Triad of:
Cool IV fluids, ice packs, bear hugger on cool, etc.
Benzodiazepines, PO/NG cyproheptadine.
RSI.
No role for antipyretics! No evidence for dantrolene. Avoid suxamethonium, fentanyl, alfentanil.
Legal highs now illegal!
=“New psychoactive substances” – no penalty for possession. Generally multiple substances taken simultaneously. Previously sold as herbal incense or “bath salts”. Now online “party pills” etc. Packaging can remain the same but product changed. Mostly from China.
Drug deaths in Scotland 3x higher than in UK as a whole, and higher than any other EU country. Since NPS are unidentifiable and typically multiple substances taken, it is hard to attribute specific deaths. 72% male. Synthetic Cannabinoids, cathinones (stimulant), phenethylamines (hallucinogenic), benzodiazepines. Can be smoked or ingested.
Toxbase has nicknames, but examples are Black mamba, Exodus, Damnation.
Cannabinoids can cause tachycardia, long QT and hypokalaemia. For agitation avoid medication if possible else midazolam. Generally 6 hour effect.
Cathinones snorted or injected as well. Euphoria, intense positive emotion. Dyspnoea, palpitations. Narrow complex arrhythmia. Trismus. Acidosis. Hyperpyrexia. Effects up to 24 hours. Check CK, coagulation, LFTs.
To treat acidosis, treat everything else! Then phone Toxbase!
Phenylethylamines stimulant as above plus hallucinations. Coronary ischaemia, organ failure.
Synthetic benzodiazepines tend to have pseudoscientific names. Flumazenil not used as risk of other drugs emerging to cause seizures etc.
See Serotonin toxicity syndrome for hyperpyrexia, increased muscular activity, autonomic instability.
BRUE (Brief Resolved Unexplained Event) from AAP 2016, replacing ALTE (apparent life threatening event). “Life threatening” is unnecessarily anxiety provoking – and subjective for parents.
“Brief” is by definition less than 1 minute, but typically 20-30 seconds. Only intended for babies under 1yr.
Guidance for “low risk”:
If low risk criteria fulfilled, no investigations are required – consider gas and urinalysis if clinical concern.
Otherwise depends on history and examination. Consider:
Management could then be a period of observation, or discharge home with safety netting.
AAP advises against home cardiorespiratory monitoring given costs and false alarms vs uncertain benefit.
A US study looking at this guidance found that
But I’m disappointed there is no mention in the RHC guidance about SIDS prevention advice.
Associated with range of negative outcomes.
Adverse childhood experiences increase probability of smoking in adulthood. Physical harm in Eastern European countries increases the probability of heavy drinking by about 3.4%, but not in other regions. Exposure to child neglect (little understanding) increases the probability of alcohol abuse by 2.7% in Nordic Countries but not other macro-regions. The experience of a poor relationship with parents is a strong predictor of alcohol abuse for the female subsample in Latin countries (2% higher).
While ACE does not appear to have a substantial effect on excess weight in any macro-region, childhood trauma (physical harm) appears to have a major impact on the likelihood of being obese later in life. Obesity has a more obvious impact on chronic illness than poverty, smoking or alcohol.
Tender, warm, poorly demarcated subcutaneous nodules. Acutally a panniculitis. Classically on the shins but can be widespread.
Mostly caused by Group A strep or drugs, but can be a clue to:
NSAIDs effective. Potassium iodide used in chronic/recurrent cases (can cause hyperthyroidism)!
Steroids should rarely be considered.
A Cryopyrin disorder, found in Northern Europeans. Cryopyrin triggers an IL-1 dominated inflammatory response, and is coded for by the Cold-Induced Autoinflammatory Syndrome 1 (CIAS1) gene, also known as the NLRP3, NALP3 or PYPAF1 gene.
