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关键词： Pediatrics   Neurofibromatosis   Urology   LUTS   Bladder   Review  

摘要： The Summary Introduction Neurofibromatosis Type 1 (NF1) is an autosomal dominant, multisystem, neurocutaneous disorder. This condition has been associated with lower uri-nary tract dysfunction due to either direct genito-urinary organ involvement or spinal cord compression. Based on current literature, there are no reviews examining the relationship between NF1 and lower urinary tract dysfunction (LUTD) in the paediatric population. Methods A critical review of the literature was conducted using a systematic search of MEDLINE, PubMed and Embase yielding a total of 1285 manuscripts pub-lished up to 2019. Two independent reviewers selected studies for screening, eligibility and inclu-sion into the review. Following title, abstract and full -text review, 46 articles were analyzed. Results Within these 46 articles, 79 cases were presented. The mean patient age at the time of presentation was 6.97 +/- 9.19 years. The most common urologic presentations were irritative lower urinary tract symptoms (30%) and a newly discovered abdominopelvic mass (21%). Diagnostic investigations commonly demonstrated a retrovesical mass with direct invasion of the bladder in 58 cases (73%) and other genitourinary organ involvement in 39 cases (39%). Throughout the total case volume, LUTD was present in 49%. Pathology of malignancy was most commonly malignant peripheral nerve sheath tumour and rhabdomyosarcoma (20% and 14%, respectively). When indicated, surgical management of the lower urinary tract included both radical and partial cystectomy. Conservative management of urinary retention included clean intermittent catheterization (56%), suprapubic catheterization (22%), vesicostomy creation (11%) and mitrofanoff creation (11%). Conclusion A complete urologic evaluation including clinical, radiologic, and possibly pathologic investigation is warranted and should be performed for both diagnosis and appropriate management of LUTD in patients with NF1.

关键词： Hydrocephalus   Neurofibromatosis   Aqueduct stenosis   ETV   VP shunt  

摘要： Objectives: Aqueduct stenosis (AS) and fourth ventricle outflow obstruction are rare associations of neurofibromatosis type 1 (NF1), resulting in ventriculomegaly and hydrocephalus requiring surgical treatment. This study aims to identify the prevalence of AS and its patterns of clinical presentation, aetiology and treatment in the paediatric complex NF1 population. Patients and Methods: Patients with NF-1 aged 0-18 years were recruited from the Regional Genetic Family Register, following institutional review board approval. Magnetic resonance imaging data and clinical documents were reviewed with respect to clinical presentation, degree of ventriculomegaly, aetiological factors and management of AS and fourth ventricle outflow obstruction. Results: 24 of the 233 paediatric patients seen within the NHS highly specialised service for complex NF1 were found to have AS or and fourth ventricle outflow obstruction. This included 13 males and 11 females with a mean age of 9 years 5 months (range 8 months - 17 years). The majority of patients with AS or fourth ventricle outflow obstruction presented with symptoms of raised intracranial pressure associated with ventriculomegaly and/or hydrocephalus (n = 18). However, in 25 % of patients, AS was an incidental finding on MRI and was observed both in the presence (n = 2) and absence (n = 4) of ventriculomegaly. In the majority of cases a single cause of AS was identified (n = 16), of which rectal plate thickening (n = 7) was most frequently observed. The remaining 8 patients had multiple causes of AS, in which rectal plate thickening (n = 7) and aqueductal webs (n = 5) were the most common observations. Surgery was performed on all patients with evidence of raised pressure (n = 8) by performing endoscopic third ventriculostomy (ETV) (n = 5) or ventriculoperitoneal (VP)-shunting (n = 3). Tectal plate thickening was most frequently observed in patients who underwent ETV (n = 3), followed by aqueductal web (n = 1) and T2-signal c

关键词： Post-operative paediatric cerebellar mutism syndrome   Cerebellar mutism syndrome   Posterior fossa syndrome   Radiology   Anatomical substrates   Pathophysiology  

摘要： Background Post-operative paediatric cerebellar mutism syndrome is a well-recognized complication following posterior fossa tumour resection in children. Over the past few decades, imaging has played an important role in understanding this disorder. Aim This review article aims to focus on the disorder from a radiological perspective, summarizing the salient radiological evidence related to the anatomical structures, pathophysiology, and risk factors related to this disorder. Conclusion Radiological studies have been integral to the improved understanding of this condition. Future large multicentre studies and quantitative analysis techniques will be vital in further refinement of our understanding of this complex condition.

关键词： Coronary sinus stenosis   paediatric chest pain   angiography  

摘要： Undiagnosed coronary sinus obstruction can be a source of paediatric exertional chest pain and death. We present a case series of three interesting children who developed only exertional angina from various degrees of coronary sinus stenosis or atresia. We discuss the diagnosis and management of each case and the potential pathophysiology involved with their symptoms.

