Avascular necrosis in sickle cell (homozygous S) patients: Predictive clinical and laboratory indices

Background: Pathogenetic mechanism as well as laboratory and clinical correlates of osteonecrosis in sickle cell have not been fully investigated. The aim of this study is to investigate the predictive value of the steady state white cell and platelet count as well as the frequency of bone pain crisis per annum to detect sickle cell patients who will eventually develop avascular necrosis (AVN).Patients and Methods: A 5 year retrospective analysis of 122 homozygous S (HbSS) patients, aged 6‑49 years (mean age 24.7 ± 7 years), out of which 16 patients (13.1%) had developed AVN within the years under review.Results: The prevalence of AVN in sickle cell patients was determined to be 13.1 per 1000. The steady state white cell count, platelet count, frequency of bone pain crisis and hematocrit, was compared in patients that develop AVN and those who had not over the period. Only the steady state platelet count was found to differ significantly (P = 0.011) between these two patient groups and to correlate positively (Pearson correlation coefficient = −0.251) with development of AVN. The hematocrit, white cell count, and frequency of bone pain crisis were found neither to differ significantly nor correlate with the development of AVN.Conclusion: In conclusion, patients with a raised steady state platelet count may have a higher tendency to develop AVN and may require closer orthopedic review and prophylactic intervention.Key words: Avascular necrosis, homozygous S, platelet count, sickle cell anemia, white cell coun

Prevalence and associations of symptomatic renal papillary necrosis in sickle cell anemia patients in South‑Eastern Nigeria

Aim: To assess the prevalence and associations of symptomatic renal papillary necrosis (RPN) in sickle cell anemia patients.Patients and Methods: The case notes of homozygous hemoglobin (Hb) S patients diagnosed with RPN were retrospectively assessed. Diagnosis was based on microscopic hematuria and positive ultrasound findings. Their steady state diastolic blood pressure, Hb, leukocyte count, platelet count, serum direct bilirubin, and aspartate transaminase, were obtained by automated analyzers. These were evaluated for any relationship with the occurrence of RPN.Results: Two hundred and twenty patients were assessed aged 6–55 years with a median age of 24 years. The prevalence of symptomatic RPN was found to be 2.3%. RPN was positively associated with the female gender (Chi‑square P value 0.001), but not with any other clinical or laboratory variable. However, other predictors of disease severity were positively associated with RPN such as age, diastolic blood pressure 0.180 (P = 0.016), serum aspartate transaminase, serum bilirubin 0.145 (0.027), Hb, and leukocyte count − 0.155 (P = 0.003).Conclusion: The prevalence of symptomatic RPN is low in this group of homozygous S patients and occurs more commonly in females. Improvement in care for these patients will reduce these chronic complications.Keywords: Female gender, microscopic hematuria, renal papillary necrosis, sickle cell anemi

Multiple myeloma in Nigeria: An insight to the clinical, laboratory features, and outcomes

Aim: In developing African nations, late presentation and occurrence of complications adversely affects survival. This study aims at identifying initial clinical and basic laboratory features of multiple myeloma (MM), which will aid the physician to entertain a high index of suspicion and therefore target his investigations in order to prevent late presentation and avert complications.Materials and Methods: A retrospective analysis of 32 patients diagnosed and managed in Nigeria, West Africa was done. Information on the clinical, laboratory, and radiological data as well as response to treatment was obtained at presentation, 3, 6, 12, and 24 months and analyzed.Results: The median age at diagnosis was 62 years, 17 (53.1%) males and 15 (46.9%) females. The median duration of follow‑up was 24 weeks (range, 2-288 weeks). The average percentage of bone marrow plasmacytosis at diagnosis was 38%. Clinical features at presentation were anemia (71.9%) and bone pains (78.1%), while pathological fractures were found in 69%, and nephropathy in 13.8%. The longest duration of survival of 288 and 252 weeks were recorded in patients on melphalan and prednisolone with or without thalidomide.Conclusion: Presence of bone pain and anemia in elderly patients should alert the clinician to investigate along the lines of MM. Majority of patients have osteolytic lesions on X‑ray and pathological fractures, and benefit from melphalan based combinations in situations where facilities for transplant are not available.Key words: Clinical features, chemotherapy, laboratory features, multiple myeloma, Nigeria, treatmen

Stroke prevalence amongst sickle cell disease patients in Nigeria: a multi-centre study

Background: Stroke is a life-changing, debilitating complication of sickle cell disease (SCD). Previous studies had recorded high stroke prevalence amongst this group of patients. Nigeria has a large population of people affected by this condition and this study aims to assess the stroke prevalence in this large population.Methodology: Stroke prevalence data from 14 physicians working in 11 tertiary health centres across the country was collated by doctors using the sickle cell registers and patient case notes. This data was then collated and used to obtain the overall stroke prevalence in adult and children.Results: The stroke prevalence in sickle cell disease patients in Nigeria was observed to be 12.4 per 1000 patients. Prevalence in the adult patients was 17.7 per 1000 patients and 7.4 per 1000 patients in children. Twenty three percent of the affected patients had more than stroke episode.Conclusion: The stroke prevalence in Nigeria is lower than previously recorded rates and further studies will be required to investigate other factors which may play a role.Keywords: sickle cell, stroke, Nigeria, prevalenc

Cardiovascular testing recovery in Latin America one year into the COVID-19 pandemic: An analysis of data from an international longitudinal survey.

