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Biomedical subjects

A Valencia

Publications and source records attributed to A Valencia.

134 records · Page 8Linked to original sources
PubMed1979

Mutations affecting pigmentation in man: I. Neuroectodermal melanolysosomal disease.

We describe a syndrome identified in three consanguineous families who had two and probably four common ancestors five generations ago. The syndrome is characterized by profound dysfunction of the central nervous system, silver-leaden colored hair, abnormal melanosomes and melanocytes, and abnormal inclusion bodies in fibroblasts, bone marrow histiocytes and lymphocytes which appear to represent abnormal lysosomal bodies. Because of the biochemical relationships between melanin-melanosomes and neuromelanin, we think that all the manifestations of the condition are related to and represent pleiotropic effects of a newly identified gene in man in its homozygous state. Biochemical reactions of the cells of these patients indicate presence of tyrosinase in the melanosomes.and show that the substance accumulated in cultured fibroblasts and in the bone marrow histiocytes is a PAS and Oil-red-O positive material but is Oil-red-O negative after extraction; it has the typical reactions of melanin withe the Masson and Fontana stain, but cannot be considered typical melanin, since without stain it is colorless. The ultrastructural studies showed round granules with variable matrix, similar in fibroblast and bone marrow, and with variable intensity of reaction to osmium. This mutation principally affects the neuroectoderm, but also the mesoderm.

Child, Preschool↗
PubMedDate not supplied

Necrotizing soft tissue lesions after a volcanic cataclysm.

A volcanic cataclysm of major proportions, the fourth largest in terms of total casualties in the history of mankind, wiped out the town of Armero, Colombia, in 1985 resulting in over 23,000 deaths and 4,500 wounded. Among the hundreds of survivors who were transferred to hospitals in the capital city of Bogotá, there was as overwhelming number who developed necrotizing fasciitis. These patients constitute, perhaps, the single largest group of this type of lesions in the recorded literature. Thirty-eight patients with well established necrotizing fasciitis were identified at 4 selected hospitals in Bogotá; 8 of them presented with zygomycetic infection (mucormycosis), a highly lethal entity. Many additional cases were treated at other hospitals in Bogotá and several cities in Colombia. The main clinical features of these 38 patients affected by necrotizing fasciitis are reviewed, with special emphasis on the patients with mucormycosis. Patients with necrotizing fasciitis had an overall mortality rate of 47.7%; patients with mucormycosis, 80%. A plea is made for an early diagnosis, utilizing tissue sampling and microbiological studies, so that prompt and radical treatment can be instituted. This is especially pertinent in situations of natural disasters resulting in massive numbers of casualties and seriously injured survivors.

Amputation, Surgical↗
PubMedDate not supplied

[Systemic-psychodynamic model for family evaluation].

In this paper a family evaluation instrument called systemic-psychodynamic family evaluation model is described. Also, the second stage of the validation study of this instrument is presented (which deals with the inter-observers variation). Twenty families were studied. They were assessed always by the same interviewers designated as experts. They are all family therapy specialists and their assessment was used as the evaluation reference standard or "gold standard". The observers were psychiatrists without previous training in family therapy. For the purpose of the interview, both experts and observers were blind to the medical diagnosis of the patients. During the first stage of the validation study the observers did not have a reference guide which resulted in a low concordance rating. For the second stage, a 177 item guide was used and a considerable increase in the concordance rating was observed. Validation studies like the one used here are of considerable value to increase the reliability and further utilisation of evaluation instruments of this type.

Evaluation Studies as Topic↗
PubMedDate not supplied

[Double-blind clinical trial comparing 5 days of dirithromycin versus 7 days of diacetylmidecamycin in acute bronchitis and acute exacerbations of chronic bronchitis].

BACKGROUND: Dirythromycin has several pharmacokinetic characteristics (long half life and high tissue concentrations) which suggest the possibility of administering shorter treatments than those conventionally used. The aim of this study was to determine and compare the efficacy of a 5 day treatment with dirythromycin once a day, versus diacetylmidecamycin twice a day over 7 days in the treatment of patients with acute bronchitis and acute exacerbations of chronic bronchitis. METHODS: A parallel, multicentric, randomized, double blind clinical study was carried out in 8 Spanish hospitals. RESULTS: One hundred seventy-four patients were included in the study, with 87 (80 evaluable) being randomly assigned to receive dirythromycin (500 mg/day over 5 days) and 87 (83 evaluable) diacetylmidecamycin (600 mg, twice daily over 7 days). A favorable symptomatic response (cure or improvement) was observed in 72/80 of the first group (90%) and in 74/83 (89.2%) of the second group. No statistically significant differences were found in the efficacy and safety between the two treatment groups in either the evaluable patients or on intention to treat analysis. CONCLUSIONS: The results of this study suggest that the administration of dirythromycin, once a day over 5 days, is as safe and effective as diacetylmidecamycin, twice a day over 7 days, in the treatment of acute bronchitis and acute exacerbations of chronic bronchitis.

Acute Disease↗