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

No abstract available.
Hemoptysis*

The Graf stabilization has been introduced in treating lumbar spinal disorder associated with posterior instability. This study reviewed some problems of the Graf instrumentation as a soft stabilizer. The purpose of this study is to analyse the problems of the soft stabilization in spinal instability. We reviewed 145 cases which were operative treatment using the Graf instrument for lumbar spinal disorder associated with posterior instability at our department from May, 1991 to Dec, 1995. The mean follow up periods was 29 months ranging from 24 months to 6 years 8 months. Of the 145 cases, 22 cases were showed the problem. The diagnostic method were simple x-ray, flexion-extension lateral stress view and CT scan. Results were as follows : Adjacent segmental instability was 10 cases(6.9%), disc space narrowing was 8 cases(5.5%), screw loosening was 3 cases(2.1%) and breakage of the Graf band was 1 case(0.6%). The problems of the soft stabilization were adjacent segmental instability, disc space narrowing, screw loosening, and breakage of the Graf band. But the rate of adjacent segmental instability and disc space narrowing was lower than other lumbar spinal instrumentation.
Follow-Up Studies ; Tomography, X-Ray Computed

STUDY DESIGN: This is a retrospective study analyzing 73 patients treated by decompression, pedicular screw instrumentation and posterior lumbar interbody fusion (PLIF) for lumbosacral spondylolithesis and symptomatic spinal stenosis. we used laminected laminar and spinous process instead of iliac bone. OBJECTIVES: This is to evaluate fusion rate and clinical results of PLIF with laminected laminar and spinous process. MATERIALS AND METHODS: PLIF in 73 patients with lumbosacral disorders who carried out at Pohang St. Mary's Hospital from March 1994 to January 1996. Fusion rate was evaluated by simple X-ray and dynamic view. Clinical effect was evaluated by Kirkaldy-Willis criteria. RESULTS: The solid fusion was achieved at 67 cases(91.8%) and the average period of fusion was 6.4 months. Complications were 2 cases of superficial skin infection and 3 cases of root irritation. The functional results by Kirkaldy-Willis were as follows ; excellent 40 cases, good 23 cases, fair 9 cases and poor 1 case. CONCLUSIONS: This PLIF procedure combined with transpedicular instrumentation showed sufficient fusion rate and excellent clinical results and improvement in vertebral alignment.
Decompression ; Gyeongsangbuk-do ; Humans ; Retrospective Studies ; Skin ; Spinal Stenosis

No abstract available.
Bronchitis, Chronic* ; Humans

Epoxy resins,which are irritant and allergen, are now used for surface coatings, electrical insulations, adhesives, and construction industry throughout the world. However, epoxy resin contact dermatitis has not been reported in the Korean literature. We report two cases of epoxy resin contact dermatitis which were occupationally related. They have been in the same work, painting dye and resin mixtures on the glasses for decoration. They showed positive patch test reactions to epoxy resin 1, and their own materials containing resins and accelerators which were proved to be -countinue-
Adhesives ; Construction Industry ; Dermatitis, Contact* ; Eyeglasses ; Glass ; Occupations ; Paint ; Paintings ; Patch Tests

No abstract available.
Uterus*

Elephantiasis neuromatosa is one of 3 types of cutaneous tumors in Von Recklinghausen's disease. This 28 year-old male patient visited for adult-fist sized tumor associated with hairy pigmented patch on right antecubital fossa, and various sized tumors and cafe-au-lait spots on whole body of about 27 years duration. Past history was noncontributory. Family history revealed that his father and son had similar manifestations. Laboratory findings were noncontributory. Histopathology of cafe-au-lait spot and hairy pigmented patch revealed increase of melain in basal cell layer of the epidermis and increase in the concentratiton and activity of the melanocytes in basal cell layer of the epidermis. Histopathology of the mass revealed slightly thin, wavy fibrs lying in loosely textured strands extending in varying directions and a fairly large number of nuclei that are ovel to spindle-shaped and fairly uniform in size.
Adult ; Cafe-au-Lait Spots ; Deception ; Elephantiasis* ; Epidermis ; Fathers ; Humans ; Male ; Melanocytes ; Neurofibromatosis 1

Although a lot of articles examining concomitant intrauterine and ectopic pregnancy have been presented, we have not found any reports on ectopic implantation of chorionic villi in the vagina with normal intrauterine pregnancy. We experienced a case of chorionic villi on the vaginal wall as a hemorrhagic mass during the second trimester of pregnancy. A pregnant woman (gestational age of 16 weeks and 1 day) visited our hospital due to vaginal bleeding. There was no abnormal finding except vaginal hemorrhagic mass, and the mass revealed a product of conception.
Chorion* ; Chorionic Villi* ; Female ; Fertilization ; Humans ; Pregnancy ; Pregnancy Trimester, Second* ; Pregnancy* ; Pregnancy, Ectopic ; Pregnant Women ; Uterine Hemorrhage ; Vagina