中间代谢先天性缺陷中的并发疾病。
Intercurrent illness in inborn errors of intermediary metabolism.
作者信息
Dixon M A, Leonard J V
机构信息
Hospital for Sick Children, London.
出版信息
Arch Dis Child. 1992 Nov;67(11):1387-91. doi: 10.1136/adc.67.11.1387.
Abstract
Metabolic decompensation may occur in patients with disorders of intermediary metabolism during intercurrent illness. To prevent complications it is normal practice to change the diet to an 'emergency regimen'. The mainstay of this is a high carbohydrate intake, using soluble glucose polymer, given as frequent drinks by day and during the night. Additional therapy is given for some disorders. Practical details of the treatment are outlined.
摘要
患有中间代谢紊乱的患者在并发疾病期间可能会发生代谢失代偿。为预防并发症，通常的做法是将饮食改为“应急方案”。其主要内容是高碳水化合物摄入，使用可溶性葡萄糖聚合物，白天和夜间频繁饮用。针对某些疾病还会给予额外治疗。文中概述了治疗的实际细节。
相似文献
1
Intercurrent illness in inborn errors of intermediary metabolism.中间代谢先天性缺陷中的并发疾病。
Arch Dis Child. 1992 Nov;67(11):1387-91. doi: 10.1136/adc.67.11.1387.
2
The dietary therapy of inherited metabolic disease.遗传性代谢疾病的饮食疗法
Prog Food Nutr Sci. 1975;1(7-8):507-30.
3
[Diet therapy of some inborn errors of metabolism].[某些先天性代谢缺陷的饮食疗法]
Pediatria (Napoli). 1974 Sep 30;82(2):260-97.
4
An introduction to nutritional treatment in inborn errors of metabolism--different disorders, different approaches.先天性代谢缺陷的营养治疗介绍——不同疾病，不同方法。
Southeast Asian J Trop Med Public Health. 2003;34 Suppl 3:198-201.
5
Dietary management of inborn errors of metabolism.先天性代谢缺陷的饮食管理
Indian J Pediatr. 2002 May;69(5):421-6. doi: 10.1007/BF02722635.
6
The dietary treatment of inborn errors of metabolism.先天性代谢缺陷的饮食治疗。
Proc Nutr Soc. 1976 May;35(1):31-6.
7
[Diet therapy of patients with congenital metabolic defects].[先天性代谢缺陷患者的饮食治疗]
Fortschr Med. 1978 May 11;96(18):971-2.
8
Continuous venovenous hemofiltration in the management of acute decompensation in inborn errors of metabolism.
J Pediatr. 1991 Jun;118(6):879-84. doi: 10.1016/s0022-3476(05)82198-5.
9
Inborn errors of metabolism: the need for sugar.先天性代谢缺陷：对糖的需求
J Hum Nutr. 1979 Apr;33(2):146-54. doi: 10.3109/09637487909143365.
10
Early recognition of metabolic decompensation.早期识别代谢失代偿。
Arch Dis Child. 1997 Jun;76(6):555-6. doi: 10.1136/adc.76.6.555.
引用本文的文献
1
Disorders of organic acid metabolism and epilepsy.有机酸代谢紊乱与癫痫
Acta Epileptol. 2024 Aug 20;6(1):24. doi: 10.1186/s42494-024-00167-2.
2
Acute Metabolic Decompensation of Isovaleric Acidemia Presenting as Persistent Metabolic Acidosis in a Middle-Aged Man: A Case Report.一名中年男性因异戊酸血症急性代谢失代偿表现为持续性代谢性酸中毒：病例报告
Cureus. 2024 Aug 19;16(8):e67253. doi: 10.7759/cureus.67253. eCollection 2024 Aug.
3
The Reciprocal Interplay between Infections and Inherited Metabolic Disorders.感染与遗传性代谢紊乱之间的相互作用
Microorganisms. 2023 Oct 12;11(10):2545. doi: 10.3390/microorganisms11102545.
