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C Nezelof

Publications and source records attributed to C Nezelof.

At least 55 records · Page 3Linked to original sources
PubMed1989

Congenital ciliary aplasia in two siblings. A primitive disregulation of ciliogenesis?

Congenital ciliary aplasia was demonstrated in two siblings with clinical history of primary ciliary dyskinesia. Ultrastructural histochemistry of successive bronchial biopsies revealed the predominance of immature mucous cells and the total absence of ciliated or preciliated cells in the respiratory epithelium. This original disorder may represent a unique variant of primary ciliary dyskinesia with primitive disregulation of ciliogenesis.

Biopsy↗
PubMed1989

Lymphadenopathy in renal transplant patients treated with immunosuppressive antibodies (OKT3 and anti-thymocyte globulin). A report of nine cases.

A clinicopathological study of nine patients who developed systemic lymphadenopathy following renal transplantation and immunosuppressive therapy (OKT3 and anti-thymocyte globulin) showed a rapidity of onset and disappearance of lymphadenopathy (nine of nine cases), a frequent association of systemic signs (nine of nine cases), and a heterogeneity of histological patterns ranging from diffuse lymphoid hyperplasia to one incorrectly considered to be immunoblastic lymphoma. The coexpression of both light chains was useful in the exclusion of the diagnosis of B lymphoma. These posttransplant lymphoproliferative syndromes seem to represent an allergic reaction to the introduction of foreign protein resembling serum sickness rather than a viral infection favored by immunosuppression, although in one case (with pseudolymphomatous features) a virus was the likely mechanism.

Adolescent↗
PubMed1989

The disappearance of germinal centers in chronic lymphadeno-hepato-splenomegaly syndrome in childhood: report of three cases.

Three cases of a syndrome featuring massive splenomegaly, gross generalized lymphadenopathy, and moderate hepatomegaly are reported. Spleen weights ranged from 800 to 2400 g. Gradual depletion of lymphoid germinal centers, and prominent infiltration of the splenic and lymph node cords with plasma cells, immunoblasts and actively dividing B cells were the most distinctive histological features. The liver in two cases showed portal infiltrates. A marked hypergammaglobulinemia, a decrease in blood cholesterol level and hematological abnormalities related to hypersplenism were observed. The condition begins early in life and runs a chronic course, of up to 25 years. There was a family history in only one instance. Since there was no generalized immunodeficiency nor local depletion of T cells or dendritic reticulum cells, a failure in the local regulation of the immune response and possible cytokine production is postulated. This condition underlines the pivotal role of the local organization of the germinal centers in cellular cooperation and in the carrying out and regulation of the immune response.

Child↗
PubMed1989

Primary intestinal myopathy, a cause of chronic idiopathic intestinal pseudoobstruction syndrome (CIPS): clinicopathological studies of seven cases in children.

Clinicopathological data on seven instances of primary intestinal myopathy in children are reported. The ages of the patients ranged from eleven months to thirteen years. A persistent intestinal obstruction was the main and constant clinical feature. An ineffective intestinal propulsion was documented on manometric studies. Various urological abnormalities were present in three cases. One patient died and six survive but are dependent on enteral and parenteral nutrition. The morphological findings consisted of degenerative changes involving the muscular layers of the intestinal wall. These changes varied from cytoplasmic vacuolation to definite atrophy and disappearance of the muscular fibers. An extensive interstitial fibrosis underlined these atrophic changes in the late stages of the disease. A familial history was identified in three cases, one consistent with an autosomal dominant transmission.

Child↗
PubMed1989

Solitary intestinal fibromatosis: a rare cause of neonatal obstruction.

Solitary intestinal fibromatosis (SIF) is an unusual cause of neonatal intestinal obstruction. A fibromatosis located in the first part of the duodenum, close to the pylorus, was associated with a focus of heterotopic pancreas and mimicked congenital pyloric stenosis. It illustrates the difficulties encountered in the diagnosis and surgical management of this condition.

Child↗
PubMed1989

[T lymphoma of the nasopharynx, a rare entity. Apropos of a case].

We report one case of nasal T-cell lymphoma (NTL) that we studied histologically and with a panel of monoclonal antibodies. It is a rare etiology of the so-called lethal midline granuloma. The diagnosis of NTL if often difficult because of spreading necrosis, cellular polymorphism and necessity of getting frozen snap sections for immunohistochemical techniques. We describe histological, immunohistochemical features of NTL and its usual follow up. Then we compare them with those of malignant lymphomas of the Waldeyer's ring which are very different although they are located very near to NTL. These differences raise the hypothesis of a possible role of the local environment.

Aged↗
PubMed1989

[Anatomoclinical approach in idiopathic pulmonary hemosiderosis. Apropos of 12 cases].

Idiopathic pulmonary hemosiderosis (IPH) is an infrequent condition whose severe and unpredictable prognosis justifies extensive etiologic investigations. We have assembled 12 cases of IPH (nine children), three adults). In seven patients, pulmonary bleeding was demonstrated upon bronchoalveolar bleeding was demonstrated upon bronchoalveolar lavage (BAL) that recovered numerous hemosiderin-laden alveolar macrophages (Golde index: 247 +/- 53). All 12 patients underwent a surgical lung biopsy. Light microscopy studies showed hemosiderosis, often with lymphoid hyperplasia (n = 11), and occasionally with large germinal centers (n = 4), interstitial mastocytes (n = 7), ferric tattoo of the elastic network (n = 4), and a variable degree of interstitial necrosis (n = 4). Ultrastructural studies were performed in six cases and showed swelling of capillary endothelial cells (n = 5), interruptions in the endothelium (n = 3), tattoo of basement membranes (BMs) and elastic tissue (n = 3), intracapillary platelet aggregates (n = 2), and focal thickening of capillary BMs (n = 2). Four biopsies were studied using immunofluorescence (IF): no deposits of immune complexes were found. Indirect IF assays for antiglomerular and alveolar BMs was performed in two patients and was negative in both.

