Hydrocephalus Secondary to Intraventricular Myxopapillary Ependymoma: Case Report

Ependymomas are a somewhat diverse category of glial tumors that often develop from the lining of the brain’s ventricles, or the spinal cord’s central canal. They make up 5% of all neuroepithelial tumors, 10% of paediatric brain tumors, and up to 33% of brain tumors in children under the age of three. Hydrocephalus is one of the complications, and it can be identified as progressive macrocephaly or increasing head circumference crossing percentiles, nausea, vomiting, poor appetite, irritability, and regression of developmental milestones.

Hepatolithiasis:Epidemiology,presentation,classification and management of a complex disease

The term hepatolithiasis describes the presence of biliary stones within the intrahepatic bile ducts,above the hilar confluence of the hepatic ducts.The disease is more prevalent in Asia,mainly owing to socioeconomic and dietary factors,as well as the prevalence of biliary parasites.In the last century,owing to migration,its global incidence has increased.The main pathophysiological mechanisms involve cholangitis,bile infection and biliary strictures,creating a self-sustaining cycle that perpetuates the disease,frequently characterised by recurrent episodes of bacterial infection referred to as syndrome of“recurrent pyogenic cholangitis”.Furthermore,long-standing hepatolithiasis is a known risk factor for development of intrahepatic cholangiocarcinoma.Various classifications have aimed at providing useful insight of clinically relevant aspects and guidance for treatment.The management of symptomatic patients and those with complications can be complex,and relies upon a multidisciplinary team of hepatologists,endoscopists,interventional radiologists and hepatobiliary surgeons,with the main goal being to offer relief from the clinical presentations and prevent the development of more serious complications.This comprehensive review provides insight on various aspects of hepatolithiasis,with a focus on epidemiology,new evidence on pathophysiology,most important clinical aspects,different classification systems and contemporary management.

Comparative study on WHO Western Pacific Region and World Federation of Chinese Medicine Societies international standard terminologies on traditional medicine: Diseases of Paediatrics(Part 2)

The following is a continued analysis about some terms related to paediatrics in traditional Chinese medicine （TCM） included in the WHO International Standard Termi- nologies on Traditional Medicine in the Western Pacific Region （abbreviated as the WPRO Standard）[1] and the International Standard Chinese-English Basic Nomenclature of Chinese Medicine compiled by World Federation of Chinese Medicine Societies （abbreviated as the WFCMS Standard）[2], according to explanations made in the book entitled A Great Dictionary of Traditional Chinese Medicine[3], Chinese-English Dictionary of Traditional Chinese Medicine[4] and the studies made in the book entitled International Standardization of English Translation of Traditional Chinese Medicine： Study of Theory, Summarization of Practice and Exploration of Methods[5].

Flexible bronchoscopy for foreign body aspiration in children:A single-centre experience

BACKGROUND The technological evolution of bronchoscopy has led to the widespread adoption of flexible techniques and their use for both diagnostic and therapeutic purposes.Currently,there is an active debate regarding the comparative efficacy and safety of rigid vs flexible bronchoscopy in the treatment of foreign body aspiration.AIM To evaluate our experience with tracheobronchial foreign body extraction using flexible bronchoscopy and provide a literature overview.METHODS This was a single-centre retrospective study.Twenty-four patients were enrolled between January 2017 and January 2023.Medical records of patients aged below 18 years who were admitted to authors’affiliated institution with a suspected diagnosis of foreign body aspiration were collected from hospital’s database to Microsoft Excel 2019.Data were analysed using MedCalc Statistical Software.RESULTS Patient ages varied from 9 months to 11 years.The median age was 23.5 months,95%confidence interval(CI)19.49-44.77.We observed age clustering in children with foreign body aspiration at our institution with three age subgroups:(1)0-25 months;(2)40-60 months;and(3)120-140 months.We expectancy of an organic tracheobronchial foreign body was significantly higher in 0-25 months subgroup than that in older ones when subgroups 40-60 and 120-140 months were combined together(odds ratio=10.0,95%CI:1.44-29.26,P=0.0197).Successful foreign body extraction was performed in all cases.Conversion to a rigid bronchoscope was not required in any of the cases.No major complications(massive bleeding,tracheobronchial tree perforation,or asphyxia)were observed.CONCLUSION Flexible bronchoscopy is an effective and safe method for tracheobronchial foreign body extraction in children.

