Permanent URI for this collection
Browse
Recent Submissions
Publication P15 Scabies in pediatric population in Nepal: a study from tertiary-care referral children’s hospital(Oxford University Press, 2025-12-19) Poudel, Pratima; Parajuli, Niraj; Pandey, Pritam; Acharya, Bimal; Sharma, Puja; Shrestha, Amrita; Pandey, Pritam; Medical and DentalIntroduction Scabies is one of the neglected diseases which has global impact and long-term health sequelae. Despite it being a common dermatological problem in Nepal, there has been a lack of research conducted determine the prevalence of this disease. As a result, we remain unaware of the true impact it has on the population. The aim of the study is to estimate the prevalence of scabies in pediatric population which is currently lacking. The comlications are an important aspect of the disease which largely goes unreported so the issue has not have a needed focus from the government. Materials and methods This retrospective study was conducted at Kanti Children’s Hospital, the only pediatric referral government run hospital of Nepal. All patients who visited department of Dermatology from November 2019 to June 2021 and diagnosed with scabies clinically were selected as cases. Patients were further categorized according to sex, age group, season of scabies acquisition, and presence of complications. Results Our study revealed the prevalence of scabies to be at 5.29% according to the hospital data. The most common age group for scabies was in age group 1–3 years (30.4%) followed by the age group 1month-1 year (24.6%). More than half of patients (n = 178, 57.6%) got infected during the winter season followed by spring season (n = 77, 24.9%). Secondary infections were present in 28 (9.1%) patients. Secondary eczematization was present in 23 patients (7.4%). Scabetic nodules were present in 40 patients (12.9%).Publication A Child With Thickened Skin: When the Skin Biopsy Tells a Different Story(Wiley, 2025-11-20) Govardhan, Chaitra; Prithvi, Ashwini; Walker, Louise; Talakola, Prameela; Udensi, Louisa; Walker, Louise; Talakola, Prameela; Udensi, Louisa; Medical and DentalNo abstract availablePublication Risk-stratification-based telecare of infantile hemangioma during COVID-19 pandemic(Pediatric Dermatology Association, 2020) Rabani, Ahmed; Subhani, Fazal-E-Rabi; Danish, Fazal-I-Akbar; Danish, Fazal-I-Akbar; Medical and DentalThe authors review the literature on infantile hemangioma and suggest in the current moment of COVID-19 pandemic a judicious use of telematic medicine that ensures in cases less at risk of complications an early specialist intervention and a timely start of therapy. In case of hemangiomas at risk of complications persists the need for personal assistance.Publication Seizures: Management in children(MA Healthcare, 2020-03-02) Luff, Ed; Luff, Ed; Medical and DentalEpilepsy is a common neurological condition and one that school nurses are likely to deal with due to its prevalence in the population. It is therefore important to know how to manage a child having a seizure in the community. Having an appreciation of the long-term management with antiepileptic drugs to try to prevent and control seizures is useful. Children suffering from epilepsy may experience a seizure at any time, often without warning, so an understanding of the correct first aid measures and the use of emergency medication is key. It is also important to know when further medical help is required as well as when it may not be. This article will give a brief overview of the management of seizures in children, covering both prevention and initial management in the community setting.Publication Acute encephalopathy related to cardio-facio-cutaneous syndrome with KRAS mutation(Wiley, 2020-12-30) Monaghan, Marie; Hannon, Faith; Likeman, Marcus; Stoodley, Neil; Donaldson, Alan; Olliffe, David; Carter, Michael; Mallick, Andrew; Donaldson, Alan; Olliffe, David; Medical and DentalBackground: Cardio-facio-cutaneous syndrome (CFC) is a rare genetic condition affecting up to 300 people globally. The cardinal features of CFC are congenital cardiac anomalies, distinctive facial appearances and skin changes, however, it is also associated with feeding problems, short stature, macrocephaly, hypotonia, speech delay, learning disability and seizures. CFC has phenotypic overlap with Noonan and Costello Syndrome and these are all considered RASopathies. CFC is associated with