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Osteoarticular complications of sickle cell disease in children
Abstract
Introduction
Sickle cell disease is the most common molecular disease. The sickling of the haemoglobin S followed by micro-vascular occlusion leads to complications observable in several tissues. Osteoarticular complications represent the most frequent pattern of hospitalization of children with sickle cell disease. This review discusses osteoarticular complications of sickle cell disease in children.
Materials and methods
This study is a review concerning children <15 years of age. Phenotypes retained are SS, SC and AS, with or without thalassaemia or foetal haemoglobin. Osteomyelitis, arthritis and osteonecrosis are included. Ficat’s classification was used for osteonecrosis of femoral head.
Discussion
Osteomyelitis is the most frequent complication. It is classically due mainly to salmonella species, but currently, several studies have found other micro-organisms ( Staphylococcus aureus , Streptococcus pneumonia , klebsiella). Multiple sites are affected at the same time. Long bones are the most affected and according to studies, the most frequent are the humerus, the tibia or the femur. Delay to diagnosis often leads to chronic osteomyelitis, with a high risk of orthopaedic sequela. Arthritis can affect all joints. Also due to salmonella and S. aureus , they can have multiple locations. The hip and knee are the favoured sites. Inadequate treatment can result in the destruction of the joints. Avascular osteonecrosis occurs later, after the age of 10. Their evolution is progressive and insidious, explaining the late diagnosis. Osteonecrosis of the femoral head is the most frequent, and poses the problem of hip arthroplasty in children.
Conclusion
The osteoarticular complications must be hunted in children with sickle cell disease in order to diagnose them early. A quick and efficient treatment enables to avoid serious orthopaedic sequela.
... Our findings are in agreement with more recent studies that have reported Staphylococcus aureus to be the commonest causative organism in SCD patients. [21][22][23] However, other previous authors found Salmonella to be the commonest causative organism. 24,25 in this study, only six sequestrectomies out of the 17 patients who had chronic osteomyelitis were done during the study period. ...
Aims
Sickle cell disease (SCD) is an autosomal recessive inherited condition that presents with a number of clinical manifestations that include musculoskeletal manifestations (MM). MM may present differently in different individuals and settings and the predictors are not well known. Herein, we aimed at determining the predictors of MM in patients with SCD at the University Teaching Hospital, Lusaka, Zambia.
Methods
An unmatched case-control study was conducted between January and May 2019 in children below the age of 16 years. In all, 57 cases and 114 controls were obtained by systematic sampling method. A structured questionnaire was used to collect data. The different MM were identified, staged, and classified according to the Standard Orthopaedic Classification Systems using radiological and laboratory investigations. The data was entered in Epidata version 3.1 and exported to STATA 15 for analysis. Multiple logistic regression was used to determine predictors and predictive margins were used to determine the probability of MM.
Results
The cases were older median age 9.5 (interquartile range (IQR) 7 to 12) years compared to controls 7 (IQR 4 to 11) years; p = 0.003. After multivariate logistic regression, increase in age (adjusted odds ratio (AOR) = 1.2, 95% confidence interval (CI) 1.04 to 1.45; p = 0.043), increase in the frequency of vaso-occlusive crisis (VOC) (AOR = 1.3, 95% CI 1.09 to 1.52; p = 0.009) and increase in percentage of haemoglobin S (HbS) (AOR = 1.18, 95% CI 1.09 to 1.29; p < 0.001) were significant predictors of MM. Predictive margins showed that for a 16-year-old the average probability of having MM would be 51 percentage points higher than that of a two-year-old.
Conclusion
Increase in age, frequency of VOC, and an increase in the percentage of HbS were significant predictors of MM. These predictors maybe useful to clinicians in determining children who are at risk. Cite this article: Bone Joint Open 2020;1-6:175–181.
... Our findings are in agreement with more recent studies that have reported Staphylococcus aureus to be the commonest causative organism in SCD patients. [21][22][23] However, other previous authors found Salmonella to be the commonest causative organism. 24,25 in this study, only six sequestrectomies out of the 17 patients who had chronic osteomyelitis were done during the study period. ...
Aims
Sickle cell disease (SCD) is an autosomal recessive inherited condition that presents with a number of clinical manifestations that include musculoskeletal manifestations (MM). MM may present differently in different individuals and settings and the predictors are not well known. Herein, we aimed at determining the predictors of MM in patients with SCD at the University Teaching Hospital, Lusaka, Zambia.
Methods
An unmatched case-control study was conducted between January and May 2019 in children below the age of 16 years. In all, 57 cases and 114 controls were obtained by systematic sampling method. A structured questionnaire was used to collect data. The different MM were identified, staged, and classified according to the Standard Orthopaedic Classification Systems using radiological and laboratory investigations. The data was entered in Epidata version 3.1 and exported to STATA 15 for analysis. Multiple logistic regression was used to determine predictors and predictive margins were used to determine the probability of MM.
Results
The cases were older median age 9.5 (interquartile range (IQR) 7 to 12) years compared to controls 7 (IQR 4 to 11) years; p = 0.003. After multivariate logistic regression, increase in age (adjusted odds ratio (AOR) = 1.2, 95% confidence interval (CI) 1.04 to 1.45; p = 0.043), increase in the frequency of vaso-occlusive crisis (VOC) (AOR = 1.3, 95% CI 1.09 to 1.52; p = 0.009) and increase in percentage of haemoglobin S (HbS) (AOR = 1.18, 95% CI 1.09 to 1.29; p < 0.001) were significant predictors of MM. Predictive margins showed that for a 16-year-old the average probability of having MM would be 51 percentage points higher than that of a two-year-old.
Conclusion
Increase in age, frequency of VOC, and an increase in the percentage of HbS were significant predictors of MM. These predictors maybe useful to clinicians in determining children who are at risk. Cite this article: Bone Joint Open 2020;1-6:175–181.
Infection of the axial skeleton and the flat and small bones represents a growing proportion of all cases of osteomyelitis. The most common causative agent is Staphylococcus aureus, but this varies with the site involved and the pathogenesis. In contrast to osteomyelitis of the long bones, osteomyelitis of the flat and irregular bones presents with low-grade or no fever and poorly localized symptoms. The erythrocyte sedimentation rate almost invariably is elevated, and the C-reactive protein concentration rises and falls in a pattern that mirrors the clinical course, rendering it a valuable test with which to monitor the success of therapy. Initial plain radiographs often are normal, and bone scan usually is more sensitive to confirm the diagnosis early in the course of the illness. Antimicrobial therapy is based on the patient's age and site of infection. Complications are rare with expedient diagnosis and appropriate therapy.
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