Diseases

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Open AccessReview

Auxo-Endocrinological Approach to Celiac Children

by Mauro Bozzola, Cristina Meazza and Alberto Villani

Diseases 2015, 3(2), 111-121; https://doi.org/10.3390/diseases3020111 - 19 Jun 2015

Cited by 2 | Viewed by 5135

Celiac disease is a permanent genetically determined intolerance to gluten that generally presents with gastrointestinal symptoms in young children and extraintestinal manifestations (endocrinological, dermatological, neurological, etc.) later. Furthermore, many studies demonstrate the close association between celiac and endocrine diseases, including growth and [...] Read more.

Celiac disease is a permanent genetically determined intolerance to gluten that generally presents with gastrointestinal symptoms in young children and extraintestinal manifestations (endocrinological, dermatological, neurological, etc.) later. Furthermore, many studies demonstrate the close association between celiac and endocrine diseases, including growth and pubertal disorders, type I diabetes mellitus and autoimmune thyroid diseases, probably due to the presence of a common genetic predisposition. Follow-up for celiac children after the start of gluten-free diet is mandatory to avoid complications such as growth hormone deficiency. The present review deals with the problem of the diagnosis of endocrine-associated diseases in celiac children and gives suggestions for correct management and follow-up of these patients. Full article

(This article belongs to the Special Issue Celiac Disease)

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Open AccessOpinion

The Perceived Social Burden in Celiac Disease

by Carolina Ciacci and Fabiana Zingone

Diseases 2015, 3(2), 102-110; https://doi.org/10.3390/diseases3020102 - 19 Jun 2015

Cited by 21 | Viewed by 7301

Abstract

In the present paper, we discuss the change in celiac disease (CD) awareness and perception through patients’ concerns and the most recent literature. Nowadays CD has moved in the public awareness (both doctors and population) from a rare disease to a common one [...] Read more.

In the present paper, we discuss the change in celiac disease (CD) awareness and perception through patients’ concerns and the most recent literature. Nowadays CD has moved in the public awareness (both doctors and population) from a rare disease to a common one and the gluten free diet (GFD) is no longer the exclusive therapy for CD patients but is becoming a popular health choice for everybody. Gluten-free food, once hard to find and requiring home preparation, is now available at restaurants and grocery stores. However, the quality of life of those affected by CD seems to be still compromised and this is particularly true for those who find it difficult to adhere to a GFD and those who were asymptomatic at the time of diagnosis. Intervention at diagnosis and follow-up to improve the patients’ adaptation to the condition and its limitations should be implemented. Full article

(This article belongs to the Special Issue Celiac Disease)

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Open AccessReview

Celiac Disease: Diagnostic Standards and Dilemmas

by Dharmesh H. Kaswala, Gopal Veeraraghavan, Ciaran P. Kelly and Daniel A. Leffler

Diseases 2015, 3(2), 86-101; https://doi.org/10.3390/diseases3020086 - 16 Jun 2015

Cited by 16 | Viewed by 18766

Abstract

Celiac Disease (CD) affects at least 1% of the population and evidence suggests that prevalence is increasing. The diagnosis of CD depends on providers being alert to both typical and atypical presentations and those situations in which patients are at high risk for [...] Read more.

Celiac Disease (CD) affects at least 1% of the population and evidence suggests that prevalence is increasing. The diagnosis of CD depends on providers being alert to both typical and atypical presentations and those situations in which patients are at high risk for the disease. Because of variable presentation, physicians need to have a low threshold for celiac testing. Robust knowledge of the pathogenesis of this autoimmune disease has served as a catalyst for the development of novel diagnostic tools. Highly sensitive and specific serological assays including Endomysial Antibody (EMA), tissue transglutaminase (tTG), and Deamidated Gliadin Peptide (DGP) have greatly simplified testing for CD and serve as the foundation for celiac diagnosis. In addition, genetic testing for HLA DQ2 and DQ8 has become more widely available and there has been refinement of the gluten challenge for use in diagnostic algorithms. While diagnosis is usually straightforward, in special conditions including IgA deficiency, very young children, discrepant histology and serology, and adoption of a gluten free diet prior to testing, CD can be difficult to diagnose. In this review, we provide an overview of the history and current state of celiac disease diagnosis and provide guidance for evaluation of CD in difficult diagnostic circumstances. Full article

(This article belongs to the Special Issue Celiac Disease)

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Open AccessReview

Medication Trials for Hyperphagia and Food-Related Behaviors in Prader–Willi Syndrome

by Jennifer L. Miller, Theresa V. Strong and Janalee Heinemann

Diseases 2015, 3(2), 78-85; https://doi.org/10.3390/diseases3020078 - 03 Jun 2015

Cited by 16 | Viewed by 11388

Abstract

Prader-Willi syndrome (PWS) is a neurodevelopmental disorder caused by the absence of paternally expressed, imprinted genes on chromosome 15q11-13. Individuals with PWS characteristically have poor feeding and lack of appetite in infancy, followed by the development of weight gain and then uncontrolled appetite [...] Read more.

