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1.
J Foot Ankle Surg ; 58(6): 1215-1222, 2019 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-31679675

RESUMEN

Hallux valgus is a prevalent condition. Many open surgical methods of correction have been described. We performed a percutaneous, extra-articular distal metatarsal osteotomy for mild to moderate bunion deformity. The minimally invasive technique was used in 217 feet (180 patients; age 49 ± 4.7 years, mean ± standard deviation) at 4 centers in North America (Center 1: November 2012 to March 2017; Center 2: January 2010 to May 2016; Center 3: October 2013 to June 2016; Center 4: January 2015 to June 2017). The procedure was used in simultaneous bilateral cases in 28 patients (15.6%) and in nonsimultaneous bilateral cases in 9 patients (5.0%). Immediate postoperative weightbearing was used in all cases. The mean preoperative intermetatarsal angle, hallux abductus angle, and tibial sesamoid position were 14.6° ± 3.5°, 30.7° ± 7.8°, and 5.4 ± 1.4, respectively. At final follow-up of 9.3 ± 6.1 months, the mean intermetatarsal angle, hallux abductus angle, and tibial sesamoid position were 4.7° ± 2.8°, 8.4° ± 6.1°, and 2.0 ± 1.0, respectively (p < .0001 for all comparisons). No major complications were noted. All 217 osteotomies achieved union; 3 feet (1.4%) in 3 patients (1.7%) experienced asymptomatic malunion. Superficial pin-site infection was seen in 42 (19.4%) of the 217 feet (39 patients, 21.7%). The radiographic results of this percutaneous technique appear to be reproducible across multiple centers, and the technique is useful when correcting intermetatarsal and hallux abductus angles. This percutaneous osteotomy for realignment of the first ray allows immediate postoperative weightbearing and, in this initial review, appears to be safe and effective, even in simultaneous bilateral cases.


Asunto(s)
Juanete/cirugía , Hallux Valgus/cirugía , Huesos Metatarsianos/diagnóstico por imagen , Huesos Metatarsianos/cirugía , Osteotomía/métodos , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Juanete/diagnóstico por imagen , Estudios de Cohortes , Femenino , Hallux Valgus/diagnóstico por imagen , Humanos , Masculino , Persona de Mediana Edad , Radiografía , Estudios Retrospectivos , Adulto Joven
2.
J Pediatr Orthop ; 37(7): 479-483, 2017.
Artículo en Inglés | MEDLINE | ID: mdl-26371943

RESUMEN

BACKGROUND: Osteogenesis imperfecta (OI) is a heritable skeletal disorder characterized by bone fragility and short stature that is usually due to mutations in 1 of the 2 genes that code for collagen type I α-chains. The association between hip dysplasia and OI has not been systematically investigated. In this single-center study, we retrospectively reviewed all cases of OI associated with hip dysplasia to describe clinical characteristics and the effect of therapy. METHODS: We reviewed the charts of 687 patients with OI who were seen at the Shriners Hospital for Children in Montreal between 1999 and 2013 to identify patients with a diagnosis of hip dysplasia. Clinical characteristics and the course after therapeutic interventions were extracted from the charts. RESULTS: Hip dysplasia was diagnosed in 8 hips of 5 patients (4 boys, 1 girl; age at diagnosis ranged between 3 wk and 27 mo old). The prevalence of hip dysplasia and OI was therefore 0.87% (per patient). In 4 of the 5 patients (80%), OI was caused by mutations affecting the C-propeptide of collagen type I, which is otherwise rare in OI. Among the 26 patients with C-propeptide mutations followed at our institution, 4 (15%) had hip dysplasia. Pavlik harness treatment was attempted in 2 patients (3 hips) but was not effective in either case and resulted in avascular necrosis of 1 hip. Femoral varus derotational shortening osteotomies using a telescopic rod were performed in all 8 hips along with a closed reduction in 4 hips and an open reduction in 4 hips. Concomitant pelvic osteotomies were performed in 2 hips (1 patient). Surgery resulted in redislocation of 1 hip; all other surgically treated hips remained reduced. CONCLUSIONS: Clinical screening for hip dysplasia is difficult in OI owing to the bowing of the proximal femur and the risk of causing fractures. OI patients with positive C-propeptide mutation should therefore be screened for hip dysplasia by use of ultrasound. Presence of a C-propeptide mutation appears to be a risk factor for hip dysplasia (80%). It appears that Pavlik harness treatment is not useful in children with OI. The usual treatment of children with OI who pull to stand or started walking with femoral deformity is femoral osteotomy and rodding. In case of associated hip dysplasia with a dislocation, open reduction of the hip and a possible concomitant pelvic osteotomy appears to be a valid management option. LEVEL OF EVIDENCE: Level IV.


