Nager syndrome is considered a rare genetic syndrome characterized by craniofacial and radial anomalies. Pierre Robin sequence is a triad that includes micrognathia, cleft palate and glossoptosis. The present patient had typical findings of Nager syndrome and Pierre Robin sequence. He progressed to severe respiratory distress, requiring mechanical ventilation and tracheostomy. At 1 year and 11 months, he had episodes of cardiorespiratory arrest and died. In the literature review, we identified the clinical description of 44 patients with Nager syndrome. Among them, 93.1% had micrognathia, 38.6% cleft palate and 11.3% glossoptosis. Only one (2.3%) had all three features, as observed in the present patient. Therefore, despite the fact that the features of Pierre Robin sequence are common, there are few patients who have the complete triad. It is noteworthy, however, that they may be associated with respiratory distress, which may put the patient's life at risk.

No abstract

No abstract

Objective To verify the prevalence of systemic arterial hypertension (SAH) and to identify possible early predictors of SAH at ages 2 and 4 years in very low birth weight (VLBW) infants. Study Design This is a prospective cohort study including inborn children with birth weight (BW) <1,500 g. Arterial blood pressure measurements were performed at 2 and 4 years. Model 1 compared children with and those without SAH at age 4. Model 2 compared children who had SAH at ages 2 and 4 with the others. SAH was diagnosed if the systolic or/and diastolic pressures were above the 95th percentile. Results A total of 198 patients were included during the 5-year study period, of whom 56% had SAH at age 4. In model 1, white matter injury (WMI) and catch-up growth at age 2 were predictors of SAH at age 4. In model 2, bronchopulmonary dysplasia, WMI, catch-up growth at age 2, and BW were predictors of SAH at 2 and 4 years. SAH at age 2 was an independent risk factor for SAH at age 4. After a multivariate analysis of model 2, BW and catch-up growth were associated with SAH. Conclusion Prevalence of SAH was high in VLBW infants; it was associated with low BW and catch-up growth at age 2.

No abstract