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Nephrology > Cystic diseases of the kidneys > Flashcards

Cystic diseases of the kidneys Flashcards

1
Q

What is renal cystic disease?

A
  • characterized by epithelium lined cavities filled with fluid or semisolid debris within the kidneys
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2
Q

Diff cystic diseases of the kidney?

A
  • simple cyst
  • ADPKD
  • ARPKD
  • aquired cystic kidney disease
  • medullary cystic disorders
  • medullary sponge kidney
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3
Q

Simple cysts?

A
  • often asx and usually incidental findings during imaging studies
  • increase in frequency with age
  • present in up to 50% of pop over 50 yo
  • renal US, together with CT diff benign from malignant lesions in most instances
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4
Q

3 main criteria for cyst being benign?

A
  • echogenic free
  • finely demarcated (encircled)
  • enhanced back wall, good transition through cyst
  • calcifications, solid components, echogenicity all concerning - get CT
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5
Q

2 diff categories of polycystic kidney disease?

A
  • ADPKD: adult PKD
  • ARPKD: referred to infantile or childhood PKD
    abdominal mass in neonate or older infant suggestive
  • can occur with congenital fibrosis and causes death from renal failure during the first year of life
  • can be picked up on U/S at 20 weeks, progresses to ESRD early on
  • renal failure in adolescense suggested ARPKD or medullary cystic disease
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6
Q

What is adult polycystic kidney disease characterized by?

A
  • large cysts

- pts eventually die from renal failure or consequences of HTN (decreased filtration, balance of electrolytes are off)

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7
Q

Etiology of ADPKD?

A
  • msot common hereditary renal disease in US
  • affects over 500000 people
  • caused by 2 genes:
    ADPKD1: 85% of pts
    ADPKD2: 10-15%
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8
Q

When do clinical manifestations of ADPKD start?

A
  • rarely occur b/f 20-25 years, therefore affected people of childbearing age pass genetic trait on to offspring while they are still asx
  • pts present either from screening b/c of family hx, or for eval of sxs
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9
Q

Most common clinical sx of ADPKD?

A
  • acute abdominal flank pain
  • back pain
  • will have kidney stones, UTIs, HTN
  • hematuria (rupturing into renal pelvis): microhematuria at first, gross hematuria may occu
  • non-specific dull lumbar pain
  • sharp localized pain: from cyst rupture, infection or passage of renal stone
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10
Q

What are you worried about when you see blood in the urine?

A
  • cancer!!!

- ask about hx of smoking

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11
Q

What are common complications of ADPKD?

A

UTIs
pylonephritis: become septic quickly
cyst infections (fluoroquinolones)
HTN (ACEI)

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12
Q

What are common assoc with ADPKD?

A
  • mult asx hepatic cysts (30%)
  • MVP (25%), aortic valve abnormalities
  • cerebral aneurysms (10%)
  • diverticulosis
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13
Q

Disease progression of ADPKD?

A
  • renal fxn impairment is variable: 50% progress to ESRF by age 60
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14
Q

Associations of poor prognosis in ADPKD?

A
  • ADPKD1 gene
  • male
  • black race
  • HTN
  • early age of clinical presentation
  • episodes of gross hematuria
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15
Q

Dx of polycystic kidney disease?

A
  • RENAL U/S is the best!!!!
  • see mult cysts throughout parenchyma
  • renal enlargement
  • increased corticol thickness
  • elongation and splaying of renal calyces
  • bilateral renal involvement
  • CT: can show more cystic involvement than US, primarily used for diff when few cysts rae seen on US
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16
Q

Dx recommendations?

A
  • based on age and number of cysts present in one or both kidneys for dx in pts with family hx
  • extrarenal involvement, particularly hepatic cysts lend support to dx
17
Q

Dx criteria for ADPKD1?

A
  • at least 2 cysts in 1 kidney or 1 cyst in each kidney in an at risk pt younger than 30
  • at least 2 cysts in each kidney in an at risk pt aged 30-59
  • at least 4 cysts in each kidney for an at risk pt aged 60 and older
18
Q

Screening for ADPKD1?

A
  • asx carriers
  • expensive
  • requires cooperation of other family members
  • supplies no anatomic info
19
Q

Tx of ADPKD1?

