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BACKGROUND: Exocrine pancreatic insufficiency (EPI) is an extremely rare complication of hemolytic uremic syndrome related to Shiga toxin-producing Escherichia coli (STEC-HUS) and, to our knowledge, only one patient has been reported to have received pancreatic enzyme replacement therapy (PERT). Furthermore, STEC-HUS is not usually included among EPI causes. CASE DIAGNOSIS/TREATMENT: We report a 4-year-old girl with STEC-HUS who required dialysis and 4 days after admission developed acute pancreatitis (ACPAN) and diabetes mellitus (DM). Amylase and lipase normalized 15 days later but on the 73rd day of admission, she presented abdominal discomfort, bloating, and bulky and malodorous stools with a low fecal elastase-1 level (FE-1) of 15.74 µg/g confirming EPI diagnosis. She received 3 months of PERT until normalization of FE-1 levels. CONCLUSIONS: In children with STEC-HUS with ACPAN or DM, a high index of suspicion for EPI is required, since its symptoms are often mild, nonspecific, or delayed. In addition, STEC-HUS should be further recognized as a cause of secondary EPI.

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ABSTRACT Background: The treatment of chronic pancreatitis does not consistently solve intestinal abnormalities, and despite the implementation of various therapeutic measures, patients often continue to experience persistent diarrhea. Therefore, it is imperative to recognize that diarrhea may stem from factors beyond pancreatic insufficiency, and intestinal inflammation emerges as a potential contributing factor. Objective: The aim of this study was to assess fecal lactoferrin and calprotectin levels as indicators of intestinal inflammation in patients with chronic pancreatitis experiencing persistent diarrhea. Methods: In this study, 23 male patients with chronic pancreatitis primarily attributed to alcohol consumption and presenting with diarrhea (classified as Bristol stool scale type 6 or 7), underwent a comprehensive evaluation of their clinical and nutritional status. Fecal lactoferrin and calprotectin levels were mea­sured utilizing immunoassay techniques. Results: The average age of the participants was 54.8 years, 43.5% had diabetes, and 73.9% were smokers. Despite receiving enzyme replacement therapy and refraining from alcohol for over 4 years, all participants exhibited persistent diarrhea, accompanied by elevated calprotectin and lactoferrin levels indicative of ongoing intestinal inflammation. Conclusion: The findings of this study underscore that intestinal inflammation, as evidenced by elevated fecal biomarkers calprotectin and lactoferrin, may contribute to explaining the persistence of diarrhea in patients with chronic pancreatitis.

RESUMO Contexto: O tratamento da pancreatite crônica não resolve de forma consistente as anomalias intestinais e, apesar da implementação de várias medidas terapêuticas, os pacientes muitas vezes continuam a apresentar diarreia persistente. Portanto, é imperativo reconhecer que a diarreia pode resultar de fatores além da insuficiência pancreática, e a inflamação intestinal surge como um potencial fator contribuinte. Objetivo: O objetivo deste estudo foi avaliar os níveis fecais de lactoferrina e calprotectina como indicadores de inflamação intestinal em pacientes com pancreatite crônica com diarreia persistente. Métodos: Neste estudo, 23 pacientes do sexo masculino com pancreatite crônica atribuída principalmente ao consumo de álcool e apresentando diarreia (classificada na escala de fezes de Bristol tipo 6 ou 7), foram submetidos a uma avaliação abrangente de seu estado clínico e nutricional. Os níveis fecais de lactoferrina e calprotectina foram medidos utilizando técnicas de imunoensaio. Resultados: A idade média dos participantes foi de 54,8 anos, 43,5% tinham diabetes e 73,9% eram fumantes. Apesar de receber terapia de reposição enzimática e abster-se de álcool por mais de 4 anos, todos os participantes apresentaram diarreia persistente, acompanhada por níveis elevados de calprotectina e lactoferrina, indicativos de inflamação intestinal contínua. Conclusão: Os achados deste estudo ressaltam que a inflamação intestinal, evidenciada pelos biomarcadores fecais elevados calprotectina e lactoferrina, pode contribuir para explicar a persistência da diarreia em pacientes com pancreatite crônica.

