Background: Gamma-glutamyltransferase (GGT) enzyme has an increasing importance in the pathophysiology and prognosis of cardiovascular diseases. It is an indirect marker of microvascular endothelial dysfunction, atherosclerosis, and elevated oxidative stress. There are no adequate data on the relationship between GGT and cardiac syndrome X. Aim: To compare serum GGT levels ...

Rett syndrome is a neurodevelopmental disorder typically caused by mutations in methyl-CpG-binding protein 2 (MECP2) in which 26% of deaths are sudden and of unknown cause. To explore the hypothesis that these deaths may be due to cardiac dysfunction, we characterized the electrocardiograms in 379 people with Rett syndrome and ...

Abstract  We report the case of a 58-year-old patient with Gilbert's syndrome and multiple cardiovascular pathologies, including aortic regurgitation with a dilated aortic root, severe mitral regurgitation, and chronic atrial fibrillation. A Bentall procedure, mitral valve repair, and modified radiofrequency MAZE procedure were performed. The management of Gilbert's syndrome in ...

Objectives: To indicate cardiogenic shock as a very rare but serious clinical consequence of untreated panhypopituitarism secondary to Sheehan's syndrome; to emphasize the importance of eliciting a detailed endocrine and obstetric history in women presenting with idiopathic heart failure; to indicate the diagnostic shortcomings of screening for thyroid dysfunction solely ...

Broken heart syndrome or stress-induced non-ischemic cardiomyopathy (SICM) has rarely been reported in the setting of myasthenic crisis. We describe a case of reversible SICM that occurred simultaneously with myasthenic crisis in a 77-year-old man without prior cardiac history, and we discuss the previous findings that support an association of ...

Improved survival in children with hypoplastic left heart syndrome has created a sub-population of children and young adults who are living with functionally univentricular physiology. Routine surveillance with comprehensive screening for structural cardiac disease, functional cardiac disease, arrhythmias, thromboembolic disease, and associated dysfunction of end organs is important. Future directives ...

Situs ambiguous is rare congenital anomaly in adults. In 2 adult patients who admitted for different cardiac problems, situs ambiguous with polysplenia was detected. A 42-year-old male admitted for radio frequent catheter ablation of atrial fibrillation, and he had left-sided inferior vena cava (IVC), hepatic segment of IVC interruption with ...

This report describes a 34-year-old male with the Wolff-Parkinson-White syndrome who presented with the unusual finding of a tachyarrhythmia-induced cardiomyopathy secondary to atrial flutter with 1:1 conduction through a left-lateral accessory pathway. Catheter ablation of the accessory connection resulted in complete normalization of cardiac function.

A 40-year-old white male received cardio-pulmonary resuscitation after cardiac arrest due to an epileptic status. Four months after the incident he developed an akinetic-rigid syndrome and a postural tremor more pronounced on the right side of the body. Brain imaging revealed bilateral lesions of the putamen and caudate nucleus. Levodopa improved ...

Congenital heart disease is one of the most common human birth defects, yet many genes and pathways regulating heart development remain unknown. A recent study in humans revealed that mutations in a single Hox gene, HOXA1 (Athabascan Brainstem Dysgenesis Syndrome, Bosley-Salih-Alorainy Syndrome) can cause severe cardiovascular malformations, some of which ...

Mesothelial/monocytic incidental cardiac excrescence (MICE) is a rare benign lesion composed of a mixture of histiocytes, mesothelial cells, fibrin, adipocytes and scattered inflammatory cells without a vascular network or supporting stroma. Its pathogenesis is controversial with some authors favoring an artifactual theory while others consider a reactive phenomenon. To date, ...

: Takotsubo syndrome, also known as ampulla cardiomyopathy, broken heart syndrome, idiopathic apical ballooning syndrome, and stress-induced myocardial stunning, has been first described by Japanese authors in 1996 and subsequently specified in 2001; it derives from the resemblance between the ancient round-bottomed, narrow-necked Japanese fishing pots used to trap octopus ...

