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  • 11
    Electronic Resource
    Electronic Resource
    Springer
    Archives of orthopaedic and trauma surgery 116 (1997), S. 500-503 
    ISSN: 1434-3916
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract There is a hypothesis that hyperplastic callus (HC) in osteogenesis imperfecta (OI) is not merely a rare complication but could actually be inherited, although this idea has not yet been investigated. We described two cases, a mother and son, with mild 01, normal scleral colour and no dentinogenesis imperfecta, who repeatedly had HC in their femur. Familial occurrence of HC was found in 13 cases in 5 families among 21 cases in 7 families with a familial background of OI in the literature (including this report). This is higher than the reported incidence of HC, 1.5% (5 cases of 333), and the mode of transmission is concomitant with autosomal dominant inheritance in all these families. Since a review of 47 cases in the literature shows that HC occurs independently of scleral colour and the degree of bone fragility, it may be an additional criterion for subdivision within each type of the Sillence classification.
    Type of Medium: Electronic Resource
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  • 12
    Electronic Resource
    Electronic Resource
    Springer
    Archives of orthopaedic and trauma surgery 117 (1998), S. 464-467 
    ISSN: 1434-3916
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We retrospectively reviewed nine tibial lengthenings in seven achondroplastic patients. The callotasis method was used, and a unilateral type lengthener, either the Dynamic Axial Fixator (Orthofix, Italy; eight legs) or the High Functional Fixator (Matsumoto Co., Japan; one leg), was applied. The distracted length averaged 14.6 (range 10–18) cm. The minimum diameter of the callus was measured using a ruler on anteroposterior and lateral radiographs. The callus diameter ratio (%) was calculated as the callus diameter divided by the original diaphysis diameter. For periods during axial loading and after removal of the fixator in each patient, a single regression line was drawn on the callus diameter ratio data using the least squares method, and the diameter change rate (%/day) was evaluated by inclination of this line. The diameter change rates during axial loading were negative in six legs, but those after fixator removal were positive in all legs, and the latter were significantly greater than the former. The diameter change rates after fixator removal on the anteroposterior radiographs were negatively correlated with the callus diameter ratio at the time of fixator removal (r = 0.84, P = 0.0008). Simple axial loading may not be a sufficient mechanial environment for restoration of the physiological shape, and it is important to be aware that we cannot expect the callus diameter to increase by this means alone.
    Type of Medium: Electronic Resource
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  • 13
    Electronic Resource
    Electronic Resource
    Springer
    Archives of orthopaedic and trauma surgery 117 (1998), S. 468-470 
    ISSN: 1434-3916
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Spondyloepiphyseal dysplasia congenita (SEDC) can be accompanied by hypoplasia of the odontoid and/or lax ligaments which may lead to spinal cord compression. Since an early diagnosis of myelopathy is essential for rational treatment, identification of the high-risk factors is imperative. Sixteen patients were retrospectively reviewed, 10 male and 6 female, and their average age at the time of examination was 18 (range 3–37) years. Myelopathy was seen in 6 individuals. Five were associated with atlantoaxial subluxation, and the critical value of space available for the spinal cord at the level of the atlas was 10 mm or less. Their height was less than –7SD of average Japanese of the same age, and all were associated with severe coxa vara. Although SEDC itself is a risk factor, marked short stature of less than –7SD and severe coxa vara are particularly high-risk factors for cord compression at the level of the atlas.
    Type of Medium: Electronic Resource
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  • 14
    Electronic Resource
    Electronic Resource
    Springer
    Archives of orthopaedic and trauma surgery 119 (1999), S. 100-102 
    ISSN: 1434-3916
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report on a patient with chronic idiopathic thrombocytopenic purpura (ITP) who went into remission after femoral lengthening. Although it is possible that spontaneous remission (frequency 5%–10%) of chronic ITP coincided with the femoral lengthening, limb lengthening could also have caused the thrombocytosis. This case suggests a close relationship between osteogenesis and hematopoiesis during regenerate bone formation. Limb lengthening can therefore be defined as the formation not only of bone and soft tissue but also of hematopoietic tissue.
