• Title/Summary/Keyword: Necrotizing vasculitis

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A Case of Wegener's Granulomatosis that Presented as a Single Lung Mass (단일 폐종괴로 발견된 Wegener 육아종증 1예)

  • Oh, In Jae;Jeong, Jong Pil;Kim, Soo Ok;Son, Jun Gwang;Ban, Hee Jung;Lim, Jung Hwan;Cho, Gye Jung;Ju, Jin Young;Kim, Kyu Sik;Kim, Yu Il;Lim, Sung Chul;Kim, Young Chul
    • Tuberculosis and Respiratory Diseases
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    • v.63 no.1
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    • pp.88-93
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    • 2007
  • Wegener's granulomatosis is a disease with an unknown etiology that is characterized by necrotizing granulomatous vasculitis involving the upper and lower respiratory tract and the kidneys. The typical pulmonary findings are bilaterally involved multiple variable sized nodules. We report a case of Wegener's granulomatosis that presented as a single lung mass. A male patient presented with a nasal obstruction, arthralgia, cough, and intermittent dyspnea. The chest radiograph showed a mass, approximately 4.5 cm in diameter, in the right lower lobe. Lung cancer or tuberculosis was initially considered. However, the clinical, laboratory and pathological findings of the mass indicated Wegener's granulomatosis. The patient was administered prednisolone and cyclophosphamide, and improved temporarily. Unfortunately, the immunocompromised patient expired as a result of respiratory failure with pneumonia.

Seven-Year Follow Up of Microscopic Polyangiitis Presenting with Rapidly Progressive Glomerulonephritis (급속 진행성 사구체신염으로 시작된 현미경적 다발성 동맥염(Microscopic Polyangiitis) 환자의 7년간의 장기 추적관찰 1례)

  • Oh, Jin-Won;Kim, Pyung-Kil;Lee, Jae-Seung;Jeong, Hyeon-Joo
    • 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.

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Toxicity Studies of DA-l25, an Anthracycline Antitumor Antibiotic : Intravenous Repeated Doses for 26 Weeks in Beagle Dogs (Anthracycline계 항암성 항생물질 DA-125의 Beagle dog에 대한 26주 반복정맥투여독성시험)

  • 차신우;박종일;정태천;신호철;하창수;김형진;양중익;한상섭;노정구
    • Biomolecules & Therapeutics
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    • v.4 no.2
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    • pp.127-137
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    • 1996
  • This study was performed to investigate the toxicity of DA-125 in beagle dogs, an anthracycline antitumor antibiotic. The dogs were administered DA-125 i.v. at 0.0023, 0.0375, 0.15 and 0.6 mg/kg/day, 6 days/week for 26 weeks. At 0.6 mg/kg, all male and female dogs were either sacrificed moribundly or dead during the 26-week treatment. The dogs revealed inactivity, salivation, dark bloody discharge, swelling of the subcutaneous injection site, abscess, and ulceration in the abdominal wall and legs. At 0.15 mg/kg, anorexia, salivation, and swelling of the injection site were observed. The food consumption was decreased with a statistical significance at 6 and 12 weeks treatment in males of 7.6 mg/kg. At 0.0375, 0.15 and 0.6 mg/kg, body weights were decreased significantly in a dose-related fashion after 17 weeks treatment. Total white blood cell counts for male dogs at 0.6 mg/kg were lower than those of control dogs after 13 weeks treatment, which appeared mainly due to decreased neutrophils. At 0.15 mg/kg, testicular atrophy was found in all males by gross pathology and the testicular weights were significantly decreased when compared to those of control males. Microscopically, the testis showed moderate atrophy of the seminiferous tubules and marked decrease in number of spermatozoa in the epididymal tubules. At 0.6 mg/kg, petechia or echymotic hemorrhage was observed in gastrointestinal tract, heart, lungs, and other organs at the necropsy, Marked atrophy of thymus were observed in both males and females. In addition, severe testicular atrophy was noted in all males. Microscopically, gastrointestinal tract showed hemorrhage, epithelial denudation, hypermucus secretion, and atrophy of intestinal villi. Seminiferous tubules of the atrophic testis were lined with Sertoli cells only and devoid of germ cells. Severe oligospermia or aspermia was present in the epididymal tubules. Bone marrow showed marked depletion of hemopoietic cells. In addition, marked atrophy was found in the lymphoid tissue of gastrointestinal tract, various Iymph nodes, and thymus. Injection sites showed marked inflammatory response with necrosis, necrotizing vasculitis, thrombus formation, and ulceration in the skin. According to the present results, no observed effect level appeared to be 0.0375 mg/kg. At 0.15 mg/kg, testis was a target organ, while at 0.6 mg/kg hemopoietic tissue, gastrointestinal tract, and testis were considered to be target organs. At 0.6 mg/kg the test compound seems to inflict a damage on the blood vessels causing hemorrhage in the various organs and tissues.

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The Prognostic Indicies of Pneumocystis Carinii Pneumonia in Immunocompromised Patients other than Acquired Immune Deficiency Syndrome (비 AIDS 면역 결핍 환자들에서 발생한 주폐포자충 폐렴의 예후인자)

  • Park, Wann;Kim, Yoo-Kyum;Lee, Jin-Seong;Ahn, Jong-Jun;Hong, Sang-Bum;Shim, Tae-Sun;Lim, Chae-Man;Lee, Sang-Do;Kim, Woo-Sung;Kim, Dong-Soon;Kim, Won-Dong;Koh, Youn-Suck
    • Tuberculosis and Respiratory Diseases
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    • v.45 no.4
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    • pp.805-812
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    • 1998
  • Background: Among the variety of opportunistic infections, pneumonia comprises the major morbidity in immunocompromised patients. Pneumocystis carnii pneumonia (PCP) and cytomegalovirus (CMV) pneumonia are common infectious illness of immunocompromised hosts. Although there are many reports regarding to the co-infection of PCP and CMV diagnosed by bronchoalveolar lavage (BAL) fluid examination, the effects of CMV co-infection on the outcome of PCP is still controversial. The purpose of this investigation is to evaluate the effects of CMV detected by BAL fluid examination on the clinical course of PCP in the immunocompromised patients other than human immunodeficiency virus infection. Method: Ten patients with PCP were enrolled and retrospective analysis of their medical records were done. HIV infected persons were excluded. The PCP was diagnosed by BAL fluid examination with Calcofluor-White staining. CMV was detected in BAL fluid by Shell-vial culture system. Chest radiographic findings were reviewed. We used Fisher's exact test and Mann-Whitney U test for statistical analysis of data. Results: The underlying disorders of patients were idiopathic pulmonary fibrosis (n=1), renal transplantation (n=4), necrotizing vasculitis (n=l), systemic lupus erythematosus (n=1), brain tumor (n=1), chronic myelogenous leukemia (n=1), unidentified (n=1). There were no difference in clinical course, APACHE III score, arterial blood gas analysis, white blood cell count, lymphocyte count, serum albumin concentration, chest radiographic findings and mortality between patients with PCP alone (n=4) and those with CMV co-infection (n=6). Univariate analysis regarding to the factors that associated with mortality of PCP were revealed that the application of mechanical ventilation (p=0.028), the level of APACHE III score (p=0.018) and serum albumin concentration (p=0.048) were related to the mortality of patients with PCP. Conclusion: The clinical course of PCP patients co-infected by CMV were not different from PCP only patients. Instead, accompanied respiratory failure, high APACHE III score and poor nutritional status were associated with poor outcome of PCP.

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