Autosomal dominant, PTEN gene (10q23). See OMIM.
Clinically –
In some cases intestinal polyps, papilloedema, immunodeficiency.
Lipid transfer protein. One of the allergen families. Cross reactions therefore seen with fruit (stoned fruit but also raspberry), nuts, seeds (eg linseed/flaxseed), pulses, even cereals, tomatoes, vegetables (lettuce! Cabbage!). You may also see reactions only to composite foods eg pizza, curry, due to multiple allergens being present, but only producing reaction due to co-factors.
A less common cause of Pollen food syndrome than PR10 allergy. Thought of as a Mediterranean thing but increasing reports from Northern and Western Europe. Plane tree and mugwort have LTP but not thought to be the usual cause for sensitisation (except maybe in China). In N/W Europe, often birch sensitised too but not to be confused with PR10 type PFS!
Important to identify because heat stable (so not affected by heat, processing, digestion etc in the way PR10 allergens are) and potential for severe reactions.
So do component testing if atypical (eg unusually severe) reactions to fruit.
LTP allergy also seems to be more likely to cause reactions of varying severity, compared with primary food allergy, with co-factors perhaps more important. Eating multiple different plant foods at the same time seems to be the most likely cause of co-factor associated severe reactions. Of course, co-factors can co-exist too (alcohol and dancing, for example). So some would advise:
Danger that with LTP allergy you show sensitisation to multiple foods, and then you end up on a restricted diet without knowing whether there is allergy or not.
Peach allergen Pru p 3 is a good surrogate for LTP allergy, even if peach hasn’t been a problem! If not available, you could test with SPT reagent for peach that is rich in pru p 3 (but might be false positive due to other components being present. London plane and mugwort allergy would also support.
Wheat is a bit tricky – the wheat LTP Tri a 14 is only 45% homologous with Pru p 3 so may get missed. Given the co-factor issue, probably good to do Tri a 19 (omega 5 gliadin, as in exercise induced anaphylaxis) as well.
Where hay fever and atypical reactions to nuts, do the LTPs Ara h 9 (peanut), Cor a 8 (hazelnut), Jug r 3 (walnut). You would do the other components to exclude primary food allergy which can co-exist with LTP sensitisation.
Food challenges have limited use in this situation – if positive, unclear whether LTP is the cause, and if negative, perhaps because of co-factor issue! Exercise challenge?? May just need a bigger dose!
Individualize, to balance risk of reaction against dietary restriction.
Safest fruit/veg appear to be potato, carrot/root vegetables, beans, peas, melon, cashew and pistachio. Avoid pips and skin. Banana is hit and miss.
Good results with Pru p 3 sublingual and oral peach juice immunotherapy in Spain and Portugal.
Julie McCabe died in 2011 as a result of anaphylaxis to Para-Phenylenediamine (PPD) in hair dye. Black henna is known to contain PPD, so may sensitise.
Clarifies an aspect of the Montgomery decision in a way that supports healthcare professionals getting consent. The decision can be found here.
In Montgomery, the Supreme Court said that a doctor ‘is under a duty to take reasonable care to ensure that the patient is aware of any material risks involved in any recommended treatment and of any reasonable alternative or variant treatments’. In the McCulloch case, the Supreme Court was asked to decide what legal test was applicable when assessing whether an alternative treatment was ‘reasonable’. Was it the Montgomery test or was it the Hunter –v- Hanley test? Put another way, was the decision about whether to discuss an alternative treatment with a patient one of clinical judgement, or was it one for the Court to assess and determine?
The decision was that a doctor (or other healthcare professional) who has decided that a treatment is not a ‘reasonable alternative treatment’ for a particular patient will not be negligent in failing to inform the patient of that alternative treatment if the doctor’s view is supported by a responsible body of medical opinion. In other words, this decision involves an exercise of clinical judgement and any challenge to that decision by a patient is therefore to be determined by the Hunter –v- Hanley test. In the circumstances of the McCulloch case, the application of that legal test resulted in the claim being rejected by the Court.
The court said this:
“Taking a hypothetical example – say that there are ten possible treatment options; the doctor, exercising his or her clinical judgment, decides that only four of them are reasonable and that decision to rule out six is supported by a responsible body of medical opinion. The doctor is not negligent by failing to inform the patient about the other six even though they are possible alternative treatments.
“The narrowing down from possible alternative treatments to reasonable alternative treatments is an exercise of clinical judgment to which the professional practice test should be applied. The duty of reasonable care would then require the doctor to inform the patient not only of the treatment option that the doctor is recommending but also of the other three reasonable alternative treatment options (plus no treatment if that is a reasonable alternative option) indicating their respective advantages and disadvantages and the material risks involved in each treatment option.”
[Michael Stewart, Central Legal Office]
=mpox (considered less stigmatising?).
Emerging infection particularly in men who have sex with men. Reached the UK in 2022.
New variant (Clade I) has high mortality, started in Central Africa but first case now reported in Europe (Sweden, 2024).
Incubation period is 21 days.
Besides blistering rash, can cause fever, myalgia, lymphadenopathy.
Smallpox vaccine considered effective.
Firstly, is there a suicide risk?
Then, consider mental health needs. Is there an alcohol or drug issue?
Are they known to social work? Are there any child protection issues for the young person? Their siblings or other family members? If young person is over 16 then consider Adult Protection measures (Scotland Act 2007).
A proper mental health assessment requires that they are physically well enough (consider intoxication, sedation, pain etc). Consider competency (which can be impaired temporarily by physical illness).
Consider:
=CVID. Another terribly named condition.
Usually presents in adulthood but about 20% in childhood. Typically recurrent infections of ears, sinuses, lungs – usual bugs, not funny ones.
Bronchiectasis may develop. In some cases granulomas develop.
Lymphadenopathy +/- splenomegaly is sometimes a feature.
Autoimmunity is an important feature – low red cells or platelets, thyroid disease.
Enteropathy and arthritis can be seen.
Low IgG, usually IgA, sometimes IgM. Functional antibodies (to pneumococcus, tetanus, Hib) low.
Immunoglobulin replacement – IVIG or subcut immunoglobulin, regularly.
Prophylactic antibiotics in some cases. Screen for infections esp chronic GI.
In research, many studies are non-randomized, so risk of bias.
Newcastle-Ottawa scale is one attempt to assess bias formally – judged on:
So things like cohort not being representative; control group coming from different population; measurement being rather subjective; duration of follow up – all increase risk of bias.
Suspicious if small study with big effect!
Symptoms of light headedness, dizziness, tunnel vision (pre-syncopal), potentially followed by collapse, which can be remembered in most cases. Can be brief posturing or clonus due to hypoxia, but only for a few seconds.
Recovery is fast, within seconds or a minute, once circulation to brain improves – requires that person is left lying on ground and not propped up!
They look pale, feel hot, but once on ground go clammy/sweaty. Heart rate and blood pressure typically low.
Common in teenagers, with growth spurt.
Can be reflex, from pain/fright/emotion. So with blood tests or immunisations, for example – NOT anaphylaxis.
Other differentials are epilepsy and POTS.
Patient/family info at www.stars.org.uk.
A creative way of learning/teaching about illness. But biases around being for kids, or frivolous, or simplistic.
But actually the ability to use images, fonts or other text effects means you can express things in ways both delicate and brutal that might require a lot of reading. Visual understanding is often more intuitive. By combining the two you are involving the different parts of the brain that handle language and image processing, and research shows understanding is enhanced.
They teach observational skills – you read but you must also interpret what is implied.
See MediKidz and GraphicMedicine.org.