Resolution of pulmonary hypertension with low-molecular-weight heparin, steroid, and prostacyclin analogue therapy: could it be early-phase pulmonary veno-occlusive disease?

Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension. The authors present a case of neuroblastoma with progressive dyspnea, hypoxemia, and pulmonary hypertension. A computed tomography scan of the chest revealed septal thickening and diffuse micronodules. The patient was diagnosed as PVOD and was successfully treated with low-molecular-weight heparin (LMWH) and corticosteroid.

Abdominal tuberculosis in a child presenting with radiological evidence of pneumatosis intestinalis and portal venous gas.

Pneumatosis intestinalis, in association with portal venous gas, is a rare finding in children and young adults. In radiological studies, it is characterized by gas-filled cysts within the bowel-wall. It is often a sign of the serious significant underlying illness and is associated with a poor prognosis. A case of pneumatosis intestinalis and portal venous gas associated with abdominal tuberculosis in a child is presented here. Despite responding well to anti-tubercular treatment, he died suddenly at home, two months after discharge. It is recommended that cases with pneumatosis intestinalis should be carefully observed, although symptoms appear to be improving.

Comparison of safety and effectiveness of two different transfusion rates in children with severe anemia.

We compared the safety and efficacy of 2 transfusion regimens in children with severe anemia (hemoglobin <5 g/dL) and without overt signs of congestive heart failure requiring transfusion of packed red blood cells (PRBCs). Forty-three patients were randomly divided into 2 groups: group A and group B. The transfusion regimens consisted of continuous infusion of PRBC at a rate of 1 mL/kg/h for group A and 3 mL/kg/h for group B. The patients were closely monitored for any clinical signs of heart failure throughout transfusion. Heart and respiratory rate, and arterial blood pressure were measured hourly for 6 hours during transfusion. None of the patients developed any signs of cardiac failure during or after the transfusion. The mean heart rate, respiratory rate, diastolic and systolic blood pressure were similar in both groups throughout transfusion. Group A needed significantly more PRBC units than group B to attain the same hemoglobin increase. Transfusion of PRBC at a rate of 3 mL/kg/h, and at a rate of 1 mL/kg/h, is a safe regimen for children with severe anemia of gradual onset requiring transfusion therapy.

An unreported side effect of risperidone in children: sinus arrest with long pauses causing syncope.

Risperidone has been reported as a well-tolerated antipsychotic in children. The most common side effects of risperidone are anxiety, headaches, insomnia, and agitation, which frequently appear with high doses. Although it is known that risperidone affects the cardiac conduction system, symptomatic bradycardia has not been reported previously in children. We report a child who developed syncope because of sinus arrest with long pauses (>3 seconds) with therapeutic doses of risperidone. This case suggests that close monitoring is mandatory even during therapeutic treatment with risperidone.

A predisposing factor for spontaneous choledochal cyst perforation: esophageal dilatation procedures.

Cyst rupture is one of the rarest complications of choledochal cysts (CC). We report an 8-year-old boy with CC rupture leading to bile peritonitis following repeated esophageal dilatations for corrosive stricture, and discuss how the esophageal dilatation procedures might constitute a predisposing factor for CC perforation.