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1.  Hydroxyurea and colonic ulcers: a case report 
BMC Gastroenterology  2014;14:134.
Background
Hydroxyurea at a relatively low dose is frequently prescribed to induce hemoglobin F production in patients with sickle cell and β-thalassemia diseases because of its good efficacy and safety profiles. However, a potentially fatal gastrointestinal ulceration was recently found and herein reported.
Case presentation
A thirty-seven-year-old man with transfusion dependent hemoglobin E/β-thalassemia disease was treated with hydroxyurea to induce hemoglobin F production since 2007 without incident. From 2008 to April 2010, episodes of hematochezia, mucous diarrhea and epigastric pain intermittently manifested. Four colonoscopies done during the period repeatedly showed ulcerative lesions from the terminal ileum to the ascending colon with a non-specific histo-pathologic finding. Subsequently, ulcerative lesions also developed at the pharynx, histo-pathologic findings of which were not different from those in the colon. These ulcerative lesions resolved within a month after discontinuing hydroxyurea in April 2010 and have not recurred since.
Conclusion
The findings suggested role of hydroxyurea in the pathogenesis of these ulcers, and that it must be immediately discontinued to prevent further damage to the digestive mucosa.
doi:10.1186/1471-230X-14-134
PMCID: PMC4127553  PMID: 25082414
Hydroxyurea; Colonic and oral ulcers; Thalassemia
2.  Lymphomatoid granulomatosis associated with azathioprine therapy in Crohn disease 
BMC Gastroenterology  2014;14:127.
Background
Lymphomatoid granulomatosis (LYG) is a rare Epstein-Barr virus-associated lymphoproliferative disorder. It most often occurs in patients with immunodeficiency and the clinical course ranges from indolent behavior to that of an aggressive malignancy. Pulmonary, central nervous system and dermatological manifestations are most common. To our knowledge this is the first reported case of LYG related to azathioprine therapy in Crohn disease.
Case presentation
A twenty-six year old Caucasian woman with colonic Crohn disease on maintenance azathioprine therapy presented with right upper quadrant pain and fever. Diagnostic imaging revealed extensive liver, pulmonary and cerebral lesions. A diagnosis of LYG was made based on the pattern of organ involvement and the immunohistochemical features on liver and lung biopsy.
Conclusions
Thiopurine therapy for inflammatory bowel disease is associated with an increased incidence of lymphoproliferative disorders. This report highlights the diagnostic challenges associated with LYG. As long-term thiopurine therapy remains central to the management of inflammatory bowel diseases it is essential that both patients and clinicians are aware of this potential adverse outcome.
doi:10.1186/1471-230X-14-127
PMCID: PMC4105046  PMID: 25022612
Lymphoproliferative disorders; Crohn disease; Inflammatory bowel disease; Azathioprine; Thiopurines
3.  Gastrinoma and neurofibromatosis type 2: the first case report and review of the literature 
BMC Gastroenterology  2014;14:110.
Background
Gastroenteropancreatic neuroendocrine tumors have occasionally been described in association with neurofibromatosis type 1, whereas an association with neurofibromatosis type 2 has never been reported.
Case presentation
This report refers to an Italian 69 year old woman with neurofibromatosis type 2 and a pancreatic gastrinoma. In the past she had encephalic meningiomas, a tongue schwannoma and bilateral acoustic neurinomas. She presented with weight loss and a long-term history of diarrhea, responsive to proton pump inhibitors. Upper gastrointestinal endoscopy revealed peptic ulcer of the duodenal bulb. Blood tests were normal, except for the elevation of plasma gastrin (1031 pg/ml; reference value <108) and chromogranin A (337 U/L; reference value <36). After secretin stimulation testing, the plasma gastrin level rose to 3789 pg/ml. The abdomen magnetic resonance imaging and gallium68-DOTATOC positron emission tomography scan demonstrated the presence of a 1.2 x 2 cm lesion in the pancreatic head and a liver metastatis. Pancreatic endoscopic ultrasound with fine needle aspiration revealed cytomorphologic features suggestive of pancreatic gastrinoma. Brain magnetic resonance showed a pituitary microadenoma. There was no evidence of hyperparathyroidism. The genetic test for multiple endocrine neoplasia type 1 syndrome mutation was negative.
Conclusion
This report focuses on the first case of coexistence of gastrinoma with neurofibromatosis type 2. Although the clinical relevance of this association remains to be determined, our case report will surely give cause for due consideration.
doi:10.1186/1471-230X-14-110
PMCID: PMC4082280  PMID: 24961548
Gastrinoma; Neurofibromatosis type 2; Multiple endocrine neoplasia syndrome type 1; Neuroendocrine tumors; Zollinger Ellison syndrome
4.  Hepatobiliary cystadenocarcinoma without mesenchymal stroma in a female patient: a case report 
BMC Gastroenterology  2014;14:109.
Background
Hepatobiliary cystadenocarcinoma is a rare epithelial malignant neoplasm of the liver or extrahepatic bile ducts. Early diagnosis of hepatobiliary cystadenocarcinoma is difficult because of its asymptomatic features and rarity. Moreover, the molecular pathogenesis of hepatobiliary cystadenocarcinoma remains unclear. Herein, we described a case of hepatobiliary cystadenocarcinoma in female with chronic hepatitis B and repeated hepatolithiasis.
