Confidentiality

December 17, 2014Ethicsconfidentiality, information governance, patient recordsadmin

For information to be confidential in law it must:

not be common knowledge among lots of people, for example, the
content of a discussion between a patient and a health professional;
and be useful and not irrelevant or trivial

Explicit consent is not required for the following purposes:

Where patient information is used for the routine clinical care of that patient – for example between health professionals and intra NHS multidisciplinary teams
Where patient information is used for administration and management purposes, for example, waiting list management.
Child protection purposes (see below)

Most patients understand that their information must be shared within
the healthcare team. However, patients do not expect this to be shared
with others who will not be involved in their care.

Aggregated and management information is used to plan and monitor
progress of the organisation in its delivery of services. This is
generally outside the scope of the Data Protection Act 1998 on the
basis that a living individual could not be identified from such data.

Anonymized/coded information would normally fall outside the scope of
the Data Protection Act 1998, but care must be taken with all coded
and anonymised information as it may still be possible to identify
individuals, e.g. with rare diseases, drug treatments or statistical
analyses within a small population.

For other uses, it is your responsibility to make sure that patients
are aware of the wider uses of their information and to get their
permission. It is your responsibility to make sure that you provide
versions in any community languages or meet other accessibility
requirements.

You should:

* make clear to patients when information is or may be disclosed
(shared) to others involved in their health care;

* make sure that patients are aware of the choices that are available
to them on how their information may be disclosed and used;

* check with patients to make sure that they have no concerns or
questions about how their information will be disclosed and may be
used;

* answer any questions personally or direct patients to others who can
answer their questions; and

* respect the rights of patients and help them to access their health
records if they have asked to do this.

Intra NHS Information Sharing NHS Scotland policy

3rd party info eg family history or info coming from an identifiable
family member – may itself be confidential, even if in patient’s
record, so 3rd parties should be told if their identity might be
revealed, and given opportunity to decline.

Beware use of automated reports for insurance etc, where only minimum
necessary info is appropriate, and would be wrong to disclose more
than that.

Option to opt out of data analysis emphasized – not clear how this
would be done.

Training seen as tick box rather than education.

£100 000 fine for Aberdeen city council for a home worker accidentally uploading files regarding vulnerable children to an online file store. A fine has never been issued for formal sharing of information. “Blagging” is also an issue, where a 3rd party obtains information by ostensibly acting as a healthcare professional or family member.
Guidance from 2003 still applies – obtain confirmation of identity (or
phone number etc) from second source. If breach occurs, individual
should be informed.

See also Data Protection for Caldicott principles etc.

Child Protection

Scottish Government’s Sharing Information about Children at Risk: A Guide to Good Practice (2003) states:-

“If there is reasonable concern that a child may be at risk of harm this will always override a professional or agency requirement to keep information confidential. All professionals and service providers have a responsibility to act to make sure that a child whose safety or welfare may be at risk is protected from harm.”

The National Guidance for Child Protection in Scotland (2014) states:

‟Harm‟ means the ill treatment or the impairment of the health or development of the child, including, for example, impairment suffered as a result of seeing or hearing the ill treatment of another. In this context, ‟development‟ can mean physical, intellectual, emotional, social or behavioural development and ‟health‟ can mean physical or mental health.”

Recent advice has also been received from the Scottish Government, having consulted with the Information Commissioner’s Office, regarding the impact of GDPR and the Data Protection Act 2018 in this area. The Information Commissioner’s Officer has confirmed:

“It is important that those whose work brings them into contact with children and young people continue to share child protection concerns in the same way as they did previously. Child protection matters at the significant harm level equate to sharing/processing being necessary to protect the vital interests of the child where reliance on consent may be prejudicial to that purpose. The same lawful purposes are provided for in Articles 6:1(b) and 9:2(c) of the GDPR for personal and special category data so nothing has changed at that level”.

It is important to be open and transparent and make people aware that we will share information when we suspect a child or young person is at risk of harm. It is also important to record any decision to share or not to share information and reasons for doing so.

Relevant Acts/Policies

* Human Rights Act 1998

* Freedom of Information (Scotland) Act 2002

* NHSS Code of Practice on Protecting Patient Confidentiality.

