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Case Report
Pena-Shokeir I Syndrome in a Newbonrn Infant
Myung Gil Han, Kyu Young Kim, Dong Woo Son, Bo Young Yoon, Kyung Hee Park, Do Hyun Kim, Hyung Ro Moon
Clin Exp Pediatr. 1997;40(5):721-725.   Published online May 15, 1997
Pena-Shokeir I syndrome is a multiple malformation syndrome displaying characteristics of camptodactyly, multiple ankylosis, severe muscle weakness, facial anomalies (low set ears, hypertelorism, depressed tip of nose), polyhydramnios, fetal growth retardation & pulmonary hypoplasia which are inherited by autosomal recessive trait. We experienced 1 case of Pena-Shokeir I syndrome in a neonate (41 weeks, 2.08Kg). This patient suffered from dyspnea. Respiratory...
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...
Herditary Anhidrotic Ectodermal Dysplasiain Twins
Myung Gil Han, Do Hyun Kim, Hyung Ro Moon
Clin Exp Pediatr. 1996;39(7):1005-1009.   Published online July 15, 1996
Hereditary anhidrotic ectodermal Dysplasia is a congenital disease displaying characteristics of anhidrosis, hypotrichosis and dental defect which are caused by developmental anomaly of ectodermal epidermis and its appendages. We experienced two cases of hereditary anhidrotic ectodermal dysplasia in two-year and four-month old twin brothers. These patients suffered from intermittent high fever early in life which brought them to our clinical attention. However the diagnosis of...
Acanthosis Nigricans and TypeII Diabetes in Childhood
In Na Jeong, Hyung Ro Moon
Clin Exp Pediatr. 1996;39(3):404-410.   Published online March 15, 1996
The authors report two cases of type II diabetes with and without a acanthosis nigricans in children and a case of obese child with acanthosis nigricans encountered at Seoul Eul Ji Hospital in 1994 with review of literatures on relationship of obesity, acanthosis nigricans and type II diabetes. In case 1, 14-year old girl, BMI was 24 (weight, 63.5Kg ; height,...
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
Two Cases of Tetrahydrobiopterin Deficiency
Jee Won Choi, Sei Won Yang, Hyung Ro Moon, Se Chin Cho
Clin Exp Pediatr. 1995;38(3):397-403.   Published online March 15, 1995
Tetrahydrobiopterin(BH4) deficiency is a rare type of hyperphenylalaninemia and usually leads to a progressive neurologic deterioration despite early dietary control of blood phenylalanine concentration. We experienced two cases of BH4 deficiency in brother and sister, confirmed by biochemical study of blood and urine. They had suffered from a progressive neurologic illness such as mental retardation, severe hypotonia, seizure, and athetotic movements...
Original Article
Pheochromocytoma in Children
Tae Sue Ha, Jae Hong Park, Il Soo Ha, Hae Il Cheong, Yong Choi, Hyung Ro Moon, Gui Won Park
Clin Exp Pediatr. 1994;37(7):961-968.   Published online July 15, 1994
We Reviewed 10 hypertensive children with pheochromocytoma retrospectively and the following results were obtained. 1) Out of 10 patients, 7 were male and 3 female. Age ranged from 5.5 years to 13.8 years and their median age was 9.9 years. 2) They complained of sweating, lethargy, headache, or chest pain and so on, Hypertension were noticed in all patients. Heart murmurs were...
The Effect of Long-Acting Gonadotropin-Releasing Hormone (GnRH) Analog on the Growth Velocity and Final Adult Height in the Children with True Precocious Puberty
Sei Won Yang, Hyung Ro Moon
Clin Exp Pediatr. 1994;37(3):309-316.   Published online March 15, 1994
The final adult height in the children with true precocious puberty are destined to be short due to excessive bone maturation, compared to the growth velocity, regardless of its etiologies. To improve this final shortness, long-acting GnRH analog have been tried to the children with true precocious puberty. We evaluated the parameters of the growth, including the growth velocity, height...
The Correlation between the Bone Age and Predicted Adult height, Final Adult Height in the Children with Congenital Adrenal Hyperplasia, Especially 21-Hydroxylase Deficiency
Sei Won Yang, Hyung Ro Moon
Clin Exp Pediatr. 1994;37(3):300-308.   Published online March 15, 1994
We studied the correlation between the bone age and the predicted adult height, final adult height in the 69 children (30 salt losing form and 39 non-salt losing form) diagnosed as 21-hydroxylase deficiency, retrospectively. The results were as belows; 1) The bone age was similar to the chronological age in the children with salt-losing form (5.3±3.5 years, respectively), but the bone age...
