Non-insulinoma pancreatogenous hypoglycemia syndrome (NIPHS)/Nesidioblastosis as the underlying cause of recurrent hypoglycemia in a diabetic adult.

Non-insulinoma pancreatogenous hypoglycemia syndrome (NIPHS), without previous bariatric surgery, is a rare form of hypoglycemia in adult patients and is associated with nesidioblastosis. Adult-onset nesidioblastosis in diabetic patients is rare and histologically identical to "non-insulinoma pancreatogenous hypoglycemia syndrome (NIPHS)". Nesidioblastosis is rare in adults and clinically and biochemically mimics Insulinoma. In the literature, there have only been four cases of adult nesidioblastosis that followed diabetes mellitus. We report a case of nesidioblastosis in a 36-year-old diabetic female presenting with dizziness, sweating, and palpitations for three years. Selective non-invasive techniques failed to detect a tumor. Based on the pursuit of an insulinoma, a distal pancreatectomy specimen was received at our laboratory, and a diagnosis of nesidioblastosis was made. She is currently on follow-up with a favorable outcome. The definitive diagnosis of nesidioblastosis is made on a histological basis. The preferred form of treatment is pancreatic surgical resection. Nesidioblastosis should be taken into consideration in cases where diabetes transforms into hyperinsulinemic hypoglycemia.

The influence of institutional pancreaticoduodenectomy volume on short-term outcomes in the Brazilian public health system: 2008-2021 / A influência do volume institucional de duodenopancreatectomia nos resultados de curto prazo no sistema público de saúde brasileiro: 2008-2021

ABSTRACT Introduction: pancreaticoduodenectomy is a complex surgical procedure that can result in high rates of complications and morbimortality. Due to its complexity, the establishment of referral centers has increased in recent decades. This study aims to evaluate the influence of the institutional volume of pancreaticoduodenectomy for periampullary cancer on short-term outcomes in the Brazilian public health system. Methods: this study used a population-based approach and investigated the number of pancreaticoduodenectomies performed by institutions within Brazil's public health system between 2008 and 2021. High-volume institutions were defined as those that performed more than two standard deviations above the mean number of procedures per year. Specifically, if a center performed eight or more pancreaticoduodenectomies annually, it was considered a high-volume institution. Results: in Brazil, 283 public hospitals performed pancreaticoduodenectomy for cancer between 2008 and 2021. Only ten hospitals performed at least eight pancreaticoduodenectomies per year, accounting for approximately 3.5% of the institutions. High-volume institutions had a significantly lower in-hospital mortality rate than low-volume institutions (8 vs. 17%). No significant differences between groups were observed for length of stay, hospitalizations using the ICU, and ICU length of stay. The linear regression model showed that the number of hospital admissions for pancreaticoduodenectomy and age were significantly associated with hospital mortality. Conclusion: institutional pancreaticoduodenectomy volume implies a lowering of in-hospital mortality. The findings of this nationwide study can affect how the public health system manages pancreaticoduodenectomy care.

RESUMO Introdução: a duodenopancreatectomia é um procedimento complexo com alta risco de complicações e morbimortalidade. Devido à sua complexidade, a constituição de centros de referência tem aumentado nas últimas décadas. Este estudo tem como objetivo avaliar a influência do volume institucional de duodenopancreatectomia para câncer periampular em resultados de curto prazo no sistema público de saúde brasileiro. Métodos: este estudo utilizou uma abordagem de base populacional e investigou o número de duodenopancreatectomia realizadas por instituições do sistema público de saúde do Brasil entre 2008 e 2021. Definimos instituições de alto volume como aquelas que realizaram mais de dois desvios padrão acima do número médio de procedimentos por ano. Especificamente, um centro seria considerado de alto volume se realizasse oito ou mais duodenopancreatectomias anualmente. Resultados: no Brasil, 283 hospitais públicos realizaram duodenopancreatectomia por câncer entre 2008-2021. Apenas dez hospitais realizaram pelo menos oito duodenopancreatectomias anualmente, correspondendo a aproximadamente 3,5% das instituições. As instituições de alto volume tiveram uma taxa de mortalidade intra-hospitalar significativamente menor do que as instituições de baixo volume (8 vs. 17%). Não foram observadas diferenças significativas entre os grupos quanto ao tempo de internação, internações na UTI e tempo de internação na UTI. O modelo de regressão linear mostrou que o número de internações para duodenopancreatectomia e a idade estiveram significativamente associados à mortalidade hospitalar. Conclusão: o volume institucional da duodenopancreatectomia implica em diminuição da mortalidade intra-hospitalar. Os achados deste estudo nacional podem afetar a forma como o sistema público de saúde gerencia os cuidados com a duodenopancreatectomia..

