scholarly journals Study of Prevalance of Nephropathy among Sickle Cell Disease Patients in Waghodia Region, Vadodara, Gujarat

Author(s):  
Charmi C. Thakkar ◽  
Inampudi Sailaja

Introduction: Sickle-cell disease (or drepanocytosis) is a life-long blood disorder Characterized by red blood cells that assume an abnormal, rigid, sickle shape. Sickle cell disease (SCD) has several complications, including chronic renal failure, manifesting with hypertension (high blood pressure) proteinuria (protein loss in the urine), hematuria (redblood cells in urine) and worsening anaemia. Progression to end-stage renal failure confers a poor prognosis. Objective: The objective of the study was to determine the Prevalence of Nephropathy among sickle cell disease patients. Materials and Methods: This cross sectional study includes a total 150 participants who suffering from sickle cell anemia and attending our Institute. Renal function test and Urine examination of all participants was done. Estimated Glomerular Filtration Rate (eGFR) calculated using the Cockroft Gault formula. Comparison of results was done between Sickle cell trait and Sickle cell disease Group. Results: The mean age of the SCA patients were 25.54±10 years. Maximum participants are found to be from age group 25-30 yr(n=35) followed by 20-25 yr(n=30). Of the 150 SCA patients, 89 (59.33%), and 61 (40.66%) were males and females, respectively. The Mean value of S.Creatinine of SCT group is 0.73±0.46 mg/dl and SCD is 1.0±0.35 mg/dl, while the Mean value of eGFR is 134.19±87.21 ml/min and 124.20 ±58.25 ml/min in SCT and SCD Group respectively. Conclusions: From our study we conclude that the Derangement of Kidney function in sickle cell disease is frequent in our setting especially among young adult. It concerns SCD as well as SCT patients. Albuminuria is more frequent in homozygote patients and its prevalence increase with age. Age ≥ 25 years is associated with high risk of CKD in SCA group and albuminuria in SCD.

Author(s):  
Vijay Shah ◽  
Akash Patel ◽  
Praful Bambharoliya ◽  
Jigisha Patadia

Introduction: Sickle Cell Disease (SCD) is an inherited chronic haemolytic anaemia. The diseased person suffers from various complications such as anaemia, frequent infection, fever, hand-foot syndrome, stroke, etc. Puberty changes includes the appearance of the secondary sexual characteristics, increase in height, change in body composition and development of reproductive capacity. Aim: To study the sexual maturity and effect of multiple blood transfusions in adolescents suffering from SCD. Materials and Methods: It was a cross-sectional study conducted on 35 adolescents of age group 11 to 15 years, suffering from SCD. Study was conducted over a period of six months from March 2018 to September 2018 at Department of Paediatrics. SCD was diagnosed by Haemoglobin (Hb) electrophoresis. Weight and height were measured of all the participants. For assessing the sexual maturity, Tanners staging was used. Unpaired t-test was done for data analysis. Results: The mean age of the patients was 13.03±1.7 years. There were 25 males and 10 females. The mean age of male patients between Tanner stage 2(14.63±0.52 years) and Tanner stage 3 (14.75±0.5 years) was significantly higher than the Indian data for males (11.3 and 12.8 years, respectively). The mean age of female patients between Tanner stages 2 (13.5±2.12 years) and Tanner stage 3 (14.33±1.16 years) was higher than the Indian reference data for girls (10.2 and 11.6 years respectively). Conclusion: This study concluded that adolescents with SCD were significantly shorter in height and weight than the standard reference population. Sexual maturity is delayed in adolescents with sickle cell anaemia.


1981 ◽  
Vol 27 (2) ◽  
pp. 314-316 ◽  
Author(s):  
E F Roth ◽  
P A Bardfeld ◽  
S J Goldsmith ◽  
E Radel ◽  
J C Williams

Abstract Data on plasma hydroxybutyrate dehydrogenase activity (I) and myoglobin concentration were used to evaluate painful sickle cell crises. I was increased during non-crisis steady state in patients with sickle cell disease as compared to normal values (232, SD 79.7 vs 85, SD 33 Sigma units/mL). During crisis, the mean value for I increased further to 379 (SD 139) Sigma units/mL. For 12 patients evaluated both during steady state and crisis, there was a mean increase in plasma I of 131% (SD 76%). Repeated determinations of I in sickle cell disease patients during several months while they were in steady state showed that baseline I varied by no more than 20% from the mean. Plasma myoglobin in patients with sickle cell disease was not above normal, but during crisis 21 of 39 patients tested had increased plasma myoglobin concentrations. Our data suggest that I may be a useful indicator of sickle cell crisis when the patient's own baseline value is available for comparison. Plasma myoglobin measurements give evidence of muscle damage during crisis with high specificity but low sensitivity.


