Síndrome de quilomicronemia familiar: experiencia pediátrica en Argentina / Familial chylomicronemia syndrome: pediatric experience in Argentina

El síndrome de quilomicronemia familiar (SQF) es unaenfermedad autosómica recesiva rara, con una prevalencia1:200 000 - 1:1 000 000, y se caracteriza por quilomicronemiaen ayunas y niveles muy elevados de triglicéridos (> 880 mg/dl). LPL es el gen más frecuentemente afectado, luego APOC2,GPIHBP1, APOA5 y LMF1; todos ellos comprometen la función de la lipoproteinlipasa endotelial. El SQF suele presentarseen la infancia con dolor abdominal recurrente, xantomaseruptivos, retraso del crecimiento, pancreatitis y, en ocasiones,asintomático. El tratamiento convencional es la restriccióndietética de grasas. Se muestra el resultado clínico de 20 pacientes pediátricoscon SQF reclutados de 4 hospitales en Argentina.

Familial chylomicronemia syndrome (FCS) is a rare autosomalrecessive disease, prevalence 1:200,000 - 1:1,000,000, andis characterized by fasting chylomicrons and very hightriglycerides > 880 mg/dl. LPL is the most frequentlyaffected gene, then APOC2, GPIHBP1, APOA5, LMF1, all ofthem compromising the function of lipoproteinlipase. FCScommonly presents in childhood with recurrent abdominalpain, eruptive xanthomas, failure to thrive, pancreatitis, andsometimes asymptomatic. The conventional treatment isdietetic fat restriction. The clinical outcome of 20 pediatric patients with FCS recruited from 4 hospitals in Argentina is reported.

Association between coagulation function and prognosis in patients with acute pancreatitis / 南方医科大学学报

OBJECTIVE@#To explore the correlation of coagulation function with the severity and prognosis of acute pancreatitis (AP) and identify the laboratory markers for early prediction and dynamic monitoring of the prognosis of AP.@*METHODS@#We retrospectively analyzed the clinical data of patients with AP admitted less than 72 h after onset to our hospital from December 1, 2017 to November 30, 2018. The correlation of coagulation function-related markers at admission and their changes during hospitalization with the prognosis of the patients was analyzed.@*RESULTS@#We screened the data of a total of 1260 patients with AP against the inclusion and exclusion criteria, and eventually 175 patients were enrolled in this analysis, among whom 52 patients had severe AP (SAP) and 12 patients died. Logistic regression analysis identified vWF: Ag, PT, PC, AT Ⅲ and D-dimer markers at admission as independent risk factors for predicting SAP and death. Dynamic monitoring of the changes in coagulation function-related markers in the disease course had greater predictive value of the patients' prognosis, and the indicators including vWF: Agmax, PTmax, APTTmax, TTmax, FIBmin, D-dimermax, PLTmin, PCmin, PLGmin, AT Ⅲmin, and their variations were all independent risk factors for predicting SAP and death. ROC analysis suggested that dynamic monitoring of the changes in the indicators, especially those of △vWF: Ag, △PT, △APTT, △FIB, △TT, △D-dimer, △PLT, △PC, △AT Ⅲ, △PLG, could effectively predict SAP and death in these patients (with AUC range of 0.63-0.84).@*CONCLUSION@#Patients with AP have vascular endothelial injuries and coagulation disorders. The markers including vWF: Ag, PT, PC, AT Ⅲ and D-dimer at admission are independent risk factors for predicting SAP and death, and dynamic monitoring of the changes in vWF: Ag、PT、APTT、TT、FIB、D-dimer、PLT、PC、AT Ⅲ and PLG can further increase the predictive value.

Pancreatitis aguda en niños con enfermedades hematooncológicas: aspectos clínicos y tratamiento / Acute pancreatitis in children with hemato-oncological diseases: clinical aspects and treatment

Resumen La pancreatitis aguda es una enfermedad inflamatoria del páncreas. Se observa con mayor frecuencia en niños bajo tratamiento por alguna enfermedad hematooncológica y se asocia principalmente con la administración de L-asparaginasa. Identificar esta complicación de forma temprana y establecer un plan terapéutico adecuado puede mejorar el pronóstico y reducir el riesgo de otras complicaciones. En este trabajo se realizó una revisión crítica de la literatura actual, con especial énfasis en los aspectos clínicos, el diagnóstico y el tratamiento de la pancreatitis aguda en niños con cáncer.

