The Journal of Bone & Joint Surgery

The Journal of Bone & Joint Surgery, Volume 86, Issue 3

Scientific Articles | March 01, 2004

Steroid Treatment and the Development of Scoliosis in Males with Duchenne Muscular Dystrophy

Benjamin A. Alman, MD, FRCSC¹; S. Naweed Raza¹; W. Douglas Biggar, MD, FRCPC²

¹ Department of Surgery and Program in Developmental Biology, The Hospital for Sick Children, 555 University Avenue, Toronto, ON M5G 1X8, Canada. E-mail address for B.A. Alman: benjamin.alman@sickkids.ca
² Bloorview MacMillan Children's Centre, 350 Rumsey Road, Toronto, ON M4G 1R8, Canada

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The authors did not receive grants or outside funding in support of their research or preparation of this manuscript. They did not receive payments or other benefits or a commitment or agreement to provide such benefits from a commercial entity. No commercial entity paid or directed, or agreed to pay or direct, any benefits to any research fund, foundation, educational institution, or other charitable or nonprofit organization with which the authors are affiliated or associated.

Investigation performed at the Bloorview MacMillan Children's Centre, The Hospital for Sick Children, and the University of Toronto, Toronto, Ontario, Canada

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J Bone Joint Surg Am, 2004 Mar 01;86(3):519-524

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Abstract

Background: Scoliosis due to progressive muscle weakness occurs in almost all males with Duchenne muscular dystrophy, and it progresses relentlessly. Previous studies have shown that corticosteroid treatment slows the decline in muscle strength and stabilizes muscle strength in patients with this disease. We hypothesized that steroids may also attenuate the development of scoliosis. The purpose of this study was to compare the prevalence of scoliosis in male patients with Duchenne muscular dystrophy who received steroids with a control group of such patients who did not.

Methods: A group of seven to ten-year-old boys with Duchenne muscular dystrophy who were able to walk were enrolled in a nonrandomized comparative study to determine the effect of deflazacort (a derivative of prednisone) on muscle strength and pulmonary function. Thirty patients were treated with deflazacort (treatment group), and twenty-four were not (control group). The patients were matched for age and pulmonary function at baseline. To assess the development of scoliosis, the patients in each group were followed for at least five years. Survival curves were plotted to determine the chance of scoliosis of =20° developing. The difference between the groups with respect to the chance of scoliosis developing was determined with Kaplan-Meier analysis.

Results: A curve of =20° developed during the follow-up period in sixteen (67%) of the twenty-four patients in the control group but in only five (17%) of the thirty patients in the treatment group. Fifteen of the twenty-four patients in the control group underwent spine surgery, at a mean age of thirteen years, whereas only five of the thirty patients in the treatment group underwent spine surgery, at a mean age of fifteen years. Kaplan-Meier analysis demonstrated a significant difference between the two groups with regard to development of scoliosis of =20° (p < 0.001). Cataracts developed in ten patients in the treatment group, and stress fractures developed in three patients in the treatment group. Patients in the treatment group weighed a mean of 3.7 kg more than did those in the control group.

Conclusions: Steroid treatment slows the progression of scoliosis in males with Duchenne muscular dystrophy; however, longer-term evaluation will be necessary to determine if the treatment prevents the development of scoliosis or just delays its onset. At the very least, steroid treatment delays the need for spinal surgery.

Level of Evidence: Therapeutic study, Level II-1 (prospective cohort study). See Instructions to Authors for a complete description of levels of evidence.