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La fibrosis quística (FQ) es un trastorno hereditario, de las glándulas de secreción exocrina, considerada la causa más frecuente de insuficiencia pancreática exocrina (IPE) en la infancia. Esta revisión resume el panorama del tratamiento de reposición enzimática en la IPE asociada a la FQ, las formulaciones disponibles, su dosificación y modo de administración, así como las limitaciones y desafíos actuales y las posibles áreas del desarrollo futuro

Cystic fibrosis (CF) is an inherited disorder of the exocrine secretion glands, considered the most frequent cause of exocrine pancreatic insufficiency (EPI) in childhood. This review summarizes the landscape of enzyme replacement therapy in PEI associated with CF, the available formulations, their dosage and mode of administration, as well as the current limitations and challenges and potential areas for future development.

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BACKGROUND AND AIMS: Irritable bowel syndrome (IBS) is one of the most frequent disorders in clinical practice, with a mean 7.6-10.8% worldwide prevalence. A study showed that 6.1% of patients with diarrhea-predominant IBS (IBS-D) had severe exocrine pancreatic insufficiency (EPI). We aimed to identify the prevalence of EPI based on fecal elastase stool testing (Fel-1) in IBS-D and the clinical characteristics that may predict the diagnosis of EPI. METHODS: Patients aged > 18 years presenting to tertiary hospital outpatient clinics with IBS-D completed validated questionnaires and gave a stool sample where Fel-1 concentration was measured. Patients with Fel-1 < 100 µg/g represented EPI and > 100 to < 200 µg/g underwent testing for pancreatic pathology with laboratory and endoscopic ultrasound (EUS) evaluation. RESULTS: One hundred forty patients (mean age 60 years, females 75.7%) were studied. EPI was found in 5% (95% CI 2.2-10.4), and pancreatic steatosis was the main EUS finding (71%). Dyspepsia was an independent factor associated with EPI (OR 34.7; 95% CI 4.95-366.37, p = 0.0007). After pancreatic enzyme replacement therapy (PERT), patients showed a significant improvement in the Bristol stool scale (p < 0.0001), bowel movements per day (p < 0.005), distension score (0.0009), pain score (0.0277) and IBS severity (0.0034). CONCLUSION: EPI is present in 5% of patients who fulfill Rome IV criteria for D-IBS, and dyspepsia was an independent symptom strongly associated with EPI. Pancreatic steatosis was the main endoscopic ultrasound finding. After PERT therapy, patients had significantly improved stool frequency, stool consistency, abdominal pain, distension and IBS severity score.

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Cystic fibrosis is a monogenic and autosomal recessive disease. It is caused by mutations in the Cystic Fibrosis Transmembrane Conductance Regulator gene responsible for encoding the CFTR protein. Involvement of the gastrointestinal and respiratory systems is the main clinical manifestation. In this case, we report a heterozygous CFTR patient harboring class I (p.Gly542*) and class V (c.2657+5G>A) mutations. The importance of this case report lies in the clinical features because the patient, aged 3 years, presented with early exocrine pancreatic insufficiency, which can be considered atypical, as most individuals with this genotype are pancreatic sufficient or develop pancreatic insufficiency later in life. This report aims at presenting the tests requested that contributed to the patient's diagnosis, as well as at understanding the association between these mutations and their phenotypic presentation. Interpretation of the genotype-phenotype relationship represents a challenge, as genetic analysis alone is not sufficient to clearly predict severity of the disease. This is because the significant phenotypic heterogeneity existing among patients with the same genotype may exert socioeconomic and sociocultural influences, or by the action of CFTR modifiers, such as environmental and modifying genes, which can alter the protein's function and exert an impact on the individual's phenotype.

