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06-05-2009, 09:51 AM
Cong. Polycystic kid



ªHereditary disease Ch. Ch. By presence of multiple cysts.
Infantile rare, aut. R. , ♂ = ♀
Adult type commoner, pres in middle age autosomal
dominant ♂ = ♀

Path.
Both kid, are enlarge +
Multiple cysts compressing renal parenchyma.

Cysts are not communicating to each other.
C/P
1- Adult type 80 / 100.000
age > 40 yrs.
Dull aching pain.
Haematuria.
Hypert. - C.R.F.
2- Infantile type still birth with difficult labour
(huge size of kid)
infant may live for few months & then
die from uremia.
O/E §++ kid - surface nodular.
Other cong anomalies such as polycystic liver.

D.D. Hydronephrosis.
Pyonephrosis.
Tumour.
Solitary cyst.

Diag. 1I.V.P. : spider leg appearance (calyces are elongated &
attenuated)
1 Sonar.

There is a gene for the adult type located on the short arm of
chromosome N = 16.
ttt 1- Hypert., infection , renal failure.
Screening by U/S & DNA analysis to detect asympt. Cases of relatives.
Counseling for those > 18 yrs.
Source: Internal Medicine Book of Dr.Osama Mahmoud (Ain Shams University)