We conducted a monthly epidemiological survey to determine the birth prevalence of Robin sequence (RS) and the use of various therapeutic approaches for it.

OBJECTIVE AND DESIGN: To determine the incidence of and possible risk factors for unexpected sudden infant deaths (SID) and severe apparent life-threatening events (S-ALTE) that occurred within 24 hours of birth. This was a monthly epidemiologic survey. PATIENTS AND METHODS: Throughout 2009, every pediatric department in Germany was asked to report such cases of unexplained SID or S-ALTE in term infants after a good postnatal adaptation (10-minute Apgar score ≥ 8) to the Surveillance Unit for Rare Pediatric Conditions in Germany. The latter has a capture rate of > 95%. S-ALTE was defined as acute cyanosis/pallor and unconsciousness, requiring bagging, intubation and/or cardiac compressions. Hospitals that reported a case were asked to return an anonymized questionnaire and discharge letter as well as the autopsy protocol in SID cases. RESULTS: Of 43 cases reported, 17 fulfilled entry criteria, yielding an incidence of 2.6 in 100 000 live births. There were 7 deaths (ie, 1.1/100 000); 6 of the 10 S-ALTE infants were neurologically abnormal at discharge. Twelve infants were found lying on their mother's chest or abdomen, or very close to and facing her. Nine events occurred in the first 2 hours after birth; 7, were only noticed by a health professional despite the mother being present and awake. CONCLUSIONS: SID or S-ALTE may occur in the first 24 hours after birth, particularly within the first 2 hours. Events seem often related to a potentially asphyxiating position. Parents may be too fatigued or otherwise not able to assess their infant's condition correctly. Closer observation during these earliest hours seems warranted.

Objective: To explore whether and how population based data from a regional quality control programme can be used to investigate the hypothesis that small for gestational age (SGA) very low birthweight infants (VLBW, 90th centile, severe malformations, siblings of multiple births, and gestational age (GA) 29 weeks. Outcomes of interest were death, severe IVH, PVL, and RDS. Multivariable analyses were performed by Cox proportional hazard and logistic regression models. Results: Within the data validation procedure, an increase in proportions of both VLBW (from 0.95% in 1991 to 1.11% in 1996; +17%) and SGA (from 22.7% to 27.4%; +21%) infants became apparent (p<0.05). The study population consisted of 1623 infants (173 SGA). Mortality was 12.1% (n = 196), with an adjusted hazard ratio for SGA infants of 2.54, 95% confidence interval (CI) 1.70 to 3.79. Both groups were at similar risk of severe IVH (adjusted odds ratio 0.93, 95% CI 0.5 to 1.65) and PVL (1.54, 95% CI 0.78 to 2.87), but SGA infants had less RDS (0.57, 95% CI 0.35 to 0.93). Male sex, multiple birth, hypothermia (<35.5°C), and sepsis were associated with IVH and RDS. Infants admitted to hospitals with <36 VLBW admissions/year had increased mortality (adjusted hazard ratio 1.56, 95% CI 1.12 to 2.18). Conclusions: SGA VLBW infants are at increased risk of death, but not of IVH and PVL, and at decreased risk of RDS. That mortality is higher in smaller hospitals needs further investigation.