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Case Report
Oncology
Targeted busulfan and fludarabine-based conditioning for bone marrow transplantation in chronic granulomatous disease
Hee Young Ju, Hyoung Jin Kang, Che Ry Hong, Ji Won Lee, Hyery Kim, Sang Hoon Song, Kyung-Sang Yu, In-Jin Jang, June Dong Park, Kyung Duk Park, Hee Young Shin, Joong-Gon Kim, Hyo Seop Ahn
Clin Exp Pediatr. 2016;59(Suppl 1):S57-S59.   Published online November 30, 2016

Chronic granulomatous disease (CGD) is a primary immunodeficiency disease caused by impaired phagocytic function. Hematopoietic stem cell transplantation (HSCT) is a definitive cure for CGD; however, the use of HSCT is limited because of associated problems, including transplantation-related mortality and engraftment failure. We report a case of a patient with CGD who underwent successful HSCT following a targeted busulfan and...

Immunology
Concomitant use of corticosteroid and antimicrobials for liver abscesses in patients with chronic granulomatous disease
Kyung-Sue Shin, Mu Suk Lee
Clin Exp Pediatr. 2016;59(4):196-201.   Published online April 30, 2016

Chronic granulomatous disease (CGD) is a rare inherited disorder caused by defective nicotinamide adenine dinucleotide phosphate oxidase enzyme and characterized by recurrent bacterial and fungal infections. Although liver abscess is a common manifestation of CGD, its management in CGD patients is not well-defined. In addition, the generalized guidelines for treating liver abscesses do not necessarily apply to CGD patients. Corticosteroids...

Original Article
Long-term outcome of patients with p22phox-deficient chronic granulomatous disease on Jeju Island, Korea
Hyun Sik Kang, Geol Hwang, Kyung-Sue Shin
Clin Exp Pediatr. 2015;58(4):129-135.   Published online April 22, 2015
Purpose

This study investigated the long-term clinical outcomes of patients with p22phox-deficient chronic granulomatous disease (CGD) on Jeju Island and retrospectively evaluated the effects of interferon-gamma (IFN-γ) prophylaxis.

Methods

The medical records of 15 patients with CGD were retrospectively reviewed. The efficacy of IFN-γ prophylaxis was evaluated by comparing the frequency of severe infections before and after starting continuous prophylaxis with IFN-γ.

Results

At the...

Case Report
A case report of chronic granulomatous disease presenting with aspergillus pneumonia in a 2-month old girl
Eun Lee, Seak Hee Oh, Ji Won Kwon, Byoung Ju Kim, Jinho Yu, Chan Jeoung Park, Soo Jong Hong
Clin Exp Pediatr. 2010;53(6):722-726.   Published online June 23, 2010

Chronic granulomatous disease (CGD) is an uncommon inherited disorder caused by mutations in any of the genes encoding subunits of the superoxide-generating phagocyte NADPH oxidase system, which is essential for killing catalase producing bacteria and fungi, such as Aspergillus species, Staphylococcus aureus, Serratia marcescens, Nocardia species and Burkholderia cepacia. In case of a history of recurrent or persistent infections, immune...