Adolescent↗
PubMed1988

PTH mRNA transcription analysis in infantile tumors associated with hypercalcemia.

The ability of infantile hypercalcemic tumors (three rhabdoid renal tumors, one cellular mesoblastic nephroma, and one hepatoblastoma) to produce parathyroid hormone (PTH) was tested using RNA-DNA hybridization. Results were compared with those obtained in one lung epidermoid carcinoma and one parathyroid adenoma from adult patients. Elevated plasma immunoreactive PTH (iPTH) concentrations were observed in three of five children. The only tumor in which PTH-RNA hybridization could be detected was the parathyroid adenoma. The integrity of the RNA preparations was further confirmed by positive hybridization obtained with a glucagon DNA probe in both normal pancreas and the rhabdoid tumors. Quantitative bone histomorphometry of tumor-bearing nude mice showed a reduction in bone formation and increased bone resorption, the opposite of what occurs in hyperparathyroidism. The PTH-like protein, which was detected by radioimmunoassays (RIA) in the sera of three patients, could not be correlated with tumor PTH mRNA transcription within the limits of our assays. In order to explain this discrepancy, we suggest that the tumors produce a factor (not PTH) which, in turn, elicits the excess iPTH which we detected by RIA.

Aged↗
PubMed1988

[Intestinal transplantation in children].

A long period of experimental work has allowed to study successively hypothermic preservation of the small bowel, autotransplantation and finally allotransplantation. After this initial phase of experimental surgery, a certain number of intestinal transplantations in humans could be attempted as cyclosporine became available. Following a brief of report of 3 intestinal transplantations recently performed by the group of the hôpital des Enfants-Malades in Paris, indications for intestinal transplantation are provided. Subtotal resections of the small bowel in children or adolescents and extensive resections in neonates account for most of the indications. Despite significant progress, further advances in intestinal transplantation need to be made, because the small bowel poses unique problems in that it seems to represent a compendium of all the particularities and difficulties of other organ transplantations.

Animals↗
PubMed1987

Omenn's syndrome--pathologic arguments in favor of a graft versus host pathogenesis: a report of nine cases.

Histologic, histochemical, and histoenzymatic investigations of nine cases of Omenn's disease showed generalized lymphoid depletion, including B cells and all T-cell subpopulations; an apparent proliferation of alpha-naphthyl acetate esterase-, acid phosphatase-, OKM1-positive macrophages and T6 interdigitating cells; a thymic hypoplasia with arrest of hassallian epithelial maturation; starlike fibrinous deposits in the bone marrow; and extensive cutaneous lesions characterized by hyperkeratosis, apoptotic cell death associated with the intraepidermal presence of T4+ and T8+ cells, localized necrosis of the basement membrane, expression of Ia antigens by malpighian cells, and progressive loss of the T6+ Langerhans' cells. These lesions, mainly the skin and bone marrow changes, are reminiscent of those observed in acute graft versus host reaction. Although a blood chimerism has never been demonstrated, these pathologic observations support the hypothesis of graft versus host disease in a primary cellular immunodeficiency and the persistence of the proliferating maternal cells in the peripheral target organs.

Bone Marrow↗
PubMed1987

Histiocytes X and X body reactivity with concanavalin A, peanut agglutinin and BSPT.

The ultrastructural and histochemical exploration of histiocytes X done by the means of Concanavalin A (Con A), peanut agglutinin (PNA) and BSPT demonstrate that the plasma membrane of histiocytes X shared some properties with all the other cells and is also specialized. The rod part of the X body has the same properties as the plasma membrane while its vesicular part differs and is closer to the inner membrane system of the cell. In consequence it is suggested that the rod part of the X body is a specialized pathway or shuttle for receptor linked glycoprotein exchanges in highly specialized cells.

Benzothiazoles↗
PubMed1987

Is true hermaphroditism a primary germ cell disorder?

Eleven cases of true hermaphroditism, 6 raised as female and 5 as male, were seen in the 20-year period from 1965 to 1985. External genitalia varied from Prader types II to IV. The ovotestis (11/22) was the most common gonad, and a marked variation in macroscopic and microscopic appearances with five patterns of distribution of gonadal tissue was noted. Ovulation was observed in ovarian tissue, and spermatogonia, but no spermatozoa, in testicular tubules. A hypothesis is developed suggesting abnormal testicular differentiation, and a model is presented to explain observed patterns of gonadal tissue distribution. A uterus was present in all cases, but a hemiuterus or lateralized uterus was noted in the 5 cases in which only testicular or predominantly testicular tissue was observed on the contralateral side. Karyotype analysis showed 46 XX in 5 cases (1 with an H-Y antigen) and a mosaic involving 46 XY in the remaining 5. Spontaneous puberty was achieved in 3 of the females, and of the 4 males with residual testicular tissue, all are prepubertal.

Disorders of Sex Development↗