A Pediatric Case of Inflammatory Bowel Disease with Renal Amyloidosis

Hyesun Hyun,Eujin Park,Ji Hyun Kim,Myung Hyun Cho,강희경,Jin Soo Moon,문경철,하일수,정해일 대한소아신장학회 2018 Childhood kidney diseases Vol.22 No.2

Amyloidosis is a rare disease that results from the deposition of extracellular protein in various body tissues, causing progressive organ dysfunction. Secondary renal amyloidosis is a rare but serious complication of chronic inflammatory bowel disease, particularly in patients with Crohn’s disease or ulcerative colitis. We report a case of secondary renal amyloidosis in a pediatric patient who reported a 16- year history of “very early onset inflammatory bowel disease”. Intensive treatment including repeated infliximab infusions improved clinical parameters of inflammatory bowel disease, although renal dysfunction showed progression. Amyloidosis should be considered in patients with IBD, particularly if they suffered disease progression.

A Pediatric Case of Inflammatory Bowel Disease with Renal Amyloidosis

Hyun, Hyesun,Park, Eujin,Kim, Ji Hyun,Cho, Myung Hyun,Kang, Hee Gyung,Moon, Jin Soo,Moon, Kyung Chul,Ha, Il-Soo,Cheong, Hae Il Korean Society of Pediatric Nephrology 2018 Childhood kidney diseases Vol.22 No.2

Amyloidosis is a rare disease that results from the deposition of extracellular protein in various body tissues, causing progressive organ dysfunction. Secondary renal amyloidosis is a rare but serious complication of chronic inflammatory bowel disease, particularly in patients with Crohn's disease or ulcerative colitis. We report a case of secondary renal amyloidosis in a pediatric patient who reported a 16-year history of "very early onset inflammatory bowel disease". Intensive treatment including repeated infliximab infusions improved clinical parameters of inflammatory bowel disease, although renal dysfunction showed progression. Amyloidosis should be considered in patients with IBD, particularly if they suffered disease progression.

Efficacy and safety of losartan in childhood immunoglobulin A nephropathy: a prospective multicenter study

Hyun Hyesun,Ahn Yo Han,Park Eujin,Choi Hyun Jin,Han Kyoung Hee,Lee Jung Won,Kim Su Young,Yang Eun Mi,Suh Jin-Soon,Shin Jae Il,Cho Min Hyun,Koo Ja Wook,Kim Kee Hyuck,Park Hye Won,Ha Il-Soo,Cheong Hae I 대한소아신장학회 2023 Childhood kidney diseases Vol.27 No.2

Purpose: Angiotensin-converting enzyme inhibitors and angiotensin II receptor blockers (ARBs) are frequently employed to counteract the detrimental effects of proteinuria on glomerular diseases. However, the effects of ARBs remain poorly examined in pediatric patients with immunoglobulin A (IgA) nephropathy. Herein, we evaluated the efficacy and safety of losartan, an ARB, in pediatric IgA nephropathy with proteinuria. Methods: This prospective, single-arm, multicenter study included children with IgA nephropathy exhibiting proteinuria. Changes in proteinuria, blood pressure, and kidney function were prospectively evaluated before and 4 and 24 weeks after losartan administration. The primary endpoint was the difference in proteinuria between baseline and 24 weeks. Results: In total, 29 patients were enrolled and received losartan treatment. The full analysis set included 28 patients who received losartan at least once and had pre- and post-urinary protein to creatinine ratio measurements (n=28). The per-protocol analysis group included 22 patients who completed all scheduled visits without any serious violations during the study period. In both groups, the mean log (urine protein to creatinine ratio) value decreased significantly at 6 months. After 24 weeks, the urinary protein to creatinine ratio decreased by more than 50% in approximately 40% of the patients. The glomerular filtration rate was not significantly altered during the observation period. Conclusions: Losartan decreased proteinuria without decreasing kidney function in patients with IgA nephropathy over 24 weeks. Losartan could be safely employed to reduce proteinuria in this patient population. ClinicalTrials.gov trial registration (NCT0223277)

C3 Glomerulonephritis associated with Anti-complement Factor H Autoantibodies in an Adolescent Male: A Case Report

Hyun, HyeSun,Kang, Hee Gyung,Cho, UiJu,Ha, Il-Soo,Cheong, Hae Il Korean Society of Pediatric Nephrology 2021 Childhood kidney diseases Vol.25 No.1

