Stem Cell Therapy for Avascular Necrosis of Femoral Head in Sickle Cell Disease: Report of 11 Cases and Review of Literature

Abstract Background and Objective: Avascular necrosis of femoral head is a common Issue faced by orthopaedic surgeons which ranges between 10-18% but in sickle cell Disease the incidence reaches 30%. There is no definite treatment except joint arthroplasty. Regenerative medicine is an option to cure or delay joint arthroplasty. We report here our experience with injection of ABMDO to manage ANFH and report our long term results,progression of the ANFH if any and delay in THA (Total Hip Arthoplasty).Patients and Methods: Sixty-Three (63) consecutive patients with SCD with ANFH were examined, thoroughly investigated and those who had ANFH < grade II were consented to receive ABMDO. Pre-operatively patients were clinically assessed using Visual analogue scale (VAS), Modified Harris Hips Score (MHHS) and Azam-Sadat Score (ASS) for Quality of Life Score for Chronic Hip Disease. Ten milliliter of bone marrow was aspirated under local anesthesia and was placed in 20 CC culture media. Osteoblasts were cultured from the bone marrow aspirated. Under anesthesia using 3 mm cannulated drill, the osteonecrosed lesion was drilled and 5 million osteoblasts were injected at the lesion site. Patients were evaluated in out patient clinic after two weeks. At four months a repeat MRI was done and patients were followed up a minimum for 2 years.Results: The average age was 25.93±5.48 years. There were 41 (65%) females and 22 (35%) males. The mean hemoglobin S was 83.2±5.1 percent. The average follow up was 49.05±12.9 ( range 24-60) months. VAS significantly improved from 7.79±1.06 at 2 weeks 4.07±1.08 p<0.0001 continued to improve for the next 24 months 2.38±0.55 (P<0.0001). MHHS improved from 41.77±5.37 to 73.19± 6.48 at 4 months (P<0.001) and at 24 months it was 88.93±3.6 (p<0.001). The ASS also significantly improved from 2.76±0.49 preoperatively to 7.92±0.09 (p<0.0001) at 24 months. A comparison of the MRI’s of before and after osteoblast implantation revealed new bone formation and amelioration of the avascular lesions. Three patients were unhappy with the outcome and one patients repeated attacks of the vaso-occlusive crisis within six months of the osteoblasts injection.Conclusions: The results give credence to our earlier short follow up results that osteoblasts transplantation has a great potential in healing of avascular lesions. Our study fits the criteria of Phase II clinical trial and We believe a larger study equivalent to Phase III numbers and include patients not only with sickle cell disease but also steroid induced and idiopathic avascular necrosis.

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Inhibiting in function of Gene in sickle Cell Disease

National Journal of Research in Ayurved Science ◽

10.52482/ayurlog.v1i01.108 ◽

2018 ◽

Vol 1 (01) ◽

Author(s):

Ashay Harish Nandeshwar

Keyword(s):

Stem Cell ◽

Sickle Cell Disease ◽

Cell Therapy ◽

Sickle Cell ◽

Stem Cell Therapy ◽

Genetic Diseases ◽

Marrow Transplant ◽

Genome Project ◽

Base Substitution ◽

Cell Disease

I was much struck with the fact that Sickle-Cell Disease and most of the genetic diseases are incurable. The knowledge I gained about base substitution mutation in DNA and the Human Genome Project, showed me light to the possibility of a cure to the disease. Stem Cell Therapy was the first thing that came into my mind, while thinking of a cure to SCD. Eventually I found that researches about using stem cell therapy for curing SCD were already being carried in various academic and research institutions across the globe. As a result, bone marrow transplant became a ray of hope for the sufferers of the disorder; but still, the scientists and doctors would assert the non-existence of a definite cure to the disease

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

American Journal of Hematology ◽

10.1002/ajh.25453 ◽

2019 ◽

Vol 94 (6) ◽

pp. E160-E162 ◽

Cited By ~ 1

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

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

Blood ◽

10.1182/blood.v112.11.2477.2477 ◽

2008 ◽

Vol 112 (11) ◽

pp. 2477-2477 ◽

Cited By ~ 2

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%

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

Cahiers de Santé ◽

10.1684/san.2011.0244 ◽

2011 ◽

Vol 21 (2) ◽

pp. 89-92

Author(s):

John Florent Mouba ◽

Mylène Mimbila ◽

Léonie Ester Lentombo ◽

Jean-François Thardin ◽

Alain Ondo

Keyword(s):

Femoral Head ◽

Sickle Cell Disease ◽

Avascular Necrosis ◽

Sickle Cell ◽

Cell Disease

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Risk of avascular necrosis of the femoral head in children with sickle cell disease on hydroxyurea: MRI evaluation

Pediatric Blood & Cancer ◽

10.1002/pbc.27503 ◽

2018 ◽

Vol 66 (2) ◽

pp. e27503 ◽

Cited By ~ 1

Author(s):

Adekunle D. Adekile ◽

Renu Gupta ◽

Abdullah Al-Khayat ◽

Ahmed Mohammed ◽

Said Atyani ◽

...

Keyword(s):

Femoral Head ◽

Sickle Cell Disease ◽

Avascular Necrosis ◽

Sickle Cell ◽

Cell Disease ◽

Mri Evaluation

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Avascular necrosis of the femoral head in sickle cell disease /Orak hucreli anemide avaskuler nekroz

Journal of Turgut Ozal Medical Center ◽

10.5455/jtomc.2016.08.091 ◽

2017 ◽

pp. 1

Author(s):

Neslihan Karakurt ◽

Mehmet Deveci

Keyword(s):

Femoral Head ◽

Sickle Cell Disease ◽

Avascular Necrosis ◽

Sickle Cell ◽

Cell Disease

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