Radan Keil

Diagnostic efficacy of ERCP in cholestatic infants and neonates ― a retrospective study on a large series

BACKGROUND AND STUDY AIMS Cholestatic jaundice in infants is a serious condition, requiring timely and accurate diagnostic evaluation. Our aim was to determine the safety and diagnostic efficacy of endoscopic retrograde cholangiopancreatography (ERCP) in the diagnosis of cholestatic liver disease in neonates and infants. PATIENTS AND METHODS ERCP procedures in cholestatic infants performed in our endoscopy unit between December 1998 and March 2008 were reviewed retrospectively (n = 104 children, 48 boys, 56 girls; mean age 7 weeks, range 3 - 25 weeks; mean weight 4.05 kg, range 1.5 - 4.8 kg). Endoscopic findings were compared with final diagnoses. Statistical analysis was performed and sensitivity, specificity, positive (PPV) and negative (NPV) predictive values of ERCP were calculated both separately for each diagnosis and on aggregate. RESULTS Cannulation of the papilla was successful in 95 of 104 patients (success rate 91.3 %). Biliary atresia of any type was found in 51 children (53.7 %), with a sensitivity of 86 %, a specificity of 94 %, a PPV of 96 %, and a NPV of 100 %. Choledochal cysts were found in seven children (7.4 %), with a sensitivity of 100 %, a specificity of 90 %, PPV of 86 %, and NPV of 100 %. Biliary stones were found in seven patients (7.4 %). Other structural pathology was found in six patients, and no abnormality was seen in 24 patients. No severe complications occurred during or after ERCP. CONCLUSIONS ERCP in cholestatic infants, when performed in an expert center, is a safe and reliable procedure that can detect biliary tract abnormalities (e. g. biliary atresia, bile duct stones or choledochal cysts) with high sensitivity and specificity. Laparotomies can be prevented in infants by demonstrating normal patency of the biliary tract by ERCP or by magnetic resonance cholangiography if improvements in this technique are made.

Clinical monitoring: infliximab biosimilar CT-P13 in the treatment of Crohn’s disease and ulcerative colitis

Abstract Objective: The infliximab biosimilar CT-P13 (Remsima®, Inflectra®) was approved in Europe for the treatment of inflammatory bowel disease (IBD) based on extrapolation of data from patients with rheumatic disease. Because there are limited published reports on clinical outcomes for IBD patients treated with CT-P13, we monitored responses to induction treatment with this biosimilar in patients with Crohn’s disease (CD) or ulcerative colitis (UC) in centres across the Czech Republic. Material and methods: Fifty-two patients with CD (n = 30) or UC (n = 22) were treated with 5 mg/kg CT-P13 for up to 14 weeks. Effectiveness of therapy was evaluated with the Crohn’s Disease Activity Index (CDAI) or the Mayo Scoring System (MSS) in patients with CD or UC, respectively, before and after 14 weeks. Additional goals were to evaluate weight changes, serum C-reactive protein (CRP) levels, and complications/adverse events. Results: In patients with CD, remission (CDAI <150) was achieved in 50.0% of cases, and partial response (≥70-point decrease in CDAI score from baseline) in the remaining 50.0%. In patients with UC, remission (total score on partial Mayo index ≤2 points) was achieved in 40.9% of cases, partial response (≥2-point decrease in partial Mayo score from baseline) in 54.5%, and no response in 4.5%. There were statistically significant improvements in CDAI, MSS and CRP serum levels after 14 weeks of therapy, and body weight increased. Four adverse events were identified (n = 1 each): lower-extremity phlebothrombosis, herpes labialis, pneumonia and allergic reaction. Conclusions: This prospective observational study provides evidence of the effectiveness of CT-P13 in IBD.

Pediatric gastrointestinal endoscopy: European Society of Gastrointestinal Endoscopy (ESGE) and European Society for Paediatric Gastroenterology Hepatology and Nutrition (ESPGHAN) Guideline Executive summary

This Executive summary of the Guideline on pediatric gastrointestinal endoscopy from the European Society of Gastrointestinal Endoscopy (ESGE) and the European Society for Paediatric Gastroenterology Hepatology and Nutrition (ESPGHAN) refers to infants, children, and adolescents aged 0 - 18 years. The areas covered include: indications for diagnostic and therapeutic esophagogastroduodenoscopy and ileocolonoscopy; endoscopy for foreign body ingestion; endoscopic management of corrosive ingestion and stricture/stenosis; upper and lower gastrointestinal bleeding; endoscopic retrograde cholangiopancreatography, and endoscopic ultrasonography. Percutaneous endoscopic gastrostomy and endoscopy specific to inflammatory bowel disease (IBD) have been dealt with in other Guidelines and are therefore not mentioned in this Guideline. Training and ongoing skill maintenance will be addressed in an imminent sister publication.