variants in genes involved in the RAS/MAPK intracellular signalling pathway and is most frequently caused by mutations in BRAF (approximately 75%), MAP2K1 and MAP2K2 (approximately 25%) and infrequently KRAS (approximately 2%). Case report: We describe a 7 year old girl with CFC caused by a pathogenic KRAS variant. She has distinctive facial features, pulmonary stenosis and atrial septal defect, multiple pigmented naevi and lymphoedema. She also has Dandy-Walker Syndrome with hydrocephalus, poor feeding and faltering growth requiring gastrostomy feeding. She has right eye amblyopia and right optic nerve atrophy. She is able to speak and walk independently but required additional educational support. She presented with fever without a prodrome and a prolonged focal seizure affecting the left side of her body. She had a marked left-sided hemiplegia. Magnetic resonance imaging showed extensive right hemispheric high T2 intensity, swelling of the grey matter structures, and initial restricted diffusion. Infective, auto-immune, and metabolic causes were excluded. Acute encephalopathy with brain oedema has been previously reported in cases of CFC associated with BRAF mutations, but this is the first report in a patient with CFC due to a KRAS mutation. It is postulated that aberrant ERK signalling may lead to excessive central nervous system cytokine production leading to neurotoxicity and encephalopathy. KRAS is upstream from BRAF and this case suggests that aberrant signalling upstream from BRAF can also lead to acute encephalopathy.Publication Consensus exercise identifying priorities for research in the field of general surgery of childhood in the UK(Oxford University Press, 2021-02-28) Hall, Nigel; Rees, Clare; Rhodes, Hannah; Williams, Alun; Vipond, Mark; Evans, David; Wood, Richard; Sutcliffe, Jonathan; Vipond, Mark; Medical and DentalBackground: The evidence base underlying clinical practice in children's general surgery is poor and high-quality collaborative clinical research is required to address current treatment uncertainties. The aim of this study was, through a consensus process, to identify research priorities for clinical research in this field amongst surgeons who treat children. Methods: Questions were invited in a scoping survey amongst general surgeons and specialist paediatric surgeons. These were refined by the study team and subsequently prioritized in a two-stage modified Delphi process. Results: In the scoping survey, a total of 226 questions covering a broad scope of children's elective and emergency general surgery were submitted by 76 different clinicians. These were refined to 71 research questions for prioritization. A total of 168 clinicians took part in stage one of the prioritization process, and 157 in stage two. A 'top 10' list of priority research questions was generated for both elective and emergency general surgery of childhood. These cover a range of conditions and concepts, including inguinal hernia, undescended testis, appendicitis, abdominal trauma and enhanced recovery pathways. Conclusion: Through consensus amongst surgeons who treat children, 10 priority research questions for each of the elective and emergency fields have been identified. These should provide a basis for the development of high-quality multicentre research projects to address these questions, and ultimately improve outcomes for children requiring surgical care.Publication Vitamin C Deficiency Presenting as an Acute Limp in Childhood(Springer, 2020-07-14) Kyprios, Andrew; Kyprios, Andrew; Medical and DentalA previously well three-year-old boy attended with right knee pain and an antalgic gait. There was no history of fever and bloods including inflammatory markers were normal. A diagnosis of transient synovitis (irritable hip) was made and managed conservatively. One month later, he represented with increasing pain, now localising to his left hip, waking him at night with difficulty weight-bearing. No effusion was seen on ultrasound and bloods remained normal. He was discharged home but came back a week later with worsening polyarticular pain, a new maculopapular rash, significant bruising and new dental decay. A clinical diagnosis of vitamin C deficiency was made secondary to dietary insufficiency, and this was confirmed on blood testing. Within six weeks, his symptoms had fully improved on oral ascorbic acid (vitamin C) and he was engaging in dietetic input and