Abstracts from the Scottish Paediatric Society St Andrew’s Day Paediatric Symposium: Royal College of Physicians of Edinburgh,Edinburgh,Friday 27 November 2015
Available accessResearch articleFirst published online May, 2016
Abstracts from the Scottish Paediatric Society St Andrew’s Day Paediatric Symposium: Royal College of Physicians of Edinburgh,Edinburgh,Friday 27 November 2015
Trends in acute medical admissions to hospitals in Scotland 2000–2013 – transformation before our eyes
Maryam Al-Mahtot, Rebecca Barwisemunro and Steve Turner
Child Health, Royal Aberdeen Children’s Hospital, UK
Background: There are increasing pressures on hospital inpatient services but relatively little is known regarding trends in acute medical paediatric admissions. The aim of this study was to describe if and how the characteristic of children admitted to acute medical paediatric units hospital in Scotland have changed between 2000 and 2013. The aim of a wider programme of research was to identify how any increased demand on paediatric services might be managed in future.
Methods: All admissions of individuals aged up to 16 years to Scottish hospitals between 1st January 2000 and 31st December 2013 were provided by the Information Services Division of the Scottish Government. All the relevant governance approvals were obtained. Acute admissions to medical paediatrics were extracted and analysed.
Results: There were 574,043 admissions, the median age was 2.2 years and 56% were male. There was a 41% increase in the number of admissions between 2000 and 2013 and this rise was wholly explained by a rise in admissions which were discharged on the same day; there was a 15% fall in the number of children discharged ≥ one day after being admitted. There was no evidence of a stepwise increase in admissions during 2004 when A&E waiting times were set at 4 h and the Out-of-Hours Service was established. The mean duration of stay fell in a linear manner from 1.9 days in 2000 to 1.0 days in 2013. The proportion of children readmitted in the same calendar month was 8% throughout. The following characteristics remained stable over the period of interest: mean age, socioeconomic distribution and most common diagnoses. Overall, 27% of admissions were from the least affluent quintile and 14% from the most affluent quintile. Twelve diagnoses explained 49% of all acute admissions and were gastroenteritis, unspecified viral infection, tonsillitis, croup, acute upper respiratory tract infection, bronchiolitis, acute lower respiratory tract infection, asthma, urinary tract infection, wheeze, nausea and vomiting, febrile convulsion.
Conclusions: The management of children with acute medical paediatric conditions has changed in Scotland. Within the limitations of the data available, the characteristics of children and their conditions remain essentially unchanged and the need is greatest among our poorest communities. These changes may be explained by shifts in parent health-seeking behaviour, thresholds for referring children to hospital and inpatient management of children.
Rising incidence of classical and non-classical coeliac disease in Lothian
Martin F Lister, Peter M Gillett and Naomi Fulton
Department of Paediatric Gastroenterology and Nutrition, Royal Hospital for Sick Children, UK
Background: The incidence of coeliac disease is increasing in many countries, including Scotland.
Objectives: We aimed to update our previous research1,2 into the incidence and presentation of coeliac disease in Southeast Scotland.
Population: All patients under 16 years of age diagnosed with coeliac disease in Lothian in the five years from 1st January 2010 to 31st of December 2014.
Methods: All positive anti-tissue transglutaminase results from Lothian are reported to the clinical lead for the regional coeliac service, at the RHSC in Edinburgh and followed up. Depending on their level of antibody and symptoms, most will have their diagnosis confirmed with endoscopy and biopsy. A retrospective cohort study of electronic case records of coeliac disease diagnoses was performed. The number of serology tests requested in under 16s in the region was also assessed as a proxy for increasing awareness.
