Tag Archives: gastrointestinal issues

Haemolytic Uraemic Syndrome (HUS)

With thanks to Dr David Gardiner, one of our current paediatric FY2 doctors at Homerton University Hospital, for updating us on HUS.

News story in 1999
News story from 2018.  Less than 3% of patients die of HUS but 20-30% experience adverse renal outcomes.  Think about it in children with bloody diarrhoea and, often, no fever.

Presentation:

  • Profuse diarrhoea that typically turns bloody after 1-3 days
  • Abdominal pain (crampy)
  • Vomiting
  • Fever (sometimes)
  • Oedema
  • 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

Investigations:

  • 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

Management:

  • Refer to secondary care urgently
  • Strict input/output fluid monitoring
  • Correction of anaemia
  • Correction of electrolyte imbalances
  • Antihypertensive therapy if required
  • Dialysis
  • Furosemide to induce diuresis
  • Report to PHE – can’t go back to school until 2 negative stool samples

More resources:

Kidney Research website on HUS

https://patient.info/doctor/haemolytic-uraemic-syndrome-pro#ref-8

May 2018 newsletter published

Cyclical vomiting this month as the message from the front line, BESS as a learning point for those monitoring the size of an infant’s head, milia also for the babies and the perennial problem of whether or not montelukast works to control episodic wheeze.  Do leave comments below:

December 2017 reading list

Retinoblastoma mnemonic this month.  Plus information on lower sugar content recipes for the reintroduction of cows milk into a child’s diet, labial adhesions, 6 in 1 vaccine and don’t miss infantile spasms as early treatment improves overall prognosis.  Do leave comments below.

November 2017 PDF

Children’s cancer information this month – prevalence and red flags, a link to the excellent immunisation resource – Oxford vaccine group – for all those questions about individual immunisations that you can’t always answer,  NICE’s recent UTI update and infant dyschezia.  Do leave comments below.

October 2017 PDF digest

Local anaesthetic cream this month (why do some places not use it in the under 1’s?), a link to useful “flash card” learning in the paediatric ED from Leicester, new Movicol doses, diphtheria and the last instalment of urinalysis with bilirubin and urobilinogen.  A reminder also to please discuss children with glycosuria and a high BM with a paediatrician – most children have type 1 diabetes and are at risk of DKA at diagnosis.  Do leave comments below:

Potted background, assessment and management of vitamin D deficiency

Vitamin D deficiency in children with thanks to Dr Jini Haldar, paediatric registrar at Whipps Cross University Hospital.

Introduction

Vitamin D is an essential nutrient needed for healthy bones, and to control the amount of calcium in our blood. There is recent evidence that it may prevent many other diseases.  There are many different recommendations for the prevention, detection and treatment of Vitamin D deficiency in the UK.  The one outlined below is what we tend to do at Whipps Cross Hospital.

 Prevention

The Department of Health and the Chief Medical Officers recommend a dose of 7-8.5 micrograms (approx. 300 units) for all children from six months to five years of age. This is the dose that the NHS ‘Healthy Start’ vitamin drops provide. The British Paediatric and Adolescent Bone Group’s recommendation is that exclusively breastfed infants receive Vitamin D supplements from soon after birth. Adverse effects of Vitamin D overdose are rare but care should be taken with multivitamin preparations as Vitamin A toxicity is a concern. Multivitamin preparations often contain a surprisingly low dose of Vitamin D.

Indications for measurement of vitamin D

 1. Symptoms and signs of rickets/osteomalacia

  • Progressive bowing deformity of legs
  • Waddling gait
  • Abnormal knock knee deformity (intermalleolar distance > 5 cm)
  • Swelling of wrists and costochondral junctions (rachitic rosary)
  • Prolonged bone pain (>3 months duration)

2. Symptoms and signs of muscle weakness

  • Cardiomyopathy in an infant
  • Delayed walking
  • Difficulty climbing stairs

3. Abnormal bone profile or x-rays

  • Low plasma calcium or phosphate
  • Raised alkaline phosphatase
  • Osteopenia or changes of rickets on x-ray
  • Pathological fractures

4. Disorders impacting on vitamin D metabolism

  • Chronic renal failure
  • Chronic liver disease
  • Malabsorption syndromes, for example, cystic fibrosis, Crohn’s disease, coeliac disease
  • Older anticonvulsants, for example, phenobarbitone, phenytoin, carbamazepine

5. Children with bone disease in whom correcting vitamin D deficiency prior to specific treatment would be indicated:

 

Symptoms and signs in children of vitamin D deficiency

1. Infants: Seizures, tetany and cardiomyopathy

2. Children: Aches and pains: myopathy causing delayed walking; rickets with bowed legs, knock knees, poor growth and muscle weakness

3. Adolescents: Aches and pains, muscle weakness, bone changes of rickets or osteomalacia

 

Risk factors for reduced vitamin D levels include:

  • Dark/pigmented skin colour e.g. black, Asian populations
  • Routine use of sun protection factor 15 and above as this blocks 99% of vitamin D synthesis
  • Reduced skin exposure e.g. for cultural reasons (clothing)
  • Latitude (In the UK, there is no radiation of appropriate wavelength between October and March)
  • Chronic ill health with prolonged hospital admissions e.g. oncology patients
  • Children and adolescents with disabilities which limit the time they spend outside
  • Institutionalised individuals
  • Photosensitive skin conditions
  • Reduced vitamin D intake
  • Maternal vitamin D deficiency
  • Infants that are exclusively breast fed
  • Dietary habits – low intake of foods containing vitamin D
  • Abnormal vitamin D metabolism, abnormal gut function, malabsorption or short bowel syndrome
  • Chronic liver or renal disease

