Hereditary kidney diseases are an important and specific group of kidney diseases having often systemic and multiorgan characteristics and a tendency to progress to chronic renal failure. The genetic background of the majority of hereditary nephropathies has been reported, an important step has been made to uncover their etiology and pathogenesis, and recently new innovative therapies have been proposed.
Gastrointestinal tract (GIT) involvement in hereditary kidney diseases presents in different forms, extents, and intensities. Though only a minor part of hereditary kidney disease, GIT involvement is an integral component of the clinical picture; for example, liver cysts are an important component of autosomal dominant polycystic kidney disease.
Even mild symptoms of GIT involvement can be an initial and potentially serious extrarenal manifestation of hereditary nephropathy, which deserves early diagnosis and initiation of treatment.