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Related Concept Videos

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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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Hypoglycemia is a blood glucose level below 70 mg/dL. It commonly occurs in individuals using insulin or insulin-secreting drugs, but may also arise in non-diabetic conditions. People with type 1 diabetes are at the highest risk because they depend on exogenous insulin. People with type 2 diabetes are also at risk, especially when treated with insulin or medications such as sulfonylureas, which increase insulin release regardless of blood glucose levels. It develops when insulin levels exceed...
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Hyperglycemia01:29

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Hyperglycemia is an abnormally high blood glucose level. It is diagnosed by fasting glucose ≥126 mg/dL, 2-hour oral glucose tolerance test (or OGTT) ≥200 mg/dL, random glucose ≥200 mg/dL with symptoms, or HbA1c ≥6.5%. However, HbA1c results may be unreliable in certain conditions, such as anemia or hemoglobinopathies, and the diagnosis should be confirmed unless classic symptoms are present. Postprandial hyperglycemia is typically considered significant when glucose...
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Type I Diabetes III: Clinical Manifestations01:19

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Type 1 diabetes mellitus typically presents with rapid-onset symptoms due to the body’s inability to utilize glucose in the absence of insulin. Since insulin is required for glucose uptake into cells, its deficiency leads to hyperglycemia and cellular energy deprivation, resulting in characteristic clinical features.Polyuria and PolydipsiaOne of the earliest, most prominent symptoms is polyuria (excessive urination). When blood glucose concentrations rise above the renal threshold, the...
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Diabetes Mellitus: Overview and Type I Subtype01:22

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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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Insulin: Dosing Regimen and Adverse Effects01:16

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Insulin-replacement therapy usually includes both long-acting insulin (basal) and short-acting insulin (to cater to postprandial needs). In a diverse group of type 1 diabetes patients, the average daily insulin dose is typically 0.5-0.7 units/kg body weight. However, obese patients and pubertal adolescents may need more due to insulin resistance.
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Persistent hyperinsulinaemic hypoglycaemia in infancy.

Pratik Shah1, Huseyin Demirbilek1, Khalid Hussain1

  • 1Department of Developmental Endocrinology Research Group, Clinical and Molecular Genetics Unit, Institute of Child Health, University College London; Department of Paediatric Endocrinology, Great Ormond Street Hospital for Children, London.

Seminars in Pediatric Surgery
|June 17, 2014
PubMed
Summary
This summary is machine-generated.

Persistent hyperinsulinaemic hypoglycaemia in infancy (PHHI) is a common cause of neonatal hypoglycemia. Early diagnosis and management, including advanced imaging and surgical options like laparoscopy, are crucial to prevent neurological damage.

Keywords:
DiffuseFocalHyperinsulinaemic hypoglycaemia

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Area of Science:

  • Pediatric Endocrinology
  • Neonatal Medicine
  • Surgical Gastroenterology

Background:

  • Persistent hyperinsulinaemic hypoglycaemia in infancy (PHHI) is a leading cause of severe neonatal hypoglycemia.
  • Unregulated insulin secretion can lead to significant neurological impairment if not managed promptly.
  • PHHI presents histologically as diffuse, focal, or atypical disease, impacting treatment strategies.

Purpose of the Study:

  • To highlight the heterogeneity of PHHI and its clinical implications.
  • To emphasize the importance of early diagnosis and management to prevent brain injury.
  • To discuss the role of advanced imaging and surgical techniques in managing PHHI.

Main Methods:

  • Review of histological classifications of PHHI (diffuse, focal, atypical).
  • Utilisation of 18F-DOPA-PET/CT scans for differentiating diffuse and focal PHHI.
  • Analysis of genetic defects in key insulin secretion regulatory genes.
  • Comparison of surgical interventions: lesionectomy, near-total pancreatectomy, and laparoscopic approaches.

Main Results:

  • 18F-DOPA-PET/CT effectively distinguishes between diffuse and focal PHHI.
  • Focal PHHI is sporadic and treatable with lesionectomy.
  • Diffuse PHHI, often inherited, may require near-total pancreatectomy if medical therapy fails.
  • Laparoscopic surgery offers minimally invasive options for pancreatic resection in PHHI.

Conclusions:

  • Early recognition and tailored management are vital for preventing long-term complications of PHHI.
  • Advanced imaging and genetic analysis aid in precise diagnosis and treatment planning.
  • Surgical interventions, particularly laparoscopic techniques, are advancing the treatment of PHHI, balancing efficacy with reduced morbidity.