Catarina Graça Rodrigues

Can Red Cell Distribution Width Be Used as a Marker of Crohn's Disease Activity?

Introduction Recently, it has been suggested an association between red cell distribution width (RDW) and Crohns disease activity index (CDAI), but its use is not yet performed in daily clinical practice. Objectives To determine whether RDW can be used as a marker of Crohns disease (CD) activity. Methods This was a cross-sectional study including patients with CD, observed consecutively in an outpatient setting between January 1st and September 30th 2013. Blood cell indices, erythrocyte sedimentation rate (ESR), and C-reactive protein were measured. CD activity was determined by CDAI (active disease if CDAI ≥ 150). Associations were analyzed using logistic regression (SPSS version 20). Results 119 patients (56% female) were included in the study with a mean age of 47 years (SD 15.2). Twenty patients (17%) had active disease. The median RDW was 14.0 (13–15). There was an association between RDW and disease activity (p = 0.044). After adjustment for age and gender, this association remained consistent (OR 1.20, 95% CI 1.03–1.39, p = 0.016). It was also found that the association between RDW and disease activity was independent of hemoglobin and ESR (OR 1.36, 95% CI 1.08–1.72, p = 0.01) and of biologic therapy (OR 1.19, 95% CI 1.03–1.37, p = 0.017). A RDW cutoff of 16% had a specificity and negative predictive value for CDAI ≥ 150 of 88% and 86%, respectively. Conclusion In this study, RDW proved to be an independent and relatively specific marker of CD activity. These results may contribute to the implementation of this simple parameter, in clinical practice, aiming to help therapeutic decisions.

Is there a proximal shift in the distribution of colorectal adenomas

Introduction Several studies have shown a proximal shift of colorectal cancer (CRC) during the last decades. However, few have analyzed the changing distribution of adenomas over time. Aim The aim of this study was to compare the site and the characteristics of colorectal adenomas, in a single center, during two periods. Methods We conducted a retrospective, observational study in a single hospital of adenomas removed during a total colonoscopy in two one-year periods: 2003 (period 1) and 2012 (period 2). Patients with inflammatory bowel disease, familial adenomatous polyposis, hereditary non-polyposis colorectal cancer syndrome, or history of CRC were excluded from the study. The χ2 statistical test was performed. P values less than 0.05 were considered statistically significant. Results During the two considered periods, a total of 864 adenomas from 2394 complete colonoscopies were analyzed: 333 adenomas from 998 colonoscopies during period 1 and 531 adenomas from 1396 colonoscopies during period 2. There was a significant increase in the proportion of adenomatous polyps in the proximal colon from period 1 to 2 (30.6% to 38.8% (p = 0.015)). Comparing the advanced features of adenomas between the two periods, it was noted that in period 2, the number of adenomas with size ≥1 cm (p = 0.001), high-grade dysplasia (p = 0.001), and villous features (p < 0.0001) had a significant increase compared to period 1. Conclusion Incidence of adenomatous polyps in the proximal colon as well as adenomas with advanced features has increased in the last years. This finding may have important implications regarding methods of CRC screening.

Actinomycosis Causing Recurrent Perianal Fistulae

Actinomycosis is a rare but easily curable infection that should be considered in the differential diagnosis of perianal fistulizing disease. We present the case of a 26-year-old woman with complex perianal fistulae, including trans-sphincteric and suprasphincteric fistulous tracts and a rectovaginal fistula, requiring multiple abscess drainages, seton placement, and antibiotic courses, with little improvement. After extensive investigation, Actinomyces spp. was identified in anal cytology. The patient underwent a 6-week course of intravenous penicillin followed by oral amoxicillin, with remarkable improvement. This case illustrates the importance of pursuing less common diagnoses in refractory complex perianal disease, such as actinomycosis.

