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Case Report
Neurology
Megalencephaly-capillary malformation-polymicrogyria syndrome: the first case report in Korea
Yeon-Chul Choi, Mi-Sun Yum, Min-Jee Kim, Yun-Jung Lee, Tae-Sung Ko
Clin Exp Pediatr. 2016;59(Suppl 1):S152-S156.   Published online November 30, 2016

Megalencephaly-capillary malformation-polymicrogyria syndrome (MCAP), previously known as macrocephaly-cutis marmorata telangiectatica congenita and macrocephaly-capillary malformation syndrome, is a rare multiple-malformation syndrome that is characterized by progressive megalencephaly, capillary malformations of the midline face and body, or distal limb anomalies such as syndactyly. Herein, we report a female infant case that satisfies the recently proposed criteria of MCAP and describe the distinctive...

Original Article
Effect of adenotonsillar hypertrophy on right ventricle function in children
Jin Hwan Lee, Jung Min Yoon, Jae Woo Lim, Kyung Og Ko, Seong Jun Choi, Jong-Yeup Kim, Eun Jung Cheon
Clin Exp Pediatr. 2014;57(11):484-488.   Published online November 30, 2014
Purpose

Chronic upper airway obstruction causes hypoxemic pulmonary vasoconstriction, which may lead to right ventricle (RV) dysfunction. Adenotonsillar hypertrophy (ATH) is the most common cause of upper airway obstruction in children. Therefore, we aimed to evaluate RV function in children with ATH.

Methods

Twenty-one children (male/female, 15/6; mean age, 92.3¡¾39.0 months; age range, 4-15 years) with ATH and 21 healthy age- and gender-matched...

Correlation between the morning hypertension on ambulatory blood pressure monitoring and the left ventricular mass in children
Hyun Jung Kim, Kyung Hee Kim, Hong Ryang Kil
Clin Exp Pediatr. 2014;57(9):403-409.   Published online September 30, 2014
Purpose

Although high morning blood pressure (BP) is known to be associated with the onset of cardiovascular events in adults, data on its effects in children with hypertension are limited. Our retrospective study aimed to define the clinical characteristics of children with morning hypertension (MH) and to determine its associated factors.

Methods

We reviewed 31 consecutive patients with hypertension, confirmed by the ambulatory...

Articulation error of children with adenoid hypertrophy
Tae-Hoon Eom, Eun-Sil Jang, Young-Hoon Kim, Seung-Yun Chung, In-Goo Lee
Clin Exp Pediatr. 2014;57(7):323-328.   Published online July 23, 2014
Purpose

Adenoid hypertrophy is a physical alteration that may affect speech, and a speech disorder can have other negative effects on a child's life. Airway obstruction leads to constricted oral breathing and causes postural alterations of several oro-facial structures, including the mouth, tongue, and hyoid bone. The postural modifications may affect several aspects of speech production.

Methods

In this study, we compared articulation...

