Scottish Paediatric Society Summer Meeting,June 2014

In utero acquisition of herpes simplex virus

Ian Tallach¹, Pamela Molyneaux¹ and Samuel Stafrace²

¹Royal Aberdeen Children's Hospital, UK

²Raigmore Hospital, UK

Abstract

In the United Kingdom, diagnosis of neonatal herpes simplex virus (HSV) infection is estimated at 1.65 in 100,000 live births.¹ Congenital (antenatally-acquired) HSV represents around 5% of these cases.² Either HSV1 or HSV2 can be responsible. For both congenital and perinatally acquired HSV, the highest risk is to the infants of mothers with primary genital herpes during pregnancy, although, in 60–80% of cases, mothers have no history of genital herpes.³ We describe a female infant with congenital HSV, whose mother had genital ulceration at 11 weeks’ gestation.

Three aspects of this case deserve particular mention and potentially inform current practice.

- The mother had chorioamnionitis at the time of delivery, which may have potentiated in utero transmission of HSV as well as precipitating preterm labour. In the most extensive review of congenital HSV infection,³ 64% of these infants were born prematurely and only half of these had a suggestive rash. We suggest that any unexplained skin lesions at birth be tested for HSV DNA and that suspicion should be heightened by prematurity and/or maternal bacterial infection.

- In the context of neonatal HSV, focal magnetic resonance imaging (MRI) changes to the temporal lobes are unusual. However, these findings have only recently been reported in a small case series.⁴ In this infant, MRI anomalies preceded abnormal clinical signs by three months. She later presented with profound neurodisability.

- Recent data from a double-blind, placebo-controlled trial of acyclovir treatment in babies with neonatal HSV,⁵ shows that oral acyclovir suppression therapy for six months, following initial intravenous treatment, positively influences neurodevelopment at one year. In this infant, there were several episodes of cutaneous reactivation during oral acyclovir treatment. Viral isolates demonstrated high-grade resistance to acyclovir. A month after cessation of acyclovir, HSV2 isolates were again susceptible.

An eye to a diagnosis

Jennifer McInally, Timothy Lavy, Ruth McGowan, and Judith Simpson

Southern General Neonatal Unit, Southern General Hospital, UK

Abstract

We describe two cases where ophthalmology assessment led directly to a diagnosis in infants presenting with a constellation of dysmorphic features.

Case 1 was a baby delivered at 34 + 2 weeks’ gestation following preterm, prolonged rupture of membranes. On examination, he was noted to have a microphallus, cryptoorchidism and a hypoplastic scrotum. His karyotype was confirmed as 46XY and a heart murmur was documented. He was transferred to our institution on day 8 of life for assessment by the paediatric endocrine and cardiology teams. Echocardiogram identified multiple atrial septal defects and a persistent ductus arteriosus. He subsequently developed marked hypocalcaemia and was noted to have low T lymphocyte subsets. In view of these findings, a diagnosis of DiGeorge syndrome was suspected but no 22q deletion was identified on mutation analysis. At three weeks of age, he underwent ophthalmology assessment, which identified bilateral optic disc colobomata, raising the possibility of a diagnosis of CHARGE syndrome. This was subsequently confirmed when a mutation of CHD7, the gene responsible for over half of the cases of this condition, was identified.¹ Whilst phenotypic overlap between CHARGE and 22q deletion syndromes has been described in the medical literature, it was identification of his colabomata which prompted a diagnosis in this case.

Case 2 was a term baby who presented with mild dysmorphism and signs of upper airway obstruction at 6 h old. She was referred to our institution on day 8 of life for ear, nose and throat (ENT) assessment. Microlaryngoscopy and bronchoscopy were performed and revealed a small but structurally normal airway. In view of her dysmorphism, she was examined by a clinical geneticist who noted that she had low set ears, a long philtrum and wide interdigital spaces, but stated that no definitive diagnosis was immediately apparent. Ophthalmology review the following day detected a large right-sided retinal lesion consistent with a retinoblastoma. This prompted targeted genetic analysis, looking specifically for a deletion on the long arm of chromosome 13 known to be associated with an increased risk of retinoblastoma, dysmorphic facial features and intellectual impairment.² This deletion was subsequently identified.

