“Medicine inhabits an inexact territory with terrifying hazards, and the best way to avoid them is to demand honesty from everyone. The first truth that we must accept is that human beings are not perfect… We must lose the fear that we’ll be blamed if [our patients] find fault” (Margaret Mccartney, BMJ)
The discipline of medicine concerns the manipulation of knowledge under uncertainty (Siddharta Mukherjee).
Essential investigation in sepsis, particularly where unusual organisms or deep seated infection eg endocarditis.
The volume of the sample is important. Small volumes have higher false negative rate, and are slower to become positive – 6ml superior to 2ml [j Peds 1996].
Traditionally, thought to be most effective when done at time of pyrexia, but there is little evidence for this. In a study of 1,436 adult patients with bacteremia and fungemia, the likelihood of documenting bloodstream infections was not significantly enhanced by collecting blood specimens for culture at the time that patients experienced temperature spikes. Nor was there any benefit for any subgroup eg patient age, gender, white blood cell count and specific cause of bacteremia. [J Clin Microbiol. 2008 Apr; 46(4): 1381–1385. doi: 10.1128/JCM.02033-07]
Also traditionally, considered negative at 48 hours. Canadian study of 98 positive blood cultures in babies up to 90 days of age found 96% of true pathogenic cultures were positive at 24 hours, with 100% positive at 36 hours. Mean time to positivity was 14.4 hours in pathogenic bacteria and 23.2 hours in contaminants. [DOI: 10.1093/jpids/piv078]
US study of 256 non-critically ill babies up to 60 days of age found median time to positive blood cultures of 16.6 hours for pathogens cf 25.1 for contaminants, for CSF cultures 14 hours for pathogens cf 40 for contaminants. 82% of pathogens positive within 24hrs for both blood and CSF [can’t see figures for 36/48hrs yet, full text embargoed?][Hosp Peds 2020]
Another US study of 392 cases (outside of PICU) found 96% of pathogens positive by 36hrs (95% CI 95-98), and 99% at 48hrs. But not clear how many of these would have been well enough to go home at 36 hours. Estimated that observation >36hrs would identify 1 bacteraemic infant for 1250-2778 infants [Biondi, JAMAped2014]. Note that there were significant differences between organisms (E coli faster, staph slower), and between sites (sample volumes? time to inoculation?). No correlation with degree of fever, interestingly.
Current Western Australian guideline, FeBRILe3, appears to be safe.
Canadians have published a new position statement too.
In a tertiary neonatal unit, 72 hours was considered necessary. [ADC Fetal & Neonatal 2001]
The clinical criteria for a unilateral lambdoid synostosis consist of an ipsilateral occipital flattening, a depressed ipsilateral ear lobe (inferior movement) and a parallelogram-like shape in the posterior view. All three of these signs were present in the eight synostotic infants. Furthermore, all children had developed a compensatory contralateral parietooccipital bulging that led to a slanted tree top-like shape of the head at follow-up. Normal posterior view (ie ears level) and anterior movement of the ear excludes LS [but photo looks like ipsi anterior movement in LS – is it contralat in PP??? No mention of anterior bossing, not obvious in photo].
German study – all LS cases obvious clinically. Where positional plagiocephaly was doubted, USS demonstrated patent sutures.
[Arch Dis Child 2015;100:152-157 doi:10.1136/archdischild-2014-305944]
Measure the oblique diameter left (ODL) and oblique diameter right (ODR) lines are drawn from points located 40° either side of the antero-posterior (AP) line. 40° is typically where deformation most notable. Express as difference (the Oblique diameter difference (ODD) = ODL−ODR) or else ratio between the ODL and the ODR (oblique diameter difference index, or ODDI).
[European Journal of Pediatrics March 2006, Volume 165, Issue 3, pp 149-157]
Dutch RCT of 6 months of helmet therapy (n=84 infants aged 5 to 6 months with moderate to severe skull deformation, exclusions were prems, muscular torticollis, craniosynostosis, or dysmorphic features). Full recovery was achieved in 10 of 39 (26%) participants in the helmet therapy group and 9 of 40 (23%) participants in the natural course group (odds ratio 1.2, 95% confidence interval 0.4 to 3.3, P=0.74). All parents reported one or more side effects.
[van Wijk RM BMJ 2014; 348 (); g2741]
Some evidence for bedding pillows (but SUDI risk?) and stretching exercises.
2018 classification (ISVVA.org) – rather functional and lacking in poetry!
Basically benign tumours, involving blood vessels. Seen in 12% of all infants – more common in girls, whites, premature infants, twins and are babies born to mothers of higher maternal age! Mostly seen in head and neck region, including the face, but can be anywhere.
Tumours distinguished from malformations.
Cutaneous/mucosal haemangiomata usually develop after birth, appearing in the first 8 weeks of life. They then develop and grow for 6-12 months, often resembling a strawberry. Most then start to reduce and fade gradually, although it can take up to 9 years. Often there will be complete disappearance with no cosmetic defect, but there may well be scarring, telangiectasia, or loose fibro-fatty tissue.
