Cushing’s disease

Excessive cortisol due to an ACTH secreting tumour. In children, Cushing’s syndrome usually due to steroid treatment.

Manifests as dramatic weight gain, usually with angry striae, growth arrest (in terms of height), change in facial appearance, buffalo hump, hypertension.

These findings are hard to spot given that obesity with striae (and hypertension) in children common. Main clue is height centile below mean (and below MPH). Delayed bone age also a clue, as both of these things tend to be above normal in obese children.

Investigation is tricky as pituitary adenomas are not always seen on MRI and petrosal sinus sampling for cortisol is sometimes required! Even exclusion is tricky, requiring 24hr urinary cortisol collection over 3 days, low dose dexamethasone suppression testing.

CRH test done as part of work up, to see if ACTH is ectopic, which is exceptionally rare. Increased response to CRH test is almost diagnostic for Cushing’s though! High dose dexamethasone suppression rarely done.

Alpha-gal allergy

Described in 2015, revolutionary in that allergy is to an oligosaccharide (ie a sugar, not a protein), specifically galactose-alpha-1,3-galactose.

Accounts for anaphylaxis to cetuximab, a cancer drug, but even more bizarrely, allergy to red meat (beef and pork). The latter appears to follow sensitization through a tick bite, so is really only an issue in endemic areas eg parts of United States and Europe, Australia.

Anaphylaxis to red meat can be immediate or delayed, with or without exercise induction!

In a small series of beef allergic patients reported in 2003 (strong family history), skin prick and labial contact tests only positive in minority.  All positive on IgE.  In another series, most beef allergic were also gelatine allergic.  Interestingly, a proportion of “idiopathic” anaphylaxis turned out SPT positive for gelatine. 

Bovine specific albumin is another possible allergen for beef allergy.

In Asia allergy described to galacto-oligosaccharides in milk formula, also a carbohydrate!

Thought to be T cell indepedent!

IgE test available.

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.

Radiology

A quiz! Choose from:

  • Heads
  • Chests
  • Abdomens
  • Bones
[qwiz repeat_incorrect=”false”]

[q topic=”Heads”] 6/12 old, referred as plagiocephaly.  OFC 99.7th.  Mild developmental delay. ?facial asymmetry and impalpable lambdoid suture. 
 
 

[c]IFNob3cgbWUgdG hlIGFuc3dlcg==[Qq]

[f]IEJlbmlnbiBwcm9taW5lbmNlIG9mIHZlbnRyaWNsZXMgYW5kIGV4dHJhIGF4aWFsIHNwYWNlLsKgIFBsYWdpb2NlcGhhbHkgZXZpZGVudC7CoCBCb3RoIHBhcmVudHMgaGVhZHMgb3ZlciA5Nw==dGg=IGNlbnRpbGUu[Qq]

[q topic=”Chests”] 3yr old, had eaten chicken nuggets the previous night and choked.

He developed cough, the following morning grunting. On admission marked increased work of breathing and wheeze.   
 

[c]IFNob3cgbWUgdG hlIGFuc3dlcg==[Qq]

[f]IEh5cGVyaW5mbGF0ZWQgcmlnaHQgbHVuZyBkdWUgdG8gYmFsbCB2YWx2ZSBlZmZlY3QuwqAgMiBsYXJnZSBmb29kIGJvbHVzZXMgb24gYnJvbmNob3Njb3B5LsKg[Qq]

[q unit=”Chests” topic=”Chests”] 15yr old, Bronchiectasis, asthma 
 

[c]IFNob3cgbWUgdG hlIGFuc3dlcg==[Qq]

[f]IE1jbGVvZHMgc3luZHJvbWUgKFN3eWVy4oCTSmFtZXPigJNNY0xlb2QmIzgyMTc7cyBzeW5kcm9tZSAoU0pNUykgaXMgYSByYXJlIGNvbmRpdGlvbiBjaGFyYWN0ZXJpc2VkIGJ5IHVuaWxhdGVyYWwgaHlwZXJsdWNlbmN5IG9mIGEgcGFydCBvZiwgb3IgdGhlIGVudGlyZSwgbHVuZy4gUHJlc3VtZWQgYWNxdWlyZWQsIHNlY29uZGFyeSB0byB2aXJhbCBicm9uY2hpb2xpdGlzL3BuZXVtb25pdGlzIGluIGNoaWxkaG9vZA==LsKgIA==UHJlc2VudHMgd2l0aCBmZXcgc3ltcHRvbXMsIGlmIGFueSHCoCBDYW4gbWltaWMgcG5ldW1vdGhvcmF4

