Paediatric Pearls

This service, for parents who are anxious about their relationship with their baby and/or child under 3, resides within North East London NHS Foundation Trust and serves Redbridge, Waltham Forest, Barking and Dagenham and Havering communities.

The PPIMHS teams are made up of psychiatrists, community mental health practitioners and psychotherapists/psychologists and they accept referrals from Health Visitors, GPs, midwives, Children’s Centres workers or other health professionals.  Click here for their referral form.  They may signpost elsewhere after the initial consultation if appropriate or they will offer the parent/carer and infant/child 9-12 sessions to work on the parent-infant relationship.

Groups particularly at risk of having problems with bonding include families with ex-premature babies who have spent a significant amount of time on the Special Care Baby Unit, those where the baby has feeding issues or is difficult to soothe, those where breastfeeding failed to establish and those where there was a traumatic birth or difficult conception and/or pregnancy.  Many of the parents on their case load have a personal history of disturbed attachments and are keen not to let history repeat itself.  A recent audit showed that 41% of their mothers had some sort of mental health diagnosis which means that 59% did not.  Click here for an information leaflet about their service that you might like to give to your patients.

Mums with postnatal depression or post-partum psychosis should be referred directly to a perinatal psychiatrist rather than PPIMHS.  Parents struggling with a crying baby or fussy toddler but with no bonding issues should be referred to their health visitor.  The PPIMHS team is a tier 3 (specialised) service concentrating primarily on the parent-infant relationship.

Symptoms in the baby that might suggest a bonding problem:

extreme clingy behaviours, fussy, difficult to soothe, abnormal self-soothing behaviours (eg. head-banging, hair-pulling, scratching), excessive sleep problems, extreme feeding problems, lack of verbal and non-verbal communication, stiff or floppy posture, extreme fearfulness or watchfulness, lack of interest in the world, no comfort sought from parents, avoids eye contact with parents, smiles very little.

Symptoms in the parent:

high anxiety and panic about the baby, excessive A and E or GP presentations, feeling frightened of harming the baby, lack of separation between parent and baby, baby never put down, excessive sterilising of bottles and toys, detached feelings about the baby, no pride in their development, anger about baby as if baby intends to upset the parent, feelings of failure as a parent, inability to cope.

There is some evidence around this issue and around maternal stress during pregnancy and the effect of high maternal cortisol levels on the foetus’ developing brain.  I have asked the Waltham Forest PPIMHS psychologists to write a bit about that and correct anything I have written about their service!

Most children who are dehydrated presenting to UK emergency departments can be rehydrated orally. 

• Give 50ml/kg ORS solution over 4hrs, plus ORS solution for maintenance, often and in small amounts (even by syringe or spoon)
• Continue breast feeding
• Consider supplementing with usual fluids (but not fruit juices or carbonated drinks) if a child without red flag symptoms or signs (see http://www.nice.org.uk/CG84) refuses to take sufficient ORS solution.  Don’t give solids.
• Consider giving ORS solution via ng tube if child is unable to take it or continues to vomit (esp. with red flag symptoms/signs)
• Monitor carefully

This is a worked example for a 3 year old child weighing 14kgs who has been assessed as about 5% dehydrated.

Maintenance = 100mls/kg for first 10kgs and 50mls/kg for next 10 kgs = 1000mls + 200mls = 1200mls over 24 hours

Replacement = 5 x 14 x 10 = 700mls over the first 4 hours (extra to maintenance needs)

Therefore the child needs 225mls per hour for the first 4 hours (1200/24 + 700/4), followed by 50mls (1200/24) per hour.

The 225 mls is best given as 18 mls every 5 minutes or 56mls every 15 minutes if vomiting seems to have stopped or if using nasogastric tube.

They should have 5mls/kg = 70mls extra diarolyte (ORS) with each diarrhoeal stool or vomit.

