乳糖酶缺乏

乳糖吸收不良和乳糖不耐受最常见的原因是乳糖酶不持久。虽然在 2-3 岁之前很少见，但它可以在儿童期的任何年龄出现。

患者应仔细尝试各种食物来找到他们的乳糖阈值。 下列乳制品由于乳糖水平较低可能被耐受：硬乳酪、活性酸奶、活性凝乳、活性乳酪和谷物奶混合物。

无乳糖和低乳糖牛奶(例如，豆奶、椰奶、燕麦奶和谷物奶)在市场上均可买到

有严重症状的患者应积极地发现和避免含乳糖的乳制品和非乳制品。[1]Matthews SB, Waud JP, Roberts AG, et al. Systemic lactose intolerance: a new perspective on an old problem. Postgrad Med J. 2005 Mar;81(953):167-73.https://pmj.bmj.com/content/81/953/167.longhttp://www.ncbi.nlm.nih.gov/pubmed/15749792?tool=bestpractice.com[56]Eadala P, Waud JP, Matthews SB, et al. Quantifying the "hidden" lactose in drugs used for the treatment of gastrointestinal conditions. Aliment Pharmacol Ther. 2009 Mar 15;29(6):677-87.http://www.ncbi.nlm.nih.gov/pubmed/19035974?tool=bestpractice.com[59]Moore BJ. Dairy foods: are they politically correct? Nutr Today. 2003 May-Jun;38(3):82-90.http://www.ncbi.nlm.nih.gov/pubmed/12813186?tool=bestpractice.com 许多加工食品和方便食品，以及一些减肥食品，乳糖含量较高但却无相应标记。一些对可发酵寡糖、双糖、单糖和多元醇 (FODMAP) 含量低的饮食有反应的肠易激综合征患者可能会随着乳糖和 FODMAP 的减少而出现胃肠症状改善。[52]Deng Y, Misselwitz B, Dai N, et al. Lactose intolerance in adults: biological mechanism and dietary management. Nutrients. 2015 Sep 18;7(9):8020-35.https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4586575/http://www.ncbi.nlm.nih.gov/pubmed/26393648?tool=bestpractice.com[60]Zheng X, Chu H, Cong Y, et al. Self-reported lactose intolerance in clinic patients with functional gastrointestinal symptoms: prevalence, risk factors, and impact on food choices. Neurogastroenterol Motil. 2015 Aug;27(8):1138-46.http://www.ncbi.nlm.nih.gov/pubmed/26095206?tool=bestpractice.com

诊断时应请营养师会诊（之后每年一次），如果症状持续存在且提示有隐性乳糖来源时也应咨询营养师。营养师不但强调患者需长期食用低乳糖（或无乳糖）乳制品，而且还建议患者采用富含钙和维生素 D 的饮食。[50]Holick MF, Binkley NC, Bischoff-Ferrari HA, et al; Endocrine Society. Evaluation, treatment, and prevention of vitamin D deficiency: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2011 Jul;96(7):1911-30.https://academic.oup.com/jcem/article/96/7/1911/2833671http://www.ncbi.nlm.nih.gov/pubmed/21646368?tool=bestpractice.com[51]The Scientific Advisory Committee on Nutrition (SACN) recommendations on vitamin D. Independent report: SACN vitamin D and health report. Jul 2016 [internet publication].https://www.gov.uk/government/publications/sacn-vitamin-d-and-health-report 尽管缺乏证据支持对乳糖不耐受患者补充钙和维生素 D 的有效性，[52]Deng Y, Misselwitz B, Dai N, et al. Lactose intolerance in adults: biological mechanism and dietary management. Nutrients. 2015 Sep 18;7(9):8020-35.https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4586575/http://www.ncbi.nlm.nih.gov/pubmed/26393648?tool=bestpractice.com 但大多数临床医生在实践中支持这种方法。关于以其它营养素来源进行替代以保持足够的能量和蛋白质摄入的建议也非常重要。

