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Case Report
A Case of Congenital Megacystis Due to Non-Obstructive Urinary Retention Associated with Intestinal Malrotation and Hypoperistalsis
Tai Young Ham, Jeong Hoon Kim, Ji Hong Kim, Jae Seung Lee, Seok Ju Han, Choon Sik Yoon, Soon Won Hong
Clin Exp Pediatr. 2004;47(1):95-99.   Published online January 15, 2004
Congenital megacystis with bilateral hydroureteronephrosis is most commonly associated with posterior urethral valves, prune-belly syndrome, vesicoureteral reflux, or nonrefluxing, nonobstructive megaureters. Among the other cause of congenital megacystis, megacystis-microcolon-intestinal hypoperistalsis syndrome(MMIHS), which is characterized by megacystis, microcolon and hypoperistalsis of the intestines with resultant abdominal distension, is a rare cause of functional obstruction of urinary tracts in childhood. It was...
Original Article
A Study on the Effects of the Early Use of Nasal CPAP in the Weaning of Mechanical Ventilators
Yeoung Ju Kim, Byun Kyung Jung, Sang Geel Lee
Clin Exp Pediatr. 2003;46(12):1200-1206.   Published online December 15, 2003
Purpose : This study was conducted for the use of nasal continuous positive airway pressure (CPAP), by comparing the early use of non-invasive nasal CPAP with low intermittent mandatory ventilation(low IMV) and endotracheal CPAP in weaning a mechanical ventilator from infants with moderate respiratory distress syndrome(RDS). Methods : Thirty infants in the study group, with moderate RDS from November 2001 to...
Case Report
A Case of Keratitis, Ichthyosis and Deafness(KID) Syndrome
Lan Kim, Dong Hwan Lee
Clin Exp Pediatr. 2003;46(11):1135-1138.   Published online November 15, 2003
KID syndrome was named after the initials of the major three symptoms of the disease; keratitis, ichthyosis, and deafness. The syndrome was first introduced by Dr. Burns in 1915 who described one of his patients with those symptoms. In 1981, Dr. Skinner and his colleagues reported 17 patients who had keratitis, ichthyosis, and deafness. They also called the disease KID...
Original Article
Comparison of Clinical Courses According to the Existence of Patent Ductus Arteriosus in Respiratory Distress Syndrome
Ju Hee Seong, Hyeon Joo Lee, Hyun Kee Hong, Chong-Woo Bae, Yong-Mook Choi
Clin Exp Pediatr. 2003;46(11):1080-1084.   Published online November 15, 2003
Purpose : To compare perinatal characteristics, clinical courses, and overall morbidity between respiratory distress syndrome(RDS) with patent ductus arteriosus(PDA) and RDS without PDA in neonates. Methods : Eighty-three neonates who were diagnosed and treated for RDS in the neonatal intensive care unit(NICU) from Jan. 2000 to Dec. 2002 were included in this study. RDS was complicated with PDA(group A) in...
Case Report
A Case of Sandifer Syndrome
Mira Lee, Soo Hyun Kim, Yong Joo Kim, In Joon Seol
Clin Exp Pediatr. 2003;46(10):1036-1039.   Published online October 15, 2003
Sandifer syndrome is a rare manifestation of gastroesophageal reflux in children, occurring in association with abnormal movement of the head and neck and recurrent episodes of seizure after feeding. This syndrome may be misdiagnosed as infantile seizure and musculoskeletal disorder and may be associated with failure to thrive, anemia, aspiration pneumonia, and esophagitis. In this paper, Sandifer syndrome in a...
Original Article
Heart Rate Variability and Autonomic Activity in Patients Affected with Rett Syndrome
Deok Young Choi, Jin Ha Chang, Hee Jung Chung
Clin Exp Pediatr. 2003;46(10):996-1002.   Published online October 15, 2003
Purpose : In Rett syndrome patients, the incidence of sudden death is greater than that of the general population, and cardiac electrical instability including fatal cardiac arrhythmia is a main suspected cause. In this study, we are going to find out the possible cause of the higher risk of sudden death in Rett patients by the evaluation of heart rate...
