Category Archives: General paediatrics

Necrotizing fasciitis

General paediatrics, Infectious disease

Life threatening infective necrosis of superficial tissue fascia, often more extensive than would be suspected by appearance of overlying skin.  Can start in previously normal skin, with an insignificant entry site!

Group A streptococcus mostly, but can be polymicrobial especially in immunocompromised.

The top three early presenting clinical features are swelling, pain and erythema but these are entirely non-specific, so initial misdiagnosis common (almost three-quarters of patients in 1 review). More specific features are:

  • pain out of proportion to the physical findings;
  • failure to improve despite broad-spectrum antibiotics;
  • presence of bullae in the skin; and gas in the soft tissue on plain X-ray.

Other possible characteristics described:

  • tense oedema extending beyond margin of erythema
  • loss of sensation
  • LRINEC score in adults based on lab criteria (high glucose, high creatinine, high CRP, high WCC, low sodium, low Hb) has 76% sensitivity, NPV 88.1%.

Early surgical exploration is the best approach in the uncertain case; and early surgical debridement is key to control. IVIG may be of benefit.

Notifiable in Scotland.

Group A Streptococcus

General paediatrics, Infectious disease

Various haemolytic groups, GAS is beta haemolytic.  Other groups of beta haemolytic identified by Rebecca Lancefield.

  • Beta haemolytic Group B is seen in the genitourinary tract of women and is an important pathogen in neonates.
  • Groups C and G both cause invasive disease, both have M proteins, more common throat carriage in developing countries. Maybe also responsible for acute rheumatic fever.
  • Alpha-haemolytic (strep viridans) are associated with line infections and endocarditis.
  • Gamma-haemolytic include enterococci, of which faecium is usually resistant to amoxicillin but faecalis is usually sensitive. Of note, some enterococci are now resistant to vancomycin (VRE).

Exercise induced anaphylaxis

Allergy, General paediatrics, Immunology

Rare but well recognized allergic condition, about half related to a food trigger.

Anaphylaxis can occur with any degree of exercise, does not need to be extreme, but typically follows submaximal exercise pretty quickly.  Deaths are fortunately rare.

Possible mechanisms:

  • Exercise induced increase in gastric permeability
  • increased tissue transglutaminase activity in gut
  • Exercise induced blood flow redistribution
  • Mast cell heterogeneity
  • Basophil trigger by increased plasma osmolality
  • Mast cell trigger by acidosis

All sound plausible but little evidence!

Co-factors play a role in many cases eg alcohol (but unpredictable), NSAIDs (85% will have reduced threshold, and severity increases too), infection, heat/cold – some evidence too for menstruation, anti-acids, stress, sleep deprivation (as in anaphylaxis).  Co-factors also multiply risk of reaction.

Food triggers

Mostly wheat, including hidden sources eg soap, shampoo, cream.

Diagnosis

SPT useful, also IgE omega-5-gliadin.  But gold standard is provocation test (and still only 70% sensitive).  No uniform protocol – use same as for exercise induced bronchoconstriction? Do 1 hour after meal with suspected trigger.

Management

Prescribe adrenaline auto-injector.  Avoid exercise 4-6 hours after food intake.  Discuss unplanned exercise!

Turners syndrome

General paediatrics, Genetics

45XO but mosaics occur.

  • Short stature
  • facial naevi, sphinx like bright eyes
  • Webbed neck
  • Puffy hands and feet as baby
  • Cystic hygroma ie soft tissue mass in neck or intra-thoracic
  • Streak ovaries ie infertile
  • middle ear probs
  • learning difficulties – not really, but maths can be a problem

Acute otitis media

Common, General paediatrics, Infectious disease

Probably 60% of infections are mixed viral/bacterial! Pneumococcus, Haemophilus (non capsulated), Moraxella catarrhalis. Group A Strep is characterized by older age, higher local aggressiveness (ie tympanic perforation and mastoiditis) but lower rates of fever and respiratory symptoms.

AOM is associated with dummy use, adenoids/tonsillitis.

