High dose pulsatile dexamethasone therapy in children with opsoclonus-myoclonus syndrome.

Opsoclonus-myoclonus syndrome (OMS) is a rare movement disorder characterized by chaotic eye movements, myoclonus, and ataxia associated with severe irritability. Different treatment modalities including steroids and cyclophosphamide have been tried in the past often with significant side effects and variable success. Here we present 11 children, diagnosed with OMS between 1999 and 2005 and treated with high dose dexamethasone pulses. Main symptoms at presentation were opsoclonus (11/11), ataxia and/or myoclonus (11/11), irritability (10/11) associated with a neuroblastoma in four children. Number of dexamethasone pulses ranged from 6 to 60 pulses. No major side effects were reported. In 6/11 children a complete and sustained remission of OMS symptoms was achieved after 6 to 29 pulses of dexamethasone. Two children from this group have a normal development and no neurological sequelae. Two further children have minor delays in fine- and gross-motor skills. Two children despite a complete recovery of OMS symptoms have persisting developmental problems. 5/11 children still require regular dexamethasone pulses in addition to daily prednisolone (n = 1) or have received cyclophosphamide pulses meanwhile (n = 2). All children continue to have developmental and neurological difficulties. In summary treatment with high dose pulsatile dexamethasone appears to be safe and beneficial in a subgroup of patients with OMS.

Ventricular flutter in a neonate--severe electrolyte imbalance caused by urinary tract infection in the presence of urinary tract malformation.

Male infants under the age of 3 months presenting with pyelonephritis in the presence of urinary tract malformation (UTM) are prone to transient pseudohypoaldosteronism. This may resemble congenital adrenal hyperplasia (CAH). Hyponatremia, hyperkalemia, dehydration, and metabolic acidosis are the primary findings that permit the diagnosis of CAH. We report a case of transient pseudohypoaldosteronism resulting from pyelonephritis and vesicouretric reflux. The 17-day-old boy presented with a salt-losing episode simulating adrenal insufficiency. An initial diagnosis of CAH was made. The severe metabolic imbalance resulted in ventricular flutter that resolved after correction of the metabolic acidosis and the electrolyte and volume depletion. Early diagnosis is essential because both conditions are potentially fatal and treatment differs significantly. Differential diagnosis may be achieved by urinalysis and abdominal ultrasound scan.

[Acute and long-term effects of captopril in resistance-induced pulmonary hypertension in childhood and adolescence]. / Akut- und Langzeiteffekte von Captopril bei widerstandsbedingter pulmonaler Hypertonie im Kindesund Jugendalter.

7 children and adolescents with pulmonary hypertension were examined with regard to the effect of Captopril as well on the pressure in the pulmonary artery and in the right ventricle as on the resistance in the systemic and pulmonary circulation and on the cardiac output. After initial application of Captopril 0.2 mg/kg body weight distinct oscillations of the data were measured, but with the exception of a small decrease of the mean pulmonary artery pressure (-8.5%) no determinable change was noticed. A decrease of the pulmonary artery resistance (-17%), the mean pulmonary artery pressure (-10%) and the right ventricular pressure (-3%) was found under continuous application of Captopril 0.3 mg/kg body weight daily for a period of 7 months. The cardiac output increased (+16%) and the systemic resistance decreased (-16%). The changes of data were not statistically significant. Captopril did not have a reliable therapeutic effect on the pressure and resistance in the pulmonary circulation in children and adolescents with pulmonary hypertension.