Pediatric Gastrointestinal Endoscopy: European Society of Pediatric Gastroenterology Hepatology and Nutrition (ESPGHAN) and European Society of Gastrointestinal Endoscopy (ESGE) Guidelines

This guideline refers to infants, children, and adolescents ages 0 to 18 years. The areas covered include indications for diagnostic and therapeutic esophagogastroduodenoscopy and ileocolonoscopy; endoscopy for foreign body ingestion; corrosive ingestion and stricture/stenosis endoscopic management; upper and lower gastrointestinal bleeding; endoscopic retrograde cholangiopancreatography; and endoscopic ultrasonography. Percutaneous endoscopic gastrostomy and endoscopy specific to inflammatory bowel disease has been dealt with in other guidelines and are therefore not mentioned in this guideline. Training and ongoing skill maintenance are to be dealt with in an imminent sister publication to this.

What is the role of endoscopic retrograde cholangiopancreatography in assessing traumatic rupture of the pancreatic in children

Abstract Background and study aims: Trauma is one of the most common causes of morbidity and mortality in the pediatric population. The diagnosis of pancreatic injury is based on clinical presentation, laboratory and imaging findings, and endoscopic methods. CT scanning is considered the gold standard for diagnosing pancreatic trauma in children. Patients and methods: This retrospective study evaluates data from 25 pediatric patients admitted to the University Hospital Motol, Prague, with blunt pancreatic trauma between January 1999 and June 2013. Results: The exact grade of injury was determined by CT scans in 11 patients (47.8%). All 25 children underwent endoscopic retrograde cholangiopancreatography (ERCP). Distal pancreatic duct injury (grade III) was found in 13 patients (52%). Proximal pancreatic duct injury (grade IV) was found in four patients (16 %). Major contusion without duct injury (grade IIB) was found in six patients (24%). One patient experienced duodeno-gastric abruption not diagnosed on the CT scan. The diagnosis was made endoscopically during ERCP. Grade IIB pancreatic injury was found in this patient. One patient (4%) with pancreatic pseudocyst had a major contusion of pancreas without duct injury (grade IIA). Four patients (16%) with grade IIB, III and IV pancreatic injury were treated exclusively and nonoperatively with a pancreatic stent insertion and somatostatine. Two patients (8%) with a grade IIB injury were treated conservatively only with somatostatine without drainage. Eighteen (72 %) children underwent surgical intervention within 24 h after ERCP. Conclusion: ERCP is helpful when there is suspicion of pancreatic duct injury in order to exclude ductal leakage and the possibility of therapeutic intervention. ERCP can speed up diagnosis of higher grade of pancreatic injuries.

Alagille Syndrome Mimicking Biliary Atresia in Early Infancy.

Alagille syndrome may mimic biliary atresia in early infancy. Since mutations in JAG1 typical for Alagille syndrome type 1 have also been found in biliary atresia, we aimed to identify JAG1 mutations in newborns with proven biliary atresia (n = 72). Five biliary atresia patients with cholestasis, one additional characteristic feature of Alagille syndrome and ambiguous liver histology were single heterozygotes for nonsense or frameshift mutations in JAG1. No mutations were found in the remaining 67 patients. All “biliary atresia” carriers of JAG1 null mutations developed typical Alagille syndrome at the age of three years. Our data do not support association of biliary atresia with JAG1 mutations, at least in Czech patients. Rapid testing for JAG1 mutations could prevent misdiagnosis of Alagille syndrome in early infancy and improve their outcome.

Crohn's disease: Is there a place for neurological screening?

Abstract Objective. Neurological complications of inflammatory bowel diseases (IBDs) are not rare but are under-diagnosed; some are probably immune-mediated. Several previous studies have suggested a higher incidence of demyelinating diseases such as multiple sclerosis in IBD patients. In this single-center, prospective, observational study, the authors focus on T2 focal white-matter lesions of the central nervous system on magnetic resonance imaging (MRI) in IBD patients that may be due to demyelination. Material and methods. A total of 70 patients with Crohns disease were examined before beginning anti-TNF-α therapy. These patients were treated with azathioprine, mesalazine or both. Patients were examined by a neurologist to detect possible signs of demyelinating disease, and patients underwent brain MRI (native T1, T2, and FLAIR sequences). Results. Thirty-seven patients (53%) exhibited abnormalities on neurological examination, and 26 patients (37%) displayed abnormalities on MRI. In seven cases, these MRI abnormalities (periventricular lesions) were suspected to be due to demyelination. Cerebral spinal fluid investigation (including polyclonal bands) was completely negative in five cases and was borderline in one case, and multiple sclerosis was confirmed in one case. Pathological MRI findings in 19 other patients were clinically nonsignificant; most were nonspecific sporadic lesions in white matter or mild atrophy. Conclusions. The results support previous data that the frequency of neurological findings in IBD patients is generally underestimated. With the extension of biological anti-TNF-α treatment for IBD, the possibility of a higher risk of developing multiple sclerosis should be considered.

Intestinal lymphangiectasia: A rare cause of gastrointestinal bleeding?

