ESPE Abstracts (2023) 97 P2-173

ESPE2023 Poster Category 2 Bone, Growth Plate and Mineral Metabolism (27 abstracts)

Caval calcium infusion is the best solution for patients with hereditary vitamin D resistant rickets (HVDRR)

Ihab Abdulhamid 1 & Doaa Khater 2

1Zagazig Faculty of medicine, Zagazig, Egypt. 2Alexandria Faculty of medicine, Alexandria, Egypt

Hereditary vitamin D-resistant rickets (HVDRR) is an autosomal recessive disorder characterized by end organ resistance to 1α,25-dihydroxyvitamin D3 (1,25D3). The syndrome is recognized by severe early onset rickets with bowing of the lower extremities, short stature, often alopecia and severe hypocalcemia.

Objectives: To study the effectiveness of our designed protocol of continuous high dose intracaval calcium infusion for the treatment of HVDRR.

Patients and Methods: We studied 12 patients with HVDRR, four of them had alopecia. Age of starting treatment protocol ranged from 2 -12 years. Daily infusions ranging from 1-2.2 g elemental calcium with oral phosphate from 1-2 g and oral calcium from 3.2–4.2 g elemental calcium were given for a period of 8-18 months. Followed by maintenance on oral calcium equivalent to 6.2 - 8g elemental calcium per day with oral phosphate 1-2 g. Measurements of serum calcium, phosphate and serum alkaline phosphatase were obtained before, during and after the calcium infusions. Urea and electrolytes, serum parathyroid hormone and vitamin D metabolites were measured prior to calcium infusion, then repeated monthly. Kidney ultrasound was done every 6-months. Skeletal X-ray was done before, 6 and 12 months of treatment.

Results: The daily intra-caval infusions of calcium with oral phosphate and calcium resulted in marvelous clinical, biochemical and radiological responses with normalization of calcium, phosphate, alkaline phosphatase and parathyroid hormone in 8-24 months with improvement in height slandered deviation. The patients showed no radiological evidence of nephrocalcinosis on follow up.

Conclusion: The use of intra-caval calcium infusions followed by high dose oral calcium with oral phosphate is an effective method for treatment of HVDR.

Volume 97

61st Annual ESPE (ESPE 2023)

The Hague, Netherlands
21 Sep 2023 - 23 Sep 2023

European Society for Paediatric Endocrinology 

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