BNF for Children 2011-2012

BNFC 2011–2012 2.12 Lipid-regulating drugs 125Side-effects gastro-intestinal disturbances; headache,dizziness; allergic reactions including chills, feverIndication and doseTreatment and prophylaxis of haemorrhage incongenital factor IX deficiency (haemophilia B)Consult haematologistAvailable from CSL Behring (Mononine c ), BPL (Replenine c -VF, Dried Factor IX Fraction), Grifols (AlphaNine c )Note Preparation of recombinant coagulation factor IX(nonacog alfa) available from Wyeth (BeneFIX c )FACTOR XIII FRACTION, DRIED(Human Fibrin-stabilising Factor, Dried)Cautions vaccination against hepatitis A (p. 607) andhepatitis B (p. 608) may be requiredSide-effects rarely allergic reactions and feverIndication and doseCongenital factor XIII deficiencyConsult haematologistAvailable from CSL Behring (Fibrogammin c P)FRESH FROZEN PLASMAFresh frozen plasma is prepared from the supernatant liquidobtained by centrifugation of one donation of whole bloodCautions need for compatibility; vaccination againsthepatitis A (p. 607) and hepatitis B (p. 608) may berequiredContra-indications circulatory overload; avoid use asa volume expanderSide-effects allergic reactions including chills, fever,bronchospasm; acute respiratory distress syndromeIndication and doseReplacement of coagulation factors or otherplasma proteins where their concentration orfunctional activity is critically reducedConsult haematologistAvailable from Regional Blood Transfusion ServicesNote Children under 16 years should only receive virucidallyinactivated preparations of fresh frozen plasma, sourcedfrom ‘low prevalence BSE regions’ such as the USANote A preparation of solvent/detergent treated humanplasma (frozen) from pooled donors is available from Octapharma(Octaplas c )PROTEIN C CONCENTRATEProtein C is prepared from human plasmaCautions hypersensitivity to heparins; vaccinationagainst hepatitis A (p. 607) and hepatitis B (p. 608)may be requiredSide-effects very rarely fever, bleeding, dizziness, andhypersensitivity reactionsIndication and doseCongenital protein C deficiencyConsult haematologistAvailable from Baxter (Ceprotin c )2.12 Lipid-regulating drugsAtherosclerosis begins in childhood and raised serumcholesterolin children is associated with cardiovasculardisease in adulthood. Lowering the cholesterol, withouthindering growth and development in children andadolescents, should reduce the risk of cardiovasculardisease in later life.The risk factors for developing cardiovascular diseaseinclude raised serum cholesterol concentration,smoking, hypertension, impaired glucose tolerance,male sex, ethnicity, obesity, triglyceride concentration,chronic kidney disease, and a family history of cardiovasculardisease. Heterozygous familial hypercholesterolaemiais the most common cause of raisedserum cholesterol in children; homozygous familialhypercholesterolaemia is very rare and its specialisedmanagement is not covered in BNF for Children. Familialhypercholesterolaemia can lead to a greater risk ofearly coronary heart disease and should be managed bya specialist.Secondary causes of hypercholesterolaemia should beaddressed, these include obesity, diet, diabetes mellitus,hypothyroidism (see below), nephrotic syndrome,obstructive biliary disease, glycogen storage disease,and drugs such as corticosteroids.Management The aim of management of hypercholesterolaemiais to reduce the risk of atherosclerosiswhile ensuring adequate growth and development. Childrenwith hypercholesterolaemia (or their carers) shouldreceive advice on appropriate lifestyle changes such asimproved diet, increased exercise, weight reduction, andnot smoking; hypertension should also be managedappropriately (section 2.5). Drug therapy may also benecessary and is discussed below.Hypothyroidism Children with hypothyroidism shouldreceive adequate thyroid replacement therapy beforetheir requirement for lipid-regulating treatment isassessed because correction of hypothyroidism itselfmay resolve the lipid abnormality. Untreated hypothyroidismincreases the risk of myositis with lipidregulatingdrugs.Drug treatment in heterozygous familial hypercholesterolaemiaLifestyle modifications alone areunlikely to lower cholesterol concentration adequatelyin heterozygous familial hypercholesterolaemia anddrug treatment is often required. Lipid-regulatingdrugs should be considered by the age of 10 years.The decision to initiate drug treatment will depend onthe child’s age, the age of onset of coronary heartdisease within the family, and the presence of othercardiovascular risk factors. In children with a familyhistory of coronary heart disease in early adulthood,drug treatment before the age of 10 years, and acombination of lipid-regulating drugs may be necessary.Drug treatment in secondary hypercholesterolaemiaIf 6–12 months of dietary and other lifestyleinterventions has failed to lower cholesterol concentrationadequately, drug treatment may be indicated inchildren 10 years and older (rarely necessary in youngerchildren) who are at a high risk of developing cardiovasculardisease.Choice of drugs Experience in the use of lipidregulatingdrugs in children is limited and they shouldbe initiated on specialist advice.Statins are the drugs of first choice in children and aregenerally well tolerated; atorvastatin and simvastatinare the preferred statins. Other lipid-regulating drugscan be used if statins are ineffective or are not tolerated.2 Cardiovascular system