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Case Report
2 Cases with Adult Respiratory Distress Syndrome in Infant and Young Child
Hye Jung Joo, Im Jae Park, Joon Soo Lee, Soo Young Lee, Byeung Ju Jeoung, Kyu Earn Kim, Ki Young Lee, Hyunee Yim, Soon Won Hong, Woo Hee Jung
Clin Exp Pediatr. 1992;35(8):1118-1126.   Published online August 15, 1992
Adult respiratory distress syndrome has been described as a clinical syndrome of respiratory distress and need for mechanical ventilation with positive end-expiratory pressure, diffuse pulmonary infiltration on thoracic roentgenogram, impaired pulmonary compliance, decreased lung volumes and capacities, increased alveoloarterial oxygen gradient and hyaline membrane formation. It is the clinical manifestation of injury to the terminal alveolocapillary unit as a result...
A Case of Prune Belly Syndrome ssociated with Turner Syndrome
Chang Soo Oh, Sang Muk Choi, Jae Ock Park, Chang Hwi Kim, Sang Jhoo Lee
Clin Exp Pediatr. 1992;35(7):978-983.   Published online July 15, 1992
The Prune belly syndrome is a rare congenital anomaly characterized by lax, wrinkled abdominal wall, cryptorchidism and urinary tract anomalies. But it has a wide spectrum of clinical presentation and combine with other anomalies such as pulmonary, skeletal, digestive, cardiovascular and other system. We experienced a case of prune-belly syndrome associated with Turner syndrome in a 18 month old female...
Hutchinson-Gilford Progeria Syndrome
Moon Whan Lee, Byoung Geun Lee, Pyung Han Hwang, Dae Yeol Lee, Jung Soo Kim
Clin Exp Pediatr. 1992;35(7):971-977.   Published online July 15, 1992
Hutchinson-Gilford Progeria Syndrome is an extremely rare condition that was initially reported by Jonathan Hutchinson in 1886 and further described by Hastings Gilford in 1904. Clinical manifestations are evident by the first or second year of life with the physical characteris-tics of the elderly. Progeric patients ordinarily develop atherosclerosis and die of cardiac or cerebral vascular disease between 7 and 27...
Original Article
A Clinical Aspect of the Hemolytic Uremic Syndrome
Hye Won Park, Tae Sun Ha, Il Soo Ha, Hae Il Cheong, Yong Choi, Kwang Wook Ko
Clin Exp Pediatr. 1992;35(7):909-920.   Published online July 15, 1992
We reviewed the medical records of 14 children (8 girls, 6 boys), diagnosed as hemolytic uremic syndrome at Seoul National University Children뭩 Hospital from 1981 to 1990. The age at presentation ranged from 1 month to 10 years, with a mean age of 2.7 years. Only eight (57.1%) of the children had diarrheal prodrome and five (35.7%) had grossly bloody...
Case Report
A Case of McKusick-Kaufman Syndrome
Han Joo Kong, Young Mi Jee, In Kyung Sung, Byung Churl Lee
Clin Exp Pediatr. 1992;35(6):829-833.   Published online June 15, 1992
We experienced a case of Mckusick-Kaufman syndrome in a 14 day-old female neonate. She had hydrometrocolpos with vaginal atresia and polydactyly of feet associated with bilateral hydronephrosis and umbilical hernia. Hydronetrocolpos with vaginal atresia was corrected by abdominoperineal-vaginal pull through operation on 21st day of life. A brief review of related literatures was made.
Original Article
Clinical Observation on Reye syndrome According to the Onset of Age in Children
Kyung Hee Kim, Baik Hee Lee, Myung Ik Lee, Don Hee Ahn, Keun Chan Sohn
Clin Exp Pediatr. 1992;35(6):788-794.   Published online June 15, 1992
A clinical observation according to the age of onset was made on 40 children with Reye syndrome who had been admitted to pediatric department of National Medical Center during 11 year period from January 1979 to December 1989. 1) Annual distribution showed the largest number in 1982. There was no significant difference in seasonal distribution. 2) Cases of infant comprised 22 (55.0%)...
