Forum for Science, Industry and Business

Sponsored by:     3M 
Search our Site:

 

Low blood oxygen may lead to heart defects in children with sickle cell disease

28.04.2010
Children with sickle cell disease who also have lower blood oxygen levels while both asleep and awake are likely to have heart abnormalities, researchers at Washington University School of Medicine in St. Louis and other institutions have found.

Heart problems are fairly common in young adults with sickle cell disease, but physicians don't fully understand why. The researchers demonstrated that lower oxygen saturation in the blood was linked to the heart structure seen in the 44 children studied.

Sickle cell disease is an inherited blood disorder affecting red blood cells, which contain hemoglobin, the substance that carries oxygen from the lungs to all parts of the body. In patients with this disease, red blood cells contain an abnormal type of hemoglobin that causes the normally round, flexible red blood cells to become stiff and sickle- or crescent-shaped. The sickle cells can't pass through tiny blood vessels, which can prevent blood from reaching some tissues and can result in tissue and organ damage, pain and stroke.

In addition, sickle cells are short lived and lead to a shortage of red blood cells and anemia, which make the heart grow bigger because it has to work harder, says Mark C. Johnson, MD, associate professor of pediatrics at Washington University School of Medicine and first author of the study.

In this study, the first to analyze sleep studies and echocardiograms of children with sickle cell disease, these heart abnormalities were found in the left pumping chamber, or left ventricle, of the children's hearts. The findings included an enlarged left ventricle, called ventricular hypertrophy, and abnormal blood filling of the left ventricle, called diastolic dysfunction. Both are associated with early death in adults with sickle cell disease, but the meanings of the same results in children are unclear.

"This suggests that the beginning of adult heart disease may start in children, but we need to follow these patients longitudinally to strengthen the meaning of the findings," says Michael R. DeBaun, MD, a Washington University sickle cell disease specialist at St. Louis Children's Hospital and senior author of the study, published online in Blood First Edition April 8, 2010.

"Many researchers assumed it was the anemia alone that makes the heart enlarge, but this study suggests it's not that simple," Johnson says.

Researchers began the study thinking that the abnormalities in the left ventricle, the heart's main blood-pumping chamber, would be the result of sleep-disordered breathing, such as sleep apnea, commonly found in children with sickle cell disease. Sleep-disordered breathing has previously been associated with left ventricle hypertrophy and with diastolic dysfunction in children and adults. But only about one-fifth of the children had some evidence of obstructive apnea hypopnea, or recurrent episodes of upper airway collapse and obstruction during sleep.

Researchers also thought they would find lower oxygen saturation while patients were sleeping compared to when they were awake. However, the average oxygen saturation while asleep and awake were similar. Only about one-fourth of patients had average oxygen saturation values below normal.

The echocardiograms showed that 46 percent of participants had left ventricle hypertrophy in which the chamber of the left ventricle was enlarged. The research team's analysis showed that for every 1 percent drop in oxygen saturation, there was a measurable increase in the mass of the heart's left ventricle.

"The average oxygen saturation of 97 percent in these children is in a normal range, but the patients with slightly lower levels had enlarged hearts," Johnson says. "A relatively small change in oxygen levels caused a big change in the heart. That's what makes us think there's something under the surface that we don't quite understand yet."

The team also measured an indicator of pulmonary hypertension, or high blood pressure in the lungs, the major cause of death in adults with sickle cell disease. When the pulmonary hypertension level is raised in adults with sickle cell disease, it is a strong predictor of death within 24 months. However, despite the strong association between pulmonary hypertension and sleep abnormalities, no such association was demonstrated in this study, DeBaun says.

Johnson and DeBaun say more sleep and cardiac studies are needed to confirm the association of left ventricle abnormalities with low blood oxygen levels. In the future, the researchers plan to look at other indicators to find potential new therapies to prevent progression of the disease.

Johnson M, Kirkham F, Redline S, Rosen C, Yan Y, Roberts I, Gruenwald J, Marek J, DeBaun M. Left Ventricular Hypertrophy and Diastolic Dysfunction in Children with Sickle Cell Disease are Related to Asleep and Waking Oxygen Desaturation. Blood First Edition. Prepublished online April 8, 2010.

Funding for this study was provided by the National Heart, Lung, and Blood Institute.