Re-evaluation of TSH Screening TEST in Neonates
Jin Young Song, Dong Woo Son, Beyong Il Kim, Sei Won Yang, Jung-Hwan Choi, Chong Ku Yoon, Hyung Ro Moon
Clin Exp Pediatr. 1993;36(11):1502-1506.   Published online November 15, 1993
Five years ago, we made the cut-off value of TSH by dry filter paper method as 15μU/ml to screening congenital hypothyrodism. Since then, 1,210 term neonates, who had no perinatal problems, screening test with this cut-off point. Neonates had been recalled for measurement of serum T4/TSH to rule out congenital hypothyroidism if their TSH value by screening tests reveal more...
The Variability of Growth Hormone(GH) Response to Growth Hormone-Releasing Hormone(GHRH) according to the intrinsic Growth Hormone Secretory Rhythm in Children with Normal Growth Hormone Reserve
Sei Won Yang, Hyung Ro Moon
Clin Exp Pediatr. 1993;36(3):312-319.   Published online March 15, 1993
The diagnostic value of GHRH in assenssing GH secretion in biochemical GH suficient short children was examined. GHRH(1㎍/kg i.v bolus) was given to three groups (upslope, trough, downslope) arbitrarily classified according to the basal pulsatile GH secretory pattern before GHRH administration. Cmax following GHRH administration were variable and overlapping. Two children in downslope group, three children in though group, and one...
Growth Promoting Effect of Recombinant methionyl-Growth Hormone in Children with Chronic Renal Failure
Heon Seok Han, Yong Choi, Hyung Ro Moon
Clin Exp Pediatr. 1992;35(10):1355-1360.   Published online October 15, 1992
The authors studied the growth-promoting effect of yeast-derived methionyl-hGH in short statured children with uremic chronic renal failure. The subjects were prepubertal chronic renal insufficient patients whose height standard deviation scores were below -2.0. Two boys and two girls participated in this study. Chronic renal insufficiency was the only cause for the short stature. HGH was given to the subjects in dose...
Case Report
A Case of Hypomelanosis of Ito Accompanying Turner Syndrome
Kyung Un No, Dong Wook Kim, Dong Joo Shin, Hyung Ro Moon
Clin Exp Pediatr. 1992;35(8):1157-1163.   Published online August 15, 1992
Hypomelanosis of Ito has been known to show the characteristic hypopigmentation of the skin over the trunk and the extremities following Blaschko lines, and to accompany abnormalities of the central nervous system, the skeletal system, the eye and so on. Lately, a variety of chromosomal anomalies, especially mosaicism, have been reported in association with hypomelanosis of Ito, which is believed...
Original Article
Standard of Ear Size in Korean Children
Heon Seok Han, Hyung Ro Moon
Clin Exp Pediatr. 1992;35(8):1089-1095.   Published online August 15, 1992
Measurement of 1298 Korean Children in Seoul (male 661; female 637) resulted in normal standard data for ear length and width by their age and sex. 1) Ear length and width were as follows respectively : 36.0¡¾2.5mm 21.5¡¾2.5mm in male neonate, 56.3¡¾3.1mm 30.6¡¾3.2mm at 6yr boy, 63.2¡¾3.5mm 33.4¡¾3.2mm in 14 yr adolescent male, 35.5¡¾2.0mm 21.2¡¾2.1mm in female neonate, 54.6¡¾3.1mm 28.7¡¾2.4mm in...
Case Report
A Case of WDHA(Watery Diarrhea Hypokalemia Achiorhydria) Syndrome
Nam Seon Beck, Jun Jai Kim, Beom Soo Park, Jeong Kee Seo, Hyo Seop Ahn, Hyung Ro Moon, Je Geun Chi
Clin Exp Pediatr. 1992;35(4):575-580.   Published online April 15, 1992
VIP secreting tumors are rare in children but they produce a dramatic clinical picture, the most prominent feature of which is profuse, watery diarrhea and hypokalemia. A 5-year-old girl was brought to Seoul National University Children's Hospital for evaluation of profuse watery diarrhea. She presented with watery diarrhea, hypokalemia, and stunted growth, and had experienced these problems for four years...
Original Article
Clinical study on lipoid pneumonia caused by aspiration of squalene.
Dong Joo Shin, Beom Soo Park, Moon Sun Yang, Heon Seok Han, Young Yull Koh, Hyung Ro Moon, Chang Yeon Lee
Clin Exp Pediatr. 1991;34(5):654-661.   Published online May 31, 1991
A clinical observation was made on 14 cases of lipoid pneumonia, who visited department of pediatrics, Seoul National University Children’s Hospital from August, 1988 to June, 1990. The results were as follows; The ratio of male to female was 10 : 4. Age at diagnosis was 1.1±1. 3 years, and ranged from 3 months to 4.9 years. The age at taking Squalene ranged...