Case Report
A Case of Klippel-Trenaunay Syndrome Combinded with Sturge-Weber Syndrome
Mi Young Kim, So Young Lee, Na Yeon Kim, Sun Ju Lee, Won Duck Kim, Sung Min Cho, Dong Seok Lee, Doo Kwun Kim, Sung Min Choi
Clin Exp Pediatr. 2003;46(9):909-912.   Published online September 15, 2003
Klippel-Trenaunay syndrome is a rare mesodermal phakomatosis characterized by cutaneous haemangiomata(usually unilateral and involving an extremity), venous varicosities and osseous and soft tissue hypertrophy, of the affected limb. Sturge-Weber syndrome, also a mesodermal pharkomatosis, is characterized by a port-wine nevus, which is present from birth and covers the face and cranium in the territory of the first division of the...
Original Article
Risk Factors for the First-Year Relapse in Children with Nephrotic Syndrome
Hye Kyoung Shin, Ji Hee Kim, Kee Hwan Yoo, Young Sook Hong, Joo Won Lee, Soon Kyum Kim
Clin Exp Pediatr. 2003;46(9):889-892.   Published online September 15, 2003
Purpose : This study aimed to evaluate risk factors of the first year relapse in children with nephrotic syndrome(NS) without the need for biopsy. Methods : We reviewed, retrospectively, 78 children diagnosed with steroid responsive nephrotic syndrome between July 1997 and June 2002. Median years to follow up were 4.4 years(range : 1-5 years). We divided the patients into two groups(group...
Correlation between Chest Radiographic Findings and Respiratory Indices in Neonates with Respiratory Distress Syndrome
Yong-Hwan Chung, Youn-Jin Park, Chong-Woo Bae, Dong-Wook Sung
Clin Exp Pediatr. 2003;46(7):655-660.   Published online July 15, 2003
Purpose : The relationship between chest X-ray findings and respiratory indices, including the arterial-alveolar oxygen partial pressure ratio(a/APO2) and the ventilatory index(VI), indicators of the clinical respiratory status in neonates with respiratory distress syndrome(RDS), was examined in the present study. Methods : The records of 50 neonates, randomly chosen from 174 neonates treated with pulmonary surfactant(PS) in the Neonatal Intensive Care...
Case Report
A Case of Goltz Syndrome
Dong Hoon Lee, Chul Han Park, Ji Min Park, Set Byul Park, Heung Sik Kim, Young Wook Ryoo, Kyu Suk Lee, Hee Jung Lee
Clin Exp Pediatr. 2003;46(6):606-609.   Published online June 15, 2003
Goltz syndrome(focal dermal hypoplasia) is a rare disorder characterized by ectodermal and mesodermal dysplasia described in 1962 by Goltz. In Korea, one case of Goltz syndrome was reported in 1994. The inheritance mode is mostly X-linked dominant. Skin abnormality is the most common manifestation including hypoplasia of the dermis. Skeletal involvement such as syndactyly, polydactyly, scoliosis, kyphosis and spina bifida...
A Case of Lesch-Nyhan Syndrome
Joon-Sung Kim, Jae-Seung Lee, Ha-Young Noh, Byung-Ju Kim, Young-Jong Woo, Jee-Min Park, Myung-Gwan Kim, Gu-Hwan Kim, Han-Wook Yoo
Clin Exp Pediatr. 2003;46(5):505-509.   Published online May 15, 2003
Lesch-Nyhan syndrome is an X-linked recessive disorder characterized by hyperuricemia, choreoathetosis, spasticity, mental retardation, and compulsive, self-injurious behavior. This disorder results from a complete deficiency of the purine salvage enzyme, hypoxanthine-guanine phosphoribosyl transferase(HPRT). We report here on a case of Lesch-Nyhan syndrome in a 1-year, 7-month-old male who presented with frequent vomiting, failure to thrive, and developmental delay. The diagnostic...
Five Cases of Shaken Baby Syndrome
Ok Yeon Cho, Kwon Hoe Huh, Do Jun Cho, Dug Ha Kim, Ki Sik Min, Ki Yang Yoo, Yul Lee
Clin Exp Pediatr. 2003;46(4):404-408.   Published online April 15, 2003
Shaken baby syndrome is a serious form of child abuse, mostly involving children younger than 2 years. It results from extreme rotational cranial acceleration induced by violent shaking. The characteristic injuries include subdural hemorrhage, retinal hemorrhage, and fracture of ribs or long bones. If physicians have no recognition of, or suspicion about, shaken baby syndrome, this syndome is difficult to...
