Abstract:
:Lysinuric protein intolerance (LPI) is an autosomal recessive disease caused by defective transport of the cationic amino acids lysine, arginine, and ornithine at the cell membrane. About 80 patients with LPI have been described worldwide, almost half of them in Finland. The symptoms appear in early childhood as a failure to thrive, growth retardation, muscular hypotonia, and episodes of stupor after protein-rich meals. Twenty-nine Finnish patients (current median age 24.8 years, range 3.7-47.9 years) over a mean follow-up time of 18.1 years (range 1.2-27.2 years) had 57 fractures after minor trauma, mostly in childhood. Their 440 skeletal radiographs showed severe osteoporosis (13/29), controversially abnormal thickening of cortex of the metacarpals (7/29), or thin cortices of the long bones (5/29), endplate impression of vertebrae (8/29), rickets-like metaphyses (2/29), or early destruction of cartilage (3/29). Skeletal maturation was delayed by 1-5 years in 23 of 24 patients. There was no correlation between fracture incidence, radiological bone structure, and delayed skeletal maturation.
journal_name
Skeletal Radioljournal_title
Skeletal radiologyauthors
Svedström E,Parto K,Marttinen M,Virtama P,Simell Odoi
10.1007/BF00191519subject
Has Abstractpub_date
1993-01-01 00:00:00pages
11-6issue
1eissn
0364-2348issn
1432-2161journal_volume
22pub_type
杂志文章abstract::A case of Ollier's disease is demonstrated. Large osteolytic lesions in the left femur and hemipelvis visible on the roentgenograms represent enchondromas. On CT and MRI studies numerous fine translucencies are demonstrated in the surrounding bone, corresponding to multiple cartilaginous foci. These findings may be co...
journal_title:Skeletal radiology
pub_type: 杂志文章
doi:10.1007/BF00242138
更新日期:1992-01-01 00:00:00
abstract::We report on a case of a deforming arthropathy in a young man with a lysosomal storage disorder. A 31-year-old man with a known diagnosis of mannosidosis presented with a painful swollen right elbow. Radiographs of his right elbow showed a disorganised joint with multiple fragments resembling the appearances of a neur...
journal_title:Skeletal radiology
pub_type: 杂志文章
doi:10.1007/s002560000213
更新日期:2000-06-01 00:00:00
abstract::This 50-year-old woman presented with progressive pain in the left thigh. Radiographs showed a 9 x 6 cm soft-tissue mass located at the lateral border of the left femur. Magnetic resonance (MR) examination showed an eccentric, nonhomogeneous, soft-tissue mass abutting the femur. The preoperative differential diagnosis...
journal_title:Skeletal radiology
pub_type: 杂志文章
doi:10.1007/BF00190994
更新日期:1992-01-01 00:00:00
abstract::Extramedullary plasmacytomas are rare neoplasms arising from proliferations of monoclonal plasma cells. In primary form, these malignancies occur without other sites of plasma cell disease. Secondary extramedullary plasmacytomas occur in association with multiple myeloma and may be discovered during initial intramedul...
journal_title:Skeletal radiology
pub_type: 杂志文章
doi:10.1007/s00256-020-03514-9
更新日期:2020-12-01 00:00:00
abstract:OBJECTIVE:The purpose of this article was to evaluate the radiologic findings of adult pelvis and appendicular skeletal Langerhans cell histiocytosis (LCH), emphasizing the CT and MR findings. MATERIALS AND METHODS:The images of nine patients with pathologically proven LCH (five men and four women; mean age, 37.11 yea...
journal_title:Skeletal radiology
pub_type: 杂志文章
doi:10.1007/s00256-010-1078-y
更新日期:2011-11-01 00:00:00
abstract::Multiple hereditary osteochondromatosis is an uncommon autosomal dominant condition in which patients are predisposed to the development of chondrosarcoma. We report a case of a patient who developed a secondary low-grade chondrosarcoma in this setting. The tumor was associated with an unusual multinodular invasive gr...
journal_title:Skeletal radiology
pub_type: 杂志文章
doi:10.1007/s002560050560
更新日期:1999-09-01 00:00:00
abstract:OBJECTIVE:This study aims to quantitatively characterize the distribution of subchondral bone density across the human femoral head using a computed tomography derived measurement of bone density and a common reference coordinate system. MATERIALS AND METHODS:Femoral head surfaces were created bilaterally for 30 patie...
journal_title:Skeletal radiology
pub_type: 杂志文章
doi:10.1007/s00256-011-1270-8
更新日期:2012-06-01 00:00:00
abstract:OBJECTIVE:To describe the normal motion pattern at the midcarpal compartment during active radial-ulnar deviation of the wrist using dynamic MRI, and to determine the observer performance for measurements obtained in asymptomatic volunteers. METHODS:Dynamic MRI of 35 wrists in 19 asymptomatic volunteers (age mean 30.4...
