Background Aim: This article aimed to sensitize the healthcare providers who care for patients with sickle cell disease (SCD) during pregnancy, to the multiple causes of jaundice in sickle hepatopathy, and sift the ma...Background Aim: This article aimed to sensitize the healthcare providers who care for patients with sickle cell disease (SCD) during pregnancy, to the multiple causes of jaundice in sickle hepatopathy, and sift the maze in establishing the dominant cause. Case Presentation: This is a case of a 28-yr-old Gravida 1 Para 0 + 0 health worker, with sickle cell anaemia and background history of peptic ulcer disease, total right hip replacement and previous multiple blood transfusions due to haemolytic crisis. She presented with upper abdominal pain and progressively deepening jaundice. There was minimal relief of the abdominal pain with anti-ulcer medications. She had tender hepatomegaly, positive Murphy’s sign and deranged liver function parameters. She was also positive for viral hepatitis B markers. A remarkable finding on abdominal ultrasonography was that of a gallbladder with normal wall thickness and multiple stones. She was managed conservatively on intravenous fluids, antibiotics and Livolin forte (Phosphatidylcholine) with improved liver function parameters within a week. Conclusion: Since sickle cell hepatopathy is multifactorial, the importance of unravelling the dominant cause of jaundice or liver dysfunction in these patients and the need for immediate intervention are necessary for effective and targeted care, hence this case report. Our patient had background chronic hepatitis B with a superadded acute cholecystitis with non-obstructing gallstones, and possible subsequent bacterial hepatitis, which responded to antibiotics. The most likely dominant cause of jaundice was chronic hepatitis B that was precipitated by acute cholecystitis.展开更多
Objectives: The study is aimed at determining that errors in assigning genotypes to intending couples do exist;and the impact of these errors on parents. Methods: The study was conducted at the children clinics in Enu...Objectives: The study is aimed at determining that errors in assigning genotypes to intending couples do exist;and the impact of these errors on parents. Methods: The study was conducted at the children clinics in Enugu and Abakiliki, south east Nigeria. It is a cross-sectional retrospective study in which a review of the records of all the children attending 2 private clinics in Enugu and Abakaliki of Enugu and Ebonyi states respectively, over a 3-year period was done. Results: A total of 6006 children attended the children clinics over the study period. Twenty three (23) of them had sickle cell anaemia. Out of the 23 cases 10 (43.5%) were males and 13 (56.5%) were females. Male to female ratio was 1:1.3. The commonest features were abdominal and leg pains, involving 20 (87%) and 21 (91.3 %) respectively. Among the 23 parents that had their genotypes repeated, 9 males had different genotypes from what they had during courtship or before marriage. The genotypes of all the females that could recall their genotypes before marriage or during courtship were the same after a repeat test. Conclusions: There exist errors in assigning genotypes to parents which at the end made them have children with sickle cell anemia, unwittingly, with serious consequences bordering on strained relationship between the parents with the children bearing the brunt.展开更多
Background: Nigeria contributes 30% to the global sickle cell anaemia burden. Cholelithiasis occurs commonly in children with sickle cell anaemia and may remain undiagnosed, mimic abdominal vaso-occlusive crises or be...Background: Nigeria contributes 30% to the global sickle cell anaemia burden. Cholelithiasis occurs commonly in children with sickle cell anaemia and may remain undiagnosed, mimic abdominal vaso-occlusive crises or become complicated by calculus cholecystitis. Early identification of children with sickle cell anaemia who have Cholelithiasis reduces the chances of misdiagnosis, mistreatment and improves outcomes. There is limited knowledge about the prevalence of Cholelithiasis among Nigerian children with sickle cell anaemia. The present study aimed to determine the prevalence of Cholelithiasis in children with sickle cell anaemia in Gombe, northeast Nigeria. Methodology: This was a cross-sectional analysis of children with sickle cell anaemia being followed-up at the sickle cell centre of the Federal Teaching Hospital, Gombe, northeast Nigeria. We consecutively recruited 294 children aged 2 - 17 years while in steady state from June to November 2017. Quantitative data were collected from parents/caregivers via a researcher administered questionnaire and cholecystosonography performed after a minimum of 8-hour fast. Predictors of Cholelithiasis were examined using binary logistic regression. Results: The mean age of children with sickle cell anaemia was 9.0 ± 4.5 years. The prevalence of Cholelithiasis in children with sickle cell anaemia in steady state was 4.8%. Children aged 15 - 17 years had 12 times higher odds of having Cholelithiasis [AOR = 12.268 (95% CI = 1.3 - 112.8)]. Conclusion: The prevalence of Cholelithiasis in children with sickle cell anaemia though generally low, increases progressively with age to peak during middle to late adolescence.展开更多