Asunto(s)
Colágeno Tipo I/genética , Luxación Congénita de la Cadera/genética , Mutación , Osteogénesis Imperfecta/genética , Preescolar , Femenino , Fémur/diagnóstico por imagen , Fémur/fisiopatología , Fémur/cirugía , Luxación Congénita de la Cadera/complicaciones , Luxación Congénita de la Cadera/diagnóstico , Humanos , Lactante , Recién Nacido , Masculino , Osteogénesis Imperfecta/complicaciones , Osteotomía/efectos adversos , Osteotomía/métodos , Radiografía , Estudios Retrospectivos , Factores de Riesgo , Ultrasonografía
3.
Hum Mol Genet ; 23(5): 1250-9, 2014 Mar 01.
Artículo en Inglés | MEDLINE | ID: mdl-24163128

RESUMEN

There is emerging evidence for reduced muscle function in children with neurofibromatosis type 1 (NF1). We have examined three murine models featuring NF1 deficiency in muscle to study the effect on muscle function as well as any underlying pathophysiology. The Nf1(+/-) mouse exhibited no differences in overall weight, lean tissue mass, fiber size, muscle weakness as measured by grip strength or muscle atrophy-recovery with limb disuse, although this model lacks many other characteristic features of the human disease. Next, muscle-specific knockout mice (Nf1muscle(-/-)) were generated and they exhibited a failure to thrive leading to neonatal lethality. Intramyocellular lipid accumulations were observed by electron microscopy and Oil Red O staining. More mature muscle specimens lacking Nf1 expression taken from the limb-specific Nf1Prx1(-/-) conditional knockout line showed a 10-fold increase in muscle triglyceride content. Enzyme assays revealed a significant increase in the activities of oxidative metabolism enzymes in the Nf1Prx1(-/-) mice. Western analyses showed increases in the expression of fatty acid synthase and the hormone leptin, as well as decreased expression of a number of fatty acid transporters in this mouse line. These data support the hypothesis that NF1 is essential for normal muscle function and survival and are the first to suggest a direct link between NF1 and mitochondrial fatty acid metabolism.


Asunto(s)
Desarrollo de Músculos/genética , Músculos/metabolismo , Neurofibromatosis 1/genética , Neurofibromatosis 1/metabolismo , Neurofibromina 1/genética , Neurofibromina 1/metabolismo , Animales , Peso Corporal , Resorción Ósea/genética , Huesos/metabolismo , Huesos/patología , Modelos Animales de Enfermedad , Genes Letales , Heterocigoto , Homocigoto , Humanos , Ratones , Ratones Noqueados , Fibras Musculares Esqueléticas/metabolismo , Fibras Musculares Esqueléticas/patología , Fibras Musculares Esqueléticas/ultraestructura , Fuerza Muscular/genética , Músculos/patología , Músculos/ultraestructura , Neurofibromina 1/deficiencia , Tamaño de los Órganos
4.
Clin Orthop Relat Res ; 467(4): 985-91, 2009 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-18946710

RESUMEN

UNLABELLED: The treatment and prognosis of labral tears of the hip depend primarily on whether there is concomitant injury of the adjacent acetabular articular cartilage. We asked whether a delamination cyst on the preoperative plain radiographs correlated with delamination of the acetabular articular cartilage at the time of hip arthroscopy. We reviewed the preoperative radiographs of 125 consecutive hips that had a labral tear at hip arthroscopy for the presence of a delamination cyst. A delamination cyst was defined as an acetabular subchondral cyst either directly adjacent to a lateral acetabular cyst or in relation to a subchondral crack in the anterosuperior portion of the acetabulum. All patients with acetabular cartilage delamination at arthroscopy were identified. There were 16 patients with delamination cysts on radiographs and 15 patients with cartilage delamination at arthroscopy. A delamination cyst on the preoperative anteroposterior and/or frog lateral radiographs of the hip accurately predicted acetabular cartilage delamination, especially in hips with labral tears not caused by a major trauma. A delamination cyst is a previously unrecognized and novel radiographic sign that can preoperatively identify acetabular cartilage delamination in patients with labral tears, thereby facilitating the selection of the appropriate surgery and determining prognosis. LEVEL OF EVIDENCE: Level II, diagnostic study. See the Guidelines for Authors for a complete description of levels of evidence.


