Category Archives: General paediatrics

Bronchiolitis treatment

General paediatrics, Respiratory

NICE guidance updated 2021.

Essentially supportive.  Oxygen, feeding, respiratory support if necessary.  Beware secondary bacterial infection, lobar collapse, pneumothorax, concomitant UTI.

Hypertonic saline

Not recommended by NICE. Cochrane 2010, patients treated with nebulized 3% saline had a significantly shorter mean length of hospital stay compared to those treated with nebulized 0.9% saline (MD -1.16 days, 95% CI -1.55 to -0.77, P < 0.00001).   No significant adverse events related to 3% saline inhalation were reported.  Not recommended for emergency departments as 2 doses didn’t seem to affect clinical scores.

But subsequent studies less impressive.  SABRE trial randomized 317 infants to 3% hypertonic saline nebulised every 6 h from admission compared with nothing (ie, standard care). No difference between the two arms of the study in time to discharge [Legg and Cunningham, Arch Dis Child 2015;100:1104-1105].

But AAP guidelines recommend nebulized hypertonic saline for infants hospitalized with bronchiolitis, with the expectation of reducing bronchiolitis scores and length of stay when it is expected to last more than 72 hours. Some think potentially an advantage for hypertonic saline in reducing admission rates from the emergency department [DOI: 10.5863/1551-6776-21.1.7]

High Flow

Franklin study of early high flow oxygen use – babies were randomized as soon as they needed oxygen.  Not generally what happens, of course.

  • Outcome was “escalation of care” ie signs requiring further intervention eg tachycardia, hypoxia. Intervention was high flow, of course.  Rate was 23%, but nearly a third of these did not meet the prespecified criteria (so doctor just decided they “needed” high flow anyway).
  • In sites without PICU, 28% of standard therapy group required escalation of care.  This is way in excess of the rate we would report in our unit.
  • No differences in length of stay or duration of O2 therapy.
  • Of 167 who “failed” on standard care, 61% responded to high flow.
  • No age effect.

So it seems to me that if you have facility to do high flow, you will find that at least a quarter of your oxygen dependent bronchiolitis babies “need” it.  I’m not sure this is a useful or meaningful study.  Babies may be more comfortable on high flow, and you may prevent the odd ICU admission, which is definitely worth considering. [N Engl J Med 2018 Mar 22;378(12):1121-1131.]

BIDS study

A study to see if safe to discharge babies with less than normal saturations.  RCT of 308 infants, no need to admit if sats >92% AND >50% feed requirements.

For those needing admission, start oxygen only if <90%, and only discharge once sats >90% continuously over 4hr period including sleep, and taking >75% feeds! Exclude babies with risk factors (<3/12, ex-prem, CLD etc– should have sats >92%)

Compared with standard pulse oximeter parameters (treat <94%), no difference in adverse events eg high dependency, readmission.  Excluded prems, recent oxygen therapy, CF or other chronic lung disease, immune deficit. [Edinburgh, Steve Cunningham, Lancet 2015; 386: 1041–48]

Study in emergency departments (n=213) found that babies discharged with artificially raised saturations (+3%) actually were less likely to be readmitted than babies with true oxygen saturations, suggesting that it’s a poor predictor (probably true for other respiratory conditions, too).

Combined bronchodilator and steroids

One study found better outcomes with neb adrenaline and systemic steroids but subgroup analysis and not adequately powered.

Immunotherapy

Allergy, General paediatrics, Immunology, , ,

First described in the literature in 1908! For egg. Noon and Freeman described grass immunotherapy in 1911. First double-blind trial was by William Frankland in 1954 for subcutaneous grass immunotherapy for seasonal asthma.

Should always be done with extreme caution, if at all, if asthma.  Available for wasp/bee stings, grass and tree pollen.  Lots of evidence for food allergies, especially in younger children eg peanut immunotherapy.  House dust mite sublingual now approved by NICE for resistant allergic rhinitis.

Some evidence that immunotherapy for rhinitis also prevents asthma, which fits with “one airway” hypothesis.

Immunotherapy should be initiated and monitored in a specialist centre experienced in immunotherapy.

Text message reminders doubles adherence – even non-personalised.

Immunomodulation with omalizumab appears to improve success rates of immunotherapy. Treatment with dupilumab (anti-IL4/13) reduces specific IgE levels in people with atopic dermatitis (clinical effect unknown just now).

