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15 Cards in this Set
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Childhood polycystic kidney disease (PCKD) :
***is what kind of Gentic Dz |
autosomal recessive Dz
mapped to the short arm of = |
chromosome 6
(HLA chromo 6) |
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In childhood PCKD, the kidneys are =
3ct |
huge
spongy contain numerous cysts caused by = |
dilatation of collecting ducts and tubules that severely compromise kidney function.
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***Childhood PCKD is associated clinically with =
3ct |
cysts of the =
-liver -pancreas -lungs Treatment includes = 2ct |
dialysis
kidney transplant. |
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Which is less common?
(ARPKD) autosomal recessive polycystic kidney Dz or (ADPKD) autosomal dominant polycystic kidney Dz |
Autosomal recessive (ARPKD)
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Polycystic kidney disease
Diagnosis made by prenatal ultrasound or in early infancy with abdominal masses and renal failure. |
End-stage renal failure develops in childhood
Patients also typically have liver involvement for example = |
Portal and interlobular fibrosis
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ADP KD
is the most common or least common kidney disease = |
Most common inherited cause of kidney disease.
In Caucasians the of rate is = |
One\1000 Caucasians
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ADPKD
has two disease Loci = Autosomal dominant |
PKD 1 coded for =
PKD 2 coded for = |
Polycystine-1
Polycystine-2 Ultrasound is the screening investigation for relatives. |
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Ultrasound diagnostic criteria in patients with positive family history =
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- <30 yo = 2 cysts unilateral or bilateral
- 30-59 yo = 2 cysts in both kidneys - >60 yo = 4 cysts and both kidneys |
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A.D. PKD
ultrasound criteria for positive family history age brackets |
- <30 yo =
- 30-59 yo = - >60 yo = |
2 cysts unilateral or bilateral
2 cysts in both kidneys 4 cysts and both kidneys |
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***It is estimated that up to 8% of patients with adult polycystic kidney disease have =
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cerebral aneurysms.
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***It is estimated that up to 8% of patients with _______ have cerebral aneurysms.
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adult polycystic kidney disease
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***It is estimated that up to __% of patients with adult polycystic kidney disease have cerebral aneurysms.
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8%
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(tend to have HTN)***
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adult polycystic kidney disease
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adult polycystic kidney disease Pt tend to have what Dys Fctn =
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(tend to have HTN)***
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First line Tests is For AKPD =
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Ultra Sound ***
+ |
Genetic testing is possible, but this is costly and is not used as a first-line screening tool.
Its utility is mostly in evaluating patients who volunteer to be live kidney donors, with a family history of AKPD but negative imaging studies. |