60% of placebo treated children were pain free within 24 hours of presentation [Cochrane 2004, PMID 14973951]. Antibiotics do not increase this proportion. At 2-7 days after presentation, antibiotics reduce by a third the number of children who still have pain (only 14% of the original number), giving a NNT of 15. Too few cases of complications eg mastoiditis to be able to comment on whether antibiotics are useful for preventing such complications.NICE clinical knowledge summary gives NNT of 4000 to prevent 1 case of mastoiditis!

Management is therefore primarily good analgesia. No role for decongestants/antihistamines (Cochrane/NICE).

SIGN guideline 66 on AOM in primary care does not recommend routine antibiotics (but if used, Amoxicillin or co-amox for 5 days recommended). SIGN warns that evidence is poor in infants or in severe disease.

NICE clinical knowledge summary states that at initial presentation, pain and fever should be treated with paracetamol or ibuprofen, at maximum doses if necessary.  No benefit from using both (though poor quality evidence).  For most children antibiotics can be delayed until day 4 of illness (mean duration of illness is 4 days).

Eardrops containing analgesia and anaesthetic (phenazone and lidocaine = “Otigo”) work within 10 minutes – they also significantly reduce the number of people who go on to have antibiotics. Not to be used instead, however, and not to be used where perforation/discharge.

Antibiotics should be offered however at presentation to people who are systemically unwell. Depending on severity, antibiotics should be considered where child is under 2yrs with bilateral otitis media, or if there is perforation and/or discharge in the ear canal.  This advice is also reflected in the British National Formulary for children.

Delaying antibiotics certainly reduces prescriptions, metanalysis of 4 studies in Cochrane did not find any significant difference in pain at 3-7 days, although all but 1 reported some benefit in the immediate treatment group.

A study comparing delayed antibiotics with vs without a prescription (ER based) found high and comparable parental satisfaction rates with both approaches [Chao Peds 2008 PMID 18450878]. In the Cochrane review of delayed antibiotics for URTI, which looked at both adults and children, immediate antibiotics were felt to be more likely to confer modest benefits than delayed antibiotics, with no differences in complication rates between immediate vs delayed antibiotics. Immediate antibiotics had slightly higher levels of patient satisfaction than delayed antibiotics but of marginal clinical significance (92% versus 87%). Concluded that as no evidence for benefit of delayed vs no prescription, best to offer nothing (likely to result in the least antibiotic use). [Cochrane 2011 Delayed antibiotics in URTI, PMID 17636757]

BNFc does suggest antibiotics if:

  • no improvement after 72 hours,
  • clinical deterioration,
  • systemically unwell,
  • at high risk of serious complications (eg in immunosuppression, cystic fibrosis),
  • mastoiditis is present,
  • under 2 years of age with bilateral otitis media.

BNFc also suggests that perforation of the tympanic membrane usually heals spontaneously without treatment; but treat if there is no improvement (eg pain or discharge persists).

Lancet 2006 [pmid 17055944] meta-analysis supports the idea of treating under 2s with bilateral signs (NNT=4), but unlike the BNFc supports treating otorrhoea (NNT=3), as does Cochrane!

Exponential increase in drug resistance and multiresistance. Given how effective placebo is, an effective drug has to do considerably better! Amoxicillin no longer useful (but still recommended by NICE), cefaclor and TMP-SMX not good for borderline resistant pneumococci, azithromycin does not achieve MIC for Hib/Pneumo in ear (although cure rates may not be all that bad…). Co-amox in double dose ie 90 mg/kg/d in 2 divided doses is effective, but increasing resistance.

Treatment leads to higher numbers of resistant species in nasopharynx, esp dually resistant bugs. Azithromycin persists in body for several weeks so is excellent for inducing resistance. Despite overall trend towards reducing antibiotic usage in AOM, most reduction in amoxicillin, with increased prescribing of quinolones and azithromycin. So don’t treat at all unless added features (unless under a year). [Ron Dagan, Beersheva]

Lots of potential complications:

  • Acute mastoiditis = a type of osteomyelitis, with potential for intracranial spread and meningitis/cerebral abscess formation.  Classic signs are erythema, swelling and tenderness behind the ear, with deviation of the pinna.
  • Gradenigo syndrome = intratemporal extension of AOM, causing VI nerve palsy (via apex of petrous temporal bone).
  • Grisel’s syndrome = non-traumatic subluxation of the atlanto-axial joint caused by inflammation of the adjacent tissues.  Clue is trismus and torticollis.

