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Persisterende hyperinsulinemische hypoglykemie bij kinderen: gedifferentieerde aanpak bij een heterogeen syndroom.
| Content Provider | Semantic Scholar |
|---|---|
| Author | Vroede, M. De Groenendaal, Floris |
| Copyright Year | 2004 |
| Abstract | Persistent hyperinsulinemic hypoglycaemia in infancy: differentiated approach to a heterogeneous syndrome – Persistent hyperinsulinemic hypoglycaemia in infancy (PHHI) presents a diagnostic and therapeutic challenge for the treating physician: increased glucose requirements, detectable insulin levels at the point of hypoglycaemia, inappropriately low blood levels of free fatty acids and ketone bodies are characteristic of this condition. – Despite recent developments in understanding its pathophysiology, treatment remains difficult and there are many long-term complications. – Adequate treatment strategies are needed to prevent severe neurological damage. – As there is a high possibility that hyperinsulinism may only be transient, aggressive pharmacological treatment is necessary for 4 to 6 weeks before moving on to surgical intervention. – In the light of recent knowledge, routine subtotal pancreatectomy in children is no longer justifiable. An attempt should first be made to differentiate between focal and diffuse hypersecretion of insulin by using interventional radiology techniques, notably pancreatic venous sampling. This then enables targeted partial pancreatectomy. literatuur 1 Bruining GJ. Recent advances in hyperinsulinism and the pathogenesis of diabetes mellitus. Curr Opin Pediatr 1990;2:758-65. 2 Glaser B, Thornton P, Otonkoski T, Junien C. Genetics of neonatal hyperinsulinism. Arch Dis Child Fetal Neonatal Ed 2000;82:F79-86. 3 Saudubray JM, De Lonlay P, Touati G, Martin D, Nassogne MC, Castelnau P, et al. Genetic hypoglycaemia in infancy and childhood: pathophysiology and diagnosis. J Inherit Metab Dis 2000;23:197-214. 4 Aynsley-Green A, Hussain K, Hall J, Saudubray JM, Nihoul-Fékété C, De Lonlay-Debeney P, et al. Practical management of hyperinsulinism in infancy. Arch Dis Child Fetal Neonatal Ed 2000;82: F98-107. 5 Menni F, De Lonlay P, Sevin C, Touati G, Peigné C, Barbier V, et al. 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Pancreatic venous samplings in infants and children with primary hyperinsulinism. Pediatr Radiol 1989;19: 100-3. 25 Dubois J, Brunelle F, Touati G, Sebag G, Nuttin C, Thach T, et al. Hyperinsulinism in children: diagnostic value of pancreatic venous sampling correlated with clinical, pathological and surgical outcome in 25 cases. Pediatr Radiol 1995;25:512-6. 26 Crétolle C, Fékété CN, Jan D, Nassogne MC, Saudubray JM, Brunelle F, et al. Partial elective pancreatectomy is curative in focal form of permanent hyperinsulinemic hypoglycaemia in infancy: a report of 45 cases from 1983 to 2000. J Pediatr Surg 2002;37:155-8. Aanvaard op 14 mei 2003 Ned Tijdschr Geneeskd 2004 17 januari;148(3) 129 |
| Starting Page | 694 |
| Ending Page | 695 |
| Page Count | 2 |
| File Format | PDF HTM / HTML |
| Volume Number | 148 |
| Alternate Webpage(s) | https://www.ntvg.nl/system/files/publications/2004101250001a.pdf |
| Language | English |
| Access Restriction | Open |
| Content Type | Text |
| Resource Type | Article |
