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Review Article
Nephrology (Genitourinary)
Obesity and chronic kidney disease: prevalence, mechanism, and management
Hyung Eun Yim, Kee Hwan Yoo
Clin Exp Pediatr. 2021;64(10):511-518.   Published online April 6, 2021
· Obesity is strongly associated with the development and progression of chronic kidney disease.
· Altered renal hemodynamics, metabolic effects, and lipid nephrotoxicity may play a key role in the development of obesity-related kidney disease.
· Children born to obese mothers are at increased risk of developing obesity and chronic kidney disease later in life.
· A multilevel approach is needed to prevent obesity and related chronic diseases.
Original Article
Cardiology
Infantile Marfan syndrome in a Korean tertiary referral center
Yeon Jeong Seo, Ko-Eun Lee, Gi Beom Kim, Bo Sang Kwon, Eun Jung Bae, Chung Il Noh
Clin Exp Pediatr. 2016;59(2):59-64.   Published online February 29, 2016
Purpose

Infantile Marfan syndrome (MFS) is a rare congenital inheritable connective tissue disorder with poor prognosis. This study aimed to evaluate the cardiovascular manifestations and overall prognosis of infantile MFS diagnosed in a tertiary referral center in Korea.

Methods

Eight patients diagnosed with infantile MFS between 2004 and 2014 were retrospectively evaluated.

Results

Their median age at the time of diagnosis was 2.5 months (range,...

Case Report
Aortic valve replacement surgery for a case of infantile Takayasu arteritis
Hye Won Kwon, Yoon Jung Suh, Ji Seok Bang, Bo Sang Kwon, Gi Beom Kim, Eun Jung Bae, Woong Han Kim, Chung Il Noh
Clin Exp Pediatr. 2012;55(7):254-258.   Published online July 17, 2012

Takayasu arteritis is a chronic inflammatory disease of unknown etiology primarily affecting the aorta and its major branches and usually occurring in the second or third decade of life. Here, we report a case of Takayasu arteritis in a 10-month-old patient. The infant presented with signs of congestive heart failure and severe aortic regurgitation. Echocardiography and computed tomography angiography showed...

Original Article
Clinical characteristics and prevalence of vitamin D insufficiency in children less than two years of age
Ji Hyun Yoon, Cheong Soo Park, Ji Young Seo, Yun Sun Choi, Young Min Ahn
Clin Exp Pediatr. 2011;54(7):298-303.   Published online July 31, 2011
Purpose

To evaluate the clinical characteristics of vitamin D deficiency and its association with iron deficiency anemia (IDA).

Methods

A total of 171 children aged less than two years underwent 25-hydroxyvitamin D3 tests between January 2007 and July 2009. The study was classified into two groups: normal and vitamin D insufficiency, by their vitamin 25-hydroxyvitamin D3 levels.

Results

In total, 120 children were in the...

Case Report
An atypical phenotype of hypokalemic periodic paralysis caused by a mutation in the sodium channel gene SCN4A
Yang Hee Park, June Bum Kim
Clin Exp Pediatr. 2010;53(10):909-912.   Published online October 31, 2010

Familial hypokalemic periodic paralysis is an autosomal-dominant channelopathy characterized by episodic muscle weakness with hypokalemia. The respiratory and cardiac muscles typically remain unaffected, but we report an atypical case of a family with hypokalemic periodic paralysis in which the affected members presented with frequent respiratory insufficiency during severe attacks. Molecular analysis revealed a heterozygous c.664 C>T transition in the sodium...

Original Article
Risk factor for pituitary dysfunction in children and adolescents with Rathke's cleft cysts
Han Hyuk Lim, Sei Won Yang
Clin Exp Pediatr. 2010;53(7):759-765.   Published online July 31, 2010
Purpose

This study evaluated the clinical manifestations of and risk factors for pituitary insufficiency in children and adolescents with Rathke's cleft cysts.

Methods

Forty-four patients with Rathke's cleft cysts younger than 19 years who visited Seoul National University Children's Hospital between January 1995 and September 2009 were enrolled. Rathke's cleft cysts were confirmed histologically through an operation in 15 patients and by brain...

Outcome and risk factors of pediatric hemato-oncology patients admitted in pediatric intensive care unit
Bo Eun Kim, Eun Ju Ha, Keun Wook Bae, Seongguk Kim, Ho Joon Im, Jong Jin Seo, Seong Jong Park
Clin Exp Pediatr. 2009;52(10):1153-1160.   Published online October 15, 2009
Purpose : To evaluate the risk factors for mortality and prognostic factors in pediatric hemato-oncology patients admitted to the pediatric intensive care unit (PICU). Methods : We retrospectively reviewed the medical records of pediatric hemato-oncology patients admitted at the PICU of the Asan Medical Center between September 2005 and July 2008. Patients admitted at the PICU for perioperative or terminal...
The impact of early detection through school urinary screening tests of membranoproliferative glomerulonephritis typeⅠ
Sung-Hoon Chung, Sung-Sin Park, Sung-Do Kim, Byoung-Soo Cho
Clin Exp Pediatr. 2007;50(11):1104-1109.   Published online November 15, 2007
Purpose : Since 1998, school urinary screening tests have been performed on Korean school children. We could detect and treat so many asymptomatic chronic renal disease in early stage. We investigated the efficacy of school urinary screening tests from children with membranoproliferative glomerulonephritis (MPGN) type I. Methods : We analyzed the characteristics and prognosis of 18 patients with MPGN type I...
Review Article
Evaluation of function and disorders of the adrenal gland in neonates
Il Tae Hwang
Clin Exp Pediatr. 2007;50(3):236-240.   Published online March 15, 2007
Majority of sick full term newborns have adequate adrenal cortical function in response to stress. Acutely ill neonates with a basal cortisol level less than 15 g/dL (414 nmol/L) suggest adrenal insufficiency and require function testing of adrenal function. In premature infant, immaturity of hypothalamic-pituitary adrenal axis (HPA axis), may limit the ability to increase cortisol production in response...
Original Article
Evaluation of Prognostic Factors in Corrected Transposition of the Great Arteries at Mid-term Follow-up
Young-Hwan Song, Hyok-Joo Kwon, Gi-Beom Kim, Soo-Jung Kang, Eun-Jung Bae, Chung-Il Noh, Yong-Soo Yun, Jeong-Ryul Lee, Yong-Jin Kim, Joon-Ryang Rho
Clin Exp Pediatr. 2003;46(2):154-161.   Published online February 15, 2003
Purpose : The prognosis of patients with corrected transposition of the great arteries(C-TGA) is variably affected by associated intracardiac defects, systemic right ventricular function, tricuspid valve competence, and conduction disturbances. This study aims to evaluate the importance of those factors at mid-term follow-up. Methods : Medical records of 94 patients(males 58, females 36; mean age at last follow-up, 12? years; mean...
Case Report
A Case of Neonatal Cholestasis with Arthrogryposis Multiplex Congenita and Renal Tubular Insufficiency(ARC Syndrome)
Hi Soo Rhee, Soon Young Kim, Nam Sun Baik, Il Soo Ha, Jeong Kee Seo
Clin Exp Pediatr. 1996;39(1):126-130.   Published online January 15, 1996
We report a case of ARC syndrome with arthrogryposis multiplex congenita, renal tubular insufficiency and cholestasis. The Patient presented in the early neonatal period with micrognathia, low set ears, high arched palate, multiple joint contracture, conjugated hyperbilirubinemia and failure to thrive. He died at the age of 1 month despite medical therapy. Findings of renal tubular insufficiency included persistent renal...
Original Article
Clinical Study of Subarterial Ventricular Septal Defect
Yon Sook Rho, Yong Won Park, Sang Woo Kim, Bon Il Ku, Sang Joon Oh, Hong Sup Lee, Chang Ho Kim
Clin Exp Pediatr. 1995;38(4):493-500.   Published online April 15, 1995
Purpose : The incidence of subarterial ventricular septal defect(SA VSD) ranges 25-30% among oriental patients with VSDs, which is greater than 5% reported in western. Natural history of the disease is characterized by progressive aortic valve prolapse(AVP), frequently subarterial VSD, we evaluated clinical characteristics emphasizing on the incidence of AVP and the degree of AI as aging. Methods : Study subjects...
