• Maintenance immunosuppression with intermittent intravenous IL-2 receptor antibody therapy in renal transplant recipients.

      Gabardi, Steven; Catella, Jennifer; Martin, Spencer T; Perrone, Ronald; Chandraker, Anil; Magee, Colm C; McDevitt-Potter, Lisa M; Abdominal Organ Transplant Clinical Specialist–Departments of Transplant Surgery and Pharmacy Services, and the Renal Division, Brigham and Women's Hospital, Boston, MA, USA. sgabardi@partners.org (2011-09)
      To report what we believe to be the first 2 cases of long-term (>24 months) intermittent intravenous interleukin-2 receptor antibody (IL-2RA) therapy for maintenance immunosuppression following renal transplantation.
    • The major cause of multiple sclerosis is environmental: genetics has a minor role--commentary.

      Hutchinson, Michael; St Vincent's University Hospital. mhutchin@iol.ie (2012-02-01)
    • Major cost savings associated with biologic dose reduction in patients with inflammatory arthritis.

      Murphy, C L; Awan, S; Sullivan, M O; Chavrimootoo, S; Bannon, C; Martin, L; Duffy, T; Murphy, E; Barry, M (Irish Medical Journal, 2015-01)
      The purpose of this study was to explore whether patients with Inflammatory Arthritis (IA) (Rheumatoid Arthritis (RA), Psoriatic Arthritis (PsA) or Ankylosing Spondylitis (AS)) would remain in remission following a reduction in biologic dosing frequency and to calculate the cost savings associated with dose reduction. This prospective non-blinded non-randomised study commenced in 2010. Patients with Inflammatory Arthritis being treated with a biologic agent were screened for disease activity. A cohort of those in remission according to standardized disease activity indices (DAS28 < 2.6, BASDAI < 4) was offered a reduction in dosing frequency of two commonly used biologic therapies (etanercept 50 mg once per fortnight instead of weekly, adalimumab 40 mg once per month instead of fortnightly). Patients were assessed for disease activity at 3, 6, 12, 18 and 24 months following reduction in dosing frequency. Cost saving was calculated. 79 patients with inflammatory arthritis in remission were recruited. 57% had rheumatoid arthritis (n = 45), 13% psoriatic arthritis (n = 10) and 30% ankylosing spondylitis (n = 24). 57% (n = 45) were taking etanercept and 43% (n = 34) adalimumab. The percentage of patients in remission at 24 months was 56% (n = 44). This resulted in an actual saving to the state of approximately 600,000 euro over two years. This study demonstrates the reduction in biologic dosing frequency is feasible in Inflammatory Arthritis. There was a considerable cost saving at two years. The potential for major cost savings in biologic usage should be pursued further.
    • Male fertility in cystic fibrosis.

      Chotirmall, S H; Mann, A K; Branagan, P; O'Donohoe, C; Lyons, A M; Flynn, M G; Gunaratnam, C; O'Neill, S J; McElvaney, N G; Respiratory Research Division, Education & Research Centre, Beaumont Hospital, Dublin. schotirmall@rcsi.ie (2011-04-05)
      Infertility rates among males with cystic fibrosis (CF) approximate 97%. No information is currently available within Ireland determining an understanding of fertility issues and the best methods of information provision to this specialized group. This study aimed to determine understanding and preferred approaches to information provision on fertility issues to Irish CF males. A Descriptive Study utilizing prospective coded questionnaires was mailed to a male CF cohort (n=50). Sections included demographics, fertility knowledge & investigation. Response rate was 16/50 (32%). All were aware that CF affected their fertility. More than two-thirds (n=11) were able to provide explanations whilst only one-third (n=5) provided the correct explanation. Significant numbers stated thoughts of marriage and a future family. Half have discussed fertility with a healthcare professional (HCP). Mean age of discussion was 21.9 years. One third preferred an earlier discussion. The commonest first source for information was written material which was also the preferred source. Three-quarters requested further information preferring again, written material. Significant gaps in sex education of Irish CF males exist. Discussion should be initiated by HCPs and centre-directed written material devised to address deficiencies.
    • Male lupus: a diagnosis often delayed--a case series and review of the literature.

