ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1981.tb01636.x

出版商:Blackwell Publishing Ltd    

年代:1981

数据来源: WILEY

摘要:

AbstractA survey study was done on the Prader‐Willi syndrome in Japan during the 5‐year period between 1975 and 1979. The total number of cases was 137 and detailed data were obtained on 61 cases. The patients had muscular hypotonia during infancy. After the age of 3 years, obesity developed and diabetes mellitus was often observed in the cases older than 9 years. As for minor anomalies, almond eyes were the most common, and next was a fish‐shaped mouth. In some cases, hypogonadism was due to hypofunction of the testes, though in others it was due to a disorder of the hypotha

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1981.tb01637.x

出版商:Blackwell Publishing Ltd    

年代:1981

数据来源: WILEY

摘要:

AbstractAutopsy findings in two cases of Prader‐Willi syndrome were described. Case 1. was a 4–3/12 year‐old boy who had hypotonia in the neonatal period, hypogonadism, mental retardation and obesity. Autopsy revealed edema, congestion and focal bleeding in the lung, cardiac hypertrophy, localized degeneration of corpus callosum, nesidioblastosis, fatty liver and absence of testicular tissue. Case 2. was a3–11/12 year‐old boy who had hypotonia in the neonatal period, hypogonadism, mental retardation, obesity and recurrent respiratory tract infections. Autopsy revealed multiple thrombosis, pulmonary changes including edema, congestion, focal bleeding and infarcts, cardiac hypertrophy, fatty liver and absence of testicular tissue. These findings were thought to be summarized in the following three categories; 1) terminal changes characterized by pulmonary edema and congestion and cardiomegaly probably due to respiratory failure, 2) aquired changes due to metabolic abnormalities in this syndrome including fatty liver, nesidioblastosis and focal degeneration of the central nervous system; and 3) congenital abnormalities characterizing hypogonadism. (Acta Paediatr Jpn 23(3):289

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1981.tb01638.x

出版商:Blackwell Publishing Ltd    

年代:1981

数据来源: WILEY

摘要:

AbstractTo elucidate the pathogenesis of hypotonia in the Prader‐Willi syndrome, a histological approach towards the five cases was adopted, and traditional histological, histochemical and electron microscopic studies were perfomed. As a result, in light microscopic study type 2 fiber atrophy was found in all the five cases examined, and a combination with type 2 predominance in 3 cases. In addition, localized myopathic change was found in one case and regenerated fiber in another. In electron microscopic study, dilatation of sercoplasmic reticulum, mitochondrial swelling, increased glycogen particles, disappearance of myofilament and partial streaming of Z disc were observed. The presence of these findings led us to propose the hypothesis that there were some disorders in trophic influence on the muscle from the central nervous sytem, mainly in intrauterine life, of subjects with the Prader‐Willi syndrome. (Acta Paediatr Jpn 23(3): 294–300

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1981.tb01639.x

出版商:Blackwell Publishing Ltd    

年代:1981

数据来源: WILEY

摘要:

AbstractTen patients with typical Prader‐Willi syndrome were studied for their short stature, hypogonadism, and obesity. Tne following results were obtained.1) GH secretion was variable, ranging from subnormal to normal, although all shared short stature in common.2) Two of the 4 adolescent patients were diagnosed as having hypo‐gonadotropic hypogonadism. The remainder disclosed normal response to LH‐RH stimulation. Of the two patients with normal LH‐RH stimulation test, one showed normal testosterone production.3) In one child who developed overt diabetes, there remained elevated basal plasma insulin and depressed RBC‐insulin binding, despite weight control. There appears to be a significant heterogeneity in endo crinological derangement of Prader

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1981.tb01640.x

出版商:Blackwell Publishing Ltd    

年代:1981

数据来源: WILEY

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1981.tb01641.x

出版商:Blackwell Publishing Ltd    

年代:1981

数据来源: WILEY

摘要:

AbstractHuman lymphoblastoid cells (NC–37) were infected with two strains of herpes simplex virus type 1 (HSV–1). Persistent infections with two strains (a freshly isolated strain, Seike strain, and Miyama strain) of HSV–1 were established in NC–37 cells. In NC–37 cells infected with HSV–1 (Seike), the growth of cells was inhibited,6–72% of viable cells were positive for HSV‐antigen by immuno‐fluorescence, and the percentage of HSV‐antigen positive cells seemed to be inversely related to that of viable cells. Growth of cells and infectious virus were seen for more than 396 days without external support. NC‐37 cells infected with HSV‐1 were subcultured with fresh medium containing human gammaglobulin derivatives. When the percentage of HSV‐antigen positive cells was low (6%), the percentages of HSV‐antigen positive cells decreased to nearly 0% on the 6th day. When the percentage was high (47‐52%), it was not reduced to 0% after one subculture. It was reduced to nearly 0% after three subcultures, and no infectious virus was detected in the treated cells and cultured fluids after more than 16 days.The results of the present study support the speculation that HSV continues to associated with lymphoid cells for a long period of time after appearance of neutralizing antibody, at least for two weeks, and the lymphoid cells infected with HSV have a relation to the pathogenesis of herpetic encephalitis. (Acta Pa

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1981.tb01642.x

出版商:Blackwell Publishing Ltd    

年代:1981

数据来源: WILEY

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1981.tb01643.x

出版商:Blackwell Publishing Ltd    

年代:1981

数据来源: WILEY

摘要:

AbstractThe urinary concentrations of total protein, low molecular weight proteins, amylase, and total amino acid were determined from 104 healthy newborn infants during the first 15 days of life. The urine samples were collected after spontaneous voiding every two hours. Neonatal proteinuria was found in 70 per cent of 2 day old infants. No infant older than 8 days showed proteinuria. The ratio of mean value of β2‐microglobulin to creatinine was highest on the 5th day of life, followed by a rapid decrease after the 6th day. On the 15th day the β2‐microglobulin concentration was still high. The mean ratios of total amino acid to creatinine and amylase to creatinine increased gradually during the first 9 days of life, and then decreased rapidly after the 10th day. The highest urinary concentration of other low molecular weight proteins was found between the 7th and 8th day. On the other hand, the lowest concentration of albumin was found between the 7th and 8th day. These findings suggest that the proximal tubular function is immature in the newborn period. (Acta Paediatr Jpn 23(3):326–3

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1981.tb01644.x

出版商:Blackwell Publishing Ltd    

年代:1981

数据来源: WILEY

摘要:

AbstractSince little is known about the functions of eosinophils, I have examined some properties of eosinophils, for the purpose of knowing the role of eosinophils against bacterial infection.Eosinophils were obtained from peritoneal fluid and peripheral blood of a girl and five adults with eosinophilia. The isolated eosinophil preparations contained 82˜92% eosinophils.Chemotaxis was measured by an agarose plate method. Bactericidal activity was determined using a modified method of Quie. NBT dye reduction test was performed by the method of Baehner‐Nathan. CL was measured by the method of Yanai.Eosinophils showed decreased levels of chemotactic response, as compared with neutrophils. Although the peritoneal eosinophils showed similar bactericidal activity, the peripheral eosinophils had a lower activity than neutrophils, especially against St. aureus.NBT dye reduction capacity of eosinophils increased during phagocytosis. CL burst of eosinophils was greater and lasted longer; however, the maximum peak was found later than that in that of neutrophi

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1981.tb01645.x

出版商:Blackwell Publishing Ltd    

年代:1981

数据来源: WILEY