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Sample records for hormone syndrome complicated

  1. Pregnancy Complications: HELLP Syndrome

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    ... baby Common illnesses Family health & safety Complications & Loss Pregnancy complications Preterm labor & premature birth The newborn intensive care ... Point, NY 10980 Close X Home > Complications & Loss > Pregnancy complications > HELLP syndrome HELLP syndrome E-mail to a ...

  2. Primary intracranial neuroendocrine tumor with ectopic adrenocorticotropic hormone syndrome: A rare and complicated case report and literature review.

    Science.gov (United States)

    Liu, Hailong; Zhang, Mingshan; Wang, Xuan; Qu, Yanming; Zhang, Hongwei; Yu, Chunjiang

    2016-07-01

    Neuroendocrine tumors (NETs) and ectopic adrenocorticotropic hormone (ACTH) syndrome are frequent in adult patients. However, primary intracranial NETs, exhibiting immunonegativity for ACTH, high serum ACTH level and treated with anterior skull base reconstruction, are rare and complicated. We herein present a case of a primary intracranial NET immunonegative for ACTH, resulting in ectopic ACTH syndrome. A 40-year-old woman presented with intermittent rhinorrhea, rapid weight gain, polydipsia, polyuria, hypertension, dimness, bilateral exophthalmus, diminution of vision in the left eye and pigmentation of the skin of the face and trunk. Computed tomography (CT) and magnetic resonance imaging scans revealed a sizeable enhancing tumor in the anterior cranial fossa, which infiltrated the sphenoid and ethmoid sinuses bilaterally, the left maxillary sinus and the nasal cavity. Abdominal CT scans revealed bilateral adrenal hyperplasia. The biochemical findings included hypokalemia and high glucose, cortisol, plasma ACTH, 24-h urinary free cortisol and testosterone levels. The neoplasm was exposed through a right frontal craniotomy, while anterior skull base reconstruction was performed during surgery. The intracranial surgery achieved gross removal of the tumor; however, part of the tumor remained in the nasal cavity. Histopathological examination of the surgical specimen confirmed the diagnosis of a low-grade small-cell NET, exhibiting immunonegativity for ACTH. A postoperative abdominal CT scan demonstrated bilateral regression of the adrenal gland hyperplasia and the serum ACTH level returned to normal after 16 days. To the best of our knowledge, there are no previous reports of primary intracranial NETs, immunohistochemically negative for ACTH, resulting in ectopic ACTH syndrome.

  3. Complications of nephrotic syndrome

    OpenAIRE

    Se Jin Park; Jae Il Shin

    2011-01-01

    Nephrotic syndrome (NS) is one of the most common glomerular diseases that affect children. Renal histology reveals the presence of minimal change nephrotic syndrome (MCNS) in more than 80% of these patients. Most patients with MCNS have favorable outcomes without complications. However, a few of these children have lesions of focal segmental glomerulosclerosis, suffer from severe and prolonged proteinuria, and are at high risk for complications. Complications of NS are divided into two c...

  4. Complications of nephrotic syndrome.

    Science.gov (United States)

    Park, Se Jin; Shin, Jae Il

    2011-08-01

    Nephrotic syndrome (NS) is one of the most common glomerular diseases that affect children. Renal histology reveals the presence of minimal change nephrotic syndrome (MCNS) in more than 80% of these patients. Most patients with MCNS have favorable outcomes without complications. However, a few of these children have lesions of focal segmental glomerulosclerosis, suffer from severe and prolonged proteinuria, and are at high risk for complications. Complications of NS are divided into two categories: disease-associated and drug-related complications. Disease-associated complications include infections (e.g., peritonitis, sepsis, cellulitis, and chicken pox), thromboembolism (e.g., venous thromboembolism and pulmonary embolism), hypovolemic crisis (e.g., abdominal pain, tachycardia, and hypotension), cardiovascular problems (e.g., hyperlipidemia), acute renal failure, anemia, and others (e.g., hypothyroidism, hypocalcemia, bone disease, and intussusception). The main pathomechanism of disease-associated complications originates from the large loss of plasma proteins in the urine of nephrotic children. The majority of children with MCNS who respond to treatment with corticosteroids or cytotoxic agents have smaller and milder complications than those with steroid-resistant NS. Corticosteroids, alkylating agents, cyclosporin A, and mycophenolate mofetil have often been used to treat NS, and these drugs have treatment-related complications. Early detection and appropriate treatment of these complications will improve outcomes for patients with NS.

  5. Complications of nephrotic syndrome

    Directory of Open Access Journals (Sweden)

    Se Jin Park

    2011-08-01

    Full Text Available Nephrotic syndrome (NS is one of the most common glomerular diseases that affect children. Renal histology reveals the presence of minimal change nephrotic syndrome (MCNS in more than 80% of these patients. Most patients with MCNS have favorable outcomes without complications. However, a few of these children have lesions of focal segmental glomerulosclerosis, suffer from severe and prolonged proteinuria, and are at high risk for complications. Complications of NS are divided into two categories: disease-associated and drug-related complications. Disease-associated complications include infections (e.g., peritonitis, sepsis, cellulitis, and chicken pox, thromboembolism (e.g., venous thromboembolism and pulmonary embolism, hypovolemic crisis (e.g., abdominal pain, tachycardia, and hypotension, cardiovascular problems (e.g., hyperlipidemia, acute renal failure, anemia, and others (e.g., hypothyroidism, hypocalcemia, bone disease, and intussusception. The main pathomechanism of disease-associated complications originates from the large loss of plasma proteins in the urine of nephrotic children. The majority of children with MCNS who respond to treatment with corticosteroids or cytotoxic agents have smaller and milder complications than those with steroid-resistant NS. Corticosteroids, alkylating agents, cyclosporin A, and mycophenolate mofetil have often been used to treat NS, and these drugs have treatment-related complications. Early detection and appropriate treatment of these complications will improve outcomes for patients with NS.

  6. Lemierre's Syndrome Complicating Pregnancy

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    M. Thompson

    2007-01-01

    Full Text Available Lemierre's syndrome is an anaerobic suppurative thrombophlebitis involving the internal jugular vein secondary to oropharyngeal infection. There is only one previous case report in pregnancy which was complicated by premature delivery of an infant that suffered significant neurological damage. We present an atypical case diagnosed in the second trimester with a live birth at term. By reporting this case, we hope to increase the awareness of obstetricians to the possibility of Lemierre's syndrome when patients present with signs of unabating oropharyngeal infection and pulmonary symptoms.

  7. West syndrome, vigabatrine, adrenocorticotropic hormone

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    Ünsal Yılmaz

    2014-03-01

    Full Text Available Objective: Limited data are available on the etiology, clinical approach, treatment and outcome in West syndrome. In the present study, we aimed to document clinical characteristics, etiology and treatment response in children with West syndrome. Methods: Hospital charts of children who were diagnosed with West syndrome between July-2011 and December- 2013 and who had a follow-up at least 12-month, were reviewed retrospectively. Results: 38 patients (14 females, 24 males, mean aged 27.1±7.60 months were included. The mean age of seizure onset, interval to diagnosis, and follow-up period were 6.23±4.27 months, 1.36±1.58 months, and 19.3±5.86 months respectively. Perinatal asphyxia (13, tuberous sclerosis (2, cortical dysplasia (2, encephalitis (1, asphyxia due to aspiration (1, congenital cytomegalovirus infection (1, perinatal infarct (1, nonketotic hyperglycinemia (1 and Prader Willi syndrome (1 were the identified causes. The etiology could not be ascertained in the remaining 15 children. Psychomotor development was mildly retarded in 12, moderately retarded in 13, and severely in 13 patients at onset, and did not change significantly at month 12. The initial therapy was synthetic adrenocorticotropic hormone in 11, vigabatrin in 17, levetiracetam in 8 and valproate in 2 patients. At 12th month of therapy, 15 patients were seizure-free, 12 patients showed more than 50% decrease in seizure frequency, and remaining 11 patients showed no significant reduction in seizure frequency. Conclusion: Besides the perinatal asphyxia as most frequent cause, a wide variety of disorders can present as West syndrome. Although, a 12-month-long treatment achieves seizure control in half of the patients, not beneficial effect on psychomotor development was seen. J Clin Exp Invest 2014; 5 (1: 86-92

  8. Respiratory Complications in Children with Prader Willi Syndrome.

    Science.gov (United States)

    Tan, H-L; Urquhart, D S

    2017-03-01

    Prader Willi syndrome, resulting from the partial deletion or lack of expression of a region of genes on the paternal chromosome 15, has a number of phenotypic features which predispose affected patients to ventilatory problems. These include generalised hypotonia, abnormal arousal and ventilatory responses to hypoxia and hypercapnia, scoliosis and frequently, obesity. The spectrum of the resulting respiratory complications thus runs from sleep disordered breathing, to aspiration and respiratory functional impairment. While the use of growth hormone, in conjunction with multidisciplinary clinical management, is currently the cornerstone of clinical care of these patients, concerns have been raised following reports of sudden death shortly after growth hormone initiation. This review summarizes the respiratory complications commonly seen and draws together the published literature on the impact of growth hormone in relation to various respiratory parameters, aiming to provide the reader with the necessary information to manage these patients as safely as possible. Copyright © 2016 Elsevier Ltd. All rights reserved.

  9. A rare syndrome: Thyroid hormone resistance

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    Yunus İlyas Kibar

    2011-09-01

    Full Text Available Resistance to thyroid hormone syndrome (RTH is a rare disorder, usually inherited as an autosomal dominant trait. Patients with RTH are usually euthyroid but can occasionally present with signs and symptoms of thyrotoxicosis or rarely with hypothyroidism. We present a patient with interesting syndrome as RTH but no family history. Goiter, increased weight gain and normal mental status were observed despite high serum thyroid hormones and normal TSH levels

  10. Sex hormone replacement in Turner syndrome

    DEFF Research Database (Denmark)

    Trolle, Christian; Hjerrild, Britta; Cleemann, Line Hartvig

    2012-01-01

    osteoporosis seen in Turner syndrome. But sex hormone insufficiency is also involved in the increased cardiovascular risk, state of physical fitness, insulin resistance, body composition, and may play a role in the increased incidence of autoimmunity. Severe morbidity and mortality affects females with Turner...... syndrome. Recent research emphasizes the need for proper sex hormone replacement therapy (HRT) during the entire lifespan of females with TS and new hypotheses concerning estrogen receptors, genetics and the timing of HRT offers valuable new information. In this review, we will discuss the effects...

  11. Paroxysmal tonic upward gaze complicating Angelman syndrome.

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    Fukumura, Shinobu; Watanabe, Toshihide; Takayama, Rumiko; Tsutsumi, Hiroyuki

    2015-01-01

    Paroxysmal tonic upward gaze is a childhood oculomotor syndrome characterized by episodes of conjugate upward deviation of the eyes. Its pathogenesis is unknown, and the etiology is heterogeneous. We describe a 2-year-old girl with Angelman syndrome who developed paroxysmal tonic upward gaze at 9 months of age. She presented with developmental delay, blond hair, jerky movements, ataxia, and epilepsy. Genetic testing revealed a maternal deletion of 15q11-13, confirming Angelman syndrome. This is the first report of Angelman syndrome complicated by paroxysmal tonic upward gaze. Both transient paroxysmal tonic upward gaze and Angelman syndrome have been associated with dopaminergic neurons. We speculate that the dopaminergic abnormalities present in Angelman syndrome may cause paroxysmal tonic upward gaze. Copyright © 2015 Elsevier Inc. All rights reserved.

  12. [Human growth hormone and Turner syndrome].

    Science.gov (United States)

    Sánchez Marco, Silvia Beatriz; de Arriba Muñoz, Antonio; Ferrer Lozano, Marta; Labarta Aizpún, José Ignacio; Garagorri Otero, Jesús María

    2017-02-01

    The evaluation of clinical and analytical parameters as predictors of the final growth response in Turner syndrome patients treated with growth hormone. A retrospective study was performed on 25 girls with Turner syndrome (17 treated with growth hormone), followed-up until adult height. Auxological, analytical, genetic and pharmacological parameters were collected. A descriptive and analytical study was conducted to evaluate short (12 months) and long term response to treatment with growth hormone. A favourable treatment response was shown during the first year of treatment in terms of height velocity gain in 66.6% of cases (height-gain velocity >3cm/year). A favourable long-term treatment response was also observed in terms of adult height, which increased by 42.82±21.23cm (1.25±0.76 SDS), with an adult height gain of 9.59±5.39cm (1.68±1.51 SDS). Predictors of good response to growth hormone treatment are: A) initial growth hormone dose, B) time on growth hormone treatment until starting oestrogen therapy, C) increased IGF1 and IGFBP-3 levels in the first year of treatment, and D) height gain velocity in the first year of treatment. Copyright © 2015 Asociación Española de Pediatría. Publicado por Elsevier España, S.L.U. All rights reserved.

  13. [Terson syndrome. Complicated clinical course].

    Science.gov (United States)

    Daus, W; Käsmann, B; Alexandridis, E

    1992-02-01

    A report is given on three patients who developed spontaneous subarachnoidal bleeding, together with vitreous hemorrhage (Terson's syndrome). In two patients the vitreous hemorrhage was unilateral and showed spontaneous resorption within 15 months. The third patient presented with bilateral vitreous hemorrhage, which first showed slow spontaneous resorption. Five months later, however, secondary vitreous bleeding occurred. Vitrectomy was therefore performed in both eyes. Post-operatively, both eyes developed focal vitreoretinal reproliferations with retinal tears, which led to retinal detachment that was successfully treated by surgery. Because of these observations we can make the following suggestions: in patients presenting with unilateral vitreous hemorrhage one should first watch the spontaneous resorption of the hemorrhage. If there is delayed blood resorption in bilateral vitreous hemorrhage and at the beginning of the hemorrhage, a pars plana vitrectomy should be performed. In one patient an additional ocular symptom peripheral paresis of the facial nerve; a second patient presented with paresis of the oculomotor nerve. Both resolved spontaneously within 15 months.

  14. Cauda Equina Syndrome Secondary to Complicated Diverticulitis

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    M. ter Horst

    2013-10-01

    Full Text Available A 58-year-old woman presented to the emergency department with cauda equina syndrome and sepsis. The symptoms were attributed to a complicated episode of sigmoid diverticulitis. MRI showed that the diverticulitis had caused an intra-abdominal fistula to a presacrally localized abscess expanding into the spinal canal, compressing the cauda equina nerves. Although Hartmann's procedure was performed, the neurological symptoms persisted, causing the patient to remain partially paraplegic. This case report illustrates that cauda equina syndrome is a condition that can also be caused by intra-abdominal pathology such as diverticulitis.

  15. Idiopathic pulmonary hemosiderosis complicated by Down syndrome.

    Science.gov (United States)

    Watanabe, Hirofumi; Ayusawa, Mamoru; Kato, Masataka; Chou, Ami; Komori, Akiko; Abe, Yuriko; Matsumura, Masaharu; Kamiyama, Hiroshi; Izumi, Hiroyuki; Takahashi, Shori

    2015-10-01

    We report the case of a 9-year-old girl with Down syndrome (DS) diagnosed with idiopathic pulmonary hemosiderosis (IPH). Although acute pneumonia complicated by hemolytic anemia was suspected, IPH was finally diagnosed on bronchoscopy. Treatment with prednisolone achieved good clinical response. An association between IPH and DS was not able to be identified, but immunological issues in DS may contribute to the onset of IPH. Recurrent and intractable respiratory symptoms with marked infiltrative shadows in the bilateral lungs and complicated by severe anemia in patients with DS should suggest IPH. © 2015 Japan Pediatric Society.

  16. Lower limb compartment syndrome as a complication of radical hysterectomy

    OpenAIRE

    Oman, Sarah A.; Schwarz, Daniel; Muntz, Howard G.

    2016-01-01

    Highlights ? Compartment syndrome is a rare complication of prolonged lithotomy position. ? We report a case of compartment syndrome complicating radical hysterectomy. ? Prompt diagnosis is crucial to reduce morbidity from compartment syndrome. ? Awareness of this complication allows surgeons to address modifiable risk factors.

  17. Hormonal and biochemical disturbances in Down's syndrome.

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    Hestnes, A; Stovner, L J; Husøy, O; Følling, I; Fougner, K J; Sjaastad, O

    1991-06-01

    Clinical and laboratory endocrine variables in 29 adult institutionalized patients with Down's syndrome were compared with those of matched controls consisting of other mentally retarded patients from the same institution. Of the clinical variables, testes volume and body height were significantly lower in patients with Down's syndrome than in control patients. The thyroid function tests documented a higher average TSH level in Down's syndrome than in other mentally retarded patients. However, there was no clear-cut correlation between TSH and thyroid hormone levels. The data indicate that there is a tendency towards primary thyroid dysfunction in Down's syndrome. In addition, there is some evidence indicating a relative failure of TSH secretion. In male patients, estradiol was elevated compared to controls. FSH and LH also seemed slightly higher in the study group, but the differences only reached statistical significance when patients on chronic medication were omitted. Prolactin was significantly greater in the Down's syndrome patients than in the controls, both over the entire sample and in the subgroup of men with Down's syndrome, with P-values of around 0.001. The elevation of prolactin was not due to medication and did not correlate to thyroid function or difficulties during blood sampling. In females, the difference was not statistically significant. Laboratory tests that may be associated with endocrine disease or might indicate disease which could influence the endocrine status, were also included in this study. Compared with the controls, ESR, creatinine and uric acid levels were higher in Down's syndrome patients, while albumin was lower, all with P-values lower than 0.001. Vitamin B12 was moderately lower in Down's syndrome patients than in controls (P less than 0.05).

  18. Growth Hormone Response after Administration of L-dopa, Clonidine, and Growth Hormone Releasing Hormone in Children with Down Syndrome.

    Science.gov (United States)

    Pueschel, Seigfried M.

    1993-01-01

    This study of eight growth-retarded children with Down's syndrome (aged 1 to 6.5 years) found that administration of growth hormone was more effective than either L-dopa or clonidine. Results suggest that children with Down's syndrome have both anatomical and biochemical hypothalamic derangements resulting in decreased growth hormone secretion and…

  19. Guillain?Barr? syndrome complicating dengue fever: Two case reports

    OpenAIRE

    Boo, Yang Liang; Aris, Mohd Azman M.; Chin, Pek Woon; Sulaiman, Wan Aliaa Wan; Basri, Hamidon; Hoo, Fan Kee

    2015-01-01

    Guillain?Barr? syndrome is a rare neurological manifestation associated with dengue infection. More common antecedent infections include Campylobacter jejuni and Cytomegalovirus infection. Here, we report two cases of Guillain?Barr? syndrome complicating dengue infection.

  20. Antiphospholipid Syndrome Complicated by Unilateral Pleural Effusion

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    Yasutaka Mitamura

    2013-07-01

    Full Text Available Antiphospholipid syndrome (APS with pleural effusion is extremely rare. A 75-year-old man was admitted to our hospital for spreading erythema on his trunk and extremities, as well as dyspnea. One year before admission, he had visited us with a 1-year history of erythema and purpura on his legs and occasional fever. Given the diagnosis of APS, we initiated a combination therapy of aspirin and warfarin, but the skin lesions had gradually worsened. A biopsy specimen revealed marked thrombosis in the dermal and subcutaneous small vessels. In addition, chest X-ray and computed tomography demonstrated a large pleural effusion in the left lung. He underwent repeated drainage of the pleural effusion but the effusion recurred. We added oral prednisolone 30 mg daily to his prior anticoagulant therapy. The skin lesions and pleural effusion rapidly improved and disappeared without any complication. Corticosteroids might be a choice of treatment for intractable pleural effusion in APS patients.

  1. Growth hormone deficiency: an unusual presentation of floating harbor syndrome.

    Science.gov (United States)

    Galli-Tsinopoulou, Assimina; Kyrgios, Ioannis; Emmanouilidou, Eleftheria; Maggana, Ioanna; Kotanidou, Eleni; Kokka, Paraskevi; Stylianou, Charilaos

    2011-01-01

    Floating-Harbor Syndrome (FHS) is a very rare condition of unknown etiology characterized by short stature, delayed bone age, characteristic facial features, delayed language skills and usually normal motor development. This syndrome has only once been associated with growth hormone deficiency and precocious puberty in the same patient. We describe a 5 4/12 year-old girl with the typical features of FHS in whom growth hormone deficiency was diagnosed and two years later central precocious puberty was noted. The patient showed a good response to human recombinant growth hormone as well as gonadotropin releasing hormone analogue treatment.

  2. Slipped Capital Femoral Epiphysis as a Complication of Growth Hormone Therapy

    Directory of Open Access Journals (Sweden)

    Shuo-Yu Wang

    2007-01-01

    Full Text Available Slipped capital femoral epiphysis (SCFE is a rare complication of growth hormone (GH therapy. Here, we report three patients who developed SCFE during GH therapy. The first two patients had hypopituitarism and had started GH therapy at the age of 15 years 6 months and 13 years 9 months, respectively. SCFE developed 4 years and 1 year after GH therapy, respectively. The third patient had Prader-Willi syndrome with obesity and hypogonadism and began GH therapy at the age of 12 years and 11 months. SCFE developed 2 months after starting GH therapy. Pain over the hip joints or over the knees is an early sign of SCFE. Despite recommendation, none of the three patients continued GH therapy. A high index of suspicion during GH therapy in patients at high risk of SCFE is important for early diagnosis and appropriate management. [J Formos Med Assoc 2007;106(2 Suppl:S46-S50

  3. Pregnancy complications in polycystic ovary syndrome patients.

    Science.gov (United States)

    Katulski, Krzysztof; Czyzyk, Adam; Podfigurna-Stopa, Agnieszka; Genazzani, Andrea R; Meczekalski, Blazej

    2015-02-01

    Infertility is a widely disputed problem affecting patients suffering from polycystic ovary syndrome (PCOS). As a serious dysfunction, it frequently occurs in PCOS patients. It is, therefore, important to devote more attention to pregnancy in PCOS sufferers. According to various data, the risk of miscarriage in PCOS women is three times higher than the risk of miscarriage in healthy women. Unfortunately, the risk of most frequent pregnancy pathologies is also higher for PCOS patients, as gestational diabetes (GD), pregnancy-induced hypertension and pre-eclampsia, and small for gestational age (SGA) children. Impaired glucose tolerance and GD in pregnant PCOS patients occur more frequently than in healthy women. A quadruple increase in the risk of pregnancy-induced hypertension linked to arterial wall stiffness has also been observed in PCOS patients. The risk of pre-eclampsia, the most severe of all complications, is also four times higher in those suffering from PCOS. Pre-eclampsia is also more frequent in patients presenting additional risk factors accompanying PCOS, such as obesity or GD. At that point, it should be mentioned that PCOS patients are under 2.5 higher risk of giving birth to SGA children than healthy women. It appears that SGA can be linked to insulin resistance and insulin-dependent growth dysfunction. Therefore, PCOS pregnant women are patients of special obstetrical care.

  4. Compartment syndrome and popliteal vascular injury complicating unicompartmental knee arthroplasty

    NARCIS (Netherlands)

    Kort, Nanne Pieter; Van Raay, Jos J. J. A. M.; van Horn, Jim R.

    Popliteal vascular injury and the compartment syndrome of the leg are rare but important complications of knee arthroplasties. Early diagnosis and treatment are of paramount importance in preventing the devastating complications of these conditions. To our knowledge, these complications have not

  5. Hormonal effect on the inner ear : two endocrine syndromes

    OpenAIRE

    Bonnard, Åsa

    2017-01-01

    Hearing loss is a major problem in our society with more than 5 % of the population world-wide suffering from disabling hearing loss according to the WHO. There are many endocrine syndromes associated with hearing loss. In both Turner syndrome and Pendred syndrome hearing loss is a prominent feature often leading to a need for hearing rehabilitation with hearing aids or cochlear implants. Hormonal treatment might also affect the inner ear directly and studies have shown a negative effect on h...

  6. Eye Complications of Acquired Immune Deficiency Syndrome (AIDS ...

    African Journals Online (AJOL)

    Skoludek_R

    Eye Complications of Acquired Immune Deficiency Syndrome (AIDS). Part 1. Ocular surface and anterior segment manifestations. Wani MGa, Eye Department, Mutare Provincial Hospital, P O Box 2481, Mutare, Zimbabwe. Introduction. Acquired Immune Deficiency Syndrome (AIDS) is the leading cause of sickness and ...

  7. [Reye's syndrome--a severe complication of salicylate therapy].

    Science.gov (United States)

    Vengerovskiĭ, A I; Baturina, N O; Saratikov, A S

    2000-01-01

    Clinical and experimental data are reviewed on the Reye's syndrome--a heavy complication accompanying the therapy of viral infections in children by salicylates. Disorders in the bioenergetics of fatty acid oxidation and ammonia utilization are considered in the context of clinical manifestations of the Reye's syndrome.

  8. Growth hormone positive effects on craniofacial complex in Turner syndrome.

    Science.gov (United States)

    Juloski, Jovana; Dumančić, Jelena; Šćepan, Ivana; Lauc, Tomislav; Milašin, Jelena; Kaić, Zvonimir; Dumić, Miroslav; Babić, Marko

    2016-11-01

    Turner syndrome occurs in phenotypic females with complete or partial absence of X chromosome. The leading symptom is short stature, while numerous but mild stigmata manifest in the craniofacial region. These patients are commonly treated with growth hormone to improve their final height. The aim of this study was to assess the influence of long-term growth hormone therapy on craniofacial morphology in Turner syndrome patients. In this cross-sectional study cephalometric analysis was performed on 13 lateral cephalograms of patients with 45,X karyotype and the average age of 17.3 years, who have received growth hormone for at least two years. The control group consisted of 13 Turner syndrome patients naive to growth hormone treatment, matched to study group by age and karyotype. Sixteen linear and angular measurements were obtained from standard lateral cephalograms. Standard deviation scores were calculated in order to evaluate influence of growth hormone therapy on craniofacial components. In Turner syndrome patients treated with growth hormone most of linear measurements were significantly larger compared to untreated patients. Growth hormone therapy mainly influenced posterior face height, mandibular ramus height, total mandibular length, anterior face height and maxillary length. While the increase in linear measurements was evident, angular measurements and facial height ratio did not show statistically significant difference. Acromegalic features were not found. Long-term growth hormone therapy has positive influence on craniofacial development in Turner syndrome patients, with the greatest impact on posterior facial height and mandibular ramus. However, it could not compensate X chromosome deficiency and normalize craniofacial features. Copyright © 2016 Elsevier Ltd. All rights reserved.

  9. Fusobacterium necrophorum pharyngitis complicated by Lemierre's syndrome

    National Research Council Canada - National Science Library

    Fortini, Alberto; Boccadori, Costanza; Oggioni, Roberto; Faraone, Antonio; Mangani, Valerio; Nenci, Gabriele

    2016-01-01

    .... Blood cultures were positive for Fusobacterium necrophorum. CT scan detected a parapharyngeal abscess, a right internal jugular vein thrombosis, and multiple bilateral lung abscesses, suggesting the diagnosis of Lemierre's syndrome...

  10. Growth hormone deficiency and diabetes insipidus as a complication of endoscopic third ventriculostomy.

    Science.gov (United States)

    Tafuri, Kimberly S; Wilson, Thomas A

    2012-12-01

    Endoscopic third ventriculostomy (ETV) has become the procedure of choice for the treatment of obstructive hydrocephalus in children and adults. Endocrinological complications of ETV in children are rare. Diabetes insipidus (DI) is the most common and accounts for only 0.5% of complications from ETV. The majority of documented cases are transient. To date, there are no documented cases of multiple pituitary hormone deficiencies. We present here a 6-year-old girl with growth hormone deficiency and permanent DI which developed as a complication of ETV. This patient is unique in both demonstrating multiple pituitary hormone deficiencies and the classical triphasic response of DI after ETV. We postulate that these complications were caused by compression of the pituitary stalk and hypothalamic injury during the procedure. We compare our case presentation to experimental studies conducted in rats.

  11. [Obstructive sleep apnea syndrome: Metabolic complications].

    Science.gov (United States)

    Frija-Orvoën, E

    2016-06-01

    Strongly linked to the presence of obesity, the obstructive sleep apnea syndrome is an independent risk factor for abnormalities of glucose metabolism ranging from simple impaired glucose tolerance to type 2 diabetes. It is also a risk factor for dyslipidemia, metabolic syndrome and non-alcoholic fatty liver disease. The pathological mechanisms underlying these associations remain to be precisely discovered, but intermittent hypoxia is probably one of the major factors. The place of obstructive apnea treatment in the management of metabolic conditions remains unclear. Copyright © 2016 SPLF. Published by Elsevier Masson SAS. All rights reserved.

  12. Noonan syndrome and Turner syndrome patients respond similarly to 4 years' growth-hormone therapy

    DEFF Research Database (Denmark)

    Lee, Peter A; Ross, Judith L; Pedersen, Birgitte Tønnes

    2015-01-01

    BACKGROUND: Turner syndrome (TS) and Noonan syndrome (NS) are distinct syndromes associated with short stature and other similar phenotypic features. We compared the responses to growth hormone (GH) therapy of TS and NS patients enrolled in the NordiNet® International Outcome Study (IOS) or the A...

  13. Growth Hormone Therapy in Adults with Prader-Willi Syndrome

    Science.gov (United States)

    Vogt, Karen S.; Emerick, Jill E.

    2015-01-01

    Prader-Willi syndrome (PWS) is characterized by hyperphagia, obesity if food intake is not strictly controlled, abnormal body composition with decreased lean body mass and increased fat mass, decreased basal metabolic rate, short stature, low muscle tone, cognitive disability, and hypogonadism. In addition to improvements in linear growth, the benefits of growth hormone therapy on body composition and motor function in children with PWS are well established. Evidence is now emerging on the benefits of growth hormone therapy in adults with PWS. This review summarizes the current literature on growth hormone status and the use of growth hormone therapy in adults with PWS. The benefits of growth hormone therapy on body composition, muscle strength, exercise capacity, certain measures of sleep-disordered breathing, metabolic parameters, quality of life, and cognition are covered in detail along with potential adverse effects and guidelines for initiating and monitoring therapy. PMID:28943608

  14. Growth Hormone Therapy in Adults with Prader-Willi Syndrome

    Directory of Open Access Journals (Sweden)

    Karen S. Vogt

    2015-04-01

    Full Text Available Prader-Willi syndrome (PWS is characterized by hyperphagia, obesity if food intake is not strictly controlled, abnormal body composition with decreased lean body mass and increased fat mass, decreased basal metabolic rate, short stature, low muscle tone, cognitive disability, and hypogonadism. In addition to improvements in linear growth, the benefits of growth hormone therapy on body composition and motor function in children with PWS are well established. Evidence is now emerging on the benefits of growth hormone therapy in adults with PWS. This review summarizes the current literature on growth hormone status and the use of growth hormone therapy in adults with PWS. The benefits of growth hormone therapy on body composition, muscle strength, exercise capacity, certain measures of sleep-disordered breathing, metabolic parameters, quality of life, and cognition are covered in detail along with potential adverse effects and guidelines for initiating and monitoring therapy.

  15. Correlation between Hormonal Statuses and Metabolic Syndrome in Postmenopausal Women.

    Directory of Open Access Journals (Sweden)

    Saeideh Ziaei

    2013-06-01

    Full Text Available To compare the hormonal status in postmenopausal women with and without metabolic syndrome.In this cross sectional study 110 postmenopausal women were enrolled. Participants completed a questionnaire and underwent a medical exam and serum evaluation for serum lipids including cholesterol (Chol, high density lipoprotein cholesterol (HDL, low density lipoprotein (LDL, triglyceride (TG, fasting blood sugar (FBS, sex hormone binding globulin (SHBG, estradiol and testosterone. Metabolic syndrome was defined according to the definition of the National Cholesterol Education Program- Adult Treatment Panel III. In this study P value less than 0.05 was accepted as significant.There were significant differences between the two groups of participants with and without metabolic syndrome in age, years after menopause, BMI, weight, SHBG and testosterone (p< 0.01.SHBG and testosterone are the most significant correlated factors to metabolic syndrome in postmenopausal women.

  16. Cockayne syndrome: a case with hyperinsulinemia and growth hormone deficiency.

    Science.gov (United States)

    Park, S K; Chang, S H; Cho, S B; Baek, H S; Lee, D Y

    1994-02-01

    Cockayne syndrome is a rare autosomal recessive disorder of childhood characterized by cachectic dwarfism with senile-like appearance, mental retardation, photosensitive dermatitis, loss of adipose tissue, pigmentary degeneration of retina, microcephaly, deafness, skeletal and neurologic abnormalities. We describe here an 18 year old boy with Cockayne syndrome who had, in addition to the typical features of the disorder, fasting hyperinsulinemia and growth hormone deficiency.

  17. [Bouveret syndrome--a rare gallstone complication].

    Science.gov (United States)

    Hürlimann, R; Enzler, M; Binswanger, R O; Meyenberger, C

    1995-08-01

    A 62 year old woman presented with the symptoms of a gastric outlet obstruction together with severe metabolic hypochloremic alcalosis. A gallstone in the duodenum with gastric outlet obstruction was diagnosed by abdominal ultrasonography. In a one-stage surgical procedure, cholecystectomy, enterolithotomy and repair of the cholecytoduodenal fistula were performed. The postoperative course was uneventful. Pathogenesis, clinical findings, diagnostic procedures and therapeutic options of gastric outlet obstruction secondary to a gallstone impacted in the duodenal bulb (Bouveret's Syndrome) are discussed.

  18. Cardiovascular complications in the acquired immunodeficiency syndrome.

    Science.gov (United States)

    Barbaro, Giuseppe; Silva, Erika Ferrari Rafael da

    2009-01-01

    The introduction of highly active antiretroviral therapy (HAART) has significantly improved the clinical outcome of HIV disease, with increased survival rates. However, the introduction of HAART has generated a contrast in the cardiac manifestations of AIDS. In developed countries, we observed an approximate 30% reduction in the prevalence of HIV-associated cardiomyopathy, possibly related to a reduction of opportunistic infections and myocarditis. In developing countries, however, where the availability of HAART is limited and the pathogenic impact of nutritional factors is significant, we observed an increase of approximately 32% in the prevalence of HIV-associated cardiomyopathy and a related high mortality rate from congestive heart failure. Also, some HAART regimens in developed countries, especially those including protease inhibitors, have been shown to cause, in a high proportion of HIV-infected patients, a iatrogenic metabolic syndrome (HIV-lipodystrophy syndrome).This is associated with an increased risk of atherosclerosis-related cardiovascular events even in young HIV-infected people. A better understanding of the molecular mechanisms responsible for this syndrome will lead to the discovery of new drugs that will reduce cardiovascular risk in HIV-infected patients receiving HAART.

  19. Medullary Thyroid Carcinoma with Ectopic Adrenocorticotropic Hormone Syndrome

    Directory of Open Access Journals (Sweden)

    Hong Seok Choi

    2014-03-01

    Full Text Available Ectopic adrenocorticotropic hormone (ACTH syndrome is caused most frequently by a bronchial carcinoid tumor or by small cell lung cancer. Medullary thyroid carcinoma (MTC is a rare etiology of ectopic ACTH syndrome. We describe a case of Cushing syndrome due to ectopic ACTH production from MTC in a 48-year-old male. He was diagnosed with MTC 14 years ago and underwent total thyroidectomy, cervical lymph node dissection and a series of metastasectomies. MTC was confirmed by the pathological examination of the thyroid and metastatic mediastinal lymph node tissues. Two years after his last surgery, he developed Cushingoid features, such as moon face and central obesity, accompanied by uncontrolled hypertension and new-onset diabetes. The laboratory results were compatible with ectopic ACTH syndrome. A bilateral adrenalectomy improved the clinical and laboratory findings that were associated with Cushing syndrome. This is the first confirmed case of ectopic ACTH syndrome caused by MTC in Korea.

  20. Comparison of the Levels of LH and FSH, TSH, Prolactin, Progesterone and Estradiol Hormones between Iranian Infertile Women with Polycystic Ovary Syndrome and Healthy Women

    Directory of Open Access Journals (Sweden)

    Amir Hossein Hashemi

    2016-12-01

    Full Text Available Polycystic ovary syndrome (PCOS with the prevalence of 5 to 7% among Iranian women is a leading cause of infertility and endocrine disorder. Metabolic disorders such as increased levels of LH and FSH hormones in these patients was common and influences health of women with PCOS in long-term. Treatment of female infertility and other complications in many cases need to regulate hormones and receive exogenous hormone, and then the effect of female hormones on the disease is very important. In this study, levels of Luteinizing hormone (LH and Follicle stimulating hormone (FSH and other female hormones among Iranian women with PCOS and infertility and healthy people were measured in this regard and values were compared. The result of this study showed that LH and progesterone hormone levels were significantly different in this syndrome than healthy women.

  1. Pregnancy complicated by cyclic vomiting syndrome successfully treated with amitriptyline.

    Science.gov (United States)

    Tamai, Hanako; Kinugasa, Masato

    2015-06-01

    Some reports suggest that pregnancy leads to exacerbation of cyclic vomiting syndrome. We report a case of pregnancy complicated by cyclic vomiting syndrome that was successfully resolved with amitriptyline, with the subsequent birth of a healthy term neonate. A 27-year-old pregnant woman with cyclic vomiting syndrome was referred to our hospital at 12 weeks of gestation. After excluding other diagnoses, we administered amitriptyline, which had been discontinued temporarily at the time of pregnancy diagnosis. Once a therapeutic dose was achieved, her vomiting attacks ceased and the remainder of her prenatal course was uneventful. At 40 weeks of gestation, she delivered a female neonate. Prevention of vomiting attacks using appropriate agents is essential for the management of pregnancies complicated by cyclic vomiting syndrome.

  2. Progressive sliding hiatal hernia as a complication of Menkes' syndrome.

    Science.gov (United States)

    Shiihara, Takashi; Kato, Mitsuhiro; Honma, Tomomi; Kimura, Toshiyuki; Matsunaga, Akira; Kodama, Hiroko; Hayasaka, Kiyoshi

    2002-05-01

    We report a 19-month-old boy with Menkes' syndrome that was complicated by a progressive sliding hiatal hernia. He presented with convulsions, developmental delay, elongation and tortuosity of major cerebral arteries, and diverticulae of the bladder at 4 months of age. Based on the diagnosis of Menkes' syndrome, treatment with intravenous or subcutaneous copper-histidine administration was initiated at 6 months of age. At 13 months of age, he vomited frequently owing to sliding hiatal hernia, which progressed rapidly and required surgical treatment. Connective tissue abnormalities are characteristic complications of Menkes' syndrome. Sliding hiatal hernia is probably one of the connective tissue manifestations and should be carefully evaluated in patients with Menkes' syndrome demonstrating recurrent gastrointestinal and/or respiratory symptoms.

  3. Oxandrolone in growth hormone-treated girls with Turner syndrome

    NARCIS (Netherlands)

    Menke, Leonie Alexandra

    2010-01-01

    Turner syndrome (TS) is a disorder in females that is caused by the complete or partial absence of the second sex chromosome. The main characteristics are gonadal dysgenesis and short stature, with adult patients being on average 20 cm shorter than healthy women. Growth hormone (GH) therapy

  4. Growth hormone and the heart in Noonan syndrome

    NARCIS (Netherlands)

    Noordam, C.

    2009-01-01

    BACKGROUND: The clinical hallmarks of Noonan syndrome (NS) are facial dysmorphism, short stature and cardiac defects. As one of the common cardiac defects in NS is hypertrophic cardiomyopathy, there have been concerns regarding cardiac safety since the start of human growth hormone (hGH) therapy for

  5. Hormonal contraception in obesity, the metabolic syndrome, and diabetes

    DEFF Research Database (Denmark)

    Skouby, S.O.

    2010-01-01

    The rate of obesity worldwide is currently at epidemic proportions. As part of obesity, the metabolic syndrome describes a clustering of metabolic abnormalities that increase the cardiovascular and diabetes risk. In particular, women from developing countries have diabetes in the reproductive age...... diabetes, hormonal contraception should therefore be part of the highly needed preconception care and metabolic control...

  6. Hormonal Contraception in obestiy, the metabolic syndrome, and diabetes

    DEFF Research Database (Denmark)

    Skouby, Sven O.

    2010-01-01

    The rate of obesity worldwide is currently at epidemic proportions. As part of obesity, the metabolic syndrome describes a clustering of metabolic abnormalities that increase the cardiovascular and diabetes risk. In particular, women from developing countries have diabetes in the reproductive age...... diabetes, hormonal contraception should therefore be part of the highly needed preconception care and metabolic control...

  7. [Arteriovenous fistula in patients with a complicated postphlebitic syndrome].

    Science.gov (United States)

    Quiñones Castro, M; Fernández de la Vara Sánchez, L; Perera González, F; Alvarez Sánchez, J A

    1991-01-01

    An investigation, which goal is to evidencing AV fistulas in patients with a complicated postthrombotic syndrome, as well as to determinate the efficacy of noninvasive methods used for the study of AV fistulas in such patients, is presented. So, patients with a complicated postphlebitic syndrome interned into the Instituto de Angiología were studied. Twenty-five patients were included in the study, 9 of these patients (36%) had arteriovenous fistulas, arteriographically evidenced. Haemodynamic study do not support the evidence of such communications.

  8. Maternal and fetal outcome of pregnancy complicated by HELLP syndrome.

    Science.gov (United States)

    Gasem, Turki; Al Jama, Fathia E; Burshaid, Sameera; Rahman, Jessica; Al Suleiman, Suleiman A; Rahman, Mohammad S

    2009-12-01

    The study evaluated the maternal and fetal outcome in 64 pregnancies complicated by HELLP syndrome. A retrospective analysis of the medical records was performed of patients with HELLP syndrome managed at this tertiary Obstetric unit between January 1996 and December 2005, who were admitted for preeclamsia/eclampsia and had documented evidence of hemolysis, elevated liver enzymes and low platelet count. Maternal and neonatal complications were recorded and analyzed. The incidence of HELLP syndrome in the study was 8.3%. Mean gestational age at delivery was 32.4 +/- 4.2 weeks and mean birth weight was 1851 +/- 810 g. Forty-two percent of the patients had deliveries <32 weeks and 28% IUGR. Respiratory distress syndrome was the main indication for NICU admissions (33.9%). The PNM rate was 20%. Maternal morbidity rate was 34%. The most common maternal complications were abruptio placentae (36.4%) and DIC (31.8%). There was no maternal death. Once the diagnosis of HELLP syndrome is confirmed, the management depends on several obstetric and maternal variables like gestational age, severity of laboratory abnormalities and fetal status. As soon as the maternal condition is stabilized and fetal assessment is obtained, prompt delivery of the fetus is indicated. It is not yet established whether expectant management in preterm pregnancies with HELLP syndrome would improve perinatal outcome.

  9. [Obesity, hormonal and metabolic abnormalities in adolescent girls with polycystic ovary syndrome].

    Science.gov (United States)

    Ságodi, László; Lombay, Béla; Vámosi, Ildikó; Barkai, László

    2013-08-04

    Polycystic ovary syndrome is associated with metabolic abnormalities, such as dyslipidemia, obesity, glucose intolerance, which are also components of the metabolic syndrome. Central obesity and insulin resistance appear to play an important role in the pathogenesis of polycystic ovary syndrome, perhaps via subsequent steroidogenic dysregulation. The aim of the authors was to assess metabolic and hormonal abnormalities in adolescent girls with polycystic ovary syndrome. The study included 52 adolescents diagnosed with polycystic ovary syndrome based on the Rotterdam criteria. Anthropometric, hormonal and metabolic parameters were evaluated among all subjects. 20 healthy, age-matched, non-obese, regularly menstruating girls were used as controls. Of the 52 patients, 15 patients were born with low-birth-weight and 37 patients were born with normal birth weight. Oral glucose tolerance test was performed in all patients and controls. The age of patients was 16.8±3.1 years, and the age of controls was 16.95±2.1 years. Among patients with polycystic ovary syndrome the prevalence of overweight and obesity was 35% (n = 18), while impaired fasting glucose occurred in one patient, impaired glucose tolerance in 8 patients, insulin resistance in 25 patients and metabolic syndrome in 12 patients. Serum triglyceride levels in patients and controls were 1.4±0.8 and 0.9±0.3 mmol/l, respectively (pMetabolic abnormalities and obesity were more severe and more frequent in patients with low-birth-weight compared to those born with normal weight. There was a negative correlation between birth weight and body mass index SDS values and a positive correlation between fasting insulin levels and body mass index SDS (r = 0.37) in patients born with low-birth-weight. Abnormal glucose metabolism is frequently present in adolescents with polycystic ovary syndrome. It is possible that early diagnosis of polycystic ovary syndrome in adolescence may prevent some of the long-term complications

  10. Fahr Syndrome Unknown Complication: Overactive Bladder

    Directory of Open Access Journals (Sweden)

    Devrim Tuglu

    2014-01-01

    Full Text Available A 38-year-old male patient was admitted to our outpatient department because of frequency and urgency incontinence. During evaluation it was detected that the patient was suffering from frequency which was progressive for one year, feeling of incontinence, and urgency incontinence. There was no urologic pathology detected in patient’s medical and family history. Neurologic consultation was requested due to his history of boredom, reluctance to do business, balance disorders, and recession for about 3 years. Brain computerized tomography (CT scan revealed that amorphous calcifications were detected in the bilaterally centrum semiovale, basal ganglia, capsula interna, thalami, mesencephalon, pons and bulbus, and the bilateral cerebellar hemispheres. We have detected spontaneous neurogenic detrusor overactivity without sphincter dyssynergia after evaluating the voiding diary, cystometry, and pressure flow study. We consider the detrusor overactivity which occurred one year after the start of the neurological symptoms as the suprapontine inhibition and damage in the axonal pathways in the Fahr syndrome.

  11. Quadrilateral space syndrome: a rare complication of thoracic surgery.

    Science.gov (United States)

    Nishimura, Motohiro; Kobayashi, Masao; Hamagashira, Kenichiro; Noumi, Shinpachiro; Ito, Kazuhiro; Kato, Daishiro; Shimada, Junichi

    2008-10-01

    We report a case of quadrilateral space syndrome related to thoracic surgery. A 21-year-old man underwent video-assisted thoracic surgery for a left-sided pneumothorax. After the operation, he presented with difficulties in left arm abduction and paresthesia over the lateral aspect of the shoulder and upper arm. Deltoid muscle atrophy and tenderness over the quadrilateral space were also observed. On further examination, he was diagnosed with isolated paralysis of the axillary nerve, the so-called quadrilateral space syndrome. This is a rare complication, but it interferes with the activities of daily living, and thus one should pay attention to this syndrome.

  12. Superior Mesenteric Artery Syndrome: An Infrequent Complication of Scoliosis Surgery

    Directory of Open Access Journals (Sweden)

    Metin Keskin

    2014-01-01

    Full Text Available Superior mesenteric artery syndrome is a rare condition that causes a proximal small intestinal obstruction due to contraction of the angle between the superior mesenteric artery and the aorta. Scoliosis surgery is one of the 15 reasons for superior mesenteric artery syndrome, which can present with acute or chronic manifestations. Although conservative treatment is usually possible, surgical treatment is required in certain cases that cannot be treated using conservative methods. In this paper, we describe a patient who developed superior mesenteric artery syndrome after scoliosis surgery and was treated with duodenojejunostomy due to failure and complications of conservative treatment.

  13. Spontaneous liver rupture in pregnancy complicating HELLP syndrome: case report

    Directory of Open Access Journals (Sweden)

    Sariyeh Golmahammadlou

    2014-10-01

    Conclusion: Spontaneous liver rupture associated with HELLP syndrome is a rare and life-threatening complication of pregnancy. Unruptured liver hematoma is also a rare condition during pregnancy with a very difficult diagnosis. Using clinical diagnostic tests such as CT scan or MRI would be helpful to improve clinical outcomes.

  14. Fatal Lemierre's syndrome as a complication of chronic otitis media ...

    African Journals Online (AJOL)

    The patient developed septic shock and microemboli, and subsequently died. Conclusion. This case report illustrates that untreated chronic middle ear infection can lead to potentially fatal complications such as Lemierre's syndrome, and emphasises the importance of timeous treatment of chronic middle ear pathology.

  15. Arterial complications of vascular Ehlers-Danlos syndrome.

    Science.gov (United States)

    Eagleton, Matthew J

    2016-12-01

    Vascular Ehlers-Danlos syndrome (EDS) is a relatively rare genetic syndrome that occurs owing to disorders in the metabolism of fibrillary collagen. These defects affect the soft connective tissues resulting in abnormalities in the skin, joints, hollow organs, and blood vessels. Patients with these defects frequently present at a young age with spontaneous arterial complications involving the medium-sized arteries. Complications involving the hollow organs, such as spontaneous colonic perforation, are observed as well. Given the fragility of the soft tissue, open and endovascular intervention on patients with vascular EDS is fraught with high complication rates. A PubMed search was performed to identify manuscripts published related to vascular EDS. This search included more than 747 articles. These findings were cross-referenced using key terms, including endovascular, embolization, surgery, genetics, pathophysiology, connective tissue disorders, vascular complications, systematic review, type III collagen, and COL3A1. The references in key articles and review articles were evaluated for additional resources not identified in the PubMed search. Care must be taken to balance the risk of intervention vs the risk of continued observation. Life-threatening hemorrhage, however, mandates intervention. With careful, altered approaches to tissue handling, endovascular approaches may provide a safer option for managing the arterial complications observed in patients with vascular EDS. Additional hope may also be found in the use of pharmacologic agents that reduce the incidence and severity of the arterial complications. Copyright © 2016 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.

  16. Shrinking lung syndrome complicating pediatric systemic lupus erythematosus

    Energy Technology Data Exchange (ETDEWEB)

    Burns, Natalie S. [University of Washington Medical Center, Department of Radiology, Seattle, WA (United States); Stevens, Anne M. [Seattle Children' s Hospital, Division of Rheumatology, Department of Pediatrics, Seattle, WA (United States); Iyer, Ramesh S. [University of Washington School of Medicine, Seattle Children' s Hospital, Department of Radiology, Seattle, WA (United States)

    2014-10-15

    Systemic lupus erythematosis (SLE) can affect the lungs and pleura, usually manifesting with pleural effusions or diffuse parenchymal disease. A rare manifestation of SLE is shrinking lung syndrome, a severe restrictive respiratory disorder. While pleuropulmonary complications of pediatric SLE are common, shrinking lung syndrome is exceedingly rare in children. We present a case of a 13-year-old girl previously diagnosed with lupus, who developed severe dyspnea on exertion and restrictive pulmonary physiology. Her chest radiographs on presentation demonstrated low lung volumes, and CT showed neither pleural nor parenchymal disease. Fluoroscopy demonstrated poor diaphragmatic excursion. While shrinking lung syndrome is described and studied in adults, there is only sparse reference to shrinking lung syndrome in children. (orig.)

  17. Oxandrolone in growth hormone-treated girls with Turner syndrome

    OpenAIRE

    Menke, Leonie Alexandra

    2010-01-01

    Turner syndrome (TS) is a disorder in females that is caused by the complete or partial absence of the second sex chromosome. The main characteristics are gonadal dysgenesis and short stature, with adult patients being on average 20 cm shorter than healthy women. Growth hormone (GH) therapy increases adult height with 5 to 12 cm and the addition of the weak androgen oxandrolone (Ox) may further increase adult height. This thesis describes the results of the first randomized, double-blind, pla...

  18. Growth Hormone Treatment in Turner's Syndrome: A Real World Experience

    OpenAIRE

    Vijay Sheker Reddy Danda; Sreedevi, P.; Arun, G.; P Srinivas Rao

    2017-01-01

    Objective: Short stature is a universal clinical feature of Turner's syndrome (TS). Growth failure begins in fetal life, and adults with TS are on an average 20 cm shorter than the normal female population. Since there is a paucity of data from India regarding the effect of growth hormone (GH) on TS patients, we retrospectively analyzed the data of TS patients who are on GH treatment. Methods: This hospital-based observational retrospective study was conducted in a tertiary care hospital of H...

  19. The syndrome of inappropriate antidiuretic hormone: prevalence, causes and consequences.

    LENUS (Irish Health Repository)

    Hannon, M J

    2010-06-01

    Hyponatraemia is the commonest electrolyte abnormality found in hospital inpatients, and is associated with a greatly increased morbidity and mortality. The syndrome of inappropriate antidiuretic hormone (SIADH) is the most frequent cause of hyponatraemia in hospital inpatients. SIADH is the clinical and biochemical manifestation of a wide range of disease processes, and every case warrants investigation of the underlying cause. In this review, we will examine the prevalence, pathophysiology, clinical characteristics and clinical consequences of hyponatraemia due to SIADH.

  20. Bone cement implantation syndrome: A dreaded complication during arthroplasty

    Directory of Open Access Journals (Sweden)

    Jaspreet Kaur Nagpal

    2015-01-01

    Full Text Available Bone cement implantation syndrome is a known cause of intraoperative morbidity and mortality in patients undergoing cemented arthroplasty. It is a rare but potentially fatal complication. A case of elderly female patient is reported who developed this complication during hemi arthroplasty of right hip. Within a few minutes of cementation, she developed severe hemodynamic derangement and shock with hypoxemia. Aggressive and early resuscitative measures led to a favorable outcome. Risk factors, etiopathogenesis and management of this condition are discussed in brief.

  1. Slipped capital femoral epiphysis in a patient with Turner syndrome receiving growth hormone therapy.

    Science.gov (United States)

    Nasrallah, Mona P; Der-Boghossian, Asdghig H; Haidar, Rachid K

    2012-01-01

    To report a case of slipped capital femoral epiphysis in a young patient with Turner syndrome (TS) receiving growth hormone therapy and to emphasize the importance of keeping this orthopedic condition in mind during management of this patient group. Clinical, laboratory, and radiographic findings are presented, and risk factors for slipped capital femoral epiphysis are discussed. A child with TS presented for medical assessment because of a limp but with no history of trauma or febrile illness. Growth hormone therapy had been administered for 1 year because of her short stature. Physical examination and pelvic radiography of the patient showed the presence of bilateral slipped capital femoral epiphysis. She underwent bilateral pinning in situ, and growth hormone therapy was terminated. At follow-up after more than 2 years, no sequelae were noted. Patients with TS are at high risk for developing certain orthopedic conditions, such as slipped capital femoral epiphysis. Furthermore, slipped capital femoral epiphysis is a known complication of growth hormone therapy in growing children. A limp, hip pain, knee pain, or thigh pain might be a symptom of slipped capital femoral epiphysis in patients with TS, especially those receiving growth hormone therapy. Prompt recognition and treatment of this condition are important for prevention of sequelae.

  2. Syndrome of inappropriate antidiuretic hormone secretion related to Guillain-Barré syndrome after laparoscopic cholecystectomy

    Directory of Open Access Journals (Sweden)

    Mensure Yilmaz Çakirgöz

    2014-06-01

    Full Text Available Background and objectives: Guillain-Barré Syndrome is one of the most common causes of acute polyneuropathy in adults. Recently, the occurrence of Guillain-Barré Syndrome after major and minor surgical operations has been increasingly debated. In Guillain-Barré syndrome, syndrome of inappropriate antidiuretic hormone secretion and dysautonomy are generally observed after maximal motor deficit. Case report: A 44-year-old male patient underwent a laparoscopic cholecystectomy for acute cholecystitis. After the development of a severe headache, nausea, diplopia, and attacks of hypertension in the early postoperative period, a computer tomography of the brain was normal. Laboratory tests revealed hyponatremia linked to syndrome of inappropriate antidiuretic hormone secretion, the patient's fluids were restricted, and furosemide and 3% NaCl treatment was initiated. On the second day postoperative, the patient developed numbness moving upward from the hands and feet, loss of strength, difficulty swallowing and respiratory distress. Guillain-Barré syndrome was suspected, and the patient was moved to intensive care. Cerebrospinal fluid examination showed 320 mg/dL protein, and acute motor-sensorial axonal neuropathy was identified by electromyelography. Guillain-Barré syndrome was diagnosed, and intravenous immune globulin treatment (0.4 g/kg/day, 5 days was initiated. After 10 days in the intensive care unit, at which the respiratory, hemodynamic, neurologic and laboratory results returned to normal, the patient was transferred to the neurology service. Conclusions: Our case report indicates that although syndrome of inappropriate antidiuretic hormone secretion and autonomic dysfunction are rarely the initial characteristics of Guillain-Barré syndrome, the possibility of postoperative syndrome of inappropriate antidiuretic hormone secretion should be kept in mind. The presence of secondary hyponatremia in this type of clinical presentation may

  3. Decrease of thymic hormone serum level in Cockayne syndrome.

    Science.gov (United States)

    Bensman, A; Dardenne, M; Bach, J F; Valleteau de Mouillac, J; Lasfargues, G

    1982-02-01

    Previous reports concerning children with Cockayne syndrome had described decreased T cell proliferative responses and renal anomalies which could be associated with immunologic disturbances. Herein, the thymic function was evaluated by measuring the serum level of thymic hormone. This serum level was found to be undetectable or decreased in seven cases of Cockayne syndrome. Active serum concentrations varied between 0 and 1/8, whereas normal children of the same age show activity in the range between 1/16 and 1/64. In contrast, T cell function, explored by phytohemagglutinin and Concavalin A responses, and mixed lymphocyte cultures was normal. Whether or not this premature sign of immunological aging is primary or secondary to other manifestations of the syndrome is still difficult to assess.

  4. Transient acute liver failure complicating transurethral resection syndrome.

    Science.gov (United States)

    Tuccori, Marco; Guidi, Benedetta; Montagnani, Sabrina; Fornai, Matteo; Antonioli, Luca; Blandizzi, Corrado; di Paolo, Marco

    2010-09-01

    Transurethral resection (TUR) syndrome, resulting from dilutional hyponatraemia for excessive absorption of irrigating fluid, represents the most relevant complication of transurethral resection of prostate (TURP). Ethanol is used as a tracer in the irrigant solution to monitor fluid absorption with a breathalyser. An unusual case of transient acute liver failure complicating TUR syndrome is reported. A 54-year-old male patient, without risk factors for the development of toxic hepatitis, was subjected to TURP for treatment of benign prostatic hyperplasia. Fluid absorption (2275 ml), estimated by breathalyser, exceeded maximum allowed absorption (2000 ml) only at the end of the surgical intervention. No signs of possible toxicity were evident in the few hours following the intervention. About 10 h after the end of TURP, the patient developed sweating, vomiting and diarrhoea. Laboratory analysis revealed severe hyponatraemia (116 meq/l) with signs of severe liver impairment (total bilirubin 5.8 mg/dl, alanine aminotransferase 56,500 U/l, aspartate aminotransferase 32,700 U/l), kidney failure (serum creatinine 1.93 mg/dl) and serum ethanol levels of 219 mg/dl (0.2%). The patient was treated with acetylcysteine 150 mg/kg i.v. and furosemide 50 mg i.v. Liver and renal functions improved in few days and recovered completely within 30 days. The TUR syndrome observed in this case was probably extravascular in nature, and could have been identified and prevented by measuring ethanol levels 10 min after ending the surgical procedure. The performance of such a test should be strongly recommended to all surgeons. The clinicians attributed the development of liver impairment in this case to ethanol toxicity. However, further studies are warranted to confirm whether hepatic injury can represent a possible complication of TUR syndrome when ethanol solution is used as irrigant fluid.

  5. Syndrome of Inappropriate Secretion of Antidiuretic Hormone Cholestasis and Pericardial Effusion Due to Brucellosis Infection: A Case Report

    Directory of Open Access Journals (Sweden)

    Ahmet Cumhur Dülger

    2010-01-01

    Full Text Available Syndrome of inappropriate secretion of antidiuretic hormone (SIADH is an extremely rare complication of infectious diseases. A rare case of brucellosis complicated by syndrome of inappropriate secretion of antidiuretic hormone (SIADH cholestasis and pericardial involvement is reported. A 27-year-old woman was admitted for fever, abdominal pain, and scleral icterus. Her medical history revealed no recent use of diuretic agents. In addition to cholestasis and elevated liver enzymes, euvolemic hyponatremia, hypouricemia, low plasma osmolality, and high urinary osmolality were also detected. Surrenal and thyroid tests were also within normal range. Echocardiography revealed minimal pericardial effusion with normal cardiac functions. The final diagnosis was SIADH due to Brucellosis. Hyponatremia, cholestasis, and pericardial disease were resolved with effective antibrucellar treatment with streptomycine and doxycycline. After completing treatment of brucellosis, there was not any more evidence of cholestasis and pericardial fluid.

  6. Neurologic complications of cerebral angiography in childhood moyamoya syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Robertson, R.L.; Chavali, R.V.; Robson, C.D.; Barnes, P.D.; Burrows, P.E. [Department of Radiology, Children`s Hospital Medical Center, Boston, MA (United States); Eldredge, E.A. [Department of Anesthesia, Children`s Hospital Medical Center and Harvard Medical School, Boston, MA (United States); Scott, R.M. [Department of Neurosurgery, Children`s Hospital Medical Center and Harvard Medical School, Boston, MA (United States)

    1998-11-01

    Purpose. To determine the incidence of neurologic complications of cerebral angiography in children with moyamoya syndrome (MMS) as compared to children without MMS. Materials and methods. One-hundred-ninety consecutive cerebral angiograms obtained in 152 children were evaluated. Sixty of these angiograms were obtained in 40 children with MMS. Patients underwent neurologic evaluation prior to and after the procedure. For this study, a neurologic complication was defined as any new focal neurologic deficit or alteration in mental status occurring during the procedure or within the ensuing 24 hours. Results. There were 2 neurologic complications within 24 hours of angiography, one in the MMS group and one in the non-MMS group. One patient with MMS became mute following angiography. The symptom resolved within 12 hours. One patient without MMS being examined postoperatively for residual arteriovenous malformation developed intracranial hemorrhage requiring reexploration 12 hours after the angiogram. Using a two-tail Fisher`s exact test, there was no significant statistical difference in the ischemic (P = 0.3) or hemorrhagic (P = 1.0) complication rates between the group of patients with MMS and the non-MMS groups. Conclusion. The risk of a neurologic complication from cerebral angiography in children with MMS is low and not statistically different from the risk in children with other cerebrovascular disorders. (orig.) With 8 tabs., 37 refs.

  7. Netherton Syndrome in a Neonate with Possible Growth Hormone Deficiency and Transient Hyperaldosteronism

    Directory of Open Access Journals (Sweden)

    Chatziioannidis Ilias

    2015-01-01

    Full Text Available Netherton syndrome, a rare autosomal recessive genetic disorder, is classified as an ichthyosiform syndrome. In this report we present the case of a neonate with erythroderma shortly after birth, accompanied by severe hypernatremia, recurrent infections, transient hyperaldosteronism, and signs of growth hormone (GH deficiency. DNA molecular analysis in the SPINK5 gene revealed heterozygosity in our index patient for 238insG and 2468delA frameshift mutations in exons 4 and 26, respectively, in the maternal allele and 1431-12G>A splice-site mutation in intron 15 in the paternal allele as well as the missense variation E420K in homozygous state. Combination of the identified mutations along with transient hyperaldosteronism and possible GH deficiency have not been described before. Accordingly, the importance of early multidisciplinary approach is highlighted, in order to reach accurate diagnosis, initiate prompt treatment, and ensure survival with fewer disease complications.

  8. Incretin hormone secretion in women with polycystic ovary syndrome

    DEFF Research Database (Denmark)

    Svendsen, Pernille Fog; Nilas, Lisbeth; Madsbad, Sten

    2009-01-01

    . Polycystic ovary syndrome (PCOS) is associated with insulin resistance, and the pathophysiologic mechanisms behind PCOS resemble those of type 2 diabetes mellitus; therefore, women with PCOS may have alterations in the incretin hormone response. Metformin is widely used in the treatment of both type 2...... diabetes mellitus and PCOS. Metformin may exert some of its effect on glucose metabolism by increasing GLP-1 biosynthesis and secretion and thereby increasing the incretin effect. The objective of the study was to measure incretin hormone secretion in women with PCOS and to evaluate the effect of metformin...... treatment. Cross-sectional comparison of 40 women with PCOS (19 lean and 21 obese) and 26 healthy control women (9 lean and 17 obese) and longitudinal evaluation of the effects of 8 months of metformin 1000 mg twice daily in women with PCOS were performed. Plasma concentrations of GIP and GLP-1 were...

  9. Refeeding Syndrome: An Important Complication Following Obesity Surgery

    Directory of Open Access Journals (Sweden)

    Sonja Chiappetta

    2016-01-01

    Full Text Available Background: Refeeding syndrome (RFS is an important and well-known complication in malnourished patients, but the incidence of RFS after obesity surgery is unknown and the awareness of RFS in obese patients as a postsurgical complication must be raised. We present a case of RFS subsequent to biliopancreatic diversion in a morbidly obese patient. Case Report: A 48-year-old female patient with a BMI of 41.5 kg/m2 was transferred to our hospital due to Wernicke‘s Encephalopathy in a global malabsorptive syndrome after biliopancreatic diversion. Parenteral nutrition, vitamin supplementation and high-dosed intravenous thiamine supplementation were initiated. After 14 days, the patient started to develop acute respiratory failure, and neurological functions were impaired. Blood values showed significant electrolyte disturbances. RFS was diagnosed and managed according to the NICE guidelines. After 14 days, phosphate levels had returned to normal range, and neurological symptoms were improved. Conclusion: Extreme weight loss following obesity surgery has been shown to be associated with undernutrition. These patients are at high risk for evolving RFS, even though they may still be obese. Awareness of RFS as a postsurgical complication, the identification of patients at risk as well as prevention and correct management should be routinely performed at every bariatric center.

  10. [Psychiatric and psychological complications in obstructive sleep apnea syndrome].

    Science.gov (United States)

    Gałecki, Piotr; Florkowski, Antoni; Zboralski, Krzysztof; Pietras, Tadeusz; Szemraj, Janusz; Talarowska, Monika

    2011-01-01

    It is estimated, that symptoms of obstructive sleep apnea syndrome (OSAS) affect 2 to 4% of the middle-aged population and their prevalence increases with age (over 50% people aged 65 and older suffer from OSAS). Among risk factors of OSAS we can distinguish: the male sex, race, overweight and obesity, thyroid hypofunction, age and alcohol abuse. Obstructive sleep apnea results in the absence (apnea) or reduction (hypopnea) of airflow lasting at least 10 s despite normal respiratory exertion. The apnea and hypopnea result in decreased oxygen saturation levels in the blood (hypoxemia). A number of consequences of OSAS can appear including: disruption of the sleep cycle, fragmentation of the sleep cycle, sleepiness, fatigue, headaches, cognitive impairments, irritability and mood disturbance, the higher risk of accidents at work and car accidents, the decrease of the quality of life and the higher risk of cardiovascular diseases. Besides various psychiatric and psychological complications presented in this review can occur in obstructive sleep apnea syndrome.

  11. Acute Respiratory Distress Syndrome Complicating Strongyloides stercoralis Hyperinfection

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    Ming-Ju Tsai

    2011-03-01

    Full Text Available Strongyloidiasis is endemic in tropic and subtropic areas, but is currently seldom encountered in developed area like Taiwan. We present an elder man with acute respiratory distress syndrome complicating Strongyloides stercoralis hyperinfection. There was no significant clue initially for diagnosing this patient as having S. stercoralis hyperinfection. Neither peripheral eosinophilia nor significant hemoptysis was noted. Bronchoscopy played a critical role to define the unexpected cause of his progressive pulmonary infiltrates. The correct diagnosis was soon made by recognition of the worm in bronchioloalveolar lavage cytology, and specific treatment was initiated promptly. For a septic patient with progressive pulmonary infiltrates, bronchoscopic studies including cytology may be necessary for defining the cause. Hyperinfection strongyloidiasis should be considered as a cause of acute respiratory distress syndrome in immunocompromised patient, especially with the presence of chronic gastrointestinal symptoms.

  12. [Hemolytic uremic syndrome induced by gemcitabine. A poorly recognized complication?].

    Science.gov (United States)

    Graas, M P; Houbiers, G; Demolin, G; Stultiens, A; Focan, C

    2012-12-01

    This report is concerned with the development of an hemolytic uremic syndrome (HUS) in 6 patients (3 males, 3 females, aged 53 to 73) suffering from an advanced cancer and treated by protracted (>= 4 months) infusions of gemcitabine. Over 4 to 14 months, the patients received 13-34 infusions delivering a cumulative dose oscillating between 9 and 29 g/m2. A progressive alteration of renal function preceeded the acute syndrome. After interruption of gemcitabine and symptomatic treatment, the evolution of haemolytic anemia was generally favourable. This was not the case for renal dysfunction: 2 complete and 1 partial resolution of renal insufficiency were noted, but 1 case required chronic dialysis. Based on the authors experience, the frequency of an HUS complication after protracted gemcitabine treatment could be as high as 2.7 %.

  13. Anti-Müllerian hormone and polycystic ovary syndrome.

    Science.gov (United States)

    Bhide, Priya; Homburg, Roy

    2016-11-01

    Anti-Müllerian hormone (AMH) is expressed by the granulosa cells of the pre-antral and small antral follicles in the ovary. It is significantly higher in women with polycystic ovary syndrome (PCOS) due to an increased number of antral follicles and also a higher production per antral follicle. It is postulated to have an inhibitory role in folliculogenesis and may play an important role in the pathophysiology of anovulation associated with PCOS. Measurement of the serum AMH levels is very useful for the identification of PCOS and has been suggested as a diagnostic criterion. An international standardisation of the AMH assay, large population-based studies and a global consensus are needed before its incorporation into routine diagnosis. Serum AMH levels add significant value to the clinical markers for the prediction of hyperresponse to controlled ovarian stimulation for in vitro fertilisation treatment and development of ovarian hyperstimulation syndrome. Copyright © 2016. Published by Elsevier Ltd.

  14. Thromboembolism complicating the treatment of lupus anticoagulant hypoprothrombinemia syndrome.

    Science.gov (United States)

    Vinet, Evelyne; Rich, Eric; Senécal, Jean-Luc

    2006-10-01

    Lupus anticoagulant hypoprothrombinemia syndrome (LAHPS) is a rare disorder characterized by a bleeding tendency due to factor II deficiency associated with the presence of lupus anticoagulant (LAC) autoantibodies. We describe a patient with systemic lupus erythematosus and LAHPS in whom successful treatment of central nervous system bleeding due to severe factor II deficiency was followed by a major thromboembolic complication. Literature review revealed 2 other patients with LAHPS who developed thrombosis resulting from the treatment of factor II deficiency. We suggest that factor II deficiency counterbalances the prothrombotic effect of LAC in LAHPS, and correcting this deficiency may promote thromboembolism.

  15. Guillain-Barre syndrome complicating chikungunya virus infection.

    Science.gov (United States)

    Agarwal, Ayush; Vibha, Deepti; Srivastava, Achal Kumar; Shukla, Garima; Prasad, Kameshwar

    2017-06-01

    Chikungunya virus (CHIKV) is a mosquito-borne alphavirus which presents with symptoms of fever, rash, arthralgia, and occasional neurologic disease. While outbreaks have been earlier reported from India and other parts of the world, the recent outbreak in India witnessed more than 1000 cases. Various systemic and rarely neurological complications have been reported with CHIKV. We report two cases of Guillain-Barré syndrome (GBS) with CHIKV. GBS is a rare neurological complication which may occur after subsidence of fever and constitutional symptoms by several neurotropic viruses. We describe two cases of severe GBS which presented with rapidly progressive flaccid quadriparesis progressing to difficulty in swallowing and breathing. Both required mechanical ventilation and improved partly with plasmapharesis. The cases emphasize on (1) description of the rare complication in a setting of outbreak with CHIKV, (2) acute axonal as well as demyelinating neuropathy may occur with CHIKV, (3) accurate identification of this entity during outbreaks with dengue, both of which are vector borne and may present with similar complications.

  16. Common complication of crush injury, but a rare compartment syndrome

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    Shaikh Nissar

    2010-01-01

    Full Text Available Compartment syndrome (CS is a common complication of crush injury but it is rare to find bilateral gluteal compartment syndrome (BGCS. Only six cases of BGCS have been reported in the literature. This syndrome has been reported after crush injury, drug overdose, surgical positioning, and vascular surgery. Apart from CS, crush injury is associated with multi-system adverse effects and these patients are at high risk for renal failure and sepsis. CS patients may present with dehydration; coagulation disorders; elevated creatine phosphokinase and myoglobin levels; hyperkalemia and hypocalcaemia, which may cause life-threatening arrhythmias and therefore need urgent and aggressive therapy. The early goal in these patients is prevention of acute renal failure with aggressive fluid therapy, alkalinization of urine, and forced diuresis. Early treatment of hyperkalemia, antibiotic therapy, immunoprophylaxis, and wound care will minimize the risk of arrhythmias and sepsis. CS must be considered when any patient is diagnosed with crush injury syndrome. CS is defined as elevation of interstitial/intracompartmental pressure, leading to microvascular and myoneural dysfunction and secondary hypoxia; it may cause functional loss or even death if not detected early and treated properly. The increase in pressure in one or all compartments of the gluteal region causes CS with devastating effects on muscle and neurovascular bundles. CS is traditionally diagnosed on the basis of five ′p′s: pain, pallor, paraesthesia, pulselessness and paralysis. Diagnosis of gluteal CS is difficult as the peripheral pulses are preserved and the condition is usually only diagnosed when neurological abnormality is noticed. Diagnosis of CS can be made by direct measurement of the compartment pressure and magnetic resonance imaging or computerized tomography. Gluteal CS is managed by fasciotomy and debridement of necrosed tissue, with secondary closure of fascia. A high index of

  17. Impact of preoperative hormonal stimulation on postoperative complication rates after hypospadias repair: a meta-analysis.

    Science.gov (United States)

    Chao, Min; Zhang, Yin; Liang, Chaozhao

    2017-06-01

    To improve the surgical outcome of hypospadias repair surgery, preoperative hormonal stimulation (PHS) has been proposed. We conducted a meta-analysis to evaluate the impact of preoperative hormonal stimulation (PHS) treatment on complication rates following hypospadias repair surgery. A comprehensive literature search up to June 1st, 2015 was carried out for relevant studies. After literature identification and data extraction, relative ratio (RR) was calculated to compare postoperative complication rates. Heterogeneity among individual studies was tested using the Cochran χ2 Q test and quantified by calculating the I2 index. Meta-regression was applied to find potential affective factors. Overall, 428 patients from 6 studies had undergone primary hypospadias repair, of which 171 (39.95%) received some form of PHS with human chorionic gonadotropin (HCG), dihydrotestosterone (DHT) or testosterone (T). They underwent three different types of surgical techniques, including onlay island flap (N.=277), tubularized incised plate (N.=99) and Koyanagi urethroplasty (N.=52). These 6 studies classified the complication rates based on PHS. The relative ratio (RR) for a complication occurring following PHS use was 1.18 (95% CI: 0.70-2.00, Z=0.91, P=0.539). Significant heterogeneity (I2=47.1%, P=0.092) among various research literature was found and meta-regression was undertaken for the heterogeneity, but surgical technique, mean age of patients at time of surgery, types of PHS and the quality of studies were not the cause of heterogeneity. Use of T, DHT and HCG prior to hypospadias repair does not appear to increase the incidence of postoperative complications, but further investigation is needed.

