• Childhood Kidney Diseases
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• v.5 no.2
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• pp.213-218
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• 2001

Microscopic Polyangiitis(MPA) belongs to a spectrum of systemic vasculits, and particularly antineutrophil cytoplasmic autoantibodies(ANCA)-associated small-vessel vasculitis which is characterized by involvement of the lung and kidney. The diagnosis of MPA is often difficult to make, and delayed because of the variability of the clinical presentation. Renal biopsies have a very important diagnostic and prognostic value in MPA. We experienced a case of microscopic polyangiits which was confirmed by renal biopsy and positive serum perinuclear ANCA, associated with alveolar hemorrhage and gastrointestinal bleeding. We began methylprednisolone pulse therapy, combined with a low dose of cyclophosphamide and plasmapheresis therapy. ACE inhibitor and Ca channel blocker were used when proteinuria and hypertention developed. On admission, the patient's lab findings showed BUN l17 mg/dL, Cr 2.3 mg/dL, while on the 60th hospital day BUN/Cr values fell to 20.8 mg/dL / 1.6 mg/dL and though proteinuria persisted, the patient's condition was tolerable and is currently under observation on an out-patient basis. The last lab values were BUN 26 mg/dL / Cr 1.6 mg/dL. (J. Korean Soc Pediatr Nephrol 2001 ; 5 : 213-18)

• Childhood Kidney Diseases
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• v.12 no.1
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• pp.99-104
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• 2008

Microscopic polyangiitis(MPA) is a systemic necrotizing vasculitis that involves many organ systems including the skin, joint, kidneys, and lungs. In spite of early diagnosis and intensive care, the five-year actuarial patient and kidney survival rates are 65% and 55%. We experienced a case in 7-year-old girl of microscopic polyangiitis presenting with rapidly progressive glomerulonephritis which was confirmed by renal biopsy and positive serum perinuclear antineutrophil cytoplasmic autoantibodies(p-ANCA). The diagnosis of patients first renal biopsy was MPA, p-ANCA-associated crescentic glomerulonephritis. The patients second renal biopsy was done 5 years 6 months later since first renal biopsy, and pathologic diagnosis was chronic sclerosing glomerulonephritis, advanced, due to MPA. We began methylprednisolone pulse therapy, combined with a low dose of cyclophosphamide and plasmapheresis therapy. ACE inhibitor, angiotensin II receptor blocker, and cyclophosphamide were used until now and the patients current age is 14 years old. On admission, the patients laboratory findings showed BUN 117 mg/dL and Cr 2.3 mg/dL, while on the hospital day BUN and Cr values fell to 20.8 mg/dL and 1.6 mg/dL. But renal function was progressed to chronic failure with latest laboratory data BUN 51.7 mg/dL and Cr 3.2 mg/dL. ACE inhibitor, angiotensin II receptor blocker and small dose of immunosuppressant with close observation is the key to maintain the patient survival.

• 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.

• Journal of the Korean Society of Radiology
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• v.83 no.6
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• pp.1418-1425
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• 2022

Elderly patients with a history of chronic alcoholism presented to our hospital with episodes of melena, abdominal pain, and anemia. During admission, hemorrhagic cystic lesion at the pancreas was observed on abdominal CT. Transcatheter angiography confirmed active bleeding foci and arterial embolization was performed. After the procedure, the bleeding was resolved. The authors report two cases of hemosuccus pancreaticus and pancreaticocolic fistula associated with pancreatitis, a rare cause of gastrointestinal bleeding, treated with vascular intervention.

• Journal of the Korean Society of Radiology
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• v.82 no.4
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• pp.826-837
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• 2021

Immunoglobulin G4-related disease (IgG4-RD) is a chronic inflammatory condition involving multiple organs, including the salivary or lacrimal glands, orbit, pancreas, bile duct, liver, kidney, retroperitoneum, aorta, lung, and lymph nodes. It is histologically characterized by tissue infiltration with lymphocytes and IgG4-secreting plasma cells, storiform fibrosis, and obliterative phlebitis. In the thoracic involvement of IgG4-RD, mediastinal lymphadenopathy and perilymphangitic interstitial thickening of the lung are the most common findings. Peribronchovascular and septal thickening and paravertebral band-like soft tissue are characteristic findings of IgG4-RD. Other findings include pulmonary nodules or masses, ground-glass opacity, alveolar interstitial thickening, pleural effusion or thickening, mass in the chest wall or mediastinum, and arteritis involving the aorta and coronary artery. Radiologic differential diagnosis of various malignancies, infections, and inflammatory conditions is needed. In this review, we describe the imaging findings of IgG4-RD and the radiologic differential diagnoses in the thorax.