 

Management depends on the patient’s characteristics:

 A. No risk factors

No investigations, lifestyle advice* and consider prevention of risk factors

 

B. Risk Factors Only

1. Children under the age of 5 years: Lifestyle advice* and vitamin D supplementation.

Purchase OTC or via Healthy Start

Under 1 year: 200 units vitamin D once daily

1 – 4 years: 400 units vitamin D once daily

 

2. Children 5 years and over – offer lifestyle advice*

 

 

C. Risk Factors AND Symptoms, Signs

Lifestyle advice*

Investigations:

  • Renal function, Calcium, Phosphate, Magnesium (infants), alkaline phosphatase,
  • 25-OH Vitamin D levels, Urea and electrolytes, parathyroid hormone

 

Children can be managed in Primary Care as long as:

  • No significant renal impairment
  • Normal calcium (If <2.1 mmol/l in infants, refer as there is a risk of seizures)

If further assessment is required consider referral to specialist. **

Patient’s family is likely to have similar risk of Vitamin D deficiency – consider investigation ant treatment if necessary.

 

 

*Life style advice

 

1. Sunlight

Exposure of face, arms and legs for 5-10 mins (15-25 mins if dark pigmented skin) would provide good source of Vitamin D. In the UK April to September between 11am and 3pm will provide the best source of UVB. Application of sunscreen will reduce the Vitamin D synthesis by >95%. Advise to avoid sunscreen for the first 20-30 minutes of sunlight exposure. Persons wearing traditional black clothing can be advised to have sunlight exposure of face, arms and legs in the privacy of their garden.

2. Diet

Vitamin D can be obtained from dietary sources (salmon, mackerel, tuna, egg yolk), fortified foods (cow, soy or rice milk) and supplements. There are no plant sources that provide a significant amount of Vitamin D naturally.

 

  **Criteria for referral
  • Criteria for management in primary care not met
  • Deficiency established with absence of known risk factors
  • Atypical biochemistry (persistent hypophosphatemia, elevated creatinine)
  • Failure to reduce alkaline phosphatase levels within 3 months
  • Family history (parent, siblings) with severe rickets
  • Infants under one month with calcium <2.1mmmol/l at diagnosis as risk of seizure.  (Check vitamin D level of mothers in this group immediately and treat, particularly if breast feeding.)
  • If compliance issues are anticipated or encountered during treatment.
  • Satisfactory levels of vitamin D not achieved after initial treatment.

 

  Vitamin D levels, effects on health and management of deficiency

level effects

management

< 25 nmol/l (10micrograms/l) Deficient.  Associated with rickets, osteomalacia Treat with high dose vitamin D

Lifestyle advice AND vitamin D (ideally cholecalciferol)

• 0 – 6 months: 3,000 units daily

• 6 months – 12 yrs: 6,000 units daily

• 12 – 18 yrs: 10,000 units daily

vitamin D 25 – 50 nmol/l (10 – 20micrograms/l Insufficient and associated with disease risk Over the counter (OTC) Vitamin D supplementation (and maintenance therapy following treatment for deficiency) should be sufficient.

 

• Lifestyle advice and  vitamin D supplementation

< 6 months: 200 – 400 units daily (200 units may be inadequate for breastfed babies)

Over 6 months – 18 years: 400 – 800 units daily

50 – 75 nmol/l (20 – 30micrograms/l) Adequate Healthy Lifestyle advice
> 75 nmol/l (30 micrograms/l) Optimal Healthy None

 

Course length is 8 – 12 weeks followed by maintenance therapy.

 

 Checking of levels again

As Vitamin D has a relatively long half-life levels will take approximately 6 months to reach a steady state after a loading dose or on maintenance therapy. Check serum calcium levels at 3 months and 6 months, and 25 – OHD repeat at 6 months. Review the need for maintenance treatment.  NB:  the Barts Health management protocol uses lower treatment doses for a minimum of 3 months and then there is no need for repeat blood tests in the majority of cases of children satisfying the criteria for management in primary care.

 Serum 25 OHD after 3 months treatment Action

level action review
>80nmol/ml Recommend OTC prophylaxis and lifestyle advice as required
50 – 80 nmol/mL Continue with current treatment dose reassess in 3 months
< 50 nmol/mL Increase dose or, in case of non-adherence/concern refer to secondary care.  

It is essential to check the child has a sufficient dietary calcium intake and that a maintenance vitamin D dose follows the treatment dose and is continued long term.

Follow-up:

Some recommend a clinical review a month after treatment starts, asking to see all vitamin and drug bottles. A blood test can be repeated then, if it is not clear that sufficient vitamin has been taken.

Current advice for children who have had symptomatic Vitamin D deficiency is that they continue a maintenance prevention dose at least until they stop growing. Dosing regimens vary and clinical evidence is weak in this area. The RCPCH has called for research to be conducted.  The RCPCH advice on vitamin D is at http://www.rcpch.ac.uk/system/files/protected/page/vitdguidancedraftspreads%20FINAL%20for%20website.pdf

JINI HALDAR

 

June 2015 published

Gianotti Crosti this month, updated “Working Together” safeguarding document, epistaxis and malaria.  Also links to a few other useful documents recently uploaded to the Primary Care Guidelines part of the website, with thanks to Redbridge and West Suffolk.  All comments welcome.

February 2015 (just)

Have just uploaded February 2015 newsletter – with 4.5 hours of February to go….

NICE on gastro-oesophageal reflux disease, how to recognise speech delay, more viral exanthems, resus cards and information on forthcoming allergy courses.  Do leave comments below: