摘要:

ABSTRACT.A 30‐week gestation boy developed severe unilateral interstitial emphysema whilst being ventilated. This was successfully treated by selective bronchial intubation. The problem of right upper lobe collapse was overcome by using an endotracheal tube with an additional side hol

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1982.tb09390.x

出版商:Blackwell Publishing Ltd    

年代:1982

数据来源: WILEY

摘要:

ABSTRACT.A female infant born to a mother who had an elevated serum alpha‐fetoprotein during early pregnancy, presented a combinstion of epidermolysis bullosa and aplasia cutis congenita. She developed evidence of upper gastrointestinal obstruction and died at the age of 43 hours. Post‐mortem examination showed the presence of pyloric atresia and electron microscopy of skin biopsies showed epidermolysis bullosa simplex. Examination of the placenta revealed a unique abnormality of the membranes, indicating the existence of two sacs. This case and the previously reported cases, which are reviewed, suggest an autosomal recessive inheritance. Serum alphafetoprotein estimation, ultrasonography and fetoscopy with skin biopsy are suggested as a means of pre‐natal diagnosis in future pregna

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1982.tb09391.x

出版商:Blackwell Publishing Ltd    

年代:1982

数据来源: WILEY

摘要:

ABSTRACT.A 17‐month‐old boy who contracted a severe generalized BCG infection is reported. He was treated for 2 years with streptomycin, isoniazide and rifampicin and is now, at the age of 5 years, apparently cured. The boy had an unexplained discrepancy between humoral and cellular PPD reactivity for several years, but conventional immunological studies including plasma immunoglobulin levels, blast transformation as well as MLC reactivity were all normal. This is, to the best of our knowledge, the first child reported who has survived a generalized BCG infect

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1982.tb09392.x

出版商:Blackwell Publishing Ltd    

年代:1982

数据来源: WILEY

摘要:

ABSTRACT.A 6 month‐old girl with vomiting, hypotonia and motor retardation was found to have elevated blood lactate, pyruvate, and branched chain amino acids essociated with ketoglutaric aciduria. The combination of a congenital lactic acidosis with a variant form of maple syrup urine disease and ketoglutaric aciduria suggested a defect of a single component, common to pyruvate dehydrogenase, to branched chain ketoacid dehydrogenase, and to α‐ketoglutarate dehydrogenase. Dihydrolipoyl dehydrogenase is the common component (E3). The three enzyme activities and the E3component activity were found to be reduced in liver and cultured fibroblasts, thus confirming that a single defect of this component can result in a multiple deflciency involving several oxidative decarboxylation react

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1982.tb09393.x

出版商:Blackwell Publishing Ltd    

年代:1982

数据来源: WILEY

摘要:

Book reviewed in this article:W. M. Lieberman:Pediatric gastroenterology. Case studies.L. Hersov&I. Berg:Out of school. Modern perspectives in truancy and school refusal.D. M. Berwick, S. Cretin&E. Keeler:Cholesterol, children and heart disease.G. P. Sholevar, R. M. Benson&B. J. Blinder:Emotional disorders in children and adolescents.B. E. Clayton, P. Jenkins&J. M. Round:Paediatric chemical pathology. Clinical tests and reference ranges.

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1982.tb09394.x

出版商:Blackwell Publishing Ltd    

年代:1982

数据来源: WILEY

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1982.tb09395.x

出版商:Blackwell Publishing Ltd    

年代:1982

数据来源: WILEY