Miller Fisher syndrome: a case with pattern of pure sensory polyneuropathy concomitant with anti-GQ1B antibody

Miller Fisher syndrome is characterized by the acute onset of ophthalmoplegia, ataxia, and areflexia. Anti-GQ1b antibodies are useful markers for the differential diagnosis of Miller Fisher syndrome. We describe the case of a seven-year-old male who presented with a four-day history of diplopia and ophthalmoplegia following a febrile flu-like illness with sore throat. On examination he was found to have ataxia, areflexia and ophthalmoplegia, and a diagnosis of Miller Fisher syndrome was made after the exclusion of other conditions and concomitant with electrophysiological findings on electromyography. Although this disorder has a rare incidence, it should still be considered in the differential diagnosis in our country.     

Immunosuppressive therapy in congestive cardiomyopathy in childhood

Two patients aged ten and fourteen years presented with clinical signs of acute viral myocarditis and cardiac failure. Within three months both showed clinical deterioration with development of severe mitral and tricuspid incompetence. From their third month of illness myocardial antibodies were constantly found by indirect immunofluorescence technique. Both were treated with prednisone 2 mg/kg/d and azothioprine 2-2,5 mg/kg/d from their ninth and third month of illness for fifteen and twelve months respectively. Both showed considerable improvement within weeks with resolution of their valve incompetence, diminution of heart size and normalization of their ECGs. Results from myocardial biopsies suggest that a number of patients with chronic congestive cardiomyopathy may have long lasting viral inflammation of the myocardium or myocardial fiber destruction by autoimmunmechanisms after viral infection. Our cases probably had acute myocarditis which led to chronic congestive cardiomyopathy. Immunosuppressive therapy seems to be justified in cases where chronic inflammation or destruction of the myocardium is suspected because of the results of cardiac biopsies, myocardial scintigraphy or demonstration of myocardial antibodies.     

Neonatal enterovirus infections: emphasis on risk factors of severe and fatal infections

OBJECTIVES: Neonatal enterovirus infections have diverse manifestations, from asymptomatic to fatal. An understanding of the risk factors associated with severe cases might help to reduce enterovirus-related morbidity and mortality. METHODS: From July 1989 through June 1998, neonates with virus culture-confirmed nonpolio enterovirus infection at Chang Gung Children's Hospital were enrolled in the study and divided into three groups: nonspecific febrile illness; aseptic meningitis; and hepatic necrosis with coagulopathy (HNC). Demographic factors, clinical manifestations, laboratory data and outcome were analyzed to reveal factors associated with clinical severity and fatality. RESULTS: There were 146 cases including 43 neonates with nonspecific febrile illness, 61 with aseptic meningitis and 42 with HNC. By multiple logistic regression analysis, the most significant factors associated with HNC were prematurity, maternal history of illness, earlier age of onset (< or =7 days), higher white blood cell count (WBC > or =15 000/mm3) and lower hemoglobin (< or =10.7 g/dl). In 10 (24%) of 42 cases, HNC was fatal. In comparison with nonfatal cases of HNC, fatal cases had higher WBC, lower hemoglobin, higher bilirubin and higher incidence of concurrent myocarditis. Multivariate analysis showed the most significant factors associated with fatality from HNC to be total bilirubin >14.3 mg/dl (adjusted odds ratio, 29.1; 95% confidence interval, 2.5 to 355.5; P = 0.007) and concurrent myocarditis (adjusted odds ratio, 13.7; 95% confidence interval, 1.1 to 177.2; P = 0.04). Intravenous immunoglobulin did not correlate with clinical outcomes in cases with HNC. CONCLUSIONS: Prematurity, maternal history of illness, earlier age of onset, higher WBC and lower hemoglobin are significant factors associated with HNC; higher total bilirubin and concurrent myocarditis were most significantly associated with fatality from HNC.     

