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Since the inception of the associating liver partition and portal vein ligation for staged hepatectomy (ALPPS) procedure, many centres have used this technique for patients who would otherwise be considered unresectable due to insufficient future liver remnant. In this report, we presented the case of a paediatric patient with recurrent hepatocellular carcinoma who underwent monosegment ALPPS (M-ALPPS) hepatectomy preserving segment 1 as the sole liver remnant using indocyanine green (ICG) as a fluorescence guide.

Undifferentiated embryonal sarcoma of the liver (UESL) is a rare liver tumour that occurs mainly in children. Herein, we aimed to identify any differences in clinical characteristics and survival between adult and paediatric patients with UESL.From 1975 to 2015 in the Surveillance, Epidemiology and End Results (SEER) database, patients diagnosed with UESL were identified and divided into paediatric (18 years) and adult (≥18 years) groups. We then compared the clinical characteristics, management, and overall survival (OS) of adults and children diagnosed with UESL.We analysed 113 patients with UESL (81 children and 32 adults). UESL was significantly more common in adult male than paediatric male patients (71.9% vs. 48.2%; P = 0.022). When compared to adult patients, paediatric patients were more likely to receive chemotherapy (93.8% vs. 65.6%; P 0.001). Adults had a significantly worse OS than paediatric patients (5-year OS, 30.0% vs. 81.2%; P 0.001). Univariate analysis found that adult age, surgical therapy and chemotherapy were associated with OS. Multivariate analysis revealed that adult age, SEER summary stage and surgical therapy were independent prognostic factors for OS.UESL had a male predominance among adult patients. Moreover, the prognosis of adult patients with UESL was significantly worse than that of paediatric patients. Surgery and chemotherapy should be considered in the treatment of patients with UESL.

OBJECTIVES: To quantify the availability of authorised, age-appropriate paediatric medicines in clinical practice in the Netherlands and to identify gaps by assessing dispensing practice in a paediatric hospital. METHODS: The availability of age-appropriate formulations was assessed by conducting a survey on the use of pharmacy compounded medicines among the paediatric hospitals in the Netherlands, and by analysing dispensing data of oral medication from the inpatient pharmacy of the largest paediatric hospital in the Netherlands. The age-appropriateness of the dispensed formulations was assessed on two aspects: dose-capability and acceptability. Liquid drug products that are unsuitable due to the presence of potentially harmful excipients, were identified based on the dosage in clinical practice. RESULTS: For 129 out of 139 drug substances included in the survey (93%), at least one of the eight respondents stated to use a pharmacy compounded product to meet the needs of their paediatric patients. The age-appropriateness of medicines dispensed from the inpatient pharmacy increased with age, and was higher for non-intensive care unit (ICU) patients than for ICU patients. We identified 15 drug products causing excipient exposure above the European Medicines Agency-recommended values. CONCLUSIONS: This study confirms there is still a large need for age-appropriate formulations in daily clinical practice. Pharmacy compounding for paediatric patients remains essential for many indications. The need for potentially harmful excipients in compounded products should be critically assessed. ispartof: EUROPEAN JOURNAL OF HOSPITAL PHARMACY vol:28 issue:6 pages:306-312 ispartof: location:England status: published

The majority of patients who sustained small (low % total body surface area [TBSA]) burns are assessed in an outpatient setting. This can be a traumatic experience, particularly, for paediatric patients. During the initial assessment pharmacological and non-pharmacological adjuncts, such as distraction therapy, can be employed to provide an environment that may reduce and minimise distress. In this study, we investigated whether distraction therapy reduces objective pain scores during the outpatient assessment of small acute burns in children.Two cohorts of patients were formed. In the first group (group A), the children received analgesia and routine nursing care. In the second group (group B), the children received specialist distraction therapy in addition to the same analgesia and nursing care. We recorded patient demographics, TBSA, anatomical sites and mechanism of burn. The Wong Baker™ visual analogue scale (VAS) was used to convert perceived pain, as reported by the patient or parent, into a numerical value at three set intervals during the consultation.50 patients were recruited with 32 patients in group A and 18 in group B. A larger TBSA positively correlated with higher pain scores (p0.05). At the start of the consultation the mean pain score without distraction therapy was 1.55 and with specialist distraction therapy was 0.33 (p0.05). The mean peak pain score during dressing change without distraction therapy was 6.80 and with specialist distraction was 4.47 (p0.05). At the end of the procedure, the mean pain score was 2.12 without distraction therapy and with specialist distraction 1.75.This study of pain scores in small acute paediatric burns has shown that distraction therapy provided by a qualified play specialist can reduce maximal pain by over 2 points on the Wong-Baker™ VAS. Consideration should be given to ensure that distraction therapy is available at all times during initial consultations for children who have sustained small burns.

An 8-year-old boy presented with fever, vomits, bloody diarrhoea, and blurred vision. The patient was diagnosed with Haemolytic Uraemic Syndrome (HUS) due to the symptoms and a positive Verotoxin stool test. Funduscopic examination showed retinal involvement in both eyes, peri-papillary paleness, retinal haemorrhages, and soft "Purtscher Fleckens" exudates. A favourable outcome was achieved after hospital admission and systemic treatment. Dialysis treatment was not needed due the preserved diuresis. Although Purtscher-like retinopathy is very uncommon, ocular examination is mandatory in patients with pancreatitis, autoimmune diseases, and thrombotic microangiopathies, such as HUS.

