Pub Date : 2017-09-01DOI: 10.4103/MJDRDYPU.MJDRDYPU_39_17
M. Zafar, S. Sinha, S. Aggarwal, M. Bhargava
Background: Monoclonal light chain gammopathies are uncommon subsets of plasma cell disorders which usually present as diagnostic challenge. Materials and Methods: Twenty cases of monoclonal light-chain gammopathy were identified after screening 150 plasma cell disorders at a tertiary care referral center of North India and were analyzed for clinical profile and treatment outcomes. Results: Out of 20 cases of monoclonal light-chain gammopathy, 65% (13/20) were light-chain multiple myeloma (LCMM) type, 20% (4/20) were light-chain deposition disease (LCDD) type, and 15% (3/20) had primary amyloidosis (AL). Renal failure (65% of cases) was the most common presentation. All the patients with LCDD presented with renal failure (4/4) while as 61% of LCMM (8/13) and 33% of AL (1/3) presented with renal failure. Five patients presented with anemia and all were LCMM type. Two patients presented with lytic bone lesions (LCMM type) and one patient presented with plasmacytoma (LCMM). Overall response rate after 4 cycles of induction therapy was 92.3% in LCMM group, 100% in LCDD group, and 33.33% in AL group (excluding one patient who expired before the start of treatment). LCMM showed 23% partial remission (PR), 30.77% very good PR, 38.46% complete response (CR), and 5% no response (NR). LCDD showed PR 75% and CR 25%. AL showed PR 33.33% and NR 5%. Conclusions: Renal failure is a common presentation of monoclonal light chain gammopathies, and it should alert the treating physician for the underlying uncommon plasma cell disorder.
{"title":"Spectrum of monoclonal light-chain gammopathy in a tertiary care hospital","authors":"M. Zafar, S. Sinha, S. Aggarwal, M. Bhargava","doi":"10.4103/MJDRDYPU.MJDRDYPU_39_17","DOIUrl":"https://doi.org/10.4103/MJDRDYPU.MJDRDYPU_39_17","url":null,"abstract":"Background: Monoclonal light chain gammopathies are uncommon subsets of plasma cell disorders which usually present as diagnostic challenge. Materials and Methods: Twenty cases of monoclonal light-chain gammopathy were identified after screening 150 plasma cell disorders at a tertiary care referral center of North India and were analyzed for clinical profile and treatment outcomes. Results: Out of 20 cases of monoclonal light-chain gammopathy, 65% (13/20) were light-chain multiple myeloma (LCMM) type, 20% (4/20) were light-chain deposition disease (LCDD) type, and 15% (3/20) had primary amyloidosis (AL). Renal failure (65% of cases) was the most common presentation. All the patients with LCDD presented with renal failure (4/4) while as 61% of LCMM (8/13) and 33% of AL (1/3) presented with renal failure. Five patients presented with anemia and all were LCMM type. Two patients presented with lytic bone lesions (LCMM type) and one patient presented with plasmacytoma (LCMM). Overall response rate after 4 cycles of induction therapy was 92.3% in LCMM group, 100% in LCDD group, and 33.33% in AL group (excluding one patient who expired before the start of treatment). LCMM showed 23% partial remission (PR), 30.77% very good PR, 38.46% complete response (CR), and 5% no response (NR). LCDD showed PR 75% and CR 25%. AL showed PR 33.33% and NR 5%. Conclusions: Renal failure is a common presentation of monoclonal light chain gammopathies, and it should alert the treating physician for the underlying uncommon plasma cell disorder.","PeriodicalId":36033,"journal":{"name":"Medical Journal of Dr. D.Y. Patil University","volume":"10 1","pages":"424"},"PeriodicalIF":0.0,"publicationDate":"2017-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46762511","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2017-09-01DOI: 10.4103/0975-2870.218179
R. Saxena, M. Jain, Harshvardhan Singh, R. Meena
{"title":"Osteosarcoma of first metacarpal with bilateral lung metastasis: A rare case report","authors":"R. Saxena, M. Jain, Harshvardhan Singh, R. Meena","doi":"10.4103/0975-2870.218179","DOIUrl":"https://doi.org/10.4103/0975-2870.218179","url":null,"abstract":"","PeriodicalId":36033,"journal":{"name":"Medical Journal of Dr. D.Y. Patil University","volume":"10 1","pages":"485"},"PeriodicalIF":0.0,"publicationDate":"2017-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46526150","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2017-09-01DOI: 10.4103/MJDRDYPU.MJDRDYPU_66_17
Jennifer M. Grimm
{"title":"Is infectious mononucleosis a pediatric disease","authors":"Jennifer M. Grimm","doi":"10.4103/MJDRDYPU.MJDRDYPU_66_17","DOIUrl":"https://doi.org/10.4103/MJDRDYPU.MJDRDYPU_66_17","url":null,"abstract":"","PeriodicalId":36033,"journal":{"name":"Medical Journal of Dr. D.Y. Patil University","volume":"10 1","pages":"445"},"PeriodicalIF":0.0,"publicationDate":"2017-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45651818","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2017-09-01DOI: 10.4103/MJDRDYPU.MJDRDYPU_41_17
