Cholangiocarcinoma: an endoscopist’s perspective

The incidence of cholangiocarcinoma is on the rise. This may be related in part to higher detection rates secondary to sophisticated endoscopic modalities. These tumors pose a significant diagnostic dilemma. High index of suspicion, careful interpretation of serum markers and utilization of endoscopic techniques, including endoscopic retrograde cholangiopancreatography and endoscopic ultrasound, help to establish the diagnosis. Imaging modalities are crucial in the evaluation of these tumors. They help to define the extent of the native lesion, which may dictate its resectability, as well as evaluate for metastasis. Therapeutic options are somewhat limited, short of surgical resection. Newer chemotherapeutic agents, as well as endoscopy-targeted therapy, may improve the overall treatment success rate, although experience is somewhat limited at this time. Endosocpic intervention is essential for palliation.     

Muehrcke’s lines in an adolescent boy with Kawasaki disease

Clinical Rheumatology -     

The endoscopist’s role in the diagnosis and management of pancreatic cancer

Pancreatic cancer remains one of the most lethal malignancies with little improvement in survival over the past several decades in spite of advances in imaging, risk factor identification, surgical technique and chemotherapy. This disappointing outcome is mainly due to failures to make an early diagnosis. In fact, the majority of the patients present with inoperable advanced stages of the disease. Though some of the new tumor markers are promising, we are still in search of the one that has a high sensitivity and accuracy, yet is inexpensive and easy to obtain. The paradigm of management has shifted from up-front surgery followed by adjuvant chemotherapy to neoadjuvant chemoradiation followed by surgery, especially for borderline resectable cancers and even for some resectable cancers. In this article, we will critically assess the limitations of tumor markers and review the advancements in endoscopic techniques in the management of pancreatic cancer.     

Long-term response rates to infliximab therapy for Crohn’s disease in an outpatient cohort

BACKGROUND:

Infliximab’s efficacy in the induction and maintenance of remission in luminal Crohn’s disease has been confirmed by randomized, controlled trials. Less clearly described are long-term outcomes in the clinical practice setting since the establishment of regularly scheduled, every eight-week maintenance infliximab infusions. Existing reports describing clinical practice outcomes are limited by short durations of follow-up or by the use of episodic dosing, or focus on safety data rather than clinical outcomes.

OBJECTIVE:

To examine induction and maintenance responses to infliximab in an outpatient inflammatory bowel disease clinic.

METHODS:

A retrospective chart review was performed. Clinical outcomes were infliximab induction and maintenance responses, defined as the ability to stop and remain off corticosteroids while not requiring additional therapy for active disease.

RESULTS:

One hundred thirty-three patients were identified with records sufficiently detailed to be analyzed. Of these, 117 patients (88%) demonstrated a clinical response to induction; 104 of 117 (89%) were on concomitant immunosuppressive therapy; 80 of 104 on azathioprine/6-mercaptopurine (77%); and 24 of 104 on methotrexate (23%). The mean duration of clinical response was 94 weeks (95% CI 78.8 to 109.2). The proportion of patients who maintained response at 30 weeks was 83.2%, at 54 weeks was 63.6% and at 108 weeks was 44.9%. Adverse events occurred for 15 of 117 patients (12.8%), consisting of nine infusion reactions, four serum sickness-like reactions, one rash and one infection.

CONCLUSION:

Patients treated with infliximab therapy for luminal Crohn’s disease in our outpatient clinic achieved excellent induction and maintenance of response rates, confirming the real-life efficacy of maintenance infliximab established in clinical trials.     

Carrier detection in severe von Willebrand’s disease

Von Willebrands disease (vWD) is an inherited bleeding disorder, caused by a defect of von Willebrands factor (vWF), a multimeric high molecular weight glycoprotein. It is sub-characterised into types 1, 2 and 3 vWD depending on quantitative or qualitative defect of vWF. Prenatal diagnosis and carrier detection are often sought by families affected by type 3 vWD. Five diagnosed index patients with severe type 3 vWD and their family members were the subjects of this study. Two markers (vWF 1 and vWF 2) were used in the polymorphic region of ATCT repeats (variable number of tandem repeat, VNTR) within intron 40 of the vWF gene. The study was done to determine the allelic frequency of these markers in normal Indians and to identify the carriers in type 3 vWD in India. In the five families studied by both markers (vWF 1 and vWF 2), Mendelian inheritance of the VNTR bands were demonstrated, and in all cases the band seen in offspring was present in the parents. Heterozygosity is seen in 78% of normal controls for vWF 1 marker and 80% for vWF 2 marker. Since the heterozygosity frequency of these two markers is high, 70% of carriers of vWD could be successfully detected by this method. It is thus concluded that VNTR gene tracking may be a useful approach for carrier detection in the type 3 vWD in developing countries.     

