Citations

Background and objectives: Goodpasture’s syndrome (GS) is a rare, life-threatening autoimmune disease. Although the coexistence of anti-neutrophil cytoplasmic antibody (ANCA) with Goodpasture’s syndrome has been recognized, the impacts of ANCA vasculitis on mortality and resource utilization among patients with GS are unclear. Materials and Methods: We used the National Inpatient Sample to identify hospitalized patients with a principal diagnosis of GS from 2003 to 2014 in the database. The predictor of interest was the presence of ANCA-associated vasculitis. We tested the differences concerning in-hospital treatment and outcomes between GS patients with and without ANCA-associated vasculitis using logistic regression analysis with adjustment for other clinical characteristics. Results: A total of 964 patients were primarily admitted to hospital for GS. Of these, 84 (8.7%) had a concurrent diagnosis of ANCA-associated vasculitis. Hemoptysis was more prevalent in GS patients with ANCA-associated vasculitis. During hospitalization, GS patients with ANCA-associated required non-significantly more mechanical ventilation and non-invasive ventilation support, but non-significantly less renal replacement therapy and plasmapheresis than those with GS alone. There was no significant difference in in-hospital outcomes, including organ failure and mortality, between GS patients with and without ANCA-associated vasculitis. Conclusions: Our study demonstrated no significant differences between resource utilization and in-hospital mortality among hospitalized patients with coexistence of ANCA vasculitis and GS, compared to those with GS alone.

AbstractBackgroundWhile acute kidney injury (AKI) is commonly reported following hematopoietic stem cell transplant (HCT), the incidence and impact of AKI on mortality among patients undergoing HCT are not well described. We conducted this systematic review to assess the incidence and impact of AKI on mortality risk among patients undergoing HCT.MethodsOvid MEDLINE, EMBASE and the Cochrane Databases were searched from database inceptions through August 2019 to identify studies assessing the incidence of AKI and mortality risk among adult patients who developed AKI following HCT. Random-effects and generic inverse variance method of DerSimonian–Laird were used to combine the effect estimates obtained from individual studies.ResultsWe included 36 cohort studies with a total of 5144 patients undergoing HCT. Overall, the pooled estimated incidence of AKI and severe AKI (AKI Stage III) were 55.1% (95% confidence interval (CI) 46.6–63.3%) and 8.3% (95% CI 6.0–11.4%), respectively. The pooled estimated incidence of AKI using contemporary AKI definitions (RIFLE, AKIN and KDIGO criteria) was 49.8% (95% CI 41.6–58.1%). There was no significant correlation between study year and the incidence of AKI (P = 0.12) or severe AKI (P = 0.97). The pooled odds ratios of 3-month mortality and 3-year mortality among patients undergoing HCT with AKI were 3.05 (95% CI 2.07–4.49) and 2.23 (95% CI 1.06–4.73), respectively.ConclusionThe incidence of AKI among patients who undergo HCT remains high, and it has not changed over the years despite advances in medicine. AKI after HCT is associated with increased short- and long-term mortality.

Abstract Human cytomegalovirus (HCMV) infections are among the most common complications arising in transplant patients, elevating the risk of various complications including loss of graft and death. HCMV infections are also responsible for more congenital infections worldwide than any other agent. Congenital HCMV (cCMV) infections are the leading nongenetic cause of sensorineural hearing loss and a source of significant neurological disabilities in children. While there is overlap in the clinical and laboratory approaches to diagnosis of HCMV infections in these settings, the management, follow-up, treatment, and diagnostic strategies differ considerably. As yet, no country has implemented a universal screening program for cCMV. Here, we summarize the issues, limitations, and application of diagnostic strategies for transplant recipients and congenital infection, including examples of screening programs for congenital HCMV that have been implemented at several centers in Japan, Italy, and the United States.

