Susanne Schibli
Boston Children's Hospital
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Featured researches published by Susanne Schibli.
Current Opinion in Pulmonary Medicine | 2002
Susanne Schibli; Peter R. Durie; Elizabeth Tullis
With the recognition of the close link between nutritional status and pulmonary function in cystic fibrosis (CF), treatment and prevention of malnutrition have become a major focus in the modern therapeutic approach for patients with CF. Thereby, pancreatic enzyme replacement therapy plays a central role. This article reviews key publications on important aspects of pancreatic enzyme replacement therapy contained in the literature over the last 12 months. New insights into the pathogenesis of exocrine pancreatic disease, efficacy and dosing of pancreatic enzyme preparations, occurrence of fibrosing colonopathy, enzyme replacement in the context of enteral nutrition, and assessment of pancreatic function are addressed.
Clinical Gastroenterology and Hepatology | 2006
Susanne Schibli; Mary Corey; Kevin J. Gaskin; Lynda Ellis; Peter R. Durie
BACKGROUND & AIMS Although pancreatic stimulation tests quantify acinar and ductal exocrine pancreatic function, no standard methodology exists. We evaluated the impact of several variables on test accuracy. METHODS We performed a retrospective analysis of pancreatic stimulation tests, which involved continuous stimulation with cholecystokinin and secretin, 3 sampling periods (20-min each), and perfusion markers to correct for intestinal losses. Results were recalculated using the following variables: no correction for losses; shortened sampling time (20-min); no correction and shortened sampling time; and enzyme concentration. We examined how these variables influenced measurements of pancreatic secretion and classification of pancreatic function status (sufficient or insufficient). RESULTS We analyzed 363 tests in control patients (20), and patients with cystic fibrosis (137), Shwachman-Diamond syndrome (40), or other pancreatic or intestinal disorders (166). Recovery of pancreatic juice varied markedly between tests (median, 59%; range, 4%-106%) and was significantly poorer during the first 20-minute period compared with the 2 subsequent periods (P < .01). Failure to correct for intestinal losses underestimated secretory capacity (median trypsin output reduced by >50%, P < .0001) and shortened sampling time increased test variability. Both variables together resulted in greater discrepancies. More than 25% of the pancreatic-sufficient patients with impaired pancreatic function were misclassified as pancreatic insufficient when uncorrected output plus a shortened sampling time or enzyme concentration were used to define categories. CONCLUSIONS Pancreatic function tests using brief aspiration periods without marker perfusion or measures of concentration greatly underestimate pancreatic secretory capacity and misclassify the clinical status of an unacceptably large number of patients.
Journal of Pediatric Gastroenterology and Nutrition | 2017
Thomas Greuter; Fabio Bertoldo; Roman Rechner; Alex Straumann; Luc Biedermann; Jonas Zeitz; Benjamin Misselwitz; Michael Scharl; Gerhard Rogler; Ekaterina Safroneeva; Raja Affendi Raja Ali; Christian Braegger; Klaas Heyland; Pascal Mueller; Andreas Nydegger; Laetitia Marie Petit; Susanne Schibli; Raoul I. Furlano; Johannes Spalinger; Michela Schäppi; Samuel Antonio Zamora; Florian Froehlich; Denise Herzog; Alain Schoepfer; Stephan R. Vavricka
Background: There is a paucity of data on extraintestinal manifestations (EIM) and their treatment in pediatric patients with inflammatory bowel disease (IBD). Methods: Since 2008, the Pediatric Swiss IBD Cohort Study has collected data on the pediatric IBD population in Switzerland. Data on 329 patients were analyzed retrospectively. Results: A total of 55 patients (16.7%) experienced 1–4 EIM (39 Crohn disease, 12 ulcerative colitis, and 4 IBD-unclassified patients). At IBD onset, presence of EIM was more frequent than in the adult population (8.5% vs 5.0%, P = 0.014). EIM were more frequent in Crohn disease when compared to ulcerative colitis/IBD-unclassified (22.5% vs 10.3%, P = 0.003). The most prevalent EIM were peripheral arthritis (26/329, 7.9%) and aphthous stomatitis (24/329, 7.3%). Approximately 27.6% of all EIM appeared before IBD diagnosis. Median time between IBD diagnosis and occurrence of first EIM was 1 month (−37.5–149.0). Thirty-one of the 55 patients (56.4%) were treated with 1 or more anti–tumor necrosis factor (TNF) agents. IBD patients with EIM were more likely to be treated with anti-TNF compared to those without (56.4% vs 35.0%, P = 0.003). Response rates to anti-TNF depended on underlying EIM and were best for peripheral arthritis (61.5%) and uveitis (66.7%). Conclusions: In a cohort of pediatric patients with IBD, EIM were frequently encountered. In up to 30%, EIM appeared before IBD diagnosis. Knowledge of these findings may translate into an increased awareness of underlying IBD, thereby decreasing diagnostic delay. Anti-TNF for the treatment of certain EIM is effective, although a substantial proportion of new EIM may present despite ongoing anti-TNF therapy.