Prader-Willi syndrome (PWS) is a neurodevelopmental disorder caused by the absence of paternally expressed, imprinted genes on chromosome 15q11-13. Individuals with PWS characteristically have poor feeding and lack of appetite in infancy, followed by the development of weight gain and then uncontrolled appetite and lack of satiety, sometime after the age of two. The overwhelming drive to eat is coupled with reduced energy expenditure and decreased caloric requirements, thus, individuals with PWS will become severely obese unless their food intake is strictly controlled. The mechanisms underlying hyperphagia in PWS remain incompletely understood, and to date no drugs have proven effective in controlling appetite. However, clinical trials have started for several medications, which may provide therapeutic options for those with PWS. These medication trials may also provide insight into potential treatments for obesity in the general population. Ideally, these treatments will help alleviate the complex metabolic issues that are part of this syndrome. Full article

(This article belongs to the Special Issue Prader-Willi Syndrome)

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Open AccessArticle

Factors Associated with Post-Progression Survival in Patients with Advanced Hepatocellular Carcinoma Treated with Sorafenib

by Taiga Otsuka, Shunya Nakashita, Kimihiko Yanagita, Keisuke Ario, Hiroaki Kawasoe, Seiji Kawazoe, Yuichiro Eguchi and Toshihiko Mizuta

Diseases 2015, 3(2), 68-77; https://doi.org/10.3390/diseases3020068 - 15 May 2015

Cited by 3 | Viewed by 4941

Abstract

Sorafenib exerts modest antitumor activity in patients with advanced hepatocellular carcinoma (HCC), and radiological progressive disease (rPD) does not always correspond to so-called clinical progressive disease (cPD). We evaluated 101 patients who initiated sorafenib treatment for HCC and assessed post-progression survival (PPS) using [...] Read more.

Sorafenib exerts modest antitumor activity in patients with advanced hepatocellular carcinoma (HCC), and radiological progressive disease (rPD) does not always correspond to so-called clinical progressive disease (cPD). We evaluated 101 patients who initiated sorafenib treatment for HCC and assessed post-progression survival (PPS) using the Cox proportional hazards model. PPS was calculated from the date of the first rPD until the date of death or the last follow-up. Using Cox model analysis of the 76 patients who experienced first rPD, we identified the Child-Pugh class, Eastern Cooperative Oncology Group performance status, the best antitumor response during treatment (using Response Evaluation Criteria in Solid Tumors (RECIST) Version 1.1) and α-fetoprotein levels as independent factors affecting PPS. When these factors were used to define scores ranging from zero to five with a cutoff value of two, PPS of patients who received best supportive care (BSC) after rPD was not statistically significantly different from that of patients who received post-rPD therapy with scores ≥2 (p = 0.220). In contrast, the PPS for the post-rPD therapy group was significantly longer compared with the BSC patients with scores <2 (p < 0.001). Patients who scored ≥2 at their first rPD were judged cPD and as candidates for BSC. Full article

(This article belongs to the Special Issue Targeted Therapy of Hepatocellular Carcinoma: Present and Future)

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Open AccessReview

Growth Hormone Therapy in Adults with Prader-Willi Syndrome

by Karen S. Vogt and Jill E. Emerick

Diseases 2015, 3(2), 56-67; https://doi.org/10.3390/diseases3020056 - 16 Apr 2015

Cited by 9 | Viewed by 7507

Abstract

Prader-Willi syndrome (PWS) is characterized by hyperphagia, obesity if food intake is not strictly controlled, abnormal body composition with decreased lean body mass and increased fat mass, decreased basal metabolic rate, short stature, low muscle tone, cognitive disability, and hypogonadism. In addition to [...] Read more.

Prader-Willi syndrome (PWS) is characterized by hyperphagia, obesity if food intake is not strictly controlled, abnormal body composition with decreased lean body mass and increased fat mass, decreased basal metabolic rate, short stature, low muscle tone, cognitive disability, and hypogonadism. In addition to improvements in linear growth, the benefits of growth hormone therapy on body composition and motor function in children with PWS are well established. Evidence is now emerging on the benefits of growth hormone therapy in adults with PWS. This review summarizes the current literature on growth hormone status and the use of growth hormone therapy in adults with PWS. The benefits of growth hormone therapy on body composition, muscle strength, exercise capacity, certain measures of sleep-disordered breathing, metabolic parameters, quality of life, and cognition are covered in detail along with potential adverse effects and guidelines for initiating and monitoring therapy. Full article

(This article belongs to the Special Issue Prader-Willi Syndrome)

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Open AccessReview

Helicobacter pylori-Mediated Protection against Extra-Gastric Immune and Inflammatory Disorders: The Evidence and Controversies

by Karen Robinson

Diseases 2015, 3(2), 34-55; https://doi.org/10.3390/diseases3020034 - 27 Mar 2015

Cited by 19 | Viewed by 8014

Abstract

A large number of studies link H. pylori infection with a reduced risk of developing extra-gastric conditions such as allergy, asthma, inflammatory bowel disease, coeliac disease and multiple sclerosis. The strength of the evidence for these protective associations is quite variable, and published [...] Read more.

A large number of studies link H. pylori infection with a reduced risk of developing extra-gastric conditions such as allergy, asthma, inflammatory bowel disease, coeliac disease and multiple sclerosis. The strength of the evidence for these protective associations is quite variable, and published studies often do not agree. This review article discusses some of the reasons for these discrepancies, and the difficulties faced when designing studies. Examples of some protective disease associations are described in detail, where the evidence is most abundant and thought to be more reliable. The most convincing of these are supported by published mechanistic data, for example with animal models, or incidence of disease exacerbation in humans following H. pylori eradication. Although controversial, this field is very important as the prevalence of H. pylori is decreasing throughout the world whilst many chronic diseases are becoming more common. These trends are likely to continue in the future, therefore it is important that we fully understand if and how H. pylori confers protection. Full article

(This article belongs to the Special Issue Helicobacter Pylori Infection)

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Diseases, Volume 3, Issue 2 (June 2015) – 7 articles , Pages 34-121

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