Jieh-Neng Wang

Complete atrioventricular block following myocarditis in children.

AbstractComplete atrioventricular block (CAVB) can be either congenital or acquired in children. Acquired CAVB is occasionally seen in myocarditis patients. To determine the etiology, natural history, and outcome of children with acquired nonsurgical CAVB, we retrospectively reviewed nine children who had suffered CAVB caused by suspected infectious myocarditis. All of them had CAVB with a wide QRS escape ventricular rhythm on admission. Three of them had ventricular tachycardia in addition to CAVB. Seven of them had a preceding upper respiratory tract infection. All of them had congestive heart failure. Five of them had Stokes–Adams seizures. Three etiologies were identified in four of the children. All patients received inotropic agents and emergency temporary pacing. In all except one case, the cardiac rhythm returned to sinus rhythm within 10 days. During a follow-up period of 9 to 96 months, all were asymptomatic and drug-free. Electrocardiograms showed that four patients were completely normal, there was complete RBBB in four and left anterior fascicular block in one patient. We conclude that although CAVB associated with myocarditis can be life-threatening, the long-term prognosis is good if patients are diagnosed early and proper management is employed.n

Pulmonary Cavernous Hemangiomatosis Treated with Interferon Alfa-2a

Pulmonary hemangiomatosis is a rare, usually fatal disorder characterized by diffuse proliferation of blood vessels within the thorax. We describe a 7-year-old boy with cavernous-type pulmonary hemangiomatosis successfully treated with interferon alfa-2a. He presented with respiratory distress and hemoptysis that were alleviated during a 2-year follow-up period.

Mycoplasma pneumoniae infection associated with Kawasaki disease

Chelation with dimercaprosuccinic acid (DMSA) (30 mg/kg daily) increased urinary excretion in all cases. Treatment was pursued 5 d to 2 mo until recovery appeared to be complete. Three of the patients benex8e ted from antihypertensive treatment with nicardipine. Environmental inquiry showed that the source of mercury was a broken medical thermometer in the case of one family and small quantities of the metal brought from the workplace by a family member in the other case. Eradication of the source was obtained in all cases. The purpose of this report is to demonstrate that acrodynia can mimic the clinical and biochemical manifestations of pheochromocytoma and should be considered and excluded during the investigation of suspected cases. Biologically, the increase in urinary catecholamines frequently occurs, as our four cases attest. This usually concerns norepinephrine and epinephrine. Cheek et al. showed that inorganic mercury by injection can markedly increase sympathetic activity and epinephrine levels in rats, and would therefore be expected to mimic the signs and symptoms of pheochromocytoma (4). In inorganic mercury poisoning, the metal combines with the sulphydryl group of Sadenosylmethionine , which acts as a co-factor for catecholamine-o-methyltransferase (COMT). COMT inhibition leads to accumulation of catecholamines (2). This accumulation of epinephrine and norepinephrine could explain the haemodynamic symptoms of acrodynia (4). Although acrodynia is rare, its diagnosis should be considered in any child with signs of behavioural change, skin rash and hypertension by a single urine analysis (1, 2). There exists no correlation between the level of mercury and the clinical severity or even the evolution of the disease. We believe that mercury poisoning should be considered initially in any child with signs of sweating, behavioural change, skin rash, hypertension and that pheochromocytoma might be considered in the differential diagnosis. Recognition of the variable manifestations of the disease and prevention of further exposure are the most important aspects of management.

Laryngeal Candidiasis in Children

Candidiasis of the larynx is rare and often related to immunocompromised hosts. We here report a case of laryngeal candidiasis in an immunocompetent infant. The diagnosis was obtained by direct fibre-optic laryngoscopy with specimens submitted for culture. He received anti-fungal medication and was quite well at 1-year follow up. The pertinent literature is also reviewed.

Once-daily vs. twice-daily intrapleural urokinase treatment of complicated parapneumonic effusion in paediatric patients : a randomised, prospective study

To evaluate the effective dose frequency (once daily vs. twice daily) of intrapleural urokinase treatment in children who required tube thoracostomy for drainage of a complicated parapneumonic effusion, we designed a randomised prospective study in a tertiary medical centre in Taiwan. From June 2002 to January 2005, 30 paediatric patients with complicated parapneumonic effusion who had received chest tube drainage were randomised 1u2003:u20031 to the once‐daily (urokinase 5000–6000u2003IU/kg/dose) or twice‐daily (urokinase 2500–3000u2003IU/kg/dose) treatment. We compared clinical manifestations and outcomes in both groups. There were no differences in pleural effusion characteristics between the groups. Six patients had Streptococcus pneumoniae, one had Staphylococcus aureus, one had Group AStreptococcus, and 22 had unknown pathogens. There were no significant differences between the once‐ vs. twice‐daily group in the amount of drained pleural fluid (564.9u2003±u2003422.1u2003ml vs. 560.5u2003±u2003198.6u2003ml, respectively), fever duration after chest tube insertion (4.3u2003±u20033.2u2003days vs. 5.3u2003±u20032.7u2003days), or total admission days (14.3u2003±u20033.9u2003days vs. 14.6u2003±u20033.0u2003days) (pu2003>u20030.05 for all). Only two patients (one in each group) required the surgery. Thus, we found that both once‐ and twice‐daily administration of urokinase were similarly efficacious, and resulted in good clinical outcomes. Both obviated the need for surgery in most (93%) cases of pneumonia with complicated parapneumonic effusion in this series. A larger, multicentre study is necessary to verify our findings.