TO THE EDITOR: Primary intestinal lymphangiectasia is a rare cause of exudative enteropathy. Symptoms include diarrhoea, peripheral oedema, lymph oedema of the limbs, malaise and weight loss. A 25year-old female was admitted to our hospital because of gastrointestinal bleeding, which had occurred after blunt trauma of the abdomen. The endoscopic procedures (gastroscopy, push enteroscopy, wireless capsule) showed oedematous mucosa with scattered white spots, white villi, chyle-like matter covering the mucosa and diffuse bleeding of the small intestine. The other procedures (lymphoscintigraphy, lymphography) showed a picture of hyperplasia and dysplasia of the lymphatic vessels. In accordance with these investigations the final diagnosis of primary intestinal lymphangiectasia was established. The patient was treated with an middle chain triglycerids (MCT) enriched diet, lanreotide and, finally, by tranexamic acid. During therapy, there was a decrease in loss of blood and proteins. However, other complications occurred during the treatment (chylothorax, respiratory failure, recurrent sepsis, thrombosis of vena cava superior). The patient is still hospitalized in the intensive care unit, and her prognosis remains uncertain. Bleeding of the gastrointestinal tract as the main symptom of this disease is rarely described. There are few studies on the treatment of primary intestinal lymphangiectasia, most of them being case studies. The effectiveness of an MCT enriched diet along with a decrease in consumption of other fats has been described. In cases of disease limited to just part of the small intestine, surgical resection is usually the treatment of choice. Some investigators described good effects of steroids in patients with increased acute inflammatory markers. Other papers described the efficacy of using somatostatin analogues. In our patient, tranexamic acid as a somatostatin analogue led to a decrease in blood and protein loss. Despite treatment of symptoms, the prognosis of patients with primary intestinal lymphangiectasia remains uncertain. The rare incidence and heterogeneity of patients make it difficult to gain a better understanding of this disease.

The role of endoscopic retrograde cholangiopancreatography in choledochal cysts and/or abnormal pancreatobiliary junction in children

Introduction Biliary cysts with an abnormal pancreatobiliary junction are one of the most common pancreatobiliary malformations. The main symptom is cholestasis; endoscopic retrograde cholangiopancreatography (ERCP) plays a key role in diagnosis. Aim Retrospective evaluation of ERCP performed to diagnose abnormalities of the pancreatobiliary junction. Material and methods We retrospectively evaluated ERCP performed to diagnose abnormalities of the pancreatobiliary junction, mainly choledochal cysts, in 112 children between 1990 and 2011. Results We performed 112 examinations of 50 children with abnormal pancreatobiliary junction and choledochal cysts (15 males and 35 females, average age: 5 years, range: 1 month – 15 years). Cysts were associated with a common channel in 37 (74%) cases, were not associated with a common channel in 9 (18%) cases, and in 3 (6%) cases the common channel lacked cysts. We performed endoscopic papilla sphincterotomy on 33 (66%) patients; endoscopic drainage was performed 62 times, including 17 patients without papilla sphincterotomy. In 15 (30%) cases, we only performed sphincterotomy. Extraction of lithiasis was done in 2 (4%) cases. Both ERCP and magnetic resonance cholangiopancreatography (MRCP) were performed on 13 patients. There was concordance of the choledochal cyst and of the type of cyst in both methods; however, common channels could not be observed by MRCP. There was 1 serious complication (perforation after sphincterotomy) and 11 (9%) mild adverse events. Conclusions When performed at an expert centre, ECRP is a safe and reliable procedure for children with choledochal cysts and/or an abnormal pancreatobiliary junction. Endoscopic retrograde cholangiopancreatography remains a reasonable alternative because MRCP has a limited ability to diagnose the precise anomalies of the pancreatobiliary junction and has no therapeutic capabilities.

Adherence, risk factors of non-adherence and patient’s preferred treatment strategy of mesalazine in ulcerative colitis: multicentric observational study

Abstract Objectives: Compliance to therapy is a key factor in the efficacy of treatment in clinical practice. The aim of our study was to evaluate the rate of compliance with mesalazine in patients with ulcerative colitis (UC), to examine risk factors of noncompliance and especially find ways on how adherence can be improved. Materials and methods: A total of 198 outpatients with UC completed two anonymous questionnaires including information on basic demographics, details of patient´s disease and the use of mesalazine medication and quality of life. Results: We found noncompliance (percentage of used medication per day less than 80%) with 5-ASA in 21.2% patients. Our study proved that the education level of patients significantly influenced the compliance of patients using mesalazine. A significant difference (p = .014) was found between the compliance of patients with secondary school education (84.1 ± 16.73) and those with university education (94.1 ± 9.9). The majority of patients preferred mesalazine once daily and are less likely to forget to take medication in the morning. Better quality of life was observed based on our data from WHOQOL-BREF questionnaire in statistically significant way in patients using concomitant therapy of immuosuppressive or biological therapy, lower daily doses and using sachets not tablets. Conclusions: Our study proved that compliance with mesalazine in patients with UC was related only to education level. If we target mesalazine therapy based on patient’s preferences, we can improve the adherence with mesalazine. Our data could be beneficial for the treatment strategy in clinical practice.