    Type of Medium: Electronic Resource
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  • 15
    Electronic Resource
    Electronic Resource
    Springer
    Archives of orthopaedic and trauma surgery 119 (1999), S. 470-473 
    ISSN: 1434-3916
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The cardinal feature of Maffucci syndrome is the coexistence of enchondromatosis and vascular anomalies. The patients are usually normal at birth, the disease becoming evident at any time up to puberty. Association of soft-tissue swelling has been described in this syndrome but has not been listed as a sign or symptom. We report three cases in which the initial sign was diffuse swelling of the dorsum of the hand and/or foot. This was noticed at birth in cases 1 (girl) and 3 (girl) and at the age of 1 month in case 2 (boy), who otherwise appeared normal except for case 3 who had a rectovestibular fistula. The pathology of the swollen soft tissue was adipose tissue associated with various degrees of thickened and fibrotic septum containing dilated lymphatic channels. These dilated channels within the septum were so subtle that they were initially overlooked in cases 1 and 2. Awareness that swelling of the dorsum of the hand or foot can be the sole sign and symptom of Maffucci syndrome before the typical indications become evident helps the physician establish an early diagnosis of this condition.
    Type of Medium: Electronic Resource
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  • 16
    Electronic Resource
    Electronic Resource
    Springer
    Archives of orthopaedic and trauma surgery 117 (1998), S. 262-264 
    ISSN: 1434-3916
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We investigated whether the callus formation in the humerus during the distraction period of limb lengthening proceeds at a higher rate than that in the femur and tibia. Ten achondroplastic patients underwent 3 bilateral humerus, 3 bilateral femur and 4 bilateral tibia lengthenings. To reduce the confounding effect of bone size, we used bone mineral apparent density (BMAD) to compare the three groups; this is a volumetric bone mineral density measurement. BMAD in the distracted callus space was evaluated at 8 weeks after the start of distraction using dual-energy X-ray absorptiometry (mean ± SD; g/cm3): in the humerus (0.24 ± 0.08) it was significantly higher than in the tibia (0.10 ± 0.02), while there was no difference between the humerus and femur (0.35 ± 0.11). We conclude that the callus formation in the humerus during the distraction period of limb lengthening proceeded at a significantly faster rate than in the tibia, but there was no significant difference between the humerus and femur.
    Type of Medium: Electronic Resource
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  • 17
    ISSN: 1432-0827
    Keywords: Key words: Fibroblast growth factor — Bone lengthening — Bone formation.
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine , Physics
    Notes: Abstract. Bone lengthening by callotasis is one of the most useful methods not only for the treatment of short extremities but also for extensive bone defects; however, the procedure takes a long time especially for the consolidation of the distracted callus. In this study, effects of a single local injection of recombinant human fibroblast growth factor-2 (FGF-2 or basic FGF) on callotasis bone lengthening were examined in rabbits. Ten days after the osteotomy at the middle of the tibia and the installment of an external fixator, the osteotomized site was distracted at a rate of 1.4 mm/day for 7 days, resulting in 9.8 mm lengthening. On the final day of distraction, 200 μg of FGF-2 in 150 μl of saline solution or vehicle alone was injected into the center of the distracted callus. Injection of FGF-2 increased bone formation at the distracted callus radiologically and histologically. A significant effect on bone mineral content (BMC) at the callus was observed as early as 2 weeks, and FGF-2 increased the BMC about twofold at 5 weeks after a normal remodeling process. We conclude that the callotasis method in combination with FGF-2 injection at the consolidation step could be clinically beneficial to shorten the bone lengthening period.