Abstract Acute pancreatitis is an inflammatory disease of the pancreas. It is currently seen more frequently in children undergoing treatment for a hemato-oncological disease and it is mainly associated with the administration of L-asparaginase. The early identification of this complication and the establishment of an appropriate therapeutic plan can improve its prognosis and reduce the risk of other complications. In this article, we make a critical review of the current literature, with special emphasis on the clinical aspects, diagnosis, and treatment of acute pancreatitis in children with cancer.

Retinopatía de Purtscher, como complicación inusual de la pancreatitis / Purtscher's Retinopathy, a Rare Complication of Pancreatitis

Introducción: La retinopatía de Purtscher o ceguera por pancreatitis es una complicación poco frecuente de la pancreatitis aguda. Objetivo: Presentar a una paciente que en el curso de una pancreatitis aguda desarrolla una retinopatía de Purtscher como complicación infrecuente. Caso clínico: Paciente de piel blanca, de sexo femenino de 52 años de edad, con antecedentes de salud, que ingresa en el servicio de cirugía con el diagnóstico de pancreatitis aguda litiásica, con elementos clínicos, humorales e imaginológicos de esta entidad. Durante su ingreso presenta pérdida brusca de la visión y es diagnosticada durante su exploración oftalmológica de una retinopatía Purtscher, con resolución del cuadro a los 3 meses, previo tratamiento con esteroides por vía oral. Conclusiones: La retinopatía de Purtscher es una complicación oftalmológica poco frecuente de la pancreatitis, por lo que en todo paciente con diagnóstico de pancreatitis y alteraciones visuales hay que sospechar esta entidad(AU)

Introduction: Purtscher's retinopathy or blindness due to pancreatitis is a rare complication of acute pancreatitis. Objective: To present the case of a patient who develops Purtscher's retinopathy as a rare complication during acute pancreatitis. Clinical case: 52-year-old female white-skinned patient without a previous history of medical conditions, admitted to the surgery service with a diagnosis of acute lithiasic pancreatitis, showing clinical, humoral and imaging elements characteristic of this entity. During her admission, she presented sudden vision loss. During her ophthalmological examination, she was diagnosed with Purtscher's retinopathy. The condition disappeared at three months, after treatment with oral steroids. Conclusions: Purtscher's retinopathy is a rare ophthalmological complication of pancreatitis, a reason why this entity should be suspected in all patients diagnosed with pancreatitis and visual disturbances(AU)

Pancreatitis aguda. Diagnóstico y enfrentamiento inicial en la práctica clínica / Acute pancreatitis. Diagnosis and initial approach in clinical practice

Acute pancreatitis is a prevalent disease, with variable clinical course: several patients recover quickly and uneventfully, while others require treatment in critical care units with long hospital stay and even with a considerable mortality. The patient's symptoms, laboratory tests and radiological images allow diagnosis without major difficulties. However, early identification of more severe cases can be difficult, and it determines the adequate selection of the hospitalization unit and the quick initiation of the appropriate therapy. In this paper we give some practical treatment guidelines for the everyday clinical practice: immediate severity stratification, fluid replacement and pain control. Early enteral nutrition, monitorization of severe cases in critical care units, adequate therapeutic but no prophylactic use of antibiotics assures the best treatment results. (AU)

Preeclampsia as an inaugural manifestation of primary hyperparathyroidism: A case report / Pré-eclâmpsia como manifestação inaugural de hiperparatiroidismo primário: Um caso clínico

Abstract Primary hyperparathyroidism is an endocrine disorder characterized by hypercalcemia and elevated or inappropriately normal levels of parathyroid hormone. The diagnosis is based on a biochemical evaluation, and a neck ultrasound is the first choice during pregnancy to access the parathyroid glands. Manifestations during pregnancy are rare and can be present with life-threatening complications, so the diagnosis is challenging. The conservative treatment is limited, and there is not enough data about its safety and efficacy during pregnancy. Surgery is the only curative treatment, and a parathyroidectomy performed during the second or third trimesters is considered safe. Recently, some authors suggested an association between primary hyperparathyroidism and preeclampsia. We describe a case of preeclampsia with severe features at 27 weeks of gestational age. The severity of the preeclampsiamotivated an early termination of the pregnancy by cesarean section. During the postpartum period, the patient presented life-threatening complications, such as severe hypercalcemia and acute pancreatitis. An ultrasound exam found two parathyroid nodules, suggestive of parathyroid adenomas. The patient recovered after the pharmacological correction of the calcemia levels.

