Diagnosis of herpetic uveitis and keratouveitis

The use of seven benzoyl-L-amino acids for the detection of carboxypeptidase activities in strains of Gram-negative aerobic and facultatively anaerobic bacteria is reported. A simple overnight assay was designed using ninhydrin for the demonstration of the amino acid released by hydrolysis. Detection of carboxypeptidase activity was shown to have some taxonomic relevance within the Enterobacteriaceae; it was also useful for differentiation within the genus Acinetobacter and for distinction between Pseudomonas aeruginosa and Pseudomonas fluorescens.     

Mortality and causes of death in a Swedish series of systemic sclerosis patients

OBJECTIVES: To analyse survival rates and the causes of death in a systemic sclerosis (SSc) population, and to evaluate the occurrence of fatal malignant neoplasms and their possible association with oral cyclophosphamide (CYC) treatment. METHODS: Survival was calculated for 249 SSc patients followed up for up to 13 years. Mean (SD) follow up was 5.8 (4.2) years. The 49 decreased patients were subdivided according to causes of death and its relation to SSc. Fatal malignancies in CYC treated patients were compared with those occurring in non-CYC treated patients. RESULTS: The overall 5 and 10 year survival rates were 86% and 69% respectively. There was a 4.6-fold increased risk of death, as compared with the general population. Prognosis was worse in the diffuse cutaneous involvement (dSSc) and male subgroups than in the limited cutaneous involvement (1SSc) and female subgroups. Of the 49 deaths, 24 were attributable to pulmonary complications such as pulmonary fibrosis, pulmonary hypertension, pneumonia or pulmonary malignancy. Treatment with oral CYC did not increase the risk of dying of cancer. CONCLUSIONS: Mortality is increased both in the SSc population as a whole and in its different subsets (dSSc and 1SSc). Prognosis is worst among male patients with dSSc. However, the 5 year survival rate was better than those reported from earlier studies. Most patients die of cardiopulmonary disease. Five of seven fatal lung cancers were adenocarcinomas, possibly caused by chronic inflammatory disease of the lung. In this study, CYC treatment was not associated with an increased incidence of fatal malignant neoplasms.     

Mortality and causes of death of 344 Danish patients with systemic sclerosis (scleroderma)

A variety of pressures to change curriculum have resulted in revision of pathology teaching in most medical schools in the United States. Responses obtained from 71% of schools on a recent survey of pathology teaching indicate the following: There are wide variations in the extent of use of various teaching modalities with resulting emergence of different teaching formats which can be categorized as "traditional," "enhanced traditional," predominantly "problem based" or "case oriented," entirely "problem based," or some form of "hybrid" of traditional and problem based. The traditional lecture and laboratory continue to be the primary modes of teaching in the vast majority (74%) of schools, however, 53% also use other approaches, eg, small group discussion, case studies, or conferences to enhance instruction. The lecture remains an important component of instruction in all major models. The form and extent of the laboratory use varies remarkably from minimal to large number of hours of instruction. The laboratory material is incorporated into small group discussion or conferences in many schools. The use of small group discussions (presently, by 79% of schools) has increased since the last survey conducted in 1986. The mean curricular time (presently 188 hours) has dropped, however, it is not easy to quantify pathology teaching in many schools with increasing integration. Instruction is entirely integrated, multidisciplinary in 28% of schools. Computer-aided instruction is used in some form to varying extents in 66% of schools, with the majority using it as a supplementary tool. There are persistent concerns about faculty time, and high student-faculty ratio.     

Sudden hearing loss and uveitis as a form of presentation of neurosarcoidosis

BACKGROUND: The pathologies and size of adrenal lesions removed through the open posterior and laparoscopic routes share remarkable similarities. This study aims at comparing the relative merits of these two approaches. METHODS: Patients with adrenal tumors operated on by posterior approach (n = 56) and transabdominal laparoscopic approach (n = 12) between January 1981 and May 1997 were retrospectively reviewed. RESULTS: The two groups were comparable in terms of age, and the position, size, and weight of the tumor. The operative time of posterior adrenalectomy was significantly shorter than that of laparoscopic adrenalectomy (median 120 minutes versus 160 minutes), whereas laparoscopic adrenalectomy was associated with less parenteral analgesic requirement (median 0 mg versus 225 mg), a shorter hospital stay (median 3 days versus 5 days), and a shorter duration off work (median 11 days versus 26 days). The estimated blood loss was also significantly reduced in the laparoscopic group (median 50 mL versus 150 mL). CONCLUSIONS: Laparoscopic adrenalectomy is replacing posterior adrenalectomy to become the procedure of choice for the majority of patients undergoing adrenalectomy.     

Clinical and biological markers of secondary uveitis: results of a discriminant analysis

BACKGROUND: The association of uveitis and systemic disease is well known. Patients suffering from uveitis often undergo a extensive battery of tests in order to detect underlying disease, but the efficiency of such screening is uncertain. The aim of this study was to investigate useful clinical data for recognizing secondary uveitis. PATIENTS AND METHODS: We conducted a prospective analysis of 115 patients with uveitis of unknown etiology. All of them were included in an extensive protocol study. Four groups were considered: specific ocular disease (SOD), idiopathic uveitis, HLA-B27 associated uveitis without arthritis (HLA-B27-AU) and secondary uveitis. Groups were compared by analysis of variance for continuous variables, and chi 2 test or Student's t-test for discrete variables. A stepwise multiple discriminant analysis was performed for ranking the variables in order of their usefulness for distinguishing idiopathic and secondary uveitis. RESULTS: We diagnosed 11 SOD (9.6%), 54 idiopathic uveitis (47%), 6 HLA-B27-AU (5.2%) and 41 secondary uveitis (35.7%). The discriminant analysis showed that age, an elevated erythrocyte sedimentation rate, presence of cutaneous lesion, joint pain and genital ulcers are the strongest predictors of secondary uveitis. This model classification functions detected 92.5% of idiopathic uveitis and 72% of secondary uveitis. The global percentage of patients with a correct diagnosis was 84.6%. CONCLUSIONS: Anamnesis, physical examination and basic laboratory tests are sufficient tools for the diagnostic approach of the majority of patients with uveitis. Subsequent diagnostic procedures must be planned in each patient to confirm a specific disease.     