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Progressive muscle weakness develops over time in patients with Duchenne muscular dystrophy. This weakness decreases the ability to walk and is a major factor contributing to the development of scoliosis^1,2. The progressive weakness generally leads to death secondary to cardiopulmonary dysfunction in the third decade of life. Scoliosis, which progresses relentlessly, develops once the patient begins to use a wheelchair full-time. Several studies have shown that scoliosis develops in almost all patients with Duchenne muscular dystrophy, with the exception of those who have a stable, hyperextended spine^1-3. The current management of progressive scoliosis in Duchenne muscular dystrophy is a posterior spinal arthrodesis with segmental spinal instrumentation, usually to the pelvis^4,5. Since there is progressive loss of muscle strength, pulmonary function also deteriorates with age, which increases the risks during anesthesia. The current recommendations for treatment of scoliosis in Duchenne muscular dystrophy are to perform surgery before the patient's pulmonary function becomes poor enough to preclude safe anesthesia^4,5.

Duchenne muscular dystrophy is caused by a mutation in the dystrophin gene⁶. Although the protein product of this gene has a variety of functions in many cell types, it has a predominant role in maintaining the integrity of the cell membrane in myocytes. In Duchenne muscular dystrophy, this function is lost and the cell membranes lose their integrity, resulting in the death of muscle cells over time⁷. Steroids help to maintain the integrity of the cell membranes and to decrease inflammation associated with cell death⁸. In an attempt to counteract the effect of the defective dystrophin protein, prednisone has been used to treat Duchenne muscular dystrophy. Early trials demonstrated a slowing of the expected decline in muscle function for the duration of the time for which the prednisone was administered^9,10. Unfortunately, steroids are associated with major side effects, such as osteopenia, weight gain, and cataract formation. Also, the stabilizing effect on muscle function decreases once the steroid therapy is discontinued. As the long-term results of steroid treatment are not known, its use in Duchenne muscular dystrophy remains controversial.

Deflazacort is an oxazolone derivative of prednisone that was thought to have a decreased prevalence of side effects^11-13. Because of this, several investigators have studied its use for conditions for which steroid administration would be required for a prolonged period, such as chronic inflammatory disorders^14,15. A randomized controlled trial showed that deflazacort was as effective as prednisone in preserving muscle strength in boys with Duchenne muscular dystrophy¹⁶.

In 1993, a trial of deflazacort was begun at our institution to determine its effect on physical and pulmonary function in patients with Duchenne muscular dystrophy¹⁷. Analysis of physical function included observation of the patient's ability to perform the following tasks without aids or assistance: walk outdoors, walk 10 m in less than fifteen seconds, walk up a flight of stairs in fifteen seconds, and get up from the floor. Our institutional review board approved this study, and the patients' parents gave informed consent for their child to participate. The parents of all seven to ten-year-old boys with Duchenne muscular dystrophy who were able to walk were offered the choice of giving their child deflazacort. Fifty-four boys were enrolled in the study; according to the decision made by their families, thirty children were treated with deflazacort (treatment group) and twenty-four were not (control group).

Although a randomized controlled trial design was not utilized, the patients in the treatment and control groups were quite similar, at the beginning of the study, with respect to pulmonary function, weight, height, and the performance of a variety of physical function tests (Table I). The study of physical function showed a dramatic difference between groups with regard to the rate of decline over time, with the patients taking deflazacort able to walk independently for two and a half years longer than the patients in the control group: the mean age at which the patients stopped walking was 12.3 years (range, nine to fifteen years) and 9.8 years (range, eight to thirteen years), respectively. There was also a significant decrease in the rate of decline in pulmonary function, with a mean of 88% (range, 58% to 90%) of the predicted value at fifteen years of age in the treatment group as opposed to 39% (range, 28% to 56%) in the control group. Because of these promising early results, the boys who were taking deflazacort continued to receive this medication. All patients in the initial study continued to be observed, and the current study was performed approximately nine years after enrollment. Since treatment with steroids stabilizes muscle strength and prolongs the patient's ability to walk, we hypothesized that it would also decrease the prevalence of scoliosis in these patients.

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TABLE I Characteristics of the Two Groups at Baseline

	Treatment Group	Control Group
No. of patients	30	24
Pulmonary function*(% predicted forced vital capacity)	85 ± 12	85 ± 15
Weight*(kg)	21 ± 6	21 ± 3
Age*(yr)	8.5 ± 3.8	8.9 ± 3.9

The values are given as the mean and standard deviation.