Resumo O hiperparatiroidismo primário é umdistúrbio endócrino caraterizado pela elevação do cálcio sérico associada a níveis de paratormona elevados ou inapropriadamente normais. O diagnóstico é baseado em análises bioquímicas, e, na gravidez, o exame de imagem de primeira linha é a ecografia cervical. É uma doença rara na gravidez, e pode se apresentar com complicações ameaçadoras de vida, pelo que o seu diagnóstico é desafiante. O tratamento médico disponível é limitado, havendo poucos dados relativos à sua eficácia e segurança na gravidez. A cirurgia é o único tratamento curativo, e pode ser realizada no segundo ou terceiro trimestres. Tem sido descrita uma relação entre hiperparatiroidismo primário e pré-eclâmpsia. Apresenta-se um caso de uma grávida de 27 semanas com pré-eclâmpsia com critérios de gravidade, o que obrigou ao término da gravidez por cesariana. Verificou-se agravamento clínico no período pós-parto, com aparecimento de complicações graves, tais como hipercalcemia grave e pancreatite aguda. Ecograficamente, constataram-se duas massas paratiróideias sugestivas de adenomas da paratiroide. A doente recebeu tratamento médico, e teve melhora apenas após a correção dos níveis de cálcio sérico.

Pancreatitis aguda grave en un paciente fumador / Serious acute pancreatitis in a smoking patient

Se describe el caso clínico de un paciente de 51 años de edad con antecedente patológico personal de ser un fumador empedernido, el cual acudió al Cuerpo de Guardia del Hospital Provincial Docente Dr. Joaquín Castillo Duany de Santiago de Cuba por presentar disnea asociada a cifras elevadas de tensión arterial y livedo reticular en la cara anterior abdominal. Debido al cuadro clínico y a los resultados de los exámenes complementarios, fue trasladado a la Unidad de Cuidados Intensivos, donde posteriormente se le diagnosticó pancreatitis aguda grave, tras realizar una laparotomía exploratoria y hallar zonas isquémicas en las asas intestinales. El paciente mantuvo una evolución tórpida y falleció a causa de una disfunción múltiple de órganos.

The case report of a 51 years patient with personal pathological history of being a heavy smoker is described, who went to the emergency room of Dr. Joaquín Castillo Duany Teaching Provincial Hospital in Santiago de Cuba due to a dyspnea associated with high figures of blood pressure and livedo reticularis in the abdominal anterior face. Due to the clinical pattern and the results of the complementary tests, he was referred to the Intensive Cares Unit, where later on he was diagnosed serious acute pancreatitis, after an exploratory laparotomy where ischemic areas in the intestinal loops were found. The patient maintained a torpid clinical course and he died because of a multiple dysfunction of organs.

Perfil clínico y resultados de la pancreatitis aguda en la Unidad de Cuidados Intensivos e Intermedios de un hospital general / Clinical profile and results of acute pancreatitis in the Intensive and Intermediate Care Unit of a general hospital

RESUMEN La pancreatitis aguda (PA) es un proceso inflamatorio agudo del páncreas que, de acuerdo con la clasificación de Atlanta del 2012, puede ser leve, moderada o grave. Objetivo: Describir las características epidemiológicas, clínicas, terapéuticas y los resultados de pacientes con PA ingresados a la unidad de cuidados intensivos e intermedios de un hospital general y compararlos con los descritos en la literatura nacional e internacional. Materiales y métodos: Estudio observacional de pacientes con PA atendidos en un periodo de 3 años. Resultados: Se incluyeron 59 casos; predominó el sexo femenino (54,2%), la edad media fue de 59,3 años, la etiología más frecuente fue biliar (84,7%). Los puntajes de severidad promedio al ingreso fueron APACHE II de 12,4 puntos, SOFA de 4,9 puntos y Marshall modificado de 2,8 puntos; la falla orgánica más frecuente fue la respiratoria (47,5%). La estancia media en cuidados fue 13,9 días y en el hospital fue de 23,3 días. Ningún paciente con PA leve o moderada falleció durante su estancia hospitalaria, 6 pacientes con PA grave fallecieron durante su estancia hospitalaria (20% de los casos de PA grave). Conclusión: Los casos de nuestro hospital tuvieron un perfil clínico y terapéutico semejante al descrito en la literatura mundial y latinoamericano. Se evidenció una estancia hospitalaria mayor a la descrita en trabajos recientes, pero nuestra mortalidad fue menor.

