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Paper 1
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
Autosomal dominant polycystic kidney disease is the most common hereditary cause of end stage renal failure; however the kidneys usually appear enlarged due to the innumerable cysts. The cysts can be variable in size and can become very large rather than the small cysts described in the question.
Normal and Abnormal Development of the Biliary Tree
Published in Gianfranco Alpini, Domenico Alvaro, Marco Marzioni, Gene LeSage, Nicholas LaRusso, The Pathophysiology of Biliary Epithelia, 2020
Autosomal dominant polycystic kidney disease is one of the more common hereditary diseases in man, occurring in 1:1000 individuals. It is characterized by the progressive development and enlargement of multiple fluid-filled cysts in the kidneys that may ultimately lead to end-stage renal disease. Hepatic involvement can be completely incidental, as illustrated in (Fig. 7A). Alternatively, the liver itself may have massive involvement (Fig. 7C), not unlike the renal disease (Fig. 7D). The cysts are lined by a simple biliary epithelium (Fig. 7B). Careful histomorphometric analysis demonstrates a strong positive correlation between the density of biliary microhamartomas (in essence, von Meyenburg complexes) and the severity of the polycystic liver disease.93 This finding supports the view that hepatic cysts in ADPKD result from cystic dilatation of biliary microhamartomas, with separation over time of the cysts from the biliary ducts from which they are derived.
Echogenic Kidneys
Published in Amar Bhide, Asma Khalil, Aris T Papageorghiou, Susana Pereira, Shanthi Sairam, Basky Thilaganathan, Problem-Based Obstetric Ultrasound, 2019
Amar Bhide, Asma Khalil, Aris T Papageorghiou, Susana Pereira, Shanthi Sairam, Basky Thilaganathan
Kidneys are considered echogenic when they look brighter than the adjacent liver or spleen. The degree of echogenicity does not seem to be correlated with fetal outcome, and the difficulty is determining if the echogenic kidneys are a normal variant or an indicator of severe renal disease. The presence of positive family history of kidney disease is common in autosomal dominant polycystic kidney disease and, although typically the condition only manifests in adult life, early manifestation with pre-natal findings have been reported.
Morning blood pressure surge in early autosomal dominant polycystic kidney disease and its relation with left ventricular hypertrophy
Published in Renal Failure, 2021
Abdülmecit Yildiz, Saim Sag, Cuma Bulent Gul, Sümeyye Güllülü, Fatma Ezgi Can, Ömer Bedir, Mehmet Fethullah Aydin, Ayşegül Oruç, Sadettin Demirel, Suat Akgür, Mustafa Güllülü, Alparslan Ersoy
Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease and is characterized by extra-renal manifestations ranging from liver disease to various cardiac abnormalities such as mitral valve prolapses [1]. In addition, some cardiovascular (CV) abnormalities like left ventricular hypertrophy (LVH) and endothelial dysfunction (ED) have been reported in young normotensive ADPKD patients with preserved kidney function. However, in normal populations, both LVH and ED are commonly found in hypertensive and elderly patients but not in normotensive and young individuals [2]. Although some pathophysiologic events such as borderline hypertension (HT), increased sympathetic activity, and abnormal ciliary activity on endothelial cells have been suggested as a cause of LVH and ED in early ADPKD patients, no clear cause has been identified until now [3]. Some studies on ADPKD patients evaluated the 24-h ambulatory blood pressure (BP) characteristics and found impaired circadian variation in BP [4,5]. However, to our knowledge, no study sought MBPS and Its relation with CV abnormalities, which are common in ADPKD patients. Morning hours are characterized by the highest incidence of major cardiovascular events, including myocardial infarction, stroke, or sudden death. The most likely reason for this is the activation of the sympathetic nervous system in the early hours of the day leads to a rapid increase in blood pressure (BP), known as MBBS. Chronically higher levels of MBPS may result in structural alterations in arterial vessels lead to LVH in the myocardium and ED in the vascular bed.
Excess healthcare costs in patients with autosomal dominant polycystic kidney disease by renal dysfunction stage
Published in Journal of Medical Economics, 2021
Patrick Gagnon-Sanschagrin, Yawen Liang, Myrlene Sanon, Dorothee Oberdhan, Annie Guérin, Martin Cloutier
Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited kidney disease, with a prevalence of 4.3 per 10,000 in the United States (US)1. ADPKD is characterized by the development of renal cysts that disrupt urine concentration and may cause symptoms such as hypertension, hematuria, abdominal pain, and urinary tract infections2,3. In early stages, the disease is usually asymptomatic for decades, with renal function appearing normal from compensatory mechanisms; in later stages, the disease may cause functional deterioration4. However, a significant number of patients with ADPKD can progress through chronic kidney disease (CKD) stages at a younger age and reach end-stage renal disease (ESRD) at a faster rate5. In most patients, ADPKD eventually progresses to end-stage renal disease requiring renal replacement therapy (ESRD-RRT), such as long-term dialysis or kidney transplant3. Accordingly, ADPKD disproportionately accounts for 7–10% of all ESRD cases in Europe4 and about 5% in the US reported by USRDS (United States Renal Data System).
The Effect of Smoking on Endothelial Dysfunction in Autosomal Dominant Polycystic Kidney Disease Patients with Preserved Renal Function
Published in Renal Failure, 2021
Cuma Bulent Gul, Abdulmecit Yildiz, Saim Sag, Aysegul Oruc, Alparslan Ersoy, Sumeyye Gullulu
Autosomal dominant polycystic kidney disease (ADPKD) is the most prevalent heritable kidney disease. This condition may progress as far as the phase of end-stage renal disease in some patients. Moreover, ADPKD patients also have increased cardiovascular morbidity compared with their non-ADPKD counterparts who have functional kidney impairment of similar magnitude [1]. Endothelial dysfunction (ED) is considered to be the earliest discernable phase of the atherosclerotic process [2,3]. ED is seen very early during the course of ADPKD, long before renal function starts to deteriorate. Although many factors ranging from disease-specific abnormalities to nontraditional risk factors have been held responsible for the development of ED in the early phase of ADPKD, the main cause of ED is still unclear [4].