1.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
Journal of the Korean Pediatric Society 1992;35(5):704-712
No abstract available.
Granulomatous Disease, Chronic*
;
Trimethoprim-Sulfamethoxazole Combination
2.Disseminated Cutaneous Sporotrichosis with Fungal Sinusitis As An Initial Presentation of Underlying Myeloproliferative Neoplasm
Wei Hsi Chang ; Juliana Wai Theng Lee ; Soo Ching Gan ; Ting Guan Ng
Malaysian Journal of Dermatology 2022;48(Jun 2022):80-83
Summary
Sporotrichosis is a rare and chronic granulomatous subcutaneous mycotic infection caused by
a dimorphic fungus, Sporothrix schenckii. We describe a patient with disseminated cutaneous
sporotrichosis who was later diagnosed with myeloproliferative neoplasm and discuss the challenges
and importance in diagnosing this rare condition.
Sporotrichosis
;
Granulomatous Disease, Chronic
;
Myeloproliferative Disorders
3.Pathogens of first-episode pulmonary infection in 141 children with chronic granulomatous disease.
Hui LIU ; Shunying ZHAO ; Haiming YANG ; Jinrong LIU ; Hui XU ; Xiaolei TANG ; Yuelin SHEN ; Xiaoyan ZHANG ; Xiaohui WEN ; Yuhong ZHAO ; Ping CHU ; Huimin LI
Chinese Medical Journal 2024;137(4):502-504
4.Comparison of Various Detection Methods of Mycobacterium Species in Formalin-Fixed Paraffin-Embedded Tissue with Chronic Granulomatous Inflammation.
Hyun Seung LEE ; Hyoungnam LEE ; Soyoung IM ; Yun Su LEE ; Kyo Young LEE ; Yeong Jin CHOI
Korean Journal of Pathology 2010;44(3):259-266
BACKGROUND: To determine the most effective method for detecting mycobacteria in formalin- fixed paraffin-embedded (FFPE) tissue, we compared the results of Ziehl-Neelsen stain (ZNS) and mycobacterial culture with those of polymerase chain reaction (PCR) and real-time quantitative PCR (RQ-PCR). METHODS: We analyzed 54 cases diagnosed as chronic granulomatous inflammation. In all cases, ZNS and nested PCR using three different primers, IS6110, Mpb64 and IS6110/Rpobeta were done. RQ-PCR with the IS6110/Rpobeta primer was done in 51 cases. RESULTS: Mycobacteria were identified by ZNS in 15/54 (27.8%) cases. RQ-PCR had the highest sensitivity (80.0%) compared to PCR with IS6110 (73.3%), Mpb64 (60.0%) and IS6110/Rpobeta (73.3%). Specificity was higher in all PCR experiments (79.5-82.1%) than in RQ-PCR (69.4%) experiments. The false negative rate was lowest for RQ-PCR (20.0%) than for PCR with IS6110 (26.7%), Mpb64 (40.0%) and IS6110/Rpobeta (26.7%). The false positive rate was highest for RQ-PCR (30.6%) compared to PCR with IS6110 (20.5%), Mpb64 (17.9%) and IS6110/Rpobeta (20.5%). CONCLUSIONS: RQ-PCR had the highest sensitivity, and the lowest false negative rate, but it also had a higher false positive rate than PCR for detection of mycobacteria in FFPE tissues.
Granulomatous Disease, Chronic
;
Inflammation
;
Mycobacterium
;
Polymerase Chain Reaction
;
Sensitivity and Specificity
5.A Case of Palpebral Sarcoidosis Associated with Granulomatous Uveitis.
Journal of the Korean Ophthalmological Society 1990;31(6):819-823
Sarcoidosis is a chronic granulomatous disease of unknown cause characterized by widespread occurence of epithelioic cell. The authors experienced a case of sarcoidosis in 34-year-old female who has lacrimal gland enlargement, severe subcutaneous nodules and bilateral granulomatous uveitis. Histopathologic examination of the subcutaneous nodule of lid revealed sarcoidosis. In the course of corticosteroid therapy, subcutaneous nodules and enlarged lacrimal gland subsided gradually.
Adult
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Female
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Granulomatous Disease, Chronic
;
Humans
;
Lacrimal Apparatus
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Sarcoidosis*
;
Uveitis*
6.Concomitant use of corticosteroid and antimicrobials for liver abscesses in patients with chronic granulomatous disease.
Korean Journal of Pediatrics 2016;59(4):196-201
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 are commonly used to control granulomatous complications, such as inflammatory gastrointestinal and genitourinary lesions, in patients with CGD, Corticosteroids have also been used in combination with antimicrobials to treat refractory infections in patients with CGD. Because corticosteroids are capable of suppressing symptomatic inflammation, all potential infections must be adequately controlled prior to corticosteroid initiation. We report 3 typical CGD cases with liver abscesses refractory to conventional treatments that were successfully treated with the concomitant use of corticosteroid and antimicrobials. It remains unclear whether corticosteroid therapy is required for liver abscesses in CGD refractory to conventional treatments. However, based on our observations, use of corticosteroids in combination with optimal antimicrobials should be considered for refractory liver abscesses in CGD.
