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1.
Am J Gastroenterol ; 118(9): 1664-1670, 2023 09 01.
Artículo en Inglés | MEDLINE | ID: mdl-37141538

RESUMEN

INTRODUCTION: Guidelines endorse pancreatic cancer screening in genetically susceptible individuals. We conducted a prospective, multicenter study to determine yield, harms, and outcomes of pancreatic cancer screening. METHODS: All high-risk individuals undergoing pancreatic cancer screening at 5 centers from 2020 to 2022 were prospectively enrolled. Pancreas findings were designated as low-risk (fatty or chronic pancreatitis-like changes), intermediate-risk (neuroendocrine tumor [NET] <2 cm or branch-duct intraductal papillary mucinous neoplasm [IPMN]), or high-risk lesions (high-grade pancreatic intraepithelial neoplasia/dysplasia, main-duct IPMN, NET >2 cm, or pancreatic cancer). Harms from screening included adverse events during screening or undergoing low-yield pancreatic surgery. Annual screening was performed using endoscopic ultrasound and or magnetic resonance cholangiopancreatography. Annual screening for new-onset diabetes using fasting blood sugar was also performed ( ClinicalTrials.gov : NCT05006131). RESULTS: During the study period, 252 patients underwent pancreatic cancer screening. Mean age was 59.9 years, 69% were female, and 79.4% were White. Common indications were BRCA 1/2 (36.9%), familial pancreatic cancer syndrome kindred (31.7%), ataxia telangiectasia mutated (3.5%), Lynch syndrome (6.7%), Peutz-Jeghers (4.3%), and familial atypical multiple mole melanoma (3.5%). Low-risk lesions were noted in 23.4% and intermediate-risk lesions in 31.7%, almost all of which were branch-duct IPMN without worrisome features. High-risk lesions were noted in 2 patients (0.8%), who were diagnosed with pancreas cancer at stages T2N1M0 and T2N1M1. Prediabetes was noted in 18.2% and new-onset diabetes in 1.7%. Abnormal fasting blood sugar was not associated with pancreatic lesions. There were no adverse events from screening tests, and no patient underwent low-yield pancreatic surgery. DISCUSSION: Pancreatic cancer screening detected high-risk lesions with lower frequency than previously reported. No harms from screening were noted.


Asunto(s)
Carcinoma Ductal Pancreático , Neoplasias Intraductales Pancreáticas , Neoplasias Pancreáticas , Humanos , Femenino , Persona de Mediana Edad , Masculino , Neoplasias Intraductales Pancreáticas/patología , Estudios Prospectivos , Detección Precoz del Cáncer , Páncreas/patología , Neoplasias Pancreáticas/diagnóstico por imagen , Neoplasias Pancreáticas/patología , Carcinoma Ductal Pancreático/patología
2.
Sci Rep ; 12(1): 17619, 2022 10 21.
Artículo en Inglés | MEDLINE | ID: mdl-36271289

RESUMEN

Guidelines have shifted to now recommend endoscopic eradication therapy for Barrett's esophagus (BE) with low and high-grade dysplasia. Previously, esophagectomy was the standard therapy for high-grade dysplasia. However, it is unclear to what degree ablation therapy has affected utilization of esophagectomy. In this retrospective observational cohort study of BE patients without cancer from the Premier Healthcare Database, the prevalence of utilization of endoscopic ablation therapy and of esophagectomy in BE were calculated and temporal trends were evaluated. A total of 938, 333 BE cases were included in the study. There was a significantly increasing trend of ablation over the period 2006 to 2010 (Annual Percentage Change (APC); 95% CI 0.56% [0.51%, 0.61%]), a significantly decreasing trend for the period 2011 to 2015 (APC; 95% CI - 0.15% [- 0.20%, - 0.11%]), and a shallow increasing trend for the period 2016 to 2019 (APC; 95% CI 0.09% [0.06%, 0.11%]). For esophagectomy, there was a significantly decreasing trend for the period 2006 to 2009 (APC; 95% CI - 0.03% [- 0.04%, - 0.02%]; P < 0.001) that corresponded to the uptrend in utilization of endoscopic ablation. There was a stable trend of esophagectomy over the period 2010 to 2019 (APC; 95% CI - 0.0006% [- 0.0002%, 0.0005%]; P = 0.1947). Adoption and increased utilization of endoscopic ablation therapy for BE has coincided with a decrease in esophagectomy, and is the predominate method of therapy for BE with dysplasia.


