Category Archives: General paediatrics

Premature atrial extrasystoles

Cardiology, General paediatrics

PACs for short.  PAC

So you get extra atrial beats, from somewhere in the atria outside the SA node.  The P waves therefore look odd, esp if they fall on top of a T wave.  They can even be upside down if close to the AV node, the depolarisation is therefore in reverse and the PR interval is abnormally short.  You usually do get a QRS after but sometimes it is blocked completely, and sometimes you get a RBBB pattern (RBBB has a longer refractory period).

You usually get a compensatory pause as the SA node is reset.

So the patient may feel a fast, extra beat, then a skip.

Considered normal!  Can be very frequent eg bigeminy (every other beat is a PAC), and you can get runs (“couplets”).  But if you have an abnormal heart already eg WPW, then it may be a trigger for a re-entrant tachycardia.

UK vaccine schedule

General paediatrics, Immunology

Changes depending on availability (and cost) of new vaccines, changes in epidemiology.  And levels of public acceptance!  Recommendations made by JCVI (Joint committee on vaccines and immunization).  Most recent change is introduction of MenB (Bexsero).

  • At, 2, 3 and 4 months, a 5 in 1 vaccine containing diphtheria/tetanus/pertussis with polio and Hib is given (Pediacel).
  • Prevnar (pneumococcal conjugate, PCV-13) is given at 2 and 4 months with a booster at 12-13 months
  • MenC now given at 3 months only (other 2 doses dropped), in between Prevnar, with a booster at MMR time.
  • Oral rotavirus vaccine is now given at 2 and 3 months.
  • Bexsero (MenB) vaccine is given at 2 and 4 months, with booster at 12-13 months.

At 12-13 months, MMR – along with boosters of Hib/MenC (Menitorix), Prevnar and MenB

Annual nasal influenza vaccines are being phased in over next few years, currently all primary school and ages 2-4yrs.  Will eventually be all up to 16.

At 3yrs 4 months- 5yrs, preschool booster – DTP/Polio (Repevax, no Hib) and MMR again.

At 13yrs, BCG has been dropped as a universal vaccine. There is now a booster of MenC, along with Tetanus, diphtheria (low dose) and polio (no pertussis, Revaxis).

Girls between 12 and 14yrs get 2 doses of HPV vaccine, at least 6/12 apart.

Over 65s get scheduled PPV (pneumococcal polysaccharide, once) and annual influenza.

Over 70s get a single Shingles vaccine.

The acellular pertussis vaccine (3 or 5 antigens cf 3000 in whole cell) is associated with less reactions (but less effective and immunity shorter lasting); IPV (injectable) polio vaccine has same efficacy as OPV (oral, live, Sabin, herd immunity), plus no vaccine associated disease.

These newer vaccines have fewer reactions, and do not contain thiomersal. Not that there’s any evidence against mercury, but plan to eliminate it has been in place for several years.

There was also an issue with loss of Hib efficacy when using 3 in 1 DTP for primary immunizations, which is not seen with Pediacel.

No individual boosters for tetanus are available. Choice is between Infanrix (DTaP), Repevax or Revaxis.

Instead of BCG for all adolescents, risk factor approach introduced: BCG will be offered to all infants in health boards with incidence over 40/10 000 (none in Scotland), and to those with parent or grandparent from high incidence area.

Rhinitis QOL

Allergy, General paediatrics, Immunology

You can just use generic SF-36 questionnaire, statistically significant differences between patients and controls were observed in seven of nine dimensions in the SF-36 questionnaire. Or RQLQ score (mini-form also available). (J Allergy Clin Immunol 1997;99:S815-9.) SF-36 particuarly highlights mental adverse effects, RQLQ highlights sleep disturbance.