Vaso-occlusive crisis is a common clinical manifestation in children with sickle cell anaemia, frequently warranting in-patient management with intravenous fluids, analgesics and occasionally anti-malaria and antibiot...Vaso-occlusive crisis is a common clinical manifestation in children with sickle cell anaemia, frequently warranting in-patient management with intravenous fluids, analgesics and occasionally anti-malaria and antibiotics. Pain is the principal symptom of vaso-occlusive crisis and it can affect any part of the body. Pentazocine is a well-known opioid analgesic, commonly used in the treatment of painful crisis in children affected by sickle cell anaemia. It was discovered in the 1950s and was initially thought to be addiction—proof, but this has been disproved over the years by reports of pentazocine dependence/addiction especially in patients living with chronic pain. There have been some case reports of pentazocine abuse/dependence among sickle cell disease patients in the literature, most of which are from Nigeria but none involved joint addiction by siblings. We therefore, report the case of two siblings, known sickle cell anaemia (HbSS) patients, referred to our emergency paediatric unit with history of recurrent painful crisis over a 10-month period leading to abuse of pentazocine. They were jointly managed successfully by the paediatric hematology and the pain and palliative units of the University of Ilorin Teaching Hospital and helped to overcome their abuse and addiction.展开更多
Background: Sickle cell anaemia (SCA) is a serious, multisystem, genetic disorder affecting millions of children worldwide. The disease causes numerous complications that interfere with the health-related quality of l...Background: Sickle cell anaemia (SCA) is a serious, multisystem, genetic disorder affecting millions of children worldwide. The disease causes numerous complications that interfere with the health-related quality of life (HRQoL) of these children including an impact on educational, physical and psychosocial development. Few studies have described the clinical spectrum and quality of life of children with SCA living in a low-resource area. Objectives: This study aimed to determine the clinical spectrum and HRQoL among children living with sickle cell anaemia (SCA) in northwest Tanzania. Methods: This hospital-based cross-sectional study took place at Tertiary and teaching hospital, Bugando Medical Centre, Mwanza Tanzania. The study enrolled children ages 2 - 12 years old with SCA attending the Bugando Medical Centre sickle cell clinic. Health related quality of life was measured using the Pediatric Quality of Life, Brief Generic Core Scale after translating from English into a Swahili version. Important SCA complications were assessed using a structured questionnaire. Results: From October 2016 to March 2017, 204 children were enrolled. Participants presented at a median age of 6 years [IQR 4 - 9]. Among children with SCA the most common clinical signs at the time of enrolment were pale in 69.6% (142/204), jaundice in 65.9% (134/204), oxygen saturation 90% in 25% (51/204) and splenomegaly in 19% (39/204). Severe anaemia was observed in 30.9% (63/204). A majority reported vaso-occlusive crisis (166/204, 81.4%), and very few had experienced a prior stroke (5/204, 2.5%). Using a modified Likert scale, a total of 41/204 (20.1%) children had poor HRQoL indicated by low scores on PedsQL<sup>TM</sup> and 163/204 (79.9%) children had high scores, indicating good HRQoL. On multivariate analysis, age ≥ 5 years (p-value < 0.001), haemoglobin < 7 g/dl (p-value = 0.001) and >3 hospitalizations per year (p-value = 0.008) were associated with poor HRQoL. Conclusion: SCA complications, negatively impact the HRQoL of children l展开更多
