two cousins: Chediak-Higashi syndrome vs Griscelli syndrome, with rare associations.

    Science.gov (United States)

    Reddy, R Raghunatha; Babu, Balaji M; Venkateshwaramma, B; Hymavathi, Ch

    2011-07-01

    Silvery hair is a rare clinical manifestation which is a common presentation in a group of rare syndromes which usually present in the pediatric age group together termed as "silvery hair syndrome," consisting of Chediak-Higashi syndrome (CHS), Griscelli syndrome (GS), and Elejalde disease. CHS is a rare autosomal recessive disorder. It is characterized by mild pigment dilution (partial oculocutaneous albinism), silvery blond hair, severe phagocytic immunodeficiency, bleeding tendencies, recurrent pyogenic infections, progressive sensory or motor neurological defects. GS is also a rare autosomal recessive disorder characterized by reduced skin pigmentation, often regarded as partial albinism and silvery grey hair combined with immunodeficiency. To make correct diagnosis and to differentiate between CHS and GS, it requires light microscopic examination of skin and hair shafts, immunological and peripheral blood smear evaluation. They have been reported to be associated with some common clinical association as a part of the syndrome due to pigmentary delusion, neurological dysfunction, and severe life-threatening infections due to neutrophil phagocytosis dysfunction. There are reports of few rare associations and varied presentations and variable mean survival age. We report two cases with common presentation of silvery hair but varied systemic and clinical manifestations and survival in two cousin brothers from the same family.

  9. [Syndrome X vs metabolic syndrome].

    Science.gov (United States)

    Morales Villegas, Enrique

    2006-01-01

    Himsworth in 1939 postulated that Diabetes Mellitus type 2 (DM2) was not only an insulin deficiency state but also a cellular insulin insensitivity disease. Thirty years later, DeFronzo and Reaven demonstrated that insulin resistance (IR) preceded and predisposed for DM2 and atherosclerotic-cardiovascular-disease (ACVD). Reaven was the first to point out the relationship between IR and with hyperglycemia, dyslipidosis, and hypertension as mediators for ACVD, creating the concept of Syndrome X (SX) in 1988. WHO and, thereafter, other medical societies and medical groups, mainly ATP-III, in 2002, based on the difficulty of diagnosing IR in a simple, reliable, and inexpensive way, proposed and published the Metabolic Syndrome (MS) concept, as a group of five variables, i.e., obesity, hyperglycemia, hypertriglyceridemia, low HDL, and hypertension, as an easy clinical approximation to suspect and treat an increased cardiometabolic risk. Nowadays, there are deep and extensive controversies on this issue; however, these controversies do not really exist since all discordant points of view are rather quantitative and not qualitative in nature. This article is aimed at differentiating and harmonizing the complementary concepts of SX and MS, at analyzing why MS is a good "clinical window" to look for IR and its underlying manifestations, and finally to accept that the MS concept complements, but does not substitute or antagonize, traditional scales used to asses cardiovascular risk, such as the Framingham scale.

  10. Intensive care unit family satisfaction survey.

    Science.gov (United States)

    Lam, S M; So, H M; Fok, S K; Li, S C; Ng, C P; Lui, W K; Heyland, D K; Yan, W W

    2015-10-01

    To examine the level of family satisfaction in a local intensive care unit and its performance in comparison with international standards, and to determine the factors independently associated with higher family satisfaction. Questionnaire survey. A medical-surgical adult intensive care unit in a regional hospital in Hong Kong. Adult family members of patients admitted to the intensive care unit for 48 hours or more between 15 June 2012 and 31 January 2014, and who had visited the patient at least once during their stay. Of the 961 eligible families, 736 questionnaires were returned (response rate, 76.6%). The mean (± standard deviation) total satisfaction score, and subscores on satisfaction with overall intensive care unit care and with decision-making were 78.1 ± 14.3, 78.0 ± 16.8, and 78.6 ± 13.6, respectively. When compared with a Canadian multicentre database with respective mean scores of 82.9 ± 14.8, 83.5 ± 15.4, and 82.6 ± 16.0 (Pcare were concern for patients and families, agitation management, frequency of communication by nurses, physician skill and competence, and the intensive care unit environment. A performance-importance plot identified the intensive care unit environment and agitation management as factors that required more urgent attention. This is the first intensive care unit family satisfaction survey published in Hong Kong. Although comparable with published data from other parts of the world, the results indicate room for improvement when compared with a Canadian multicentre database. Future directions should focus on improving the intensive care unit environment, agitation management, and communication with families.

