3641. [Parental age in embryonic neoplasms].3642. [Acute monocytic leukemia with chromosome 11 anomalies].3643. [Bourneville's tuberose sclerosis in children (author's transl)].
In 20 out of a series of 30 children with Bourneville's disease, the diagnosis was made from the association of epilepsy and multiple achromic spots, before the appearance of adenomas. A total of 27 of these 30 children had these spots, and their characteristics are reviewed, as well as the average age of onset and frequency of the other typical cutaneous signs noted in this group: sebaceous adenomas, Koenen's tumors, and leather-grained skin plaques. Severe epilepsy was a constant finding and was often resistant to treatment: West's syndrome (18 cases), and Lennox Gastaut's syndrome (16 cases). Severe mental deficiency was also an almost constant finding and was increased by behavioural disorders: 11 out of the 13 children aged more than 5 years. Other locations of the disease included more particularly the ocular (8 cases), skeletal (11 cases), and renal (1 case) regions. In 5 cases, the disease had been transmitted by a parent, but in 3 of these it had not been recognized.
3644. [Utilization of chromosomal markers in cancer research].
The mouse is an unsuitable species for cytogenetical studies to the extent that it has 40 acrocentric chromosomes and the only criterion which could be used to differentiate them is size. We envisaged using in the case of cell grafts donors or recipients of different sex. This technique has, however, been used to a limited extent. Among the other markers which have been utilized, T6T6 of CBA mice must be mentioned. The discovery in 1966 by Léonard and Dekundt of the presence in AKR mice of fusions of the Robertson type (between chromosomes 6 and 15) has generated new interest in experimental work based on the utilisation of chromosome markers. Being interested in the mechanisms of radio-induced leukemia, the authors described how they have introduced the chromosome marker of AKR mice into the C57B1 strain which is very sensitive to the induction of radio-induced leukemias.
3645. [Bone marrow karyotype in refractory anemia with excess of myeloblasts].3646. [Cancer and DNA repair].3647. [Cowden disease: report of 8 cases in 2 families (author's transl)].
作者: P Laugier.;R Kuffer.;L Olmos.;N Hunziker.;M Rougier.;G Fiore-Donno.
来源: Ann Dermatol Venereol. 1979年106卷5期453-63页
These 8 cases of multiple hamartoma syndrome are the first reported in Switzerland. Two men aged respectively of 65 and 28 years presented a cobblestone appearance of the gingiva and of the tongue ("pebbly tongue"), which suggested Cowden disease. This diagnosis was confirmed by the discovery of numberous skin lesions, a thyroid goiter, gastrointestinal polyposis, and a mild skelettal dysmorphosis. The older patient had a grade one carcinoma of the bladder; his son and his daughter presented the syndrome, which was also discovered in the family of the second patient (his father, his brother, his sister and one of her daughters). The ultrastructural study of two skin biopsies demonstrated anomalies of the fibroblasts and collagen fibers.
3648. [Peutz-Jeghers syndrome].3649. [Contribution of cytogenetics in the study of hemopathies].3650. [Inflammatory variable epidermal naevus (atypical I.L.V.E.N.? A new entity?) (author's transl)].
The authors report a case of two sisters, having one of them left hemiatrophy, with congenital verrucose lesions, in plaques and lineary, bilateral, in extremities, presenting bouts of inflammatory extensive evolution (mostly on the atrophic side), with spontaneous resolution. Histologically the lesions match with those of Nevil's. The authors discuss the nosologic data with that of Nevil's, with epidermic nevus of Solomon and with variable erythrokeratodermis.
3651. [Cancer of the endometrium. The evaluation of high risk cases (author's transl)].
The epidemiology of cancer of the endometrium has been reviewed by going through the literature in order to find a high risk category. A particular type seems to be discernable: obestiy, endometrial hyperplasia, and the contribution of endogenous or exogenous oestrogens and lack of progesterone are the most marked elements. These factors for cancer of the endometrium are similar to those for cancer of the breast. It has been astonishing to find that these two hormone dependent cancers have a very similar background.
