거대요관과 거대방광이 동반된 선천성 신성요붕증 1예

이은정,이규백,서정열,김향,박흥재,김영학,권칠훈,이영래 대한신장학회 2001 대한신장학회잡지 Vol.20 No.4

고령인구에서 신기능 부전의 유병률과 위험인자

서정열,정수석,김향,이규백 대한신장학회 2004 대한신장학회잡지 Vol.23 No.1

성인에서 장기간 Furosemide오용과 연관된 신수질 석회화

김윤구,이윤하,이규백,장세호,김대중,오하영,김보현,김미경,조윤숙 대한신장학회 1996 대한신장학회잡지 Vol.15 No.4

Genetic diagnosis of autosomal dominant polycystic kidney disease: linkage analysis versus direct mutation analysis

( Kyu Beck Lee ) 대한신장학회 2016 Kidney Research and Clinical Practice Vol.35 No.2

Mutations of the PKD2 Gene in Korean Patients with Autosomal Dominant Polycystic Kidney Disease

Kyu Beck Lee,Curie Ahn,Un Kyung Kim 한국유전학회 2006 Genes & Genomics Vol.28 No.2

상염색체 우성 다낭신 환자에서 신장 해부학적 지표에 따른 고혈압과 신기능의 변화

이규백(Kyu Beck Lee),김향(Hyang Kim),이영래(Young Rae Lee) 대한신장학회 2001 Kidney Research and Clinical Practice Vol.20 No.2

상염색체 우성 다낭신(ADPKD)은 다낭신 유전자를 받은 환자에서 점진적으로 신장에 낭종들의 수와 크기가 커져서 신혈관과 신실질을 압박하여 고혈압, 신부전을 발생시킨다. 그리고 ADPKD 환자에서 고혈압과 신부전 발생 이전부터 신장의 해부학적 변화가 발생한다. 따라서 오랜 기간의 임상경과를 가진 ADPKD에서 신장 해부학적 지표에 따른 고혈압과 신기능 변화를 평가하는 것은 중요하다. 그리고 신장 해부학적 지표를 ADPKD의 임상경과를 예측할 수 있을지를 알아보는 것은 의미 있다. 본 연구에서 강북삼성병원을 내원한 ADPKD환자 가운데 복부초음파검사로 진단하고, 해부학적 구조를 정확히 볼 수 있는 복부 전산화단층촬영을 시행한 67명의 환자를 대상으로 신장 해부학적 지표에 따른 고혈압, 신기능을 비교하였다. 한명의 방사선과 전문의가 복부 전산화단층촬영 필름을 분석하여 신장의 해부학적 지표로 신장의 길이, 용적, RSI(anatomical renal severity index), 최대 신낭종직경을 측정하였다. ADPKD 환자(평균 연령 45±12세, 남녀비42:25) 가운데 고혈압은 41명(61%)에서 있었으며, 신기능 부전 (크레아티닌 청소율50mL/min/1.73m2 이하)은 15명(22%)에서 있었다. 정상 혈압 군에 비하여 고혈압 군에서 신장길이, 신장용적, RSI, 최대 신낭종직경 모두 유의하게 증가되어 있었다(p<0.01)(신장용적 360±154mL/1.73 m2 대 833±585mL/1.73 m2 ). 정상 신기능 군에 비하여 신부전 군에서 신장길이, 신장용적, RSI, 최대 신낭종직경 모두 유의하게 증가되어 있었다(p<0.01)(신장용적499±335mL/1.73 m2 대 1171±700mL/1.73m2 ). 환자의 크레아티닌 청소율은 신장 해부학적 지표인 신장길이(r= -0.39), 신장용적(r= -0.49), RSI(r= -0.39), 최대 신낭종직경(r= -0.38)과 유의한 상관관계가 있었다(p<0.01). 본 연구 결과 ADPKD 환자에서 신장 해부학적 지표인 신장길이, 신장용적, RSI, 최대 신낭종직경은 고혈압, 신기능과 상관관계를 가지고, 고혈압 군과 신부전 군에서 증가되어 있다. ADPKD에서 신장의 낭종이 많아지고, 신장이 커질수록 고혈압이 발생하고, 신기능이 감소함을 객관으로 증명하였다. 향후에 ADPKD환자의 임상경과를 보는데 이러한 신장 해부학적 지표를 유용하게 사용할 수 있을 것으로 생각된다. Autosomal dominant polycystic kidney disease (ADPKD) is a common hereditary disorder, and its major morbidities are hypertension and renal failure. It is a characteristic feature of ADPKD that renal cysts increase in size and number with age continuously. Hypertension and renal failure in ADPKD result from compression of residual normal renal parenchyma by expanding cysts, since renal tissue is trapped within the poorly distensible renal capsule. Renal structural deformities in ADPKD occur prior to hypertension and renal failure. The present study was undertaken to explore the potential role of renal cyst enlargement in initiating hypertension and renal failure in ADPKD. We therefore measured renal structural indices by computed tomography(CT) to examine the relation between clinical progression and renal structural deformities. Sixty-seven adult subjects(45±12 years, rnale-female ratio 42: 25) with ADPKD were studied at our PKD clinic from 1997 to 2000, and a complete abdominal CT was performed on all subjects. One radiologist measured the renal structural indices which were renal length, volume, RSI(anatomical renal severity index) and maximal cyst size. The renal structural indices were significantly greater in hypertensive group compared to the normotensive group (hypertensive 833±585 vs. normotensive 360±154mL/ 1.73m------------², p<0.01). The renal structural indices were significantly greater in renal failure group compared to the normal renal function group(renal failure 1,171±700 vs. normal 499±335mL/1.73m², p<0.01). The renal function in ADPKD correlated with the renal structural indices. We concluded that the clinical progression in ADPKD correlates with the renal structural indices well. These structural indices provide considerable information about the progression of ADPKD.

