Abstract:
BACKGROUND:Osteogenesis imperfecta (OI) is a heterogeneous group of collagen-related disorders characterized by osteopenia, bone fractures, spine deformities, and nonskeletal complications. Cardiopulmonary complications are the major cause of morbidity and mortality in adults with OI. The cause of such problems was often attributed solely to the presence of large scoliosis curves affecting pulmonary function and, indirectly, cardiovascular health. However, recent studies suggest this may not be the case. Therefore, determining the relationships and causative agents of cardiopulmonary problems in patients with OI, specifically pulmonary impairment, is important to improving the overall wellbeing, quality of life, and survival of these patients. QUESTIONS/PURPOSES:(1) Is cardiopulmonary fitness in OI solely related to the presence of scoliosis? (2) What is the prevalence of heart and lung complications in this adult population? (3) Does the presence of pulmonary impairment impact quality of life in adults with OI? METHODS:This is a prospective observational cross-sectional study. Within 1 year, each participant (n = 30) completed pulmonary function testing, echocardiogram, ECG, chest CT, AP spine radiography, and quality-of-life assessments (SF-36, St. George's Respiratory Questionnaire, Functional Outcomes of Sleep Questionnaire, and Pittsburgh Sleep Quality Index). In terms of pulmonary function, we differentiated restrictive and obstructive physiology using the ratio of forced expiratory volume over one second to forced vital capacity (FEV1/FVC), with restrictive lung physiology defined as FEV1/FVC > 0.8 and obstructive lung physiology as FEV1/FVC < 0.7. Spine radiographs were evaluated for scoliosis. Chest CT images were reviewed to qualitatively assess the lungs. The statistical analysis involved a Kruskall-Wallis test with Bonferroni's correction and a bivariate correlation analysis using Spearman's rho correlation coefficient (p < 0.05). RESULTS:Sixteen of 23 participants with restrictive lung physiology had scoliosis; their ages ranged from 19 years to 67 years. There was no correlation between the magnitude of the scoliosis curve and deficient pulmonary function (R = 0.08; p = 0.68). Seven participants had normal pulmonary function. The average scoliosis curve was 44 ± 29°. Thirteen participants had abnormal ECG findings while 10 had abnormal echocardiogram results. All but two individuals with abnormal chest CT results were found to have bronchial wall thickening. There were no differences in pulmonary or cardiac findings between OI types, except for FVC and total lung capacity, which were lower in individuals with Type III OI than in those with other types of OI. FEV1/FVC correlated with St. George's Respiratory Questionnaire (R = 0.429; p = 0.02) but not with Functional Outcomes of Sleep Questionnaire (R = -0.26; p = 0.19) or SF-36 scores (physical component summary: R = -0.037, p = 0.85; mental component summary: R = -0.204, p = 0.29). CONCLUSIONS:The lack of a relationship between decreased pulmonary function and the severity of scoliosis suggests that restrictive lung physiology in this population is likely because of factors intrinsic to OI and not entirely because of thoracic cage deformities. The fact that pulmonary impairment influences self-perceived quality of life exemplifies how detrimental such complications may be to everyday functioning. This also reinforces the importance of determining the underlying cause of cardiopulmonary impairment in this population to set clear clinical guidelines of care. LEVEL OF EVIDENCE:Level II, prognostic study.
journal_name
Clin Orthop Relat Resjournal_title
Clinical orthopaedics and related researchauthors
Khan SI,Yonko EA,Carter EM,Dyer D,Sandhaus RA,Raggio CLdoi
10.1097/CORR.0000000000001400subject
Has Abstractpub_date
2020-12-01 00:00:00pages
2833-2843issue
12eissn
0009-921Xissn
1528-1132journal_volume
478pub_type
杂志文章abstract::Pseudothrombophlebitis syndrome is the occurrence of calf pain and swelling caused by extrinsic compression of the popliteal vessels by an enlarging Baker's cyst or by calf inflammation that occurs as the result of a ruptured Baker's cyst. Few cases of pseudothrombophlebitis syndrome have been reported in patients les...
