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Original Article
Abnormal Electrogastrography and Gastric Emptying Time in Patients with Cyclic Vomiting Syndrome during Symptom Free Interval
Nam Seon Beck
Clin Exp Pediatr. 1997;40(2):209-216.   Published online February 15, 1997
Purpose : Cyclic vomiting syndrome is characterized by repeated, unpredictable, explosive and unexplained bouts of vomiting and recur after intervals of uncertain length. In the absence of either known etiology or pathogenesis, deciphering the mystery of cyclic vomiting syndrome presents us with a daunting clinical challenge. As we seek to understand this mysterious vomiting disorder of unknown etiology and pathogenic mechanism,...
Pulmonary Air Leak Syndrome Associated with Respiratory Diseasein Infancy and Childhood
Hae Kyung Lee
Clin Exp Pediatr. 1997;40(2):189-193.   Published online February 15, 1997
Purpose : Pneumothorax, pneumomediastinum and subcutaneous emphysema rarely complicate pediatric respiratory disease such as bronchial asthma, bronchiolitis, bronchopneumonia. But physicians unfamiliar with natural history of these complications may feel that surgical intervention is necessary. So this study was done to to recognize the pulmonary air leak syndrome associated with respiratory disease in infancy and childhood. Methods : I reviewed retrospectively the medical...
The Pelvic Changes of Patients with Down Syndrome in Korea
Jong Park, Kwan Cheol Oh, Kyoung Sim Kim, Ki Bok Kim
Clin Exp Pediatr. 1997;40(2):159-166.   Published online February 15, 1997
Purpose : Since Caffey et al. first described the anomaly in pelvis among the Down patients, decreased iliac and pelvic indexes have been helpful in diagnosing the syndrome. For Korean cases, however, no definitive data are available as yet, prompting us to evaluate the pelvic changes in Korean Down's patients. Methods : Subjected to this study were 68 children with Down's syndrome. As...
Clinicopathological Study about Childhood Primary Nephrotic Syndrome Resistant to 4-week Daily Steroid Therapy
Jung Sue Kim, Il Soo Ha, Hae Il Cheong, Yong Choi
Clin Exp Pediatr. 1996;39(12):1729-1735.   Published online December 15, 1996
Purpose : Steroid-resistant nephrotic syndrome in children is difficult to manage and tends to progress to chronic renal failure. We studied clinicopathological correlations in primary nephrotic syndrome in children resistant to 4-week daily steroid therapy. Methods : Among children who had been admitted to Seoul National University Children's Hospital during the period between Oct. 1985 and Jul. 1995 and diagnosed as primary nephrotic syndrome, 87...
Case Report
A Case of Tethered Cord Syndrome
Sook Yeong Jeon, Ki Hyoung Lee, Moon Sung Park, Ok Wha Kim
Clin Exp Pediatr. 1996;39(11):1636-1640.   Published online November 15, 1996
Tethered cord syndrome is one of the spinal dysraphism with low-lying conus medullaris frequently associated with spinal lipoma, diastematomyelia and thick filum. The clinical manifestation is variable from no overt symptoms for a long time to back pain, scoliosis, progressive neurological deficit of legs and incontinence. Therefore the diagnosis requires a strong clinical suspicion and aggressive investigation. We experienced a case of 1 day-old male...
A Case of Nezelof's Syndrome
Hyeon Tae Kim, Nok Hong Kim, Eun Yeong Seol, Ki Hyun Chun, Kung Ho Lee, Mun Ki Cho, Kung Ran Choi
Clin Exp Pediatr. 1996;39(11):1620-1626.   Published online November 15, 1996
Nezelof's syndrome(combined immunodeficiency with immunoglobulin) is a hereditary primary immunodeficiency characterized by recurrent chronic pulmonary infections, oral and cutaneous candidiasis, failure to thrive, chronic diarrhea, skin infection, urinary tract infection, gram-negative sepsis, severe progressive varicella infection, lymphopenia, diminished lymphoid tissue, abnormal structure of the thymus, and presence of normal or increased levels of one or more of the major immunoglobulin classes, but with impaired antibody...
