Extreme hyperferritinaemia greater than 3000 μg/L is uncommon in adult patients. The highest serum ferritin values are seen in HLH, but the differential diagnosis for serum ferritin greater than 3000 μg/L remains broad with iron overload and liver disease being the most common causes.

Background The epidemiology of acute kidney injury (AKI) in children in sub-Sahara Africa (SSA) is poorly described. The aim of this study was to establish the incidence, etiology, and outcomes of community-acquired AKI in pediatric admissions in Southern Malawi. Methods We conducted a prospective observational study of pediatric admissions to a tertiary hospital in Blantyre between 5 February and 30 April 2016. Children were screened for kidney disease on admission with measurement of serum creatinine and assessment of urine output. The clinical presentation, etiology, and management of children with AKI were documented. Results A total of 412 patients (median age 4 years, 52.6% male, and 7.5% human immunodeficiency virus [HIV] infected) were included in the study. Forty-five patients (10.9%) had AKI (Kidney Disease: Improving Global Outcomes [KDIGO] criteria), which was stage 3 in 16 (35.6%) patients. Sepsis and hypoperfusion, most commonly due to malaria ( n = 19; 42.2%), were the causes of AKI in 38 cases (84.4%). Three patients (6.7%) underwent peritoneal dialysis (PD) for AKI: 2 of them recovered kidney function, and the other one died. In-hospital mortality was 20.5% in AKI and 2.9% if no kidney disease was present ( p < 0.0001). Seventeen (47.2%) patients with kidney disease had persistent kidney injury on hospital discharge. Conclusion Acute kidney injury occurs in 10.9% of pediatric admissions in Malawi and is primarily due to infections, particularly malaria. Acute kidney injury results in significantly increased in-hospital mortality. Urgent interventions are required to eliminate preventable causes of death in this region.

Purpose of the reviewImmunoglobulin G4-related disease (IgG4-RD) is a systemic fibroinflammatory disorder affecting nearly all organs, including the kidney. Tubulointerstitial nephritis (IgG4-TIN) is the most common form of IgG4-related kidney disease (IgG4-RKD) and is the focus of this concise review.ObjectiveThe study aims to describe when IgG4-TIN should be suspected and to summarize the diagnosis, treatment, and natural history of the disease.Sources of informationOvid MEDLINE, Google Scholar, and PubMed were searched for full-text English language articles up to January 2016. References included in the manuscript were chosen at the authors’ discretion based on their relevance to the subject of the review.FindingsIgG4-TIN should be considered in patients presenting with abnormal urinalysis, abnormal kidney function, renal lesions on imaging, and elevated IgG, IgE, or hypocomplementemia. Diagnosis of IgG4-TIN requires a combination of histologic features (plasma cell-enriched TIN with >10 IgG4+ plasma cells/hpf, +/− TBM immune complex deposits in many cases) and at least one of the following:Characteristic radiologic findings (small peripheral low-attenuation cortical nodules, round or wedge-shaped lesions, or diffuse patchy involvement)Elevated serum IgG4 levelCharacteristic findings of IgG4-RD in other organsOther conditions such as lupus, vasculitis, diabetic nephropathy, and lymphoma must be excluded, as these can also present with IgG4+ plasma cells in the renal parenchyma. IgG4-TIN is generally responsive to steroids and B cell depletion with rituximab, but relapses are common and patients require close long-term follow-up.LimitationsAvailable data on IgG4-TIN are from retrospective observational studies.ImplicationsIgG4-TIN is a distinct and emerging subtype of interstitial nephritis. Nephrologists must be aware of this entity and how to definitively diagnose and treat it. Prospective studies and ideally multi-center clinical trials are needed to study the epidemiology, treatment, and natural history of this disease.

Key Clinical MessageLymphangiomas are benign tumors of the lymphatic vessels, which can be inflammatory and occasionally steroid‐responsive. IgG4‐related disease (IgG4‐RD) is a recently defined fibro‐inflammatory condition. We describe a novel association between reactive IgG4+ plasma cells and cystic lymphangioma in a young woman who had a dramatic clinical response to steroids.

Background: In Ontario, kidney transplants have risen by 4% annually in recent years. An understanding of how this will affect the future annual number of posttransplant follow-up visits informs how to organize and deliver care. Objective: We projected the required number of annual posttransplant follow-up nephrology visits to inform posttransplant care delivery. Design: Population-based retrospective cohort study. Setting: Linked databases from Ontario, Canada (population 14 million). Patients: Incident kidney transplant recipients from years 2008 to 2013. Measurements: Frequency, distance traveled, and current and projected visits for posttransplant follow-up. Methods: Assuming a graft survival of 13 years and using the mean number of posttransplant clinic visits in years 1, 2, and 3, we forecasted the number of clinic visits needed in the year 2027. Results: Using data from 2443 recipients, the mean (SD) number of clinic visits per recipient was 14.0 (9.2) in the first year after transplant, and 3.9 (6.2) and 3.0 (5.3) in the second and third year, respectively. If transplant rates rise by 4% per year until 2027, the estimated annual visits number will increase from 30 622 to 43 948. The median (25th, 75th percentile) distance between transplant center and patient’s home was 30 (13, 65) km. The median round-trip travel distance for these visits in the first year after transplantation was 603 km per recipient, and median driving cost was Can$344 (2017). Limitations: Regarding patient expense, limitations include that distances traveled were calculated orthodromically, and we did not account for patient cost of follow-up beyond that of vehicular travel. Regarding follow-up projections, limitations include the assumption that graft life span will not change, follow-up patterns do not differ between donor kidney type, and we did not survey stakeholders as to their preferred method of follow-up. Conclusion: We quantified the increase in posttransplant visits when regional annual rates of transplantation rise. Strategies recognizing the burden of these visits may enhance patient-centered care, as it is unclear how some patients manage costs, nor how the current health care system will manage the demand.