A COMPARISON OF THE EFFECTIVENESS OF CYSTEAMINE AND PHOSPHOCYSTEAMINE IN ELEVATING PLASMA CYSTEAMINE CONCENTRATION AND DECREASING LEUKOCYTE FREE CYSTINE IN NEPHROPATHIC CYSTINOSIS
A COMPARISON OF THE EFFECTIVENESS OF CYSTEAMINE AND PHOSPHOCYSTEAMINE IN ELEVATING PLASMA CYSTEAMINE CONCENTRATION AND DECREASING LEUKOCYTE FREE CYSTINE IN NEPHROPATHIC CYSTINOSIS
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DOI：
10.1203/00006450-198806000-00018
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发表时间：
1988-06-01
影响因子：
3.6
通讯作者：
SCHNEIDER, JA
中科院分区：
文献类型：
--
作者：
SMOLIN, LA;CLARK, KF;SCHNEIDER, JA
Cysteamine (.beta.-mercaptoethylamine, MEA) is currently used to treat children with nephropathic cystinosis. In this study MEA was compared to phosphocysteamine (MEAP), a phosphorothioester that tastes and smells better than MEA, with respect to its ability to elevate plasma MEA and deplete leukocytes of cystine. Studies were performed in six children with nephropathin cystinosis ranging in age from 1 to 10 yr. After equimolar oral doses of either MEA or MEAP plasma cysteamine was determined by sodium borohydride reduction followed by high performance liquid chromatography separation and electrochemical detection. Leukocyte cystine was measured before and 1 an 6 h after drug administration. Peak plasma MEA was obtained. Leukocyte cystine was measured before and 1 and 6 hr after drug administration. Peak plasma MEA was obtained 30 min to 1 h after a dose and was not significantly different when MEA (48.6 .+-. 10.7, mean .+-. SD) or MEAP (54.1 .+-. 20.2) was given. Significant plasma MEA concentrations were seen as early as 15 min after an oral dose, indicating rapid absorption. Analysis of vomitus indicated that hydrolysis of the phosphate group of MEAP occurs in the stomach. The percent decrease in leukocyte cystine content obtained with MEA administration (61.9%) was not significantly different from the decrease observed when MEAP was administered (65.3%). MEA and MEAP appear to be equally effective in their cystine-depleting properties.