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Resumen Introducción: la pancreatitis crónica (PC) es una inflamación progresiva del páncreas que puede llevar a su destrucción e insuficiencia irreversibles. Es una patología desafiante para el médico, debido a que establecer su diagnóstico puede tomar meses e incluso años, el seguimiento de pacientes suele ser problemático y el conocimiento sobre la clínica y epidemiología en Colombia es incompleto. Este estudio pretende hacer una descripción de pacientes con PC en un centro de referencia en gastroenterología de Cali, Colombia. Metodología: estudio de corte transversal de pacientes adultos con PC confirmada por criterios clínicos y radiológicos entre 2011 y 2017. Resultados: se incluyeron 36 pacientes con PC. La mayoría fueron hombres (72,2%) y la media de edad fue 56 (+ 15,1) años. El dolor abdominal crónico fue la presentación clínica más común (83,3%). Cerca de un cuarto de pacientes presentaba diabetes mellitus (22,2%). Se consideró etiología idiopática en 58,3%, alcohólica en el 11,0% y biliar en el 11,0%. La tomografía axial computarizada (TAC), resonancia magnética (RMN) y colangiopancreatografía por RMN fueron los métodos diagnósticos más usados (60,5%), con los que en su mayoría se visualizó atrofia (53,1%), dilatación de conductos (49,0%) y calcificaciones del páncreas (34,7%). Conclusión: los síntomas inespecíficos de la PC en fases iniciales y su largo curso clínico favorecen al subdiagnóstico de esta patología. Los resultados presentados pueden contribuir a la futura creación de escalas clínicas locales que orienten estudios radiológicos y genéticos tempranos, con el fin de lograr un diagnóstico oportuno y mejorar la calidad de vida de estos pacientes.

Abstract Introduction: Chronic pancreatitis (CP) is a progressive inflammation of the pancreas that can lead to irreversible damage and failure. This condition poses great challenges to physicians since its diagnosis can take months or even years. Patient follow-up is often problematic and knowledge about its clinical presentation and epidemiology in Colombia is scarce. This study aims to describe patients with CP treated at a gastroenterology reference center in Cali, Colombia. Methodology: Cross-sectional study in adult patients with CP confirmed based on clinical and radiological criteria between 2011 and 2017. Results: 36 patients with CP were included. The majority were men (72.2%), and the mean age was 56 (+15.1) years. Chronic abdominal pain was the most common clinical presentation (83.3%). About a quarter of patients had diabetes mellitus (22.2%). Etiology was idiopathic in 58.3%, alcoholic in 11.0%, and biliary in 11.0%. Computed tomography (CT), magnetic resonance imaging (MRI), and MRI cholangiopancreatography were the most commonly used diagnostic methods (60.5%), showing mostly atrophy (53.1%), duct dilation (49.0%), and pancreatic calcifications (34.7%). Conclusion: Nonspecific symptoms of CP in early stages and its long clinical course favor the underdiagnosis of this condition. The results presented may contribute to the future creation of local clinical scales that guide early radiological and genetic studies to achieve a timely diagnosis and improve the quality of life of these patients.

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PURPOSE: The objective of this study was to describe the clinical phenotypes of children and adolescents with cystic fibrosis (CF); and to assess the role of pancreatic insufficiency and neonatal screening in diagnosis. METHODS: A cross-sectional study was conducted, which included 77 patients attending a reference center of CF between 2014 and 2016. Epidemiological data, anthropometric measurements, and the presence of pulmonary, pancreatic, gastrointestinal and hepatobiliary manifestations were evaluated based on clinical data and complementary examinations. RESULTS: Of the 77 patients, 51.9% were male, with a median age of 147 months (7.0-297.0 months), and the majority showed adequate nutritional status. The most common phenotype was pulmonary (92.2%), followed by pancreatic (87.0%), with pancreatic insufficiency in most cases. Gastrointestinal manifestation occurred in 46.8%, with constipation being the more common factor. Hepatobiliary disease occurred in 62.3% of patients. The group with pancreatic insufficiency was diagnosed earlier (5.0 months) when compared to the group with sufficiency (84.0 months) (p=0.01). The age of diagnosis was reduced following implementation of neonatal screening protocols for CF (6.0 months before vs. 3.0 months after, p=0.02). CONCLUSION: The pulmonary phenotype was the most common, although extrapulmonary manifestations were frequent and clinically relevant, and should mandate early detection and treatment. Neonatal screening for CF led to earlier diagnosis in patients with pancreatic failure, and therefore, should be adopted universally.