• Childhood Kidney Diseases
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• v.6 no.1
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• pp.114-119
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• 2002

Takayasu's arteritis(TA) is a chronic idiopathic vasculitis mainly involving the aorta and its main branches, such as brachiocephalic, carotid, subclavian, vertebral and renal arteries, as well as coronary and pulmonary arteries The clinical features usually reflect limb or organ ischemia resulting from gradual stenosis of involved arteries. We experienced a case of idiopathic Takayasu's arteritis with negative tuberculin test involving multiple main branch arteries at active stage without pulse. We treated this patient with combined therapy of steroid and azathioprine, with remission of disease activity. (J Korean Soc Pediatr Nephrol 2002 ;6 : 114-9)

• Journal of the korean academy of Pediatric Dentistry
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• v.36 no.4
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• pp.586-590
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• 2009

Leukocyte adhesion deficiency is rare inherited defect on phagocytic function resulting lack of leukocyte cell surface expression of $\beta2$ integrin molecule that are essential for leukocyte adhesion to endothelial cells and chemotaxis. Clinical features of patients with leukocyte adhesion deficiency type I include recurrent necrotic infection of the skin mucous membranes, and intestinal tract with septicemia, and omphalitis arising from delayed umbilical cord separation. Oral manifestations are severe progressive periodontitis with alveolar bone loss, periodontal pockets, and partial and total premature loss of the deciduous and permanent dentitions. We report a case of leukocyte adhesion deficiency type I in a 5-year-old child with severe periodontitis. In order to prevent local and systemic infection, we controlled periodontal disease with periodic oral prophylaxis. Oral swabs and blood cultures were perfomed for suspected infection, so that optimal measures were taken through the use of appropriate antibiotics.

• Journal of Veterinary Clinics
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• v.20 no.3
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• pp.274-277
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• 2003

The purpose of this study was to determine the range of resistive index(RI) of the cranial pancreaticoduodenal artery(cPDA) in the normal dog's and to assess the possibility of clinical application of RI for diagnosing a pancreatitis. Five patients with acute pancreatitis and 17 healthy adult beagle dogs were used. Duplex color Doppler ultrasonographic examination was performed by using a real-time scanner with 11-MHz linear-array transducer. The dogs were restrained in dorsal recumbency without the use of chemical agent. The mean RI of the normal pancreas(0.63$\pm$0.04) was significantly(t = 5.79, p = 0.001) different from acute pancreatitis dog(0.75$\pm$0.04). Duplex color Doppler ultrasonography was an useful technique for detecting and measuring RI of the cPDA. The evaluation of RI of the cPDA may be a valuable supportive diagnostic procedure for evaluating the pancreatitis or suspected in dogs.

• Journal of Chest Surgery
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• v.36 no.3
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• pp.215-218
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• 2003

A 65-year-old male was admitted to our hospital complaining of painful swelling of right sternocostoclavicular area. In the past history, he had no specific disease including trauma. After admission, chest CT and neck CT showed right empyema and right cervical abscess. Empyemectomy was performed through open thoracotomy and fistulous tract was detected on right parietal pleura and right sternocostoclavicular area. Osto-myelitis was also detected on right sternocostoclavicular area and removal of right cervical abscess, partial resection of proximal clavicle, resection of chondral portion of 1st rib, and partial resection of manubrium were performed. Empyema that extends from sternocostoclavicular osteomyelits, as in this case, is rare. Herein we report a case of loculated empyema with sternocostoclavicular osteomyelitis and neck abscess.

• Journal of Chest Surgery
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• v.38 no.9 s.254
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• pp.648-651
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• 2005

Calcific constrictive pericarditis is considered to be a nonspecific response to chronic inflammation. This disease has become rare, because the cause of it is usually tuberculosis, which has the tendency to decrease. Other possible causes of it include radiation, rheumatoid disorders, sarcoidosis, and trauma. Whatever the etiology, it can lead to cardiac tamponade by reducing cardiac diastolic filling. We report, herein, the case of a patient with heart failure by a calcific pericardial ring.