journal_title:Skeletal radiology
pub_type: 杂志文章
doi:10.1007/s00256-019-03266-1
更新日期:2020-02-01 00:00:00
abstract::Fractures of the anterior process of the calcaneus are often missed. This error follows from the tendency to focus exclusively on the mortise and malleoli when a history of ankle trauma is supplied. Seven patients with this fracture are presented. The anatomy, mechanism of injury, clinical presentation, and the radiog...
journal_title:Skeletal radiology
pub_type: 杂志文章
doi:10.1007/BF00349747
更新日期:1985-01-01 00:00:00
abstract::Hip resurfacing arthroplasty is an increasingly common procedure for osteoarthritis. Conventional radiographs are used routinely for follow-up assessment, however they only provide limited information on the radiological outcome. Various complications have been reported in the scientific literature although not all ar...
journal_title:Skeletal radiology
pub_type: 杂志文章,评审
doi:10.1007/s00256-010-1081-3
更新日期:2011-07-01 00:00:00
abstract:OBJECTIVE:To analyse to what extent MR imaging based decisions were correct in determining the surgical procedure in patients with osteosarcoma of the distal femur. DESIGN:We compared the findings on MR imaging that determined the surgical strategy with the actual surgical findings or histopathological specimen. We as...
journal_title:Skeletal radiology
pub_type: 杂志文章
doi:10.1007/s002560050302
更新日期:1997-11-01 00:00:00
abstract::A patient is described with lower extremity mesomelic dwarfism associated with bilateral congenital elbow, hip, and knee dislocations. Rhomboid-shaped tibiae and delayed ossification of the primary fibular ossification centers were demonstrated at birth. Plain films and magnetic resonance imaging revealed that the tib...
journal_title:Skeletal radiology
pub_type: 杂志文章
doi:10.1007/BF00350660
更新日期:1989-01-01 00:00:00
abstract:OBJECTIVE:In diagnosing osteomyelitis (OM) both MRI and [18 F]FDG PET-CT proved to be accurate modalities. In anticipation of the advent of hybrid PET/MRI scanners we analyzed our patient group to give direction to future imaging strategies in patients with suspected OM. MATERIALS AND METHODS:In this retrospective stu...
journal_title:Skeletal radiology
pub_type: 杂志文章
doi:10.1007/s00256-014-1844-3
更新日期:2014-05-01 00:00:00
abstract::Four cases of osteoid osteomas are presented. An uncharacteristic magnetic resonance finding of soft tissue edema is reported. This observation should not be misinterpreted as indicating a more aggressive pathologic process and, thereby, exclude osteoid osteoma from the differential. ...
journal_title:Skeletal radiology
pub_type: 杂志文章
doi:10.1007/BF00191523
更新日期:1993-01-01 00:00:00
abstract:OBJECTIVE:MRI signal intensity of pediatric bone marrow can be difficult to interpret using conventional methods. Chemical shift imaging (CSI), which can quantitatively assess relative fat content, may improve the ability to accurately diagnose bone marrow abnormalities in children. METHODS:Consecutive pelvis and extr...
journal_title:Skeletal radiology
pub_type: 杂志文章
doi:10.1007/s00256-016-2403-x
更新日期:2016-09-01 00:00:00
abstract:OBJECTIVE:To compare polytomography (PT) and computed tomography (CT) for visualizing fractures and arthrodeses, with and without metal hardware, to determine whether CT could adequately replace PT. DESIGN AND PATIENTS:An ex vivo bovine model containing fractures in three planes, reduced with metal hardware, was creat...
journal_title:Skeletal radiology
pub_type: 杂志文章
doi:10.1007/s002560050526
更新日期:1999-06-01 00:00:00
abstract::Osteosarcoma is the commonest primary malignant bone tumour in children and adolescents, the majority of cases being conventional intra-medullary high-grade tumours affecting the appendicular skeleton. Treatment is typically with a combination of neo-adjuvant chemotherapy, tumour resection with limb reconstruction and...
journal_title:Skeletal radiology
pub_type: 杂志文章,评审
doi:10.1007/s00256-018-3079-1
更新日期:2019-04-01 00:00:00
abstract:OBJECTIVE:To demonstrate and determine the frequency and location of calcification within cadaveric knees with or without calcification typical of calcium pyrophosphate dihydrate (CPPD), utilizing histologic, radiographic and MR imaging techniques. DESIGN AND PATIENTS:Ten cadaveric knees of elderly individuals that de...
journal_title:Skeletal radiology
pub_type: 杂志文章
doi:10.1007/s00256-004-0767-9
更新日期:2004-07-01 00:00:00
abstract:OBJECTIVE:To define the possible mechanism of posture-dependent symptoms of spinal stenosis by measuring the effect of low back posture on morphologic changes of the intervertebral discs and spinal canal in healthy young people. DESIGN:Twenty healthy young volunteers underwent magnetic resonance imaging while supine w...
journal_title:Skeletal radiology
pub_type: 杂志文章
doi:10.1007/s002560050596
更新日期:2000-04-01 00:00:00
abstract::Sarcoidosis is a systemic disease that histologically typically shows non-caseating granulomas. The most common radiologic finding is hilar and mediastinal adenopathy. Patients with widely disseminated disease may show involvement of the peripheral appendicular skeleton in 1-13% of such cases. A primary skeletal prese...
