Sine the success of human growth hormone (GH) synthesis by DNA recombinant technology, these GH products are widely used in the treatment of children with growth hormone deficiency. Recently, methionyl-GH has been produced in the yeast, Saccaromyces cerevisiae 2150, by the use of a DNA recombinant method in Korea. The purpose of this study was to investigate the clinical efficacy, side effect and immunogenicity of this GH product during therapy of 3year in 22 children with naive GH deficiency. The subjects of this study are aged 4.5~17.9 years, diagnosed by the failure of plasma GH to respond to insulin induced hypoglycemia, arginine and/or clonidine loading and height below -2 standard deviation of mean for their chronological age. Each subjcct received GH 0.45IU/kg/week, intramusculary in 3 devided dose, During treatment, vital signs, height and body weight checked before and every 3 months. Blood count urinalysis, blood chemistry, bone age and measurement of thyroid hormone were performed before, every 3months in year 1, and every 6 months in year 2 and 3. Assay of antibody against GH were performed before, year 1,2 and 3. The height velocity was significantly increased from an average baseline level of 2.9+/-1.1cm/year to 7.9+/-1.9cm/year after the first year, 6.6+/-1.1cm/year during the second year, and 5.9+/-1.2cm/year during the third year of GH treatment. The height standard deviation score for chronological age was decreased from an average baseline of 3.5+/-1.3 to 2.9 1.2 after the first year, 2.7 +/-1.1 during the second year, and 2.5+/-1.2 during the year of GH treatment. The increment in height age was significantly increased from an average baseline of 0.6+/-0.2 year to 1.3+/-0.3 year in year 1, 1.1+/-0.3 year in year 2, and 1.0+/-0.2 year in year 3. The increment in bone age was significantly increased from an average baseline of 0.6+/-0.2 year to 1.2+/-0.2 in year 1, 1.3+/-0.3 year in year , 2, and 1.1+/-0.3 year in year 3. Antibody against hGH was observed in 3 of the 22 patients(13.5%) in year 1 and 1 and in 2 of the 17 patients(17.7%) in year 3. And unwanted side effects were not observed in any of the 22 patients. These results suggest that this yeast derived recombinant methionyl growth hormone appears to be a safe very effective product for treating of children with GH deficiency.
Arginine ; Body Weight ; Chemistry ; Child* ; Clonidine ; DNA ; Growth Hormone* ; Human Growth Hormone ; Humans ; Hypoglycemia ; Insulin ; Korea ; Plasma ; Thyroid Gland ; Urinalysis ; Vital Signs ; Yeasts*

A Histologic finding of the Cutis marmorata telangiectatica congenita( CMTC) is non-specific, but dilated capillaries and increased number of vessels are often observed. Capillary hemangioma, mostly represented by strawberry nevus, may show various clinical manifestations. We report a case of Cutis marmorata telangiectatica congenita, which may be an unusual clinical presentation of capillary hemangioma.
Capillaries* ; Fragaria ; Hemangioma, Capillary* ; Nevus

The injury of the epiphysis or epiphyseal plate produces undesirable effects on the normal growth of the bone, such as bone bridge, growth arrest and angular deformity. Authors performed vascularized fibular epiphysis and epiphyseal plate transplantation in 7 years old girl, who received the excision of the exostosis on distal ulna, followed by progressive varus deformity of right forearm and growth arrest of distal ulna. With follow
Congenital Abnormalities ; Epiphyses ; Exostoses ; Female ; Forearm ; Growth Plate ; Humans ; Methods ; Transplantation ; Ulna

Hereditary anhidrotic ectodermal dysplasia is a rare condition characterized by underdeveloped ectodermal structure including the skin, teeth or skin appendages. The patient has characteristic feature of anhidrosis, hypotrichosis and defective dentition. We experienced a case of hereditary anhidrotic ectodermal dysplasia in a l-month-old male infant who had unexplained recurring fever, anhidrosis and characteristic facial feature, so we established the diagnosis with clinical feature and skin biopsy.
Biopsy ; Dentition ; Diagnosis ; Ectoderm ; Ectodermal Dysplasia* ; Fever ; Humans ; Hypohidrosis ; Hypotrichosis ; Infant ; Male ; Skin ; Tooth

