scholarly journals Histopathology of aseptic necrosis of the femoral head in sickle cell disease

2010 ◽  
Vol 35 (8) ◽  
pp. 1145-1150 ◽  
Author(s):  
Martin Mukisi-Mukaza ◽  
Anne Gomez-Brouchet ◽  
Monique Donkerwolcke ◽  
Maurice Hinsenkamp ◽  
Franz Burny
Keyword(s):  
Femoral Head ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
Aseptic Necrosis

ASEPTIC NECROSIS OF THE FEMORAL HEAD IN SICKLE-CELL DISEASE

Rheumatology ◽  
1986 ◽  
Vol 25 (1) ◽  
pp. 34-39 ◽  
Author(s):  
W. W. EBONG ◽  
T. M. KOLAWOLE
Keyword(s):  
Femoral Head ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
Aseptic Necrosis

scholarly journals Cell therapy in aseptic necrosis of the femoral head in patients with sickle cell disease

Bionatura ◽  
2016 ◽  
Vol 1 (3) ◽  
Author(s):  
Aymara Maria Baganet-Cobas ◽  
◽  
José Armando Galván-Cabrera ◽  
Mariela Forrellat-Barrios ◽  
Norma D. Fernández-Delgado ◽  
...  
Keyword(s):  
Femoral Head ◽  
Sickle Cell Disease ◽  
Cell Therapy ◽  
Sickle Cell ◽  
Cell Disease ◽  
Aseptic Necrosis

scholarly journals Causal role of L-glutamine in sickle cell disease painful crises: a Mendelian randomization analysis

2019 ◽  
Author(s):  
Yann Iboudo ◽  
Melanie E. Garrett ◽  
Pablo Bartolucci ◽  
Carlo Brugnara ◽  
Clary B. Clish ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Small Molecules ◽  
Causal Relationship ◽  
Sickle Cell ◽  
Drug Targets ◽  
Mendelian Randomization ◽  
Cell Disease ◽  
Aseptic Necrosis ◽  
Randomization Analysis

ABSTRACTIn a recent clinical trial, the metabolite L-glutamine was shown to reduce painful crises in sickle cell disease (SCD) patients. To confirm this observation and identify other metabolites implicated in SCD clinical heterogeneity, we profiled 129 metabolites in the plasma of 705 SCD patients. We tested correlations between metabolite levels and six SCD-related complications (painful crises, cholecystectomy, retinopathy, leg ulcer, priapism, aseptic necrosis) or estimated glomerular filtration rate (eGFR), and used Mendelian randomization (MR) to assess causality. We found a causal relationship between L-glutamine levels and painful crises (N=1,278, odds ratio (OR) [95% confidence interval] = 0.68 [0.52 – 0.89], P=0.0048). In two smaller SCD cohorts (N=299 and 406), the protective effect of L-glutamine was observed (OR=0.82 [0.50-1.34]), although the MR result was not significant (P=0.44). We identified 66 significant correlations between the levels of other metabolites and SCD-related complications or eGFR. We tested these correlations for causality using MR analyses and found no significant causal relationship. The baseline levels of quinolinic acid was associated with prospectively ascertained survival in SCD patients, and this effect was dependent on eGFR. Metabolomics provide a promising approach to prioritize small molecules that may serve as biomarkers or drug targets in SCD.

scholarly journals Spontaneous healing of avascular necrosis of the femoral head in sickle cell disease

2019 ◽  
Vol 94 (6) ◽  
pp. E160-E162 ◽  
Author(s):  
Nelda P. Itzep ◽  
Siddharth P. Jadhav ◽  
Celeste K. Kanne ◽  
Vivien A. Sheehan
Keyword(s):  
Femoral Head ◽  
Sickle Cell Disease ◽  
Avascular Necrosis ◽  
Sickle Cell ◽  
Cell Disease ◽  
Spontaneous Healing

Hydroxyurea Use Is Associated with Avascular Necrosis of the Femoral Head among Children with Sickle Cell Disease

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 2477-2477 ◽  
Author(s):  
Kris Michael Mahadeo ◽  
Suzette Oyeku ◽  
Karen Moody ◽  
Swapmil N. Rajpathak ◽  
Abraham Groner ◽  
...  
Keyword(s):  
Risk Factor ◽  
Femoral Head ◽  
Sickle Cell Disease ◽  
Avascular Necrosis ◽  
Sickle Cell ◽  
Odds Ratio ◽  
Demographic Characteristics ◽  
Cell Disease ◽  
Health Maintenance ◽  
Laboratory Values