Asunto(s)
Esófago de Barrett , Neoplasias Esofágicas , Humanos , Esófago de Barrett/cirugía , Esofagectomía/métodos , Estudios Retrospectivos , Neoplasias Esofágicas/cirugía , Endoscopía
3.
Clin Transl Gastroenterol ; 12(2): e00311, 2021 02 22.
Artículo en Inglés | MEDLINE | ID: mdl-33617190

RESUMEN

INTRODUCTION: Studies have shown that dysplasia in Barrett's esophagus (BE) has a predilection for the right hemisphere. There is limited information on the longitudinal distribution. The aim was to determine both the longitudinal and circumferential distributions of dysplasia and early neoplasia from 3 prospective studies. METHODS: This is a pooled analysis from 3 prospective studies of patients with treatment-naive BE. Both circumferential and longitudinal locations (for BE segments greater than 1 cm) of dysplastic and early neoplastic lesions were recorded. RESULTS: A total of 177 dysplastic and early neoplastic lesions from 91 patients were included in the pooled analysis; of which 59.3% (n = 105) were seen on high-definition white light endoscopy, 29.4% (n = 52) on advanced imaging, and 11.2% (n = 20) with random biopsies. The average Prague score was C3M5. Of 157 lesions within BE segments greater than 1 cm, 49 (34.8%) lesions were in the proximal half, whereas 92 lesions (65.2%) were in the distal half (P < 0.001). The right hemisphere of the esophagus contained 55% (86/157) of the total lesions compared with 45% (71/157) for the left hemisphere (P = 0.02). This was because of the presence of high-grade dysplasia being concentrated in the right hemisphere compared with the left hemisphere (60% vs 40%, P = 0.002). DISCUSSION: In this pooled analysis of prospective studies, both low-grade dysplasia and high-grade dysplasia are more frequently found in the distal half of the Barrett's segment. This study confirms that the right hemisphere is a hot spot for high-grade dysplasia. Careful attention to these locations is important during surveillance endoscopy.


Asunto(s)
Esófago de Barrett/patología , Neoplasias Esofágicas/patología , Esófago de Barrett/diagnóstico por imagen , Biopsia , Interpretación Estadística de Datos , Neoplasias Esofágicas/diagnóstico por imagen , Esofagoscopía , Femenino , Humanos , Masculino , Microscopía Confocal
5.
Pancreatology ; 21(1): 31-33, 2021 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-33309015

RESUMEN

BACKGROUND: Limited data exists on the association or prevalence of pancreatitis in children with COVID-19. METHODS: This is a retrospective study of pediatric patients admitted to a large health system in New York (Northwell Health System) from March 1, 2020-June 1, 2020 during the COVID-19 pandemic. RESULTS: 8159 pediatric patients were admitted to our healthcare system during the study period, of which 112 were diagnosed with COVID-19 (1.37%). Thirteen were diagnosed with pancreatitis for a point prevalence of 0.16% (13/8159) for all patients admitted. Of the thirteen patients admitted with pancreatitis, two patients were COVID-19 positive for a point prevalence of 1.8% (2/112) among COVID-19 patients compared to 0.14% (11/8047) in the non-COVID-19 population. CONCLUSIONS: This study shows that pancreatitis can occur in pediatric patients with COVID-19 and may be more common in the COVID-19 population.