  11. Syndromic diarrhea/Tricho-hepato-enteric syndrome

    Directory of Open Access Journals (Sweden)

    Fabre Alexandre

    2013-01-01

    Full Text Available Abstract Syndromic diarrhea/Tricho-hepato-enteric syndrome (SD/THE is a rare and severe bowel disorder caused by mutation in SKIV2L or in TTC37, 2 genes encoding subunits of the putative human SKI complex. The estimated prevalence is 1/1,000,000 births and the transmission is autosomal recessive. The classical form is characterized by 5 clinical signs: intractable diarrhea of infancy beginning in the first month of life, usually leading to failure to thrive and requiring parenteral nutrition; facial dysmorphism characterised by prominent forehead and cheeks, broad nasal root and hypertelorism; hair abnormalities described as woolly and easily removable; immune disorders resulting from defective antibody production; intrauterine growth restriction. The aetiology is a defect in TTC37, a TPR containing protein, or in the RNA helicase SKIV2L, both constituting the putative human ski complex. The ski complex is a heterotetrameric cofactor of the cytoplasmic RNA exosome which ensures aberrants mRNAs decay. The diagnosis SD/THE is initially based on clinical findings and confirmed by direct sequencing of TTC37 and SKIV2L. Differential diagnosis with the other causes of intractable diarrhea is easily performed by pathologic investigations. During their clinical course, most of the children require parenteral nutrition and often immunoglobulin supplementation. With time, some of them can be weaned off parenteral nutrition and immunoglobulin supplementation. The prognosis depends on the management and is largely related to the occurrence of parenteral nutrition complications or infections. Even with optimal management, most of the children seem to experience failure to thrive and final short stature. Mild mental retardation is observed in half of the cases. Abstract in French Les diarrhées syndromiques ou syndrome tricho-hepato-enterique (SD/THE sont un syndrome rare et sévère dont l’incidence est estimée à 1 cas pour 1 million de naissances et la

  12. The catastrophic antiphospholipid syndrome in children.

    Science.gov (United States)

    Go, Ellen J L; O'Neil, Kathleen M

    2017-09-01

    To review the difficult syndrome of catastrophic antiphospholipid syndrome, emphasizing new developments in the diagnosis, pathogenesis and treatment. Few recent publications directly address pediatric catastrophic antiphospholipid syndrome (CAPS). Most articles are case reports or are data from adult and pediatric registries. The major factors contributing to most pediatric catastrophic antiphospholipid syndrome include infection and the presence of antiphospholipid antibodies, but complement activation also is important in creating diffuse thrombosis in the microcirculation. Treatment of the acute emergency requires anticoagulation, suppression of the hyperinflammatory state and elimination of the triggering infection. Inhibition of complement activation appears to improve outcome in limited studies, and suppression of antiphospholipid antibody formation may be important in long-term management. CAPS, an antibody-mediated diffuse thrombotic disease of microvasculature, is rare in childhood but has high mortality (33-50%). It requires prompt recognition and aggressive multimodality treatment, including anticoagulation, anti-inflammatory therapy and elimination of inciting infection and pathogenic autoantibodies.

