Nonalcoholic fatty liver disease and the risk of post-endoscopic retrograde cholangiopancreatography pancreatitis: the hidden danger.

BACKGROUND: Nonalcoholic fatty liver disease (NAFLD) has been identified as risk factor for several diseases; however, its association with post endoscopic retrograde cholangiopancreatography pancreatitis (PEP) has not been studied. AIMS: To assess whether NAFLD is a risk factor for the development of PEP. METHODS: We performed a retrospective multicenter study. All patients who underwent ERCP during 2013-2016 at either the Shaare Zedek Medical Center in Jerusalem or EMMS Nazareth hospital and who had a diagnosis of NAFLD by abdominal imaging were eligible for inclusion. Four hundred and one patients were included, among them, 38 (9.5%) were diagnosed with PEP according to clinical, laboratory and radiological criteria. RESULTS: In univariate analysis, the following risk factors were associated with increased risk for PEP; Fatty liver (OR 2.363, p = .01), elevated levels of aspartate transaminase (OR 1.008, p = .04), ALT (OR 0.979, p = .0007), alkaline phosphatase (OR 1.008, p = .01), gamma-glutamyl transferase (OR 1.014, p = .0005) and total bilirubin (OR 1.141, p = .005). In multivariate logistic regression analysis, only NAFLD showed statistically significant association with PEP (OR 3.224, 95% CI 1.548-6.713, p = .001) with receiver operator characteristics (ROC) area under the curve (AUC) of 0.8156. CONCLUSION: NAFLD was shown to be a risk factor for PEP. Therefore, we suggest considering prophylactic pancreatic stenting and/or NSAID's suppositories among these patients.

Colonoscopy in Nonagenarians Is Safe and May Be Associated with Clinical Benefit.

OBJECTIVES: Data regarding colonoscopy in patients older than 90 years old is scarce. Yet the number of colonoscopies done on nonagenarians is rising. We aimed to determine the yield, safety, and therapeutic benefits of colonoscopy in these patients. DESIGN: Case-control study of older patients who underwent colonoscopy. SETTING: Gastroenterology institute at an academic medical center. PARTICIPANTS: Patients older than 90 years (n = 128) compared with patients aged 80 to 89 years (n = 218) who underwent colonoscopy. INTERVENTION: Colonoscopy. MEASUREMENTS: Indication for the procedure, completion rates, adequacy of preparation, complications, colonoscopic findings, 30-day mortality, advanced adenoma and carcinoma detection rate, treatment, and long-term survival of patients diagnosed with colorectal cancer. RESULTS: Mean ages were 83.3 and 92.2 years old. Nonagenarians were more likely to undergo a colonoscopy while hospitalized (56.2 vs 23.4%; P < .001) and to undergo the examination due to rectal bleeding or sigmoid volvulus (35.2 vs 25.2 and 10.9 vs 0.5%, respectively; P < .001) and less likely for surveillance or constipation (11.7 vs 25.7 and 0 vs 6.9%, respectively; P < .001). Completion rates and severe adverse events were comparable. The 30-day mortality was 3.9% in nonagenarians and 0.4% in octogenarians (P = .02). Advanced adenomas and carcinoma were more common in nonagenarians (25.8 vs 16.5%, P = .03, and 14.8 vs 6.4%, P = .01, respectively). Increasing age, inpatient status, past polypectomy surveillance, and anemia were associated with higher rates of carcinoma. Half of the nonagenarians diagnosed with adenocarcinoma underwent surgery compared with 100% of octogenarians (P = .01). Among nonagenarians with colorectal cancer who died, mean survival was 605 (interquartile range = 11-878) days in those who underwent surgery and 112 (48-341) in those treated conservatively (P = .055 log-rank test). CONCLUSION: Colonoscopy in nonagenarians has a high yield and is generally safe. Colonoscopy findings lead to surgery in more than half of these patients and was associated with a median survival of 20 months.

