• Journal of Chest Surgery
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• 제31권11호
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• pp.1102-1105
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• 1998

소아기의 만성 육아종성 질환은 탐식세포의 선천적인 장애로 탐식된 세균이나 진균류를 살상하는 기능이 상실되고 따라서 반복적으로 화농성 질환이 재발하는 병이다. 폐는 만성 육아종성 질환에서 가장 흔히 침범되는 장기이며 폐감염은 이 질환에 의한 사망률의 약 절반을 차지한다. 만성 육아종성 질환에서 수술적 치료는 그 역할이 아직 명확하게 알려져 있지 않지만 폐감염의 원인 균에 대한 빠르고 정확한 진단이 적절한 항생제 선택에 매우 중요하기 때문에 폐엽절제술과 같은 수술적 접근법이 정확하고 빠른 진단을 통해 환자회복을 더 빠르게 하고 항생제의 침투를 더 쉽게 하여 환자에게 도움을 준다. 본 환아는 1개월된 남자환아로 만성 육아종성 질환으로 인한 폐감염으로 좌하엽절제술과 수술후 항생제, 항진균제와 면역글로불린, Interferon-gamma를 투여 받고 감염조절이 되었고 퇴원후 외래 추적관찰중이다.

• Childhood Kidney Diseases
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• 제7권2호
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• pp.217-222
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• 2003

저자들은 만성 육아종 질환에 동반된 APSGN을 경험하였기에 문헌 고찰과 함께 보고한다.

• Pediatric Infection and Vaccine
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• 제21권1호
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• pp.53-58
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• 2014

CGD 환자에서는 면역 체계의 결함으로 인한 반복적인 감염과 육아종 형성이 특징적이다. CGD 환자에서 육아종의 형성에 의한 식도 폐쇄는 매우 드문 합병증 중 하나로 CGD 환자의 육아종 형성에 의한 식도 폐쇄의 치료에 대해서는 불현성 감염이 동반될 가능성이 있어 스테로이드 치료와 항생제 치료에 대한 논란이 있어왔다. 저자들은 항생제 치료에 반응하지 않는 육아종 형성에 의한 식도 폐쇄 증상을 보인 8세 만성육아종질환 환자를 보고하는 바이다. 환자는 스테로이드 치료 이후에 임상적 증상과 영상 검사 상의 급격한 호전을 보였으나 스테로이드 치료를 중단한 지 한 달 뒤 환자의 식도 폐쇄 증상은 재발하였고, 이는 스테로이드를 재 투약 후 호전되었다. 스테로이드 중단 이후 한 차례 다시 재발하였으나, 항생제 치료 후 호전되었고 이후 추가적 재발 없이 경과 관찰 중이다.

• Clinical and Experimental Pediatrics
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• 제59권4호
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• pp.196-201
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• 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 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.

• Journal of Genetic Medicine
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• 제10권1호
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• pp.1-6
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• 2013

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 $p22^{phox}$). 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.

• Clinical and Experimental Pediatrics
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• 제53권6호
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• pp.722-726
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• 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 deficiency should be investigated. Particularly, in the case of uncommon infections such as aspergillosis in early life, CGD should be considered. We describe here a case of CGD that presented with invasive pulmonary aspergillosis in a 2-month-old girl. We confirmed pulmonary aspergillosis noninvasively through a positive result from the culture of bronchial alveolar lavage fluid, positive serological test for $Aspergillus$ antigen and radiology results. She was successfully treated with Amphotericin B and recombinant IFN-${\gamma}$ initially. Six weeks later after discharge, she was readmitted for pneumonia. Since there were infiltrates on the right lower lung, which were considered as residual lesions, voriconazole therapy was initiated. She showed a favorable response to the treatment and follow-up CT showed regression of the pulmonary infiltrates.

