Successful treatment of chronic bullous dermatosis of childhood with colchicine.
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Biomedical subjects
Publications and source records attributed to A Zeharia.
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The growth charts of 15 patients with anorexia nervosa during the prepubertal and pubertal period were carefully reconstructed. All 15 (13 females, 2 males) had been suffering from anorexia nervosa for at least 6 months prior to referral. Mean age at referral was 13.3 +/- 1.3 years and mean duration of anorectic symptoms was 17 +/- 8 months. In all 15 cases growth arrest had been present for 13 +/- 8.5 months prior to admission. During the follow-up period, catch-up growth to different degrees was observed in the 13 patients (11 females, 2 males) who remained under treatment for at least 1 year. On the assumption that stunting of growth during the prepubertal and pubertal period is a very frequent, if not a constant, sign of anorexia nervosa of 6 months duration or more, it could be considered an additional criterion for diagnosis of the disease. The projected height should be included in the calculation of ideal weight. Since patients with growth arrest are usually referred first to a general pediatrician or a pediatric endocrinologist, it is recommended that a detailed history of caloric intake, as well as the attitude of the patient to food, be obtained in each case in order to confirm the diagnosis of anorexia nervosa.
3-Methylglutaconic aciduria has been described in two distinct syndromes. In one there was deficient 3-methylglutaconyl coenzyme A hydratase in fibroblast extracts where the only clinical manifestation was retarded speech development. In the second syndrome, the enzyme activity was normal but prominent neurological deterioration was noted. We describe two siblings with 3-methylglutaconic aciduria with normal enzyme activity who had choreoathetoid movements, optic atrophy, and mild developmental delay. The boy demonstrated developmental improvement in his second year of life, and his sister developed well, with normal school performance. These patients represent a new clinical variant of the second syndrome with a relatively favorable prognosis.
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A 22-month-old girl with the typical manifestations of Alagille syndrome presented with acute right hemiparesis. Cerebral angiographic studies demonstrated the presence of complete occlusion of both internal carotid arteries with the formation of a collateral network of vessels compatible with the diagnosis of Moyamoya disease. This rare association has not been reported previously.
The 24-h secretory pattern of hGH was studied by the aid of a continuous blood withdrawal pump in three Laron-type dwarfism (LTD) patients and compared with that in sex- and age-matched normal control subjects. It was found that the secretion of hGH was enhanced in the LTD patients, but in all three the diurnal secretory profile, as expressed by the number of pulses and the sleep-related maximal pulse, was preserved. In the younger LTD patients, women aged 19 and 21 years, the number of pulses was 9 and 7 (compared with 6 in the control subjects), the maximal hGH pulse amplitude was 164 and 280 ng/ml (compared with 135 ng/ml), the area under the curve (AUC) was 560 and 780 (compared with 268), and the average integrated concentration (A-IC) of hGH was 33.9 and 23.4 ng/ml (compared with 11.3). In the older LTD patient, a man aged 27 years, the hGH secretion was lower than that in the LTD women, but still higher than that in normal matched controls: the number of pulses was 4 (control 2), maximal pulse amplitude 67 ng/ml (control 19), AUC 231 (control 51), and A-IC 9.9 ng/ml (control 2.2). The decline in hGH secretion, which is characteristic of advancing age in normal subjects, seems to occur in LTD as well. Metabolic clearance rate (MCR) and production rate (PR) of hGH were measured in the 19-year-old female LTD patient. Her MCR was 73.4 ml/m2 per min, and her PR was 2,480 ng/ml compared with 158.3 and 839, respectively, in a normal subject. The exaggerated PR explains the elevation of plasma hGH in this syndrome and is probably the result of a lack of negative feedback due to insulin-like growth factor deficiency.
Two infants developed hyperkalemia shortly after cessation of prolonged ACTH therapy for infantile spasms. We wish to call for cautious approach at time of cessation of prolonged ACTH therapy because of possible unexpected and only partially understood hazardous side effects such as hyperkalemia.
Between July 1983 and February 1984, eight children with adenovirus Type 3 infection, proven by virus isolation from sputum, stool or nasopharyngeal swabs and a fourfold increase in complement fixation antibody titers against the virus, were treated in our department. All eight patients had fever lasting at least 7 days, hepatomegaly, diffuse pulmonary infiltrates and abnormal liver function tests. Seven of the patients exhibited dyspnea and pulmonary wheezing. Six of the patients developed changes in state of consciousness, and three had repeated convulsions. EEG patterns in three of the patients were compatible with encephalopathy. Other clinical manifestations included: follicular tonsillitis in two patients, diarrhea in two, pneumothorax in one, and shock with disseminated intravascular coagulation in one. The spectrum of adenovirus Type 3 infection reported here has been described previously only in the viral hemorrhagic fevers. This adenovirus Type 3 infection shares the potential for disseminated disease that has been described previously for Type 7, simulating Reye's syndrome.
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Thirty-four infants and children were prescribed bifonazole 1% shampoo as a treatment for lesions of scalp seborrheic dermatitis. All but one were cured or showed improvement within a short period. No serious side effects were observed. We conclude that bifonazole 1% shampoo is an effective and safe treatment for lesions of scalp seborrheic dermatitis in infants and young children.