Prealbumin and retinol binding protein serum concentrations in the Indiana type hereditary amyloidosis.
Prealbumin and retinol binding protein serum concentrations in the Indiana type hereditary amyloidosis.
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印第安纳型遗传性淀粉样变性中前白蛋白和视黄醇结合蛋白的血清浓度。
DOI：
10.1002/art.1780261211
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发表时间：
1983
影响因子：
--
通讯作者：
Dwulet,FE
中科院分区：
文献类型：
--
作者：
Benson,MD;Dwulet,FE
测定了印第安纳州一个家族性系统性淀粉样变性家系的68名成员的血清前白蛋白和视黄醇结合蛋白（RBP）浓度。对患有这种类型淀粉样变性的个体的直肠和肌肉活检材料的免疫组织化学研究显示抗前白蛋白染色的淀粉样沉积物。9例淀粉样变患者血清前白蛋白和RBP浓度均显著低于正常对照组和正常对照组。此外，21例淀粉样变性患者的后代的平均RBP血清浓度显着降低。一个更重要的发现是，根据血清RBP浓度，后代可以分为2个不同的组。一组代表了大约50%的儿童，其血清前白蛋白和RBP浓度与其患病父母没有显著差异。第二组的血清前白蛋白和RBP浓度与正常对照组和非受累亲属无显著差异。这些发现表明，前白蛋白和RBP血清浓度在印第安纳州型遗传性淀粉样变性患者中是降低的，并且这些血清异常可能在临床疾病发展之前很久就存在。他们认为，具有这种遗传异常的个体可以在疾病的临床表现之前被识别。
Serum prealbumin and retinol binding protein (RBP) concentrations were determined for 68 members of a kindred in Indiana with a familial type of systemic amyloidosis. Immunohistochemical studies on rectal and muscle biopsy material from individuals with this type of amyloidosis revealed staining of amyloid deposits with anti‐prealbumin. Both the serum prealbumin and RBP concentrations were significantly depressed in 9 patients with amyloidosis when compared with normal controls and unaffected kin. In addition, the mean RBP serum concentration of 21 offspring of the patients with amyloidosis was significantly depressed. A more significant finding was that on the basis of the serum RBP concentrations, the offspring could be divided into 2 distinct groups. One group represented approximately 50% of the children and had serum prealbumin and RBP concentrations not significantly different from their afflicted parents. The second group had serum prealbumin and RBP concentrations not significantly different from those of normal controls and non‐affected kin. These findings show that prealbumin and RBP serum concentrations are depressed in patients with the Indiana type of hereditary amyloidosis and that these serum abnormalities may be present long before development of clinical disease. They suggest that individuals with this genetic abnormality may be identified prior to clinical expression of the disease.