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.
Ectodermal Dysplasia*

No abstract available.
Ectodermal Dysplasia*

Pneumomediastinum is a relatively uncommon, infrequently reported entity, In the evaluation of these entity, it is important to exclude pathological causes, including Boerhaave's syndrome which carries a high mortality. Spontaneous pneumomediastinum is related to excessive intraalveolar pressure leading to rupture of perivascular alveoli in the setting of a Valsalva maneuver without communication to gut material. So, it has a benign self-limited course and rarely requires medical intervention. On the contrary, secondary pneumomediastinum caused by instrumental, traumatic, and spontaneous perforation of esophagus. Although the prognosis have been improved since the advent of broad-spectrum antibiotics and nutritional support, pneumomediastinum due to esophageal perforation still has a high morbidity and mortality. The most important prognostic factor is the time interval between perforation and initiation of therapy, and an awareness and a high clinical suspicion is critical in the early diagnosis and treatment. Recently, we have experienced 2 cases of pneumomediastinum, one case was spontaneous pneumomediastinum and the other may be caused by instrumental esophageal perforation. We report the clinical course of the patients with a current literature review.
Anti-Bacterial Agents ; Early Diagnosis ; Esophageal Perforation ; Esophagus ; Fibrinogen ; Humans ; Mediastinal Emphysema* ; Mortality ; Nutritional Support ; Prognosis ; Rupture ; Valsalva Maneuver

BACKGROUND: Mixed venous oxygen saturation (SO2) monitoring turned out to be invaluable in following up systemic cardiovascular status. But balloon-tipped flow-directed thermodilution catheter (Swan-Ganz catheter) insertion carries additional inherent risks other than those of central venous catheterization, and it costs much more expense than simple venous catheter. There has been a lot of papers which argue that central venous catheterization may substitute for the Swan-Ganz catheterization. In addition, it may be very difficult or impossible to insert the Swan-Ganz catheter in pediatric patients. This study was performed to determine whether Swan-Ganz catheterization might be replaced by the central venous catheterization in regards to SO2. METHODS: In 17 pediatric open heart surgery (OHS) patients, from the central venous catheter, the tip of which had been located in the center of right atrium (RA) and pulmonary artery, blood samples were drawn for gas analysis simultaneously, which was done immediately after emergence from cardiopulmonary bypass. RESULTS: There were no significant differences between RA blood and mixed venous blood gas analyses except oxygen saturation. The oxygen saturations of both were linearly correlated with each other. The relation was SO2=17 0.8 SRAO2 (R=0.77, p<0.05) CONCLUSIONS: It may be concluded that RA blood may be used for blood gas analysis in place of mixed venous blood immediately after pediatric open heart surgery.
Blood Gas Analysis ; Cardiopulmonary Bypass* ; Catheterization, Central Venous ; Catheterization, Swan-Ganz ; Catheters ; Central Venous Catheters ; Heart Atria ; Heart* ; Humans ; Oxygen* ; Pulmonary Artery ; Thermodilution ; Thoracic Surgery*

No abstract available.
Child* ; Endoscopy* ; Gastrointestinal Diseases* ; Humans

No abstract available.
Humans*

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