PURPOSE: Growth hormone(GH) has not only growth promoting effect but also various metabolic effects. We evaluated GH effects by anthrometric data, biochmical data, electrolytes and simple CT in patients with Prader-Willi syndrome. METHODS: Nine children with Prader-Willi syndrome(PWS) were studied. The children were treated with GH(0.6U/kg/week) for 6 months. Before and after therapy we measured height, weight, waist, hip, and thigh. Blood sampling for eletrolytes, HgA1C, lipid profiles and other biochemistry were done in all patients before and after therapy. We also compared fat distribution with scan. RESULTS: Height standard deviation (SD) score increased from -0.7 to -0.5 and weight SD score decreased from 5.3 to 4.9. Body mass index(BMI) decreased from 28.2kg/m2 to 27.2kg/m2. But the changes in height, weight and BMI were not significant statistically. The waist/hip ratio decreased from 1.04 to 0.97(P<0.05), Thigh circumference had been decreased from 58.2+/-21.7cm to 49.9+/-6.9cm insignificantly. The visceral fat were decreased from 7,613+/-1,760 to 5,022+/-1,533 after GH therapy, and thigh muscle mass was increased from 6,358+/-1,616 to 7,175+/-2,155 (P<0.05). Total cholesterol and triglyceride decreased and HDL cholesterol increased after therapy although they were insignificant statistically. There were no differences in electrolytes, HgA1C, other biochemistry(Ca, P, protein, albumin, BUN, Cr) before and after therapy. CONCLUSION: In children with PWS, waist/hip ratio and fat mass were reduced and muscle mass was increased after GH therapy. There was tendency that total cholesterol and triglyceride decreased and HDL cholesterol increased after therapy. We confirmed that GH therapy had not only growth promoting effect but also metabolic effect on lipid and protein metabolism in children with PWS.
Biochemistry ; Child ; Cholesterol ; Cholesterol, HDL ; Electrolytes ; Growth Hormone* ; Hip ; Humans ; Intra-Abdominal Fat ; Metabolism ; Prader-Willi Syndrome* ; Thigh ; Triglycerides

PURPOSE: The purpose of this study is to compare general survival rate and survival rate according to expectable prognostic factors by analyzing the result of treating a patient of squamous cell carcinoma. MATERIALS AND METHODS: From Mar. 1999 to Feb. 2011, 151 patients were pathologically confirmed as squamous cell carcinoma of limbs and body in our hospital, and among those patients, 51 patients underwent the surgical treatment. This study included 41 patients who underwent the surgical treatment and were followed-up for more than 12 months. The mean age of population was 64.4 years. 31 males and 10 females were included. Wide excision with following skin grafts or flaps for reconstruction (29 cases) was mostly performed, but amputation (12 cases) was also performed for cases with extremities where resection margin was difficult to obtain and cases with neural or vascular invasion. 8 patients underwent chemotherapy or radiotherapy after resection, and 33 underwent the operation only. Stages were classified by AJCC Classification, survival rate was calculated by Kaplan-Meier method and survival rate of groups was compared by Log-rank test. For the expectable prognostic factors related to survival rate, location of primary lesion, cause of disease, pathologic grade, staging, surgical method, additional anticancer therapy were examined and each survival rate was compared. RESULTS: The average follow-up period was 65.2 (12-132) months. Thirty patients survived out of 41 patients till last follow up. The overall survival rate in 5 years was 77%. Three cases (7.3%) had local recurrence, and 7 cases (17.0%) had metastasis. The average period of recurrence from operation was 27 (18-43) months. Possible prognostic factors such as location of primary lesion, cause of disease, pathologic grade, staging, additional anticancer therapy showed no significant difference in survival rates. However, patients with amputation showed significantly lower survival rate than those with wide excision. CONCLUSION: In analysis the results of treating 41 cases of squamous cell carcinoma, the overall 5-year survival rate was 77%. And, among the several prognostic factors, only the surgical method was significant statistically.
Amputation ; Carcinoma, Squamous Cell ; Extremities ; Female ; Follow-Up Studies ; Humans ; Male ; Neoplasm Metastasis ; Recurrence ; Skin ; Survival Rate ; Transplants

PURPOSE:Growth retardation is a serious clinical problem in children with chronic renal failure(CRF). Dialysis and renal transplantation do not provide an improvement in growth velocity. Possible causes of growth retardation are nutritional deficiency, electrolyte imbalance, uremia, renal asteodystrophy and chronic anemia. However, catch-up growth cannot be achieved after correcting these factors. There is no concordance about disturbances of growth hormone(GH)-insulin-like growth factor-I (IGF-I) axis. in CRF. This study was designed to evaluate the growth status, IGF-I, GH and the effect of GH in CRF. METHODS:Twelve children with CRF(five were treated conservative, seven were transplanted) were included. IGF-I, stimulated GH, 24 hour integrated concentration of GH (IC-GH)were measured. Six were given rhGH(0.1U/kg/day) for average one year. RESULTS: 1)Growth velocity(GV) was 3.2+/-0.8cm/yr(conservative therapy:3.3+/-0.7, transplanted:2.9+/-0.8). Height standard deveation score(SDS) was -2.4+/-1.3cm/yr(conservative therapy group:-3.3+/-1.4, transplanted group:-1.3+/-0.4). Bone age lagged 2.1+/-13yr behind chronological age. 2) IGF-I concentrations were normal. 3)Stimulated GH levels were normal(16.6+/-3.3ng/ml) except one patient. Twenty- four hour IC-GH were less than 3.2ng/ml in 4 patients. 4)After GH therapy, GV increased 3.3+/-0.7cm/yr to 5.4+/-0.8cm/yr and Ht SDS increased -3.3+/-1.4 to -2.9+/-1.5 in the conservatively treated group. GV increased 2.9+/-0.8cm/yr to 5.5+/-1.8cm/yr and Ht SDS increased -1.3+/-0.4 to -0.8+/-0.5 in the transplanted group. CONCLUSION: Stimulated GH was normal but spontaneous secretion of GH was decreased in some patients with CRF. This neurosecretory dysfunction may be one causative factor in CRF. For these patients GH replacement therapy will be effective in promoting growth.
Anemia ; Axis, Cervical Vertebra ; Child* ; Dialysis ; Growth Hormone* ; Humans ; Insulin-Like Growth Factor I ; Kidney Failure, Chronic* ; Kidney Transplantation ; Malnutrition ; Uremia

