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Topical Steroid Withdrawal

Dermatology, General paediatrics

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.

[Joint statement from National Eczema Society and BAD 2024 – https://eczema.org/blog/topical-steroid-withdrawal-updated-joint-statement/]

Fetal alcohol syndrome

Cardiology, Clinical, Community

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.

Cognitive impairment, ADHD/impulsivity, visual/hearing impairments, physical complications.

SIGN guideline 156 (and now NICE).

Assess –

  • Alcohol exposure
  • 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).

Management

SPECIFIC parenting course developed in Salford.

National organisation for FASD has algorithms etc.

Congenital cataracts

Clinical, Congenital, General paediatrics, Genetics, Neonatology

Differential:

  • Varicella
  • Rubella
  • CMV
  • Toxoplasmosis
  • HSV
  • Syphilis, HIV
  • Genetic non syndromic – various
  • Syndromes – Downs/Patau
  • X-linked Lowe syndrome (LD and proximal tubular dysfunction)
  • Autosomal dominant myotonic dystrophy (various eye problems), Neurofibromatosis type 2
  • Zellweger (includes neonatal adrenoleukodystrophy and infantile Refsum) – peroxisomal
  • Galactosaemia, Cockayne syndrome

GLP-1 receptor agonists

Endocrinology, Gastro, General paediatrics, Nutrition, OPD

=Glucagon-like peptide receptor agonists eg semaglutide.

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.

Catatonia

General paediatrics, Mental health, Neurology, Psychology

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.

  • Neurological – Anti-NMDA receptor encephalitis, MS, SLE
  • Space occupying lesion
  • Infection – encephalitis, cerebral malaria, HIV, syphilis, SSPE
  • Medication – withdrawal, alcohol, benzodiazepines, gabapentin, Zolpidem
  • Metabolic – DKA, hepatic encephalopathy, hypercalcaemia, porphyria, pellagra
  • Endocrine – Addison, Cushing, hyperthyroidism, phaeochromocytoma

Management

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.

[J Psychopharm 2023]

Second Opinions

Ethics, Generic

Extensive guidance now at RCPCH.

Social media and access to Google mean families can find out about novel and unproven interventions/diagnoses. Some notorious cases where it has resulted in conflict between clinicians and families.

External second opinion (ESO) system requires “experts”, who have intimate knowledge of the case, without bias. All of which can be problematic.

Parents do NOT have a legal right to request a second opinion. Seeking an ESO could however been as within a doctor’s professional duty, if “in the best interests of the child”. A doctor does however need to to demonstrate they have provided appropriate care for their patients, which includes showing they have weighed risks against benefits, and made reasonable and logical decisions. These decisions must be in line with ‘a responsible body of medical opinion’.

GMC guidance specifically requires doctors to “respect the patient’s right to seek a second opinion”, as they have the right to make free, informed health care choices – to do that involves clarifying clinical facts and defining treatment options. So refusing a request for an ESO could breach a doctor’s obligation to respect patients’ rights and to provide the highest standard of care.

Courts will take account of professional and clinical guidance that it is good practice to request an ESO, as a way of determining what constitutes a responsible body of opinion. Of course it is not always feasible to obtain a second (or expert) opinion, particularly in situations where a decision must be made quickly.

Nuffield Council on bioethics review went to government – https://cdn.nuffieldbioethics.org/wp-content/uploads/NCOB-Disagreements-Critical-Care-Indepdent-Review-FINAL.pdf

Hypercalcaemia

Endocrinology, General paediatrics, Renal

Do PTH to see if hyperparathyroidism (primary, or secondary to adenoma).

Do urinary calcium:creatinine ratio to exclude familial hypercalcaemic hypocalciuria (24 hour better?).

USS kidneys for nephrocalcinosis and to exclude tumour – CT head and tumour markers.

Genetics for Williams syndrome. Panels for nephrocalcinosis.

Treatment is hyperhydration.

Presenting a poster

Communication, Generic, Teaching and training

Eposters now of course as well as conference A1 old school ones.

Design

Same as with a presentation, really. Use headings and subheadings. Bullet points. Don’t mix up fonts and font sizes too much. Beware getting too close to the edges, and avoid the temptation to fill in white space! Beware jargon and acronyms.

The A4 test – if you can read your poster printed at A4, then it should work ok as an A1 poster too.

More tips here – https://xerte.shef.ac.uk/play.php?template_id=1259#page1

And use of images here – https://www.sheffield.ac.uk/library/copyright/using-images-and-other-media

Presenting

Have a pointer so you can talk through your poster easily. Turn back to your audience frequently and establish eye contact.

If a new person joins midway through, welcome them (if only with eye contact and a smile) and afterwards check if they need it explained again.

Prepare your “elevator pitch” – 3 sentence synopsis: What is it about? What did you find? Why is it important?

Keep bigger picture in mind, as some people might need more background info.

Present your poster as you would anything else – like a story. Context, characters, surprises, meaning, future.

Handouts – beware, you might stop someone talking to you by giving them a handout.

Business cards (if you don’t put your contact details on a handout).

Network – if someone particularly interested, offer to have a chat later rather than ignoring other people.

Get feedback – random comments sometimes indicate that you haven’t explained yourself fully, or you haven’t appreciated a different angle, so ask for clarification.

Thank people for interest – potential future colleagues/employers!

Rabies

General paediatrics, Infectious disease

Incubation period can be more than 1yr!

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

For more on bites, see PHS bat bites page and PHS other animal bites page.

Country and category of exposure

Determine “combined country or animal risk” – Rabies risks by country – GOV.UK

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.

Rashes affecting palm/soles

Clinical, Dermatology, General paediatrics

A short sweet list.

Acutely, Hand-Foot-Mouth (Coxsackie, or another enterovirus) is the most common. Measles can affect your palms/soles but would be everywhere…

Gianotti-Crosti syndrome typically affects hands.

Kawasaki and Toxic shock prior to desquamation.

Chronically, Scabies, pompholyx (type of eczema), Pustular psoriasis, Secondary syphilis, Janeway lesions (endocarditis).