Case presentation
A 65-year-old woman with medical history of latent hepatitis B virus infection, repeated choledocholisthiasis, and cholecystitis was admitted due to a heterogeneous cystic mass (5.6 cm × 4 cm) shown on abdominal ultrasonography during regular physical checkup. The patient complained about irregular bowel movements with intermittent diarrhea for two months before presentation. Computed tomography (CT) disclosed a multiloculated cystic lesion in the left hepatic lobe with the presence of intraductal stones and dilatation of intrahepatic ducts. Histological results obtained from left lobectomy specimens showed hepatobiliary cystadenocarcinoma without accompanied mesenchymal stroma.
Conclusion
Notably, hepatobiliary cystadenocarcinoma without mesenchymal stroma seldom occurs in women and is usually associated with poor prognosis. We present the rare findings in this patient and suggest that chronic inflammatory insults in the intrahepatic bile ducts might shed light on the cystadenocarcinogenesis.
doi:10.1186/1471-230X-14-109
PMCID: PMC4065581  PMID: 24934314
Hepatobiliary cystadenocarcinoma; Biliary tumors; Hepatolithiasis; Cholestasis
5.  Intestinal obstruction due to dual gastrointestinal atresia in infants: diagnosis and management of 3 cases 
BMC Gastroenterology  2014;14:108.
Background
Several types of congenital lesions can cause complete or incomplete obstruction of the intestine. Our purpose is to present 3 neonates with dual intestinal type I atresia, i.e., simultaneous obstructive lesions at 2 locations in which the atresia manifested as diaphragm-like tissue.
Case presentation
All 3 cases were female infants ranging in age from 2 to 14 months. The common symptom in all cases was intermittent persistent vomiting. In some cases the vomitus was bilious, and other symptoms included abdominal distention and delayed meconium passage. Prior surgeries at another hospital were unsuccessful at relieving the symptoms in one case. One case had dual lesions in the colon, one dual lesions in the duodenum, and one atresia at both the distal portion of the ileum and the descending colon. Surgical exploration and removal of the lesions at our hospital was successful in all cases, and the infants were discharged in good condition.
Conclusions
Type I atresia can manifest as a diaphragm-like tissue obstructing the continuity of gastrointestinal tract, and in rare cases multiple areas may be present. Base on the intermittent nature of the associated symptoms, diagnosis can be difficult and is often delayed. Physicians should be aware of this condition during the work-up of an infant with persistent intermittent vomiting.
doi:10.1186/1471-230X-14-108
PMCID: PMC4064280  PMID: 24928109
Type I Atresia; Gastrointestinal diaphragm; Congenital; Intestinal obstruction; Infant
6.  Coexistence of HBsAg and HBsAb in a difficult-to-treat chronic hepatitis B: loss of HBsAg with entecavir plus tenofovir combination 
BMC Gastroenterology  2014;14:94.
Background
Some reports have documented the coexistence of Hepatitis B surfage Antigen (HBsAg) and anti-HBsAg antibodies (HBsAb) in patients with chronic hepatitis B (CHB), often in the absence of amino acid substitutions in the HBsAg sequences of the Hepatitis B Virus (HBV) genome able to explain an immunological escape variant.
HBV genome has a very compact coding organization, with four partially overlapping open reading frames (ORFs). Because the reverse transcriptase region (rt) of HBV polymerase overlaps the HBsAg ORF, it is possible that some mutations in the HBsAg region correspond to mutations in the rt ORF, conferring resistance to current antiviral therapies.
This unique case explores the response to antiviral therapies of a CHB with concurrent HBsAg and HBsAb positivity, and analyse the clinical implications of possible mutations in rt and HBsAg ORFs.
Case presentation
Here we describe the case of a 59 year-old Italian man suffering from Hepatitis B envelope Antigen (HBeAg) positive CHB with concurrent HBsAb positivity. By ultra-deep pyro-sequencing (UDPS) technique, mutations conferring immunological escape or resistance to antiviral therapies were found neither in HBsAg nor in HBV rt ORFs, respectively.
The patient was unsuccessfully treated with interferon, adefovir monotherapy and adefovir plus entecavir combination. Surprisingly, during entecavir plus tenofovir combination, anti-HBe seroconversion and HBsAg loss were observed, while the titer of HBsAb persisted.
Conclusions
Concurrent HBsAg/HBsAb positivity in active CHB is a clinical and virological dilemma. In this setting, there are not consistent data about the response to conventional therapies and the immunological balance between host and virus remains so far unexplained. This is, to our knowledge, the first case described of a CHB with HBsAg/HBsAb positivity, wild type for clinically relevant mutations in HBsAg and rt ORFs, successfully treated with a combination of nucleot(s)ide analogues (NAs).
doi:10.1186/1471-230X-14-94
PMCID: PMC4031327  PMID: 24885182
HBeAg positive chronic hepatitis B; HBsAg; Anti-HBs; Coexistence; Ultra-deep pyro-sequencing; Immunological escape; Nucleos(t)ide analogues; Combination; Entecavir; Tenofovir
7.  Sigmoid colon cancer arising in a diverticulum of the colon with involvement of the urinary bladder: a case report and review of the literature 
BMC Gastroenterology  2014;14:90.