Resumen: Introducción: El seguimiento telefónico es una posible alternativa para la atención médica de niños con COVID-19. Nuestro objetivo es describir la experiencia del seguimiento telemático realizado en un hospital terciario. Pacientes y métodos: Estudio descriptivo retrospectivo de los niños con diagnóstico confirmado o probable de COVID-19 atendidos en la consulta de seguimiento del Hospital La Paz entre marzo y junio del 2020. Se realizaron llamadas cada 48 h hasta desaparecer los síntomas y posteriormente semanales hasta estar 14 días asintomáticos. Resultados: Se incluyó a 72 niños con una mediana de edad de 83,5 meses (RIC = 16,3-157,5); 46 eran varones (63,9%) y 14 tenían comorbilidades (19,4%); 32 pacientes (44,4%) habían requerido ingreso hospitalario. Se confirmó diagnóstico de COVID-19 en 33 niños por PCR y en 7 por serología. De los confirmados por PCR, seroconvirtió el 67,7%. Se demostraron otras etiologías en 7 pacientes (5 Mycoplasma pneumoniae, uno parvovirus y uno citomegalovirus).La mediana de duración de síntomas fue 25,5 días (RIC = 13,8-37), con mediana de tiempo de seguimiento de 28 días (RIC = 21-39). Se realizó una mediana de 6 llamadas (RIC = 4-8) por niño; 19 pacientes (26,4%) refirieron empeoramiento en el seguimiento, precisando 14 (19,4%) ser revaluados en Urgencias. Un niño necesitó reingresar, siendo la evolución favorable. Conclusiones: Ante sospecha de COVID-19, es recomendable el seguimiento médico por la prolongada duración de síntomas y el riesgo de empeoramiento. Las consultas telefónicas son útiles y seguras para el seguimiento de casos leves y pacientes hospitalizados tras el alta, permitiendo reducir controles presenciales y el consumo de otros recursos. Abstract: Introduction: Telemedicine is an attractive option for the follow-up of paediatric patients with SARS-CoV-2 infection. The aim of this article is to describe the experience with telephone consultations in a tertiary hospital. Patients and methods: Retrospective descriptive study of children with confirmed or probable diagnosis of COVID-19 attended by telephone consultations in Hospital La Paz (Madrid) between March and June 2020. Patients were referred from the Emergency Department after being discharged from the hospital. Telephone consultations were made every 48 hours until symptoms resolved, then weekly until completing 14 days without symptoms. Results: A total of 72 children were included, with median age of 83.5 months (IQR = 16.3-157.5). Of those 46 (63.9%) were male, and 14 (19.4%) had comorbidities. There were 32 (44.4%) hospital admissions. COVID-19 diagnosis was confirmed in 33 children by RT-PCR, and in 7 by serology tests. The seroconversion rate was 67.7% in those patients with a positive RT-PCR. Other infections were found in 7 patients (5 Mycoplasma pneumoniae, 1 parvovirus, and 1 cytomegalovirus).Median symptom duration was 25.5 days (IQR = 13.8-37), while median follow-up duration was 28 days (IQR = 21-39). The median number of telephone consultations per patient was 6 (IQR = 4-8). Clinical worsening was reported in 19 (26.4%) during follow-up, and 14 (19.4%) were re-evaluated in the Emergency Department. One patient required hospital admission, but he had a favourable outcome. Conclusions: Children with suspected SARS-CoV-2 infection should be followed-up due to prolonged duration of symptoms, and the risk of clinical deterioration. Telephone consultations are a useful and safe alternative for the follow-up of patients with mild symptoms, and for children discharged from the hospital.

IntroductionTriage is a key principle in the effective management of major incidents. There is currently a paucity of evidence to guide the triage of children. The aim of this study was to perform a comparative analysis of nine adult and paediatric triage tools, including the novel ‘Sheffield Paediatric Triage Tool’ (SPTT), assessing their ability in identifying patients needing life-saving interventions (LSIs).MethodsA 10-year (2008–2017) retrospective database review of the Trauma Audit Research Network (TARN) Database for paediatric patients (15. Primary analysis was conducted on patients with complete prehospital physiological data with planned secondary analyses using first recorded data. Performance characteristics were evaluated using sensitivity, specificity, undertriage and overtriage.Results15 133 patients met TARN inclusion criteria. 4962 (32.8%) had complete prehospital physiological data and 8255 (54.5%) had complete first recorded physiological data. The majority of patients were male (69.5%), with a median age of 11.9 years. The overwhelming majority of patients (95.4%) sustained blunt trauma, yielding a median ISS of 9 and overall, 875 patients (17.6%) received at least one LSI. The SPTT demonstrated the greatest sensitivity of all triage tools at identifying need for LSI (92.2%) but was associated with the highest rate of overtriage (75.0%). Both the Paediatric Triage Tape (sensitivity 34.1%) and JumpSTART (sensitivity 45.0%) performed less well at identifying LSI. By contrast, the adult Modified Physiological Triage Tool-24 (MPTT-24) triage tool had the second highest sensitivity (80.8%) with tolerable rates of overtriage (70.2%).ConclusionThe SPTT and MPTT-24 outperform existing paediatric triage tools at identifying those patients requiring LSIs. This may necessitate a change in recommended practice. Further work is needed to determine the optimum method of paediatric major incident triage, but consideration should be given to simplifying major incident triage by the use of one generic tool (the MPTT-24) for adults and children.

