Are routine investigations in acute paediatric admissions justified?

Aim: To assess the appropriateness of investigations and their impact on management of acute paediatric admissions. Method: All investigations performed within the first six hours in all consecutive paediatric admissions (n=138) over a 6 week period, in a teaching general hospital, were recorded retrospectively and the results were analysed for normality/ abnormality and effect on patient management. Results: Out of a total of 480 emergency investigations performed on 89 patients, the complete blood count, electrolytes and chest X-rays were the three investigations most likely to be deranged and influenced management in 5% (n=3), 25% (n=8) and 46% (n=6) of subjects with an abnormal result, respectively. Impact on management was more evident on analysing a subgroup admitted with mild gastroenteritis, in whom serum electrolytes were abnormal in 50% (n=19), and 42% (n=8) of these needed a change in their management. Conclusion: Rationalising the number of investigations in acute paediatric admissions would result in less discomfort to children and in a significant cost benefit.peer-reviewe

Is 24 hour observation in hospital after stopping intravenous antibiotics in neonates justified?

Background: Antibiotics are given empirically for suspected sepsis in up to 75% of neonates on the Neonatal and Paediatric Intensive Care Unit (NPICU), after completion of a septic screen. Treatment is discontinued on day 3 if cultures remain negative or after 7-14 days with proven sepsis and, until recently, these neonates are then observed for an additional period of 24 hours before being discharged from hospital. Aim: To assess whether the 24 hour observation period after stopping antibiotics is clinically justified and, if not, whether neonates can be discharged safely on the same day when antibiotics are stopped. Methods: A consecutive sample of 95 babies admitted to NPICU, and who received antibiotics, from December 2006 to January 2008 were analysed prospectively. Their clinical presentation, predisposing risk factors for neonatal sepsis, investigations, antibiotic details and medical management including respiratory support were recorded, and correlated with all events that may have occurred during the observation period after stopping antibiotics. Results: No adverse events were documented in the 24 hour period after antibiotics in all 95 neonates in this study and, therefore, there was no association with any potential predisposing risk factors. Conclusion: The need to observe neonates for a period prior to discharge after stopping antibiotics is not supported on clinical grounds and, as a result of this study, has been discontinued. Neonates can be discharged from hospital safely and immediately on stopping antibiotics, thus reducing hospital stay and an estimated cost saving of approximately €18,000 to the service provider per annum.peer-reviewe

Management of fever in children under 5 years of age within paediatric emergency department

Background Fever is one of the commonest reasons for presentation to healthcare services. It is often a cause of concern for parents and can be diagnostically challenging for healthcare professionals. Abiding by established guidelines on fever management is therefore of utmost importance to ensure proper use of antipyretics within the community. Objectives This audit aimed to assess the management of children aged up to 5 years presenting with fever to the Paediatric Emergency Department at Mater Dei Hospital, including assessing awareness of NICE guidelines on fever management amongst paediatric doctors and nurses. Method Data was collected from Paediatric Emergency Department Assessment Sheets for January 2017. An online questionnaire based on the NICE guidelines for fever management in children under 5 years was distributed to staff working within the Department of Child and Adolescent Health. Results 255 children aged up to 5 years presented with fever at the Paediatric Emergency Department in January 2017. 22.7% of children were aged between 24 months up to 3 years. The average time for first medical contact was 51 minutes. 23.9% presented with fever of 1 day duration. Respiratory rate was not recorded in 62% of cases whilst blood pressure was only recorded in 19.1% where applicable. Antipyretics were given in 73.8% of febrile cases despite distress only being documented in 6.8%. Conclusion More education is required on proper antipyretic use and pre-discharge advice. Routine measurement and documentation of all parameters should be encouraged both on initial assessment and pre-discharge.peer-reviewe

The relationship between infecting organisms and underlying structural anomalies in children with urinary tract infections

