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Biomedical subjects

M Hilgartner

Publications and source records attributed to M Hilgartner.

35 records · Page 2Linked to original sources
PubMed1983

Reversal of neutropenia with intravenous gammaglobulin in autoimmune neutropenia of infancy.

Intravenous gammaglobulin (IVIgG) was used to treat autoimmune neutropenia of infancy in two males with repeated infections. The neutrophil count increased significantly in both patients with the initial IVIgG therapy; 1 patient went into remission. The neutrophil count in the other remained above baseline for 3 wk, and a subsequent booster infusion also caused the neutrophil count to increase. The patients have remained clinically well since their treatment began. Serial studies of antineutrophil antibody and serum lysozyme, performed to elucidate the mechanism of action, suggested decreased neutrophil destruction, perhaps by Fc receptor blockade, as well as decreased synthesis of antineutrophil antibody. Neutrophil function was not impaired after the neutrophil count increased. Many patients with immune neutropenia have a benign course, but those who have significant infections could be treated, acutely or prophylactically, with intravenous gammaglobulin.

Agranulocytosis↗
PubMed1983

Transient appearance of the lupus anticoagulant in three family members sharing the A11B35DR4 haplotype.

A prolonged partial thromboplastin time in a patient was investigated, as well as those of his family members. One sibling, the mother, and the patient all shared a lupus-like anticoagulant and low Factor XII levels in association with the A 11, Bw 35, DR 4 haplotype previously found in familial lupus erythematosis. The rest of the family had normal coagulation profiles and the entire family had normal findings when tested for ANA, complement and immune complexes. After being confirmed in a second coagulation laboratory, the lupus-like anticoagulants in the three affected members of the family disappeared upon retesting 3-8 weeks later.

Adolescent↗
PubMed1981

Chelation therapy in beta-thalassemia major. III. The role of splenectomy in achieving iron balance.

Transfusion requirements for 1978 were compiled for 79 patients with thalassemia major (ages 1 to 29 years) who were maintained at hemoglobin concentrations of greater than 10 gm/dl. In 46 patients with intact spleens, the mean transfusion requirement was 258 ml/kg/year, and there was a clear increase with age. The transfusion history prior to 1978 had no influence on the increase of transfusion requirement with age. In contrast, in 33 splenectomized patients, the mean transfusion requirement was 203 ml/kg/year and it did not increase with age. Urinary iron excretion in response to deferoxamine increased with age, with no obvious difference between splenectomized and nonsplenectomized patients. The ability to achieve iron balance with a daily dose of 20 mg/kg of deferoxamine was a function of the transfusion requirement splenectomized patients with lower blood requirements generally achieved negative iron balance, whereas nonsplenectomized patients did not. We conclude that the spleen should be removed when the transfusion requirement exceeds 250 ml/kg/year, which usually occurs between 6 and 8 years of age. In young patients with intact spleens, a higher dose of deferoxamine may be use in order to prevent hemosiderosis.

Adolescent↗
PubMedDate not supplied

A human monoclonal antibody against the CD4-binding site of HIV1 gp120 exhibits potent, broadly neutralizing activity.

A human monoclonal antibody (HuMAb) against HIV1, 1125H, was isolated from an asymptomatic, seropositive haemophiliac. This antibody was specific for gp120, and its binding to gp120 was inhibited by soluble CD4, indicating that its epitope was in or near the CD4-binding site. 1125H antibody recognized a variety of divergent HIV1 strains, including most laboratory strains tested as well as some early passage isolates. Commensurate with its specificity and high apparent affinity, 1125H exhibited potent neutralizing activity against IIIB, MN, RF and SF-2 strains. The epitope recognized by 1125H was destroyed by reduction of disulphide bonds, but not by removal of N-linked sugars. Thus, the epitope was conformationally determined and did not involve carbohydrate. Data from radioimmunoprecipitation/SDS-PAGE analysis of proteolytically cleaved viral lysate further indicated that the epitope of 1125H was not affected by cleavage at the V3 loop of gp120, provided that gp120 disulphide bonds remained intact. The potential use of HuMAb 1125H in passive immunotherapy against HIV is discussed as well as the importance of including its epitope in an AIDS vaccine.

Acquired Immunodeficiency Syndrome↗
PubMedDate not supplied

Acute obstructive pancreatitis secondary to a duodenal hematoma.

An 18-year-old male with hemophilia presented with symptoms and signs of upper intestinal obstruction. Evaluation was consistent with an intramural duodenal hematoma and obstructive pancreatitis. As it is not possible to distinguish between these two disorders on a clinical basis, it is important to realize that pancreatitis may occur in such patients more often than is recognized.

Adolescent↗