Juvenile xanthogranuloma

Dermatology, General paediatrics

Well circumscribed, raised yellow/brown firm papule or nodule, typically solitary. Can be congenital but otherwise typically very young boys, head and neck area, asymptomatic.

Can affect the iris – presents with a red eye…

Can ulcerate, otherwise they tend to atrophy and disappear after 3-6 years.

Seen in 10% of Neurofibromatosis type 1.

Can rarely be multiple and internal (liver, bone marrow etc). Screening of asymptomatic cases probably only justified if multiple.

Differential – mastocytoma, Langerhans histiocytosis, molluscum.

Moral Distress

Ethics, Generic

Moral distress – when you feel an internal moral compulsion to act a certain way but cannot do so because of external constraints. Your morals are usually guided by ethical principles, such as beneficence and autonomy, as well as by professional virtues. Moral injury is the result of repeated experiences in which individuals act or witness actions by others that are incongruous with their moral beliefs.

The negative emotional consequences of moral distress and moral injury are depression, decreased quality of life, and burnout.

Examples are where organisational or legal rules restrict clinical practice – eg access to abortion in the US being restricted after Dobbs vs Jackson Women’s health organisation decision.

One way of dealing with moral distress is to continue practicing the professional virtues of integrity, compassion, self–effacement, self-sacrifice, and humility while maintaining patients’ best interests.

Self-effacement and self-sacrifice are the virtues that say that your wishes/feelings may need to come second to some greater good. May be uncomfortable, but doesn’t mean you are doing wrong.

Humility is the idea that what you think/believe isn’t necessarily right, and certainly won’t be right for everyone. So acting against your own morals is sometimes necessary when you are taking into account other people’s views.

Discussing these issues and feelings with colleagues will always help. Seniors should promote and cultivate a positive culture where less experienced feel able to talk openly about their feelings and identify their moral distress, frustration, and outrage without fear. Professionalism means inviting others to listen and being willing to speak openly about the constraints of practice.

Ultimately, the ideal would be compassion but without overidentification with or indifference to our patients’ plight. This is of course harder for those who may have experienced discrimination (lower socioeconomic groups, women, and racial or ethnic groups historically underrepresented).

DOI: 10.1097/ACM.0000000000005476 

Variceal bleeding

Emergency medicine, Gastro, General paediatrics

Due to portal hypertension from chronic liver disease.

Potential for large losses – may need local major haemorrhage protocol (FFP, platelets etc) – typically if blood loss >150mls/min, or else 20% blood volume loss in <1 hour (normal blood volume is 80ml/kg).

In adults, they try not to transfuse above 80 – thought that excessive transfusion may increase bleeding.

Terlipressin preferred to octreotide – from age 12. IV injection every 4 hours. No evidence for Tranexamic acid!

NG tube may cause more trauma…

In adults, Glasgow-Blatchford score used. Authors are Oliver and Mary Blatchford (couple?) – he was actually in Paisley at the time…

UTI prevention

Common, General paediatrics, Infectious disease

For lower tract:

  • Cranberry juice still not definitely proven.
  • Methenamine tablets found to be equivalent to trimethoprim prophylaxis – licensed for adults only but BNFc gives dose for children. Needs acidic urine to work so don’t use citrates at same time.
  • D-mannose some evidence – from health food shops! Capsules I think, prob no dose for children.
  • Citrates?
  • NICE CKS specifically advises AGAINST use of these non-drug products, with exception of methenamine! Prob because self initiated short course trimethoprim superior?

Social determinants of health

Sociology

David Gordon of International Poverty Research centre at Bristol has parody of Chief Medical Officer’s top ten tips for health – Number 1 is “don’t be poor”.

1Don’t smoke. If you can, stop. If you can’t, cut down.Don’t be poor. If you are poor, try not to be poor for too long.
2Follow a balanced diet with plenty of fruit and vegetables.Don’t live in a deprived area. If you do, move.
3Keep physically activeDon’t be disabled or have a disabled child.
4Manage stress by, for example, talking things through and making time to relax.Don’t work in a stressful low-paid manual job.
5If you drink alcohol, do so in moderation.Don’t live in damp, low quality housing or be homeless.
6Cover up in the sun, and protect children from sunburn.Be able to afford to pay for social activities and annual holidays.
7Practise safer sex.Don’t be a lone parent.
8Take up cancer screening opportunities.Claim all benefits to which you are entitled.
9Be safe on the roads: follow the Highway Code.Be able to afford to own a car.
10Learn the First Aid ABC: airways, breathing and circulation.Use education as an opportunity to improve your socio-economic position.

