Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019270 (hernia)
 15,856 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Although asymptomatic in most patients, extrarenal manifestations of ADPKD may become more clinically relevant with the increasing life expectancy of affected patients. They mainly encompass cysts in other organs than the kidney (liver: 94%, seminal vesicle: 40%, pancreas: 9%, arachnoid membrane: 8%, and spinal meningeal, 2%) and connective tissue abnormalities (mitral valve prolapse: 25%, intracranial aneurysms: 8%, and abdominal hernia: 10%). Their recognition may spare the patient from other, useless investigations (eg, when an arachnoid cyst is incidentally found) or lead to the implementation of prophylactic or therapeutic measures (eg, screening, sometimes followed by the treatment of an asymptomatic intracranial aneurysm in at-risk patients, or, in the presence of a severe polycystic liver disease, avoidance from estrogens and treatment aimed to slow cyst growth).
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PMID:Extrarenal manifestations of autosomal dominant polycystic kidney disease. 2021 20

Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited renal disease, with 50-75% of these patients requiring renal replacement therapy (RRT). The outcome of peritoneal dialysis (PD) in ADPKD with end-disease renal disease (ESRD) is not clearly defined, more so in developing countries. We conducted a retrospective analysis of the outcomes and economics of PD in these ESRD patients and compared them with other causes of ESRD on PD. Data were reviewed of all the PD patients who were followed-up at our institute from January 2007 to December 2011. The inclusion criteria were ADPKD patients who chose PD as the dialysis modality (Group 1), while age and gender-matched ESRD (other than ADPKD) patients who were started on PD during the same period were considered as the other group (Group 2). A total of 26 ADPKD patients underwent PD with an average size of kidneys among ADPKD ESRD patients of 15.2 + 2.1 cm. The overall peritonitis rates were similar among the compared groups. The median survival for the first peritonitis episodes were 1.2 and 1.8 years (95% confidence interval 0.82-1.91) for the control and ADPKD groups, respectively. The overall patient survival was 22 among PKD while five patients died among the control group. Among PKD, one patient died due to intra-cerebral bleed while one patient had severe cyst hemorrhage and infection, while three others had peritonitis and sepsis. Hernia was observed in four ADPKD patients, once on PD that was surgically corrected and PD was resumed in all. Two patients lost the catheter due to peritonitis while one patient had membrane failure while one underwent surgical exploration due to diverticulosis. PD treatment was not prevented by voluminous kidneys in any of these patients and no patient ceased PD treatment due to insufficient peritoneal space. Besides this, the cost on PD was much less as compared with that on hemodialysis (HD). PD is a reasonable mode of RRT among ADPKD, where HD is not possible or contraindicated with lesser risks to bleeding and infections, and the cost benefit favoring PD in general.
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PMID:Is CAPD a viable option among ADPKD with end stage renal disease population in India? Its outcomes and economics. 2635 61

Autosomal dominant polycystic kidney disease (ADPKD) is the most commonly inherited renal disorder and the fourth most common cause of end-stage renal disease. ADPKD is a systemic disease with multiple extrarenal manifestations, including cystic involvement of other organs, such as the liver and pancreas, and connective tissue abnormalities. The prevalence of hernias is higher in patients with ADPKD. It has been hypothesized that these hernias are the result of abnormal extracellular matrix production and/or increased intra-abdominal pressure from the cyst burden. We present a case of a 56-year-old female with polycystic kidney disease who was admitted for an incarcerated ventral hernia. The patient presented with obstructive symptoms concerning for bowel impingement. The patient underwent operative management, and during the procedure, an incarcerated liver cyst was identified in the hernia sac. This was successfully reduced, and the hernia was repaired with mesh.
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PMID:A Case Report of a Ventral Hernia Containing a Liver Cyst in a Patient with Autosomal Dominant Polycystic Kidney Disease. 3196 37