Grass/pollen allergy

EAACI indications for rhinoconjunctivitis immunotherapy includes:

  • mild rhinitis for reasons of asthma control.
  • Moderate-severe symptoms.

Bad asthma and poor compliance are contraindications. Bad asthma was an exclusion criterium for most of the studies, so no evidence of safety.  Poor evidence for under 5yrs (for HDM).

Polysensitization common, US tend to to treat all, Europe tends to pick 1 or 2 most useful, at intervals. 17 fatalities to date with immunotherapy. 1 per million injections (risk with sublingual much less, even though used for higher risk patients). Large local reactions common but don’t predict further reactions. Risk of systemic reaction only increased if recurrent. Consider predosing with antihistamine, else reduce dose.

Sublingual immunotherapy (SLIT) in children aged 4 to 12  years with grass pollen-allergic rhinitis/rhinoconjunctivitis significantly  reduced symptoms and medication use, well tolerated, and no serious treatment-related  events were reported. [Journal of Allergy & Clinical Immunology.  130(4):886-93.e5, 2012 Oct.]

In metanalysis by Dhami S et al, overall standardized mean difference (SMD) of -0.53 (95% CI -0.63, -0.42) in symptoms scores, roughly equal numbers of SCIT and SLIT studies, roughly equivalent scores.  When looking just at children, benefit seems less (SMD -0.25 (95% CI -0.46, -0.05)).  Continuous treatment probably slightly better than pre/co-season treatment.  Manufacturers suggest “disease modifying effect” of treatment beyond first year, which has theoretical rationale.  Four studies of long term outcome, demonstrates continuing benefit if treatment continued beyond first year [Allergy 2017]. 

Grazax (grass) can be safely administered by general practitioners (£80 per month, licensed from 5yrs up): tablet needs to be kept under tongue for at least 1 minute, first dose should be monitored by doctor for 20 minutes.  Don’t eat or drink for 5 minutes.

Give antihistamines for local effects. Oral blistering occurs!  Isolated cases of eosinophilic oesophagitis but impossible to link of course.

Contains fish gelatine but no reported problems in those with fish allergy.  Severe asthma contraindication (in children, defined as <80% predicted FEV1 on treatment).

Aim to start 4 months before season starts, although still some benefit if started 2-3 months before.  If no benefit in first season, no point continuing (according to Grazax own SPC).

Symptom relief begins only in the second season of therapy?  BNFc says continue only for 3 yrs.  Not approved by SMC for Scotland, because no great evidence for benefit after first year – would need individual patient treatment request.  Rosie says children wouldn’t tolerate daily doses for months and years.

Pollinex subcut, given into middle third of upperarm.  2 versions: Trees, and 13 grasses incl rye. 3 injections at 7-14 day intervals each year (£450 each), before season starts.  Maintenance kit of 4 vials also available, presumably if additional benefit thought possible.  Manufacturer recommends using for 3 successive years. Asthma and beta blocker treatment are relative contraindications.  Age 6+, not in SMC at all. [Metanalysis, Chest 2008; 133:589, Journal of Allergy & Clinical Immunology. 115(4):676-84, 2005 Apr.]

Some evidence for short course – in multicentre study of adults (not UK) with grass mix SPT positive rhinitis, 8 subcutaneous injections of placebo or Lolium perenne (LPP) were administered in 4 visits (2 jabs each visit, 30 mins apart, different arms) over 3 weeks between January and April. Combined symptom and medication score (CSMS) measured over the peak pollen season was reduced 15.5% (P = .041) during the peak period and −17.9% (P = .029) over the entire pollen season. Also lower rhinoconjunctivitis QOL global score (P = .005) compared with placebo group.

Asthma

Dhami metanalysis immunotherapy for asthma in kids – symptoms improved, less medication, esp HDM, grass, cat/dog. Not just severe asthma! But no prolonged benefit.

1 study of adults with poor control, HDM. Reduces time to first exacerbation.

Mite allergy prevention study n=111 HDM treatment effective. 2002 PAT study reduced asthma at 10yr follow up after grass/pollen treatment.

[Gillian Vance, Newcastle]

Eczema

House dust mite immunotherapy with SLIT (3 doses per week) shown to have some benefit in RCT from Brazil (66 children and adults) using SCORAD eczema severity questionnaire. Placebo group showed 35% improvement over 18 months, SLIT group showed 55%. No difference in Dermatology Life Quality index, pruritus score or any of the various other measures used!