Pediatric Radiology | 2010
Sylvia C. Wetli; Eva S. Gralla; Susanne Schibli; Enno Stranzinger
In Alagille syndrome, routine follow-up imaging of the liver plays an important role in detecting early parenchymal changes and to evaluate portal hypertension. Modern contrast-enhanced imaging methods not only allow early detection of focal liver lesions, but also enable further characterization of their nature and guide biopsy procedures. We present the US and MR imaging findings of hepatocellular carcinoma and a regenerating nodule in a 3-year-old child with Alagille syndrome.
Journal of Pediatric Gastroenterology and Nutrition | 2010
Michael Stormon; Lynda Ellis; Susanne Schibli; Johanna M. Rommens; Peter R. Durie
Background: Because the acinar cells of the exocrine pancreas in patients with Shwachman-Diamond syndrome (SDS) are severely depleted, we hypothesized that a similar deficiency may be present in acinar cells of the parotid gland. Patients and Methods: We determined serum pancreatic isoamylase and parotid amylase activities in 16 patients with SDS, 13 healthy controls, and 13 disease controls (cystic fibrosis or fibrosing pancreatitis). Parotid amylase and electrolyte concentrations were measured in stimulated parotid gland secretions. Starch digestion was assessed by breath hydrogen testing in patients with SDS (with and without enzyme supplements) and healthy controls. Results: Serum pancreatic and parotid isoamylase values were lower in the patients with SDS than in the healthy controls (P < 0.0001 and P = 0.0002, respectively). Serum pancreatic isoamylase, but not parotid isoamylase, was significantly lower in the disease controls than in the healthy controls (P < 0.0001 and P = 0.17, respectively). Secreted parotid gland amylase concentration (units per milligram of protein) in patients with SDS was lower than that in the healthy controls (P = 0.04), whereas the disease controls were comparable to the healthy subjects (P = 0.09). Secreted parotid chloride concentration was inversely correlated with amylase concentration in the patients with SDS (P = 0.01), but no correlation was seen in the healthy controls or disease controls. When patients with SDS ingested starch without enzyme supplementation, their breath hydrogen excretion was significantly higher than that in the healthy controls (P = 0.009). Following starch ingestion with enzymes, breath hydrogen in the patients with SDS was lower (P < 0.05) than with no enzyme treatment, and no different from controls (P = 0.37). Conclusions: Mutations in the SBDS gene cause a generalized functional abnormality of exocrine acinar cells.
