ObjectiveTo obtain multicentre data on the prevalence of normal, high or conditional (intermediat... more ObjectiveTo obtain multicentre data on the prevalence of normal, high or conditional (intermediate) blood velocity in the cerebral arteries among children with sickle cell disease (SCD) in Nigeria.DesignA prospective observational study in five tertiary healthcare institutions. By transcranial Doppler (TCD) ultrasonography, cerebral artery peak systolic blood velocity (PSV) was determined in 193 children with SCD and time averaged mean of the maximum blood velocity (TAMMV) in a different cohort of 115 children. This design was to make the findings relevant to hospitals with TCD equipment that measure either PSV or TAMMV.SettingNigeria.Participants308 children (126 girls, 182 boys; age 2–16 years).Main outcome measuresPercentage of children with SCD who have normal, high or intermediate (often termed conditional) PSV or TAMMV.ResultsIn the cohort of 193 children, PSV was normal in 150 (77.7%), high in 7 (3.6%) and conditional in 36 (18.7%). In the cohort of 115 children, TAMMV was no...
Introduction: Previous studies had enlisted renal medullary carcinoma (RMC) as the seventh nephro... more Introduction: Previous studies had enlisted renal medullary carcinoma (RMC) as the seventh nephropathy in sickle cell disease (SCD). Clinical experience has contradicted this claim and this study is targeted at refuting or supporting this assumption. Objective: To estimate the prevalence of RMC and describe other renal complications in SCD. Materials and methods: 14 physicians (haematologists and urologists) in 11 tertiary institutions across the country were collated from patients' case notes and hospital SCD registers. Results: Of the 3,596 registered sickle patients, 2 (0.056%) had been diagnosed with RMC over a ten year period, thereby giving an estimated prevalence rate of 5.6 per 100,000. The most common renal complication reported by the attending physicians was chronic kidney disease (CKD). The frequency of routine renal screening for SCD patients varied widely between centres-most were done at diagnosis, annually or biannually. Conclusion: The ten year prevalence of RMC in Nigerian SCD patients was determined to be 5.6 (estimated incidence of 0.56). RMC is not more common in SCD patients and therefore cannot be regarded as a "Seventh Sickle nephropathy". Most of the managing physicians reported that the commonest nephropathy observed in their SCD patients was chronic kidney disease.
Stroke is a well-known debilitating complication of sickle cell disease. It is a leading cause of... more Stroke is a well-known debilitating complication of sickle cell disease. It is a leading cause of death in children as well as adults with this condition, with a reported risk of first cerebrovascular accident (CVA) in the first 20 years of life being 0.761 per hundred patient years 1. A Jamaican cohort study reported a prevalence rate of 7.8% in 310 patients of all genotypes aged
Background: Renal impairment is routinely assessed using the estimated glomerular filtration rate... more 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.
pism, and an approximately similar ratio developed erectile dysfunction; they also had lower stea... more pism, and an approximately similar ratio developed erectile dysfunction; they also had lower steady-state leucocyte counts. Other clinical and laboratory indicators of disease severity in SCD did not positively correlate with the occurrence of priapism, and this may imply an alternative pathogenetic mechanism.
Plasma cell leukemia (PCL) is a rare and an aggressive disease, accounting for only 2%–3% of all ... more Plasma cell leukemia (PCL) is a rare and an aggressive disease, accounting for only 2%–3% of all plasma cell dyscrasias. Diagnosis is made by the presence of more than 2 × 109/L plasma cells in the peripheral blood or monoclonal plasmacytosis more than 20% of the plasma cells in the peripheral blood. PCL has limited treatment options comprising of the conventional multiple myeloma (MM) treatment drugs and a very poor prognosis. We report a case of a 55-year-old woman who presented with a history of high-grade fever, generalized weakness, petechial rashes, and transfusion-dependent anemia of about 3 months' duration. Peripheral blood film revealed moderate leukocytosis with 30% plasma cells. Bone marrow aspirate also showed significant plasmacytosis. Immunophenotyping confirmed peripheral blood plasmacytosis. The patient initially responded to chemotherapy but succumbed to the disease.