Long QT syndrome in children.

Long QT syndrome is a disease that can cause syncope, seizures and sudden death. From June 1990 to June 1996, 11 children (male/female: 714; ages: 1 day-13 years with a median of 5.4 years) from different families were found to have long QT syndrome. Their corrected QT intervals (QTc) were 0.46-0.59 sec (median: 0.53 sec). All patients had normal hearing. One patient had Marfan syndrome with mitral valve prolapse. The presentation symptoms were: sudden death (2), seizures (6) and syncope (3). Their electrocardiogram abnormalities included: torsades de pointes (7), sinus bradycardia (4), T wave abnormalities (4), monomorphic ventricular tachycardia (2) and congenital complete atrioventricular block (1). All patients were treated with beta-blockers and one had pacemaker implantation. In a follow-up period of 0.5-6 years, 6 were symptom free, 1 died of ventricular tachycardia and 2 had recurrent syncope.

Myocarditis with complete atrioventricular block associated with herpes simplex virus infection: report of one case.

Myocarditis with complete atrioventricular block is a very unusual complication of the herpex simplex infection. We report a 10-year-old boy infected very likely by the herpes simplex virus and who presented with high fever, erythema multiforme, complete atrioventricular block, and Adams-Stokes seizures. Emergent temporary pacemaker was performed for bradycardia. A sixteen-fold rise in herpes simplex antibody titer by a complement fixation method occurred within two weeks. Normal cardiac rhythm recovered 11 days later with a sequela of complete right bundle branch block after 2 years follow-up.

Suprasellar embryonal carcinoma : Report of one case

Intracranial embryonal carcinoma is a rare germ cell tumor found predominantly in the pineal region and, to a lesser extent, in the suprasellar region. The case of a 12-year-old female with a history of secondary amenorrhea for 6 months is reported; her symptoms included decreased visual acuity, dizziness and postprandial vomiting over a 1-month period. A huge suprasellar mass was found by computed tomography. Serum alpha-fetoprotein (AFP) and beta-human chorionic gonadotropin (beta-HCG) levels were elevated. The tumor was subtotally resected; pathologic and immunocytochemical findings were compatible with embryonal carcinoma. The patient died three weeks after operation. The case is described and pertinent literature is reviewed.

PTPN11 Mutations in LEOPARD Syndrome: Report of Four Cases in Taiwan

BACKGROUND/PURPOSEnLEOPARD syndrome (LS) is a rare, autosomal dominant disorder. The typical clinical presentation includes multiple lentigines and cardiac defects. Mutation analysis of the PTPN11 gene is feasible. We report four cases of LS, which were confirmed by molecular genetic study.nnnMETHODSnThe clinical features and mutations of the four patients were summarized.nnnRESULTSnThe diagnosis of all four patients was made when lentigines appeared during childhood. Three cases had hypertrophic cardiomyopathy. No electrocardiographic conduction abnormality was noted in any of the cases. Three patients had hypertelorism and three had short stature. Two patients, identical twins, presented with the atypical phenotype of tongue protrusion and hepatosplenomegaly at birth. Twin B had mild mental retardation. Case 4 had moderate hearing impairment. Point mutation of the PTPN11 gene was found in all patients.nnnCONCLUSIONnLS has typical skin manifestations. All patients should undergo a comprehensive examination, especially echocardiography and electrocardiography. The diagnosis can be confirmed by genetic study.

Risk Factors of Total Anomalous Pulmonary Venous Connection Surgery

Background: Total anomalous pulmonary venous connection is a rare congenital heart disease that needs to be surgically corrected. In our study, we retrospectively evaluated the factors associated with mortality and morbidity in patients undergoing total anomalous pulmonary venous connection surgery. nPatients and methods: Retrospective data were collected on 34 patients with isolated total anomalous pulmonary venous connection seen from 1990 to 2015. The median age at time of repair was 36 days (range, 1 to 2520 days) with median weight of 3.465 kg (range, 1.4 to 20.7 kg). Pulmonary venous obstruction, reintervention, associated factors for mortality and overall mortality were recorded and analyzed. nResults: There were 16 deaths (13 surgical deaths and 3 late deaths due to pulmonary venous restenosis and reintervention). Preoperative pulmonary venous obstruction was presented in 21 patients (21/34, 61.8%) and was found to be associated with overall mortality (p=0.0052). Postoperative pulmonary venous restenosis was also a significant risk factor for mortality (p=0.0421). Emergent surgery is a risk factor for mortality (p=0.004). The associated risk factors, such as pre-operation ventilator FiO2, pre-operation inotropic use, postoperation ventilator FiO2 and post-operation epinephrine use were also associated with patient mortality. The initial SpO2 when arriving at our hospital was a protective factor for mortality. nConclusion: The overall mortality of total anomalous pulmonary venous connection patients has improved in recent years. However, preoperative pulmonary venous obstruction was still an important risk factor for mortality. Postoperative pulmonary venous restenosis was also a risk factor for mortality. Heart failure and emergent surgery were associated with higher mortality rates.