• Childhood Kidney Diseases
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• v.11 no.1
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• pp.118-125
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• 2007

Wegener's granulomatosis(WG) is a necrotizing granulomatous small vessel vasculitis with a clinical predilection for involvement of the upper airways, lungs and kidneys. The disease usually manifests in adults between 25 and 50 years of age, but it can also rarely occur-in childhood with some features different from those of adults. WG may be easily overlooked in young patients by misinterpretion of the symptoms as caused by an infectious disease of the respiratory tract. Delayed diagnosis and treatment of the disease may cause more rapid progression of the glomerulonephritis to end stage renal disease. We report a boy who was diagnosed with WG with involvement of multiple organs at 13 years of age.

• Tuberculosis and Respiratory Diseases
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• v.41 no.2
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• pp.152-157
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• 1994

Histiocytosis-X is a tenn used to define three diseases with similar morphologic characteristics : Letterer-Siwe diseae, Hand-Schuller-Christian disease and Eosinophilic granuloma. In general, they differ in terms of their age of onset, severity of clinical course and site of involvement. Eosinophilic granuloma typically is seen in young adults. Eosinophilic granuloma is diagnosed in the presense of diffuse pulmonary infiltrate, bony involvement. However, the pulmonary radiologic findings of eosinophilic granuloma are variable accordinary to stage of disease. therefore pathologic diagnosis of involving site is essential for confirmative diagnosis of eosinophilic grananuloma. Pathologically. the three disease are characterized by granulomatous infiltration of alveolar septa and bronchial walls and often involvement of bone. The hallmark of this disease is proliferation of the Langerhans' cell. The identifying feature is the X-body or Birbeck granule that is present in Langerhans' cells and histiocytic cells found in the lung of EG patient. We report a case of bilatera1, recurrent and spontaneous pneumothoraces in a 21 year old man with pulmonary histiocytosis-X, which is confirmed by eosinophilc granuloma in bone marrow biopsy and ultrastructural examination in cells obtained from BAL.

• Pediatric Infection and Vaccine
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• v.29 no.3
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• pp.166-172
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• 2022

Blau syndrome is a systemic autoinflammatory disease presenting with non-caseating granulomatous dermatitis, chronic uveitis, and arthritis. It is caused by a gain-of-function variant of the nucleotide-binding oligomerization domain protein 2 gene, which leads to the overactivation of inflammatory cytokines and eventually causes autoinflammation. Since the symptoms of Blau syndrome are nonspecific and usually do not appear simultaneously, it is challenging to differentiate Blau syndrome from other inflammatory disorders. This is a case report of a 13-month-old boy who had suffered from recurrent skin rash and fever. The patient was previously misdiagnosed as refractory Kawasaki disease twice and was treated with intravenous immunoglobulin and systemic glucocorticoid, which only resulted in transient improvement of the symptoms. He was eventually diagnosed with Blau syndrome.

• Clinical and Experimental Pediatrics
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• v.51 no.10
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• pp.1096-1101
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• 2008

Purpose : Patients with chronic granulomatous disease (CGD) have genetic mutations in a component of the NADPH oxidase enzyme that is necessary for the generation of the superoxide anion. The profound defect in innate immunity is reflected by the patients susceptibility to catalase-positive bacteria and fungi. In addition, CGD patients display signs of persistent inflammation, which is not associated only with deficient superoxide anion production. The aim of this study was to elucidate the cytokine responses in CGD patients after $TNF-{\alpha}$ stimulation. Methods : Heparinized blood samples were collected from 8 CGD patients and 10 healthy volunteers. Monocytes ($1{\times}10^6cell/well$) isolated by the magnet cell isolation system were incubated with a constant amount of $TNF-{\alpha}$ (10 ng/mL) at $37^{\circ}C$ for 6 h. Incubated cells were harvested at 60-min intervals for IL-8 and IL-10 mRNA analysis, and the supernatant was collected at the same intervals to determine IL-8 and IL-10 expression. Monocytes from healthy volunteers were also incubated with antioxidants followed by $TNF-{\alpha}$ stimulation for IL-8 and IL-10 expression. Results : In CGD patients, a high expression of IL-8 together with a significantly higher IL-10 expression than in the healthy controls was seen after $TNF-{\alpha}$ stimulation. Moreover, normal monocytes treated with antioxidants exhibited increased IL-8 responses. Conclusion : The absence of phagocyte-derived reactive oxidants in CGD might be associated with a dysregulated production of pro- and antiinflammatory cytokines. Additional research related to reactive oxidants is needed to clarify the role of cytokines in CGD patients.

• Korean Journal of Veterinary Service
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• v.42 no.1
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• pp.39-42
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• 2019

Tetanus is an acute, often fatal, and infectious disease of all species of domestic animals caused by the neurotoxin of Clostridium tetani (C. tetani). This disease is usually known to develop after microbial contamination in the deep or penetrating wound sites. In February 2017, a farmer who was raising 76 cows injected foot and mouth disease vaccine to three or more cows with one syringe. Their clinical symptoms were observed 2 to 16 days after the vaccination. The initial symptoms were stiffness, rigidity of the neck and limbs, pricked ears, and prolapse of the third eyelid. Subsequently, there was recumbency with extension of the limbs, convulsions and opistotonus and the affected 20 cows were all died. Two dead cows were submitted to Animal and Plant Quarantine Agency for disease diagnosis. At necropsy, a focal edematous abscess of 15 to 20 cm in diameter was grossly observed in the subcutaneous and intramuscular tissue of scapular region and filled with a large amount of greenish pus. The feed was full in oral cavity and slightly observed in the trachea and lobes of lung. Histopathologically, focal granulomatous nodules with eosinophilic materials in the tissue were observed. In the lung, aspiration pneumonia and gram (+) bacteria were seen. The C. tetani was isolated in samples anaerobically cultured using reinforced clostridial medium and identified by PCR. To our knowledge, no previous outbreak of tetanus in cattle has affected such a high number of animals; neither has it been associated with misuse of the same syringe and needle to administer multiple individuals.

