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Without prolonged fasting, healthy individuals maintain blood glucose levels above 3.5 mM due to a well-adapted neuroendocrine counterregulatory system that effectively prevents acute hypoglycemia, a potentially life-threatening condition. The primary clinical scenarios for hypoglycemia encompass diabetes treatment, inappropriate production of endogenous insulin or insulin-like substances by tumors, and the use of glucose-lowering agents in non-diabetic individuals. Notably, hypoglycemia in the...
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Type 2 diabetes, characterized by insulin resistance, arises when the insulin receptors on cells lose responsiveness to insulin, diminishing the cell's capacity to take up glucose, resulting in elevated blood glucose levels. To receive a diagnosis of Type 2 diabetes, a series of blood glucose tests are necessary to assess whether the blood glucose falls within normal parameters. If the result is out of the normal range, a patient may be diagnosed as prediabetic or diabetic, depending on the...
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For most patients, experiencing several weeks of polyuria, polydipsia, fatigue, and significant weight loss may indicate the presence of diabetes. Furthermore, adults displaying the phenotypic appearance of type 2 diabetes (particularly those who are obese and not initially insulin-requiring), may have islet cell autoantibodies, suggesting autoimmune-mediated β cell destruction and a diagnosis of latent autoimmune diabetes of adults (LADA). The categorization of glucose homeostasis is...
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Diabetes mellitus is a chronic metabolic disorder characterized by high blood glucose levels due to inadequate insulin production, insulin resistance, or both. The condition affects millions worldwide and can significantly impact their health and quality of life.
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Repaglinide (Prandin) and Nateglinide (Starlix), known as glinides, are oral insulin secretagogues that stimulate insulin release from pancreatic β cells by closing the ATP-sensitive potassium channels (KATP channel). Repaglinide controls insulin release from pancreatic β cells by managing potassium efflux. It shares two binding sites with sulfonylureas and also has a unique site, indicating overlapping mechanisms of action. With a rapid onset and a 4-7 hour duration, it effectively...
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The therapy for diabetes aims to alleviate hyperglycemia-related symptoms, prevent acute metabolic decompensation, and reduce chronic end-organ complications. Glycemic control is evaluated through short-term (self-monitoring, continuous glucose monitoring) and long-term (A1c, fructosamine) metrics, enabling near real-time tracking of blood glucose levels and reflecting glycemic control over specific time frames.
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Hypoglycemia in Patients With LAMA2-CMD.

Clara Gontijo Camelo1, Cristiane de Araújo Martins Moreno1, Mariana Cunha Artilheiro1

  • 1Department of Neurology, Faculdade de Medicina da Universidade de São Paulo (FMUSP), São Paulo, SP, Brazil.

Pediatric Neurology
|March 19, 2023
PubMed
Summary

Patients with LAMA2-related congenital muscular dystrophy (CMD) frequently experience hypoglycemia, especially those with severe symptoms or extremely low weight. Early glucose monitoring and prompt treatment are crucial for managing this risk.

Keywords:
Genotype-phenotypeHypoglycemiaLAMA2-CMDWeight

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Area of Science:

  • Neurology
  • Genetics
  • Metabolic Disorders

Background:

  • Hypoglycemia is a known complication in LAMA2-CMD patients.
  • Previous assessments of hypoglycemia frequency, risk factors, and genotype/phenotype correlations in LAMA2-CMD are lacking.

Purpose of the Study:

  • To systematically assess the frequency, risk factors, and genotype/phenotype correlations of hypoglycemia in LAMA2-CMD.
  • To identify patient subgroups at higher risk for hypoglycemia.

Main Methods:

  • A retrospective cohort study of 48 LAMA2-CMD patients.
  • Patients were categorized into hypoglycemic and non-hypoglycemic groups.
  • Comparison included gait, epilepsy, intellectual disability, constipation, reflux, gastrostomy, weight, scoliosis, ventilator/feeding device use, swallowing status, and mutation type.

Main Results:

  • 31.2% of patients experienced at least one hypoglycemic episode; 16.6% had two or more.
  • Hypoglycemia correlated with nonambulant status, ventilator/feeding device use, and impaired swallow function.
  • Extremely low weight increased the likelihood of recurrent hypoglycemia by fivefold.
  • Missense variants were associated with a reduced risk of hypoglycemia.

Conclusions:

  • LAMA2-CMD patients are at significant risk for hypoglycemia, particularly those with severe phenotypes or loss-of-function variants.
  • Patients with extremely low weight face a heightened risk.
  • Active blood glucose monitoring during fasting or infection is recommended; healthcare providers must be prepared for identification and treatment.