含乳糖酶的益生菌可以帮助乳糖消化。[55]He T, Priebe MG, Zhong Y, et al. Effects of yogurt and bifidobacteria supplementation on the colonic microbiota in lactose-intolerant subjects. J Appl Microbiol. 2008 Feb;104(2):595-604.http://www.ncbi.nlm.nih.gov/pubmed/17927751?tool=bestpractice.com 然而，对这些制剂的潜在益处的研究评估出现了一些不一致的结果且它们的作用尚不明确。

乳糖酶制剂容易获得，治疗后通常允许乳糖不耐受者随意摄入部分或全部乳制品。补充真菌源乳糖酶尤其有效，且耐受性良好。[61]Montalto M, Nucera G, Santoro L, et al. Effect of exogenous beta-galactosidase in patients with lactose malabsorption and intolerance: a crossover double-blind placebo-controlled study. Eur J Clin Nutr. 2005 Apr;59(4):489-93.https://www.nature.com/articles/1602098http://www.ncbi.nlm.nih.gov/pubmed/15674309?tool=bestpractice.com[62]Ojetti V, Gigante G, Gabrielli M, et al. The effect of oral supplementation with Lactobacillus reuteri or tilactase in lactose intolerant patients: randomized trial. Eur Rev Med Pharmacol Sci. 2010 Mar;14(3):163-70.http://www.ncbi.nlm.nih.gov/pubmed/20391953?tool=bestpractice.com

针对经选择患者组全部患者的治疗推荐

虽然乳糖吸收不良并不导致钙吸收不良，但回避乳制品可能影响获得最理想的骨矿化。[63]Jackson KA, Savaiano DA. Lactose maldigestion, calcium intake, and osteoporosis in African-, Asian-, and Hispanic-Americans. J Am Coll Nutr. 2001 Apr;20(2 Suppl):198-207S.http://www.ncbi.nlm.nih.gov/pubmed/11349943?tool=bestpractice.com 当乳制品被去除或显著减少时、与钙和维生素D缺乏有关的严重疾病或骨量减少或需要双能X线扫描的患者，所减少的钙摄入应使用其它富含钙的食物或钙补充剂来补偿。[2]Heyman MB; American Academy of Pediatrics, Committee on Nutrition. Lactose intolerance in infants, children, and adolescents. Pediatrics. 2006 Sep;118(3):1279-86.http://pediatrics.aappublications.org/content/118/3/1279.longhttp://www.ncbi.nlm.nih.gov/pubmed/16951027?tool=bestpractice.com[64]Greer FR, Krebs NF; American Academy of Pediatrics Committee on Nutrition. Optimizing bone health and calcium intakes of infants, children, and adolescents. Pediatrics. 2006 Feb;117(2):578-85.http://pediatrics.aappublications.org/content/117/2/578.fullhttp://www.ncbi.nlm.nih.gov/pubmed/16452385?tool=bestpractice.com

针对经选择患者组全部患者的治疗推荐

当膳食中去除或显著减少乳制品、发生与钙和维生素 D 缺乏有关的严重疾病或骨质减少或者对于需要 DEXA 扫描的患者，应考虑根据当地医疗规程补充维生素 D，并且每 6-12 个月监测一次维生素 D 状况。[50]Holick MF, Binkley NC, Bischoff-Ferrari HA, et al; Endocrine Society. Evaluation, treatment, and prevention of vitamin D deficiency: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2011 Jul;96(7):1911-30.https://academic.oup.com/jcem/article/96/7/1911/2833671http://www.ncbi.nlm.nih.gov/pubmed/21646368?tool=bestpractice.com[51]The Scientific Advisory Committee on Nutrition (SACN) recommendations on vitamin D. Independent report: SACN vitamin D and health report. Jul 2016 [internet publication].https://www.gov.uk/government/publications/sacn-vitamin-d-and-health-report

