Castleman's disease(CD) is rare in childhood. It is defined as a localized nodal hyperplasia in mediastinum or cervical area. It is also called angiofollicular lymph node hyperplasia, lymph nodal hamartoma, giant lymph node hyperplasia. It was first described in 1956 by Castleman et al. as a lesion of mediastinal mass. The etiology of CD is not clear. The histologic classification... |
Purpose : Although the chronic sinusitis is one of the most common and troublesome respiratory diseases in children, the pathogenesis still remains unclear. It is suggested that many of the immunologic factors including allergic conditions may contribute the nasal inflammatory changes. This study was designed to evaluate and demonstrate the possible role of various immunologic factors on the pathogenesis of... |
Purpose : This study was performed to detect the diseases of congenital hypothyroidism by the neonatal mass screening test early and to demonstrate the possible role of topical iodinated antiseptics(povidone-iodine, PVP-Ⅰ) on transient hyperthyrotropinemia in newborn infants. Methods : We performed neonatal screening tests for inborn errors of metabolism since 1985 by Guthrie test for PKU, maple syrup urine disease, histidinemia,... |
Purpose : To know the relationship between serum vitamin A level and clinical symptoms in measles patients and to re-evaluate the measles vaccination schedule, we performed this study. Methods : From Jan. to Jun. 1994, we checked serum vitamin A levels and compared with clinical symptoms, laboratory findings, complications and vaccination status in 153 patients with positive measles IgM by ELISA. Results :... |
Purpose : Intrabventricular hemorrhage is a common neuropathologic finding in premature and low birth weight infants. It is deeply related with neonatal death and neurologic sequelae. We want to know the incidence and the relating factors of intracranial hemorrhage in premature and low birth weight infants. Methods : We performed craniosonography in 170 premature and low birth weight infants in 3-7... |
Congenital lipoid adrenal hyperplasia is the rarest type among salt losing types of congenital adrenal hyperplasia. The defect of this disorder is in the cholesterol side chain cleavage enzyme(P450SCC)which converts cholesterol to pregnenolone. We experienced a case of 20,22 desmolse deficiency in a 21-day old phenotypically female who was admitted to our hospital due to lethargy and dark skin pigmentation. The... |
Sjören-Larsson Syndrome is a rare disorder chracterized by congenital icthyosis, spastic diplegia or tetraplegia and mental retardation. The inheritance is autosomal recessive, due to deficiency of alcohol dehydrogenase activity of fatty acid matabolism. We have experienced a case of Sjören-Larsson Syndrome in a 16 month-old male children who have dried thick skin, developmental delay, and spastic diplegia. He was much improved clinically... |
Phenylketonuria in metabolic disorder that results from a deficiency of the hepatic phenylalanine hydroxylase. But among patients with hyperphenylalaninemia, the defect resides in one of the enzymes necessary for production or recycling of tetrahydrobiopterin (BH4). The reduction of BH4 affects not only phenylalanine metabolism but also formation of the tyrosine related neurotransmitter, dopamin and tryptophan related neurotransmitter, serotonin. Administration of L-dopa... |
Intracranial hemorrhage is the most common neuropathologic finding in premature infants. But in full-term infants, It is less common and rarely causes death. We found out intracranial hemorrhages in 21 full-term neonates by real-time neurosonography and concluded as followings. 1) Among 21 neonates, 17 infants were male and 4 infants were female. 2) In 11(52.3%) infants the hemorrhage was detected within 7... |
Iron deficiency remains the most common cause of anemia in infants and children despite increasing availability of iron-fortified foods. We screened out anemia in 9-month old infants in well baby clinic to know the prevalence of anemia and the weaning status. The results were as follows. 1) Among 345 infants screened, 24 infants(7%) were found to have anemia. 2) The king of anemia... |
We made a clinical study on 10 cases of histiocytosis syndrome who had been admitted to the pediatric department of Soon Chun Hyang University Hospital from Jan 1982 to Dec. 1991. The results were obtained as follows 1) The sex incidence revealed male predominance with the ratio 4:1. 2) Among 10 cases, 4 cases were classified as eosinophilic granuloma, 1 case as Letterer-Siwe... |
To determine the normal neonatal adrenal gland size, ultrasonographic examinations were performed in 145 newborn infants. They were divided into 3 groups according to the days of age. The group I is 1~3 days, group II is 6~9days and group III is 21~50days of age. 1) The adrenal gland size was as follows. In group I, the length was 29.05mm and the... |
The association of colonic atresia in patients with Down syndrome is a rare anomaly. The incidence of congenital atresia of the gastrointestinal tract has been estimated to be about one in 1500 births. Colonic atresia is rarer still, and is throut to comprise about 5% to 10% of this group. This intestinal atresia occurs in about 30% to 50% of... |
Laryngotracheoesophageal cleft is a rare congenital anomaly characterized by a midline defect of variable length between the posterior larynx and trachea and the anterior wall of the esophagus which was first reported by Richter in 1792. The male, birth weight 2780 gm, was born our hospital. After birth the infant breathed spontaneously, cried immediately but weak and did well initially but... |
