{"title":"Pathophysiologic basis of haemolysis in patients with sickle cell disease in steady state and in hyperhaemolytic states: Aetiopathogenesis, management, and mitigation","authors":"Sagir G. Ahmed, U. Ibrahim","doi":"10.4103/njbcs.njbcs_55_22","DOIUrl":"https://doi.org/10.4103/njbcs.njbcs_55_22","url":null,"abstract":"Sickle cell disease (SCD) is characterized by red cell sickling, tissue infarcts, pain and haemolysis. Haemolysis leads to anaemia, transfusion and vasculopathic multi-organ damage (VMOD). Every SCD patient maintains a chronic steady state haemolysis (SSH), which is often aggravated to hyperhaemolysis (HH) by inherited and/or acquired comorbidities. Hence, this article aims to present an updated and comprehensive narrative literature review of aetiopathogenesis, management and mitigation of SCD haemolysis in steady state and in various hyperhaemolytic states. Literature search revealed SSH is initiated by steady state sickling due to tissue hypoxia and is driven by lactic acidemia, Bohr effect, low pyruvate kinase activity, reduced oxygen affinity of HbS, lipid peroxidation, eryptosis, senescence antigen expression, Fc-receptor or ligand mediated erythro-phagocytosis, xanthine oxidase (XO) hyperactivity and intravascular red cells lysis. SSH is often aggravated to chronic or acute HH by various acquired and/or inherited haemolytic comorbidities such as G6PD deficiency, hereditary spherocytosis (HS), acute/chronic hypersplenic or acute hepatic sequestration, infective erythrocytotropism and erythrocytopathy, haemophagocytic syndrome, transfusion reaction, alloimmune, autoimmune and drug-induced haemolysis. While transfusion provides short-term solution for severe haemolysis and anaemia in SCD, long-term solution must include mitigation of haemolysis by using HbF enhancers, HbS oxygen affinity modifiers, XO inhibitors, immune modulators for immune-haemolysis, use of anti-oxidants to minimize peroxidation, avoidance of oxidants if patient is also G6PD deficient, administering antibiotics/vaccinations to treat/prevent infections, splenectomy for comorbid HS or any recalcitrant hypersplenic splenomegaly. This narrative review underscores importance of managing SSH and HH in order to alleviate anaemia, minimize transfusion, and prevent VMOD in SCD.","PeriodicalId":19224,"journal":{"name":"Nigerian Journal of Basic and Clinical Sciences","volume":"25 1","pages":"10 - 23"},"PeriodicalIF":0.1,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"81565313","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}
{"title":"VACTERL association in Kano, North-West Nigeria: A case report","authors":"I. Ahmadu, M. Abubakar, M. Asani, I. Aliyu","doi":"10.4103/njbcs.njbcs_1_22","DOIUrl":"https://doi.org/10.4103/njbcs.njbcs_1_22","url":null,"abstract":"VACTERL association is a term used to describe the simultaneous occurrence of at least 3 of the following congenital malformations: vertebral anomalies, anal atresia, cardiac malformation, tracheo-oesophageal fistula, renal dysplasia, and limb abnormalities. Most cases occur sporadically with an estimated incidence rate of 1 in 10,000 – 40,000 births. We report the case of an 11-month-old infant that presented with a complaint of recurrent cough and difficulty in breathing since 1 month of life. There is also the history of delayed motor developmental milestones. Physical examination and investigations revealed the presence of VACTERL association (bilateral talipes equinovarus deformity, multiple cardiac shunt lesions, and malformation of the 5th lumbar vertebrae with dural ectasia and lumbosacral scoliosis). She has been on oral diuretics and is currently on follow-up in pediatric cardiology, orthopedic, and neurosurgery clinics. This case report highlights the presence of a rare case of the VACTERL association in Kano, North-West Nigeria.","PeriodicalId":19224,"journal":{"name":"Nigerian Journal of Basic and Clinical Sciences","volume":"1 1","pages":"95 - 97"},"PeriodicalIF":0.1,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"83959700","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}
Abdullah Habib, Usman Abba, H. Abdullahi, S. Ibrahim