C3 glomerulonephritis (C3GN), a rare condition associated with dysregulation of the alternative pathway of the complement system, is histopathologically characterized by isolated or dominant C3 deposition in the renal glomeruli. We report a case of C3GN associated with anti-complement factor H (CFH) autoantibodies and CHF-related protein deficiency in an adolescent male. A 16-year-old adolescent male was admitted to a hospital with a 1-month history of generalized edema prior to presentation. Persistent microscopic hematuria and low serum C3 levels were incidentally detected at 7 and 10 years of age, respectively. Laboratory test results revealed hypoalbuminemia, nephrotic-range proteinuria, microscopic hematuria, and normal serum creatinine levels. The serum C3 and C4 levels were 17 mg/dL (normal 80-150 mg/dL) and 22 mg/mL (17-40 mg/mL), respectively. Renal biopsy showed typical features of C3GN. Further investigations revealed positive results on plasma anti-CFH autoantibody testing and a homozygous deletion of CFHR1 and CFHR3, which encode CFH-related proteins 1 and 3, respectively. Proteinuria persisted despite treatment with intravenous methylprednisolone, mycophenolate mofetil, and angiotensin-receptor blocker; however, his renal function remained stable. In conclusion, anti-CFH autoantibodies serve as important contributors to C3GN. This is the first case report that describes C3GN in an adolescent Korean male with anti-CFH autoantibodies and homozygous CFHR1 and CFHR3 deletion.

Prognostic assessment of sarcomatous histologic subtypes of ovarian carcinosarcoma

( Hyun Jin Kim ),( Hyun-mi Lee ),( Mi Kyung Kim ),( Yoo-kyung Lee ),( In-ho Lee ),( Ki-heon Lee ),( Hyesun Kim ) 대한산부인과학회 2017 Obstetrics & Gynecology Science Vol.60 No.4

Objective Ovarian carcinosarcoma is a rare subtype of this disease that has not been thoroughly investigated. The aim of this study was to evaluate the prognostic factors and out comes in patients with ovarian carcinosarcoma. Methods All patients with histologically confirmed ovarian carcinosarcoma who were treated at Cheil General Hospital and Women`s Healthcare Center between January 2000 and December 2015 were identified and analyzed. Data were extracted from medical records, and statistical analyses were performed to determine correlations between clinicopathological parameters and survival outcomes. Results Of the 822 patients diagnosed with ovarian cancer over 16 years, 11 (1.3%) had ovarian carcinosarcoma histology. Every patient underwent surgery as the initial treatment followed by intravenous adjuvant chemotherapy. Only 18.1% of cases were early stage (I or II) while 81.8% were advanced stage (III or IV) according to the FIGO (International Federation of Gynecology and Obstetrics) classification. Six cases were of the homologous subtype (54.5%) and five were of the heterologous subtype (45.5%). There was no significant difference in survival according to stage (P=0.24). The heterologous subtype and residual disease were associated with poor disease-free survival (P=0.02 and P=0.04) and overall survival (P=0.02 and P=0.04), On multivariate analysis, the histological subtype was an independent prognostic factor (P=0.02). Conclusion Optimal cytoreduction without gross residual disease and a homologous subtype are favorable prognostic factors in terms of disease relapse and survival.

Surface modification of polymer nanoparticles with native albumin for enhancing drug delivery to solid tumors

Hyun, Hyesun,Park, Joonyoung,Willis, Kiela,Park, Ji Eun,Lyle, L. Tiffany,Lee, Wooin,Yeo, Yoon Elsevier 2018 Biomaterials Vol.180 No.-

<P><B>Abstract</B></P> <P>Albumin is a promising surface modifier of nanoparticulate drug delivery systems. Serving as a dysopsonin, albumin can protect circulating nanoparticles (NPs) from the recognition and clearance by the mononuclear phagocytic system (MPS). Albumin may also help transport the NPs to solid tumors based on the increased consumption by cancer cells and interactions with the tumor microenvironment. Several studies have explored the benefits of surface-bound albumin to enhance NP delivery to tumors. However, it remains unknown how the surface modification process affects the conformation of albumin and the performance of the albumin-modified NPs. We use three different surface modification methods including two prevalent approaches (physisorption and interfacial embedding) and a new method based on dopamine polymerization to modify the surface of poly(lactic-co-glycolic acid) NPs with albumin and compare the extent of albumin binding, conformation of the surface-bound albumin, and biological performances of the albumin-coated NPs. We find that the dopamine polymerization method preserves the albumin structure, forming a surface layer that facilitates NP transport and drug delivery into tumors via the interaction with albumin-binding proteins. In contrast, the interfacial embedding method creates NPs with denatured albumin that offers no particular benefit to the interaction with cancer cells but rather promotes the MPS uptake via direct and indirect interactions with scavenger receptor A. This study demonstrates that the surface-bound albumin can bring distinct effects according to the way they interact with NP surface and thus needs to be controlled in order to achieve favorable therapeutic outcomes.</P> <P><B>Graphical abstract</B></P> <P>[DISPLAY OMISSION]</P>