This study was aimed at evaluating the iron status of multiple blood transfused sickle cell anaemia (SCA) patients in Benin City, Nigeria. A total of 86 subjects participated in the study, comprising of 30 multiple tr...This study was aimed at evaluating the iron status of multiple blood transfused sickle cell anaemia (SCA) patients in Benin City, Nigeria. A total of 86 subjects participated in the study, comprising of 30 multiple transfused SCA subjects, 30 of rarely transfused SCA subjects and 26 of age and sex matched healthy control subjects. Serum ferritin was determined by enzyme linked immuno sorbent assay technique. Serum iron and Total iron binding capacity was determined by spectrophometric method. The mean serum ferritin concentration was elevated in the sickle cell anaemia patients whose multiple transfusions (MT) are more than those who were rarely transfused (RT) as compared with the control groups (p < 0.001). There was a positive correlation between the serum ferritin and the number of units of blood transfused (r = 0.719, p = 0.000). This study revealed that a high level of serum ferritin, percentage transferrin saturation and a reduction in total iron binding capacity were observed in sickle cell anaemia patients who received ≥3 units of packed cells in one year.展开更多
Background: Hepatitis C is an infectious disease of the liver caused by the Hepatitis C virus (HCV) resulting to a chronic Hepatitis. Chronic HCV infection constitutes a serious health challenge in places where preval...Background: Hepatitis C is an infectious disease of the liver caused by the Hepatitis C virus (HCV) resulting to a chronic Hepatitis. Chronic HCV infection constitutes a serious health challenge in places where prevalence is substantial. In Nigeria, there is a high risk because donor blood is not routinely screened for HCV. Patients with sickle cell anaemia (SCA) are considered a subset of the population at higher risk of acquiring the virus, due to their frequent needs for transfusion of blood and its products. However, the magnitude of HCV infection has not been adequately measured in our general population and specific data on HCV in SCA patients are scanty, hence a prospective case controlled study to determine the prevalence of HCV antibodies in transfused SCA patients attending the sickle cell anaemia clinic in the University of Ilorin Teaching Hospital (UITH), Ilorin was taken. Objective: To determine the prevalence of Hepatitis C virus antibodies among transfused children with SCA in Ilorin. Subjects and Method: Eighty two transfused SCA children aged 6 months to 14 years were recruited consecutively from February 2008 to January 2009 while eighty four non transfused SCA children of the same age range recruited over the same period served as controls. Hepatitis C virus antibody screening was done using a second generation ELISA method. Results: The overall prevalence of HCV antibody was 3.0%, while it was 3.7% and 2.4% in the transfused and non transfused SCA patients respectively (χ2 = 0.23, p = 0.68). The patients were also comparable across the social class when subcategorized into high and low social class (χ2 = 0.37, p = 1.00 (subjects), χ2 = 0.42, p = 1.00 (controls). Conclusion: The prevalence of Hepatitis C virus anti- bodies in transfused SCA patients is low. The difference in prevalence between transfused and nontransfused SCA patient was not statistically significant. This was cautiously interpreted due to the hospital based premise of the work. Therefore, Hepatitis C virus antibody acquisit展开更多
Haemoglobinopathies are group of diseases characterized by abnormalities both quantitative and qualitative in the synthesis of haemoglobin. Haemoglobinopathies consist of sickle cell anaemia (SCA), thalassaemia (βT) ...Haemoglobinopathies are group of diseases characterized by abnormalities both quantitative and qualitative in the synthesis of haemoglobin. Haemoglobinopathies consist of sickle cell anaemia (SCA), thalassaemia (βT) and variant haemoglobins. In India, they are responsible for the largest number of genetic disorders and hence are of great public health hazardous. In India major concerned haemoglobinopathic disorders are sickle cell anaemia and β-thalassaemia. Of the several abnormal haemoglobin molecules, four which are widely prevalent in India include: HbS, HbβT, HbE and HbD. Examination of 6463 individuals showed high incidences for haemoglobin variants, HbS and HbβT in different ethnic groups, the frequency being varies from 0% - 20% and 0% - 9% respectively. The frequency of HbS in Brahmins is 4.17%, in Kalar 5.41%, in Rajput 2.04%, in Muslims 3.73% in Maratha 2.08% in Bania 9.09% while in Teli it is 3.65%. Among the Scheduled castes and Nomadic tribal groups HbS ranges from 1% - 12%;in backward caste categories it varies from 3% - 16%;while in Scheduled tribes it ranges from 0% - 20%. The high magnitude of sickle cell trait has been