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血清IgG水平异常患者中放射性碘化IgG的代谢。II. 低丙种球蛋白血症。

Metabolism of radio-iodinated IgG in patients with abnormal serum IgG levels. II. Hypogamma-globulinaemia.

作者信息

Wells J V, Fudenberg H H

出版信息

Clin Exp Immunol. 1971 Dec;9(6):775-83.

Abstract

The metabolism of radio-iodinated IgG was studied in seven patients with IgG deficiency (<600 mg/100 ml). The group comprised two patients with primary hypogamma-globulinaemia, one with primary hypogamma-globulinaemia and nodular lymphoid hyperplasia, and one each with chronic lymphatic leukaemia (CLL), γG multiple myeloma, renal homotransplantation and the nephrotic syndrome. All patients had markedly reduced plasma and total body pools of normal IgG with normal distribution (intravascular and extravascular pools). An increase in the initial urinary excretion of free isotope in one patient was attributed to a degree of protein denaturation. Compared to the normal range of 20–60 mg/kg/day for IgG synthesis, the patients with primary deficiency and the patient with CLL all had markedly reduced synthesis of normal IgG (2, 5, 4 and 7 mg/kg/day respectively). These patients also had a prolonged or normal plasma T½ and a normal or decreased fractional turnover rate (FTR). The patients with deficiency of normal IgG secondary to multiple myeloma, renal homotransplant or nephrotic syndrome all had an IgG synthesis rate at the lower limit of the normal range with values of 19, 21 and 19 mg/kg/day respectively. They had increased catabolism with a short plasma T½ and increased FTR. An assessment of the factors controlling the metabolism of normal IgG is important in the individual patient as regards the management of their recurrent infections with therapeutic human γ-globulin.

摘要

对7例IgG缺乏(<600mg/100ml)患者的放射性碘化IgG代谢情况进行了研究。该组包括2例原发性低丙种球蛋白血症患者、1例原发性低丙种球蛋白血症合并结节性淋巴样增生患者,以及慢性淋巴细胞白血病(CLL)、γG多发性骨髓瘤、肾同种移植和肾病综合征患者各1例。所有患者正常IgG的血浆和全身池均显著减少,但分布正常(血管内池和血管外池)。1例患者游离同位素的初始尿排泄增加归因于一定程度的蛋白质变性。与IgG合成的正常范围20 - 60mg/kg/天相比,原发性缺乏患者和CLL患者正常IgG的合成均显著减少(分别为2、5、4和7mg/kg/天)。这些患者的血浆半衰期也延长或正常,分数周转率(FTR)正常或降低。继发于多发性骨髓瘤、肾同种移植或肾病综合征的正常IgG缺乏患者的IgG合成率均处于正常范围下限,分别为19、21和19mg/kg/天。他们的分解代谢增加,血浆半衰期缩短,FTR增加。对于个体患者而言,评估控制正常IgG代谢的因素对于使用治疗性人γ球蛋白治疗其反复感染的管理非常重要。

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Pediatrics. 1952 Jun;9(6):722-8.

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