ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1987.tb02243.x

出版商:Blackwell Publishing Ltd    

年代:1987

数据来源: WILEY

摘要:

AbstractPediatrics has arrived at a turning point. When diseases in children and the trend of demand for health care in Japan are observed, new fields are expanding which differ from traditional pediatrics, which set its sights on reduction of the death rate and control of infectious diseases; the new pediatrics aims at functional recovery and optimal growth and development, and involves what has to be done mainly in outpatient clinics. Pediatricians who work in these institutions must make their efforts in the ambulatory field and establish a form of ambulatory pediatrics which meets the needs of our “softening” society. Our efforts are necessary for the well‐being of our juniors. I present a framework for the new ambulatory pediatrics and enumerate its various

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1987.tb02244.x

出版商:Blackwell Publishing Ltd    

年代:1987

数据来源: WILEY

摘要:

AbstractWe studied 233 patients with biliary atresia seen between 1953 and 1985. Specimens of the extrahepatic bile ducts and liver which were taken at the time of corrective operation were examined histologically. We noted the presence of inflammation in the extrahepatic bile ducts, which confirmed in retrospect the necessity for early operation. The results of these studies are discussed in connection with the pathogenesis, pathophysiology and clinical features of this disease.

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1987.tb02245.x

出版商:Blackwell Publishing Ltd    

年代:1987

数据来源: WILEY

摘要:

AbstractThe authors have devised a new, simple method for the diagnosis of adrenoleukodystrophy (ALD) using a dried blood spot sample. Fatty acid from the dried blood spot was extracted and methylated with HCl‐methanol. Fatty acid methyl esters were analyzed by gas chromatography‐mass spectrometry. Fatty acid composition of the blood spot from five patients with ALD and from five healthy controls was determined from the mass chromatogram of the m/z 143 ion, [(CH2)6COOCH3]±. The ratios of tetracosanoic acid to docosanoic acid (C24:0/C22:0) and of hexacosanoic acid to docosanoic acid (C26:0/C22:0) were significantly greater in ALD patients than in controls. The C24:0/C22:0 ratios of ALD patients and of controls were 2.08 ± 0.33 and 1.47 ± 0.16 (mean ± SD), respectively. The C26:0/C22:0 ratios of ALD patients and of controls were 0.31 ± 0.024 and 0.092 ± 0.041, respectively. The fatty acid composition of the dried blood spot did not change at room temperature within a week. Since the specimens can be sent by mail, this method could be applied to the screening of other “peroxisomal” diseases, such as neonatal ALD and Zellweger synd

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1987.tb02246.x

出版商:Blackwell Publishing Ltd    

年代:1987

数据来源: WILEY

摘要:

AbstractIt is generally considered that in complete right bundle branch block, the electrocardiographic standard for the diagnosis of right ventricular hypertrophy is not applicable.According to Cohen's criteria, right ventricular overload exists if thallium‐201 myocardial perfusion demonstrates the right ventricular wall, and in this light we attempted to reassess the validity of the electrocardiographic standard for right ventricular hypertrophy.Twenty‐three children with complete right bundle branch block (abbreviated as CRBBB) were evaluated. The agreement and disagreement ratios of the electrocardiographic standard with Cohen's criteria was 20/23 and 3/23 respectively. Even in patients without intracardiac anomalies, right ventricular overload was suspected by Cohen's criteria.The electrocardiographic standard was in agreement with Cohen's criteria in most of these patie

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1987.tb02247.x

出版商:Blackwell Publishing Ltd    

年代:1987

数据来源: WILEY

摘要:

AbstractFourteen patients with paroxysmal supraventricular tachycardia (PSVT) underwent serial electrophysiologic studies using transesophageal atrial pacing (EPS‐EP), to determine the predictive value of this method in the selection of antiarrhythmic therapy and to study the mechanism of the tachycardia.In each patient, PSVT could be reproducibly initiated by burst and/or programmed stimuli. After control EPS‐EP, the effects of several drugs (diltiazem, disopyramide, propranolol, digoxin, procainamide and verapamil) on the ability to initiate PSVT or to bring about “tachycardia zone” were examined. The mechanisms of PSVT were also investigated using an esophageal electrode, EPS‐EP, and Ca++‐antagonists.Drug responses were variable but in most cases tachycardia could not be initiated after drug administration. Four types of supraventricular tachycardia are described. In eight out of nine patients, good therapeutic control was obtained. This study shows that serial EPS‐EP can provide rapid identification of appropriate antiarrhythmic therapy, and is useful in studying the mech

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1987.tb02248.x

出版商:Blackwell Publishing Ltd    

年代:1987

数据来源: WILEY

摘要:

AbstractA patient with congenital nephrotic syndrome with focal segmental glomerular sclerosis is reported. There was moderate renal insufficiency, proximal renal tubular acidosis, hematuria and glycosuria. The glomerular basement membrane showed irregular thickening, splitting of the lamina densa and reticulation of the lamina rara interna. In addition to the features of the nephrotic syndrome and renal insufficiency, tubular dysfunction is one of the early manifestations of this disease.

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1987.tb02249.x

出版商:Blackwell Publishing Ltd    

年代:1987

数据来源: WILEY

摘要:

AbstractWe describe a 2‐year‐old boy with a non‐palpable right radial pulse associated with underdevelopment of the arm. An arteriogram showed a narrowing of the distal part of the right subclavian, axillary and brachial arteries, and poor collateral circulation. This patient illustrates a rare cause of an absent radial pulse, other than Takayasu's di

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1987.tb02250.x

出版商:Blackwell Publishing Ltd    

年代:1987

数据来源: WILEY

摘要:

AbstractWe present a 14‐year‐old girl with Turner's syndrome, agenesis of the corpus callosum, Hashimoto's thyroiditis and horseshoe kidney. She was admitted to our hospital because of short stature. She had some of the somatic stigmata of Turner's syndrome, was slightly mentally retarded, but had no neurological abnormalities. Chromosome study showed a 45, x karyotype. Her thyroid function was normal, but both the antithyroglobulin and antimicrosomal antibody titers were elevated, suggesting lymphocytic chronic thyroiditis. An intravenous pyelogram and an abdominal CT scan showed a horseshoe kidney. CT scan of the brain showed agenesis of the corpus callosum.As far as we are aware, this is the first report of Turner's syndrome with agenesis of the corpus callosum, Hashimoto's thyroiditis and horseshoe kid

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1987.tb02251.x

出版商:Blackwell Publishing Ltd    

年代:1987

数据来源: WILEY

ISSN:1328-8067    

DOI:10.1111/j.1442-200X.1987.tb02252.x

出版商:Blackwell Publishing Ltd    

年代:1987

数据来源: WILEY