Give parents written information to go home with so they understand that diarrhoea may continue for a few days but this does not matter as long as they are able to get enough fluid in the top end.  The NICE guideline parent information is at http://guidance.nice.org.uk/CG84/PublicInfo/pdf/English.

More background to pertussis with thanks to Dr Rupa Vora

• whooping cough is caused by Bordetella pertussis, a gram negative pleomorphic bacillus. It is spread by aerosol transmission and the bacteria cause damage by attaching to the respiratory cilia
• it occurs in clusters every 2-5 years during the summer months. We currently have an outbreak with the HPA provisionally reporting 665 cases in the first quarter of 2012 (cf. 1040 cases in 2011, 421 in 2010)
• cases have dropped dramatically since pertussis vaccinations have been introduced. Acellular pertussis vaccination is given at 2 and 3 months, followed by a pre-school booster.  However, protection wanes quickly and has virtually disappeared by 12 years old
• incubation period is 3-12 days and children are most infectious in the first 2-3 weeks. They are most likely to present in the second phase of illness at 3-4 weeks
• can present with coryza (1^st stage which lasts a couple of weeks), paroxysms of cough, difficulty feeding and pneumonia. Younger infants (<6months) may not present with the characteristic ‘whoop’. Older children and adults often present with a persistent cough
• complications include chronic cough (“100 day cough”), hypoglycaemia, seizures, encephalopathy and intracranial haemorrhage
• any infant is vulnerable and up to 50% may need hospitalisation.  Especially vulnerable are ex-prems and those with underlying cardiology, respiratory or neurological problems.  
• In England and Wales, whooping cough is statutorily notifiable.  The diagnosis is usually made on clinical grounds without the requirement for laboratory confirmation
• The UK Health Protection Agency advises a 7 day course of erythromycin or clarithromycin (or azithromycin for 3-5 days if under 4 weeks) to reduce spread.  A pernasal swab to confirm or refute B. pertussis as the causative organism can be carried out.  If the cough has been present for more than two weeks and the child is in the community, serum serology can be sent to Colindale.  See table below:

Appropriate laboratory tests for a sporadic case of pertussis reported to HPA on clinical suspicion (with thanks to Dr Maria O’Callaghan): 

 NPA – nasopharyngeal aspirate; PNS – pernasal swab;

RSIL – Respiratory and Systemic Infections Laboratory, Colindale

Useful websites:

HPA: www.hpa.org.uk/Topics/InfectiousDiseases/InfectionsAZ/WhoopingCough/

NHS Choices: www.nhs.uk/Conditions/Whooping-cough/Pages/Introduction.aspx

With apologies for missing March – time management issues….

Functional abdominal pain this month with a link to a handy patient information leaflet, pubertal and growth issues, neonatal spots and some not-for-the-faint-hearted youtube videos on intraosseous line insertion for the ED practitioners amongst you.  Do leave comments!

With thanks to Fionnuala O’Driscoll, Speech and Language Therapist at Wood Street Specialist Children’s Services for the table below:

With thanks to Dr Amy Rogers for unravelling endocrinology for the better understanding of all non-endocrinologists.  All girls under 9 and boys under 8 with signs of puberty should be referred to a paediatrician and you could leave it at that.  But for those who want to know a bit more about it (or check up on what we do about it…) read on!

The British Society of Paediatric Endocrinology and Diabetes (BPSED) is recognised by the Royal College of Paediatrics and Child Health (RCPCH) as the society responsible for this field of paediatric medicine. It is currently chaired by Professor Dattani of Great Ormond Street Hospital and represents the only U.K. society responsible for governing the training of doctors in paediatric endocrinology and diabetes and actively supporting the ongoing training and education of allied healthcare professionals in this specialist area. Lots of resources available at www.bsped.org.uk

The hormones of puberty: Hypothalamic-Pituitary-Gonadal Axis:

Hormones of puberty

(http://www.ohioshaolindo.com/China%27s%20Arts/image016.jpg)

Start of puberty in girls = palpable breast bud (B2)

Start of puberty in boys = testicular vol >3.5ml

Androgens promote other secondary sexual characteristics: smelly feet, acne, body odour and mood swings!

