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Seborrhoeic dermatitis

Dermatology, General paediatrics

Scaly skin condition in babies, particularly affecting scalp. Red/orange scales, oily, flaky.  Not terribly itchy.

Affects sebaceous (greasy) zones – face, scalp, centre of chest, skin folds (neck, groin).  Often nappy rash. V common (includes dandruff!).  Inside, outside and around ears can be affected.  Eyelids can be affected (blepharitis).

Babies get transient type – cradle cap (scalp) and nappy area, clears after a few months.  In adults, affects face (esp around nose and ears) and can affect chest and upper back. Can lead to hair loss but more usually cosmetic concerns.

Thought to be caused by overgrowth of Malassezia yeast.  Can be severe in HIV.

Differential is psoriasis.  Scales in psoriasis are thicker and whiter – unusual for face to be affected.

Treatment

For cradle cap in babies, usually enough just to use daily mild shampoo and gentle brushing.  Shampoo may make it worse if the diagnosis is actually atopic dermatitis!  Failing that, coconut oil (NOT olive oil, which has potential to damage skin, apparently) or other moisturizer left in overnight or applied just before bath.

For scalp, Zinc pyrithione, selenium or ketoconazole shampoo (but only licensed for older children).  Leave 5-10 mins before rinsing.  Descaling treatment available – coconut oil and salicylic acid, needs hours.

For body, moisturizer, topical steroids+antifungal eg Canesten HC, Trimovate.

For blepharitis, use baby shampoo to lift flakes.

In adults, anti -androgens.

LEAP-On study

Allergy, General paediatrics, Immunology

Follow-up from LEAP study, after both groups (eating and avoiding) told to avoid peanuts for 12 months. The rate of adherence to avoidance in the follow-up study was high (90.4% in the peanut-avoidance group and 69.3% in the peanut-consumption group). Peanut allergy at 72 months was significantly more prevalent among participants in the peanut-avoidance group than among those in the peanut-consumption group (18.6% [52 of 280 participants] vs. 4.8% [13 of 270].

[George du Toit, Gideon Lack in London, DOI: 10.1056/NEJMoa1514209]

Enterovirus

Common, General paediatrics, Infectious disease

Mild: fever +/- rash, hand/foot/mouth, herpangina, pleurodynia, pharyngitis, conjunctivitis, croup.

Serious: meningitis, encephalitis, acute paralysis, neonatal sepsis, myo/pericarditis, hepatitis, chronic infection (immunocompromised).

Meningo-encephalitis in neonates usually associated with other organ involvement. In adolescents headache can be severe and symptoms last several weeks! Early CSF can show around 1000 neutrophils! Prognosis good, although some subtypes with encephalitis highly aggressive eg EV-71 outbreak in Taiwan in assoc with hand/foot/mouth (78 deaths).

Acute flaccid paralysis can occur cf polio. Particularly Enterovirus EV-D68 (also associated with respiratory disease), some clusters.  Increased incidence across Europe including Wales since April 2016.

Neonatal disease can be severe, mimicking bacterial sepsis or HSV. Maternal history is often elusive.

Virus is shed in throat and stool (rectal swab quicker than stool!), can also be detected in CSF, blood and urine.

Role of IVIG is unproven but antibody plays an important role in immune response to EV. Pleconaril in enteroviral meningitis RCT, 38% to 50% improvement in symptoms in the drug-treated group with improvement noted as early as 24 hours after initiation of therapy – no longer available.

[Current Opinion in Pediatrics. 13(1):65-9, 2001]

Human Parechovirus has been described in Japan, Canada and now the Netherlands, causing neonatal sepsis or encephalitis in about 10% of cases where culture suggests enterovirus but PCR is negative. [Clin Infect Dis. 2006 Jan 15;42(2):204-10]

Multiple sclerosis

Genetics, Neurology

Only 3-5% of cases of MS have symptoms before the age of 16. Most have a relapsing-remitting course, particularly in the beginning, typically with one to two relapses per year. The frequency of attacks does seem to predict disease severity and earlier evolution to secondary progressive phase.

Although it takes longer in kids to develop persistent disability, they still develop it at a relatively young age, for example the third or fourth decade of life. That is of course going to have significant effects on life course, including work and family life.

Even at onset, cognitive function is often reduced, which will also affect education and socialisation. So clearly there is interest in disease modifying treatments.

Presentation in younger children often follows an infectious illness. Cognitive impairment is more common relative to older kids.