The INCAPS COVID Investigators Group, listed by name in the Appendix, thank cardiology and imaging professional societies worldwide for their assistance in disseminating the survey to their memberships. These include alphabetically, but are not limited to, American Society of Nuclear Cardiology, Arab Society of Nuclear Medicine, Australasian Association of Nuclear Medicine Specialists, Australia-New Zealand Society of Nuclear Medicine, Belgian Society of Nuclear Medicine, Brazilian Nuclear Medicine Society, British Society of Cardiovascular Imaging, Conjoint Committee for the Recognition of Training in CT Coronary Angiography Australia and New Zealand, Consortium of Universities and Institutions in Japan, Danish Society of Cardiology, Gruppo Italiano Cardiologia Nucleare, Indonesian Society of Nuclear Medicine, Japanese Society of Nuclear Cardiology, Moscow Regional Department of Russian Nuclear Medicine Society, Philippine Society of Nuclear Medicine, Russian Society of Radiology, Sociedad Española de Medicina Nuclear e Imagen Molecular, Society of Cardiovascular Computed Tomography, and Thailand Society of Nuclear Medicine.Peer reviewe

Identifying rail asset maintenance processes: a human-centric and sensemaking approach

Efficient asset maintenance is key for delivering services such as transport. Current rail maintenance processes have been mostly reactive with a recent shift towards exploring proactive modes. The introduction of new ubiquitous technologies and advanced data analytics facilitates the embedding of a ‘predict-and-prevent’ approach to managing assets. Successful, user-centred integration of such technology is still, however, a sparsely understood area. This study reports results from a set of interviews, based on Critical Decision Method, with rail asset maintenance and management experts regarding current procedural aspects of asset management and maintenance. We analyse and present the results from a human-centric sensemaking timeline perspective. We found that within a complex sociotechnical environment such as rail transport, asset maintenance processes apply not just at local levels, but also to broader, strategic levels that involve different stakeholders and necessitate different levels of expertise. This is a particularly interesting aspect within maintenance that has not been discussed as of yet within a process-based and timeline-based models of asset maintenance. We argue that it is important to consider asset maintenance activities within both micro (local) and macro (broader) levels to ensure reliability and stability in transport services. We also propose that the traditionally distinct notions of individual, collaborative and artefact-based sensemaking are in fact all in evidence in this sensemaking context, and argue that a more holistic view of sensemaking is therefore appropriate by placing these results within an amended Recogntion Primed Decsion making model

Implant Associated Surgical Site Infection in Orthopaedics: A Regional Hospital Experience

No Abstrac

Frequency and clinical impact of ETV6/RUNX1, AF4‑MLL, and BCR/ABL fusion genes on features of acute lymphoblastic leukemia at presentation

Background: Variations in disease presentation and outcome of leukemia treatment has been associated with the presence of certain mutant genes. Three major translocations (ETV6‑RUNX1, BCR‑ABL, and AF4‑MLL) in acute lymphoblastic leukemia (ALL) have been shown to affect treatment outcome. This study is aimed at assessing the relationship between these translocations and the presence of other indicators of disease severity (white cell count, hemoglobin concentration, platelet count, and hematocrit) in ALL.Patients and Methods: Forty chemotherapy naïve patients aged between 9 months and 54 years had their marrow samples analyzed for the prevalent mutations. Their clinical and laboratory details on presentation were also obtained.Results: Abnormal genes detected were BCR/ABL1 major transcript in 5 (12.5%), ETV6/RUNX1 in 2 (5.0%), MLL/AF4 none and none of the patients had more than one fusion gene. There was no relationship between the presence of these fusion genes and the clinical and laboratory features of ALL. An association exists between the fusion genes and ethnic origin of the patients (P = 0.005). There is no significant association between the abnormal fusion genes detected and some laboratory features of prognostic importance, which include total white blood cell count (P = 0.416) and FAB subtype (P = 0.576).Conclusion: Presence of fusion the genes BCR/ABL1, ETV6/RUNX1, and MLL/AF4 does not have any impact on the clinical and laboratory features of ALL at presentation.Key words: Acute lymphoblastic leukemia, BCR/ABL1, ETV6/RUNX1, fusion genes, MLL/AF

Important clinical and laboratory correlates of glomerular filtration rate in sickle cell anemia

Background: Renal impairment is routinely assessed using the estimated glomerular filtration rate (eGFR) and it may be helpful to obtain certain clinical or laboratory markers, which show relationship with glomerular filtration rate (GFR) in sickle cell disease (SCD).Aim: To assess the relationship between important clinical and laboratory parameters in SCD, and the eGFR.Patients and Methods: Steady state clinical and laboratory data were obtained from 228 homozygous SCD patients seen over a 7‑year period. The GFR was estimated using (isotope dilution mass spectrometry) traceable modification of diet in renal disease (MDRD) and Cockroft–Gault methods. The correlation coefficient and independent t‑test were done to assess the level of significance between the eGFR_MDRD and the known indicators of disease severity.Results: The serum alkaline phosphatase (ALP) and serum direct bilirubin levels both showed significant inverse relationship with eGFR_MDRD P = 0.012 and P = 0.24, respectively. The patients’ age, Hb, leukocyte count, platelet count, serum direct bilirubin and aspartate transaminase did not show a significant correlation. The eGFR_MDRD was more discriminatory revealing that 20.3% of the patients had suboptimal GFR. Proteinuria in steady state was observed in 20.3% of the patients.Conclusion: High serum direct bilirubin and ALP are associated with a deteriorating eGFR_MDRD. Other clinical and laboratory indicators of disease severity in SCD do not show the relationship with the GFR. MDRD_GFR estimation seemed to be a more appropriate method of estimating GFR in SCD.Key words: Cockroft–Gault, glomerular filtration rate, modification of diet in renal disease sickle cel