Prune-belly syndrome: therapeutic options including in utero placement of a vesicoamniotic shunt.

The prune-belly syndrome (PBS) consists of abdominal wall distention with deficiency of the abdominal wall musculature, urinary tract abnormalities, and cryptorchidism. The impaired drainage of the bladder leads to oligohydramnios and pulmonary hypoplasia. We present 4 cases of PBS diagnosed by prenatal sonography. In 2 cases, vesicoamniotic shunt therapy was not indicated because of a poor prognosis based on sonographic and laboratory findings; the pregnancies were terminated. In another case, treatment was not performed because of a twin pregnancy, and the neonate with PBS died the day of delivery by cesarean section at 31 weeks' menstrual age. In the other case, vesicoamniotic shunt therapy was successfully performed, and a healthy child was delivered. Several conditions must be met for vesicoamniotic shunt therapy to have a good chance of success: the karyotype must be normal, other malformations must be excluded by careful sonographic examination, and renal function must be normal, as determined by serial analyses of fetal urine. Generally, the shunt should be inserted as early as possible.

[Prevention of neonatal anemia with recombinant human erythropoietin: a cost-benefit analysis]. / Die Prophylaxe der Frühgeborenenanämie mit rekombinantem humanen Erythropoietin: eine Kosten-Nutzen-Analyse.

A few years ago recombinant human erythropoietin (rh-EPO) has been introduced for the prophylaxis of anaemia of prematurity. Aim of this controlled study was a cost-effectiveness analysis of the prophylaxis with rh-EPO versus sole transfusion with packed red blood cells. In the study group 33 infants (gestational age 30 +/- 2 weeks, birthweight 1217 g +/- 244 g) were treated with rh-EPO beginning on the fifth day of life for a six week period. They received 750 IE rh-EPO/kg/week and transfusion with packed red blood cells when indicated. In the historic control group 33 infants (gestational age 29.2 +/- 1.9 weeks, birthweight 1181 g +/- 205 g) did not receive rh-EPO, patients were only transfused. Indication and guidelines for transfusion were identical for both groups. The number of transfusions was registered after 2 and 4 weeks of life and by the time of hospital discharge. The cost analysis was carried out by using current prices for packed red blood cells including material and processing and prices for rh-EPO (Neo-Recormon, Boehringer Mannheim). Infants in the study group received 1.39 +/- 1.94 transfusions per patient while patients in the control group needed 2.7 +/- 1.93 transfusions per patient (p < 0.05). Cost for treatment was slightly increased in the study group (DM 536,- vs. DM 459,-). Prophylaxis of anaemia of prematurity with recombinant human erythropoietin proved to be effective. Compared with sole blood transfusion treatment, expenses for the prophylaxis with rh-EPO were only little higher.

[Combination therapy of high frequency oscillatory ventilation, NO inhalation and surfactant replacement in a child with acute respiratory distress syndrome]. / Kombinierter Einsatz von Hochfrequenzoszillationsbeatmung, NO-Applikation und Surfactantsubstitution beim kindlichen ARDS.

We report about a child with severe ARDS after burning trauma who did not respond to conventional treatment with controlled pressure ventilation under conditions of permissive hypercapnia and changing of the infants's body position. A combined treatment with high frequency oscillatory ventilation, inhalation of nitric oxide and surfactant replacement improved the pulmonary status. Twelve days after the accident the boy could be extubated and 5 weeks later he could be discharged without any pulmonary and neurologic handicap. The use of these therapeutic tools may help to avoid the necessity of the invasive extracorporeal life support.

Pharmacokinetics of oral fluconazole in premature infants.

UNLABELLED: Systemic infections with Candida albicans in neonates are a frequent and well recognized problem. The therapeutic gold standard in this situation is the combined intravenous antimycotic treatment with amphotericin B and flucytosine. Potential adverse effects of this regimen have encouraged the search for desirable alternatives. We report on the use of oral fluconazole in neonates with Candida albicans septicaemia. Three premature infants were treated with four courses of therapy. Pharmacokinetic studies were performed during each course. At oral doses of 4.5-6 mg/kg once a day, serum levels of fluconazole were within the therapeutic range during the entire dosage interval. Follow up showed microbiological and clinical cure in all patients with no side-effects. In one patient a dosage of 4 mg/kg per day lead to a microbiological relapse with sub-therapeutic serum levels. CONCLUSIONS: Oral fluconazole seems to be a safe and effective treatment for Candida albicans septicaemia even in premature infants.