Therapies and outcomes of congenital hyperinsulinism-induced hypoglycaemia
| Autor: | Karen E. Cosgrove, Pratik Shah, Indi Banerjee, Mark J. Dunne, Jacqueline Nicholson, Maria Salomon-Estebanez |
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| Jazyk: | angličtina |
| Rok vydání: | 2019 |
| Předmět: |
Pediatrics
medicine.medical_specialty Endocrinology Diabetes and Metabolism 030209 endocrinology & metabolism Disease Brain damage Sulfonylurea Receptors 03 medical and health sciences 0302 clinical medicine Endocrinology Pancreatectomy Gastrointestinal Agents Diabetes mellitus Internal Medicine Diazoxide Neurodisability Medicine Humans 030212 general & internal medicine Potassium Channels Inwardly Rectifying Precision Medicine Antihypertensive Agents Invited Review business.industry medicine.disease Glucagon Hypoglycemia Treatment Outcome Congenital hyperinsulinism Congenital Hyperinsulinism medicine.symptom business Hyperinsulinism medicine.drug Rare disease |
| Zdroj: | Banerjee, I, Salomon Estebanez, M, Shah, P, Nicholson, J, Cosgrove, K & Dunne, M 2019, ' Therapies and outcomes of congenital hyperinsulinism-induced hypoglycaemia ', Diabetic Medicine, vol. 36, no. 1, pp. 9-21 . https://doi.org/10.1111/dme.13823 Diabetic Medicine Banerjee, I, Salomon-estebanez, M, Shah, P, Nicholson, J, Cosgrove, K E & Dunne, M J 2018, ' Therapies and outcomes of congenital hyperinsulinism-induced hypoglycaemia ' Diabetic Medicine . DOI: 10.1111/dme.13823 |
| Popis: | Congenital hyperinsulinism is a rare disease, but is the most frequent cause of persistent and severe hypoglycaemia in early childhood. Hypoglycaemia caused by excessive and dysregulated insulin secretion (hyperinsulinism) from disordered pancreatic β cells can often lead to irreversible brain damage with lifelong neurodisability. Although congenital hyperinsulinism has a genetic cause in a significant proportion (40%) of children, often being the result of mutations in the genes encoding the KATP channel (ABCC8 and KCNJ11), not all children have severe and persistent forms of the disease. In approximately half of those without a genetic mutation, hyperinsulinism may resolve, although timescales are unpredictable. From a histopathology perspective, congenital hyperinsulinism is broadly grouped into diffuse and focal forms, with surgical lesionectomy being the preferred choice of treatment in the latter. In contrast, in diffuse congenital hyperinsulinism, medical treatment is the best option if conservative management is safe and effective. In such cases, children receiving treatment with drugs, such as diazoxide and octreotide, should be monitored for side effects and for signs of reduction in disease severity. If hypoglycaemia is not safely managed by medical therapy, subtotal pancreatectomy may be required; however, persistent hypoglycaemia may continue after surgery and diabetes is an inevitable consequence in later life. It is important to recognize the negative cognitive impact of early‐life hypoglycaemia which affects half of all children with congenital hyperinsulinism. Treatment options should be individualized to the child/young person with congenital hyperinsulinism, with full discussion regarding efficacy, side effects, outcomes and later life impact. What's new? Congenital hyperinsulinism is a heterogeneous disorder of hypoglycaemia with high morbidity and few therapeutic options.Investigation and treatment principles have been described in several reviews but there has been little emphasis on medium‐ to long‐term outcomes.In the present review, we describe outcomes of congenital hyperinsulinism to provide a long‐range view of the clinical impact of current therapeutic strategies. |
| Databáze: | OpenAIRE |
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