Asunto(s)
Acetábulo/patología , Cartílago Articular/patología , Quistes/patología , Lesiones de la Cadera/patología , Ligamentos Articulares/patología , Acetábulo/diagnóstico por imagen , Acetábulo/lesiones , Adolescente , Adulto , Anciano , Artrografía , Artroscopía , Cartílago Articular/diagnóstico por imagen , Cartílago Articular/lesiones , Quistes/diagnóstico por imagen , Femenino , Humanos , Laceraciones , Ligamentos Articulares/diagnóstico por imagen , Ligamentos Articulares/lesiones , Masculino , Persona de Mediana Edad , Valor Predictivo de las Pruebas , Adulto Joven
5.
Case Rep Orthop ; 2014: 982171, 2014.
Artículo en Inglés | MEDLINE | ID: mdl-25580335

RESUMEN

Osteomyelitis of the pubis symphysis is a rare condition. There have been various reports in the literature of inflammation and osteomyelitis as well as septic arthritis of pubic symphysis. However, due to the fact that these conditions are rare and that the usual presenting symptoms are very nonspecific, osteomyelitis of the pubic symphysis is often misdiagnosed, thus delaying definitive treatment. We present a case that to our knowledge is the first case in literature of osteomyelitis of the pubic symphysis in a 17-year-old boy with juvenile idiopathic arthritis (JIA), which was initially misdiagnosed and progressed to bilateral adductor abscesses. A high suspicion of such condition should be considered in a JIA patient who presents with symphysis or thigh pain.

6.
PLoS One ; 8(2): e56790, 2013.
Artículo en Inglés | MEDLINE | ID: mdl-23457615

RESUMEN

Bone morphogenetic proteins (BMPs) are recognized for their ability to induce bone formation in vivo and in vitro. Their osteogenic and osteoinductive properties are tightly regulated by the secretion of specific BMP antagonists, which have been shown to physically bind and sometimes be blocked by the extracellular proteoglycan heparan sulphate side chains (from hereon referred to as HS). The purpose of this study was to investigate if local application of 5 µg of HS proteoglycan to a bone regenerate site in a mouse model of distraction osteogenesis (DO) can accelerate bone healing and affect the expression of key members of the BMP signaling pathway. DO was performed on the right tibia of 115 adult male wild-type mice. At mid-distraction (day 11), half the group was injected locally with 5 µg of HS, while the other half was injected with saline. The mice were sacrificed at 2 time-points: mid-consolidation (34 days) and full consolidation (51 days). The distracted tibial zone was then collected for analysis by µCT, radiology, biomechanical testing, immunohistochemistry, and histology. While µCT data showed no statistically significant difference in bone formation, the results of biomechanical testing in stiffness and ultimate force were significantly lower in the HS-injected bones at 51 days, compared to controls. Immunohistochemistry results also suggested a decrease in expression of several key members of the BMP signaling pathway at 34 days. Furthermore, wound dehiscence and infection rates were significantly elevated in the HS group compared to the controls, which resulted in a higher rate of euthanasia in the treatment group. Our findings demonstrate that exogenous application of 5 µg of HS in the distracted gap of a murine model had a negative impact on bone and wound healing.


Asunto(s)
Heparitina Sulfato/farmacología , Osteogénesis por Distracción , Osteogénesis/efectos de los fármacos , Animales , Fenómenos Biomecánicos , Proteínas Morfogenéticas Óseas/metabolismo , Regulación hacia Abajo/efectos de los fármacos , Masculino , Ratones , Ratones Endogámicos C57BL , Complicaciones Posoperatorias , Transducción de Señal/efectos de los fármacos , Tibia/citología , Tibia/efectos de los fármacos , Tibia/crecimiento & desarrollo , Tibia/cirugía , Microtomografía por Rayos X
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