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The renal system
Published in Laurie K. McCorry, Martin M. Zdanowicz, Cynthia Y. Gonnella, Essentials of Human Physiology and Pathophysiology for Pharmacy and Allied Health, 2019
Laurie K. McCorry, Martin M. Zdanowicz, Cynthia Y. Gonnella
Polycystic kidney disease is a hereditary disorder that can have an autosomal dominant (adult form) or autosomal recessive (childhood form) pattern of inheritance. Cysts develop in both kidneys and gradually increase in size. Enlarging cysts can destroy normal kidney tissue and lead to a progressive loss of renal function that may culminate in renal failure. The recessive form of the disease may cause renal failure in childhood, whereas the dominant form progresses more slowly and generally does not lead to renal failure until the patient enters their 60s or 70s.
SBA Answers and Explanations
Published in Vivian A. Elwell, Jonathan M. Fishman, Rajat Chowdhury, SBAs for the MRCS Part A, 2018
Vivian A. Elwell, Jonathan M. Fishman, Rajat Chowdhury
Adult polycystic kidney disease is one of the most common inherited disorders in humans, affecting approximately 1 in 1000 individuals and accounting for 10 per cent of cases of end-stage renal failure. It is inherited as an autosomal dominant condition with a late-onset mode of presentation. Eighty-five per cent of cases have been localized to a gene on the short arm of chromosome 16 (PKD1 gene). A second gene (PKD2), responsible for around 15 per cent of cases, has been localized to the long arm of chromosome 4. The corresponding gene products have been named polycystin-1 and polycystin-2, although their exact function is unknown.
Urology
Published in Kaji Sritharan, Samia Ijaz, Neil Russell, Tim Allen-Mersh, 300 Essentials SBAs in Surgery, 2017
Kaji Sritharan, Samia Ijaz, Neil Russell, Tim Allen-Mersh
Which of the following statements is NOT true regarding polycystic kidneys? The condition is characterised by multiple cysts in both kidneys.There is a strong association with intracranial berry aneurysms.Polycystic kidney disease usually presents between 30 and 60 years of age.The condition is most commonly inherited as an autosomal-dominant form.Ultrasonography is of no use in the diagnosis.
A survey of the radiological follow-up of unruptured intracranial aneurysms in the United Kingdom
Published in British Journal of Neurosurgery, 2023
Samuel Hall, Ashraf Abouharb, Ian Anderson, Andrew Bacon, Anuj Bahl, Howard Brydon, Graham Dow, Ioannis Fouyas, James Galea, Anthony Ghosh, Nihal Gurusinghe, Mahmoud Kamel, Pawan Minhas, Patrick Mitchell, David Mowle, Nitin Mukerji, Ramesh Nair, John Norris, Hiren Patel, Jash Patel, Krunal Patel, Jerome St George, Mario Teo, Ahmed Toma, Rikin Trivedi, Chris Uff, Anna Visca, Daniel C. Walsh, Edward White, Peter Whitfield, Diederik Bulters
Examples of where questions may have introduced bias included: the age used to distinguish where practices in UIA follow-up may change, the size of aneurysm to distinguish different treatment strategies, and the examples of what might constitute high-risk features. The latter included previous SAH, family history, and adult polycystic kidney disease although there are many more which were not explicitly mentioned. Despite many clinicians treating those with polycystic kidney disease as a higher risk group, there is little evidence to support this.25 There would also be a case to include aneurysm morphology although this was omitted due to difficulties with its definition. Similarly, smoking and hypertension could have been included although were omitted as it was felt that they should be managed medically and not alter the decision whether to intervene.
Infliximab for the treatment of patients with checkpoint inhibitor associated acute tubular interstitial nephritis
Published in OncoImmunology, 2021
Jamie S. Lin, Omar Mamlouk, Umut Selamet, Amanda Tchakarov, William F. Glass, Rahul A. Sheth, Rachel M. Layman, Ramona Dadu, Noha Abdel-Wahab, Maen Abdelrahim, Adi Diab, Cassian Yee, Ala Abudayyeh
We retrospectively reviewed the medical records of all patients who received CPI treatment, developed AKI, and had a diagnostic kidney biopsy at The University of Texas MD Anderson Cancer Center (MDACC) from 2016 to 2020 (Supplemental Table 1). This retrospective study was approved by the MDACC Institutional Review Board in accordance with the Declaration of Helsinki. We identified 85 cases who had AKI from 2016 to 2020. Eight cases had biopsy-proven ATIN treated with GC (equivalent prednisone dose of at least 0.5 mg/kg) and infliximab (5 mg/kg). We collected two additional cases with presumed CPI-associated ATIN. Due to the high-risk potential for renal hemorrhage, kidney biopsy was deferred in two cases (Case 3 and 10). The patient in Case 3 had a solitary kidney and on anticoagulation (clopidogrel and aspirin) for a recent transient ischemic attack. The patient in Case 10 had a history of polycystic kidney disease. For all cases, we collected the following information: age, sex, cancer diagnosis, name and class of CPI, potential nephrotoxic medications, serum creatinine at baseline and during AKI, date of last follow-up, urine sediment, proteinuria, serological markers, kidney pathology findings, infliximab adverse effects, and tumor status.
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.