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

BACKGROUND: Methemoglobinemia(MetHb) induced by dapsone overdose is not uncommon in Korea, especially in rural area. For treatment of dapsone-induced methemoglobinemia, methylene blue(MB) and activated charcoal(AC) should be used. To date, no reports have compared the amount of MB used between MB alone with MB & AC combined group(MB+AC). And also between moderate (MetHb<35%) and severe (MetHb>35%) intoxicated group defined by initial MetHb level. Authors hypothesized that less amount of MB can be used if MB and AC was used together and larger amount of MB is necessary to reduce MetHb level to asymptomatic level in severely intoxicated group. METHODS: From Jan 1990 to Dec 1996, a total of 54 patients who received treatment for dapsone intoxication were subject of study, The study was done retrospective chart analysis for initial MetHb level, total amount of MB and AC. Wilcoxon rank sum test and Chi-sqiare test was used to compare the total dosage of MB used for each group. Linear regression analysis was used between initial MetHb and the total amount of MB. Results were considered statistically significant when p<0.05. RESULTS: For MB alone and MB+AC group, the differences in total amount of MB used were statistically significant with mean dosage of 7.14+/-1.1mg/kg and 4.28+/-0.7mg/kg, respectively. And total amount of MB used between moderate and severe intoxicated group, the differences were statistically significant with mean dosage of 5.16+/-1.1mg/kg vs. 10.98+/-1.9mg/kg, respectively. There was significant correlation between initial MetHb level (X) and the amount of methylene blue (Y), Y=0.3X-2.42 (r2=0.41, p=0.0001) in MB alone, Y=0.186X-1.95(r2=0.21, p=0.034) in MB+AC respectively. CONCLUSION: For methemoglobinemia induced by dapsone, total amount of MB can be reduced especially in severe Intoxicated group if AC use was combined in treatment modality. There was significant correlationship between initial MetHb level and total amount of MB used.
Charcoal* ; Dapsone* ; Humans ; Korea ; Linear Models ; Methemoglobinemia ; Methylene Blue* ; Retrospective Studies

No Abstract Available.
Rosacea*

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

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

PURPOSE:Most but not all intrauterine growth retardation(IUGR) children has catch-up growth postnatally. However, nothing is known about the predictive parameters on the catch-up growth. The aim of this study was to describe serum IGF-I, free IGF-I, IGFBP-1, and IGFBP-3 levels in IUGR children and to correlate these hormone values with auxologic parameters to investigate their value on the postnatal growth pattern. METHODS:Among children with IUGR born at Ewha Womans University from Jan. 1995 to Aug. 1998, 16 children with IUGR at 3 years of age and 12 age-matched normal controls were studied for auxologic and biologic parameters. We measured height, weight, and serum levels of insulin-like growth factor(IGF)-I, free IGF-I, IGF binding protein(BP)-1, IGFBP-3 using immunoradiometric kits. RESULTS: 1)Among 16 children with IUGR at 3 years of age, 13 children had catch-up growth(81.3%), but 3 children remained short stature(18.7%). The height standard deviation score(SDS) in children with IUGR and control were -0.3+/-0.8 and 0.8+/-0.7, respectively(P<0.05) and weight SDS were -0.7+/-0.9 and 1.1+/-1.1, respectively(P>0.05). 2)Serum IGF-I levels in children with IUGR at 3 years of age and normal control were 90.9+/-35.4ng/mL and 68.4+/-24.4ng/mL, respectively(p>0.05) and free IGF-I were 0.9+/-0.5ng/mL and 0.6+/-0.3ng/mL(p>0.05), IGFBP-1 were 50.5+/-30.5ng/ mL and 52.3+/-23.2ng/mL(p>0.05), IGFBP-3 were 4,116.7+/-1,062.2ng/mL and 4,058.4+/-808.5ng/mL(p>0.05), respectively. 3)In children with IUGR at 3 years of age, height SDS in IUGR children with catch-up growth and those without catch-up growth were 0.002+/-0.6 and -1.5+/-0.7, respectively(P<0.001), but there were no differences in weight SDS, body mass index, IGF-I, free IGF-I, IGFBP-1 and IGFBP-3. 4)There were no significant correlations between height gain and any growth factors. CONCLUSION: The results show that there is no difference in the levels of IGF-I, free IGF-I, IGFBP-1 and IGFBP-3 in IUGR children at 3 years of age compared to age-matched normal control, suggesting that other factors rather than IGF-I, free IGF-I, IGFBP-1, IGFBP-3 may cause short stature in IUGR.
Body Mass Index ; Child* ; Female ; Fetal Growth Retardation* ; Humans ; Insulin-Like Growth Factor Binding Protein 1* ; Insulin-Like Growth Factor Binding Protein 3* ; Insulin-Like Growth Factor I* ; Intercellular Signaling Peptides and Proteins