      Ambrose, N L; Kearns, G; Mohammad, A; Rheumatology Department, Beaumont Hospital, Dublin, Ireland. nambrose2001@yahoo.co.uk (2011-03)
      Systemic lupus erythematosus (SLE) is an auto-immune disease that is characterised by autoantibody production. Male lupus is rare, apart from at either end of the age spectrum.
    • Male lupus: a diagnosis often delayed--a case series and review of the literature.

      Ambrose, N L; Kearns, G; Mohammad, A; Rheumatology Department, Beaumont Hospital, Dublin, Ireland., nambrose2001@yahoo.co.uk (2012-02-01)
      INTRODUCTION: Systemic lupus erythematosus (SLE) is an auto-immune disease that is characterised by autoantibody production. Male lupus is rare, apart from at either end of the age spectrum. AIM: In this series, we review the histories of six male lupus patients attending our service. RESULTS: Our patients presented in middle age and tended to develop haematological abnormalities, renal involvement and neurological manifestations which preceded the onset of their skin and joint complaints. Our patients accrued damage rapidly and overall did badly. They tended to respond sub-optimally to standard treatments. These cases highlight the need an increased awareness that male SLE patients present with a wide variety of symptoms, and that they accrue damage quickly. There is a need for timely diagnosis and appropriate initiation of treatment. This may help avoid preventable organ damage and increase the survival of men with SLE.
    • Malformation risks of antiepileptic drug monotherapies in pregnancy: updated results from the UK and Ireland Epilepsy and Pregnancy Registers.

      Campbell, E; Kennedy, F; Russell, A; Smithson, W H; Parsons, L; Morrison, P J; Liggan, B; Irwin, B; Delanty, N; Hunt, S J; et al. (2014-09)
      Antiepileptic drug (AED) exposure during pregnancy increases the risk of major congenital malformations (MCMs). The magnitude of this risk varies by AED exposure. Here we provide updated results from the UK Epilepsy and Pregnancy Register of the risk of MCMs after monotherapy exposure to valproate, carbamazepine and lamotrigine.
    • Malignancy in scleroderma patients from south west England: a population-based cohort study.

      Siau, Keith; Laversuch, C J; Creamer, P; O'Rourke, K P; Department of Medicine, Great Western Hospital, Swindon, SN3 6BB, UK, keith@siau.org. (2010-01-08)
      The pathophysiological relationship between scleroderma and malignancy remains poorly understood. Although some previous studies have demonstrated an increased malignancy risk in patients with scleroderma, others have been inconclusive. We aimed to determine if patients with scleroderma had an increased risk of malignancy compared to an age- and sex-matched local South West England population, and if there were any important differences between scleroderma patients with and without malignancy. Methods of this study are as follows. Notes were obtained on all local scleroderma patients (n = 68) locally, and those diagnosed with malignancy verified by contacting each patient's general practitioner. Expected malignancy figures were obtained from age- and sex-stratified regional prevalence data provided by the South West Cancer Intelligence Service registry. Among the patients, 22.1% with scleroderma were identified with concurrent malignancy. Affected sites were of the breast (n = 5), haematological system (n = 5), skin (n = 4), and unknown primary (n = 1). Overall, malignancy risk was found to be increased in scleroderma (RR = 3.15, 95% CI 1.77-5.20, p = 0.01). In particular, this risk was the highest for haematological malignancies (RR = 18.5, 95% CI 6-43, p = 0.03), especially for non-Hodgkin's lymphoma (RR = 25.8, 95% CI 5-75, p = 0.10). The majority of patients (86.7%) developed malignancy after the onset of scleroderma (mean = 6.9 years). Age of >70 and patients with limited scleroderma were significant risk factors for a patient with scleroderma to have a concurrent malignancy; however, no increased risk was found in patients with any particular pattern of organ involvement, cytotoxic usage or serology. To conclude, in this small patient cohort, we have found that scleroderma is associated with an increased risk of malignancy. This risk is statistically significant in patients with limited scleroderma. Patients who are elderly and those with limited disease should be closely scrutinized at follow-up appointments.
    • Malignant melanoma and breast carcinoma: a bidirectional correlation.

