Biomedical subjects
Publications and source records attributed to J Rees.
Paraneoplastic neurological syndromes cause severe neurological dysfunction and may lead to the diagnosis of a potentially curable cancer. The range of clinical presentations of these syndromes continues to grow and new antibody associations are constantly being described. Conventional immunosuppressive therapy is rarely helpful and the prognosis of these conditions remains poor.
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Small hepatocellular carcinomas (HCCs) in cirrhotic livers are difficult to detect. This study aimed to assess if small HCCs can be better identified using specific macroscopic sampling criteria and Lipiodol (iodized oil) localization on radiographs, and to evaluate thereby their incidence and morphology. Prospective cirrhotic recipients of orthotopic liver transplantation (OLT) were administered hepatic arterial Lipiodol at angiography. Posttransplantation, the explant livers were sliced and inspected as per routine for unusual nodules. If unusual nodules were found or HCC clinically suspected, the slices underwent soft-tissue radiography. Tissue samples were then taken as per protocol from all nodules with diameter > or =1 cm/unusual appearance/exophytic bulge, and from foci of radiographic Lipiodol uptake or high soft-tissue density. One hundred three lesions were assessed and classified histologically as HCC (n = 55) or regenerative nodule (n = 39) or borderline (n = 9). Sampling according to predetermined macroscopic criteria proved an effective method for identifying small HCCs: 44 cancers were detected, most <5 mm in diameter, additional to those picked up on routine assessment. Green nodules were more likely to be HCC (P < .0001). The cancers were all multifocal, and had characteristic histological features of HCC except for 12 unusual fibrotic cancers in livers with alcoholic cirrhosis. Lipiodolization alone identified one additional HCC. Lipiodol retention on x-ray is fairly specific for HCC (83%), and can even identify lesions 2 mm in diameter. But its sensitivity is poor (45%), and its routine use to identify HCCs in explant livers is not recommended.
'Dementia care mapping' (DCM) is a method of assessing care standards as received by dementia victims. Through the application of this tool, short-comings in care practice can be highlighted in the hope of achieving improvements. The method was employed by the Elderly Mentally III (EMI) unit of a National Health Service trust in South Wales. Baseline evaluations were undertaken on each of the EMI wards containing dementia patients, and reports delivered to the appropriate members of staff. Mean care scores for the unit as a whole were found to lie in the "good' category, as defined by the method. Staff training in the underlying principles of DCM will be followed by re-evaluations of care standards to evaluate the outcome of the attempts made to engender improvements.
The authors present the case of a patient with recurrent hyperparathyroidism who was shown on thallium-technetium subtraction scintigraphy, ultrasound, and magnetic resonance imaging to have recurrent parathyroid tissue in the right side of the neck. The thallium scan also showed intense uptake centrally in the chest, mimicking an intrathoracic adenoma. This was subsequently shown on plain films and a radionuclide bone scan to be due to Paget's disease of the sternum. This case illustrates the value of a combined imaging strategy in preventing an unnecessary thoracotomy after a false-positive nuclear medicine scan.
OBJECTIVE: Muscles are essential components of our sensorimotor system that help maintain balance and perform a smooth gait, but it is unclear whether arthritic damage adversely affects muscle sensorimotor function. Quadriceps sensorimotor function in patients with knee osteoarthritis (OA) was investigated, and whether these changes were associated with impairment of functional performance. METHODS: Quadriceps strength, voluntary activation, and proprioceptive acuity (joint position sense acuity) were assessed in 103 patients with knee OA and compared with 25 healthy control subjects. In addition, their postural stability, objective functional performance (the aggregate time for four activities of daily living), and disabilities (lequesne index) were also investigated. RESULTS: Compared with the control subjects, the patients with knee OA had weaker quadriceps (differences between group mean 100N, CI 136, 63N), poorer voluntary activation (20% CI 13, 25%) that was associated with quadriceps weakness, and impaired acuity of knee joint position sense (1.28 degrees, CI 0.84, 1.73 degrees). As a group the patients were more unstable (p = 0.0017), disabled (10, CI 7, 11), and had poorer functional performance (19.6 seconds, CI 14.3, 24.9 seconds). The most important predictors of disability were objective functional performance and quadriceps strength. CONCLUSIONS: In patients with knee OA, articular damage may reduce quadriceps motoneurone excitability, which decreases voluntary quadriceps activation thus contributing to quadriceps weakness, and diminishes proprioceptive acuity. The arthrogenic impairment in quadriceps sensorimotor function and decreased postural stability was associated with reduced functional performance of the patients.
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OBJECTIVE: The National Institutes of Health have recognized the use of botulinum toxin (Botox) as a therapeutic agent to treat many ophthalmologic and otolaryngologic disorders. There are three reports in the literature regarding the use of Botox to treat cricopharyngeal dysphagia, all describing good results. In the larger study, the toxin was administered under general anaesthetic. This article discusses CT-guided injection of Botox: a relatively noninvasive, out-patient procedure. We also discuss failure of injection in one case and complications in another. CONCLUSION: The patients described experienced improvement in their dysphagia symptoms following injection.
