More on the coca-cola urine story this month, NF type I, cerebellar ataxia and restless legs syndrome. Do leave comments below.
With thanks to Dr David Gardiner, one of our current paediatric FY2 doctors at Homerton University Hospital, for updating us on HUS.
- Profuse diarrhoea that typically turns bloody after 1-3 days
- Abdominal pain (crampy)
- Fever (sometimes)
- Reduced urine output (abrupt onset) but also polyuria/normal urine output (rarer)
- Neurological complications: seizure, coma, cranial nerve palsies, confusion, hallucinations
- Classic triad – anaemia, uraemia and thrombocytopaenia
- Most common in children under the age of 5
- B/P – hypertension
- Blood film: Fragmentation and signs of haemolysis (Coombs test negative)
- Raised WCC and neutrophils, low platelets, low Hb
- Raised LDH
- Clotting screen typically normal (cf DIC)
- Raised bilirubin, low albumin
- Urea and creatinine raised
- Stool for PCR E.Coli
- Refer to secondary care urgently
- Strict input/output fluid monitoring
- Correction of anaemia
- Correction of electrolyte imbalances
- Antihypertensive therapy if required
- Furosemide to induce diuresis
- Report to PHE – can’t go back to school until 2 negative stool samples
Updated rhinitis guideline (2017) from the British Association of Allergy and Clinical Immunology http://www.bsaci.org/Guidelines/rhinitis-2nd-edition-guideline
- Allergic rhinitis is common and affects 10–15% of children and 26% of adults in the UK
- Affects quality of life, school and work attendance, and is a risk factor for development of asthma.
- Diagnosed by history and examination, supported by specific allergy tests.
- Topical nasal corticosteroids are the treatment of choice for moderate to severe disease
- Combination therapy with intranasal corticosteroid plus intranasal antihistamine is more effective than either alone and provides second line treatment for those with rhinitis poorly controlled on monotherapy
- Immunotherapy is highly effective when the specific allergen is the responsible driver for the symptoms
- Treatment of rhinitis is associated with benefits for asthma
- Non-allergic rhinitis also is a risk factor for the development of asthma
- Non-allergic rhinitis may be a presenting complaint for systemic disorders such as granulomatous / eosinophilic polyangiitis, sarcoidosis
Case history: a 5-month-old boy was referred to clinic because his head circumference had jumped from below the 25th to the 75th centile and his GP felt that he had a prominent anterior fontanelle. He was developmentally normal with some noticeable frontal bossing. There had been concerns about his mother having had “hydrocephalus” when she was a baby.
Benign enlargement of the subarachnoid space in infancy (BESS)
- usually involves the frontal lobe subarachnoid spaces
- characterised clinically by a widened fontanelle, macrocephaly and/or frontal bossing
- M > F
- often a family history
- majority are neurodevelopmentally normal
- head circumference climbs through the centiles, plateauing on one of the top 2 centiles in late infancy
- unclear pathophysiology
- a transient accumulation of cerebrospinal fluid in the frontal region or delayed development or function of the arachnoid villi at the sagittal sinus?
- cranial ultrasound / MRI show extra fluid around the brain frontally but no ventricular enlargement
- There’s a more scientific and detailed radiological description at https://radiopaedia.org/articles/benign-enlargement-of-the-subarachnoid-space-in-infancy
- can be complicated by chronic subdural haemorrhage possibly secondary to the stretching of subdural veins (Papasian, 2000)
- type 1 glutaric aciduria also presents with increasing head size but these children are not developmentally normal and have other signs on their cranial imaging (Biswas, 2016)
- more information at J Pediatr Neurosci. 2014 May-Aug; 9(2): 129–131 although I’m not convinced of the need for the follow up imaging advocated here, especially if it requires a general anaesthetic
- The literature suggests that BESS resolves spontaneously by 2 years.
- The macrocephaly is likely to persist
The head circumference of the baby presented above plateaued between the top 2 centiles at 10 months. He remains neurodevelopmentally normal.
Picture courtesy of Dr Abdel-Rahman Abdel-Halim, from the case https://radiopaedia.org/cases/29
- Run to a place of safety
- Turn your phone onto silent
- Turn off vibrate
- Only when safe call police on 999
- Caused by a tick-borne spirochaete of the Borrelia species, which is spread by a bite from an infected tick
- Ticks live in many woodland and grassy areas but only a small number carry the bacteria that causes Lyme disease
- 2,000 to 3,000 diagnoses each year in England and Wales.
- erythema migrans rash, examples here.
Flu-like symptoms to start with. Other symptoms include migratory inflammatory arthritis, uveitis, pain or numbness, trouble with memory, heart block, pericarditis
ELISA and immunospot testing are used for diagnosis but false negatives are possible especially in first 4 weeks
Treated with doxycycline or amoxicillin
Children above the age of 5 in the UK can usually cope with viral gastroenteritis without needing medical input. They vomit a few times, move on to the diarrhoeal stage, get thirsty and a bit dehydrated and start drinking just as it all stops, thereby successfully rehydrating themselves and getting on with their lives. So if a vomiting 9 year old is brought to us by a parent who says they’ve been admitted 4 times before for iv fluids , it is probably worth taking a closer look. The shocked, prostrate child we saw in the ED this weekend (a re-presentation) may have cyclical vomiting. More information about this here. Early treatment with anti-emetics and benzodiazepines may help avoid the need for iv fluids.
Journal Club is a revamped monthly feature in the Paediatric Pearls newsletter. I’m happy to receive submissions from any primary or secondary care journal club you are running as long as the paper is relevant to front line health professionals working with children. Please contact me through the contact page.
With thanks this week to Dr Saskia Wills who took us through a paper on the need (or not) for LPs in children with complex febrile seizures. Her full presentation is here.
- The definition of a febrile seizure in this paper is a seizure in a child 6 months to 5 years with a fever >38o and without an underlying CNS infection or a history of afebrile seizures
- They occur in 2-4% of children <5yrs (peak at 12-18 months)
- They are classified as complex if they last >15 minutes, have a focal onset, or there are multiple episodes within 24 hours
- They are often associated with viral infections, especially HHV6
- The risk is slightly higher in boys and those with a family history of febrile convulsion
- 1/3 of children will have another febrile seizure in the future, but very few (2.4%) go on to have epilepsy. (The risk of epilepsy, which varies with different presenting features, is discussed here)
- In a retrospective French study of otherwise well children presenting with complex febrile seizures, only 5 out of 839 (0.7%) had confirmed bacterial meningitis. All of these had had a prolonged seizure plus some ongoing abnormal neurology or sign suggestive of CNS infection. The study concluded that in children with complex febrile seizures but no other signs of CNS infection, LP usually isn’t necessary. The risk of proven CNS disease is higher in those under 1yr and with a prolonged seizure. This study didn’t look at children who had other risk factors for meningitis, such as immunodeficiency.
Paper studied: Guedj R1, Chappuy H2 et al. Do All Children Who Present With a Complex Febrile Seizure Need a Lumbar Puncture? Ann Emerg Med. 2017 Jul;70(1):52-62. PubMed Link.