noticed in the Pardhan (20.31%) followed by the Marar (16.10%), the Dhiwar (11.90%), the Gond (11.89%), the Mahar (11.81%) and the Bania (9.90%). A considerable high frequency (9.27%) of β-thalassaemia has been observed among the Sindhi population. Sporadic occurrence of HbβT and HbD among other communities suggested the gradual spread of the genes into the region. The present findings in 11 communities with the thalassaemia syndrome suggest that the β-thalassaemia is accompanied by raised level of HbA2. Unusual greater mean RBC and WBC suggest the high concentration of hypochromic microcytosis in anaemia. The mean MCV and MCH in HbβT and HbD are much lower than the normal ranges compared to HbS. The mean MCHC is much lower in HbβT, HbDD and HbS than the normal range. The cumulative gene frequency of haemoglobinopathies in India is 4.2%. With a population of over 1 billi展开更多
Sickle cell disease (SCD) has been regarded as an inflammatory and pro-?coagulatory disease with profound cardiovascular abnormalities including propensity for ventricular arrhythmogenesis. Tp-e and Tpe/QTc ratio howe...Sickle cell disease (SCD) has been regarded as an inflammatory and pro-?coagulatory disease with profound cardiovascular abnormalities including propensity for ventricular arrhythmogenesis. Tp-e and Tpe/QTc ratio however has?been proposed as better indicators of arrythmogenesis and has?been shown to be prolonged in many inflammatory conditions and correlate with levels of inflammatory markers. However, correlation between Tpe/QTc ratio and the level of highly sensitive C-reactive protein (hs-CRP) and plasminogen activator inhibitor (PAI) have not been reported in SCD. This study aims at evaluating Tp-e Interval and Tp-e/QTc ratio among steady state Sickle cell disease patients in relationship to the degree of anaemia, inflammatory and profibrotic markers.?Methodology:?A cross-sectional hospital-based study comprises?30 sickle cell anaemia patients in steady state with an?equal number of controls having genotype HbAA and HbAS respectively.Clinical, laboratory and ECG parameters were obtained.?Results:?A total of 90 participants?are?with mean age 24.2?±?5.6. The study showed that sickle cell disease patient had significantly lower level of PCV and higher level of PAI, platelet and total white cell count (p value??0.05). C-reactive protein was also higher in them. 76.7% of HbSS patients had abnormal ECG. QTc and Tp-e were also prolonged in sickle cell disease patients compared with controls. An association was found between the level of PCV, PAI and prolonged Tp-e and QTc.?Conclusion:?Sickle cell disease patients have higher levels of inflammatory markers and abnormal ECG patterns are common in them. Moreover, the levels of these inflammatory markers correlate with Tp-e parameters.展开更多
Objective:Human Parvovirus B19 is known to cause significant morbidity and mortality and among diverse patient population groups.Among patients with sickle all disease,who have high red cell turn over due to chronic h...Objective:Human Parvovirus B19 is known to cause significant morbidity and mortality and among diverse patient population groups.Among patients with sickle all disease,who have high red cell turn over due to chronic haemolysis,infection with parvovirus B19 can cause severe life threatening transient aplastic crisis.This study was conducted to determine the Seroprevalence of parvovirus B19 infection and to provide basic epidemiological data on parvovirus B19 infection among children with sickle cell anaemia in Jos,north central Nigeria.Methods: In a hospital based cross sectional survey,200 children aged 1-18 years confirmed to have sickle cell anaemia attending the paediatric sickle cell clinic of Jos university teaching hospital were studied.A questionnaire was designed to obtain basic socio-demographic information,& other relevant aspect of patients′ history.Blood samples were taken for anti parvo-virus B19 serology.Results: The over all prevalence rates of parvo-virus B19 immunoglobulin(IgG) and immunoglobulin(IgM) antibodies were 39.5% and 3.5% respectively,Conclusion: This study confirms that parvo-virus B19 infection is prevalent among children with sickle cell anaemia in Jos,North Central Nigeria.There is a need for further studies to fully evaluate the clinical impact of the infection on our sickle cell anaemia patients.展开更多