Asunto(s)
COVID-19/complicaciones , Pancreatitis/etiología , Adolescente , COVID-19/epidemiología , Niño , Preescolar , Femenino , Hospitalización , Humanos , Lactante , Masculino , New York/epidemiología , Pancreatitis/epidemiología , Prevalencia , Estudios Retrospectivos , Resultado del Tratamiento
6.
Endosc Ultrasound ; 9(5): 337-344, 2020.
Artículo en Inglés | MEDLINE | ID: mdl-33106466

RESUMEN

BACKGROUND AND AIMS: Guidelines recommend either EUS or magnetic resonance cholangiopancreatography (MRCP) for intermediate risk of choledocholithiasis. There is a lack of evidence that supports proceeding with EUS if the MRCP is negative and if clinical suspicion still exists. METHODS: This is a retrospective study of all patients who underwent EUS to assess for choledocholithiasis at a tertiary care referral center from July 2013 to October 2019. RESULTS: A total of 593 patients underwent EUS for evaluation for choledocholithiasis. Of the 593 patients, 35.2% (209/593) had an MRCP. 73.2% (153/209) had a negative MRCP while 26.8% (56/209) had a positive MRCP. Of the group of patients who underwent EUS with a negative MRCP, 15% (23/153) were positive for choledocholithiasis on EUS. Of these, 91% (21/23) were also positive for sludge or stones on endoscopic retrograde cholangiopancreatography and thus 14% (21/153) of the EUS were "true positives." There were no clinical or laboratory factors predictive of choledocholithiasis on univariate analysis in the EUS plus negative MRCP group. When further analyzing the MRCP negative group into MRCP-/EUS+ and MRCP-/EUS-subgroups, a total bilirubin >3 mg/dL predicted a bile duct stone (55% vs. 32%, P = 0.05). CONCLUSION: The diagnostic yield of EUS for suspected choledocholithiasis in the setting of a negative MRCP is 14% in our cohort. EUS should be considered in patients with intermediate risk of choledocholithiasis with a negative MRCP if the clinical suspicion is still present, and especially if the total bilirubin is above 3 mg/dL.

8.
World J Gastroenterol ; 26(17): 2030-2039, 2020 May 07.
Artículo en Inglés | MEDLINE | ID: mdl-32536772

RESUMEN

Radiofrequency ablation (RFA) is very effective for eradication of flat Barrett's mucosa in dysplastic Barrett's esophagus after endoscopic resection of raised lesions. However, in a minority of the time, RFA may be ineffective at eradication of the Barrett's mucosa. Achieving complete eradication of intestinal metaplasia can be challenging in these patients. This review article focuses on the management of patients with dysplastic Barrett's esophagus refractory to RFA therapy. Management strategies discussed in this review include optimizing the RFA procedure, optimizing acid suppression (with medical, endoscopic, and surgical management), cryotherapy, hybrid argon plasma coagulation, and EndoRotor resection.


Asunto(s)
Esófago de Barrett/terapia , Neoplasias Esofágicas/prevención & control , Esofagoscopía/métodos , Esófago/patología , Reflujo Gastroesofágico/terapia , Antiácidos/uso terapéutico , Coagulación con Plasma de Argón/métodos , Esófago de Barrett/etiología , Esófago de Barrett/patología , Terapia Combinada/métodos , Criocirugía/métodos , Esofagoscopía/instrumentación , Esófago/diagnóstico por imagen , Esófago/cirugía , Reflujo Gastroesofágico/complicaciones , Reflujo Gastroesofágico/patología , Humanos , Ablación por Radiofrecuencia/métodos , Resultado del Tratamiento
9.
Endoscopy ; 52(10): 864-870, 2020 10.
Artículo en Inglés | MEDLINE | ID: mdl-32408362

