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1.
Orphanet J Rare Dis ; 19(1): 380, 2024 Oct 15.
Artículo en Inglés | MEDLINE | ID: mdl-39407304

RESUMEN

BACKGROUND: Legg-Calvé-Perthes disease (LCPD) commonly occurs among adolescents, threatening their health. However, the potential mechanism underlying LCPD remains unclear. miR-214-3p is shown as a critical role in LCPD development with unspecified upstream regulators. METHODS: Levels of miR-214-3p and circCDR1as in healthy controls and LCPD patients were determined by qRT-PCR. The role of circCDR1as/miR-214-3p axis in LCPD was determined by testing the cell viability and apoptosis in TC28 cells and primary chondrocytes. Regulation between circCDR1as and miR-214-3p was examined by RIP and ChIP assays. The inflammatory response and angiogenesis were evaluated by M2 macrophage polarization and HUVECs tumor formation. RESULTS: circCDR1as was overexpressed in LCPD patients with a negative correlation with miR-214-3p. Inhibition of circCDR1as alleviated the cell viability and apoptosis of DEX-treated chondrocytes, stimulated M2 macrophage polarization and angiogenesis. miR-214-3p was proved as a downstream effector to participate in circCDR1as mediated actions. circCDR1as recruited PRC2 complex to epigenetically suppress miR-214-3p. CONCLUSION: Our study illustrated the role and mechanism of circCDR1as in LCPD development by targeting miR-214-3p, highlighting its potential in the therapy for LCPD.


Asunto(s)
Enfermedad de Legg-Calve-Perthes , MicroARNs , ARN Circular , Humanos , MicroARNs/genética , MicroARNs/metabolismo , Enfermedad de Legg-Calve-Perthes/genética , Enfermedad de Legg-Calve-Perthes/metabolismo , Enfermedad de Legg-Calve-Perthes/patología , ARN Circular/genética , ARN Circular/metabolismo , Apoptosis/genética , ARN Largo no Codificante/genética , ARN Largo no Codificante/metabolismo , Femenino , Masculino
2.
J Orthop Surg Res ; 19(1): 560, 2024 Sep 11.
Artículo en Inglés | MEDLINE | ID: mdl-39261944

RESUMEN

BACKGROUND: Transient synovitis of the hip is the most common cause of limping in paediatric emergency departments. There is no consensus regarding routine follow-up after hip synovitis among children, and there are no standardized criteria for selecting cases that warrant follow-up due to persistent or recurring symptoms to rule out the possibility of Legg-Calvé-Perthes disease. Delayed treatment of Legg-Calvé-Perthes disease may increase the risk of developing early secondary coxarthrosis. Understanding the prevalence of Legg-Calvé-Perthes disease among paediatric patients with transient synovitis of the hip is of paramount importance and could empower both parents and paediatricians to make well-informed decisions when selecting follow-up care for children, thus ensuring that no cases of Legg-Calvé-Perthes disease are missed among diagnosis paediatric patients with transient synovitis of the hip. The aim of this review was to estimate the prevalence of Legg-Calvé-Perthes disease among paediatric patients with transient synovitis of the hip. METHODS: This study was conducted in strict accordance with the PRISMA guidelines and was registered with PROSPERO. The PubMed, Embase, and Cochrane Library databases were comprehensively searched up to July 2024 to identify relevant studies. The inclusion criteria were as follows: patients diagnosed with transient synovitis of the hip; patients aged up to 18 years; and studies with a minimum of 10 cases of paediatric transient synovitis of the hip. To pool the prevalence rates from individual studies, we utilized a random-effects meta-analysis. To assess the quality of the included studies in detail, we employed the Joanna Briggs Institute's quality assessment checklist. RESULTS: A total of 19 studies were ultimately included for the final analysis, with 2,617 paediatric cases of transient synovitis of the hip. The results of meta-analysis revealed that the pooled prevalence estimate of Legg-Calvé-Perthes disease among all paediatric patients with transient synovitis of the hip was 2.7% (95% CI 1.4-5.1). Significant heterogeneity was observed across the studies included in this analysis (I2 = 79.990%; P = 0.000). The pooled prevalence estimate of Legg-Calvé-Perthes disease among paediatric patients with recurrent or persistent transient synovitis of the hip was 36.3% (95% CI 21.6-54.2). Significant heterogeneity was also observed across the studies included in this analysis (I2 = 51.519%; P = 0.036). Furthermore, the follow-up period varied from 6 weeks to 24 months. The primary diagnostic imaging modality utilized for identifying Perthes disease was X-ray. CONCLUSION: Our study revealed that among paediatric patients with transient synovitis of the hip, routine X-ray follow-up of the hips after 6 weeks to rule out Legg-Calvé-Perthes disease is warranted only in patients who exhibit persistent or recurrent symptoms.


