Abstract:
Cyclic vomiting syndrome (CVS) in children is characterized by frequent hospitalizations, multiple comorbidities, and poor quality of life. In the absence of robust data, the treatment of CVS remains largely empiric starting with the 2008 NASPGHAN Consensus Statement recommendations of cyproheptadine for children < 5 years of age and amitriptyline for those ≥ 5 years with propranolol serving as the second-line agent. Comprehensive management begins with lifestyle alterations, and extends to medications, supplements, and stress reduction therapies. Standard drug therapy is organized by the four phases of the illness: (1) interictal (preventative medications and mitochondrial supplements), (2) prodromal (abortive agents), (3) vomiting (fluids/energy substrates, antiemetics, analgesics, and sedatives) and (4) recovery (supportive care and nutrition). Because the response to treatment is heterogeneous, clinicians often trial several different preventative strategies including NK1 antagonists, cautious titration of amitriptyline to higher doses, anticonvulsants, Ca2+-channel blockers, and other TCA antidepressants. When the child remains refractory to treatment, reconsideration of possible missed diagnoses and further mono- or combination therapy and psychotherapy can be guided by accompanying comorbidities (especially anxiety), specific subphenotype, and when available, genotype. For hospital intervention, IV fluids with 10% dextrose, antiemetics, and analgesics can lessen symptoms while effective sedation in some instances can truncate severe episodes.
CONCLUSIONS: Although management of CVS remains challenging to the clinician, approaches based upon recent literature and accumulated experience with subgroups of patients has led to improved treatment of the refractory and hospitalized patient. What is Known: • Cyclic vomiting syndrome is a complex disorder that remains challenging to manage. • Previous therapy has been guided by the NASPGHAN Consensus Statement of 2008. What is New: • New prophylactic approaches include NK1 antagonists and higher dosages of amitriptyline. • Strategies based upon comorbidities and subphenotype are helpful in refractory patients.
摘要:
儿童周期性呕吐综合征(CVS)的特征是频繁住院,多种合并症,和生活质量差。在缺乏可靠数据的情况下,从2008年NASPGHAN共识声明建议5岁以下儿童使用赛庚啶,5岁以上儿童使用阿米替林,普萘洛尔作为二线药物,CVS的治疗在很大程度上仍然是经验性的.全面管理始于生活方式的改变,延伸到药物,补充剂,和减压疗法。标准药物治疗由疾病的四个阶段组织:(1)间期(预防性药物和线粒体补充剂),(2)前驱(败血药),(3)呕吐(液体/能量底物,止吐药,镇痛药,和镇静剂)和(4)恢复(支持性护理和营养)。因为对治疗的反应是异质的,临床医生经常试验几种不同的预防策略,包括NK1拮抗剂,谨慎滴定阿米替林至高剂量,抗惊厥药,Ca2+-通道阻断剂,和其他TCA抗抑郁药。当孩子仍然难以治疗时,重新考虑可能的漏诊和进一步的单一或联合治疗和心理治疗可以通过伴随的合并症(尤其是焦虑)指导,特定的亚表型,如果有的话,基因型。对于医院干预,含10%葡萄糖的静脉输液,止吐药,和镇痛药可以减轻症状,而在某些情况下有效的镇静可以缩短严重发作。
结论:尽管CVS的管理对临床医生来说仍然具有挑战性,基于最近的文献和对患者亚组积累的经验的方法改善了对难治性住院患者的治疗.已知:•周期性呕吐综合征是一种复杂的疾病,仍然难以控制。•先前的治疗一直以2008年NASPGHAN共识声明为指导。新的:•新的预防方法包括NK1拮抗剂和更高剂量的阿米替林。•基于合并症和亚表型的策略对难治性患者有帮助。