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Biomedical subjects

C Nezelof

Publications and source records attributed to C Nezelof.

At least 109 records · Page 6Linked to original sources
PubMed1983

Persistence of Müllerian ducts in male pseudohermaphroditism, and its relationship to cryptorchidism.

Twenty-two cases of male pseudohermaphroditism with persistence of Müllerian duct derivatives were reviewed. In 12 cases of mixed gonadal dysgenesis and five cases of dysgenetic male pseudohermaphroditism, testosterone-dependent steps of sex differentiation were also impaired, and testicular dysgenesis was prominent, even in the younger age group. The capacity of testes to inhibit the ipsilateral Müllerian duct was correlated with testicular descent: it is suggested that testicular dysgenesis explains both the functional and topographical testicular abnormalities observed in these male pseudohermaphrodites. Five other patients were cryptorchid but externally normally virilised, persistence of Müllerian derivatives representing their only abnormality of sex differentiation. Testicular structure was usually normal or showed changes attributable to long-standing cryptorchidism. It is suggested that in these patients, persistence of Müllerian derivatives is due to an inborn error of metabolism, affecting the binding of anti-Müllerian hormone to its receptor and that failure of testicular descent is caused by mechanical restraint by the abdominal Müllerian organs.

Cryptorchidism↗
PubMed1983

Infantile renal tumors associated with hypercalcemia. Characterization of intermediate-filament clusters.

Three cases of hypercalcemic infantile renal tumors were studied to identify the proteins related to their cytoplasmic 8- to 10-nm filament clusters. Immunofluorescence microscopy, using antibodies against vimentin, cytokeratin, and actin, led to the characterization of vimentin-positive filament clusters. We discuss the possibility of a mesenchymal origin for these tumors.

Cytoskeleton↗
PubMed1983

[Primary immunologic deficiencies and cancer. 5 anatomo-clinical case reports].

Five patients with primary immunodeficiency and cancer are presented. Two children with ataxia-telangiectasia developed acute lymphoblastic leukemia and malignant lymphoma of B-like origin with chromosome damage and unusual prevalence of antibodies to E.B.V. early antigen. A bone sarcoma occurred in a patient with common variable hypogammaglobulinemia. At least two infants who died with severe combined immunodeficiency had at autopsy congenital myelomonocytic leukemia and malignant lymphoma. These cases indicate the high risk for development of cancer in patients with primary abnormalities of the immune system and suggest the heterogeneity and complexity of pathogenic mechanisms.

Agammaglobulinemia↗
PubMed1983

[Histiocytosis X].

Explore the source record for details and available documents.

Histiocytosis, Langerhans-Cell↗
PubMed1983

[Recurrent fibroma of the fingers in children: an anatomo-clinical entity].

The authors report 2 cases presenting with juvenile fibromatosis of the fingers. The disease was remarkable for its relapses and the presence of intracytoplasmic eosinophilic inclusions. The cause of this original anatomo-clinical entity is discussed. No argument was found in favor of a viral origin.

Diagnosis, Differential↗
PubMed1983

[Epididymo-deferens anastomosis. Experimental study in the rat].

Fifty Wistar rats underwent right epididymo-deferens anastomosis by microsurgery, 1 to 4 weeks after ligation of the right epididymo-deferens loop. The left testis was removed in the first 32 rats. The others also underwent anastomosis but without prior ligation. Amongst the 25 rats now studied by mating, vaso-epididymography and dye injection, we have found 14 obstructions, 5 cases of permeability without fertilisation and 6 fertile and permeable animals. This series demonstrates for the first time the possibility of success of such anastomosis in the animal. They are designed to replace the classical latero-lateral anastomosis. The latter causes a spermatic granuloma by epididymal fistula. Recanalisation of the granuloma explains the frequency of postoperative oligospermia in patients with normal testes. The first results in man are very encouraging.

Animals↗
PubMed1982

An original hypercalcemic infantile renal tum or without bone metastasis: heterotransplantation to nude mice: report of two cases.

Two cases of hypercalcemic infantile renal tumor without macroscopic bone metastases are reported. Optical and ultrastructural characteristics as well as biological and clinical data are sufficiently different from those of other infantile renal tumors to justify the individualization of an original tumor entity. The hypercalcemia is not clearly understood but may be related, in one case, with a high N terminal PTH serum level. Ultrastructural elements (secretory granules) and polypeptide hormone products (glucagon) are discussed in relation with APUD cells characteristics.

Animals↗
PubMed1982

[Congenital fibromatosis. Anatomical and clinical study on three observations (author's transl)].

Three congenital fibromatosis are studied. Six years later, the subcutaneous and skeletal lesions of the first patient have disappeared with no recurrence. Regression was also observed in the second one, a generalized fibromatosis with skeletal, subcutaneous and almost certainly pulmonary and laryngeal lesions. The last case is now progressing (vertebral lesion) with a vascular invasion, the prognostic of which is unknown. A review is made about microscopical appearance, origin and difficulties in diagnosis.

