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Purpose of Review <p>This review summarizes current guidelines and emerging evidence pertaining to the screening and management of endocrine manifestations of cystic fibrosis (CF) in the pediatric population, including when endocrinology consultation is indicated.</p> Recent Findings <p>Due to advances over time, people with CF (PwCF) have improved health outcomes, quality of life, and longevity. More than 90% of individuals with CF are eligible for highly effective modulator therapy (HEMT), those with gating mutations as early as 1 month of age and those with at least one F508del mutation as early as 2 years of age. This brings hope that PwCF will experience a protective effect regarding pancreatic beta cell mass preservation, growth, weight gain, and bone health. On the other hand, there may be relatively more adipose mass gain with HEMT.</p> Summary <p>The specific areas covered include nutrition principles, growth attenuation and pubertal delay, overweight and obesity, dysglycemia and CF-related diabetes (CFRD), CF-related hypoglycemia, and CF-related bone disease (CFBD). The traditional CF “legacy” diet consisting of high-fat, high-caloric intake requires a paradigm shift towards more nutrient dense, anti-inflammatory foods rich in antioxidants and lower in saturated fats to promote optimal nutritional status, health, and longevity. Effective management of endocrine complications in PwCF requires collaboration with the CF care team, along with awareness of comorbid systemic complications (e.g., pancreatic insufficiency, gastroparesis, liver disease), respiratory function, HEMT use (including initiation, pauses, and resumption), and corticosteroid exposure history.</p>

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Endocrine Complications of Cystic Fibrosis in the Era of Early Highly Effective Modulator Initiation

  • 期刊论文

  • 发表日期：

    2025年7月11日

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  • Dhakshitha Peddisetty,
  • Diane Paralusz,
  • Danielle Mein,
  • Lindsay Pappas,
  • Rebecca Hicks

摘要

Purpose of Review

This review summarizes current guidelines and emerging evidence pertaining to the screening and management of endocrine manifestations of cystic fibrosis (CF) in the pediatric population, including when endocrinology consultation is indicated.

Recent Findings

Due to advances over time, people with CF (PwCF) have improved health outcomes, quality of life, and longevity. More than 90% of individuals with CF are eligible for highly effective modulator therapy (HEMT), those with gating mutations as early as 1 month of age and those with at least one F508del mutation as early as 2 years of age. This brings hope that PwCF will experience a protective effect regarding pancreatic beta cell mass preservation, growth, weight gain, and bone health. On the other hand, there may be relatively more adipose mass gain with HEMT.

Summary

The specific areas covered include nutrition principles, growth attenuation and pubertal delay, overweight and obesity, dysglycemia and CF-related diabetes (CFRD), CF-related hypoglycemia, and CF-related bone disease (CFBD). The traditional CF “legacy” diet consisting of high-fat, high-caloric intake requires a paradigm shift towards more nutrient dense, anti-inflammatory foods rich in antioxidants and lower in saturated fats to promote optimal nutritional status, health, and longevity. Effective management of endocrine complications in PwCF requires collaboration with the CF care team, along with awareness of comorbid systemic complications (e.g., pancreatic insufficiency, gastroparesis, liver disease), respiratory function, HEMT use (including initiation, pauses, and resumption), and corticosteroid exposure history.