만성 염증성 탈수초 다발성 신경근병증이 동반된 낭창성 신염

이규백(Kyu Beck Lee),김향(Hyang Kim),이상종(Sang Jong Lee),박찬필(Chan Pil Park),박문향(Moon Hyang Park),박범준(Bum Joon Park),김현승(Hyun Seung Kim),이한보(Han Bo Lee),이준(Jun Lee),오재인(Jae In Oh) 대한신장학회 2000 Kidney Research and Clinical Practice Vol.19 No.3

Systemic lupus erythematosus(SLE) is a multisys-temic disease. Peripheral neuropathy occurs in about 10% of patients with SLE. Chronic inflammatory demyelinating polyneurpathy has been reported rarely in SLE. We experienced a case of chronic inflammatory polyneuropathy in lupus nephritis. 32-year-old housewife presented to chronic progressive muscle weakness and heavy proteinuria. Kidney biopsy showed compatible with lupus nephritis (WHO Class V, membranous nephropathy). Nerve conduction studies showed reduction in conduction velocity and sural nerve biopsy revealed demyeli-nating polyneuropathy. Steroid therapy led to improvement in clinical symptoms and proteinuria.

루프스 신염의 치료

이규백 ( Kyu Beck Lee ) 대한신장학회 2009 Kidney Research and Clinical Practice Vol.28 No.2

칼슘길항제가 급성 허혈성 신손상후 Renin 유전자 발현에 미치는 효과

이규백(Kyu Beck Lee),차대룡(Dae Ryong Cha),김용섭(Yong Seop Kim),조원용(Won Yong Cho),김형규(Hyoung Kyu Kim) 대한내과학회 1997 대한내과학회지 Vol.53 No.3

N/A Objectives: lschemic acute renal failure(ARF) is characterized by an abrupt and sustained decline in GFR within minutes to days after renal ischemia and not immediately reversed on restoration of renal blood flow. The typical delay of a few days to a few weeks suggests reversible parenchymal damage awaiting cell regeneration for functional recovery. Many potentially cell damaging factors, such as ATP depletion, plasma membrane phospholipid degradatian and superoxide-induced membrane damage, play a central part in ischemic injury. More recently, much attention has been focused on the role of calcium, especially ischemic cell injury and the possible therapeutic role of calcium channel blockers emerged from studies conducted several years ago. In the past, it was thought that activation of renin-angiotensin system plays a role in the pathogenesis of ARF. Now the role of angiotensin in human renal ischemia also appears to be controversial. The following study was done in order to investigate the effect of a calcium channel blocker, nifedipine, on gene expression of renin during acute ischemic renal injury. Methods: The Sprague-Dawley rats were divided into 4 groups, group I(n=3) as the control, group II (n=3) as the sham operation group, group III(n=15) as the ischemic renal injury group without nifedipine pretreatment, and group IV(n=15) as the ischemic renal injury model by right nephrectomy and left renal artery clamping for 40 minutes with systemic nifedipine pretreatment(10mg/kg), 1n ischemic renal injury model(group III and IV), rats were further divided into three subgroups according to reperfusion time of 1,24,72 hours. The non-ischemic right kidney removed at the time of initial procedure served as paired control. Total renal RNA was extracted by Chomczynskis method and electrophoresis was done in a 1% agarose gel containing 2,2M formaldehyde. Northern was performed at 42℃ with isotope labeled renin probe for 18 hours, Autoradiographs were obtained and quantitated by a densitometer measured at 530nm. Results: 1) The expression of renin gene was markedly decreased after renal ischemia and slowly recovered to one half of the control level after 72 hours of reperfusion. 2) Renin gene expression pattern of ischemic renal injury with prior nifedipine treatment was similar to the ischemic group without nifedipine pretreatment. Conclusion: These findings suggest that the renin gene expression was markedly decreased after renal ischemia and slowly recovered. Systemic nifedipine pretreatment does not have a significant effect on gene expression pattern of renin in ischemic renal injury.

Duplication and deletion of 21 hydroxylase gene among the normal Korean subjects and adrenogenital syndrome patients

Dong Kyu Jin,Nam Seon Beck,Phil Soo Oh,Hye Zin Whang,Si Whan Koh,Jung Sim Kim,Myung Ryurl Oh 대한의학유전학회 1997 대한의학유전학회지 Vol.1 No.1

Steroid 21 hydroxylase deficiency is a major cause of congenital adrenal hyperplasia (CAH) and is caused by genetic impairment of the gene (CYP21B). In the human genome, CYP21B is located within the MHC class Ⅲ region on the short arm of chromosome 6. Most of the genes in this region are highly polymorphic and crowded. Also the CYP21B gene is accompanied by its pseudogene (CYP21A) and tandemly arranged with two genes of the fourth component of complement. A highly complex gene cluster in this area may predispose genetic instability of CYP21, i.e. mutations. In this study, we tried to investigate the frequency of duplication and deletion of CYP21 and patterns of the genetic alterations of these genes. We also compared the genetic alterations in normal subjects with those of the CAH patients. The results showed that 15% of the normal Korean population have duplication or deletion of CYP21. There was one normal subject heterozygous for the deletion of CYP21B. of the 5 CAH patients examined, 2 were found to show abnormal patterns. One was a large-scale gene conversion and the other a gene conversion associated with deletion involving both CYP21B and C4 locus Ⅱ gene. Through this study, we came to the conclusion that the duplication or even deletion of CYP21 and C4 might be quite a common event in the Korean population and these rearrangements must be regarded as polymorphisms. It could contribute to a high incidence of CAH by providing a genetic pool of instable CYP21.