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Biomedical subjects

R Buckley

Publications and source records attributed to R Buckley.

At least 19 recordsLinked to original sources

The visual prognosis following HLA-matched keratoplasty for high-risk patients.

We report the clinical details of 30 HLA-matched corneal grafts with a mean follow-up of 29 months. Few of the patients were immunologically naive as almost all had received previous unmatched grafts. The two-year survival rate was 77%, comparable with other series. A majority of patients obtained good vision, and this was statistically superior to a recent large series. Almost all remained on their topical medication indefinitely. The contralateral visual acuity was surprisingly good, but there are few published data for comparison. We were unable to document an enhanced prognosis with a higher degree of HLA-A, B or DR match, possibly because of the small numbers.

Adult

Excimer laser superficial keratectomy for proud nebulae in keratoconus.

Contact lens intolerance in keratoconus may be due to the formation of a proud nebula at or near the apex of the cone. Excimer laser superficial keratectomy was performed as an outpatients with proud nebulae as treatment patients with proud nebulae as treatment for their contact lens intolerance. The mean period of contact lens wear before the development of intolerance was 13.4 years (range 2 to 27 years). Following the development of intolerance, three patients abandoned contact lens wear in the affected eye while the remainder experienced a reduction in comfortable wearing time (mean = 3.75 hours; range: 0-14 hours). All patients had good potential Snellen visual acuity with a contact lens of 6/9 (nine eyes) and 6/12 (one eye). The proud nebulae were directly ablated with a 193 nm ArF excimer laser using a 1 mm diameter beam. Between 100-150 pulses were sufficient to ablate the raised area. Patients experienced no pain during the procedure and reported minimal discomfort postoperatively. In all cases flattening of the proud nebulae was achieved. Seven patients were able to resume regular contact lens wear (mean wearing time = 10.17 hours; range 8 to 16 hours). In three patients, resumption of contact lens wear was unsuccessful because of cone steepness. All patients achieved postoperative Snellen visual acuity of 6/12 or better with a contact lens. Four patients experienced a loss of one line in Snellen acuity. The mean follow up period was 8.3 months (range 2 to 17 months). Excimer laser superficial keratectomy is a useful technique for the treatment of contact lens intolerance caused by proud nebulae in patients with keratoconus. Penetrating keratoplasty is thus avoided.

Contact Lenses

Risk factors for hepatitis C virus infection among injecting drug users in Sydney.

OBJECTIVE: To study risk factors for hepatitis C virus (HCV) infection in injecting drug users (IDUs) from central Sydney. SETTING AND SUBJECTS: All IDUs attending a primary health care facility in central Sydney between December 1991 and November 1992 who underwent HCV antibody testing. METHODS: Information was obtained retrospectively from client forms routinely completed at the time of medical consultation. Additional information on injecting history and practice was obtained from the registration forms of subjects who also attended the needle syringe exchange programme at the same health care facility. RESULTS: Of the 201 IDUs tested, 118 (59%) had HCV antibodies, which did not differ significantly between males and females. HCV prevalence increased significantly with age, being highest in IDUs who were aged 35 years or more (93%) and lowest in IDUs aged under 20 years (17%). HCV prevalence increased significantly with time since first injecting, from 26% for IDUs who had injected for less than 3 years to 94% for those who had injected for more than 10 years. HCV prevalence was also significantly higher in heterosexual IDUs as compared with homosexual male IDUs, and in opiate users as compared with stimulant users, even after adjustment for age and duration of injecting. HCV prevalence was strongly associated with exposure to hepatitis B virus, but was not associated with exposure to HIV. CONCLUSION: Recent HCV transmission indicates ongoing injecting risk behaviour despite HIV prevention efforts, and underlies the potential for increased transmission of HIV through the sharing of injecting equipment. Within the population of IDUs, those who are heterosexual or inject heroin appear to be at increased risk of HCV infection.

Adolescent

Symptomatic bradycardia following the administration of sublingual nitroglycerin.

A case of severe bradycardia with junctional escape immediately following sublingual nitroglycerin is described. Bradycardia is a rare, but well-documented, complication of nitroglycerin and is often reported in the setting of myocardial ischemia or infarction. This particular case reported was complicated by the presence of an angiographically documented congenital coronary anomaly, but did not have either electrocardiographic or enzymatic evidence of ischemia or infarction during this event. Previously reported cases were reviewed, as well as potential mechanisms for this phenomenon. All cases reported to date were easily corrected with passive leg elevation or atropine.

Administration, Sublingual

Limbal transplantation in the management of chronic contact-lens-associated epitheliopathy.

