Common form of childhood epilepsy. cf juvenile absence epilepsy.
One or more of:
Quite different from childhood absence epilepsy! Less nice.
Not v uncommon in obese children/adults, which is logical. But non-alcoholic fatty liver disease (97% of which is Metabolic dysfunction associated steatotic liver disease (MASLD)) may progress to steatofibrosis, non-alcoholic steatohepatitis (NASH), cirrhosis, liver failure and hepatocellular carcinoma. It is now the most common cause of liver disease in adolescents and amongst the top three indications for liver transplantation in adults.
Data from 2020 in US found prevalence of 20% for obesity and MASLD among 12-17yr olds, with about 70% of obese adolescents affected by MASLD. More common in boys, particularly among Mexican American adolescents. Adolescents with MASLD had significantly higher triglyceride levels and alanine transaminase (ALT) levels, along with lower high-density lipoprotein (HDL) cholesterol. Insufficient physical activity and poor diet quality were key risk factors, not surprisingly.
And not all cases are obese.
Alcoholic fatty liver disease is a different thing – depends on type of beverage, genetic risk factors, drinking pattern, duration of exposure etc so unpredictable.
Particularly likely to progress when co-exists with metabolic syndrome (obesity esp high waist circumference, high blood pressure, high insulin resistance, high lipids). Some polymorphisms also contribute higher risk of progressive disease. So family history important too.
Some drugs can contribute, including methotrexate, steroids, valproate.
On examination, look for acanthosis nigricans (marker of insulin resistance) as well as signs of chronic liver disease. Hepatomegaly suggests an alternative diagnosis.
Essentially to assess co-morbidity and exclude other causes:
So pretty much as for viral or autoimmune hepatitis.
Non-invasive measures of fibrosis available in some centres.
[Caroline says only if double normal)
Patient information leaflets and guidance for NAFLD are available via the Children’s Liver Disease Foundation at https://www.childliverdisease.org
[BSPGHAN 2020 guideline]
Can cause allergy like symptoms eg wheezing, itching, swelling, nasal congestion. Also headaches, abdo pain. Anaphylaxis reported.
Found in foods, medicines, beauty products.
High levels:
Pineapple juice high but fruit itself not listed!?
Medium:
Benzoates related. A variety of other foods esp fruits but also avocado, vegetables, nuts, coffee, beer too.
If suspected, exclude high salicylate foods only to begin with, and limit medium salicylate foods if possible. 4/52 minimum. Includes topical including anything with “Natural plant extracts”.
If improves, excluded medium too for further 2/52 to see if additional benefit.
(draft BDA FAISG)
Superceded by SIGN/BTS 2019.
For suspected asthma, where child unable to do spirometry, then watchful waiting or trial of treatment for specified time period. Choice of treatment depends on severity and frequency of symptoms – “typically 6 weeks inhaled steroid”, “very low dose”.
Start regular preventer treatment or escalate treatment if you are getting frequent symptoms, viz:
Start regular preventer if asthma attack in previous 2 years!
Same table for all ages now, and same steroid doses!
Step 1 – very low dose inhaled corticosteroid (ICS). OR leukotriene receptor antagonist (LRTA) if under 5.
Step 2 – Add LRTA if under 5, else inhaled long acting Beta agonist (LABA) if 5+.
Step 3 – If no response to LABA, stop and increase ICS dose. If some benefit from LABA continue and increase ICS dose, or consider trial of LTRA.
Step 4 – high dose therapies: increase ICS dose to medium, or add slow release theophylline. Refer for specialist care.
Very low dose is 50mcg 2 puffs twice daily of beclometasone. Low dose is double that, medium 200mcg 2 puffs twice daily.
QVAR and fluticasone are double the efficacy of beclometasone so doses are halved. Ciclesonide is somewhere in between.
“Highly allergic” or “severely allergic” can mean low threshold for reacting, or severe previous reaction, or both!
