ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1973.tb17064.x

出版商:Blackwell Publishing Ltd    

年代:1973

数据来源: WILEY

摘要:

SUMMARYThree boys, 4, 5, and 10 years old, with psycho‐motor retardation, slightly gargoyle‐like faces and recurrent infections were found to have vacuolized lymphocytes in the blood and bone marrow as well as diffuse skeletal alterations. Electron microscopy of liver biopsy specimens revealed vacuoles, presumably representing enlarged lysosomes engorged with storage material. The liver. a‐mannosidase activity was somewhat low but not absent, while several other acid hydrolases in the liver had very high activities. The a‐mannosidase activity was low also in white blood cells but not in plasma or urine. The liver and urine contained very high amounts of mannose‐rich compounds. It is concluded that the patients suffer from man

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1973.tb17065.x

出版商:Blackwell Publishing Ltd    

年代:1973

数据来源: WILEY

摘要:

SUMMARYIn a group of 35 children first classified as “chronic hepatitis”, the study of the histo‐logical patterns revealed only 16 cases as actual chronic hepatitis: 3 persistent nonaggres‐sive hepatitis (type l), 6 chronic aggressive hepatitis (type 2a) and 7 chronic aggressive hepatitis (type2 b). As previously reported, there was a prevalence of females. The incidence increases with age. A history of acute hepatitis was found in only 4 of the 16 children. Australia antigen was positive in only 3 of 9 children tested.Persistent nonactive hepatitis has a benign evolution while chronic active hepatitis with histological aggressivity shows acute rapid It It is for this reason that massive and prolonged corticosteroid therapy has to be utilized in the latter group, since the few control studies have demonstrated a longer s

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1973.tb17066.x

出版商:Blackwell Publishing Ltd    

年代:1973

数据来源: WILEY

摘要:

SUMMARYFrom among more than 1 200 children aged 3–48 months of low socio‐economic families living in rural, suburban and urban Lebanon two contrasting groups were selected according to their Index of Thriving for detailed study at home (53 Thriving and 62 Failing to Thrive). Family, socio‐economic, living conditions, health, and dietary data were obtained. Numerous statistically significant differences were found in all categories of data between the two groups, with poorer status invariably shown by the Failing to Thrive families. The implications of the multi‐factorial nature of the causation of failure to thrive are di

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1973.tb17067.x

出版商:Blackwell Publishing Ltd    

年代:1973

数据来源: WILEY

摘要:

SUMMARYRenal function was investigated in 16 cases of nephroblastoma, in whom nephrectomy had been performed 2–19 years previously. All but one of these patients were also given postoperative radiation. The average radiation dose to the remaining kidney ranged from about 250 to about 1 700 rads in 17–63 days.None of the patients had clinically manifest renal dysfunction. The glomerular filtration rate was examined in 16 patients, the clearance of PAH and the filtration fraction in 15, the renal sodium balance in 8 and the acid‐base balance in 13.The glomerular filtration rate and the clearance of PAH were 70–100 per cent of the normal values for healthy children with 2 kidneys. Subclinical impairment of the sodium balance control was found in 6 patients, and of the acid‐base balance in 9. Abnormal regulation of the acid‐base balance was more noticeable in the patients given higher radiation doses. The glomerular filtration rate, clearance of PAH, filtration fraction and the sensitivity of the sodium balance control were not correlated to the rad

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1973.tb17068.x

出版商:Blackwell Publishing Ltd    

年代:1973

数据来源: WILEY

摘要:

SUMMARYThree infants, in whom the malabsorption syndrome, small intestinal. mucosal damage and clinical cow's milk intolerance were found, were challenged with cow's milk after initial treatment with breast milk. The small intestinal mucosa was investigated with light and electron microscopy both before and after provocation.Clinically one patient reacted rapidly in a few hours and showed round cell infiltration in the surface epithelium and lamina propria 20 hours after a single challenge. Electron microscopy showed short microvilli, abnormal nuclei and thickened basement lamina in the surface epithelium. Two patients reacted slowly. One of them showed similar changes 4 days after commencement of the provocations, but the changes were much more evident 38 days later, when symptoms were also apparent. At this time large accumulations of lysosomes in the apical part of the surface epithelial cell and marked thickening of the basal lamina with accumulations of whirly collagen fibres were detected. The third patient reacted in a milder way, both clinically and morphologically. This study indicates that cow's milk, apparently through its protein fraction, may damage the surface epithelium of the small intestinal mucosa. These alterations during provocation periods resemble those found in coe‐liac disease during gluten provocatio

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1973.tb17069.x

出版商:Blackwell Publishing Ltd    

年代:1973

数据来源: WILEY

摘要:

SUMMARYThe activities of various enzymes in the ery‐throcytes of newborn infants, suffering from transitory hyperbilirubinaemia, have been studied. Phosphoglucose isomerase (p‐value = 92%) and glyceraldehyde‐3‐phosphate de‐hydrogenase (p‐value = 87%) show a decrease in icteric children. These p‐values were regarded as sufficient for furth'er investigations, because bilirubin level is influenced by many different factors. Because of the importance of glyceraldehyde‐3‐phosphate dehydrogenase for the intraerythrocyte 2,3‐diphosphoglycer‐ate level, 2,3‐diphosphoglycerate was measured and correlated to serum bilirubin. A steady decline in icteric children i

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1973.tb17070.x

出版商:Blackwell Publishing Ltd    

年代:1973

数据来源: WILEY

摘要:

SUMMARYTen infants with early congenital syphilis are presented. Hepatosplenomegaly is a marked feature in all the infants. The majority of them were of low birth weight. Syphilitic pemphigus was present in 5, and hydrops fe talis in 4. Five infants survived, including one witn hydrops fetalis. Early diagnosis and intensive therapy are necessary for survival. A high index of swpicion is required for early and more frequent diagnosis of this condition.

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1973.tb17071.x

出版商:Blackwell Publishing Ltd    

年代:1973

数据来源: WILEY

摘要:

SUMMARYThe hypothalamic pituitary adrenal system was studied in normal Egyptian children and in groups of children with some common viral infections including measles, chicken pox, mumps and poliomyelitis. Results showed a significant rise in plasma 17‐OHCS levels in all acute viral infections. ACTH and metyrapone tests revealed that the hypothalamic‐pituitary‐adrenal axis was functioning adequately. The value of corticosteroids in the management of acute viral infections is discussed in the light of our clinical and laboratory fin

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1973.tb17072.x

出版商:Blackwell Publishing Ltd    

年代:1973

数据来源: WILEY

摘要:

SUMMARYTransport of lysine and glycine was studied in cultured fibroblasts of a patient with Lowe's oculocerebrorenal syndrome. Although patients with this syndrome demonstrate renal and intestinal amino acid transport defects, no abnormality was found in our study.

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1973.tb17073.x

出版商:Blackwell Publishing Ltd    

年代:1973

数据来源: WILEY