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Biomedical subjects

J Oleske

Publications and source records attributed to J Oleske.

50 records · Page 3Linked to original sources

Human immunodeficiency virus (HIV) infection in children.

HIV infection in pediatric patients is a multisystem chronic disease that manifests as a clinical spectrum from asymptomatic infection through symptomatic infection with opportunistic infections and malignancies. The hematopoietic system is involved early in the systemic manifestations of this disease. The hematologic abnormalities seen are most probably a reflection of persistent viral infection, inflammation, and immune dysregulation, and may be complicated by secondary infections, chronic disease, drug toxicities, and nutritional deficiencies. Anemia and lymphopenia are commonly found in adult AIDS patients. Although both are also seen in pediatric patients, lymphopenia is much less common. Atypical lymphocytes with plasmacytoid characteristics have been identified in both adults and children. Pediatric bone marrow evaluation has shown an increase in plasma cells and plasmacytoid lymphocytes. Besides these findings, adult marrow findings include an increase in reticulum and lymphocytes appearing in a diffuse or aggregate pattern.

Acquired Immunodeficiency Syndrome↗

Tuberculosis as a manifestation of the acquired immunodeficiency syndrome (AIDS).

Tuberculosis has not been well documented as a complication of the acquired immunodeficiency syndrome (AIDS). We studied 48 cases of mycobacterial diseases among a group of 136 adult patients with AIDS over a 43-month period. Twenty-nine of them had severe and unusual manifestations of disease due to Mycobacterium tuberculosis, predominantly extrapulmonary and disseminated. Tuberculosis was more common among Haitians (4/8) and intravenous drug abusers (24/102) than among homosexuals who did not abuse drugs (0/22). Twelve of 21 patients with tuberculosis who were treated responded well, whereas three developed progressive disease indicative of treatment failure. Severe and unusual presentation of overwhelming tuberculosis in appropriate clinical circumstances may be considered an infection predictive of the presence of AIDS.

Acquired Immunodeficiency Syndrome↗

Natural killer cell function and interferon generation in patients with primary immunodeficiencies.

Patients with primary immunodeficiency disorders were evaluated for three aspects of natural defense: natural killer (NK) cells which lyse HSV-infected fibroblasts [NK(HSV-FS)], NK cells which lyse K562 tumor targets [NK(K562)], and interferon-alpha generation. In addition, capacity to make interferon upon challenge with other commonly used inducers was also evaluated. Most patients with severe combined immunodeficiency disease (SCID) and deficits of both T- and B-cell function demonstrated normal NK function with one or both targets. Six of eight SCID patients generated interferon-alpha at or below the lower limit of normal while only two made clearly normal levels. Six of 10 patients with Wiskott-Aldrich syndrome (WAS) had normal NK(K562) and five of 10 generated normal levels of interferon-alpha but all had severely deficient NK(HSV-FS). Patients with Bruton's agammaglobulinemia demonstrated normal NK and interferon generation, as did patients with common variable immunodeficiency, even when subdivided into patients with T-cell proliferative deficiencies and those with only hypogammaglobulinemia. Natural defense parameters may help categorize patients with SCID and WAS and help define these heterogeneous diseases.

Agammaglobulinemia↗

Suppression of immune function in growth hormone-deficient children during treatment with human growth hormone.

Inasmuch as growth hormone is known to interact with the immune system, we studied immune functions including immunoglobulins, cell surface markers, mitogen responses, and polymorphonuclear cell function in eight children with growth hormone deficiency, ages 1 to 17 years, before and during treatment with human growth hormone for 12 to 16 months. Before treatment immune functions were normal in all children. Treatment with human growth hormone did not significantly affect serum immunoglobulins, polymorphonuclear cell function, or percent T cells. However, percent B cells decreased to subnormal levels in seven of seven patients. T helper/suppressor ratios decreased in all patients, to subnormal values in seven of eight patients; and mitogen responses decreased to below normal in all. The decline of percent B cells was transient in all patients, of T helper/suppressor ratios in seven of eight, and mitogen responses in five of eight patients. In vitro incubation of lymphocytes with growth hormone resulted in no changes in cell surface markers or mitogen responses. Although the depression of immune functions resulted in no increased rate of infections during the observation period, we do not know the possible effects of prolonged treatment and therefore caution against the indiscriminate use of human growth hormone. The effects of biosynthetically obtained growth hormone on immune function remain to be determined.

Adolescent↗

Frequent detection and isolation of cytopathic retroviruses (HTLV-III) from patients with AIDS and at risk for AIDS.