S. Chandanwale, Rahul Jadhav, R. Rao, Piyusha Naragude, Sunita Bhamnikar, J. Ansari
Background: Malignant ovarian tumors have worst prognosis among all gynecological malignancies and highest case fatality rate. There is a steady increase in the age-standardized incidence rate in India. Most cases are diagnosed late when the symptoms such as abdominal distension become apparent. Objective: The aim is to study the histomorphological features of malignant tumors of the ovary and to find out the frequency of various malignant ovarian tumors with respect to age and clinical features. Subjects and Methods: Fifty malignant ovarian tumors were included in the study. Clinical and histomorphological features were studied and correlated. Results: Maximum number of malignant ovarian tumors occurred in between 50–60 years and 21–30 years of age. Pain and lump in the abdomen was the most common presenting symptom. Right ovary was commonly involved. CA-125 blood levels were elevated in 54% of cases. Ultrasound and color Doppler examinations showed irregular solid tumor, ascites, papillary structures, large multilocular solid tumor, and high Doppler content. Histopathology diagnosis was aided by immunohistochemistry. Maximum malignant tumors (n = 31) were surface epithelial tumors, and serous cystadenocarcinoma was most common. Conclusion: Malignant ovarian tumors are the leading cause of death from gynecologic malignancy in females. Clinical symptoms, radiological findings, and other ancillary investigations such as CA-125 are the keys in establishing the preoperative diagnosis of malignant ovarian tumors. Malignant surface epithelial tumors are most common, and serous cystadenocarcinoma is the most common histological type.
{"title":"Clinicopathologic study of malignant ovarian tumors: A study of fifty cases","authors":"S. Chandanwale, Rahul Jadhav, R. Rao, Piyusha Naragude, Sunita Bhamnikar, J. Ansari","doi":"10.4103/MJDRDYPU.MJDRDYPU_41_17","DOIUrl":"https://doi.org/10.4103/MJDRDYPU.MJDRDYPU_41_17","url":null,"abstract":"Background: Malignant ovarian tumors have worst prognosis among all gynecological malignancies and highest case fatality rate. There is a steady increase in the age-standardized incidence rate in India. Most cases are diagnosed late when the symptoms such as abdominal distension become apparent. Objective: The aim is to study the histomorphological features of malignant tumors of the ovary and to find out the frequency of various malignant ovarian tumors with respect to age and clinical features. Subjects and Methods: Fifty malignant ovarian tumors were included in the study. Clinical and histomorphological features were studied and correlated. Results: Maximum number of malignant ovarian tumors occurred in between 50–60 years and 21–30 years of age. Pain and lump in the abdomen was the most common presenting symptom. Right ovary was commonly involved. CA-125 blood levels were elevated in 54% of cases. Ultrasound and color Doppler examinations showed irregular solid tumor, ascites, papillary structures, large multilocular solid tumor, and high Doppler content. Histopathology diagnosis was aided by immunohistochemistry. Maximum malignant tumors (n = 31) were surface epithelial tumors, and serous cystadenocarcinoma was most common. Conclusion: Malignant ovarian tumors are the leading cause of death from gynecologic malignancy in females. Clinical symptoms, radiological findings, and other ancillary investigations such as CA-125 are the keys in establishing the preoperative diagnosis of malignant ovarian tumors. Malignant surface epithelial tumors are most common, and serous cystadenocarcinoma is the most common histological type.","PeriodicalId":36033,"journal":{"name":"Medical Journal of Dr. D.Y. Patil University","volume":"10 1","pages":"430"},"PeriodicalIF":0.0,"publicationDate":"2017-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47805129","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2017-09-01DOI: 10.4103/MJDRDYPU.MJDRDYPU_260_16
N. Kaur, A. Pachisia, P. Saxena, V. Sashindran, P. Puri
Ecthyma gangrenosum (EG) is a relatively rare skin manifestation that is most commonly described in Pseudomonas aeruginosa bacteremia. It is more frequently seen in immunocompromised individuals. We report a case of 60-year-old renal transplant recipient on triple immunosuppressants and diabetes mellitus type 2 on insulin therapy who developed EG-like lesions due to disseminated mycobacterial tuberculosis (MTB) infection. To the best of our knowledge, this is the first case report of EG-like lesions associated with disseminated kochs.