HIV/AIDS

Immunology, Infectious disease

2015 37m worldwide living with HIV, vast majority in Sub-Saharan Africa. Growth is N Africa and Middle East.

20 years since introduction of ART. Still over 1 m deaths per year. Only about a third have access to ART. $250 per child per year.

West Africa seroprevalence only 1-2%, S Africa 25% of sexually active!!!

Treatment for pregnant women actually very good coverage in South and East Africa, much poorer elsewhere.

WHO now recommends treatment for everyone at diagnosis, regardless of CD4, symptoms etc!

In UK 5000 new cases per year, peaked in 2005 (8000), pretty stable now, still mostly migrant associated. Average age of HIV pos child is now 13.6!!! No neonatal cases in Scotland for years.

Majority of known patients have undetectable viral load!

Maternal transmission

Previous advice to breast feed exclusively for 6/12 then stop, now WHO say at least 10-12 months, with no recommendation on stopping. Mixed feeding is now considered acceptable. But this weighs benefits of breast feeding against low rate of transmission of under 2%.

In UK, completely discourage breast feeding, but “not a child protection issue”! Still controversial. Check with your local team!

Transition

Focus now on optimisation of health status through childhood rather than survival! Worry that significant numbers of deaths in survivors of childhood HIV.

Future

Treatment interruptions (not good outcomes in adults)?  Reduction in lab monitoring? Still new (delayed) toxicities eg portal hypertension with DDI. Depot injections!

Treatment

Kaletra + nevirapine first line. Commissioning in England influences treatment recommendations. PENTA 2016 now recommends ART for all children.

Dolutegravir has best results, side effect profile and low interactions (Integrase strand transfer inhibitors (INSTIs)).

Hydrolysed Formulas

Allergy, General paediatrics, Immunology, Nutrition

Alternatives and variations on cow’s milk based formulas:

  • Extensively hydrolysed – protein is broken down so good for cow’s milk protein allergy.  Not very nice tasting!  Can be whey-based eg Pepti (which includes lactose, so more palatable but no good for lactose intolerance), else casein-based eg Nutramigen (lactose free).
  • Partially hydrolysed – better tasting but symptoms may persist if true allergy
  • Anti-reflux “stay down”
  • Soya – good for cow’s milk protein allergy but cross reactivity can occur, plus theoretical phyto-oestrogen effect so avoid if under 6 months.  But the only one you can use if you are vegan or have galactosaemia.

Aptamil Pepti is made by Milupa (which is where GP’s will find it on electronic prescribing system). Not suitable for vegetarians and not Halal!

Some are lactose free, others not. Some have medium chain triglycerides as main fat source, eg Pepti Junior, Pregestimil, Peptisorb.

Nutramigen contains prebiotics – should therefore not be given to preterm babies (theoretical risk of gut translocation), and should be made up at room temperature (so not suitable for prep machines).

For those who require a vegetarian or halal diet, the only suitable extensively hydrolysed infant milk is SMA Althéra. Of the amino acid formulas, all are halal, and Neocate/Alfamino are vegetarian. None are vegan friendly.

SMA Alfamino does not have coconut oil, unlike some of the others.  No evidence that there is sufficient coconut protein in formula to cause an allergic reaction but it often gets accused of suspected reactions.

SUDEP

General paediatrics, Neurology

Definition is sudden unexpected death with or without seizures, after exclusion of status epilepticus, where PM does not identify another cause.  Cases almost exclusively in symptomatic epilepsy.

SPEN response to determination of Sheriff Duff (that SUDEP should be discussed with all patients with epilepsy) –

  • Most data on SUDEP from adults.
  • Risk is unlikely to be predictable at time fo diagnosis so debatable that this is the appropriate time to discuss it.
  • No evidence that increased supervision of sleep is of benefit.  So arguable that discussion will cause unnecessary anxiety.

Berg AT, Shinnar S, Testa FM, Levy SR, Smith SN, Beckerman B. Mortality in childhood-onset epilepsy. Arch Pediatr Adolesc Med. 2004 Dec;158(12):1147-52.