  13. Syndromes with supernumerary teeth.

    Science.gov (United States)

    Lubinsky, Mark; Kantaputra, Piranit Nik

    2016-10-01

    While most supernumerary teeth are idiopathic, they can be associated with a number of Mendelian syndromes. However, this can also be a coincidental finding, since supernumerary teeth occur in 6% or more of the normal population. To better define this relationship, we analyzed the evidence for specific associations. We excluded conditions with a single affected patient reported, supernumerary teeth adjacent to clefts or other forms of alveolar disruption (as secondary rather than primary findings), and natal teeth, which can involve premature eruption of a normal tooth. Since, the cause of supernumerary teeth shows considerable heterogeneity, certain findings are less likely to be coincidental, such as five or more supernumerary teeth in a single patient, or locations outside of the premaxilla. We found only eight genetic syndromes with strong evidence for an association: cleidocranial dysplasia; familial adenomatous polyposis; trichorhinophalangeal syndrome, type I; Rubinstein-Taybi syndrome; Nance-Horan syndrome; Opitz BBB/G syndrome; oculofaciocardiodental syndrome; and autosomal dominant Robinow syndrome. There is also suggestive evidence of an association with two uncommon disorders, Kreiborg-Pakistani syndrome (craniosynostosis and dental anomalies), and insulin-resistant diabetes mellitus with acanthosisnigricans. An association of a Mendelian disorder with a low frequency manifestation of supernumerary teeth is difficult to exclude without large numbers, but several commonly cited syndromes lacked evidence for clear association, including Hallermann-Streiff syndrome, Fabry disease, Ehlers-Danlos syndrome, Apert and Crouzon syndromes, Zimmermann-Laband syndrome, and Ellis-van Creveld syndrome. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

  14. HEPATORENAL SYNDROME

    Directory of Open Access Journals (Sweden)

    Matjaž Hafner

    2001-12-01

    Full Text Available Background. Hepatorenal syndrome (HRS is acommon complication of advanced hepatic disease characterizedby marked abnormalities in arterial circulation and byrenal failure. An extreme arteriolar vasodilatation located inthe splanchnic circulation results in a reduction of total systemicvascular resistence and arterial hypotension. Vasoconstrictionoccurs in the renal circulation as in all other extrasplanchnicvascular territories. In the kidney, marked renalvasoconstriction results in a low glomerular filtration rate.Conclusions. The diagnosis of HRS is currently based on exclusionof other causes of renal failure. Prognosis of patientswith HRS is very poor. Liver transplantation is the best therapeuticoption, but it is seldom applicable due to the short survivalexpectancy of most patients with HRS, particularly thosewith the rapidly progressive type of HRS. New therapies developedduring the last few years, such as the use of systemicvasoconstrictors or transjugular intrahepatic portosystemicshunts (TIPS appear promising. Such treatments are of interestnot only as a bridge to liver transplantation but also as atherapy for patients who are not candidates for transplantation.

  15. 22q11.2 Deletion syndrome is associated with perioperative outcome in tetralogy of Fallot.

    Science.gov (United States)

    Mercer-Rosa, Laura; Pinto, Nelangi; Yang, Wei; Tanel, Ronn; Goldmuntz, Elizabeth

    2013-10-01

    We sought to investigate the impact of 22q11.2 deletion on perioperative outcome in tetralogy of Fallot. We conducted a retrospective review of patients with tetralogy of Fallot who underwent complete surgical reconstruction at The Children's Hospital of Philadelphia between 1995 and 2006. Inclusion criteria included diagnosis of tetralogy of Fallot and known genotype. Fisher exact and Mann-Whitney tests were used for categoric and continuous variables, respectively. Regression analysis was used to determine whether deletion status predicts outcome. We studied 208 subjects with tetralogy of Fallot, 164 (79%) without and 44 (20%) with 22q11.2 deletion syndrome. There were no differences in sex, race, gestational age, age at diagnosis, admission weight, and duration of mechanical ventilation. Presenting anatomy, survival, complications and reoperations were also comparable between patients with and without 22q11.2 deletion syndrome. Those with 22q11.2 deletion syndrome had more aortopulmonary shunts preceding complete surgical repair (21% vs 7%, P = .02). This association was present after adjustment for presenting anatomy (stenosis, atresia, or absence of pulmonary valve and common atrioventricular canal) and surgical era. In addition, those with 22q11.2 deletion syndrome had longer cardiopulmonary bypass time (84 vs 72 minutes, P = .02) and duration of intensive care (6 vs 4 days, P = .007). Genotype affects early operative outcomes in tetralogy of Fallot resulting, in particular, in longer duration of intensive care. Future studies are required to determine factors contributing to such differences in this susceptible population. Copyright © 2013 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.

