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Bronchiectasis

Kim C, Kim DG

The frequency of diagnosing bronchiectasis is increasing around the world. Cystic fibrosis is the most common inherited cause of bronchiectasis, but there is increasing recognition of significant numbers of patients...
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A Case of Meningococcal Sepsis and Meningitis with Complement 7 Deficiency in a Military Trainee

Sim SH, Heo JY, Kim EC, Choe KW

  • KMID: 2391845
  • Infect Chemother.
  • 2013 Mar;45(1):94-98.
Complement component 7 (C7) deficiency leads to the loss of complement lytic function, and affected patients show increased susceptibility to encapsulated organisms infection, especially Neisseria meningitidis. Recently, we have experienced...
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Pulmonary Features of Hyperimmunoglobulin E (Job's)syndrome

Min BJ, Shin JS, Lee IS, Shin YK

  • KMID: 2318398
  • Tuberc Respir Dis.
  • 2002 Jun;52(6):651-656.
Hyperimmunoglobulin E syndrome, otherwise known as Job's syndrome, is an immune disorder characterized by an abnormal elevation of the circulating immunoglobulin E level, and recurrent infections of the skin and...
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Two Cases of Cytomegalovirus Retinitis as a Manifestation of Good's Syndrome

Kim HI, Chang HH, Park GY, Kwon EG, Kim HH, Kim SW, Lee JM

  • KMID: 2263801
  • Korean J Med.
  • 2011 Aug;81(2):266-274.
Good's syndrome (GS) is a rare cause of acquired combined B- and T-cell immunodeficiency in adults. GS is also known as a rare form of paraneoplastic syndrome of thymoma that...
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A Case of Thymoma with Immunodeficiency (Good's Syndrome) with Disseminated Tuberculosis

Jung H, Sohn KM, Kim YS, Song CH, Sohn EH

Good's syndrome is the rare association of immunodeficiency with thymoma, characterized by hypogammaglobulinemia, depleted B-cells, diminished T-cells and inversion of the CD4/CD8 ratio. Thymectomy does not usually improve hypogammaglobulinemia; thus,...
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A Korean familial case of hereditary complement 7 deficiency

Kim MK, Lee KY, Lee JH

Meningococcal infections can be associated with abnormalities of the complement system, which contains 5 terminal complement proteins. Furthermore, deficiencies in 1 of these 5, complement component 7 (C7), leads to...
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Effective Treatment of Intractable Diarrhea Associated with Common Variable Immunodeficiency with Oral Budesonide

Jeon JH, Lim DH, Park CH, Kwon HC, Jung JY, Park KW, Lee SI

  • KMID: 2269320
  • Korean J Med.
  • 2014 May;86(5):637-640.
Common variable immunodeficiency (CVID) is the most common symptomatic primary antibody deficiency syndrome and has a high prevalence of gastrointestinal complications. We report the case of a 36-year-old male with...
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A Case of IgM Deficiency with B Cell Deficiency Detected by ABO Discrepancy in a Patient with Acute Osteomyelitis

Jung CL, Cha MK, Jun BH, Hong KS

ABO discrepancy refers to an inconsistency between red cell and serum typings and has various causes, including hypogammaglobulinemia. IgM deficiency is a rare disorder that may accompany several conditions such...
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Higher Morbidity of Monogenic Inflammatory Bowel Disease Compared to the Adolescent Onset Inflammatory Bowel Disease

Kim KY, Lee EJ, Kim JW, Moon JS, Jang JY, Yang HR, Ko JS

PURPOSE: Monogenic inflammatory bowel disease (IBD) patients do not respond to conventional therapy and are associated with a higher morbidity. We summarized the clinical characteristics of monogenic IBD patients and...
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Pulmonary Complications in Primary Immunodeficiency Diseases

Kim KH, Kim KJ, Lee TJ, Chun JK, Kim DS

  • KMID: 2314571
  • Pediatr Allergy Respir Dis.
  • 2007 Dec;17(4):372-383.
PURPOSE:Primary immunodeficiency diseases are disorders in which part of the body's immune system is missing or does not function properly due to intrinsic defects in the immune system. These patients...
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A case of granulomatous lung disease in a patient with Good's syndrome

Lee SH, Lee SM, Yang SC, Yoo CG, Kim YW, Shim YS, Han SK

Good's syndrome is extremely rare. This adult-onset condition is characterized by a thymoma with immunodeficiency, low B- and T-cell counts, and hypo-gammaglobulinemia. The initial clinical presentation is either a mass-lesion...
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Prevalence of Primary Immunodeficiency in Korea

Rhim JW, Kim KH, Kim DS, Kim BS, Kim JS, Kim CH, Kim HM, Park HJ, Pai KS, Son BK, Shin KS, Oh MY, Woo YJ, Yoo Y, Lee KS, Lee KY, Lee CG, Lee JS, Chung EH, Choi EH, Hahn YS, Park HY, Kim JG

This study represents the first epidemiological study based on the national registry of primary immunodeficiencies (PID) in Korea. Patient data were collected from 23 major hospitals. A total of 152...
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The Association between Asthma and Invasive Pneumococcal Disease: A Nationwide Study in Korea

Kwak BO, Choung JT, Park YM

The purpose of this study was to investigate the association between asthma and invasive pneumococcal disease (IPD) in Korea. A retrospective population-based cohort study was conducted using the Korean Health...
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Is Whole Exome Sequencing Clinically Practical in the Management of Pediatric Crohn's Disease?

Oh SH, Baek J, Kim KM, Lee EJ, Jung Y, Lee YJ, Jin HS, Ye BD, Yang SK, Lee JK, Seo EJ, Lim HT, Lee I, Song K

BACKGROUND/AIMS: The aim of this study was to identify the profile of rare variants associated with Crohn's disease (CD) using whole exome sequencing (WES) analysis of Korean children with CD...
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Lower Respiratory Tract Diseases Caused by Common Respiratory Viruses among Stem Cell Transplantation Recipients: A Single Center Experience in Korea

Hong KW, Choi SM, Lee DG, Cho SY, Lee HJ, Choi JK, Kim SH, Park SH, Choi JH, Yoo JH, Lee JW

PURPOSE: To describe the incidence, clinical courses, and risk factors for mortality of lower respiratory tract diseases (LRDs) caused by common respiratory viruses (CRVs) in stem cell transplantation (SCT) recipients. MATERIALS...
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