Anand S K, Chan J C, Lieberman E
Am J Dis Child. 1975 Jul;129(7):810-3. doi: 10.1001/archpedi.1975.02120440036008.
Renal function studies were done in five children with infantile polycystic disease (IPCD)of kidneys and liver and in four with congenital hepatic fibrosis (CHF). Glomerular filtration rate was reduced in all IPCD patients and in two of four CHF patients. Urinary concentrating ability following water deprivation and vasopressin administration was impaired in all IPCD patients and in three of four CHF patients. During control period, all patients had asymptomatic metabolic acidosis with total carbon dioxide content less than or equal to 20.5 millimols/liter, and net acid excretion (NAE) was reduced in all but one. Ammonium chloride was administered to seven patients; NAE increased in all, but the increments were subnormal in four. The inability to excrete maximally concentrated urine and an adequate amount of net acid may best be explained by abnormal tubular structure or alterations in medullary architecture secondary to progressive scarring, or both.
对5例患有肝肾婴儿型多囊病（IPCD）的儿童和4例先天性肝纤维化（CHF）患者进行了肾功能研究。所有IPCD患者以及4例CHF患者中的2例肾小球滤过率降低。禁水和给予血管加压素后，所有IPCD患者以及4例CHF患者中的3例尿浓缩能力受损。在对照期，所有患者均有无症状代谢性酸中毒，总二氧化碳含量小于或等于20.5毫摩尔/升，除1例外所有患者的净酸排泄（NAE）均降低。对7例患者给予氯化铵；所有患者的NAE均增加，但4例患者的增加低于正常水平。不能排出最大浓缩尿和足够量的净酸，最好用肾小管结构异常或进行性瘢痕形成继发的髓质结构改变来解释，或两者兼而有之。