Comparative study on WHO Western Pacific Region and World Federation of Chinese Medicine Societies international standard terminologies on traditional medicine: Diseases of Paediatrics(Part 1)

The following is a brief analysis about some terms related to paediatrics in traditional Chinese medicine （TCM） included in the WHO International Standard Terminologies on Traditional Medicine in the Western Pacific Region （abbreviated as the WPRO Standard）EI~ and the Interna- tional Standard Chinese-English Basic Nomenclature of Chinese Medicine compiled by the World Federation of Chinese Medicine Societies （abbreviated as the WFCMS Standard）, according to explanations made in the book entitled A Concise Dictionary of Traditional Chinese Medicinet,

Comparative study on WHO Western Pacific Region and World Federation of Chinese Medicine Societies international standard terminologies on traditional medicine: Diseases of Paediatrics(Part 3)

The following is a continued analysis about the third group of terms related to paediatrics in TCM included in the WHO International Standard Terminologies on Traditional Medicine in the Western Pacific Region （abbreviated as the WPRO Standard） and the International Standard Chinese- English Basic Nomenclature of Chinese Medicine compiled by World Federation of Chinese Medicine Societies （abbreviated as the WFCMS Standard）,

Cardioembolic Stroke as a Sequela of General Ventricular Hypokinesia Secondary to Acute Myocarditis in a 16-Year-Old Male

Paediatric cardiac disease is an established cause of ischemic stroke in the neonatal and infantile groups. These diseases may be congenital or acquired. However, clinical myocarditis is in itself, relatively uncommon in older children. The most common pathogen is Coxsackie virus B. The offending agent instigates an immune response, which causes myocardial oedema with eventual systolic and diastolic dysfunction. Cardioembolic stroke can occur secondary to an intra-mural thrombus in a dysfunctional atrium or ventricle. We describe the case of an adolescent male with acute myocarditis complicated by a thromboembolic stroke. After initial management of acute pulmonary oedema and heart failure with restricted ejection fraction (HFrEF), the child developed seizure-like symptoms on the 10th day of hospitalization, prompting urgent neuro-radio diagnosis, which revealed acute infarcts in the cerebellum and frontal lobe of the cerebrum. We believe this case to be of clinical relevance because;1) The diagnosis of an acute stroke in children is often delayed due to the atypical clinical presentation and often the absence of traditional stroke-like symptoms, and 2) There is a lack of sufficient high-quality evidence regarding the predictors and the immediate management of stroke in paediatric heart disease, as well as inadequate data on prevalence and incidence in paediatric cardioembolic strokes.

Evolution of Developmental Behaviour Paediatrics(DBP)In the Chinese Speaking Regions in Asia

1 DBP as a Subspecialty under Paediatrics and Child Health Developmental Paediatrics was established first in the United Kingdomin 1950’s at the Newcomen Clinic at

Evaluation of Entrance Skin Dose from Paediatric Diagnostic X-Ray Examination

As children are prone to be more radiosensitive than adults, it is imperative to assess the Entrance Skin Doses (ESDs) for patients being examined by X-rays, in order to ensure the optimization of dose while considering a number of other fickles. The ESD received by 50 paediatrics (aged 1-13 years) undergoing 8 types of X-ray examinations were measured at Federal Teaching Hospital, Ido-Ekiti, Ekiti, Nigeria, within a period of February 2019 to March 2020 using thermoluminescent dosimeters. The mean ± SD of ESDs were 0.85 ± 0.32, 2.04 ± 0.75, 0.60 ± 0.07, 0.62 ± 0.22, 0.57 ± 0.24, 1.75 ± 0.76, 0.93 ± 0.31 and 0.63 ± 0.06 mGy for Chest, Skull, Hand, Forearm, Knee, Abdomen, Leg and Feet, respectively. The mean ESDs were found to be within the recommended reference dose in all examinations, except for the Chest examination which was higher. The data obtained in this study will serve as existing data in Nigeria for future research works, as it would assist in optimizing dose to patients, especially the paediatrics.

