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*

Bone materials diggd up from the Haemi nameless martyrdom holy place burial sites were severely broken into fragments of various size, clay matrix filled the bone marrow cavities, grass roots were spreaded into the periosteum and the fragments were very soft enough to be flaken. To make permanent specimens, bony fragments impregnated within unsaturated polyester resin with catalyzer MEKP, promotor 8% cobalt octoate, and UV absorber. Permanent specimens were so very transparent that all the macroscopic structures can be observed, and they were hard enough not to be broken or cracked. This method of preserving bony fragments is considered to be used in archaeologic preservation and in making teaching materials of various human organs or tissues.
Bone Marrow ; Burial* ; Cobalt ; Humans ; Methods ; Periosteum ; Poaceae ; Polyesters ; Teaching Materials

Cyclodialysis and ciliochoroidal detachment was performed in one eye of three rabbits and in one eye of another three rabbits respectively. 0.1 ml of 10% sodium fluorescein was injected intracamerally after aspiration of aqueous humor and the eyeball was enucleated between 30 minutes and one hour after sodium fluorescein injection and prepared for fluorescence microscopy. Sodium fluorescein concentration in supraciliary space was much greater in group with cyclodialysis or ciliochoroidal detachment than in normal control group. These results suggest that in the eye with cyclodialysis, aqueous humor may gain access freely to supraciliary space through the cleft between anterior chamber and supraciliary space and then is removed rapidly and in the eye with ciliochoroidal detachment, aqueous humor may pass through uveoscleral outflow pathway.
Anterior Chamber ; Aqueous Humor* ; Fluorescein ; Microscopy, Fluorescence ; Rabbits

BACKGROUND: The Soonchunhyang University Hospital Bloodless Center was established in 2000, and more than 2,000 bloodless surgeries have been performed there since. In this study, the lowest postoperative Hb/preoperative Hb (Hblow/pre) ratio and mortality rates of patients who underwent bloodless surgery were analyzed for each maximum surgical blood order schedule (MSBOS) category to assess whether MSBOS can be used as a predictor of successful completion of bloodless surgery. METHODS: A total of 971 patients were included. MSBOS was defined as the average number of units of RBCs transfused during each elective surgery. We used the Hblow/pre ratio as an alternative to intraoperative blood loss. Frequency of Hblow/pre ratios < or =0.5, use of transfusion alternatives, and mortality rates were compared across MSBOS categories. RESULTS: Out of the 971 patients, 701 (72.2%) were categorized as type and screen (T&S), 184 (18.9%) as MSBOS 1, 64 (6.6%) as MSBOS 2, and 22 (2.3%) as MSBOS 4. Transfusion alternatives were used by 397 (40.9%) patients. The frequency of the use of simultaneous erythropoietin and iron, hemostatics, acute normovolemic hemodilution, and Cell Saver (Haemonetics corp., USA) was higher in patients in the higher MSBOS categories. Six (0.6%) patients died within 30 days of surgery. Hblow/pre ratios tended to be lower as the level of MSBOS category increased. CONCLUSIONS: Surgeries in the higher MSBOS categories tended to be associated with high blood loss and mortality. Active use of transfusion alternatives is recommended in patients in high MSBOS categories who are scheduled to undergo bloodless surgery.
Blood Loss, Surgical/mortality/prevention & control ; Bloodless Medical and Surgical Procedures/*methods/mortality ; Erythropoietin/therapeutic use ; Hemodilution ; Hemoglobins/analysis ; Hemostatics/therapeutic use ; Hospitals, University ; Humans ; Iron/therapeutic use ; Outcome Assessment (Health Care) ; Tertiary Care Centers

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*