The presence of this diagnostic tetrad, added to laboratory data and clinical history, could be helpful in the differential diagnosis to identify patients with MSK. We identified a peculiar tetrad associated with MSK: 1) hypoechoic medullary areas, 2) hyperechoic spots, 3) microcystic dilatation of papillary zone, 4) multiple calcifications (linear, small stones or calcified intracystic sediment) in each papilla. Ultrasound examination showed bilateral renal cysts, usually small and located in the renal medulla, and microcalcifications located in the medulla or within the cysts. Seven patients underwent computed tomography all of them received ultrasound. Urinary tract infections (44%), nephrolithiasis (33%), microscopic hematuria (50%) and proteinuria (44%) were reported. Seventy-two percent of patients presented with chronic kidney disease, 22% required hemodialysis. Patients were referred to our outpatient clinic because of renal impairment (44%), family history of nephropathy (17%), nephrolithiasis or an established diagnosis of MSK (39%). Specifically, we focused on ultrasound imaging. We reviewed their clinical and family history, laboratory data and imaging studies. Out of 4321 patients, 18 had a diagnosis of MSK. We conducted a retrospective study of patients seen between January 1st 2009 and January 1st 2019 in our clinic. We identified a cohort of patients from our outpatient clinic with established diagnosis of MSK to outline some ultrasonographic characteristics that may help establish a diagnosis. Intravenous urography is still considered the gold standard for diagnosis. Medullary sponge kidney (MSK) is a rare disease characterized by cystic dilatation of papillary collecting ducts.
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