Swimming and asthma

General paediatrics, Respiratory, ,

For competitive swimmers, note FINA doping control rules (based on the World Anti-Doping Agency regulations). Most asthma medications are on the Prohibited List including inhaled steroids and inhaled Beta2 agonists.

Therefore, elite athletes with asthma must apply for special permission to use these medications, known as the “Therapeutic Use Exemption” (TUE) program.  If competing at FINA events, then you apply directly to the FINA Doping Control Review Board to have their applications considered. Lower level athletes should apply to their national anti-doping body.

Epilepsy

General paediatrics, Neurology

Historically, people with epilepsy were “considered to have unique powers, even hailed as geniuses, regarded as having a sacred disease and leading sacred lives”.&nbsp;&nbsp;But then demonisation, persecution, social rejection. “Epileptic personality” described by psychiatry in 20th century. [Sacred lives, Ian Bone]

Self control is central to our self image and the manner in which we and society believe we should behave. Epilepsy jeopardises this. Patients often conceal. Guilt, loss of confidence and low mood common after seizures.

Definition: At least 2 unprovoked (or reflex) seizures, occurring more than 24hrs apart; else one unprovoked (or reflex) seizure and probability of further seizures similar to that seen in those who have had 2 unprovoked seizures (ie at least 60%); or recognized epilepsy syndrome.  Also part of the definition is that epilepsy is considered “resolved” if age dependent syndrome and past applicable age, or else those who have been seizure free for 10 years and off medication for 5 years. (ILAE 2014)

Note that “seizure” does not have any real medical meaning!  Transient signs/symptoms due to excessive or synchronous neuronal activity in brain (ILAE 2017) – but implies you can tell whether caused by abnormal brain activity, which can be hard!

First assessment:

  • NICE standard is that patient is seen within 2 weeks by a specialist! NICE guideline just says seen by doctor with training and expertise in epilepsy.
  • First assessment should include description of event, age/timing of event, frequency of events.
  • Physical examination of neurology, cardiac, mental state and development.
  • Presence/absence of developmental, learning or schooling problems.

Investigations

EEG

Despite increasing sophistication, interpreting EEG remains an inexact science! Irregular background activity overlaps with detectable abnormality. Plus, only really picks up activity at surface of brain, and can miss simple partial seizures (but not tonic-clonic generalised). Review in 2000 did not find much evidence base. Requires dialogue between referrer and neurophysiologist. Diagnosis remains principally clinical – eg more than 1 tonic-clonic seizure, or multiple absences! Incidence of epileptiform activity in asymptomatic individuals appears to be about 1%, of which a few percent will develop epilepsy over the subsequent years. Abnormal activity is certainly more likely if structural abnormality, but still many will be and remain asymptomatic.<

50% of children with epilepsy have normal EEGs – so not a particularly useful test! Not only that, but in studies, at least 20% of “epileptics” were ultimately given a non-epileptic diagnosis! So request EEG with caution; should be used for confirming clinical opinion, or to guide treatment, not where symptoms are vague. Hence a firm diagnosis of epilepsy and then decisions about treatment may take some time; difficult for families to understand, but it is quite safe to be cautious esp considering the implications of a mistaken diagnosis.

If EEG is negative, then proceed to a sleep EEG (where child is woken by parents at 3am, kept awake then brought to department and allowed to fall asleep during monitoring). This has 80% sensitivity. Failing that, Medilog or Video with continous monitoring. This is good for distinguishing non-epileptic tonic-clonic seizures, but does not rule out co-existing epilepsy.

Good for:

  • status in PICU patients, or non-convulsive status
  • where children too young to describe their symptoms
  • absences – typical absence epilepsy have 3Hz spike and wave with hyperventilation. Highly specific, although absences can be seen in complex partial seizures, which would also be obvious on EEG.
  • indicating underlying brain disease (abnormal background)
  • specific syndromes
  • cognitive impairments that may be seizure related

Beware – an EEG finding of partial epilepsy may not have a surgical lesion! Must be used in conjunction with MRI. A generalized epilepsy may have multiple foci, so if you are unlucky to capture just one you will be misled. Similarly, if secondary generalization occurs rapidly, its partial nature may be missed.