journal_title:Skeletal radiology
pub_type: 杂志文章
doi:10.1007/s00256-008-0479-7
更新日期:2008-08-01 00:00:00
abstract:OBJECTIVE:To assess the impact of different types of physical activity types on longitudinal knee joint structural changes over 48 months in overweight and obese subjects. MATERIALS AND METHODS:We included 415 subjects with a BMI ≥ 25 kg/m2, Kellgren-Lawrence scores ≤ 3 at baseline and Whole-Organ Magnetic Resonance I...
journal_title:Skeletal radiology
pub_type: 杂志文章
doi:10.1007/s00256-020-03642-2
更新日期:2021-01-06 00:00:00
abstract::Mesenchymal chondrosarcoma of bone is a rare high-grade variant of chondrosarcoma, which typically has central intramedullary location. The tumor is characterized by admixture of highly anaplastic small round malignant cells and islands of mineralized low-grade hyaline cartilage. It is most unusual for this tumor to a...
journal_title:Skeletal radiology
pub_type: 杂志文章
doi:10.1007/s00256-017-2626-5
更新日期:2017-07-01 00:00:00
abstract:OBJECTIVE:To compare performances in diagnosing intertrochanteric hip fractures from proximal femoral radiographs between a convolutional neural network and orthopedic surgeons. MATERIALS AND METHODS:In total, 1773 patients were enrolled in this study. Hip plain radiographs from these patients were cropped to display ...
journal_title:Skeletal radiology
pub_type: 杂志文章
doi:10.1007/s00256-018-3016-3
更新日期:2019-02-01 00:00:00
abstract::Amyloidosis is a heterogeneous group of disorders characterized by extracellular deposition of unique protein fibrils. Amyloidosis may be hereditary or acquired, and the deposits may be focal, localized, or systemic in distribution. The least common presentation of an amyloid deposition is as a discrete mass called am...
journal_title:Skeletal radiology
pub_type: 杂志文章,评审
doi:10.1007/s00256-008-0621-6
更新日期:2009-03-01 00:00:00
abstract:OBJECTIVE:Pathological rotation of the leg is a well-known problem in paediatric orthopaedics. In this study the normal development of tibial torsion during growth was evaluated by computed tomography (CT). DESIGN:Seventy-eight normal individuals (52 children, 26 adults) aged 3-51 years were examined with CT. In axial...
journal_title:Skeletal radiology
pub_type: 杂志文章
doi:10.1007/s002560100388
更新日期:2001-09-01 00:00:00
abstract:OBJECTIVE:To establish a novel method of quantifying meniscal deformation using loaded MRI. More specifically, the goals were to evaluate the (1) accuracy, (2) inter-rater reliability, (3) intra-rater reliability, and (4) scan-rescan reliability. The secondary purpose of this experiment was to evaluate group difference...
journal_title:Skeletal radiology
pub_type: 杂志文章
doi:10.1007/s00256-014-2022-3
更新日期:2015-01-01 00:00:00
abstract::Metallosis is an uncommon complication following total knee arthroplasty that leads to osteolysis and implant loosening due to chronic inflammatory reaction. Abrasion between the metallic surfaces of the implant releases metallic debris that interacts with the periprosthetic soft tissues and causes chronic synovitis. ...
journal_title:Skeletal radiology
pub_type: 杂志文章
doi:10.1007/s00256-014-1860-3
更新日期:2014-08-01 00:00:00
abstract:OBJECTIVE:To report the application of dynamic contrast-enhanced subtraction MRI for detecting recurrences in aggressive or malignant soft tissue tumors. DESIGN:The imaging studies consisted of static (T1- and T2-weighted spin-echo) acquisitions, followed by dynamic conventional spin-echo short TR/TE images (at 45 s, ...
journal_title:Skeletal radiology
pub_type: 杂志文章
doi:10.1007/s002560050428
更新日期:1998-09-01 00:00:00
abstract::In part I, we reviewed the varied clinical presentations, pathogenesis, histologic findings, radiologic findings, and treatment of intramedullary cartilaginous lesions of bone. In this section, we will evaluate our cases and consultations of juxtacortical cartilaginous tumors. Radiographic differential diagnosis inclu...
journal_title:Skeletal radiology
pub_type: 杂志文章,评审
doi:10.1007/s002560050466
更新日期:1999-01-01 00:00:00
abstract:OBJECTIVE:To evaluate the evolution of the ultrasonographic pubo-femoral distance (PFD) before and after Pavlik harness treatment for developmental dysplasia of the hip (DDH) in newborns. PATIENTS AND METHODS:Twenty-five patients (16.7 ± 10.4 days; 19 females, six males) diagnosed with DDH and treated using the Pavlik...
journal_title:Skeletal radiology
pub_type: 杂志文章
doi:10.1007/s00256-017-2668-8
更新日期:2017-09-01 00:00:00