• Tuberculosis and Respiratory Diseases
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• 제59권1호
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• pp.86-92
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• 2005

Goodpasture's syndrome is a disease that is characterized by hemoptysis, anemia, and glomerulonephritis with renal failure. Goodpasture reported a case of a young man who expired as a result of a pulmonary hemorrhage and glomerulonephritis at the recovery phase after an influenza infection in 1919. In 1958, Stanton et al. described a combined case of these two diseases as Goodpasture's syndrome. Since then, antiglomerular basement membrane antibody(anti-GBM Ab) has been confirmed to play an important role in the mechanism of this syndrome, and it was reported that this syndrome was an autoimmune disease. The triad of alveolar hemorrhage, glomerulonephritis and circulating anti-GBM Ab forms the basis of a diagnosis of Goodpasture's syndrome. When patients are affected by disease, the relief of symptoms can be accomplished by eliminating the anti-GBM Ab from the circulatory system through hemodialysis, plasmapheresis and immunoabsorption. However, the patients usually die from a massive pulmonary hemorrhage when the diagnosis or treatment is delayed. The incidence of Goodpasture's syndrome is common in the western world, but it is extremely rare in Korea with only five cases being reported. In three of these cases, pulmonary hemorrhage and renal failure was the initial manifestation. Therefore, hemodialysis or plasmapheresis were absolutely essential treatments. We report a case of Goodpasture's syndrome in Korea with a normal renal function.

• 생명과학회지
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• 제33권2호
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• pp.208-215
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• 2023

최근 수십년간 노화연구의 영역은 유전자 수준에서부터 세포 수준을 거쳐 혈액을 교환하는 in vivo 모델까지 진보를 거듭하면서 발전하고 있다. 예쁜꼬마선충에서 수명을 연장시킬 수 있는 유전자의 존재가 알려지면서, 유전체학, 단백질체학, 대사체학, 전사체학과 같은 다양한 분석방법이 사용되면서 보다 다양한 노화 연관 표적분자들이 발견되었다. 따라서, 표적분자들 간의 상호관계에 대한 연구결과도 증가하고 있다. 또한, 두 실험동물을 외과적으로 결합시킨 개체병렬결합 방법을 사용한 노화연구로 노화 현상을 역행시킬 수도 있는 인자가 보고되었고, 젊은 개체의 혈액 내에 존재할 수 있는 노화 역행 인자를 찾기 위한 더 정확하고, 효과적인 연구로 확장되면서, 노화연구의 방법에 새로운 패러다임이 확립되었다. 2022년 실험동물에 정교하게 혈액을 교환할 수 있는 장치에 대한 논문이 발표되었고, 이 장치를 사용한 연구가 노화 역행에 영향을 줄 수 있는 새로운 결과를 제시하였다. 새롭게 고안된 장치와 그로 인한 결과뿐만 아니라 젊은 혈액 또는 조건화된 혈액을 주입하여 얻은 최신 연구결과로 처음 발표되었던 GDF11 외에도 혈액 내에 존재하는 노화 역행 후보물질로서 β2m, TIMP2, VCAM1, Gpld1, clusterin과 같은 혈액 내 용해성 인자뿐만 아니라 mcicroglia 세포와 neuroinflammation과 같은 생화학적 현상이 직접적으로 노화요인으로 증명되고 있다. 이 총설에서는 이 같은 노화연구에 대한 최신 결과에 대해 논의하고자 한다.

• 대한한방성인병학회지
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• 제10권1호
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• pp.53-61
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• 2005

Guillain-Barre syndrome (GBS) is a group of autoimmune syndromes consisting of demyelinating and acute axonal degenerating forms of the disease. Typically, Gullain-Barre syndrome can be diagnosed from the patient's symptoms and physical examination such as the rapid onset of weakness, paralysis and loss of reflexes. In most patients, resolution is complete or near complete. Treatment consists of supportive care, ventilatory management (in about one third of patients), and specific therapy with intravenous immunoglobulin or plasmapheresis. This clinical report is about suspected acute severe Guillain-Barre syndrome patient, 61-year-old man had quadriplegia, facial palsy, dysphasia, respiratory failure. After 5 weeks of East-West integrative medicine therapy - Conventional Conservative therapy(plasmaphresis and intravenous immunoglobulin) and Korean traditional medicine(Sasang medicine and acupuncture treatment) - most symptoms improved.

• Clinical and Experimental Pediatrics
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• 제55권7호
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• pp.249-253
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• 2012

We report a case of a 13-year-old girl with acute lymphoblastic lymphoma- leukemia, who presented with a cardiac metastasis in the right ventricle, resulting in a pulmonary embolism. At the time of her leukemia diagnosis, a cardiac mass was incidentally found. The differential diagnosis for this unusual cardiac mass included cardiac tumor, metastasis, vegetation, and thrombus. Empirical treatment was initiated, including anticoagulation and antibiotics. She underwent plasmapheresis and was administered oral prednisolone for her leukemia. Five days later, she experienced sudden hemodynamic collapse and required extracorporeal membrane oxygenation insertion and emergency surgery. These interventions proved futile, and the patient died. Pathology revealed that the cardiac mass comprised an aggregation of small, round, necrotic cells consistent with leukemia. This is the first known case of acute lymphoblastic leukemia presenting as a right ventricular mass, with consequent fatal acute pulmonary embolism. A cardiac mass in a child with acute leukemia merits investigation to rule out every possible etiology, including vegetation, thrombus, and even a mass of leukemic cells, which could result in the fatal complication of pulmonary embolism.