• Clinical and Experimental Pediatrics
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• 제58권4호
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• pp.129-135
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• 2015

Purpose: This study investigated the long-term clinical outcomes of patients with $p22^{phox}$-deficient chronic granulomatous disease (CGD) on Jeju Island and retrospectively evaluated the effects of interferon-gamma (IFN-${\gamma}$) prophylaxis. Methods: The medical records of 15 patients with CGD were retrospectively reviewed. The efficacy of IFN-${\gamma}$ prophylaxis was evaluated by comparing the frequency of severe infections before and after starting continuous prophylaxis with IFN-${\gamma}$. Results: At the time of the analysis, 14 patients were alive, with a median age of 14.3 years. The diagnosis of CGD was made at a median age of 2.4 years, and the median age at onset of severe infection was 0.3 years. Thirteen of the 15 patients had their first severe infection within the first year of life. The overall incidence of severe infection was 1.36 infections per patient-year; pneumonia, suppurative lymphadenitis, and skin and subcutaneous abscesses were the most common infections. Aspergillus species were the most frequently isolated microorganisms, present in 15.8% of isolates. IFN-${\gamma}$ did not significantly change the rate of severe infection. The survival rate for patients after 2 years of age was 93%; there was a prolonged survival plateau beyond the age of 2. Conclusion: Compared with cases of X-linked CGD reported in other studies, patients with CGD on Jeju Island did not show obviously different clinical manifestations, but they had a significantly higher survival rate. Further studies with a substantially longer period of observation, and with more patients under intensive surveillance are necessary to elucidate the prophylactic efficiency of IFN-${\gamma}$.

• Tuberculosis and Respiratory Diseases
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• 제66권4호
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• pp.309-313
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• 2009

There are several respiratory diseases that show chronic granulomatous inflammation for the histologic finding. Among them, sarcoidosis and tuberculosis are not easy to differentiate when the clinical and radiological features present similar patterns. The increasing incidence of nontuberculous mycobacteria pulmonary disease is making it more difficult for clinicians to arrive at a proper diagnosis. A 69 year old male patient visited our hospital with chronic cough as his chief compliant. His radiologic findings were multiple enlarged mediastinal lymphadenpathies with innumerable micronodules and multiple patch infiltrations. The spleen biopsy finding showed chronic granulomatous inflammation, and Mycobacterium avium was identified on the bronchoscopic culture. Because of these findings, we treated him with drugs for nontuberculous mycobacteria disease other than sarcoidosis. However, during the treatment, his symptoms and radiological features became aggravated. Thus, we reviewed the radiologic and pathologic findings and decided to treat him with steroid, which relieved his symptoms and improved the radiologic findings. We report here on a case of sarcoidosis that was initially misdiagnosed as nontuberculous mycobacteria pulmonary disease.

• Journal of Chest Surgery
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• 제8권2호
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• pp.153-158
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• 1975

Actinomycosis is a chronic suppurative granulomatous disease due to Actinomyces israelii characterized by multiple abscess and sinus tract formation with dense fibrous scarring. This disease, especially thoracic infection, is very rare in Korea so we are not famiIliar with to make diagnosis and treatment. Otherwise the unspecificity of the clinical symptoms and the lack of adequate examination recedure (as anaerobic fungus culture) are the causes of misdiagnosis. Thoracic actinomycosis is very similar to chronic infectious disease of the lung and chest or thoracic neoplasm. Recently we experienced a case of thoracic actinomycosis (bronchopulmonary) which had been confused with chronic lung abscess and pathologically confirmed as broncho-pulmonary actinomycosis. The purpose of this report is to review our experience more thorouly to enhance consideration of Artinomycosis.

• Medical Lasers
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• 제8권2호
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• pp.80-83
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• 2019

Necrobiosis lipoidica (NL) is a rare, idiopathic, chronic granulomatous inflammatory disease of collagen degeneration with the risk of ulceration. Many procedures have been proposed to treat this rare disease. In this study, we applied LDM^®-MED for the management of NL, and this condition in our patient was chronic and refractory to other therapeutic options. To the best of our knowledge, no study has explored treatment of NL using ultrasound. Our results suggest that application of LDM^®-MED seems to be an effective treatment option for NL. Long-term and systematic studies are needed to determine whether such application of LDM^®-MED will be an innovative and effective treatment option for NL and various kinds of chronic wounds.