Fracture liaison services in Ireland-how do we compare to international standards?

In this first na tional survey of public hospitals in The Republic of Ireland, we found fracture liaison services (FLS) to be heterogeneous, limited in many cases and poorly supported. A national strategy is urgently needed to support the implementation and operation of an FLS, and thus help reduce the burden of fragility fractures for patients and the healthcare system. INTRODUCTION: Fragility/low-trauma fractures are a global concern, whose incidence is rising as the population ages. Many are preventable, and people with a prior fragility fracture are at particularly high risk of further fractures. This patient group is the target of the International Osteoporosis Foundation (IOF) Capture the Fracture campaign, advocating global adoption of fracture liaison services (FLS), with the aim of preventing secondary fragility fractures. We wished to determine the current availability and standards of an FLS in Ireland, ahead of the launch of a National FLS database. METHODS: We devised a questionnaire encompassing the thirteen IOF standards for an FLS and asked all 16 public hospitals with an orthopaedic trauma unit in Ireland, to complete for the calendar year 2019 in patients aged ≥ 50 years. RESULTS: All sites returned the questionnaire, i.e. 100% response rate. Nine hospitals stated that they have an FLS, additionally one non-trauma hospital running a FLS responded, and were included. These 10 FLS had identified and managed 3444 non-hip fractures in the year 2019. This figure represents 19% of the expected non-hip fragility fracture numbers occurring annually in Ireland. Implementation of the IOF standards was very variable. All sites reported being inadequately resourced to provide a high-quality service necessary to be effective. CONCLUSION: The existence and functioning of FLS in Ireland are heterogeneous and suboptimal. A national policy to support the implementation of this programme in line with international standards of patient care is urgently needed.

Investment in epilepsy monitoring units improves epilepsy care-experience in a regional neuroscience centre.

An evaluation of the clinical yield of inpatient long-term video-EEG (vEEG) in a new epilepsy monitoring unit (EMU) was undertaken, with findings compared to the centre's prior method of bedside vEEG recording in a standard neurology ward, as reported in 2004. A retrospective analysis of neurophysiology reports for all adults who underwent elective vEEG monitoring in the EMU at Cork University Hospital between January 2015 and July 2016 was conducted. Of 115 vEEG studies in the EMU, 100 (87.0%) were deemed diagnostically conclusive, 14 (12.2%) failed to catch any clinical events and showed normal EEG throughout, and one (0.9%) captured spells of unclear clinical significance - the corresponding figures reported in 2004 for bedside vEEGs were 21.3%, 77% and 1.6%, respectively. The EMU offers a more effective method of recording inpatient vEEG, which aids decision-making and improves clinical outcomes. Some evidence-based measures which could further enhance diagnostic yield are discussed.

Headache Management in Community Pharmacies.

Many headache patients self-medicate and choose their preferred analgesic from a range of acute analgesics available on the high-street and from community pharmacies. Little is known about their presenting symptoms, headache diagnosis and their preferred treatments. A questionnaire was distributed to community pharmacies in the Munster region of Ireland and was administered to patients requesting treatment for headache. A total of 1023 completed questionnaires were received, 76.7% (n=765) were female and 51.8% were aged 18-39 years. 53.3% (n=542) were not previously diagnosed by a GP and 49.6% (n=502) had never sought advice from a pharmacist. According to the symptoms described, 32% (n=327) had episodic migraine and a further 15.2% (n=155) had probable episodic migraine. 30.3% (n=310) had tension type headache. 10.7% (n=105) had chronic daily headache. Codeine based products were the preferred treatment choice for 43.1% (n=441). Triptans were the most effective, 68.6% (n=166), in those for whom they were previously prescribed. 11.8% (n=121) had medication-overuse. The community pharmacist is an important resource in headache management. An expanded role incorporating best practice management guidelines has the potential to improve the outcomes for many headache sufferers.

The Clinical Utility of a Low Serum Ceruloplasmin Measurement in the Diagnosis of Wilson Disease.

The first step in screening for potential Wilson disease is serum ceruloplasmin testing, whereby a level of less than 0.2g/L is suggestive of the disease. We aimed to determine what proportion of an Irish population had a low ceruloplasmin level, whether low measurements were appropriately followed-up and what were the clinical outcomes. We conducted a retrospective review of all serum ceruloplasmin measurements between August 2003 and October 2009 in a large tertiary referral centre in Southern Ireland. Clinical data, serum ceruloplasmin, liver function tests, urinary copper and liver biopsy reports were all recorded where available. 1573 patients had a serum ceruloplasmin measurement during the 7-year study period. 96 patients (6.1%) had a ceruloplasmin level < 0.2g/L and of these only 3 patients had Wilson disease. There was only 1 new diagnosis. Only 27 patients (28.1%) had some form of confirmatory testing performed. In our centre's experience, the positive predictive value of a significantly low ceruloplasmin level is 11.1% (95% CI 2.91-30.3%). In practice a low serum ceruloplasmin measurement is often not followed by appropriate confirmatory testing. Measuring serum ceruloplasmin as a singular diagnostic test for Wilson disease or as part of the battery of unselected liver screening tests is inappropriate and low-yield.

