We formulate a realistic demographic model that captures the pattern of inheritance of the S gene, which is responsible for the most common genetic defect, namely, sickle-cell anaemia (SCA), using general pair formations. The model equation is implicitly solved via the Laplace transform technique, while the existence of a unique solution is proved by applying the contraction mapping principle. One of the main results is the boundedness of the solution. A fundamental reason for the persistence of SCA is probably due to the role played by the selective advantage of the abnormal S gene over the normal haemoglobin A in tropical regions, and the fact that carriers are more fertile and survive longer (a property known as hybrid vigor), because they are essentially asymptomatic. We also discuss possible public health policies.
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http://dx.doi.org/10.1007/s11538-006-9148-y | DOI Listing |
Pediatr Blood Cancer
January 2025
Department of Audiology and Speech Therapy, Universidade Federal de São Paulo, Sao Paulo, Brazil.
Introduction: Sickle cell anemia has a genetic origin characterized by an autosomal recessive inheritance pattern. The nervous system may be subject to vaso-occlusion and, consequently, affect the proper functioning of the central portion of hearing.
Objective: To assess central auditory skills and analyze short- and long-latency auditory evoked potentials in children with sickle cell disease.
Neurol Sci
January 2025
Hematology Unit, Careggi University Hospital, Florence, Italy.
Background: The coexistence of sickle cell anemia and multiple sclerosis in a single patient presents a rare and challenging clinical scenario, possibly favoured by the interplay between chronic inflammatory states and autoimmune processes.
Methos/results: We present the case of a 36-year-old woman with sickle cell anemia who developed progressive neurological symptoms leading to frequent falls and paraparesis; magnetic resonance imaging showed many periventricular, infratentorial, and both cervical and dorsal spinal cord lesions, leading to a diagnosis of multiple sclerosis. After a multidisciplinary approach the patient was successfully started on ofatumumab.
Tissue Cell
January 2025
Department of Biology, Universidade Estadual Paulista (UNESP), São Paulo, Brazil; Campus de Três Lagoas, Universidade Federal de Mato Grosso do Sul (CPTL/UFMS), Mato Grosso do Sul, Brazil. Electronic address:
Sickle cell disease (SCD) is a hereditary hemolytic anemia associated with the alteration of the membrane composition of the sickle erythrocytes, the loss of glycolysis, dysregulation of the pyruvate phosphatase pathway, and changes in nucleotide metabolism of the sickle red blood cell (RBC). This review provides a comprehensive overview of the impact of the presence of Hb S, which leads to the disruption of the normal RBC metabolism. The intricate interplay between the redox and energetic balance in erythrocytic cells, where the glycolysis, pentose phosphate pathway, and methemoglobin reductase pathways are all altered in sickle RBC, is a key focus.
View Article and Find Full Text PDFInt Arch Otorhinolaryngol
January 2025
Department of Audiology, All India Institute of Speech and Hearing, Mysore, Karnataka, India.
Sickle cell anemia (SCA) is a genetic disorder with clinical manifestations due to circulatory changes, leading to adverse effects on the auditory system that might impact auditory processing, such as auditory discrimination and speech perception ability. This condition is associated with the severity level of anemia. The purpose of the present study was to investigate the influence of anemia severity on auditory discrimination ability and speech perception in noise among SCA patients with normal hearing sensitivity.
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