Original Article
Changes in MaternalAge and the Incidence of Down Syndrome
Chang Weon Oh, Sung Su Lim, Ki Bok Kim, Won Jin Kee
Clin Exp Pediatr. 1996;39(11):1512-1519.   Published online November 15, 1996
Purpose : The incidence of Down syndrome, the most common chromosoaml anomaly, increases with the advanced maternal age. Recently, however, the incidence of Down symdrome was reported to have decreased with wide acceptance of prenatal diagnosis and planned parenthood, prompting us to re-evaluate the incidence of Down syndrome in relation to changes in maternal age. Methods : Subjected to study were 296 Down cases: 26...
Clinical Course of Atrioventricular Septal Defect(AVSD) in Down and non Down syndrome
Un Seok Nho, Myung Chul Hyun, Sang Bum Lee
Clin Exp Pediatr. 1996;39(9):1288-1294.   Published online September 15, 1996
Purpose : It is well known that 1/3 - 1/2 of Atrioventricular septal defect(AVSD) patients also have Down syndrome which may influence the clinical course of AVSD. To know the anatomic type of AVSD and the effect of Down syndrome on the clinical course of AVSD, we studied 37 cases( Down group: 14 cases, non Down group: 23 cases) who were diagnosed and...
Case Report
A Case of Intestinal Neuronal Dysplasia
Se Young Kim, Yon Ho Choe, Jeong Kee Seo, In One Kim, Chong Jai Kim
Clin Exp Pediatr. 1996;39(8):1151-1157.   Published online August 15, 1996
Intestinal neuronal dysplasia(IND) is a disease characterized clinically by symptoms of intestinal obstruction and pathologically by hyperplasia of the submucosal and myenteric plexuses with formation of giant ganglia. Chronic intestinal pseudo-obstruction is a clinical diagnosis, composed of myopathic form and neuropathic form, and normal intestinal histology. Intestinal neuronal dysplasia is a neuropathic form of chronic intestinal pseudo-obstruction. The clinical presentation and the course of IND...
A Case of Mauriac's syndrome
Se Young Kim, Choong Ho Shin, Il Soo Ha, Hae Il Cheong, Sei Won Yang, Yong Choi, Hyung Ro Moon
Clin Exp Pediatr. 1996;39(7):1020-1024.   Published online July 15, 1996
Mauriac's syndrome was described in the 1920s, as a triad of poorly controlled insulin dependent diabetes mellitus, profound growth retardation, and hepatomgaly. Following the wide availability of insulin and intensification of diabetic control, this entity has become quite rare. A 9-year-old female child was transferred to pediatric OPD of SNUCH because of hyperglycemia, short stature, and visual disturbance. Five years prior to admission, she...
Original Article
Molecular Genetic Screening for the SRY(Sex Determining Region of the Y chromosme) Gene in Turner Syndrome Patients
Jung-Yeon Shim, Han-Wook Yoo
Clin Exp Pediatr. 1996;39(7):915-923.   Published online July 15, 1996
Purpose : Turner syndrome is among the most common cytogenetic abnormalities associated with X chromosome. In only 40-60% of Turner syndrome, 45,X monosomy is found and the high prenatal mortality of non-mosaic 45,X has led to the hypothesis that liveborn 45,X individuals may be cryptic mosaics. The presence of Y chromosome is significant because it predisposes affected individuals with Turner syndrome to gonadoblastoma formation....
