A Case of Atypical Familial Mediterranean Fever With Pseudopolyposis-Like Mucosal Changes in the Ileum.

A 15-year-old male patient presented with recurrent fever. Three months prior, he experienced repeated fevers of 38°C, headaches, and malaise for three days. He experienced repeated fevers over 38°C for >72 hours two weeks prior to the current visit. A computed tomography scan showed enlarged lymph nodes around the ileum, suggesting familial Mediterranean fever (FMF) or inflammatory bowel disease. Endoscopic examination revealed a deformed Bauhin valve and inflammatory changes in the ileum, making inflammatory bowel disease unlikely. Thus, FMF was suspected, and after a thorough explanation, the patient was treated with colchicine, and his symptoms improved. Genetic testing revealed a mutation in the MEFV gene P369S-R408Q, and atypical FMF was diagnosed.

A Case of Pancreaticobiliary Maljunction with Repeated Pancreatitis Due to Protein Plugs in a Short Period.

A 70-year-old woman was admitted to the hospital 1 month prior to presentation with acute pancreatitis due to pancreaticobiliary maljunction. After discharge, she was referred for elevated hepatobiliary enzyme levels. She was diagnosed with an acute pancreatitis flare-up. Computed tomography revealed dilation of the common duct compared to the previous admission. Considering the protein plug formation as the cause, endoscopic retrograde cholangiopancreatography (ERCP) was performed after improvement. ERCP revealed a defect in the duct, suspected to be caused by protein plugs, which were removed using a balloon after endoscopic papillary balloon dilatation. An analysis revealed that this component was a protein. No recurrence of pancreatitis was observed after the treatment.

Takayasu's arteritis associated with Crohn's disease treated with infliximab.

A 23-year-old woman presented with fever, diarrhea, bloody stools, and arthralgia that did not improve despite previous treatments and was diagnosed with Crohn's disease. Remission was achieved after the introduction of infliximab, nutritional therapy, and 5-aminosalicylic acid treatment. However, the patient's blood sedimentation rate remained elevated without symptom recurrence, except for abdominal pain in the following year. Aortic wall thickening in the thoracic descending aorta was also observed on computed tomography. Accumulation in the thoracic descending aorta and abdominal aorta was confirmed using positron emission tomography-computed tomography. The patient was diagnosed with Takayasu's arteritis. The patient's abdominal symptoms resolved, and her blood sedimentation rate normalized after steroid administration.

Gastrocolic fistula caused by transverse colon cancer: a case report.

BACKGROUND: A gastrocolic fistula is an unusual communication between the colon and the stomach. Although colon cancer is the most common malignant cause of gastrocolic fistula in the Western world, the incidence of gastrocolic fistula due to colon cancer is 0.3% in operated cases. CASE PRESENTATION: A 68-year-old man presented with anorexia, general malaise, weight loss, and vomiting of fecal matter. Investigations revealed that the patient had a large nonmetastatic splenic flexure tumor that was diagnosed as colon cancer and had invaded the stomach and pancreas. An upper gastrointestinal series confirmed a gastrocolic fistula. Left hemicolectomy, distal gastrectomy, distal pancreatectomy, and splenectomy were performed. Histology revealed transverse colon cancer, which was UICC stage (8th edition) pT4bN1bcM0 pStage IIIC. Adjuvant chemotherapy was not performed. There was no recurrence or metastasis one year after surgery. We reviewed 17 cases including our case of a gastrocolic fistula caused by colon cancer. Neoadjuvant chemotherapy was not given to any of the patients, and en bloc resections were conducted in all cases. Adjuvant chemotherapy was given to almost all of the patients. There was no recurrence or metastasis. CONCLUSIONS: For gastrocolic fistula caused by advanced colon cancer, secure en bloc surgical resection was the initial treatment in all 17 reported cases including the present case, and adjuvant chemotherapy may contribute to a better prognosis.

Mesalazine-Induced Diffuse Lung Injury in a Patient Without Respiratory Signs and Symptoms.

Mesalazine is a drug used to treat ulcerative colitis and Crohn's disease, and is known to rarely cause lung injury. We show herein a unique case who developed this drug-induced injury. A 17-year-old boy presented with fever and anorexia after administration of mesalazine. Computed tomography showed extensive ground-glass opacities with peripheral distribution in both lungs. He had general weakness, but had no respiratory symptoms such as cough and dyspnea. With prednisolone, which is primarily aimed at controlling ulcerative colitis, the extensive opacity in both lungs were improved. All patients with this drug-induced lung injury reported to date have had respiratory symptoms, but this patient had no subjective respiratory symptoms and had no abnormalities in respiratory rate and oxyhaemoglobin saturation. Although very rare, we do believe that this clinical course will provide some suggestive information on treatment for patients with similar course in the future.