Investigations

MRI of brain and spine, looking for demyelinating lesions.

Monoclonal bands in CSF.

Treatment

Steroids 10-30mg/kg for 3-5 days effective. No good evidence for IVIG. Where acute life threatening symptoms, plasmapheresis may be effective where steroids fail.

In adults good evidence for interferon Beta in relapsing-remitting disease, IM or SC depending on product, reduces relapse rate and probably slows progression of disability. Glatiramer is a synthetic product with similar effects.

Second line treatments in adults include Natalizumab, mitoxantrone and cyclophosphamide.

Differential diagnosis

  • lysosomal storage disorders,
  • various mitochondrial diseases,
  • other neurometabolic disorders,
  • Krabbe, Metachromatic leukodystrophy, X-linked adrenoleukodystrophy, Fabry, Niemann-Pick C, Chidak-Higashi.  [Clue is in the name, leukodystrophy]

Since these are genetic conditions, essential for management and genetic counselling.

J Weisfeld-Adams http://brain.oxfordjournals.org/content/138/3/517

Mitochondrial inheritance

Genetics

A mother with a mitochondrial DNA gene mutation will pass this abnormal gene to all of her children. The children will all be affected, but with different degrees of severity.

As it is not possible to predict how the children will be affected, this is immensely difficult for planning a family.

Leigh disease

General paediatrics, Genetics, Neurology

Infantile subacute necrotizing encephalopathy.

Clinically heterogenous, lots of different genes.  Can be X-linked, mitochondrial or recessive!!!  Main genetic problem is mitochondrial complex defect, but same disease can be caused by pyruvate dehydrogenase defect (actually a complex of enzymes).

Baby’s initial development may appear normal, although there may be failure to thrive.  Lactate can be raised in serum.  Progressive, often rapid, neurological deterioration including hypotonia, dystonia, seizures.

Lesions (necrotic, gliosis, spongiosis) seen in basal ganglia, brainstem, cerebellum, spinal chord.  CSF lactate and pyruvate may be raised, even if serum normal.

See mitochondrial inheritance.

 

Hospitality

Ethics, Generic

It is acceptable for staff to receive small tokens of gratitude from a relative or carer in appreciation of care and treatment received. These are typically cards, chocolates or biscuits. Where staff are offered gifts of greater value these must be politely refused. [bottles of wine?  Whisky?]  If this is difficult they must refer the matter to their line manager.

Hospitality

It is acceptable for staff to receive small promotional items, e.g. pens, calendars, diaries. However,

  • staff must not accept any offer of a gift or hospitality from any individual or organisation which stands to gain or benefit from a decision NHS Lanarkshire maybe involved in determining, or who is seeking to do business with NHS Lanarkshire.
  • staff must not accept any offer, by way of gift or hospitality, which could give rise to a reasonable suspicion of influence on their part to show favour, or disadvantage, to any individual, organisation or company.
  • staff should consider whether there may be a reasonable perception that any gift received by their spouse or partner or by any company in which they have an interest, or by a partnership of which they are a partner, can or would influence their judgement.

Note – the term ‘gift’ includes benefits such as provision of services at a cost below that generally charged to members of the public.

Modest hospitality may be acceptable provided it is normal and reasonable in the circumstances e.g. lunches in the course of a working visit. Any hospitality accepted should be similar in scale to that which the NHS as an employer would be likely to offer.

All other offers of hospitality should be declined.

Staff should register with their line manager all such modest hospitality which they wish to accept, using the hospitality register declaration form (Appendix 3). In cases of doubt, staff should seek advice from their line manager.

If the nature of the event dictates a level of hospitality which exceeds this, then the individual should ensure that their line manager is fully aware of the circumstances and approves their attendance. An example of such an event might be an awards ceremony involving a formal dinner. If the line manager grants approval to attend, the individual should declare their attendance in the register of hospitality held by their line manager. The approving manager must ensure that this will not result in any future conflict of interest.

