Medullary cystic kidney

This code describes a rare, inherited kidney disorder characterized by the formation of multiple cysts primarily in the renal medulla, leading to progressive kidney dysfunction. It is a form of nephronophthisis, often resulting in end-stage renal disease.

Use this code for patients diagnosed with medullary cystic kidney disease, also known as nephronophthisis. Documentation should clearly indicate the presence of medullary cysts and the associated clinical presentation, such as polyuria, polydipsia, and progressive renal impairment.

• Differentiate from other cystic kidney diseases like autosomal dominant polycystic kidney disease (ADPKD), which has distinct genetic and clinical features.
• Consider sequencing with codes for chronic kidney disease (CKD) stages, as medullary cystic kidney disease often progresses to renal failure.
• Ensure documentation specifies "medullary" to distinguish from other types of renal cysts.