reward systems to maintain a more balanced diet.Publication G629(P) Pilot study of neurodevelopmental diagnostic clinic model in school-aged children, for whom there is diagnostic uncertainty regarding the presence of autistic spectrum disorder(BMJ Publishing Group, 2020-10-25) Austreng, Lucy; McAuley, Charlotte; Thurston, Victoria; de Gressi, Susanna; Austreng, Lucy; McAuley, Charlotte; Thurston, Victoria; de Gressi, Susanna; Medical and DentalBackground Locally, neurodevelopmental assessment of primary school aged children with social and communication difficulties involves collation of reports from the educational setting, then assessment by a paediatrician. Often, no formal diagnostic tool or face-to-face multi-professional assessment is carried out. For some children, this model of working can lead to a delay in conclusions due to complexities with that child. This pilot was a model for multi-professional working between a paediatrician and clinical psychologist using structured assessments for a group of children that presented diagnostic challenge. Aims To pilot a neurodevelopmental clinical model of joint working between a paediatrician and psychologist. To collect data regarding clinician and patient experience, time from initial referral to diagnosis and identify the advantages and pitfalls of this method of working. To provide information for the MDT reviewing the pathway for Autistic Spectrum Disorder (ASD) diagnosis. Method Cases were selected by the paediatrician. Data collected included demographics, presenting problems, time from referral to receiving a final diagnosis, diagnostic outcome and experience feedback questionnaires. Diagnostic Interview for Social and Communication Disorders (DISCO) and Autism Diagnostic Observation Schedule (ADOS) assessments were carried out. Results Thirteen children were enrolled in the pilot from 2017 to 2019 with a median age of nine years, four months. Eleven participants were male. Five of the children were given a final diagnosis of ASD. Other children received a diagnosis of Attention Deficit Hyperactivity Disorder (ADHD) or other cognitive or attachment difficulties. Six families completed feedback questionnaires. Most parents felt that the process helped their understanding of their child. Follow-up support was identified as an area for improvement. The clinicians found the process helpful, particularly with patients for whom there was disparity of opinion about that child. Conclusion This model demonstrated positive patient experience, increased diagnostic accuracy and clinicians reported improved job satisfaction. It is likely to shorten initial referral to diagnosis time and offer a model of working that is closer to NICE guidance. Cost implications would need to be considered. Should this model of working be commissioned, areas for improvement would include follow up after diagnosis, and the robustness of the administration around the clinic.Publication Learning Curve Analyses for Achieving Satisfactory Procedural Completion Rates in Paediatric Oesophagogastroduodenoscopy(Wiley, 2020-03-01) Barraclough, Harriet; Siau, Keith; Ward, Stephen; Dunckley, Paul; Hawkes, Neil; Thomson, Mike; Narula, Priya; Dunckley, Paul; Medical and DentalBackground: The learning curve in paediatric oesophagogastroduodenoscopy (OGD) is unknown. Using ≥95% D2 (second part of the duodenum) intubation rates as a marker of technical competency, we conducted learning curve analyses to identify when trainees achieve competency in paediatric OGD. Factors associated with competency were also evaluated. Methods: This nationwide study analysed data from paediatric OGD procedures prospectively entered into the UK endoscopy training e-portfolio between 2014 and 2018. Moving average and learning curve cumulative summation analyses were performed to determine procedural numbers required to achieve ≥95% D2 intubation rates. Factors associated with D2 intubation were assessed using a multivariable binary logistic regression approach. Results: A total of 8929 procedures performed by 61 trainees were identified. These 61 trainees had recorded a mean of 124.6 procedures (range 22–571, interquartile range 165). By moving average analysis, 95% D2 intubation was achieved after 79 procedures. By learning curve cumulative summation analysis, 81.6% of trainees were competent after 100 procedures. Multivariable factors associated with unassisted procedural completion