Osteogenesis Imperfecta: One Disease, Two or More Faces: A Case Report

Being such a rare condition in paediatrics, osteogenesis imperfecta (OI) is not a diagnosis which is made often. It is however, a diagnosis necessitating early diagnosis and timeous and effective management to improve morbidity and increase the quality of life for our patients. We report two cases of osteogenesis imperfecta in this case report to highlight the different phenotypic presentations. Both of these patients are unique in their presentations and each case highlights the importance of a high clinical index of suspicion by the practitioner in making the diagnosis of osteogenesis imperfecta. The first case is a patient who was diagnosed with osteogenesis imperfecta on day one of life. She had disproportionate short stature, blue sclera, a small chest and bowing of her lower limbs with swellings and tenderness over both of her femurs. A babygram radiograph revealed multiple fractures, with the presence of callus formation at some fracture sites suggesting intrauterine fractures. The second case is a patient who had normal anthropometry and was well at birth. She was subsequently diagnosed at two weeks of age when she presented to the Chris Hani Baragwanath Academic Hospital with an E. coli meningitis and she was suspected to have a right clavicular fracture and possibly rib fractures as she had pain on palpation over these areas. She was noted to have no blue sclera. Subsequent X-rays confirmed a right clavicular fracture as well as left and right rib fractures at different stages of healing. A lateral skull radiograph revealed Wormian bones. With no available genetic testing in South Africa, both diagnoses were made clinically. Both of our patients were started on zoledronic acid at three months of age and were followed up by the Metabolic Unit at the Chis Hani Baragwanath Academic Hospital. This case report of two patients highlights the characteristics important in diagnosing and treating this uncommon condition with varying phenotypical presentations, thus ensuring that the diagnosis is not missed or misdiagnosed: one disorder, two different faces.

Use of endolumenal functional lumen imaging probe in investigating paediatric gastrointestinal motility disorders

Investigating gastrointestinal(GI)motility disorders relies on diagnostic tools to assess muscular contractions,peristalsis propagation and the integrity and coordination of various sphincters.Manometries are the gold standard to study the GI motor function but it is increasingly acknowledged that manometries do not provide a complete picture in relation to sphincters competencies and muscle fibrosis.Endolumenal functional lumen imaging probe(EndoFLIP)an emerging technology,uses impedance planimetry to measure hollow organs cross sectional area,distensibility and compliance.It has been successfully used as a complementary tool in the assessment of the upper and lower oesophageal sphincters,oesophageal body,the pylorus and the anal canal.In this article,we aim to review the uses of EndoFLIP as a tool to investigate GI motility disorders with a special focus on paediatric practice.The majority of EndoFLIP studies were conducted in adult patients but the uptake of the technology in paediatrics is increasing.EndoFLIP can provide a useful complementary data to the existing GI motility investigation in both children and adults.

Paediatric gastrointestinal endoscopy in the Asian-Pacific region:Recent advances in diagnostic and therapeutic techniques

