Coeliac crisis with severe hypokalaemia in an adult

Coeliac crisis is a rare life-threatening presentation of coeliac disease, in which acute dramatic metabolic derangements are present. It is observed mainly in children less than two years of age. In adults, coeliac disease usually has an indolent course and presents with mild gastrointestinal symptoms or may even be asymptomatic and present with long term complications including anaemia, osteoporosis and infertility. This case describes a 38 year old gentleman who presented with acute diarrhoea that led rapidly to severe metabolic disturbances including life threatening hypokalaemia. This case illustrates the heterogeneous clinical course of coeliac disease and the importance of considering it in the differential diagnosis of adult patients presenting with acute diarrhoea and metabolic disturbances.peer-reviewe

Factors related to fatigue in systemic lupus erythematosus : a cohort cross-sectional study

Background Fatigue is the most prevalent symptom in Systemic Lupus Erythematosus (SLE) as it is present in up to 90% of patients; it is considered to be the most disabling symptom in around half of the patients [1,2]. Its aetiology is multi-factorial and there is conflicting evidence on the relationship between fatigue and SLE disease activity, and between fatigue and vitamin D deficiency. The Ad Hoc Committee on Systemic Lupus Erythematosus Response Criteria for Fatigue recommended the Fatigue Severity Scale (FSS) for the measurement of fatigue in SLE [2,3].peer-reviewe

Translation and validation of the Fatigue Severity Scale, Pittsburgh Sleep Quality Index and Modified Health Assessment Questionnaire into the Maltese language, in a cohort of Maltese Systemic Lupus Erythematosus patients

PURPOSE: The assessment of fatigue, sleep quality and functional disability requires the use of validated instruments such as the Fatigue Severity Scale (FSS), the Pittsburgh Sleep Quality Index (PSQI) and the Modified Health Assessment Questionnaire (mHAQ) respectively. The aim of this study was to translate and validate these instruments into the Maltese Language. METHOD: Forward translation from the original English version into Maltese was carried out by two translators. The two versions were compiled to produce a preliminary initial Maltese translation. This was translated back into English by two other translators. This led to the development of the pre-final version of the Maltese translation, which was pilot-tested in 20 bilingual patients with systemic lupus erythematosus. RESULTS: Psychometric testing revealed good reliability of the Maltese translation of the three questionnaires. Cronbach’s alpha of the Maltese versions of the FSS, PSQI and mHAQ were 0.877, 0.859 and 0.897 respectively, showing good internal consistency. Validity of the Maltese version of the FSS was shown, since it had a significant positive correlation with visual analogue scale for fatigue (r=0.809, p<0.001). CONCLUSION: The Maltese translations were thus finalised, and could be used for clinical assessment or research purposes.peer-reviewe

Vitamin D : its role in the musculoskeletal system and beyond

Vitamin D deficiency has a high prevalence due to inadequate exposure to sunlight and its limited presence in foods. Vitamin D deficiency has well-known consequences on the musculoskeletal system, namely osteoporosis and frequent falls in the elderly, in view of its effect on calcium absorption. The discovery of the vitamin D receptor in many cells and its ability to regulate the transcription of over 200 genes, has created interest with regards to the role of vitamin D in the modulation of cell growth, inflammation and immune functions. Guidelines recommend screening individuals at risk of vitamin D deficiency, and supplementing when necessary.peer-reviewe

Cardiovascular risk assessment and management in rheumatoid arthritis : are guidelines being followed?

Aim: The aim of the audit was to determine whether the cardiovascular risk assessment and management in rheumatoid arthritis patients at Mater Dei Hospital is in concordance with the recommendations by the European League Against Rheumatism (EULAR). Background: Patients who suffer from rheumatoid arthritis have an increased risk of morbidity and mortality from cardiovascular disease. This is due to both the high prevalence of traditional risk factors, and systemic inflammation. Method: This audit was carried out retrospectively on 91 patients by using the medical notes to collect data on demographics, co-morbidities, drug history and cardiovascular risk assessment and management over a two year period (August 2010 to July 2012). The data was then analysed in order to assess whether the management of cardiovascular risk in rheumatoid arthritis patients was in concordance with EULAR recommendations. Results: Cardiovascular risk factors were documented as follows over the two year period audited: weight in 27.5%, BMI in 0%, smoking status in 72.5%, blood pressure in 72.5%, blood glucose in 72.5% and lipid profile in 54.9%. Smoking cessation advice was given in 15.8% and advice on other lifestyle changes in 14.3%. 81.1% of hypertensive patients were on treatment recommended as first-line by the guidelines and HbA1c was adequately controlled in 85.7% of diabetic patients in whom it was monitored. Conclusion: Cardiovascular risk factors are highly prevalent in rheumatoid arthritis patients. This audit identified aspects of cardiovascular risk assessment that require improvement. This would enable a better identification of cardiovascular risk factors that could be treated in order to reduce the patients’ cardiovascular morbidity and mortality.peer-reviewe

Vitamin D supplementation in systemic lupus erythematosus patients with vitamin D deficiency and insufficiency : the effect on disease activity, fatigue and interferon signature gene expression