  16. Sotos syndrome.

    Science.gov (United States)

    Baujat, Geneviève; Cormier-Daire, Valérie

    2007-09-07

    Sotos syndrome is an overgrowth condition characterized by cardinal features including excessive growth during childhood, macrocephaly, distinctive facial gestalt and various degrees of learning difficulty, and associated with variable minor features. The exact prevalence remains unknown but hundreds of cases have been reported. The diagnosis is usually suspected after birth because of excessive height and occipitofrontal circumference (OFC), advanced bone age, neonatal complications including hypotonia and feeding difficulties, and facial gestalt. Other inconstant clinical abnormalities include scoliosis, cardiac and genitourinary anomalies, seizures and brisk deep tendon reflexes. Variable delays in cognitive and motor development are also observed. The syndrome may also be associated with an increased risk of tumors. Mutations and deletions of the NSD1 gene (located at chromosome 5q35 and coding for a histone methyltransferase implicated in transcriptional regulation) are responsible for more than 75% of cases. FISH analysis, MLPA or multiplex quantitative PCR allow the detection of total/partial NSD1 deletions, and direct sequencing allows detection of NSD1 mutations. The large majority of NSD1 abnormalities occur de novo and there are very few familial cases. Although most cases are sporadic, several reports of autosomal dominant inheritance have been described. Germline mosaicism has never been reported and the recurrence risk for normal parents is very low (therapies are mostly symptomatic, including phototherapy in case of jaundice, treatment of the feeding difficulties and gastroesophageal reflux, and detection and treatment of hypoglycemia. General pediatric follow-up is important during the first years of life to allow detection and management of clinical complications such as scoliosis and febrile seizures. An adequate psychological and educational program with speech therapy and motor stimulation plays an important role in the global development of the

  17. Raynaud syndrome.

    Science.gov (United States)

    Valdovinos, Sergio Toledo; Landry, Gregory J

    2014-12-01

    Raynaud syndrome (RS) was first described by the French physician Maurice Raynaud in 1862 with the characteristic tricolor change featuring pallor (ischemic phase), cyanosis (deoxygenation phase), and erythema (reperfusion phase) induced by cold or stress. Although the underlying pathophysiological mechanism is unclear, alterations in activity of the peripheral adrenoceptor have been implicated, specifically an enhanced smooth muscle contraction due to overexpression or hyperactivity of postsynaptic alpha 2 receptors. There are 2 ways that RS can appear clinically; isolated, formerly referred as Raynaud disease or now primary RS and in association with other conditions, usually connective tissue disorders (eg, Sjögren syndrome, systemic lupus erythematosus, scleroderma, and rheumatoid arthritis), frequently called Raynaud phenomenon or secondary RS. The estimated prevalence in the general population is 3%-5%, with a higher prevalence in women than in men. The diagnosis is mainly clinical, based on patient descriptions of skin changes. Upper extremity pulse-volume recording is used to rule out proximal arterial obstruction. The differentiation between a vasospastic vs and obstructive mechanism is made using digital pressures and photoplethysmography, where an obstructive mechanism has decreased pressures and blunted waveforms. Cold challenge testing, such as ice water immersion with temperature recovery, is highly sensitive but lack specificity. Serologic screening (antinuclear antibody and rheumatoid factor) is advocated to rule out associated connective tissue disorders. Most patients with RS can be managed conservatively, with avoidance of cold exposure or hand warming. For those in whom conservative management is inadequate, a number of pharmacologic and surgical therapies have been used. Owing to lack of complete understanding of the underlying pathophysiology, targeted therapy has not been possible; rather, therapy has been focused on the use of general

  18. Griscelli syndrome.

    Science.gov (United States)

