Extrarenal Complications of Polycystic Kidney Disease Medical Presentation

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Published on : Apr 23, 2019 | Views : 2423 | Downloads : 1

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Extrarenal Complications of Polycystic Kidney Disease Francesca Mancuso
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Major Extrarenal Complications Cerebral aneurysms Hepatic and pancreatic cysts Cardiac valve disease Colonic diverticula Abdominal wall and inguinal hernia Seminal vesicle cysts
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Cerebral Aneurysm Most severe complication Prevalence increases with age 5 percent in young adults As high as 20 percent in patients 60 years and older Aneurysm rupture most often occurs with larger aneurysms, usually before the age of 50 years and/or in patients with poorly controlled hypertension Most common symptoms: acute onset of severe headache, frequently associated with nausea and vomiting
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Cerebral Aneurysm Screening Routine screening for high-risk patients Previous rupture, positive family history of an intracerebral bleed or intracranial aneurysm, high-risk occupation in which loss of consciousness would place the patient or others at extreme risk, prior to surgery that is likely to be associated with hemodynamic instability with hypertension Screen patients who require chronic anticoagulation for disorders such as deep vein thrombosis or atrial fibrillation. Recommend NOT screening children - aneurysmal rupture is extremely rare Initial negative radiographic studies → rescreen every five years those who have a family history of aneurysm, intracerebral bleeding, or cerebrovascular accident Time-of-flight MRA without gadolinium and high-resolution CTA We generally prefer to use MRA for screening and use CTA only if there are contraindications to MRA; both high sensitivity
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Hepatic & Pancreatic Cysts Hepatic cyst prevalence increases with age 10 to 20% below the age of 30 years 50 to 70% over the age of 60 years Women may develop cysts at an earlier age, and massive cysts occur almost exclusively in women, particularly those who have had several pregnancies Asymptomatic with preserved hepatic function Rarely develop pain (which may require decompression of the cyst if it is persistent and severe) and/or cyst infection (which requires therapy with an antimicrobial, such as a fluoroquinolone) Acute pain usually results from cyst infection or hemorrhage and rarely from rupture or torsion Pancreatic cysts occur in approximately 7 to 36 percent of patients
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Cardiac Valvular abnormalities detected by cardiac ultrasonography in 25 to 30% of patients Most common abnormalities: mild mitral valve prolapse and aortic regurgitation Less frequent lesions include mitral and/or tricuspid regurgitation Generalized abnormalities in collagen and/or extracellular matrix may be responsible Increased incidence of coronary aneurysms and coronary artery dissection Asymptomatic pericardial effusions appear to occur at increased frequency in patients with ADPKD Have heart murmurs or signs or symptoms of cardiac dysfunction ⇒ assess by echocardiography
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Diverticula & Hernias Diverticula Colonic diverticula are found in many ADPKD patients on maintenance dialysis Symptoms include abdominal pain (which may be difficult to distinguish from the pain induced by the renal cysts), diarrhea, and heme-positive stools. Diverticular disease of the duodenum, presenting with nausea, vomiting, abdominal pain, malabsorption, bile or pancreatic duct obstruction, may also be associated with ADPKD Hernias Abdominal wall hernias found in 45% of patients with ADPKD Patients treated with continuous ambulatory peritoneal dialysis are also at increased risk for an indirect inguinal hernia
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Seminal Vesicle Cysts Seminal vesicle cysts are present in 40 percent of male ADPKD patients Rarely result in infertility Defective sperm motility is another cause of male infertility in ADPKD