Behaviour management for anxious paediatric dental patients is challenging. Solutions include education and sedation. Various drugs have been used to effectively sedate paediatric patients during treatment. The aim of this study was to review literature on the sedation of paediatric patients. The study specifically looked at those reviews covering the combination of two sedation methods in case of more challenging paediatric patients. The study undertook a literature review focused on studies using nitrous oxide, Midazolam, or a combination of the two substances. An electronic search was done on EBSCOhost to source articles published from 1979 to 2019. A deeper form of sedation can be achieved for paediatric patients when using a combination of nitrous oxide, oxygen and a hypnotic agent such as Midazolam. Dealing with the anxiety levels of paediatric patients is a challenge for dental health providers. Two of the main strategies used to deal with anxious children are behaviour management and sedation. A critical review of journal articles on the use of nitrous oxide and oxygen in combination with Midazolam was therefore undertaken. The findings suggest that, in order to achieve a deeper form of sedation, the combination of nitrous oxide, oxygen and Midazolam works well to reduce discomfort, anxiety and/or pain in paediatric patients.

Background: Radiation protection in paediatric radiology deserves special attention as children are supposed to be more sensitive to radiation than adults. Aim: This study was designed to evaluate the radiation doses for paediatric patients undergoing diagnostic chest X-ray examination. Materials and Methods: This was a cross-sectional survey, which targeted paediatric patients of (0-10) years who were referred by their physicians for chest X-rays examination. The data used for this work were generated using a Thermoluminescence dosimeter (TLD) chip. One TLD chips was placed in the front of the chest to measure the Entrance Surface Dose (ESD) (µGy) and another at the back of the patient directly opposite the one in front to measure the Exit Dose (ED) (µGy). Patient dose, age, ESD, ED and absorbed dose were obtained. The obtained data were analyzed using descriptive statistics. Results: The mean ESD, ED and absorbed dose values were 1.8330± 1.122mGy, 1.0913± 0.7505mGy and 0.7520 ± 3.8160mGy respectively. The ED values in chest X-rays were 0.73± 0.30mGy for age group

The Klippel-Feil syndrome is a congenital anatomical defect which occurs due to the failure of fusion of two or more cervical vertebrae at the neck region. Features include a short neck, low hairline at the back of the head, and restricted movement of the upper spine. This case study includes audiological test battery of a 3 years old with Klippel-Feil syndrome with features of hearing impairment, severely dysplastic internal acoustic meatus [IAM] along with delayed speech and language development. A detailed audiological evaluation was done and the management options were been further discussed. Key words: Klippel-Feil syndrome, hearing loss, CI, ABI.

Introduction: Hospital Selayang started the liver transplant program in 2002. We report a total of 81 liver transplant cases until year 2015. Among these, paediatric cases constitute almost half. This report aims to review the demography and outcome of paediatric liver transplant cases. Methodology: Case notes of all paediatric patients underwent liver transplant from year 2002 to 2015 were retrospectively reviewed. Results: A total of 38 paediatric cases received liver transplantations from year 2002 to 2015. Age at transplantation ranged from 11 months to 16 years old (mean age 6 years) and weight ranged from 6.47 to 63 kilogram (mean 18kg). There were 20 males and 18 females, 20 of them were Malay, whereas Chinese and Indian were 13 and 4 respectively. Eighteen cases were living-related and 20 cases were cadaveric liver transplants. For recipient blood group, O+ is the commonest. Biliary atresia was the most common indication for liver transplant (22 cases; 58%), followed by intrahepatic cholestasis disorders (5 cases; 13%) and metabolic disease (4 cases; 10%). Post transplantation, there were 6 (16%) biliary complications, 12 (32%) vascular complications, 26 (68%) developed early onset infection, 13% had acute rejection and 2 graft failure. Out of the 38 transplants, 79% of them survive after 1 year, and 69.7% survive after 5 years. The common causes of mortality were sepsis and vascular complications. Conclusion: Despite limited resources, a successful paediatric liver transplant programme has been established in Hospital Selayang with good survival rate.

Pharmacovigilance is a tool proposed during the post-marketing process of the pharmaceutical product lifecycle to monitor drug safety in everyday life and to identify adverse drug reactions. The identification of adverse reactions, however, is a significant cause of concern and a challenge to pharmacovigilance structures. Regulators use three basic principles in determining the risk-benefit balance to decide whether to approve a drug or a biological product and to maintain it on the market: safety, quality and effectiveness. In particular, paediatric patients, especially new-borns and infants, are at risk of drug-related adverse reactions. Drugs are also prescribed in an unlicensed and/or off-label manner to new-borns, infants and teenagers, leading paediatric patients to a higher risk of experiencing adverse drug reactions (ADRs). ADRs in children < 2 years of age are often reported and can often be alarming. The practise of paediatric pharmacovigilance needs to be strengthened by stimulating spontaneous paediatric reporting and successful post-marketing surveillance. The current study highlights the importance of paediatric pharmacovigilance and the role of different stakeholders like healthcare providers, regulators, and consumers in increasing the ADR reporting rate. Also, it discusses the pharmacovigilance tools and various initiatives that are taken by various regulatory authorities like the United States, the United Kingdom, Japan, and India.

The present study aimed at estimating organ and effective doses from computed tomography (CT) scans of paediatric patients in three hospitals in Brazzaville, Congo Republic. A total of 136 data on paediatric patients, from 0.25 (3 months) to 15 years old, who underwent head, chest, abdomen – pelvis (AP) and chest – abdomen – pelvis (CAP) CT scans was considered. The approach followed in the present study to compute organ doses was to use pre-calculated volume CT dose index (CTDIvol) – and 100 milliampere-second (mAs) – normalized organ doses determined by Monte Carlo (MC) simulation. Effective dose were then derived using the international commission on radiological protection (ICRP) publications 60 and 103 formalism. For comparison purposes, effective dose were also computed using dose-length product (DLP) – to – effective dose conversion factors. A relatively high variation in organ and effective doses was observed in each age group due to the dependence of patient dose on the practice of technicians who perform the CT scan within the same facility or from one facility to another, patient size and lack of adequate training of technicians. In the particular case of head scan, the brain and the eye lens were delivered maximum absorbed doses of 991.81 mGy and 1176.51 mGy, respectively (age group 10-15 y). The maximum absorbed dose determined for the red bone marrow was 246.08 mGy (age group 1-5 y). This is of concern as leukaemia and brain tumours are the most common childhood cancers and as the ICRP recommended absorbed dose threshold for induction of cataract is largely exceeded. Effective doses derived from MC calculations and ICRP publications 60 and 103 tissues weighting factors showed a 0.40-17.61 % difference while the difference between effective doses derived by the use of k- factors and those obtained by MC calculations ranges from 0.06 to 224.87 %. The study has shown that urgent steps should be taken in order to significantly reduce doses to paediatric patients to levels observed in countries where dose reduction techniques are successfully applied.

