[Urinary lithiasis in children: 54 cases].
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Biomedical subjects
Publications and source records attributed to D Claus.
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The article introduces a patient of 14 years of age in whom clubfeet as well as paraspasticity had developed from his sixth year of life. Instead of the previously suspected heredodegenerative disease, a lipoma was found in the distal spinal canal which had prevented ascension of the spinal cord and had thus produced the described phenomenon.
Cranial computerized tomography was carried out in 110 patients with cerebellar ataxia [53 with Friedreich's ataxia, 4 with Marie's spastic ataxia, 51 with cerebellar atrophy, and 2 patients with olivopontocerebellar atrophy). In CT scans, cerebellar atrophies are found to be of various localization and partially of characteristic distribution. CT, therefore, greatly helps to distinguish different types of cerebellar and spinocerebellar atrophies and allows the differentiation of cerebellar atrophies of various origins from other diseases, such as multiple sclerosis.
The clinical and pathologic findings of a case of cystic partially differentiated nephroblastoma in a 5-month-old boy is reported. We believe that it probably represents the differentiated counterpart of nephroblastoma. After nephrectomy, the child was given a short course single cytotoxic therapy with vincristine for 6 months.
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Cranial computerized tomography was carried out in 69 patients with cerebellar ataxia (45 with Friedreich's ataxia, 4 with Marie's spastic ataxia, 14 with cerebellar atrophy, and one patient with olivo-pontocerebellar atrophy). In CT scans cerebellar atrophy is found to be of various localisation and partially of characteristic distribution. CT, therefore, greatly helps to distinguish different types of cerebellar and spino-cerebellar atrophy and also distinguishes separate cerebellar atrophy of various origin from other diseases like multiple sclerosis.
Symmetrical calcification of the basal ganglia was found in 2% of 8000 computerized tomography (CT) scans. Of 19 cases, only 2 were detectable on conventional skull films. The less prominent calcifications were most often found in the region of the pallidum, the knee of the internal capsule. Also, the lesions were generally symmetrical. Thus these factors must be considered basic morphological characteristics of the pathophysiological process. Additional neurological disorders were present in 6 patients. Neurological symptoms in the remaining 13, when present, depended on the extent of the lesion. The most common finding was tremor, although disturbances of fine motor control, transient lateralizing signs, and seizures were also noted. No particular constellation of symptoms or signs permitted accurate clinical localization of the lesions. The patients could be divided into three groups on the basis of clinical findings: (1) young people with marked cerebral calcinosis ('idiopathic' calcification of basal ganglia), (2) patients with hypoparathyroidism, and (3) older patients with relatively little calcification. Most patients with calcific lesions will be included in the third group. Any calcification of basal ganglia detected by CT scans demands careful evaluation of calcium metabolism.
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The authors report the case of a 2-year-old girl with hepatomegaly and failure to thrive in whom the diagnosis of congenital hepatic fibrosis was first considered on the basis of the histological examination of a percutaneous liver biopsy. Further radiographic and ultrasonic investigations of the biliary tree showed a choledocal cyst and dilatation of the intrahepatic ducts. Surgical operation consisted in complete removal of the cyst with hepaticojejunostomy. The congenital intrahepatic abnormalities associated with the choledochal cyst are commented.
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In animal experiments Costall and Naylor (1977) showed that Tiaprid, a benzamide derivative of the orthopamids, inhibits dopamine receptors in the caudate nucleus and putamen but not in the mesolimbic system. Involuntary movements in patients with extrapyramidal movement disorders were reduced by 300 mg daily. Good results were obtained in 56% of 93 patients (25 in this study and 68 from the literature). On the whole the efficacy of Tiaprid seems far better than that of neuroleptics, and side effects are definitely fewer.
One hundred and twenty two cases of severe hyaline membrane disease are reported. 68 of them survived (57%). Adverse clinical, radiological and laboratory factors, and their effects on the early mortality rate, are analysed with particular reference to the referring centers, delay in admission, transport and the critical state of most infants on admission. The follow-up of 29 survivors treated before 1974 has been examined with reference to birthweight and assisted respiration. Four (30%) of the twelve infants with birthweights below 2000 g had major neurological sequelae. Only two out of the 17 babies with a birthweight over 2000 g had minor mental disturbances.
Several agarolytic Bacillus strains have been isolated. The properties agree with those described by Wieringa (1941) for Bacillus agar-exedens. These strains are the first reisolates since the original cultures were lost. A second group of isolates is related to the agarolytic B. palustris var. gelaticus of Sickles and Shaw (1934). B. agar-exedens requires carbohydrates for growth. In mineral-glucose media growth is inhibited by peptone at pH values of about 7 or less. Under alkaline conditions no inhibition by peptone is observed. A method for the enrichment of B. agar-exedens is described.
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A case of congenital chliride diarrhoea with functional ileus, early diagnosed in a premature infant of North-Africa parentage, is described. A short review is given of the clinical and biological manifestations of the disease in the newborn. Especially, the influence of parenteral therapy on the course as well as on the development of potentially fatal complications is discussed.