Department of Medicine, Division of Digestive Health and Liver Diseases, University of Miami, Leonard M. Miller School of Medicine, Miami, Florida, USA.
Department of Pediatrics, Division of Gastroenterology, University of Florida, College of Medicine, Gainesville, Florida, USA.
Pediatr Pulmonol. 2024 Sep;59 Suppl 1:S53-S60. doi: 10.1002/ppul.26958. Epub 2024 Mar 19.
Patients with Cystic Fibrosis (CF) are at increased risk of acute (AP) and chronic (CP) pancreatitis, and their complications. The extent of remaining healthy pancreatic parenchyma determines the risk of developing future episodes of pancreatitis, as well as pancreatic exocrine or endocrine insufficiency. Pancreatitis may be the presenting symptom of CF, and genetic testing is especially important in pediatrics. AP and recurrent AP are managed with intravenous fluid hydration and pain control, in addition to early refeeding and treatment of complications. With the use of modulator therapy in CF, pancreatic function may be restored to some extent. CP related pain is managed with analgesics and neuromodulators, with surgery if indicated in specific situations including TPIAT as a possible type of surgical intervention. Long-term sequelae of CP in patients with CF include exocrine pancreatic insufficiency treated with pancreatic enzyme replacement therapy, fat-soluble vitamin deficiencies and associated metabolic complications such as bone disease/osteoporosis, pancreatogenic diabetes, and less commonly, pancreatic cancer. We review the presentation and etiologies of pancreatitis in CF patients as well as the management of AP and CP primarily in children.
囊性纤维化 (CF) 患者发生急性胰腺炎 (AP) 和慢性胰腺炎 (CP) 及其并发症的风险增加。剩余健康胰腺实质的程度决定了未来发生胰腺炎以及胰腺外分泌或内分泌功能不全的风险。胰腺炎可能是 CF 的首发症状，基因检测在儿科尤为重要。AP 和复发性 AP 的治疗包括静脉补液和止痛，此外还需要早期重新喂养和治疗并发症。随着 CF 调节剂治疗的应用，胰腺功能可能在一定程度上得到恢复。CP 相关疼痛采用镇痛药和神经调节剂治疗，如果存在特定情况（包括 TPIAT 作为一种可能的手术干预类型）则进行手术。CF 患者 CP 的长期后遗症包括外分泌胰腺功能不全，采用胰酶替代治疗，脂溶性维生素缺乏以及相关代谢并发症，如骨病/骨质疏松症、胰源性糖尿病，以及不太常见的胰腺癌。我们回顾了 CF 患者胰腺炎的表现和病因，以及主要在儿童中 AP 和 CP 的管理。
