Hypertrophic osteoarthropathy secondary to Eisenmenger syndrome can produce severe disabling pain. We report two cases treated by intravenous pamidronate infusion with good symptomatic relief. Such therapy can greatly improve the quality of life of end-stage cyanotic congenital heart patients.

Adams-Oliver syndrome (AOS) is a rare condition defined by combination of cutis aplasia and transverse limb abnormalities. Some authors have described a possible association between this syndrome and portal hypertension (PH) due to hepatoportal sclerosis (HPS). We present a boy with AOS who developed a progressive splenomegaly and hypersplenism at ...

  Dyshidrotic eczema is one of the rare cutaneous adverse effects of intravenous immunoglobulin therapy, usually seen in adults. We herein report the first pediatric case of severe dyshidrotic eczema occurring after intravenous immunoglobulin therapy for Kawasaki syndrome.

ABSTRACT: The Ganser syndrome is rare in children and in adolescents. A case of Ganser syndrome in a 14-year-old girl, with three of the four essential features, is presented. After rapid resolution in two weeks, Ganser symptoms reappear seven months later accompanied, this second time, by previous depressive symptoms. This ...

BACKGROUND: Sclerosing mediastinitis (ScM) is a rare, potentially life-threatening disorder, idiopathic in roughly half the cases. Systemic symptoms not attributable to sclerosis often appear in idiopathic ScM. Mast cell activation disease (MCAD) is a potential cause of these symptoms and also can cause sclerosis. ScM has not previously been associated ...

Alendronate is a widely used bisphosphonate in the treatment of osteoporosis. Although it has been proven to be a very useful drug, it has some side effects as well. In this paper, we describe a case of nephrotic syndrome due to alendronate administration. A 36-year-old man was admitted to the ...

ABSTRACT: INTRODUCTION: McCune-Albright syndrome is a complex inborn disorder due to early embryonal postzygotic somatic activating mutations in the GNAS1 gene. The phenotype is very heterogeneous and includes polyostotic fibrous dysplasia, typically involving the facial skull, numerous cafe-au-lait spots and autonomous hyperfunctions of several endocrine systems, leading to hyperthyroidism, hypercortisolism, ...

Background Demographic differences in health outcomes have been reported for chronic diseases, but few data exist on these differences in trauma (defined as acute, life-threatening injuries). Objective To investigate the relationship between the systemic inflammatory response syndrome score after trauma and race/ethnicity and socioeconomic status. Methods A retrospective chart review ...

The occurrence of nephrotic syndrome following a bee sting is rarely reported in the literature. Hypersensitivity is believed to be the precipitating factor for the renal disease. We report a two-year-old boy, who developed generalized edema and decreased urine output, seven days after a bee sting. Physical examination and laboratory ...

We report a case of Churg-Strauss syndrome (CSS) in a patient with multiple cerebral infarctions and psychotic symptoms. A 67-year-old man presented a high-grade fever and delirium. He was clinically diagnosed with Churg-Strauss syndrome on the basis of the presence of asthma, neuropathy, blood eosinophilia, and increased myeloperoxidase-specific anti-neutrophil cytoplasmic ...

A case of gallbladder malignant melanoma in a 62-year-old woman is reported. The gallbladder, resected for tumor mass seen on ultrasound examination, revealed neoplastic epithelioid cells with dark granules in the cytoplasm. These cells with pigment were positive immunohistochemically for S-100, HMB45 and vimentin. The patient, affected by dysplastic nevus ...

Treating the elderly is often problematic, especially when congenital heart disease is diagnosed. The aim of this study is to present the case of a 72-year old woman with depression syndrome, paroxysmal atrial fibrillation and past cerebral stroke, in whom persistent ductus arteriosus Botalli was diagnosed. Due to exacerbation of ...