* NHSS Information Governance standards 2005

Note that Scotland leads patient data protection with Fairwarning software.

VZV encephalitis

December 17, 2014General paediatrics, Infectious disease, Neurologyvzvadmin

VZV encephalitis presentation not different from usual, viz fever, headache, altered consciousness, etc. But can be subacute onset.

The more common neuro manifestation of VZV in young children is post-infectious cerebellitis – usually mild and self limiting, child not unwell but risk of secondary
hydrocephalus in more severe cases.

There is also a well recognized association in childhood between VZV infection and stroke, usually presents after the rash has cleared – typically 3 months [London study, Miravet & Danchaivijitr 2007). Post-VZV thromboembolism also seen rarely eg lower limb DVT.

Encephalitis can present early, even before rash (which may
not be very obvious either). PCR for VZV DNA in the CSF is positive in around a third of patients; VZV specific IgG seen in 90% in CSF. Compare levels to serum as a way
of confirming CNS involvement.

Usually (not for cerebellitis), steroids given eg 60-80 mg of prednisolone daily for 3 to 5 days) is given (Gilden & Kleinschmidt-DeMasters 2000) esp where MRI evidence of vasculitis.
[encephalitis article, source?]

Chickenpox (Varicella Zoster virus)

December 17, 2014General paediatrics, Infectious diseasevzvadmin

A systemic viral illness characterised by widespread vesicles. Like other herpesviruses, it retains the ability to lie dormant in ganglions after initial infection, possibly reactivating in the future as Herpes Zoster (= shingles).

Age and immunodeficiency are the most important predictors of severity, with adults and infants having more severe disease. Cellular rather than humoral immunodeficiency is associated with more severe disease. Nonetheless, most cases of severe disease (and 90% of the deaths) occur in previously healthy people.

Average age is falling, now under 5 (presumably increased day care) cf underdeveloped countries, where average age is older eg teens. Peak incidence March to May.

There are 3 genotypes but they are virtually identical, and only 1 circulates in Europe; it remains theoretically possible, therefore, to get chickenpox twice! But more likely is that one or more episodes were due to another virus eg enterovirus.

Clinical

Starts as small spots, spreading over the trunk within 24 hr. There is usually no prodrome in children, can be 1-2 days in adults. Within 24-48 hours the classic vesicles, followed by pustules, develop. Burst to leave a scab which falls off after 4-5 days. There may be several crops of these blisters which follow the same pattern. The classical distribution is on the trunk but lesions can be widespread and can affect the mouth, the eyes and the urethra.

Onset of the rash is often associated with fever and other systemic symptoms. The disease can however be subclinical (or at least unrecognized) in some cases. Secondary cases in a household on the other hand tend to be more severe, probably due to increased viral load.

In the immunocompromised, there may be a prodrome ie symptoms at the time of initial viraemia, around day 7 of the incubation period. The classic prodrome is of abdominal symptoms and/or hyponatraemia, and can precede the classic vesicular rash by several days (up to 3 weeks). Abdominal manifestations include severe pain, diarrhoea, or vomiting which are explained by hepatitis, gastritis, oesophagitis, pancreatitis, and paralytic ileus owing to viral spreading from the posterior nerve roots that supply abdominal organs. The mechanism of hyponatraemia is presumed to be SIADH.

See Congenital varicella for infection in pregnancy and neonatal period.

Diagnosis

Usually clinical. If in doubt, PCR and culture can be done on skin scrapings (particularly from the base of lesions, but potentially also from the crust) or from other sites eg CSF.

IgM can be positive from as early as 1-2 days after appearance of the rash. But serology is not 100% sensitive. Antibodies persist lifelong and are useful for indicating previous exposure and hence immunity; they may however be passed on passively through transfusion. Not very good for confirming response to vaccination, as levels are 10 times lower than after disease.