Aspiration pneumonia associated with achalasia.

In this case, a chest computed tomography scan of a young patient with pneumonia revealed esophageal obstruction, which led to the diagnosis of aspiration pneumonia due to achalasia. This report highlights achalasia and other gastrointestinal obstructions as one of the causes of aspiration pneumonia.

Serotonin-positive neuroendocrine neoplasm with pancreatic ductal dilation in which cytological immunostaining contributed to the diagnosis.

A 52-year-old man was transported via an ambulance because of syncope and the passage of tarry stools, which had been noted the previous day. He was diagnosed with upper gastrointestinal bleeding from a gastric ulcer and underwent endoscopic hemostasis. Prior to endoscopy, abdominal computerized tomography performed for gastrointestinal bleeding revealed pancreatic duct dilation. After discharge, abdominal imaging revealed a strongly enhancing tumor (5 mm) with caudal pancreatic duct dilation. Endoscopic retrograde pancreatography revealed that the main pancreatic duct was interrupted at the body. Pancreatic juice cytology was class III, and additional immunostaining were positive for chromogranin A, synaptophysin, and serotonin, suggesting a pancreatic neuroendocrine neoplasm (NEN). Distal pancreatectomy was performed and a yellowish-white solid lesion was found in the pancreatic duct. Pathological examination revealed narrowing of the pancreatic duct, extensive stromal fibrosis, and proliferation of tumor cells with small round nuclei and eosinophilic vesicles. Furthermore, the immunostaining findings of the resected specimen corresponded with those of the cytology. A diagnosis of NEN G1 (WHO classification) with Ki-67 index < 1% was made. Imaging of the pancreatic duct tend to be normal or show no involvement of the duct in pancreatic neuroendocrine neoplasms; however, there have been a few reports of stenosis due to fibrosis around the pancreatic duct. Serotonin positivity was previously documented to be significantly higher in patients with fibrosis. In lesions with pancreatic ductal stenosis, the addition of immunostaining to pancreatic juice cytology was thought to be useful in differentiating pancreatic cancer from pNEN.

Spontaneous retroperitoneal bleeding secondary to celiac artery compression syndrome.

Clinicians should consider celiac artery compression syndrome as the cause of ruptured visceral aneurysm and dissection and ask patients for unexplained chronic abdominal symptoms. Endovascular embolization with metallic coil placement is the first-line treatment, and surgery can be avoided in some cases.

Pancreatic panniculitis in a patient with pancreatic adenosquamous carcinoma.

Pancreatic panniculitis is a rare complication of pancreatic diseases. We aimed to evaluate a case of pancreatic panniculitis. A 58-year-old woman was referred to our hospital with complaints of painful cutaneous nodules on her limbs. Various diagnostic tests confirmed pancreatic panniculitis and pancreatic adenosquamous carcinoma. We diagnosed pancreatic panniculitis by a skin nodule biopsy that revealed fine basophilic material within anucleate cells and neutrophil infiltration. Abdominal imaging detected a tumor with necrosis on the pancreas and endoscopic ultrasound-guided fine-needle aspiration revealed it as an adenocarcinoma. The patient underwent pancreatoduodenectomy after neoadjuvant chemotherapy. The tumor was composed of differentiated adenocarcinoma and squamous cell carcinoma and diagnosed as adenosquamous carcinoma. This is the first report of pancreatic panniculitis in a patient with adenosquamous cell carcinoma of the pancreas.

Ceftriaxone pseudolithiasis detected by computed tomography and followed up until resolution.

Introduction: Ceftriaxone is a third-generation cephalosporin antibiotic that has been widely used to treat various infectious diseases. We report a case of ceftriaxone pseudolithiasis that was detected by computed tomography (CT) and followed up until it was resolved. Case: A 76-year-old woman with diabetes mellitus and renal impairment, but no history of gallstones, was diagnosed with septic shock due to renal and lung abscesses and treated with ceftriaxone. On day 22 after admission, abdominal CT revealed a gallstone, which increased in size up to day 50. Ceftriaxone was stopped on day 50, and the gallstone resolved completely after 10 weeks. Conclusion: Ceftriaxone pseudolithiasis should be cautiously considered, specifically in a patient with renal impairment and a prolonged treatment period.

Balloon-Occluded Trans-Arterial Chemo-Embolization Technique with Repeated Alternate Infusion of Cisplatin Solution and Sparse Gelatin Slurry (RAIB-TACE) for Large Hepatocellular Carcinoma Nodules More than 7 cm in Diameter.