Washington University School of Medicine's 2,100 employed and volunteer faculty physicians also are the medical staff of Barnes-Jewish and St. Louis Children's hospitals. The School of Medicine is one of the leading medical research, teaching and patient care institutions in the nation, currently ranked fourth in the nation by U.S. News & World Report. Through its affiliations with Barnes-Jewish and St. Louis Children's hospitals, the School of Medicine is linked to BJC HealthCare.

Beth Miller | EurekAlert!
Further information:
http://www.wustl.edu

More articles from Health and Medicine:

nachricht Penn vet research identifies new target for taming Ebola
12.01.2017 | University of Pennsylvania

nachricht The strange double life of Dab2
10.01.2017 | University of Miami Miller School of Medicine

All articles from Health and Medicine >>>

The most recent press releases about innovation >>>

Die letzten 5 Focus-News des innovations-reports im Überblick:

Im Focus: Designing Architecture with Solar Building Envelopes

Among the general public, solar thermal energy is currently associated with dark blue, rectangular collectors on building roofs. Technologies are needed for aesthetically high quality architecture which offer the architect more room for manoeuvre when it comes to low- and plus-energy buildings. With the “ArKol” project, researchers at Fraunhofer ISE together with partners are currently developing two façade collectors for solar thermal energy generation, which permit a high degree of design flexibility: a strip collector for opaque façade sections and a solar thermal blind for transparent sections. The current state of the two developments will be presented at the BAU 2017 trade fair.

As part of the “ArKol – development of architecturally highly integrated façade collectors with heat pipes” project, Fraunhofer ISE together with its partners...

Im Focus: How to inflate a hardened concrete shell with a weight of 80 t

At TU Wien, an alternative for resource intensive formwork for the construction of concrete domes was developed. It is now used in a test dome for the Austrian Federal Railways Infrastructure (ÖBB Infrastruktur).

Concrete shells are efficient structures, but not very resource efficient. The formwork for the construction of concrete domes alone requires a high amount of...

Im Focus: Bacterial Pac Man molecule snaps at sugar

Many pathogens use certain sugar compounds from their host to help conceal themselves against the immune system. Scientists at the University of Bonn have now, in cooperation with researchers at the University of York in the United Kingdom, analyzed the dynamics of a bacterial molecule that is involved in this process. They demonstrate that the protein grabs onto the sugar molecule with a Pac Man-like chewing motion and holds it until it can be used. Their results could help design therapeutics that could make the protein poorer at grabbing and holding and hence compromise the pathogen in the host. The study has now been published in “Biophysical Journal”.

The cells of the mouth, nose and intestinal mucosa produce large quantities of a chemical called sialic acid. Many bacteria possess a special transport system...

Im Focus: Newly proposed reference datasets improve weather satellite data quality

UMD, NOAA collaboration demonstrates suitability of in-orbit datasets for weather satellite calibration

"Traffic and weather, together on the hour!" blasts your local radio station, while your smartphone knows the weather halfway across the world. A network of...

Im Focus: Repairing defects in fiber-reinforced plastics more efficiently

Fiber-reinforced plastics (FRP) are frequently used in the aeronautic and automobile industry. However, the repair of workpieces made of these composite materials is often less profitable than exchanging the part. In order to increase the lifetime of FRP parts and to make them more eco-efficient, the Laser Zentrum Hannover e.V. (LZH) and the Apodius GmbH want to combine a new measuring device for fiber layer orientation with an innovative laser-based repair process.

Defects in FRP pieces may be production or operation-related. Whether or not repair is cost-effective depends on the geometry of the defective area, the tools...

All Focus news of the innovation-report >>>

Anzeige

Anzeige

Event News

12V, 48V, high-voltage – trends in E/E automotive architecture

10.01.2017 | Event News

2nd Conference on Non-Textual Information on 10 and 11 May 2017 in Hannover

09.01.2017 | Event News

Nothing will happen without batteries making it happen!

05.01.2017 | Event News

 
Latest News

Solar Collectors from Ultra-High Performance Concrete Combine Energy Efficiency and Aesthetics

16.01.2017 | Trade Fair News

3D scans for the automotive industry

16.01.2017 | Automotive Engineering

Nanoparticle Exposure Can Awaken Dormant Viruses in the Lungs

16.01.2017 | Life Sciences

VideoLinks
B2B-VideoLinks
More VideoLinks >>>