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Biomedical subjects

H Lackner

Publications and source records attributed to H Lackner.

At least 37 records · Page 2Linked to original sources

Successful treatment of CMV retinitis with ganciclovir after allogeneic marrow transplantation.

Cytomegalovirus (CMV) infection of the retina is a well recognized complication in patients with the acquired immune deficiency syndrome but is rarely seen after bone marrow transplantation (BMT). Among a variety of drugs ganciclovir so far appears to be the most effective therapy for CMV retinitis, but in previous studies relapses occurred in all patients in whom ganciclovir was interrupted. We report the clinical findings in a 22-year-old BMT recipient who developed bilateral exudative CMV retinitis 64 days after BMT despite prophylactic treatment with high-titer CMV-immunoglobulins and transfusions of CMV-negative blood products and donor bone marrow. During a 12 day course of treatment with 7.5 mg/kg/day of ganciclovir the CMV retinitis improved and viruria ceased on day 4 of therapy. In contrast to the previous reports, CMV retinitis in this patient continued to improve even after ganciclovir was stopped and eventually complete healing of all intraretinal lesions as well as total reconstitution of the visual acuity was achieved. He is now free of disease and without relapse of CMV retinitis more than 1 year after transplantation.

Adult

[Cyclosporin A in severe aplastic anemia in children].

Severe aplastic anemia should be treated with bone marrow transplantation if possible. Various clinical and experimental data support the view that the major pathogenetic defect in SAA is a dysregulated cellular immune response which in turn has a negative effect upon hematopoiesis. Therefore a large percentage of patients react favorably to treatment with immunosuppressive agents as antithymocyte globulin and high-dose methylprednisolone. Data concerning the efficiency of Cyclosporine A treatment are limited until now. We present in this report our own experience with Cyclosporine A treatment in three children with severe aplastic anemia who lack a bone marrow donor. Included is also a review of the present therapeutic possibilities.

Adolescent

[Malignant bone marrow infiltration in nuclear magnetic resonance tomography].

Magnetic resonance imaging (MRI) is currently the only non-invasive method detecting changes of bone marrow. While yellow bone marrow produces a high signal intensity, which is similar to subcutaneous fat, any other cellular infiltration of the bone marrow causes a decrease of signal intensity because of replacement of marrow fat cells. In this report we describe two patients, who underwent MRI because of clinical symptoms of coxitis, in order to exclude Perthes disease. Both cases showed decreased signal intensity of the bone marrow particularly of the proximal femura, highly indicative of cellular bone marrow infiltration. Bone marrow aspiration in these patients showed acute lymphocytic leukemia in one case and marrow infiltration by neuroblastoma cells respectively in the other case. Because of its high sensitivity, MRI is also suitable in detecting focal bone marrow disease. For these cases a biopsy of these focal bone marrow lesions can be performed in order to stage the disease properly as in the presence of neuroblastoma or malignant lymphoma presenting with localized disease, however, with focal bone marrow metastases on MRI. In addition MRI can also be used to follow up the disease as well as therapy by showing the regression of these bone marrow lesions.

Adrenal Gland Neoplasms

Hermansky-Pudlak syndrome: a clinicopathologic study.

The Hermansky-Pudlak syndrome is a rare disease characterized by multisystemic involvement. Seven families with the disorder were identified in the Puerto Rican population of one municipal hospital, suggesting that the incidence in the Puerto Rican community is sufficient to warrant both dissemination of information about the disease and further investigation. The present study was an attempt to achieve both of these goals.

Adolescent

Unrecognized pulmonary embolism presenting as disseminated intravascular coagulation.

Six patients are described in whom disseminated intravascular coagulation of uncertain cause was found to be due to occult pulmonary embolism. The peripheral blood smear showed thrombocytopenia in all patients and schistocytes in four. Coagulation studies revealed increased levels of fibrinogen/fibrin degradation products (six of six patients), positive results for fibrin monomer (five patients), prolonged thrombin times (four patients), hypofibrinogenemia (three patients), prolonged prothrombin times (two patients), and decreased plasma coagulation factors (two patients). Pulmonary embolism was confirmed by lung scanning or pulmonary angiography. Institution of full-dose heparin therapy was associated with hemostatic and clinical improvement in all patients. The association of disseminated intravascular coagulation with occult pulmonary embolism merits recognition since full-dose heparinization is required for successful therapy.

