Biomedical subjects

Gurkan Erkula

Publications and source records attributed to Gurkan Erkula.

8 recordsLinked to original sources

PubMed2007

Symposium on the musculoskeletal aspects of Marfan syndrome: meeting report and state of the science.

The National Marfan Foundation sponsored a symposium in August 2005 to review recent progress in the area of Marfan-related musculoskeletal research. Orthopaedic surgeons, molecular geneticists, medical geneticists, and pain specialists met to review a variety of topics. This report reviews and summarizes the proceedings of the symposium, with emphasis on future directions for study that were identified in the course of the meeting. Areas covered include clinical detection, diagnosis, growth, spine deformity, molecular mechanisms, dural ectasia, protrusio acetabuli, and pain in Marfan syndrome.

Acetabulum↗

PubMed2006

Protrusio acetabuli in Marfan syndrome: age-related prevalence and associated hip function.

BACKGROUND: Protrusio acetabuli is known to occur in patients with Marfan syndrome, but its prevalence, its effects on hip function, and its possible association with the subsequent development of degenerative hip disease have not been studied in a large population. Nevertheless, some clinicians have recommended prophylactic hip surgery for preadolescents with Marfan syndrome and protrusio acetabuli. METHODS: We performed a cross-sectional study of 173 patients (346 hips) with Marfan syndrome who were interviewed and examined for calculation of the Iowa hip score. Anteroposterior radiographs of the pelvis were made, and two radiographic indices of acetabular depth were measured: (1) the center-edge angle of Wiberg and (2) the acetabular-ilioischial distance. The presence of protrusio was defined with use of two extant definitions: (1) a center-edge angle of >50 degrees or (2) an acetabular-ilioischial distance of >/=3 mm in male patients or >/=6 mm in female patients. Linear regression analyses were performed between these radiographic indices of acetabular depth and patient age, Iowa hip scores, the magnitude of the radiographic joint space, and range of motion. RESULTS: The prevalence of protrusio acetabuli was 27% according to the center-edge angle criterion and 16% according to the acetabular-ilioischial distance criterion. The prevalence of protrusio increased until the age of twenty years and remained stable after the age of twenty years. Slight but significant negative correlations were detected between the two radiographic indices of acetabular depth and both the Iowa hip score and the summed range of motion (p < 0.02 for all). No significant relationship was found between the two radiographic indices and pain scores. In patients with Marfan syndrome who were more than forty years old, the Iowa hip scores for hips with protrusio were not significantly lower than those for hips without protrusio. CONCLUSIONS: In patients with Marfan syndrome, the prevalence of protrusio acetabuli increases during the first two decades of life and then plateaus in terms of both population-wide prevalence and radiographic severity. In this population, protrusio generally is not associated with severely problematic hip function but it is associated with slightly decreased range of motion of the hip. We concluded that prophylactic surgical intervention is not indicated for most patients with Marfan syndrome who have a radiographic diagnosis of protrusio.

Acetabulum↗

PubMed2004

Evaluation of the muscles around the knee in rabbits whose anterior cruciate and/or medial collateral ligaments were dissected.

INTRODUCTION: The biological response of the muscles around the knee in chronic ligamentous instability was investigated in an animal study. MATERIALS AND METHODS: There were four groups of 6- to 9-month-old adult New Zealand albino rabbits (2500-3300 g). The animals were divided into groups according to the ligament that was surgically sectioned: group A anterior cruciate ligament (ACL), group B medial collateral ligament (MCL), group C both ACL and MCL, and group D served as the control group undergoing no surgical intervention. Three months after surgery, biopsy specimens of the vastus lateralis, rectus femoris, biceps femoris, extensor digitorum longus, and gastrocnemius muscles of the rabbits were obtained. Electron-microscopic cross-sections of the biopsy specimens were evaluated using the new predetermined atrophy parameters. RESULTS: Atrophy was found in the biopsy specimens of the quadriceps muscles in groups A and C (p<0.005). Unimportant changes were seen in the hamstrings, extensor digitorum longus, and gastrocnemius muscles (p>0.05). Only in the group undergoing MCL dissection were no changes observed in the muscles (p>0.05). CONCLUSION: It is concluded that ACL lesions affect the biomechanics of the knee negatively and this situation causes atrophy, especially in the quadriceps muscle. An MCL lesion alone does not cause an important problem in the surrounding musculature, probably because of its spontaneous healing capacity. New criteria for assessment of atrophy in the muscles employing electron-microscopic evaluation are suggested.

