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3581. [Haptoglobin groups in the course of primary bronchial cancer].3582. [Disseminated cutaneous osteosis. A familial form].3583. [Retinoblastoma and interstitial deletion of 13q (author's transl)].
The authors report an observation of retinoblastoma associated with partial monosomy 13q [46, XY, t (2;10) (q21;q24), inv(9), del(13)(q13q213)] and discuss the origin of this exceptional childhood tumor by gene mutation of chromosome rearrangement.
3584. [Carcinoid tumors and syndromes].3585. [Medullary carcinoma of the thyroid gland. Apropos of 4 cases].
作者: F Demard.;J Vallicioni.;J Monticelli.;H Duplay.;M Gaillot.;G Cagnol.
来源: J Fr Otorhinolaryngol Audiophonol Chir Maxillofac. 1980年29卷8期507-11, 513-5页 3586. [Translocations t(2;8) and t(8;22) in continuous cell lines of African Burkitt's lymphoma].
Chromosome studies were performed by EBV positive African Burkitt lymphoma cell lines. Variant translocations were found in 4 cases: two with t(2;8) (p12;q23 ou 24) and two with t(8;22) (q23 or 24;q11). Identical translocations were observed in EBV positive or negative non-african lymphomas or Burkitt type leukemia. These results show the uniqueness of Burkitt lymphoma leukemia and stress the importance of chromosome 8 rearrangement in this tumor.
3587. [New complex translocation uncovering a masked Philadelphia chromosome].3588. [Dyskeratosis congenita Zinsser-Cole-Engman form. Report of two affected brothers (author's transl)].
作者: G Lorette.;M Guérois.;B Arbeille-Brassart.;M Y De Russe.;C Binet.;P Boulard.;C Carli-Basset.
来源: Ann Dermatol Venereol. 1980年107卷8-9期799-805页
Cutaneous pigmentation, lingual leukoplasia and dystrophic changes of nails are present in the two cases. The other clinical manifestations are dental alterations, epiphora, loss of dermal ridges of the pulp with hyperhidrosis, atrophic skin of the dorsum of the hands. Dysphagia and bone marrow hypoplasia are present in one case. The proband (case 1) has normal values for the following: hemoglobin electrophoresis, pyruvate kinase, marrow and blood chromosome analysis. Biopsy of pigmented skin showed an atrophic epidermis with orthokeratotic-hyperkeratosis; in the higher dermis there were several melanophores. Multiple layers of vasal lamina are seen under electron microscopy. The parents and the two daughters are free of clinical or hematologic manifestations. The mother and her two affected sons have A1-BW 27 HLA haplotype. X-linked transmission is discussed.
3589. [Acute non-lymphoblastic leukemias during Hodgkin's and non-Hodgkin's lymphomas in remission: a distinct cytogenetic entity].3590. [Recent advances in the cytogenetics of malignant hemopathies].3591. [Acoustic neuroma and tuberous sclerosis. Familial inquiry (author's transl)].3592. [Familial, malignant, chemodectoma bilateral in a 16-year-old girl].
作者: J Verhulst.;M Portmann.;P Boissiéras.;C Deminière.;J Piton.
来源: Rev Laryngol Otol Rhinol (Bord). 1980年101卷5-6期251-61页 3593. [Ultrasonography appearance of recessive hepatorenal polycystic disease in children (author's transl)].
Six cases of recessive hepatorenal polycystic disease in infants and young children were explored by intravenous urography and ultrasonography. Sufficient specific ultrasonography findings for making a diagnosis were obtained. The kidneys are large and produce strong echos, with inversion of the normal strength of medullary when compared to cortical echos, and poor definition of the renal sinus. Strong echos are also obtained from the liver, especially in the juvenile forms, because of the large amount of fibrosis. Large intrahepatic biliary ectasias were discovered in a 3-year-old child.
3594. [Sarcomatous degeneration of a familial cavernous angiomatosis (Bean's syndrome) (author's transl)].