  18. Pregnancy complications in women with polycystic ovary syndrome.

    Science.gov (United States)

    Palomba, Stefano; de Wilde, Marlieke A; Falbo, Angela; Koster, Maria P H; La Sala, Giovanni Battista; Fauser, Bart C J M

    2015-01-01

    The great majority of studies performed so far concerning women diagnosed with polycystic ovary syndrome (PCOS) have focused on diagnosis, menstrual cycle abnormalities, hirsutism and infertility. Although progress has been made in developing methods for achieving a pregnancy and reducing multiple gestations in women with PCOS, little attention has been paid to pregnancy complications and subsequent child outcomes. This review aims to summarize current knowledge regarding the clinical and pathophysiological features of pregnancy and children in women with PCOS. A literature search up to April 2015 was performed in PubMed, Medline, the Cochrane Library and Web of Science without language restriction. All articles were initially screened for title and abstract and full texts of eligible articles were subsequently selected. Systematic reviews with meta-analysis were initially included for each specific subject. Recent randomised controlled trials (RCTs), which were not included in the systematic reviews, were also included. In addition to evidence from meta-analyses or RCTs, we used non-randomized prospective, uncontrolled prospective, retrospective and experimental studies. When specific data for patients with PCOS were lacking, results from general population studies were reported. Women with PCOS exhibit a clinically significant increased risk of pregnancy complications compared with controls. Data which were not adjusted for BMI or other confounders demonstrated in PCOS a 3-4-fold increased risk of pregnancy-induced hypertension and pre-eclampsia, a 3-fold increased risk of gestational diabetes and 2-fold higher chance for premature delivery. Features characteristic of PCOS, such as hyperandrogenism, obesity, insulin resistance and metabolic abnormalities, may contribute to the increased risk of obstetric and neonatal complications. Limited available data suggest that offspring of women with PCOS have an increased risk for future metabolic and reproductive

  19. The role of hormones of adipose tissue in the development pregnancy complications in obese women

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    N A Petunina

    2013-03-01

    Full Text Available Obesity in pregnancy is a risk factor for complications for both the mother and of the fetus. Adipose tissue hormones (leptin, adiponectin, resistin are secreted by the human placenta and regulate the function of trophoblast.The review presents data from the literature on the role of adipocytokines in the development of gestational diabetes and preeclampsia in obesity women. The article considers the criteria and algorithms for the diagnosis of gestational diabetes recommended by the World Health Organization and the International Association of research groups for diabetes and pregnancy.

  20. Growth hormone therapy in a girl with Turner syndrome and diabetes type 1 - case report.

    Science.gov (United States)

    Obara-Moszynska, Monika; Banaszak, Magdalena; Niedziela, Marek

    2015-01-01

    The studies indicate the complex etiology of abnormal glucose metabolism in the Turner syndrome (TS). In the light of these carbohydrate disorders a therapy with recombinant growth hormone (rGH) in TS may be associated with complications, as growth hormone has a diabetogenic potential. Perinatal history is unknown since the patient was adopted at the age of 4 years. At 11 years old, due to typical phenotype, TS was diagnosed. The karyotype was 45,X[43]/46,X,i(X)(q10)[7]. At the same age, basing on laboratory results, insulin dependent diabetes was diagnosed and the conventional insulin therapy was initiated. During the hospitalization, at the age of 12 years, the patient was 123.5cm (-4.4SD). At the same age rGH tre-atment was initiated, with the dose 0.045 mg/kg/d. After 3 months of therapy the height velocity rose to 8.2 cm/ year. At the age of 13 years, substitution with 17β-estradiol was started. After 3 years and 4 months the growth hormone treatment was stopped because of poor height velocity. The final height of the patient was 140 cm (-4,OSD). Two years after the end of rGH treatment the height was 141.2 cm. After termination of rGH treatment the need for daily insulin dose decreased from 50-60U/d to 38-44U/d. The decision of rGH therapy in TS with diabetes is certainly difficult. While starting the growth hormone treatment the clinician must keep in mind the risk of metabolic complications, but also the awareness that gives the patient a chance to improve the final height. In terms of the proper psycho-emotional development the reduction of growth deficit is very important. © Polish Society for Pediatric Endocrinology and Diabetology.

  1. [Terson syndrome: a frequently missed ophthalmologic complication in subarachnoid hemorrhage].

    Science.gov (United States)

    Wiethölter, S; Steube, D; Stotz, H P

    1998-01-01

    The syndrome of intra-vitreous bleeding in association with subarachnoid hemorrhage (SAH) was first described by the French ophthalmologist Albert Terson. In the last 10 years, 31 articles describing 202 cases of Terson's syndrome (TS) were published. Only 3 out of the 31 were printed in non-ophthalmological journals. The findings of our prospective study underline the fact that too little attention is paid to TS in the early treatment of patients with SAH. Between 1/95 and 8/97, 89 patients with spontaneous SAH (7% of all admissions) were transferred to our hospital for post acute phase rehabilitation. Out of these, 13 patients (19 eyes) could be diagnosed with TS. This corresponds to an incidence of 14.6% of all patients with SAH (previous studies: 2-27%). However, only one patient had been correctly diagnosed with TS in the referring clinic. Early recognition of TS is of high importance since diminuation of visual acuity even to functional blindness, complicated in the bilateral case, can hamper the rehabilitative process considerably. Moreover, complications can lead to significant and irreversible damage, i.e. proliferative vitreo-retinopathy (PVR), retinal breaks, traction amotio, and cataract. Suspicion of TS is raised in either cooperative patients complaining of compromised visual acuity or in patients where funduscopy shows vitreous opacity. According to our results, visual evoked potentials (VEP) have only a limited role in diagnosis because of their low sensitivity. Not infrequently, however, VEP may point to accompanying optic nerve atrophy, thereby suggesting conservative treatment. Absolute indications for surgical interventions are PVR and its sequelae; relative indications are subjective visual impairment, impediment of rehabilitation, or lack of spontaneous resorption of the hemorrhage. The surgical procedure of choice is the pars plana vitrectomy (PPV). Rare complications of this operation are retinal damage, endophthalmitis, and reoccurrence of

  2. A Rare Complication of Radiofrequency Tonsil Ablation: Horner Syndrome

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    Cuneyt Kucur

    2015-01-01

    Full Text Available Chronic tonsillitis is a common disease, and several different surgical techniques are used to treat this condition. In recent years, techniques such as radiofrequency ablation and coblation have been commonly used for tonsil surgery. In this report, we present the cases of two pediatric patients who developed ptosis, miosis, and enophthalmos (Horner syndrome after radiofrequency ablation for tonsil reduction and discuss the technique of radiofrequency ablation of the tonsils. In the early postoperative period, miosis and ptosis were observed on the right side in one patient and on the left side in the other patient. Both patients were treated with 1 mg/kg/day methylprednisolone, which were tapered by halving the dose every 3 days. Miosis and ptosis improved after treatment in both patients. Along with the case presentation, we discuss the effectiveness and complications of radiofrequency ablation of the tonsils. These unusual complications of tonsil ablation may help ENT physicians who do not yet have a preferred surgical technique for tonsillectomy to make an informed decision. Limited data are available about the possible complications of radiofrequency ablation of the tonsils. The present report contributes to the literature on this topic.

  3. MRI examination of West syndrome complicated by periventricular leukomalacia (PVL)

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    Fukuda, Kuniaki; Yamashita, Akiko; Endo, Shoichi [National Kagawa Children' s Hospital, Zentuji (Japan); Gouda, Tomoko

    2001-10-01

    The magnetic resonance imaging (MRI) findings of 19 low-birth-weight infants with periventricular leukomalacia (PVL) and cerebral palsy, 6 with West syndrome as a complication (group III) and 13 without it (group II), were compared with those of normal 13 low-birth-weight infants without PVL or cerebral palsy (group I) to investigate the pathogenetic mechanism of West syndrome. All of the subjects were born between 1989 and 1997, and there were no significant differences between the three groups in birth weight or age at the time of the examination. Although the gestation period was significantly longer in the group III (infants with West syndrome) than in group I (control infants), there was no significant difference was seen between group I and group II (infants with PVL and cerebral palsy). PVL was diagnosed by MRI, and the diagnostic standards included periventricular high signal intensity on T2-weighted images, decreased cerebral white matter, and deformity or enlargement of the lateral ventricles. The area of periventricular high signal intensity was significantly larger in group II and group III than in group I, and the occipital lobe white matter was significantly thinner in the former than the latter. The lateral ventricles were significantly larger in group II than in the other two groups. The area of the corpus callosum and the thickness of the splenium and trunk of the corpus callosum were significantly smaller in group II and group III than in group I, but the corpus callosum was significantly shorter in group III than in the other two groups. Findings in the brain stem showed a significantly smaller pons and medulla oblongata in group III than in group I, but there were no significant differences between group II and group I. The results of this study indicate that lower brainstem lesions as well as cerebral lesions are associated with the development of West syndrome. (K.H.)

  4. Epidemiological and sociodemographic factors associated with complicated alcohol withdrawal syndrome.

    Science.gov (United States)

    Monte-Secades, R; Blanco-Soto, M; Díaz-Peromingo, J A; Sanvisens-Bergé, A; Martín-González, M C; Barbosa, A; Rosón-Hernández, B; Tejero-Delgado, M A; Puerta-Louro, R; Rabuñal-Rey, R

    2017-10-01

    To analyse the influence of epidemiological and sociodemographic factors in complicated alcohol withdrawal syndrome (AWS). A multicentre, observational prospective study was conducted on consecutively added patients with AWS hospitalised in internal medicine departments. We recorded sociodemographic, epidemiological, clinical and progression data. Complicated AWS was defined as that which progressed with seizures or delirium tremens. We studied 228 episodes of AWS in 219 patients. The mean age was 54.5 years (SD, 11.5), and 90.8% were men. AWS was the cause for hospitalisation in 39.9% of the patients. Some 27.1% of the cases presented seizures, and 32.4% presented delirium tremens. The daily quantity of alcohol ingested was 17.8 standard drink units (SD, 21.4), with 16.6 years of dependence (SD, 11.3). The pattern of alcohol abuse was regular in 82.8% of the patients. Some 38.4% of the patients were married or had a partner, and 45.6% had children. Some 72.7% of the patients were unemployed or retired. Some 68.5% had only completed primary studies. Some 4.8% consumed cannabis, 5.2% consumed cocaine and 3% consumed opioids. The independent variables related to complicated AWS were consumption of a drug other than alcohol (OR, 5.3; 95% CI 1.5-18.7), low education level (OR, 3.4; 95% CI 1.6-7.3) and hospitalisation for AWS (OR, 2.9; 95% CI 1.5-5.6). The model's receiver operating characteristic area was 0.718 (95% CI 0.643-0.793). Concomitant drug abuse and a low educational level could help identify patients at risk of complicated AWS. Copyright © 2017 Elsevier España, S.L.U. and Sociedad Española de Medicina Interna (SEMI). All rights reserved.

  5. COMPARISON OF THE EFFECTIVENESS OF HORMONAL AND NON-HORMONAL METHODS OF TREATMENT OF EARLY MANIFESTATIONS OF CLIMACTERIC SYNDROME

    OpenAIRE

    Виктор Евсеевич Радзинский; Анна Андреевна Костромина

    2017-01-01

    Early manifestations of climacteric syndrome (hot flashes, night sweats, depression, and others) are widespreadin middle-aged women. Hot flashes , in particular, dramatically reduce the quality of life of these women, which in many cases requires treatment. Selection of individual adequate and affordable therapy is one of the main issues of modern gynecology. The aim of the study was to evaluate the effectiveness of different hormonal and non-hormonal methods of managing of of climacteric...

  6. Gastrointestinal variant of Lemierre's syndrome complicating ruptured appendicitis

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    Fadi Al Akhrass

    2015-01-01

    Full Text Available Fusobacterium necrophorum is a non-spore-forming, obligate anaerobic, filamentous, gramnegative bacillus that frequently colonizes the human oral cavity, respiratory tract, and gastrointestinal tract. Fusobacterium species have rarely been implicated in cases of gastrointestinal variant of Lemierre's syndrome. We describe a case of F. necrophorum bacteremia associated with suppurative porto-mesenteric vein thrombosis (PVT following acute ruptured appendicitis. In addition, we list the documented twelve cases of Fusobacterium pylephlebitis. Recanalization of the porto-mesenteric veins and relief of the extrahepatic portal hypertension were achieved with early empiric antibiotic and local thrombolytic therapy. Our patient's case underscores the importance of recognizing Fusobacterium bacteremia as a possible cause of suppurative PVT after disruption of the gastrointestinal mucosa following an acute intraabdominal infectious process. Early treatment of this condition using anticoagulation and endovascular thrombolysis as adjunctive therapies may prevent PVT complications.

  7. Growth without growth hormone in combined pituitary hormone deficiency caused by pituitary stalk interruption syndrome

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    Sang Soo Lee

    2017-03-01

    Full Text Available Growth hormone (GH is an essential element for normal growth. However, reports of normal growth without GH have been made in patients who have undergone brain surgery for craniopharyngioma. Normal growth without GH can be explained by hyperinsulinemia, hyperprolactinemia, elevated leptin levels, and GH variants; however, its exact mechanism has not been elucidated yet. We diagnosed a female patient aged 13 with combined pituitary hormone deficiency (CPHD caused by pituitary stalk interruption syndrome (PSIS. The patient has experienced recurrent hypoglycemic seizures since birth, but reached the height of 160 cm at the age of 13, showing normal growth. She grew another 8 cm for 3 years after the diagnosis, and she reached her final adult height of 168 cm which was greater than the midparental height, at the age of 16. The patient's blood GH and insulin-like growth factor-I levels were consistently subnormal, although her insulin levels were normal. Her physical examination conducted at the age of 15 showed truncal obesity, dyslipidemia, and osteoporosis, which are metabolic features of GH deficiency (GHD. Herein, we report a case in which a PSIS-induced CPHD patient attained her final height above mid parental height despite a severe GHD.

  8. Macrophage activation syndrome: a potentially fatal complication of rheumatic disorders.

    Science.gov (United States)

    Sawhney, S; Woo, P; Murray, K J

    2001-11-01

    To review the precipitating events, clinical features, treatment, and outcome of macrophage activation syndrome (MAS). Retrospective review of cases of MAS from a prospectively collected database of children with rheumatic diseases from 1980 to 2000. Nine patients (eight girls) were considered to have evidence of MAS. The primary diagnosis was systemic onset juvenile idiopathic arthritis in seven, enthesitis related arthritis in one, and chronic infantile neurological cutaneous articular syndrome in one. Mean age of onset was 5.7 years, and duration prior to MAS, 4.2 years. No medication was identified as a trigger. Eight had infections prior to MAS; specific infectious agents were identified in four. High grade fever, new onset hepatosplenomegaly, and lymphadenopathy were common clinical features. Platelet counts fell dramatically, from an average of 346 to 99 x 10(9)/l. Mean erythrocyte sedimentation rate (in three patients) fell from 115 to 28 mm/h. Eight had abnormal liver function during the disease course, and six had coagulopathy. Bone marrow examination supported the diagnosis with definite haemophagocytosis in four of seven. All received high dose steroids (eight intravenous, one oral), five cyclosporin, two cyclophosphamide, and one antithymocyte globulin. Two of three patients with significant renal impairment died. MAS is a rare and potentially fatal complication of childhood rheumatic disorders. Most of our patients were female, and most cases were preceded by infection. Bone marrow studies support the diagnosis. Deranged renal function may be a poor prognostic sign. Aggressive early therapy is essential.

  9. Colonic stricture as a complication of haemolytic uraemic syndrome

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    Megan Grinlinton

    2017-12-01

    Full Text Available Haemolytic uraemic syndrome (HUS is an infectious disease that can rapidly become life-threatening in the paediatric population. A number of long-term complications may arise from HUS including the rare development of colonic strictures. In this case report, we present two cases with similar presentations of colonic strictures following HUS. Case 1 is a 17-month-old female who developed HUS and multiple complications including a sigmoid colonic stricture. Once the stricture was resected, her recovery was rapid and complete. Case 2 is a 3-year-old male who developed severe HUS requiring dialysis. After developing a small bowel obstruction, a laparotomy demonstrated caecal disease, pan-colonic inflammation, a calcified appendix and a strictured descending colon. A second operation revealed strictures at the transverse, descending and sigmoid colon. Once the diseased part of bowel had been removed his recovery was complete. This report demonstrates the diagnostic difficulty and patient morbidity that may arise from post-HUS colonic strictures. A contrast study is the recommended investigation of choice in patients presenting with ongoing gastrointestinal symptoms after an acute infection with HUS.

  10. Complications of bariatric surgery: dumping syndrome, reflux and vitamin deficiencies.

    Science.gov (United States)

    Tack, Jan; Deloose, Eveline

    2014-08-01

    Bariatric surgical procedure are increasingly and successfully applied in the treatment of morbid obesity. Nevertheless, these procedures are not devoid of potential long-term complications. Dumping syndrome may occur after procedures involving at least partial gastric resection or bypass, including Roux-en-Y gastric bypass (RYGB) and sleeve gastrectomy. Diagnosis is based on clinical alertness and glucose tolerance testing. Treatment may involve dietary measures, acarbose and somatostatin analogues, or surgical reintervention for refractory cases. Gastro-esophageal reflux disease (GERD) can be aggravated by vertical banded gastroplasty and sleeve gastrectomy procedures, but pre-existing GERD may improve after RYGB and with adjustable gastric banding. Nutrient deficiencies constitute the most important long-term complications of bariatric interventions, as they may lead to haematological, metabolic and especially neurological disorders which are not always reversible. Malabsorptive procedures, poor postoperative nutrient intake, recurrent vomiting and poor compliance with vitamin supplement intake and regular follow-up are important risk factors. Preoperative nutritional assessment and rigourous postoperative follow-up plan with administration of multi-vitamin supplements and assessment of serum levels is recommended in all patients. Copyright © 2014. Published by Elsevier Ltd.

  11. Compartment syndrome, rhabdomyolysis and risk of acute renal failure as complications of the lithotomy position.

    NARCIS (Netherlands)

    Bocca, G.; Moorselaar, R.J.A. van; Feitz, W.F.J.; Staak, F.H.J.M. van der; Monnens, L.A.H.

    2002-01-01

    Compartment syndrome, rhabdomyolysis and the risk of acute renal failure are potential complications of the lithotomy position. A six-year-old girl is described who developed a compartment syndrome with rhabdomyolysis after prolonged surgery in the lithotomy position. This complication occurred

  12. [Pulmonary complications after extracorporeal circulation. ECC lung syndrome].

    Science.gov (United States)

    Foliguet, B

    1977-01-01

    Pulmonary complications after cardiac surgery under extracorporeal circulation remain frequent and sometimes grave, in spite of the great progress which has been made over the past 20 years in the methods of cardiorespiratory assistance. The authors analyse the clinical and radiological repercussions of perfusion on the lung, in 40 patients operated under ECC for coronary revascularisation. The simutaneous study of the arterial, and mixed venous blood gasses and of the alveolar gases, in 20 of these patients showed the constant occurrence of a shunt syndrome, without alveolar hypoventilation or disorders in peripheral circulatory flow. Ventilatory alcalosis, hypocapnia, hypoxemia and the rise in the alveolar arterial oxygen gradient is increased during the second post-operative day. Among the variables studied (duration of ECC, degree of hypothermia, duration of the intervention, duration of anesthesia, pleurotomy) only the latter intervened in a statistically significant manner in this study, in the increase in hypoxemia. 46 pulmonary biopsies carried out before and after ECC in 23 coronary patients were examined with the electron microscope. The initial alveolar involvement affects the septal microcirculation with signs of an increase in capillary permeability leading to an interstitial and epithelial destruction. The use of a membrane oxygenator prevents some of the alveolar lesions, as has been proved by the study of five pulmonary biopsies carried out in dogs submitted to ECC of long duration. Catherterization of the pulmonary artery carried out in 35 patients by means of a SWAN-GANZ catheter, before the intervention enabled supervision of the degree of importance and speed of the hemodynamic variations in the pulmonary circulation during the different phases of ECC (during the phase of ventricular fibrillation). The rise in the flow of left output can lead to the occurrence of negative pulmonary intravascular pressures which can be prejudicial for capillary

  13. Complications and challenges associated with polycystic ovary syndrome: current perspectives

    Directory of Open Access Journals (Sweden)

    Palomba S

    2015-07-01

    Full Text Available Stefano Palomba,1 Susanna Santagni,1 Angela Falbo,1 Giovanni Battista La Sala1,21Unit of Obstetrics and Gynecology, Arcispedale Santa Maria Nuova-Scientific Institute of Treatment and Care (IRCCS, Reggio Emilia, 2Department of Obstetrics and Gynecology, University of Modena and Reggio Emilia, Modena, ItalyAbstract: Polycystic ovary syndrome (PCOS represents the most common endocrine dysfunction in fertile women and it is considered a heterogeneous and multifaceted disorder, with multiple reproductive and metabolic phenotypes which differently affect the early- and long-term syndrome’s risks. Women with PCOS present an adverse reproductive profile, including a high risk of pregnancy-induced hypertension, preeclampsia, and gestational diabetes mellitus. Patients with PCOS present not only a higher prevalence of classic cardiovascular risk factors, such as hypertension, dyslipidemia, and type-2 diabetes mellitus, but also of nonclassic cardiovascular risk factors, including mood disorders, such as depression and anxiety. Moreover, at the moment, clinical data on cardiovascular morbidity and mortality in women with PCOS are controversial. Finally, women with PCOS show an increased risk of endometrial cancer compared to non-PCOS healthy women, particularly during premenopausal period. Currently, we are unable to clarify if the increased PCOS early- and long-term risks are totally due to PCOS per se or mostly due to obesity, in particular visceral obesity, that characterized the majority of PCOS patients. In any case, the main endocrine and gynecological scientific societies agree to consider women with PCOS at increased risk of obstetric, cardiometabolic, oncology, and psychological complications throughout life, and it is recommended that these women be accurately assessed with periodic follow-up.Keywords: cardiovascular disease, infertility, polycystic ovary syndrome, PCOS, pregnancy

  14. Outcome of contemporary acute coronary syndrome complicated by ventricular tachyarrhythmias.

    Science.gov (United States)

    Orvin, Katia; Eisen, Alon; Goldenberg, Ilan; Gottlieb, Shmuel; Kornowski, Ran; Matetzky, Shlomi; Golovchiner, Gregory; Kuznietz, Jairo; Gavrielov-Yusim, Natalie; Segev, Amit; Strasberg, Boris; Haim, Moti

    2016-02-01

    To evaluate the incidence and prognostic implications of ventricular tachyarrhythmias (VTAs) complicating acute myocardial infarction (MI). We evaluated 7669 MI patients [ST elevation (n = 3573) and non-ST-elevation acute coronary syndrome (ACS) (n = 4096)] from the Acute Coronary Syndrome Israeli Survey for the incidence of VTA. Ventricular tachyarrhythmia occurred in 3.8% of patients [2.1% early (≤ 48 h) and 1.7% late (>48 h) VTA]. In-hospital mortality rates were higher for patients with VTA when compared with patients with no VTA (P < 0.001). Consistent with these findings, multivariable analysis demonstrated that early and late VTAs were associated with increased risk of in-hospital death [hazard ratio (HR) = 3.84; 95% confidence interval (CI) 1.77-6.78, P < 0.001, and HR = 8.23; 95% CI 4.84-13.98, P < 0.001, respectively]. In contrast, post-discharge outcomes demonstrated that only late VTA was independently associated with a significant increased risk of 30-day mortality (HR = 5.17; 95% CI 1.54-17.27, P = 0.007) with a trend towards an increased 1-year mortality risk (HR = 1.69; 95% CI 0.79-3.62, P = 0.17). The long-term risk associated with in-hospital VTA was driven by sustained ventricular tachycardia (VT) (HR = 3.28; 95% CI 1.92-5.60, P < 0.001) but not ventricular fibrillation (HR = 1.27; 95% CI 0.65-2.49, P = 0.47). Our findings suggest that in patients with ACS, both early and late VTAs are associated with an increased risk of in-hospital mortality. However, only late VTA, mostly sustained VT, is associated with long-term adverse outcome. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2015. For permissions please email: journals.permissions@oup.com.

  15. Central neurogenic diabetes insipidus, syndrome of inappropriate secretion of antidiuretic hormone, and cerebral salt-wasting syndrome in traumatic brain injury.

    Science.gov (United States)

    John, Cynthia A; Day, Michael W

    2012-04-01

    Central neurogenic diabetes insipidus, syndrome of inappropriate secretion of antidiuretic hormone, and cerebral salt-wasting syndrome are secondary events that affect patients with traumatic brain injury. All 3 syndromes affect both sodium and water balance; however, they have differences in pathophysiology, diagnosis, and treatment. Differentiating between hypernatremia (central neurogenic diabetes insipidus) and the 2 hyponatremia syndromes (syndrome of inappropriate secretion of antidiuretic hormone, and cerebral salt-wasting syndrome) is critical for preventing worsening neurological outcomes in patients with head injuries.

  16. Osteomalacia complicating renal tubular acidosis in association with Sjogren's syndrome.

    Science.gov (United States)

    El Ati, Zohra; Fatma, Lilia Ben; Boulahya, Ghada; Rais, Lamia; Krid, Madiha; Smaoui, Wided; Maiz, Hedi Ben; Beji, Soumaya; Zouaghi, Karim; Moussa, Fatma Ben

    2014-09-01

    Renal involvement in Sjogren's syndrome (SS) is not uncommon and may precede other complaints. Tubulointerstitial nephritis is the most common renal disease in SS and may lead to renal tubular acidosis (RTA), which in turn may cause osteomalacia. Nevertheless, osteomalacia rarely occurs as the first manifestation of a renal tubule disorder due to SS. We herewith describe a 43-year-old woman who was admitted to our hospital for weakness, lumbago and inability to walk. X-ray of the long bones showed extensive demineralization of the bones. Laboratory investigations revealed chronic kidney disease with serum creatinine of 2.3 mg/dL and creatinine clearance of 40 mL/min, hypokalemia (3.2 mmol/L), hypophosphatemia (0.4 mmol/L), hypocalcemia (2.14 mmol/L) and hyperchloremic metabolic acidosis (chlorine: 114 mmol/L; alkaline reserve: 14 mmol/L). The serum alkaline phosphatase levels were elevated. The serum levels of 25-hydroxyvitamin D and 1,25-dihydroxy vitamin D were low and borderline low, respectively, and the parathyroid hormone level was 70 pg/L. Urinalysis showed inappropriate alkaline urine (urinary PH: 7), glycosuria with normal blood glucose, phosphaturia and uricosuria. These values indicated the presence of both distal and proximal RTA. Our patient reported dryness of the mouth and eyes and Schirmer's test showed xerophthalmia. An accessory salivary gland biopsy showed changes corresponding to stage IV of Chisholm and Masson score. Kidney biopsy showed diffuse and severe tubulo-interstitial nephritis with dense lymphoplasmocyte infiltrates. Sicca syndrome and renal interstitial infiltrates indicated SS as the underlying cause of the RTA and osteomalacia. The patient received alkalinization, vitamin D (Sterogyl ®), calcium supplements and steroids in an initial dose of 1 mg/kg/day, tapered to 10 mg daily. The prognosis was favorable and the serum creatinine level was 1.7 mg/dL, calcium was 2.2 mmol/L and serum phosphate was 0.9 mmol/L.

  17. Thyroid, cortisol and growth hormone levels in adult Nigerians with metabolic syndrome.

    Science.gov (United States)

    Udenze, Ifeoma Christiana; Olowoselu, Olusola Festus; Egbuagha, Ephraim Uchenna; Oshodi, Temitope Adewunmi

    2017-01-01

    The similarities in presentation of cortisol excess, growth hormone deficiency, hypothyroidism and metabolic syndrome suggest that subtle abnormalities of these endocrine hormones may play a causal role in the development of metabolic syndrome. The aim of this study is to determine the levels of cortisol, thyroid and growth hormones in adult Nigerians with metabolic syndrome and determine the relationship between levels of these hormones and components of the syndrome. This was a case control study conducted at the Lagos University Teaching Hospital, Lagos, Nigeria. Participants were fifty adult men and women with the metabolic syndrome, and fifty, age and sex matched males and females without the metabolic syndrome. Metabolic syndrome was defined based on the NCEP-ATPIII criteria. Written Informed consent was obtained from the participants. Socio demographic and clinical data were collected using a structured questionnaire. Venous blood was collected after an over-night fast. The Ethics committee of the Lagos University Teaching Hospital, Lagos, Nigeria, approved the study protocol. Comparison of continuous variables was done using the Student's t test. Correlation analysis was employed to determine the associations between variables. Statistical significance was set at Psignificantly decreased (psignificantly increased ( psignificantly with waist circumference (p=0.004), glucose (p= 0.002), total cholesterol ( p=0.001) and LDL- cholesterol ( psignificant correlation with waist circumference (p=0.004). Cortisol and growth hormone levels were similar in metabolic syndrome and controls. Cortisol however had a positive significant correlation with waist/hip ratio (pcortisol and growth hormones correlated with components of the syndrome. A therapeutic role may exit for these hormones in the management of metabolic syndrome and related disorders.

  18. Complications and challenges associated with polycystic ovary syndrome: current perspectives

    Science.gov (United States)

    Palomba, Stefano; Santagni, Susanna; Falbo, Angela; La Sala, Giovanni Battista

    2015-01-01

    Polycystic ovary syndrome (PCOS) represents the most common endocrine dysfunction in fertile women and it is considered a heterogeneous and multifaceted disorder, with multiple reproductive and metabolic phenotypes which differently affect the early- and long-term syndrome’s risks. Women with PCOS present an adverse reproductive profile, including a high risk of pregnancy-induced hypertension, preeclampsia, and gestational diabetes mellitus. Patients with PCOS present not only a higher prevalence of classic cardiovascular risk factors, such as hypertension, dyslipidemia, and type-2 diabetes mellitus, but also of nonclassic cardiovascular risk factors, including mood disorders, such as depression and anxiety. Moreover, at the moment, clinical data on cardiovascular morbidity and mortality in women with PCOS are controversial. Finally, women with PCOS show an increased risk of endometrial cancer compared to non-PCOS healthy women, particularly during premenopausal period. Currently, we are unable to clarify if the increased PCOS early- and long-term risks are totally due to PCOS per se or mostly due to obesity, in particular visceral obesity, that characterized the majority of PCOS patients. In any case, the main endocrine and gynecological scientific societies agree to consider women with PCOS at increased risk of obstetric, cardiometabolic, oncology, and psychological complications throughout life, and it is recommended that these women be accurately assessed with periodic follow-up. PMID:26261426

  19. Surgical complications of hemolytic uremic syndrome: Single center experiences

    Directory of Open Access Journals (Sweden)

    Hooman Nakysa

    2007-01-01

    Full Text Available Purpose: To determine the prevalence, outcome and prognostic factors in children with hemolytic uremic syndrome (HUS who underwent laparotomy. Materials and Methods: The medical records of 104 patients with HUS who presented to our center between 1986 and 2003 were reviewed retrospectively. Data were analyzed using Student′s t test for comparing means, Fisher′s exact test for frequencies and Pearson′s correlation for finding the correlations. Results: 78% of cases presented with vomiting and diarrhea. Seven out of 104 needed surgical exploration. The indications of surgery were acute abdomen, severe abdominal distention and the sign of peritonitis. The findings at laparotomy were intussusceptions, perforation (colon, ileum, gangrene of entire colon, rectosigmoidal tearing, duodenal obstruction and toxic megacolon. Pathological findings were transmural infarction in two cases in which staged surgical management was performed (cecostomy, resection, later anastomosis. Four out of seven patients died because of pulmonary failure, coma and multiple organ failure ( P< 0.05 compared to those who did not need laparotomy. The patients requiring surgery were young (< 3 years, had high leukocyte count (>20000 mm 3 and low albumin level (< 3g/dl ( P< 0.05. Conclusion: Surgical complications of HUS are rare but are assorted with high mortality due to respiratory failure and multiple organ failure. Early decision of laparotomy associated with intensive care, including mechanical ventilation, adequate dialysis and ultrafiltration, are recommended.

  20. Quantitative liver functions in Turner syndrome with and without hormone replacement therapy

    DEFF Research Database (Denmark)

    Gravholt, Claus Højbjerg; Poulsen, Henrik Enghusen; Ott, Peter

    2007-01-01

    Studies have documented elevated levels of liver enzymes in many females with Turner syndrome (TS). Histology has shown a range of changes. Treatment with female hormone replacement therapy (HRT) reduces liver enzymes.......Studies have documented elevated levels of liver enzymes in many females with Turner syndrome (TS). Histology has shown a range of changes. Treatment with female hormone replacement therapy (HRT) reduces liver enzymes....

  1. EFFECT OF GROWTH-HORMONE TREATMENT ON CRANIOFACIAL GROWTH IN TURNERS SYNDROME

    NARCIS (Netherlands)

    RONGENWESTERLAKEN, C; VANDERBORN, E; PRAHLANDERSEN, B; VONTEUNENBROEK, A; MANESSE, P; OTTEN, BJ; VANDERTWEEL, [No Value; KUIJPERSJAGTMAN, AM; VANDERWAAL, HAD; DRAYER, NM; WIT, JM; VANDERBRANDE, JL

    A cephalometric study was performed in 19 patients with Turner's syndrome, aged 8.7-16.5 years. A lateral roentgencephalogram was taken before and after two years of treatment with biosynthetic growth hormone in a dose of 24 IU/m2/week. During two years of growth hormone treatment, the mandibular

  2. Cognitive and Adaptive Advantages of Growth Hormone Treatment in Children with Prader-Willi Syndrome

    Science.gov (United States)

    Dykens, Elisabeth M.; Roof, Elizabeth; Hunt-Hawkins, Hailee

    2017-01-01

    Background: People with Prader-Willi syndrome (PWS) typically have mild to moderate intellectual deficits, compulsivity, hyperphagia, obesity, and growth hormone deficiencies. Growth hormone treatment (GHT) in PWS has well-established salutatory effects on linear growth and body composition, yet cognitive benefits of GHT, seen in other patient…

  3. DOES GROWTH-HORMONE TREATMENT OF PATIENTS WITH TURNERS SYNDROME CAUSE AN ABNORMAL BODY SHAPE

    NARCIS (Netherlands)

    GERVER, WJM; DRAYER, NM; VANES, A

    The effect of human growth hormone on the body shape of 51 patients with Turner's syndrome (aged 6-19 years) was evaluated. Biosynthetic growth hormone was given in a dose of 24 IU/m2 body surface/week for two years. Karyotype analysis on peripheral blood was performed. Patients older than 12 years

  4. Growth hormone treatment in Turner's syndrome: A real world experience

    Directory of Open Access Journals (Sweden)

    Vijay Sheker Reddy Danda

    2017-01-01

    Full Text Available Objective: Short stature is a universal clinical feature of Turner's syndrome (TS. Growth failure begins in fetal life, and adults with TS are on an average 20 cm shorter than the normal female population. Since there is a paucity of data from India regarding the effect of growth hormone (GH on TS patients, we retrospectively analyzed the data of TS patients who are on GH treatment. Methods: This hospital-based observational retrospective study was conducted in a tertiary care hospital of Hyderabad. The data such as height, weight, and bone age of 16 patients who are diagnosed with TS on GH therapy for at least 6 months were included in the study. All the patients were treated with human recombinant GH at the dose of 0.3 mg/kg/week administered as daily subcutaneous injections. Results: The mean age at diagnosis was 12.7 years. The mean height at the start of GH therapy was 1.26 m, and mean height standard deviation score (HSDS was-0.61 when compared to Turner's specific reference data. With a mean duration of GH therapy of 25 months, the mean height at the end of therapy was 1.37 m and the mean height as per HSDS was + 0.37 resulting in a mean height gain of + 0.99 HSDS. Conclusion: Our observation shows that girls with TS benefit from early diagnosis and initiation of treatment with GH.

  5. Growth Hormone Treatment in Turner's Syndrome: A Real World Experience.

    Science.gov (United States)

    Reddy Danda, Vijay Sheker; Sreedevi, P; Arun, G; Rao, P Srinivas

    2017-01-01

    Short stature is a universal clinical feature of Turner's syndrome (TS). Growth failure begins in fetal life, and adults with TS are on an average 20 cm shorter than the normal female population. Since there is a paucity of data from India regarding the effect of growth hormone (GH) on TS patients, we retrospectively analyzed the data of TS patients who are on GH treatment. This hospital-based observational retrospective study was conducted in a tertiary care hospital of Hyderabad. The data such as height, weight, and bone age of 16 patients who are diagnosed with TS on GH therapy for at least 6 months were included in the study. All the patients were treated with human recombinant GH at the dose of 0.3 mg/kg/week administered as daily subcutaneous injections. The mean age at diagnosis was 12.7 years. The mean height at the start of GH therapy was 1.26 m, and mean height standard deviation score (HSDS) was-0.61 when compared to Turner's specific reference data. With a mean duration of GH therapy of 25 months, the mean height at the end of therapy was 1.37 m and the mean height as per HSDS was + 0.37 resulting in a mean height gain of + 0.99 HSDS. Our observation shows that girls with TS benefit from early diagnosis and initiation of treatment with GH.

  6. Prader-Willi Syndrome: New perspectives and effects of growth hormone treatment in children

    NARCIS (Netherlands)

    R.F.A. de Lind van Wijngaarden (Roderick)

    2009-01-01

    textabstractThis thesis encompasses studies embedded in the Dutch national growth hormone trial for children with Prader-Willi syndrome (PWS). The syndrome was fi rst described by Prader, Labhart, and Willi in 1956, reporting a combination of obesity, short stature, cryptorchidism, oligophrenia, and

  7. Severe Postoperative Complications may be Related to Mesenteric Traction Syndrome during Open Esophagectomy

    DEFF Research Database (Denmark)

    Ambrus, R; Svendsen, L B; Secher, N H

    2017-01-01

    .036). For patients who developed flushing, heart rate and plasma 6-keto-PGF1α also increased ( p = 0.001 and p severe postoperative complications were related to Grade II flushing ( p = 0.037). CONCLUSION: Mesenteric traction syndrome manifests more frequently during open than...... robotically assisted esophagectomy, and postoperative complications appear to be associated with severe mesenteric traction syndrome.......BACKGROUND: During abdominal surgery, traction of the mesenterium provokes mesenteric traction syndrome, including hypotension, tachycardia, and flushing, along with an increase in plasma prostacyclin (PGI2). We evaluated whether postoperative complications are related to mesenteric traction...

  8. Five-year response to growth hormone in children with Noonan syndrome and growth hormone deficiency.

    Science.gov (United States)

    Zavras, Niki; Meazza, Cristina; Pilotta, Alba; Gertosio, Chiara; Pagani, Sara; Tinelli, Carmine; Bozzola, Mauro

    2015-10-06

    Noonan syndrome (NS) is an autosomal dominant disorder characterized by specific features including short stature, distinctive facial dysmorphic features, congenital heart defects, hypertrophic cardiomyopathy, skeletal anomalies and webbing of the neck. Molecular screening has shown that the majority of individuals with NS have a mutation in the PTPN11 gene. Noonan syndrome children may show an impaired growth hormone (GH)/insulin-like growth factor axis. Moreover, recombinant human GH (rhGH) has been shown to improve growth rate in patients with NS, although data are still limited. In the present study, we assessed growth response following GH therapy (0.25 mg/Kg/week) in 5 (2 M and 3 F) GH-deficient NS patients (NSGHD, mean age 8.5 years) and in 5 (2 M and 3 F) idiopathic GH deficient (IGHD, mean age 8.6 years) patients. We also evaluated the safety of rhGH therapy in NS patients with GHD. At the beginning of GH treatment, height and growth rate were statistically lower in NSGHD children than in IGHD ones. During the first three years of rhGH therapy, NSGHD patients showed a slight improvement in height (from -2.71 SDS to -2.44 SDS) and growth rate (from -2.42 SDS to -0.23 SDS), although the values were always significantly lower than in IGHD children. After five years of rhGH treatment, height gain was higher in IGHD children (mean 28.3 cm) than in NSGHD patients (mean 23.6 cm). During the first five years of rhGH therapy, regular cardiological and haematological check-ups were performed, leading to the conclusion that rhGH therapy was safe. In conclusion, pre-pubertal NS children with GHD slightly increased their height and growth rate during the first years of GH therapy, although the response to rhGH treatment was significantly lower than IGHD children. Furthermore, the therapy appeared to be safe since no severe adverse effects were reported, at least during the first five years. However, a close follow-up of these patients is mandatory, especially to

  9. Pharmacologic management of bone-related complications and bone metastases in postmenopausal women with hormone receptor-positive breast cancer

    Directory of Open Access Journals (Sweden)

    Yardley DA

    2016-05-01

    Full Text Available Denise A Yardley1,2 1Sarah Cannon Research Institute, Nashville, TN, USA; 2Tennessee Oncology, Nashville, TN, USA Abstract: There is a high risk for bone loss and skeletal-related events, including bone metastases, in postmenopausal women with hormone receptor-positive breast cancer. Both the disease itself and its therapeutic treatments can negatively impact bone, resulting in decreases in bone mineral density and increases in bone loss. These negative effects on the bone can significantly impact morbidity and mortality. Effective management and minimization of bone-related complications in postmenopausal women with hormone receptor-positive breast cancer remain essential. This review discusses the current understanding of molecular and biological mechanisms involved in bone turnover and metastases, increased risk for bone-related complications from breast cancer and breast cancer therapy, and current and emerging treatment strategies for managing bone metastases and bone turnover in postmenopausal women with hormone receptor-positive breast cancer. Keywords: breast cancer, bone metastases, hormone receptor-positive, bone-related complications, interventions, management and management strategies, estrogen receptor-positive

  10. Late effects of early growth hormone treatment in Down syndrome.

    Science.gov (United States)

    Myrelid, Å; Bergman, S; Elfvik Strömberg, M; Jonsson, B; Nyberg, F; Gustafsson, J; Annerén, G

    2010-05-01

    Down syndrome (DS) is associated with short stature and psychomotor delay. We have previously shown that growth hormone (GH) treatment during infancy and childhood normalizes growth velocity and improves fine motor skill performance in DS. The aim of this study was to investigate late effects of early GH treatment on growth and psychomotor development in the DS subjects from the previous trial. Twelve of 15 adolescents with DS (3 F) from the GH group and 10 of 15 controls (5 F) participated in this follow-up study. Fifteen other subjects with DS (6 F) were included as controls in anthropometric analyses. Cognitive function was assessed with the Leiter International Performance Scale-Revised (Leiter-R) and selected subtests of the Wechsler Intelligence Scale for Children, Third edition (WISC-III). The Bruininks-Oseretsky Test of Motor Proficiency, Second edition (BOT-2), was used to assess general motor ability. Although early GH treatment had no effect on final height, the treated subjects had a greater head circumference standard deviation score (SDS) than the controls (-1.6 SDS vs. -2.2 SDS). The adolescents previously treated with GH had scores above those of the controls in all subtests of Leiter-R and WISC-III, but no difference in Brief IQ-score was seen between the groups. The age-adjusted motor performance of all subjects was below -2 SD, but the GH-treated subjects performed better than the controls in all but one subtest. The combined finding of a greater head circumference SDS and better psychomotor performance indicates that DS subjects may benefit from early GH treatment.

  11. Pseudotumor Cerebri Resulting in Empty Sella Syndrome and Multiple Pituitary Hormone Deficiencies

    Science.gov (United States)

    2017-09-16

    34 ~ ~ ~,,, "’vs l!?~ We present a case of PTC with empty sella syndrome and multiple pituitary hormone deficiencies that was undiagnosed for several ...REPORT TYPE 09/16/2017 Poster 4. TITLE AND SUBTITLE Pseudotu1nor Cercbri Rc~ulling in E1npty ~ella Syndrome and J\\ilultiple Pituitary Honnone...specific neurologic symptoms or more rarely with pitu itary dysfunction. This case highlights a patient with PTC and secondary empty sella syndrome whose

  12. Complications des syndromes drepanocytaires majeurs a l'hopital ...

    African Journals Online (AJOL)

    Le paludisme a été diagnostiqué dans 31,65%. Conclusion : Les CVO, le paludisme, les anémies, la cholécystite et l'ostéomyélite étaient les complications les plus rencontrées chez les drépanocytaires à l'HNN. Mots clés : Drépanocytose, Complications, Niamey, Niger. ABSTRACT. COMPLICATIONS OF MAJOR SICKLE ...

  13. Neuromyelitis Optica in Pregnancy Complicated by Posterior Reversible Encephalopathy Syndrome, Eclampsia and Fetal Death

    OpenAIRE

    Igel, Catherine; Garretto, Diana; Robbins, Matthew S.; Swerdlow, Michael; Judge, Nancy; Dayal, Ashlesha

    2014-01-01

    Neuromyelitis optica (NMO) is a demyelinating syndrome characterized by optic neuritis and acute myelitis with poor recovery and a progressive course. We report a poor outcome complicated by posterior reversible encephalopathy syndrome (PRES) and eclampsia and review available literature and current evidence for anticipation of adverse fetal and maternal effects. After a pregnancy complicated by multiple admissions for painful NMO exacerbations, a primiparous patient with seropositive NMO pre...

  14. A rare case of normotensive HELLP syndrome complicated with massive ascites: Spontaneous resolution

    Directory of Open Access Journals (Sweden)

    Ahmed Samy El-Agwany

    2016-01-01

    Full Text Available HELLP develops in approximately 0.1–0.8% of pregnancies overall and as many as 15–20% of patients with HELLP syndrome do not have antecedent hypertension or proteinuria. The risk factor for development of ascites is extensive structural damage of the microvasculature in patients complicated by HELLP. The aim of this study is to report a case with HELLP syndrome complicated with massive ascites after vaginal delivery that eventually resolved spontaneously.

  15. The abdominal compartment syndrome : A complication with many faces

    NARCIS (Netherlands)

    Berger, P.; Nijsten, MWN; Paling, JC; Zwaveling, JH

    Background: Lately renewed attention has been given to the abdominal compartment syndrome. Despite of this there still remain a lot of controversies with regard to the pathophysiological mechanisms underlying this syndrome and the therapeutic options. Methods: Two cases of patients with this

  16. Grisel's syndrome: A rare complication following traditional uvulectomy

    African Journals Online (AJOL)

    Grisel's syndrome is a nontraumatic atlantoaxial subluxation, usually secondary of an infection or an inflammation at the head and neck area, or after surgery in the same area. Patients typically suffer from painful torticollis. Diagnosis of Grisel's syndrome is largely based on suspicion of the patient who has recently ...

  17. Growth hormone deficiency and pituitary malformation in a recurrent Cat-Eye syndrome: a family report.

    Science.gov (United States)

    Jedraszak, Guillaume; Braun, Karine; Receveur, Aline; Decamp, Matthieu; Andrieux, Joris; Rabbind Singh, Amrathlal; Copin, Henri; Bremond-Gignac, Dominique; Mathieu, Michèle; Rochette, Jacques; Morin, Gilles

    2015-10-01

    Growth hormone deficiency affects roughly between one in 3000 and one in 4000 children with most instances of growth hormone deficiency being idiopathic. Growth hormone deficiency can also be associated with genetic diseases or chromosome abnormalities. Association of growth hormone deficiency together with hypothalamic-pituitary axis malformation and Cat-Eye syndrome is a very rare condition. We report a family with two brothers presenting with growth delay due to a growth hormone deficiency associated with a polymalformation syndrome. They both displayed pre-auricular pits and tags, imperforate anus and Duane retraction syndrome. Both parents and a third unaffected son displayed normal growth pattern. Cerebral MRI showed a hypothalamic-pituitary axis malformation in the two affected brothers. Cytogenetic studies revealed a type I small supernumerary marker chromosome derived from chromosome 22 resulting in a tetrasomy 22pter-22q11.21 characteristic of the Cat-Eye syndrome. The small supernumerary marker chromosome was present in the two affected sons and the mother in a mosaic state. Patients with short stature due to growth hormone deficiency should be evaluated for chromosomal abnormality. Family study should not be underestimated. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

  18. Pregnancy-related deaths and complications in women with vascular Ehlers-Danlos syndrome.

    Science.gov (United States)

    Murray, Mitzi L; Pepin, Melanie; Peterson, Suzanne; Byers, Peter H

    2014-12-01

    The purpose of this study was to characterize the nature and magnitude of pregnancy risks in women with vascular Ehlers-Danlos syndrome. Pregnancy-related death rate was determined by a review of pedigrees of families with vascular Ehlers-Danlos syndrome. Maternal morbidity was characterized through semistructured interviews with women with vascular Ehlers-Danlos syndrome or their next of kin. Pregnancy-related deaths occurred in 30 of 565 deliveries (5.3%). There was no difference in Kaplan-Meier survival curves between parous versus nulliparous women with vascular Ehlers-Danlos syndrome. Interviews with 39 women indicated that 46% of deliveries were uncomplicated. The most common pregnancy-related complications were third-/fourth-degree lacerations (20%) and preterm delivery (19%). Life-threatening complications occurred in 14.5% of deliveries and included arterial dissection/rupture (9.2%), uterine rupture (2.6%), and surgical complications (2.6%). There were 5 maternal deaths in 76 deliveries (6.5%). The risk of pregnancy-related complications is increased in women with vascular Ehlers-Danlos syndrome compared with the general population; however, survival data indicate that pregnancy does not appear to affect overall mortality compared with nulliparous women with vascular Ehlers-Danlos syndrome. The data were insufficient to determine whether mode or timing of delivery influenced risk of complications. Women with vascular Ehlers-Danlos syndrome should be engaged in a shared decision-making process when contemplating pregnancy and pregnancy management.

  19. Hormones

    Science.gov (United States)

    Hormones are your body's chemical messengers. They travel in your bloodstream to tissues or organs. They work ... glands, which are special groups of cells, make hormones. The major endocrine glands are the pituitary, pineal, ...

  20. Le syndrome de Budd-Chiari: une complication rare de la ...

    African Journals Online (AJOL)

    L'atteinte hépatique au cours de la sarcoïdose est une localisation fréquente, habituellement asymptomatique. La cholestase anictérique et l'hypertension portale représentent ses principales complications. Le syndrome de Budd-Chiari est une complication peu connue qui demeure exceptionnelle. Nous rapportons un ...

  1. Iliotibial band syndrome following hip arthroscopy: An unreported complication

    Directory of Open Access Journals (Sweden)

    Roberto Seijas

    2016-01-01

    Conclusions: This is a newly described observation within followup of hip arthroscopy. These findings may help orthopedic surgeons when planning rehabilitation after hip arthroscopy, including stretching exercises to prevent this syndrome.

  2. Fusobacterium necrophorum Pharyngitis Complicated by Lemierre’s Syndrome

    National Research Council Canada - National Science Library

    Faraone, Antonio; Fortini, Alberto; Nenci, Gabriele; Boccadori, Costanza; Mangani, Valerio; Oggioni, Roberto

    2016-01-01

    .... Blood cultures were positive for Fusobacterium necrophorum. CT scan detected a parapharyngeal abscess, a right internal jugular vein thrombosis, and multiple bilateral lung abscesses, suggesting the diagnosis of Lemierre’s syndrome...

  3. Posterior reversible encephalopathy syndrome: An atypical postpartum complication

    National Research Council Canada - National Science Library

    Paul, Debashish; Kulkarni, SachinNarayan; Choudhury, MiliDas; Maity, GD

    2016-01-01

    Posterior reversible encephalopathy syndrome (PRES) is presented by headache, altered mental status, blurring of vision, vomiting and seizure in conjunction with radiological finding of posterior cerebral white matter edema...

  4. Sick sinus syndrome as a complication of mediastinal radiation therapy

    Energy Technology Data Exchange (ETDEWEB)

    Pohjola-Sintonen, S.; Toetterman, K.J.K.; Kupari, M. (Helsinki Univ. Central Hospital (Finland))

    1990-06-01

    A 33-year-old man who had received mediastinal radiation therapy for Hodgkin's disease 12 years earlier developed a symptomatic sick sinus syndrome requiring the implantation of a permanent pacemaker. The sick sinus syndrome and a finding of an occult constrictive pericarditis were considered to be due to the previous mediastinal irradiation. A ventricular pacemaker was chosen because mediastinal radiotherapy also increases the risk of developing atrioventricular conduction defects.

  5. Reproductive hormones and metabolic syndrome in 24 testicular cancer survivors and their biological brothers

    DEFF Research Database (Denmark)

    Bandak, M.; Jorgensen, N.; Juul, A.

    2017-01-01

    of luteinizing hormone and follicle-stimulating hormone were elevated (p ≤ 0.001), while total testosterone, free testosterone, inhibin B and anti-Müllerian hormone were lower (p ≤ 0.001) in testicular cancer survivors than in their biological brothers. The prevalence of metabolic syndrome was similar and apart......Testicular cancer survivors have impaired gonadal function and increased risk of metabolic syndrome when compared to healthy controls. However, because of the fetal etiology of testicular cancer, familial unrelated healthy men might not be an optimal control group. The objective of this study...... was to clarify if testicular cancer survivors have impaired gonadal function and increased risk of metabolic syndrome when compared to their biological brothers. A cross-sectional study of testicular cancer survivors (ClinicalTrials.gov number, NCT02240966) was conducted between 2014 and 2016. Of 158 testicular...

  6. Final height in girls with Turner's syndrome treated with once or twice daily growth hormone injections. Dutch Advisory Group on Growth Hormone

    NARCIS (Netherlands)

    T.C.J. Sas (Theo); T. Vulsma (Thomas); W.J. Gerver; S.L.S. Drop (Stenvert); H.M. Reeser (Maarten); M. Jansen (Maarten); S.M.P.F. de Muinck Keizer-Schrama (Sabine); Th. Stijnen (Theo); A. van Teunenbroek (Arne); A.C.S. Hokken-Koelega (Anita); J.J.J. Waelkens (Johan); G.G. Massa; H.A. Delemarre-van de Waal (Henriette)

    1999-01-01

    textabstractOBJECTIVES: To study final height in girls with Turner's syndrome treated with once or twice daily injections of growth hormone (GH) in combination with low dose ethinyl oestradiol. DESIGN: Until final height was reached, the effect of fractionated

  7. Acute brain ischemia as a complication of the Ehlers-Danlos syndrome, the case series.

    Science.gov (United States)

    Pajak, Michal; Majos, Marcin A; Szubert, Wojciech; Stefanczyk, Ludomir; Majos, Agata

    2014-10-01

    Vascular type of Ehlers-Danlos syndrome involves many severe complications leading not only to organ-specific symptoms but often ends in a sudden death. The aim of this paper was to present a diagnostic possibilities and its efficiency rate in patients with vascular complications of Ehlers-Danlos syndrome who suffered from artery dissection resulting in acute brain or limb ischemia. We analysed three patients with diagnosed Ehlers-Danlos syndrome who were referred to radiology department for diagnostic imaging of affected vascular beds, each experienced brain ischemia. The paper also aims at offering some general recommendations for patients suffering from possible complications of type IV Ehlers-Danlos syndrome basing on our own experience and available literature data. © The Author(s) 2013 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  8. Scimitar syndrome and pregnancy, complicated with severe preeclampsia.

    Science.gov (United States)

    Yuksel, Mehmet Aytac; Imamoglu, Metehan; Dincgez Cakmak, Burcu; Oncul, Mahmut; Madazli, Riza

    2014-06-01

    Scimitar syndrome (pulmonary venolobar syndrome) is a rare anomaly of venous return to the heart, most commonly consisting of partial or total anomalous pulmonary venous return from the right lung. This is the report of a case of a 29-year-old woman at 31 weeks of gestation of pregnancy who was previously diagnosed with scimitar syndrome. MR angiography and PET-CT results which were obtained before pregnancy demonstrated vascular malformation in the inferior part of the right lung. No specific treatment was planned throughout the pregnancy due to the absence of any symptoms. The patient's first physical examination was unremarkable except mild hypertension. In her follow-up, severe preeclampsia was developed and the patient had undergone a cesarean section of a live birth at 34 weeks and 2 days of gestation. This is the first case of scimitar syndrome with pregnancy in which the cardiac status of the patient deteriorated coincidentally due to the development of another manifestation such as severe preeclampsia besides the syndrome itself.

  9. Pharmacological management of metabolic syndrome and its lipid complications

    Directory of Open Access Journals (Sweden)

    T Binesh Marvasti

    2010-09-01

    Full Text Available Obesity epidemic has been spread all over the world in the past few decades and has caused a major public health concern due to its increasing global prevalence. Obese individuals are at higher risks of developing dyslipidemic characteristics resulting in increased triglyceride and LDL-cholesterol content and reduced HDL-cholesterol levels. This disorder has profound implications as afflicted individuals have been demonstrated to be at increased risk of development of hypertension, atherosclerosis, type 2 diabetes and cardiovascular diseases. Today, this phenotype is designated as metabolic syndrome. According to the criteria set by the International Diabetes Federation (IDF, for a patient to be diagnosed with metabolic syndrome, the person must have central obesity plus any two of the following conditions: raised TG, reduced HDL-cholesterol, raised blood pressure, and increased fasting plasma glucose. Current National Cholesterol Education Program Adult Treatment Panel III (NCEP-ATP III guidelines for the treatment of patients with the metabolic syndrome encourage therapies that lower LDL cholesterol and TG and raise HDL-cholesterol. Primary intervention often involves treatment with statins to improve the lipid profiles of these patients. However, recent studies suggest the potential of newly identified drugs including thiazolidinediones, GLP-1 agonists, and DPP-4 inhibitors that seem to be promising in reducing the level of progression of metabolic syndrome related disorders. This review discusses the current pharmacological treatments of the metabolic syndrome with the above mentioned drugs.

  10. Correlation between ovarian morphology and biochemical and hormonal parameters in polycystic ovary syndrome

    Science.gov (United States)

    Inan, Cihan; Karadag, Cihan

    2016-01-01

    Objective: To determine the biochemical and hormonal differences in polycystic ovary syndrome (PCOS) patients with and without polycystic ovary (PCO) morphology and to evaluate the outcomes resulting from those differences. Methods: The study included a total of 83 patients with PCOS; 43 of them had PCO morphology (Group-I) and 40 did not (Group-II). Serum LDL, HDL, total cholesterol, triglyceride (TG), total testosterone (T), follicle stimulating hormone (FSH), luteinizing hormone (LH), 17b-estradiol (E2), prolactin (PRL), thyroid stimulating hormone (TSH), sex hormone binding globulin (SHBG), glucose and insulin levels were determined. Homoeostatic model assessment insulin resistance (HOMA-IR) index was calculated. Results: The two groups were similar with respect to BMI. The systolic and diastolic blood pressure measurements of Group-I were significantly lower (pinsulin resistance, dyslipidemia and cardiovascular diseases due to effects of hyperandrogenism. PMID:27375725

  11. What is the value of growth hormone therapy in Prader Willi syndrome?

    Science.gov (United States)

    Bridges, Nicola

    2014-02-01

    Prader Willi syndrome (PWS) is a genetic condition caused by loss of the paternal copy of a region of imprinted genes on chromosome 15. There is severe muscular hypotonia in the neonatal period, with the onset of hyperphagia and food-seeking behaviour in childhood. All individuals with PWS have developmental delay. Without careful control of food intake and the food environment, individuals with PWS become morbidly obese and are likely to die as young adults from the complications of obesity. The aims of growth hormone (GH) treatment in PWS are distinct from the use of GH in other conditions-although GH does increase final height in PWS, the main benefits of treatment are improved body composition and better exercise capacity, which can help with the aim of preventing obesity. GH trials in PWS have demonstrated improved muscle bulk, reduced fat mass and increased levels of physical activity. GH has also been demonstrated to improve attainment of developmental and cognitive milestones in children with PWS. GH treatment appears to change respiratory status in PWS, possibly because of growth of lymphoid tissue at the start of treatment. Respiratory assessment is recommended prior to, and just after starting GH treatment. Ideal age for starting GH is not clear, although there has been a trend towards starting at younger ages. It may be that GH treatment in childhood confers benefits into adult life. There are less data to support continuing GH treatment into adult life.

  12. Ductal carcinoma In-Situ in turner syndrome patient undergoing hormone replacement therapy: A case report

    Directory of Open Access Journals (Sweden)

    Rashmi Bawa

    2016-03-01

    Full Text Available Turner’s syndrome is a rare congenital disease which affects about 1 in every 2500-3000 live-born females. This happens due to chromosomal abnormalities in a phenotypic female, causing increased gonadotropin concentrations and low concentrations of estrogens from infancy. As a result, hormone replacement therapy is started in most adolescent Turner syndrome patients to initiate and sustain sexual maturation. Accordingly, most Turner’s syndrome patients undergo several decades of estrogen replacement therapy, from puberty to post-menopausal age. The highly publicized findings of the Women’s Health Initiative have called into question the appropriateness of hormone replacement therapy in adolescents with Turner’s syndrome. Those concerns were mostly theoretical extrapolations, as few prospective studies of cancer occurrence in women with Turner syndrome have been reported. Consequently, several recent publications have challenged those extrapolations, based on the assertion that the levels of hormone replacement in Turner syndrome patients are well below the physiologic levels observed in normal menstruating women, as well as the fact that these women are significantly younger than those studied by the Women’s Health Initiative. In discord to those reports, we present a case of ductal carcinoma in-situ in a 40-year-old Turner patient, who had undergone over two decades of combined hormone replacement therapy. The patient underwent an elective excisional biopsy for a palpable mass, with histopathology revealing a complex fibroadenoma with a nidus of ductal carcinoma in-situ. The lesion was noted to be estrogen receptor positive and progesterone receptor negative, with heavy staining for HER-2/Neu receptor. The patient was treated with tamoxifen. While a rare case, it is imperative for the astute clinician to keep in mind the consequences of long-term hormone replacement therapy in Turner’s syndrome patients in order to avoid missed

  13. Case of polyhydramnios complicated by Opitz G/BBB syndrome.

    Science.gov (United States)

    Tajima, Hiroko; Itoh, Hiroaki; Mochizuki, Ayako; Nakamura, Yuki; Kobayashi, Yukiko; Hirai, Kyuya; Suzuki, Kazunao; Sugihara, Kazuhiro; Ohishi, Akira; Ohzeki, Takehiko; Kanayama, Naohiro

    2010-08-01

    Opitz G/BBB syndrome is a congenital disorder characterized by midline defects, such as hypertelorism, cleft lip and/or palate, hypospadias, and by dysphagia often caused by laryngo-tracheo-esophageal abnormalities. We experienced a case of polyhydramnios in a male dichorionic diamniotic (DD) twin, who was diagnosed with Opitz G/BBB syndrome after birth. It is suggested that severe dysphagia was causatively associated with the development of polyhydramnios. In cases of Opitz G/BBB syndrome, boys are usually more heavily affected than girls, who generally manifest only hypertelorism. In the differential diagnosis of polyhydramnios of unidentified cause in male fetuses, it may be helpful to consider maternal facial characteristics, especially hypertelorism.

  14. Down Syndrome Increases the Risk of Short-Term Complications After Total Hip Arthroplasty.

    Science.gov (United States)

    Boylan, Matthew R; Kapadia, Bhaveen H; Issa, Kimona; Perfetti, Dean C; Maheshwari, Aditya V; Mont, Michael A

    2016-02-01

    Down syndrome is the most common chromosomal abnormality and is associated with degenerative hip disease. Because of the recent increase in life expectancy for patients with this syndrome, orthopaedic surgeons are likely to see an increasing number of these patients who are candidates for total hip arthroplasty (THA). Using Nationwide Inpatient Sample (NIS) data from 1998 to 2010, we compared the short-term adverse outcomes of THA among 241 patients with Down syndrome and a matched 723-patient cohort. Specifically, we assessed: (1) incidence of THA; (2) perioperative medical and surgical complications during the primary hospitalization; (3) length of stay; and (4) hospital charges. The annual mean number of patients with Down syndrome undergoing THA was 19. Compared to matched controls, Down syndrome patients had an increased risk of perioperative (OR, 4.33; PDown syndrome patients had significantly higher incidence rates of pneumonia (P=.001), urinary tract infection (PDown syndrome patients were 26% longer (Pevaluation and pre-operative consultation for a patient with Down syndrome who is a candidate for THA, orthopaedic surgeons should educate the patient, family and their clinical decision makers about the increased risk of medical complications (pneumonia and urinary tract infections), surgical complications (wound hemorrhage), and lengths of stay compared to the general population. Copyright © 2016 Elsevier Inc. All rights reserved.

  15. Moving bullet syndrome: a complication of penetrating head injury.

    Science.gov (United States)

    Zafonte, R D; Watanabe, T; Mann, N R

    1998-11-01

    Penetrating injuries, by definition, result in retained bullets or fragments. Usually, these fragments are removed surgically during wound debridement. Occasionally, the position of the bullet may preclude removal if it is thought that surgery could exacerbate neurologic damage. Complications from retained fragments are uncommon. One rare complication is the spontaneous migration of the fragment. Two cases of spontaneous migration of retained bullets are presented. In both cases neurologic deterioration was noted and computed tomographic imaging was diagnostic. In one case, this complication delayed transfer from the acute care hospital to rehabilitation. In the other case, the migrating bullet was removed during the inpatient rehabilitation stay. Each person improved neurologically after the migrating bullet fragment was removed. Additionally, functional progress was marked in both persons and symptomatic relief noted. Rehabilitation physicians caring for survivors of penetrating brain injuries need to be aware of this potentially devastating phenomenon.

  16. Exacerbation of BMI after cessation of growth hormone therapy in patients with Prader-Willi syndrome.

    Science.gov (United States)

    Oto, Yuji; Tanaka, Yuriko; Abe, Yoshiko; Obata, Kazuo; Tsuchiya, Takayoshi; Yoshino, Atsunori; Murakami, Nobuyuki; Nagai, Toshiro

    2014-03-01

    Long-term treatment with growth hormone (GH) in patients with Prader-Willi syndrome (PWS) improves not only height velocity, height standard deviation score, and final height, but also the degree of obesity and body composition abnormalities. Anecdotally, PWS patients tend to suffer from severe obesity and its complications after cessation of GH therapy. However, there have been no studies to investigate changes in body mass index (BMI) and adipose tissue distribution after cessation of GH therapy in young PWS patients. Therefore, we investigated changes in the BMI-standard deviation score (SDS) and adipose tissue distribution after cessation of GH therapy in PWS patients. We evaluated 14 PWS patients. BMI-SDS was calculated at 0, 6, 12, 18, and 24 months before and after cessation of GH treatment. We also evaluated subcutaneous adipose tissue (SAT) (cm(2)) and visceral adipose tissue (VAT) (cm(2)) area in 8 of the 14 study patients with single slice abdominal computed tomography at the level of the umbilicus. The BMI-SDS significantly increased at 6, 12, 18, and 24 months after cessation of GH therapy (P = 0.039, P = 0.008, P = 0.003, P = 0.003, respectively). There was a tendency toward increases in VAT at 12 and 24 months after cessation of GH therapy, but the increases did not reach statistical significance (P = 0.062, P = 0.125, respectively). Therefore, cessation of GH therapy in PWS patients worsened BMI. To maintain good body composition and prevent complications of obesity, long-term use of GH in adult PWS patients may be advisable. © 2014 Wiley Periodicals, Inc.

  17. Post-Fundoplication Dumping Syndrome: A Frequent "Rare" Complication.

    Science.gov (United States)

    Kataria, Rahul; Linn, Sandar; Malik, Zubair; Abbas, Abbas E; Parkman, Henry; Schey, Ron

    2018-01-01

    Gastroparesis, caused by delayed emptying of the stomach, has been shown to be associated with Nissen fundoplication. However, symptomatic rapid emptying of the stomach is rare after Nissen fundoplication, and its treatment is often challenging. We report 2 patients with dumping-like syndrome post-fundoplication with marked improvement of symptoms after dietary management and medical treatment.

  18. empty sella syndrome complicating meningitis: a case report

    African Journals Online (AJOL)

    communicating hydrocephalus with empty sella syndrome. (Refer. films I and 11). She was treated with carbamazepine 600mg daily in divided doses for seizure control and oral dexamethasone 4mg tid to reduce intracranial pressure. She responded well with cessation of seizures and was referred for ventricular shunting.

  19. Clinical features and respiratory complications in Myhre syndrome

    NARCIS (Netherlands)

    McGowan, Ruth; Gulati, Ramkumar; McHenry, Pamela; Cooke, Alexander; Butler, Sandra; Keng, Wee Teik; Murday, Victoria; Whiteford, Margo; Dikkers, Frederik G.; Sikkema-Raddatz, Brigit; van Essen, Ton; Tolmie, John

    2011-01-01

    We describe the clinical characteristics of 4 singleton cases, 3 males and 1 female, with Myhre Syndrome (OMIM 139210), who were born to non-consanguineous parents. Three cases had no family history of similarly affected individuals but 1 male's mother had short stature, some facial features

  20. Primary growth hormone insensitivity (Laron syndrome and acquired hypothyroidism: a case report

    Directory of Open Access Journals (Sweden)

    Corneli Ginevra

    2011-07-01

    Full Text Available Abstract Introduction Primary growth hormone resistance or growth hormone insensitivity syndrome, also known as Laron syndrome, is a hereditary disease caused by deletions or different types of mutations in the growth hormone receptor gene or by post-receptor defects. This disorder is characterized by a clinical appearance of severe growth hormone deficiency with high levels of circulating growth hormone in contrast to low serum insulin-like growth factor 1 values. Case presentation We report the case of a 15-year-old Caucasian girl who was diagnosed with Silver-Russell syndrome at the age of four and a half years. Recombinant growth hormone was administered for 18 months without an appropriate increase in growth velocity. At the age of seven years, her serum growth hormone levels were high, and an insulin-like growth factor 1 generation test did not increase insulin-like growth factor 1 levels (baseline insulin-like growth factor 1 levels, 52 μg/L; reference range, 75 μg/L to 365 μg/L; and peak, 76 μg/L and 50 μg/L after 12 and 84 hours, respectively, from baseline. The genetic analysis showed that the patient was homozygous for the R217X mutation in the growth hormone receptor gene, which is characteristic of Laron syndrome. On the basis of these results, the diagnosis of primary growth hormone insensitivity syndrome was made, and recombinant insulin-like growth factor 1 therapy was initiated. The patient's treatment was well tolerated, but unexplained central hypothyroidism occurred at the age of 12.9 years. At the age of 15 years, when the patient's sexual development was almost completed and her menstrual cycle occurred irregularly, her height was 129.8 cm, which is 4.71 standard deviations below the median for normal girls her age. Conclusion The most important functional tests for the diagnosis of growth hormone insensitivity are the insulin-like growth factor 1 generation test and genetic analysis. Currently, the only effective

  1. Cat eye syndrome and growth hormone deficiency with pituitary anomalies: a case report and review of the literature.

    Science.gov (United States)

    Melo, Cláudia; Gama-de-Sousa, Susana; Almeida, Filipa; Rendeiro, Paula; Tavares, Purificação; Cardoso, Helena; Carvalho, Sónia

    2013-10-15

    Cat eye syndrome is a rare congenital disease characterized by the existence of a supernumerary chromosome derived from chromosome 22, with a variable phenotype comprising anal atresia, coloboma of the iris and preauricular tags or pits. We report a girl with cat eye syndrome, presenting short stature, with growth hormone deficiency due to posterior pituitary ectopia. Short stature is a common feature of this syndrome, and the association with a structural pituitary anomaly has been described, however growth hormone deficiency and the underlying mechanisms are rarely reported. A review on short stature and growth hormone deficiency in cat eye syndrome is conducted. © 2013 Elsevier B.V. All rights reserved.

  2. Eye Complications of Acquired Immune Deficiency Syndrome (AIDS ...

    African Journals Online (AJOL)

    HIV associated eye disease now occurs in 50-90% of patients at one point in the course of their illness. Loss of sight is a feared complication because of its impact on the management of the systemic disease and the additional distress experienced by the patient who now has to rely on others for assistance with activities of ...

  3. [Current comprehensive diagnosis of pancreatobiliary cancer complicated by jaundice syndrome].

    Science.gov (United States)

    Kharchenko, V P; Liutfaliev, T A; Kunda, M A

    2000-01-01

    The study was undertaken to enhance the efficiency of diagnosis of pancreatobiliary cancer complicated by jaundice by using the potentialities of currently available instrumental studies at most. The data of examination of 256 patients with this disease are presented. Based on diagnostic findings, the authors have constructed a diagnostic model of precision recognition of cancer of the extrahepatic bile ducts, pancreatic head, Vater's papilla.

  4. Relation between Hormonal Disorders and Components of Metabolic Syndrome in Patients with Primary Hypothyroidism

    Directory of Open Access Journals (Sweden)

    Т.Yu. Yuzvenko

    2016-09-01

    Full Text Available During the last decade plenty of the researches dedicated to the problem of hypothyroidism were published, that radically changed views to the value of thyroid pathology on the whole. Neurohumoral changes are considered as a nosotropic factor of hypothyroidism development in persons with metabolic syndrome (MS. Aim of the research is to study the features of hormonal disorders and their correlation with the components of metabolic syndrome in patients with primary hypothyroidism. Materials and methods. The study involved 80 patients with primary hypothyroidism: 61 had metabolic syndrome and 19 did not have metabolic syndrome. Results. Statistically significant increased levels of leptin, insulin, cortisol, C-peptide were revealed in patients with hypothyroidism and metabolic syndrome while the most marked changes were found in patients with multiple metabolic abnormalities. Conclusions. The interrelations between hyperleptinemia and fasting glucose, glycated hemoglobin, insulin levels, thyroid-stimulating hormone, index HOMA were determined indicating the modulating role of chronic hyperglycemia, hormonal disorders and insulin resistance in the expression and realization of the biological action of leptin in patients with hypothyroidism and metabolic syndrome.