In summary, these cases highlight the importance of ophthalmology assessment in the diagnostic work up of infants with dysmorphic features. They also demonstrate the value of a holistic approach to their diagnosis and management.

Food for thought – a case of Glut1 deficiency syndrome

Iain Chalmers¹, Mona Rahim¹, Christine Findlay¹, Sameer Zuberi², Mary O'Regan², Eleanor Reavey³ and Barbara Cochrane⁴

¹Paediatric Department, Crosshouse Hospital, UK

²Fraser of Allander Neurosciences Unit, Royal Hospital for Sick Children, UK

³West of Scotland Genetics Service, Southern General Hospital, UK

⁴Paediatric Dietetics, Royal Hospital for Sick Children, UK

Abstract

A six-year-old boy who had recently moved from Poland was referred to Community Paediatrics due to concerns regarding speech, behaviour and poor social interaction. He was found to have moderate learning difficulties and was making no progress in school despite one-to-one support from a Polish-speaking assistant. While being assessed, he also presented to his GP with concerns about his walking. He seemed to tire easily and had an unsteady gait, which worsened with exercise and was demonstrated well on the video captured by mother. Examination in clinic was unremarkable other than microcephaly (occipitofrontal circumference 0.4th centile) and initial screening investigations (full blood count, thyroid function tests, creatinine kinase, genetic array, fragile X) were normal.

He then presented acutely to hospital following a generalised clonic seizure that occurred shortly after waking. Examination and blood sugar were normal. Subsequent electroencephalography (EEG) demonstrated episodes of nonconvulsive status epilepticus. Further questioning revealed a history of probable absence seizures occurring over the past two to three years. Anticonvulsant therapy was commenced. Magnetic resonance imaging (MRI) brain was normal. Molecular genetic testing confirmed a pathogenic sequence variant in the SLC2A1 gene (SLC2A1:c.[634C > T];[=];p.[(Arg212Cys)];[=]), confirming a diagnosis of Glut1 deficiency syndrome. He was commenced on a classical 3:1 ketogenic diet and is in the process of establishing this.

Glut1 deficiency is associated with a broad spectrum of phenotypes reflecting the consequences of cerebral energy deprivation. These include learning disability, epilepsy, chronic ataxia and spasticity mimicking cerebral palsy, paroxysmal exercise-induced dyskinesia and migraine. It is caused by genetic defects in the Glut1 transporter, responsible for glucose transport across the blood brain barrier, resulting in inadequate cerebral spinal fluid (CSF) glucose for cerebral metabolism. Ketogenic diet therapies are the treatments of choice as they provide the brain with an alternative fuel source to glucose.

In the last three years, genetic testing has been available in Scotland and even though testing is principally undertaken in neurology centres, almost 30 Scottish patients have been identified varying in age from one year to the sixth decade. This case highlights the different ways in which affected patients may present to paediatric services. Physicians must have a high level of awareness of this disorder as treatment may resolve or dramatically improve symptoms and prevent progressive neurodisability.

Lumps, bumps and bilious vomits: a rare but important cause

Louisa Bertram, Jane Grassie, Gopi Menon, Claire Clark, Mark Brougham and K McKenzie

Neonatal Unit, Simpson Centre for Reproductive Health, Royal Infirmary of Edinburgh, UK

Abstract

Introduction: Infantile myofibromatosis (IM) is a rare mesenchymal disorder characterised by the proliferation of benign tumours in the skin, muscle, bone and viscera with a reported incidence of 1/400,000. It is, however, the commonest fibrous tumour of infancy and childhood, and should be included in the differential diagnosis of any presentation of solitary or multiple skin nodules.

Clinical case: A female infant was born at 37 weeks’ gestation by vaginal delivery to a primiparous mother. Labour was induced for prolonged rupture of membranes. There was no family history of note and antenatal scans were considered normal. Her birth weight was 0.4th centile and head circumference 2nd centile.

She was admitted to the neonatal unit with recurrent dusky episodes that resolved quickly. A paraspinal subcutaneous nodule was incidentally noted at 15 h. By 24 h, further nodules were noted in paraspinal, inguinal and popliteal areas. They were skin-coloured, 1–3 cm diameter, firm and tethered to underlying structures.

