Prevalence and risk factors of burden among caregivers of older emergency department patients.

The number of older individuals that live independently at home is rising. These older individuals often rely on caregivers who have a similar age and health status. Therefore, caregivers may experience a high burden. We determined the prevalence and associating factors of burden among caregivers of older patients in the emergency department (ED). A cross-sectional study of primary caregivers of patients aged ≥ 70 years visiting the ED of a Dutch teaching hospital was performed. Structured interviews were conducted with patients and their caregivers. Caregiver burden was measured using the caregiver strain index (CSI). Additionally, data from questionnaires and medical records were extracted to determine potential associating factors. Univariate and multivariate regression analyses were conducted to identify independent determinants for burden. Seventy-eight caregivers (39%) experienced a high burden. Multivariate analysis showed a significant association between high caregiver burden and patients with cognitive impairment or dependency for instrumental activities of daily living (IADL) and more self-reported hours of care per day. Almost 40% of older patients in the ED have a caregiver who experiences a high burden. Formal assessment in the ED may help provide adequate care to the patients and their caregivers.

Long-term follow-up including extensive complement analysis of a pediatric C3 glomerulopathy cohort.

BACKGROUND: C3 glomerulopathy (C3G) is a rare kidney disorder characterized by predominant glomerular depositions of complement C3. C3G can be subdivided into dense deposit disease (DDD) and C3 glomerulonephritis (C3GN). This study describes the long-term follow-up with extensive complement analysis of 29 Dutch children with C3G. METHODS: Twenty-nine C3G patients (19 DDD, 10 C3GN) diagnosed between 1992 and 2014 were included. Clinical and laboratory findings were collected at presentation and during follow-up. Specialized assays were used to detect rare variants in complement genes and measure complement-directed autoantibodies and biomarkers in blood. RESULTS: DDD patients presented with lower estimated glomerular filtration rate (eGFR). C3 nephritic factors (C3NeFs) were detected in 20 patients and remained detectable over time despite immunosuppressive treatment. At presentation, low serum C3 levels were detected in 84% of all patients. During follow-up, in about 50% of patients, all of them C3NeF-positive, C3 levels remained low. Linear mixed model analysis showed that C3GN patients had higher soluble C5b-9 (sC5b-9) and lower properdin levels compared to DDD patients. With a median follow-up of 52 months, an overall benign outcome was observed with only six patients with eGFR below 90 ml/min/1.73 m2 at last follow-up. CONCLUSIONS: We extensively described clinical and laboratory findings including complement features of an exclusively pediatric C3G cohort. Outcome was relatively benign, persistent low C3 correlated with C3NeF presence, and C3GN was associated with higher sC5b-9 and lower properdin levels. Prospective studies are needed to further elucidate the pathogenic mechanisms underlying C3G and guide personalized medicine with complement therapeutics.

The impact of an emergency care access point on pediatric attendances at the emergency department: An observational study.

OBJECTIVE: Crowding is a growing concern in general and pediatric Emergency Departments (EDs). The Emergency Care Access Point (ECAP) - a collaboration between general practitioners and the ED - has been established to reduce the number of self-referrals and non-urgent ED visits. The aim of this study was to determine the impact of an ECAP on pediatric attendances in the ED. METHODS: Retrospective analysis of 3997 pediatric patients who visited the ED of a regional teaching hospital in the Netherlands, one year before and one year after the implementation of an ECAP. Patient characteristics, presented complaints and diagnoses, throughput times, and follow-up between the study groups were compared, both during office hours and after-hours. RESULTS: After ECAP implementation, a 16.3% reduction in pediatric ED visits was observed. ECAP implementation was associated with a decline in self-referrals by 97.2%. Presented complaints, ED diagnoses and acuity were similar pre- and post-ECAP. However, consultations and follow-up were required more frequently. The admission rate during nights increased (49.3% versus 64.0%). Overall admission rates were similar. CONCLUSIONS: The implementation of an ECAP was associated with a reduction of pediatric ED use, including a considerable but expected decline in pediatric self-referrals. Patient acuity pre- and post-ECAP was similar. Our results suggest that this primary care intervention might help reduce the workload in a pediatric ED. Future studies are warranted to further investigate this hypothesis and to evaluate the impact of an ECAP in other healthcare settings. These future efforts need to include patient oriented outcomes.