There has been a rapid expansion in the knowledge of paediatric gastroenterology over the recent decade,with a fast-growing repertoire of diagnostic techniques and management strategies for a wide spectrum of childhood gastrointestinal(GI)diseases.Paediatric GI endoscopy is a core competency every paediatric gastroenterologist should possess,and represents one of the most common procedures performed in children for both diagnostic and therapeutic purposes.Yet there remains a dearth of literature on the utility and outcomes of paediatric GI endoscopy in the Asia-Pacific region.Data on the diagnostic value of paediatric GI endoscopy would be an important aspect of discussion,with the emergence of inflammatory bowel disease(IBD)and eosinophilic GI disease as increasingly common endoscopic diagnoses.Time-based trends in paediatric GI endoscopy do point towards more IBD and gastroesophageal reflux disease-related complications being diagnosed,with a declining incidence of GI bleeding.However,the real-world diagnostic value of endoscopy in Asia must be contextualised to the region-specific prevalence of paediatric GI diseases.Helicobacter pylori infection,particularly that of multidrug-resistant strains,remains a highly prevalent problem in specific regions.Paediatric functional GI disorders still account for the majority of childhood GI complaints in most centres,hence the diagnostic yield of endoscopy should be critically evaluated in the absence of alarm symptoms.GI therapeutic endoscopy is also occasionally required for children with ingested foreign bodies,intestinal polyposis or oesophageal strictures requiring dilation.Endoscopic haemostasis is a potentially life-saving skill in cases of massive GI bleeding typically from varices or peptic ulcers.Advanced endoscopic techniques such as capsule endoscopy and balloon-assisted enteroscopy have found traction,particularly in East Asian centres,as invaluable diagnostic and therapeutic tools in the management of IBD,obscure GI bleeding and intestinal polyposis.State of the art endoscopic diagnostics and therapeutics,including the use of artificial intelligence-aided endoscopy algorithms,real-time confocal laser endomicroscopy and peroral endoscopic myotomy,are expected to gain more utility in paediatrics.As paediatric gastroenterology matures as a subspecialty in Asia,it is essential current paediatric endoscopists and future trainees adhere to minimum practice standards,and keep abreast of the evolving trends in the diagnostic and therapeutic value of endoscopy.This review discusses the available published literature on the utility of paediatric GI endoscopy in Asia Pacific,with the relevant clinical outcomes.

Ten-year review of trends in children with type 1 diabetes in England and Wales

This review describes the prevalence,incidence,and demographics of children and young people(CYP)with type 1 diabetes in England and Wales using data from the United Kingdom National Paediatric Diabetes Audit(NPDA)and has almost 100%submission from all paediatric diabetes centres annually.It is a powerful benchmarking tool and is an essential part of a long-term quality improvement programme for CYP with diabetes.Clinical characteristics of this population by age,insulin regimen,complication rates,health inequalities,access to diabetes technology,socioeconomic deprivation and glycaemic outcomes over the past decade is described in the review.The NPDA for England and Wales is commissioned by the United Kingdom Healthcare Quality Improvement Partnership as part of the National Clinical Audit for the United Kingdom National Service Framework for Diabetes.The rising incidence of Type 1 diabetes is evidenced in the past decade.Reduction in national median glycated hemoglobin for CYP with diabetes is observed over the last 10 years and the improvement sustained by various initiatives and quality improvement programmes implemented with universal health coverage.

Acute spinal subdural haematoma complicating a posterior spinal instrumented fusion for congenital scoliosis:A case report

BACKGROUND Acute spinal subdural haematoma(ASSH)is a rare and potentially devastating condition with a variable prognosis.Previously described subdural haematomas were thought to have occurred spontaneously or be related to major or minor iatrogenic or traumatic injuries caused by surgery,spinal puncture or epidural anaesthesia.Other contributing pathologies have been described,such as intradural tumours or spinal arteriovenous malformations.ASSH has also been associated with anticoagulation therapy,haemostatic abnormalities and risk factors such as pregnancy.To the best of our knowledge,this case study described the first reported occurrence of an ASSH during spinal surgery in a paediatric patient.The patient was not known to have any coagulopathies,and no obvious vascular lesions were documented.The surgical procedure did not directly involve the dura mater,and no evident intraoperative dural tears were found.CASE SUMMARY We reported and discussed a case of ASSH complicating a posterior spinal instrumented fusion during surgery for paediatric congenital scoliosis.This condition has not been previously described.We made recommendations for facing such an occurrence,explored its aetiology in the context of malformation and discussed the benefits of neuromonitoring during scoliosis correction and the management protocol.We conducted a PubMed literature review for cases of paediatric ASSH and other closely related disorders.We reviewed recommendations regarding neuromonitoring and treatment management in such cases.CONCLUSION ASSH is a rare complication of posterior spinal instrumented fusion.Published cases are more often associated with anticoagulation therapy or coagulopathy.Neuromonitoring is strongly recommended to detect and assess neurological status,thus enabling rapid diagnosis and treatment and facilitating early spinal decompression and a return to a normal neurological status.