Asunto(s)
Enfermedad de Legg-Calve-Perthes , Sinovitis , Enfermedad de Legg-Calve-Perthes/epidemiología , Humanos , Prevalencia , Niño , Sinovitis/epidemiología , Preescolar , Femenino , Adolescente , Masculino , Articulación de la Cadera
3.
BMC Musculoskelet Disord ; 25(1): 753, 2024 Sep 20.
Artículo en Inglés | MEDLINE | ID: mdl-39304887

RESUMEN

PURPOSE: Legg Calve Perthes disease (LCPD) is a paediatric hip disorder caused by ischemia of the femoral epiphysis, causing femoral head deformity when untreated. This study aims to determine if previously validated pelvic obliquity radiographic parameters, used for assessing acetabular retroversion in developmental dysplasia of the hip, are applicable to patients with LCPD and its prognostic value. METHOD: A retrospective study of patients with Legg Calve Perthes disease was carried out, analysing 4 pelvic parameters: Ilioischial Angle, Obturator Index, Sharp's Angle and Acetabular Depth-Width Ratio (ADR). The differences between healthy and affected hips were studied, and subsequently, it was assessed whether these parameters have prognostic value in the disease outcome. RESULTS: Statistically significant differences have been obtained in the ilioischial angle, obturator index and ADR, between the affected and healthy hip. However, only the Acetabular Depth-Width Ratio showed predictive value for the disease outcome. CONCLUSION: Although this study revealed differences in pelvic parameters between healthy and diseased hips, with only the ADR showing statistical significance in the disease's evolution and prognosis, further studies with larger sample sizes are necessary.


Asunto(s)
Acetábulo , Enfermedad de Legg-Calve-Perthes , Enfermedad de Legg-Calve-Perthes/diagnóstico por imagen , Enfermedad de Legg-Calve-Perthes/epidemiología , Humanos , Estudios Retrospectivos , Acetábulo/diagnóstico por imagen , Masculino , Femenino , Niño , Radiografía , Preescolar , Pronóstico , Adolescente , Articulación de la Cadera/diagnóstico por imagen
4.
J Orthop Res ; 42(11): 2362-2372, 2024 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-39180284

RESUMEN

The understanding and treatment of prearthritic hip disease has evolved remarkably over the past 20 years. The principal investigator and the multicenter Academic Network of Conservational Hip Outcomes Research (ANCHOR) group have had a key role in improving the quality of care for these patients suffering from the three most common prearthritic conditions: femoroacetabular impingement, developmental dysplasia of the hip, and residual Legg-Calve-Perthes Disease. We aim to demonstrate that based on the six quality of care dimensions as defined by Donabedian, our 20-year academic journey has markedly improved the quality of care for young patients with prearthritic hip disease.


Asunto(s)
Pinzamiento Femoroacetabular , Humanos , Pinzamiento Femoroacetabular/terapia , Enfermedad de Legg-Calve-Perthes/terapia , Mejoramiento de la Calidad , Calidad de la Atención de Salud , Displasia del Desarrollo de la Cadera/terapia
5.
J Pediatr Orthop ; 44(10): 592-600, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-39187945

RESUMEN

INTRODUCTION: The wide-abduction A-frame brace contains the femoral head to improve its remodeling in Legg-Calvé-Perthes disease (LCPD). There is no study of the effect of brace adherence on hip outcomes. The purpose of this study was to determine if patient adherence to A-frame brace treatment is associated with improved hip abduction range of motion and radiographic outcomes in children with LCPD. METHODS: This retrospective study included patients aged 4 to 11 years with LCPD treated with an A-frame brace. Patients aged >11 and those treated with osteotomy before completing brace treatment were excluded. Built-in temperature sensors measured brace wear. Hip abduction was measured on examination before and after bracing. Deformity index (DI) and sphericity deviation score (SDS) were measured from radiographs at the 2-year follow-up and healed stage, respectively. Pearson correlation and multiple regression analyses were performed. RESULTS: Fifty-seven patients (44 male; 77%) were included with a mean age of 7.0±1.6 at brace treatment and mean adherence of 0.66±0.28. Brace adherence was associated with increased hip abduction ( R =0.36; P =0.006) and decreased DI ( R =-0.37; P =0.042) across all patients, and decreased SDS in patients <9 years old at the time of brace treatment ( R =-0.58; P =0.024). A +0.50 increase in adherence was associated with +9.4° hip abduction ( P =0.018), -0.13 DI ( P =0.027), and -17.7 SDS ( P =0.019). CONCLUSIONS: Adherence to A-frame brace treatment was associated with increased hip abduction, decreased femoral head deformity, and increased sphericity. Patients and parents can be counseled regarding brace adherence to maximize outcomes of treatment. LEVEL OF EVIDENCE: III-Therapeutic Study.


Asunto(s)
Tirantes , Enfermedad de Legg-Calve-Perthes , Cooperación del Paciente , Rango del Movimiento Articular , Humanos , Masculino , Estudios Retrospectivos , Femenino , Enfermedad de Legg-Calve-Perthes/terapia , Enfermedad de Legg-Calve-Perthes/diagnóstico por imagen , Preescolar , Niño , Cooperación del Paciente/estadística & datos numéricos , Resultado del Tratamiento , Radiografía , Articulación de la Cadera/diagnóstico por imagen , Articulación de la Cadera/fisiopatología , Estudios de Seguimiento
6.
Acta Orthop ; 95: 472-476, 2024 Aug 28.
Artículo en Inglés | MEDLINE | ID: mdl-39192775