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OBJECTIVE: To assess the benefits of pancreatic enzyme replacement therapy (PERT) in children with complicated severe acute malnutrition. STUDY DESIGN: We conducted a randomized, controlled trial in 90 children aged 6-60 months with complicated severe acute malnutrition at the Queen Elizabeth Central Hospital in Malawi. All children received standard care; the intervention group also received PERT for 28 days. RESULTS: Children treated with PERT for 28 days did not gain more weight than controls (13.7 ± 9.0% in controls vs 15.3 ± 11.3% in PERT; P = .56). Exocrine pancreatic insufficiency was present in 83.1% of patients on admission and fecal elastase-1 levels increased during hospitalization mostly seen in children with nonedematous severe acute malnutrition (P <.01). Although the study was not powered to detect differences in mortality, mortality was significantly lower in the intervention group treated with pancreatic enzymes (18.6% vs 37.8%; P < .05). Children who died had low fecal fatty acid split ratios at admission. Exocrine pancreatic insufficiency was not improved by PERT, but children receiving PERT were more likely to be discharged with every passing day (P = .02) compared with controls. CONCLUSIONS: PERT does not improve weight gain in severely malnourished children but does increase the rate of hospital discharge. Mortality was lower in patients on PERT, a finding that needs to be investigated in a larger cohort with stratification for edematous and nonedematous malnutrition. Mortality in severe acute malnutrition is associated with markers of poor digestive function. TRIAL REGISTRATION: ISRCTN.com: 57423639.

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ABSTRACT Objective: To determine the prevalence of malnutrition in patients attending an adult cystic fibrosis (CF) program and to investigate the associations of malnutrition with the clinical characteristics of those patients. Methods: This was a cross-sectional study involving patients with clinically stable CF patients (16 years of age or older). The patients underwent clinical assessment, nutritional assessments, pulmonary function tests, and pancreatic function assessment. They also completed a questionnaire regarding diet compliance. On the basis of their nutritional status, the patients were classified divided into three groups: adequate nutrition; at nutritional risk; and malnutrition. Results: The study has included 73 patients (mean age, 25.6 ± 7.3 years), 40 of whom (54.8%) were female. The mean body mass index was 21.0 ± 3.0 kg/m2 and the mean FEV1 was 59.7 ± 30.6% of predicted. In this sample of patients, 32 (43.8%), 23 (31.5%), and 18 (24.7%) of the patients were allocated to the adequate nutrition, nutritional risk, and malnutrition groups, respectively. The logistic regression analysis identified three independent factors associated with the risk of malnutrition: Shwachman-Kulczycki score, percent predicted FEV1; and age. Conclusions: Malnutrition remains a common complication in adolescents and adults with CF, despite dietary advice. Malnutrition is associated with age, clinical severity, and lung function impairment.

RESUMO Objetivo: Determinar a prevalência de desnutrição em pacientes de um programa para adultos com fibrose cística (FC) e investigar a relação da desnutrição com as características clínicas desses pacientes. Métodos: Trata-se de um estudo transversal com pacientes com FC clinicamente estáveis (com 16 anos de idade ou mais). Os pacientes foram submetidos a avaliação clínica, avaliação nutricional, testes de função pulmonar e avaliação da função pancreática. Eles também preencheram um questionário sobre sua adesão à dieta proposta. Os pacientes foram divididos em três grupos, de acordo com seu estado nutricional: nutrição adequada, risco nutricional e desnutrição. Resultados: O estudo incluiu 73 pacientes (média de idade: 25,6 ± 7,3 anos), dos quais 40 (54,8%) eram do sexo feminino. A média do índice de massa corporal foi de 21,0 ± 3,0 kg/m2, e a média do VEF1 foi de 59,7 ± 30,6% do previsto. Nessa amostra de pacientes, 32 (43,8%) ficaram no grupo com nutrição adequada, 23 (31,5%) ficaram no grupo com risco nutricional e 18 (24,7%) ficaram no grupo com desnutrição. A análise de regressão logística identificou três fatores independentes relacionados com o risco de desnutrição: escore de Shwachman-Kulczycki, VEF1 em % do previsto e idade. Conclusões: A desnutrição ainda é uma complicação comum em adolescentes e adultos com FC, não obstante o aconselhamento dietético. A desnutrição está relacionada com a idade, a gravidade clínica e o comprometimento da função pulmonar.

