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Paper 4
Published in Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw, The Final FRCR, 2020
Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw
The imaging appearances are consistent with medullary sponge kidney. On intravenous urograms the appearance used to be likened to a paintbrush; otherwise known as a ‘striated nephrogram’. Other causes for this appearance bilaterally include acute pyelonephritis, acute tubular necrosis, hypotension and autosomal recessive polycystic kidney disease. The latter is associated with paediatric patients. The history does not fit with hypotension, acute pyelonephritis or acute tubular necrosis. Urine dip would typically be abnormal in pyelonephritis and acute tubular necrosis, either showing signs of nitrites, blood or protein. If these conditions were suspected clinically, then answers B and D may be appropriate.
Urinary system
Published in Aida Lai, Essential Concepts in Anatomy and Pathology for Undergraduate Revision, 2018
Medullary sponge kidney Collecting ducts in medulla are dilatedCysts occur in papillaeSymptoms: – pain if calculi form in cysts– haematuriaComplications: – infections– formation of renal calculi
Gastrointestinal
Published in Vincent Helyar, Aidan Shaw, The Final FRCR, 2017
Rare, autosomal recessive disease. Patients often present aged 10–30 years with recurrent cholangitis. Predisposes to infection, inflammation, stones and cholangiocarcinoma. Associated with medullary sponge kidney and renal cysts.
Analysis of urinary exosomes applications for rare kidney disorders
Published in Expert Review of Proteomics, 2020
Isabella Panfoli, Simona Granata, Giovanni Candiano, Alberto Verlato, Gianmarco Lombardi, Maurizio Bruschi, Gianluigi Zaza
Additionally, we previously carried out a comprehensive comparative proteomic analysis of urinary microvesicles and exosomes (isolated by differential ultracentrifugation) of 15 patients with ADPKD and 15 patients with medullary sponge kidney (MSK) disease to identify differences in terms of the mechanism of cystogenesis and identify putative diagnostic biomarkers [82,86]. MSK is a rare congenital disorder characterized by dilation of the collecting ducts in the renal papillae, urinary acidification, and concentration defects, cystic anomalies of precalyceal ducts associated with a high risk of nephrocalcinosis, and recurrent kidney stones [101]. Despite sporadic genetic associations [102,103] and the dysregulation of a few biologic factors [104–106], the underlying mechanisms of pathogenesis are not yet well understood.
Exome sequencing of Saudi Arabian patients with ADPKD
Published in Renal Failure, 2019
Fahad A. Al-Muhanna, Abdullah M. Al-Rubaish, Chittibabu Vatte, Shamim Shaikh Mohiuddin, Cyril Cyrus, Arafat Ahmad, Mohammed Shakil Akhtar, Mohammad Ahmad Albezra, Rudaynah A. Alali, Afnan F. Almuhanna, Kai Huang, Lusheng Wang, Feras Al-Kuwaiti, Tamer S. Ahmed Elsalamouni, Abdullah Al Hwiesh, Xiaoyan Huang, Brendan Keating, Jiankang Li, Matthew B. Lanktree, Amein K. Al-Ali
Polycystic kidney disease (PKD) is the most common inherited multi-systemic disease characterized by the progressive development of kidney cysts with consequent enlargement of the kidney and progression toward end-stage renal disease (ESRD) [1,2]. Kidney cysts range in size from microscopic to many centimeters in diameter, leading to inflammation, regional ischemia, cytokine release, tubular obstruction, and subsequent loss of kidney function. Two forms of PKD include autosomal dominant polycystic kidney disease (ADPKD) and autosomal recessive polycystic kidney disease (ARPKD) that are typically distinguishable by their pattern of inheritance, as well as age of onset, hepatic fibrosis, arterial hypertension, kidney morphology, and cyst location [3]. ADPKD is the most prevalent inherited form of kidney disease with an incidence of 1:500 to 1:1000 individuals and is observed in approximately 7%–10% of patients with ESRD [4,5]. Extra-renal formation of cysts, mainly in the liver and pancreas, abnormalities in connective tissues, and aortic and intracranial aneurysms have been reported in ADPKD patients [6]. Hypertension is prevalent among ADPKD patients and usually precedes loss of glomerular filtration rate [7]. Additional cystic kidney phenotypes exist that could be misdiagnosed as ADPKD including multiple simple cysts, autosomal dominant tubulointerstitial nephritis, tuberous sclerosis complex, medullary sponge kidney, HNF1B nephropathy, nephronopthisis, and others [8]. Genetic testing could be useful to clarify the diagnosis in patients with features inconsistent with typical ADPKD.
Fluoroless endourological surgery for high burden renal and proximal ureteric stones: A safe technique for experienced surgeons
Published in Arab Journal of Urology, 2021
Elias M. Ayoub, Ali Bourgi, Josee Alsouki, Sleiman Merhej, Pierre Conort
Patients included in this study were not candidates to any restriction related to their age, medical history or stone characteristics. The mean (range) age was 47.3 (24–81) years. Recurrent urinary lithiasis was encountered in 57.9% of patients, of whom 19 had cystinuria and nine medullary sponge kidney disease. In all, 10 patients were under chronic use of anti-platelet drugs, which were not discontinued before the operation; these patients were candidates for fURS only.