Results: A total of 168 patients (117 female, 51 male) were identified in Lothian over the study period. The total number diagnosed in 2010 was 21, 2011: 29, 2012: 34, 2013: 35 and 2014: 49. The incidence has more than doubled from 2010 to 2014 (14.4 to 33.7/100,000, respectively). Classical cases3 rose from 8.2 to 19.2/100,000. Non-classical cases rose from 4.8 to 12.4/100,000. Although there was an overall increase in serology tests requested (2274, 2556, 2617, 2984 and 3489 in 2010, 2011, 2012, 2013 and 2014, respectively), the proportion of positive tests also rose. Median age at diagnosis was eight years (range 1–15 years).
Conclusions: The incidence of both classical and non-classical presentations of coeliac disease continues to rise in Lothian. The proportion of positive results is increasing confirming a true rise in the incidence of coeliac disease. Better awareness and a lower threshold to test are clearly good. Comparison once again with other areas of Scotland would be a key piece of information for clinicians and Coeliac UK to help target awareness campaigns and improve diagnosis.
References
WhiteLEMerrickVMBannermanE. The rising incidence of celiac disease in Scotland. Paediatrics2013; 132: 923–924.WhiteLEBannermanEMcGroganP. Childhood coeliac disease diagnoses in Scotland 2009–2010: the SPSU project. Arch Dis Child2013; 98: 52–56.LudvigssonJFLefflerDABaiJC. The Oslo definitions for coeliac disease and related terms. Gut2013; 62: 43–52.
Implementation of the 2012 NICE early onset neonatal sepsis guideline: a Glasgow experience
S Kirolos, J Mitchell, J Burgess-Shannon, N Smee, K Mcmanus, C Cockburn, C Abernethy, A Powls and L Jackson
Neonatal Unit, Princess Royal Maternity, UK
Introduction: Early onset neonatal sepsis commonly presents in the first 24 h of life and is associated with high morbidity and mortality. Where maternal intrapartum antibiotics remain important in the prevention of early onset neonatal sepsis, postnatal observation for infants, with or without antibiotics, has also been shown to reduce adverse outcomes.
The 2012 NICE EOS guideline introduced the neonatal early warning score for monitoring infants at risk of sepsis. Changes in practice included indications for commencing IV antibiotics and reduction in length of time on treatment as guided by clinical condition, infection markers and blood culture results. It also emphasised parents as partners in care and providing written information to those with an infant at risk of early onset neonatal sepsis.
Aim: We aimed to implement the NICE EOS guideline in two Glasgow maternity hospitals. Following implementation, we identified areas where adherence could be optimised.
Method: Daily sessions were held for two weeks to educate medical and nursing staff on the new guidance. Over the first four months of implementation, all infants on the postnatal wards on a neonatal early warning score chart alone or on antibiotics were included. Information regarding identification of infants at risk of sepsis, indications for antibiotics and appropriate discontinuation of antibiotics was collected. Neonatal early warning score charts were analysed to determine the adequacy of observations and appropriate review of infants.
Results: The guideline was implemented in November 2014 in the Princess Royal Maternity and April 2015 in the Southern General Maternity. The average number of infants on neonatal early warning score charts per month was similar between units, as were indications for observation and antibiotics. Overall results showed no increase in length of time on antibiotics, length of stay or number of lumbar punctures performed. Areas identified for improvement included achieving three hourly observations for at least 24 h and documentation of parental discussion. No infants during the study period became unwell with sepsis when on neonatal early warning score chart observation alone.
Discussion: Implementation of this guideline involved educating staff who care for infants at risk of early onset neonatal sepsis. No infants came to harm due to sepsis from changes to guidance or indications for IV antibiotics. The increased length of stay and increased number of lumbar punctures performed, reported by English units where this guideline was implemented, were not seen during this study period. This has a significant impact on maternal bed occupancy in busy tertiary units. Further education is required to ensure awareness and continued adherence to this guideline.