RESUMEN

BACKGROUND AND PURPOSE:  Developmental dysplasia (DDH) and Legg-Calvé-Perthes disease (LCPD) are common indications for total hip arthroplasty (THA) at a young age, and may be associated with increased revision risk. We aimed to investigate the 10-year cumulative aseptic cup revision and overall revision risk of THA, and investigated whether these are increased compared with THA for primary osteoarthritis (OA) in patients below 55 years. METHODS:  All THAs (2007-2019) in patients under the age of 55 for the indications OA, DDH, and LCPD were extracted from the Dutch Arthroplasty register. The 10-year cumulative incidences of aseptic cup failure and overall revision were assessed for the 3 groups, with death as a competing risk. Cox regression analysis was used. RESULTS:  24,263 THAs were identified: 20,645 (85%) for OA, 3,032 (13%) for DDH, and 586 (2%) for LCPD. The 10-year cumulative revision risk for aseptic cup failure was 3.4% (95% confidence interval [CI] 3.0-3.8) for OA, 3.4% (CI 2.4-3.4) for DDH, and 1.7% (CI 0.2-3.1) for LCPD. The 10-year cumulative overall revision risk was 6.0% (CI 5.6-6.5) for OA, 6.0% (CI 4.9-7.2) for DDH, and 5.1% (2.7-7.5) for LCPD. The multivariable Cox regression analysis for aseptic cup failure yielded hazard ratios of 0.7 (0.5-1.2) for DDH, and 0.8 (0.3-2.1) for LCPD compared with OA. No statistically significant differences for overall revision were found. CONCLUSION:  THA performed for DDH or LCDP in patients under the age of 55 was not associated with a statistically significant increased risk of aseptic cup revision or overall revision, compared with THA performed for primary OA in the same age group.


Asunto(s)
Artroplastia de Reemplazo de Cadera , Enfermedad de Legg-Calve-Perthes , Osteoartritis de la Cadera , Falla de Prótesis , Sistema de Registros , Reoperación , Humanos , Artroplastia de Reemplazo de Cadera/efectos adversos , Artroplastia de Reemplazo de Cadera/estadística & datos numéricos , Reoperación/estadística & datos numéricos , Países Bajos/epidemiología , Masculino , Femenino , Persona de Mediana Edad , Osteoartritis de la Cadera/cirugía , Enfermedad de Legg-Calve-Perthes/cirugía , Enfermedad de Legg-Calve-Perthes/epidemiología , Displasia del Desarrollo de la Cadera/cirugía , Adolescente , Niño , Adulto , Factores de Riesgo
7.
Semin Musculoskelet Radiol ; 28(4): 447-461, 2024 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-39074727

RESUMEN

The pediatric hip undergoes significant changes from infancy through adolescence. Proper maturation is crucial for the development of a stable and functional hip joint. Imaging interpretation of the pediatric hip requires distinguishing normal variants and maturation patterns from pathology. We review femoral ossification centers, variants, and conditions that affect the proximal femur, such as Legg-Calvé-Perthes disease; the acetabulum, such as developmental hip dysplasia; the acetabular labrum, such as femoroacetabular impingement; and synovial pathology in children through adolescence. Understanding the spectrum of hip conditions and using advanced imaging techniques are essential for the accurate diagnosis and effective management of pediatric hip disorders.


Asunto(s)
Articulación de la Cadera , Enfermedad de Legg-Calve-Perthes , Humanos , Niño , Articulación de la Cadera/diagnóstico por imagen , Enfermedad de Legg-Calve-Perthes/diagnóstico por imagen , Adolescente , Preescolar , Lactante , Pinzamiento Femoroacetabular/diagnóstico por imagen , Artropatías/diagnóstico por imagen , Acetábulo/diagnóstico por imagen , Diagnóstico por Imagen/métodos
8.
Int J Paleopathol ; 46: 62-73, 2024 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-39079280

RESUMEN

OBJECTIVE: The first case of Legg-Calvé-Perthes disease (LCPD) in Greece is presented. LCPD, a rare disease, is discussed using the Digital Atlas of Ancient Rare Diseases (DAARD), which tests the benefits of the database for diagnosing and contextualizing the new case with 42 archaeological cases of LCPD recorded in the DAARD. MATERIALS: A 30-40-year-old, probable male individual was found at the archaeological site of Olympia, Greece, dating to 500-700 CE. METHODS: Biological sex, age-at-death and pathological changes were investigated using macroscopic and osteometric methods. The DAARD provided the typical characteristics of LCPD. RESULTS: Pathological changes in both hip joints without any other related changes in the skeleton corresponded to the skeletal features of LCPD. The DAARD produced 42 cases of LCPD, most of which from Europe, with a preference for male sex and unilateral involvement of the hip joint. CONCLUSIONS: The DAARD aids in diagnosing rare diseases and interpreting new cases in the context of already known studies. SIGNIFICANCE: This study shows that the DAARD has the potential to help researchers move beyond the level of single case studies and create a broader picture of the history of rare diseases. LIMITATIONS: This paper focuses on the benefits of the DAARD in relation to LCPD but not all rare diseases have been included in the database. SUGGESTIONS FOR FURTHER RESEARCH: More rare diseases from archaeological contexts should be added to the DAARD to create a base for the interpretation of their history and expand our understanding of rare diseases in the past.


Asunto(s)
Enfermedad de Legg-Calve-Perthes , Enfermedades Raras , Humanos , Masculino , Enfermedad de Legg-Calve-Perthes/patología , Enfermedad de Legg-Calve-Perthes/historia , Adulto , Enfermedades Raras/patología , Enfermedades Raras/historia , Historia Antigua , Grecia , Paleopatología , Femenino , Atlas como Asunto/historia
9.
Open Vet J ; 14(5): 1172-1181, 2024 May.
Artículo en Inglés | MEDLINE | ID: mdl-38938425

