Category Archives: General paediatrics

Adrenal insufficiency

Cortisol over 800 excludes a problem, normal response to Syncathen test (ACTH) at 1 hr should be over 470nmol/l (over 6yrs), 650 (under 6yrs).

Primary adrenal insufficiency – congenital adrenal hyperplasia (mostly 21OH deficiency), else autoimmune, genetic, infiltrative, adrenleukodystrophy (and exogenous steroid suppression).

Classic Addisonion picture – low sodium, high potassium, hyperpigmentation (can look slim and tanned, so misleadingly healthy!).

Physiological hydrocortisone replacement =10mg/m2/d in 3-4 divided doses.  Medication alert bracelet recommended, in case of crisis.  Monitor growth, BP.

If unwell, double HC dose (if varying doses, double highest dose) and give it three times daily. IM if necessary, +/- infusion.

Screen time

Sedentary time spent in front of screens programs metabolism and brain neurochem.  Similar to addiction.

But it’s complicated! Partly because there are so many different kinds of activity that can now be done with mobile phones and tablets, and because it’s difficult to control or randomize.

How people interact with screens is changing too. In the mid-2000s in the US, children over 8 spent an average of 6.43 hours a day on electronic media, but this was mainly watching TV.  Evidence of stress response (reduced cortisol increase) on waking after using screens for average 3 hours a day.

But now mobile devices are the centre piece of young people’s social lives.  Boys tend to spend more time gaming, girls more time on social networking.

Effect of high levels of screen time does not seem to be attenuated by equivalent exercise.

High levels of screen time (over 4 hours daily) is associated with poorer school performance. Social skills are poorer. These children tend to form cliques with shared interest, that create further social isolation.

Violent games and media are associated with aggression in children as young as pre-school. Aggression in children can be manifest physically, or verbally, or relationally (ignoring, excluding, spreading rumours). There is also significantly more hostile attribution bias, where you interpret behaviour (such as not being invited to a party) as hostile, even when it is not, or at least ambiguous.

Parental involvement matters – how frequently parents watch TV with their children, discuss content with them, and set limits on time spent playing video games.

Exposure to media violence must also be seen within a risks/resilience approach. [Gentile and Coyne, Aggressive Behaviour 2010] ]

Some evidence that some “social” games themed around cooperation and construction eg Animal crossing have benefits. Similarly, links between media and relational violence pretty weak, but that could be because relational violence content isn’t really examined! Actual content probably more important than time spent playing. Note that in that study of Animal Crossing, background mental health problems seemed to reduce benefit.

Maize allergy

Maize is also known as corn in English, but in America “corn” refers to wheat, so potential for confusion! Commonly used in Mexican cooking.

Allergy to maize is extremely rare. It is not one of the 14 allergens that has to be highlighted under UK/European law on ingredient labels. Cross reactivity with wheat, rice and other cereals seen on lab tests but rarely clinically relevant. It does seem to fit more with Southern European fruit allergy syndromes, including sunflower seeds.

Foods:

  • Sweetcorn, corn on the cob
  • Popcorn
  • Cornflakes and other breakfast cereals
  • Corn flour (used as a thickener so can be low level in lots of different things)
  • Baking powder often contains corn flour
  • Custard
  • Tortilla chips, tacos, nachos
  • Most wraps are made of wheat but some are made with maize or a mixture of the 2
  • Frazzles, Doritos, Squares, Hula hoops, Monster munch, Wotsits, Pom bears, Skips
  • Some of the toddler snacks by Organix/Ellas Kitchen etc
  • Cornmeal, used to make polenta and grits

Potentially corn flour could appear in tablets/medicines.

There are some reports of severe allergic reactions to fructose syrup derived from maize/corn, which is used in lots of things (including beer and other drinks). This probably isn’t a problem for most people with maize/corn allergy though, so you should only avoid this if anaphylaxis or likely previous reactions to it.

Corn oil certainly poses no allergy risk, as processing removes any allergenic proteins.

Prolactin

Secreted from the pituitary, but also a stress hormone (can be used to distinguish pseudo seizures from epileptic seizures). So can go up to 1000 in healthy people. Always worth repeating a high result at least 24hrs hours later, after a 20 minute rest.

Important because of prolactinomas, which can cause:

  • gynaecomastia
  • galactorrhoea
  • delayed puberty
  • space occupying lesion effects – headaches, visual field defects

Any lesion in the vicinity of the pituitary may also cause raised prolactin so not specific.

In children, high levels can be due to presence of macroprotein isoforms, which are not considered pathological – lab can check.

Macrolides

Erythromycin, clarithromycin, azithromycin.

Bacteriostatic not bacteriocidal, but doesn’t necessarily mean inferior.

Broad spectrum, including things that aren’t even bacterial! eg Bordetella pertussis, syphilis.

Diarrhoea and vomiting as main side effects, not an allergy as such. True allergy is virtually unheard of! Other important issues:

  • Risk of pyloric stenosis in neonates
  • Prolong QT, so beware other things that also prolong QT including electrolyte disturbance

Arnold Chiari malformation

Thought to be congenital but often only picked up in adulthood when symptoms develop.

Type 1 most common, where cerebellar tonsils protrude into spinal canal, potentially putting pressure on brainstem, spinal cord and cause obstruction to flow of cerebrospinal fluid.

Can be found incidentally. Symptoms however include:

  • headache (especially occipital)
  • neck pain
  • numbness or paraesthesiae of fingers, arms, lower limbs
  • Coordination problems, dizziness

Can be complicated by upper spinal syringomyelia.

Rarely familial.

