Hereditary pancreatitis in a family of Aboriginal descent

Hereditary pancreatitis is an autosomal dominant condition characterized by recurrent episodes of acute pancreatitis, usually starting in childhood. We present a family who was ascertained when an 11-year-old girl presented with an episode of acute pancreatitis. Her father and other family members had also had recurrent bouts of acute pancreatitis. Genetic testing revealed a pathogenic mutation in the cationic trypsinogen gene in the proband, her father and her paternal grandmother. As far as we are aware, this is the first Aboriginal kindred with mutation-proven hereditary pancreatitis. Hereditary pancreatitis is an important differential diagnosis to consider in a patient with recurrent episodes of acute pancreatitis with no obvious precipitating cause. This family is of Aboriginal descent and the implications of the family's background are also discussed when considering the aetiology of the condition. We emphasize the need to ascertain a full family history from patients with a history of repeated episodes of acute pancreatitis and also emphasize the need to avoid ethnic stereotypes when assessing patients.     

CHRONIC FIBROSING PANCREATITIS IN CHILDHOOD: Report of a Case and Literature Review

Chronic fibrosing pancreatitis in childhood is an uncommon condition of unknown etiology with a variety of clinical presentations, histopathologic features, and outcomes. The diagnosis is one of exclusion (of hereditary or secondary pancreatitis), which should include histological assessment. The histological features of this condition have been described, to our knowledge, in nine published cases. We report a case in a 13-year-old male, who presented with obstructive jaundice and subsequently had evidence of endocrine and exocrine pancreatic insufficiency, despite a surgical decompression of the pancreatic-biliary duct system.     

儿童重症急性胰腺炎的特点与治疗

儿童急性胰腺炎病因主要包括感染、药物诱导、外伤和先天性胆道畸形等.儿童胰腺炎发病率低于成人,但呈增多趋势,至今儿童重症急性胰腺炎(severe acute pancreatitis,SAP)仍鲜有资料可循.腹痛和呕吐是SAP早期主要症状,也可能以休克或多器官功能障碍起病.目前仍没有适合儿童SAP病情严重程度的评分标准,有限资料认为入院24h血液脂肪酶升高程度、白蛋白水平和WBC计数有助于病情和预后的判断.血液净化可以有效减轻炎症反应、改善器官功能和稳定液体平衡,可能是儿童SAP新的有潜力的治疗措施.     

Gallstone pancreatitis in children: atypical presentation and review

Pancreatitis in children is less common than in adults and is mostly related to trauma, infection and anatomical anomalies. Gallstone pancreatitis is an even rarer entity in infants and children. We present an unusual case of gallstone pancreatitis in a 17- month-old girl who presented with jaundice of 1 month duration. The laboratory studies showed conjugated hyperbilirubinaemia with associated acute pancreatitis and pseudo-hyponatraemia secondary to the markedly elevated lipid profile. Further evaluations revealed obstruction at the Ampulla of Vater with no evidence of a choledochal cyst and no mass lesion seen in the pancreas. The patient initially underwent urgent decompressive cholecystostomy with intraoperative cholecystography. When the biochemical parameters improved, the patient underwent formal cholecystectomy with common bile duct exploration, extraction of multiple impacted stones in the ampullary region and Fogarty balloon sphincterotomy. The post-operative course was uneventful. Conclusion:Acute gallstone pancreatitis in children may present as jaundice or abdominal pain. Recognition, early diagnosis and surgical intervention are the mainstay for a good outcome.     

Hereditary pancreatitis: An updated review in pediatrics

Hereditary Pancreatitis (HP) has emerged as a significant cause of acute, acute recurrent and chronic pancreatitis in the pediatric population. Given that it presents similarly to other causes of pancreatitis, a positive family history and/or isolation of a gene mutation are vital in its designation. Inheritance patterns remain complex, but mutations involving the PRSS1, SPINK1, CFTR and CTRC genes are commonly implicated. Since being first described in 1952, dozens of genetic alterations that modify the action of pancreatic enzymes have been identified. Among children, these variants have been isolated in more than 50% of patients with chronic pancreatitis. Recent research has noted that such mutations in PRSS1, SPINK1 and CFTR genes are also associated with a faster progression from acute pancreatitis to chronic pancreatitis. Patients with HP are at increased risk of developing diabetes mellitus, exocrine pancreatic insufficiency, and pancreatic adenocarcinoma. Management follows a multi-disciplinary approach with avoidance of triggers, surveillance of associated conditions, treatment of pancreatic insufficiency and use of endoscopic and surgical interventions for complications. With significant sequela, morbidity and a progressive nature, a thorough understanding of the etiology, pathophysiologic mechanisms, diagnostic evaluation, current management strategies and future research considerations for this evolving disease entity in pediatrics is warranted.     

