Isolated Renal Involvement by IG4-Related Disorder Mimicking Multiple Myeloma, a Diagnosis Not to Miss.

IG4-related disorder (IgG4-RD) with isolated kidney involvement is rare. IG4-RD is a fibroinflammatory disorder leading to polyclonal activation of plasma cell and can affect kidney, orbital tissues, salivary glands, pancreas, bile duct, lymph nodes, and can cause inflammatory mass in any organ. Isolated kidney involvement is rare in this order. We share a case of isolated kidney involvement by this order presenting as enlarged kidneys with renal impairment. Kidney biopsy showed CD138 plasma cell interstitial nephritis. The biopsy also showed kappa light chain along IgG on immunofluorescence and was reported as light chain deposition disease initially. In view of hyperproteinemia and initial renal biopsy finding, workup was done for myeloma. Bone marrow biopsy showed around 20% of plasma cell infiltration. Skeletal survey did not show any lytic lesions and immunofixation did not reveal any paraprotein. Flowcytometry of the bone marrow showed nonclonal plasma cell. In view of negative workup for myeloma and nonclonal cells, re-evaluation of the kidney biopsy was done. Biopsy was reanalyzed for both IgG and IgG4. It showed 30 IgG4 cells per high-power field with a ratio of IgG4 / IgG of 40%. The staining for IgM, IgA C3, and C1q was negative. The patient was labeled as having plasma cell interstitial nephritis due toIgG4-RD. The patient responded well to oral prednisolone. It is important not to miss this potentially treatable and reversible condition by staining the biopsy sample for both IgG and IgG4 in clinically suspected cases.

An Alternate In-Plane Technique of Ultrasound-Guided Internal Jugular Vein Cannulation.

BACKGROUND: Commonly used ultrasound-guided internal jugular vein (IJV) cannulation techniques, short axis out of plane and long axis in-plane, have significantly reduced complications but failed to eliminate them because of technical difficulties. OBJECTIVE: This article describes a new anteroposterior short axis in-plane technique that combines advantage of in-plane technique to track the needle tip and short axis view of visualizing nearby anatomical structures by placing the probe on the side of the neck, oriented anteroposteriorly, perpendicular to the long axis of neck. This view visualizes IJV and its relationship to the carotid artery in short axis. The puncture needle is passed in-plane anteroposteriorly from the anterior aspect of the neck. Visualizing the needle, carotid artery, and IJV in single frame minimizes complications. METHODS: A prospective evaluative clinical trial was conducted in patients who require IJV cannulation for various reasons by performers experienced in ultrasound-guided IJV cannulations. The efficacy of the technique is indicated by 3 primary outcome measures: access time, number of attempts and success rate, and safety by secondary outcome measure, which is the incidence of mechanical complications. RESULTS: Seventy-five patients were enrolled. The average number of attempts was 1.17 (standard deviation 0.44), the access time was 27.12 s (standard deviation 21.47), and the success rate was 100%. This technique had 12% incidence of posterior venous wall punctures and 2.66% misplacements and no other complications. CONCLUSION: Anteroposterior short axis in-plane technique is relatively novel and could be alternatively used safely and effectively in place of existing techniques for IJV cannulation.

Pros and Cons of Aspirin Prophylaxis for Prevention of Cardiovascular Events in Kidney Transplantation and Review of Evidence.

Kidney transplant recipients have traditional and nontraditional risk factors which can lead to coronary artery disease and sudden death with a functional graft loss. Aspirin has been used traditionally for prevention of cardiovascular and cerebrovascular accidents. It has beneficial effects in secondary prevention of cardiovascular events in general population. Its use for primary prophylaxis is still disputed. Bleeding and theoretical risk of nephrotoxicity are the major concerns about its use. The data on aspirin in kidney transplant population is sparse. This review will focus on various pros and cons of aspirin use for prevention of cardiovascular events in kidney transplant recipients and a way forward.

Microscopic polyangiitis complicated by intracerebral hemorrhage and pulmonary hemorrhage in a pediatric patient.

PATIENT: Female, 10 FINAL DIAGNOSIS: Polyangiitis Symptoms: Intracranial hemorrhage • swelling • oliguria MEDICATION: Cyclophosphamide • prednisolone Clinical Procedure: Plasmapheresis Specialty: Paediatric nephrology • nephrology • paediatrics. OBJECTIVE: Rare disease. BACKGROUND: MPO ANCA-associated vasculitis is very rare in children. Renal disease is almost universally present but lung and central nervous system involvement are not commonly reported. CASE REPORT: We present a pediatric case of microscopic polyangiitis with the unusual presentation of pauci-immune glomerulonephritis, intracerebral hemorrhage and pulmonary hemorrhage. The neurological and pulmonary symptoms settled after treatment with cyclophosphamide and plasmapheresis. However, there was no renal recovery and the patient was rendered dialysis-dependent. CONCLUSIONS: We believe that this is the first reported case of pediatric microscopic polyangiitis with central nervous system involvement. The disease may have a subclinical presentation resulting in chronic damage to different organs. Prompt treatment of acute disease with immunosuppression and plasmapheresis can halt disease progression in these patients.

Clinical efficacy of sevelamer hydrochloride in patients with end-stage renal disease: a retrospective study.

INTRODUCTION: Sevelamer hydrochloride (Renagel) is frequently used as a second-line phosphate binder in patients on renal replacement therapy. Many studies have shown that sevelamer can improve vascular calcification, serum uric acid and low-density lipoprotein (LDL) cholesterol levels. The main objectives of this study were to assess the efficacy of sevelamer against calcium-based phosphate binders, as well as its tolerability and side-effect profile. METHODS: This was a retrospective study that included all patients on renal replacement therapy (between 2008 and 2011) who had previously received calcium-based binders for ≥ 6 months and were subsequently switched to sevelamer. Data collected from the patients' medical records included demographics, as well as renal parameters three months prior to sevelamer treatment, and at three and six months post treatment. The study excluded patients on multiple, concomitant phosphate binders or with functioning renal transplants, and those who were noncompliant or had inadequate follow-up blood investigations. RESULTS: A total of 39 patients were included in the study. No major side effects were reported by any of the patients. There were improvements in calcium, phosphate, uric acid and LDL cholesterol levels at three and six months post-sevelamer treatment. CONCLUSION: We found sevelamer to be superior to calcium-based phosphate binders in reducing serum calcium, phosphate, uric acid and LDL cholesterol levels in our patient population with advanced renal bone disease. Sevelamer also appears to be well tolerated with no significant side effects.