Paroxysmal Nocturnal Hemoglobinuria (PNH) is an uncommon hematologic disease characterized by an abnormal sensitivity of blood cells to the lytic action of serum complement. We experienced one case of PNH in Yeungnam University Hospital from May 1983 to May 1989. The patients was followed up without severe complications of 4 years since diagnosis with the only conservative treatments such as washed blood transfusion, adrenal corticosteroids, androgens, folate and iron preparation, intermittently.
Adrenal Cortex Hormones ; Androgens ; Blood Cells ; Blood Transfusion ; Complement System Proteins ; Diagnosis ; Folic Acid ; Hematologic Diseases ; Hemoglobinuria, Paroxysmal* ; Humans ; Iron

No abstract available.
Child* ; Humans ; Occult Blood*

No abstract available.
Ductus Arteriosus* ; Indomethacin*

No abstract available.

No abstract available.

No abstract available.
Child* ; Humans ; Infant, Newborn* ; Plasma* ; Renin*

No abstract available.
Child* ; Humans ; Peritoneal Dialysis, Continuous Ambulatory*

"It was reported that polymorphism of TNF alpha gene was present in promoter region and involves the substitution of guanine by adenosine in the uncommon (TNFA 2) allele. In this study, we investigated the significance of TNFA gene polymorphism in relation to various clinical characteristics and autoantibody profiles in SLE as well as comparing it with that of other countries, and also studied its association with peripheral TNF-a production in vitro. TNFA genotyping was performed in 126 SLE patients and 300 controls using DNA extracted from peripheral leucocytes. The biallelic polymorphism at position -308 of the TNFA promotor was determined by Ncol- RFLP. Peripheral mononuclear cell production of TNF-a was investigated by bioassay using L-929 cell cytotoxicity. The TNFA ""1 homozygote was a predominant allele (77.0%) in SLE, which was not different from the controls. TNFA ""2 homozygate was extremely rare in both patients and controls (0.8%, 1.3% respectively). The clinical manifestations between TNFA '1 and TNFA""2 did not differ. The production of autoantibodies including dsDNA, anti-La, anti-nRNP and anti-Sm was not different between two alleles, whereas anti- Ro antibody was more frequent in TNFA""1/TNFA '1 than in TNFA'1/TNFA'2 (62.1% vs 38.4%, P=0.022). The polymorphism of TNFA gene did not influence the lipopolysaccharide stimulated peripheral mononuclear cell production of TNF-a (1356+/-293 vs 1119+/-385 pg/ml; TNFA'1/TNFA'1, TNFA'1/TNFA'2 respectively). These results suggested that promoter polymorphism of TNFA was not directly involved in the susceptibility of SLE and was not responsible for differential peripheral TNF-a production, but TNFA ' may be associated with anti-Ro antibody production."
Adenosine ; Alleles ; Autoantibodies ; Biological Assay ; DNA ; Guanine ; Homozygote ; Humans ; Lupus Erythematosus, Systemic* ; Polymorphism, Restriction Fragment Length ; Promoter Regions, Genetic ; Tumor Necrosis Factor-alpha*

We experienced a case of childhood derrnatomyositis associated with calcinosis universalis in a 3-year-old boy. The skin lesion showed generalized, hard nodules and ulcers, 0, 3-1 cm in cliameter, or the trunk and buttock. The hitopathologic findings revealed patch fat necrosis and calcium deposits in the subcutis.
Buttocks ; Calcinosis* ; Calcium ; Child, Preschool ; Dermatomyositis* ; Fat Necrosis ; Humans ; Male ; Skin ; Ulcer

No abstract available.
Ascorbic Acid* ; Child* ; Humans