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Year : 2003  |  Volume : 13  |  Issue : 2  |  Page : 232-233
Autosomal recessive polycystic kidney disease


Nanak Sonography Centre, 281, A.B. Road, Dewas (M.P), India

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How to cite this article:
Bapna P K, Dube S, Singh G, Jain A. Autosomal recessive polycystic kidney disease. Indian J Radiol Imaging 2003;13:232-3

How to cite this URL:
Bapna P K, Dube S, Singh G, Jain A. Autosomal recessive polycystic kidney disease. Indian J Radiol Imaging [serial online] 2003 [cited 2019 Nov 21];13:232-3. Available from: http://www.ijri.org/text.asp?2003/13/2/232/28667
Sir,

Autosomal recessive polycystic kidney disease is a rare condition seen in 1: 6000 to 1:16000 live births.[3] This syndrome on sonography presents as bilaterally enlarged, echogenic kidneys which maintain their smooth reniform shape. The increased echogenicity of the kidneys is due to innumerable ectatic collecting tubules which provide a multitude of reflective surfaces. Dilatation of renal collecting tubules is accompanied by hepatic cysts, bile duct proliferation and periportal fibrosis. [1]

A 20 Year old muslim woman presented for routine obstretic sonography with 7 months amenorrhoea. No previous sonography or investigations had been done. The sonogram showed a 30 weeks live intrauterine pregnancy with marked renal enlargement, echogenic kidneys with well maintained out line. [Figure - 1]

The fetal liver was normal and there was no sonographic evidence of any hepatic cysts or periportal fibrosis. The liquor was normal for gestational age and the bladder well distended. [Figure - 2]

The limbs of the fetus were normal with no obvious radial defect [Figure - 3]. The cardiac size and configuration was normal. The fetal face appeared grossly normal.No obvious cranio-spinal defect.

This condition presents with a wide spectrum ranging from minimal or no hepatic involvement to predominant hepatic or renal involvement. Renal failure is the predominant feature of the perinatal form where as hepatic involvement is a feature of childhood form. [1]

The prognosis of this condition depends on severity of renal involvement, amniotic fluid volume, preservation of renal out line and evaluation of the bladder. Oligohydramnious and failure to evaluate the bladder carry a poor prognosis. [1]

 
   References Top

1.Diagnostic Ultrasound Vol-2, Carol. M.Rumack ( Mosby 1991) Page 793.  Back to cited text no. 1    
2.Diagnostic Radiology "Urogenital Imaging" Jaypee 1996 Page 68.  Back to cited text no. 2    
3.The NICER Year book 1996 Page 371 Sonography.  Back to cited text no. 3    

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Correspondence Address:
P K Bapna
Nanak Sonography Centre, 281, A.B. Road, Dewas (M.P)
India
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Source of Support: None, Conflict of Interest: None


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    Figures

[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]



 

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