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Published on: November 16, 2011
Hyperglycaemia and insulinopenia in a neonate with cystic fibrosis
Tania Siahanidou1, Helen Mandyla, Stavros Doudounakis
1Neonatal Unit, First Department of Paediatrics, Athens University, "Aghia Sophia" Children's Hospital, Athens, Greece. siahanidou@hotmail.com
Insights
Abnormal glucose tolerance, a complication of cystic fibrosis (CF), can occur in newborns. This case highlights that CF-related diabetes mellitus (CFRD) and insulinopenia may be present from birth, suggesting earlier onset than previously recognized.
Area of Science:
- Pediatric Endocrinology
- Metabolic Disorders
- Genetics
Background:
- Cystic Fibrosis (CF) is a genetic disorder affecting multiple organs.
- Abnormal glucose tolerance, leading to CF-related diabetes mellitus (CFRD), is a known complication, typically diagnosed later in childhood.
- The prevalence of CFRD in children under 10 years is less than 2%, with the youngest reported case at 6 months.
Observation:
- A neonate diagnosed with CF presented with severe hyperglycemia, indicative of a diabetic range.
- This neonate exhibited marked insulinopenia, a deficiency in insulin production.
- The observed insulinopenia and impaired glucose tolerance persisted at 6 and 15 months of age.
Findings:
- Insulinopenia is identified as the primary cause of abnormal glucose tolerance and CFRD.
- This case demonstrates that CFRD and associated insulinopenia can manifest in the neonatal period, much earlier than previously documented.
- The findings suggest that glucose intolerance may be a much earlier complication of CF than typically diagnosed.
Implications:
- This case challenges the established timeline for CFRD development in cystic fibrosis patients.
- It suggests that the incidence of glucose intolerance and diabetes in neonates and infants with CF may be significantly underestimated.
- Further research and careful monitoring of glucose metabolism in infants with CF are warranted to understand the true prevalence and early management strategies for CFRD.
Unlabelled:
Abnormal glucose tolerance is a frequent late complication of cystic fibrosis (CF), but the prevalence of CF-related diabetes mellitus (CFRD) in children less than 10 y old is less than 2%. The youngest child with CFRD reported to date was 6 mo of age. Insulinopenia is the primary cause of abnormal glucose tolerance/CFRD, but it is unknown whether it may begin in the neonatal period. We describe a case of a neonate with CF who presented with hyperglycaemia in the diabetic range and marked insulinopenia. Insulinopenia and impaired glucose tolerance were permanent findings at 6 and 15 mo of age.
Conclusion:
This case suggests that abnormal glucose tolerance/diabetes may occur much earlier in the course of CF, even during neonatal age. Careful follow-up and further studies in CF infants could reveal that the real incidence of glucose intolerance and diabetes in this age group has been underestimated.
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