The Changes of the Bone Mineral Density in the Girls with Turner Syndrome, using Recombinant Human Growth Hormone
Sei Won Yang
Clin Exp Pediatr. 1996;39(6):839-845.   Published online June 15, 1996
Purpose : Estrogen deficiency causes sexual infantilism in Turner syndrome, which could decrease the bone mineral density(BMD) since birth. This decreased BMD might be contributed by the decreased growth hormone(GH) secretion. To improve the decreased BMD, estrogen therapy is recommended, especially after the pubertal age, but estrogen therpay during childhood can accelerate the epiphyseal fusion, resulting in shorter final height. There is...
Effect of Steroid Therapy on Bone Mineral Density in Children with Minimal Change Nephrotic Syndrome
Byeong Hee Son, Woo Yeong Chung, Chul Ho Kim
Clin Exp Pediatr. 1996;39(6):822-828.   Published online June 15, 1996
Purpose : Osteoporosis and growth failure have been known one of the serious side effects of corticosteroid therapy especially in children. This study was designed to evaluate the effect of long-term administration of steroids on bone mineral density(BMD) in children with nephrotic syndrome and its relationship to cumulative steroid dose, the duration of the rapy and sex. Methods : The BMD of...
Case Report
Antiphospholipid Syndrome Presented with Pulmonary Embolism and Deep Venous Thrombosis in Child
Ran Lee, Il Soo Ha, Hae Il Cheong, Yong Choi
Clin Exp Pediatr. 1996;39(5):722-726.   Published online May 15, 1996
Antiphospholipid syndrome is a thrombotic disorder and the serologic marker of the syndrome is antiphospholipid antibody(lupus anticoagulant, anticardiolpin antibody, or both). In a 13-year-old girl who presented with dyspnea, pulmonary embolism and femoral vein thrombosis were demonstrated by lung scan and abdominal ultrasonography. She had 3 out of 11 criteria for the diagnosis of SLE, such as thrombocytopenia, positive antinuclear antibody, low C3...
Isolated Splenic Infarction in a Girl with Systemic Lupus Erythematosus
Kyung Ran Park, Hee Eun Lee, Yun Ae Jeon, Il Soo Ha, Hae Il Cheong, Joong Gon Kim, Yong Choi
Clin Exp Pediatr. 1996;39(4):562-566.   Published online April 15, 1996
Splenic infarction is a rare disease and usually detected as a complication of SBE, atrial fibrillation, hemoglobinopathy, or myeloproliperative disorders. Although thrombotic event, such as deep vein, cerebral or renal thrombosis, is a frequent complication of SLE, there have been only a couple of cases documenting splenic infarction complicated in SLE. This is, to our knowledge, the first case report...
Original Article
Surfactant Protein SP-A, B, and C: Purification and Biochemical Characterization
Chong-Woo Bae, Yong-Mook Choi, Joo Hyun Kang, Kil Lyong Kim, Kyung Soo Hahn
Clin Exp Pediatr. 1996;39(2):218-229.   Published online February 15, 1996
Purpose : Several kinds of exogenous pulmonary surfactants (SF), either synthetic or animalderived, are being used for the replacement therapy in respiratory distress syndrome (RDS) of newborn, especially in premature infants, and improved the neonatal mortality and morbidity. Because synthetic preparations are lack of surfactant protein (SP) and animal-derived preparations cause immunogenecity of heterogenous SP, there have been great necessity for the development of next...
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
Report of Nationwide Epidemiology of Aseptic Meningitis Outbreak in 1993 in Korea
Sung Hee Oh, Moo-Song Lee, Jin Han Kang, Chang Hwi Kim, Chong Young Park, Young Mo Sohn, Hoan Jong Lee, Chung Sik Chun, Sang Mann Shin
Clin Exp Pediatr. 1996;39(1):42-52.   Published online January 15, 1996
Purpose : There has been no nationwide report pertaining to the epidemiology of aseptic meningitis, although a great numer of patients have been diagnosed of the illness. Therefore, we report an explosive outbreak of aseptic meningitis occured in a nationwide scale in 1993. Methods : Aseptic meningitis epidemiology surveillence was performed retrospectively on the patients diagnosed of aseptic meinigitis from January...