Abstract Hydroxyurea therapy is associated with reduced morbidity among patients with sickle cell disease (SCD). Avascular necrosis of the femoral head (AVN) is one potentially debilitating complication of SCD. In this study, we examined the relationship between hydroxyurea use and the prevalence of AVN among children with SCD. We performed a retrospective chart review of 202 children with SCD, aged 10–21 years, followed in the pediatric hematology program at the Children’s Hospital at Montefiore (Bronx, NY) between July 2007 and 2008. Abstracted data included age, ethnicity, SCD genotype, frequency of hospitalization, hip radiograph results, laboratory data and hydroxyurea use. Hip radiographs were performed prospectively as part of SCD health maintenance from 2005–2008. Forty-four patients were excluded because they did not have a screening hip radiograph. Descriptive statistics were calculated for independent variables. T-tests and chi-square tests were used to compare clinical and demographic characteristics of children with and without AVN. Multivariate logistic regressions were used to estimate the odds ratio of having AVN among SCD patients. Our final sample consisted of 158 patients whose demographic characteristics are listed in Table 1. The prevalence of AVN was 16.5% (n=26). Of the clinical variables analyzed, we identified significant associations between the presence of AVN and hydroxyurea use (p=.005), as well as older age (p=.013) (Table 1.) Children with AVN had significantly lower mean lactic dehydrogenase levels (LDH) (p=.04) and higher mean corpuscular volumes (MCV) (p=.012). (Table 2.) After controlling for gender, ethnicity, sickle cell genotype, and frequency of hospitalizations, age was also found to be associated with AVN (OR 1.15, 95% confidence interval (CI): 1.01,1.31, p=0.033). SCD patients on hydroxyurea had higher odds of having AVN compared to non-users (OR 3.51, 95% CI: 1.31, 9.38, p= 0.013). Laboratory values (MCV, Hemoglobin, LDH and Hematocrit) had a high degree of collinearity and were removed from the final model. In summary, the prevalence of AVN in our sample was 16.5%. This is substantially higher than the prevalence of approximately 6% reported by the Cooperative Study of Sickle Cell Disease for comparative age groups in a prospective study1. SCD patients exposed to hydroxyurea were three times more likely to have AVN than those not exposed to this drug. Vaso-occlusive pain crisis is a recognized risk factor for AVN, thus we could expect a higher rate of AVN among patients on hydroxyurea. However, the odds ratio of 3.5 is unexpectedly high and warrants further investigation into the role of hydroxyurea as a risk factor for AVN. Nonetheless, these preliminary results suggest that more stringent screening regimens for AVN may be indicated among this subset of patients. Table 1. Clinical characteristics of patients with and without avn *p<0.05 **p<0.01 No AVN (N =132) AVN (N = 26) Age * 15.7 years 17.4 years Sex Male 64 (49%) 17 (65%) Ethnicity Black 110 (83%) 23 (88%) Hispanic 22 (17%) 3 (12%) HgbSS 84 (64%) 20 (77%) HgbSC 38 (29%) 4 (15%) HgbSBthal0 5(3.8%) 2 (8%) Hgb SC HgbSBthal+ 5 (3.8%) 0 On Hydroxyurea** 38 (29%) 15 (58%) # Hospitalizations 0 60 (45%) 10 (38%) 1–5 64 (49%) 14 (54%) >5 8 (6%) 2 (8%) Table 2. Mean Laboratory Values for Patients With And Without AVN No AVN AVN *p<0.05 (N =132) (N = 26) WBC 10.7 k/uL 10.5 k/uL Hgb 9.4 gm/dL 9.6 gm/dL MCV* 83 fL 89 fL Platelets 381 k/uL 376 k/uL Reticulocyte 7.70% 8.10% Ferritin 369.8 ng/mL 438.7 ng/mL LDH* 471.6 U/L 389 U/L Creatinine 0.6 mg/dL 0.6 mg/dL Hgb F 9.80% 11.30%

The Natural History of Asymptomatic Osteonecrosis of the Femoral Head in Adults with Sickle Cell Disease

2006 ◽  
Vol 88 (12) ◽  
pp. 2565-2572 ◽  
Author(s):  
Ph. Hernigou ◽  
A. Habibi ◽  
D. Bachir ◽  
F. Galacteros
Keyword(s):  
Femoral Head ◽  
Sickle Cell Disease ◽  
Natural History ◽  
Sickle Cell ◽  
Cell Disease ◽  
History Of

Physical Therapy Alone Compared with Core Decompression and Physical Therapy for Femoral Head Osteonecrosis in Sickle Cell Disease

2006 ◽  
Vol 88 (12) ◽  
pp. 2573-2582 ◽  
Author(s):  
Lynne D. Neumayr ◽  
Christine Aguilar ◽  
Ann N. Earles ◽  
Harry E. Jergesen ◽  
Charles M. Haberkern ◽  
...  
Keyword(s):  
Physical Therapy ◽  
Femoral Head ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Core Decompression ◽  
Cell Disease ◽  
Femoral Head Osteonecrosis