RESUMEN

BACKGROUND : A significant proportion of duodenoscope-transmitted infections have resulted from errors related to reprocessing. Remote video auditing (RVA) is a tool that can monitor reprocessing compliance but it has not been previously evaluated in a tertiary care setting. The aims of this study were to evaluate: 1) RVA feasibility in a tertiary care setting (defined as the ability to audit every step of duodenoscope reprocessing without delaying the next procedure due to unavailability of duodenoscopes); and 2) the use of RVA as a compliance monitoring tool. METHODS : This was a prospective study at a tertiary care center. A video camera with offsite monitoring was installed in March 2018. Auditors delivered a compliance score after each procedure. The duodenoscope was not used until it passed the audit. Feasibility and compliance data were collected from April 2018 to August 2019 after a 1-month run-in phase. Both per-step compliance and overall 100 % compliance rates were measured. RESULTS : Of 743 duodenoscope reprocessing procedures, 32 666 individual steps were audited and 99.9 % of the steps were fully viewable. The mean time per audit was 38.3 minutes, the mean duodenoscope turnover time was 76.1 minutes, and there were no delays to the next procedure due to unavailability of duodenoscopes. The per-step compliance rate was 99.5 % but the overall 100 % compliance rate was 90.3 %. CONCLUSIONS : The use of RVA in duodenoscope reprocessing was feasible and promoted sustained high-level compliance in a tertiary care center.


Asunto(s)
Duodenoscopios , Contaminación de Equipos , Colangiopancreatografia Retrógrada Endoscópica , Contaminación de Equipos/prevención & control , Humanos , Estudios Prospectivos , Centros de Atención Terciaria
10.
Gastrointest Endosc Clin N Am ; 30(2): 291-307, 2020 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-32146947

RESUMEN

Minimally invasive endoscopic antireflux therapies are critical for bridging the gap between medical and surgical treatments for gastroesophageal reflux disease (GERD). Although multiple endoscopic devices have been developed, perhaps some of the most exciting options that are currently evolving are the full-thickness suturing techniques using widely available and low-cost platforms. Full-thickness endoscopic suturing can allow for a highly durable recreation of the anatomic and functional components of a lower esophageal sphincter, which are deficient in patients with GERD. Proper patient selection, endoscopic hiatal hernia evaluation, and standardized suturing methods are necessary to ensure success of endoscopic suturing for antireflux therapy.


Asunto(s)
Endoscopía/métodos , Esfínter Esofágico Inferior/cirugía , Reflujo Gastroesofágico/cirugía , Técnicas de Sutura/tendencias , Terapias en Investigación/tendencias , Humanos , Selección de Paciente , Resultado del Tratamiento
12.
Clin Transl Gastroenterol ; 10(12): e00107, 2019 12.
Artículo en Inglés | MEDLINE | ID: mdl-31833863

RESUMEN

INTRODUCTION: Little is known about the additive yield of wide-area transepithelial sampling with computer-assisted three-dimensional analysis (WATS-3D) after a thorough examination with advanced imaging. The aim was to evaluate the adjunctive yield of WATS-3D after advanced imaging. METHODS: This is an observational cohort study from January 2017 to December 2018 for consecutive patients who underwent an examination that consists of high-definition white light endoscopy (HDWLE), narrow-band imaging (NBI), volumetric laser endomicroscopy (VLE), and Seattle protocol (SP) biopsies (collectively termed HDWLE-NBI-VLE-SP examination). Raised lesions were removed by endoscopic resection. Areas suspicious for dysplasia on NBI and VLE were biopsied. This was followed by random biopsies and WATS-3D brush biopsies. RESULTS: One hundred thirty-eight cases were included in this study. Thirty-five cases (25% of the total) were identified as some degree of dysplasia on the HDWLE-NBI-VLE-SP examination. Adjunctive use of WATS-3D yielded an additional 12 new cases of dysplasia (9 with crypt dysplasia and 3 with low-grade dysplasia [LGD]), for added yield of 34.3% (=12/35, 95% confidence interval 14.6%-62.2%). When restricting the analysis to LGD and higher, 21 dysplastic cases (15% of the total cases) were identified by HDWLE-NBI-VLE-SP, while WATS-3D found 4 additional new cases (3 with LGD and 1 with high-grade dysplasia) for an added yield of 19% (=4/21, 95% confidence interval 0.6%-45.7%). DISCUSSION: The addition of WATS-3D to an already thorough examination with HDWLE-NBI-VLE-SP may increase the yield of dysplasia detection.