SCD is one of the most prevailing homogeneous inherited haemoglobinopathies causing a plethora of various clinical complications to the patients. The high mortality and morbidity severely concern the Western community...SCD is one of the most prevailing homogeneous inherited haemoglobinopathies causing a plethora of various clinical complications to the patients. The high mortality and morbidity severely concern the Western community, where numerous clinical trials and research for a cure are in process. In order to alleviate patients from the severe symptoms of the disease, avoiding the side effects, Botanical Medicine exhibits concrete evidence, as a gold candidate, to be the salvation to the problem. The Preferred Reporting Items for Systematic Review (PRISMA) protocol has been used to achieve extensive research on the topic, focusing on the identification and evaluation of the phytochemical properties of common medicinal plants. Meta-analysis has also been implemented on the results of published literature. Forest plots have been plotted, comparing and evaluating the results’ validity and significance. The meta-analysis results have undoubtedly demonstrated the importance and significance of the medicinal plants and their properties against various clinical complications, focusing on the pathogenicity of SCD. Surprisingly, their effectiveness to suppress haemoglobin polymerisation and increase the Fe<sup>2+</sup>/Fe<sup>3+</sup> ratio in patients, enhanced the normal morphological erythrocytes’ appearance by suppressing the sickle shape of drepanocytes. Research made on the epidemiology of SCD associates the disease with the geographical frequency of malaria infection. Based on the natural selection theory of Charles Darwin, nature aids in the population’s survival by the endemicity of various medicinal plants in areas with increased SCD patients. Limitations to the medicinal plants’ consumptions and further therapeutic options have been discussed.展开更多
文摘Background Aim: This article aimed to sensitize the healthcare providers who care for patients with sickle cell disease (SCD) during pregnancy, to the multiple causes of jaundice in sickle hepatopathy, and sift the maze in establishing the dominant cause. Case Presentation: This is a case of a 28-yr-old Gravida 1 Para 0 + 0 health worker, with sickle cell anaemia and background history of peptic ulcer disease, total right hip replacement and previous multiple blood transfusions due to haemolytic crisis. She presented with upper abdominal pain and progressively deepening jaundice. There was minimal relief of the abdominal pain with anti-ulcer medications. She had tender hepatomegaly, positive Murphy’s sign and deranged liver function parameters. She was also positive for viral hepatitis B markers. A remarkable finding on abdominal ultrasonography was that of a gallbladder with normal wall thickness and multiple stones. She was managed conservatively on intravenous fluids, antibiotics and Livolin forte (Phosphatidylcholine) with improved liver function parameters within a week. Conclusion: Since sickle cell hepatopathy is multifactorial, the importance of unravelling the dominant cause of jaundice or liver dysfunction in these patients and the need for immediate intervention are necessary for effective and targeted care, hence this case report. Our patient had background chronic hepatitis B with a superadded acute cholecystitis with non-obstructing gallstones, and possible subsequent bacterial hepatitis, which responded to antibiotics. The most likely dominant cause of jaundice was chronic hepatitis B that was precipitated by acute cholecystitis.
文摘Objectives: The study is aimed at determining that errors in assigning genotypes to intending couples do exist;and the impact of these errors on parents. Methods: The study was conducted at the children clinics in Enugu and Abakiliki, south east Nigeria. It is a cross-sectional retrospective study in which a review of the records of all the children attending 2 private clinics in Enugu and Abakaliki of Enugu and Ebonyi states respectively, over a 3-year period was done. Results: A total of 6006 children attended the children clinics over the study period. Twenty three (23) of them had sickle cell anaemia. Out of the 23 cases 10 (43.5%) were males and 13 (56.5%) were females. Male to female ratio was 1:1.3. The commonest features were abdominal and leg pains, involving 20 (87%) and 21 (91.3 %) respectively. Among the 23 parents that had their genotypes repeated, 9 males had different genotypes from what they had during courtship or before marriage. The genotypes of all the females that could recall their genotypes before marriage or during courtship were the same after a repeat test. Conclusions: There exist errors in assigning genotypes to parents which at the end made them have children with sickle cell anemia, unwittingly, with serious consequences bordering on strained relationship between the parents with the children bearing the brunt.