Author(s):  
Jada M Selma ◽  
Hannah Song ◽  
Christian Rivera ◽  
Simone Andrea Douglas ◽  
Abhiramgopal Akella ◽  
...  

Sickle cell disease (SCD) is the most common hereditary blood disorder in the United States. SCD is frequently associated with osteonecrosis, osteoporosis and osteopenia and other bone related complications such as vaso-occlusive pain, ischemic damage, osteomyelitis, and bone marrow hyperplasia known as sickle bone disease (SBD)1,2. Previous SBD models have failed to distinguish the age- and sex-specific characteristics of bone morphometry. In this study, we use the Townes mouse model of SCD to study the pathophysiological complications of SBD in both SCD and sickle cell trait. Changes in bone microarchitecture and bone development were assessed by high-resolution quantitative micro-computed tomography (microCT) and the 3D reconstruction of femurs from male and female mice. Our results indicate that SCD causes bone loss and sex-dependent anatomical changes in bone. Particularly, SCD female mice are prone to trabecular bone loss while cortical bone degradation occurs in both sexes. Additionally, we describe the impact of genetic knockdown of cathepsin K and E-64 mediated cathepsin inhibition on SBD.


Author(s):  
Flávia Carvalho ◽  
Ariani Souza ◽  
Ana Ferreira ◽  
Simone Neto ◽  
Ana Oliveira ◽  
...  

Purpose To describe the reproductive variables associated with different sickle cell disease (SCD) genotypes and the influence of contraceptive methods on acute painful episodes among the women with the homozygous hemoglobin S (HbSS) genotype. Methods A cross-sectional study was conducted between September of 2015 and April of 2016 on 158 women afflicted with SCD admitted to a hematology center in the Northeast of Brazil. The reproduction-associated variables of different SCD genotypes were assessed using the analysis of variance (ANOVA) test to compare means, and the Kruskal-Wallis test to compare medians. The association between the contraceptive method and the acute painful episodes was evaluated by the Chi-square test. Results The mean age of women with SCD was 28.3 years and 86.6% were mixed or of African-American ethnicity. With respect to the genotypes, 134 women (84.8%) had HbSS genotype, 12 women (7.6%) had hemoglobin SC (HbSC) disease genotype, and 12 (7.6%) were identified with hemoglobinopathy S-beta (S-β) thalassemia. The mean age of HbSS diagnosis was lower than that of HbSC disease, the less severe form of SCD (p < 0.001). The mean age of menarche was 14.8 ± 1.8 years for HbSS and 12.7 ± 1.5 years for HbSC (p < 0.001). Among women with HbSS who used progestin-only contraception, 16.6% had more than 4 acute painful episodes per year. There was no statistically significant difference when compared with other contraceptive methods. Conclusion With respect to reproduction-associated variables, only the age of the menarche showed delay in HbSS when compared with HbSC. The contraceptive method used was not associated with the frequency of acute painful episodes among the HbSS women.


2017 ◽  
Vol 4 (2) ◽  
Author(s):  
Dr. Rajesh Kumar Ajagallay ◽  
Dr. Gaukaran Janghel ◽  
Dr. Vimal Chandra Bhagat ◽  
Viyata Chanda ◽  
Dr. Rakesh Kumar Agrawal ◽  
...  

Background: Sickle cell disease is an inherited blood disorder in which the body produces abnormal shaped red blood cells (RBC). The disease affects both biological and psychosocial aspects of patients. Aim: Present study aimed at investigating the different mental health dimensions used by heterozygous and homozygous sickle cell anemic patients. Method: The cross-sectional study design with the total 100 sickle cell anemic adolescents of both the sexes were selected in 10 to 20 year age groups, from various hospitals and health clinics of Chhattisgarh, India. The correlation analysis was used for analyzing the data. Results: Total 100 patients were selected which consisted of 30 homozygous and 70 heterozygous adolescent patients with sickle cell gene. The Emotional Stability was which higher significantly correlated with the intelligence quotients, IQ (r = .387, p< .001) than the other dimensions. Only two dimensions of mental health viz. emotional stability (t- 2.38; p<.018) and self-concept (t- 2.32; p<.001) of sickle cell patients which differed among heterozygous and homozygous patients.


