A clinical audit of thalassaemia management at the Lady Ridgeway Hospital for Children, Colombo

Samarakoon, P S; Wijesuriya, A P

doi:10.4038/sljch.v40i2.3090

Cited by 7 publications

(7 citation statements)

References 12 publications

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“…Hence, nutritional care is very important in the management of thalassemia. The mean number of blood transfusions in a Sri Lankan study was 10.4 in the past year which was similar to our observations [18]. In the study done at Ahmedabad, India, the frequency of transfusion was once or more in a month among 25.6% cases which were also similar to our findings [19].…”

Section: Original Research Articlesupporting

confidence: 90%

Clinico-epidemiological profile of thalassemia patients in a tertiary care center

Singh

Dayal

et al. 2019

Int J Pediatr Res

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Introduction: Thalassemias are inherited blood disorders characterized by abnormal hemoglobin production. There are two main types, alpha thalassemia, and beta-thalassemia. Beta Thalassemia Major (also called Cooley's Anemia) is a serious illness. Symptoms appear in the first two years of life and include paleness of the skin, poor appetite, irritability, and failure to grow. There is absent or decreased production of normal hemoglobin, resulting in microcytic anemia of varying degrees. The thalassemias have a distribution concomitant with areas where P. falciparum malaria is common. The alpha thalassemias are concentrated in Southeast Asia, Malaysia, and southern China. The beta thalassemias are seen primarily in the areas surrounding the Mediterranean Sea, Africa, and Southeast Asia. Proper treatment includes routine blood transfusions and other therapies which can lead to iron overload and contraction of infections such as HIV, HCV, and HBV shortening the life span of patients. On the other hand, inadequate transfusions may lead to severe anemia and debility. Material and Methods: This retrospective record-based cross-sectional study was conducted in S.N. Medical College, Agra. Results: In our tertiary center, we have 103 registered patients of thalassemia, in which 44 are females and 59 are males. Out of the 103 thalassemia cases, 95 (92.2%) belonged to beta-thalassemia major, 4(3.8%) of betathalassemia intermediate, and 1 (0.9%) of beta-thalassemia minor category. Fever was the most common presenting symptom. Pallor and hepatomegaly were the most common signs among cases. Conclusion: Hemoglobinopathies are the commonest hereditary disorders in India and pose a major health problem. The data on the prevalence of β-thalassemias and other hemoglobinopathies in different caste/ethnic groups of India is scarce.

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Section: Original Research Articlesupporting

confidence: 90%

Clinico-epidemiological profile of thalassemia patients in a tertiary care center

Singh

Dayal

et al. 2019

Int J Pediatr Res

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“…It revealed that these factors do not have any impact on the HRQoL of children with thalassemia similar to previous studies [11,17]. Further, all these patients were basically managed by the government sector hospitals where the blood transfusion facilities are available [8]. Therefore, this is a mark of the improvement of quality of care given to the thalassemia patients in Sri Lanka during the last few decades.…”

Section: Discussionsupporting

confidence: 73%

“…These patients are basically managed by government hospitals, which have safe blood transfusion facilities. The main thalassemia center of Sri Lanka is located in Kurunegala and it is supported and guided by a team of international experts under the supervision of the Ministry of Health [8].…”

Section: Introductionmentioning

confidence: 99%

Health Related Quality of Life of Children with Thalassemia Major in Two Selected Hospitals in Sri Lanka

