The course may be fatal in congenital cases (30-40%). Patients subsequently develop delayed cognitive and motor milestones intellectual disability, and autism spectrum disorder. Congenital disease (15% of cases) is the most severe form and includes severe generalized weakness at birth with respiratory distress, hypotonia, and feeding difficulty. Five forms are currently recognized: congenital, early childhood, juvenile, adult-onset and late-onset. The clinical manifestations also cover a wide range and may differ within and between affected families. The age of onset is highly variable, from prenatal to adulthood. It appears to be more prevalent in the Saguenay-Lac-St-Jean region-Quebec, Canada (1/600), suggesting a founder effect. This reduced tissue sensitivity to epinephrine resulted in lower endogenous glucose production and less inhibition of insulin-stimulated glucose uptake.It is the most frequent adult muscular dystrophy and has an estimated prevalence ranging from 1/215,000 in Taiwan to 1/5,500 in Croatia. 14 also demonstrated that patients with type diabetes had reduced whole-body tissue sensitivity to epinephrine, which was exacerbated by intensive glycemic control. 34 demonstrated that type 1 diabetic subjects with blunted counterregulatory responses to hypoglycemia had reduced β-adrenergic sensitivity compared to patients with normal counterregulatory responses to hypoglycemia and healthy control subjects. 3- 6, 19, 31It is also possible that patients with hypoglycemia-associated autonomic failure also have reduced adrenergic sensitivity (i.e., tissue responsiveness to circulating epinephrine). Hypoglycemia-associated autonomic failure is an acutely acquired syndrome that should be differentiated from classical diabetic autonomic neuropathy. This indicates that the genesis of hypoglycemic symptoms is multifocal and is probably mainly generated from central nervous system (CNS) efferent pathways. 3- 5However, it should be noted that epinephrine infusion in the presence of euglycemia to achieve levels commonly seen during hypoglycemia only produces 20% of the total neurogenic symptom scores found during hypoglycemia. 4, 5, 9The cholinergic-mediated symptoms include diaphoresis, hunger, and paresthesias. 5, 8Neurogenic symptoms and signs associated with elevated epinephrine levels include shakiness, anxiety, nervousness, palpitations, sweating, dry mouth,pallor, and pupil dilation. 5, 7, 8These symptoms are activated by the ANS and are mediated in part by sympathoadrenal release of catecholamines (norepinephrine and epinephrine)from the adrenal medullae and acetylcholine from postsynaptic sympathetic nerve endings. ![]() Neurogenic(autonomic) symptoms are triggered by a falling glucose level and cause patients to recognize that they are experiencing a hypoglycemic episode. Symptoms of hypoglycemia are divided into two categories. Clinical Impact of Iatrogenic Hypoglycemia The authors also concluded that hypoglycemia occurs more often than previously reported 2 in insulin-treated type 2 diabetes and with sufficient frequency to cause significant morbidity. Self-reports of severe hypoglycemia in type 2 diabetic subjects were lower than in type 1 diabetic subjects. Predictors for hypoglycemia for the insulin-treated type 2 diabetic subjects included a history of previous hypoglycemia ( P< 0.0001) and duration of insulin treatment ( P = 0.014). For individuals with type 1 diabetes,predictors of hypoglycemia included a history of previous hypoglycemia( P = 0.006). The type 1 diabetic subjects reported a rate of 43 events per patient per year, whereas subjects with type 2 diabetes reported a rate of 16 events per patient per year. Of the 267 subjects, 155 reported 572 incidents of hypoglycemia. 13 randomly surveyed individuals ( n = 267) with type 1 diabetes and insulin-treated type 2 diabetes to prospectively record hypoglycemic events encountered over a 4-week period. Institutional Subscriptions and Site Licensesĭonnelly et al. ![]()
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