Binder Syndrome Infant Born from Mother with Cholelithiasis / 대한소아내분비학회지

Binder syndrome is a maxillonasal dysostosis characterized by midface and nasal hypoplasia. It is sometimes associated with short terminal phalanges of fingers and toes and transient radiological features of chondrodysplasia punctata. It is associated with vitamin K deficiency during pregnancy. We describe here a baby with Binder syndrome who was born from mother with cholelithiasis during pregnancy.

Sex Differences in Body Composition in Prepubertal Korean Children by Dual-energy X-ray Absorptiometry / 대한소아내분비학회지

PURPOSE: To find sexual dimorphism of total body composition (BC) and to establish regression equations for each BC by anthropometric data in prepubertal children. METHODS: Cross-sectional study of 118 healthy children (58 boys, 60 girls, age; 5.1-9.9y) was done. We examined total BC (bone mineral contents, BMC; lean tissue mass, LTM; and fat mass, FM) and regional BC by dual-energy x-ray absorptiometry (DXA). We evaluated the differences in BC between both sexes by independent T-test and performed regression analysis for each BC using SPSS ver 15.0. RESULTS: In total BC, boys had more LTM than girls (P<0.01), while girls had more FM (P=0.05). The percent body fat (PBF) of girls was higher than that of boys (15.0+/-5.4 vs. 11.6+/-5.2, P<0.01). In regional BC, boys had more LTM than girls except arms region. Girls had greater leg and gynoid FM than boys. As a result, girls showed more PBF in all regions. When we did multiple regression analysis including age and anthropometric values, height (Ht) and weight (Wt) were related to BMC and LTM. The regression equations were as follows. For boys: BMC (g)=12.2xHt (cm)+16.1xWt (kg)- 1,025, LTM (kg)= 0.21xHt+0.35xWt-14.3. For girls: BMC (g)=8.0xHt+21.1xWt-881, LTM (kg)=0.18xHt+0.31xWt-10.7. CONCLUSION: We identified sex differences in total BC and regional fat distribution and made regression equations for each BC in prepubertal Korean children.

Pamidronate Treatment in Vertebral Fracture after Off-therapy of Medulloblastoma / 대한소아내분비학회지

A twenty-one year old man, who had been treated for medulloblastoma 6 years ago, complained progressive right knee joints pain and back pain. Multiple thoracic vertebral compression fracture was detected in radiography. The bone mineral density(BMD) was in osteoporotic range in duel energy X-ray absorpometry. Growth hormone deficiency and primary hypothyroidism were detected after endocrine function test. We treated him by cyclic intravenous pamidronate because he had complained severe back pain and difficulty to walk. After pamidronate therapy, the pain was decreased and the BMD was increased. No severe adverse effect was noted. This is the first case report of pamidronate therapy in patient who had osteoporotic vertebral compression fracture due to a sequela of childhood medulloblastoma.

Pachydermoperiostosis in a 19 Year-old Boy Presenting as an Acromegaly-like Syndrome / 대한소아내분비학회지

A nineteen year-old boy with progressive enlargement of the joints and distal extremities, clubbing, coarse facial features and hyperhidrosis was investigated. In physical examination, thickening of the scalp with furrowing (cutis verticis gyrata) and greasy thickening of skin (pachyderma) was prominent. His endocrine profile was normal. Radiological studies demonstrated bilateral symmetrical periosteal new bone formation with acroosteolysis and incidental microadenoma of pituitay gland. After extensive investigation to exclude systemic and endocrine causes, the patient was diagnosed as pachydermoperiostosis (PDP). PDP is a rare syndrome manifested clinically by finger clubbing, extremity enlargement, hypertrophic skin changes, and periosteal bone formation. The pathogenesis of the disorder has not been clarified though few endocrine abnormalities were seen. To aware of these clinical phenotype would help to differentiate PDP from acromegaly.