Investigation of an association between onset of narcolepsy and vaccination with pandemic influenza vaccine, Ireland April 2009-December 2010.

In 2011, the Irish Medicines Board received reports of onset of narcolepsy following vaccination against influenza A(H1N1)pdm09 with Pandemrix. A national steering committee was convened to examine the association between narcolepsy and pandemic vaccination. We conducted a retrospective population-based cohort study. Narcolepsy cases with onset from 1 April 2009 to 31 December 2010 were identified through active case finding. Narcolepsy history was gathered from medical records. Pandemic vaccination status was obtained from vaccination databases. Two independent experts classified cases using the Brighton case definition. Date of onset was defined as date of first healthcare contact for narcolepsy symptoms. Incidence of narcolepsy in vaccinated and non-vaccinated individuals was compared. Of 32 narcolepsy cases identified, 28 occurred in children/adolescents and for 24 first healthcare contact was between April 2009 and December 2010. Narcolepsy incidence was 5.7 (95% confidence interval (CI): 3.4­8.9) per 100,000 children/adolescents vaccinated with Pandemrix and 0.4 (95% CI: 0.1­1.0) per 100,000 unvaccinated children/adolescents (relative risk: 13.9; absolute attributable risk: 5.3 cases per 100,000 vaccinated children/adolescents). This study confirms the crude association between Pandemrix vaccination and narcolepsy as observed in Finland and Sweden. The vaccine is no longer in use in Ireland. Further studies are needed to explore the immunogenetic mechanism of narcolepsy.

A patient with a rare leukodystrophy related to lamin B1 duplication.

The hereditary leukodystrophies are rare disorders caused by molecular abnormalities leading to destruction of or failure of development of central white matter. For almost 30 years there has been increasing recognition of later onset Autosomal Dominant Leukodystrophy (ADLD). We report the first genetically confirmed case of lamin B1 duplication causing ADLD from Ireland.

Eating local: influences of habitat on the diet of little brown bats (Myotis lucifugus).

We employ molecular methods to profile the diet of the little brown bat, Myotis lucifugus, and describe spatial and temporal changes in diet over their maternity season. We identified 61 prey species of insects and 5 species of arachnid. The largest proportion of prey (∼32%) were identified as species of the mass-emerging Ephemeroptera (mayfly) genus Caenis. Bats roosting in agricultural settings had lower dietary richness than those occupying a roost located on a forest fragment in a conservation area. We detected temporal fluctuations in diet over the maternity season. Dipteran (fly) species dominated the diet early in the season, replaced later by species of mayfly. Because our methodology provides species-level identification of prey, we were able to isolate environmental indicator species in the diet and draw conclusions about the location and type of their foraging habitat and the health of these aquatic systems. The species detected suggested that the bats use variable habitats; members of one agricultural roost foraged on insects originating in rivers or streams while those in another agricultural roost and the forest roost fed on insects from pond or lake environments. All source water for prey was of fair to good quality, though no species detected are intolerant of pollution thus the habitat cannot be classified as pristine. Our study outlines a model system to investigate the abiotic and biotic interactions between habitat factors through this simple food chain to the top predator.

Is staging beneficial for Fowler-Stephens orchiopexy? A systematic review.