Background: Vitamin D deficiency is highly prevalent among patients with systemic lupus erythematosus (SLE) [1]. Evidence from multiple studies has shown that vitamin D deficiency in SLE is associated with a higher disease activity [2]. There is conflicting evidence with regards to the relationship between fatigue and vitamin D level [3,4].peer-reviewe

Tenofovir as rescue therapy following clinical failure to lamivudine in severe acute hepatitis B

Acute hepatitis B (AHB) is a self-limiting condition in more than 95% of cases. Treatment is however recommended in patients with severe AHB (<1% of cases), aiming to prevent liver failure and death. Various nucleos(t)ide analogues (NA) have been found to be effective in severe AHB, although NA-resistant strains causing AHB have been also recently reported. The use of tenofovir in severe AHB has only been described in 3 cases (1 adult and 1 infant with HBV mono-infection, 1 adult with HBV/HIV co-infection). We hereby report a 47-year-old treatment-naïve male, who developed severe AHB and was initially treated with lamivudine (LMV). Initial rapid biochemical response was followed by biochemical breakthrough after 9 days, suggesting LMV resistance. Rescue therapy with ‘add-on’ tenofovir brought about a sustained improvement in biochemical, serological and virological markers until HBsAg was lost after 4 months. Thus, this is the second adult HBV mono-infected patient, who responded successfully to tenofovir in severe AHB.peer-reviewe

Health status of COPD patients undergoing pulmonary rehabilitation : a comparative responsiveness of the CAT and SGRQ

The authors would like to thank all the participants who participated in this study and the CEO of the state general hospital for his support.The St. George’s Respiratory Questionnaire (SGRQ) and chronic obstructive pulmonary disease (COPD) assessment test (CAT) are the measures used to assess health status. This study aims to examine the responsiveness of these tools by severity of dyspnoea category in patients with COPD. Forty-nine COPD patients who underwent a 12-week pulmonary rehabilitation (PR) programme were assessed at baseline, 12 weeks and at 28-week follow-up. Patients were categorized into two groups by severity of dyspnoea category (i.e. mild to moderate (modified Medical Research Council (mMRC) 1–2) and severe to very severe (mMRC 3–4)) using the mMRC dyspnoea scale. Effect size (ES) was computed as estimates of responsiveness. The SGRQ demonstrated greater responsiveness by total sample (SGRQ, ES = 0.87; CAT, ES = 0.75) and for the mMRC 3–4 category (SGRQ, ES = 0.91; CAT, ES = 0.76) on completion of PR. At 28-week follow-up, overall comparable responsiveness of the CAT and SGRQ was identified by total sample (SGRQ, ES = 0.75; CAT, ES = 0.74) and by severity of dyspnoea category. The symptom, impact and activity domains of the SGRQ showed good responsiveness, with greater ESs obtained overall for the mMRC 3–4 category. On completion of PR, the SGRQ demonstrates a greater responsiveness with COPD patients, especially in relation to the mMRC 3–4 category, while both the CAT and SGRQ show comparable responsiveness on follow-up.peer-reviewe

Vitamin D and interferon signature gene expression in systemic lupus erythematosus : a cross-sectional cohort study

Background: Vitamin D deficiency is more prevalent in patients with systemic lupus eythematosus (SLE) as a result of sun avoidance. The potential negative impact of vitamin D deficiency on the disease activity of SLE has been shown in a number of studies. The expression of the interferon signature genes in SLE correlates positively with disease activity, and these genes are thought to mediate the clinical manifestations of the disease. The aim of this study was to establish whether a relationship exists between serum 25-hydroxyvitamin D level and the interferon signature gene expression in whole blood of SLE patients.peer-reviewe

Characterisation of Patients with Systemic Lupus Erythematosus in Malta: A Population Based Cohort Cross-Sectional Study

Systemic Lupus Erythematosus (SLE) is a multisystemic autoimmune disorder. The aim of this study was to characterise the SLE patients living in Malta in order to estimate the prevalence and incidence of SLE and characterise the clinical presentation as well as identify any unmet needs. 107 SLE patients who fulfilled SLICC classification criteria were identified. These were invited to participate in the study by means of an interview, blood and urine tests, and filling of the following questionnaires: Fatigue Severity Scale (FSS), visual analogue scale (VAS) for fatigue, Hospital Anxiety and Depression Scale (HADS), VAS for pain, Pittsburgh Sleep Quality Index (PSQI), and modified Health Assessment Questionnaire (mHAQ). The estimated prevalence of SLE in Malta is 29.3 patients per 100,000 and the estimated incidence is 1.48 per 100,000 per year. 93.5% of SLE patients were female, and the mean age at diagnosis was 33.1 years. 60.8% were overweight or obese and body mass index (BMI) had a significant positive correlation with daily dose of prednisolone (R=0.177, p=0.046). 20.7% and 3.3% had a moderate and high disease activity, respectively, as measured by SLEDAI-2K. Disease activity had a significant positive correlation with functional disability measured by mHAQ (R=0.417, p3.7) and 57.6% had a high level of anxiety (HADS ≥8). This study has identified a number of unmet needs of SLE patients, including obesity, uncontrolled disease activity, fatigue, and anxiety