Feeds were commenced on day 2 and she passed meconium. Initial milky vomits and nasogastric aspirates progressed to a large bilious vomit on day 3. On abdominal ultrasound volvulus was suspected and a contrast study confirmed duodenal obstruction.

At laparotomy, multiple firm nodular lesions were found throughout the small bowel associated with circumferential narrowing. There were two areas of complete obstruction, and 24 cm of bowel was resected and a stoma formed. There was subsequent multidisciplinary discussion involving the surgical, oncology and pathology departments. Histopathology and immunocytochemistry results were consistent with IM.

She now has a functionally short gut requiring parenteral nutrition. Her maximum feed toleration has been 5 ml per hour and her weight remains below the 2nd centile. Systemic chemotherapy will be considered when she is older unless progression requires earlier intervention, and genetic counselling is planned.

Discussion: This case illustrates the potential for life-threatening complications associated with seemingly benign subcutaneous lumps. It highlights the value of consulting the literature and of taking a multidisciplinary approach with unusual clinical presentations.

Half the patients with IM present in the perinatal period with solitary or multicentric lesions with or without visceral involvement. Nonvisceral lesions show spontaneous regression and have an excellent prognosis. Visceral IM, although only accounting for 25–35% of cases, is associated with significant morbidity and a mortality of up to 76% and usually requires surgery or chemotherapy. Aetiology remains unknown, although autosomal dominant mutations and recessive inheritance have been described.

Blisters and bright spots: highland experience of an Asian epidemic pathogen

M Harvey, K Mackenzie and S Ghayyda

Raigmore Hospital, UK

Abstract

Background: A five-month-old male was admitted with pyrexia, poor feeding and lethargy, requiring stimulation. There was a short history of cough, one vomit and reduced urine output. He had presented four days prior to the out-of-hours GP service and was diagnosed with hand, food and mouth disease. Symptomatic management with antipyretics resulted in initial improvement.

On arrival, he was pale and mottled with a central capillary refill time of 3 s. He was lethargic and responded to pain. There was a fine macular rash on his abdomen. He required volume resuscitation and commenced antimicrobial therapy with ceftriaxone and acyclovir. As he improved, it became apparent he had flaccid paralysis of the right upper limb, predominantly affecting the C5 distribution.

Investigations: Full blood count (FBC) showed thrombocytosis and mild neutrophilia, with subsequent mild lymphopenia. Cerebrospinal fluid (CSF) white blood cell 73 (2% polymorphs) red blood cell 83, glucose 3.1 mmol/l, protein 0.4 g/l. Blood, urine and CSF cultures were negative. Chest X-ray showed no evidence of infection. Nasopharyngeal aspirate polymerase chain reaction (PCR) was positive for Enterovirus, subsequently identified as EV71.

Magnetic resonance imaging (MRI) revealed focal high signal and restricted diffusion at the pontomedullary junction and small bilateral foci of cerebellar enhancement. Repeat MRI on day 10 showed resolution of these findings, with focal high signal within the right cervical cord, extending from C2/C3 to C6.

Clinical course: He progressed well, with recovery of some spontaneous movement in the affected limb. Outpatient review at two months demonstrated further improvement with persisting proximal weakness. Electromyography showed long duration polyphasic potentials consistent with denervation and reinervation, suggestive of ongoing recovery of a lower motor neurone lesion. He remains under outpatient paediatric and physiotherapy review.

Discussion: EV71 is an endemic pathogen with a propensity to cause epidemics, particularly in young children. The most common clinical manifestation is that of ‘hand, foot and mouth disease’ indistinguishable to that caused by Coxsackie virus, which belongs to the same subgroup of the Picornaviridae family. EV71 is also a recognised cause of aseptic meningitis, encephalitis and polio-like paralysis in childhood. Outbreaks have been reported in Europe and Australasia, with frequent epidemics in Japan, Malaysia, Singapore and Vietnam.

This is the first case of EV71 encephalomyelitis in our institution and is of particular importance, given the clinical presentation and MRI findings. Similar cases in this age group and severity of presentation have progressed to require paediatric intensive care unit admission.

Calcium, one to watch post therapeutic hypothermia!