Background
Colon cancer can arise from the mucosa in a colonic diverticulum. Although colon diverticulum is a common disease, few cases have been previously reported on colon cancer associated with a diverticulum. We report a rare case of sigmoid colon cancer arising in a diverticulum with involvement of the urinary bladder, which presented characteristic radiographic images.
Case presentation
A 73-year-old man was admitted to our hospital for macroscopic hematuria. Computed tomography and magnetic resonance imaging revealed a sigmoid colon tumor that protruded into the urinary bladder lumen. The radiographs showed a tumor with a characteristic dumbbell-shaped appearance. Colonoscopy showed a type 1 cancer and multiple diverticula in the sigmoid colon. A diagnosis of sigmoid colon cancer with involvement of the urinary bladder was made based on the pathological findings of the biopsied specimens. We performed sigmoidectomy and total resection of the urinary bladder with colostomy and urinary tract diversion. Histopathological findings showed the presence of a colovesical fistula due to extramurally growing colon cancer. Around the colon cancer, the normal colon mucosa was depressed sharply with lack of the muscular layer, suggesting that the colon cancer was arising from a colon diverticulum.
Conclusion
The present case is the first report of sigmoid colon cancer arising in a diverticulum with involvement of the urinary bladder. Due to an accurate preoperative radiological diagnosis, we were able to successfully perform a curative resection for sigmoid colon cancer arising in a diverticulum with involvement of the urinary bladder.
doi:10.1186/1471-230X-14-90
PMCID: PMC4030041  PMID: 24884743
Colon cancer; Diverticulum; Urinary bladder invasion
8.  Severe cholestatic jaundice after a single administration of ajmaline; a case report and review of the literature 
BMC Gastroenterology  2014;14:60.
Background
Ajmaline is a pharmaceutical agent now administered globally for a variety of indications, particularly investigation of suspected Brugada syndrome. There have been previous reports suggesting that repetitive use of this agent may cause severe liver injury, but little evidence exists demonstrating the same effect after only a single administration.
Case presentation
A 33-year-old man of Libyan origin with no significant past medical history underwent an ajmaline provocation test for investigation of suspected Brugada syndrome. Three weeks later, he presented with painless cholestatic jaundice which peaked in severity at eleven weeks after the test. Blood tests confirmed no evidence of autoimmune or viral liver disease, whilst imaging confirmed the absence of biliary tract obstruction. A liver biopsy demonstrated centrilobular cholestasis and focal rosetting of hepatocytes, consistent with a cholestatic drug reaction. Over the course of the next few months, he began to improve clinically and biochemically, with complete resolution by one year post-exposure.
Conclusion
Whilst ajmaline-related hepatotoxicity was well-recognised in the era in which the drug was administered as a regular medication, clinicians should be aware that ajmaline may induce severe cholestatic jaundice even after a single dose administration.
doi:10.1186/1471-230X-14-60
PMCID: PMC3977671  PMID: 24694003
Ajmaline; Drug-induced liver injury; Brugada syndrome; Liver; Pathology
9.  Peritoneal metastatic adenocarcinoma possibly due to a gastric duplication cyst: a case report and literature review 
BMC Gastroenterology  2014;14:48.
Background
Gastric duplication cysts are rare congenital abnormalities, and malignant transformation of these duplications is also thought to be rare.
Case presentation
During a routine health checkup, a 28-year-old man underwent abdominal sonography followed by computed tomography (CT) with contrast agent, which revealed a cystic lesion with no enhancement. Laparoscopic surgery showed a 10 × 10 cm cyst adhering to the gastric corpus. However, attempts to remove the lesion en bloc were unsuccessful, and the ruptured cyst had contaminated the peritoneal cavity. Gastric duplication was diagnosed from microscopic examination of the cyst. Seven months later, the patient suffered a progressive increase in ascites, and repeated cytological analysis showed small nests of adenocarcinoma cells, with primary lesion unknown. Diagnostic laparoscopy showed multiple white nodules scattered over the surface of the liver, greater omentum, and peritoneum. Biopsy of the omental nodules confirmed adenocarcinoma, while carcinomatosis was diagnosed in the peritoneum.
Conclusions
Clinical presentation and chronological developments indicated that the malignancy probably originated from the gastric duplication cyst. This case highlights the importance of accurate preoperative diagnosis and optimal surgical management for gastric duplication as well as considering the potential existence of malignant transformation during surgical evaluation of adult patients with gastric duplication cysts.
doi:10.1186/1471-230X-14-48
PMCID: PMC3994556  PMID: 24641252
10.  Orthotopic liver transplantation in situs inversus adult from an ABO-incompatible donor with situs inversus 
BMC Gastroenterology  2014;14:46.
