Glycemic variability in patients with Wolfram syndrome is lower than in type 1 diabetes

Abstract

Aims Wolfram syndrome (WFS) is diagnosed as coexistence of diabetes mellitus and optic atrophy, where pancreatic beta cell destruction is associated with neurodegeneration. Typically, WFS necessitates insulin treatment similar to type 1 diabetes (T1D), but the mechanism of beta cell mass reduction leading to hyperglycemia is different.MethodsThe aim of the study was to assess glycemic variability using the continuous glucose monitoring (CGM) system in seven pediatric patients with genetically confirmed WFS and compare the results with data obtained from 21 propensity score-matched patients with T1D. The “GlyCulator” application was used for the calculation of glycemic variability indices.ResultsCGM recordings showed similarities in glycemic variability among WFS patients, but differing from those of the T1D group. Coefficient of variation (%CV), CONGA4h, and GONGA6h were significantly (p < 0.05) lower in WFS patients (28.08 ± 7.37, 54.96 ± 11.92, and 55.99 ± 10.58) than in T1D patients (37.87 ± 14.24, 74.12 ± 28.74, p = 0.02, and 80.26 ± 35.05, respectively). In WFS patients, the percentage of values above 126 mg/dL was 69.79 (52.08–77.43), whereas in patients with T1D, the percentage was significantly lower—47.22 (35.07–62.85, p = 0.018). Curiously, a tendency toward a lower percentage of measurements below 70 mg/dL was noted in the WFS group [0 (0–7.29)] in comparison with the T1D group [6.25 (0–18.06), p = 0.122]. WFS patients had a significantly higher C-peptide level (0.31 ± 0.2 ng/mL) than T1D patients (0.04 ± 0.04 ng/mL; p = 0.006).ConclusionsPatients with WFS show smaller glycemic variability than individuals with T1D, and this may be associated with persistent residual insulin secretion.

Genetic and clinical aspects of Wolfram syndrome 1, a severe neurodegenerative disease

“…No differences have been found between children/adolescents with insulin-dependent, antibodynegative DM and control subjects or between adult WFS1 mutation carriers and control subjects. Thus, retinal thinning is a clinical feature of WS1 and may be a potential marker of disease progression in WS1 patients (69).…”

Diabetes mellitus, diabetes insipidus, optic atrophy, and deafness: A case of Wolfram (DIDMOAD) syndrome

“… 15 Patients with WS show smaller glycemic variability than individuals with type 1 diabetes mellitus, and this may be associated with persistent residual insulin secretion. 16 …”

Monogenic Diabetes in Children and Adolescents: Recognition and Treatment Options

“…Even though the presence of visual abnormalities accompanying diabetes in patients with WFS may lead to a misdiagnosis of T1D with diabetic retinopathy in some children and adolescents, the clinical courses of these two types of diabetes are different. Patients with WFS are autoantibody negative and are characterized by lower daily insulin requirements, low frequency of ketoacidosis, better glycemic control, and lower HbA1c levels in comparison to patients with T1D [106–108]. …”