OBJECTIVE: It is sometimes difficult to obtain complete/partial response of large hepatocellular carcinoma (HCC) nodules by trans-arterial chemoembolization/embolization (TACE/TAE). The aim is retrospective investigation of tumor response of large HCC nodules (>7 cm) treated by the new TACE technique, repeated alternate infusion of cisplatin solution, and sparse gelatin slurry under balloon occlusion (RAIB-TACE). Materials and Methods. A microballoon catheter was placed at a proximal portion of the hepatic artery (subsegmental to the lobar level hepatic artery), and alternate infusion of cisplatin solution and sparse gelatin slurry were repeated under balloon occlusion until stasis of gelatin slurry beyond the catheter was seen. RAIB-TACE of multiple proximal hepatic and extrahepatic collateral arteries were performed to treat hemi-lobe or more of the liver while avoiding infusion into gastric and cystic arteries for 19 large nodules (>7 cm) in 19 patients without portal venous invasion. All patients underwent dynamic CT/MRI 1-3 months after RAIB-TACE, and tumor response of each large nodule was evaluated based on modified RECIST criteria. RESULTS: CR, PR, SD, and PD were achieved in 11, 8, 0, and 0 nodules, respectively. CR and PR were considered as success, and the ratio of success was 100%. Major complications were abscess formation in the necrotic nodule (n = 1) which was treated by drainage tube placement, and subsegmental level liver infarction (n = 1) which was treated by drainage tube placement, and subsegmental level liver infarction (. CONCLUSION: New TACE technique, RAIB-TACE, was useful to achieve successful response of large HCC nodules.

Alcohol Consumption and the Risk for Developing Pancreatitis: A Case-Control Study in Japan.

OBJECTIVE: This study aimed to estimate the association of alcohol consumption and pancreatitis in Japan. METHODS: We performed a nationwide case-control study, which included 982 patients (574 patients with acute pancreatitis and 408 patients with chronic pancreatitis) and 1015 controls who were individually matched for sex, age, hospital, and time of their first hospital visit. Conditional logistic regression was used to assess the association of alcohol consumption and smoking with pancreatitis. RESULTS: The patients had a mean (SD) age of 57.6 (17.0) years; 71.8% were male. Compared with nondrinkers, alcohol consumption of less than 20 g/d was not associated with the risk for total pancreatitis (odds ratio [OR], 1.0; 95% confidence interval [CI], 0.7-1.4). In patients with acute pancreatitis, the ORs (95% CI) for alcohol consumption of 20 ≤ ∼ < 40 g/d, 40 ≤ ∼ < 60 g/d, 60 ≤ ∼ < 80 g/d, 80 ≤ ∼ < 100 g/d, and 100 g/d or greater were 1.7 (0.9-3.0), 3.1 (1.6-5.9), 4.2 (2.1-8.2), 5.3 (2.4-12.0), and 6.4 (3.4-12.4), respectively. In patients with chronic pancreatitis, the ORs (95% CI) for alcohol consumption of 20 ≤ ∼ < 40 g/d, 40 ≤ ∼ < 60 g/d, 60 ≤ ∼ < 80 g/d, 80 ≤ ∼ < 100 g/d, and 100 g/d or greater were 2.6 (1.2-5.5), 3.2 (1.5-7.1), 9.2 (4.1-20.3), 13.0 (5.3-31.6), and 19.6 (8.2-46.8), respectively. CONCLUSIONS: Our study precisely measured the quantitative effect of alcohol on the risk for developing pancreatitis.

Pancreatic duct drainage using EUS-guided rendezvous technique for stenotic pancreaticojejunostomy.

The patient was a 30-year-old female who had undergone excision of the extrahepatic bile duct and Roux-en-Y hepaticojejunostomy for congenital biliary dilatation at the age of 7. Thereafter, she suffered from recurrent acute pancreatitis due to pancreaticobiliary maljunction and received subtotal stomach-preserving pancreaticoduodenectomy. She developed a pancreatic fistula and an intra-abdominal abscess after the operation. These complications were improved by percutaneous abscess drainage and antibiotic therapy. However, upper abdominal discomfort and the elevation of serum pancreatic enzymes persisted due to stenosis from the pancreaticojejunostomy. Because we could not accomplish dilation of the stenosis by endoscopic retrograde cholangiopancreatography, we tried an endoscopic ultrasonography (EUS) guided rendezvous technique for pancreatic duct drainage. After transgastric puncture of the pancreatic duct using an EUS-fine needle aspiration needle, the guidewire was inserted into the pancreatic duct and finally reached to the jejunum through the stenotic anastomosis. We changed the echoendoscope to an oblique-viewing endoscope, then grasped the guidewire and withdrew it through the scope. The stenosis of the pancreaticojejunostomy was dilated up to 4 mm, and a pancreatic stent was put in place. Though the pancreatic stent was removed after three months, the patient remained symptom-free. Pancreatic duct drainage using an EUS-guided rendezvous technique was useful for the treatment of a stenotic pancreaticojejunostomy after pancreaticoduodenectomy.

