title: [Brachmann-De Lange syndrome: Typus degenerativus amstelodamensis. Report of 5 cases and review of the literature].
abstract: Five cases with Brachmann-de Lange's syndrome are reported. The main idea is to point out the most important clinical, radiological, anatomico-pathological and genetic characteristics. The first three cases were studied at the Hospital de Especialidades of the I.M.S.S. at Peubla, Pue, while cases 4 and 5 correspond to the Hospital to de zona of the I.M.S.S., at Acapulco, Gro. With the exception of case 4, karyotype studies were carried out in the rest, including the parents, showing normal results. At present, only cases 3 and 5 are alive, since the rest died due to multiple infectious processes. In the first case, the anatomicopathological studies showed new findings, such as a diverticulum in the first portion of the duodenum with aberrant pancreas, absence of the middle lobe of the right lung and bilateral adrenocortical adenomas. Finally, pertinent literature, where it is shown that the etiology is still in darkness and that the world frequency for this syndrome reaches the figure of 353 cases.
mesh_terms: D002675:Child, Preschool; D003635:De Lange Syndrome / Q000209:etiology / Q000235:genetics / Q000473:pathology*; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male
vernacular_title: Síndrome de Brachmann-De Lange: "Typus degenerativus amstelodamensis". Comunicación de 5 casos y revisión de la leteratura.