Quality-of-life parameters measured by the RQLQ questionnaire:

  • Sleep – Lack of a good night’s sleep, Wake during the night, Difficulty getting to sleep
  • Non-hay-fever symptoms – Tiredness, Fatigue, Worn out, Reduced productivity, Poor concentration, Thirst, Headache
  • Practical problems -Need to blow nose repeatedly,Need to rub nose/eyes,Inconvenience of having to carry tissues or handkerchief
  • Nasal symptoms -Stuffy/blocked,Sneezing,Runny,Itchy
  • Eye symptoms – Itchy, Watery,  Swollen,  Sore
  • Emotions – Irritable,  Frustrated,  Impatient or restless, Embarrassed by nose/eye symptoms
  • Activities eg  Bicycling Cooking Dancing Doing home maintenance Doing housework Gardening Eating out Jogging, exercising, or running Attending public events Driving a car Watching TV or a movie Singing Mowing the lawn Playing with pets Doing regular social activities Talking (public speaking) Studying or doing homework Taking a test or quiz Visiting friends or relatives Going for a walk Having sexual intercourse Carrying out activities at work Reading Playing sports

Juniper and Guyatt

 

Bruising

Child protection, Common, General paediatrics

Typical areas of accidental bruising:

Pattern of accidental bruising
Maguire, 2010

Typical pattern of abusive bruising:

Pattern of abusive bruising
Maguire, ADC Ed & Pract 2010

From prospective longitudinal study of children (<6 years):

  • 6.7% of premobile children had at least one bruise (2.2% of babies who could not roll over and 9.8% in those who could)
  • Most common site affected in all groups was below the knees, followed by ‘facial T’ and head in premobile and early mobile.
  • The ears, neck, buttocks, genitalia and hands were rarely bruised (<1%).
  • Gender, season or the level of social deprivation not associated with bruising patterns, although having a sibling increased the mean number of bruises.
  • There was considerable variation in the number of bruises recorded between different children, which increased with developmental stage, and was greater than the variation between numbers of bruises in collections from the same child over time – so some kids do just bruise more than others?

[Arch Dis Child 2014]

Differential

Are you sure it isn’t a Mongolian blue spot? Or capillary haemangioma? Or erythema nodosum?

Cupping in Chinese culture! Dermatitis artefacta?

  • Thrombocytopenia. Note film can show clues – inclusion granules in Chediak-Higashi.
  • Factor deficiency – bleeding from umbilical stump classic for XIII deficiency. Girls can have bleeding problems even if carriers rather than completely factor deficient.
  • Glanzmann’s thrombasthenia – platelet count normal! But severe eg fingertip bruising and bleeding from vaccination sites. Other platelet defects similarly.

History

Haematomas after Vit K at birth or immunisations? Bleeding from umbilical stump or Guthrie test? Dental treatment? Joint swelling or pseudoparalysis that might suggest a haemarthrosis?

Family history? 30% of haemophilias de novo mutations.

Skin/joint hypermobility/elasticity? See Ehlers-Danlos.

Listeria

Congenital, General paediatrics, Infectious disease, Neonatology

An intracellular gram positive rod – not many of those, apart from in probiotic drinks!  Resistant to cold and salt, so particularly a problem in ready to eat foods eg deli meats, hot dogs (unless steaming hot), cheese esp soft (incl blue veined, but excl mozzarella), raw and cooked poultry, ice cream, raw vegetables, raw and smoked fish (unless in shelf stable form). Melons and hummus have been sources in US.  In adults, tends to affect those with underlying health problems.

Infection in pregnancy often undiagnosed. May cause preterm labour or intrauterine death. Infection at birth may be severe, with classic fine papular rash, widespread microabscesses and granulomas, and bacteria visible on gram stain of the meconium. Or infection may be late in onset eg 1-2 weeks, with meningitis (note low counts cf other causes), else endocarditis, osteomyelitis etc.

Preterm meconium staining of the amniotic fluid (MSAF) is a “classic” feature – was observed in 4.3% of infants below 33/40. No maternal or infant listeriosis was identified in any of the 1000 cases. MSAF was associated with prolonged rupture of the membranes and severe (grade 3/4) intraventricular haemorrhage (OR 2), not sepsis or mortality. (Simpsons, Arch Dis Child Fet 2004)

Surveillance study of bacterial meningitis in infants aged <90 days in the UK 2010-11 showed that then usual three bacteria (GBS, E. Coli and Listeria) remained dominant, their frequency varied significantly by month of life. In the first 30 days of life. L. monocytogenes was the third most common bacteria, responsible for 6% of cases. The median age of meningitis due to L. monocytogenes was 13 days (IQR 3–18 days) with the oldest infant being 29 days; Listeria meningitis was therefore not seen beyond the 1st month of life. Of the 11 cases of Listeria meningitis, a good number (although a minority) were preterm and most first became unwell when at home. 2 cases had serious complications but no deaths.

