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

• Korean Journal of Radiology
• /
• 제22권8호
• /
• pp.1416-1435
• /
• 2021

Non-infectious granulomatous lung disease represents a diverse group of disorders characterized by pulmonary opacities associated with granulomatous inflammation, a relatively nonspecific finding commonly encountered by pathologists. Some lesions may present a diagnostic challenge because of nonspecific imaging features; however, recognition of the various imaging manifestations of these disorders in conjunction with patients' clinical history, such as age, symptom onset and duration, immune status, and presence of asthma or cutaneous lesions, is imperative for narrowing the differential diagnosis and determining appropriate management of this rare group of disorders. In this pictorial review, we describe the pathologic findings of various non-infectious granulomatous lung diseases as well as the radiologic features and high-resolution computed tomography imaging features.

• Journal of Chest Surgery
• /
• 제28권10호
• /
• pp.928-931
• /
• 1995

Sarcoidosis is a granulomatous disease of unknown etiology. It frequently involves thoracic lymph node and pulmonary parenchyme characterized by non-caseating granulomas and varying degrees of fibrosis. We have experienced a pulmonary sarcoidosis in the left lower lobe of the lung in a 40 year old man who underwent left lower lobectomy by video-assisted thoracic surgery.

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

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

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

• Clinical and Experimental Pediatrics
• /
• 제59권4호
• /
• pp.196-201
• /
• 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
• /
• 제10권1호
• /
• pp.1-6
• /
• 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.

• 대한수의학회지
• /
• 제60권2호
• /
• pp.85-88
• /
• 2020

A 12-month-old domestic rabbit died suddenly without specific clinical signs. Gross examination revealed irregular yellowish hepatic nodules with pus in the submandibular muscles, lungs, uterus, and small intestines. Histopathological examination of the liver showed granulomatous inflammation with acid-fast-positive bacteria. Mycobacterium bovis SB1040 was identified by polymerase chain reaction and spoligotyping, and Pasteurella multocida was isolated from the multiple lesions. This report demonstrates the pathological features of rare bovine tuberculosis (bTB) in a domestic rabbit, the first case in the Republic of Korea. To ensure public safety, we recommend routine monitoring of rabbits to control the incidence of bTB.

• Annals of Clinical Neurophysiology
• /
• 제5권1호
• /
• pp.39-41
• /
• 2003

Sarcoidosis is a multisystemic granulomatous disease of unknown etiology ogenesis, and most frequently presented with bilateral hilar lymphadenopathy, pulmonary infiltration, skin and eye lesion. However, neurological involvement including peripheral neuropathy is relatively rare. We report a patient who had sensorimotor polyneuropathy without other systemic symptoms or organ involvements frequently reported in sarcoidosis. Laboratory investigation suggestive of sarcoidosis lead to sural nerve biopsy for confirmation, which demonstrated noncaseating granulomatous changes. Sarcoidosis shoud be included in the differential diagnosis in subacute polyneuropathy even if there is no usual symptoms or signs suggestive of the systemic disease.

• Clinical and Experimental Pediatrics
• /
• 제53권6호
• /
• pp.722-726
• /
• 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.