      Ho, W L; Comber, H; Hill, A D K; Murphy, G M; Department of Dermatology, Beaumont Hospital, Dublin 9, Ireland, drwlho@yahoo.com. (2009-03-05)
      BACKGROUND: Epidemiologic and genetic studies have suggested a bidirectional association between breast carcinoma (BC) and malignant melanoma (MM). OBSERVATION: We present a series of patients with MM and BC detected in our department within a span of 6 months, raising concerns for the high associations between the two malignancies. This led us to match the concordance of the two tumours in the National Irish Cancer Registry. CONCLUSION: The national figures provide evidence of a link between BC and MM. We recommend increased awareness among clinicians leading to more detailed surveillance of both second primary tumours. All MM patients with a family history of BC should be referred to a breast clinic. Women above the age of 40 with MM should undergo annual mammography and those less than 40 may be better evaluated with a breast MRI. All breast cancer patients should be made aware of the significance of changing moles and those with suspicious lesions referred to a dermatologist for evaluation.
    • Malignant melanoma and breast carcinoma: a bidirectional correlation.

      Ho, W L; Comber, H; Hill, A D K; Murphy, G M; Department of Dermatology, Beaumont Hospital, Dublin 9, Ireland. drwlho@yahoo.com (2012-02-01)
      BACKGROUND: Epidemiologic and genetic studies have suggested a bidirectional association between breast carcinoma (BC) and malignant melanoma (MM). OBSERVATION: We present a series of patients with MM and BC detected in our department within a span of 6 months, raising concerns for the high associations between the two malignancies. This led us to match the concordance of the two tumours in the National Irish Cancer Registry. CONCLUSION: The national figures provide evidence of a link between BC and MM. We recommend increased awareness among clinicians leading to more detailed surveillance of both second primary tumours. All MM patients with a family history of BC should be referred to a breast clinic. Women above the age of 40 with MM should undergo annual mammography and those less than 40 may be better evaluated with a breast MRI. All breast cancer patients should be made aware of the significance of changing moles and those with suspicious lesions referred to a dermatologist for evaluation.
    • Malignant otitis externa: An Australian case series.

      Royal College of Surgeons Ireland, Otolaryngology, Beaumont Hospital, Dublin,, Ireland. (2012-02-01)
      OBJECTIVES: To establish a clinicopathological profile of malignant otitis externa (MOE) in an Australian tertiary referral institution. STUDY DESIGN: Retrospective cohort outcomes study. METHODS: 24 patients were identified with MOE between January 1998 and July 2007. Patients were classified into Radiological Grades I-IV. Laboratory investigations Including C-reactive protein (CRP), white cell count (WCC), glycosylated haemoglobin (HBA1c) and average glucose level over admission were recorded. RESULTS: Radiological Grade was significantly associated with duration of therapy (rank correlation 0.57, p = 0.004). CRP was a useful indicator confirming disease resolution. Diabetics with MOE had elevated average blood sugar levels during their Hospital admission (p < 0.001) and had poor overall glycaemic control represented by Elevated HBA1c scores (p < 0.001). CONCLUSIONS: Malignant otitis externa is a rare disease, which is best managed in a multidisciplinary team setting. This practical grading system can be used to predict the duration of therapy at time of diagnosis, which enables the efficient utilisation of Hospital resources. Poorly controlled diabetics are more susceptible to developing. MOE than diabetics with satisfactory glycaemic control and may represent a subgroup of more brittle diabetics. CRP combined with appropriate clinical and radiological investigations is useful in assessing disease resolution.
    • Malignant peripheral nerve sheath tumour of the bladder associated with neurofibromatosis I.

