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Henoch-Sch$\ddot{o}$nlein 자반병 신염으로 의심했던 현미경적 다발혈관염 1례
임종근,문경철,구자욱,Im, Jong Geun,Moon, Kyung Chul,Koo, Ja Wook 대한소아신장학회 2012 Childhood kidney diseases Vol.16 No.2
Henoch-Sch$\ddot{o}$nlein 자반병 신염과 유사한 증상을 가지는 10세 여자에게 발생한 급격한 신기능 저하를 동반하지 않은 현미경적 다발혈관염 1례를 경험하였기에 보고하는 바이다. 이 혈관염의 경우. 진단 시점 또는 치료 시작할 때의 신장기능 저하가 심할수록 병의 예후가 나쁘므로[20], 사구체 여과율이 떨어지기 전에 빠른 진단과 치료가 필요하다. Henoch-Sch$\ddot{o}$nlein 자반병이 의심될 경우 단백뇨와 혈뇨가 지속되면, 다른 혈관염과의 감별을 위해 ANCA, 신장 조직검사가 도움을 줄 것으로 생각된다. Microscopic polyangiitis (MPA) is systemic small vessel vasculitis that is very rare in childhood. MPA is characterized by pauci-immune necrotizing small vessel vasculitis without clinical or pathological evidence of necrotizing granulomatous inflammation. Approximately 90% of patients have glomerulonephritis that is accompanied by a variety of other organ involvement. A 10-year-old girl visited our clinic with clinical manifestations suggestive Henoch-Sch$\ddot{o}$nlein purpura nephritis such as purpuric skin rash, abdominal pain, arthralgia on both knees, massive proteinuria and microscopic hematuria. So initially we suspected Henoch-Sch$\ddot{o}$nlein purpura nephritis. However, later her perinuclear-antineutrophil cytoplasmic antibodies(p-ANCA) test was positive, and her renal biopsy was consistent with microscopic polyangiitis. We began steroid therapy, combined with cyclophosphamide, ACE inhibitor. Currently she is a 12-year old, and until now she has been regularly examined in the outpatient. We report a case of microscopic polyangiitis initially suspected with Henoch-Sch$\ddot{o}$nlein purpura nephritis.
김수영,김성헌,문경철,신재일,정현주,Kim, Su-Young,Kim, Seong-Heon,Moon, Kyung-Chul,Shin, Jae-Il,Jeong, Hyen-Joo 대한소아신장학회 2012 Childhood kidney diseases Vol.16 No.1
C1q 신병증은 1985년 Jennett와 Hipp에 의해 발표된 이래 어린 영아에서부터 청 장년층에 까지 발표되어 왔으나 아직 임상병리학적으로 논란이 많은 질환이다. 저자들은 어린 남매에서 스테로이드 저항성의 콩팥증후군의 임상 양상을 보이며 병리 조직학적으로 국소분절사구체경화증과 메산지움에 C1q의 현저한 침착을 보인, C1q 신증을 발표하는 바이다. C1q nephropathy is a distinct clinicopathologic entity, characterized by mesangial immunoglobulin and complement deposits, predominantly C1q, with no evidence for systemic lupus erythematosus. Clinically it may present as nephrotic syndrome and non-nephrotic proteinuria per se or associated with microscopic hematuria, gross hematuria, hypertension, or renal insufficiency. So far there is only one report about a familial case of C1q nephropathy (in two sisters). We present two cases of familial C1q nephropathy with nephrotic syndrome which was steroid resistant, but partially remitted with cyclosporine.
안정희,윤정림,문경철,구자욱,Ahn, Jung-Hee,Yoon, Jung-Rim,Moon, Kyung-Chul,Koo, Ja-Wook 대한소아신장학회 2010 Childhood kidney diseases Vol.14 No.1
Obesity-related glomerulopathy (ORG)는 단백뇨를 보이는 비만아에서 신장 조직학적 소견상 사구체 비대, 국소 분절성 사구체 경화 소견이 관찰되고 기저막 비후, 국소 간질조직 증식, 중등도의 발 돌기 소실 등을 보이는 질환으로 정의된다. 특발성 국소분절 사구체경화증과 비교하여 신증후군이 적으며 콜레스테롤 상승이 적고, 병의 진행이 느리다는 점에서 차이가 있다고 알려져 있으며, 체중감소를 통해 신장 기능이 회복될 수 있다고 알려져 있다. 본 증례의 9세된 비만아는 임상증상과 신장 조직검사에서 ORG로 진단되어 치료하였으나, 지속적인 체중 증가와 단백뇨로 말기 신부전으로 진행하여 소아 ORG 환아에서는 드문 경과를 취하여 이를 보고하는 바이다. Obesity-related glomerulopathy (ORG) is a secondary form of focal and segmental glomerulosclerosis (FSGS) manifesting as proteinuria and progressive renal dysfunction that results from maladaptive glomerular response to increasing adiposity. Reports of ORG progressing to end stage renal diseases in rare in the pediatric population. We report a 9-year-old boy with obesity (body mass index $35\;kg/m^2$) who was diagnosed with ORG presenting with proteinuria. He was diagnosed with obesity-related glomerulopathy based on the laboratory, urinary, and kidney biopsy finding. In spite of treatment with angiotensin- converting enzyme (ACE) inhibitor and/or, angiotensin-receptor blocking agent, the degree or amount of proteinuria increased and renal function declined continuously. His BMI did not decrease and eventually progressed to chronic renal failure. Consequently, obese patients should be monitored for proteinuria, which may be the first manifestation of FSGS, a lesion that may be associated with serious renal sequelae.