RESUMEN

Background: Canine Legg Calvé Perthes disease (LCPD) occurs during the growth period, and the cause of ischemic necrosis of the femoral head during growth remains unclear. If LCPD-affected femoral head-derived mesenchymal stem cells (LCPD-MSCs) can be generated, they can be used as a new tool for the pathophysiological analysis of canine LCPD. Aim: To generate affected femoral head-derived mesenchymal stem cells (MSCs) from dogs with LCPD and investigate the mRNA expression levels of angiogenesis-related factors and osteogenic differentiation potency of LCPD-MSCs. Methods: This study was performed using affected femoral heads from dogs diagnosed with LCPD and underwent femoral head and neck ostectomy. The necrotic tissue was harvested from the LCPD-affected femoral head and cultured statically (LCPD group, n = 6). Canine bone marrow-derived MSCs (BM-MSCs) were used as controls (control group, n = 6). First, the morphology of the cultured cells was observed, and the expression of CD29, CD34, CD44, CD45, CD90, and major histocompatibility complex class II was analyzed using flow cytometry. Additionally, the trilineage differentiation potency of the LCPD-affected head-derived adherent cells was examined. Furthermore, the expression levels of HIF1A, VEGFA, VEGFB, and PDGFB mRNAs and the bone differentiation potency of LCPD-affected head-derived adherent cells were investigated. Results: LCPD-affected femoral head-derived adherent cells showed a fibroblast-like morphology, and the expression of cell surface antigens was similar to that of BM-MSCs. In addition, LCPD-affected femoral head-derived adherent cells showed the same trilineage differentiation potency as BM-MSCs and were consistent with MSC characteristics. Furthermore, the mRNA expression levels of angiogenesis-related factors could be objectively measured in LCPD-MSCs and those MSCs had bone differentiation potency. Conclusion: In the present study, canine LCPD-MSCs were successfully generated, suggesting their usefulness as a tool for pathological analysis of LCPD in dogs.


Asunto(s)
Enfermedades de los Perros , Cabeza Femoral , Enfermedad de Legg-Calve-Perthes , Células Madre Mesenquimatosas , Animales , Perros , Enfermedad de Legg-Calve-Perthes/veterinaria , Enfermedad de Legg-Calve-Perthes/patología , Células Madre Mesenquimatosas/fisiología , Enfermedades de los Perros/patología , Cabeza Femoral/patología , Diferenciación Celular , Osteogénesis , Masculino , Células Cultivadas , Femenino
10.
J Pediatr Orthop ; 44(8): 468-475, 2024 Sep 01.
Artículo en Inglés | MEDLINE | ID: mdl-38835293

RESUMEN

BACKGROUND: While physical activity (PA) is important during youth, it is unclear if children and adolescents with developmental dysplasia of the hip (DDH), Legg-Calvé-Perthes disease (LCPD), or slipped capital femoral epiphysis (SCFE) are expected or encouraged to return to PA once they have healed. This study examines the orthopaedic surgeons' role in advising pediatric hip patients on the PA they should engage in, assessing their practice, opinions, and consensus when making recommendations. METHODS: Orthopaedic surgeons were invited from member lists of 4 hip study groups. The survey included demographics, opinions regarding PA, and 10 case scenarios that queried respondents on the duration and intensity of PA as well as the restrictions on activity type that they would recommend for DDH, LCPD, or SCFE patients. Consensus was evaluated on a scale ranging from 0 to 1, with a value of 0 indicating no agreement among respondents and a value of 1 indicating complete agreement. RESULTS: A total of 51 orthopaedic surgeons responded. While 94% agreed that it is important for school-aged hip patients to return to PA after they have healed, 53% believed that PA may compromise the hip and contribute to the development of osteoarthritis. Average standardized consensus was 0.92 for suggesting the patient engage in some amount of PA, 0.44 for suggesting the recommended daily minimum of 60 minutes of moderate-to-vigorous physical activity (MVPA), and 0.33 for suggesting restrictions on activity type. The most frequently selected restrictions included avoiding impact activities (93%, 235/254) and contact activities (58%, 147/254), followed by weight-bearing activities (24%, 62/254). Respondents were not aware of existing PA guidelines, although 57% expressed interest in following guidelines and 39% were uncertain. CONCLUSIONS: While there is consensus among orthopaedic surgeons that children with chronic hip conditions should engage in PA, there is considerable variation when recommending the recommended daily MVPA minimum and placing restrictions on activity type. This study suggests interest among orthopaedic surgeons in developing PA guidelines that optimize outcomes for pediatric hip patients. LEVEL OF EVIDENCE: Level II-Survey study.


Asunto(s)
Ejercicio Físico , Enfermedad de Legg-Calve-Perthes , Cirujanos Ortopédicos , Epífisis Desprendida de Cabeza Femoral , Humanos , Niño , Adolescente , Femenino , Epífisis Desprendida de Cabeza Femoral/cirugía , Masculino , Displasia del Desarrollo de la Cadera/cirugía , Encuestas y Cuestionarios , Consenso , Pautas de la Práctica en Medicina/estadística & datos numéricos
11.
Acta Orthop ; 95: 333-339, 2024 06 18.
Artículo en Inglés | MEDLINE | ID: mdl-38887211

