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

Accessory auricle is a relatively rare congenital anomaly arised from the abnormaI development of the auricular tubercles or from the tissues surrounding the second, third and fourth branchial clefts. Clinically this anomaly is a small akin-colored tag or nodule, soft and globular or cartilaginous consistency on or near the tragus along a line drawn from the tragus to the angle of the mouth or along the anterior margin of the sternomastoid muscle. The lesion is usually solitary and located in the preauricular area but may be multiple and rarely bilateral. We present a typical caae of accessory auricles in 4-day-old female infant. The patient had two skin-colored firm nodules on the left preauricular area and well pedunculated one on the Ieft buccal area near the angle of the rnouth. Histopathologic find.ings showed numerous pilosebaceous units, abundant subcutaneous fat and cartilage tissue in the center of the lesion.
Branchial Region ; Cartilage ; Female ; Humans ; Infant ; Mouth ; Subcutaneous Fat

PURPOSE: To evaluate the clinical manifestations of various glomerular diseases in children, a clinicopathological study was performed in 52 children who had renal biopsy. The type and relative incidence of the glomerular pathologies were analyzed, and the clinical predictability and usefulness of renal biopsy in glomerular diseases were assessed. METHODS: Medical records of fifty two children with renal disease who had undergone percutaneous renal biopsy under ultrasonic guidance at Chungnam University Hospial from October 1995 to August 2003 were reviewed. In addition, we compared the clinical findings before renal biopsy with the pathological diagnosis. RESULTS: The male to female ratio was 1.6:1 and they were 9.8 2.6 years old on average. The chief complaints for biopsy were hematuria in 22 cases which was the most common (42.3%), proteinuria in 16 cases(30.8%), and hematuria & proteinuria(26.9%). Among the 22 cases of hematuria, there were 15 cases of gross hematuria(68.2%) and 7 cases of microscopic hematuria(31.8%). In terms of histopathologic diagnosis, most of them were primary glomerular diseases(84.6%), which included IgA nephropathy(28.8%), thin glomerular basement membrane disease(25.0%), focal segmental glomerulosclerosis(FSGS)(11.5%), membranous proliferative glomerulonephritis(7.7%), minimal change lesion(3.8%), acute poststreptococcal glomerulonephritis(3.8%) and membranous glomerulonephritis(3.8%). The clinical manifestations and pathologic diagnosis were not correlated. CONCLUSION: The clinical manifestations could not predict the pathological diagnosis. Therefore, renal biopsy would be inevitable in diagnosis of glomerular diseases for effective management and assessment of prognosis.
Biopsy* ; Child* ; Chungcheongnam-do ; Diagnosis ; Female ; Glomerular Basement Membrane ; Hematuria ; Humans ; Immunoglobulin A ; Incidence ; Male ; Medical Records ; Pathology ; Prognosis ; Proteinuria ; Ultrasonics

This study is aimed to elucidate the biological nature of the precancerous lesions and to evaluate whether the ethanol alters 3'-Methyl-4-dimethylaminoazobenzene (3'-Me-DAB) induced experimental hepatocarcinogenesis. A total of 108 Sprague-Dawley male rats were used for the experiment and divided into 6 groups according to 3'-Me-DAB and ethanol administration. Administration of the drugs were carried out daily by nasogastric tube insertion and the animals were sacrificed at different interval. A part of right lateral lobe was prepared for the histological examination. Cell kinetics of the immunohistochemical method for bromodeoxyuridine (BrdU). The administration of 3'-Me-DAB induced oval cell proliferation, hyperplastic nodule, cholangiofibrosis and carcinoma in the liver. The mean labelling indices, the percentages of BrdU labelled cells, of hepatocytes were increased by administration of 3'-Me-DAB, only to reverse after cessation of the drug (2.58 vs 0.61). The labelling indices of the oval cells were also affected by the administration and cessation of 3'-Me-DAB (11.41 vs 4.48). In contrast, the cholangiofibrosis did not decrease but were still increasing following cessation of 3'-Me-DAB administration (4.37 vs 5.17 and 8.25 vs 11.29). These finding that the hyperplastic nodule and particularly the cholangiofibrosis have an autonomous proliferative potential and are definite precancerous lesions in the experimental hepatocarcinogenesis. Short term administration of ethanol decreased the incidence of development of the precancerous lesions, but did not affect the labelling indices in all the pathologic lesions of hepatocarcinogenesis.
Male ; Humans ; Rats ; Animals ; Incidence