Determining the pattern and prevalence of excessive alcohol consumption in pregnancy
Glasgow by measuring biomarkers in meconium
Carolyn Abernethy, Karen McCall, Gail Cooper, Donetta Favretto, E Bertol, David Young and Helen Mactier
Princess Royal Maternity Hospital, UK
Background: Pregnant women commonly under-report alcohol consumption. The half-life of maternal biomarkers is short, therefore accurate data for developing and monitoring public health strategies are not available. Ethanol crosses the placenta and is partly conjugated in the fetus to fatty acid ethyl esters and ethyl glucuronide, which are deposited in meconium. Raised fatty acid ethyl esters (>600 ng/g) and ethyl glucuronide (>30 ng/g) levels in meconium are indicative of alcohol consumption in pregnancy.[1,2] Recent evidence suggests ethyl glucuronide may be a more useful biomarker.[2]
Aims: To assess the feasibility of fatty acid ethyl esters and ethyl glucuronide measurement in meconium as an estimate of alcohol consumption in pregnancy and to explore relationships between these biomarkers and demographic factors including maternal age, parity, smoking, ethnicity and socio-economic status.
Methods: A sample of meconium was sought from every infant ≥ 36 weeks’ gestation born every eighth day at Princess Royal Maternity, Glasgow. Newly delivered mothers were asked to retain the first meconium nappy. If retrospective written, informed consent was given, the sample was frozen and analysed for fatty acid ethyl esters and ethyl glucuronide concentration (University of Firenze and Padova, Italy).
Results: Two hundred and thirty-five samples of meconium were obtained (67% of eligible babies). Fatty acid ethyl esters were detected in all. Ninety-eight (42%) samples had fatty acid ethyl ester concentrations >600 ng/g, including four samples with fatty acid ethyl esters below the limit of quantitation (10 ng/g). Ethyl glucuronide was detectable in 93 (40%) samples. Twenty-three (10%) had ethyl glucuronide levels < limit of quantitation and 35 (15%) had ethyl glucuronide levels >30 ng/g. No mother reported heavy alcohol consumption in pregnancy. Fatty acid ethyl ester was weakly correlated with ethyl glucuronide (Pearson 0.327; p < 0.001). There was no correlation between either biomarker and birth weight, head circumference, maternal age, parity, smoking, ethnicity or postcode.
Conclusions: The prevalence of raised alcohol biomarkers in meconium in Glasgow is significant and similar to that reported from other studies. Further investigation of this is required.
References
JoyaXFrigulsBOrtigosaS. Determination of maternal-fetal bio-markers of prenatal exposure to ethanol: a review. J Pharm Biomed Anal2012; 69: 209–222.HimesSKDukesKATrippT. Clinical sensitivity and specificity of meconium fatty acid ethyl ester, ethyl glucuronide, and ethyl sulfate for detecting maternal drinking during pregnancy. Clin Chem2015; 61: 523–532.
Generously funded by Scottish Government
Seasonal and weekday variations in acute medical admissions to hospitals in Scotland 2000–2013 – shall we wait until Monday?
Rebecca Barwisemunro, Maryam Al-Mahtot and Steve Turner
Child Health, Royal Aberdeen Children’s Hospital, UK
Background: There are increasing pressures on hospital inpatient services but relatively little is known for acute medical paediatric admissions. The aim of this study was to describe day-by-day variation in the number of children admitted to acute medical paediatric units hospital in Scotland between 2000 and 2013. The aim of a wider programme of research was to identify how any increased demand on paediatric services might be managed in future.
Methods: All admissions of individuals aged up to 16 years to Scottish hospitals between 1st Jan 2000 and 31st December 2013 were provided by the Information Services Division of the Scottish Government. All the relevant approvals were obtained. Acute admissions to medical paediatrics were identified. Admissions were grouped by month and day of week. The time of admission for 2013 and number of admissions during holidays were identified for NHS Grampian.
