All posts by admin

Haemophagocytic syndromes

General paediatrics, Haem/Oncology, Immunology, Rheumatology

A group of disorders, including haemophagocytic lymphohistiocytosis, Macrophage activation syndrome, and PIMS-TS. Suspect when these unexplained or unusually severe, particularly in combination:

Anti-emetics

Common, General paediatrics,

Oral Ondansetron use for gastroenteritis has become v popular in many emergency departments.  In 1 study of 18 EDs, where it was a standard in nearly half all cases, there was no overall improvement in rates of either intravenous rehydration (remained around 18%) or hospital admission. There was a small decrease in re-attendance rates.

There was also a wide variation between institutions: perhaps the problem is not using Ondansetron it correctly eg not giving it soon enough, or rushing into IV fluids before allowing the drug time to work

Same group looked at Ondansetron in diabetic children with vomiting, again, usage increased from 0 to 67%. Admission rates dropped from 62% to 49% between these eras, as did use of IV fluids, but Ondansetron had no independent benefit.

From Archimedes Blog.  (Freedman S et al. JAMA Pediatr 2014;168:321–29, see also Editorial)( (Leung J et al. J Pediatr 2014. doi.org/10.1016/j.jpeds.2014.10.020). )

Tremor

General paediatrics, Neurology

Essential tremor develops insidiously and progresses slowly.  May start in a single limb, but it becomes bilateral over time.  Flexion-extension movement of the wrist, frequency of 4 to 12 Hz. May involve head (yes-yes or no-no). Worsens with stress, fatigue, and may increase with some voluntary activities eg holding a fork or cup. Rest, beta blockers, and alcohol help.  Often a family history.

Compare Cerebellar tremor – low-frequency (less than 5 Hz), intention tremor.  May include postural element (ie at rest).  Other signs include abnormalities of gait and speech, nystagmus, dysdiadochonesis (inability to perform rapid repeated hand movements).  Titubation is the word given to rhythmic movements of head/neck seen in cerebellar disease.

So ask patient to extend arms.  Do Finger-to-nose, finger-to-finger, and heel-to-shin testing (Cerebellar).  Observe drinking from glass, writing name, drawing spiral (or draw within lines of pre-printed spiral).  Check for tone (rigidity), esp when busy using other limb, eg draw a circle in the air) – basal ganglia, eg Parkinsons. Check gait (shuffling?  Ataxic?), eye movements.

Look for signs of space occupying lesion, thyroid or liver disease.  Any chance of intermittent hypoglycaemia?  Panic disorder?  Withdrawal?

Domperidone

Cardiology, Gastro, General paediatrics

Children with congenital heart disease – Consider stopping domperidone therapy or discuss with parents/carers and ensure that cardiac monitoring is regularly performed. Consider offering an alternative treatment where appropriate.

Other children with established reflux or nausea and vomiting – Take no immediate action in patients already established on domperidone.   Consider reducing the dose (where appropriate) to 250microgram/kg three times a day at the next convenient review. Consider routine cardiac monitoring where there are concerns (e.g. cardiovascular instability,
concomitant CYP3A4 inhibitors prescribed).

In new patients, always give a proper trial of feed thickeners before considering pharmacological intervention – at least two weeks. In more serious cases, and after the introduction of thickeners then consider the benefits and risks of medical anti-reflux/anti-acid secretion treatment.

If domperidone is to be used, give an initial maximum of
250micrograms/kg three times a day. Where reflux or nausea is refractory to
this then give increased doses to a maximum of 400micrograms/kg (max
20mg) three times a day and recommend regular cardiac monitoring.

Patient Information Leaflet entitled “Domperidone for gastrooesophageal
reflux” available from www.medicinesforchildren.org.uk

SUDI – Risks

General paediatrics

Long list of known risk factors, even though mechanism not clearly understood!

Age is probably the main risk factor – mostly 5-10 weeks of age. Very few in later infancy (although sudden death is described in all ages cf SUDEP).

From Scottish study –

So preterm, low birth weight boys with socially deprived unmarried mothers who smoke are at highest risk. But many of these factors compound and confound – 78% have at least 2 risk factors, only 0.8% have no risk factors. If you exclude “non-modifiable risk factors” (social deprivation, etc), only 5.3% have no risk factors.

Prone sleeping is no longer a major factor since it has been discouraged for years. Might be protective for preterms, where found to promote cortical arousal (CA) responses (protective in term infants). Horne 2013

36% of excess infant mortality in US South due to SUDI (90% of excess mortality in Kentucky!  59% due to non-hispanic black population).