Case Report
A Case of D13 Ring Chromosome Syndrome
Sung Lae Park, Ho Jun Im, Jae Hun Shin, Hahng Lee, Myung Soo Lyu, Yong Kyun Paik
Clin Exp Pediatr. 1992;35(5):713-717.   Published online May 15, 1992
A case of D13 Ring Chromosome Syndrome, confirmed by chromosome study, was described. She was 8 years and 4 months old, and menifested facial abnormalities, psychomotor retardation and other multiple congenital abnormalities.
Two Cases of Cornelia de Lange Syndrome
Yoon Jong Yoo, Ki Chan Na, Ho Seong Yoo, Sang Kee Park, Yong Bong Park
Clin Exp Pediatr. 1992;35(5):684-690.   Published online May 15, 1992
The Cornelia de Lange syndrome is characterized by severe growth and mental retardation, typical face, and low-pitched growling cry. We had experienced two cases of Cornelia de Lange syndrome in korean male infants. The baby showed microbrachycephaly, typical appearance of face with low forehead, bushy eyebrows, long curely eyelashs, thin lip with cleft palate, low set ears, generalized hirsutism and growth retardation...
A Case of Prader Willi Syndrome
K.N. Kim, Y.T. Jang, S.Y. Choi, W.S. Kim
Clin Exp Pediatr. 1992;35(5):678-683.   Published online May 15, 1992
Prader willi syndrome is characterized by infantile hypotonia, mental retardation, hyperphagia with obesity, and hypogonadism. The etiology of Prader Willi syndrome is still unknown; however, a hypothalamic defect has been postulated and recently, the deletion of long arm of chromosome 15 has been postulated and recently, the deletion of long arm of chromosome 15 has been considered as a cause...
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
The Incidence of Hypothyroidism in Children with Down Syndrome
Seong Hyeon Jeon, Chun Ho Cho, Kyoung Sim Kim, Ki Bok Kim
Clin Exp Pediatr. 1992;35(4):534-538.   Published online April 15, 1992
A review of Thyroid function tests was performed on 32 children with Down syndrome confirmed by cytogenetic examination at the Dept. of Pediatrics, Kwangju Christian Hospital from Jan.1989 to Feb.1990. 1) Twenty among 32 children with Down syndrome were male, with the sex ratio being 1.7 : 1. Their ages ranged from 3 days to 5 years, with the mean age...
Clinical Observation of Meconium Aspiration Syndrome: Prognostic Implication of Early Meconium Suctioning
Dae Hyun Kim, Dong Hoon Ko, Young Jong Woo, Young Youn Choi, Tae Ju Hwang
Clin Exp Pediatr. 1992;35(4):484-491.   Published online April 15, 1992
The authors observed the clinical findings of 36 patients with meconium aspiration syndrome(MAS) to evaluate the effect of early appropriated meconium suctioning on the morbidity and mortality of the syndrome. Patients in the study group, 20 babies born in Chonnam university Hospital, received appropriate meconium suction through endotracheal tube during and rig-ht after the delivery, while the patients in the...
Case Report
A Case of 7q-Syndrome
Ja Hyun Park, Baek Gil Lee, Il Kyung Kim, Jung Sik Min, Chang Hee Choi
Clin Exp Pediatr. 1992;35(3):422-427.   Published online March 15, 1992
7q-syndrome, although rare, is a well defined syndroime which usually arises de novo. Characteristic clinical features include severe growth and metal retardation, microcephaly, low birth weight, broad nasal bridge with bulbous nasal tip, large, low set ears and genital anomalies. We experienced a male newborn with clinical features suggestive of chromosomal anomaly which was confirmed to be a 7q-(q32-qter) syndrome...