ABSTRACT Acute pancreatitis (AP) is an acute inflammatory process of the pancreas that, according to the 2012 Atlanta classification, can be mild, moderate or severe. Objective: Describe the epidemiological, clinical, therapeutic and outcomes of patients with AP admitted to the intensive care and intermediate care unit of a general hospital and compare them with those described in the national and international literature. Materials and methods: Observational study of patients with AP treated over a period of 3 years. Results: 59 cases were included; the female sex prevailed (54.2%), the average age was 59.3 years, the most frequent etiology was biliary (84.7%). Average entry severity scores were APACHE II of 12.4 points, SOFA of 4.9 points and Marshall modified of 2.8 points; The most frequent organ failure was respiratory (47.5%). The average stay in care was 13.9 days and in the hospital it was 23.3 days. No patients with mild or moderate AP died during their hospital stay, 6 patients with severe AP died during their hospital stay (20% of cases of severe AP). Conclusion: The cases of our hospital had a clinical and therapeutic profile similar to that described in the world and Latin American literature. A hospital staying was greater than that described in recent works, but our mortality was lower.

Acute pancreatitis and recurrent acute pancreatitis: an exploration of clinical and etiologic factors and outcomes / Pancreatite aguda e pancreatite aguda recorrente: uma exploração de fatores clínicos e etiológicos e desfechos

ABSTRACT Objective: To describe the epidemiology and clinical features of acute pancreatitis and recurrent acute pancreatitis in children. Methods: Observational and retrospective study with an analytical component. Patients were classified into two groups: Acute pancreatitis and recurrent pancreatitis. The relationship with each parameter obtained was analyzed using the chi-squared test, Student's t-test, or the Mann-Whitney U test. Results: There were 130 patients with acute pancreatitis; recurrent pancreatitis was diagnosed in 23.8% of the cases. The most frequent causes were anatomical (29.6%), pharmacological (19.2%), and biliary (14.6%), although in 29.2% etiology was not identified. Fasting lasted 3.5 ± 3.8 days and parenteral nutrition was indicated in 26.9% of the cases for 10.8 ± 11.3 days. A statistical association with anatomical (p = 0.02) and pharmacological causes (p = 0.01) was found in the recurrent pancreatitis group; no other differences between acute pancreatitis and recurrent pancreatitis groups were observed. The mortality rate was 3.1%, it was not attributable to acute pancreatitis in any cases. Conclusion: Acute pancreatitis is associated with a high frequency of acute recurrent pancreatitis. Severity and complications did not show statistically significant differences in this investigation. Anatomical etiologies were the most relevant cause in this cohort. Fasting time and parenteral nutrition use were relevant. Genetics testing is required in this population.

RESUMO Objetivo: Descrever a epidemiologia e as características clínicas da pancreatite aguda e da pancreatite aguda recorrente em crianças. Métodos: Estudo observacional e retrospectivo com um componente analítico. Os pacientes foram classificados em dois grupos: pancreatite aguda e pancreatite recorrente. A relação com cada parâmetro obtido foi analisada com o teste de qui-quadrado, teste t de Student ou teste U de Mann-Whitney. Resultados: Foram analisados 130 pacientes com pancreatite aguda; pancreatite recorrente foi diagnosticada em 23,8% dos casos. As causas mais frequentes foram anatômicas (29,6%), farmacológicas (19,2%) e biliares (14,6%), embora em 29,2% a etiologia não tenha sido identificada. O jejum durou 3,5 ± 3,8 dias e a nutrição parenteral foi indicada em 26,9% dos casos por 10,8 ± 11,3 dias. Uma associação estatística com causas anatômicas (p = 0,02) e farmacológicas (p = 0,01) foi encontrada no grupo com pancreatite recorrente; não foram observadas outras diferenças entre os grupos pancreatite aguda e pancreatite recorrente. A taxa de mortalidade foi de 3,1% e nenhum caso foi atribuível à pancreatite aguda. Conclusão: A pancreatite aguda está associada à alta frequência de pancreatite aguda recorrente. A gravidade e as complicações não apresentaram diferenças estatísticas nesta investigação. Etiologias anatômicas foram a causa mais relevante nesta coorte. O tempo de jejum e o uso de nutrição parenteral foram relevantes. Testes genéticos são necessários em nossas populações.

