Fiala P, Slugen I, Fukal J, Furková K
Katedry patológie ILF v Bratislave.
Bratisl Lek Listy. 1990 Jun;91(6):461-5.
Only one case of congenital nephrotic syndrome was recorded in a series of 2,500 bioptic examinations of the kidney performed over a period of 20 years. In necroptic material examined over the same period of time congenital nephrotic syndrome was recorded three times. At the first bioptic examination of the kidneys only minimal changes of glomeruli were diagnosed. Rebiopsy exhibited proliferation of mesangial cells, focal segmental glomerular sclerosis, cystically dilated tubules in the juxtamedular region of the cortex, and focal tubulointerstitial reactive changes, which in accordance with the clinical findings were assessed as congenital nephrotic syndrome of the Finnish type. Comparison of the findings observed at the first biopsy and at rebiopsy demonstrates the importance of proper collection of a representative sample for renal biopsy.
在20年期间进行的2500例肾脏活检检查中，仅记录到1例先天性肾病综合征。在同一时期检查的坏死组织中，先天性肾病综合征记录到3次。首次肾脏活检检查时仅诊断出肾小球有轻微变化。再次活检显示系膜细胞增生、局灶节段性肾小球硬化、皮质近髓区域肾小管囊性扩张以及局灶性肾小管间质反应性改变，根据临床 findings 评估为芬兰型先天性肾病综合征。首次活检和再次活检结果的比较表明了正确采集代表性肾脏活检样本的重要性。
