NICE on faltering growth this month, paediatric stroke, a reminder of the new epilepsy classification and a contribution from the safeguarding team on what constitutes a “legal high”? Do leave comments below:
The burns triage tool this month plus a bit on urinalysis (pH) and the start of our decoding the FBC series. Also a reminder about the MAP guideline for management of CMPA in primary care, a link to some good courses on this topic and to a document I have put together on milks to use in the UK for CMPA.
Vitamin D deficiency in children with thanks to Dr Jini Haldar, paediatric registrar at Whipps Cross University Hospital.
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.
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:
- Osteogenesis imperfecta
- Idiopathic juvenile osteoporosis
- Osteoporosis secondary to glucocorticoids, inflammatory disorders, immobility
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
- 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
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.
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
|< 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
|>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.
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
Sick and tired – the truth about infantile reflux
By Dr Tom Waterfield
We have all had that difficult conversation regarding “reflux” when a tired parent has come to us with their “sicky child” and an unshakeable belief that their baby has gastro-oesophageal reflux disease. There is often enormous pressure to provide a solution but how do we decide which children need treatment and what treatments should we use? In view of the recent concerns regarding the use of Domperidone I have chosen to review the current evidence base for the management of this common problem.
The North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition(NASPGHAN) and the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition (ESPGHAN) produced a useful guideline document in 20091. This concise 50 page document discusses the evidence base for all aspects of gastro-oesophageal reflux and some of the key points are outlined below.
1) Physiological Gastro-oesophageal reflux (GER) is common; around 50% of healthy infants will display symptoms of GER. These “happy spitters” will be gaining weight and healthy1.
2) Faltering growth is unlikely to be due to GERD and alternate diagnosis such as cow’s milk protein allergy should be considered1.
1) The Gold-Standard investigation to make a positive diagnosis of GERD is an impedance study. This has largely replaced the pH study. In this study the changes in the electrical impedance (ie, resistance) between multiple electrodes located along an oesophageal catheter are used to measure reflux. Unlike a pH study the impedance study will also be able to detect non-acidic reflux1.
2) In the majority of cases there will be no role for any other diagnostic test for GERD1
Try to avoid treating simple GER. Reassurance is often all that is required. Before starting any treatment have a frank discussion regarding the risks and benefits1.
2) Positioning “Tummy Time”
There is evidence that lying prone improves GERD when compared with lying supine or semi-erect. It is however, not recommended that children sleep prone due to the associated risk of sudden infantile death (SIDS). A sensible compromise might involve allowing the child to lie prone when awake and supervised by the parent. Semi-supine positions (such as sitting in a car seat) are not recommended and may exacerbate reflux symptoms1.
3) Thickened Feeds
Commercially available thickened feeds (anti-reflux feeds) are safe and relatively effective at reducing visible regurgitation1.
4) Buffering agents and Alginates
There is very little evidence to support the use of alginates (e.g. Gaviscon Infant) in the treatment of GERD although their use is likely to be safe1.
5) H2RAs and PPIs (Unlicensed treatments)
Antacid treatment with Histamine 2 Receptor Antagonsists (HR2As) is effective at healing proven oesophagitis in adults but there is very little data to support their use in infancy. H2RAs such as Ranitidine are relatively safe but their effectiveness is unproven and there are high rates of tachyphylaxis thereby limiting their usefulness in the long term1.
Proton Pump Inhibitors (PPIs) such as Lansoprazole and Omeprazole do not demonstrate tachyphylaxis and can be used for longer term acid suppression. Despite this however, randomised placebo controlled studies have failed to demonstrate a benefit of (PPIs) over placebo when treating GERD in infants1.
Some studies have suggested that long term acid suppression with PPIs and H2RAs can lead to increased rates of pneumonia and gastroenteritis1.
6) Prokinetics (unlicensed)
ESPGHAN and NASPGHAN advise against the use of all prokinetic agents including Erythromycin and Domperidone. There is no reliable evidence to support their effectiveness at treating GERD in infants and there have been concerns raised over the potentially cardiotoxic effects of Domperidone2.
Reflux is very common with half of infants having some symptoms. In the majority of cases reassurance is all that is required. If symptoms are severe and persistent and an alternate diagnosis is unlikely then consider thickened feeds and “tummy time” as a first line treatment. If this is unsuccessful then consider antacids but be aware that the evidence base for these treatments is limited and they are being used off license. Prokinetics play no part in managing GERD in infants and Domperidone use may be cardiotoxic2.
1) Pediatric Gastroesophageal Reflux Clinical Practice Guidelines: Joint Recommendations of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition(NASPGHAN) and the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition (ESPGHAN)Journal of Pediatric Gastroenterology and Nutrition. 49:498–547 # 2009
2) Domperidone: limited benefits with significant risk for sudden cardiac death. Hondeghem LM.J CardiovascPharmacol. 2013 Mar;61(3):218-25.
Eczema this month, a reminder of treatment of infections and links to some useful audit tools from NICE. Next month, scabies. Another excellent website on food and nutrition in toddlers with a bit on do’s and don’ts of faddy eating and a paper on whether treating ADHD reduces crime. Do leave comments.
Article by Dr Hajera Sheikh, paediatric registrar
Assessment in Secondary Care
• Lifestyle Assessment
• Menstrual History
• Obstructive Sleep Apnoea: Snoring, difficulty breathing during sleep, morning headaches or fatigue
• Symptoms of co-morbidity including psychological
• Drug use (particularly glucocorticoids and atypical antipsychotics)
• Family history, particularly diabetes <40 yrs, early heart disease <60 yrs
• Height, weight, BMI
• Obesity pattern: generalised, central (greater risk of adverse cardiovascular outcomes), buffalo hump and neck (may be suggestive of Cushing syndrome)
• Blood pressure
• Pubertal assessment
• Acanthosis nigricans (indicative of insulin resistance, first seen round neck and axillae)
• Signs of endocrinopathy
• Dysmorphisms: (Look out for early onset obesity, learning difficulties, deafness, epilepsy, retinitis, dysmorphic features, hypogonadism)
• Thyroid function
• Fasting lipids (total and HDL cholesterol), triglycerides
• Liver function, including ALT
• Fasting glucose and insulin not usually done first line
Refer to Paediatric Obesity/Endocrinology or other specialist service if further investigation is required
• Genetic studies
• Thyroid studies: T3, thyroid antibodies, calcium, phosphate
• Cushing syndrome investigations
• Oral glucose test
• PCOS studies (LH, FSH, adrenal androgens, Sex Hormone Binding Globulin, prolactin, pelvic ultrasound)
• Sleep Study
Dysmorphic and monogenic syndromes associated with obesity:
Main clinical obesity associated syndromes:
• Autosomal dominant
Biemond syndrome (some cases)
• Autosomal recessive
Biemond Syndrome(some cases)
• X-linked inheritance
• Single gene lesions affecting leptin metaboilsm
Congenital leptin deficiency
Leptin receptor mutation
Prohormone convertase 1 mutation
Melanocortin 4 mutation
Clinical features suggesting obesity may be secondary to another condition or syndrome
• Severe unremitting obesity
• Disorders of the eyes
Retinal problems, especially retinitis pigmentosa
Narrow palpebral fissures
Abnormally positioned palpebral fissures
Severe squint (eg Prader-Willi)
• Skeletal abnormalities
• Sensorineural deafness (eg Alstrom syndrome: sensorineural deafness, diabetes mellitus, retinal dystrophy, obesity)
• Microcephaly and/or abnormally shaped skull
• Mental retardation
• Renal abnormalities
• Cardiac abnormalities