Acquired and hereditary long-QT syndromes are important causes of sudden cardiac death. Both categories are characterized by abnormally prolonged cardiac repolarization arising from a complex interaction between genetic and environmental factors. This produces a potentially dangerous substrate for polymorphic ventricular tachycardia and sudden cardiac death. In this review, the pathophysiologic, ...

Noonan syndrome (NS) is one of the most common non chromosomal syndrome presenting to the cardiac anesthesiologist for the management of various cardiac lesions, predominantly pulmonary stenosis (PS) (80%) and hypertrophic obstructive cardiomyopathy (HOCM) (30%). The presence of HOCM in NS makes these children susceptible to acute congestive heart failure ...

Brugada syndrome (BS) is a cardiac rhythm disturbance that predisposes patients to sudden cardiac death. Brugada is classically described with specific electrocardiographic (EKG) findings of ST elevation and right bundle branch block in precordial leads and is an often unrecognized contributor to sudden cardiac death. We present a case of ...

Abstract  Atrial myxomas are the most common benign tumors of the heart and are difficult to diagnose due to a wide variety of presenting symptoms. We present a patient with a five-year history of visual loss, vertigo, ataxia, tinnitus, and bone lesions that resolved after diagnosis and resection of an ...

Cantrell syndrome is a rare condition of congenital defects of 5 developmentally associated structures: the abdominal wall, sternum, diaphragm, pericardium, and heart. Few patients survive, and even fewer survive with good outcomes of quality of life. We present a case with the pentalogy and profound cardiac malformations. Despite repeated life-threatening ...

Resuscitation and the development of abdominal compartment syndrome (ACS) are closely associated and frequently overlapping critical care topics. Elevated intra-abdominal pressure (IAP) can cause major deterioration of cardiac function by affecting preload, contractility, and afterload. Pathologically elevated IAPs are often compounded by the presence of shock leading to imminent organ ...

We report the case of a 12-year-old girl with Tako-tsubo cardiomyopathy. She was successfully treated in our Pediatric Intensive Care Unit for acute left ventricular failure, which occurred after a brief submersion in sea water. At 2-month follow-up she showed an almost complete recovery of cardiac function. We consider Tako-tsubo ...

PURPOSE: Cardiac pheochromocytoma with multiple endocrine neoplasia syndrome (MENS) is rare. We present a rare case of concurrent cardiac pheochromocytoma and pituitary adenoma, a rare variant of the MEN syndromes and a review of the literature with special emphasis on diagnosis and treatment. METHODS: Different from the single MENS type ...

Although pericardial effusion is a well-known feature of Churg-Strauss syndrome, cardiac tamponade has rarely been encountered. The present report describes a case of Churg-Strauss syndrome that presented as an acute cholecystitis and was complicated by tamponade. Histopathological exam of both pericardium and gall bladder was conclusive for Churg-Strauss syndrome.

Sudden cardiac arrest (SCA) in children is a rare, but catastrophic event. Children with cardiac pathology at particular risk include those with congenital long QT syndrome (CLQTS) and hypertrophic cardiomyopathy. CLQTS is a genetic disorder of the cardiac ion channels and is associated with significant risk of malignant ventricular arrhythmias ...

Mounier-Kuhn syndrome is a rare condition that combines tracheobronchomegaly (TBM) and severe tracheobronchomalacia. Symptoms can be severe with recurrent bronchopulmonary infections and cough-induced syncope. Therapeutic management is non-specific and limited to chest physiotherapy and antibiotics during infectious exacerbations. We report a case of Mounier-Kuhn syndrome that was successfully managed by ...

It is well recognized that vitiligo displays the Koebner phenomenon. However, the impact of this phenomenon on hair removal in these patients has little been considered. This is a challenging case of a woman with hirsutism and vitiligo who opted to have laser hair removal treatment.

