The Etiology of Acute Recurrent Pancreatitis in Children

Lucidi, Vincenzina; Alghisi, Federico; Dall’Oglio, Luigi; D’Apice, Maria Rosaria; Monti, Lidia; Angelis, Paola De; Gambardella, Stefano; Angioni, Adriano; Novelli, Giuseppe

doi:10.1097/mpa.0b013e318214fe42

Cited by 74 publications

(61 citation statements)

References 40 publications

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“…Sánchez-Ramirez et al 18 observaram 36 crianças com diagnóstico de PA e 19 com pancreatite recorrente, concluindo que em cerca de 35% dos casos a causa era idiopática, mesmo após investigação completa, conforme já apontado em estudos anteriores, que descrevem valores entre 10-20% de PA idiopática em crianças 19,20 . Ainda, evidências recentes apontam que parte significativa dos casos de pancreatite idiopática em crianças está relacionada a mutações genéticas diversas 18,21 , responsáveis pela ocorrência da doença diretamente ou predisponentes desta, muitas das quais localizadas no braço longo do cromossomo 7 (7q35), onde muitos genes envolvidos com a transcrição e regulação do tripsinogênio estão localizados 22 .…”

Section: Pancreatite Aguda Recorrente Hereditária: Aspectos Genéticosunclassified

Acute pancreatitis in pediatrics: a systematic review of the literature

2012

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Objective: To describe the main epidemiological, clinical, diagnostic and treatment aspects of children with acute pancreatitis.Sources: Systematic review of MEDLINE and SciELO databases in the last 5 years on acute pancreatitis in children, as well as consultation of relevant references on the texts obtained. Summary of the findings:Cases of acute pancreatitis in children have received growing attention in recent years, and an increase in the number of cases has been reported in several studies. The main etiologies in children involve biliary disease, drug-induced pancreatitis, recurrent hereditary pancreatitis and trauma, and up to 30% of cases have no defined etiology. The diagnosis is based on the combination of clinical and laboratory aspects with the increase of acinar enzymes and radiologic tests. Initial support treatment, with proper volume replacement and correction of the metabolic disturbances, besides specific nutritional therapy, are the fundamental points in the handling of acute conditions. Long term complications are unusual, and mortality rates are inferior to the rates for the adult population. Conclusions:The early diagnosis and the appropriate handling can contribute to a better outcome for the child with pancreatitis and to prevent the immediate and late complications related to the disease. More studies are required to better explain aspects related to the clinical and radiological diagnosis of pancreatitis in children, as well as aspects related to the nutritional therapy for this age group. J Pediatr (Rio J). 2012;88(2):101-14: Pancreatitis, pediatrics, nutritional support, morbidity, enteral nutrition. ResumoObjetivo: Descrever os principais aspectos epidemiológicos, clínicos, diagnósticos e do tratamento de crianças com pancreatite aguda. Fontes dos dados:Realizada revisão sistemática das bases de dados MEDLINE e SciELO nos últimos 5 anos sobre pancreatite aguda em crianças, bem como consultadas referências relevantes dos textos obtidos. Síntese dos dados:Os casos de pancreatite aguda em crianças recebem crescente atenção nos últimos anos, sendo verificado um aumento na incidência da doença em diversos estudos. As principais etiologias em crianças envolvem doença biliar, pancreatite secundária a medicamentos, pancreatite hereditária recorrente e trauma, sendo até 30% dos casos sem etiologia definida. O diagnóstico baseia-se na combinação de aspectos clínicos, laboratoriais com elevação das enzimas acinares e testes radiológicos. Tratamento de suporte inicial, com reposição volêmica adequada e correção dos distúrbios metabólicos, além de terapêutica nutricional específica, são os pontos fundamentais no manejo dos quadros agudos. Complicações a longo prazo são incomuns, e as taxas de mortalidade, inferiores às da população adulta. Conclusões:O diagnóstico precoce e o manejo apropriado podem contribuir para a melhor evolução da criança com pancreatite e prevenir as complicações imediatas e tardias relacionadas à doença. Mais estudos são necessários para melhor elucidar aspectos re...

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Section: Pancreatite Aguda Recorrente Hereditária: Aspectos Genéticosunclassified

Acute pancreatitis in pediatrics: a systematic review of the literature

2012

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“…In non-CF children, a single episode of acute pancreatitis can lead to insulin requiring diabetes mellitus (67). Pancreatic-sufficient CF patients are most susceptible to acute pancreatitis during adolescence and young adulthood (68)(69)(70), while pancreatic-insufficient patients rarely develop acute pancreatitis. Based on the CF ferret, inflammation association with the onset of exocrine pancreas decline appears closely linked to spontaneous glucose abnormalities and worsening of glucose tolerance.…”

