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*

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

No abstract available.
Pseudohypoaldosteronism*

Epithelioid hemangioendothelioma of liver is a very rare tumor of vascular origin, the most peculiar feature of which is that it is composed of endothelial cells closely resembling epithelial cells. We present a primary epithelioid hemangioendothelioma of liver in a 40-year-old male. This tumor was composed of an ill-defined yellowish white, 7 x 4cm sized, firm, solid mass and small satellite nodules in the right lobe of liver. Microscopically, two types of tumor cells-dendritic and epithelioid-were identified. The neoplastic cells infiltrated into sinusoids and intrahepatic veins. The background of tumor showed marked sclerotic change and focal proliferation of bile ductules. Confirmation of the endothelial origin of these cells was provided by positive immunoperoxidase staining for factor VIII-related antigen, and by electron mi-croscopic demonstration of Weibel-Palade body. This is the first case of epithelioid heman- gioendothelioma of liver documented in Korea. We report this case in view of its scarcity and distinctive morphologic features that allow differentiation from sclerosing carcinoma and angiosarcoma.

The development of endotracheal tubes with high-volume and low-pressure cuffs has decreas ed the incidence of associated tracheal injury. If room air used for inflation of the cuff during general anesthesia using N2O-O2, mixtures, however, endotracheal tube cuff result in distention and potential pressure changes. Therefore, tracheal pressure injury may occur during long general anesthesia(especially, head and neck surgery). For the prevention of this problems and the determination of proper methods, we performed this study relating to nitrous oxide diffusion into a gas filled endotracheal tube cuff.
Anesthesia, General ; Diffusion ; Head ; Incidence ; Inflation, Economic ; Neck ; Nitrous Oxide*

A giant hydronephrosis caused by ureteropelvic junction stricture in a 16-month-old male infant was presented. After the size and volume of giant hydronephrosis were estimated by retrograde pyelography, nephrectomy was performed. We report this case with review of literatures.
Constriction, Pathologic ; Humans ; Hydronephrosis* ; Infant ; Male ; Nephrectomy ; Urography