AIM Aspartate aminotransferase (AST) is an enzyme expressed in several organs; therefore, AST elevation may reflect outside of liver pathology. AST elevation may also be associated with macro-AST (m-AST). The aim of this study was to evaluate the long-term course of children with prolonged isolated AST elevation and the prevalence of m-AST in our cohort. METHODS We retrospectively reviewed the medical charts of children diagnosed with prolonged isolated AST elevation and were evaluated for m-AST. RESULTS Thirty-two patients were included. AST elevation persisted for a median of 66.6 months and ranged from 1.23 to 12-fold upper limit of normal (ULN). Twenty-two percent were m-AST positive and 44% had borderline levels of m-AST. A statistically significant difference was found for age at presentation between the borderline and the positive m-AST groups (31 vs. 69 months, respectively. p = 0.045). None of the patients with elevated AST developed significant liver disease. CONCLUSION We confirm the benign course of prolonged isolated AST elevation in general and m-AST in particular. A fifth of the patients with isolated AST elevation were m-AST positive. No differences have been found in AST levels between negative, borderline or positive m-AST.

Objectives Immunosuppressed paediatric patients with rheumatic disease (RD) may be at risk for severe or critical disease related to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. Data remain scarce on coronavirus disease 2019 (COVID-19) outcomes in paediatric RD patients. The aim of this study was to determine the seroprevalence of SARS-CoV-2 IgG and to describe COVID-19 outcomes in immunosuppressed paediatric RD patients. Methods Patients diagnosed with RD before age 18 years and treated with at least one immunosuppressive medication for at least 3 months were enrolled from a tertiary paediatric rheumatology practice in New York and also underwent routine SARS-CoV-2 IgG testing from May to November 2020. A total of 571 patients were screened and 262 were enrolled. SARS-CoV-2 IgG-positive subjects were assessed for symptoms of COVID-19 infection. SARS-CoV-2 PCR results were recorded where available. Demographic, diagnostic, medication and outcome data were collected. Results Of 262 subjects (186 female), 35 (13%) were SARS-CoV-2 IgG positive; 17 (49%) had symptoms suggestive of COVID-19. Of the 17 patients who had SARS-CoV-2 PCR testing, 11 (65%) were PCR positive, 7 of whom were IgG positive. Most SARS-CoV-2 IgG-positive subjects were not PCR tested. The most common symptoms in IgG- and/or PCR-positive subjects were fever, fatigue and cough. No SARS-CoV-2 IgG- or PCR-positive subject developed severe or critical COVID-19 or required hospitalization. Conclusions This is the first report of clinical outcomes of SARS-CoV-2 infection and seroprevalence of SARS-CoV-2 IgG in a large cohort of paediatric RD patients. Most SARS-CoV-2 IgG-positive subjects had no symptoms of COVID-19 infection. Symptomatic subjects all had mild COVID-19 symptoms, suggesting that the risk of severe or critical COVID-19 in immunosuppressed paediatric RD patients is minimal.

Deviations from the normal appearance of pale, pink and healthy mucosa require close monitoring or intervention and may be a sign of an underlying systemic disorder. Therefore, it is important that there is timely identification of abnormalities, appropriate management in primary care and onward referral for investigation to specialist services when required. Oral ulceration is the most common soft tissue abnormality in children, with recurrent aphthous stomatitis being the most common type of ulceration. This article discusses the various causes of oral ulceration in addition to vesiculobullous conditions, which may affect the paediatric patient. CPD/Clinical Relevance: It is important that GDPs are aware of the range of causes of ulcers and blisters presenting in children and are aware of when to refer.

