The Effectiveness of Cinacalcet as an Adjunctive Therapy for Hereditary 1,25 Dihydroxyvitamin D3-Resistant Rickets

Journal Title: Journal of Clinical Research in Pediatric Endocrinology - Year 2017, Vol 9, Issue 2

Abstract

High doses of oral calcium or long-term calcium infusions are recommended to correct the hypocalcemia and secondary hyperparathyroidism in patients with hereditary 1,25 dihydroxyvitamin D3-resistant rickets (HVDRR). Preliminary studies revealed that calcimimetics may be a safe and effective therapeutic choice in children with secondary hyperparathyroidism. Our aim was to observe the efficacy of cinacalcet in the normalization of secondary hyperparathyroidism and hypophosphatemia in two siblings aged 2.5 years and 6 months with HVDRR who did not respond to traditional treatment regimes. Both patients were admitted to the hospital with severe hypocalcemia. They were treated with high doses of calcitriol and calcium infusions intravenously. Secondary hyperparathyroidism was normalized temporarily, but did not improve completely. Cinacalcet (0.25 mg/kg) once a day along with the high doses of oral calcium and calcitriol was added to the treatment schedule. After 3 months, biochemical and radiologic findings reverted to normal. Our findings indicate that cinacalcet is effective in normalizing the hyperparathyroidism and hypophosphatemia in these cases and in improving the bone pathology.

Authors and Affiliations

Ayşehan Akıncı, İsmail Dündar, Meltem Kıvılcım

Keywords

Related Articles

Comparison of Updated Weight and Height Percentiles with Previous References in 6-17-Year-Old Children in Kayseri, Turkey

Objective: To compare updated weight and height percentiles of 6-17-year-old children from all socio-economic levels in Kayseri with previous local references and other national/international data. Methods: The second s...

Pitfalls with Vitamin D Research in Musculoskeletal Disorders and Recommendations on How to Avoid Them

Reports suggesting that vitamin D may have extraskeletal roles have renewed interest in vitamin D research and stimulated publication of an increasing number of new studies each year. These studies typically assess vitam...

Time Trends, Regional Variability and Seasonality Regarding the Incidence of Type 1 Diabetes Mellitus in Romanian Children Aged 0-14 Years, Between 1996 and 2015

Objective: The incidence of type 1 diabetes mellitus in children is highly variable in the world. The aim of our study was to: 1) analyze the evolution of the incidence of childhood type 1 diabetes in Romania between 199...

Pathogenesis of Thalassemia Major-associated Osteoporosis: A Review with Insights from Clinical Experience

Due to increasing life expectancy in thalassemia major (TM), osteoporosis is emerging as a significant problem. Its aetiology is multifactorial, culminating in increased bone resorption and impaired remodelling. Hypogona...

Higher-Than-Conventional Subcutaneous Regular Insulin Doses in Diabetic Ketoacidosis in Children and Adolescents

Objective: To evaluate the effect of initial insulin dosage on glycemic control in the first 48 hours of subcutaneous regular insulin therapy after resolution of diabetic ketoacidosis (DKA). Methods: Records of patients...

Download PDF file
  • EP ID EP240475
  • DOI 10.4274/jcrpe.3486
  • Views 157
  • Downloads 0

How To Cite

Ayşehan Akıncı, İsmail Dündar, Meltem Kıvılcım (2017). The Effectiveness of Cinacalcet as an Adjunctive Therapy for Hereditary 1,25 Dihydroxyvitamin D3-Resistant Rickets. Journal of Clinical Research in Pediatric Endocrinology, 9(2), 172-178. https://europub.co.uk/articles/-A-240475