Connexins regulate cell functions in pancreatic stellate cells.

OBJECTIVES: Pancreatic stellate cells (PSCs) play a pivotal role in pancreatic fibrosis associated with chronic pancreatitis and pancreatic cancer. Connexins (Cxs) allow direct intercellular communications as components of gap junction but also play important roles in the regulation of cell proliferation, cell differentiation, and tissue development. We here examined the expression of Cxs and Cx-mediated regulation of cell functions in PSCs. METHODS: Human PSCs were isolated from patients undergoing operation for chronic pancreatitis or pancreatic cancer. The expression of Cxs was examined by reverse transcription polymerase chain reaction, Western blotting, and immunofluorescent staining. The roles of Cxs in PSC functions were examined by using carbenoxolone, a broad-spectrum Cx inhibitor, and small interfering RNA for Cx43. RESULTS: Human activated PSCs expressed a variety of Cxs including Cx43 both in vitro and in vivo. Carbenoxolone inhibited platelet-derived growth factor-BB-induced proliferation and migration, and type I collagen expression in PSCs. In addition, carbenoxolone inhibited the activation of quiescent PSCs to a myofibroblastlike phenotype. Decreased Cx43 expression by small interfering RNA resulted in decreased proliferation and type I collagen expression. CONCLUSIONS: Pancreatic stellate cells expressed a variety of Cxs. Connexins, especially Cx43, might regulate the cell functions and activation of PSCs.

Early detection of low enhanced pancreatic parenchyma by contrast-enhanced computed tomography predicts poor prognosis of patients with acute pancreatitis.

OBJECTIVES: The usefulness of early severity assessment of acute pancreatitis (AP) by contrast-enhanced computed tomography (CECT) was investigated. METHODS: Data were obtained from a 2007 nationwide survey in Japan. Clinical data of 983 patients with AP were analyzed. All were examined by CECT on the day of admission. RESULTS: Early findings of CECT demonstrated that low enhanced pancreatic parenchyma (LEPP) was associated with the incidence of organ failure (OF), multiple OF, and infectious complications as well as mortality (P < 0.0001). Next, patients were further divided into 4 groups according to the CECT findings, which focused on the LEPP and peripancreatic collections (PPCs). The LEPP/PPC (+/+) group was characterized as high morbidity and high mortality. The incidence of OF (28.2%), multiple OF (15.5%), and mortality (11.4%) in patients assigned to the (+/+) group was significantly higher than in those assigned to the other groups. The incidence of infectious complications was significantly higher in patients assigned to the (+/+) group (16.7%), the (+/-) group (9.0%), and the (-/+) group (7.0%) than those assigned to the (-/-) group (1.8%). CONCLUSIONS: The detection of LEPP and PPC was a useful CECT finding for the early assessment of the severity of AP.

Do genetic variants in the SPINK1 gene affect the level of serum PSTI?

BACKGROUND: The serine protease inhibitor Kazal type 1 (SPINK1), also known as pancreatic secretory trypsin inhibitor (PSTI), is a peptide secreted by pancreatic acinar cells. Genetic studies have shown an association between SPINK1 gene variants and chronic pancreatitis or recurrent acute pancreatitis. The aim of this study was to clarify whether the SPINK1 variants affect the level of serum PSTI. METHODS: One hundred sixty-three patients with chronic pancreatitis or recurrent acute pancreatitis and 73 healthy controls were recruited. Serum PSTI concentrations were determined with a commercial radioimmunoassay kit. RESULTS: Ten patients with the p.N34S variant, 7 with the IVS3+2T>C variant, two with both the p.N34S and the IVS3+2T>C variants, and one with the novel missense p.P45S variant in the SPINK1 gene were identified. The serum PSTI level in patients with no SPINK1 variants was 14.3 ± 9.6 ng/ml (mean ± SD), and that in healthy controls was 10.7 ± 2.2 ng/ml. The PSTI level in patients carrying the IVS3+2T>C variant (5.1 ± 3.4 ng/ml), but not in those with the p.N34S variant (8.9 ± 3.5 ng/ml), was significantly lower than that in the patients without the SPINK1 variants and the healthy controls. The serum PSTI level in the patient with the p.P45S variant was 4.9 ng/ml. Low levels of serum PSTI (<6.0 ng/ml) showed sensitivity of 80 %, specificity of 97 %, and accuracy of 96 % in the differentiation of IVS3+2T>C and p.P45S carriers from non-carriers. CONCLUSION: Serum PSTI levels were decreased in patients with the IVS3+2T>C and p.P45S variants of the SPINK1 gene.

