Autosomal dominant non-syndromic unilateral renal hypoplasia: A condition not previously reported in the literature
Aamir Jalal Al-Mosawi
Background: Non-syndromic unilateral renal hypoplasia is a congenital condition associated with one small kidney (hypoplastic) that generally has normal residual parenchyma but smaller calyces. A transmission of unilateral renal hypoplasia from a parent to offspring has never been described in the literature. Patients and Methods: A boy withunilateral renal hypoplasia whose mother also had the condition suggesting an autosomal dominant inheritance is described. Results: A boy whose mother known to have unilateral renal hypoplasia was hospitalized at about the age of 18 months because of a gastrointestinal infection presented with vomiting, diarrhea, dehydration, and an elevated urea level of 86 mg/dl. Serum creatinine was 2 mg/ dl. Correction of dehydration resulted in rather rapid lowering of blood urea to normal. However, abdominal ultrasound showed right renal hypoplasia. Conclusion: The first case of non-syndromic unilateral renal hypoplasia transmitted in an autosomal dominant fashion is reported.