ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1990.tb00775.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1990.tb00776.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

Three‐allele restriction fragment length polymorphisms (RFLPs) for the restriction endonuclease Bam HI are known at the hypoxanthine‐guanine phosphoribosyltransferase (HPRT, E.C.2.4.2.8.) gene locus. The alleles are expressed phenotypically on Southern blots as three distinct pairs of fragments that hybridize to HPRT cDNA: i)a 22‐kilobase (kb) / 25‐kb pair, ii) a 12‐kb / 25‐kb pair, and iii) a 22‐kb / 18‐kb pair. Allele frequencies in 119 unrelated Japanese people were 0.38 for the 22‐kb / 25‐kb allele, 0.43 for the 12‐kb / 25‐kb allele, and 0.19 for the 22‐kb / 18‐kb allele, an average heterozygosity of 66% in Japanese females, a higher rate than in Caucasian females. Five out of nine carriers of partial or complete HPRT deficiency showed heterozygou

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1990.tb00777.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

Antibody‐dependent cellular cytotoxicity (ADCC) and natural killer (NK) ativity were examined using MT‐2 cells persistently infected by HTLV‐1 as target cells, and mononuclear cells as effector cells, from helathy one‐week‐old newborn babies, infants, children and adults. More than 10% of ADCC was observed in 17 newborn babies out of 22 (77.3%) and in all 67 healthy one‐month‐old babies to adults, by adding serum from anti‐HTLV‐1 positive carriers. When anti‐HTLV‐1 negative serum was added, less than 10% of ADCC was observed. If infants without anti‐HTLV‐1 antibodies were breast‐fed they had the possibility of HTLV‐1 vertical transmission. There was no significant decrease in NK activity between 90 healthy newborn babies, infants, children, or adults. These results suggest that ADCC and NK activity protect against the transmission o

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1990.tb00778.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

The effects of psychosine on the metabolism of myelin associated glycolipids such as galactocerebroside and sulfatide in mouse brain cell cultures were investigated in order to clarify the mechanism of demyelination in globoid cell leukodystrophy (Krabbe's disease). The incorporation of 3H‐galactose into cerebroside and sulfatide was studied in the presence of psychosine (1–3 μg/ml medium). These data indicated that psychosine inhibited the incorporation of 3H‐galactose into cerebroside and sulfatide not in astroglial cell culture but in oligodendroglial cell culture. Oligodendrocytes produce myelin in the central nervous shystem, and cerebroside and sulfatide are major components of myelin. These results suggest that psychosine influences the lipid metabolisms of myelin and subsequently leads to the demyelination in Krabbe's d

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1990.tb00779.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

Metabolic disturbances of pancreatic hormones in obstructive jaundice in infancy were evaluated experimentally and clinically. In our experimental study, using young rats, the level of plasma insulin (IRI) gradually increased after ligation of the common bile duct. These levels were a little lower than those in the non‐treated controls. The level of plasma glucagon (IRG) incerased remarkably 4 weeks after ligation of the common bile duct. Clinically, there were no significant differnces in the levels of IRI and IRG among normal controls and cases of neonatal hepatitis and congenital biliary atresia (CBA). In CBA patients, these levels can be correlated with the progression of hepatic fibrosis; an increase in IRG and a decrease in teh IRI/IRG mol ratio was noticed in patients with grade III of hepatic fibrosis. These indicate that, in obstructive jaundice in infancy, the more severe the hepatic damage due to obstructive jaundice, the higher the level of plasma glucagon concentration will ris

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1990.tb00780.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

In order to establish the normal range of urinary growth hormone (GH) level for age and sex, which is important in the clinical application of urinary GH to diagnosis, we measured GH in the first morning urine specimens of 270 normal subjects aged 3 to 20 years. Urinary GH levels in patients wit documented GH deficiency were compared with those of the normal controls. In normal subjects, urinary GH levels showed a statistically significant change with age. They were relatively high in infancy and mid‐puberty, reaching a peak at 11 to 12 years in girls and at 13 to 14 years in boys. Urinary GH levels in patients with complete GH deficiency were apparently lower (<‐2.0SD) than those of the normal hildren, while the levels in patients withpartial GH deficiency overlapped with the normal range. When assessing GH secretion by using urinary GH measurement, consideration of age and sex is required, since the level changes isgnificantly wit these fact

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1990.tb00781.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

Three patients with vitamin D‐dependent rickets type II were given massive doses of 1α‐hydroxyvitamin D3for 29 to 36 months and their calcium‐phosphate balance was studied during treatment and one month after cesation of treatment. During treatment fasting hypercalciuria was observed in patient 1 and an increased rate of calcium excretion after calcium loading in patients 1 and 2. In these patients, calcium excretion was parallel with the serum 24, 25‐dihydroxyvitamin D. These findings suggested that the responsiveness to 1,25‐dihydroxyvitamin D improved during long‐term treatment of these two patients with vitamin D‐dependent r

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1990.tb00782.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

Twelve patients on continuous ambulatory peritoneal dialysis (CAPD) were studied in order to evaluate the progression of renal osteodystrophy (ROD). All patients received doses of 0.01 ‐ 0.02 μg/kg of 1 alpha vitamin D3(1α ‐ D3) and 0.1 ‐ 0.15 g/kg of calcium carbonate for 12 ‐ 18 months. Serum total protein, total calcium (Ca), creatinine, inorganic phosphate alkaline phosphatase (ALP), and n‐terminal parathyroid hormone were measured regularly. The radiological bone appearance for ROD or rickets and the height standard deviation score were compared between the outset and the end of this study. An increase of Ca values and a decrease of ALP values correlated with a suppression of hyperparathyroidism, and the hyperphosphatemia was controlled in the majority of our patients throughout this study. Two patients had ROD and rickets at the outset of this study, and all patients but one had neither ROD nor rickets at the end of this study. Growth retardation improved or remained stable in 10 patients. Our results indicate that adequate doses of 1α ‐ D3and calcium carbonate are effective in the prevention of ROD and rickets in pa

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1990.tb00783.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY

摘要:

The occurrence of hemorrhagic disease due to vitamin K (VK) deficiency beyond the neonatal period has come under investigation in Japan. In 1980 the 1st nationwide survey was conducted in Japan by Nakayama and others [1], and was followed by the 2nd nationwide survey in 1985 by Hanawa [2]. The present survey was designed to further monitor the incidence of this disease in Japan during the 3‐year period from July 1985 to June 1988. Questionnaires were sent to 1,315 hospitals having more than 200 beds, located throughout Japan. Responses were received from 775 hospitals, for an answer rate of 58.9%. The total number of reported cases was 175, including 129 idiopathic type, 28 secondary type and 18 near‐miss type. In this survey it was revealed that the incidence rate of the idiopathic type of vitamin K deficiency in infancy (VKDI) has decreased remarkably, to about one‐fourth that reported in the first survey. The declining incidence rate of VK deficiency in Japan is considered to be the result of ever more widespread prophylactic administration of VK during the neonatal period, as most occurrences of VK deficiency in infancy are preventable by prophylactic administration of VK from the neonatal period. However, in 16 cases of the idiopathic type of VK deficiency found in the present survey, VK had been administered at least once during or after the neonatal period. This shows the heterogeneity of this cond

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1990.tb00784.x

出版商:Blackwell Publishing Ltd    

年代:1990

数据来源: WILEY