Results: There were 574,043 admissions, the median age was 2.2 years and 56% were male. The busiest day of the week was Monday (16% of admission) and the quietest was Saturday (12% of all admissions). The proportion of children readmitted after admission Mon–Fri was 7.8% and was 7.3% for weekends. Children admitted on Friday (44%) were most likely and Sunday admissions were least likely to be discharged on the same day as admitted (40%). Whilst admissions with upper respiratory tract infection were more common on Mondays and least common on Saturdays, admissions with febrile convulsions and meningitis were evenly distributed across all days of the week. In NHS Grampian, public holidays which fell on weekdays experienced weekend-like activity and admissions reached a peak at 11 am which persisted to 11 pm.
Conclusions: As a whole, the number of presentations to acute medical units in Scotland, and possibly their management, differs on weekdays and weekends. Presentations with more serious conditions do not follow this pattern. The reasons for these patterns are likely to be complex and include parents adopting “watchful waiting” at weekends and the restriction in primary and secondary care services at weekends.
Audit on vitamin D levels in children with spina bifida
Cathryn Cheng, Lindsay Cosgrove, Tallur, Paul Eunson, Mark Gaston, Kandasamy, Jimmy Lam and Emma Moore
Paediatric Neurology Department, Royal Hospital for Sick Children, UK
The authors carried out an audit on vitamin D levels in patients with spina bifida at the Royal Hospital for Sick Children in Edinburgh from September 2014 to August 2015.
Vitamin D is essential for good bone health, which is important for children with spina bifida who can have significant neuromuscular weakness and immobility and fractures may go unnoticed due to lack of sensation.
The objectives of the audit were to determine vitamin D status in children with spina bifida, to determine the adherence to local vitamin D treatment guidelines if the levels were low and to determine any clinical correlation.
A total of 60 children with spina bifida were scheduled to attend the monthly spina bifida clinic at the Royal Hospital for Sick Children between September 2014 and August 2015. Their data including age, gender, underlying diagnosis, level of mobility, comorbidities, history of fracture as well as vitamin D levels and treatment recorded were collected retrospectively from their medical records on the Trak computer system.
The children in the audit were between five months old and 19 years of age. Thirty-two were female and 28 male. Five of the 60 patients did not attend. Of the 55 patients who did attend, 29 had vitamin D levels taken. According to local guidelines, two were deficient (range less than 25 nmol/l), 16 were insufficient (range 25–50 nmol/l), 10 were adequate (range over 50 nmol/l). One sample was unsuitable for analysis.
As regards treatment, one of the two patients in the deficient range and 13 of the 16 in the insufficient range were offered treatment.
Nine patients were documented as having had a previous fracture, including one from birth trauma. Three of the patients had not had a vitamin D level, five had levels in the insufficient range and one in the adequate range.
This audit highlights the importance of vitamin D for bone health in children with spina bifida. Out of the 29 patients who had vitamin D levels taken, 18 (i.e. 62%) were found to be low. Although the numbers are too small to draw any statistical conclusions, it is interesting to note and may be clinically significant that out of the six patients with spina bifida who had vitamin D levels done and who had been documented as having had a previous fracture, five had low levels.
Abstracts for clinical presentations
Withdrawal of ventilator care at home in a neonate – a case report
Prakash Loganathan1, Simpson Judith1, Paul Boutcher2 and Allan Jackson1
1NHS Greater Glasgow & Clyde, UK
2Children’s Hospice Association of Scotland-Robin House Hospice
Introduction: There are approximately 800,000 live births in the United Kingdom each year and of these nearly 2500 die within the first month of life.1 Many of these infants are born with a life-limiting condition and are likely to require palliative care.2 An important consideration in the provision of this care is to identify the optimal environment for the family. Here we report the withdrawal of ventilator support at home in a neonate.