Non-insulinoma pancreatogenous hypoglycemia syndrome (NIPHS)/Nesidioblastosis as the underlying cause of recurrent hypoglycemia in a diabetic adult

ABSTRACT Non-insulinoma pancreatogenous hypoglycemia syndrome (NIPHS), without previous bariatric surgery, is a rare form of hypoglycemia in adult patients and is associated with nesidioblastosis. Adult-onset nesidioblastosis in diabetic patients is rare and histologically identical to "non-insulinoma pancreatogenous hypoglycemia syndrome (NIPHS)". Nesidioblastosis is rare in adults and clinically and biochemically mimics Insulinoma. In the literature, there have only been four cases of adult nesidioblastosis that followed diabetes mellitus. We report a case of nesidioblastosis in a 36-year-old diabetic female presenting with dizziness, sweating, and palpitations for three years. Selective non-invasive techniques failed to detect a tumor. Based on the pursuit of an insulinoma, a distal pancreatectomy specimen was received at our laboratory, and a diagnosis of nesidioblastosis was made. She is currently on follow-up with a favorable outcome. The definitive diagnosis of nesidioblastosis is made on a histological basis. The preferred form of treatment is pancreatic surgical resection. Nesidioblastosis should be taken into consideration in cases where diabetes transforms into hyperinsulinemic hypoglycemia.

Pancreatoduodenectomy due to lipomatous pseudohypertrophy of the pancreas / Pancreatoduodenectomia por pseudohipertrofia lipomatosa do pâncreas

ABSTRACT BACKGROUND: Lipomatous pseudohypertrophy of the pancreas, pancreatic lipomatosis, pancreatic steatosis, non-alcoholic fatty pancreatic disease, or fatty pancreas is an extremely rare disease, characterized by the organ enlargement and a localized or diffuse replacement of pancreatic acinar cells by mature adipose tissue, preserving the pancreatic ductal system and islets of Langerhans. AIMS: To report a rare case of lipomatous pseudohypertrophy of the pancreas in a symptomatic patient and the surgical treatment employed. METHODS: A 24-year-old male patient with weight loss (10 kilograms in 8 months), hyperglycemia, severe and recurrent acute abdominal pain, epigastric discomfort associated with nausea, vomiting, and jaundice for 40 days. Magnetic resonance imaging was performed, revealing an irregular lipomatous pseudohypertrophy of the pancreas, measuring 6.0 × 5.6 cm in the head, uncinate process, and part of the body of the pancreas. The pancreatic duct dilation was diffuse and irregular, associated with atrophy of the remnant parenchyma, particularly in the tail of the pancreas. The patient underwent pancreatoduodenectomy without total mesopancreas excision followed by pancreatojejunostomy. RESULTS: The postoperative course was uneventful, the length of stay in the ICU was two days, and the patient was discharged on the seventh postoperative day. CONCLUSIONS: The disease treatment depends on the signs and symptoms at presentation and a pancreatoduodenectomy is indicated in patients with severe and recurrent abdominal pain.

