No abstract available.
Malformations of Cortical Development* ; Seizures*

No abstract available.
Malformations of Cortical Development* ; Seizures*

PURPOSE:Noonan syndrome(NS) is characterized by short stature, congenital heart disease, and typical facies. Recombinant human growth hormone(GH) has been reported to improve growth rate in a similar fashion to that seen in Turner syndrome. We investigated the clinical characteristics and growth reponses to GH therapy in children with NS. METHODS:The cases of sixty seven patients with NS were reviewed retrospectively. Ten of the 65 patients were assessed height, weight and pubertal stage every 3 months during GH therapy. RESULTS:Webbed neck(70%), delayed development(59.7%), low set posterior hairline(56.7%), eye abnormalities(56.7%) and mental retardation(55.2%) were the leading clinical characteristics. Short stature below the 3rd percentile was presented in 73.8 %. Growth patterns in NS children were variable and the evaluation of their growth must be individualized. The increments of height SDS were significant in children with GH therapy(height SDS:from -2.8+/-.6 to -2.3+/-.9, growth velocity:from 4.4+/-.8 cm to 9.2+/-.9 cm during first year, and 6.1+/-.1 cm during second year) (P<0.05). CONCLUSION: This study characterized the clinical profiles in Korean children with NS, which should be further extended with more children with NS. Additionally, the significant increase in final adult height after GH therapy in children with NS should be observed.
Adult ; Child* ; Facies ; Heart Defects, Congenital ; Humans ; Noonan Syndrome* ; Retrospective Studies ; Turner Syndrome

PURPOSE:Noonan syndrome(NS) is characterized by short stature, congenital heart disease, and typical facies. Recombinant human growth hormone(GH) has been reported to improve growth rate in a similar fashion to that seen in Turner syndrome. We investigated the clinical characteristics and growth reponses to GH therapy in children with NS. METHODS:The cases of sixty seven patients with NS were reviewed retrospectively. Ten of the 65 patients were assessed height, weight and pubertal stage every 3 months during GH therapy. RESULTS:Webbed neck(70%), delayed development(59.7%), low set posterior hairline(56.7%), eye abnormalities(56.7%) and mental retardation(55.2%) were the leading clinical characteristics. Short stature below the 3rd percentile was presented in 73.8 %. Growth patterns in NS children were variable and the evaluation of their growth must be individualized. The increments of height SDS were significant in children with GH therapy(height SDS:from -2.8+/-.6 to -2.3+/-.9, growth velocity:from 4.4+/-.8 cm to 9.2+/-.9 cm during first year, and 6.1+/-.1 cm during second year) (P<0.05). CONCLUSION: This study characterized the clinical profiles in Korean children with NS, which should be further extended with more children with NS. Additionally, the significant increase in final adult height after GH therapy in children with NS should be observed.
Adult ; Child* ; Facies ; Heart Defects, Congenital ; Humans ; Noonan Syndrome* ; Retrospective Studies ; Turner Syndrome

Human epithelial cell line immortalized by Ad12-SV40 hybrid virus was transfected with plasmid containing HPV-16 via calcium-phosphate method. Subsequently, 8 clonal cell lines were obtained after G418 selection. Among these clonal cells, clonal cell-4(C-4) and clonal cell-6(G-6) showed increases of tumorigenic cellular properties such as saturation density, soft agar colony formation and cell aggregation. Morphological alteration such as appearane of foci was observed on these two clones after passage 6 and 7(50 to 55 days after transfection). When clonal cells and control cells were treated with MNNG(0.01ug/ml), both C-4 and C-6 showed increases of tumorigenic cellular properties and the level of increase was much more elevated as compared to clonal cells prior to MNNG treatment. Appearance of foci formation was observed in C-4 and C-6 after passage-6. After passage-8, control cells and all clonal cells showed morphological alterations. It seems that treatment of cells containing HPV-16 DNA with MNNG increases tumorigenic properties of the cells and expedite morphological transformations. (continue)
Agar ; Cell Aggregation ; Cell Line ; Clone Cells ; DNA ; Epithelial Cells* ; Human papillomavirus 16 ; Humans* ; Methylnitronitrosoguanidine ; Plasmids

Acute tubular necrosis has been rarely reported as a complication of typhoid fever in the literature. We experienced four cases of acute tubular necrosis associated with typhoid fever in children. Patients showed significant titer of widal reaction associated with acute renal failure and one of them rised in 2 months after onset of clinical symptoms. Renal biopsy findings were compatible with acute tubular necrosis and immune complexes were not deposited in the glomeruli. Clinical and urinary findings were normalized by hemodialysis and antibiotic therapies. In conclusion, close longterm follow up of widal titer is mandatory in acute tubular necrosis, especially when associated with high fever.
Acute Kidney Injury ; Antigen-Antibody Complex ; Biopsy ; Child ; Fever ; Follow-Up Studies ; Humans ; Necrosis* ; Renal Dialysis ; Typhoid Fever*

Two cases of skull metastasis from thyroid carcinoma are presented. The one case shows multiple involvement inthe skull base with sphenoid sinus, frontal bone, and rib. The other case is solitary extensive involvement to theright parietal bone. The follicular caroinoma of the thyroid shows a strong tendency toward vascualr invasion and dissemination through blood stream to the lungs, bones, liver, brain, and other distant sites. The sternum, thevetebrae, and the pelvis are the most common sites in bone metastasis of thyroid carcinoma, followed by the femur,shoulder girdle, skull, and rib. Metastatic caroinoma to the skull base, including sphenoid sinus s extremelyrare. Authors experienced recently 2 cases of metastatic carcinoma of the thyroid to the skull and report withreview of literature.
Brain ; Frontal Bone ; Liver ; Lung ; Neoplasm Metastasis ; Parietal Bone ; Pelvis ; Ribs ; Rivers ; Skull Base ; Skull ; Sphenoid Sinus ; Sternum ; Thyroid Gland ; Thyroid Neoplasms