PURPOSE: Fowler and Stephens showed that by dividing the spermatic vessels a high intra-abdominal testis could be placed in the scrotum. Testicular atrophy is a potential complication of this technique. We conducted a systematic review to determine whether single or 2-stage Fowler-Stephens orchiopexy results in better testicular viability. MATERIALS AND METHODS: We searched electronic databases, clinical trial registries and gray literature. We included reports describing boys younger than 18 years with a primary outcome of "testicular viability and position." We performed a meta-analysis using random effects models. Heterogeneity was assessed using forest plot and I(2) statistic. RESULTS: We identified 1,807 citations and included 61 articles. Single stage Fowler-Stephens orchiopexy was discussed in 9 articles, a 2-stage procedure in 36 and both approaches in 16. There were no randomized controlled trials, and most studies were cohort or case series. The pooled estimate of success rates was 80% for single stage Fowler-Stephens orchiopexy (95% CI 75 to 86) and 85% for 2-stage Fowler-Stephens orchiopexy (95% CI 81 to 90). The pooled odds ratio of single stage vs 2-stage Fowler-Stephens orchiopexy was 2.0 (95% CI 1.1 to 3.9) favoring the 2-stage procedure. There was no difference in the success rate between laparoscopic and open techniques in either single or 2-stage Fowler-Stephens orchiopexy. There was no evidence of asymmetry on the funnel plot. There were no complications reported with single stage, while ileus, hematoma and infection were the most common complications with 2-stage Fowler-Stephens orchiopexy. CONCLUSIONS: Both techniques have a fairly high success rate but 2-stage Fowler-Stephens orchiopexy appears to carry a higher rate of success than the single stage approach (85% vs 80%, OR 2 in favor of 2-stage). Laparoscopic and open techniques had the same success rate. However, the level of evidence of the studies was low, and a study of a more robust design, such as a randomized controlled trial, should be performed.

Duration of weaning, starter intake, and weight gain of dairy calves fed large amounts of milk.

When calves are weaned abruptly off large amounts of milk, weight gain is reduced as a result of low intake of starter. We compared gradual and abrupt weaning of 40 calves allowed to drink up to 12kg of milk/d by automated feeders, housed in groups of 4, and weaned at 41 d abruptly or over 3 gradual weaning periods (4, 10, or 22 d), with one calf within each group randomly allocated to each treatment, balancing for sex and birth weight. During the milk-feeding period, the calves weaned over 22 d drank the least milk and ate the most starter, but these calves had the lowest total digestible energy intake and weight gains. The abruptly weaned calves had the highest digestible energy intakes and weight gains during the period before weaning. During the 9 d following weaning, the calves weaned over 22 and 10 d ate more starter and had better weight gains than abruptly weaned calves and those weaned over 4 d. Abruptly weaned calves lost weight during this period. In summary, gradual weaning improved starter intake, but because of reduced milk availability, this resulted in reduced total digestible energy intake before weaning. Weaning over 10 d resulted in the best overall weight gains over the study.

National narcolepsy survey.

Narcolepsy is characterised by excessive daytime sleepiness and cataplexy and has a prevalence of 25 per 100,000. We suspect this is higher than presently seen in the Republic of Ireland. We aimed to calculate the Irish prevalence of Narcolepsy and to examine current management practices. We conducted an online survey of respiratory physicians, neurologists, paediatric neurologists, and psychiatrists with an interest in sleep disorders (73% response rate). Of this group, a total of 16 physicians managed 180 patients prior to January 2009. A clinical diagnosis alone was reached in 67 (41%) patients, the remainder by polysomnography or multiple sleep latency testing. No patients were diagnosed by cerebro-spinal fluid analysis of hypocretin levels. While 70 (42%) patients received modafanil, only 7 (4%) were treated with sodium oxybate. Even allowing for missing data it is apparent that Narcolepsy is hugely under-diagnosed in Ireland, however, current practises adhere with new international guidelines.

Freeze-fracture studies of the thecal membranes of Gonyaulax polyedra: circadian changes in the particles of one membrane face.

Intramembrane faces were visualized in the marine dinoflagellate Gonyaulax polyedra by the freeze-fracture technique, in order to test a prediction of a membrane model for circadian oscillations--i.e;, that membrane particle distribution and size change with time in the circadian cycle. Cells from each of four cell suspensions in continuous light (500 1x, 20-21 degrees C) were frozen, without fixation or cryoprotection, at four circadian times in a cycle. This paper reports findings concerning the membranes associated with the theca, particularly the cytoplasmic membrane and the membrane of the large peripheral vesicle. While the number and size distribution of the particles of the PF face of the cytoplasmic membrane were constant with time, those of the EF face of the peripheral vesicle doubled in number at 18 h circadian time as compared with 06 h. Particles of the 120-A size class, in particular, were more numerous at 12 and 18 h circadian time than at 00 and 06 h. While the finding does not provide definitive confirmation of the membrane hypothesis for circadian rhythms, it is consistent with this model. It is suggested that the peripheral vesicle may be the site of bioluminescence in Gonyaulax.

Size variation and the distribution of hemimetabolous aquatic insects: two thermal equilibrium hypotheses.

Adult body size and fecundity of a number of hemimetabolous aquatic insects depend largely on thermal conditions during the larval period. Small adults and reduced fecundity result when temperatures are either warmed or cooled with respect to more optimal thermal conditions. Temperature apparently affects adult size by altering the larval growth rate and the timing and rate of adult tissue development for each larva. The data suggest a new interpretation for the geographic distribution of aquatic insects.