Dystonia in association with peripheral trauma is a well-described clinical syndrome. The syndrome goes by many names--"traumatic" dystonia, "fixed" dystonia, peripherally induced dystonia, or complex region pain syndrome (CRPS) dystonia. We reviewed the role of peripheral trauma in the development of dystonia, focusing on 4 subtypes--cervical dystonia, focal limb dystonia, ...

Reflex sympathetic dystrophy syndrome (RSDS) is a painful condition that usually follows regional trauma. We report the case of a 13-year-old girl that was seen for a painful swelling of the right hand associated with palmar hyperhidrosis, which occurred after a trauma to the hand. Bone scan images showed early ...

In the past decade, major progress has been made in our understanding of fibromyalgia syndrome (FMS). Various triggers have been implicated as contributing to symptom development in FMS when genetically susceptible individuals are challenged. A substantial amount of data points towards the association between trauma and chronic widespread pain/fibromyalgia syndrome ...

Currently, significant amount of epidemiologic evidence is present to suggest that metabolic syndrome increases the risk of developing colorectal cancer. This evidence is based on studies of the evaluate determinants of the metabolic syndrome (obesity), clinical consequences of metabolic syndrome (type 2 diabetes and hypertension) and serum component of metabolic ...

Moyamoya vascular pattern and dural arteriovenous fistula (dAVF) are rare vascular abnormalities and both can be secondary to head trauma. The role of dural angiogenesis in the pathophysiology of vascular malformation is rather unclear. We report a unique case of moyamoya vasculopathy simultaneously associated with dAVF after heavy head trauma. ...

Much evidence supports a pivotal role for inflammation in atherosclerosis. C-reactive protein (CRP), the prototypic marker of inflammation in humans, is a cardiovascular risk marker and may also promote atherogenesis. CRP levels are increased in metabolic syndrome and hypertension and confer increased risk of cardiovascular events in patients in these ...

Metabolic syndrome is a collection of anthropomorphic and metabolic derangements that, taken together, constitute a strong predictor of stroke, cardiovascular disease, type 2 diabetes, and perhaps other disorders. Diagnosing the metabolic syndrome according to presently accepted definitions requires a mixture of anthropomorphic and laboratory measurements. In indigent patient populations or ...

This case report describes how eculizumab reversed neurologic impairment and improved renal damage in severe atypical hemolytic uremic syndrome. A 50-year-old female, after presenting with diarrhea and abdominal pain, developed pancolitis, acute renal failure, and thrombocytopenia. The patient underwent total abdominal colectomy. Pathology confirmed ischemic colitis with scattered mesenteric microthrombi. ...

To present a case of Terson's syndrome consequent to ruptured intracranial aneurism treated successfully with a combined therapeutic approach. Case report. A 37 year old man was urgently referred to the UCC Tuzla with sudden headache, vomiting and unconsciousness. CT scan showed ruptured aneurism of anterior communicant artery with signs ...

Alström syndrome (AS) is a very rare autosomal recessively inherited disorder that can lead to infantile-onset dilated cardiomyopathy, blindness, hearing impairment, obesity, diabetes, hepatic and renal dysfunction. AS is caused by mutations in the ALMS1 gene, which is located at chromosome 2p13. The life span of patients with AS rarely ...

Following on from impressive economic development and urbanization, China is currently experiencing a high prevalence of metabolic syndrome. Patients with metabolic syndrome suffer from the "The Deadly Quartet" of hyperglycemia, hypertriglyceridemia, hypertension, and central (or upper body) obesity. Current treatment strategies directed towards metabolic syndrome tend to be limited to ...

The prevalence of the metabolic syndrome is increased in patients with schizophrenia compared with the general population. The strong interindividual differences in susceptibility to developing the metabolic syndrome suggests that the genetic makeup is a modulating factor. Part of the genetic puzzle can possibly be explained by variations in the ...

