Analysis of factors conditioning inappropriate visits in a paediatric emergency department.

Recent studies estimated that about 20-30% of visits in a paediatric emergency department (PED) are inappropriate. Nonurgent visits have been negatively associated with crowding and costs, causing longer waiting and dissatisfaction among both parents and health workers. We aimed to analyze possible factors conditioning inappropriate visits and misuse in a PED. We performed a cross-sectional study enrolling children accessing an Italian PED from June 2022 to September 2022 who received a nonurgent code. The appropriateness of visits, as measured by the "Mattoni SSN" Project, comprises combination of the assigned triage code, the adopted diagnostic resources, and outcomes. A validated questionnaire was also administered to parents/caregivers of included children to correlate their perceptions with the risk of inappropriate visit. Data were analyzed using independent-samples t-tests, Wilcoxon-Mann-Whitney tests, chi-square tests, and Fisher's exact tests. The factors that were found to be associated with inappropriate visits to the PED were further evaluated by univariable and multivariable logistic regression analyses. Almost half (44.8%) of nonurgent visits resulted inappropriate. Main reasons for parents/caregivers to take their children to PED were (1) the perceived need to receive immediate care (31.5%), (2) the chance to immediately perform exams (26.7%), and (3) the reported difficulty in contacting family paediatrician (26.3%). Inappropriateness was directly related to child's age, male gender, acute illness occurred in the previous month, and skin rash or abdominal pain as complaining symptoms.     Conclusion: This study highlights the urgent need to finalize initiatives to reduce misuse in accessing PED. Empowering parents' awareness and education in the management of the most frequent health problems in paediatric age may help to achieve this goal. What is Known: • About 20-30% of pediatric urgent visits are estimated as inappropriate. • Several factors may be associated with this improper use of the emergency department, such as the misperception of parents who tend to overrate their children's health conditions or dissatisfaction with primary care services. What is New: • This study evaluated almost half of pediatric emergency department visits as inappropriate adopting objective criteria. • Inappropriateness was directly related to the child's age, male gender, acute illness that occurred in the previous month, and skin rash or abdominal pain as complaining symptoms. Educational interventions for parents aimed at improving healthcare resource utilization should be prioritized.

Frequency and type of domestic injuries among children during COVID-19 lockdown: what changes from the past? An Italian multicentre cohort study.

Accidents are the main cause of injury in children, more than half events happen at home. Aims of this study were to assess if SARS-CoV-2 lockdown influence emergency department (ED) visits due to children domestic accident (DAs) and to identify factors associated with hospitalization. This was a multicentre, observational, and retrospective cohort study involving 16 EDs in Italy and enrolling children (3-13 years) receiving a visit in ED during March-June 2019 and March-June 2020. Risk factors for hospitalization were identified by logistic regression models. In total, 8860 ED visits due to domestic accidents in children occurred before (4380) and during (4480) lockdown, with a mean incidence of DA of 5.6% in 2019 and 17.9% in 2020 (p < 0.001) (IRR: 3.16; p < 0.001). The risk of hospitalization was influenced by the type of occurred accident, with fourfold higher for poisoning and twofold lower risk for stab-wound ones. In addition, a higher risk was reported for lockdown period vs 2019 (OR: 1.9; p < 0.001), males (OR: 1.4; p < 0.001), and it increased with age (OR: 1.1; p < 0.001).    Conclusions: The main limitation of this study is the retrospective collection of data, available only for patients who presented at the hospital. This does highlight possible differences in the total number of incidents that truly occurred. In any case, the COVID-19 lockdown had a high impact on the frequency of DAs and on hospitalization. A public health campaign aimed at caregivers would be necessary to minimize possible risks at home. What is Known: • In Italy, domestic accidents are the second leading cause of paediatric mortality after cancer. • During the first SARS-CoV-2 lockdown in 2020, a sharp decrease in the total number of Emergency Departments visits for all causes was observed, both in children and in adults. What is New: • During the first SARS-CoV-2 lockdown in 2020, domestic accidents involving children increased threefold from the previous year. • Higher risk of hospitalization was showed in minors accessing during 2020 vs 2019, in males than in females and it increased with advancing age. Considering the type of injury, a significant higher risk of hospitalization for poisoning was observed.

Reliability of different estimated glomerular filtration rate as measures of renal function in children with sickle cell disease.