Camfield CS, Camfield PR, Veuglers PJ: Death in children with epilepsy: a population-based study. Lancet 2002;359:1891-1895

Weber P, Bubl R, Blauenstein U, Tillmann BU, Lütschg J. Sudden unexplained death in children with epilepsy: a cohort study with an eighteen-year follow-up.  Acta Paediatr. 2005 May;94(5):564-7.

Living with Epilepsy

General paediatrics, Neurology

Driving

In UK, driving is forbidden after a single epileptic seizure.  If seizure happens after age of 5, permanent ban on driving HGV, passenger service vehicles, racing.

Reinstatement of license depends on seizure free period, and whether seizures have occurred only during sleep or not.  You can reapply if you haven’t had an attack for at least a year.  If you had a seizure because your doctor changed or reduced your anti-epilepsy medicine, you can reapply when:

  • the seizure was more than 6 months ago
  • you’ve been back on your previous medication for 6 months
  • you haven’t had another seizure in that time

If your seizures have only been during sleep, you need at least 12 months seizure free.  If seizures have been when awake as well as in sleep, then must have been 3 years with only seizures in sleep.

If however generalized spike and wave activity on EEG, then reinstatement cannot occur.  DVLA seem to retain the right to decide on individual basis.

Death and Accidents

SUDEP (sudden unexpected death in epilepsy) should be discussed at or around the time of diagnosis.  There is a tendency for professionals to avoid talking about it.  Evidence from review of cases is that deteriorating seizure control in preceding 3-6 months common, as is lack of regular clinic review.

Discuss safety esp bathing, swimming, cycling.

There are numerous devices and tools available.  Unfortunately, no single type of monitor (movement, breathing, ECG, EEG) likely to be sensitive.  But important to have balanced approach – consider risk of events, likelihood of false alarms, and the fact that no device can guarantee that SUDEP will not occur.

The best way to prevent SUDEP is to stop seizures occurring in the first place!

BMJ safety checklist

EpSMon is a patient app.

Another useful source of objective information is the latest Cochrane Review on SUDEP interventions.

Other things to consider:

  • Medical ID cards or Jewellery
  • Seizure alert dogs
  • Seizure alarms – phone app, else local authority may be able to provide, else charities
  • Anti-suffocation pillows
  • protective headgear

In a UK review of epilepsy deaths.  15% related to status epilepticus, 15% SUDEP.  Majority due to co-existing morbidities.  24% considered preventable, usually related to fragmentation of care, support for families in responding to emergencies, and hospital response to acutely unwell child (incl status). [BPS poster]

Contraception

Women taking anticonvulsants and oral contraceptives have 25 times the risk of pill failure as normally expected, due to action of some anticonvulsants (eg phenytoin, phenobarbitone, primidone, carbamazepine, and ethosuximide) on the hepatic microsomal enzyme system, resulting in a dramatic decrease of circulating oestrogens.  Discuss with pharmacist, as complicated!

Contraception also important if potential teratogenic effect esp valproate.

Family Support

Complement deficiency

General paediatrics, Immunology, Infectious disease

Complement cascade can be triggered by classical (antibody binding to antigen, but also directly by C-reactive protein an other substances), lectin (mannan binding lectin, which recognise glycoproteins not typically found in higher order animals) or alternative pathways.  The alternative pathway is the oldest, and constitutes a constant low level autoactivation, at the ready to explode!  Properdin acts here, and may ineract directly with bacteria.

Deficiency can cause susceptibility to infection, but see also hereditary angioedema and atypical HUS.

C3 is the common factor, so deficiency leads to severe infection with encapsulated organisms eg Pneumococcus, Haemophilus, Meningococcus.  Deficiency in terminal and alternative pathways lead almost exclusively to problems with meningococcus!

Properdin

Stabilizes C3/C5 convertase enzymes.  Deficiency (X-linked) associated with fulminant meningococcal infection, especially with unusual types eg W135, Y!  Phagocytosis more important for type B disease? Life time risk for affected individual is about 50%!  Interestingly, mean age of presentation is 14yrs.  Recurrence is actually unusual, presumably due to intact immune memory.

Subtypes characterized by absent, low level and dysfunctional properdin seen.  Standard screening with C3, C4 and CH50 are normal.  Good family history is more important.

Good antibody responses to Men ACWY vaccine.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1905414/

 

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2952982/

 

https://www.ncbi.nlm.nih.gov/pubmed/19758139