Complications of varicella occur in the immunocompetent, as well as the immunocompromised :

Bacterial superinfection of the rash, potentially cellulitis, even necrotizing fasciitis. Streptococcal infections are more common after chickenpox, and not just in the skin.
Pneumonitis
Meningoencephalitis – usually after onset of rash but may present before. High mortality.
Hepatitis
Glomerulonephritis
Haemorrhagic disease (purpura fulminans) – with shock, DIC
Post varicella cerebellar ataxia – sudden onset but 2-3 weeks later (immune)

Spread

Incubation period is 10-21 days, typically 14 days. Longer in some situations (eg post ZIG). Infectivity (by aerosol spread) begins up to 48 hours before onset of the rash and persists until all lesions have dried (crusted), usually 1-2 weeks but may be longer in the immunocompromised. In theory transmission from a moist spot can occur but infection is generally by respiratory route.

Infectious or isolation period is until all lesions crusted, or else 5 days after the last spot appears. UK HPA guidance is available on exclusion periods from school or nursery on health grounds. Infectivity can persist in the immunocompromised for up to several weeks. Note also that after VZIG administration, incubation period is prolonged.

Treatment

Chickenpox can be treated with aciclovir or the like. Oral aciclovir in the healthy child reduces fever by 1 day and symptoms by 15-30%; not clear if complications are reduced. NEJM 1991;325(22):1539-44. Not standard practice, however! Adolescents and adults should be offered it.

Oral aciclovir only works if given in first 24 hours; IV most effective if given within 72 hours of onset of the rash.

A variety of topical products are available eg calamine. Antihistamines.

Various studies have found an association between severe soft tissue infection and use of ibuprofen (in children, anyway – rate ratio 4.9 [Br J Clin Pharmacol. 2008 Feb;65(2):203-9. doi: 10.1111/j.1365-2125.2007.02997.x]). Likely to be confounding, but in absence of prospective studies, NICE recommends avoiding non-steroidal anti-inflammatories in varicella [https://cks.nice.org.uk/chickenpox#!scenario].

Prevention

See Vaccines for details on live attenuated vaccine.

Post-exposure prophylaxis (PEP) is required for at risk groups who have had significant exposure. This includes:

pregnant women,
immunodeficient (except those receiving regular normal immunoglobulin),
susceptible neonates exposed up to 7 days after birth (which includes mum developing chickenpox up to 7 days before or after birth), or any neonate (susceptible or otherwise) that require intensive or prolonged special care).

Specific Varicella Zoster Immunoglobulin (VZIG) is effective at reducing the likelihood of disease, and where disease occurs it tends to be less severe. It is given intramuscularly. Should be given within 10 days of exposure. Protection lasts 3 weeks, after which a further dose should be considered if re-exposure occurs and antibody has not persisted. Can prolong period of infectivity though. See Greenbook for more details.

Aciclovir can also be used for PEP.

Varivax vaccine is also licensed for this indication, but of course contraindicated in the type of patient who might need it: 18% of healthy contacts developed varicella compared with 78% who did not get vaccine. Of the vaccine recipients who nonetheless developed varicella, the majority only had mild disease (with less than 50 skin lesions). Evidence only really supports PEP within three days following exposure.

Congenital varicella

December 17, 2014Congenital, Infectious disease, NeonatologyVirus, vzvadmin

If a pregnant woman is infected during the first trimester (risk extends to 20th week), a fetus has a 1% chance of developing widespread scarring, hypoplastic limbs, cataracts and brain lesions (congenital varicella). Affected infants have a poor neurodevelopmental outlook.

Risk of neonatal VZV (severe, disseminated disease in newborn) if chickenpox is contracted by the mother 4 days before birth, to 2 days after (risk 20%). Before that, good chance that maternal immune response will protect baby, after that the dose of infection transmitted to the baby via the umbilical cord is likely to be small (although will still be exposed to droplets).

See Maternal for advice on varicella exposure in pregnancy and in neonates.

Varicella Zoster Virus vaccine

December 16, 2014General paediatrics, Immunology, Infectious diseasevaccine, vzvadmin

Following varicella vaccine introduction in Australia, coverage of greater than 80% at 2 years of age was achieved, with varicella hospitalizations reduced by almost 70%. 40% of hospitalized children were immunocompromised. Of hospitalized children age-eligible for varicella vaccine, 80% were unimmunized, including all cases (3) requiring intensive care. No deaths.