Case Report: CP was a term baby with Trisomy 13 complicated by gastroschisis, intestinal atresia and bowel perforation. The neonatal and surgical teams met with the family to explain the life-limiting nature of this condition. Following these discussions his parents opted for active management with the aim of taking their baby home enterally fed. To this end CP underwent a laparotomy with primary closure of gastroschisis, excision of atresia and over sewing of perforation on day one of life. It was not possible to restore bowel continuity during his surgery; however, this was achieved following a second planned laparotomy aged six weeks. Unfortunately he deteriorated following this and extensive perforated necrotising enterocolitis was identified at a third laparotomy. His parents were counselled that ongoing intensive care in this situation was futile and they expressed a strong desire for his end of life care to take place at home. This case report describes the planning and elaborates the processes involved in facilitating the home withdrawal of ventilator support.
A family with steroid-responsive autism
Joe Symonds1, Sameer Zuber1, Margaret Wilson1, Kerstin Bumke1, Liam Dorris1 and Gavin Cobbs2
1Fraser of Allander Neurosciences Unit, Royal Hospital for Children, UK
2Caird House, NHS Lanarkshire, UK
Summary: We present a family of three siblings. The older two had been given diagnoses of autism by the time the youngest presented with epileptic seizures. Investigation of the youngest sibling revealed a highly abnormal electroencephalogram which was characterised by electrical status epilepticus in slow wave sleep. The older siblings then had electroencephalograms which were also highly abnormal. All three children demonstrated an improvement in language and behaviour when treated with oral prednisolone.
Index case: The index case, a five-year-old boy, presented in December 2014 with a nocturnal convulsive epileptic seizure lasting approximately 1 min. Four limb jerking was accompanied by drooling from the corner of his mouth. The following morning he was noted to be very different. He was not speaking and was described by his mother as being unusually “quiet and reserved.” A 24 h ambulatory electroencephalogram was arranged. This showed very frequent epileptic activity and electrical status epilepticus in slow wave sleep. He was treated with four weeks of oral prednisolone. He gradually regained his previous language ability. Repeat electroencephalogram two months later demonstrated a clear improvement, with no evidence of electrical status epilepticus in slow wave sleep.
Older siblings: Older brother of the index case, aged nine, had been diagnosed with autism at the age of four. Having gained language skills up to the age of two years he went through a period of regression in speech and behaviour. Since then gradually gained language skills and was able to speak in three to four word sentences at the age of nine. Electroencephalogram at the age of nine was near identical to that of his younger brother. He was also treated with oral prednisolone. Both electroencephalogram and language ability improved markedly.
An older sister of the index case, aged eight, had been diagnosed with autism at the age of five. At the age of two she could say several people’s names, several colours and could sing ‘twinkle twinkle little star’. She then lost all expressive language. Electroencephalogram at the age of seven was more abnormal than her brothers’ and also showed electrical status epilepticus in slow wave sleep. After treatment with oral prednisolone the electroencephalogram improved markedly and she started to say the names of her brothers.
Message: We would like to use this case to highlight the importance of considering electrical status epilepticus in slow wave sleep as a cause of ‘autistic regression’. We would like to discuss the increasing evidence, particularly from genetic studies, supporting a theory of shared neurobiology between epilepsy and autism.
Ebola Virus Disease – challenges in the management of paediatric cases
Rod Kelly
Neonatal Transport Office; Scotstar – Neonatal Transport Team, UK
The Ebola epidemic in West Africa was described by the World Health Organisation as the ‘most severe acute public health emergency seen in modern times’.1 Mortality rates in children were high: 90% in infants under one year and 80% in children between the ages of one and four years, compared to 65% for children older than 15 and adults.2 Over 12,000 children were left orphaned in Sierra Leone.3 Despite the high mortality rates and high incidence of child safeguarding issues, doctors and nurses with paediatric experience were under-represented in those volunteering for deployment.4
As part of the NHS response organised by UK-Med on behalf of DFID (Department for International Development), I was deployed to Save the Children’s Ebola Treatment Centre (ETC) in Kerry Town, Sierra Leone.
This case report describes the clinical course of a six-year-old boy with Ebola virus disease, to illustrate the challenges encountered when working in the ETC and in survivor clinics in a resource deplete setting, as well as some of the positive aspects of volunteering.