Cg==

[Qq] 

[q unit=”Heads”] 5yr old periorbital cellulitis, high fever and CRP 
 
 

[c]IFNob3dtZXRo ZWFuc3dlciE=

Cg==[Qq]

[f]IDZtbSBzaGFsbG93IGNvbGxlY3Rpb24gdHJhY2tzIGFsb25nIHRoZSBsYXRlcmFsIHdhbGwgb2YgbGVmdCBldGhtb2lkIHNpbnVzIC8gZXh0cmEgY29uYWwgcG9ydGlvbiBvZiB0aGUgbWVkaWFsIGxlZnQgb3JiaXQ=[Qq]


[/qwiz]

 

 

 

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

Shared Decision Making

[NICE guidance 2021] See also participatory medicine and family centred care.

First bullet point is that this should be embedded at organisational level! Includes:

  • provide access to patient decision aids (PDAs) or information about risks and benefits
  • review how “information systems” might help record discussions and decisions, for example through patient held record
  • prompt patients (through media, posters, letters) to ask questions about options, and “making the decision that’s right for me

Staff training:

  • evidence based model eg three-talk model (introduce choice, describe options, help explore preferences and make decision)
  • communication skills – avoiding jargon, explaining technical terms
  • communicating with families and others the patient would like involved

Note that NHS England has “accessible information standards”.

In practice:

  • Agree an agenda
  • Ensure the person understands they can take part as fully as they want in making choices about their treatment or care
  • When it comes to tests or treatments, explain what the health care aim is of each option, and discuss how that might align with patients “aims, priorities and wider goals”
  • chunk and check information
  • check understanding eg teach back
  • Give information away at time of discussion or very soon after, including contact details
  • Write letters to the patient rather than to their doctor, in line with Academy of Medical Royal Colleges’ guidance on writing outpatient clinic letters to patients (and to patient, unless they don’t want a copy).

Communicating risk:

  • Make clear how information applies to them personally, and how much uncertainty applies
  • Use mixture of numbers, pictograms and “icon arrays” (repeated icons, with different colours, to see proportions), to allow people to see both positive AND negative framing
  • Be aware that different people interpret terms such as ‘risk’, ‘rare’, ‘unusual’ and ‘common’ in different ways
  • Use absolute risk rather than relative risk. For example, the risk of an event increases from 1 in 1,000 to 2 in 1,000, rather than the risk of the event doubles.
  • Use 10 in 100, rather than 10%
  • Use the same denominator
  • State both positive and negative framing

Participatory medicine

Patient led campaign for better involvement of patients in their own care.

Manifesto has 5 principles:

  • Share and listen – acknowledge patients are experts in their own lives and bodies, use plain language.
  • Respect one another
  • Share information responsibly – help patients access the information they need, and respect confidentiality
  • Promote curiosity – be clear even when it is difficult to explain, encourage patients to do their own research and get involved in patient communities
  • Be a teambuilder – treat patients as collaborators, respect their goals, values and preferences

These are shared responsibilities, the patient has their own duty to be honest, ask questions, advocate themselves.

Institute of Medicine describes a continuously learning system (in respect of healthcare in the US), the second feature of which is Patient-clinician partnerships. This is explained as “engaged, empowered patients – a health care system anchored on patient needs and perspectives, and promoting the inclusion of patients, families and other care givers as vital members of the team“.

Unclear this happens on any significant level, especially when it comes to communication outside the hospital or clinic room. Studies have found that when patient portals or messaging systems are used, patients often respond with further questions or comments, which suggests unmet need and desire to engage further. Other studies have found that only a tiny minority of messages in such systems are initiated by the health care team, which suggests a reactive rather than active contribution to the partnership.