Dyssegmental dysplasia Silverman-Handmaker type in a consanguineous Druze Lebanese family: long term survival and documentation of the natural history.

We report on a male infant born with clinical and radiographic evidence of a lethal form of dyssegmental dysplasia not comparable to Silverman-Handmaker type, who had a prolonged survival of more than eight months. He had ocular and central nervous system abnormalities which have not been previously described. His course included significant feeding and respiratory difficulties, severe physical and psychomotor retardation, and recurrent fever of unknown etiology believed to be of central origin. The relatively long survival of this infant enabled us to focus on the natural history of this rare syndrome. The infant was born to first cousin parents of Druze Lebanese origin supporting an autosomal recessive mode of inheritance for the condition. This is the first documentation of dyssegmental dysplasia Silverman-Handmaker type in a family of Druze Lebanese ethnicity.

A prospective, multicenter, randomized study of high versus low positive end-expiratory pressure during extracorporeal membrane oxygenation.

To test the hypothesis that increased positive end-expiratory pressure (PEEP) could prevent deterioration of pulmonary function and lead to more rapid recovery of lung function, we randomly assigned 74 patients undergoing extracorporeal membrane oxygenation (ECMO) at four centers to receive either high (12 to 14 cm H2O) or low (3 to 5 cm H2O) PEEP. The two groups were similar in terms of weight, gestational age, diagnosis, and pre-ECMO course. All other aspects of care were identical. Dynamic lung compliance was measured at baseline and every 12 hours. Radiographs of the chest were obtained daily. Survival rates were similar in the two groups: 36 of 40 for low PEEP and 34 of 34 for high PEEP. The duration of ECMO therapy was 97.4 +/- 36.3 hours in the high-PEEP group and 131.8 +/- 54.5 hours in the low-PEEP group (p less than 0.01). Dynamic lung compliance throughout the first 72 hours of ECMO was significantly higher in patients receiving high PEEP. Radiographic appearance of the lungs correlated well with lung compliance: patients receiving high PEEP had significant deterioration of the radiographic score less frequently than those receiving low PEEP. High PEEP also was associated with significantly fewer complications. We conclude that PEEP of 12 to 14 cm H2O safely prevents deterioration of pulmonary function during ECMO and results in more rapid lung recovery than traditional lung management with low PEEP.

Intestinal lymphonodular hyperplasia of childhood: patterns of presentation.

In this retrospective analysis we searched for a constellation of signs or symptoms attributable to childhood lymphonodular hyperplasia (LNH). Of 147 children with documented LNH reviewed, 43% had lesions in the small bowel, and 57% in the large bowel. Children in this study presented with complaints of abdominal pain (58%) and bright red blood per rectum (32%). Physical examination revealed little except right lower quadrant (RLQ) abdominal tenderness and "fullness" in 35%. The pain was periumbilical, dull-cramping, rarely acute, and nonradiating. The hematochezia was most commonly streaky red in mucoid strands adhering to the stools, with no associated tenesmus. Three clinical patterns emerged: (a) Under 1 year of age most patients were male, with painless bleeding and pancolonic LNH. (b) Between 2 and 6 years, although the LNH was predominantly colonic, pain and bleeding occurred equally. (c) From 7 years old on, the main symptom was abdominal pain, but LNH distribution was nearly equal between the small bowel and the colon. To date, our long-term follow-up of the children with isolated LNH has revealed no sequelae.

Upper airway obstruction during sleep in children.

Cinefluoroscopic findings are described in nine children with sleep-related upper airway obstruction who are asymptomatic while awake. Asleep, these patients show strikingly similar changes in the region of the hypopharynx. During inspiration, the tongue and hypopharyngeal soft tissues are approximate, obliterating the hypopharyngeal air space causing intermittent and almost complete obstruction to air flow. The value of cinefluoroscopic studies on sleeping children with clinical symptoms of airway obstruction during sleep is emphasized.

On the subject of caring for and caring about patients.

Recognition of the growing familiarity on the part of physicians in approaching patients is presented with discussion of alternative approaches to establishing rapport and, at the same time, respecting the rights and preserving the dignity of the patient.

Osteomyelitis of the calcaneus secondary to heel pad puncture: a case report.

Heel pad puncture, a well-established procedure for obtaining hematologic samples from infants, may be complicated by acute osteomyelitis of the calcaneus. One such case is presented which involved a 3½-month-old female. Physicians are cautioned that calcaneal osteomyelitis should be suspected if soft tissue swelling and erythema are present around the heel area.