Main risk factor now is co-sleeping, esp on sofa, although this is commonly associated with alcohol/drug use. Blair & Sidebotham BMJ 2009

Note used mattress is risk factor in Scotland – never replicated elsewhere.

Dummies are protective, even though they fall out – part of some national safe sleep recommendations but not in UK (perhaps because mechanism unclear?).

Parental mental health associated – if both have a mental health disorder, OR for SIDS =6, more if substance abuse disorder – but smoking/social deprivation explains 50% of this risk.

4% of unselected cases had long QT mutations [NZ] – increased to 16% when cases guided by cardiac genetics.  But poor uptake of screening!

A previous maltreatment report emerged as a significant predictor of SIDS and other SUID. After adjusting for baseline risk factors, the rate of SIDS was more than 3 times as great among infants reported for possible maltreatment (hazard ratio: 3.22; 95% CI: 2.66, 3.89).  [US, PMID 24139442]

SUDI – prevention

General paediatrics, ,

NICE CG194 [postnatal care] covers.

Smoking cessation advice.

Discuss with parents safer practices for bed sharing:

  • making sure the baby sleeps on a firm, flat mattress, lying face up (rather than face down or on their side)
  • not sleeping on a sofa or chair with the baby
  • not having pillows or duvets [bumpers etc] near the baby

“Strongly advise” parents not to share a bed with their baby if:

  • baby was low birth weight, 
  • or if either parent:
    • has had 2 or more units of alcohol [not zero tolerance! Interesting…]
    • smokes
    • has taken medicine that causes drowsiness
    • has used recreational drugs.

And that’s it!!!

Note that the word “risk” is not used, just association!  Boys as being at higher risk not mentioned!

PreBotzinger complex (preBotC) is a multi-functional neuronal network that is critically involved in the response to hypoxic and hypercapnic challenges.

Note increased brain oxygen requirement during “active sleep” cf quiet sleep.  Only apparent between 2 weeks and 5 months.  [Horne 2014].

Dutch recommendations include: (a) pre-term neonates born after 32 weeks should be placed in a supine position; (b) twins should not sleep in the same bed (‘co-bedding’); (c) use of a pacifier is recommended once breastfeeding is well underway; and (d) use of stabilization pillows is not recommended [PMID 23425715]

Psychosocial interventions

General paediatrics, Psychology

Flashpoints are transition eg from nursery to primary, to secondary, to adult services.

At diagnosis, constantly try to normalise.

Other triggers are new or difficult situations: staff changes esp specialist nurses. Effect on parent’s work, parent’s role in family, child’s fears.

Past experience of medical condition, procedure, hospital/doctors will colour.

Parenting in chronic illness – limit setting vs laxity (love!) in face of illness.

Behaviour as communication of fear, displeasure!

Signs and symptoms – changes in appearance, mood, behaviour, thoughts.

Support at diagnosis: names, phone numbers! Normalise experience and feelings. Signpost peer support, online or other. Written. Practical eg financial, family routines. Joint working for consistent info. Deciding what chats are appropriate with child present. Reiteration.

“Other people in your situation have tried x, y and z. Do any of those sound good?”

Pre-5: encourage play and exploration, avoid interfering with parental proximity.

5-7 May develop magical thinking (I think, and it comes true). Guilt, punishment, contagion? Accept what other children say as true! Imitate parental behaviour. Death as reversible.

Drawing! Check understanding of bodily functions.

Sue Robinson, hospital passport (Janie donnan). For primary school age, app for teens to follow.

Concrete reminder of achievements and rewards.

Alphabet. Backwards!

Hand on tummy, feel rise and fall.

Guidance for parents!

Sucrose. Video for juniors, showing expected techniques.

Functional analysis (ABC) – immediate antecedent (context as much as events), consequences (esp people’s actions, any difference in attention (anything given or taken away)?  What would usually happen otherwise?) use diary again. Bedside table! 5-7 days max, can be repeated. Review within 2 weeks.

Pacing – beware boom/bust cycles. Rest before exhausted but maintains daily activity.

Activity record: enjoyment vs pain impact.

Smart goal. Low hanging fruit first! Goal diary – did you achieve it? Rate pain. How did you feel?

If/then plan – beware abandoning at first set back. If you can’t get to school one day, then what will you do? Phone to update? Try harder next day?

