Glycemic control and complications in glycogen storage disease type I: Results from the Swiss registry
| Autor: | Michel Hochuli, Nathalie Kaiser, Fabian Meienberg, Matthias Gautschi, Giatgen A. Spinas, Lenka Bosanska, Matthias R. Baumgartner |
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| Přispěvatelé: | University of Zurich, Hochuli, Michel |
| Jazyk: | angličtina |
| Rok vydání: | 2019 |
| Předmět: |
Blood Glucose
Male 0301 basic medicine 1303 Biochemistry Endocrinology Diabetes and Metabolism 10265 Clinic for Endocrinology and Diabetology Glycogen Storage Disease Type I 030105 genetics & heredity Biochemistry Cohort Studies 0302 clinical medicine Endocrinology Bone Density Glycogen storage disease Registries Blood Glucose Measurement 610 Medicine & health Glycogen storage disease type I 1310 Endocrinology 2712 Endocrinology Diabetes and Metabolism Cohort Female medicine.symptom Switzerland Adult medicine.medical_specialty Adolescent Hypoglycemia Asymptomatic Adenoma Liver Cell Young Adult 03 medical and health sciences Disease registry 1311 Genetics Internal medicine 1312 Molecular Biology Genetics medicine Humans Molecular Biology Glycemic business.industry medicine.disease Cross-Sectional Studies Glucose 10036 Medical Clinic business 030217 neurology & neurosurgery |
| DOI: | 10.7892/boris.136910 |
| Popis: | Regular carbohydrate intake to avoid hypoglycemia is the mainstay of dietary treatment in glycogen storage disease type I (GSDI). The aim of this study was to evaluate the quality of dietary treatment and glycemic control in a cohort of GSDI patients, in relation to the presence of typical long-term complications.Data of 25 patients (22 GSD subtype Ia and 3 GSDIb, median age 20y) from the Swiss hepatic glycogen storage disease registry was analyzed cross-sectionally. Frequency and type of hypoglycemia symptoms were assessed prospectively using a structured questionnaire. Diagnostic continuous glucose monitoring (CGM) was performed as part of usual clinical care to assess glycemic control in 14 patients, usually once per year with a mean duration of 6.2 ± 1.1 consecutive days per patient per measurement.Although maintenance of euglycemia is the primary goal of dietary treatment, few patients (n = 3, 13%) performed capillary blood glucose measurements regularly. Symptoms possibly associated with hypoglycemia were present in 13 patients (57%), but CGM revealed periods of low glucose (4 mmol/l) in all patients, irrespective of the presence of symptoms. GSDIa patients with liver adenomas (n = 9, 41%) showed a higher frequency and area under the curve (AUC) of low blood glucose than patients without adenomas (frequency 2.7 ± 0.8 vs. 1.5 ± 0.7 per day, AUC 0.11 ± 0.08 vs. 0.03 ± 0.02 mmol/l/d; p 0.05). Similarly, the presence of microalbuminuria was also associated with the frequency of low blood glucose. Z-Scores of bone density correlated negatively with lactate levels.The quality of glucose control is related to the presence of typical long-term complications in GSDI. Many patients experience episodes of asymptomatic low blood glucose. Regular assessment of glucose control is an essential element to evaluate the quality of treatment, and increasing the frequency of glucose self-monitoring remains an important goal of patient education and motivation. CGM devices may support patients to optimize dietary therapy in everyday life. |
| Databáze: | OpenAIRE |
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