The study, led by University of Iowa researchers and published online the week of Nov. 8 in the Early Edition of the Proceedings of the National Academy of Sciences, could help predict the severity of the disease in patients and may lead to new therapies for growth defects in people with CF.
Growth defects are common in people with CF and have been blamed, in part, on low levels of the growth-promoting hormone called insulin-like growth factor 1 (IGF1). Traditionally, the malnutrition and lung inflammation that accompany CF have been blamed for the decreased levels of IGF1. However, even patients who are relatively healthy often do not reach their full growth potential, and newborns with CF often are smaller at birth than healthy babies.
To investigate the relationship between neonatal IGF1 levels and growth patterns in CF, the research team studied newborn pigs with a CF-causing gene mutation. This animal model, which was generated by the UI researchers and colleagues at the University of Missouri in 2008, has many of the same symptoms and complications that are seen in humans with CF.
"By examining IGF1 at this time point, we eliminated consequences of lung inflammation, which is absent at birth, and malnutrition, because nutrition in utero is provided by the mother," explained Leah Reznikov, Ph.D., UI postdoctoral fellow in internal medicine and co-first author of the study. "We found that IGF1 levels were significantly reduced at birth in CF newborn pigs."
In addition, the UI researchers found that newborn CF pigs had shorter, smaller bones than pigs without CF suggesting that decreased IGF1 levels are associated with the growth defects, and that IGF1 levels may be reduced even before the pigs are born.
These findings led Reznikov and colleagues, including co-first author Mark Rogan, M.D., a former UI postdoctoral fellow in internal medicine, to examine levels of IGF1 in newborn humans with CF.
By testing blood samples collected through the Iowa Neonatal Metabolic Screening Program and the Iowa Department of Public Health, the researchers found that infants with CF have reduced IGF1 levels compared to healthy infants.
"Collectively, these findings suggest that IGF1 deficits begin very early in the course of CF disease and reductions in IGF1 may, in part, explain growth defects observed at birth in infants with CF," Reznikov said. "The findings also imply that IGF1 may serve as a potential biomarker of the disease and may be useful in prognostication, care and treatment of people with CF."
Patients with CF currently receive replacement pancreatic enzymes and insulin supplementation to counteract effects of CF. One possibility raised by the new findings is that IGF1 supplementation, beginning in infancy, might also be beneficial for growth in patients with CF. However, Reznikov cautioned that more testing is needed before this approach could be tested in humans.
"We would like to increase the sample size in our human studies and examine other parameters to better understand the relationship among CF, IGF1 and growth defects," she said."
If these test results are positive, Reznikov noted that the CF pigs would provide an excellent preclinical system to test whether IGF1 supplementation would be beneficial early in CF.
In addition to Reznikov and Rogan, the research team included Michael Welsh, M.D., UI professor of internal medicine and molecular physiology and biophysics and a Howard Hughes Medical Institute investigator, and David Stoltz, M.D., Ph.D., UI assistant professor of internal medicine. UI researchers Alejandro Pezzulo; Nicholas Gansemer; Joseph Zabner; Douglas Fredericks; and Paul McCray Jr.; and University of Missouri researchers Melissa Samuel and Randall Prather also contributed to the study.
The research was funded in part by grants from the National Institutes of Health and the Cystic Fibrosis Foundation.
STORY SOURCE: University of Iowa Health Care Media Relations, 200 Hawkins Drive, Room W319 GH, Iowa City, Iowa 52242-1009
MEDIA CONTACT: Jennifer Brown, 319-356-7124, firstname.lastname@example.org
Jennifer Brown | EurekAlert!
Smart Data Transformation – Surfing the Big Wave
02.12.2016 | Fraunhofer-Institut für Angewandte Informationstechnik FIT
Climate change could outpace EPA Lake Champlain protections
18.11.2016 | University of Vermont
A multi-institutional research collaboration has created a novel approach for fabricating three-dimensional micro-optics through the shape-defined formation of porous silicon (PSi), with broad impacts in integrated optoelectronics, imaging, and photovoltaics.
Working with colleagues at Stanford and The Dow Chemical Company, researchers at the University of Illinois at Urbana-Champaign fabricated 3-D birefringent...
In experiments with magnetic atoms conducted at extremely low temperatures, scientists have demonstrated a unique phase of matter: The atoms form a new type of quantum liquid or quantum droplet state. These so called quantum droplets may preserve their form in absence of external confinement because of quantum effects. The joint team of experimental physicists from Innsbruck and theoretical physicists from Hannover report on their findings in the journal Physical Review X.
“Our Quantum droplets are in the gas phase but they still drop like a rock,” explains experimental physicist Francesca Ferlaino when talking about the...
The Max Planck Institute for Physics (MPP) is opening up a new research field. A workshop from November 21 - 22, 2016 will mark the start of activities for an innovative axion experiment. Axions are still only purely hypothetical particles. Their detection could solve two fundamental problems in particle physics: What dark matter consists of and why it has not yet been possible to directly observe a CP violation for the strong interaction.
The “MADMAX” project is the MPP’s commitment to axion research. Axions are so far only a theoretical prediction and are difficult to detect: on the one hand,...
Broadband rotational spectroscopy unravels structural reshaping of isolated molecules in the gas phase to accommodate water
In two recent publications in the Journal of Chemical Physics and in the Journal of Physical Chemistry Letters, researchers around Melanie Schnell from the Max...
The efficiency of power electronic systems is not solely dependent on electrical efficiency but also on weight, for example, in mobile systems. When the weight of relevant components and devices in airplanes, for instance, is reduced, fuel savings can be achieved and correspondingly greenhouse gas emissions decreased. New materials and components based on gallium nitride (GaN) can help to reduce weight and increase the efficiency. With these new materials, power electronic switches can be operated at higher switching frequency, resulting in higher power density and lower material costs.
Researchers at the Fraunhofer Institute for Solar Energy Systems ISE together with partners have investigated how these materials can be used to make power...
16.11.2016 | Event News
01.11.2016 | Event News
14.10.2016 | Event News
02.12.2016 | Medical Engineering
02.12.2016 | Agricultural and Forestry Science
02.12.2016 | Physics and Astronomy