IgG4-related disease (IgG4-RD) is an immune-mediated condition associated with chronic fibroinflammatory lesions that can affect nearly any organ. IgG4-related hepatobiliary and pancreatic diseases are IgG4-RD involving the hepatobiliary and pancreatic system, which is characterized with elevated serum IgG4 concentrations, large numbers of IgG4 positive lymphoplasma cells infiltration in affected organs, storiform fibrosis, and imaging changes of organ morphology. Due to the lack of reliable biomarkers, histopathology is still an important basis for diagnosis. The pathogenesis of IgG4-related hepatobiliary and pancreatic diseases has not been clarified. This review focuses on the recent advances in intestinal microecology-immunology, host genetics-immunity and recurrence monitoring of IgG4-related hepatobiliary and pancreatic diseases.
Autoimmune Diseases/diagnosis* ; Gastrointestinal Microbiome ; Humans ; Immunoglobulin G ; Immunoglobulin G4-Related Disease/pathology* ; Pancreatic Diseases

Autoimmune pancreatitis (AIP) is a unique subtype of chronic pancreatitis, which shares many clinical presentations with pancreatic ductal adenocarcinoma (PDA). The misdiagnosis of AIP often leads to unnecessary pancreatic resection. F-FDG positron emission tomography/ computed tomography (PET/CT) could provide comprehensive information on the morphology, density, and functional metabolism of the pancreas at the same time. It has been proved to be a promising modality for noninvasive differentiation between AIP and PDA. However, there is a lack of clinical analysis of PET/CT image texture features. Difficulty still remains in differentiating AIP and PDA based on commonly used diagnostic methods. Therefore, this paper studied the differentiation of AIP and PDA based on multi-modality texture features. We utilized multiple feature extraction algorithms to extract the texture features from CT and PET images at first. Then, the Fisher criterion and sequence forward floating selection algorithm (SFFS) combined with support vector machine (SVM) was employed to select the optimal multi-modality feature subset. Finally, the SVM classifier was used to differentiate AIP from PDA. The results prove that texture analysis of lesions helps to achieve accurate differentiation of AIP and PDA.
Adenocarcinoma ; diagnostic imaging ; Algorithms ; Autoimmune Diseases ; diagnostic imaging ; Diagnosis, Differential ; Fluorodeoxyglucose F18 ; Humans ; Pancreatic Neoplasms ; diagnostic imaging ; Pancreatitis ; diagnostic imaging ; Positron Emission Tomography Computed Tomography ; Support Vector Machine

BACKGROUND/AIMS: In this study, we aimed to evaluate the predictive value of localized stenosis of the main pancreatic duct (MPD) for early detection of pancreatic cancer.METHODS: Among 689 patients who underwent endoscopic retrograde pancreatography from January 2008 to September 2018, 19 patients with MPD findings were enrolled. These patients showed findings for indicating suspicious pancreatic cancer at an early stage (FiCE); FiCE was defined as a single, localized stenosis in the MPD without a detectable mass (using any other imaging methods) and without other pancreatic diseases, such as definite chronic pancreatitis, intraductal papillary mucinous neoplasm, and autoimmune pancreatitis. Final diagnoses were established by examining resected specimens or through follow-up examinations after an interval of >5 years.RESULTS: Among 19 patients with FiCE, 11 underwent surgical resection and 8 were evaluated after a >5-year observation period. The final diagnosis of the MPD stenosis was judged to be pancreatic cancer in 9 patients (47%), including 3 with intraepithelial cancer, and to be a non-neoplastic change in 10. The sensitivity, specificity, and accuracy of preoperative pancreatic juice cytology were 75%, 100%, and 88%, respectively.CONCLUSIONS: The predictive value of FiCE for pancreatic cancer prevalence was 47%. Histological confirmation with pancreatic juice cytology is necessary before surgical resection.
Cholangiopancreatography, Endoscopic Retrograde ; Constriction, Pathologic ; Diagnosis ; Follow-Up Studies ; Humans ; Mucins ; Pancreatic Diseases ; Pancreatic Ducts ; Pancreatic Juice ; Pancreatic Neoplasms ; Pancreatitis ; Pancreatitis, Chronic ; Prevalence ; Sensitivity and Specificity

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.
Adolescent* ; Child* ; Constipation ; Cross-Sectional Studies ; Cystic Fibrosis* ; Diagnosis ; Exocrine Pancreatic Insufficiency ; Gastrointestinal Diseases ; Humans ; Infant, Newborn ; Liver Diseases ; Male ; Neonatal Screening ; Nutritional Status ; Phenotype*

