First part of information on gangs this month, plus HbA1c units compared, last bit on orthopaedic feet, a warning about phenytoin overdose and a couple of links to good relevant courses. Do leave comments below:
Dr Yasmeen Moin is one of our senior registrars, currently nursing a broken leg and making good use of her time writing accessible articles for Paediatric Pearls! This month she has summarised all the salient points about Kawasaki Disease which is not as rare as people think. Her article is below, under the characteristic “strawberry tongue”.
Kawasaki disease (KD) is an acute febrile childhood vasculitis affecting medium sized arteries, particularly the coronary arteries.
It most commonly affects children aged between 6 months and 4 years, however it can occur in children up to the age of 16.
Diagnosis is based on persistent fever, of 5 days or more, plus 4 of the following:
1) Conjunctivitis - bilateral, bulbar, non suppurative
2) Lymphadenopathy - cervical >1.5 cm
3) Rash - widespread, polymorphous, NOT vesicular
4) Lips and oral mucosa - red cracked lips, “strawberry” tongue, erythematous oral cavity
5) Changes of extremities – erythema, oedema of palms and soles initially, then peeling of skin at a later stage
Fever is often >39°, remittent and unresponsive to antibiotics and antipyretics.
Incomplete KD can occur where not all diagnostic criteria are met. It is more common in children less than a year old. Think KD when prolonged unexplained fever even if less than four diagnostic criteria.
Children with KD are often extremely irritable. Think KD if fever, irritable and skin manifestations.
KD is often accompanied by concurrent infections which may be misleading.
Why is it important to diagnose?
KD is the commonest cause of acquired cardiac disease in the UK and USA with 25% of untreated cases of KD developing cardiac complications. Timely treatment reduces coronary artery damage by up to 75%. Children less than 1 year of age with incomplete KD have a higher risk of developing cardiac sequelae.
I’ve not seen one before, how common is it?
KD is relatively common with an annual incidence in the UK and United States of approximately 9-12 per 100 000 children under 5 years (compared to an age- matched incidence of meningococcal disease of approximately 1 per 100 000).
What do I need to do?
Children with suspected KD should be referred to the Paediatric team urgently, as early treatment significantly reduces the risk of long term cardiac artery damage.
If there is a high index of suspicion, referral should be made before 5 days of fever.
Diagnosis is clinical; however there are characteristic laboratory findings:
Elevated WBCs, mainly neutrophils
Markedly elevated ESR and CRP
Normocytic, normochromic anaemia
Mildly elevated LFTs, mainly ALT
Normal platelet count that increases markedly in week two of illness
How is it treated?
Treatment involves admission to a paediatric ward and administration of IV immunoglobulin (IVIG). This causes rapid defervescence and clinical improvement in 80% of cases. High dose aspirin is also commenced (dosing regimen will vary according to local policy). A second dose of IVIG may be required if there is no response to the initial dose. Anti-viral IgG in IVIG may interfere with the efficacy of live vaccines.
An ECHO, looking in particular for coronary artery aneurysms, is usually performed during the initial admission and then at 6 weeks (or sooner, again depending on local policy).
Once the fever has resolved, aspirin is continued at a low dose and in those children with a normal ECHO at 6 weeks, it is discontinued.
What follow up should I expect?
Long term, often lifelong, paediatric cardiology follow up is required. Repeat ECHOs are carried out regularly (as per local policy). Lifelong aspirin and anticoagulant therapy may be required if aneurysms persist, if they are >8mm and if stenoses are identified. Further investigations, such as coronary angiography, may need to be carried out. The long term effects of KD on adult coronary disease are unclear but the family should be given advice on minimising atheroma risk factors. Early monitoring of blood pressure and lipids should be considered.
Parents should be reassured that KD is not contagious and that most children make a full recovery without any long term sequelae.
Kawasaki disease. The importance of prompt recognition and early referral Volume 42, No.7, July 2013 Pages 473-476
Surveillance of Complete Kawasaki Disease in the UK and Ireland, RCPCH
Whipps Cross Paediatric Unit Guidelines
http://circ.ahajournals.org/content/110/17/2747.full.pdf+html provides a full text 26 page in-depth discussion on everything to do with Kawasaki Disease. Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease. A Statement for Health Professionals From the Committee on Rheumatic Fever, Endocarditis and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association. Jane W. Newburger, MD, MPH;Masato Takahashi, MD et al. Circulation. 2004; 110: 2747-2771
Delayed sleep phase this month and chronotherapy which sounds like quite an undertaking. Also a link to a new parent’s guide to picking up and talking about sexual abuse, links to handy recent uploads to the site, the BSACI guideline on allergic rhinitis and more banging on about vitamin D supplementation – please.
