Very early glucose tolerance abnormalities in children with cystic fibrosis
Marie Blanquer1, Laurence Le Clainche1, Elise Bismuth2
1University Robert Debré Hospital, Cystic Fibrosis Center, 48 bd Serurier, 75019 Paris, France.
Insights
Early diabetes and glucose intolerance can occur in infants with cystic fibrosis (CF), sometimes resolving spontaneously. Diagnosis in this age group is challenging, impacting prognosis and nutritional status.
Area of Science:
- Pediatrics
- Endocrinology
- Pulmonology
Background:
- Cystic Fibrosis (CF) is often associated with diabetes, worsening patient prognosis.
- Abnormal glucose tolerance in CF correlates with reduced lung function and poor nutritional status.
- Limited data exist on glucose tolerance abnormalities in infants with CF.
Observation:
- Three infants with cystic fibrosis presented with abnormal glucose tolerance before one year of age.
- Two infants were diagnosed with very early-onset diabetes before six months of age.
- All patients experienced early Pseudomonas aeruginosa lung infections and suboptimal nutritional status.
Findings:
- Despite early dysglycaemia, including very early diabetes, none required long-term insulin therapy.
- Glycaemia levels spontaneously improved over time in all observed cases.
- Early pulmonary infection and poor nutrition were common factors in these infants.
Implications:
- This case series expands the understanding of early dysglycaemia spectrum in cystic fibrosis.
- Highlights diagnostic challenges of glucose metabolism disorders in infants with CF.
- Suggests potential for spontaneous glycaemic improvement in select young CF patients.
Abstract:
Diabetes is a comorbidity of cystic fibrosis (CF) that worsens prognosis. Abnormal glucose tolerance is associated with decreased lung function and poorer nutritional status. Data are lacking on glucose tolerance abnormalities in young children. We report three infants with abnormal glucose tolerance, beginning under the age of one year, including two cases of very early diabetes which started before the age of six months. None of our patients required long-term insulin treatment, and glycaemia spontaneously improved. All three patients had early pulmonary infection with Pseudomonas aeruginosa and poor nutritional status. This case series presents three unique patients with early dysglycaemia, then improvement over time. This adds to the understanding of the spectrum of early dysglycaemia in CF and highlights the difficulty of diagnosis in this age group.
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