Pigment protein complex from gonyaulax.

A water-soluble peridinin-chlorophyll-protein complex from the dinoflagellate Gonyaulax polyedra was found to have a molecular weight of about 38,000. The complex could be disrupted by digestion with proteolytic enzymes. No electron transfer was observed when the complex was irradiated.

Smoking and anaesthesia: the pharmacological implications.

Anaesthetists are generally familiar with the peri-operative implications of cigarette smoking. Although there are a number of publications dealing with the wider pharmacological implications of cigarette smoking, the specific interactions which are of direct relevance to anaesthetists are less well known. This review gives an overview of those interactions which are of clinical relevance to anaesthetists and provides, where possible, an explanation of the mechanism. Recent improvements in the understanding of biotransformation of drugs, including streamlining of the classification of hepatic enzymes has allowed better understanding of drug interactions and enables the mechanistic prediction of those involving new drugs.

Botulism in injecting drug users, Dublin, Ireland, November-December 2008.

In November and December 2008, six cases of suspect wound botulism were reported in heroin injecting drug users, all residents in Dublin, Ireland. Patients were aged between 23-42 years of age; four cases were male; one patient died shortly after admission. The patients presented to four different hospitals across the city. Botulism in injecting drug users in Ireland was last reported in 2002.

Natalizumab induced cutaneous sarcoidosis-like reaction.

We present a case of a drug-induced sarcoidosis -like reaction (DISR) occurring following initiation of Natalizumab for multiple sclerosis. The reaction was purely cutaneous, and disappeared following drug withdrawal. We highlight this case to the practicing neurologists, with warning to be wary of a new rash on immunomodulatory therapies.

Aetiology and prognosis of encephalopathic patterns on electroencephalogram in a general hospital.

The purpose of this study was to investigate the frequency and clinical outcome of patients with encephalopathic electroencephalograms (EEGs) in a neurophysiology department based in a general hospital. We performed a retrospective review of all EEGs obtained during an 18-month period in a large tertiary referral hospital. The referral reasons for EEG, the diagnoses reached, and patient outcomes were reviewed according to EEG severity. One hundred and twenty-three patients with encephalopathic EEGs were reviewed. The most common referral reason found was for an assessment of a possible first-onset seizure. The most common diagnosis found was one of dementia or learning disability. Of patients who were followed-up for a median of 19 months, 20.7% had died. The mortality rate generally increased according to the severity of the encephalopathy on EEG. However, 21.4% of those patients with excessive theta activity only on EEG had died. This study highlights an increased mortality even in the apparently 'milder' degrees of EEG abnormalities.

Iatrogenic anterior diaphragmatic hernia in childhood.

Congenital anterior diaphragmatic hernias (ADH) account for 2 - 6 % of diaphragmatic defects, whereas acquired ADH are rare. These hernias are most often the result of blunt or penetrating trauma. This is the first report of iatrogenic ADH in childhood. Two children with asymptomatic, iatrogenic ADH were diagnosed at 6 and 12 months of age, respectively. Both had undergone previous cardiac surgery requiring pericardial drainage. The ADH was seen on an incidental chest X-ray in one patient, and during a laparoscopic-assisted gastrostomy in the other. Both were successfully repaired laparoscopically. Subxiphoid pericardial drains may result in iatrogenic ADH. A high index of suspicion after cardiac surgery is necessary to avoid missing this potentially serious complication. Laparoscopy is a useful tool in both the diagnosis and repair of this condition.

Neurology referrals to a liaison psychiatry service.

The objective of the present study was to assess the activity of the Liaison Psychiatry service of Cork University Hospital in relation to all in-patient neurology referrals over a 12-month period. Of 1685 neurology admissions, 106 (6%) were referred to liaison psychiatry for assessment. 91 referrals (86%) met criteria for a psychiatric disorder according to DSM-IV, the commonest being major depression (24%) and somatoform disorder (23%). Patients with multiple sclerosis or epilepsy comprised nearly half of all referrals (48 cases; 45%). Approximately 20% of M.S. in-patients (21 cases) were referred for psychiatric assessment, with the corresponding figure in epilepsy being 25% (18 cases). Although only 106 (6%) neurology in-patients were referred to liaison psychiatry, psychiatric diagnoses were documented in 327 (20%) discharge forms, presumably reflecting previous diagnosis. The above findings indicate that psychiatric illness is common among neurology inpatients screened by liaison psychiatry yet referral rates are relatively low in terms of the overall number of neurology in-patients. Psychiatric disorders were diagnosed in 86% of referrals indicating high concordance between neurologists and liaison psychiatry regarding the presence of a psychiatric disorder.