A Case of Netherton's Syndrome in a Newborn
Eun-Hee Lee, Ellen Ai-Rhan Kim, Ki-Soo Kim, Beom-Jin Cho, Jai-Kyoung Koh, Soo-Young Pi
Clin Exp Pediatr. 2003;46(4):389-392.   Published online April 15, 2003
Netherton's syndrome is an unusual disorder which consists of triad of ichtyosiform dermatosis, multiple defects of hair shaft and an atopic diathesis. The finding of bamboo hair is pathognomic in Netherton's syndrome and the ichthyosiform dermatosis may consist of either ichtyosis linearis circumflexa or congenital ichthyosiform erythroderma. Often, variability in the clinical features leads to a delay in diagnosis in...
A Case of Townes-Brocks Syndrome
Hong Seop So, Hyun Ju Choi, Hye Sun Yoon, Jin Soon Hwang, Keun Chan Sohn
Clin Exp Pediatr. 2003;46(4):382-384.   Published online April 15, 2003
Townes-Brocks syndrome is an uncommon autosomal dominant condition first described by Townes and Brocks in 1972. We experienced a newborn female who presented with clinical findings of Townes-Brocks syndrome in an otherwise unaffected family. The patient showed the full spectrum of anomalies including anterior placed anus, triphalangeal thumb, preaxial polydactyly, pre-auricular tags, microtia without opening, sensorineural hearing loss and unilateral...
Familial Sick Sinus Syndrome
In-Hye Nam, Sung-Hee Cheon, Hong-Ryang Kil
Clin Exp Pediatr. 2003;46(2):195-197.   Published online February 15, 2003
Sick sinus syndrome(SSS) constitutes a spectrum of cardiac arrhythmia, including sinus bradycardia, sinus pause-arrest, sinoatrial block, slow escape rhythm, bradyarrhythmia and tachyarrhythmia. SSS is relatively uncommon in children but its exact incidence is unknown because diagnostic criteria are not uniform and most children with SSS, in general are asymptomatic. SSS may be primary(organic sinus node disease) or secondary(cardiac surgery comprises...
Original Article
Characterization of B Cells of Lymph Nodes and Peripheral Blood in a Patient with Hyper IgM Syndrome
Dong Soo Kim, Kyuong Mi Shin, Woo Ick Yang, Jeon-Soo Shin, Chang Hwa Song, Eun Kyeong Jo
Clin Exp Pediatr. 2003;46(2):128-136.   Published online February 15, 2003
Purpose : Hyper IgM syndrome(HIGM) is characterized by severe recurrent bacterial infections with decreased serum levels of IgG, IgA, and IgE but elevated IgM levels. Recently, it has been classified into three groups; HIGM1, HIGM2 and a rare form of HIGM. HIGM1 is a X-linked form of HIGM and has now been identified as a T-cell deficiency in which mutations...
Case Report
A Case of Raine Syndrome
Hye Jin Park, Jeong Jin Lee, Jeong Sik Seo, Hyo Jin Kim, Je Yong Choi, Jun Hwa Lee, Un Seok Nho, Hai Lee Chung, Woo Taek Kim
Clin Exp Pediatr. 2003;46(1):91-94.   Published online January 15, 2003
Raine syndrome was described as an unknown syndrome in 1989. It is characterized by severe craniofacial anomalies with microcephaly, hypoplastic nose, depressed nasal bridge, exophthamos/protosis, gum hypertrophy, cleft palate, low-set ears, small mandible, narrow chest, wide cranial sutures and choanal atresia or stenosis, by generalized osteosclerosis with subperiosteal thickening of ribs, clavicles and diaphysis of long bones, and by intracranial...
A Case of Moyamoya Disease in a Child with Alagille Syndrome
Mi Rang Lim, So Yaun Lee, Deok Soo Kim, Kyung Mo Kim, Tae Sung Ko
Clin Exp Pediatr. 2003;46(1):86-90.   Published online January 15, 2003
Alagille syndrome is a autosomal dominant disorder characterized by intrahepatic bile duct paucity and resultant chronic cholestasis in combination with cardiac(mainly peripheral pulmonary stenosis), skeletal, ocular, and facial abnormalities. In addition to the pulmonary stenosis, in large series, anecdotal reports of vascular lesions have concerned the renal artery, aorta, hepatic artery, carotid artery, celiac artery or subclavian artery. Theses diffuse...