RESUMO RACIONAL: Pseudo-hipertrofia lipomatosa do pâncreas, lipomatose pancreática, esteatose pancreática, doença pancreática gordurosa não alcoólica ou pâncreas gorduroso é uma doença extremamente rara, caracterizada por aumento do órgão e substituição localizada ou difusa de células acinares pancreáticas por tecido adiposo, preservando o sistema ductal pancreático e as ilhotas de Langerhans. OBJETIVOS: Relatar um caso raro de pseudo-hipertrofia lipomatosa do pâncreas, em paciente sintomático e o tratamento cirúrgico empregado. MÉTODOS: Paciente do sexo masculino, 24 anos, com queixas de emagrecimento (10 quilos nos últimos 8 meses), hiperglicemia, dor abdominal aguda intensa e recorrente, desconforto epigástrico associado a náuseas, vômitos e icterícia há 40 dias. A ressonância magnética (RM) revelou pseudo-hipertrofia lipomatosa irregular do pâncreas, medindo 6,0 × 5,6 cm na cabeça, processo uncinado e parte do corpo do pâncreas. A dilatação do ducto pancreático era difusa e irregular, associada à atrofia do parênquima remanescente, principalmente na cauda do pâncreas. O paciente foi submetido à duodenopancreatectomia sem excisão total do mesopâncreas seguida de pancreatojejunostomia. RESULTADOS: O pós-operatório transcorreu sem intercorrências, o tempo de internação na UTI foi de 2 dias, com alta hospitalar no sétimo dia. CONCLUSÕES: O tratamento da doença depende dos sinais e sintomas na apresentação e a duodenopancreatectomia é indicada em pacientes com dor abdominal intensa e recorrente.

Esteatose pancreática: um novo diagnóstico e desafio terapêutico na Gastroenterologia / Pancreatic steatosis: a new diagnosis and therapeutic challenge in gastroenterology

ABSTRACT Fat infiltration in the pancreas is called pancreatic steatosis and it has several synonyms such as pancreatic lipomatosis, non-alcoholic fatty pancreatic disease, lipomatous pseudohypertrophy, fatty replacement, fatty pancreas and fatty infiltration. Pancreatic steatosis describes a disease ranging from infiltration of fat in the pancreas to pancreatic inflammation, and development of pancreatic fibrosis. There are multiple aetiologies of this condition, such as metabolic syndrome, alcohol intake, viral infections, toxins, congenital syndromes, etc. Pancreatic steatosis is usually diagnosed by trans-abdominal ultrasound, computed tomography scan and magnetic resonance imaging. Fatty infiltration in pancreas may lead to pancreatitis, diabetes mellitus and may be a predisposing cause of pancreatic cancer. Now a day, pancreatic steatosis is a common incidental finding during abdominal ultrasonography for other reasons and is a new challenge in Gastroenterology. But there is no guideline for pancreatic steatosis till now. In this review article, we are trying to give an overall idea (aetiologies, diagnosis, management, clinical significances) on pancreatic steatosis.

RESUMO A infiltração de gordura no pâncreas é chamada de esteatose pancreática ou lipomatose pancreática e tem vários sinônimos tais como: doença pancreática gordurosa não alcoólica, pseudo hipertrofia lipomatosa, reposição gordurosa, pâncreas gorduroso e infiltração gordurosa. A esteatose pancreática descreve uma doença que vai desde a infiltração de gordura no pâncreas até a inflamação pancreática com o desenvolvimento de fibrose pancreática. Existem múltiplas causas dessa condição, como síndrome metabólica, ingestão de álcool, infecções virais, toxinas, síndromes congênitas, etc. A esteatose pancreática é geralmente diagnosticada por ultrassom trans-abdominal, tomografia computadorizada ou ressonância magnética. A infiltração gordurosa no pâncreas pode levar à pancreatite e pode ser uma causa predisponente ao câncer de pâncreas. Hoje em dia, a fibrose pancreática é um achado incidental comum durante a ultrassonografia abdominal realizada por outras razões e é um novo desafio na Gastroenterologia. Mas não há diretriz para esteatose pancreática até agora. Neste artigo de revisão, objetivamos dar uma ideia geral sobre esteatose pancreática.

Evolution of incidence, mortality and cost of nontraumatic abdominal emergencies treated in Brasil in a period of nine years

SUMMARY OBJECTIVE: To evaluate the incidence, mortality and cost of non-traumatic abdominal emergencies treated in Brazilian emergency departments. METHODS: This paper used DataSus information from 2008 to 2016 (http://www.tabnet.datasus.gov.br). The number of hospitalizations, costs - AIH length of stay and mortality rates were described in acute appendicitis, acute cholecystitis, acute pancreatitis, acute diverticulitis, gastric and duodenal ulcer, and inflammatory intestinal disease. RESULTS: The disease that had the highest growth in hospitalization was diverticular bowel disease with an increase of 68.2%. For the period of nine years, there were no significant changes in the average length of hospital stay, with the highest increase in gastric and duodenal ulcer with a growth of 15.9%. The mortality rate of gastric and duodenal ulcer disease increased by 95.63%, which is significantly high when compared to the other diseases. All had their costs increased but the one that proportionally had the highest increase in the last nine years was the duodenal and gastric ulcer, with an increase of 85.4%. CONCLUSION: Non-traumatic abdominal emergencies are extremely prevalent. Hence, the importance of having updated and comparative data on the mortality rate, number of hospitalization and cost generated by these diseases to provide better healthcare services in public hospitals.