Vitamin D Deficiency in a Portuguese Cohort of Patients with Inflammatory Bowel Disease: Prevalence and Relation to Disease Activity

Background and Aims: Vitamin D deficiency is more common in inflammatory bowel disease (IBD) patients than in the general population. However, there are conflicting data about predictive factors of vitamin D deficiency and its potential association with disease activity. The aims of this study were to determine the prevalence and predictive factors of vitamin D deficiency and to evaluate a possible association with disease activity. Methods: A prospective observational study was conducted, including patients with IBD from January to July 2016. The Endocrine Society guidelines were considered for defining levels of serum 25-hydroxyvitamin D (25-OH-D) as follows: deficient (< 20 ng/mL, < 10 ng/mL being severe deficiency), insufficient (21–29 ng/mL), and adequate (> 30 ng/mL). Results: A total of 152 patients (52% men; 47.2 ± 17.3 years) were included, of whom 70% had Crohn’s disease (CD). Thirty-seven percent of patients were on immunosuppressors and 17% were on biologics. The majority were outpatients (88.2%). Mean 25-OH-D levels were 17.1 ± 8 ng/mL (CD: 16.7 ± 8 ng/mL vs. ulcerative colitis: 17.6 ± 7 ng/mL, p = 0.1). Inadequate levels were present in 90.8% of patients (deficiency: 68.4%; insufficiency: 22.4%). A significant negative correlation between 25-OH-D levels and age (r = –0.2, p = 0.04), C-reactive protein (CRP) levels (r = –0.22, p = 0.004), and Harvey-Bradshaw index (HBi) (r = –0.32, p = 0.001) was found. Patients with severe deficiency showed a higher CRP (0.6 vs. 1.4 mg/dL, p = 0.03), erythrocyte sedimentation rate (ESR) (22 vs. 31 mm/h, p = 0.03), and HBi (2 vs. 5, p < 0.001) and lower hemoglobin (13.6 vs. 12.7 g/dL, p = 0.02). There was no association between vitamin D deficiency and gender, type, extent, and duration of disease, surgery, and other measures of disease activity, such as ESR, hemoglobin (these 2 items except for severe deficiency), fecal calprotectin, or Truelove and Witts classification. Conclusions: There is a high prevalence of inadequate levels of vitamin D in IBD patients, particularly deficiency (68.4%). There seems to exist an association between lower levels of vitamin D and higher disease activity, especially in CD.

Correction: Pancreatoscopy-guided laser lithotripsy in a patient with difficult ductal stone

CORRECTION Alexandrino G, Lourenço L, Rodrigues CG et al. Pancreatoscopy-guided laser lithotripsy in a patient with difficult ductal stone. Endoscopy 2018, doi:10.1055/a-0574-2278 In the above mentioned article one author was missing in the authors’ list. Correct is: Gonçalo Alexandrino, Luís Lourenço, Catarina G. Rodrigues, David Horta, Jorge Reis, Jorge Canena. This was corrected in the online version on April 6, 2018.

Pancreatoscopy-guided laser lithotripsy in a patient with difficult ductal stone

A 63-year-old man with heavy alcohol consumption was referred to our institution for upper abdominal pain, weight loss, and a computed tomography scan showing signs of chronic pancreatitis (parenchymal calcifications and atrophy of the pancreatic body/tail). A magnetic resonance cholangiopancreatography was performed, showing Wirsung dilation, namely of the tail, and an abrupt stop in the pancreatic body of unknown cause (▶Fig. 1). Endoscopic ultrasound revealed an intraductal stone in the pancreatic body (7.4mm), and a dilated Wirsung in the body and tail (▶Fig. 2). The patient underwent endoscopic retrograde cholangiopancreatography (ERCP). Pancreatography showed an irregular Wirsung contour in the head and irregularity in the body–tail transition, suggestive of an intraductal stone (▶Fig. 3). Pancreatic sphincterotomy was performed, and the calculus was crossed with the guidewire, but it was impossible to cross it with a 6-mm dilation balloon. After hydrostatic balloon dilation of the pancreatic head (up to 6mm), a pancreatoscope (Spyglass Direct Visualization System; Boston Scientific, Marlborough, Massachusetts, USA) was advanced over a 0.025-inch guidewire to reach a large intraductal stone of 7–8mm in size (▶Fig. 4, ▶Video1). After targeting the stone, laser bursts (Holmium laser, Auriga XL; Boston Scientific) of less than 5 seconds were delivered through the aqueous medium using a 365-μm diameter fiber (energy level 1200mJ; frequency of 12Hz). After stone fragmentation, ductal clearance was achieved with an 8.5-mm extraction balloon. Two pancreatic stents (12 cm, 7 Fr) were placed. ▶ Fig. 1 Magnetic resonance cholangiopancreatography, showing an abrupt stop at the pancreatic body, followed by major dilation of the remaining body and tail. ▶ Fig. 2 Endoscopic ultrasound revealed a large intraductal stone in the pancreatic body (7.4mm), and a dilated Wirsung in the body and tail.