Adenoma

The role of the activated clotting time in heparin administration and neutralization for cardiopulmonary bypass.

Precise guidelines for heparin administration and neutralization during cardiopulmonary bypass (CPB) are not established. To a large extent, the uncertainty originates from a disparity between the heparin dosage, the plasma heparin concentration, and the clinical heparin effect. We investigated these relationships in 44 consecutive patients at New York University Medical Center. Following serial loading doses of heparin (2 and 4 mg/kg) there was a wide variation in both the measured clinical heparin effect (activated clotting time--ACT) and the plasma heparin concentration. When the Act was compared to the heparin concentration, there was no linear relationship noted after heparin does commonly employed for CPB. The calculated heparin sensitivity varied over a fourfold range and was not related to the baseline antithrombin III activity. At the completion of CPB, heparin was neutralized with a 2 mg/kg protamine dose regardless of the total heparin dose. Heparin levels fell from 4.17 +/- 1.29 to 0.19 +/- 0.20 units/ml. Additional protamine was given to 49% of the patients as the ACT had not returned to pre-heparin levels. The total protamine dose rarely exceeded 3 mg/kg. This technique resulted in the administration of 30% to 50% less protamine than predicted by other commonly used protocols. In the subsequent 4 hours after protamine administration, heparin levels remained insignificant. Mild heparin rebound was found in two patients (4.5%) but was not associated with excessive bleeding. Following bypass a comparison of heparin levels and ACTs demonstrated the ACT to be a poor indicator of residual circulating heparin. These data show: (1) that neither the heparin dosage nor the plasma heparin concentration can accurately predict the magnitude of the clinical heparin effect in patients undergoing CPB and emphasize the importance of the ACT as the best available measurement of safe anticoagulation, (2) heparin "rebound" was not clinically significant, and (3) heparin was neutralized with 2 to 3 mg/kg protamine in virtually all patients, regardless of the total heparin dose.

Adult

The diagnostic value of the serum lactic dehydrogenase determination in the evaluation of unexplained thrombocytosis.

Thrombocytosis may reflect a primary myeloproliferative disorder or be a secondary reaction to other pathologic processes. Patients with persistent unexplained thrombocytosis meeting criteria of primary thrombocythemia had elevated levels of serum lactic dehydrogenase, whereas comparable patients with secondary thrombocytosis did not. The findings of an elevated serum lactic dehydrogenase supports the diagnosis of a myeloproliferative syndrome in patients who have unexplained thrombocytosis, and should be useful in the differential diagnosis of this hematologic abnormality.

Adult

Easy bruising, thrombocytopenia, and elevated platelet immunoglobulin G in Graves' disease and Hashimoto's thyroiditis.

Platelet IgG levels, count, and function and easy bruising or bleeding were studied in 25 patients with Graves' disease and 12 with Hashimoto's thyroiditis (normal value for platelet IgG 10.7 +/- 4.5 ng [SD]/10(6) platelets). Eight of 22 patients with Graves' disease and normal platelet counts had elevated platelet IgG averaging 38 +/- 4.0 ng (SEM) (range, 24 to 60). Four of 10 patients with Hashimoto's thyroiditis and normal platelet counts ahd elevated platelet IgG averaging 45 +/- 7.2 ng (range, 27 to 66). Five patients with thrombocytopenia had platelet counts averaging 53000 +/- 12000/microL (SEM) and elevated platelet IgG averaging 154 +/- 40 ng (range, 27 to 300). Twelve of 15 patients with a history of easy bruising or bleeding had elevated platelet IgG compared to five of 22 without easy bruising (p < 0.001). Four of six with elevated platelet IgG had one or more abnormal in-vitro platelet aggregation measurements (particularly with epinephrine) compared to none of six with normal platelet IgG levels (p = 0.03). We conclude that elevated platelet IgG is associated with easy bruising and thrombocytopenia in about half of patients with Graves' disease or Hashimoto's thyroiditis.