Public Health England have published 24 years data on listeria septicaemia and meningitis. 97% of all cases presented in the first 30 days of life. Bacteraemia is more common but tends to be early onset (<7 days of age) whereas most meningitis were late onset.

It is also prudent to consider the possibility of Listeria infection in older infants (and therefore add amoxicillin) if:

  • Gram-positive rods are seen in the cerebrospinal fluid,
  • if the infant is immunocompromised
  • or if the clinical response to empirical therapy is suboptimal

Treat with high dose amoxicillin/ampicillin. Gentamicin is synergistic but does not penetrate intracellular compartment (or CSF) – can be stopped after a week assuming good clinical improvement. For allergic, TMP-SMX (Septrin) is best alternative! Cephalosporins are useless! Treat for 2 weeks if no meningitis, at least 3 weeks if meningitis, longer if abscesses or heart involvement.

[Okike, Arch Dis Child 2015;100:426-431]

Hepatitis B

Gastro, General paediatrics, Infectious disease

Highly infectious blood borne virus. A single contaminated needle stick injury carries a significant risk of transmission. In endemic areas, significant rates of postnatal infection in children, presumably from minor trauma.

Acute infection develops over 1-6 weeks and can be fulminant. Symptoms are non-specific fever, lethargy, abdo pain.  15% have serum sickness type  symptoms in pre-icteric phase viz fever, arthralgia, urticaria. Jaundice then develops.   Various antigens, which may or may not clear as antibodies produced, used to diagnose, judge stage of infection, and infectivity.

  • HBsAg – shows Acute or chronic hepatitis B infection. Can be negative in acute fulminant disease.
  • Anti HBs (or Anti HBs) – Immunity to hepatitis B, postinfective (only 6% of patients) or with active or passive immunization
  • Anti HBc IgM – first antibody to appear, even before HBsAg.  High titer: acute hepatitis; Low titer: chronic infection
  • Anti HBc IgG – Past exposure to hepatitis B, or maternal antibody crossing placenta in young infants
  • HBeAg – highly infectious.  Without treatment, 85% of children clear HBe.
  • anti HBe – not immune, but low infectivity. Do not develop chronic hepatitis; but low risk of hepatocellular carcinoma persists.

NB there may be an interval following the disappearance of a hep B antigen before its antibody becomes detectable.

For acute hepatitis, no specific treatment is required, just supportive. If fulminant, some people use antivirals, but basically the issue is whether you need to transplant – see below.

Carrier rate (ie chronic infection, = HBsAg pos for 6/12, and hence potential for serious sequelae) is higher in males and greatest in those infected in first 3 years of life.  Also higher in those with mild symptoms viz anicteric with minimal elevation of transaminases.

Some carriers will be inactive with anti-HBe, low levels of DNA (<100 000 copies on PCR) and normal transaminases. Liver disease progresses very slowly if at all, although hepatocellular carcinoma risk is still higher than normal (but much less than in HbeAg positive). Monitor LFTs every 6-12 months.

In active chronic infection, DNA levels are high indicating active replication, transaminases are 2x the upper limit of normal or higher, and biopsy will show stage 4 histological activity or more. HBeAg is usually positive although some mutants will be negative. Adults with chronic hep B have 20% cirrhosis rate after 10 years and 37% after 15 years. Alcohol intake is an independent factor. Hepatocellular Ca rate is about half that.

When to biopsy is tricky, partly because the clinical course is unpredictable but also because treatment is ineffective. Transaminases can be raised transiently, so repeat after 1-3 months. Histological cirrhosis has a poor prognosis. With treatment, 32% of patients with chronic disease clear HBeAg cf 11% of untreated (metanalysis). There is no benefit in patients with normal LFTs.

Do USS and alpha fetoprotein every 2 years as screening for hepatocellular Ca for both inactive carriers and chronically infected.

Other management points –

  • immunisation of household contacts
  • vaccination against hepatitis A (in low prevalence areas)
  • avoiding alcohol
  • safe sexual practices
  • weight reduction
  • Careers advice
  • Immunosuppressive drugs may activate hepatitis B infection. Equally, immunosuppressed children may have hepatitis B infection without serological evidence – so do PCR.