文摘Background: Nigeria contributes 30% to the global sickle cell anaemia burden. Cholelithiasis occurs commonly in children with sickle cell anaemia and may remain undiagnosed, mimic abdominal vaso-occlusive crises or become complicated by calculus cholecystitis. Early identification of children with sickle cell anaemia who have Cholelithiasis reduces the chances of misdiagnosis, mistreatment and improves outcomes. There is limited knowledge about the prevalence of Cholelithiasis among Nigerian children with sickle cell anaemia. The present study aimed to determine the prevalence of Cholelithiasis in children with sickle cell anaemia in Gombe, northeast Nigeria. Methodology: This was a cross-sectional analysis of children with sickle cell anaemia being followed-up at the sickle cell centre of the Federal Teaching Hospital, Gombe, northeast Nigeria. We consecutively recruited 294 children aged 2 - 17 years while in steady state from June to November 2017. Quantitative data were collected from parents/caregivers via a researcher administered questionnaire and cholecystosonography performed after a minimum of 8-hour fast. Predictors of Cholelithiasis were examined using binary logistic regression. Results: The mean age of children with sickle cell anaemia was 9.0 ± 4.5 years. The prevalence of Cholelithiasis in children with sickle cell anaemia in steady state was 4.8%. Children aged 15 - 17 years had 12 times higher odds of having Cholelithiasis [AOR = 12.268 (95% CI = 1.3 - 112.8)]. Conclusion: The prevalence of Cholelithiasis in children with sickle cell anaemia though generally low, increases progressively with age to peak during middle to late adolescence.
文摘Vaso-occlusive crisis is a common clinical manifestation in children with sickle cell anaemia, frequently warranting in-patient management with intravenous fluids, analgesics and occasionally anti-malaria and antibiotics. Pain is the principal symptom of vaso-occlusive crisis and it can affect any part of the body. Pentazocine is a well-known opioid analgesic, commonly used in the treatment of painful crisis in children affected by sickle cell anaemia. It was discovered in the 1950s and was initially thought to be addiction—proof, but this has been disproved over the years by reports of pentazocine dependence/addiction especially in patients living with chronic pain. There have been some case reports of pentazocine abuse/dependence among sickle cell disease patients in the literature, most of which are from Nigeria but none involved joint addiction by siblings. We therefore, report the case of two siblings, known sickle cell anaemia (HbSS) patients, referred to our emergency paediatric unit with history of recurrent painful crisis over a 10-month period leading to abuse of pentazocine. They were jointly managed successfully by the paediatric hematology and the pain and palliative units of the University of Ilorin Teaching Hospital and helped to overcome their abuse and addiction.
文摘Background: Sickle cell anaemia (SCA) is a serious, multisystem, genetic disorder affecting millions of children worldwide. The disease causes numerous complications that interfere with the health-related quality of life (HRQoL) of these children including an impact on educational, physical and psychosocial development. Few studies have described the clinical spectrum and quality of life of children with SCA living in a low-resource area. Objectives: This study aimed to determine the clinical spectrum and HRQoL among children living with sickle cell anaemia (SCA) in northwest Tanzania. Methods: This hospital-based cross-sectional study took place at Tertiary and teaching hospital, Bugando Medical Centre, Mwanza Tanzania. The study enrolled children ages 2 - 12 years old with SCA attending the Bugando Medical Centre sickle cell clinic. Health related quality of life was measured using the Pediatric Quality of Life, Brief Generic Core Scale after translating from English into a Swahili version. Important SCA complications were assessed using a structured questionnaire. Results: From October 2016 to March 2017, 204 children were enrolled. Participants presented at a median age of 6 years [IQR 4 - 9]. Among children with SCA the most common clinical signs at the time of enrolment were pale in 69.6% (142/204), jaundice in 65.9% (134/204), oxygen saturation 90% in 25% (51/204) and splenomegaly in 19% (39/204). Severe anaemia was observed in 30.9% (63/204). A majority reported vaso-occlusive crisis (166/204, 81.4%), and very few had experienced a prior stroke (5/204, 2.5%). Using a modified Likert scale, a total of 41/204 (20.1%) children had poor HRQoL indicated by low scores on PedsQL<sup>TM</sup> and 163/204 (79.9%) children had high scores, indicating good HRQoL. On multivariate analysis, age ≥ 5 years (p-value < 0.001), haemoglobin < 7 g/dl (p-value = 0.001) and >3 hospitalizations per year (p-value = 0.008) were associated with poor HRQoL. Conclusion: SCA complications, negatively impact the HRQoL of children l
文摘This study was aimed at evaluating the iron status of multiple blood transfused sickle cell anaemia (SCA) patients in Benin City, Nigeria. A total of 86 subjects participated in the study, comprising of 30 multiple transfused SCA subjects, 30 of rarely transfused SCA subjects and 26 of age and sex matched healthy control subjects. Serum ferritin was determined by enzyme linked immuno sorbent assay technique. Serum iron and Total iron binding capacity was determined by spectrophometric method. The mean serum ferritin concentration was elevated in the sickle cell anaemia patients whose multiple transfusions (MT) are more than those who were rarely transfused (RT) as compared with the control groups (p < 0.001). There was a positive correlation between the serum ferritin and the number of units of blood transfused (r = 0.719, p = 0.000). This study revealed that a high level of serum ferritin, percentage transferrin saturation and a reduction in total iron binding capacity were observed in sickle cell anaemia patients who received ≥3 units of packed cells in one year.