Chronic Use of Statins and Their Effect on Prevention of Post-Endoscopic Retrograde Cholangiopancreatography Pancreatitis.

Background and Aims: Post-endoscopic retrograde cholangiopancreatography (ERCP) pancreatitis (PEP) is one of the major complications of ERCP. Thus, several non-invasive as well as invasive strategies have been investigated as preventative therapies for PEP with various efficacy. Methods: We enrolled any patients who underwent ERCP both at the Shaare Zedek Medical Center in Jerusalem and EMMS Nazareth hospital. Association between use of statins and different variables were assessed with univariate tests (chi-squared for categorical variables). Predictors of incidence of PEP and severity of pancreatitis were computed using conditional logistic regression, correcting for potential confounding factors. Results: 958 subjects were analyzed. Average age was 62.04 ± 21.18 years (median 68 years). Most of the patients were female (n = 558, 58.2%), Jewish (n = 827, 86.3%), and inpatients (n = 631, 65.9%). Only few ERCPs were performed emergently (n = 40, 4.2%). Twenty-Seven patients repeated the exam. Overall incidence of PEP/hyperamylasemia was 16.8% (n = 161); with a 5.6% (n = 54) incidence of hyperamylasemia and a 11.2% (n = 107) incidence of pancreatitis. Overall, 6 cases of severe pancreatitis were identified. The logistic regression analysis demonstrated that chronic use of statins is a protective factor in preventing development of PEP/hyperamylasemia [OR 0.436 [95%CI 0.303-0.627], p < 0.001]; Particularly, the PEP OR was of 0.318 [95%CI 0.169-0.597], p < 0.001 and the hyperamylasemia OR was of 0.565 [95%CI 0.372-0.859], p = 0.008. No significant predictor could be found for the risk of developing severe PEP. Conclusions: Our data support the possibility of exploiting statins as preventive agents for PEP. However, further studies, mainly RCTs, are warranted in order to replicate our findings.

Hearing Loss in Patients with Inflammatory Bowel Disease.

BACKGROUND AND AIMS: Inflammatory bowel disease (IBD) has many characteristics of autoimmune diseases. Sensorineural hearing loss has been reported in many autoimmune diseases. Little is known about hearing loss in patients with IBD. METHODS: A prospective blinded comparative study was conducted over a 3-year period. IBD patients and controls underwent a complete otorhinolaryngeal examination and eudiometry test. RESULTS: Altogether 105 participants (76 patients and 29 controls) took part in this study. Mean age was 36, 51 % were males, and 40 % of the patients were presently hospitalized due to IBD exacerbation. Audiometric examination revealed that any hearing loss (mild to severe) was found in 29 (38 %) of the IBD population, compared to 4 (14 %) of the control group (p = 0.02). Extraintestinal manifestation (EIM) was present in 33/76 (43 %) of IBD patients. Any hearing loss and moderate to severe hearing loss were found in 17/33 (52 %) and 7/33 (21 %) in the EIM-positive group compared to 12/43 (28 %) and 4/43 (9 %) in the EIM-negative group (p = 0.036 and p = 0.14, respectively). Out of patients over the age of 40 with other EIMs, all 11/11 (100 %) of patients had any hearing loss compared to 8/12 (66 %) of patients over the age of 40 without other EIMs, p = 0.035. CONCLUSIONS: Hearing loss may be another EIM of IBD. It is found in 38 % of IBD patients and in up to 52 % of patients with other EIMs and increases over the age of 40. Early hearing evaluation should be recommended to these high-risk IBD patients.

Radiofrequency ablation for the treatment of radiation proctitis.

