Osteonecrosis of the Humeral Head in Sickle Cell Disease

Milner, Paul F.; Kraus, Alfred; Sebes, Jeno; Sleeper, Lynn A.; Dukes, Kimberly A.; Embury, Stephen H.; Bellevue, Rita; Koshy, Mabel; Moohr, John W.; Smith, J. Orson

doi:10.1097/00003086-199304000-00018

Cited by 75 publications

(45 citation statements)

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“…A subset of patients, however, exists with frequent severe vaso-occlusive crisis and osteonecrosis (unpublished data). The frequency of ␣-thal trait was not significantly higher in the latter group unlike in American SCD patients [3,[5][6][7]. It has been hypothesized that this subset may have some other underlying genetic predisposition.…”

Section: Introductionmentioning

confidence: 77%

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Frequency of the 677 C→T mutation of the methylenetetrahydrofolate reductase gene among Kuwaiti sickle cell disease patients

et al. 2001

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Sickle cell disease (SCD) is relatively mild among Kuwaiti Arabs. However, an atypical subset of patients exists with frequent, severe vaso-occlusive crisis and osteonecrosis. The thermolabile variant of MTHFR, resulting from a C→T mutation at nucleotide 677, has been shown to be associated with hyperhomocysteinemia, which is an important risk factor for premature vascular disease. We have screened an unselected group of 41 Kuwaiti SCD patients (33 SS and 8 S␤ 0 -thal) attending the Hematology Clinic of Kuwait University Teaching Hospital for the MTHFR mutation, using a PCR-RFLP method. The patients were aged 2-41 years (mean of 12.8 ± 8.6). One (2.4%) individual was homozygous for the mutation while 15 (36.6%) were heterozygous, giving an allele frequency of 20.7%. Twenty-one patients (14 SS and 7 S␤ 0 -thal) were screened for osteonecrosis using MRI of the hip (spin-echo T1-and T2-weighted images). Seven (33.3%) had varying degrees of osteonecrosis, among whom the frequency of the 677 C→T allele was 21.4%. The frequency was identical among those without osteonecrosis. Although the allele frequency is higher among our patients compared to American SS patients, our results do not suggest an association with osteonecrosis. Am.

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Section: Introductionmentioning

confidence: 77%

“…Previous studies among American SS patients reported an association of osteonecrosis with co-existent ␣-thal trait [5][6][7]. The hypothesis was that the higher Hct predisposes these patients to certain complications associated with high viscosity, such as retinopathy and osteonecrosis.…”

Section: Discussionmentioning

confidence: 99%

Frequency of the 677 C→T mutation of the methylenetetrahydrofolate reductase gene among Kuwaiti sickle cell disease patients

et al. 2001

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“…The vaso-occlusive crises, also could affect the vertebrate and ribs [11]. Osteonecrosis was found in the epiphysis of almost 41% of adults with sickle cell disease [12][13][14]. Low whole body bone mineral content also found among the SCD more than the controls with normal hemoglobin.…”

Section: Sickle Cell Disease (Scd)mentioning

confidence: 99%

Could Denosumab Support Bone Integrity in Sickle Cell Disease

2015

J Hematol Thromb

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Sickle cell anaemia is a congenital disease characterized by painful vaso-occlusive crises. It is associated with vitamin D deficiency, osteoporosis and recurrent attacks of micro-infarctions lead to bone osteonecrosis. The most common sites of osteonecrosis in sickle cell disease are the femoral and humeral heads which could lead to severe pain and extensive physical disability. Denosumab is a new human monoclonal antibody product; it is a receptor activator of nuclear factor kappa B ligand RANKL. That decreases osteoclastic bone restoration. It is Food and Drug Administration FDA approved for osteoporosis for women with high risk of fracture; and it is also being used for the treatment and prevention of bone loss in patients undergoing hormone ablation therapy for breast and prostate cancer. Denosumab is not restricted for sickle cell disease patients within its approved indications. It has less nephrotoxicity effect than the bisphosphonates which is a big advantage for sickle cell patients. It also used as an adjuvant agent to reduce metastatic bone pain in solid tumors. The extension of Denosumab use for sickle cell patients' osteoporosis and osteonecrosis deserve a randomized multi central trail aiming to improve bone integrity and possible reduction of the devastating pain in sickle cell disease patients.