    Type of Medium: Electronic Resource
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  • 18
    Electronic Resource
    Electronic Resource
    Springer
    Archives of gynecology and obstetrics 264 (2000), S. 49-50 
    ISSN: 1432-0711
    Keywords: Key words Microperforate hymen ; Hysterofiberoscopy ; Withdrawal bleeding
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Microperforate hymen is a congenital obstructive vaginal membrane with a tiny opening. It is usually detected long before menarche because of recurring vulvovaginitis and urinary tract infection. We report a case of microperforate hymen in a 27-year-old women presenting oligomenorrhea, in whom the location of the tiny opening on the hymenal membrane was detected by withdrawal bleeding without anesthesia, and definitively diagnosed preoperatively by using hysterofiberscopy.
    Type of Medium: Electronic Resource
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  • 19
    Electronic Resource
    Electronic Resource
    Springer
    International orthopaedics 19 (1995), S. 282-284 
    ISSN: 1432-5195
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Résumé Nous avons entrepris des recherches sur la relation entre l'ostéoporose de la colonne vertébrale et l'arthrose du genou au moyen de l'absorptiométrie à rayons X et de la radiographie standar sur 82 femmes sélectionnées au hasard (l'âge moyen est de 77 ans). La densité minérale de l'os (BMD=»Bone Mineral Density«) de la colonne lombaire a été évaluée par l'absorptiométrie. Nous avons évalué les informations provenant des radiographies des genoux pour classer l'arthrose en 2 groupes: »Normal« et »Evolue«. La BMD du groupe type »Evolue« était bien supérieure à celle du groupe type »Normal« (p〈0.01).
    Notes: Summary We investigated the relation between osteoporosis of the spine and osteoarthritis of the knee using dual energy X-ray absorptiometry of the lumbar spine to measure bone mineral density and radiographs of the knee in 82 randomly selected females (mean age 77.5 years). Radiographs of the knee were divided into a normal and severe group. The bone mineral density of the severe group was significantly more than that of the normal group.
    Type of Medium: Electronic Resource
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  • 20
    Electronic Resource
    Electronic Resource
    Springer
    International orthopaedics 20 (1997), S. 359-362 
    ISSN: 1432-5195
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Résumé. Nous avons examiné après traitement 28 allongements tibiaux chez 14 patients souffrant d’achondroplasie et ayant un âge moyen de 15 ans (étalement: de 9 à 24 ans) afin de comparer l’efficacité de la physiothérapie et de l’orthèse dans la prévention de l’équin. Une physiothérapie à raison de 15 minutes par jour à domicile (n = 12) n’a pas été efficace, même dans le cas de patients marchant et participant à des activités sociales telles que l’école ou le travail. La déformation en équin est apparue avec un allongement de 30% et a atteint une moyenne de 30 degrés avec un allongement de 50%. En revanche, l’orthèse avec un port d’au moins 16 heures par jour (n = 10) s’est révélée efficace dans la prévention de la déformation jusqu’à un allongement d’au moins 50%, la différence avec la physiothérapie étant significative (p 〈0.01) au-delà d’un allongement de 30%. Lorsque nous évaluons les deux méthodes de physiothérapie et de traitement par orthèse en fonction du mode d’étirement des tissus mous, l’effet de la physiothéraphie est intermittent et en général d’une durée insuffisante alors que celui de l’orthèse est continu et peut avoir une durée appropriée si l’appareil est porté assez longtemps. Il semble essentiel pour l’allongement des parties molles d’adopter une méthode leur appliquant un étirement continu et prolongé.
    Notes: Summary. We have reviewed 28 tibial lengthenings in 14 patients with achondroplasia at an average age of 15 years, and compared the effectiveness of physiotherapy and an orthosis in preventing an equinus deformity. Physiotherapy of 15 min a day was ineffective even when the patients were able to walk. An orthosis worn for 16 h a day prevented equinus deformity up to at least 50% of lengthening, and the difference from physiotherapy was significant at more than 30% of lengthening.
    Type of Medium: Electronic Resource
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