BACKGROUND AND STUDY AIMS: The main endoscopic therapy for radiation proctitis is argon plasma coagulation (APC); however treatment is not always successful. Radiofrequency ablation (RFA) is a possible treatment for radiation proctitis but data are scarce. The aim of this study was to report on the safety and efficacy of RFA in the treatment of radiation proctitis. PATIENTS AND METHODS: This study was an open-label, retrospective, multicenter study of patients with chronic hemorrhagic radiation proctitis who were treated with RFA. Data included a three-item symptom score, the number of packed red blood cell transfusions, the lowest hemoglobin concentration, and complications, during the 6 months prior to and after RFA. Clinical success was defined as a decrease in the symptom score. Biological success was defined as an increase in the hemoglobin rate with equal or decreased number of transfusions required. RESULTS: A total of 17 patients underwent a median of 2 RFA sessions (range 1 - 4), without perioperative complications. Symptom scores decreased in 16 patients (clinical success 94 %), from a mean score of 3.6 (median 4) to 1.4 (median 1) (P < 0.01). Two patients developed rectal ulceration, with no local symptoms. During the 6 months after RFA, hemoglobin concentration increased in all 17 patients (from mean 8.3 ± 2.8 g/dL [median 7.5] to 11.3 ± 2.2 g/dL [median 11.0]; P < 0.01). Among 13 patients who were transfusion dependent prior to RFA (mean 7.2 ± 7.7 transfusions [median 4]), 9 patients (69 %) were weaned off transfusions after RFA. A significant increase in the hemoglobin level was observed in this subgroup of patients (from mean 7.2 ± 1.4 g/dL [median 7.3] to 10.7 ± 1.5 g/dL [median 10.5]; P < 0.001). Biological success was 100 %. CONCLUSIONS: RFA seems to significantly decrease clinical symptoms and increase the hemoglobin concentration, thus reducing the need for transfusions.

Radiofrequency ablation for the treatment of gastric antral vascular ectasia.

BACKGROUND AND STUDY AIMS: The traditional endoscopic treatment for gastric antral vascular ectasia (GAVE) is argon plasma coagulation, but results are not always positive. Radiofrequency ablation (RFA) is a new endoscopic therapy that may be an attractive option for the treatment of GAVE. The aim of this study was to assess the efficacy and safety of RFA for the treatment of GAVE. PATIENTS AND METHODS: This was an open-label, retrospective, case series study. The main outcome measures were number of red blood cell (RBC) packs transfused (transfusion requirement) and hemoglobin concentrations (g/dL) in the 6 months prior to and after RFA. Success was defined as a decrease in transfusion requirement in the 6 months after RFA compared with before treatment. RESULTS: A total of 24 patients underwent a mean of 1.8 ± 0.8 RFA sessions. No complications were reported. One patient was referred for additional argon plasma coagulation during follow-up. The mean number of RBC packs decreased in all 23 transfusion-dependent patients, from a mean of 10.6 ± 12.1 during the 6 months prior to RFA, to a mean of 2.5 ± 5.9 during the 6 months after RFA treatment (P < 0.001), and 15 patients (65.2 %) were weaned off transfusions completely. An increase in the hemoglobin concentration was reported in all patients after RFA (from 6.8 ± 1.4 g/dL to 9.8 ± 1.8 g/dL; P < 0.001). CONCLUSION: RFA for the treatment of GAVE seems feasible and safe, and significantly reduced the need for RBC transfusion and increased the hemoglobin level in this retrospective case series.

Unexpected FDG-PET uptake in the gastrointestinal tract: endoscopic and histopathological correlations.

