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Applicability of protocols from high-income countries in a resource limited setting; real world data of histopathology, clinical features and long-term outcome of Hodgkin Lymphoma in Sri Lanka.

ABSTRACT: Background:: There is a significant disparity in global cancer care and out-come between countries. We aimed to provide data on characteristics, average cost of treatment and survival estimates in patients with Hodgkin Lymphoma in Sri Lanka. Methods: All patients diagnosed with Hodgkin Lymphoma between 01.05.2013 and 01.10.2020 were included in the analysis. Findings: Classical Hodgkin Lymphoma(cHL) diagnosed in 85%; 68% presented with B symptoms and 61% had advanced stage of disease. Treatment was discontinued by 23% either before or just after starting treatment of whom 72% percent were females. The complete response (CR) rate of patients who continued treatment was 86% while the estimated five-year survival rate is 92%. Seventeen percent of these patients died but only two percent due to Hodgkin Lymphoma or associated treatment in the group which continued treatment compared to 45% in the group who defaulted treatment (p-value 0.0002). Five-year survival rate of patients who defaulted treatment was 50% while patients who continued treatment have an estimated five-year survival rate of 90%. Average cost of first line treatment was between US$ 2280 and US$ 7642. First treatment failure may incur substantially higher health care costs. Interpretation: This is the only well characterized study on long-term survival of patients with Hodgkin Lymphoma in Sri Lanka. We have shown that it is possible to successfully apply western treatment and supportive care protocols to the local population. This published data will help to bench mark and improve the treatment and develop blood cancer care in the local setting.

SUBMITTER: Hewamana S

PROVIDER: S-EPMC8267551 | biostudies-literature |

REPOSITORIES: biostudies-literature

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Project description:BackgroundThe phenotype of Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) patients is determined by the type of DMD gene variation, its location, effect on reading frame, and its size. The primary objective of this investigation was to determine the frequency and distribution of DMD gene variants (deletions/duplications) in Sri Lanka through the utilization of a combined approach involving multiplex polymerase chain reaction (mPCR) followed by Multiplex Ligation Dependent Probe Amplification (MLPA) and compare to the international literature. The current consensus is that MLPA is a labor efficient yet expensive technique for identifying deletions and duplications in the DMD gene.MethodologyGenetic analysis was performed in a cohort of 236 clinically suspected pediatric and adult myopathy patients in Sri Lanka, using mPCR and MLPA. A comparative analysis was conducted between our findings and literature data.ResultsIn the entire patient cohort (n = 236), mPCR solely was able to identify deletions in the DMD gene in 131/236 patients (DMD-120, BMD-11). In the same cohort, MLPA confirmed deletions in 149/236 patients [DMD-138, BMD -11]. These findings suggest that mPCR has a detection rate of 95% (131/138) among all patients who received a diagnosis. The distal and proximal deletion hotspots for DMD were exons 45-55 and 6-15. Exon 45-60 identified as a novel in-frame variation hotspot. Exon 45-59 was a hotspot for BMD deletions. Comparisons with the international literature show significant variations observed in deletion and duplication frequencies in DMD gene across different populations.ConclusionDMD gene deletions and duplications are concentrated in exons 45-55 and 2-20 respectively, which match global variation hotspots. Disparities in deletion and duplication frequencies were observed when comparing our data to other Asian and Western populations. Identified a 95% deletion detection rate for mPCR, making it a viable initial molecular diagnostic approach for low-resource countries where MLPA could be used to evaluate negative mPCR cases and cases with ambiguous mutation borders. Our findings may have important implications in the early identification of DMD with limited resources in Sri Lanka and to develop tailored molecular diagnostic algorithms that are regional and population specific and easily implemented in resource limited settings.

Project description:BackgroundDiabetic retinopathy (DR) is one of the leading causes for complete loss of vision among working-aged adults around the world. The present study aims to evaluate the rate of DR and its risk factors among the adults with young-onset diabetes from a tertiary care setting in Sri Lanka.MethodsA consecutive sample of 1,007 individuals referred from multiple centers, were invited for the study. Ophthalmological evaluation was done, with dilated indirect ophthalmoscopy by an Ophthalmologist. Retinopathy was classified according to the International Clinical DR Disease Severity Scale. An interviewer-administered questionnaire was used to collect socio-demographic and anthropometric details. Seated blood pressure, Fasting Blood Glucose (FBG), HbA1c and urine microalbumin were also measured. Data were analysed using SPSSv14. A binary logistic regression analysis was performed in all patients, with 'presence of DR' as the dichotomous dependent variable and other independent covariates.ResultsSample size was 684 (response rate-67.9%), mean age was 37.1?±?5.9 years and 36.0% were males. Mean duration of diabetes was 5.2?±?4.0 years. Previous retinal screening had been done in 51.0% by a non-specialist doctor and in 41.5% by a consultant ophthalmologist. Rate of any degree of DR in the study population was 18.1% (Males 16.4%, Females 20.0%; P?=?NS). In patients with DR, majority had mild Non-Proliferative DR (NPDR) (57.2%), while 32.2% had moderate NPDR, 0.8% had severe NPDR and 9.7% had maculopathy. Mean age, duration of diabetes, systolic (SBP) and diastolic blood pressure (DBP), FBG, HbA1c and urine microalbumin levels were significantly higher amongst the patients with DR. The results of the binary logistic regression indicate that the duration of diabetes (OR:1.24), HbA1c (OR:1.19), age (OR:1.11), urine Microalbumin (OR:1.11) and DBP (OR:1.04) all were significantly associated with DR.ConclusionsIn this large multi center study, nearly one in five adults with young-onset diabetes was found to have retinopathy. Age, duration of diabetes, HbA1C and urine Microalbumin levels were significantly associated with the presence of retinopathy, while HbA1c was also a significant factor determining severity. Nearly 50% of the study population has never undergone retinal screening by an ophthalmologist, highlighting the need for well organized screening programs.

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Applicability of protocols from high-income countries in a resource limited setting; real world data of histopathology, clinical features and long-term outcome of Hodgkin Lymphoma in Sri Lanka.

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