Avascular necrosis of the femoral head in children with sickle cell disease (Libreville, Gabon)

Mouba, John Florent; Mimbila, M.; Lentombo, Léonie Ester; Thardin, Jean-François; Ondo, A

doi:10.1684/san.2011.0244

Cited by 5 publications

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“…In our study, the mean age at the time of diagnosis of AOFH of 11.51 ± 2.17 years is slightly lower than that found in the literature, which often varies from 12 to 14 years [12,[24][25][26][27]. However, these studies report a slightly younger age of onset: 7 years [13,28], 6 years [26] and even 5 years for osteonecrosis of the shoulder [27]. The particularity of our serie is that nearly half of the children were under 11 years old (45.16%), whereas Mouba [13] and Man and Koren [27] reported a prevalence of AOFH clearly greater after the age of 11.…”

Section: Discussioncontrasting

confidence: 81%

“…Our pediatric series is one of the highest among those reported in Africa: Mouba in Gabon reported 22 cases over 10 years [13]. Akakpo-Numado in Togo reported 14 (including 8 HbSS and 6 HbSC) over a period of 20 years, but these were data from a pediatric surgery department and not from a treatment center for sickle cell disease [14].…”

Section: Discussionmentioning

confidence: 85%

“…Little data exist on AOFH in African children suffering from sickle cell disease. It appears that the diagnosis is most often made at the stage of joint destruction [13,14]. This work aimed at…”

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confidence: 99%

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Aseptic Osteonecrosis of the Femoral Head in Children Living with Sickle Cell Disease at the National Reference Center for Sickle Cell Disease of Brazzaville, Congo

FO,

ASW,

et al. 2023

J Blood Disord Med

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Introduction: Aseptic Osteonecrosis of the Femoral Head (AOFH) is a degenerative complication of sickle cell disease. In the absence of care, it is a source of disability through painful lameness and functional impotence that it causes over time. Its data in african children are scarce. This work aimed at determining its prevalence and describing its characteristics. Methodology: It was about a descriptive study conducted over a4 years- period at the CNRDr on children suffering from homozygous sickle cell disease and presenting with ONFH. The studied variables were epidemiological, clinical and paraclinical, collected from medical records. The Postel Merle-d’Aubigné functional score was used to assess the functional status of the hips and the Arlet and Ficat classification for radiological staging. Results: The study involved 31 children (18 boys and 13 girls). The prevalence of AOFH was 2.15%. The average age was 11.51 ± 2.17 years old. Almost half were under 11 (45.16%). At diagnosis, the damage was bilateral in 10/31 cases, bringing the number of pathological hips to 41/62. Hip pain was constant and associated with lameness in 83.87% of cases. Hip function was good, fair, and poor respectively in 41.94%, 54.84% and 3.22% of cases. One child presented with two stiff hips. Radiologically, stages I and II on one hand and III and IV on the other hand of Arlet and Ficat were respectively found in 45.16% and 54.84% of cases. Conclusion: We report one of the most important series of black Africa. AOFH affects young children. The diagnosis is frequently late, made at the stage of functional impotence with joint destruction. The search for predictive factors is important to improve the prognosis of these young patients.

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Section: Discussioncontrasting

confidence: 81%

Section: Discussionmentioning

confidence: 85%

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Aseptic Osteonecrosis of the Femoral Head in Children Living with Sickle Cell Disease at the National Reference Center for Sickle Cell Disease of Brazzaville, Congo

FO,

ASW,

et al. 2023

J Blood Disord Med

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“…Following hip replacement surgery, vaso-occlusive crises and other complications commonly occur. ONFH morbidity has mostly been described in small, crosssectional, single-institution studies, [38][39][40][41][42][43] with findings that might not be generalizable to a larger population of SCD patients. In our novel approach, we built a large SCD cohort from the socioeconomically diverse state of California, using an iterative process based on methods described in other SCD epidemiologic studies.…”

Section: Discussionmentioning

confidence: 99%

Osteonecrosis of the Femoral Head in Sickle Cell Disease: Prevalence, Comorbidities and Surgical Outcomes in California