    Emanuel, Patrick O; Sternberg, Lauren J; Phelps, Robert G

    2007-01-01

    The dermatology staff was called to evaluate abnormal hair on a 22-month-old Hispanic girl whose parents were first cousins. Her medical history was significant for leptomeningitis with subsequent neurologic devastation, gastroesophageal reflux disease, and recurrent respiratory infections. Her hospital course was complicated by sepsis, liver dysfunction, pan-cytopenia, and disseminated intravascular coagulation. She had developed normally for the first year of life. At 13 months she became progressively lethargic and developed floppy muscle tone; a delay in mental and motor milestones was recognized. Results of a metabolic workup were negative. On examination she was noted to have generalized excessively fair skin when compared with her parents. She had silver-gray hair (Figure 1) and white eyebrows and body hair. Her maternal grandfather and granduncles had silver hair since childhood, but were without health problems. A maternal family member was said to have light skin. The presumed diagnosis before pathologic examination was Chediak-Higashi syndrome. Hematoxylin and eosin stain tests revealed prominent melanocytes in the basal layer of the epidermis. The melanocytes were large and distended with a large volume of melanin (Figure 2). The adjacent keratinocytes were completely devoid of melanin. Application of Masson-Fontana ammoniac silver stain highlighted prominent melanocytic melanin and a relative paucity of melanin in the adjacent keratinocytes (Figure 3). Microscopic examination of her hair revealed clumps of melanin of various sizes and shapes irregularly distributed throughout the hair shaft. Ultrastructural examination of the epidermis showed the melanocytes were distended by an accumulation of large stage IV mature melanosomes. Peripheral blood smear failed to show abnormal granules, even after repeated examination. Based on the clinical features and the pathologic findings, a diagnosis of Griscelli syndrome type 2 was made.

  19. Mosaic Turner syndrome and hyperinsulinaemic hypoglycaemia

    DEFF Research Database (Denmark)

    Alkhayyat, H.; Christesen, Henrik Thybo; Steer, J.

    2006-01-01

    BACKGROUND: A common and well recognised feature of Turner's syndrome (partial or total monosomy X) is impaired glucose tolerance or type 2 diabetes mellitus. A small percentage of patients with Turner's syndrome have a complex mosaic karyotype with atypical clinical features and mental retardation......'s syndrome is important as persistent hypoglycaemia may lead to brain damage in addition to the risk of mental retardation. Further studies are required to understand whether the mosaic over--or underexpression of unidentified X chromosome gene(s) in the pancreatic beta-cells leads to hyperinsulinaemic...

  20. Burnout Among Anesthetists and Intensive Care Physicians.

    Science.gov (United States)

    Mikalauskas, Audrius; Benetis, Rimantas; Širvinskas, Edmundas; Andrejaitienė, Judita; Kinduris, Šarūnas; Macas, Andrius; Padaiga, Žilvinas

    2018-01-01

    Burnout is a syndrome of depersonalization, emotional exhaustion, and low personal accomplishment. Little is known about burnout in physicians. Our objective was to determine the prevalence of burnout among anesthetists and intensive care physicians, and associations between burnout and personal, as well as professional, characteristics. In total, 220 anesthetists and intensive care physicians were contacted by email, asking them to participate in the study. For depression screening the PHQ-2 questionnaire, for problem drinking, CAGE items were used. Burnout was measured by the Maslach Burnout Inventory. Overall, 34% anesthetists and intensive care physicians indicated high levels of emotional exhaustion, 25% indicated high levels of depersonalization, and 38% showed low personal accomplishment. Burnout was found more frequent among subjects with problem drinking (OR 3.2, 95% CI 1.5-6.8), depressiveness (OR 10.2, 95% CI 4.6-22.6), cardiovascular disorders (OR 3.4, 95% CI 1.7-7.1), and digestive disorders (OR 2.2, 95% CI 1.2-4.0). Some favorite after-work activities positively correlated with burnout, such as sedative medications abuse (OR 4.8, 95% CI 1.8-12.5), alcohol abuse (OR 2.4, 95% CI 1.3-4.5), eating more than usual (OR 1.9, 95% CI 1.1-3.5), and transferring the accumulated stress to relatives (OR 2.8, 95% CI 1.4-5.5). In contrast, reading of non-medical literature seemed to have a protective effect (OR 0.5, 95% CI 0.2-0.9). Burnout was highly prevalent among anesthetists and intensive care physicians with two fifths of them meeting diagnostic criteria. It was strongly correlated with problem drinking, depressiveness, cardiovascular and digestive disorders, use of sedatives and overeating.