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가역성 양측 기저핵 병변을 동반한 반복적인 급성 요독성 뇌병증
최자성 ( Ja Sung Choi ),김정훈 ( Jeong Hun Kim ),전병수 ( Byung Soo Jeon ),박경석 ( Kyung Suk Park ),우명렬 ( Myoung Lyeol Woo ),이상철 ( Sang Choel Lee ),이은자 ( Eun Ja Lee ),윤수영 ( Soo Young Yoon ) 대한신장학회 2008 Kidney Research and Clinical Practice Vol.27 No.1
Uremic encephalopathy is a well-known complication in uremia and rarely occurs with involuntary movements which represent basal ganglia lesion. We experienced a cases of recurrent acute uremic encephalopathy associated with reversible bilateral basal ganglia lesion. A 49-year-old man with end stage renal disease due to diabetic nephropathy on continuous ambulatory peritoneal dialysis (CAPD) treatment for the last 2 months was admitted for mental change. His blood sugar was 60 mg/dl, and his mentality was recovered after glucose solution infusion. Five days after admission, he suddenly developed dysarthria and chorea. Brain magnetic resonance imaging (MRI) demonstrated low and high signals on bilateral basal ganglia in T1-weighted and T2-weighted images, respectively. After intensified CAPD treatment, his neurologic manifestations were resolved. One month after discharge, follow-up brain MRI showed decreased size of low attenuation on the basal ganglia. Two months later, he repeatedly developed dysarthria and involuntary movement of extremities. Newly developed bilateral symmetric T1 low and T2 high signal lesion on the basal ganglia was repeatedly observed on brain MRI. At that time, (his) Kt/V was 1.33. So we decided on him to switch from CAPD to hemodialysis, after then his symptoms disappeared.
자가면역 췌장염의 동반증상 없이 발생한 IgG4 연관 경화성 담관염
천송욱 ( Song Wook Chun ),최자성 ( Ja Sung Choi ),강버들 ( Beo Deul Kang ),김유진 ( Yu Jin Kim ),한기준 ( Ki Jun Han ),조현근 ( Hyeon Geun Cho ),오화은 ( Hwa Eun Oh1 ),조재희 ( Jae Hee Cho ) 대한소화기학회 2013 대한소화기학회지 Vol.62 No.1
IgG4-related systemic diseases are characterized by a diffuse or mass forming inflammatory reaction rich in lymphocytes and IgG4-positive plasma cells (lymphoplasmacytic infiltration), fibrosclerosis of variable organs and obliterative phlebitis. They usually involve various organs including the pancreas, bile duct, gallbladder, salivary gland, retroperitoneum, kidney, lung, and prostate. However, most of them are accompanied by autoimmune pancreatitis, and good response to steroid treatment is one of the hallmarks of this disease. We report a case of an 67-year-old man with IgG4 associated sclerosing cholangitis, who was diagnosed by endoscopic retrograde cholangiopancreatography and successfully treated with steroid therapy. (Korean J Gastroenterol 2013; 62:69-74)
위십이지장동맥 동맥류와 십이지장 사이 누공에서의 만성 출혈
김인태 ( In Tae Kim ),최자성 ( Ja Sung Choi ),김범수 ( Pum Soo Kim ),송순영 ( Soon-young Song ),김유진 ( Yu Jin Kim ) 대한내과학회 2016 대한내과학회지 Vol.91 No.3
Gastroduodenal artery (GDA) aneurysm is a very rare event, but it induces life-threatening clinical manifestations upon rupture or bleeding. The causes of GDA aneurysm are atherosclerosis, infection, trauma, surgery, iatrogenic lesions, mycotic or tuberculous disease, and autoimmune disease. We report the case of a 77-year-old female who presented with melena and vomiting. Upper gastrointestinal endoscopy revealed a 4 cm pulsatile extrinsic mass with a duodenal fistula at the duodenal bulb. Abdominal computed tomography showed a large aneurysm of the GDA. We successfully conducted transcatheter embolization of the aneurysm. After the procedure, the patient did not present with melena, and her hemoglobin level was stable. Follow-up endoscopy showed that the GDA aneurysm-duodenal fistula had decreased in size and was leaking a small amount of blood. An arteriography was performed and showed no evidence of contrast filling in the aneurysmal sac. (Korean J Med 2016;91:277-282)
증례 : 호흡기 ; 제1형 신경섬유종증 환자에서 발생한 흉강내 악성말초신경초종 1예
최진이 ( Jin Yi Choi ),김정훈 ( Jeong Hun Kim ),최자성 ( Ja Sung Choi ),정재호 ( Jae Ho Chung ),최정은 ( Jeong Eun Choi ),오화은 ( Hwa Eun Oh ),박찬섭 ( Chan Sub Park ) 대한내과학회 2009 대한내과학회지 Vol.76 No.2