Not usually useful to do after multiple seizures, unless type or frequency changes significantly, in which case a new syndrome/prognosis may have developed.</p>

<p><em>”The brain is subject to maturation; there are multiple protecting and triggering factors, often unpredictable. Seizures may be rare and easy to treat for months and years, but may become more frequent and difficult to control later on. But in many children a precise syndromic diagnosis can be made, and a good final prognosis can be expected in most cases.”</em></p>

Other investigations

All children with recurrent seizures should have an ECG with calculated QTc. &nbsp;Children under 2 with epilepsy or with recurrent focal seizures (other than CECTS) should have an elective MRI brain scan. &nbsp;In most other cases the course is predictable. A normal MRI does not rule out a small dysplastic lesion, equally the finding of a lesion does not mean that it is the cause of the epilepsy. However, at least you can exclude a tumour or malformation.</p>

Focal/partial often have acquired or congenital lesions, often specific precipitating factors eg sleep, startle!</p>

Differential

Management

<p>Consider:</p>

  • Drug treatment (see below) – and risk with prenatal exposure
  • Education – written, peer support
  • Specialist epilepsy nurse review
  • Emergency medication, if appropriate

First aid advice

  • <li><a href=”http://scottishpaeds.org.uk/2017/01/31/living-with-epilepsy/”>Safety advice</a></li>

<li>When/how to access health services</li>

</ul>

</div>

Treatment

Should not be started after first tonic-clonic generalized seizure. Try not to start before EEG done as may mask features.

Refer to tertiary if –

  • child fails to respond to two AEDs appropriate to the epilepsy in adequate dosages over a period of 6 months (SIGN), or 3 over 12 months (NICE)
  • children less than 2 years with epilepsy as defined

SPEN network has pathways for first seizure, new diagnosis, continuing seizures.

See also Living with Epilepsy

VZV encephalitis

General paediatrics, Infectious disease, Neurology

VZV encephalitis presentation not different from usual, viz  fever, headache, altered consciousness, etc. But can be subacute onset.

The more common neuro manifestation of VZV in young children is post-infectious cerebellitis  – usually mild and self limiting, child not unwell but risk of secondary
hydrocephalus in more severe cases.

There is also a well recognized association in childhood  between VZV infection and stroke, usually presents after the rash has cleared – typically 3 months [London study, Miravet & Danchaivijitr 2007).  Post-VZV thromboembolism also seen rarely eg lower limb DVT.

Encephalitis can present early, even before rash (which may
not be very obvious either).  PCR for VZV DNA in the CSF is positive in around a third of patients; VZV specific IgG seen in 90% in CSF. Compare  levels to serum as a way
of confirming CNS involvement.

Usually (not for cerebellitis), steroids given eg 60-80 mg of prednisolone daily for 3 to 5 days) is given (Gilden & Kleinschmidt-DeMasters  2000) esp where MRI evidence of vasculitis.
[encephalitis article, source?]

Chickenpox (Varicella Zoster virus)

General paediatrics, Infectious disease

A systemic viral illness characterised by widespread vesicles. Like other herpesviruses, it retains the ability to lie dormant in ganglions after initial infection, possibly reactivating in the future as Herpes Zoster (= shingles).

Clinical

Congenital varicella

Congenital, Infectious disease, Neonatology,

If a pregnant woman is infected during the first trimester (risk extends to 20th week), a fetus has a 1% chance of developing widespread scarring, hypoplastic limbs, cataracts and brain lesions (congenital varicella). Affected infants have a poor neurodevelopmental outlook.

Risk of neonatal VZV (severe, disseminated disease in newborn) if chickenpox is contracted by the mother 4 days before birth, to 2 days after  (risk 20%).  Before that, good chance that maternal immune response will protect baby, after that the dose of infection transmitted to the baby via the umbilical cord is likely to be small (although will still be exposed to droplets).

See Maternal for advice on varicella exposure in pregnancy and in neonates.

 

 

Varicella Zoster Virus vaccine

General paediatrics, Immunology, Infectious disease,

Following varicella vaccine introduction in Australia, coverage of greater than 80% at 2 years of age was achieved, with varicella hospitalizations reduced by almost 70%. 40% of hospitalized children were immunocompromised.  Of hospitalized children age-eligible for varicella   vaccine, 80% were unimmunized, including all cases (3) requiring intensive care.  No deaths.

