scholarly journals SP135RENAL INVOLVEMENT IN SICKLE CELL DISEASE IN TRIBAL WESTERN INDIA

2015 ◽  
Vol 30 (suppl_3) ◽  
pp. iii422-iii422
Author(s):  
Mohan Patel ◽  
Vivek Kute ◽  
Praksh Ugale ◽  
Rajesh Valvi
2013 ◽  
Vol 2013 ◽  
pp. 1-7 ◽  
Author(s):  
Harshada K. Kangne ◽  
Farah F. Jijina ◽  
Yazdi M. Italia ◽  
Dipti L. Jain ◽  
Anita H. Nadkarni ◽  
...  

Objective. Sickle cell disease has variable clinical manifestations. Activation of neutrophils plays an important role in the initiation and propagation of vaso occlusive crises which can be analysed by determining the expression of neutrophil antigens such as CD16, CD32, and CD62L. The common FcγR polymorphisms (FcγRIIA and FcγRIIIB) are considered to influence clinical presentation. This study focuses on distribution of FcγR polymorphisms and their association with neutrophil activity among the patients from western India.Methods. In this paper 127 sickle cell anemia patients and 58 patients with sickle-β-thalassemia (median age12±8.58 years) with variable clinical phenotypes along with 175 normals were investigated. FcγRs polymorphisms were analysed by RFLP and AS-PCR. Activation of neutrophils was measured by flow cytometry.Results. The genotypic frequency of the H/R genotype of FcγRIIA and the NA1/NA1 genotype of FcγRIIIB was significantly decreased in patients compared to normals (P-0.0074,P-0.0471, resp.). We found a significant difference in the expression of CD32 and CD62L among the patients as against normals. A significantly higher expression of CD32 was seen in the milder patients with the H/H genotype (P-0.0231), whereas the expression of CD16 was higher in severe patients with the NA2/NA2 genotype (P-0.0312).Conclusion. The two FcγR polymorphisms had significant association with variable phenotypes of sickle cell disease. The expression of CD62L decreased in our patients indicating activation of neutrophils.


2010 ◽  
pp. n/a-n/a
Author(s):  
Khushnooma Y. Italia ◽  
Dipty Jain ◽  
Kanjaksha Ghosh ◽  
Roshan B. Colah

2019 ◽  
Vol 4 (4) ◽  
pp. 125 ◽  
Author(s):  
Kapilkumar Dave ◽  
Palanivel Chinnakali ◽  
Pruthu Thekkur ◽  
Shrey Desai ◽  
Chandrakant Vora ◽  
...  

In a tribal area of western India, a non-governmental organization implemented a comprehensive sickle cell disease (SCD) program at a secondary level hospital. In a cohort of SCD patients registered during December 2015 to June 2017, we assessed rates of lost to follow-up (LTFU) during the follow-up period using routinely collected data. We compared the uptake of proven interventions and indicators of disease severity from one year prior to registration until the end of the study (June 2018). Of 404 patients, the total follow-up duration was 534 person-years (PY). The rate (95% CI) of LTFU was 21 (17.5–25.3) per 100 PY. The proportion of people who received the pneumococcal vaccine improved from 10% to 93%, and coverage of hydroxyurea improved from 3.5% to 88%. There was a statistically significant decrease in rates (per 100 PY) of pain crisis (277 vs 53.4), hospitalization (49.8 vs 42.2), and blood transfusion (27.4 vs 17.8) after enrollment in the SCD program. Although clinical intervention uptake was high, one quarter of the patients were LTFU. The study demonstrated significant reductions in disease severity in SCD patients.


2000 ◽  
Vol 103 (2) ◽  
pp. 122-123 ◽  
Author(s):  
M.B. Mukherjee ◽  
R.R. Surve ◽  
K. Ghosh ◽  
R.B. Colah ◽  
D. Mohanty

2014 ◽  
Vol 25 (7) ◽  
pp. 1319-1325 ◽  
Author(s):  
Abhay Tidake ◽  
Pranil Gangurde ◽  
Anup Taksande ◽  
Ajay Mahajan ◽  
Pratap Nathani

AbstractIntroductionCardiovascular events and complications are the leading cause of mortality and morbidity in patients with sickle cell disease. Cardiac abnormalities occur frequently and at an early stage in sickle cell anaemia patients, despite being more evident in adulthood. Sickle cell anaemia patients are increasingly able to reach adulthood owing to improved healthcare, and may, therefore, suffer the consequences of chronic cardiac injury. Thus, the study of cardiac abnormalities is essential in childrenObjectiveThe aim of this study was to determine the echocardiographic changes in left ventricular function in children suffering from sickle cell disease in Mumbai, Western India.MethodsThe study comprised of 48 cases of sickle cell anaemia and 30 non-anaemic controls with normal haemoglobin and electrophoresis pattern. M-mode, two-dimensional, and Doppler echocardiographic measurements of patients and controls were performed according to the criteria of the American Echocardiography Society.ResultsOn Doppler study, the A wave height was increased and the E/A ratio was decreased, whereas the deceleration and isovolumetric relaxation times were prolonged, which is typically seen in slowed or impaired myocardial relaxation (p<0.001). Although chamber dilatations were present, echocardiographic parameters showed no statistically significant correlation with severity of anaemia and age among the sickle cell patients.ConclusionsWe conclude that the increased left ventricular stiffness, compared with controls, might be due to fibrosis related to ischaemia caused by SS disease in addition to wall hypertrophy.


2015 ◽  
Vol 32 (2) ◽  
pp. 215-220 ◽  
Author(s):  
S. V. Deshpande ◽  
S. S. Bhatwadekar ◽  
Parth Desai ◽  
Tarang Bhavsar ◽  
Ankit Patel ◽  
...  

2013 ◽  
Vol 21 (2) ◽  
pp. 186-189 ◽  
Author(s):  
Harshada K. Kangne ◽  
Farah F. Jijina ◽  
Yazdi M. Italia ◽  
Dipti L. Jain ◽  
Anita H. Nadkarni ◽  
...  

Author(s):  
Pinkal Shah ◽  
V. M. Bhagat ◽  
Komal Patel ◽  
Chandani Patel

Background: Sickle haemoglobin is highly prevalent in western India. Sickle cell disease (SCD) is the generic term for the group of haemoglobinopathies caused by the occurrence of haemoglobin S (Hbs) in the homozygous form –sickle cell anaemia (Hbss) or as the heterozygous combination of Hbs with another abnormal haemoglobin such as Hbsc or beta –thalassaemias (Hbs b-thal). While doing autopsy in case of death with no apparent cause, the possibility of death may be due to vaso-occlusive crisis in sickle cell disease should be kept in mind. The findings at autopsy are variation of features which may or may not be directly connected to death. The goal is to draw awareness among physicians and relatives on need of autopsy as to minimize future unexpected death from complication or crisis and to enhance knowledge on both parties.Methods: This was a study of autopsy specimens received between January 2015 to December 2015 at tertiary care hospital.Results: Total of 679 autopsy cases were received, out of which sickled erythrocytes were detected in 25 cases. The mean age at death was 30 years, a male/female ratio of 1.5:1 and peak mortality was in the 2nd to 4th decades of life. The commonest presentation was sudden death. The cause of death in middle aged patients were vaso occlusive crisis, in paediatric patients were infection and in older patients were chronic organ damage.Conclusions: Early diagnosis, prompt treatment and extended screening programme are necessary in prevalent tribal belt of western India to reduce morbidity and mortality. we should also introduce awareness programmes in tribal belt of western India.


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