Browsing by Author "Katugaha, N."

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Cardiac functions in older patients with haemoglobin E- β thalassaemia
(Sri Lanka Medical Association, 2007) Premawardhena, A.P.; Wanninayake, S.; Dolapihilla, S.N.; Kapuruge, L.; Katugaha, N.; Olivieri, N.F.; Weatherall, D.J.
INTRODUCTION: Cardiac disease accounts for most deaths in patients with thalassaemia. Little is known about cardiac functions in ageing patients with thalassaemia OBJECTIVE: To study cardiac functions in older patients with haemoglobin E - p thalassaemia. DESIGN, SETTING AND METHODS: All patients with haemoglobin E - p thaiassaemia over the age of 24 attending the Thalassaemia Centre at Kurunegala were studied. Data were collected on biographs, transfusion load, body iron loads, ischaemic heart disease risk factors and symptoms and signs of heart disease. Serum lipids, 2D-echo and exercise ECG in addition to basic biochemical investigations were assessed in all. Those with a positive exercise ECG underwent stress echocardiography. Data from age-sex matched "healthy" individuals from the same geographic area were used for comparison. RESULTS: Total of 26 patients and 26 controls were studied. The median age was 34.25 years (24-50). Fourteen females were present in either group. 24% of controls, but-none in thalassaemic families had a family history of ischaemic heart disease. Twelve patients with thalassaemia and one control had a positive exercise ECG. All 12 with positive exercise ECG had normal stress echocardiography. Severe hypocholesterolaemia (mean total cholesterol 90.5 vs 376.9mg/dl) was present in all patients with thalassaemia. Pulmonary hypertension was noted in 7 patients with thalassaemia (not in controls). Nine patients with thalassaemia but none of the controls showed diastolic dysfunction. CONCLUSION: The study shows unique cardiac abnormalities that occur in patients with haemoglobin E- β thalassaemia. The marked hypocholesterolaemia may reduce the risk of ischaemic heart disease but significant right heart damage seems to occur in these chronically anaemic patients.
Is the beta thalassaemia trait of clinical importance?
(Wiley-Blackwell, 2008) Premawardhena, A.; Arambepola, M.; Katugaha, N.; Weatherall, D. J.
Although the beta thalassaemia trait affects millions of people worldwide, there have been no controlled studies to determine whether it is associated with any clinical disability or abnormal physical signs. To address this question, 402 individuals were studied: 217 with beta thalassaemia trait, of whom 154 were aware of the diagnosis and 63 were unaware until after the completion of the study; 89 normal controls; and 96 controls with mild hypochromic anaemia. There was a significant increase in symptoms ascribable to anaemia and episodes of pyrexia in those with the beta thalassaemia trait that were not influenced by prior knowledge that they had this condition. There was no difference in physical findings, notably splenomegaly, between those with beta thalassaemia trait and either control group
Survival and complications in patients with haemoglobin E thalassaemia in Sri Lanka: a prospective, longitudinal cohort study.
(Elsevier Ltd, 2022) Premawardhena, A.P.; Ediriweera, D.S.; Sabouhanian, A.; Allen, A.; Rees, D.; de Silva, S.; Perera, W.; Katugaha, N.; Arambepola, M.; Yamashita, R.C.; Mettananda, S.; Jiffry, N.; Mehta, V.; Cader, R.; Bandara, D.; St Pierre, T.; Muraca, G.; Fisher, C.; Kirubarajan, A.; Khan, S.; Allen, S.; Lamabadusuriya, S.P.; Weatherall, D.J.; Olivieri, N.F.
Background: Worldwide, haemoglobin E β-thalassaemia is the most common genotype of severe β-thalassaemia. The paucity of long-term data for this form of thalassaemia makes evidence-based management challenging. We did a long-term observational study to define factors associated with survival and complications in patients with haemoglobin E thalassaemia. Methods: In this prospective, longitudinal cohort study, we included all patients with haemoglobin E thalassaemia who attended the National Thalassaemia Centre in Kurunegala, Sri Lanka, between Jan 1, 1997, and Dec 31, 2001. Patients were assessed up to three times a year. Approaches to blood transfusions, splenectomy, and chelation therapy shifted during this period. Survival rates between groups were evaluated using Kaplan-Meier survival function estimate curves and Cox proportional hazards models were used to identify risk factors for mortality. Findings: 109 patients (54 [50%] male; 55 [50%] female) were recruited and followed up for a median of 18 years (IQR 14-20). Median age at recruitment was 13 years (range 8-21). 32 (29%) patients died during follow-up. Median survival in all patients was 49 years (95% CI 45-not reached). Median survival was worse among male patients (hazard ratio [HR] 2·51, 95% CI 1·16-5·43), patients with a history of serious infections (adjusted HR 8·49, 2·90-24·84), and those with higher estimated body iron burdens as estimated by serum ferritin concentration (adjusted HR 1·03, 1·01-1·06 per 100 units). Splenectomy, while not associated with statistically significant increases in the risks of death or serious infections, ultimately did not eliminate a requirement for scheduled transfusions in 42 (58%) of 73 patients. Haemoglobin concentration less than or equal to 4·5 g/dL (vs concentration >4·5 g/dL), serum ferritin concentration more than 1300 μg/L (vs concentration ≤1300 μg/L), and liver iron concentration more than 5 mg/g dry weight of liver (vs concentration ≤5 mg/g) were associated with poorer survival. Interpretation: Patients with haemoglobin E thalassaemia often had complications and shortened survival compared with that reported in high-resource countries for thalassaemia major and for thalassaemia intermedia not involving an allele for haemoglobin E. Approaches to management in this disorder remain uncertain and prospective studies should evaluate if altered transfusion regimens, with improved control of body iron, can improve survival.
Symptoms and signs of β thalassaemia trait: results of the first comparative study
(Sri Lanka Medical Association, 2007) Premawardhena, A.P.; Weerasinghe, M.; Kottachchi, D.; Arambepola, M.; Katugaha, N.; Samarakoon, S.; Otivieri, N.F.; Weatherall, D.J.
OBJECTIVE: Thalassaemia trait is considered a mild and asymptomatic condition. However patients with this disease may be symptomatic. Should these be attributed to the disease? Does pre-knowledge about the diagnosis affect patients' symptoms? Previous studies were affected by the lack of a control arm. This is the first controlled study which compares symptoms of (3 thalassaemia trait with matched controls and also looks at how prior knowledge of the disease affects symptoms. DESIGN, SETTING AND METHODS: We administered a questionnaire to 146 individuals who knew that they had thalasseamia trait (Group 1} and to 248 "normal" volunteers who did not know their "thalassaemic status". Eleven symptoms were assessed. All individuals were examined by the same investigator and had a full blood count from an automated analyzer and a thalassaemia screening with High Performance Liquid Chromatography (HPLC- Bio Rad). RESULTS: The FBC and HPLC data showed that of the 248 "normal" controls , 63 had p thalassaemia trait (Group 2), and a further 96 had MCV< 80 and MCH <27 without thalassaemia (Group 3), presumably due to iron deficiency. 89 had normal red cell indices and normal HPLC (Group 4). Comparison of the four groups showed that "anaemic symptoms" like headache, exercise intolerance and lethargy occurred in significantly higher numbers in Groups 1 and 2 compared to Group 4. Comparison of Group 1 and Group 3 did not show any significant difference suggesting a similar mechanism for symptoms. Group 2 did not differ significantly from Group 1 in anyway. CONCLUSIONS: The p thalasseamia trait can be a symptomatic disorder and the symptoms arc those of anaemia. Symptoms are not affected or caused by being aware of the diagnosis.