Journal of Crohns & Colitis | 2014
Daniela Rogler; Nicolas Fournier; Valérie Pittet; Patrick Bühr; Klaas Heyland; Michael Friedt; Rebekka Koller; Vanessa Rueger; Denise Herzog; Andreas Nydegger; Michela Schäppi; Susanne Schibli; Johannes Spalinger; Gerhard Rogler; Christian Braegger
BACKGROUND Inflammatory bowel disease (IBD) starting during childhood has been assumed to impair quality of life (QoL) of affected children. As this aspect is crucial for further personality development, the health-related quality of life (HRQOL) was assessed in a Swiss nationwide cohort to obtain detailed information on the fields of impairment. METHODS Data were prospectively acquired from pediatric patients included in the Swiss IBD Cohort Study. IBD activity was evaluated by PCDAI and PUCAI. The age adapted KIDSCREEN questionnaire was evaluated for 110 children with IBD (64 with Crohns disease 46 with ulcerative colitis). Data were analyzed with respect to established reference values of healthy controls. RESULTS In the KIDSCREEN index a moderate impairment was only found for physical wellbeing due to disease activity. In contrast, mental well-being and social support were even better as compared to control values. A subgroup analysis revealed that this observation was restricted to the children in the German speaking part of Switzerland, whereas there was no difference compared to controls in the French part of Switzerland. Furthermore, autonomy and school variables were significantly higher in the IBD patients as compared to controls. CONCLUSIONS The social support for children with IBD is excellent in this cohort. Only physical well-being was impaired due to disease activity, whereas all other KIDSCREEN parameters were better as compared to controls. This indicates that effective coping and support strategies may be able to compensate the burden of disease in pediatric IBD patients.
European Journal of Gastroenterology & Hepatology | 2014
Denise Herzog; Nicolas Fournier; Patrick Buehr; Rebekka Koller; Vanessa Rueger; Klaas Heyland; Andreas Nydegger; Johannes Spalinger; Susanne Schibli; Christian Braegger
Objectives Growth retardation is a frequent complication of paediatric inflammatory bowel disease (IBD). Only a few studies report the final height of these patients, with controversial results. We compared adult height of patients with paediatric IBD with that of patients with adult-onset disease. Methods Height data of 675 women 19–44 years of age and 454 men 23–44 years of age obtained at inclusion in the Swiss IBD cohort study registry were grouped according to the age at diagnosis: (a) prepubertal (men⩽13, women⩽11 years), (b) pubertal (men 13–22, women 11–18 years) and (c) adult (men>22, women>18 years of age), and compared with each other and with healthy controls. Results Male patients with prepubertal onset of Crohn’s disease (CD) had significantly lower final height (mean 172±6 cm, range 161–182) compared with men with pubertal (179±6 cm, 161–192) or adult (178±7 cm, 162–200) age at onset and the general population (178±7 cm, 142–204). Height z-scores standardized against heights of the normal population were significantly lower in all patients with a prepubertal diagnosis of CD (−0.8±0.9) compared with the other patient groups (−0.1±0.8, P<0.001). Prepubertal onset of CD emerged as a risk factor for reduced final height in patients with prepubertal CD. No difference for final height was found between patients with ulcerative or unclassified IBD diagnosed at prepubertal, pubertal or adult age. Conclusion Prepubertal onset of CD is a risk for lower final height, independent of the initial disease location and the necessity for surgical interventions.
Pediatric Gastroenterology, Hepatology & Nutrition | 2014
Denise Herzog; Patrick Buehr; Rebekka Koller; Vanessa Rueger; Klaas Heyland; Andreas Nydegger; Johannes Spalinger; Susanne Schibli; Christian Braegger
Purpose Gender differences in paediatric patients with inflammatory bowel disease (IBD) are frequently reported as a secondary outcome and the results are divergent. To assess gender differences by analysing data collected within the Swiss IBD cohort study database since 2008, related to children with IBD, using the Montreal classification for a systematic approach. Methods Data on gender, age, anthropometrics, disease location at diagnosis, disease behaviour, and therapy of 196 patients, 105 with Crohns disease (CD) and 91 with ulcerative or indeterminate colitis (UC/IC) were retrieved and analysed. Results The crude gender ratio (male : female) of patients with CD diagnosed at <10 years of age was 2.57, the adjusted ratio was 2.42, and in patients with UC/IC it was 0.68 and 0.64 respectively. The non-adjusted gender ratio of patients diagnosed at ≥10 years was 1.58 for CD and 0.88 for UC/IC. Boys with UC/IC diagnosed <10 years of age had a longer diagnostic delay, and in girls diagnosed with UC/IC >10 years a more important use of azathioprine was observed. No other gender difference was found after analysis of age, disease location and behaviour at diagnosis, duration of disease, familial occurrence of IBD, prevalence of extra-intestinal manifestations, complications, and requirement for surgery. Conclusion CD in children <10 years affects predominantly boys with a sex ratio of 2.57; the impact of sex-hormones on the development of CD in pre-pubertal male patients should be investigated.