Adult Respiratory Distress Syndrome in Infancy
Hoo Jae Hann, Young Mi Hong, Seung Joo Lee
Clin Exp Pediatr. 1992;35(2):245-251.   Published online February 15, 1992
Adult Respiratory distress Syndrome (ARDS) is a clinical entity characterized by respiratory distress and diffuse pulmonary infiltrates due to increased permeability after various kinds of insult to alveolar-capillary membrane. We experienced a case of ARDS in a 3-1/2 month old girl after Pseudomonas pneumonia, cardiac arrest and generalized convulsion. This is the report on her clincal and autopsy findings with brief review...
A Cases of Goldenhar's Syndrome
Jin Eun Hyun, Eun Hee Park, Hee Young Jeon, Whwa Jin Byeun, Young Mok Hwang
Clin Exp Pediatr. 1992;35(1):135-139.   Published online January 15, 1992
Goldenhar's syndrome is a syndrome consisting of epibulbar dermid or lipodermoid, preauricular skin appendices and blind ended fistulas and vertebral anomalies. We experienced a case of Goildenhar's syndrome is a one month-old boy who showed asymmetric face, preauricular skin tag, hemiertebrae, rib anomaly and cyanotic congenital heart disease. A review of the references concerning Goldenhar's syndrome was made birdefly.
Original Article
A case of transient myeloproliferative disorder in Down's syndrome.
Dong Uk Kim, Woo Ki Lee, Eung Won Park, Kwang Woo Kim
Clin Exp Pediatr. 1991;34(12):1740-1744.   Published online December 31, 1991
Transient myeloproliferative disorder has been reported in infants with Down’s syndrome. This disorder is clinically and hematologically indistinguishable from congenital leukemia. In contrast to congenital leukemia, complete clinical and hematological recovery occurs within weeks to months of diagnosis without antileukemic treatment. We experienced a case of Transient myeloproliferative disorder with Down’s syndrome. This 4-day-old female neonate showed jaundice, hepatomegaly, fever, dyspnea, mongoloid face and congenital heart...
A case of 13-ring chromosome syndrome.
Jong Soo Lee, Yong Tae Jung, Byung Hak Lim, Im Ju Kang
Clin Exp Pediatr. 1991;34(12):1736-1739.   Published online December 31, 1991
We have experienced a case of 13-ring chromosome syndrome in 9 month old girl who had microcephaly, trigonocephaly, hypertelorism, microophthalmia, broad prominent nasal bridge, high arched palate & speech defect. On X-ray study, brain computerized tomograpy showed Dandy-Walker variant. Chromosome study revealed 13-ring chromosome. Her mother had mild mental retardation, short stature, microcephaly, trigonocephaly, hypertelorism and micrognathia. A brief review of the literatures was also...
A case of acute megakaryoblastic leukemia with Down syndrome.
Sung Jin Chang, Sung Min Sohn, Heung Sik Kim, Chin Moo Kang, Dong Seok Jeon
Clin Exp Pediatr. 1991;34(12):1730-1735.   Published online December 31, 1991
We experienced a case of acute megakaryoblastic leukemia (M7) with Down syndrome. The patient was admitted due to premature SGA, which revealed characteristic facial figure of Down syndrome. M7 was diagnosed with PB smear which showed abundant megakaryoblast and confirmed by using the monoclonal antiplatelet glycoprotein IIb/IIIa antibody (J 15). On 25th hospital day, the patient gained weight but discharged without further treatment of M7. Authors...
Three cases of Watson-Alagille syndrome.