{"title":"Prevalence of thyroid dysfunction in women with dysfunctional uterine bleeding in Kano, North-Western Nigeria","authors":"Abdullah Habib, Usman Abba, H. Abdullahi, S. Ibrahim","doi":"10.4103/njbcs.njbcs_2_23","DOIUrl":"https://doi.org/10.4103/njbcs.njbcs_2_23","url":null,"abstract":"Context: Thyroid hormones play a key role in the menstrual and reproductive functions of women. Thyroid dysfunction is marked by a large number of menstrual aberrations. Objective: To determine the incidence of thyroid dysfunction in dysfunctional uterine bleeding (DUB) and its correlation with menstrual patterns. Materials and Methods: The study population consisted of 64 DUB patients aged between 19 and 45 years and an equal number of age-matched women with a normal menstrual cycle. Plasma concentrations of thyroid-stimulating hormone (TSH), free thyroxine (T4), and free triiodothyronine (T3) of the participants were measured. Laboratory analyses were conducted on Elecsys 2010 immunology analyzer. Statistical analysis was performed with Microsoft Excel software, and the data were transferred into IBM SPSS version 21.0 for processing. A P-value of <0.05 was considered significant. Results: Among the 64 women with DUB, 21.9% had thyroid dysfunction, of which 14.1% had overt hypothyroidism, 6.2% had subclinical hypothyroidism, and 1.5% had overt hyperthyroidism. The main menstrual complaint among hypothyroid DUB patients was menorrhagia. The only presentation in hyperthyroidism was oligomenorrhea. Conclusion: The prevalence of abnormal thyroid function in this study was high; therefore, screening of DUB patients for thyroid function abnormalities is suggested.","PeriodicalId":19224,"journal":{"name":"Nigerian Journal of Basic and Clinical Sciences","volume":"22 1","pages":"51 - 56"},"PeriodicalIF":0.1,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"87901104","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}
{"title":"Computed tomographic study on the prevalence of the Onodi cell","authors":"B. Ominde, J. Ikubor, P. Igbigbi","doi":"10.4103/njbcs.njbcs_20_22","DOIUrl":"https://doi.org/10.4103/njbcs.njbcs_20_22","url":null,"abstract":"Context: The prevalence of the Onodi cell varies in different populations. Its involvement in sinus pathology is characterized by varied clinical symptoms due to its proximity to the internal carotid artery and optic nerve. Endoscopic surgical management of sinus pathologies poses a great risk of injury to these neurovascular structures. Aim: This study elucidates the prevalence of the Onodi cell in a Nigerian Teaching Hospital. Setting and Design: This was a retrospective cross-sectional study conducted in the Radiology unit of a Teaching Hospital in Nigeria. Materials and Methods: Brain computed tomography (CT) images of 336 patients aged ≥20 years were evaluated for the presence of the Onodi cell. The study was approved by the institution before accessing the digital images. The age and gender of patients and the presence and location of the Onodi cell were recorded. Statistical Analysis Used: Data were entered in Statistical Package for Social Sciences version 23 for analysis of prevalence and subsequent comparisons using the Chi-square test. The significance level was considered at P < 0.05. Results: The prevalence of Onodi cell was 16.4% and was only observed unilaterally with a slightly lower frequency on the right (27, 8.0%) than the left (28, 8.3%). The prevalence did not show any significant side or gender differences (P > 0.05). Conclusion: The Onodi cell was present in the studied population, thus highlighting the need for their preoperative recognition on CT to abate iatrogenic complications during endoscopic surgeries.","PeriodicalId":19224,"journal":{"name":"Nigerian Journal of Basic and Clinical Sciences","volume":"31 1","pages":"30 - 33"},"PeriodicalIF":0.1,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"81529771","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}