Infecciones intraabdominales: Puesta al día y recomendaciones de la sociedad Argentina de infectología / Intra-abdominal infections. Update and recommendations

Las infecciones intraabdominales constituyen un grupo de procesos intra y retroperitoneales, desde cuadros localizados hasta infecciones complicadas, sepsis o shock séptico, con elevada mortalidad. Representan la tercera causa de sepsis y la segunda causa de muerte en unidades de terapia intensiva. El tratamiento antimicrobiano debe iniciarse lo antes posible, especialmente en pacientes en estado crítico, pero también es fundamental el procedimiento de control del foco. Dada la importancia del tema, representantes de la Sociedad Argentina de Infectología junto con especialistas en Terapia Intensiva elaboraron estas recomendaciones sobre su diagnóstico, tratamiento y prevención. A tal fin, revisaron y analizaron la bibliografía publicada sobre el tema en los últimos 10 años, además de la opinión de expertos y datos locales. El documento ofrece herramientas básicas de diagnóstico basadas en criterios clínicos y microbiológicos, orientación sobre esquemas antibióticos empíricos y dirigidos según foco de origen, lugar de adquisición (comunidad o asociadas al cuidado de la salud) y gravedad de la infección, duración del tratamiento, importancia del control del foco y medidas preventivas para reducir el riesgo de infección del sitio quirúrgico. Asimismo, se presenta un algoritmo sencillo de diagnóstico y tratamiento para uso en la actividad asistencial. El trabajo pone en evidencia la preocupación por el tratamiento de las infecciones intraabdominales, estableciendo pautas locales para mejorar su diagnóstico, tratamiento y prevención, con el objeto de disminuir morbimortalidad, días de internación, costos y resistencia antimicrobiana.

Intra-abdominal infections represent a group of intra and retroperitoneal processes, ranging from localized infections to complicated ones, sepsis and septic shock, associated with a significant mortality rate. They are the third most commonly identified cause of sepsis and the second cause of death in the intensive care unit. Although antimicrobial therapy must be started as soon as possible, especially in critically ill patients, the source control procedure is highly relevant. On account of the importance of this subject, members of the Argentine Society of Infectious Diseases (SADI) and intensive care specialists joined to develop recommendations on diagnosis, treatment, and prevention of intra-abdominal infections. The literature published within the last 10 years was reviewed and analyzed, in addition of expert opinions and local data. This statement provides a basic tool for diagnosis based on clinical and microbiological criteria, orientation on empirical antimicrobial therapy schemes according to source, acquisition place (community or healthcare associated infections), infection severity, treatment duration, importance of source control, and preventive measures aimed to reduce surgical site infection risk. Likewise, it provides a simple algorithm for diagnosis and treatment for use in clinical practice. The work reveals the concern about the management of intra-abdominal infections, establishing local guidelines to optimize diagnosis, treatment and prevention, with the aim of reducing morbidity, mortality, length of stay, costs and antimicrobial resistance.

Analysis of influencing factors of severity in acute pancreatitis using big data mining

SUMMARY OBJECTIVES To evaluate the epidemiological characteristics of acute pancreatitis (AP) and explore potential relationships between these factors and severity. METHODOLOGY Data-sets of 5,659 patients with AP from health statistics and the Information Center of Jiangsu province, between 2014 and 2016, were analyzed. A self-organizing map (SOM) neural network was used for data clustering. RESULTS Biliary acute pancreatitis (BAP) (86.7%) was the most frequent etiological factor. A total of 804 (14.2%) patients had severe acute pancreatitis (SAP). The mean age of patients was 53.7 + 17.3 (range 12~94y). Most of the AP patients were married (75.4%); 6% of mild /moderately severe AP (MAP/MASP) patients were unmarried, which was less than SAP patients (P=0.016). AP patients with blood type AB in the general population (8.8%) was significantly lower than that of AP cases (13.9%) (P=0.019) and SAP cases(18.7%) (P=0.007). The number of AP patients in southern Jiangsu was much higher than that in northern Jiangsu province, especially in Nanjing (1229, 21.7%). The proportion of acute alcoholic pancreatitis (AAP) in the north of Jiangsu (Xuzhou 18.4%) was much higher than that in southern Jiangsu (Suzhou 2.6%). The whole sample was divided into five classes by SOM neural network. If BAP patients were male, old, divorced, and blood type AB or B, they were more likely to develop SAP. Middle-age, unmarried or divorced male patients with blood type B/AB who suffered from HAP or AAP were also more likely to develop SAP. CONCLUSIONS The number of unmarried patients with MAP/MASP was smaller than that of SAP. Blood types AB and B were more frequent in AP, especially in SAP. The differences between southern Jiangsu and northern Jiangsu, in number of AP patients and the proportion of AAP, were significant. In class I and class IV, the ratio of SAP was much higher than in other classes and the whole sample.