Background
Situs inversus is a rare congenital anomaly characterized by the complete inversion of thoracic and abdominal organs. Liver transplantation in such patients or from donors in situs inversus is technically challenging because of the reversed anatomic structures. A small number of successful liver transplantation cases concerning situs inverus in either recipients or donors have been recently reported with different graft position and orientation. Here we reported an extremely rare case of liver retransplantation from an ABO incompatible situs inversus donor to an adult situs inversus recipient.
Case presentation
A 53-year-old complete situs inversus man developed graft failure due to severe biliary complication after his first liver transplantation from a situs solitus donor. Re-transplantation was performed using a graft liver from a likewise situs inversus donor. Although the blood type between donor and recipient was incompatible, the post-operative outcome was excellent under proper prophylaxis to the antibody-mediated rejection.
Conclusion
To the best of our knowledge, this is the first report of liver transplantation from situs inversus to situs inversus in adult recipient. Liver transplantation using situs matching donor makes the procedure much easier at the surgical point of view, which has a benefit of less potential surgical complications. Furthermore, ABO-incompatibility is acceptable for donor allocation in cases that both donor and recipient are situs inversus.
doi:10.1186/1471-230X-14-46
PMCID: PMC3975226  PMID: 24625305
Liver transplantation; Retransplantation; Situs inversus; Abo incompatible
11.  Two patients with intestinal failure requiring home parenteral nutrition, a NOD2 mutation and tuberculous lymphadenitis 
BMC Gastroenterology  2014;14:43.
Background
Mutations in the NOD2 gene are a significant risk factor to acquire intestinal failure requiring home parenteral nutrition. Tuberculous lymphadenitis is the main manifestation of extrapulmonary tuberculosis. Defects in the innate immunity, including NOD2 mutations, may increase the risk for acquiring infections caused by M. tuberculosis. An association of intestinal failure, mutations in the NOD2 gene and tuberculous lymphadenitis has not been described before.
Case presentation
We report of two patients with intestinal failure secondary to mesenteric ischemia. Both patients presented with fever and weight loss while receiving long term home parenteral nutrition. Both of them were found to have mutations in the NOD2 gene. Catheter related infections were ruled out. FDG-PET-CT scans initially obtained in search for another infectious focus that would explain the symptoms unexpectedly showed high FDG uptake in mediastinal lymph nodes. Direct or indirect evidence proved or was highly suggestive for tuberculous lymphadenitis. Intravenous tuberculostatic therapy was started and led to a reversal of symptoms and to resolution of the lesions by FDG-PET-CT.
Conclusion
Mutations in the NOD2 gene may put patients both at an increased risk for acquiring M. tuberculosis infections as well as at an increased risk of intestinal failure after extensive intestinal resection. Thus we suggest to specifically include reactivated and opportunistic infections in the differential diagnosis of suspected catheter related infection in patients with intestinal failure who carry mutations in their NOD2 gene.
doi:10.1186/1471-230X-14-43
PMCID: PMC3995967  PMID: 24597572
NOD2; Intestinal failure; Tuberculous lymphadenitis; Catheter related blood stream infection
12.  The concurrent association of inflammatory polymyositis and Crohn’s ileo-colitis in a Sri Lankan man: a case report of a rare association and literature review 
BMC Gastroenterology  2014;14:35.
Background
Crohn’s disease is a relapsing, systemic inflammatory disease affecting the gastrointestinal tract with associated extraintestinal manifestations and immune disorders. Among the few cases reported, the association of Crohn’s disease with polymyositis varies in its complexity and severity. We report here the first known case of inflammatory polymyositis leading to rhabdomyolysis in a male patient diagnosed with Crohn’s ileocolitis.
Case presentation
A 42-year-old previously healthy man presented with acute polymyositis leading to rhabdomyolysis. The acute nature of the illness raised the suspicion of an infective, toxic, or metabolic insult, which was excluded during further investigations. Prolonged low-grade fever and raised inflammatory markers led to the suspicion of inflammatory polymyositis, which was confirmed by electromyography and muscle histology. In the absence of an infective cause, the concurrent association of prolonged diarrhea containing blood and mucous after recovery from an acute phase of myositis proved a diagnostic challenge. Ileocolonoscopy findings of extensive aphthous ulceration with skip lesions extending to the terminal ileum, and histology showing polymorph infiltration of the lamina propria, transmural involvement, and micro abscess formation was suggestive of Crohn’s disease. Sensory motor axonal peripheral neuropathy, which is another rare association of inflammatory bowel disease, was also present.
Conclusion
An unrecognized genetic predisposition or altered gut permeability causing disruption of the gut immune barrier triggering an immune response against skeletal muscles may have contributed to this unique association. Both polymyositis and Crohn’s ileocolitis responded well to corticosteroids and azathioprine, which is supportive of their immune pathogenesis. Myositis can be considered to be a rare extraintestinal manifestation of Crohn’s disease and can be used in the differential diagnosis of corticosteroid or hypokalemia-induced myopathy in Crohn’s disease.
doi:10.1186/1471-230X-14-35
PMCID: PMC3938905  PMID: 24552185
Inflammatory polymyositis; Crohn’s disease; Rhabdomyolysis; Ileocolitis
13.  Acute gastric dilatation due to a superior mesenteric artery syndrome: an autopsy case 
BMC Gastroenterology  2014;14:37.