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TABLE I Characteristics of the Two Groups at Baseline

	Treatment Group	Control Group
No. of patients	30	24
Pulmonary function*(% predicted forced vital capacity)	85 ± 12	85 ± 15
Weight*(kg)	21 ± 6	21 ± 3
Age*(yr)	8.5 ± 3.8	8.9 ± 3.9

The values are given as the mean and standard deviation.

Materials and Methods

Materials and Methods | Results | Discussion | References

Fifty-four boys, between the ages of seven and ten years, who had a diagnosis of Duchenne muscular dystrophy confirmed by either a DNA test or a muscle biopsy and who were able to walk were enrolled in the study. The families of thirty of the patients chose deflazacort therapy for their children, and twenty-four did not. The reasons for not choosing deflazacort treatment included concerns about potential side effects and that the medication might not make a difference in the long term. The results of pulmonary function tests, the weights and heights of the patients, and physical function were similar for the two groups at the time of enrollment (Table I)¹⁷. Deflazacort was administered orally, once a day, at a dose of 0.9 mg/kg/day, which is at the high end of the range of acceptable doses for this drug. In a prior study, a similar dose (6 mg/kg/week) was utilized¹⁶. All patients were followed every four to six months with physical examination, pulmonary function testing, and, when indicated, radiographic examination. A pediatrician and an orthopaedic surgeon performed the physical examination, at which the height, weight, pulse, and blood pressure were recorded; the back was observed for deformity; the range of motion of the extremities was evaluated; and the chest was examined by auscultation and the abdomen, by palpation. A physiotherapist performed a musculoskeletal examination independent of the orthopaedic surgeon, and this examination included assessment of the strength of the upper and lower extremities, joint range of motion, and spinal alignment. Pulmonary function testing was carried out with use of a spirometer and included measurement of total vital capacity and forced vital capacity. The same nurse assisted all of the patients during the pulmonary function tests, and the same spirometry apparatus was utilized for the duration of the study. Percentages of predicted values were calculated with use of a computer, which obtained pulmonary function data directly from the spirometer. Liver function and electrolyte balance were monitored in the treatment group. Vision was evaluated annually by an ophthalmologist, whose examination included visualization of the eye chambers with an ophthalmoscope and testing of visual acuity with use of a standard eye chart. After the patients began to use a wheelchair full-time, they were evaluated annually by a cardiologist, whose examination included an echocardiogram.

There were no differences in the physiotherapy or orthotic regimens between the two groups, and the treatment group did not make additional visits to the Muscular Dystrophy Clinic. The cost of the medical treatment was borne by a provincial health insurance plan.

The development of other medical or musculoskeletal problems was recorded. Medical conditions other than the usual childhood illnesses developed in three patients. Gastric stress ulcers developed in one patient in the control group, and problems with gastric motility requiring referral to a gastroenterologist and medical management developed in one patient in each group.

Spinal radiographs were made once the patients began using a wheelchair on a full-time basis. One-meter-long posteroanterior and lateral spine radiographs were made with the patient sitting in a seat designed to hold the radiographic cassette at a fixed distance from the back, as previously reported^2,4. The entire spine from the sacrum to the first cervical vertebra was included on the radiographs. An observer blinded to the drug treatment measured the scoliosis with the Cobb technique¹⁸.

At our institution, surgery is recommended for scoliosis of =20° as well as for curves of <20° when pulmonary function has declined to <40% of predicted values. Operative records were reviewed for intraoperative blood loss, operative complications, and postoperative complications, including those related to instrumentation.

