Fanconi anaemia with bilateral diffuse pulmonary arterio venous fistulae: a case report

<p>Abstract</p> <p>Background</p> <p>We report a patient with cytogenetically confirmed Fanconi anaemia with associated diffuse bilateral pulmonary arterio-venous fistulae. This is only the second reported case of diffuse pulmonary arterio-venous fistulae with Fanconi anaemia.</p> <p>Case Presentation</p> <p>A 16 year old Sri Lankan boy, with a cytogenetically confirmed Fanconi anaemia was admitted to University Medical Unit, National Hospital of Sri Lanka for further assessment and treatment. Both central and peripheral cyanosis plus clubbing were noted on examination. The peripheral saturation was persistently low on room air and did not improve with supplementary Oxygen. Contrast echocardiography failed to demonstrate an intra cardiac shunt but showed early crossover of contrast, suggesting the possibility of pulmonary arterio-venous fistulae. Computed tomography pulmonary angiogram was inconclusive. Subsequent right heart catheterisation revealed bilateral diffuse arterio-venous fistulae not amenable for device closure or surgical intervention.</p> <p>Conclusion</p> <p>To our knowledge, this is the second reported patient with diffuse pulmonary arterio-venous fistulae associated with Fanconi anaemia. We report this case to create awareness among clinicians regarding this elusive association. We recommend screening patients with Fanconi anaemia using contrast echocardiography at the time of assessment with transthoracic echocardiogram. Though universal screening may be impossible given the cost constraints, such screening should at least be performed in patients with clinical evidence of desaturation or when a therapeutic option such as haematopoietic stem cell transplantation is considered. Treatment of pulmonary arteriovenous fistulae would improve patient outcome as desaturation by shunting worsens the anaemic symptoms by reducing the oxygen carrying capacity of blood.</p

Sublingual nitroglycerin for early blood pressure control in hypertensive emergencies: observations from an emergency department clinical audit in Sri Lanka

Abstract Objective Hypertensive emergencies are potentially life threatening and require prompt blood pressure control with intravenous agents. Preparation of intravenous infusions is time consuming. Usefulness of sublingual nitroglycerin in this setting is not known. We aimed to assess the benefit of sublingual nitroglycerin as a bridge to IV therapy. In a clinical audit in an emergency department, patients presenting with hypertensive emergencies requiring intravenous nitroglycerin were administered single spray of sublingual nitroglycerin awaiting commencement of intravenous infusion. Blood pressure was monitored every 5 min to observe the degree and speed of reduction. Results Thirty-seven patients met the selection criteria. Mean age was 65.8 years (SD 7.04), and 29 were males (88.4%). Mean values of systolic, diastolic and mean blood pressures on admission were 217, 137, 163 mmHg. At 5 and 10 min after sublingual nitroglycerin, mean reduction of mean arterial blood pressure by 12.3 and 16.3% was achieved. Only 2 patients (5.4%) showed an overcorrection of blood pressure. Minimum of 15 min were required to set up a nitroglycerin intravenous infusion. Sublingual nitroglycerin spray allows rapid blood pressure control in hypertensive emergencies and is a useful bridge during the time to prepare intravenous infusion

Cardiac involvement in dengue infection

Introduction: Dengue is endemic in the tropics, and complications involving organ systems are seen with varying incidence. Methodology: We performed a systematic review. MEDLINE, EMBASE, Scopus SciVerse, Google Scholar, and LILACS were searched for papers providing information on cardiac involvement in dengue. Results: Cardiac involvement is not uncommon in dengue infection and is often transient, but may be associated with significant morbidity and even mortality. Direct viral invasion, immune mechanisms, electrolyte imbalance, derangement of intracellular calcium ion storage, lactic acidosis, and ischemia due to hypotension all play a role in myocardial dysfunction. The manifestations of cardiac involvement include clinical, electrocardiographic, echocardiographic, cardiac enzyme, and histopathologic abnormalities. Echocardiography appears to be a useful tool for detecting myocardial involvement and should be performed in patients with electrocardiographic abnormalities or hemodynamic instability. Treatment is largely supportive, though there are some anecdotal reports of improvements with specific agents. Conclusions: Knowledge on cardiac manifestations in dengue is limited, and further studies are needed to establish the exact pathophysiology and role of specific agents in the prevention and treatment of cardiac complications in dengue.</jats:p

Knowledge of prescribed medication information among patients with limited English proficiency in Sri Lanka

