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摘要： Direct microscopy exam in calcofluor white stain of a skin biopsy and periodic acid Schiff (PAS) stain of the paraffin block revealed septate hyphae. Pulmonary and, less frequently, skin infection presentations have been previously published [1]. [Extracted from the article]

关键词： Pulmonary hypertension   children   young adults   heart disease   Association for European Paediatric and Congenital Cardiology  

摘要： Pulmonary hypertension is a complex and progressive condition that is either idiopathic or heritable, or associated with one or multiple health conditions, with or without congenital or acquired cardiovascular disease. Recent developments have tremendously increased the armamentarium of diagnostic and therapeutic approaches in children and young adults with pulmonary hypertension that is still associated with a high morbidity and mortality. These modalities include non-invasive imaging, pharmacotherapy, interventional and surgical procedures, and supportive measures. The optimal, tailored diagnostic and therapeutic strategies for pulmonary hypertension in the young are rapidly evolving but still face enormous challenges: Healthcare providers need to take the patient's age, development, disease state, and family concerns into account when initiating advanced diagnostics and treatment. Therefore, there is a need for guidance on core and advanced medical training in paediatric pulmonary hypertension. The Association for European Paediatric and Congenital Cardiology working group "pulmonary hypertension, heart failure and transplantation" has produced this document as an expert consensus statement;however, all recommendations must be considered and applied in the context of the local and national infrastructure and legal regulations.

关键词： corticosteroids   Crohn disease   inflammatory bowel disease   paediatric gastroenterology   ulcerative colitis  

摘要： Objectives:The aim of the study was to evaluate the use of steroids within the paediatric inflammatory bowel disease (PIBD) population at a tertiary paediatric centre over a year;to identify cases of steroid dependency;and assess factors associated with steroid ***:The prevalent PIBD population (May 1, 2017-April 30, 2018) were reviewed. Data were collected retrospectively from patient records and entered into an online steroid assessment tool (modified for paediatrics).Results:A total of 229 patients (181 Crohn disease, 31 ulcerative colitis [UC], and 17 inflammatory bowel disease-unclassified) were included. Of the 229 patients 38 (16.6%) received oral steroids;12 of 38 (31.6%) receiving >3-month course. Eleven of 38 (28.9%) received >1 steroid course (maximum 2). Of the 229 patients 37 (16.2%) had exclusive enteral nutrition, with 26 of 37 (11.4% total cohort) avoiding steroid use during the study *** disease activity had a negative correlation with steroid use (11/127 [8.7%] vs 27/102 [26.5%] P<0.01), and steroid dependency (3/127 [2.4%] vs 12/102 [11.8%] P<0.01). Patients with UC were more likely to be steroid dependent (5/31 [16.1%] UC vs 10/198 [5.1%];P=0.02);as were network-managed patients (8/11 [72.7%] vs 7/27 [25.9%];P=0.01). Fourteen of 15 (93.3%) of steroid-dependent patients had active steroid sparing strategies in place (eg, commencement, switching, or optimization of therapies).Conclusions:We have described rates of steroid use and dependency within our PIBD population. Exclusive enteral nutrition served as a steroid sparing tool in 11.4% of the total cohort. Replication of this study in other paediatric centres would allow comparative analysis.

关键词： pyeloplasty   supranormal function   MAG3   pelvic diameter   hydronephrosis  