Carolyn Abernethy and Chris Lilley

Neonatal Unit, Princess Royal Maternity Hospital, UK

Abstract

We present the cases of two infants who underwent therapeutic hypothermia for hypoxic ischaemic encephalopathy (HIE) who went on to develop hypercalcaemia. Both infants had evidence of subcutaneous fat necrosis and nephrocalcinosis.

FJ was cooled for 72 h after birth for severe HIE. She went on to develop seizures that were treated with phenobarbitone. She was discharged home at a month of age bottle feeding. FJ was seen at 6 weeks with poor feeding, poor weight gain and increased tone. She was admitted for nasogastric feeding and had routine bloods performed which showed calcium of 4.02 mmol/L. On examination, she had subcutaneous fat necrosis in her cheeks and upper arms. Renal ultrasound showed nephrocalcinosis.

GH was cooled for 24 h for moderate HIE, This was stopped early due to rapid improvement in her condition. She was reviewed by the surgeons for bilious vomiting but had normal contrast. GH established feeds and was discharged home bottle feeding well. She was reviewed in clinic with poor weight gain despite taking volumes >160 ml/kg/day. Routine bloods taken at this point showed calcium 4.06 mmol/l. On examination, she had a very small area of subcutaneous fat necrosis on her back. Renal ultrasound showed nephrocalcinosis.

Both infants were admitted to the neonatal intensive care unit for treatment and monitoring. They were treated with intravenous furosemide and hyperhydration. Both required intravenous pamidronate and low calcium feeds.

Total body therapeutic hypothermia is the gold standard of care for infants with HIE.

Subcutaneous fat necrosis is a rare complication of therapeutic hypothermia and these infants are at risk of developing hypercalcaemia. It is important to examine these infants fully paying particular attention to their skin and if any signs of subcutaneous fat necrosis is found these infants should have their calcium levels monitored. Calcium is monitored regularly during admission in the neonatal unit, but does not tend to be checked prior to discharge. It may be beneficial to measure the blood calcium of these infants prior to discharge and at potentially at first clinic review.

The postgraduate certificate in child health – a method of ensuring coverage of the level 2 RCPCH curriculum

AT MacLaren, S Henderson, E Brincat and DM Tappin

University of Glasgow, NHS Greater Glasgow and Clyde, UK

Abstract

Aims: Paediatric training is split into Levels 1, 2, and 3 with each level having a separate curriculum. Scottish trainees identified the Level 2 curriculum as being poorly addressed in their training. We aimed to address the Level 2 curriculum more robustly by designing a brand new programme to ensure that structured paediatric teaching could be provided to all trainees in the Scotland. 

Methods: To achieve this, we derived intended learning outcomes from the Royal College of Paediatrics and Child Health (RCPCH) curriculum. These were organised into systems that are addressed in five taught sections. Each section involves 30 h of distance learning and 30 h of face-to-face teaching. We have made extensive use of distance learning technologies such as Adobe Connect® and Moodle Virtual Learning Environment®. Formative assessment is used throughout the course with a summative assignment being submitted at the end of each section. Our course gained accreditation from the University of Glasgow as a Postgraduate Certificate (PGCert) at masters’ level. Funding was secured from NHS Education for Scotland allowing the certificate to be provided to all level 2 paediatric trainees in Scotland free of charge.

Results: The first PGCert in Child Health commenced in August 2010. Since starting, 83 trainees have enrolled with only 5 dropping out (6%). All dropouts occurred in the first year of the course. Thirty-nine trainees have, so far, completed the PGCert (47%). Thirty-nine trainees are currently enrolled on the PGCert for session 2013/2014. Feedback from trainees has been very positive with 95% of trainees from 2010 to 2013 agreeing or strongly agreeing that they were happy with the teaching provided.

Conclusion: The PGCert in Child Health is now a well-established component of level 2 training in Scotland enabling robust coverage of the RCPCH curriculum to all Scottish trainees. The use of distance learning technologies has allowed us to deliver a curriculum in a way that is sympathetic to service demands and minimises travelling to and from study days. The feedback from trainees has been extremely positive declaring the education as being of a very high standard.