included: lifetime procedure count (P < 0.001), higher trainee seniority (P < 0.001), patient age (P = 0.002), outpatient status (P < 0.001), and attendance at a national Basic Skills OGD course (P = 0.011). Conclusions: This study demonstrates that, on average, 79 procedures in paediatric OGD are required to attain the competency outcome of ≥95% D2 intubation rates. By 100 procedures, 81.6% of our sample had achieved ≥95% D2 intubation. The minimum procedural count of 100 set by the UK and international training programmes can be used alongside existing objective assessment measures to safeguard competency within a training cohort.Publication Randomized clinical trial of DTaP5-HB-IPV-Hib vaccine administered concomitantly with meningococcal serogroup C conjugate vaccines during the primary infant series(Elsevier, 2020-07-17) Oliver, Jennifer; Sadorge, Christine; Boisnard, Florence; Snape, Matthew; Tomlinson, Richard; Mann, Rebecca; Rudd, Peter; Bhakthavalsala, Shyam; Faust, Saul; Heath, Paul; Hughes, Stephen; Borrow, Ray; Thomas, Stephane; Finn, Adam; Bhakthavalsala, Shyam; Medical and DentalBackground: Concomitant administration of vaccines simplifies delivery. DTaP5-HB-IPV-Hib is a fully liquid, combination vaccine against 6 diseases. This study evaluated the compatibility of DTaP5-HB-IPV-Hib with 2 different meningococcus group C conjugate (MCC) vaccines in infants. Methods: In a phase 3, open-label study, 284 healthy infants from 11 UK centres received DTaP5-HB-IPV-Hib at age 2, 3, and 4 months; 13-valent pneumococcal conjugate vaccine (PCV13) at 2 and 4 months; a Haemophilus influenzae type b (Hib)-MCC vaccine and a measles/mumps/rubella vaccine at 12 months. Participants were randomised 1:1 to receive either an MCC-detoxified tetanus toxin vaccine (MCC-TT; n = 141) or an MCC-Corynebacterium diphtheriae CRM197 protein vaccine (MCC-CRM; n = 143) at 3 and 4 months. The primary outcome was seroprotection rate (SPR) to MCC (percent with rabbit complement serum bactericidal antibody titer ≥8). Results: Per protocol analysis, MCC SPRs were 100 and 96.4 one month after the first dose, 100 and 99.1 after the second dose, and 100 and 97.3 after the third (booster) dose of MCC in the MCC-TT and MCC-CRM groups, respectively. One month after all 3 doses of DTaP5-HB-IPV-Hib, immunoglobulin G anti-polyribosylribitol phosphate SPRs (% ≥0.15 µg/mL) were 97.8 in the MCC-TT group and 100 in the MCC-CRM group; anti-hepatitis B antigen SPRs (% ≥10 mIU/mL) were 96.8 and 96.3 in the MCC-TT and MCC-CRM groups, respectively. All participants were seroprotected against diphtheria and tetanus (≥0.01 IU/mL) and poliovirus types 1, 2, and 3 (≥8 dilution), and seroresponse rates to all pertussis antigens were ≥90.4%. Two vaccine-related serious adverse events (transient severe abdominal pain and crying) occurred concomitantly in 1 participant in the MCC-CRM group. Adverse event rates were similar to other studies of DTaP5-HB-IPV-Hib, with pyrexia ≥38 °C in 10.9% of participants following any dose. Conclusions: DTaP5-HB-IPV-Hib can be effectively used in a 2-, 3-, and 4-month infant priming schedule when given with 2 doses of MCC.Publication cGAS-mediated induction of type I interferon due to inborn errors of histone pre-mRNA processing(Nature Research, 2020-11-23) Uggenti, Carolina; Leppelly, Alice; Depp, Marine; Badrock, Andrew; Rodero, Mathieu; El-Daher, Marie-Therese; Rice, Gillian; Dhir, Somdutta; Wheeler, Ann; Dhir, Ashish; Albawardi, Waad; Fremond, Marie-Louise; Seabra, Luis; Doig, Jennifer; Blair, Natalie; Martin-Niclos, Maria; Mina, Erica; Rubio-Roldan, Alejandro; Garcia-Perez, Jose; Sproul, Duncan; Rehwinkel, Jan; Hertzog, Jonny; Boland-Auge, Anne; Olaso, Robert; Deleuze, Jean-Francois; Baruteau, Julien; Brochard, Karine; Buckley, Jonathan; Cavallera, Vanessa; Cereda, Cristina; De Waele, Liesbeth; Dobbie, Angus; Doummar, Diane; Elmslie, Frances; Koch-Hogrebe, Margarete; Kumar, Ram; Lamb, Kate; Lamb, Kate; Medical and DentalInappropriate stimulation or defective negative regulation of the type I interferon response can lead to autoinflammation. In genetically uncharacterized cases of the type I interferonopathy Aicardi–Goutières syndrome, we identified biallelic mutations in LSM11 and RNU7-1, which encode components of the replication-dependent histone pre-mRNA–processing complex. Mutations were associated with the misprocessing of canonical histone transcripts and a disturbance of linker histone stoichiometry. Additionally, we observed an altered distribution of nuclear cyclic guanosine monophosphate–adenosine monophosphate synthase (cGAS) and enhanced interferon signaling mediated by the cGAS–stimulator of interferon genes (STING) pathway in patient-derived fibroblasts. Finally, we established that chromatin without linker histone stimulates cyclic guanosine monophosphate–adenosine monophosphate (cGAMP) production in vitro more efficiently. We conclude that nuclear histones, as key constituents of chromatin, are essential in suppressing the immunogenicity of self-DNA.Publication 1176 Outpatient clinics – what do families value?