摘要： Objectives To study related factors and clinical significance of supranormal function in paediatric patients with pelvi-ureteric junction obstruction, and to predict which factors cause renal function overestimation. Patients and Methods Patients who underwent pyeloplasty from 2012 to 2017 were prospectively collected. Variables were compared between patients with and without supranormal function on Tc-99m-mercaptoacetyltriglycine renal scan (supranormal defined as differential renal function [DRF] >= 55%). Univariate, multivariate logistic and linear regressions analyses were performed. Results Of 100 patients, 18 were excluded because of comorbidities. Nine patients (11.5%) showed preoperative supranormal function. The preoperative anteroposterior pelvic diameter (APD;24 mm vs 35 mm, P = 0.026) and the ratio between preoperative pelvic and kidney volumes (0.2 vs 0.6, P = 0.003) were higher in supranormal kidneys. For each unit increase in the preoperative ratio between pelvic and kidney volumes, the risk of supranormal function rose 3.23-times (95% confidence interval [CI] 1.051-9.955). A preoperative APD >= 30 mm was a reliable predictor of supranormal function (area under the curve 0.804, 95% CI 0.707-0.902), with 88.9% sensitivity. Patients with either preoperative supranormal function or preoperative APD >= 30 mm had a greater reduction in renal function after pyeloplasty. Conclusion Supranormal function is related to large hydronephrosis where geometrical features are modified. A preoperative APD >= 30 mm is a reliable predictive factor of supranormal function. Preoperative renal function is overestimated either in supranormal patients or severe hydronephrotic kidneys. DRF should be interpreted with caution in kidneys with large hydronephrosis with or without supranormal function. Surgical indication should not entirely rely upon DRF.

关键词： blood bank   cigarette   hemotherapy   metals   tobacco  

摘要： Background and Objectives Smokers currently have no defined restrictions for blood donation. However, cigarette smoke contains toxic substances such as carbon monoxide (CO) and trace elements that can affect the packed red blood cells (PRBCs) quality and safety of transfusion. This study evaluated the effects of smoking on the concentration of essential and trace elements and on carboxyhemoglobin (COHb) levels in PRBCs from smoker donors. Materials and Methods A matched case-control study was conducted to compare COHb levels, determined by the CO-oximetry method, and levels of trace (Cd, Pb, Cr, Ni, As and Hg) and essential (Ca, Mg, Cu, Fe, Mn, Mo, Se and Zn) elements evaluated by inductively coupled plasma mass spectrometry, in PRBCs from smoker (n = 36) and non-smoker (n = 36) donors at Hospital de Clinicas de Porto Alegre, Brazil. Results Mean COHb level was 14 times higher in the PRBCs obtained from smoker donors (5 center dot 9 [4 center dot 0-9 center dot 1] vs. 0 center dot 4 [0 center dot 2-0 center dot 8]%). Cadmium (1 center dot 0 [1 center dot 0-1 center dot 8] mu g/l vs. undetectable) and lead (27 [21-36] vs. 19 [14-26] mu g/l) levels were significantly higher in the PRBCs from smokers. Moreover, except for molybdenum, levels of all essential elements were lower in smoker PRBCs. Conclusion The PRBCs donated by smokers contain toxic elements that are probably not safe for transfusion in children. Our results might support changes in the current guidelines of blood banks to improve the transfusion safety through inclusion of inquiry about smoking in the clinical screening, labelling and reserve PRBCs from smoker donors for adults or less critical recipients.

关键词： antifungal agents   deep fungal infection   epidemiology   lymphadenitis   Paracoccidioides brasiliensis   paracoccidioidomycosis  

摘要： The acute-subacute form of paracoccidioidomycosis (PCM) is a severe systemic mycosis that affects children and adolescents from endemic regions, leading to generalised lymphadenopathy, fever, weight loss, anaemia, eosinophilia, hypoalbuminemia and hypergammaglobulinemia. The objective of this study is to describe the clinical and laboratorial characteristics of acute-subacute PCM, to determine a mortality risk factor and to propose a test for non-survival hazard related to the disease. Children and adolescents diagnosed with PCM, under 15 years were included in the study. Their epidemiological, clinical and laboratorial data were obtained from the hospital records. Descriptive analysis, comparison of means, univariate logistic regression, multivariate logistic regression and a ROC curve were performed in order to identify significant information (P < .05). Through a period of 38 years, 141 children and adolescents were diagnosed with acute-subacute PCM. The main antifungal agent used for the treatment was sulfamethoxazole-trimethoprim (SMX-TMP). The complication rate was 17%, the relapse rate was 7.8% and the mortality rate was 5.7%. A low albumin dosage was identified as a predictor factor for mortality. The cut-off for serum albumin was 2.18 g/dL, above which, the survival rate is 99.1%. Thus, simple clinical and laboratorial examinations may lead to the diagnosis of acute-subacute PCM, and the beginning of the treatment is encouraged even before the isolation of the fungus in biological samples, preventing unfavourable outcomes. Patients with an albumin dosage <= 2.18g/dL must receive special attention, preferably hospitalised, during the first four weeks of treatment for presenting an elevated mortality hazard.