Blood Coagulation Disorders

The relationship of coagulation factors to clinical complications of acute pancreatitis.

Alterations in coagulation factors have been reported during acute pancreatitis. Therefore the relationship of coagulation measurements to complications of pancreatitis was evaluated prospectively in 35 patients in whom 130 serial coagulation profiles were performed, consisting of fibrinogen, platelet count (PC), fibrinogen-fibrin-related-antigen (FR-antigen), prothrombin time (PT), partial thromboplastin time, thrombin time, euglobulin clot lysis, and Factors II, V and VII-X levels. During attacks of acute pancreatitis, over-all mean initial fibrinogen and PC of 268 mg. per 100 ml. and 214,000 per cubic millimiter rose significantly (p less than 0.005) to peaks of 362 mg. per 100 ml. and 477,800 per cubic millimeter by day 6 to 10. Mean initial FR-antigen of 4.8 microgram per milliliter rose to peak 7.4 microgram per milliliter on day 5. In 21 patients with mild pancreatitis, mean highest fibrinogen, PC, FR-antigen, and PT were 329 mg. per 100 ml., 361,500 per cubic millimeter, 5.3 microng per milliliter and 14.1 seconds. These values were significantly higher (p less than 0.05 to 0.01) in patients with severe pancreatitis, being 422 mg. per 100 ml. 528,000 per cubic millimeter, 13.7 microng per milliliter, and 15.5 seconds, respectively. Evaluation of the relationship of coagulation measurements to early clinical features showd that mean initial fibrinogen levels were significantly higher (p less than 0.05 to 0.01) in patients with initial amylase greater than 1,000 Somogyi units percent, serum glutamic oxaloacetic transaminase (SGOT) greater than 250 S.F.U. percent, and initial 72 hour PAO2 less than 75 mm. Hg. Early hypoxemia also correlated (p less than 0.05) with elevated initial FR-antigen levels. Impaired early renal function correlated (p less than 0.01) with elevated initial PC only. Early hypocalcemia did not correlate with coagulation measurements. These findings demonstrate that marked changes in coagulation parameters occur during acute pancreatitis and are related to over-all morbidity. Correlation of early coagulation measurements with amylase levels and with respiratory, renal, and hepatic dysfunction suggests that enzyme-related intravascular coagulation may be implicated in the pathogenesis of these complications of pancreatitis.

Acute Disease

[Propane-diol-(1.3) fatty acid esters as metabolites of postmortal triglyceride catabolism (author's transl)].

The investigation of 72 samples of human subcutaneous fat tissue stored in closed small bottles at room temperature showed that every third sample decomposed with the formation of unknown metabolites. The presence of these compounds could already be recognized in the crude lipid extracts by characteristic 1H-NMR signals, increasing in intensity with time of storage. After isolation, the unknown metabolites could be identified as di- or mono fatty acid esters of propane-diol-(1.3). Thus, the known scheme of postmortal breakdown of triglycerides is to be supplemented by a further remarkable pathway.

Adipose Tissue

On the "easy bruising" syndrome with normal platelet count. A study of 75 patients.

Seventy-five consecutive patients with normal platelet counts were investigated for "easy bruising." All had a normal coagulation profile, and all except four were women. None were on aspirin or other antiplatelet agents. Two specific groups could be delineated. In type I (44 patients, mean age, 35), platelet function was normal to supranormal. Megathrombocyte number was elevated in 60% of patients and correlated with the presence of antiplatelet antibody in 30% of patients. In type II (31 patients, mean age, 34), platelet function was abnormal: impaired epinephrine aggregation (primary and secondary wave) in 97%, impaired connective tissue aggregation in 77%, and impaired ADP aggregation in 42%. Megathrombocyte number was elevated in 71%, and antiplatelet antibody was present in 38% of patients. The "easy bruising" syndrome can be differentiated into two categories: type I, in which a platelet abnormality is unlikely, and type II, in which a platelet abnormality exists. Elevated incidence of antiplatelet antibody in both groups suggests a possible autoimmune cause.

Adenosine Diphosphate