Pulmonary Arterial Hypertension (PAH), elevated blood pressure in the lungs, is a rare but serious disorder of the blood vessels in the lungs. Although it usually affects adults, it may also develop in childhood. The symptoms in children with PAH are different from those in adults. Children are more likely to suffer brief and sudden periods of unconsciousness than adults. There is also evidence of a characteristic pattern of associated illnesses, including congenital heart defects and other genetic disorders. These are among the findings of a registration study carried out by Professor Rolf Berger from the Paediatric Cardiology department of the Beatrix Children’s Hospital UMCG. Berger concludes that information obtained from research performed in adults with PAH should not be used for diagnosing and treating children with the same condition, without further consideration. The results of the study were recently published in The Lancet.
The article in The Lancet is the first to use data from the so-called TOPP registration (Tracking Outcomes and Practice in Paediatric Pulmonary Hypertension). This world-wide registration was set up in 2008 by Berger and a number of colleagues in order to analyze the specific presentation of PAH in children. 31 centres in 19 countries around the world took part by registering and correlating data on this unique and relatively small group of 456 children.
Berger and colleagues identified various age-related characteristics of PAH, including a specific pattern of clinically relevant associated conditions. A relatively large number of children with PAH also have a genetic defect, such as Down’s syndrome. Sudden and brief periods of unconsciousness (syncope) are another specific symptom in children with PAH. Berger concludes that the specific presentation of PAH in children makes it difficult to extrapolate data obtained from studies in adults. He is arguing the case for research into treatment and results of treatment for PAH specifically among children. The TOPP registration is expected to provide a wealth of information about aspects including treatment strategies, results, the progress of the disease and specific risk factors during childhood.
PAH affects three in every million adults and is even rarer among children. The life expectancy for people with this serious disease is often short. Half of untreated adults diagnosed with PAH die within three years. The life expectancy for children is possibly even shorter. PAH can only be treated with drugs for 10-15 years, and the drugs have only been tested on adults. Very little is known about their use and efficacy when used on children.
The Netherlands has created a unique situation by concentrating all its expertise in a single national centre of excellence for the care of children with PAH in the Beatrix Children’s Hospital in Groningen. As a result, this hospital has the specialized knowledge and experience needed to treat around sixty young PAH patients. Its facilities include the latest equipment for diagnosing the disease and its progression, and the technology for developing new diagnostic techniques specifically for use on children. The hospital offers all the available treatment options for PAH, both drug and non-drug treatment, specially adapted for young its patients.
More information is available from the UMCG press office via +31 (0)50 361 22 00. A summary of the article in The Lancet can be found here: http://www.thelancet.com/journals/lancet/article/PIIS0140-6736(11)61621-8/fulltext#article_upsell
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