For the study on the effect of retinoic acid on the formation of palatal rugae and the cleft palate, retinoic acid was administered orally 150mg/kg of body weight by gastric tube at GD 10.5 to Sprague-Dawley rats. The pregnant rats were sacrificed on GD 17.5 under ether anesthesia, and laparatomized. After removal of uterus, the number of pregnant sacs and fetuses were counted. The fetuses weighed, the MEE (medial edge epithelium) thickness measured and the mitotic figures counted after routine processing and H·E stain. All the palates were photographed, and the number of rugae & the rugal pattern analysed. TEM photographs of MEE cells were observed after routine processing. The results were as follows ; 1. Rat fetus body weight after retinoic acid treatment increased significantly compared with the control group. 2. Mitotic figures in the retinoic acid treated group increased significantly compared with control group. 3. In the retinoic acid treated group, 79.3% of fetuses had cleft palates. Among fetuses with cleft palates, complete cleft palates were 10.6%, incomplete cleft palate 89.4%. Incomplete clefts were of two types ; median type (cleft palate at the intermolar region) and soft palate type (cleft posterior to the 8th rugae). Median type was 64.6% and the soft palate type 35.4%. 4. 2.3% of the fetuses had the numerical anomaly of the palatal rugae in the control group, but that of retinoic acid treated group 87.7%. 5. 17.4% of palatal rugae of the control group was disrupted, but 100% of the retinoic acid treated group disrupted. 6. Rugal papillae were observed in the 15.1% of fetuses of the control group and 63.1% of fetuses of the retinoic acid treated group. 7. Longitudinal rugae were observed in 19% of fetuses of the retinoic acid treated group, but not in the control group. 8. In TEM photographs, cytoplasmic processes, intercellular space, and desmosomes decreased. Swelling of mitochondria & ER were also found in the retinoic acid treated groups. According to the above results, it appears that there is close relationship between palatal rugae and cleft palates, and that excess retinoic acid induces disruption of pattern and numerical variations of rat fetus palate rugae. Also retinoic acid has an inhibitory effect on the proliferation of medial edge epithelial cells of palatal shelves. The cleft palates may be induced by the above mentioned retinoic acid effects. But, the exact mechanisms of retinoic acid on cleft palate formation is not thoroughly known and should be further studied.
Anesthesia ; Animals ; Body Weight ; Cleft Palate* ; Cytoplasm ; Desmosomes ; Epithelial Cells ; Ether ; Extracellular Space ; Fetus ; Mitochondria ; Palate ; Palate, Soft ; Rats ; Rats, Sprague-Dawley ; Tretinoin* ; Uterus

Colloid Milium is a degenerative disease in the dermal connective tissue characterized by the development of yellowish translucent papules or plaques on sun exposed skin. Clinically, there are two types of colloid milium namely, a juvenile type which starts before puberty and an adult type which involves sun exposed areas of adults. In both types the lesion is limited to the skin. Histologically colloid milium, like amyloid, shows homogenous, fissured aggregates, staining faintly eosinophilic, and occupying most or all of the involved. dermal papillae. A case of colloid. milium in 46 year old female is reported. About 4 years ago numerous yellowis4 translucent papules, 0. 1~0. 2cm in diameter developei on nose and lateral aspects of both eyes, which were gradually increasing in its size. Diagnosed confirmed by clinical features and histopathologic finding. Literature were reviewed.
Adolescent ; Adult ; Amyloid ; Colloids ; Connective Tissue ; Eosinophils ; Female ; Humans ; Middle Aged ; Nose ; Puberty ; Skin ; Solar System

PURPOSE: This study was made to evaluate the clinical characteristics and the treatment of the gangrene of the male genitalia manifested with progressive life threatening disease. MATERIALS AND METHODS: Between June 1992 and December 1998, medical records for patients with Fournier's gangrene were reviewed with respect to age at presentation, associated disease, and clinical course and the treatment. RESULTS: The age of patients ranged from 29 to 92 years. The prodromal sign noted were usually low abdominal and inguinal pain. There were no differences in the causative pathogens compared to those of previous reports. The common underlying diseases were diabetes and chronic liver disease. Hypoalbuminemia and leukocytosis were found in all. Early radical debridement and aggressive antibiotics therapy were performed in all cases. Reconstructive surgery of defected skin was performed 7 to 45 days(mean 33.6) after debridement and period of hospital stay ranged from 14 to 123 days(mean 35.8). The motality rate was 13.3%. CONCLUSIONS: It is thought that the motality rate of Fournier`s gangrene is reduced by early aggresseve debridement of the wound and empirical triple antibiotic therapy, and the comprehension of clinical course is important as well.
Anti-Bacterial Agents ; Comprehension ; Debridement ; Fournier Gangrene ; Gangrene* ; Genitalia, Male ; Humans ; Hypoalbuminemia ; Length of Stay ; Leukocytosis ; Liver Diseases ; Male ; Medical Records ; Prodromal Symptoms ; Skin ; Wounds and Injuries