Asunto(s)
Adenocarcinoma/prevención & control , Esófago de Barrett/diagnóstico , Mucosa Esofágica/diagnóstico por imagen , Neoplasias Esofágicas/prevención & control , Esofagoscopía/métodos , Imagenología Tridimensional , Adenocarcinoma/patología , Adulto , Anciano , Anciano de 80 o más Años , Esófago de Barrett/patología , Esófago de Barrett/terapia , Biopsia , Progresión de la Enfermedad , Mucosa Esofágica/patología , Neoplasias Esofágicas/patología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Imagen de Banda Estrecha/métodos , Estudios Prospectivos , Estudios Retrospectivos , Manejo de Especímenes
14.
BMJ Open Gastroenterol ; 6(1): e000340, 2019.
Artículo en Inglés | MEDLINE | ID: mdl-31749979

RESUMEN

OBJECTIVE: Volumetric laser endomicroscopy (VLE) is an advanced imaging modality used in Barrett's oesophagus (BE) to help identify dysplasia in the oesophagus. VLE criteria exist for oesophageal dysplasia but not for dysplasia in the gastric cardia. The aim of this study was to determine if there are in vivo VLE features that can predict gastric cardia dysplasia in BE. DESIGN: This was a single-centre observational cohort study from August 2016 to August 2018. Patients were included if they had BE, were undergoing a VLE exam as standard of care, and had a suspicious target laser marked at the gastric cardia. The following VLE features were correlated to histology to determine if an association existed between histology subtype and VLE feature: wide crypts, irregular surface, one large isolated gland, multiple glands, and complex glands. RESULTS: A total of 110 in vivo gastric cardia targets in 77 patients with BE were analysed. The following abnormalities were identified: 61 wide crypts, 34 isolated glands, 16 irregular surfaces, 15 multiple glands, and 11 complex glands. Complex glands were the only VLE feature that correlated to any histology subtype. They were present in 71% of targets with high-grade dysplasia (HGD), T1a cancer or T1b cancer and had a sensitivity, specificity, and accuracy of 71%, 99%, and 85%, respectively. Of the 10 patients with complex glands on VLE and HGD/cancer on histology, 4 had a normal-appearing mucosa (40%) on endoscopy. CONCLUSION: Identification of complex glands on VLE may aid in detection of HGD or early cancer in the gastric cardia in BE. This is particularly important, as dysplasia at the gastric cardia can be difficult to see endoscopically.

15.
Gastrointest Endosc ; 90(4): 570-578, 2019 10.
Artículo en Inglés | MEDLINE | ID: mdl-31078571

RESUMEN

BACKGROUND AND AIMS: Peroral endoscopic myotomy (POEM) has emerged as a promising treatment option for achalasia and other foregut dysmotility disorders. However, much of the current postprocedural care, such as mandatory admission and routine esophagrams, has been adapted from current surgical practices and may not in fact be necessary. Here, we describe our algorithm and outcomes for same-day discharge. METHODS: Outcomes of 103 consecutive patients who underwent POEM for achalasia and other foregut dysmotility disorders from January 2015 to December 2018 were analyzed. Patients were discharged on the same day without esophagrams following a predetermined algorithm based on procedural adverse events and postprocedural pain. Patients were closely monitored after discharge for adverse events at 24 and 48 hours and then routinely in the office setting. RESULTS: Of the 103 POEMs, 101 were completed successfully. A total of 62.4% of patients were discharged safely on the same day, 29.7% were admitted for mild pain, and 7.9% were admitted for observation for other reasons. Overall, there were no serious adverse events at any time point. Univariate analysis identified duration of disease greater than 3 years, longer length of procedure (50.9 vs 68.5 min, P < .0001), and longer length of myotomy (7.2 vs 8.5 cm, P < .0068) as significant factors associated with postprocedural pain requiring admission. CONCLUSIONS: Although same-day discharge and foregoing routine esophagram have been suggested by many, this routine has not been systematically implemented. This series suggests that an algorithm for same-day discharge based on postprocedure chest pain and procedural complexity is both safe and feasible.