We describe the clinical management of 6 patients who developed a chronic corneal epitheliopathy 1-18 years after commencing soft contact lens wear. All had a history of exposure to thiomersal in contact lens fluids. The corneal changes were characterised by epithelial haze and superficial stromal vascularisation which extended from the limbus towards the visual axis. Five patients were observed for a minimum of 18 months after stopping contact lens wear before undergoing limbal transplantation. A good result was obtained in 1 patient who had worn a contact lens in one eye only. Recurrent epithelial changes were observed on the recipient eyes of the remaining patients who had previously worn contact lenses bilaterally, and in 1 patient epithelial haze also developed adjacent to the donor site in the previously clinically normal donor eye. All 5 patients experienced an improvement in symptoms post-operatively but in 2 patients the visual acuity later deteriorated because of epithelial irregularity. The sixth patient has not had surgery. We conclude that limbal stem cell dysfunction in chronic contact-lens-associated epitheliopathy may be subclinical and that autograft transplantation in bilaterally exposed patients may fail to restore the epithelial phenotype of the host eye whilst jeopardising the epithelial integrity of the donor eye by depleting its stem cell reserve.

Adolescent

The Greater Vancouver Mental Health Service Society: 20 years' experience in urban community mental health.

Caring for people in the community with persistent and disabling mental illnesses presents a major challenge to government, planners and mental health professionals. The success with which mentally disabled people are integrated into community life says much about the society in which we live. This article describes the experience of the Greater Vancouver Mental Health Service Society in offering community-based mental health services to persons with schizophrenia and other major mental disorders over the past 20 years. The key to its success lies in a decentralized, relatively non hierarchical organizational structure which allows committed and skilled multidisciplinary teams to work with patients and their families in their community. The resulting services are fully integrated within the fabric of the community and are responsive to local needs. Partnerships among professionals, patients, families and community agencies result in work that is creative, productive and effective.

Adult

The multi-service network: reaching the unserved multi-problem individual.

A small number of multi-problem, service-resistant individuals in every metropolitan community consume extraordinary amounts of human service at great cost to publicly-funded agencies with less than satisfactory benefit to the individual. This paper describes an innovative collaboration among mental health, alcohol/drug treatment, corrections, forensic, and social and housing agencies to provide more effective services at less cost. The theory of action was that (1) inter-agency communication and (2) external controls developed by core service agencies increase the efficacy of treatment and reduce the cost of caring for multi-problem clients. Agencies refer clients to the Multi-Service Network who are then screened for problematic multi-agency involvement. Case conferences result in individual service plans. Three illustrative cases are described and the results of two evaluative studies summarized. Cost of care for clients appears to have been reduced. Agencies appear to have benefited from improved information and communication. Clients' behavior was stabilized by external controls and more adequate attention to their needs.

Activities of Daily Living

Perioperative cefazolin prophylaxis in hip fracture surgery.

Antibiotics given perioperatively are thought to decrease the occurrence of postoperative wound infection. The duration of treatment for hip fracture surgery is empirical. This randomized, double-blind, single-hospital clinical study was carried out to assess the effect of both antibiotic use and duration of use on wound infections in hip fracture surgery. Wound infection rates in three groups of patients were compared: those who received four doses of cefazolin (108 patients), those who received one dose of cefazolin and three doses of placebo (83 patients) and those who received four placebo doses (121 patients). Results showed an infection rate of 1.6% for the four-dose group, 2.4% for the one-dose group and 3.7% for the placebo group. These differences were not statistically significant, even when both treatment groups were combined and compared with the placebo group. The authors conclude that until more patients are added, empirical use of antibiotics should be continued in patients who undergo hip fracture surgery.

Adult

Seronegative arthritis associated with serological evidence of Yersinia infection in Australia.

Infection due to yersinia enterocolitica is a common antecedent illness in patients with reactive arthritis in Scandinavia, but appears to be less frequent in other countries. In order to examine the frequency of yersinia infection in patients with seronegative arthritis in Australia we examined 22 patients, 15 with ankylosing spondylitis (AS) and seven with Reiter's syndrome (RS). A sensitive ELISA assay was used to detect serum antibodies to the most common serotypes. Six patients (29%) had positive yersinia serology, all were HLA B27 and four had a history of diarrhea preceding the onset of their disease. Four patients with positive yersinia serology had AS and two had RS. Antibodies were directed against Y. enterocolitica biotype 0:3 in three cases, Y. enterocolitica 0:9 in two cases and Y. enterocolitica 0:8 in one subject. Twenty-nine control subjects (13 HLA B27) had no serum antibodies to yersinia. The results of this study indicate that preceding yersinia infection occurs in a significant (p less than 0.05; compared to controls) proportion of patients with HLA B27 related seronegative arthropathies.

