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Titolo:
POSTMORTEM FINDINGS IN THE NIJMEGEN BREAKAGE SYNDROME
Autore:
VANDEKAA CA; WEEMAES CMR; WESSELING P; SCHAAFSMA HE; HARALDSSON A;
Indirizzi:
UNIV NIJMEGEN HOSP,DEPT PATHOL,POB 9101 6500 HB NIJMEGEN NETHERLANDS UNIV NIJMEGEN,DEPT PATHOL NIJMEGEN NETHERLANDS UNIV NIJMEGEN,DEPT PEDIAT NIJMEGEN NETHERLANDS UNIV UTRECHT,DEPT PATHOL UTRECHT NETHERLANDS
Titolo Testata:
Pediatric pathology
fascicolo: 5, volume: 14, anno: 1994,
pagine: 787 - 796
SICI:
0277-0938(1994)14:5<787:PFITNB>2.0.ZU;2-8
Fonte:
ISI
Lingua:
ENG
Soggetto:
CHROMOSOMAL INSTABILITY DISORDER; HYPERSENSITIVITY; IMMUNODEFICIENCY;
Keywords:
ATAXIA TELANGIECTASIA; AUTOPSY; CHROMOSOMAL INSTABILITY DISORDERS; HISTOPATHOLOGY; NIJMEGEN BREAKAGE SYNDROME;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Science Citation Index Expanded
Citazioni:
17
Recensione:
Indirizzi per estratti:
Citazione:
C.A. Vandekaa et al., "POSTMORTEM FINDINGS IN THE NIJMEGEN BREAKAGE SYNDROME", Pediatric pathology, 14(5), 1994, pp. 787-796

Abstract

Autopsy findings for two patients with the Nijmegen breakage syndrome(NBS) are presented. This syndrome has the same type of immunologic and cytogenetic abnormalities as ataxia telangiectasia (AT). In NBS, however, microcephaly is found and progressive cerebellar ataxia and oculocutaneous telangiectasia ale lacking. The demonstrate a clear neuropathologic difference between these two syndromes, as the diffuse cortical cerebellar degeneration characteristic of AT was absent in NBS. Inthe thymus the histologic picture was suggestive of simple simple dysplasia. Lymphoid tissues were slightly atrophic but otherwise structurally normal. In one of the two presented cases an extranodal diffuse large cell malignant non-Hodgkin lymphoma of B cell immunoblastic type was found in Waldeyer's ring, in the small and large intestines, and in the brain, whose sequelae had caused death. Six of the 19 patients known with certainty to have this syndrome have developed lymphoid malignancy, which indicates that these patients are prone to develop malignancies.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 25/11/20 alle ore 18:48:28