Silva

Peiris

2020

jhsir

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Introduction: Thalassemia is an inherited hemoglobin disorder. There are two types; thalassemia major and minor. Thalassemia major directly affects children’s physical, emotional, social and school functions. Health Related Quality of Life (HRQoL) is an important indicator to assess the health of human. Methods: A cross sectional study was conducted using 60 children (age 2-12 years) with thalassemia major who attended clinics in two selected hospitals in Sri Lanka from July to September 2018. Demographic data and risk factors were collected using a self-administered questionnaire. HRQoL data were collected using the Pediatric Quality of Life Inventory Version 4.0 Generic Core Scale. Data were analyzed using SPSS. Results: Mean values of the total functioning scores of physical, emotional, social and school were 89.42 (±9.20), 87.83 (±10.43), 95.00 (±7.53) and 78.75 (±14.13) respectively. Age of the children was significantly associated with emotional functioning (p=0.046), school functioning (p=0.023), psychosocial health (p=0.021) and the total summary score (p=0.006). Total number of blood transfusions per year of the patient was significantly associated with physical functioning (p=0.002), psychosocial health (p=0.045) and total summary score (p=0.034). Conclusions: Age and the total number of blood transfusions per year significantly affect the HRQoL of children with thalassemia major. Gender, type of iron chelation and age at first blood transfusion did not affect HRQoL of the children.Keywords: HRQoL, Thalassemia major, Haemoglobin disorder

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“…Similarly, two recent studies done in India [ 17 ] and Pakistan [ 18 ] reported very high mean serum ferritin values, 2767 and 4236 µg/l, respectively among patients with thalassaemia. An audit done in 2010 in another centre of Sri Lanka noted that almost 50% of patients with β-thalassaemia major had serum ferritin above 2500 µg/l [ 19 ]. This was much higher than what we report; in our cohort only 20% had serum ferritin above 2500 µg/l.…”

Section: Main Textmentioning

confidence: 99%

“…None of the patients had hypothyroidism, hypoparathyroidism or diabetes mellitus. Conversely a Sri Lankan study done among 61 patients few years back reported 7 patients with cardiac failure, 2 with diabetes mellitus, 1 with hypoparathyroidism [ 19 ]. Hepatic alanine aminotransferase were elevated in 13 (24%) of our patients.…”

Section: Main Textmentioning

confidence: 99%

Body iron status of children and adolescents with transfusion dependent β-thalassaemia: trends of serum ferritin and associations of optimal body iron control

et al. 2018

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ObjectiveThis cross sectional study aims to describe the body iron status, trends of serum ferritin and associations of optimal body iron control in patients aged below 16 years with transfusion dependent β-thalassaemia attending Paediatric and Adolescent Thalassaemia Centres of the Colombo North Teaching Hospital of Sri Lanka.ResultsOut of 54 children, 51% were males and a majority were aged 11–16 years; 83% had β-thalassaemia major while 13% had HbE β-thalassaemia. Mean serum ferritin was 1778(± 1458) µg/l and 29% had optimal serum ferritin (below 1000 µg/l). Trend of mean serum ferritin over time showed gradual decline between 2011 and 2017 and longitudinal trend of individual patients at yearly intervals showed gradual rise until 5 years of age and plateauing thereafter. All except two patients were receiving iron chelator medication of which the most commonly used was oral deferasirox (92%). The most common iron-related complications were short stature (24.1%) and pubertal delay (42.8% of > 14 years). None of the patients had hypothyroidism, hypoparathyroidism or diabetes. Optimal serum ferritin levels were significantly associated with the diagnosis of thalassaemia at a later age (23.6 vs 9.0 months) and higher family income (OR-4.81;95%CI 1.17–19.67) however was not associated with the age of the patient or duration of transfusion.Electronic supplementary materialThe online version of this article (10.1186/s13104-018-3634-9) contains supplementary material, which is available to authorized users.

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A clinical audit of thalassaemia management at the Lady Ridgeway Hospital for Children, Colombo

Abstract: Objective: To assess the adherence of management of beta thalassaemia major patients at Lady Ridgeway Hospital to an established standard treatment protocol

Cited by 7 publications

References 12 publications

Clinico-epidemiological profile of thalassemia patients in a tertiary care center

Clinico-epidemiological profile of thalassemia patients in a tertiary care center

Health Related Quality of Life of Children with Thalassemia Major in Two Selected Hospitals in Sri Lanka

Body iron status of children and adolescents with transfusion dependent β-thalassaemia: trends of serum ferritin and associations of optimal body iron control

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