High-dose intravenous immunoglobulin for severe platelet alloimmunization.
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Biomedical subjects
Publications and source records attributed to T R Kinney.
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The anaerobic diphtheroid Propionibacterium acnes was identified as the organism responsible for "shunt" nephritis in two adolescents with ventriculoatrial shunts inserted previously for the management of medulloblastoma. Only three cases of "shunt" nephritis secondary to infection with this common skin commensal organism are known to have been reported. The rarity of such reports may be related to the lack of good anaerobic laboratories, the length of time necessary for cultures to show growth, the poor ability of certain blood culture media to support growth of this organism, and the failure of physicians to consider Propionibacterium acnes as a true pathogen in certain clinical situations.
A family is described in which venous thrombosis developed in five members as early as 14 years of age. Routine coagulation studies, plasma antithrombin III, factor V, plasminogen, beta-thromboglobulin, fibrinopeptide A, prothrombin fragment F1+2, and thrombin-antithrombin III complex were all within normal limits. However, defective release of vascular plasminogen activator was observed on several occasions in all five subjects as compared with a control population of 125 persons (0.04 Committee on Thrombolytic Agents [CTA] units/ml plasma as compared with 0.21 CTS units/ml). In addition, levels of factor VII/von Willebrand's factor were significantly elevated above the normal range in this pedigree.
Chest computed tomography (CT) is often used to detect and monitor pulmonary nodules in children undergoing chemotherapy for underlying sarcoma. Three children underwent resection of nodules which persisted during chemotherapy. In two children, all resected nodules were benign; in the other, a mixture of benign and malignant nodules was found. Our findings suggest that nodular scar formation after successful eradication by chemotherapeutic agents may be a mechanism for the persistence of pulmonary nodules. It should not be assumed that persistence of pulmonary nodules on CT after appropriate chemotherapy indicates inadequate response.
A patient with refractory T-cell acute lymphoblastic leukemia was treated with eight courses of the adenosine deaminase inhibitor, 2'-deoxycoformycin (dCF), over a 5-month period. After developing resistance to dCF, he responded to treatment with the combination of dCF and 9-beta-D-arabinofuranosyladenine (ara-A). We monitored the levels in plasma and urine of adenosine, 2'-deoxyadenosine, and ara-A as well as the accumulation of their nucleotide derivatives in erythrocytes and circulating lymphoblasts. We also monitored the activities of adenosine deaminase and S-adenosylhomocysteine (AdoHcy) hydrolase and the concentrations of AdoHcy and S-adenosylmethionine in lymphoblasts. Production of 2'-deoxyadenosine was related to both the duration of dCF infusion and the magnitude of cytolysis that occurred during treatment: much more 2'-deoxyadenosine was produced by dCF infusion when disease was active than by the same infusion given during remission. Resistance to dCF was associated with a decrease of greater than 90% in the amount of deoxyadenosine 5'-triphosphate accumulated by circulating lymphoblasts. Infusion of dCF resulted in increases of up to 20-fold in the concentration of AdoHcy in circulating lymphoblasts, causing a decrease in the S-adenosylmethionine:AdoHcy ratio (the "methylation index") from a pretreatment value of greater than 40:1 to less than 4:1. This ratio decreased to 2.5:1 during combined treatment with dCF and ara-A, which caused nearly complete inactivation of lymphoblast AdoHcy hydrolase. Decline in the methylation index was accompanied by inhibition of the methylation of newly synthesized lymphoblast RNA. Impaired ability to catabolize AdoHcy may have contributed to the cytolytic responses to dCF and ara-A, as well as to hepatic and central nervous system toxicity associated with their combined use.
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Sequential studies of cellular and humoral immunity were conducted in an infant with the Wiskott-Aldrich syndrome prior to and after a splenectomy for uncontrollable hemorrhage. All measures of cellular immunity showed gradual improvement during the 8-month period after surgery. Serum isohemagglutinins, diphtheria and tetanus antibodies, and the percentage of immunoglobulin-bearing B cells did not change significantly from presplenectomy values. The serum IgE concentration declined from a high of 10,800 IU/ml at 1 month postsplenectomy to a low of 860 IU/ml at 5 months after surgery and the IgG concentration gradually decreased from a high of 1880 mg/dl presplenectomy to a low of 620 mg/dl 8 months later. The platelet count ranged from 64,000 to 206,000/mm3 for the first 6 months after splenectomy. It decreased precipitously 6.5 months after the operation; at the same time there was a marked rise in platelet-bound IgG antibody (PB-IgG). The PB-IgG declined rapidly following vincristine therapy and, after another rise, declined more gradually following steroid therapy.
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Peripheral blood globin synthesis studies were done in 11 patients with delta beta-thalassaemia trait, Hb S-delta beta-thalassaemia or delta beta/betao-thalassaemia from two black and two Caucasian families. All patients had elevated Hb F and normal or decreased Hb A2 levels and 10 had family studies confirming the diagnosis. In addition, four unrelated non-thalassaemic patients with elevated Hb F levels also had peripheral blood globin synthesis studies. The beta/alpha specific activity globin synthesis ratios in the three blacks with delta beta-thalassaemia trait were 0.60--1.04. In the four Caucasians with delta beta-thalassaemia traint, the beta/alpha ratios were 0.58--1.02. These results demonstrate a wide range of ratios overlapping those of normal controls (0.99 +/- 0.06). The betas/alpha ratios in three blacks with Hb S-delta beta-thalassaemia ranged from 0.66 to 1.00, similar to those of patients with delta beta-thalassaemia trait. In the black patient with delta beta/betao-thalassaemia, the gamma/alpha ratio was 0.67. The beta/alpha peripheral blood ratios in the four non-thalassaemia patients with elevated Hb F ranged from 1.00 to 1.11, similar to those of normal controls. These studies indicate that a decreased beta/alpha ratio is not an invariable finding in delta beta-thalassaemia in blacks or Caucasian patients and that globin synthesis data alone is insufficient to diagnose definitively heterozygotes for delta beta-thalassaemia or to distinguish this trait from non-thalassaemic haematological disorders associated with a normal percentage of Hb A2 and an elevated level of Hb F.
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Computerized tomography was used to evaluate the severity of six episodes of suspected intracranial bleeding in four patients with hemophilia. In all instances the CT scan rapidly provided information on the extent and location of the intracranial hemorrhage. Results of the initial scan provided a rational basis for therapy, and subsequent scans were a noninvasive means of evaluating the effects of treatment. If available, computerized tomography is a valuable aid in the management of the hemophiliac patient with intracranial bleeding.
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A 5-year-old boy with acute lymphoblastic leukemia in complete continuous remission developed life-threatening varicella pneumonia and acute respiratory insufficiency (ARI). The child recovered after 92 hours of partial venoarterial perfusion with a membrane oxygenator. Functional asplenia developed. Serial pulmonary function tests after perfusion indicate moderately severe restrictive lung disease which has slightly improved during an 18 month period.
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