Observations on two members of the Swedish family with congenital dyserythropoietic anaemia,type III

Abstract: Two affected individuals of the Swedish family with CDA, type III, in which the disease is transmitted as an autosomal dominant character, were studied. Both cases displayed features hitherto undescribed in this family but described in patients with CDA, type III, in whom the inheritance may have been as an autosomal recessive character. Such features were: (a) haemosiderinuria, (b) grossly disorganised erythroblast nuclei, (c) differences in the ultrastructural appearances of individual nuclei within the same multinucleate erythroblast and (d) intraerythroblastic inclusions resembling precipitated globin chains. In both cases the giant mononucleate erythroblasts and the multinucleate erythroblasts had total DNA contents up to 28c (1c = haploid DNA content) and 48c respectively, and some DNA synthesising bi- and multinucleate erythroblasts contained one or more nuclei which were unlabelled with ³H-thymidine. These findings are similar to those in patients with the autosomal recessive type of disease. Thus no major phenotypic differences are yet apparent between cases of CDA, type III, with different patterns of inheritance. Analysis of the surface erythrocyte proteins of the 2 Swedish CDA, type III, patients with monoclonal antibodies recognising Band 3, glycophorins A, B, C and D, Rh, CD44, CD47, CD55, CD58, CD59, Lutheran, Kell, LW and acetylcholinesterase did not reveal any gross abnormality of expression of these proteins. A slightly altered expression of blood group antigens A and H was revealed by the lectins Dolichos biflorus and Ulex europaeus and the Mr of Band 3 as judged by SDS polyacrylamide gel electrophoresis was also slightly reduced, suggesting that there may be minor alterations in the degree of N-glycosylation of some red cell membrane constituents.     

Patients with Alcohol Problems in the Emergency Department,Part 1: Improving Detection*

Abstract. Medical and social problems related to alcohol use are frequently seen in the ED. Often, the tempo of emergency medicine practice seems to preclude assessment beyond that required by the acute complaint. However, detection of ED patients with alcohol problems can occur using brief screening tools. This article was developed by members of the SAEM Substance Abuse Task Force, and describes screening tools that have been used successfully to identify atrisk and dependent drinkers. Their brevity, reproducibility, and accuracy vary somewhat, but screening can be realistically performed in the busy ED setting. The early detection of patients with alcohol problems would provide the opportunity for early intervention, and may reduce subsequent morbidity and mortality in this patient population.     

Paranasal sinus bony anatomic variations and mucosal abnormalities: CT analysis for endoscopic sinus surgery

Coronal plane computerized tomographic (CT) scanning has dramatically improved the imaging of paranasal sinus anatomy as compared to sinus radiographs. Increasingly, subtle bony anatomic variations and mucosal abnormalities of this region are being detected. Data regarding the “background” prevalence of these findings are needed to determine their clinical relevance. A detailed analysis of coronal plane CT scans of the paranasal sinuses obtained in 202 consecutively imaged patients was conducted. Special attention was directed toward identifying bony anatomic variations and mucosal abnormalities. Anatomic variations studied included pneumatization of the middle turbinate, paradoxical curvature of the middle turbi-nate, Haller's cells, and pneumatization of the unci-nate process. Such bony anatomic variations were detected in 131 (64.9%) of 202 patients and were found with a similar frequency in patients scanned for sinus complaints and in those scanned for nonsinus reasons. Mucosal abnormalities were detected in 168 (83.2%) of 202 patients. For those patients scanned during the evaluation of sinus-like complaints, mucosal abnormalities were noted in 153 (92.2%) of 166 cases, and were predominantly detected in the anterior ethmoid region. For patients scanned during nonsinus evaluations, mucosal abnormalities were detected in 15 (41.7%) of 36 cases, without predilection for the anterior ethmoid region. Discussion regarding the prevalence and clinical significance of paranasal sinus bony anatomic variations and mucosal abnormalities is included as a guide to assist the otolaryngologist and/or radiologist in the evaluation of coronal sinus CT scans.     

Poxvirus-based vaccine therapy for patients with advanced pancreatic cancer

Purpose  

An open-label Phase 1 study of recombinant prime-boost poxviruses targeting CEA and MUC-1 in patients with advanced pancreatic cancer was conducted to determine safety, tolerability and obtain preliminary data on immune response and survival.     

Waiting: the experience of persons in a critical care waiting room.

The purposes of this study were to discover the essences of the experience of waiting, to provide new knowledge about what it is like to wait, and to contribute to nursing knowledge. Participants were 12 persons who were family members or friends of persons in an adult critical care unit. The Parse research method was used to answer the research question: What is the structure of the lived experience of waiting? The central finding of this study was this structure: The lived experience of waiting is a vigilant attentiveness surfacing amid an ambiguous turbulent lull as contentment emerges with uplifting engagements. The structure is discussed in relation to nursing knowledge and in relation to how it can inform future research and practice. © 2002 John Wiley & Sons, Res Nurs Health 25:58–67, 2002.     

Keeping the focus on public health: the struggles of a tobacco prevention task force.

This case study examines a nonlegislative task force as it struggled to reach internal consensus despite external political constraints. The study highlights the convergence of politics and science, revealing complex issues likely to be confronted by advocates and public health officials. Three themes capture participants' experiences: context, sizing up the opportunities and constraints; task force process, tacit strategy to operate outside the political context and play the science card; and aftermath, a glass half full. The task force took advantage of ambiguous parameters, crafting a comprehensive statewide plan to reduce tobacco use and breaking out of the common public health paradigm of allowing budget considerations to drive program design. These internal victories could not sustain a policy success in the legislature. However, the group's product sets science-based standards for future program development, and the task force's process provides valuable insights into other states developing tobacco prevention and control policies.     

Histology of a lengthened human tibia.

We describe the histology of a specimen taken from an amputated leg seven months after a 15 cm bone gap in the tibia had been closed by bone transport. Lengthening appeared to have occurred by repeated minor trauma to the bone, with the fractured trabeculae in sufficiently close contact for the repair process to proceed. Osteogenesis did not occur through a cartilage phase, but the fracture gaps were bridged by collagen fibres, around which new bone formed. Microfractures had repaired by primary healing with woven bone and with no microcallus. Small regions of bone were necrotic. Resorption of the necrotic bone and remodelling of the immature bundle and woven bone were still at an early stage, suggesting that complete remodelling in man may take years rather than months.