继发性疾病和潜在疾病引起的乳糖吸收不良的治疗包括潜在原因的治疗。例如，通过静脉输液和全肠外营养治疗小肠损伤；口服补液治疗急性胃肠炎；多西环素治疗小肠细菌过度生长；免麦胶膳食治疗乳糜泻；以及奥曲肽、静脉输液和止吐剂治疗癌症化疗所致症状。

诊断时应请营养师会诊（之后每年一次），如果症状持续存在且提示有隐性乳糖来源时也应咨询营养师。营养师不但强调患者需长期食用低乳糖（或无乳糖）乳制品，而且还建议患者采用富含钙和维生素 D 的饮食。[50]Holick MF, Binkley NC, Bischoff-Ferrari HA, et al; Endocrine Society. Evaluation, treatment, and prevention of vitamin D deficiency: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2011 Jul;96(7):1911-30.https://academic.oup.com/jcem/article/96/7/1911/2833671http://www.ncbi.nlm.nih.gov/pubmed/21646368?tool=bestpractice.com[51]The Scientific Advisory Committee on Nutrition (SACN) recommendations on vitamin D. Independent report: SACN vitamin D and health report. Jul 2016 [internet publication].https://www.gov.uk/government/publications/sacn-vitamin-d-and-health-report 尽管缺乏证据支持对乳糖不耐受患者补充钙和维生素 D 的有效性，[52]Deng Y, Misselwitz B, Dai N, et al. Lactose intolerance in adults: biological mechanism and dietary management. Nutrients. 2015 Sep 18;7(9):8020-35.https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4586575/http://www.ncbi.nlm.nih.gov/pubmed/26393648?tool=bestpractice.com 但大多数临床医生在实践中支持这种方法。关于以其它营养素来源进行替代以保持足够的能量和蛋白质摄入的建议也非常重要。

一些有严重乳糖不耐受症状的患者可能需要暂时限制乳制品以改善症状。

含乳糖酶的益生菌可以帮助乳糖消化。[55]He T, Priebe MG, Zhong Y, et al. Effects of yogurt and bifidobacteria supplementation on the colonic microbiota in lactose-intolerant subjects. J Appl Microbiol. 2008 Feb;104(2):595-604.http://www.ncbi.nlm.nih.gov/pubmed/17927751?tool=bestpractice.com 然而，对这些制剂的潜在益处的研究评估出现了一些不一致的结果且它们的作用尚不明确。

乳糖酶制剂容易买到，且可以让乳糖不耐受者能够自由摄入部分或全部牛奶制品。 补充来自真菌的乳糖酶特别有效，耐受性好。[61]Montalto M, Nucera G, Santoro L, et al. Effect of exogenous beta-galactosidase in patients with lactose malabsorption and intolerance: a crossover double-blind placebo-controlled study. Eur J Clin Nutr. 2005 Apr;59(4):489-93.https://www.nature.com/articles/1602098http://www.ncbi.nlm.nih.gov/pubmed/15674309?tool=bestpractice.com[62]Ojetti V, Gigante G, Gabrielli M, et al. The effect of oral supplementation with Lactobacillus reuteri or tilactase in lactose intolerant patients: randomized trial. Eur Rev Med Pharmacol Sci. 2010 Mar;14(3):163-70.http://www.ncbi.nlm.nih.gov/pubmed/20391953?tool=bestpractice.com

针对经选择患者组全部患者的治疗推荐

虽然乳糖吸收不良并不导致钙吸收不良，但避免乳制品摄入可能影响获得最佳的骨矿化。[63]Jackson KA, Savaiano DA. Lactose maldigestion, calcium intake, and osteoporosis in African-, Asian-, and Hispanic-Americans. J Am Coll Nutr. 2001 Apr;20(2 Suppl):198-207S.http://www.ncbi.nlm.nih.gov/pubmed/11349943?tool=bestpractice.com 当乳制品被去除或显著减少时、与钙和维生素D缺乏有关的严重疾病或骨量减少或需要双能X线扫描的患者，所减少的钙摄入应使用其它富含钙的食物或钙补充剂来补偿。[2]Heyman MB; American Academy of Pediatrics, Committee on Nutrition. Lactose intolerance in infants, children, and adolescents. Pediatrics. 2006 Sep;118(3):1279-86.http://pediatrics.aappublications.org/content/118/3/1279.longhttp://www.ncbi.nlm.nih.gov/pubmed/16951027?tool=bestpractice.com[64]Greer FR, Krebs NF; American Academy of Pediatrics Committee on Nutrition. Optimizing bone health and calcium intakes of infants, children, and adolescents. Pediatrics. 2006 Feb;117(2):578-85.http://pediatrics.aappublications.org/content/117/2/578.fullhttp://www.ncbi.nlm.nih.gov/pubmed/16452385?tool=bestpractice.com