증례 : 말기 신부전 환자에서 발생한 원발성 신장 혈관육종 1예
김선문 ( Sun Moon Kim ),노한 ( Han Ro ),김치원 ( Chi Weon Kim ),차란희 ( Ran Hui Cha ),김연수 ( Yon Su Kim ),김성권 ( Suhng Gwon Kim ),문경철 ( Kyung Chul Moon ) 대한내과학회 2007 대한내과학회지 Vol.72 No.3
원발성 신장 혈관육종은 매우 드문 악성 종양으로, 그 예후가 불량하다. 외국에서는 20예 정도가 보고되어 왔으나, 아직까지 우리나라에서는 보고된 바가 없다. 저자들은 신장출혈로 발현한 원발성 신장 혈관육종 1예를 경험하였기에 이를 보고한다. 특히 본 증례의 경우, 자발성 신장출혈로 발현하였고, 말기 신부전증으로 투석 치료를 받는 중에 발생하였던 점이 이전의 보고와 다르며, 증상 발현부터 사망까지 3개월여 동안의 방사선학적 검사와 두 차례의 수술 등으로 임상경과를 상세하게 알 수 있었다. 신장출혈을 동반한 환자에서 드물지만, 신장 혈관육종 등이 가능함을 알고 향후 감별진단에 고려해야 하겠다. Primary renal angiosarcoma is a rare and fatal malignant tumor. About 20 cases have been reported in the literature, but there has been no report of renal angiosarcoma in Korea. A 67-year old man with end-stage renal disease was admitted to the hospital for left flank pain. A computed tomography examination revealed a subcapsular hemorrhage in the left kidney. The patient was discharged after 2 weeks of observation. However, at 6 weeks after the discharge, the patient revisited the outpatient clinic due to aggravating pain, and a left nephrectomy was performed. The histopathological diagnosis was angiosarcoma. Three weeks after the nephrectomy, a re-operation was performed because of uncontrolled intra-abdominal bleeding. The surgical findings revealed massive metastasis. The patient died of an uncontrolled hemorrhage about 1 week after the second surgery. To our knowledge, this is the first report of a renal angiosarcoma in a patient with end-stage renal disease. It should be noted that a renal angiosarcoma may be a cause of a retroperitoneal hematoma. (Korean J Med 72:322-325, 2007)
급성 A형 간염 환자에서 발생한 간질성 신염과 IgA 신병증의 병발에 의한 급성 신부전 : 1예 보고
한승석 ( Seung Seok Han ),김민경 ( Min Kyung Kim ),최병용 ( Byoung Yong Choi ),민희석 ( Hee Suk Min ),김학령 ( Hack Lyoung Kim ),정용진 ( Chun Soo Lim ),임춘수 ( Chun Soo Lim ),이현주 ( Hyun Ju Lee ),문경철 ( Kyung Chul Moon ) 대한신장학회 2007 Kidney Research and Clinical Practice Vol.26 No.6
제1형 당뇨병 환자에서 병발한 막증식성 사구체신염 1례
최현진,조희연,강주형,양세원,정해일,최용,문경철,하일수,Choi Hyun-Jin,Cho, Hee-Yeon,Kang Ju-Hyeong,Yang Sei-Won,Cheong Hae-Il,Choi Yong,Moon Kyung-Chul,Ha Il-Soo 대한소아신장학회 2005 Childhood kidney diseases Vol.9 No.1
저자들은 당뇨병성 신병증의 자연경과에 맞지 않는 요검사상 이상 소견을 보인 제 1형 당뇨병을 가진 소아에서 신생검을 시행하여 막증식성 사구체 신염을 진단하였기에 보고하는 바이다. Renal diseases other than diabetic nephropathy can occur In diabetic patients. Urinary abnormalities or clinical courses inconsistent with the natural progression of diabetic nephropathy are suggestive of non-diabetic renal diseases and should lead to more extensive investigations. Presence of non-diabetic renal diseases in diabetic patients can alter the treatment plan and the prognosis. We report a 9-year-old girl who had type 1 membranoproliferatiye glomerulonephritis as well as type 1 diabetes mellitus.
김민석,박인애,박선후,박성신,김활웅,문경철,김영아,이혜승,박기화,서정욱,이현순,함의근,Kim, Min-Suk,Park, In-Ae,Park, Sun-Hoo,Park, Sung-Shin,Kim, Hwal-Wong,Moon, Kyung-Chul,Kim, Young-Ah,Lee, Hye-Seung,Park, Ki-Wha,Seo, Jeoug-Wook,Lee, Hy 대한세포병리학회 1999 대한세포병리학회지 Vol.10 No.1
The authors analysed 2,653 cases of transthoracic fine needle aspiration cytology of the lung to evaluate the diagnostic accuracy and its limitation. A comparison was made between the original cytologic and the final histologic diagnoses on 1,149 cases from 1,074 patients. A diagnosis of malignancy was established in 38.3% benign in 48.1%, atypical lesion in 2.3%, and inadequate one in 11.9% of the cases. Statistical data on cytologic diagnoses were as follows; specificity 98.9%: sensitivity of procedure, 76.8%: sensitivity of diagnosis, 95.5%: false positive 5 cases: false negative 18 cases: predictive value for malignancy, 98.8%: predictive value for benign lesion, 79.5%: overall diagnostic efficiency, 87.5%: typing accuracy in malignant tumor, 80%.