Carlos Bousoño García

The Importance of Adherence and Compliance with Treatment in Cystic Fibrosis

The therapeutic requirements of patients with CF are highly complex. Many patients require continuous care at home with many prophylactic medications, such as nebulised or oral antibiotics, pancreatic enzymes, mucolytic agents, vitamin and nutritional supplements as well as daily physiotherapy and a healthy lifestyle with adequate nutrition and exercise. These treatments are intensified and become more complicated during the exacerbation of the disease. Throughout its evolution, other pathologies associated with CF may also occur which require extra treatment regimens.

Manejo de la alergia a proteína de leche de vaca por los gastroenterólogos españoles

INTRODUCTION Food allergy is an increasing health problem in the developed world. Cows milk protein is the main cause of food allergy in infants. Without an appropriate diagnostic workup, there is a high risk of both over- and underdiagnosis and therefore, over and undertreatment. The objective of our study was to analyze the variability in cows milk protein allergy (CMPA) management by pediatric gastroenterologists in Spain. METHODS A fifty item questionnaire, including open and closed items in a Likerts scale from 0 to 5, was drafted and distributed through the Spanish Society for Pediatric Gastroenterology, Hepatology and Nutrition (SEGHNP) e-mail list. RESULTS Seventy-three questionnaires were received back out of 321. Only 3 of the items achieved concordance greater than 90%. Thirty-three percent considered oral challenge to be necessary for the diagnosis of CMPA under any circumstance. Twenty-five percent considered that symptom improvement after cows milk removal was enough for the diagnosis. Oral challenge was performed at home by 83.5% in non-IgE mediated cases. Extensively hydrolyzed casein formulas were the treatment of choice for 69.9%. Soy formulas were the last option. Almost all respondents were aware of the existence of clinical guidelines on CMPA, being European Society of Pediatric Gastroenterology, Hepatology and Nutrition guidelines the most followed (64.4%). Twenty-three percent considered that their knowledge about allergy was inadequate. CONCLUSIONS Although CMPA is a prevalent condition that pediatric gastroenterologists have been treating for decades, we found a huge variability on its management. There is potential for improvement in this field among pediatric gastroenterologist in the future.

Alergia a proteínas de leche de vaca con afectación cutánea, ¿es necesaria la derivación al dermatólogo?

1. Pascual Pérez AI, Méndez Sánchez A, Segarra Cantón O, Espin Jaime B, Santiago Jiménez Treviño S, Bousoño García C, et al. Manejo de la alergia a proteína de leche de vaca por los gastroenterólogos españoles. An Pediatr (Barc). 2018;89:222--9. 2. Husain Z, Schwartz RA. Food allergy update: More than a peanut of a problem. Int J Dermatol. 2013;52:286--94. 3. Martorell-Aragonés A, Echeverría-Zudaire L, Alonso-Lebrero E, Boné-Calvo J, Martín-Muñoz MF, Nevot-Falcó S, et al., Food allergy committee of SEICAP (Spanish Society of Pediatric Allergy, Asthma and Clinical Immunology). Position document: IgE-mediated cow’s milk allergy. Allergol Immunopathol (Madr). 2015;43:507--26. 4. Longo G, Berti I, Burks AW, Krauss B, Barbi E. IgE-mediated food allergy in children. Lancet. 2013;382:1656--64.

2016: epidemia de enfermedad inflamatoria intestinal en Asturias

The term epidemic refers to the occurrence of a number of cases of a specific disease that exceeds the expected incidence in a given geographical area. The Hospital Universitario Central de Asturias (HUCA) is the regional referral centre for inflammatory bowel disease (IBD) for the paediatric population of the Autonomous Community of Asturias, Spain. Historically, the department of paediatric gastroenterology diagnosed an average of 2 new cases of IBD each year. However, in year 2016 the department diagnosed 15 new cases. With the aim of finding an explanation to this striking increase in incidence, we reviewed the health records of children (aged 0--14 years) that received a diagnosis of IBD in the paediatric gastroenterology outpatient clinics of the HUCA between December 2015 and January 2017. We summarised the epidemiological, clinical and laboratory characteristics of these cases and compared them with those from previous years. Fifteen new cases were diagnosed in the period under study. The mean age at diagnosis was 10 years, and one patient had onset at the atypically early age of 19 months. In previous years, the outpatient clinics of the HUCA had managed 23 patients with a median age of 11 years, the youngest of whom was 2 years old. The increase in the incidence of IBD was of approximately 650%, from 2 per 100,000 to 2 per 10,000 inhabitants per year. In 2016, 46.7% of new cases were diagnosed in male patients compared to 74% in the group of cases diagnosed in previous years (P = .03). In 2016, most of the patients that received the diagnosis resided in an urban area (66.6%), and Oviedo and Gijón were the cities where the incidence increased most, while in previous year, the distribution of cases was more dispersed throughout the entire region of Asturias. In 2016, Crohn disease (CD) accounted for 66.7% of the cases, and the most frequent presenting symptoms were bloody stools and abdominal pain. In previous years, the distribution of

Insulin Resistance in Cystic Fibrosis: Management

Abstract Cystic fibrosis-related diabetes is the most common comorbidity in subjects with cystic fibrosis. The hallmark of cystic fibrosis-related diabetes is the reduction of β-cell mass, leading to insulin insufficiency. However, insulin resistance (IR) may arise from decreased glucose uptake by muscle (peripheral IR) and through impaired suppression of hepatic glucose production (hepatic IR). Increased prevalence of overweight and obesity, sedentary lifestyle, high-calorie diets, fat-soluble vitamin deficiency, elevated oxidative stress, and increased life expectancy are reasons to believe that IR can achieve an important role in the development of glucidic disorders and macrovascular complications previously recognized in patients with cystic fibrosis. In this chapter we explain the principal mechanisms of IR in cystic fibrosis and propose preventive and nutritional management strategies for this problem.