Introduction: Urinary tract infections (UTIs) are a common cause of morbidity in childhood with potential for renal scarring and reflux nephropathy which can lead to hypertension and end-stage renal failure. Aim: The aim of this study was to investigate the relationship between the infecting organism and any underlying anomalies of the urinary tract which may predispose to the development of infections and which may alter the management of children with UTIs. Methods and results: A total of 72 cases of UTI were recorded retrospectively (in- and outpatients), with ages ranging from 3 days to 48 months (mean 9.5, median 5 months). Fifty seven (79%) of patients had their first reported urinary tract infection under the age of 1 year.. Fifty eight (80.6%) were E. coli infections. These presented at an older age than non-E. coli infections. Investigations were abnormal in 31 (43%) cases. The mean age for first infection in patients with abnormal investigations was 7.7 months (median 2 months), younger than those with no renal tract abnormalities. Organisms other than E. coli were rarely found when no significant abnormalities were detected with investigation by US and MCUG and this was a statistically significant difference (p<0.001). Renal scarring was identified in 10 (13.9%) patients. Discussion: This study confirms that non-E. coli UTI is associated with underlying renal pathology and that early infections with any organism are more likely to be associated with underlying abnormalities. We also outline an algorithm based on the recent NICE 2007 guidelines which will be adopted by the Paediatric Department, Mater Dei Hospital for the investigation of UTI.peer-reviewe

Overview of the blood transfusion policy in preterms on the Neonatal Intensive Care Unit

Preterm infants on the Neonatal Intensive Care Unit receive a greater number of red cell transfusions than any other hospitalised group. Over the past twenty years research has focused on setting standards to determine when it is necessary to transfuse packed cells in this cohort, whilst exploring the use of red cell growth factors and other substrates judiciously in order to reduce and/or avoid red cell transfusions and limit donor exposure. One hundred and eighty-one blood transfusions were administered to 106 preterms less than 35 weeks gestation on the NICU during 2009 in Malta. The median (range) volume of blood used from each bag supplied by the Blood Transfusion Department was 25.8mls (10-50mls), the rest of which was discarded. Risk factors for transfusion included Extremely Low Birth Weight (less than 1kg) and a gestation of less than 30 weeks. The blood transfusion guidelines presently in use on the local NICU were reviewed and compared with more restrictive guidelines on other units and suggestions made to reduce transfusions in line with these guidelines. A reduction in transfusion aliquots provided for neonates to just 50mls from the customary 250mls in a dedicated single-donor programme will safeguard limited health resources and minimise donor exposure.peer-reviewe

Xanthogranulomatous pyelonephritis : the case of a perplexing kidney

A previously healthy 2-year-old girl presented with turbid urine and pallor. She was otherwise asymptomatic and afebrile. Clinical examination revealed a large ballotable right-sided abdominal mass. Ultrasound revealed an enlarged right kidney with loss of the normal renal architecture, which was replaced with multiple hypoechoic nodules. A number of renal calculi were observed with typical acoustic shadowing. A contrast-enhanced CT scan demonstrated the diagnostic ‘bear’s paw’ sign, multiple calculi, retroperitoneal lymphadenopathy and a small right psoas abscess. A dimercaptosuccinic acid (DMSA) scan demonstrated a non-functioning kidney.peer-reviewe

A local study of radiological findings in children diagnosed with urinary tract infection

BACKGROUND: Urinary tract infection (UTI) is a common cause of morbidity in children, requiring investigation to avoid complications. This study aims to: describe the microorganisms grown on culture according to age at presentation look for a relationship between abnormal US and/or MCUG results and age and clinical presentation explore whether an abnormal MCUG was associated with abnormal US explore whether a DMSA provides additional information to that already given by an US and/or MCUGMETHODOLOGY: All children less than 16 years of age hospitalised for upper or lower UTI, from the 1st January 2019 up till 31st December 2019 were included. Demographics, urine culture and imaging results were collected through the hospital clinical system.RESULTS: A total of 96 children were included. E. Coli was the commonest causative organism across all ages. 30 children underwent both US and MCUG, whilst 14 underwent US, MCUG and DMSA. Age and clinical presentation did not highlight a statistically significant relationship with abnormal findings on US and/or MCUG. 20% of children had a normal US but an abnormal MCUG. There were no abnormal DMSA results for children with normal combination US and MCUG.CONCLUSION: MCUG is still warranted in cases of normal baseline US, irrespective of age and gender. Given that our study was not powered enough, it was not possible to ascertain whether DMSA adds additional information in children with a normal US and MCUG. Further studies with larger cohorts are warranted with the aim of reducing DMSA referrals, radiation, and costs.peer-reviewe

