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We tested the hypothesis that female carriers of ornithine transcarbamylase (OTC) deficiency have cerebral dysfunction as a consequence of episodic hyperammonemia. Seven such carriers were identified by pedigree analysis and protein-tolerance testing. The Wechsler Adult Intelligence Scale was used for evaluation. All IG scores were above 100, but in the protein-tolerant siblings, the full-scale and performance IQ scores were higher by 5.6 and 9.9 points, respectively (P less than 0.02). The absolute difference between the verbal and performance IQ scores was greater in the OTC carriers by 7.8 points (P less than 0.01). This study suggests that clinically asymptomatic carriers of OTC deficiency may have intellectual deficits, perhaps as a result of episodic hyperammonemia.

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SummaryNitrogen washout measurements and blood-gas analyses were made on 32 newborn infants with severe RDS at continuous positive airway pressures (CPAP) of 5, 10, and 15 cm H2O. Increases in airway pressure resulted in significant increases in Pa02 and functional residual capacity (FRC). It also produced significant decreases in alveolar turnover rates of the "fast" and "slow" alveolar spaces of a two-space lung model. Changes in CPAP did not significantly affect the distribution of ventilation.The changes in PaO2. due to changes in CPAP, did not correlate well with changes in &/wt nor wi& changes in'alveolar turnover rates. Thus, the effects of increasing CPAP on Pa02 were not simply due to increases in FRC. The changes in Pa02 are due to a complex relationship between changes in FRC, alveolar turnover rates, and to other alterations in cardiopulmonary function that are yet to be fully understood. SpeculationResults from this study show that large portions of the lung have alveolar turnover rates below normal. Increasing airway pressures could lead to decreases in the rate of pulmonary perfusion and to an improvement in the ventilation-perfusion ratio of these alveolar units. This could partially account for the observation that increases in CPAP generally produce increases in PaO2.CPAP (4, 12) has become a mainstay of therapy in the treatment of newborn infants with RDS. In order to optimally manage these the infants were 33.7 -+ 0.4 wk and 2013 + 93 g. Some infants were studied several times during the course of their disease so that 52 sets of data were gathered at infant postnatal ages ranging from 4 to 152 h (mean 38 h). Four of the infants required mechanical ventilation due to the inability to maintain Paon of 60 mm Hg with airway pressure greater than 15 cm HzO and FiOz of 1.0. Three of these four infants died of intraventricular hemorrhages. A fourth infant died of necrotizing enterocolitis. All four infants that died also had pneumothoraces, and were the only infants with pneumothoraces. EQUIPMENTOpen circuit nitrogen washout techniques were used to determine FRC, alveolar ventilation (VA), distribution of ventilation, and alveolar turnover rates. Respiratory nitrogen concentrations were measured by placing the Nitralyzer (model 391 nitrogen analyzer, Warren E. Collins, Inc., Braintree, MA) needle of the pickup head directly in the endotracheal tube; thus, the nitrogen signal to recording delay was only 15 msec for 90% response. Flow rates of respiration were measured by a heated Fleisch "00" pneumotachometer (Instrumentation Association, Inc., New York, NY).and integrated electronically (IC-RESP respiratory flow rate integrator, Gilson Medical Electronics, Inc., Middleton, WI) to obtain tidal volumes. The logarithmic output of the nitrogen analyzer and exhaled tidal volumes were recorded on an X-Y plotter (model 815 X-Y recorder, MFE Corp., Salem, NH). Calculations of VA and FRC and multiple space curve analyses were made directly on the recorded X-Y washout traces.infants the clinician needs to kn...

During the calendar year of 1974, the Intermountain Newborn Intensive Care Center at the University of Utah Medical Center had 603 admissions. A representative group of 293 charts were reviewed which indicated that 44% of these children were intubated from hours to weeks. The overall mortality rate for the 293 children was 29%. Eighteen of the 603 children were diagnosed as having subglottic stenosis. Fifteen of these children appeared to have acquired subglottic stenosis secondary to endotracheal intubation. Three children had congenital subglottic stenosis. Tracheostomy was necessary in the management of 15 patients. Ten of the 18 patients have survived and two of these patients still have tracheostomy tubes in place. The survival and thickness of the stenotic area are inversely proportional to the birth weight and the duration of intubation. Endoscopic excision, dilatation and stenting were techniques utilized in the treatment of these stenotic lesions. The extubation technique utilized is described. The factors involved in the production of acquired subglottic stenosis are presented along with suggestions to decrease the incidence of this problem in the intubated child.

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A home phototherapy program for healthy, term newborns with hyperbilirubinemia (n = 62) was implemented, and results were compared prospectively with a group of term neonates who met enrollment criteria but were treated in-hospital (n = 55). The purpose of the study was to prospectively assess the feasibility, safety, and effectiveness of home phototherapy in treating uncomplicated neonatal jaundice. Infants were required to be greater than 24 hours old and to weigh at least 2,270 g (5 lb). Enrolling physicians were instructed to select infants whose clinical diagnoses and bilirubin levels allowed an adequate margin of error for a trial of home phototherapy and whose parents were capable of managing the added responsibilities of home therapy. At the time the phototherapy equipment was delivered and set up in their home, parents received extensive instruction, including how to record pertinent ongoing data on a home flow sheet. Serum bilirubin levels were measured at least daily. The preponderant diagnosis listed by enrolling physicians was "physiologic jaundice." No parent reported significant complications, and no infant required rehospitalization. Bilirubin levels decreased as rapidly in the home group as in the hospitalized control group, and duration of treatment averaged 2.8 days. About $18,000 was saved by treating these 62 infants at home compared with in-hospital costs in our community. We found home phototherapy to be a feasible, safe, and effective alternative to in-hospital phototherapy for otherwise healthy, jaundiced infants with motivated and capable parents.