No abstract available.
Xanthomatosis*

No abstract available.
Pseudohypoaldosteronism*

Apert syndrome is an uncommon, congenital disorder characterised by malformation of the skull, most often acrocephaly or oxycephaly, in association with symmetrical syndactyly of both hands and feet. It is due to disturbance in the growth of bone and soft tissue affecting principally the head, the hands and the feet. The original description was presented by Troquart in 1886, and acrocephaloyndactyly was named by Apert in 1906. Since then, more than 200 cases have been reported in the world upto 1970. Recently, we have experienced three for typical Apert syndrome and made a brief related literature review
Acrocephalosyndactylia* ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Craniosynostoses ; Foot ; Hand ; Head ; Skull ; Syndactyly

Aneurysm of the vein of Galen is a rare midline arteriovenous malformation, usually presenting with cardiac failure in infancy or with hydrocephalus and raised intracranial pressure in older children. We experienced a case of the vein of Galen aneurysm diagnosed with computed tomographic (CT) features and magnetic resonance image (MRI), a new imaging modality. Our case was a 3 day-old male neonate and had a nonspesific symptom of high fever without cardiac failure. A brief review of related literature was made.
Aneurysm* ; Arteriovenous Malformations ; Cerebral Veins* ; Child ; Fever ; Heart Failure ; Humans ; Hydrocephalus ; Infant, Newborn ; Intracranial Pressure ; Male ; Veins*

We performed anorectal physiologic studies to evaluate the pelvic floor musculature and its innervation in 73 pregnant women(35 primiparous, 38 multiparous) who had been recruited into a study of pudendal nerve damage and its recovery in vaginal delivery as part of a prospective investigation. These women had all delivered by vaginal route with pros terolateral episiotomy. Pudendal nerve terminal motor latency was significantly increased 2~3 days after delivery but substantial recovery occurred in the first 2 months after delivery nearly to the pre-delivery value. Maximum average resting pressure was not affected by delivery. In contrast, maximum average squeeze pressure was decreased significantly 2~3 days after delivery and some recovery occurred in the first 6 months after delivery, which still remained significantly low relative to pre-delivery value. Perineal descent was significantly increased 2~3 days after delivery but substantial recovery occurred in the first 2 months after delivery, which still remained significantly low relative to pre-delivery value and thereafter no significant recovery was found. These results suggest pudendal nerve damage occurrs during vaginal delivery which recovers in the first 2 months after delivery but functional disturbance in pelvic floor sphincter muscuature persists thereafter, and we support avoidance of further vaginal delivery after previous damage to the innervation of pelvic floor sphincter musculature.
Episiotomy ; Female ; Humans ; Pelvic Floor ; Prospective Studies ; Pudendal Nerve*

PURPOSE: Instead of a chaotic non circadian environmental approach, continuing regular day-night rhythm in neonatal nurseries may benefit the development of preterm infants. The purpose of this study was to define the effects of cycled lighting on circadian rhythms of premature infants. METHODS: The experimental group included 15 preterm infants, and the control group, 15 premature infants in the NICU of a general hospital in Seoul. All infants were between 32 and 37 weeks' postconceptional age. The experimental group infants were provided with cycled lighting before discharge. The incubator or bassinet cover of the experimental group was off between 7 AM and 7 PM, and was covered between 7 PM and 7 AM. RESULTS: There were significant differences in the NNNS score (p=.039), and some significant differences in the sleep-activity pattern between the experimental group and the control group, but distinct differences in sleep-activity patterns between the two groups could not be defined. CONCLUSION: The results of this study suggest that cycled lighting can be helpful in the neurobehavioral development of preterm infants. By modifying the NICU environment to provide a more developmentally supportive milieu, nursing professionals can better meet the infants' physiologic and neurobehavioral needs.
Circadian Rhythm ; Hospitals, General ; Humans ; Incubators ; Infant ; Infant, Newborn ; Infant, Premature ; Light ; Lighting ; Nurseries ; Child Health