Long-Term Follow-Up in a Girl with Cystic Fibrosis and Diabetes Since the First Year of Life.

Valentina Fattorusso,Alida Casale,Adriana Franzese,Valeria Raia,Enza Mozzillo

doi:10.1007/s13300-017-0289-9

Valentina Fattorusso, Alida Casale + Show 3 more

Open Access

https://doi.org/10.1007/s13300-017-0289-9

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Journal: Diabetes Therapy	Publication Date: Aug 2, 2017
Citations: 6	License type: CC BY 4.0

Affiliation: University of Naples Federico II

Abstract

Diabetes mellitus is the most common comorbidity in cystic fibrosis (CF). Recently, more attention has been paid to early glucose metabolism derangements (GMDs). The subject of this report is a female patient, affected by CF since 3 months of age. She presented with intermittent diabetes during early childhood. At the age of 10 years, oral glucose tolerance test (OGTT) was performed and showed glucose intolerance (IGT) status; glargine insulin therapy was started. At the age of 13 years, CF-related diabetes with fasting hyperglycemia occurred, so rapid insulin at meals was added. During the following year, clinical and nutritional status improved. Stable clinical conditions were observed in the following 3 years. This is the first case of very long-term follow-up concerning a CF patient with GMDs. Our case confirms the importance of paying attention to early GMDs in very young CF patients and seems to suggest that earlier therapy could ameliorate CF natural history.

Highlights

Diabetes mellitus is the most common comorbidity in cystic fibrosis (CF) people, occurring in 40–50% of adult patients and in a variable number of children and adolescents
More attention has been paid to other less severe glucose metabolism derangements (GMDs), since prediabetes may be related to increased morbidity, and early treatment may improve the clinical course in patients with CF [1]
According to the American Diabetes Association (ADA) classification, diabetes diagnosis is made in CF patients who present fasting blood glucose values greater than 7 mmol/l or glycemia greater than 11.1 mmol/l at time 120 of the oral glucose tolerance test (OGTT); the former condition is classified as CF-related diabetes–fasting hyperglycemia positive (CFRD-FH?) and negative (CFRD-FH-) respectively

Summary

INTRODUCTION

Diabetes mellitus is the most common comorbidity in cystic fibrosis (CF) people, occurring in 40–50% of adult patients and in a variable number of children and adolescents. At the age of 10 years, OGTT was performed according current guidelines [1] away from pulmonary exacerbation and steroid therapy; it showed IGT status (glycemia peak at T90 of 16.88 mmol/L); contemporary clinical conditions were worsening [increased number of pulmonary exacerbations, worsening of BMI z score, and mean forced expiratory volume in 1 s % (FEV1%)]. Stable clinical conditions were observed in the following 3 years When she was 16 years old, an increased rate of pulmonary exacerbations was observed, FEV1% significantly decreased to 70.5%, BMI z score was -0.5 (Table 1), and HbA1c reached a value of 66.1 mmol/mol

DISCUSSION

Findings

CONCLUSION