{"title":"Diagnostic upper gastrointestinal endoscopy in Kaduna, Northwest Nigeria: Indications and findings","authors":"H. Yahya","doi":"10.4103/njbcs.njbcs_1_23","DOIUrl":"https://doi.org/10.4103/njbcs.njbcs_1_23","url":null,"abstract":"Context: An upper gastrointestinal endoscopy (UGIE) is now a routine procedure and is a guide to the prevalence and nature of gastrointestinal tract diseases in an area. Aim: To report indications and findings for UGIE in a tertiary hospital in Kaduna, Nigeria, and compare with past reports in the region and from the rest of Nigeria and Africa. Settings and Design: A retrospective review of endoscopy records (2014–2021) of consecutive patients referred to the endoscopy unit of Barau Dikko Teaching Hospital, Kaduna, Nigeria. Statistical Analysis: The data were analyzed using IBM SPSS version 22. Results: Over seven years, 1,525 patients had diagnostic UGIE: mean age = 44.8 years (range 8–100), 61% ≥ 40 years, and 55.7% females. Dyspepsia (70%) and hematemesis/melena (14%) were the most common indications. Superficial gastritis (60%) was the most common endoscopic finding. Peptic ulcer disease (PUD) was diagnosed in 9.6% of patients: Gastric ulcer (6.6%) was more common than duodenal ulcer (3.6%) and more frequent in males than females (9.5% vs. 4.3%, P = 0.000) and in those ≥ 40 years compared with < 40 years (7.8% vs 5%, P = 0.032). Duodenal ulcer was also more common in males (6% vs 1.8%, P = 0.000). Erosive gastritis (20.5%), hiatus hernia (14.4%), esophagitis (13.7%), atrophic gastritis (8.3%), nodular gastritis (6.6%), esophageal varices (6.1%), and gastric carcinoma (1.2%) were other findings. There was no abnormality detected in 6% of patients. Conclusion: We reported a marked change in the pattern of some gastroduodenal diseases in our region. The patients are older and predominantly female. The prevalence of PUD has markedly declined with gastric ulcer now almost twice as common as duodenal ulcer.","PeriodicalId":19224,"journal":{"name":"Nigerian Journal of Basic and Clinical Sciences","volume":"106 1","pages":"82 - 88"},"PeriodicalIF":0.1,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"77185862","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}
Y. Akhtar, M. Khan, Muhammad S Khan, M. Malik, Gulalai Rehman, Seemin Kashif
{"title":"Relationship of serum adiponectin levels with lipid profile in diabetic and non-diabetic pregnant women","authors":"Y. Akhtar, M. Khan, Muhammad S Khan, M. Malik, Gulalai Rehman, Seemin Kashif","doi":"10.4103/njbcs.njbcs_40_22","DOIUrl":"https://doi.org/10.4103/njbcs.njbcs_40_22","url":null,"abstract":"Context: Adiponectin levels correlate positively with high-density lipoprotein (HDL), and negatively with serum triglyceride (TAG), low-density lipoprotein (LDL), and serum total cholesterol (TC). Aims: To find the relationship of serum adiponectin levels with lipid profiles in diabetic and non-diabetic pregnant women. Settings and Design: The study was performed in four public hospitals and one private tertiary care hospital in Peshawar, Pakistan. This study was a cross-sectional study. Methods and Material: Group A included 100 non-diabetic pregnant women, and Group B included 100 women with gestational diabetes mellitus. Inclusion criteria were a single fetus, 24 to 40 weeks pregnancy, with or without gestational diabetes. Exclusion criteria were multiple pregnancies, pre-existing diabetes, and other comorbidities. The serum adiponectin level and lipid profile of both groups were measured. Statistical Analysis Used: Descriptive analysis of all the variables was done. Independent-sample T-tests, Pearson's correlations, and binary logistic regression analyses were performed on SPSS version 20. Results: Adiponectin level was significantly lower in Group B (2.17 ± 1.84 μg/mL) as compared to Group A (9.94 ± 4.81 μg/mL). HDL level was significantly lower than normal in Group B. TC and LDL levels were higher than normal in both groups. A statistically significant relationship was found between adiponectin and HDL only. Binary logistic regression analysis showed that low adiponectin levels could predict diabetes in pregnancy. Conclusions: Regression analysis could not establish any relationship between adiponectin and lipid profile. However, adiponectin levels could show a relationship with the diabetic group. Hence adiponectin levels could possibly prove to be a biomarker of GDM.","PeriodicalId":19224,"journal":{"name":"Nigerian Journal of Basic and Clinical Sciences","volume":"53 1","pages":"46 - 50"},"PeriodicalIF":0.1,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"83412767","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}