Introduction: The number of patients with complex chronic conditions (CCC) has increased in the last 20 years or so. There is limited data as regards the prevalence of CCC in the paediatric population and its impact on hospital admissions. The main objectives of this study are to determine the proportion of CCC in the paediatric hospital population and compare them with other groups of patients admitted (acute and chronic). Patients and methods: A descriptive, retrospective study was carried out in a tertiary maternity-paediatric hospital (from December 2016 to November 2017). All patients admitted into the Paediatric Department were recruited with a fortnightly frequency. A series of demographic, clinical, and pregnancy data were collected. In order to identify the level of complexity of the patients, the Clinical Risk Group (CRG) was used, with 3 groups being created: acute, chronic, and CCC. Statistics analysis was performed using SPSS v24. Results: A total of 1433 patients were included. The proportion of CCC on the Paediatric Ward was 14.4%. The CCC were older patients, mainly admitted due to decompensation or progression of their underlying disease, had a longer admission time, and required support in the Paediatric Intensive Care Unit (PICU) more often than that of the other sub-groups. Just under half (44.7%) of the CCC were carriers of a technological device. Of the total of long stays (>1 month), 71.3% had CCC. Conclusions: Patients with CCC require long hospital stays, a greater need of intensive care, and use of technology. New approaches to treatment and follow-up need to be established. They should be cost-effective, and at the same time decrease the impact of the disease on the children and their family. Resumen: Introducción: En las últimas décadas se ha incrementado el número de pacientes crónicos complejos (PCC). Existen pocos datos referentes a la prevalencia de PCC en población pediátrica y su impacto en los ingresos hospitalarios. Los objetivos principales de este trabajo son determinar en nuestro medio la proporción de PCC en hospitalización pediátrica y compararlos con otros grupos de pacientes ingresados (agudos y crónicos). Pacientes y métodos: Estudio descriptivo retrospectivo realizado en un hospital materno-infantil terciario (de diciembre 2016 a noviembre 2017). Se reclutaron a todos los pacientes ingresados en el servicio de Pediatría con una periodicidad quincenal. Se recogieron una serie de variables demográficas, clínicas y de gestión. Para identificar el grado de complejidad de los pacientes se utilizó el sistema de clasificación Clinical Risk Group (CRG) y se crearon 3 grupos: agudos, crónicos y PCC. Para el análisis estadístico se usó SPSS v24. Resultados: Se incluyeron 1.433 pacientes. La proporción de PCC en planta de Pediatría fue del 14,4%. Los PCC fueron pacientes de mayor edad, ingresaron principalmente por descompensación o progresión de su enfermedad de base, estuvieron ingresados más tiempo y precisaron soporte en la Unidad de Cuidados Intensivos Pediátricos (UCIP) con mayor frecuencia que los otros subgupos. Un 44,7% de los PCC era portador de dispositivos tecnológicos. Del total de largas estancias (>1 mes), el 71,3% fue de PCC. Conclusiones: Los PCC requieren de estancias hospitalarias largas, mayor necesidad de cuidados intensivos y uso de tecnología. Es necesario plantear nuevos enfoques de tratamiento y seguimiento que sean costo-eficientes a la vez que disminuyan el impacto de la enfermedad en el niño y su familia.

Introduction Telemedicine is a real-time interactive communication method where patients are assessed and treated remotely. Epilepsy is one of the most frequent neurological disorders in childhood. However, specialists and subspecialists are scarce in rural settings. In neurology, telemedicine has been shown to be useful in the treatment of epilepsy and other neurodevelopmental disorders. Methods We gathered data from patients diagnosed with epilepsy and managed through telemedicine consultations from January 2011 to February 2020. We collected data on the number of consultations, town/city of origin, treatment, aetiology, seizure type, treatment adherence, loss to follow up, discharges, and comorbidities. Results We identified a total of 467 patients (180 girls and 287 boys). The largest age group was pre-school children, with 160 cases (34.2%). Seizures were focal in 253 cases (54.2%) and generalised in 214 (45.8%). Seizure aetiology was unknown in the majority of patients (69.3%). Ninety percent of patients presented good treatment adherence, and 76.8% were seizure-free. Regarding treatment, 351 patients (75%) were receiving monotherapy, and the most frequently prescribed drug was magnesium valproate, in 225 patients (64.2%). A total of 158 patients (33.8%) presented 1 comorbidity, and 19 (4%) presented 2 comorbidities. Conclusions In our study, telemedicine proved to be effective in the treatment and follow-up of paediatric patients with epilepsy living in rural settings. Its usefulness in other neuropaediatric disorders should also be evaluated.

Objectives To show the feasibility of magnetic double-J-stent (mDJS) removal without general anaesthesia in infants and children. Methods A retrospective analysis of mDJS removals was conducted between February 2018 and July 2020 in a cohort of 32 consecutive paediatric patients. Only patients with unilateral ureteric stenting were included. Stent retrieval was performed in an outpatient setting. In males the junction of the retrieval-catheter and the mDJS was confirmed by transabdominal ultrasound. All patients were followed-up for 4-12 weeks after stent removal. Results Thirty-two patients (54% males) were included. Median age was 3.8 years (range 1 month - 15 years). Ureteral stents remained in place for a median of 67.5 days (range 2 days - 6 months). General anaesthesia was necessary in one single patient due to expressed patient's and caregiver's wish. Thirty-one patients had stent removal without need for general anaesthesia. Thereof, nitrous oxide was used in 12 patients (37.5%), fentanyl in 3 patients (9.4%), midazolam in 3 patients (9.4%), and 17 patients (53.1%) did not need sedation at all. Seven patients (21.9%) being 8 months or younger had received peroral saccharosis. No complications occurred during stent removal. Retrieval was successful at first catheterization in 30 patients (93.8%). Two male patients needed a second catheterization (6.3%). Conclusion The use of magnetic DJS is safe in the paediatric population and spares general anaesthesia during removal in almost all patients.

Aims: To compare the clinical efficacy of 1% 2-chloroprocaine and 0.5% bupivacaine heavy for saddle block in patients undergoing perianal day care surgeries. Settings and Design: After obtaining Institutional ethical clearance and written informed consent, 80 patients posted for elective perianal surgeries at SNMC and HSK Hospital were allocated into two groups by computer generated random numbers (1:1 ratio). Group A received 2ml of 1% 2-Chloroprocaine and Group B received 2ml of 0.5% Bupivacaine heavy. Double-blinding was done where neither the patient nor the investigator knew about the drug. Materials and Methods: In the OT standard anesthetic protocols were employed. Saddle block was given with bupivacaine heavy and 2-chloroprocaine and the following parameters, time to eligibility for discharge from hospital, length of stay in PACU, time to ambulate and void urine and any other complications were noted. Statistical analysis used: Data were entered in MS-Excel and analysed in SPSS V22. Descriptive statistics were represented with percentages, Mean with SD. Chi-square test, Independent t-test were calculated. P Results: Mean time for eligibility to discharge from hospital between groups were statistically significant with group A having less mean time (235.8+22.8 min) as compared to group B(337.1+16.4 min). Conclusion: Saddle block with 2-Chloroprocaine in perianal day care surgeries provides adequate duration and depth of surgical anesthesia with the advantages of faster block resolution and earlier hospital discharge. Keywords: Saddle anesthesia, Ambulatory surgeries, 2- Chloroprocaine.