A review of acute pancreatitis in the era of COVID-19

Acute pancreatitis is uncommon in childhood with an estimated incidence of approximately 1 in 10,000 children per year. It is an important condition, which may escape prompt diagnosis and is associated with significant morbidity and mortality. Most often, it will result in an acute hospital admission. The course of this disease is unpredictable and ranges from self-resolving mild illness to significantly severe disease with high risk of mortality or complications due to progression to multiorgan failure. Considerable advances have occurred in management which is now focused on multidisciplinary approach with extensive investigation and minimally invasive endoscopic interventions resulting in improved prognosis. In recent years, incidence of acute pancreatitis in children has risen, either due to improved awareness or reflective of true rise. Since 2020 there are emerging data suggesting an association of COVID-19 with acute pancreatitis. The best approach to diagnosis and management of acute pancreatitis in children and young people is largely extrapolated from adult practice. This review presents a brief summary of normal physiology and pathophysiology relating to pancreatitis, a suggested approach to investigation and diagnosis and summarizes available evidence to inform management in children and young people. We will also explore the latest data collected linking COVID-19 to pancreatitis.     

儿童胰腺炎的营养管理

近几十年来,儿童胰腺炎发病率逐年增高,但是由于儿童胰腺炎相关的临床研究有限,儿童胰腺炎的营养管理指南大多基于成人的研究。目前大量成人胰腺炎的临床研究显著改变了胰腺炎的营养管理策略,但儿童胰腺炎的营养管理仍明显落后于成人。文章通过复习成人胰腺炎营养管理建议,结合有限的儿童临床研究来讨论儿童胰腺炎的营养管理并用于指导临床。     

Acute pancreatitis associated with varicella infection in an immunocompetent child

Acute pancreatitis associated with varicella-zoster infection is a rare event, particularly in immunocompetent children. We report on a case of acute pancreatitis in a 6-year old girl presenting with acute abdominal pain less than 72 hours after the onset of a typical vesicular rash. The diagnosis was confirmed through hyperamylasaemia, ultrasonographic findings of oedematous pancreatitis and acute seroconversion to varicella-zoster virus, after excluding more common causes of acute pancreatitis. Conservative treatment was initiated, with complete resolution of symptoms and absence of local or systemic complications on follow up. A review of the literature revealed only three previously reported cases, with very different outcomes, highlighting the need to consider this potentially fatal complication in every child presenting with acute abdominal pain during the course of varicella disease.     

儿童胆源性胰腺炎的临床特点

目的探讨儿童胆源性胰腺炎的临床特点。方法回顾分析2011年1月至2015年7月儿童胆源性胰腺炎10例的临床资料。结果 10例患儿中,男2例、女8例,平均年龄(6.1±4.9)岁,平均住院天数(8.4±3.8)d。胆囊结石4例,胆汁淤积4例,胆管结石2例,胆总管囊肿2例,胰腺分裂症1例。10例患儿均以腹痛为主要临床表现。均有血淀粉酶升高,最高值106~922 U/L;血脂肪酶升高6例,为22~2 000 U/L;血丙氨酸氨基转移酶异常6例,低钾血症2例,低血糖1例,酸碱失衡4例。腹部B超提示4例有胰腺炎改变,CT则提示均有胰腺炎改变。予禁食、补液、维持水电解质平衡、奥美拉唑抑制胃酸、奥曲肽抑制胰酶分泌等治疗。9例患儿内科保守治疗好转出院,1例转外科手术治疗。结论儿童胆源性胰腺炎发病率低,临床症状不典型,小年龄患儿胰胆管系统畸形较多见,内科保守治疗效果相对好。     