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Desde hace más de 70 años se conoce la asociación de diarrea con diabetes mellitus. En pacientes diabéticos su prevalencia es de alrededor del 20%. Sus manifestaciones clínicas son diversas, y representa un reto diagnóstico y terapéutico. Existen ciertos diagnósticos de mayor prevalencia en pacientes diabéticos que en la población general. Las distintas etiologías relacionadas pueden ser diagnosticadas adecuadamente a través de la historia clínica y pruebas diagnósticas complementarias. Los medicamentos utilizados por el paciente diabético para el manejo de su enfermedad, frecuentemente causan diarrea crónica, por lo que se debe profundizar en los antecedentes farmacológicos al momento de estudiar la diarrea. Los pacientes diabéticos pueden presentar otras condiciones patológicas asociadas, como enfermedad celíaca o colitis microscópica, cuya molestia única es la diarrea. La función del páncreas exocrino puede estar disminuida en el paciente diabético, frecuentemente llevando a insuficiencia pancreática exocrina. Factores dietarios, como los edulcorantes libres de azúcar y otros agentes, pueden causar diarrea en el paciente diabético. La presencia de condiciones como la neuropatía autonómica y neuropatía periférica secundarias a la diabetes mellitus, pueden explicar desordenes como la disfunción anorrectal y la incontinencia fecal. Finalmente, la enteropatía diabética per se o con sobrecrecimiento bacteriano asociado, puede causar diarrea. Lograr un control glicémico adecuado constituye el pilar del tratamiento de la diarrea en el diabético, después de esto existen medidas adicionales que se aplican según el contexto especifico del paciente. En el presente artículo se revisan las causas de mayor incidencia diarrea en el paciente diabético y los mecanismos fisiopatológicos implicados

The association of diarrhea with diabetes mellitus has been known for more than 70 years. In diabetic patients its prevalence is around 20%.Its clinical manifestations are diverse, and represents a diagnostic and therapeutic challenge.There are certain diagnoses of higher prevalence in diabetic patients than in the general population.The different related etiologies can be adequately diagnosed through the clinical history and complementary diagnostic tests.The medications used by the diabetic patient to manage their disease often cause chronic diarrhea, so the pharmacological background should be studied at the time of the study of diarrhea.Diabetic patients can present other associated pathological conditions, such as celiac disease or microscopic colitis, which only discomfort is diarrhea.Exocrine pancreatic function may be decreased in the diabetic patient, frequently leading to exocrine pancreatic insufficiency. Dietary factors, such as sugar-free sweeteners and other agents, can cause diarrhea in the diabetic patient.The presence of conditions such as autonomic neuropathy and peripheral neuropathy secondary to diabetes mellitus may explain disorders such as anorectal dysfunction and faecal incontinence. Finally, diabetic enteropathy alone or with associated bacterial overgrowth can cause diarrhea.Achieving adequate glycemic control is the pillar of the treatment of diarrhea in the diabetic, after which there are additional measures that are applied according to the specific context of the patient.This article reviews the causes of higher diarrhea incidence in the diabetic patient and the pathophysiological mechanisms involved

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Autoimmune pancreatitis (AIP) is an uncommon disease that represents a diagnostic challenge unless it is considered as a cause of acute pancreatitis, pancreatic exocrine insufficiency and a pancreatic mass. This entity is under diagnosed and successful medical therapy is available. In this paper, we will describe a case of a 59 year-old, Hispanic woman diagnosed with autoimmune pancreatitis, a disease previously believed to affect typically older men. We will review the definition, types, clinical manifestations, radiological features, serology, histopathological findings, treatment strategies and diagnostic criteria of autoimmune pancreatitis

La pancreatitis autoinmune (PAI) es una enfermedad rara que se presenta como un reto diagnóstico a menos que sea considerada como causa de pancreatitis aguda, insuficiencia pancreática exocrina y masa pancreática. Es una enfermedad sub diagnosticada y existe una terapia médica satisfactoria. En este trabajo, describiremos un caso de una mujer hispana de 59 años diagnosticada de pancreatitis autoinmune, una enfermedad que se creía previamente que afectaba típicamente a hombres de avanzada edad. Revisaremos la definición, los tipos, las manifestaciones clínicas, hallazgos radiológicos, serología, hallazgos histopatológicos, estrategias de tratamiento y criterios diagnósticos de la pancreatitis autoinmune

