Raised intracranial pressure on the front line this month, menorrhagia in adolescents, psychological first aid and some normal reference values for children from Royal College of Paediatrics and Child Health.
Do leave comments below.
Raised intracranial pressure on the front line this month, menorrhagia in adolescents, psychological first aid and some normal reference values for children from Royal College of Paediatrics and Child Health.
Do leave comments below.
Part 2 of Medically Unexplained Symptoms this month. Also antibiotics in cystitis, too many investigations in community acquired pneumonia, carotenaemia and heart murmurs in unwell children. Do leave comments below:
June 2018 features include the rotavirus immunisation, febrile myoclonus, investigating normochromic anaemia, complications of sinusitis and the first in our adolescence series. Please do leave comments below:
NICE on Lyme disease this month – just in time for the weather to pick up and the tics to start biting. Also a reminder on the risk factors for SIDS, what to do in a terrorist attack, how to manage a child with a non-blanching rash and a discussion on the use of the antistreptolysin O titre. Do leave comments below:
April 2017 brings children who were not brought, lucozade, hypertension, leucocytes in urine and macrocytosis. I am not sure of the linking theme… Do leave comments below.
MCV- Mean Corpuscular Volume. (with thanks to Dr Xanna Briscoe and Prof Irene Roberts)
A measure of the size of the red blood cells.
Raised MCV- macrocytosis– may occur with or without anaemia. Physiological macrocytosis in the absence of anaemia occurs in neonates, especially those with Down syndrome, and during pregnancy.
Macrocytic anaemia- may be secondary to nutritional deficiencies in B12 and Folate leading to ineffective or abnormal erythropoiesis. This is easily diagnosed using simple blood tests. Where deficiency is excluded bone marrow examination may be required to identify rare causes such as myelodysplasia or Fanconi anaemia.
There are several drugs that may lead to macrocytosis, some of which are commonly used in the paediatric population. These include several chemotherapeutic agents, antibiotics and antiviral medications. It is also seen in congenital heart disease, hypothyroidism and Down Syndrome.
Additional investigations- guided by the history. It is important to check a reticulocyte count if a macrocytic anaemia is discovered. Reticulocytes are immature erythrocytes- which are large, and indicate increased erythropoiesis. Chronic reticulocytosis may falsely elevate the MCV. The absence of a raised reticulocyte count in the presence of severe anaemia suggests an inability of the bone marrow to produce red cells, eg due to inherited or acquired red cell aplasia.
Kaferle, Joyce, and Cheryl E. Strzoda. “Evaluation of macrocytosis.” American family physician 79.3 (2009).
Microcytosis– small red blood cells. Typically seen in iron deficiency anaemia; in the paediatric population at different ages the cause differs. In younger children and toddlers lack of supplementation may lead to deficiency. This is a particular issue in those that drink large volumes of cows milk as a substitute for iron containing foods. The main differential diagnosis is beta- or alpha-thalassaemia trait. Measurement of serum ferritin is the most useful test to identify iron deficiency- this will be low in iron deficiency and normal in beta- or alpha-thalassaemia trait.
In adolescence the pubertal growth spurt, and menorrhagia may be a causative factor. Further investigation will aid in determining the causes of microcytic anaemia (see below).
W Owen Uprichard, James Uprichard. Investigating microcytic anaemia. BMJ 2013;346:f3154
As published in April 2017 Paediatric Pearls newsletter…..
Increased MCV (macrocytosis) | Decreased MCV (microcytosis) |
Vitamin B12 DeficiencyFolic Acid DeficiencyAlcohol Abuse
Liver disease Marrow aplasia Myelofibrosis Reticulocytosis Hypothyroidism |
Iron Deficiency AnemiaThalassemiaHemoglobinopathy
Anemia of Chronic Disease Sideroblastic Anemia Chronic Renal Failure Lead Poisoning |
Source: http://www.fpnotebook.com/HemeOnc/Lab/MnCrpsclrVlm.htm
MCV is expressed in femtoliters = 10^-15 liters
MCV cutoffs vary by age and by lab reference
MCV Normal Range:
MCV Cutoffs for Microcytic Anemia:
Some sources advocate MCV <78 and others <82
If you only asked for FBC and the child is more than 6 months old, try this:
Anaemia of chronic disease | Thal trait (alpha OR beta) | Iron deficiency anaemia | Thal trait + IDA | Haemoglobinopathy | |
Hb | ↓ | N / ↓ | ↓ / ↓↓ | ↓ / ↓↓ | ↓ or ↑ |
MCV | N / ↓ | ↓ / ↓↓ | ↓ / ↓↓ | ↓ / ↓↓ | ↓ or ↑ |
MCH | N / ↓ | ↓ / ↓↓ | ↓ / ↓↓ | ↓ / ↓↓ | ↓ or ↑ |
RBC | ↓ | N/↑ | N / ↓ | N / | ↓ or ↑ |
RDW | N | N | ↑ | ↑ | ↓ or ↑ |
Therefore a child of 6 months or older with hypochromic, microcytic anaemia with an increased RDW has presumed iron deficiency. They could have thalassaemia trait as well….