BACKGROUND: There is no reliable marker for detecting early renal disease in early children with sickle cell disease (SCD). Estimation of glomerular filtration rate (eGFR) as derived from the height/plasma creatinine formula is dependent on the accuracy of the creatinine analytical method used. The aim of this study was to evaluate different equations for eGFR. METHODS: Children aged 5-16 years recruited. mGFR was obtained using plasma disappearance of Inutest/Iohexol, serum creatinine (SCr) was measured either by standard laboratory method or by tandem mass spectrometry (MSMS). Estimated GFR was then calculated either by "Bedside Schwartz method" or by the full-age spectrum (FAS) equation. FINDINGS: A total of 79 patients (mean age 9.8 ± 4.0 years). A revised eGFR constant was calculated for Schwartz equation from the slope of the plot of height/plasma creatinine versus mGFR. Mean values for mGFR (132.7 ± 32.1 ml/min/1.73m2) and eGFR methods compared: eGFR from standard SCr was significantly higher (144.2 ± 37.3 ml/min/1.73m2, p = 0.008). The MSMS eGFR showed the lowest SD (SD = 27.5), while both FAS eGFR and FAS-height eGFR showed the highest correlation coefficient (r = 0.67). INTERPRETATION: eGFR calculation based on height and SCr determined with MSMS traceable creatinine is more reliable than Schwartz formula using jaffe/enzymatic methods in SCD children.

The impact of COVID-19 on a tertiary care pediatric emergency department.

The restrictive measures required to face the recent outbreak of the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) may impact patterns of healthcare utilization. Our aim was to provide an insight into the change in the use of a pediatric emergency department (ED) during the SARS-CoV-2 pandemic. The medical records of the children seen in our pediatric ED during March and April 2020 were retrospectively reviewed. Consequently, these were compared to the medical records of 2018 and 2019 from the same time period and from other control periods (January-February 2019 and 2020, and July-August 2018 and 2019). The total number of ED visits declined by 73% from 2019 to 2020 (3051 vs 818). Significant variations were observed in the distribution of children between triage categories: the proportion of patients who was given a green-code showed a 0.59-fold decrease in comparison to 2019 (95% CI 0.5-0.69), while a relative increase in the proportion of yellow codes was observed (OR 1.46, 95% CI 1.2-1.78).Conclusion: Quarantine measures significantly impacted on the total number of patients and on the reasons for visiting them in our pediatric ED. This substantial decrease in pediatric care may either be due to lower rates of acute infections because of social distancing, or to parents' or caregivers' reticence to risk exposure to SARS-CoV-2 in a health-care setting. What is known: • A recent outbreak of a novel coronavirus responsible for a severe acute respiratory syndrome is spreading globally. • Restrictive measures may impact patterns of healthcare utilization, as observed in other previous outbreaks. What is new: • This study shows significant variations in the distribution of children among triage categories during the COVID-19 pandemic. • Discharge diagnosis was significantly different as well, in particular a relative increase in the proportion of children presenting with traumatic injuries and a decrease of viral infections were observed.

Characteristics and risk factors for SARS-CoV-2 in children tested in the early phase of the pandemic: a cross-sectional study, Italy, 23 February to 24 May 2020.

BackgroundVery few studies describe factors associated with COVID-19 diagnosis in children.AimWe here describe characteristics and risk factors for COVID-19 diagnosis in children tested in 20 paediatric centres across Italy.MethodsWe included cases aged 0-18 years tested between 23 February and 24 May 2020. Our primary analysis focused on children tested because of symptoms/signs suggestive of COVID-19.ResultsAmong 2,494 children tested, 2,148 (86.1%) had symptoms suggestive of COVID-19. Clinical presentation of confirmed COVID-19 cases included besides fever (82.4%) and respiratory signs or symptoms (60.4%) also gastrointestinal (18.2%), neurological (18.9%), cutaneous (3.8%) and other unspecific influenza-like presentations (17.8%). In multivariate analysis, factors significantly associated with SARS-CoV-2 positivity were: exposure history (adjusted odds ratio (AOR): 39.83; 95% confidence interval (CI): 17.52-90.55; p < 0.0001), cardiac disease (AOR: 3.10; 95% CI: 1.19-5.02; p < 0.0001), fever (AOR: 3.05%; 95% CI: 1.67-5.58; p = 0.0003) and anosmia/ageusia (AOR: 4.08; 95% CI: 1.69-9.84; p = 0.002). Among 190 (7.6%) children positive for SARS-CoV-2, only four (2.1%) required respiratory support and two (1.1%) were admitted to intensive care; all recovered.ConclusionRecommendations for SARS-CoV-2 testing in children should consider the evidence of broader clinical features. Exposure history, fever and anosmia/ageusia are strong risk factors in children for positive SARS-CoV-2 testing, while other symptoms did not help discriminate positive from negative individuals. This study confirms that COVID-19 was a mild disease in the general paediatric population in Italy. Further studies are needed to understand risk, clinical spectrum and outcomes of COVID-19 in children with pre-existing conditions.

