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Biomedical subjects

C Nezelof

Publications and source records attributed to C Nezelof.

At least 73 records · Page 4Linked to original sources
PubMed1987

[Severe epithelio-exfoliative colitis in infants. I. Clinical data].

Two cases of colitis with neonatal onset are reported. They were characterized by their severity which made early exclusive parenteral nutrition necessary, by the lack of ulcerations visible at endoscopy and of biological inflammatory signs and by an associated neural deafness. In spite of prolonged digestive and corticosteroid treatment, a colectomy had to be performed in both cases. The specificity of the histologic abnormalities of the colon individualizes this severe colitis in infancy.

Colitis↗
PubMed1987

[Severe epithelio-exfoliative colitis in infants. Anatomical data].

Amongst the uncommon forms of congenital severe colitis, we wish to draw attention to a peculiar and probably previously never described condition that we propose calling provisionally, epithelio-exfoliative colitis. This condition appears to be characterized by the following features: its early beginning within the first weeks of life; the smooth, glossy appearance of the mucosa, without ulcerations visible to the naked eye; the prevalent degenerative changes of the epithelial cells which become vacuolated, break away prematurely from the basement membrane and finally exfoliate within the glandular lumens; the distension and rupture of the glands, the mucous contents of which intrude into the lamina propria and induce a localized, mild and non suppurative inflammatory reaction; accessory reactive traits: intense mucus production actively regenerating epithelium (high mitotic activity, syncytial cells) and increase of the cholinergic fibers within the lamina propria. Although patchily distributed, these lesions involve the colon exclusively. The cause of epithelio-exfoliative colitis is unknown. However, the ultrastructural studies and immunocytochemical investigations using anti-collagen IV, antilaminin, anti-fibronectin antibodies disclose in some glands localized thinning and rupture of the basement membrane. These data suggest a primary disorder within the molecular arrangement of either the basement membrane itself or the proteins which anchor the glandular cells to the basement membrane.

Colitis↗
PubMed1986

Cytogenetic study of cell lines from an infantile hypercalcemic renal tumor.

Four cell lines were obtained in vivo and in vitro from an infantile hypercalcemic renal tumor, which is considered to be a new tumor entity. Biochemical characteristics of the cells, studied after heterotransplantation into nude mice, were similar to those observed in the original tumor. One of the two in vitro cell lines originated from the initial tumor, the other from transplanted tumors in nude mice. One of the two in vivo cell lines originated in nude mice from serial grafts of the initial tumor, the other from grafts of the first in vitro cell line. All cell lines showed a human diploid karyotype, except for the cell line obtained directly from the tumor. In the latter, the karyotypes showed either a regional duplication of the long arm of chromosome #5 or a duplication of the long arm of chromosome #21. These two rearrangements did not appear simultaneously in the same cells, and their frequencies changed at each passage. The study of these different cell lines showed a remarkable karyotype stability, which did not prevent successful grafting into nude mice.

Animals↗
PubMed1986

Prenatal diagnosis in 200 pregnancies with a 1-in-4 risk of cystic fibrosis.

Prenatal diagnosis of cystic fibrosis was performed in 200 pregnancies with a 1-in-4 risk, and was based on significant modifications in amniotic fluid taken at 17, 18, 19 weeks of pregnancy, of six enzymatic assays: gamma-glutamyl-transpeptidase, aminopeptidase M, and alkaline phosphatase (total and isoenzymes). On the basis of normal values, normal outcome was predicted in 135 pregnancies reaching term, all the babies were normal. On the basis of significantly abnormal enzymatic values, an affected fetus was predicted in 56 pregnancies, 53 were terminated, and 3 went to term; the infants were affected. There were discrepancies in enzymatic values in nine cases, in eight cases normal outcome was predicted, six babies were normal and two were affected; in one case an affected baby was predicted, the pregnancy went to term and the baby is normal. Criteria giving evidence for cystic fibrosis in fetuses have been described: macroscopic observation of a typical meconium ileus, significant increase of albumin content in the meconium, and PAS-positive mucus-like material in some pancreatic acini. Using these criteria, diagnosis of cystic fibrosis has been confirmed in all the examined fetuses. The recurrence rate of cystic fibrosis was 22.5% in 147 diagnoses in which the index case had cystic fibrosis without a history of meconium ileus at birth, but was 47.5% when the index case had meconium ileus. The results of the study suggest that prenatal diagnosis of cystic fibrosis can be performed with an accuracy of 98%.

Amniocentesis↗
PubMed1986

Cytogenetic study of malignant histiocytosis transplanted into nude mice; presence of translocation between chromosomes 5 and 6 and a unique marker (13q+).

Fluid from a pleural effusion in a child with malignant histiocytosis was grafted into nude mice. Cytogenetic studies were performed on the xenografted cells which revealed a diploid karyotype with a translocation t (5;6) and a marker (13q+). This study reports a new rearrangement which has not been described previously in malignant histiocytosis.

Animals↗
PubMed1986

Ectopic G-29 and G-37 glucagon secretion by hypercalcemic infantile renal tumors.

Four hypercalcemic infantile renal tumors were shown to secrete glucagon-like peptides. These unusual tumors were histologically classified as rhabdoid tumors of the kidney (3 cases) and a cellular mesoblastic nephroma (1 case). Elevated G-29 and G-37 glucagon levels were detected in the plasma and tumor extracts as well as in the supernatants of cultured tumor explants. Three of these tumors were heterotransplanted into the nude mice and serially passaged from a mouse to another. The glucagon level decreased in the transplanted tumor extracts with the number of passage.