Eun Kyeong Bom, Dae Hyun Kim, Dong Hoon Koh, Young Youn Choi, Jae Sook Ma, Tae Ju Hwang
Clin Exp Pediatr. 1991;34(12):1699-1706.   Published online December 31, 1991
Watson-Alagille syndrome is a kind of familial intrahepatic biliary atresia associated with charac- teristic face and cardiovascular anomaly. Growth and developmental delay, anomalies of the eyes such as posterior embryotoxon, deformities of the skeletal system are often the manifestations of this syndrome. We experienced three cases of Watson-Alagille syndrome recently. These patients showed charac- teristic faces and had peripheral pulmonary artery stenosis diagnosed by pulmonary...
a case of type II lissencephaly; Walker-Earburg syndrome.
Ae Yong Kim, Jung Ho Lee, Yong Sub Kim, Kyeng Sook Cho, Jong Dai Jo
Clin Exp Pediatr. 1991;34(11):1598-1604.   Published online November 30, 1991
Lissencephaly is a rare abnormality of brain development characterized by incomplete neuronal migration and a smooth cerebral surface. Three types of lissencephaly are recognized. Type I is characterized by microcephaly and dysmorphic facies. Type II usually lacks characteristic facies but exhibits macrocephaly from hydrocephalus, eye anomaly, and/or congenital muscular dystrophies. Type III is characterized by microcephaly without any characteristic dysmorphic facial features. We have experienced...
A case of cerebro-oculo-facio-skeletal syndrome.
Sung Shin Park, Ju Seok Maeng, Jae Ok Park, D. H Lee, S. J Lee
Clin Exp Pediatr. 1991;34(11):1593-1597.   Published online November 30, 1991
COFS syndrome has been recognized as an autosomal recessive, apparently degenerative problem of the brian and spinal cord that usually manifest before birth. We experienced a female neonate of COFS syndrome, with multiple malformation wiich were, charaterized by low birth weight, microcephaly, microphthalmia, blepharophimosis, nystagmus, deep-set eyes, optic nerve hypoplsia, prominent nasal brideg, upper lip overlapping lower lip, micrognathia, retrognathia, widely set nipple, contracture of...
A case of 11q deletion syndrome.
Hye Kyeong Nam, Rhee Choi, Chang Hwi Kim, Sang Jhoo Lee
Clin Exp Pediatr. 1991;34(11):1587-1592.   Published online November 30, 1991
11q-syndrome is a rare chromosomal anomaly. We experienced a case of llq deletion syndrome in a female infant. It was diagnosed by clinical features and chromosomal study. She had multiple anomalies such as flat occiput, hypertelorism, low set malformed ear, anteverted nostril, small carp-shaped mouth, micrognathia, hypertrophic pyloric stenosis and ventricular septal defect. Chromosomal study showed partial terminal deletion of the long arm of chromosome...
A case of de Novo trisomy 12p syndrome.
Young Huuk Lee, Kir Young Kim
Clin Exp Pediatr. 1991;34(11):1581-1586.   Published online November 30, 1991
A case of de novo trisomy 12p syndrome has deen experienced in newborn male child recently. This male child manifested normal birth weight, weak crying, poor sucking, generalized hypotonia, multiple congenital anomalies on face and digits, and progressive psychomotor retardation. The diagnosis was made on the basis of typical morphologic features and chromosomal study. As this is the first case in Korea, it is...
Surfactant replacement therapy in neonatal respiratory distress syndrome.
Chan Ok Park, Boung Yul Lim, Byeong Gie Yeo, Ji Ho Song, Eun Kyung Sohn, Chong Woo Bae, Sa Jun Chung, Chang Il Ahn
Clin Exp Pediatr. 1991;34(9):1211-1222.   Published online September 30, 1991
Respiratory distress syndrome (RDS) in tiny infants remains a major medical challenge as they are less tolerant of supportive care. In 1959, after identification of the pulmonary surfactant (S), S replacement therapy were used to treat RDS. This idea was first successfully tested on RDS infants by Fujiwara, et. al” in 1980. Now the trial and use of S for the treatment in RDS...
A case of Rubinstein-Taybi syndrome.