Abstract Background Patients’ knowledge on prescribed medications play a key role in the long term management of cardiac diseases and in determining their outcome. The present study evaluates the knowledge about prescribed medication among cardiac patients and aim to identify factors influencing knowledge. Methods A descriptive-cross-sectional study was conducted among 200 adult patients attending clinics at the Cardiology Unit of the National Hospital of Sri Lanka. Knowledge assessment focused on four different sections; drug name, dose, frequency and indication. The total score of 60 was calculated by giving each component the following weighted scores; drug name = 20, indication = 20, drug dose = 10 and frequency = 10. A binary logistic regression analysis to evaluate factors associated with ‘good knowledge’ (total score ≥ 40) was performed. Results Among 200 participants 56.5% (n = 113) were males. Mean age was 59.7 ± 8.2 years and a majority (n = 170, 85.0%) were older than 50 years of age. Sinhala was the primary language of 91.5% (n = 183) of participants, while English was the primary language in only two of the study participants (1.0%). Eighty four percent of the participants were educated up to secondary education or above, while 2.5% (n = 5) had no formal education. The overall knowledge (total score-60) on prescribed medications among the study population was ‘poor’ (score ≤ 20) in 46%, ‘adequate’ (score 21–40) in 36.5% and ‘good’ (score ≥ 40) in 17.5%. The results of the binary logistic regression analysis indicates that Secondary (OR-1.53) and Tertiary levels (OR-2.79) of education, self-reported perception of illness as being Moderate (OR-1.23) or Severe (OR-1.70) and being educated by a doctor (as reported by patients) (OR-1.69) significantly increased the odds of having a ‘Good Knowledge of Drugs’. Majority of the patients were unable to read and understand the information written in English. The doctor’s contributed towards educating on drug information only in 33.0% of the patients. Conclusion In a resource-poor setting in patients with Limited English Proficiency, lower level of education and misperception of illness severity resulted in reduced knowledge on prescribed medications. Furthermore, being educated by a doctor significantly improved knowledge. However the doctors’ contribution at present to deliver quality health information to their patients was at an unsatisfactory level. </jats:sec

Patterns and correlates of adult height in Sri Lanka

The present study examines patterns and socioeconomic and demographic correlates of adult height among Sri Lankan adults. Data were available for height and socio-demographic factors from a nationally representative cross-sectional sample of 4477 subjects above 18 years. Recruitment was between 2005 and 2006. Mean age of all subjects was 46.1±15.1 years. Mean height of males and females were 163.6±6.9cm and 151.4±6.4cm respectively. Mean height showed a significant negative correlation with age (p<0.001, r=-0.207). Highest mean height in females 154.0±5.9cm and males 165.6±6.9cm were observed in those born after 1977. Rural females (151.4±6.2cm) were significantly taller than the urban (151.3±7.2cm). However, this was not observed in males. In multivariate analysis, year of birth, level of education and household income were significantly associated with height. Height demonstrated a significant negative correlation with systolic blood pressure (r=-0.032), presence of diabetes (r=-0.069), total cholesterol (r=-0.106), HDL cholesterol (r=-0.142) and LDL cholesterol (r=-0.104). Height was associated with household income and level of education in Sri Lanka and demonstrated a distinct increasing trend over successive generations

Knowledge of prescribed medication information among patients with limited English proficiency in Sri Lanka

Abstract Background Patients’ knowledge on prescribed medications play a key role in the long term management of cardiac diseases and in determining their outcome. The present study evaluates the knowledge about prescribed medication among cardiac patients and aim to identify factors influencing knowledge. Methods A descriptive-cross-sectional study was conducted among 200 adult patients attending clinics at the Cardiology Unit of the National Hospital of Sri Lanka. Knowledge assessment focused on four different sections; drug name, dose, frequency and indication. The total score of 60 was calculated by giving each component the following weighted scores; drug name = 20, indication = 20, drug dose = 10 and frequency = 10. A binary logistic regression analysis to evaluate factors associated with ‘good knowledge’ (total score ≥ 40) was performed. Results Among 200 participants 56.5% (n = 113) were males. Mean age was 59.7 ± 8.2 years and a majority (n = 170, 85.0%) were older than 50 years of age. Sinhala was the primary language of 91.5% (n = 183) of participants, while English was the primary language in only two of the study participants (1.0%). Eighty four percent of the participants were educated up to secondary education or above, while 2.5% (n = 5) had no formal education. The overall knowledge (total score-60) on prescribed medications among the study population was ‘poor’ (score ≤ 20) in 46%, ‘adequate’ (score 21–40) in 36.5% and ‘good’ (score ≥ 40) in 17.5%. The results of the binary logistic regression analysis indicates that Secondary (OR-1.53) and Tertiary levels (OR-2.79) of education, self-reported perception of illness as being Moderate (OR-1.23) or Severe (OR-1.70) and being educated by a doctor (as reported by patients) (OR-1.69) significantly increased the odds of having a ‘Good Knowledge of Drugs’. Majority of the patients were unable to read and understand the information written in English. The doctor’s contributed towards educating on drug information only in 33.0% of the patients. Conclusion In a resource-poor setting in patients with Limited English Proficiency, lower level of education and misperception of illness severity resulted in reduced knowledge on prescribed medications. Furthermore, being educated by a doctor significantly improved knowledge. However the doctors’ contribution at present to deliver quality health information to their patients was at an unsatisfactory level.</p