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Effect of cultured thymic epithelium transplantation in a patients with severe combined immunodeficiency

Yun SK, Cha BH, Jeoun WS, Kim DS

  • KMID: 1690862
  • J Korean Pediatr Soc.
  • 1992 Oct;35(10):1443-1448.
No abstract available.
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Effect of interferon-gamma treatment on interstitial pneumonia in a patient with severe combined immunodeficiency

Kim KH, Kang HY, Kim DS

In severe combined immunodeficiency disease, both T and B cell functions are diminished or absent and affected usually succumb to overwhelming infection within the first year of life. We are...
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Disseminated BCG Infection in a patient with Severe Combined Immunodeficiency

Han TI, Kim IO, Kim WS, Yeon KM

Disseminated mycobacterial infection after bacillus Calmette-Guerin (BCG)vaccination is a very rare disorder, occurring mostly in patients with immunologic deficiency. We report a case of disseminated BCG infection in a 16-month-old...
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A Case of Omenn Syndrome in a Patient Treated for Chronic Diarrhea and Severe Exfoliative Erythrodermia

Yum MS, Lee SY, Kim HB, Yoo HW, Ghim T, Hong SJ

  • KMID: 2314377
  • Pediatr Allergy Respir Dis.
  • 2005 Jun;15(2):174-179.
Omenn syndrome (OS) is a peculiar, autosomal recessive severe combined immunodeficiency (SCID) associated with early-onset, generalized, exudative erythrodermia, lymphadenopathy, hepatosplenomegaly, hypereosinophilia, elevated serum IgE, and normal to highly activated, yet...
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A Case of Chronic Intractable Diarrhea with IgA, IgG2 and IgG4 Deficiency

Ahn SR, Kim YM, Nam SO, Park JH, Lee CH

  • KMID: 2275146
  • Korean J Pediatr Gastroenterol Nutr.
  • 2001 Sep;4(2):243-248.
In most cases, acute diarrhea in childhood heals spontaneously, but it may become the form of chronic diarrhea in immunodeficient children and then cause weight loss, dehydration, malabsorption and malnutrition. The immunodeficient...
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Thymic epithelial cells of severe combined immunodeficiency (SCID) mice

Lee I, Yu E, Ikehara S

To characterize thymic epithelial cells of SCID (severe combined immunodeficiency) mice in comparison with those of Balb C mice, we did an immunohistochemical study using cortical and medullary epithelial cell...
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The Complex Surgical Management of the First Case of Severe Combined Immunodeficiency and Multiple Intestinal Atresias Surviving after the Fourth Year of Life

Guana R, Garofano S, Teruzzi E, Vinardi S, Carbonaro , Cerrina A, Morra I, Montin D, Mussa A, Schleef J

Severe combined immunodeficiency (SCID) is a life-threatening syndrome of recurrent infections and gastrointestinal alterations due to severe compromise of T cells and B cells. Clinically, most patients present symptoms before...
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A Clinical Study of Primary Immunodeficiency Disease in a Single Center in Seoul from 1996 to 2004

Lee JH, Sohn WY, Park HY, Hwang SJ, Seo WH, Kim SJ, Ahn SH, Jo EK, Ahn KM, Lee SI

  • KMID: 2314398
  • Pediatr Allergy Respir Dis.
  • 2005 Dec;15(4):368-380.
PURPOSE: Although primary immunodeficiency disorders are relatively rare, early diagnosis provides the opportunity to reduce morbidity and mortality. The aim of this study was to investigate disease distribution, clinical manifestations,...
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Adenosine Deaminase Activities in Lymphocytes of Leprosy Patients

Kim YP, Choi YS

  • KMID: 1678740
  • Korean J Dermatol.
  • 1983 Aug;21(4):395-399.
Leprosy is an infectious diseases caused by Mycobacterium leprae. It is considered to be manifested in person with an impaired. immune system and is divided into two polar forms; the...
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X-linked Severe Combined Immunodeficiency Syndrome: The First Korean Case with gamma c Chain Gene Mutation and Subsequent Genetic Counseling

Jo EK, Kumaki S, Wei D, Tsuchiya S, Kanegane H, Song CH, Noh HY, Kim YO, Kim SY, Chung HY, Kim YH, Kook H