  5. Pneumomediastinum Complicating Diabetic Ketoacidosis and Boerhaave’s Syndrome

    Directory of Open Access Journals (Sweden)

    Samer Alkhuja

    2013-01-01

    Full Text Available An 18-year-old man presented with altered mental status. He was found to have diabetic ketoacidosis. Chest X-ray showed pneumomediastinum. After intubation for air-way protection, an oral-gastric tube was placed. A chest computed tomography scan showed the tip of the oral-gastric tube to be in the right hemithorax. The patient underwent a thoracotomy and was managed in the intensive care unit. Both diabetic ketoacidosis and Boerhaave's syndrome should be considered as possible causes of pneumomediastinum in a patient with similar presentation. Boerhaave's syndrome should be ruled out prior to the insertion of an oral-gastric tube to avoid further morbidities.

  6. An unusual complication after craniofacial surgery for Apert syndrome

    Directory of Open Access Journals (Sweden)

    Abhay A Lune

    2014-01-01

    A case of Apert syndrome who had undergone craniofacial surgery elsewhere 4 years back presented to us with purulent discharge near the lateral orbital margin of right orbit, watering and redness of the right eye. He had telltale signs of this syndrome in the form of skull deformities such as brachycephaly, frontal bony prominence, mid-face hypoplasia, proptosis and syndactyly of both hands and feet. There was a surgical scar of previous craniofacial surgery over the bi-coronal region. He had a discharging granuloma over the lateral orbital margin and the adjacent lower eyelid had developed cicatricial ectropion. X-ray and computed tomography scan orbit confirmed the clinical suspicion of osteomyelitis of the underlying zygomatic bone at the site of miniplate and screw fixation of the earlier surgery. He was treated with excision of granuloma and extraction of loose screw and infected miniplate while ectropion was corrected by rotation advancement of temporal skin flap.

  7. Macrophage activation syndrome: a potentially fatal complication of rheumatic disorders

    OpenAIRE

    Sawhney, S.; Woo, P; Murray, K.

    2001-01-01

    AIMS—To review the precipitating events, clinical features, treatment, and outcome of macrophage activation syndrome (MAS).
METHODS—Retrospective review of cases of MAS from a prospectively collected database of children with rheumatic diseases from 1980to 2000.
RESULTS—Nine patients (eight girls) were considered to have evidence of MAS. The primary diagnosis was systemic onset juvenile idiopathic arthritis in seven, enthesitis related arthritis in one, and chronic infant...

  8. Pulsatile luteinising hormone releasing hormone for ovulation induction in subfertility associated with polycystic ovary syndrome

    NARCIS (Netherlands)

    Bayram, N.; van Wely, M.; Vandekerckhove, P.; Lilford, R.; van der Veen, F.

    2000-01-01

    BACKGROUND: In normal menstrual cycles, gonadotrophin releasing hormone (GnRH) secretion is pulsatile, with intervals of 60-120 minutes in the follicular phase. Treatment with pulsatile GnRH infusion by the intra-venous or subcutaneous route using a portable pump has been used successfully in

  9. Impact of metformin on anti-Müllerian hormone in women with polycystic ovary syndrome

    DEFF Research Database (Denmark)

    Madsen, Helen N; Lauszus, Finn; Trolle, G. Birgitta

    2015-01-01

    Conclusions on the effect of metformin on circulating anti-Müllerian hormone (AMH) levels in women with polycystic ovary syndrome (PCOS) are ambiguous. We performed a secondary analysis of a randomized, double-blind, placebo-controlled cross-over trial. Fifty-six women with hyperandrogenemic PCOS...

  10. The effect of oxandrolone on voice frequency in growth hormone-treated girls with Turner syndrome

    NARCIS (Netherlands)

    Menke, L.A.; Sas, T.C.J.; Koningsbrugge, S.H. van; Ridder, M.A. de; Zandwijken, G.R.; Boersma, B.; Dejonckere, P.H.; Muinck Keizer-Schrama, S.M.P.F. de; Otten, B.J.; Wit, J.M.

    2011-01-01

    OBJECTIVES/HYPOTHESIS: Oxandrolone (Ox) increases height gain but may also cause voice deepening in growth hormone (GH)-treated girls with Turner syndrome (TS). We assessed the effect of Ox on objective and subjective speaking voice frequency in GH-treated girls with TS. STUDY DESIGN: A multicenter,

  11. PSYCHOSOCIAL EFFECTS OF 2 YEARS OF HUMAN GROWTH-HORMONE TREATMENT IN TURNER SYNDROME

    NARCIS (Netherlands)

    SLIJPER, FME; SINNEMA, G; AKKERHUIS, GW; BRUGMANBOEZEMAN, A; FEENSTRA, J; DENHARTOG, L; HEUVEL, F

    1993-01-01

    Thirty-eight girls with Turner syndrome were treated for 2 years with human growth hormone. Both parents and patients carried out assessments of the effects of treatment on various aspects of psychosocial functioning. The children used the Piers-Harris Self-Concept Scale and the Social Anxiety Scale

  12. Growth hormone therapy, muscle thickness, and motor development in Prader-Willi Syndrome: An RCT

    NARCIS (Netherlands)

    Reus, L.; Pillen, S.; Pelzer, B.J.; Velden, J.A.M. van der; Hokken-Koelega, A.C.; Zwarts, M.; Otten, B.J.; Nijhuis-Van der Sanden, M.W.G.

    2014-01-01

    OBJECTIVE: To investigate the effect of physical training combined with growth hormone (GH) on muscle thickness and its relationship with muscle strength and motor development in infants with Prader-Willi syndrome (PWS). METHODS: In a randomized controlled trial, 22 infants with PWS (12.9 +/- 7.1

  13. Personalized treatment of obesity complicated with obstructive sleep apnea syndrome

    Directory of Open Access Journals (Sweden)

    N V Strueva

    2014-03-01

    Full Text Available Obstructive sleep apnea (OSA and obesity are mutually burdening clinical conditions. Weight loss is quite effective for the control of breathing disorders during sleep, but the impact of OSA treatment on the dynamics of body weight in obese patients remains poorly studied. Presented clinical case features a significant reduction in body weight in a patient with morbid obesity complicated by severe OSA with contemporary approach and without CPAP therapy due to high patient adherence to therapy and absence of comorbidities.

  14. A Rare Case of Morel-Lavallee Syndrome Complicating an Anterior Dislocation of Hip Joint.

    Science.gov (United States)

    Nekkanti, Supreeth; Vijay, C; Theja, Sujana; Shankar, R Ravi; Verma, Anubhav

    2016-01-01

    Hip dislocations are serious injuries as hip joint is an extremely stable joint. It requires a significant amount of force to produce such an injury. Anterior dislocations are uncommon. Potential complications of anterior hip dislocations are a neurovascular injury to femoral vessels or acetabular fractures. We report a rare late complication of Morel-Lavallee syndrome occurring 3 weeks after an anterior dislocation of the hip in a 43-year-old male. The patient presented to us with history. Morel-Lavallee syndrome is a rare complication. However if diagnosed early can be successfully treated with minimal burden to the patient. The authors recommend surgeons to have a high index of suspicion for this syndrome and a stringent follow-up examination of the patient.

  15. [Clinical Characteristics and Gene Mutations of Gilbert Syndrome Complicated with Myeloproliferative Neoplasm].

    Science.gov (United States)

    Li, Xing-Xin; Shi, Jun; Huang, Zhen-Dong; Shao, Ying-Qi; Nie, Neng; Zhang, Jing; Ge, Mei-Li; Huang, Jin-Bo; Zheng, Yi-Zhou

    2017-04-01

    To investigate the clinical characteristics and gene mutations of patients with Gilbert syndrome complicated with myeloproliferative neoplasms (MPN). Peripheral blood samples from 1 patient with Gilbert syndrome complicated with MPN and his son were collected to analyse all exon mutations of UGT1A1 gene. The patient with leukocytosis, thrombocythemia, mild anemia and positive JAK2/V617F mutation was initially diagnosed as MPN. The hyperbilirubinemia suggested concurrent disease. Further gene evaluation disclosed a insertion mutation in the (TA)6TAA box, and a missense mutation(G→A) at 211 bp of exon 1, corresponding to the deficiency in the bilirubin-conjugating enzyme uridine-diphosphoglucuronosyl transferase1A1 (UGT1A1). His son only carried some polymorphism mutation without manifestation of this disease. It is a first report case of MPN complicated with Gilbert syndrome that can highlight the differential diagnosis for hyperbilirubinemia.

  16. Nephrotic syndrome complicated with portal, splenic, and superior mesenteric vein thrombosis

    Directory of Open Access Journals (Sweden)

    Bong Soo Park

    2014-09-01

    Full Text Available Thromboembolism is a major complication of nephrotic syndrome. Renal vein thrombosis and deep vein thrombosis are relatively common, especially in membranous nephropathy. However, the incidence of portal vein and superior mesenteric vein (SMV thrombosis in patients with nephrotic syndrome is very rare. To date, several cases of portal vein thrombosis treated by anticoagulation therapy, not by thrombolytic therapy, have been reported as a complication of nephrotic syndrome. Here, we report a case of portal, splenic, and SMV thrombosis in a patient with a relapsed steroid dependent minimal change disease who was treated successfully with anticoagulation and thrombolytic therapy using urokinase. Radiologic findings and his clinical conditions gradually improved. Six months later, a complete remission of the nephrotic syndrome was observed and the follow-up computed tomography scan showed the disappearance of all portal vein, splenic vein, and SMV thrombi.

  17. Hormone-induced rat model of polycystic ovary syndrome: A systematic review.

    Science.gov (United States)

    Noroozzadeh, Mahsa; Behboudi-Gandevani, Samira; Zadeh-Vakili, Azita; Ramezani Tehrani, Fahimeh

    2017-12-15

    Despite polycystic ovary syndrome (PCOS) being one of the most common endocrine disorders affecting reproductive-aged women, the etiopathogenesis and mechanisms of this syndrome remain unclear. Considering the ethical limitations in human studies, animal models that reflect many features of PCOS are crucial resources to investigate this syndrome. We aimed to introduce the most suitable rat model of PCOS that closely mimics the endocrine, ovarian and metabolic disturbances of human PCOS phenotype, while maintaining normal reproductive system morphology in adulthood, in order to further more detailed investigations about PCOS. We searched Pubmed, Science direct, and Web of science between 1990 and 2016, for relevant English manuscripts, using keywords including the "Polycystic Ovary Syndrome AND Rat Model" to generate a subset of citations relevant to our research. Included were those articles that compared at least both ovarian histology or estrous cycle and reproductive hormonal profiles in hormone-induced rat model of PCOS and controls. Differences in the findings between hormone-induced PCOS rats appear to be a result of the degree of transplacental transfer of the steroid administered into the fetus, dose and type of hormone, route of administration and timing and duration of exposure. We conclude that prenatal hormone-induced rat model with a lower dose and shorter time of exposure during the critical period of fetal development that exhibits endocrine, ovarian and metabolic disturbances similar to PCOS in women, while maintaining normal reproductive system morphology in adulthood is more suitable than postnatal hormone-induced rat model to facilitate studies regarding PCOS. Copyright © 2017 Elsevier Inc. All rights reserved.

  18. Ewing's Sarcoma of the Kidney Complicated by a Wunderlich Syndrome

    Science.gov (United States)

    Manescu, Mihai Razvan; Sahyoun, Achraf; Froment, Nicolas; Crisan, Nicolae; Girot, Vincent

    2015-01-01

    The Wunderlich syndrome found after the rupture of primitive renal Ewing's sarcoma is not a situation that we find often in everyday practice. The clinical findings are not specific, which is why the differential diagnosis must be made with a multitude of benign and malignant renal masses until the correct diagnosis can be made by the pathologist. The CT and MRI images are not characteristic. One treatment option is the multidisciplinary approach; however, the prognosis remains poor for patients with metastatic disease. PMID:25922782

  19. Superior sagittal sinus thrombosis: a rare complication in a child with nephrotic syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Pirogovsky, A.; Adi, M.; Barzilai, N. [Dept. of Radiology, Kaplan Medical Center, Rehovot (Israel); Dagan, A.; Sinai, L.; Sthoeger, D. [Div. of Paediatrics, Kaplan Medical Center, Rehovot (Israel); Tabachnik, E. [Div. of Paediatrics, Kaplan Medical Center, Rehovot (Israel); Paediatric ICU, Kaplan Hospital, Rehovot (Israel)

    2001-10-01

    A 2-year-old boy with new-onset nephrotic syndrome developed recurrent vomiting, apathy and papilloedema. Superior sagittal sinus thrombosis was diagnosed on cranial CT and MRI. He gradually recovered after treatment with heparin, fresh frozen plasma and warfarin with complete resolution of the thrombosis after 1 month. Superior sagittal sinus thrombosis is an extremely rare complication of nephrotic syndrome in children. Early diagnosis is essential for institution of anticoagulation therapy and a successful outcome. (orig.)

  20. Hypothalamic-Pituitary-Adrenal Suppression and Iatrogenic Cushing's Syndrome as a Complication of Epidural Steroid Injections

    OpenAIRE

    Leary, Joyce; Swislocki, Arthur

    2013-01-01

    Epidural steroid injections are well accepted as a treatment for radicular back pain in appropriate candidates. While overall incidence of systemic side effects has not been well established, at least five biochemically proven cases of iatrogenic Cushing's Syndrome have been reported as complications of epidural steroid treatment. We present an additional case of iatrogenic Cushing's Syndrome and adrenal suppression in a middle-aged woman who received three epidural steroid injections over a...

  1. Hereditary multiple exostoses: from genetics to clinical syndrome and complications

    Energy Technology Data Exchange (ETDEWEB)

    Vanhoenacker, Filip M.; Hul, Wim van; Wuyts, Wim; Willems, P.J.; Schepper, Arthur M. de

    2001-12-01

    Objective: To give an overview of genetic, clinical and radiological aspects in two families over four generations with known hereditary multiple exostoses (HME). Methods and material: After linkage analysis in both families to localize the defective gene, mutation analysis was performed in these genes to identify the underlying mutation. In the 31 affected individuals, location, number and morphology and evolution of exostosis, evolution of remodeling defects at the metaphysis, and the extent of possible complications were evaluated on clinical and imaging (plain radiography, computed tomography (CT), and magnetic resonance imaging (MRI)) data over a lifetime period. Results and conclusions: Both families demonstrate the gene defect in the same EXT-2 gene locus on chromosome 11p. Exostoses are preferentially located in the lower extremity (hip, knee and lower leg), humerus, and forearm. Any other bone may be involved, except for the calvaria of the skull and the mandible. Exostoses are rather sessile than pedunculated. Exostosis is rarely present at birth but develops gradually and may persist to grow slowly after closure of the growth plates. Preferential expression of the remodeling defect was seen in the hip, distal femur (trumpet-shaped metaphysis) and forearm (shortening of the ulna with secondary bowing of the radius and development of a pseudo-Madelung deformity). These radiological manifestations start at the age of 4-5 years and become more obvious as the enchondral bone formation progresses with age. Reported complications in these families consist of local entrapment phenomenons (vessel, tendon, nerve), frictional bursitis, and sarcomatous transformation. MRI was able to suggest these complications and is the imaging technique of choice in the evaluation of symptomatic exostoses.

  2. [Vitamin D deficiency rickets complicating Dorfman-Chanarin syndrome].

    Science.gov (United States)

    Barraud, C; Cano, A; Boulay, C; Milh, M; Bollini, G; Chabrol, B

    2015-04-01

    Vitamin D deficiency rickets remains a public health issue in many parts of the world. In France, this diagnosis has almost disappeared since 1992 with routine vitamin D supplementation for children. Therefore, it is more difficult for doctors to identify risk factors and early signs of this disease. In this article, we report a rickets diagnosis acquired by vitamin D deficiency in a child who presented with the onset of a genu valgum and difficulty walking at the age of 9½ years. This patient was a Comorian child followed up from his birth for Dorfman-Chanarin syndrome. Dorfman-Chanarin syndrome is a rare disease, with about 80 cases reported in the literature. It belongs to the group of neutral lipid storage diseases (NLSD) characterized especially on the skin by ichthyosis. This child presented risk factors for vitamin D deficiency (dark skin color, prolonged and exclusive breastfeeding, premature end of supplementation, and particularly severe ichthyosis) that should have alerted us to the risk of vitamin D deficiency and the need for supplementation. This case highlights the importance of vitamin D, especially if there are risk factors such as ichthyosis, and the need to remain watchful in monitoring all chronic diseases. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

  3. Radiation fibrosis syndrome: neuromuscular and musculoskeletal complications in cancer survivors.

    Science.gov (United States)

    Stubblefield, Michael D

    2011-11-01

    Radiation-induced toxicity is a major cause of long-term disability after cancer treatment. Radiation fibrosis describes the insidious pathologic fibrotic tissue sclerosis that can occur in response to radiation exposure. Radiation fibrosis syndrome describes the myriad clinical manifestations of progressive fibrotic tissue sclerosis resulting from radiation treatment. Radiation-induced damage can include "myelo-radiculo-plexo-neuro-myopathy," causing muscle weakness and dysfunction and contributing to neuromuscular injury. Similarly, radiation damage to neuromuscular structures contributes to radiation-induced trismus and cervical dystonia in head and neck cancer survivors. This narrative review discusses the pathophysiology, anatomy, evaluation, and treatment of neuromuscular, musculoskeletal, and functional disorders that can result as late effects of radiation treatment. Rehabilitation medicine physicians with extensive training in neuromuscular and musculoskeletal medicine as well as in the principles of functional restoration are uniquely positioned to help lead efforts to improve the quality of life for cancer survivors with radiation fibrosis syndrome. Copyright © 2011 American Academy of Physical Medicine and Rehabilitation. Published by Elsevier Inc. All rights reserved.

  4. Fusobacterium necrophorum Pharyngitis Complicated by Lemierre’s Syndrome

    Directory of Open Access Journals (Sweden)

    Antonio Faraone

    2016-01-01

    Full Text Available We report the case of an 18-year-old woman who was referred to our outpatient clinic because of a 2-week history of sore throat, high fever, and neck tenderness unresponsive to a 7-day amoxicillin/clavulanic acid course. Infectious mononucleosis was initially suspected, but an extremely high value of procalcitonin and clinical deterioration suggested a bacterial sepsis, prompting the patient admission to our internal medicine ward. Blood cultures were positive for Fusobacterium necrophorum. CT scan detected a parapharyngeal abscess, a right internal jugular vein thrombosis, and multiple bilateral lung abscesses, suggesting the diagnosis of Lemierre’s syndrome. The patient was treated with a 2-week course of metronidazole and meropenem with a gradual clinical recovery. She was thereafter discharged home with metronidazole and amoxicillin/clavulanic acid for 14 days and a 3-month course of enoxaparin, experiencing an uneventful recovery. The present case highlights the importance of taking into consideration the Lemierre’s syndrome whenever a pharyngotonsillitis has a severe and unusual course.

  5. Premature Birth with Complicated Perinatal Course Delaying Diagnosis of Prader-Willi Syndrome

    Science.gov (United States)

    Ciana, G.; Fertz, M. C.; Pecile, V.; Demarini, S.

    2011-01-01

    Prader-Willi syndrome in the newborn is essentially characterized by marked hypotonia, feeding difficulties, hypogonadism, and possible characteristic facial features. However, diagnosis at this age may be particularly difficult, and dysmorphic features may be subtle or absent. Prematurity can furthermore delay clinical features recognition and typical complications due to preterm birth may contribute to divert the diagnosis. We describe a preterm baby with a complicated perinatal course later diagnosed as PWS. PMID:22606524

  6. Premature Birth with Complicated Perinatal Course Delaying Diagnosis of Prader-Willi Syndrome

    Directory of Open Access Journals (Sweden)

    G. Ciana

    2011-01-01

    Full Text Available Prader-Willi syndrome in the newborn is essentially characterized by marked hypotonia, feeding difficulties, hypogonadism, and possible characteristic facial features. However, diagnosis at this age may be particularly difficult, and dysmorphic features may be subtle or absent. Prematurity can furthermore delay clinical features recognition and typical complications due to preterm birth may contribute to divert the diagnosis. We describe a preterm baby with a complicated perinatal course later diagnosed as PWS.

  7. Complications in mechanically ventilated patients of Guillain–Barre syndrome and their prognostic value

    OpenAIRE

    Archana Becket Netto; Taly, Arun B.; Kulkarni, Girish B.; G S Uma Maheshwara Rao; Shivaji Rao

    2017-01-01

    Introduction: The spectrum of various complications in critically ill Guillain?Barre syndrome (GBS) and its effect on the prognosis is lacking in literature. This study aimed at enumerating the complications in such a cohort and their significance in the prognosis and mortality. Materials and Methods: Retrospective case record analysis of all consecutive mechanically ventilated patients of GBS in neurology Intensive Care Unit (ICU) of a tertiary care institute for 10 years was done. Demograph...

  8. Turner syndrome in Albania and the efficacy of its treatment with growth hormone.

    Science.gov (United States)

    Hoxha, Petrit; Babameto-Laku, Anila; Vyshka, Gentian; Gjoka, Klodiana; Minxuri, Dorina; Myrtaj, Elira; Çakërri, Luljeta

    2015-11-01

    The aim of this study was the evaluation of Turner syndrome inside the Albanian population, its clinical, cytological and genetic characteristics, the accompanying pathologies, and the efficacy of the treatment with the growth hormone. We performed a retrospective analysis of 59 patients suffering from this syndrome (aging from 5 to 23 years old). The diagnosis of female patients suffering from Turner syndrome is delayed, with a mean age at the moment of diagnosis of 13.74 years (5-23 years). The main reason for seeking medical advice was the growth retardation or a delayed puberty. Available data for 52 patients showed that the most frequent accompanying pathologies were the following: thyroid autoimmune disorders (59%), cardiovascular anomalies (43%), renal pathologies (41%), hearing impairment (4.3%) and hypertension (3.3%). Follow-up for the growth rate was possible for 52 patients out of the total of 59 patients. Twenty-five of the female patients suffering Turner syndrome and forming part of our study sample were treated with growth hormone for a period averaging 3 years and 4 months. A variety of reasons was identified as responsible for the missed treatment in 27 patients. We saw an enhanced growth (in terms of body height) within the treated subgroup, when compared with the untreated subgroup (27 patients), especially during the first 3 years of the follow-up. No side effects of this treatment were reported. Both groups of patients initiated as well a sexual hormone therapy (estrogens and progesterone) for inducing puberty at the age of 12 years. Further work is needed for an early diagnosis of this syndrome, the prompt treatment with growth hormone and the monitoring of accompanying disorders. This will ensure a better quality of life and an improvement of the longevity of patients suffering from the Turner syndrome.

  9. Empty sella syndrome associated with hormone deficiency in adults; Silla turca vacia asociada a disfuncion hormonal en adultos

    Energy Technology Data Exchange (ETDEWEB)

    Oleaga, L.; Paja, M.; Goni, F.; Grande, J.; Grande, D. [Hospital de Basurto. Bilbao (Spain); Merino, M. [Hospital General Yague (Spain); Delgado, A. [Hospital Marques de Valdecilla. Santander (Spain)

    1999-07-01

    The objective of this study was to correlate the magnetic resonance (MR) images in patients with hormone deficiencies with the clinical data and the hormonal status. We studied 11 cases ef empty sella with different peripheral pituitary deficiencies. Hormone levels were determined according to standard laboratory methods. All the patients underwent MR imaging. The studies were carried out with a 1 Tesla superconducting magnet, using the cranial cavity for transmission and reception. Segittal and coronal T1-weighted spin-echo sequences (TR/TE: 600/15 ms), axial T2-weighted spin-echo sequences (TR/TE: 3,500/19/93 ms) and gadolinium-enhanced (=.2 cc/kg body weight) sagital and coronal T1-weighted spin-echo sequences (TR/TE: 600/15 ms) were employed. Six of the patients presented partial or total hypopituitarism associated with the syndrome of inappropriate antidiuretic hormone secretion (SIADH); there was one case of panhypopituitarism without SIADH and four cases of primary hypothyroidism, there of which were associated with pituitary deficiency, MR imaging revealed five cases of partially empty sella with residual pituitary gland on the sella floor and six cases in which the sella was completely empty. This study also identified six cases of normally situated neurohypophysis, another four in which the neurohypophysis could not be identified and one case of ectopic neurohypophysis. MR imaging is the technique of choice in the study of abnormal hypothalamic-pituitary activity. Empty and partially empty sella should be included among the frequent causes of hypopituitarism, although there is no clear relationship between the degree of adenohypophyseal insufficiency and the degree of atrophy of this system as viewed in MR images. In some cases, this entity may be the radiological sign of a phase in the development of an autoimmune inflammatory process involving the pituitary gland. (Author) 16 refs.

  10. Posterior reversible encephalopathy syndrome: An atypical postpartum complication

    Directory of Open Access Journals (Sweden)

    Debashish Paul

    2016-01-01

    Full Text Available Posterior reversible encephalopathy syndrome (PRES is presented by headache, altered mental status, blurring of vision, vomiting and seizure in conjunction with radiological finding of posterior cerebral white matter edema. Data suggest that most cases occur in young middle-aged with marked female preponderance, hypertension being the most common cause. In this case, it was diagnosed in a normotensive patient in the postnatal period that underwent cesarean section. The initial symptoms had misled toward a diagnosis of postdural puncture headache. Symptomatic treatment was started immediately in the ICU. This is an interesting case as the patient was a normotensive one without any other contributory factors and there was unanticipated delay in diagnosing the case until the time we could get a magnetic resonance imaging report.

  11. Growth hormone deficiency in a Nigerian child with Turner's syndrome

    African Journals Online (AJOL)

    IRORO YARHERE

    The Nigerian Health Journal, Volume 16 No 2, April to June 2016 ... endorsed GH treatment for short girls with Turner syndrome. ... FASTING BLOOD SUGAR. ... levels have been normal since her follow-up and she has gained 6 cm in the past 1 year .... Cognitive function in some children with TS is grossly reduced when.

  12. Growth hormone receptor deficiency (Laron syndrome) in black ...

    African Journals Online (AJOL)

    Non-Caucasians with growth honnone receptor (GHR) deficiency/Lamn syndrome among the approximately 180 recognised cases are rare, and include a Japanese and 3. African Americans. Black African siblings, a brother and a sister seen initially at 11 years 9 months and 5 years 6 months of age respectively were -7,4 ...

  13. Growth hormone receptor deficiency (Laron syndrome) in black ...

    African Journals Online (AJOL)

    Non-Caucasians with growth honnone receptor (GHR) deficiency/Lamn syndrome among the approximately 180 recognised cases are rare, and include a Japanese and 3 African Americans. Black African siblings, a brother and a sister seen initially at 11 years 9 months and 5 years 6 months of age respectively were -7,4 ...

  14. 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.

  15. Myositis, rhabdomyolysis and compartment syndrome complicating influenza A in a child

    OpenAIRE

    Skellett, Sophie Clare; Dhesi, Rosepal

    2009-01-01

    A previously fit and healthy 8-year-old boy died following severe complications of influenza A. He developed lethargy and vomiting before presentation. On presentation to medical attention, on day 4 of his illness, he was in extremis and had extensive myositis, rhabdomyolysis, renal failure and compartment syndrome, which were resistant to supportive medical management.

  16. Myositis, rhabdomyolysis and compartment syndrome complicating influenza A in a child.

    Science.gov (United States)

    Skellett, Sophie Clare; Dhesi, Rosepal

    2009-01-01

    A previously fit and healthy 8-year-old boy died following severe complications of influenza A. He developed lethargy and vomiting before presentation. On presentation to medical attention, on day 4 of his illness, he was in extremis and had extensive myositis, rhabdomyolysis, renal failure and compartment syndrome, which were resistant to supportive medical management.

  17. Wish to conceive and concerns to develop cardiovascular complications during pregnancy in patients with Turner syndrome

    NARCIS (Netherlands)

    Hagen, I.M. van; Duijnhouwer, A.L.; Kate-Booij, M.J. ten; Dykgraaf, R.H.; Duvekot, J.J.; Utens, E.M.; Roos-Hesselink, J.W.

    2017-01-01

    INTRODUCTION: Turner syndrome (TS) is associated with subfertility and infertility. Nevertheless, an increasing number of women become pregnant through oocyte donation. The wish to conceive may be negatively influenced by the fear of cardiovascular complications. The aim was to investigate the wish

  18. Wish to conceive and concerns to develop cardiovascular complications during pregnancy in patients with Turner syndrome

    NARCIS (Netherlands)

    I.M. van Hagen (Iris); A.L. Duijnhouwer (Anthonie L.); M.J. ten Kate-Booij (Marianne); R.H.M. Dykgraaf (Ramon); J.J. Duvekot (Hans); E.M.W.J. Utens (Elisabeth); J.W. Roos-Hesselink (Jolien)

    2017-01-01

    textabstractIntroduction: Turner syndrome (TS) is associated with subfertility and infertility. Nevertheless, an increasing number of women become pregnant through oocyte donation. The wish to conceive may be negatively influenced by the fear of cardiovascular complications. The aim was to

  19. Terson's syndrome after endoscopic colloid cyst removal: case report and a review of reported complications.

    NARCIS (Netherlands)

    Boogaarts, H.; Grotenhuis, A.

    2008-01-01

    The authors report on a patient with Terson's syndrome after endoscopic colloid cyst resection of the third ventricle. This never reported complication in neuroendoscopy is explained by an increased intracranial pressure due to rinsing. Increased rinsing was needed to prevent the ventricles from

  20. Gut hormones in the treatment of short-bowel syndrome and intestinal failure

    DEFF Research Database (Denmark)

    Jeppesen, Palle B

    2015-01-01

    PURPOSE OF REVIEW: The approval of teduglutide, a recombinant analog of human glucagon-like peptide (GLP) 2, by the US Food and Drug Administration (Gattex) and the European Medicines Agency (Revestive) has illustrated the potential of selected gut hormones as treatments in patients with short......-bowel syndrome and intestinal failure. Gut hormones may improve the structural and functional intestinal adaptation following intestinal resection by decreasing a rapid gastric emptying and hypersecretion, by increasing the intestinal blood flow, and by promoting intestinal growth. This review summarizes...

  1. Amelioration of Hypophosphatemic Rickets and Osteoporosis With Pamidronate and Growth Hormone in Lowe Syndrome

    Directory of Open Access Journals (Sweden)

    Jia-Woei Hou

    2009-09-01

    Full Text Available The oculocerebrorenal syndrome of Lowe, an X-linked multisystem disorder, was diagnosed in a male patient who presented with typical abnormalities of the eyes, kidneys and nervous system. Besides congenital cataracts, renal tubular dysfunction and psychomotor retardation, the patient had also suffered from profound failure to thrive, growth hormone deficiency, severe osteoporosis with hypophosphatemic rickets, and progressive renal dysfunction since early childhood, which were attributed to the metabolic derangements following Fanconi syndrome. Direct sequencing of the OCRL1 gene (responsible for the oculocerebrorenal syndrome of Lowe revealed a de novo c.2282_2283insT in exon 20, which resulted in premature termination of translation (D762X. After monthly intravenous administration of pamidronate since the age of 17.8 years, his urine creatinine clearance and tubular resorption of phosphate increased slightly and bone mineral density was much improved (Z score increased from −7.3 to −3.3 without deterioration of renal function. Simultaneous growth hormone therapy enhanced the positive response. The beneficial osseous and renal effects of the bisphosphonate, along with growth hormone treatment in Lowe syndrome with hypophosphatemia, may be related to reduced renal calcium and phosphate excretion.

  2. Imbalance between thyroid hormones and the dopaminergic system might be central to the pathophysiology of restless legs syndrome: a hypothesis

    Directory of Open Access Journals (Sweden)

    Jose Carlos Pereira Jr.

    2010-01-01

    Full Text Available Data collected from medical literature indicate that dopaminergic agonists alleviate Restless Legs Syndrome symptoms while dopaminergic agonists antagonists aggravate them. Dopaminergic agonists is a physiological regulator of thyroid-stimulating hormone. Dopaminergic agonists infusion diminishes the levels of thyroid hormones, which have the ability to provoke restlessness, hyperkinetic states, tremors, and insomnia. Conditions associated with higher levels of thyroid hormones, such as pregnancy or hyperthyroidism, have a higher prevalence of Restless Legs Syndrome symptoms. Low iron levels can cause secondary Restless Legs Syndrome or aggravate symptoms of primary disease as well as diminish enzymatic activities that are involved in dopaminergic agonists production and the degradation of thyroid hormones. Moreover, as a result of low iron levels, dopaminergic agonists diminishes and thyroid hormones increase. Iron therapy improves Restless Legs Syndrome symptoms in iron deprived patients. Medical hypothesis. To discuss the theory that thyroid hormones, when not counterbalanced by dopaminergic agonists, may precipitate the signs and symptoms underpinning Restless Legs Syndrome. The main cause of Restless Legs Syndrome might be an imbalance between the dopaminergic agonists system and thyroid hormones.

  3. Fatal Lemierre’s syndrome as a complication of chronic otitis media with cholesteatoma

    Directory of Open Access Journals (Sweden)

    Tashneem Harris

    2016-12-01

    Full Text Available Background. Lemierre’s syndrome is septic thrombophlebitis of the internal jugular vein, initiated by an infection of the head and neck region. This septic thrombophlebitis gives rise to septic microemboli that can disseminate throughout the body to form septic infarctions and abscesses, with the most frequent site being pulmonary. Methods. We discuss the case of a 14-year-old male with Lemierre’s syndrome as a complication of chronic middle ear infection. Results. The patient developed septic shock and microemboli, and subsequently died. Conclusion. This case report illustrates that untreated chronic middle ear infection can lead to potentially fatal complications such as Lemierre’s syndrome, and emphasises the importance of timeous treatment of chronic middle ear pathology.

  4. 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.

  5. Spinal fusion for scoliosis in Rett syndrome with an emphasis on early postoperative complications.

    Science.gov (United States)

    Gabos, Peter G; Inan, Muharrem; Thacker, Mihir; Borkhu, Buttugs

    2012-01-15

    Retrospective case-control study. To examine the postoperative complications of posterior spinal fusion in a population of patients with Rett syndrome (RS). Scoliosis is a common feature of RS, a progressive neurologic disorder affecting almost exclusively females. Despite this, there is little published information regarding the surgical treatment of scoliosis in this disorder. Sixteen consecutive female patients with RS treated by posterior spinal fusion and unit rod instrumentation for progressive scoliosis between 1995 and 2003 were evaluated. Only patients with a minimum of 2-year follow-up were included. Preoperative medical conditions and postoperative complications were recorded. As a control group, we randomly selected 32 spastic quadriplegic patients who underwent the identical procedure during the same time period, selected from our database and matched according to age, level of neurologic impairment, and medical complexity. There was a high rate of early medical complications in the RS patients, with 28 major and 37 minor complications. Only 1 patient did not have a major medical complication, and every patient had at least 1 minor gastrointestinal and/or respiratory complication. Major respiratory complications occurred in 10 patients (63%) and comprised 61% of all major complications. Major gastrointestinal complications occurred in 6 patients (37%) and comprised 21% of all major complications. Other major complications included disseminated intravascular coagulopathy (1 patient), subacute bacterial endocarditis (1 patient), sacral decubiti requiring surgical debridement (2 patients), and extensive bilateral heterotopic ossification of the hips (1 patient). There were no cases of instrumentation failure, pseudarthrosis, deep infection, or need for rod revision. Postoperative complication scores were similar to those in patients with spastic quadriplegic pattern cerebral palsy. Spinal fusion for scoliosis in RS can give a satisfactory technical result

  6. Do the interactions between glucocorticoids and sex hormones regulate the development of the Metabolic Syndrome?

    Directory of Open Access Journals (Sweden)

    Marià eAlemany

    2012-02-01

    Full Text Available The metabolic syndrome is basically a maturity-onset disease. Typically, its manifestations begin to flourish years after the initial dietary or environmental aggression began. Since most hormonal, metabolic or defense responses are practically immediate, the procrastinated response don't seem justified. Only in childhood, the damages of the metabolic syndrome appear with minimal delay. Sex affects the incidence of the metabolic syndrome, but this is more an effect of timing than absolute gender differences, females holding better than males up to menopause, when the differences between sexes tend to disappear. The metabolic syndrome is related to an immune response, countered by a permanent increase in glucocorticoids, which keep the immune system at bay but also induce insulin resistance, alter the lipid metabolism, favor fat deposition, mobilize protein and decrease androgen synthesis. Androgens limit the operation of glucocorticoids, which is also partly blocked by estrogens, since they decrease inflammation (which enhances glucocorticoid release. These facts suggest that the appearance of the metabolic syndrome symptoms depends on the strength (i.e. levels of androgens and estrogens. The predominance of glucocorticoids and the full manifestation of the syndrome in men are favored by decreased androgen activity. Low androgens can be found in infancy, maturity, advanced age, or because of their inhibition by glucocorticoids (inflammation, stress, medical treatment. Estrogens decrease inflammation and reduce the glucocorticoid response. Low estrogen (infancy, menopause again allow the predominance of glucocorticoids and the manifestation of the metabolic syndrome. It is postulated that the equilibrium between sex hormones and glucocorticoids may be a critical element in the timing of the manifestation of metabolic syndrome-related pathologies.

  7. Dynamics of hormonal status in women of different age groups in hemorrhagic fever with renal syndrome

    Directory of Open Access Journals (Sweden)

    Murzabaeva R.Т.

    2012-06-01

    Full Text Available Objective: To study the hormonal parameters in women of different age groups in hemorrhagic fever with renal syndrome. Materials and methods: We have studied the content of cortisol, thyrotropic hormone (TTH, triiodothyro-nine (T3, free thyroxin (FT4, luteinizing hormone (LH, follicle-stimulating hormone (FSH, testosterone, estradiol, progesterone, prolactin in blood serum of 62 women with moderate (33 and severe (29 HFRS forms age (17-62. They were divided into 2 groups: the first group (33 patients women with the normal menstrual cycle, the second group (29 women consisted of patients in climacteric period. Results: TTH secretion increase, T3 and FT4 — decrease with their normalization to the recovery period were registered in the thyroid system of the compared groups. Blood cortisol level was high during the illness. Gonadotropic hypophysis function study demonstrated that LH and blood prolactin concentrations were increased since oliguria period; FSH was authentic reduced. The indices of these hormones were restored to the normal level by the reconvalescence period. LH and FSH contents were authentic higher in women of the second group in comparison with the first group. The hyperprolactinemia was observed in both women groups during the whole period of disease. The increased progesterone and testosterone concentrations have been manifested in blood serum. The estradiol concentration had different direction tendencies. Conclusion: Thus, the complex study of hypophysic- thyreoid and gonadotropic hormone state of adrenal system and the sexual hormone levels in women of different age groups in HFRS revealed the hormone status indces changes due to the period and severity of the disease, connected with the virus action, intoxication, the general inflammation reactions and their age.

  8. [Mauriac syndrome--a rare complication of type 1 diabetes mellitus].

    Science.gov (United States)

    Schmetz, Anne V N; Dekker-Maas, Mariëlle H; den Breejen, Marianne P; Sas, Theo C J

    2012-01-01

    The treatment of children with type 1 diabetes mellitus has improved dramatically over the last few decades. The maintenance of acceptable metabolic control, nevertheless, remains challenging because the success of treatment is so dependent on patient compliance. Children with type 1 diabetes and poor metabolic control are at risk of developing Mauriac syndrome, a condition characterised by hepatomegaly, growth retardation and cushingoid features. A similar complication may occur in type-1 or type-2 adult diabetics; namely, glycogenic hepatopathy. We describe two children, a 12-year-old girl and a 16-year-old boy, who presented with classic symptoms of Mauriac syndrome. After metabolic control was achieved, reduction of hepatomegaly and the disappearance of cushingoid features were observed, proving the reversibility of the syndrome. Awareness that this syndrome still exists despite improved insulin therapy is crucial for earlier recognition and treatment.

  9. Response of motor complications in Cockayne syndrome to carbidopa-levodopa.

    Science.gov (United States)

    Neilan, Edward G; Delgado, Mauricio R; Donovan, Melissa A; Kim, Sara Y; Jou, Rita L; Wu, Bai-Lin; Kang, Peter B

    2008-08-01

    Gait difficulties, tremors, and coordination difficulties are common features of Cockayne syndrome that are consequences of leukodystrophy, cerebellar atrophy, and demyelinating neuropathy, but no pharmacotherapy for these disabling symptoms is available. To determine whether carbidopa-levodopa relieves tremors and other motor complications of Cockayne syndrome. Mutation analysis and case report study. Hospital clinic and genetics research laboratory. Patients We studied 3 patients with Cockayne syndrome, a rare autosomal recessive neurodegenerative disorder for which no known treatments are available. Intervention Carbidopa-levodopa therapy. Status of tremors, ability to perform daily tasks, serial physical examinations, and results of handwriting samples. All 3 patients had a clear reduction in tremors and improvements in handwriting and manipulation of utensils and cups. Patients with Cockayne syndrome should be evaluated carefully for movement disorders. A clinical trial should be considered to evaluate this therapy further.

  10. A Case Report: Jacobsen Syndrome Complicated by Paris-Trousseau Syndrome and Shone's Complex.

    Science.gov (United States)

    Malia, Laurie A; Wolkoff, Leslie I; Mnayer, Laila; Tucker, Joseph W; Parikh, Nehal S

    2015-10-01

    A preterm infant presenting with a congenital cardiac malformation and thrombocytopenia was found to have a karyotype showing a terminal deletion of the long arm of chromosome 11 of the segment 11q24.1-11qter consistent with Jacobsen syndrome. The infant was later diagnosed with Paris-Trousseau syndrome, commonly associated with Jacobsen syndrome. Because children with cardiac malformations often require high-risk surgical procedures in the early neonatal period, those with platelet dysfunction require prompt identification at birth.

  11. Gingival fibromatosis and growth hormone deficiency syndrome--report of a rare case and review of literature.

    OpenAIRE

    Radhakrishnan S; Rajan P

    2003-01-01

    Oikarinen et al in 1989 reported a syndrome associated with generalized gingival fibromatosis and growth hormone deficiency. This is a case report of a 15-year-old female patient who presented to the Government Dental College, Chennai with generalized gingival fibromatosis and growth hormone deficiency. Interestingly, the histopathology of the excised gingival overgrowth showed dense collagenous connective tissue in which were strewn calcified structures that resembled cementum. This syndrome...

  12. Final height in girls with Turner's syndrome treated with once or twice daily growth hormone injections

    OpenAIRE

    Sas, T.; Keizer-Schrama, S; Stijnen, T.; van Teunenbroek, A; Hokken-Koelega, A.; Waelkens, J.; Massa, G.; Vulsma, T.; Gerver, W; Reeser, H; de Waal, H E D.-v.; Jansen, M.; Drop, S.; The, D.

    1999-01-01

    OBJECTIVES—To study final height in girls with Turner's syndrome treated with once or twice daily injections of growth hormone (GH) in combination with low dose ethinyl oestradiol.
DESIGN—Until final height was reached, the effect of fractionated subcutaneous injections given twice daily was compared with once daily injections of a total GH dose of 6 IU/m2/day. Twice daily injections were given as one third in the morning and two thirds at bedtime. All girls concurrently ...

  13. The effect of the type of membrane on intradialytic complications and mortality in crush syndrome.

    Science.gov (United States)

    Ozturk, Savas; Kazancioglu, Rumeyza; Sahin, Gulizar Manga; Turkmen, Aydin; Gursu, Meltem; Sever, Mehmet Sukru

    2009-01-01

    There are not enough data about the type of the membrane that should be used in acute intermittent hemodialysis (IHD) in patients with crush syndrome where intradialytic complication rate is high. The effects of dialyzers on outcome have been investigated in this study. Patients who required IHD due to crush syndrome after a big earthquake that struck Marmara in 1999 have been studied. Hemodynamic and biochemical analyses at the time of admission were examined. The patients were divided into three groups according to the type of dialyzers (viz., hemophan, polysulfone, and combined). Forty-five patients were included in the study (mean age: 33.9 +/- 13.3 years, mean HD session per patient: 8.8 +/- 6.1). In all, 408 dialyzers were used during IHD therapy (21% hemophan). The types of dialyzers used were hemophan (8 patients), polysulfone (18 patients), and the combination of the two (19 patients). The demographic and biochemical parameters related to crush syndrome were not different statistically. All sessions were anticoagulant-free. Hypotension and coagulation of sets were the main intradialytic complications. Five (11%) patients died, but there was no correlation between mortality rates and the type of the dialyzer used. Serum albumin, blood pressure, and thrombocyte counts were found to be related to mortality. No effect of the type of dialysis membrane on outcome was detected in patients with crush syndrome. Other potential factors, which may responsible for the complications and mortality, should be investigated.

  14. Review of Garcinia mangostana and its Xanthones in Metabolic Syndrome and Related Complications.

    Science.gov (United States)

    Tousian Shandiz, Hourieh; Razavi, Bibi Marjan; Hosseinzadeh, Hossein

    2017-08-01

    Metabolic syndrome is coexistence of abdominal obesity, hyperglycemia, hyperlipidemia and hypertension that causes cardiovascular diseases, diabetes and their complications, low quality and short lifespan. Garcinia mangostana and its xanthones such as α-mangostin have been shown desirable effects such as anti-obesity, anti-hyperglycemic, anti-dyslipidemia, anti-diabetic and antiinflammatory effects in experimental studies. Various databases such as PubMed, Scopus and Web of Science with keywords of 'Garcinia mangostana', 'mangosteen', 'α-mangostin', 'metabolic syndrome', 'hypoglycemic', 'antihyperglicemic', 'antidiabetic', 'hypotensive', 'antihypertensive', 'atherosclerosis', 'arteriosclerosis' and 'hyperlipidemia' have been investigated in this search without publication time limitation. This study reviewed all pharmacological effects and molecular pathways of G. mangostana and its xanthones in the management of metabolic syndrome and its complications in in-vitro and in-vivo studies. Based on these studies, mangosteen and its xanthones have good potential to design human studies for controlling and modification of metabolic syndrome and its related disorders such as obesity, disrupted lipid profile, diabetes and its complications. Copyright © 2017 John Wiley & Sons, Ltd. Copyright © 2017 John Wiley & Sons, Ltd.

  15. HELLP syndrome--a syndrome of hemolysis, elevated liver enzymes and low platelet count--complicating preeclampsia-eclampsia.

    Science.gov (United States)

    Reubinoff, B E; Schenker, J G

    1991-10-01

    The course of preeclamptic/eclamptic patients may be complicated by HELLP syndrome, a syndrome of intravascular hemolysis (H), elevated liver enzymes (EL) and low platelet count (LP). These patients typically present at early third trimester with epigastric or right upper quadrant pain, nausea and vomiting. They may present without the clinical signs of preeclampsia (hypertension and proteinuria or edema), thus an initial wrong nonobstetric diagnosis is not uncommon. The most frequent maternal complication is intravascular coagulopathy (30%). Placental abruption and acute renal failure are also common. Ten cases of maternal deaths were reported among 295 cases reviewed in the English language literature, while the perinatal mortality rate was 226/1000. The grave prognosis for mother and fetus warrants physician awareness in order to accomplish early diagnosis and proper management. This paper is a review of the literature in English.

  16. Clinically defined chemotherapy-associated bowel syndrome predicts severe complications and death in cancer patients.

    Science.gov (United States)

    Vehreschild, Maria J G T; Meissner, Arne M K; Cornely, Oliver Andreas; Maschmeyer, Georg; Neumann, Silke; von Lilienfeld-Toal, Marie; Karthaus, Meinholf; Wattad, Mohammed; Staib, Peter; Hellmich, Martin; Christ, Hildegard; Vehreschild, Jörg Janne

    2011-12-01

    Neutropenic patients are at risk of abdominal complications and yet the incidence and impact of these complications on patients' morbidity and mortality have not been sufficiently evaluated. We aimed to assess a clinical rule for early detection of abdominal complications leading to death or transfer to intensive care in patients with chemotherapy-associated neutropenia. This observational multicenter study was carried out in seven German hematology-oncology departments. For inclusion, neutropenia of at least 5 consecutive days was required. Risk factors for "transfer to intensive care" and "death" were assessed by backward-stepwise binary logistic regression analyses. Chemotherapy-associated bowel syndrome was defined as a combination of fever (T ≥37.8 °C) and abdominal pain and/or lack of bowel movement for 72 hours or more. Five hundred and twenty-one neutropenic episodes were documented in 359 patients. The incidence of chemotherapy-associated bowel syndrome was 126/359 (35%) in first episodes of neutropenia. Transfer to intensive care occurred in 41/359 (11%) and death occurred in 17/359 (5%) first episodes. Chemotherapy-associated bowel syndrome and duration of neutropenia were identified as risk factors for transfer to intensive care (PChemotherapy-associated bowel syndrome and mitoxantrone administration were identified as risk factors for death (P=0.005; OR 4.611; 95% CI 1.573-13.515 and P=0.026; OR 3.628; 95% CI 1.169-11.256). The occurrence of chemotherapy-associated bowel syndrome has a significant impact on patients' outcome. In future interventional clinical trials, this definition might be used as a selection criterion for early treatment of patients at risk of severe complications.

  17. Clinical significance of fluctuations in thyroid hormones after surgery for Cushing's syndrome.

    Science.gov (United States)

    Tamada, Daisuke; Kitamura, Tetsuhiro; Onodera, Toshiharu; Hamasaki, Toshimitsu; Otsuki, Michio; Shimomura, Iichiro

    2015-01-01

    Patients with Cushing's syndrome (CS) frequently develop hyperthyroidism after surgery due to SITSH (syndrome of inappropriate secretion of TSH) and this SITSH contributed to the symptoms of steroid withdrawal syndrome (SWS). However, the duration of fluctuations in thyroid hormones after surgery for CS remains unknown. The aim of this prospective study was to investigate the clinical course of fluctuation in thyroid hormone level in CS patients after surgery. Thyroid hormone levels [free T3 (FT3), free T4 (FT4) and TSH] and serum cortisol levels were measured before and 1, 3, 6 and 12 months after surgery in 8 patients with active CS (3 pituitary CS and 5 adrenal CS). FT3 levels were above the normal range in 75% of patients up to 6 months after surgery, but returned to the normal range by 12 months. However, TSH levels were not suppressed below the normal range throughout the first 12 months after surgery. Serious symptoms of SWS appeared during the 6-month period after surgery, but disappeared with normalization of thyroid function at 12 months, which was not related to the recovery of function hypothalamus-pituitary-adrenal axis after CS surgery. Therefore, T3 toxicosis could result in deterioration of SWS after surgery for CS. These results indicate that physicians need to take T3 toxicosis into consideration in the pathological evaluation of SWS within 12 months after surgery for CS.

  18. Growth hormone treatment improves vitality and behavioural issues in children with Prader-Willi syndrome.

    Science.gov (United States)

    Böhm, Birgitta; Ritzén, E Martin; Lindgren, Ann Christin

    2015-01-01

    Prader-Willi syndrome is a neurogenetic disorder, with characteristics such as obesity, short stature, muscular weakness, intellectual deficiencies and deviant social behaviour. This study evaluated whether growth hormone treatment of children with Prader-Willi syndrome resulted in possible and lasting effects on their cognition and behaviour. We randomised six girls and 13 boys to either a treatment group or a control group. The treatment group received growth hormone (Genotropin(®) 0.033 mg/kg/day) for 2 years, while the control group did not receive treatment in the first year and then received a double dose in the second year. Treatment was then stopped in both groups for 6 months. Both groups showed the same intellectual disabilities at the start of the study, and no difference was found after the first and second years. The parents reported that the children showed increased vitality during treatment. When treatment was stopped, the children showed a marked exacerbation of behavioural problems, a significant increase in body fat and a decrease in insulin-like growth factor 1 levels. We believe this is the first study to show that abrupt-ceasing growth hormone treatment led to a successive deterioration in behavioural problems in children with Prader-Willi syndrome. ©2014 Foundation Acta Paediatrica. Published by John Wiley & Sons Ltd.

  19. Sleep architecture in Sheehan's syndrome before and 6 months after growth hormone replacement therapy.

    Science.gov (United States)

    Ismailogullari, Sevda; Tanriverdi, Fatih; Kelestimur, Fahrettin; Aksu, Murat

    2009-02-01

    To characterize the sleep parameters in patients with growth hormone (GH) deficiency in Sheehan's syndrome adults and to assess the effects of 6-month GH replacement therapy (GHRT). Twenty-two women with Sheehan's syndrome, (mean age; 49.1+/-2.2 years), and 12 women with similar age (mean age; 51.3+/-3.8 years) and body mass index as control subjects were included in the study. Under baseline conditions, women received adequate hormone replacement therapy for all hormonal deficiencies other than GH. Twelve patients received recombinant GH (Genotropin; Pfizer Stockholm, Sweden) (treatment group) and eight patients received placebo (placebo group) for 6 months. Two patients had only baseline evaluation and were not followed up prospectively. Two polysomnography (PSG) recordings were performed on the patients group, one in the baseline period and the other at the sixth month of treatment (either GH or placebo). Control group had only baseline PSG. GH deficient females with Sheehan's syndrome have more NREM (95.9+/-1.5% and 88.6+/-0.9%, respectively; psyndrome patients under baseline conditions.

  20. Intrathecal baclofen withdrawal syndrome- a life-threatening complication of baclofen pump: a case report.

    Science.gov (United States)

    Mohammed, Imran; Hussain, Asif

    2004-08-09

    Intrathecal baclofen pump has been used effectively with increasing frequency in patients with severe spasticity, particularly for those patients who are unresponsive to conservative pharmacotherapy or develop intolerable side effects at therapeutic doses of oral baclofen. Drowsiness, nausea, headache, muscle weakness, light-headedness and return of pretreatment spasticity can be caused by intrathecal pump delivering an incorrect dose of baclofen. Intrathecal baclofen withdrawal syndrome is a very rare, potentially life-threatening complication of baclofen pump caused by an abrupt cessation of intrathecal baclofen. A 24-year-old man with a past medical history of cerebral palsy and spastic quadriparesis developed hyperthermia, disseminated intravascular coagulation, rhabdomyolysis, acute renal failure and multisystem organ failure leading to a full-blown intrathecal baclofen withdrawal syndrome. Intrathecal baclofen pump analysis revealed that it was stopped due to some programming error. He was treated effectively with supportive care, high-dose benzodiazepines and reinstitution of baclofen pump. The episodes of intrathecal baclofen withdrawal syndrome are mostly caused by preventable human errors or pump malfunction. Educating patients and their caregivers about the syndrome, and regular check-up of baclofen pump may decrease the incidence of intrathecal baclofen withdrawal syndrome. Oral baclofen replacement may not be an effective method to treat or prevent intrathecal baclofen withdrawal syndrome. Management includes an early recognition of syndrome, proper intensive care management, high-dose benzodiazepines and prompt analysis of intrathecal pump with reinstitution of baclofen.

  1. Nontraumatic atlantoaxial subluxation (Grisel syndrome): a rare complication of otolaryngological procedures.

    Science.gov (United States)

    Yu, Kathy K; White, David R; Weissler, Mark C; Pillsbury, Harold C

    2003-06-01

    At the conclusion of the report, the readers should be able to recognize the rare complication of adenotonsillectomy of Grisel syndrome, discuss its pathogenesis, and provide early, effective treatment. Analysis of the clinical presentation, plain radiographs, computed tomography scans, and magnetic resonance imaging scans of a child with Grisel syndrome after adenotonsillectomy. Retrospective study of the case record and a review of the literature regarding the pathogenesis, treatment modalities, outcome, and prognosis after early and delayed treatment of Grisel syndrome. Pathophysiology of atlantoaxial subluxation revolves around the periodontoid vascular plexus that drains the posterosuperior pharyngeal region. Infectious and inflammatory emboli cause synovial engorgement, weakening paraspinal ligaments. Radiological studies play an important role in diagnosis. Treatment consists of cervical immobilization, muscle relaxants, analgesics, and antibiotics. Full resolution is expected with early conservative management. Failure to recognize the syndrome can lead to catastrophic results. Recognition of Grisel syndrome in addition to other rare complications of adenotonsillectomy requires a high index of suspicion. Early intervention is the critical factor for a positive outcome. However, delayed diagnosis is common and can result in catastrophic consequences, including neurological deficits, cosmetic deformity and, in rare instances, paralysis and death in the short term.

  2. Hypothalamic-Pituitary-Adrenal Suppression and Iatrogenic Cushing's Syndrome as a Complication of Epidural Steroid Injections

    Directory of Open Access Journals (Sweden)

    Joyce Leary

    2013-01-01

    Full Text Available Epidural steroid injections are well accepted as a treatment for radicular back pain in appropriate candidates. While overall incidence of systemic side effects has not been well established, at least five biochemically proven cases of iatrogenic Cushing's Syndrome have been reported as complications of epidural steroid treatment. We present an additional case of iatrogenic Cushing's Syndrome and adrenal suppression in a middle-aged woman who received three epidural steroid injections over a four-month period. We review this case in the context of previous cases and discuss diagnostic and management issues.

  3. Hypothalamic-Pituitary-Adrenal Suppression and Iatrogenic Cushing's Syndrome as a Complication of Epidural Steroid Injections.

    Science.gov (United States)

    Leary, Joyce; Swislocki, Arthur

    2013-01-01

    Epidural steroid injections are well accepted as a treatment for radicular back pain in appropriate candidates. While overall incidence of systemic side effects has not been well established, at least five biochemically proven cases of iatrogenic Cushing's Syndrome have been reported as complications of epidural steroid treatment. We present an additional case of iatrogenic Cushing's Syndrome and adrenal suppression in a middle-aged woman who received three epidural steroid injections over a four-month period. We review this case in the context of previous cases and discuss diagnostic and management issues.

  4. HELLP Syndrome Complicated with Postpartum Subcapsular Ruptured Liver Hematoma and Purtscher-Like Retinopathy

    Directory of Open Access Journals (Sweden)

    Daniela Cernea

    2012-01-01

    Full Text Available Purtscher's retinopathy is usually associated with trauma, acute pancreatitis, vasculitis, lupus, and bone fractures. It was rarely described postpartum in patients with preeclampsia as well as associated with HELLP syndrome. We present a case of a multiparous patient aged 44 with severe preeclampsia and postpartum HELLP syndrome complicated with Purtscher-like retinopathy and large ruptured subcapsular liver hematoma that required emergency abdominal surgery after premature delivery of a dead fetus. Postsurgical outcome was favorable regarding both liver function and visual acuity.

  5. Compartment syndrome of the thigh complicating surgical treatment of ipsilateral femur and ankle fractures

    Science.gov (United States)

    Moore, M. R.; Garfin, S. R.; Hargens, A. R.

    1987-01-01

    A 26-year-old man presented with ipsilateral femur and ankle fractures. The patient was treated with interlocking nail of his femur fracture, followed by open reduction and internal fixation of his ankle fracture under tourniquet control. Postoperatively, the patient developed compartment syndrome of his thigh with elevated pressures, requiring decompressive fasciotomies. This case illustrates the possible complication of treating a femur fracture with intramedullary nailing and then immediately applying a tourniquet to treat an ipsilateral extremity fracture. Because of the complication with this patient, we feel the procedure should be staged, or a tourniquet should be avoided if possible.

  6. Relationship between reproductive aging, body composition, hormonal status and metabolic syndrome in postmenopausal women.

    Science.gov (United States)

    Ziaei, S; Sayahi, M; Faghihzadeh, S

    2011-12-01

    To determine the relationship between different anthropometric indicators, reproductive aging and hormonal status in postmenopausal women with and without metabolic syndrome. One hundred and forty non-surgical, postmenopausal women were enrolled to this cross-sectional study. Each participant completed a questionnaire and underwent a medical examination, including measurements of body mass index, weight and body composition criteria. Body composition was evaluated with measurements of the waist-to-hip circumference ratio and skinfold thickness (triceps, suprailiac and front thigh). Levels were determined of serum lipids, including cholesterol, high density lipoprotein cholesterol, low density lipoprotein cholesterol and triglycerides, and also fasting blood sugar, sex hormone binding globulin (SHBG), estradiol and testosterone. The metabolic syndrome was defined according to the definition of the National Cholesterol Education Program Adult Treatment Panel III. Logistic regression analysis revealed a significant association between weight (p < 0.001), age (p < 0.01) and SHBG (p = 0.021) with risk for metabolic syndrome. Age, SHBG and weight are critical correlates of metabolic syndrome in postmenopausal women.

  7. Treatment of Turner's syndrome with recombinant human growth hormone (somatrem).

    Science.gov (United States)

    Lippe, B; Rosenfeld, R G; Hintz, R L; Johanson, A J; Frane, J; Sherman, B

    1988-01-01

    This report extends to 3 years the prospective study of the effects of somatrem alone or in combination with oxandrolone on growth in Turner's syndrome. Sixty-seven patients completed the 1-year study period during which all treatment groups had statistically increased height velocities as compared to the control group. Oral glucose tolerance and insulin responses remained unchanged after 1 year of somatrem treatment. The group receiving oxandrolone experienced an increase in integrated glucose response and the group receiving combined therapy an increase in both integrated glucose and insulin responses. During the second and third years the somatrem group remained on the same dose and treatment schedule and grew at mean velocities of 5.4 +/- 1.1 and 4.6 +/- 1.4 cm/year. The dose of oxandrolone was reduced by 50% during the second and third years for the combination group. The somatrem dose remained unchanged. This group had height velocities of 7.4 +/- 1.4 cm and 6.1 +/- 1.5 cm/year. The control group and the group treated with oxandrolone alone were converted to combined therapy at the lowered oxandrolone dose. Their growth rates during the second year were 8.3 +/- 1.2 and 7.1 +/- 1.6 cm/year, respectively. Using bone age determinations and the methods of Bayley and Pinneau, all groups currently show predicted increases in final adult height.

  8. Neuronal and Hormonal Perturbations in Postural Tachycardia Syndrome

    Directory of Open Access Journals (Sweden)

    Philip L Mar

    2014-06-01

    Full Text Available The Postural Tachycardia Syndrome (POTS is the most common disorder seen in autonomic clinics. Cardinal hemodynamic feature of this chronic and debilitating disorder of orthostatic tolerance is an exaggerated orthostatic tachycardia (≥30 bpm increase in HR with standing in the absence of orthostatic hypotension.There are multiple pathophysiological mechanisms that underlie POTS. Some patients with POTS have evidence of elevated sympathoneural tone. This hyperadrenergic state is likely a driver of the excessive orthostatic tachycardia. Another common pathophysiological mechanism in POTS is a hypovolemic state. Many POTS patients with a hypovolemic state have been found to have a perturbed renin-angiotensin-aldosterone profile. These include inappropriately low plasma renin activity and aldosterone levels with resultant inadequate renal sodium retention. Some POTS patients have also been found to have elevated plasma angiotensin II (Ang-II levels, with some studies suggesting problems with decreased angiotensin converting enzyme 2 activity and decreased Ang-II degradation. An understanding of these pathophysiological mechanisms in POTS may lead to more rational treatment approaches that derive from these pathophysiological mechanisms.

  9. Neuromyelitis optica in pregnancy complicated by posterior reversible encephalopathy syndrome, eclampsia and fetal death.

    Science.gov (United States)

    Igel, Catherine; Garretto, Diana; Robbins, Matthew S; Swerdlow, Michael; Judge, Nancy; Dayal, Ashlesha

    2015-03-01

    Neuromyelitis optica (NMO) is a demyelinating syndrome characterized by optic neuritis and acute myelitis with poor recovery and a progressive course. We report a poor outcome complicated by posterior reversible encephalopathy syndrome (PRES) and eclampsia and review available literature and current evidence for anticipation of adverse fetal and maternal effects. After a pregnancy complicated by multiple admissions for painful NMO exacerbations, a primiparous patient with seropositive NMO presented at 31 + 3/7 weeks with eclampsia, HELLP and subsequent fetal death. MRI confirmed PRES. NMO may be associated with eclampsia and leads to adverse maternal and fetal outcomes. Posited mechanisms include antibody-mediated placental damage and a heightened risk of eclampsia-associated PRES. Further characterization of the course of NMO and its relationship with pregnancy outcomes in larger series would be invaluable.

  10. Reproductive hormones and metabolic syndrome in 24 testicular cancer survivors and their biological brothers.

    Science.gov (United States)

    Bandak, M; Jørgensen, N; Juul, A; Lauritsen, J; Kier, M G G; Mortensen, M S; Oturai, P S; Mortensen, J; Hojman, P; Helge, J W; Daugaard, G

    2017-07-01

    Testicular cancer survivors have impaired gonadal function and increased risk of metabolic syndrome when compared to healthy controls. However, because of the fetal etiology of testicular cancer, familial unrelated healthy men might not be an optimal control group. The objective of this study was to clarify if testicular cancer survivors have impaired gonadal function and increased risk of metabolic syndrome when compared to their biological brothers. A cross-sectional study of testicular cancer survivors (ClinicalTrials.gov number, NCT02240966) was conducted between 2014 and 2016. Of 158 testicular cancer survivors included, 24 had a biological brother who accepted to participate in the study. Serum levels of reproductive hormones and prevalence of metabolic syndrome according to International Diabetes Federation Criteria and National Cholesterol Education Program (Adult Treatment Panel III) criteria comprised the main outcome measures of the study. Median age was similar in testicular cancer survivors and their biological brothers [44 years (IQR 39-50) vs. 46 (40-53) years respectively (p = 0.1)]. In testicular cancer survivors, follow-up since treatment was 12 years (7-19). Serum levels of luteinizing hormone and follicle-stimulating hormone were elevated (p ≤ 0.001), while total testosterone, free testosterone, inhibin B and anti-Müllerian hormone were lower (p ≤ 0.001) in testicular cancer survivors than in their biological brothers. The prevalence of metabolic syndrome was similar and apart from HDL-cholesterol, which was lower in testicular cancer survivors (p = 0.01); there were no differences in the individual components of the metabolic syndrome between testicular cancer survivors and their brothers. In conclusion, gonadal function was impaired in testicular cancer survivors, while we did not detect any difference in the prevalence of metabolic syndrome between testicular cancer survivors and their biological brothers. © 2017 American

  11. A case of possible Kounis syndrome as a complication of scombroid syndrome

    Directory of Open Access Journals (Sweden)

    Stefano Rusconi

    2017-11-01

    Full Text Available Kounis syndrome is defined as the concurrence of acute coronary syndromes such as coronary spasm or acute myocardial infarction with conditions associated with activation of inflammatory mediators such histamine, arachidonic acid and various cytokines and chemokines. Recently, a variety of unusual etiologies have been reported, including scombroid syndrome. We present a case of a woman without previous history of cardiac diseases or cardiovascular risk factors, who presented to emergency department after the onset of flushing, asthenia, palpitations, burning sensation in the mouth having just eaten tuna. The electrocardiogram revealed a sinus tachycardia with diffuse ST segment depression. After therapy, in a short time symptoms recovered and a second electrocardiogram no longer showed any ST changes. These electrocardiographic changes observed in our case were probably due to transitory coronary vasospasm as described in type I variant of Kounis syndrome.

  12. Final height in girls with Turner's syndrome treated with once or twice daily growth hormone injections. Dutch Advisory Group on Growth Hormone

    NARCIS (Netherlands)

    Sas, T. C.; de Muinck Keizer-Schrama, S. M.; Stijnen, T.; van Teunenbroek, A.; Hokken-Koelega, A. C.; Waelkens, J. J.; Massa, G. G.; Vulsma, T.; Gerver, W. J.; Reeser, H. M.; Delemarre-van de Waal, H. E.; Jansen, M.; Drop, S. L.

    1999-01-01

    To study final height in girls with Turner's syndrome treated with once or twice daily injections of growth hormone (GH) in combination with low dose ethinyl oestradiol. Until final height was reached, the effect of fractionated subcutaneous injections given twice daily was compared with once daily

  13. [Tumor lysis syndrome in a pregnancy complicated with acute lymphoblastic leukemia].

    Science.gov (United States)

    Álvarez-Goris, M P; Sánchez-Zamora, R; Torres-Aguilar, A A; Briones Garduño, J C

    2016-04-01

    Acute leukemia is rare during pregnancy, affects about 1 in 75,000 pregnancies, of all leukemias diagnosed only 28% are acute lymphoblastic leukemia, this is a risk factor to develop spontaneous tumor lysis syndrome, it's a oncologic complication potentially deadly if the prophylactic treatment its avoided. Cases of acute lymphoblastic leukemia associated with pregnancy has been poorly documented in the literature the association of these two entities to pregnancy is the first report published worldwide, so the information is limited.

  14. Generalized tetanus could be complicated with Guillain-Barré syndrome.

    Science.gov (United States)

    Lee, Jae Hoon; Hwang, Yun Su; Cho, Ji Hyun

    2016-07-01

    A retrospective analysis of patients diagnosed with tetanus was conducted to evaluate the occurrence of Guillain-Barré syndrome (GBS). Two of 13 tetanus cases were complicated with GBS. Their symptoms and signs related to GBS improved markedly after a 5-day infusion of intravenous immunoglobulin. Physicians should keep in mind that GBS can be an important cause of muscle weakness in patients with tetanus. Copyright © 2016 The Author(s). Published by Elsevier Ltd.. All rights reserved.

  15. A Rare Case of Neonatal Complicated Appendicitis in a Child with Patau’s Syndrome

    OpenAIRE

    Valentina Pastore; Fabio Bartoli

    2014-01-01

    Neonatal appendicitis is a rare condition with high mortality rate. Signs and symptoms are often nonspecific, imaging modalities are not always diagnostic, and preoperative diagnosis is difficult with subsequent delay and complications. Its pathophysiology may be different from appendicitis in older children and comorbidities can be found. We report a case of a female neonate with Patau’s syndrome, intestinal malrotation, and Fallot tetralogy in whom perforated appendix, probably occurring du...

  16. Generalized tetanus could be complicated with Guillain–Barré syndrome

    Directory of Open Access Journals (Sweden)

    Jae Hoon Lee

    2016-07-01

    Full Text Available A retrospective analysis of patients diagnosed with tetanus was conducted to evaluate the occurrence of Guillain–Barré syndrome (GBS. Two of 13 tetanus cases were complicated with GBS. Their symptoms and signs related to GBS improved markedly after a 5-day infusion of intravenous immunoglobulin. Physicians should keep in mind that GBS can be an important cause of muscle weakness in patients with tetanus.

  17. The metabolic syndrome and disturbances in hormone levels in long-term survivors of disseminated testicular cancer

    NARCIS (Netherlands)

    Nuver, J; Smit, AJ; Wolffenbuttel, BHR; Sluiter, WJ; Hoekstra, HJ; Sleifer, DT; Gietema, JA

    2005-01-01

    Purpose The metabolic syndrome may be an important risk factor for cardiovascular disease in long-term survivors of testicular cancer (TC). We investigated the associations between hormone levels and the metabolic syndrome in these men. Patients and Methods We included TC patients cured by

  18. Beneficial Effects of Long-Term Growth Hormone Treatment on Adaptive Functioning in Infants With Prader-Willi Syndrome.

    Science.gov (United States)

    Lo, Sin T; Festen, Dederieke A M; Tummers-de Lind van Wijngaarden, Roderick F A; Collin, Philippe J L; Hokken-Koelega, Anita C S

    2015-07-01

    The aim of this study was to investigate the effect of growth hormone treatment on adaptive functioning in children with Prader-Willi syndrome. Vineland Adaptive Behavior Scale (VABS) was assessed during a randomized controlled trial (RCT) and after 7 years of growth hormone treatment. In the RCT, 75 children (42 infants and 33 prepubertal children) with Prader-Willi syndrome were included. Subsequently, 53 children were treated with long-term growth hormone. Our study demonstrates a marked delay in adaptive functioning in infants and children with Prader-Willi syndrome, which was associated with older age and lower intelligence. Results of the repeated measurements show that the earlier growth hormone treatment was started during infancy, the better the adaptive skills were on the long-term.

  19. Retinal vascular tortuosity in DiGeorge syndrome complicated by solar retinopathy.

    Science.gov (United States)

    De Niro, Jennifer E; Randhawa, Sandeep; McDonald, H Richard

    2013-01-01

    To report a case of vascular tortuosity associated with DiGeorge syndrome that was complicated by solar retinopathy. Case report and literature review. A 56-year-old woman with DiGeorge syndrome with secondary schizophrenia and developmental delay presented with decreased vision that was worse in her left eye. Ocular examination revealed bilateral retinal vascular tortuosity involving both the arteries and veins. Both eyes had an abnormal foveal light reflex with a central yellowish hue, which was more pronounced in the left eye. Optical coherence tomography showed disruption of the photoreceptor inner segment-outer segment junction and retinal pigment epithelium centrally, which was also more prominent in the left eye. DiGeorge syndrome is associated with retinal vascular tortuosity in a large percentage of patients. The patient's decreased vision is likely caused by solar retinopathy (prolonged sun gazing as a result of the secondary schizophrenia and developmental delay).

  20. Gingival fibromatosis and growth hormone deficiency syndrome--report of a rare case and review of literature.

    Science.gov (United States)

    Radhakrishnan, S; Rajan, P

    2003-01-01

    Oikarinen et al in 1989 reported a syndrome associated with generalized gingival fibromatosis and growth hormone deficiency. This is a case report of a 15-year-old female patient who presented to the Government Dental College, Chennai with generalized gingival fibromatosis and growth hormone deficiency. Interestingly, the histopathology of the excised gingival overgrowth showed dense collagenous connective tissue in which were strewn calcified structures that resembled cementum. This syndrome is being reported for the second time after its first case report in 1989 by Oikarinen et al. We are herewith reporting this case for its rarity with a brief review of literature of syndromes associated with generalized gingival fibromatosis.

  1. Complications in mechanically ventilated patients of Guillain-Barre syndrome and their prognostic value.

    Science.gov (United States)

    Netto, Archana Becket; Taly, Arun B; Kulkarni, Girish B; Uma Maheshwara Rao, G S; Rao, Shivaji

    2017-01-01

    The spectrum of various complications in critically ill Guillain-Barre syndrome (GBS) and its effect on the prognosis is lacking in literature. This study aimed at enumerating the complications in such a cohort and their significance in the prognosis and mortality. Retrospective case record analysis of all consecutive mechanically ventilated patients of GBS in neurology Intensive Care Unit (ICU) of a tertiary care institute for 10 years was done. Demographic, laboratory, and treatment details and outcome parameters were recorded. Among the 173 patients were 118 men and 55 women (2.1:1), aged 1-84 years. The average number of ICU complications per patient was 6.8 ± 1.8 (median = 7, range = 1-12). The most common complication was tracheobronchitis (128). Other pulmonary complications were found in 36 patients. The next was metabolic hyponatremia (115) hypokalemia (67), hypocalcemia (13), stress hyperglycemia (10), hyperkalemia (8), hypernatremia (9). Sepsis (40), UTI (47), dysautonomia (27), hypoalbuminemia (76), anemia (75), seizures (8), paralytic ileus (5), bleeding (4), anoxic encephalopathy (3), organ failures (12), deep vein thrombosis (7), and drug rashes (1) were also noted. The complications, considered significant in causing death, Hughes scale ≤ 3 at discharge, prolonged mechanical ventilation (>21 days) and hospitalization (>36 days) were pneumonia, hyponatremia, hypokalemia, urinary infection, tracheobronchial infections, hypoalbuminemia, sepsis, anemia dysautonomia. Active monitoring and appropriate and early intervention by the clinician will improve the quality of life of these patients and reduce the cost of prolonged mechanical ventilation and ICU stay.