Kaplan-Meier survival curves were created to assess the risk of the development of a scoliotic curve of =20°. The proportion of patients in whom a curve developed in each year following enrollment in the study was compared between the treatment and control groups. The Kaplan-Meier analysis was performed with the aid of SPSS software (version 11.0; SPSS Science, Chicago, Illinois). Patients who did not comply with the medication regimen or who stopped taking the deflazacort because the family decided to discontinue the treatment were still analyzed in the treatment group. In the control group, surgery was performed on two patients with a curve of <20° (18° and 19°); in both, pulmonary function had declined to <40% of the predicted values. Although the natural history of this disease strongly suggests that scoliotic deformity of =20° would have developed in these two patients, we cannot know this for sure. For this reason, we analyzed the data on these patients in two ways: as if a curve of =20° had not developed and as if it had.

Results

Materials and Methods | Results | Discussion | References

The mean age at the time of follow-up was sixteen years (range, fifteen to eighteen years) in both groups. The mean duration of follow-up was 7.3 years (range, five to eight years) in the series as a whole, 7.2 years (range, five to eight years) in the treatment group, and 7.4 years (range, five to eight years) in the control group. There was no significant difference in the duration of follow-up between the treatment and control groups. All patients were alive at the time that this study was completed, and none had been lost to follow-up.

The treatment group had better pulmonary function (mean, 72% of the predicted value; range, 50% to 88%) than did the control group (mean, 35%; range, 20% to 48%). Three families discontinued the deflazacort treatment for their child. The discontinuation of the therapy was due to poor compliance in one case, excessive weight gain in another, and the family's belief that the medication was not making a difference in the third.

As previously reported¹⁷, the patients in the treatment group were able to walk independently for significantly longer (a mean of two and a half years longer) than those in the control group. They stopped walking at a mean age of 12.3 years (range, nine to fifteen years) compared with a mean age of 9.8 years (range, eight to thirteen years) in the control group (Table II). They were also able to climb a flight of stairs without any assistance for a mean of one and a half years longer. Manual muscle testing showed a relative decline in muscle strength, with a slower decline in the treatment group than in the controls. Five years after enrollment in the study, the muscle strength in the treatment group was, on the average, one-third grade better than that in the control group. There was no difference between the rate of decline in strength between the upper and lower extremities in either group. Cataracts, none of which interfered with visual acuity or required surgical treatment, developed in ten patients in the treatment group. The patients in the treatment group weighed a mean of 3.7 kg more than those in the control group. We did not routinely perform total body scans to look for stress fractures, but symptomatic stress fractures developed in three patients in the treatment group. One developed in the lumbar spine; one, in the distal part of the femur; and one, in the distal part of the tibia. All healed after a short period of immobilization and/or rest. The patients with stress fractures were evaluated and managed for osteopenia by an endocrinologist.

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TABLE II Comparison of the Two Groups at the Time of Latest Follow-up

	Treatment Group	Control Group
Age stopped walking*(yr)	12.3 ± 2.7	9.8 ± 1.8
Age stopped climbing stairs*(yr)	10.9 ± 1.9	9.2 ± 1.0
Pulmonary function*(% predicted forced vital capacity)	72 ± 11	35 ± 10
Age at op. (for patients who underwent spinal fusion and instrumentation)*	12.9 ± 2.4	15.1 ± 2.0

The values are given as the mean and standard deviation.

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TABLE II Comparison of the Two Groups at the Time of Latest Follow-up

	Treatment Group	Control Group
Age stopped walking*(yr)	12.3 ± 2.7	9.8 ± 1.8
Age stopped climbing stairs*(yr)	10.9 ± 1.9	9.2 ± 1.0
Pulmonary function*(% predicted forced vital capacity)	72 ± 11	35 ± 10
Age at op. (for patients who underwent spinal fusion and instrumentation)*	12.9 ± 2.4	15.1 ± 2.0

The values are given as the mean and standard deviation.

