Background A wide range of reports on the incidence of diabetic ketoacidosis (DKA) at the onset of type 1 diabetes mellitus (T1DM) in children have been published worldwide. Reports from Saudi Arabia are limited. The aim of this study was to assess the incidence, clinical pattern and severity of DKA in children with newly diagnosed T1DM and the association of autoimmune conditions with initial DKA occurrence at King Abdulaziz Medical City – Jeddah. Methods This retrospective chart review was conducted during the period 2005–2015. All newly diagnosed T1DM children during the study period were investigated (n = 390). Data were collected on the demographic characteristics, body mass index (BMI), DKA severity, length of hospital stay and follow-up data on the type of diabetes therapy. Results The incidence of DKA among newly diagnosed T1DM pediatric patients was 37.7% (n = 147). Moderate and severe DKA cases were significantly higher among female children (p = 0.04). Patients diagnosed with DKA had lower BMI (20.87 ± 5.21) than their counterparts (p = 0.03). The median length of hospital stay was higher among severe DKA compared to moderate and mild cases (5.0, 4.5 and 4.0 days, respectively). Conclusions The incidence of DKA among newly diagnosed T1DM is still high compared to developed countries; however, it is relatively lower than previous reports in Saudi Arabia. Immediate interventions, such as awareness campaigns, are vital to reduce the burden of this preventable health sequela among children with DM.

Rationale:Alström syndrome is an autosomal recessive disorder characterized by hearing loss, blindness, obesity, non-insulin dependent diabetes, and others.Patient concern:A 10 years old Saudi girl, who presented with diabetic ketoacidosis and found to have hearing loss and blindness.Diagnosis:Alström syndrome.Interventions:Multidisciplinary team approach, with echocardiography, hearing test, eye exam and genetic test for Alström syndrome.Outcomes:The patient has retinitis pigmentosa, bilateral hearing loss, double diabetes with weakly positive anti-insulin antibodies and DNA analysis showed novel mutation for Alström syndrome.Lessons:the combination of obesity, diabetes, hearing loss and blindness should alert the physician to test for Alström syndrome.