LOW PROLINE DIET IN TYPE-I HYPERPROLINAEMIA
LOW PROLINE DIET IN TYPE-I HYPERPROLINAEMIA
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DOI：
10.1136/adc.46.245.72
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发表时间：
1971-01-01
影响因子：
5.2
通讯作者：
WOLFF, OH
中科院分区：
文献类型：
--
作者：
HARRIES, JT;PIESOWICZ, AT;WOLFF, OH
1型高脯氨酸血症的诊断是在一个7个月大的婴儿谁提出了低钙性惊厥和吸收不良。血浆脯氨酸水平明显升高，尿中脯氨酸、羟脯氨酸、甘氨酸排泄量增加；神经发育迟缓，伴有脑电图、肾道和骨骼异常。在9月龄时限制饮食中的脯氨酸导致血浆脯氨酸水平迅速降至正常水平，并且持续低脯氨酸饮食直到27月龄，这时显示出持续的生化缺陷。饮食治疗期间，生长良好，智力发育改善，脑电图、肾、骨、肠异常消失。脯氨酸应被视为生长中的婴儿的“半必需”氨基酸。
A diagnosis of Type I hyperprolinaemia was made in a 7-month-old infant who presented with hypocalcaemic convulsions and malabsorption. The plasma levels of proline were grossly raised and the urinary excretion of proline, hydroxyproline, and glycine was increased; neurological development was delayed and there were associated abnormalities of the electroencephalogram, renal tract, and bones. Restriction of dietary proline at the age of 9 months resulted in a prompt fall of plasma levels of proline to normal, and a low proline diet was continued until the age of 27 months when persistence of the biochemical defect was shown. During the period of dietary treatment, growth was satisfactory, mental development improved, and the electroencephalogram, and the renal, skeletal, and intestinal abnormalities disappeared. Proline should be regarded as a `semi-essential' amino acid in the growing infant.