小儿佝偻病血中微量元素的观察

对７１例确诊为不同阶段的佝偻病患儿同时进行观察血液中微量元素锌、铜、铅以及血清钙、磷、碱性磷酸酶的变化。结果表明，佝偻病组的血铸、血钙、血磷均低于正常组，血铅、ＡＫＰ高于正常组，血铜则两组无大差异，但铜／锌比值正常组为０．２２±０．６３明显低于佝偻病组０．３６±０．５５，ｔ＝３．２０，Ｐ＜０．０５．各期佝偻病微量元素的变化与正常儿对比，在佝偻病的初期血磷略低于正常，血锌明显减少，碱性磷酸酶升高。在疾病的激期钙、磷、锌明显低于正常，碱性磷酸酶及铅升高，恢复期各元素基本恢复正常。     

佝偻病患者的全血微量元素分析

为探讨佝偻病与血微量元素的关系,检测了326例佝偻病患儿全血微量元素水平。结果显示,本组佝偻病40．1％的病例血锌低于第25百分位数值,病情严重组的血锌值明显低于病情较轻组,且有统计学意义,按全血锌百分位数值分布及结合资料,显示病情愈重低血锌病例愈多;病情较轻组的铁水平较病情严重组低,且有统计学意义,按全血铁百分位数值分布及结合资料亦显示,病情严重的佝偻病组铁在中位数以上的病例数较病情较轻的佝偻病组多,有统计学意义;本组佝偻病69．6％的病例血铅含量超过100μg/L,病情严重组的血铅值明显高于病情较轻组,且有统计学意义,按全血铅百分位数值分布结合资料,显示病情愈重高血铅的病例愈多。提示佝偻病的治疗需提高血锌水平及防治铅中毒。     

Hepatorenal tyrosinemia

In 1957 Sakai and Kitagawa in Japan reported the clinical and biochemical findings in a patient with tyrosinemia, tyrosyluria, liver cirrhosis, and renal rickets. Subsequently, reports were published from various countries of other patients with hepatorenal tyrosinemia (HRT). 4-Hydroxyphenylpyruvate dioxygenase deficiency was originally proposed as the cause of HRT. However, in 1977 Lindblad et al. found that succinylacetone, which accumulates in the serum and urine from patients with HRT, inhibits delta-aminolevulinic acid (ALA) dehydratase in vitro. They suggested that the primary enzyme deficiency in patients with HRT was fumarylacetoacetate hydrolase, and this was soon confirmed. Thus, the elucidation of the pathogenesis of this disease has led to the possibility that, if a reliable newborn screening method could be developed, the prognosis of these patients would be improved. Early treatment would require a diet low in phenylalanine and tyrosine, administration of 2-(2-nitoro-4-trifluoromethylbenzoyl)-1,3-cyclohexanedione (NTBC), and liver transplantation.     

佝偻病婴幼儿血微量元素测定及临床研究

为探讨婴幼儿佝偻病和血微量元素锌、铁、铜、钙、镁、铅、镉水平的关系,以2003-2004年在保健门诊就诊的佝偻病婴幼儿120例为研究组,随机选择同一时期进行常规保健的健康婴幼儿100例为对照组,用原子吸收分光光度法对两组小儿进行了血锌、铁、铜、钙、镁、铅、镉含量测定。结果表明,佝偻病组血锌(52.42±12.80)μmol/L,与对照组相比差异非常明显(P<0.001);血铁(7.21±2.5)μmol/L,血铅(65±18.23)μg/L,与对照组相比差异明显(P<0.05)。可见佝偻病婴幼儿多伴有锌、铁缺乏和血铅偏高,治疗的同时要注意补锌补铁,并要防治铅过量。     

155例佝偻病患者的血铅水平分析

为探讨佝偻病与血铅的关系，检测了120例中重度佝偻病患儿及35例亚临床型佝偻病病例的血铅水平。结果显示，本组86．5％的病例血铅含量超过100μg/L，血铅与血钙，磷，碱性磷酸酶无明显的相关关系，各期佝偻病在血铅分级上无明显差异。提示佝偻病需防治铅中毒。     

62例佝偻病患儿头发钙元素分析

为了解佝偻病患儿头发钙含量情况，将测得的62例佝偻病患儿发钙值进行了分析。结果表明，男女佝偻病患儿头发钙含量均高于正常值，其中男患儿头发钙与正常值相比，差异具有高度显著性意义(P＜0．01)；男女佝偻病患儿与体检儿头发钙比较，前者均高于后者，但无统计学意义；男女体检儿及男女佝偻病患儿之间头发钙含量差异无显著性。结果提示，佝偻病患儿发钙值并不比正常值低，相反还高于正常值，故不能简单地用发钙值高低来反映体内钙的营养状况。     

佝偻病与微量元素营养关系临床研究

通过５０例佝偻病儿头发中微量元素锌、铜、铁的检测，并与健康儿童对照研究，发现患儿有明显的缺锌（Ｐ〈０．００１），结果提示，缺锌对佝偻病的发生，发展起重要作用，佝偻病与缺锌密切相关。