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Biomedical subjects

M W Hilgartner

Publications and source records attributed to M W Hilgartner.

At least 91 records · Page 5Linked to original sources
PubMed1985

Total knee arthroplasty in hemophilia.

Twenty-four total knee arthroplasties were performed in fourteen disabled patients with hemophilia. The average age of the patients at operation was thirty-five years. Twenty-one of the implants that were used were total condylar prostheses. Using The Hospital for Special Surgery knee-rating system after two to nine years of follow-up, the result in fifteen knees was rated as excellent; in six, as good; and in one, as fair. Two patients had a poor result that was attributable to late infection. Pain and function were markedly improved, and the average gain in range of motion was 23 degrees. Postoperative complications, in addition to the infections, included one subcutaneous hematoma, one hemolytic anemia, and one instance of inhibition to Factor VIII. The technical problems in treatment were formidable. Total knee arthroplasty in a hemophiliac can be successful, but it should be performed only with strict hematological supervision. The surgeon should be prepared to treat many potential postoperative complications.

Adult↗
PubMed1985

Long-term follow-up of hemophiliacs with lymphocytopenia or thrombocytopenia.

Immunologic abnormalities resembling those seen in patients with the acquired immunodeficiency syndrome (AIDS) are frequently observed in multitransfused but otherwise healthy individuals with hemophilia. To determine whether there was clinical or laboratory evidence to suggest an abnormality of immunoregulation in persons with hemophilia before the recognition of AIDS, we examined data collected by the Hemophilia Study Group from 1975 to 1979 on 1,551 patients with factor VIII deficiency. The prevalence of lymphocytopenia and thrombocytopenia in patients over 5 years of age on entry was found to be 9.3% (94/1,013) and 5.0% (26/518), respectively. These rates were significantly different from a normal population (P less than .00001 and less than .0003). No cases meeting the definition of AIDS were noted during the study. However, on follow-up in 1984 of a cohort of 79 patients with thrombocytopenia or lymphocytopenia on two or more occasions during the study, eight patients (10%) with AIDS-related abnormalities, including idiopathic thrombocytopenic purpura, non-Hodgkin's lymphoma, generalized lymphadenopathy, and oral moniliasis without obvious cause were identified. Of the 79 patients, liver disease accounted for five of the ten deaths (12.6% mortality) observed during a minimum follow-up of five years after detection of cytopenia. Only one death was attributed to bleeding in the absence of liver disease. We conclude that (a) the frequency of lymphocytopenia and thrombocytopenia was increased in multitransfused factor VIII-deficient hemophiliacs before the advent of AIDS, and (b) persistent lymphocytopenia and thrombocytopenia appear to be strongly associated with liver disease, which was the leading cause of death in a cohort of hemophiliacs followed five or more years.

Acute Kidney Injury↗
PubMed1984

A heparin-like anticoagulant in an 8-month-old boy with acute monoblastic leukemia.

An 8-month-old male with acute monoblastic leukemia died during induction chemotherapy of severe bleeding refractory to repeated infusions of platelets and clotting factors. A heparin effect was suggested by prothrombin time (PT) of 26 seconds, partial thromboplastin time (PTT) of 94 seconds, thrombin time 240 seconds, and reptilase time 18.4 seconds, with a fibrinogen of 88 mg/dl. Both plasma mixed with the patient's urine and the patient's plasma had their thrombin times corrected toward normal by both PF4 and protamine. Synergism of the anticoagulant with antithrombin III was demonstrated not only by enhanced inhibition of thrombin but also by an increased rate of formation of thrombin--antithrombin III complexes in the presence of the anticoagulant, which was eliminated by preincubation with heparinase. Since the anticoagulant activity was not found in the blasts themselves, it is presumed that the anticoagulant is heparin/heparan liberated from the endothelial lining by products of the cell destruction secondary to chemotherapy.

Acute Disease↗
PubMed1984

Modulation of mononuclear phagocyte system function and circulating immune complexes by lyophilized concentrates in patients with classic hemophilia.

Infusions of lyophilized antihemophilic factor concentrates in patients with hemophilia were found to affect both circulating immune complexes and in vivo mononuclear phagocyte system function. Following infusion nine patients had simultaneous assessment of serial immune complex levels and mononuclear phagocyte system clearance of IgG-sensitized autologous erythrocytes relative to a previously established baseline. Nine patients also had a separate second sequence, and two a third sequence, of serial immune complex measurements in relation to infusions. The net change in immune complexes over the 2- to 4-hr interval following infusion was consistent in 10 of 11 study pairs (P less than 0.01) despite different antihemophilic factor preparations for each study and different individual patient responses. This change could not be explained by immunochemical rearrangement in infusate and serum since in vitro mixing experiments showed no relationship to in vivo results. Change in mononuclear phagocyte system function showed a strong correlation with change in complexes (r = 0.70; P less than 0.05). It is suggested that infusions of antihemophilic factor can modulate the mononuclear phagocyte system which in turn alters immune complex levels. Both of these effects could potentially influence immune regulation which has been shown to be abnormal in other hemophiliac patient groups.

