ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1992.tb00936.x

出版商:Blackwell Publishing Ltd    

年代:1992

数据来源: WILEY

摘要:

A prospective study was performed in Okazaki, Japan, to attempt to establish a more effective system of prophylaxis for vitamin K deficiency in infancy (VKDI). During the first year, a Normotest (Hepaplastintest) was performed in all infants at one week and at one month of age. Two mg of vitamin K was administered orally to those whose Normotest values were below 40%. i.e., the non‐prophylactic administration of vitamin K (NPVK). During the second year of the study, all newborn infants received prophylactic vitamin K (PVK) within 24 hours of birth and at one week of age. The dosage was repeated at one month of age depending on the Normotest value. A total of 7,059 infants, comprising 93.3% of the live births in the city of Okazaki, were enrolled in this study. Data from 5,431 of these infants were used in the analysis of the results. In the NPVK group, 20 of the 2,791 infants had Normotest values below 40% at one month of age while 20 of the 2,640 in the PVK group had low values despite the prophylactic administration of vitamin K. Considering the prevalence of low Normotest values (40%) at one month of age and the predicted Normotest values, it was concluded that the month of birth (June September), the age of the mother (21–29 years), the birth order (first‐born) and male sex are risk factors for vitamin K deficiency in in

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1992.tb00937.x

出版商:Blackwell Publishing Ltd    

年代:1992

数据来源: WILEY

摘要:

Blood Spot 17α‐hydroxyprogesterone (17‐OHP) concentrations in neonates, especially in premature babies, were determined in relation to 1) the gestational age at birth, 2) the equivalent age of gestation at blood sampling and 3) the birth weight. The 17‐OHP concentrations were found to be higher with prematurity. Accordingly, the cut‐off limit in screening for congenital adrenal hyperplasia (CAH) in premature infants is proposed as 20 ng/ml. Ideal cut‐off limits were set by the equivalent age of gestation at blood sampling. Cut‐off limits on the basis of gestational age at birth and birth weight are also suggested, where the sampling age is not so advanced. The rate of false positivity in premature infants can be reduced by

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1992.tb00938.x

出版商:Blackwell Publishing Ltd    

年代:1992

数据来源: WILEY

摘要:

Perceptual sound localization is determined from differences in time and loudness of signals arriving separately at bilateral ears, with the time difference being the more important one. The author examined the development of sound lateralization using a self‐recording device developed by Sato. It measures the sensitivity of sound lateralization on the basis of time and loudness differences separately. Subjects were 59 children aged 2 to 18 years and 12 adults who were audiologically and neurologically normal. Sound stimulus was 500Hz continuous band noise. (1) The testable rate was increased in children older than the age of 4, and all children over 7 years of age could be tested. (2) Children over 4 years of age could lateralize sound on the basis of time difference. Sensitivity to time differences improved rapidly, showing a steep curve, in children from the ages of 4 to 6, and then increased slowly until adulthood. (3) Sensitivity to loudness differences increased and standard deviation decreased with age. There is a possibility that children aged 4 can discriminate loudness difference almost as well as adult

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1992.tb00939.x

出版商:Blackwell Publishing Ltd    

年代:1992

数据来源: WILEY

摘要:

The histological and histochemical findings in the respiratory muscles of a patient with severe neonatal nemaline myopathy are described. The patient suffered from frequent pneumonia associated with vomiting due to gastroesophageal reflux and died at 3 months from respiratory failure. The diaphragm was moderately involved and the intercostal muscles mildly involved. Core/targetoid structures were observed in the diaphragm and intercostal muscles.

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1992.tb00940.x

出版商:Blackwell Publishing Ltd    

年代:1992

数据来源: WILEY

摘要:

Etiology and pathogenesis of MCA/MR in 1,023 patients (618 male; 405 female) with mental retardation were studied. Of 1,023 patients, there were 563 cases (317 male; 246 female) with MCA (55%). Among the MCA patients, there were 303 (156 male; 147 female) whose primary etiology was clarified (53.8%). Among the 260 patients with MCA/MR of unknown etiology, there were 23 with recognizable syndromes of unknown etiology and 7 previously reported by us as possibly having a new malformation syndrome. We had 569 patients with mental retardation of unknown etiology including 236 (41.5%) who were involved with MCA.

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1992.tb00941.x

出版商:Blackwell Publishing Ltd    

年代:1992

数据来源: WILEY

摘要:

Over a 14 year period, there were 20 patients who presented with staphylococcal empyema from whom methicillin‐resistantStaphylococcus aureus(MRSA) was isolated. Twelve cases were community‐acquired and 8 were hospital‐acquired infections. Patients were treated with penicillinase‐resistant penicillin, cephalosporin or carbapenem in combination with or without aminoglycoside. They were also treated with drainage or thoracentesis. However, they were refractory to treatment and 7 patients, 6 of whom were suffering from bacteremia, died. One bacteremic patient was treated with vancomycin and was cured. In an area of endemic MRSA, vancomycin may be the first choice in the initial treatment of staphylococcal empyema until antimicrobial susceptibility can be det

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1992.tb00942.x

出版商:Blackwell Publishing Ltd    

年代:1992

数据来源: WILEY

摘要:

A patient with 3‐hydroxy‐3‐methylglutaric aciduria was diagnosed using gas chromatography mass spectrometry. The patient had severe metabolic acidosis, hypoglycemia and hyperammonemia and excreted abnormal amounts of 3‐methylglutaconic, 3‐hydroxy‐3‐methylglutaric, 3‐methylglutaric, 3‐hydroxyisovaleric and glutaric acids in the urine. 3‐Hydroxy‐3‐methylglutaric acid appeared as two peaks on the chromatogram after trimethylsilylation. One was a tri‐trimethylsilyl and the other a di‐trimethylsilyl derivative. 3‐Methylglutaconic acid appeared as three peaks: cis‐, trans‐ and cyclic cis‐isomers. The structure of these derivatives was elucidated by deuterium‐labeled trimethylsilyl derivatization. The di‐trimethylsilyl derivative of 3‐hydroxy‐3‐methylglutaric acid and the cyclic cis‐isomer of 3‐methylglutaconic acid do not appear to have been previously described. After treatment with leucine restriction milk, the excretion of leucine catabolites decreased but 3‐methylglutaconic and 3‐hydroxy‐3‐methylglutaric acids continued to be excreted at abnormally high levels. It is concluded that these two metabolites are necessary for the chemical diagnosis of HMG‐CoA lyase deficiency. This patient is the fir

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1992.tb00943.x

出版商:Blackwell Publishing Ltd    

年代:1992

数据来源: WILEY

摘要:

An unusual variant of chromosome 16, 16p+, in three generations is described. This is the first clinical report of 16p+ in Japan.

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1992.tb00944.x

出版商:Blackwell Publishing Ltd    

年代:1992

数据来源: WILEY

摘要:

A 7 year old boy, who presented with streptococcal infection, was found to have a low serum complement level (CH50). The C9 component was undetectable. His CH50 rose to the normal value and remained normal for at least three weeks, but decreased to one‐third of the normal level three months later. Family studies were consistent with a familial C9 deficiency, with autosomal co‐dominant inherita

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1992.tb00945.x

出版商:Blackwell Publishing Ltd    

年代:1992

数据来源: WILEY