Original Article
A Clinical Study of the Acute Respiratory Distress Syndrome in Children
Taek Jin, Dong Soo Kim, Dong Hwan Shin
Clin Exp Pediatr. 2003;46(1):42-50.   Published online January 15, 2003
Purpose : The acute respiratory distress syndrome(ARDS), an acute form of severe alveolar-capillary injury evolving after a direct or indirect lung insult is thought to be a common cause of respiratory failure though not many clinical studies on the subject have been made yet. Methods : Between January 1992 and December 2001, we conducted a retrospective study on 33 children who...
Case Report
A Case of Pseudo-Bartter's Syndrome Due to Hypertrophic Pyloric Stenosis
Yoon Hee Kim, Yoon Hee Kim, Tae Ho Lee, Tae Ho Lee, Hong Bae Kim, Hong Bae Kim
Clin Exp Pediatr. 2002;45(11):1430-1434.   Published online November 15, 2002
We report a 3-month old boy admitted to our hospital with Bartter's syndrome like symptoms and laboratory findings, which were vomiting, failure to thrive, hypochloremic and hypokalemic metabolic alkalosis associated with hyperreninemia, hyperaldosteronism and normal blood pressure. However, the urine chloride level was low. Hypertrophic pyloric stenosis was diagnosed through abdominal ultrasonography. Fredet-Ramstedt operation was done after electrolyte correction. After...
A Case of Melkersson-Rosenthal Syndrome
Dae Hun Pee, Yo Won Na, Ki Young Chang, Woo Keun Seo, Kee Hyoung Lee, Baik-Lin Eun
Clin Exp Pediatr. 2002;45(10):1292-1297.   Published online October 15, 2002
Melkersson-Rosenthal syndrome(MRS) is a rare disorder, having a symptom triad of recurrent facial palsy, orofacial swelling and fissured tongue(lingua plicata). This disorder is usually recurrent or progressive, and monosymptomatic or oligosymptomatic forms have been reported to be more common than classic forms. Generally, MRS occurs in young adults at the end of the second decade of life and incidence of the disease in childhood...
A Case of Systemic Capillary Leak Syndrome in a Child
Taeg Young Lee, Yoo Mi Chung, Hee Gyung Kang, Il Soo Ha, Hae Il Cheong, Yong Choi
Clin Exp Pediatr. 2002;45(10):1292-1297.   Published online October 15, 2002
Systemic capillary leak syndrome(SCLS) is a rare disorder of unknown etiology, which is characterized by recurrent attacks of hypotension, hemoconcentration, and hypoalbuminemia. Urinary or enteric loss of protein is not demonstrated. It is often associated with monoclonal gammopathy, but does not manifest multiple myeloma. Since Clarkson et al. described the first case in a 34- year-old woman, about 50 cases have been reported in...
A Case of Fetal Valproate Syndrome Associated with Both Elbow Joint Contractures
Yun Chang Choi, Eun Young Kim, Kynug Rye Moon, Young Il Rho
Clin Exp Pediatr. 2002;45(10):1288-1292.   Published online October 15, 2002
Fetal valproate syndrome has been described as a distinctive pattern of minor anomalies of the face and digits. This pattern has not been delineated completely but appears to involve brachycephaly with a high forehead, shallow orbit, ocular hypertelorism, small nose, small mouth, low set posteriorly rotated ears, long overlapping fingers and toes, and hyperconvex fingernail. Cleft palate and congenital heart disease have occasionally been...
Original Article
Mutational Analysis of MECP2 Gene in 34 Rett Syndrome
Sang Jo Park, Tae Gyu Hwang, Byeong Hee Son, Chul Min Kim
Clin Exp Pediatr. 2002;45(10):1263-1272.   Published online October 15, 2002
Purpose : Rett syndrome(RTT) is an X-linked dominant neurodevelopmental disorder affecting 1 per 10,000-15,000 female births worldwide. It was initially described by Andreas Rett in 1966. RTT involves developmental regression characterized stereotypic hand movements, tremors, gait apraxia, seizures, deceleration of head growth after the age of 6-18 months. The disease-causing gene was identified as MECP2 on chromosome Xq28. We carried out mutational analysis of MECP2...
