Social media storm in 2023 onwards, although already talked about prior to 2021.
No medical definition, of course. So hard to research or talk about. Concerns are around skin inflammation in previously unaffected areas, flares when you stop using topical steroids, and chronic red, inflamed skin (potentially thickened/lichenified).
Social media talks about “Sleeve sign” (palms less affected than other areas of arm), “headlight sign” (mid-face less affected than rest).
There are a range of different issues:
Erythroderma – widespread inflammation (more than 90% of body surface area), with accompanying problems with sleep, temperature control, blood pressure, fluid balance. Serious, often needs hospital treatment.
Papulopustular lesions – can be side effect of topical steroids, esp on face. Can obviously get worse on stopping steroids. Steroids can cause acne/rosacea, so you might be better with antibiotics etc. Or could be infection eg herpes, hand/food/mouth.
Tachyphylaxis – where previously effective medicine stops working. Seen with other medicines too.
Other physical symptoms eg lethargy, dizziness. Beware adrenal suppression!
Natural progression of eczema – steroids work, so when you stop giving them, the eczema often comes back.
Contact dermatitis related to preservative in creams.
Try to be supportive of concerns! Agree practical plans, offering alternatives to topical steroids if people would otherwise stop using them.
Salford study in 2021 found 1.8% rate in school (3.6% including possible cases) when actively sought – none of whom had previously diagnosed developmental problem. Estimated 2-4% in population.
Facial features (3 “sentinel” – small eyes, smooth philtrum, thin upper lip) – computer based tools available.
Brain pathology (growth ie OFC else scan)
Confirming alcohol exposure can be tricky – ED attendances? Blood alcohol levels? Police involvement? Using self completed form perhaps more reliable than saying face to face. Diagnosis can be made without good history if all 3 facial features present.
No safe limit for alcohol exposure in pregnancy. “When did you find out you were pregnant?”
Assess facial photo when NOT smiling! Other features are hirsutism, epicanthic folds, clown eyebrows, ptosis, flat nasal bridge.
Brain domains – need 3 or more. Neurodevelopmental and speech/language and sensory integration (occupational therapy) assessment. Only valid in school age children. So diagnosis in preschool only possible if microcephaly or similar.
Diagnosis is FASD +/- sentinel facial features, or “at risk” indeterminate (because too young to do proper assessment, for example).
Indicated as an adjunct to a reduced-calorie diet and increased physical activity for chronic weight management in adults with an initial BMI ≥30 kg/m² (or ≥27 kg/m² with at least one weight-related comorbidity).
NICE recommends as an adjunct to lifestyle measures only when:
it is used for a maximum of 2 years,
within a specialist weight management service, and
in patients who have at least 1 weight-related comorbidity and have BMI ≥35 kg/m² (or a BMI between 30 kg/m² to <35 kg/m² who “meet the criteria for referral to specialist weight management services”).
NICE recommends using lower BMI thresholds (eg 2.5 kg/m² less) for people from South Asian, Chinese, other Asian, Middle Eastern, Black African or African-Caribbean family backgrounds.
RCT data showed that patients receiving semaglutide lost an average of 6% to 16% of their total body weight compared with controls, when combined with other behavioural modifications. It has also demonstrated cardiovascular benefits: in adults aged 45 and older who have concurrent cardiovascular disease (but no history of diabetes), semaglutide reduces the overall risk of major cardiac events (heart attack, stroke, or cardiovascular death) by 20% at a mean follow-up of 40 months.
Common adverse effects include explosive diarrhoea, nausea, eggy burps, headache, fatigue, and hypoglycaemia in diabetic patients. Contraindicated in history of thyroid medullary cancer or MEN2.
Liraglutide is similar but was inferior in 1 head to head trial. Daily SC injections.
Tirzepatide is a dual glucose-dependent insulinotropic polypeptide and GLP-1 receptor agonist. Weekly SC injections. RCTs showed weight loss of 18-20%. Similar indications and contra-indications.
Reminds me of a certain 90s album, and the book “Awakenings” by Oliver Sacks, but still used to describe a dramatic presentation of neurological regression.