RESUMEN

BACKGROUND AND PURPOSE: It is controversial as to which patients affected by Legg-Calvé-Perthes disease (LCPD) benefit from containment surgery. This population-based study based on data from a national quality registry aims to assess the incidence of LCPD and to explore which factors affect the decision for surgical intervention. METHODS: This observational study involved 309 patients with unilateral LCPD reported between 2015 and 2023 to the Swedish Pediatric Orthopedic Quality Register (SPOQ). Descriptive statistics and logistic regression models were used for analysis. RESULTS: In 2019, the assessed incidence of LCPD in the Swedish population of 2-12-year-olds was 4.2 per 105. 238 (77%) were boys with a mean age of 6 years. At diagnosis, 55 (30%) were overweight or obese, rising to 17 patients (39%) and 16 patients (40%) at 2-year follow-up for surgically and non-surgically treated groups, respectively. At diagnosis, affected hips had reduced abduction compared with healthy hips, and their abduction remained restricted at the 2-year follow-up. Surgically treated patients had inferior abduction compared with non-surgically treated ones at diagnosis. The adjusted risk for containment surgery increased with age and in the presence of a positive Trendelenburg sign but decreased with greater hip abduction. CONCLUSION: We found a lower national yearly incidence (4.2 per 105) than previously reported in Swedish studies. A higher proportion of overweight or obese patients compared with the general Swedish population of 4-9-year-olds was identified. Increasing age, positive Trendelenburg sign, and limited hip abduction at diagnosis correlated with increased surgical intervention likelihood.


Asunto(s)
Enfermedad de Legg-Calve-Perthes , Sistema de Registros , Humanos , Enfermedad de Legg-Calve-Perthes/cirugía , Enfermedad de Legg-Calve-Perthes/epidemiología , Suecia/epidemiología , Masculino , Niño , Femenino , Preescolar , Incidencia , Estudios de Cohortes , Procedimientos Ortopédicos/estadística & datos numéricos , Procedimientos Ortopédicos/métodos , Factores de Riesgo
12.
J Orthop Res ; 42(10): 2277-2286, 2024 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-38796746

RESUMEN

Legg-Calvé-Perthes disease (LCPD) is a childhood hip disorder characterized by ischemic injury to the epiphysis of the femoral head, but changes to the metaphysis have also been implicated in its pathogenesis. Quantitative magnetic resonance imaging (MRI) relaxation time mapping techniques are potentially useful to detect injury in LCPD, but studies to date have focused on the epiphysis. The purpose of this study was to assess whether T2, T1ρ, adiabatic T1ρ, and adiabatic T2ρ relaxation times can detect early metaphyseal changes in an LCPD piglet model. Complete epiphyseal ischemia of one femoral head was surgically induced and confirmed using contrast-enhanced MRI in n = 10 6-week-old piglets; the contralateral side was unoperated. The bilateral hips were imaged 1 week after surgery in vivo at 3T MRI using relaxation time mapping and contrast-enhanced MRI. Relaxation times and thicknesses of the metaphyseal primary and secondary spongiosa were measured and compared between the ischemic and contralateral-control femoral heads using paired t-tests. In the ischemic femoral heads, T2 relaxation times were significantly increased in the primary spongiosa (6.7 ± 9.8 ms, p = 0.029), and T2, T1ρ, adiabatic T1ρ, and adiabatic T2ρ relaxation times were significantly decreased in the secondary spongiosa (respectively: -13.3 ± 9.3 ms, p = 0.013; -32 ± 23 ms, p < 0.001; -43 ± 41 ms, p = 0.009; and -39 ± 13 ms, p < 0.001). The secondary spongiosa thickness was also significantly decreased in the ischemic femoral heads (p < 0.001). In conclusion, T2, T1ρ, adiabatic T1ρ, and adiabatic T2ρ relaxation time mapping techniques can detect early changes in the metaphysis following ischemic injury to the epiphysis of the femoral head in a piglet model of LCPD.


Asunto(s)
Modelos Animales de Enfermedad , Cabeza Femoral , Enfermedad de Legg-Calve-Perthes , Imagen por Resonancia Magnética , Animales , Enfermedad de Legg-Calve-Perthes/diagnóstico por imagen , Imagen por Resonancia Magnética/métodos , Porcinos , Cabeza Femoral/diagnóstico por imagen , Cabeza Femoral/irrigación sanguínea , Epífisis/diagnóstico por imagen
13.
DNA Cell Biol ; 43(6): 288-297, 2024 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-38648538

RESUMEN

Legg-Calve-Perthes disease (LCPD) is an idiopathic avascular necrosis of the pediatric femoral head. Bone remodeling and bone structural genes have the potential to contribute to the progression of LCPD when there is disequilibrium between bone resorption and bone formation. A case-control study was performed to search for associations of several common polymorphisms in the genes Receptor Activator for Nuclear Factor κappa B (RANK), Receptor Activator for Nuclear Factor κappa B Ligand (RANKL), osteoprotegerin (OPG), interleukin (IL)-6, and type 1 collagen (COL1A1) with LCPD susceptibility in Mexican children. A total of 23 children with LCPD and 46 healthy controls were genotyped for seven polymorphisms (rs3018362, rs12585014, rs2073618, rs1800795, rs1800796, rs1800012, and rs2586498) in the RANK, RANKL, OPG, IL-6, and COL1A1 genes by real-time polymerase chain reaction with TaqMan probes. The variant allele (C) of IL-6 rs1800795 was associated with increased risk of LCPD (odds ratio [OR]: 3.8, 95% confidence interval [CI]: [1.08-13.54], p = 0.033), adjusting data by body mass index (BMI) and coagulation factor V (FV), the association with increased risk remained (OR: 4.9, 95% CI: [1.14-21.04], p = 0.025). The OPG polymorphism rs2073618, specifically GC-GG carriers, was associated with a more than fourfold increased risk of developing LCPD (OR: 4.34, 95% CI: [1.04-18.12], p = 0.033) when data were adjusted by BMI-FV. There was no significant association between RANK rs3018362, RANKL rs12585014, IL-6 rs1800796, COL1A1 rs1800012, and rs2586498 polymorphisms and LCPD in a sample of Mexican children. The rs1800975 and rs2037618 polymorphisms in the IL-6 and OPG genes, respectively, are informative markers of increased risk of LCPD in Mexican children.


