What is osteodystrophy (renal rickets)?

Updated: Jun 19, 2020
  • Author: Horacio B Plotkin, MD, FAAP; Chief Editor: Jatinder Bhatia, MBBS, FAAP  more...
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Answer

Answer

In end-stage renal disease, renal 1-hydroxylase is diminished or lost, and excretion of phosphate is defective. This leads to low levels of 1,25(OH) 2 vitamin D, hypocalcemia, and failure of osteoid calcification. Osteodystrophy (ie, renal rickets) is the only type of rickets with a high serum phosphate level. It can be adynamic (a reduction in osteoblastic activity) or hyperdynamic (increased bone turnover).

Calcium receptors (CaRs) have been discovered in bone, kidney, and intestine and also in organs not directly related to calcium regulation. Mutations that cause loss of function in the CaRs result in familial benign hypocalciuric hypercalcemia and neonatal severe hyperparathyroidism. Familial benign hypocalciuric hypercalcemia is usually associated with heterozygous inactivating mutations of the CAR gene, whereas neonatal severe hyperparathyroidism is usually due to homozygous inactivation of the CAR gene.

Familial benign hypocalciuric hypercalcemia is generally asymptomatic and is characterized by mild to moderate, lifelong hypercalcemia; relative hypocalciuria; and normal intact parathyroid hormone. Individuals with neonatal severe hyperparathyroidism frequently develop life-threatening hypercalcemia.

Treatment of these patients includes phosphate binders, a low phosphate intake, and calcitriol and other vitamin D analogs.


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