• Childhood Kidney Diseases
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• v.21 no.2
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• pp.165-168
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• 2017

Focal segmental glomerulosclerosis (FSGS) in children, which is a kind of nephrotic syndrome showing steroid resistance, usually progresses to a substantial number of end stage renal disease (ESRD). Although the pathogenesis of primary FSGS is unclear, several recent studies have reported that FSGS is associated with circulating immune factors such as soluble urokinase-type plasminogen activator receptor (suPAR) or anti-CD40 autoantibody. We report a successfully treated case of a 19-year-old female patient who experienced a recurrence of primary FSGS. After the diagnosis of FSGS, the patient progressed to ESRD and received a kidney transplantation (KT). Three days later, recurrence was suspected through proteinuria and hypoalbuminemia. She has been performed plasmapheresis and high dose methylprednisolone pulse therapy and shown remission status without increasing proteinuria for four years after KT. In conclusion, strong immunosuppressive therapy may be helpful for a good prognosis of recurrent FSGS, suppressing several immunologic circulating factors related disease pathogenesis.

• Annals of Clinical Neurophysiology
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• v.6 no.1
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• pp.43-47
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• 2004

We report a case of 36-year-old woman with myasthenia gravis (MG) combined with mediastinal leiomyosarcoma (LMS) and Stevens-Johnson syndrome (SJS). She was admitted to ICU with the symptoms of acute onset headache, diplopia, ptosis, dysphagia, general weakness, and respiratory difficulty for several days. Physical examination revealed tachypnea, decreased breath sounds and dullness to percussion in right lower chest. Neurologic examination showed ptosis, diplopia, decreased gag reflexes, and generalized proximal weakness. Laboratory studies revealed increased serum acetylcholine receptor antibodies and positive Tensilon test. Chest CT showed a huge mass in the right middle mediastium but no evidence of thymic enlargement. Mediastinal LMS was diagnosed by ultrasound-guided needle biopsy. The myasthenic symptoms were fluctuated in spite og intravenous immunoglobulin, plasmapheresis, and corticosteroid. During therapy, SJS developed. She died 4 months after the onset of the myasthenic symptoms despite the chemotherapy for LMS.

• Clinical and Experimental Pediatrics
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• v.61 no.2
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• pp.37-42
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• 2018

Hemolytic uremic syndrome (HUS) is often encountered in children with acute kidney injury. Besides the well-known shiga toxin-producing Escherichia coli-associated HUS, atypical HUS (aHUS) caused by genetic complement dysregulation has been studied recently. aHUS is a rare, chronic, and devastating disorder that progressively damages systemic organs, resulting in stroke, end-stage renal disease, and death. The traditional treatment for aHUS is mainly plasmapheresis or plasma infusion; however, many children with aHUS will progress to chronic kidney disease despite plasma therapy. Eculizumab is a newly developed biologic that blocks the terminal complement pathway and has been successfully used in the treatment of aHUS. Currently, several guidelines for aHUS, including the Korean guideline, recommend eculizumab as the first-line therapy in children with aHUS. Moreover, life-long eculizumab therapy is generally recommended. Further studies on discontinuation of eculizumab are needed.

• Journal of Chest Surgery
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• v.25 no.10
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• pp.1035-1040
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• 1992

Myasthenia gravis is an autoimmune disease in which the thymus gland is thought to play a central role in the pathogenesis. Thymectomy is now standard therapy, and improvements in surgical techniques, anesthesia, and respiratory care and the use of plasmapheresis have markedly reduced the operative morbidity. We experienced 7 myasthenia gravis with maximal thymectomy from November 1989. As compared with the results of previous classic transsternal thymectomy group the 25 patients who were operated from June 1979 to December 1991[Group B], excellent surgical result was obtained in maximal thymectomy group[Group A]. During follow-up period that ranged from a month to 10.7 years[mean 2 years], the remission rate for the entire group was 15.6 percent and an additional 71.9 percent had improvement[87.5 percent benefited]. In Group A, the remission rate was 42.9 percent with 100 percent of the patients benefiting from operations. But in Group B, 8 percent had remission and a total of 80 percent benefited. In both Groups, there was no operative mortality but there was one case`s mortality in Group B during follow-up period.

• Proceedings of the Membrane Society of Korea Conference
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• 1997.10a
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• pp.79-80
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• 1997

1. 서론 : 열유도 상분리법(TIPS)은 일반적으로 상온에서 적정용매가 없는 고분자 소재를 아용하여 고분자-희석제 2성분계의 혼합물을 적절한 냉각 속도로 상분리 온도 이하로 급냉시켜 연속상과 분산상의 상분리를 일으킨 후 matrix전체에 다공성을 부여하여 분리막을 제조하는 공정이다. 폴리올레핀계 고분자를 중심으로 나일론11. 폴리카보네이트, PVC, ABS 수지 등을 소재로 plasmapheresis, 인공심폐기, breathing wear 등의 용도로 많이 응용되어 왔으며 현재 한외여과나 정밀여과 등 수투과 공정 및 battery separator 등으로의 응용이 활발하게 연구되고 있다. TIPS공정에 의해 제조된 고분자 분리막은 기존의 분리막에 비해 내열성 및 내약품성이 우수하며 여러 가지 다양한 변수로 부터 막의 미세구조를 조절할 수 있어 기공의 크기 및 모양이 조절 가능하다. 본 연구에서는 TIPS 공정을 이용한 고분자 분리막의 제조시 take-up speed와 air gap 등을 변화시켜 이에 따른 영향을 조사하였고 또한 cold stretching에 의한 구조변화를 알아보았다.