CD8 + T-LYMPHOCYTES INFILTRATE THE MYOCARDIUM IN FULMINANT HERPES VIRUS MYOCARDITIS

We report two cases of fulminant viral myocarditis in previously healthy children. They were caused by herpes simplex virus (HSV)-1 (in a boy aged 3 years) and Epstein-Barr virus (EBV) (in a boy aged 12 months). We obtained the diagnosis of HSV-1 myocarditis by immunohistochemistry and the diagnosis of EBV myocarditis by in situ hybridization. Histologic examination of heart tissue from the two boys revealed mononuclear cell infiltration of the myocardium. Immunohistochemical staining identified these cells as CD8 + T-lymphocytes. CD8 + T-lymphocytes induced by herpes virus infections may play an important role in the damage to heart muscle fibers seen in fulminant myocarditis in previously healthy children. To our knowledge, this is the first report of HSV-1 or EBV myocarditis (at least in children) in which viral infection has been demonstrated in the myocardium.     

CD8+ T-lymphocytes infiltrate the myocardium in fulminant herpes virus myocarditis

We report two cases of fulminant viral myocarditis in previously healthy children. They were caused by herpes simplex virus (HSV)-1 (in a boy aged 3 years) and Epstein-Barr virus (EBV) (in a boy aged 12 months). We obtained the diagnosis of HSV-1 myocarditis by immunohistochemistry and the diagnosis of EBV myocarditis by in situ hybridization. Histologic examination of heart tissue from the two boys revealed mononuclear cell infiltration of the myocardium. Immunohistochemical staining identified these cells as CD8+ T-lymphocytes. CD8+ T-lymphocytes induced by herpes virus infections may play an important role in the damage to heart muscle fibers seen in fulminant myocarditis in previously healthy children. To our knowledge, this is the first report of HSV-1 or EBV myocarditis (at least in children) in which viral infection has been demonstrated in the myocardium.     

Myocarditis and haemolytic uraemic syndrome.

A 13 year old girl is reported who presented with haemolytic uraemic syndrome (HUS) due to Escherichia coli O157:H7 infection. She died during the acute phase of the illness after an episode of unexplained sudden circulatory collapse. Postmortem examination confirmed the diagnosis of HUS and showed histological evidence of myocarditis manifested by the presence of inflammatory cell infiltration in the myocardium and around the conducting system.     

Fatal parvovirus B19 myocarditis in children and possible dysimmune mechanism

We report 2 cases of previously healthy children, who developed, after a common parvovirus B19 infection, a sudden inflammatory response, involving predominantly T cell, directed against myocardium and leading to fatal outcome. These cases and several published case reports further our understanding of fulminating parvovirus myocarditis in children.     

Juvenile Idiopathic Arthritis-Its Rare Presentation

The authors report a case of systemic onset juvenile idiopathic arthritis who presented with fever, excruciating neck pain and stiffness followed by respiratory difficulty after few days of onset of illness. The unusual features of the case were: cervical spondylitis without any peripheral joint involvement, myocarditis leading to congestive cardiac failure, though pericarditis being the common cardiovascular manifestation of SOJIA was also associated.     

Cardiac Involvement in Postnatal Rubella

ABSTRACT. Two cases of rubella myocarditis are reported: a 6-year-old boy who developed a complete, distal to His, atrioventricular block, during the third day of illness; and a 12-year-old boy who developed myocarditis with congestive heart failure 20 days following rubella infection. Although permanent pacing was required in the first patient, six years later he showed a normal growth and maintained normal activity. The second patient has deteriorated markedly and 6 months after the initial illness he had severe heart failure. It should be noted that myocarditis with abnormalities of the conduction system or congestive heart failure may be a complication to postnatal rubella.     