关键词： inflammatory bowel disease   treatment   corona virus disease 2019   Crohn disease   children  

摘要： Introduction: With the current coronavirus disease 2019 (COVID-19) pandemic, concerns have been raised about the risk to children with inflammatory bowel diseases (IBD). We aimed to collate global experience and provide provisional guidance for managing paediatric IBD (PIBD) in the era of COVID-19. Methods: An electronic reporting system of children with IBD infected with SARS-CoV-2 has been circulated among 102 PIBD centres affiliated with the Porto and Interest-group of ESPGHAN. A survey has been completed by major PIBD centres in China and South-Korea to explore management during the pandemic. A third survey collected current practice of PIBD treatment. Finally, guidance points for practice have been formulated and voted upon by 37 PIBD authors and Porto group members. Results: Eight PIBD children had COVID-19 globally, all with mild infection without needing hospitalization despite treatment with immunomodulators and/or biologics. No cases have been reported in China and South Korea but biologic treatment has been delayed in 79 children, of whom 17 (22%) had exacerbation of their IBD. Among the Porto group members, face-to-face appointments were often replaced by remote consultations but almost all did not change current IBD treatment. Ten guidance points for clinicians caring for PIBD patients in epidemic areas have been endorsed with consensus rate of 92% to 100%. Conclusions: Preliminary data for PIBD patients during COVID-19 outbreak are reassuring. Standard IBD treatments including biologics should continue at present through the pandemic, especially in children who generally have more severe IBD course on one hand, and milder SARS-CoV-2 infection on the other. Supplemental digital content: An infographic accompanying this article can be found at.

关键词： SARS-CoV-2   COVID-19   paediatric   laboratory   biosafety  

摘要： There is currently limited knowledge about the transmission risks of the SARS-CoV-2 virus and its associated disease COVID-19 from routine clinical specimens. The first study to be published on the initial 41 cases of COVID-19 infections admitted in Wuhan detected SARS-CoV-2 RNA in the blood of 6/41 (15%) of patients (Huang et. al., 2020) . However, another study conducted on 1070 clinical specimens collected from confirmed COVID-19 patients in China showed the highest positive rates of SARS-CoV-2 from rRT-PCR testing of respiratory specimens such as bronchoalveolar lavage, sputum and nasopharyngeal swabs (32% - 93%). In contrast, only 1% of blood specimens and none of the urine specimens tested positive (Wang et. al., 2020).

关键词： child   emergency   hospital   parent   triage  

摘要： Objective To determine whether the characteristics and outcomes of parent-referred children differ substantially from general practitioner (GP)-referred children attending a metropolitan ED. Methods Prospective cross-sectional observational study of consecutive patients aged less than 16 years of age attending a mixed adult and paediatric metropolitan teaching hospital ED in Melbourne. Data collected included patient characteristics (age, sex, arrival mode and triage category) and outcomes (admission, specialty consultation, treatment time >1 h, intravenous or nasogastric therapy, procedural sedation, procedures, imaging studies and pathology collection). Results The outcome data of 4580 patients was analysed. Patients brought by parents directly to the ED did not differ from patients referred by their GP in age, sex, or triage category, but were more likely to arrive by ambulance (13% vs 2%, P < 0.01). Low-urgency GP-referred children were more likely to require a procedure (22% vs 15%, risk difference [RD] 0.07, 95% confidence interval [CI] 0.02-0.13) and pathology tests (13% vs 8%, RD 0.05, 95% CI 0.01-0.1) than parent-referred children, but were similar for other outcomes. The proportion of low-urgency children who had no ED-specific care interventions was not significantly different between the groups (27% vs 32%, RD -0.06, 95% CI -0.11 to 0). Conclusions Children brought to ED directly by their parents or carers did not differ substantially from GP-referred children in their characteristics or outcomes. The use of referral source and triage categories to determine patient suitability for diversion to general practice may need to be reconsidered.

