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Laryngomalacia

Intermittent squeaky inspiratory noise from collapsing larynx during respiration. Usually from birth.

Often worse when lying on back, or with colds, or with reflux (vomits).  Worse if hypognathia eg Pierre-Robin sequence.

Clinical diagnosis usually. Settles in first few months of life.

Will need intervention if significantly increased work of breathing, cyanosis or apnoeas, or growth failure.

Juvenile xanthogranuloma

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

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 integritycompassionselfeffacementself-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

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…

Social determinants of health

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

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!

Stroke in children

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 – marker for CVS disease, genetic. 
[RCPCH guideline May 2017]

Oratory

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.

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

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

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

NICE has list of type 1 vs non type 1 allergy signs/symptoms – some overlap, eg vomiting, diarrhoea, itch.

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
  • For peanut, hazelnut or cashew, if in doubt do component tests Ara h 2, Cor a 14, Ana o 3 respectively as well (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)
  • “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. Cor a 14 (cut off 0.64%) has 73 and 95%, Ana o 3 (cut off 0.4) 96 and 94%.

Common hidden allergens (!): celery, mustard, cochineal, lupin, soy, fenugreek, other legumes such as pea/bean/lentil protein, insects/mealworm, pink peppercorns).