• Tuberculosis and Respiratory Diseases
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• v.44 no.5
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• pp.1146-1157
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• 1997

Fibrosing mediastinitis is a rare disease which is characterized by excessive fibrosis of mediastinum and symptoms caused by compression and obstruction of mediastinal structures. Although the pathogenesis of this disease is unknown, granulomatous infection is cinsidered to be the most common cause of this disease. Histoplasmosis is the most common etiology, especially in the endemic areas in United States. Tuberculosis is another etiology of fibrosing mediastinitis. We experienced two cases of fibrosing mediastinitis associated with tuberculous infection.

• Asian Pacific Journal of Cancer Prevention
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• v.14 no.12
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• pp.7257-7260
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• 2013

To determine the pattern of disease in patients presenting with breast lumps and to determine the sensitivity and specificity of fine needle aspiration cytology of benign and malignant diseases as a diagnostic tool by correlating with histopathology findings. This retrospective study was carried out in the Department of Pathology, Maharaja Agrasen Medical College, Agroha, from Jan 2008 to April 2012. Fine needle aspiration cytology was performed on 370 cases and out of these 52 cases were received in the Department for histopathological examination. Fibroadenoma was the most common disease encountered, in 88 (24%), with a peak incidence in second and third decade of life. Fibrocystic disease was second, being common in the third and fourth decades of life. Peak incidences of duct ectasia, granulomatous and tubercular mastitis were seen in the third decade. Gynaecomastia showed two peak incidences in second and sixth decades of life. Out of total 370 cases undergoing fine needle aspiration, benign cases were 316 (85.4%), malignant and suspicious were 54 (14.6%) and 10 (2.70%) respectively. Out of total 22 histological confirmed malignant lesions 19 were interpreted as malignant cytologically while two as suspicious and one as benign. All thirty histologically confirmed benign cases were diagnosed as benign cytologically. The sensitivity, specificity, positive and negative predictive values were 98%, 100%, 100% and 96.4% respectively. FNA cytology is highly accurate for diagnosis of breast masses. However, the clinician should correlate FNA cytological results with physical examination and imaging findings to prevent false negative and false positive events and to obtain optimal management of their patients.

• Journal of Korean Neurosurgical Society
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• v.30 no.6
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• pp.777-780
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• 2001

Cholesterol granuloma of frontal bone is a rare disease which usually occurs at the lateral part of the supraorbital ridge. This expanding lesion grows slowly and extends into the orbit and anterior cranial fossa. The most common symptom is proptosis. This granuloma is composed of a granulomatous reaction surrounding cholesterol crystals. Surgical treatment involves aspiration of contents and stripping or curettage of the lining which is highly successful. We experienced a case of cholesterol granuloma of frontal bone with huge intracranial extension, which was cured by surgical removal. The clinical features, radiologic, and pathologic finding were discussed and the pertinent literatures were reviewed.

• Archives of Plastic Surgery
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• v.37 no.5
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• pp.708-711
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• 2010

Purpose: The sheath of tendon is uncommon site of tuberculous involvement as compared to other parts of the body. Especially, tuberculous tenosynovitis affecting flexor tendon of the hand is a rare condition. In recent years, furthermore, the incidence of tuberculosis is increasing in our country. Tuberculous tenosynovitis is a chronic, slowly destructive disease, which is difficult to diagnosis before operation, but can be definitively diagnosed by the pathologic microscopic examination. Early detection and surgical excision combined with antituberculous medication is important. We report a rare case of tuberculous tenosynovitis of the flexor tendon of the hand. Method: A 12-year-old woman presented with a painless, nontender mass on palmar side between distal interphalangeal joint and proximal area of metacarpophalangeal joint of the left third finger. We had surgical excision of the involved flexor tendon sheath and studied histopathologically. Result: The histopathological findings were chronic granulomatous inflammation with caseating necrosis consistent with tuberculosis. We started antituberculous medication. Conclusion: Tuberculous tenosynovitis is a rare condition, especially involving on the flexor tendon of the hand. But because of increasing tendency of tuberculosis, it is important to differentiate it from other tumors of the hand.

• Clinical and Experimental Pediatrics
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• v.49 no.6
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• pp.696-699
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• 2006

Tolosa-Hunt syndrome is a rare disease in children characterized by dull, persistent pain around the affected eye and ophthalmoplegia caused by granulomatous inflammation in the cavernous sinus, superior orbital fissure, or orbit. Although spontaneous remission can occur, corticosteroids frequently have a dramatic response; however, recurrence can transpire after complete remission. We report an 11-year-old girl with Tolosa-Hunt syndrome who responded to corticosteroid promptly, without complications, but suffered three recurrences of headache and retro-orbital pain and required maintenance on a low dose of steroid.