Keshgegian A A, Chatten J
Arch Pathol Lab Med. 1979 Feb;103(2):73-5.
The autopsy records and material of 14 cases of karyotype-proved trisomy 13 were reviewed. There were one or more foci of nodular renal blastema in six cases. Patients with nodular renal blastema had lived up to five days; patients in whom foci were not found at autopsy had lived six to 37 days. The presence of nodular renal blastema did not substantially correlate with other renal anomalies or the number or severity of extrarenal anomalies. The presence of nodular renal blastema in trisomy 13 expands the spectrum of syndromes associated with nodular renal blastema and supports its relationship with disorders of growth regulation. The presence of the lesions only in very young infants suggests that most, or all, foci of nodular renal blastema regress with age.
回顾了14例经核型证实为13三体综合征患者的尸检记录及相关材料。6例患者存在一个或多个结节性肾胚基灶。有结节性肾胚基灶的患者存活了5天;尸检未发现病灶的患者存活了6至37天。结节性肾胚基灶的存在与其他肾脏异常或肾外异常的数量及严重程度无显著相关性。13三体综合征中结节性肾胚基灶的存在扩展了与结节性肾胚基灶相关的综合征谱,并支持其与生长调节紊乱的关系。这些病变仅在非常年幼的婴儿中出现,提示大多数或所有结节性肾胚基灶会随着年龄增长而消退。