Hemolytic-uremic syndrome following bone marrow transplantation in adults for hematologic malignancies

One hundred and sixty eight adult patients with B-cell non-Hodgkin's lymphoma (NHL) and other hematologic malignancies who underwent autologous or allogeneic bone marrow transplantation (BMT) were investigated for the subsequent development of hemolytic-uremic syndrome (HUS). All patients were conditioned with cyclophosphamide and total body irradiation. When examined at 3-month intervals for the first year post-BMT, all patients had uniform measurements of hematocrit (Hct) and serum creatinine. Sixteen patients who initially exhibited Hct and creatinine values that were normal range for the BMT populations developed a sudden decrease in Hct and increase in creatinine between 3 and 11 months post-BMT and fulfilled the clinical and laboratory criteria for HUS. None of these patients had known active cytomegalovirus infection, graft-versus-host disease, or cyclosporine administration. The degree of decrease in Hct and creatinine elevation ranged from solely laboratory abnormalities to a clinically significant syndrome. Twelve of the 16 patients developed acute clinical complications of congestive heart failure, hypertension (HTN), or peripheral edema. Twelve patients required red blood cell support, whereas only four patients required platelet transfusions. Both hemolytic anemia and thrombocytopenia have resolved in virtually all cases. At a mean follow up of 18 months postdiagnosis, creatinine elevations have persisted along with HTN. All patients have survived without life-threatening long-term sequelae. With the increasing use of BMT as a curative modality for patients with hematologic malignancies, it becomes important to prospectively monitor patients for the development of HUS and its potential long-term impact on renal function.     

Sleep apnoea caused by rheumatoid arthritis

Sleep apnoea syndrome (SAS) is a rarely documented, but possibly lethal, complication of the instability of the cervical spine in rheumatoid arthritis. Five patients with SAS of a central or peripheral origin are presented, and the problems of recognizing and diagnosing the syndrome are discussed. We hope that clinicians will become more aware of the existence and the different aetiologies of SAS, thus improving early recognition and appropriate treatment. Adequate treatment has proven to increase survival in peripheral SAS and seems to be successful in doing so in central SAS.      

Investigating the inner speech of people who stutter: evidence for (and against) the covert repair hypothesis

In their Covert Repair Hypothesis, Postma and Kolk (1993) suggest that people who stutter make greater numbers of phonological encoding errors, which are detected during the monitoring of inner speech and repaired, with stuttering-like disfluencies as a consequence. Here, we report an experiment that documents the frequency with which such errors are made. Thirty-two people who stutter (PWS) and thirty-two normally fluent controls, matched for age, gender and education, recited tonguetwisters and self-reported any errors they perceived themselves to have made. In 50% of trials the tonguetwisters were recited silently and errors reported were those detected in inner speech. Compared to controls, PWS produced significantly more word-onset and word-order errors. Crucially, this difference was found in inner as well as in overt speech. Comparison of experimenter ratings and participants' own self-ratings of their overt speech revealed similar levels of accuracy across the two groups, ruling out a suggestion that PWS were simply more sensitive to the errors they made. However, the frequency of participants' inner-speech errors was not correlated to their SSI4 scores, nor to two other measures of stuttering severity. Our findings support Postma and Kolk's contention that, when speech rate is held constant, PWS make, and therefore detect, more errors of phonological encoding. They do not, however, support the hypothesis that stuttering-like disfluencies in everyday speech stem from covert repairs of errors of phonological encoding. Learning outcomes: Readers will learn about three current psycholinguistic theories of stuttering, and how speech-errors elicited during tonguetwister recitation can be used to explore the controversies that exist surrounding: (a) Whether or not people who stutter are more prone to making language production errors; and (b) The extent to which stuttering-like disfluencies stem from covert repairs of language-production errors.