Risk Factors of Persistent Hydrocephalus in Children with Brain Tumor: A Retrospective Analysis.

OBJECT: Hydrocephalus is one of the main complications of brain tumors in children, being present in about 50% of cases at the time of the tumor diagnosis and persisting up to 10-40% of cases after surgical resection. This is a single-institution retrospective study on the variables that may predict the need for treatment of persistent hydrocephalus in pediatric patients presenting with a brain tumor. METHODS: Retrospective case note review of 43 newly diagnosed brain tumors in children referred between April 2012 and January 2018 to our regional pediatric neuro-oncology service was carried out. Diagnosis of hydrocephalus was carried out using both preoperative and postoperative MRI to determine Evans' index (EI) and the fronto-occipital horn ratio (FOHR) from each scan. Simple logistic regression was used to analyze categorical variables as appropriate. A p value <0.05 was considered significant. RESULTS: Forty-three children were analyzed, 26 males and 17 females with a median age at diagnosis 10.4 years (IQR: 5.2-13.5). Hydrocephalus was present in 22/43 children (51%) preoperatively; in 8/22 children (36%) with hydrocephalus undergoing tumor resection, hydrocephalus persisted also in the postoperative period. An EI >0.34 (p = 0.028) and an FOHR >0.46 (p = 0.05) before surgery were associated with a higher prevalence of persistent hydrocephalus and therefore to the need for a cerebrospinal fluid drain device in the postoperative phase. CONCLUSION: Preoperative identification of children at risk for developing persistent hydrocephalus would avoid delays in planning the permanent cerebrospinal fluid drain devices. This study finds that an EI >0.34 and an FOHR >0.46 at diagnosis could impact on the therapeutic management of children with hydrocephalus associated with brain tumors. Prospective and larger-scale studies are needed to standardize this approach.

Possible association between the integrity of cerebellar pathways and neurocognitive performance in children with posterior fossa tumors.

BACKGROUND: Cerebellar tumor survivors often exhibit neuropsychological deficits that could be related to alterations in cerebro-cerebellar networks. This is a pilot study designed to understand if diffusion tensor imaging (DTI)-based tractography is able to identify possible correlations between cerebellar white matter structure and cognitive outcome in children on long-term follow-up for posterior fossa (PF) tumors who were thoroughly assessed for neuropsychological functioning. METHODS: DTI-based tractography was performed in pediatric patients with PF tumors. Fractional anisotropy (FA) and volumetric measurements of spinocerebellar, dentorubrothalamocortical and corticopontocerebellar tracts were analyzed. Cognitive and neuropsychological functioning was assessed by the Wechsler Intelligence Scale for Children-IV Edition (WISC-IV) and the Developmental Neuropsychological Assessment (NEPSY II). The associations between Full-Scale Intelligence Quotient (FSIQ), NEPSY-II scores, and fiber tracts were tested by the Spearman rank correlation coefficient. RESULTS: Seven patients (median age at diagnosis five years, range, 3-13) treated for medulloblastoma (2/7; 29%) and pilocytic astrocytoma (5/7; 71%) were retrospectively evaluated. All children had complete surgery. The median FSIQ was 84 (range, 67-93). Patients presented with several deficits on many NEPSY-II tasks; in particular, memory was impaired in nearly half of them. FSIQ and neurocognitive tasks significantly correlated with specific corticopontocerebellar tracts. CONCLUSION: Children on follow-up for PF tumor showed scattered cognitive impairments, including deficits in long-term and immediate memory. Tractography allowed us to describe a possible association between the integrity of cerebellar pathways and neurocognitive performance, suggesting that the myelinization of these fibers may represent an indicator for the development of long-term cognitive sequelae.