A Complex Case of Cholestasis in a Patient with ABCB4 and ABCB11 Mutations

The low-phospholipid-associated cholelithiasis (LPAC) syndrome is a form of symptomatic cholelithiasis occurring in young adults, characterized by recurrence of symptoms after cholecystectomy and presence of hepatolithiasis. The case refers to a healthy 39-year-old Caucasian male who presented with abdominal pain and jaundice. His blood tests showed conjugated hyperbilirubinemia and elevated liver enzymes (total bilirubin 6.65 mg/dL, γ-glutamyltransferase 699 IU/L) and abdominal computed tomography revealed dilation of common bile duct and left intrahepatic ducts. Magnetic resonance cholangiopancreatography identified choledocholithiasis, retrieved by endoscopic retrograde cholangiopancreatography, after which there was a worsening of jaundice (total bilirubin 23 mg/dL), which persisted for several weeks, possibly due to ciprofloxacin toxicity. After an extensive workup including liver biopsy, the identification of two foci of hepatolithiasis on reevaluation abdominal ultrasound raised the hypothesis of LPAC syndrome and the patient was started on ursodeoxycholic acid, with remarkable improvement. Genetic testing identified the mutation c.1954A>G (p.Arg652Gly) in ABCB4 gene (homozygous) and c.1331T>C (p.Val444Ala) in ABCB11 gene (heterozygous). In conclusion, we describe the unique case of an adult male with choledocholithiasis, hepatolithiasis, and persistent conjugated hyperbilirubinemia after retrieval of stones, fulfilling the criteria for LPAC syndrome and with possible superimposed drug-induced liver injury, in whom ABCB4 and ABCB11 mutations were found, both of which had not been previously described in association with LPAC.

Pseudocholangiocarcinoma Sign: Management of Portal Cavernoma Biliopathy with Fully-Covered Self-Expandable Metal Stent

