Reference values of bone mineral density of proximal femur for Southeast Asian children and adolescents.

Bone mineral density (BMD) of the proximal femur helps evaluate bone density in children with reduced mechanical loading of the lower extremities. This study provides the first reference values of bone mineral density of proximal femur according to age and sex for Southeast Asian children and adolescents.   OBJECTIVES: The study aimed to (1) establish normative data of BMD of the proximal femur (femoral neck and total hip), measured by dual-energy X-ray absorptiometry (DXA), for healthy Thai children aged 5 to 18 years and (2) ascertain the relationships between BMD, growth, and puberty. METHODS: Proximal femur scans of 170 boys and 191 girls obtained from DXA (Lunar Prodigy Pro, GE, and software enCORE version 7.53) were un-analyzed and then re-analyzed with the upgraded software enCORE version 17 SP2 for BMD assessment. The bone mineral apparent density of the femoral neck (FNBMAD) was calculated. RESULTS: Sex and Tanner stage-specific BMD normative data were generated. BMD values of the femoral neck and total hip increased with age and pubertal progression. FNBMAD values were not markedly influenced by age and puberty. Using multiple linear regression analysis, age and weight affected FNBMD and total hip BMD in boys and girls, but height and Tanner stage only influenced girls. Age did not significantly influence FNBMAD in either sex. Tanner stage weakly influenced FNBMAD only in boys. CONCLUSIONS: We established normative reference data for BMD of the proximal femur measured by DXA in Thai children aged 5 to 18 years. Our reference data will help clinicians and researchers assess and interpret the BMD of the proximal femur for Southeast Asian children.

Bone mineral density in primarily preadolescent children with hemoglobin E/ß-thalassemia with different severities and transfusion requirements.

BACKGROUND: Children with ß-thalassemia major and ß-thalassemia intermedia frequently have low bone mass. However, studies of bone mineral density (BMD) in children with transfusion-dependent (TD) or non-transfusion-dependent (NTD) hemoglobin (Hb) E/ß-thalassemia are scarce. OBJECTIVES: To determine the prevalence of low bone mass among mostly preadolescent children with NTD and TD Hb E/ß thalassemia and the related factors. METHODS: We investigated the BMD of the lumbar spine (LSBMD) and total body (TBBMD), measured by dual-energy X-ray absorptiometry, of 59 children with NTD Hb E/ß-thalassemia and 50 with TD Hb E/ß-thalassemia. RESULTS: The median age of the patients was 10.4 (6.2-13.5) years in the NTD group and 10.3 (5.9-14.1) years in the TD group. These children had a relatively low prevalence of low bone mass (NTD: 1.7%-10.2%; TD: 4%-14%). The values varied with the bone site measured and the BMD size-adjustment method used (height age vs. bone age). The NTD group had significantly lower TBBMD Z-scores (adjusted for height age) than the TD group. The proportion of patients with low lumbar spine bone mass (adjusted for bone age) was significantly higher for the TD group than for the NTD group. CONCLUSIONS: Our study demonstrates that most children with either disease had normal BMD. Patients with the NTD form had a lower TBBMD than those with the TD form. Low bone mass affected the lumbar spine of patients with TD Hb E/ß-thalassemia more than those with the NTD form.

A novel mutation in the beta-subunit of the epithelial sodium channel gene (SCNN1B) in a Thai family with Liddle's syndrome.

Liddle's syndrome is a rare form of autosomal dominant hypertension with early penetrance and cardiovascular sequelae. It is caused by missense or frameshift mutations in the epithelial sodium channel (ENaC) gene resulting in excessive salt and water resorption from the distal nephron, volume expansion, and suppression of plasma renin activity and serum aldosterone secretion. Treatment with an antagonist of the amiloride-sensitive ENaC, amiloride or triamterine, can correct hypertension and biochemical abnormalities in Liddle's syndrome by closing the sodium channels. Missense and truncation mutations at the C-terminus of the ENaC gene have been found in two of the three genes encoding beta- and gamma-subunits of ENaC. We report here a Thai family with Liddle's syndrome caused by a novel P615H missense mutation in the proline-rich domain of the SCNN1B gene coding for the beta-subunit of ENaC. This mutation occurs within the conserved proline-rich (PY) motif at the C-terminal end and emphasizes the critical role of this motif in ENaC internalization. The presence of severe hypertension and/or a suggestive family history of hypertension with or without hypokalemia in young children should always raise a suspicion of Liddle's syndrome.

Glycemic control and the psychosocial benefits gained by patients with type 1 diabetes mellitus attending the diabetes camp.

OBJECTIVE: The aim of this study was to evaluate the effectiveness of diabetes camp on glycemic control, knowledge, and psychosocial benefits among patients with type 1 diabetes (T1D). Glycemic control among patients with infrequent and frequent self-monitoring of blood glucose (SMBG) was also compared. METHODS: During a 5-day camp, 60 patients were taught diabetes self-management education (DSME). After camp, patients were divided into two groups based on frequency of SMBG (<3 versus 3-4 times/day) and were followed up until 6-month post-camp. Patients' HbA1c levels and knowledge were assessed at baseline, 3- and 6-month post-camp. Patients' impressions towards camp were assessed. RESULTS: In both SMBG groups, HbA1c levels decreased significantly at 3-month post-camp but did not sustain at 6-month monitoring. The patients with frequent SMBG had a lower mean HbA1c level. A significant improvement in knowledge was noted and sustained up to 6-month post-camp. The patients found diabetes camp of benefit and felt they could better cope with diabetes. CONCLUSIONS: Although the effect of the diabetes camp on glycemic control was short-lived, an improvement in knowledge and a better attitude towards having diabetes were seen among participants. PRACTICE IMPLICATIONS: The psychosocial benefits and knowledge gained by patients attending diabetes camp underline the importance of including a camp in a diabetes management plan. To improve patients' long-term glycemic control, a continuous education is required.

Etiology and glycemic control among Thai children and adolescents with diabetes mellitus.

BACKGROUND: There is little data on etiology and metabolic control in children and adolescents with diabetes in developing countries. OBJECTIVE: Determine the etiology of diabetes in Thai youths and to evaluate their glycemic control. MATERIAL AND METHOD: The authors retrospectively reviewed the case records of 157 patients seen at the Department of Pediatrics, Siriraj Hospital between 2003 and 2004. RESULT: Type 1 diabetes (T1D) comprised 83%, type 2 diabetes (T2D) 13%, and other types 4%. GAD65 and IA-2 antibodies were positive in 63% and 61% of T1D patients, and 0% and 9% of T2D patients, respectively. There were an increasing number of new cases, both T1D and T2D, during the study period compared with a previous study conducted at the hospital. Mean HbA1c in T1D and T2D were 8.9 +/- 2.1% and 6.2 +/- 1.80%, respectively (p < 0.001). CONCLUSION: Based on the present study, glycemic control appeared to be more satisfactory in T2D patients than those with T1D. Glycemic control among T1D patients was comparable to others in developed countries.