Int J Nephrol Renovasc Dis 6:139–142įagagnini S, Heinrich H (2017) Risk factors for gallstones and kidney stones in a cohort of patients with inflammatory bowel diseases. Acta Paediatr 36:107–111Ĭury DB, Moss AC, Schor N (2013) Nephrolithiasis in patients with inflammatory bowel disease in the community. Koshida R, Sakazume S, Maruyama H, Okuda N, Ohama K, Asano S (1994) A case of pseudo-Bartter’s syndrome due to intestinal malrotation. In cases where typical features are not present (e.g.Igrutinović Z, Peco-Antić A, Radlović N, Vuletić B, Marković S, Vujić A, Rasković Z (2011) Pseudo-Bartter syndrome in an infant with congenital chloride diarrhoea. The differential is narrow when the entire kidney is affected and cross-sectional imaging has been obtained and is largely limited to renal tuberculosis, however, this usually results in a shrunken calcified putty kidney. The presence of an inflammatory reaction in adjacent tissues often requires a large operative field and an anterolateral transperitoneal approach 5. Surgical nephrectomy is usually curative 4,5. If xanthogranulomatous pyelonephritis becomes established, no conservative or medical therapies exist. As such, the signal is heterogeneous on all sequences. MRI appearances mirror the heterogeneous nature of the mass with solid and cystic components surrounding a central staghorn calculus. In most cases, there is little, if any, renal function in the affected kidney 1. There is also a more focal form of xanthogranulomatous pyelonephritis, where a small low attenuation mass with an associated calculus is seen adjacent to a calyx or in one pole of a kidney. Sometimes there is a perinephric extension with thickening of Gerota's fascia. Calcification and a staghorn calculus can be better delineated on CT scan. The calyces, in contrast, are dilated giving a multiloculated appearance that has been likened to the paw print of a bear ( bear's paw sign) 3. The normal renal outline is lost and enlarged with a paradoxical contracted renal pelvis. CTĬT findings are most helpful in reaching the correct diagnosis. Ultrasound examination demonstrates an enlarged and distorted renal outline, with loss of the normal renal architecture and (usually) a centrally-located shadowing calculus. A calculus is not always present in such cases, it is not possible to make a plain film diagnosis. Plain radiograph findings are difficult to distinguish from a routine staghorn calculus, although fragmentation and enlargement of the renal outline may be seen. In other instances, this represents diffuse xanthogranulomatous pyelonephritis of one moiety of a duplex system Sometimes a truly focal process in a normal kidney Two forms of the disease are recognized both macroscopically and on imaging 1,5: This staging was originally described in a pediatric population but can be applied to adults. Stage III: the disease extends into the perirenal and pararenal spaces or diffuse retroperitoneum Stage II: involves renal parenchyma as well as an extension to perirenal/perinephric fat Stage I: the disease is confined to the renal parenchyma only One method of staging (originally proposed by Malek et al.) is based on the degree of involvement of the adjacent tissues 6: The inflammatory process eventually extends into the perinephric tissues and even adjacent organs 5. Foamy (lipid-laden) macrophages predominate 1,4. The kidney is eventually replaced by a mass of reactive tissue, surrounding the usually present (90%) inciting staghorn calculus with associated hydronephrosis of a greater or lesser degree. Xanthogranulomatous pyelonephritis is, as the name suggests, a chronic granulomatous process believed to be the result of subacute/chronic infection inciting a chronic but incomplete immune reaction 1,4. Various bacteria are isolated, however, the most commonly isolated species are Escherichia coli and Proteus mirabilis 1,4. Hematuria and flank pain are sometimes encountered 4.ĭespite often absent urinary tract symptoms, pyuria and positive urinary cultures are present in the majority of cases (95% and 60% respectively) 2. The clinical presentation is typically vague, consisting of constitutional symptoms such as malaise, weight loss and low-grade fever. There is also an increased incidence in patients with diabetes mellitus. There is a 2:1 female predilection, presumably relating to an increased incidence of urinary tract infections and thus struvite (staghorn) calculi. Xanthogranulomatous pyelonephritis is seen essentially in all age groups, but most frequently presents in middle-aged to elderly patients 1,5.
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