• Annals of Clinical Neurophysiology
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• 제8권1호
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• pp.94-97
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• 2006

Bickerstaff's brainstem encephalitis (BBE) is a rare disorder with acute ophthalmoparesis, ataxia, consciousness disturbance, and pyramidal signs of suspected autoimmune origin. A-62-year-old woman developed acute diplopia, dysarthria, gaze-paretic nystagmus and gait ataxia. Her mental status fell subsequently into stupor. Brain MRI and nerve conduction study showed no significantly abnormal findings. Electroencephalography revealed diffuse low voltage slowings. After treating with intravenous immunoglobulin, she demonstrated rapid clinical recovery. This case suggests that immunoglobulin can be an alternative option in BBE treatment, especially when plasmapheresis and corticosteroids are difficult or contraindicated.

• Journal of Chest Surgery
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• 제54권5호
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• pp.396-399
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• 2021

A 58-year-old man, incapable of maintaining oxygen saturation with mechanical ventilation, was admitted to our hospital for veno-venous extracorporeal membrane oxygenation (ECMO) treatment. He was diagnosed with acute respiratory distress syndrome (ARDS) due to influenza A pneumonia. His condition stabilized with antibiotics and steroid administration, but weaning from ECMO failed due to post-infectious pulmonary sequelae. On day 84 after admission, he underwent bilateral lung transplantation. In the postoperative phase, he did not regain consciousness even after discontinuation of sedatives for 3 days. However, spontaneous pupillary reflex and eye movements were preserved, while communication and upper and lower limb movements were affected. The nerve conduction study was diagnostic of Guillain-Barré syndrome. He was managed with intravenous immunoglobulins and plasmapheresis. Mild recovery of the facial muscles was seen, but he died 24 days post-surgery due to progressive ARDS and sepsis.

• Annals of Clinical Neurophysiology
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• 제6권2호
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• pp.92-97
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• 2004

Background: There were several studies comparing prognostic factors in Guillain-Barre syndrome treated with intravenous immunoglobulin and plasmapheresis. However, there were controversies in what were significant factors and there were few studies so far comparing the therapeutic outcomes in patients treated with immunoglobulin. This study was aimed to determine the prognostic factors which affected the therapeutic outcome of Guillain-Barre syndrome treated with intravenous immunoglobulin. Method: We retrospectively reviewed the medical records of patients with Guillain-Barre syndrome admitted to our hospital between January 1999 and March 2004. All patients were treated with intravenous immunoglobulin. Outcome and prognosis were followed up after four weeks using the overall disability sum score. Results: Thirty-six patients were enrolled in this study. According to the clinical and electrophysiological findings, 17 patients were AIDP, 10 were axonal forms, two were mixed and seven had electrophysiologically no evidence of abnormalities. At a follow-up of four weeks, disabilities at the nadir (p<0.001) and admission (P<0.012), initial manifestations of bulbar symptom (P<0.024) and electrodiagnostic features (P<0.013) were significantly correlated with outcome in patients treated with intravenous immunoglobulin. But only disabilities at the nadir (P<0.033) and electrodiagnostic features (P<0.018) were significant in the multivariate logistic regression analysis. Conclusion: Among the patient treated with intravenous immunoglobulin, the outcomes were significantly different according to the neurological status at the nadir. Therefore early diagnosis, administration of intravenous immunoglobulin and preventing complications during acute stages are essential to minimize neurological deficit and shorten the periods of recovery.

• Clinical and Experimental Pediatrics
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• 제46권10호
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• pp.1040-1043
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• 2003

저자들은 9세 남아에서 고혈압과 뇌혈관염이 동반된 상태에서 뇌증을 보인 HSP 신염 1례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

• Tuberculosis and Respiratory Diseases
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• 제45권4호
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• pp.888-895
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• 1998

Malaria is one of the most common infectious diseases in the world. Plasmodium falciparum, accounting for nearly all malaria mortality, kills an estimated 1 to 2 million persons yearly and has several features that make it deadlist of malarias. While cerebral malaria is the most common presentation of severe disease, acute lung injury associated with malaria is uncommon but serious and fatal complication. We report two cases of severe malaria with ARDS and multi-organ failure. All two patients traveled to foreign countries, Kenya, Papua New Guinea where choroquine-resistant malaria is distributed. The first case, which developed cerebral malaria, hypoglycemia, multi-organ failure, and ARDS, treated with quinine and mechanical ventilator, but expired due to oxygenation failure. Autopsy showed acute necrotizing infiltration, diffuse eosinophilic fibrinoid deposits along the alveolar space, and alveolar macrophage with malaria pigment The second case also developed multi-organ failure, followed by ARDS, and was treated with quinine, exchange transfusion, plasmapheresis, and mechanical ventilator. He recovered with residual restrictive lung change after treatment.

• Childhood Kidney Diseases
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• 제5권2호
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• pp.213-218
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• 2001

현미경적 다발성 동맥염은 폐출혈과 급속 진행성 사구체신염을 특징으로 하는 전신성 혈관염의 일종으로 소아에서 매우 드문 질환이다. 저자들은 폐출혈과 급성신부전을 동반한 7년 9개월된 여자 환아에서 신장조직 검사와 p-ANCA(perinuclear antineutrophil cytoplasmic autoantibodies) 검사로 현미경적 다발성 동맥염을 진단할 수 있었으며 이후 methyl-prednisolone pulse therapy와 cyclophosphamide, 4회의 혈장 교환 치료를 병행하였다. 환아 내원 당시 BUN 117 mg/dL, Cr 2.3 mg였으나 입원 제 60병일째 BUN 20.8 mg/dL, Cr 1.6 mg 으로 감소하고 혈뇨, 단백뇨는 지속되었으나 폐출혈 소견 호전되고 전신상태 양호하여 현재 외래 추적 관찰 중에 있다. 이에 국내외의 문헌 고찰과 함께 보고하는 바이다.