Classically stupor, mutism, rigidity (or “waxy flexibility”), but can also be “excited”, particularly in children, where it presents with agitation, catalepsy, sterotypies, echolalia/praxia.
Many of these features are seen in autism anyway, but could present in autism too.
Associated with psychiatric (eg schizophrenia) but also wide spectrum of medical conditions. Some of these are obviously differentials as well.
Assessment includes full history and examination obviously, with particular consideration to possible toxins/drugs, also DVT and pressure ulcers as complications.
Rating scales available.
EEG is important to exclude non convulsive status. “Extreme delta brush” suggests anti-NMDAR encephalitis.
Benzodiazepine challenge test – usually lorazepam – can produce responsiveness. IV or IM can be used if oral administration tricky. Zolpidem has been used for challenge too.
Escalating benzodiazepine doses used for treatment. Electroconvulsive shock therapy has traditionally been used.
Nasty viral infection that travels slowly through the nervous system until it reaches the brain. Around 30 people have survived but often with severe disability.
A 15yr old girl from Milwaukee survived with only mild neurological sequelae (reported in 2005) after treatment with induced coma (midazolam, barbiturates, ketamine) and antivirals (amantadine and ribavirin). The aim was to maintain burst suppression until rabies antibodies in the CSF started to increase (day 8). Extubated on day 27. Since then this approach has been tried multiple times, with poorly reported outcomes – has been criticised for lack of evidence of benefit but also for poor evidence for underlying therapeutic principles [Alan Jackson, neurologist at University of Calgary].
Any warm blooded animal is a potential risk, including bats, monkeys and rodents as well as cats, dogs and foxes. Animals behaving abnormally represent a higher risk of infection (but normal appearance and behaviour do not exclude rabies) – unprovoked bites obviously suggest abnormal behaviour, and carry greater risk than provoked bites.
Domestic dogs or cats behaving normally at 15 days after an exposure would not have been infectious at the time of the exposure.
The risk of infection depends on:
country of exposure – see below
category of exposure – see below – higher risk with broken skin, including single or multiple transdermal bites, severe lacerations, or where mucous membranes or an existing skin lesion have been contaminated by the animal’s saliva or other body fluid (intact skin is a barrier against infection).
site (on body) of exposure
whether the patient is immunosuppressed or has any allergies
any previous rabies vaccinations or immunoglobulin treatment
Determine category of exposure – grade 1-3, with 1 being no physical contact with saliva, 3 being direct contact. Slightly different rules for bats!
Combine the 2 according to the risk matrix –
Green get nothing, obviously, amber get rapid vaccine schedule (days 0, 3, 7, 21) – modified if fully immunised already or immunosuppressed. Red get rapid vaccine schedule plus Human Rabies Immunoglobulin.
Typically closes around 12 months of age. Can be bulging (meningitis?), sunken (dehydration), pulsatile (normal!), large (hypothyroidism), small (craniosynostosis). But actually not very predictive of any of these things in isolation.
For example – in Brazilian study of babies with craniosynostosis compared with babies with fontanelle that closed by 6 months, only 36% sensitive for craniosynostosis, and positive predictive value 59%. [https://doi.org/10.1016/j.jped.2021.10.004]
Feno (if available) >35ppb diagnostic, age 5+. If not diagnostic, measure BDR with spirometry. Diagnose asthma if the FEV1 increase is 12% or more from baseline (or if the FEV1 increase is 10% or more of the predicted normal FEV1).
If spirometer not available, measure PEF twice daily for 2 weeks. Diagnose asthma if PEF variability (expressed as amplitude percentage mean – the difference between the highest and lowest PEFR readings on a given day, divided by the mean of those readings, averaged over period) is 20% or more.
Failing that, either positive skin prick testing to house dust mite or measure total IgE level plus blood eosinophil count. Raised total IgE plus Eos >0.5 considered diagnostic! [Because highlights underlying atopy cf viral wheeze?]
Under 5, prescribe steroids [not just salbutamol!] for 8-12 weeks and review. [No dosage guide for under 5s!?] Then do objective test when they reach 5! If no response, check technique, consider environmental triggers (mould, smoke etc), consider alternative diagnosis, refer.
If making asthma diagnosis, record basis for this in notes.