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OBJECTIVE: To evaluate the safety and efficacy of a novel microbial lipase (NM-BL) in a liquid formulation for the treatment of exocrine pancreatic insufficiency (EPI) in patients with cystic fibrosis (CF) in a phase IIa proof-of-concept study. STUDY DESIGN: We conducted a double-blind, randomized, placebo controlled crossover study in patients with cystic fibrosis and exocrine pancreatic insufficiency. Adolescent and adult patients with CF were randomized to receive NM-BL or placebo for 1 week as replacement for their usual pancreatic enzyme formulation. They were subsequently crossed-over to the alternate study treatment. The coefficient of fat absorption was evaluated as the primary endpoint. Symptoms and adverse events were evaluated as secondary endpoints. RESULTS: A total of 35 patients were randomized into the study and 22 patients completed both treatment periods. During treatment with NM-BL, the coefficient of fat absorption was significantly greater (72.7%) compared with placebo (53.8%) with a difference between groups of 18.8% (P < .001). Subjective assessment of stool fat and stool consistency also improved under treatment with NM-BL. Adverse events were mostly gastrointestinal in nature and were more common in the group receiving NM-BL. CONCLUSIONS: Currently available pancreatic enzyme products are limited because of the lack of liquid formulations and being largely porcine based. The novel microbial lipase NM-BL was safe and effective in this short term trial. The trial provided clinical proof-of-concept for this novel microbial lipase as a treatment for EPI in CF. A larger phase 2 dose ranging trial is warranted. TRIAL REGISTRATION: ClinicalTrials.gov: NCT01710644.

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OBJECTIVES: To assess whether pancreatic function is impaired in children with severe acute malnutrition, is different between edematous vs nonedematous malnutrition, and improves by nutritional rehabilitation. STUDY DESIGN: We followed 89 children with severe acute malnutrition admitted to Queen Elizabeth Central Hospital in Blantyre, Malawi. Stool and blood samples were taken on admission and 3 days after initial stabilization to determine exocrine pancreatic function via fecal elastase-1 (FE-1) and serum trypsinogen and amylase levels. RESULTS: A total of 33 children (37.1%) had nonedematous severe acute malnutrition, whereas 56 (62.9%) had edematous severe acute malnutrition. On admission, 92% of patients showed evidence of pancreatic insufficiency as measured by FE-1 <200 µg/g of stool. Patients with edematous severe acute malnutrition were more likely to have low FE-1 (98% vs 82.8%, P = .026). FE-1 levels remained low in these individuals throughout the assessment period. Serum trypsinogen was elevated (>57 ng/mL) in 28% and amylase in 21% (>110 U/L) of children, suggesting pancreatic inflammation. CONCLUSION: Exocrine pancreatic insufficiency is prevalent in children with severe acute malnutrition and especially in children with edematous severe acute malnutrition. In addition, biochemical signs suggestive of pancreatitis are common in children with severe acute malnutrition. These results have implications for standard rehabilitation treatment of children with severe acute malnutrition who may benefit from pancreatic enzyme replacement therapy. TRIAL REGISTRATION: ISRCTN.com: 13916953.

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A IPE é uma doença relativamente comum em cães e rara em gatos, na qual o pâncreas é incapaz de garantir a função secretória enzimática. Um cão sem raça definida de seis anos foi atendido no Hovet -Metodista, apresentando-se caquético, com pelame opaco e histórico de fezes pastosa e em grande volume. Após anamnese, exame físico e descarte de Doença intestinal inflamatória, corpo estranho e endocrinopatia, suspeitou-se de Insuficiência pancreática exócrina, confirmando com o resultado dos exames complementares (hemograma e Imunorreatividade semelhante à tripsina sérica). Após algumas semanas da administração de Extrato de pancreatina, junto com os tratamentos complementares, houve regressão dos sintomas e conclusão do caso.

EPI is a relatively common in dogs and quite rare in cats, in which the pancreas is unable to assure its enzyme secretory function. An mixed breed dog, six years old was treated at the Metodista - Veterinary Hospital, presenting cachectic condition, opaque fur and history of large amounts of pasty feces. After anamnesis, physical examination and discarding intestinal inflammatory disease, foreign body or endocrinopathy, emerged the suspicion of exocrine pancreatic insufficiency which was confirmed with complementary tests (blood test and Tripsin-like immunoreactivity). A few weeks into the treatment, administering pancreatic enzyme extracts alongside complementary care, there was clear regression of the symptoms and conclusion of the case.