Asunto(s)
Procedimientos Quirúrgicos Ambulatorios/métodos , Endoscopía del Sistema Digestivo/métodos , Acalasia del Esófago/cirugía , Esfínter Esofágico Inferior/cirugía , Miotomía/métodos , Adulto , Anciano , Algoritmos , Trastornos de la Motilidad Esofágica/cirugía , Estudios de Factibilidad , Femenino , Hospitalización , Humanos , Masculino , Persona de Mediana Edad , Dolor Postoperatorio/tratamiento farmacológico , Dolor Postoperatorio/epidemiología , Readmisión del Paciente , Complicaciones Posoperatorias/epidemiología , Estudios Retrospectivos
16.
Tex Heart Inst J ; 46(1): 28-31, 2019 02.
Artículo en Inglés | MEDLINE | ID: mdl-30833834

RESUMEN

Primary cardiac tumors are rare, and most are benign. Intimal sarcomas are among the rarest of the malignant cardiac tumors; they are aggressive and associated with a poor prognosis. Whereas transesophageal echocardiography has been instrumental in evaluating cardiac masses, other imaging methods, such as cardiac magnetic resonance, have proved invaluable in accurately characterizing these masses. We present the case of a 49-year-old woman in whom we diagnosed a primary intimal sarcoma of the left atrial appendage, and we discuss the importance of multimodal imaging in the evaluation and diagnosis of cardiac masses.


Asunto(s)
Ecocardiografía Transesofágica/métodos , Neoplasias Cardíacas/diagnóstico , Imagen por Resonancia Cinemagnética/métodos , Imagen Multimodal , Sarcoma/diagnóstico , Apéndice Atrial , Biopsia , Diagnóstico Diferencial , Femenino , Humanos , Persona de Mediana Edad
17.
Am Soc Clin Oncol Educ Book ; 37: 301-310, 2017.
Artículo en Inglés | MEDLINE | ID: mdl-28561672

RESUMEN

Pancreatic cancer is a growing problem in oncology, given slowly rising incidence and continued suboptimal outcomes. A concerted effort to reverse this tide will require prevention, early diagnosis, and improved systemic therapy for curable disease. We focus on these aspects in detail in this study. Hereditary pancreatic cancer is an underappreciated area. With the growing use of genomics (both somatic and germline) in cancer care, there is increasing recognition of hereditary pancreatic cancer cases: around 10% of all pancreatic cancer may be related to familial syndromes, such as familial atypical multiple mole and melanoma (FAMMM) syndrome, hereditary breast and ovarian cancer, Lynch syndrome, and Peutz-Jeghers syndrome. Screening and surveillance guidelines by various expert groups are discussed. Management of resectable pancreatic cancer is evolving; the use of multiagent systemic therapies, in the adjuvant and neoadjuvant settings, is discussed. Current and emerging data, along with ongoing clinical trials addressing important questions in this area, are described. Surveillance recommendations based on latest ASCO guidelines are also discussed. Finally, the multimodality management of borderline resectable pancreatic cancer is discussed. The various clinicoanatomic definitions of this entity, followed by consensus definitions, are described. Then, we focus on current opinions and practices around neoadjuvant therapy, discussing chemotherapy and radiation aspects, and the role of surgical resection.


