Terminal acute myelogenous leukemia in a patient with congenital agranulocytosis

Congenital agranulocytosis terminating in acute myelogenous leukemia has been previously reported in only two cases of adolescent males. We describe the clinical and laboratory features of a 13-year-old male with congenital agranulocytosis, treated with G-CSF with initial good neutrophil response, who subsequently developed acute myeloid leukemia. This rare complication may define a preleukemic subset of patients for whom G-CSF therapy is ineffective. The diagnostic challenges of this case are presented.     

Metabolic disorders in survivors of myocardial infarct

Several lines of evidence have clearly established the role of lipoproteins as risk factors for the development of atherosclerosis. Epidemiologic studies from different countries have found that about one third of myocardial infarction survivors under 60 years of age are hyperlipidemic. The acute stress reaction occurring in the first hours following an acute myocardial infarction causes distinct changes in the patient's metabolic profile, these changes include a significant reduction of total cholesterol and cholesterol associated with low density lipoproteins and a usually mild elevation of blood glucose. With the purpose of establishing the prevalence and severity of lipoprotein disorders found in myocardial infarction survivors living in Mexico city we conducted a prospective study of 106 consecutive admissions to the coronary care unit at the National Institute of Cardiology with the fully proven diagnosis of acute myocardial infarction, we included only patients younger than 60 years of age that could be sampled within the first 72 hours of the appearance of typical symptoms, at this time the coronary risk factor profile was assessed and blood samples were drawn (acute sample). After three months of the diagnosis we sampled 81 of the original 106 patients (chronic sample). The comparison of these 81 patients showed remarkable differences in the lipid values obtained on each sample. The mean value for total cholesterol in the acute sample was 225 mg/dl whereas the corresponding value for the chronic sample was 240.5 mg/dl (p less than 0.005). This difference was also highly significant for the low density fraction. On the basis of the chronic sample analysis we estimated a prevalence of hyperlipoproteinemia of 35.8%. (II: 18.5%, III: 2.5%, IV: 14.8%), an additional subgroup of 10 patients (12.3%) had the hypo-HDL phenotype raising the number of subjects at risk for atherosclerosis to as high as 48.1% considering only the lipoproteins. The prevalence figures for the rest of the risk factors were as follows: 70.3% for tobacco smoking, 35.8% for Systemic Arterial Hypertension, 33.4% for Obesity and 30.8% for Diabetes Mellitus. Among the group of 81 patients, 17 were known diabetics, eight additional cases of Diabetes Mellitus were diagnosed at the chronic phase (two with fasting hyperglycemia and six with diagnostic oral glucose tolerance tests). The "acute plase" glycemia for these eight subjects was significantly higher (mean: 98.4 mg/dl) than the corresponding value for the non diabetic patients (mean: 83.4 mg/dl p less than 0.002), the seventeen known diabetics had a mean glycemia of 150.6 mg/dl in the acute sample.(ABSTRACT TRUNCATED AT 400 WORDS)     

Are Texas dentists burned out?

Burnout is a syndrome of emotional exhaustion, depersonalization, and reduced personal accomplishment that can occur among individuals who do "people work" of some kind. Health care providers, including dentists, are at high risk for burnout because their occupation often requires intense interaction with fearful, demanding patients on a daily basis. The purpose of this study was to assess the prevalence of burnout among Texas dentists using a standardized measurement device, the Maslach Burnout Inventory (MBI). Dentists attending the 1989 Texas Dental Association Annual Session were surveyed using the MBI. The respondents' results were analyzed and compared to each other as well as to the norms of medicine and to society (human service workers nationwide). Among the Texas sample, male dentists scored significantly higher for emotional exhaustion and depersonalization than did female dentists. When compared to the norms of medical workers, Texas dentists scored higher burnout in the depersonalization subscale, but lower burnout for personal accomplishment. The Texas sample again scored lower for burnout in the personal accomplishment subscale when compared to societal norms. At no time did the Texas sample fall into the high range of experienced burnout as defined by the survey instrument. Texas dentists did not appear "burned out" as a group and actually demonstrated an increased sense of personal accomplishment in their profession.     

Axotomy-induced changes in the expression of a type III neuronal intermediate filament gene

The effect of axotomy on the expression of the 57 kDa neuronal intermediate filament (IF) protein in adult rat dorsal root ganglion (DRG) neurons was examined. This IF protein is known to have an exclusively neuronal localization but is considerably more limited in its distribution in the nervous system than the neurofilament (NF) triplet proteins. The 57 kDa neuronal IF protein is similar (and perhaps identical) to the protein "peripherin" and is known to be the product of a Type III IF gene. Since the down-regulated expression of NF proteins (products of type IV IF genes) has been well established, it was of interest to determine whether the novel 57 kDa IF protein was regulated in a similar or different manner from that of the NFs in axotomized neurons. In vitro pulse-labeling of DRGs with 35S-methionine: cysteine followed by 2-dimensional gel electrophoresis/fluorography revealed that the synthesis of the 57 kDa neuronal IF protein was increased 2 weeks after sciatic nerve crush. Immunocytochemical studies using a polyclonal antibody to the 57 kDa neuronal IF protein showed that the immunodetectable levels of this protein increased in DRG neurons after peripheral axotomy. In the normal DRG, staining was localized almost exclusively to small-sized neurons. At 2 weeks after axotomy, however, large- and medium-sized neurons also became immunoreactive; in addition, the overall level of staining in the DRG was greater than normal. Quantitative analysis of in situ hybridizations of DRG neurons with a 35S-labeled cDNA probe specific for the 57 kDa neuronal IF protein revealed a significant increase in the level of 57 kDa IF mRNA in the large-sized (greater than 1000 microns2) neurons 2 weeks after axotomy; the level of 57 kDa IF mRNA in the small neurons was not different from normal at that time. Finally, using a newly developed paradigm for examining the composition of regenerating axons by axonal transport, we determined that significant amounts of the 57 kDa neuronal IF protein were conveyed into the regrowing axonal sprouts of DRG neurons. When DRG neurons were conditioned by a previous axotomy (a crush axotomy of the distal sciatic nerve 2 weeks earlier) and then stimulated to regenerate axons by a second crush axotomy located very close to the DRG, the regenerating sprouts incorporated and conveyed significantly more 57 kDa IF protein by slow axonal transport than did those elaborated by unprimed DRG neurons.(ABSTRACT TRUNCATED AT 400 WORDS)     