TB or not TB: A rare case of disseminated oligodendroglial cell-like leptomeningeal tumour disguised as tuberculous meningitis
Anthony Wiggins, Jothy Kandasamy, Chandrasekaran Kaliaperumal, Pasquale Gallo, Paul Eunson and James Ironside
Department of Neurology and Neurosurgery, Royal Hospital for Sick Children
Introduction: Tuberculosis masquerading as malignancy is well documented. We present an unusual case of a disseminated oligodendroglial cell-like leptomeningeal tumour, which was initially treated as tuberculous meningitis.
Case: A six-year-old boy presented to his optician with a new squint. He was urgently reviewed in hospital with papilloedema, a right abducens palsy and a partial left facial nerve palsy. He was otherwise asymptomatic.
MRI of his brain demonstrated hydrocephalus and widespread nodular meningeal enhancement around the brainstem, consistent with tuberculous meningitis. A ventricular access device was inserted and small numbers of acid-fast bacilli were seen on CSF microscopy. Spinal MRI showed intramedullary lesions at T10–T11; consistent with tuberculomas.
He commenced anti-tuberculosis therapy and steroids, but remained dependent on CSF drainage and required a ventriculo-peritoneal shunt. After some improvement in his cranial nerve palsies, he was discharged home.
There was no growth on TB culture from multiple CSF samples. CSF cytology was negative and other tests for TB were also negative. Unfortunately he developed a sub-dural haematoma which required surgical evacuation. An intra-operative dural biopsy was normal.
He remained well but there was radiological disease progression despite ongoing anti-TB therapy. After eight months an open cauda equina biopsy was performed. Neuropathological analysis diagnosed an oligodendroglial cell-like leptomeningeal tumour, with immunoreactivity for S100 protein and synaptophysin in the tumour cells. His anti-TB treatment was stopped and he was commenced on chemotherapy. An MRI at three months has demonstrated some response to treatment.
Discussion: This type of tumour has only been described recently, with a small number of case reports that match the clinical, radiological and histological findings as this case. Patients present aged 2–7 years old with mild symptoms in relation to the extent of their disease, and progression is often slow. Hydrocephalus and cranial nerve palsies are common and classic radiological appearances are that of ‘bubbly’ enhancement of the meninges, particularly in the posterior fossa, and intramedullary spinal cord lesions. The immunohistochemistry was in keeping with this entity and there was no chromosome 1 p/19q co-deletion. Although the imaging findings were typical for tuberculosis, the lack of treatment response led to further investigations. The positive CSF AFB and negative initial dural biopsy were also misleading.
Conclusions: This case represents an example of an extremely rare and only recently described tumour. It highlights the importance of ongoing diagnostic scepticism when a condition does not respond to conventional therapy.
Biotin-thiamine-responsive basal ganglia disease: a rare but important cause of encephalopathy
Jed Bamber, Claire Cockburn and Tong Yeo
Fraser of Allander Neurosciences Unit, Royal Hospital for Children
Introduction: Biotin-thiamine-responsive basal ganglia disease is a rare genetic neurometabolic disorder. It is characterised by subacute encephalopathy with confusion, seizures, dysarthria and dystonia following a history of febrile illness. If left untreated, the disease can progress to severe quadriparesis and even death.
Case Description: An eight-year-old boy of non-consanguineous Indian heritage presented with a two-day history of progressively unsteady gait, whole body tremors, confusion and dysarthria following a coryzal illness. On examination he had almost continuous whole body tremor. Tone was generally increased with cogwheel rigidity, upper and lower limb hyperreflexia, and bilateral ankle clonus. Signs of autonomic dysfunction with fluctuations in blood pressure and diaphoresis were also present. First-line investigations including infection markers, CXR and ECG were normal. MRI Head revealed symmetrical, bilateral T2 signal abnormality with markedly swollen caudate nuclei and putamen. He was started on intravenous methylprednisolone for three days and high dose biotin (10 mg/kg/day) and thiamine (600 mg/day) via nasogastric tube. He remained nasogastrically fed for one week by which time he had made good clinical progress and was able to restart oral feeding. Symptomatic dystonia improved with oral baclofen. Since discharge, he has returned to baseline and is back at school. While awaiting confirmation of underlying gene mutation, investigations for mitochondrial disorders including skin and muscle biopsies have been performed and are normal. Following discharge his CSF thiamine result has returned as low.
