Cystic Fibrosis and ABPA

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November 1, 2007

Towards an early diagnosis: Cystic fibrosis patients with fungal

complication

By Tanuja Rohatgi,

Checkbiotech -Basel,Switzerland*

http://www.checkbiotech.org/orphan_News_treatmentandhealth.aspx?

infoId=1565

Many people with Cystic Fibrosis suffer from a condition called

Allergic Brochopulmonary Apergillosis (ABPA) that only worsens the

health of the individual. However, recent research with an ABPA

marker could lead to better diagnosis and treatment.

In our lungs may reside an opportunistic fungus called Aspergillus

fumigatus. This fungus is ubiquitous in nature and inhaled by

everybody. However, in people with Cystic Fibrosis (CF) and asthma,

it can often lead to a condition called Allergic Bronchopulmonary

Aspergillosis (ABPA)

What occurs is fungal spores are trapped in the lungs of CF patients

due to the accumulation of thick mucus, which is one of the symptoms

of CF. The constant present of the spore in the lungs leads to an

immune response known as a hypersensitive allergic reaction. This

further aggravates the condition of patients suffering from CF.

Diagnosis of ABPA in CF patients is both difficult and time-

consuming because of the overlapping signs and symptoms caused by CF

and ABPA.

Understanding the problem, Dr. Philipp Latzin and his group at the

University of Bern and University of Munich Children's hospital set

out to identify a possible blood marker that would help detect the

development of ABPA in CF patients.

During their quest for a marker, Dr. Latzin's research team followed

a group of CF patients and found elevated levels of a protein in the

serum of CF patients with ABPA. This protein is called TARC and is

involved in a pathway which promotes an immune response to certain

fungi. The group has reported their findings in a recent issue of

European Respiratory Journal.

What makes this marker all the more interesting is its specificity

and the fact that, unlike other available serological markers, the

levels of TARC are considerably elevated early in the course of

disease, before the appearance of clinical symptoms.

Early diagnosis means a chance for a better quality of life with a

possibility of an early and effective treatment for CF patients with

ABPA. Dr. Latzin told Checkbiotech, " If TARC really proves to be

able to detect ABPA patients early in the disease course, this could

mean earlier - and possibly shorter - treatment and lead to fewer

complications. "

A call for support

As good as TARC looks in the preliminary studies that Dr. Latzin's

team recently published, there is still some work to be done before

TARC can be accepted as a certified marker. Dr. Latzin explained

that the next step would be to conduct much larger studies to

clearly establish TARC as a diagnostic marker for ABPA in CF.

However, larger studies require a significant amount of funding. " We

are planning further studies, but we lack the necessary funding at

the moment, " acknowledged Dr. Latzin. Success in larger trials would

allow physicians to gain quicker access to a valuable tool to help

people with CF.

ABPA in Cystic Fibrosis

To live we need to breath, but with every single breath we end up

inhaling countless number of microbes, which are ubiquitous in the

air. Most of these microbes end up in our lungs, where they can grow

comfortably at human body temperature. Fortunately, our body's

immune system prevents these microbes from becoming a problem.

When our body is functioning correctly, most microbes simply do no

affect us. However if our body is already suffering from another

disease, then it is much more susceptible to microbes that often

would never cause a problem.

Cystic fibrosis (CF) is one such condition, where the body's immune

response is highly compromised. CF is a hereditary disorder and

occurs as a result of inheritance of two copies of the defective

gene, one from each parent. People affected with CF often develop

problems in the lungs, pancreas and the digestive tract.

In the lungs, the cells responsible for producing mucus produce

excessive amount which leads to difficulty in breathing. The thick

mucus is not only produced in the lungs, it is also produced in the

digestive tract, which compromises the ability of the affected

person to digest food properly. Normally the CF symptoms arise

during early childhood and lead to progressive disability and early

death.

Currently, there is no cure for CF except for lung transplantation.

It is possible to detect CF prior to birth by genetic testing or in

early childhood by a simple sweat test. In the United States, 1 in

3900 children is born with CF, with a high prevalence rate among

Caucasian populations. What makes matters worse for CF patients is

the high probability of acquiring secondary complications such as

ABPA.

Contact:

Philipp Latzin

Division of Respiratory Medicine

Children's University Hospital of Berne

Switzerland

philipp.latzin@...

Tanuja Rohatgi is a Science Writer for Checkbiotech in Basel,

Switzerland

Source: Checkbiotech