Asunto(s)
Remodelación Ósea , Predisposición Genética a la Enfermedad , Interleucina-6 , Enfermedad de Legg-Calve-Perthes , Osteoprotegerina , Niño , Preescolar , Femenino , Humanos , Masculino , Remodelación Ósea/genética , Estudios de Casos y Controles , Colágeno Tipo I/genética , Cadena alfa 1 del Colágeno Tipo I/genética , Interleucina-6/genética , Enfermedad de Legg-Calve-Perthes/genética , México , Osteoprotegerina/genética , Polimorfismo de Nucleótido Simple , Ligando RANK/genética , Receptor Activador del Factor Nuclear kappa-B/genética
14.
J Pediatr Orthop ; 44(7): e618-e624, 2024 Aug 01.
Artículo en Inglés | MEDLINE | ID: mdl-38659309

RESUMEN

INTRODUCTION: One of the most popular containment procedures for Legg-Calvé-Perthes disease (LCPD) is proximal femur varus osteotomy (PFO). While generally successful in achieving containment, PFO can cause limb length discrepancy, abductor weakness, and (of most concern for families) a persistent limp. While many studies have focused on radiographic outcomes following containment surgery, none have analyzed predictors of this persistent limp. The aim of this study was to determine clinical, radiographic, and surgical risk factors for persistent limp 2 years after PFO in children with LCPD. METHODS: A retrospective review of a prospectively collected multicenter database was conducted for patients aged 6 to 11 years at disease onset with unilateral early-stage LCPD (Waldenström I) who underwent PFO. Limp status (no, mild, and severe), age, BMI, and pain scores were obtained at initial presentation, 3-month, and 2-year postoperative visits. Preoperative and follow-up radiographs were used to measure traditional morphologic hip metrics including acetabular index (AI), lateral center-edge angle (LCEA), and femoral neck-shaft angle (NSA). Univariate analysis as well as multivariate logistic regression models were used to analyze factors associated with mild and severe limp at the 2-year visit. RESULTS: A total of 95 patients met the inclusion criteria, and of these 50 patients underwent concomitant greater trochanter apophysiodesis (GTA) at the time of PFO. At the 2-year visit, there were 38 patients (40%) with a mild or severe limp. Multivariate logistic regression revealed no significant radiographic factors associated with a persistent limp. However, lower 2-year BMI and undergoing GTA were associated with decreased rates of persistent limp regardless of age ( P <0.05). When stratifying by age of disease onset, apophysiodesis appeared to be protective against any severity of limp in patients aged 6 to 8 years old ( P = 0.03), but not in patients 8 years or older ( P = 0.49). CONCLUSIONS: Persistent limp following PFO is a frustrating problem that was seen in 40% of patients at 2 years. However, lower follow-up BMI and performing a greater trochanter apophysiodesis, particularly in patients younger than 8 years of age, correlated with a lower risk of postoperative limp.


Asunto(s)
Enfermedad de Legg-Calve-Perthes , Osteotomía , Humanos , Enfermedad de Legg-Calve-Perthes/cirugía , Enfermedad de Legg-Calve-Perthes/diagnóstico por imagen , Osteotomía/métodos , Osteotomía/efectos adversos , Niño , Masculino , Femenino , Estudios Retrospectivos , Factores de Riesgo , Fémur/cirugía , Fémur/diagnóstico por imagen , Complicaciones Posoperatorias/etiología , Estudios de Seguimiento
15.
Bone Joint J ; 106-B(5 Supple B): 40-46, 2024 May 01.
Artículo en Inglés | MEDLINE | ID: mdl-38688483

RESUMEN

Aims: Ganz's studies made it possible to address joint deformities on both the femoral and acetabular side brought about by Perthes' disease. Femoral head reduction osteotomy (FHRO) was developed to improve joint congruency, along with periacetabular osteotomy (PAO), which may enhance coverage and containment. The purpose of this study is to show the clinical and morphological outcomes of the technique and the use of an implemented planning approach. Methods: From September 2015 to December 2021, 13 FHROs were performed on 11 patients for Perthes' disease in two centres. Of these, 11 hips had an associated PAO. A specific CT- and MRI-based protocol for virtual simulation of the corrections was developed. Outcomes were assessed with radiological parameters (sphericity index, extrusion index, integrity of the Shenton's line, lateral centre-edge angle (LCEA), Tönnis angle), and clinical parameters (range of motion, visual analogue scale (VAS) for pain, Merle d'Aubigné-Postel score, modified Harris Hip Score (mHHS), and EuroQol five-dimension five-level health questionnaire (EQ-5D-5L)). Early and late complications were reported. Results: The mean follow-up was 39.7 months (standard deviation (SD) 26.4). The mean age at surgery was 11.4 years (SD 1.6). No major complications were recorded. One patient required a total hip arthroplasty. Mean femoral head sphericity increased from 46.8% (SD 9.34%) to 70.2% (SD 15.44; p < 0.001); mean LCEA from 19.2° (SD 9.03°) to 44° (SD 10.27°; p < 0.001); mean extrusion index from 37.8 (SD 8.70) to 7.5 (SD 9.28; p < 0.001); and mean Tönnis angle from 16.5° (SD 12.35°) to 4.8° (SD 4.05°; p = 0.100). The mean VAS improved from 3.55 (SD 3.05) to 1.22 (1.72; p = 0.06); mean Merle d'Aubigné-Postel score from 14.55 (SD 1.74) to 16 (SD 1.6; p = 0.01); and mean mHHS from 60.6 (SD 18.06) to 81 (SD 6.63; p = 0.021). The EQ-5D-5L also showed significant improvements. Conclusion: FHRO associated with periacetabular procedures is a safe technique that showed improved functional, clinical, and morphological outcomes in Perthes' disease. The newly introduced simulation and planning algorithm may help to further refine the technique.