Discussion: MRI findings of bilateral T2 signal abnormality in the caudate nucleus and putamen are typical of biotin-thiamine-responsive basal ganglia disease. These findings combined with low CSF thiamine and his clinical progress on treatment support a diagnosis of biotin-thiamine-responsive basal ganglia disease. Biotin-thiamine-responsive basal ganglia disease was first described in a case series of 10 patients of middle eastern origin in 1998, each presenting with similar clinical features of encephalopathy, dysarthria and dystonia that responded to treatment with biotin and thiamine. An autosomal recessive mutation of SLC19A3 gene on chromosome 2 has since been attributed as the cause of biotin-thiamine-responsive basal ganglia disease. SLC19A3 codes for hTHTR2, a thiamine transporter. Thiamine is an important co-factor in the production of neurotransmitters and myelin in addition to carbohydrate and lipid metabolism. Administration of biotin and thiamine early in the disease course results in partial or complete improvement within days. Biotin-thiamine-responsive basal ganglia disease should be considered in any child presenting with features of encephalopathy and high T2 signal in the caudate and putamen on MRI.
‘Think, test, telephone – but first we must get patients to attend’
Sarah Alexander, Louise Bath, Harriet Miles and Kathryn Noyes
Diabetes Dept, RHSC
On 26th March 2015 a previously well 15-year-old boy was carried into Accident and Emergency by his foster father. He had agonal breathing with oxygen saturations of 97%. His heart rate was 77 bpm and blood pressure unrecordable. He was cold and mottled with a GCS of 3. He had tonic posturing of his upper limbs with eye deviation. His temperature was 31°C, blood glucose 25 and ketones 5.7. A blood gas showed a pH of 6.6, pCO2 8.5 and BE-32.
There was a proceeding four-day history of headaches and lethargy with an acute 12 h history of being ‘vacant’ and having been incontinent.
He was managed acutely with rapid sequence induction and ventilation. He received Lorazepam, fluids and antibiotics. A CT head demonstrated a swollen brain with venous sinus thrombosis. He was admitted to Intensive Care and required inotropic support and an intravenous insulin infusion.
Bloods demonstrated an acute kidney injury for which he required haemofiltration for four weeks. An ECG demonstrated a broad complex rhythm with ST segment changes and an Echo showed reduced cardiac function with a mildly dilated left ventricle.
There was significant neurological concern on first weaning sedation. An MRI scan showed changes initially thought to be in-keeping with severe hypoxic ischaemic injury; however, sedation was successfully weaned and he successfully extubated. Further reviews of the MRI scan have suggested changes could be longstanding from the neonatal period or secondary to a seizure at presentation. The patient has made a remarkable recovery.
Ongoing problems at discharge were significant but continue to improve. He had hypertension, left upper limb weakness and paraesthesia and has subsequently suffered two unexplained fractures to bones in the left hand. His cardiac function continues to recover and he was found to have short stature, a delayed bone age and low vitamin D level. He has ongoing Renal, Cardiac, Endocrine, Diabetes, Orthopaedic and Neurology input. His outpatient diabetes management continues to go well and his most recent HbA1c is 53.
Diabetes can present insidiously over weeks to months with symptoms as nonspecific as lethargy, low mood and irritability. Primary care health campaigns encourage General Practitioners to ‘think, test, telephone’; however, children and young people continue to present in severe life-threatening diabetic ketoacidosis. There is a clear need for ongoing primary care and concurrent public awareness campaigns.