Automated cytological detection of Barrett’s neoplasia with infrared spectroscopy

Journal of Gastroenterology - Development of a nonendoscopic test for Barrett’s esophagus would revolutionize population screening and surveillance for patients with Barrett’s...     

Linked color imaging improves the visibility of various featured colorectal polyps in an endoscopist’s visibility and color difference value

Purpose

Linked color imaging (LCI) by laser endoscopy is a novel narrow band light observation. In this study, we analyzed the efficacy of LCI for improving the various featured colorectal polyp’s visibility utilizing a subjective endoscopist’s visibility scoring and objective color difference (CD) value.

Methods

We retrospectively reviewed two pictures both with white light (WL) and LCI for 54 consecutive neoplastic polyps 2–20 mm in size. All pictures were evaluated by four endoscopists according to a published polyp visibility score from four (excellent visibility) to one (poor visibility). Additionally, we calculated CD value between each polyp and surrounding mucosa in LCI and WL using an original software.

Results

The mean polyp visibility scores of LCI (3.11 ± 1.05) were significantly higher than those of WL (2.50 ± 1.09, P < 0.001). The ratio of an endoscopist’s poor visibility (polyp visibility scores 1 and 2) was significantly lower in LCI (27.9%) than WL (55.6%, P < 0.001). With respect to the CD analysis, the CD value of LCI was significantly higher than that of WL (33.3 ± 13.9 vs. 20.7 ± 13.6, P < 0.001). In a subgroup analysis, the polyp visibility scores and CD values of LCI about 24 diminutive polyps (≤5 mm) were higher than those of WL (3.29 ± 0.99 vs. 2.12 ± 0.99, P < 0.001; 31.6 ± 12.8 vs. 14.7 ± 7.6, P < 0.001). Additionally, the polyp visibility scores and CD values of LCI for polyps with any location, size, histology, and morphology were significantly higher than those of WL.

Conclusions

LCI improved the various featured polyp’s visibility compared to WL in both polyp visibility scores and CD value.

     

Methotrexate is an efficient therapeutic alternative in children with thiopurine-resistant Crohn’s disease

Abstract

Objective. This study aimed to investigate the role of methotrexate (MTX) in the maintenance of clinical remission and mucosal healing in children with Crohn’s disease (CD), in whom azathioprine (AZA) treatment failed. Materials and methods. This was a retrospective, longitudinal cohort study which included all children who were diagnosed with CD during a period of 10 years and who received MTX for ≥12 months after failed AZA treatment. Remission was assessed clinically, defined by Pediatric Crohn’s Disease Activity Index as a score of ≤10 and no need for the reintroduction of the remission induction therapy. In the subset of patients with sustained clinical remission, the rate of mucosal healing was endoscopically assessed. Endoscopic lesions were assessed by Simple Endoscopic Score for CD. Each patient served as his or her own historical control. Results. Of the 32 included patients, 22 (68.7%) remained in the stable clinical remission after a period of 12 months and 14 (43.8%) did not experience relapse during the whole follow up (median duration 2.9 years; range 1–4.8 years). From all patients who were in clinical remission during the entire follow up (n = 14), endoscopy was performed in eight (57%) patients and showed complete mucosal healing macroscopically (Simple Endoscopic Score for CD score of 0) and microscopically in seven out of eight (87.5%) patients. Conclusion. MTX was found to be an efficient therapeutic alternative in the thiopurine-resistant patients, enabling the complete mucosal healing.     

Abernethy malformation with multiple aneurysms: incidentally found in an adult woman with Caroli’s disease

Abernethy malformation is a rare anomaly with partial or complete congenital absence of the portal vein and subsequent development of extrahepatic portocaval shunts. We present the case of a 28-year-old woman who was incidentally diagnosed with type II Abernethy malformation and multiple aneurysms during an investigation for nonspecific abdominal pain and fever. The patient had been diagnosed with Caroli’s disease at the age of 10 and liver cirrhosis, portal hypertension a few years before. To the best of our knowledge, this is the first case reported with all such congenital anomalies associated together. Ultrasound, computed tomography, including three-dimensional reconstruction, and magnetic resonance imaging were performed which revealed a side-to-side shunt between the extrahepatic portal vein and the inferior vena cava, multiple aneurismal cystic dilation of the spleen artery and left renal artery, and extensive intrahepatic bile duct cysitic dilation with calculus formation. Etiology, clinical significance and management strategies with regard to these abnormalities are discussed.     

Hyperhomocysteinemia in patients with Crohn’s disease

Background

Thromboembolic complications have been reported in patients with Crohn’s disease. Among the contributing factors, hyperhomocysteinemia has been described, although controversial data exist. The aim of our study was to assess the incidence of hyperhomocysteinemia in a nonselected group of patients with Crohn’s disease and to determine whether it might represent a risk marker for thrombosis in such patients.

