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

The cutaneous metastaaes from carcinoma of breast produce four definite clinical types: inflammatory carcinoma, telangiectatic carcinoma, nodular carcinoma, and carcinoma en cuirasse. We present a case of 51-year-old woman who had purpuric, hard, sclerotic plaque with ulceration, crust and. several pea-sized hemorrhagic papulovesicular eruptions on the right chest wall for one year. Histopathological findings show metastatic ductal carcinoma but, the primary focus was not determined.
Breast ; Carcinoma, Ductal ; Female ; Humans ; Middle Aged ; Thoracic Wall ; Ulcer

No abstract available.
Antibody Formation* ; Dust* ; Immunotherapy* ; Pyroglyphidae*

To study the development of distraction at the fracture site according to inaccurate insertion of the distal screw in treatment of femoral shaft fracture with interlocking intramedullary nail, we prepared 24 femoral bone model, fixed them with interlocking intramedullary nails after artificially making transverse fracture or spiral fracture at mid 1/3 of femoral shaft and distal 1/3 of femoral shaft. The cephalocaudal insertion angles of the distal screws ranged from 5.2 to 45.4. As the insertion angles increased, the distances between the fractured sites ranged from 0.1 to 8.2mm. Distractions splited more than 5mm were seen in the average insertion angle of 34.3+/-9.4 (28.2 -45.4 ). When the insertion was made few millimeter proximal from the center of the distal screw hole, which was parallel to the transverse section of the femur model, fracture distraction ranged from minimum 1.8mm to maximum 5.0mm. In conclusion, the cephalocaudal insertion angle or location of the distal screw can be reason for the distraction between the fractured sites in internal fixation with intramedullary nail in long bone like femur. To prevent the distraction of fractured site, it is important to make the insertion of the distal screw parallel to the transverse section of the femur and to insert into the center of the screw hole.
Femur ; Fracture Fixation, Intramedullary*

We experience a case of plexiform neurofibroma in a 10-year-old girl who had unusual giant pigmentation and multiple skcletal abnormalities including pectus cariimtum. Histologic examination of the lesion taken from right upper arm permitted a diagnosis of plexiform neurofibroma.
Arm ; Child ; Diagnosis ; Female ; Humans ; Neurofibroma, Plexiform* ; Pigmentation*

We experience a case of plexiform neurofibroma in a 10-year-old girl who had unusual giant pigmentation and multiple skcletal abnormalities including pectus cariimtum. Histologic examination of the lesion taken from right upper arm permitted a diagnosis of plexiform neurofibroma.
Arm ; Child ; Diagnosis ; Female ; Humans ; Neurofibroma, Plexiform* ; Pigmentation*

Normal lymphocyte transfer (NLT) test is composed of two peaks of delaycd. hypersensitivity reaction of erythema and/or induration developed at the site of intradermal inoculatioa of allogenic lymphocyte. It has been suggested that NLT test is a px.siblc homograft raaction, how var tlv exact mecha.nism is remained to be resolved. In leprosy, there has been accumulating evidenc. indicating defective cell mediated. immunity detected by varioua immunological tests. The present study was undertaken to evaluate the immune capability of patients with leprosy using NLT test. A total of 20 hea,lthy volunteers, 20 lepromatous and 22 tuberculoid leprosy patients who were under antileprosy chemotherapy with DDS for varying periods, entered this study. Lymphocytes from hepatitis associati antigen (HAA) negative donor were isolated with Ficoll- Hypaque density gradient method and the cell concentration was adjusted by normal saline so as to give 2, 5 million cells per 0. 1 ml. Each recipient was given 0. 1 ml lymphocyte suspension on the flexor surface of th right forearm intrad.ermally using 1 ml tuberculi n syringe with a 26 gauge needle. The appearance and the size of induration was checked daily for 2 weeks. In this atudy, the NLT reaction pattem was classified as follows: a. normal pattem showing the first peak and second peak of reaction. b. first peak only showing the first peak, but no second peak, of reaction. c. flat pattem showing neitger the first peak nor the second peak of reaction The results were as follows: 1. There were normal pattem in sixteen persons(80%) and first peak only in 4 persons(20%) among twenty healthy human volunteers. 2. There were normal pattem in four patients(20%), first peak only in six patients(30%) and flat pattem in ten patients(50%) among twenty letromatous leprosy patients. 3. There were normal pattem in twelve patients(54.5%), first peak only in eight patients(36.4%) and flat pattem in two patients(9.1%) among twenty-two tuberouloid leprosy patients. These findings suggest that NLT test may be useful in evaluating the immune capability in leprosy and host factor in belived to be important in the development of both first and second peaks of NLT test.
Allografts ; Diatrizoate ; Drug Therapy ; Erythema ; Forearm ; Healthy Volunteers ; Hepatitis ; Humans ; Hypersensitivity ; Immunologic Tests ; Leprosy* ; Leprosy, Tuberculoid ; Lymphocytes* ; Needles ; Syringes ; Tissue Donors ; Volunteers

No abstract available.
Immunization, Passive* ; Immunoglobulins*

Expression of the nm23 and E-cadherin proteins has been studied in a number of tumors. Reduced expression of the nm23 and E-cadherin proteins seems to be associated with metastasis or disease progression in some tumors, including breast carcinoma. To assess the role of nm23 and E-cadherin in tumor differentiation and metastasis of breast carcinoma, immunohistochemical staining for the nm23 and E-cadherin proteins was performed in paraffin embedded tumor samples from 86 breast carcinomas. The results were as follows: 1) Expression of the nm23 protein in breast carcinoma was strong positive in 32 cases (37.2%), weak positive in 26 cases (30.2%), and negative in 28 cases (32.6%) of the cases. Expression of the nm23 protein in breast carcinoma decreased according to histological grade and lymph node metastasis, but was not statistically significant. 2) Expression of the E-cadherin protein in breast carcinoma was strong positive in 50 cases (58.1%), weak positive in 24 cases (27.9%), and negative in 12 cases (14%) of the cases. Expression of the E-cadherin protein in breast carcinoma decreased according to histological grade and lymph node metastasis, but was not statistically significant. 3) There was a statistically significant correlation between the expression of the nm23 protein and the E-cadherin protein in breast carcinoma (p<0.05). These results suggest that the expression of the nm23 and E-cadherin proteins is related to tumor differentiation, and may also be an useful prognostic factor in breast carcinoma.
Breast Neoplasms* ; Breast* ; Cadherins* ; Disease Progression ; Lymph Nodes ; Neoplasm Metastasis ; Paraffin