(BMJ Publishing Group, 2022-11-04) Austreng, Lucy; Soans, Edward; Allen, Joanna; Kyprios, Andrew; Brooks-Moizer, Alexander; Lamb, Kate; Austreng, Lucy; Soans, Edward; Allen, Joanna; Kyprios, Andrew; Brooks-Moizer, Alexander; Lamb, Kate; Medical and DentalNo abstract availablePublication 1089 Unscheduled care presentations for children in Gloucestershire during the SARS-CoV-2 pandemic(BMJ Publishing Group, 2021-11-24) Jackson, Louisa; Bennett, Imelda; Freebrey, Clare; Teare, Becky; Jackson, Louisa; Bennett, Imelda; Freebrey, Clare; Teare, Becky; Medical and Dental; Nursing and Midwifery Registered; Admin and ClericalNo abstract availablePublication IMMU-02. CH14.18 in the treatment of high-risk neuroblastoma: A meta-analysis(Oxford University Press, 2021-06-01) Abdel-Khaleq, Sameerah; Alim, Lina; Johnston, Atholl; Adam, Khloud; Alim, Lina; Adam, Khloud; Medical and DentalNo abstract availablePublication 1402 Dedicated administrative support improves children and young people’s advance care planning documentation(BMJ Publishing Group, 2022-11-24) Homfray, Gareth; Sellers, Isabel; McAuley, Charlotte; Homfray, Gareth; Sellers, Isabel; McAuley, Charlotte; Medical and DentalNo abstract availablePublication 801 Evaluating the use of chest radiographs in a paediatric emergency department(BMJ Publishing Group, 2021-09-30) Collicott, Nathan; Perry, Sophie; Begley, Roisin; Collicott, Nathan; Medical and DentalAbstract Background Requests for chest radiographs in children attending the Bristol Royal Hospital for Children (BRHC) ED were noted to be increasing 2017–2019, with predictable winter peaks, particularly in November. Previous work questions the utility of chest radiographs and their impact on management of common paediatric presentations to ED, including LRTI, bronchiolitis and wheeze. Objectives To describe the patient cohort undergoing chest radiographs in a paediatric ED within a tertiary children’s hospital in terms of age, indication and final ED diagnosis. To quantify the proportion of abnormal (‘positive’) and normal (‘negative’) chest radiographs, as reported by radiologists, with respect to indication and age. To quantify the proportion of patients undergoing a chest radiograph receiving antibiotics and whether these were prescribed before or after imaging. Methods Retrospective review of patients who attended the BRHC Paediatric ED undergoing chest radiograph in the first two weeks of November 2017–2019. Data for age (split into 0–11 months, 1–4y, 5–10y and 11+ groups), indication for radiograph, the final coded ED diagnosis, radiograph findings and antibiotic treatment were collected. Results The 1–4y age group represented the largest proportion of radiographs, accounting for 53% of all requests. Table 1 shows the five most common indications are shown below. CAP/LRTI was the most common indication in all age groups, accounting for 55% of total requests. View inline View popup Abstract 801 Table 1 Stated indications The five most common final coded diagnoses are shown below. URTI and bronchiolitis are commonly coded discharge diagnoses in this cohort. View inline View popup Abstract 801 Table 2 Discharge diagnoses Over three years, 302 chest radiograph requests were made. Across all groups, 67% were reported as normal. In the 0–1y age group, 83% of radiographs were normal. 