Páncreas divisum y pancreatitis: su importancia en la práctica cotidiana / Pancreas divisum and pancreatitis: its importance in everyday practice

Pancreas divisum (PD) is the most common congenital anatomical variant of the pancreas. Its etiological implication in recurrent acute pancreatitis (RAP) and chronic pancreatitis (CP) has been recurrently questioned. Normal anatomy and variants: 80-90% of the population has normal anatomy, with excretion of exocrine pancreatic secretion to the duodenum by the major papilla. Three anatomical variants of PD have been described: classic PD with visible ventral duct, but total absence of fusion; PD with absence of ventral duct; and incomplete PD, with a rudimentary connection between the ventral and dorsal ducts. Clinical implication: This anatomical variant is symptomatic in less than 5% of the carriers, being associated to higher prevalence in patients with RAP However, the relationship between PD and RAP is considered probable, only in cases of association with mutation of the CFTR gene. Obstructive CP can develop in the segment drained by the dorsal duct. Diagnosis: Magnetic resonance cholangiopancreatography (MRCP) is the most sensitive diagnostic method. Other non-invasive diagnostic methods are endosonography and computed tomography (CT), the latter with lower performance. Treatment: The current trend in acute pancreatitis (AP) where PD is assumed as an etiological factor, is endoscopic resolution, with papillotomy with or without a pancreatic stent.

El páncreas divisum (PD) es la variante anatómica congénita más frecuente del páncreas. Su implicancia etiológica en pancreatitis aguda recurrente (PAR) y pancreatitis crónica (PC) ha sido frecuentemente cuestionada. Anatomía normal y variantes: 80-90% de la población presenta anatomía normal, con salida de secreción pancreática exocrina al duodeno por la papila mayor. Se han descrito 3 variantes anatómicas: PD clásico con conducto ventral visible, pero ausencia total de fusión; PD con ausencia de conducto ventral; y PD incompleto, con conexión rudimentaria entre los conductos ventral y dorsal. Implicancia clínica: Esta variante anatómica da síntomas en menos de 5% de los portadores, asociándose a mayor prevalencia en pacientes con PAR. Sin embargo, se considera probable la relación entre PD y PAR, solo en casos de asociación con mutación del gen CFTR. Pancreatitis crónica (PC) obstructiva se puede desarrollar en el segmento drenado por el conducto dorsal. Diagnóstico: La colangiopancreatografía por resonancia magnética (CPRM) es el método diagnóstico más sensible. Otros métodos diagnósticos no invasivos son la endosonografía y tomografía computada (TC), este último de menor rendimiento. Tratamiento: La tendencia actual en PA donde se asume PD como factor etiológico, es la resolución endoscópica, con papilotomía con o sin stent.

Pancreatitis autoinmune tipo 1: reporte de caso / Autoimmune pancreatitis type 1: a case report

La pancreatitis autoinmune tipo 1 es una enfermedad de baja prevalencia siendo más frecuente en varones, se encuentra incluida dentro de las enfermedades relacionadas a IgG4. Esta patología puede debutar como un síndrome colestásico y el diagnóstico se realiza según los criterios del consenso internacional para pancreatitis autoinmune (ICDC) que incluye una imagen típica, serología, compromiso de otros órganos, histología y respuesta al tratamiento con corticoides. Presentamos el caso de una mujer de 52 años con antecedente de artritis reumatoide sin tratamiento que acude con tiempo de enfermedad de 2 meses caracterizado por dolor abdominal en hipocondrio derecho de moderada intensidad asociado a ictericia, coluria, hipocolia, xeroftalmia, xerostomía y pérdida de peso de 3 kg. Al examen físico se evidencia ictericia, hipertrofia simétrica de glándulas submandibulares, leve dolor en epigastrio. En los exámenes auxiliares existe patrón colestásico con hiperbilirrubinemia a predominio directo. En los estudios de imágenes se evidencia colédoco dilatado, con aumento difuso del volumen del páncreas con captación tardía de contraste. En el estudio inmunológico se evidenció IgG4 en 610 u/L y ANA 1/640. Se inició tratamiento con corticoides con respuesta clínica y de laboratorio favorable. En conclusión, se debe sospechar de pancreatitis autoinmune ante un cuadro de dolor abdominal y colestasis extrahepática con imágenes sugestivas de páncreas inflamatorio, en el que se deben complementar los criterios ICDC para confirmar el diagnóstico