Can be further differentiated by depth (superficial tend to be raised and bright red, deep are generally darker red or even purple/blue, they can also be mixed) and extent/pattern (focal or segmental).
Typically they are in the skin and soft tissues, but can sometimes affect the liver or airways. Associated with GLUT-1 positive staining on biopsy.
Much less common. Present at birth and do not progress, although they may grow proportionally with child. Oval or round, plaques or exophytic. Some rapidly involute during the first year of life but otherwise they are permanent.
Grow slowly compared with vascular tumours. Usually present at birth but perhaps inconspicuous until child grows. Can involve arteries, veins, lymphatics in various combinations.
Capillary malformations most common – dilated capillaries, classic port wine stain (naevus flammeus). Darken over time, do not regress. Can be associated with bone or soft tissue overgrowth. Multiple can be associated with underlying AVM!
Nevus simplex is the classic “stork bite” at the nape, eyelid or forehead at birth. Lighter, regress.
Venous malformation more ill defined, bluish, easily compressible. Multifocal tend to be autosomal dominant. Some syndromes eg Blue rubber bleb naevus syndrome (widespread, including palms/soles).
Lympoedema and cystic hygroma are the lymphatic versions.
Leadership is not the same as management: yes, it’s about people and systems and getting things done. But it’s more about inspiration, long term goal setting, encouraging people in their own journeys.
Can all be a bit alpha and masculine. Yet lots of evidence that a compassionate style is more effective. Study by Jonathan Haidt (New York University) shows that if employees are moved by the compassion or kindness of their leaders (a state he terms elevation), the more loyal they become to him or her, even if it isn’t directed at them personally.
We are especially sensitive to signs of trustworthiness in our leaders, and react strongly to “arsehole” behaviour.
Not only does an angry response erode loyalty and trust, it also inhibits creativity by jacking up stress levels. Positions of power tend to lower our natural inclination for empathy, so it is particularly important as a leader to be self aware, and actively practice seeing situations form their employee’s perspective.
Key challenges – junior doctor training, MCNs, HEAT targets, centralization vs local demand. Opportunities: improvement, efficiency.
Circle of influence (Steven Covey) – small subset of circle of concerns. Note potential for stress and disillusionment in face of concerns, at time of need for motivation and creativity! Always potential for extending circle of influence…
SWOT analysis: strengths, weaknesses, opportunities, threats. Build on strengths, mitigates weaknesses, capitalize on opportunities, tackle threats head on.
To maintain trust and confidence – stay in touch, know your people. Have a common platform rather than being seen as separate.
Transactional vs transformational styles:
Vision – relevant, strategically worthwhile over years, concordant – should stretch capabilities and self-image
Determines Mission: standards and values
Then in turn Goals (organisational), Strategies, Action plans
Having the broader goals and strategies helps services align, and allows stepwise change within a comfort zone rather than radical revolution with panic
SMART Plus objective – specific, measurable etc Plus clarification about why it’s important, acknowledgement and recognition.
Barriers should be flagged up as next steps – need to keep a “wildly important goal” (WIG) on the agenda of each meeting to maintain perspective.
Deciding how to distribute health care costs may include looking backwards at what behaviours have contributed to a condition (eg tattoo removal may not be publicly funded, but removal of a disfiguring skin lesion where suffering is equivalent is), or may look forward to how behaviour might affect the effectiveness of a treatment (eg liver transplant with continued alcohol excess). Sometimes looking forward and looking backwards have the same outcome, but not necessarily.
There are a number of arguments against these attitudes:
The liberal egalitarian response is to hold individuals responsible for their choice, but not for the consequences of their choice. The egalitarian view is that everyone should have equal opportunities, regardles of their natural or social advantages/disadvantages at birth. Of course, it can often be debated whether “choice” is ever truly distinct and independent of circumstance! The liberal view is that there should be no formal or informal barriers (although not necessarily compensation for the disadvantaged).
So it would be appropriate to tax smokers an amount related to the increased health costs of smoking. It would not be fair to tax some smokers more than others, even if the costs of their treatment might be more – it is the choice that matters. This avoids all the objections above, apart from the moralistic one: but at least decisions on lifestyle taxes are made democratically, not by health care providers.
Does not solve the problem of whether behaviours can truly be considered a choice, when they are often predictable based on socio-economic factors. Plus, not all types of behaviour can be taxed – physical inactivity? Poor health care seeking behaviour? Unsafe sex?
Cappelen and Norheim, J Med Ethics 2005;31:476–480. doi: 10.1136/jme.2004.010421
Traditional M&M (mortality and morbidity) meetings – Many errors are not reviewed, and the key protagonists often not present when a case is being discussed; fail to engage affected families. This lack of transparency in the context of the Francis report is at odds with our duty of candour to patients when things go wrong.
Much energy is spent in the NHS concluding whether errors, adverse incidents and deaths are ‘avoidable’ or ‘preventable’.