X-linked severe combined immunodeficiency (X-SCID) is a rare, life-threatening immune disorder, caused by mutations in the gamma c chain gene, which encodes an essential component of the cytokine receptors for...
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The First Newborn Screening Study of T-Cell Receptor Excision Circle and κ- Deleting Recombination Excision Circle for Severe Combined Immunodeficiency in Korea: A Pilot Study

Son S, Kang JM, Kim JM, Sung S, Kim YS, Lee H, Kim BR, Lee YK, Ko SY, Shin SM, Kim YJ

PURPOSE: Severe combined immunodeficiency (SCID) is the most serious form of primary immunodeficiency. Infants with SCID are susceptible to life-threatening infections. To establish newborn screening for SCID in Korea, we...
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Use of Stem Cell in Fetal Therapy: Current Status and Future Perspectives

Lee SW

During the past decades, there has been a great evolution in the field of fetal therapy for congenital defects. Prenatal screening or diagnostic methods including non-invasive and invasive methods and...
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Pneumocystis jirovecii pneumonia in pediatric patients: an analysis of 15 confirmed consecutive cases during 14 years

Kim KR, Kim JM, Kang JM, Kim YJ

PURPOSE: Pneumocystis jirovecii pneumonia occurs in various immunocompromised patients. Despite the prophylaxis strategies in clinical practice, certain patients develop P. jirovecii pneumonia. This study was performed to investigate pediatric cases...
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Adoptive transfer of Porphyromonas gingivalis heat shock protein epitope-specific T-cell lines into SCID mice in experimental atherosclerosis

Choi J, Witztum J

Bacterial heat shock protein has been one of the components that are responsible to induce autoimmune disease mechanisms in the pathogenesis of atherosclerosis due to high level of homology in...
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Flow Cytometry for the Diagnosis of Primary Immunodeficiency Diseases: A Single Center Experience

Kwon WK, Choi S, Kim HJ, Huh HJ, Kang JM, Kim YJ, Yoo KH, Ahn K, Cho HK, Peck KR, Jang JH, Ki CS, Kang ES

PURPOSE: While there is an urgent need for diagnosis and therapeutic intervention in patients with primary immunodeficiency diseases (PIDs), current genetic tests have drawbacks. We retrospectively reviewed the usefulness of...
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Leukemic Phase of Mantle Cell Lymphoma, Blastic Variant

Jeon HJ, Lee MJ, Park YH, Chung CH

  • KMID: 2143354
  • Korean J Hematol.
  • 2000 Nov;35(3-4):292-296.
A case with mantle cell lymphoma, blastic variant, involving the peripheral blood in a 78- year-old female was described. The circulating blast-like cells suggested the possibility of acute leukemia. The...
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HR-1 Mice: A New Inflammatory Acne Mouse Model

Jang YH, Lee KC, Lee SJ, Kim DW, Lee WJ

BACKGROUND: There is no appropriate in vivo animal model that reflects the inflammatory response of human acne. OBJECTIVE: This study investigated the effect of Propionibacterium acnes on the development of inflammatory...
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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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CRISPR/Cas9-mediated knockout of Rag-2 causes systemic lymphopenia with hypoplastic lymphoid organs in FVB mice

Kim JI, Park JS, Kim H, Ryu SK, Kwak J, Kwon E, Yun JW, Nam KT, Lee HW, Kang BC

Recombination activating gene-2 (RAG-2) plays a crucial role in the development of lymphocytes by mediating recombination of T cell receptors and immunoglobulins, and loss of RAG-2 causes severe combined immunodeficiency...
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Safety and Biodistribution of Human Bone Marrow-Derived Mesenchymal Stromal Cells Injected Intrathecally in Non-Obese Diabetic Severe Combined Immunodefi ciency Mice: Preclinical Study

Quesada MP, García-Bernal D, Pastor D, Estirado A, Blanquer M, García-Hernández AM, Moraleda J, Martínez

BACKGROUND: Mesenchymal stromal cells (MSCs) have potent immunomodulatory and neuroprotective properties, and have been tested in neurodegenerative diseases resulting in meaningful clinical improvements. Regulatory guidelines specify the need to perform...
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