{"title":"Ecthyma gangrenosum like lesions in disseminated mycobacterial tuberculosis infection in a renal transplant recipient","authors":"N. Kaur, A. Pachisia, P. Saxena, V. Sashindran, P. Puri","doi":"10.4103/MJDRDYPU.MJDRDYPU_260_16","DOIUrl":"https://doi.org/10.4103/MJDRDYPU.MJDRDYPU_260_16","url":null,"abstract":"Ecthyma gangrenosum (EG) is a relatively rare skin manifestation that is most commonly described in Pseudomonas aeruginosa bacteremia. It is more frequently seen in immunocompromised individuals. We report a case of 60-year-old renal transplant recipient on triple immunosuppressants and diabetes mellitus type 2 on insulin therapy who developed EG-like lesions due to disseminated mycobacterial tuberculosis (MTB) infection. To the best of our knowledge, this is the first case report of EG-like lesions associated with disseminated kochs.","PeriodicalId":36033,"journal":{"name":"Medical Journal of Dr. D.Y. Patil University","volume":"10 1","pages":"492"},"PeriodicalIF":0.0,"publicationDate":"2017-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42634578","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2017-09-01DOI: 10.4103/MJDRDYPU.MJDRDYPU_17_17
M. Sharma
{"title":"Obstructive sleep apnea and endocrine disorders","authors":"M. Sharma","doi":"10.4103/MJDRDYPU.MJDRDYPU_17_17","DOIUrl":"https://doi.org/10.4103/MJDRDYPU.MJDRDYPU_17_17","url":null,"abstract":"","PeriodicalId":36033,"journal":{"name":"Medical Journal of Dr. D.Y. Patil University","volume":"10 1","pages":"467"},"PeriodicalIF":0.0,"publicationDate":"2017-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"44150494","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2017-09-01DOI: 10.4103/MJDRDYPU.MJDRDYPU_9_17
M. Nandi, A. Hazra, M. Das, S. Bhattacharya, U. Sarkar
Objective: The objective of this study is to delineate the clinical and laboratory profile of infectious mononucleosis due to Epstein-Barr virus (EBV) infection in children admitted to tertiary care teaching hospitals. Materials and Methods: Retrospective observational multicentric analysis of clinical and laboratory features of children between 1 month to 12 years with a diagnosis of infectious mononucleosis due to EBV infection confirmed by positive serology over a 12-month period after seeking approval from the Institutional Ethics Committee. Results: Out of 66 children screened, 53 were included in final analysis. The majority were aged between 5 and 8 years with male: female ratio of 1.2:1. Most presentations were during the monsoon months. The common clinical features were fever (100%), splenomegaly (86.7%), and cervical lymphadenopathy (73.5%) in contrast to the classical triad of fever, sore throat, and generalized lymphadenopathy described in the literature. There were no age differences in clinical findings except for generalized and cervical lymphadenopathy and hepatomegaly which were commoner in 9–12 years age band. Although the incidence of common findings matched with previously published studies, there were some notable differences. While frequencies of upper eyelid edema, epitrochlear lymphadenopathy, and splenomegaly were more, those of rash and sore throat were less. Lymphocytosis and presence of atypical lymphocytes were relatively less common in our series. All children recovered. Conclusions: This multicentric study on profiling childhood infectious mononucleosis, possibly first of its kind from Eastern India, has documented clinical and laboratory features associated with this condition. These data can serve as a reference for future studies.
