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1.
J Pediatr ; 234: 123-127, 2021 07.
Artículo en Inglés | MEDLINE | ID: mdl-33465347

RESUMEN

OBJECTIVE: To provide information on evolution over time of leg length discrepancy in patients with syndromic and isolated lateralized overgrowth. STUDY DESIGN: This retrospective study investigates leg length discrepancy longitudinally in 105 patients with lateralized overgrowth either isolated (n = 37) or associated with Beckwith-Wiedemann spectrum (n = 56) or PIK3CA-related overgrowth spectrum (n = 12). Discrepancy was measured by standard methods and categorized as minor, mild, severe, and critical, based on the thresholds of 1, 2 and 5, respectively. RESULTS: The period of observation from diagnosis was 1.7 ± 2.6 to 9.0 ± 6.0 years. Leg length discrepancy was 11.0 ± 7.2 mm at diagnosis and 17.1 ± 14.4 mm at last visit. Both final leg length discrepancy and change over time were correlated with discrepancy at diagnosis (r2 = 0.45, P < .001 and r2 = 0.05, P = .019, respectively). Among minor leg length discrepancy at diagnosis, 47.5% remained minor, 40.0% become mild, and 12.5% severe. Among patients with discrepancy classified as severe at diagnosis, 84.6% remained severe and 15.4% evolved to critical. The isolated lateralized overgrowth group showed a milder evolution over time compared with Beckwith-Wiedemann spectrum and PIK3CA-related overgrowth spectrum groups. Among patients with Beckwith-Wiedemann, those with paternal chromosome 11 uniparental disomy had more severe leg length discrepancy at diagnosis and evolution over time. CONCLUSIONS: Leg length discrepancy associated with isolated or syndromic lateralized overgrowth tends to worsen with growth and correlates with discrepancy at first observation. Among the genotypic groups, isolated lateralized overgrowth tends to have a milder evolution, whereas Beckwith-Wiedemann spectrum predisposes to a more severe outcome, especially if associated with paternal chromosome 11 uniparental disomy genotype.


Asunto(s)
Síndrome de Beckwith-Wiedemann , Pierna , Genotipo , Humanos , Estudios Retrospectivos , Disomía Uniparental
2.
Arthroscopy ; 31(12): 2335-41, 2015 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-26321111

RESUMEN

PURPOSE: To evaluate if the degree of chondral fragmentation affected extracellular matrix (ECM) production in cartilage fragment autograft implantation in vitro. METHODS: Cartilage was taken from 5 donors undergoing total hip replacement (mean age, 65.6 years; standard deviation [SD], 3). The cartilage was minced to obtain 4 groups with different fragment sizes: (1) "fish scale" (diameter, 8 mm; thickness, 0.3 mm), (2) cubes with 2-mm sides, (3) cubes with 1-mm sides, and (4) cartilage paste (< 0.3 mm). The cultures were maintained in chondrogenic medium for 6 weeks. Biochemically, a proteoglycan (PG):DNA ratio was calculated as the best approximation of ECM production per cell. The ratio between PG released in the culture medium and the PG in the neocartilage (PGrel:PG) was used as a matrix stability index. Histologically, the slides were stained with safranin O fast green and collagen type II immunostaining. The titration of safranin O-positive cells and the Bern score were calculated. RESULTS: Regarding the PG:DNA ratio, group 4 performed significantly better than groups 1 (P = .001) and 3 (P = .02), whereas group 2 performed better than group 1 (P = .03). No significant difference was found regarding the PGrel:PG ratio and safranin O-positive cells. Regarding the Bern score, group 4 performed significantly better than groups 1 (P = .02), 2 (P = .04), and 3 (P = .03). CONCLUSIONS: We conclude that human cartilage fragmentation significantly affects ECM production in vitro. Increased fragmentation enhances ECM production. CLINICAL RELEVANCE: Assuming a similar behavior in vivo, we recommend mincing the cartilage into small pieces when performing the cartilage fragment autograft implantation technique in order to increase ECM production. Further in vitro studies investigating cartilage of younger nonarthritic donors, as well as in vivo studies, are needed.


Asunto(s)
Cartílago/lesiones , Cartílago/trasplante , Matriz Extracelular/metabolismo , Anciano , Animales , Artroplastia de Reemplazo de Cadera , Autoinjertos , ADN/metabolismo , Humanos , Proteoglicanos/metabolismo
3.
Curr Rev Musculoskelet Med ; 7(4): 292-301, 2014 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-25129702

RESUMEN

High tibial osteotomy (HTO) is a widely accepted and performed procedure to treat medial knee arthrosis. The aim of this review is to evaluate the different surgical options in medial knee arthrosis, focusing on indications, patient's selection, long-term follow-up and survival analysis of HTO. Comparison and pooling of results are challenging because of different evaluation systems, small cohort number, and different surgical techniques. No differences have been described between opening and closing wedged HTO in terms of outcomes. Excellent early survivorship and good clinical outcomes were reported also with concomitant procedures. Correct indications, preoperative workup/planning, and technique selection are essential in achieving good results. The choice between opening and closing wedge osteotomy, graft selection in opening wedge HTO, comparison between HTO and unicompartmental knee arthroplasty, and the results of revised HTO to total knee replacement are currently under debate and will be discussed in the present review.

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