Usefulness of an specific out patient clinic on hereditary kidney diseases: A different approach based on the family tree

Hereditary kidney diseases (HKDs) are a heterogeneous group of kidney diseases that account for 10% to 15% of patients who start renal replacement therapy (RRT), and could account for an even greater percentage in the paediatric population. 1 HKDs require a diagnostic approach that is different from other nephropaties since its pathogenesis is due to a mutation.The diagnosis goes beyond the affected patients, it includes the family.Ait is necessary to construct a family tree including both affected and non affected individuals.Autosomal dominant polycystic kidney disease (ADPKD), which is due to a mutation in the PKD1 or PKD2 2 gene, is the most common hereditary kidney disease and accounts for 6-10% of patients in RRT.It is characterised by the growth of kidney cysts that lead to a decline in kidney function until RRT is required. 3In addition, it is associated with other manifestations: hypertension (HTN), 4 polycystic liver disease, brain aneurysms and valve diseases. 5Alport syndrome (AS) is classified according to its pattern of inheritance: 85% X-linked owing to a mutation in COL4A5 6 and 15% autosomal recessive