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What are the renal manifestations and clinical features of p

admin TAGS: 2017-10-10 17:50

Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease and is the fourth most common cause of end-stage renal disease. ADPKD is a systemic disease that can manifest a variety of extra-renal manifestations, significantly affecting patient quality of life, morbidity and mortality. This article provides a systematic overview of ADPKD's gastrointestinal manifestations.

ADPKD renal manifestations

What are the renal manifestations and clinical features of polycystic kidney disease?
What are the renal manifestations and clinical features of polycystic kidney disease? 

Polycystic liver disease

Liver cysts are the most common manifestations of ADPKD, the incidence of patients aged 15-24 is 58%, and the incidence of patients over 35 years of age is as high as 94%. Female more common, and female liver cyst volume is greater than men (5.27 ml vs 1.94 ml). The number of cysts and the volume of cysts increased with age, and the size of the liver increased by 0.9% -3.2% per year.

About 20% of patients with polycystic liver disease (PCLD) may have clinical symptoms. Common symptoms are: bloating, early satiety, nausea, vomiting, esophageal reflux, difficulty breathing and mechanical low back pain. Uterine prolapse, Budd-Chiari syndrome, inferior vena cava, portal vein and bile duct-related symptoms are rare.

Cyst itself can also occur infection, reverse, rupture, bleeding, cystadenoma or cystadenocarcinoma. The incidence of cyst infection was 3%, and the diagnostic gold standard was cystic culture and / or neutrophil positive. The typical performance of patients with fever, leukocytosis, right upper abdominal pain.

Liver cysts, size classification

Type 1: large cysts of the liver (10 cm) <10; type II: liver parenchyma diffuse a number of medium size cysts, but still retains most of the normal substance; type III: a large number of small cysts full of the whole, only minimal Partial normal substance.

Diverticular disease

Studies have shown that ADPKD is associated with perticulitis in patients with ERSD; in addition, ADPKD secondary to ESRD patients with increased risk of perticular disease. The incidence of perticular disease in patients with ADPKD who received renal replacement therapy (RRT) was 40%, compared with 0% for patients with non-ADPKD.

hernia

ADPKD patients with higher incidence of hernia, retrospective studies have shown that ADPKD ESRD patients with hernia incidence of 45%, while the same age, same sex non-ADPKD ESRD patients with hernia incidence was only 16%. ADPKD patients with inguinal hernia (P <0.001), incisional hernia (P = 0.019) and umbilical hernia (P = 0.007) were more common. In addition, ADPKD peritoneal dialysis patients with higher risk of hernia: ADPKD peritoneal dialysis male patients with bilateral inguinal hernia incidence rate of up to 46.2%, while non-ADPKD peritoneal dialysis patients only 3%.

Bile duct dilatation

A number of existing cases reported that ADPKD patients may have Caroli disease - multiple intrahepatic bile duct dilatation. The study found that 40% of ADPKD patients with CT scan shows common bile duct dilatation (defined as diameter> 7 mm), but not ADPKD patients only 9%.

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