Clinical characteristics and growth pattern were evaluated in 42 patients with classical congenital adrenal hyperplasia treated since diagnosis. And final height was evaluated in 16 patients who had reached final adult height with regard to clincal form, degree of hormonal control, and age of initial treatment. The results were as follows; 1) Among 42 patients with classical congenital adrenal hyperplasia, the male to female sex ratio was 1:1.8 (15 males and 27 females), and the frequency of the salt-wasting form (SW) and simple virilizing form (SV) was same (21 SW and 21 SV). 2) The length of time to initial treatment averated 4.2 months (range: 9 days-49 months) for salt-wasting form and 8.8 years (range: 1 month-21 years) for simple virilizing form. 3) In seven cases, precocious puberty developed during treatment and the average age on development was 6.8 years for salt-wasting form and 5.4 years for simple virilzing form. 4) The pattern of growth from birth throughout childhood in salt-wasting patients showed poor growth initially. The average height in simple virilizing patients was above the mean in the first years of life, declining steadily (falling below the mean by age 12), until the end of the growt period. 5) Bone ages were retarded initially in salt-wasting patients, thereafter accelerating by age 3, meanwhile in simple virilizing patients advanced initially. 6) In 16 patients who had reached final adult height, analysis of final height with regard to clinical form, degree of hormonal control, and age of initial treatment indicated that neither of them seem to after the height outcome. In conclusion, classical congenital adrenal hyperplasia patients seem to achieve final height consistently below the mean for the general population. Factors to affect the height outcome (clinical form, degree of hormonal control, age of initial treatment) may influence concomitently, and another factor may be present.
Adrenal Hyperplasia, Congenital* ; Adult ; Diagnosis ; Female ; Humans ; Male ; Parturition ; Puberty, Precocious ; Sex Ratio

In the past several years, total hip replacement arthroplasty has become an established procedure in the management of painful arthritic hips in older adults. It is still gaining an increasing popularity as experience has accumulated and indications broadened. The authors reviewed the first 47 total hip replacement arthroplasties in 41 patients performed at the Department of Orthopedic Surgery, Seoul National Univerity Hospital, during the period of 4 years and 8 months, from April 1972 to November 1976. The longest follow-up was 4 years and 8 months and the shortest 1 month, the average being 2 years and 1 month. The youngest age was 17 years and the oldest 71 years, the average being 41 years. There were 26 males and 15 females. The underlying etiology was as follows: primary osteoarthritis 4 hips, avascular necrosis 12 hips, septic hip residua 18 hips (pyogenic 11 hips, tuberculosis 7 hips), rheumatoid arthritis 3 hips, failed previous operation 3 hips (failed endoprosthesis 2 hips, failed total hip replacement 1 hip), secondary osteoarthritis 5 hips (congenital dislocation 4 hips, Legg-Perthes disease 1 hip), and nonunion of femoral neck 2 hips. Following types of prosthesis were used; Sbarbaro 3, Charnley 8 (including custom-made extralong, narrow stem and extra-small stem prostheses), Muller 22, and Trapezoidal-28 14. The results were evaluated by the methods of Harris and d'Aubigne, both preoperatively and postoperatively, In the average, Harris' score improved from 57 to 92 (gains of 35) and the d'Aubigne rating from 10.4 to 16.8 (gains of 6.2) following operations.
Adult ; Arthritis, Rheumatoid ; Arthroplasty ; Arthroplasty, Replacement, Hip ; Dislocations ; Female ; Femur Neck ; Follow-Up Studies ; Hip ; Humans ; Legg-Calve-Perthes Disease ; Male ; Necrosis ; Orthopedics ; Osteoarthritis ; Prostheses and Implants ; Seoul ; Tuberculosis

Total hip arthroplasty was performed on a 32 year old man with hemophilia A. A series of complications were encountered; namely, femoral shaft fracture during arthroplasty, hemolytic anemia, Factor VII inhibition, pseudotumor formation and wound dehiscence and sepsis as a result of shortage of cryoprecipitate supply, and general debilitation and septicemia from fhe use of immunosuppressive agents, finally leading to death 67 days after operation. Initially, 399 bottles of cryoprecipitate (each containing approximately 100 units of Factor VII) were anticipated. In practice, however, a total of 660 bottles and packs had been consumed on the patient when the supply was exhausted. Total hip arthroplasty can be carried out on hemophilia patients, provided that the planning be impeccable and the supply of antihemophilic factor virtually unlimited.
Anemia, Hemolytic ; Arthritis ; Arthroplasty ; Arthroplasty, Replacement, Hip ; Factor VII ; Hemophilia A ; Humans ; Immunosuppressive Agents ; Sepsis ; Wounds and Injuries

