Raucci Umberto, Borrelli Osvaldo, Di Nardo Giovanni, Tambucci Renato, Pavone Piero, Salvatore Silvia, Baldassarre Maria Elisabetta, Cordelli Duccio Maria, Falsaperla Raffaele, Felici Enrico, Ferilli Michela Ada Noris, Grosso Salvatore, Mallardo Saverio, Martinelli Diego, Quitadamo Paolo, Pensabene Licia, Romano Claudio, Savasta Salvatore, Spalice Alberto, Strisciuglio Caterina, Suppiej Agnese, Valeriani Massimiliano, Zenzeri Letizia, Verrotti Alberto, Staiano Annamaria, Villa Maria Pia, Ruggieri Martino, Striano Pasquale, Parisi Pasquale
Pediatric Emergency Department, Bambino Gesù Children's Hospital, Institute for Research, Hospitalization and Health Care (IRCCS), Rome, Italy.
Division of Neurogastroenterology and Motility, Department of Pediatric Gastroenterology, University College London (UCL) Institute of Child Health and Great Ormond Street Hospital, London, United Kingdom.
Front Neurol. 2020 Nov 2;11:583425. doi: 10.3389/fneur.2020.583425. eCollection 2020.
Cyclic Vomiting Syndrome (CVS) is an underdiagnosed episodic syndrome characterized by frequent hospitalizations, multiple comorbidities, and poor quality of life. It is often misdiagnosed due to the unappreciated pattern of recurrence and lack of confirmatory testing. CVS mainly occurs in pre-school or early school-age, but infants and elderly onset have been also described. The etiopathogenesis is largely unknown, but it is likely to be multifactorial. Recent evidence suggests that aberrant brain-gut pathways, mitochondrial enzymopathies, gastrointestinal motility disorders, calcium channel abnormalities, and hyperactivity of the hypothalamic-pituitary-adrenal axis in response to a triggering environmental stimulus are involved. CVS is characterized by acute, stereotyped and recurrent episodes of intense nausea and incoercible vomiting with predictable periodicity and return to baseline health between episodes. A distinction with other differential diagnoses is a challenge for clinicians. Although extensive and invasive investigations should be avoided, baseline testing toward identifying organic causes is recommended in all children with CVS. The management of CVS requires an individually tailored therapy. Management of acute phase is mainly based on supportive and symptomatic care. Early intervention with abortive agents during the brief prodromal phase can be used to attempt to terminate the attack. During the interictal period, non-pharmacologic measures as lifestyle changes and the use of reassurance and anticipatory guidance seem to be effective as a preventive treatment. The indication for prophylactic pharmacotherapy depends on attack intensity and severity, the impairment of the QoL and if attack treatments are ineffective or cause side effects. When children remain refractory to acute or prophylactic treatment, or the episode differs from previous ones, the clinician should consider the possibility of an underlying disease and further mono- or combination therapy and psychotherapy can be guided by accompanying comorbidities and specific sub-phenotype. This review was developed by a joint task force of the Italian Society of Pediatric Gastroenterology Hepatology and Nutrition (SIGENP) and Italian Society of Pediatric Neurology (SINP) to identify relevant current issues and to propose future research directions on pediatric CVS.
周期性呕吐综合征（CVS）是一种诊断不足的发作性综合征，其特征为频繁住院、多种合并症以及生活质量差。由于未认识到复发模式且缺乏确诊检查，该病常被误诊。CVS主要发生在学龄前或学龄早期，但也有婴儿期和老年期发病的报道。其发病机制在很大程度上尚不清楚，但可能是多因素的。最近的证据表明，异常的脑-肠通路、线粒体酶病、胃肠动力障碍、钙通道异常以及下丘脑-垂体-肾上腺轴对触发环境刺激的反应亢进均与之有关。CVS的特征是急性、刻板且反复发作的强烈恶心和无法控制的呕吐，具有可预测的周期性，发作间期可恢复至基线健康状态。与其他鉴别诊断区分开来对临床医生而言是一项挑战。尽管应避免进行广泛且有创的检查，但建议对所有CVS患儿进行旨在识别器质性病因的基线检查。CVS的管理需要个体化定制治疗。急性期的管理主要基于支持性和对症治疗。在短暂的前驱期使用中止发作药物进行早期干预可用于尝试终止发作。在发作间期，生活方式改变等非药物措施以及给予安慰和预期指导似乎作为预防性治疗有效。预防性药物治疗的指征取决于发作强度和严重程度、生活质量受损情况以及发作治疗是否无效或引起副作用。当儿童对急性或预防性治疗仍有抵抗，或发作与既往不同时，临床医生应考虑潜在疾病的可能性，可根据伴随的合并症和特定亚表型指导进一步的单一或联合治疗及心理治疗。本综述由意大利儿科学会胃肠病学、肝病学和营养学会（SIGENP）和意大利儿科学会神经病学学会（SINP）的联合工作组编写，旨在确定当前相关问题并提出关于儿童CVS的未来研究方向。