  2. [Quality of life of patients at late terms after surgical treatment of complicated forms of diabetic foot syndrome].

    Science.gov (United States)

    Grachev, T V; Levchik, E Iu

    2010-01-01

    Quality of life were explored in an open prospective controlled study in 116 patients with complicated diabetic foot syndrome (experimental group) and 30 diabetics without this syndrome (control group) by questioning of quality of life (questionnaires SF-36, Functional Scale of Lower Extremity) in long terms (6-18 months) after surgical treatment. Advantages of foot "saving" resections in surgical treatment of complicated diabetic boot syndrome were detected: patients without amputations had the physical wellbeing score and the Functional Scale of Lower Extremity number, comparable with the group of diabetics without diabetic foot syndrome. The integral mental wellbeing scores were low in all observed groups of patients.

  3. Restless legs syndrome and pregnancy or delivery complications in China: a representative survey.

    Science.gov (United States)

    Liu, Gangqiong; Li, Ling; Zhang, Jinying; Xue, Rui; Zhao, Xiaoyan; Zhu, Kui; Wang, Yunzhe; Xiao, Lili; Shangguan, Jiahong

    2016-01-01

    To perform a detailed epidemiological study of Chinese women and the relationship between restless legs syndrome (RLS) in pregnancy and any associated complications during delivery. A total of 3874 pregnant women (18-40 years) who had delivered babies at The First Affiliated Hospital of Zhengzhou University from May 2011 to May 2014 were enrolled in the study. Using a face-to-face interview questionnaire, data were collected pertaining to RLS incidence in pregnancy and any associated complications during delivery. The relationship between RLS frequency and pregnancy or delivery complications was further investigated. Among 12.3% of the eligible participants, RLS prevalence occurred at least weekly. Older pregnant women were more likely to suffer from RLS. Individuals who experienced RLS at least weekly reported a statistically higher frequency of excessive daytime sleepiness and an increased prevalence of hypertension, cardiovascular disease, and preeclampsia compared with those without RLS. The study revealed that RLS was frequent in Chinese pregnant women, especially in those with pregnancy or delivery complications such as hypertension, cardiovascular disease, and preeclampsia. Restless legs syndrome was also associated with excessive daytime sleepiness. Copyright © 2015 Elsevier B.V. All rights reserved.

  4. Complications of Stevens-Johnson syndrome beyond the eye and skin.

    Science.gov (United States)

    Saeed, Hajirah; Mantagos, Iason S; Chodosh, James

    2016-02-01

    Ocular and cutaneous disease are common chronic sequelae of Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) and have been well described in the literature. Long-term complications affecting other organ systems have not been so well described. The purpose of this review article is to highlight non-ocular and non-cutaneous chronic complications of SJS/TEN. The PubMed database was searched for the keywords "Stevens-Johnson syndrome" and "toxic epidermal necrolysis" through September 1, 2014. Relevant articles were then reviewed in full. 138 articles in the English language were found that described chronic sequelae of SJS/TEN. Our search revealed six affected organ systems other than the eyes and integument, with chronic sequelae from SJS/TEN: respiratory, gastrointestinal/hepatic, oral, otorhinolaryngologic, gynecologic/genitourinary, and renal. Complications involving these organs systems appeared likely to reduce the quality of life for SJS/TEN survivors. SJS/TEN is a multi-organ disease requiring multidisciplinary care from a variety of specialists. Affected patients have complex hospital stays, and their quality of life may be severely impacted by multiple long-term complications. We believe that preventative care in the acute setting might limit the development and progression of many of the sequelae described above. Copyright © 2015 Elsevier Ltd and ISBI. All rights reserved.

  5. [Major complications of acquired renal cystic disease: Wünderlich syndrome and papilar carcinoma.

    Science.gov (United States)

    Rodríguez-Zarco, Enrique; Vallejo-Benítez, Ana; Delgado Cotán, Antonio; Pereira-Gallardo, Sofía

    2017-07-01

    We report a case of acquired renal cystic disease associated with renal dialysis and endstage renal disease. The patient suffered the two major complications related with acquired renal cystic disease; hemorrhage and renal carcinoma. Our case is a patient with acquired renal cystic disease, single kidney after surgery for renal clear cell carcinoma four years earlier, who developed a Wünderlich syndrome (WS). The histological study of the nephrectomy specimen showed a renal papillary carcinoma in the context of acquired renal cystic disease after surgery for a WS. Renal hemorrhage is a serious complication that can lead to a fatal outcome. Patients undergoing dialysis should be monitored actively due to the risk of developing acquired renal cystic disease and associated complications.

  6. Obesity hypoventilation syndrome, sleep apnea, overlap syndrome: perioperative management to prevent complications.

    Science.gov (United States)

    Raveendran, Raviraj; Wong, Jean; Singh, Mandeep; Wong, David T; Chung, Frances

    2017-02-01

    The prevalence of sleep disordered breathing (SDB) is increasing proportional to the prevalence of obesity. Although anesthesiologists are familiar with obstructive sleep apnea (OSA) - the most common SDB, anesthesiologists may not be aware of other SDB such as obesity hypoventilation syndrome (OHS) and overlap syndrome (combination of OSA and chronic obstructive pulmonary disease). The present review provides an update of information regarding the perioperative management of OHS and overlap syndrome. OHS and overlap syndrome are associated with significant comorbid conditions and more perioperative morbidity than OSA alone. Similar to OSA, most of the OHS patients are undiagnosed. An increase in serum bicarbonate level is a surrogate marker of hypercapnia. Because 90% of OHS patients have OSA, preoperative screening for OSA combined with estimation of serum bicarbonate level may detect the majority of the patients with OHS. In patients with OSA, OHS, and overlap syndrome, improvement in the perioperative outcome has been shown by initiating positive airway pressure therapy. Identification and preoperative optimization of these high-risk patients are most important. A protocol-based risk mitigation is necessary for improving the intraoperative and postoperative outcome of these patients. As a perioperative physician, anesthesiologists have a key role in the management of patients with SDB.

  7. Noncontraceptive use of oral combined hormonal contraceptives in polycystic ovary syndrome-risks versus benefits.

    Science.gov (United States)

    Dokras, Anuja

    2016-12-01

    The use of steroid sex hormones for noncontraceptive benefits has been endorsed by several medical societies. In women with polycystic ovary syndrome (PCOS), hormonal contraceptives are first-line therapy for concurrent treatment of menstrual irregularity, acne, and hirsutism. The association of PCOS with obesity, diabetes, and dyslipidemia frequently brings up the debate regarding risks versus benefits of hormonal contraceptives in this population. In women with PCOS, the lack of large-scale studies evaluating the risks with varying doses of ethinyl estradiol, types of progestins, and presence of confounding factors such as obesity, smoking, and other cardiometabolic comorbidities is a significant limitation in these deliberations. Although it is important to assess the absolute risk for major morbidities including cardiovascular events, currently, there are a paucity of long-term data for these outcomes in PCOS. Most of the current studies do not suggest an increase in risk of prediabetes/diabetes, clinically significant dyslipidemia, inflammatory changes, or depressive/anxiety symptoms with oral contraceptive pill use. Screening of women with PCOS for cardiometabolic and psychiatric comorbidities is routinely recommended. This information should be used by health care providers to individualize the choice of hormonal contraceptive treatment, adequately counsel patients regarding risks and benefits, and formulate an appropriate follow-up plan. Copyright © 2016 American Society for Reproductive Medicine. Published by Elsevier Inc. All rights reserved.

  8. Metabolic Syndrome, Hormone Levels, and Inflammation in Patients with Erectile Dysfunction

    Directory of Open Access Journals (Sweden)

    Miguel Ángel Arrabal-Polo

    2012-01-01

    Full Text Available Background. The end point of this study was to investigate the prevalence of MS in patients with ED in comparison with control subjects and to analyse the association with acute phase reactants (CRP, ESR and hormone levels. Methods. This case-control study included 65 patients, 37 with erectile dysfunction, according to the International Index of Erectile Function (IIEF from the Urology Department of San Cecilio University Hospital, Granada (Spain and 28 healthy controls. The prevalence of metabolic syndrome was calculated according to ATP-III criteria. Hormone levels and acute phase parameters were studied in samples drawn. Results. The ATP-III criteria for MS were met by 64.9% of the patients with ED and only 9.5% of the controls (P<0.0001, OR = 17.53, 95% CI: 3.52–87.37. Binary logistic regression analysis showed a strong association between patients with ED and MS, even after additional adjustment for confounding factors (OR = 20.05, 95% CI: 1.24–32.82, P<0.034. Patients with hypogonadism presented a significantly higher prevalence of metabolic syndrome. Multiple linear regression analysis showed that systolic BP and CRP predicted 0.46 (model R2 of IIEF changes. Conclusion. Chronic inflammation found in patients with ED might explain the association between ED and metabolic syndrome.

  9. Hormonal and adiposity state of women with polycystic ovary syndrome: implication of adiponectin and leptin

    Directory of Open Access Journals (Sweden)

    Aleksandra Atanasova Boshku

    2016-12-01

    Full Text Available Obesity and insulin resistance are frequently seen comorbidities in patients with polycystic ovary syndrome (PCOS, affecting the already disturbed metabolism of these patients. Disturbed secretion of adiponectin and leptin could be one of the contributing factors of obesity and insulin resistance in patients with PCOS. The aim of this study was to determine the levels of adiponectin and leptin in PCOS patients, as well as their association with other components of the syndrome. This cross-sectional study determined clinical, hormonal, and biochemical markers in 61 women with PCOS and 56 controls. There was a statistically significant difference in adiponectin and leptin between the groups (p>0.001. There was a significant negative correlation between adiponectin, body mass index (BMI, and waist circumference (r= -0.478; -0.452, p<0.001 and a negative correlation with testosterone, free androgen index (FAI, insulin, and the homeostasis model assessment for insulin resistance (HOMA-IR. A positive correlation between adiponectin, sex hormone binding globulin (SHGB, and fasting glucose levels was present. Correlation analysis of leptin with other metabolic parameters showed a positive correlation with BMI, waist circumference, insulin, and HOMA-IR. A significant inverse correlation was present between leptin and SHGB. In conclusion, adiponectin and leptin may serve as potential biomarkers of insulin resistance. Determining levels of adiponectin and leptin in the early course of this syndrome may enable earlier diagnosis of insulin resistance, or even early prevention in PCOS patients.

  10. Association between sex hormone-binding globulin (SHBG and metabolic syndrome among men

    Directory of Open Access Journals (Sweden)

    Emmanuela Quental Callou de Sá

    Full Text Available CONTEXT AND OBJECTIVE: Metabolic syndrome consists of a set of factors that imply increased risk of cardiovascular diseases. The objective here was to evaluate the association between sex hormone-binding globulin (SHBG, sex hormones and metabolic syndrome among men. DESIGN AND SETTING: Retrospective analysis on data from the study "Endogenous oestradiol but not testosterone is related to coronary artery disease in men", conducted in a hospital in São Paulo. METHODS: Men (aged 40-70 who underwent coronary angiography were selected. The age, weight, height, waist circumference, body mass index and prevalence of dyslipidemia, hypertension and diabetes of each patient were registered. Metabolic syndrome was defined in accordance with the criteria of the Third Report of the National Cholesterol Education Program Expert Panel on Detection, Evaluation and Treatment of High Blood Cholesterol in Adults (NCEP-ATPIII. Serum samples were collected to assess the levels of glucose, total cholesterol, HDL-cholesterol (high density lipoprotein, triglycerides, albumin, SHBG, estradiol and total testosterone (TT. The levels of LDL-cholesterol (low density lipoprotein were calculated using Friedewald's formula and free testosterone (FT and bioavailable testosterone (BT using Vermeulen's formula. RESULTS: 141 patients were enrolled in the study. The prevalence of metabolic syndrome was significantly higher in the first SHBG tercile than in the second and third terciles. A statistically significant positive association between the SHBG and TT values was observed, but no such association was seen between SHBG, BT and FT. CONCLUSION: Low serum levels of SHBG are associated with higher prevalence of metabolic syndrome among male patients, but further studies are required to confirm this association.

  11. Increased Need for Gastrointestinal Surgery and Increased Risk of Surgery-Related Complications in Patients with Ehlers-Danlos Syndrome

    DEFF Research Database (Denmark)

    Kulas Søborg, Marie-Louise; Leganger, Julie; Rosenberg, Jacob

    2017-01-01

    BACKGROUND/AIMS: Ehlers-Danlos syndromes (EDSs) constitute a rare group of inherited connective tissue diseases, characterized by multisystemic manifestations and general tissue fragility. Most severe complications include vascular and gastrointestinal (GI) emergencies requiring acute surgery...

  12. Complete androgen insensitivity syndrome and anti-Müllerian hormone levels before and after laparoscopic gonadectomy

    Directory of Open Access Journals (Sweden)

    Maki Kusumi

    2017-08-01

    Full Text Available We report cases of two sisters with complete androgen insensitivity syndrome (CAIS. A complete female appearance, blind-ending vagina, and testes in the pelvis are characteristics of CAIS. Prophylactic laparoscopic gonadectomy was performed in both cases. Anti-Müllerian hormone (AMH level is known to be very high in patients with CAIS; AMH is secreted by Sertoli cells and testosterone suppresses the secretion. In our cases, serum AMH was very high before gonadectomy and dramatically decreased after gonadectomy. AMH could be the diagnostic feature for patients with CAIS.

  13. Complicated Cholelithiasis: An Unusual Combination of Acute Pancreatitis and Bouveret Syndrome

    Directory of Open Access Journals (Sweden)

    Ioannis Baloyiannis

    2012-07-01

    Full Text Available Bouveret syndrome is a rare form of gallstone ileus. The purpose of the present study was to present the unusual case of a female patient with complicated cholelithiasis manifested as a combination of acute pancreatitis and concomitant Bouveret syndrome. A 61-year-old female patient was admitted to the emergency department complaining of mid-epigastric and right upper quadrant abdominal pain radiating band-like in the thoracic region of the back as well as repeated episodes of vomiting over the last 24 h. The initial correct diagnosis of pancreatitis was subsequently combined with the diagnosis of Bouveret syndrome as a computed tomography scan revealed the presence of a gallstone within the duodenum causing luminal obstruction. After failure of endoscopic gallstone removal, a surgical approach was undertaken where gallstone removal was followed by cholecystectomy and restoration of the anatomy by eliminating the fistula. The concomitant pancreatitis complicated the postoperative period and prolonged the length of hospital stay. However, the patient was discharge on the 45th postoperative day. Attempts for endoscopic removal of the impacted stone should be the initial therapeutic step. Surgery should be reserved for cases refractory to endoscopic intervention and when definite treatment is the actual challenge.

  14. Heterotopic triplet pregnancy complicated with ovarian hyperstimulation syndrome following in vitro fertilization.

    Science.gov (United States)

    Tomić, Vlatka; Tomić, Jozo; Kuna, Krunoslav; Zigmundovac-Klaić, Durda

    2011-12-01

    Heterotopic pregnancy is a rare event that occurs in less than 1% of pregnancies following in vitro fertilization and embryo transfer, especially when complicated with ovarian hyperstimulation syndrome. We report a case of a 31-year-old woman in the 6th gestational week of pregnancy achieved after in vitro fertilization, who was complaining of acute lower abdominal pain and distension, breathing difficulties and vaginal spotting. Transvaginal ultrasound examination and laboratory tests confirmed the ovarian hyperstimulation syndrome in the presence of two viable gestational sacs. The patient's condition worsened five days later with sudden onset of sharp abdominal pain, nausea and vomiting, along with impaired laboratory test values. Laparoscopy was attempted, but enlarged ovaries and adhesions prevented further procedure, which was then converted to mini-laparotomy. Operative removal of the right tubal pregnancy resulted in uncomplicated course of the intrauterine twin pregnancy and delivery of two healthy neonates by cesarean section at 37 weeks of gestation. Clinicians need to be aware of this rare complication where ovarian hyperstimulation syndrome coexists with heterotopic pregnancy after in vitro fertilization and embryo transfer procedure. Enlarged ovaries may mask accurate ultrasound diagnosis, but timely surgical intervention can prevent fatal consequences and result in normal course and outcome of intrauterine pregnancy.

  15. [The use of growth hormone to treat endocrine-metabolic disturbances in acquired immunodeficiency syndrome (AIDS) patients].

    Science.gov (United States)

    Spinola-Castro, Angela Maria; Siviero-Miachon, Adriana A; da Silva, Marcos Tadeu Nolasco; Guerra-Junior, Gil

    2008-07-01

    Acquired Immunodeficiency Syndrome (Aids) was initially related to HIV-associated wasting syndrome, and its metabolic disturbances to altered body composition. After Highly Active Antiretroviral Therapy (HAART) was started, malnutrition has declined and HIV-associated lipodystrophy syndrome has emerged as an important metabolic disorder. Aids is also characterized by hormonal disturbances, principally in growth hormone/insulin-like growth factor 1 (GH/IGF-1) axis. The use of recombinant human GH (hrGH) was formerly indicated to treat wasting syndrome, in order to increase lean body mass. Even though the use of hrGH in lipodystrophy syndrome has been considered, the decrease in insulin sensitivity is a limitation for its use, which has not been officially approved yet. Diversity in therapeutic regimen is another limitation to its use in Aids patients. The present study has reviewed the main HIV-related endocrine-metabolic disorders as well as the use of hrGH in such conditions.

  16. The role of levosimendan in acute heart failure complicating acute coronary syndrome

    DEFF Research Database (Denmark)

    Nieminen, Markku S; Buerke, M.; Cohen-Solal, A.

    2016-01-01

    defined as exclusion criteria in trials and registries. As a consequence, guideline recommendations are mostly driven by observational studies, even though these patients have a particularly poor prognosis compared to heart failure patients without signs of coronary artery disease. In acute heart failure......Acute heart failure and/or cardiogenic shock are frequently triggered by ischemic coronary events. Yet, there is a paucity of randomized data on the management of patients with heart failure complicating acute coronary syndrome, as acute coronary syndrome and cardiogenic shock have frequently been...... are generally less common than with other inotropic and vasoactive therapies, with the notable exception of hypotension. The decision to use levosimendan, in terms of timing and dosing, is influenced by the presence of pulmonary congestion, and blood pressure measurements. Levosimendan should be preferred over...

  17. Capgras syndrome: a clinical manifestation of watershed cerebral infarct complicating the use of extracorporeal membrane oxygenation.

    Science.gov (United States)

    Dejode, J M; Antonini, F; Lagier, P; Martin, C

    2001-08-01

    Ischaemic cerebral accidents are frequent following extracorporeal membrane oxygenation (ECMO), especially after fixing the reinjection cannula in the right primitive carotid artery, which leads to an interruption in downstream flow. We describe a rare and unusual symptom of cerebral ischaemic accident that is known as Capgras syndrome. This feature is interesting because it may be documented by computed tomography (CT) scan and particular electroencephalography signals. It appears that our observation represents the first documented case of Capgras syndrome complicating ECMO. This incident emphasizes the potential hazards associated with right common artery ligature for venoarterial extracorporeal membrane oxygenation (VAECMO). In addition, it shows that this psychiatric symptom (that has been interpreted psychodynamically for many years) can have an organic basis, which should be studied.

  18. Organophosphate intermediate syndrome with neurological complications of extrapyramidal symptoms in clinical practice

    Directory of Open Access Journals (Sweden)

    Mark B. Detweiler

    2014-01-01

    Full Text Available Organophosphates (OPs are ubiquitous in the world as domestic and industrial agricultural insecticides. Intentional poisoning as suicides attempts are clinical phenomena seen in emergency departments and clinics in agricultural areas. Intermediate syndrome with the neurological complication of extra pyramidal symptoms following acute OP ingestion may occur in pediatric and adult cases. While death is the most serious consequence of toxic OP doses, low levels of exposure and nonfatal doses may disrupt the neurobehavioral development of fetuses and children in addition to bring linked to testicular cancer and male and female infertility. These are disturbing. Chronic and acute toxicity from OPs are barriers to the health of our present and future generations. Symptoms and treatment of acute and chronic OP exposure are briefly referenced with inclusion of the intermediate syndrome. Suggestions for local and systemic reduction of the acute and long term consequences of OP ingestion are opined.

  19. Pregnancy Complications and the Risk of Metabolic Syndrome for the Offspring.

    Science.gov (United States)

    Ryckman, Kelli K; Borowski, Kristi S; Parikh, Nisha I; Saftlas, Audrey F

    2013-06-01

    Metabolic syndrome is a growing problem globally, and is a contributor to non-communicable diseases such as type II diabetes and cardiovascular disease. The risk of developing specific components of the metabolic syndrome such as obesity, hyperlipidemia, hypertension, and elevated fasting blood sugar has been largely attributed to environmental stressors including poor nutrition, lack of exercise, and smoking. However, large epidemiologic cohorts and experimental animal models support the "developmental origins of adult disease" hypothesis, which posits that a significant portion of the risk for adult metabolic conditions is determined by exposures occurring in the perinatal period. Maternal obesity and the rate of complications during pregnancy such as preterm birth, preeclampsia, and gestational diabetes continue to rise. As our ability to reduce perinatal morbidity and mortality improves the long-term metabolic consequences remain uncertain, pointing to the need for further research in this area.

  20. Iatrogenic Cushing’s Syndrome Caused by Topical Corticosteroid Application and Its Life Threatening Complications

    Directory of Open Access Journals (Sweden)

    Razzaghy Azar

    2015-11-01

    Full Text Available Introduction The use of topical corticosteroids in children and adults, especially in covered areas and for a prolonged duration, may result in Cushing’s syndrome and suppression of the hypothalamic-pituitary-adrenal axis. Case Presentation The present report describes the cases of three children and a 25-year-old man who presented with clinical features of Cushing’s syndrome and adrenal insufficiency following prolonged use of clobetasol cream. Two of the children continued with hypertension even after discontinuation of clobetasol, and were treated with angiotensin-converting enzyme inhibitors. One of these two children had developed acute renal failure, hypertensive encephalopathy, and permanent visual loss prior to admission. Conclusions Whenever a cushingoid appearance is accompanied by adrenal insufficiency, the exogenous use of steroids, especially topical creams, should be explored. Hypertension may persist even after cessation of use and may result in life threatening complications.

  1. [Cardiovascular complications of Kawasaki syndrome: results of a French multicenter study].

    Science.gov (United States)

    Chantepie, A; Mauran, P; Lusson, J R; Vaillant, M C; Bozio, A

    2001-07-01

    Between March 1, 1995 and February 29, 1996, a multicentric prospective study was conducted in France in order to analyze the cardiovascular complications in Kawasaki syndrome, and to describe the echocardiographic features and the outcome of coronary lesions. Forty-nine cases of Kawasaki syndrome were observed. There were 32 boys and 17 girls (sex ratio: 1:9). The age at diagnosis was under one year in ten cases (20.4%), between one and five years in 27 cases (55.1%) and more than five years in 12 cases (24.5%). The complete diagnostic criteria were present in 42 cases (85.1%). Forty-five children (91.8%) were given intravenous immunoglobulin treatment but only 20 (40.8%) received this treatment within the seven days following the onset of the illness. Cardiovascular complications consisted of: pericardial effusion in 12 cases (24.5%), coronary dilation in seven cases (14.3%), coronary aneurysms of moderate size in seven cases (14.3%,) with hypokinetic left ventricle in two cases. No death was reported. All patients with coronary dilation and four patients with coronary aneurysms had a normal size of coronary arteries at echocardiography within the nine months of the follow-up. Among the three other patients, after a follow-up of two years, one still has a small coronary aneurysm and two have a normal size of coronary arteries. Despite a delayed administration of immunoglobulin therapy in the majority of patients in this study, outcome of coronary lesions was favorable and severe cardiac complications were rare in the acute phase of the Kawasaki syndrome.

  2. Acute Headache at Emergency Department: Reversible Cerebral Vasoconstriction Syndrome Complicated by Subarachnoid Haemorrhage and Cerebral Infarction

    Directory of Open Access Journals (Sweden)

    M. Yger

    2015-01-01

    Full Text Available Introduction. Reversible cerebral vasoconstriction syndrome is becoming widely accepted as a rare cause of both ischemic and haemorrhagic stroke and should be evocated in case of thunderclap headaches associated with stroke. We present the case of a patient with ischemic stroke associated with cortical subarachnoid haemorrhage (cSAH and reversible diffuse arteries narrowing, leading to the diagnosis of reversible vasoconstriction syndrome. Case Report. A 48-year-old woman came to the emergency department because of an unusual thunderclap headache. The computed tomography of the brain completed by CT-angiography was unremarkable. Eleven days later, she was readmitted because of a left hemianopsia. One day after her admission, she developed a sudden left hemiparesis. The brain MRI showed ischemic lesions in the right frontal and occipital lobe and diffuse cSAH. The angiography showed vasoconstriction of the right anterior cerebral artery and stenosis of both middle cerebral arteries. Nimodipine treatment was initiated and vasoconstriction completely regressed on day 16 after the first headache. Conclusion. Our case shows a severe reversible cerebral vasoconstriction syndrome where both haemorrhagic and ischemic complications were present at the same time. The history we reported shows that reversible cerebral vasoconstriction syndrome is still underrecognized, in particular in general emergency departments.

  3. Gastrointestinal Surgery and Related Complications in Patients with Ehlers-Danlos Syndrome

    DEFF Research Database (Denmark)

    Burcharth, Jakob; Rosenberg, Jacob

    2012-01-01

    Introduction: Ehlers-Danlos syndrome (EDS) is a rare inherited group of connective tissue diseases characterized by joint hypermobility, skin hyperextensibility and bruising tendency. Common features of patients with EDS include vascular and gastrointestinal perforations. The purpose of this syst......Introduction: Ehlers-Danlos syndrome (EDS) is a rare inherited group of connective tissue diseases characterized by joint hypermobility, skin hyperextensibility and bruising tendency. Common features of patients with EDS include vascular and gastrointestinal perforations. The purpose...... of this systematic review is to address gastrointestinal diseases and the complications associated with surgical treatment of diseases relating to the gastrointestinal system in patients with EDS. Methods: PubMed search including the Medical Subject Heading (MeSH) terms 'Ehlers-Danlos Syndrome' and 'Gastrointestinal...... Diseases', and an Embase search including the Map Term to Subject Heading 'Ehlers-Danlos Syndrome' with 'AND' function of the keyword 'Gastrointestinal'. Results: The literature search resulted in inclusion of 53 articles after application of eligibility criteria. The primary results drawn from...

  4. [Extracranial vertebral artery aneurysm complicating Klippel-Feil syndrome: case report].

    Science.gov (United States)

    Shimizu, S; Kojima, T; Morooka, Y; Tanaka, K; Nakagawa, Y; Kuroki, M

    1996-10-01

    The Klippel-Feil syndrome is one of the spinal malformations characterized by fusion of the cervical vertebrae. It is well known that the malformation can cause some neurologic disorders. However, an aneurysm in the vertebral artery associated with the Klippel-Feil syndrome is extremely rare, with only one case having been reported in the literature. We present a 39-year-old female with a sudden onset of disturbed consciousness. Lateral cervical x-ray films showed the Klippel-Feil syndrome and hypermobility between C1 and C2. MRI showed multiple infarctions in the posterior circulation, including bilateral thalami. Right vertebral angiogram identified the extracranial vertebral artery aneurysm as the source of the emboli. The patient gradually recovered with conservative therapy. The aneurysm was thought to be produced by chronic arterial trauma secondary to excessive movement between C1 and C2. We conclude that an extracranial vertebral artery aneurysm is a serious complication in a patient with the Klippel-Feil syndrome.

  5. MACROPHAGE ACTIVATION SYNDROME AS A COMPLICATION OF SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS – CASE REPORT

    Directory of Open Access Journals (Sweden)

    Viktorija Kerin

    2014-05-01

    Full Text Available 800x600 Abstract Macrophage activation syndrome (MAS is a life-threatening complication of systemic juvenile idiopathic arthritis (SJIA. MAS is characterized by systemic inflammation caused by excessive or uncontrolled release of proinflammatory cytokines (cytokine storm. The diagnostic hallmark are hemophagocytic macrophages, that could be present in bone marrow, liver, spleen or lymph nodes. Clinical features are similar to a flare of the underlying rheumatic disease which makes early recognition and choice of the appropriate treatment difficult. Diagnosis is made according to the preliminary diagnostic guidelines for MAS complicating SJIA.We report a case of an 11 years old girl with MAS as an initial presentation of SJIA. She was successfully treated with high doses of glucocorticoid and cyclosporine. After discontinuation of glucocorticoid therapy she developed a new flare of the disease which was successfully treated with interleukin 1 blocking agent anakinra.         

  6. Efficacy of metformin on pregnancy complications in women with polycystic ovary syndrome: a meta-analysis.

    Science.gov (United States)

    Feng, Li; Lin, Xiao-Fang; Wan, Zhi-Hua; Hu, Dan; Du, Yu-Kai

    2015-01-01

    To evaluate the efficacy of metformin administration throughout pregnancy on pregnancy-related complications in women with polycystic ovary syndrome (PCOS). MEDLINE and ScienceDirect were searched to retrieve relevant trials. The endpoint was the incidence of complications of pregnancy, gestational diabetes mellitus (GDM), pre-eclampsia (PE), miscarriage and premature birth included. Five studies with 502 PCOS patients with metformin administration throughout pregnancy and 427 controls who used metformin just to get conception were included in our meta-analysis. In study group, a significantly lower change of emerging miscarriage and premature birth was observed, the pooled relative risk (RR) was 0.32 (95% confidence interval (CI): 0.19-0.56) for miscarriage and 0.40 (95%CI: 0.18-0.91) for premature birth. No significant difference was demonstrated in emerging GDM and PE. Metformin therapy throughout pregnancy can reduce the RR of miscarriage and premature birth incidence in PCOS patients with no serious side effects.

  7. Long QT syndrome and torsades de pointes complicating mitral valve replacement

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    Shegu Gilbert

    2016-09-01

    Full Text Available Severe QT interval prolongation >500 ms occurs in one quarter of cardiac surgical patients in the perioperative period while moderate prolongation occurs in most of them. Prolonged QT interval may be associated with torsades de pointes and lead to sudden cardiac death. Because of the high incidence of prolonged QT in cardiac surgery patients and its perioperative adverse outcomes, it is vital to identify it early and take necessary precautions. We report and discuss the catastrophic events and management of two patients with long QT syndrome complicating mitral valve replacement.

  8. Long QT syndrome and torsades de pointes complicating mitral valve replacement.

    Science.gov (United States)

    Gilbert, Shegu; Singh, Devender; Jesuraj, M Lawrance

    2016-09-01

    Severe QT interval prolongation >500ms occurs in one quarter of cardiac surgical patients in the perioperative period while moderate prolongation occurs in most of them. Prolonged QT interval may be associated with torsades de pointes and lead to sudden cardiac death. Because of the high incidence of prolonged QT in cardiac surgery patients and its perioperative adverse outcomes, it is vital to identify it early and take necessary precautions. We report and discuss the catastrophic events and management of two patients with long QT syndrome complicating mitral valve replacement. Copyright © 2015 Cardiological Society of India. Published by Elsevier B.V. All rights reserved.

  9. Bilateral gluteal compartment syndrome complicated by rhabdomyolysis and acute kidney injury in a patient with alcohol intoxication

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    Jae Young Cho

    2012-12-01

    Full Text Available Bilateral gluteal compartment syndrome is a rare clinical entity that can be complicated by rhabdomyolysis or acute kidney injury (AKI. We report the a case of a 30-year-old woman without any comorbid diseases who was diagnosed with bilateral gluteal compartment syndrome complicated by rhabdomyolysis and dialysis-requiring AKI, which was caused by prolonged immobilization under the influence of alcohol. Although the patient’s renal function recovered fully after 5 sessions of hemodialysis, sciatic neuropathy caused by gluteal compartment syndrome led to permanent foot drop.

  10. [Inferior myocardial infarction complicated by complete heart block and cardiac arrest following a gadolinium injection: A case of Kounis syndrome].

    Science.gov (United States)

    Demoulin, R; Poyet, R; Capilla, E; Cardinale, M; Tortat, A V; Pons, F; Brocq, F-X; Jego, C; Foucault, G; Cellarier, G-R

    2017-11-01

    Kounis syndrome is an allergic acute coronary syndrome. It occurs on healthy or pathological arteries. Its complications, although often benign, can lead to cardiac arrest and death. Its triggering factors are multiple and include contrast products used in diagnostic imaging. We report the case of an 81 years old patient affected by hepatocellular carcinoma, who presented a type 2 Kounis syndrome with inferior myocardial infarction, complicated by cardiac arrest related to complete heart block following a gadoteric acid injection. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

  11. Complications in mechanically ventilated patients of Guillain–Barre syndrome and their prognostic value

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    Archana Becket Netto

    2017-01-01

    Full Text Available Introduction: The spectrum of various complications in critically ill Guillain–Barre syndrome (GBS and its effect on the prognosis is lacking in literature. This study aimed at enumerating the complications in such a cohort and their significance in the prognosis and mortality. Materials and Methods: Retrospective case record analysis of all consecutive mechanically ventilated patients of GBS in neurology Intensive Care Unit (ICU of a tertiary care institute for 10 years was done. Demographic, laboratory, and treatment details and outcome parameters were recorded. Results: Among the 173 patients were 118 men and 55 women (2.1:1, aged 1–84 years. The average number of ICU complications per patient was 6.8 ± 1.8 (median = 7, range = 1–12. The most common complication was tracheobronchitis (128. Other pulmonary complications were found in 36 patients. The next was metabolic hyponatremia (115 hypokalemia (67, hypocalcemia (13, stress hyperglycemia (10, hyperkalemia (8, hypernatremia (9. Sepsis (40, UTI (47, dysautonomia (27, hypoalbuminemia (76, anemia (75, seizures (8, paralytic ileus (5, bleeding (4, anoxic encephalopathy (3, organ failures (12, deep vein thrombosis (7, and drug rashes (1 were also noted. The complications, considered significant in causing death, Hughes scale ≤ 3 at discharge, prolonged mechanical ventilation (>21 days and hospitalization (>36 days were pneumonia, hyponatremia, hypokalemia, urinary infection, tracheobronchial infections, hypoalbuminemia, sepsis, anemia dysautonomia. Conclusion: Active monitoring and appropriate and early intervention by the clinician will improve the quality of life of these patients and reduce the cost of prolonged mechanical ventilation and ICU stay.

  12. Acute morbidity and complications of thigh compartment syndrome: A report of 26 cases

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    Kanlic Enes M

    2010-08-01

    Full Text Available Abstract Background To describe the patient population, etiology, and complications associated with thigh compartment syndrome (TCS. TCS is a rare condition, affecting less than 0.3% of trauma patients, caused by elevated pressure within a constrained fascial space which can result in tissue necrosis, fibrosis, and physical impairment in addition to other complications. Compartment releases performed after irreversible tissue ischemia has developed can lead to severe infection, amputation, and systemic complications including renal insufficiency and death. Methods This study examines the course of treatment of 23 consecutive patients with 26 thigh compartment syndromes sustained during an eight-year period at two Level 1 trauma centers, each admitting more than 2,000 trauma patients yearly. Results Patients developing TCS were young (average 35.4 years and likely to have a vascular injury on presentation (57.7%. A tense and edematous thigh was the most consistent clinical exam finding leading to compartment release (69.5%. Average time from admission to the operating room was 18 +/- 4.3 hours and 8/23 (34.8% were noted to have ischemic muscle changes at the time of release. Half of those patients (4/8 developed local complications requiring limb amputations. Conclusion TCS is often associated with high energy trauma and is difficult to diagnose in uncooperative, obtunded and multiply injured patients. Vascular injuries are a common underlying cause and require prompt recognition and a multidisciplinary approach including the trauma and orthopaedic surgeons, intensive care team, vascular surgery and interventional radiology. Prompt recognition and treatment of TCS are paramount to avoid the catastrophic acute and long term morbidities.

  13. [Influence of duration of menopause, anthropometric and hormonal parameters on metabolic syndrome].

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    Simoncig-Netjasov, Aleksandra; Vujović, Svetlana; Ivović, Miomira; Tancić-Gajić, Milina; Drezgić, Milka

    2010-01-01

    Hypoestrogenic status in the menopausal women shows a shift to a central android fat distribution and metabolic syndrome (MIS). Related metabolic changes and hypertension increase the risk for cardiovascular (CV) diseases. The aim of this study was to investigate the influence of duration of menopause, anthropometric and hormonal parameters on metabolic syndrome. 50 obese women were examined with BMI = 31.92 = 5.83 kg/m2, age 54.40 +/- 3.64, time since menopause 5.90 +/- 5.46 years. Control group consisted of 37 normal weight women with BMI = 23.50 +/- 2.13 kg/m2, age 53.92 +/- 3.95, time since menopause 5.96 +/- 4.92 years. Anthropometric characteristics and blood pressure were measured. Blood was taken at 8 am for: fasting glucose, triglycerides, cholesterol, HDL, LDL, apolipoprotein A (ApoA), apolipoprotein B (ApoB), lipoprotein(a) (Lp(a)), C-reactive protein (CRP), fibrinogen, FSH, LH, prolactin, estradiol, progesterone, testosterone and sex hormone binding globulin (SHBG). In obese women significant negative correlations were found for: BMI anid HDL (p menopause and waist/hip ratio (p menopause and HDL (p menopausal endocrine changes cause metabolic and hemodynamic imbalances, which contribute to risk for cardiovascular diseases.

  14. Benign Phyllodes Tumor Mimicking a Malignancy in a Turner Syndrome Woman with Hormone Replacement Therapy: A Case Report

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    Lee, Woong Jae; Chong, Se Min; Pang, Jae Choon; Seo, Jae Seung; Byun, Jun Soo; Seok, Ju Won [Chung-Ang University Medical Center, Chung-Ang University College of Medicine, Seoul (Korea, Republic of); Shin, Hee Jung; Gong, Gyung Yub [Asan Medical Center, University of Ulsan College of Mdeicine, Seoul (Korea, Republic of)

    2010-12-15

    Phyllodes tumor of the breast is a relatively rare fibroepithelial tumor. Turner syndrome is a condition that affects approximately 50 per 100,000 females and includes total or partial absence of one X chromosome in all or part of the cells, reduced final height, absence of female sex hormone, and infertility. In this case report, we describe the first case of a benign phyllodes tumor mimicking a malignancy at breast US in a 26-year-old woman with Turner syndrome who had been undergoing hormone replacement therapy

  15. Shiga toxin-associated hemolytic uremic syndrome complicated by intestinal perforation in a child with typical hemolytic uremic syndrome.

    Science.gov (United States)

    Chang, Hye Jin; Kim, Hwa Young; Choi, Jae Hong; Choi, Hyun Jin; Ko, Jae Sung; Ha, Il Soo; Cheong, Hae Il; Choi, Yong; Kang, Hee Gyung

    2014-02-01

    Hemolytic uremic syndrome (HUS) is one of the most common causes of acute renal failure in childhood and is primarily diagnosed in up to 4.5% of children who undergo chronic renal replacement therapy. Escherichia coli serotype O157:H7 is the predominant bacterial strain identified in patients with HUS; more than 100 types of Shiga toxin-producing enterohemorrhagic E. coli (EHEC) subtypes have also been isolated. The typical HUS manifestations are microangiopathic hemolytic anemia, thrombocytopenia, and renal insufficiency. In typical HUS cases, more serious EHEC manifestations include severe hemorrhagic colitis, bowel necrosis and perforation, rectal prolapse, peritonitis, and intussusceptions. Colonic perforation, which has an incidence of 1%-2%, can be a fatal complication. In this study, we report a typical Shiga toxin-associated HUS case complicated by small intestinal perforation with refractory peritonitis that was possibly because of ischemic enteritis. Although the degree of renal damage is the main concern in HUS, extrarenal complications should also be considered in severe cases, as presented in our case.

  16. Methimazole-Induced Goitrogenesis in an Adult Patient With the Syndrome of Resistance to Thyroid Hormone

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    Kathleen Glymph DO

    2014-10-01

    Full Text Available Patients with the syndrome of resistance to thyroid hormone (RTH have clinical (tachycardia and anxiety and biochemical (elevated thyroid hormones level features of hyperthyroidism. Based on previous reports in pediatric patients with the RTH, antithyroid treatment in these patients is not indicated. Clinical and biochemical sequel of antithyroid therapy in an adult patient with RTH was not previously reported. A 63-year-old African American female with history of RTH was treated with a therapy consisting of methimazole 15 mg daily and atenolol. Methimazole treatment resulted in reduction in thyroid hormone level while the patient’s TSH increased with a peak of 24.88 mIU/L. Having achieved biochemical euthyroidism, the patient developed thyroid gland enlargement associated with progressive symptoms of dysphagia and dyspnea. Examination demonstrated globally enlarged firm thyroid gland with areas of nodularity in both lobes. A computed tomography of the neck showed enlarged thyroid gland with extension around bilateral sternocleidomastoid muscles and compression onto the trachea. Methimazole therapy was discontinued and patient was treated just on atenolol. Over 12 months following discontinuation of methimazole, the patient experienced marked clinical and radiographic improvement of the goiter size associated with TSH reduction to 1.26 mIU/L and modest free thyroxine increase as expected in RTH. It seems appealing to treat patients with the RTH with antithyroid medications. However, in these patients decrease in thyroid hormone levels will stimulate TSH production, which can, in turn, predispose to goiter formation. Our report supports prior observations in children with RTH that treatment with methimazole is not indicated in adult patients with RTH.

  17. Clinical and hormonal features of women with polycystic ovary syndrome living in rural and urban areas.

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    Katulski, Krzysztof; Czyzyk, Adam; Podkowa, Natalia; Podfigurna-Stopa, Agnieszka; Ignaszak, Natalia; Paczkowska, Katarzyna; Slawek, Sylwia; Szpurek, Dariusz; Meczekalski, Blazej

    2017-09-21

    Polycystic ovary syndrome (PCOS) is one of the most common endocrinopathies among women at reproductive age, but its pathology remains unknown. From epidemiological studies it is known that endogenous, mainly genetic and exogenous, environmental factors are of importance. The aim of the study was to compare the phenotype of women diagnosed with PCOS from urban and rural areas of Poland. According to the knowledge of the authors, this is first such study. The retrospective study included 3,877 PCOS patients: 2511 women living in cities and 1,366 village inhabitants, aged between 18 - 45 years. Clinical data, including medical history, body mass, height and hirsutism severity was obtained from each patient. Hormones were also tested in each patient: follicle stimulating hormone, luteinizing hormone, prolactin, estradiol [E2], testosterone, dehydroepiandrosterone sulphate [DHEAS], thyroid stimulating hormone, free thyroxin, insulin [INS], 17 hydroxyprogesterone, cortisol [CORT]) and metabolic (75g oral glucose tolerance test, Chol - total cholesterol, HDL-C - high density lipoprotein cholesterol, LDL-C low density lipoprotein cholesterol, and the TG (triglicerides) profile. PCOS women from urban areas had a higher mean serum concentration of E2 in comparison to the inhabitants of rural areas. Women from cities had a lower mean level of DHEAS, CORT, and INS measured in the morning than rural residents. Insulin-resistance, using homeostasis model assessment, was more pronounced among women from villages. The prevalence of menstrual disorders, in general, was higher in PCOS women living in rural comparing to urban areas. The clinical and biochemical indices differed significantly between women diagnosed with PCOS living in cities and villages. In general in Poland, the PCOS phenotype is more severe in women living in rural areas. This study shows that different living conditions significantly affect the PCOS phenotype.

  18. The Correlation of Lab Data, Hormone Peptides, Quality of Life, and Different Traditional Chinese Medicine Syndrome Groups in Type 2 Diabetes Patients

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    Ching-Min Luo

    2013-04-01

    Full Text Available The aim of this study is to explore the correlation of laboratory data, hormone peptides, and quality of life with different traditional Chinese medicine (TCM syndrome groups in type 2 diabetes patients. Of 513 registered patients, 179 subjects aged between 20 and 65 years and having type 2 diabetes mellitus (T2DM for more than 1 year were enrolled in the study. All the participants were asked to fill out a questionnaire on diabetic TCM syndrome groups, which was designed by professional TCM doctors, and two questionnaires on the quality of life (QOL, WHOQOL-BREF Taiwan version and Medical Outcomes Study (MOS Short Form-12 (SF-12. The biochemical characteristics and hormone peptide levels were collected at the same time. The patients in any one of the six TCM syndrome groups had the trend to have worse QOL. Especially, patients with qi deficiency had worse life quality on every aspect compared to those without qi deficiency and were fatter than others. We also found that the subjects who had qi deficiency, qi stagnation, and yin deficiency at the same time had worsened condition. We consider that patients with qi deficiency may also be at a higher risk of developing other complications. They need more advanced health care than others. This self-reported questionnaire will be a reference for health care workers screening those T2DM patients who have a higher possibility of developing other complications. Especially in remote areas, where there is a lack of medical resources, an easy-to-use tool such as the one in the present study for detecting and evaluating disease conditions is needed.

  19. Growth hormone, gender and face shape in Prader-Willi syndrome.

    Science.gov (United States)

    de Souza, Mauren Abreu; McAllister, Catherine; Suttie, Michael; Perrotta, Concetta; Mattina, Teresa; Faravelli, Francesca; Forzano, Francesca; Holland, Anthony; Hammond, Peter

    2013-10-01

    Prader-Willi syndrome is a neurodevelopmental disorder resulting from the absence of expression of paternally expressed gene(s) in a highly imprinted region of chromosome 15q11-13. The physical phenotype includes evidence of growth retardation due to relative growth hormone deficiency, small hands and feet, a failure of normal secondary sexual development, and a facial appearance including narrow bifrontal diameter, almond-shaped palpebral fissures, narrow nasal root, and thin upper vermilion with downturned corners of the mouth. Anecdotally, the face of individuals with PWS receiving hGH treatment is said to "normalize." We used dense surface modelling and shape signature techniques to analyze 3D photogrammetric images of the faces of 72 affected and 388 unaffected individuals. We confirmed that adults with Prader-Willi syndrome who had never received human growth supplementation displayed known characteristic facial features. Facial growth was significantly reduced in these adults, especially in males. We demonstrated that following human growth hormone (hGH) supplementation, vertical facial growth of affected individuals falls within the normal range. However, lateral and periorbital face shape and nose shape differences in affected children who have received hGH therapy remain sufficiently strong to be significantly discriminating in comparisons with age-sex matched, unaffected individuals. Finally, we produced evidence that age at initiation and length of treatment with hGH do not appear to play a role in normalization or in consistent alteration of the face shape of affected individuals. This is the first study to provide objective shape analysis of craniofacial effects of hGH therapy in Prader-Willi syndrome. Copyright © 2013 Wiley Periodicals, Inc.

  20. [PRES (Posterior Reversible Encephalopathy Syndrome): potential complication of hypertensive crisis. Case report and literature review].

    Science.gov (United States)

    Vergura, Michele; Prencipe, Michele; Del Giudice, Antonio Maria; Grifa, Rachele; Miscio, Filomena; Pennelli, Anna Maria; Popolizio, Teresa; Simeone, Anna; Ferrara, Mariangela; Leone, Maurizio; Aucella, Filippo

    2017-04-01

    Posterior reversible encephalopathy syndrome (PRES) is a clinical-radiological syndrome, usually reversible, characterized by vasogenic oedema in cerebral posterior regions in patients with autoimmune diseases, nephropathies, hypertensive crisis, eclampsia and exposure to cytotoxic drugs. The main symptoms are: headache, nausea, vomiting, seizures, visual disturbance and altered consciousness. Complications as cerebral hemorrhage and recurrences are rare. We describe a case of a 65 years old woman, affected by chronic kidney disease, recently exacerbated, diabetes and hypertension in treatment, who showed an heterogeneous clinical presentation with vomiting, headache, blurred vision and impaired consciousness during an episode of acute hypertension. After an adjustement of the antihypertensive treatment we observed a regression of symptoms in one week. FLAIR sequences on MRI showed cerebral bilateral vasogenic oedema in posterior regions, typical for PRES. This case was suggestive for PRES and a prompt adjustement of the antihypertensive treatment was critical for clinical recovery. Brain MRI was crucial for diagnosis. It is important for clinicians to recognize PRES as a possible complication of renal disease and hypertensive crisis. Copyright by Società Italiana di Nefrologia SIN, Rome, Italy.

  1. Boerhaave syndrome as a complication of colonoscopy preparation: a case report

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    Winkler Michael

    2011-11-01

    Full Text Available Abstract Introduction Colonoscopy is one of the most frequently performed elective and invasive diagnostic interventions. For every colonoscopy, complete colon preparation is mandatory to provide the best possible endoluminal visibility; for example, the patient has to drink a great volume of a non-resorbable solution to flush out all feces. Despite the known possible nauseating side effects of colonoscopy preparation and despite the knowledge that excessive vomiting can cause rupture of the distal esophagus (Boerhaave syndrome, which is a rare but severe complication with high morbidity and mortality, it is not yet a standard procedure to provide a patient with an anti-emetic medication during a colon preparation process. This is the first report of Boerhaave syndrome induced by colonoscopy preparation, and this case strongly suggests that the prospect of being at risk of a severe complication connected with an elective colonoscopy justifies a non-invasive, inexpensive yet effective precaution such as an anti-emetic co-medication during the colonoscopy preparation process. Case presentation A 73-year-old Caucasian woman was scheduled to undergo elective colonoscopy. For the colonoscopy preparation at home she received commercially available bags containing soluble polyethylene glycol powder. No anti-emetic medication was prescribed. After drinking the prepared solution she had to vomit excessively and experienced a sudden and intense pain in her back. An immediate computed tomography (CT scan revealed a rupture of the distal esophagus (Boerhaave syndrome. After initial conservative treatment by endoluminal sponge vacuum therapy, she was taken to the operating theatre and the longitudinal esophageal rupture was closed by direct suture and gastric fundoplication (Nissen procedure. She recovered completely and was discharged three weeks after the initial event. Conclusions To the best of our knowledge, this is the first report of a case of

  2. Detrimental Complications Of Meconium Aspiration Syndrome And Their Impact On Outcome.

    Science.gov (United States)

    Shaikh, Mehmood; Irfan Waheed, Khawaja Ahmad; Javaid, Sulman; Gul, Rafia; Hashmi, Muhammad Almas; Fatima, Syeda Tehseen

    2016-01-01

    Meconium aspiration syndrome (MAS) is respiratory distress in an infant born through meconium stained amniotic fluid (MSAF) whose signs cannot be otherwise explained. MAS is associated with different complications. Many studies have been conducted in developed world to find the outcome associated with MAS but data from developing countries is lacking. Present study was conducted to determine the impact of chemical pneumonitis, pulmonary hypertension (PHN) and air leak on outcome of new-borns with MAS. This cross sectional descriptive study was conducted in a tertiary care Neonatology unit. The babies diagnosed with MAS were included in the study. All patients were monitored for development of complications. Outcome in terms of mortality was recorded. Outcome was compared by chisquare test and p-value Mean gestation was 37±0.56 weeks and birth weight was 2.87±0.49 kg. Male to female ratio was 1.57:1 and mean age of admission was 9.59±5.27 hours. Chemical pneumonitis, PHN and chemical pneumonitis with PHN were observed in 23.6%, 20.8% and 26.4% neonates respectively. Mortality rate was 19.44%. The mortality was highest in babies having chemical pneumonitis with PHN (p-value=0.013) followed by chemical pneumonitis group (p-value=0.02) and PHN group (p-value=0.032). About three fourth babies with MAS developed one or more complications. Mortality is significantly increases with the development of complications so as more the complications more is the mortality.

  3. Hypothalamic-pituitary-gonadal axis hormones and cortisol in both menstrual phases of women with chronic fatigue syndrome and effect of depressive mood on these hormones

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    Nas Kemal

    2004-12-01

    Full Text Available Abstract Background Chronic fatigue syndrome (CFS is a disease which defined as medically unexplained, disabling fatigue of 6 months or more duration and often accompanied by several of a long list of physical complaints. We aimed to investigate abnormalities of hypothalamic-pituitary-gonadal (HPG axis hormones and cortisol concentrations in premenopausal women with CFS and find out effects of depression rate on these hormones. Methods We examined follicle stimulating hormone (FSH, luteinizing hormone (LH, estradiol, progesterone and cortisol concentrations in 43 premenopausal women (mean age: 32.86 ± 7.11 with CFS and compared matched 35 healthy controls (mean age: 31.14 ± 6.19. Patients were divided according to menstrual cycle phases (follicular and luteal and compared with matched phase controls. Depression rate was assessed by Beck Depression Inventory (BDI, and patients with high BDI scores were compared to patients with low BDI scores. Results There were no significant differences in FSH, LH, estradiol and progesterone levels in both of menstrual phases of patients versus controls. Cortisol levels were significantly lower in patients compared to controls. There were no significant differences in all hormone levels in patients with high depression scores versus patients with low depression scores. Conclusion In spite of high depression rate, low cortisol concentration and normal HPG axis hormones of both menstrual phases are detected in premenopausal women with CFS. There is no differentiation between patients with high and low depression rate in all hormone levels. Depression condition of CFS may be different from classical depression and evaluation of HPG and HPA axis should be performed for understanding of pathophysiology of CFS and planning of treatment.

  4. [Floating-Harbor syndrome: first case in Italy associated with growth hormone deficiency].

    Science.gov (United States)

    Femiano, P; Castaldo, V; Scarano, G

    2000-04-01

    The Floating-Harbor Syndrome (FHS) is a rare entity characterised by a clinical triad: facial dysmorphism, retarded speech development and short stature with delayed bone age. The case of a 9-year and 8/12 months old boy showing the typical features of this syndrome associated to a severe GH deficiency is reported. At diagnosis of FHS, the weight was 16 kg ( 90 degrees percentile). The evaluation of growth hormone secretion by two Insulin Tolerance Tests confirmed an insufficient GH peaks response (2.7 ng/ml and 4.6 ng/ml). He had a slight mental delay (IQ: 60) involving language skills. After 1 year of treatment with r-hGH (0.6 IU/kg/week), an increase in growth velocity (11 cm/year) in the length (123 cm), and in body weight (20.5) has been observed. This case is the 22nd report with this syndrome and the first in Italy. The association between GH deficiency and FHS, and the beneficial effects of a substitutive treatment are described.

  5. The association of metabolic syndrome with adipose tissue hormones and insulin resistance in patients with COPD without co-morbidities.

    Science.gov (United States)

    Minas, Markos; Kostikas, Konstantinos; Papaioannou, Andriana I; Mystridou, Parthena; Karetsi, Eleni; Georgoulias, Panagiotis; Liakos, Nikolaos; Pournaras, Spyros; Gourgoulianis, Konstantinos I

    2011-12-01

    Chronic obstructive pulmonary disease (COPD) and metabolic syndrome represent common causes of morbidity and mortality in ageing populations. The effect of the co-existence of COPD and metabolic syndrome on adipose tissue hormones and insulin resistance as well as the differences between COPD patients with and without metabolic syndrome have not been adequately studied. The prevalence of metabolic syndrome, based on Adult Treatment Panel III (ATP III) criteria, was evaluated in 114 male patients with COPD without significant co-morbidities. Pulmonary functions tests (PFTs), arterial blood gases, quality of life and BODE index were assessed. Blood samples were obtained for the assessment of adipose tissue hormones and insulin resistance. The overall prevalence of metabolic syndrome was 21%, being more prevalent in earlier stages of COPD. Patients with COPD and metabolic syndrome were younger with higher body-mass index (BMI), had better pulmonary function, less static hyperinflation and air-trapping, better diffusing capacity for carbon monoxide and BODE index. These patients had higher levels of leptin, lower levels of adiponectin and increased insulin resistance, as expressed by HOMA index, compared with patients without metabolic syndrome. Metabolic syndrome was more prevalent in younger patients with less severe COPD. These patients may constitute a specific COPD phenotype with greater leptin to adiponectin imbalance and insulin resistance, despite smaller impairment in PFTs. The prognosis and differences of these patients compared with other COPD phenotypes needs to be determined in prospective studies.

  6. Moyamoya Disease in an 18-Month-Old Female Caucasian Complicated by Cerebral Hyperperfusion Syndrome Following Indirect Revascularization.

    Science.gov (United States)

    Murchison, James; Wilson, John M; Ray, Coby; Ginsberg, Jessica; Nagy, Laszlo

    2017-10-09

    BACKGROUND Cerebral hyperperfusion syndrome is a rare complication of indirect revascularization due to moyamoya disease, but has not been reported previously in the pediatric population. We present a case of an 18-month-old girl with moyamoya disease that was treated with bilateral pial synangiosis and had complications consistent with cerebral hyperperfusion syndrome. This case report discusses the pathophysiological mechanisms involved in cerebral hyperperfusion in moyamoya syndrome. CASE REPORT An 18-month-old female Caucasian presented with seizures and weakness of the left side. Angiography confirmed bilateral cerebral moyamoya disease that was worse on the right side. Indirect revascularization with pial synangiosis was first performed on the right side to allow for healing. Five months later, pial synangiosis was then performed on the left side. Postoperatively, the patient experienced increased intracranial pressure (ICP), suggesting cerebral hyperperfusion syndrome. She was treated with a repeat lumbar puncture, a lumbar drain, and a lumbar shunt. CONCLUSIONS This report demonstrates a case of cerebral hyperperfusion syndrome as a complication of moyamoya disease in a pediatric patient. Although the patient progressed well after placement of a lumbar shunt, this case demonstrates the occurrence of cerebral hyperperfusion syndrome as a complication of revascularization in pediatric patients and highlights the need for further research in this area.

  7. Early dumping syndrome is not a complication but a desirable feature of Roux-en-Y gastric bypass surgery.

    Science.gov (United States)

    Laurenius, A; Engström, M

    2016-10-01

    Early dumping syndrome after gastric bypass surgery due to rapid delivery of hyperosmolar nutrients into the bowel causing intense symptoms is often described as a complication. Twelve patients, mean age 47 years, were interviewed approximately 9 years post-operation. The interviews were audiotaped and transcribed verbatim, followed by an inductive content analysis to reveal patients' experience of the dumping syndrome. The core category 'Dumping syndrome is a positive consequence of Roux-en-Y gastric bypass surgery and a tool to control food intake' was identified based on the following four sub-categories: (i) 'The multidimensional emergence and effects of dumping syndrome', (ii) 'Dumping syndrome as something positive although unpleasant', (iii) 'Developing coping mechanisms and ingenious strategies' and (iv) 'My own fault if I expose myself to dumping syndrome'. From the patients' perspective, dumping syndrome gives control over food intake; although the symptoms were unpleasant, patients considered dumping syndrome as a positive protection against over-consumption. Hence, healthcare professionals should not present dumping syndrome as a complication but rather as an aid to control eating behaviour and excessive food intake. © 2016 World Obesity.

  8. Coronary spasm secondary to cefuroxime injection, complicated with cardiogenic shock - a manifestation of Kounis syndrome: case report and literature review.

    Science.gov (United States)

    Mitsis, Andreas; Christodoulou, Evi; Georgiou, Panayiota

    2017-03-01

    Kounis syndrome is defined as the coincidental occurrence of an acute coronary syndrome with hypersensitivity reactions following an allergic event. The three reported variants of Kounis syndrome are vasospastic allergic angina, allergic myocardial infarction and stent thrombosis with occluding thrombus. The syndrome is caused by various inflammatory mediators. The pathophysiological characteristics of Kounis syndrome involve coronary artery spasm and/or atheromatous plaque erosion or rupture during an allergic reaction. Several causes have been described to induce Kounis syndrome, and their number is increasing rapidly. The haemodynamic effect of the syndrome complicated by cardiogenic shock seems to combine allergic shock with extensive peripheral vasodilation and myocardial suppression with the characteristics of cardiogenic shock. Treatment of Kounis syndrome is challenging because it needs management of both cardiac and allergic manifestation simultaneously. We present a case report of type I Kounis syndrome, with coronary spasm secondary to cefuroxime injection complicated with cardiogenic shock. A brief review of the literature on the various facets of this condition is also provided.

  9. Four Cases of Atopic Dermatitis Complicated by Sjogren's Syndrome: Link between Dry Skin and Autoimmune Anhidrosis

    Directory of Open Access Journals (Sweden)

    Shun Kitaba

    2011-01-01

    Full Text Available We report four adult cases of atopic dermatitis (AD complicated by Sjogren's syndrome (SS. The patients fulfilled diagnostic criteria for AD and SS. All cases showed persistent itchy dry skin and eczematous lesions complicated by sicca symptoms including dry eyes and dry mouth with moderate joint pain. One case manifested annular erythema and another manifested widespread discoid erythema. To investigate the underlying cause of dry skin in these cases, sweating function was evaluated using a quantitative sudomotor axon reflex test (QSART in which the axon reflex is stimulated by acetylcholine iontophoresis. The sweating latency time was significantly prolonged in eczematous skin of AD and AD/SS compared to normal controls. Axon reflex (AXR sweat volume was also significantly reduced in AD (normal and eczematous skin and AD/SS (normal and eczema compared to normal control. In contrast, the direct sweat volume of lesional or non-lesional AD skin induced by direct stimulation with acetylcholine was only slightly reduced compared to that in normal controls, but not in SS and lesional skin of AD/SS patients. These results suggest that the impaired sweat response in AD is attributable to an abnormal sudomotor axon reflex, which is accelerated and modulated when complicated by SS resulting in dry skin in the present cases.

  10. The effect of oxandrolone on body proportions and body composition in growth hormone-treated girls with Turner syndrome.

    NARCIS (Netherlands)

    Menke, L.A.; Sas, T.C.J.; Zandwijken, G.R.; Ridder, M.A. de; Stijnen, T.; Muinck Keizer-Schrama, S.M.P.F. de; Otten, B.J.; Wit, J.M.

    2010-01-01

    OBJECTIVE: Untreated girls with Turner syndrome (TS) have short stature, relatively broad shoulders, a broad pelvis, short legs, a high fat mass and low muscle mass. Our objective was to assess the effect of the weak androgen oxandrolone (Ox) on body proportions and composition in growth hormone

  11. Growth hormone treatment for Turner syndrome in Australia reveals that younger age and increased dose interact to improve response

    NARCIS (Netherlands)

    Hughes, Ian P.; Choong, Catherine S.; Harris, Mark; Ambler, Geoffrey R.; Cutfield, Wayne S.; Hofman, Paul L.; Cowell, Chris T.; Werther, George; Cotterill, Andrew; Davies, Peter S. W.

    P>Objective To investigate response to growth hormone (GH) in the first, second and third years of treatment in the total clinical cohort of Turner syndrome (TS) patients in Australia. Context Short stature is the most common clinical manifestation of TS. GH treatment improves growth. Design

  12. Case Report: A Rare Cause of Complicated Urinary Tract Infection in a Woman with Herlyn-Werner-Wunderlich Syndrome.

    Science.gov (United States)

    Tsai, Jun-Li; Tsai, Shang-Feng

    2016-11-01

    Urinary tract infection is a common disease in the general population. However, in patients with frequent urinary tract infection, it is important to determine any treatable cause to avoid recurrence. Herlyn-Werner-Wunderlich syndrome or OHVIRA syndrome is a very rare congenital anomaly with uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. The earliest presentation of this syndrome is hematocolpos that develops during menstruation and results in dysmenorrhea and a pelvic mass shortly after menarche. Herein, we report a patient with Herlyn-Werner-Wunderlich syndrome manifested with unusual symptoms, delayed onset and without surgery. The unique point of this patient is the partial obstruction of cervico-vaginal junction. Early diagnosis and timely treatment of OHVIRA syndrome can prevent long-term complications, such as recurrent urinary tract infection and infertility. A high index of suspicion is required, even though OHVIRA syndrome is extremely rare and may have an atypical presentation.

  13. Methionyl human growth hormone and oxandrolone in Turner syndrome: preliminary results of a prospective randomized trial.

    Science.gov (United States)

    Rosenfeld, R G; Hintz, R L; Johanson, A J; Brasel, J A; Burstein, S; Chernausek, S D; Clabots, T; Frane, J; Gotlin, R W; Kuntze, J

    1986-12-01

    Seventy girls with Turner syndrome, 4 to 12 years of age, were randomly assigned to receive either no treatment (control) or methionyl human growth hormone (0.125 mg/kg three times per week), oxandrolone (0.125 mg/kg/day), or combination hGH plus oxandrolone therapy. Baseline growth rates averaged 4.3 cm/yr, and all were within 2 SD of mean growth velocity for age in girls with Turner syndrome. Sixty-seven girls remained in the study for a minimum of 1 year. Growth rates and growth velocity (in standard deviations for age in girls with Turner syndrome) were control 3.8 cm/yr (-0.1 SD), hGH 6.6 cm/yr (+2.3 SD), oxandrolone 7.9 cm/yr (+3.7 SD), and combination therapy 9.8 cm/yr (+5.4 SD). Mean bone ages advanced 1.0 years (hGH), 1.3 years (oxandrolone), and 1.6 years (combination). However, median increments in height age/bone age (delta HA/delta BA) ratios ranged from 1.0 to 1.1 for treatment groups, compared with 0.8 for the controls. Predicted adult height by the method of Bayley-Pinneau increased 2.5 cm for hGH or oxandrolone alone, and 3.2 cm for combination treatment. These data indicate that both hGH and oxandrolone can significantly stimulate short-term skeletal growth in patients with Turner syndrome, and potentially increase final adult height.

  14. A harmful traditional practice in newborns with adrenocorticotropic hormone resistance syndrome: branding.

    Science.gov (United States)

    Baştuğ, Osman; Korkmaz, Levent; Korkut, Sabriye; Halis, Hülya; Güneş, Tamer; Kurtoğlu, Selim

    2016-12-01

    Branding refers to a traditional practice of creating 'therapeutic' burns with hot iron rods over the skin in order to treat various diseases. Although branding is a harmful practice for the body, it has been used for various illnesses including physiologic jaundice in newborns, pneumonia, and convulsions. It causes serious morbidity and delays seeking proper medical care in neonates. Innovations of modern medicine and the use of evidence-based medicine should be preferred instead of these traditional practices. We present a branded mature newborn baby who was diagnosed as having adrenocorticotropic hormone resistance syndrome. This problem is very rare in Turkey; however, it is a very important health issue and has social aspects. Therefore, this case is presented to increase awareness.

  15. The syndrome of inappropriate antidiuretic hormone: current and future management options.

    LENUS (Irish Health Repository)

    Sherlock, Mark

    2010-06-01

    Hyponatraemia is the commonest electrolyte abnormality, and syndrome of inappropriate antidiuretic hormone (SIADH) is the most frequent underlying pathophysiology. Hyponatraemia is associated with significant morbidity and mortality, and as such appropriate treatment is essential. Treatment options for SIADH include fluid restriction, demeclocycline, urea, frusemide and saline infusion, all of which have their limitations. The introduction of the vasopressin-2 receptor antagonists has allowed clinicians to specifically target the underlying pathophysiology of SIADH. Initial studies have shown good efficacy and safety profiles in the treatment of mild to moderate hyponatraemia. However, studies assessing the efficacy and safety of these agents in acute severe symptomatic hyponatraemia are awaited. Furthermore, the cost of these agents at present may limit their use.

  16. Persistent hiccups in cancer patient: A presentation of syndrome of inappropriate antidiuretic hormone induced hyponatremia

    Directory of Open Access Journals (Sweden)

    Alka Goyal

    2013-01-01

    Full Text Available Hyponatremia is quite common in cancer patients, but the presentation as persistent hiccups is not common. Literature over hiccups development due to hyponatremia is quite scant. Hiccups are of various types, persistent hiccups are those that last more than 48 h and remains less than 1 month. Hiccups lasting more than 24 h require investigation for an underlying organic etiology, with hyponatremia included in the differential diagnosis. This paper discusses a carcinoma lip patient presented with the persistent hiccups and unconsciousness post-operatively. The patient was initially responded with trials of both metoclopramide and Ryle′s tube insertion, but eventually, his hiccups resolved only after treatment of hyponatremia. Patient′s clinical course and investigations suggest an etiology of syndrome of inappropriate antidiuretic hormone (SIADH secretion behind the hyponatremia. Study suggested that SIADH linked hyponatremia should be considered in the differential diagnosis of cancer patients with refractory hiccups.

  17. Syndrome of Inappropriate Antidiuretic Hormone Secretion Due to Amantadine: 3 Cases in the Literature

    Directory of Open Access Journals (Sweden)

    Akif Acay

    2014-02-01

    Full Text Available To report a patient who developed a syndrome of inappropriate antidiuretic hormone secretion (SIADH related with amantadine. A 62 year-old male, who has been followed for Parkinson%u2019s disease for 16 years. Three months ago, amantadine added to his existing treatment. About 4 weeks after the initiation of treatment, confusion, constant sleepiness, lethargy, asthenia, fatigue and muscle weakness appeared. The blood anlysis showed hyponatremia, so he was diagnosed as SIADH and amantadine treatment was stopped. Then his symptoms gradually improved and the sodium levels was turned into the normal ranges. Amantadine may cause SIADH should be used with caution in patients with Parkinson%u2019s disease.

  18. Low-dose growth hormone and human immunodeficiency virus-associated lipodystrophy syndrome: a pilot study

    DEFF Research Database (Denmark)

    Andersen, O; Haugaard, S B; Flyvbjerg, A

    2004-01-01

    BACKGROUND: Treatment with high doses (2-6 mg day(-1)) of human growth hormone (hGH) in patients with human immunodeficiency virus (HIV)-associated lipodystrophy syndrome (HALS) has been shown to increase concentrations of total insulin-like growth-factor-I (IGF-I) more than twofold greater than...... the normal upper range and is accompanied by adverse effects such as joint pain and glucose intolerance. MATERIALS AND METHODS: We performed a 16-week open-labelled prospective pilot study in six male HALS patients using a s.c. low-dose hGH, 0.7 mg day(-1), aiming to examine the impact on total and free IGF...

  19. Vancomycin intoxication in a patient with inappropriate antidiuretic hormone syndrome and diarrhea

    Directory of Open Access Journals (Sweden)

    Patricia Hidalgo-Collazos

    2015-07-01

    Full Text Available Vancomycin is an antibiotic used for infections by gram-positive bacteria with a two-compartment pharmacokinetic model. Its monitoring has an established therapeutic range (10-20 mg/L to prevent nephrotoxicity and ototoxicity due to supratherapeutic levels, and inefficiency and development of resistance by subtherapeutic levels. Nephrotoxicity for vancomycin monotherapy at standard doses according to pathogen and typical regimens (usual dose: 15-20 mg/kg/12 h is rare and usually reversible. Moreover, monitoring plasma concentrations allows to achieve concentrations within therapeutic range to allow safe and effective drug use. The renal hypoperfusion can cause pre-renal damage, resulting in elevated levels of serum creatinine, resulting in decreased antibiotic elimination and nephrotoxicity. We report a case of unexpected vancomycin nephrotoxicity in a patient with syndrome Inappropriate antidiuretic hormone secretion associated paraneoplastic

  20. Syndrome of Inappropriate Antidiuretic Hormone Secretion (SIADH in Strongyloides stercoralis hyperinfection

    Directory of Open Access Journals (Sweden)

    Deepshikha Nag Chowdhury

    2014-01-01

    Full Text Available Strongyloides stercoralis (S. stercoralis is a soil transmitted intestinal roundworm that has a unique ability to multiply within the human host and reinfect the human carrier by a process of autoinfection. By this property, S. stercoralis can persist as an occult infection for many decades. In situations of immunosuppression or other permissive gastrointestinal conditions, there occurs a massive increase in parasite multiplication. The parasites penetrate through the intestinal mucosa and are carried in circulation and can cause multisystem involvement. We report a case of a 76-year-old Columbian male who presented with intractable vomiting and hyponatremia who was then diagnosed to have syndrome of inappropriate antidiuretic hormone (SIADH. The patient′s symptoms improved after treatment with two doses of ivermectin and his serum sodium levels returned to normal. S. stercoralis infection should be suspected in patients from endemic regions who present with gastrointestinal symptoms and unexplained hyponatremia.

  1. Abdominal compartment syndrome – the prevention and treatment of possible lethal complications following hip arthroscopy: a case report

    Science.gov (United States)

    2014-01-01

    Introduction Intra-abdominal hypertension and abdominal compartment syndrome have been increasingly recognized as a hip arthroscopy complication over the past decade. In the absence of consensus definitions and treatment guidelines, the diagnosis and management of intra-abdominal hypertension and abdominal compartment syndrome remains variable from institution to institution. Case presentation We report the occurrence of the extravasation of fluid into the abdomen during arthroscopic treatment of femoroacetabular impingement combined with resection of trochanteric bursa and our management of the condition in a 55-year old Caucasian woman. Conclusions We present an algorithm of treatment of abdominal compartment syndrome, as a hip arthroscopy complication, according to the consensus definitions and recommendations of the World Society of the Abdominal Compartment Syndrome. In the algorithm options, we have included paracentesis and percutaneous catheter decompression as the main point of treatment. Our algorithm will have a broader clinical impact on orthopedic surgery, anesthesiology and emergency medicine. PMID:25394557

  2. Abdominal compartment syndrome--the prevention and treatment of possible lethal complications following hip arthroscopy: a case report.

    Science.gov (United States)

    Ciemniewska-Gorzela, Kinga; Piontek, Tomasz; Szulc, Andrzej

    2014-11-14

    Intra-abdominal hypertension and abdominal compartment syndrome have been increasingly recognized as a hip arthroscopy complication over the past decade. In the absence of consensus definitions and treatment guidelines, the diagnosis and management of intra-abdominal hypertension and abdominal compartment syndrome remains variable from institution to institution. We report the occurrence of the extravasation of fluid into the abdomen during arthroscopic treatment of femoroacetabular impingement combined with resection of trochanteric bursa and our management of the condition in a 55-year old Caucasian woman. We present an algorithm of treatment of abdominal compartment syndrome, as a hip arthroscopy complication, according to the consensus definitions and recommendations of the World Society of the Abdominal Compartment Syndrome. In the algorithm options, we have included paracentesis and percutaneous catheter decompression as the main point of treatment. Our algorithm will have a broader clinical impact on orthopedic surgery, anesthesiology and emergency medicine.

