• Tuberculosis and Respiratory Diseases
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• v.59 no.1
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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.

• Journal of Life Science
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• v.33 no.2
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• pp.208-215
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• 2023

In recent decades, the field of aging research has progressed from the genetic and cellular levels to in vivo models of blood exchange. Since genes capable of extending the lifespan in C. elegance have been reported, various potential target molecules have been discovered through genomics, proteomics, metabolomics, and transcriptomics. Accordingly, research on the interactions between target molecules has also been increasing. The parabiosis method, in which two experimental animals are surgically combined, was introduced, and a factor that could reverse the aging phenomenon was discovered using this method. The parabiosis method is used to find more accurate and effective aging-reversal factors that could exist in young blood. As more new evidence has been revealed, the parabiosis method has established a new paradigm for aging research. Moreover, a device capable of exchanging blood elaborately in laboratory animals was published in 2022 and presented new results necessary for aging reversal. Since GDF11, was reported, many other anti-aging candidates that are soluble factors in blood, such as β2m, TIMP2, VCAM1, Gpld1, and clusterin, have been discovered. In addition, mcicroglia cells and neuroinflammation have been directly proven to be aging factors. These latest research results were obtained by parabiosis, the newly designed device for plasmapheresis, and injecting young blood or conditioned blood methods. In this review, we discuss the latest research results using the device and young blood administration in old mice.

• The Journal of Korean Oriental Chronic Disease
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• v.10 no.1
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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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• v.55 no.7
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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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• v.8 no.1
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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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• v.54 no.5
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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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• v.6 no.2
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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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• v.46 no.10
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• pp.1040-1043
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• 2003

Henoch-Shonlein purpura(HSP) is a systemic small-vessel vasculitis that primarily affects the skin, gastrointestinal tract, joints, and kidneys. The nervous system may be involved, less commonly than other organs. When the central nervous system(CNS) was involved, headache, changes in mental status, seizures, and focal neurologic deficits have been reported. Hypertension, uremic encephalopathy, metabolic abnomalities, electrolyte abnormalities, or cerebral vasculitis were suggested as possible causes of the neurologic manifestation. Diagnosis of vasculitic involvement of CNS is difficult. Magnetic resonance imaging of the brain is the modality of choice for the evaluation of the CNS disease. Steroid or plasmapheresis are used in treatment of cerebral vasculitis. We experienced a case of 9-year-old boy who had presented with Henoch-Schonlein purpura nephritis complicating encephalopathy accompanied by hypertension and cerebral vasculitis. Brain MRI showed multiple small nodular-linear pattern enhancing lesions in whole cerebral hemispheres and focal increased T2 signal in the right basal ganglia. We used intravenous immunoglobulin in treatment of cerebral vasculitis. We report this case with a brief review of related literature.

• Tuberculosis and Respiratory Diseases
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• v.45 no.4
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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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• 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)