Long-Term Outcomes of Pediatric Renovascular Hypertension

Chung, Hyun,Lee, Jae Hwan,Park, Eujin,Hyun, Hyesun,Ahn, Yo Han,Jae, Hwan Jun,Kim, Gi Beom,Ha, Il Soo,Cheong, Hae Il,Kang, Hee Gyung Karger Medical and Scientific Publishers 2017 Kidney & blood pressure research Vol. No.

<P>Background/Aims: Renovascular hypertension (RVHT) is an important cause of childhood hypertension. This study evaluated the clinical characteristics and outcomes of Korean children with RVHT. Methods: Children treated for RVHT between 2000 and 2015 at our center were retrospectively reviewed. Results: Forty-six children were followed for a median of 6.5 (0.66-27.23) years. Forty-five percutaneous transluminal angioplasties (PTAs) were performed in 32 children. At the last visit, clinical benefit was observed in 53.3% of children. Patients with comorbid cerebrovascular disease (CVD) showed less favorable long-term outcomes after PTA (clinical benefit in 41.7% vs. 61.1% in others) and higher restenosis rates (50% vs. 31.6% in others). Surgical procedures (bypass or nephrectomy) were performed in 8 patients. After surgery, blood pressure was normalized in 2 patients, improved in 3 patients, and unchanged in the remaining patients. Between PTA group (n=21) and medication group (n=14), percentage of atrophic kidneys became higher after follow-up period in medication group than in PTA group (60.0% vs. 26.1%, P=0.037). Conclusion: Aggressive treatment of pediatric RVHT yielded fair outcomes in our cohort. CVD comorbidity was associated with relatively poor PTA outcomes. To confirm our findings, larger cohort studies with a longer follow-up period are warranted. (c) 2017 The Author(s) Published by S. Karger AG, Basel</P>

Case Report : Acute Pseudogout of the Neck: "Crowned Dens" Revisited

( Hyesun Lee ),( Hyeon Su Kim ),( Ui Hong Jung ),( Hyun Hee Kwon ),( Young Hwan Lee ),( Sung Hoon Park ) 대한류마티스학회 2016 대한류마티스학회지 Vol.23 No.2

Calcium pyrophosphate dihydrate crystal deposition disease is associated with an acute mono- or pauciarthritis, termed “pseudogout” in elderly patients, involving a large joint (including the knees, ankles) or a chronic arthropathy manifesting as mild joint pain and stiffness. Pseudogout is a crystal-deposition disease of peripheral joints, usually encountered in elderly patients. However, acute presentation of pseudogout around the odontoid process comprises a “crowned-dens” appearance, and requires contemplation of differential diagnoses. We recently experienced a case of pseudogout in the cervical spine presenting with fever and acute neck pain that was successfully treated with a colchicine and low-dose oral steroid. We reported this case with a review of the relevant literature. (J Rheum Dis 2016;23:122-124)

통합적 기업이미지 관리에서의 칼라마케팅의 역할

오현정(Oh Hyun-jung),이혜선(Lee Hyesun) 한국색채학회 2005 한국색채학회 논문집 Vol.19 No.1

한국인의 요오드 섭취와 요오드 상한섭취량

이현숙 (Lee,Hyun Sook ),민혜선 (Min,Hyesun ) 韓國營養學會 2011 Journal of Nutrition and Health Vol.44 No.1

The present study reviewed the effects of excess iodine intake on thyroid function and the incidence of thyroid disease and discussed the scientific basis for establishing a tolerable upper intake level (UL) of iodine for Koreans. ULs are defined as “the highest level of daily nutrient intake that is likely to pose no risk of adverse effects to almost all individuals in the general population.” Koreans consume excess iodine from seaweed, and iodine intake is strongly influenced by seaweed consumption. However, no dose-response data derived from subjects consuming excess iodine frequently but not continuously during a lifetime are available. Therefore, the Korean DRI committee set the iodine UL to reduce the risk of adverse health effects by excess iodine intake for Koreans with distinctive seaweed-eating habits. (Korean J Nutr 2011 44(1): 82 ~ 91)