Asunto(s)
Cabeza Femoral , Enfermedad de Legg-Calve-Perthes , Osteotomía , Adolescente , Niño , Femenino , Humanos , Masculino , Acetábulo/cirugía , Acetábulo/diagnóstico por imagen , Cabeza Femoral/diagnóstico por imagen , Cabeza Femoral/cirugía , Enfermedad de Legg-Calve-Perthes/cirugía , Enfermedad de Legg-Calve-Perthes/diagnóstico por imagen , Imagen por Resonancia Magnética , Osteotomía/métodos , Rango del Movimiento Articular , Estudios Retrospectivos , Tomografía Computarizada por Rayos X , Resultado del Tratamiento
16.
J Pediatr Orthop ; 44(7): e612-e617, 2024 Aug 01.
Artículo en Inglés | MEDLINE | ID: mdl-38651529

RESUMEN

AIMS: Legg-Calve-Perthes disease (LCPD) is a diagnosis of exclusion. Various conditions, such as skeletal dysplasias, can closely mimic LCPD and these must be ruled out to provide appropriate treatment, prognosis, and counseling. Traditionally, genetic testing has not been readily available in pediatric orthopaedic practice. Furthermore, the clinical value of genetic testing patients with LCPD is unclear. With the advance of next-generation sequencing (NGS) technology, genetic testing has become clinically available as a lab test. The purposes of this study were to assess the clinical utility of genetic testing in select patients with LCPD and to determine the patient characteristics of those who tested positive for skeletal dysplasia. METHODS: This is an IRB-approved, retrospective study of 63 consecutive patients who presented with Perthes-like symptoms and/or x-ray findings and who had genetic testing. The reason(s) for genetic testing included bilateral hip disease, family history of LCPD, short stature, suspected skeletal dysplasia, atypical radiographic findings, and/or combinations of these reasons. RESULTS: Of the 63 patients, 19 patients (30%) were found to have a pathogenic gene variant. In 8 of the 19, a variety of skeletal dysplasia was diagnosed. The remaining 11 patients were found to be carriers of autosomal recessive disorders. All 19 patients were referred for genetic counseling. Of the 8 patients found to have skeletal dysplasia, 3 had bilateral disease, 3 were <10 percentile in height, 1 had a family history of "LCPD," and 3 had atypical x-ray findings. In addition to the pathogenic variants, numerous genetic variants of unknown significance were found with 2 gene variants showing exactly the same variant found in 2 unrelated patients. CONCLUSIONS: With 30% of the patients showing pathogenic results, genetic testing of select patients with Perthes-like disease is valuable in detecting an underlying genetic disorder or a carrier status of a genetic disorder.


Asunto(s)
Pruebas Genéticas , Enfermedad de Legg-Calve-Perthes , Humanos , Enfermedad de Legg-Calve-Perthes/genética , Estudios Retrospectivos , Masculino , Femenino , Niño , Pruebas Genéticas/métodos , Preescolar , Adolescente , Lactante , Secuenciación de Nucleótidos de Alto Rendimiento , Variación Genética , Diagnóstico Diferencial
17.
JBJS Case Connect ; 14(2)2024 Apr 01.
Artículo en Inglés | MEDLINE | ID: mdl-38635784

RESUMEN

CASE: We describe the unique case of a 20-year-old man with a history of Legg-Calve-Perthes disease, hip dysplasia, and osteochondral fragmentation of the medial femoral head. We performed arthroscopic femoroplasty and femoral head allografting, followed by a valgus-producing derotational femoral osteotomy (DFO) and periacetabular osteotomy (PAO). At 1-year follow-up, the patient achieved osseous union and complete femoral head healing with return to his active hobbies. CONCLUSION: We describe the successful utilization of arthroscopic allografting for medial femoral head osteochondral fragmentation. To our knowledge, this is the first report on femoral head arthroscopic allografting before DFO and PAO.


Asunto(s)
Cabeza Femoral , Enfermedad de Legg-Calve-Perthes , Masculino , Humanos , Adulto Joven , Adulto , Cabeza Femoral/diagnóstico por imagen , Cabeza Femoral/cirugía , Enfermedad de Legg-Calve-Perthes/diagnóstico por imagen , Enfermedad de Legg-Calve-Perthes/cirugía , Enfermedad de Legg-Calve-Perthes/complicaciones , Osteotomía , Fémur/cirugía , Progresión de la Enfermedad , Aloinjertos
18.
Bone Joint J ; 106-B(5): 501-507, 2024 May 01.
Artículo en Inglés | MEDLINE | ID: mdl-38688522