Ulcerated lesions as a risk factor for Henoch-Schonlein purpura nephritis

Objective: To determine the correlation between the severity of Henoch-Schonlein purpura skin manifestations and development of nephritis and to characterise the disease within the Maltese paediatric population. -- Design: A retrospective analysis of the 96 cases diagnosed with Henoch-Schonlein purpura at Mater Dei Hospital between January 2008 and January 2016. Clinical notes were reviewed and anonymised data regarding the presentation, progression and follow-up of these cases was entered into a database. -- Results: 96 cases met the inclusion criteria with a male to female ratio of 1.35:1 and with a mean age at presentation of 6.4 years (interquartile range 3.5 years). 99% had the typical rash at presentation with 75% having other associated clinical findings. Renal involvement was found in 36.5%: isolated proteinuria in 19.8%, isolated haematuria in 13.5%, haematuria, proteinuria and hypertension in 3.1% and nephrotic range proteinuria in 2% of cases. A severe rash at presentation was shown to be a prognostic indicator for renal involvement. -- Conclusion: Henoch-Schonlein purpura in the Maltese paediatric population is similar in incidence to that quoted in the literature. The majority of cases are uncomplicated and the outcome is frequently favourable. The presence of a severe rash at presentation significantly increases the risk of renal involvement and long term complications.peer-reviewe

Early postnatal genetic diagnosis for Joubert Syndrome Type 14

BACKGROUND: Joubert syndrome is an autosomal recessive condition characterised by a midbrain-hindbrain malformation giving rise to the characteristic “molar tooth sign” on MRI. Common signs and symptoms include neonatal breathing dysregulation, hypotonia and developmental delay. A spectrum of conditions referred to as Joubert syndrome and Related Disorders (JSRD) have also been described, referring to the presence of multiorgan involvement in addition to the neurological signs and symptoms characterising “pure Joubert”.CASE PRESENTATION: We report a case of JS type 14 (homozygous pathogenic variant in TMEM237) in a male neonate referred antenatally with a brain malformation and bilateral cystic kidneys on ultrasound and MRI, raising the suspicion of a ciliopathy. Clinical features at birth included hypotonia, hydrocephalus, ocular colobomas and breathing difficulties which required mechanical ventilation.CONCLUSION: Antenatal suspicion followed by confirmation of JS in the immediate postnatal period, has enabled early timely intervention by a multidisciplinary team, thus optimising parental counselling, developmental outcomes, and monitoring for potential future complications.peer-reviewe

Nephrocalcinosis in a 13-year-old girl with type 1 diabetes mellitus complicated by Mauriac syndrome

Mauriac syndrome is a rare complication of poorly-controlled type 1 diabetes mellitus (T1DM) characterised by hepatomegaly, short stature, and pubertal delay. We report the case of a 13-year-old girl with T1DM who presented to our hospital with hyperglycaemia and hepatomegaly, and was also found to have bilateral medullary nephrocalcinosis. It is hypothesised that chronic hyperglycaemia resulting from long-term insulin under-dosage led to chronic acidosis which caused hypercalciuria and consequently, nephrocalcinosis. Poor glycaemic control, Mauriac syndrome and nephrocalcinosis, may be associated with socio-economic difficulties. Both medical optimisation and psychosocial support should be provided to reach optimal glycaemic targets, reverse the features of Mauriac syndrome, and prevent worsening of nephrocalcinosis that could potentially lead to chronic renal impairment.peer-reviewe