Chen Zaisheng, Li Jian
Department of Pediatric Hematology, Fujian Medical University Union Hospital, Fuzhou 350001, China.
Zhonghua Er Ke Za Zhi. 2014 Nov;52(11):854-8.
To analyze the clinical characteristics and the course of diagnosis and therapy of PEG-asparaginase associated pancreatitis (AAP) in childhood, and to reveal the pathophysiology of AAP, enhance the ability of diagnosis and treament.
Data of 13 cases with AAP in childhood seen from March 2011 to March 2014 were analyzed with regard to clinical manifestations, laboratory findings, imaging feature and treatment.
AAP was found in 12 of acute lymphoblastic leukemia (ALL) and 1 of non-Hodgkin's lymphoma (NHL), 8 were boys and 5 were girls, with a mean age 6 years. In 12 cases AAP occurred during the induction-remission treatment, in 1 case during the maintenance- intensification phase. AAP occurred after a median of two doses, and 9 d (median) from the latest administration of PEG-asparaginase. The major manifestations of AAP was abdominal pain (11/13) . At the time of AAP diagnosis during the first 48 hours the median peak serum amylase and serum lipase levels were 559 U/L (range 118-1 585, upper normal limit: 125) and 934 U/L (range 221-1 673, upper normal limit: 300). Three cases with serum amylase and serum lipase levels above 3 times upper normal limit were repeatedly complicated with pancreatic pseudocyst; 11 patients had abnormal CT imaging, 8 cases revealed effusion around the pancreas, and 4 cases had pseudocyst. Therapy with ulinastatin, octreotide acetate, glucocorticoid could relieve abdominal pain significantly. Three cases underwent abdominal puncture drainage and 5 cases fulfilled nasojejunal nutrition therapy. Nine of them were cured, 4 developed pseudocyst, in 2 AAP vanished gradually and 2 died with pseudocyst.
The major manifestations of AAP were abdominal pain, but sometimes apparent and sometimes latent. Condition of acute pancreatitis may exacerbate rapidly and easily. Early identification had significance. Pancreatic pseudocyst suggested a poor prognosis.
分析儿童聚乙二醇化天冬酰胺酶相关性胰腺炎(AAP)的临床特征及诊治过程,揭示AAP的病理生理机制,提高诊断和治疗能力。
分析2011年3月至2014年3月期间收治的13例儿童AAP患者的临床表现、实验室检查结果、影像学特征及治疗情况。
13例AAP患者中,12例为急性淋巴细胞白血病(ALL),1例为非霍奇金淋巴瘤(NHL);男8例,女5例,平均年龄6岁。12例AAP发生在诱导缓解治疗期间,1例发生在维持强化期。AAP发生的中位剂量为2剂,距最后一次使用聚乙二醇化天冬酰胺酶的中位时间为9天。AAP的主要表现为腹痛(11/13)。在AAP诊断后的前48小时,血清淀粉酶和脂肪酶的中位峰值水平分别为559 U/L(范围118 - 1585,正常上限:125)和934 U/L(范围221 - 1673,正常上限:300)。3例血清淀粉酶和脂肪酶水平高于正常上限3倍的患者反复并发胰腺假性囊肿;11例患者CT成像异常,8例显示胰腺周围积液,4例有假性囊肿。乌司他丁、醋酸奥曲肽、糖皮质激素治疗可显著缓解腹痛。3例行腹腔穿刺引流,5例进行鼻空肠营养治疗。其中9例治愈,4例形成假性囊肿,2例AAP逐渐消失,2例因假性囊肿死亡。
AAP的主要表现为腹痛,但有时明显有时隐匿。急性胰腺炎病情可能迅速且容易加重。早期识别具有重要意义。胰腺假性囊肿提示预后不良。