Roundtable: survival and outcome of the extremely low-birthweight infant.
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Biomedical subjects
Publications and source records attributed to H Harrison.
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We report the identification and initial family and population studies of a previously undescribed serum protein polymorphism with two allelic forms. It was discovered in Hutterites, a reproductively isolated religious sect, and is also present in Australian aborigines and a sample of Chicago residents. A two-allele model is consistent with the segregation pattern observed in five kindreds within our initial study group. This polymorphism, provisionally designated SPPM-158, appears as a horizontal (charge-based) doublet in silver-stained ISO-DALT high-resolution two-dimensional electrophoresis gels. It is a low-concentration polypeptide (approximately 1 mg/dL) that has an apparent MWSDS of 43.6 kD and an isoelectric point of approximately 5.5. We infer that it circulates as a multimer or in a high-molecular-weight (greater than 200 kD) complex with other proteins because it is not observed in normal body fluids derived from physiologically ultrafiltered plasma such as amniotic fluid, urine, or cerebrospinal fluid; however, it is present in urine of patients with glomerular proteinuria. The high heterozygosity rates imply utility of this new serum protein marker for both forensic and population studies.
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A seven-week-old infant with multiple areas of coarctation of the abdominal aorta and renal artery pathology underwent staged surgical repair of his malformations in our medical center. The clinical and routine echocardiographic evaluations suggested the diagnosis of atypical coarctation of the aorta. The exact anatomical characteristics of his vascular malformations were established by repeated angiocardiographic studies, which should be performed in any patient in which the diagnosis of atypical coarctation of the aorta is suspected.
Two infants are reported each with a mass of heterotopic gastric tissue in the hypopharynx causing airway obstruction. Endoscopic laser removal of the tumours proved a satisfactory method of treatment. Gastric heterotopia in the head and neck region is rare but should be considered as a cause of stridor in infants.
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Infant orthotopic cardiac transplantation has been recently applied to various forms of congenital heart disease with encouraging short-term results. Between June 1986 and September 1987 we evaluated 16 infants for orthotopic cardiac transplantation. Fourteen had hypoplastic left heart syndrome, one had endocardial fibroelastosis with aortic atresia, and one had anomalous pulmonary arterial origin of the left main coronary. Eight families accepted the treatment program and eight families refused (two because of associated anomalies and six on philosophical grounds). Of the eight patients who were candidates for orthotopic cardiac transplantation, one died 6 hours after diagnosis, one was allowed to die after 60 days because of acquired neurologic complications, and another had congenital cytomegalic virus infection. The remaining five patients (four with hypoplastic left heart syndrome, one with anomalous pulmonary arterial origin of the left main coronary) had orthotopic cardiac transplantation. The operation was performed with absorbable polydioxanone suture with deep hypothermia and circulatory arrest in four neonates for hypoplastic left heart syndrome (average time 47 minutes) and bicaval cannulation and continuous bypass in one 11-month-old infant for anomalous origin of the left main coronary. In-house retrieval was used in all. One neonate died of complications as a result of pretransplant donor heart dysfunction and size discrepancy, whereas the remaining three neonates and one infant survived and are home 23 months, 12 months, and 8 months (the patients with hypoplastic left heart syndrome) and 17 months (the patient with anomalous origin of the left main coronary) postoperatively. Triple-drug immunosuppression included cyclosporine, azathioprine, and prednisone. Rejection was diagnosed by clinical evaluation of child activity and monocyte cell cycle analysis from peripheral blood samples without myocardial biopsies. Routine echocardiograms, electrocardiograms, and chest x-ray films were not helpful. Six episodes of rejection were successfully treated in four patients. Twelve-month postoperative catherization in one patient (hypoplastic left heart syndrome) showed appropriate graft growth, no aortic or pulmonary anastomotic strictures, normal right and left ventricular function, and no coronary artery disease. We conclude that infant orthotopic cardiac transplantation is an acceptable procedure for severe forms of untreatable congenital heart disease. The excellent short-term results warrant continued application of orthotopic cardiac transplantation.
The antidiuretic effect of two prostaglandin synthetase inhibitors, ibuprofen (25 mg/kg/day) and indomethacin (2 mg/kg/day), was studied in patients aged 8 to 18 years with hereditary nephrogenic diabetes insipidus. Ibuprofen (studied in five patients) did not have demonstrable effects on urine volume, free water clearance, or osmolar clearance, but fractional excretion of sodium decreased from a mean of 0.38% to 0.19% (P less than 0.05). In contrast, indomethacin (studied in three patients) was associated with a decrease in mean urine volume from 5.8 to 2.8 mL/min and a decrease in mean free water clearance from 3.1 to 1.1 mL/min (both P less than 0.05). Fractional excretion of sodium decreased from 0.77% to 0.27% (P less than 0.01) and was accompanied by an increase in serum urea nitrogen level (P less than 0.01) and a decrease in urea nitrogen clearance (P less than 0.025). Thus, prostaglandin synthetase inhibitors are not uniformly effective in treatment of nephrogenic diabetes insipidus. The inhibitory effect of indomethacin on urine volume and free water clearance in our patients may have been mediated by an enhancement of antidiuretic hormone (ADH)-stimulated cyclic adenosine monophosphate generation, or by increased ADH-independent water reabsorption resulting from an increase in solute reabsorption and consequent medullary hypertonicity.
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This study examined the reliability and validity of the Preschool Play Scale (PPS), a measure of the developmental level of play, on a sample of 60 handicapped children. Results demonstrated acceptable reliability and validity for the scale as a whole. However, the need for some refinement of the scale was revealed by the findings. Categories of play that can be observed only infrequently need to be reconsidered. The data also suggest that the criteria for deciding whether a behavior is absent or whether there was no opportunity to observe the behavior need to be spelled out more clearly for the scale.
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The expression of traits linked to pathogenicity was studied in a population of Escherichia coli K1 strains. It was found that E. coli K1 strains isolated from extraintestinal infection harbor the ColV plasmid and express mannose-resistant hemagglutinating activity type VI with a high frequency. The presence of these properties may play a role in the ability of some E. coli K1 serogroups to invade.
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