Three patients, all in the control group, had pulmonary function values of <40% of the predicted values. Two of these three patients underwent surgery when the curve measured <20°. The remaining patients in the series were advised to have surgery when the curve magnitude reached =20°. A curve of =20° developed in sixteen (76%) of the twenty-one remaining patients in the control group. Surgery was performed in fifteen of these patients, at an average age of thirteen years (range, eleven to seventeen years); at the time of surgery, the mean magnitude of the curve was 35° (range, 20° to 60°). In the treatment group, no patient had a pulmonary function value of <40% of the predicted value and only five (17%) had a curve of =20°. All five of these patients underwent surgery, at a mean age of fifteen years (range, eleven to seventeen years). There was no substantial difference between the treatment and control groups with regard to the estimated intraoperative blood loss (920 mL [range, 680 to 1250 mL]) and 960 mL [range, 600 to 1320 mL], respectively). No intraoperative complications were recorded in the treatment group, whereas two patients in the control group sustained fractures of the lamina during instrumentation. Two of the patients in the treatment group in whom scoliosis developed were among the three boys who had stopped using the deflazacort.

Kaplan-Meier analysis was performed, first with the assumption that scoliosis of =20° would have developed in the two patients in the control group who underwent surgery with a curve of <20° and then again with the assumption that scoliosis of =20° would not have developed in those two patients. Regardless of how these two patients were considered, Kaplan-Meier analysis (Fig. 1) showed a significant decrease in the rate of scoliosis with deflazacort treatment (p < 0.001).

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Fig. 1: Kaplan-Meier curve for the proportion of boys in whom a curve of =20° did not develop at each year following the enrollment in the trial. There was a significant difference (p < 0.001) in the chance of a curve of =20° developing between the treatment (tx) and control (no tx) groups. Starting four years after enrollment in the study, there was a significant difference between the treatment and control groups at each year (95% confidence intervals do not overlap). Ninety-five percent confidence intervals are given for the point eight years following enrollment in this study.

Discussion

Materials and Methods | Results | Discussion | References

Steroid use has previously been shown to slow the decline in pulmonary function and muscle strength in patients with Duchenne muscular dystrophy^9,10,14-17. We have now demonstrated that steroid use by these patients also attenuates the development of scoliosis. The patients in this study were treated with deflazacort for a much longer duration than has been reported in previous studies^9,10. Most investigators administered steroids only until the patients began using a wheelchair full-time. Our study showed an effect of treatment on skeletal deformity even while the patient was using the wheelchair full-time. It may be necessary to continue steroid therapy after the patient begins using a wheelchair full-time to alter the development of scoliosis, as scoliosis developed at a higher rate in the patients who stopped using the medication in our study. The future of the spines of these patients once skeletal maturity is reached is unclear. However, it may be similar to that in individuals who become paraplegic—i.e., scoliosis may occur in skeletally immature individuals but not in skeletally mature individuals^19,20.

This study was not a randomized controlled trial. Although the patients were well matched for baseline characteristics and the family rather than the physicians chose the treatment, it is possible that there was a selection bias. For example, it is possible that the families who saw more physical deterioration in their child decided against steroid treatment and this resulted in the selection of a less severely affected treatment group. Because of ethical issues surrounding the use of a medication associated with potential side effects, a randomized controlled trial to determine changes in the prevalence of scoliosis with long-term steroid treatment may never be performed in this patient population. Despite this short-coming, the difference that we detected between the groups was quite large. In addition, the outcome in the treatment group was substantially different from the reported natural history of scoliosis in Duchenne muscular dystrophy^1,3,5. Thus, it is likely that longer-term steroid treatment significantly attenuates the development of scoliosis.

A number of side effects are associated with steroid use. There is the risk of cataracts, which developed in ten (33%) of the thirty patients in our treatment group. None of these patients had changes in their visual acuity and thus far have not required surgery for the cataracts, but it is possible that they will require surgical intervention in the future. Steroids may cause excessive weight gain, and the patients in our treatment group weighed a mean of 3.7 kg more than the controls. Another troubling potential side effect is osteopenia, which can result in an increased rate of fractures; three patients in the treatment group had symptomatic stress fractures. All of the fractures healed with immobilization and rest, but all three patients were evaluated and treated by an endocrinologist for low bone density. It is also possible that steroid-induced osteopenia makes spinal surgery more difficult, with an increased rate of laminar fracture during instrumentation, or that it results in increased intraoperative blood loss. We did not observe either of these problems in the five patients in the treatment group who underwent surgery. However, such problems may become apparent in studies of larger numbers of patients. In the future, treatment with bisphosphonates, which have been shown to reduce osteopenia in adults treated with steroids, may solve the problem of osteopenia in these patients²¹.