Timing of puberty is dependent on socioeconomic status, nutritional and genetic factors.

In the UK early puberty = <8years girls, <9 years boys. Interpret in conjunction with family history (age of maternal puberty), ethnicity (Afro-Caribbean or mixed race = commoner to have early menarche), BMI (overweight = associated with early puberty), social factors (adoption and lower SEC = early puberty) and past medical events. Approx 10% girls achieve menarche whilst still in primary school.

RED FLAG: ARRESTED PUBERTY = SERIOUS PATHOLOGY

Pubertal development may be:

1)       Concordant (following the normal pattern), i.e. breast buds, pubic hair then menses/increased testicular vol, pubic hair then penile enlargement).

OR

2)       Discordant, e.g. progressive breast enlargement with no pubic hair, or penile enlargement with small testicles. Suggests over activity of sex hormone (oestrogen/testosterone) production in the periphery, i.e. adrenals, gonads (ovaries/testes) or tumours.

Causes of precocious puberty

Central precocious puberty (gonadotrophin dependent)

• Idiopathic = Most common cause in girls (10 times more common than boys). Slowly progressing (breast and pubic hair growth, modest growth spurt, bone age advanced <1yr, few changes on pelvic USS) or more aggressive (height velocity greater, bone age advanced >1 yr). Ovarian and uterine enlargement (>2ml) on USS. Pubertal response to stimulation test. Boys: testicular enlargement, virilisation, pubertal response to stimulation test.

• Tumour = Second most common cause in both sexes (at least half of all boys presenting with central precocious puberty), typically a hypothalamic hamartoma

• Optic glioma (NF)
• Longstanding/severe peripheral secretion
• Abnormal brain (hydrocephalus, septo-optic dysplasia)
• CNS damage (infection/trauma/ low dose irradiation)
• Adoption
• HCG production (CNS or peripheral tumours)
• Hypothyroidism

Peripheral precocious puberty (gonadotrophin independent): peripheral oestrogen

Thelarche

Ovary tumour or cyst (including McCune-Albright syndrome and massive ovarian oedema)

Drug/dietary sources

Testicle/liver tumour

Peripheral precocious puberty (gonadotrophin independent): peripheral androgen

Adrenarche

Atypical congenital adrenal hyperplasia (CAH)

Adrenal tumour (including Cushing’s)

Testicular tumour

Testotoxicosis (male limited family history) and McCune-Albright syndrome (irregular café-au-lait patches, bony changes on xray and ovarian cysts (that may be huge).

Investigation

Examination alone is often enough to determine if this is “true” puberty or just early thelarche (breast development) or pubarche (body hair), especially if combined with bone age.

• X-ray right wrist (bone age)
• Pelvic and abdominal USS (looking for tumours, cysts, size and position of gonads/internal genitalia)

Definitive test for central precocious puberty = GnRH stimulation test:

1)       Measure LH, FSH, oestrogen/testosterone at base-line.

2)       Give GnRH and monitor serial response of LH and FSH (20mins and 60mins)

Interpretation:

MRI hypothalamus, pituitary and brain in aggressive forms of early puberty, in girls less than 6 years of age, any child with neurological signs, and all boys.

Other tests to consider:

• TFTs (elevated TSH in hypothyroidism can mimic FSH, inducing early testicular and ovarian enlargement)
• Tumour markers (HCG can be produced from pineal, hepatic and testicular tumours) and alpha fetoprotein will be raised but LH/FSH will be low and non-stimulatable.
• Testosterone, oestrogen, morning LH/FSH

Treatment

1)       Early thelarche or pubarche: None. However, if pubarche associated with being overweight in girls, important to control weight, otherwise at increased risk of polycystic ovary syndrome (PCOS) and Type 2 diabetes later in life.