AIM: To investigate the nature and significance of unexpected positron emission tomography with fluorodeoxyglucose (FDG-PET) uptake within the gastrointestinal tract (GIT). METHODS: Patients with unexpected FDG-PET findings in the GIT were evaluated. All patients had a previous confirmed malignancy, either solid or lymphoproliferative. The radiologic reports were performed by experienced radiologists with an exclusive PET expertise. Endoscopy, i.e., esophagogastroduodenoscopy (EGD) and colonoscopy, and histopathological evaluation of all findings was performed in all patients in accordance to the FDG-PET results. The findings from each of these modalities were compared to each other. Both clinically significant and insignificant findings were assessed. RESULTS: Seventy-two patients were endoscopically evaluated. Twenty-seven patients (37.5%) had primarily a lymphoproliferative tumor and 45 (62.5%) had solid tumors. In 50 patients (69.4%) the endoscopic examination revealed lesions in the same anatomical areas as the FDG-PET findings. Among these 50 patients, malignant and premalignant lesions i.e., adenomatous polyps were found in 16 (32%) and 9 (18%) patients, respectively. Inflammation was noted in an additional 20 patients (40%). Compared to primary solid tumors, a background of primary lymphoproliferative malignancy was more likely to reveal an additional primary malignancy (15.6% vs 33.3%, respectively, P < 0.01). EGD compared to colonoscopy, revealed altogether 11 (25.6%) new malignancies compared to 5 (17.2%), respectively, P = 0.12. No GIT clinically significant findings were overseen by the FDG-PET. CONCLUSION: Unexpected FDG uptake in the GIT is commonly encountered and may contain significant findings. Endoscopy evaluation is justified in order to detect these additional findings.

Endoscopic retrograde cholangiopancreatography in neonatal cholestasis.

BACKGROUND: Endoscopic retrograde cholangiopancreatography (ERCP) is not as widely used in children as in adults and is performed in few specialized centers. The aim of the present study was to review the experience of ERCP in children younger than 3 months in a national referral center. METHODS: A retrospective chart review was performed of all of the babies younger than 3 months who underwent ERCP between 2000 and 2010. Data on demographics, diagnosis, type of anesthesia, treatments, and complications were collected. RESULTS: A total of 27 babies, 14 boys, were examined. Median age was 55 days (range 33-89). Ultrasound was normal in 16 infants, whereas others included small gallbladder (4), biliary stones (3), and dilated bile ducts (3). Thirteen infants underwent earlier liver biopsy, which was inconclusive. ERCP led to the diagnosis of biliary atresia in 13 infants who had subsequent surgery. In others, ERCP showed choledochal cyst (1), biliary stones (2), dilated bile ducts (1), and normal examination (6); there were 5 failures. The final diagnoses in our cohort were extrahepatic biliary atresia (15), biliary stones (5), neonatal hepatitis (4), choledochal cyst (1), paucity of intrahepatic bile duct (1), and congenital hepatic fibrosis (1). Diagnoses in the failed ERCP group included biliary atresia (2), bile duct paucity (1), and biliary stones (2). In 4 (19%) infants with clinical suspicion of extrahepatic biliary atresia, a normal ERCP ruled out the diagnosis and avoided an intraoperative cholangiogram. No complications, including pancreatitis, were reported. CONCLUSIONS: ERCP in infants is feasible and has no complications. It may serve as an additional diagnostic tool in neonatal cholestasis in inconclusive cases and may prevent more invasive procedures. ERCP may be part of the algorithm of neonatal cholestasis when it is available and other investigations fail to confirm a diagnosis.

Effects of guided imagery with relaxation training on anxiety and quality of life among patients with inflammatory bowel disease.

BACKGROUND: Inflammatory Bowel Disease (IBD) impacts quality of life (QoL). Psychological factors influence the course of the disease and should be targeted for intervention. METHODS: Our study was a prospective, randomised control trial. Fifty-six outpatients were randomly chosen and allocated to a treatment group or a waiting-list control group. Treatment group patients attended three relaxation-training sessions and received an audio disc for home practice. Evaluations performed pre and post-treatment: state anxiety was assessed with the State-Trait Anxiety Inventory, QoL with the IBD Questionnaire. The Visual Analogue Scale assessed pain, depression, stress and mood. Patients completed a symptom monitoring diary. The control group's symptoms were monitored without study-related treatment. RESULTS: Thirty-nine subjects completed the study and were included in the data analysis. Following the relaxation-training intervention, the treatment group's (n = 18) measured results showed a statistically significant improvement as compared to the control group (n = 21) (time by treatment interaction): anxiety levels decreased (p < 0.01), QoL and mood improved (p < 0.05), while levels of pain and stress decreased (p < 0.01). CONCLUSIONS: Findings indicate IBD patients may benefit from relaxation training in their holistic care. New studies as well as further investigation of the subject are warranted.