While the long-term implications of steroid treatment for scoliosis in patients with Duchenne muscular dystrophy remain unknown, this study showed that steroid treatment does slow the development of scoliosis. Most of the patients who were treated with deflazacort did not have a curve of =20° by sixteen years of age. The future for these patients is still unclear. It is not known whether progressive curves will develop later in life or whether there is a subset of patients who will never have a curve severe enough to require surgical intervention. A troubling possibility is that the patients may require surgery at a later time, when pulmonary or cardiac function is so poor that surgery is more dangerous. We have been monitoring the cardiac and pulmonary functions in these boys closely, and at the time of writing the pulmonary function measures in the treatment group have remained close to those observed at baseline. Cardiac function has also been closely monitored and has remained close to normal.

There are risks and benefits associated with the use of steroid medications in patients with Duchenne muscular dystrophy, and decisions about their use must be made on an individual basis by the patients and their parents in collaboration with medical caregivers. At the very least, deflazacort treatment prolongs the time before spinal surgery is needed. It is possible that the slowed progression of scoliosis during adolescence, a time of rapid spinal growth, may obviate the need for surgery in a greater proportion of patients than expected. However, it is also possible that spinal deformity will occur later in life and be associated with greater surgical risks because of poor pulmonary or cardiac function. Only longer-term evaluation of these patients will determine the ultimate outcome.

References

Materials and Methods | Results | Discussion | References

Wilkins KE, Gibson DA. The patterns of spinal deformity in Duchenne muscular dystrophy. J Bone Joint Surg Am.1976;58: 24-32.5824 1976

Smith AD, Koreska J, Moseley CF. Progression of scoliosis in Duchenne muscular dystrophy. J Bone Joint Surg Am.1989;71: 1066-74.711066 1989

Oda T, Shimizu N, Yonenobu K, Ono K, Nabeshima T, Kyoh S. Longitudinal study of spinal deformity in Duchenne muscular dystrophy. J Pediatr Orthop.1993;13: 478-88.13478 1993 [PubMed][CrossRef]

Alman BA, Kim HK. Pelvic obliquity after fusion of the spine in Duchenne muscular dystrophy. J Bone Joint Surg Br.1999;81: 821-4.81821 1999 [PubMed][CrossRef]

Weimann RL, Gibson DA, Moseley CF, Jones DC. Surgical stabilization of the spine in Duchenne muscular dystrophy. Spine.1983;8: 776-80.8776 1983 [PubMed][CrossRef]

Malhotra SB, Hart KA, Klamut HJ, Thomas NS, Bodrug SE, Burghes AH, Bobrow M, Harper PS, Thompson MW, Ray PN, Worton RG. Frame-shift deletions in patients with Duchenne and Becker muscular dystrophy. Science.1988;242: 755-9.242755 1988 [PubMed][CrossRef]

Zubrzycka-Gaarn EE, Bulman DE, Karpati G, Burghes AH, Belfall B, Klamut HJ, Talbot J, Hodges RS, Ray PN, Worton RG. The Duchenne muscular dystrophy gene product is localized in sarcolemma of human skeletal muscle. Nature.1988;333: 466-9.333466 1988 [PubMed][CrossRef]

Jacobs SC, Bootsma AL, Willems PW, Bar PR, Wokke JH. Prednisone can protect against exercise-induced muscle damage. J Neurol.1996;243: 410-6.243410 1996 [PubMed][CrossRef]