ObjectiveTo obtain multicentre data on the prevalence of normal, high or conditional (intermediat... more ObjectiveTo obtain multicentre data on the prevalence of normal, high or conditional (intermediate) blood velocity in the cerebral arteries among children with sickle cell disease (SCD) in Nigeria.DesignA prospective observational study in five tertiary healthcare institutions. By transcranial Doppler (TCD) ultrasonography, cerebral artery peak systolic blood velocity (PSV) was determined in 193 children with SCD and time averaged mean of the maximum blood velocity (TAMMV) in a different cohort of 115 children. This design was to make the findings relevant to hospitals with TCD equipment that measure either PSV or TAMMV.SettingNigeria.Participants308 children (126 girls, 182 boys; age 2–16 years).Main outcome measuresPercentage of children with SCD who have normal, high or intermediate (often termed conditional) PSV or TAMMV.ResultsIn the cohort of 193 children, PSV was normal in 150 (77.7%), high in 7 (3.6%) and conditional in 36 (18.7%). In the cohort of 115 children, TAMMV was no...
Introduction: Previous studies had enlisted renal medullary carcinoma (RMC) as the seventh nephro... more Introduction: Previous studies had enlisted renal medullary carcinoma (RMC) as the seventh nephropathy in sickle cell disease (SCD). Clinical experience has contradicted this claim and this study is targeted at refuting or supporting this assumption. Objective: To estimate the prevalence of RMC and describe other renal complications in SCD. Materials and methods: 14 physicians (haematologists and urologists) in 11 tertiary institutions across the country were collated from patients' case notes and hospital SCD registers. Results: Of the 3,596 registered sickle patients, 2 (0.056%) had been diagnosed with RMC over a ten year period, thereby giving an estimated prevalence rate of 5.6 per 100,000. The most common renal complication reported by the attending physicians was chronic kidney disease (CKD). The frequency of routine renal screening for SCD patients varied widely between centres-most were done at diagnosis, annually or biannually. Conclusion: The ten year prevalence of RMC in Nigerian SCD patients was determined to be 5.6 (estimated incidence of 0.56). RMC is not more common in SCD patients and therefore cannot be regarded as a "Seventh Sickle nephropathy". Most of the managing physicians reported that the commonest nephropathy observed in their SCD patients was chronic kidney disease.
Stroke is a well-known debilitating complication of sickle cell disease. It is a leading cause of... more Stroke is a well-known debilitating complication of sickle cell disease. It is a leading cause of death in children as well as adults with this condition, with a reported risk of first cerebrovascular accident (CVA) in the first 20 years of life being 0.761 per hundred patient years 1. A Jamaican cohort study reported a prevalence rate of 7.8% in 310 patients of all genotypes aged
Background: Renal impairment is routinely assessed using the estimated glomerular filtration rate... more 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.
pism, and an approximately similar ratio developed erectile dysfunction; they also had lower stea... more pism, and an approximately similar ratio developed erectile dysfunction; they also had lower steady-state leucocyte counts. Other clinical and laboratory indicators of disease severity in SCD did not positively correlate with the occurrence of priapism, and this may imply an alternative pathogenetic mechanism.
Plasma cell leukemia (PCL) is a rare and an aggressive disease, accounting for only 2%–3% of all ... more Plasma cell leukemia (PCL) is a rare and an aggressive disease, accounting for only 2%–3% of all plasma cell dyscrasias. Diagnosis is made by the presence of more than 2 × 109/L plasma cells in the peripheral blood or monoclonal plasmacytosis more than 20% of the plasma cells in the peripheral blood. PCL has limited treatment options comprising of the conventional multiple myeloma (MM) treatment drugs and a very poor prognosis. We report a case of a 55-year-old woman who presented with a history of high-grade fever, generalized weakness, petechial rashes, and transfusion-dependent anemia of about 3 months' duration. Peripheral blood film revealed moderate leukocytosis with 30% plasma cells. Bone marrow aspirate also showed significant plasmacytosis. Immunophenotyping confirmed peripheral blood plasmacytosis. The patient initially responded to chemotherapy but succumbed to the disease.
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