AIM This study aimed to investigate parenchymal changes in the liver in paediatric patients with cystic fibrosis (CF) and to analyse diagnostic performance of two-dimensional shear wave elastography (2D-SWE) for the detection of hepatic involvement. METHODS Patients with CF treated and followed at our centre were evaluated prospectively. All patients underwent liver tissue stiffness (TS) measurements by 2D-SWE, in addition to routine clinical assessments, laboratory work-up and abdominal ultrasound imaging. Data from patients with CF were compared with healthy control subjects. RESULTS This study included 39 patients with CF and 37 healthy controls. Patients had a mean body weight of 29.9 (16.6-55) kg, mean age of 9 (5-17) years, mean height of 130 (107-172) cm and a mean body mass index of 16.1 (12.8-21.4) kg/m2 . Average SWE values of the liver were 1.02 (0.70-1.60) m/s in patients with CF (n = 39) and 0.89 (0.60-1.35) m/s in healthy controls (n = 37). Cystic fibrosis patients had significantly increased tissue stifness by liver elastography compared to controls (P = 0.005). CONCLUSION Parenchymal liver changes may occur early in cystic fibrosis, which cannot be detected by conventional ultrasonography but may be demonstrated by 2D-SWE. Based on this cross-sectional study, 2D-SWE may be a promising, simple and non-invasive modality for objective monitoring of patients with cystic fibrosis who require lifelong follow-up, by providing numerical data for tissue stiffness early in the disease.

AimsHyponatraemia is the most common body fluid disorders but often goes unnoticed. Our laboratory incorporated a standardised procedure to help clinicians detect moderate/severe hyponatraemia. The study aims were to evaluate the outcomes on patient care and clinicians’ satisfaction.MethodsThe study, observational and retrospective, included 1839 cases, adult and paediatric patients, with sodium concentration ResultsThe median hyponatraemia length decreased significantly from 4.95 hours (2.08–16.57) in the first period to 2.17 hours (1.06–5.39) in the second period. The lack of hyponatraemia patients follow-up was significantly less after the procedure implementation. The survey was answered by 92 (60 senior specialists and 32 residents) out of 110 clinicians surveyed. Ninety of them (98%) answered positively.ConclusionsWe have demonstrated the reduction in the time for diagnosing and management by physicians, the higher uniformity in the time required to solve hyponatraemia episodes following our laboratory procedure and the clinicians’ satisfaction.

BACKGROUND: TMC207-C211 (NCT02354014) is a Phase 2, open-label, multicentre, single-arm study to evaluate pharmacokinetics, safety/tolerability, antimycobacterial activity and dose selection of bedaquiline (BDQ) in children (birth to METHODS: Patients received 24 weeks’ BDQ with an anti-MDR-TB background regimen (BR), followed by 96 weeks of safety follow-up. Results of the primary analysis are presented based on data up to 24 weeks for Cohort 1 (≥12–RESULTS: Both cohorts had 15 patients, of whom respectively 53% and 40% of Cohort 1 and Cohort 2 children had confirmed/probable pulmonary MDR-TB. Most patients completed 24 weeks´ BDQ/BR treatment (Cohort 1: 93%; Cohort 2: 67%). Geometric mean BDQ area under the curve 168h values of 119,000 ng.h/mL (Cohort 1) and 118,000 ng.h/mL (Cohort 2) at Week 12 were within 60–140% (86,200–201,000 ng.h/mL) of adult target values. Few adverse event (AE) related discontinuations or serious AEs, and no QTcF >460 ms during BDQ/BR treatment or deaths occurred. Of MGIT-evaluable patients, 6/8 (75%) Cohort 1 and 3/3 (100%) Cohort 2 culture converted.CONCLUSION: In children and adolescents aged ≥5–

Yi Ren, Tiehua Zheng, Lei Hua, Fuzhou Zhang, Yangwei Ma, Jianmin Zhang Department of Anesthesiology, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health, Beijing, People’s Republic of ChinaCorrespondence: Jianmin ZhangDepartment of Anesthesiology, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health, No. 56, South Lishi Road, Beijing, 100045, People’s Republic of ChinaTel +861059616415Fax +861059616429Email zjm_bch@163.comPurpose: The Nuss procedure is a recognized treatment for adolescent pectus excavatum that results in severe postoperative pain. Erector spinae plane block (ESPB) is a novel technique that provides postoperative analgesia and reduces opioid consumption. Our aim is to explore whether ESPB produces analgesia similar to thoracic epidural anaesthesia (TEA) in paediatric patients undergoing Nuss procedure.Study Design and Methods: This randomized, controlled, non-inferiority trial will enrol 300 paediatric patients undergoing Nuss surgery. Participants will be randomly assigned 1:1 to receive ESPB or TEA preoperatively. The primary, joint endpoint is the average numeric rating scale (NRS) score and cumulative sufentanil consumption. The secondary endpoints are pain scores and sufentanil consumption at different time points after surgery, analgesia-related side effects, and other postoperative complications. Data will be analysed by the intention-to-treat principle.Discussion: This study investigates the effect of ESPB on postoperative opioid consumption and pain scores and intend to provide a new strategy of analgesia management for Nuss procedure in paediatric patients.Keywords: postoperative pain, erector spinae plane block, pectus excavatum, Nuss procedure, opiates