Attacks of periodic fever are very brief eg 1-2 days – apart from fever, an urticarial rash is sometimes seen, limb pain/arthralgia occurs. Abdominal pain and arthritis occur rarely. Sensorineural hearing loss is characteristic.
Amyloidosis affects 25%, which is high cf other periodic syndromes.
Diagnosis is by genetics.
Steroids are often used but benefit is inconsistent; interleukin 1 (IL-1) receptor antagonist Anakinra shows promise.
Familial cold autoinflammatory syndrome (FCAS) is a similar condition, also related to Cryopyrins. Cold induced obviously, but without the deafness, and amyloidosis is rare.
NOMID/CINCA are also related – the names say it all: Neonatal onset multisystem inflammatory disorder, and chronic infantile neurological cutaneous and articular syndrome. Papilloedema and uveitis potentially leading to blindness occur; there is epiphyseal bone formation; hepatosplenomegaly; and a chronic meningitis with deafness. There is no known treatment, sadly.
Mostly Dutch and French. Not to be confused with Hyper IgE syndrome or Hyper IgM syndrome. Big database in Nijmegen. A genetic syndrome (autosomal recessive, explained by MVK (mevalonate kinase) gene mutations on chromosome 12p – see Omim) with recurrent febrile attacks starting under 1yr of age.
Attacks last 3-7 days, so may or may not be shorter than TRAPS, occur every 4-8 weeks. Features are:
Diagnosis is by finding of high IgD (>100U/ml); most also have high IgA (with or without raised IgG and IgM), which is an important clue.
Increased Mevalonic acid in urine during fever.
Febrile attacks in response to immunizations often reported, so may be another clue.
Attacks tend to diminish with age without completely disappearing; amyloidosis seems to occur only rarely (cf TRAPS). Simvastatin is supposed to help!
First described in a family of Irish descent, hence “Hibernian”, now called TRAPS (TNF receptor assoc periodic syndrome), and now described in a wide range of different ethnicities.
Various mutations of TNF-Receptor Super Family 1A (TNFSF1A) seen, on chromosome 12p (same as HIDS but different gene). These mutations are dominant and penetrate poorly, with only a small proportion developing disease.
Onset is typically around 3yrs of age but varies widely. Periodicity also varies widely: typically every 5-6 weeks. Fever for 3 days heralds onset of other symptoms, which then last for usually 5 days or more (cf Familial Mediterranean Fever):
About 14% develop amyloidosis.
Diagnosis is mainly clinical. Must have at least 6/12 history of recurrent inflammatory symptoms, with at least one of the above features, episodes must last at least 5/7 on average (even if variable), with response to steroids but not colchicine. Other affected family members will obviously increase your suspicion. Ethnic group does not seem to have any bearing.
Steroids reduce severity but not frequency of attacks. NSAIDS help fever. Etanercept appears to prevent; colchicine does not (hence one of the diagnostic criteria above!).
[Medicine 2002;81(5):349-68 PMID 12352631]
Short attacks of fever, usually lasting 1-3 days, recurring at varying intervals (periodic), cf Behcet’s.
Most children develop severe abdominal pain with the episodes, due to sterile peritonitis.
Pleuritis, leading to chest pain, arthritis, myalgia and skin rashes may also occur.
Most cases are from Arabic Turkish, Armenian or Jewish background. Inheritance is autosomal recessive. The gene has been cloned and four mutations have been identified.
Colchicine is the treatment of choice. Some patients may develop amyloidosis; certain mutations are at higher risk.
Recurrent fever and aphthous stomatitis, mostly. However, there is no regular periodicity of the symptoms, and episodes of fever may last for weeks.
Traditionally associated with Mediterranean or Eurasia but in UK mostly white Caucasian.
Other features –
BPSU study found 1 case every 2 weeks in the UK. Median age of onset was 6yrs – but diagnosis 11yrs!
Colchicine as regular preventive treatment, else immunosuppressive treatment. Topical steroids or short courses oral steroids.