关键词： Colistin   Pharmacokinetics   Pharmacodynamics   Paediatrics   Multidrug-resistant bacteria  

摘要： Use of colistin in children is rising in line with the increase of multidrug-resistant Gram-negative bacteria (MDR-GNB). In adults, a colistin loading dose is recommended to achieve therapeutic concentrations within 12-24 h. Here we aimed to describe the pharmacokinetic (PK) parameters of a loading dose versus a recommended initial dose of intravenous colistimethate sodium (CMS) in paediatric patients. A prospective, open-label, PK study was conducted in paediatric patients (age 2-18 years) with normal renal function. Patients (n = 20) were randomly assigned to receive either a CMS loading dose (LD group) of 4 mg of colistin base activity (CBA)/kg/dose or a standard initial dose (NLD group) of 2.5 mg (12-h interval) or 1.7 mg (8-h interval) of CBA/kg/dose. Serial blood samples were collected. Plasma concentrations of formed colistin were measured by LC-MS/MS. PK parameters were reported. Acute kidney injury (AKI) was monitored by serum creatinine and urine NGAL. The median (interquartile range) age and body weight were 8.5 (3.5-11.3) years and 21.5 (13.5-20.0) kg. The mean (standard deviation) of first-dose PK parameters of the LD group versus the NLD group were: C max , 6.1 (2.4) vs. 4.1 (1.3) mg/L;AUC(0-t) , 26.5 (12.5) vs. 13.5 (3.6) mg/L-h;V-d , 0.7 (0.4) vs. 0.6 (0.3) L/kg;and t 1/2 , 2.9 (0.6) vs. 2.6 (0.4) h. No patient developed AKI by serum creatinine criteria. A CMS loading dose is beneficial for improvement of colistin exposure without increased AKI. A higher daily dose of CMS should be considered, especially for MDR-GNB treatment. (C) 2020 Elsevier B.V. and International Society of Chemotherapy. All rights reserved.

关键词： bacteraemia   central catheterisation   chlorhexidine   critical care   infection control  

摘要： Aim The aims of the study are to evaluate the impact of a 4% chlorhexidine (CHG4%) bathing on the occurrence of central-line-associated bloodstream infection (CLABSI) and to identify risk factors (RFs) for CLABSI in our population. This is a retrospective monocentric cohort study in the paediatric surgical intensive care unit at the Necker Enfants Malades Hospital, Paris, France. Methods All hospitalised patients with central venous catheters (CVCs) in 2015 were included. CHG4% bathing was prescribed in CLABSI high-risk patients, defined by the presence of exposition factors (EFs): constitutive or acquired immunosuppression, presence of an invasive medical device (IMD) and the carriage of Staphylococcus aureus. The overall 2015 CLABSI incidence rate was compared with 2014 CLABSI incidence rate (before CHG4% bathing). Results In all, 775 patients were analysed. Some 182 had at least one EF, and 49 received CHG4%. The incidence rates of CLABSI in 2014 and 2015 were, respectively, 6.1 and 2.3/1000 days CVC (P < 0.01). The presence of at least one EF was associated with the CLABSI's occurrence: odds ratio = 15.13 (95% confidence interval: 4.26-53.71;P < 0.0001), particularly acquired immunosuppression, IMD and S. aureus colonisation. Other RFs were age 16 days. Conclusions This study showed a significant reduction in incidence of CLABSI after introduction of a targeted CHG4% bathing protocol. Presence of IMD, S. aureus colonisation, immunosuppression, age 16 days were CLABSI RFs. Regarding the literature, the presence of IMD seems to be underestimated in CLABSI prevention.

关键词： Drug-related problems   Incidence   Malaysia   Paediatrics   Risk factors  

摘要： Background Drug-related problems are relatively common among hospitalised patients and may be detrimental to patients and even increase healthcare costs. Characterising drug-related problems allows remedial actions to be in place to minimise the frequency and ensure higher medication safety for the patients involved. Currently, the incidence of drug-related problems among hospitalised paediatric patients in Malaysia is unknown. Objective To determine the incidence, types and intervention acceptance, as well as the risk factors associated with drug-related problems in a Malaysian general paediatric ward. Setting General paediatric ward in Universiti Kebangsaan Malaysia Medical Centre. Method A cross-sectional observational study was conducted from March to May 2019. Data were collected from patients' medical charts, clinical notes and medical records for problem identification based on the modified Pharmaceutical Care Network Europe (PCNE) classification V 8.02. The incidence, types, causes and intervention acceptance as well as the risk factors were assessed. Main outcome measure Drug-related problems based on the modified PCNE classification V 8.02. Results In total, 225 paediatric patients with a total of 694 prescriptions were included in this study. The incidence of drug-related problems was 52.9% (198 prescriptions with problems in 119 patients). The main types of problems were treatment safety (68 prescriptions, 34.3%), incomplete prescription (56 prescriptions, 28.3%) and un-optimised drug treatment (35 prescriptions, 17.7%). The main causes for the problems were necessary information not provided (n = 63, 30.1%), inappropriate dose selection (n = 47, 22.5%) and medication served without a valid prescription (n = 40, 19.1%). Of the 143 interventions proposed to prescribers by the pharmacist, 117 (81.8%) were accepted. The number of prescriptions was found to be the only risk factor associated with DRPs (odds ratio, 1.91;95% confidence interval, 1.54-2.38;p <