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Congenital pigmented naevi

Dermatology, General paediatrics

Particularly giant or multiple lesions usually present at birth but can be shortly after.

Number of naevi increases through childhood, but high volatility, eg over 3yr period, about a third change pattern, sun burn increases number. Halo pattern of disappearance  (with depigmentation) rare.

Can be associated with neurocutaneous melanosis; if suspected then neuroimaging is advisable, seeking evidence of leptomeningeal melanotic lesions.

Malignant change in childhood is possible, but only really in lesions greater than 1% BSA size.  Melanoma detection relies on morphology eg ABCDE criteria (asymmetry, border irregularity, color variegation, diameter (6 mm, and evolving).  ABCDE very low specificity in kids but melanoma does present in children (and pigmented naevi are risk factor).  Dermoscopic changes in blood vessels important.

Beckers naevus tends to present at puberty,  usually torso or upper arm, irregular, hairy.

Polymicrogyria

General paediatrics, Neurology

One of the most common malformations of cortical development.  

Bilateral perisylvian polymicrogyria (BPP) is the most common subtype , causes the congenital bilateral perisylvian syndrome:

  • oromotor dysfunction,
  • cognitive impairment,
  • epilepsy.

Many possible causes eg vascular or hypoxaemic insults (eg twin-to-twin transfusion syndrome) and congenital cytomegalovirus infection. Genetic causes also important – some syndromes eg 1p36.3 and 22q11.2 deletion syndromes. PIK3R2  gene defects are the most common genetic cause of BPP in patients with normal head size, and the second (to  PIK3CA ) most common cause of polymicrogyria associated with large head size. RTTN gene  also associated with isolated BPP.

[Lancet Neurology, The, 2015-12-01, Volume 14, Issue 12, Pages 1182-1195]

Spiritual care

Communication, Generic

See also Difficult conversations.

Taking a spiritual history

  • do you consider yourself religious or spiritually minded?
  • where do you get inner strength from?

[Larry Culliford podcast]

[RCPsych leaflet]

All staff play a role in spiritual care.  Definition – “Allow people to explore their innermost feelings and ask the most difficult questions about suffering, illness and death”. Aim to help those in need find peace of mind.

Many levels – speaking with dignity and respect, training in bereavement, specialist spiritual care provided by department of spiritual care and wellbeing.  Spiritual care volunteers also available.

[NHSL spiritual care guidelines]

[Scottish government guidance CEL 2008.]

Staff care also important eg reflective practice, mindfulness, Schwartz rounds etc.

LGBT issues

Ethics

Sexual orientation vs sexual practices vs gender identity.

“Coming out”  means primarily acknowledging your own lesbian, gay, bisexual or transgender identity to yourself.  Coming out to others is not a one-off experience, LGBT people have to make decisions on whether to (or not) disclose, often on a daily basis. This can be an ongoing source of stress and distress.  Heterosexism – assumption of heterosexuality, +/- judgment of its superiority in terms of moral value.

Harassment in the workplace can lead to the organisation and/or the individual being found liable and having to pay compensation.

The umbrella term transgender includes transsexual people and transvestites.

A trans-man is someone who transitions from a female label at birth to a male gender identity.  When the transition is complete, their trans identity could be considered a part of their past medical history, rather than an on-going identity.

Trans vestites (medicalized? “Cross-dressers” better?) have no desire for any permanent transition but enjoy aspects of the opposite gender and may have a temporary identity including a different name.

Trans sexual protected under Equality act by EU gender directive 2007.

It is the impact on the individual that determines whether bullying, harassment or discrimination has occurred, not the intentions of the perpetrator.

Gender recognition act 2004 allows trans sexual people to apply for full legal recognition of their acquired gender (evidence must be provided).  It is also a crime to disclose previous gender without express permission.

Under legislation it is also illegal to discriminate against someone on the basis that they are heterosexual!

[Good LGBT practice in NHS document, Stonewall Scotland]

Dyschezia

Common, Gastro, General paediatrics, OPD

Baby strains and cries to pass stool but it comes out soft or not at all.  Functional gastrointestinal disorder thought to occur in 0.9 – 5% of infants under 6 months (Rome IV criteria).

Due to poor co-ordination of pelvic floor muscles with increased intra-abdominal pressure generated during stooling. Seen in babies up to 9 months.

Studies have reported symptoms of discomfort around passing normal stool in up to 18% of babies, not all of these children will strictly meet the diagnostic criteria for dyschezia.

Differentiating from true constipation etc requires a clinical history and a normal clinical examination, with the key difference being that the stool is not hard in dyschezia.

No medication (or any form of rectal stimulation) required, can be expected to resolve spontaneously.

Urate

Community, General paediatrics, Genetics, Metabolic, Neurology

Product of amino acid metabolism.

In developmental delay, an abnormally high or low result is significant viz:

  • Glycogen Storage disorder
  • Purine disorders eg Lesch Nyhan
  • Molybdenum Cofactor deficiency – other features are microcephaly, hyperekplexia

High urate levels can also be risk factor for urolithiasis (stones in urinary tract)

Parenting and permanence orders

Child protection, Ethics

Permanence order is mechanism for local authority to apply for parental rights and responsibilites to be removed from parents.

Not specifically detailed in law (2007) but “threshold test” must be satisfied:

  1. living with parent poses threat of serious detriment to welfare of child
  2. the need to safeguard and promote welfare of child is paramount consideration
  3. that it is better for the child that the order be made, than that the order not be made

Supreme Court decision In the matter of EV (A Child) (No 2) (Scotland) 2017 –

Not duty of parents to prove parenting ability, but for social work to prove lack of ability with full assessment (or adequate records and sworn evidence of non-engagement).

Also, although allegations of harm may be sufficient reason to place child in care, not sufficient for seeking “permanence order”.  Given that this may mean waiting on criminal proceedings to be completed, children may be stuck in hearing system for longer than before.

[https://andersonstrathern.co.uk/news-insight/supreme-court-permanence-order-decision-lessons-learned/]

 

Cerebral Arteriovenous malformation (AVM)

Cardiology, General paediatrics, Neurology

Present with hydrocephalus or else cardiac failure in neonatal period. Clue is that heart is structurally normal!

Life time risk of haemorrhage is high, presenting with headache or seizure.  Annual rate about 6%.  Focal neuro signs rare prior to haemorrhage!  Risk slightly higher in deep locations eg basal ganglia.

Once haemorrhage has occurred, risk of further haemorrhage increases significantly.  Microsurgery, radiosurgery or embolization techniques used alone or in combination.