La IPE es una enfermedad relativamente común en perros y en gatos rara, en la que el páncreas no Metodista es capaz de asegurar la función de la enzima secretora. Un perro de raza mixta 6 anos fue visto en Hovet - Metodista, presentando caquéctico, con heces pastosas pieles opacas e históricos y de gran volumen. Después de la anamnesis, la exploración física y la eliminación de la enfermedad inflamatoria del intestino, cuerpo extrario y enfermedades endocrinas, se sospechó la insuficiencia pancreática exocrina, confirmando IPE con el resultado de pruebas de laboratorio (hemograma y Inmunorreactividad similar a la tripsina en suero). Después de unas semanas de la administración de extracto de pancreatina, junto con los tratamientos complementarios, sus síntomas y el cierre del caso.

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A IPE é uma doença relativamente comum em cães e rara em gatos, na qual o pâncreas é incapaz de garantir a função secretória enzimática. Um cão sem raça definida de seis anos foi atendido no Hovet -Metodista, apresentando-se caquético, com pelame opaco e histórico de fezes pastosa e em grande volume. Após anamnese, exame físico e descarte de Doença intestinal inflamatória, corpo estranho e endocrinopatia, suspeitou-se de Insuficiência pancreática exócrina, confirmando com o resultado dos exames complementares (hemograma e Imunorreatividade semelhante à tripsina sérica). Após algumas semanas da administração de Extrato de pancreatina, junto com os tratamentos complementares, houve regressão dos sintomas e conclusão do caso.(AU)

EPI is a relatively common in dogs and quite rare in cats, in which the pancreas is unable to assure its enzyme secretory function. An mixed breed dog, six years old was treated at the Metodista - Veterinary Hospital, presenting cachectic condition, opaque fur and history of large amounts of pasty feces. After anamnesis, physical examination and discarding intestinal inflammatory disease, foreign body or endocrinopathy, emerged the suspicion of exocrine pancreatic insufficiency which was confirmed with complementary tests (blood test and Tripsin-like immunoreactivity). A few weeks into the treatment, administering pancreatic enzyme extracts alongside complementary care, there was clear regression of the symptoms and conclusion of the case.(AU)

La IPE es una enfermedad relativamente común en perros y en gatos rara, en la que el páncreas no Metodista es capaz de asegurar la función de la enzima secretora. Un perro de raza mixta 6 anos fue visto en Hovet - Metodista, presentando caquéctico, con heces pastosas pieles opacas e históricos y de gran volumen. Después de la anamnesis, la exploración física y la eliminación de la enfermedad inflamatoria del intestino, cuerpo extrario y enfermedades endocrinas, se sospechó la insuficiencia pancreática exocrina, confirmando IPE con el resultado de pruebas de laboratorio (hemograma y Inmunorreactividad similar a la tripsina en suero). Después de unas semanas de la administración de extracto de pancreatina, junto con los tratamientos complementarios, sus síntomas y el cierre del caso.(AU)

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Context Fecal elastase is a noninvasive test for pancreatic insufficiency diagnosis. Objectives Evaluate the usefulness of fecal elastase 1 for the indication of exocrine pancreatic insufficiency among former alcohol addicts and patients with chronic pancreatitis. Methods Forty-three patients with chronic pancreatitis and thirty-three asymptomatic former alcohol addicts entered the study. The levels of fecal elastase 1 were measured using a commercial kit. Pancreatic imaging findings were used to categorize the groups. Results The levels of fecal elastase 1 were significantly lower in the patients than in the former alcohol addicts and in the group with tissue calcifications, duct alterations, or atrophy. With a cutoff level of 100 μg/g, the sensitivity of fecal elastase 1 in chronic pancreatitis was 46.51% and its specificity was 87.88% with a positive predictive value of 83.33% and a negative predictive value of 55.77%. When patients were stratified according to the severity of their pancreatitis, the sensitivity was 6.25% for mild pancreatitis and 70.37% for marked pancreatitis. Conclusion Low level of fecal elastase 1 was associated with marked rather than mild chronic pancreatitis; however, it may be useful to indicate pancreatic exocrine insufficiency in asymptomatic former alcohol addicts. .

