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Lu B.R.,Section of Pediatric Gastroenterology | Lu B.R.,Stanford University | Esquivel C.O.,Stanford University
Pediatric Transplantation

With advances in medical treatments, patients with CF are having improved quality of life and living longer. Although pulmonary disease is still the leading cause of morbidity and mortality, this longevity has allowed for the development of other organ dysfunction, mainly liver and pancreas. This review discusses the abdominal organ complications and the role of abdominal organ transplantation in CF. Liver failure and portal hypertension complications are the most common indicators for liver transplantation in CF, and five-yr survival for isolated liver transplantation is >80%. Deficiency of pancreatic enzymes is almost universal and up to 40% of patients with CF can develop insulin-dependent diabetes, although the role of pancreas transplantation is less clear and needs further research. Finally, the need for lung transplantation should always be assessed and considered in combination with liver transplantation on a case-by-case basis. © 2010 John Wiley & Sons A/S. Source

Guandalini S.,Section of Pediatric Gastroenterology
Expert Review of Clinical Immunology

Probiotics have had many applications in the past few years, and inflammatory conditions of the GI tract - including chronic disorders such as inflammatory bowel disease (IBD) - have received the most attention by investigators. In fact, the experimental basis to expect clinical efficacy of probiotics in IBD is quite robust. In spite of this however, only minimal evidence of benefit by any probiotic is currently available in Crohn's disease, either in adult or in pediatric populations. In ulcerative colitis, on the other hand, several probiotic formulations and especially the proprietary preparation VSL#3 (a high-concentration mixture) have been found effective as adjuvant therapy, both in inducing and maintaining remission. © 2010 Expert Reviews Ltd. Source

Jericho H.,Section of Pediatric Gastroenterology | Adams P.,Ann and Robert H. Lurie Childrens Hospital of Chicago | Zhang G.,Ann and Robert H. Lurie Childrens Hospital of Chicago | Rychlik K.,Ann and Robert H. Lurie Childrens Hospital of Chicago | Saps M.,Ann and Robert H. Lurie Childrens Hospital of Chicago
Journal of Pediatrics

Objectives To assess whether the gastroparesis cardinal symptom index (GCSI), or any individual symptom, is associated with delayed gastric emptying in children, and to assess understanding of symptoms associated with delayed gastric emptying. Study design Fifty children (36 F), 5-18 years of age, undergoing gastric emptying scintigraphy (GES) at Lurie Children's Hospital in Chicago, Illinois, completed Likert-type GCSI and symptom comprehension questionnaires. Correlation of GES results (normal or abnormal) with questionnaire results using the Wilcoxon rank sum test. Results Seventy percent of subjects had a normal GES. Children reported understanding most terms of GCSI (average score 2.59, range 0-3). The GCSI was not associated with delayed gastric emptying. Nausea was associated with delayed gastric emptying only (numerical P =.04, word P =.02). Results were not altered when poorly understood terms were excluded. Conclusions The GCSI is not associated with delayed gastric emptying in children. Lack of association does not seem to be related to lack of understanding. Nausea alone was the only symptom that showed an association with delayed gastric emptying on GES. © Copyright 2014 Mosby Inc. All rights reserved. Source

Lifschitz C.,Section of Pediatric Gastroenterology
Acta Scientiarum Polonorum, Technologia Alimentaria

The purpose of this review is to present information regarding new effects for certain nutrients other than those traditionally known. Zinc has been found to prevent and reduce the duration of common colds. In developing countries, zinc has been shown to reduce the duration and severity of diarrhea and even decrease relapses. Iron supplementation in iron deficient children, has been shown to improve several aspects of brain function. In studies where iron was given to the mother, 3 of 5 randomized, controlled trials showed a beneficial effect of iron supplementation on the Psychomotor Development Index at some time points, whereas 2 did not. The chances for infants supplemented with docosahexaenoic acid in the first year of life of having at least 1 event of allergic manifestation or upper respiratory infection or at least 1 event of wheezing/asthma, wheezing/asthma/atopic dermatitis, any allergy, or an upper respiratory tract infection during the first 3 years of life were significantly lower than in the non supplemented group. Epidemiological studies have established a relationship between low levels of serum vitamin D and reduced lung function in healthy adults and asthma onset and severity in children. There was a trend for an independent association between higher levels of maternal circulating 25(OH)D3 levels in pregnancy and decreased odds of lower respiratory tract infections in offspring. © Copyright by Wydawnictwo Uniwersytetu Przyrodniczego w Poznaniu. Source

Septer S.,Section of Pediatric Gastroenterology | Slowik V.,Section of Pediatrics | Morgan R.,Georgetown University | Dai H.,Childrens Mercy Hospital | Attard T.,Section of Pediatric Gastroenterology
Hereditary Cancer in Clinical Practice

Background: Lifetime risk of thyroid cancer associated with FAP has been reported as 1-2%. The mean age at diagnosis of thyroid carcinoma in FAP has been reported at 28 years. The aims of this paper are to better understand gene mutations associated with thyroid cancer and refine surveillance recommendations for patients with FAP. Methods: We performed a search in Pubmed, Ovid Medline and Embase with the terms ("Thyroid Gland"[Mesh] OR "Thyroid Neoplasms"[Mesh]) AND "Adenomatous Polyposis Coli"[Meshdenomatous Polyposis Coli"[Mesh] to identify subjects with thyroid cancer and FAP. As a reference group for APC mutations in the unselected FAP population, we used the UMD-APC database referenced in the Orphanet portal, which includes APC mutation data on 2040 individuals with FAP. Results:There were 115 reported cases of thyroid cancer in patients with FAP (95 female: 11 male) with an average age of 29.2 years. Gene mutation testing results were reported in 48 patients. On comparing the prevalence of APC mutation in the population of FAP patients with thyroid cancer and the prevalence of the same mutation in the reference population an increased odds ratio was evident in individuals harboring an APC mutation at codon 1061 (OR: CI 4.1: 1.7-8.9). Analysis of the prevalence of thyroid cancer in individuals with FAP segregated by the region of the gene affected shows an increased risk of thyroid cancer in individuals harboring mutations proximal to codon 512 (OR 2.6, p 0.0099). Conclusions: There is increased risk for thyroid cancer in individuals with APC mutations at the 5' end (proximal to codon 528) along with the established high risk group harboring mutation at codon 1061. It is suggested that these patients might benefit from directed surveillance by annual ultrasound from age 18 years onwards. © 2013 Septer et al.; licensee BioMed Central Ltd. Source

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