2)       Supportive, let nature take its course: coping with periods, behavioural and cosmetic changes. School need to be aware.

3)       Triptorelin or Goserelin injection = long acting GnRH analogues (given every 4-12 weeks depending on preparation used and body’s response to it). Will slow down or stop development. Continued until child’s peers are entering puberty, typically aged 10-11years. GH in addition, may result in improved final height, in girls.

Additional notes on discordant sexual development:

Thelarche: commonly present from infancy, non-progressive, may be unilateral. No treatment required.

Ovarian (and adrenal, testicle or liver) tumours secreting oestrogen are rare and often present with a palpable mass and prominent breast development with no other signs of puberty.

Thelarche-like symptoms are produced from oestrogen containing medications.

Adrenal androgens are the commonest cause of early virilisation leading to sexual hair growth, body odour, acne, greasy hair and mood swings.

Adrenarche = normal maturation of the adrenal glands leading to enhanced secretion of the androgen DHEA. Usually co-existent with the onset of normal puberty and contributes most of the androgenic component of puberty in young females. Premature adrenarche may be familial, spontaneous, or with an ill-understood association with hydrocephalus. No treatment required. A proportion of girls affected will proceed to PCOS, especially if they gain excessive weight.

Atypical CAH (mild, non-salt wasting type) mimics adrenarche and can be easily differentiated by a urinary steroid profile or a short Synacthen test and measurement of 17 alpha hydroxyprogesterone. A pubertal response to stimulation testing will require treatment with both hydrocortisone and GnRH agonists to achieve a reasonable final height.

Non-iatrogenic Cushing’s syndrome tends to be accompanied by excess adrenal androgen secretion and hirsuitism.

Isolated premature menarche is relatively common disorder of unknown aetiology. USS demonstrates prepubertal uterus with no endometrial lining between bleeds. Differential diagnosis includes rare local lesions, e.g. sarcoma, abuse and vaginal foreign body.

Mild, transient breast enlargement occurs in approx 50% boys in early puberty, but severe persistent gynaecomastia is increasingly common, possibly secondary to nutritional excess or environmental chemicals. Usually accompanies early puberty but can be pre-pubertal. Investigations for ectopic oestrogen secretion, karyotype, liver and thyroid function are usually normal. If present for >18m, may require surgical removal. If diagnosed early, treatment with anti-oestrogen medication such as anastrozole may have some benefit.

Click here for this month’s PDF digest!  It ‘s quite hard providing a balance of information for GPs and ED juniors now that I am only doing the one newsletter.  I think we’ve succeeded this month with neurodevelopmental milestones in Down’s syndrome and essential tremor aimed mainly at GPs and pulled elbow, anaphylaxis and the FEAST study aimed more towards the emergency medicine practitioners.  Many thanks to my colleagues who have contributed this month.  The FEAST video makes fascinating and inspiring watching for any health professional, regardless of specialty.  Do leave comments, questions, suggestions!

With thanks to my colleague, Dr Su Li, for summarising this 2011 NICE guideline for Paediatric Pearls.

Anaphylaxis: assessment to confirm an anaphylactic episode and the decision to refer after emergency treatment for a suspected anaphylactic episode

December 2011

www.nice.org.uk/cg134

Anaphylaxis is a severe, life-threatening, generalised hypersensitivity reaction involving

• the airway (pharyngeal or laryngeal oedema) and/or
• breathing (bronchospasm, tachypnoea) and/or
• circulation (hypotension, tachycardia).

There can often be skin and mucosal changes. Patients presenting with these signs and symptoms should be diagnosed as having ‘suspected anaphylaxis’.

Anaphylaxis may be an allergic response that is

• immunologically IgE mediated (foods, venoms, drugs, latex) or
• non-immunologically mediated or
• idiopathic (significant clinical effects with no obvious cause).

