Testosterone deficiency in the adult males

Testosterone deficiency leads to multiple problems but can be difficult to diagnose. However, replacement therapy can be rewarding and a life changer for the patient.peer-reviewe

A case of accidental Ricin poisoning

A case report of accidental, mild ricin poisoning by ingestion of ten Castor beans by a 70 year old man. The clinical presentation is followed by a discussion about the toxicity of ricin, its use and misuse and the contrast between the toxicity of pure ricin and the ingestion of castor beans. Management of castor bean poisoning is also discussed.peer-reviewe

Dopa-responsive dystonia and hyperprolactinaemia : a novel association in two sisters

Dopa-Responsive Dystonia (DRD) is a rare hereditary condition of childhood-onset dystonia which responds dramatically to treatment with levodopa. It was first described in 1971 as a "hereditary progressive basal ganglia disease with marked diurnal fluctuation". We describe a novel association between DRD and hyperprolactinaemia occurring in two sisters at the onset of puberty.peer-reviewe

Charcot neuroarthropathy : pathogenesis, diagnosis and medical management

Charcot neuroarthropathy (CN) is a progressive degenerative arthropathy which rarely complicates diabetes mellitus. Most commonly, though not exclusively affecting the foot, it seems to be determined by the interaction of neuropathy, osteopaenia and proinflammatory cytokines on a calcified peripheral vasculature that maintains its ability to vasodilate despite widespread arteriosclerosis. Although often unrecalled, this arthropathy is probably triggered by trauma. Diagnosis is essentially clinical, given the paucity and non-specificity of radiological and biochemical findings at the acute stage. CN should be considered in the differential diagnosis of any diabetic patient presenting with a warm swollen lower extremity. Bone turnover markers, magnetic resonance imaging and radioisotope scanning may be useful diagnostic aids. Offloading is essential and improves limb survival. There is considerable interest, though limited data, on the benefits of bisphosphonates and calcitonin. The possible roles of ultrasound and radiotherapy need to be assessed in larger trials. Failure to institute corrective measures at an early stage results in a foot that is prone to deformity, ulceration, amputation and loss of function. It is hoped that a better understanding of the aetiopathogenesis at a cytokine level will allow the targeting of new effective agents.peer-reviewe

Remission during pregnancy of severe Chronic Hypertension due to 11-ß Hydroxylase Deficiency

When hypertension is a result of an underlying identifiable abnormality, the latter's early discovery can lead to a timely cure of the hypertension and the prevention of its complications. We present a case of high blood pressure lowered by pregnancy, suggestive of an endocrine cause. This was confirmed following a detailed history which revealed severe hypertension intractable to therapy, yet which remitted during pregnancy. A diagnosis of 11-beta hydroxylase deficiency was made consequent upon the finding of raised serum 11-desoxycorticosterone levels. The blood pressure was finally controlled with glucocorticoid replacement therapy and spironolactone.peer-reviewe

An unusual cause of shortness of breath and fever

A case of a 57 year old man who presented with shortness of breath and fever is discussed . The patient was suffering from multiple lung abscesses. As a right sided cardiac source of septic emboli was suspected, the patient underwent transthoracic echocardiography; this revealed a right atrial myxoma. The case, its complicated course and outcome are presented. This is followed by a discussion on infected atrial myxomas and the importance of early echocardiography in cases with a similar presentation.peer-reviewe

The changing role of the national audit office in public accountability

The purpose of the paper is to gain insight into the changing role of the National Audit Office (NAO) in public accountability with the backdrop of the legal amendments that instituted the Office. The paper seeks to analyse the strength of the Office’s legal mandate, evaluate the understandability of NAO reports as well as discuss the Office’s bearing on public policy. The discussion draws upon semi-structured interviews conducted with three NAO Officers and thirteen main users of NAO reports, consisting of Public Accounts Committee (PAC) Members, Opinion Makers and Public Officials. The analysis takes stock of relevant PAC transcripts and of visits made to PAC meetings.peer-reviewe

Presentation and management of diabetic ketoacidosis in adults in Malta

Aim: The aim of this audit was to assess adherence to local guideline in the management of Diabetic Ketoacidosis (DKA). Method: Patients admitted with DKA between April 2013 and March 2015 were identified and data was retrospectively collected from patients’ confidential files and Isoft®. Data collected included initial parameters recorded and biochemical investigations taken (initial and subsequent assessment of pH, HCO3-, blood glucose, potassium levels and urinary ketones), insulin regime started and intravenous fluid administered. Results: During the established time period 40 cases of DKA were identified in 18 patients. Median age was 33 years with a female preponderance of 60%. Six patients had newly diagnosed diabetes mellitus while 8 patients had more than one admission of DKA. All cases had capillary blood glucose monitoring (BGM) and/or venous random blood (plasma) glucose (RBG) checked and pH and HCO3- recorded on admission. 0.9% sodium chloride was the intravenous fluid started in all cases (as recommended by the guideline) and a median of 6.75L was prescribed during the first 24 hours. The median time spent on intravenous insulin infusion was 42.7 hours while the median time to pH >7.30, HCO3- >15mmol/L and negligible urinary ketones were 6.88, 12.83 and 34.5 hours respectively. Subcutaneous insulin was started at a median time of 48.21 hours from initiation of DKA protocol. Conclusion: This audit showed good adherence to local guideline. The great discrepancy between the time to pH >7.3 and the time to negligible urinary ketones highlights the need to introduce tools to measure systemic ketone production in the management of DKA with an update in the current local clinical practice guideline.peer-reviewe

Design activity in Malta : a macro-economic analysis

The present economic analysis focuses on design and innovation. It compares Malta to leading design innovators in the EU, namely Germany, Italy, Sweden and Finland. Findings include positioning of Malta in relation to these focus countries, overall, by firm size and by sector. Furthermore, manufacturing fares timidly in design innovation both internally in relation to other sectors, and also when compared to other EU Member States. Within services, IT and consultancy are relatively high design innovation spenders, whereas financial services come in at the lower end. The analysis also evaluates the multiplier effects of design services and the inter-industry linkages involved in design activity.peer-reviewe

Generalized gangliosidosis in Malta

The purpose of this paper is to describe the clinical features of four cases of generalized gangliosidosis, two of which in siblings. In two of the cases, and in one of the siblings, B-galactosidase deficiency was demonstrated in blood and urine samples. The point that emerges from these cases is the realization that the clinical distinction between the various ganglioside lipidoses is now no 'longer possible, even in the clinically 'typical' case the resemblance to one or other of the classical Tay-Sachs or Niemann Pick is meaningless without the aid of specialized enzymatic tests. Moreover, the dramatic finding of the cherry-red spots in the retina is now no longer pathognomonic of classical Tay-Sachs disease, as was formerly widely held by clinicians. In every case the exact metabolic defect should be pin-pointed before a definite diagnosis can be made. It therefore becomes mandatory to investigate all patients in this group by special chemical techniques.peer-reviewe