Author(s):  
Varsha Kose ◽  
Saurabh Kose

Background: Pregnancy in sickle cell women has numerous obstetrical, nonobstetrical and fetal complications. Our objective was to study the pregnancy outcome in women with sickle cell disease/trait.Methods: A descriptive cross sectional study in 2 years study period was conducted. 57 women with sickle cell disease/trait were identified. They were attending the antenatal clinic and were admitted in obstetric ward and followed till 7 days after delivery.Results: Out of total 57 women 49 women (85.96%) were sickle cell trait (AS pattern) and 8 women (14.03%) were sickle cell disease (SS pattern). In women with sickle cell trait Pregnancy induced hypertension was the most common complication. Anaemia and hypothyroidism was the common associated medical problem. 5 babies need NICU admission, 6 babies PBU admission and 33 babies given to mother side just after birth. In women with sickle cell disease severe anaemia, preeclampsia, oligohydramnios and intrauterine growth restriction, lower segment caesarean section for preterm baby was the most common complication. 1 baby required NICU admission, 5 babies require PBU admission and only 2 babies given to mother just after delivery. There were no maternal mortality and neonatal mortality.Conclusions: Maternal morbidity and neonatal morbidity is more in sickle cell disease women. They require early diagnosis, premarital and preconceptional counselling, good multidisciplinary obstetrics and neonatal care and early referral to higher centre.


Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 8-9
Author(s):  
Abiola Oladapo ◽  
Elyse Swallow ◽  
Allison Briggs ◽  
Miriam L. Zichlin ◽  
Bjorn L Mellgard

Introduction: Sickle cell disease (SCD) is an inherited blood disorder affecting ~100,000 individuals in the US. SCD is considered a chronic, lifelong condition that requires comprehensive management. Vaso-occlusive crises (VOCs) are the most common complications of SCD, resulting in intense pain and potential irreversible organ damage. The objective of this study was to characterize the demographic and clinical characteristics of patients with SCD. Methods: A retrospective database analysis was conducted using data from the IBM MarketScan Commercial Claims and Medicare-Supplemental Claims database (July 1, 2013 to June 30, 2018). Patients were included if they met the following criteria: ≥2 diagnoses of SCD on different claims between July 1, 2013 and January 1, 2017, ≥6 years of age on January 1, 2017, and continuous enrollment throughout the 1-year study period (January 1, 2017 to December 31, 2017). Descriptive statistics were used to assess patient demographics (age and sex) and clinical characteristics (Charlson Comorbidity Index [CCI] and other selected comorbidities). In addition, the following outcomes were assessed: the proportion of patients who experienced ≥1 VOC, the frequency of VOCs by care setting, the duration of inpatient VOCs, the monthly VOC risk, and the time between subsequent VOCs. Results: A total of 8174 patients met the inclusion criteria. The mean (± standard deviation [SD]) age was 40.8 (±19.5) years and 63.5% of the patients were female. The mean (±SD) CCI was 0.6 (±1.3), with chronic pulmonary disease, diabetes, renal disease, cerebrovascular disease, and stroke identified as the most common comorbidities. Approximately 20% (n=1659) of patients experienced ≥1 VOC and the mean monthly VOC risk was 0.07 (±0.19). Among patients with ≥1 VOC, the mean (±SD) number of VOCs was 5.2 (±7.7) and the median (interquartile range) time from first to second VOC was 2.4 (0.5-8.2) months. Approximately 18% (n=1461) of patients experienced ≥1 VOC managed in an outpatient setting and 10% (n=844) of patients experienced ≥1 VOC managed in an inpatient setting. Approximately 8% (n=646) of patients experienced ≥1 inpatient and ≥1 outpatient VOC during the study period. Among patients with ≥1 outpatient VOC, the mean (±SD) number of outpatient VOCs was 4.6 (±6.9); among patients with ≥1 inpatient VOC, the mean (±SD) number of inpatient VOCs was 2.2 (±2.3) and the mean (±SD) inpatient VOC duration was 6.6 (±6.4) days. Conclusions: VOCs are common complications of SCD, affecting a subset of patients who often experience recurrent VOC episodes requiring professional health care. VOCs are associated with a significant disease burden on the patient and, potentially, the health care system. Disclosures Oladapo: Takeda:Current Employment, Current equity holder in publicly-traded company.Swallow:Analysis Group, Inc.:Current Employment.Briggs:Analysis Group, Inc.:Current Employment.Zichlin:BMS:Other: Employee of Analysis Group Inc., which received consulting fees.Mellgard:Baxalta US Inc., a Takeda company:Current Employment, Current equity holder in publicly-traded company.


Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 30-31
Author(s):  
Omar Abughanimeh ◽  
Mahammed Khan Suheb ◽  
Steven Ebers ◽  
Julie Eclov ◽  
Robin High ◽  
...  

Background. Sickle cell disease (SCD) is a group of blood disorders that results from point mutations causing different hemoglobinopathies, including hemoglobin SS disease, hemoglobin SC disease, and sickle cell beta-thalassemia. According to the Centers for Disease Control and Prevention (CDC), it is estimated that SCD affects 100,000 Americans. SCD occurs among one out of every 365 African-America births, and one out of every 16,300 Hispanic births. Approximately, one in 13 African-America babies is born with sickle cell trait. Despite being a common disease, patients with SCD have less access to comprehensive team care than patients with other genetic diseases. The state of Nebraska has a population close to two million, where the three most common races are Caucasians (78.1%), Hispanic (11.4%) and African American (5.2%). Despite the racial distribution, SCD is not uncommon in Nebraska. Herein, we report the first database of SCD in the state of Nebraska by reviewing the records of the two biggest tertiary care centers in the state: The University of Nebraska Medical Center (UNMC) and Children's Hospital & Medical Center. Methods: This is a retrospective study. After an IRB approval, we retrospectively reviewed charts of 358 patients who had an ICD-10 code related to SCD and had their care at UNMC or Children's Hospital & Medical Center since January 2014. Inclusion criteria included any patient, regardless of age, who had a confirmed diagnosed of SCD regardless of the genotype. Data was collected to create a comprehensive data base for both adults and children. Children were defined as any individual who is following with pediatric hematology as an outpatient or was born on or after January 1, 2002. Results: A total of 358 patients were reviewed with 355 patients included in the study. Ninety six of them were adults with SCD (mean age of 28.9 years) while 82 were children with SCD (mean age 5.7 years), and 178 patients with sickle cell trait. Table 1 summarizes the demographics for adults and children with SCD. Only 30 out 96 patients from the adult group were employed. Sixty three adult patients were prescribed hydroxyurea compared to 32 children. Regarding simple red blood cell transfusion, the adult group had a median of 2.1 units/year (0-99.2 units/year) which was less than the pediatric group 5.8 unit/year (0-95.6). Twenty one adult patients had alloantibodies, with Anti-E as the most common , while only three children had alloantibodies. Table 2 summarizes the SCD complications for both adults and children per genotype. Our study showed that having SS genotype was associated with higher risk for acute chest syndrome (p= 0.0023) and iron overload (p=0.039). Moreover, SS genotype was predictor of more emergency room (ER) visits and admissions compared to the SC genotype. Since January 2014, the mean number for clinic visits was higher for children compared to adults (30 vs 14.3 visits, p&lt;.001). However, the mean number for ER visits was higher in adults compared to children (8.1 vs 3.8 visits, p&lt;.001). Also, adults spent more days in the hospital with a median of 20 days (1-748 days) compared to pediatrics who had a median of four days (1-94 days). The probability of admission once presenting to the ER was also higher in the adults group compared to pediatrics group (0.56 vs 0.44). During the study period, six adults patients died and none from the pediatric group. Conclusion : SCD and its complications represents a serious issue in the state of Nebraska. It is also associated with high acute health care utilization. Our study showed that most of the cases are in the urban areas of the state of Nebraska. Also, we noticed that adults have more demands and lack of care compared to children, and they also don't follow in clinics as children do. This project represents the first step in the plan to improve the care of patients with sickle cell disease in Nebraska, through establishing a comprehensive data base and a comprehensive sickle cell disease clinic for both adults and children. Disclosures Gundabolu: BioMarin: Consultancy; Bristol Myers Squibb pharmaceuticals: Consultancy.


Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 3361-3361
Author(s):  
Wafaa E. Ibrahim ◽  
Ashraf M. Abdelmonem ◽  
Nermin H. Mahmoud ◽  
Waleed Mostafa

Abstract Introduction: Renal failure is one of the most serious complications of sickle cell diseases; it affects 5% – 18% and leads to early death. Protienuria is highly correlated with risk for future chronic renal failure. Aim: to estimate the prevalence of microalbuminuria among Egyptian children and adults with sickle hemoglobinopathy, and to evaluate the effect ACE inhibitor on positive cases. Subject and method: 60 patients (mean age 13.03±9.15) with sickle hemoglobinopathy were included. Patients were divided into 3 groups: sickle cell disease (group I) constituted 20%, Sickle ß-Thalassemia (group II) 46.6% and sickle cell trait (group III) 33.4%. Informed consent was obtained from patients and/or guardians and study approval by local IRB was obtained. Microalbuminuria was measured by turbidimetric assay in 24 hour urine collection. Patients with microalbuminuria were started on a 4 weeks trial of ACE inhibitor after excluding any contraindication for the drug. Patients were closely monitored for side effects and repeat microalbuminuria assay was done at the end of the trial period. Data were coded, entered and processed using SPSS (version 11). Results: The prevalence of microalbuminuria was 32.5% in sickle cell disease, and sickle β thalassemia while in sickle cell trait was 5 % (p=0.02). The highest level of microalbuminuria (93.3+-146.27) was observed in sickle cell disease patients followed by sickle β thalassemia (63.82+-172.38) followed by sickle cell trait(18.75+-12.86). Compared to patients with no microalbuminuria, the group with positive microalbuminuria was significantly older (18.31±4.48 vs. 9.24± 4.96 years), had higher number of blood transfusions / year (7.08±3.55 vs. 5.11±4.05), higher mean number of hemolytic crisis (2.38±1.98 vs. 1.11±1.19), higher mean number of pain crisis (2.92±1.61 vs. 2.2±1.48), and higher mean serum creatinine (0.63±0.31 vs. 0.47±0.19) (p<0.01). After 4 weeks trial of ACE inhibitor there was a statistically significant reduction (77.9% reduction) in number of cases with microalbuminuria as well as level of microalbuminuria (dropped from 171.91 ± 251.60 to 27.11 ± 13.99) (p= 0.005). Conclusion: Microalbuminuria is a significant problem among patients with sickle cell disease. ACE inhibitors have significant impact on improving microalbuminuria.


Author(s):  
Gupta Umesh Prasad ◽  
Bhandari Amrit ◽  
Giri Dhruba ◽  
Adhikari Sushmita ◽  
Paudel Sangita ◽  
...  

Sickle cell disease (SCD) is prevalent in malaria-endemic areas because the gene for sickle cell provides its carrier with resistance against malaria. In Nepal, malaria is prevalent in Terai, hence the susceptibility of SCD is high in this region. Being indigenous to the Terai, thousands of people in the Tharu communities of the Banke districting Nepal are believed to have suffered from sickle cell disease. The objective of this study was to find out the status of sickle cell disease among the Tharu population of Banke district, Nepal. A cross-sectional, experimental study was performed among systematically randomly selected 275 samples from 3 Village Development Committee (VDCs). All the samples were first screened for the presence of sickle hemoglobin using the sickle solubility test method in Bheri Zonal Hospital. Then all sickle solubility positive samples were further processed for alkaline hemoglobin electrophoresis by using Interlab GenioS electrophoresis instrument. Out of a total 275 samples, 33 (12.0%) samples were confirmed as sickle solubility test positive. Among which, sickle cell trait was the most common disorder found grossing to 81.8%, followed by homozygous sickle cell disease; (15.2 %). One case (3.0%) of compound heterozygous sickle beta-thalassemia was also found.The Males were found to be more affected than females with ratio of 1.4:1.1. The highest frequency of SCD was found to be in 11-20 age groups comprising about 36.4%. Dangaura Tharu (51.5%) was the most common ethnic group with this disorder. The findings of this study indicate SCD is prevalent among the Tharu population in Banke district of Province-5, Nepal


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