LECT-2 amyloidosis: what do we know?

Amyloidosis is a rare group of diseases characterized by abnormal folding of proteins and extracellular deposition of insoluble fibrils. It can be localized to one organ system or can have systemic involvement. The kidney is the most common organ to be involved in systemic amyloidosis often leading to renal failure and the nephrotic syndrome. The two most common types of renal amyloidosis are immunoglobulin light chain-derived amyloidosis (AL) and reactive amyloidosis (AA). A novel form of amyloidosis (ALECT2) derived from leukocyte chemotactic factor 2 (LECT-2) and primarily involving the kidneys was first described by Benson et al in 2008. The liver was subsequently identified as the second most common organ involved in ALECT2 amyloidosis. LECT-2 is a unique protein that can form amyloid deposits even in its unmutated form. Patients with ALECT2 present with minimal proteinuria in contrast to other forms of amyloidosis especially AL and AA. They may present with slightly elevated serum creatinine. Nephrotic syndrome and hematuria are rare. ALECT2 can be found in association with other types of amyloidosis as well as malignancies or autoimmune diseases. ALECT2 may be confused with amyloidosis associated with light and heavy chain monoclonal gammopathy if the immunofluorescence is positive with anti-light chain and anti-AA sera. The other organs involved are the duodenum, adrenal gland, spleen, prostate, gall bladder, pancreas, small bowel, parathyroid gland, heart, and pulmonary alveolar septa, but consistently uninvolved organs included brain and fibroadipose tissue. A renal biopsy along with characteristic features found on immunohistochemistry and mass spectrometry is diagnostic of ALECT2. ALECT2 should be suspected when all markers for AL and AA are negative. Proper diagnosis of ALECT2 can determine need for supportive care versus more aggressive interventions.

Vaccination in Chronic Kidney Disease.

Infections after cardiovascular disease are the second most common cause of death in the chronic kidney disease population. Vaccination is an important component of maintaining health and wellness in patients with kidney disease. There is a changing epidemiologic landscape for several vaccine-preventable illnesses from childhood to adulthood and unfounded public perception of safety concerns. Several mechanisms have been proposed to cause inadequate vaccine protection in this high-risk group with chronic kidney disease. These have led to recent advances in new designs for vaccination strategies in kidney disease. In this article, we discuss the current evidence and recommendations for vaccination in those with kidney disease and needing renal replacement therapy (dialysis and transplant).

Diuretics in the Management of Cardiorenal Syndrome.

The leading cause of death worldwide is cardiovascular disease. The heart and the kidneys are functionally interdependent, such that dysfunction in one organ may cause dysfunction in the other. By one estimate, more than 60% of patients with congestive heart failure develop chronic kidney disease. Volume overload and congestion are hallmarks of heart failure, and these findings are associated with severe symptoms and poor outcomes. Given the importance of congestion, diuretics remain a cornerstone of heart failure management. However, diuretic treatment remains largely empirical, with little evidence currently available to guide decisions. In this review, we discuss the pathophysiology of cardiorenal syndrome, the pharmacology of loop diuretics, mechanisms of diuretic resistance, and evidence-based treatment paradigms.

Diabetes mellitus treatment-Related medical knowledge among health care providers.

OBJECTIVE: To compare the knowledge of physicians, residents and medical students in diagnosis, use of insulin and oral medication in management of Type 2 Diabetes Mellitus (DM) working in different healthcare specialties. METHODS: A cross sectional survey of faculty, residents and medical students of different subspecialties in a single center was conducted. Questionnaire consisting of 20 questions was used. These questions were designed to assess knowledge about diagnosis, nomenclature of different insulin/oral medications and management of DM. There were 4 answers to every question with only one correct answer based on ADA guidelines and most recent literature. RESULTS: The overall percentage correctly answered questions was ∼74% for IM faculty, 64% for EM faculty, 71% for IM residents, 60% for FM residents, 56% for EM residents and 59% for students. Questions based on knowledge of insulin nomenclature and characteristics were answered correctly 74% of the time by IM faculty, 62% by EM faculty, 66% by IM residents, 69% by FM residents, 45% by EM residents and 49% by medical students. Questions on the use of insulin and inpatient DM management were answered correctly 66% for IM faculty, 54% for EM faculty, 66% for IM residents, 46% for FM residents, 55% for EM Residents, and 44% medical students. Questions based on oral medications and DM diagnosis were answered correctly by 81% for IM faculty, 73% for EM faculty, 78% for IM Resident, 76% FM Resident, 64% for EM residents and 79% for students. CONCLUSION: This study demonstrates the need for focused educational initiatives required in all subspecialties involved in management of diabetes mellitus for safe and efficient management of diabetes mellitus.

Infective Endocarditis in Hemodialysis Patients.

BACKGROUND AND AIM OF THE STUDY: Infective endocarditis (IE) is a major cause of morbidity and mortality in patients with end-stage renal disease (ESRD). The study aim was to evaluate the demographic, clinical, microbiological and inpatient mortality data of IE in hemodialysis patients. METHODS: Data were analyzed for all IE patients admitted to the authors' 800-bed tertiary care hospital between January 2001 and December 2014. These data included demographics, comorbidities, clinical and microbiological characteristics, echocardiographic findings, complications, outcomes, and in-patient mortality. RESULTS: A total of 296 patients, including 52 on hemodialysis, was admitted with a diagnosis of IE. The median age of patients with ESRD (28 females, 24 males) was 55.9 ± 15.47 years. The prevalences of comorbidities such as hypertension (80%) and diabetes mellitus (46%) were significantly higher in ESRD patients, whereas other comorbidities were similarly distributed in both groups. The mitral valve was the most commonly involved (55.8%), followed by aortic (21.7%), tricuspid (21.2%) and pulmonary (1.9%) valves. Staphylococcus aureus was the most common organism (40%), followed by Enterococcus sp. (13.7%), Gram-negative staphylococci (13.7%), coagulase-negative staphylococci (11.5%), and Streptococcus sp. (5.7%). Polymicrobes were found in 11.5% of patients and cultures were negative in 19%. The mean ejection fraction in these patients was 42 ± 4.19% and the mean area of vegetation was 63.5 ± 40 mm2. The in-hospital course of 11 patients was complicated by embolic events, while three patients had acute heart failure and one patient had heart block secondary to IE. A total of four patients (7.7%) died during the index hospitalization. CONCLUSIONS: IE in patients receiving chronic hemodialysis is a very frequent occurrence. Its diagnosis is complex and its presence should be considered in all hemodialysis patients with bacteremia. In the present study the etiology was shown to be multifactorial, with the mitral valve being the most commonly involved and S. aureus the most common organism.

Pneumococcal Vertebral Osteomyelitis after Epidural Injection: A Rare Event.

Streptococcus pneumoniae vertebral infections have rarely been reported. Herein, we report a case of pneumococcal vertebral osteomyelitis with paraspinal and epidural abscesses as well as concomitant bacteremia following epidural injection. This will be the second case in the literature reporting pneumococcal vertebral osteomyelitis related to epidural manipulation.