Autosomal recessive polycystic kidney disease

Case contributed by Bahman Rasuli
Diagnosis almost certain

Presentation

34 weeks gestational age fetus with abnormality seen in routine obstetric ultrasound. Severe oligohydramnios (AFI<2cm). AC: 40 weeks. BPD, HC and FL:34 weeks

Patient Data

Age: 30 years
Gender: Female

Both kidneys are markedly enlarged and show increased parenchymal echo-pattern with decreased cortico-medullary differentiation. No stones or masses. No pelvicalyceal dilatation bilaterally.

The right kidney averages  8.5 x 5.6cm 

The left kidney averages 8.4 x 4.7 cm 

Diffuse bilateral renal tiny parenchymal cysts with increased parenchymal echo pattern, suggestive of autosomal recessive polycystic kidney disease.

 

Case Discussion

The findings are those of autosomal recessive polycystic kidney disease (ARPKD).

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