The etiologies and the clinical manifestations of hypoglycemia in infancy and childhood.
Nam Seon Beck, Dong Joo Shin, Heon Seok Han, Sei Won Yang, Hyung Ro Moon
Clin Exp Pediatr. 1991;34(4):480-486.   Published online April 30, 1991
To analysis age at onset, clinical manifestations, etiologies, complication and management of hypoglycemia, the authors reviewed seventeen cases of hypoglycemia who were admitted to Seoul National University Children’s Hospital because of hypoglycemic symtoms from March, 1982 to February, 1990. In neonate group, which included five patients, their initial symtoms were apnea, cyanosis, sweating and hand rigidity, with the causes of two hyperinsulinemic hypoglycemias, one...
A case of hypomelanosis of Ito accompanying ureteral duplication and hypomelanotic scalp hair.
Dong Woo Son, Beom Soo Park, Heon Seok Han, Hae Il Jung, Yong Choi, Hyung Ro Moon, Seon Hoon Kim, Hwang Choi
Clin Exp Pediatr. 1991;34(2):281-286.   Published online February 28, 1991
Hypomelanosis of Ito (incontinentia pigmenti achromians) is a cutaneous abnormality consisting of bizarre, whorly, linear, or patchy hypopigmentation over variable portion of body surface. Multiple assocaited defects in other systems occur in three quarters of the affected individuals. Most common- ly, the central nervous system, eye, and musculoskeletal structures are involved. It is suggested that the cutaneous abnormality, which is often detectable at birth...
Clinical Observation on isolated TRH deficient Congenital Hypothyroidism.
Heon Seok Han, Hyung Ro Moon
Clin Exp Pediatr. 1990;33(10):1388-1393.   Published online October 31, 1990
Clinical features and endocrine function of 3 children with isolated TRH deficient congenital hypothyroidism followed at Seoul National University Children’s Hospital from Aug, 1986 to Aug. 1990 were reviewed. During above period 262 congenital hypothyroid patients were followed at endocrine clinic, number of congenital primary hypothyroidism was 218 cases (83.2%), and that of congenital secondary hypothyroidism was 44 cases (16.8%). Of the congenital primary hypothyroidism,...
Seventeen Years Experience on Inborn Errors of Metabolism A Study for Future Development of Inborn Errors of Metabolism in Korea.
Hong Jin Lee, Hyung Ro Moon
Clin Exp Pediatr. 1990;33(8):1031-1036.   Published online August 31, 1990
In Korea, the study of inborn errors of metabolism is very primitive. Even the incidence and prevalence of the disease of inborn errors of metabolism are not known in Korea. We have done this study of inborn errors of metabolism in Korea and concluded that: 1) The varieties and incidences of inborn errors of metabolism in Korea might be relatively diverse and high. 2) Nation wide mandatory...
Four Cases of Attempted Suicide in Childhood.
Young Don Lee, Hyung Ro Moon
Clin Exp Pediatr. 1990;33(5):666-670.   Published online May 31, 1990
Suicides and attempted suicides in childhood are not as rare as in commonly believed by foreign literatures. However a few cases of suicide in childhood were reported in Korea until the present or up to the present. A suicide attempt is usually interpreted as a cry for help and an indication of an individuafs frustration, distress and feelings of inadequacy to cope in some...
Three cases of Chronic Meningitis.
Soon Mee Park, In Sang Jeon, Hyung Ro Moon
Clin Exp Pediatr. 1990;33(4):544-552.   Published online April 30, 1990
Chronic menigitis may be defined as symptoms and signs of meningeal inflammation lasting four weeks or longer. It has many infectious and noninfectious causes. Tuberculous and fungal infections are the most common causes. But no cause is found in the significant portions of chronic meningitis(idiopathic meningitis), although most appear to respond to treatment either with anti-tuberculosis drugs or steroids. Intereastingly eosinophilic pleocytosis in the cerebrospinal...
Pituitary Function on Patients with Sellar and Suprasellar Tumor.
Sei Won Yang, Hyung Ro Moon, Byung Kyu Cho
Clin Exp Pediatr. 1990;33(4):491-498.   Published online April 30, 1990
We observed the status of pituitary hormones (growth hormone, TSH, ACTH, gonadotropin and ADH) in the patients with intrasellar and suprasellar tumors (Table 1 and 2) before and after treatment of tumors. They were estimated from the results of various provocative tests to the pituitary hormones after confimation of normal thyroid function. The results were as belows (Table 3 and 4): 1) Most patients with craniopharyngioma...
Two cases of Goldenhar's Syndrome.