135 patients received antibiotics; 62% were prescribed after chest radiograph. Conclusions Our data shows that use of chest x-rays has steadily increased over the studied period. Although BTS guidelines suggest relatively limited indications for chest x-rays in the setting of CAP in children, this remains the most common reason given for our patients to undergo a chest radiograph. Importantly, a significant majority of x-rays were reported as normal, especially among infants. Targeting chest radiographs more effectively may be helpful for several reasons. Firstly, there is limited evidence that they are helpful in distinguishing bacterial pneumonia from other aetiologies. Although the retrospective nature of our data poses challenges, it suggests that clinicians tend to make antibiotic treatment decisions at least partly based on x-ray findings. Secondly, all other things being equal, a patient undergoing x-ray will spend longer in ED than one who does not. This potentially has an impact on patient flow, even more important with the current need for social distancing. We plan to use this data as a baseline for future quality improvement work with the aim of reducing chest X-ray use in patients unlikely to benefit.Publication OC43 Follow-up practices for paediatric patients with intestinal failure graduating off home parenteral nutrition(BMJ Publishing Group, 2025-08-20) Eg, Zhong Ning; Munden, Oriana; Talbot, Jonathan; Wiskin, Anthony; Eg, Zhong Ning; Medical and DentalNo abstract availablePublication Quality of Life Impact of Varicella on Children and Their Families in the UK: The QoLPoX Study(Lippincott, Williams & Wilkins, 2025-09-12) Marlow, Robin; Roderick, Marion; Oliver, Jenny; Jordan, Zoe; Rowbotham, Isabel; Jones, Steve; Wilkins, Suzanne; Thompson, Clara; Whittaker, Liz; Bethell, Delia; Shackley, Fiona; Cathie, Katrina; Heath, Paul; Langlands, Jenny; Lopez-Bernal, Jamie; Amirthalingham, Gayatri; Rodrigues, Fernanda; Finn, Adam; Thompson, Clara; Medical and DentalBackground: Varicella (chickenpox) is a ubiquitous disease of childhood in the UK, yet highly effective and safe vaccines have been routinely used in other countries for years. Previous UK assessments of varicella vaccines did not meet the National Institute of Health and Care Excellence £20-30k/quality-adjusted life year (QALY) cost utility threshold, in part due to limited data quantifying the quality of life (QoL) lost. Methods: We carried out a prospective multicenter observational study recruiting families while their child had acute varicella or secondary complications. We recruited severe cases admitted to hospital and mild community cases from childcare. QoL was assessed using standard tools: EQ5D-5L + CHU9 for children and EQ5D-5L for both carers. Assessments were carried out daily in the hospitalized arm until discharge, weekly for 1 month and then at 6 months. In the community arm, assessments were daily until back to normal health. Results: Between 2018 and 2022, we obtained data on 55 community and 140 hospitalized cases. Mean QALY loss in children was 6.7 (6.1-7.0)/1000 community cases and 46.7 [95% confidence interval (CI): 44.5-51.6]/1000 hospitalized. Primary carers lost 2.4 (95% CI: 2.2-2.8)/1000 community cases or 20.2 (95% CI: 19.4-22.3)/1000 hospitalized. Secondary carers lost 0.7 (0.3-0.7)/1000 community cases or 15.8 (95% CI: 15.5-18.1)/1000 hospitalized. Conclusions: This detailed prospective assessment of QoL loss from varicella found it to be 1.5-10× larger in children than used in previous economic assessments and quantified the QALY loss for both primary and secondary carers. These data have been used to parameterize new assessments of UK varicella vaccine cost utility.Publication Fifteen-minute consultation: Cardiac murmurs in the Newborn Infant Physical Examination (NIPE)(BMJ Publishing Group, 2021-06-29) Oswal, Abhishek; Holman, Jennifer; Oswal, Abhishek; Holman, Jennifer; Medical and DentalThe finding of a cardiac murmur on the initial newborn examination is common but may be a source of anxiety for practitioners due to worries about missing critical congenital heart defects (CHDs). This article aims to provide an approach to this common finding, in particular, reviewing the evidence base behind features of the history, examination and subsequent non-specialist investigations which may increase the likelihood of CHDs. The aim of this structured approach is to give clinicians confidence in dealing with this common clinical finding, to be able to pick out those infants most at risk of having critical CHDs.Publication 7711 Risks of surgery for temporal lobe epilepsy to the episodic memory of children: a systematic review and meta-analysis(BMJ Publishing Group, 2025-05-30) Haque, Nya; Lloyd, Megan; Reddy, Shreeja; Leandro, Lorna; Stuart, Beth; Huntley, Alyson; Atan, Denize; Leandro, Lorna; Medical and DentalNo abstract available