Abdullah Aloboudi, A. Al Hammad, Jaffar AlFaraj, Ali Al Hussain
{"title":"Correlation between skeletal manifestations of sickle cell disease (SCD) and transcranial doppler imaging velocities","authors":"Abdullah Aloboudi, A. Al Hammad, Jaffar AlFaraj, Ali Al Hussain","doi":"10.4103/njbcs.njbcs_57_22","DOIUrl":"https://doi.org/10.4103/njbcs.njbcs_57_22","url":null,"abstract":"Context: Sickle cell disease (SCD), a genetic disorder, is a debilitating illness. Among the different complications of SCD, cerebrovascular complication results in stroke in pediatric population. The cause of stroke in those patients is attributed to vasculopathy, stenosis, or vaso-occlusion of distal internal carotid arteries. Another body system that is commonly affected is the musculoskeletal system. Both skeletal manifestations and vasculopathy in Circle of Willis in SCD are known consequences of the same underlying pathology. Aim: This study was conducted to assess the correlation between osseous findings and vasculopathy of Circle of Willis. Settings and Design: It was a chart review study conducted during December 2021 to January 2022. Children suffering from SCD were the study population. Materials and Methods: The sampling was done by using convenience purposive sampling technique. The data were collected as observations using picture archiving and communication system and then entered Excel spreadsheet. Statistical Analysis Used: The data were analyzed using SPSS, Version 28. The descriptive statistics including frequencies and percentages for the skeletal manifestations and transcranial Doppler imaging (TCDI) readings were calculated. To assess the association between skeletal manifestations and TCDI, a Chi-square test of independence was performed. Results: The findings showed that abnormal TCDI readings exhibited higher frequency on abnormal skeletal manifestations as compared to normal TCDI readings. Conclusion: The study showed that an osseous manifestation of disease was associated with vasculopathy of Circle of Willis vasculature. Therefore, osseous manifestations of SCD can be associated with the chance of stroke among the children since it is associated with the vasculopathy of Circle of Willis.","PeriodicalId":19224,"journal":{"name":"Nigerian Journal of Basic and Clinical Sciences","volume":"9 1","pages":"66 - 69"},"PeriodicalIF":0.1,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"82231576","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}
Syed Faisal Iradat, M. Uddin, S. Nabi, Zaheeruddin Asif
{"title":"COVID-19: An evidence-based and disaster response methodology","authors":"Syed Faisal Iradat, M. Uddin, S. Nabi, Zaheeruddin Asif","doi":"10.4103/njbcs.njbcs_49_22","DOIUrl":"https://doi.org/10.4103/njbcs.njbcs_49_22","url":null,"abstract":"The world is gradually getting out of the grip of COVID-19 pandemic, although there are still high number of cases in some countries. Most of the initial attempts to predict and project the course of pandemic were hypothetical or based on historical data, as no current and specific data were available in the early days of pandemic. Most governments followed the policy of “flattening the curve” in order to avoid overwhelming their health systems. Most of the world also followed the policy of forced lockdowns to stop the spread of the virus. However, these policies produced did not produce consistent results across the globe. To investigate the impact of various policy measures on the reported outcomes, this research analyzed the actual COVID-19 data up till May 29, 2021, and the associated outcomes. Using global COVID-19 death rate as a base, the death rates of various countries were analyzed to gauge the efficacy of lockdown measures through probabilistic