Cheol Hee Hwang, Dong Gue Lee, Myung Sug Nam, Moon Kee Cho
Clin Exp Pediatr. 1991;34(8):1146-1150.   Published online August 31, 1991
Rubinstein-taybi syndrome is characterized by typical skeletal abnormalities such as broad thumb and large first toe, mental and motor retardation, and facial abnormalities. In addition, defects of the cardiovascular system and the urinary tract may be present. We have experienced a case of Rubinstein-Taybi syndrome which a five-year-old boy had broad thumb and large first toe, mental and motor retardation, and facial abnormaiities (palpebral...
The relationship between diuresis and pulmonary function in respira- tory distress syndrome of the newborn.
Eun Ae Park, Yung Sook Ko, Gyoung Hee Kim
Clin Exp Pediatr. 1991;34(8):1086-1092.   Published online August 31, 1991
The purpose of this study was to determine the relationship between improvement of pulmonary, function and diuresis in respiratory distress syndrome. This study was based on 24 cases of RDS infants admitted in NICU of Ewha Womans University from January 1988 to December 1990. The results were as follows. 1) Mean birth weight of study cases was 2.11 kg with gestational age between 29〜39 weeks. 2) Maximum...
A case of a variant of Pierre Robin syndrome -cerebrocostomandibular syndrome-.
Hye Jin Lee, Eun Jin Mun, Ock Seung Jeong, Son Sang Seo, Jeong Ja Kang
Clin Exp Pediatr. 1991;34(7):1022-1028.   Published online July 31, 1991
We experienced a case of cerebrocostomandibular syndrome in newborn consisted of costal deficiency, and micrognathia. It is a variant of Pierre Robin syndrome, and cleft palate and glossoptosis are frequently present. Respiratory difficulty due to rib dysplasia result in poor prognosis for survival. Brief review of literature was made.
A case of Prune Belly syndrome associated with holoprosencephaly.
Myung Hee Youn, Mi Sung Jeong, Hyung Ja Park, Shinna Kim, Keum Min Park, Song Ja Chin
Clin Exp Pediatr. 1991;34(7):1015-1021.   Published online July 31, 1991
This is brief report of a case of prune belly syndrome associated with holoprosencephaly in one-day old neonate. At birth, physical examination revealed skull bone defect, anophthalmia, severe hypotelorism, arhinia with proboscis, micrognathia, webbed neck, severely dilated abdomen with thin wall, agenesis of external urogenital organs and anus, equinovalgus and genu valgus. Autopsy findings were alobar holoprosencephaly with hydrocephalus, hypoplasia of lung and ribs, markedly dilated...
A case of Bartter's syndrome.
Kyu Young Lee, Byoung Soo Cho, Hah Young Lee, Jay Bernstein
Clin Exp Pediatr. 1991;34(6):849-856.   Published online June 30, 1991
Bartter’s syndrome in characterized by renal potassium wasting, hypokalemic alkalosis, hyper- reninemia, normal blood pressure and resistance to the blood pressure augmenting effect of angioten- sin, accompanied by often hypomagnesemia and increased production of certain renal prostaglandins. The primary defect in this rare disorder is considered to be a renal tubular dysfunction. We had experienced a case of Bartter’s syndrome in 9 months old...
A case of pneumoperitoneum secondary to pulmonary airleak and a similar case of abdominal subcutaneous emphysema.
Sei Joong Ko, Moon Chul Kim, Jai Eok Kim
Clin Exp Pediatr. 1991;34(6):837-842.   Published online June 30, 1991
Pneumoperitoneum in association with pulmonry airleak syndrome is rarely encountered in spite of the rather frequent occurence of the pulmonary airleak syndrome in the neonates and the premature babies under ventilator therapy. In most cases of pneiimoperitoneum, the major cause is gastrointes- tinal perforation which evidently requires emergency surgery. Nevertheless, no such aggressive procedure as explolaparotomy is needed in case of pneumoperitoneum secondary to...