On the basis of the 6 cases reported here and scattered published cases, the existence of a childhood chronic immunoblastic lymphadenopathy syndrome is proposed. It is characterized by the following features: A systemic immunoblastic proliferation with varying degrees of maturation resembling the B lymphocyte hyperimmune disorder observed in angioimmunoblastic lymphadenopathy but with no deposits of interstitial amorphous material or vascular proliferation. Disseminated superficial and deep lymphadenopathy. Chronic, pronounced splenomegaly. A constant thrombocytopenia. A polyclonal hypergammaglobulinemia, with markedly elevated antibody titers to various agents. An early onset and a course of several years (up to 20), interrupted in half the cases by the occurrence of a virus-associated (EBV, Papova) neoplastic process or a fatal viral infection. Low natural killer (NK) cell activity in 2 cases.

B-Lymphocytes↗

PubMedDate not supplied

Lymphohistiocytosis: a multi-factorial syndrome of macrophagic activation clinico-pathological study of 38 cases.

Lymphohistiocytosis (LH) is defined by a widespread infiltrate of non-malignant lymphocytes and macrophages, involving principally the liver, spleen and central nervous system and associated with a severe lymphoid atrophy. For this histological study, 38 juvenile cases of LH were selected and reviewed. Morphological and histochemical studies revealed that the macrophages were activated. They appeared to be OKM1, OKT9 and HLA Dr positive and showed a patent or occult erythro and thrombocytophagocytosis. Comparatively, most of the lymphoid cells did not bear any immunoglobulin and did not label with a wide panel of monoclonal antibodies including T3, T4, T6, T8, T11. The review of the clinical and biological data of these 38 cases suggests that LH does not represent a single entity but can be regarded as a non-specific response to various causes. Three different conditions associated with LH can be isolated. The first group consists of patients in whom the disease is characterized by an early onset, high familial incidence and an inevitably fatal course. This category contains the largest number of cases (29 out of 38) and corresponds to the classic description of Familial Erythrophagocytic Reticulosis by Farquhar and Claireaux. The second group includes mainly male patients and children over 2 years old. Inheritance is not a predominant feature. The course of the disease is comparatively long. A viral infection was present in three of the seven cases. This category shares many of the clinico-pathological features of the Virus Hemophagocytic Syndrome and is presumably related to a chronic and latent viral infection. A third group can be identified on the basis of the presence of pigmentation abnormalities and immunodeficiency disorders probably associated with cytoskeletal abnormalities. This group includes Chediak-Higashi disease and Griscelli's disease. The fact that some genetic factors and immunodeficiency disorders are present in these three groups is probably significant and suggests that LH is a condition brought about by multiple factors--of which the common denominator is an apparent activation of the Mononuclear Phagocytic System.

Biopsy↗

PubMedDate not supplied

Nucleolar organizer regions in congenital mesoblastic nephroma.

A review of 78 renal tumors in patients under 6 months of age revealed 35 congenital mesoblastic nephromas (CMNs). Based on cellular criteria, 14 were classified as classical, 4 as partly cellular, and 17 as cellular CMN. The mean ages were 24, 11, and 70 days, respectively. There were 13 intrarenal tumors (stage I) but 9 classical, 3 partly cellular, and 5 cellular CMNs extended to the perirenal fat (stage II) and 5 cellular tumors ruptured (stage III). In order to assess cellular proliferative activity, silver staining of nucleolar organizer region (Ag-NOR) proteins was performed on 19 CMNs. The number of Ag-NOR dots per cell was significantly lower in classical and partly cellular CMN than in cellular CMN, whatever the stage (P < .01). Within the cellular CMNs, the mean number of Ag-NOR dots was statistically higher in the single case that recurred with fatal outcome (P < .02). Counting of Ag-NOR dots appears to be a reproducible method for evaluating the biologic potential of CMNs. The number of Ag-NOR dots, DNA content measurements, the histological subclassification, and the presence or absence of tumor at the surgical margins may be useful features in selecting those patients who will benefit from further treatment after nephrectomy.

Female↗

PubMedDate not supplied

[Familial granulomatous disease: histopathological and histogentic data].

The lesions seen in chronic familial granulomatosis are variable and seen in varied combinations: granulomas, often disseminated, pseudotuberculous lesions, histiocytes with a high content in lipofuscins seen in the liver, lymph nodes, spleen and lungs in particular. These lesions may be explained at least in part by a congenital defect in leucocytes, which is peculiar to this hereditary disease, and which concernes bactericidal functions.

Granulomatous Disease, Chronic↗

PubMedDate not supplied

[Familial syndrome combining short small intestine, intestinal malrotation, pyloric hypertrophy and brain malformation. 3 anatomoclinical case reports].

Anatomoclinical study of 3 cases of an exceptional malformative condition characterized by: --extreme shortness of the small intestine, --mesenterium commune, --hypertrophic pylorus, --malformation of the central nervous system (heterotopia, absence of operculum temporale). Clinically this malformative condition is characterized by failure and inertia of the intestinal peristalsis producing at intervals of 10-15 days episodes of subocclusion, the repetition of which causes death. The syndrome is familial and seems to be of autosomal recessive inheritance. The absence of mechanical obstruction, the repeated failure of colostomy and ileostomy, the normal aspect of the myenteric plexuses verified by cytoenzymatic and silver stains allow to individualize this anatomoclinical syndrome and to rule out the hypothesis of Hirschsprung's disease, Chagas' disease, idiopathic megacolon or hypoplasia of the myenteric plexuses. The association of cerebral malformations leads to consider the responsibility of a lack of synthesis of a same specific intermediate factor which is up to now poorly determined, implicated in the neuronal migration and neuromuscular transmission.

Abnormalities, Multiple↗

PubMedDate not supplied

[Neuroblastoma in children. Anatomoclinical study of 46 cases].

The authors stress the difficulty of diagnosis of a neuroblastoma in its undifferentiated when the demonstration of nervous differentiation is necessary. The factors which may play a role in the prognosis of these tumours are analysed. In this series are included the age of the child, the location of the tumour, the existence of certain early metastases (bone), the possibility of complete surgical removal and alterations in the tumour secondary to chemotherapy and radiotherapy. Histoenzymatic study of histio and organotypic cultures as well as chromosomal analysis were possible in a number of cases.

Abdominal Neoplasms↗

PubMedDate not supplied

[Hepatic Chiari syndrome in a newborn].

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Hepatic Veins↗

PubMedDate not supplied

[Idiopathic chronic idiopathic type, Dubin-Johnson type].

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Child↗