Methods

Fifty consecutive patients were recruited, and clinical and laboratory variables were compared between those without and those with hyperhomocysteinemia. In the latter, gene mutations in N5-N10-methyltetrahydrofolate reductase were searched for, and clinical and laboratory variables were related to hyperhomocysteinemia. The presence/absence of thrombotic episodes in both groups was determined.

Results

Both groups had similar clinically active disease, with higher C-reactive protein values found in those with hyperhomocysteinemia. Hyperhomocysteinemia was found in 46 % of patients. Of these, 74 % had moderate, 13 % intermediate, and 13 % severe increase in serum homocysteine levels. No relationship was found between homocysteine levels, and age, vitamin B12 levels, folic acid levels, Crohn’s Disease Activity Index score, and CRP values. Gene mutations were found in 5 (22 %) patients, 2 homozygotes and 3 heterozygotes. None of the patients with or without hyperhomocysteinemia had episodes of venous or arterial thrombosis, or stroke.

Conclusions

Hyperhomocysteinemia is frequent in patients with Crohn’s disease, and it could be a cofactor for the pathogenesis of thrombotic episodes.     

Remission with Cabergoline in Adolescent Boys with Cushing’s Disease

Cabergoline is a long-acting dopamine receptor agonist used for treatment of patients with uncured Cushing’s disease (CD) and, as a first-line treatment, was used in only limited numbers of patients. This report presents two adolescent boys with CD who were treated with cabergoline. Two adolescent boys with clinical and laboratory findings of CD are presented. No pituitary adenoma was detected by radiological investigation in either patient. Adrenocorticotropic hormone (ACTH) hypersecretion and lateralization was found by inferior petrosal sinus sampling in both patients. The initial cabergoline dose was 1mg/week and was adjusted up to 1.5 mg/week in the second patient, based on his urinary free cortisol (UFC) level. The patients responded to cabergoline treatment with normal UFC levels on the 4th and 6th months of treatment. The boys reached complete remission at the end of the 17th and 24th months, respectively. Cabergoline is effective in the control of cortisol secretion and can be considered as a first-line treatment in cases of CD. Conflict of interest:None declared.     

Does deep sedation with propofol affect adenoma detection rates in average risk screening colonoscopy exams?

AIM To determine the effect of sedation with propofol on adenoma detection rate(ADR) and cecal intubation rates(CIR) in average risk screening colonoscopies compared to moderate sedation.METHODS We conducted a retrospective chart review of 2604 firsttime average risk screening colonoscopies performed at MD Anderson Cancer Center from 2010-2013. ADR and CIR were calculated in each sedation group. Multivariable regression analysis was performed to adjust for potential confounders of age and body mass index(BMI). RESULTS One-third of the exams were done with propofol(n = 874). Overall ADR in the propofol group was significantly higher than moderate sedation(46.3% vs 41.2%, P = 0.01). After adjustment for age and BMI differences, ADR was similar between the groups. CIR was 99% for all exams. The mean cecal insertion time was shorter among propofol patients(6.9 min vs 8.2 min; P 0.0001).CONCLUSION Deep sedation with propofol for screening colonoscopy did not significantly improve ADR or CIR in our population of average risk patients. While propofol may allow for safer sedation in certain patients(e.g., with sleep apnea), the overall effect on colonoscopy quality metrics is not significant. Given its increased cost, propofol should be used judiciously and without the implicit expectation of a higher quality screening exam.     

Intestinal cancer in patients with Crohn’s disease

Background Surveillance of intestinal cancer in Crohn’s disease (CD) has often been advocated. To date, no clear evidence exists whether CD patients are at special risk for intestinal cancer. An increased incidence of small bowel adenocarcinoma is suggested. However, recent figures also suggest an increased risk of CD associated colorectal cancer. We report our experience with 10 cases of CD complicated by intestinal adenocarcinoma. Materials and methods Our institutional database included 330 patients treated for CD between 1988–2005. Data of patients that developed carcinoma within Crohn’s lesions of either small or large bowel were analyzed. Results Ten patients were diagnosed with CD complicated by carcinoma. In nine patients, cancer was present in the colorectum and in one, in Crohn’s ileitis. Tumors were in conjunction with fistulae in three and developed within strictures in five patients. Mean age at the time of diagnosis of CD was 43 years. Mean duration of CD until diagnosis of cancer was 14 years. Only five patients were diagnosed for cancer preoperatively. Staging revealed advanced tumors in almost all patients. Mean survival after surgery was 29 months (2–149 months). Conclusions Cancer risk in CD and especially in Crohn’s colitis may still be underestimated. Delayed diagnosis resulted in a poor prognosis. The value of colonoscopy as surveillance tool is questioned by the fact that in our patients, carcinoma was diagnosed in some patients preoperatively by routine colonoscopy. Therefore, additional markers should be identified to detect CD patients at risk.