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Down syndrome is the commonest chromosomal anomaly. It is often associated with hypothyroidism, which may rarely present with cardiac tamponade as the earliest manifestation and prompt treatment with L-thyroxine is life saving. A six-month-old female child diagnosed as a case of Down syndrome presented with shortness of breathing for last ...

The cardiorenal syndrome (CRS) is a complex clinical syndrome in which dysfunction of either the heart or the kidneys affects the functioning of the other organ system. Many therapies used in heart failure have further detrimental effects on renal function. Cardiac resynchronization therapy (CRT) is a relatively new form of ...

A 16-year-old boy affected by Sotos syndrome was referred to our clinic for cardiac evaluation in order to play noncompetitive sport. Physical examination was negative for major cardiac abnormalities and rest electrocardiogram detected only minor repolarization anomalies. Transthoracic echocardiography showed left ventricular wall thickening and apical trabeculations with deep intertrabecular ...

Obesity and hypertension, major risk factors for the metabolic syndrome, render individuals susceptible to an increased risk of cardiovascular complications, such as adverse cardiac remodeling and heart failure. There has been much investigation into the role that an increase in the renin-angiotensin-aldosterone system (RAAS) plays in the pathogenesis of metabolic ...

A 2-year-old black girl was diagnosed with Sweet syndrome and cutis laxa, and she was given corticosteroid therapy. At that presentation, a cardiac evaluation revealed nothing unusual. Nine days later, she emergently presented with respiratory distress, and circulatory collapse rapidly developed. A 2-dimensional Doppler echocardiogram showed a dilated and poorly ...

Toxic epidermal necrolysis (TEN) and Stevens-Johnson syndrome (SJS) are rare but severe cutaneous adverse reactions related to a variety of medications. Although rare, SJS and TEN have a significant impact on public health because of high mortality. Also, if the SJS/TEN physiopathology is still unclear, a specific immune response to ...

Life-threatening dermatologic conditions include Rocky Mountain spotted fever; necrotizing fasciitis; toxic epidermal necrolysis; and Stevens-Johnson syndrome. Rocky Mountain spotted fever is the most common rickettsial disease in the United States, with an overall mortality rate of 5 to 10 percent. Classic symptoms include fever, headache, and rash in a patient ...

Stevens-Johnson syndrome (SJS) and its more severe variant, toxic epidermal necrolysis (TEN), are relatively rare but have high mortality rates. Survivors are frequently afflicted with severe blinding ocular surface diseases. Recent literature in the areas of ophthalmology and dermatology illustrate that the ophthalmologist's role should no longer be considered secondary ...

Toxic anterior segment syndrome is an acute, sterile, anterior segment inflammation that occurs after any anterior segment surgery. The condition is an increasingly recognized complication of adult cataract surgery. Here we report the clinical findings of a case of toxic anterior segment syndrome in a child after uneventful congenital cataract ...

Fluconazole uncommonly causes Stevens-Johnson syndrome. A young Indian man who developed this adverse effect after his second dose of fluconazole is described. The characteristics of previously reported individuals with fluconazole-associated Stevens-Johnson syndrome and toxic epidermal necrolysis are also summarized: three out of five of the patients were immunocompromised, five out ...

We describe an uncommon cause of cardiogenic shock following cesarean delivery in a 24-year-old multiparous woman at 26 weeks of gestation. Hemodynamic instability was erroneously attributed to amniotic infection syndrome and sepsis, which resulted in delayed diagnosis and treatment of tuberculous constrictive pericarditis. Inotropic support, pericardectomy, and implantation of a ...

Most drug hypersensitivity reactions show skin symptoms. The most severe cutaneous manifestations include pustular and bullous skin eruptions. These 2 manifestations can lead to acute generalized exanthematous pustulosis or Stevens-Johnson syndrome and toxic epidermal necrolysis. These complications are rare, but should be known to any doctor prescribing drugs because they ...