SARS-COV-2 infection in children and newborns: a systematic review.

A recent outbreak of a novel Coronavirus responsible for a Severe Acute Respiratory Syndrome (SARS-CoV-2) is spreading globally. The aim of this study was to systematically review main clinical characteristics and outcomes of SARS-CoV-2 infections in pediatric age. An electronic search was conducted in PubMed database. Papers published between 1 January and 1 May 2020 including children aged 0-18 years were selected. Sixty-two studies and three reviews were included, with a total sample size of 7480 children (2428/4660 males, 52.1%; weighted mean age 7.6 years). Patients showed mainly mild (608/1432, 42.5%) and moderate (567/1432, 39.6%) signs of the infection. About 2% of children were admitted to the pediatric intensive care unit. The most commonly described symptoms were fever (51.6%) and cough (47.3%). Laboratory findings were often unremarkable. Children underwent a chest CT scan in 73.9% of all cases, and 32.7% resulted normal. Overall, the estimated mortality was 0.08%. A higher proportion of newborns was severely ill (12%) and dyspnea was the most common reported sign (40%).Conclusion: SARS-CoV-2 affects children less severely than adults. Laboratory and radiology findings are mainly nonspecific. Larger epidemiological and clinical cohort studies are needed to better understand possible implications of COVID-19 infection in children.What is Known:• A novel Coronavirus has been recently identified as responsible for a new Severe Acute Respiratory Syndrome (SARS-CoV-2) spreading globally.• There is limited evidence on SARS-CoV2 infection in children.What is New:• Systematically reviewed available evidence showed that children with SARS-CoV-2 infection may have a less severe pattern of disease in comparison to adults.• Blood tests and radiology findings are mainly nonspecific in children but may help to identify those who are severely ill.

Universal Recommendations for the Management of Acute Diarrhea in Nonmalnourished Children.

OBJECTIVE: Despite a substantial consistency in recommendations for the management of children with acute gastroenteritis (AGE), a high variability in clinical practice and a high rate of inappropriate medical interventions persist in both developing and developed countries.The aim of this study was to develop a set of clinical recommendations for the management of nonseverely malnourished children with AGE to be applied worldwide. METHODS: The Federation of International Societies of Pediatric Gastroenterology, Hepatology, and Nutrition (FISPGHAN) Working Group (WG) selected care protocols on the management of acute diarrhea in infants and children aged between 1 month and 18 years. The WG used a 3-step approach consisting of: systematic review and comparison of published guidelines, agreement on draft recommendations using Delphi methodology, and external peer-review and validation of recommendations. RESULTS: A core of recommendations including definition, diagnosis, nutritional management, and active treatment of AGE was developed with an overall agreement of 91% (range 80%-96%). A total of 28 world experts in pediatric gastroenterology and emergency medicine successively validated the set of 23 recommendations with an agreement of 87% (range 83%-95%). Recommendations on the use of antidiarrheal drugs and antiemetics received the lowest level of agreement and need to be tailored at local level. Oral rehydration and probiotics were the only treatments recommended. CONCLUSIONS: Universal recommendations to assist health care practitioners in managing children with AGE may improve practitioners' compliance with guidelines, reduce inappropriate interventions, and significantly impact clinical outcome and health care-associated costs.

Comparison of Recommendations in Clinical Practice Guidelines for Acute Gastroenteritis in Children.

OBJECTIVE: Acute gastroenteritis (AGE) is a major cause of child mortality and morbidity. This study aimed at systematically reviewing clinical practice guidelines (CPGs) on AGE to compare recommendations and provide the basis for developing single universal guidelines. METHODS: CPGs were identified by searching MEDLINE, Cochrane-Library, National Guideline Clearinghouse and Web sites of relevant societies/organizations producing and/or endorsing CPGs. RESULTS: The definition of AGE varies among the 15 CPGs identified. The parameters most frequently recommended to assess dehydration are skin turgor and sunken eyes (11/15, 73.3%), general appearance (11/15, 66.6%), capillary refill time, and mucous membranes appearance (9/15, 60%). Oral rehydration solution is universally recognized as first-line treatment. The majority of CPGs recommend hypo-osmolar (Na 45-60 mmol/L, 11/15, 66.6 %) or low-osmolality (Na 75 mmol/L, 9/15, 60%) solutions. In children who fail oral rehydration, most CPGs suggest intravenous rehydration (66.6%). However, nasogastric tube insertion for fluid administration is preferred according by 5/15 CPGs (33.3%). Changes in diet and withdrawal of food are discouraged by all CPGs, and early refeeding is strongly recommended in 13 of 15 (86.7%). Zinc is recommended as an adjunct to ORS by 10 of 15 (66.6%) CPGs, most of them from low-income countries. Probiotics are considered by 9 of 15 (60%) CPGs, 5 from high-income countries. Antiemetics are not recommended in 9 of 15 (60%) CPGs. Routine use of antibiotics is discouraged. CONCLUSIONS: Key recommendations for the management of AGE in children are similar in CPGs. Together with accurate review of evidence-base this may represent a starting point for developing universal recommendations for the management of children with AGE worldwide.

