Background: Medullary sponge kidney (MSK) is a rare disease characterized by cystic dilatation of papillary collecting ducts. Intravenous urography is still considered the gold standard for diagnosis. 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. Methods: We conducted a retrospective study of patients seen between January 1st 2009 and January 1st 2019 in our clinic. Out of 4321 patients, 18 had a diagnosis of MSK. We reviewed their clinical and family history, laboratory data and imaging studies. Specifically, we focused on ultrasound imaging. Results: 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%). Seventy-two percent of patients presented with chronic kidney disease, 22% required hemodialysis. Urinary tract infections (44%), nephrolithiasis (33%), microscopic hematuria (50%) and proteinuria (44%) were reported. Seven patients underwent computed tomography; all of them received ultrasound. Ultrasound examination showed bilateral renal cysts, usually small and located in the renal medulla, and microcalcifications located in the medulla or within the cysts. Conclusion: 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. 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.

Ultrasound to address medullary sponge kidney: A retrospective study / Pisani, I.; Giacosa, R.; Giuliotti, S.; Moretto, D.; Regolisti, G.; Cantarelli, C.; Vaglio, A.; Fiaccadori, E.; Manenti, L.. - In: BMC NEPHROLOGY. - ISSN 1471-2369. - 21:1(2020), pp. 430-438. [10.1186/s12882-020-02084-1]

Ultrasound to address medullary sponge kidney: A retrospective study

Pisani I.
;
Giacosa R.;Giuliotti S.;Regolisti G.;Cantarelli C.;Vaglio A.;Fiaccadori E.
Writing – Review & Editing
;
Manenti L.
2020-01-01

Abstract

Background: Medullary sponge kidney (MSK) is a rare disease characterized by cystic dilatation of papillary collecting ducts. Intravenous urography is still considered the gold standard for diagnosis. 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. Methods: We conducted a retrospective study of patients seen between January 1st 2009 and January 1st 2019 in our clinic. Out of 4321 patients, 18 had a diagnosis of MSK. We reviewed their clinical and family history, laboratory data and imaging studies. Specifically, we focused on ultrasound imaging. Results: 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%). Seventy-two percent of patients presented with chronic kidney disease, 22% required hemodialysis. Urinary tract infections (44%), nephrolithiasis (33%), microscopic hematuria (50%) and proteinuria (44%) were reported. Seven patients underwent computed tomography; all of them received ultrasound. Ultrasound examination showed bilateral renal cysts, usually small and located in the renal medulla, and microcalcifications located in the medulla or within the cysts. Conclusion: 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. 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.
2020
Ultrasound to address medullary sponge kidney: A retrospective study / Pisani, I.; Giacosa, R.; Giuliotti, S.; Moretto, D.; Regolisti, G.; Cantarelli, C.; Vaglio, A.; Fiaccadori, E.; Manenti, L.. - In: BMC NEPHROLOGY. - ISSN 1471-2369. - 21:1(2020), pp. 430-438. [10.1186/s12882-020-02084-1]
File in questo prodotto:
File Dimensione Formato  
BMCNephrol2020 medullary sponge disease.pdf

accesso aperto

Descrizione: articolo principale
Tipologia: Versione (PDF) editoriale
Licenza: Creative commons
Dimensione 2.75 MB
Formato Adobe PDF
2.75 MB Adobe PDF Visualizza/Apri

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11381/2881703
Citazioni
  • ???jsp.display-item.citation.pmc??? ND
  • Scopus 9
  • ???jsp.display-item.citation.isi??? 7
social impact