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 purpose of this study is to determine whether magnetic resonance imaging(MRI) can assist in deci- sion-making in the treatment of acute posterolateral complex injuries. We reviewed retrospectively seven patients with acute posterolateral complex injury, among the 79 cases that had taken cruciate ligament recon- struction. MRI findings were then correlated with the results of examination under anesthesia or operi lateral reconstruction. Based on surgical and clinical findings, there were six complete and four partial posterolateral complex injuries. MRI was able to accurately describe the extent of injury in each case. All ten patients had concomitant anterior cruciate ligament tears, and seven were three posterior cruciate ligament injuries. The iliotibial band was avulsed from Gerdys tubercle in seven patients. In knees with complete tears, separation of the coronary ligament of the lateral meniscus was noted on MRI. A characteristic bone contusion on the anteromedial femoral condyle was present in nine patients. Popliteal tendon tearing was observed in eight cases, and biceps femoris was torn in six patients. In knee with complete tears, tears of the lateral collateral ligament complex was noted both on MRI and intraoperatively. A high-quality MRI in patients sustaining these injuries will assist the surgeon in directly applying surgical techniques in a timely fashion.
Anesthesia ; Anterior Cruciate Ligament ; Contusions ; Humans ; Knee* ; Lateral Ligament, Ankle ; Ligaments ; Magnetic Resonance Imaging* ; Menisci, Tibial ; Posterior Cruciate Ligament ; Retrospective Studies ; Tendons

No abstract available.
Peritoneal Dialysis, Continuous Ambulatory* ; Peritonitis, Tuberculous*

We report a case of metastatic carcinoma of the skin in 62-year-old male who showed multiple nodules on the anterior chest, scalp, face and left axilla for 2 months duration. He suffered from lower abdominal discomfortness for 6 months. Histopathologic findings revealed atypical cell nests and some tubular or glandular formation in the dermis and subcutaneous fat tissue. Material in the lumen showed diastase resistant and PAS positive, and alcian blue positive at pH 2.4, but negative pH 0.4. So it represented sialomucin. We did not find primary site of malignant tumor. In view of clinical and histopathological findings, it was considered to be originated from the large intestine.
Alcian Blue ; Amylases ; Axilla ; Dermis ; Humans ; Hydrogen-Ion Concentration ; Intestine, Large ; Male ; Middle Aged ; Scalp ; Sialomucins ; Skin* ; Subcutaneous Fat ; Thorax

Three cases of giant pituitray adenoma are reported. Two cases were operated by transsphenoidal approach and a case by transcranial approach. Transsphenoidal approach showed satisfactory results despite of marked suprasellar extension of tumors. We report rare giant pituitary adenomas with review of relevant literatures.
Adenoma ; Pituitary Neoplasms*

No abstract available.
Ectodermal Dysplasia*

No abstract available.
Ectodermal Dysplasia*

No abstract available.
Humans*

No abstract available.
Busan* ; Child ; Child, Hospitalized* ; Child, Preschool* ; Hepatitis B Surface Antigens* ; Humans