We evaluated the renal vascular indices in children and adolescents with sickle cell disease (SCD) using Doppler ultrasonography. We also assessed the renal hemodynamics alterations in patients with homozygous SCD and sickle beta-thalassemia (sickle β-thalassemia). We studied 75 patients (age range = 3-20 years; M = 9.95 ± 4.15) with SCD: 42 patients suffering from homozygous SCD and 33 patients diagnosed with sickle β-thalassemia. Thirty, age- and sex-matched, normal subjects were also included as a control group. Both patients and control groups had Doppler assessment of pulsatility (PI) and resistivity (RI) indices of main renal, segmental, interlobar, and arcuate arteries. Both PIs and RIs were significantly higher in SCD patients, compared with the control group. Among patients, PIs and RIs in the main renal, segmental, interlobar, and arcuate arteries were significantly higher in patients with homozygous SCD as compared with those with sickle β-thalassemia (p values <0.01, <0.001, <0.001, and <0.001 for PIs and <0.001, <0.001, <0.001, and <0.01 for RIs, respectively). We concluded that renal vascular resistance is raised in children and adolescents with SCD. This is more pronounced in patients with homozygous SCD as compared with those with sickle β-thalassemia.
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http://dx.doi.org/10.1177/0161734614553831 | DOI Listing |
Blood Rev
November 2024
Division of Haematology/Oncology, The Hospital for Sick Children, Toronto, Ontario, Canada; Departments of Paediatrics and Medicine, University of Toronto, Ontario, Canada. Electronic address:
Background: Detection of sickle cell disease (SCD) could be improved with greater use of point-of-care testing (POCT). This review assessed the accuracy of POCTs for SCD in children and adolescents.
Methods: We systematically searched EMBASE, PubMed, Cochrane libraries, registries and conference proceedings from inception to 28th February 2023.
West Afr J Med
November 2024
Department of Medicine, ABUTH, Zaria.
Background: Chronic Kidney Disease (CKD) among patients with Sickle Cell Disease (SCD) notably HbSS and HbSC are increasingly being recognized due to improved health care a comparative severity of CKD among these subgroups is not known in ABUTH Zaria.
Objective: This study was to assess the severity of kidney function among stable patients with HbSS and HbSC in ABUTH, Zaria.
Method: A cross-sectional descriptive study involving steady state 188 HbSS and 22 HbSC attending Haematology Clinic in ABUTH Zaria.
Hum Gene Ther
November 2024
ART-TG, Inserm US35, Corbeil-Essonnes, France.
Sickle cell disease (SCD) is a prevalent inherited blood disorder arising from a single point mutation that results in substitution of valine with glutamic acid in the Beta hemoglobin chain, making red blood cells assume a banana shape under low oxygen state. It is most prevalent in sub-Saharan Africa, affecting approximately 2% of the population in Mali. This study aimed to evaluate the iron status and associated hematological parameters in SCD patients at steady state in an environment with a high prevalence of iron deficiency.
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October 2024
Department of Biotechnology, National Institute of Technology, Raipur, Chhattisgarh, 492010, India.
Sickle cell diseases are widespread in regions encompassing the Mediterranean, Middle East, sub-Saharan Africa, and specific parts of Asia, primarily due to the abnormal production of hemoglobin S. This genetic blood disorder stems from a mutation in the beta-globin gene, a crucial component of hemoglobin and the heme-containing protein found in red blood cells. Point mutations in the hemoglobin gene can be inherited as a heterozygous or homozygous pattern.
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