Reducing antibiotic exposure in the neonatal unit

Christina Lang, Andrew Arnott, Timothy Brook, Alice Horne, William Hurst, Sarah Kelly, Madeleine Payne, Hannah Pert, Natalie Bee, Julie-Clare Becher, Ruth Gibbs, Mairi MacInnes and Judith Orme

The Simpson Neonatal Unit, UK

Abstract

Background: Antibiotics are commonly prescribed for symptomatic newborn infants although the prevalence of early onset sepsis (EOS) is very low. Prolonged antibiotic duration in the face of negative cultures results in exposure to side effects, antibiotic resistance, increased workload, increased stay and in the preterm infant is associated with necrotising enterocolitis and death. An aim of antibiotic stewardship is to reduce unnecessary antibiotics in patients without infection.

Aim: We aimed to evaluate the impact of automatic stop orders (ASO) and C-reactive protein (CRP) on the duration of antibiotics in newborn infants admitted with suspected EOS but who subsequently had negative cultures.

Methodology: Three audits were evaluated over two years in three separate but consecutive time periods. Each audit examined the use of antibiotics in term and preterm infants who were admitted to the Simpson neonatal unit (NNU) for suspected EOS. Where an ASO was used, it was applied on starting antibiotics and limited antibiotic duration to 48 h if cultures proved to be negative and clinical concern was low. Where CRP measurement was performed, this occurred at 36–48 h and antibiotics discontinued if CRP <10 mg/dl, cultures were negative and clinical concern was low.

Audit 1 occurred over six weeks (45 infants) during which ASOs and CRP were not utilised. Audit 2 took place over five months (128 infants) when ASOs alone were policy. Audit 3 took place over five months (104 infants) when both ASOs and CRP were policy. Case notes and electronic records were reviewed and data analysed using Microsoft Excel.

Results: The percentage of all infants receiving >48 h of antibiotics was 55%, 41% and 32% in audits 1, 2 and 3, respectively.

The percentage of all infants receiving a full five days of treatment with antibiotics was 27% and 21% in audits 2 and 3 (not available for audit 1).

In both audits 2 and 3, use of an ASO on admission reduced the number of infants receiving unintended antibiotics (>48 h <5 d) from 47% to 5%.

Conclusion: The use of ASOs effectively prevents infants from getting unnecessary extra doses of antibiotics when the intention is to stop at 48 h, evidenced by the significant reduction in this group of babies when ASO is actually used.

Use of CRP results in less babies overall receiving >48 h of antibiotics in the face of negative cultures, particularly those receiving five days.

Congenital nasal pyriform aperture stenosis and pituitary abnormalities: case series of 20 patients and a management guideline for early identification of pituitary insufficiency

SC Chen¹, H McDevitt², WA Clement³, D Wynne³, A Mason¹, M Donaldson¹, SF Ahmed¹ and MG Shaikh¹

¹Paediatric Endocrinology, Royal Hospital for Sick Childen, UK

²Neonatology, Royal Hospital for Sick Children, UK

³Paediatric Otolaryngology, Royal Hospital for Sick Children, UK

Abstract

Introduction: Congenital nasal pyriform aperture stenosis (CNPAS) is an increasingly recognised cause of upper airway obstruction secondary to bony overgrowth of the nasal process of the maxilla. It is a separate entity from choanal atresia. Clinical features of airway obstruction include tachypnoea, episodes of cyanosis or apnoea, nasal congestion, poor feeding and resistance felt whilst passing a nasogastric tube. Diagnosis is confirmed with craniofacial computed tomography imaging. CNPAS is associated with holoprosencephaly, of which solitary median maxillary central incisor (SMMCI) is the least severe form. Studies have described pituitary abnormalities in up to 40%.

Objective: We aimed to determine the use of baseline endocrine investigations and magnetic imaging resonance (MRI) brain in assessing endocrine dysfunction.

Method: Retrospective case note review of patients diagnosed with CNPAS between 2000 and 2013 in a tertiary paediatric unit.

Results: Twenty patients (13 F:7 M) were identified. Sixteen were diagnosed during the neonatal period at median (range) age 10 (1–28) days of whom 13 needed surgical correction; while 4 patients diagnosed later at age 2, 6, 11 and 60 months were all managed conservatively. SCMMI was detected in 12 (60%) patients.