  3. Association of the luteinizing hormone/choriogonadotropin receptor gene polymorphism with polycystic ovary syndrome.

    Science.gov (United States)

    Bassiouny, Yasmin Ahmed; Rabie, Walaa Ahmed; Hassan, Ayman Ahmed; Darwish, Rania Kamal

    2014-06-01

    This study aimed at evaluating possible associations of the single nucleotide polymorphism (SNP) in luteinizing hormone/choriogonadotropin receptor (LHCGR) gene G935A and polycystic ovary syndrome (PCOS) phenotype. The study included 100 PCOS female patients and 60 healthy female control subjects. The patients were recruited from the Gynecology out-patient clinic, Kasr Al-Aini Hospital, Cairo University. All candidates underwent full history taking and clinical examination with calculation of body mass index. Serum and EDTA samples were collected from each patient after a written consent. A hormonal profile was done for each patient as well as DNA analysis of the G935A polymorphism of LHCGR gene. In PCOS group, 26% were homozygous (AA), 27% were heterozygous (GA) and 47% were wild genotype (GG), while in controls 30% were heterozygous and 70% were wild genotype (OR: 2.25; CI: 1.16-4.386; p value: 0.012). The homozygous 935A individuals were at higher risk to develop PCOS than controls (OR: 1.80; CI: 1.54-2.09; p value genetic variant, which is associated with PCOS in a sample of the Egyptian population. These results may provide an opportunity to test this SNP at the LHCGR gene in fertile or infertile women with family history to assess their risk of PCOS.

  4. Syndrome of inappropriate antidiuretic hormone secretion induced by the phytotherapy Harpagophytum procumbers: case report

    Directory of Open Access Journals (Sweden)

    Renata Reis Carvalho

    Full Text Available Abstract Introduction: The syndrome of inappropriate antidiuretic hormone secretion (SIADH is the inability of antidiuretic hormone (ADH suppression, compromising the mechanisms of water excretion and urinary concentration. It manifests as hyponatremia and its symptoms, especially neurological. There are many causes that trigger such disease, notably: central nervous system disorders, malignant neoplasm, drugs and others. Case Report: A 65 years female hypertensive patient presented clinical and laboratory manifestations of hyponatremia due to SIADH. It happened twice under use of herbal medication for osteoarthritis treatment. Discussion: The drug-related hyponatremia can be triggered by direct effect of the drug or by association with SIADH. The clinical manifestations presented could have been related to psychiatric condition and may have severe outcome if not properly diagnosed. The association of an herbal medicine to SIADH could be confirmed after a new episode of hyponatremia related to Harpagophytum procumbers reintroduction. Our literature review did not find this herbal medicine associated with SIADH, so far. Conclusion: SIADH may be caused by herbal medicine described from now on their association in the literature.

  5. Syndrome of inappropriate antidiuretic hormone secretion induced by the phytotherapy Harpagophytum procumbers: case report.

    Science.gov (United States)

    Carvalho, Renata Reis; Donadel, Camila Dermínio; Cortez, Arthur Fernandes; Valviesse, Vitor Ribeiro Gomes de Almeida; Vianna, Pedro Felipe de Almeida; Corrêa, Bruno Bordallo

    2017-03-01

    The syndrome of inappropriate antidiuretic hormone secretion (SIADH) is the inability of antidiuretic hormone (ADH) suppression, compromising the mechanisms of water excretion and urinary concentration. It manifests as hyponatremia and its symptoms, especially neurological. There are many causes that trigger such disease, notably: central nervous system disorders, malignant neoplasm, drugs and others. A 65 years female hypertensive patient presented clinical and laboratory manifestations of hyponatremia due to SIADH. It happened twice under use of herbal medication for osteoarthritis treatment. The drug-related hyponatremia can be triggered by direct effect of the drug or by association with SIADH. The clinical manifestations presented could have been related to psychiatric condition and may have severe outcome if not properly diagnosed. The association of an herbal medicine to SIADH could be confirmed after a new episode of hyponatremia related to Harpagophytum procumbers reintroduction. Our literature review did not find this herbal medicine associated with SIADH, so far. SIADH may be caused by herbal medicine described from now on their association in the literature.

  6. Levels of endocrine hormones and lipids in male patients with carpal tunnel syndrome

    Directory of Open Access Journals (Sweden)

    Hülya Uzkeser

    2011-12-01

    Full Text Available Objectives: This study was performed to evaluate the relationship between endocrine hormones, lipid levels and clinical parameters in male patients with carpal tunnel syndrome (CTS.Materials and methods: Fifteen male patients with CTS and 16 healthy controls were included in the study. Serum free T3, free T4, thyroid-stimulating hormone (TSH, free testosterone, dehydroepiandrosterone sulfate, triglyceride and total cholesterol levels were analyzed. Symptom severity and hand function were assessed using the Boston Carpal Tunnel Questionnaire in clinical examination.Results: Serum free T3, free T4, TSH, free testosterone, dehydroepiandrosterone sulfate, triglyceride and total cholesterol levels were similar between CTS patients and controls (p> 0.05. Also, there was no statistically significant correlation between laboratory parameters and clinical characteristics in patients with CTS (p> 0.05.Conclusion: The serum free T3, free T4, TSH, free testosterone, dehydroepiandrosterone sulfate, triglyceride and total cholesterol levels seem within normal range in male CTS patients. Further studies are needed to investigate association endocrine factors, lipid levels such as triglyceride and total cholesterol with CTS in male and female patients.

  7. Bronchiolitis obliterans complicating a pneumothorax after Stevens-Johnson syndrome induced by lamotrigine

    Directory of Open Access Journals (Sweden)

    Wu-ping Wang

    2015-03-01

    Full Text Available Bronchiolitis obliterans (BO was defined as a nonreversible obstructive lung disease in which the bronchioles are always compressed and narrowed by fibrosis or inflammation. In the severe event of lung collapse after BO, surgical intervention is often recommended, and conservative therapy is thought to be ineffective. Here, we report the case of a 9-year old girl clinically diagnosed as having bronchiolitis obliterans with abrupt occlusion of the right B4b bronchus. After a lamotrigine-induced Stevens-Johnson syndrome (SJS occurred, she presented with total collapse of the right lung on admission, which was subsequently complicated by a pneumothorax during conservative treatment, but with the re-expansion of the right upper lobe after intervention. The case indicates the possibility of reversing pulmonary atelectasis in BO. Thus, surgery may not be necessary.

  8. Bronchiolitis obliterans complicating a pneumothorax after Stevens-Johnson syndrome induced by lamotrigine.

    Science.gov (United States)

    Wang, Wu-ping; Ni, Yun-feng; Wei, Yao-ning; Li, Xiao-fei; Cheng, Qing-shu; Lu, Qiang

    2015-03-01

    Bronchiolitis obliterans (BO) was defined as a nonreversible obstructive lung disease in which the bronchioles are always compressed and narrowed by fibrosis or inflammation. In the severe event of lung collapse after BO, surgical intervention is often recommended, and conservative therapy is thought to be ineffective. Here, we report the case of a 9-year old girl clinically diagnosed as having bronchiolitis obliterans with abrupt occlusion of the right B4b bronchus. After a lamotrigine-induced Stevens-Johnson syndrome (SJS) occurred, she presented with total collapse of the right lung on admission, which was subsequently complicated by a pneumothorax during conservative treatment, but with the re-expansion of the right upper lobe after intervention. The case indicates the possibility of reversing pulmonary atelectasis in BO. Thus, surgery may not be necessary. Copyright © 2012. Published by Elsevier B.V.

  9. Budd-Chiari syndrome: a rare and life-threatening complication of Crohn’s disease

    Science.gov (United States)

    Simoes, Camila C; Ghouri, Yezaz A; Merwat, Shehzad N

    2018-01-01

    Budd-Chiari syndrome (BCS) is characterised by obstruction of hepatic venous outflow and may be triggered by the prothrombotic state associated with inflammatory bowel disease (IBD). We reported a case of Crohn’s disease (CD) that presented with anasarca, ascites, symptomatic hepatomegaly, elevated liver enzymes, increased prothrombin time and low albumin. Oesophagogastroduodenoscopy and colonoscopy confirmed active CD. Abdominal CT showed hepatic vein thrombosis. Liver biopsy revealed severe perivenular sinusoidal dilation with areas of hepatocyte dropout, bands of hepatocyte atrophy and centrizonal fibrosis, suggestive of BCS. The patient was treated with steroids for CD and systemic anticoagulants for BCS. His liver function and enzymes normalised, and he reported symptomatic improvement. The precise mechanism responsible for increased hypercoagulability in IBD remains unclear. Early recognition and treatment for possible thrombotic complications of CD is critical to prevent potentially fatal events like pulmonary embolism or liver failure. PMID:29348288

  10. Chronic Disseminated Candidiasis Complicated by Immune Reconstitution Inflammatory Syndrome in Child with Acute Lymphoblastic Leukemia

    Directory of Open Access Journals (Sweden)

    Olga Zając-Spychała

    2016-01-01

    Full Text Available Hepatosplenic candidiasis also known as chronic disseminated candidiasis is a rare manifestation of invasive fungal infection typically observed in patients with acute leukemia in prolonged, deep neutropenia. Immune reconstitution inflammatory syndrome (IRIS is an inflammatory disorder triggered by rapid resolution of neutropenia. Diagnosis and treatment of IRIS are still challenging due to a variety of clinical symptoms, lack of certain diagnostic criteria, and no standards of treatment. The diagnosis of IRIS is even more difficult in patients with hematological malignancies complicated by “probable” invasive fungal infection, when fungal pathogen is still uncertain. We report a case of probable hepatic candidiasis in 4-year-old boy with acute lymphoblastic leukemia. Despite proper antifungal therapy, there was no clinical and radiological improvement, so diagnosis of Candida-related IRIS was made and corticosteroid therapy was added to antifungal treatment achieving prompt resolution of infection symptoms.

  11. Herlyn-Werner-Wunderlich Syndrome Complicated with Pyocolpos: An Unusual Cause of Postabortal Sepsis.

    Science.gov (United States)

    Sharma, Deepti; Janu, M K; Gaikwad, Ramesh; Usha, M G

    2011-01-01

    Obstructive mullerian anomalies give rise to a spectrum of clinical presentations and are uncommon in routine gynecologic practice. The patient usually becomes symptomatic in early reproductive years. Recurrent pelvic pain, dysmenorrhea, enlarging abdominopelvic mass, and abnormal vaginal discharge are the common presenting symptoms. We describe a rare case of a mullerian anomaly getting diagnosed 13 years after attaining menarche during the evaluation of postabortal sepsis. Patient presented 2 weeks following evacuation carried out for missed abortion, with acute abdominal pain, fever and foul smelling discharge per vaginum. The anomaly was identified as uterus didelphys with obstructed left hemivagina and ipsilateral renal agenesis (Herlyn-Werner-Wunderlich syndrome) complicated by pyocolpos. She was successfully managed by single-stage transvaginal septum resection under laparoscopic control.

  12. Abdominal compartment syndrome – the prevention and treatment of possible lethal complications following hip arthroscopy: a case report

    OpenAIRE

    Ciemniewska-Gorzela, Kinga; Piontek, Tomasz; Szulc, Andrzej

    2014-01-01

    Introduction Intra-abdominal hypertension and abdominal compartment syndrome have been increasingly recognized as a hip arthroscopy complication over the past decade. In the absence of consensus definitions and treatment guidelines, the diagnosis and management of intra-abdominal hypertension and abdominal compartment syndrome remains variable from institution to institution. Case presentation We report the occurrence of the extravasation of fluid into the abdomen during arthroscopic treatmen...

  13. Thyroid hormone resistance syndrome caused by heterozygous A317T mutation in thyroid hormone receptor β gene: Report of one Chinese pedigree and review of the literature.

    Science.gov (United States)

    Guo, Qing-Hua; Wang, Bao-An; Wang, Chen-Zhi; Wang, Min; Lu, Ju-Ming; Lv, Zhao-Hui; Mu, Yi-Ming

    2016-08-01

    Thyroid hormone resistance syndrome (THRS) is a rare disorder with increased concentrations of free thyroxine (FT4) and triiodothyronine (FT3), but normal or slightly increased thyroid-stimulating hormone (TSH). The mutations in the thyroid hormone receptor β (THRβ) gene are thought to be the main pathogenesis. The aims of this study were to present 1 pedigree of Chinese THRS, summarize their clinical characteristics, and analyze the gene mutation. The clinical characteristics and thyroid function of the proband and his family members were collected. Gene mutations were analyzed by DNA sequencing. The proband and his mother exhibited symptoms of hyperthyroidism, such as palpitations, heat intolerance, and perspiration. The mother also had atrial fibrillation. The rest of the kindred did not display clinical manifestations of hyper- or hypothyroidism. DNA sequencing revealed a heterozygous G>A missense mutation at position 949 in Exon 9 of THRβ both in the patient and his mother, which led to the transition from alanine to threonine at position 317 of THRβ protein (A317T), whereas the rest of the kindred did not share this mutation. The proband and his mother were diagnosed with pituitary resistance to thyroid hormone. Oral administration of methimazole was stopped and β-receptor blockers were administrated. We present 1 pedigree of THRS with heterozygous A317T mutation in THRβ gene in the proband and his mother, which is the first reported mutation in Chinese and provides a comprehensive review of available literature.

  14. Correlation of skin changes with hormonal changes in polycystic ovarian syndrome: A cross-sectional study clinical study

    Directory of Open Access Journals (Sweden)

    B Vijaya Gowri

    2015-01-01

    Full Text Available Background: Polycystic ovarian syndrome (PCOS is a heterogenous collection of signs and symptoms that when gathered, form a spectrum of disorder with disturbance of reproductive, endocrine and metabolic functions. Aim: The aim of this study is to correlate the skin manifestations with hormonal changes and to know the incidence and prevalence of skin manifestations in patients with PCOS. Materials and Methods: A total of 40 patients with PCOS were examined during 1 year time period from May 2008 P to May 2009. Detailed clinical history was taken from each patient. PCOS was diagnosed on the basis of ultrasonography. Hormonal assays included fasting blood sugar, postprandial blood sugar, follicle-stimulating hormone, luteinizing hormone, thyroid stimulating hormone, dehydroepiandrostenedione, prolactin, free testosterone, fasting lipid profile and sex hormone binding globulin. The results obtained were statistically correlated. Results: In our study, the prevalence of cutaneous manifestations was 90%. Of all the cutaneous manifestations acne was seen in highest percentage (67.5%, followed by hirsutism (62.5%, seborrhea (52.5%, androgenetic alopecia (AGA (30%, acanthosis nigricans (22.5% and acrochordons (10%. Fasting insulin levels was the most common hormonal abnormality seen in both acne and hirsutism, whereas AGA was associated with high testosterone levels. Conclusion: The prevalence of cutaneous manifestations in PCOS was 90%. Hirsutism, acne, seborrhea, acanthosis nigricans and acrochordons were associated with increased levels of fasting insulin, whereas AGA showed higher levels of serum testosterone.

  15. Internal jugular vein: Peripheral vein adrenocorticotropic hormone ratio in patients with adrenocorticotropic hormone-dependent Cushing′s syndrome: Ratio calculated from one adrenocorticotropic hormone sample each from right and left internal jugular vein during corticotrophin releasing hormone stimulation test

    Directory of Open Access Journals (Sweden)

    Sachin Chittawar

    2013-01-01

    Full Text Available Background: Demonstration of central: Peripheral adrenocorticotropic hormone (ACTH gradient is important for diagnosis of Cushing′s disease. Aim: The aim was to assess the utility of internal jugular vein (IJV: Peripheral vein ACTH ratio for diagnosis of Cushing′s disease. Materials and Methods: Patients with ACTH-dependent Cushing′s syndrome (CS patients were the subjects for this study. One blood sample each was collected from right and left IJV following intravenous hCRH at 3 and 5 min, respectively. A simultaneous peripheral vein sample was also collected with each IJV sample for calculation of IJV: Peripheral vein ACTH ratio. IJV sample collection was done under ultrasound guidance. ACTH was assayed using electrochemiluminescence immunoassay (ECLIA. Results: Thirty-two patients participated in this study. The IJV: Peripheral vein ACTH ratio ranged from 1.07 to 6.99 ( n = 32. It was more than 1.6 in 23 patients. Cushing′s disease could be confirmed in 20 of the 23 cases with IJV: Peripheral vein ratio more than 1.6. Four patients with Cushing′s disease and 2 patients with ectopic ACTH syndrome had IJV: Peripheral vein ACTH ratio less than 1.6. Six cases with unknown ACTH source were excluded for calculation of sensitivity and specificity of the test. Conclusion: IJV: Peripheral vein ACTH ratio calculated from a single sample from each IJV obtained after hCRH had 83% sensitivity and 100% specificity for diagnosis of CD.

  16. Early-onset compartment syndrome of the limbs, a rare complication of meningococcal septicemia in infants?

    Directory of Open Access Journals (Sweden)

    Norbert Pallua

    2015-09-01

    Full Text Available Meningococcal (Neisseria meningitides septicemia is a feared diagnosis in febrile infants presenting with a petechial rash. Despite a good susceptibility to penicillin which usually results in an assumed bacterial clearance from blood cultures early on; extensive organ damage often mandates a prolonged period of aggressive supportive therapy. In this context, overall outcomes can dependent on specific, organ-related complications and thus should be anticipated. In the reported case, a rare sudden occurrence of early-onset compartment syndrome of all extremities highlights muscle ischemia as a possible cause of unresponsiveness to intensive care treatment despite assumed bacterial clearance. Among other conclusions, the presented case mandates a close monitoring of both clinical and laboratory features of muscle damage as a standard measure of care designed to trigger emergency surgical decision making. Most importantly, we anticipate a likely significant improvement of long-term outcomes based on the notion of meningococcal sepsis-induced compartment syndrome in infants being an under-diagnosed entity.

  17. Association between polycystic ovary syndrome and the risk of pregnancy complications

    Science.gov (United States)

    Yu, Hai-Feng; Chen, Hong-Su; Rao, Da-Pang; Gong, Jian

    2016-01-01

    Abstract Background: Polycystic ovary syndrome (PCOS) is inconsistently associated with increased risk of adverse pregnancy outcomes. The purpose of this meta-analysis was to summarize the evidence regarding the strength of the association between pregnancy in women with PCOS and pregnancy complications. Methods: We systematically searched PubMed, EmBase, and the Cochrane Library to identify observational studies up to January 2016. The primary focus was pregnancy outcomes, including gestational diabetes mellitus (GDM), preeclampsia, pregnancy-induced hypertension (PIH), preterm delivery, cesarean delivery, oligohydramnios, and polyhydramnios. Effect estimates were pooled using the random-effects model. The analysis was further stratified by factors that could affect these associations. Results: We included 40 observational studies that reported data on a total of 17,816 pregnancies with PCOS and 123,756 pregnancies without PCOS. Overall, PCOS in pregnancy was associated with greater risk of GDM, preeclampsia, PIH, preterm delivery, cesarean delivery, miscarriage, hypoglycemia, and perinatal death. However, PCOS in pregnancy had little or no effect on oligohydramnios, polyhydramnios, large-for-gestational age (LGA), small-for-gestational-age (SGA), fetal growth restriction (FGR), preterm premature membrane rupture, fasting blood glucose (FBG), high-density lipoprotein (HDL), low-density lipoprotein (LDL), triglyceride, total cholesterol, congenital malformation, macrosomia, and respiratory distress syndrome. Subgroup analysis suggested that these associations might be influenced by study design and pre-BMI. Conclusion: PCOS in pregnancy is associated with a significantly increased risk of adverse pregnancy, fetal, and neonatal outcomes. PMID:28002314

  18. PRES (posterior reversible encephalopathy syndrome), a rare complication of tacrolimus therapy.

    LENUS (Irish Health Repository)

    Hodnett, P

    2009-11-01

    With increasing numbers of solid organ and hematopoietic stem cell transplantations being performed, there have been significant increases in the use of immunosuppressive agents such as cyclosporine and tacrolimus. Posterior reversible encephalopathy syndrome (PRES) is a serious complication of immunosuppressive therapy use following solid organ or stem cell transplants. Clinical findings including headache, mental status changes, focal neurological deficits, and\\/or visual disturbances. Associated with these are characteristic imaging features of subcortical white matter lesions on computed tomography (CT) or magnetic resonance imaging (MRI). The changes in the subcortical white matter are secondary to potentially reversible vasogenic edema, although conversion to irreversible cytotoxic edema has been described. These imaging findings predominate in the territory of the posterior cerebral artery. Many studies have shown that the neurotoxicity associated with tacrolimus may occur at therapeutic levels. In most cases of PRES, the symptom complex is reversible by reducing the dosage or withholding the drug for a few days. While PRES is an uncommon complication, it is associated with significant morbidity and mortality if it is not expeditiously recognized. MRI represents the most sensitive imaging technique for recognizing PRES. This report highlights the value of MRI in prompt recognition of this entity, which offers the best chance of avoiding long-term sequelae.

  19. Preresection Obesity Increases the Risk of Hepatobiliary Complications in Short Bowel Syndrome

    Directory of Open Access Journals (Sweden)

    Alan N. Langnas

    2012-09-01

    Full Text Available Patients developing the short bowel syndrome (SBS are at risk for hepatobiliary disease, as are morbidly obese individuals. We hypothesized that morbidly obese SBS individuals would be at increased risk for developing hepatobiliary complications. We reviewed 79 patients with SBS, 53 patients with initial body mass index (BMI < 35 were controls. Twenty-six patients with initial BMI > 35 were the obese group. Obese patients were more likely to be weaned off parenteral nutrition (PN (58% vs. 21%. Pre-resection BMI was significantly lower in controls (26 vs. 41. BMI at 1, 2, and 5 years was decreased in controls but persistently increased in obese patients. Obese patients were more likely to undergo cholecystectomy prior to SBS (42% vs. 32% and after SBS (80% vs. 39%, p < 0.05. Fatty liver was more frequent in the obese group prior to SBS (23% vs. 0%, p < 0.05 but was similar to controls after SBS (23% vs. 15%. Fibrosis (8% vs. 13% and cirrhosis/portal hypertension (19% vs. 21% were similar in obese and control groups. Overall, end stage liver disease (ESLD was similar in obese and control groups (19% vs. 11% but was significantly higher in obese patients receiving PN (45% vs. 14%, p < 0.05. Obese patients developing SBS are at increased risk of developing hepatobiliary complications. ESLD was similar in the two groups overall but occurs more frequently in obese patients maintained on chronic PN.

  20. Hermansky-Pudlak syndrome Complicated by Pulmonary Fibrosis: Radiologic-Pathologic Correlation and Review of Pulmonary Complications

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    Tatiana Kelil

    2014-01-01

    Full Text Available Hermansky-Pudlak syndrome (HPS is a rare autosomal recessive disorder characterized by oculocutaneous hypopigmentation, platelet dysfunction, and in many cases, life-threatening pulmonary fibrosis. We report the clinical course, imaging, and postmortem findings of a 38-year-old female with HPS-related progressive pulmonary fibrosis, highlighting the role of imaging in assessment of disease severity and prognosis.

  1. A Case of Acute Budd-Chiari Syndrome Complicating Primary Antiphospholipid Syndrome Presenting as Acute Abdomen and Responding to Tight Anticoagulant Therapy

    Directory of Open Access Journals (Sweden)

    Naofumi Chinen

    2016-01-01

    Full Text Available A 34-year-old woman with primary antiphospholipid syndrome was admitted to the Gastroenterology Department of our hospital with fever, acute abdomen, watery diarrhea, and extremely high levels of inflammatory parameters. She had a history of left lower limb deep vein thrombosis and pulmonary embolism and was taking warfarin potassium. Acute gastroenteritis was suspected and an antibiotic was administered, but symptoms progressed. Abdominal ultrasonography showed occlusion of the left hepatic vein and the middle hepatic vein and her D-dimer level was high. Accordingly, Budd-Chiari syndrome was diagnosed and high-dose intravenous infusion of heparin was initiated. Her abdominal symptoms improved and the levels of inflammatory parameters and D-dimer decreased rapidly. It is known that antiphospholipid syndrome can be complicated by Budd-Chiari syndrome that usually occurs as subacute or chronic onset, but acute onset is rare. It is difficult to diagnose acute Budd-Chiari syndrome complicating antiphospholipid syndrome and this complication generally has a poor outcome. However, the present case can get early diagnosis and successful treatment with tight anticoagulant therapy.

  2. Prevalence of metabolic syndrome: association with risk factors and cardiovascular complications in an urban population.

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    Gisela Cipullo Moreira

    Full Text Available INTRODUCTION: Metabolic syndrome (MS is a set of cardiovascular risk factors and type 2 diabetes, responsible for a 2.5-fold increased cardiovascular mortality and a 5-fold higher risk of developing diabetes. OBJECTIVES: 1-to evaluate the prevalence of MS in individuals over 18 years associated with age, gender, socioeconomic status, educational levels, body mass index (BMI, HOMA index and physical activity; moreover, to compare it to other studies; 2-to compare the prevalence of elevated blood pressure (BP, high triglycerides and plasma glucose levels, low HDL cholesterol and high waist circumference among individuals with MS also according to gender; 3-to determine the number of risk factors in subjects with MS and prevalence of complications in individuals with and without MS aged over 40 years. METHODS: A cross-sectional study of 1369 Individuals, 667 males (48.7% and 702 females (51.3% was considered to evaluate the prevalence of MS and associated factors in the population. RESULTS: The study showed that 22.7% (95% CI: 19.4% to 26.0% of the population has MS, which increases with age, higher BMI and sedentary lifestyle. There was no significant difference between genders until age ≥70 years and social classes. Higher prevalence of MS was observed in lower educational levels and higher prevalence of HOMA positive among individuals with MS. The most prevalent risk factors were elevated blood pressure (85%, low HDL cholesterol (83.1% and increased waist circumference (82.5%. The prevalence of elevated BP, low HDL cholesterol and plasma glucose levels did not show significant difference between genders. Individuals with MS had higher risk of cardiovascular complications over 40 years. CONCLUSION: The prevalence of MS found is similar to that in developed countries, being influenced by age, body mass index, educational levels, physical activity, and leading to a higher prevalence of cardiovascular complications after the 4th decade of life.

  3. Wish to conceive and concerns to develop cardiovascular complications during pregnancy in patients with Turner syndrome.

    Science.gov (United States)

    van Hagen, Iris M; Duijnhouwer, Anthonie L; Ten Kate-Booij, Marianne J; Dykgraaf, Ramon H M; Duvekot, Johannes J; Utens, Elisabeth M W J; Roos-Hesselink, Jolien W

    2017-03-01

    Turner syndrome (TS) is associated with subfertility and infertility. Nevertheless, an increasing number of women become pregnant through oocyte donation. The wish to conceive may be negatively influenced by the fear of cardiovascular complications. The aim was to investigate the wish to conceive and the concerns about cardiovascular complications during pregnancy in women with TS. The patient association for TS invited all members of ≥18 years old (n = 344) to complete a specifically developed, disease-specific questionnaire, including questions about fertility, wish to conceive, attempts and concerns. Results were compared with previously published results of this questionnaire in women with congenital heart disease. The questionnaire was completed by 89 women (median age 30.1 years, Q1-Q3 = 22.9-39.4). Of them, 51% had 45, X0-monosomy and 38% had ≥1 cardiac abnormality. Seventeen women (19%) had attempted to become pregnant and 12 of them succeeded to become pregnant. Women who had not undertaken attempts to conceive (81%), considered themselves mainly too young or had no partner. Of the total sample, 58% were concerned about the influence of pregnancy on their cardiovascular status. This was higher (75%) in the sample of women with TS and cardiac abnormalities, than in women with congenital heart disease from a previously published cohort (21%), (p concerns about pregnancy complications between women with TS who respectively had or had not attempted to become pregnant. Women with TS, especially those with cardiac abnormalities, show serious concerns about the risks pregnancy may have. Patients should be timely counseled and specifically asked about their concerns. Psychosocial care should be provided when necessary.

  4. Ectopic adrenocorticotropic hormone syndrome in a case of duodenal neuroendocrine tumor presenting with liver metastasis

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    J Khare

    2018-01-01

    Full Text Available Ectopic adrenocorticotropic hormone (ACTH syndrome is an uncommon disorder and comprises about 15% of all patients with Cushing's syndrome (CS. Duodenal carcinoids are rare, indolent tumors usually associated with a benign progression. We hereby report a rare case of CS resulting from ectopic ACTH secretion from a duodenal neuroendocrine tumor (NET presenting with liver metastasis. A 37-year-old female presented with abdominal discomfort and dyspepsia of 1-month duration. Ultrasound abdomen suggested a well-defined hypoechoic lesion in the left lobe of the liver, suggestive of neoplasia. On clinical examination, she had Cushingoid features and persistent hypokalemia. Midnight ACTH and cortisol levels were grossly elevated at 1027 pg/ml (n < 46 pg/ml and 87.56 μg/dl (n < 7.5 μg/ml, respectively. Both overnight and high-dose dexamethasone suppression test confirmed nonsuppressed cortisol levels - 86.04 and 84.42 μg/dl (n < 1.8 μg/ml, respectively. Magnetic resonance imaging brain showed a structurally normal pituitary gland. Computed tomography scan of the abdomen revealed hepatic lesion with bilateral adrenal enlargement. A diagnosis of ectopic ACTH-dependent CS was made. Intraoperatively, a duodenal lesion of 0.5 cm × 0.5 cm was identified alongside an 8 cm × 6 cm exophytic lesion in segment IV of the liver. Frozen section of the duodenal lesion was positive for NET. She underwent a Whipple's surgery, cholecystectomy, and left hepatic lobectomy. Postoperatively, she showed clinical and biochemical remission. Herewith, we report the third case of duodenal carcinoid tumor presenting as ectopic ACTH syndrome and the first with liver metastasis.

  5. Growth hormone secretion decreases with age in paediatric Prader-Willi syndrome.

    Science.gov (United States)

    Cohen, M; Harrington, J; Narang, I; Hamilton, J

    2015-08-01

    Growth hormone deficiency is a common feature of Prader-Willi syndrome; however, biochemical deficiency is not uniformly demonstrated. Criteria for GH treatment in paediatric PWS vary with some countries requiring documentation of biochemical GH deficiency. Data regarding the significance of age in the interpretation of GH stimulation test results, particularly in infants, are lacking. We aimed to assess age-related trends in the prevalence of biochemical GH deficiency in infants and children with PWS. A retrospective chart review was conducted. Data from children with Prader-Willi syndrome that had GH stimulation tests performed at the Hospital for Sick Children in Toronto between the years 2000 and 2012 were collected. Charts of 47 children 0·4-15·5 years of age with PWS that had GH stimulation tests were reviewed. Biochemical GH status in relation to age and body mass index. Thirty-two of 47 patients (68%) were biochemically GH deficient. GH deficiency was significantly associated with older age (r = 0·45, P = 0·02) and higher body-mass-index z-score (r = 0·45, P = 0·02). Biochemical GH deficiency was less prevalent up to 18 months of age (3/11 27%) compared with older children (29/36 [81%]; P = 0·001). A higher prevalence of GH deficiency was also detected in obese patients (14/16 [88%]) compared with nonobese patients (18/31 [58%]; P = 0·04). The utility of performing GH stimulation tests as an indication of GH status under 18 months of age in Prader-Willi syndrome is questionable. If performed, results should be carefully interpreted in the context of age. © 2015 John Wiley & Sons Ltd.

  6. The first case of H5N1 avian influenza infection in a human with complications of adult respiratory distress syndrome and Reye's syndrome.

    Science.gov (United States)

    Ku, A S; Chan, L T

    1999-04-01

    Avian influenza virus was not known to cause systemic infection in humans before. We report a 3-year-old boy with good past health who developed pneumonia caused by H5N1 avian influenza A virus (A/Hong Kong/156/97). The virus was isolated from a tracheal aspirate. There were complications of Reye's syndrome, adult respiratory distress syndrome, and multiple organ system failure. He had a history of receiving aspirin. His adult respiratory distress syndrome did not respond to endotracheal surfactant replacement therapy. He died 6 days after admission. Clinicians should be alert to the importance of a new human influenza strain.

  7. Increased rates of complications in singleton pregnancies of women previously diagnosed with polycystic ovary syndrome predominantly in the hyperandrogenic phenotype

    NARCIS (Netherlands)

    de Wilde, Marlieke A.|info:eu-repo/dai/nl/413993809; Lamain-de Ruiter, Marije; Veltman-Verhulst, Susanne M.; Kwee, Anneke|info:eu-repo/dai/nl/290465648; Laven, Joop S.; Lambalk, Cornelis B.; Eijkemans, Marinus J.C.|info:eu-repo/dai/nl/156353253; Franx, Arie|info:eu-repo/dai/nl/157009939; Fauser, Bart C.J.M.|info:eu-repo/dai/nl/071281932; Koster, Maria P.H.

    2017-01-01

    Objective To study the presence of several maternal and neonatal complications in a cohort of women with hyperandrogenic as well as normoandrogenic polycystic ovary syndrome (PCOS) and women with PCOS who received different fertility treatments. Design Prospective multicenter cohort study. Setting

  8. Diverticulitis complicated by fistulous communication of sigmoid colon with anterior sacral meningocele in a patient with Marfan syndrome

    OpenAIRE

    Samet, Jonathan D.; Johnson, Pamela T.; Horton, Karen M.; Fishman, Elliot K.

    2015-01-01

    Rare in the general public, dural ectasia is a common finding in patients with Marfan syndrome. Complications are not frequent but include constipation, urinary retention, and meningitis. Presented here is a case of bacterial meningitis secondary to fistulous communication between a sacral meningocele and sigmoid colon in the setting of diverticulitis.

  9. A rare complication of total knee arthroplasty: Type l complex regional pain syndrome of the foot and ankle

    Directory of Open Access Journals (Sweden)

    Gözde Özcan Söylev

    2016-10-01

    Full Text Available Complex regional pain syndrome (CRPS is a painful and disabling disorder that usually affects the extremities. This complication may affect the knee joint after total knee arthroplasty (TKA. We report a unique case of CRPS of the foot and ankle, which was an unusual involvement site for CRPS after TKA.

  10. Adrenal hormone response and psychophysiological correlates under psychosocial stress in individuals with irritable bowel syndrome.

    Science.gov (United States)

    Sugaya, Nagisa; Izawa, Shuhei; Kimura, Kenta; Ogawa, Namiko; Yamada, Kosuke C; Shirotsuki, Kentaro; Mikami, Ikuyo; Hirata, Kanako; Nagano, Yuichiro; Nomura, Shinobu; Shimada, Hironori

    2012-04-01

    In this study, we investigated levels and relative ratios of adrenal hormones (including cortisol, dehydroepiandrosterone [DHEA], and DHEA-sulfate [DHEA-S]) and their psychophysiological correlates under acute psychosocial stress in individuals with irritable bowel syndrome (IBS). Fifty-three college students participated in the study (male: 42, female: 11; mean age: 22.64years), including 13 individuals with IBS (IBS group) and 40 individuals without IBS (control group). The participants were exposed to a standardized laboratory stressor, which included delivering a speech and performing a mental arithmetic task. We measured subjective stress levels and salivary cortisol, DHEA, and DHEA-S levels at relevant time points before, during, and after the tasks. DHEA-S level and the DHEA-S/DHEA ratio in the IBS group were significantly lower than those in the control group, and the cortisol/DHEA-S ratio in the IBS group was higher than that in the control group throughout the experiment. In the IBS group, the appraisal of a threat was positively correlated with cortisol levels (r=0.61), and the appraisal of controllability was negatively correlated with cortisol levels (r=-0.64) and with the cortisol/DHEA ratio (r=-0.71). The control group showed a significant positive correlation between the appraisal of threat and cortisol levels (r=0.32). The present study indicates that individuals with IBS had lower DHEA-S levels, and that their stressful cognitive appraisals under acute psychosocial stress caused the effects of cortisol to dominate. This adrenal hormone response may be involved in exacerbating abdominal symptoms in individuals with IBS. Copyright © 2012 Elsevier B.V. All rights reserved.

  11. Characterization of adult onset growth hormone deficiency syndrome in patients with hypothalamopituitary diseases: Asian Indian data

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    Bandgar T

    2008-01-01

    Full Text Available Background: Hardly any data is available on Adult onset growth hormone deficiency (AOGHD in Patients with hypothalamopituitary diseases in India. Aims: To characterize Asian Indian AOGHD syndrome in hypothalamopituitary diseases. Settings and Design: Cross-sectional analysis of data from a tertiary care hospital. Materials and Methods: Thirty patients with AOGHD were compared with 30 age-, sex-, body mass index-matched controls with respect to endocrine evaluation, biochemistry, body composition (BC, bone mineral density (BMD, cardiovascular risk profile and quality of life (QoL. Statistical Analysis Used: Comparisons were performed using two-tailed Student′s test (SPSS Software version 10.0. Results: Most of the patients had abnormal BC with central obesity [Truncal FM (%: males {33.9±4.4 (patient vs. 29.31±6.2 (control; P -0.027}; females {39.87±5.93 (patient vs. 35.76±3.16 (control; P - 0.025}] and poor QoL. Patients aged over 45 years did not show low bone mass or lipid abnormalities as compared to controls. Low BMD and abnormal lipid profile {Triglycerides [mg/dl]:170.55±72.5 (patient vs101.24±31.0 (control; P -0.038}; {very low density lipoprotein cholesterol [mg/dl]: 33.54±14.9 (patient vs. 20.25±6.18 (control; P - 0.05} was seen in female patients less than 45 years of age. Conclusions: Male and female (more than 45 years AOGHD patients have increased cardiovascular risk factors and poor QoL while BMD is unaffected. Females less than 45 years of age have the major characteristics of AOGHD and would be the group to benefit maximally with recombinant human Growth Hormone treatment, which is similar to that in the western literature.

  12. Growth Hormone Treatment Increases Plasma Irisin Concentration in Patients with Turner Syndrome.

    Science.gov (United States)

    Wikiera, B; Zawadzka, K; Łaczmański, Ł; Słoka, N; Bolanowski, M; Basiak, A; Noczyńska, A; Daroszewski, J

    2017-02-01

    Irisin (Ir) deficiency may be a contributing factor in metabolic disease. This study aimed to investigate the effect of supraphysiological doses of recombinant human growth hormone (rhGH) on Ir plasma concentration in relation to metabolic disorders, including obesity and other components of metabolic syndrome. We studied 36 girls with Turner syndrome (mean age 8.2 years) treated with rhGH (0.05 mg/kg/day). Anthropometric data and fasting blood levels [e. g., Ir, insulin, glucose, glycated hemoglobin (HbA1c), IGF-1, IGFBP-3, cholesterol, insulin resistance (HOMA-IR), and β-cell function (HOMA-β)] were analyzed prior to and following rhGH therapy [mean (SD) follow-up of 1.47 (0.89) years]. Insulin sensitivity (Matsuda index) was calculated before and after the glucose load. Following rhGH therapy, an increase in IGF-1 [mean (SD) of 119.40 (62.47) ng/ml to 439.08 (209.91) ng/ml, p=0.000], Ir [2.10 (1.03) μg/ml to 2.48 (0.78) μg/ml, p=0.036], HOMA-IR [median (IQR) of 0.64 (0.45-1.30) to 0.92 (0.67-2.36), p=0.0206], and HOMA-β values [45.00 (27.69-72.00) to 81.53 (51.43-132.00), p=0.0447] were observed. Multiple regression analysis yielded no associations between Ir and metabolic and hormonal parameters before rhGH treatment; however, on rhGH, the model (R2=0.56, adjusted R2=0.45) showed positive associations between Ir and IGF-1 standard deviation score and HbA1c, and negative associations between Ir and fasting blood glucose, HDL-cholesterol, and triglycerides. Despite manifestation of insulin resistance, rhGH application had a positive effect on Ir regulation, and restored physiological conditions of lipid and glucose metabolism. © Georg Thieme Verlag KG Stuttgart · New York.

  13. Growth hormone receptor (GHR) gene polymorphism and scoliosis in Prader-Willi syndrome.

    Science.gov (United States)

    Butler, Merlin G; Hossain, Waheeda; Hassan, Maaz; Manzardo, Ann M

    2017-12-06

    A growth hormone receptor (GHR) gene polymorphism impacts sensitivity to endogenous and exogenous growth hormone (GH) to moderate growth and development. Increased sensitivity may accelerate spinal growth and contribute to scoliosis, particularly in GH-deficient and treated populations such as Prader-Willi syndrome (PWS). Therefore, we examined the relationship between GHR genotype and scoliosis (case and control) in PWS cohorts. We utilized a case-control design in a study of 73 subjects (34M; 39F) with genetically confirmed PWS in 32 individuals previously diagnosed with moderate to severe scoliosis (mean age=16.9±10.2years; age range of 1 to 41years) and 41 adults with no evidence of scoliosis (mean age=30.8±9.7years; age range of 18 to 56years). The GHR gene polymorphism was determined using PCR specific primers to capture the two recognized GHR gene fragment sizes [i.e., full length (fl) or exon 3 deletions (d3)]. Twenty-three (72%) of the 32 case subjects with scoliosis required surgical correction with an approximately equal balance for gender and PWS genetic subtype among cases and 41 control subjects without scoliosis. The GHR d3/d3 genotype was identified in N=2 of 8 (25%) cases with scoliosis and the d3/fl genotype was identified in N=11 of 25 (44%) cases with scoliosis but the distribution difference did not statistically differ. The GHR fl/fl genotype was correlated with a significantly faster rate and heavier weight gain among case subjects. Our examination of demographic and genetic markers associated with scoliosis and surgical repair in PWS found no evidence to support differences in gender, PWS genetic subtype or GHR d3 allele distributions among the case vs control groups. Those with fl/fl alleles were heavier than those with d3/d3 or d3/fl genotypes and warrant further study with a larger sample size and possibly to include other vulnerable populations requiring growth hormone treatment. Copyright © 2017 Elsevier Ltd. All rights reserved.

  14. Concomitant psychiatric problems and hormonal treatment induced metabolic syndrome in gender dysphoria individuals: a 2 year follow-up study.

    Science.gov (United States)

    Colizzi, Marco; Costa, Rosalia; Scaramuzzi, Francesca; Palumbo, Claudia; Tyropani, Margarita; Pace, Valeria; Quagliarella, Luca; Brescia, Francesco; Natilla, Lilia Carmen; Loverro, Giuseppe; Todarello, Orlando

    2015-04-01

    Several studies indicate increased prevalence of metabolic syndrome (MetS) among patients with psychiatric disorders as well as among individuals with gender dysphoria (GD) treated by cross-sex hormonal treatment. However, the MetS prevalence among hormone treated GD individuals suffering from psychiatric problems has not been detected. From a sample of 146 GD patients we selected 122 metabolically healthy individuals in order to investigate the prevalence of MetS after the beginning of the cross-sex hormonal treatment in a 2 year follow-up assessment. Furthermore, we assessed differences in MetS prevalence between hormone treated GD patients with and without concomitant psychiatric problems. When treated with hormone therapy, GD patients reported changes in several parameters which are clustered in MetS, with statistically significant differences compared to baseline. Glyco-insulinemic alterations were more pronounced in male to female patients (MtFs). However, weight gain, waist circumference increases, blood pressure increases, and lipid alterations were similar in MtFs and female to male patients (FtMs). 14.8% of the sample at year 1 and 17.2% at year 2 developed MetS. Among patients with concomitant psychiatric problems, 50% at year 1 and 55% at year 2 developed MetS against 8% at year 1 and 10% at year 2 of patients without concomitant psychiatric problems. This study indicates that sex hormones induce MetS in a relatively low proportion of healthy GD individuals and especially during the first year of hormonal treatment. Most importantly, concomitant psychiatric problems are associated with considerably greater MetS prevalence in hormone treated GD individuals. Copyright © 2015 Elsevier Inc. All rights reserved.

  15. Osteomalacia complicating renal tubular acidosis in association with Sjogren′s syndrome

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    Zohra El Ati

    2014-01-01

    Full Text Available Renal involvement in Sjogren′s syndrome (SS is not uncommon and may precede other complaints. Tubulointerstitial nephritis is the most common renal disease in SS and may lead to renal tubular acidosis (RTA, which in turn may cause osteomalacia. Nevertheless, osteomalacia rarely occurs as the first manifestation of a renal tubule disorder due to SS. We herewith describe a 43-year-old woman who was admitted to our hospital for weakness, lumbago and inability to walk. X-ray of the long bones showed extensive demineralization of the bones. Laboratory investigations revealed chronic kidney disease with serum creatinine of 2.3 mg/dL and creatinine clearance of 40 mL/min, hypokalemia (3.2 mmol/L, hypophosphatemia (0.4 mmol/L, hypocalcemia (2.14 mmol/L and hyperchloremic metabolic acidosis (chlorine: 114 mmol/L; alkaline reserve: 14 mmol/L. The serum alkaline phosphatase levels were elevated. The serum levels of 25-hydroxyvitamin D and 1,25-dihydroxy vitamin D were low and borderline low, respectively, and the parathyroid hormone level was 70 pg/L. Urinalysis showed inappropriate alkaline urine (urinary PH: 7, glycosuria with normal blood glucose, phosphaturia and uricosuria. These values indicated the presence of both distal and proximal RTA. Our patient reported dryness of the mouth and eyes and Schirmer′s test showed xerophthalmia. An accessory salivary gland biopsy showed changes corresponding to stage IV of Chisholm and Masson score. Kidney biopsy showed diffuse and severe tubulo-interstitial nephritis with dense lymphoplasmocyte infiltrates. Sicca syndrome and renal interstitial infiltrates indicated SS as the underlying cause of the RTA and osteomalacia. The patient received alkalinization, vitamin D (Sterogyl ®, calcium supplements and steroids in an initial dose of 1 mg/kg/day, tapered to 10 mg daily. The prognosis was favorable and the serum creatinine level was 1.7 mg/dL, calcium was 2.2 mmol/L and serum phosphate was 0.9 mmol/L.

  16. Growth hormone therapy for Prader–willi syndrome: challenges and solutions

    Science.gov (United States)

    Grugni, Graziano; Sartorio, Alessandro; Crinò, Antonino

    2016-01-01

    Prader–Willi syndrome (PWS) is characterized by a dysregulation of growth hormone (GH)/insulin-like growth factor I axis, as the consequence of a complex hypothalamic involvement. PWS’ clinical picture seems to resemble the classic non-PWS GH deficiency (GHD), including short stature, excessive body fat, decreased muscle mass, and impaired quality of life. GH therapy is able to ameliorate the phenotypic appearance of the syndrome, as well as to improve body composition, physical strength, and cognitive level. In this regard, however, some pathophysiologic and clinical questions still remain, representing a challenge to give the most appropriate care to PWS patients. Data about the prevalence of GHD in PWS children are not unequivocal, ranging from 40% to 100%. In this context, to establish whether the presence (or not) of GHD may have a different effect on clinical course during GH therapy may be helpful. In addition, the comparison of GH effects in PWS children diagnosed as small for gestational age with those obtained in subjects born appropriate for gestational age is of potential interest for future trials. Emerging information seems to demonstrate the maintenance of beneficial effects of GH therapy in PWS subjects after adolescent years. Thus, GH retesting after achievement of final height should be taken into consideration for all PWS patients. However, it is noteworthy that GH administration exerts positive effects both in PWS adults with and without GHD. Another critical issue is to clarify whether the genotype–phenotype correlations may be relevant to specific outcome measures related to GH therapy. Moreover, progress of our understanding of the role of GH replacement and concomitant therapies on bone characteristics of PWS is required. Finally, a long-term surveillance of benefits and risks of GH therapy is strongly recommended for PWS population, since most of the current studies are uncontrolled and of short duration. PMID:27330297

  17. RESULTS OF LONG-TERM THERAPY WITH GROWTH-HORMONE IN 2 DOSE REGIMENS IN TURNER SYNDROME

    NARCIS (Netherlands)

    NIENHUIS, HE; RONGENWESTERLAKEN, C; WIT, JM; OTTEN, BJ; KEIZERSCHRAMA, SMPFD; DRAYER, NM; DELEMARREVANDEWAAL, HA; VULSMA, T; OOSTDIJK, W; WAELKENS, JJJ

    1993-01-01

    Girls with Turner syndrome were divided according to age (group A 6-12 years, and group B 12-19 years) and human growth hormone (GH) dose regimen (A1 and B1, three injections/week; A2 and B2, six injections/week). All groups responded to GH, 24 IU/M2/week, with an increase in height velocity, though

  18. A case of Bardet-Biedl syndrome complicated with intracranial hypertension in a Japanese child.

    Science.gov (United States)

    Saida, Ken; Inaba, Yuji; Hirano, Makito; Satake, Wataru; Toda, Tatsushi; Suzuki, Yutaka; Sudo, Asuka; Noda, Shunsuke; Hidaka, Yoshihiko; Hirabayashi, Kazutaka; Imai, Hiroki; Kurokawa, Toru; Koike, Kenichi

    2014-09-01

    Bardet-Biedl syndrome (BBS) is a rare heterogeneous autosomal recessive disorder characterized by rod-cone dystrophy, postaxial polydactyly, truncal obesity, hypogonadism, learning disability, and renal anomaly that are caused by ciliary dysfunction. 16 genes have been associated with the BBS phenotype. Although recent pathophysiological studies using animal models have shown that ciliary dysfunction may induce hydrocephalus, there have been no reports of BBS with intracranial hypertension. We here describe a 9-year-old Japanese girl who was diagnosed as having BBS and later received renal transplantation due to chronic renal failure. She also exhibited intracranial hypertension, including papilledema and increased intrathecal pressure (260-300 mmH2O), but her brain magnetic resonance imaging was normal. No genetic abnormalities were detected by DNA chip analysis or exome sequencing. Her papilledema improved following administration of acetazolamide. This is the first report of a case of BBS complicated with intracranial hypertension and its treatment. Copyright © 2013 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

  19. Vestibular rehabilitation following surgical repair for Superior Canal Dehiscence Syndrome: A complicated case report.

    Science.gov (United States)

    Carender, Wendy J; Grzesiak, Melissa

    2018-02-01

    Superior Canal Dehiscence Syndrome (SCDS) causes auditory and vestibular symptoms. Following surgical repair of the dehiscence, patients often experience dizziness and imbalance. This case report describes a postoperative vestibular exercise program, focusing on the principles of central compensation and habituation, and how it was modified for a patient with delayed progress secondary to strabismus and visual vertigo. A 63-year-old male with history of strabismus eye surgery, right hearing loss, aural fullness, and sensitivity to loud sounds was referred for vestibular rehabilitation (VR). He was seen for one preoperative and six postoperative PT visits over eight months. Outcome measures two weeks postoperative were as follows: Dizziness Handicap Inventory (DHI) 38/100; Timed Up & Go (TUG) 9.92 seconds; Dynamic Gait Index (DGI) 16/24; and a 3-line difference in Dynamic Visual Acuity (DVA). Improved outcomes at discharge included: DHI 18/100; TUG 6.87 seconds; DGI 23/24; and 1-line difference in DVA. He was able to return to work and previously enjoyed recreational activities. Postoperative vestibular rehabilitation programs are functionally and symptomatically beneficial following surgical repair for SCDS. Deviations from expected recovery should be addressed to achieve optimal outcomes as demonstrated in this complicated case report.

  20. Relationships between oral MUC1 expression and salivary hormones in burning mouth syndrome.

    Science.gov (United States)

    Kang, Jeong-Hyun; Kim, Yoon-Young; Chang, Ji-Youn; Kho, Hong-Seop

    2017-06-01

    To investigate possible relationships among oral mucosal epithelial MUC1 expression, salivary female gonadal hormones and stress markers, and clinical characteristics in patients with burning mouth syndrome (BMS). Thirty post-menopausal female patients with BMS (60.0±5.0 years) were included. Clinical and psychological evaluations were performed and the expression level of oral mucosal epithelial MUC1 was analyzed. The levels of cortisol, dehydroepiandrosterone (DHEA), 17β-estradiol, progesterone, chromogranin A, and blood contamination were determined from unstimulated whole saliva (UWS) and stimulated whole saliva (SWS) samples. Salivary progesterone level had significant positive correlations with oral mucosal epithelial MUC1 expression level and with salivary cortisol and DHEA levels. The salivary level of 17β-estradiol showed significant positive correlations with period of symptom duration, severity of effects of oral complaints on daily life, and results from psychological evaluations. Cortisol level in UWS and cortisol/DHEA ratio in UWS and SWS had negative correlations with severity of oral burning sensation significantly. The severity of taste disturbance had positive correlations with results from psychometry significantly. Dysregulated psychoendocrinological interactions might affect oral mucosal MUC1 expression and severity of oral burning sensation in post-menopausal BMS patients. Copyright © 2017 Elsevier Ltd. All rights reserved.

  1. Effects of Hormone Therapy on Oxidative Stress in Postmenopausal Women with Metabolic Syndrome

    Directory of Open Access Journals (Sweden)

    Martha A. Sánchez-Rodríguez

    2016-08-01

    Full Text Available The aim of this study was to determine the effect of oral hormone therapy (HT on oxidative stress (OS in postmenopausal women with metabolic syndrome (MetS. A randomized, double blind, placebo-controlled trial was carried out. We formed four groups of 25 women each; healthy (HW and MetS women (MSW were assigned to HT (1 mg/day of estradiol valerate plus 5 mg/10 day of medroxiprogesterone or placebo. We measured plasma lipoperoxides, erythrocyte superoxide dismutase and glutathione peroxidase, total plasma antioxidant status and uric acid, as OS markers. Alternative cut-off values of each parameter were defined and a stress score (SS ranging from 0 to 7 was used as total OS. MetS was defined according to National Cholesterol Education Program Adult Treatment Panel III (NCEP-ATPIII criteria. Participants were seen at baseline, 3 and 6 months. After 6 months, MetS decreased in MSW-HT (48%, their triglycerides and high-density lipoprotein cholesterol (HDL-c improved; in the other groups no difference was found. SS in MSW-HT decreased (3.8 ± 0.3 to 1.7 ± 0.3, p < 0.05 and OS was also reduced (44%, this effect was evident since 3 mo. HW-HT with high OS also decreased (40%. In placebo groups there was no change. Our findings suggest that HT improve lipids and OS associated to MetS in postmenopausal women.

  2. Body mass index (BMI) in Turner Syndrome before and during growth hormone (GH) therapy.

    Science.gov (United States)

    Blackett, P R; Rundle, A C; Frane, J; Blethen, S L

    2000-02-01

    To study whether body mass index (BMI) is different in girls with Turner syndrome (TS) compared to normal girls, and whether BMI in TS is affected by growth hormone (GH) treatment. A retrospective cross-sectional study. 2468 girls with TS enrolled in the National Cooperative Group Study (NCGS), a collaborative surveillance study for assessing GH-treated children. BMI and BMI standard deviation score (BMI SDS) at baseline and during GH treatment were computed from height and weight data. BMI in TS patients increases with age as expected. However, BMI SDS increased starting at about age 9 y. A similar pattern of increase in BMI SDS was observed after each year of GH treatment for up to 4 y, but GH treatment did not change the magnitude of increase. BMI and BMI SDS curves before and during GH treatment were essentially superimposable. These findings suggest that mechanisms specific for TS are responsible for the age-related increase in BMI SDS. This increase was unaffected by GH treatment.

  3. Metabolic and hormonal aspects of polycystic ovary syndrome: the impact of diet.

    LENUS (Irish Health Repository)

    O'Connor, Annalouise

    2010-11-01

    Polycystic ovary syndrome (PCOS) is a common, chronic endocrine condition affecting young women of reproductive age. It is characterised by hyperandrogenaemia, and profound menstrual and ovulatory dysfunction with consequent sub-fertility. A clustering of metabolic aberrations is commonly associated with this condition and these include insulin resistance, disordered lipid metabolism and chronic low-grade inflammation. Overweight and obesity, as well as a degree of adipose tissue dysfunction, are present in a large proportion of women with PCOS, and where present, magnify the inherent hyperandrogenaemia characteristic of the condition, in addition to worsening the metabolic profile. Diet and lifestyle interventions are among the first-line treatments for PCOS, and weight reduction through energy restriction has been shown to exert positive influences on both metabolic and hormonal aspects of this condition. Alterations in carbohydrate amount and type have also been investigated, and more recently, dietary fatty acids, with a particular emphasis on PUFA, have been shown to have a positive impact within this population group. Although it is likely that diet is not the root cause of PCOS, it represents a modifiable variable with the potential to improve the health of women with this condition. Work to date has provided insights into the role of diet in PCOS; however, further work is required to determine the role of nutrients specifically within the context of PCOS, in order to develop more effective, evidence-based dietary guidelines for this condition.

  4. Low-dose growth hormone and human immunodeficiency virus-associated lipodystrophy syndrome: a pilot study

    DEFF Research Database (Denmark)

    Andersen, Ove; Haugaard, Steen B; Flyvbjerg, A

    2004-01-01

    BACKGROUND: Treatment with high doses (2-6 mg day(-1)) of human growth hormone (hGH) in patients with human immunodeficiency virus (HIV)-associated lipodystrophy syndrome (HALS) has been shown to increase concentrations of total insulin-like growth-factor-I (IGF-I) more than twofold greater than.......01). Patients reported improvements of lipodystrophy, which was supported by a decreased waist-to-thigh ratio (P = 0.01), and waist-to-hip ratio (P = 0.06). Ratio of peripheral to trunk soft tissue mass increased (P = 0.01, measured by dual-energy X-ray absorptiometry scans) and a trend towards reduction......' treatment of lipodystrophic HIV-infected patients with hGH, 0.7 mg day(-1), increased total and free IGF-I twofold and appeared safe and tolerable. The potential of low-dose hGH in the treatment of HIV-lipodystrophy awaits examination by placebo-controlled, randomized trials....

  5. Expression of anti-Müllerian hormone in letrozole rat model of polycystic ovary syndrome.

    Science.gov (United States)

    Du, Dan-Feng; Li, Xue-Lian; Fang, Fang; Du, Mei-Rong

    2014-01-01

    Polycystic ovary syndrome (PCOS) is a common endocrine disorder in women of reproductive age with anti-müllerian hormone (AMH) two to three times higher, but the mechanism of increased AMH, excessive follicles and follicle stagnation in PCOS still needs further research. Female Sprague-Dawley rats were treated with a gavage of 1.0 mg/kg of letrozole carboxymethylcellulose solution once daily for 21 consecutive days. Serum steroid concentrations, ovarian morphology, ovarian expression of AMH and AMH-RII protein were determined and their relationships were studied. According to the morphology and endocrinology, the letrozole model group was a successful PCOS model. Serum AMH and ovarian local expression of AMH and AMH-RII were both increased in letrozole model group. The elevated AMH had a positive correlation with T, growing follicle count and a negative correlation with body weight. The letrozole model group is a good animal model for the study of AMH in PCOS patients with obesity or insulin resistance. The increased serum AMH level in PCOS is the consequence of the androgen-induced excess of small antral follicles. These results lead to the hypothesis that reducing AMH may become a therapeutic target of PCOS, which is worth further research.

  6. Expression of anti-Müllerian hormone in two rat models of polycystic ovary syndrome.

    Science.gov (United States)

    Du, Dan-Feng; Li, Xue-Lian; Zheng, Sai-Hua

    2016-12-01

    Anti-Müllerian hormone (AMH) levels are two to three times higher in patients with polycystic ovary syndrome (PCOS), but the mechanism of increased AMH levels in PCOS remains unclear. The purpose of our experiment was to investigate a change in AMH levels in two kinds of commonly used rat models and to determine an ideal model for future research of AMH in the pathogenesis of PCOS. Thirty female Sprague Dawley rats were treated using two modeling methods: implantation of a levonorgestrel silastic implant or injection with sodium prasterone sulfate plus human chorionic gonadotropin (hCG). Rats in the control group were implanted with a blank silastic stick. Serum steroid concentrations, ovarian morphology and ovarian expression of AMH and AMH-receptor II (RII) proteins were determined and their correlations were studied. The results from the levonorgestrel and hCG group were closer to those displayed by human PCOS patients than the sodium prasterone sulfate and hCG group. Ovarian local expression of AMH and AMH-RII was increased in these both models compared with the control group; however, an elevation of serum AMH concentration was not observed (12.53 ± 0.99 ng/ml and 13.22 ± 1.09 ng/ml vs 16.30 ± 0.98 ng/ml). The levonorgestrel and hCG model is more suitable for the study of PCOS in puberty. © 2016 Japan Society of Obstetrics and Gynecology.

  7. [The treament of hyponatremia secundary to the syndrome of inappropriate antidiuretic hormone secretion].

    Science.gov (United States)

    Runkle, Isabelle; Villabona, Carles; Navarro, Andrés; Pose, Antonio; Formiga, Francesc; Tejedor, Alberto; Poch, Esteban

    2013-12-07

    The syndrome of inappropriate antidiuretic hormone secretion (SIADH) is the most frequent cause of hyponatremia in a hospital setting. However, detailed protocols and algorithms for its management are lacking. Our objective was to develop 2 consensus algorithms for the therapy of hyponatremia due to SIADH in hospitalized patients. A multidisciplinary group made up of 2 endocrinologists, 2 nephrologists, 2 internists, and one hospital pharmacist held meetings over the period of a year. The group worked under the auspices of the European Hyponatremia Network and the corresponding Spanish medical societies. Therapeutic proposals were based on widely-accepted recommendations, expert opinion and consensus guidelines, as well as on the authors' personal experience. Two algorithms were developed. Algorithm 1 addresses acute correction of hyponatremia posing as a medical emergency, and is applicable to both severe euvolemic and hypovolemic hyponatremia. The mainstay of this algorithm is the iv use of 3% hypertonic saline solution. Specific infusion rates are proposed, as are steps to avoid or reverse overcorrection of serum sodium levels. Algorithm 2 is directed to the therapy of SIADH-induced mild or moderate, non-acute hyponatremia. It addresses when and how to use fluid restriction, solute, furosemide, and tolvaptan to achieve eunatremia in patients with SIADH. Two complementary strategies were elaborated to treat SIADH-induced hyponatremia in an attempt to increase awareness of its importance, simplify its therapy, and improve prognosis. Copyright © 2013 Elsevier España, S.L. All rights reserved.

  8. Disorders of water metabolism: diabetes insipidus and the syndrome of inappropriate antidiuretic hormone secretion.

    Science.gov (United States)

    Verbalis, Joseph G

    2014-01-01

    Disorders of body fluids are among the most commonly encountered problems in the practice of clinical medicine. This is in large part because many different disease states can potentially disrupt the finely balanced mechanisms that control the intake and output of water and solute. It therefore behooves clinicians treating such patients to have a good understanding of the pathophysiology, the differential diagnosis and the management of these disorders. Since body water is the primary determinant of the osmolality of the extracellular fluid (ECF), disorders of body water homeostasis can be divided into hypoosmolar disorders, in which there is an excess of body water relative to body solute, and hyperosmolar disorders, in which there is a deficiency of body water relative to body solute. The classical hyperosmolar disorder is diabetes insipidus (DI), and the classical hypoosmolar disorder is the syndrome of inappropriate antidiuretic hormone secretion (SIADH). This chapter first reviews the regulatory mechanisms underlying water and sodium metabolism, the two major determinants of body fluid homeostasis. The major disorders of water metabolism causing hyperosmolality and hypoosmolality, DI and SIADH, are then discussed in detail, including the pathogenesis, differential diagnosis and treatment of these disorders. © 2014 Elsevier B.V. All rights reserved.

  9. Growth hormone therapy, muscle thickness, and motor development in Prader-Willi syndrome: an RCT.

    Science.gov (United States)

    Reus, Linda; Pillen, Sigrid; Pelzer, Ben J; van Alfen-van der Velden, Janielle A A E M; Hokken-Koelega, Anita C S; Zwarts, Machiel; Otten, Barto J; Nijhuis-van der Sanden, Maria W G

    2014-12-01

    To investigate the effect of physical training combined with growth hormone (GH) on muscle thickness and its relationship with muscle strength and motor development in infants with Prader-Willi syndrome (PWS). In a randomized controlled trial, 22 infants with PWS (12.9 ± 7.1 months) were followed over 2 years to compare a treatment group (n = 10) with a waiting-list control group (n = 12). Muscle thickness of 4 muscle groups was measured by using ultrasound. Muscle strength was evaluated by using the Infant Muscle Strength meter. Motor performance was measured with the Gross Motor Function Measurement. Analyses of variance were used to evaluate between-group effects of GH on muscle thickness at 6 months and to compare pre- and posttreatment (after 12 months of GH) values. Multilevel analyses were used to evaluate effects of GH on muscle thickness over time, and multilevel bivariate analyses were used to test relationships between muscle thickness, muscle strength, and motor performance. A significant positive effect of GH on muscle thickness (P muscle thickness and muscle strength (r = 0.61, P muscle thickness and motor performance (r = 0.81, P muscle strength and motor performance (r = 0.76, P muscle thickness, which was related to muscle strength and motor development in infants with PWS. Catch-up growth was faster in muscles that are most frequently used in early development. Because this effect was independent of GH, it suggests a training effect. Copyright © 2014 by the American Academy of Pediatrics.

  10. The Syndrome of Inappropriate Secretion of AntiDiuretic Hormone in Patients With Brucellosis.

    Science.gov (United States)

    Dulger, Ahmet Cumhur; Aslan, Mehmet; Ceylan, Mehmet Resat; Olmez, Sehmus; Karadas, Sevdegul; Akdeniz, Hayrettin

    2015-09-01

    Various studies have shown that a number of infectious disease causes syndrome of inappropriate antidiuretic hormone (SIADH). However, the relationship between infectious disease and SIADH is not yet fully known. In this prospective study, we aimed to assess the presence of SIADH in patients with brucellosis. Thirty-five patients with acute brucellosis were retrospectively reviewed. The diagnosis of brucellosis was performed using the Wright test in connection with blood culture. SIADH was defined by euvolemic hyponatremia (serum sodium level lower than 135 mEq/l) with increased urinary sodium excretion (urinary sodium higher than 40 mmol/l). Of the 35 patients, 19 (54%) had SIADH; 20 (57%) also had hypouricemia (uric acid level lower than 4 mg/dl). Additionally, all of the studied patients had a high mean urinary sodium excretion rate (mean 132 mmol/l; range 40-224). Most importantly, the hyponatremic patients were more likely to have a lower albumin level (P brucellosis. The presence of SIADH could be a diagnostic tool for diagnosing brucellosis. Further larger randomized studies may confirm these findings. © 2014 Wiley Periodicals, Inc.

  11. The Association between Parathyroid Hormone Levels and the Cardiorenal Metabolic Syndrome in Non-Diabetic Chronic Kidney Disease.

    Science.gov (United States)

    Saab, Georges; Whaley-Connell, Adam; Bombeck, Andrew; Kurella Tamura, Manjula; Li, Suying; Chen, Shu-Cheng; McFarlane, Samy I; Sowers, James R; Norris, Keith; Bakris, George L; McCullough, Peter A

    2011-01-01

    AIMS: The relationship between parathyroid hormone (PTH) and the cardiorenal metabolic syndrome was examined among non-diabetic persons with chronic kidney disease (CKD). METHODS: In a cross-sectional analysis, the relationship between PTH levels and the cardiorenal metabolic syndrome was investigated in 3,215 non-diabetic participants in the National Kidney Foundation-Kidney Early Evaluation Program (KEEP 2.0) found to have CKD (eGFR cardiorenal metabolic syndrome increased along increasing PTH quartiles (31.7, 33.8, 37.3, and 48.7%, respectively, p for trend cardiorenal metabolic syndrome were 16% (p = 0.18), 35% (p = 0.006), and 80% (p cardiorenal metabolic syndrome. The association of PTH with the cardiorenal metabolic syndrome was not modified by age or gender (p for interaction was not significant for both modifiers). CONCLUSIONS: Among an outpatient non-diabetic population with CKD, higher PTH levels were associated with a higher prevalence of the cardiorenal metabolic syndrome.

  12. Ghrelin is independently associated with anti-mullerian hormone levels in obese but not non-obese women with polycystic ovary syndrome.

    Science.gov (United States)

    Garin, Margaret C; Butts, Samantha F; Sarwer, David B; Allison, Kelly C; Senapati, Suneeta; Dokras, Anuja

    2017-03-01

    Ghrelin is an endogenous appetite stimulant that may have a role in ovarian function. Women with polycystic ovary syndrome have anovulation and frequently weight management issues; however the associations between ghrelin and hormonal markers in polycystic ovary syndrome have not been well studied. In order to characterize the association between total ghrelin levels and ovarian function and the possible modification of this relationship by obesity, we examined total ghrelin levels and anti-mullerian hormone, total testosterone, and insulin in obese and non-obese women with and without polycystic ovary syndrome. Total ghrelin levels were lower in obese women with polycystic ovary syndrome (n = 45) compared to obese controls (n = 33) (p = 0.005), but similar in non-obese women with polycystic ovary syndrome (n = 20) compared to non-obese controls (n = 21) (p = NS). In the obese polycystic ovary syndrome group, anti-mullerian hormone was associated with ghrelin levels independent of age, insulin, and total testosterone (p = 0.008). There was no association between total ghrelin and anti-mullerian hormone levels in non-obese women with polycystic ovary syndrome, non-obese controls, or obese controls (p = NS). Our results provide evidence for a potential relationship between ghrelin and ovarian function in obese women with polycystic ovary syndrome that was not observed in non-obese women with polycystic ovary syndrome or controls.

  13. Abnormalities of the axial and proximal appendicular skeleton in adults with Laron syndrome (growth hormone insensitivity)

    Energy Technology Data Exchange (ETDEWEB)

    Kornreich, L.; Konen, O.; Schwarz, M.; Horev, G. [Schneider Children' s Medical Center of Israel, Imaging Department, Petah Tiqwa (Israel); Tel Aviv University, Sackler Faculty of Medicine, Tel Aviv (Israel); Siegel, Y. [Rabin Medical Center, Imaging Department, Petah Tiqwa (Israel); Jackson Memorial Hospital, Department of Radiology, Thoracic Section, Miami, FL (United States); Hershkovitz, I. [Tel Aviv University, Department of Anatomy and Anthropology, Sackler Faculty of Medicine, Tel Aviv (Israel); Laron, Z. [Schneider Children' s Medical Center of Israel, Endocrinology and Diabetes Research Unit, Petah Tiqwa (Israel); Tel Aviv University, Sackler Faculty of Medicine, Tel Aviv (Israel)

    2008-02-15

    To investigate abnormalities in the skeleton (with the exclusion of the skull, cervical spine, hands and feet) in patients with Laron syndrome, who have an inborn growth hormone resistance and congenital insulin-like growth factor-1 (IGF-1) deficiency. The study group was composed of 15 untreated patients with Laron syndrome (seven male and eight female) aged 21-68 years. Plain films of the axial and appendicular skeleton were evaluated retrospectively for abnormalities in structure and shape. The cortical width of the long bones was evaluated qualitatively and quantitatively (in the upper humerus and mid-femur), and the cortical index was calculated and compared with published references. Measurements were taken of the mid-anteroposterior and cranio-caudal diameters of the vertebral body and spinous process at L3, the interpedicular distance at L1 and L5, and the sacral slope. Thoracic and lumbar osteophytes were graded on a 5-point scale. Values were compared with a control group of 20 healthy persons matched for age. The skeleton appeared small in all patients. No signs of osteopenia were visible. The cortex of the long bones appeared thick in the upper limbs in 11 patients and in the lower limbs in four. Compared with the reference values, the cortical width was thicker than average in the humerus and thinner in the femur. The vertebral diameters at L3 and the interpedicular distances at L1 and L5 were significantly smaller in the patients than in the control subjects (P < 0.001); however, at L5 the canal was wider, relative to the vertebral body. The study group had a higher rate of anterior osteophytes in the lumbar spine than the controls had, and their osteophytes were also significantly larger. In the six patients for whom radiographs of the upper extremity in its entirety were available on one film, the ulna appeared to be rotated. In one 22-year-old man, multiple epiphyses were still open. Congenital IGF-1 deficiency leads to skeletal abnormalities

  14. Clinical correlates of complicated grief among individuals with acute coronary syndromes

    Directory of Open Access Journals (Sweden)

    Pini S

    2015-10-01

    Full Text Available Stefano Pini,1 Camilla Gesi,1 Marianna Abelli,1 Alessandra Cardini,1 Lisa Lari,1 Francesca Felice,2 Rossella Di Stefano,2 Gianfranco Mazzotta,3 Francesco Bovenzi,4 Daniele Bertoli,5 Lucia Borelli,4 Paola Michi,1 Claudia Oligeri,3 Alberto Balbarini,2 Vijaya Manicavasagar6 1Department of Clinical and Experimental Medicine, Psychiatry Sector, University of Pisa, Pisa, 2Department of Surgical, Medical, Molecular and Critical Area Pathology, University of Pisa, Pisa, 3Unit of Cardiology, Ospedale Sant’Andrea, La Spezia, 4Cardio-Respiratory Department, Ospedale Campo di Marte, Lucca, 5Unit of Cardiology, Ospedale San Bartolomeo, Sarzana (SP, Italy; 6Black Dog Institute, University of New South Wales, Sydney, NSW, Australia Objective: The study aimed at exploring bereavement and complicated grief (CG symptoms among subjects without a history of coronary heart disease (CHD at the time of a first acute coronary syndrome (ACS and to evaluate the relationship of CG symptoms and ACS. Method: Overall, 149 subjects with ACS (namely, acute myocardial infarct with or without ST-segment elevation or unstable angina, with no previous history of CHD, admitted to three cardiac intensive care units were included and evaluated by the Structured Clinical Interview for Complicated Grief (SCI-CG, Hamilton Depression Rating Scale, Hamilton Anxiety Rating Scale, and the 36-item Short-Form Health Survey (MOS-SF-36. Results: Of the total sample of 149 subjects with ACS, 118 (79.2% met criteria for DSM-5 persistent complex bereavement disorder. Among these, subjects who lost a partner, child, or sibling were older (P=0.008, less likely to be working (P=0.032, and more likely to be suffering from hypertension (P=0.021, returned higher scores on the SCI-CG (P=0.001 and developed the index ACS more frequently between 12 and 48 months after the death than those who lost a parent or another relative (P≤0.0001. The occurrence of ACS 12–48 months (P=0.019 after the loss was

  15. Clinical features and growth hormone receptor gene mutations of patients with Laron syndrome from a Chinese family.

    Science.gov (United States)

    Ying, Yan-Qin; Wei, Hong; Cao, Li-Zhi; Lu, Juan-Juan; Luo, Xiao-Ping

    2007-08-01

    Laron syndrome is an autosomal recessive disorder caused by defects of growth hormone receptor (GHR) gene. It is characterized by severe postnatal growth retardation and characteristic facial features as well as high circulating levels of growth hormone (GH) and low levels of insulin-like growth factor I (IGF-I) and insulin-like growth factor binding protein-3 (IGFBP-3). This report described the clinical features and GHR gene mutations in 2 siblings with Laron syndrome in a Chinese family. Their heights and weights were in the normal range at birth, but the growth was retarded after birth. When they presented to the clinic, the heights of the boy (8 years old) and his sister (11 years old) were 80.0 cm (-8.2 SDS) and 96.6 cm (-6.8 SDS) respectively. They had typical appearance features of Laron syndrome such as short stature and obesity, with protruding forehead, saddle nose, large eyes, sparse and thin silky hair and high-pitched voice. They had higher basal serum GH levels and lower serum levels of IGF-I, IGFBP-3 and growth hormone binding protein (GHBP) than normal controls. The peak serum GH level after colonidine and insulin stimulations in the boy was over 350 ng/mL. After one-year rhGH treatment, the boy's height increased from 80.0 cm to 83.3 cm. The gene mutation analysis revealed that two patients had same homozygous mutation of S65H (TCA -->CCA) in exon 4, which is a novel gene mutation. It was concluded that a definite diagnosis of Laron syndrome can be made based on characteristic appearance features and serum levels of GH, IGF-I, IGFBP-3 and GHBP. The S65H mutation might be the cause of Laron syndrome in the two patients.

