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Martinac I, Bogović M, Batinica S, et al. Kongenitalni hiperinzulinizam- -novosti o nastanku, dijagnosticiranju i lijecenju bolesti. [Congenital hyperinsulinism--novel insights into etiology, diagnosis and treatment]. Lijec Vjesn. 2012;134(9):286-92
Martinac, I., Bogović, M., Batinica, S., Sarnavka, V., Frković, S. H., Matić, T., Jakić-Razumović, J., Rubin, O., Luetić, T., Kusec, V., Ramadza, D. P., Begović, D., Benjak, V., Dasović-Buljević, A., Antabak, A., Cavar, S., Kukin, D., Srsen-Medancić, S., & Barić, I. (2012). Kongenitalni hiperinzulinizam- -novosti o nastanku, dijagnosticiranju i lijecenju bolesti. [Congenital hyperinsulinism--novel insights into etiology, diagnosis and treatment]. Lijecnicki vjesnik, 134(9), 286-92.
Martinac, Iva, et al. "Kongenitalni hiperinzulinizam- -novosti o nastanku, dijagnosticiranju i lijecenju bolesti." [Congenital hyperinsulinism--novel insights into etiology, diagnosis and treatment]. Lijecnicki vjesnik vol. 134,9 (2012): 286-92.
Martinac I, Bogović M, Batinica S, Sarnavka V, Frković SH, Matić T, Jakić-Razumović J, Rubin O, Luetić T, Kusec V, Ramadza DP, Begović D, Benjak V, Dasović-Buljević A, Antabak A, Cavar S, Kukin D, Srsen-Medancić S, Barić I. Kongenitalni hiperinzulinizam- -novosti o nastanku, dijagnosticiranju i lijecenju bolesti. [Congenital hyperinsulinism--novel insights into etiology, diagnosis and treatment]. Lijec Vjesn. 2012 Sep-Oct;134(9):286-92. Croatian. PMID: 23297514.
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Lijec Vjesn. 2012 Sep-Oct;134(9):286-92.

[Congenital hyperinsulinism--novel insights into etiology, diagnosis and treatment].

Lijecnicki vjesnik

[Article in Croatian]
Iva Martinac, Marko Bogović, Stipe Batinica, Vladimir Sarnavka, Sanda Huljev Frković, Toni Matić, Jasminka Jakić-Razumović, Otmar Rubin, Tomislav Luetić, Vesna Kusec, Danijela Petković Ramadza, Davor Begović, Vesna Benjak, Andrea Dasović-Buljević, Anko Antabak, Stanko Cavar, Dijana Kukin, Suzana Srsen-Medancić, Ivo Barić

Affiliations

  1. Klinika za pedijatriju Medicinskog fakulteta Sveucilista u Zagrebu, KBC Zagreb.

PMID: 23297514

Abstract

Congenital hyperinsulinism (CHI) is a major cause of persistent hypoglycemia in the neonatal and early infancy periods. Althought the disease is relatively rare with incidence of about 1:25 000-50 000 live births, the importance of the disease should not be underestimated. Namely, prompt recognition and management of patients with CHI is essential, if permanent neurological impairment is to be avoided. CHI is caused by mutations in one of the 7 genes involved in the regulation of insulin secretion in pancreatic beta-cells. It is important to introduce specific medical therapy as soon as diagnosis is established. Severe, neonatal forms of CHI are often resistant to medications, thus they require surgical procedure. The preoperative genetic testing and scintigraphy are indicated to distinguish histological subtypes of the disease (focal vs. diffuse CHI). Patients with focal disease are usually cured after pancreatic resection, while diffuse disease has much worse prognosis. This manuscript offers novel insights into CHI and emphasizes the role of early diagnosis as crucial for succesful treatment that was recently enriched with novel options.

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