Avascular necrosis significantly impairs quality of life in sickle cell disease

Pain is the leading cause for hospitalization in patients with sickle cell disease (SCD). While the characteristics of SCD pain can vary widely between patients and between phases of the disease (e.g. vasoocclusive crisis pain vs. chronic pain), similar neuronal mechanisms likely underlie the various aspects of nociceptive processing. In the peripheral nervous system, small unmyelinated C fibers and lightly-myelinated Aδ fibers detect and transmit noxious stimuli. Both classes of neurons express members of the transient receptor potential (TRP) family, a group of ligand gated ion-channels that are activated by thermal, chemical, and mechanical stimuli. Promiscuous TRP channel family members are activated by a wide range of stimuli, many of which are dysregulated in patients with SCD and transgenic SCD mouse models. In 2011, our lab published the first report of TRP channel contributions to rodent SCD pain. Since that time, additional basic and clinical research efforts have investigated the genetic and biochemical status of TRP channels in SCD, placing particular focus on TRPV1. This review will discuss these advances and highlight the clinical SCD presentations that have not yet been studied, but which may be mediated by TRP channel activity.

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Neuronal transient receptor potential (TRP) channels and noxious sensory detection in sickle cell disease

Sadler

Stucky

2019

Neuroscience Letters

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Avascular necrosis: Is there a role for biomechanical examination as a potential modality for its detection and treatment?

Longworth

Chockalingam

2016

International Musculoskeletal Medicine

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Sickle cell avascular necrosis: Prevalence and clinical profiles in a tertiary hospital northwestern Nigeria

et al. 2020

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Acute painful crises are the hallmark of Sickle Cell Anaemia (SCA). However, chronic daily pain also occurs in SCA patients with a high frequency. Avascular Necrosis (AVN) of the femur is an important cause of chronic pain and adversely affects their quality of life. The aim is to determine the prevalence of AVN among SCA patients in Zaria and describe some of its clinical and laboratory features. A cross-sectional study of 58 SCA patients in steady state. Data on gender, age, presence of radiologically diagnosed AVN, number of pain crises, blood transfusions in the previous 12 months and laboratory parameters were collated. Data were analyzed using JASP version 0.11.1.0. A critical level of α of 0.05 was set. Females constituted 40 out of 58 (69.0%) of the study participants. The median age was 23 (19.8, 28.0) years. AVN was present in 6 out of 58 (10.3%). There was no relationship between gender and AVN (FET, p=1.00, OR=0.889 95% CI 0.147, 5.359). There was no age difference between patients with AVN and those without AVN (29.92 vs 29.45, MWU=153.500, p=0.956). Patients with AVN had lower mean rank HCT levels (24.08 vs 30.13, MWU=123.500, p=0.414), higher pain episodes (31.67 vs 29.25, MWU=169.000,p=0.747), number of blood transfusions (33.42 vs 29.05, MWU=179.500, P=0.549) and platelets (34.00 vs 28.98, MWU=183.000, p=0.499) compared to those without AVN. Avascular necrosis is common among SCA patients in Zaria. These patients have more blood transfusions and bone pain episodes compared to those without AVN.

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Avascular necrosis significantly impairs quality of life in sickle cell disease

Cited by 3 publications

References 32 publications

Neuronal transient receptor potential (TRP) channels and noxious sensory detection in sickle cell disease

Neuronal transient receptor potential (TRP) channels and noxious sensory detection in sickle cell disease

Avascular necrosis: Is there a role for biomechanical examination as a potential modality for its detection and treatment?

Sickle cell avascular necrosis: Prevalence and clinical profiles in a tertiary hospital northwestern Nigeria

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