‘Avoidability’ is an arbitrary conclusion – what matters, surely, is the care that the child received. Professional analysis of the care given reassures parents that their child’s life is of primary importance, and may provide some comfort that their experience will benefit other children.
Root cause analysis (RCA) tracks the origins of an adverse event back to find causes – too simplistic?
cf ‘Safety-II’ approach – focuses on understanding how things usually go right, and only then exploring why things occasionally go wrong. Rare serious events, although easy to identify, often have complex aetiology, and factors may be difficult to modify. In contrast, “normal” behaviour may be easier to understand and to influence.
Parents’ own questions should inform professional discussion. Analysis should go beyond identifying what the child died from, to considering why a child died of that condition, in that place, at that time.
“The investigation of medical error, adverse events and child mortality each requires a distinct approach that revolves around a continuous cycle of reporting, professional scrutiny and follow-through of SMART actions. These processes should separately feed into a properly formatted clinical governance meeting, the purpose of which is to provide assurance to hospital boards and other regulatory bodies that there exists coordinated oversight of risk management, clinical effectiveness, audit and patient experience.”
[James Fraser, Bristol – Arch Dis Child doi:10.1136/archdischild-2015-309536 ]
Swelling, usually acute, non-pitting. May be erythema too. Typically affects face, especially lips, tongue, eyes, but can be limbs, even internal!
Usually related to urticaria (wheals). As with urticaria, can be allergy – clue is consistent trigger, pattern of recurrent episodes – but can have other causes.
Angioedema without urticaria – consider hereditary or drugs, especially NSAIDs and ACE inhibitors.
Online survey of 400 people aged 50-70, only 2.6% of people could explain what overdiagnosis means.
Definition: when a disease is detected that would not cause any harm during the lifetime of the patient.
Without better public understanding, difficult for them to make informed decisions about their health.
Ghanouni et al. A survey of public definitions of the term ‘overdiagnosis’ in the United Kingdom. BMJ Open 7 April 2016.
Presents with anything from subtle anorexia, fatigue, rashes, abdo/joint pain to acute liver failure. Jaundice does not relate to degree of histological fibrosis.
Check Prothrombin time, glucose, ammonia, lactate to monitor liver disease. Response to Vitamin K at 8 hours is prognostic, so refer to specialist centre if poor.
Type 1 (60%) is ANA/SMA (=small muscle) positive, usually presents as a viral hepatitis ie jaundice, raised transaminases, but can present insidiously, even with established portal hypertension, or as acute on chronic. Type 2 is LKM1 (liver/kidney/microsomal) positive, similar clinical presentation but probably more jaundice and cirrhosis, less impairment in synthesis. Seronegative hepatitis has been described in up to 20%. Antibodies to soluble liver antigen described. Quite common to see pANCA, too (not sensitive or specific). The role of these autoantibodies in disease is unclear!
As with other autoimmune diseases, strong association with HLA types. DR4 associated with less severe disease, lower rate of relapse (but older presentation). Has been reported in association with immune disorders eg autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED).
Alkaline phosphatase rarely exceeds 4x normal and generally remains less than 2x normal. Raised immunoglobulins are a clue, with a selective increase in IgG (up to 3x higher than the upper level of normal) seen in 75-85% (kids and acute less likely). But not specific – also seen in Wilson’s disease; other causes should be excluded too. Interface hepatitis on biopsy, with sharp differentiation between inflammatory zone and normal liver tissue. Findings don’t always match biochemistry. Nonetheless, diagnosis is ultimately clinical! Diagnostic scoring system available. Differential also includes chronic hepatitis C and drug induced liver injury.
Treat with steroids, azathioprine (remember TPMT polymorphisms) when improving, else mycophenylate. 85% achieve remission, biochemically within 6-12 weeks, histologically 6-12 months. More than double normal enzymes is an indication for treatment, else bridging or multilobular necrosis. Progressive fibrosis is associated with liver inflammation, and poor response to treatment (inability to suppress inflammation within 12 months) is associated with progression to cirrhosis (54%) and transplantation (15%). But fibrosis can be stopped or slowed in the majority, and even cirrhosis can regress with treatment. Aim is normal transaminases, immunoglobulins, histology – biopsy is still gold standard for assessing fibrosis.
Unlikely to outgrow. Some trials of withdrawing treatment after remission, only a minority manage more than a year without relapse. Azathioprine 2mg/kg daily effective. Ciclosporin and MMF are second line agents. Hepatobiliary cancers and lymphoma risk is increased and should be screened for.
Transplant may be necessary eg severe acute presentation with poor response to treatment. Results are good, recurrence is rare but well described.
Can be features of other disorders eg primary biliary cirrhosis, primary sclerosing cholangitis. Biliary changes are commonly seen in autoimmune hepatitis in any case.
Can be part of polyendocrine syndrome type 1.
[Heneghan, Lancet 2013; 382: 1433–44 http://dx.doi.org/10.1016/S0140-6736(12)62163-1]
See also PMID 22495399