Baseline endocrine investigations were performed in the neonatal period in 11/20 and MRI brain in 12/20 patients with 7/20 having both. Hypoplastic/ectopic posterior pituitary was identified in one patient, who was also found to have panhypopituitarism. Two patients were referred later for evaluation of short stature and investigated at age three and five years, of which one had an ectopic posterior pituitary together with abnormal baseline endocrine function (insulin-like growth factor 1). The other patient had normal pituitary on MRI but was also diagnosed with growth hormone deficiency.

Available height standard deviation score (SDS) data at one year on 60% of our patients identified both the late-diagnosed growth hormone deficient patients, with SDS of −2.6 and −3.6, respectively.

Conclusion: CNPAS management requires a multispeciality and consistent approach in evaluation of the endocrine axis. All CPNAS patients at diagnosis should have MRI brain and baseline endocrine investigations that will allow early recognition and treatment of pituitary insufficiency, minimising surgical risks. Growth monitoring for at least one year is recommended as height SDS at one year is a good predictor for pituitary function.

Polysomnography in children with achondroplasia under the age of 5 within one tertiary centre

Natalie Russell, Helen McDevitt and Phillip Davies

Southbank Child Development Centre, UK

Abstract

Background: Achondroplasia is the most prevalent form of skeletal dysplasia.¹ Up to 85% of children with achondroplasia will suffer with sleep-related breathing disorder (SRBD)² and the central apnoeas that occur are related to sudden infant death.³ The American Academy of Paediatrics advises screening for SRBD by polysomnography before one month of age.⁴ The Royal College of Paediatrics and Child Health advise that children with respiratory symptoms or abnormal sleep studies be reviewed by a respiratory paediatrician.⁵

Objectives: To assess whether patients with achondroplasia within a tertiary centre are having respiratory investigation in concordance with current standards of practice.

Population: All patients under the care of one tertiary centre under the age of five years. Patients under the age of five years with ‘other skeletal dysplasias’ were also audited for comparison of results.

Methods: Review of casenotes and clinical information databases to gather information on number of children having sleep studies, age at initial sleep study, results, subsequent management and whether there had been respiratory review.

Results: Ten children with achondroplasia were identified. Nine patients had a sleep study, occurring at a median age of eight months (range 1–37 months). Five patients had respiratory symptoms. Five had polysomnography and four had only saturation study or transcutaneous oxygen and carbon dioxide monitoring. Seven patients had an abnormal first study. Four children required subsequent active management and three are undergoing a ‘watchful wait’. Four patients were seen by the respiratory team at a median age of 8.5 months (1–36 months). Three children with other skeletal dysplasias were identified. Two children had a polysomnography occurring at a median age of 8.5 days (range 3 days to 2 weeks). Both studies were abnormal and both children required further active management. One patient was reviewed by the respiratory team.

Conclusions: Ninety percent of patients with achondroplasia under 5 have had a sleep study at a median age of eight months. One of these patients presented with an apparent life-threatening event at two months of age, prior to sleep study, and required emergency foramen magnum decompression. Of the studies that were performed, a high number were abnormal with children requiring further active management. To protect against the ill-effects of SRBD, it is recommended that children have initial screening within the first month of life and that there is liaison with the respiratory team when there are respiratory symptoms or if there is abnormal sleep study.

What is the knowledge and experience of trainees in RHSC Glasgow in paediatric lumbar puncture?

Sandy Kirolos, Iona Morgan, Fiona Russell and Marie Spiers

Emergency Department, Royal Hospital Sick Children, UK

Abstract

Aim: Lumbar puncture (LP) is a common procedure performed in children, and is routinely indicated as part of a septic screen in neonates and infants below the age of three months. The aim of this project was to determine the range of knowledge and experience of trainees in the Royal Hospital for Sick Children (RHSC) Glasgow regarding paediatric LP, with a view to producing guidance to improve practice.

Method: Surveys were distributed among paediatric trainees, advanced nurse practitioners and EM trainees, at varying stages of training (from ST1 to ST8), working within the acute receiving unit and Emergency Department during their training. Information obtained included number of paediatric LPs performed, whether any formal training had been provided and questions looking into the details of carrying out the procedure specific to paediatrics. The results were then collated and used to create a document to provide guidance on the basics and safety aspects of the procedure.