If you asked for other tests or are at liberty to repeat the blood test, here are some suggested extra investigations and their interpretation:
Investigation | Iron deficiency anaemia | Thalassaemia trait | Sideroblastic anaemia | Chronic disease |
Ferritin | decreased | Normal | increased | Normal/increased |
Iron | Decreased | Normal | Increased | Decreased |
TIBC | increased | Increased | Normal | decreased |
transferrin | decreased | normal | Normal | Decreased |
Hb electropheresis | normal | Β thalassaemia- raised A2
α trait- normal |
normal | normal |
Part 2 of “Decoding the full blood count” with thanks to Dr Alexandra Briscoe, paediatric registrar at Whipps Cross University Hospital, and Professor Irene Roberts, professor of paediatric haematology at Oxford.
Haematocrit/ packed cell volume- the proportion of blood that is made up of cells (not plasma); it is measured as a percentage or fraction.
Low haematocrit is seen in anaemia, though it will not tell you the direct cause for the anaemia.
Raised haematocrit is seen in polycythaemia, in the newborn infant this is termed Neonatal Polycythaemia.
Defined as a venous haematocrit > 65%, occurring in 0.4-5% of healthy newborns. Symptoms are believed to be due to hyperviscosity. On examination children appear plethoric, and may have multi-systemic symptoms. These include- CNS features of irritability, cerebrovascular accidents and seizures. Apnoea and respiratory distress occur as a result of decreased pulmonary blood flow. In addition infants may demonstrate poor feeding, and may in rare cases develop necrotising enterocolitis (NEC.) Renal effects include renal vein thrombosis, oliguria, proteinuria and haematuria. Hypoglycaemia and thrombocytopenia (Vlug, 2013) are also seen commonly.
The development of polycythaemia occurs secondary to increased erythropoiesis as a consequence of chronic fetal hypoxia. IUGR and placental insufficiency- due to post-dates pregnancies, pre-eclampsia and maternal smoking, increase the incidence of polycythaemia. Infants of diabetic mothers, those with Beckwith –Weidemann, and congenital thyrotoxicosis are also at increased risk.
There has been much debate as to whether delayed cord clamping increases the incidence of polycythaemia. Current NICE guidelines recommend cord clamping between 1-5 minutes after delivery, provided there is no concern regarding the infant’s heart rate or need for resuscitation. In a Cochrane review of cord clamping practices and neonatal outcomes in 2013 McDonald et al found that delayed cord clamping was associated with increased risk of jaundice requiring phototherapy, however beneficial outcome in terms of iron stores- with a 50% reduction in iron deficiency at 3-6 months. They reported no difference in incidence of polycythaemia in 5 trials measuring this outcome.
Current management of symptomatic polycythaemia is a partial exchange transfusion.
Vlug RD, Lopriore E, Janssen M, et al. Thrombocytopenia in neonates with polycythemia: incidence, risk factors and clinical outcome. Expert Rev Hematol. 2015 Feb. 8 (1):123-9. [Medline].
ID: CD004074 McDonald, Susan J, Middleton, Philippa, Dowswell, Therese Morris, Peter S
Effect of timing of umbilical cord clamping of term infants on maternal and neonatal outcomes
Cochrane Database of Systematic Reviews 2013
DOI: 10.1002/14651858.CD004074.pub3
US: http://onlinelibrary.wiley.com/doi/10.1002/14651858.CD004074.pub3/abstract
(taken from http://www.medicinenet.com/hematocrit/page2.htm)
The normal ranges for haematocrit depend on the age and, after adolescence, the sex of the individual. The normal ranges are:
These values may vary slightly among different laboratories.
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
2. Symptoms and signs of muscle weakness
3. Abnormal bone profile or x-rays
4. Disorders impacting on vitamin D metabolism
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:
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:
Children can be managed in Primary Care as long as:
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.
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.
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
Article by Dr Hajera Sheikh, paediatric registrar
Assessment in Secondary Care
History:
• 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
Examination:
• 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)
Investigations (directed)
• Urinalysis
• 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
Aetiology
• Genetic studies
• Thyroid studies: T3, thyroid antibodies, calcium, phosphate
• Cushing syndrome investigations
For co-morbidities
• 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:
• Chromosomal
Prader-Willi syndrome
Trisomy 21
• Autosomal dominant
Biemond syndrome (some cases)
• Autosomal recessive
Aistrom syndrome
Bardet-Biedl syndrome
Biemond Syndrome(some cases)
Carpenter syndrome
Cohen syndrome
• X-linked inheritance
Borjeson-Forssman-Lehmann syndrome
• 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
Colobomata
Retinal problems, especially retinitis pigmentosa
Narrow palpebral fissures
Abnormally positioned palpebral fissures
Severe squint (eg Prader-Willi)
• Skeletal abnormalities
Polydactyly
Syndactyly
Kyphoscoliosis
• Sensorineural deafness (eg Alstrom syndrome: sensorineural deafness, diabetes mellitus, retinal dystrophy, obesity)
• Microcephaly and/or abnormally shaped skull
• Mental retardation
• Hypotonia
• Hypogonadism
Crptorchidism
Micropenis
Delayed puberty
• Renal abnormalities
• Cardiac abnormalities
Neglect and emotional abuse is the safeguarding topic this month. ED advice on the management of minor head injuries, a report from BPSU in hypocalcaemic fits secondary to vitamin D deficiency, the new UK immunisation poster and a bit on crying babies. Hope you find it all helpful. Comments welcome below