Pyrexia of Unknown Origin

Clinical, General paediatrics, Immunology, Infectious disease

A technical term, not just a fever without obvious source! Essentially presence of confirmed fever for 8 days or more in a child in whom a careful thorough history and physical examination, and preliminary laboratory data fail to reveal a probable cause.

Long list of possible causes, long lists of possible tests – do thorough history and repeated examinations, then follow the clues!

In kids, infection is the commonest cause. But can be connective tissue disorder, or malignancy.

Beware factitious fever – admission sensible.

If possible, stop all drugs. Antipyretics may obscure the pattern of fever, and can occasionally be its cause (drug fever is one cause).

Unless the child is critically ill, try not to give antibiotics. If the diagnosis remains obscure, go back and take the history again, examine the child (fully) again, send the specimens again!

Special points in history/examination

  • Travel – malaria can present 6-12 months later. Typhoid.
  • Ethnicity – tuberculosis
  • Outdoor activities – rats/ticks as vectors of infectious diseases
  • Animal contact – cows/sheep (brucellosis), cats (cat scratch)
  • Mouth ulcers (IBD, Behcets, PFAPA)
  • Periodicity – see Periodic fever
  • Sinus tenderness, nasal congestion (sinusitis)
  • Bone/spine tenderness – discitis, vertebral osteomyelitis

Tests

  • 3 sets of blood cultures, different sites, different times (at least a few hours apart), off antibiotics – standard for endocarditis
  • ASOT
  • EBV, CMV
  • LDH, CK
  • ANA/RF
  • Urine/stool culture
  • Swab everything!
[Rosie Hague, Current peds 2001]

Stroke in children

Emergency medicine, General paediatrics, Neurology

Rare but happens.

Differential:

Can be due to arterial or venous occlusion.  50:50 in kids cf adults (80% infarct). Haemorrhagic can be due to rupture into infarct.

Presents with focal signs, headache, seizures most commonly. Else dysphasia, vomiting!, confusion. Fever! Acute signs often lacking or fluctuant cf history!  FAST criteria only 78% sensitive. 

NIH stroke severity scale has paeds version. 

Risk factors

Black/Asian

Cardiac (esp surgery, right to left shunt)

Sickle cell – esp anaemia, acute chest syndrome, HbS or HbS/Beta thal

Thrombophilia

Liver/kidney disease (secondary prothrombotic tendency)

VZV within 1yr, enteroviruses, HIV.

Vasculitis – Moya Moya (peaks at 5-9yr else adulthood), SLE, other

Cocaine, glue.

Marfans, homocysteinuria, Fabry’s disease, Neurofibromatosis

Cancer, radiotherapy

Hypoglycaemia. 

Management

High flow O2, 10ml/kg saline 

Imaging within 1hr. 

BP – avoid high and low? Cf adults

Monitor for RICP

Treat with aspirin.

Tests

  • CTA/MRA at time of CT/MRI
  • Echo
  • (Transcranial doppler in sickle cell- via temporal bony window)
  • Hbopathy screen
  • Cholesterol
  • Lupus anticoagulant, Anti cardiolipin ab (ACLA), consider beta 2GP1
  • Homocysteine
  • Alpha galactosidase
  • Lipoprotein A – a kind of LDL but induces vascular inflammation, so a marker for CVS disease 
[RCPCH guideline May 2017]

Oratory (including presentation skills)

Communication, Generic, Teaching and training

We quickly get used to doing case presentations, and talking with colleagues and patients, but we don’t really learn the skills of speaking with conviction, which Winston Churchill called “the most precious gift of all the talents bestowed upon men”.

Important for career development, advocacy and leadership.

At the age of 22yr Winston Churchill said the essential components of oratory were diction, rhythm, accumulation of argument, analogy and emotion.

Presentation skills

It’s pretty obvious when a presentation is done badly – and so it is obvious what you need to do to give a good presentation.