Adrenal Cortex Hormones
;
Granulomatous Disease, Chronic*
;
Humans
;
Inflammation
;
Liver Abscess*
;
Liver*
;
NADP
;
Oxidoreductases
7.A Case of Acute Poststreptococcal Glomerulonephritis Associated with Chronic Granulomatous Disease.
Hyung Tae KIM ; Chong Guk LEE ; Jung Wook SEO ; Han Seong KIM
Journal of the Korean Society of Pediatric Nephrology 2003;7(2):217-222
Chronic granulomatous disease(CGD) is a rare inherited disorder of phagocytic cells which results in a susceptibility to infections by catalase-positive bacteria and fungi, as well as granuloma formation. And acute poststreptococcal glomerulonephritis(APSGN) is one of the most common glomerular lesions of gross hematuria in children. We experienced a case of APSGN accompanied with CGD presenting with a liver granuloma.
Bacteria
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Child
;
Fungi
;
Glomerulonephritis*
;
Granuloma
;
Granulomatous Disease, Chronic*
;
Hematuria
;
Humans
;
Liver
;
Phagocytes
8.A Case of Acute Poststreptococcal Glomerulonephritis Associated with Chronic Granulomatous Disease.
Hyung Tae KIM ; Chong Guk LEE ; Jung Wook SEO ; Han Seong KIM
Journal of the Korean Society of Pediatric Nephrology 2003;7(2):217-222
Chronic granulomatous disease(CGD) is a rare inherited disorder of phagocytic cells which results in a susceptibility to infections by catalase-positive bacteria and fungi, as well as granuloma formation. And acute poststreptococcal glomerulonephritis(APSGN) is one of the most common glomerular lesions of gross hematuria in children. We experienced a case of APSGN accompanied with CGD presenting with a liver granuloma.
Bacteria
;
Child
;
Fungi
;
Glomerulonephritis*
;
Granuloma
;
Granulomatous Disease, Chronic*
;
Hematuria
;
Humans
;
Liver
;
Phagocytes
9.A Case of Esophageal Obstruction Complicated in a Patient with Chronic Granulomatous Disease: Esophageal obstruction in Chronic Granulomatous Disease.
Ji Soo KIM ; Jinsol HWANG ; Young Hun CHOI ; Woo Sun KIM ; Joong Gon KIM
Korean Journal of Pediatric Infectious Diseases 2014;21(1):53-58
Chronic granulomatous disease (CGD) is an inherited immunodeficient disease characterized by recurrent infections and granuloma formation. Granulomatous obstruction of esophagus is one of the rare complications of CGD. The use of steroids and antimicrobials for esophageal obstruction by granuloma in CGD patients has been controversial due to the possibility of concomitant inapparent infection. We report a case of esophageal obstruction in an 8-year-old CGD patient showing the poor response to antibiotics therapy. However, dramatic improvement of symptoms and radiologic findings of esophageal obstruction were achieved after steroid therapy. One month after discontinuation of steroid, esophageal obstruction recurred and the patient was re-treated with steroid. After that time, he experienced one more recurrence of esophageal obstruction. This symptom subsided after antibiotics therapy without steroid and he has been followed up to the present without further relapse.
Anti-Bacterial Agents
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Child
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Esophageal Stenosis
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Esophagus
;
Granuloma
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Granulomatous Disease, Chronic*
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Humans
;
Recurrence
;
Steroids
10.Chronic Granulomatous Disease on Jeju Island, Korea.
Journal of Genetic Medicine 2013;10(1):1-6
Chronic granulomatous disease (CGD) is a rare inherited disorder of a defective NADPH oxidase enzyme, resulting in very low or no production of superoxide and subsequent reactive oxygen species. Consequently, patients with CGD are highly susceptible to severe bacterial and fungal infections. CGD is a genetically heterogeneous disease caused by defects in any one of the genes encoding the NADPH oxidase components. CGD generally affects about 3-4 per 1,000,000 individuals; thus, it is surprising that the prevalence of CGD on Jeju Island is 34.3 per 1,000,000 individuals. At present, 20 patients with CGD from 14 unrelated families on Jeju Island have been identified; nine males and 11 females. All patients with CGD tested on Jeju Island had an identical and homozygous mutation (c.7C>T in CYBA, p.Q3X in p22phox). Therefore, all patients were autosomal recessive form of CGD. This strongly suggests that the unique and identical mutation in CYBA may be inherited from a common proband. Using mutation-specific primers to detect the mutated allele in CYBA, the frequency of subjects carrying a mutated allele was 1.3% of enrolled subjects from Seogwipo City. Further studies are necessary to elucidate how frequently this mutant allele occurs in the population on Jeju Island. Additionally, it is important to construct a national registry system to understand the pathophysiology of CGD and develop a strategy for long-term therapy.
Alleles
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Female
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Granulomatous Disease, Chronic
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Humans
;
Korea
;
Lifting
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Male
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NADPH Oxidase
;
Prevalence
;
Reactive Oxygen Species
;
Superoxides