RESUMO OBJETIVO: Avaliar a evolução da Incidência, mortalidade e custo das urgências abdominais não traumáticas atendidas nos serviços de emergência do Brasil durante o período de nove anos. MÉTODOS: Este trabalho utilizou informações do DataSus de 2008 a 2016, (http://www.tabnet.datasus.gov.br). Foram analisados número de internações, valor médio das internações (AIH), valor total das internações, dias de permanência hospitalar e taxa de mortalidade das seguintes doenças: apendicite aguda, colecistite aguda, pancreatite aguda, diverticulite aguda, úlcera gástrica e duodenal, e doença inflamatória intestinal. RESULTADOS: A doença que teve o maior crescimento do número de internações foi a doença diverticular do intestino, com o valor de 68,2%. Ao longo dos nove anos não houve grandes variações da média de permanência hospitalar, sendo que o maior aumento foi o da úlcera gástrica e duodenal, com crescimento de 15,9%. A taxa de mortalidade da doença por úlcera gástrica e duodenal teve um aumento de 95,63%, consideravelmente significante quando comparada com as outras doenças. Todas tiveram seus valores de AIH aumentados, porém, a que proporcionalmente teve o maior aumento nos últimos nove anos foi a úlcera gástrica e duodenal, com um acréscimo de 85,4%. CONCLUSÃO: As urgências abdominais de origem não traumática são de extrema prevalência, por isso a importância em ter dados atualizados e comparativos sobre a taxa de mortalidade, o número de internações e os custos gerados por essas doenças, para melhor planejamento dos serviços públicos de saúde.

Fatal pancreatic pseudocyst co-infected by Raoultella planticola: an emerging pathogen.

Raoultella planticola is an aerobic Gram-negative bacterium belonging to the Enterobacteriaceae family. Initially identified in the 1980s, its pathogenic potential was further recognized when the first case of bacteremia was reported. Since then, only a few infections caused by this pathogen have been described. Although considered an opportunistic agent, fatal outcomes are associated with the infection by this pathogen, since it is more prevalent among the patients with immunodeficiency. The authors report the case of a middle-aged man diagnosed with end-stage renal disease and alcoholic pancreatitis, who was admitted to the emergency department with septic shock. Physical examination disclosed peritoneal irritation and a laparotomy was undertaken. Purulent peritonitis was found as well as a retroperitoneal abscess, which was drained. The postoperative period was troublesome, and the patient died. The autopsy showed a ruptured, infected pancreatic cyst and purulent peritonitis, among other findings. The culture of the peritoneal fluid and two blood sample sets were positive for R. planticola. The authors call attention to the importance of this emerging pathogen associated with severe gastrointestinal infections.

Diabetes mellitus secundario a episodio único de pancreatitis aguda: caso clínico y revisión de la literatura / Diabetes mellitus after a single episode of acute pancreatitis: report of one case

Diabetes mellitus secondary to pancreatic diseases is classified as type 3C and it can appear after a single episode of acute pancreatitis. We report a 25 years old female with a family history of diabetes. She was admitted to the hospital due to an acute pancreatitis of biliary origin. She received parenteral nutrition and required up to 82 units of insulin per day to maintain blood glucose levels. A CT scan showed a pseudocyst. A percutaneous drainage, followed by necrotic tissue debridement were performed. She was discharged two months after admission requiring insulin to maintain metabolic control.

Genetic risk for alcoholic chronic pancreatitis.

In recent years many studies have examined the genetic predisposition to pancreatic diseases. Pancreatic disease of an alcoholic etiology was determined to be a multi-factorial disease, where environmental factors interact with the genetic profile of the individual. In this review we discuss the main results from studies examining the frequency of genetic mutations in alcoholic chronic pancreatitis.