胰胆管发育异常合并儿童胰腺炎21例临床分析

目的　探讨胰胆管发育异常合并儿童胰腺炎的临床特点。方法　选取中国医科大学附属盛京医院小儿消化内科2017年6月至2020年9月胰腺炎住院患儿175例,分两组：观察组为因胰胆管发育异常所致,21例;对照组为非胰胆管发育异常、非结石因素导致的胰腺炎154例。收集两组临床资料进行回顾性分析。结果　胰腺炎中胰胆管发育异常发生率为12.0%,其中,观察组平均发病年龄（4.1±2.4）岁,略低于对照组（6.0±4.7）岁,差异无统计意义（P＞0.05）;观察组女性构成比为66.7%,明显高于对照组的51.3%,差异有统计学意义（P＜0.05）。观察组谷氨酸氨基转移酶、门冬氨酸氨基转移酶、γ-谷氨酰基转移酶、血清总胆红素较对照组明显升高（P＜0.01）。磁共振胰胆管造影（MRCP）对于胰胆管发育异常的阳性检出率最高,为89.5%。主要影像学表现为胰胆管合流异常、胰胆管先天变异、先天性胆管扩张症等。结论　由胰胆管发育异常所致的儿童胰腺炎,发病年龄较小,且女性儿童多见。当胰腺炎患儿合并胆汁淤积、肝功能异常时应尽早行MRCP等影像学检查以提高胰胆管发育异常的检出率。内科治疗可暂时缓解症状,尽早行介入治疗或外科手术治疗可以改善预后。     

Pancreatitis as an unusual presentation of rhabdomyosarcoma

The most common etiologies of acute pancreatitis in children are trauma, multi‐system disease, drugs, infections, idiopathic and congenital anomalies of the pancreaticobiliary system. Acute pancreatitis is rarely associated with underlying childhood malignancies. We report a 12‐year‐old male with acute pancreatitis as the presenting symptom of an alveolar metastatic rhabdomyosarcoma. Pediatr Blood Cancer 2009;52:879–880. © 2009 Wiley‐Liss, Inc.     

Continuous regional arterial infusion effective for children with acute necrotizing pancreatitis even under neutropenia

Severe acute pancreatitis is one of the critical conditions that may develop in children with cancer. The leading cause of death due to acute pancreatitis is infectious pancreatitis or circulation collapse. Therefore, patients who develop acute pancreatitis while undergoing chemotherapy or after hematopoietic transplantation are at risk for a life‐threatening and fatal course. We treated 140 patients with malignancy from April 2002 to March 2009 at our hospital and encountered 3 patients under neutropenia who developed severe acute pancreatitis. Two of them were successfully treated with continuous regional arterial infusion of a protease inhibitor and antibiotic even under agranulocytosis. Another patient was treated with conventional therapy with intravenous antibiotics plus a protease inhibitor and total or partial parenteral nutrition. Even though the two patients treated with continuous regional arterial infusion presented much more severe conditions, their symptoms resolved earlier. In conclusion, acute pancreatitis is one of the severe complications of childhood malignancy. Even under agranulocytosis, continuous regional arterial infusion of a protease inhibitor and antibiotic was well tolerated and effective among our cases and might reduce early death due to pancreatitis.     

L-asparaginase-induced severe necrotizing pancreatitis successfully treated with percutaneous drainage

L-asparaginase is a key component of the antileukemic therapy in children with acute lymphoblastic leukemia (ALL). Pancreatitis has been noted to be a complication in 2-16% of patients undergoing treatment with L-asparaginase for a variety of pediatric neoplasms. Most cases of pancreatitis associated with L-asparaginase toxicity are self-limiting and respond favorably to nasogastric decompression and intravenous hyperalimentation. However, in rare instances, hemorrhagic pancreatitis or necrosis may occur. L-asparaginase-induced pancreatitis is an uncommon but potential lethal complication of the treatment of leukemia. We present a pediatric patient with leukemia and a severe, L-asparaginase-induced necrotizing pancreatitis, treated successfully with percutaneous drainage used to flush the infected necrotic parts.     

Traumatic pancreatitis: long-term review of initial non-operative management in children

OBJECTIVE: To assess the safety and long-term efficacy of an initial non-operative approach to the management of pancreatic trauma in children. METHODOLOGY: Retrospective case-note review of children admitted to our institution with the diagnosis of pancreatic trauma over a 10-year period from 1985 to 1994 inclusive. RESULTS: Fourteen children were identified with a diagnosis of traumatic pancreatitis. Eleven of the injuries were secondary to motor vehicle accidents or trauma from a bicycle handlebar. In seven patients there were associated intra-abdominal injuries and in eight patients the traumatic pancreatitis was complicated by development of a pseudocyst. Three pseudocysts settled without intervention, two resolved with percutaneous external drainage and the remaining three required open surgery. The mean in-patient stay was 21.2 days. The average length of follow-up was 7.7 years with no observed long-term complications. CONCLUSIONS: Non-operative management of traumatic pancreatitis in the absence of complete duct transection is safe in children and does not appear to be associated with adverse sequelae.     