Adolescent↗
PubMed1984

The use and mechanism of action of intravenous immunoglobulin in the treatment of immune haematologic disease.

In summary, intravenous gammaglobulin appears to be a very useful treatment of immune haematologic disease. Almost all patients with ITP treated with IVIg will have an increase in their platelet counts lasting at least 2 weeks. Some of these patients will derive long-term benefit from the IVIg and many can maintain their platelet counts by periodic single outpatient infusions. Patients with immune neutropenia are also likely to benefit by IVIg treatment which may be of particular benefit due to its not interfering with phagocyte function. RES blockade is a well-documented mechanism of action immediately after IVIg infusion and platelet antibody synthesis may decrease in some patients; IVIg does not appear to protect platelets from platelet antibodies.

Acute Disease↗
PubMed1984

AIDS in hemophilia.

Explore the source record for details and available documents.

Acquired Immunodeficiency Syndrome↗
PubMed1984

Modulation of mononuclear phagocyte function by intravenous gamma-globulin.

To assess the effects on mononuclear phagocyte function of i.v. gamma-globulin treatment in idiopathic thrombocytopenic purpura, we examined in vivo and in vitro mononuclear phagocyte function in 11 patients before and after therapy. All patients, both splenectomized and non-splenectomized, demonstrated a prolongation of in vivo clearance of autologous IgG-sensitized erythrocytes (p less than 0.01). Concurrent in vitro assessment of blood monocyte function showed decreased IgG-sensitized erythrocyte (EA) rosette formation (mean +/- SD: 31.6% +/- 8.2 vs 24.5% +/- 9.5; p less than 0.03) and decreased affinity of Fc receptor-specific IgG oligomer binding (9.9 +/- 16.3 vs 1.8 +/- 2.1 X 10(8) M-1; p less than 0.008), but no consistent change in the estimate of the maximum number of binding sites. Phagocytosis of two different EA probes was decreased (EhuA:0.49 +/- 0.26 vs 0.25 +/- 0.14 erythrocyte/monocyte/hr; p less than 0.02, EoxA: 1.76 +/- 0.66 vs 1.27 +/- 0.67 erythrocyte/monocyte/hr, p less than 0.05). The change in in vivo mononuclear phagocyte system clearance was significantly correlated with the change in the association constant for oligomer binding (r = 0.98, p less than 0.05). These data demonstrate that i.v. gamma-globulin infusions induce alterations of mononuclear phagocyte function that are not dependent on the presence of autologous serum containing infusate. The change in apparent Fc receptor affinity rather than receptor number may reflect an altered Fc receptor population with different binding properties.

Adolescent↗
PubMed1983

Intravenous use of gammaglobulin in the treatment of chronic immune thrombocytopenic purpura as a means to defer splenectomy.

Intravenous gammaglobulin was used to treat 12 children with chronic immune thrombocytopenic purpura in order to avoid splenectomy. The average rise in platelet count with initial treatment was 226,000/microliters. Currently, one patient is in remission, four patients maintain platelet counts greater than 40,000/microliters without treatment, four patients maintain platelet counts greater than 40,000/microliters with single maintenance infusions of IV IgG at four- or 10-week intervals; three patients did not respond to treatment. In nine of 12 patients, splenectomy was avoided or at least postponed. In responding patients, we were able to discontinue immunosuppressive medication. Platelet count rises with initial IV IgG therapy were correlated with both platelet antibody levels and with a better long-term outcome. Toxicity was minimal.

Adolescent↗
PubMed1983

Platelet-associated immunoglobulin G in childhood idiopathic thrombocytopenic purpura.

Platelet-associated IgG was studied in children with acute and chronic ITP and in patients with thrombocytopenic SLE, using the microtiter solid-phase radioimmunoassay. Of the children with acute ITP, 85% had elevated PAIgG levels. The degree of elevation of PAIgG at onset of disease did not correlate with the development of chronicity. Of the children with acute ITP, clinically and hematologically indistinguishable from the rest, 15% had normal PAIgG values. All of 22 children with chronic ITP had elevated PAIgG values. Although there was good correlation between the platelet count and the PAIgG value in children with chronic ITP, the association was not as striking in those with acute ITP; thus, factors in addition to the level of PAIgG may contribute to the thrombocytopenia in the latter group. Patients with SLE and thrombocytopenia had higher values of PAIgG than would be predicted from the platelet count; the PAIgG value is probably not the only factor determining the degree of immune thrombocytopenia.

Acute Disease↗
PubMed1983

Intravenous gammaglobulin treatment of chronic idiopathic thrombocytopenic purpura.