针对经选择患者组全部患者的治疗推荐

当膳食中去除或显著减少乳制品、发生与钙和维生素 D 缺乏有关的严重疾病或骨质减少或者对于需要 DEXA 扫描的患者，应考虑根据当地医疗规程补充维生素 D，并且每 6-12 个月监测一次维生素 D 状况。[50]Holick MF, Binkley NC, Bischoff-Ferrari HA, et al; Endocrine Society. Evaluation, treatment, and prevention of vitamin D deficiency: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2011 Jul;96(7):1911-30.https://academic.oup.com/jcem/article/96/7/1911/2833671http://www.ncbi.nlm.nih.gov/pubmed/21646368?tool=bestpractice.com[51]The Scientific Advisory Committee on Nutrition (SACN) recommendations on vitamin D. Independent report: SACN vitamin D and health report. Jul 2016 [internet publication].https://www.gov.uk/government/publications/sacn-vitamin-d-and-health-report

推荐所有的早产儿进行母乳喂养和/或含乳糖配方奶喂养。 对出现乳糖不耐受症状的婴儿可以在几周至2个月期间给予乳糖酶补充剂。证据 B短期使用乳糖酶补充剂或低乳糖配方奶：有中等质量证据显示出现发育性乳糖酶缺乏的早产婴儿短期使用乳糖酶补充剂或低乳糖配方奶可获益。[2]Heyman MB; American Academy of Pediatrics, Committee on Nutrition. Lactose intolerance in infants, children, and adolescents. Pediatrics. 2006 Sep;118(3):1279-86.http://pediatrics.aappublications.org/content/118/3/1279.longhttp://www.ncbi.nlm.nih.gov/pubmed/16951027?tool=bestpractice.com受试者＜200名的随机对照临床试验（RCT）、受试者＞200名且方法学存在缺陷的随机对照临床试验（RCT）、方法学存在缺陷的系统评价或者高质量的观察性（队列）研究。

由于没有证据显示母乳或含乳糖的配方奶对于早产婴儿有短期或长期的有害作用，所以乳糖酶补充剂可以与含乳糖的配方奶或母乳一起给予。证据 B含乳糖配方奶和母乳的应用：有中等质量的证据显示含乳糖配方奶和母乳对有发育性乳糖酶缺乏的早产婴儿无短期或长期的有害影响。[66]Erasmus HD, Ludwig-Auser HM, Paterson PG, et al. Enhanced weight gain in preterm infants receiving lactase-treated feeds: a randomized, double-blind, controlled trial. J Pediatr. 2002 Oct;141(4):532-7.http://www.ncbi.nlm.nih.gov/pubmed/12378193?tool=bestpractice.com受试者＜200名的随机对照临床试验（RCT）、受试者＞200名且方法学存在缺陷的随机对照临床试验（RCT）、方法学存在缺陷的系统评价或者高质量的观察性（队列）研究。

