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Clinical features and outcomes of focal segmental glomerulosclerosis pathologic variants in Korean adult patients

 Young Eun Kwon  ;  Seung Hyeok Han  ;  Jeong Hae Kie  ;  Seong Yeong An  ;  Yung Ly Kim  ;  Kyoung Sook Park  ;  Ki Heon Nam  ;  Ah Young Leem  ;  Hyung Jung Oh  ;  Jung Tak Park  ;  Tae Ik Chang  ;  Ea Wha Kang  ;  Shin-Wook Kang  ;  Kyu Hun Choi  ;  Beom Jin Lim  ;  Hyeon Joo Jeong  ;  Tae-Hyun Yoo 
 BMC NEPHROLOGY, Vol.15(52) : 1-9, 2014 
Journal Title
Issue Date
Age of Onset ; Asian Continental Ancestry Group* ; Biomarkers/blood ; Comorbidity ; Creatinine/blood* ; Female ; Glomerulosclerosis, Focal Segmental/blood ; Glomerulosclerosis, Focal Segmental/diagnosis ; Glomerulosclerosis, Focal Segmental/ethnology* ; Glomerulosclerosis, Focal Segmental/pathology* ; Glomerulosclerosis, Focal Segmental/therapy ; Humans ; Incidence ; Kidney Failure, Chronic/diagnosis ; Kidney Failure, Chronic/ethnology ; Kidney Failure, Chronic/prevention & control ; Male ; Middle Aged ; Proteinuria/epidemiology ; Republic of Korea/epidemiology ; Retrospective Studies ; Risk Factors ; Survival Rate ; Treatment Outcome
Focal segmental glomerulosclerosis ; Pathology ; Outcome
Many studies have shown that clinical characteristics and outcomes differ depending on pathologic variants of focal segmental glomerulosclerosis (FSGS). However, these are not well defined in Asian populations.
This retrospective study evaluated clinical features and outcomes of pathologic FSGS variants in 111 adult patients between January 2004 and December 2012. Primary outcome was the composite of doubling of baseline serum creatinine concentrations (D-SCr) or onset of end-stage renal disease (ESRD). Secondary outcome included complete (CR) or partial remission (PR).
There were 70 (63.1%), 20 (18.0%), 17 (15.3%), 3 (2.7%), and 1 (0.9%) patients with not-otherwise specified (NOS), tip, perihilar, cellular, and collapsing variants, respectively. At presentation, nephrotic-range proteinuria occurred more commonly in tip lesion than in other variants. The overall 5-year renal survival rate was 76.8%. During a median follow-up of 34.5 months, only 1 (5.0%) patient with a tip lesion reached the composite end point compared to 2 (11.8%) and 12 (17.1%) patients in perihilar and NOS variants, but this difference was not statistically significant in an adjusted Cox model. However, tip lesion was associated with a significantly increased probability of achieving CR (P = 0.044).
Similar to other populations, Korean adult patients with FSGS have distinct clinical features with the exception of a rare frequency of cellular and collapsing variants. Although pathologic variants were not associated with overall outcome, the tip variant exhibited favorable outcome in terms of achieving remission. Further studies are required to delineate long-term outcome and response to treatment of the pathologic variants.
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1. College of Medicine (의과대학) > Dept. of Hospital Medicine (입원의학과) > 1. Journal Papers
1. College of Medicine (의과대학) > Dept. of Internal Medicine (내과학교실) > 1. Journal Papers
1. College of Medicine (의과대학) > Dept. of Pathology (병리학교실) > 1. Journal Papers
Yonsei Authors
Kang, Shin Wook(강신욱) ORCID logo https://orcid.org/0000-0002-5677-4756
Kang, Ea Wha(강이화)
Kwon, Young Eun(권영은)
Kim, Yung Ly(김영리)
Nam, Ki Heon(남기헌) ORCID logo https://orcid.org/0000-0001-7312-7027
Park, Kyoung Sook(박경숙)
Park, Jung Tak(박정탁) ORCID logo https://orcid.org/0000-0002-2325-8982
An, Seong Yeong(안성영)
Oh, Hyung Jung(오형중)
Yoo, Tae Hyun(유태현) ORCID logo https://orcid.org/0000-0002-9183-4507
Lim, Beom Jin(임범진) ORCID logo https://orcid.org/0000-0003-2856-0133
Leem, Ah Young(임아영) ORCID logo https://orcid.org/0000-0001-5165-3704
Chang, Tae Ik(장태익)
Jeong, Hyeon Joo(정현주) ORCID logo https://orcid.org/0000-0002-9695-1227
Choi, Kyu Hun(최규헌) ORCID logo https://orcid.org/0000-0003-0095-9011
Han, Seung Hyeok(한승혁) ORCID logo https://orcid.org/0000-0001-7923-5635
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