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학교 신체검진 상 우연히 발견된 제2형 막증식성 사구체신염 1예
김형중,곽병옥,배재욱,김교순,임소덕,Kim, Hyung-Joong,Kwak, Byung-Ok,Bae, Jae-Uk,Kim, Kyo-Sun,Lim, So-Dug 대한소아신장학회 2010 Childhood kidney diseases Vol.14 No.1
막증식성 사구체 신염은 메산지움의 증식과 사구체 모세혈관의 비후를 동반하는 진행성 신질환으로 병리학적 소견에 따라 1, 2, 3형으로 분류할 수 있다. 이 질환은 일차성 사구체신염의 약 2%가량을 차지하는데, 대개의 경우 만성적으로 진행하는 경과를 보이며 다른 사구체신염에 비하여 신기능 소실로 진행하는 경우가 많지만 효과적인 치료법이 정립되어있지 않고 다양한 치료가 시도되고 있다. 학교 집단신체검사에서 소변검사가 포함되기 시작하면서 제1형 막증식성 사구체신염은 수차례 보고되었고 이들에 대한 조기 치료가 신질환의 진행을 늦추는데 효과적인 것으로 알려져 있는데 반해, 제2형 막증식성 사구체신염이 학교 집단신체검사에서 발견된 예는 현재까지 국내에서 보고된 바 없기에 문헌고찰과 함께 보고하는 바이다. Type II membranoproliferative glomerulonephritis (MPGN) is characterized by thickening of the glomerular basement membrane owing to electron-dense deposits on electron microscopy. We experienced a case of type II MPGN in a child presenting with proteinuria, hematuria on school urinary screening tests. He had been treated with losartan and enalapril. This is the first case report of type II MPGN detected by school urinary screening tests in Korea. Thus we report a case of 10-years-old male with type II MPGN with a review of brief literature.
A Case of "Full-house" Nephropathy in a Non-lupus Patient
유하영,손미경,조명현,곽병옥,박혜원,임소덕,정소정,김교순,Yoo, Ha Yeong,Son, Mikyung,Cho, Myung Hyun,Kwak, Byung Ok,Park, Hye Won,Lim, So Dug,Chung, Sochung,Kim, Kyo Sun Korean Society of Pediatric Nephrology 2014 Childhood kidney diseases Vol.18 No.2
"full-house" 면역 복합체 침착은 루푸스 신병증의 진단적인 조직 소견이다. 이 증례 보고에서 12세 남자 환아는 지속적인 현미경적 혈뇨와 단백뇨를 주소로 내원하였다. 그는 신장 조직 검사에서 "full-house" 신병증을 진단받았으나 전신성 홍반성 낭창과 관련한 어떠한 임상적 징후나 혈청학적인 결과를 보이지 않았다. 비록 "full-house" 신병증이 전신성 홍반성 낭창에 선행하는 질병인지에 대해서는 명확히 밝혀진 바는 없으나, 루푸스와 관련된 증상이나 혈청학적인 자가 항체가 출현할 수 있으므로, 추적 관찰을 요한다. 대부분의 현미경적 혈뇨는 좋은 예후를 가지고 있고, 보통은 소변검사를 정기적으로 검사하며 관찰한다. 그러나 이 증례에서 보듯이 소변검사 이상이 발견 되었을 때 오랫동안 무증상으로 잠복하는 "full-house" 신병증 또한 고려되어야 하며, 이는 몇몇의 임상적인 증상 및 혈청학적 소견이 음성이었던 환자가 전신성 홍반성 낭창으로 진행할 수 있기 때문이다. Histopathologic evidence of "full-house" immune complex deposits is a pathognomonic feature of lupus nephritis. This report presents the case of a 12-year-old boy with persistent microscopic hematuria and proteinuria. He was diagnosed with "full-house" nephropathy based on a renal biopsy. However, there was no other clinical or biological evidence of systemic lupus erythematosus (SLE). Although the potential for isolated "full-house" nephropathy preceding SLE is unclear, such patients should be followed for clinical signs and autoantibodies of SLE. In most cases, microscopic hematuria has a good prognosis, and follow-up usually requires only regular urinalysis. However, we should be aware of isolated "full-house" nephropathy that remains asymptomatic for a long time, as few patients with no clinical signs and negative serology ultimately develop SLE.