High-dose intravenous gammaglobulin (IVIgG) was given to 12 children and adults with chronic idiopathic thrombocytopenic purpura (ITP) to avoid splenectomy or because they either failed to respond to or required maintenance with high doses of steroids and/or immunosuppressives. The average platelet count increase to initial therapy was 239,500/microliters (range 23,000-790,000). A concomitant IgG Fc receptor blockade, measured by IgG-sensitized 51Cr-labeled autologous erythrocytes, was seen in 11 of 11 patients tested, both splenectomized and not splenectomized, lasting 3-4 wk. Six or more months after treatment, 2 children are in remission, 2 children and 2 adults are stable requiring no therapy with platelet counts of approximately 50,000 and 30,000, respectively, 3 children require maintenance IVIgG therapy at 2-10-wk intervals, and 1 child and 2 adults have become refractory to further IVIgG. Splenectomy was not performed in 4 children. Two adults were able to discontinue daily prednisone. The 3 patients who became unresponsive to Swiss Red Cross gamma-globulin (IgSRK) therapy did so in conjunction with a markedly elevated platelet-associated IgG and IgM. Serum IgM increased an average of 103 mg/dl after the IVIgG infusions. No significant side effects were seen.

Adolescent↗
PubMed1983

The use of factor eight inhibitor by-passing activity (FEIBA immuno) product for treatment of bleeding episodes in hemophiliacs with inhibitors.

FEIBA (factor eight inhibitor by-passing activity) Immuno was used to achieve hemostasis in 46 patients with factor VIII inhibitors with titers greater than 4 Bethesda units, and 3 patients with factor IX inhibitors. One-hundred and sixty-five bleeding episodes were treated with 50-70 U/kg; 102 of these episodes occurred in joints. 20 in mucous membranes, 33 muscle and soft tissue, and 10 were emergency episodes including 3 central nervous system and 4 surgical procedures. Ninety-three percent of the bleeding episodes were controlled, while 7% were not controlled: 36% were controlled by one infusion in 12 hr, another 42% with 1 or more infusions in 36 hrs and an additional 14% were controlled in more than 36 hr. There were no serious side effects, and while the inhibitor titer rose in 10 of the patients, the product continued to be efficacious.

Clinical Trials as Topic↗
PubMed1982

Immunoregulatory properties of childhood leukemias.

Investigation of in vitro humoral immune responses and immunoregulatory properties of leukemic cell was carried out in 17 children with acute leukemia prior to therapy. Leukemias were of the non-T, non-B-cell type in 13 patients and of T-cell origin in four. Bone marrow and peripheral blood cells consisted of 24-96% lymphoblasts and were generally deficient in surface Ig-positive cells. Induction of Ig secreting cells in response to pokeweed mitogen was markedly decreased in marrow and peripheral mononuclear cell cultures of leukemic patients. Co-culture of leukemic cells with normal lymphocytes led to marked deviations from the expected Ig secreting-cell response of the cell mixtures. The predominant effect was enhancement, as was the case with eight non-T, non-B-cell and one T-cell leukemia samples. Suppression of the Ig secreting-cell response was observed in only three instances, two with non-T, non-B-cell and one with T-cell leukemia samples. These findings implicate non-T, non-B as well as more differentiated leukemic cells in having the potential for modifying Ig production by B cells.

Adolescent↗
PubMed1980

Hemophilic arthropathy: effect of home care on treatment patterns and joint disease.

To evaluate the efficacy of home care therapy on hemophilic arthropathy, data were analyzed in 19 patients with hemophilia who had been on home therapy for more than four years. Usage of replacement material, number of bleeding episodes, and clinical and radiographic assessment of joint status were evaluated. Patients were divided into three treatment groups (prophylaxis, combination, and episodic care) for further comparison. In all treatment groups, a significant decrease in product usage occurred with age (P < 0.01). The number of bleeding episodes also decreased significantly with age (P < 0.01). The patients' joints which were clinically and radiographically normal on entry into home therapy remained free of arthropathic changes. The outcome of diseased joints varied across treatment groups, with a majority of these joints remaining stable. Younger patients evidenced new and progressive arthropathy, whereas older patients demonstrated stable arthropathy. Although no single treatment protocol appears to be indicated for all patients with hemophilpia, a treatment goal may be to treat younger patients actively in order to preserve normal joint status, stablize diseased joints, and prevent subsequent disability.

Adolescent↗
PubMed1980

Depressed serum somatomedin activity in beta-thalassemia.

We have found bioassayable somatomedin activity to be subnormal in 20 of 32 children and adults with beta-thalassemia. The levels were comparable to values reported in growth hormone-deficient subjects. Since patients with thalassemia are not growth hormone deficient, the data suggest the possibility of defective hepatic biosynthesis of somatomedin. Increased iron stores in these patients, who have secondary hemosiderosis of many organs, including the liver, may depress somatomedin activity. Therapy for one year with daily subcutaneous infusions of the iron-chelating agent deferoxamine had no effect on mean bioassayable serum somatomedin activity.

Adolescent↗