Autoimmune pancreatitis type 1 (AIP) is the pancreatic manifestation of IgG4-related disease. The most frequent presentation of AIP is with obstructive jaundice. For definite diagnosis of type 1 Autoimmune pancreatitis international consensus diagnosis criteria (ICDC) for AIP are used. ICDC criteria include pancreatic parenchymal imaging, ductal imaging, serology, other organ involvement, histology, and response to steroid. We report a 52-years-old woman with rheumatoid arthritis without treatment presented with two months of abdominal pain in up-right quadrant with moderate intensity. She also presented jaundice, acholia, xerophtalmia, xerostomia, and a weight loss of 5 pounds. On examination jaundice, symmetrically enlarged submandibular glands, and epigastric pain was observed. On laboratory, a cholestasis pattern and conjugated bilirubin predominance was found. CT Abdominal, CMR revealed a dilated common bile duct with a diffuse pancreatic enlargement with delayed enhancement. Immunological studies show a IgG4 610 u/l and ANA 1/640. The patient responds to steroid clinically and in the laboratorial values. In conclusion, autoimmune pancreatitis type 1 should be suspected in case of an obstructive jaundice with a pancreatic inflammatory image, and complete ICDC criteria for a definite diagnosis

Pancreatitis autoinmune: desafío diagnóstico y tratamiento actual / Autoimmune pancreatitis: diagnostic challenges and current treatment

Autoimmune pancreatitis (AIP) is an inflammatory disease of the pancreas. The mechanism of the disease is not completely known. However, AIP shows cellular and humoral immunity elements, the most important being helper and regulatory T lymphocytes as well as B-lymphocytes and plasmocytes, participating in the fibroinflammatory process. Two histologic types have been described with different clinical characteristics. Type 1 AIP is part of a systemic condition associated with an increase of IgG4, while type 2 is a pancreatic disease, frequently associated with inflammatory bowel disease. From the clinical point of view, a third category is described when the classification is not possible at the moment of the diagnosis. The most important differential diagnosis of AIP is pancreatic cancer and it can be difficult, because current diagnostic methods used, including biopsy, have low specificity and sensitivity. AIP patients recover rapidly after steroid therapy, which can be useful even in differential diagnosis. Long-term prognosis is good: more than half of type 1 and almost all cases of type 2 patients have favorable outcome without recurrence and without severe consequences.

La pancreatitis autoinmune (PAI) es una enfermedad inflamatoria del páncreas. El mecanismo fisiopatológico no es completamente conocido. Sin embargo, presenta elementos de inmunidad celular y humoral, siendo de mayor importancia los linfocitos T-helper, T-reguladores, linfocitos B y plasmocitos, que participan en el desarrollo de la enfermedad. Se reconocen dos tipos histológicos con características clínicas también distintas. El tipo 1 forma parte de una enfermedad sistémica relacionada a aumento de IgG4, mientras el tipo 2 es una enfermedad pancreática, aunque con frecuencia asociada a enfermedad inflamatoria intestinal. Desde el punto de vista clínico, existe una tercera categoría, que se presenta cuando en el momento del diagnóstico de PAI la tipificación clínicamente no es posible. El diagnóstico diferencial más importante de la PAI es el cáncer de páncreas y puede ser clínicamente difícil. Los métodos actuales de diagnóstico incluyen la biopsia pero tienen un rendimiento bajo. La PAI responde rápidamente al tratamiento con esteroides, hecho que puede ser útil aún en el diagnóstico diferencial. Su pronóstico a largo plazo es bueno: más de la mitad de los casos tipo 1 y casi todos los casos tipo 2 evolucionan sin recaída y sin consecuencias graves a largo plazo.

Acute pancreatitis complicating dengue hemorrhagic fever

Abstract Dengue infection can have spectrum of manifestations, often with an unpredictable clinical progression and outcome. There have been increasing reports of atypical manifestations. Abdominal pain or tenderness and persistent vomiting (warning signs) are present in the majority of cases with severe dengue prior to clinical deterioration. We report a 10-year-old child who presented with fever, persistent vomiting, and abdominal pain. A diagnosis of acute pancreatitis was made. This is a very infrequently reported complication of dengue hemorrhagic fever.