文摘Background: Hepatitis C is an infectious disease of the liver caused by the Hepatitis C virus (HCV) resulting to a chronic Hepatitis. Chronic HCV infection constitutes a serious health challenge in places where prevalence is substantial. In Nigeria, there is a high risk because donor blood is not routinely screened for HCV. Patients with sickle cell anaemia (SCA) are considered a subset of the population at higher risk of acquiring the virus, due to their frequent needs for transfusion of blood and its products. However, the magnitude of HCV infection has not been adequately measured in our general population and specific data on HCV in SCA patients are scanty, hence a prospective case controlled study to determine the prevalence of HCV antibodies in transfused SCA patients attending the sickle cell anaemia clinic in the University of Ilorin Teaching Hospital (UITH), Ilorin was taken. Objective: To determine the prevalence of Hepatitis C virus antibodies among transfused children with SCA in Ilorin. Subjects and Method: Eighty two transfused SCA children aged 6 months to 14 years were recruited consecutively from February 2008 to January 2009 while eighty four non transfused SCA children of the same age range recruited over the same period served as controls. Hepatitis C virus antibody screening was done using a second generation ELISA method. Results: The overall prevalence of HCV antibody was 3.0%, while it was 3.7% and 2.4% in the transfused and non transfused SCA patients respectively (χ2 = 0.23, p = 0.68). The patients were also comparable across the social class when subcategorized into high and low social class (χ2 = 0.37, p = 1.00 (subjects), χ2 = 0.42, p = 1.00 (controls). Conclusion: The prevalence of Hepatitis C virus anti- bodies in transfused SCA patients is low. The difference in prevalence between transfused and nontransfused SCA patient was not statistically significant. This was cautiously interpreted due to the hospital based premise of the work. Therefore, Hepatitis C virus antibody acquisit
文摘Haemoglobinopathies are group of diseases characterized by abnormalities both quantitative and qualitative in the synthesis of haemoglobin. Haemoglobinopathies consist of sickle cell anaemia (SCA), thalassaemia (βT) and variant haemoglobins. In India, they are responsible for the largest number of genetic disorders and hence are of great public health hazardous. In India major concerned haemoglobinopathic disorders are sickle cell anaemia and β-thalassaemia. Of the several abnormal haemoglobin molecules, four which are widely prevalent in India include: HbS, HbβT, HbE and HbD. Examination of 6463 individuals showed high incidences for haemoglobin variants, HbS and HbβT in different ethnic groups, the frequency being varies from 0% - 20% and 0% - 9% respectively. The frequency of HbS in Brahmins is 4.17%, in Kalar 5.41%, in Rajput 2.04%, in Muslims 3.73% in Maratha 2.08% in Bania 9.09% while in Teli it is 3.65%. Among the Scheduled castes and Nomadic tribal groups HbS ranges from 1% - 12%;in backward caste categories it varies from 3% - 16%;while in Scheduled tribes it ranges from 0% - 20%. The high magnitude of sickle cell trait has been noticed in the Pardhan (20.31%) followed by the Marar (16.10%), the Dhiwar (11.90%), the Gond (11.89%), the Mahar (11.81%) and the Bania (9.90%). A considerable high frequency (9.27%) of β-thalassaemia has been observed among the Sindhi population. Sporadic occurrence of HbβT and HbD among other communities suggested the gradual spread of the genes into the region. The present findings in 11 communities with the thalassaemia syndrome suggest that the β-thalassaemia is accompanied by raised level of HbA2. Unusual greater mean RBC and WBC suggest the high concentration of hypochromic microcytosis in anaemia. The mean MCV and MCH in HbβT and HbD are much lower than the normal ranges compared to HbS. The mean MCHC is much lower in HbβT, HbDD and HbS than the normal range. The cumulative gene frequency of haemoglobinopathies in India is 4.2%. With a population of over 1 billi