• Clinical and Experimental Pediatrics
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• v.54 no.8
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• pp.317-321
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• 2011

Children who suffer from steroid-resistant nephrotic syndrome (SRNS) require aggressive treatment to achieve remission. When intravenous high-dose methylprednisolone fails, calcineurin inhibitors, such as cyclosporine and tacrolimus, are used as the first line of treatment. A significant number of patients with SRNS progress to end-stage renal disease if remission is not achieved. For these children, renal replacement therapy can also be problematic; peritoneal dialysis may be accompanied by significant protein loss through the peritoneal membrane, and kidney allograft transplantation may be complicated by recurrence of SRNS. Plasmapheresis and rituximab were initially used for treatment of recurrent SRNS after transplantation; these are now under consideration as rescue therapies for refractory SRNS. Although the prognosis of SRNS is complicated and unfavorable, intensive treatment in the early stages of the disease may achieve remission in more than half of the patients. Therefore, timely referral of pediatric SRNS patients to pediatric nephrology specialists for histological and genetic diagnosis and treatment is highly recommended.

• Annals of Clinical Neurophysiology
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• v.8 no.1
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• pp.6-15
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• 2006

Intravenous immunoglobulin (IVIg) is the treatment of choice for many autoimmune neuropathic disorders such as Guillain-Barre syndrome (GBS), chronic inflammatory Demyelinating neuropathy (CIDP), and multifocal motor neuropathy (MMN). IVIg is preferred because the adverse reactions are milder and fewer than the other immune-modulating methods such as steroid, other immunosuppressant such as azathioprine, and plasmapheresis. IVIg also has been used in other autoimmune neuromuscular disorders (inflammatory myopathy, myasthenia gravis, and Lambert-Eaton myasthenic syndrome) and has been known as safe and efficient agent in these disorders. Since IVIg would get more indications and be used more commonly, clinicians need to know the detailed mechanism of action, side effects, and practical points of IVIg.

• Annals of Clinical Neurophysiology
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• v.13 no.2
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• pp.97-100
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• 2011

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired immune-mediated polyneuropathy. Corticosteroids, intravenous immunoglobulin (IVIG) and plasmapheresis have been reported to be effective treatment. Rarely, CIDP can occur in the patients with HIV infection. The clinical features and electrophysiological findings of CIDP are known to be similar in patients with and without HIV infection. We report a 30-year-old male with HIV infection associated CIDP who improved after the administration of intravenous immunoglobulin and long term oral prednisone.

• The Korean Journal of Medicine
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• v.93 no.6
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• pp.518-524
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• 2018

Hypertriglyceridemia a major cause of acute pancreatitis, accounting for up to 10% of all cases. The pathophysiological mechanism of hypertriglyceridemia-induced acute pancreatitis (HTGP) is presumed to involve the hydrolysis of triglycerides by pancreatic lipase resulting in an excess of free fatty acids and elevated chylomicrons, which are thought to increase plasma viscosity and induce ischemia and inflammation in pancreatic tissue. Although the clinical course of HTGP is similar to other forms of acute pancreatitis, the clinical severity and associated complications are significantly higher in patients with HTGP. Therefore, an accurate diagnosis is essential for treatment and prevention of disease recurrence. At present, there are no approved guidelines for the management of HTGP. Different treatment modalities such as apheresis/plasmapheresis, insulin, heparin, fibric acids, and omega-3 fatty acids have been successfully implemented to reduce serum triglycerides. Following acute phase management, lifestyle modifications including dietary adjustments and drug therapy are important for the long-term management of HTGP and the prevention of relapse. Additional studies are required to produce generalized and efficient treatment guidelines for HTGP.

• Journal of Yeungnam Medical Science
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• v.34 no.1
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• pp.123-127
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• 2017

Drug-induced immune hemolytic anemia (DIIHA) is a rare side effect of drugs. DIIHA may cause a systemic inflammatory response that results in acute multi-organ failure and death. Ceftizoxime belongs to the class of third generation cephalosporins, which are the most common drugs associated with DIIHA. Herein, we present a case of a 66-year-old man who developed fatal DIIHA after receiving a second dose of ceftizoxime. He was admitted to receive photodynamic therapy. He had a history of a single parenteral dose of ceftizoxime 3 months prior to admission. On the day of the procedure - shortly after the infusion of ceftizoxime - the patient's mental status was altered. The blood test results revealed hemolysis. Oliguric acute kidney injury developed, and continuous renal replacement therapy had to be applied. On the suspicion of DIIHA, the patient underwent plasmapheresis. Diagnosis was confirmed by a detection of drug-dependent antibody with immune complex formation. Although his hemolysis improved, his liver failure did not improve. He was eventually discharged to palliative care, and subsequently died.