Losartan reduces trinitrobenzene sulphonic acid-induced colorectal fibrosis in rats.

BACKGROUND: Intestinal fibrosis is a challenging clinical condition in several fibrostenosing enteropathies, particularly Crohn's disease. Currently, no effective preventive measures or medical therapies are available for intestinal fibrosis. Fibrosis, due to an abnormal accumulation of extracellular matrix proteins, is a chronic and progressive process mediated by cell/matrix/cytokine and growth factor interactions, but may be a reversible phenomenon. Of the several molecules regulating fibrogenesis, transforming growth factor-beta 1 (TGF-b1) appears to play a pivotal role; it is strongly induced by the local activation of angiotensin II. The levels of both TGF-b1 and angiotensin II are elevated in fibrostenosing Crohn's disease. AIMS: To evaluate the in vivo effect of losartan - an angiotensin II receptor antagonist - on the course of chronic colitis-associated fibrosis and on TGF-b1 expression. METHODS: Colitis was induced by intrarectal instillation of trinitrobenzene sulphonic acid (TNBS) (15 mg/mL) while losartan was administered orally daily by gavage (7 mg/kg/day) for 21 days. Three groups of rats were evaluated: control (n=10); TNBS treated (n=10); and TNBS + losartan treated (n=10). Inflammation and fibrosis of the colon were evaluated by macro- and microscopic score analysis. Colonic TGF-b1 levels was measured using ELISA. RESULTS: Twenty-one days after induction, losartan significantly improved the macro- and microscopic scores of fibrosis in the colonic wall and reduced TGF-b1 concentration. CONCLUSIONS: Prophylactic oral administration of losartan reduces the colorectal fibrosis complicating the TNBS-induced chronic colitis, an effect that appears to be mediated by a downregulation of TGF-b1 expression.

The effect of restraint stress on the normal colon and on intestinal inflammation in a model of experimental colitis.

Stress may induce development of inflammation in animal models of colitis. The effects of restraint stress on oxidative damage and on antioxidants in the normal colonic mucosa were studied. The effect of stress on the severity of indicators of inflammation, as well as the importance of mucosal substance P (SP) as a mediator of this effect were investigated in the TNBS-colitis model. Restraint stress significantly increased malondialdehyde levels and reduced levels of low-molecular-weight-antioxidants in the normal colon. ATP and the mucosal "energy charge" decreased substantially with chronic stress. Chronic stress worsened the extent of inflammation in 2,4,6-trinitrobenzene sulfonic acid (TNBS)-induced colitis. Mucosal SP content was not affected by exposure to chronic stress but increased after induction of colitis. The increase was greater when colitis was induced after exposure to stress. We conclude that chronic restraint stress causes oxidative damage to the normal colon and aggravates intestinal inflammation induced by TNBS. This effect may be mediated by SP.

Should stored serum of patients previously tested for celiac disease serology be retested for transglutaminase antibodies?

