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ADPKD sturcture

Polycystic Kidney Disease (PKD)

Polycystic Kidney Disease (PKD)

Polycystic kidney disease (PKD) is an inherited disorder in which clusters of cysts develop primarily within your kidneys. Cysts are noncancerous round sacs containing water-like fluid. The cysts vary in size and, as they accumulate more fluid, they can grow very large.

Although kidneys usually are the most severely affected organs, polycystic kidney disease can cause cysts to develop in your liver and elsewhere in your body. The disease causes a variety of serious complications.

Shape and size of the Kidney

Structure of the normal kidney to compare with Polycystic Kidney Disease (PKD)

Types of Polycystic kidney disease (PKD) 

There are two types of polycystic kidney diseases.That includes         

         1. Autosomal dominant Polycystic Kidney disease (ADPKD)

        2.  Autosomal Recessive Polycystic Kidney Disease (ARPKD)

        Rare and usually fatal before 1 year of life.

Autosomal dominant polycystic kidney disease (ADPKD). Signs and symptoms of ADPKD often develop between the ages of 30 and 40. In the past, this type was called adult polycystic kidney disease, but children can develop the disorder.

ADPKD sturcture

Image of Autosomal dominant Polycystic kidney disease(PKD)

Only one parent needs to have the disease in order for it to pass along to the children. If one parent has ADPKD, each child has a 50 percent chance of getting the disease. This form accounts for about 90 percent of cases of polycystic kidney disease.

Autosomal recessive polycystic kidney disease (ARPKD). This type is far less common than is ADPKD. The signs and symptoms often appear shortly after birth. Sometimes, symptoms don’t appear until later in childhood or during adolescence.

ARPKD

Sturucture of Autosomal Recessive Polycystic Kidney Disease (ARPKD) Polycystic Kidney Disease (PKD)

Both parents must have abnormal genes to pass on this form of the disease. If both parents carry a gene for this disorder, each child has a 25 percent chance of getting the disease.

Clinical features of ADPKD

Pain in the renal angles, haematuria, headache

Gastronitestinal complaints

Nocturia, frequently, polyuria

Slowly developing hypertension

Both kidneys palpated and surface nodular

Uremia in late stages

Other systemic involvement in ADPKD

  1. Genetourinary

Hypernephroma, renal calculi, ovarian cyst

        B. Gastro intestinal

Hepatic cysts, pancreatic cysts, diverticuli

       C. C.V.S

Hypertension, aortic and mitral valve abnormalities, thoracic aortic aneurysm

       D. Central Nervous System

Berry aneursms (Subarachnois haemorrahage)

       E. Haematopoietic

Erythrocytosis

Diagnosis of Polycystic Kidney Disease (PKD)

Clinical features and family history of polycystic disease.

Renal ultra sonogrphy : Demonstrates bilateral renal cysts; follow up renal ltrasound in 2-3 years may show increasing renal cystic involvement.

Total abdominal ultrasonography : may be done to demonstrate hepatic, pancreatic, ovarian cysts.

CT Scan of abdomen

Treatment for PKD

Haematuria : Bed rest, analgesics, hydration

Bladder and renal parenchymal infection,hypertension, subarachnois haemorrahage

Avoiding renal trauma.

Complications of Polycystic Kidney Disease

A common complication of polycystic kidney disease is high blood pressure. Kidney failure is another common problem for people with polycystic kidney disease.

Polycystic kidney disease varies greatly in its severity, and some complications are preventable. Lifestyle changes and medical treatments may help reduce damage to your kidneys from complications, such as high blood pressure.

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