This guideline does not make any drug recommendations. These can be found at http://www.resus.org.uk/pages/reaction.pdf.

Patient Centred Care

• Treatment and care should take into account patient’s needs and preferences
• Patients should have the opportunity to make informed decisions about their care and treatment, in partnership with health care professionals
• Good communication between healthcare professionals and patients is essential
• Families and carers should be given the information and support they need
• Care of young people in transition between paediatric and adult services should be planned and managed according to the best practice guidance described in ‘Transition: getting it right for young people’

Recommendations

• Document acute clinical features of the suspected anaphylaxis
• Record the time of onset
• Record the circumstances immediately before the onset of symptoms to help identify possible triggers

• Consider taking blood samples for mast cell tryptase if reaction is thought to be immunologically mediated or idiopathic
  • First sample as soon after emergency treatment given
  • Second sample 1-2 hours (no more than 4 hours) from onset of symptoms
  • A further sample may be required at follow up with the allergy specialist to measure baseline mast cell tryptase

• Children who have had emergency treatment should be admitted to hospital under the care of the paediatric team.  The resus council suggests observing the child for a pragmatic (no evidence yet) 6 hours because of the risk of a biphasic reaction.
• Offer the child/parents a referral to an allergy specialist (see www.bsaci.org for registered allergy clinics)
• Offer the child/parents an adrenaline injector in the interim period whilst waiting for a specialist appointment

• Before discharge, offer the child/parents
  • Information about anaphylaxis (signs, symptoms, risk of recurrence of symptoms (biphasic reaction)).  Parent information leaflet here.
  • Information about what to do if a reaction occurs (use adrenaline injector, call emergency services)
  • Demonstration on how to use an adrenaline injector see http://www.youtube.com/watch?v=pgvnt8YA7r8 for a clear American description of how to use it.
  • Advice about how to avoid potential triggers
  • Information about the need for referral and the referral process to an allergy specialist
  • Information about patient support groups

Research Recommendations

• Mast cell tryptase is not always elevated in children, particularly if food is thought to be the allergen or if respiratory compromise is the main clinical feature. It is recommended that further studies be carried out to identify other potential chemical inflammatory mediators.
• There is limited evidence on biphasic reactions. Follow up studies are recommended.
• There are no studies on length of observation period following emergency treatment for suspected anaphylaxis
• There is limited data on the annual incidence or anaphylactic reactions and their associated outcomes.
• The Guideline Development Group feel that referral to specialist services and/or the provision of adrenaline injectors are likely to benefit patients who have experienced a suspected anaphylaxis as a result of decreased anxiety and ongoing support. This benefit is yet to be quantified.

Dr Furqan Ahmed is an Emergency Medicine middle grade doctor seconded to paediatrics for a few months as part of his training.  I hope he is learning from us, we are certainly picking up things from him.  He has put together the following guide to “pulled elbow” or “radial head subluxation” for Paediatric Pearls.

Pulled elbow^, Nursemaid’s elbow, is a dislocation of the elbow joint caused by a sudden pull on the extended, pronated arm. The technical term for the injury is radial head subluxation.

Pathophysiology:

The etiology is slippage of the head of the radius under the annular ligament. The distal attachment of the annular ligament covering the radial head is weaker in children than in adults, allowing it to be more easily torn.

As children age, the annular ligament strengthens, making the condition less common. The oval shape of the proximal radius in cross-section contributes to this condition by offering a more acute angle posteriorly and laterally, with less resistance to slippage of the ligament when axial traction is applied to the extended and pronated forearm.

Causes, incidence, and risk factors

Radial head subluxation is a common pediatric presentation generally occurring between the ages of 1 and 4 years, although it can happen anytime between 6 months of age and 7 years. After age 3, children’s joints and ligaments gradually grow stronger, making radial head subluxation less likely to occur.

The injury occurs when a child is pulled up too hard by the hand or wrist. It is often seen after someone lifts a child up by one arm (eg. when trying to lift the child over a curb or high step).