How confident? What benefits, what difficulties?

Visualisation – can child describe a scene easily? Else unlikely to work.  Personally relevant dream place. Safe and happy. Real or imagined. Describe it in as much detail as you can – all senses. As long as possible; but 5 mins is plenty.

Record positive achievements.

Positivity – but listen empathetically.

Negative beliefs.

 

[NES study day – Liz Hunter, Ashley Sikoura]

Febrile Convulsions

Common, General paediatrics, Neurology

Typical febrile convulsions are:

  • age 6 months to 6 years
  • Normal neurodevelopment
  • generalized, tonic-clonic

Most important differential is CNS infection eg encephalitis, meningitis.  These tend to present with posturing, impaired conscious level, or focal seizures. 15% of patients presenting with status epilepticus with fever have meningitis (observational study) – although low rate of LP so underestimate? I suspect there would have been other features to suggest meningitis beforehand.  Stiff neck? Fear of doing LP due to RICP from fit and/or meningitis, so do CT first if in ICU or abnormal neurology else as soon as no contraindication. If in doubt, treat empirically for meningitis (+/- herpes encephalitis, although risk unknown) with antibiotics and steroids. [Chin RFM, Arch Dis Child 2005;90:66-9.(Ed by Kneen)]

About 30-35% of febrile convulsions in the absence of CNS infection however have one or more complex features:

  • focal onset,
  • duration >10 minutes,
  • or multiple seizures during the illness episode

Febrile status epilepticus, a subgroup of complex febrile convulsions with seizures lasting more than 30 minutes, occur in about 5% of cases.  [BMJ 2015; 351 doi: http://dx.doi.org/10.1136/bmj.h4240 ]

Recurrence

One third of children with febrile convulsions will experience further seizures; age is the single, strongest, and most consistent risk factor. Most recurrences will occur during the first year and over 90% recur within two years (so unlikely to happen later). Other risk factors for recurrence are –

  • family history of febrile convulsions (but not epilepsy) in a first degree relative,
  • children whose initial seizure occurred with a relatively low fever,
  • multiple initial seizures occurring during the same febrile episode.

Surprisingly, status in an otherwise normal child does not appear to significantly increase the risk for further febrile seizures or the development of epilepsy.

Information for Families

From European Journal of Pediatrics 2021:

Prevention of peanut allergy

Allergy, General paediatrics, Immunology,

Use of peanut oil in eczema creams had OR 8 for peanut allergy but retrospective.

Filaggrin deficiency has OR 5 for food allergy, only 3 for eczema!

So could skin protection (particularly in babies with eczema) before early weaning prevent food allergy? Preliminary studies suggest 35- 50% response. Evidence that peanut consumption of household predicts peanut allergy in baby – presumably by skin sensitization.

Bamba peanut snack

Jewish children in the UK have a prevalence of peanut allergy that is 10-fold higher than that of Jewish children in Israel. This difference is not accounted for by differences in atopy, social class, genetic background, or peanut allergenicity. Israeli infants consume peanut in high quantities in the first year of life, Bamba (peanut snack, like a Wotsit) often used for weaning, so most infants have been exposed by age 12 months. [Du Toit  J Allergy Clin Immunol. 2008 Nov;122(5):984-9]

Gideon Lack at Evangelina hospital in London did LEAP study (Learning about Peanut Allergy), randomized infants with severe eczema and/or egg allergy to receive either no peanut until age 3yr, else an age-appropriate peanut snack (Bamba or smooth peanut butter, 6g) three times a week.   Among the 530 infants in the intention-to-treat population who initially had negative results on the skin-prick test, the prevalence of peanut allergy at 60 months of age was 13.7% in the avoidance group and 1.9% in the consumption group (86% reduction, P<0.001). 98 participants had baseline positive SPT results, only 12% had a positive challenge so most continued the protocol.  Another 10% with SPT>4mm were excluded from the start.

Adherence to the diet was excellent.  Dust samples were taken from some participants’ beds, peanut levels were significantly higher for kids in consumption group.  There was a higher rate of urticaria in the consumption group.

IgE greater than 10 in peanut avoiding group had 100% PPV for allergy.  Peanut-specific IgG4 antibody seems to be linked to tolerance – it went up more in the consumption group, and IgG4:IgE ratio was generally lower in allergic group (most had IgG4 under 1000). [NEJM 2015; DOI: 10.1056/NEJMoa1414850]  See also LEAP-On study, which is the follow up at 72 months, still significant difference.

