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Using specially trained instructors, the PKDS programme switches between the classroom and an outdoor mini track where children "drive" Porsche pedal cars and face traffic lights, road signs and zebra crossings.
In many reports genetics are not clear, but there is a strong suggestion of autosomal dominant inheritance, (31) either representing type I (GCK in PKD) or type II (GCKD).
Seven of our 20 cases (35%) were diagnosed as such only retrospectively; 5 were ARPKD (cases 1-5) and 2 were ADPKD (case 6 and 7), demonstrating that PKD is the most important entity in the differential diagnosis of GCK.
A renal biopsy revealed diffuse glomerulocystic change and the PKD workup was initiated but was complicated by the fact that the girl was adopted.
Overall, renal dysplasia is a common finding in GCK and in our series was the second most frequent diagnosis after PKD. Dysplastic kidneys are often cystic and represent an important differential diagnosis of GCK, known as multicystic dysplastic kidney (MCDK).
(89) Absence of cysts in other organs and/or concurrent hepatic fibrosis helps exclude classic PKD. (85,88,190)
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- PKE protein kinase