Compound heterozygous mutations in the C6 gene of a child with recurrent infections.

The complement system plays an important role in both the innate and adaptive immune system. Patients with inherited complement deficiencies have an increased risk of systemic bacterial infections. Deficiencies of the terminal complement pathway are especially associated with invasive meningococcal disease. Here, we report a case of a boy that presented with arthritis and recurrent bacterial and viral infections. Extensive analyses revealed decreased complement activity of both classical and alternative pathway, indicating a deficiency of C3 or one of the factors of the terminal complement pathway. Mutational analysis of the C6 gene identified two compound heterozygous mutations. An unknown missense aberration was found that involves the loss of a cysteine, possibly affecting the 3D structure of the protein. Furthermore, a known splice site variation was identified that results in a 14% shorter protein, due to transcription of amino acids that are normally intronic until a stop codon is reached (exon-intron boundary defect). It is known that the protein with this latter aberration is still functionally active when present with other C6 mutations and therefore, the consequences of the combination of the identified variations have been studied. Quantitative ELISAs showed that at least one allele produced a circulating C6 molecule that can be incorporated in the membrane attack complex, likely the truncated protein. In the present case we observed relapsing bacterial and viral infections, but no meningococcal disease. The reduced complement activity can be explained by the identified genetic variations in C6, as recombinant C6 supplementation corrected complement function in vitro.

Severe infantile Bordetella pertussis pneumonia in monozygotic twins with a congenital C3 deficiency.

UNLABELLED: Bordetella pertussis or whooping cough is a vaccine-preventable disease that still remains a serious infection in neonates and young infants. We describe two young infants, monozygotic twins, with a severe B. pertussis pneumonia of whom one needed extracorporeal membrane oxygenation. Diagnostic work-up of unexplained hematuria and proteinuria during the illness revealed low serum complement component 3 (C3) levels. During follow-up, C3 levels remained low (400-600 mg/L). Extensive analysis of the persistent low C3 levels revealed an unknown heterozygous mutation in the C3 gene in both siblings and their mother. This C3 mutation in combination with the specific virulence mechanisms of B. pertussis probably contributed to the severe disease course in these cases. CONCLUSION: We propose that genetically caused complement disorders should be considered when confronted with severe cases of B. pertussis infection.

Risk factors for renal injury in children with a solitary functioning kidney.

OBJECTIVE: The hyperfiltration hypothesis implies that children with a solitary functioning kidney are at risk to develop hypertension, proteinuria, and chronic kidney disease. We sought to determine the presenting age of renal injury and identify risk factors for children with a solitary functioning kidney. METHODS: We evaluated 407 patients for signs of renal injury, defined as hypertension, proteinuria, an impaired glomerular filtration rate, and/or the use of renoprotective medication. Patients were subdivided on the basis of type of solitary functioning kidney and the presence of ipsilateral congenital anomalies of the kidney and urinary tract (CAKUT). The development of renal injury was analyzed with Kaplan-Meier analysis. Risk factors were identified by using logistic regression models. RESULTS: Renal injury was found in 37% of all children. Development of renal injury increased by presence of ipsilateral CAKUT (odds ratio [OR] 1.66; P = .04) and age (OR 1.09; P < .001). Renal length was inversely associated with the risk to develop renal injury (OR 0.91; P = .04). In all patients, the median time to renal injury was 14.8 years (95% confidence interval 13.7-16.0 years). This was significantly shortened for patients with ipsilateral CAKUT (12.8 years, 95% confidence interval 10.6-15.1 years). CONCLUSIONS: Our study determines independent risk factors for renal injury in children with a solitary functioning kidney. Because many children develop renal injury, we emphasize the need for clinical follow-up in these patients starting at birth.