Lowest observed adverse effect level is the formal term for the minimum dose at which you react! An eliciting dose (ED) can then be calculated through multiple food challenges for a stated proportion of the allergic population. For example, 1 peanut =150mg protein. ED 1 (where 1% of allergic population will react) is 1.3mg. But range of reported values – type of peanut allergy? Entry criteria? Boiled or roasted? Criteria for stopping challenge?
Higher eliciting dose predicts future tolerance, at least for milk and peanut, maybe not for egg.
Children probably more sensitive although ED 10 similar for children and adults.
Conflicting evidence on severity of reaction vs threshold. Certainly trace amounts can cause anaphylaxis in some cases. Higher fat content delays absorption, so the reaction (when it happens) can still be severe…
ED05 of 1.5mg for peanut seems safe [Hourihane].
Being cross allergic to other things might help predict – in French study, 3 phenotypes emerged from cluster analysis:
Complicated though – thresholds vary up to 10x for individuals in sequential challenges (n=14)!
On the day factors – alcohol/meds, infection. Exercise is a factor in 15-20% of anaphylaxis episodes according to German/Austrian registries.
TRACE peanut study – 45% reduction in threshold for exercise and sleep deprivation (independent) esp at lower eliciting doses. ED1 and ED5 remain above 0.2 though.
Sleep deprivation (sleep overs!) worsened severity of reactions too, by 48% – exercise 28%, not significant. Repeated challenges also seemed to increase severity. [score for severity using Practall criteria. JACI 2022 Dua]
Lower thresholds had higher BAT and SPT – 8mm cut off had 100% sensitivity and NPV for severe reactions, and 92% specificity. Has nomogram combining all tests! SPT 6mm had 92% sensitivity, 95% specificity and 100% NPV for low threshold. [JACI 2020, Santos study – LEAP cohort]
Particulate contamination risk seems to be a particular problem for milk in chocolate – big range of values found cf peanut.
UK and Europe look at processes as the way to assess risk of contamination with allergens. But not consistent between countries of the EU, let alone globally.
Quantitative methods would appear to make sense – how much allergen is actually present in a given sample? But of course one sample may differ from another (“particulate contamination”). Also difficult to establish minimum eliciting or threshold dose (consider denaturation of the allergen during processing, derived ingredients eg glucose syrup from wheat, soya lecithin, effect of food matrix, individual factors).
Australia and NZ use lowest observable adverse event level (LOAEL) already – warnings required within 10x concentration of LOAEL (“VITAL” threshold, as in toxicology). Studies from Europe and US have found most advisory warnings used for ingredients below the VITAL threshold.
But then it depends on how much you eat, as well as presence of other co-factors that are known to contribute to risk of anaphylaxis.
[BMJ 2011;343:830][Allergy 2021, Paul Turner]
Not a true nut (along with chestnut, pine nut, tiger nut, doughnut etc), classified as a fruit, from palm family. Allergy is pretty rare, but is possible, usually only seen in people with other allergies. Associated with macadamia, almond, walnut, hazelnut, lentil and latex allergy.
The major allergens are storage proteins (7s globulin=vicillin like), so not affected by heat.
In EU, does not have to be specifically highlighted on labels, but in US FDA do include it under nuts!
Other names , particularly when used in cosmetics:
Cocos, cocamide sulphate, cocamide DEA, CDEA, Cocamidopropyl betaine.
People who react when they eat coconut don’t necessarily have a problem with it on their skin, but this may depend on whether they have eczema. Where people do react to skin products, complicated, because one of the intermediates in manufacturing (found at very low level) appears to be a more likely cause of type 1 reactions than coconut itself or one of the listed coconut derivatives.
Contact dermatitis triggered by coconut much more common than type 1 allergy.
Sources of coconut:
Incubation period 1-4 days. Infectious period from day before symptoms appear, to 5 days after symptoms appear. Virus shedding can persist for months in immunocompromised (as other viruses).
