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急性低磷酸酶血症

Acute hypophosphatasemia.

作者信息

McKiernan F E, Shrestha L K, Berg R L, Fuehrer J

机构信息

Center for Bone Disease, Marshfield Clinic, 1000 North Oak Avenue, Marshfield, WI, 54449, USA,

出版信息

Osteoporos Int. 2014 Feb;25(2):519-23. doi: 10.1007/s00198-013-2447-x. Epub 2013 Aug 3.

Abstract

UNLABELLED

The temporal evolution of a low serum alkaline phosphatase value may relate to its cause. Precipitous lowering of serum alkaline phosphatase below the lower range of normal is uncommon and may indicate severe physiologic stress and increased short-term mortality.

INTRODUCTION

The differential diagnosis of a low serum alkaline phosphatase (ALP) value (hypophosphatasemia) is wide ranging, anecdotal, and unfamiliar. The temporal evolution of hypophosphatasemia may relate to its cause. The purpose of this study is to report conditions and circumstances associated with precipitous lowering of serum ALP below the lower range of normal.

METHODS

Marshfield Clinic IRB approved use of their electronic medical record to search for subjects with at least two serum ALP values ≤ 40 U/L (normal 40-125 U/L). When the temporal evolution of the qualifying ALP values indicated a precipitous lowering from usually normal serum ALP values, the subject was deemed to have acute hypophosphatasemia. Thirty years of laboratory data and 10 years of clinical narrative were analyzed. Associated diagnoses, clinical circumstances, and short-term mortality were recorded.

RESULTS

A total of 458,767 subjects had 2,584,051 serum ALP values, and 5,190 (1.1 %) subjects had at least two serum values ≤ 40 U/L. A detailed review of 1,276 subjects selected on the basis of their lowest ALP value and age identified 190 subjects with acute hypophosphatasemia. Acute hypophosphatasemia was recorded during periods of major trauma/surgery, multisystem failure, acute anemia, blood product transfusions (often massive), apheresis, hypomagnesemia, and acute caloric restriction. Twenty-eight subjects (15 %) died within 35 days of their nadir serum ALP.

CONCLUSION

Acute hypophosphatasemia is associated with profound illness or physiologic stress and followed by increased short-term mortality. The temporal evolution of hypophosphatasemia may relate to its cause.

摘要

未标注

血清碱性磷酸酶值的时间演变可能与其病因有关。血清碱性磷酸酶急剧降至正常范围下限以下的情况并不常见,可能表明存在严重的生理应激和短期死亡率增加。

引言

血清碱性磷酸酶(ALP)值低(低磷酸酶血症)的鉴别诊断范围广泛、缺乏系统性且鲜为人知。低磷酸酶血症的时间演变可能与其病因有关。本研究的目的是报告与血清ALP急剧降至正常范围下限以下相关的情况和情形。

方法

马什菲尔德诊所机构审查委员会批准使用其电子病历搜索血清碱性磷酸酶值至少有两次≤40 U/L(正常范围40 - 125 U/L)的受试者。当符合条件的碱性磷酸酶值的时间演变表明从通常正常的血清碱性磷酸酶值急剧下降时,该受试者被视为患有急性低磷酸酶血症。分析了30年的实验室数据和10年的临床记录。记录相关诊断、临床情况和短期死亡率。

结果

共有458,767名受试者有2,584,051次血清碱性磷酸酶值,5190名(1.1%)受试者至少有两次血清值≤40 U/L。对根据最低碱性磷酸酶值和年龄选择的1276名受试者进行详细审查,确定了190名患有急性低磷酸酶血症的受试者。急性低磷酸酶血症记录于重大创伤/手术、多系统衰竭、急性贫血、血液制品输注(通常大量)、单采、低镁血症和急性热量限制期间。28名受试者(15%)在其血清碱性磷酸酶最低点后35天内死亡。

结论

急性低磷酸酶血症与严重疾病或生理应激有关,随后短期死亡率增加。低磷酸酶血症的时间演变可能与其病因有关。

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