Granulocyte-specific antigens, such as NA1, NA2, NBI, NB2, NC1, NDI, NE1, are a group of antigens specifically expresesed only on the granulocytes. Antibodies against these are involved in some clinical disorders such as alloimmune neonatal neutropenia(ANN), autoimmune neutropenia(AIN), and transfusion-related acute lung injury(TRALI). We investigated the frequencies of NA1, NA2, NB1, and Mart antigens among Koreans by the granulocyte indirect immunofluorescence test employing flow cytometry. The subjects were 105 Koreans(male 65, female 40), whose mean age was 31.7+/-8.2 years (range 16~57). The antigen and gene frequencies were as follows, NA1, 0.78, 0.53, NA2, 0.75, 0.50, NB1 0.86, 0.62, and Mart, 1.00, 1.00, respectively. The proportions of NB 1 -positive granulocytes among NB 1-positive individuals were variable(range, 27~100%). Through this study, the authors procured granunlocyte-specifiic antigen papnel, which is essential in the identification of causative antibody(-ies) in immune neutropenias.
Antibodies ; Female ; Flow Cytometry ; Fluorescent Antibody Technique, Indirect ; Gene Frequency ; Granulocytes ; Humans ; Lung ; Neutropenia

BACKGROUND: A and B transferase are glycosyltransferase that transfer N-acetylgalactosamine and D- galactose to H antigen, respectively and lead to the expression of A and B phenotypes in ABO blood group system. Reduced or no activities of serum A and B transferase were observed in some A and B subgroup individuals. Determining the activities of serum A and B transferase can be useful in discriminating rare A and B subgroups. MATERIALS AND METHODS: ABO typing, saliva test, adsorption elution test and serum transferase assay were performed on samples from 12 individuals showing ABO discrepancy or weakened cell typing reactions which were referred to the Seoul National University Hospital to confirm their ABO blood types. Serum transferase activity was assayed by determining the ability of serum to convert group 0 RBCs into A or B cells. RESULTS: Determination of serum ABO transferase activity was useful in the identification of Ael (3 cases), B. (2 cases), Bm (1 case), Am (1 case), Bx (1 case), 0 with weakened anti-A or anti-B (3 cases), and A without anti-B due to hypogammaglobulinemia (1 case). CONCLUSION: Determining serum A and B glycosyltransferase activity was proven to be a simple and useful tool for the classification of several ABO subgroups.(Korean J Blood Transfusion 10(1): 27-33, 1999)
ABO Blood-Group System ; Adsorption ; Agammaglobulinemia ; B-Lymphocytes ; Blood Transfusion ; Classification* ; Galactose ; Phenotype ; Saliva ; Seoul ; Transferases

No abstract available.
Erythrocytes*

No abstract available.
Erythrocytes*

Bromidrosis is a disorder characterized by rancid body odor which influences a patient's social life and mental health. The therapeutic modalities and the mechanism of bromidrosis have been carefully studied, however, there have been few reports about the genetic inheritance of bromidrosis. We investigated the family history of 42 patients who were operated on for bromidrosis and followed up to the third generation in 10 cases. The results were as follows: Results of investigation which were followed up the second generation. The fathers of five patients and the mothers of 11 patients had bromidrosis in 18 male patients. The fathers of six patients and the mothers of 12 patients had bromidrosis in 24 female patients. Thirty-four patient (81.0%) among a total of 42 have a single parent with bromidrosis. Result of investigation which were followed up to the third generation Bromidrosis was occurred in 17 of 42 patients (40.5%) in the second generation, and 18 of 27 patients (66.7%) in the third generation. In one case, a father transmitted bromidrosis to his three sons, and as a result, X-linked inheritance could be ruled out Bromidrosis was not skipped in every generation of all families. We on conclude that bromidrosis is an autosomal dominant inherited disorder.
Fathers ; Female ; Genes, X-Linked ; Humans ; Male ; Mental Health ; Mothers ; Odors ; Single Parent ; Wills*

Infantile myofibromatosis is an uncommon, benign, probably hamai tomatous proliferation of myofibroblasts. This lesions are most often present before the age of 2 years, show a male predomenance and may be multiple or solitary. The solitary lesions arise in the dermis, subcutis, or deep soft tissue, most commonly in the head and neek region or trunk. The elinical coirse is benign and spontaneous regression is not infrequent. A female newborn presented with a turmr lesion on the upper portion of the right eyelid. Physical examination showed a firm, non-tender, fixed, erythematous, 3 x 3cm sized tumor. Histologic examinatign revealed a spindle cell tumor in the deimis with a whorled arrangemant and grouped fescicles. There was no evidence for potential visceval and bony involvement. Follow-up examination at 6 months of aige revealed a remarkable degree of spoitaneous involution.
Dermis ; Eyelids ; Female ; Follow-Up Studies ; Head ; Humans ; Infant, Newborn ; Male ; Myofibroblasts ; Myofibromatosis ; Physical Examination

Three cases of giant pituitray adenoma are reported. Two cases were operated by transsphenoidal approach and a case by transcranial approach. Transsphenoidal approach showed satisfactory results despite of marked suprasellar extension of tumors. We report rare giant pituitary adenomas with review of relevant literatures.
Adenoma ; Pituitary Neoplasms*

No abstract available.
Thanatophoric Dysplasia*

Macrostomia is a relatively rare malformation. There have been many surgical methods for the correction of macrostomia. The old method, a simple straight line closure , did not include reconstruction of the orbicularis oris muscle. Postoperatively, a depressed scar and severe contraction on the cheek were found, especially with animation. As a result, many authors have known that reconstruction of the orbicularis oris muscle was very important to avoid a depressed scar for the correction of macrostomia. The tendency toward lateral displacement of the reconstructed commissure has been attributed to linear scar contraction, and Z-plasty is advocated to prevent this deformity. However, we found that the Z-plasty scar may be conspicuous when the patient smiles. So we performed reconstruction of the orbicularis oris muscle to avoid a depressed scar, and straight line closure to avoid a conspicuous scar. In order to prevent linear scar contracture due to straight line closure, we overcorrected the new commissure. We treated 6 cases of macrostomia form May 1, 1996 to April 30, 1999 using straight line closure and reconstruction of the orbicularis oris muscle. Periods of follow-up were from 12 months to 35 months, with an average of 20,2 months. Every patient was analyzed clinically by ordinary scale method and anthropometrically by the ratio of abnormal distances to normal distances between cheilion and crista philtri. In 3 clinical assessments : symmetry of the commissure was excellent: degree of the scar at rest was good: and degree of depression with animation was excellent. The anthropometrical ratio was 1:1.05. Despite the fact that the linear scars were perpendicular to the minimal skin tension lines, the scars were inconspicuous. The muscle repair provided reconstruction to the modiolus and gave a natural appearance to the commissure, however it did not seem to provide sufficient bulkiness around the commissure. The straight line skin closure and repair of the orbicularis oris muscle provide effective functional and aesthetic reconstruction.
Cheek ; Cicatrix ; Congenital Abnormalities ; Contracture ; Depression ; Follow-Up Studies ; Humans ; Macrostomia* ; Skin

No abstract available.