[Pediatric Infectious Disease Journal. , 17 December 2012]

Eczema

November 12, 2014Dermatology, General paediatrics, Immunologypractical, Steroidsadmin

NICE (CG57, 2007) diagnostic criteria: itchy skin plus 3+ :

visible flexural dermatitis (or visible dermatitis on the cheeks and/or extensor areas in children <18/12) – can look like dirt where darkened esp neck
personal history of flexural dermatitis (or dermatitis on the cheeks and/or extensor areas in children <18/12)
personal history of dry skin (xerosis) in the last 12 months
personal history of asthma or allergic rhinitis (or history of atopic disease in a first-degree relative of children aged <4yr)
onset of signs and symptoms under the age of 2 years (do not use this criterion in children under 4 years).

On examination, may also see lichenification (increased skin markings due to chronic rubbing), excoriations caused by scratching, and hyperlinear palms (look at thenar eminence).

Babies tend to get eczema on the face. Older infants get it on extensor surfaces. From walking age, antecubital and popliteal fossae. In children of Asian, black Caribbean and black African ethnic groups, extensor surfaces tend to be affected rather than flexures, and discoid or follicular patterns may be more common.

Severe (NICE criteria): widespread areas of dry skin, incessant itching, redness (with or without excoriation, extensive skin thickening, bleeding, oozing, cracking and alteration of pigmentation).

Cf Moderate: frequent itching, redness (with or without excoriation and localised skin thickening)

Risk factors

Eczema (or atopic dermatitis, same thing) associated with parental allergies (OR 1.94), parent-reported infection after birth (OR 1.45), parent-reported jaundice (OR 1.27). Being a migrant (OR 0.63) and keeping a dog (OR 0.78) are protective. Prenatal probiotics did not reduce risk (Lactobacillus rhamnosus GG, LGG). Respiratory viral infections in pregnancy associated with wheezing illness in infants (mothers with asthma) and eczema. [Australia, Ped All Imm 2014:25:151]. Febrile and gynae infections in pregnancy associated with eczema in child (Italy, Ped All Imm 2014:25:159]. Children of Japanese mothers with higher anxiety scores during pregnancy were more likely to be affected by eczema!

Immunology

Many roads lead to Rome! Can be seen as combination of impaired innate and distorted adaptive immunity, interacting in framework of cutaneous immune system with suboptimal barrier function. Epidermal barrier can be genetically disturbed, but gene expression (eg of filaggrin) is also influenced by microenvironment eg Th2 cells. Some genetic structural abnormalities can be seen to induce nonspecific inflammatory reaction (eg SPINK5 defects). And almost certainly, epigenetic regulation will affect gene expression in both keratinocytes and immune cells.

Facilitated antigen presentation mediated by IgE bound to high affinity IgE receptor FcEpsilonRI, on dendritic cells, is an important part of it. Vitamin D plays a key roll, stimulates Toll like receptors, increasing pro-inflammatory cytokines but studies equivocal whether low (or indeed high) levels increase risk.

Eczema can exist without IgE against aeroallergens and food allergens, so is primarily a non IgE mediated disease, although both IgE and non-IgE hypersensitivity can co-exist, particularly in babies.

Still not understood how eczema and Staph aureus interact. Colonization appears to amplify local inflammatory reaction, but also increases IgE against a large spectrum of other things (possibly including self proteins). It is as if there is something specifically different about how immune cells handle Staph. Toll like receptors esp TLR-2 recognize Staph aureus cell wall. Overgrowth of staph during flares is associated (preceded?) by loss of diversity of skin microbiome.

Unmasking of the HSV entry receptor Nectin-1 in adherence junctions, among other things, contribute to eczema herpeticum. See Flares, below.

Atopy patch test (using intact protein allergens) can prove type IV reactions. Correlates with oral food challenge. Hyper IgE syndromes are important differential for AD, STAT3 mutations, skin (and other) abscesses, recurrent pneumonia (and pneumatocoele), connective tissue and skeletal abnormalities, mucocutaneous candidiasis. DOCK8 type Hyper IgE syndrome show sensitization predominantly against food allergens, which is a clue (STAT3 and AD show predominantly sensitization to aeroallergens).

Normal looking skin is not immunobiologically normal! Invisible inflammation, hence still needs treatment. Pimecrolimus and betamethasone both normalize expression of filaggrin, the later is perhaps a better anti-inflammatory but steroids reduce expression of enzymes for lipid and protein synthesis, so may impair skin barrier restoration.

