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Although children in general and patients with type 1 diabetes mellitus, in particular, are exempted from fasting during Ramadan, many elect to observe the fast. There is a sizeable amount of opinion and research data that warrants revisiting. This is a narrative nonsystematic review to explore the views and supporting data on Ramadan fasting and to examine the safety of fasting and its impact on diabetes control and management in children and adolescents. The key areas covered include epidemiology, the physiology of fasting, risk stratification, counseling strategy, nutrition advice, insulin therapy adjustment with a particular focus on multiple injection regimen, and insulin pump therapy. Findings from various studies and expert opinions were appraised and presented to illustrate points of agreements and differences. This review should enhance knowledge and form the basis to clear some doubts and differences of opinions surrounding the issue of diabetes and Ramadan fasting in young people. It should also empower healthcare professionals to develop consensus based on the most up-to-date advice and the best possible support to patients and families regarding fasting during Ramadan. [ABSTRACT FROM AUTHOR]

Neonatal diabetes and hypothyroidism (NDH) syndrome was first described in 2003 in a consanguineous Saudi Arabian family where two out of four siblings were reported to have presented with proportionate IUGR, neonatal non-autoimmune diabetes mellitus, severe congenital hypothyroidism, cholestasis, congenital glaucoma, and polycystic kidneys. Liver disease progressed to hepatic fibrosis. The renal disease was characterized by enlarged kidneys and multiple small cysts with deficient cortico-medullary junction differentiation and normal kidney function. There was minor facial dysmorphism (depressed nasal bridge, large anterior fontanelle, long philtrum) reported but no facial photographs were published. Mutations in the transcription factor GLI-similar 3 ( GLIS3) gene in the original family and two other families were subsequently reported in 2006. All affected individuals had neonatal diabetes, congenital hypothyroidism but glaucoma and liver and kidney involvement were less consistent features. Detailed descriptions of the facial dysmorphism have not been reported previously. In this report, we describe the common facial dysmorphism consisting of bilateral low-set ears, depressed nasal bridge with overhanging columella, elongated, upslanted palpebral fissures, persistent long philtrum with a thin vermilion border of the upper lip in a cohort of seven patients with GLIS3 mutations and report the emergence of a distinct, probably recognizable facial gestalt in this group which evolves with age. © 2016 Wiley Periodicals, Inc. [ABSTRACT FROM AUTHOR]

Background: Raising the awareness of childhood diabetes symptoms can reduce the frequency of diabetic ketoacidosis (DKA) at onset of type 1 diabetes (T1D). However, data on the effectiveness of such interventions are limited. The aim of the study was to describe trends of DKA at onset of childhood T1D during 2005-2014 and assess the impact of a diabetes awareness campaign launched late 2010. Methods: Data of children < 12 years presented with DKA at diagnosis were analyzed according to age, gender and year of diagnosis. The frequency and severity of DKA before and during the 4 years campaign were compared. Results: During 2005-2014, 44.9% (243/541) of children diagnosed with T1D presented with DKA. Of these, 22.7% had pH < 7.1. In both genders DKA was higher in children < 6 years (47.8% vs. 40%; p < 0.01) and more severe in < 3 years old compared to older children (30% vs. 20%; p < 0.01). Following the awareness campaign DKA rate dropped from 48% in 2010 to 39% in 2014 and 15.8% had severe DKA compared to 26.1% in 2005-2010 (p < 0.01). This trend was observed in both genders and across age groups. In children < 3 years the reduction in DKA frequency and severity was not statistically significant (p = 0.15 and p = 0.42, respectively). Conclusions: In NWSA, the frequency and severity of DKA at onset of childhood T1D were reduced following 4 years awareness campaign; but the rate is still high. Maintaining the campaign may result in further improvement following a longer period of observation. [ABSTRACT FROM AUTHOR]

Background: Wolcott-Rallison syndrome (WRS) is caused by recessive EIF2AK3 gene mutations and characterized by permanent neonatal diabetes (PNDM), skeletal dysplasia, and recurrent hepatitis. The frequency of this rare syndrome is largely unknown. Objectives: To define the frequency and spectrum of WRS in the Kingdom of Saudi Arabia (KSA) based onpublished data. Methods: The Medline database was searched for published articles on WRS. The number of reported cases from KSA was compared to the total number of WRS cases reported worldwide. The genotype and phenotype of WRS patients from KSA were reviewed. Results: Ten articles describing 23 WRS patients from 12 Saudi families from 1995 to 2012 were identified. This figure accounts for 27.7% (23/83) of the patients and 22.2% (12/54) of the families with WRS reported worldwide until January 2013. All Saudi patients with WRS presented with PNDM, and they represent 59% of all PNDM cases from WRS. At reporting, 73% of patients experienced recurrent hepatitis, 56.5% had skeletal abnormalities, and 39.1% of them were dead. There was a variation in the phenotype even between affected siblings. Genetic diagnosis was confirmed in all 12 families with no correlation between the genotype and phenotype. Eight of the nine EIF2AK3 mutations were only reported in these families, and one was shared with a patient from Qatar, a neighboring Arab state. Conclusions: No study on the frequency of WRS has been published. However, the available data indicate that KSA has the largest collection of patients with WRS worldwide, and nine of the identifiable EIF2AK3 mutations appear to be confined to Arabs. Establishing a national or international registry for WRS would provide more reliable data on this rare condition. [ABSTRACT FROM AUTHOR]