  16. Os peroneum friction syndrome complicated by sesamoid fatigue fracture: a new radiological diagnosis? Case report and literature review

    Energy Technology Data Exchange (ETDEWEB)

    Bashir, Waseem A.; Connell, David A. [Royal National Orthopaedic Hospital NHS Trust, Department of Radiology, London, Middlesex (United Kingdom); Lewis, Steve [Craven Cottage, Fulham Football Club, London (United Kingdom); Cullen, Nicholas [The Royal National Orthopaedic Hospital NHS Trust, Department of Orthopaedics, London, Middlesex (United Kingdom)

    2009-02-15

    Injuries to the peroneal tendons are relatively common worldwide but tendon rupture without significant trauma is uncommon. Ankle mechanics can be seriously affected by disruption of one or both of the peroneal tendons although complete rupture can also remain asymptomatic. Accessory ossicles are sesamoid bones and are common findings in routine radiology of the foot and ankle. Although in the vast majority these ''os'' are normal variants of anatomy, they can lead to painful syndromes and suffer fractures and even undergo degenerative changes in response to overuse and trauma. Although similar syndromes have been discussed in the surgical literature, there is a lack of literature describing the use of modern imaging in the accurate diagnosis and its subsequent assistance towards appropriate management of os peroneum friction syndrome complicated by sesamoid fatigue syndrome. This article presents the plain film, sonographic and magnetic resonance imaging findings in a case of os peroneum friction syndrome complicated by a sesamoid fatigue fracture as well as reviewing the pertinent literature. (orig.)

  17. Prevalence of malnutrition-inflammation complex syndrome and its correlation with thyroid hormones in chronic haemodialysis patients.

    Science.gov (United States)

    Chávez Valencia, Venice; Mejía Rodríguez, Oliva; Viveros Sandoval, Martha Eva; Abraham Bermúdez, Juan; Gutiérrez Castellanos, Sergio; Orizaga de la Cruz, Citlalli; Roa Córdova, Martha Alicia

    2017-10-25

    Low levels of thyroid hormones, total triiodothyronine (T3) and free triiodothyronine (FT3) in haemodialysis patients is a marker of malnutrition and inflammation and are predictors of mortality. The aim of this study was to determine the prevalence of malnutrition-inflammation complex syndrome in haemodialysis and its relationship with the thyroid hormones thyrotropin, T3, FT3 and free thyroxine (FT4), as well as to evaluate the prevalence of low FT3 syndrome and its correlation with nutritional and inflammatory markers. Cross-sectional, analytical and comparative study that enrolled 128 haemodialysis patients: 50.8% females; mean age 45.05±17.01 years; mean time on haemodialysis 45.4±38.8 months; 29.7% diabetics; 79.7% with hypertension. Serum thyroid hormones thyrotropin, T3, FT3 and FT4 concentrations were measured and Malnutritition-Inflammation Score (MIS) was applie to diagnostic. Mean thyroid hormone values were: thyroid hormones thyrotropin 2.48±1.8 mIU/ml (range: 0.015-9.5), T3 1.18±0.39 ng/ml (range 0.67-2.64), FT3 5.21±0.96pmol/l (range: 3.47-9.75); FT4 1.35±0.4 ng/ml (range: 0.52-2.57). Malnutrition-inflammation complex syndrome prevalence was 53.9%; 11.7% presented low FT3 levels. Serum T3 and FT3 concentrations inversely correlated with Malnutritition-Inflammation Score (MIS), while FT4 correlated positively with Malnutrition-Inflammation Score. In the linear regression analysis, low FT3 was associated with IL-6 (β= 0.265, p=.031), C-reactive protein (CRP) (β= -0.313, p=.018) and albumin (β= 0.276, p=.002). Low T3 and FT3 levels are correlated with malnutrition and inflammation parameters. Malnutrition-inflammation complex syndrome can affect serum concentrations of thyroid hormones. Copyright © 2017 Sociedad Española de Nefrología. Published by Elsevier España, S.L.U. All rights reserved.

  18. Male Child with Van Wyk-Grumbach's Syndrome and Other Complications of Long-Standing Primary Hypothyroidism: A Case Report

    Directory of Open Access Journals (Sweden)

    Ahmed Omran

    2012-01-01

    Full Text Available Primary hypothyroidism in the juvenile population generally leads to retardation of linear growth and delay or even arrest of puberty. However, in rare conditions, children with long-standing hypothyroidism present with signs of Van Wyk-Grumbach's syndrome (VWGS which include juvenile hypothyroidism, delayed bone age, and pseudoprecocious puberty. We report a rare case of prepubertal male child from Asian origin, presented with long-standing untreated hypothyroidism complicated with VWGS and other complications including obesity, short stature, hepatomegaly, asymptomatic mild pericardial effusion, and pituitary hyperplasia.

  19. Atypical Presentation of Intracardiac Floating Thrombi in Hypereosinophilic Syndrome Complicated With Stroke and Systemic Embolization: A Case Report.

    Science.gov (United States)

    Lai, Chih-Hung; Chang, Szu-Ling; Lin, Wei-Wen; Hsiung, Ming-Chon; Juan, Yu-Hsiang; Wang, Tzu-Lin

    2015-10-01

    Hypereosinophilic syndrome (HES) describes a disorder characterized by persistent peripheral blood eosinophilia with evidence of multiple target organs damage caused by eosinophilia. HES most commonly involves the heart, and cardiac involvement typically presents in the form of endomyocarditis or myocarditis with apical mural thrombus formation.We present a case with atypical cardiac presentation with massive intracardiac fragile thrombi, causing peripheral emboli and strokes.HES can present as floating thrombi with thin attachment to the left ventricle, and clinicians should also be vigilant of thromboembolic complications and initiate early therapy to prevent or reduce the potential complications of HES.

  20. The increase of alpha-melanocyte-stimulating hormone in the plasma of chronic fatigue syndrome patients

    Directory of Open Access Journals (Sweden)

    Shishioh-Ikejima Nobue

    2010-08-01

    Full Text Available Abstract Background Despite extensive research, no reliable biological marker for chronic fatigue syndrome (CFS has yet been identified. However, hyperactivation of melanotrophs in the pituitary gland and increased levels of plasma alpha-melanocyte-stimulating hormone (α-MSH have recently been detected in an animal model of chronic stress. Because CFS is considered to be caused partly by chronic stress events, increased α-MSH plasma levels may also occur in CFS patients. We therefore examined α-MSH levels in CFS patients. Methods Fifty-five CFS patients, who were previously diagnosed within 10 years of with the disease, were enrolled in this study. Thirty healthy volunteers were studied as controls. Fasting bloods samples were collected in the morning and evaluated for their plasma levels of α-MSH, adrenocorticotropic hormone (ACTH, serum cortisol and dehydroepiandrosterone sulfate (DHEA-S. Mean levels of α-MSH were compared between the CFS and control groups using Welch's t test. Results The mean plasma α-MSH concentration in the CFS group (17.9 ± 1.0 pg/mL was significantly higher than that in healthy controls (14.5 ± 1.0 pg/mL, p = 0.02. However, there was a wide range of values in the CFS group. The factors correlated with the plasma α-MSH values were analyzed using Spearman's rank correlation. A negative correlation was found between the duration of the CFS and the plasma α-MSH values (p = 0.04, rs = -0.28, but no correlations with ACTH, cortisol or DHEA-S levels were identified (p = 0.55, 0.26, 0.33, respectively. The CFS patients were divided into two groups: patients diagnosed for ≤ 5 years' duration, and those diagnosed for 5-10 years' duration. They were compared with the healthy controls using one-way ANOVA and Tukey-Kramer multiple comparison tests. The mean α-MSH concentration in the ≤ 5 years group was 20.8 ± 1.2 pg/mL, which was significantly higher than that in the healthy controls (p Conclusions CFS patients with

  1. Does anemia-polycythemia complicating twin-twin transfusion syndrome affect outcome after fetoscopic laser surgery?

    Science.gov (United States)

    Donepudi, R; Papanna, R; Snowise, S; Johnson, A; Bebbington, M; Moise, K J

    2016-03-01

    Twin anemia-polycythemia sequence (TAPS) can occur as a unique disease or as a complication of twin-twin transfusion syndrome (TTTS). Middle cerebral artery (MCA) Doppler studies are not currently part of the routine evaluation of monochorionic twins since they are not used in the Quintero staging system. As such, the true incidence of TAPS is unknown. We aimed to compare the characteristics and outcomes of twin pregnancies with TTTS complicated by spontaneous anemia-polycythemia vs those with TTTS alone. This was a secondary analysis of data collected prospectively from a cohort of 156 consecutive patients undergoing fetoscopic laser surgery for TTTS, between October 2011 and August 2014. TAPS was defined as discordance in the preoperative MCA peak systolic velocity (PSV), with one twin fetus having MCA-PSV ≤ 1.0 multiples of the median (MoM) and the other having MCA-PSV ≥ 1.5 MoM. Maternal demographics as well as preoperative, operative and postoperative variables were analyzed. Included in the final analysis were 133 patients with complete records: 11 cases with TTTS with anemia-polycythemia and 122 cases with TTTS alone. There was no difference in maternal body mass index, gestational age (GA) at procedure, rate of preterm prelabor rupture of membranes or GA at delivery between the two groups. Patients with TTTS and anemia-polycythemia were more likely to be older (P = 0.03) and parous (P = 0.04) and had a significantly lower number of placental anastomoses (P = 0.01). The dual live-birth rate was similar for both groups (P = 0.76). Cases of TTTS with anemia-polycythemia were more likely to be found in parous and older women and were characterized by fewer vascular anastomoses. TTTS with anemia-polycythemia was not associated with worse perinatal outcome after laser therapy. Copyright © 2015 ISUOG. Published by John Wiley & Sons Ltd. Copyright © 2015 ISUOG. Published by John Wiley & Sons Ltd.

  2. Cardiac complications in a metamizole-induced type I Kounis syndrome

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    Jose F. Martínez Juste

    2016-04-01

    Full Text Available ABSTRACT Kounis syndrome is defined as the coincidental occurrence of allergic reaction and acute coronary syndrome secondary to vasospasm. Anti-inflammatory drugs are included as one of the multiple causes. Current data available about this syndrome come from case reports. We present the case of a patient who suffered Kounis syndrome with cardiogenic shock and asystole after intravenous infusion of Metamizole, and in which no lesions were observed in coronariography.

  3. Jacobsen syndrome: report of a patient with severe eye anomalies, growth hormone deficiency, and hypothyroidism associated with deletion 11 (q23q25) and review of 52 cases.

    OpenAIRE

    Pivnick, E K; Velagaleti, G V; Wilroy, R S; Smith, M E; Rose, S R; Tipton, R E; Tharapel, A T

    1996-01-01

    We have evaluated a patient with Jacobsen syndrome. The patient presented with growth retardation, hypotonia, trigonocephaly, telecanthus, downward slanting palpebral fissures, bilateral inferior colobomas (of the iris, choroid, and retina), hydrocephalus, central nervous system (CNS) abnormalities, and an endocardial cushion defect, features commonly seen in Jacobsen syndrome. Endocrine evaluation showed growth hormone deficiency and central hypothyroidism. Chromosome analysis showed a 46,XX...

  4. Prenatal management and perinatal outcome in giant placental chorioangioma complicated with hydrops fetalis, fetal anemia and maternal mirror syndrome

    Directory of Open Access Journals (Sweden)

    García-Díaz Lutgardo

    2012-07-01

    Full Text Available Abstract Background Giant placental chorioangiomas have been associated with a number of severe fetal complications and high perinatal mortality. Case presentation We report a case of giant chorioangioma with fetal hydrops, additionally complicated by severe anemia, mild cardiomegaly with hyperdinamic heart circulation and maternal mirror syndrome. Intrauterine blood transfusion and amniodrainage was performed at 29 weeks. Worsening of the fetal and maternal condition prompted us to proceed with delivery at 29 + 5 weeks. The newborn died 3 hours later due to pulmonary hypoplasia and hemodynamic failure. Maternal course was favourable, mirror syndrome resolved in the second day and the patient was discharged four days following delivery. Conclusions In the case described here, fetal condition got worse despite of the anemia correction and amniodrainage. Our outcome raises the issue whether additional intrauterine clinical intervention, as intersticial laser, should have been performed to stop further deterioration of the fetal condition when progressive severe hydrops develops.

  5. Case reports Juvenile idiopathic arthritis complicated by amyloidosis with secondary nephrotic syndrome – effective treatment with tocilizumab

    Directory of Open Access Journals (Sweden)

    Małgorzata Kwiatkowska

    2015-08-01

    Full Text Available A case report of a boy with juvenile idiopathic arthritis since the age of 2 years, generalized onset, complicated by nephrotic syndrome due to secondary type A amyloidosis is presented. In the patient the disease had an especially severe course, complicated by frequent infections, making routine treatment difficult. Amyloidosis was diagnosed in the 5th year of the disease based on a rectal biopsy. Since the disease onset the boy has been taking prednisolone and sequentially cyclosporine A, methotrexate, chlorambucil, etanercept, and cyclophosphamide. Clinical and laboratory remission was observed after treatment with tocilizumab. After 42 months of treatment with tocilizumab the boy’s condition is good. There is no pain or joint edema, and no signs of nephrotic syndrome.

  6. Milk alkali syndrome—an unusual syndrome causing an unusual complication

    OpenAIRE

    George, S.; Clark, J.

    2000-01-01

    Milk alkali syndrome is rare and although pancreatitis secondary to hypercalcaemia is well recognised, there has only been one other reported case of pancreatitis secondary to the milk alkali syndrome. Such a case, caused by self medication of over the counter medication, is reported.


Keywords: milk alkali syndrome; pancreatitis; over the counter medication

  7. Male Child with Van Wyk-Grumbach's Syndrome and Other Complications of Long-Standing Primary Hypothyroidism: A Case Report

    OpenAIRE

    Ahmed Omran; Jing Peng; Biswas Shrestha; Muhammad Usman Ashhab; Fei Yin

    2012-01-01

    Primary hypothyroidism in the juvenile population generally leads to retardation of linear growth and delay or even arrest of puberty. However, in rare conditions, children with long-standing hypothyroidism present with signs of Van Wyk-Grumbach's syndrome (VWGS) which include juvenile hypothyroidism, delayed bone age, and pseudoprecocious puberty. We report a rare case of prepubertal male child from Asian origin, presented with long-standing untreated hypothyroidism complicated with VWGS and...

  8. Systemic inflammation: a key factor in the pathogenesis of cardiovascular complications in obstructive sleep apnoea syndrome?

    LENUS (Irish Health Repository)

    Ryan, S

    2012-02-01

    Obstructive sleep apnoea syndrome (OSAS) is a highly prevalent disease and is recognised as a major public health burden. Large-scale epidemiological studies have demonstrated an independent relationship between OSAS and various cardiovascular disorders. The pathogenesis of cardiovascular complications in OSAS is not completely understood but a multifactorial aetiology is likely. Inflammatory processes have emerged as critical in the pathogenesis of atherosclerosis at all stages of atheroma formation. Increased levels of various circulating markers of inflammation including tumour necrosis factor alpha (TNFalpha), interleukin 6 (IL6), IL-8 and C-reactive protein (CRP) have been reported as associated with future cardiovascular risk. There is increasing evidence of elevated inflammatory markers in OSAS with a significant fall after effective treatment with continuous positive airway pressure. This evidence is particularly strong for TNFalpha, whereas studies on IL6 and CRP have yielded conflicting results possibly due to the confounding effects of obesity. Cell culture and animal studies have significantly contributed to our understanding of the underlying mechanisms of the association between OSAS and inflammation. Intermittent hypoxia, the hallmark of OSAS, results in activation of pro-inflammatory transcription factors such as nuclear factor kappa B (NF-kappaB) and activator protein (AP)-1. These promote activation of various inflammatory cells, particularly lymphocytes and monocytes, with the downstream consequence of expression of pro-inflammatory mediators that may lead to endothelial dysfunction. This review provides a critical analysis of the current evidence for an association between OSAS, inflammation and cardiovascular disease, discusses basic mechanisms that may be responsible for this association and proposes future research possibilities.

  9. Single minimal incision fasciotomy for the treatment of chronic exertional compartment syndrome: outcomes and complications.

    Science.gov (United States)

    Drexler, Michael; Rutenberg, T Frenkel; Rozen, N; Warschawski, Y; Rath, E; Chechik, O; Rachevsky, G; Morag, G

    2017-01-01

    Chronic exertional compartment syndrome (CECS) is a common injury in young athletes, causing pain in the involved leg compartment during strenuous exercise. The gold standard treatment is fasciotomy, but most of the reports on its effectiveness include relatively small cohorts and relatively short follow-up periods. This study reports the long-term results of a large cohort of young athletes who underwent single-incision fasciotomy for CECS. This a retrospective case-series study. All patients treated by fasciotomies performed for CECS between 2007 and 2011, in a tertiary medical institution. CECS was diagnosed following history taking and clinical evaluation, and confirmed by compartment pressure measurements. Ninety-five legs that underwent single-incision subcutaneous fasciotomy were included. Data on the numerical analog scale (NAS), Tegner activity score, and quality-of-life (QOL) as measured via the short form-12 (SF-12) were retrieved from all patients preoperatively and at the end of follow-up. The average time to diagnosis was 22 months and the mean follow-up was 50.1 months. Sixty-three legs underwent anterior compartment fasciotomy (an additional 30 legs also underwent lateral compartment release), and two legs underwent lateral and peroneal compartment releases. The average change in Tegner score was an improvement of 14.6 points. Similarly, the patients reported a significant improvement in the SF-12 and NAS scores. Satisfaction rates were high (average 75.5 %). The main complications were wound infection (2 patients) and nerve injuries (4 patients). Eight patients had recurrence. Single-incision fasciotomy leads to long-term improvement in the activity level and QOL of patients with CECS.

  10. Cardiac complications and immunophenotypic profile of infectious mononucleosis syndrome in children.

    Science.gov (United States)

    Papadopoulou-Legbelou, Kyriaki; Papadopoulou-Alataki, Efimia; Fleva, Alexandra; Spanou, Sofia; Pavlitou, Aikaterini; Varlamis, George

    2012-03-01

    To investigate cardiac complications in infectious mononucleosis patients and to associate them with biochemical and immunological parameters, as well as with spleen ultrasound findings. Cross-sectional study with follow-up. Tertiary care pediatric unit, in the city of Thessaloniki, Greece. Twenty-five children (15 boys, aged 1-11.6 years) suffering from infectious mononucleosis were studied during the acute phase and after 3-6 months. Cardiac evaluation comprised of electrocardiogram, echocardiogram, and measurement of creatine phosphokinase, creatine phosphokinase cardiac isoenzyme, and troponin levels. Biochemical and immunological tests included serum transaminases, serum amylase, CD3+/CD8+ T-lymphocytes subpopulation and CD4+/CD8+ T-lymphocytes ratio. During acute phase, all children had splenomegaly and normal serum amylase values. 17 patients had elevated serum transaminases. Percentages of CD3+/CD8+ T-lymphocytes subpopulation were elevated and CD4+/CD8+ ratio was decreased in all patients. Echocardiography revealed mild pericardial effusion in 13 patients (10/21 with Epstein-Barr infection, 3/4 with cytomegalovirus infection), but none presented with myocarditis. Four out of these 13 patients also had markedly elevated liver enzymes, 10/13 had significant splenomegaly and 12/13 presented very low CD4+/CD8+ T-lymphocytes ratio. Pericardial effusion demonstrated a statistically significant association solely with very low CD4+/CD8+ T-lymphocytes ratio (mononucleosis syndrome, asymptomatic pericardial effusion could be associated with very low CD4+/CD8+ ratio (<0.5). Further studies would extend and confirm such an association.

  11. A Case of Crescentic Glomerulonephritis Complicated with Hypocomplementemic Urticarial Vasculitis Syndrome and ANCA-Associated Vasculitis

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    Marenao Tanaka

    2017-11-01

    Full Text Available Systemic urticaria in a 64-year-old woman was diagnosed as leukocytoclastic vasculitis by a punch biopsy of the skin. Her physical findings improved after prescription of prednisolone at a dose of 20 mg/day, but the skin rash relapsed with renal dysfunction, proteinuria, and hematuria when the dose of prednisolone was reduced over a period of 9 months to 1 mg/day. She was admitted to our institute for further examination, when urinary protein and plasma creatinine levels were 0.8 g/day and 1.7 mg/dL, respectively. Complement analysis showed that levels of total hemolytic component, component C3 fraction, and component C4 fraction were 30∼60% of normal values and the titer of anti-neutrophil cytoplasmic antibody for myeloperoxidase (MPO-ANCA was 89 EU (normal range, <10 EU, though there were no immunologic disorders such as systemic lupus erythematosus. Cellular crescentic glomerulonephritis was observed by light microscopy, and immunofluorescent studies showed positive staining for IgG, IgM, C3, C4, and C1q. Electron microscopy showed mesangial and subendothelial deposits with circumferential mesangial interposition. She fulfilled the diagnostic criteria for hypocomplementemic urticarial vasculitis syndrome (HUV, and ANCA-associated vasculitis (AAV was also indicated by small vessel vasculitis and positive MPO-ANCA. Steroid pulse therapy with methylprednisolone followed by oral prednisolone improved her general condition and hypocomplementemia, and MPO-ANCA became negative. HUV and AAV are distinct clinical disorders, though both affect small blood vessels. Here we report a case of AAV-complicated HUV with crescentic glomerulonephritis.

  12. Venous and arterial thrombo-embolic complications of hormonal treatment in a male-to-female transgender patient.

    LENUS (Irish Health Repository)

    Mullins, G M

    2012-02-03

    We present a male-to-female (MTF) transgender patient admitted with a pulmonary embolism. The patient had been treated with high-dose oestrogens since the age of 16. Following a prolonged period of hypotension, our patient sustained cerebral border zone infarcts. There was evidence of bilateral carotid stenosis on Doppler ultrasound. We discuss the treatment and vascular complications of gender dysphoria.

  13. Growth hormone therapy for Prader–Willi syndrome: challenges and solutions

    Directory of Open Access Journals (Sweden)

    Grugni G

    2016-06-01

    Full Text Available Graziano Grugni,1,2 Alessandro Sartorio,1,2 Antonino Crinò3 1Division of Auxology, 2Experimental Laboratory for Auxo-endocrinological Research, San Giuseppe Hospital, Istituto Auxologico Italiano, Verbania, 3Autoimmune Endocrine Diseases Unit, Bambino Gesù Children’s Hospital, Rome, Italy Abstract: Prader–Willi syndrome (PWS is characterized by a dysregulation of growth hormone (GH/insulin-like growth factor I axis, as the consequence of a complex hypothalamic involvement. PWS’ clinical picture seems to resemble the classic non-PWS GH deficiency (GHD, including short stature, excessive body fat, decreased muscle mass, and impaired quality of life. GH therapy is able to ameliorate the phenotypic appearance of the syndrome, as well as to improve body composition, physical strength, and cognitive level. In this regard, however, some pathophysiologic and clinical questions still remain, representing a challenge to give the most appropriate care to PWS patients. Data about the prevalence of GHD in PWS children are not unequivocal, ranging from 40% to 100%. In this context, to establish whether the presence (or not of GHD may have a different effect on clinical course during GH therapy may be helpful. In addition, the comparison of GH effects in PWS children diagnosed as small for gestational age with those obtained in subjects born appropriate for gestational age is of potential interest for future trials. Emerging information seems to demonstrate the maintenance of beneficial effects of GH therapy in PWS subjects after adolescent years. Thus, GH retesting after achievement of final height should be taken into consideration for all PWS patients. However, it is noteworthy that GH administration exerts positive effects both in PWS adults with and without GHD. Another critical issue is to clarify whether the genotype–phenotype correlations may be relevant to specific outcome measures related to GH therapy. Moreover, progress of our

  14. Cerebellar Mutism Syndrome and Other Complications After Surgery in the Posterior Fossa in Adults

    DEFF Research Database (Denmark)

    Wibroe, Morten; Rochat, Per; Juhler, Marianne

    2018-01-01

    only for speech and motor complications (P value = 0.01 and 0.02). Speech and language complications occurred more frequently in midline tumors compared with lateral tumors (40% vs. 7%; P = 0.004). Surgical complications were similar to other studies. CONCLUSIONS: We propose that speech and language...... of the few known risk factors for CMS in children. Thus, the cerebellar midline seems to be a vulnerable region for speech and language complications also in adults....

  15. Accurate long-term prediction of height during the first four years of growth hormone treatment in prepubertal children with growth hormone deficiency or Turner Syndrome.

    Science.gov (United States)

    Ranke, Michael B; Lindberg, Anders; Brosz, Mathias; Kaspers, Stefan; Loftus, Jane; Wollmann, Hartmut; Kołtowska-Haggstrom, Maria; Roelants, Mathieu

    2012-01-01

    The study aim was to develop and validate models for long-term prediction of growth in prepubertal children with idiopathic growth hormone deficiency (GHD) or Turner syndrome (TS) for optimal, cost-effective growth hormone (GH) therapy. Height was predicted by sequential application of annual prediction algorithms for height velocity in cohorts of GHD (n = 664) and TS (n = 607) as documented within KIGS (Pfizer International Growth Database). As height prediction models also require an estimate of weight, new algorithms for weight increase during the first to fourth prepubertal years on GH were developed. When height was predicted from the start of GH treatment, the predicted and observed mean (SD) gain over 4 years was 30.4 (3.4) cm and 30.1 (4.9) cm, respectively, in GHD patients, and 27.2 (2.2) cm and 26.6 (3.5) cm, respectively, in TS patients. For all 4 years, gains of weight SD scores (SDS) were accurately described as a function of weight SDS and observed gain in height SDS (R(2) > 0.89). In GHD and TS patients treated with GH, an accurate prepubertal long-term prediction of height development in groups is possible. Based on this, an optimal individual height outcome could be simulated. Copyright © 2012 S. Karger AG, Basel.

  16. Limited Diagnostic Utility of Plasma Adrenocorticotropic Hormone for Differentiation between Adrenal Cushing Syndrome and Cushing Disease.

    Science.gov (United States)

    Hong, A Ram; Kim, Jung Hee; Hong, Eun Shil; Kim, I Kyeong; Park, Kyeong Seon; Ahn, Chang Ho; Kim, Sang Wan; Shin, Chan Soo; Kim, Seong Yeon

    2015-09-01

    Measurement of the plasma adrenocorticotropic hormone (ACTH) level has been recommended as the first diagnostic test for differentiating between ACTH-independent Cushing syndrome (CS) and ACTH-dependent CS. When plasma ACTH values are inconclusive, a differential diagnosis of CS can be made based upon measurement of the serum dehydroepiandrosterone sulfate (DHEA-S) level and results of the high-dose dexamethasone suppression test (HDST). The aim of this study was to assess the utility of plasma ACTH to differentiate adrenal CS from Cushing' disease (CD) and compare it with that of the HDST results and serum DHEA-S level. We performed a retrospective, multicenter study from January 2000 to May 2012 involving 92 patients with endogenous CS. The levels of plasma ACTH, serum cortisol, 24-hour urine free cortisol (UFC) after the HDST, and serum DHEA-S were measured. Fifty-seven patients had adrenal CS and 35 patients had CD. The area under the curve of plasma ACTH, serum DHEA-S, percentage suppression of serum cortisol, and UFC after HDST were 0.954, 0.841, 0.950, and 0.997, respectively (all P<0.001). The cut-off values for plasma ACTH, percentage suppression of serum cortisol, and UFC after HDST were 5.3 pmol/L, 33.3%, and 61.6%, respectively. The sensitivity and specificity of plasma ACTH measurement were 84.2% and 94.3%, those of serum cortisol were 95.8% and 90.6%, and those of UFC after the HDST were 97.9% and 96.7%, respectively. Significant overlap in plasma ACTH levels was seen between patients with adrenal CS and those with CD. The HDST may be useful in differentiating between these forms of the disease, especially when the plasma ACTH level alone is not conclusive.

  17. Growth hormone therapy of Turner's syndrome: beneficial effect on adult height.

    Science.gov (United States)

    Rosenfeld, R G; Attie, K M; Frane, J; Brasel, J A; Burstein, S; Cara, J F; Chernausek, S; Gotlin, R W; Kuntze, J; Lippe, B M; Mahoney, C P; Moore, W V; Saenger, P; Johanson, A J

    1998-02-01

    To carry out a multicenter, prospective, randomized trial of human growth hormone (GH), alone or in combination with oxandrolone (OX), in patients with Turner's syndrome (TS). In an initial phase lasting 12 to 24 months, 70 girls with TS, verified by karyotype, were randomly assigned to one of four groups: (1) observation, (2) OX, (3) GH, or (4) GH plus OX. After completion of the first phase, group 3 subjects continued to receive GH only. All other subjects were treated with GH plus OX. Subjects were followed up until attainment of adult height and/or cessation of treatment. Data from this trial were compared with growth characteristics of 25 American historical subjects with TS (matched for age, height, parental target height, and karyotype) who never received either GH or androgens. Of the 70 subjects enrolled, 60 completed the clinical trial. The 17 subjects receiving GH alone all completed the trial and reached a height of 150.4+/-5.5 cm (mean +/- SD), 8.4+/-4.5 cm taller than their mean projected adult height at enrollment (95% confidence interval [CI]: 6.3 to 10.6 cm). The 43 subjects receiving GH plus OX attained a mean height of 152.1+/-5.9 cm, 10.3+/-4.7 cm taller than their mean projected adult height (95% CI: 8.9 to 11.7 cm). The historical control subjects had a mean adult height of 144.2+/-6.0 cm, precisely matching their original projected adult height of 144.2+/-6.1 cm. GH, either alone or in combination with OX, is capable of stimulating short-term growth and augmenting adult height in girls with TS. With early diagnosis and initiation of treatment, an adult height of more than 150 cm is a reasonable goal for most girls with TS.

  18. A possible link between luteinizing hormone and macrophage migration inhibitory factor levels in polycystic ovary syndrome.

    Science.gov (United States)

    Calan, Mehmet; Kume, Tuncay; Yilmaz, Ozgur; Arkan, Tugba; Kocabas, Gokcen Unal; Dokuzlar, Ozge; Aygün, Kemal; Oktan, Mehmet Asi; Danıs, Nilay; Temur, Muzaffer

    2016-08-01

    Macrophage migration inhibitory factor (MIF) is a multifunctional cytokine that plays a role in metabolic and inflammatory processes. Increasing evidence suggests that there is a link between MIF and ovulation. We aimed to evaluate plasma MIF levels in women with polycystic ovary syndrome (PCOS) and to determine whether MIF levels differ between the follicular phase and mid-cycle of the menstrual cycle in eumenorrheic women. Ninety women with PCOS and 80 age- and BMI-matched healthy eumenorrheic women were consecutively recruited into this prospective observational study. For all subjects, plasma MIF levels in the early follicular phase were measured by ELISA; for the 40 healthy controls, MIF levels were also measured during mid-cycle of the same menstrual cycle. Plasma MIF levels were significantly higher in women with PCOS than in eumenorrheic women (14.16 ± 1.59 vs. 10.39 ± 0.70 ng/ml; p < 0.001). MIF levels were significantly higher at mid-cycle than in the follicular phase in eumenorrheic women (11.15 ± 0.61 vs. 10.56 ± 0.82 ng/ml; p < 0.001). MIF was positively correlated with BMI, high sensitivity C-reactive protein (hs-CRP), and homeostasis model assessment of insulin resistance (HOMA-IR) in both groups. MIF was positively correlated with luteinizing hormone (LH) and free-testosterone only in the PCOS group. Binary logistic regression analyses revealed that the odds ratio (OR) for PCOS independently increases linearly with elevated MIF (OR = 1.385, 95% CI = 1.087-1.764, p = 0.017). MIF may play a crucial role in the reproductive system in women, including the development of PCOS and normal ovulation.

  19. Comparative Analysis of Psychological, Hormonal, and Genetic Factors Between Burning Mouth Syndrome and Secondary Oral Burning.

    Science.gov (United States)

    das Neves de Araújo Lima, Emeline; Barbosa, Natália Guimarães; Dos Santos, Ana Celly Souza; AraújoMouraLemos, Telma Maria; de Souza, Cleber Machado; Trevilatto, Paula Cristina; da Silveira, Ericka Janine Dantas; de Medeiros, Ana Miryam Costa

    2016-09-01

    The objective of this study was to evaluate the association between psychological, hormonal, and genetic factors with the development of burning mouth syndrome (BMS) and secondary oral burning (SOB) in order to provide a better characterization and classification of these conditions. Cross sectional study. Patients with complaints of mouth burning registered at the Oral Diagnostic Service of the Federal University of Rio Grande do Norte between 2000 and 2013. The sample consisted of 163 subjects divided into a group of patients with BMS (n = 64) and a group of subjects with SOB (n = 99). The following variables were analyzed: passive and stimulated saliva flow, stress levels and phase, depression, anxiety, serum cortisol and dehydroepiandrosterone (DHEA) levels, and the presence of polymorphisms in the interleukin 6 (IL-6) gene. The results showed significant differences in the presence of xerostomia (p = 0.01), hyposalivation at rest (p < 0.001) and symptoms of depression (p = 0.033) between the two groups, which were more prevalent in the BMS group. DHEA levels were lower in the BMS group (p = 0.003) and were sensitive and specific for the diagnosis of this condition. Genetic analysis revealed no significant association between the polymorphisms analyzed and the development of BMS. These results suggest a possible role of depression, as well as of reduced DHEA levels, as associated factors for development of BMS. © 2016 American Academy of Pain Medicine. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

  20. Limited Diagnostic Utility of Plasma Adrenocorticotropic Hormone for Differentiation between Adrenal Cushing Syndrome and Cushing Disease

    Directory of Open Access Journals (Sweden)

    A Ram Hong

    2015-09-01

    Full Text Available BackgroundMeasurement of the plasma adrenocorticotropic hormone (ACTH level has been recommended as the first diagnostic test for differentiating between ACTH-independent Cushing syndrome (CS and ACTH-dependent CS. When plasma ACTH values are inconclusive, a differential diagnosis of CS can be made based upon measurement of the serum dehydroepiandrosterone sulfate (DHEA-S level and results of the high-dose dexamethasone suppression test (HDST. The aim of this study was to assess the utility of plasma ACTH to differentiate adrenal CS from Cushing' disease (CD and compare it with that of the HDST results and serum DHEA-S level.MethodsWe performed a retrospective, multicenter study from January 2000 to May 2012 involving 92 patients with endogenous CS. The levels of plasma ACTH, serum cortisol, 24-hour urine free cortisol (UFC after the HDST, and serum DHEA-S were measured.ResultsFifty-seven patients had adrenal CS and 35 patients had CD. The area under the curve of plasma ACTH, serum DHEA-S, percentage suppression of serum cortisol, and UFC after HDST were 0.954, 0.841, 0.950, and 0.997, respectively (all P<0.001. The cut-off values for plasma ACTH, percentage suppression of serum cortisol, and UFC after HDST were 5.3 pmol/L, 33.3%, and 61.6%, respectively. The sensitivity and specificity of plasma ACTH measurement were 84.2% and 94.3%, those of serum cortisol were 95.8% and 90.6%, and those of UFC after the HDST were 97.9% and 96.7%, respectively.ConclusionSignificant overlap in plasma ACTH levels was seen between patients with adrenal CS and those with CD. The HDST may be useful in differentiating between these forms of the disease, especially when the plasma ACTH level alone is not conclusive.

  1. Corticotropin-releasing hormone receptor 1 gene variants in irritable bowel syndrome.

    Directory of Open Access Journals (Sweden)

    Naoko Sato

    Full Text Available BACKGROUND: Corticotropin-releasing hormone (CRH acts mainly via the CRH receptor 1 (CRH-R1 and plays a crucial role in the stress-induced pathophysiology of irritable bowel syndrome (IBS. Several studies have demonstrated that variants of the CRH-R1 gene carry a potential risk for depression, but evidence for an association between CRH-R1 genotypes and IBS is lacking. We tested the hypothesis that genetic polymorphisms and haplotypes of CRH-R1 moderate the IBS phenotype and negative emotion in IBS patients. METHODS: A total of 103 patients with IBS and 142 healthy controls participated in the study. Three single-nucleotide polymorphisms of the CRH-R1 gene (rs7209436, rs242924, and rs110402 were genotyped. Subjects' emotional states were evaluated using the Perceived-Stress Scale, the State-Trait Anxiety Inventory, and the Self-rating Depression Scale. RESULTS: The TT genotype of rs7209436 (P = 0.01 and rs242924 (P = 0.02 was significantly more common in patients with IBS than in controls. Total sample analysis showed significant association between bowel pattern (normal, diarrhea, constipation, or mixed symptoms and the T allele of rs7209436 (P = 0.008, T allele of rs242924 (P = 0.019, A allele of rs110402 (P = 0.047, and TAT haplocopies (P = 0.048. Negative emotion was not associated with the examined CRH-R1 SNPs. CONCLUSION: These findings suggest that genetic polymorphisms and the CRH-R1 haplotypes moderate IBS and related bowel patterns. There was no clear association between CRH-R1 genotypes and negative emotion accompanying IBS. Further studies on the CRH system are therefore warranted.

  2. [Effect of Transcutaneuos Acupoint Electrostimulation on Serum Sex Hormone Levels and Expression of Ovarian Steroid Hormone Metabolic Enzymes in Polycystic Ovary Syndrome Rats].

    Science.gov (United States)

    Zhou, Jian-yong; Zhang, Xiao-yue; Yu, Mei-ling; Lu, Sheng-feng; Chen, Xia

    2016-02-01

    To observe the effect of transcutaneuos acupoint electrostimulation(TAES) on ovarian serum sex hormone levels and ovarian follicle granular cell aromatase cytochrome P 450 (P 450 arom) protein and follicle theca cell cytochrome P 450 17 α-hydroxylase/c 17-20 lyase cytochrome P 450 (P 450 c 17 α) protein expression in polycystic ovary syndrome (PCOS)rats, so as to explore its mechanisms underlying improvement of PCOS. METHODS Forty SD rats were randomly divided into four groups: normal control, model, medication and TAES (10 rats/group). The PCOS model was established by giving (gavage) the animals with letrozole solution (1.0 mg/kg, once daily for 21 consecutive days). Rats of the medication group were treated with Clomiphene (1 mg/kg) once daily for 7 days, and those of the TAES group were treated with electrical stimulation (2 Hz, 3 mA) of "Guanyuan" (CV 4) and "Sanyinjiao" (SP 6) areas for 30 min, once daily for 7 consecutive days. The rats body weight and bilateral ovarian weight were detected, and the ovarian structure and follicular development degree were observed under light microscope after H. E. stain, and the serum testosterone (T), estradiol (E2), luteotrophic hormone (LH) and follicle-stimulating hormone (FSH) contents were detected using radioimmunoassay. The expression of ovarian P 450 arom (for production of estrogen)protein and P 450 c 17 α (for production of androgen) protein was detected by using immunohistochemical stain and Western blot, respectively. The body weight, bilateral ovary weight, serum T and LH contents, and ratio of LH/FSH, and ovarian P 450 c 17 α immunoactivity and protein expression levels in the model group were all significantly increased compared with the normal control group (P ovary weight, serum T and LH contents, ratio of LH/FSH, and ovarian P 450 c 17 α immunoactivity and protein expression levels, and the decreased ovarian P 450 arom immunoactivity and protein expression levels were reversed in the TAES group (P 0

  3. Cardiac complications in a metamizole-induced type I Kounis syndrome.

    Science.gov (United States)

    Juste, Jose F Martínez; Garces, Tomas Ruiz; Enguita, Rafael Gonzalez; Blasco, Pedro Cia; Trallero, Jara Altemir

    2016-01-01

    Kounis syndrome is defined as the coincidental occurrence of allergic reaction and acute coronary syndrome secondary to vasospasm. Anti-inflammatory drugs are included as one of the multiple causes. Current data available about this syndrome come from case reports. We present the case of a patient who suffered Kounis syndrome with cardiogenic shock and asystole after intravenous infusion of Metamizole, and in which no lesions were observed in coronariography. Copyright © 2013 Sociedade Brasileira de Anestesiologia. Published by Elsevier Editora Ltda. All rights reserved.

  4. Thoracic outlet syndrome after the Nuss procedure for pectus excavatum: Is it a rare complication?

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    Nagasao, Tomohisa; Morotomi, Tadaaki; Kuriyama, Motone; Kogure, Tetsukuni; Kudo, Hirro; Hamamoto, Yusuke; Tamai, Motoki

    2017-10-01

    The present study aims to elucidate the frequency of thoracic outlet syndrome after the Nuss procedure for pectus excavatum and the conditions in which thoracic outlet syndrome is likely to develop. A retrospective study including 85 pectus excavatum patients (58 males and 27 females) was conducted. Thoracic outlet syndrome was defined as a condition in which the patient has numbness, lassitude, or pain of the upper limbs at rest or during motion of the upper limbs. The frequency of the thus-defined thoracic outlet syndrome was evaluated in 85 patients. Age, sex, Haller indices, and the positions of the correction bars were compared between the patients who developed thoracic outlet syndrome and those who did not. Preadolescent patients (18 out of 85) did not develop postoperative thoracic outlet syndrome. In total, 15.2% of adult male patients (7 out of 46) and 33% of adult female patients (7 out of 21) developed postoperative thoracic outlet syndrome. For both male and female groups, Haller indices were significantly greater for patients who had postoperative thoracic outlet syndrome than for those who did not. Correction bars were generally placed at higher intercostal spaces in patients who developed postoperative thoracic outlet syndrome than in those who did not. A considerable percentage of adult patients develop thoracic outlet syndrome after the Nuss procedure for pectus excavatum. Maturity of the thoracic wall, femininity, severity of the deformity (represented by greater Haller indices), and placement of correction bars at superior intercostal spaces are risk factors for postoperative thoracic outlet syndrome. Copyright © 2017 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.

  5. [Syndrome of inappropriate antidiuretic hormone secretion following adjuvant CDDP and 5-FU administration in a patient with esophageal carcinoma].

    Science.gov (United States)

    Horio, Takuya; Tsujimoto, Hironori; Akase, Takayoshi; Sakamoto, Naoko; Yaguchi, Yoshihisa; Hiraki, Shuuichi; Aiko, Satoshi; Takechi, Hanako; Hase, Kazuo; Maehara, Tadaaki

    2010-10-01

    A case of hyponatremia following the first course of systemic adjuvant chemotherapy with cisplatin (CDDP) and 5-FU in a previously treated patient with esophageal cancer is reported. A 61-year-old man was admitted to our hospital for adjuvant chemotherapy after transthoracic esophagectomy and 3-field lymphadenectomy for esophageal cancer. Six days following chemotherapy, his serum sodium concentration was found to be 118 mEq/L, without edema or dehydration. This hyponatremic state was diagnosed as the syndrome of inappropriate secretion of antidiuretic hormone (SIADH) induced by CDDP, based on the hypo-osmolality of his serum and urine, and an inappropriate level of plasma vasopressin.

  6. Effects of ginseng on peripheral blood mitochondrial DNA copy number and hormones in men with metabolic syndrome: A randomized clinical and pilot study.

    Science.gov (United States)

    Jung, Dong-Hyuk; Lee, Yong-Jae; Kim, Chun-Bae; Kim, Jang-Young; Shin, Seung-Hun; Park, Jong-Ku

    2016-02-01

    It has been observed that mitochondrial dysfunction is associated with an increased risk of metabolic syndrome. There is growing evidence that hyperactivity of the hypothalamus-pituitary-adrenal (HPA) axis and hormone (testosterone and growth hormone) deficiency may lead to metabolic syndrome. Recent studies have reported that ginseng treatment improves mitochondrial and HPA-axis function and increases anabolic hormone secretion. The objective of this study was to investigate the effect of red ginseng (RG) on metabolic syndrome, hormones, and mitochondrial function using leukocyte mitochondrial DNA copy number in men with metabolic syndrome. We performed a randomized, double-blind, placebo-controlled study in 62 subjects who were not taking drugs that could affect their metabolic function. A total of 62 men with metabolic syndrome were randomly assigned to either an RG group (3.0g/day) or a placebo group for 4 weeks. We analyzed changes in metabolic syndrome components, leukocyte mitochondrial DNA copy number, hormones (total testosterone, IGF-1, cortisol, and DHEAS) and inflammatory markers (C-reactive protein, ferritin) from baseline to 4 weeks. Significant improvement in mitochondrial function (95% CI -44.9 to -1.3) and an increase in total testosterone (95% CI -70.1 to -1.0) and IGF-1(P=0.01) levels were observed in the RG group when compared with the placebo group. Diastolic blood pressure (95% CI 2.0-9.4) and serum cortisol (95% CI 1.1-5.5) significantly decreased in the RG group. We found evidence that RG had a favorable effect on mitochondrial function and hormones in men with metabolic syndrome. Copyright © 2015 Elsevier Ltd. All rights reserved.

  7. Severe Renal Hemorrhage in a Pregnant Woman Complicated with Antiphospholipid Syndrome: A Case Report

    Directory of Open Access Journals (Sweden)

    Shohei Kawaguchi

    2011-01-01

    Full Text Available Antiphospholipid syndrome is a systemic autoimmune disease with thrombotic tendency. Consensus guidelines for pregnancy with antiphospholipid syndrome recommend low-dose aspirin combined with unfractionated or low-molecular-weight heparin because antiphospholipid syndrome causes habitual abortion. We report a 36-year-old pregnant woman diagnosed with antiphospholipid syndrome receiving anticoagulation treatment. The patient developed left abdominal pain and gross hematuria at week 20 of pregnancy. An initial diagnosis of left ureteral calculus was made. Subsequently abdominal-pelvic computed tomography was required for diagnosis because of the appearance of severe contralateral pain. Computed tomography revealed serious renal hemorrhage, and ureteral stent placement and pain control by patient-controlled analgesia were required. After treatment, continuance of pregnancy was possible and vaginal delivery was performed safely. This is the first case report of serious renal hemorrhage in a pregnant woman with antiphospholipid syndrome receiving anticoagulation treatment and is an instructive case for urological and obstetrical practitioners.

  8. Pediatric Miller Fisher Syndrome Complicating an Epstein-Barr Virus Infection.

    Science.gov (United States)

    Communal, Céline; Filleron, Anne; Baron-Joly, Sandrine; Salet, Randa; Tran, Tu-Anh

    2016-10-01

    Miller Fisher syndrome, a variant of Guillain-Barré syndrome, is an acute inflammatory demyelinating polyradiculoneuropathy that may occur weeks after a bacterial or viral infection. Campylobacter jejuni and Haemophilus influenzae are frequently reported etiological agents. We describe a boy with Miller Fisher syndrome following Epstein-002DBarr virus primary infectious mononucleosis. He presented with bilateral dysfunction of several cranial nerves and hyporeflexia of the limbs but without ataxia. Miller Fisher syndrome was confirmed by the presence of anti-GQ1b antibodies in a blood sample. Epstein-Barr virus was identified by polymerase chain reaction and serology. Epstein-Barr virus should be considered as a Miller Fisher syndrome's causative agent. The physiopathology of this condition may involve cross-reactive T-cells against Epstein-Barr virus antigens and gangliosides. Copyright © 2016 Elsevier Inc. All rights reserved.

  9. Analysis of complications of prone position in acute respiratory distress syndrome: quality standard, incidence and related factors.

    Science.gov (United States)

    Jové Ponseti, E; Villarrasa Millán, A; Ortiz Chinchilla, D

    The monitoring system based on standards of quality allows clinicians to evaluate and improve the patient's care. According to the quality indicators recommended by Sociedad Española de Medicina Intensiva Crítica y Unidades Coronarias, and due to the importance of prone position (PP) as a treatment in patients with acute respiratory distress syndrome, it is fundamental to keep accurate record of serious adverse events occurring during the prone position procedure and its posterior analysis. To establish fulfilment of the Sociedad Española de Medicina Intensiva Crítica y Unidades Coronarias standards of quality according to the register of serious complications. To identify the incidence of serious complications registered as well as to identify possible factors related to these complications. Retrospective, cross-sectionsl descriptive study, polyvalent ICU (16 beds). Study population Patients with acute respiratory distress syndrome treated with PP (January 2012-December 2013). Study variables PP recording, accidental extubation, removal of catheters, decubitus ulcers (DU), ETT obstruction, urgency of the procedure, hours in PP, nutritional intake, type of feeding tube, food regurgitation/retention and use of prokinetics/muscle relaxant. The study sample comprised 38 cases, with an adequate record of complications in 92.1% of the cases. DU were the only serious complication recorded, with a 25.7% incidence. Possible factors related to DU: more hours in PP in patients developing DU (p= .067). Less incidence of DU in well-nourished patients (p= .577). 82.9% of patients were not appropriately nourished. The percentage of records duly completed is very high. The presence of DU (grade 1-2 mostly) is to be noted. There is no stastistical significance, although a trend is obversed, between DU and hours in PP. Copyright © 2017 Sociedad Española de Enfermería Intensiva y Unidades Coronarias (SEEIUC). Publicado por Elsevier España, S.L.U. All rights reserved.

  10. Ketogenic Diet and Hormonal Therapy in Prevention of Evolution of West Syndrome to Lennox-Gastaut

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2009-08-01

    Full Text Available Medical records of 98 patients diagnosed with West syndrome and monitored at Sanggye Paik Hospital, Seoul, Korea, for at least 3 years were retrospectively reviewed to assess etiology, age at onset, value of various therapies, and the rate of evolution from West syndrome to Lennox-Gastaut syndrome.

  11. [Association between moderate-severe bronchiolitis and syndrome of inappropriate antidiuretic hormone secretion in emergency departments].

    Science.gov (United States)

    Toledo del Castillo, B; González Ruiz de León, E; Rivas García, A; Vázquez López, P; Miguez Navarro, M C; Marañón Pardillo, R

    2016-01-01

    To identify clinical characteristics that may lead to the early recognition of patients admitted to the hospital for moderate-to-severe bronchiolitis with urine results associated with the syndrome of inappropriate antidiuretic hormone secretion (SIADH). A prospective observational study was conducted, spanning the bronchiolitis epidemic season (October 2012-March 2013), including all children who were admitted to the hospital with a diagnosis of moderate-to-severe bronchiolitis. The following criteria were used to establish a diagnosis of SIADH: urine sodium level of 40 mmol/L or greater, urine osmolarity above 500 mosm/Kg, and urine density of 1020 g/L or greater. Demographic characteristics, ventilation mode and clinical outcome were also analyzed. A comparison was made between those patients that met urine SIADH criteria and those who did not. A total of 126 children were included, and 23 (18.6%) of them had urine SIADH criteria. Patients in this group had a higher incidence of pneumonia and/or atelectasis on chest X-Ray (21.7% vs. 1.9%, P=.002), worse response to bronchodilator treatment with nebulized adrenaline (69,5% vs. 28,1%, P=.016), more need for respiratory assistance (high flow oxygen therapy (17.4% vs. 7.7%, p=.016), or non-invasive mechanical ventilation (13% vs. 5.8%, P=.034), and more admissions to the PICU (26.1% vs. 6.8%, P=.007). Patients older than one month with acute moderate bronchiolitis and urine SIADH criteria have worse clinical courses and more need for non-invasive mechanical ventilation, PICU admission, and have a higher incidence of pneumonia on chest X-ray. For that reason, it is recommended to collect a urine sample from these patients to allow an early diagnosis of SIADH, and thus early treatment of fluid and electrolyte abnormalities. Copyright © 2014 Asociación Española de Pediatría. Published by Elsevier España, S.L.U. All rights reserved.

  12. Clinical and endocrine characteristics in atypical and classical growth hormone insensitivity syndrome.

    Science.gov (United States)

    Burren, C P; Woods, K A; Rose, S J; Tauber, M; Price, D A; Heinrich, U; Gilli, G; Razzaghy-Azar, M; Al-Ashwal, A; Crock, P A; Rochiccioli, P; Yordam, N; Ranke, M B; Chatelain, P G; Preece, M A; Rosenfeld, R G; Savage, M O

    2001-01-01

    Classical growth hormone insensitivity syndrome (GHIS) comprises a dysmorphic phenotype, extreme short stature (height SDS low IGF-I and IGFBP-3. Wide clinical variation is recognised with classical and atypical forms. We aimed to delineate features of the milder "atypical" GHIS phenotype, and to determine whether this correlates with milder auxological and biochemical features. Fifty-nine patients from a European series of 82 patients with GHIS, with strict diagnostic criteria of GHIS, were studied and assigned to classical or atypical GHIS groups according to facial phenotype, i.e. "classical" required 2 of 3 recognized GHIS features (frontal bossing, mid-facial hypoplasia and depressed nasal bridge), "atypical" required 0 or 1 of these facial features. Classical and atypical GHIS groups were compared in terms of (1) phenotypic features, including high-pitched voice, sparse hair, blue sclera, hypoglycaemia, microphallus, (2) birth length, height SDS, and (3) basal IGF-I, IGF-II, IGFBP-1, IGFBP-3, GHBP and increase in IGF-I on IGF-I generation testing. Fifty patients [24 males, 26 females, aged 8.6 +/- 4.6 years (mean +/- SD)] had "classical GHIS", 9 patients (7 males, 2 females, aged 7.8 +/- 4.1 years) had "atypical GHIS", 7 with normal facies. Atypical GHIS patients had lesser height deficit (Ht SDS -4.0 +/- 1.4) compared to classical GHIS (-6.7 +/- 1.4), less reduction in IGFBP-3 SDS (atypical -5.5 +/- 3.3; classical -8.6 +/- 2.4), and more had normal GHBP (>10% binding). Other variables were also less frequent in atypical GHIS patients: high-pitched voice 11% (70% classical), sparse hair 11% (42% classical), blue sclera 0% (38% classical), hypoglycaemia 11% (42% classical), and microphallus 14% (1 of 7 males), compared to 79% of classical (19 of 24 males). Atypical GHIS patients, with relatively normal facial appearance, demonstrate less height defect and biochemical abnormalities compared to classical patients. GH insensitivity may be present in children with

  13. The effect of oxandrolone on voice frequency in growth hormone-treated girls with Turner syndrome.

    Science.gov (United States)

    Menke, Leonie A; Sas, Theo C J; van Koningsbrugge, Sophie H L; de Ridder, Maria A J; Zandwijken, Gladys R J; Boersma, Bart; Dejonckere, Philippe H; de Muinck Keizer-Schrama, Sabine M P F; Otten, Barto J; Wit, Jan M

    2011-09-01

    Oxandrolone (Ox) increases height gain but may also cause voice deepening in growth hormone (GH)-treated girls with Turner syndrome (TS). We assessed the effect of Ox on objective and subjective speaking voice frequency in GH-treated girls with TS. A multicenter, randomized, placebo (Pl)-controlled, double-blind study was conducted. One hundred thirty-three patients were included and treated with GH (1.33 mg/m2/d) from baseline, combined with Pl or Ox in a low (0.03 mg/kg/d) or conventional (0.06 mg/kg/d) dose from the age of 8 years and estrogens from the age of 12 years. Yearly from starting Ox/Pl until 6 months after discontinuing GH+Ox/Pl, voices were recorded and questionnaires were completed. At start, mean (±standard deviation [SD]) voice frequency SD score (SDS) was high for age (1.0±1.2, Pvoices tended to lower on GH+Ox 0.03 (P=0.09) and significantly lowered on GH+Ox 0.06 (P=0.007). At the last measurement, voice frequency SDS was still relatively high in GH+Pl group (0.6±0.7, P=0.002) but similar to healthy girls in both GH+Ox groups. Voice frequency became lower than -2 SDS in one patient (3%) on GH+Ox 0.03 and three patients (11%) on GH+Ox 0.06. The percentage of patients reporting subjective voice deepening was similar between the dosage groups. Untreated girls with TS have relatively high-pitched voices. The addition of Ox to GH decreases voice frequency in a dose-dependent way. Although most voice frequencies remain within the normal range, they may occasionally become lower than -2 SDS, especially on GH+Ox 0.06 mg/kg/d. Copyright © 2011 The Voice Foundation. Published by Mosby, Inc. All rights reserved.

  14. Corticotropin-Releasing Hormone Receptor 2 Gene Variants in Irritable Bowel Syndrome.

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    Hazuki Komuro

    Full Text Available Corticotropin-releasing hormone (CRH plays an important role in the pathophysiology of irritable bowel syndrome (IBS and regulates the stress response through two CRH receptors (R1 and R2. Previously, we reported that a CRHR1 gene polymorphism (rs110402, rs242924, and rs7209436 and haplotypes were associated with IBS. However, the association between the CRHR2 gene and IBS was not investigated. We tested the hypothesis that genetic polymorphisms and haplotypes of CRHR2 are associated with IBS pathophysiology and negative emotion in IBS patients.A total of 142 IBS patients and 142 healthy controls participated in this study. Seven single nucleotide polymorphisms (SNPs of the CRHR2 gene (rs4722999, rs3779250, rs2240403, rs2267710, rs2190242, rs2284217, and rs2284220 were genotyped. Subjects' psychological states were evaluated using the Perceived-Stress Scale, the State-Trait Anxiety Inventory, and the Self-Rating Depression Scale.We found that rs4722999 and rs3779250, located in intronic region, were associated with IBS in terms of genotype frequency (rs4722999: P = 0.037; rs3779250: P = 0.017 and that the distribution of the major allele was significantly different between patients and controls. There was a significant group effect (controls vs. IBS, and a CRHR2 genotype effect was observed for three psychological scores, but the interaction was not significant. We found a haplotype of four SNPs (rs4722999, rs3779250, rs2240403, and rs2267710 and two SNPs (rs2284217 and rs2284220 in strong linkage disequilibrium (D' > 0.90. We also found that haplotypes of the CRHR2 gene were significantly different between IBS patients and controls and that they were associated with negative emotion.Our findings support the hypothesis that genetic polymorphisms and haplotypes of CRHR2 are related to IBS. In addition, we found associations between CRHR2 genotypes and haplotypes and negative emotion in IBS patients and controls. Further studies on IBS and the CRH

  15. Clinical guidelines for management of diabetes insipidus and syndrome of inappropriate antidiuretic hormone secretion after pituitary surgery.

    Science.gov (United States)

    Lamas, Cristina; del Pozo, Carlos; Villabona, Carles

    2014-04-01

    Changes in water metabolism and regulation of vasopressin (AVP) or antidiuretic hormone (ADH) are common complications of pituitary surgery. The scarcity of studies comparing different treatment and monitoring strategies for these disorders and the lack of prior clinical guidelines makes it difficult to provide recommendations following a methodology based on grades of evidence. This study reviews the pathophysiology of diabetes insipidus and inappropriate ADH secretion after pituitary surgery, and is intended to serve as a guide for their diagnosis, differential diagnosis, treatment, and monitoring. Copyright © 2013 SEEN. Published by Elsevier Espana. All rights reserved.

  16. Leptin, insulin and thyroid hormones in a cohort of Egyptian obese Down syndrome children: a comparative study

    Directory of Open Access Journals (Sweden)

    Yahia Sohier

    2012-10-01

    Full Text Available Abstract Background Obesity is a major worldwide health problem. It is commonly observed in Down syndrome individuals than in the general population. The reason for increased risk of obesity in DS is unclear. The current study was designed to clarify differences in some obesity- related hormones in a group of prepubertal Down syndrome children. Methods Thirty six Egyptian children with Down syndrome were enrolled in this study, divided according to their body mass index (BMI into 23 obese and13 non obese. Another group of 43 non Down children were recruited, they were divided according to their BMI into 20 patients having simple obesity and 23 non obese, as control groups. Fasting blood samples were collected for estimation of fasting blood glucose (FBG, insulin, leptin, free thyroxin (FT4, thyroid stimulating hormones (TSH and creatine kinase (CK. Insulin resistance was assessed by Homeostasis Model Assessment method (HOMA-IR. The ratio of leptin to BMI (LEP/BMI was used as an index of leptin resistance. Results Median values of FBG, insulin, and HOMA-IR were significantly higher in Down versus non Down groups, while median values of leptin and leptin resistance were non-significantly different among Down versus non Down groups. Median TSH values were non- significantly different between obese Down and obese non Down. Although the median values of TSH and FT4 were within normal range in Down groups, four cases of subclinical hypothyroidism were encountered. Leptin levels were correlated with insulin and IR but not with TSH in Down groups. Conclusion Increased circulating leptin, a marker of leptin resistance in obese children with Down syndrome seems to be similar to that in children with simple obesity. Elevated FBG and insulin in obese Down children highlights the presence of early IR. Associated myopathy evidenced by mildly elevated CK levels could be an added factor for obesity in such group of patients.

  17. Growth Hormone Research Society Workshop Summary: Consensus Guidelines for Recombinant Human Growth Hormone Therapy in Prader-Willi Syndrome

    Science.gov (United States)

    Tony, Michèle; Höybye, Charlotte; Allen, David B.; Tauber, Maïthé; Christiansen, Jens Sandahl; Ambler, Geoffrey R.; Battista, Renaldo; Beauloye, Véronique; Berall, Glenn; Biller, Beverly M. K.; Butler, Merlin G.; Cassidy, Suzanne B.; Chihara, Kazuo; Cohen, Pinchas; Craig, Maria; Farholt, Stense; Goetghebeur, Mireille; Goldstone, Anthony P.; Greggi, Tiziana; Grugni, Graziano; Hokken-Koelega, Anita C.; Johannsson, Gudmundur; Johnson, Keegan; Kemper, Alex; Kopchick, John J.; Malozowski, Saul; Miller, Jennifer; Mogul, Harriette R.; Muscatelli, Françoise; Nergårdh, Ricard; Nicholls, Robert D.; Radovick, Sally; Rosenthal, M. Sara; Sipilä, Ilkka; Tarride, Jean-Eric; Vogels, Annick; Waters, Michael J.

    2013-01-01

    Context: Recombinant human GH (rhGH) therapy in Prader-Willi syndrome (PWS) has been used by the medical community and advocated by parental support groups since its approval in the United States in 2000 and in Europe in 2001. Its use in PWS represents a unique therapeutic challenge that includes treating individuals with cognitive disability, varied therapeutic goals that are not focused exclusively on increased height, and concerns about potential life-threatening adverse events. Objective: The aim of the study was to formulate recommendations for the use of rhGH in children and adult patients with PWS. Evidence: We performed a systematic review of the clinical evidence in the pediatric population, including randomized controlled trials, comparative observational studies, and long-term studies (>3.5 y). Adult studies included randomized controlled trials of rhGH treatment for ≥ 6 months and uncontrolled trials. Safety data were obtained from case reports, clinical trials, and pharmaceutical registries. Methodology: Forty-three international experts and stakeholders followed clinical practice guideline development recommendations outlined by the AGREE Collaboration (www.agreetrust.org). Evidence was synthesized and graded using a comprehensive multicriteria methodology (EVIDEM) (http://bit.ly.PWGHIN). Conclusions: Following a multidisciplinary evaluation, preferably by experts, rhGH treatment should be considered for patients with genetically confirmed PWS in conjunction with dietary, environmental, and lifestyle interventions. Cognitive impairment should not be a barrier to treatment, and informed consent/assent should include benefit/risk information. Exclusion criteria should include severe obesity, uncontrolled diabetes mellitus, untreated severe obstructive sleep apnea, active cancer, or psychosis. Clinical outcome priorities should vary depending upon age and the presence of physical, mental, and social disability, and treatment should be continued for as

  18. A Case of Pulmonary Carcinoid Tumor with a Superimposed Aspergilloma Presenting As a Covert Ectopic Adrenocorticotropic Hormone Syndrome

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    Kyoung Jin Kim

    2017-06-01

    Full Text Available Ectopic adrenocorticotropic hormone (ACTH syndrome is a challenging diagnosis only responsible for approximately 10% of Cushing syndrome cases. It has been associated with a variety of benign and malignant tumors including a carcinoid tumor accompanied by aspergilloma in our case that was significantly difficult to be detected. We report a patient over 70 years old with uncontrolled hypertension and hypokalemia presenting with generalized edema. Laboratory results revealed ACTH-dependent Cushing syndrome, but imaging studies did not show any discrete lesions secreting ACTH. The petrosal to peripheral ACTH gradient resulted in no evidence of pituitary adenoma. As the only lesion suspicious for ectopic ACTH secretion was a right lower round cystic lesion that did not appear to be a carcinoid tumor on computed tomography scan of the chest, the patient underwent video-assisted thoracic surgical resection to provide a definitive diagnosis. The final diagnosis was a small ectopic ACTH-secreting carcinoid tumor with unusual superimposed aspergilloma in the periphery of the lung. Postoperatively, the abnormal endocrine levels were normalized, and all of the clinical symptoms and signs were ameliorated. This is an informative case of ectopic ACTH syndrome (EAS that was the cause of hypokalemia, hypertension, metabolic alkalosis, and hypercortisolism despite its poorly specific cushingoid morphology and uncommon imaging findings. Therefore, we recommend that clinicians investigate any possible lesion as a potential source of EAS.

  19. The circadian variation in Anti-Müllerian hormone in patients with polycystic ovary syndrome differs significantly from normally ovulating women

    DEFF Research Database (Denmark)

    Bungum, Leif Johan; Franssohn, Florencia; Bungum, Mona Berger Håkonsen

    2013-01-01

    To improve the biologic understanding of the Polycystic Ovarian Syndrome (PCOS) condition by examining the circadian variation and relationship between Anti Müllerian Hormone (AMH), gonadotropins and ovarian steroids in PCOS patients compared to normally ovulating and menstruating women....... By comparing the pattern of co-variation between AMH and Luteinizing Hormone, two compounds closely linked to hyperandrogenism and anovulation in PCOS, the involvement of the Hypothalamic-Pituitary-Ovarian axis in PCOS pathology could be elucidated....

  20. [Morel-Lavallée syndrome and post-traumatic nodular fat necrosis: Two post-traumatic complications mimicking cellulitis].

    Science.gov (United States)

    Moulin, C; Barthélémy, I; Emering, C; D'Incan, M

    Dermal and subcutaneous inflammation following direct trauma is initially evocative of soft-tissue infection. However, two differential diagnoses must be considered: Morel-Lavallée syndrome and post-traumatic nodular fat necrosis. Case 1: a 51-year-old woman fell off her motorbike and had dermabrasions on her right and left tibial ridges that rapidly developed into dermo-hypodermitis of the entire limb. There was no improvement after 3 weeks of antibiotics. The patient was apyretic. She had a soft, non-inflammatory tumefaction on the inner aspect of her left knee. Ultrasound revealed subcutaneous collection in both legs. The surgeons confirmed a diagnosis of Morel-Lavallée syndrome and drained the two collections. Progress was good and the patient healed without major consequences. Case 2: following a fall on her stairs, a 40-year-old woman presented dermabrasions and haematomas on her left leg. Antibiotic therapy failed to prevent the progression of dermo-hypodermitis. The patient remained apyretic and there was no inflammatory syndrome. A CT scan showed thickening of a subcutaneous fat and fluid collection, resulting in diagnosis of post-traumatic nodular fat necrosis. Management was surgical and the outcome was good. These two cases show two post-traumatic cutaneous complications: Morel-Lavallée syndrome and post-traumatic nodular fat necrosis. Morel-Lavallée syndrome occurs after tangential trauma next to richly vascularized tissue. Post-traumatic nodular fat necrosis is defined as necrosis of adipocytes. In both cases, diagnosis is confirmed by imagery (Ultrasonography, tomography). Our two case reports show that inflammatory presentation of both Morel-Lavallée syndrome and post-traumatic nodular fat necrosis can lead to diagnostic and therapeutic errors while a surgical procedure is necessary since tissue necrosis can occur. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

  1. Primary biliary cirrhosis complicated by transverse myelitis in a patient without Sjögren's syndrome

    Directory of Open Access Journals (Sweden)

    Micheli A

    2005-01-01

    Full Text Available Transverse myelitis is an acute inflammatory process, affecting one or more segments of the spinal cord. Its association with primary biliary cirrhosis has been documented in only four cases - all along with Sjögren's syndrome. Herein, we report for the first time, a patient who developed recurrent acute transverse myelitis in association with primary biliary cirrhosis without any clinical or histological indication of Sjögren's syndrome. A 42-year-old woman with primary biliary cirrhosis developed acute onset quadriparesis and urinary retention. Diagnostic evaluation excluded the presence of Sjögren's syndrome, other autoimmune syndromes, infections and multiple sclerosis. Magnetic resonance imaging of the spinal cord disclosed signal intensity abnormalities from C1 to T2 after gadolinium enhancement. As diagnosis of acute transverse myelitis was prominent, the patient was treated with intravenous methylprednisolone. The patient had a fair outcome despite an early recurrence of the symptoms after treatment withdrawal.

  2. Anesthetic Implications of Emergent Cesarean Section in a Parturient with Marfan Syndrome Complicated by Ascending Aortic Aneurysm and Heart Failure

    Directory of Open Access Journals (Sweden)

    Young Sung Kim

    2014-07-01

    Full Text Available Cardiovascular comorbidities to the Marfan syndrome may induce hemodynamic instability especially in the parturients during labor or delivery. For anesthesiologists, it is challenging to maintain hemodynamic stability during Cesarean section in those patients with Marfan syndrome. Remifentanil is an ultra-short-acting opioid with rapid onset and offset of action which provides cardiovascular stability during surgery. Together with remifentanil, the use of a laryngeal mask airway can reduce the risk of hypertensive response followed by tracheal intubation. We describe the successful administration of remifentanil and application of laryngeal mask airway for emergent Cesarean section performed under general anesthesia in a patient with Marfan syndrome complicated by ascending aortic aneurysm and heart failure. The use of remifentanil (loading dose of 1 μg/kg for 1 min, 2 min before induction; thereafter continuous infusion dose of 0.1 μg/kg/min was useful to maintain hemodynamic stability of the parturient throughout the surgery without neonatal respiratory depression. Keywords: Ascending Aortic Aneurysm; Cesarean section; Laryngeal mask airway; Marfan syndrome; Remifentanil

  3. Complications after surgical treatment of femoral neck fractures in men with alcohol dependence syndrome: retrospective register analysis of 154 cases.

    Science.gov (United States)

    Kosola, Jussi; Kaipia, Antti; Laitinen, Minna K; Nieminen, Jyrki

    2017-07-01

    One-third of hip fractures occur in men. The causes underlying hip fractures in men differ from those in women and include alcohol abuse. This retrospective register study evaluated the trends and results associated with different surgical treatment methods for nondisplaced and displaced femoral neck fractures in male patients with alcohol dependence syndrome. Men with hip fractures were identified from a local district hospital database. Alcohol dependence syndrome was identified as a diagnosis in medical records. For displaced fractures, implant survival after total hip arthroplasty was significantly lower compared to hemiarthroplasty. For nondisplaced fractures, implant survival of cannulated screws was significantly lower compared to sliding hip screws. Overall patient survival for males with alcohol dependence syndrome with hip fracture was 62% at 1 year and 49% at 2 years. Patient survival in this population did not differ between displaced and nondisplaced fractures or among different surgical methods. Patients with alcoholism who had documented evidence of alcohol dependence syndrome represented nearly half of patients sliding hip screws should be considered over multiple cannulated screws. In patients with displaced fracture, total hip arthroplasty was associated with a significantly higher risk of complications leading to revision compared to hemiarthoplasty. Prognostic Level III.

  4. Efficacy of Noninvasive Ventilation in a Patient with Hypercapnic Respiratory Failure Complicating Eisenmenger’s Syndrome

    OpenAIRE

    Ana Jaureguizar Oriol; Salvador Díaz-Lobato; Francisco León Román; Sagrario Mayoralas Alises

    2017-01-01

    Eisenmenger’s syndrome is a severe type of congenital heart disease characterized by severe pulmonary arterial hypertension. In the cases that the pressure in pulmonary circulation exceeds the systemic pressure, there appears a right-to-left shunting of blood. Consequently, the syndrome exists hypoxemia and cyanosis. Hypercapnia is not common in these patients; however, it might coexist with hypoxemic failure if there are other restrictive pathologies associated. Meanwhile, it has been descri...

  5. The effect of combined inositol supplementation on maternal metabolic profile in pregnancies complicated by metabolic syndrome and obesity.

