Multifocal Osteonecrosis in a 3-Year-old Child With Sickle Beta Plus Thalassemia

Bhasin, Neha; Price, Nathan L.; Desoky, Sarah M.

doi:10.1097/mph.0000000000002155

Cited by 2 publications

(1 citation statement)

References 16 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Avascular necrosis of the femoral head of the hip during pregnancy has been reported in less than 100 cases [ 12 ], and no cases of osteonecrosis affecting femoral meta diaphysis and/or condyle have been reported in pregnancy to our knowledge. Although osteonecrosis is a well-known complication of sickle cell disease (prevalence of which increases with the presence of coexisting alpha-thalassemia trait) [ 13 ], it has been reported in only a few cases with sickle cell trait and sickle-beta-thalassemia trait, with osteonecrosis primarily affecting the femoral head in these cases [ 14 , 15 ].…”

Section: Discussionmentioning

confidence: 99%

Osteonecrosis of Bilateral Distal Femurs in a Pregnant Patient Following Antenatal Betamethasone

Ali¹,

Al-Sudani²,

Tan³

2022

Cureus

View full text Add to dashboard Cite

Corticosteroid therapy is a known risk factor for osteonecrosis, more commonly with chronic use and high cumulative dose. Osteonecrosis (avascular necrosis) has been described in pregnancy involving primarily the femoral head. To our knowledge, only rare cases of femoral meta diaphysis or knee osteonecrosis in pregnancy have been documented in the literature.We report a 28-year-old woman with sickle cell trait and beta-thalassemia trait who developed severe bilateral knee pain shortly after corticosteroid therapy. She was 34-weeks pregnant when she presented with the signs of preterm labor and was found to have oligohydramnios and preeclampsia. She was given two intramuscular injections of betamethasone 12 mg one day apart to enhance the fetal lung maturity. Within hours of the second injection, she developed acute and severe bilateral knee pain affecting her mobility and ambulation. Bilateral knee x-rays were unremarkable. Given the severity and persistence of her pain, magnetic resonance imaging (MRI) of bilateral lower extremities was done few days later and showed signs of early osteonecrosis involving bilateral distal femoral meta diaphysis and right lateral femoral condyle.Other than the steroid therapy she had received, no additional extrinsic risk factors for osteonecrosis were identified. Potential intrinsic risk factors were thought to include her combined sickle-beta-thalassemia traits and pregnancy. She was diagnosed with steroid-induced osteonecrosis, given the temporal relationship. Her presentation was unique, because osteonecrosis affected unreported sites during pregnancy, and it started shortly after a brief course of antenatal steroid. She was treated conservatively with analgesics, and outpatient orthopedic follow-up was recommended. She was advised to avoid prolonged weight-bearing and strenuous activities. On a follow-up appointment two months later, she was still complaining of bilateral knee pain with ambulation though it was less severe. She did not return for follow-up thereafter.We suggest the possibility of osteonecrosis in pregnancy involving uncommon sites, such as distal femur and femoral condyle in this case, following one or two doses of systemic steroid. Obstetricians need to consider osteonecrosis when evaluating an unexplained musculoskeletal pain after betamethasone that is used for preterm labors. More studies, including reporting more cases with unusual presentation and prospective studies following pregnant patients receiving steroid therapy, are needed to better understand the causes, associations, management, and clinical course of osteonecrosis in pregnancy.

show abstract

Section: Discussionmentioning

confidence: 99%

Osteonecrosis of Bilateral Distal Femurs in a Pregnant Patient Following Antenatal Betamethasone

Ali¹,

Al-Sudani²,

Tan³

2022

Cureus

View full text Add to dashboard Cite

show abstract

Real‐World Evidence of Crizanlizumab Showing Reductions in Vaso‐Occlusive Crises and Opioid Usage in Sickle Cell Disease

DeBonnett,

Joshi,

Silva‐Pinto

et al. 2024

European J of Haematology

View full text Add to dashboard Cite

ObjectiveAccess to crizanlizumab, a disease‐modifying therapy for sickle cell disease (SCD), was provided through a managed access program (MAP, NCT03720626). The present analysis evaluated the impact of 12 months of crizanlizumab treatment on vaso‐occlusive crises (VOCs), and on the use of opioids for VOC‐related pain relief, in patients with SCD from the MAP.MethodsFrom June 2018 to January 2023, 112 patients with a history of recurrent VOCs completed 12 months of crizanlizumab (5 mg/kg) treatment and were monitored for adverse events (AEs).ResultsCrizanlizumab led to reductions of 18.0% and 36.2% in the proportions of patients having ≥ 1 home‐ and ≥ 1 healthcare‐managed VOCs. Median absolute changes (interquartile range) from baseline in the rates of home‐ and healthcare‐managed VOCs were −3.0 (−6.0, −1.0) and −2.0 (−4.0, 0), respectively. Data stratified by genotype and prior hydroxyurea use showed similar reductions in VOC rates. A 35.5% reduction in the proportion of patients requiring opioids was noted. AEs were consistent with earlier reports, and no new safety concerns were identified.ConclusionsCrizanlizumab demonstrated potential benefits in attenuating VOC episodes, irrespective of SCD genotype and prior hydroxyurea use, and in lowering opioid usage. The safety of crizanlizumab was consistent with earlier reports.Trial Registration: The MAP has been registered at ClinicalTrials.gov with the ID, NCT03720626

show abstract

Multifocal Osteonecrosis in a 3-Year-old Child With Sickle Beta Plus Thalassemia

Cited by 2 publications

References 16 publications

Osteonecrosis of Bilateral Distal Femurs in a Pregnant Patient Following Antenatal Betamethasone

Osteonecrosis of Bilateral Distal Femurs in a Pregnant Patient Following Antenatal Betamethasone

Real‐World Evidence of Crizanlizumab Showing Reductions in Vaso‐Occlusive Crises and Opioid Usage in Sickle Cell Disease

Contact Info

Product

Resources

About