3652. [Intestinal polyposis (author's transl)].
Recto-colonic polyposis is typical of pre-malignant lesions. Recognition and the application of appropriate treatment offer true prophylaxis against recto-colonic carcinoma in sufferers. Careful study should be made of other family members, the only way in which latent forms may be discovered before the stage of malignant change.
3653. [A new case of acute lymphoblastic leukemia with Philadelphia chromosome in the child (author's transl)].
The systematic study of the medullary karyotype in the course of haemopathies has led us to a new case of Philadelphia chromosome in an acute lymphoblastic leukemia. This was a case of a 13 year old child. Is this a particular class of haemopathy? Do such observations put a question on the dual theory of the origins of blood germ cells? The elaborate medullary karyotype in the course of haemopathy may solve this problem. A rigorously nosological classification is essential for appraising the therapeutic plan chosen and making a pronosis.
3654. [Familial carcinoma of the colon (author's transl)].3655. [Translocation t(8;14) in acute lymphoblastic leukaemia, Burkitt type (author's transl)].
作者: R Berger.;A Bernheim.;G Flandrin.;M T Daniel.;G Schaison.;J C Brouet.;J Bernard.
来源: Nouv Presse Med. 1979年8卷3期181-3页
Two cases of infantile acute lymphoblastic leukaemia, Burkitt type, are reported. The cytogenetic study revealed a translocation t(8;14) similar to that of Burkitt's lymphoma. This chromosomal rearrangement is considered as a further argument which favours uniqueness of the two diseases.
3656. [A new variety of acute non-promyelocytic leukemia with t(15;17)].
作者: R Berger.;A Bernheim.;M T Daniel.;F Valensi.;G Flandrin.
来源: C R Seances Acad Sci D. 1979年288卷1期177-9页
Three cases of a new variety of acute leukemia have been reported. The main features were: hyperleukocytosis made of large-sized blasts with a double shaped nucleus, few or no granulations in the cytoplasm, and in a few cell faggots or unique Auer rods; mycloperoxydase reaction was positive. This feature was associated with disseminated intravascular coagulation syndrome and t(15;17)(q22;q21) translocation in the majority of mitoses.
3658. [Clinical, pathologica and genetic analysis of 53 cases of broncho-pulmonary cancer, still surviving after 5 years (author's transl)].
The authors take up again the clinical, pathological and genetic analysis of 53 bronchial cancers with a survival exceeding 5 years. The operation samples were reexamined; a H.L.A. grouping was done in every cases. On the whole, it concerned patients under 60 in good general condition; the extension of their lesions only required a limited exeresis (lobectomy). The epidermoid form predominates, the lymph node invasion is rare. Seventy four per cent of the patients carry at least one of the following antigens: W 19, A 10, B 5, BW 35. But the study did not prove the existence of a genetic factor conditioning the survival.
3659. [Multiple familial follicular hamartoma (author's transl)].
Multiple cystic and proliferative follicular lesions localized on the face and the genitalia of several members of an Italian family are described. Transmission seems to be autosomal dominant, with weak penetration and variable expressivity.
3660. [Fertility and the progeny of children surviving cancer treatment (author's transl)].
Little is known about the late effects of damage to the gonads in children treated for cancer. This investigation requires a prolonged surveillance. Radiotherapy sterilizes the ovaries above a dose of about 500 rads in an adult. Therefore, to protect at least one ovary in partial irradiation of a child's abdomen in mandatory whenever possible, either by restriction of the irradiated volume, or transposition of the ovary. Combined chemotherapies do not seem to inhibit endocrine function but it is too early to appreciate the effects of different combinations of drugs on reproductive function in girls. In boys, alkylating drugs cause an oligo or azoospermia, either temporary or permanently. The genetic risks in the descendants of children treated for cancer are still unknown.
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