Surgery in Jehovah’s Witnesses

This is a retrospective study of the outcome of surgical procedures in patients who were Jehovah's Witnesses. Over a 75-month period, 58 Jehovah's Witness patients had 78 surgical procedures at the Vancouver General Hospital. Three patients had preexisting anaemia of less than 100 g.L-1 haemoglobin. Postoperative haemoglobin concentration decreased below 50 g.L-1 in three patients. One patient had a postoperative haemoglobin of 34 g.L-1 (haematocrit 10.1 per cent) and survived. One patient died from uncontrollable postoperative haemorrhage. Perioperative morbidity was not uncommon, including significant hypotension (eight cases), cardiac arrhythmias (six), myocardial ischaemia (three), excessive bleeding (four), postoperative nausea or syncope (four), and wound or urinary tract infection (four).     

Pre-operative parenteral nutrition in patients with oesophageal cancer: a prospective, randomised clinical trial

A prospective randomised clinical trial was conducted to examine the efficacy of 2 weeks pre-operative parenteral nutrition (PPN) for the prevention of complications following surgery for oesophageal cancer. Forty patients were studied, the diet of twenty being supplemented by pre-operative parenteral nutrition. There were no significant differences in age, nutritional status, tumour staging and histology between the two groups of patients. The use of PPN resulted in a significant gain in body weight and nitrogen but failed to produce an overall reduction in post-operative morbidity and mortality rates. However patients receiving PPN exhibited two types of changes in serum albumin levels. Those with a fall in serum albumin levels associated with an increase in body weight (indicating an expansion of extracellular volume) had a significantly higher incidence of post-operative pulmonary complications than the group exhibiting a rise in serum albumin levels concomitant with increase in body weight. These data suggested that two weeks PPN might not be adequate in certain patients and a longer period of PPN is required. They also show no clinical benefit from the routine use of pre-operative parenteral nutrition in all patients, but do not exclude benefit in selected groups.     

Abortive neovascular outgrowths discovered during vitrectomy for diabetic vitreous haemorrhage

Abortive neovascular outgrowths from the retina were identified in 13 out of 34 eyes (38%) undergoing vitrectomy for diabetic vitreous haemorrhage. Postoperatively, fluorescein angiography of the lesions demonstrated a characteristic smoke-stack pattern of dye leakage. Each outgrowth appears to represent a focal variant of preretinal neovascularisation complicating retinal capillary non-perfusion and inner retinal ischaemia, their distinctive features being determined primarily by the absence of a cortical vitreous scaffold on the retinal surface at the time of vasoproliferation.     

Advances in liquid chromatography and related methodologies for therapeutic drug monitoring

In this article the merits of current liquid chromatography (LC) columns and techniques are reviewed, to include the following topics: (1) a brief introduction to rational therapeutic drug monitoring (TDM) to justify drug measurements; (2) selected recent survey results from the College of American Pathologists (CAP) to establish the current utilization pattern of LC for TDM in the USA; (3) LC analyses of major classes of drugs--antiarrhythmics, antidepressants, antiepileptics, antimicrobials, cyclosporine, and others--with emphasis on analysis of these drugs in human serum or plasma, by focusing on the less usual, reversed-phase functional groups such as CN and phenyl, and by the use of "mini" columns, silica and polymeric columns, the emphasis being reduced on the well-established C-18 columns; (4) high-speed LC; (5) various approaches of direct sample analysis--solvent extraction, automated sample processing, column switching, micro-injections, micellar chromatography, electrochemical detection with photolytic derivation, and the internal surface reversed-phase column of Pinkerton; (6) microbore LC drug analysis; (7) clinical chiral separation; and (8) overall conclusions.     

Trisomy 22 in acute myeloid leukemia: a marker for myeloid leukemia with monocytic features and cytogenetically cryptic inversion 16

Trisomy 22 is an uncommon chromosomal abnormality in acute myeloid leukemia. Recent studies, however, have shown an association between trisomy 22 and acute myeloid leukemia with a monocytic component, and in particular, acute myelomonocytic leukemia with marrow eosinophilia. Furthermore, it has also been suggested that trisomy 22 was in fact only a secondary chromosomal change occurring in acute myeloid leukemia with inv(16). In this report, we analyze the morphological, cytogenetic, and molecular findings of three cases of acute myeloid leukemia with trisomy 22 but without cytogenetic evidence of inv(16). The results indicate a consistent association between trisomy 22 and inv(16), the latter being cytogenetically cryptic in some cases. This finding is of potential diagnostic and therapeutic significance.