A
  • aimed at preventing complications and preserving renal fxn:
    tx of HTN (ACEI)
  • management of infections: all of these penetrate cyst walls and attain therapeutic levels:
    bactrim, chloramphenicol, ciprofloxin
  • dialysis: for ESRF
  • transplant: for ESRF
  • bilateral nephrectomy prior to transplantation in pts with large kidneys or hx of frequent and persistent UTIs
20
Q

What is ARPKD?

A
  • sxs of autosomal recessive PKD can begin before birth
  • often called infantile PKD
  • children born with ARPKD usually develop kidney failure within a few years
  • severity of disease varies, babies with the worst cases die hours or days after birth
  • children with infantile version may have sufficient renal fxn for normal activities for a few years
  • people with juvenile version may live into their teens and 20s and usually will have liver problems as well
21
Q

Clinical presentation of infantile ARPKD?

A
  • hx of oligohydramnios (small amt or lack of amniotic fluid)
  • difficult delviery because of enlarged fetal kidneys
  • resp distress may be present
  • pneumomediastinum and pneumothorax are common
  • renal fxn usually compromised - hence the lack of amniotic fluid
22
Q

Sxs of ARPKD in children?

A
  • high BP
  • UTIs
  • frequent urination
  • disease usually affects liver, spleen, and pancreas, resulting in low blood cell counts, varicose veins, and hemorrhoids
  • because kidney fxn is cruicial for early physical development, children with ARPKD are usually smaller than average size
23
Q

How is ARPDK dx?

A
  • US imaging of fetus or newborn reveals cysts in kidneys but doesn’t distinguish b/t cysts of recessive or dominant PKD
  • US exam of relatives can be helpful, for ex - parent or grandparent with ADPKD cysts could help confirm dx of ADPKD in a fetus or child, it is extremely rare but not impossible for person with recessive PKD to become a parent
  • Because ARPKD tends to scar the liver, US imaging of liver also aids in dx
24
Q

How is ARPKD tx?

A
  • control of HTN
  • abx for UTIs
  • eating increased amts of nutritious food improves growth in children with ARPKD
  • in some cases GHs are used
  • in response to kidney failure, ARPKD pts must receive dialysis or transplantation
25
Q

Acquired cystic kidney disease?

A
  • development of cysts in pt with chronic renal failure or ESRD undergoing dialysis
  • carcinomas may complicate the disorder
  • annual screening after 3-4 years on dialysis to r/o malignancy
  • CT scan is dx of choice in acquired cystic kidney disease
26
Q

What are medullary cystic disorders?

A
  • part of a group of congenital tubulointerstitial nephropatheis known as juvenile nephronophthisis - medullary cystic disease complex
  • rare autosomal dominant disease
27
Q

Presentation of medullary cystic disorder?

A
  • anemia
  • nocturia (troubles with potty training)
  • prolonged childhood enuresis
  • low urine osmols
  • eye deformities
  • short stature
  • failure to thrive
  • low BP
  • eventually, signs of renal failure
28
Q

Progression of medullary cystic disorder?

A
  • results in ESRF during adolescence of early adulthood
29
Q

Dx of medullary cystic disorder?

A
  • cysts are really small: 1-2 mm

- radiography and bx are not always successful at detecting these small cysts

30
Q

Tx of medullary cystic disorder?

A
  • focus on controlling sxs, minimizing complications and slowing progression of the disease
  • because of loss of water and salt, the pt will need a liberal intake of both to avoid dehydration
  • tx HTN
  • tx renal failure sequelae
  • dialysis
  • transplant (preferred)
31
Q

What is medullary sponge kidney?

A
  • common, benign disorder
  • often detected incidentally on abdominal radiographs
  • manifests as a result of passage of renal calculus: 10% of pts with renal stones
  • Renal failure isn’t a feature of this condition
  • kidneys look large and spongey, kidney fxn is just fine
32
Q

Dx and tx medullary sponge kidney?

A
  • IVP: characteristic pattern of contrast filled cysts: bouquet of flowers or a bunch of grapes

tx:
UTIs, and renal calculus formation

33
Q

differences b/t ARPKD and ADPKD?

A
  • ADPKD: most common form, almost always bilateral, sxs typically develop in 40s
  • ARPKD: less common, begins in utero, and can lead to fetal and neonatal death, surviving infants have sig reduced life expectancy usually due to renal and hepatic failure

Nephrology (15 decks)

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