SVT

Supraventricular tachycardia – where abnormal rhythm initiated above atrioventricular node, so narrow complex (with normal conduction down bundles of His). Typically rate over 220, with abrupt onset (can be inferred from history).

ECG showing SVT and effect of adenosine
SVT showing effect of adenosine

Accessory pathway that bypasses the AVN is the usual reason, esp infants, but nodal reentrants quite common in older kids. Get a 12 lead before doing anything, unless absolutely impossible.

An accessory pathway usually has a delta wave (upsloping PR). If retrograde conduction, then inverted P wave seen immediately after the QRS. If a nodal reentrant, P wave is subsumed into QRS complex.

If decompensated then synchronised cardioversion (under IM/IN ketamine).

Vagal manoeuvres do work, such as doing a valsalva manoeuvre (get child to blow through straw!), bag of icy water held to face (count to 5). Older kids can stand on their hands! Carotid sinus massage is the least effective.

But best to go straight to IV adenosine if access available. Adenosine causes sinus arrest, which feels terrifying for child. Within a few seconds, wears off and spontaneous return of rhythm, hopefully a normal one. Can also induce wheeze. Give in antecubital fossa, so as close to heart as possible.

Starting dose lower if over 1yr. Then increase by 50-100mcg/kg each time. If adenosine doesn’t work:

  • check max dose (different for neonates and older children, 12mg max)
  • check rapid bolus and flush
  • check diagnosis is correct.

Digoxin is good for babies and infants but needs 2 loading doses 6 hours apart, and you may not get a result until then. Propanolol is contraindicated in asthma. Amiodarone should be used before 3rd shock. Verapamil is contraindicated under 1 year as can cause arrest.

Prognosis

SVT usually settles down by 6-12 months but may recur around the age of 8yrs. Onset outside infancy will probably not resolve spontaneously. Prevention needs to be discussed: some families will be happy just treating episodes as they occur. Otherwise use digoxin in infancy to prevent, propanolol later.

Rare forms

Rare forms:

  • Permanent form of Junctional Reciprocating Tachycardia (PFJRT) – chronic SVT, so may go unrecognised and then cause cardiomyopathy. The accessory pathway is resistant to DC shock and adenosine. Inverted P wave seen.
  • Ectopic atrial tachycardia – due to an ectopic focus. May also be chronic. Demonstrates warm up and slow down in rate, whereas others tend to have fixed rate. First degree block seen. Resistant to DC shock and adenosine.
  • Atrial flutter usually seen only in congenital heart disease but does occur in neonates with normal hearts! In which case it tends not to recur. Ventricular rate divides neatly into 300.
  • Atrial fibrillation – needs anticoagulation before DC conversion so control rate first with digoxin while loading warfarin.

Schistosomiasis

Egg in freshwater taken up by snails.  Cercariae released into water and penetrate skin.  With this first infection, urticaria, discrete raised lesions 1-3cm and immune response (to dying larvae, rather than living!).  These then migrate into lungs, so acute schistosomiasis causing immune complex deposition, lymphadenopathy, eosinophilia, pulmonary infiltrates. 

Larvae mature in liver.  Adult worms migrate to mesenteric vessels of bowel, where eggs are laid.  Chronic blood loss from gut lesions. Hepatomegaly, splenomegaly, eventually varices if fibrotic. 

Worms can live 3-10 years.  Immunity only really develops where lots of dead worms, not necessarily high worm burden!  Eggs only start appearing 8 weeks after infection, and multiple samples required (intermittent excretion, perhaps every few days). 

Serology only really useful in travellers as persists? Praziquantel often causes abdo pain, rash, headache.  Only acts on adult worms.   

Latex allergy

The name for pure natural rubber. Found all over the place – foam (mattresses), condoms, balloons, seals, adhesives. In hospitals, BP cuffs, elastic bandages, catheters and ET tubes, pulse oximeters… Not so often in surgical gloves now. Can cause mild and severe (anaphylaxis) reactions, plus delayed (non type 1) allergy. Allergy first described in 1979, became epidemic in 1980s.

The rubber tree Hevea brasiliensis is not the same as “rubber plants” (Ficus) you get as pot plants, although you can be allergic to those too, of course.

About half of latex allergic patients also have fruit allergies, especially avocado, banana, kiwi, melon but also chestnut and tree nuts.

Certain high risk groups:

  • Spina bifida
  • Health care workers

Atopic or irritant dermatitis may also be caused by rubber chemicals rather then latex itself.

Diagnosis

  • Blood IgE test – as with other IgE tests, potential for false positives esp with grass/fruit allergy.
  • Skin prick test with standardised latex
  • Prick through suspected glove! Needs latex free environment, of course. Potential for reaction to powder, rather than latex…
  • Glove test – wet hand! Risk of anaphylaxis.

Risk of Anaphylaxis

As with other allergies, seems to vary between individuals. And previous reactions do not reliably predict future reactions.

With health care, difficult. First on surgical list. Label patient. Latex free environment, as far as possible. Reports of probable reactions from IV fluids and needle puncture of bungs in IV sets.

Need for careful occupational advice.

Fabry’s disease

Alpha-galactosidase defect, one of the lysosomal storage disorders, with accumulation in various tissues.

X-linked but females get disease, so not correct to call them carriers.

Classically, “pain attacks”, affecting the extremities. In the abdomen, can mimic appendicitis. Due to accumulation in nerves. Since nothing to really see on examination, easily misdiagnosed as functional.

Other features:

  • Renal impairment and failure.
  • Angiokeratomas – a more specific feature, but not always present, and seen in other lysosomal disorders.
  • Corneal changes
  • Cerebrovascular and cardiac problems

[Omim]