Long-period pancreatic stenting for painful chronic calcified pancreatitis required higher medical costs and frequent hospitalizations compared with surgery.

OBJECTIVES: The aim of this study was to compare the benefits between endoscopic drainage and surgical drainage of the pancreatic duct for patients with chronic calcified pancreatitis. METHODS: A total of 68 patients were classified into endoscopic (n = 34) or surgical (n = 34) treatment groups. Patients receiving endoscopic treatment were further divided into 2 subgroups: a short-period group, patients who could discontinue serial pancreatic stenting within 1 year (n = 19); and a long-period group, patients who needed pancreatic drainage by serial endoscopic stenting for more than 1 year (n = 15). The medical records of these patients were retrospectively analyzed. RESULTS: Hospital stays, frequency of hospitalizations, and medical expense were similar between the short-period endoscopic treatment group and surgery group. On the other hand, patients in the long-period endoscopic treatment group required significantly longer hospital stays, more frequent hospitalizations, and had higher medical expenses than the short-period endoscopic treatment group as well as than the surgery group. CONCLUSIONS: Patients who underwent serial endoscopic stenting for more than 1 year showed no benefit compared with surgical treatment in terms of the frequency of hospital stays and medical costs.

Perfusion computed tomography findings of autoimmune pancreatitis.

OBJECTIVES: The aim of this study was to clarify the pancreatic blood perfusion in patients with autoimmune pancreatitis (AIP) and the changes after steroid treatment. METHODS: Perfusion computed tomography was performed in 11 patients with AIP and 12 control subjects. Pancreatic volumetric blood flow (F(V)), volume of distribution (V(D)), and blood transit time τ were determined from a single-compartment kinetic model. Nine patients with AIP were reexamined by perfusion computed tomography after corticosteroid administration. RESULTS: The pancreatic F(V) values of the 11 patients with AIP (82.7/min) were significantly lower than those of control subjects (163.5/min, P = 0.0006). On the other hand, the pancreatic V(D) and τ values were not significantly different between AIP and normal. After steroid treatment, the F(V) values of 9 reexamined patients with AIP (76.2/min) were significantly elevated (109.8/min, P = 0.0391). However, the changes of the values after the treatment differed in degree among individuals. The values of 4 patients were dramatically elevated to greater than 100/min, whereas those of 4 other patients did not improve well. The value of the remaining patient whose initial F(V) value was normal (168.09/min) did not change after the treatment. CONCLUSIONS: Pancreatic volumetric perfusion was attenuated in AIP patients. The perfusion was improved after the steroid treatment.

Genetic background is different between sentinel and recurrent acute pancreatitis.

BACKGROUND AND AIM: Previous studies have shown an association of variants in trypsin-associated genes, such as cationic trypsinogen (PRSS1) and serine protease inhibitor, Kazal type-1 (SPINK1) with pancreatitis. However, whether these genetic variants are associated with acute pancreatitis (AP) remains largely unknown, especially when the first attack is separated from recurrent attacks. METHODS: A total of 261 patients with AP (174 with a sentinel attack, and 87 with recurrent attacks) and healthy controls were genotyped for the p.R122H mutation in the PRSS1 gene, p.N34S and IVS3 + 2T > C variants in the SPINK1 gene, the p.G191R variant in the anionic trypsinogen gene, the p.E32del variant in the mesotrypsinogen (PRSS3) gene, and the -2518G > A variant in the monocyte chemoattractant protein-1 gene by polymerase chain reaction-restriction enzyme digestion and direct sequencing. RESULTS: Patients with recurrent attacks were younger. The proportions of biliary pancreatitis and severe cases were lower, and that of idiopathic pancreatitis was higher in patients with a sentinel attack than in those with recurrent attacks. The frequencies of the genetic variants examined did not differ between controls and patients with sentinel pancreatitis. The frequencies of the PRSS1 p.R122H mutation, SPINK1 p.N34S variant, and PRSS3 p.E32del variant, but not other genetic variants, were higher in patients with recurrent attacks than in controls or those with a sentinel attack. CONCLUSIONS: The PRSS1 p.R122H mutation, SPINK1 p.N34S, and PRSS3 p.E32del variants were associated with recurrent, but not sentinel AP. The genetic background could possibly be different between sentinel and recurrent AP.