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PubMed1981

The cerebro-hepato-renal syndrome of Zellweger: similarity to and differentiation from the DiGeorge syndrome.

A child with the cerebro-hepato-renal syndrome of Zellweger, who was originally diagnosed as having the DiGeorge syndrome, was studied and transplanted unsuccessfully with cultured thymus. The pertinent literature is reviewed and the importance of distinguishing the two disorders emphasized. Autopsy studies reveal that transplanted cultured thymic fragments can attract lymphoid aggregates as early as 2 wk after transplantation.

Abnormalities, Multiple↗
PubMedDate not supplied

[Immune status of adult population of the Bryansk region living in territory polluted by radionuclides].

Clinical and immunological investigation with immune status evaluation of three groups of adult population of Bryansk Region was performed. The first group included 165 persons living in Vyshkov (settlement of town type in Bryansk Region) contaminated with radionuclides as a result of Chernobyl accident. The second group included 68 persons living in Vyshkov, immunological monitoring of those was performed. The third group consisted of 114 persons living on the "clean" area of Pochep (Bryansk Region). On both areas (contaminated Vyshkov and "clean" Pochep) the large percent of persons (three quarters of all investigated ones) had clinical manifestations of immune deficiency. The immune status of Vyshkov inhabitants was characterized by T-helper/inductor activation. That was expressed in significant increase of CD4+, CD4+/CD8+ in comparison of control group of primary donors and to "clean" Pochep inhabitants and in stable decrease of average values of serum IgG in comparison to control group, IgG and IgM in comparison to Pochep group. Maximum high values of T-helpers under lowest T-suppressor/killer values were observed at clinical symptoms which may be stipulated by radiation factor (loss of hair and teeth, surplus weight, predisposition to bleedings) and in persons working in cattle-breeding.

Adult↗
PubMed1994

Depressed immunity and the development of cancer.

Various disorders of immune competence are associated with increases in uncommon tumors, particularly non-Hodgkin's lymphomas (NHLs), which often exhibit unusual features: they are frequently extranodal, show a broad spectrum of lesions, ranging from benign polyclonal hyperplasia to frankly malignant monoclonal lymphomas, and are frequently localized to the brain. Of 7136 tumors in organ transplant recipients, the predominant lesions are NHLs, carcinomas of the skin and lips, carcinomas of the vulva/perineum, Kaposi's sarcoma (KS), and renal carcinomas. Skin cancers present unusual features: predominance of squamous cell carcinomas, young age of the patients, and a high incidence of multiple tumors. Cancers of the vulva/perineum occur at a much younger age than in the general population. In AIDS patients the most common malignancy is KS, which occurs in 14%, whereas NHLs afflict 2.9%. A variety of other tumors occur in these patients. The most common tumors in patients with primary immunodeficiency states are NHLs (49%), leukemias (13%), various carcinomas (9%), and Hodgkin's disease (7%).

Acquired Immunodeficiency Syndrome↗
PubMed1994

[Diagnostic imaging in immunosuppressed subjects: malignant tumors].

Each state of immunodeficiency is complicated by an increased incidence of certain types of cancer. The risk factors in the development of malignancies increase in a time-dependent relationship with the age of the defect in cell-mediated immunity and some tumors will develop at fairly distinct time intervals during immunodeficiency. Thus, Kaposi's sarcoma appears mostly when the degree of immunodeficiency is slight (average time: 23 months), non-Hodgkin's lymphoma is prevalent in an intermediate phase (average time: 37 months) and some carcinomas will appear only if immunodeficiency lasts many years. Many hypotheses have been suggested as to the biological mechanisms by which an immunity defect contributes to the development of malignancies: the most credible of them is the one suggesting the action of some oncogenic viruses which are not sufficiently controlled by the immune system. Kaposi's sarcomas and non-Hodgkin's lymphomas are the most common types of tumor in AIDS patients. Other tumors--except for Hodgkin's lymphoma--are seldom reported in HIV patients. However, the incidence of carcinomas is thought to be likely to increase in the next few years, because the longer survival of HIV patients, without strengthening their immune system, will unfortunately allow the tumors with longer onset time to develop. As for Kaposi's sarcomas and non-Hodgkin's lymphomas, the major epidemiological data are reported, together with some etiopathogenetic hypotheses and the main clinical and radiologic patterns. Other solid tumors in HIV patients and their clinical and radiologic features are reported too. Our series of cases was collected by the GICAT (Italian Cooperative Group on AIDS-related Tumors) which coordinates epidemiological studies, supports medical research and standardizes the treatment of patients suffering from HIV-related tumors.

Acquired Immunodeficiency Syndrome↗
PubMed1987

Mycobacterium avium-complex infections in normal and immunodeficient mice.

Specific pathogen-free C57Bl/6 X DBA/2 (B6D2) F1 hybrid mice were challenged aerogenically with M. avium, M. intracellulare and M. scrofulaceum and the resulting lung and spleen infections were followed over a period of several months. Growth of the mouse virulent M. avium 724 and M. intracellulare D673 was more extensive in the susceptible (Balb/c or C57Bl/6) mice than it was in the resistant A/J and B6D2 strains. Oral challenge of normal C57Bl/6 mice with virulent M. avium-complex serotypes resulted in substantial infection of the gut-associated lymphoid tissues, the lungs and spleen. No infection developed when the mice were infected orally with the avirulent MAC serotypes. T-cell depleted Balb/c (nude or Thxb) mice infected with virulent M. avium developed markedly enhanced lung and spleen infections compared to those seen in the immunocompetent controls. T-cell depletion did not potentiate the systemic growth of the avirulent MAC strains. The significance of these growth patterns (especially in the T-cell depleted mouse) is discussed in relation to the development of life-threatening M. avium-complex infections in patients with Acquired Immune Deficiency Syndrome.

