Category Archives: General paediatrics

Sedation

[NICE guideline]

Ensure that healthcare professional and assistant available and trained, with resus and monitoring equipment available.  Should have a record of practical experience of sedation under supervision, WBAs etc.

Document informed consent.

Confirm time of last food and fluid – fasting not required for minimal sedation eg nitrous oxide, or where child will maintain verbal contact.  Else 2-4-6 fasting rule.

Prepare child and  family psychologically.

For painless procedure eg imaging, use choral (under 15kg), midazolam.  For painful procedures, use nitrous oxide, oral/IN midazolam +/- local anaesthetic.

If unsuitable, ketamine or IV midazolam (+/- fentanyl) – combination of opioid and ketamine requires anaesthetic training.

[http://www.nice.org.uk/guidance/CG112/chapter/1-Guidance]

Antihistamines

First generation have prominent CNS effects, usually drowsiness (which an be useful, eg to help sleep in chronic itch) but can be hyperactivity, paradoxically!

  • Ketotifen – age 3+ only. Sugar free 300ml bottle (Zaditen) is only £17. Give with food. 1mg per 5ml, dose is 1mg twice daily from age 3 so 250 (1.25ml)-500 (2.5ml) mcg below that?
  • Promethazine hydrochloride (have to specify HCl as also comes as teoclate) 2+ for allergy but 1/12+ for sedation! Unlicensed for sedation! Phenergan 100ml sugar free £4.
  • Hydroxyzine 6/12 and over, but special order only. Risk of long QT.
  • Alimemazine – BNFc gives dose from 6 months (specialist use only and unlicensed until 2). Solution is minimum £89 a small bottle and often higher.

Second generation have lower incidence of these side effects. BSACI peanut and tree nut guidance specifies “long acting antihistamines with rapid action eg cetirizine”. Resus council anaphylaxis guideline says “non-sedating oral antihistamines in preference to chlorphenamine” may be given following initial stabilisation.

Benadryl (brand name) can be either cetirizine or acrivastine.  Latter marketed as “fastest acting allergy capsule” – I suspect the only allergy capsule.

Tmax (plasma) lowest for Rupatadine (0.75hr), levocetirizine (0.8), cetirizine (1).  Chlorphenamine is 2.8, worse than diphenhydramine (best of the sedating antihistamines at 1.7).  Loratadine (1.2) and Acrivastine (1.4) not far behind; Desloratadine has a range of values from 1-3, which is unhelpful, Fexo is definitely slow at 2.6.

In terms of clinical effect on wheal (because tissue distribution different from plasma), cetirizine, acrivastine, levocetirizine equivalent (1hr), diphenhydramine, fexo, desloratadin 2 hrs, CPM 3hrs (!).  Rupatadine is 2 hrs, despite the short Tmax!

No particular reason to think formulation matters. Possibly liquid might work directly in the mouth and throat, but only if you don’t swallow it immediately (gargle!?).

Some examples of capsules beating liquids for specific drugs (!?) but prob not much difference between absorption from tablet, liquid etc. Prob easier to swallow liquid if oral angioedema, but liquid bolus might actually be harder than tiny tablet…

[J Investig Allergol Clin Immunol 2006; Vol. 16, Supplement 1: 3-12]

Congenital pigmented naevi

Particularly giant or multiple lesions usually present at birth but can be shortly after.

Number of naevi increases through childhood, but high volatility, eg over 3yr period, about a third change pattern, sun burn increases number. Halo pattern of disappearance  (with depigmentation) rare.

Can be associated with neurocutaneous melanosis; if suspected then neuroimaging is advisable, seeking evidence of leptomeningeal melanotic lesions.

Malignant change in childhood is possible, but only really in lesions greater than 1% BSA size.  Melanoma detection relies on morphology eg ABCDE criteria (asymmetry, border irregularity, color variegation, diameter (6 mm, and evolving).  ABCDE very low specificity in kids but melanoma does present in children (and pigmented naevi are risk factor).  Dermoscopic changes in blood vessels important.

Beckers naevus tends to present at puberty,  usually torso or upper arm, irregular, hairy.

Polymicrogyria

One of the most common malformations of cortical development.  

Bilateral perisylvian polymicrogyria (BPP) is the most common subtype , causes the congenital bilateral perisylvian syndrome:

  • oromotor dysfunction,
  • cognitive impairment,
  • epilepsy.

Many possible causes eg vascular or hypoxaemic insults (eg twin-to-twin transfusion syndrome) and congenital cytomegalovirus infection. Genetic causes also important – some syndromes eg 1p36.3 and 22q11.2 deletion syndromes. PIK3R2  gene defects are the most common genetic cause of BPP in patients with normal head size, and the second (to  PIK3CA ) most common cause of polymicrogyria associated with large head size. RTTN gene  also associated with isolated BPP.

[Lancet Neurology, The, 2015-12-01, Volume 14, Issue 12, Pages 1182-1195]

Dyschezia

Baby strains and cries to pass stool but it comes out soft or not at all.  Functional gastrointestinal disorder thought to occur in 0.9 – 5% of infants under 6 months (Rome IV criteria).

Due to poor co-ordination of pelvic floor muscles with increased intra-abdominal pressure generated during stooling. Seen in babies up to 9 months.

Studies have reported symptoms of discomfort around passing normal stool in up to 18% of babies, not all of these children will strictly meet the diagnostic criteria for dyschezia.

Differentiating from true constipation etc requires a clinical history and a normal clinical examination, with the key difference being that the stool is not hard in dyschezia.

No medication (or any form of rectal stimulation) required, can be expected to resolve spontaneously.

Urate

Product of amino acid metabolism.

In developmental delay, an abnormally high or low result is significant viz:

  • Glycogen Storage disorder
  • Purine disorders eg Lesch Nyhan
  • Molybdenum Cofactor deficiency – other features are microcephaly, hyperekplexia

High urate levels can also be risk factor for urolithiasis (stones in urinary tract)

Parenting and permanence orders

Permanence order is mechanism for local authority to apply for parental rights and responsibilites to be removed from parents.

Not specifically detailed in law (2007) but “threshold test” must be satisfied:

  1. living with parent poses threat of serious detriment to welfare of child
  2. the need to safeguard and promote welfare of child is paramount consideration
  3. that it is better for the child that the order be made, than that the order not be made

Supreme Court decision In the matter of EV (A Child) (No 2) (Scotland) 2017 –

Not duty of parents to prove parenting ability, but for social work to prove lack of ability with full assessment (or adequate records and sworn evidence of non-engagement).

Also, although allegations of harm may be sufficient reason to place child in care, not sufficient for seeking “permanence order”.  Given that this may mean waiting on criminal proceedings to be completed, children may be stuck in hearing system for longer than before.

[https://andersonstrathern.co.uk/news-insight/supreme-court-permanence-order-decision-lessons-learned/]

 

Cerebral Arteriovenous malformation (AVM)

Present with hydrocephalus or else cardiac failure in neonatal period. Clue is that heart is structurally normal!

Life time risk of haemorrhage is high, presenting with headache or seizure.  Annual rate about 6%.  Focal neuro signs rare prior to haemorrhage!  Risk slightly higher in deep locations eg basal ganglia.

Once haemorrhage has occurred, risk of further haemorrhage increases significantly.  Microsurgery, radiosurgery or embolization techniques used alone or in combination.