Animals↗

PubMed2003

Rib deformity in scoliosis.

Rib deformity in scoliosis is of interest because it may help in the diagnosis, and also, in some pronounced cases, it may need correction by costoplasty. There are, however, debates about its use in diagnosis, because some authors think that rib deformity is not closely related to either the magnitude or the extent of rotation of the curve. In order to define the relation between rib deformity and scoliosis, 11 patients were recruited who were to undergo scoliosis surgery and thoracoplasty, and anteroposterior (AP) T1-S1 standing radiographs, computerized tomography (CT) scans, and three-dimensional (3D) reconstructions were obtained. From the radiographs, the most rotated vertebra, the Cobb angle, the apex and the type of the curve were determined. From the CT scans and 3D reconstructions, the exact level of the rib deformity measured was matched with the corresponding vertebral level. In this way, the most rotated vertebra and the most prominent part of the rib cage deformity were identified. The most rotated vertebra was found to be at the same level in both radiographs and CT scans in only five patients. In the rest of the patients, CT scans showed it either one level higher or lower than it appeared on the radiograph. The most prominent part of the rib cage deformity was at the same level as the most rotated vertebra in two patients, and in the rest of the patients it was one, two or three vertebral levels lower. There was no association between the Cobb angle, vertebral rotation and rib deformity. A CT scan is necessary preoperatively in patients who will undergo a costoplasty, to determine the exact levels of the prominence. However, a scanogram or a 3D reconstruction is required for exactly matching the most prominent part of the rib cage deformity to the corresponding vertebral level.

Adolescent↗

PubMed2003

The spinoglenoid ligament: an anatomic study.

The spinoglenoid ligament and its anatomic variations are described in 27 shoulders from 15 cadavers. In each shoulder one or two distinct spinoglenoid ligaments originated from the base of the spine of the scapula; they inserted on the neck of the scapula or the shoulder joint capsule. In the 19 shoulders in which only one spinoglenoid ligament was present, it inserted into the neck of the scapula in 14 cases and into the shoulder joint capsule in five instances. In the eight shoulders in which there were two ligaments, one inserted into the neck of the scapula and the other into the shoulder joint capsule. We did not observe any hypertrophic spinoglenoid ligaments that may have compressed the suprascapular nerve.

Cadaver↗

PubMed2002

Spine deformity correction in Marfan syndrome.

STUDY DESIGN: Retrospective review of records and radiographs with postoperative questionnaire of patients with Marfan syndrome receiving surgical correction for spinal deformity. OBJECTIVE: To characterize and analyze the challenges of surgical correction for spinal deformity in Marfan syndrome and to present recommendations that may reduce morbidity and increase success for these procedures. SUMMARY OF BACKGROUND DATA: Spinal deformities are common in Marfan syndrome and usually refractory to conservative management. The few, smaller studies of surgical intervention reported on earlier surgical techniques but suggested increased complication rates. METHODS: Records and radiographs of 39 patients with confirmed Marfan syndrome who underwent surgical management at either of two institutions for primary scoliosis (n = 26), kyphosis (n = 7), or deformity secondary to previous surgery elsewhere (n = 6) were reviewed. Presentation features, complications, and therapeutic results were analyzed. Low back outcome scores were generated from a questionnaire given to patients after surgery. RESULTS: Increased blood loss and rates of infection (10%), dural tear (8%), instrumentation fixation failure (21%), pseudarthrosis (10%), and coronal (8%) and sagittal (21%) curve decompensation were noted. Infection was often associated with dural tear and decompensation with extreme correction. One patient died of valvular insufficiency 11 weeks after surgery. CONCLUSION: The cardiopulmonary condition of patients with Marfan syndrome should be evaluated and planned for before surgery. Preoperative computed tomograph to assess bony adequacy for fixation and magnetic resonance imaging to evaluate dural ectasia are indicated. Attention paid to the sagittal profile, extension of fusion to vertebrae neutral and stable in both planes, minimization of soft tissue dissection, and avoidance of extreme correction may prevent curve decompensation.

