<unk>  is an autosomal recessive congenital disorder characterized by<unk>  fibrosis,<unk><unk>  and<unk>  dysgenesis. It is usually fatal soon after birth.An association with NPHP3 has been described. It was characterized in 1959.
Renal–hepatic–pancreatic dysplasia
Hemihyperplasia–multiple lipomatosis syndrome
Bile duct hamartoma
Rete tubular ectasia
Asplenia with cardiovascular anomalies
Multicystic dysplastic kidney
Annular pancreas
Multiple hamartoma syndrome