Background
Superior mesenteric artery (SMA) syndrome occurs when the third portion of duodenum becomes tightly compressed between the SMA and the abdominal aorta (AA). Several causes of the SMA syndrome have been postulated such as marked weight loss, external compression of the abdomen, anatomic variation, and surgical alterations of anatomy. This is an autopsy case of a subject with atypical duodenal obstruction related SMA syndrome.
Case presentation
A 71-year-old woman died one and a half days after eating a large meal of roast meat and vegetables and experiencing subsequent nausea and abdominal pain. At autopsy, fatal acute gastric dilatation was confirmed. The posterior parietal peritoneum around the duodenum was scarred and pulled the root of the mesentery involving the SMA. The complex compressed and narrowed the third portion of the duodenum. The root of the mesentery was also thickened and had adhered to the surface of the duodenum, which may have been due to past peritonitis and disturbance of duodenal motility. Aggregation of an excessively large food mass obstructed the lumen of the duodenum. The cause of death was diagnosed as SMA syndrome with intra-duodenal aggregation of an excessively large mass of food in the narrowed duodenal lumen.
Conclusion
This is an atypical fatal case of acute gastric dilatation, through an excessively large mass of food obstruction at the latent narrowed duodenum.
doi:10.1186/1471-230X-14-37
PMCID: PMC3946598  PMID: 24555911
Superior mesenteric artery; Duodenum; Mesentery; Scar; Food mass
14.  Ulcerative jejunitis in a child with celiac disease 
BMC Gastroenterology  2014;14:29.
Background
Celiac disease can present in children and adults with a variety of manifestations including a rare complication known as ulcerative jejunitis. The latter has been associated with refractory celiac disease in adult onset patients. The objective of this case report is to describe the first pediatric case of ulcerative jejunitis in celiac disease, diagnosed by capsule endoscopy, which was not associated with refractory celiac disease.
Case presentation
The 9 year old girl presented with a history of abdominal pain and vomiting. Laboratory investigations revealed a slightly elevated IgA tissue transglutaminase antibody level in the setting of serum IgA deficiency. Initial upper endoscopy with biopsies was not conclusive for celiac disease. Further investigations included positive IgA anti-endomysium antibody, and positive HLA DQ2 typing. Video capsule endoscopy showed delayed appearance of villi until the proximal to mid jejunum and jejunal mucosal ulcerations. Push enteroscopy with biopsies subsequently confirmed the diagnosis of celiac disease and ulcerative jejunitis. Immunohistochemical studies of the intraepithelial lymphocytes and PCR amplification revealed surface expression of CD3 and CD8 and oligoclonal T cell populations. A repeat capsule study and upper endoscopy, 1 year and 4 years following a strict gluten free diet showed endoscopic and histological normalization of the small bowel.
Conclusion
Ulcerative jejunitis in association with celiac disease has never previously been described in children. Capsule endoscopy was essential to both the diagnosis of celiac disease and its associated ulcerative jejunitis. The repeat capsule endoscopy findings, one year following institution of a gluten free diet, also suggest that ulcerative jejunitis is not always associated with refractory celiac disease and does not necessarily dictate a poor outcome.
doi:10.1186/1471-230X-14-29
PMCID: PMC4016221  PMID: 24524552
Ulcerative jejunitis; Celiac disease; Pediatric; Capsule endoscopy
15.  Multiple aseptic splenic abscesses in a 15 year old patient 
BMC Gastroenterology  2014;14:20.
Background
Splenic abscesses in children are rare. In recent years aseptic abscesses have been recognized as a new disease entity, especially in adults.
Case presentation
We present a rare case of a 15 year old girl with aseptic abscesses, in whom antibiotic therapy comprising metronidazole and meropenem was partly beneficial in improving the patient’s clinical condition and inflammatory parameters. Eventually corticosteroid therapy led to complete and long lasting resolution of symptoms. Further diagnostic work-up revealed autoimmune thyroiditis, but no signs of inflammatory bowel disease.
Conclusion
Aseptic splenic abscesses should always prompt clinicians to initiate further diagnostics to determine a potential underlying condition and a regular follow-up. Anaerobic bacteria may play a role in the pathogenesis of the disease and besides corticosteroid treatment antibiotics covering anaerobes may be beneficial.
doi:10.1186/1471-230X-14-20
PMCID: PMC3922344  PMID: 24502393
Abscess; Aseptic; Anaerobic bacteria; Metronidazole; Inflammatory bowel disease
16.  Duodenal mucosal damage is associated with proliferative activity of Brunner’s gland hamartoma: a case report 
BMC Gastroenterology  2014;14:14.
Background
Brunner’s gland hamartoma is a rare tumor, predominantly found in the fifth to sixth decades of life. Generally, it is a single pedunculated polyp, rarely larger than 5 cm. Asymptomatic cases are found incidentally, but cases with a large polyp tend to have gastrointestinal bleeding and/or obstructive symptoms. Polyp size increases in a time-dependent manner, however, the growth mechanism is unknown. We report a Japanese male case in his mid-twenties with an over 6 cm sized polyp.