Sustained low influenza vaccination in health care workers after H1N1 pandemic: a cross sectional study in an Italian health care setting for at-risk patients.

BACKGROUND: Despite consistent recommendations by all Public Health Authorities in support of annual influenza vaccination for at-risk categories, there is still a low uptake of influenza vaccine in these groups including health care workers (HCWs). Aim of this observational two-phase study was to estimate the immunization rates for influenza in four subsequent seasons and for pandemic H1N1 influenza in HCWs of a University Hospital, and to investigate its distribution pattern and the main determinants of immunization. Phase 1 data collection was performed in 2009-2010, during the peak of H1N1 pandemic. Phase 2 data collection, aimed to investigate seasonal influenza vaccination coverage in the three seasons after pandemic, was performed in 2012-2013. METHODS: The overall H1N1 vaccination rate was derived by the Hospital immunization registry. In 2010, the personnel of three Departments (Infectious Diseases, Pediatrics and Gynecology/Obstetrics) completed a survey on influenza. A second-phase analysis was performed in 2012 to investigate influenza vaccination coverage in three consecutive seasons. RESULTS: The first-phase survey showed a low coverage for influenza in all categories (17 %), with the lowest rate in nurses (8.1 %). A total of 37 % of health care workers received H1N1 vaccine, with the highest rate among physicians and the lowest in nurses. H1N1 vaccination was closely related to the Department, being higher in the Department of Infectious Diseases (53.7 %) and Pediatrics (42.4 %) than in Gynecology/Obstetrics (8.3 %). The second-phase survey showed the lowest rate of influenza vaccination in 2012/13 season. The main reasons for not being vaccinated were "Unsure of the efficacy of vaccine" and "Feel not at-risk of getting influenza or its complications". Despite recommendations, influenza vaccine uptake remains poor. CONCLUSION: Immunization is largely perceived as a personal protection rather than a measure needed to prevent disease spreading to at-risk patients. Compulsory vaccination against influenza should be considered as a possible strategy, at least in health institutions where at-risk patients are admitted.

Detection and assessment of postoperative pain in children with cognitive impairment: A systematic literature review and meta-analysis.

BACKGROUND AND OBJECTIVE: Children with cognitive impairment (CI) are at risk of experiencing pain. Several specific pain rating scales have been developed to date. Thus, the aim of this meta-analysis was to estimate the degree of reliability of different pain assessment scales for the postoperative pain in children with CI. DATABASES AND DATA TREATMENT: PubMed, Scopus and Web of Science databases were approached: all studies validating and/or using pain assessment tool in children (0-20 years) with CI published in English from the 1st of January 2000 to the 1st of January 2022 were included. Only studies reporting the interclass correlation coefficient (ICC) to evaluate the concordance between caregivers' and external researchers' scores were eligible. RESULTS: Twelve studies were included (586 children with CI, 60% males; weighted mean age 9.9 years - range 2-20). Five of them evaluated the Non-Communicating Children's Pain Checklist-Postoperative Version (NCCPC-PV) scale whereas four the original and revised Face, Legs, Activity, Cry, Consolability (FLACC) scale. The analysis showed an overall ICC value of 0.76 (0.74-0.78) for the NCCPC-PV scale, with a high heterogeneity index (I2  = 97%) and 0.87 (0.84-0.90) for the FLACC scale, with a discrete I2 index (59%). CONCLUSIONS: The NCCPC-PV and FLACC pain rating scales showed the strongest evidence for validity and reliability for assessing postoperative pain in children with CI. However, due to the high heterogeneity of the studies available, these results should not be considered conclusive. SIGNIFICANCE: This review is focused on the assessment of pain in children with CI in the postoperative period. Simplified observation-based pain assessment tools that rely on evaluating non-verbal expressions of pain should be recommended for children with difficulties to communicate their feelings. Even if there is a high degree of heterogeneity in clinical presentations among youth with CI, two tools (NCCPC-PV and FLACC) have emerged as reliable and valid in this population.

