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  • Maternal PKU – Part 2: Twins

    This is part 2 of Angela’s story on PKU and Pregnancy.... Read more

  • Maternal PKU – Part 1

    During a recent study day in London, Angela Thomas shared her experience of tackling the subject of PKU and Pregnancy.... Read more

  • Specific nutrition as an integrated part of pressure ulcer care

    Pressure ulcers are a common problem in all healthcare sectors, affecting quality of life and increasing healthcare costs. Providing sufficient amounts of protein... Read more

  • Targeting immunity: an illustration of HIV disease

    Infection with the human immunodeficiency virus (HIV) results in progressive destruction of the immune system, ultimately resulting in opportunistic infections and AIDS. HIV-infection, affecting 40 million ... Read more

  • Immunopharmacology of non-digestible carbohydrates, a breakthrough for clinical nutrition?

    Early in human history, nutrition and pharmacology were closely linked as people derived their medicines ... Read more

PKU and other metabolic diseases, now and then…

June 29, 2008
    As part of meatbolic awareness week over in the UK, I thought I’d write this blog for people who want to know more about meatbolic diseases and what we, as medics, hope the future holds for them.

      In recent years, many genetic mutations have been discovered that lead to metabolic disorders. It is now scientifically possible for individuals or couples to choose to be tested for these genes, this is usually a route only taken by people who have these diseases running in their family. This is to give parents more information about the likelihood that their children will develop such diseases.

        One such disorder occurs when newborns are missing a particular gene that helps metabolize the amino acid phenylketonuria (PKU). The build up of dangerous levels of PKU, and the resulting brain damage, can be prevented with a strict diet. Heel prick testing is common practice in most developed countries for all newborns, this is to discover PKU at as young an age as possible and this compulsory testing has greatly reduced the damage from this relatively common metabolic abnormality.

          There are other benefits to diagnosing metabolic diseases as soon as possible in addition to prompt treatment, if it is available. Early identification can also minimize the amount of testing a child has to undergo throughout their childhood, and can provide parents, Health Care Professionals, and teachers with a clearer picture of what to expect in the future. This is why it is so important to catch these diseases early.

            In the future we may be able to screen newborns for many more diseases, letting doctors start more  treatment before a child has suffered symptoms and possible brain damage. Similarly, genetic tests for carriers should enable more couples to be aware of genetic risks to their offspring, and permit prenatal diagnosis. This is an ethical question though and should be one answered on a case by case basis, taking in to account the best interests of the parents and, more importantly, the child

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