摘要： Background The long-term effects of anti-TNF alpha therapy in ulcerative colitis are debatable. Aim To examine whether anti-TNF alpha therapy changed the colectomy proportion and reduced the use of corticosteroids. Methods A nationwide cohort study among patients (age 0-20) diagnosed with ulcerative colitis through 1995-2015 from Danish health registries. We calculated the cumulative 5-year risk of colectomy after diagnosis and used a Cox regression model for comparison between a historical pre-anti-TNF alpha cohort 1 (1995-2003) and a cohort 2 for the era of anti-TNF alpha (2004-2015). Based on anti-TNF alpha users, defined as patients who had at least four anti-TNF alpha treatments within 4 months, we examined the subsequent need for corticosteroids. Results We identified 3001 patients from 1995 to 2015. The 5-year cumulative proportion of colectomy in cohort 2 was 9.7% (95% confidence interval [CI] 8.4-11.1) and 12.3% (95% CI 10.4-14.6) in cohort 1. The adjusted 5-year hazard ratio (HR) was 0.76 (95% CI 0.60-0.96) for colectomy in cohort 2 compared to cohort 1. A total of 334 patients received anti-TNF alpha treatments, and 16.8% (56/334) were prescribed corticosteroids in the subsequent 3-month period. Corticosteroid treatment declined with follow-up after 6 and 12 months, 5.4% and 1.2%, respectively. Conclusion In patient's <= 20 years, the HR for colectomy within a period of 5 years from the time of diagnosis was reduced in the era of anti-TNF alpha compared to a historical cohort. In patients treated with anti-TNF alpha, prescriptions of corticosteroids were virtually ceased after 12 months.

关键词： assessment   asylum seeker   detention   paediatric   refugee health  

摘要： AimAsylum seekers (ASs) report high rates of trauma and difficulty accessing health and educational services. This study aims to ascertain the needs of paediatric ASs managed by the tertiary Western Australian paediatric Refugee Health Service (RHS), including demographic features, the range of health and psychosocial issues and ongoing management challenges. MethodsAn audit of multidisciplinary RHS assessments, health records and hospital admissions for new AS patients (<16years) between July 2012 and June 2016 was undertaken. ResultsRecords for 110 ASs were reviewed (mean age 6 years, standard deviation 4.72years). Multiple issues (medical, psychological, developmental, educational) were identified after the first tertiary assessment (median 4, interquartile range (IQR) 3-6) compared to referral sources (median 1, IQR 0-2, P<0.001). The median number of issues per child at audit completion was 6 (IQR 4-7, P<0.001). Multiple refugee adverse childhood experiences were identified, with all experiencing >3 (median 4, IQR 4-5). Most had detention experience (107/110, 97.2%), family separation (91/108, 84%) and interrupted education (41/46, 89.2%). The median duration of detention was 7 months (IQR 3-12.5 months) at time of initial review across multiple sites (median 2, IQR 1-3 locations). High rates of hospital interaction were evident, with 45.4% requiring hospital admission and 36% presenting to the emergency department. The median number of outpatient appointments attended per child was 5 (IQR 2-8). Parental and child mental health concerns were identified in 53.6 and 46.3%, respectively. ConclusionsPaediatric ASs have complex trauma backgrounds with exposure to multiple adverse events within disrupted family units. The majority of Western Australian ASs assessed demonstrated negative health or education sequelae compounded by detention not previously identified prior to comprehensive paediatric review. Our data support the urgent removal of ASs from held detention