Asunto(s)
Adenocarcinoma/terapia , Terapia Neoadyuvante , Neoplasias Pancreáticas/terapia , Adenocarcinoma/genética , Adenocarcinoma/patología , Humanos , Neoplasias Pancreáticas/genética , Neoplasias Pancreáticas/patología , Factores de Riesgo , Supervivencia , Neoplasias Pancreáticas
18.
Pancreas ; 46(2): 237-243, 2017 02.
Artículo en Inglés | MEDLINE | ID: mdl-27846134

RESUMEN

OBJECTIVES: Tobacco exposure is an established risk factor for pancreatic cancer and chronic pancreatitis; however, its role in pancreatic insufficiency is not clear. METHODS: This controlled, cross-sectional study examined smokers and nonsmokers with no history of pancreatic disease. Histories and validated inventories of alcohol and tobacco use were obtained, and pancreatic insufficiency was assessed using the fecal elastase-1 assay. RESULTS: Of 7854 patients approached, 226 were interviewed and 200 enrolled. The rates of pancreatic insufficiency [18% (18/100)] and severe pancreatic insufficiency [10% (10/100)] were significantly higher in smokers than in controls [6% (6/100), P = 0.009 and 1% (1/100), P = 0.010, respectively]. On multivariate logistic regression, the risk of pancreatic insufficiency in smokers was significantly increased [odds ratio, 4.34 (1.37-13.75); P = 0.012], controlling for alcohol use and relevant covariates. Tobacco exposure was associated with the highest odds ratio for pancreatic insufficiency. Alcohol consumption was strongly associated with tobacco exposure (P < 0.001), but not with pancreatic insufficiency by multivariate analysis (P = 0.792). CONCLUSIONS: This study suggests that tobacco exposure is independently associated with pancreatic exocrine insufficiency in patients without a prior diagnosis of pancreatic disease. Tobacco exposure seems to have greater detrimental effects on pancreatic function than alcohol in this population.


Asunto(s)
Insuficiencia Pancreática Exocrina/diagnóstico , Insuficiencia Pancreática Exocrina/etiología , Fumadores/estadística & datos numéricos , Fumar/efectos adversos , Consumo de Bebidas Alcohólicas/efectos adversos , Estudios Transversales , Heces/enzimología , Femenino , Humanos , Modelos Logísticos , Masculino , Persona de Mediana Edad , Análisis Multivariante , Elastasa Pancreática/metabolismo , Factores de Riesgo
19.
Clin Exp Gastroenterol ; 9: 197-207, 2016.
Artículo en Inglés | MEDLINE | ID: mdl-27555793

RESUMEN

Hereditary pancreatitis (HP) is a rare cause of acute, recurrent acute, and chronic pancreatitis. It may present similarly to other causes of acute and chronic pancreatitis, and often there has been a protracted evaluation prior to the diagnosis of HP. Since it was first described in 1952, multiple genetic defects that affect the action of digestive enzymes in the pancreas have been implicated. The most common mutations involve the PRSS1, CFTR, SPINK1, and CTRC genes. New mutations in these genes and previously unrecognized mutations in other genes are being discovered due to the increasing use of next-generation genomic sequencing. While the inheritance pathways of these genetic mutations may be variable and complex, sometimes involving coinheritance of other mutations, the clinical presentation of patients tends to be similar. Interactions with environmental triggers often play a role. Patients tend to present at an early age (prior to the second decade of life) and have a significantly increased risk for the development of pancreatic adenocarcinoma. Patients with HP may develop sequelae of chronic pancreatitis such as strictures and fluid collections as well as exocrine and endocrine insufficiency. Management of patients with HP involves avoidance of environmental triggers, surveillance for pancreatic adenocarcinoma, medical therapy for endocrine and exocrine insufficiency, pain management, and endoscopic or surgical treatment for complications. Care for affected patients should be individualized, with an emphasis on early diagnosis and multidisciplinary involvement to develop a comprehensive treatment strategy.

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