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


METHODS
The 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.


RESULTS
CGM 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).


CONCLUSIONS
Patients with WFS show smaller glycemic variability than individuals with T1D, and this may be associated with persistent residual insulin secretion.
Department of Pediatrics, Oncology, Hematology and Diabetology, Medical University of Lodz, Sporna Str. 36/50, 91-738, Lodz, Poland. agnieszka.zmyslowska@umed.lodz.pl.
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Zmyslowska
Department of Pediatrics, Oncology, Hematology and Diabetology, Medical University of Lodz, Sporna Str. 36/50, 91-738, Lodz, Poland.
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Fendler
Department of Pediatrics, Oncology, Hematology and Diabetology, Medical University of Lodz, Sporna Str. 36/50, 91-738, Lodz, Poland.
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Szadkowska
Department of Clinical Genetics, Medical University of Lodz, Lodz, Poland.
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Borowiec
Department of Pediatrics, Diabetology and Endocrinology, Medical University of Gdansk, Gdańsk, Poland.
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Mysliwiec
Department of Pediatrics, Oncology, Hematology and Diabetology, Medical University of Lodz, Sporna Str. 36/50, 91-738, Lodz, Poland.
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Baranowska-Jazwiecka
Department of Pediatrics, Diabetology and Endocrinology, Medical University of Gdansk, Gdańsk, Poland.
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Buraczewska
Department of Pediatrics, Oncology, Hematology and Diabetology, Medical University of Lodz, Sporna Str. 36/50, 91-738, Lodz, Poland.
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Fulmanska-Anders
Department of Pediatrics, Oncology, Hematology and Diabetology, Medical University of Lodz, Sporna Str. 36/50, 91-738, Lodz, Poland.
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Mianowska
Department of Pediatrics, Oncology, Hematology and Diabetology, Medical University of Lodz, Sporna Str. 36/50, 91-738, Lodz, Poland.
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Pietrzak
Department of Pediatrics, Oncology, Hematology and Diabetology, Medical University of Lodz, Sporna Str. 36/50, 91-738, Lodz, Poland.
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Rzeznik
Department of Pediatrics, Oncology, Hematology and Diabetology, Medical University of Lodz, Sporna Str. 36/50, 91-738, Lodz, Poland. Wojciech.Mlynarski@umed.lodz.pl.
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Mlynarski
D000293:Adolescent; D001786:Blood Glucose / Q000378:metabolism*; D002648:Child; D003922:Diabetes Mellitus, Type 1 / Q000378:metabolism*; D005260:Female; D006442:Glycated Hemoglobin / Q000032:analysis; D006801:Humans; D006943:Hyperglycemia / Q000473:pathology; D050417:Insulin-Secreting Cells / Q000473:pathology; D008297:Male; D057216:Propensity Score; D014929:Wolfram Syndrome / Q000378:metabolism*
D001786:Blood Glucose; D006442:Glycated Hemoglobin
Continuous glucose monitoring system; GlyCulator; Type 1 diabetes; Wolfram syndrome