Michael Perkins’ EAT study of early introduction of 6 common allergens in non-high risk babies showed if strict adherence to protocol eg 2g weekly of peanut, then every case of peanut allergy could be prevented.

Risk of peanut allergy in high risk babies estimated to be about 14%. Cost benefit analysis suggests better to go for early introduction WITHOUT initial testing, as high rate of false positives. Yet in the US at least, lots of early screening happening (median number of foods tested =10!), rarely followed by oral challenge. If you tested every high risk baby in the US with IgE, it would cost $900m…

 

 

 

Influenza – treatment

General paediatrics, Infectious disease, Respiratory

Oseltamivir and Zanamivir licensed for treatment and the former also for post-exposure prophylaxis.

Cochrane review 2014 found little evidence of benefit.  But this was based on community studies in healthy populations.

“Use of neuraminidase inhibitors in influenza” [October 2015, Academy of Medical Sciences] indicates that the use of antivirals can be beneficial in certain situations, but of limited use in others. Additionally, a recent review “Expert opinion on neuraminidase inhibitors for the prevention and treatment of influenza – review of recent systematic reviews and meta-analyses” August 2017, European Centre for Disease] supports use as treatment and prophylaxis.

UKHSA therefore recommends:

“Complicated” influenza defined as

  • requiring hospital admission,
  • signs of LRTI eg resp distress, chest signs
  • CNS signs
  • Worsening of underlying medical condition

Risk factors include:

  • infants under 6 months; 
  • neurological, hepatic, renal, pulmonary and chronic cardiac disease ; diabetes mellitus;
  • severe immunosuppression (includes prednisolone r≥2mg/kg/day for ≥1 week);
  • morbid obesity (Z score BMI 3.33 or higher).

Oseltamivir (Tamiflu) PO or NG is first line, unless severe immunosuppression and A(H1N1) variant prone to resistance dominant – then zanamavir.

  • Start treatment within 48hrs, do not wait on lab confirmation of diagnosis.  May still be benefit in life threatening illness when started up to 5 days after onset. Rule is within 36hrs if child and zanamavir.
  • should NOT be used in otherwise healthy patients but can be considered if felt to be “at serious risk of developing complications”.
  • Test for resistance if no response after 5 days of treatment.

Presumes flu-like illness and circulating influenza, CMO publishes advice when surveillance levels cross threshold.  Highly virulent strain would change things, of course. Further details including use of anti-virals at HPS.

Oseltamavir dose is twice daily for 5 days. Oral only, capsules can be opened and added to something sugary (very bitter).  Liquid preparation available but limited supply, prioritise for infants.  Give by NG if necessary.  Licensed for treatment at all ages now, prophylaxis only over 1 yr.  Diarrhoea and vomiting are the only significant side effects.  

The earlier it is started the better: starting within 12 hours reduced duration of illness by 3 days, start within 48 hours and only 1 day benefit. 5% of childhood infections will become resistant whereas this is unusual in adults, probably due to kids having higher viral loads in primary infection. Consider resistance if no benefit after 5 days treatment.

Zanamivir used for adolescents 12 years and older – taken again twice daily for 5 days by diskhaler (age less important than ability to use device!). No resistance, very low rate of side effects (wheeze!). Zanamavir is preferred in severely immunocompromised AND (probable) A(H1N1)pdm09 disease, as resistance is higher . Unlicensed IV form of zanamavir is available on compassionate named patient basis.

[Antiviral PHE guidance]

Prophylaxis

For prophylaxis, vaccination best!  But consider using anti-virals (NICE recommendations) for post-exposure prophylaxis with Oseltamivir where:

  • 13yrs or older
  • Have a risk factor, as above
  • Influenza A or B is circulating, as above
  • Present within 48hrs of close contact exposure
  • Have not had flu vaccine for this season, or too recently for it to have been effective (within 14 days), or the wrong type, or have a condition that means vaccine may not be fully effective, or localised outbreak eg care home

Dose is once daily for 10 days. Treatment for up to 6 weeks might be required during an epidemic.

Other comments

Amantadine not recommended – targets M2 protein, only effective against type A influenza, rapid resistance and side effects common.

2 adolescents in Japan have committed suicide while on oseltamavir, plus there have been a number of other neuropsychiatric reports.

Peramivir is IV preparation with marketing authorization in EU, not yet available in UK.