Triggers

Stress, humidity, extremes of temperature can cause flares of atopic eczema. But what causes a flare in some can apparently help in others! Any factors causing flares should be avoided where possible.

UVB causes sunburn. UVA (longer wavelength) more relevant to photosensitivity reactions. Both cause long term skin damage. EU commission recommends UVA protection making up at least 1/3 of total suncream SPF. A few available on prescription for photodermatosis (incl vitiligo) but not for eczema per se. Lipscreen (Uvistat) available for chronic/rec HSV labialis. Sunsense is lotion.

Avoid wool in contact with skin. No evidence that bio detergent or fabric softener a major problem. Avoiding excessive product and not overloading machine (so disperses fully) prob more important.

Management

See eczema management.

Parental support

National eczema society, British Association of Dermatologists, Eczema Outreach Scotland.

Scombroid Poisoning

November 7, 2014Allergy, Immunologyfood, toxinadmin

Differential diagnosis to allergy. Basically Histamine poisoning, rather than release of endogenous stores! Mild examples are not uncommon but severe cases rare. Clue is that several people who eat the same seafood meal fall ill with similar allergic symptoms!

Histamine and other amines are produced by bacteria from certain amino acids (can occur during production eg Swiss cheese or by spoilage). Particularly affects fish of the Scombridae family (viz tuna, mahi mahi, bluefish, sardines, mackerel, amberjack, and abalone) but can be any food containing the right amino acids and subject to the right bacterial enzymes.

Initial symptoms, not surprisingly, include tingling or burning sensations in the mouth, rash, itching. Headaches are frequently seen. May progress to nausea, vomiting and diarrhoea.

Depending on the distribution of the histamine in the food, not everyone at a meal may be affected. Cooking or freezing do not reduce the toxic effect. Contamination is not apparently detectable by the eye or nose.

Differential – see episodic autonomic symptoms.

Fish/shellfish/mollusc allergy

November 7, 2014Allergy, Immunologyfish, foodadmin

Seafood as a term includes fish and shellfish. Shellfish isn’t the best term as it actually includes things without shells (octopus/squid, for example). Food labelling legislation refers to crustaceans (jointed things eg prawns/shrimp) and molluscs (which sometimes have shells, eg mussels/clams) but not well understood terms publicly.

But allergy to one does not imply allergy to the other! In fact, shellfish allergy is linked strongly to house dust mite allergy rather than fish, probably since they are all invetebrates with the same sort of Tropomysins. Co-sensitivity is however relatively common (20-40% of fish allergic also allergic to shellfish) so must simply be atopic disposition!

Chordata (finned fish) subdivide roughly into bottom feeders, mackerel/tuna [perciformes] group, salmon/trout [salmoniformes] group and the rest (so called “bony fish”) but this doesn’t mean much in allergy terms. The best studied allergen Gad c1, found in cod, is a parvalbumin. These are found in muscle, esp slow twitch white flesh (cf dark muscled, fast fish eg tuna, swordfish). At least 50% of those with allergy to one type of fish will be allergic to another; there are no good predictors for this. Cod allergy typically means allergy to herring, plaice and mackerel but not great published evidence. Anaphylaxis UK and Allergy UK do not discuss cross reactivity at all (risk of cross contamination, of course). “White fish” is used as a grouping but doesn’t really have much biological meaning (and confusing because there is a N American white fleshed fish called whitefish…).

The rate of cross reactivity between different kinds of shellfish/molluscs is high since there is less variation in tropomysins.

Crustaceans includes crab, lobster, prawn, shrimp, langoustine. Most commonly eaten as prawn crackers or scampi. Molluscs include oysters, clams, mussels, squid, octopus. Allergy to these is more common in countries where these are commonly eaten viz Spain, Japan – which is not surprising when you think about it, although typically surprising to the people who live there.