Cardiac involvement in postnatal rubella

Two cases of rubella myocarditis are reported: a 6-year-old boy who developed a complete, distal to His, atrioventricular block, during the third day of illness; and a 12-year-old boy who developed myocarditis with congestive heart failure 20 days following rubella infection. Although permanent pacing was required in the first patient, six years later he showed a normal growth and maintained normal activity. The second patient has deteriorated markedly and 6 months after the initial illness he had severe heart failure. It should be noted that myocarditis with abnormalities of the conduction system or congestive heart failure may be a complication to postnatal rubella.     

Complications of varicella zoster

Primary infection with varicella zoster is characterized by a generalized vesicular rash usually without significant systemic illness. Encephalitis, pneumonitis, pancreatitis, nephritis, Reye and Guillan-Barre syndrome transvers myelitis, myocarditis have been reported before, but there is not any case having all these system to be involved during the same infection in a sequential manner ending up with multiorgan failure. We wanted to represent 21-month-old boy had a multiorgan failure due to varicella zoster infection.     

Severe influenza B myocarditis and myositis.

OBJECTIVE: To report an influenza B infection with associated myocarditis and severe skeletal myositis. DESIGN: Case report. SETTING: Cardiac intensive care unit in a university-affiliated children's hospital. PATIENT: A 4-yr-old girl. RESULTS: The patient was successfully supported with extracorporeal membrane oxygenation for profound myocardial dysfunction and a combination of plasmapheresis and continuous venovenous hemodialysis for rhabdomyolysis and acute renal failure. CONCLUSIONS: This case provides a reminder that patients presenting with viral illness or myoglobinuria accompanied by renal failure, with or without associated myocarditis, may be demonstrating symptoms of influenza B.     

心肌炎的诊断与治疗

心肌炎是指心肌的局限性或弥漫性的炎性病变为主要表现的疾病.近年来随着对心肌炎动物模型实验研究及患者临床数据研究的不断深入,在心肌炎诊治方面出现了一些新进展.无创性心脏磁共振检查对心肌炎的的诊断有重大的价值,但不能替代心内膜心肌组织活检.心肌炎的治疗主要是对症辅助支持处理,主要为积极治疗休克、心力衰竭及心律失常等综合治疗,尤其是暴发性心肌炎患者的治疗.因目前缺乏大量的相关临床研究数据,对于心肌炎的诊治仍需进一步探索.     

Fatal Epstein-barr Virus Myocarditis in a Child with Repetitive Myocarditis

Fatal Epstein-Barr virus (EBV) myocarditis occurred in a 9-year-old female with a history of two prior discrete episodes of myocarditis, the first associated with chicken pox and the second of undetermined origin. Serologic studies during the fatal episode were characteristic of acute EBV infection, and EBV genome was detected by polymerase chain reaction (PCR) amplification of DNA extracted from cutopsy heart and liver. PCRs for enteroviruses and cardiac viral culture were negative. An intense mononuclear cell infiltrate in the myocardium consisted entirely of T cells, without identifiable B cells. Human leukocyte antigen HLA-DR analysis using frozen tissue obtained postmortem revealed antigens DR4 and DR13. DR4 is associated with some autoimmune disorders, as well as idiopathic dilated cardiomyopathy. We postulate that an aberrant immune response, possibly associated with the DR4 locus, was responsible for the repetitive episodes of myocarditis in this patient.     

Cardiac-Related Varicella Mortality in Childhood: A Literature Review With Clinical Experience

Among unvaccinated populations, primary varicella zoster virus (VZV) infection results in a minor childhood illness for the majority of individuals. Mortality is rare, and fatalities associated with cardiac complications are exceptional. In a population where routine VZV vaccination is not practised, we report a death in a previously healthy child secondary to VZV myocarditis. A literature review of cardiac-related VZV mortality in childhood is included. This identified a further 13 cases where death was associated with or attributable to, direct involvement of the myocardium or conducting system with what is frequently considered a benign childhood illness.     

