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J Pediatr Endocrinol Metab ; 36(8): 803-807, 2023 Aug 28.
Artigo em Inglês | MEDLINE | ID: mdl-37248699

RESUMO

OBJECTIVES: CHI is a relevant cause of persistent and severe hypoglycemia and the ABCC8 gene mutation is one of most common cause of the disease. Two main types of CHI have been described, diffuse and focal form. Octreotide is a medication utilized in case of diazoxide-unresponsive forms of CHI. For those CHI focal forms where is decided either to manage medically or until resolutive surgery is completed, octreotide can be administered as subcutaneous injection or as continuous subcutaneous infusion via insulin pump. However, it is unclear how to adjust the drug's daily basal pattern when a pump is used. CASE PRESENTATION: We present a case of an infant with a diazoxide-unresponsive focal form of CHI, due to ABCC8 mutation ABCC8, treated with octreotide. To better evaluate the glycemic trend, a CGM was placed. In order to achieve a better personalization of the therapy we utilized an insulin pump for octreotide administration. CONCLUSIONS: The adoption of the CGM and insulin pump, allowed a better personalization of the therapy and a reduction of acute carbohydrate intake, promoting a good auxological growth before resolutive surgery. What is new? Octreotide administered with an insulin pump in patient with CHI allows a wide modulation of the daily therapy. The CGM allows a continuous and a less painful control of the glycemic trend in a patient with CHI. Different basal rates, given via insulin pump may allow a better personalization of the therapy. Prevention of hypoglycemia reduces the acute introduction of carbohydrates, promoting normal growth..


Assuntos
Hiperinsulinismo Congênito , Diazóxido , Octreotida , Humanos , Masculino , Lactente , Hiperinsulinismo Congênito/tratamento farmacológico , Diazóxido/administração & dosagem , Diazóxido/uso terapêutico , Octreotida/administração & dosagem , Octreotida/uso terapêutico , Automonitorização da Glicemia , Resultado do Tratamento , Insulina/uso terapêutico
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