We tested the hypothesis that high cardiorespiratory fitness (fitness) is associated with lower levels of arterial stiffness in 1035 (age 52±6 years) men with and without the metabolic syndrome. Arterial stiffness was derived from brachial-ankle pulse wave velocity (baPWV). Fitness was directly measured by peak oxygen uptake during a standard ...

Metabolic syndrome has been proposed as a risk factor for stroke and transient ischaemic attack. One pathophysiological mechanism could be impairment of endothelial function. Thus, we hypothesized that cerebral vasomotor reactivity would be decreased in patients with metabolic syndrome, compared to patients without metabolic syndrome. In this retrospective analysis, 83 ...

Acne keloidalis nuchae (AKN) is an inflammatory disease related to the spectrum of keloidal scarring alopecias. No association with internal disease has been described so far. We describe a case series of four Indian patients who showed an association of AKN with acanthosis nigricans, a skin marker for the metabolic ...

 The prevalence of the metabolic syndrome is increasing, especially in young individuals. Most of the previous studies that have investigated the association between dietary fibre intake and the metabolic syndrome are cross-sectional or of short duration, and their results are inconsistent. The present study investigated whether dietary fibre intake during ...

In this study, we assessed whether the apolipoprotein B/apolipoprotein A1 ratio (ApoB/ApoA1) is related to metabolic syndrome (MS) and its components in an urban Chinese population. A total of 709 community residents were enrolled. Metabolic syndrome was defined according to the International Diabetes Federation definition in 2005. The high ApoB/ApoA1 ...

The metabolic syndrome is conceptualized as a clustering of risk factors--including insulin resistance, dyslipidemia, central adiposity, and elevated blood pressure (BP)--that increase the risk for cardiovascular disease and type 2 diabetes mellitus. Recent evidence suggests that markers of systemic inflammation may be included in the definition of the syndrome and ...

The metabolic syndrome is becoming increasingly prevalent in the general population and carries significant incremental morbidity and mortality. It is associated with multi-organ involvement and increased all-cause mortality, resembling a precocious aging process. The mechanisms that account for this phenomenon are incompletely known, but it is becoming clear that longevity ...

Background. Metabolic syndrome is a risk factor for nephrolithiasis. This study was performed to evaluate the clinical and biochemical profile of calcium-oxalate nephrolithiasis in stone formers with metabolic syndrome. Methods. A total of 526 recurrent stone formers, 184 of them with metabolic syndrome, and 214 controls were examined on a ...

Metabolic syndrome has been associated with impaired health-related quality of life (HRQoL) in several studies. Many studies used only one HRQoL measure and failed to adjust for important confounding variables, including obesity, depression and comorbid conditions. To investigate the relationship between metabolic syndrome and HRQoL using multiple measures. We also ...

Psoriasis is a chronic inflammatory disease of the skin and is associated with an increased risk of cardiovascular atherosclerosis. Metabolic syndrome, a conglomerate of various clinical and biochemical parameters is a significant predictor of atherosclerotic disease and the associated risk for cardiovascular events in such patients. To investigate the prevalence ...

The aim was to explore the prevalence of five components of metabolic syndrome with respect to gender and health-promoting lifestyle behaviours. Age- and gender-specific strategies might be useful as an approach to controlling metabolic syndrome. Prevention or delaying the onset of metabolic syndrome is of utmost importance in terms of ...

The metabolic syndrome is associated with increased risk for development of both cardiovascular disease and type 2 diabetes in humans. Because experimental data and clinical experience have shown that metabolic syndrome and caloric restriction have, at least partly, opposite pathophysiological pathways, the activation of sirtuins may constitute a pharmacological approach ...

Anthropometric indices to obesity were evaluated as predictors of metabolic syndrome risk factors. Our purpose was to explore an optimal or more reliable anthropometric indicator and optimal cut-off points for obesity on metabolic syndrome in Chinese adults. The survey was conducted involving 2947 participants, aged 20 or above with cross-sectional ...

