Ask-Upmark Kidney and Tubulointerstitial Nephritis in a Woman with Severe Renal Failure

Abstract

Ask-Upmark kidney is a rare diagnosis of segmental hypoplasia in pediatric population clinically characterized by severe hypertension potentially treatable with partial to total nephrectomy. Although originally was described only as a congenital anomaly, recent data suggest to be caused by vesicoureteral reflux, either in utero or in early childhood and pyelonephritis. The case we reported indicates that Ask-Upmark kidney should be considered as potential cause of hypertension and renal failure both in children and adults. The renal biopsy is necessary for early diagnosis and may consent to normalize blood pressure with nephrectomy; however, if renal damage is severe and progressive with tubulointerstitial nephritis, surgical management is excluded and renal transplant should be considered.

Imaging of bilateral Ask-Upmark kidney

“…The radiological findings in this patient were examinations make a non-invasive diagnosis of AUK possible. The pathogenesis of this condition is controversial [1][2][3]. However, the case we described here indicates that the pathogenesis of AUK is most likely related to developmental anomalies of the kidneys and the urinary tract, rather than renal scarring.…”

“…The patient's blood pressure was successfully controlled with an angiotensin converting enzyme inhibitor, and was stabilized at 120/70 mmHg, with elevated serum aldosterone levels and plasma renin activity (274.0 pg/mL and 20.7 ng/mL/h, respectively). Renal segmental hypoplasia, known as Ask-Upmark kidney (AUK), is an extremely rare renal abnormality, grossly characterized by one or more sharply separated hypoplastic segments that overlie the dilated calyces and are retracted with capsular grooving [1,2]. The radiological findings in this patient were examinations make a non-invasive diagnosis of AUK possible.…”