Benign bile duct strictures are commonly caused by stones, bile duct injury, sclerosing cholangitis, chronic pancreatitis, and congenital abnormalities. Bile duct changes caused by portal hypertension represent another group of uncommon causes of benign stricture. The earliest evidence of portal biliopathy was described by Hunt in 1965. Portal cavernoma biliopathy (PCB) is a rare condition caused by cavernomatous transformation of an extra-hepatic portal vein thrombosis (EHPVT). It is rarely symptomatic; however, due to compression of the biliary tree, it can result in biliary obstruction, choledocholithiasis, jaundice, and cholangitis. Endoscopic therapy for symptomatic PCB is a matter of discussion since this condition is rare. We present a rare case of symptomatic PCB with a common bile duct (CBD) stricture, refractory to conventional endoscopic therapy in a patient unfit for surgery. Owing to the risk of repeated plastic stent exchange, we chose a more definite endoscopic approach with temporary placement of fully-covered self-expandable metal stent (fcSEMS). In this paper, we also report our successful results following application of fcSEMS. An 82-year-old man presented to our hospital with fever, right-upper quadrant abdominal pain, and jaundice. He had undergone cholecystectomy at another hospital for acute cholecystitis in 2002, complicated with an EHPVT. In addition, he had a history of aortic stenosis, hypertension, and congestive heart failure. His laboratory values were indicative of leukocytosis (16.700/uL) and elevated liver enzymes, with a total bilirubin of 8.9 mg/dL. Abdominal ultrasound revealed dilated intrahepatic bile ducts (IHBD) and CBD, as well as an EHPVT. The patient was diagnosed with acute cholangitis and treated with antibiotics. Endoscopic retrograde cholangio-pancreatography (ERCP) showed asymmetric stenosis in the middle portion of the CBD (compatible with portal biliopathy type I) and some small stones above it, with dilation of the proximal CBD and IHBD (Fig. 1A). After sphincterotomy and careful stone extraction with Dormia’s basket, a 10-Fr plastic stent was placed. Balloon dilation was avoided due to hemobilia risk, which has been reported before. Abdominal computed tomography (CT) scan with contrast revealed a portal cavernoma compressing the middle segment of the CBD and causing the stricture seen during ERCP (Fig. 2). The patient was not a candidate for portal decompression (transjugular portosystemic shunt was not feasible and he was unfit for surgery due to his advanced age and comorbidities), so progressive biliary plastic stenting was performed every 3 months. However, after one year, there was no improvement in the CBD stenosis and the patient still had elevated liver enzymes and IHBD dilation. Following failure of progressive plastic stenting, temporary placement of a 80×10-mm fcSEMS (Wallflex; Boston Scientific, Marlborough, MA, USA) was planned. The stent was deployed (Fig. 1B) and removed 6 months later (thereafter) Received: July 25, 2016 Revised: October 12, 2016 Accepted: October 27, 2016 Correspondence: Luís Carvalho Lourenço Department of Gastroenterology, Hospital Professor Doutor Fernando Fonseca, IC 19, 2720-276, Amadora, Lisbon, Portugal Tel: +351-91-829-3978, Fax: +21-434-55-66, E-mail: luisclourenco@gmail.com

Pseudomyxoma peritonei: a clinical case of this poorly understood condition.

Background Pseudomyxoma peritonei is an uncommon condition with an estimated incidence of one to two per million (worldwide) per year. It is characterized by the peritoneal deposition of mucinous tumors, most commonly of the appendix, and occasionally from the ovary, coupled by mucinous ascites. Case presentation We report the case of a 76-year-old woman who presented with increased abdominal girth and dyspnea for 2 weeks. She was diagnosed as a case of pseudomyxoma peritonei. She was submitted to right oophorectomy, omentectomy, and pseudomyxoma debulking. The histology was compatible with a mucinous tumor of colorectal/appendicular origin. Chemotherapy was not administered because of her functional status. Two years and 8 months later, she refers with postprandial fullness and has moderate ascites.

Severe Odynophagia and Rapidly Progressive Dysphagia as an Unusual Presentation of Eosinophilic Gastroenteritis

A50-year-old asthmatic woman presented to the emergency department with a 2-week history of severe odynophagia, rapidly progressive dysphagia for solids, and abdominal distention, without fever, abdominal pain, or weight loss. The patient experienced several episodes of mild and self-limited odynophagia in the past year. She denied caustic ingestion, alcohol consumption, or taking any medications other than inhaled steroids and desloratadine. Physical examination was unremarkable except for slightly distended abdomen. Blood tests showed leukocytosis (15,400/mL), eosinophilia (5800/mL), mild elevation of C-reactive protein (4.85 mg/dL), and normal albumin. Computed tomography scan revealed marked circumferential wall thickening of the lower two-thirds of the esophagus and jejunum, minimal right-sided pleural effusion, and mesenteric lymphadenopathy (Figures A and B). Endoscopic findings were normal, but histologic examination demonstrated an intense eosinophilic infiltration (>20 eosinophils/high-power field) in mucosal biopsies from the esophagus (Figure C), stomach, and duodenum (Figure D). Levels of serum immunoglobulin E and erythrocyte sedimentation rate were normal. The symptoms got progressively worse, and the peripheral eosinophil count rose above 8900/mL. Because of the lack of evidence of extragastrointestinal disease and after exclusion of parasitic disease (absence of epidemiologic context, negative stool ova and parasites test, negative serologies for Strongyloides stercoralis and Toxocara canis), we established the diagnosis of eosinophilic gastroenteritis (EG) and started a course of oral