Adult

Association of complement allotype C4B2 with anterior uveitis.

We have studied allotypes of the fourth component of complement (C4) in 44 patients with inflammatory eye disease in order to define genetic susceptibility factors further. Twenty-six patients had uveitis (18 had anterior uveitis) and 18 patients had retinal vasculitis. There was an increased incidence of the C4B2 allotype in patients with anterior uveitis (pc less than 0.002), especially in HLA B27 positive males. In contrast, there was no increased incidence of specific allotypes in patients with posterior uveitis or retinal vasculitis. This genetic association may form part of a disease susceptibility supratype in patients with anterior uveitis.

Alleles

Appearance of multiple benign paraproteins during early engraftment of soy lectin T cell-depleted haploidentical bone marrow cells in severe combined immunodeficiency.

Recent advances in the prevention of graft-versus-host disease through postthymic T-cell depletion have allowed the use of haploidentical bone marrow cells for immunologic reconstitution of severe combined immunodeficiency disease. We report a male infant with severe combined immunodeficiency (with normal adenosine deaminase) who developed two IgG kappa and one IgA lambda paraproteins 7 weeks following the administration of 1.4 X 10(9) maternal bone marrow cells depleted of postthymic T cells by soy lectin agglutination and sheep erythrocyte rosetting. Serum IgG rose from 128 to 820 mg/dl, and IgA from 0 to 2400 mg/dl, peaking at 10 weeks postgrafting. By 14 weeks posttransplantation T-cell numbers and function had risen to normal (all dividing T cells had the donor karyotype) and paraprotein concentrations began to decline. These observations strongly suggest that the later-appearing T cells regulated the B-cell clones from which the paraproteins were derived. Failure of such function to appear could account for the increased incidence of B-cell lymphomas in severe combined immunodeficiency.

B-Lymphocytes

Variability in B cell maturation and differentiation in X-linked agammaglobulinemia.

Among seven males with X-linked agammaglobulinemia in an extended pedigree, serum immunoglobulins and antibodies were extremely low in all but one who had a normal IgA (78 mg/dl) and tetanus antibodies (1:19,683). Following bacteriophage phi X 174 immunizations, the oldest failed to clear phage and had no primary or secondary antibody responses. The youngest had normal phage clearance, low primary and secondary antibody responses, and no amplification or switching to IgG. The other four affected had normal or slightly delayed phage clearance, low primary and secondary responses, but some amplification and switching from IgM to IgG which increased with age. Normal percentages of surface immunoglobulin positive cells were present in the two youngest patients, but all seven affected had very low percentages of cells reacting with monoclonal antibodies to B cell surface antigens. Immunoglobulin production by cultured blood B cells was very low and not increased by pokeweed mitogen. However, a majority of Epstein-Barr virus (EBV)-transformed lymphoblastoid cells derived from the blood of four of the patients bore IgD and IgM and reacted with all of the monoclonal antibodies to B cell antigens. Culture supernatants from those lines contained significant quantities of IgM and lesser amounts of IgG and IgA. The studies presented here provide further support for the hypothesis that the primary abnormality in X-linked agammaglobulinemia affects B cells at more than one stage of development rather than just at the level of the pre-B cell.

Adolescent

In vivo inactivation of erythrocyte S-adenosylhomocysteine hydrolase by 2'-deoxyadenosine in adenosine deaminase-deficient patients.

The cytotoxic nucleoside 2'-deoxyadenosine is excreted in excessive amounts by individuals with genetic deficiency of adenosine deaminase, and may be in part responsible for the severe combined immune dysfunction from which they suffer. Earlier studies from this laboratory showed that 2'-deoxyadenosine causes the irreversible inactivation of the enzyme S-adenosylhomocysteine hydrolase by an active site-directed, "suicide-like" process. In this communication we have demonstrated similar inactivation of S-adenosylhomocysteine hydrolase in hemolysate and in intact erythrocytes, as well as a striking deficiency of S-adenosylhomocysteine hydrolase activity in the erythrocytes of three adenosine deaminase-deficient patients. In vivo suicide-like inactivation of S-adenosylhomocysteine hydrolase by 2'-deoxyadenosine may contribute to the cytotoxicity of 2'-deoxyadenosine and to the immune dysfunction in adenosine deaminase deficiency.

Adenosine Deaminase