Archives of Disease in Childhood | 2013
Denise Herzog; Markus A. Landolt; Patrick Buehr; Klaas Heyland; Daniela Rogler; Rebekka Koller; Vanessa Rueger; Valérie Pittet; Andreas Nydegger; Johannes Spalinger; Michela Schäppi; Susanne Schibli; Christian Braegger
Objectives Whether behavioural and emotional maladjustment is more prevalent in children with inflammatory bowel disease (IBD) than in healthy controls remains controversial. The aim of this study was to assess paediatric IBD patients for problems with emotional and behavioural adjustment and to examine associations with clinical and demographic variables. Methods Data from paediatric patients with IBD enrolled in the Swiss IBD Cohort Study and the results of both the parent-rated Strengths and Difficulties Questionnaire (SDQ) and the self-reported Child Depression Inventory (CDI) were analysed. Of the 148 registered patients, 126 had at least one questionnaire completed and were included. Results The mean age of 71 patients with Crohns disease (44 males, 27 females) was 13.4 years, and 12.8 years for the 55 patients with ulcerative or indeterminate colitis. The mean duration of disease was 1.2 and 2.7 years, respectively. The total score of the SDQ was abnormal in 11.4% of cases compared to 10% in the normal population. Abnormal sub-scores were found in 20.2% of subjects for the domain of emotional problems and in 17.1% for problems with peers. The total CDI T score indicated a significantly lower prevalence of clinical depression in IBD patients than in normal youth. No correlation between the total SDQ scores or the CDI T scores and gender, type or duration of IBD, inflammatory markers or disease scores was found. Conclusions The prevalence of problems with behavioural and emotional adjustment among Swiss paediatric IBD patients is low and comparable to that of the normal population.
Inflammatory Bowel Diseases | 2016
Sophie Yammine; Sylvia Nyilas; Carmen Casaulta; Susanne Schibli; Philipp Latzin; Christiane Sokollik
Background:Extraintestinal manifestations are common among patients with inflammatory bowel disease (IBD), whereas pulmonary involvement is considered rare. However, chronic lung diseases begin with subclinical changes of the small airways and often originate in childhood. Pulmonary involvement, particularly of the small airways, can be assessed using novel inert gas washout tests. Methods:In this prospective, single-center study, 30 children and adolescents (mean age, 14 years; SD, ±2.6; 13 boys) with IBD (mean disease duration, 3.2 years; SD, ±2.8), and 32 healthy age-matched controls, performed nitrogen multiple-breath washout, double-tracer gas single-breath washout, and diffusion capacity for carbon monoxide. Patients with IBD additionally performed spirometry, plethysmography, and measurement of exhaled nitric oxide. Results:Patients with IBD demonstrated no abnormalities in classical lung function tests. There was no difference between active disease and remission. The lung clearance index, a very sensitive indicator for small airway function, did not differ between patients with IBD and healthy controls (mean difference [95% confidence interval] −0.01 [−0.28 to 0.25]). Specific markers for peripheral lung ventilation (Sacin and Scond) were also within the normal range (0.002 [−0.003 to 0.008] and −0.002 [−0.020 to 0.015], respectively). No association was found between measures of lung function and IBD subtype, clinical disease activity scores, laboratory values, treatment modalities, or disease duration. Conclusions:In our cohort of pediatric and adolescent patients with IBD without respiratory symptoms, there was no evidence of significant lung disease on extensive screening testing. General screening of asymptomatic patients therefore appears unnecessary and is not recommended in this population.