Clinical Assessment of Neonatal Transient Tricuspid Insuffieciency - Doppler Echocardiographic Study-
Se Geun Park, Dong Gun Park, Ji Hee Park, Chang Sung Son, Joo Won Lee, Young Chang Tockgo
Clin Exp Pediatr. 1993;36(6):785-790.   Published online June 15, 1993
Eight neonates with transient tricuspid insufficiency are presented which was confirmed clinical and two dimensional echocardiographic assessment. We found that two dimensional Doppler echocardiography was very useful in the detection of transient tricuspid insufficiency during neonatal age as noninvasive method. Transient tricuspid insufficiency is a clinical disorder in the newborn period caused by myocardial dysfunction, secondary to asphyxia with or without...
A case of Addison's disease.
Cheol Hee Hwang, Dong Kui Lee, Myung Sug Nam, Mun Ki Cho
Clin Exp Pediatr. 1991;34(8):1169-1174.   Published online August 31, 1991
Addison’s disease is a rare disorder resulting from progressive adrenocortical destruction regardless of the nature of underlying process. A 5-year-old boy with Addison’s disease without other endocrine disorder presented with a brief review of the literature. The patient was admitted to out hospital because of poor appetite and generalized darkish brown pigmentation of skin and mucous membrane. On physical examination, skin and mucous membrane were...
A case of Transient Tricuspid Insufficiency of Newborn(TTI).
Young Kyoo Shin, Eui Kyung Jung, Sang Hee Kim, Chang Sung Son, Joo Won Lee, Young Chang Tockgo
Clin Exp Pediatr. 1990;33(4):534-538.   Published online April 30, 1990
Transient Tricuspid Insufficiency(TTI) of newborn is a feature of myocardial dysfunction caused by myocardial hypoxemia secondary to reduced arterial oxygen content and excessive right ventricular work consequent to pulmonary hypertension.' Since Doppler echocardiography has been used to diagnose cardiac disease, it allows recording of regurgitant turbulent flow pattern. We report one case of TTI in a 36 week-gestational-aged newborn detected by continuous wave Dopplar echocardiography...
A Case of Adrenoleukodystrophy.
Kee Sung Yang, Soo Tack Bae, Hong Bae Kim, Ji Sub Oh
Clin Exp Pediatr. 1989;32(3):430-437.   Published online March 31, 1989
We experienced a case of Adrenoleukodystrophy (ALD), which is a genetically determined metabolic disorder associated with progressive demyelination of brain white matter and adrenal insuficiency. An 11 year old male revealed rapidly evolving neuologic progression, decreased adrenal reserve, increased plasma content of very long chain fatty acids (VLCFAs) and typical radiological findings. We report here the clinical findings of this patient with brief review of...
A Case of Addison's Disease.
In Hoon Lee, H. D. Lee, Hee Ju Jeon, Chan Yung Kim
Clin Exp Pediatr. 1988;31(12):1689-1695.   Published online December 31, 1988
A 9-year-old girl with Addison’s disease without other endocrine disorder presented with a brief review of the literature. The patient was transferee! to our hospital because of darkish brown pigmentation of whole body and generalized weakness which had misunderstood cyanotic heart disease. On physical examination, she was poorly nourished and below 10 percentile in weight and height. Skin and mucous membrane were diffusely darkish brown,...
Case Report
A Case of Isolated Congenital Tricuspid Insufficiency.
Dong Hyun Choo, Dong Kyoon Kim, Jung Yeon Choi, Yong Soo Yoon, Chang Yee Hong
Clin Exp Pediatr. 1984;27(8):814-819.   Published online August 31, 1984
Isolated congenital tricuspid insufficiency is a rare anomaly and may be caused by abnormal chordae tendinae, papillary muscle, or valve leaflets. The cause of these lesions is generally ascribed to incomplete undermining of primitive endocardial cushion by sinusoidal space, which differentiate the cushion into the various valvular structures. Carvallo’s sign, most characteristic clinical sign in tricuspid insufficiency, is augumentation of systolic...