Sickle Cell Disease as a Cause of Osteonecrosis of the Femoral Head

1991 ◽  
Vol 325 (21) ◽  
pp. 1476-1481 ◽  
Author(s):  
Paul F. Milner ◽  
Alfred P. Kraus ◽  
Jeno I. Sebes ◽  
Lynn A. Sleeper ◽  
Kimberly A. Dukes ◽  
...  
Keyword(s):  
Femoral Head ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease

scholarly journals Management of osteonecrosis of the femoral head in children with sickle cell disease: results of conservative and operative treatments at skeletal maturity

2018 ◽  
Vol 12 (1) ◽  
pp. 47-54
Author(s):  
C. Mallet ◽  
A. Abitan ◽  
C. Vidal ◽  
L. Holvoet ◽  
K. Mazda ◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Femoral Head ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Skeletal Maturity ◽  
Weight Bearing ◽  
Long Term Results ◽  
Harris Hip Score ◽  
Cell Disease ◽  
Femoral Head Osteonecrosis

Purpose Sickle cell disease (SCD) is the most common cause of femoral head osteonecrosis (ONFH) during childhood with an overall prevalence of 10%. In children, spontaneous revascularization can occur, as in Legg-Calve-Perthes disease. Consequently, the aim of treatment is to restore proper hip containment to prevent joint arthritis. This is the first study reporting long-term results at skeletal maturity of non-operative and surgical treatments for ONFH in SCD children. Methods All children with ONFH due to SCD were retrospectively reviewed. At initial evaluation, extension of osteonecrosis was radiographically defined using Catterall, lateral pillar Herring and Ficat classifications. Subluxation of the femoral head with Reimers migration index > 30% required surgical treatment including femoral varus osteotomy and/or pelvic osteotomies. Conservative treatment including non-weight bearing and physiotherapy was performed in the remaining cases. Outcomes were assessed at skeletal maturity using the Harris Hip Score (HHS) and the Stulberg classification. Total hip arthroplasty and Stulberg 5 were defined as failures. Results A total of 25 hips in 17 patients were included (mean follow-up 7.5 years SD 3.4). Mean age at diagnosis was 11.4 years SD 2.9. In all, 15 hips (60%) were classified Catterall 3 and 4 and Herring B and C. A total of 13 patients (52%) underwent surgical treatment. At skeletal maturity, mean HHS was good (81 SD 17), 12 hips (48%) were classified Stulberg 1 and 2, seven hips (28%) were classified Stulberg 3 and 4. Conclusion Both treatments led to good functional results with 75% of congruent hips at skeletal maturity. Level of Evidence IV

scholarly journals Fragment D-dimer levels: an objective marker of vaso-occlusive crisis and other complications of sickle cell disease

Blood ◽  
1986 ◽  
Vol 68 (1) ◽  
pp. 317-319 ◽  
Author(s):  
DV Devine ◽  
TR Kinney ◽  
PF Thomas ◽  
WF Rosse ◽  
CS Greenberg
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Latex Agglutination ◽  
Cell Disease ◽  
Aseptic Necrosis ◽  
Clinical Complications ◽  
Asymptomatic Patients ◽  
Coagulation Tests ◽  
The Relationship ◽  
D Dimer

Abstract Although abnormalities in coagulation tests have been reported during vaso-occlusive crises in patients with sickle cell disease, objective, readily performed laboratory tests that document the occurrence of this complication have not been available. We examined the relationship between fibrin D-dimer levels and the occurrence of complications in patients with sickle cell disease, using a commercially available latex bead agglutination assay. The patients were either asymptomatic, hospitalized for vaso-occlusive crisis, or had other complications of sickle cell disease including leg ulcers, chronic cholecystitis, aseptic necrosis, joint pain and infection. Fifty-seven percent of 187 samples on 96 patients had elevated levels of fibrin D-dimer. Ninety percent of 75 samples from asymptomatic patients were negative for fibrin D-dimer (less than 1 microgram/ml) but 97% of 29 samples from patients with vaso-occlusive crisis and 85% of 83 samples from patients with other complications of sickle cell disease were positive. In serial studies, worsening or amelioration in clinical complications were reflected in increasing or decreasing levels of fibrin D-dimer, respectively. The molecular species of fibrin identified by the latex agglutination test was shown to be fragment D-dimer by successive immunoprecipitation and protein blot analysis. We conclude that the complications of sickle cell disease, including vaso-occlusive crisis, result in the production of fibrin D-dimer, and its detection may be used as a marker for the presence of the complication.

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