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Two female sibling full-term newborns developed respiratory distress shortly after birth, which progressed to respiratory failure. Tracheal lavage demonstrated presence of surfactant protein A (SP-A), but little surfactant protein B (SP-B), without aberrant surfactant protein C (SP-C). On a lung biopsy performed in both infants, prominent type II pneumocyte hyperplasia was evident. Through ultrastructural examination an absence of normally formed lamellar bodies was determined, with numerous irregular electron dense bodies within the type II pneumocytes. These electron dense bodies could also be identified in the alveolar spaces and alveolar macrophages. No alveolar tubular myelin was present. Abnormally high immunoreactivity for surfactant proteins SP-A, proSP-B, SP-B, and proSP-C was demonstrated by light microscopy. Presence of incompletely processed immunopositive proSP-B, but not proSP-C was observed in the alveolar lumina. No mutations in either the SP-B or SP-C gene were identified by sequence analysis of amplified cDNA. We conclude that these siblings exhibit an inherited surfactant deficiency characterized by abnormal accumulations of surfactant proteins within the pneumocytes. This abnormal accumulation may be due to a primary secretory defect, a defect in surfactant phospholipids, or an abnormal interaction between the phospholipids and surfactant proteins.

The purposes of this study were to characterize the changes in renal sympathetic nerve activity (RSNA) and baroreflex function at birth in conscious sheep. One hour after delivery by cesarean section, RSNA increased by 239 +/- 24% compared with fetuses. The upper and lower plateau values of the baroreflex function curves for RSNA, expressed as the percent maximum achieved in the fetus, were greater (P < 0.05) at 1 h (260 +/- 41 and 142 +/- 40%) and 5 h of age (254 +/- 34 and 100 +/- 19%) than in fetuses (100 and 10 +/- 3%), respectively. Curve midpoint pressures also were higher (P < 0.05) at 1 h (62 +/- 3 mmHg) and 5 h (66 +/- 4 mmHg) than in fetuses (51 +/- 2 mmHg). No changes in the sensitivity (gain) of the RSNA response to baroreceptor stimulation were seen. The baroreflex response curves for heart rate showed similar increases in the curve midpoint pressures, while gain did not change. To determine whether the high circulating levels of angiotensin II (ANG II) in the newborn period contribute to the rise in RSNA or the resetting of the baroreflex toward higher pressures, the angiotensin-converting-enzyme inhibitor, enalaprilat, was administered to five lambs before delivery. No differences in the increase in RSNA after birth were detected between control and treated animals. The curve midpoint pressures of both the RSNA and heart rate baroreflex response curves were significantly less (P < 0.05) in the treated compared with control lambs.(ABSTRACT TRUNCATED AT 250 WORDS)

The present studies were designed to assess the contribution of onset of respiration, separation from the placenta, and a decrease in environmental temperature on the increase in renal sympathetic nerve activity (RSNA) that occurs at birth. In the first series of experiments, heart rate (HR), mean arterial blood pressure (MABP), and RSNA were recorded in chronically instrumented near-term fetal sheep (n = 12) before and during in utero ventilation (V), V + oxygenation (V + O), and V + O + umbilical cord occlusion (V + O + CO). RSNA increased by 49 +/- 16% during V alone (P < 0.05), whereas no additional changes were seen with V + O or V + O + CO. HR and MABP did not change with any intervention. In a second series of experiments (n = 10), changes in fetal HR, MABP, and RSNA in response to in utero cooling were recorded. Cooling of the fetal core temperature by -3.1 +/- 0.2 degree C produced a rapid and sustained increase in RSNA (330 +/- 155%), HR (25 +/- 11%), and MABP (10 +/- 2%) consistent with generalized sympathoexcitation. In a third series of studies (n = 3), we found that brain stem transection between the rostral pons and posterior hypothalamus abolishes the increases in RSNA seen at birth. These results suggest that cooling is a major contributor to the postnatal rise in RSNA and that brain centers at the level of or above the hypothalamus are involved in mediating sympathoexcitation at birth.

Amelia is a very rare form of limb reduction defect. The incidence of isolated amelia with or without other limb reductions is 0.4 per 100,000 births. We report a cluster of three cases diagnosed prenatally. One was isolated tri-amelia and two were isolated tetra-amelia.

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