Peripheral blood lymphocytes from patients with the acquired immunodeficiency syndrome (AIDS) or with signs or symptoms that frequently precede AIDS (pre-AIDS) were grown in vitro with added T-cell growth factor and assayed for the expression and release of human T-lymphotropic retroviruses (HTLV). Retroviruses belonging to the HTLV family and collectively designated HTLV-III were isolated from a total of 48 subjects including 18 of 21 patients wih pre-AIDS, three of four clinically normal mothers of juveniles with AIDS, 26 of 72 adult and juvenile patients with AIDS, and from one of 22 normal male homosexual subjects. No HTLV-III was detected in or isolated from 115 normal heterosexual subjects. The number of HTLV-III isolates reported here underestimates the true prevalence of the virus since many specimens were received in unsatisfactory condition. Other data show that serum samples from a high proportion of AIDS patients contain antibodies to HTLV-III. That these new isolates are members of the HTLV family but differ from the previous isolates known as HTLV-I and HTLV-II is indicated by their morphological, biological, and immunological characteristics. These results and those reported elsewhere in this issue suggest that HTLV-III may be the primary cause of AIDS.

Acquired Immunodeficiency Syndrome↗

Immune deficiency syndrome in children.

The present epidemic of acquired immune deficiency syndrome (AIDS) was originally described in homosexual men and subsequently in intravenous drug abusers, Haitians, and hemophiliacs. Profound defects in cell-mediated immunity (CMI) are associated with Kaposi's sarcoma and a variety of serious opportunistic infections. Recently, we and others have encountered a group of children with an otherwise unexplained immune deficiency syndrome and infections of the type found in adults with AIDS. In this report, we describe eight children from the Newark, NJ, metropolitan area born into families with recognized risks for AIDS. These patients have had recurrent febrile illnesses, failure to thrive, hypergammaglobulinemia, and depressed CMI. Four of these children have died. Our experience suggests that children living in high-risk households are susceptible to AIDS and that sexual contact, drug abuse, or exposure to blood products is not necessary for disease transmission.

Acquired Immunodeficiency Syndrome↗

Controversial techniques in allergy treatment.

The treatment of allergies is based more on sound scientific principles today than it was 20 years ago; however, allergy control still suffers from controversial diagnostic and treatment methods. Controlled studies have failed to support the skin titration (Rinkel method) used to establish an optimal dose for the relief of hay fever symptoms. Determining the clinical usefulness of cytotoxic testing is fraught with lack of clinical correlations and a high percentage of false positives. The results of double-blind studies demonstrated that cytotoxic testing is not a reliable diagnostic technique. Similarly, controlled studies do not corroborate the clinical usefulness proponents advocate for sublingual and provocative subcutaneous testing for diagnosing and treating allergy. The use of autogenous-urine immunization has no proven efficacy and can predispose the patient to potential harmful effects. These diagnostic modalities have not proved to be helpful and should not be employed in the assessment of the allergic patient.

Allergens↗

Reconstitution of normal megakaryocytopoiesis and immunologic functions in Wiskott-Aldrich syndrome by marrow transplantation following myeloablation and immunosuppression with busulfan and cyclophosphamide.

Three patients with Wiskott-Aldrich syndrome received transplants of marrow from their HLA-A, B, C, D identical siblings after myeloablation with busulfan, 2 mg/kg/day x 4 days, followed by immunosuppression with cyclophosphamide, 50 mg/kg/day x 4. Sustained engraftment of lymphoid and hematopoietic elements was documented in each case. Platelet counts in excess of 100,000/cu mm were restored 20--50 days posttransplant and remain in the normal range 6--12 mo later. Platelets exhibit normal size and in vitro aggregation. The patients produce isoagglutinins and antibodies to other polysaccharides. The use of busulfan in moderate dosages as a myeloablative agent, coupled with cyclophosphamide, may offer an improved alternative to the use of lethal total body irradiation as a preparative regimen for complete correction of Wiskott-Aldrich syndrome by marrow transplantation.

Bone Marrow Transplantation↗

Ataxia telangiectasia with thrombasthenia, platelet dysfunction, and a chromosomal translocation. A monoclonal defect?

A patient with ataxia telangiectasia presenting with a severe recurrent bleeding diathesis characterized by a prolonged bleeding time, normal platelet counts and clot retraction, absent platelet aggregation, and normal platelet factor 3 availability is described. These findings are indicative of a thrombasthenic-like pattern associated with multiple membrane receptor site defects. Chromosomal studies revealed a 14/14 tandem translocation involving chromosomal band 14q32 in peripheral T-lymphocytes; this chromosomal marker was not found in peripheral B-lymphocytes, direct bone marrow preparations, or skin fibroblasts. We postulate that the platelet functional defect demonstrated in this patient occurred in a clone of abnormal platelet stem cells possibly containing the chromosomal marker. This defect could be analogous to the situation in chronic myelogenous leukemia in which similar platelet functional disorders have been noted and in which the marker Philadelphia chromosome has been present on megakaryocytes. Our patient would also appear to be at high risk for the development of a T-cell malignancy.

Adult↗