Examining the life-cycle of the Coblator II device:Increases in paediatric post-tonsillectomy haemorrhage after six years of use

All paediatric tonsillectomies were examined from 2012–2019 at a single tertiaryreferral institution,and all were performed by one of two paediatric otolaryngologists.One exclusively used the diathermy,the other exclusively used the Coblator II.Two Coblator units were purchased simultaneously in 2012 and not replaced.There was no significant difference in number of tonsillectomies performed(1298 via diathermy,1221 via Coblator),nor in postoperative day of bleed,patient age,indication for procedure,and no patient had an underlying coagulopathy.The most common indication for tonsillectomy in both groups was sleep-disordered breathing.There was no significant difference in postoperative haemorrhage rates between groups for the first six years of the study(0%-1.4%/year).Years 7 and 8 saw the Coblator group haemorrhage rate significantly increase(0%-0.6%diathermy group vs 2%-3%Coblator group),though still fell within accepted rate of postoperative haemorrhage.The devices were then replaced,and the differences in haemorrhage disappeared.There appears to be a significant increase in paediatric post-tonsillectomy haemorrhage when using a Coblator device greater than six years old.This may suggest a useful lifespan for the Coblator II device.

Evidence of voriconazole pharmacokinetic variability in children and adolescents with haematological disease: proposal for therapeutic drug monitoring optimisation

Objective:Voriconazole(VCZ)is a triazole antifungal agent widely used in immunocompromised patients with suspected or proven invasive fungal infections.The achievement of therapeutic range(1-5 mg/L)is essential to maximize VCZ efficacy,as its pharmacokinetics is characterized by a wide inter-and intra-individual variability.This study aims to quantify the variability of VCZ trough concentrations in children and adolescents with haematological diseases and optimize therapeutic drug monitoring in clinical practice.Methods:We analysed the monitoring concentrations of all children(<18 years old)treated with VCZ in the Haematology Department of Robert DebréHospital between January 2014 and December 2016.Demographic,clinical data,and VCZ dosing and monitoring concentrations measured by high-performance liquid chromatography with ultraviolet detection(HPLC-UV)were analysed.Non-parametric tests were performed using SPSS IBM 24.0.Results:380 trough VCZ concentrations at steady-state(Ctrough,ss)were available in 79 children:45.6%had first Ctrough,ss in the therapeutic range at first monitoring,46.8%had Ctrough,ss below 1 mg/L and 7.6%had Ctrough,ss over 5 mg/L.Forty-one patients were treated with recommended doses but only 53%of them reached the therapeutic range.There was no impact of age,sex,biological parameters,or indication of VCZ on Ctrough,ss values.The number of Ctrough,ss in the therapeutic range increases with the number of monitoring per patient following dosage adaptations.Conclusion:The wide inter-and intra-individual variability of VCZ trough concentrations at recommended doses confirm the need to standardize VCZ monitoring and identify factors to be considered to prospectively adapt treatment for each patient.

Neuroprotective measures in children with traumatic brain injury

Traumatic brain injury(TBI) is a major cause of death and disability in children. Severe TBI is a leading cause of death and often leads to life changing disabilities in survivors. The modern management of severe TBI in children on intensivecare unit focuses on preventing secondary brain injury to improve outcome. Standard neuroprotective measures are based on management of intracranial pressure(ICP) and cerebral perfusion pressure(CPP) to optimize the cerebral blood flow and oxygenation, with the intention to avoid and minimise secondary brain injury. In this article, we review the current trends in management of severe TBI in children, detailing the general and specific measures followed to achieve the desired ICP and CPP goals. We discuss the often limited evidence for these therapeutic interventions in children, extrapolation of data from adults, and current recommendation from paediatric guidelines. We also review the recent advances in understanding the intracranial physiology and neuroprotective therapies, the current research focus on advanced and multi-modal neuromonitoring, and potential new therapeutic and prognostic targets.