Lung Clearance Index May Detect Early Peripheral Lung Disease in Sickle Cell Anemia.

Rationale: Chronic lung injury is common in sickle cell anemia (SCA) and worsens outcomes. Sensitive lung function tests might predict reversible disease that might benefit from therapeutic interventions. Objectives: To evaluate whether lung clearance index (LCI) (measuring global ventilation inhomogeneity), intraacinar ventilation inhomogeneity (Sacin), and conductive ventilation inhomogeneity (Scond) are more frequently abnormal than lung volumes in young people with SCA. Methods: Nitrogen multiple-breath washout, spirometry, and body plethysmography were cross-sectionally evaluated at steady state in subjects with SCA (hemoglobin SS) and healthy control subjects aged 8-21 years from London, United Kingdom. Results: Thirty-five patients (51% boys, mean ± SD age, 16.4 ± 3.5 yr) and 31 control subjects (48% boys; 16.2 ± 3.2 yr) were tested. There were significant differences between the study and control groups in mean LCI (mean difference, 0.42 units; 95% confidence interval [CI], 0.22 to 0.63; P = 0.0001), Sacin (mean difference, 0.014 units; 95% CI, 0.001 to 0.026; P = 0.04), forced expiratory volume in 1 second (FEV1) (mean difference, -0.79 z-scores; 95% CI, -1.28 to -0.30; P = 0.002), forced vital capacity (FVC) (mean difference, -0.80 z-scores; 95% CI, -1.28 to -0.31, P = 0.002), and total lung capacity (mean difference, -0.76 z-scores; 95% CI, -1.25 to -0.29, P = 0.002), but not in Scond and FEV1-to-FVC ratio. Whereas 29% (10 of 35) of patients had LCI > 95th percentile of control subjects, 23% (8 of 35) had abnormal FEV1 (<5th percentile of the reference population). Conclusions: LCI detected slightly more abnormalities than lung volumes in young people with SCA. Significant differences from control subjects in LCI and Sacin but not in Scond and FEV1-to-FVC ratio suggest that the lung function changes were most likely owing to patchy peripheral lung disease.

The burden of sleep disordered breathing in children with sickle cell disease.

Children with sickle cell disease (SCD) have an increased risk of sleep disordered breathing (SDB) compared with the general pediatric population. There has been a growing research interest on this field in recent years, yet many questions regarding risk factors and clinical implications of SDB remain unclear. The aim of this review is to provide a concise narrative and systematic synthesis of the available evidence on the epidemiology, clinical presentation, complications, and management, of SDB in children with SCD. An electronic search was conducted on studies published from the 1st of January 2000 to the 31st of December 2020 in PubMed/Medline, Scopus, and Cochrane databases. All studies focusing on SDB in children with SCD aged from 0 to 20 years were included. Studies were eligible for inclusion if available in the English language. A quantitative synthesis of the included studies was performed. Only studies focusing on specific treatment outcomes were included in a meta-analytic process. A total of 190 papers were initially identified. After screening the title and abstract, 112 articles were evaluated for eligibility. At the end of the selection process, 62 studies were included in the analysis. Sleep disordered breathing is associated with worse neurological, neurocognitive, and cardiological outcomes, whereas the association with frequency or severity of vaso-occlusive pain events and acute chest syndrome was not clarified. Therapeutic interventions like adenotonsillectomy or oxygen supplementation may result in a significant increase in mean nocturnal oxygen saturation but effective clinical implications remain still unclear.

Long-term oxygen therapy in children with sickle cell disease and hypoxaemia.