Effect of Nasal Continuous Positive Airway Pressure after Early Surfactant Therapy in Moderate Respiratory Distress Syndrome
Eun Ji Kim, Hae Sook Kim, Man Hoe Hur, Sang Geel Lee
Clin Exp Pediatr. 2002;45(10):1204-1212.   Published online October 15, 2002
Purpose : Early surfactant therapy with either gentle ventilation, high-frequency ventilation or aggressive weaning of mechanical ventilation are principles for the treatment of respiratory distress syndrome(RDS). We studied to determine the accessibility of noninvasive nasal continuous positive airway pressure(CPAP) rather than mechanical ventilation by invasive intubation after early surfactant therapy. Methods : The study group consisted of 14 infants who were born and diagnosed with...
Case Report
A Case of Satoyoshi Syndrome Presented with Progressive Muscular Spasm and Alopecia
Kyung Ran Son, Jin Hwa Kook, Byung Ju Kim, Sung Jin Kim, Jae Sook Ma
Clin Exp Pediatr. 2002;45(9):1165-1169.   Published online September 15, 2002
Satoyoshi syndrome(generalized Komuragaeri disease) is a rare disorder of unknown cause, characterized by progressive, painful, intermittent muscle spasms and alopecia. Endocrinopathy with amenorrhea, secondary skeletal abnormalities, and diarrhea or unusual malabsorption are frequently seen. It seems that autoimmunity may play a role in its pathogenesis. We report a 13-year-old girl with characteristic manifestations of the syndrome. She was treated with...
A Case of Alport Syndrome Associated with Diffuse Esophageal Leiomyomatosis
Jin Young Jung, Cheol-Min Kim, Yean Jung Lim, Ja Hyung Kim, Chong Woo You, Bo-Hwa Choi, Soo-Joung Hong, Young Seo Park
Clin Exp Pediatr. 2002;45(9):1160-1164.   Published online September 15, 2002
University of Ulsan, Seoul, Korea We report a case of Alport syndrome associated with esophageal leiomyomatosis, presenting as recurrent pneumonia. A 5-year old girl who had a history of cataract visited the out patient clinic with a complaint of recurrent wheezing and respiratory difficulty which had started five months previously. Chest magnetic resonance image(MRI) and esophagography, checked on the suspicion of...
Two Cases of Wolff-Parkinson-White Syndrome in a Family
Chan Uhng Joo, So Hee Lim, Pyung Han Hwang
Clin Exp Pediatr. 2002;45(9):1150-1154.   Published online September 15, 2002
Wolff-Parkinson-White(WPW) syndrome is characterized by electrographic evidence of ventricular preexcitation, which predisposes to supraventicular arrhythmias. Familial occurrence of WPW syndrome is uncommon. We observed two affected siblings in a family. Five members of the family underwent 12-lead electrocardiography and echocardiography. Although known genetic abnormality of the 7q34-q36(PRKAG2) for the familial WPW syndrome was evaluated, the mutation was not detected in...
A Case of Stevens-Johnson Syndrome Plus Vanishing Bile Duct Syndrome Associated with Ibuprofen Use
Jee Yee Choi, Su Young Kim, Soon Ok Byun, Jae Hong Park
Clin Exp Pediatr. 2002;45(9):1146-1149.   Published online September 15, 2002
Acute vanishing bile syndrome is a cause of progressive cholestasis. It is most often drug or toxin related. It's pathogenesis is unknown. Stevens-Johnson syndorme is a well-recognized immune complex-mediated hypersensitivity reaction. It is induced by drug or infection. It has classic systemic, mucosal, and dermatologic manifestations. We report a case of a 14 years old female suffering from Stevens-Johnson syndrome...
Original Article
Clinical Characteristics and Genetic Analysis of Prader-Willi Syndrome
Ji Eun Lee, Kwang Bin Moon, Jong Hee Hwang, Eun Kyung Kwon, Sun Hee Kim, Jong Won Kim, Dong Kyu Jin
Clin Exp Pediatr. 2002;45(9):1126-1133.   Published online September 15, 2002
Purpose : Prader-Willi syndrome(PWS) is a complex disorder affecting multisystems with characteristic clinical features. Its genetic basis is an expression defect in the paternally derived chromosome 15q11-q13. We analyzed the clinical features and genetic basis of PWS patients for early detection and treatment. Methods : We retrospectively studied 24 patients with PWS in Department of Pediatrics, Samsung Medical Center, from...
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