History and exam

Key diagnostic factors

common

presence of risk factors

Key risk factors strongly associated with medullary sponge kidney include, hemi-hypertrophy, Beckwith-Wiedemann's syndrome, Caroli's disease, congenital hepatic fibrosis, Ehlers-Danlos' syndrome, family history of polycystic kidney disease, and chronic kidney disease.

Other diagnostic factors

common

previous nephrolithiasis

Reported incidence of medullary sponge kidney in recurrent stone formers is variable, ranging from 3% to 20%.[3][10]

flank pain

Patients may present with flank pain from nephrolithiasis or pyelonephritis due to infected stones. Some patients have chronic severe pain.

haematuria

Microscopic or gross haematuria is commonly found.

May be due to nephrolithiasis or infection.

costovertebral angle tenderness

May be due to nephrolithiasis or pyelonephritis.

dysuria

Patients with medullary sponge kidney often have history of recurrent urinary tract infections (UTIs).

May present with symptoms of UTI such as painful urination.

urinary urgency

Patients with medullary sponge kidney often have history of recurrent urinary tract infections (UTIs).

May present with symptoms of UTI such as urinary urgency.

increased frequency of urination

Patients with medullary sponge kidney often have history of recurrent urinary tract infections (UTIs).

May present with symptoms of UTI such as increased frequency of urination.

uncommon

groin pain

As stones pass through the ureter, pain may radiate towards the groin.

Risk factors

strong

hemi-hypertrophy

Medullary sponge kidney may occur with congenital defects such as hemi-hypertrophy (enlargement of one side of the body).

Beckwith-Wiedemann's syndrome

Medullary sponge kidney may occur with other congenital defects such as Beckwith-Wiedemann's syndrome (congenital overgrowth).

Caroli's disease

Medullary sponge kidney may occur with other congenital defects such as Caroli's disease (fibropolycystic liver disease).

congenital hepatic fibrosis

Medullary sponge kidney may occur with other congenital defects such as congenital hepatic fibrosis.

Ehlers-Danlos' syndrome

Medullary sponge kidney may occur with other congenital defects such as Ehlers-Danlos' syndrome (connective tissue disorder).

family history of polycystic kidney disease

Medullary sponge kidney may occur with other congenital defects such as autosomal-dominant polycystic kidney disease.

chronic kidney disease

Medullary sponge kidney may occur with kidney disease thought to be due to recurrent and/or infected stones.

weak

hypercalciuria

Some studies have found hypercalciuria in most Medullary sponge kidney (MSK) patients (88%), whereas others have reported it less frequently (9% to 44%).[23][26] Other studies found similar frequencies of hypercalciuria in stone formers with and without MSK.[11][27][28]

female sex

Some studies have suggested Medullary sponge kidney is more common in women, whereas others have found no difference between the sexes.[11]

hyperparathyroidism

Medullary sponge kidney (MSK) may be associated with primary hyperparathyroidism.[4] However, the hyperparathyroidism seems to follow MSK.

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