journal_title:Clinical orthopaedics and related research
pub_type: 杂志文章
doi:
更新日期:1994-11-01 00:00:00
abstract::The successful treatment of osteomyelitis with commercially prepared gentamicin-polymethylmethacrylate (PMMA) (Septopal) beads and surgical debridement has led to the use of this technique in the United States. However, commercially prepared gentamicin-PMMA beads are not currently available to orthopedic surgeons in t...
journal_title:Clinical orthopaedics and related research
pub_type: 杂志文章
doi:
更新日期:1992-11-01 00:00:00
abstract:BACKGROUND:Patient satisfaction surveys play an increasingly important role in United States healthcare policy and serve as a marker of provided physician services. In attempts to improve the patient's clinical experience, focus is often placed on components of the healthcare system such as provider interaction and oth...
journal_title:Clinical orthopaedics and related research
pub_type: 杂志文章
doi:10.1097/CORR.0000000000000927
更新日期:2019-12-01 00:00:00
abstract::Articular cartilage repair remains a clinical and scientific challenge with increasing interest focused on the transplantation of chondrogenic cells. This study evaluated the repair response during a 1-year period after implantation of allogenic perichondrium cell polylactic acid composite grafts into 3.7 x 5 mm osteo...
journal_title:Clinical orthopaedics and related research
pub_type: 杂志文章
doi:10.1097/00003086-199707000-00029
更新日期:1997-07-01 00:00:00
abstract::Twenty-three patients with idiopathic scoliosis were tested for upper extremity proprioceptive function. All subjects had documented progression of deformity, with an average curvature of 34 degrees. The average ages for scoliotics and 18 control subjects were 16.1 and 20.8 years, respectively. Controls had no spinal ...
journal_title:Clinical orthopaedics and related research
pub_type: 杂志文章
doi:
更新日期:1986-12-01 00:00:00
abstract::When inserting screws into a vertebral pedicle, the surgeon usually exposes the back part of the vertebra and uses his or her anatomic knowledge to align the drill in the proper direction. A slight error in direction may result in an important error in the position of the tip of the screw. This is done with no direct ...
journal_title:Clinical orthopaedics and related research
pub_type: 临床试验,杂志文章
doi:10.1097/00003086-199704000-00011
更新日期:1997-04-01 00:00:00
abstract::Neonatal osteomyelitis has been frequently reported, but only rarely as a complication of repeated heel punctures. The need for constant monitoring of premature newborns and those having low birthweight and serious deformities necessitates repeated blood samples, usually obtained by heel puncture. Os calcis osteomyeli...
journal_title:Clinical orthopaedics and related research
pub_type: 杂志文章
doi:
更新日期:1981-05-01 00:00:00
abstract::The effect of immobility on the biomechanical properties of an anterior cruciate bone-ligament-bone unit of rhesus monkey is reported. Wild primates were used and the captivity time was minimized to lessen disuse changes from cage confinement. The ligament-bone specimens were tested in tension to failure under high st...
journal_title:Clinical orthopaedics and related research
pub_type: 杂志文章
doi:
更新日期:1977-03-01 00:00:00
abstract::The current study analyzed subjects having a total knee arthroplasty to determine the incidence of condylar lift-off and correlate lift-off with the alignment of the femoral component with respect to the transepicondylar axis. Twenty-five subjects, implanted with a posterior stabilized total knee prosthesis, were aske...
journal_title:Clinical orthopaedics and related research
pub_type: 杂志文章
doi:10.1097/00003086-200210000-00022
更新日期:2002-10-01 00:00:00
abstract::Structural changes in the anatomy of the soft tissues as a result of long-standing congenital dislocation of the hip (CDH) are seldom described in the literature. Similarly, the anatomy of a hip 52 years after a pelvic support osteotomy for CDH seems not to have been previously observed. A long-term follow-up autopsy ...
journal_title:Clinical orthopaedics and related research
pub_type: 杂志文章
doi:
更新日期:1991-03-01 00:00:00
abstract::Thirty infected total knee arthroplasties were investigated in 29 patients over an average interval of 42 months. Eleven infections began in the immediate perioperative period. Six developed from postoperative wound-healing problems. The remainder were late infections. Staphylococcus was found in 16 infections, gram-n...
