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  • Author: Suttipong Wacharasindhu x
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Suttipong Wacharasindhu, Vichit Supornsilchai, Suphab Aroonparkmongkol and Thaninee Sahakitrungrueng

Abstract

Background: Pubertal growth data in Thai children has been reported as cross-sectional studies. There is no longitudinal study in Thai children. Objective: Investigate the longitudinal growth data in normal Thai children including the relationship between age at pubertal onset and other growth parameters. Material and method: Eighty-eight normal children (44 boys, 44 girls) were longitudinally assessed for the growth and puberty until they reached their final adult height. Pubertal staging was assessed by the Tanner method. Results: Mean age of pubertal onset was 10.2 ± 1.2 years for girls and 12.2 ± 1.0 years for boys. Total pubertal height gain was 18.3 ± 4.0 cm for girls and 22.3 ± 4.4 cm for boys. Total pubertal height gain had a negative correlation with age at pubertal onset for girls, but not for boys. Conclusion: The onset of puberty was not much changed from previous studies. Girls with early puberty had a higher pubertal height gain. This might be due to a compensatory mechanism. These longitudinal growth data can be used as a reference in clinical practices for Thai children.

Open access

Suttipong Wacharasindhu, Suphab Aroonparkmongkol, Taninee Sahakitrungrueng, Vichit Supornsilchai and Chansuda Bongsebandhu-phubhakdi

Abstract

Background: Girls with sign of secondary sexual characteristic before the age of 8 years are defined as precocious puberty (PP). Early activation of hypothalamo-pituitary-gonadal axis causes central PP. Without treatment, some become short adult height and early menstruation may develop.

Objective: Report the final adult height in precocious puberty girls who have been treated with GnRH agonist.

Methods: Seventy girls with PP and early puberty (EP) who were treated with GnRH agonist have been followed up until they reach final height (FH). FH was compared with predicted adult height (PAH) before treatment and mid-parental height (MPH).

Results: All subjects were treated with GnRH agonist for a mean duration of 1.9 0.6 years. FH was significantly higher than PAH before treatment (156.6±5.1 vs. 151.6±6 cm, p <0.001). FH is positively correlated with MPH. However, treatment with GnRH agonist in EP girls and in PP girls with bone age of more than 12.5 years or those who already had menstruation may have less benefit.

Conclusion: GnRH agonist can enhance FH in girls with PP. Early treatment in those with bone age of less than 12.5 years or before menstruation may result in good outcome.

Open access

Suttipong Wacharasindhu, Ouypuen Panamonta, Lucy Shapiro, Louise A. Metherell, Martin O. Savage and Helen L. Storr

Abstract

Background

Growth hormone insensitivity (GHI) or Laron syndrome can result from GH receptor (GHR) or postreceptor defects, such as in GH binding or transduction, or insulin-like growth factor 1 (IGF-1) synthesis. Multiple defects in GHI have been reported in cohorts from the Middle East, Ecuador, and the Mediterranean, but rarely reported from Southeast Asia.

Methods

Genomic DNA was isolated from peripheral blood leukocytes of young Thai sisters with severe short stature. Coding exons, including the intronic boundaries of the GHR were amplified from genomic DNA by PCR, and products were purified and sequenced. Serum GH, IGF-1, and IGF binding protein-3 were assayed immunometrically.

Results

We found an extreme GHI phenotype and a homozygous mutation in exon 7 of GHR.

Conclusions

This mutation can cause a new donor splice site and interfere with mRNA splicing. To our knowledge, these are first cases of Laron syndrome in Thais confirmed by genotyping.

Open access

Vichit Supornsilchai, Yodporn Hiranras, Chansuda Bongsebandhu-phubhakdi and Suttipong Wacharasindhu

Abstract

Background: Klinefelter syndrome may present as precocious puberty, which can be either central precocious puberty or peripheral precocious puberty, caused by an extragonadal germ cell tumor.

Objective: Report two cases of Klinefelter syndrome that presented with precocious puberty due to a β-hCG producing mediastinal tumor.

Method: Review of the clinical history, physical examination, and laboratory investigations.

Results: Pseudo-precocity developed some years before diagnosis of -hCG producing tumor. The patients did not have typical physical features of this syndrome. The testes were small and had loose consistency.

Conclusion: Klinefelter syndrome must be excluded in all boys presenting with precocious puberty due to a β-hCG producing tumor. Conversely, patients with Klinefelter syndrome should be regularly checked for β-hCG and α-fetoprotein levels. In those cases, the patients can be diagnosed and treated early. With the early treatment, they will be able to attain normal adult height and have fewer complications from the tumor.

Open access

Termpong Dumrisilp, Vichit Supornsilchai, Suttipong Wacharasindhu, Suphab Aroonparkmongkol and Taninee Sahakitrungruang

Abstract

Background

Children and adolescents with type 1 diabetes mellitus (T1D), even those with intensive insulin treatment regimens, often have higher glycated hemoglobin (HbA1c) levels than adults.

