ANCA-associated kidney disease preceded by orbital pseudotumor.

INTRODUCTION: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and IgG4-related disease (IgG4-RD) are distinct immune disorders with overlapping clinical and laboratory features. While ANCA positivity excludes IgG4-RD in the 2019 ACR/EULAR classification, this criterion is not uniformly applied, and AAV can form inflammatory masses in various organs and show increase in IgG4 + plasma cells, similar to IgG4-RD. CASE DIAGNOSIS/TREATMENT: A 5-year-old female with history of orbital mass diagnosed as IgG4-RD presents with acute kidney injury. She has a myeloperoxidase ANCA, and kidney biopsy shows pauci-immune crescentic glomerulonephritis and acute tubulointerstitial nephritis with increased IgG4 + plasma cells and tubular basement membrane (TBM) deposits. CONCLUSION: In isolation, TBM deposits and increased IgG4 + plasma cells are suggestive of IgG4-RD. In the context of a positive ANCA and pauci-immune crescentic glomerulonephritis, however, increased IgG4 + plasma cells due to AAV are favored. In cases with features of IgG4-RD, ANCA positivity suggests an alternate diagnosis of AAV to be more likely.

Risk factors for severe acute kidney injury after pediatric hematopoietic cell transplantation.

BACKGROUND: Acute kidney injury (AKI) is common after hematopoietic cell transplantation (HCT) and is associated with poorer outcomes. Risk factors for AKI after pediatric HCT are not fully understood. The study objective was to assess unique risk factors for AKI in the HCT population and evaluate post-HCT AKI patterns. METHODS: We conducted a retrospective cohort study of patients < 21 years of age who underwent HCT at Seattle Children's Hospital/Fred Hutchinson Cancer Center from September 2008 to July 2017 (n = 484). We defined AKI using KDIGO criteria. We collected demographics, baseline HCT characteristics, post-HCT complications, and mortality. Multinomial logistic regression was used to estimate association between AKI and potential risk factors. We used adjusted Cox proportional hazard ratios to evaluate differences in mortality. RESULTS: One hundred and eighty-six patients (38%) developed AKI. Seventy-nine (42%) had severe AKI and 27 (15%) required kidney replacement therapy. Fluid overload was common in all groups and 67% of those with severe AKI had > 10% fluid overload. Nephrology was consulted in less than 50% of those with severe AKI. In multivariable analysis, risk of severe AKI was lower in those taking a calcineurin inhibitor (CNI). Risk of death was higher in severe AKI compared to no AKI (RR 4.6, 95% CI 2.6-8.1). CONCLUSIONS: AKI and fluid overload are common in pediatric patients after HCT. Severe AKI occurred less often with CNI use and was associated with higher mortality. Future interventions to reduce AKI and its associated complications such as fluid overload are approaches to reducing morbidity and mortality after HCT. A higher resolution version of the Graphical abstract is available as Supplementary information.

Pathology findings in pediatric patients with COVID-19 and kidney dysfunction.

BACKGROUND: Acute kidney injury (AKI) is seen in one-fifth of pediatric patients with COVID-19 requiring hospital admission, and is associated with increased morbidity, mortality, and residual kidney impairment. The majority of kidney pathology data in patients with COVID-19 is derived from adult case series and there is an overall lack of histologic data for most pediatric patients with COVID-19. METHODS: We assembled a multi-institutional cohort of five unvaccinated pediatric patients with COVID-19 and associated kidney dysfunction with available histology. RESULTS: Three complex patients with current or prior SARS-CoV-2 infection had multifactorial thrombotic microangiopathy with clinical features of hemolytic uremic syndrome (in two) or disseminated intravascular coagulation (in one); one died and another developed chronic kidney disease stage 5. Two with recently preceding SARS-CoV-2 infection presented with nephrotic syndrome; one had IgA vasculitis and one had minimal change disease. Within a short follow-up time, none has returned to baseline kidney function. CONCLUSION: Although uncommon, COVID-19-associated kidney injury can have significant morbidity in the unvaccinated pediatric and adolescent population. A higher resolution version of the Graphical abstract is available as Supplementary information.

Dysfunctional voiding: update on evaluation and treatment.

