J Pediatr Endocrinol Metab 2018; aop Lakshmipathi Naik Banavath, Rakesh Kumar*, Devi Dayal, Jaivinder Yadav, Naresh Sachdeva, Joseph L. Mathew, Pankaj C. Vaidya and Meenu Singh Glucose intolerance in children with cystic fibrosis: a developing country’s perspective https://doi.org/10.1515/jpem-2018-0222 Received May 19, 2018; accepted August 27, 2018 Abstract Background: Cystic fibrosis-related diabetes (CFRD) is a common comorbidity reported in patients with cystic fibrosis (CF). There is a dearth of data on glucose intoler- ance or CFRD in children with CF from developing coun- tries. So, we planned to study the prevalence of abnormal glucose tolerance (AGT) in children with CF and its rela- tion with the duration and severity of CF. Methods: We performed an oral glucose tolerance test (OGTT) on children (2–18 years old) having CF for at least 6 months. Two-hour plasma glucose levels on OGTT were correlated with various disease-related factors. Results: Out of the 25 children enrolled, there were 18 boys and seven girls. The mean age and duration of CF were 7.9 ± 4.3 and 3.16 ± 2.5 years, respectively. AGT was observed in 16 (64%) children with CF including three (12%) children with CFRD. Children with a duration of CF of 3 years had significantly higher prevalence (81.8%) of AGT when compared with duration ≤3 years (p-value < 0.05). Twelve out of 17 (70.6%) children were colonized with Pseudomonas and 12 out of 15 (80%) children >6 years of age had AGT. There was a positive correlation of 2-h glucose value on OGTT with duration of CF and number of hospitalizations with acute pulmonary exacerbations. Conclusions: The majority of children having CF for >3 years and/or age >6 years developed AGT. In our clini- cal setting, an annual screening with OGTT to detect AGT may be required at an early age and duration of CF. Keywords: CFRD; children; cystic fibrosis; glucose intoler- ance; OGTT. Introduction Cystic fibrosis (CF) is the most common genetic autosomal recessive disease among Caucasians with an estimated prevalence of 1/3400 live births in the United States, 1/3600 in Canada and 1/2400 in the United Kingdom [1]. In India, the estimated prevalence of CF is 1/43,000–1/100,000 [2]. Due to the increase in the lifespan of patients with CF, due to advances in medical and nutritional therapy and better control of acute pulmonary infections over the last few decades, CF-related comorbidities have become more fre- quent during the patients’ lifetime. Cystic fibrosis-related diabetes (CFRD) is the most common comorbidity seen in 2% of children, 19% of adolescents and 40%–50% of adults as per recent western data [3]. The International Society for Pediatric and Adolescent Diabetes (ISPAD) recommends annual screening with oral glucose toler- ance test (OGTT) for CFRD in all children with CF begin- ning by 10 years of age [4]. Undernutrition, poor pulmonary status and female gender are identified as contributing factors for the worsen- ing course of the disease and the development of CFRD [5]. Females with CF have a 3.5-fold higher risk of developing CFRD [5]. In India, children with CF are diagnosed late due to a low index of suspicion and scarcity of available diag- nostic facilities [6]. Further, children with CF in India have more frequent pulmonary exacerbations due to undernutri- tion, poor infection control measures and increased airway bacterial colonization rate [7]. All these factors could lead to early onset of insulin resistance and increased preva- lence of abnormal glucose tolerance (AGT) among children with CF in India and other developing countries. There is only one study from India that has reported the preva- lence of AGT in children with CF above 6 years of age [8]. We planned this study to assess the prevalence of AGT in *Corresponding author: Dr. Rakesh Kumar, MD, Pediatric Endocrinology and Diabetes Division, Advanced Pediatrics Centre (APC), Postgraduate Institute of Medical Education and Research (PGIMER), Sector 12, Chandigarh 160012, India, Phone: +91-7087008480, E-mail: drrakesh.pgi@gmail.com. http://orcid.org/0000-0002-0039-2142 Lakshmipathi Naik Banavath, Devi Dayal and Jaivinder Yadav: Pediatric Endocrinology and Diabetes Division, Postgraduate Institute of Medical Education and Research (PGIMER), Sector 12, Chandigarh, India. http://orcid.org/0000-0002-7026-3469 (J. Yadav) Naresh Sachdeva: Department of Endocrinology, Postgraduate Institute of Medical Education and Research (PGIMER), Sector 12, Chandigarh, India Joseph L. Mathew, Pankaj C. Vaidya and Meenu Singh: Pediatric Pulmonology Division, Postgraduate Institute of Medical Education and Research (PGIMER), Sector 12, Chandigarh, India Brought to you by | University of Sussex Library Authenticated Download Date | 9/24/18 7:51 PM