{"title":"Infectious mononucleosis due to epstein-barr virus infection in children: A profile from eastern India","authors":"M. Nandi, A. Hazra, M. Das, S. Bhattacharya, U. Sarkar","doi":"10.4103/MJDRDYPU.MJDRDYPU_9_17","DOIUrl":"https://doi.org/10.4103/MJDRDYPU.MJDRDYPU_9_17","url":null,"abstract":"Objective: The objective of this study is to delineate the clinical and laboratory profile of infectious mononucleosis due to Epstein-Barr virus (EBV) infection in children admitted to tertiary care teaching hospitals. Materials and Methods: Retrospective observational multicentric analysis of clinical and laboratory features of children between 1 month to 12 years with a diagnosis of infectious mononucleosis due to EBV infection confirmed by positive serology over a 12-month period after seeking approval from the Institutional Ethics Committee. Results: Out of 66 children screened, 53 were included in final analysis. The majority were aged between 5 and 8 years with male: female ratio of 1.2:1. Most presentations were during the monsoon months. The common clinical features were fever (100%), splenomegaly (86.7%), and cervical lymphadenopathy (73.5%) in contrast to the classical triad of fever, sore throat, and generalized lymphadenopathy described in the literature. There were no age differences in clinical findings except for generalized and cervical lymphadenopathy and hepatomegaly which were commoner in 9–12 years age band. Although the incidence of common findings matched with previously published studies, there were some notable differences. While frequencies of upper eyelid edema, epitrochlear lymphadenopathy, and splenomegaly were more, those of rash and sore throat were less. Lymphocytosis and presence of atypical lymphocytes were relatively less common in our series. All children recovered. Conclusions: This multicentric study on profiling childhood infectious mononucleosis, possibly first of its kind from Eastern India, has documented clinical and laboratory features associated with this condition. These data can serve as a reference for future studies.","PeriodicalId":36033,"journal":{"name":"Medical Journal of Dr. D.Y. Patil University","volume":"10 1","pages":"438"},"PeriodicalIF":0.0,"publicationDate":"2017-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49177495","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2017-09-01DOI: 10.4103/MJDRDYPU.MJDRDYPU_280_16
A. Singh, Vinay Mathur, Ramesh Tanger, Arun Gupta
Prune belly syndrome (PBS) is characterized by deficient development of abdominal muscles that causes the skin of the abdomen to wrinkle like a prune, bilateral cryptorchidism, abnormalities of the urinary tract. The etiology of PBS is unclear and possible familial genetic inheritance was reported in some of the studies. We are presenting here a case with the absence of the muscle in the right side of the abdomen as hernia, thinning of the muscle on left side with bilateral cryptorchidism, and abnormalities of the urinary tract. It is the partial presentation of the PBS.
{"title":"Partial prune belly syndrome: A rare case report","authors":"A. Singh, Vinay Mathur, Ramesh Tanger, Arun Gupta","doi":"10.4103/MJDRDYPU.MJDRDYPU_280_16","DOIUrl":"https://doi.org/10.4103/MJDRDYPU.MJDRDYPU_280_16","url":null,"abstract":"Prune belly syndrome (PBS) is characterized by deficient development of abdominal muscles that causes the skin of the abdomen to wrinkle like a prune, bilateral cryptorchidism, abnormalities of the urinary tract. The etiology of PBS is unclear and possible familial genetic inheritance was reported in some of the studies. We are presenting here a case with the absence of the muscle in the right side of the abdomen as hernia, thinning of the muscle on left side with bilateral cryptorchidism, and abnormalities of the urinary tract. It is the partial presentation of the PBS.","PeriodicalId":36033,"journal":{"name":"Medical Journal of Dr. D.Y. Patil University","volume":"10 1","pages":"470"},"PeriodicalIF":0.0,"publicationDate":"2017-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45169639","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2017-09-01DOI: 10.4103/MJDRDYPU.MJDRDYPU_31_17
H. Prasad
{"title":"Fibrocartilaginous dysplasia: A rare but distinct entity","authors":"H. Prasad","doi":"10.4103/MJDRDYPU.MJDRDYPU_31_17","DOIUrl":"https://doi.org/10.4103/MJDRDYPU.MJDRDYPU_31_17","url":null,"abstract":"","PeriodicalId":36033,"journal":{"name":"Medical Journal of Dr. D.Y. Patil University","volume":"10 1","pages":"503"},"PeriodicalIF":0.0,"publicationDate":"2017-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"44077074","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2017-09-01DOI: 10.4103/MJDRDYPU.MJDRDYPU_90_17
E. Okocha, O. Manafa, C. Aneke, E. C. Onwuzuruike, C. N. Ibeh, O. Chukwuma
Background: Sickle cell anemia (SCA) is associated with intense oxidative stress; optimal antioxidant levels are essential to prevent oxidant tissue damage. Objective: The objective of this study was to evaluate superoxide dismutase (SOD) activity and Vitamin C levels in individuals with SCA, heterozygous sickle cell (heterozygous hemoglobin AS [HbAS]), and normal (hemoglobin AA [HbAA]) hemoglobin phenotypes in comparison with objective scores of disease severity (in those with SCA). Subjects and Methods: A total of ninety participants were recruited, including thirty SCA (in steady state), thirty HbAS, and thirty HbAA. From each participant, 5 ml of venous blood was collected; 3 ml was dispensed into plain tubes and serum was extracted for the estimation of SOD activity and Vitamin C level. Serum SOD activity was measured using a semi-automated spectrophotometric procedure, while serum Vitamin C level was estimated by the enzyme-linked immunosorbent assay technique. The remaining 2 ml was used for hemoglobin electrophoresis and full blood count estimation. Objective score of disease severity was calculated for SCA individuals using a scoring system. Results: The mean serum activity of SOD was significantly lower in SCA compared with HbAS and HbAA participants (9.45 ± 3.39 U/ml vs. 12.87 ± 2.17 U/ml and 13.24 ± 2.10 U/ml, P 0.05, respectively). Serum SOD activity was significantly correlated with objective score of disease severity in SCA participants, while Vitamin C level was not (r = −0.529, P = 0.02 and r = −0.349, P = 0.14, respectively). Conclusion: Serum SOD activity is a predictor of disease severity in Nigerian individuals with SCA.