Adesina

Brunson

Gotlib

et al. 2016

Blood

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Introduction Osteonecrosis of the femoral head (ONFH) is characterized by insidious onset of hip joint pain, limited range of motion and disability in sickle cell disease (SCD) patients. Prevalence of ONFH, rates of hip replacement surgery and post-operative course have not been previously described in population-based cohort studies. We retrospectively reviewed a large SCD patient cohort from California's Office of Statewide Planning and Development (OSHPD) Patient Discharge Database (PDD), and report the risk factors and clinical outcomes for SCD patients with ONFH. Methods Patients were ascertained from the PDD from 1991 to 2013, through an iterative search algorithm using ICD-9 codes. In addition to descriptive statistics and univariate comparisons with the chi-square test, we used multivariable Cox proportional hazards models (with age as the time scale), to analyze factors associated with ONFH diagnosis, including sex, SCD severity and antecedent acute chest syndrome (ACS; data available from 2003-2013). Patients with more severe SCD were defined as those who averaged 3 of more hospitalizations per year. Antecedent ACS was considered as a time-dependent covariate to first ONFH diagnosis after 2003. We estimated the cumulative incidence of ONFH, accounting for the competing risk of death, by SCD severity and antecedent ACS diagnoses, with differences assessed by the Gray's test for equality. We also determined rates of all re-admissions to the PDD and emergency department within 30-90 days after hip replacement surgery, including re-admissions for venous thromboembolism (VTE) and painful vasoocclusive crises (VOC). All data were analyzed using SAS 9.4 software, and results presented as hazard ratios (HR) and 95% confidence intervals (CI). Results Of the 6,237 SCD patients identified, 1,356 (22%) were diagnosed with ONFH (Table 1). Three hundred and eight of the SCD patients with ONFH underwent hip arthroplasty. The median ages of ONFH diagnosis and hip replacement surgery were 27 and 36 years, respectively. In Cox regression analyses over the entire study period, both males (HR 1.13, CI 1.01 - 1.27) and SCD severity (HR 2.75, CI 2.44 - 3.11) were associated with ONFH. In Cox regression analyses from 2003-2013, SCD severity (HR 2.89, CI 2.48 - 3.36) and antecedent ACS (HR 1.56, CI 1.31 - 1.84) were independently associated with ONFH diagnosis. The cumulative incidence curves corroborate the association between SCD severity, antecedent ACS, and ONFH (Figures 1 and 2; p<0.0001)). By 40 years of age, 36% of patients with severe SCD (vs. 14% without severe disease) and 36% with antecedent ACS (vs. 12% without ACS) had developed ONFH. Among SCD patients with ONFH, 43%, 51% and 58% were re-admitted postoperatively within 30, 60 and 90 days, respectively. At 60 and 90 days after discharge for hip arthroplasty, 22% and 28% were readmitted for VOC, respectively. Conclusions The overall prevalence of ONFH in this SCD cohort was 22%, and approximately 23% of the ONFH patients underwent hip arthroplasty (5% of the entire SCD cohort). Thus, ONFH is a common complication of SCD, which often requires surgical intervention. ONFH was independently associated with SCD severity and ACS, as has been previously suggested (Kato, Gladwin et al. 2007). Readmission within 30-60 days after hip surgery was common in this SCD cohort, and often due to painful VOC. Of the 144 readmissions within 90 days of discharge from hip replacement surgery, only 2 (~1%) were due to VTE, which is lower than reported in non-sickle cell patients undergoing hip replacement surgery. Our large, populations-based cohort study provides insight into the frequency and risk factors of ONFH in SCD patients, and also reveals that post-operative readmissions are common. References Kato, G. J., M. T. Gladwin and M. H. Steinberg (2007). "Deconstructing sickle cell disease: reappraisal of the role of hemolysis in the development of clinical subphenotypes." Blood Rev 21(1): 37-47. Cumulative incidence of ONFH among SCD patients in the Patient Discharge Database, 1991-2013 (n=6,237) Cumulative incidence of ONFH among SCD patients in the Patient Discharge Database, 1991-2013 (n=6,237) Figure 1 Cumulative incidence of ONFH among SCD patients entering the Patient Discharge Database after 10/1/2003 (n=1,538) Figure 1. Cumulative incidence of ONFH among SCD patients entering the Patient Discharge Database after 10/1/2003 (n=1,538) Figure 2 Figure 2. Disclosures Adesina: bluebird bio: Honoraria.

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“…The head of the femur is the most common seat [4,5]. In Africa, the avascular osteonecrosis of the femoral head (AOFH) affects sometimes very young children [6]. In the Congo, nearly half of them are under the age of 11, and the diagnosis is often late, at the stage of joint destruction corresponding to the radiological stages III or IV of Arlet and Ficat [7].…”

Section: Introductionmentioning

confidence: 99%

Risk Factors of avascular Osteonecrosis of the Femoral Head in Children at the National Reference Center for Sickle Cell Disease in Brazzaville, Congo

Atipo-Tsiba

Bilongo-Bouyou

Bango³

et al. 2023

IBRR

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Introduction: The avascular osteonecrosis of the femoral head (AOFH) is a common complication of sickle cell disease (SCD). It exposes to lameness and sometimes to a very debilitating handicap. It is a source of desocialization, particularly in developing countries where prosthetic surgery remains inaccessible to the majority. This survey aimed to identify the risk of occurence AOFH. Materials and Methods: It was a case-control study carried between october 2017 and september 2021 at the National Reference Center for SCD « Antoinette Sassou Nguesso » in Brazzaville. It concerned 31 children with clinical and radiographic signs of AOFH. Clinical (age at diagnosis of SCD, frequency of vasoocclusive crises and hospitalization for vasoocclusive crises, number of blood transfusion) as well as hematological examination (blood count in the intercritical period) and hydroxyurea treatment were compared with those of 62 children with no clinical and radiographic signs of AOFH. The chi2 statistical test and the odds ratio were used for the comparison (P ˂ 0.05). Results: The sex ratio was 1.38 versus 0.93 (p = 0.50). The mean age of diagnostic of SCD was 3.76 ± 2.56 years versus 3.94 ± 2.01 years (p = 0.81). Logistic regression showed that SCD children with AOFH had a significantly higher rate of annual frequency of VOC (4.16 ± 1.18 vs 2.91 ± 1.68; P = 0.015), annual frequency of hospitalization for VOC (3.74±1.65 vs 1.45 ± 1.28; P = 0.000) and number of blood transfusions (3.38 ± 2.69 vs 2.42 ± 2.32; P = 0.03). Conclusion: Emphasis should be placed on the prevention and early management of acute complications of SCD. The role of hydroxyurea should be clarified by further work.

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Avascular necrosis of the femoral head in children with sickle cell disease (Libreville, Gabon)

Cited by 5 publications

References 0 publications

Aseptic Osteonecrosis of the Femoral Head in Children Living with Sickle Cell Disease at the National Reference Center for Sickle Cell Disease of Brazzaville, Congo

Aseptic Osteonecrosis of the Femoral Head in Children Living with Sickle Cell Disease at the National Reference Center for Sickle Cell Disease of Brazzaville, Congo

Osteonecrosis of the Femoral Head in Sickle Cell Disease: Prevalence, Comorbidities and Surgical Outcomes in California

Risk Factors of avascular Osteonecrosis of the Femoral Head in Children at the National Reference Center for Sickle Cell Disease in Brazzaville, Congo

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