cystic kidney dse

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cystic kidney dse

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Polycystic kidney dse:

Polycystic kidney dse Dr Mohammed Abdelsattar

ADPKD:

ADPKD ADPKD is the most common inherited cystic kidney disease progressive enlargement of kidney cysts and typically leads to renal failure by middle age .

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People who have inherited ADPKD often do not notice anything unusual until they are 30 to 40 years old. The first noticeable symptoms may include: ❖ high blood pressure ❖ back or side pain ❖ increase in the size of the abdomen ❖ blood in the urine ❖ frequent bladder or kidney infections

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> U/S is I most reliable method for detecting PKD > CT scan (computed tomography scan) may detect smaller cysts that cannot be found by an ultrasound. using genetic testing to determine whether your DNA contains either the PKD1 or PKD2 mutations that cause ADPKD

Extrarenal manifestations:

Extrarenal manifestations > Polycystic liver disease and hepatic cysts Cysts of the seminal vesicles, pancreas, and arachnoid Membrane >Intracranial aneurysms >Mitral valve prolapse >Colonic diverticulosis and diverticulitis >Abdominal hernia

U/S criteria of ADPKD:_:

U/S criteria of ADPKD:_

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Hematuria : Both microscopic and macroscopic hematuria are common Cyst hemorrhage occurs in ~60% of individuals

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Nephrolithiasis : About 20% of patients with ADPKD have kidney stones. The composition of these is typically uric acid or calcium oxalate

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Urinary tract infection : Most are caused by Enterobacteriaceae . . Cyst aspiration should be considered when the clinical setting and imaging are suggestive and blood and urine cultures are negative. Quinolone antibiotics (e.g., ciprofloxacin or levofloxacin ) are usually the antibiotics of choice because they are effective in treating gram-negative organisms and accumulate in cysts. Trimethoprim-sulfamethoxazole is an alternative.

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Pain: Pain is the most common symptom (around 60%) Most often it is attributed to compression of surrounding structures by expanding cysts, but it can also be caused by hemorrhage into a cyst, cyst rupture, or infection; stones or tumor should be excluded

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ESRD: late outcome of patients with ADPKD; 45% of patients with ADPKD develop renal insufficiency by 60 years of age and progress to dialysis or renal transplantation.

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Certain people have an increased risk of kidney failure. They include: ❖ men ❖ patients with the most common form of PKD (ADPKD) ❖ patients with high blood pressure ❖ patients with protein or blood in their urine ❖ women with high blood pressure who have had more than three pregnancies.

When to screen for intracranial aneurysms:

When to screen for intracranial aneurysms >in patients with a strong family history of ruptured intracranial >Aneurysm especially age (20-50y) >high-risk occupations (e.g., pilots) >patient with known ADPKD who has new-onset or severe headache

Autosomal-recessive polycystic renal disease:

Autosomal -recessive polycystic renal disease >combinations of bilateral renal cystic disease and congenital hepatic fibrosis >presents in infancy and childhood >ARPKD is generally considered to be the infantile form of polycystic kidney disease, >Appear early in life (ADPKD appear late in life)

ttt:

ttt Treatment is primarily supportive care Control of HTN is important to slow disease progression ACE-I, ARB Treating complications of renal failure Hyperkalemia , hyperphosphatemia , hypocalcemia , acidosis Treating UTI and renal cyst infections Avoidings nephrotoxic agents (NSAIDS)

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