摘要:

In order to detect deletion mutation and/or gene conversion in the 21‐hydroxylase (21‐OH) gene, we adopted the polymerase chain reaction (PCR) method followed by electrophoresis. Two pairs of synthesized primers, Ta/lb and 2a/2b, each corresponding to the sequence at the 5′ portion of the 21‐OH gene, were set for PCR.Taql digestion of amplified DNA from normal individuals using Ta/lb as primers gave the following three fragments: an active 21‐OH gene‐derived 559 bp fragment, and pseudogene‐derived 364 and 195 bp fragments. Of 16 patients with 21‐hydroxylase deficiency (21‐OHD) studied, 6 (37%) lacked the 559 bp fragment. These 6 patients also lacked both the 331 and 117 bp Mval fragments of the PCR product which were obtained with the primers 2a/2b, both being derived from the active 21‐OH gene. These results indicate that 6 of the 16 patients have either deletion of the 21‐OH gene or conversion of the gene to its tandemly located pseudogene. The method described here provides a rapi

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1991.tb01512.x

出版商:Blackwell Publishing Ltd    

年代:1991

数据来源: WILEY

摘要:

In this study, we investigated both the efficacy of growth hormone (GH) in the improvement of growth disturbances in uremia and the effects on the remaining kidney, using uremic rats. Twenty 5/6 nephrectomized uremic rats were divided into two groups. One half of them were injected intraperitoneally with 2IU of human recombinant growth hormone (r‐hGH) twice daily. The others were injected only with the vehicle. Sham operated control rats were also injected with the vehicle. These rats were pairfed and observed for as long as 4 weeks, during which, the growth in stature in uremic rats without r‐hGH was markedly retarded, with a gain of only 15.8 ±3.7 mm of body length. It was significantly less than the 48.0 ± 5.1 mm (p<0.01) gained by control sham‐operated rats. By contrast uremic rats treated with r‐hGH gained 37.8 ±2.9 mm, which was significantly greater than uremic rats without r‐hGH (p<0.01) but less than controls (p<0.01). Increments of body stature paralleled those of skeletal bones. The food utilization rate was significantly improved in uremic rats treated by r‐hGH. Throughout the experiment, serum creatinine and urea nitrogen levels remained constant and there were no intergroup differences in the uremic rats including insulin like growth factor I (IGF‐1) and other chemical parameters except for plasma GH levels. These results suggest that r‐hGH stimulates somatic growth in rats with chronic renal failure through improving food utilization and a probable direct effect on

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1991.tb01513.x

出版商:Blackwell Publishing Ltd    

年代:1991

数据来源: WILEY

摘要:

The precision and reliability of the stable microbubble test as a predictor of respiratory distress syndrome (RDS) were studied. In blind experiments, the stable microbubble test was performed by three analysts in quadruplicate on six different amniotic fluid samples obtained from pregnant women whose babies developed RDS and on three samples prepared from mature controls. Three‐way analysis of variance revealed that there were statistically significant differential effects of the analyst, observer, and sampling on the stable microbubble values, and also that there were statistically significant interactions between the analyst and observer as well as between the analyst and sampling. However, the magnitude of these effects was not large enough to modify the interpretation of the test results. We conclude that the rapidity, simplicity and reliability of the stable microbubble test allow for its use as a bedside procedure in identifying infants who are likely to develop RD

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1991.tb01514.x

出版商:Blackwell Publishing Ltd    

年代:1991

数据来源: WILEY

摘要:

As a model system for mucocutaneous lymph node syndrome (MCLS), we have advocated and used mice which had been rendered tolerant toStreptococcus pyogenes‐associatedantigens by neonatal infection with group A fteta‐hemolytic streptococci, because these mice have shown a variety of peculiar bioimmunological characteristics bearing a striking resemblance to those of MCLS patients. The results of our current investigations reaffirmed the reliability of the animal model by indicating that mice subjected to neonatal infection with 5.pyogenes, or inoculation with streptococcal pyrogenic exotoxin (SPE) in Freund's adjuvant, were perfect counterparts of patients with MCLS on account of their platelet activation and hyperaggregability in response to provocative treatment, which are familiar findings in this dise

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1991.tb01515.x

出版商:Blackwell Publishing Ltd    

年代:1991

数据来源: WILEY

摘要:

The main cause of death in Kawasaki disease is myocardial infarction due to thrombotic occlusion of a coronary aneurysm. Intracoronary thrombolytic treatment was performed in 15 patients with Kawasaki disease with giant coronary aneurysms. Three patients had acute myocardial infarction, four demonstrated silent myocardial infarction, three suffered chest pain and five did not show ischemia features but had massive thrombus in the coronary aneurysms. Urokinase was infused into the coronary aneurysms as a bolus of 8,000 to 10,000 units/kg via a catheter over 10 minutes. Partial but significant coronary recanalization was achieved after injection of urokinase in a patient with acute myocardial infarction. Complete resolution of massive intracoronary thrombi was observed in 3 of 15 patients, and partial resolution was recognized in 4 cases. In 7 patients, the size of thrombus did not change. Recurrence of the thrombus was observed in 4 patients by serial two‐dimensional echocardiography. Urokinase was readmmistered and two showed significant reduction in the thrombus. All patients have been followed for more than 2 years with longest 8 years (mean: 3.3 yrs), and none have had a recurrence of myocardial infarction or died. These findings suggest that intracoronary urokinase is useful for the treatment and prevention of myocardial infarction in Kawasaki diseas

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1991.tb01516.x

出版商:Blackwell Publishing Ltd    

年代:1991

数据来源: WILEY

摘要:

A total of 370 children who underwent upper gastrointestinal endoscopy (534 procedures) were retrospectively studied. All procedures were performed successfully. Side effects were demonstrated in only one case (0.2%), which was complicated by perioral cyanosis during the procedure. As to preendoscopic medication, it was thought that topical pharyngeal anesthesia alone may well be used for school‐age children over 7 years of age, general anesthesia with endotracheal intubation for neonates, and intravenous sedation for infants and younger children. Of 370 patients studied, 70.8% had endoscopic diagnoses. The most common diagnosis was gastritis (136 cases), followed by peptic ulcer (75), duodenitis (29), and esophagitis (20). Several conclusions for indications were drawn from the present study. Pediatric upper gastrointestinal endoscopy is a safe and useful method for diagnostic and therapeutic approach, if adequate preendoscopic medication is chose

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1991.tb01517.x

出版商:Blackwell Publishing Ltd    

年代:1991

数据来源: WILEY

摘要:

Humoral and cellular immune responses were examined in 115 children with varicella and in 21 children who had been immunized with live varicella vaccine. Antibody determined with antibody‐dependent cell‐mediated cytotoxicity (ADCC) was detected earlier than neutralizing antibody (NT) in both groups. While humoral and cellular immunity were detected simultaneously in the natural varicella group, cellular immunity was detected earlier than humoral immunity in the immunized group. This suggests that ADCC, rather than NT, is involved in the early stages of recovery from varicella‐zoster virus (VZV) infe

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1991.tb01518.x

出版商:Blackwell Publishing Ltd    

年代:1991

数据来源: WILEY

摘要:

The epidemiology of rotavirus gastroenteritis was studied in the Matsuyama district from January to May 1988. Among strains of rotaviruses detected 46 showed the group C‐specific PAGE pattern of virus RNA. Group C rotavirus gastroenteritis occurred following an epidemic of group A rotavirus infection, primarily in children aged 4–7 years but rarely in those aged 0–1 years. This was seriologically confirmed after the epidemic. Unlike the previous strains detected in Matsuyama in 1985–1986, PAGE of the RNA showed arrangement of the 5th, 6th and 7th segments at similar intervals, suggesting virologic variation. The symptoms of fever, diarrhea and vomiting were milder than in group A rotavirus infection used as a

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1991.tb01519.x

出版商:Blackwell Publishing Ltd    

年代:1991

数据来源: WILEY

摘要:

A search of the literature has revealed only 12 reports of herpes zoster during infancy in Japan. Of these, seven cases were thought to be mother‐child infections where the mother had been infected with varicella and transmitted the VZV to the fetus, producing postnatal herpes zoster. The earliest case of maternal varicella infection occurred at 14 weeks of gestation. In the remaining 6 cases infection took place between the 17th and 32nd week of gestation. Postnatal onset of herpes zoster in the infants occurred within three months in two cases and at seven to eight months in the other four cases. In two cases it was unclear whether it was a mother‐child infection. In three cases no clinical manifestations of maternal VZV infection were observed, but in these cases during early infancy the patients might have acquired asymptomatic infect

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1991.tb01520.x

出版商:Blackwell Publishing Ltd    

年代:1991

数据来源: WILEY

摘要:

Serum and urinary galactitol levels were examined in two patients with classical galactosemia. Even under strict dietary therapy, galactitol levels were much higher than those of healthy controls. In a 6‐day‐old patient who had eaten large amounts of galactose before diagnosis, it took more than two weeks for urinary galactitol to decrease to the stable value. In an 8‐year‐old case, more than ten days were required for urinary galactitol to reach the base line level after a galactose load of 1.25 g/kg. In both patients, the urinary galactose levels decreased rapidly. After loading with a small amount of galactose (5 g, equivalent to 200 ml of milk) in the latter patient, higher levels of urinary and serum galactitol were maintained for a long time, as compared with galactose, but they returned to baseline level after 24 hours. From these results, we discussed the use of urinary galactitol as an index to check galactose

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1991.tb01521.x

出版商:Blackwell Publishing Ltd    

年代:1991

数据来源: WILEY