Other ways this injury may happen include:

• Breaking a fall with the arm
• Rolling over in an unusual way
• Swinging a young child from the arms while playing

Signs and symptoms

When the injury occurs, the child usually begins crying right away and refuses to use the arm because of elbow pain.

• The child may hold the arm slightly bent (flexed at 15-20 degrees) at the elbow and pressed up against the abdominal area (pronated).
• The child will move the shoulder, but not the elbow. Some children stop crying as the first pain goes away, but continue to refuse to move the elbow.
• Tenderness at the head of the radius may be present.
• Erythema, warmth, oedema, or signs of trauma are absent.
• Distal circulation, sensation, and motor activity are normal

Treatment

Inform child and caregiver that the reduction may be uncomfortable, but the discomfort will end quickly after reduction. Parents should not attempt these manoeuvres at home unless advised by a physician.

To resolve the problem, the affected arm must be held with one hand/finger on the radial head and the other grasping the hand making sure the elbow is in 90° of flexion. While applying compression between these two hands, the forearm of the patient is gently supinated and the arm flexed. The manipulator will usually feel a “click” if the manoeuvre is done properly, the child will feel momentary pain, and usually within 5 minutes, the forearm will be functioning well and painlessly.  NB: although a ‘click’ signifies reduction, absence of a ‘click’ is noted in some successful reductions.

Differential diagnoses:

• Fracture, Elbow
• Fracture, Wrist

• Hand Injury, Soft Tissue

Indication for xray:

Child not using arm 30 minutes after a reduction.  External signs of trauma such as swelling, abrasions, or ecchymoses.

Consultations

If radiographic findings demonstrate no fracture, repeat attempts at reduction are unsuccessful, and the child does not regain normal function after 30-40 minutes, the safest management is to support the arm in a sling (or splint and sling) and have the child reevaluated in 1-2 days time.

Prognosis

The prognosis is excellent. Parents can be reassured that no permanent injury results from this condition.

For those who have had one occurrence, the chance of recurrence is approximately 20-25%.  Those 24 months and younger may have the greatest risk of recurrence.

References

1. ^ Krul M, van der Wouden JC, van Suijlekom-Smit LW, Koes BW (2012). “Manipulative interventions for reducing pulled elbow in young children”. Cochrane Database Syst Rev (1): CD007759. doi:10.1002/14651858.CD007759.pub3. PMID 22258973. 
2. ^ Toupin P, Osmond MH, Correll R, Plint A (September 2007). “Radial head subluxation: how long do children wait in the emergency department before reduction?”. CJEM 9 (5): 333–7. PMID 17935648. http://www.cjem-online.ca/v9/n5/p333. 
3. ^ Kaplan, RE; Lillis, KA (2002 Jul). “Recurrent nursemaid’s elbow (annular ligament displacement) treatment via telephone.”. Pediatrics 110 (1 Pt 1): 171–4. PMID 12093966. 
4. ^ Macias CG, Bothner J, Wiebe R (July 1998). “A comparison of supination/flexion to hyperpronation in the reduction of radial head subluxations”. Pediatrics 102 (1): e10. PMID 9651462. http://pediatrics.aappublications.org/cgi/pmidlookup?view=long&pmid=9651462.

Down’s syndrome occurs in 1:1000 live births and is the commonest identifiable cause of learning difficulties.  There is a significant variability in appearance, personality and levels of general health and independence.  Some medical problems are over-represented in people with Down’s syndrome and, for this reason, this group of children are followed up regularly by a team usually based in the local child development centre.  In Waltham Forest the multidisciplinary Wood Street children’s specialist service team oversee the growth and development of the children with Down’s syndrome, ensuring their health needs are met and their potential for learning maximised.  Click here for their generic Specialist Children’s Services referral form.  See February 2012′s PDF for links to more resources.  I have asked the Wood Street team to add some comments and pathway information below…