estimates and relative lack of uncertainty. Brier score was calculated to find the accuracy of probabilistic estimates. The data show high divergence in infection, death, and growth rates of the virus in different countries. The research also includes comparing the effects of virus in year 2020 and 2021, and the effect of vaccination. It can be seen that the collective world response was not commensurate with the actual risks involved. The paper concludes by emphasizing the need for specific evidence-based governance and disaster response management to face similar challenges in the future.","PeriodicalId":19224,"journal":{"name":"Nigerian Journal of Basic and Clinical Sciences","volume":"71 2 1","pages":"1 - 9"},"PeriodicalIF":0.1,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"87718239","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}
U. Bello, Lawal B. Abdullahi, Soladoye A. Abdulmajeed
{"title":"Laparoscopic reduction of intussusception in a 13-year-old girl with multiple intestinal polyps: A case report","authors":"U. Bello, Lawal B. Abdullahi, Soladoye A. Abdulmajeed","doi":"10.4103/njbcs.njbcs_46_22","DOIUrl":"https://doi.org/10.4103/njbcs.njbcs_46_22","url":null,"abstract":"Intussusception has been one of the common causes of intestinal obstruction in infancy and early childhood period with a challenge in diagnosis in older children. It is the telescoping of a segment of bowel into another, commonly a proximal segment into a distal one. Intussusception in infancy and early childhood is usually primary or idiopathic as there is no associated pathological lead point involved. However, in older children and adult, there is most often time an identifiable lead point that predisposes to the intussusception.","PeriodicalId":19224,"journal":{"name":"Nigerian Journal of Basic and Clinical Sciences","volume":"06 1","pages":"89 - 91"},"PeriodicalIF":0.1,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"86311594","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}
{"title":"Effects of nutritional and socioeconomic status on puberty","authors":"Omosalewa Oyewole, A. Adediran, A. Oduwole","doi":"10.4103/njbcs.njbcs_39_22","DOIUrl":"https://doi.org/10.4103/njbcs.njbcs_39_22","url":null,"abstract":"Context: The onset of puberty is determined by genetic and environmental factors like socioeconomic and nutritional status. Previous studies have shown a disparity in the effect of socioeconomic factors on menarche and sexual maturation. Aim: This study was carried out to determine the effect of nutrition and socioeconomic status (SES) on puberty. Settings and Design: It is a descriptive, cross-sectional study among adolescent female secondary school students in Abeokuta, Nigeria. Materials and Methods: Subjects selected responded to questions on the proforma. Body mass index (BMI) was calculated from weight and height that were measured. Statistical Analysis: The data were analyzed using Microsoft ExcelR (Microsoft Corporation SP2 software microsoft office 2010 professional plus (PC) Intel Corporation's 386 microprocessor Redmond, Washington, USA) 2010 and SPSS 22.0 (SPSS Inc., Illinois, USA). Results: Participants' mean age and BMI were 14.4 ± 1.9 years and 19.1 ± 2.9 kg/m2, respectively. Subjects from high socioeconomic class (SEC) attained puberty and menarche earlier (12.0 ± 1.5 and 12.9 ± 1.5 years) than those from low SEC (13.5 ± 1.3 and 13.7 ± 1.1 years). These were statistically significant for breast development and menarche. Nutritional status had a negative correlation (r = -0.098) with menarcheal age but positive with pubic hair (r = 0.702) and breast development (r = 0.306). Conclusion: The mean age of menarche occurred earlier in this study when compared to most previous studies in Nigeria. Improved SES and nutritional status influenced the early age of onset of puberty and attainment of menarche.","PeriodicalId":19224,"journal":{"name":"Nigerian Journal of Basic and Clinical Sciences","volume":"63 1","pages":"40 - 45"},"PeriodicalIF":0.1,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"77908072","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}