Ah Young Yun, Nam Sun Baik, Young Ah Lee, Hyung Ro Moon
Clin Exp Pediatr. 1990;33(3):380-385.   Published online March 31, 1990
Goldenhar’s syndrome is a syndrome consisting of (1) epibulbar dermoids and/or lipodermoids (2) preauricular skin tags and blind-ended fistulas and (3) vertebral anomalies. Two cases of Goldenhar’s syndrome which showed asymmetric facies, epibubar dermoids, preaur- icular skin tags blind-ended fistulas in 7 month-old girl and 2 month-old boy were reported. In one of our cases cleft of vertebrae, hypoplasia of left lung and cyanotic...
Clinical Study of Mycoplasma Pneumoniae Infection in Children.
Jong Woon Choi, Hoan Jong Lee, Byung Kiu Park, Hyung Ro Moon
Clin Exp Pediatr. 1990;33(3):315-323.   Published online March 31, 1990
A retrospective analysis of clinical findings in 43 patients with M. pneumoniae infection who visited Department of Pediatrics of Seoul National University Hospital from January, 1987 till December, 1987 was performed. 1) The peak incidence of age was 5 to 9 years of age (20 cases; 46.5%), and male-to-female ratio was 1.05:1. 2) Monthly distribution showed a high frequency in April (10 cases; 23.3%) and seasonal distribution showed...
A Clinical Study of Staphylococcus Aureus Infection in Children.
Yong Joon Shin, Young Pyo Chang, Dong Kyu Jin, Byung Kiu Park, Hoan Jong Lee, Hyung Ro Moon
Clin Exp Pediatr. 1990;33(1):35-41.   Published online January 31, 1990
We reviewed 113 patients with Staphylococcus aureus infection and 219 strains of Staphylococcus aureus isolated from pediatric patients of Seoul National University Hospital from Jan. 1, 1987 to Dec. 31, 1987. The results were as follows: 1) The proportion of MSSA was 50.2% of total Staphylococcus aureus isolated and that of MRSA was 49.8%. Staphylococcus aureus were isolated from pus (33.8%), urine (16.9%), ear discharge (8. 2%),...
A Case of Mixed Gonadal Dysgenesis.
Byung Ran Yun, Jae Il Sohn, Sei Won Yang, Hyung Ro Moon, Je Geun Chi
Clin Exp Pediatr. 1989;32(12):1757-1762.   Published online December 31, 1989
Mixed gonadal dysenesis is a disorder of sexual differentiation which is characterized by streak gonad associated with contralateral tesis, persistent Mullerian duct structures and/or sex chromosomal mosaicism most commonly with 45, X/46, XY. The authors experienced a case of mixed gonadal dysgensis in a 6-year old child who was reared as female and admitted due to clitoral enlargement. The patient had asymmetrical gonads consisted with...
Post-Exercise Pulmonary Function Changes in Asthmatic Children.
Kyung Ae Yoon, Young Yull Koh, Hyung Ro Moon
Clin Exp Pediatr. 1989;32(8):1074-1085.   Published online August 31, 1989
To determine the frequency, severity, time course and relations of exercise induced changes in lung function, forty-one asthmatic children and fifteen control children were subjected to free running and forced vital capacity (FVC), one-second forced expiratory volume (FEV1), maximum mid-expiratory flow rate (FEF 25-75%), and peak expiratory flow rate (PEFR) were measured before and serially for 60 minutes following exercise. The results were as follows. 1) Significant...
Late Infantile Metachromatic Leukodystrophy-Arylsulfatase A Assay in 24h Urine.
Hong Jin Lee, Yong Joon Shin, Yong Seung Hwang, Hyung Ro Moon, Jeong Seon Seo
Clin Exp Pediatr. 1989;32(7):978-983.   Published online July 31, 1989
A 2 year 5 month old female infant with late infantile metachromatic leukodystrophy was diagnosed by typical clinical and laboratory findings and Arylsulfatase A assay in 24h urine. The patient’s mother was confirmed as a heterozygote by Arylsulfatase A activities in 24h urine which was about one half of normal control groups.
Bone Age Determination and Hand Radiographic Findings in Children With Russell-Silver Syndrome.
Hun Kyu Lim, Sei Won Yang, Hyung Ro Moon
Clin Exp Pediatr. 1989;32(6):823-833.   Published online June 30, 1989
Russell.Silver syndrome is a disease of unknown etiology characterized by prenatal onset dwarfism irrespective of full term gestation, growth retardation, body asymmetry, craniofacial dysproportion with a triangular face, short and incurved little finger, etc. Forty.two children were diagnosed as Russell.Silver syndrome by Silver’s criteria at SNU Children' s Hospital from Apr. 1981 till Aug. 1988, and we reviewed clinical data and hand radiographs of...
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