저자 등은 1형 신경섬유종증 환자에서 발생한 흉강 내 악성말초신경초종 1예를 경험하였고 신경섬유종증을 가진 환자에서 흉강 내종괴를 보일 때 악성말초신경초종도 감별진단에 포함해야 할 것으로 생각된다. A malignant peripheral nerve sheath tumor (MPNST) is a rare soft tissue tumor defined as any malignant tumor arising from or differentiating toward the cells of the peripheral nerve sheath. It is one of the malignant tumors associated with neurofibromatosis type 1 (von Recklinghausen`s disease). They occur most commonly on the lower and upper extremities, trunk, head, and neck, while intrathoracic tumors are very rare. We report an intrathoracic MPNST in a 66-year-old female with neurofibromatosis type 1. (Korean J Med 76:234-237, 2009)
알코올성 간경변 환자에서 다량의 복수로 발현된 막증식성 사구체신염의 양상을 동반한 IgA 신병증
송선옥 ( Sun Ok Song ),이승원 ( Seung Won Lee ),이희우 ( Hee Woo Lee ),강버들 ( Beo Deul Kang ),동시헌 ( Shi Heon Dong ),최자성 ( Ja Sung Choi ),송지선 ( Ji Sun Dong ),윤수영 ( Soo Young Yoon ),이상철 ( Sang Choel Lee ) 대한신장학회 2009 Kidney Research and Clinical Practice Vol.28 No.2
IgA nephropathy can occur commonly in alcoholic liver cirrhosis and is the most common form of secondary IgA nephropathy. Defective clearance of IgA-containing complexes by liver is thought to contribute to the development of IgA nephropathy in alcoholic cirrhosis. Although IgA deposition is found up to 64% in autopsy cases of alcoholic cirrhosis, most patients have mild clinical symptoms, and nephrotic range proteinuria occurs rarely in these patients. We report a case of IgA nephropathy with a membranoproliferative pattern that is detected with unusual massive ascites in a patient with alcoholic liver disease. A 60-year-old male patient was referred to our hospital for evaluation and management of abdominal distension. Abdominal ultrasonographic findings were compatible with diffuse liver cirrhosis with splenomegaly and large amount of ascites. He had nephrotic range proteinuria, azotemia, hyperlipidemia, and hematuria in dipstick. Renal biopsy performed under the impression of acute nephritis revealed mesangial and endocapillary proliferative glomerulonephritis with double contour of capillary loop. Immunofluorescence findings showed mesangial IgA and C3 deposit, compatible with IgA nephropathy. He was treated with high dose steroid, and steroid was tapered during 2 months. Steroid treatment induced complete remission state, and ascites was resolved.
새로운 PROS1 유전자 변이에 따른 S 단백 결핍증에 의한 간문맥-비장정맥-장간막정맥 혈전증
황의태 ( Eui Tae Hwang ),강원식 ( Won Sik Kang ),박진우 ( Jin Woo Park ),이지현 ( Ji Hyun Lee ),한현정 ( Hyun Jeong Han ),신상용 ( Sang Yong Shin ),김희진 ( Hee Jin Kim ),최자성 ( Ja Sung Choi ) 대한소화기학회 2014 대한소화기학회지 Vol.64 No.2
Protein S (PS), a vitamin K-dependent glycoprotein, performs an important role in the anticoagulation cascade as a cofactor of protein C. Because of the presence of a pseudogene and two different forms of PS in the plasma, protein S deficiency (PSD) is one of the most difficult thrombophilias to study and a rare blood disorder associated with an increased risk of thrombosis. We describe a unusual case of previously healthy 37-year-old man diagnosed with portal splenic mesenteric vein thrombosis secondary to PSD. The patient was admitted to the hospital due to continuous nonspecific abdominal pain and nausea. Abdominal computed tomography revealed acute venous thrombosis from inferior mesenteric vein to left portal vein via splenic vein, and laboratory test revealed decreased PS antigen level and PS functional activity. Conventional polymerase chain reaction and direct DNA sequencing analysis of the PROS1 gene demonstrated duplication of the 166th base in exon 2 resulting in frame-shift mutation (p.Arg56Lysfs*10) which is the first description of the new PROS1 gene mutation to our knowledge. Results from other studies suggest that the inherited PSD due to a PROS1 gene mutation may cause venous thrombosis in a healthy young man without any known predisposing factor. (Korean J Gastroenterol 2014,64:110 114)