journal_title:Clinical orthopaedics and related research
pub_type: 杂志文章
doi:
更新日期:1985-10-01 00:00:00
abstract::Theoretical consideration of the factors involved in maintaining vascular equilibrium and experimental evidence in animals and man indicate that at high tissue pressures there may be a sharp decline in blood flow and, in certain situations, complete cessation of blood flow even at tissue pressures below mean arterial ...
journal_title:Clinical orthopaedics and related research
pub_type: 杂志文章
doi:10.1097/00003086-197511000-00004
更新日期:1975-11-01 00:00:00
abstract::Eight patients with unstable fractures involving the articular surface and metaphyseal-diaphyseal bone of the proximal or distal tibia associated with severe soft-tissue injury or compounding wound were treated with irrigation, debridement, tetanus inoculation, antibiotic prophylaxis, and combined internal fixation wi...
journal_title:Clinical orthopaedics and related research
pub_type: 杂志文章
doi:
更新日期:1990-07-01 00:00:00
abstract::Tension is necessary to maintain and restore the mechanical properties of soft connective tissues. Conversely, reduced tension states such as produced by immobilization weaken mechanical properties and facilitate joint contracture. We assessed the effect of low torque-long duration stretching to increase the range of ...
journal_title:Clinical orthopaedics and related research
pub_type: 杂志文章
doi:10.1097/BLO.0b013e31803212bf
更新日期:2007-03-01 00:00:00
abstract::Two unrelated patients with Kniest dysplasia had the same substitution of a CG dinucleotide in exon 12 of the COL2A1 gene that resulted in alternative splicing and interruption of the triple helix of a1(II) chains of Type II collagen. In early childhood, they shared the typical clinical and radiographic features of Kn...
journal_title:Clinical orthopaedics and related research
pub_type: 杂志文章
doi:
更新日期:1997-08-01 00:00:00
abstract::Isolated human chondrocytes derived from healthy and osteoarthritic (OA) cartilage were cultured in high density in a newly designed microculture system. The severity of OA was graded according to a modified histopathologic score originally described by Mankin et al. Chondrocytes from adult patients with OA showed 35S...
journal_title:Clinical orthopaedics and related research
pub_type: 杂志文章
doi:
更新日期:1989-05-01 00:00:00
abstract::If the underlying pathology of the syndrome of fat embolism is the presence of circulating emboli of neutral fat with adherent platelets and red blood cells, then one could expect certain changes in normal vascular cellular elements and fat metabolism, with resultant alterations in pulmonary function. In our series of...
journal_title:Clinical orthopaedics and related research
pub_type: 杂志文章
doi:
更新日期:1976-03-01 00:00:00
abstract::A model was developed to identify and compare the local wound factors that induce acute osteomyelitis in a prospective, controlled investigation. When compared with wounds containing either virulent bacteria or dead bone, statistical analysis disclosed a significant increase in the incidence of osteomyelitis when viru...
journal_title:Clinical orthopaedics and related research
pub_type: 杂志文章
doi:
更新日期:1993-01-01 00:00:00
abstract:UNLABELLED:Patient demand and surgeon interest in hip resurfacing has recently increased, but surgeons in the United States are relatively inexperienced with this procedure. We determined the learning curve associated with hip resurfacing and compared the rate of early complications of the first 650 hip resurfacings be...
journal_title:Clinical orthopaedics and related research
pub_type: 杂志文章
doi:10.1007/s11999-009-1106-1
更新日期:2010-02-01 00:00:00
abstract:BACKGROUND:Stiffness after a TKA can cause patient dissatisfaction and diminished function, therefore it is important to characterize predictors of ROM after TKA. Studies of AP translation in conscious individuals disagree whether AP translation affects maximum knee flexion angle after implantation of a highly congruen...
journal_title:Clinical orthopaedics and related research
pub_type: 杂志文章
doi:10.1007/s11999-013-3274-2
更新日期:2014-02-01 00:00:00
abstract::Clinical failures of all-polyethylene tibial components in total knee arthroplasty generally have been failures of design, not materials. The current study was designed to compare a modern congruent all-polyethylene tibial component with a metal-backed tibial component with the same articular design and geometry. All ...