Weightlessness: a matter of gravity

Circular dichroism (CD) and infrared spectroscopy (IRS) studies of aqueous solutions of fourth N-terminal peptides of histone H4 with different chain length were carried out under various conditions. It was shown that all studied peptides had conformation of extended left-handed helix as well as poly-1-proline II at the acidic and neutral pH, in moderate ionic strength (0,15), in 80% ethanol, 0,2 M sodium dodecylsulphate, in 8 M urea and 5 M guanidinum hydrochloride. This conformation was changed by raising temperature, under transition to the range of basic pH and in the concentrated solutions of CaCl2 (5M).     

Incidence and prevalence of different uveitis entities in Finland

A prospective, open, multicentre study was performed to investigate the efficacy and safety of long-term treatment with cyclosporin in adults with severe atopic dermatitis. Subjects were treated for a maximum of 48 weeks. For the first 8 weeks, cyclosporin was administered at 2.5 mg/kg per day. The dose was then adjusted according to response. Disease activity was monitored using the six-area, six-sign score and the proportion of skin involved. Pruritus and sleep disturbance were assessed using four-point scales. Response was further evaluated on a five-point scale. Adverse events, blood pressure and serum biochemistry were monitored. Tolerability was assessed on a five-point scale. One hundred subjects were enrolled and 65 completed 48 weeks of treatment. Withdrawals occurred due to remission (three), inadequate response (seven), protocol violations (11) and adverse events (14, of which seven were probably treatment related). Cyclosporin produced rapid and highly significant improvements in all indices of disease activity. Sixty-five subjects considered that they had shown a considerable improvement or complete clearance of disease. Most patients relapsed after cessation of treatment, but neither signs nor symptoms had returned to baseline severity 8 weeks later. Blood pressure and serum creatinine levels increased slightly, and in one subject renal impairment was a major factor contributing to withdrawal of the drug. Overall, 85 subjects rated the tolerability of cyclosporin as good or very good. The results indicate that cyclosporin has a place in the long-term treatment of severe atopic dermatitis provided that appropriate patients are selected and careful monitoring is performed.     

Uniparental disomy: a review of causes and clinical sequelae

1) Uniparental disomy (UPD) results from the exceptional derivation of a pair of the offspring chromosome from one parent only and has been documented thus far for chromosomes 2, 4, 5, 6, 7, 11, 13, 14, 15, 20, 21, 22 both X's and the XY pair. Its consequences on the phenotype may result from three potentially harmful effects, namely isodisomy, interference with genomic imprinting and, occasionally the vestigial aneuploidy from which UPD may have originated. 2) In isodisomy, the uniparental pair is partially or entirely homozygous, through the duplication of a same chromosomal DNA template, thus bringing about an increased risk of recessive disorders. As a result, conditions such as cystic fibrosis, a type of osteogenesis imperfecta, thalassemia alpha or beta, retinoblastoma, rod monochromacy, etc., have now been reported. 3) Duplication of both homologues of a parental pair in a diploid genome is called heterodisomy. Both iso- and heterodisomy may also cause disruption of the genomic imprints normally modifying the differential expression of some maternal and paternal genes or gene sequences needed for eugenic growth and development, in the course of normal biparental inheritance. Such a disturbance can be one of the causes of congenital clinical entities as well defined as Angelman, Prader-Willi or Beckwith-Wiedemann syndromes and some new syndromes, for instance for UPD 7 mat, UPD 14 mat and, probably also 14 pat. 4) All in all, UPD can cause morbidity or lethality by altering imprinting processes, mimicking certain deletions or duplications, generating recessive disorders or prompting malignant tumor development. 5) In the clinical field, UPD occasionally upsets some mendelian tenets of traditional inheritance, and raises, the question of the evolutional role plaid by genomic imprinting (GI). An hypothetical opinion is that one of GI potential side effects is a biased intergenerational preferential display or skip of parental features. This could be so because some of the inherited genes or gene domains only gain maternal or paternal expression in the offspring, as a function of their parental imprint.     

Prison health: a matter of priority

Carboxyhaemoglobin (COHb) concentrations were determined by differential spectrophotometry in blood of 60 healthy adult subjects from various locations in Lagos. Half of these were either occasional or regular tobacco cigarette smokers. Our findings showed that the Lagos dweller has elevated COHb concentration ranging between 7.6%-9.9%, several folds higher than permitted by Air Quality Standards. The range and scatter of COHb in smokers were wider (7.4%-13.0%) than in non smokers. In particular, COHb concentrations were significantly higher in regular smokers than in non smokers by Fisher's exact test (p < 0.0006). Elevated COHb concentrations among smokers were related to frequency of tobacco use (p < 0.01). There was however no statistically significant difference in COHb concentrations when the regular and occasional smokers were taken as a group and compared with the non smokers. Haematocrit measurements showed that a degree of anaemia was present in most of the subjects tested irrespective of smoking status (mean packed cell volume = 36.1). It is inferred from this data that the Lagos dweller has high ambient concentrations of COHb and that these may be further aggravated by cigarette smoking.