OBJECTIVE: @entity19372 ( @entity19372 ) is a disorder characterized by tubular dilation of renal collecting ducts and cystic dilation of medullary pyramids that has been associated with stone disease. The significance of @entity1790 and the mechanisms by which it occurs are incompletely understood. We describe clinical and metabolic features of @entity1790 in a cohort of @entity1 with @entity19372 . METHODS: Records were reviewed of 56 @entity1 , all with radiographic diagnosis of @entity19372 and data collected pertaining to presentation, stone events and recurrences, stone composition, and metabolic profile to perform a descriptive study with median 3.7 years follow-up. RESULTS: @entity1790 was confirmed radiographically in 39/56 @entity1 (69.6%). No @entity1 without evidence of @entity1790 developed a stone event, whereas 13/39 (33%) of those with @entity1790 developed a recurrent stone event. Stones were composed of @entity3835 , @entity3835 , @entity78 apatite, and @entity414 . Metabolic profile was obtained for 26 of 39 (67%) stone-forming @entity1 demonstrating abnormalities in 22/26 (84.6%). These included @entity5200 , 58% (15/26); low urine volume, 35% (9/26); hyperuricosuria, 27% (7/26); hypocitraturia, 19% (5/26); elevated urine @entity1372 , 15% (4/26); and @entity5201 , 12% (3/26). CONCLUSION: Many @entity1 with @entity19372 have no evidence of @entity1790 . Among those who do, recurrence is common, and metabolic profile and composition are varied as in the general stone-forming population.

XXXX in @entity19372 : evaluation of clinical and metabolic features.

@entity1 :: ('9606', 'Species') :: ['patients', 'patient'] @entity5201 :: ('MESH:D006959', 'Disease') :: ['hyperoxaluria'] @entity3835 :: ('MESH:D002129', 'Chemical') :: ['calcium oxalate monohydrate', 'calcium oxalate dihydrate'] @entity19372 :: ('MESH:D007691', 'Disease') :: ['MSK', 'Medullary sponge kidney', 'medullary sponge kidney'] @entity78 :: ('MESH:C020243', 'Chemical') :: ['calcium phosphate'] @entity1372 :: ('MESH:D012964', 'Chemical') :: ['sodium'] @entity1790 :: ('MESH:D053040', 'Disease') :: ['Nephrolithiasis', 'nephrolithiasis'] @entity5200 :: ('MESH:D053565', 'Disease') :: ['hypercalciuria'] @entity414 :: ('MESH:D014527', 'Chemical') :: ['uric acid']
@entity1790 :: (MESH:D053040,Disease) :: ['Nephrolithiasis']