Sneaky places you find seafood:

Soups eg bouillabaisse
Pate
Seafood “crab” sticks – usually fish, not crab!
Worcestershire sauce (anchovy)
Pizza (anchovy)
Caesar salad (anchovy)

Reactions to seafood may not be allergic:

Anisakis is fish parasite, worldwide distribution, with a range of different allergens. Larvae can cause immediate allergic response, but infection can also produce inflammatory symptoms of varying kinds, depending on where in the digestive tract the larvae are deposited.
Scombroid toxicity esp associated with salmon, tuna, mackerel. Besides flushing, vomiting and wheezing, there can be severe headache and dizziness. Onset is minutes to hours.
Ciguatoxin poisoning associated with reef fish eg sea bass, snapper. Onset is slower – 30 minutes to hours, besides cramps, D+V there can be myalgia and paraesthesiae.
Shellfish (mostly bivalves) can be the source of a range of toxins with effects as diverse as paraesthesia, myalgia, ataxia, even seizures. Mostly D+V though.

Cod IgE >20 gives 95% PPV, other cut offs lacking. IgE>8 (>5 for salmon) predicts objective symptoms and non tolerance (cf partial tolerance).

Given importance of omega 3, unnecessary restriction should be avoided – Canning fish reduces immunogenicity, challenge? Interestingly, many fish allergens seem to get MORE allergenic with heating, not less – Gad c 1 is known to become airborne in steam without denaturing! Various cases of fatal anaphylaxis simply to inhaling vapour of frying fish (aerosolised proteins, NOT smell, that causes reaction).

[JACI 2017 letter – small numbers though]

Food additives and allergy

November 5, 2014Allergy, Dermatology, Immunologyadmin

Sorbic acid used as preservative. Very low level of toxicity, as rapidly metabolized (a fatty acid). A few reports of contact dermatitis and pseudo-allergy only.

Similarly with Tartrazine, MSG, Benzoate – in most children, there is very limited evidence for any role of food additives in causing non-allergic food hypersensitivity. Reactions may be more common in children with chronic urticaria and angioedema. While other symptoms including migraine, gastrointestinal disturbances and arthralgia have been attributed to food additives, there are no reproducible and consistent data from DBPC studies to support this.

Natural additives eg Annatto can also cause problems in some patients!

Sulphites (sulfites, eg sodium metabisulfite) and natural salicylates may cause skin (usually contact dermatitis, but can be angioedema), GI, respiratory problems (even anaphylaxis) but these are best termed adverse reactions as they have a pharmacological basis. Patch and IgE test available, however. Very common in our food – in fresh foods to control browning, soft drinks, dried foods (as preservative), wine and beer. Yet very rare in childhood and therefore hard to spot. Also seen in in anaesthetic solutions, antibiotics, adrenaline (!!!), cosmetics. Sulphites can have effects when used topically, orally or parenterally – mostly seen in those with asthma. Can be acute or chronic. Given the problem with using adrenaline, may need to be treated with steroids, antihistamines, bronchodilators instead! [Clinical & Experimental Allergy. 39(11):1643-51, 2009 PMID 19775253]

Salicylates are a large group of assorted foods and other things that can cause problems including anaphylaxis. Natural salicylates are generally acetylated so no need to automatically avoid them if intolerance to aspirin/NSAIDs.

[PJ Turner, J Paeds Child health 2010]

Hirschsprung’s disease

October 29, 2014Gastro, General paediatrics, Neonatology, Surgicaladmin

Congenital absence of ganglion cells in distal bowel, beginning at the internal sphincter and extending proximally. Fails to relax, hence functional obstruction. Mostly present as neonates, with delayed meconium and abdo distension, else mostly under 2yr with intractable constipation, failure to thrive, repeated vomiting. Rarely soiling! 1/3 of these later presenting patients present with enterocolitis, with fever, shock, bloody stools. Probably due to abnormal colonic mucosa, a problem that seems to persist even after surgery.

Some series describe patients without symptoms in neonatal/infancy period!

1 in 5000 births. 4:1 males:females. FH 30%. Assoc with downs, cong deafness.

Empty rectum on AXR (ie gas pattern disappears)! [insert axr] May be forceful expulsion at PR (which may also spoil diagnostic value of barium or manometry studies). Barium enema can be useful but high false neg in first 3/12, and need to avoid PR examination and enemas for preceding 48hr else distal narrowing lost.

Treatment – resection of affected segment. Postop do well, risk of anal hypertonicity (enterocoitis), stricture.

Scottishpaeds

General paediatrics with a Scottish flavour