Animals↗
PubMed1991

[Epstein-Barr-virus-induced lymphoproliferative diseases].

Infections with EBV are of increasing importance in pediatrics. Molecular and immunological methods have led to important advances in our understanding of the biological properties of this virus and the immunological consequences to its host. EBV-associated lymphoproliferative disorders mainly arise in immunodeficient patients by the unchecked polyclonal outgrowth of EBV-infected B-cells. On the other hand, primary genetic changes in B-cells (chromosomal translocation and oncogene deregulation) may also lead to induction of monoclonal lymphoma.

Acquired Immunodeficiency Syndrome↗
PubMed1992

Selective polysaccharide antibody deficiency in familial DiGeorge syndrome.

A family including three children with DiGeorge syndrome is described. One child died in the neonatal period from cardiac anomalies accompanying complete DiGeorge syndrome. The two surviving siblings shared a common set of pharyngeal pouch anomalies and immunodeficiency consistent with partial DiGeorge syndrome, and other morphologic anomalies characteristic of the velocardiofacial syndrome with which familial DiGeorge syndrome is associated (reviewed in reference 1). Both had normal karyotypes. Both presented with recurrent otitis media and sinopulmonary infections, CD4+ T cell lymphopenia, and defective DCH skin test responses to recall T cell antigens. Both had low serum IgM levels and IgG4 levels at the lower limits of normal. Immunization with bacterial polysaccharides resulted in impaired IgG antibody responses to the same set of antigens (H. influenzae polyribophosphate and S. pneumoniae capsular serotypes 9N and 14), while responses to protein antigens were intact. Both siblings were treated successfully with intravenous gamma globulin. The pattern of selective antibody deficiency in these patients with familial DiGeorge syndrome suggests a heritable lesion in certain regulatory antipolysaccharide CD4+ T cell subpopulations.

Adult↗
PubMed1978

[Thymus implant in a case of DiGeorge syndrome (author's transl)].

A boy with III-IV pharingeal pouch malformative syndrome, hypocalcemia and recurrent infections is presented. The immunological work-up disclosed deficiency in the specific cellular immunity with normal serum immunoglobulin levels. An adult thymus in Millipore diffusion chambers was implanted in the rectus adominalis muscle. One week later clinical and analytical data suggesting immunological reconstitution was recorded. Two years later the patient remains in good health, T and B lymphocyte percentages are normal, being the only immunological abnormality a low serum level of IgG.

DiGeorge Syndrome↗
PubMed1972

Relations between immunity and malignancy.

A higher incidence of malignancy as well as greater susceptibility to infection has been found to be associated with primary immunodeficiencies. An increased incidence of leukemia has been associated with X-linked infantile agammaglobulinemia-an isolated defect of humoral immunities. An increased frequency of a wide variety of malignancies have been found to accompany several different forms of primary immunodeficiency. Secondary immunodeficiencies produced by immunosuppressant therapy to facilitate renal transplantation have also been found to have far too much cancer to be explained by chance assocaition. Many experimental associations between immunity and malignancy have also been encountered, indicating that these two adaptive processes have an essential relationship that must be elucidated.

Agammaglobulinemia↗
PubMed1983

[Kaposi's sarcoma and opportunistic infections in young patients without antecedents liable to involve immunodepression].

The acquired immune deficiency syndrome (AIDS) is characterized by the occurrence, in a subject under 60, of Kaposi's sarcoma and/or severe opportunistic infection due to deficiency of the normal cell-mediated immune defence mechanism against the causative agents. In a number of patients, AIDS is preceded by fever, weight loss, profuse sweating, polyadenopathy or diarrhoea. In an epidemiological study conducted by a French multidisciplinary group from March to December, 1982, 25 cases were collected, including 11 cases of isolated Kaposi's sarcoma, 3 cases of Kaposi's sarcoma plus opportunistic infection, and 15 cases of single or multiple opportunistic infection. From this study and international studies, several points of interest have emerged, viz: (1) AIDS is present in France under multiple clinical aspects; (2) a focus of endemic AIDS has been discovered first in Haiti, then in Equatorial Africa; (3) the cause of AIDS is unknown, though presumed to be a virus (retrovirus?); (4) the disease is characterized by selective deficiency of T4 ("helper") lymphocytes; (5) the subjects at risk are male homosexuals, especially those with several partners, but not as much in France as in the USA; (6) none of the French patients was a drug-addict or a haemophiliac.

Acquired Immunodeficiency Syndrome↗
PubMed1981

T-cell subset analysis by monoclonal antibodies in primary immunodeficiencies.

T-cell subsets have been analysed in 23 cases of primary immunodeficiency with monoclonal antibodies. Functional assays investigating T-cell function--proliferative response to mitogens, antigens, and allogeneic cells, cytotoxicity generated against allogeneic target cells and helper function to pokeweek mitogen-induced immunoglobulin synthesis--were performed in parallel in the same patients. The results enabled us to delineate four groups of patients. The first group consisted of patients in whom marker and function studies show concordant data, with either normal or strongly decreased T-cell number and function. The second group consisted of patients in whom various degrees of functional abnormality coexist with subnormal T-cell number and increased suppressor T-cell proportion. In the third group, we collected all patients who showed functional deficiencies without marker abnormalities. Finally, there was a small group composed of patients whose T-cell pattern was strongly suggestive of abnormal differentiation.

Adenosine Deaminase↗