Adolescent↗

PubMed2002

Growth and maturation in Marfan syndrome.

Understanding the growth pattern in Marfan syndrome is important for prediction of expected growth, prevention of excessive growth by hormone therapy, timing of surgical epiphysiodesis for cessation of growth, and instituting brace treatment for scoliosis. In this study, we analyze growth patterns and generate growth charts for persons with Marfan syndrome. From the charts of 180 clinically diagnosed Marfan patients, longitudinal height and weight measurements were obtained. From this data, growth charts and growth velocity charts were generated for males and females. Skeletal maturation was studied by determining the Risser signs from the x-rays of 71 males and 56 females. From 22 female patients, age of menarche was available and retrieved either by reviewing the charts or contacting the patients. Mean length at birth was 53 +/- 4.4 cm for males and 52.5 +/- 3.5 cm for females. Mean final height was 191.3 +/- 9 cm for males and 175.4 +/- 8.2 cm for females. Mean birth weight was 3.51 +/- 0.74 kg for males and 3.48 +/- 0.68 kg for females. The puberty-associated peak in growth velocity was 2.4 years earlier than the gender-matched general population for males with Marfan syndrome and 2.2 years earlier for females. Age of menarche was 11.7 +/- 2 years of age, which is also early compared to the general population. This study suggests that the growth spurt and pubertal skeletal maturation occur early in Marfan syndrome. The growth curves generated should help more accurately predict adult stature, as well as monitor progression toward it.

Adolescent↗

PubMedDate not supplied

Plain radiography of the lumbosacral spine in Marfan syndrome.

BACKGROUND CONTEXT: Marfan syndrome is a connective tissue disorder that results from a defect in the production of fibrillin. These patients tend to have several osseous anomalies of the lumbosacral spine. PURPOSE: This study examines the effectiveness of plain radiographic findings in predicting Marfan syndrome. STUDY DESIGN/SETTING: Case-control study. PATIENT SAMPLE: Fourteen height-matched controls and 33 patients with Marfan syndrome were obtained from our genetics clinic or through the National Marfan Foundation. OUTCOME MEASURES: Determined using measurements taken on plain radiographs. METHODS: Five measurements were acquired of the lumbosacral spine from the radiographs of both groups: interpedicular distance, scalloping value, sagittal canal diameter, vertebral body width and transverse process width. RESULTS: The following measurements were significantly larger in patients with Marfan syndrome: interpedicular distance at L1-L5 (p<.0001); sagittal diameters of the vertebral canal at L4-S2 (p<.01); transverse process to vertebral body width ratio at L2-L5 (p<.01). There was no significant difference in the scalloping values from L1-L5 between the patients with Marfan syndrome and the controls. A multivariate regression analysis generated the following criteria for plain film diagnosis of Marfan syndrome (two criteria need to be met for diagnosis): interpedicular distance at L5 greater than or equal to 36.0 mm, sagittal diameter at L5 greater than or equal to 13.5 mm or transverse process to vertebral width ratio at L3 greater than or equal to 2.25. CONCLUSION: Based on this criteria, patients can be diagnosed with Marfan syndrome with a high sensitivity (81.8%) but a low specificity (58.3%). Thus, plain radiography can be a useful means of screening patients with Marfan syndrome.

Adult↗