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Prolonged QT interval

An abnormal finding on ECG.

QT interval changes with heart rate, so usually calculated as corrected QT (QTc), where average QT is divided by square root of RR interval (ie 1 second, if heart rate 60).

Associated with dysrhythmia, especially torsades de pointes (polymorphic ventricular tachycardia).

Seen with:

  • Genetic predisposition – Long QT syndrome
  • Certain drugs – antipsychotics (eg chlorpromazine, quetiapine), antiarrhythmics (!?), tricyclic antidepressants (eg amitriptyline), other antidepressants (eg citalopram, venlafaxine), antihistamines (terfenadine, but also loratadine, diphenhydramine, astemizole), macrolides, quinine.

Roseola

Or erythema subitum, or Sixth disease.  Dramatic viral rash.  Caused by Herpesvirus 6 and 7.

Classically high fever, URTI symptoms or no focus, potentially febrile convulsion!  Then rash appears as fever subsides, day 2-4.

Rash is widespread fine maculopapular, can be pale haloes around spots, mostly on trunk.  Can be in mouth!  Not itchy.

Pityriasis rosea

A viral rash, sometimes dramatic, can last for months!  Typically young adults. Some characteristic features:

  • Viral illness itself can be trivial
  • Usually not itchy
  • Herald patch – starts with an oval scaly macule, can grow up to 5cm in diameter. Looks like ringworm, but then:
  • Then multiple smaller scaly oval macules or plaques, typically chest and back – up to 20 days after appearance of herald patch. Classically “pine tree” distribution –  think drooping branches extending out from midline.

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]

Spiritual care

See also Difficult conversations.

Taking a spiritual history

  • do you consider yourself religious or spiritually minded?
  • where do you get inner strength from?

[Larry Culliford podcast]

[RCPsych leaflet]

All staff play a role in spiritual care.  Definition – “Allow people to explore their innermost feelings and ask the most difficult questions about suffering, illness and death”. Aim to help those in need find peace of mind.

Many levels – speaking with dignity and respect, training in bereavement, specialist spiritual care provided by department of spiritual care and wellbeing.  Spiritual care volunteers also available.

[NHSL spiritual care guidelines]

[Scottish government guidance CEL 2008.]

Staff care also important eg reflective practice, mindfulness, Schwartz rounds etc.