The panc 3 score predicting severity of acute pancreatitis / Escore panc 3 na predição da severidade de pancreatite aguda

Background: About 20% of cases of acute pancreatitis progress to a severe form, leading to high mortality rates. Several studies suggested methods to identify patients that will progress more severely. However, most studies present problems when used on daily practice. Objective: To assess the efficacy of the PANC 3 score to predict acute pancreatitis severity and its relation to clinical outcome. Methods: Acute pancreatitis patients were assessed as to sex, age, body mass index (BMI), etiology of pancreatitis, intensive care need, length of stay, length of stay in intensive care unit and mortality. The PANC 3 score was determined within the first 24 hours after diagnosis and compared to acute pancreatitis grade of the Revised Atlanta classification. Results: Out of 64 patients diagnosed with acute pancreatitis, 58 met the inclusion criteria. The PANC 3 score was positive in five cases (8.6%), pancreatitis progressed to a severe form in 10 cases (17.2%) and five patients (8.6%) died. Patients with a positive score and severe pancreatitis required intensive care more often, and stayed for a longer period in intensive care units. The PANC 3 score showed sensitivity of 50%, specificity of 100%, accuracy of 91.4%, positive predictive value of 100% and negative predictive value of 90.6% in prediction of severe acute pancreatitis. Conclusion: The PANC 3 score is useful to assess acute pancreatitis because it is easy and quick to use, has high specificity, high accuracy and high predictive value in prediction of severe acute pancreatitis.

Racional: Cerca de 20% dos casos de pancreatite aguda evoluem de forma severa, acompanhados de alta mortalidade. Diversos estudos têm sugerido métodos para identificar pacientes que evoluirão com maior gravidade. Entretanto, a maioria apresenta problemas em sua utilização na prática diária. Objetivo: Avaliar a eficácia do escore PANC 3 na predição da severidade da pancreatite aguda e sua relação com o desfecho clínico. Métodos: Pacientes com pancreatite aguda foram avaliados quanto ao sexo, idade, índice de massa corporal (IMC), etiologia da pancreatite, necessidade de cuidados intensivos, tempo de internação hospitalar, período necessário de cuidados intensivos e mortalidade. O escore PANC 3 foi determinado nas primeiras 24 h do diagnóstico e comparado ao grau de pancreatite aguda da classificação de Atlanta Revisada. Resultados: Dos sessenta e quatro pacientes, cinquenta e oito preencheram os critérios necessários para inclusão no estudo. O escore PANC 3 foi positivo em cinco casos (8,6%), a pancreatite evoluiu de forma severa em 10 (17,2%) e 5 (8,6%) faleceram. Pacientes com escore positivo e pancreatite severa, necessitaram mais frequentemente de cuidados intensivos e, quando necessitaram, permaneceram por período maior nas unidades de cuidados intensivos. O escore PANC 3 demonstrou sensibilidade de 50%, especificidade de 100%, acurácia de 91,4%, valor preditivo positivo de 100% e valor preditivo negativo de 90,6% na predição de pancreatite aguda severa. Conclusão: O escore PANC 3 é útil na abordagem da pancreatite aguda, por ser de fácil e rápida aplicação, apresentar alta especificidade, alta acurácia e alto valor preditivo na predição da pancreatite aguda severa.

Ascaris lumbricoides: una causa de pancreatitis aguda / Acute pancreatitis due to Ascaris lumbricoides

La ascariasis es una infección parasitaria causada por un helminto de distribución global, con más de 1.4 billones de personas infectadas en el mundo. La mayoría de estas infecciones ocurren en países en vías de desarrollo de América latina y Asia. El helminto usualmente se aloja en el intestino delgado en forma silente pero puede causar obstrucción intestinal o peritonitis perforativa, siendo más común en la niñez. A su vez, puede migrar a través de la ampolla de Vater y producir pancreatitis, colecistitis, colangitis y, en forma menos frecuente, absceso hepático. El objetivo de nuestra comunicación es notificar un caso de pancreatitis aguda secundaria a Ascaris lumbricoides, siendo ésta una complicación infrecuente pero grave de una enfermedad endémica como la ascariasis.

Ascariasis is a helminthic infection of global distribution with more than 1.4 billion persons infected throughout the world. The majority of infections occur in the developing countries of Latin America and Asia. This helminth usually lives harmlessly in small intestine but can also cause intestinal obstruction or perforation peritonitis that is common in childhood. Ascaris can also migrate through ampulla of Vater to produce pancreatitis, cholecystitis, cholangitis and, rarely, hepatic abscess. The main goal of this article is to present a case of an acute pancreatitis due to Ascaris lumbricoides, an uncommon but severe complication of an endemic disease such as ascariasis.

Pancreatite aguda / Acute pancreatitis

Os autores fazem uma revisão atualizada da abordagem clínica da pancreatite aguda, com ênfase nas medidas a serem aplicadas na emergência.

The authors disclose an updated review of clinical management of acute pancreatitis, focusing on measures to be applied in emergency.

Microscopic polyangiitis with crescentic glomerulonephritis initially presenting as acute pancreatitis

No abstract available.