婴儿型先天性多囊肾如伴有肺发育不全,或有内眦赘皮、耳低位,四肢畸型,称为Pottcr综合征。它是常染色体隐性遗传病,国内少见报道,日本报道3例,现报告1例。 一、临床资料 患儿,男,早产,因新生儿肺炎血气呈代谢性酸中毒,于1986年9月15日转入我院。一般尚可,吃奶好,尿量正常。体检:体重2.2kg,身长50cm,发育营 Infantile congenital polycystic kidney disease such as associated with lung hypoplasia, or epicanthus, low ear, limb deformities, known as Pottcr syndrome. It is autosomal recessive disease, rare in China, reported in Japan in 3 cases, 1 case is reported. First, the clinical data Children, men and premature birth, neonatal pneumonia was metabolic acidosis, on September 15, 1986 into our hospital. Generally acceptable, good milk, urine output is normal. Physical examination: weight 2.2kg, body length 50cm, development camp