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!

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!

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…

Do you know your valgus from your varus?  Or your myclonic epilepsy from your sleep myoclonus?  A link this month to new asthma patient information leaflets and some reminders of NICE’s “Do not do recommendations” in feverish children.  Also the BSACI egg allergy guideline.  Do leave comments on any of these topics below.

December 2011 has snippets of information on torticollis (backed up with lots more information on the website), unconscious children, alkaline phosphatase and a link to the Map of Medicine’s recent algorithm for cough in children.  Also some pointers for your safeguarding training needs.  Download it here.

Our local educational psychologists are running drop in sessions on the 3rd Wednesday of every month at their base in Leyton, E10.  The current flyer which includes contact details is here and sessions will be on-going in 2012 even if not listed here.  They tell me that they would be happy to run EP drop in sessions or parent workshops/training/support groups  at local GP surgeries and jointly with GPs or other medical colleagues  – GPs are welcome to contact them to discuss.  Their Urdu speaking colleague runs sessions in a local Mosque as well.

October’s edition is joint again this month on account of my right radius being fractured and its being too difficult to type and format text boxes with just my left hand…  I am obviously not quite as good at ice-skating as I thought I was.  All the topics this month should be of interest to both the ED and primary care teams anyway:  a paper on paediatric early warning scores, the start of our neurodevelopment series, an update on services for bereaved children and their families and some useful links on the subject of head-lice.

It is not uncommon for us to be referred not-yet-ambulant children just past the 18 month “upper limit of normal” age of walking.  The majority of these children are using means other than crawling to get around.  I had vague recollections of having seen a table once detailing the 97th centile for walking in children who bottom shuffle, commando crawl or roll everywhere but I spent 2 or 3 fruitless hours searching the literature for it a couple of months ago.  So I was ironically excited this month to find that Archives of Disease in Childhood had reproduced it!  One of our current registrars, Dr Amy Rogers, has kindly put together an article for Paediatric Pearls with nuggets from that paper (Sharma A Developmental Examination: Birth to 5 Years. Arch Dis Child Educ Pract Ed 2011;96:162-175 doi:10.1136/adc.2009.175901) which summarises normal development and when it would be prudent to refer children for further developmental assessment:

Approach to developmental assessment – birth to 1 year: Motor¹

 1)    Elicit parental/carer concerns.  Questions to ask: 

• Do you have any concerns about the way your baby moves his arms/legs or body? Have you ever noticed any odd or unusual movements?
• Has your baby ever been too floppy or too stiff?
• Does your baby have a strong preference for one hand and ignore the other hand?

 2)    Gather information on social/biological risk factors:

 Risk factors for poor developmental outcomes

 3)    Observe/elicit behavior and interpret findings

 Note posture and movement.  Examine tone.  Elicit primary (Moro, grasp and asymmetrical tonic neck reflex) and support reflexes (downward, sideward and forward).  Video clips of all these reflexes can be seen at http://library.med.utah.edu/pedineurologicexam/html/newborn_n.html.

 What is not normal?

• Fisting of hands beyond 3 months
• Poor head control at 4 months
• Primitive reflexes beyond 6 months
• Flexor hypertonia in lower limbs beyond 9 months
• Not sitting unsupported with straight spine by 10 months
• Not walking by 18 months

 BUT preterm infants often have delayed motor milestones, early hypotonia and longer lasting asymmetrical tonic neck reflex.  Children with atypical pre-walking movement patterns (ie. non-crawlers) are late in achieving independent sitting and walking.

 Pre-walking movement pattern and motor milestones (97th percentile)²

Refer if concerned as delayed motor development may be a marker for motor disorders and may have a negative impact on a child’s performance in the cognitive and social developmental domains.  There is more information on delayed walking in a Patient Plus article written for health professionals available at http://www.patient.co.uk/doctor/Delay-In-Walking.htm.

 1         Sharma A Developmental Examination Birth to 5 Years. ADC Educ Pract Ed 2011;96:162-175

2         Robson P. Prewalking locomotor movements and their use in predicting standing and walking. Child Care Health Dev 1984;10:317-30