Section: Methodsmentioning

confidence: 99%

Abnormal endocrine pancreas function at birth in cystic fibrosis ferrets

Olivier¹,

Yi²,

Sun³

et al. 2012

J. Clin. Invest.

120

143

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Diabetes is a common comorbidity in cystic fibrosis (CF) that worsens prognosis. The lack of an animal model for CF-related diabetes (CFRD) has made it difficult to dissect how the onset of pancreatic pathology influences the emergence of CFRD. We evaluated the structure and function of the neonatal CF endocrine pancreas using a new CFTR-knockout ferret model. Although CF kits are born with only mild exocrine pancreas disease, progressive exocrine and endocrine pancreatic loss during the first months of life was associated with pancreatic inflammation, spontaneous hyperglycemia, and glucose intolerance. Interestingly, prior to major exocrine pancreas disease, CF kits demonstrated significant abnormalities in blood glucose and insulin regulation, including diminished first-phase and accentuated peak insulin secretion in response to glucose, elevated peak glucose levels following glucose challenge, and variably elevated insulin and C-peptide levels in the nonfasted state. Although there was no difference in lobular insulin and glucagon expression between genotypes at birth, significant alterations in the frequencies of small and large islets were observed. Newborn cultured CF islets demonstrated dysregulated glucose-dependent insulin secretion in comparison to controls, suggesting intrinsic abnormalities in CF islets. These findings demonstrate that early abnormalities exist in the regulation of insulin secretion by the CF endocrine pancreas. IntroductionCystic fibrosis (CF) is caused by defects in the CF transmembrane conductance regulator (CFTR) chloride channel. Cystic fibrosisrelated diabetes (CFRD) is a common complication of CF and affects 20%-25% of adolescents and 40%-50% of individuals over 30 years of age (1,2). CFRD is associated with worsening clinical status, including reduced pulmonary function, increased frequency of pulmonary exacerbations, and a decline in nutritional status (3-7). Furthermore, CFRD leads to increased mortality compared with CF patients without diabetes (4,8). Thus, early diagnosis and treatment are vital to improving clinical outcome of CFRD patients.While the pathophysiology of CFRD is multifactorial, delayed insulin secretion appears to be a key hallmark of disease progression (9-12), and the health of CF patients is improved by insulin therapy prior to and following diagnosis of overt diabetes (13,14). Partial insulin deficiency occurs in part due to islet loss associated with exocrine pancreas disease (15-18). However, CF pancreata at CFRD autopsy demonstrate that remaining islets contain roughly half the number of insulin-positive cells found in non-CF controls (17,18), and this degree of β cell loss is thought to be insufficient to explain diabetes (19). Thus, insulin deficiency in CFRD is relative and not absolute. All stages of CFRD are characterized by abnormalities in circulating insulin levels (10,12,20,21). Impaired first-phase insulin (IFPI) responses are common in CFRD patients, but also occur in approximately 50% of CF children with normal glucose tolerance (9). Cu...

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“…Causes of recurrent pancreatitis in pediatrics are different than those in adults. While hereditary pancreatitis is one of the causes that should be excluded by genetic testing for (CFTR, PRSS1, and SPINK1 gene mutations) [5] and this was negative. Also the imaging studies showed no sign of pancreatic inflammation of duct injury in any of the imaging this patient had.…”

Section: Discussionmentioning

confidence: 99%

“…There is no history of abdominal trauma and his initial episode of pancreatitis was evaluated extensively and it was considered idiopathic. Since that time has had at least five known episodes of pancreatitis with mild elevation of his amylase and lipase (range: 200 -300), he had negative genetic testing for the hereditary pancreatitis common mutations (CFTR, PRSS1, and SPINK1 gene mutations) [5]. He was found to be heterozygous for HLA DQ2, but celiac serologies were negative.…”

Section: Case Reportmentioning

confidence: 99%

Rare Case of Duplication Cyst was Misdiagnosed as Pancreatic Pseudocyst

Absah

2013

J Med Cases

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A 7-year-old boy presents with a history of recurrent pancreatitis. His abdominal pain started in 2007, but his first episode of pancreatitis was in January 2010. Since that time has had at least five known episodes of pancreatitis with mild elevation of his amylase and lipase (range: 200 -300). There is no history of abdominal trauma and he had negative genetic testing for the hereditary pancreatitis (CFTR, PRSS1, and SPINK1 gene mutations). He had numerous imaging studies revealing a persistent cystic lesion near the pancreatic head, since his first episode of pancreatitis. Computed tomography of the abdomen showed thick-walled U-shaped cystic structure lying between the head of the pancreas, the inferior gastric antrum and the proximal descending duodenum. An abdominal US demonstrated a bi-lobed thick-walled cystic mass with a positive rim sign. Recurrent pancreatitis is uncommon in pediatric patients. Causes of recurrent pancreatitis in pediatrics are different than those in adults. While hereditary pancreatitis is one of the causes, the genetic testing was negative and there was no sign of pancreatic injury or inflammation in any of the images. Pancreatic pseudocyst is a complication of acute pancreatitis and usually has a thin wall instead of a thick wall, and it's unlikely to be present during the first episode of pancreatitis. Foregut duplication cyst is a rare (about 1 in 4,500) congenital anomaly that can happen anywhere throughout the GI tract. The US finding of a rim sign is a typical appearance of a duplication cyst and usually is managed by surgical resection. Even though foregut duplication is a rare congenital anomaly, it should always be considered in the differential diagnosis of cases of chronic pancreatis, especially with the presence of thick walled cystic formation on imaging studies.

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The Etiology of Acute Recurrent Pancreatitis in Children

Abstract: Recurrent pancreatitis in children has several etiologies. Genetic testing confirms the high frequency of CFTR mutations. This suggests that it is of some value to identify patients with late-onset CF and CFTR-related disorders.

Cited by 74 publications

References 40 publications

Acute pancreatitis in pediatrics: a systematic review of the literature

Acute pancreatitis in pediatrics: a systematic review of the literature

Abnormal endocrine pancreas function at birth in cystic fibrosis ferrets

Rare Case of Duplication Cyst was Misdiagnosed as Pancreatic Pseudocyst

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