Griggs RC, Moxley RT 3rd, Mendell JR, Fenichel GM, Brooke MH, Pestronk A, Miller JP. Prednisone in Duchenne dystrophy. A randomized, controlled trial defining the time course and dose response. Clinical Investigation of Duchenne Dystrophy Group. Arch Neurol.1991;48: 383-8.48383 1991

Mendell JR, Moxley RT, Griggs RC, Brooke MH, Fenichel GM, Miller JP, King W, Signore L, Pandya S, Florence J, Schierbeker J, Robinson J, Kasier K, Mandel S, Gilder B. Randomized, double-blind six-month trial of prednisone in Duchenne's muscular dystrophy. N Engl J Med.1989;320: 1592-7.3201592 1989 [PubMed][CrossRef]

Hahn BH, Hahn TJ. Methods for reducing undesirable side effects of glucocorticoids. Adv Exp Med Biol.1984;171: 301-11.171301 1984

Gray RE, Doherty SM, Galloway J, Coulton L, de Broe M, Kanis JA. A double-blind study of deflazacort and prednisone in patients with chronic inflammatory disorders. Arthritis Rheum.1991;34: 287-95.34287 1991 [PubMed][CrossRef]

Arizon JM, Anguita M, Valles F, Montero A, Sancho M, Lopez-Rubio F, Latre JM, Calleja F, Casares J, Roman M, Segura C, Concha M. Preliminary experience with deflazacort, a new synthetic steroid with fewer undesirable side effects, in heart transplant patients. J Heart Lung Transplant.1993;12: 445-9.12445 1993 [PubMed]

Angelini C, Pegoraro E, Turella E, Intino MT, Pini A, Costa C. Deflazacort in Duchenne dystrophy: study of long-term effect. Muscle Nerve. 1994;17:386-91. Erratum in: Muscle Nerve.1994;17: 833.17833 1994 [CrossRef]

Mesa LE, Dubrovsky AL, Corderi J, Marco P, Flores D. Steroids in Duchenne muscular dystrophy—deflazacort trial. Neuromuscul Disord.1991;1: 261-6.1261 1991 [PubMed][CrossRef]

Bonifati MD, Ruzza G, Bonometto P, Berardinelli A, Gorni K, Orcesi S, Lanzi G, Angelini C. A multicenter, double-blind, randomized trial of deflazacort versus prednisone in Duchenne muscular dystrophy. Muscle Nerve.2000; 23: 1344-7.231344 2000 [PubMed][CrossRef]

Biggar WD, Gingras M, Fehlings DL, Harris VA, Steele CA. Deflazacort treatment of Duchenne muscular dystrophy. J Pediatr.2001;138: 45-50.13845 2001 [PubMed][CrossRef]

Morrissy RT, Goldsmith GS, Hall EC, Kehl D, Cowie GH. Measurement of the Cobb angle on radiographs of patients who have scoliosis. Evaluation of intrinsic error. J Bone Joint Surg Am.1990;72: 320-7.72320 1990

Mayfield JK, Erkkila JC, Winter RB. Spine deformity subsequent to acquired childhood spinal cord injury. J Bone Joint Surg Am.1981;63: 1401-11.631401 1981 [PubMed]

Lancourt JE, Dickson JH, Carter RE. Paralytic spinal deformity following traumatic spinal-cord injury in children and adolescents. J Bone Joint Surg Am.1981;63: 47-53.6347 1981 [PubMed]

Reid IR, Schooler BA, Stewart AW. Prevention of glucocorticoid-induced osteoporosis. J Bone Miner Res.1990;5: 619-23.5619 1990 [CrossRef]

Topics

duchenne's muscular dystrophy ; steroids ; scoliosis ; pulmonary function ; steroid therapy

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Benjamin A. Alman, S. Naweed Raza, W. Douglas Biggar; Steroid Treatment and the Development of Scoliosis in Males with Duchenne Muscular Dystrophy. The Journal of Bone & Joint Surgery. 2004 Mar;86(3):519-524.

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