Category Archives: General paediatrics

Tissue viability

Typically a combination of moisture damage and pressure. Prevention obviously essential. Wash, clean and dry first.

Treat any infection.

For excoriation –

  • Medi Derma S barrier cream
  • Medi Derma S film for more severe – comes as pump spray or topical applicator

Dravet syndrome

Previously Severe myoclonic epilepsy of infancy. Charlotte Dravet described in 70s. Characterised by:

  • Refractory epilepsy
  • Onset in infancy
  • Associated neurodevelopmental problems

Due to defect in SCN1A gene on chromosome 2q24 (a sodium channel), usually de novo. Many mutations, don’t predict severity, unfortunately.

Accounts for about 7% of epilepsy presenting in first 3 years of life.

Onset around 5-8 months, often with febrile illness so can look like typical febrile convulsion. But often prolonged. Neurodevelopmental problems come later…

Later though, multiple seizure types. Hypotonia, ataxia, spasticity all seen. Dysautomnia can be a feature. ADHD and autistic traits common later.

EEG can be normal, or vary over time, with multifocal or generalised changes, photosensitivity too.

Post operative ENT complications

Post adenoidectomy

Trickling blood behind uvula or hanging clot are reasons for re-exploration.

Post tonsillectomy

Secondary haemorrhage typically at 5-7 days.  Beware constant swallowing! White slough normal. Yellow (with pain) might mean infection so Corsodyl or peroxide gargles, 3-6x daily, possibly antibiotics.

History of bleeding but clear fossae – assess general condition and exclude bleeding disorder (family history), safety net.

If small clot, observe to see if enlarging. Every 15 mins or more if still spitting/swallowing. IV access and fast. Sit up.

Active bleeding needs anaesthetist for airway management, crossmatch too. Tonsil tray – adrenaline solution soaked swab applied to bleeding spot with Magill’s forceps until reaches theatre. Ice packs for back of neck. Post exploration NG tube to keep stomach empty.

Tranexamic acid, DDAVP? Eg vWD

Measles

Notifiable.

Incubation period is 8-14 days. Starts with a prodrome of cough, coryza, conjunctivitis and fever lasting 2-6 days. Then the rash appears: brick red, maculopapular, starts behind ears, spreads from face on to trunk and then everywhere including palms/soles. Discrete spots may then coalesce. With time the rash may darken (“stain”) and may desquamate. The child is typically irritable – compare other common childhood rashes.

Koplik’s spots are pathognomic but easily missed as they appear early in the illness, disappearing within a few days of the rash starting. They are grey or white spots on the buccal mucosa opposite the 2nd molars.

Case definition is rash 3+ days, fever, cough, conjunctivitis or coryza plus diagnostic lab result. Diagnosis is by throat swab (or urine) for PCR. Send blood for IgG/M too.

Management

  • Check vulnerable contacts eg unvaccinated, immunosuppressed but also infants and pregnant women. There is not an explicit definition for close contact. Ideally vaccination should be offered within 3 days.
    • Pregnant vaccinated women should be fine, if in doubt do rapid antibody levels, give HNIG (Human Normal Immunoglobulin, NOT MMR) if necessary, repeat serology at 3/52.
    • Infants under 6/12 should get HNIG, unless mother has had natural measles (or born before 1970!). Else MMR, unless 6-8/12 old and a household contact or high risk.
  • Respiratory protective equipment should be worn when caring for confirmed or suspected cases viz FFP3 respirator.

[HPN Scottish Guidance Dec 2013]

Vaccination

Measles now endemic again in England and many European countries, with cases increasing year on year with only a slight reversal during lockdown. Before vaccines, pretty much inevitable part of childhood.

Andrew Wakefield in 1998 didn’t help – no immediate problem with his false paper (due to herd immunity), first death not until 2006, at which point rate 13x higher than pre-Wakefield. Vaccine hesitancy continues to be one of the biggest global health challenges of our time.

Measles after MMR occurs 7-14 days post-vaccination. Very rare after booster. Tends to be mild fever, rash and conjunctivitis.

Porto Outbreak

March-April 2018, 96 confirmed cases in a hospital in Porto, Portugal. Mostly vaccinated Health care workers!!!

Atypical presentations – mac-pap rash only, low fever.

Chances of an “escape variant” not covered by vaccine almost zero.

Hyper IgM syndrome

A group of primary immunodeficiencies characterised by inability to class switch, so high IgM but low IgA and G, leading to susceptibility to infection but also autoimmune problems.

Misnomer because high IgM is a clue but not always found!

Various inheritance patterns but mostly X-linked so variable penetrance/severity. Most affect CD40 ligand production.

Usually presents in infancy – skin, lung, sinus, eye infections. Particularly prone to Pneumocystic pneumonia, histoplasmosis, cryptosporidium.