Case Report
Toxic Shock Syndrome in a 13 Year Old Boy
Ji Hye Kim, Sung Hee Oh
Clin Exp Pediatr. 1995;38(12):1706-1712.   Published online December 15, 1995
Toxic Shock Syndrome(TSS), known to be mediated by toxins produced by Staphy¡ⓒlococcus aureus, is a potentially fatal multisystemic illness unless treated properly. Although the reported cases of TSS were primarily among menstruating women, more cases of TSS among children, nonmenstruating women and male adults have been reported recently. In Korea, however, TSS has not drawn much attention yet, and no...
Original Article
Cushing Syndrome in Children and Adolescents
Choong Ho Shin, Sei Won Yang, Hyung Ro Moon
Clin Exp Pediatr. 1995;38(12):1677-1685.   Published online December 15, 1995
Purpose : Cushing syndrome occurs rarely in children and adolescents. The clinical manifestation is variable and growth is usually adversely affected. Methods : We retrospectively analyzed 8 patients with Cushing syndrome between the ages of 1 and 14 year who were admitted to the pediatric department of Seoul National University Children's Hospital during the period from 1978 to 1995. Results : 1)...
Case Report
Nutcracker Syndrome: Report of A Case
Ki Joong Kim, Bu Heon Lee, In Cheol Park, Kwang Woo Ko, In One Kim
Clin Exp Pediatr. 1995;38(11):1588-1592.   Published online November 15, 1995
The Nutcracker syndrome refers to compression of the left renal vein between the aorta and the superior mesenteric artery, associated with hematuria, abdominal pain, and varicocele formation. We report a 13-year-old boy with intermittent gross hematuria for 14 months before admission. Ultrasonography shows narrowing of proximal left renal- vein and dilatation of distal left renal vein at the point of superior...
Original Article
Clinical Characteristics of Patients with Tumer' Syndrome according to Karyotypic Differences
Eun Young Kim, Kyoung Sim Kim, Kibok Kim, Won Jin Kee
Clin Exp Pediatr. 1995;38(11):1460-1469.   Published online November 15, 1995
Purpose : To assess the differences of clinical features according to various karyotypes in Turner syndrome. Methods : Subjected to study were 45 patients with Turner syndrome, including 5 newborns, from March 1974 to April 1994. They were divided into 3 groups according to karyotypes: 45,X, mosaicism, and structural aberration; and the clinical features were compared. Also structural aberration groups, 46,XXp-and...
Case Report
A Case of Meckel-Gruber Syndrome
Hye Jin Lee, Eun Ae Park, Gyoung Hee Kim
Clin Exp Pediatr. 1995;38(9):1299-1303.   Published online September 15, 1995
We report a case of male neonate that showed multiple congenital anomalies that could be designated as Meckel-Gruber syndrome. The principle signs of Meckel-Gruber syndrome are encephaolcelem, polydactly, and polycystic kidneys with normal karyotype. Due to rarity of the Meckel-Gruber syndrome, the cause was still unknown. At present, autosomal recessive is thought to be as one of the causes. This malformation...
A Case of Spontaneously Remitted Congenital Minimal Change Nephrotic Syndrome
Tae Sun Ha, Kyung Hee Lee, Beom Soo Park, Heon Seok Han
Clin Exp Pediatr. 1995;38(9):1288-1292.   Published online September 15, 1995
Nephrotic syndrome is a condition with severe proteinuria, hypoalbuminemia, and edema. When the syndrome develops within the first 3 months of life, it is generally considered as congenital and the overall outcome in this group seems to be worse than in miniaml change lesion that occurs at an older age regardless of the pathologic findings. A female infant with congenital nephrotic...