Case presentation
A 26-year-old man presented black stools and anemia. Endoscopic examination revealed a large pedunculated polyp at gastroduodenal junction. The polyp, subsequently resected by distal gastrectomy, was lobulated with random surface erosions and sized 6.4 × 3 cm. Histological examination revealed that the polyp arose from duodenal mucosa and was composed of hyperplastic Brunner’s glands in lobules separated by fibromuscular septa, associated with lymphocytic infiltrate and lymphoid follicles. No evidence of malignancy was found. Thus, the lesion was diagnosed as Brunner’s gland hamartoma. Further immunohistochemical studies indicated that gastric foveolar metaplasia is associated with surface epithelium covering upper two thirds of the polyp, showing immunohistochemical positivity for mucin 5 AC (MUC5AC). Below the metaplastic surface epithelium, Brunner’s glands had high proliferative activity (MIB-1 labeling index: 7.9%). The similar staining pattern was observed at surface erosive sites (MIB-1 labeling index in Brunner’s glands: 9%). On the other hand, surface epithelium in the lower side of the polyp still preserved intestinal nature, containing CDX2-positive nuclei and MUC2-positive goblet cells. Brunner’s glands below the surface epithelium with intestinal characteristics showed low proliferative activity (MIB-1 labeling index: 0.77%).
Conclusion
Proliferative activity of Brunner’s glands was high at the sites with surface erosion and also below the epithelium showing gastric foveolar metaplasia. As gastric foveolar metaplasia occurs along with a mucosal repair process in the duodenum, mucosal damages underlay the hamartomatous proliferation of Brunner’s glands and eventually resulted in a formation of large polypoid mass in this case.
doi:10.1186/1471-230X-14-14
PMCID: PMC3898372  PMID: 24422755
Brunner’s gland hamartoma; Brunner’s glands; Gastric foveolar metaplasia; MUC5AC; MIB-1; Mucosal damage
17.  Concurrent autoimmune pancreatitis and primary Biliary cirrhosis: a rare case report and literature review 
BMC Gastroenterology  2014;14:10.
Background
Both autoimmune pancreatitis (AIP) and primary biliary cirrhosis (PBC) are related to various diseases. But the concurrence of AIP and PBC is extremely rare, with only 2 cases reported. Here we report the concurrence of AIP and PBC in a Chinese patient for the first time.
Case presentation
A 65-year-old male was admitted to our hospital with jaundice, pruritus, mild abdominal pain and darkening urine. Serum alkaline phosphatase, γ-glutamyltransferase, bilirubin and IgG4 were prominently elevated. The antimitochondrial antibody was positive. Radiological examination revealed diffusive enlargement of the pancreas. Pancreatic biopsy showed lymphoplasmacytic infiltration, fibrosis and abundant IgG4+ plasma cells. The patient was diagnosed with AIP and PBC. Nasobiliary tube was placed to facilitate biliary drainage. A combination therapy of steroid and UDCA was administered and the patient was gradually recovered, during which the patient was complicated with biliary infecion, herpes zoster and pulmonary abscess.
Conclusion
We present this case together with literature evidence to support the concurrence of AIP and PBC, share our experience of using combination therapy with steroid and UDCA, and raise the awareness of infectious complications in immunosuppressed patients.
doi:10.1186/1471-230X-14-10
PMCID: PMC3897989  PMID: 24410827
Autoimmune pancreatitis; Primary biliary cirrhosis; Steroid; Ursodeoxycholic acid; Infection
18.  Osteonecrosis of the jaw in a Crohn’s disease patient following a course of Bisphosphonate and Adalimumab therapy: a case report 
BMC Gastroenterology  2014;14:6.
Background
Bisphosphonates have a widespread indication for osteoporosis and are also applied in cancer patients with skeletal-related conditions. Bisphosphonate-associated osteonecrosis of the jaw (BRONJ) is a feared side effect which is hard to treat and often affects patient´s quality of life in an extensive manner. Adalimumab (Humira®), a fully human recombinant antibody specific for tumor necrosis factor- α, is approved for treatment in patients with Inflammatory Bowel Disease like ulcerative colitis or Crohn’s disease.
Case presentation
In March 2013, a 36-year-old female presented with right-sided perimandibular swelling, recurrent facial pain and exposed necrotic bone after previous extraction of tooth 47. She had the medical history of Crohn’s disease for more than one decade with chronic active enterocolitis, fistula disease as well as previous oral manifestation and was currently treated with Adalimumab since September 2008. Due to steroid-induced osteoporosis, diagnosed in 2004, she received oral Bisphosphonates (Risedronate) from 2004 until 2007 followed by two infusions of Zoledronic acid in 2008 and 2009.
Conclusion
This patient with a medical history of Crohn’s disease and gastrointestinal remission under Adalimumab therapy presented with osteonecrosis of the jaw after suspended oral and intravenous Bisphosphonate therapy implicating that the biologic therapy with an anti-TNF-α antibody might promote the manifestation of osteonecrosis and compromise oral healing capacity.
doi:10.1186/1471-230X-14-6
PMCID: PMC3890650  PMID: 24400722
Osteonecrosis of the jaw; Bisphosphonate; Adalimumab; Crohn’s disease
19.  Allopurinol use in pregnancy in three women with inflammatory bowel disease: safety and outcomes: a case series 
BMC Gastroenterology  2013;13:172.