OBJECTIVE: To evaluate the acceptability and safety profile of nocturnal long-term oxygen therapy (LTOT) in children with sickle cell disease (SCD) and chronic hypoxaemia. DESIGN: Retrospective cohort study. PATIENTS, SETTING AND INTERVENTION: Children with SCD who started LTOT from 2014 to early 2019 in two tertiary hospitals in London, UK were retrospectively enrolled. Patients who started disease-modifying therapies <12 months before LTOT or while on LTOT were excluded. MAIN OUTCOME MEASURES: Minor and major adverse events during LTOT were reported. Laboratory and clinical data, transcranial Doppler (TCD) scans and overnight oximetry studies performed at steady state within 12 months before and after starting LTOT were compared. RESULTS: Nineteen children (10 males; median age 12 years, range 6-15) were included. Nearly half of them (9/19; 47%) were on hydroxyurea at baseline. No child discontinued LTOT because of intolerance or poor adherence. No major adverse events were reported. Laboratory data did not show significant changes in haemoglobin and reticulocyte count after 1 year of follow-up. No statistically significant change in the incidence of vaso-occlusive pain events was noted (median annual rate from 0.5 to 0 episode per patient/year; p=0.062). Overnight oximetry tests performed while on LTOT showed improvements in all oxygen saturation parameters (mean overnight and nadir SpO2, % of time spent with SpO2 <90%) compared with the baseline. CONCLUSION: LTOT is a safe and feasible treatment option for children with SCD and chronic hypoxaemia.

A multimodal approach to the study of children treated for posterior fossa tumor: A review of the literature and a pilot study.

OBJECTIVE: The aims of the present study were: (1) to review the literature on long-lasting cognitive sequelae in children treated for Posterior Fossa Tumor and (2) to investigate anatomic functional relations in a case series of 7 children treated for PFT using magnetic resonance imaging (MRI) post-processing methods. METHODS: We retrospectively analyzed MRIs of children who underwent complete surgical resection of PFT and performed extensive neuropsychological evaluation. Tumor, ventricular volumes, and VPS insertion site were drawn on T1 volumetric MRI scans and normalized to a pediatric template. Children showed worse performances on tasks tapping executive functions, memory, visuo-motor precision, and expressive language. RESULTS: Volumes of interest related to these functions showed a maximum overlap on the left vermis and the lateral ventricle enlargement, except for impaired narrative fluency -which was associated with left lateral ventricle enlargement- and narrative memory -which was related to the right vermis and the enlarged fourth ventricle. CONCLUSION: Results suggest that anatomic functional relations in children treated for PFT are related to a combination of different pathophysiological factors.

Beneficial effects of adenotonsillectomy in children with sickle cell disease.

Tonsillectomy and adenoidectomy (T&A) is frequently performed in children with sickle cell disease (SCD). Our aim was to evaluate the impact of this surgery on overnight oxygenation and rates of complications in these patients. Children with SCD who underwent T&A between 2008 and 2014 in two tertiary hospitals were retrospectively evaluated. Overnight oximetry and admission rates due to vaso-occlusive pain episodes (VOEs) and acute chest syndrome (ACS) in the year preceding and following the surgery were compared. 19 patients (10 males, 53%) with a median age of 6 years (range 3.5-8) were included. A significant increase of mean overnight arterial oxygen saturation measured by pulse oximetry (S pO2 ) (from 93±3.6% to 95.3±2.8%, p=0.001), nadir S pO2 (from 83.0±7.1% to 88±4.1%, p=0.004) and a reduction of 3% oxygen desaturation index (from a median value of 5.7 to 1.8, p=0.003) were shown. The mean annual rate of ACS decreased from 0.6±1.22 to 0.1±0.2 events per patient-year (p=0.003), while the mean cumulative rate of hospitalisations for all causes and the incidence of VOEs were not affected. T&A improved nocturnal oxygenation and was also associated with a reduction in the incidence of ACS at 1-year follow-up after surgery.

Nutrition and Lung Growth.

Experimental evidence from animal models and epidemiology studies has demonstrated that nutrition affects lung development and may have a lifelong impact on respiratory health. Chronic restriction of nutrients and/or oxygen during pregnancy causes structural changes in the airways and parenchyma that may result in abnormal lung function, which is tracked throughout life. Inadequate nutritional management in very premature infants hampers lung growth and may be a contributing factor in the pathogenesis of bronchopulmonary dysplasia. Recent evidence seems to indicate that infant and childhood malnutrition does not determine lung function impairment even in the presence of reduced lung size due to delayed body growth. This review will focus on the effects of malnutrition occurring at critical time periods such as pregnancy, early life, and childhood, on lung growth and long-term lung function.