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Recent updates in the management of infants and children with hyperinsulinism.
Thornton, Paul S.
Afiliação
  • Thornton PS; Cook Children's Medical Center, Fort Worth, Texas, USA.
Curr Opin Pediatr ; 33(4): 424-429, 2021 08 01.
Article em En | MEDLINE | ID: mdl-34001718
PURPOSE OF REVIEW: To highlight recent advances in early diagnosis and the changing treatment paradigm for hyperinsulinism (HI) which can result in shorter hospitalizations, higher rates of cure and improved neurological outcome. RECENT FINDINGS: Recent literature has shown that following publication of the pediatric endocrinology society guidelines for diagnosing hypoglycemia there have been higher rates of diagnosis of acquired and genetic HI. Studies of neurological outcome have found that poor outcomes are associated with delay between initial hypoglycemia and instigation of treatment for HI, hypoglycemic seizures and frequency of glucose <20 mg/dL. Rapid genetic testing can decrease the time from the discovery of diazoxide unresponsiveness to referral to multidisciplinary centers with the availability of 18-F-L 3,4-Dihydroxyphenylalanine positron emission tomography (18F-DOPA PET). Proper selection of patients for 18F-DOPA PET and careful interpretation of the images can result in greater than 90% cure for patients with focal HI. SUMMARY: Recent advances in the early diagnosis of HI and rapid turnaround genetic testing can lead to prompt transfer to centers with multidisciplinary care teams where proper selection of patients for 18F-DOPA PET scan gives the best opportunity for cure for patients with focal disease. Minimizing severe hypoglycemia maximizes the opportunity for improved neurological outcome.
Assuntos

Texto completo: 1 Base de dados: MEDLINE Assunto principal: Hiperinsulinismo Congênito Tipo de estudo: Diagnostic_studies / Guideline / Prognostic_studies / Screening_studies Limite: Child / Humans / Infant Idioma: En Ano de publicação: 2021 Tipo de documento: Article

Texto completo: 1 Base de dados: MEDLINE Assunto principal: Hiperinsulinismo Congênito Tipo de estudo: Diagnostic_studies / Guideline / Prognostic_studies / Screening_studies Limite: Child / Humans / Infant Idioma: En Ano de publicação: 2021 Tipo de documento: Article