Intravenous Anti-D immunoglobulin treatment of adult chronic immune thrombocytopenic purpura (ITP) / 대한내과학회지

BACKGROUND/AIMS: Immune thrombocytopenic purpura (ITP) is an autoimmune disease that is mediated by anti-platelet antibodies. Based on the pathogenesis of ITP we evaluated the efficacy of intravenous anti-D immunoglobulin for adult chronic ITP. METHODS: Fourteen patients (4 without splenectomy and 10 with splenectomy) with refractory chronic ITP were treated with 50-70 microgram/kg of intravenous anti-D immunoglobulin only once. Treatment effects were evaluated by measuring the platelet counts and hemoglobin levels. RESULTS: Five patients (36%) showed a response; improvement in the platelet count lasted for on average 7 days (range: 2~24 days). There were no serious adverse effects. CONCLUSION: Anti-D immunoglobulin, which is associated with an Fc receptor blockade, appeared to be safe and effective for the treatment of adults with chronic ITP. Further studies are needed to confirm these findings and define further potentially effective treatment protocols with intravenous anti-D immunoglobulin.

ADAMTS13 Activity in Childhood Hemolytic Uremic Syndrome(HUS)

PURPOSE: HUS usually occurs in children after infection with shiga toxin-producing microorganism(D+HUS). In contrast, non-postdiarrheal(D-) HUS occurs at any age and has a high rate of relapse and a poor prognosis. The clinical presentation of D-HUS is similar to that of thrombotic thrombocytopenic purpura(TTP). Recently severe deficiencies of ADAMTS13 were reported not only in TTP and D- HUS but also in D+ HUS during their acute phase. The purpose of the study is to evaluate the plasma ADAMTS13 activity in D+ and D-HUS. METHODS: Nineteen children with HUS(D+ HUS 12 and D- HUS 7) were enrolled. The assays of plasma ADAMTS13 activity were performed during the acute stage in the D+ HUS and at various stages of relapsing courses in the D- HUS patients by multimer assay, based on electrophoresis. RESULTS: The median plasma activity of ADAMTS13 in D+ HUS and D- HUS were 80.9%(37.8-132.4%) and 53.9%(1.0-94.1%), respectively, which were not statistically significantly different from control(86.4%, 34.2-112.3%)(P>0.05). One boy with D- HUS had severe deficiency of ADAMTS13(1.0%). His platelet count was normalized temporarily by fresh frozen plasma infusion. CONCLUSION: We have demonstrated that there is no significant difference of the plasma ADAMTS13 activity between D+ HUS, D- HUS and control. We detected severe deficiency of ADAMTS13 in one boy who presented with relapsing episodes of D- HUS. ADAMTS13 deficiency should be considered in the subgroup of D- HUS especially with early onset and recurrent courses. Plasma therapy can be beneficial in this subgroup.

A Study of Protein S Deficiency in Antiphospholipid Syndrome / 대한불임학회지

OBJECTIVE: To evaluate the abnormality of protein S in patients with recurrent spontaneous abortion due to antiphospholipid syndrome. MATERIALS AND METHOD: Antigen and activity of protein S were analyzed by enzyme immunoassay and clotting method, respectively. RESULTS: Of 18 patients with antiphospholipid syndrome, 4 patients were found to have no abnormality of protein S. There were 14 cases of protein S abnormality. Among them, there were 8 cases of type 1, 1 case of type 2, and 5 cases of type 3 protein S deficiency. CONCLUSION: So in the workup of patients with recurrent spontaneous abortion due to antiphospholipid syndrome, the evaluation for protein S is required.

The Analysis of Methylenetetrahydrofolate Reductase Mutation in Recurrent Spontaneous Abortion Associated with Hyperhomocysteinemia / 대한불임학회지

OBJECTIVE : To analyze the methylenetetrahydrofolate reductase (MTHFR) mutation in recurrent spontaneous abortion associated with hyperhomocysteinemia. MATERIAL AND METHOD: The blood Sample of habitual aborter with high fasting homocysteine level was tested by PCR-RFLP method. RESULTS: The patient was found to be a homozygosity for MTHFR gene mutation that was confirmed by the finding which is consistent with the mutation at the nucleotide 677 C to T, Corresponding to Ala to Val. CONCLUSIONS: Hyperhomocysteinemia due to MTHFR mutation is a cause of recurrent spontaneous abortion. Therefore, the MTHFR mutation should be examined in the workup of recurrent spontaneous abortion showing hyperhomocysteinemia.

Analysis of Daily Intake and Urinary Excretion of Iodine normal Control and Patient with Thyroid Disease / 대한내분비학회지

Iodine in physiological dosage is necessary for thyroid hormone. But insufficient or excessive intake of iodine could affect on thyroid function. However, little study is available on the current iodine state in Korea. To evaluate the current state of iodine intake and its effects on thyroid function, we measured the iodine intake, urinary excretion and thyroid status in 67 normal controls and 110 patients with thyroid disease from June 1991 to August 1993.The result were as follows; 1) There were no differences in the physical and daily intake of calorie between controls and patients with thyroid disease. 2) The mean daily intake of iodine was 391.4 ug in normal controls, 695.0 ug in patients with simple goiter, 672.0 ug in hypothyroidism, 453.5 ug in hyperthyroidism, and 297.4 ug in thyroid nodule. 3) The mean urinary iodine excretion was 640 ug/L in normal controls, 875 ug/L in patients with hyperthyroidism, 612 ug/L in thyroid nodule, 563 ug/L in hypothyroidism, and 549 ug/L in simple goiter. 4) There was decreasing tendency of the serum T_3, t_4 and significant increase in TSH concentration in subjects who ingest the dietary iodine above 1000 ug/day compared with the subjects who ingest below 150 ug/day(p<0.05). 5) Subjects who live in coastal area had much intake of iodine(1.8-2.5 times)than those of subjects live in inland area.In conculsion, Korean ingested more iodine than Europian and American but fewer than Japanese. Some of the patients with simple goiter and hypothyroidism patients may associated with excessive ingestion of iodine, but further systemized study will be required.

A case of plasma cell leukemia / 대한혈액학회지

No abstract available.