Background
Allopurinol is a frequently prescribed drug. In inflammatory bowel disease patients who shunt thiopurine metabolism towards more toxic and less desirable pathways, allopurinol is proving to be an effective add on therapy with good resultant disease control and less treatment side effects. As many such patients are young, the potential for pregnant women to be exposed to allopurinol is increasing. The safety of allopurinol in pregnancy is not known however.
Case presentation
We report three cases of safe use of allopurinol in pregnancy for women with inflammatory bowel disease. This included 2 patients with ulcerative colitis and 1 patient with fistulising Crohn’s disease. Allopurinol was used throughout pregnancy in all patients. All 3 pregnancies resulted in normal healthy babies born at term by Caesarean section.
Conclusion
It is important to evaluate and document the safety of allopurinol during pregnancy, as it is finding new roles in young patients. These three cases add significantly to the very limited data on allopurinol use in pregnancy. We encourage reporting of all cases of allopurinol use in pregnant patients and suggest an allopurinol pregnancy registry to document drug exposures and outcomes.
doi:10.1186/1471-230X-13-172
PMCID: PMC3878501  PMID: 24345189
Allopurinol; Pregnancy; Teratogen; Inflammatory bowel disease; Ulcerative colitis; Azathioprine; 6-mercaptopurine
20.  Combination therapy of infliximab and thalidomide for refractory entero-Behcet's disease: a case report 
BMC Gastroenterology  2013;13:167.
Background
Behcet's disease (BD) is a systemic inflammatory disease with the histopathological features of leukocytoclastic vasculitis that affects nearly all organs and systems. When it involves the intestine, it is called entero-Behcet's disease (entero-BD).
Case presentation
Here we described a 23-year-old man with entero-BD refractory to conventional therapies who responded well to the combination therapy of infliximab, an anti-tumor-necrosis-factor (TNF)-alpha antibody, and thalidomide. After combination treatment, the patient’s symptoms improved greatly and his Crohn's Disease Activity Index (CDAI) score decreased from 344 to 52 points, accompanied by a body weight increase from 53 kg to 64 kg. A follow-up endoscopy performed 10 weeks after the treatment showed significant improvement and the patient's multiple ulcers had healed well.
Conclusion
The combination therapy of infliximab and thalidomide appears to be clinically effective in a patient with refractory entero-BD. However, further studies need to be performed to evaluate the efficacy of this combination therapy.
doi:10.1186/1471-230X-13-167
PMCID: PMC4029173  PMID: 24321021
Entero-Behcet's disease; Infliximab; Thalidomide
21.  Pyoderma gangrenosum in refractory celiac disease: a case report 
BMC Gastroenterology  2013;13:162.
Background
Pyoderma gangrenosum is an inflammatory neutrophilic dermatosis characterized by painful cutaneous ulcerations and often associated with systemic inflammatory and neoplastic diseases. Here we report the first case of pyoderma gangrenosum in a patient with refractory celiac disease.
Case presentation
A 52-year-old woman with a previously diagnosed refractory celiac disease resistant to steroids and immunosuppressive drugs presented to our hospital for a rapidly growing, painful inflammatory skin lesion of the left leg. Physical examination revealed a painful lesion with focal ulceration, necrosis and pus discharge with active inflammatory borders at the external part of the left leg. Histological evaluation of a skin biopsy and analysis of inflammatory cytokines and matrix-degrading proteases in lesional skin samples confirmed the clinical suspicion of pyoderma gangrenosum. Treatment with oral prednisone was rapidly followed by a complete healing of the skin lesion but no improvement of symptoms/signs of malabsorption.
Conclusion
Treatment of the patient with systemic steroids healed the skin lesion without improving the underlying refractory celiac disease. This observation raises the possibility that refractory celiac disease and pyoderma gangrenosum may be immunologically different.
doi:10.1186/1471-230X-13-162
PMCID: PMC4222694  PMID: 24279608
Gluten; Cutaneous ulcers; Celiac disease; Pyoderma gangrenosum
22.  Annular pancreas concurrent with pancreaticobiliary maljunction presented with symptoms until adult age: case report with comparative data on pediatric cases 
BMC Gastroenterology  2013;13:153.
Background
Annular pancreas (AP) concurrent with pancreaticobiliary maljunction (PBMJ), an unusual coexisted congenital anomaly, often presented symptoms and subjected surgical treatment at the early age of life. We reported the first adult case of concurrent AP with PBMJ presented with symptoms until his twenties, and performed a literature review to analyze the clinicopathological features of such cases comparing with its pediatric counterpart.
Case presentation
The main clinical features of this case were abdominal pain and increased levels of plasma amylase as well as liver function test. A complete type of annular pancreas with duodenal stenosis was found, and dilated common bile duct with high confluence of pancreaticobiliary ducts was also observed. Meanwhile, extremely high levels of bile amylase were detected both in common bile duct and gallbladder. The patient received duodenojejunostomy (side-to-side anastomosis) as well as choledochojejunostomy (Roux-en-Y anastomosis), adnd was discharged in a good condition.
