Right-sided pulmonary aplasia: longitudinal lung function studies in two cases and comparison to results from term healthy neonates.

Agenesis of the right lung was diagnosed prenatally in two neonates born at 36 and 37 weeks, respectively. Computed tomographic scans and magnetic resonance imaging indicated that both cases had a Type 2 pulmonary agenesis, which was confirmed later by bronchoscopy. Both patients were clinically stable during the neonatal period. Serial pulmonary function tests revealed a decrease in specific respiratory system compliance (sCrs) in both neonates and a marked discrepancy between functional residual capacity measured by the nitrogen washout technique (FRCN2) and by plethysmography (FRCpleth) on follow-up. Early decrease of respiratory system compliance (Crs) and increase of respiratory system resistance (Rrs) in one infant preceded the onset of tracheal stenosis, which remained asymptomatic until the age of 8 weeks, when the infant developed acute respiratory failure requiring intubation and mechanical ventilation with high airway pressures. Aortopexy, implantation of a tissue expander into the right hemithorax, and laser ablation of fibrotic tissue at the site of tracheal stenosis were performed to achieve successful extubation. The second infant remained asymptomatic. Values for lung mechanics and volumes for both infants with pulmonary aplasia were as follows: Crs, 3.43 and 10.60 mL x kP(-1) x kg(-1); sCrs, 0.23 and 1.28 kpa(-1); Rrs, 11.1 and 7.4 kpa x s x L(-1); FRCN2, 14.9 and 10.2 mL x kg(-1); FRCpleth, 28.2 and 25.8 mL x kg(-1); FRCN2: FRCpleth ratio, 0.56 and 0.54 for patients 1 and 2, respectively. These values differed considerably from results of a control group of nine term healthy neonates (Crs, 10.0+/-1.8 mL x kPa(-1) x kg(-1); sCrs, 0.43+/-0.08 kpa(-1); Rrs, 5.10+/-0.55 kpa x s x L(-1); FRCN2, 24.0+/-2.5 mL x kg(-1); FRCpleth, 31.1+/-6.0 mL x kg(-1); FRCN2:FRCpleth ratio, 0.78+/-0.10). In conclusion, serial assessment of lung mechanics and pulmonary gas volumes detects airway obstruction early in neonates with unilateral lung agenesis. Bronchoscopy is recommended. Along with conventional surgical procedures, an expandable implant may improve management or prevent respiratory failure in selected cases.

Neuroectodermal cyst may be a rare differential diagnosis of fetal sacrococcygeal teratoma: first case report of a prenatally observed neuroectodermal cyst.

In spite of the fact that most anomalies can be accurately diagnosed prenatally, one may be confronted with an extremely rare fetal abnormality that has never been described before. We report a case of a neuroectodermal cyst arising from the posterior neuropore. After 21 weeks of gestation a fetal anechoic intra- and extra-abdominal tumor was detected. The mass grew to 75 x 41 x 33 mm at 35 weeks. Initially it was believed to be a sacrococcygeal teratoma. Serial scans and Doppler ultrasound examinations were performed, which demonstrated fetal well-being. Color Doppler imaging failed to demonstrate increased tumoral perfusion. After elective Cesarean section, the tumor was excised. The postoperative course was complicated by recurrent infections of the urinary tract due to neurological damage to the bladder. There was also impaired function of the anal sphincter. The histological finding of a monolayer of neuroepithelial cells and melanocytes led to the diagnosis of a neuroectodermal cyst.

[Candida parapsilosis infection as a complication of long-term parenteral nutrition]. / Candida parapsilosis-Sepsis als Komplikation der langfristigen parenteralen Ernährung.

In case of short bowel syndrome in infancy the total parenteral nutrition was complicated by catheter-associated Candida parapsilosis septicemia based on generalized enterococci infection. Specific problems in antifungal treatment are discussed.

[Enterococci as pathogens of severe infections in newborn infants and young infants]. / Enterokokken als Erreger schwerer Infektionen bei Neugeborenen und jungen Säuglingen.

We report about severe perinatal infections caused by enterococci during a 5 year-period. Between January 1983 and December 1987 8 infants were treated in the Department of Neonatology of the Charité-Hospital, 5 of them died. In 4 of the infants this infection was of the early onset type and in 4 infants the infection had a late onset. The clinical course of the four infants with early onset type was very similar to that of infants with group-B-streptococcus disease. The increase of severe infections caused by enterococci in 1987 (7 cases) is unclear. One cause of the increase could be, that the chemotherapy of septicemia and meningitis in the Department of Neonatology of the Charité is the combination cefotiam and gentamicin or cefotaxime and gentamicin. Enterococci are resistant to all cephalosporins and a selection of these pathogens after this therapy is possible. In vitro and clinical experience has demonstrated, that a ampicillin-gentamicin combination constitutes effective therapy for enterococcal infection.

Ambroxol for prevention and treatment of hyaline membrane disease.

To estimate the efficacy of ambroxol for clinical use in prenatal prevention and postnatal therapy of hyaline membrane disease (HMD) all available experimental and clinical data were reviewed. The administration of ambroxol in animals has a certain promoting influence on lung maturation, a high specificity to lung tissue and a favourable relationship between intended action and negative adverse effects. In clinical studies concerning the prevention of HMD, ambroxol increases the values of amniotic fluid parameters used for estimation of lung maturity and reduces the incidence of HMD at least as effectively as corticosteroids. The number of infants at less than 33 gestational weeks in these reports is small, and further studies will have to confirm the results. Ambroxol applied postnatally has beneficial effects on the course of severe HMD by increasing survival rate, by decreasing duration of oxygen need and artificial ventilation and by improving compliance. Pharmacological studies in preterm HMD-infants showed no influence of ambroxol on blood pressure and heart rate. In 3 of 8 newborns a transient increase of transcutaneous oxygen tension was seen during infusion of ambroxol. Ambroxol is quickly bound to tissue receptors which release it continuously indicating that repeated applications are as effective as continuous infusion.

Neonatal septicaemia--incidence, etiology and outcome. A 6-year analysis.

Between 1983 and 1988 we observed altogether 222 cases of neonatal septicemia and/or meningitis in our Department of Neonatology. The incidence was 8.46 per 1,000 liveborn infants. The case fatality rate amounted to 45.9%. The most frequently isolated causative agents were Escherichia coli (23.4%) followed by group B Streptococci (16.7%), Staphylococcus aureus (9.9%), Klebsiella pneumoniae species (8.8%), Serratia marcescens (7.9%), Pseudomonas aeruginosa and coagulase-negative Staphylococci each 5.9%. The report includes information about serotypes of Escherichia coli, group B Streptococci and plasmid patterns of Serratia marcescens. The latter was responsible for an outbreak of septicemia and meningitis with high mortality. The changing infection pattern reflects changes in the newborn population, especially in the patient structure of the neonatal intensive care unit, changes in the antibiotic policy and organizational problems.