E83.32

Billable

Hereditary vitamin D-dependent rickets (type 1) (type 2)

HCC Category Mapping

RxHCCHCC 43 — Other Significant Endocrine and Metabolic Disorders

0.000

What This Code Means

A genetic disorder where the body cannot properly process vitamin D, leading to weak bones and low calcium levels despite adequate vitamin D intake.

Coding Tips

•Document whether this is type 1 or type 2 in the medical record for clarity
•This condition typically presents in childhood and requires lifelong management

Clinical Significance

Hereditary vitamin D-dependent rickets (types 1 and 2) is a rare genetic disorder affecting vitamin D metabolism or receptor function, causing impaired calcium and phosphorus absorption. It requires lifelong treatment with high-dose calcitriol or calcium supplementation and has significant skeletal implications.

Documentation Requirements

✓Confirmed hereditary/genetic etiology of vitamin D-dependent rickets
✓Specification of type 1 (1-alpha-hydroxylase deficiency) or type 2 (vitamin D receptor defect) when documented
✓Laboratory data: calcium, phosphorus, alkaline phosphatase, 1,25-dihydroxyvitamin D, PTH levels
✓Skeletal imaging findings (rickets, osteomalacia)
✓Current treatment (calcitriol dosing, calcium supplementation)
✓Family history and genetic testing results if available

Commonly Confused Codes

E55.0 — Rickets, active: nutritional rickets from vitamin D deficiency, NOT a hereditary condition E83.31 — Familial hypophosphatemia: genetic phosphate wasting disorder, different mechanism E83.30 — Disorder of phosphorus metabolism, unspecified: less specific, different metabolic category M83.8 — Other adult osteomalacia: acquired osteomalacia, not hereditary

Code Hierarchy

└E83Disorders of mineral metabolism └E83.3Disorders of phosphorus metabolism and phosphatases └E83.32Hereditary vitamin D-dependent rickets (type 1) (type 2)

└E83.32Hereditary vitamin D-dependent rickets (type 1) (type 2)