We studied anti-HBs titer, positive and effective rate in relation to dosages(5, 10) and time interval after third vaccination in 23 infants born to HBsAg negative mother. The babies were divided into two groups. In one group(n=12), 5 of Hepavax was administered intramusculary at 1 month, 2 months and 6 months age, in other group (n=11), 10 of Hepavax at... |
Neonatal hydrometrocolopos is a rare congenital anomaly, which is thought to be caused by both vaginal obstruction such as imperforate hymen, transverse vaginal septum or vaginal atresia and sufficient maternal estrogenic stimulation to provoke secretion from the glands in the neonatal reproductive tract. The presence of lower abdominal mass in a female infant should always arouse the suspicion of hydrometrocolpos... |
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... |
Juvenile polyps are common and cause painless hematochezia in preschool and school-aged children. Juvenile polyps of the colon are usually solitary and considered to be inflammatory of hamatomatous in nature without malignant potential. Multiple juvenile polyposis is characterized by large numbers withch is spread to the colon or throughout the gastrointestinal tract. We experienced a nonfamilial multiple juvenile polyposis in a... |
Neonatal screening for congenital hypothroidism is important because of the possibility that mental retardation may be avoided if treatment started early. A 1 day old patient was admitted to our department of Pediatrics for congenital hypothyroidism screening. The mother was 33 years old and had been on thyroid replacement therapy since 32 years of age. During the pregnancy she was euthyroid... |
Forty one cases of serologically confirmed measles(positive measles specific IgM antibody) were evaluated among 55 cases of clinically diagnosed measles admitted to Soon Chun Hyang University Hospital from May to August 1990. The results were as follows: 1) Sex ratio was 1.6 : 1 with male predominance in 41 cases, 13 cases(31.7%) were vaccinated and 28 cases(68.3%) were unvaccinated. Primary vaccine failure... |
A 3-year-old female patient was admitted due to marked abdomainal distension. Her mother has been noticed the abdominal distension since birth, and the abdomen was enlarged progressively, but there were no subjective symptoms such as abdominal pain, vomiting nor indigestion. The radiographic findings showed huge intraabdomainal mass anterior to intestine which had septated fluid collection in it. Omental cyst was... |
Thirteen cases of phenylketonuria who were diagnosed at department of pediatrics, Soonchunhyang University Hospital, and four cases at other hospitals and other countries from July, 1984 to April, 1991 were reviewed and analyzed. The results were as follow: 1) All 17 cases were between the age of 3 days and 19 years, and the male to female ratio was 1.43:1. 2)... |
Until recently, minimal information on the development of body part identification in young children has been available although several developmental assessments include items that involve pointing to or naming body part. The purpose of this study was to examine the sequence in which body parts are learned and can be identified by very young children. The 360 children who 1-to 5-year-old were tested during the... |
We made clinical and statistical observation of 90 very low birth weight infants who were admitted at Soonchunhyang university hospital during the 6 years period from January 1985 through December 1990. The result obtained were as follows: 1) The incidence of very low birth weight infant during 6 years period was 0.68%, and the mortality rate was 488/1000 very low birth weight infants. 2) There was no obvious... |
Thymolipoma is a benign anterior mediastinal tumor composed of thymic tissue and mature adipose elements. The tumor is so rare that only 60 cases are reported in the world, and only 2 cases are reported in Korea. We have experienced two cases of thymolipoma in two thirteen-year-old boys. One had had chest pain and exertional dyspnea for 1 month but the other had no... |
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 Cyclopia in which the elements of the two eyes are completely or partially fused to form an apparently single eye in the middle of the forehead, is part of an anomaly involving not only the eyes themselves but also the anterior part of the brain and the mesodermal structures in the midline. We have experienced two cases of Cyclopia in twin. The autopsy... |
Cardinal clinical features of trisomy 8 are as follow: Absent patellae; mental retardation; facies notable or its anteverted nose, long philtrum, micrognathia and malformed ears; flexion deformities of the fingers or toes, deep plantar V-shaped cleft between the first and third interdigital web of foot, “pH capitonne”. More than 35 patients have been identified as having trisomy 8. The authors experienced a patient of... |
We reviewed 34 patients with palpable abdominal mass among 5,920 infants and children who admitted to the department of pediatrics of Soon Chun Hyang University from Jun. 1, 1986 to Aug. 31,1989. The results were as follows: 1) The incidence was 0.57% and the male: female ratio was 1:1.3. The mass was noted before 1 year of age in 23.5% of cases and under 5 years... |