Use of ketamine sedation for the management of displaced paediatric forearm fractures

AIM To determine if ketamine sedation is a safe and cost effective way of treating displaced paediatric radial and ulna fractures in the emergency department. METHODS Following an agreed interdepartmental protocol, fractures of the radius and ulna(moderately to severely displaced) in children between the age of 2 and 16 years old, presenting within a specified 4 mo period, were manipulated in our paediatric emergency department. Verbal and written consent was obtained prior to procedural sedation to ensure parents were informed and satisfied to have ketamine. A single attempt at manipulation was performed. Pre and postmanipulation radiographs were requested and assessed to ensure adequacy of reduction. Parental satisfaction surveys were collected after the procedure to assess the perceived quality of treatment. After closed reduction and cast immobilisation, patients were then followed-up in the paediatric outpatient fracture clinic and functional outcomes measured prospectively. A cost analysis compared to more formal manipulation under a general anaesthetic was also undertaken.RESULTS During the 4 mo period of study, 10 closed, moderate to severely displaced fractures were identified and treated in the paediatric emergency department using our ketamine sedation protocol. These included fractures of the growth plate(3), fractures of both radius and ulna(6) and a single isolated proximal radius fracture. The mean time from administration of ketamine until completion of the moulded plaster was 20 min. The mean time interval from sedation to full recovery was 74 min. We had no cases of unacceptable fracture reduction and no patients required any further manipulation, either in fracture clinic or under a more formal general anaesthetic. There were no serious adverse events in relation to the use of ketamine. Parents, patients and clinicians reported extremely favourable outcomes using this technique. Furthermore, compared to using a manipulation under general anaesthesia, each case performed under ketamine sedation was associated with a saving of ￡1470, the overall study saving being ￡14700. CONCLUSION Ketamine procedural sedation in the paediatric population is a safe and cost effective method for the treatment of displaced fractures of the radius and ulna, with high parent satisfaction rates.

Growth and bone health in paediatric patients with Crohn's disease receiving subcutaneous tumor necrosis factor antibody

AIM:To study whether adalimumab(ADA) was associated with improvement in growth,bone mineraldensity(BMD) and bone metabolism.METHODS:In children with Crohn's disease(CD) there is a high prevalence of growth failure and reduced BMD.Treatment with infliximab is associated with an improvement in growth.Anthropometry,paediatric CD activity index(PCDAI),bone markers and BMD was measured in 18 patients(72% females) one year before and after start of ADA with a median age of 14.4 years(range:5-19 years) at treatment start.Outcomes were indicators of growth with treatment as well as interval growth.RESULTS:Eleven(61%) children experienced catchup growth after ADA.PCDAI significantly decreased from 52.1 ± 16 to 30.4 ± 23(P ≤ 0.001).Post ADA,body mass index(BMI) standard deviation score(SDS) 0.1[range:2.7-(-0.8)] vs-1 [range:0.1-(-3.6)],P = 0.04 and △BMI SDS in children 0.3 [range:0.7-(-0.2)] vs-1.1 [range:1.2-(-2.3)],P = 0.01 in remission were significantly higher compared to those with moderate to severe inflammation.The main predictors for growth were 25-hydroxycholecalciferol and for bone mineralisation weight and height SDS.ADA had no significant influence on bone markers and BMD.CONCLUSION:Next to improvement of PCDAI,half of the children achieved a positive catch-up growth.A better nutritional status with improvement in BMI and weight is positive predictor for improved growth and bone mineralisation.

2-deoxy-2-(^(18)F)fluoro-D-glucose positron emission tomography/computed tomography imaging in paediatric oncology

Positron emission tomography(PET) is a minimally in-vasive technique which has been well validated for the diagnosis, staging, monitoring of response to therapy, and disease surveillance of adult oncology patients. Tra-ditionally the value of PET and PET/computed tomogra-phy(CT) hybrid imaging has been less clearly defined for paediatric oncology. However recent evidence has emerged regarding the diagnostic utility of these mo-dalities, and they are becoming increasingly important tools in the evaluation and monitoring of children with known or suspected malignant disease. Important indi-cations for 2-deoxy-2-(18F)fluoro-D-glucose(FDG) PET in paediatric oncology include lymphoma, brain tumours, sarcoma, neuroblastoma, Langerhans cell histiocytosis, urogenital tumours and neurofibromatosis type Ⅰ. This article aims to review current evidence for the use of FDG PET and PET/CT in these indications. Attention will also be given to technical and logistical issues, the description of common imaging pitfalls, and dosimetric concerns as they relate to paediatric oncology.