Main types A and B. B shows antigenic drift, with minor variations over time. A shows antigenic shift, with appearance of new N type usually associated with global pandemic eg Spanish flu. Influenza viruses circulate through birds and pigs, so new types typically occur through reassortment of genes across different species (Chinese food markets are a perfect petri dish).
At least one of these systemic symptoms: Fever (or feverishness), malaise, headache, myalgia;
PLUS at least one of these respiratory symptoms: cough, sore throat (!), shortness of breath.
Prevention is therefore by good hygiene viz covering nose and mouth during coughing/sneezing, wiping with disposable tissues, avoiding touching nose/eyes/mouth, washing hands (alcohol gel adequate if hands visibly clean); and immunisation.
For close patient contact, aprons, masks, gloves recommended. Similarly, consider eye protection.
For aerosol generating procedures, FFP3 (filter face piece, efficiency grade 3) masks required. Such masks can be worn for up to 8 hours if necessary. Fluid repellent gowns if extensive secretions or bodily fluids anticipated.
Neurological presentations are mostly transient, improving within 48 hours. Pre-existing neurological disorders more at risk (unusually severe seizures in known epileptic children, mostly).
Rarely acute necrotizing encephalitis (treat with high dose steroids). CT can show (classically bilateral thalamic necrosis) but MRI better of course. Not just influenza but viral, and immune mediated (no virus found in brain/CSF). Familial susceptibility described due to RANBP1 mutation. [2024 Review from Toulouse]
See immunisation. Antigenic drift means new influenza vaccines need to be developed each year, reflecting the common serotypes affecting people in other parts of the world who have already had their winter.
2021 metanalysis (37 studies) found immunisation in children was 53.3% effective against hospitalization (68.7% vs flu A/H1N1pdm09 specifically).
Only 44.3% for live-attenuated influenza vaccines cf 68.9% for inactivated vaccines.
2017 metanalysis: similar effectiveness vs asthma ED visits.
Long list of people at higher risk eg chronic cardiac/respiratory conditions. Includes pregnant women. See Green book on immunizations.
Nasal (live) and injectable (inactivated) vaccines available.
See Influenza treatment.
= excessive sweating.
Primary usually develops in adolescence, usually focal esp palms, soles, axillae. Often family history.
Secondary tends to be generalized but can be focal. Long list of causes – endocrine, neurological, chronic infection, catecholamines etc.
Treatment in children is limited to anti-perspirants and iontophoresis devices (these would have to be purchased by the family – there are tray devices for feet and hands, or pads for the body, you use 3 times a week).
For the armpits you can use aluminium hydrochloride (eg Driclor). It can cause irritation so you need to dry the armpit carefully before use (a blow dryer can be good for this).
You may sometimes need topical steroid if the skin gets very inflamed.
I believe GPs can prescribe 1% glycopyrrolate in cetomacrogol cream for use 2-4 times daily.
For widespread sweating, you can prescribe probanthine or oxybutinin as you would for adults – build up the dose slowly to avoid side effects. Clearly surgery (sympathectomy) and botox are a last resort, and you would need to find a provider happy to offer this to a young person under 14.
Patient Support is available at www.sweathelp.org (US) and https://hyperhidrosisuk.org/ (UK).
Young person site http://www.sweatometer appears to be down.
An added sound heard when listening with a stethoscope, distinct from heart sounds or other clicks or snaps.
Can indicate a structural abnormality. But can be heard in normal hearts too, esp kids.
Or “innocent” murmur. Characteristic vibratory, low pitched, crescendo-decrescendo sound – “like an Aeolian harp” – loudest along left sternal border. Never louder than grade 3. Typically gets quieter when child sits or stands up (reduced venous return?) – you would not expect a murmur caused by a structural abnormality to change much.
Present at any age. Usually goes away by adolescence.
Another innocent one, a rumble heard in the upper chest, disappears when lying down, or when neck turned or neck veins occluded gently.
Pulmonary valve closest to anterior chest wall, which might explain why you sometimes hear this. Might be confused with pulmonary stenosis or subaortic membrane.