INTRODUCTION: Tissue transglutaminase (tTG) antibodies are currently recognized as a highly sensitive indicator of celiac disease (CD). Although a high concordance rate between tTG antibodies and anti-endomysial antibodies (EMA) has been reported up to a third of known CD patients are positive for only one of these antibodies. AIM: To determine whether in laboratories in which serum samples previously examined for CD serology markers had not been discarded, these samples should be tested for tTG antibodies. METHODS: Fifty-eight stored (frozen at -70) serum samples of patients previously found to be EMA-negative but positive for one or more of the non-EMA markers: antigliadin antibodies (AGA)-IgA, AGA-IgG, antireticulin antibodies, were tested for anti-tTG antibodies (IMMCO Diagnostics). In patients found to be tTG positive, medical charts were reviewed and patients or their physicians contacted. RESULTS: Twelve of fifty-eight (20.7%) samples were found to be anti-tTG positive. These included: group A: 3/3 samples previously positive for AGA-IgA, AGA-IgG, and antireticulin antibodies. Group B: 3/16 samples positive for AGA-IgA and AGA-IgG. Group C: 3/4 samples positive for AGA-IgA and group D: 3/35 samples positive for AGA-IgG. Of the 12 positive patients, 1 was a 2-year-old boy, 5 were lost to follow up, and 7 underwent an intestinal biopsy. In 3 of these 7 patients, the biopsy was compatible with CD; 2 of these 3 patients were from group A and 1 from group B. CONCLUSIONS: In laboratories where stored serum samples are available, EMA-negative samples previously found to be positive for at least 2 other CD markers should be retested for tTG antibodies.

Leigh syndrome: anesthetic management in complicated endoscopic procedures.

BACKGROUND: Leigh's syndrome, a disorder of infancy and childhood, is characterized by gray matter degeneration and focal brainstem necrosis. It presents with special clinical features such as developmental delay, nervous system dysfunction, respiratory abnormalities, and hypertrophic cardiomyopathy that can be a real challenge to the anesthesiologist. Anesthesia or sedation has rarely been reported in patients with Leigh disease. We report our experience in sedating five children with Leigh syndrome in seven procedures undertaken in the endoscopy suite (outside the operating room). METHODS: Five children with Leigh disease, three girls and two boys, have been referred to us for percutaneous endoscopic gastrostomy (PEG) insertion and or replacement (a total of seven procedures). The average age was 2.6 years with a range of 4 months to 6 years. Informed consent was obtained from the patient's parents or guardian. An anesthesia machine, scavenging system, O(2) source and routine monitoring were available. Sedation was accomplished with propofol intravenous (i.v.) (0.5-1 mg x kg(-1)) maintained with a propofol infusion (50-100 microg x kg(-1) x min(-1)). The spontaneously breathing patients received oxygen through an oxygen facemask during the procedure and afterwards recovery was managed in the gastroenterology unit. RESULTS: All the children underwent the procedure without complications. One patient developed transient desaturation (SpO(2) 80%) for a few seconds. Body temperature, heart rate, arterial blood pressure, O(2) saturation and endtidal CO(2) were stable during the endoscopies. No special post-procedure management was required; the patients woke up at the end of the endoscopy and were able to drink and eat as usual. CONCLUSIONS: This rare mitochondrial disease presents unique management problems to the anesthesiologist when using general anesthesia. Our patients were managed appropriately before endoscopy and underwent the procedure under deep sedation. No complications occurred. We concluded that deep sedation in the endoscopy suite was safe in this small series of patients with this rare disease.

Prophylactic administration of topical glutamine enhances the capability of the rat colon to resist inflammatory damage.

Glutamine is an important nutrient for the GI tract and has been shown to exert a protective effect on the bowel. Nonetheless, in the context of IBD, data demonstrating a therapeutic role for glutamine has been inconclusive. IBD is associated with oxidative stress caused by reactive oxygen species. We aimed to investigate the effect of topical glutamine administration in rats before or after induction of colitis by trinitrobenzenosulfonic acid. In study I glutamine enemas were given beginning 2 days before or on the same day of induction of colitis. Inflammation severity was assessed by macroscopic and microscopic score and tissue myeloperoxidase activity. In study II glutamine enemas were given for 3 days without induction of colitis: mitotic index and colonic crypt length were measured, as well as water-soluble low molecular weight antioxidants and energy-rich phosphate levels (by HPLC). Results showed that glutamine significantly decreased indexes of inflammation when administered before induction of colitis. Glutamine caused an increase in the mitotic index and the levels of water-soluble low molecular weight antioxidants and energy-rich phosphates. We conclude that glutamine exerts a beneficial effect only when administered before induction of colitis, by increasing the resistance of the colonic tissue to inflammatory injury. This effect is probably mediated by increasing the antioxidant capacity and energy level of the tissue.