If the individual is invited to an event in a private capacity (e.g. as result of their qualification or membership of a professional body), they are at liberty to accept or decline the invitation without referring to their line manager. The following matters should however be considered before an invitation to an individual acting in a private capacity is accepted.
a) The individual should not do or say anything at the event that could be construed as representing the views and/or policies of NHS Lanarkshire.
b) If the body issuing the invitation has (or is likely to have, or is seeking to have) commercial or other financial dealings with NHS Lanarkshire, then it could be difficult for an individual to demonstrate that their attendance was in a private and not an official capacity. Attendance could create a perception that the individual’s independence had been compromised, especially where the scale of hospitality is lavish. Individuals should therefore exercise caution before accepting invitations from such bodies and
must inform their line manager.
c) Where suppliers of clinical products provide hospitality it should only be accepted in association with scientific meetings, clinical educational meetings or equivalent, which must be modest, normal and reasonable in the circumstances and in line with what the NHS would normally provide. Any such hospitality should be held in appropriate venues conducive to the main purpose of the event. 

Sponsorship [should be] clearly disclosed in any papers relating to the meeting; products discussed should be described in relation to the Scottish Medicines Consortium, Formulary and the active promotion of clinical products is restricted to those in the Board’s Formulary and equivalent clinical product catalogues.

Any educational meetings hosted by suppliers must be approved by the line manager.

Before accepting an offer of hospitality the individual concerned should fill in a Registering Hospitality Declaration Form (attached as appendix 3) and have it approved by their line manager. A copy of the request form will be held as part of a Hospitality Register which will be available for scrutiny by the
NHS Board, Corporate Management Team, members of the public or press should they request such information.

Legume/pulse allergy

Allergy, General paediatrics

Legumes, pulses, beans…  Some terminology first: legumes are plants in family Fabaceae (or Leguminosae).  Pulses are (strictly) those cultivated for DRY seed, as opposed to green beans, broad beans etc that are eaten fresh.  Lentil simply describes shape (“like lens”).  So other examples of legumes are peanut, lupin, tamarind, carob, alfalfa!

Very common cause of food allergy, even excluding peanut! And varies across different cultures, depending on typical pulses used.  Fifth most common cause of food allergy in Spain.  In India, often a trigger of asthma/rhinitis when being boiled.  Cross sensitivity is seen, but not automatic, and hard to predict.

Soya allergy is sometimes seen in highly atopic babies, but otherwise actually pretty rare (except in Japan) – lucky, cause gets into lots of different things eg many breads.  Soya lecithin is a common additive, used to make texture more smooth, but usually only in very small amounts. Fermentation eg soy sauce appears to significantly reduce allergenicity, soya flour also seems less allergenic than soya milk, even though most of the allergens are cupins eg 2s albumin and would therefore be considered heat stable.

Lupin is used in some continental baked goods, for example packaged waffles.  A good proportion of peanut allergic children will be allergic to it too, but as lupin is not found very commonly most will never know or have a problem with it. A few restaurants (in UK and abroad) have lupin in their allergy menus.

A couple of lentil allergens have been identified including a vicilin and a lipid transfer protein.  In my experience pappadoms can often be tolerated – there is certainly evidence that autoclaving for 30 mins can affect binding, but these are only deep fried for a few seconds.

Both the known pea allergens are vicilins, hence cross reactivity with lentil.  Chickpea allergens however are not (one a prolamin, the other a cupin) – still, cross reactivity fairly common.

French beans have an LTP so potentially severe, and potentially fruit allergies too.  Green (mung bean) and red gram are cupins, black gram appears to be something else.

[Clin Rev Allergy Immunol. 45(1):30-46, 2013 Aug]

Competency

Ethics, Generic

Gillick ruling (1985) was primarily about sharing of information with parents (use of oral contraceptive), not about going against their decision!

You should involve children and young people as much as possible in decisions about their care, even when they are not able to make decisions on their own (GMC).

Competence is not binary, it varies according to context and over time.  You need to assess ability to:

  • understand, retain, use and weigh Information about consequences of treatment/non-treatment
  • Communicate

And you need to assess context:

  • Complexity, level or risk, seriousness of consequences
  • Physical/emotional development
  • Changes in health/treatment

Even then, you should encourage involvement of parents.  In difficult situations, consider involving multidiscip team, independent advocate, child protection teams. ”You should not make unjustified assumptions about a child or young person’s best interests based on irrelevant or discriminatory factors, such as their behaviour, appearance or disability”.

At 16yrs, a young person can be presumed to have the capacity to consent.  In Scotland, parents cannot authorize treatment a competent young person (even under 16yr) has refused.  In E&W/NI, High Court can override up to 18yrs – “children and young people have a right to consent, but not to refuse treatment if this would put their health in serious jeopardy” (BMA consent toolkit).  Court rulings have gone both ways.