AbstractPulmonary embolism (PE) affects over 300,000 individuals each year in the United States and is associated with substantial morbidity and mortality. Improvements in the diagnostic performance and availability of computed tomographic pulmonary angiography and D-dimer testing have facilitated the evaluation of patients with suspected PE. High clinical suspicion is required in those with risk factors and/or those that manifest signs or symptoms of venous thromboembolic disease, with validated clinical risk scores such as the Wells and modified Wells score or the PE rule-out criteria helpful in estimating the likelihood for PE. For those with confirmed PE, patients should be categorized and triaged according to the presence or absence of shock or hypotension. Normotensive patients can be further risk-stratified using validated prognostic risk scores, as well as by using imaging and cardiac biomarkers, with those having either signs of right ventricular dysfunction on imaging studies and/or abnormal cardiac biomarkers categorized as being at intermediate-risk and requiring close monitoring and hospital admission. Early discharge and/or home therapy are possible in those that do not manifest any high-risk features. The initial treatment for most patients that are stable consists of anticoagulation, with advanced therapies such as thrombolysis, catheter-based therapies, or surgical embolectomy deferred for those at high risk. Given the heterogeneous presentations of PE and various management strategies available, the development of multidisciplinary PE response teams has emerged to help facilitate decision-making in these patients.

Obesity in children is associated with several co-morbidities including dyslipidemia. Obstructive sleep apnea (OSA) is commonly seen in obese children. In adults, diagnosis of OSA independent of obesity is associated with cardiometabolic risk factors including dyslipidemia. There is limited data on the impact of treatment of OSA on lipids in children. The objective of the study was to examine the impact of treatment of OSA on lipids in 24 obese children. Methods: Seventeen children were treated with continuous positive airway pressure (CPAP) and five underwent adenotonsillectomy. Mean apnea hypopnea index prior to treatment was 13.0 + 12.1 and mean body mass index (BMI) was 38.0 + 10.6 kg/m2. Results: Treatment of OSA was associated with improvement in total cholesterol (mean change = −11 mg/dL, p < 0.001), and low-density lipoprotein cholesterol (mean change = –8.8 mg/dL, p = 0.021). Conclusion: Obese children should be routinely screened for OSA, as treatment of OSA favorably influences lipids and therefore decreases their cardiovascular risk.

The term disappearing HDL syndrome refers to development of severe high density lipoprotein cholesterol (HDL-C) deficiency in noncritically ill patients with previously normal HDL-C and triglyceride levels. Autoimmune lymphoproliferative syndrome (ALPS) is a disorder of the immune system due to an inability to regulate lymphocyte homeostasis resulting in lymphadenopathy and hepatosplenomegaly. We describe a 17-year-old boy who was evaluated in the lipid clinic for history of undetectable or low HDL-C and low density lipoprotein cholesterol (LDL-C) levels. Past medical history was significant for ALPS IA diagnosed at 10 years of age when he presented with bilateral cervical adenopathy. He was known to have a missense mutation in one allele of the FAS protein extracellular domain consistent with ALPS type 1A. HDL-C and LDL-C levels had been undetectable on multiple occasions, though lipids had not been measured prior to the diagnosis of ALPS. He had been receiving sirolimus for immunosuppression. The HDL-C and LDL-C levels correlated with disease activity and improved to normal levels during times when the activity of ALPS was controlled. This case highlights the importance of considering ALPS as a cause of low HDL-C and LDL-C levels in a child with evidence of lymphoproliferation.

AbstractBackgroundChildhood obesity is underdiagnosed in primary care practices. Our study aimed to compare rates of documentation of diagnosis of obesity and counselling for nutrition and physical activity at an academic primary care practice prior to and following implementation of a body mass index (BMI)‐based electronic point of care clinical reminder tool.MethodsWe performed a retrospective record review of children aged 2–18 years undergoing well child visits during any of three calendar years (2009, 2011 and 2013). The electronic clinical reminder tool was implemented in September 2010. Records of children with BMI > 95th percentile were electronically searched for terms of documentation of diagnosis of obesity and nutrition and physical activity counselling. Multivariable logistic regression models were used to adjust for potential patient and provider confounders.ResultsAmong those with BMI ≥ 95th percentile, there was a statistically significant increase in documentation of diagnosis of obesity (45.5% in 2009 vs. 52.4% in 2011; P < 0.001; adjusted odds ratio 1.36; 95% CI 1.13–1.65) and in counselling for physical activity and nutrition (66.8% in 2009 vs. 75.2% in 2011; P < 0.001; adjusted odds ratio 1.33; 95% CI 1.06–1.66) following implementation of the tool. Compared with 2011, there was no statistically significant increase in documentation of obesity (50.4%) or nutrition and physical activity counselling (77.9%) in 2013.ConclusionsThe implementation of a point of care clinical reminder tool was associated with improvement in documentation of diagnosis of obesity and counselling for nutrition and physical activity. Further studies are needed to determine the impact of these automated tools on weight outcomes in children.