Neonatal enterovirus myocarditis]

Enterovirus infections in neonates are difficult to diagnose. Diphasic pattern and possibly fatal myocarditis must be anticipated. CASE REPORT: A 14-day-old girl had presented a heart failure after an initial episode of gastroenteritis and supraventricular tachycardia. Investigation demonstrated global myocardial dysfunction. Diagnosis of neonatal enterovirus myocarditis was made by polymerase chain reaction detection of viral genome. Heart failure was controlled with medical treatment. CONCLUSION: Enterovirus myocarditis is typically a biphasic illness. Rapid diagnosis of enteroviral infection in neonatal period may be made by polymerase chain reaction detection of viral genome. There is anecdoctal evidence that immunoglobulin infusions may improve outcome.     

Pathogenesis of cardiovascular complications in children following scorpion envenoming

Scorpion envenoming is known to produce serious complications in children, and the complications differ with different species seen in the world. Peripheral failure, hypotension, myocarditis and seizures are the serious complications related to scorpion envenoming. The pathogenesis of cardiovascular complications caused by the Buthotus (Buthus) tamulus species is likely to be related to the direct effect of the venom on the myocardium rather than to catecholamine-induced hypoxia, as reported earlier. A scorpion sting is a common event both in urban and rural areas and the complications are more severe in children than in adults. Though several species have been identified throughout the world, stings by the Buthotus tamulus species are most commonly seen in southern states of India. Many complications have been reported related to the venom of different species of scorpion but the pathogenesis of cardiovascular complications seems to differ with different species. Most of the published reports suggest that hypotension and myocarditis were frequently observed following scorpion stings, whereas, in our children, peripheral failure and hypotension are the complications observed initially. Myocarditis and other serious complications are observed only during follow-up. The complications and lethal effects of scorpion venom seen in our earlier study indicated that in children cardiovascular complications were seen more frequently than neurological or haematological complications. The aim of the present study is to identify the frequency and nature of the cardiovascular complications and the pathogenesis of myocarditis following scorpion envenoming.     

Management of Reye's syndrome: need for early diagnosis and intravenous treatment of stage I non-comatose cases

Clinicians and nurses should obtain a history of antecedent illness occurring within 2 weeks of the onset of vomiting. Ninety percent of school-age children will give a history of an antecedent illness (varicella or influenza-like respiratory illness) within 1 week of the onset of vomiting. The vomiting of Reye's syndrome is usually persistent, lasting for 24 to 96 hours before the onset of serious brain signs. We believe that any child with the history of flu or chickenpox within 1 week of the onset of vomiting, which lasts for more than 12 hours, and is unusually severe or is associated with lethargy, should have an SGPT (alanine aminotransferase). This laboratory measure is clearly elevated in most cases of Reye's syndrome.     

自身免疫性心肌炎发病机制和临床意义的研究进展

自身免疫受遗传、免疫、激素和环境等因素影响,病毒感染可能会触发自身免疫.在各种类型心脏病的发病过程中自身免疫可能扮演一个重要角色,被定义为由心肌功能受损引起的心肌炎症反应,是导致扩张型心肌病最常见的病因.心肌炎可以由感染和非感染因素引起,非感染因素包括伴随心肌炎的自身免性疾病.为了更好地了解自身免疫性心肌炎,该文就自身免疫性心肌炎的发病机制和临床意义作一综述.     

Congestive Heart Failure: An Uncommon Presentation of Systemic Onset Juvenile Idiopathic Arthritis (SOJIA)

Children with systemic onset Juvenile idiopathic arthritis (SOJIA) are known to develop myocarditis and congestive heart failure as a complication of disease process infrequently. Cardiac involvement causing congestive heart failure as a presenting manifestation in SOJIA is rarely described in the literature. The authors describe a case of an 11- yr- old boy with SOJIA who presented with congestive heart failure following active myocarditis, with the flare of the disease process. The cardiac manifestations, along with the disease activity were controlled with intensive immunosuppressive therapy.