journal_title:Clinical orthopaedics and related research
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1097/00003086-200011000-00015
更新日期:2000-11-01 00:00:00
abstract::The spherocentric knee, designed to allow triaxial rotation and provide intrinsic stability, includes desirable design features of metal on high density polyethelene bearing surfaces, metal support for all plastic components, metal-cement-bone interfacing for all fixation surfaces, sufficient strength to eliminate fat...
journal_title:Clinical orthopaedics and related research
pub_type: 杂志文章
doi:
更新日期:1979-11-01 00:00:00
abstract::A posterior cruciate ligament avulsion fracture (PCLAF) of the knee can be repaired through a large approach. A new technique based on internal fixation under arthroscopic control employed a posterolateral puncture. The fracture was reduced and the osseous fragment transfixed using a new forceps guide. The osseous fra...
journal_title:Clinical orthopaedics and related research
pub_type: 杂志文章
doi:
更新日期:1988-12-01 00:00:00
abstract::Cases of longstanding (median, 60 months) tarsal tunnel syndrome were decompressed surgically in 14 female and four male patients. Patients reported intermittent dysesthesia, paresthesia, or anesthesia at the medial plantar aspect of the foot. Symptoms were aggravated by physical activities. Previous trauma was noted ...
journal_title:Clinical orthopaedics and related research
pub_type: 杂志文章
doi:
更新日期:1997-10-01 00:00:00
abstract::Thirty-two consecutive subtrochanteric fractures in 32 patients were stabilized using the Medoff sliding plate and were followed up prospectively for 1 year. Two patients died during the first postoperative year. Twenty-nine of the remaining 30 (97%) fractures united by 1-year followup. Two types of plate dynamization...
journal_title:Clinical orthopaedics and related research
pub_type: 杂志文章
doi:
更新日期:1998-03-01 00:00:00
abstract::Static and fatigue tests of the small Orthofix unilateral external fixator frame and its main components were performed. The use of six pins increased torsional rigidity, but this configuration failed at lower bending loads when compared to the four-pin configuration, reflecting the uneven holding strength of the pin ...
journal_title:Clinical orthopaedics and related research
pub_type: 杂志文章
doi:
更新日期:1990-04-01 00:00:00
abstract::The differentiation of connective tissue outgrowths of adult bone marrow in response to the organic matrix of bone was observed in tissue culture by correlated histologic, electron microscopic, biochemical, and radioisotope-labeling methods. On a substratum of bone matrix gelatin, myelogenous cells degenerate and disa...
journal_title:Clinical orthopaedics and related research
pub_type: 杂志文章
doi:
更新日期:1981-01-01 00:00:00
abstract::Since 1941, distal clavicle excision has been a reliable technique for alleviating pain caused by acromioclavicular joint arthritis. Disadvantages of the procedure include permanent shoulder weakness, a lengthy recovery time ranging from weeks to months before useful function of the extremity returns, and lack of cosm...
journal_title:Clinical orthopaedics and related research
pub_type: 杂志文章
doi:
更新日期:1994-04-01 00:00:00
abstract::Our presently inadequate knowledge of the functional anatomy of the deep branch of the ulnar nerve handicaps our management of ulnar nerve lesions. The extensive anatomical variations in the distribution of this nerve preclude adherence to a textbook pattern of innervation. Electrophysiological recordings of single se...
journal_title:Clinical orthopaedics and related research
pub_type: 杂志文章
doi:
更新日期:1977-10-01 00:00:00
abstract:UNLABELLED:Ankle arthroplasty is increasingly used to treat advanced ankle arthritis. Earlier prostheses have given way to second-generation implants, on which we are accumulating medium-term data. The Scandinavian Total Ankle Replacement (STAR) is a three-component uncemented implant in wide use in Europe and the only...
journal_title:Clinical orthopaedics and related research
pub_type: 杂志文章
doi:10.1007/s11999-009-0971-y
更新日期:2010-04-01 00:00:00