Treatment

For acute, no specific treatment, just supportive. If fulminant, look for co-infections that may have precipitated episode. Main issue is whether transplant required, although American Association for study of liver diseases recommends using antivirals in adults: no great evidence but seem reasonably safe, and reduces risk of re-infection of grafted liver after transplant (pretty inevitable – HBIg post transplant also delays infection but resistant mutants come through). Lamivudine or telbivudine suggested when the anticipated duration of treatment is short; otherwise, entecavir is preferred. Treat until HBsAg cleared or indefinitely in transplants. Interferon is contraindiated.

For chronic infection:

  • Pegylated IFN alpha (5 million units a day or 10 MU thrice SC for 48 weeks) both antiviral and immunomodulatory activity.

Other issues:

  • Co-infection with HCV has a poorer prognosis even though DNA levels may be less; treatment should be considered at 1000+ copies.
  • In decompensated cirrhosis, transplantation is the only definitely effective option. Lamivudine may help (historical controls). IFN increases complications.

Hepatitis A

Gastro, General paediatrics, Infectious disease

Faeco-oral transmission.  Global distribution.

Incubation 4/52, can be asymptomatic in young children. Non-specific fever, malaise, possibly RUQ pain before jaundice appears, usually with rapid relief in symptoms.  Up to 1% however have fulminant disease with hepatic failure.

Post exposure prophylaxis for hepatitis A with hepatitis A vaccine reduces secondary infection rate from 13% to 2.8% (NNT=18).

See also viral hepatitis.

Wilson’s disease

Gastro, General paediatrics, Genetics, Neurology

= hepatolenticular degeneration. Autosomal recessive condition with copper accumulation due to impairment of biliary excretion. Leads to cirrhosis, via a stage indistinguishable from chronic active hepatitis, plus neurological disease. Caused by mutations of the ATP7B gene that codes for a copper transporting ATPase – over 300 mutations known, varying geographically.

Clinical Presentation

Usually presents in late teens but has been described as young as 3yrs. Neurological presentation tends to be older (by 5 years) although they usually have subclinical liver disease. Hepatic disease varies from elevated aminotransferases, through chronic liver disease to fulminant hepatic failure (often with Coombs negative haemolytic anaemia), about 5% of presentations.

Basal ganglia involvement leads to movement disorders viz:

  • Tremor
  • Chorea
  • Parkinsonism
  • Gait disturbances
  • Dysarthria

Other neurological signs are:

  • Psychiatric symptoms
  • Depression
  • Neuroses
  • Personality changes
  • Psychosis

It can also cause:

  • Epilepsy
  • Sunflower cataracts
  • Aminoaciduria
  • Renal stones
  • Osteomalacia with spontaneous fractures

Diagnosis

Can be tricky given multisystem disorder and limited sensitivity/specificity of tests. Heterozygotes may also have borderline results. If typical presentation then diagnosis can be made on basis of:

  • Kayser-Fleischer rings
  • Low serum ceruloplasmin levels (<0.2g/L)
  • Genetic screening of limited utility due to number of known mutations

May require extensive tests of copper metabolism especially with severe hepatic presentation, where up to 50% can have normal ceruloplasmin (an acute phase reactant) eg

  • Non-caeruloplasmin-bound serum copper
  • 24-h urinary copper excretion – can be abnormal in other chronic liver diseases, however. Excretion of >25micromol/24hr after penicillamine is a diagnostic test in children.
  • Liver copper content (>250mcg/g dry weight) – best test when others ambiguous.

In fulminant hepatic failure the following features may suggest diagnosis:

  • Haemolysis (Coombs negative)
  • Alkaline phosphatase surprisingly low viz ALP:Bilirubin ratio of less than 1 has 86% sensitivity and 50% specificity in children

Treatment

  • Diet – chocolate, liver, nuts, mushrooms, and shellfish are high in copper
  • Zinc – reduces copper absorption from gut. Monotherapy is an option for maintenance therapy.
  • Chelation
    • D-penicillamine – but note side effects, and some patients with neurological disease deteriorate on starting treatment
    • Trientine – perhaps less side effects
  • Liver transplantation – curative, except for long-standing neurological disease. Indicated for fulminant hepatic failure.

Monitoring

  • Neurological function
  • Liver function tests
  • 24hr urinary copper excretion (aim for less than 2 micromol/d)
  • Non-ceruloplasmin bound copper of 50-150mcg/L

Liver failure

Gastro, General paediatrics