仅对于有严重乳糖不耐受症状的患者推荐避免含乳糖的配方奶和母乳；如果需要，仅推荐极短期的回避。

如果没有早期的识别和迅速的治疗，先天性乳糖酶缺乏是可能威胁生命的。 腹泻的初始治疗包括静脉补充液体和电解质。

如果没有早期的识别和迅速的治疗，先天性乳糖酶缺乏是可能威胁生命的。

仅有的治疗除了治疗腹泻外就是从出生起完全回避乳糖。 治疗持续终生且包括从膳食中除去乳糖以及用无乳糖配方膳食商品来代替。

诊断时应请营养师会诊（之后每年一次）；如果症状一直持续提示有隐性乳糖来源时也应咨询营养师。营养师不但强调患者需长期食用低乳糖（或无乳糖）乳制品，而且还建议患者采用富含钙和维生素 D 的饮食。[50]Holick MF, Binkley NC, Bischoff-Ferrari HA, et al; Endocrine Society. Evaluation, treatment, and prevention of vitamin D deficiency: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2011 Jul;96(7):1911-30.https://academic.oup.com/jcem/article/96/7/1911/2833671http://www.ncbi.nlm.nih.gov/pubmed/21646368?tool=bestpractice.com[51]The Scientific Advisory Committee on Nutrition (SACN) recommendations on vitamin D. Independent report: SACN vitamin D and health report. Jul 2016 [internet publication].https://www.gov.uk/government/publications/sacn-vitamin-d-and-health-report 尽管缺乏证据支持对乳糖不耐受患者补充钙和维生素 D 的有效性，[52]Deng Y, Misselwitz B, Dai N, et al. Lactose intolerance in adults: biological mechanism and dietary management. Nutrients. 2015 Sep 18;7(9):8020-35.https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4586575/http://www.ncbi.nlm.nih.gov/pubmed/26393648?tool=bestpractice.com 但大多数临床医生在实践中支持这种方法。关于以其它营养素来源进行替代以保持足够的能量和蛋白质摄入的建议也非常重要。

针对经选择患者组全部患者的治疗推荐

虽然乳糖吸收不良并不导致钙吸收不良，但回避乳制品可能影响获得最理想的骨矿化。[63]Jackson KA, Savaiano DA. Lactose maldigestion, calcium intake, and osteoporosis in African-, Asian-, and Hispanic-Americans. J Am Coll Nutr. 2001 Apr;20(2 Suppl):198-207S.http://www.ncbi.nlm.nih.gov/pubmed/11349943?tool=bestpractice.com 当乳制品被去除或显著减少时、与钙和维生素D缺乏有关的严重疾病或骨量减少或需要双能X线扫描的患者，所减少的钙摄入应使用其它富含钙的食物或钙补充剂来补偿。[2]Heyman MB; American Academy of Pediatrics, Committee on Nutrition. Lactose intolerance in infants, children, and adolescents. Pediatrics. 2006 Sep;118(3):1279-86.http://pediatrics.aappublications.org/content/118/3/1279.longhttp://www.ncbi.nlm.nih.gov/pubmed/16951027?tool=bestpractice.com[64]Greer FR, Krebs NF; American Academy of Pediatrics Committee on Nutrition. Optimizing bone health and calcium intakes of infants, children, and adolescents. Pediatrics. 2006 Feb;117(2):578-85.http://pediatrics.aappublications.org/content/117/2/578.fullhttp://www.ncbi.nlm.nih.gov/pubmed/16452385?tool=bestpractice.com

针对经选择患者组全部患者的治疗推荐

当膳食中去除或显著减少乳制品、发生与钙和维生素 D 缺乏有关的严重疾病或骨质减少或者对于需要 DEXA 扫描的患者，应考虑根据当地医疗规程补充维生素 D，并且每 6-12 个月监测一次维生素 D 状况。[50]Holick MF, Binkley NC, Bischoff-Ferrari HA, et al; Endocrine Society. Evaluation, treatment, and prevention of vitamin D deficiency: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2011 Jul;96(7):1911-30.https://academic.oup.com/jcem/article/96/7/1911/2833671http://www.ncbi.nlm.nih.gov/pubmed/21646368?tool=bestpractice.com[51]The Scientific Advisory Committee on Nutrition (SACN) recommendations on vitamin D. Independent report: SACN vitamin D and health report. Jul 2016 [internet publication].https://www.gov.uk/government/publications/sacn-vitamin-d-and-health-report