Gastrointestinal (GI) endoscopy plays an indispensable role in the diagnosis and management of various pediatric GI disorders. While the pace of development of pediatric GI endoscopy has increased over the years, it remains sluggish compared to the advancements in GI endoscopic interventions available in adults. The predominant reasons that explain this observation include lack of formal training courses in advanced pediatric GI interventions, economic constraints in establishing a pediatric endoscopy unit, and unavailability of pediatric-specific devices and accessories. However, the situation is changing and more pediatric GI specialists are now performing complex GI procedures such as endoscopic retrograde cholangiopancreatography and endoscopic ultrasonography for various pancreatico-biliary diseases and more recently, per-oral endoscopic myotomy for achalasia cardia. Endoscopic procedures are associated with reduced morbidity and mortality compared to open surgery for GI disorders. Notable examples include chronic pancreatitis, pancreatic fluid collections, various biliary diseases, and achalasia cardia for which previously open surgery was the treatment modality of choice. A solid body of evidence supports the safety and efficacy of endoscopic management in adults. However, additions continue to be made to literature describing the pediatric population. An important consideration in children includes size of children, which in turn determines the selection of endoscopes and type of sedation that can be used for the procedure.
Adult ; Cardia ; Child* ; Cholangiopancreatography, Endoscopic Retrograde ; Diagnosis ; Endoscopes ; Endoscopy ; Endoscopy, Gastrointestinal* ; Endosonography ; Esophageal Achalasia ; Humans ; Mortality ; Pancreatic Diseases ; Pancreatitis, Chronic ; Specialization

Mediastinal pancreatic pseudocyst is a rare complication of acute or chronic pancreatitis. Pleural effusion and pneumonia are two of the most common thoracic complications from pancreatic disease, while pancreaticopleural fistula with massive pleural effusion and extension of pseudocyst into the mediastinum is a rare complication of the thorax from pancreatic disease. To the best of our knowledge, there have been no case reports of mediastinal pancreatic pseudocyst-induced esophageal fistula in Korea to date. Here in, we report a case about 43-year-old man of mediastinal pancreatic pseudocyst-induced esophageal fistula presenting with chest pain radiating toward the back and progressive dysphagia. The diagnosis was confirmed by an esophagogastroduodenoscopy and abdomen computed tomography (CT). The patient was treated immediately using a conservative method; subsequently, within 3 days from treatment initiation, symptoms-chest pain and dysphagia-disappeared. In a follow-up gastroscopy 7 days later and abdomen CT 12 days later, mediastinal pancreatic pseudocyst showed signs of improvement, and esophageal fistula disappeared without any complications.
Abdomen ; Adult ; Chest Pain ; Deglutition Disorders ; Diagnosis ; Endoscopy, Digestive System ; Esophageal Fistula* ; Fistula ; Follow-Up Studies ; Gastroscopy ; Humans ; Korea ; Mediastinum ; Methods ; Pancreatic Diseases ; Pancreatic Pseudocyst* ; Pancreatitis ; Pancreatitis, Chronic ; Pleural Effusion ; Pneumonia ; Thorax

Pancreatic malignancy is the third leading cause of cancer related death in the United States with limited viable screening options. By the end of this decade, cancers are poised to become the leading cause of death with pancreatic cancer projected to be the second leading cause of cancer related mortality. Pancreatic cystic lesions (PCLs) are found in approximately 5%–14% of patients due to the increased utilization of cross-sectional imaging, with approximately 8%–10% of pancreatic cancers originating as PCLs. Current screening guidelines have shown discrepancies between morphologic characteristics of PCLs and identifying advanced pancreatic disease. Molecular analysis has emerged as a novel technology to aid in adequate diagnosis and management decisions of PCLs. Mucinous cysts including intraductal papillary mucinous neoplasms (IPMNs) or mucinous cystic neoplasms have similar oncogenic mutations including KRAS, TP53, SMAD4, PIK3CA, PTEN, or CKDN2A, while GNAS and RNF43 mutations are specific only to IPMNs. Serous cystadenomas have been associated with a loss of tumor suppressor gene VHL, while solid-psuedopapillary neoplasms have an oncogenic mutation CTNNB1. A specific molecular marker to diagnose existing high-grade dysplasia or impending malignant transformation is yet to be identified. Moving forward it is important to advance technology in isolating and identifying high-risk molecular markers from cyst fluid while considering their increased utilization in the evaluation of PCLs.
Biomarkers, Tumor ; Cause of Death ; Cyst Fluid ; Cystadenoma, Serous ; Diagnosis ; Genes, Tumor Suppressor ; Humans ; Loss of Heterozygosity ; Mass Screening ; Mortality ; Mucins ; Neoplasms, Cystic, Mucinous, and Serous ; Pancreatic Cyst* ; Pancreatic Diseases ; Pancreatic Neoplasms ; United States

