Treatment of childhood lymphangiomas with interferon-alpha.

PURPOSE: Nonsurgical treatment of lymphangiomas has shown limited efficacy and often carries unacceptable toxicities, demonstrating the need for a more effective, less toxic therapy. PATIENTS AND METHODS: We describe two patients with lymphangiomatosis treated for 12 to 40 months with recombinant interferon-alpha. RESULTS: Both patients demonstrated stabilization or marked improvement of disease, based on clinical and radiologic findings, with minimal toxicity. CONCLUSIONS: The favorable responses to interferon-alpha therapy in these two cases suggest that this is an effective and well-tolerated treatment for lymphangiomas in children.

Medical examiners' attitudes toward organ procurement from child abuse/homicide victims.

Solid organ transplant provides lifesaving therapy for infants and children with otherwise terminal diseases, but it is severely limited by donor organ supply. Medical examiners perform a pivotal role in the organ procurement process by determining whether a "heartbeating cadaver" on life support is a medicolegally suitable donor. This descriptive questionnaire study assesses medical examiner practice and behavior regarding organ procurement from child abuse/homicide victims. Obtaining forensic evidence for judicial purposes and releasing organs to children awaiting transplantation are not necessarily conflicting values. Greater than 60% of medical examiners sampled would agree to release organs from abuse/homicide victims in the scenarios presented here if provided with requested information. Further confronting the origins of variable medical examiner practice in this area might result in the availability of additional solid organs for pediatric transplantation.

Chronic respiratory failure of infancy and childhood: clinical outcomes based on underlying etiology.

To assess whether underlying diagnosis affects morbidity and mortality outcomes in patients with chronic respiratory failure, we studied 55 patients with chronic respiratory failure of infancy and childhood (CRFIC). Entry criteria included patients with chronic respiratory failure due to static neurologic or neuromuscular conditions or secondary to other disease processes considered likely to improve or resolve over time. Subjects were grouped into those having chronic lung disease (CLD, n = 22), neurologic or neuromuscular diseases (NM, n = 21), or congenital abnormalities affecting the respiratory system (CA, n = 12). The average duration of follow-up was 21.3 months. There were no differences between groups in mortality with only four deaths (7%). Patients with CLD fared better than those with NM or CA in duration of ventilatory support, duration of tracheostomy, percentage of successful weaning from mechanical ventilation, and neurodevelopmental outcomes. Subjects with CLD had a significantly greater frequency of tracheomalacia (86%), feeding disorders (86%), and hypogammaglobulinemia G (77%). There were no differences between groups for respiratory readmissions or family dysfunction. We conclude that almost all patients with CRFIC will survive, but morbidity outcomes will vary based on the underlying diagnosis.

Diaphragmatic excursion after pleural sclerosis.

Chemical sclerosis of the pleural space is used to prevent recurrence of spontaneous pneumothorax. To test whether sclerosis restricts diaphragmatic excursion, we measured diaphragmatic excursion by ultrasonography in subjects with unilateral pleural sclerosis and compared it with diaphragmatic excursions in normal subjects, in subjects with cystic fibrosis (a diffuse bilateral lung disease), and in those who underwent surgical procedures that obliterate the pleural space. In five subjects with unilateral chemical sclerosis, diaphragmatic excursion was significantly less on the sclerosed side than on the contralateral side (10.7 +/- 1.3 vs 17.3 +/- 1.7 mm, mean +/- SEM; p less than .01). Compared with those of normal subjects, the side-to-side differences in excursion were increased by pulmonary disease (p less than .03) and additionally by unilateral sclerosis (p less than .015). There was no significant difference between diaphragmatic excursions on left and right sides of subjects without history of pleural disease. These data suggest that chemical pleural sclerosis causes a measurable reduction in diaphragmatic excursion on the affected side. The physiologic significance of this effect is not known.

Treatment of respiratory viral infection in an immunodeficient infant with ribavirin aerosol.

An infant with severe combined immunodeficiency syndrome (SCIDS) secondary to adenosine deaminase deficiency had pneumonitis and combined infection with respiratory syncytial virus (RSV) and parainfluenza virus type 3 (PIV3). Four separate courses of ribavirin were delivered by small-particle aerosol. The PIV3 disappeared during the first course, and RSV disappeared after the fourth course on the 58th hospital day. Neither virus returned during profound immunosuppression for bone marrow transplantation. Secretory antibody to both viruses was found and may have assisted in recovery. Strains of RSV from the 9th, 15th, 29th, and 55th hospital days showed similar sensitivities to ribavirin in vitro. Ribavirin can be a useful drug in the treatment of respiratory viral infections in patients with SCIDS.