Results: Over a one-week period, 27 trainees in total responded to the survey. Eighty-one percent were trained via observation with no formal training or reference source, and 15% had no training at all.

All those asked placed the child in the lateral position and identified appropriate cleaning solution. Eighty-four percent were aware that a minimum of two assistants are required. Only 24% would use local anaesthetic or analgesia. Fifty-six percent did not know what size spinal needle to use and 33% did not replace the stylet prior to removing the needle. Seventy-eight knew that three universal containers are needed and 44% were aware that 10 drops per container should be collected if possible. Forty-eight would recommend bed rest following paediatric LP.

Conclusion: The survey has shown that there is a wide variation in knowledge and practice among trainees of different stages. It also shows a general lack of training and standardisation for many aspects of the procedure. The main areas where practice was inconsistent related to the use of analgesia, the appropriate size of spinal needle and how many drops of cerebrospinal fluid (CSF) are required for basic tests performed as part of a septic screen. Accordingly, there is a need for written guidance as a resource for trainees who lack experience in paediatric LP. This will allow standardised technique thus ensuring patient safety. Repeat surveying of trainees is planned following the introduction of guidance.

Single centre experience of using a new intravenous iron therapy formulation in children with anaemia secondary to inflammatory disease

Callum G W Taylor¹, Vikki Garrick², Andrew Barclay², Richard Hansen², Paraic McGrogan² and Richard K. Russell²

¹University of Glasgow, College of Medical, Veterinary and Life Sciences, UK

²Department of Paediatric Gastroenterology (NHSGGC), Yorkhill Hospital, UK

Abstract

Objective: Anaemia is a common complication of chronic diseases including inflammatory bowel disease (IBD); it affects ∼70% of children at diagnosis of IBD.1 The associated symptoms of fatigue and poor concentration have a large impact on patient's lives. Historically oral iron therapy has often been poorly tolerated and intravenous preparations practically difficult to give.2 The purpose of this study was to summarise the initial experience of using single-dose intravenous ferric carboxymaltose (Ferinject®, Vifor Pharma) in terms of efficacy and safety in children.

Population and methods: A cohort of 18 IBD patients (17 Crohn's disease (CD) 59%, 6 ulcerative colitis 35%, 1 inflammatory bowel disease unclassified (IBDU) 6%) from Royal Hospital for Sick Children, Glasgow. Between 2012 and 2014, these patients received a total of 21 infusions (three patients received two infusions in the two-year period). Baseline full blood count (FBC) data were collected plus follow-up at two points, early (mean: five weeks) and late (mean: 15 weeks). Patients received a single weight based dose of 20 mg/kg over a 20 min period. Data were analysed using paired t-tests for normally distributed haematology data and Wilcoxon Signed-Rank tests for the nonnormally distributed ESR.

Results: Baseline haematology (Haemoglobin (Hb), mean cell volume (MCV)) was recorded prior to infusion; mean Hb was 98.6 g/dl, and MCV 78.6 fl. Data existed for 13 infusions at early follow-up, and for 15 infusions at late follow-up. At early follow-up, mean Hb was 123.5 g/dl (p < 0.0001) and MCV 86.3 fl (p = 0.008). At late follow-up, mean Hb was 115.33 g/dl (p < 0.001) and MCV 86.5 fl (p = 0.016). Erythrocyte sedementation rate (ESR) significantly decreased from a median of 32 mm/Hr at baseline to 10 mm/Hr at early (p = 0.026) and 14 mm/Hr at late follow-up (p = 0.035). One patient had a flushing episode 10 min following their infusion which self-resolved without any specific action. One patient was hospitalised due to an exacerbation of CD the day following their infusion. At early follow-up, 10 of the 13 patients had resolution of anaemia. At late follow-up, 9 of the 15 patients had resolution.

Conclusions: Intravenous ferric carboxymaltose was well tolerated and resulted in a significant improvement of Hb with resolution of anaemia in 77% of cases at early follow-up, and 60% at late. Further research will investigate how this improvement corresponds with patient symptoms, whether a larger patient group develops any adverse effects, and will also examine the outcomes of iron infusions in other patient groups.