Conclusion
AP concurrent with PBMJ usually presents as duodenal obstruction in infancy, while manifests as pancreatitis in adulthood. Careful long-term follow-up is required for children with AP considering its association with PBMJ which would induce various intractable pathologic conditions in the biliary tract and pancreas.
doi:10.1186/1471-230X-13-153
PMCID: PMC4015270  PMID: 24156788
Annular pancreas; Pancreaticobiliary maljunction; Pancreatitis
23.  A therapeutic barium enema is a practical option to control bleeding from the appendix 
BMC Gastroenterology  2013;13:152.
Background
Acute lower gastrointestinal hemorrhage originating from the appendix is rare and often intractable, because it is almost impossible to approach the bleeding point by endoscopy. We herein describe the first case of bleeding from the appendix, which was successively controlled by a therapeutic barium enema administered into the appendix.
Case presentation
A 71-year-old male visited our hospital because of melena. He has been receiving an anti-coagulation drug, ticlopidine hydrochloride, for 10 years. By an emergency colonoscopy, a hemorrhage was detected in the appendix, and the lesion responsible for the bleeding was regarded to exist in the appendix. Two hundred milliliters of 50 W/V% barium was sprayed into the orifice of the appendix using a spraying tube. The bleeding could thus be immediately stopped, and a radiological examination revealed the accumulation of barium at the cecum and the orifice of the appendix. The barium accumulation disappeared by the next day, and no obvious anal bleeding was observed. Two weeks after stopping the bleeding from the appendix, an appendectomy was performed to prevent any further refractory hemorrhaging. The patient has had no complaints of any abdominal symptoms or anal bleeding for 10 months.
Conclusions
A therapeutic barium enema is a useful procedure to control bleeding from the appendix and to avoid emergency surgery, such as partial cecectomy and hemicolectomy.
doi:10.1186/1471-230X-13-152
PMCID: PMC4020223  PMID: 24156777
Appendix bleeding; Barium enema; Intestinal hemorrhage; Appendicitis
24.  Penile metastasis from primary cholangiocarcinoma: the first case report 
BMC Gastroenterology  2013;13:149.
Background
Metastatic penile carcinoma derived from cholangiocarcinoma (CCA) has not been previously reported in the literature. Common metastatic sites for CCA include the regional lymph nodes and adjacent organs. CCAs are not highly vascularised tumours, making hematogenous metastases uncommon. Hematogenous CCA metastases commonly occur at distant organs such as the lungs, adrenal glands, and bones. Median survival for patients with metastatic disease is generally less than 1 year.
Case presentation
A 74-year-old Caucasian man consulted us after having undergone penile ultrasonography for pain and increased thickness at the base of the penis after self-examination. The patient presented with a history of hepatitis C-related cirrhosis and intrahepatic CCA, diagnosed 3 years previously. A biopsy of the corpora cavernosa on both sides revealed a carcinoma harbouring the same histological and immunophenotypical features as the primary hepatic lesion.
Conclusions
To date, there is no case of penile or urogenital system metastasis from CCA described in the literature. Therefore, this article represents the first case report of penile metastasis from CCA.
doi:10.1186/1471-230X-13-149
PMCID: PMC3854009  PMID: 24124668
Cholangiocarcinoma; Elastography; Magnetic resonance; Penile metastases
25.  A case of rectal tumor in which the shape altered with regression in short period 
BMC Gastroenterology  2013;13:146.
Background
Histological regression of solid tumors in adults receiving no treatment is rare. Specifically, spontaneous partial and complete regression of colorectal cancers account for less than 2% of such cases and those without metastasis are exceedingly rare.
Case presentation
A 60-year-old male underwent total colonoscopy following a positive fecal occult blood test at the referring hospital. A flat elevated lesion with central reddish depression, 10 mm in diameter, was detected in the lower rectum. Biopsy results from the referring hospital showed a well-differentiated adenocarcinoma and the patient was referred to our hospital for diagnosis and treatment. Preoperative colonoscopy was performed to determine the therapeutic strategy; however, we found only scar tissue and there were no endoscopic features to suggest malignancy. Biopsy from the scar revealed normal rectal mucosa and we performed diagnostic endoscopic submucosal resection with a ligation device (ESMR-L) one week later. The resected specimen showed a 1 mm well-differentiated adenocarcinoma with low-grade atypia and no lymphovascular invasion. The macroscopic type was 0-IIb, the depth of invasion was intramucosal, and the vertical and lateral margins were negative. There has been no evidence of recurrence for 18 months following treatment.
Conclusion
We report a case of a rectal tumor showing regression over a short period without treatment. Spontaneous regression of malignant tumors is a rare and unexplained phenomenon. Further research and understanding of the mechanism holds the key for treatment and prevention of cancer in the future.
doi:10.1186/1471-230X-13-146
PMCID: PMC3850788  PMID: 24090181
Rectal tumor; Regression; Endoscopic submucosal resection with a ligation device (ESMR-L)

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