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“…25 Corticosteroid use, alcohol abuse, Caisson's disease, Cushing's syndrome, and systemic lupus erythematosus are potential causes for osteonecrosis. 25,27,53,56,58,63,78 TSA is frequently indicated for the treatment of osteonecrosis of the humeral head; however, functional outcomes following surgery vary, possibly based on the etiology of osteonecrosis. 49 Those individuals who have a TSA due to osteonecrosis from steroid use seem to have better ROM outcomes than those who have osteonecrosis from trauma (Table).…”

Section: Osteonecrosismentioning

confidence: 99%

Rehabilitation Following Total Shoulder Arthroplasty

Wilcox

Arslanian²,

Millett³

2005

J Orthop Sports Phys Ther

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Total shoulder arthroplasty (TSA) is a standard operative treatment for a variety of disorders of the glenohumeral joint. Patients, who have continued shoulder pain and loss of function in the presence of advanced joint pathology, despite conservative management, are often managed by undergoing a TSA. The overall outcomes that are reported after surgical intervention are quite good and appear to be primarily determined by the underlying pathology and the tissue quality of the rotator cuff. The current Neer protocol for postoperative TSA rehabilitation is widely used and based on tradition and the basic science of soft tissue and bone healing. The purpose of this paper is to review the indications for TSA, focusing on the underlying pathologies, and to describe the variables that impact the rehabilitation program of individuals who have had a TSA. A postoperative TSA rehabilitation protocol and algorithm, founded on basic science principles and tailored toward the specific clinical condition, are presented. J Orthop Sports Phys Ther 2005;35:821-836. Key Words: physical therapy, protocols, shoulder rehabilitation T he first total shoulder arthroplasty (TSA) was performed by Jules Emile Pean in 1893 for the purpose of treating tuberculous arthritis of the shoulder. 68 Neer 81 developed a humeral prosthesis for the treatment of 4-part fractures in 1955, and in the mid-1970s he refined his prosthesis for the treatment of the degenerative humeral head. 82 TSA is a standard treatment intervention for patients with underlying advanced joint pathology who have persistent pain and loss of function despite conservative management. These pathologies include osteoarthritis (OA), 7,13,30,32,39,41,43,44,64,71,74,82,86,88,89,100,101 rheumatoid arthritis (RA), 6,34,35,38,52,61,64,76,91,102,106 cuff tear arthropathy, 5,33,64,94,95,107,111 osteonecrosis, 26,48,49,64,70,79 and fractures of the humeral head. 2,3,6,21,42,62,69,85,90,93 Over the last 25 years, surgical techniques and prostheses have advanced greatly. However, there is still considerable variability in surgical techniques, particularly the use of cement for fixation and the type of prosthesis. Despite these significant variations, the overall reported outcomes for patients that have undergone TSA are good. 31,49,64,99,104 Self-assessed health status reports of individuals who have undergone TSA are comparable to those of individuals who have undergone a total hip arthroplasty or coronary artery bypass graft.

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Osteonecrosis of the Humeral Head in Sickle Cell Disease

Cited by 75 publications

References 0 publications

Frequency of the 677 C→T mutation of the methylenetetrahydrofolate reductase gene among Kuwaiti sickle cell disease patients

Frequency of the 677 C→T mutation of the methylenetetrahydrofolate reductase gene among Kuwaiti sickle cell disease patients

Could Denosumab Support Bone Integrity in Sickle Cell Disease

Rehabilitation Following Total Shoulder Arthroplasty

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