[Pediatric Infectious Disease Journal. , 17 December 2012]

Eczema

Dermatology, General paediatrics, Immunology,

NICE (CG57, 2007) diagnostic criteria:  itchy skin plus 3+ :

  • visible flexural dermatitis (or visible dermatitis on the cheeks and/or extensor areas in children <18/12) – can look like dirt where darkened esp neck
  • personal history of flexural dermatitis (or dermatitis on the cheeks and/or extensor areas in children  <18/12)
  • personal history of dry skin (xerosis) in the last 12 months
  • personal history of asthma or allergic rhinitis (or history of atopic disease in a first-degree relative of children aged <4yr)
  • onset of signs and symptoms under the age of 2 years (do not use this criterion in children under 4 years).

On examination, may also see lichenification (increased skin markings due to chronic rubbing), excoriations caused by scratching, and hyperlinear palms (look at thenar eminence).

Risk factors

Eczema (or atopic dermatitis, same thing) associated with parental allergies (OR 1.94), parent-reported infection after birth (OR 1.45), parent-reported jaundice (OR 1.27). Being a migrant (OR 0.63) and keeping a dog (OR 0.78) are protective. Prenatal probiotics did not reduce risk (Lactobacillus rhamnosus GG, LGG).  Respiratory viral infections in pregnancy associated with wheezing illness in infants (mothers with asthma) and eczema.  [Australia, Ped All Imm 2014:25:151].  Febrile and gynae infections in pregnancy associated with eczema in child (Italy, Ped All Imm 2014:25:159].  Children of Japanese mothers with higher anxiety scores during pregnancy were more likely to be affected by eczema!

Immunology

Many roads lead to Rome!  Can be seen as combination of impaired innate and distorted adaptive immunity, interacting in framework of cutaneous immune system with suboptimal barrier function.  Epidermal barrier can be genetically disturbed, but gene expression (eg of filaggrin) is also influenced by microenvironment eg Th2 cells.  Some genetic structural abnormalities can be seen to induce nonspecific inflammatory reaction (eg SPINK5 defects).  And almost certainly, epigenetic regulation will affect gene expression in both keratinocytes and immune cells.

Facilitated antigen presentation mediated by IgE bound to high affinity IgE receptor FcEpsilonRI, on dendritic cells, is an important part of it.  Vitamin D plays a key roll, stimulates Toll like receptors, increasing pro-inflammatory cytokines but studies equivocal whether low (or indeed high) levels increase risk.

Eczema can exist without IgE against aeroallergens and food allergens, so is primarily a non IgE mediated disease, although both IgE and non-IgE hypersensitivity can co-exist, particularly in babies.

Still not understood how eczema and Staph aureus interact.  Colonization appears to amplify local inflammatory reaction, but also increases IgE against a large spectrum of other things (possibly including self proteins).  It is as if there is something specifically different about how immune cells handle Staph.  Toll like receptors esp TLR-2 recognize Staph aureus cell wall. Overgrowth of staph during flares is associated (preceded?) by loss of diversity of skin microbiome.

Unmasking of the HSV entry receptor Nectin-1 in adherence junctions, among other things, contribute to eczema herpeticum.  See Flares, below.

Atopy patch test (using intact protein allergens) can prove type IV reactions. Correlates with oral food challenge. Hyper IgE syndromes are important differential for AD, STAT3 mutations, skin (and other) abscesses, recurrent pneumonia (and pneumatocoele), connective tissue and skeletal abnormalities, mucocutaneous candidiasis. DOCK8 type Hyper IgE syndrome show sensitization predominantly against food allergens, which is a clue (STAT3 and AD show predominantly sensitization to aeroallergens).

Normal looking  skin is not immunobiologically normal!  Invisible inflammation, hence still needs treatment. Pimecrolimus and betamethasone both normalize expression of filaggrin, the later is perhaps a better anti-inflammatory but steroids reduce expression of enzymes for lipid and protein synthesis, so may impair skin barrier restoration.

Triggers

Stress, humidity, extremes of temperature can cause flares of atopic eczema. But what causes a flare in some can apparently help in others! Any factors causing flares should be avoided where possible.

UVB causes sunburn.  UVA (longer wavelength) more relevant to photosensitivity reactions.  Both cause long term skin damage.  EU commission recommends UVA protection making up at least 1/3 of total suncream SPF.  A few available on prescription for photodermatosis (incl vitiligo) but not for eczema per se.  Lipscreen (Uvistat) available for chronic/rec HSV labialis.  Sunsense is lotion.

Avoid wool in contact with skin.  No evidence that bio detergent or fabric softener a major problem.  Avoiding excessive product and not overloading machine (so disperses fully) prob more important.

Management

See eczema management.

Parental support

National eczema society, British Association of Dermatologists, Eczema Outreach Scotland.