• Journal of Chest Surgery
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• v.31 no.11
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• pp.1102-1105
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• 1998

Chronic granulomatous disease in childhood is a rare inheritable disorder of phagocytic cells in which defective production of the reactive intermediates of oxygen predisposes the patient to severe recuring pyogenic infections. The lung is the most common site of infection and pulmonary disease is the primary cause of death for greater than 50% of children with chronic granulomatous disease. Although the role of surgery in management of this disease remains undefined, rapid diagnosis of the underlying pulmonary problem is crucial to determine the most appropriate antimicrobial therapy and surgical techniques such as lobectomy of involved areas lead to more rapid recovery and thus allow the antibiotics to be more efficacious in these cases. We have treated a one month old male baby who had the chronic granulomatous disease with pulmonary infection. Wide surgical resection of the affected lobe and use of antibiotics and antifungals were carried out with good clinical results. He was well after the operation.

• Childhood Kidney Diseases
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• v.7 no.2
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• pp.217-222
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• 2003

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.

• Pediatric Infection and Vaccine
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• v.21 no.1
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• pp.53-58
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• 2014

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.

• Clinical and Experimental Pediatrics
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• v.59 no.4
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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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• v.10 no.1
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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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• v.53 no.6
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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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• v.58 no.4
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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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• v.66 no.4
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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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• v.8 no.2
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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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• v.8 no.2
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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.