3622. [Genetic aspects of early developmental arrest].3623. [Is the BK mole syndrome always an autosomal dominant trait?].
After a brief review of the characteristics of familial malignant melanoma, the authors summarize the literature about the BK mole syndrome. The clinical and histological features of this dermatosis are described. Three personal cases are also reported. The latter tend to demonstrate a certain genetic heterogeneity, while in the literature the BK mole syndrome has been described as an autosomal dominant trait.
3624. [Immunosuppressive therapy and Kaposi's sarcoma: two case reports (author's transl)].
The authors report 2 cases of Kaposi's sarcoma in whose immunosuppressive therapy is implicated. In a 47-year-old Italian man, cutaneous lesions were rapidly aggravated after a renal transplant and treatment with corticosteroids and azathioprine; new lesions appeared close to an arteriovenous fistula of the forearm. In a 83-year-old Algerian man, Kaposi's sarcoma appeared during steroid therapy for bullous pemphigoid. The role of acquired immunosuppression, local predisposition and genetical factors are reviewed and discussed for the pathogenesis of Kaposi's sarcoma.
3625. [Recto-colonic polyposes].3626. [Immunogenetic variants of mouse tumor cells obtained by mutagenesis].3627. [Increased sister-chromatid exchanges in fibroblasts from a del(13)-retinoblastoma patient (author's transl)].
Sister-chromatid exchanges were studied in fibroblasts from a child with del(13)-retinoblastoma. The skin biopsy was performed before the clinical onset of the tumor. The observed frequency of SCE, 19.65 per cell, was significantly increased as compared to that of a normal control, 14.68 per cell (t = 3.57, p < 0.001).
3628. [Late extra-ocular tumors in survivors of retinoblastoma].3629. [Skin manifestations of phakomatosis].3630. [46,XX,r(18), + mar karyotype in the niece of a leukemic patient with trisomy 21 and cri du chat chromosome (author's transl)].
The present observation concerns a female patient with a 47,XX, r(18), + mar karyotype. The size of the ring varies from mitosis to mitosis; generally it is very small. The presence of a small marker ("minute") may explain some phenotypic differences not usually observed in the "r(18) syndrome". This patient is the niece of a trisomic 21, who showed complex chromosome anomalies during the course of an acute leukemia from which he died.
3631. [Endocrine polyadenomatosis: progressive neurocristopathy (author's transl)].
Endocrine polyadenomatosis forms but a part of the larger group of neurocristopathy disorders. This term includes those affections due to lesions of cells, tissues, or organs derived from the neural crest. The common origin of the various neuro-endocrine cells within the neural crest suggests that there is a denominator of embryologic pathogenicity for the different polyendocrine affections. Knowledge of these is essential for early diagnosis of the different neuro-endocrine lesions, together with a systematic search for any familial associations.
3632. [Wermer's syndrome (author's transl)].
The authors present a brief report on a familial case of Wermer's syndrome, and review the principal characteristics of this "multiple endocrine neoplasm" which usually affects the parathyroids, pancreas, and anterior pituitary.
3633. [Familial form of bilateral acoustic neuroma].3634. [Increases in frequency of sister chromatid exchange following tumor grafts in different immunocompetent hosts].
作者: J L Poncy.;P Fritsch.;D Nolibe.;R Masse.;J Lafuma.
来源: C R Seances Acad Sci D. 1979年289卷13期923-6页
Measurement of sister chromatid exchanges (SCEs) frequency of inbred Rat or nu/nu Mice bone marrow cells, following tumour grafts, have been developed. Increase of SCEs was observed in hosts which present or not metases and with reduced survival rates after malignant tumour grafts. These results suggest a remote control of tumoral tissue by a diffused matter effect.
3636. [Familial cancer syndrome, colonic cancer and bone and soft tissue neoplasms].
作者: J Delamarre.;J L Dupas.;J P Capron.;A Rémond.;A Thévenin.;A Lorriaux.
来源: Gastroenterol Clin Biol. 1979年3卷10期778-9页 3637. [Apudomatosis and medullary cancer of the thyroid: histopathogenic, genetic and clinical data, based on a case of thyroid cancer with amyloid stroma].3639. [Deaf-mutism, lymphedema of the lower limbs and hematological abnormalities (acute leukemia, cytopenia) with autosomal dominant transmission].
Report of a syndrome constituted from sensorineural deaf mutism, lymphoedema of lower limbs with early onset and haematological anomalies (aucte myeloblastic leukaemia, cytopenia) in four individuals (three boys and two girls from two generations). This observation suggest autosomal dominant transmission, however recessive transmission cannot be formelly excluded.
3640. [Similarity of the HL-A antigens in parents of patients with acute leukemia or aplastic anemia].
The frequency of common HLA-A and -B antigens was determined in 30 couples with a child suffering from acute leukemia (AL), 34 couples with a child suffering from aplastic anemia (AA) and 58 random couples with healthy children. Increased frequency of couples sharing at least two common antigens was observed in parents of both AL and AA children.
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