Contexto O teste de elastase fecal é um teste não invasivo para diagnosticar insuficiência pancreática. Objetivos Avaliar a utilidade da elastase fecal 1 como indicador de insuficiência pancreática entre ex alcoólatras e pacientes com pancreatite crônica. Métodos Quarenta e três pacientes com pancreatite crônica e 33 ex alcoólatras assintomáticos entraram no estudo. Os níveis de elastase fecal 1 foram medidos usando kit comercial. Os achados de imagem pancreática foram usados para categorizar os grupos. Resultados Os níveis de elastase fecal 1 foram significantemente menores nos pacientes que nos ex alcoólatras e no grupo com calcificações teciduais, alterações de ductos, ou atrofia. A sensibilidade da elastase fecal 1 na pancreatite crônica foi de 46,51% e a especificidade foi de 87,88%, com valor preditivo positivo de 83,33% e valor preditivo negativo de 55,77%. Quando os pacientes foram estratificados segundo a severidade da pancreatite, a sensibilidade foi de 6,25% para pancreatite crônica leve e 70,37% para pancreatite crônica severa. Conclusão Baixo nível de elastase fecal foi associado com pancreatite crônica severa mais do que com a leve; entretanto, pode ser útil para indicar insuficiência pancreática exócrina entre os ex alcoólatras. .

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Herein the first molecular diagnosis of a Brazilian child with Shwachman-Diamond Syndrome is reported. A 6-year-old boy was diagnosed with cystic fibrosis at the age of 15 months due to recurrent respiratory infections, diarrhea and therapeutic response to pancreatic enzymes. Three sweat tests were negative. At the age of 5 years, he began to experience pain in the lower limbs, laxity of joints, lameness and frequent falls. A radiological study revealed metaphyseal chondrodysplasia. A complete blood cell count showed leukopenia (leukocytes: 3.1-3.5 x 10(3)/µL), neutropenia (segmented neutrophils: 15-22%), but normal hemoglobin, hematocrit and platelet count. A molecular study revealed biallelic mutations in the Shwachman-Bodian-Diamond Syndrome gene (183-184TA-CT K62X in exon 2 and a 258+2T-C transition) confirming the diagnosis of Shwachman-Diamond Syndrome. A non-pathologic, silent nucleotide A to G transition at position 201 was also found in heterozygosis in the Shwachman-Bodian-Diamond Syndrome gene. This is the first report to describe a Brazilian child with molecular diagnosis of Shwachman-Diamond Syndrome, a rare autosomal recessive disorder characterized by exocrine pancreatic insufficiency, intermittent or persistent neutropenia and skeletal changes. Other characteristics include immune system, hepatic and cardiac changes and predisposition to leukemia. Recurrent bacterial, viral and fungal infections are common. The possibility of Shwachman-Diamond Syndrome should be kept in mind when investigating children with a diagnosis of cystic fibrosis and normal sweat tests.

RESUMEN

Insuficiência Pancreática Exócrina (IPE) é um tipo de enfermidade gastrointestinal crônica que promovediversas alterações no organismo. O presente relato refere-se ao atendimento de um cão da raça PastorAlemão com diagnóstico de IPE com sinais clínicos de intolerância a exercícios e de insuficiência miocárdicaobservados em exames complementares antes e após a instituição do tratamento para IPE. Osautores discutem a possível participação da colecistoquinina e do déficit de taurina no desenvolvimentoda insuficiência miocárdica.

The exocrine pancreatic insufficiency (EPI) is a chronic gastrointestinal disease that promotes severalconsequences to the organism. The present case report mentions the attendance of a German Shepherddog with EPI diagnosis, intolerance to exercises and cardiac alterations in complementary examinations,before treatment. The authors argue the participation of cholecystokinin and deficit of taurine on thedevelopment of this cardiac disturbance.

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RESUMEN

Insuficiência Pancreática Exócrina (IPE) é um tipo de enfermidade gastrointestinal crônica que promovediversas alterações no organismo. O presente relato refere-se ao atendimento de um cão da raça PastorAlemão com diagnóstico de IPE com sinais clínicos de intolerância a exercícios e de insuficiência miocárdicaobservados em exames complementares antes e após a instituição do tratamento para IPE. Osautores discutem a possível participação da colecistoquinina e do déficit de taurina no desenvolvimentoda insuficiência miocárdica.(AU)

The exocrine pancreatic insufficiency (EPI) is a chronic gastrointestinal disease that promotes severalconsequences to the organism. The present case report mentions the attendance of a German Shepherddog with EPI diagnosis, intolerance to exercises and cardiac alterations in complementary examinations,before treatment. The authors argue the participation of cholecystokinin and deficit of taurine on thedevelopment of this cardiac disturbance.(AU)

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