      O'Brien, Julie; Aherne, Susan; Buckley, Orla; Daly, Padraig; Torreggiani, William C; Departments of Radiology. (Canadian Urological Association journal = Journal de l'Association des urologues du Canada, 2008-12)
      Neurofibromatosis is a hamartomatous disorder of autonomic peripheral nerve sheaths associated with peripheral nerve sheath tumours. Most tumours are neurofibromas; however, the genitourinary system is rarely involved. We present a rare case of a nerve sheath tumour of the bladder in a young patient, which was discovered to be malignant.
    • Malignant Phenylketonuria (PKU) Due to Dihydropteridine Reductase (DHPR) Deficiency

      Ventzke, A; Hoffmann, J; Crushell, E; Monavari, A; Mayne, PD; Knerr, I (Irish Medical Journal, 2015-12)
      DHPR deficiency is a rare autosomal recessively inherited metabolic disorder of tetrahydrobiopterin (BH4) regeneration. Clinical symptoms may comprise microcephaly, developmental delay, ataxia and seizures. BH4 is the cofactor for the enzyme phenylalanine (Phe)hydroxylase (PAH), and for tryptophan and tyrosine hydroxylases, both of which are essential for serotonin and dopamine biosynthesis. We present four patients in two families who are being treated at the National Centre for Inherited Metabolic Disorders (NCIMD). All are members of the Irish Traveller population. We have identified a homozygous mutation, c.353C>T, in the DHPR (QDPR) gene which, to the best of our knowledge, has not been previously described. The mainstay of treatment is a life-long Phe-restricted diet together with supplementation of L-dopa and 5-hydroxy tryptophan (5-HT) and folinic acid. In Ireland, there is neurological comorbidity in our adult DHPR patients, although the overall outcome is satisfactory and one affected female has three healthy children.
    • Malignant priapism: a case report.

      Ellanti, P; Connolly, S S; McDermott, R; Crotty, P L; Grainger, R; c/o Marjorie White-Flynn, Department of Urology, Adelaide and Meath Incorporating National Children's Hospital, Tallaght, Dublin 24, Ireland. prasad.ellanti@gmail.com (Irish journal of medical science, 2011-12)
      Metastatic involvement of the penis is most commonly from a primary malignant genitourinary tumour. It is a rare phenomenon usually reflecting disseminated malignancy associated with a poor prognosis. Metastasis to the penis mimicking priapism is extremely rare, particularly in the absence of disseminated disease.
    • Mallet Injury Management in Ireland

      Minchin, P (British Journal of Hand Therapy, 2012-12-10)
    • Management of acute dislocation of the temporomandibular joint in dental practice.

      McGoldrick, David M; Stassen, Leo F A; Dublin Dental School and Hospital, Lincoln Place, Dublin 2, Ireland. (Irish Dental Association, 2010-12)
      Acute dislocation of the temporomandibular joint is a situation that, although rare, may present to the dentist in practice at any time. A number of activities, such as removal of a tooth, may cause dislocation. The event is painful and distressing for the patient, their family and the dental team. Prompt management minimises discomfort, distress and long-term morbidity to the patient. We describe the aetiology of acute dislocation and outline a number of techniques that will aid the clinican in dealing with this event.
    • Management of acute diverticulitis: is less more?

      Hogan, Aisling; Winter, Des; Institute for Clinical Outcomes Research and Education, St. Vincent's University , Hospital, Dublin, Ireland. aislinghogan@yahoo.com (2012-02-01)
    • The management of amyotrophic lateral sclerosis.

      Phukan, Julie; Hardiman, Orla; Dept. of Neurology, Beaumont Hospital, and Trinity College Institute of Neuroscience, Dublin 9, Ireland. (2009-02)
      The terms amyotrophic lateral sclerosis (ALS) or motor neuron disease (MND) refer to a condition characterized by motor system degeneration with relative preservation of other pathways. Although there have been advances in symptomatic treatment, ALS remains an incurable condition. Advances in ALS management prolong survival but simultaneously raise challenging ethical dilemmas for physicians, patients and their families. Here, we review current practice in the management of ALS including pharmacological treatment, nutritional management, respiratory care, and evolving strategies in the management of cognitive impairment.
    • Management of an unerupted canine associated with a central giant cell granuloma.

      Leonard, T J; McNamara, C; McNamara, C M; Eastern Health Board, Regional Orthodontic Department, St. James's Hospital, Dublin 8, Ireland. (2003-02)