Management
Under 5, consider stopping ICS at 3-6 months or else within 12 months.
If helps but then symptoms recur, can try moderate ICS dose. After that, 8-12 week trial LTRA.
Uncontrolled = exacerbation requiring oral steroids, or use of SABA 3 days a week or more, or night waking once per week or more.
New section on diagnosis at time of acute presentation!
Refer to a specialist respiratory paediatrician any preschool child with an admission to hospital, or 2 or more emergency department admissions in a 12-month period.
Age 5-11, start low dose ICS. After that, assess ability to manage MART (maintenance and reliever therapy) regimen (none licensed under 12, so would be off label). Start low (Budesonide 100/3 MDI or 100/6 Turbohaler – see below for brands), 2 puffs/sucks per day in 1-2 divided doses), go to moderate (300-400mcg per day) if necessary. [No evidence for MDI, only for dry powder!]
Otherwise would be trial of LRTA, then add LABA, then increase ICS to moderate.
12+, start Anti-inflammatory reliever (AIR) therapy with prn combination ICS/LABA inhaler (only budesonide/formoterol licensed for this). This strategy had lowest rate of severe exacerbations (plus cheaper). WockAir is cheapest.
If highly symptomatic at presentation could start MART +/- oral steroids with view to stepping down.
If MART required and still symptomatic on moderate dose, check FENO and eosinophil count – refer if either high. Otherwise trial of either a LTRA or a long-acting muscarinic receptor antagonist (LAMA, eg tiotropium).
Beware neuropsychiatric side effects of LTRA/montelukast. Review annually.
Inhalers
Duoresp Spiromax 160/4.5 (powder, 12+ – on NHSL adult formulary) – For MART, 2 inhalations daily in 1-2 divided doses (up to 2 BD); PLUS 1 inhalation PRN for relief up to 8 in a day (up to 12 for a limited time but medical assessment recommended). Also comes in 320/9 but only for maintenance.
Symbicort 100/6 turbohaler (powder) 6+ for maintenance. 12+ for MART
Symbicort 200/6 (powder, 12+ – on NHSL adult formulary) MART as above. Else AIR – 1 puff PRN, up to 6-8 (up to 12 for limited time).
Symbicort 100/3 MDI 12+ MART – 4 puffs daily in 1-2 divided doses, up to 4 BD. 2 puffs PRN for relief up to 12-16 in a day (max 24)
Wockair 160/4.5 (powder) cheapest! (£19) MART 2 inhalations daily in 1-2 divided doses, up to 2 BD. 1 inhalation PRN up to 6-8 (max 12). Else AIR – as above
Fobumix 80/4.5 Easyhaler 6+ for maintenance, 12+ for MART.
Fobumix160/4.5 Easyhaler 12+ MART or AIR.
Non-pharmacological measures
House dust mite reduction measures not routinely recommended. Evidence on removal of pets from homes “paradoxical” – no benefit or tolerance if continued presence. If detectable cat antigens without cat, might be benefit to high efficiency vacuum cleaning or additional measures.
Air pollution linked to worse symptoms.
High sodium and low magnesium intake linked to asthma symptoms but poor/no evidence that intervention makes a difference. High intake of fresh fruit and vegetables is associated with less asthma and better pulmonary function but no interventional studies.
Weight loss interventions may help asthma symptoms in overweight/obese, and should be considered, but may require >10% loss for benefit.
Breathing exercises eg Papworth/Buteyko methods can lead to modest improvements in asthma symptoms and quality of life, and reduce bronchodilator requirement, in adults with asthma. Less evidence for effect on lung function or airway inflammation. Insufficient evidence in children.
Monitoring
Monitor asthma symptoms, plus check:
any admissions to hospital or attendance at an emergency department due to asthma.
time off work or school due to asthma
amount of reliever inhaler used, including a check of the prescription record
number of courses of oral corticosteroids
Asthma Control Questionnaire, the Asthma Control Test etc can be used.
FENO can be considered for monitoring in adults only. Peak flows not routinely indicated for monitoring.
Transition
Not much! Separate section on self management. Vaping/smoking. Factors that affect inhaler use eg school/social. Career plans.