Have a look at the November 2010 Paediatric Pearls PDF digest for information on bronchiolitis, heart murmurs and burns. The featured NICE guideline this month was Nocturnal Enuresis which, even with the best will in the world, has very little to do with Accident and Emergency! Those of you who are interested in the topic could look at this month’s GP version.
November’s Paediatric Pearls is now published and is available for download here. It sees the start of our 6 week check series, kicking off with information on heart murmurs. There is also a bit on bronchiolitis as the season is upon us now and a feature on the NICE guideline on nocturnal enuresis which was published at the end of October 2010.
With thanks to Dr Tom Waterfield for his work on this article as part of our series on the 6 week baby check…
The assessment of murmurs can be difficult and identification of the underlying pathology, if any, is best left to a specialist. For the purpose of the 6 week screening test all murmurs should be referred for further assessment but it is important to identify those children that require urgent assessment from the vast majority that simply require re-assurance and routine referral to a general paediatrician or paediatric cardiologist.
A hypothetical model of heart murmurs in children aged 1 month to 18 years done by an NHS economic evaluation team (http://www.crd.york.ac.uk/CRDWeb/ShowRecord.asp?ID=22002001874) suggested that only approximately 2% of these murmurs would be due to an underlying structural anomaly. I think the wide age range might undermine this statistic’s relevance to the 6 week check cohort of babies. A more widely quoted reference from 1998 reports that of 50 healthy term babies with heart murmurs, in 64% the murmur had disappeared by 6 weeks of age. None of the babies had clinically significant congenital heart disease and had all disappeared by 6 months of age but one baby had developed a different innocent murmur! Read the full text of this paper at http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1720793/pdf/v078p0F166.pdf.
If a murmur is detected it is important to consider the likelihood of there being significant underlying disease before referring for further assessment and before discussing the murmur with the parents.
Innocent murmurs are common in children and are associated with normal weight gain, normal feeding and an otherwise normal clinical examination.
The key points of clinical assessment include:
1) Assessment of the murmur
- Location on the precordium e.g. left sternal edge (Common site of innocent murmurs)
- Intensity (1/6 = almost inaudible, 3/6 = audible with a stethoscope, 6/6 easily audible without a stethoscope and associated with a palpable thrill). Murmurs are more often described now as soft or loud, harsh or musical.
- Variability with position – a feature of innocent murmurs (but not something one necessarily notices at the 6/52 check)
- Diastolic Vs Systolic – diastolic murmurs should always be treated as pathological
2) Assessment of perfusion
- A pink child with a capillary refill time <2 seconds and with good peripheral pulses (including femoral pulses) is less likely to have significant heart disease
- Absent femoral pulses may represent coarctation of the aorta
- Cyanotic heart lesions rarely present outside the immediate perinatal period
3) Assessment for signs of heart failure
- Failure to thrive and recurrent respiratory infections
- Respiratory distress with hepatomegaly. Crackles are not really heard in heart failure in infants
- Peripheral oedema and a raised JVP are NOT features of heart failure in this age group and their absence is not reassuring
If a child has a soft systolic murmur at the 6 week check and is growing well and feeding normally with an otherwise normal examination the child can be referred routinely to paediatric outpatients and the parents reassured that the murmur is likely to be due to the normal flow of blood around the heart. If there are any worrying features then the child should ideally be discussed with the paediatric registrar on call and the appropriate referral or admission discussed.
The British Heart Foundation has a factfile sheet for GPs on heart murmurs in children but it is not downloadable from http://www.bhf.org.uk because it is over 5 years old and they worry that their old factsheets may go out of date. BHF have however very kindly sent it to me and allowed me to upload it on to this site as an educational resource. Download it here.
http://www.patient.co.uk/doctor/Heart-Murmurs-in-Children.htm would be a good resource except that it still talks about antibiotics for children with heart defects when they have dental treatment and we gave up doing that in 2008 after NICE said it wasn’t necessary (http://www.nice.org.uk/nicemedia/live/11938/40014/40014.pdf).
http://www.tinytickers.org is a parental support website concerned primarily with antenatal diagnosis and screening.
http://kidshealth.org/parent/medical/heart/murmurs.html# is one of the top American sites on children’s health aimed at the general public. It provides balanced information in clear English about heart murmurs for parents who are worried that their GP has picked up a heart murmur incidentally on examining their child. Our outreach cardiologist has written a few words on innocent heart murmurs too at http://www.kidscardiologist.com/conditions/innocent-murmur.html.
If your patient does turn out to have congenital heart disease, http://www.rch.org.au/cardiology/defects.cfm?doc_id=3011 is a fantastic site from Melbourne’s Royal Children Hospital with lovely clear diagrams and explanations of different morphological anomalies.