혈복증으로 발현되어 장유착박리술로 치료된 피막성 복막 경화증 1예
김영백 ( Young Baek Kim ),유문원 ( Moon Won Yoo ),전혜정 ( Hae Jeong Jeon ),임소덕 ( So Dug Lim ),최용훈 ( Yong Hoon Choi ),김범성 ( Bum Sung Kim ),박중희 ( Jung Hee Park ),이종호 ( Jong Ho Lee ),박정환 ( Jung Hwan Park ),조영일 ( 대한신장학회 2009 Kidney Research and Clinical Practice Vol.28 No.5
Encapsulating peritoneal sclerosis (EPS) is an uncommon but fatal complication of peritoneal dialysis (PD). Recently, there were some reports of advanced EPS cases that were successfully treated by enterolysis although an intestinal perforation or leakage from intestinal anastomosis were associated with a high mortality. We experienced a case of EPS in a 53-year-old man on PD for 3.5 years without a previous history of episode of peritonitis who presented with hemoperitoneum during treatment of peritonitis. EPS was diagnosed radiologically according to typical CT findings; The series of CT scans revealed how to develop in sequence from a very subtle findings to full-blown findings of EPS. Enterolysis was performed because the patient did not respond to conservative management such as cessation of PD with transfer to hemodialysis, nutritional support and steroid therapy. In spite of intestinal perforation during surgery, he was successfully treated with enterolysis. Therefore, we report this case with review of the literature.
편측 시력 손실을 초래한 거대 안상 유피낭종의 수술적 치료
이성호(Sung-Ho Lee),고영초(Young-Cho Koh),조준(Joon Cho),오지영(Jee-Young Oh),노홍기(Hong Gee Roh),임소덕(So-Dug Lim) 대한두개저학회 2006 대한두개저학회지 Vol.1 No.1
Suprasellar epidermoid tumor presenting with visual loss is rare. A 50-year-old woman was referred for treatment of a large well demarcated cystic mass at the suprasellar region. She had a history of left visual loss for several years. To preserve the vision of right side, we underwent VEP (visual evoked potential) preoperatively and monitored VEP during the surgery. During surgery, the right optic nerve was severely compressed by the tumor, and was successfully decompressed without damage to the optic nerve. The left optic nerve was more severely compressed by the fibrotic and calcified tumor capsule around the optic canal. The 6 cm sized multilobulated tumor was subtotally resected while preserving the visual functions of the right side. We report a case of giant epidermoid tumor with unilateral visual loss, for which surgical removal was aided by VEP monitoring to save the only remaining vision.
색소피부건조증 환자에서 발생한 안면부 기저세포암의 치료 증례 보고
배재현(Jaehyun Bae),전동근(Dongkeun Jun),김지남(Jeenam Kim),이명철(Myungchul Lee),신동혁(Donghyeok Shin),임소덕(So Dug Lim),최현곤(Hyungon Choi) 대한두경부종양학회 2020 대한두경부 종양학회지 Vol.36 No.1
Xeroderma pigmentosum is a rare autosomal recessive disease, related to defects in DNA repair mechanism. It presents skin lesions on sun-exposed areas, leading to various skin cancer. Skin lesions can be treated with cryotherapy, skin resurfacing, 5-FU, Imiquimod, topical T4 endonuclease V, radiotherapy and genetic therapy, but invasive skin cancer should be treated by a surgery. We report a 12-year-old female xeroderma pigmentosum patient with recurrent basal cell carcinoma successfully treated by skin grafting. In that there is no cure for this disease, prevention and patient education is most important.
김동천 ( Dong Cheon Kim ),조준 ( Joon Cho ),조요한 ( Yo Han Cho ),노홍기 ( Hong Gee Roh ),임소덕 ( So Dug Lim ),고영초 ( Young Cho Koh ) 대한뇌종양학회·대한신경종양학회·대한소아뇌종양학회 2009 대한뇌종양학회지 Vol.8 No.2
Malignant fibrous histiocytoma(MFH) is a soft tissue sarcoma arising from the extremities and retroperitoneum in older adults. Primary intracranial MFH is extremely rare. A 29-year-old woman presented with headache showing three large masses on brain magnetic resonance image(MRI). She had undergone extended total gastrectomy with splenectomy under the diagnosis of advanced gastric cancer three years previously. Systemic positron extension tomography(PET) computed tomography(CT) scan on routine follow-up study before the first brain surgery revealed no hot spots. Three masses were totally removed at a single session. The pathology was confirmed to be typical MFH. Two months after the first brain surgery, she underwent tonsillectomy due to a rapidly growing tonsillar mass and the pathology was found out to be MFH. Postoperative neck CT with angiography for the evaluation of hypotension showed no significant finding. Except for the tonsillar mass, she had been symptom free during 10 months after the first brain surgery. Between 11th and 18th months postoperatively, multiple intracranial recurrences including metastatic lesions of the mediastinum and the spine were noticed. She died of pneumonia 20 months after the first brain surgery during salvage chemotherapy. We report a rare case of primary MFH of the brain with a past medical history of advanced gastric cancer.