4
A Novel Mutation in the OXCT1 Gene Causing Succinyl-CoA:3-Ketoacid CoA Transferase (SCOT) Deficiency Starting with Neurologic Manifestations.OXCT1基因的一种新型突变导致琥珀酰辅酶A：3-酮酸辅酶A转移酶（SCOT）缺乏，首发表现为神经症状。
Iran J Child Neurol. 2023 Spring;17(2):127-133. doi: 10.22037/ijcn.v17i2.35963. Epub 2023 Mar 15.
5
The Use of Telemedicine and Other Strategies by Registered Dietitians for the Medical Nutrition Therapy of Patients With Inherited Metabolic Disorders During the COVID-19 Pandemic.注册营养师在新冠疫情期间对遗传性代谢紊乱患者进行医学营养治疗时使用远程医疗及其他策略的情况。
Front Nutr. 2021 Apr 27;8:637868. doi: 10.3389/fnut.2021.637868. eCollection 2021.
6
A generic emergency protocol for patients with inborn errors of metabolism causing fasting intolerance: A retrospective, single-center study and the generation of www.emergencyprotocol.net.针对引起禁食不耐受的先天性代谢缺陷患者的通用急救方案：一项回顾性、单中心研究及 www.emergencyprotocol.net 的建立。
J Inherit Metab Dis. 2021 Sep;44(5):1124-1135. doi: 10.1002/jimd.12386. Epub 2021 May 4.
7
Chemical individuality in T cells: A Garrodian view of immunometabolism.T 细胞的化学个体性：免疫代谢的戈拉德观点。
Immunol Rev. 2020 May;295(1):82-100. doi: 10.1111/imr.12854. Epub 2020 Mar 31.
8
Prenatal Diagnosis of Organic Acidemias at a Tertiary Center.三级医疗中心对有机酸血症的产前诊断
Balkan J Med Genet. 2019 Aug 28;22(1):29-34. doi: 10.2478/bjmg-2019-0003. eCollection 2019 Jun.
9
The liver is a metabolic and immunologic organ: A reconsideration of metabolic decompensation due to infection in inborn errors of metabolism (IEM).肝脏是一个代谢和免疫器官：对先天性代谢缺陷（IEM）中感染所致代谢失代偿的重新认识。
Mol Genet Metab. 2017 Aug;121(4):283-288. doi: 10.1016/j.ymgme.2017.06.010. Epub 2017 Jun 24.
10
Proposed recommendations for diagnosing and managing individuals with glutaric aciduria type I: second revision.戊二酸血症I型患者诊断与管理的建议方案：第二次修订版
J Inherit Metab Dis. 2017 Jan;40(1):75-101. doi: 10.1007/s10545-016-9999-9. Epub 2016 Nov 16.
本文引用的文献
1
Muscle protein synthesis measured by stable isotope techniques in man: the effects of feeding and fasting.通过稳定同位素技术测定人体肌肉蛋白质合成：进食与禁食的影响。
Clin Sci (Lond). 1982 Dec;63(6):519-23. doi: 10.1042/cs0630519.
2
Sources of propionate in inborn errors of propionate metabolism.丙酸代谢先天性缺陷中丙酸的来源。
Metabolism. 1990 Nov;39(11):1133-7. doi: 10.1016/0026-0495(90)90084-p.
3
Acute illness in maple syrup urine disease: dynamics of protein metabolism and implications for management.枫糖尿症的急性疾病：蛋白质代谢动态及其对治疗的意义
J Pediatr. 1991 Jul;119(1 Pt 1):35-41. doi: 10.1016/s0022-3476(05)81035-2.
4
Renal tubular dysfunction in methylmalonic acidaemia.甲基丙二酸血症中的肾小管功能障碍。
Eur J Pediatr. 1991 Feb;150(4):259-63. doi: 10.1007/BF01955526.
Zotero 插件