背景:镰状细胞性贫血(SCA)与强烈的氧化应激有关;最佳抗氧化剂水平对防止氧化性组织损伤至关重要。目的:本研究的目的是评估SCA、杂合子镰状细胞(杂合子血红蛋白AS [HbAS])和正常(血红蛋白AA [HbAA])血红蛋白表型个体的超氧化物歧化酶(SOD)活性和维生素C水平，并将其与SCA患者疾病严重程度的客观评分进行比较。对象和方法:共招募90名参与者，包括30名SCA(稳定状态)，30名HbAS和30名HbAA。每位受试者采集静脉血5 ml;取3ml入平管，提取血清，测定SOD活性和维生素C水平。血清SOD活性采用半自动分光光度法测定，血清维生素C水平采用酶联免疫吸附测定技术测定。剩余2ml用于血红蛋白电泳和全血细胞计数估算。使用评分系统计算SCA个体的疾病严重程度客观评分。结果:SCA组血清SOD平均活性显著低于HbAS组和HbAA组(9.45±3.39 U/ml vs. 12.87±2.17 U/ml和13.24±2.10 U/ml, P < 0.05)。SCA受试者血清SOD活性与疾病严重程度客观评分显著相关，而维生素C水平与疾病严重程度客观评分无显著相关性(r = - 0.529, P = 0.02和r = - 0.349, P = 0.14)。结论:血清SOD活性是尼日利亚SCA患者疾病严重程度的预测因子。
{"title":"Serum superoxide dismutase activity: A predictor of disease severity in nigerian sickle cell anemia patients in steady state","authors":"E. Okocha, O. Manafa, C. Aneke, E. C. Onwuzuruike, C. N. Ibeh, O. Chukwuma","doi":"10.4103/MJDRDYPU.MJDRDYPU_90_17","DOIUrl":"https://doi.org/10.4103/MJDRDYPU.MJDRDYPU_90_17","url":null,"abstract":"Background: Sickle cell anemia (SCA) is associated with intense oxidative stress; optimal antioxidant levels are essential to prevent oxidant tissue damage. Objective: The objective of this study was to evaluate superoxide dismutase (SOD) activity and Vitamin C levels in individuals with SCA, heterozygous sickle cell (heterozygous hemoglobin AS [HbAS]), and normal (hemoglobin AA [HbAA]) hemoglobin phenotypes in comparison with objective scores of disease severity (in those with SCA). Subjects and Methods: A total of ninety participants were recruited, including thirty SCA (in steady state), thirty HbAS, and thirty HbAA. From each participant, 5 ml of venous blood was collected; 3 ml was dispensed into plain tubes and serum was extracted for the estimation of SOD activity and Vitamin C level. Serum SOD activity was measured using a semi-automated spectrophotometric procedure, while serum Vitamin C level was estimated by the enzyme-linked immunosorbent assay technique. The remaining 2 ml was used for hemoglobin electrophoresis and full blood count estimation. Objective score of disease severity was calculated for SCA individuals using a scoring system. Results: The mean serum activity of SOD was significantly lower in SCA compared with HbAS and HbAA participants (9.45 ± 3.39 U/ml vs. 12.87 ± 2.17 U/ml and 13.24 ± 2.10 U/ml, P 0.05, respectively). Serum SOD activity was significantly correlated with objective score of disease severity in SCA participants, while Vitamin C level was not (r = −0.529, P = 0.02 and r = −0.349, P = 0.14, respectively). Conclusion: Serum SOD activity is a predictor of disease severity in Nigerian individuals with SCA.","PeriodicalId":36033,"journal":{"name":"Medical Journal of Dr. D.Y. Patil University","volume":"10 1","pages":"406"},"PeriodicalIF":0.0,"publicationDate":"2017-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48663364","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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