    Science.gov (United States)

    Ferrari, Francesca; Facchinetti, Fabio; Ontiveros, Alejandra E; Roberts, Robyn P; Saade, Mia M; Blackwell, Sean C; Sibai, Baha M; Refuerzo, Jerrie S; Longo, Monica

    2016-10-01

    Myoinositol and D-chiroinositol improve insulin resistance in women with obesity and gestational diabetes and in postmenopausal women with metabolic syndrome. We previously reported that offspring born to hypertensive dams lacking endothelial nitric oxide synthase and fed a high-fat diet develop metabolic-like syndrome phenotype. The objective of the study was to investigate the effect of a mixture of myoinositol/D-chiroinositol supplementation during pregnancy on the maternal metabolic profile in pregnancies complicated by the metabolic-like syndrome and obesity using a pregnant mouse model. Female heterozygous endothelial nitric oxide synthase(-/+) mice with moderate hypertension were placed on a high-fat diet for 4 weeks to induce a metabolic-like syndrome phenotype. Similarly, wild-type C57BL/6 mice were placed on a high-fat diet for 4 weeks to induce a murine obesity model. Mice were then bred with wild-type males. On gestational day 1, dams were randomly allocated to receive either a mixture of myoinositol/D-chiroinositol in water (7.2/0.18 mg/mL, respectively) or water as control (placebo). At term (gestational day 18), maternal weights, systolic blood pressure, and a glucose tolerance test were obtained. Dams were then killed; pups and placentas were weighed and maternal blood collected. Serum levels of metabolic biomarkers relevant to diabetes and obesity (ghrelin, gastric inhibitory peptide, glucagon-like peptide 1, glucagon, insulin, leptin, resistin) were measured by a multiplex enzyme-linked immunosorbent assay. Analysis was done comparing metabolic-like syndrome-myoinositol/D-chiroinositol-treated vs metabolic-like syndrome-nontreated mice and obese-myoinositol/D-chiroinositol-treated vs obese nontreated mice. Mean systolic blood pressure was lower in metabolic-like syndrome pregnant mice treated with myoinositol/D-chiroinositol compared with placebo (P = .04), whereas there was no difference in systolic blood pressure between treated and placebo

  6. Differentiated thyroid cancer in patients with resistance to thyroid hormone syndrome. A novel case and a review of the literature.

    Science.gov (United States)

    Vinagre, João; Borges, Fátima; Costa, António; Alvelos, Maria Inês; Mazeto, Glaúcia; Sobrinho-Simões, Manuel; Soares, Paula

    2014-01-01

    Resistance to thyroid hormone (RTH) represents a syndrome in which patients present elevated circulating thyroid hormones in the presence of non-suppressed TSH. We report a novel case where a patient with RTH presented a differentiated thyroid cancer. A19 year-old female had been referred due to thyroid disease that disclosed features characteristic of a RTH. During the follow up it was detected a follicular tumor that led to the recommendation for thyroid surgical ablation, where an incidental papillary thyroid microcarcinoma (mPTC) was found. The increase of thyroglobulin (TG) levels following thyroid removal referred the patient for radioiodine treatment. Post-treatment, it was detected jugular adenopathies and the patient was subjected to cervical lymph node drainage where metastases of the mPTC were found. RTH syndrome was confirmed by the detection of a THRB germline mutation. A BRAF mutation was also found in the mPTC but not detected in the follicular adenoma or normal adjacent tissue. The young age of the patient, the rarity of BRAF mutations in childhood and the high dissemination of the malignancy, lead us to the speculation that increased TSH stimulation in a RTH background and oncogenic activation of BRAF could have served as (co) drivers and might have triggered an advanced stage of the neoplastic disease. These findings together with a review of published cases add novel information to the management of RTH patients with differentiated thyroid cancer.

  7. Acute Respiratory Distress Syndrome Complicated by Amiodarone Induced Pulmonary Fibrosis: Don't Let Your Guard Down.

    Science.gov (United States)

    Singh, Vipin Kumar; Maheshwari, Vijeta

    2017-04-01

    Amiodarone is an antiarrhythmic agent which is commonly used to treat both supraventricular and ventricular arrhythmias. This iodine containing compound has been associated with several adverse events like it tends to accumulate in several organs. Among those, the most serious is Amiodarone Pulmonary Toxicity (APT). While the incidence of this complication has decreased with the use of lower doses of amiodarone but it can occur with any dose. Pulmonary complications usually present as an acute or subacute pneumonitis. On chest X-ray and high-resolution Computed Tomography (CT), diffuse infiltrates were found. Here, we present a case in which acute respiratory distress syndrome like features were detected which got subsided after stopping tablet amiodarone. The patient was a known case of atrial fibrillation for which she was taking tablet amiodarone for the last six months.

  8. Impact of Acute Coronary Syndrome Complicated by Ventricular Fibrillation on Long-term Incidence of Sudden Cardiac Death.

    Science.gov (United States)

    Álvarez-Álvarez, Belén; Bouzas-Cruz, Noelia; Abu-Assi, Emad; Raposeiras-Roubin, Sergio; López-López, Andrea; González Cambeiro, María Cristina; Peña-Gil, Carlos; García-Acuña, José María; González-Juanatey, José Ramón

    2015-10-01

    There is little information on the effect of acute coronary syndrome complicated by ventricular fibrillation on the long-term incidence of sudden cardiac death. We analyzed this effect in a contemporary cohort of patients with acute coronary syndrome. We studied 5302 consecutive patients with acute coronary syndrome between December 2003 and December 2012. We compared mortality during and after hospitalization according to the presence or absence of ventricular fibrillation. Ventricular fibrillation was observed in 163 (3.1%) patients, and was early onset in 72.4% of these patients. In-hospital mortality was 36.2% in the group with ventricular fibrillation and 4.7% in the group without (p<.001). After a mean follow-up of 4.7 years (standard deviation, 2.6 years), mortality was 30.7% in the ventricular fibrillation group and 24.7% in the other group (P=.23). After adjusting for confounding variables, the presence of ventricular fibrillation was not associated with an increased risk of death in the follow-up period (hazard ratio=1.29; 95% confidence interval, 0.90-1.87). The cause of death was established in 72% of patients. The incidence of sudden death was 12.9% in the ventricular fibrillation group and 11.9% in the other group (P=.71). Cardiovascular-cause mortality was also similar between the 2 groups (35.5% and 34.4%, respectively. Patients with acute coronary syndrome complicated by ventricular fibrillation who survive the in-hospital phase do not appear to be at an increased risk of sudden cardiac death or other cardiovascular-cause death. Copyright © 2014 Sociedad Española de Cardiología. Published by Elsevier España, S.L.U. All rights reserved.

  9. Patients with a high risk for obstructive sleep apnea syndrome: Postoperative respiratory complications

    Directory of Open Access Journals (Sweden)

    H. Pereira

    2013-07-01

    Full Text Available Background: STOP-BANG score (snore; tired; observed apnea; arterial pressure; body mass index; age; neck circumference and gender can predict the risk of a patient having Obstructive Syndrome Apnea (OSA. The aim of this study was to evaluate the incidence STOP-BANG score ≥ 3, in surgical patients admitted to the Post-Anesthesia Care Unit (PACU. Methods: Observational, prospective study conducted in a post-anesthesia care unit (PACU during three weeks (2011. The study population consisted of adult patients after noncardiac and non-neurological surgery. Patients were classified as high risk of OSA (HR-OSA if STOP-BANG score ≥ 3 and Low-risk of OSA (LR-OSA if STOP-BANG score  3, em pacientes cirúrgicos internados na Unidade de Cuidados Pós-Anestésica (UCPA. Métodos: Estudo observacional e prospectivo conduzido numa UCPA, durante três semanas (2011. A população de estudo consistiu em doentes adultos após cirurgia não cardíaca e não neurológica. Os doentes foram considerados com alto risco de SAOS (AR-SAOS se tinham um score de STOP-BANG ≥3 e de baixo risco de SAOS (BR-SAOS se tinham score de STOP-BANG <3. Foram avaliados dados demográficos dos doentes e colhidas variáveis intraoperatórias e pós-operatórias. As características dos doentes foram comparadas através do teste de Mann-Whitney, teste t, qui-quadrado ou teste exato de Fisher. Resultados: Um total de 357 doentes foram admitidos de UCPA e 340 preencheram os critérios de inclusão. Cento e setenta e nove (52% tinham AR-SAOS. Estes doentes eram mais velhos, tinham maior probabilidade de serem do sexo masculino, tinham um Índice Massa Corporal superior, tiveram uma classificação maior no estado físico American Society Anesthesiologists, uma maior incidência de doença cardíaca isquémica, insuficiência cardíaca, hipertensão, dislipidemia e eram mais frequentemente doentes

  10. The gonadotropin-releasing hormone antagonist protocol--the protocol of choice for the polycystic ovary syndrome patient undergoing controlled ovarian stimulation

    DEFF Research Database (Denmark)

    Kol, Shahar; Homburg, Roy; Alsbjerg, Birgit

    2012-01-01

    Polycystic ovary syndrome (PCOS) patients are prone to develop ovarian hyperstimulation syndrome (OHSS), a condition which can be minimized or completely eliminated by the use of a gonadotropin-releasing hormone agonist (GnRHa) trigger. In this commentary paper, we maintain that the gonadotropin-releasing...... hormone antagonist protocol should be the protocol of choice for the PCOS patient undergoing ovarian stimulation with gonadotropins for in vitro fertilization. If an excessive ovarian response is encountered, the clinician will always have two options: either to trigger final oocyte maturation...

  11. Cochlear implantation in Pendred syndrome and non-syndromic enlarged vestibular aqueduct – clinical challenges, surgical results, and complications

    DEFF Research Database (Denmark)

    Mey, Kristianna; Bille, Michael; Cayé-Thomasen, Per

    2016-01-01

    or on populations with mixed cochlear malformations. PS/NSEVA accounts for up to 10% of congenital SNHL, rendering this a large group of cochlear implant candidates. The abnormal inner ear anatomy of these patients may be associated with a lower surgical success rate and a higher rate of complications. STUDY DESIGN......-operative risks of gushing/oozing and post-operative vertigo are the primary clinical issues in PS/NSEVA patients regarding CI. Nonetheless, the surgical success rate is high and the major complication rate is low; similar to studies of unselected series of CI recipients....

  12. A Case of Cushing’s Syndrome due to Ectopic Adrenocorticotropic Hormone Secretion from Esthesioneuroblastoma with Long Term Follow-Up after Resection

    Directory of Open Access Journals (Sweden)

    Leslee N. Matheny

    2018-01-01

    Full Text Available We present a case of a 52-year-old male who developed Cushing’s Syndrome due to ectopic adrenocorticotrophic hormone (ACTH secretion from a large esthesioneuroblastoma (ENB of the nasal sinuses. The patient initially presented with polyuria, polydipsia, weakness, and confusion. Computed tomography scan of the head and magnetic resonance imaging showed a 7 cm skull base mass centered in the right cribriform plate without sella involvement. Work-up revealed ACTH-dependent hypercortisolemia, which did not suppress appropriately after high-dose dexamethasone. Subsequent imaging of the chest, abdomen, and pelvis did not reveal other possible ectopic sources of ACTH secretion besides the ENB. His hospital course was complicated by severe hypokalemia and hyperglycemia before successful surgical resection of the tumor, the biopsy of which showed ENB. Postoperatively, his ACTH level dropped below the limit of detection. In the ensuing 4 months, he underwent adjuvant chemoradiation with carboplatin and docetaxel with good response and resolution of hypokalemia and hyperglycemia, with no sign of recurrence as of 30 months postoperatively. His endogenous cortisol production is rising but has not completely recovered.

  13. Pregnancy complicated by diabetes mellitus, superimposed preeclampsia, and adult respiratory distress syndrome: a case study.

    Science.gov (United States)

    Niesen, K M; Rajan, M F

    1994-12-01

    Pregnancy complicated by insulin-dependent diabetes mellitus carries severe physiologic risks for both the mother and the fetus. This article presents a case study to illustrate disease progression through multiple systems and psychosocial complications associated with diabetes mellitus in pregnancy. The case study also illustrates the role of nursing interventions, the use of technology, and interdisciplinary collaboration in caring for the patient and her family.

  14. The relationship between serum anti-Müllerian hormone levels and the follicular arrest for women with polycystic ovary syndrome.

    Science.gov (United States)

    Li, Jian; Li, Rong; Yu, Hua; Zhao, Shuyun; Yu, Yang; Qiao, Jie

    2015-04-01

    The aim of this study was to ascertain whether higher levels of serum anti-Müllerian hormone (AMH) are associated with the ovarian follicular arrest in women with polycystic ovary syndrome (PCOS). This prospective study compared AMH levels between serum and dominant follicular fluid (FF) in ovulatory polycystic ovary (PCO) women and anovulatory (menstrual cycle ≥60 days.) PCOS women. All 102 women provided a baseline hormone profile and underwent controlled ovarian hyperstimulation (COH). The anovulatory PCO women had a similar body mass index (BMI), antral follicle count (AFC), and baseline serum AMH levels as the ovulatory PCO women except that their median luteinizing hormone (LH; 10.0 mIU/ml), testosterone (T) (0.61 ng/l), and androstenedione (A) (3.47 ng/l) levels were significantly higher than ovulatory PCO women (4.9 mIU/m; 0.43 ng/l and 2.09 ng/l, respectively). The ovarian response to gonadotropin stimulation during COH including serum AMH on the day of HCG administration and dominant FF AMH at 36 hours after HCG administration, total follicle stimulating hormone (FSH) dose administrated, peak E2, (estrogen) levels and number of occytes retrieved were all similar between women with anovulatory and ovulatory PCO. Using multiple regression analysis it was found that an important independent determinant affecting AMH was AFC, as opposed to LH and T. Logistic regression analysis showed that the two most important factors affecting ovulation were serum LH and T, whereas serum AMH and AFC were not selected for inclusion in the model. The reduction in AMH during COH occurs as a consequence of dominant follicles with a corresponding reduction in small antral follicle number. Elevated serum AMH levels in PCO women seem to be related only to follicular excess and not follicular arrest.

  15. Efficacy of Noninvasive Ventilation in a Patient with Hypercapnic Respiratory Failure Complicating Eisenmenger’s Syndrome

    Directory of Open Access Journals (Sweden)

    Ana Jaureguizar Oriol

    2017-08-01

    Full Text Available Eisenmenger’s syndrome is a severe type of congenital heart disease characterized by severe pulmonary arterial hypertension. In the cases that the pressure in pulmonary circulation exceeds the systemic pressure, there appears a right-to-left shunting of blood. Consequently, the syndrome exists hypoxemia and cyanosis. Hypercapnia is not common in these patients; however, it might coexist with hypoxemic failure if there are other restrictive pathologies associated. Meanwhile, it has been described high prevalence of sleep disorders in Down syndrome. There is no evidence about the role of noninvasive ventilation in the management of these patients. We present a 39-year-old man, suffering of Down and Eisenmenger’s syndrome with multiple cardiac decompensations and obstructive sleep apnea (OSA, who was admitted to hospital due to severe somnolence, edema, and dyspnea. We observed a hypercapnic respiratory acidosis that ameliorated with noninvasive ventilation (NIV. The patient returned home with nocturnal NIV as a new treatment, and no further admission to hospital was seen in the following two years. To our knowledge, this is the first report about the utility of NIV in Eisenmenger’s and Down syndrome patients.

  16. A case of Alagille syndrome complicated by intraocular lens subluxation and rhegmatogenous retinal detachment

    Directory of Open Access Journals (Sweden)

    Fukumoto M

    2013-07-01

    Full Text Available Masanori Fukumoto, Tsunehiko Ikeda, Tetsuya Sugiyama, Mari Ueki, Takaki Sato, Eisuke Ishizaki Department of Ophthalmology, Osaka Medical College, Takatsuki City, Japan Abstract: This case report describes a case of Alagille syndrome with developing intraocular lens subluxation and rhegmatogenous retinal detachment 4 years after cataract surgery. A 15-year-old female patient with Alagille syndrome-associated cataracts in both eyes underwent phacoemulsification aspiration and intraocular lens implantation. Four years postoperative, intraocular lens subluxation developed in her left eye. For treatment, extraction of the dislocated intraocular lens, anterior vitrectomy, and intraocular lens fixation was performed. Three weeks later, the patient developed rhegmatogenous retinal detachment, which was well-treated by pars plana vitrectomy. Cataract surgery needs to be performed carefully in patients with Alagille syndrome due to the weakness of the zonule of Zinn. Careful postoperative observation is necessary for patients with Alagille syndrome who have undergone intraocular surgery in order to facilitate early detection of a possible rhegmatogenous retinal detachment. Keywords: Alagille syndrome, cataract, retina, surgery

  17. Bloom syndrome complicated by colonic cancer in a young Tunisian woman.

    Science.gov (United States)

    Benjazia, Elhem; Turki, Hajer; Atig, Amira; Khalifa, Mabrouk; Letaief, Amel; Bahri, Fethi; Braham, Ahlem

    2011-10-01

    Bloom syndrome (BS) is an autosomal recessive inherited disorder characterized by chromosomal instability leading to a high risk of cancer at an early age. The diagnosis should be considered in patients with short stature, photosensitivity, variable degrees of immunodeficiency, and hypogonadism. We report a 19-year-old woman, with history of dysmorphic features and recurrent infections. The diagnosis of bloom syndrome was made and confirmed cytogenetically at the age of 14 years. She developed a colon cancer revealed by venous thrombosis and anemia. She died after 15 days of the cancer diagnosis. This is the first registrated case of confirmed Bloom syndrome in Tunisian population. Copyright © 2011 Elsevier Masson SAS. All rights reserved.

  18. A case of synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome complicated by IgA nephropathy with nephrotic syndrome.

    Science.gov (United States)

    Morimoto, Katsuhiko; Nakatani, Kimihiko; Asai, Osamu; Mondori, Kuniko; Tomiwa, Kiyonori; Mondori, Takamitsu; Nakagawa, Yoshiyuki; Iwano, Masayuki; Shiiki, Hideo

    2016-05-01

    A 62-year-old man visited our hospital with a mild sore throat, high-grade fever, and clavicular pain. Seven years earlier, he had been diagnosed with synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome. His clavicles were tender and remarkably swollen. Also noted was marked pitting edema in the lower extremities and pustulosis on the palms and soles of the feet. Laboratory studies on admission showed an elevated white cell count (23,400/μl) and serum C-reactive protein level (24.4 mg/dl). Urinalysis revealed proteinuria (2+) and occult blood (3+) with numerous dysmorphic red blood cells and hyalin casts. The patient was diagnosed with recurrence of his SAPHO syndrome and started on oral glucocorticoid therapy. By day 9 after admission, he had gained 16 kg in body weight, and his proteinuria (6.4 g/day) and serum creatinine level (2.3 mg/dl) were elevated. Renal biopsy revealed mesangial proliferative glomerulonephritis with deposition of IgA and C3 in the mesangial area and along the capillary walls. The patient was diagnosed with IgA nephropathy accompanied by nephrotic syndrome. With oral prednisolone therapy, his fever, clavicular pain, and proteinuria were gradually relieved. The clinical course in this case suggests the onset of nephrotic syndrome with IgA nephropathy was associated with the recurrence of the patient's SAPHO. To our knowledge, this is the first reported case of SAPHO-associated IgA nephropathy.

  19. Shiga toxin-associated hemolytic uremic syndrome complicated by intestinal perforation in a child with typical hemolytic uremic syndrome

    OpenAIRE

    Chang, Hye Jin; Kim, Hwa Young; Choi, Jae Hong; Choi, Hyun Jin; Ko, Jae Sung; Ha, Il Soo; Cheong, Hae Il; Choi, Yong; Kang, Hee Gyung

    2014-01-01

    Hemolytic uremic syndrome (HUS) is one of the most common causes of acute renal failure in childhood and is primarily diagnosed in up to 4.5% of children who undergo chronic renal replacement therapy. Escherichia coli serotype O157:H7 is the predominant bacterial strain identified in patients with HUS; more than 100 types of Shiga toxin-producing enterohemorrhagic E. coli (EHEC) subtypes have also been isolated. The typical HUS manifestations are microangiopathic hemolytic anemia, thrombocyto...

  20. Diagnosis and management of complex regional pain syndrome complicating upper extremity recovery.

    Science.gov (United States)

    Li, Zhongyu; Smith, Beth Paterson; Smith, Thomas L; Koman, L Andrew

    2005-01-01

    Complex regional pain syndrome (CRPS) is a clinical syndrome of pain, autonomic dysfunction, trophic changes, and functional impairment. CRPS is common after hand trauma or surgery. Early diagnosis and intervention is critical for adequate recovery. The diagnosis of CRPS requires a careful history, physical examination, and supporting diagnostic testing. Optimal treatment requires a multidisciplinary approach. A large spectrum of pharmacologic interventions is efficacious in treating CRPS. Surgery may be used to relieve nociceptive foci. Patient-specific hand therapy is very important in reducing swelling, decreasing pain, and improving range of motion.

  1. Complications in Placement of a Glaucoma Drainage Device in a Patient With CREST Syndrome.

    Science.gov (United States)

    Husain, Maria Q; Alsheikh, Oday

    2017-02-01

    This case describes difficulty with conjunctival closure in an 80-year-old woman with CREST syndrome, a subset of systemic scleroderma. The patient underwent uneventful glaucoma drainage device implantation, but friability of the conjunctiva was noted during closure. Amniotic membrane was used to ensure secure closure, and the patient had a successful outcome. There is a paucity of existing studies on patients with CREST syndrome and their ocular findings. This report sheds light on ocular findings in this autoimmune disease and the necessity for alternative closure methods in special cases.

  2. The prevalence of chronic diabetic complications and metabolic syndrome is not associated with maternal type 2 diabetes

    Directory of Open Access Journals (Sweden)

    R.S. Scheffel

    2008-12-01

    Full Text Available The maternal history of type 2 diabetes mellitus (DM has been reported more frequently in patients with type 2 DM than paternal history. The aim of the present study was to determine if there was an association between maternal history of DM and the presence of chronic complications or metabolic syndrome (MetS in patients with type 2 DM. A cross-sectional study was conducted with 1455 patients with type 2 DM. All outpatients with type 2 diabetes attending the endocrine clinics who fulfilled the eligibility criteria were included. Familial history of DM was determined with a questionnaire. Diabetic complications were assessed using standard procedures. The definition of MetS used was that of the World Health Organization and the National Cholesterol Education Program's Adult Treatment Panel III report criteria. Maternal history of DM was present in 469 (32.3%, absent in 713 (49.1% and unknown in 273 patients (18.7%. Paternal history of DM was positive in 255 (17.6%, negative in 927 (63.8% and unknown in 235 patients (16.1%. The frequency of microvascular chronic complications in patients with and without a positive maternal history of DM was similar: diabetic nephropathy (51.5 vs 52.5%, diabetic retinopathy (46.0 vs 41.7%, and diabetic sensory neuropathy (31.0 vs 37.1%. The prevalence of macrovascular chronic complications and MetS was also similar. Patients with type 2 DM were more likely to have a maternal than a paternal history of DM, although maternal history of DM was not associated with an increased prevalence of chronic complications or MetS.

  3. Early Disseminated Lyme Disease with Carditis Complicated by Posttreatment Lyme Disease Syndrome

    Directory of Open Access Journals (Sweden)

    Cheryl Novak

    2017-01-01

    Full Text Available Lyme disease is an infectious disease caused by the bacterium Borrelia burgdorferi. When untreated, infection may spread to the heart, nervous system, and joints. Cardiac involvement usually manifests as abnormalities of the conduction system and bradycardia. Treatment of Lyme disease is generally effective, with a subset of patients experiencing persistent, sometimes long-term symptoms called posttreatment Lyme disease syndrome.

  4. Post-Fundoplication Dumping Syndrome: A Frequent “Rare” Complication

    Science.gov (United States)

    Kataria, Rahul; Linn, Sandar; Malik, Zubair; Abbas, Abbas E.; Parkman, Henry

    2018-01-01

    Gastroparesis, caused by delayed emptying of the stomach, has been shown to be associated with Nissen fundoplication. However, symptomatic rapid emptying of the stomach is rare after Nissen fundoplication, and its treatment is often challenging. We report 2 patients with dumping-like syndrome post-fundoplication with marked improvement of symptoms after dietary management and medical treatment. PMID:29379815

  5. Histopathology of aortic complications in bicuspid aortic valve versus Marfan syndrome: relevance for therapy?

    NARCIS (Netherlands)

    N. Grewal (Nimrat); R. Franken (Romy); B.J.M. Mulder (Barbara); M.-J. Goumans (Marie-José); J.H.N. Lindeman (Johannes); M.R.M. Jongbloed (Monique); M.C. DeRuiter (Marco); R.J.M. Klautz (Robert); A.J.J.C. Bogers (Ad); R.E. Poelmann (Robert); A.C.G.-D. Groot (Adriana C. Gittenberger-de)

    2016-01-01

    textabstractPatients with bicuspid aortic valve (BAV) and patients with Marfan syndrome (MFS) are more prone to develop aortic dilation and dissection compared to persons with a tricuspid aortic valve (TAV). To elucidate potential common and distinct pathways of clinical relevance, we compared the

  6. Dietary intake, glucose metabolism and sex hormones in women with polycystic ovary syndrome (PCOS) compared with women with non-PCOS-related infertility.

    Science.gov (United States)

    Tsai, Ya-Hui; Wang, Ting-Wen; Wei, Hsiao-Jui; Hsu, Chien-Yeh; Ho, Hsin-Jung; Chen, Wen-Hua; Young, Robert; Liaw, Chian-Mey; Chao, Jane C-J

    2013-06-28

    The present study investigated dietary intake, glucose metabolism and sex hormones in women with polycystic ovary syndrome (PCOS). A total of forty-five women (aged 25–40 years) with PCOS and 161 control women (aged 25–43 years) with non-PCOS-related infertility were recruited. Anthropometry, glucose tolerance and sex hormones were determined and dietary intake was assessed. Women with PCOS had lower serum sex hormone-binding globulin and increased BMI, waist:hip ratio, luteinising hormone, ratio of luteinising hormone: follicle-stimulating hormone, testosterone and free androgen index (FAI). Postprandial glucose, fasting insulin and insulin resistance were elevated in women with PCOS. Women with PCOS had reduced energy and carbohydrate intake but higher fat intake. Serum sex hormone-binding globulin level was negatively associated with BMI in both groups and negatively correlated with macronutrient intake in the PCOS group with hyperandrogenism. However, FAI was positively correlated with BMI, waist circumference and glucose metabolic parameters in both groups. Therefore, women with PCOS consume lower energy and carbohydrate compared with those with non-PCOS-related infertility and macronutrient intake is only negatively associated with serum sex hormone-binding globulin level in the PCOS group with hyperandrogenism.

  7. Syndrome de Stewart-Treves: complication rare de lymphœdème ...

    African Journals Online (AJOL)

    The evolution was marked by the death of the patient 1 month after the amputation. Stewart-Treves ... This is a rare complication that usually occurs in the arms of women with breast cancer who underwent mastectomy with lymph node dissection. The treatment is based on a wide excision associated with radiation therapy.

  8. The levels of Toxoplasma gondii profiin and adiponectin in obese patients complicated with or without metabolic syndrome as compared to non-obese patients

    Directory of Open Access Journals (Sweden)

    Agustin Iskandar

    2016-04-01

    Full Text Available Objective: To find out the levels of Toxoplasma gondii (T. gondii profilin and adiponectin in obese patients complicated with or without metabolic syndrome as compared to non-obese patients. Methods: This study was an observational analytic study using cross sectional design. After interview, the subjects were performed with a anthropometric test and then a metabolic syndrome panel. The levels of profilin and adiponectin were detected by using ELISA method. Results: There was a significant difference of T. gondii profilin between the obese complicated with metabolic syndrome group and the non-obese group (P = 0.00; α = 0.05, as well as the metabolically healthy obese, in which the level of profilin was significantly higher in the group as compared to the non-obese group (P = 0.001; α = 0.05. Adiponectin level of the obese complicated with metabolic syndrome group was significantly lower as compared to the metabolically healthy obese and non-obese group (P = 0.001; α = 0.05. Conclusions: The level of T. gondii profilin in obese patients was higher than that in the nonobese, whereas the level of adiponectin in obese patients complicated with metabolic syndrome was lower than that in the obese without metabolic syndrome and non-obese patients.

  9. Anti-Müllerian hormone levels in girls and adolescents with Turner syndrome are related to karyotype, pubertal development and growth hormone treatment.

    Science.gov (United States)

    Visser, Jenny A; Hokken-Koelega, Anita C S; Zandwijken, Gladys R J; Limacher, Andreas; Ranke, Michael B; Flück, Christa E

    2013-07-01

    In girls and adolescents with Turner syndrome (TS), is there a correlation between serum AMH levels and karyotype, spontaneous puberty and other biochemical markers of ovarian function, or growth hormone (GH) therapy? Serum anti-Müllerian hormone (AMH) correlates with karyotype, pubertal development, LH, FSH and are measurable in a higher percentage of TS patients under GH therapy. Most girls with TS suffer from incomplete sexual development, premature ovarian failure and infertility due to abnormal ovarian folliculogenesis. Serum AMH levels reflect the ovarian reserve in females, even in childhood. Cross-sectional study investigating 270 karyotype proven TS patients aged 0-20 years between 2009 and 2010. Studies were conducted at three University Children's hospitals in Europe. Main outcome measures were clinical data concerning pubertal development as well as laboratory data including karyotype, serum AMH, LH, FSH, estradiol (E2), inhibin B and IGF. RESULTS AND THE ROLE OF CHANCE: Serum AMH was detectable in 21.9% of all TS girls and correlated strongly with karyotypes. A measurable serum AMH was found in 77% of TS girls with karyotype 45,X/46,XX, in 25% with 'other' karyotypes and in only 10% of 45,X TS girls. A strong relationship was also observed for measurable serum AMH and signs of spontaneous puberty such as breast development [adjusted odds ratio (OR) 19.3; 95% CI 2.1-175.6; P = 0.009] and menarche (crude OR 47.6; 95% CI 4.8-472.9; P = 0.001). Serum AMH correlated negatively with FSH and LH, but did not correlate with E2 and inhibin B. GH therapy increased the odds of having measurable AMH in TS (adjusted OR 4.1; 95% CI 1.9-8.8; P < 0.001). The cross-sectional design of the study does not allow longitudinal interpretation of the data; for that further studies are needed. High percentage of non-measurable AMH levels in the cohort of TS require categorized analysis.

  10. Posterior Reversible Encephalopathy Syndrome as an Overlooked Complication of Induced Hypertension for Cerebral Vasospasm: Systematic Review and Illustrative Case.

    Science.gov (United States)

    Muhammad, Sajjad; Güresir, Ági; Greschus, Susanne; Scorzin, Jasmin; Vatter, Hartmut; Güresir, Erdem

    2016-02-01

    Delayed cerebral ischemia associated with cerebral vasospasm is a common cause of secondary neurological decline after aneurysmal subarachnoid hemorrhage (SAH). Triple-H therapy, induced hypertension, hypervolemia, and hemodilution, is often used to treat cerebral vasospasm. However, hypertensive treatment may carry significant medical morbidity, including cardiopulmonary, renal, and intracranial complications. Posterior reversible encephalopathy syndrome (PRES) is a reversible intracranial complication that has rarely been reported in the setting of induced hypertension. We present an illustrative case of PRES in a patient with induced hypertension for SAH-related cerebral vasospasm and performed a systematic review. Furthermore, the electronic database MEDLINE was searched for additional data in published studies of PRES after induced hypertension. Overall, 7 case reports presenting 10 patients who developed PRES secondary to induced hypertension were found. Eighty-two percent of the patients were women. In all cases, the clinical symptoms were attributed to cerebral vasospasm before the diagnosis of PRES. The time from onset of induced hypertension to the development of PRES was 7.8±3.8 days. After the diagnosis of PRES and careful taper down of the blood pressure, the neurological symptoms resolved almost completely within a few days in all patients. PRES in the setting of SAH is an overlooked complication of hypertensive therapy for the treatment of vasospasm. However, the diagnosis of this phenomenon is crucial given the necessity to reverse hypertensive therapy, which is contrary to the usual management of patients with vasospasm. © 2015 American Heart Association, Inc.

  11. Pulsatile gonadotrophin releasing hormone for ovulation induction in subfertility associated with polycystic ovary syndrome

    NARCIS (Netherlands)

    Bayram, N.; van Wely, M.; van der Veen, F.

    2004-01-01

    BACKGROUND: In normal menstrual cycles, gonadotrophin releasing hormone (GnRH) secretion is pulsatile, with intervals of 60-120 minutes in the follicular phase. Treatment with pulsatile GnRH infusion by the intravenous or subcutaneous route using a portable pump has been used successfully in

  12. Growth hormone combined with child-specific motor training improves motor development in infants with Prader-Willi syndrome: A randomized controlled trial

    NARCIS (Netherlands)

    Reus, L.; Pelzer, B.J.; Otten, B.J.; Siemensma, E.P.C.; Velden, J.A.M. van der; Festen, D.A.M.; Hokken-Koelega, A.C.S.; Sanden, M.W.

    2013-01-01

    Although severe motor problems in infants with Prader-Willi syndrome (PWS) are striking, motor development has never been studied longitudinally and the results of growth hormone (GH) treatment on motor development are contradictory. The authors studied whether GH treatment can enhance the effect of

  13. The effect of the weak androgen oxandrolone on psychological and behavioral characteristics in growth hormone-treated girls with Turner syndrome.

    NARCIS (Netherlands)

    Menke, L.A.; Sas, T.C.J.; Visser, M. de; Kreukels, B.P.; Stijnen, T.; Zandwijken, G.R.; Muinck Keizer-Schrama, S.M.P.F. de; Otten, B.J.; Wit, J.M.; Cohen-Kettenis, P.T.

    2010-01-01

    The weak androgen oxandrolone (Ox) increases height gain in growth-hormone (GH) treated girls with Turner syndrome (TS), but may also give rise to virilizing side effects. To assess the effect of Ox, at a conventional and low dosage, on behavior, aggression, romantic and sexual interest, mood, and

  14. The effects of GH and hormone replacement therapy on serum concentrations of mannan-binding lectin, surfactant protein D and vitamin D binding protein in Turner syndrome

    DEFF Research Database (Denmark)

    Gravholt, Claus Højbjerg; Leth-Larsen, Rikke; Lauridsen, Anna Lis

    2004-01-01

    function. In the present study we examined whether GH or hormone replacement therapy (HRT) in Turner syndrome (TS) influence the serum concentrations of MBL and two other proteins partaking in the innate immune defence, surfactant protein D (SP-D) and vitamin D binding protein (DBP). DESIGN: Study 1...

  15. Short Stature in KBG Syndrome: First Responses to Growth Hormone Treatment.

    NARCIS (Netherlands)

    Reynaert, N.; Ockeloen, C.W.; Savendahl, L.; Beckers, D.; Devriendt, K.; Kleefstra, T.; Carels, C.E.L.; Grigelioniene, G.; Nordgren, A.; Francois, I.; Zegher, F. de; Casteels, K.

    2015-01-01

    BACKGROUND: KBG syndrome is a rare disorder characterized by intellectual disability and associated with macrodontia of the upper central incisors, specific craniofacial findings, short stature and skeletal anomalies. Genetic corroboration of a clinical diagnosis has been possible since 2011, upon

  16. Kawasaki disease shock syndrome: a rare and severe complication of Kawasaki disease.

    Science.gov (United States)

    Çakan, Mustafa; Gemici, Hakan; Aktay-Ayaz, Nuray; Keskindemirci, Gonca; Bornaun, Helen; İkizoğlu, Tarkan; Çeliker, Alpay

    2016-01-01

    Kawasaki disease is an acute systemic vasculitis that occurs most commonly in young children. It affects medium-sized muscular arteries and the coronary arteries are the predominant site of involvement. Morbidity and mortality is generally due to coronary artery aneurysms that develop during the chronic phase. Although it is well known that Kawasaki disease can cause myocarditis, tachycardia and heart failure during acute stage, Kawasaki disease shock syndrome has been recently described. It is characterized by hypotension, signs and symptoms of poor perfusion and a shock-like state. Herein we describe two cases of Kawasaki disease shock syndrome that were treated in the pediatric intensive care unit and followed a course without morbidity or mortality.

  17. Complicated Lemierre Syndrome Caused by Streptococcus gordonii and Possible Rickettsial Co-Infection in a Patient with Thrombophilia Predisposition

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    Antreas Iacovou Ioannou

    2017-06-01

    Full Text Available Aims: Lemierre syndrome is a life-threating condition characterized by recent oropharyngeal infection, internal jugular vein thrombosis, and anaerobic septicemia. It is usually caused by Fusobacterium necrophorum. Methods: A young Romanian male presented with fever and rigors, mild tachypnea, hypoxia, sore throat, decayed teeth, and tenderness of the left carotid triangle. Laboratory examination indicated severe sepsis with disseminated intravascular coagulation, acute renal failure, and acute respiratory distress syndrome while the Doppler ultrasonography of the carotids revealed left internal jugular venous thrombosis. The patient was administered piperacillin/tazobactam and vancomycin intravenously, doxycycline orally, and anti-coagulation by enoxaparin based on the diagnosis of Lemierre syndrome. Meanwhile, he was complicated by bilateral diffuse pulmonary cavities and encapsulated pleural effusions and so transcutaneous drainage was performed. The patient was discharged after a month and continued his treatment with oral phenoxypenicillin and doxycycline until full radiographic improvement. He was switched to oral anti-coagulation by vitamin-K antagonists and was referred to a hematologist, a vascular-surgeon, and a dentist. Results: Streptococcus gordonii was isolated from the patient’s blood and pleural fluid cultures and serology for Rickettsial spp. IgM was positive. Thrombophilia genetic tests revealed three minor mutations for fibrinogen-455, plasminogen activator inhibitor-1, and methylenetetrahydrofolate reductase. According to the literature, S. gordonii is not usually a causative agent and Rickettsial spp. have as yet not been correlated with Lemierre syndrome. The failure of left jugular vein recanalization shows a possible causative role of the underlying thrombophilic predisposition. Discussion: Because of the syndrome’s rarity and the atypical microorganisms isolated in this case, increased awareness is advised for its

  18. Occam paradox? A variation of tapia syndrome and an unreported complication of guidewire-assisted pedicle screw insertion.

    Science.gov (United States)

    Emohare, Osa; Peterson, Erik; Slinkard, Nathaniel; Janus, Seth; Morgan, Robert

    2013-10-01

    Study Design Case report. Clinical Question The clinical aim is to report on a previously unknown association between guidewire-assisted pedicle screw insertion and neuropraxia of the recurrent laryngeal nerve (RLN), and how this may overlap with the signs of Tapia syndrome; we also report our approach to the clinical management of this patient. Methods A 17-year-old male patient with idiopathic scoliosis experienced Tapia syndrome after posterior instrumentation and arthrodesis at the level of T1-L1. After extubation, the patient had a hoarse voice and difficulty in swallowing. Imaging showed a breach in the cortex of the anterior body of T1 corresponding to the RLN on the right. Results Otolaryngological examination noted right vocal fold immobility, decreased sensation of the endolarynx, and pooling of secretions on flexible laryngoscopy that indicated right-sided cranial nerve X injury and left-sided tongue deviation. Aspiration during a modified barium swallow prompted insertion of a percutaneous endoscopic gastrostomy tube before the patient was sent home. On postoperative day 20, a barium swallow demonstrated reduced aspiration, and the patient reported complete resolution of symptoms. The feeding tube was removed, and the patient resumed a normal diet 1 month later. Tapia syndrome, or persistent unilateral laryngeal and hypoglossal paralysis, is an uncommon neuropraxia, which has previously not been observed in association with a breached vertebral body at T1 along the course of the RLN. Conclusion Tapia syndrome should be a differential diagnostic consideration whenever these symptoms persist postoperatively and spine surgeons should be aware of this as a potential complication of guidewires in spinal instrumentation.

  19. Zika virus infection complicated by Guillain-Barre syndrome--case report, French Polynesia, December 2013.

    Science.gov (United States)

    Oehler, E; Watrin, L; Larre, P; Leparc-Goffart, I; Lastere, S; Valour, F; Baudouin, L; Mallet, Hp; Musso, D; Ghawche, F

    2014-03-06

    Zika fever, considered as an emerging disease of arboviral origin, because of its expanding geographic area, is known as a benign infection usually presenting as an influenza-like illness with cutaneous rash. So far, Zika virus infection has never led to hospitalisation. We describe the first case of Guillain-Barré syndrome (GBS) occurring immediately after a Zika virus infection, during the current Zika and type 1 and 3 dengue fever co-epidemics in French Polynesia.

  20. [Indication of metformin in the management of hormonal dysfunction secondary to polycystic ovarian syndrome: prospective comparative study of 63 cases].

    Science.gov (United States)

    Boudhrâa, Khaled; Jellouli, Mohamed Amine; Amri, Mouna; Farhat, Monia; Torkhani, Fatma; Gara, Mohamed Faouzi

    2010-05-01

    Polycystic ovarian syndrome (PCOS) is the most common hormonal dysfunction in women. It's a cause of female infertility by oligoanovulation, clinical and biochemical hyperandrogenism and polycystic ovaries. Weight loss, firstly proposed in overweight or obese patient suffering from PCOS, aims to reduce hyperinsulinism and hyperandrogenism. Recently, Metformin, an insulin sensitizer, has been proposed as an alternative first line treatment for polycystic ovarian syndrome by improving hyperinsulinemia and hyperandrogenism in these women. The aim of our study, and through a literature review, is to demonstrate if Metformin should be used as a first-line drug for infertile women with this syndrome or as an adjunction to Clomifene Citrate, the longest established treatment already used in this syndrome. A prospective comparative study including 63 patients with PCOS has been done during 2 years. Women were randomly allocated to clomifene + Metformin (Metformin group, Metformin took during 8 weeks, 850 mg twice a day, plus Clomifene 100 mg per day during five days) or Clomifene only (100 mg per day during five days). All patients underwent a two- month's diet. The middle age was about 30.63 years and the body mass index (BMI) was about 29.88 kg/ m(2). We noticed a 6.2% weight loss in both groups (a non significant difference in p=0.04). The median of infertility period was about 2.49 years. The ovulation rate in the Metformin group was 53.12% (significant difference for inducing ovulation p=0.02) and 32.25% in Clomifene group (non-significant difference 0.07). There was also a significant difference for ongoing pregnancies (p=0.04). In fact, 11 on 32 patients (34%) achieved a full-term pregnancy in Metformin group versus only 4 ones on 31 patients (12.9%) in Clomifene group. Our conclusion is that Metformin is an effective addition to Clomifene Citrate in term of reestablishment of ovulation and full-term pregnancies achievement, excluding ART cycles.

  1. Association between primary Sjögren's syndrome and pregnancy complications: a systematic review and meta-analysis.

    Science.gov (United States)

    Upala, Sikarin; Yong, Wai Chung; Sanguankeo, Anawin

    2016-08-01

    Systemic autoimmune disorders may interfere with normal reproductive function resulting in negative outcome of pregnancy. Primary Sjögren's syndrome (pSS) is a common rheumatic disease that mostly affects females. There are many reports that this condition may increase risk of pregnancy complications and fetal loss. However, data regarding these adverse outcomes are scarce and inconclusive. We performed a systematic review and meta-analysis of available articles that assess the association between pSS and adverse pregnancy outcome. We comprehensively searched the databases of MEDLINE and EMBASE from their dates of inception to March 2016 and reviewed papers with validity criteria. A random-effects model was used to evaluate pregnancy complications in patients with pSS and healthy controls. From 20 full-text articles, 7 studies involving 544 patients and 1586 pregnancies were included in the meta-analysis. Fetal complications included spontaneous abortion, stillbirth, neonatal deaths, and intrauterine growth retardation. Compared with healthy pregnancy, patients with pSS had significantly higher chance of neonatal deaths (pooled odds ratio (OR) = 1.77, 95 % confidence interval (CI) 1.28 to 1.46, p = 0.01). However, there were no significant associations between pSS and premature birth (OR = 2.10, 95 % CI 0.59-7.46, p = 0.25), spontaneous abortion (OR = 1.46, 95 % CI 0.72-2.93, p = 0.29), artificial abortion (OR = 1.12, 95 % CI 0.52-2.61, p = 0.71), or stillbirth (OR = 1.05, 95 % CI 0.38-2.97, p = 0.92). There is an increased risk of fetal loss in pregnant patients with pSS. The presented evidence further supports multidisciplinary care for these patients to prevent complications during pregnancy.

  2. Acute Abdominal Compartment Syndrome as a Complication of Percutaneous Nephrolithotomy: Two Cases Reports and Literature Review

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    Jing Tao

    2016-09-01

    Full Text Available Percutaneous nephrolithotomy (PCNL is a technique commonly used to remove large or multiple kidney stones and stones in the inferior calyx, with the advantages of lower morbidity rates, decrease in post-operative pain with faster recovery. Intra-abdominal irrigation fluid extravasation which leads to abdominal hypertension is a rare complication of PCNL with little reports. Early detection of intra-abdominal extravagation is very important to prevent morbidity and mortality. We present two cases and review the literature.

  3. Sibutramine administration decreases serum anti-Müllerian hormone (AMH) levels in women with polycystic ovary syndrome.

    Science.gov (United States)

    Vosnakis, Christos; Georgopoulos, Neoklis A; Armeni, Anastasia K; Papadakis, Efstathios; Roupas, Nikolaos D; Katsikis, Ilias; Panidis, Dimitrios

    2012-08-01

    To investigate the effect of diet, physical exercise and sibutramine administration on serum anti-Müllerian hormone (AMH) levels, hormonal and metabolic parameters in overweight and obese patients with polycystic ovary syndrome (PCOS). Prospective clinical study, in an outpatient clinic setting, of 76 overweight and obese women with PCOS. All patients were placed on a hypocaloric diet, physical exercise plus sibutramine (10 mg per day) for the first month and then on either a hypocaloric diet, physical exercise plus sibutramine (10 mg per day) or a hypocaloric diet and physical exercise for the subsequent 6 months. Serum AMH levels, body composition, hormonal and metabolic features and insulin sensitivity indices were evaluated at baseline and at 4 and 7 months of treatment. Body weight reduction was greater in the sibutramine group. Moreover, serum FSH and testosterone levels decreased, and SHBG, free androgen index and all indices of insulin resistance significantly improved at 4 and 7 months. Serum AMH levels decreased only in PCOS women who received sibutramine, at both 4 and 7 months of treatment. A hypocaloric diet and a diet plus sibutramine both resulted in significant weight loss in overweight and obese women with PCOS. Patients who received sibutramine showed greater weight loss and improvement in hyperandrogenemia and insulin sensitivity after 7 months of treatment. Serum AMH levels significantly decreased at both 4 and 7 months of treatment only in PCOS women who received sibutramine, indicating a possible direct, gonadotropin independent effect of sibutramine on the ovarian production of AMH. Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.

  4. Adrenocortical Production Is Associated with Higher Levels of Luteinizing Hormone in Nonobese Women with Polycystic Ovary Syndrome

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    Luciana Tock

    2014-01-01

    Full Text Available Objective. Insulin resistance (IR and ovarian and adrenal hyperandrogenism are a common finding in women with polycystic ovary syndrome (PCOS. The aim of the present study was to access possible differences in insulin resistance, gonadotropins, and androgens production in obese and nonobese PCOS women. Study Design. We studied 37 PCOS women (16 nonobese and 21 obese and 18 nonobese controls. Fasting glucose, insulin, androgens, and gonadotropins levels were determined. Salivary cortisol was measured basal and in the morning after dexamethasone (DEX 0.25 mg. Results. Nonobese PCOS women showed higher basal salivary cortisol and serum dehydroepiandrosterone sulfate and luteinizing hormone (LH levels than controls and obese PCOS. These hormones levels did not differ between the obese and control groups. After DEX administration no differences were found between the three groups. In PCOS women, salivary cortisol levels showed negative correlation with BMI (r=-0.52; P=0.001 and insulin (r=-0.47; P=0.003 and positive correlation with LH (r=0.40; P=0.016. Conclusion. Our results show an increased adrenocortical production in nonobese PCOS women, not related to IR and associated with a normal hypothalamic-pituitary-adrenal suppression. Higher LH levels might be involved in this event.

  5. Circulating Anti-Müllerian Hormone Levels in Daughters of Women with and without Polycystic Ovary Syndrome.

    Science.gov (United States)

    Olszanecka-Glinianowicz, Magdalena; Zachurzok, Agnieszka; Drosdzol-Cop, Agnieszka; Bożętowicz-Wikarek, Maria; Owczarek, Aleksander; Gawlik, Aneta; Chudek, Jerzy; Skrzypulec-Plinta, Violetta; Małecka-Tendera, Ewa

    2016-01-01

    The aim of this study was to assess whether circulating anti-Müllerian hormone (AMH) levels in daughters of women with polycystic ovary syndrome (PCOSd) correspond with clinical and biochemical features of hyperandrogenism, polycystic ovary morphology and menstrual cycle disturbances. Menstrual cycle disturbances, hirsutism, acne and ultrasonographic ovarian morphology were assessed in 75 girls aged 13-18 years (35 PCOSd and 40 daughters of healthy women). Serum gonadotropins, androgens, sex hormone-binding globulin and plasma AMH were measured in a fasting state, and the free androgen index was calculated. A significant correlation between the AMH level and mean ovary volume was found (r = 0.36; p polycystic ovary morphology. Significantly higher AMH levels were found only in PCOSd with irregular menstruation or secondary amenorrhea. The results of logistic regression analysis showed that in that group for each 1-ng/ml increase in the AMH level, the odds ratio of the PCOS occurrence in the future was increased 1.27 times (95% CI: 1.09-1.47; p < 0.01). A higher AMH level in PCOSd is associated with menstrual cycle disturbances and larger ovarian volume but not with clinical and biochemical features of hyperandrogenism. Thus, the risk for PCOS development among genetically predisposed girls may be related to increased AMH levels. © 2016 S. Karger AG, Basel.

  6. Body mass index and gonadotropin hormones (LH & FSH) associate with clinical symptoms among women with polycystic ovary syndrome.

    Science.gov (United States)

    Esmaeilzadeh, Seddigheh; Andarieh, Maryam Ghanbari; Ghadimi, Reza; Delavar, Mouloud Agajani

    2014-09-28

    To evaluate the relevance of body mass index (BMI), luteinizing hormone (LH), follicle-stimulating hormone (FSH) and LH/FSH ratio with clinical symptoms in polycystic ovary syndrome (PCOS) women. We reviewed the medical records of all women visited in the PCOS Clinic of Babol (Iran) from 2008 to 2012. A retrospective cross-sectional study was conducted on 175 PCOS women; aged 18-38 years diagnosed based on the Rotterdam criteria. Among the PCOs women, the prevalence of oligomenorrhea, acne, and hirsutism were found to be 92.0%, 31.4%, and 78.9%, respectively. Positive finding of polycystic ovaries was observed in 89.1% of PCOS women with by using sonography. A total of 69.2% overweight/obesity patients had polycystic ovary morphology on ultrasound image. Compared with non- overweight/obesity, the adjusted OR of PCOS women for sonographic view of polycystic ovaries was 4.33 (95% CI, 1.42-13.15, p=0.001), Nevertheless, the adjusted odds ratio (OR) showed no significant associations between LH, FSH, and LH/FSH ratio with clinical symptoms in these women. The findings of this study indicated that the overweight/obese women with PCOS are at an increased risk for sonographic view of polycystic ovaries. Therefore, it is suggested that successful weight loss is the most effective method of restoring ovulation, menstruation that should be used as major advice in obese PCOS patients.

  7. Endocrine problems in children with Prader-Willi syndrome: special review on associated genetic aspects and early growth hormone treatment

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    Dong-Kyu Jin

    2012-07-01

    Full Text Available Prader-Willi syndrome (PWS is a complex multisystem genetic disorder characterized by hypothalamic-pituitary dysfunction. The main clinical features include neonatal hypotonia, distinctive facial features, overall developmental delay, and poor growth in infancy, followed by overeating with severe obesity, short stature, and hypogonadism later in development. This paper reviews recent updates regarding the genetic aspects of this disorder. Three mechanisms (paternal deletion, maternal disomy, and deficient imprinting are recognized. Maternal disomy can arise because of 4 possible mechanisms: trisomy rescue (TR, gamete complementation (GC, monosomy rescue (MR, and postfertilization mitotic nondisjunction (Mit. Recently, TR/GC caused by nondisjunction at maternal meiosis 1 has been identified increasingly, as a result of advanced maternal childbearing age in Korea. We verified that the d3 allele increases the responsiveness of the growth hormone (GH receptor to endogenous GH. This paper also provides an overview of endocrine dysfunctions in children with PWS, including GH deficiency, obesity, sexual development, hypothyroidism, and adrenal insufficiency, as well as the effects of GH treatment. GH treatment coupled with a strictly controlled diet during early childhood may help to reduce obesity, improve neurodevelopment, and increase muscle mass. A more active approach to correct these hormone deficiencies would benefit patients with PWS.

  8. Can anti-Mullerian hormone replace ultrasonographic evaluation in polycystic ovary syndrome? A review of current progress

    Directory of Open Access Journals (Sweden)

    Awadhesh Kumar Singh

    2015-01-01

    Full Text Available Several studies over the past decade have now consistently indicated that the serum anti-Mullerian hormone (AMH levels are at least 2–3-fold higher in the patients with polycystic ovary syndrome (PCOS, which also corresponds to the increased number of AMH producing preantral and small antral follicles. Moreover, AMH levels have been found to be associated in direct proportion to the follicle numbers per ovary or antral follicular count, assessed by the transvaginal ultrasound (TVS. Furthermore, AMH correlates directly with the rising serum testosterone and luteinizing hormone levels in PCOS. Hence, serum AMH in women with oligo-anovulation and/or hyperandrogenemia could indicate the presence of underlying PCOS, when reliable TVS is not feasible, or not acceptable, either due to the virginal status or psycho-social issue. In addition, the imaging quality of abdominal ultrasound is often impaired by obesity, which typically occurs in PCOS women. Indeed, PCOS occurs most commonly in young females who cannot be subjected to invasive TVS for various reasons; therefore, a desirable alternative to TVS is urgently required to diagnose the most prevalent endocrine abnormality of young women. This review will analyze the currently available evidence regarding the role of AMH in the diagnosis of PCOS.

  9. Multiple Ligament Knee Injury: Complications

    OpenAIRE

    Manske, Robert C.; Hosseinzadeh, Pooya; Giangarra, Charles E.

    2008-01-01

    Non-operative and operative complications are common following multiple ligament knee injuries. This article will describe common complications seen by the surgeon and physical therapist following this complex injury. Complications include fractures, infections, vascular and neurologic complications following injury and surgery, compartment syndrome, complex regional pain syndrome, deep venous thrombosis, loss of motion and persistent laxity issues. A brief description of these complications ...

  10. Long-term hormone replacement therapy preserves bone mineral density in Turner syndrome

    DEFF Research Database (Denmark)

    Cleemann, Line; Hjerrild, Britta E; Lauridsen, Anna L

    2009-01-01

    at baseline and follow-up (5.9+/-0.7 years). SETTING: Tertiary hospital. PARTICIPANTS: Fifty-four women with TS (43.0+/-9.95 years). Interventions Hormone replacement therapy (HRT) and calcium and vitamin D supplementation. Main outcome measures BMD (g/cm(2)) measured at lumbar spine, hip, and the non......-dominant forearm. Bone formation and resorption markers, sex hormones, IGF1, and maximal oxygen uptake. RESULTS: At follow-up, forearm BMD, radius ultradistal BMD, and hip BMD remained unchanged, radius 1/3 BMD declined (0.601+/-0.059 vs 0.592+/-0.059, P=0.03), while spine BMD increased (0.972+/-0.139 vs 1.......010+/-0.144, Poxygen uptake was significantly reduced in TS. CONCLUSION: Longitudinal changes in BMD in TS were slight. BMD can be maintained at most sites in well...

  11. [Concentration of thyrotropic hormone and free thyroxin in children with Down's syndrome].

    Science.gov (United States)

    Jiménez-López, V; Arias, A; Arata-Bellabarba, G; Vivas, E; Delgado, M C; Paoli, M

    2001-06-01

    The incidence of hypothyroidism is higher among children with Down syndrome than among children in the general population. The frequency of hypothyroidism is higher in the areas of endemic goiter than in other areas. The aim of this paper was to study the concentrations of TSH and FT4 in children with Down syndrome residents of Mérida, a region of Venezuelan Andes. At the Centro de Estudio y Prevención del Retardo Mental y Alteraciones en el Desarrollo (CEPREMAD), the thyroid function was studied in 48 children (1 month to 6 years old), who had Down syndrome, and in 123 healthy children of similar ages. All the children were referred to the Center for thyroid function evaluation. Two (4.2%) of the 48 children with Down syndrome had congenital hypothyroidism and 22 (45.8%) had subclinical hypothyroidism (high concentration of thyrotropin-TSH). Among the control children, only 14% had elevated levels of TSH. There were no differences in relation to the gender. In conclusion in children with Down syndrome, the frequency of high concentrations of TSH was three times higher than the frequency among the healthy children. The frequency of hypothyroidism was similar to that found in areas without endemic goiter.

  12. An Unexpected Innocent Complication Associated with Azacitidine Treatment of Myelodysplastic Syndrome: Erythema Annulare Centrifugum

    Directory of Open Access Journals (Sweden)

    Esra Turan Erkek

    2016-03-01

    Full Text Available Skin lesions accompanying hematological malignancies can be formed due to either direct tumor infiltration of the skin or indirect effects. Indirectly developing lesions may be a component of paraneoplastic syndrome. Erythema annulare centrifugum (EAC is considered to be a hypersensitivity reaction developed against various antigens associated with infections, drugs, and endocrine diseases. EAC, rarely seen in neoplastic diseases, has been reported in lymphoma, leukemia, histiocytosis, and prostate cancer. Here we report EAC in a patient using a hypomethylating agent, azacitidine. A 69-year-old female patient was admitted to our polyclinic with weakness and ecchymosis in her legs existing for 3 months. She was considered as having refractory anemia with excess blasts-2 according to myelodysplastic syndrome (MDS classification [1]. Because there was only hyperdiploidy in conventional cytogenetic examination, she was classified in group intermediate-2 of the International Prognostic Scoring System. She had a history of radical mastectomy and adjuvant chemoradiotherapy for breast cancer 3 years ago. She said that variously sized round and oval erythematous, itching, painless lesions had formed in the abdominal region on the 4th day of azacitidine usage (75 mg/m2/day, 7 days, s.c. (Figure 1 and 2. There were no concomitant complaints or physical examination findings except fatigue. After azacitidine was stopped, a skin biopsy was taken. In the biopsy, mild perivascular inflammatory infiltration accompanying vascular ectasia in the papillary dermis was detected. The possibility of paraneoplastic syndrome was excluded due to the disappearance of all lesions by 1 week after cessation of treatment. During the second course of azacitidine, the lesions reoccurred on the second day. Subsequently to the second course, the patient died of sepsis, which developed after pneumonia.

  13. Ewing’s Sarcoma of the Kidney Complicated by a Wunderlich Syndrome

    Directory of Open Access Journals (Sweden)

    Mihai Razvan Manescu

    2015-01-01

    Full Text Available The Wunderlich syndrome found after the rupture of primitive renal Ewing’s sarcoma is not a situation that we find often in everyday practice. The clinical findings are not specific, which is why the differential diagnosis must be made with a multitude of benign and malignant renal masses until the correct diagnosis can be made by the pathologist. The CT and MRI images are not characteristic. One treatment option is the multidisciplinary approach; however, the prognosis remains poor for patients with metastatic disease.

  14. Cervical sympathetic chain schwannoma masquerading as a carotid body tumour with a postoperative complication of first-bite syndrome.

    LENUS (Irish Health Repository)

    Casserly, Paula

    2012-01-31

    Carotid body tumours (CBT) are the most common tumours at the carotid bifurcation. Widening of the bifurcation is usually demonstrated on conventional angiography. This sign may also be produced by a schwannoma of the cervical sympathetic plexus. A 45-year-old patient presented with a neck mass. Investigations included contrast-enhanced CT, MRI and magnetic resonance arteriography with contrast enhancement. Radiologically, the mass was considered to be a CBT due to vascular enhancement and splaying of the internal and external carotid arteries. Intraoperatively, it was determined to be a cervical sympathetic chain schwannoma (CSCS). The patient had a postoperative complication of first-bite syndrome (FBS).Although rare, CSCS should be considered in the differential diagnosis for tumours at the carotid bifurcation. Damage to the sympathetic innervation to the parotid gland can result in severe postoperative pain characterised by FBS and should be considered in all patients undergoing surgery involving the parapharyngeal space.

  15. Extranodal marginal zone B cell lymphoma: An unexpected complication in children with Sjögren's syndrome.

    Science.gov (United States)

    Collado, Paz; Kelada, Aml; Cámara, Maria; Zeft, Andrew; Flagg, Aron

    2017-03-08

    Sjögren's syndrome (SS) is a systemic autoimmune disease characterized by the infiltration of lymphocytes into exocrine glands, resulting in the typical sicca symptoms. Unlike adults, primary SS is a very rare condition in childhood, and the risk of malignancy in juvenile SS (JSS) has not been defined. We report the detection of extranodal marginal zone B-cell lymphoma (EMZL) occurring in two children with SS. Fine needle aspiration of the salivary glands (SG) showed nonspecific findings that led to delayed diagnosis of SS. The diagnosis of B-cell lymphoma associated with JSS was based on morphologic and immunohistochemical staining done during the biopsy. To highlight awareness of EMZL as a timely and appropriate update of an unusual complication in children with SS. Copyright © 2017 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

  16. Thoracic air-leakage syndrome in allogeneic stem cell transplant recipients as a late complication of chronic graft-versus-host disease: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Park, Jae Wook; Kim, Song Soo; Jo, Daeg Yeon; Yun, Hwan Jung; Lee, Hyo Jin; Kim, Jin Hwan [Chungnam National University Hospital, Chungnam National University School of Medicine, Daejeon (Korea, Republic of)

    2016-08-15

    Air-leakage syndrome associated with graft-versus-host disease (GVHD) is a rare complication, but it is also reported as an independent predictor of a worse survival rate after stem cell transplantation. We report two cases of air-leakage syndrome associated with GVHD after allogeneic stem cell transplantation in acute leukemia patients who presented with spontaneous pneumomediastinum and subcutaneous emphysema, and finally death due to respiratory failure seven to eight months later.

  17. Successful pregnancy complicated by spontaneous, familial, recurrent ovarian hyperstimulation syndrome: report of two cases.

    Science.gov (United States)

    Di Carlo, Costantino; Savoia, Fabiana; Gargano, Virginia; Sparice, Stefania; Bifulco, Giuseppe; Nappi, Carmine

    2013-10-01

    We here report two cases of spontaneous, familial, recurrent ovarian hyperstimulation syndrome ended in a successful pregnancy. First case was a 26-year-old woman, gravida 3 para 0 and two previous terminations of pregnancy due to spontaneous ovarian hyperstimulation syndrome (OHSS). During her pregnancy, patient was treated with IV fluid therapy, albumin and thromboembolic prophylaxis and required pleural and peritoneal drainage. She was referred to the hospital twice, at 8 and 28 weeks'. At 32 weeks due to worsening of clinical condition, decision was made for a caesarean section. A live, healthy preterm baby of 1950 g was delivered. Second case was a 27-year-old woman, gravida 2 para 0 with one previous termination of pregnancy due to spontaneous OHSS. During her pregnancy patient was treated with IV fluid therapy, albumin, and thromboembolic prophylaxis. She did not require any pleural or peritoneal drainage. She was referred to the hospital twice, at 8 and 30 weeks. At 37 weeks of gestation, due to worsening of clinical condition decision for a caesarean section was made. A live, healthy term baby of 2700 g was delivered. Our experience seems to confirm that management of spontaneous OHSS during pregnancy should be conservative and treatment tailored to severity of symptoms.

  18. The Metabolic Syndrome and Microvascular Complications in a Murine Model of Type 2 Diabetes

    Science.gov (United States)

    Hur, Junguk; Dauch, Jacqueline R.; Hinder, Lucy M.; Hayes, John M.; Backus, Carey; Pennathur, Subramaniam; Kretzler, Matthias; Brosius, Frank C.

    2015-01-01

    To define the components of the metabolic syndrome that contribute to diabetic polyneuropathy (DPN) in type 2 diabetes mellitus (T2DM), we treated the BKS db/db mouse, an established murine model of T2DM and the metabolic syndrome, with the thiazolidinedione class drug pioglitazone. Pioglitazone treatment of BKS db/db mice produced a significant weight gain, restored glycemic control, and normalized measures of serum oxidative stress and triglycerides but had no effect on LDLs or total cholesterol. Moreover, although pioglitazone treatment normalized renal function, it had no effect on measures of large myelinated nerve fibers, specifically sural or sciatic nerve conduction velocities, but significantly improved measures of small unmyelinated nerve fiber architecture and function. Analyses of gene expression arrays of large myelinated sciatic nerves from pioglitazone-treated animals revealed an unanticipated increase in genes related to adipogenesis, adipokine signaling, and lipoprotein signaling, which likely contributed to the blunted therapeutic response. Similar analyses of dorsal root ganglion neurons revealed a salutary effect of pioglitazone on pathways related to defense and cytokine production. These data suggest differential susceptibility of small and large nerve fibers to specific metabolic impairments associated with T2DM and provide the basis for discussion of new treatment paradigms for individuals with T2DM and DPN. PMID:25979075

  19. Recessive VARS2 mutation underlies a novel syndrome with epilepsy, mental retardation, short stature, growth hormone deficiency, and hypogonadism

    KAUST Repository

    Alsemari, Abdulaziz

    2017-11-14

    Most mitochondrial and cytoplasmic aminoacyl-tRNA synthetases (aaRSs) are encoded by nuclear genes. Syndromic disorders resulting from mutation of aaRSs genes display significant phenotypic heterogeneity. We expand aaRSs-related phenotypes through characterization of the clinical and molecular basis of a novel autosomal-recessive syndrome manifesting severe mental retardation, ataxia, speech impairment, epilepsy, short stature, microcephaly, hypogonadism, and growth hormone deficiency.A G>A variant in exon 29 of VARS2 (c.3650G>A) (NM_006295) was identified in the index case. This homozygous variant was confirmed by Sanger sequencing and segregated with disease in the family studied. The c.3650G>A change results in alteration of arginine to histidine at residue 1217 (R1217H) of the mature protein and is predicted to be pathogenic.These findings contribute to a growing list of aaRSs disorders, broadens the spectrum of phenotypes attributable to VARS2 mutations, and provides new insight into genotype-phenotype correlations among the mitochondrial synthetase genes.

  20. Impaired genome maintenance suppresses the growth hormone--insulin-like growth factor 1 axis in mice with Cockayne syndrome.

    Directory of Open Access Journals (Sweden)

    Ingrid van der Pluijm

    2007-01-01

    Full Text Available Cockayne syndrome (CS is a photosensitive, DNA repair disorder associated with progeria that is caused by a defect in the transcription-coupled repair subpathway of nucleotide excision repair (NER. Here, complete inactivation of NER in Csb(m/m/Xpa(-/- mutants causes a phenotype that reliably mimics the human progeroid CS syndrome. Newborn Csb(m/m/Xpa(-/- mice display attenuated growth, progressive neurological dysfunction, retinal degeneration, cachexia, kyphosis, and die before weaning. Mouse liver transcriptome analysis and several physiological endpoints revealed systemic suppression of the growth hormone/insulin-like growth factor 1 (GH/IGF1 somatotroph axis and oxidative metabolism, increased antioxidant responses, and hypoglycemia together with hepatic glycogen and fat accumulation. Broad genome-wide parallels between Csb(m/m/Xpa(-/- and naturally aged mouse liver transcriptomes suggested that these changes are intrinsic to natural ageing and the DNA repair-deficient mice. Importantly, wild-type mice exposed to a low dose of chronic genotoxic stress recapitulated this response, thereby pointing to a novel link between genome instability and the age-related decline of the somatotroph axis.

  1. Six-year results of a randomized, prospective trial of human growth hormone and oxandrolone in Turner syndrome.

    Science.gov (United States)

    Rosenfeld, R G; Frane, J; Attie, K M; Brasel, J A; Burstein, S; Cara, J F; Chernausek, S; Gotlin, R W; Kuntze, J; Lippe, B M

    1992-07-01

    Seventy girls with Turner syndrome, verified by karyotype, were randomly assigned to observation or treatment with human growth hormone (hGH), oxandrolone, or a combination of hGH plus oxandrolone for a period of 12 to 24 months, to assess the effect of treatment on growth velocity and adult height. Subsequently, all subjects received either hGH alone or hGH plus oxandrolone. Data are presented for 62 subjects treated for a period of 3 to 6 years. When compared with the anticipated growth rate in untreated patients, the growth rate after treatment with hGH, both alone and in combination with oxandrolone, showed a sustained increase for at least 6 years. Treatment is continuing in over half of the subjects; at present, 14 (82%) of 17 girls receiving hGH alone and 41 (91%) of 45 girls receiving combination therapy exceeded their expected adult heights. Thirty girls have completed treatment; mean height for these 30 patients is 151.9 cm, compared with their mean original projected adult height of 143.8 cm. We conclude that therapy with hGH, alone and in combination with oxandrolone, can result in a sustained increase in growth rate and a significant increase in adult height for most prepubertal girls with Turner syndrome.

  2. Carbohydrate and lipid metabolism in Turner syndrome: effect of therapy with growth hormone, oxandrolone, and a combination of both.

    Science.gov (United States)

    Wilson, D M; Frane, J W; Sherman, B; Johanson, A J; Hintz, R L; Rosenfeld, R G

    1988-02-01

    To evaluate the effects of growth-promoting therapy on carbohydrate metabolism in girls with Turner syndrome, we determined glucose and insulin concentrations during oral glucose tolerance tests (OGTTs) at baseline and after 5 days, 2 months, and 12 months of treatment with growth hormone (GH), oxandrolone, or a combination of GH and oxandrolone, or after the same intervals with no therapy. Before therapy, subjects had a significantly greater glucose response during OGTT than published normal control values. There were no significant changes in mean fasting glucose, cholesterol, or triglyceride concentrations in any of the treatment groups. The integrated glucose concentrations rose significantly over baseline values in the oxandrolone group at 2 and 12 months and in the combination group at 5 days. There were significant increases in the mean integrated insulin concentrations at 2 and 12 months for the group receiving oxandrolone alone and at all three times for the group receiving combination therapy. Thus oxandrolone, alone or in combination with GH, had significant effects on carbohydrate metabolism in subjects with Turner syndrome, whereas GH alone did not.

  3. Adrenocorticotropic Hormone-Dependent Cushing’s Syndrome: Use of an octreotide trial to distinguish between pituitary or ectopic sources

    Directory of Open Access Journals (Sweden)

    Omayma T. El-Shafie

    2015-01-01

    Full Text Available Objectives: Adrenocorticotropic hormone (ACTH overproduction is usually due to a pituitary tumour which is often not visible on magnetic resonance imaging (MRI. However, ACTH overproduction may be due to an ectopic source. This study aimed to develop a simple non-invasive technique to differentiate these sources. Methods: This study took place in King Faisal Specialist Hospital & Research Centre, Riyadh, Saudi Arabia, and Sultan Qaboos University Hospital, Muscat, Oman, between 1988 and 2012. Serum cortisol levels were measured in nine patients with ACTH-dependent Cushing’s syndrome before and during a 72-hour trial of octreotide. All patients underwent computed tomography (CT scans. MRI scans were performed on six patients. Results: CT scans were abnormal in three patients with ectopic ACTH production. MRI scans showed that three patients had pituitary microadenomas. Serum cortisol levels returned to normal in those with confirmed ectopic ACTH production. No response was found in the other six patients. Conclusion: A 72-hour trial of octreotide is recommended for patients with ACTH-dependent Cushing’s syndrome and a normal pituitary MRI. This trial will be a useful alternative to petrosal sinus sampling.

  4. Treatment with urea as an alternative to tolvaptan for the syndrome of inappropriate secretion of antidiuretic hormone

    Directory of Open Access Journals (Sweden)

    I. Gómez Valbuena

    2014-07-01

    Full Text Available The syndrome of inappropriate secretion of antidiuretic hormone (SIADH is an affectation that causes hyponatremia among other symptoms. Currently the family of drugs of choice for this syndrome are vaptans, among them the tolvaptan, antagonists of vasopressin. Urea acts as an osmotic diuretic producing sodium retention. We report a case in which the change from the urea treatment with tolvaptan in SIADH allowed to keep the answer to a much lower cost. Female, 83 years old, was admitted to our hospital as he revealed severe hyponatremia (108 mEq / L over a scan from a fall at home. Suspecting SIADH during admission was prescribed on three types of treatments: Water restriction and saline for 7 days followed by 15 mg tolvaptan and finally was prescribed 15 grams of urea every 12 hours. Serum sodium levels of patients during the first treatment did not increase enough, so it was changed to tolvaptan, during which serum sodium levels rose to normal ranges. Considering that the treatment would be long-term was changed to urea, during it, the levels of serum sodium had a slight increase. The patient was discharged with the pattern of urea indefinitely.The daily cost of treatment with tolvaptan is 66,91 €, as opposed to 0,30 € of urea. In cases such as these, urea prepared like pharmacy compound, is an alternative to tolvaptán in SIADH to a markedly lower cost.

  5. Obstetric and neonatal outcome in pregnancies complicated by hemolysis elevated liver enzymes low platelet count syndrome at a tertiary care centre in India

    OpenAIRE

    Nidhi O. Bang; Meena N. Satia; Supriya Poonia

    2016-01-01

    Background: The hemolysis elevated liver enzymes low platelet count (HELLP) syndrome is a serious complication in pregnancy characterized by hemolysis, elevated liver enzymes and low platelet count occurring in 0.4 to 0.7% of all pregnancies and in 10-12% of cases with severe preeclampsia. This present study will throw some light on occurrence, diagnosis, complications, treatment, mode of delivery and the neonatal outcome. Methods: A prospective observational study was conducted in the dep...

  6. Late-onset cytomegalovirus infection complicated by Guillain-Barre syndrome in a kidney transplant recipient: case report and review of the literature.

    Science.gov (United States)

    Shaban, E; Gohh, R; Knoll, B M

    2016-04-01

    Cytomegalovirus (CMV) infection remains a common infection after solid-organ transplantation. In the general population CMV disease is associated with Guillain-Barre syndrome (GBS), an autoimmune disease leading to an acute peripheral neuropathy, in 1 of 1000 cases. Interestingly, GBS is a rarely observed complication in solid-organ transplant recipients, possibly related to maintenance immunosuppression. We describe a case of CMV infection complicated by GBS in a kidney transplant recipient and review the literature.