No abstract available.
Child* ; Humans ; Insulin-Like Growth Factor I* ; Plasma* ; Somatomedins*

No abstract available.
Cushing Syndrome*

Pupose:Recently childhood obesity is increasing and has been accompanied by an increase in the prevalence of type 2 diabetes mellitus(DM) among children and adolescents. We evaluated insulin resistance and impaired glucose tolerance(IGT) in 48 obese children and adolescents. METHODS:All subjects underwent a two-hour oral glucose tolerance test(1.75 g of glucose per kg of body weight) and then glucose and insulin levels were measured. Insulin resistance was estimated by homeostatic model assessment, and betacell function was estimated by calculating the ratio between the changes in the insulin level and glucose level during the first 30 minutes after the ingestion of glucose. RESULTS:IGT was detected in 4.2% of the 24 obese children(4-10 years of age) and 20.8% of the 24 obese adolescents(11 to 18 years of age). Type 2 DM was identified in 8.3% in children and 29.2% in adolescent obesity. Insulin levels were markedly elevated after the glucose tolerance test in subjects with IGT but a little decreased in cases with DM. Those changes of insulin levels were not significant statistically. The insulinogenic index was decreased in IGT and DM group of childhood and adolescent obesity compared to normal glucose tolerance(NGT) but it was not significant statistically. Insulin resistance, which was evaluated with fasting insulin, QUICKI and HOMA-IR, was increased in childhood obesity compared with adolescent obesity and the HOMA-IR of adolescent IGT group was significantly higher compared with NGT group(P<0.05). CONCLUSION: IGT is highly prevalent among children and adolescents with obesity. IGT was associated with insulin resistance and hyperinsulinemia, while beta-cell function was still relatively preserved. Overt type 2 diabetes was linked to insulin resistance and to beta cell failure.
Adolescent* ; Child* ; Eating ; Fasting ; Glucose Tolerance Test ; Glucose* ; Humans ; Hyperinsulinism ; Insulin Resistance* ; Insulin* ; Obesity* ; Pediatric Obesity ; Prevalence*

Pupose:Recently childhood obesity is increasing and has been accompanied by an increase in the prevalence of type 2 diabetes mellitus(DM) among children and adolescents. We evaluated insulin resistance and impaired glucose tolerance(IGT) in 48 obese children and adolescents. METHODS:All subjects underwent a two-hour oral glucose tolerance test(1.75 g of glucose per kg of body weight) and then glucose and insulin levels were measured. Insulin resistance was estimated by homeostatic model assessment, and betacell function was estimated by calculating the ratio between the changes in the insulin level and glucose level during the first 30 minutes after the ingestion of glucose. RESULTS:IGT was detected in 4.2% of the 24 obese children(4-10 years of age) and 20.8% of the 24 obese adolescents(11 to 18 years of age). Type 2 DM was identified in 8.3% in children and 29.2% in adolescent obesity. Insulin levels were markedly elevated after the glucose tolerance test in subjects with IGT but a little decreased in cases with DM. Those changes of insulin levels were not significant statistically. The insulinogenic index was decreased in IGT and DM group of childhood and adolescent obesity compared to normal glucose tolerance(NGT) but it was not significant statistically. Insulin resistance, which was evaluated with fasting insulin, QUICKI and HOMA-IR, was increased in childhood obesity compared with adolescent obesity and the HOMA-IR of adolescent IGT group was significantly higher compared with NGT group(P<0.05). CONCLUSION: IGT is highly prevalent among children and adolescents with obesity. IGT was associated with insulin resistance and hyperinsulinemia, while beta-cell function was still relatively preserved. Overt type 2 diabetes was linked to insulin resistance and to beta cell failure.
Adolescent* ; Child* ; Eating ; Fasting ; Glucose Tolerance Test ; Glucose* ; Humans ; Hyperinsulinism ; Insulin Resistance* ; Insulin* ; Obesity* ; Pediatric Obesity ; Prevalence*

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

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

Currarino triadis a unique malformation complex of congenital caudal anormalies, including anorectal malformatio (anal stenosis, anal ectopis, imperforated anus), sacral bony abnormality (scimitar or crescentic bony defect, malsegmentation) and presacral mass (meningocele, teratoma, enteric cyst or any combination of these). This triad is familial in at least half of cases and the usual symptomatology is constipation due to anorectal stenosis. The embryogenesis is presumably due to abnormal separation of the neuroectoderm from the endoderm, so this triad is in the spectrum of the split notochord syndrome. We report a case of Currarino triad in 5-month-old female patient who had chronic constipation and abdominal distention with brief review of the related literatures.
Constipation ; Constriction, Pathologic ; Embryonic Development ; Endoderm ; Female ; Humans ; Infant ; Neural Plate ; Notochord ; Pregnancy ; Teratoma