No abstract available.
Diabetes Insipidus*

No abstract available.
Growth Hormone*

A 12-year-old girl presented with a 1-week duration of hair loss associated with splitting of the hair ends and whitish dots on the occipital hairs. On microscopic examination, a longitudinal splitting of the hair shaft with reconstitution of the normal hair distal to the fracture, nodular swellings, with the appearance of broomsticks pushed into one another, at the site of whitish swellings, and the flattening and twisting of the hair shaft around the long axis were demonstrated. Minor trauma to injury-prone hair is a common cause of hair shaft defects, however the reports with the combined conditions are insufficient in the literature. We describe a patient with central trichoptilosis associated with localized trichorrhexis nodosa and pili torti.
Axis, Cervical Vertebra ; Child ; Female ; Hair ; Humans

A case of lupus vulgaris who has the typical skin lesion on the patella region of the right knee was presented. The patient is 9 years old, the generally healthy male, and is no special finding of chest X-ray but tuberculin test was strong positive. The skin lesion started about 2 years ago as baby's palm sized erythematous patch with apple jelly nodule on the right knee joint region. Diagnosis was made by characteristic of clinical appearance and histulagial finding including other laboratory data. The treatment was done with INAH, PAS, streptomycin and pyridoxine for 3 months. Now,The skin lesion is almost healed and still continue treatment and observation go on.
Child ; Diagnosis ; Humans ; Knee Joint* ; Knee* ; Lupus Vulgaris* ; Male ; Patella* ; Pyridoxine ; Skin ; Streptomycin ; Thorax ; Tuberculin Test

No abstract available.
Child* ; Diabetes Mellitus* ; Humans ; Insulin* ; Sex Hormone-Binding Globulin*

Erdheim Chester disease (ECD) is very rare non-Langerhans cell histiocytosis (LCH) which occurs in the skeletal system and multiple organs. As it is progressive, sometimes it causes fatal results. However, it is often misdiagnosed as LCH or multiple bone metastasis and, thus, is very difficult to diagnose. In Korea, only 10 cases were first reported in 1999. In particular, there have been a few orthopedic approaches or reports in English-speaking literatures, and no report has been issued in Korea. The authors performed bone biopsy in patients with knee and lower extremity pain who were referred for the integrated treatment. We attempts to report this diagnosis experience with literature review.
Biopsy ; Erdheim-Chester Disease ; Histiocytosis ; Histiocytosis, Langerhans-Cell ; Humans ; Knee ; Korea ; Lower Extremity ; Neoplasm Metastasis ; Orthopedics

Congenital hypothyroidism is one of the most common endocrine disease in childhood and it causes not only mental retardation but also growth retardation. There were many papers about evaluation of developmental outcome in congenital hypothyroidism. The aim of this study was to evaluate growth outcome in congenital hypothyroidism. We evaluated 65 patients with congenital hypothyroidism diagnosed at Yonsei University College of Medicine. The results were summerized as follows; 1) The Male to female ratio was 1:1.4 2) Among the 65 patients, under 1 year of age with 16 cases (24.6%), 1~4 years with 19 cases (29.2%), 5~10 years with 22 cases (33.8%), above 10 years with 8 cases (12.3%). 3) Among the 65 patients, 30 cases (46%) had ectopic thyroid, 18 cases (28%) had dyshormonogeneses, 10 cases (15%) had hypoplasia and 7 cases (11%) had aplasia. 4) At the initial diagnosis, all the patients showed decreased T3, T4 and increased TSH level. 5) Before treatment, bone age and height age were delayed but they were normalized after treatment. 6) There was correlation between age of initial treatment and current height percentile. 7) There was significant correlation between initial T3 level and height age delay. 8) In the 4 cases who were diagnosed and treated before the age of 4, final adult height would be achieved within normal range. In conclusion, delayed growth due to congenital hypothyroidism would be prevented by early diagnosis and treatment. Therefore, the recognition of the importance of early diagnosis and treatment is emphasized.
Adult ; Congenital Hypothyroidism* ; Diagnosis ; Early Diagnosis ; Endocrine System Diseases ; Female ; Humans ; Intellectual Disability ; Male ; Reference Values ; Thyroid Dysgenesis