Three Cases of Rotor Syndrome in Monozygotic Twin Brothers and Their Sister
Jin Hwa Jung, Jeong Ho Lee, Yong Sub Kim, Jong Dae Jo
Clin Exp Pediatr. 1995;38(9):1270-1275.   Published online September 15, 1995
We recently experienced three cases of Rotor syndrome in monozygotic twin brothers and their elder sister. The only clinical manifestation was persisting mild jaundice for several years. In all three cases serum bilirubin, mainly of direct reacting was increased with normal liver function and normal hepatic histology without pigmentation by needle liver biopsy. Indocyanine green test showed marked serum retention after...
A Case Report of Acrorenal Syndrome
Soo Hee Chang, Jo Seph Choi, Soo Chul Cho, Dae Yeol Lee
Clin Exp Pediatr. 1995;38(8):1151-1154.   Published online August 15, 1995
Acrorenal syndrome is congenital anomaly of the limbs and urinary tract of unknown etiology. Acral malformations consist of varying combinations of oligodactyly, ectrodactyly, syndactyly, brachydactyly, polydactyly, and carpal, tarsal, or metatarsal fusions of the hands and feet. Urinary tract abnormalities include unilateral renal agenesis and duplication of the collecting system. The condition is a polytopic developmental field defect(acrorenal field) and...
Original Article
Antibody Response to Capsular Polysaccharide Vaccine of Streptococcal Pneumonia in Patients with Nephrotic Syndrom
Ji Young Choi, Jae Ho Lee
Clin Exp Pediatr. 1995;38(8):1107-1115.   Published online August 15, 1995
Purpose : Although the etiology and pathogenesis of child with nephrotic syndrome remains uncertain, it has been suggested from indirect evidence that this syndrome may represent deficiency in T-cell function and abnormal humoral immune response to antigens or immynoglobulin. Children with nephrotic syndrome are susceptible to pneumococcal infection. Now, we performed this study to evaluate pneumococcal anticapsular antibody concentration and...
Case Report
A Case of Chéiak-Higashi Syndrome
Soo Jin Kim, Soo Kyung Choi, Kyung Hee Park, Ghee Young Jung, Young Ok Lee
Clin Exp Pediatr. 1995;38(7):983-987.   Published online July 15, 1995
Chéiak-Higashi syndrome is a rare autosomal recessive disease characterised by partial oculocutaneous albinism, frequent pyogenic infections and abnormal large lysosomal granules in leukocytes and other granule containing cells. We experienced a case of Chéiak-Higashi syndrome in a twenty days old male who show-ed partial albinism, recurrent infections and characteristic abnormal large granules in granuloc-ytes on peripheral blood smear and bone marrow...
A Case of Fetal Hydantoin Syndrome Associated with Cardiac Anomaly
Hae Kyung Lee, Joon Soo Park, Kyeong Bae Park, Young Chang Kim
Clin Exp Pediatr. 1995;38(6):853-856.   Published online June 15, 1995
The fetal hydantoin syndrome is broad range of morphologic and developmental disorder in infant born of epileptic mothers exposed to hydantoin during pregnancy. We experienced a case of fetal hydantoin syndrome in a male neonate who had cleft palate, cleft lip, hypertelorism, TOF, finger hypolasia with prenatal history of exposure to diphenylhydantoin. We presented this case with brief review of literatures.
Original Article
Diagnostic Value of Stable Microbubble Rating test and Efficacy of Surfactant Replacement Therapy in Neonates with Respiratory Distres syndroems
Jeong Hee Kim, Eun Ae Park, Kyung Hee Kim
Clin Exp Pediatr. 1995;38(6):760-770.   Published online June 15, 1995
Purpose : Stable microbubble rating test(SMR) plays an important role in the clinical course and the prognosis of respiratory distress syndrome(RDS) patients, because SMR can be easily performed and it can predict quickly the neonatal RDS. After the success of the pulmonary surfactant replacement therapy in RDS infants by Fujiwara and co-workers in 1980, many supporting studies were presented and...
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