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Case Report
Reninoma: a rare cause of curable hypertension
Ji Hye Kim, Ji Hyun Kim, Myung Hyun Cho, Eujin Park, Hye Sun Hyun, Yo Han Ahn, Hee Gyung Kang, Kyung Chul Moon, Il-Soo Ha, Hae Il Cheong
Clin Exp Pediatr. 2019;62(4):144-147.   Published online October 29, 2018
The most common type of refractory hypertension found in children is secondary hypertension, which is a potentially curable disease. Reninoma, a renin-secreting juxtaglomerular cell tumor, is a rare cause of severe hypertension that is usually diagnosed in adolescents and young adults. Surgical resection of the tumor completely cures the hypertension of patients with reninoma. The typical clinical presentation of reninoma...
Original Article
Oncology
Posttransplantation lymphoproliferative disorder after pediatric solid organ transplantation: experiences of 20 years in a single center
Hyung Joo Jeong, Yo Han Ahn, Eujin Park, Youngrok Choi, Nam-Joon Yi, Jae Sung Ko, Sang Il Min, Jong Won Ha, Il-Soo Ha, Hae Il Cheong, Hee Gyung Kang
Clin Exp Pediatr. 2017;60(3):86-93.   Published online March 27, 2017
Purpose

To evaluate the clinical spectrum of posttransplantation lymphoproliferative disorder (PTLD) after solid organ transplantation (SOT) in children.

Methods

We retrospectively reviewed the medical records of 18 patients with PTLD who underwent liver (LT) or kidney transplantation (KT) between January 1995 and December 2014 in Seoul National University Children's Hospital.

Results

Eighteen patients (3.9% of pediatric SOTs; LT:KT, 11:7; male to female, 9:9) were diagnosed...

Case Report
Immunology
A familial case of Blau syndrome caused by a novel NOD2 genetic mutation
Woojoong Kim, Eujin Park, Yo Han Ahn, Jiwon M. Lee, Hee Gyung Kang, Byung Joo Kim, Il-Soo Ha, Hae Il Cheong
Clin Exp Pediatr. 2016;59(Suppl 1):S5-S9.   Published online November 30, 2016

Blau syndrome (BS) is a rare autosomal dominant, inflammatory syndrome that is characterized by the clinical triad of granulomatous dermatitis, symmetric arthritis, and recurrent uveitis. Mutations in the nucleotide oligomerization domain 2 (NOD2) gene are responsible for causing BS. To date, up to 30 Blau-associated genetic mutations have been identified within this gene. We report a novel NOD2 genetic mutation...

Genetics and Metabolism
A nonsense PAX6 mutation in a family with congenital aniridia
Kyoung Hee Han, Hye Jin Lee, Il-Soo Ha, Hee Gyung Kang, Hae Il Cheong
Clin Exp Pediatr. 2016;59(Suppl 1):S1-S4.   Published online November 30, 2016

Congenital aniridia is a rare ocular malformation that presents with severe hypoplasia of the iris and various ocular manifestations. Most cases of congenital aniridia are known to be related to mutations in the paired box gene-6 (PAX6), which is an essential gene in eye development. Herein, we report a familial case of autosomal dominant congenital aniridia with four affected members...

Nephrology (Genitourinary)
Bilateral iliac and popliteal arterial thrombosis in a child with focal segmental glomerulosclerosis
Kyoung Hee Han, Ji Youn Park, Seung-Kee Min, Il-Soo Ha, Hae Il Cheong, Hee Gyung Kang
Clin Exp Pediatr. 2016;59(5):242-245.   Published online May 31, 2016

Thromboembolic complications (TECs) are clinically important sequelae of nephrotic syndrome (NS). The incidence of TECs in children is approximately 2%–5%. The veins are the most commonly affected sites, particularly the deep veins in the legs, the inferior vena cava, the superior vena cava, and the renal veins. Arterial thrombosis, which is less common, typically occurs in the cerebral, pulmonary, and...

Original Article
Intravenous fluid prescription practices among pediatric residents in Korea
Jiwon M. Lee, Younghwa Jung, Se Eun Lee, Jun Ho Lee, Kee Hyuck Kim, Ja Wook Koo, Young Seo Park, Hae Il Cheong, Il-Soo Ha, Yong Choi, Hee Gyung Kang
Clin Exp Pediatr. 2013;56(7):282-285.   Published online July 19, 2013
Purpose

Recent studies have established the association between hypotonic fluids administration and hospital-acquired hyponatremia in children. The present paper investigated the pattern of current practice in intravenous fluid prescription among Korean pediatric residents, to underscore the need for updated education.

Methods

A survey-based analysis was carried out. Pediatric residents at six university hospitals in Korea completed a survey consisting of four questions. Each...

The Preventive Effect of Dexrazoxane and Pentoxifylline on Adriamycin Induced Cardiomyopathy
Ling Zhu, Eun-Jung Bae, Il-Soo Ha, Jung-Wook Seo, Chung-Il Noh, Jung-Yun Choi, Yong-Soo Yun
Clin Exp Pediatr. 2005;48(12):1378-1384.   Published online December 15, 2005
Purpose : We hypothesized dexrazoxane(DXR) and pentoxifylline(PTX) may prevent myocardial damage in adriamycin(ADR)-induced cardiomyopathic rat model. We also investigated their effects on the myocardial apoptosis and fibrosis in ADR induced cardiomyopathy. Methods : The six-week old female Spregue-Dawley rats were divided into control group(CNT, n= 4), ADR group(n=6), ADR+DXR group(DXR, n=5), ADR+PTX group(PTX, n=6), ADR+DXR+PTX group(DXPT, n=5). ADR(5 mg/week, twice) was...
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