Although the classification of "Ullman's universal angiomatosis" has not yet been completely defined, the authors estimed it interesting to report an observation which may fall into this denomination. This observation concerns a generalized cavernous angiomatosis differing from hereditary hemorrhagic telangiectasia (Rendu-Osler type) with its conventional skin and mucous membrane manifestations and its visceral forms. The patient presented cutaneous and probably digestive generalized cavernomatosis related to blue rubber bleb naevus. In connection with this observation, the authors attempt to offer reasons in support of the distinctions which appear to exist between glomangiomatosis and glomangioma. This observation is also interesting from two other standpoints: 1. the striking lineal descent: in five generations, seven subjects presented identical angiomatoses; 2. the malignant transformation (angiosarcoma) of one of the cavernous elements.
3595. [Results of a survey of the risk factors involved in the incidence of female breast cancer (author's transl)].
A survey of the risk factors involved in the incidence of female breast cancer was conducted in the Hauts-de-Seine and Yvelines departments, near Paris. Two hundred and fifty thousand questionnaires were sent out; 18000 women replied. The family risk factor appeared in 15% of the replies, including 6% which concerned the closest relatives (mother and/or sister). Correlations are established between the various factors, and conclusions are drawn about the possibility of cancer detection according to graded risks.
3596. [Retinoblastoma in 2 twin sisters].3597. [Very long survivals of chronic granulocytic leukemia (author's transl)].
作者: J Debray.;Y Pommier.;A A Audebert.;M Krulik.;N Smadja.;D Zylberait.
来源: Sem Hop. 1980年56卷7-8期309-13页
Following busulfan induced bone marrow insufficiency, a patient witha chronic granulocytic leukemia (CGL) has had a survival lasting 18.5 years. During remission, chromosome studies on bone marrow have not shown Philadelphia chromosome (Ph 1). There was no correlation between this observation and initial pronostic factors from literature. Analysis of 14 cases (13 from literature) of prolonged survival showed that Ph 1 was absent in 4 cases when only 15% of CGL are initially Ph 1 negative. Therapeuticcaryoconversions were probable. Recently attempts have been made to eradicate the abnormal Ph 1 positive clone. Some hopeful results are reported.
3598. [Acute granulocytic leukaemia: prognostic value of medullary caryotype (author's transl)].
Thirty-nine cases of granulocytic acute leukaemia (AL) forming an homogeneous series were the object of a satisfactory chromosomal examination of the marrow by direct examination at the time of diagnosis. This group of patients was compared with another group of similar number where the conditions of patient referral, diagnosis and treatment were the same, in order to show that no selection was involved in the series studied. Medullary caryotype was found to be normal in 61,5% of cases. In such patients, the hope of complete remission and mean survival were significantly better than in cases where the caryotype was abnormal, and all the more so when the caryotype contained abnormalities in all the mitoses examined. For myeloblastic AL, the correlation between caryotype and the effects of treatment is very close (p less than 0,01). For the therapist, these results justify a new approach in the treatment of myeloblastic AL.
3599. [Triple and quadruple cancers (author's transl)].
The authors report two cases of multiple cancers (three and four) and precise the criterions of multiplicity of cancers, their frequency and their localizations. The factors incriminated in multiple carcinogenesis, often not well known and complicated, are also studied, such as family ground, age, blood groups, hormones, viruses, immunologic deficiencies and some carcinogenic drugs. Moreover, the actual progress in therapy, leading to the healing of the first tumor, may favour the secondary supervening of one or more cancers, as the survival is longer.
3600. [Pathogenic characterization of Burkitt's lymphoma in France. What is the problem? How to resolve it? Preliminary results. Role of cytogenetic abnormalities].
作者: T Philip.;G M Lenoir.;M Brunat-Mentigny.;S Bertrand.;O Gentilhomme.;G Souillet.;N Philippe.
来源: Pediatrie. 1980年35卷8期659-76页 |