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359 results on '"Jeong, Hyeon Joo"'

9. Focal segmental glomerulosclerosis and medullary nephrocalcinosis in children with ADCK4 mutations

Author

Park, Eujin, Kang, Hee Gyung, Choi, Young Hun, Lee, Kyoung Bun, Moon, Kyung Chul, Jeong, Hyeon Joo, and Nagata, Michio

Subjects
Research, Risk factors, Gene mutation -- Research, Kidney diseases -- Risk factors -- Research
Abstract

Author(s): Eujin Park [sup.1] , Hee Gyung Kang [sup.1] [sup.2] , Young Hun Choi [sup.3] , Kyoung Bun Lee [sup.4] , Kyung Chul Moon [sup.4] [sup.5] , Hyeon Joo Jeong [...], Background Mutations in the AarF domain containing kinase 4 gene (ADCK4), one of the novel genes causing steroid-resistant nephrotic syndrome (SRNS), usually manifest as isolated adolescent-onset focal segmental glomerulosclerosis (FSGS). ADCK4 interacts with components of the coenzyme Q10 (CoQ10) biosynthesis pathway. Methods The incidence and phenotypes of patients with ADCK4 mutations were investigated in a cohort of Korean pediatric patients with SRNS. Results Among the 53 patients enrolled in the study the incidence of ADCK4-associated FSGS was 7.5% (n = 4) in children aged 5 years and older with multidrug-resistant FSGS. Two additional patients were included for phenotype analyses, one detected by family screening and the other with cyclosporine-responsive FSGS. These six patients presented proteinuria without overt nephrotic syndrome at a median age of 110 (range 60-153) months, of whom five progressed to end-stage renal disease within a median period of 46 (range 36-79) months after onset. Renal biopsies revealed mitochondrial abnormalities in podocytes and tubular cells of all patients. Notably, all patients showed accompanying medullary nephrocalcinosis. None of the patients showed other extrarenal manifestations. Conclusions ADCK4 mutations should be considered in older children presenting with steroid resistant FSGS. An early diagnosis of ADCK4 mutations is essential because the condition is treatable with CoQ10 supplementation at an early stage. The association with medullary nephrocalcinosis may be an additional diagnostic indicator.

Published
2017
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13. The significance of tubulointerstitial lesions in childhood Henoch-Schonlein nephritis

Author

Lim, Beom Jin, Shin, Jae Il, Choi, Sung-eun, Rhim, Hyechang, Lee, Jae Seung, Kim, Pyung Kil, Jeong, Hyeon Joo, and Kim, Ji Hong

Subjects
Care and treatment, Physiological aspects, Usage, Prognosis, Health aspects, Kidney biopsy -- Usage -- Health aspects, Nephritis -- Prognosis -- Care and treatment -- Physiological aspects
Abstract

Author(s): Beom Jin Lim[sup.1] [sup.2] , Jae Il Shin[sup.3] , Sung-eun Choi[sup.1] , Hyechang Rhim[sup.1] , Jae Seung Lee[sup.3] , Pyung Kil Kim[sup.3] , Hyeon Joo Jeong[sup.1] , Ji Hong [...], Background Little information is currently available on the development of tubulointerstitial lesions in children with Henoch-Schonlein nephritis (HSN). To identify the impact of the development of tubulointerstitial changes in HSN, we retrospectively analyzed renal biopsies obtained from children with HSN. Methods Twenty-eight children with HSN from whom serial renal biopsies had been obtained before and after immunosuppressive therapy were enrolled in the study. The patients were divided into two groups according to the observed change in tubulointerstitial lesion development: group I (n = 15), with stable or improved tubulointerstitial lesions, and group II (n = 13), with worsened tubulointerstitial lesions. Group II patients had longer duration of proteinuria than group I patients (3.7 ± 3.7 years vs. 1.7 ± 1.7 years, p = 0.052). Results The change in serum albumin level was negatively correlated with the change in tubulointerstitial scores before and after treatment ([gamma] = -0.444, p = 0.018). Group II patients showed a significant decrease in immunoglobulin G (IgG) and IgA deposits after treatment (p = 0.039 and 0.003, respectively), while group II patients did not (p = 0.458 and 0.506, respectively). Conclusions Although the International Study of Kidney Disease in Children classification of HSN does not include tubulointerstitial lesions, they can progress during treatment and could have significant clinical implications in association with the duration of proteinuria.

Published
2016
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29. Glomerular subepithelial microparticles - a footprint for podocyte injury

Author

Kim, Yon Hee, primary, Huh, Kyu Ha, additional, Lim, Beom Jin, additional, Kim, Beom Seok, additional, Kim, Yu Seun, additional, Kim, Soon Il, additional, Kim, Myoung Soo, additional, Lee, Juhan, additional, Park, Jung Tak, additional, Yoo, Tae-Hyun, additional, Kang, Shin-Wook, additional, Han, Seung Hyeok, additional, and Jeong, Hyeon Joo, additional

Published
2021
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