• 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.

• Korean Journal of Radiology
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• v.22 no.8
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• pp.1416-1435
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• 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
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• v.28 no.10
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• pp.928-931
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• 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
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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.

• 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.

• 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.

• Korean Journal of Veterinary Research
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• v.60 no.2
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• pp.85-88
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• 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
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• v.5 no.1
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• pp.39-41
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• 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
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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.