文摘Sickle cell disease (SCD) has been regarded as an inflammatory and pro-?coagulatory disease with profound cardiovascular abnormalities including propensity for ventricular arrhythmogenesis. Tp-e and Tpe/QTc ratio however has?been proposed as better indicators of arrythmogenesis and has?been shown to be prolonged in many inflammatory conditions and correlate with levels of inflammatory markers. However, correlation between Tpe/QTc ratio and the level of highly sensitive C-reactive protein (hs-CRP) and plasminogen activator inhibitor (PAI) have not been reported in SCD. This study aims at evaluating Tp-e Interval and Tp-e/QTc ratio among steady state Sickle cell disease patients in relationship to the degree of anaemia, inflammatory and profibrotic markers.?Methodology:?A cross-sectional hospital-based study comprises?30 sickle cell anaemia patients in steady state with an?equal number of controls having genotype HbAA and HbAS respectively.Clinical, laboratory and ECG parameters were obtained.?Results:?A total of 90 participants?are?with mean age 24.2?±?5.6. The study showed that sickle cell disease patient had significantly lower level of PCV and higher level of PAI, platelet and total white cell count (p value??0.05). C-reactive protein was also higher in them. 76.7% of HbSS patients had abnormal ECG. QTc and Tp-e were also prolonged in sickle cell disease patients compared with controls. An association was found between the level of PCV, PAI and prolonged Tp-e and QTc.?Conclusion:?Sickle cell disease patients have higher levels of inflammatory markers and abnormal ECG patterns are common in them. Moreover, the levels of these inflammatory markers correlate with Tp-e parameters.
文摘Objective:Human Parvovirus B19 is known to cause significant morbidity and mortality and among diverse patient population groups.Among patients with sickle all disease,who have high red cell turn over due to chronic haemolysis,infection with parvovirus B19 can cause severe life threatening transient aplastic crisis.This study was conducted to determine the Seroprevalence of parvovirus B19 infection and to provide basic epidemiological data on parvovirus B19 infection among children with sickle cell anaemia in Jos,north central Nigeria.Methods: In a hospital based cross sectional survey,200 children aged 1-18 years confirmed to have sickle cell anaemia attending the paediatric sickle cell clinic of Jos university teaching hospital were studied.A questionnaire was designed to obtain basic socio-demographic information,& other relevant aspect of patients′ history.Blood samples were taken for anti parvo-virus B19 serology.Results: The over all prevalence rates of parvo-virus B19 immunoglobulin(IgG) and immunoglobulin(IgM) antibodies were 39.5% and 3.5% respectively,Conclusion: This study confirms that parvo-virus B19 infection is prevalent among children with sickle cell anaemia in Jos,North Central Nigeria.There is a need for further studies to fully evaluate the clinical impact of the infection on our sickle cell anaemia patients.
文摘SCD is one of the most prevailing homogeneous inherited haemoglobinopathies causing a plethora of various clinical complications to the patients. The high mortality and morbidity severely concern the Western community, where numerous clinical trials and research for a cure are in process. In order to alleviate patients from the severe symptoms of the disease, avoiding the side effects, Botanical Medicine exhibits concrete evidence, as a gold candidate, to be the salvation to the problem. The Preferred Reporting Items for Systematic Review (PRISMA) protocol has been used to achieve extensive research on the topic, focusing on the identification and evaluation of the phytochemical properties of common medicinal plants. Meta-analysis has also been implemented on the results of published literature. Forest plots have been plotted, comparing and evaluating the results’ validity and significance. The meta-analysis results have undoubtedly demonstrated the importance and significance of the medicinal plants and their properties against various clinical complications, focusing on the pathogenicity of SCD. Surprisingly, their effectiveness to suppress haemoglobin polymerisation and increase the Fe<sup>2+</sup>/Fe<sup>3+</sup> ratio in patients, enhanced the normal morphological erythrocytes’ appearance by suppressing the sickle shape of drepanocytes. Research made on the epidemiology of SCD associates the disease with the geographical frequency of malaria infection. Based on the natural selection theory of Charles Darwin, nature aids in the population’s survival by the endemicity of various medicinal plants in areas with increased SCD patients. Limitations to the medicinal plants’ consumptions and further therapeutic options have been discussed.