PURPOSE OF REVIEW: This review will be covering dysfunctional voiding, its diagnosis, and treatment options. This will focus primarily on dysfunctional voiding rather than all lower urinary tract dysfunction and we will focus on some of the newer findings and progress within this disease. RECENT FINDINGS: Dysfunctional voiding is the inappropriate sphincter and pelvic floor constriction during voiding in an otherwise neurologically normal child. This has a wide spectrum of symptoms and can lead to a number of complications such as chronic kidney disease and poor quality of life if not appropriately addressed. Dysfunctional voiding is diagnosed with a careful examination and history with further imaging including a renal ultrasound and uroflowmetry to confirm the diagnosis. Urotherapy and biofeedback are the first and second-line treatments respectively and lead to significant improvement or cure in the majority of patients. For refractory patients, additional therapy options include use of α-blockers, botulinum injection, and electroneurostimulation, though the majority of the literature surrounding the use of these therapies consists of small studies with heterogenous causes of voiding dysfunction. SUMMARY: Dysfunctional voiding is a common urologic complaint that has many excellent options for improving the patient's voiding issues and should be considered in patients with voiding dysfunction.

Caregiver burden in pediatric dialysis: application of the Paediatric Renal Caregiver Burden Scale.

BACKGROUND: Caring for a child with kidney failure on dialysis profoundly impacts caregivers' lives, yet the depth of this burden is not well understood. The Paediatric Renal Caregiver Burden Scale (PR-CBS) is a recently validated instrument used to measure caregiver burden in this population. METHODS: We performed a cross-sectional study of caregiver burden for caregivers of children with kidney failure receiving dialysis at three pediatric centers. Caregivers completed the PR-CBS instrument as part of a larger qualitative study of caregiver experience. We performed descriptive statistics. T-tests were used to examine differences between dialysis modality and within key demographics. Multivariate linear regression was utilized to assess associations between significant factors and total score. RESULTS: Ten caregivers of children receiving peritoneal dialysis (PD) and 21 receiving hemodialysis (HD) participated. Total burden score and mean score for every domain was higher for caregivers of children on HD compared to PD. PR-CBS score was significantly associated with younger child age and married status in caregivers. In adjusted multivariate analysis, dialysis modality and married marital status were significantly associated with PR-CBS score. CONCLUSIONS: This study found that dialysis caregivers experience significant caregiver burden and demonstrates the utility of the PR-CBS in an American population. We found higher burdens among HD caregivers, younger children, and married caregivers. While these findings must be replicated on a larger scale, they suggest possible areas for targeted interventions to improve the quality of life of children with kidney failure and their families. A higher resolution version of the Graphical abstract is available as Supplementary information.

Pediatric Minor Traumatic Brain Injury With Intracranial Hemorrhage: Identifying Low-Risk Patients Who May Not Benefit From ICU Admission.

BACKGROUND: Pediatric patients with any severity of traumatic intracranial hemorrhage (tICH) are often admitted to intensive care units (ICUs) for early detection of secondary injury. We hypothesize that there is a subset of these patients with mild injury and tICH for whom ICU care is unnecessary. OBJECTIVES: To quantify tICH frequency and describe disposition and to identify patients at low risk of inpatient critical care intervention (CCI). METHODS: We retrospectively reviewed patients aged 0 to 17 years with tICH at a single level I trauma center from 2008 to 2013. The CCI included mechanical ventilation, invasive monitoring, blood product transfusion, hyperosmolar therapy, and neurosurgery. Binary recursive partitioning analysis led to a clinical decision instrument classifying patients as low risk for CCI. RESULTS: Of 296 tICH admissions without prior CCI in the field or emergency department, 29 had an inpatient CCI. The decision instrument classified patients as low risk for CCI when patients had absence of the following: midline shift, depressed skull fracture, unwitnessed/unknown mechanism, and other nonextremity injuries. This clinical decision instrument produced a high likelihood of excluding patients with CCI (sensitivity, 96.6%; 95% confidence interval, 82.2%-99.9%) from the low-risk group, with a negative likelihood ratio of 0.056 (95% confidence interval, -0.053-0.166). The decision instrument misclassified 1 patient with CCI into the low-risk group, but would have impacted disposition of 164 pediatric ICU admissions through 5 years (55% of the sample). CONCLUSIONS: A subset of low-risk patients may not require ICU admission. The proposed decision rule identified low-risk children with tICH who may be observable outside an ICU, although this rule requires external validation before implementation.