Original Article
TNF-α stimulated IL-8 and IL-10 expression in monocytes from patients with chronic granulomatous disease
Kyung-Sue Shin
Clin Exp Pediatr. 2008;51(10):1096-1101.   Published online October 15, 2008
Purpose : Patients with chronic granulomatous disease (CGD) have genetic mutations in a component of the NADPH oxidase enzyme that is necessary for the generation of the superoxide anion. The profound defect in innate immunity is reflected by the patients susceptibility to catalase-positive bacteria and fungi. In addition, CGD patients display signs of persistent inflammation, which is not associated only...
Case Report
A Case of Chronic Granulomatous Disease in which Enterococci were Observed in Culture
Kyunglae Cho, Jeonghee Kim, Eunjeong Kim, Churwoo Yoo, Chunsoo Kim, Unseok Nho, Chulkyu Kim
Clin Exp Pediatr. 2000;43(6):846-850.   Published online June 15, 2000
Chronic granulomatous disease(CGD) is a genetically inherited disorder caused by the failure of phagocytic cells to produce superoxide. It is characterized by frequent and uncontrollable infection which often lead to death in early childhood. The first clinical signs may be confined to skin and manifest themselves as abscesses, pyoderma, eczema or draining sinuses. The disease was first reported in 1957...
A Month-Old Infant with Chronic Granulomatous Disease Presented as Pulmonary Aspergillosis
SY Bae, JH Jung, KH Kim, YM Hong, HS Koo, JY Seoh, KS Hong, ES Kang, YS Won, JH Yoo
Clin Exp Pediatr. 2000;43(3):444-449.   Published online March 15, 2000
Chronic granulomatous disease(CGD) is an uncommon inherited disorder in which phagocytic cells fails to produce antimicrobial superoxide(O2-) due to NADPH oxidase deficiency. This disease can be easily screened by PMA(phobol myristate acetate)-activated nitroblue tetrazolium(NBT) test. Also the disease has features of chronic pyogenic infection, granuloma formation, anemia, hypergammaglobulinemia, and leukocytosis. Treatment uses antibiotics sensitive to specific organism, surgical drainage...
A Case of Chronic Granulomatous Disease Which is Improved by Interferon-γ Therapy
Jae Choon Kim, Soon Seong Park, Myung Ja Yun, Dong Hyuk Keum
Clin Exp Pediatr. 1997;40(7):1021-1025.   Published online July 15, 1997
Chronic granulomatous disease is a group of rare disorders characterized by a disease of oxidative metabolism of phagocytes with deficit of antimicrobial activity. Also, it has clinical features of chronic pyogenic infection, granuloma formation, hypergammaglobulinemia, anemia and leukocytosis. We experienced a case of chronic granulomatous disease with chronic recurrent infection, hypergammaglobulinemia and liver abscess. It was confirmed by modified NBT test. The result of patient' s...
A Case of Chronic Granulomatous Disease Associated with Pyogenic Liver Abscess
S.K. Lee, J.H. Kim, S.Y. Kim, E.T. Oh, H.J. Kang, K.S. Kim
Clin Exp Pediatr. 1996;39(4):585-590.   Published online April 15, 1996
We have experienced a case of chronic granulomatous disease associated with pyogenic liver abscess in 10 years old male Chronic granulomatous disease(CGD) is a hereditary disorder of neutrophil with defective killing and digesting of ingested catalase-positive bacteria or fungi in spite of normal phagocytosis. It was diagnosed by clinical finding and laboratory examination including NBT test. Pyogenic liver abscess in old...
Original Article
Clinical Study on Chronic Granulomatous Disease (CGD) -Occurrence in Siblings-
Yong Hoon Jun, Sung Won Yang, Dong Wook Kim, Hoan Jong Lee, Joong Gon Kim
Clin Exp Pediatr. 1994;37(6):794-803.   Published online June 15, 1994
To analyse the age of onset, etiologic microorgnisms, clinical manifestations, managements and genetic variation of chronic granulomatous disease, the authors reviewed four patients who were diagnosed as CGD at Department of Pediatrics, Seoul National Univesity Children's Hospital. They were siblings in relationship-two of them were brothers, the others wete brother-sister. The results were as follows; 1) Initial manifestations developed within 1...
Case Report
A Case of Chronic Granulomatous Disease
So Young Lee, Dae Chul Kim, Sung Hee Oh, Hahng Lee, Hyang Eun Sohn, Wha Soon Chung, Young Hyeh Ko, Moon Hyang Park
Clin Exp Pediatr. 1992;35(5):704-712.   Published online May 15, 1992
Chronic granulomatous disease (CGD) is a hereditary disorder of neutrophil with defective killing and digesting of ingested catalase-positive bacteria or fungi in spite of normal phagocytosis. Dissemination of ingested live organisms results in multiple granuloma in reticuloendothelial system and multiple abscesses manifested by recurrent pus-draining suppurative lymphadenopathy, subcutanous abscess, or impetiginous skin rahses, pheumonitis, and hepatosplenomegaly, ultimately resulting in death. Two...
A Case of Chronic Granulomatous Disease.
C H Park, M R Roh, S C Park, J O Park, D H Lee, S J Lee
Clin Exp Pediatr. 1984;27(11):1141-1146.   Published online November 30, 1984
Chronic granulomatous disease is characterized by an inability of patients* leukocytes to generate hydrogen peroxide and to kill non-peroxide forming bacteria, such as staphylococci, E. coli and serratiae etc. We experienced a case of chronic granulomatous disease with recurrent pyogenic infection. It was diagnosed by clinical findings and laboratory examinations including NBT test. Clin- ical findings showed persistent diarrhea since birth, recurrent bacterial pneumonia, perianal abscess,...