Cystic Kidney Dz Flashcards
Renal Cystic Dz is characterized by what?
-epithium-lined cavities filled with fluid or semi-solid debris
List the 6 cystic dz of the kidney
Simple Cyst
Autosomal Dominant Polycystic Kidney Dz
Autosomal Recessive Polycystic Kidney Disease
Acquired Cystic Kidney Disease
Medullary Cystic Disorders
Medullary Sponge Kidney
Simple Cysts
-sx
- occurrence
- dx
Sx: asymptomatic, usual incidental finding
Occurrence: increased frequency with age
Dx: renal ultrasound together with CT scan to differentiate benign from malignant lesions
What three criteria do we look at in determining benign or malignant?
- echo free?
- sharply demarcated?
- enhanced back wall, tells you there is good transmission through the cysts.
*calcification, solid components, and mixed echogenicity are always worrisome on echo.
Autosomal Dominant Polycystic Kidney Disease (ADPKD)
- affects who?
- characterized by
- pts die from
- cause
- symptomatic at what age? consequences of this?
Affects: older adults (greater than 25)
Characterized by large cysts
Patients eventually die from renal failure or consequences of HTN.
Caused by ADPKD 1 (85%) and ADPKD 2 (15%)
Sx rarely occur before age 20-25, affected people of childbearing age pass the genetic trait on to offspring while they are still asymptomatic.
Autosomal Dominant Polycystic Kidney Disease (ADPKD):
- sx
- complications
- common associations
Sx:
-acute abdominal flank pain
- back pain
- hematuria
- non-specific dull lumbar pain
- sharp localized pain (from cyst rupture, infection or passage or renal stone)
Complications:
- Urinary tract infections
- pylonephritis
- cyst infections
- HTN
Associations:
-multiple asymptomatic hepatic cysts
- mitral valve prolapse
- cerebral aneurysms
- diverticulosis
Autosomal Dominant Polycystic Kidney Disease
- Progression of dz
- dx
- Diagnostic criteria
Progression:
renal function impairment is variable.
-50% progress to ESRF by 60yrs
Dx:
-renal ultrasound is best
-CT (gives better ;look at cyst)
dx Criteria:
-at least 2 cyst in 1 kidney or 1 cyst in each kidney in an at risk pt younger than 30 yearas
- at least 2 cysts in each kidney in an at risk patient aged 30-59yrs
- at least 4 cysts in each kidney for an at risk patient older than 60 (this is diagnostic)
What do you see on renal ultrasound of ADPKD
multiple cysts through parenchyma
renal enlargement
increased coritcal thickness
elongation and splaying or renal calyces
Bilateral renal involvment
ADPKD
- screening
- tx
Screening:
- asymptomatic carriers
- expensive
Tx:
-Tx HTN
- Manage infection:
- -bactrim, chloramphenicol, ciprofloxin
- Dialysis for ESRF
- Transplant for ERSF
- Bilateral nephrectomy prior to transplantation in pts w/ large kidneys or hx of frequent and persistent UTIs.
Autosomal Recessive Polycystic Kidney Disease:
- when do the sx appear?
- aka
- disease progression
- sx can begin before birth.
- often called infantile PKD
Progression:
-usually develop kidney failure within a few years, severity of dz varies…babies with worst cases die hours or days after birth.
- children w/ infantile version may have sufficient renal function for normal activities for a few years
- those with juvenile version may live into their teens and twenties and usually have liver problems as well.
ARPKD
- clinical presentation at birth
- sx
Presentation:
- Hx of oligohydramnios (unusually small amount or lack of amniotic fluid)
- difficult delivery d/t enlarged fetal kidneys
- respiratory distress may be present
- pneumomediastinum and pneumothorax are common
- renal function is usually compromised
Sx:
-HTN
- Urinary Tract Infections
- Frequent urination
- low blood cell counts, varicose veins, and hemorrhoids
- smaller than average size
ARPKD
- how is it diagnosed
- Tx
Dx:
- ultrasound imaging of fetus/newborn can reveal cysts but cannot distinguish between ARPDK and ADPKD.
- genetic testing
Tx:
-control HTN
- Abx for UTI
- eating increased amounts of nutritious food improves growth in children with ARPKD
- GH
- dialysis or transplant
Acquired Cystic Kidney Dz
- cause
- complications
- screening
- diagnostics
Cause: development of cyst in pt with chronic renal failure or ESRD undergoing dialysis
Complication: Carcinomas may complicated disorder
Screening: annual screening after 3-4yrs on dialysis to r/o malignancy
Diagnostics:
-CT*** (Dx Of Choice)
Medullary Cystic Disorders
-what is this
- presentation
- course of dz
What: part of a group of congenital tubulointerstitial nephropathies known as juvenile nephronophthisis.
Presentation:
-anemia
- nocturia
- prolonged childhood enuresis
- low urine osmols
- eye deformities
- short stature
- failure to thrive
- low BP
- Signs of Renal Failure
Course:
-results in ESRF during adolescence or early adulthood.
Medullary Cystic Disorders
- diagnostics
- tx
Dx:
- small cyst 1-2mm
- radiography and bx are not always successful at detecting these small cysts
Tx:
-control sx, minimize complications, slow progression
- d/t loss of salt and water the pt will need liberal intake of both.
- treat HTN
- Treat renal failure
- Dialysis
- Transplant (preferred)