Prevention of fibrosis in experimental colitis by captopril: the role of tgf-beta1.

BACKGROUND & AIMS: There is a body of evidence to suggest that the local activation of angiotensin II (ANG II) plays a pivotal role in fibrogenic response involving the kidney, heart, lung, pancreas and liver. In such conditions, fibrosis is mediated, at least partially, through ANG II induction of the cytokine transforming growth factor-beta1 (TGF-beta1). Both ANG II and TGF-beta1 also seem to be involved in intestinal fibrosis and stenosis, particularly in Crohn's disease. The aim of the present study was, firstly, to determine the effects of the angiotensin-converting enzyme inhibitor, captopril, on colonic fibrosis in experimental colitis in rats and, secondly, to check the role of TGF-beta1 on these effects. METHODS: Colitis was induced in rats by intracolonic administration of TNBS. Colonic fibrosis was assessed 21 days later by macroscopic and microscopic evaluation. Levels of collagen alpha1 gene expression, hydroxyproline, angiotensin II and TGF-beta1 proteins, and TGF-beta1 mRNA were measured on the colonic tissue. RESULTS: In chronic colitis, captopril significantly reduced the score of macroscopic and histologic lesions, as well as the colonic tissue levels of collagen alpha1, hydroxyproline, ANG II and TGF-beta1 proteins, and TGF-beta1 mRNA. CONCLUSIONS: These data demonstrate for the first time that the prophylactic administration of captopril is effective in preventing colonic fibrosis in TNBS-induced colitis. The antifibrotic action of captopril could be due to the blockade of TGFbeta-1 overexpression, and/or to a direct down-regulation of TGFbeta-1 transcript.

Familial dysautonomia: deep sedation and management in endoscopic procedures.

OBJECTIVES: Familial dysautonomia is a rare genetic disorder that affects the development of the central nervous system, causing GI dysfunction. Because of an improved prognosis, elective surgical procedures are more common and present a unique problem to the anesthesiologist. All patients reported in the literature underwent these interventions under general anesthesia in the operating theater. We report our preliminary experience with deep sedation in the endoscopy room in patients with this rare syndrome. METHODS: Four girls (7-16 yr old) underwent percutaneous endoscopic gastrostomy insertion and/or endoscopic retrograde cholangiopancreaticography. Preprocedure management consisted of adequate hydration and anxiolysis. Intraprocedure management consisted of stabilization of an erratic autonomic nervous system. Midazolam (0.1-0.2 mg/ kg) was administered i.v. before the procedure. Deep sedation was accomplished with propofol i.v. (0.5-1 mg/kg) and maintained with a propofol drip (50-100 microg/kg/min). Recovery was managed in the gastroenterology unit of our facility. RESULTS: Body temperature, ventilation, heart rate, blood pressure, oxygen saturation, and end-tidal CO2 were stable during the endoscopies. The patients regained consciousness at the end of the endoscopy and were able to drink or to eat as normal. Pain that could precipitate a crisis was present in two patients and was successfully treated with a simple analgesic. No other complications occurred. CONCLUSION: This rare genetic disorder presents unique management problems to the anesthesiologist, resulting in morbidity and mortality when general anesthesia is used. Our patients received appropriate management before endoscopy, and we performed the procedure under deep sedation. No complications occurred. We are thus confident that deep sedation in the endoscopy suite is safe in this rare syndrome.

Palliation of malignant bile duct obstruction: our experience with endoscopic 14 French prostheses

We evaluated the efficacy of wide 14F endoprostheses endoscopically placed in en patients with malignant extrahepatic bile duct stenosis. Large-bore stents were successfully placed in all patients. There were no early complications. Stent clogging occurred in two patients after one month and after three months in another patient. Seven patients remained free of symptoms after a mean follow-up period of 4.5 months