  • Bronchiectasis seen
  • Histoplasmosis leads to fever, cough, lymphadenopathy
  • Chronic cryptosporidium diarrhoea, progressing to cholangitis and cirrhosis
  • Failure to thrive
  • Warty or chronic papular rash
  • Osteomyelitis a particular problem for type 4 where there is less susceptibility to infection otherwise and presentation can be later in life.

Autoimmunity

  • Neutropenia
  • Thrombocytopenia
  • Thyroid disease
  • Kidney disease
  • Inflammatory bowel disease

Malignancy

Increased rate of various malignancies seen.

Paediatric multi inflammatory syndrome associated with COVID19 (PIMS-TS)

Condition seen in context of SARS-Cov2 infection, with similarities to Kawasaki syndrome.

Neutrophilia (most), lymphopenia, single or multiorgan dysfunction.  Possibly Kawasaki criteria. Exclude other infectious cause including shock syndromes and myocarditis (but don’t delay seeking advice).

Abnormal fibrinogen, d-dimer, ferritin, hypoalbuminaemia. Other features eg coagulopathy variable.

PCR for SARS-Cov2 often negative, but antibody positive.

WHO refer to PIMS-TS as Multisystem Inflammatory Syndrome in Children (MIS-C), case definition is similar but requires at least 3 days of fever and either evidence of COVID-19 on PCR or serology or a likely contact with COVID-19.

In England, PICU admission related to age 15-17yrs, female, black/Asian. Length of PICU stay generally short, some require ECMO, majority survive. [Ward, MedRxIv 2021]



Asthma and allergy stereotypes

Le Chiffre in Casino Royale may use a custom metal inhaler, but the implication is clear – he is not as masculine as James Bond.

“Mikey from “The Goonies,” who is portrayed as vulnerable and nervous and is seen taking puffs from his inhaler whenever a situation is particularly scary. Stevie from “Malcolm in the Middle” who suffers from severe asthma can barely make it through a sentence without gasping for breath and wheezing uncontrollably.

“Though he is also proclaimed a genius, it is this perceived weakness that becomes his defining characteristic.

“The stereotype even translates to cartoons, with Carl Wheezer from “Jimmy Neutron: Boy Genius” and Millhouse from “The Simpsons” represented as weak and timid individuals who are used as comic relief whenever they are upset and need a puff from an inhaler to control their symptoms triggered by anxiety.” [American lung association blog]

In the film Hitch, the lovable accountant Alfred uses his inhaler when he is scared to take action.  Until he is inspired to greater manliness, and he throws it away and mounts the steps to kiss his girl in passion, no longer shackled by his psychological, rather than medical, condition. [https://mbtimetraveler.com/tag/asthma-portrayal-in-television-and-movies/]

Even JK Rowling is guilty – see her TV show “The casual vacancy”.

Stephen King’s It has a hypochondriac asthmatic character Eddie Kaspbrak – although at least there is a genuinely terrifying scene where he has an asthma attack and his inhaler has run out – but even this has been triggered by bullying, enforcing the “nerd” stereotype.

Wheezy in Toy Story 2 is also a rather pathetic character.

Positive role models lacking. David Beckham and Harry Styles are some of the few.

Children with asthma, not surprisingly, are highly sceptical of such portrayals. Non asthmatic children obviously don’t appraise movie scenes for their meanings but they do judge the social context of the drama [https://pubmed.ncbi.nlm.nih.gov/22574393/]

Few if any other medical conditions seem to get the same treatment…

Vitamin B12

Sources are animal products including meat, fish, dairy and egg.

For vegans, yeast products (eg Marmite) or fortified breakfast cereal are the only places you get it.

High levels often seen in teenagers. Appears benign, although high levels reported with lymphoma and hypereosinophilic syndrome, plus liver disease.

Irritable Bowel Syndrome

Rome IV classification gives definition as:

Recurrent abdominal pain, at least once per week for at least 3 months, associated with at least 2 of:

  • Associated with defecation
  • Change in bowel frequency
  • Change in stool form/appearance

Bloating has been removed from diagnostic criteria as it has no predictive value, being common across all kinds of GI issues.

Subtypes then based on stool form on symptomatic days – predominantly constipation, predominantly diarrhoea, mixed constipation/diarrhoea.

Normal physical examination supports diagnosis. Tests should include FBC to exclude iron deficiency anaemia, CRP for IBD, TTG antibody for coeliac disease.

Management

Trial of lactose, fructose and wheat free diet if suspected link to consumption of these foods (non coeliac gluten sensitivity occurs).

Low FODMAP diet is challenging but can help – should be supervised by dietician.

Reassurance – making diagnosis helps justify not investigating fruitlessly.

[J Clin Med 2017]