No abstract

Background: Volume overload in patients on hemodialysis (HD) is an independent risk factor for cardiovascular mortality. B-lines detected on lung ultrasound (BLUS) assess extravascular lung water. This raises interest in its utility for assessing volume status and cardiovascular outcomes. Methods: End-stage renal disease patients on HD at the Island Rehab Center being older than 18 years were screened. Patients achieving their dry weight (DW) had a lung ultrasound in a supine position. Scores were classified as mild (0-14), moderate (15-30), and severe (>30) for pulmonary congestion. Patients with more than 60 were further classified as very severe. Patients were followed for cardiac events and death. Results: 81 patients were recruited. 58 were males, with a mean age of 59.7 years. 44 had New York Heart Association (NYHA) class 1, 24 had class 2, and 13 had class 3. In univariate analysis, NYHA class was associated with B-line classes (<0.001) and diastolic dysfunction (0.002). In multivariate analysis, NYHA grade strongly correlated with B-line classification (0.01) but not with heart function (0.95). 71 subjects were followed for a mean duration of 1.19 years. 9 patients died and 20 had an incident cardiac event. A Kaplan-Meier survival analysis demonstrated an interval decrease in survival times in all-cause mortality and cardiac events with increased BLUS scores (p = 0.0049). Multivariate Cox regression analysis showed the independent predictive value of BLUS class for mortality and cardiac events with a heart rate of 2.98 and 7.98 in severe and very severe classes, respectively, compared to patients in the mild class (p = 0.025 and 0.013). Conclusion: At DW, BLUS is an independent risk factor for death and cardiovascular events in patients on HD.

Immunoglobulin D (IgD) multiple myeloma (MM) is a very rare form of myeloma affecting less than 2% of all myeloma patients. It has a multiorgan involvement with renal failure being the key feature. We present here a case of IgD MM in a 62-year-old white male, smoker with past medical history of hypertension, who presented to emergency department with complaints of lower abdominal pain, constipation and decreased urination. Physical exam was unremarkable. Laboratory investigation showed S.Cr 5.99 mg/dL, hemoglobin 8.7 g/dL and corrected S.Ca 10.6 mg/dL. Urine dipstick showed 100 protein and TP/Cr ratio was 23. Serology was positive for serum free lambda chain level of 8,947.6 mg/L as well with free κ/λ ratio < 0.01. The results of serum and urine electrophoresis and immunofixation were also supportive of diagnosis of IgD MM. IgD level was remarkably elevated (27,300 mg/L) too. CT scan of abdomen/pelvis was negative for obstructive uropathy. Skeletal survey showed a solitary lytic lesion in the iliac crest. His kidney function deteriorated next day requiring hemodialysis. The bone marrow biopsy was positive for plasma cell hypercellularity (70-80%) and flow cytometry showed 8% monoclonal IgD lambda plasma cells. The patient was started on bortezomib and dexamethasone and he underwent bone marrow transplant 6 months later. He is doing well hematologically now but he remains dialysis-dependent. IgD MM is a very rare disease affecting younger population with poor prognosis; patients often end up on hemodialysis despite better control of the hematological component.

Background. Monoclonal immunoglobulin (MIg)-associated renal disease (MIgARD) comprises a group of disorders caused by direct deposition of paraproteins in the kidney. Allograft MIgARD is infrequently encountered and poorly characterized. Methods. First, we assessed our allograft biopsies diagnosed with MIgARD between 2007 and 2018. The cohort included the following 26 patients: proliferative glomerulonephritis with MIg deposits (PGNMID) (n = 13), AL amyloidosis (n = 5), light chain deposition disease (n = 5), light chain proximal tubulopathy (n = 2), and light chain cast nephropathy (n = 1). Second, we conducted a literature review to evaluate the rare non-PGNMID entities. We identified 20 studies describing 29 patients that were added to our cohort (total n = 42). Results. Part 1: Patients’ median age was 55 years; 31% were women, and 19% were blacks. Twelve patients (46%) lost their grafts at a median of 8 months after diagnosis. Compared to non-PGNMID, PGNMID patients had lower frequency of detectable paraproteins (31% versus 92%, P = 0.004) and hematologic neoplasms (23% versus 77%, P = 0.02). Within PGNMID group, 6 patients changed their apparent immunofluorescence phenotype between monotypic and polytypic, while all 3 patients with hematologic neoplasms had substructure on electron microscopy. Part 2: Whereas light chain cast nephropathy occurred the earliest and had the worst graft survival, AL amyloidosis occurred the latest and had the best graft survival. Conclusions. MIgARD in the kidney allograft is associated with poor prognosis. While posttransplant PGNMID can change its apparent clonality by immunofluorescence supporting oligoclonal immune responses, the presence of deposit substructure is an important indicator of underlying hematologic neoplasm. Non-PGNMID are often associated with hematologic neoplasms and varied prognosis.

or autopsy). Studies were excluded if they examined only specific comorbidities (eg, cancer, liver disease, etc); were on pediatric or pregnant patients; used healthy volunteers as controls; did not have a calculable sensitivity (Sn) and specificity (Sp) from the data presented; or were reviews, commentaries, or editorials. All articles were screened for inclusion by two independent reviewers, with 97% agreement; k ¼ 0.77, P < .001. Both reviewers decided a priori to err on the side of inclusion, and if either reviewer selected an article, it was ordered for full text review. A single reviewer then determined if the full text articles met the inclusion criteria, and any questions were discussed with the team to reach a final decision on inclusion. Sn and Sp were combined using equal weighting methods and calculated using Microsoft Excel. Results: Our search strategy yielded 4,472 articles without duplicates. Of these, 389 were ordered for full text review, and 22 were included in the final analysis. The most commonly cited use of echo to detect PE was through a combination of findings suggestive of PE. These findings were termed and defined variably across 16 studies. Terms for combined measures included: right ventricular (RV) dysfunction, RV strain, and acute cor pulmonale. These combined measures had a Sn of 57% and a Sp of 78%, and those only in point of care studies had a Sn of 60% and an Sp of 87%. The most common (n¼7) stand-alone signs used were an increased RV:LV ratio (Sn¼64%, Sp ¼85%), abnormal septal motion (Sn¼29%, Sp¼ 96%), and tricuspid insufficiency (Sn¼49%, Sp¼80%). The most specific test was visualizing a RV thrombus, with a Sp of 100% in 2 studies. However, 3 other markers showed a Sp greater than 95%: RV hypokinesis (98%, n¼4), McConnell's sign (98%, n¼3), and abnormal septal motion (96%, n¼7). The most sensitive test was an increased RV end diastolic diameter, with a Sn of 78% in 3 studies. The test with the highest diagnostic odds ratio (DOR) was RV wall hypokinesis, with a DOR of 34.7, a Sn of 39% and a Sp of 98% in 3 studies. Conclusion: Studies have consistently shown a high specificity for echo in the diagnosis of PE, making it potentially adequate as a rule-in test at the bedside in the emergency department for patients unable to get other confirmatory studies. Future research should examine if combining echo with other modalities, such as lung and deep venous thrombosis ultrasound improves accuracy.