Metabolic syndrome has been reported to have adverse effects on cognition although the results are conflicting. We investigated the association between metabolic syndrome and cognitive function in a population sample of middle-aged and older European men and whether any observed association could be explained by lifestyle or other confounding factors. ...

The metabolic syndrome is associated with nonalcoholic fatty liver disease (NAFLD) as well as with insulin resistance, inflammatory adipokines, endothelial dysfunction, and higher plasma levels of nonesterified fatty acids (NEFA), all of which may also affect the development of NAFLD. Therefore, we investigated to what extent the association between the ...

Abstract Aims: Obstructive sleep apnea/hypoapnea syndrome (OSAHS) and metabolic syndrome are both growing health concerns, owing to the worldwide obesity epidemic. This study evaluates the potential association between the parameters of metabolic syndrome and OSAHS. Methods: A total of 125 consecutive patients undergoing complete full night polysomnography were included. Insulin ...

Some reports show that the prevalence of metabolic syndrome is higher in poorly educated women. In our opinion, one probable reason for this is that these women experience more stressful events in their lives. We investigated the association between major stressful life events and the prevalence of metabolic syndrome and ...

New-onset diabetes after transplantation (NODAT) is a major posttransplant complication associated with lower allograft and recipient survival. Our objective was to determine whether metabolic syndrome pretransplant is independently associated with NODAT development. We recruited 640 consecutive incident nondiabetic renal transplant recipients from three academic centers between 1999 and 2004. NODAT ...

To estimate the prevalence of metabolic syndrome (MetS) in patients with primary antiphospholipid syndrome (PAPS). Cross sectional study of PAPS patients from the outpatient clinic. MetS was based on three international definitions: World Health Organization (WHO), Adult Treatment Panel III (ATP III), or International Diabetes Federation (IDF). Clinical history and ...

Several studies have focused on the relationship between metabolic syndrome and gastroesophageal reflux disease (GERD). They were based on GERD complications, whereas little is known about the association between metabolic syndrome and objectively measured esophageal acid exposure. The aim of our study was to assess the relationship between metabolic syndrome ...

Life expectancy of people with intellectual disabilities (IDs) is rapidly normalizing, as are prevalences of age-related cardiovascular disease in this group. We investigated the prevalence of the metabolic syndrome in the older population with ID and associations with patient characteristics. Prevalence of the metabolic syndrome, according to National Cholesterol Education ...

The metabolic syndrome is associated with coronary artery disease (CAD) and with peripheral artery disease (PAD), but the underlying mechanisms explaining these associations have not yet been completely clarified. The aim was to investigate (i) whether endothelial dysfunction can explain the association between the metabolic syndrome and CAD and/or the ...

Laparoscopic sleeve gastrectomy (LSG) is a common bariatric procedure that has several advantages over Roux-en-Y gastric bypass, but data on the effectiveness of this procedure on metabolic syndrome have rarely been reported. This case control study compared the incidence of low grade systemic inflammation, insulin resistance, anthropometrics, lipid disturbances, and ...

Metabolic syndrome (MetS) is a widely prevalent and multi-factorial disorder. The syndrome has been given several names such as insulin resistance (IR) syndrome, plurimetabolic syndrome, Reaven's syndrome, Syndrome X, and the deadly quartet. The formulation of National Cholesterol Education Program Adult Treatment Panel III (NCEP-ATP) guidelines has led to some ...

Iron overload is a major cause of morbidity and mortality in transfusion-dependent anemias. Greater understanding of the mechanisms of iron regulation and the role of toxic iron moieties and their predilection for certain organs has enhanced our ability to better target therapies in recent years, as illustrated by the Thalassemia ...

The prevalence rates of obesity and metabolic syndrome are on the rise in the United States. Epidemiological surveys suggest that the rates of these medical conditions are especially high among persons with psychiatric disorders, including post-traumatic stress disorder (PTSD). A variety of factors are thought to contribute to the risk ...