RESUMEN

Aims: The aim of this study was to produce clinical consensus recommendations about the non-surgical treatment of children with Perthes' disease. The recommendations are intended to support clinical practice in a condition for which there is no robust evidence to guide optimal care. Methods: A two-round, modified Delphi study was conducted online. An advisory group of children's orthopaedic specialists consisting of physiotherapists, surgeons, and clinical nurse specialists designed a survey. In the first round, participants also had the opportunity to suggest new statements. The survey included statements related to 'Exercises', 'Physical activity', 'Education/information sharing', 'Input from other services', and 'Monitoring assessments'. The survey was shared with clinicians who regularly treat children with Perthes' disease in the UK using clinically relevant specialist groups and social media. A predetermined threshold of ≥ 75% for consensus was used for recommendation, with a threshold of between 70% and 75% being considered as 'points to consider'. Results: A total of 40 participants took part in the first round, of whom 31 completed the second round. A total of 87 statements were generated by the advisory group and included in the first round, at the end of which 31 achieved consensus and were removed from the survey, and an additional four statements were generated. A total of 60 statements were included in the second round and 45 achieved the threshold for consensus from both rounds, with three achieving the threshold for 'points to consider'. The recommendations predominantly included self-management, particularly relating to advice about exercise and education for children with Perthes' disease and their families. Conclusion: Children's orthopaedic specialists have reached consensus on recommendations for non-surgical treatment in Perthes' disease. These statements will support decisions made in clinical practice and act as a foundation to support clinicians in the absence of robust evidence. The dissemination of these findings and the best way of delivering this care needs careful consideration, which we will continue to explore.


Asunto(s)
Consenso , Técnica Delphi , Enfermedad de Legg-Calve-Perthes , Humanos , Enfermedad de Legg-Calve-Perthes/terapia , Niño , Reino Unido , Terapia por Ejercicio/métodos , Guías de Práctica Clínica como Asunto
19.
Gait Posture ; 110: 65-70, 2024 05.
Artículo en Inglés | MEDLINE | ID: mdl-38518557

RESUMEN

BACKGROUND: Trendelenburg gait describes contralateral pelvic drop during single leg stance (SLS) with occasional lateral trunk lean compensation over the stance limb. However, quantitative research on 'uncompensated Trendelenburg' gait (pelvic drop independent of lateral trunk lean) remains sparse among populations that commonly utilize this gait pattern, such as adolescent hip pathology patients. RESEARCH QUESTION: How prevalent is uncompensated Trendelenburg among various adolescent hip pathologies and how is it related to hip load, hip abduction strength, and self-reported hip pain? METHODS: Gait, strength, and pain data were collected among 152 pre-operative patients clinically diagnosed with acetabular hip dysplasia, femoroacetabular impingement, Legg-Calvé-Perthes, or slipped capital femoral epiphysis (SCFE). Patients with ≥ 5.4° of dynamic pelvic drop in SLS were divided into a 'pelvic drop' group and screened to exclude those with excessive ipsilateral trunk lean. They were then compared to the 'stable pelvis' patients using a Mann-Whitney test. RESULTS: Dysplasia patients represented the highest proportion of the pelvic drop group (46%). The pelvic drop group showed a significant increase in self-reported hip pain (p = 0.011), maximum hip abductor moment (p = 0.002), and peak coronal power absorption at the affected hip during SLS loading response, (p < 0.001) while showing no difference in abduction strength (p = 0.381). SIGNIFICANCE: Uncompensated Trendelenburg gait may lead to increased loading of the affected hip in adolescent hip pathology patients. Disadvantageous hip biomechanics can create increased abductor muscle demand among these pathological populations, with dysplasia patients showing the highest prevalence. Maximal abduction strength did not correlate with pelvic drop. Future work should aim to identify and quantify causal factors. Increased coronal hip power absorption during weight acceptance warrants clinical attention, as there may be a detrimental, over-reliance on passive hip structures to support load among a population that that is already predisposed to hip osteoarthritis.


Asunto(s)
Pinzamiento Femoroacetabular , Marcha , Humanos , Adolescente , Femenino , Masculino , Marcha/fisiología , Pinzamiento Femoroacetabular/fisiopatología , Enfermedad de Legg-Calve-Perthes/fisiopatología , Epífisis Desprendida de Cabeza Femoral/fisiopatología , Luxación de la Cadera/fisiopatología , Fuerza Muscular/fisiología , Articulación de la Cadera/fisiopatología , Fenómenos Biomecánicos , Niño
20.
Curr Sports Med Rep ; 23(2): 45-52, 2024 Feb 01.
Artículo en Inglés | MEDLINE | ID: mdl-38315432

RESUMEN

ABSTRACT: Legg-Calvé-Perthes disease (LCPD), or idiopathic avascular necrosis of the proximal capital femoral epiphysis in children, has a variable presentation and can result in significant femoral head deformity that can lead to long-term functional deficits. Plain radiographic imaging is crucial in diagnosing LCPD and guiding treatment. Although the etiology of LCPD remains unknown, the evolution of the disease has been well characterized to include the phases of ischemia, revascularization, and reossification. The mechanical weakening during these phases of healing place the femoral head at high risk of deformity. Treatment of LCPD, therefore, focuses on minimizing deformity through operative and nonoperative strategies to reduce the risk of premature osteoarthritis. Advanced imaging using perfusion MRI may refine surgical decision making in the future, and biological treatments to improve femoral head healing are on the horizon.


Asunto(s)
Enfermedad de Legg-Calve-Perthes , Niño , Humanos , Enfermedad de Legg-Calve-Perthes/terapia , Enfermedad de Legg-Calve-Perthes/diagnóstico por imagen , Cabeza Femoral/diagnóstico por imagen , Radiografía , Imagen por Resonancia Magnética , Toma de Decisiones
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