Stevens-Johnson syndrome and toxic epidermal necrolysis are rare; however, when they occur, they usually present with severe reactions in response to medications and other stimuli. These reactions are characterized by mucocutaneous lesions, which ultimately lead to epidermal death and sloughing. We present a unique case report of Stevens-Johnson syndrome and ...

The causative factors and ocular complications of Stevens-Johnson syndrome and toxic epidermal necrolysis are reported here. Six out of seven patients developed the syndrome secondary to ingestion of sulphadoxine/pyrimethamine while one developed it as a complication of HAART (highly active antiretroviral therapy). The ocular complications were ankyloblepharon, symblepharon, chronic conjunctivitis, ...

Toxic epidermal necrolysis (Lyell syndrome) is a rare, acute, and potentially life-threatening mucocutaneous disease that is most often triggered by drugs. This is the first case of toxic epidermal necrolysis because of treatment with etofenamate of which we are aware.

Effusive-constrictive pericarditis is a clinical syndrome characterized by concurrent pericardial effusion and pericardial constriction, where constrictive hemodynamics are persistent after effusion is drained. It may present at any point along the clinical course, from the occurrence of an effusion to the development of chronic pericardial constriction. We refer an unusual ...

Toxic epidermal necrolysis (TEN) is characterized by fever, scalded appearance of the skin, and epidermolysis associated to blister formation and exfoliation, and it is caused by hypersensitivity reaction to a drug. The authors report two cases of death as a result of TEN; both referred to old aged women treated ...

The Central Toxic Keratopathy (CTK) syndrome describes a rare, acute, self-limited, non-inflammatory process that yields central corneal opacification and significant hyperopic shift after refractive surgery. Despite being exceedingly rare, certain clinical features of CTK give the condition a striking resemblance to other more serious inflammatory conditions, including diffuse lamellar keratitis ...

Nonpigmenting fixed drug eruption (NPFDE) is clinically indistinguishable from Stevens-Johnson syndrome (SJS) or toxic epidermal necrolysis (TEN) in its initial presentation. The traditional paradigm that epidermal changes are absent in NPFDE cannot be easily reconciled with the clinical resemblance to SJS/TEN. We therefore investigated whether NPFDE is pathogenetically different from ...

PURPOSE: To document a case of contact lens-induced keratitis resembling central toxic keratopathy syndrome. METHODS: A 23-year-old female developed an acute, central, stromal haze subsequent to soft contact lens overwear. Slit lamp examination revealed corrugated stromal "mud cracks," as seen in stage IV diffuse lamellar keratitis (DLK). This was accompanied ...

Stevens-Johnson syndrome and its more severe variant, toxic epidermal necrolysis, have relatively low overall incidence; however, this disease presents with high morbidity and mortality. The majority of patients develop ocular inflammation and ulceration at the acute stage. Due to the hidden nature of these ocular lesions and the concentration of ...

Clidinium bromide (N-methyl-quinuclidinyl-benzylate) is a rarely used antimuscarinic drug that is marketed in combination with chlordiazepoxide as an antispasmodic for use in irritable bowel syndrome. A case is reported of an accidental staggered overdose of clidinium bromide 50 mg in a patient using illicit chlordiazepoxide. The presenting features were mildly ...

Serotonin (5-hydroxytryptamine; 5-HT)-toxicity syndrome, an iatrogenic brain disorder induced by excessive efflux of 5-HT, has received much attention because of increasing incidents of serotonergic antidepressants. However, the neural mechanism by which extracellular 5-HT is elevated to a toxic level for the syndrome remains to be determined. The goal of the ...

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare and serious cutaneous adverse reactions. There is controversy over the use of intravenous immunoglobulin (IVIG) in the treatment of SJS or TEN. The lack of randomized controlled trials to assess the benefits and risks of IVIG is due to its ...

1) Lyell syndrome is characterised by toxic epidermal necrolysis in which epidermal detachment affects more than 30% of the body surface area. Stevens-Johnson syndrome is a minor form affecting less than 10% of the body surface area; 2) Patients who present with these cutaneous symptoms, along with throat pain, red ...