Prevalence and Associated Factors of Snoring in School-Aged Children
Sung Jong Cho, Eun Young Kim, Young Il Rho, Eun Suk Yang, Young Bong Park, Kyung-Rye Moon, Chul-Gab Lee
Clin Exp Pediatr. 2002;45(11):1340-1345.   Published online November 15, 2002
Purpose : We studied the prevalence of snoring and its association with diseases, obesity and environmental factors, as well as sleep disturbance arising from snoring, in school-aged children. Methods : The survey was performed by a special questionnaire on 1,707 children at elementary schools from 1 to 30 July 2001 in Gwangju City. The prevalence of snoring, associated factors, and sleep...
Case Report
A Case of Cystic Pancreatoblastoma Associated with Congenital Hemihypertrophy
Hyun Joo Lee, Kyuong Ha Yoo, Young Mi Hong, Kyung hee Kim, Kum Ja Choi, Joung Hyun Yoo, Sung Sook Kim
Clin Exp Pediatr. 1996;39(6):861-865.   Published online June 15, 1996
We experenced a case of cystic pancreatoblastoma associated with congenital hemihypertrophy in a 4months old male. The mass was located on the anterior side of pancreatic head without any connection to the pancreas. After exision of cystic pancreatoblastoma, chemotherapy(FAM regimen) was performed 15 times due to capsular tumor invasion. Until this time there was no drug side effect and metastasis....
Original Article
Effects of Main Pulmonary Artery Constriction on the Right Ventricle in Rabbits
Chan Uhno Joo, Dong Geun Lee
Clin Exp Pediatr. 1995;38(8):1087-1092.   Published online August 15, 1995
Purpose : constriction or banding of the pulmonary artery tocreate pulmonalry artery stenosis is a palliative procedure designed to limit pulmonary blood flow in congenital cardiac malformation with unrestricted shunt flow from the systemic to pulmonary circuit. Increased pressure-load with the procedure results in ventricular hypertrophy in right ventricle. We studied effects of the pulmonary artery constriction on the right ventricle...
A Case of Beckwith-Wiedemann Syndrome.
E.W. Park, D.U. Kim, W.H. Choi, K.W. Kim
Clin Exp Pediatr. 1991;34(1):126-129.   Published online January 31, 1991
We experienced a case of Beck with-Wiedemann Syndrome. This 1-day-old female neonate manifested gigantism, macroglossia, anomaly of the umbilical cord, hemihypertrophy, mild he- patomegaly, congenital heart disease and severe hypoglycemia. A brief review of related literature was made.
A Case of Virginal Breast Hypertrophy.
S C Hamm, S W Oh, S S Shim, H D Lee, S Y Kim, H J Park, H J Jun, C Y Kim
Clin Exp Pediatr. 1990;33(12):1741-1747.   Published online December 31, 1990
A 12-year-old girl visited due to massive breast enlargement during past 10 months. We evaluated hormonal levels and radiologic studies but could not find the cause and diagnosed as virginal or juvenile breast hypertrophy. Hormonal therapy was not effective. We relieved her physical discom- fort or mental anguish with reduction mammoplasty and report with brief review of related literatur- es.
The Two Cases of Klippel-Trenaunay Weber Syndrome.
Man Chul Ha, In Hun Lee, Yong Tak Lim, Hi Joo Chun, Hi Ju Park, Chan Yung Kim
Clin Exp Pediatr. 1988;31(3):391-397.   Published online March 31, 1988
We experienced two cases of Klippel-Trenaunay-Weber Syndrome in 13 years old boy and 10 years old girl. They had shown large nevus flammeus, varicose veins, soft tissue and bong hypatrophy. In angrogam and venogram capillary and venous hemangioma were noted, but we could not find arteriovenous filstula. We report two cases of Klippel-Trenaunay-Weber Syndrome with review of literature.
Case Report
A Case of Kocher-Debre-Semelaigne Syndrome.
Saeng Gu Cho, Song Tae Kim, Young Youn Choi, Tai Ju Hwang
Clin Exp Pediatr. 1985;28(11):1130-1135.   Published online November 30, 1985
The authors experienced a 9-year-old male patient with Kocher-Decre-Semelaigne syndrome, hypothyroidism with generalized muscular hypertrophy. He was admitted with puffy face, abdominal distension, retarded growth, mental retardation, generalized muscular hypertrophy and hypertonia. Thyroid function test revealed hypofunction and thyroid gland was not visualized by 99raTc scintiscan. The stigmas of hypothyroidism and the muscular hypertrophy disappeared promptly after synthyroid replacement. Muscular biopsy...
A Case of Klippel-Trenaunay-Parkes-Weber Syundrom and the Review of Literatures on 14 Cases Reported in Korea.
Kap Seoung Kim, Hyun Young Ahn, Young Yoon Choi
Clin Exp Pediatr. 1984;27(9):935-940.   Published online September 30, 1984
We experienced a case of Klippel-Trenaunay-Parkes-Weber Syndrome in a 6 year old boy who had large hemangioma on the left extremities and trunk, varicose veins, soft and bony hypertrophy, vascular variation in flushing aortogram and enlarged left kidney in abdominal C-T. We report this with the review of literatures on 14 cases of Klippel-Trenaunay-Parkes-Weber Syndrome which were reported in Korea.
Three Cases of Idiopathic Hypertrophic Subaortic Stenosis.
Sei Weon Yang, Dong Gyoon Kim, Jong Jin Seo, Jung Yun Choi, Yong Soo Yun, Chang Yee Hong
Clin Exp Pediatr. 1984;27(5):493-500.   Published online May 31, 1984
Idiopathic Hypertrophic Subaortic Stenosis is a primary disease of cardiac muscle that is probably congenital, is often genetically transmitted, and is characterized by asymmetric septal hypertrophy, systolic anterior motion of anterior mitral valve and disorganization of cardiac muscle cells. The authors experienced 3 cases of Idiopathic Hypertrophic Subaortic Stenosis which were confirmed by echocardiography, cardiac catheterization and angiocardiography. The literatures...
A Case of Klippel-Trenaunary-Weber Syndrome.
Do Seung Lee, Dae Yong Hwang, Jeong Sick Min, Jae Sun Park
Clin Exp Pediatr. 1983;26(5):516-520.   Published online May 31, 1983
We report a case of Klippel-Trenaunay-Weber Syndrome in a 12 years old boy who had large port-wine hemangioma, soft tissue & bony hypertrophy and varicose veins on the lower leg. On the skin lesion, there are several black papules & crops of skin colored eruptions which were found out microscopically to be intraepidermal bleeding focuses & harmatomatous change of squamous cells into sebaceous cells...
A Case Report of Klippel-Trenaunay-Weber Syndrome.
Baeg Woon Seung, Seung Hwan Kim, Son Sang Seo, Jong Woo Shin
Clin Exp Pediatr. 1982;25(6):638-642.   Published online June 30, 1982
The association of varicose vein, soft tissue and bony hypertrophy and cutaneous hemangi-oma of the port-wine variety confined to one extremity was first reported in 1900 by Klippel and Trenaunay, and then Weber. A case of Klippel-Trenaunay-Weber Syndrome with macrodactylia in a 2 months old male infant(dizygotic) is presented who was asmitted to B.N.U.H. with chief complaints of nevus fla-mmeus,...
The Kocher-Debre-Semelaigne Syndrome.
M Y Chung, S I Kwon, S J Lee, D H Kum
Clin Exp Pediatr. 1982;25(1):92-96.   Published online January 31, 1982
A 4-year old girl with the Kocher-Debre-Semelaigne syndrome, hypothyroidism with generalized muscular hypertrophy is described. Histologic examination of a calf muscle showed musclular hypertrophy with focal eosinophilic degeneration. The stigmas of hypothyroidism and the muscular hypertrophy disappeared promptly and concomitantly following therapy with desiccated thyroid. Similar cases reported in literature are briefly reviewed. The clinical and histologic changes of the...