Animals↗
PubMed1986

Histiocytosis X. Purified (T6+) cells from bone granuloma produce interleukin 1 and prostaglandin E2 in culture.

We have investigated the secretory function of cell suspensions from bone eosinophilic granulomas surgically collected in two patients with histiocytosis X. Unseparated cell preparations spontaneously produced interleukin 1 (IL-1) and prostaglandin E2 (PGE2). In order to ascertain that this secretion was due to the characteristic Langerhans cell-like histiocytosis X cells predominantly found in the bone lesions, we have purified T6+ cells by the use of a fluorescence-activated cell sorter. Such highly purified cell preparations were found to secrete IL-1 and PGE2 spontaneously in culture. Stimulation with endotoxins and treatment with interferon gamma (IFN gamma) revealed an intense IL-1 secretory function of histiocytosis X cells. Since both IL-1 and PGE2 are able to induce bone resorption in vitro, our findings are compatible with the hypothesis that histiocytosis X cells are responsible for the typical osteolytic lesion observed in histiocytosis X through the local secretion of these two mediators.

Antigens, Differentiation, T-Lymphocyte↗
PubMed1986

[Specific antigens of the papillomavirus group in various intra-epithelial proliferations. Methods of detection].

An antiserum against viral particles extracted from bovine papilloma has been obtained in rabbits (New Zealand). The microscopic assay was performed by the peroxidase - anti-peroxidase method on two laryngeal papillomas, four cervical condylomas, three cutaneous papillomas, one oral condyloma, as well as on four ano-genital condylomas. A positive nuclear labelling was observed in 5 to 10% of the tumor cells localized exclusively in the superficial layers as well as in the koilocytes in these tumors. The use of this antibody in immunocytochemical studies will provide an additional tool for the investigation of mucocutaneous proliferations.

Animals↗
PubMed1986

[Cryptorchism and surgery].

Many different anatomical situations are observed by pediatric surgeons during the surgical procedure for cryptorchidism at any age. The authors propose an easy anatomical classification: type I seems to be a small trouble of the process of migration and to be more a disturbed fixation. Type III results from a big disturbed wolfian duct process. Type II is intermediary. A series of 102 cryptorchidism operated upon at the Pediatric Surgical depart. Children's Hospital Paris, were studied according to this classification and collorated with histopathological study. The correlation is significative. The authors suggest that this protocol of surgical description could be agreed by a lot of Pediatric surgeons, whatever be the age of patient at operation, in the purpose to allow a longitudinal study from initial anatomical disorders to spermogram and at last to bring a response to the unanswered question: what, when and how, a cryptorchidism has to be cured.

Child↗
PubMed1986

Tumor-like massive thymic hyperplasia in childhood: a possible defect of T-cell maturation, histological and cytoenzymatic studies of three cases.

Contrary to the transitory, enlarged thymic shadow commonly observed in babies, tumor-like Massive Thymic Hyperplasia (MTH) is seldom encountered in infancy and even more rarely in children over 4 years. We present three cases of MTH affecting 10, 5 and 11 years old girls in whom the tremendous enlargement of the thymus (3 to 4 times the normal) led to consideration of a diagnosis of a genuine tumoral process and to either biopsy or surgical removal of the thymic mass. Optical histological examination showed a perfectly normal thymic tissue and indicated that MTH was linked with a simple and homogeneous hyperplasia of the lymphoid cells. Using the dot-like acid alpha-naphtyl acetate esterase (ANAE) as a marker of mature T cells, quantitative and comparative cytoenzymatic studies revealed a definite reduction of ANAE +, mature T cells in the cortical and medullary areas in MTH (p less than or equal to 0.001). These findings suggest that MTH represents an intrathymic accumulation of immature T cells, a condition which may express a failure of cell differentiation perhaps connected with some thymic hormonal insufficiency.

Cell Differentiation↗
PubMed1986

[The Langerhans system. Definition. Physiologic and pathologic modifications].

The Langerhans cell system including the Langerhans cell (LC), the indeterminate epidermal cell, the lymphoid interdigitating cell, and the lymph veiled cell is nowadays considered as a very peculiar subpopulation of the mononuclear phagocyte system. The authors have tried in this review to point out the main salient features, the qualitative and quantitative variations of these cells during physiological, experimental and pathological processes.

Aging↗
PubMed1986

[AIDS in children. Histopathologic study of 12 cases].

The anatomo-clinical investigation of a series of 12 cases of AIDS in infants, over two years in Hôpital des Enfants Malades (Paris) allows the following observations to be made: the frequency of materno-foetal contaminations (8/12); the relative shortness of the incubation period (3-6 months); the variable degree of the atrophy of the lymphoid tissues; the absence of vascular proliferation; the interest of demonstrating viral proteins associated with LAV virus (p 18) in imprecisely identifiable cells of lymphoid tissue; the severity of the thymic alterations, which are characterized less by a lymphocyte depletion than by a loss of the maturation of the epithelial tissue, which still preserves its hormone secretory properties.

Acquired Immunodeficiency Syndrome↗
PubMed1985

Failure of histiocytosis X cells to express i blood group antigen.

Expression of HLADR, I, i blood group antigen and T6 antigen were studied in Histiocytosis X cells and pulmonary alveolar macrophages using double labelling immunofluorescence technique or immuno-peroxidase procedure. Alveolar macrophages express simultaneously HLADR and i blood group antigen. Histiocytosis X cells, characterized by HLADR and T6 antigens, and by their ultra-structural marker do not express i antigen. These results confirm the hypothesis that histiocytosis X cells constitute a specialized sub-population of the mononuclear phagocyte system.

Blood Group Antigens↗