OBJECTIVE: To study the clinical features of patients with autoantibodies to centromere protein CENP-F and the frequency of CENP-F autoantibodies in patients with various diseases. DESIGN: Retrospective clinical and serologic study. METHODS: Thirty-six patients with anti-CENP-F were identified by a characteristic pattern of indirect immunofluorescence (IIF) on HEp-2 cells. Fifty patients with melanoma, 50 with breast cancer, 10 with lung cancer, 354 with systemic sclerosis, 120 with systemic lupus erythematosus and 50 with rheumatoid arthritis were also studied. Recombinant proteins were produced from 5 CENP-F cDNA clones representing amino acids 2192-3317 (p-F1), 5561-7126 (p-F2), 5892-6883 (p-F3), 7538-10,116 (p-F4) and 9242-10,096 (p-F5). The presence of CENP-F antigen was studied in a breast carcinoma cell line, cryosections of breast carcinoma, normal breast tissue and tonsils. RESULTS: Twenty-two of 36 patients with CENP-F antibodies had neoplasms; breast (9/22) and lung (5/22) cancer were the most common diagnoses. Thirty-three sera were available for further study; when tested for reactivity to the recombinant peptides, the sera of 21 of 21 patients with neoplasms and 5 of 12 patients with other diseases bound the C-terminal p-F4 peptide. When the terminal third of the p-F4 peptide (p-F5) was studied, a significant difference in pattern of reactivity was not detected. By comparison, the frequency of reactivity with peptides representing other domains of CENP-F was less than that with p-F4 (p-F2 > p-F3 > p-F1). CENP-F autoantibodies were not found in any of the control sera from patients with systemic lupus erythematosus, rheumatoid arthritis or systemic sclerosis or in unselected sera from various malignancies. CENP-F antigens were identified in breast carcinoma tissue but were rarely observed in normal tissues. CONCLUSIONS: A high proportion of individuals with CENP-F antibodies have neoplasia, and there is a bias among their sera for reactivity with determinants in the carboxy terminal domain of CENP-F. CENP-F antigens appear to be highly expressed in malignant tissues.
Transplantation in the presence of anti-class I antibodies usually results in allograft hyperacute rejection. Because of the perception of its uncertain clinical significance, B-cell crossmatch which identifies presence of anti-class II antibodies is not universally performed. In a retrospective study, the clinical course of renal transplant recipients with IgG anti-B-cell antibodies was analyzed and compared with case control patients transplanted contemporaneously, matched demographically and immunologically. The incidence of hyperacute, acute, and chronic rejection as well as graft loss were significantly higher in the group with anti-IgG B-cell antibodies compared to the control. We conclude that anti-B-cell IgG antibodies are harmful to allografts with a spectrum of events that include hyperacute, acute, vascular and chronic rejection. While allografts were successful in some patients, our experience suggests caution whenever anti-donor B-cell IgG is present. If transplants are performed, then more potent immunosuppression should be used.
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OBJECTIVE: Autoantibodies directed against centromere proteins (CENPs) are a serologic feature in some patients with systemic sclerosis (SSc). Previous studies have focused on autoantibodies to CENPs A, B, and C. CENP-E is a recently described 312-kd protein that also localizes to the centromere. Therefore, we studied the presence of autoantibodies to recombinant CENP-E in patients with SSc. METHODS: Sixty sera from patients with the SSc spectrum of diseases were screened for the presence of autoantibodies against CENP-E, by indirect immunofluorescence and immunoblotting using recombinant CENP-E protein. HLA class II alleles were determined by DNA oligotyping. RESULTS: Among the SSc sera, 15 of 60 (25%) demonstrated antibody reactivity with recombinant CENP-E, and 14 of these 15 sera (93%) had antibodies directed against another CENP. Anti-CENP-E was seen in 13 of 30 sera with anti-CENP (43%). All patients with anti-CENP-E had a limited form of SSc, known as the CREST variant (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasias). When patients with anti-CENPs A, B, or C were compared with patients with anti-CENP-E, no unique clinical features in the anti-CENP-E positive group were identified. Ninety-three percent of the patients with anti-CENP-E had HLA-DQB1 alleles that had polar amino acids at position 26 (primarily DQB1*05), similar to patients with other CENP autoantibodies. CONCLUSION: Antibodies to CENP-E are common in patients with SSc, and are seen in higher frequency in sera from patients with a limited form, or CREST variant, of the disease.
Fibro-osseous dysplasia is a developmental non familial, benign anomaly of bone development occurring in single or multiple bones, characterised by the replacement of normal bone by fibro-osseous tissue. We describe the case of a fourteen year old boy, which illustrates many of the difficulties confronting surgeons dealing with this uncommon condition. Complete surgical excision was required, before a definitive histological diagnosis could be established. We report the clinical, radiological and pathological features of this unusual case. We review the pathology of the condition and discuss the approaches to surgical management.
Serial isolates of Cryptococcus neoformans from 33 human immunodeficiency virus-infected patients with cryptococcosis were analyzed to determine whether persistence might result from reinfection with a new cryptococcal strain or acquisition of antifungal resistance. Isolates were subtyped by multilocus enzyme electrophoresis (MEE), electrophoretic karyotyping (EK), random-amplified polymorphic DNA (RAPD), and the CNRE-1 DNA probe, MICs of amphotericin B, fluconazole, and 5-fluorocytosine were determined. No changes in MEE or RAPD subtypes were detected in serial isolates from any patient. Isolates from 8 patients (24%) showed alterations in EK only (mobility change in two or more bands) but not with any other subtyping method. MICs did not change significantly in isolates from 30 patients. In 1 case, the fluconazole MIC increased stepwise over 18 months, suggesting development of resistance. These overall invariant subtyping and MIC results confirm previous studies suggesting that persistent cryptococcal infection is due to relapse rather than reinfection or antifungal drug resistance.
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