OBJECTIVE: To retrospectively evaluate the CT findings and clinicopathologic features in patients with gastrointestinal (GI) involvement of recurrent renal cell carcinoma (RCC). MATERIALS AND METHODS: The medical records were reviewed for 15 patients with 19 pathologically proven GI tract metastases of RCC. The CT findings were analyzed to determine the involved sites and type of involvement; lesion size, morphology, and contrast enhancement pattern; and occurrence of lymphadenopathy, ascites and other complications. RESULTS: The most common presentation was GI bleeding (66.7%). The average interval between nephrectomy and the detection of GI involvement was 30.4 ± 37.4 months. GI lesions were most commonly found in the ileum (36.8%) and duodenum (31.6%). A distant metastasis (80%) was more common than a direct invasion from metastatic lesions. The mean lesion size was 34.1 ± 15.0 mm. Intraluminal polypoid masses (63.2%) with hyperenhancement (78.9%) and heterogeneous enhancement (63.2%) were the most common findings. No patients had regional lymphadenopathy. Complications occurred in four patients, with one each of bowel obstruction, intussusception, bile duct dilatation, and pancreatic duct dilatation. CONCLUSION: GI involvement of recurrent RCC could be included in the differential diagnosis of patients with heterogeneous, hyperenhanced intraluminal polypoid masses in the small bowel on CT scans along with a relative paucity of lymphadenopathy.
Ascites ; Bile Ducts ; Carcinoma, Renal Cell* ; Diagnosis, Differential ; Dilatation ; Duodenum ; Gastrointestinal Tract ; Hemorrhage ; Humans ; Ileum ; Intussusception ; Lymphatic Diseases ; Medical Records ; Neoplasm Metastasis ; Nephrectomy ; Pancreatic Ducts ; Retrospective Studies ; Tomography, X-Ray Computed

There are increasing number of cases of serum amylase and lipase levels being examined as part of health screening, but the clinical significance of these amylase and lipase levels is unclear. When the clinicians encounter patients with elevated pancreatic enzymes, the most common causes such as acute pancreatitis, hepatic or renal dysfunction should be ruled out first by thorough history taking, physical examination, and laboratory tests. Further tests including abdominal ultrasonography or computed tomography, lipid profile, tumor marker, isoenzyme, and calculation of amylase-to-creatinine clearance ratio or polyethylene glycol precipitation test should be performed to exclude other causes. If the pancreatic enzymes are continuously elevated through repeated tests without any apparent etiology, the diagnosis is made with chronic non-pathological pancreatic hyperenzymemia (CNPH). Magnetic resonance cholangiopancreatography is very useful and important modality for the patients with CNPH but the clinical significance of magnetic resonance cholangiopancreatography with secretin stimulation is still unclear. They can be evaluated through endoscopic ultrasonography with preference but it is less suitable for follow-up. Individualized approaches should be made after considering the need for active treatment or periodic follow-up for the benign pancreatic diseases associated with CNPH. It is difficult to conclude until more long-term data are reported because there are only limited number of researches and consensus on the range of tests to be performed for diagnosis, clinical significance of benign findings and end of follow-up in patients with CNPH.
Amylases ; Cholangiopancreatography, Magnetic Resonance ; Consensus ; Diagnosis ; Endosonography ; Follow-Up Studies ; Humans ; Hyperamylasemia* ; Lipase ; Mass Screening ; Pancreas ; Pancreatic Diseases ; Pancreatitis ; Physical Examination ; Polyethylene Glycols ; Secretin ; Ultrasonography

Mediastinal pancreatic pseudocyst is a rare complication of acute or chronic pancreatitis. Pleural effusion and pneumonia are two of the most common thoracic complications from pancreatic disease, while pancreaticopleural fistula with massive pleural effusion and extension of pseudocyst into the mediastinum is a rare complication of the thorax from pancreatic disease. To the best of our knowledge, there have been no case reports of mediastinal pancreatic pseudocyst-induced esophageal fistula in Korea to date. Here in, we report a case about 43-year-old man of mediastinal pancreatic pseudocyst-induced esophageal fistula presenting with chest pain radiating toward the back and progressive dysphagia. The diagnosis was confirmed by an esophagogastroduodenoscopy and abdomen computed tomography (CT). The patient was treated immediately using a conservative method; subsequently, within 3 days from treatment initiation, symptoms-chest pain and dysphagia-disappeared. In a follow-up gastroscopy 7 days later and abdomen CT 12 days later, mediastinal pancreatic pseudocyst showed signs of improvement, and esophageal fistula disappeared without any complications.
Abdomen ; Adult ; Chest Pain ; Deglutition Disorders ; Diagnosis ; Endoscopy, Digestive System ; Esophageal Fistula ; Fistula ; Follow-Up Studies ; Gastroscopy ; Humans ; Korea ; Mediastinum ; Methods ; Pancreatic Diseases ; Pancreatic Pseudocyst ; Pancreatitis ; Pancreatitis, Chronic ; Pleural Effusion ; Pneumonia ; Thorax