Right ventricular outflow tract obstruction caused by a right coronary cusp prolapse: An unusual finding

Patients with large sub-pulmonic ventricular septal defect (VSD) present early as a results of their complications. Some present late, due to the restriction of VSD by the right coronary cusp (RCC) due to its prolapse. In this report, we present a rare case of sub-pulmonic VSD in a 33-year-old man who developed a sub-pulmonic stenosis due to the prolapse of the RCC into the right ventricular outflow tract.     

Unexpected mechanism of mitral regurgitation in a patient post ALCAPA repair: Added value of three-dimensional echocardiography

Accurate assessment of etiology of mitral regurgitation (MR) is one of the key steps in the decision-making process and further clinical management of patients with severe MR. Our clinical case illustrates the added value of three-dimensional echocardiography (3DE) in assessment of mitral valve morphology and identification of an unexpected mechanism of MR which was not previously diagnosed using conventional echocardiography. 3DE helped to choose appropriate management strategy in this patient.     

Epstein-Barr virus lymphoproliferation after bone marrow transplantation

We review 15 cases of secondary B-cell lymphoproliferative disorders that occurred among 2,475 patients who received allogeneic bone marrow transplants (BMTs) at the Fred Hutchinson Cancer Research Center (Seattle) between 1969 and 1987. The histopathologic findings in 14 of the 15 patients spanned a wide spectrum of lymphoproliferative lesions. One patient had features characteristic of angioimmunoblastic lymphadenopathy. Epstein-Barr virus (EBV) genomic sequences were identified by Southern blot analysis in each of the 13 patients evaluated. Ten of the 12 lesions evaluated originated in donor cells. In two patients, who had mixed chimerism after transplantation, the lesions originated in host cells. The combined evidence from immunoglobulin light chain staining and the analysis of immunoglobulin heavy chain gene rearrangement indicated that the lesions in most patients represented polyclonal proliferations that gave rise to clonal subpopulations. The results indicate an overall actuarial incidence of 0.6% for this complication in BMT recipients. Anti-CD3 monoclonal antibody (MoAb) treatment of acute graft-v-host disease (GVHD) and T cell depletion of the donor marrow were statistically significant risk factors, and GVHD appeared to play a contributing role, particularly in the setting of human leukocyte antigen (HLA) disparity. Two patients had no identifiable risk factors. Prophylaxis or treatment with acyclovir had no detectable effect in the patients; all but two died with uncontrolled lymphoproliferation.     

Melatonin excretion with affect disorders over age 60

Numerous studies have reported low melatonin secretion in depression, but other studies have suggested no deficit or an increase. Alterations of circadian phase or duration of melatonin secretion have also been described. Since melatonin secretion decreases as we age, it seemed interesting to examine melatonin and depression in an aging sample. Volunteers who complained of mood or sleep problems were recruited for studies in which fractional urine specimens were collected for 24 h, both at home and in the laboratory. The major metabolite, 6-sulfatoxymelatonin (aMT6s), was determined by radioimmunoassay. Of 72 volunteers aged 60-78 years, seven had current major depression and 55% had a lifetime history of an affective disorder. A 55-fold range of home aMT6s excretion rates was observed. A lifetime history of any affective disorder was significantly associated with greater log(10)[mesor] aMT6s excretion in home collections and laboratory collections, but current affective disorders were neither significantly related to melatonin excretion nor to aMT6s acrophase timing, onset, offset or duration. These results are only weakly consistent with a photoperiodic hypothesis of depression.     

Trajectories of impairment in amyotrophic lateral sclerosis: Insights from the Pooled Resource Open‐Access ALS Clinical Trials cohort

Introduction: Rate of decline of the Amyotrophic Lateral Sclerosis Functional Rating Scale‐Revised (ALSFRS‐R) score is a common outcome measure and a powerful predictor of mortality in ALS. Methods: Observed rate of decline (postslope) of ALSFRS‐R, its linearity, and its relationship to decline at first visit (preslope) were examined in the Pooled Resource Open‐Access ALS Clinical Trials cohort by using longitudinal mixed effects models. Results: Mean ALSFRS‐R postslope in 3,367 patients was ?0.99 points/month. Preslope and postslope were correlated and had powerful effects on survival. ALSFRS‐R trajectories were slightly accelerated overall, but slope and direction/degree of curvature varied. Subscore decline was sequential by site of onset. Respiratory subscore decline was the least steep. Discussion: Variable curvilinearity of ALSFRS‐R trajectories confounds interpretation in clinical studies that assume linear decline. Subscore trajectories recapitulate phenotypic diversity and topographical progression of ALS. ALSFRS‐R is better used as a multidimensional measure. Muscle Nerve 57 : 937–945, 2018