Di Ferrante N, Nichols B L, Donnelly P V, Neri G, Hrgovcic R, Berglund R K
Proc Natl Acad Sci U S A. 1971 Feb;68(2):303-7. doi: 10.1073/pnas.68.2.303.
The effects of the administration of normal human plasma to patients affected by mucopolysaccharidoses I and II (Hurler's and Hunter's syndromes) have been evaluated. The infusion was followed by a decreased urinary excretion of relatively large molecular weight glycosaminoglycans and by an increased excretion of their products of degradation. Among the latter, products of the degradation of dermatan sulfate and heparan sulfate could be demonstrated. The results indicate that normal human plasma may contain those "factors" that are involved in the normal degradation of dermatan sulfate and heparan sulfate, that are missing in the diseased states.
已评估了向受黏多糖贮积症I型和II型（胡尔勒氏综合征和亨特氏综合征）影响的患者输注正常人血浆的效果。输注后，相对大分子糖胺聚糖的尿排泄减少，其降解产物的排泄增加。在后者中，可以证明硫酸皮肤素和硫酸乙酰肝素的降解产物。结果表明，正常人血浆可能含有参与硫酸皮肤素和硫酸乙酰肝素正常降解的那些“因子”，而这些因子在患病状态下缺失。