  7. A complicated case of antepartum eclamptic fit with HELLP syndrome, acute renal failure and multiple intracranial hemorrhages: A mortality report

    Directory of Open Access Journals (Sweden)

    Ahmed Samy El-agwany

    2016-04-01

    Full Text Available HELLP is an acronym for hemolysis, elevated liver enzymes and low platelets count, affecting 0.2–12% of all pregnancies or 4–12% of those with preeclampsia. The maternal mortality reported from the literature is up 4% due to disseminated intravascular coagulation, placental abruption, acute renal failure, eclampsia, and cerebral hemorrhage. A 20 year old, G2P1, at 36 weeks of gestation, was referred to our hospital because of postictal coma state with bilateral mydriasis and epistaxis due to repeated antepartum eclamptic fits. Elevated blood pressure level 170/110 mmHg was accompanied with massive proteinuria. Cesarean section was performed and female newborn were delivered. Laboratory findings were characteristic of preeclampsia, HELLP syndrome and renal failure. The patient developed an intraventricular hematoma and an intracerebral hemorrhage with subarachnoid one, which were not suitable to neurosurgical treatment. The patient died from refractory hemolytic anemia, spontaneous bleeding of multiple organs, renal failure and intracranial hemorrhage. Preeclampsia, HELLP syndrome, and acute fatty liver of pregnancy might overlap and be associated with potentially fatal complications, including intracranial hemorrhage, as in the present case. Early detection and diagnosis are crucial to ensure appropriate management and treatment success.

  8. Growth Hormone Treatment in Children with Prader-Willi Syndrome : From infancy to adulthood

    NARCIS (Netherlands)

    N.E. Bakker (Nienke)

    2015-01-01

    markdownabstract__Abstract__ This is the fifth thesis of our research group in the field of Prader-Willi syndrome (PWS) and encompasses 6 new studies embedded in the Dutch PWS Cohort study in children and adolescents with PWS. In 1887, sir Langdon Down described an adolescent girl with short

  9. Multiple endocrinopathies (growth hormone deficiency, autoimmune hypothyroidism and diabetes mellitus in Kearns-Sayre syndrome

    Directory of Open Access Journals (Sweden)

    A. Berio

    2013-06-01

    Full Text Available Kearns-Sayre syndrome is characterized by onset before 20 years, chronic progressive external opthalmoplegia, pigmentary retinal degeneration, and ataxia (and/or hearth block, and/or high protein content in the cerebrospinal fluid in the presence of mtDNA rearrangements. Multiple endocrine dysfunction associated with this syndrome was rarely reported. In this paper, the Authors report on a female patient with Kearns-Sayre syndrome with large heteroplasmic mtDNA deletion, absence of cytochrome c oxidase in many muscle fibers, partial GH deficiency, hypothyroidism and subsequently insulin dependent diabetes mellitus (IDDM. Anti-thyroid peroxidase and antithyreoglobulin antibodies were present in high titer in serum while anti-islet cell antibodies were absent. The patient developed thyroiditis with Hashimoto encephalopathy. The presence of GH deficiency, autoimmune thyroiditis with hypothyroidism and IDDM distinguishes this case from others and confirms the association of Kearns-Sayre syndrome with multiple endocrine dysfunction. Hashimoto encephalopathy and anti-thyroideal antibodies suggest that in this patient, predisposed by a genetic factor (a mitochondrial deletion anti-thyroideal antibodies may have contributed to the hypothyroidism and, by interfering with cerebral mitochondrial function, may have caused the encephalopathy. GH deficiency and IDDM can be attributed to oxidative phosphorylation deficiency but the autoimmunity may also have played a role in the production of glandular insufficiencies. It seems important to search for endocrine autoimmunity in every case of KSS.

  10. Obstetrical complications in dichorionic twin pregnancies in women with polycystic ovary syndrome

    DEFF Research Database (Denmark)

    Jonsdottir, Fjola; Nilas, Lisbeth; Andreasen, Kirsten R

    2017-01-01

    AND METHODS: A retrospective study of 360 women with dichorionic twin pregnancies: 72 women with PCOS from a fertility clinic (years 1997-2010) and 288 women without PCOS from a hospital cohort (years 2005-2007). The obstetrical outcome was extracted from Danish National registers and supplemented by patient...... no overall impact of PCOS on the pregnancy outcome; the risks of preeclampsia, gestational diabetes and preterm delivery were comparable within the groups. However, five deliveries in the PCOS group compared with two in the control group occurred before gestational week 28. No difference in the obstetrical......INTRODUCTION: Both women with polycystic ovary syndrome (PCOS) and women with twin pregnancies have increased risk of adverse pregnancy outcome. The aim of this study was to investigate the impact of PCOS and maternal androgen levels on the outcome of dichorionic twin pregnancy. MATERIAL...

  11. Management of severe asymmetric pectus excavatum complicating aortic repair in a patient with Marfan's syndrome.

    Science.gov (United States)

    Yeung, Jonathan C; Marcuzzi, Danny; Peterson, Mark D; Ko, Michael A

    2016-05-01

    We describe the case of a 28-year old man with Marfan's syndrome and severe pectus excavatum who required an aortic root replacement for an ascending aortic aneurysm. There was a near-vertical angulation of the sternum that presented challenges with opening and exposure of the heart during aortic surgery. Furthermore, removal of the sternal retractor after aortic repair resulted in sudden loss of cardiac output. A Ravitch procedure was then performed to successfully close the chest without further cardiovascular compromise. We propose that patients with a severe pectus excavatum and mediastinal displacement seen on preoperative CT scanning should be considered for simultaneous, elective repair. © The Author 2016. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

  12. Spectrum of X-linked hydrocephalus (HSAS), MASA syndrome, and complicated spastic paraplegia (SPG1): Clincal review with six additional families

    Energy Technology Data Exchange (ETDEWEB)

    Schrander-Stumpel, C.; Hoeweler, C. [Univ. of Limburg (Netherlands); Jones, M. [Children`s Hospital, Sandiego, CA (United States)] [and others

    1995-05-22

    X-linked hydrocephalus (HSAS) (MIM{sup *}307000), MASA syndrome (MIM {sup *}303350), and complicated spastic paraplegia (SPG1) (MIM {sup *}3129000) are closely related. Soon after delineation, SPG1 was incorporated into the spectrum of MASA syndrome. HSAS and MASA syndrome show great clinical overlap; DNA linkage analysis places the loci at Xq28. In an increasing number of families with MASA syndrome or HSAS, mutations in L1CAM, a gene located at Xq28, have been reported. In order to further delineate the clinical spectrum, we studied 6 families with male patients presenting with MASA syndrome, HSAS, or a mixed phenotype. We summarized data from previous reports and compared them with our data. Clinical variability appears to be great, even within families. Problems in genetic counseling and prenatal diagnosis, the possible overlap with X-linked corpus callosum agenesis and FG syndrome, and the different forms of X-linked complicated spastic paraplegia are discussed. Since adducted thumbs and spastic paraplegia are found in 90% of the patients, the condition may be present in males with nonspecific mental retardation. We propose to abandon the designation MASA syndrome and use the term HSAS/MASA spectrum, incorporating SPG1. 79 refs., 6 figs., 2 tabs.

  13. Polycystic ovary syndrome, hyperandrogenemia, sex hormone-binding globulin, and risks of pregnancy complications in singleton pregnancies after assisted reproduction

    DEFF Research Database (Denmark)

    Naver, Klara

    2014-01-01

    Et klinisk prospektivt multicenterstudie. Projektet består af prægravid karakteristik af kvinder med og uden polycystisk ovariesyndrom (PCOS) før de opstarter fertilitetsbehandling. Når deltagerne er blevet gravide følges de i graviditeten med ultralydskanninger og målinger af insulin resistens, ...

  14. Ectopic bone formation as a complication of surgical rehabilitation in patients with Moebius' syndrome.

    Science.gov (United States)

    Franz, Marcus; Berndt, Alexander; Wehrhan, Falk; Schleier, Peter; Clement, Joachim; Hyckel, Peter

    2007-01-01

    Treatment of facial paralysis by muscular neurotization resulted in ectopic ossification in 1 of 134 cases in this department. That patient suffering from Moebius syndrome (MS) is presented. Reviewing the literature concerning MS, Hox genes and bone morphogenetic protein dysregulation, a pathogenesis of ossification in MS is suggested. The MS patient exhibited a congenital facial nerve palsy, which was treated by muscular neurotization (Lexer-Rosenthal). Because of postoperative ossification of scarred areas, osteotomy of the processus muscularis and mobilization of the masseter muscle was performed. Nevertheless, further ossification occurred at the interface between the mandible and zygoma and in two masticatory muscles. So, the construction of a neoarthrosis became necessary. Three years later, the iatrogenic bone defect had reossified despite of an active opening therapy. Ectopic ossification after muscular neurotization seems to be restricted to patients with MS and is triggered by trauma. Molecular pathogenesis: facial malformations in MS are caused by disturbances in embryonic patterning. Failure in the development of the second pharyngeal arch leads to a spatial BMP-4 dysregulation responsible for ossification after wounding of muscle fascia. Therefore, surgical rehabilitation of facial function by muscular neurotization is contra indicated in MS patients.

  15. Complicated adult right-sided Bochdalek hernia with Chilaiditi's syndrome: a case report.

    Science.gov (United States)

    Watanabe, Motonobu; Ishibashi, Osamu; Watanabe, Muneaki; Kondo, Tadashi; Ohkohchi, Nobuhiro

    2015-12-01

    An extremely rare adult case that underwent surgery for ileus caused by Bochdalek hernia associated with Chilaiditi's syndrome is presented. A 65-year-old woman complaining of upper abdominal pain presented to our hospital. Abdominal plain radiography showed increased intestinal gas, and computed tomography (CT) showed the transverse colon located above the right lobe of the liver, representing Chilaiditi's sign. She was diagnosed as having ileus and treated with decompression therapy by a nasoenteric tube. After hospitalization, the patient developed dyspnea, and CT showed intestinal herniation into the right thoracic cavity. She was diagnosed as having strangulated ileus caused by Bochdalek hernia. An emergent laparotomy was performed, and it showed a hole of 5 cm in diameter at the right hemi-diaphragm. The transverse colon was incarcerated through the hole, it was pulled back to the abdominal cavity, and a right hemicolectomy was performed because of necrotic changes. A small part of the liver was also herniated into the right thoracic cavity, and it was returned to the abdominal cavity. The defect in the diaphragm was closed by direct suture. Although the patient developed an abscess in the thoracic cavity postoperatively, she improved with antibiotic therapy and was discharged 2 months after the operation.

  16. D-Lactic Acidosis: An Underrecognized Complication of Short Bowel Syndrome

    Directory of Open Access Journals (Sweden)

    N. Gurukripa Kowlgi

    2015-01-01

    Full Text Available D-lactic acidosis or D-lactate encephalopathy is a rare condition that occurs primarily in individuals who have a history of short bowel syndrome. The unabsorbed carbohydrates act as a substrate for colonic bacteria to form D-lactic acid among other organic acids. The acidic pH generated as a result of D-lactate production further propagates production of D-lactic acid, hence giving rise to a vicious cycle. D-lactic acid accumulation in the blood can cause neurologic symptoms such as delirium, ataxia, and slurred speech. Diagnosis is made by a combination of clinical and laboratory data including special assays for D-lactate. Treatment includes correcting the acidosis and decreasing substrate for D-lactate such as carbohydrates in meals. In addition, antibiotics can be used to clear colonic flora. Although newer techniques for diagnosis and treatment are being developed, clinical diagnosis still holds paramount importance, as there can be many confounders in the diagnosis as will be discussed subsequently.

  17. JAK2 mutation and acute coronary syndrome complicated with stent thrombosis.

    Science.gov (United States)

    Inami, Toru; Okabe, Masahiro; Matsushita, Masato; Kobayashi, Nobuaki; Inokuchi, Koiti; Hata, Noritake; Seino, Yoshihiko; Shimizu, Wataru

    2016-10-01

    Acute coronary syndrome (ACS) could be a precious opportunity for patients to reveal concealed diseases other than conventional risk factors for ACS, such as hypertension, dyslipidemia, diabetes mellitus, etc. In the setting of ACS, the intracoronary and systemic prothrombotic environment has led to an increase in the risk of stent thrombosis of which mortality was higher among patients with ACS, especially with the highest mortality in patients with ST elevation myocardial infarction. The some specific conditions which were concealed beyond the cardiovascular pathophysiology except well-known risk factors for ACS and stent thrombosis might involve the onset of ACS. We describe a case of a 64-year-old man who was admitted to intensive care unit for chest pain. This case found the possibility that polycythemia vera with Janus kinase 2 (JAK2) V617F mutation might be a underlying disease of ACS with stent thrombosis, and highlighted the importance of recognizing polycythemia vera with JAK2 V617F mutation as concealed disease for cardiologists. We would like to report and review the relationship between ACS and polycythemia vera with JAK2 V617F mutation.

  18. Sinus Venosus Atrial Septal Defect Complicated by Eisenmenger Syndrome and the Role of Vasodilator Therapy

    Directory of Open Access Journals (Sweden)

    Amornpol Anuwatworn

    2016-01-01

    Full Text Available Sinus venosus atrial septal defect is a rare congenital, interatrial communication defect at the junction of the right atrium and the vena cava. It accounts for 5–10% of cases of all atrial septal defects. Due to the rare prevalence and anatomical complexity, diagnosing sinus venous atrial septal defects poses clinical challenges which may delay diagnosis and treatment. Advanced cardiac imaging studies are useful tools to diagnose this clinical entity and to delineate the anatomy and any associated communications. Surgical correction of the anomaly is the primary treatment. We discuss a 43-year-old Hispanic female patient who presented with dyspnea and hypoxia following a laparoscopic myomectomy. She had been diagnosed with peripartum cardiomyopathy nine years ago at another hospital. Transesophageal echocardiography and computed tomographic angiography of the chest confirmed a diagnosis of sinus venosus atrial septal defect. She was also found to have pulmonary arterial hypertension and Eisenmenger syndrome. During a hemodynamic study, she responded to vasodilator and she was treated with Ambrisentan and Tadalafil. After six months, her symptoms improved and her pulmonary arterial hypertension decreased. We also observed progressive reversal of the right-to-left shunt. This case illustrates the potential benefit of vasodilator therapy in reversing Eisenmenger physiology, which may lead to surgical repair of the atrial septal defect as the primary treatment.

  19. Pre- and perinatal complications in relation to Tourette syndrome and co-occurring obsessive-compulsive disorder and attention-deficit/hyperactivity disorder

    DEFF Research Database (Denmark)

    Abdulkadir, Mohamed; Tischfield, Jay A; King, Robert A

    2016-01-01

    Pre- and perinatal complications have been implicated in the onset and clinical expression of Tourette syndrome albeit with considerable inconsistencies across studies. Also, little is known about their role in co-occurring obsessive-compulsive disorder (OCD) and attention-deficit/hyperactivity d...

  20. Implantable cardioverter-defibrillator harm in young patients with inherited arrhythmia syndromes: A systematic review and meta-analysis of inappropriate shocks and complications

    NARCIS (Netherlands)

    Olde Nordkamp, Louise R. A.; Postema, Pieter G.; Knops, Reinoud E.; van Dijk, Nynke; Limpens, Jacqueline; Wilde, Arthur A. M.; de Groot, Joris R.

    2016-01-01

    Implantable cardioverter-defibrillators (ICDs) are implanted with the intention to prolong life in selected patients with inherited arrhythmia syndromes, but ICD implantation is also associated with inappropriate shocks and complications. We aimed to quantify the rate of inappropriate shocks and

  1. Relationship between maternal obesity and prenatal, metabolic syndrome, obstetrical and perinatal complications of pregnancy in Indiana, 2008-2010.

    Science.gov (United States)

    Feresu, Shingairai A; Wang, Yi; Dickinson, Stephanie

    2015-10-16

    Obesity is a serious medical condition affecting more than 30% of Indiana, and 25% of Unites States pregnant women. Obesity is related to maternal complications, and significantly impacts the health of pregnant women. The objective of this study was to describe the relationship between maternal complications and pre-pregnancy maternal weight. Using logistic regression models, we analyzed 2008 to 2010 birth certificate data, for 255,773 live births abstracted from the Indiana Vital Statistics registry. We examined the risk of reproductive factors, obstetrical complications and perinatal (intrapartum) complications for underweight, healthy weight, overweight and obese women for this population. Women who received prenatal care were more likely to be obese [adjusted odds ratio (AOR) = 1.82 (1.56-2.13)]. While women with parity of zero (0) were less likely to be obese [AOR = 0.89, 95% CI (0.86-0.91)]. Women giving birth to twins [AOR = 1.25, 95% CI (1.17- 1.33)], women delivering by Caesarian section [AOR = 2.31, 95% CI ( 2.26-2.37)], and women who previously had a Caesarian section [AOR = 1.95, 95% CI (1.88-2.02)] were more likely to be obese. There was evidence of metabolic like complication in this population, due to obesity. Obesity was significantly associated with obstetrical conditions of the metabolic syndrome, including pre-pregnancy diabetes, gestational diabetes, pre-pregnancy hypertension, pregnancy-induced hypertension and eclampsia [AOR = 5.12, 95% CI (4.47-5.85); AOR = 3.87, 95% CI (3.68-4.08); AOR = 7.66, 95% CI (6.77-8.65); AOR = 3.23, 95% CI (3.07-3.39); and AOR = 1.77, 95% CI (1.31-2.40), respectively. Maternal obesity modestly increased the risk of induction, epidural, post-delivery bleeding, and prolonged labor [AOR = 1.26, 95% CI (1.23-1.29); AOR = 1.15, 95% CI (1.13-1.18); AOR = 1.20, 95% CI (1.12-1.28); and AOR = 1.44, 95% CI (1.30-1.61)], respectively. Obese women were less likely to have blood transfusions [AOR

  2. Posterior reversible encephalopathy syndrome as a complication of Henoch-Schönlein purpura in a seven-year-old girl.

    Science.gov (United States)

    Dos Santos, Daiane; Langer, Felipe Welter; Dos Santos, Tatiane; Rafael Tronco Alves, Giordano; Feiten, Marisa; Teixeira de Paula Neto, Walter

    2017-02-01

    Introduction Henoch-Schönlein purpura is a multisystem small vessel vasculitis. Neurologic manifestations are uncommon. Posterior reversible encephalopathy syndrome is a rare complication of Henoch-Schönlein purpura with typical clinical and neuroimaging findings that occurs most commonly in the setting of severe hypertension and renal injury. Case presentation A seven-year-old girl was admitted to our institution presenting with clinical and laboratory findings suggestive of Henoch-Schönlein purpura. Glucocorticoid therapy was initiated, but five days following her admission, she developed altered consciousness, seizures, arterial hypertension, and cortical blindness. Brain MRI scan revealed areas of vasogenic oedema in parieto-occipital lobes, consistent with posterior reversible encephalopathy syndrome. She was immediately initiated on antihypertensives and antiepileptics, which successfully improved her neurologic symptoms. Further laboratory work-up disclosed a rapidly progressive glomerulonephritis secondary to Henoch-Schönlein purpura that was the likely cause of her sudden blood pressure elevation. Immunosuppressive therapy was undertaken, and at one-year follow-up, the patient exhibited complete renal and neurologic recovery. Conclusion Posterior reversible encephalopathy syndrome is a severe complication of Henoch-Schönlein purpura. If promptly diagnosed and treated, children with Henoch-Schönlein purpura presenting with posterior reversible encephalopathy syndrome usually have a good prognosis. Clinicians should be familiar with the characteristic presentation of posterior reversible encephalopathy syndrome and be aware that hypertension and renal injury may predispose Henoch-Schönlein purpura patients to developing this complication.

  3. Ictal central apnea and bradycardia in temporal lobe epilepsy complicated by obstructive sleep apnea syndrome

    Directory of Open Access Journals (Sweden)

    Yoko Nishimura

    2015-01-01

    Full Text Available We describe the case of a 12-year-old boy who developed temporal lobe epilepsy (TLE with daily complex partial seizures (CPS and monthly generalized seizures. Moreover, he frequently snored while asleep since early childhood. Polysomnography (PSG revealed severe obstructive sleep apnea with apnea–hypopnea index (AHI of 37.8/h. Video-PSG with simultaneous electroencephalography (EEG recording captured two ictal apneic episodes during sleep, without any motor manifestations. The onset of rhythmic theta activity in the midtemporal area on EEG was preceded by the onset of apnea by several seconds and disappeared soon after cessation of central apnea. One episode was accompanied by ictal bradycardia of <48 beats/min which persisted for 50 s beyond the end of epileptic activity. After treatment with carbamazepine and tonsillectomy/adenoidectomy, the seizures were well controlled and AHI decreased to 2.5/h. Paroxysmal discharges also disappeared during this time. Uncontrolled TLE complicated by sleep apnea should be evaluated for the presence of ictal central apnea/bradycardia.

  4. [Blood antioxidant enzyme activity during physiological and preeclampsia syndrome complicated pregnancy].

    Science.gov (United States)

    Tortladze, M; Kintraia, N; Parkauli, M; Sanikidze, T

    2013-02-01

    The aim of the study was determination of the role of oxidative metabolism in the pathogenesis of preeclampsia. It is established that in the blood of women during physiological pregnancy, catalase and glutathione reductase (gr) activity does not change significantly, compared with the same parameters in healthy nonpregnant women, superoxiddismutase (SOD) activity slightly increases, which indicates on the intensification of the oxidative processes in late term pregnancy. Intensification of oxidative processes during physiological pregnancy reviles the activation of the regulatory processes in the body directed to the induction placental cells proliferation and differentiation. During preeclampsia complicated pregnancy the catalase activity increases and SOD and GR activity decreases compared with the same parameters during physiological pregnancy; at the same time in patients blood superoxidradicals (О2-) are detected (by EPR method). Development of the chronic oxidative stress during preeclampsia may be related to disorders reperfusion-oxygenation of placenta tissue with following decreasing of the trophoblasts invasion, angiogenesis, disorders of placental metabolisms, reactivity of blood vessels, and increasing deepness of the preeclampsia.

  5. The relationship between retinol-binding protein 4 levels, insulin resistance, androgen hormones and polycystic ovary syndrome.

    Science.gov (United States)

    Mellati, Ali Awsat; Sharifi, Faranak; Sajadinejad, Mohsen; Sohrabi, Davood; Mazloomzadeh, Saeideh

    2012-02-01

    Retinol-binding protein 4 (RPB4), a 21-kDa peptide, is a recently identified adipokine that may contribute to the pathogenesis of polycystic ovary syndrome (PCOS). The aim of this study was to explore the association between serum RBP4 levels, androgen hormones and insulin resistance (IR) in women with PCOS. In this case-control study, 75 PCOS patients and 53 age- and body mass index (BMI)-matched control subjects referred to the Zanjan Metabolic Disease Research Center were enrolled. Serum RBP4 was measured using an enzyme-linked immunosorbent assay. BMI, waist circumference (WC), fasting levels of glucose, lipid profiles and insulin were also measured. A homeostatic model assessment of insulin resistance (HOMA-IR) value was used to determine the level of insulin resistance. PCOS cases had significantly higher serum RBP4 and insulin levels than control subjects (44130 ± 12760 vs. 32980 ± 9560 μg/L, p < 0.001, and 11790 ± 11480 vs. 7890 ± 4300 μU/L, p < 0.05, respectively), in univariable analysis. RBP4 showed a positive correlation with serum testosterone (r = 0.62, p < 0.0001), dehydroepiandrosterone sulfate (r = 0.45, p < 0.0001) and the waist circumference (r = 0.37, p < 0.001) of PCOS patients but not with other measured clinical and biochemical variables. However, no correlation was observed between serum RBP4 levels and HOMA-IR in all studied subjects. A final logistic regression analysis demonstrated that testosterone and dehydroepiandrosterone sulfate are independently associated with PCOS. These findings indicate that RBP4 is not independently associated with PCOS. The elevation of RBP4 levels in PCOS women might be influenced by androgen hormones. Further prospective studies are needed to clarify molecular mechanisms.

  6. Medical complications of bariatric surgery: focus on malabsorption and dumping syndrome.

    Science.gov (United States)

    Hammer, Heinz F

    2012-01-01

    Gastroenterologists increasingly see patients with symptoms after bariatric surgery. A number of gastrointestinal or extra-gastrointestinal symptoms should raise the suspicion of malabsorption or dumping syndrome. Little is known about long-term consequences of disordered intestinal anatomy and physiology resulting from bariatric surgical procedures. The latency phase of clinical problems is unknown, but may potentially be long, and postoperative courses over many decades have to be considered regarding the consequences of surgical alterations of gastrointestinal structure and function. Long-term nutritional requirements in patients with bariatric procedures are incompletely understood. This review focuses on the pathophysiology of long-limb Roux-en-Y gastric bypass (RYGB) because it has become the most common bariatric procedure in many parts of the world. Although several potential mechanisms for nutritional deficiencies after RYGB like deficiency of dietary intake, lack of gastric secretions, exclusion of proximal duodenum and jejunum, or asynergia between food bolus and biliopancreatic secretions have been postulated, it was only very recently that in-depth studies have been carried out to measure the extent to which the long-limb RYGB causes malabsorption. In order to improve care for these patients, specialists who are trained in understanding pathophysiological changes in digestion and absorption after bariatric surgery and who recognize and treat clinical symptoms and nutritional deficits after bariatric surgery are needed. In addition, clinical researchers should take advantage of the experimental setups provided by standardized surgical procedures, and scientific societies should design courses and scientific meetings which combine the expertise in gastroenterology, surgery and nutrition. Copyright © 2012 S. Karger AG, Basel.

  7. Hormone therapy in acne

    Directory of Open Access Journals (Sweden)

    Chembolli Lakshmi

    2013-01-01

    Full Text Available Underlying hormone imbalances may render acne unresponsive to conventional therapy. Relevant investigations followed by initiation of hormonal therapy in combination with regular anti-acne therapy may be necessary if signs of hyperandrogenism are present. In addition to other factors, androgen-stimulated sebum production plays an important role in the pathophysiology of acne in women. Sebum production is also regulated by other hormones, including estrogens, growth hormone, insulin, insulin-like growth factor-1, glucocorticoids, adrenocorticotropic hormone, and melanocortins. Hormonal therapy may also be beneficial in female acne patients with normal serum androgen levels. An understanding of the sebaceous gland and the hormonal influences in the pathogenesis of acne would be essential for optimizing hormonal therapy. Sebocytes form the sebaceous gland. Human sebocytes express a multitude of receptors, including receptors for peptide hormones, neurotransmitters and the receptors for steroid and thyroid hormones. Various hormones and mediators acting through the sebocyte receptors play a role in the orchestration of pathogenetic lesions of acne. Thus, the goal of hormonal treatment is a reduction in sebum production. This review shall focus on hormonal influences in the elicitation of acne via the sebocyte receptors, pathways of cutaneous androgen metabolism, various clinical scenarios and syndromes associated with acne, and the available therapeutic armamentarium of hormones and drugs having hormone-like actions in the treatment of acne.

  8. [Metabolic syndrome and insulin resistance in patients with prostate cancer treated with androgen deprivation hormone].

    Science.gov (United States)

    Terrier, J-E; Mottet, N

    2013-02-01

    Androgen suppression in prostate cancer is responsible for many side effects. Many studies, mostly retrospective, have found an association between androgen deprivation and increased cardiovascular morbidity. If the cardiovascular impact was chosen, an etiological explanation would be the occurrence of metabolic disorders, particularly insulin resistance. The objective of our work was to conduct a review of the literature assessing the impact of androgen deprivation on the onset of insulin resistance, the metabolic syndrome and changes in key markers of insulin resistance. A systematic review of literature from the Pubmed database search was performed using the following keywords: androgen deprivation therapy, metabolic syndrome, insulin resistance, hyperglycemia, cardiovascular diseases, cardiovascular risk, abdominal obesity. Twelve studies were included, bringing into focus a 55% prevalence of metabolic syndrome in patients treated for more than 12 months, an increase in fat mass and decreased lean body mass, an increase in abdominal subcutaneous fat and in visceral adiposity. The insulin was increased in four studies (25 to 60% three months dice). The increased insulin resistance was assessed by the Homeostasis Model Assessment (HOMA) was postponed three times (12% in two prospective cohort studies of type). The increase in triglycerides (20 to 40% at 12 months) and total cholesterol (7 to 11%) was observed in five studies, and increased LDL cholesterol and HDL in three studies (9 to 22%). All studies of this analysis appeared to converge towards the development of insulin resistance and metabolic disorders, however, no prospective cohort study of good methodological quality were identified. It would be necessary to conduct a prospective multicenter study in order to have a causal quality. Copyright © 2012. Published by Elsevier Masson SAS.

  9. Hormoners betydning for kognitiv utvikling : en studie av jenter med adrenogenitalt syndrom (AGS)

    OpenAIRE

    Schjølberg, Katrine Luisa Fernanda

    2010-01-01

    Bakgrunn: Studier av både mennesker og dyr som har vært utsatt for unormale mengder hormonkonsentrasjoner har vist endringer i utviklingen av kognitive ferdigheter. Andrenogenitalt syndrom(AGS) er en tilstand som fører til økt produksjon av androgener i fosterlivet. Denne tilstanden har vist klar sammenheng mellom dennes atypiske hormonnivå og atferd i form av både lekepreferanse og valg av lekepartner. I motsetning er litteraturen uklar på forholdet mellom deres hormonelle miljø og utvikling...

  10. The Syndrome of Inappropriate Secretion of Anti-Diuretic Hormone (SIADH) and Brucellosis.

    Science.gov (United States)

    Bala, Keziban Aslı; Doğan, Murat; Kaba, Sultan; Akbayram, Sinan; Aslan, Oktay; Kocaman, Selami; Bayhan, Gülsüm İclal; Üstyol, Lokman; Demir, Nihat

    2016-09-03

    BACKGROUND Our study aimed to demonstrate the frequency of the syndrome of inappropriate ADH secretion (SIADH) and associated factors during the course of brucellosis in children and adolescents. MATERIAL AND METHODS The study included children and adolescents aged 0-18 years old diagnosed with brucellosis between 2012 and 2014. The data were collected from patient charts. The diagnosis of brucellosis was made based on titrations >1:160 in standard Wright tube agglutination tests and/or positive culture tests. SIADH diagnosis was made based on the following criteria: euvolemic hyponatremia, serum Na+ 25 mmol/L with normal dietary salt intake), low uric acid (brucellosis.

  11. The role of levosimendan in acute heart failure complicating acute coronary syndrome: A review and expert consensus opinion.

    Science.gov (United States)

    Nieminen, Markku S; Buerke, Michael; Cohen-Solál, Alain; Costa, Susana; Édes, István; Erlikh, Alexey; Franco, Fatima; Gibson, Charles; Gorjup, Vojka; Guarracino, Fabio; Gustafsson, Finn; Harjola, Veli-Pekka; Husebye, Trygve; Karason, Kristjan; Katsytadze, Igor; Kaul, Sundeep; Kivikko, Matti; Marenzi, Giancarlo; Masip, Josep; Matskeplishvili, Simon; Mebazaa, Alexandre; Møller, Jacob E; Nessler, Jadwiga; Nessler, Bohdan; Ntalianis, Argyrios; Oliva, Fabrizio; Pichler-Cetin, Emel; Põder, Pentti; Recio-Mayoral, Alejandro; Rex, Steffen; Rokyta, Richard; Strasser, Ruth H; Zima, Endre; Pollesello, Piero

    2016-09-01

    Acute heart failure and/or cardiogenic shock are frequently triggered by ischemic coronary events. Yet, there is a paucity of randomized data on the management of patients with heart failure complicating acute coronary syndrome, as acute coronary syndrome and cardiogenic shock have frequently been defined as exclusion criteria in trials and registries. As a consequence, guideline recommendations are mostly driven by observational studies, even though these patients have a particularly poor prognosis compared to heart failure patients without signs of coronary artery disease. In acute heart failure, and especially in cardiogenic shock related to ischemic conditions, vasopressors and inotropes are used. However, both pathophysiological considerations and available clinical data suggest that these treatments may have disadvantageous effects. The inodilator levosimendan offers potential benefits due to a range of distinct effects including positive inotropy, restoration of ventriculo-arterial coupling, increases in tissue perfusion, and anti-stunning and anti-inflammatory effects. In clinical trials levosimendan improves symptoms, cardiac function, hemodynamics, and end-organ function. Adverse effects are generally less common than with other inotropic and vasoactive therapies, with the notable exception of hypotension. The decision to use levosimendan, in terms of timing and dosing, is influenced by the presence of pulmonary congestion, and blood pressure measurements. Levosimendan should be preferred over adrenergic inotropes as a first line therapy for all ACS-AHF patients who are under beta-blockade and/or when urinary output is insufficient after diuretics. Levosimendan can be used alone or in combination with other inotropic or vasopressor agents, but requires monitoring due to the risk of hypotension. Copyright © 2016 The Authors. Published by Elsevier Ireland Ltd.. All rights reserved.

  12. Three-year results of a randomized prospective trial of methionyl human growth hormone and oxandrolone in Turner syndrome.

    Science.gov (United States)

    Rosenfeld, R G; Hintz, R L; Johanson, A J; Sherman, B; Brasel, J A; Burstein, S; Chernausek, S; Compton, P; Frane, J; Gotlin, R W

    1988-08-01

    Seventy girls with Turner syndrome, 4 to 12 years of age, participated in a prospective, randomized study to determine the effects on growth of methionyl human growth hormone (met-hGH) or oxandrolone. Subjects were randomly assigned to receive either no treatment (control) or met-hGH (0.125 mg/kg three times per week), oxandrolone (0.125 mg/kg/day), or combination met-hGH plus oxandrolone. At the end of an initial period of 12 to 20 months, patients in the original control and oxandrolone groups were given combination met-hGH plus oxandrolone. At that time the dosage of oxandrolone was lowered to 0.0625 mg/kg/day. Sixty-five subjects have now completed the first 3 years of the study. Compared with the control growth rate for year 1 (3.8 cm/yr), significant increases in growth rate were seen in all 3 years of combination therapy (9.8, 7.4, and 6.1 cm/yr, respectively) and in the first 2 years of treatment with met-hGH alone (6.6, 5.4, and 4.6 cm/yr). When growth velocity was expressed as standard deviation for age in girls with Turner syndrome, significant increases relative to the control group for year 1 (-0.1 SD) were seen in all three years of both combination therapy and met-hGH alone (combination, +6.6, +4.3, +3.0 SD; met-hGH, +3.1, +2.0, +1.4 SD). After 3 years of treatment, predicted adult height by the method of Bayley-Pinneau increased 4.5 cm in the met-hGH group and 8.2 cm in the combination group.

  13. RELATIONSHIPS AMONG THYROID HORMONES AND OBESITY SEVERITY, METABOLIC SYNDROME AND ITS COMPONENTS IN TURKISH CHILDREN WITH OBESITY.

    Science.gov (United States)

    Özer, Samet; Bütün, İlknur; Sönmezgöz, Ergün; Yılmaz, Resul; Demir, Osman

    2015-08-01

    we investigated the relationships between thyroid function and obesity severity, metabolic syndrome (MS) and MS components in 260 obese children and adolescents 10-17 years of age. we aimed to determine the association of thyroid functions with obesity severity and the components of metabolic syndrome (MS) in pediatric obese patients. only obese children and adolescents were included, and divided the obese children into three groups according to body mass index (BMI)-SDS quartiles. The first quartile was group 1, the second and third quartiles were group 2, and the fourth quartile was group 3. Group 3 indicated severe obesity. The modified WHO criteria adapted for children were used to diagnose MS. We assessed anthropometric data and serum biochemical parameters, including the lipid profile and fasting glucose (FG), insulin, thyroid-stimulating hormone (TSH), free thyroxine (fT4), and free triiodothyronine (fT3) levels. Blood pressure (BP) was measured with a standard digital sphygmomanometer. The homeostasis model assessment of insulin resistance was calculated to determine insulin resistance (IR). TSH level was significantly higher in obese children with MS than that in the others (p = 0.045). Mean TSH level was not different among the BMI-SDS groups (p = 0.590). TSH levels and the fT3/fT4 ratio were not different in children with dyslipidemia, IR or hypertension (p = 0.515, 0.805, 0.973, 0.750, 0.515, and 0.805, respectively). obesity severity does not affect TSH level or the fT3/fT4 ratio in obese children and adolescents. IR is in close relationship with TSH level. Elevated TSH level is a risk factor for MS. Copyright AULA MEDICA EDICIONES 2014. Published by AULA MEDICA. All rights reserved.

  14. Anti-Müllerian hormone: plasma levels in women with polycystic ovary syndrome and with premature ovarian failure

    Directory of Open Access Journals (Sweden)

    Piotr Skałba

    2011-06-01

    Full Text Available Background: Anti-Müllerian hormone (AMH is a member of the transforming growth factor β (TGF-β family of growth and differentiation factors. AMH is produced by granulosa cells of preantral and small antral follicles.Aim of the study: The aim of the study was to assess the AMH plasma levels of healthy women in reproductive age and in women with polycystic ovary syndrome (PCOS and premature ovarian failure (POF.Material and methods: The study included 187 women.Results: AMH concentration in serum of patients with PCOS (73.68 ±25.58 pmol/l was significantly higher than in healthy women (16.97 ±5.80 pmol/l (p < 0.05. In patients with POF AMH concentrations in blood serum were very low or undetectable, and averaged 0.65 ±1.81 pmol/l. Average concentrations of AMH in the blood serum of the healthy group of patients differed significantly (p < 0.001 between the age-dependent subgroups. AMH concentration in serum of healthy women decreased with age. The average concentration of AMH in the blood serum in PCOS decreased after 30 years of age. There were no statistically significant differences between age subgroups.In the premature ovarian failure patients with secondary amenorrhea persisting for at least three years AMH plasma levels were extremely low (0.16 ±0.10 pmol/l and showed a significant difference compared to patients whose menses returned.Conclusions:1. In PCOS women the determination of AMH plasma levels can be used to assess severity of the syndrome.2. The determination of AMH in blood serum can be used as a marker of diminished ovarian reserve in premature ovarian failure women.

  15. Strigolactones as an auxiliary hormonal defence mechanism against leafy gall syndrome in Arabidopsis thaliana

    Science.gov (United States)

    Stes, Elisabeth; Depuydt, Stephen; De Keyser, Annick; Matthys, Cedrick; Audenaert, Kris; Yoneyama, Koichi; Werbrouck, Stefaan; Goormachtig, Sofie; Vereecke, Danny

    2015-01-01

    Leafy gall syndrome is the consequence of modified plant development in response to a mixture of cytokinins secreted by the biotrophic actinomycete Rhodococcus fascians. The similarity of the induced symptoms with the phenotype of plant mutants defective in strigolactone biosynthesis and signalling prompted an evaluation of the involvement of strigolactones in this pathology. All tested strigolactone-related Arabidopsis thaliana mutants were hypersensitive to R. fascians. Moreover, treatment with the synthetic strigolactone mixture GR24 and with the carotenoid cleavage dioxygenase inhibitor D2 illustrated that strigolactones acted as antagonistic compounds that restricted the morphogenic activity of R. fascians. Transcript profiling of the MORE AXILLARY GROWTH1 (MAX1), MAX2, MAX3, MAX4, and BRANCHED1 (BRC1) genes in the wild-type Columbia-0 accession and in different mutant backgrounds revealed that upregulation of strigolactone biosynthesis genes was triggered indirectly by the bacterial cytokinins via host-derived auxin and led to the activation of BRC1 expression, inhibiting the outgrowth of the newly developing shoots, a typical hallmark of leafy gall syndrome. Taken together, these data support the emerging insight that balances are critical for optimal leafy gall development: the long-lasting biotrophic interaction is possible only because the host activates a set of countermeasures—including the strigolactone response—in reaction to bacterial cytokinins to constrain the activity of R. fascians. PMID:26136271

  16. A Single Arm Pilot Study of Effects of Berberine on the Menstrual Pattern, Ovulation Rate, Hormonal and Metabolic Profiles in Anovulatory Chinese Women with Polycystic Ovary Syndrome.

    Science.gov (United States)

    Li, Lin; Li, Chengyan; Pan, Ping; Chen, Xiaoli; Wu, Xiaoke; Ng, Ernest Hung Yu; Yang, Dongzi

    2015-01-01

    To evaluate the effects of berberine on the menstrual pattern, ovulation rate, hormonal and metabolic profiles in anovulatory Chinese women with polycystic ovary syndrome. Berberine 0.4 g three times per day was given for four months to 102 anovulatory Chinese women with polycystic ovary syndrome. The menstrual pattern, ovulation rate, hormonal and metabolic profiles were compared before and after the berberine treatment. Ovulation was confirmed by serum progesterone level ≥10 ng/ml. A total of 98 of 102 subjects (96.1%) completed the four month treatment, including 69 (70.4%, 69/98) normal weight and 29 (29.6%, 29/98) overweight/obese. Fourteen women (14.3%, 14/98) had regained regular menses after berberine treatment and there was no significant difference between normal weight and overweight/obese groups. The ovulation rate was 25.0% over four months in the whole group, 22.5% in the normal weight group and 31.0% in the overweight/obese group. Sex hormone binding globulin, insulin resistance, total cholesterol, total triglyceride and low-density lipoprotein cholesterol decreased after berberine treatment in the normal weight group only. Our study found that administration of berberine alone may improve the menstrual pattern and ovulation rate in anovulatory Chinese women with polycystic ovary syndrome. Berberine can also decrease sex hormone binding globulin, insulin resistance, total cholesterol, triglycerides and low-density lipoprotein cholesterol in normal weight polycystic ovary syndrome women. Chictr.org ChiCTR-OO-13003943.

  17. [PATHOPHYSIOLOGY OF THE CARDIORENAL SYNDROME].

    Science.gov (United States)

    Balint, I; Vučak, J; Bašić-Marković, N; Klarić, D; Šakić, V Amerl

    2016-12-01

    Cardiorenal syndrome, a complex pathophysiological disorder of both the heart and kidneys, is a condition in which acute or chronic damage to one organ can lead to acute or chronic dysfunction of the other organ. Depending on primary organ dysfunction and disease duration, there are five different types of cardiorenal syndrome. Type 1 cardiorenal syndrome (acute cardiorenal syndrome) is defined as acute kidney injury caused by sudden decrease in heart function. Type 2 cardiorenal syndrome (chronic cardiorenal syndrome) refers to chronic kidney disease linked to chronic heart failure. Type 3 cardiorenal syndrome (acute renocardial syndrome) is caused by acute kidney injury that leads to heart failure. Type 4 cardiorenal syndrome (chronic renocardial syndrome) includes chronic heart failure due to chronic kidney disease. Type 5 cardiorenal syndrome (secondary cardiorenal syndrome) is reversible or irreversible condition marked by simultaneous heart and kidney insufficiency, as a result of multiorgan disease such as sepsis, diabetes mellitus, sarcoidosis, amyloidosis, etc. The pathophysiological patterns of cardiorenal syndrome are extremely complicated. Despite numerous publications, perplexed physiological, biochemical and hormonal disturbances as parts of the main pathogenic mechanisms of cardiorenal syndrome remain obscure. Even though there are guidelines for the treatment of patients with heart failure and chronic kidney disease, similar guidelines for the treatment of cardiorenal syndrome are lacking. In everyday practice, it is crucial to diagnose cardiorenal syndrome and use all diagnostic and therapeutic procedures available to prevent or alleviate kidney and heart failure.

  18. Association of insulin resistance with lipid profile, metabolic syndrome, and hormonal aberrations in overweight or obese women with polycystic ovary syndrome.

    Science.gov (United States)

    Ebrahimi-Mamaghani, Mehranghiz; Saghafi-Asl, Maryam; Pirouzpanah, Saeed; Aliasgharzadeh, Akbar; Aliashrafi, Soudabeh; Rezayi, Niloufar; Mehrzad-Sadaghiani, Mahzad

    2015-03-01

    This cross-sectional study was aimed to better clarify the associations of insulin resistance (IR) with endocrinometabolic parameters in polycystic ovary syndrome (PCOS). Anthropometric measurements, endocrine and metabolic profiles, and the presence of IR and metabolic syndrome (MetS) were assessed in 63 overweight or obese PCOS patients subdivided into insulin-resistant (IR) and insulin-sensitive (IS) groups. Fasting insulin concentration and HOMA-IR were higher (pinsulin check index (QUICKI), glucose-to-insulin ratio (pobesity (p=0.038) were more prevalent in IR group. For all PCOS patients, significant correlations of total cholesterol (TC) with dehydroepiandrosterone sulphate (DHEAS) (r=-0.27, p=0.031), HDL-C with QUICKI (r=0.26, p=0.036) were found. Partial correlations also showed significant associations between TG and BS2h (r=0.30, p=0.026) as well as TC and LH/FSH ratio (r=0.30, p=0.032). When the patients were divided into IR and IS groups, significant correlations of low-density lipoprotein cholesterol (LDL-C) with luteinizing hormone (LH) (r=0.50, p=0.017) as well as TC (r=0.42, p=0.043) and LDL-C (r=0.50, p=0.016) with LH/FSH ratio were observed in IR group. However, partial correlation suggested significant associations of HDL-C with testosterone (r=-0.35, p=0.049) as well as serum LDL-C (r=0.38, p=0.033), HDL-C (r=-0.32, p=0.047), and TC (r=0.34, p=0.056) with progesterone level only in the IS group. The findings of this study indicated that lipid abnormalities may occur in PCOS, irrespective of IR.

  19. Sleep-related breathing disorders in patients with Prader-Willi syndrome depending on the period of growth hormone treatment.

    Science.gov (United States)

    Lecka-Ambroziak, Agnieszka; Jędrzejczak, Małgorzata; Wysocka-Mincewicz, Marta; Szalecki, Mieczysław

    2017-10-12

    Sleep-related breathing disorders (SRBD) are commonly present in patients with Prader-Willi syndrome (PWS). Recombinant human growth hormone (rhGH) treatment is reported to improve breathing function in PWS, but the findings are not explicit. Screening polysomnography- polygraphy (PSG), assessing nasal respiratory flow, respiratory effort, and blood oxygen saturation, was used. Group 1 - before rhGH therapy (n = 11, mean age 3.0 years); PSG was repeated after the start of rhGH therapy in a mean time of 0.9 years in six patients (Group 1a). Group 2 - on rhGH treatment, for a mean time of four years (n = 17, mean age 8.8 years). Group 3 - without rhGH therapy due to severe obesity (n = 8, mean age 13.1 years). Group 1 - mean apnoea-hypopnoea index (AHI) was 10.2, oxygen desaturation index (ODI)- 36.3, Group 1a- AHI 12.0, ODI 60.9, Group 2-AHI 9.0, ODI 25.1, Group 3- AHI 8.2, ODI 22.0. ODI was significantly higher in Group 1a than in the other groups (p < 0.005), but not strictly related to SRBD. The results in Group 2 did not differ significantly from those of Group 1. Our study proves the high frequency of SRBD among PWS patients, with worsening of ODI after short-term rhGH therapy.

  20. Serum and follicular anti-Mullerian hormone levels in women with polycystic ovary syndrome (PCOS under metformin

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    Falbo Angela

    2010-07-01

    Full Text Available Abstract Background No data regarding metformin effects on follicular fluid anti-Müllerian hormone (AMH levels were to date available in literature. The aim of the present study was to evaluate in patients with polycystic ovary syndrome (PCOS whether metformin administration affects serum and follicular AMH levels, and whether this is related to ovarian response to the treatment. Methods Twenty young patients with PCOS who had received metformin were enrolled. Ten patients were anovulatory (Met-anov group, whereas the other 10 were ovulatory (Met-ov group but had failed to conceive. Further untreated PCOS (PCOS controls, n. 10 and healthy controls (non-PCOS controls, n. 10 who were scheduled for laparoscopic surgery were enrolled. In each subjects, clinical and biochemical evaluations were performed. AMH concentrations in blood and antral follicular fluid were assayed. Results In both Met-anov and Met-ov groups, and without difference between them, serum androgens and AMH, and indices of insulin resistance were significantly (p p Conclusions Metformin administration in patients with PCOS exerts a differential action on the ovarian AMH levels on the basis of ovulatory response. Changes in AMH levels in antral follicular fluid during metformin treatment could be involved in the local mechanisms mediating the ovulatory restoration.

  1. Short stature and growth hormone deficiency in a girl with encephalocraniocutaneous lipomatosis and Jaffe-Campanacci syndrome: a case report

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    Eun mi Choi

    2016-12-01

    Full Text Available A 9-year-old Tajikistani girl presented to Keimyung University Dongsan Medical Center for evaluation of a skin lesion on her left eyelid, focal alopecia, unilateral ventricular dilatation, and aortic coarctation. She was diagnosed with encephalocraniocutaneous lipomatosis (ECCL according to Moog's diagnostic criteria. Café-au-lait spots were found on the left side of her trunk. Multiple nonossifying fibromas were found on her left proximal humerus, left distal femur, both proximal tibias, and left proximal fibula, suggesting Jaffe-Campanacci syndrome (JCS, following imaging of the extremities. Many JCS cases with multiple Café-au-lait macules, multiple nonossifying fibromas may actually have Neurofibromatosis type-1 (NF1. Thus, comprehensive molecular analysis to exclude NF1 mutation was performed using her blood sample. The NF1 mutation was not found. Her height was under the 3rd percentile and her bone age was delayed as compared with her chronological age. Baseline growth hormone (GH level was below the normal range. Using the insulin stimulation and levo-dihydroxyphenylalanine tests, GH deficiency was confirmed. We present a case of GH deficiency with typical features of ECCL and JCS.

  2. Hormonal and metabolic effects of polyunsaturated fatty acid (omega-3 on polycystic ovary syndrome induced rats under diet

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    Elaheh Ouladsahebmadarek

    2014-02-01

    Full Text Available Objective(s: PCOS (polycystic ovary syndrome produces symptoms in approximately 5% to 10% of women of reproductive age (12–45 years old. It is thought to be one of the leading causes of female subfertility. This study aimed to confirm the role of nutrition containing omega-3 (polyunsaturated fatty acid on control of experimental PCO induced by estradiol-valerat in rats. Materials and Methods: Wistar female rats (n=40 were allocated into control (n=10 and test groups (n= 30, test group was subdivided into 3 groups: G1, received omega-3 (240 mg/kg/orally/daily; G2 and G3 groups were induced PCO by single injection of estradiol-valerate (16 mg/kg/IM. Group 3 received omega-3 (240 mg/kg/orally/daily and low carbohydrate feeding for 60 subsequent days; on sixtieth day 5 ml blood samples and ovarian tissues of all rats in the group were removed and prepared for biochemical and hormonal analysis. Results: Catalase, GPX (Glutathione peroxidase, SOD (Superoxide dismutase in groups that received omega-3showed higher levels, but MDA (malondialdehyde level was significantly decreased (P

  3. Long-term effects of recombinant human growth hormone therapy in children with Prader-Willi syndrome.

    Science.gov (United States)

    Wolfgram, Peter M; Carrel, Aaron L; Allen, David B

    2013-08-01

    Recombinant human growth hormone (hGH) therapy in children with Prader-Willi syndrome (PWS) improves linear growth, body composition, physical strength and agility, and other metabolic parameters. These benefits must be weighed against potential adverse effects, including rare occurrences of sudden death. This review summarizes recent evidence important to a benefit-risk analysis of hGH use in children with PWS. Studies consistently show that hGH improves stature, body composition, fat percentage and distribution, and other metabolic markers in children with PWS. Preliminary reports of improved cognitive development during hGH have also emerged. Scoliosis progression is influenced by growth rate, but frequency of occurrence and severity are not increased by hGH exposure. PWS genotype does not appear to affect response to hGH. Concerns about hGH-associated sudden death persist, but recent studies show either absence of change in sleep-disordered breathing or improved sleep cardiovascular function during hGH therapy. Recent studies confirm and expand reported benefits of hGH therapy in children with PWS, including a possible salutary role in cognitive development. These findings support previous assertions that hGH can reduce morbidity and improve function in children with PWS, and suggest that potential risks of such treatment are favorably balanced by its benefits.

  4. The Effect of Hormone Replacement Therapy on Dry Eye Syndrome Evaluated with Schirmer Test and Break-Up Time.

    Science.gov (United States)

    Feng, Yanhong; Feng, Gang; Peng, Shuli; Li, Hui

    2015-01-01

    Hormone replacement therapy (HRT) for dry eye syndrome (DES) is controversial in clinical practice. The goal of this study was to review relevant studies and analyze the pooled data to determine whether HRT is effective for DES. In this study, a literature search of PubMed, Embase, and Cochrane databases up to May 2015 was performed, with the search restricted to English language publications. The studies were screened after reading the abstract and full text. Only studies related to the effect of HRT on DES were included in the meta-analysis. Results of Schirmer tests with and without anesthetics and tear break-up time (BUT) values data were extracted and entered into RevMan software to meta-analyze the overall effect of HRT on DES. A total of 43 studies were identified, and 21 of these studies were found to be related to the effect of HRT on DES. Ultimately, 5 studies were included in the final meta-analysis. The pooled results revealed that HRT can affect Schirmer test results without anesthetics but does not affect Schirmer test results with anesthetics and BUT. The results indicate that HRT might improve DES symptoms when measuring basal tear production without anesthesia.

  5. Syndrome of Inappropriate Antidiuretic Hormone Secretion and Ibuprofen, a Rare Association to Be Considered: Role of Tolvaptan

    Science.gov (United States)

    Artom, Nathan; Oddo, Silvia; Giusti, Massimo; Dallegri, Franco

    2013-01-01

    The association between the syndrome of inappropriate antidiuretic hormone secretion (SIADH) and the use of nonsteroidal anti-inflammatory drugs (NSAIDs) is rare and has never been treated with an arginine vasopressin receptor antagonist. We report a unique case of SIADH associated with ibuprofen use and successfully treated with tolvaptan. A 76-year-old man came to our observation because of lumbar pain and epigastric discomfort. He was taking ibuprofen orally 400 mg bid as an analgesic treatment. Laboratory tests showed low levels of sodium (116 mmol/L) and chloride; a diagnosis of SIADH was formulated and ibuprofen was stopped immediately. Imaging tests allowed to rule out the presence of malignancies or cerebral and lung diseases. Slightly hypertonic saline infusion was administered for 3 days without significant sodium improvement; therefore, tolvaptan was started at the initial dose of 7.5 mg daily, doubled after 5 days. After 8 days of treatment the patient showed progressive increase of sodium levels up to normal values. In the following weeks tolvaptan was prescribed at progressively titrated dosage to full suspension; afterwards the sodium levels remained normal without any type of treatment. PMID:23819075

  6. Syndrome of Inappropriate Antidiuretic Hormone Secretion and Ibuprofen, a Rare Association to Be Considered: Role of Tolvaptan

    Directory of Open Access Journals (Sweden)

    Nathan Artom

    2013-01-01

    Full Text Available The association between the syndrome of inappropriate antidiuretic hormone secretion (SIADH and the use of nonsteroidal anti-inflammatory drugs (NSAIDs is rare and has never been treated with an arginine vasopressin receptor antagonist. We report a unique case of SIADH associated with ibuprofen use and successfully treated with tolvaptan. A 76-year-old man came to our observation because of lumbar pain and epigastric discomfort. He was taking ibuprofen orally 400 mg bid as an analgesic treatment. Laboratory tests showed low levels of sodium (116 mmol/L and chloride; a diagnosis of SIADH was formulated and ibuprofen was stopped immediately. Imaging tests allowed to rule out the presence of malignancies or cerebral and lung diseases. Slightly hypertonic saline infusion was administered for 3 days without significant sodium improvement; therefore, tolvaptan was started at the initial dose of 7.5 mg daily, doubled after 5 days. After 8 days of treatment the patient showed progressive increase of sodium levels up to normal values. In the following weeks tolvaptan was prescribed at progressively titrated dosage to full suspension; afterwards the sodium levels remained normal without any type of treatment.

  7. The Effect of Hormone Replacement Therapy on Dry Eye Syndrome Evaluated with Schirmer Test and Break-Up Time

    Directory of Open Access Journals (Sweden)

    Yanhong Feng

    2015-01-01

    Full Text Available Hormone replacement therapy (HRT for dry eye syndrome (DES is controversial in clinical practice. The goal of this study was to review relevant studies and analyze the pooled data to determine whether HRT is effective for DES. In this study, a literature search of PubMed, Embase, and Cochrane databases up to May 2015 was performed, with the search restricted to English language publications. The studies were screened after reading the abstract and full text. Only studies related to the effect of HRT on DES were included in the meta-analysis. Results of Schirmer tests with and without anesthetics and tear break-up time (BUT values data were extracted and entered into RevMan software to meta-analyze the overall effect of HRT on DES. A total of 43 studies were identified, and 21 of these studies were found to be related to the effect of HRT on DES. Ultimately, 5 studies were included in the final meta-analysis. The pooled results revealed that HRT can affect Schirmer test results without anesthetics but does not affect Schirmer test results with anesthetics and BUT. The results indicate that HRT might improve DES symptoms when measuring basal tear production without anesthesia.

  8. Ameliorative effects of rutin against metabolic, biochemical and hormonal disturbances in polycystic ovary syndrome in rats.

    Science.gov (United States)

    Jahan, Sarwat; Munir, Faryal; Razak, Suhail; Mehboob, Anam; Ain, Qurat Ul; Ullah, Hizb; Afsar, Tayyaba; Shaheen, Ghazala; Almajwal, Ali

    2016-12-07

    Polycystic ovary syndrome (PCOS) is the most prevalent endocrinopathy in women of reproductive age. The study was commenced to assess the favorable effects of Rutin against metabolic, biochemical, histological, and androgenic aspects of polycystic ovary syndrome in rats. Female Sprague-Dawley rats were administered letrozole (1 mg/kg) per orally (p.o) for a period of 21 days for the induction of PCOS, followed by dose of rutin (100 mg/kg and 150 mg/kg, p.o) for 15 days using 0.5% w/v CMC as vehicle. Metformin was also given as a standard control to one of the rat groups. Serum estradiol, progesterone, testosterone, serum lipid parameters, CRP and glucose levels were evaluated. Furthermore, antioxidant activity was tested using superoxide dismutase, catalase, glutathione per-oxidase and reactive-oxygen species level. Rutin flavonoid had a dose-dependent effect on androgenic levels depicting more recovery in the rutin-I treated group, while rutin-II treated groups showed better antioxidant and lipid profiles as compared with PCOS groups. A decrease in the value of C reactive protein (CRP) and a restoration in the proportion of estrous phase smears were observed in the rutin treated groups. Histopathological examination of ovary revealed a significant decrease in the number of cystic follicles in post treated groups. The effects observed with rutin were moderately similar to that with standard metformin, a widely used treatment drug for PCOS. The study provides evidence for the potential ameliorative effects of rutin against clinical and biochemical features of PCOS.

  9. Plasma Lipid Profiling of Patients with Chronic Ocular Complications Caused by Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis.

    Science.gov (United States)

    Saito, Kosuke; Ueta, Mayumi; Maekawa, Keiko; Sotozono, Chie; Kinoshita, Shigeru; Saito, Yoshiro

    2016-01-01

    Stevens-Johnson syndrome (SJS) and its severe variant, toxic epidermal necrolysis (TEN), are drug-induced acute inflammatory vesiculobullous reactions of the skin and mucous membranes, including the ocular surface. Even after recovery from skin symptoms, some SJS/TEN patients continue to suffer with severe ocular complications (SOCs). Therefore, this study aims to understand the pathophysiology of chronic SOCs. Because plasma lipid profiling has emerged as a useful tool to understand pathophysiological alterations in the body, we performed plasma lipid profiling of 17 patients who suffered from SJS/TEN-associated chronic SOCs. A lipidomics approach yielded 386 lipid molecules and demonstrated that plasma levels of inflammatory oxylipins increased in patients with SJS/TEN-associated chronic SOCs. In addition, oxidized phosphatidylcholines and ether-type diacylglycerols increased in the patients with chronic SOCs, while phosphoglycerolipids decreased. When we compared these lipidomic profiles with those of patients with atopic dermatitis, we found that patients with chronic SOCs, specifically, had decreased levels of ether-type phosphatidylcholines (ePCs) containing arachidonic acid (AA), such as PC(18:0e/20:4) and PC(20:0e/20:4). To confirm our finding, we recruited additional patients, who suffered from SOC associated with SJS/TEN (up to 51 patients), and validated the decreased plasma levels of AA-containing ePCs. Our study provides insight into the alterations of plasma lipidomic profiles in chronic SOCs and into the pathophysiology of SJS/TEN-associated chronic SOCs.

  10. Hypothalamic growth hormone-releasing hormone (GHRH) cell number is increased in human illness, but is not reduced in Prader-Willi syndrome or obesity

    NARCIS (Netherlands)

    Goldstone, Anthony P.; Unmehopa, Unga A.; Swaab, Dick F.

    2003-01-01

    Acute illness leads to increased GH, but reduced IGF-I secretion, while both are reduced in chronic illness. Prader-Willi syndrome (PWS) is a genetic obesity syndrome, with GH deficiency a feature independent of obesity. Reduced GH secretion may result from decreased hypothalamic release of

  11. A Giant Hepatic Hemangioma Complicated by Kasabach-Merritt Syndrome: Findings of Tc-99m RBC Scintigraphy and SPECT Including a Total Body Blood Pool Imaging Study

    Energy Technology Data Exchange (ETDEWEB)

    Sohn, Myung Hee; Jeong, Hwan Jeong; Lim, Seok Tae; Kim, Dong Wook; Yim, Chang Yeol [Chonbuk National University Medical School, Jeonju (Korea, Republic of)

    2009-02-15

    Kasabach-Merritt syndrome (KMS) consists of thrombocytopenia, microangiopathic hemolytic anemia, and localized consumption coagulopathy that develops within vascular hemangioma. This syndrome may also be associated with occult hemangiomas located at various sites. Tc-99m RBC scintigraphy and SPECT have proven to be reliable for confirming or excluding hemangioma. Total body blood pool imaging study during the scintigraphy also provides a means of screening for occult lesions. The authors report the case of a 29-year-old man who presented with a giant hepatic hemangioma complicated by KMS, and underwent Tc-99m RBC scintigraphy and SPECT including a total body blood pool imaging study.

  12. Hormonal Modulation in Aging Patients with Erectile Dysfunction and Metabolic Syndrome

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    Inês Campos Costa

    2013-01-01

    Full Text Available Erectile dysfunction (ED, metabolic syndrome (MetS, and hypogonadism are closely related, often coexisting in the aging male. Obesity was shown to raise the risk of ED and hypogonadism, as well as other endocrinological disturbances with impact on erectile function. We selected 179 patients referred for ED to our andrology unit, aiming to evaluate gonadotropins and estradiol interplay in context of ED, MetS, and hypogonadism. Patients were stratified into groups in accordance with the presence (or not of MetS and/or hypogonadism. Noticeable differences in total testosterone (TT and free testosterone (FT levels were found between patients with and without MetS. Men with MetS evidenced lower TT circulating levels with an increasing number of MetS parameters, for which hypertriglyceridemia and waist circumference strongly contributed. Regarding the hypothalamic-pituitary-gonadal axis, patients with hypogonadism did not exhibit raised LH levels. Interestingly, among those with higher LH levels, estradiol values were also increased. Possible explanations for this unexpected profile of estradiol may be the age-related adiposity, other estrogen-raising pathways, or even unknown mechanisms. Estradiol is possibly a molecule with further interactions beyond the currently described. Our results further enlighten this still unclear multidisciplinary and complex subject, raising new investigational opportunities.

  13. Missense mutations in the growth hormone receptor dimerization region in Laron syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Berg, M.A.; Francke, U. [Howard Hughes Medical Institute, Stanford, CA (United States)]|[Univ. of Stanford, CA (United States); Geffner, M.E.; Bersch, N. [Univ. of California, Los Angeles, CA (United States)] [and others

    1994-09-01

    Laron syndrome (LS) is an autosomal recessively inherited condition characterized by insensitivity to endogenous and exogenous GH. Affected individuals have severe episodes and other characteristic features. GH receptor gene mutations are present in all affected individuals in whom molecular studies have been reported. The GH receptor is a plasma membrane-spanning protein in which the extracellular domain binds circulating GH and the intracellular domain interacts with the JAK-2 kinase and possibly other intracellular signaling molecules. GH receptor dimerization occurs on GH binding and is thought to be required for normal signal transduction. We have studied the GH receptor genes of four unrelated individuals affected with LS from the United States, Italy, Saudi Arabia, and India. We have identified four different missense mutations that alter consecutive amino acids 152 to 155 in or near the dimerization domain of the GH receptor. One of these mutations, D152H, has been reported previously in Asian LS patients and, in in vitro studies, the mutant receptor was unable to dimerize. This report increases to over 20 the number of different GH receptor gene mutations that have been reported in LS patients and defines the first apparent mutational {open_quotes}hotspot{close_quotes} region in this gene. This cluster of mutations in patients with classic LS phenotype provides additional in vivo evidence that receptor dimerization plays an important role in signaling GH`s growth promoting and metabolic effects. Further in vitro studies of the mutations in this region are in progress.

  14. Care of girls and women with Turner syndrome: beyond growth and hormones

    Directory of Open Access Journals (Sweden)

    Caroline Culen

    2017-04-01

    Full Text Available Turner syndrome (TS, although considered a rare disease, is the most common sex chromosome abnormality in women, with an incident of 1 in 2500 female births. TS is characterized by distinctive physical features such as short stature, ovarian dysgenesis, an increased risk for heart and renal defects as well as a specific cognitive and psychosocial phenotype. Given the complexity of the condition, patients face manifold difficulties which increase over the lifespan. Furthermore, failures during the transitional phase to adult care result in moderate health outcomes and decreased quality of life. Guidelines on the optimal screening procedures and medical treatment are easy to find. However, recommendations for the treatment of the incriminating psychosocial aspects in TS are scarce. In this work, we first reviewed the literature on the cognitive and psychosocial development of girls with TS compared with normal development, from disclosure to young adulthood, and then introduce a psychosocial approach to counseling and treating patients with TS, including recommendations for age-appropriate psychological diagnostics. With this work, we aim to facilitate the integration of emphasized psychosocial care in state-of-the-art treatment for girls and women with TS.

  15. Relationship between Obesity-related Hormone Peptides and Quality of Life in Obese Women among Different Traditional Chinese Medicine Syndrome Groups

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    You-Lung Song

    2012-01-01

    Full Text Available The aim of this study was to explore the relationship between obesity-related hormone peptides and quality of life in obese women among different traditional Chinese medicine (TCM syndrome groups (證型 zhèng xíng. 260 obese women met with age between 20 and 65 years old and body mass index (BMI ≧ 27 kg/m2, were recruited. The participants filled out a questionnaire on obese TCM syndrome groups, which was designed by professional TCM doctors, and two questionnaires on quality of life (QOL, WHOQOL-BREF Taiwan version and MOS Short Form-12 (SF-12. Data of biochemical characteristics and obesity-related hormone peptides were collected at the same time. According to the responses provided, the obese subjects were classified into spleen deficiency with dampness encumbrance syndrome (脾虛濕阻證 pí xū shī zǔ zhèng; SDD, stomach heat with dampness encumbrance syndrome (胃熱濕阻證 wèi rè shī zǔ zhèng; SHD, liver depression and qi stagnation syndrome (肝鬱氣滯證 gān yù qì zhì zhèng; LDQ, dual spleen-kidney deficiency syndrome (脾腎兩虛證 pí shèn liǎng xū zhèng; SKD, yin deficiency with internal heat syndrome (陰虛內熱證 yīn xū nèi rè zhèng; YDI and a control group. For physical conditions, SDD group had significantly higher means in weight and BMI compared with the control group. The insulin and leptin levels in SHD group were significantly higher than those in the control group. The LDQ group showed marked decrease in mental condition scores compared with the control group. This study found that obese women in the SDD group were fatter than those in the control group. SHD group might have greater influence on the regulation of obesity-related hormone peptides. The LDQ group had poor QOL than the control group. Analysis of TCM syndrome groups among obese women merits further investigation.

  16. Relationship between Obesity-related Hormone Peptides and Quality of Life in Obese Women among Different Traditional Chinese Medicine Syndrome Groups.

    Science.gov (United States)

    Song, You-Lung; Lien, Chun-Ying; Chiu, Jung-Peng; Luo, Ching-Min; Liu, Chia-Yu; Chen, I-Ju; Hsu, Chung-Hua

    2012-01-01

    The aim of this study was to explore the relationship between obesity-related hormone peptides and quality of life in obese women among different traditional Chinese medicine (TCM) syndrome groups ( zhèng xíng). 260 obese women met with age between 20 and 65 years old and body mass index (BMI) ≧ 27 kg/m(2), were recruited. The participants filled out a questionnaire on obese TCM syndrome groups, which was designed by professional TCM doctors, and two questionnaires on quality of life (QOL), WHOQOL-BREF Taiwan version and MOS Short Form-12 (SF-12). Data of biochemical characteristics and obesity-related hormone peptides were collected at the same time. According to the responses provided, the obese subjects were classified into spleen deficiency with dampness encumbrance syndrome ( pí xū shī zǔ zhèng; SDD), stomach heat with dampness encumbrance syndrome ( wèi rè shī zǔ zhèng; SHD), liver depression and qi stagnation syndrome ( gān yù qì zhì zhèng; LDQ), dual spleen-kidney deficiency syndrome ( pí shèn liǎng xū zhèng; SKD), yin deficiency with internal heat syndrome ( yīn xū nèi rè zhèng; YDI) and a control group. For physical conditions, SDD group had significantly higher means in weight and BMI compared with the control group. The insulin and leptin levels in SHD group were significantly higher than those in the control group. The LDQ group showed marked decrease in mental condition scores compared with the control group. This study found that obese women in the SDD group were fatter than those in the control group. SHD group might have greater influence on the regulation of obesity-related hormone peptides. The LDQ group had poor QOL than the control group. Analysis of TCM syndrome groups among obese women merits further investigation.

  17. Vascular Ehlers-Danlos Syndrome With a Novel Missense COL3A1 Mutation Present With Pulmonary Complications and Iliac Arterial Dissection.

    Science.gov (United States)

    Gu, Guangchao; Yang, Hang; Cui, Lijia; Fu, Yuanyuan; Li, Fangda; Zhou, Zhou; Zheng, Yuehong

    2018-02-01

    Vascular Ehlers-Danlos syndrome (vEDS) is a life-threatening connective tissue disorder due to its high tendency of arterial and organ rupture. Pulmonary complications in vEDS are rare. We present a young male patient with vEDS who developed severe pulmonary complications and severe rupture of the iliac artery at different stages of his life. Vascular Ehlers-Danlos syndrome was diagnosed based on clinical manifestations and confirmed by the identification of COL3A1 gene mutation. Due to high bleeding tendency and weak cardiopulmonary capacity, conservative treatment was taken for him. To our knowledge, this is the first report of vEDS case in which the patient developed both pulmonary complications and dissection of large arteries. Our report emphasizes the importance of considering vEDS when an adolescent develops unexplained pulmonary cysts with fragility of lung tissues. Genetic counseling and close monitoring should be performed for earlier diagnosis and prevention of severe complications of large arteries. The typical presentations of vEDS were also discussed by means of a review of case reports on vEDS with pulmonary complications.

  18. Two cases of humoral hypercalcemia of malignancy complicating infantile fibrosarcoma.

    Science.gov (United States)

    Hirschfeld, Ryan; Welch, Jennifer J G; Harrison, Douglas J; Kremsdorf, Robin; Chawla, Anjulika

    2017-10-01

    We report two infants with infantile fibrosarcoma (IFS) complicated by severe hypercalcemia. Assessment demonstrated suppressed parathyroid hormone and 1,25-dihydroxyvitamin D levels with elevated circulating levels of parathyroid hormone related protein, indicating the diagnosis of humoral hypercalcemia of malignancy (HHM). HHM is a paraneoplastic syndrome rarely associated with pediatric malignancies. Hypercalcemia manifested clinically with neurologic symptoms and soft tissue calcium deposition and required aggressive management with intravenous fluids, diuretics, and supplemental electrolytes. Following treatment with neoadjuvant chemotherapy, serum calcium levels precipitously declined requiring calcium repletion. These cases highlight the improvement of hypercalcemia secondary to HHM following chemotherapy. © 2017 Wiley Periodicals, Inc.

  19. Venous thrombo-embolism as a complication of cross-sex hormone treatment of male-to-female transsexual subjects: a review.

    Science.gov (United States)

    Asscheman, H; T'Sjoen, G; Lemaire, A; Mas, M; Meriggiola, M C; Mueller, A; Kuhn, A; Dhejne, C; Morel-Journel, N; Gooren, L J

    2014-09-01

    Administration of cross-sex hormones to male-to-female transsexual subjects, usually oestrogens + often anti-androgens, such as cyproterone acetate, carries a risk of venous thromboembolism (VTE). VTE usually occurs in the first year of oestrogen administration. Ethinyl oestradiol, due to its chemical structure, was in 2003 identified as a major factor in the occurrence of VTE. Most clinics do not prescribe ethinyl oestradiol any longer, but people who take hormones without medical supervision use often oral contraceptives containing ethinyl oestradiol, many times in overdose. Cessation of use of ethinyl oestradiol and peri-operative thrombosis prophylaxis for surgery have reduced prevalence rate of VTE. Other oral oestrogens should not be overdosed, and transdermal oestrogen is to be preferred. Thrombosis prophylaxis for surgery is mandatory. It seems advisable to stop hormone use at least 2 weeks before major surgery, to be resumed only after 3 weeks following full mobilisation. © 2013 Blackwell Verlag GmbH.

  20. Impact of growth hormone therapy on quality of life in adults with turner syndrome.

    Science.gov (United States)

    Amundson, Emily; Boman, Ulla Wide; Barrenäs, Marie-Louise; Bryman, Inger; Landin-Wilhelmsen, Kerstin

    2010-03-01

    GH and/or oxandrolone are used to promote growth in Turner syndrome (TS). The aim of this study was to compare quality of life (QoL) in TS women with controls and determine the impact of growth promoting therapy on QoL in TS women. This was a cross-sectional, case-control study. The study was conducted at an outpatient clinic at Sahlgrenska University Hospital, Göteborg, Sweden. PATIENTS included 111 TS women (age range 18-59 yr) and 111 randomly selected, age-matched women (25-54 yr) from the World Health Organization Monitoring Trends and Determinants for Cardiovascular Disease project (Göteborg, Sweden) served as controls. QoL was estimated by the Psychological General Well-Being scale (anxiety, depressed mood, positive well-being, self-control, general health and vitality) and the Nottingham Health Profile (physical mobility, pain, sleep, energy, social isolation, and emotional reactions). TS women reported more social isolation than controls (P < 0.001). After age adjustment, significantly less pain (<0.05) was reported attributable to GH treatment within TS. No significant difference in any other subscales used could be shown. In TS, QoL was negatively affected by higher current age and age at diagnosis and positively affected by better body balance, fine motor function, and higher bone mineral density. Social isolation was more commonly reported in the whole TS cohort than in the population. Except for less pain, no significant impact on QoL attributable to GH treatment could be found, despite the mean +5.1 cm final height.