Advances in paediatric anaesthetic equipment and monitoring continue to be made. While the mainstay of airway intubating equipment currently is the direct laryngoscope, video laryngoscopes and endoscopes are increasing in their use. These continue to evolve, generating better quality pictures, with more sophisticated yet easier to use equipment. Vascular access in paediatric anaesthesia can be challenging with an increasing number of patients presenting with difficult intravenous access. Ultrasound has become an integral piece of equipment in the management of these children. As the population increases in weight, so the management of the obese paediatric patient is now a reality. This requires thought and careful planning of their perioperative care. Newer techniques such as high-flow nasal oxygen are useful both to prevent hypoxia at induction, but also to facilitate open airway surgery. Neurological monitoring in the form of near infrared spectroscopy and bispectral index are discussed with evidence relevant to paediatric practice. This article will discuss all of these devices and techniques with particular emphasis on paediatric anaesthetic practice.

To determine the degree of agreement and differences between macular parameters according to sex, age, laterality, ethnicity and type of optical coherence tomography (OCT) in paediatric patients.Prospective, transversal, and comparative study. OCT Spectralis and OCT Cirrus were used to obtain the macular values of both eyes in 100 patients between 5 and 15 years without ocular pathology. Due to the similarity between the values of both eyes, in the final sample we only considered the right eyes (OD) (n = 100).The average central macular thickness (CMT) was 244.2 μm with Cirrus and 261.7 μm with Spectralis. The degree of agreement for the GMC was moderate (Intraclass Correlation Coefficient: 0.66) and from low to very low in the other quadrants. The GMC values of both systems were significantly higher in men than in women (P .05). The macular retinal nerve fibre layer (mRNC), ganglion cell layer (GCC), the inner plexiform layer (IPLC), and the inner nuclear layer (INFL) had significantly higher values in men than in women (P .01). There were no statistically significant differences in the GMC by age and laterality, however it was significantly higher in ethnic European children than in ethnic Latin American children; Cirrus (P = .042) and Spectralis (P = .033).Macular parameters obtained by OCT Spectralis and OCT Cirrus should not be interchangeable in pediatric patients.

Background: The incidence of difficult airway in paediatric patients is lower than that the adult population, and the majority should be predictable. Aims: The primary aim of this trial was to evaluate the incidence of difficult airway in pediatric patients. The secondary aim was to predict difficult airway in these patients. Methods: Paediatric patients undergoing elective surgery under general anaesthesia in a tertiary university hospital were examined, and a panel of difficult airway prediction tests was performed. We recorded the incidence, risk factors for difficult airway and events associated with difficult airway together with the sensitivity and specificity of tests for difficult airway and events associated with difficult airway prediction. Results: We prospectively included 389 paediatric patients. The incidence of difficult airway was 3.6%; the incidence of events associated with difficult airway was 10%. The sensitivity for prediction of events associated with difficult airway during the pre-anaesthesia evaluation was 5.3% with the specificity 93.3%. In the operating room, the sensitivity of prediction was 15% with 97.8% specificity. Conclusion: We found minimal efficacy for preanaesthesia difficult airway prediction.

The treatment of paediatric mandibular condylar fracture (PMCF) is typically non-operative. The purpose of this study was to determine if non-operative management of PMCF results in a new condylar process of normal morphology to regenerate after closed treatment (restitutional remodelling). The specific aim of the study was to observe restitutional remodelling (RM) in PMCF and review the literature. The investigators designed and implemented a retrospective study on paediatric patients (age12) with unilateral or bilateral condyle fractures treated with non-operative treatment between January 2005 and July 2015. Patients with complete records and at least 1-year follow-up were included in the study. Primary outcome variable was RM and secondary outcome variables were occlusion, maximal incisal opening (MIO), displacement, infection, facial asymmetry, and signs of temporomandibular joint ankylosis (TMJA). The study evaluated 41 patients {n=57 PMCF, (m:f-35:6)} of unilateral (n=25) and bilateral (n=16) PMCF. Fractured condyles remodelled to normal morphology in all the cases at follow-up. The Wilcoxon test revealed a statistically significant difference in MIO from the preoperative value to postoperative (p=0.001). Occlusion (except 1) was satisfactory in all cases, at follow-up with no gross facial asymmetry. There was no sign of infection at the surgical site (anterior mandible). None of the patients showed signs of TMJA at follow-up. The result of the present study demonstrates that RM of condylar fracture occurs with non-operative management. Non-operative management should be the point of care in PMCF, owing to the rapid RM, bone regeneration, and satisfactory outcome. Review of the literature also supports closed treatment.

Rosai-Dorfman Disease (RDD) is also known as Sinus Histiocytosis of Massive Lymphadenopathy(SHML). Clinically Rosai-Dorfman Disease is characterized by massive, painless, bilateral cervicallymph node enlargement, often mimics lymphoma. Microscopically, it shows dilatation of lymphaticsinuses occupied by numerous lymphocytes and proliferation of histiocytes with abundant paleeosinophilic cytoplasm containing engulfed lymphocytes or plasma cells- emperipolesis.

Belumosudil (REZUROCK™) is a Rho-associated coiled-coil-containing protein kinase (ROCK) inhibitor that has been developed by Kadmon Pharmaceuticals for the treatment of chronic graft-versus-host disease (cGVHD) and systemic sclerosis. In July 2021, belumosudil received its first approval in the USA for the treatment of adult and paediatric patients aged ≥ 12 years with cGVHD after failure of at least two prior lines of systemic therapy. Belumosudil is under regulatory review in Australia, Canada, the UK and Switzerland for cGVHD. Clinical development for systemic sclerosis is ongoing in the USA. This article summarizes the milestones in the development of belumosudil leading to this first approval for the treatment of cGVHD. Supplementary Information The online version contains supplementary material available at 10.1007/s40265-021-01593-z.

