No abstract available.
Arthroplasty ; Femoral Fractures ; Hip

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

Hurthle cell tumors are an infrequent neoplasm of the thyroid gland in adults. Hurthle cell tumors represent 4. 5% to 10% of all primary thyroid epithelial neoplasms in the foreign literature. It has been known as Hurthle cell tumor since Ewing was the first to use this term in 1928. Tumor occurring in an infant was described by Symmers(1941) and Morrow(1945). The authors experienced a case of congenital Hurthle cell tumor of the thyroid gland in a 2 months old boy. He was admitted to the pediatric surgical department because of a growing mass in the neck since birth. Ultrasonogram showed a huge lobulated homogenous solid mass with medium level echogenicity in the region of the thyroid gland. Subtotal thyroidectomy of right lobe and total thyroidectomy of left lobe were done. The specimen measures 2x3x1.5 cm and 7x3x3 cm, respectively. It was an encapsulated, yellow gray firm and solid mass. The cut surface was smooth, glistening and homogenous. Microscopicully, the tumor was composed of tightly packed regular follicles lined by polyhedral, cuboidal, large cells with a granular acidophilic cytoplasms. The nuclei are vesicular, usually only a little bit larger than those of normal thyroid cells.
Infant ; Adult ; Male ; Female ; Humans

Many researchers report that the measurement of urine calcium concentration during antenatal period is helpful in prediction of having preeclampsia,but some don`t. The purpose of this clinical study is to evaluate the relationship of low calcium level in urine and the develpoment of preeclampsia, and effects of other variables. 24hour urine were collected and measured for the concentration of calcium and creatinine from 769 pregnant women who visited our department of obstetrics at Young-Dong Severeance hospital, Yonsei university medical college for antenatal care from March 1, 1993 to May 31, 1996. One group of 41 women developed preeclampsia and the other didn`t. There were no diffrences in ages and gestational age at urine collection between two groups, but a group with preeclampsia had an increased mean arterial blood pressure. There were signiificant difference in calcium concentration in urine and ratios of concentration of calcium and creatinine from urine collected between gestational ages of 25 weeks and 32 weeks and also in those women younger than 25 years old and their first pregnancy. Our study suggests that the measure ment of calcium level and ratios of urine calcium concentration and creatinine concentration at gestational ages between 25 weeks and 32 weeks in women younger than 25 year old with their first pregnancy is quite helpful inpredicting the develpoment of preeclampsia.
Adult ; Arterial Pressure ; Calcium ; Creatinine ; Female ; Gestational Age ; Humans ; Obstetrics ; Pre-Eclampsia* ; Pregnancy ; Pregnant Women ; Urine Specimen Collection ; Weights and Measures

The stricture of the calyx, pelvis or ureter due to renal tuberculosis had been managed like nephrectomy, partial nephrectomy, ureteroileoneocystoplasty or even pancaliceal-ileoneocystoplasty. To salvage the renal parenchyme and to treat the tuberculous renal caliceal strictures, percutaneous endocalicotomy with or without endopyelotomy was performed in 10 cases from Aug. `90 to Jan. `92. The sites of stricture were mainly upper calyx in 6 cases and lower in 4. A cold knife was used to incise the stricture and a stenting 2-sectioned(14 Fr.) endopyelotomy catheter was retained for 6 to 8 weeks. Postoperative intravenous pyelography revealed marked shrinkage of the dilated calyx in 7 cases. moderate in 1 and no change in 2(success rate, 80%). In failed 2 cases, 1 cases was performed partial nephrectomy and the other was following. There was no significant complications except 1 case of upward migration of D-J catheter. In conclusion, endocalicotomy is safe, less invasive, successful (in cases that guide wire could pass) and parenchyme preserving procedure. The retregrade pyelography is mandatory just before the surgery because stricture can be progressed during Anti-Tbc chemotherapy.
Catheters ; Constriction, Pathologic* ; Drug Therapy ; Nephrectomy ; Pelvis ; Stents ; Tuberculosis, Renal ; Ureter ; Urography

A rare case in the pattern of the origin of the celiac artery was observed in the cadaver of a 64 year old Korean male and the results are summarized as follows. The superior mesenteric artery originated from the abdominal aorta at the level of the intervertebral dusk between first and second lumbar vertebra. At a point 4cm from the origin of the superior mesenteric arterr, a common trunk appeared and ran 1cm and then divided into the common hepatic artery and the splenic artery. The common hepatic artery divided into the right gastric artery and the proper hepatic artery at a point 4.5cm after the bifurcation. A common trunk formed by the left inferior phrenic artery and the lefts gastric artery arose from the abdominal aorta about 0.8cm about the origin of the celiacomesenteric trunk.
Aorta, Abdominal ; Arteries ; Cadaver ; Celiac Artery* ; Hepatic Artery ; Humans ; Male ; Mesenteric Artery, Superior ; Spine ; Splenic Artery

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

No abstract available.
Female ; Obstetric Labor, Premature* ; Pregnancy ; Ritodrine*

The antiphospholipid antibody syndrome is an acquired multisystemic disorder characterized by persistent elevated antiphospholipid antibodies and/or hypercoagulation in veins or arteries, or both. The clinical manifestations of the antiphospholipid antibody syodrome are recurrent thrombosis, fetal loss, thrcenbocytopenia, and various cutaneous lesions. Skin lesions are the first sign of this syndrome in 41% of patients and systemic thrombosis develops in 40% of them. Livedo reticularis is the most common cutaneous finding of the antiphosphotipid antibody syndrome. Although vasculitis has not been frequently noted in antiphospholipid antibody syndrome, some vasculitis such as polyarteritis nodosa, giant cell arteritis, and other nonspecific vasculitides have been found in association with antiphospholipid antibody syndrome. We present a male patient with typical manifestations of leukocytoclastic vasculitis with deep vein thrombosis and positive antiphospholipid antibodies. It suggests that a case of antiphospholipid antibody syndorme was accompanied with cutaneous leukocytoclastic vasculitis.
Antibodies, Antiphospholipid* ; Antiphospholipid Syndrome ; Arteries ; Giant Cell Arteritis ; Humans ; Livedo Reticularis ; Male ; Polyarteritis Nodosa ; Skin ; Thrombosis ; Vasculitis* ; Vasculitis, Leukocytoclastic, Cutaneous ; Veins ; Venous Thrombosis