Acute and recurrent pancreatitis in children: etiological factors

OBJECTIVES: To describe the clinical picture and outcome, and to assess the etiological factors of acute and recurrent pancreatitis in children. METHODS: Thirty-six (65.5%) patients with acute and 19 (34.5%) with recurrent pancreatitis were studied. Mean age was 126 (41.3 SD) months; 27 (49.1%) were females. SETTING: A pediatric referral hospital. PERIOD: 2000-2005. DESIGN: Cross-sectional. VARIABLES: Clinical and laboratory data and etiological factors. STATISTICS: Chi2-test, Fisher test, OR, confidence interval, Student t-test and Mann-Whitney U-test. RESULTS: The most frequent symptom in acute and recurrent pancreatitis was abdominal pain, followed by vomiting and ileus. The severity of pancreatitis and complications were similar in both groups. Biliary stones, family history of pancreatitis, drug ingestion and hypercalcemia occurred in both groups. Abdominal trauma and acute hepatitis A occurred in patients with acute pancreatitis; triglyceride>5.65 mmol/L, pancreas divisum and DeltaF508 mutation occurred in patients with recurrent pancreatitis. No difference was observed when frequency factors between study groups were compared. CONCLUSIONS: The clinical picture and etiological factors were similar in both groups. Since one out of every three children with acute pancreatitis in this series presented recurrences, it was not considered to be a 'benign disease'. Fifteen different etiological factors were identified in two-thirds of the cases.     

儿童急性胰腺炎的肠内营养治疗进展

急性胰腺炎是儿童常见急腹症之一,机体处于高分解、高代谢状态,极易发生营养不良,合理的营养支持治疗对于疾病的康复至关重要。较之肠外营养,肠内营养能改善急性胰腺炎患儿的肠道屏障,减少肠源性感染和多脏器功能衰竭的发生率,缩短住院天数,降低住院费用,且早期肠内营养(入院48 h内)更具优势。若胃肠道功能能耐受,尽早开始肠内营养;肠内营养的实施并不局限于空肠喂养,经鼻胃管喂养亦安全、有效。     

Pancreatitis in children. A study in the North of England.

Cases of pancreatitis in children in the Newcastle Regional Board area were studied over approximately 6 years. There were 9 cases aged between 6 weeks and 15 years. In 5 children (all under 7 years of age) likely aetiological factors were found (2 cases of mumps and 1 case wach due to trauma, measles, and hereditary pancreatitis). In 4 children all over 13 years of age no likely aetiological factor was found. It is speculated that they may represent one end of the age spectrum of idiopathic pancreatitis as it occurs in adults. Hereditary pancreatis was definitely found in only one child and possibly in one other. Therefore, it probably does not represent the major cause of pancreatitis in children as has been previously thought. One child died, but all the other children have thrived since their illness.     

儿童重症急性胰腺炎的诊治进展

儿童重症急性胰腺炎的发病率较低,起病急骤凶险,且症状不典型,有些症状缺乏特异性,病死率相对较高.本文将对重症急性胰腺炎的诊断、治疗进展作一综述.     

Massive bleeding into the upper gastro-intestinal tract in hereditary chronic calcifying pancreatitis in the child.

This report deals with a young girl affected with chronic hereditary calcifying pancreatitis, which gave rise to acute life-threatening arterial bleeding into the upper gastro-intestinal tract. A pancreatic pseudocyst forming in the chronically affected pancreas spontaneously perforated into the stomach, thereby eroding a gastric wall artery. We think it is conceivable that in the future this type of complication in children with pancreatitis and pseudocyst formation might occur more frequently.     

Why not choledochal cyst?

With excision as a standard procedure for treating choledochal cyst, early diagnosis becomes a central issue in reducing morbidity and mortality from that disorder. In a retrospective analysis of 35 patients treated over a 10-year period, we found the diagnosis was delayed for an average of 11.9 months from the beginning of presenting symptoms. Three factors were responsible: (1) a random combination of non specific presenting symptoms such as pain and vomiting or jaundice and hepatomegaly frequently led to a diagnosis such as acute pancreatitis or hepatitis, in which conservative treatment was considered most appropriate; (2) the three parameters commonly used to diagnose acute pancreatitis (serum amylase, lipase, and Cam/Ccr) may all be elevated in patients with a choledochal cyst; and (3) a combination of several noninvasive examinations such as radionuclide cholescintigraphy, ultrasonography, and upper gastrointestinal X-rays may fail to diagnose some difficult cases of choledochal cyst. To obviate such problems, we recommend a routine ultrasound examination of biliary tract in children with recurrent or progressive obstructive jaundice or unexplained acute pancreatitis. When these noninvasive examinations fail to solve the problem, endoscopic retrograde cholangiopancreatography should be considered. Offprint requests to: J.-H. Chuang