BACKGROUND Caudal epidural block is a highly popular method of administering analgesia to children undergoing infraumbilical surgeries. Addition of clonidine, a centrally acting alpha 2 agonist as an adjuvant to caudal bupivacaine has added advantages of providing prolonged post-operative analgesia and also, thus reducing the number of doses of post-operative rescue analgesics. In this study, we wanted to compare the efficacy and safety of caudally administered clonidine as an adjuvant to bupivacaine. METHODS This was a prospective, comparative interventional study and was conducted in the Department of Anaesthesia at JNMC, Sawangi (Meghe) Wardha in accordance with the guidelines and permission of the institutional ethical review board. The study was carried out with 40 patients in each group randomly allocated using computer generated software, posted for infraumbilical surgeries between the age group 2 years to 7 years, American Society of Anaesthesiologists (ASA) grade I and II. Group B (control group) – Caudal epidural with 0.25 % bupivacaine 1 ml/kg + 1 ml normal saline. Group C (study group)- Caudal epidural with 0.25 % bupivacaine 1 ml/kg with clonidine 1 microgram/kg + 1 ml normal saline. RESULTS In our study, the mean duration of analgesia i.e. mean time for requirement of first rescue analgesia in group C was 9.9 ± 1.4 hours as compared to 4.7 ± 1.4 hours in group B (P < 0.0001). In this study, we used FLACC (face, legs, activity, cry, consolability) pain scale with its 0 - 10 score range for assessing the pain intensity. At FLACC pain score 4 or more, first dose of rescue analgesic was given. This score was reached at 8.8 hours in Group C (mean FLACC score at 3.8 ± 0.7) and 3.6 hours in Group B (mean FLACC score 3.4 ± 0.9) with P value (= 0.014) (significant). Group C required significantly lesser number of rescue analgesics in comparison to Group B. The number of doses of rescue analgesics required in the Group B were definitely more than that in Group C wherein 62.5 % required 3 doses and 25 % required 4 doses of rescue analgesics. On the other hand, in Group C the number of patients requiring more than 2 doses of rescue analgesia was 0 % thus proving the efficacy of clonidine in prolonging post-operative analgesia. CONCLUSIONS We concluded that caudally administered clonidine, in a dose of 1 microgram/kg as an adjuvant to 0.25 % bupivacaine 1 ml/kg, proved to be efficacious in providing prolonged analgesia both intraoperatively and post-operatively. Also, we observed that this addition, reduced the requirement of post-operative rescue analgesia and its associated side effects. KEY WORDS Caudal Epidural, Infraumbilical Paediatric Surgery, Clonidine, Bupivacaine, Postoperative Analgesia

AIM To describe the process of linguistic and content validity of the Swedish version of the PedsQL™ Gastrointestinal Symptoms Scales and Symptoms Module, measuring health-related quality of life (HRQOL) in children with gastrointestinal (GI) disorders. METHODS The establishment of linguistic and content validity was carried out in compliance with international standards on patient-reported outcome measurements. The process included forward translation, expert review and reconciliation, backward translation, backward translation review and interviews with 15 children aged 5-18 years with GI tract symptoms and 20 parents of children with GI tract symptoms aged 2-18 years. RESULTS The Swedish version of the PedsQL™ Gastrointestinal Symptoms Scales and Symptoms Module (child report 5-18 years, parent report for children 2-18 years) was achieved without major difficulties. Eight issues needed discussion after forward translation, and there was one change after backward translation and three revisions following patient and parent testing. CONCLUSION A conceptually equivalent Swedish language version of PedsQL™ Gastrointestinal Symptoms Scale and Symptoms Module for children aged 2-18 years old was developed. This enables improved HRQOL evaluations in children with GI disorders in Sweden. Future research using a larger sample is recommended to evaluate validity and reliability of the Swedish language version of the module.

Background: Bicytopenia and Pancytopenia are relatively common presentations in adults as compared to paediatric patients. Clinical features present in different manifestations in children with Pancytopenia/Bicytopenia, which can range from bone marrow suppression temporarily to marrow infiltration by life-threatening malignancies. Aim: To study the clinical and haematological profile in the paediatric age group withCytopenias. The study aims to analyze the severity of hematological findings as per causative factors in patients with Pancytopenia and Bicytopenia. Methods: Prospective Observational Study conducted in the Department of Paediatrics in Dr.RPGMC Tanda Himachal Pradesh (INDIA). The study included all patients except those falling in the exclusion criteria.It was performed after oral and written informed consent.A total of 50 children admitted with bicytopenia and pancytopenia were enrolled from March 2013 to March 2014. Results: Infection was the commonest etiology observed in 22(44%) patients with bicytopenia and pancytopenia. Bicytopenia was seen in 14 (20%) and pancytopenia in 8 (16%) cases with infection. Scrub typhus was the commonest Infection seen in 18(32%).Leukaemia was notedin 11(22%) cases. Conclusions: The commonest age group affected was 11-15 years with female domination with a Male: Female ratio of 0.78:1. The commonest symptom was fever 37(74%) then generalized weakness in 29 (58%) cases.Pallor was the most common sign in 43(86%) followed by Hepatomegaly 32(64%), Lymphadenopathy 30(60%), and Splenomeagly in 29 (58%) patients. Infection was the commonest etiology followed by leukemia and megaloblastic anaemia. Scrub typhus was the commonest infection encountered in the present study.