Objective

To delineate the medical and psychosocial factors associated with glycemic control in an unselected pediatric population with T1D.

Methods

We included a cross-section of 58 adolescents (28 boys and 30 girls) aged 13.6 ± 4.0 years with T1D ≥1 year attending a well-established pediatric diabetes clinic in Thailand. Median diabetes duration was 4.1 years (range 1–18 years). Participants were divided into 2 subgroups according to their average HbA1c level over the past year. Those with good control (HbA1c <8%) (n = 13) were compared with those with poor control (HbA1c ≥8%) (n = 45). Data collected from self-report standardized questionnaires and medical records were used to compare variables between groups.

Results

Adolescents with good control used significantly less daily insulin and had higher family income, higher scores for family support, and quality of life (QoL) than those in the group with poor control (P < 0.05). Age, sex, puberty, duration of diabetes, insulin regimen, frequency of blood glucose monitoring, and self-report adherence did not differ between groups. By univariate logistic regression, the only factor associated significantly with poor glycemic control was a QoL score <25.

Conclusion

Adolescents with T1D may be at a higher risk of poor glycemic control if they have poor QoL, impaired family functioning, poor coping skills, and lower socioeconomic status, suggesting that psychosocial interventions could potentially improve glycemic control in this population.

Open access

Sukumarn Siripunthana, Taninee Sahakitrungruang, Suttipong Wacharasindhu, Darintr Sosothikul and Vichit Supornsilchai

Abstract

Background

Regular blood transfusion and iron chelation therapy have improved the quality of life of patients with thalassemia and increased their longevity, but transfusion also increases the frequency of endocrine complications, possibly because of iron deposition in the pituitary gland or the gonads, or both.

Objective

To evaluate testicular function in patients with thalassemia major by basal hormonal study, and identify risk factors for dysfunction.

Methods

We performed a cross-sectional study of 28 patients with thalassemia major aged 11.7 ± 1.8 (8–14.9) years (15 in prepuberty, 13 in puberty with no delayed puberty) who had regular blood transfusions. A normal control group comprised 64 boys who were matched for age and Tanner genital stage.

Results

The mean level of serum ferritin in the previous year was 1,575 ± 642 ng/mL, and the onset of blood transfusion was at 3.8 ± 2.3 years and iron chelation therapy was 6.6 ± 2.8 years. The trend for anti-Müllerian hormone levels in patients and controls was similar with age, and although higher in the patients, particularly at Tanner stage II, was not significantly different. Testosterone levels were lower in the patients compared with controls; particularly at Tanner stages IV–V (290.88 vs. 537.4 ng/dL, P < 0.05). Serum follicle-stimulating hormone and luteinizing hormone levels were not significantly different between the groups at any Tanner stage.

Conclusion

Patients who received regular blood transfusions had normal Sertoli cell function. Leydig cell dysfunction may occur, even though the patients had a normal pubertal onset.

Open access

Sirawut Trepatchayakorn, Vichit Supornsilchai, Suttipong Wacharasindhu, Suphab Aroonparkmongkol and Taninee Sahakitrungruang

Abstract

Background: Reports on characteristics of pediatric diabetes in children from Southeast Asian countries are limited.

Objectives: To describe the clinical characteristics, prevalence, glycemic control, and current treatment regimens of diabetes in Thai children.

Materials and Methods: Data from 132 patients seen at our pediatric diabetes clinic at Chulalongkorn University during 2001−2013 were retrospectively reviewed.

Results: We found an increasing number of patients newly-diagnosed with type 1- (T1DM) or type 2- diabetes mellitus (T2DM). The overall proportion of T1DM was 69.7%, T2DM 23.4%, and other types 6.9%. Children with T1DM were younger at diagnosis, had higher initial glucose and glycated hemoglobin A1c (HbA1c), a lower body mass index z-score, lower C-peptide and insulin levels, and were more likely to have classic diabetes symptoms and ketoacidosis, compared with children with T2DM. Mixed diabetes phenotypes were found in about 12%−14% of these children. Glutamic acid decarboxylase and islet antigen-2 autoantibodies were found in 70% and 54% of T1DM patients, respectively, and not in T2DM patients. HbA1c in T1DM was 9.6 ± 2.2% total hemoglobin, and in T2DM was 7.9 ± 2.6%. There were no differences in HbA1c levels between different insulin regimens in the T1DM group.

Conclusion: The number of children with T1DM or T2DM has been increasing and there are overlapping phenotypes in a significant proportion of these children. Correct diagnosis requires clinical evaluation and monitoring of the clinical course. Further research is needed to determine the risk factors for the poor glycemic control found in children with T1DM.