Diagnosis, Differential↗
PubMed1982

Tumor cell line characterization of a malignant histiocytosis transplanted into nude mice.

The successful transplantation of a human malignant histiocytosis into nude mice allowed the examination of its atypical histiocytic cell proliferation. Histiocytic type cells were identified by positive reactions with acid phosphatase and non-specific esterase and with anti human DR or OKI1 antisera. Presence of OKT9 antigen and negative results obtained with most OKT antisera, rosettes, erythrophagocytosis and lysozyme corroborate the histiocytic immature state of the cells and preclude another type of tumor. All positive tests to prove a mature mononuclear phagocytic origin were attributable to the murine host cell reaction.

Animals↗
PubMed1982

Pyruvate kinase and phosphofructokinase isozymes in childhood cancers.

Pyruvate kinase and phosphofructokinase isozymic expression have been investigated in 62 childhood cancers by electrofocusing and immunoneutralization. Isozymic expression was roughly intermediate between that of the corresponding normal adult and fetal tissue. There were unique features distinguishing malignant from both adult and fetal tissues. In addition, phosphofructokinase and pyruvate kinase appeared to be excellent markers for nervous tissue and skeletal muscle differentiation. In neuroblastomas there was a close relationship between the differentiation level of tumors and their isozyme composition. The same phenomenon was observed in rhabdomyosarcomas and teratomas.

Adult↗
PubMed1982

[Diagnosis, prevalence, prognosis and treatment of the trophoblastic component in germinal tumours of the testis in the adult].

The authors report a series of 97 germinal tumours of the testis in the adult, studied first in terms of conventional histological data. Of 33 seminomas, 2 secreted HCG. The 5-year actuarial survival at stage I was 93%, and at stage II 75%. Stage II deaths revealed the existence of not purely seminomatous tumours. Amongst dysembryomas, half secreted HCG, with 3 histological groups: predominant choriocarcinomas, tumours with a trophoblastic component and "apparently pure" dysembryomas. The 3-year actuarial survival for dysembryomas was 90% at stage I and 58% at stage II. 51 patients of the series were studied retrospectively by sections with HCG peroxidase, a technique which reveals the intracytoplasmic synthesis of the hormone. Two types of cells have proved capable of such synthesis: syncytial cells, of syncytial-trophoblastic type, and small mononuclear cells. One third of seminomas and 90% of dysembryomas proved to have a trophoblastic component as demonstrated by HCG immunoperoxidases. All patients secreting HCG were HCG peroxidase positive. This equally applied to all patients with syncytial cells. Furthermore, all the indications are that HCG secretion is above all by the syncytial cells. From a diagnostic standpoint, any rise in beta HCG is synonymous with an HCG immunoperoxidase trophoblastic component. Detection of such a component using immunoperoxidase would seem to be essential for non-secreting tumours. From a prognostic standpoint, seminomas with a trophoblastic component are in fact dysembryomas and lymph node dissection should be performed, this being the only way of not missing a non-seminomatous metastasis. Therapeutically, pure seminomas are distinguished by the possibility of cure by radiotherapy. For all other tumours, orchidectomy must be followed by lymph node dissection. Subsequent treatment is decided on the basis of the results of the latter, with the exception of tumours with visceral metastases where chemotherapy must come first.

Adult↗
PubMed1982

Lipid synthesis and storage by histiocytes X. Study "in vitro" for eosinophilic granuloma.

Using 14C glycerol and 3H glycerol, in a study of lipid metabolism of two cases of eosinophilic granuloma of the bone, the authors point out that this precursor is preferentially but not electively incorporated by mononuclear cells. This fixation particularly concerns the big cells with numerous lipid droplets and few X bodies. The precursor is essentially involved in neutral lipid synthesis. As is the case with common macrophages, this lipid synthesis might express various states of cellular maturation and ageing.

Carbon Radioisotopes↗
PubMed1982

[Teratoma of the renal sinus. Apropos of a Case].

The authors report a new case of teratoma of the renal fossa. Such tumours are rare. The case reported is unusual since it involved an older child and because the small lesion was located in the renal sinus and not in the parenchyma.

Child↗
PubMed1981

T system immunodeficiencies in infancy and childhood.

Some problems concerning the diseases due to deficiencies of the T immune system in infancy and childhood are reviewed. The relationships between SCID and Nezelof syndrome and the pathogenesis of this group of diseases are particularly discussed.

Agammaglobulinemia↗
PubMed1981

[Cell populations of the human mandibular condyles during growth and in the adult (author's transl)].

Tumours of cartilaginous origin dominate the group of tumours of the mandibular condyles. These cartilaginous tumours consist in fact of hyperplasia of the growth zone or osteochondroma in the young adult or reawakening of the latter in the older adult in whom an occlusion problem originally is not without aetiological interest. Osteomas of the condyles are stabilised and ossified forms of such hypercondylism. Other tumours may be seen and are related to the original cell. Malignant tumours are rare.

Adolescent↗