Dialysis-related amyloidosis: history and clinical manifestations

Dialysis-related amyloidosis (DRA) or beta(2)-microglobulin amyloidosis (A beta(2)M) is a unique type of amyloidosis that has been described in individuals with both long-standing chronic renal disease and end-stage renal disease (ESRD). It has been associated with serious complications that significantly add to the morbidity of long-term dialysis patients. The deposition of beta(2)M in amyloid fibrils in various joint and osteoarticular surfaces leads to the clinical complaints and findings typical of this disorder. However, a visceral form with systemic organ involvement has also been described. Despite advances in the understanding of this disorder and in the delivery of dialysis, the ability to alter the incidence of DRA and its course remains uncertain.     

Plasma exchange for severe hypertriglyceridemia-induced pancreatitis in an orthotopic heart transplant recipient

We here report a 33-year old male with diabetes, hypertension and history of orthotopic heart transplantation treated by plasma exchange for severe HTG (hypertriglyceridemia) induced pancreatitis. At the time of presentation, his serum TG (triglyceride) level was 10,278 mg/dL. He underwent one of the three planned sessions of plasma exchange, resulting in a decrease in TG level from 4728 mg/dL to 1708 mg/dL. The hospital course was complicated with shock, hemorrhagic transformation of the pancreatitis and acute respiratory distress syndrome. This prevented any further plasma exchange sessions. He was subsequently discharged home in a stable state and TG level of 80 mg/dL. Plasma exchange can be safely used to manage HTG induced pancreatitis in heart transplant recipients.     

C-reactive protein and coronary heart disease: a critical review

Modestly elevated baseline concentrations of C-reactive protein (CRP), the classical acute phase protein, are associated with the long-term risk of coronary heart disease in general populations, whilst the major acute phase response of CRP following myocardial infarction is associated with death and cardiac complications. The pathogenic and clinical significance of these associations is controversial. Here we critically review the evidence and describe large-scale epidemiological studies, novel experiments and possible specific therapies which will rigorously inform the debate. We distinguish between the potential pathogenicity of high acute phase circulating CRP concentrations in individuals with substantial tissue damage and modest but persistent increases in baseline values in generally healthy subjects.     

Intractable vomiting due to a brainstem lesion in the absence of neurological signs or raised intracranial pressure

^a St Mark's Hospital, Northwick Park, Watford Road, Middlesex HA1 3UJ, UK, ^b Stobhill General Hospital, Glasgow     

The relationship between body mass index and outcomes in leukemic patients undergoing allogeneic hematopoietic stem cell transplantation

Hematopoietic stem cell transplantation (HSCT) is being used increasingly in an attempt to cure many hematological disorders. Obesity has become a world wide phenomenon and is a known risk factor for numerous medical conditions, but its role in transplant outcomes remained controversial. Total of 192 patients with acute leukemia who underwent sibling HLA matched HSCT were analyzed to find the effect of pre-transplant body mass index (BMI) on transplant outcomes such as time to engraftment, infections, graft vs. host disease (GvHD), and overall survival (OS) for the period of three yr (April 2006-March 2009). There was a significant correlation between higher pre-transplant BMI and shorter engraftment time (p = 0.010); but no relation between BMI and GvHD, infection, and OS was found. The results of this study showed that patients with higher BMI may have a shorter engraftment time; but lower, although not significant, survival rate compared to non-obese patients.