Evaluation of Growth Hormone Deficiency in Children with Cystic Fibrosis

Mohsen Reisi1, Niloufar Sharif Ahmadian2, Mahin Hashemipour3

  • 1Department of Pediatrics, Faculty of Medicine, Child Growth and Development Research Center, Research Institute for Primordial Prevention of Noncommunicable Disease, Isfahan University of Medical Sciences, Isfahan, Iran.

Advanced Biomedical Research
|September 20, 2022
PubMed

Insights

Growth hormone deficiency (GHD) affects 6.6% of children with cystic fibrosis (CF), a higher prevalence than in the general pediatric population. Further research is needed to understand the causes of GHD in CF patients.

Area of Science:

  • Pediatric Endocrinology
  • Genetics and Genetic Diseases
  • Pulmonology

Background:

  • Cystic Fibrosis (CF) is associated with chronic respiratory and gastrointestinal issues, frequently leading to growth failure in affected children.
  • Growth hormone deficiency (GHD) is a potential contributor to growth impairment in pediatric CF patients.

Purpose of the Study:

  • To determine the prevalence of GHD in children with CF presenting with stable gastrointestinal and respiratory conditions.
  • To compare GHD prevalence in CF children with a control group of children experiencing growth failure.

Main Methods:

  • A 3-year longitudinal study monitored growth indicators in 150 children (4-16 years) with CF.
  • Children with growth failure (<3% weight percentile or significant height deceleration) and stable CF underwent clonidine and L-dopa GH stimulation tests.
  • A control group of children without CF but with growth failure was included for comparative analysis.

Main Results:

  • Growth failure was observed in 24 CF patients (16%) with stable conditions.
  • GHD was diagnosed in 10 of these CF patients, resulting in a GHD prevalence of 6.6% in the CF cohort.
  • The prevalence of GHD in the control group (9 out of 30) was not significantly different from the CF group (P = 0.37).

Conclusions:

  • The prevalence of GHD in children with CF is 6.6%, significantly higher than the <1% observed in the general pediatric population.
  • The findings suggest a potential link between CF and GHD that warrants further investigation.
  • Additional research is recommended to elucidate the underlying mechanisms causing GHD in CF patients.
Abstract

Related Concept Videos

Cystic Fibrosis: Management01:24

Cystic Fibrosis: Management

Cystic fibrosis (CF) is an autosomal recessive disorder that predominantly affects individuals of Northern European descent, occurring at a rate of 1 in 3500. It is caused by a genetic mutation in a gene on chromosome 7, most commonly the ΔF508 mutation, that codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This results in thicker mucus secretions and obstruction pathologies in multiple organs, including the lungs and sinuses.
Sinus disease and chronic...
214
Cystic Fibrosis: Pathogenesis01:23

Cystic Fibrosis: Pathogenesis

Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation,...
345
Nature and Nurture01:10

Nature and Nurture

Many human characteristics, like height, are shaped by both nature—in other words, by our genes—and by nurture, or our environment. For example, chronic stress during childhood inhibits the production of growth hormones and consequently reduces bone growth and height. Scientists estimate that 70-90% of variation in height is due to genetic differences among individuals, and 10-30% of variation in height is due to differences in the environments that individuals experience,...
20.6K
Hypoglycemia and Glucagon01:15

Hypoglycemia and Glucagon

Without prolonged fasting, healthy individuals maintain blood glucose levels above 3.5 mM due to a well-adapted neuroendocrine counterregulatory system that effectively prevents acute hypoglycemia, a potentially life-threatening condition. The primary clinical scenarios for hypoglycemia encompass diabetes treatment, inappropriate production of endogenous insulin or insulin-like substances by tumors, and the use of glucose-lowering agents in non-diabetic individuals. Notably, hypoglycemia in the...
316