medigraphic.com

2020, Number 3

<< Back Next >>

Rev Cuba Endoc 2020; 31 (3)

Bone mass and steroid treatment in patients with congenital adrenal hyperplasia

Espinosa RTM, Leyva GG, Domínguez AE

Language: Spanish
References: 18
Page: 1-9
PDF size: 352.80 Kb.


ABSTRACT

Introduction: Glucocorticoid replacement therapy is still the treatment´s paradigm in the classic forms of congenital adrenal hyperplasia. Its effects on bone mineralization are not entirely clear.
Objective: Describe bone mass-related variables in congenital adrenal hyperplasia patients receiving substitute steroid treatment.
Method: A cross-sectional descriptive study was conducted exploring clinical, biochemical, hormonal and bone mineralization variables in 25 patients with congenital adrenal hyperplasia caused by 21OHase deficiency and steroid treatments.
Results: 21 women (84.0%); the largest group was of adolescents between the age of 10 and 19 years (52%).Classical forms predominated with 22 patients (88.0%), including 13 of them (52%) that were salt losers, 9 simple virilizers (36.0%) and only 3 (12.0%) of non-classical forms. The most commonly used steroid was hydrocortisone in 16 patients (64%), at an average dose of 22.10±12.00 mg daily, corresponding to 17.09±5.71 mg/m2sc/day and on average carried 14.02±6.57 years of substitute therapy. No alterations in the phosphocalcic metabolism were detected. Density and bone mineral content in the spinal column and femur showed higher values in non-classical forms of the disease, followed by simple virilizing and finally the salt loser patients, in none of the cases with statistical significance.
Conclusions: Patients with congenital adrenal hyperplasia in this study showed mostly preserved bone mass.


REFERENCES

  1. Speiser PW, Arlt W, Richard J. Auchus RJ, Baskin SL, Conway SG, et al. An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2018;103(11):4043-88.

  2. Canalis E, Bilezikian JP, Angeli A, Giustina A. Perspectives on glucocorticoid- induced osteoporosis. Bone. 2004;24:593-8.

  3. Canalis E. Mechanisms of glucocorticoid action in bone. Curr Osteoporos Rep. 2015;3:98-102.

  4. Riehl G. Glucocorticoidd therapy may reduce bone density in congenital suprarrenal hiperplasia. Clin Endocrinol. 2019. https://doi.org/10.1111/CEN14149

  5. Omneya MO‚ Shaymaa E‚ Abohashaba M. Growth and bone mineral density in egypssian children it congenital suprarrenal hyperplasia on glucocorticoid replacement therapy. A single center study. ESPE abstract. 2019:2-33.

  6. Metwalley A, El Saied AR. Bone mineral status in egypsian children with classic congenital suprarrenal hiperplasia. A single center study from Upper Egypt. Indian endocrinol Metab. 2014;18(5):700-4.

  7. Halper A‚ Sánchez B‚ Hodges J‚ Kelly A. Bone mineral density and body composition in children with congenital suprarrenal hiperplasia. Clinical endocrinology. 2018;88(6):8-18.

  8. Tielz. Fundamentals of Clinical Chemestry. Primera Edición ed. Carl A Burtis ERA, editor. Philadelphia; 2001.

  9. Burtis CA, Ashwood ER, Bruns DE, Tietz . Texbook of Clinical Chemestry and molecules diagnostics. Fourth Edition ed. Editorial Philadelphia, WB Saunders Co; 2006.

  10. Christiansen P, Molgaard C, Müller J. Normal bone mineral content in young adults with congenital suprarrenal hiperplasia due to 21-hydroxilase deficiency. Hormone Research. 2014;61(3):133-6.

  11. Comité Nacional de Endocrinología. Consideraciones para una corticoterapia segura. Arch Argent Pediatr. 2018;116(Supl 3):S71-S6.

  12. Espinosa RT, Hernández MJA, Dominguez AE. Composición corporal y factores relacionados, en pacientes con hiperplasia suprarrenal congénita. Rev Cubana de Endocrinol. 2018;29(3):178-84.

  13. Fleishman A, Ringelhein J, Feldman HA, Gordon CM. Bone Mineral status in Children with Congenital Suprarrenal Hyperplasia. J Pediatr Endocrinol Metab. 2014;20(2):227-35.

  14. Raizada N, Jyotsna VP, Upadhyay AD, Gupra N. Bone mineral density in Young adult women with congenital suprarrenal hyperplasia. Indian Journal of Endocrinology and Metabolism. 2016;20(1):62-8.

  15. Callewaert F, Boonen S, Vanderschueren D. Sex steroids and the male skeleton: a tale of two hormones. Trends Endocrinol Metab. 2010;21(2):89-95.

  16. Río LCMF, Fernández CRLL. Esteroides sexuales y metabolismo mineral óseo. Revista Ortho-tips. 2010;6(1):14-30.

  17. Henwood MT, Binkovitz L. "Update on pediatric bone health," The Journal of the American Osteopathic Association. 2009;109(1):5-12.

  18. Ceccato F, Barbot M, Albiger N, Zilio MD, Toni P, Luisetto G, et al. Long- glucocorticoid effect on bone mineral density in patients with congenital suprarrenal hyperplasia due 21-hydroxilase deficiency. Europ Journal of endocrinology. 2016;175(2):101-6.




2020     |     www.medigraphic.com