1.Congenital Agammaglobulinemia of Bruton Type.
Journal of the Korean Pediatric Society 1982;25(10):1082-1087
No abstract available.
Agammaglobulinemia*
2.X-linked agammaglobulinemia.
Dal Sik KIM ; Mi Yae YOON ; Hye Soo LEE
Korean Journal of Clinical Pathology 1991;11(3):545-548
No abstract available.
Agammaglobulinemia*
3.50 Years Ago in Journal of Academy of Pediatrics: Agammaglobulinemia.
Korean Journal of Pediatrics 2007;50(12):1266-1266
No abstract available.
Agammaglobulinemia*
;
Pediatrics*
4.A Case of X-linked Agammaglobulinemia with Delayed Growth.
Mi Jin KIM ; Ho Young KIM ; Mi Deok LEE ; Hong Seung KIM ; Young Goo SHIN ; Choon Hee CHUNG ; Kye Chul SHIN
Journal of Korean Society of Endocrinology 1999;14(1):153-159
In 1980, the clinical syndrome of X-linked agammaglobulinemia and isolated growth hormone defieiency (XLA/IGHD) was first described by Fleisher. We report here 25-year old man with isolated growth hormone deficiency and X-linked agammaglobulinemia. His height was below the third percentile. He had recurrent sinopulmonary infection, lacked circulating B lympocytes and had deficient growth hormone.
Adult
;
Agammaglobulinemia*
;
Dwarfism, Pituitary
;
Growth Hormone
;
Humans
5.Osteoarthritis of Hip in a Hypogammaglobulinemia Patient.
Jang Suk CHOI ; Jung Han KIM ; Sang Yeong CHOI
The Journal of the Korean Orthopaedic Association 2010;45(4):326-329
Hypoglobulinemia is characterized by functional or quantitative abnormalities of one or more components of the immune system. It is typically manifests as recurrent infections that usually start in childhood. These recurrent infections cause various clinical symptoms. Infectious or aseptic arthritis can develop, and especially in bone and joint abnormalities. No case of arthritis in a patients with hypogammaglobulinemia has been reported, so we report on the management of osteoarthritis of the hip in a hypogammaglobulinemia patient.
Agammaglobulinemia
;
Arthritis
;
Hip
;
Humans
;
Immune System
;
Joints
;
Osteoarthritis
;
Osteoarthritis, Hip
6.Hepatocellular Carcinoma Following Vertical Transmission of Hepatitis B Virus in a Child with X-linked Agammaglobulinemia.
Jong Gon OH ; Byung Ju KIM ; Hoon KOOK ; Young Jong WOO ; Young Youn CHOI ; Jae Sook MA ; Tai Ju HWANG ; Jong Jin SEO
Korean Journal of Pediatric Gastroenterology and Nutrition 2000;3(1):105-109
X-linked agammaglobulinemia (XLA) is a primary inherited B-cell immunodeficiency. The prevalence of neoplastic disease in patients with XLA is approximatedly 0.7%. The most frequent tumor is lymphoreticular malignancy. We report a case of hepatocellular carcinoma (HCC) in a 13-year-old boy with XLA, after probable maternal transmission of hepatitis B virus. The authors consider that the vertical transmission of hepatitis B virus might play an important role in the development of HCC in a child with XLA who could not eliminate hepatitis B virus effectively.
Adolescent
;
Agammaglobulinemia*
;
B-Lymphocytes
;
Carcinoma, Hepatocellular*
;
Child*
;
Hepatitis B virus*
;
Hepatitis B*
;
Hepatitis*
;
Humans
;
Male
;
Prevalence
7.Diagnostic significance of serum A and B glycosyltransferase assay for the classification of ABO subgroups.
Dong Hee WHANG ; Bok Yeun HAN ; Tae Hee HAN ; Hyun Jin JUNG ; Kyou Sup HAN
Korean Journal of Blood Transfusion 1999;10(1):27-33
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 O RBCs into A or B cells. RESULTS: Determination of serum ABO transferase activity was useful in the identification of Ael (3 cases), B3 (2 cases), Bm (1 case), Am (1 case), Bx (1 case), O 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.
ABO Blood-Group System
;
Adsorption
;
Agammaglobulinemia
;
B-Lymphocytes
;
Classification*
;
Galactose
;
Phenotype
;
Saliva
;
Seoul
;
Transferases
8.Pure Red Cell Aplasia Associated with Good Syndrome.
Masayuki OKUI ; Takashi YAMAMICHI ; Ayaka ASAKAWA ; Masahiko HARADA ; Hirotoshi HORIO
The Korean Journal of Thoracic and Cardiovascular Surgery 2017;50(2):119-122
Pure red cell aplasia (PRCA) and hypogammaglobulinemia are paraneoplastic syndromes that are rarer than myasthenia gravis in patients with thymoma. Good syndrome coexisting with PRCA is an extremely rare pathology. We report the case of a 50-year-old man with thymoma and PRCA associated with Good syndrome who achieved complete PRCA remission after thymectomy and postoperative immunosuppressive therapy, and provide a review of the pertinent literature.
Agammaglobulinemia
;
Humans
;
Middle Aged
;
Myasthenia Gravis
;
Paraneoplastic Syndromes
;
Pathology
;
Red-Cell Aplasia, Pure*
;
Thymectomy
;
Thymoma
9.Diagnostic significance of serum A and B glycosyltransferase assay for the classification of ABO subgroups.
Kyou Sup HAN ; Tae Hee HAN ; Dong Hee WHANG ; Bok Yeon HAN ; Hyun Jin JUNG
Journal of the Korean Society for Microbiology 1999;34(1):27-34
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
10.A Case of Group O Losing Anti-B Selectively.
Seon Ho LEE ; Joseph JEONG ; Ui Suk JEONG ; Jai Ho WEE ; Tae Woo KIM ; Jeong Hwan BAI ; Geum Sook KANG ; Sung Ryul KIM ; Seog Woon KWON
Korean Journal of Blood Transfusion 2007;18(3):244-248
We report a case of group O losing anti-B selectively. A 25-year-old male donated blood; on the donor test an ABO discrepancy was noted, and a further evaluation study was performed. ABO genotyping with an allele specific polymerase chain reaction assay revealed O/O and DNA sequencing of exons 6 and 7 of the ABO gene showed O01/O02. The serum gammaglobulin level was decreased and only 0.2% CD19 pan-B positive lymphocytes were present in a subset of lymphocytes. In a previous donor study, anti-B of the patient was lost from a third donor study and was still not detected.
Adult
;
Agammaglobulinemia
;
Alleles
;
Exons
;
Humans
;
Lymphocytes
;
Male
;
Polymerase Chain Reaction
;
Sequence Analysis, DNA
;
Tissue Donors