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تعداد آیتم قابل مشاهده باقیمانده : 3 مورد
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Evaluation and management of an infant or child with congenital hyperinsulinism

Evaluation and management of an infant or child with congenital hyperinsulinism
HI: hyperinsulinism; CVC: central venous catheter; GCK: glucokinase gene; ABCC8: ATP-binding cassette C8 gene; KCNJ11: potassium channel inwardly rectifying J11 gene; 18F-DOPA PET: 18-fluoro-L-3,4-dihydroxyphenylalanine positron emission tomography; BOHB: beta-hydroxybutyrate; FFA: free fatty acids.
* Laboratory evidence of HI during an episode of hypoglycemia (plasma glucose <50 mg/dL [2.8 mmol/L]) includes suppressed BOHB and FFA, any detectable insulin or C-peptide, and glycemic response to glucagon. Refer to UpToDate content on diagnosis of congenital HI for details.
¶ A "safety fast" is a carefully monitored fasting test with the goal of ensuring that plasma glucose remains greater than 70 mg/dL while on therapy for hypoglycemia. For a protocol, refer to UpToDate content on management of congenital HI.
Δ Among patients who respond to diazoxide, up to 50% may have negative genetic testing.
Features suggesting perinatal stress-induced HI include birth asphyxia, small for gestational age, congenital heart disease, and history of intrauterine preeclampsia.
§ A focal lesion is identified in approximately 50% of patients with recessive mutations in ABCC8 or KCNJ11.
¥ Medical management is the first-line approach for infants with the diffuse form of HI. Options depend on the severity of hypoglycemia and other clinical factors. Refer to UpToDate content on treatment of congenital HI for details.
Graphic 128425 Version 2.0

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