  • Unprepared
  • Technical issues, esp poor sound
  • Overly busy slides, or slides that don’t seem to correspond with what is being discussed
  • Simply reading slides
  • Glaring bright slide backgrounds
  • Spelling mistakes or inconsistent formatting
  • Lack of a pointer
  • Not really understanding what a table/chart is actually showing
  • Rushing at the end, not leaving time for questions

The best presentations convey the importance of the topic, discuss real life issues, are funny (some of the time – otherwise risk of sounding callous). The speaker looks at (speaks to) you.

Non-verbals

Vanessa Van Edwards identified five nonverbal patterns that distinguished the most popular TED Talks:

  1. “It’s not what you say, it’s how you say it.” People rated speakers comparably for charisma, credibility and intelligence whether they watched talks with sound or on mute!
  2. “Jazz hands rock.” The most popular talks have the most hand gestures!
  3. “Scripts kill your charisma.” More vocal variety achieves better ratings on charisma and credibility. Speakers who clearly ad libbed in their talks always get rated higher than those who stayed on script.
  4. “Smiling makes you look smarter.” The longer a TED speaker smiled, the higher their perceived intelligence ratings.
  5. “You have seven seconds.” First impressions matter a lot – people had largely formed their opinion about a speaker based on the first several seconds.

Media interviews

  • Consider the interviewer someone with their own agenda – know yours.
  • Reframe any questions you see as misleading.
  • Avoid patronising or over-explaining.
  • Patient confidentiality above all.
  • Know your message(s)
[https://doi.org/10.20935/AL2219.1]

Allergy testing

Allergy, General paediatrics

Gold standard is double blind challenge, but who has time for that? And dangerous!

Mostly based on history – combination of characteristic features without other, more likely, explanation.

EAACI guidance 2023 says where type 1 allergy suspected (signs/symptoms but also timing and consistency of reaction):

  • Do skin prick testing and/or specific IgE testing as first line
  • After that, if still doubt then for peanut, hazelnut or cashew, if in doubt do component tests Ara h 2, Cor a 14, Ana o 3 respectively (if available) – otherwise do skin prick or IgE if not done already.
  • Where peanut or sesame allergy still in doubt, do basophil activation test (BAT – if available – nowhere in Scotland, as far as I know)
  • “Reassessment of food allergic children, at regular intervals, depending on age, food and patient’s history, is suggested for possible development of spontaneous tolerance”

Ara h 2 (cut off 0.44) has 82% sensitivity and 92% specificity cf 84 and 86% for SPT of 4mm, so equivalent. Cor a 14 (cut off 0.64) has 73 and 95%, so not great sensitivity. Ana o 3 (cut off 0.4) pretty good – 96 and 94%.

If random reactions, then consider hidden allergens: celery, mustard, cochineal, lupin, soy, fenugreek, other legumes such as pea/bean/lentil protein, insects/mealworm, pink peppercorns.

Panel tests

=multiple specific IgE tests done at the same time (the ultimate being the ALEX3 test, where 280+ different antigens are tested simultaneously) – likely reduced sensitivity, compared with individual test, but more importantly, potential for false positives, with attendant harms (including iatrogenic food allergy, if that food then avoided unnecessarily).

Advances of Alex3 over Alex2 include new molecular allergens including alpha-Gal, chicken (Gal d 7), celery (Api g 7, predicts anaphylaxis), wheat (Tri a 36, and Tri a 37), oak pollen, and honeybee venom; inclusion of lentil and pea.

Bartter’s syndrome

General paediatrics, Genetics, Renal

Abnormal renal excretion, leading to low potassium.

Presents in early childhood with failure to thrive. Could also be constipation, muscle cramps and weakness (potassium needed for membrane potential, so these are all neuromuscular) and non-specific dizziness and fatigue.

Characteristic hypokalemic, hypochloremic metabolic alkalosis. High plasma renin activity and high aldosterone concentration seen.

Gitelman syndrome is similar, less severe (distal tubule, rather than ascending limb of loop of Henle) – less failure to thrive, in fact often asymptomatic detected incidentally. Might present with nocturia/polyuria.

Urinary calcium excretion distinguishes the two syndromes. Bartter’s waste calcium (more severe, after all), Gitelman retain.

Treatment is with supplementation.

Decompensation can be precipitated by diarrhoea or vomiting. Acute treatment can include potassium-sparing diuretics (spironolactone), cyclo-oxygenase inhibitors and renin-angiotensin blockers.

Pseudo-Bartter’s is due to CF.