What Abductor Repair Technique Provides the Best Functional Outcomes After Proximal Femur Endoprosthetic Reconstruction for Oncologic Indications? A Systematic Review.

BACKGROUND: There is conflicting data regarding the optimal abductor mechanism (AM) repair technique after resection of proximal femur tumors. We sought to compare functional outcomes following tumor resection and reconstruction with proximal femoral replacement based on the AM repair technique utilized. METHODS: We conducted a systematic review following the Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines. We established two study groups based on AM repair technique as follows: soft-tissue reattachment (STr) and greater trochanter preservation (GTp). In the STr group, the gluteus medius and minimus muscles were reattached to the endoprosthesis, whereas in the GTp group, the greater trochanter and gluteal tendons were preserved. The STr group was further subdivided into direct and indirect reattachments. Weighted means adjusting for sample size were calculated. RESULTS: A total of 658 patients from 12 articles were included. Patients who had STr displayed higher Musculoskeletal Tumor Society scores (75 versus 67.3%, P < 0.001), lower rates of Trendelenburg gait (33.9 versus 52.4%, P < 0.01), and ambulation with assistive devices (30.4 versus 54.9%, P < 0.001) compared to the GTp group. Within the STr group, indirect reattachment was associated with higher Musculoskeletal Tumor Society scores (87.2 versus 70.1%, P < 0.001) and lower rates of Trendelenburg gait (3.8 versus 36.3%, P < 0.001) and ambulation with assistive devices (0 versus 42.4%, P < 0.001) compared to direct reattachment. The reattachment hardware failure rate in GTp was 15%. CONCLUSION: A STr provided superior functional outcomes compared to GTp in tumor-related proximal femoral replacement. From a functional outcome perspective, the use of indirect STr was better compared to direct STr. LEVEL OF EVIDENCE: III.

Hiperostosis cortical infantil / Infantile cortical hyperostosis

RESUMEN La hiperostosis cortical infantil (HIC) o enfermedad de Caffey es un trastorno óseo autolimitado que se caracteriza por fiebre, irritabilidad, inflamación de los tejidos blandos y engrosamiento cortical de uno o más huesos. Es más común en el primer semestre de vida. Se informa el caso de un lactante de 3 meses con las características clínico-radiográficas propias de la enfermedad y los hallazgos de laboratorio más comunes que permitieron un acertado diagnóstico y seguir una conducta adecuada. El cuadro agudo se mantuvo durante pocos días y se observó resolución total del edema en cuatro semanas. El reconocimiento de esta rara infección evitará adoptar métodos diagnósticos y terapéuticos invasivos para el paciente.

ABSTRACT Infantile cortical hyperostosis (ICH) or Caffey disease is a self-limiting bone disorder characterized by fever, irritability, soft tissue swelling and cortical bone thickening. It is more common in infants < 6 months of age. We report the case of a 3-month-old infant with the clinical and radiographic characteristics of the disease and the most common laboratory findings which enabled an accurate diagnosis and appropriate management. The acute episode persisted for several days, followed by a complete resolution of the edema within four weeks. Identifying this rare infection will help avoid invasive diagnostic and therapeutic strategies.

Thyroid dysgenesis associated with dwarfism, osteoporosis and spontaneous fractures in a goat.

An 11-month-old female Saanen goat, weighing 12.7 kg, was taken to the Veterinary Hospital of the Federal University of Minas Gerais because of sternal recumbency. On clinical examination, the animal was much smaller than expected and had hair similar to that of puppies and areas of hyperpigmentation on the head and dorsocervical and dorsothoracic cranial regions. Radiographic examination revealed fractures in both femurs and severe generalized osteoporosis. Given the unfavourable prognosis, the animal was euthanized. Necropsy revealed generalized pallor, muscular atrophy of the pelvic limbs and little reserve of subcutaneous adipose tissue. Both femurs had complete and closed diaphyseal fractures. The second lumbar vertebra was severely reduced in length as a result of a fracture, with dorsal displacement of the vertebral body towards the vertebral canal and compression of the spinal cord. Long bones and vertebrae had severe cortical thinning, enlargement of the medullary canal and reduced resistance. The thyroid gland was not in its normal anatomical location. A pale red nodule (1.0 × 0.4 cm) in the serosa of the middle third of the trachea, close to the thoracic entrance, was confirmed as ectopic thyroid tissue. Microscopically, the bones had evidence of growth arrest and severe osteoporosis. The ectopic thyroid nodule was hyperplastic with severe hypertrophy of follicular cells. The spinal cord was compressed by vertebral fractures and had focally extensive and severe myelomalacia. Based on the pathological features, the case was diagnosed as thyroid dysgenesis characterized by eutopic thyroid agenesis and ectopic thyroid tissue, associated with interruption of bone growth with dwarfism, osteoporosis and spontaneous secondary fractures with compression of the lumbar spinal cord.

Enfermedad de Caffey después del primer año de vida: caso clínico / Caffey disease after the first year of life: clinical case

Objetivo: La enfermedad de Caffey o hiperostosis cor- tical infantil es una enfermedad rara que afecta uno o más huesos en los primeros meses de vida y debido a su baja inci- dencia está subdiagnosticada, y por tanto se aplican procedi- mientos invasivos innecesarios en su estudio y tratamiento. Se presenta un caso clínico atípico de enfermedad de Caffey en una paciente mayor de 1 año de edad y su resolución. Caso clínico: El servicio de Cirugía Maxilofacial del Hospital Provincial Pediátrico Eduardo Agramonte Piña de Camagüey, Cuba, atiende a una niña de 1 año y 10 meses que se encontraba hospitalizada por presentar una inflamación alarmante en la región facial y cervical precedida de un cua- dro febril y dificultad para alimentarse. Se indicaron los estu- dios apropiados, cuyos resultados, junto a las características clínicas, permitieron diagnosticar la enfermedad de Caffey. Aunque sea una enfermedad rara, es importante estudiarla para realizar un correcto análisis de cada caso y diferenciarla de otras enfermedades que requieren de conductas terapéuti- cas agresivas (AU)

Aim: Caffey's disease or infantile cortical hyperostosis is a rare disease that affects one or more bones in the first months of life and due to its low incidence, it is underdiag- nosed, and therefore unnecessary invasive procedures are applied in its study and treatment. An atypical clinical case of Caffey's disease in a patient older than 1 year and its reso- lution is presented. Case report: The Maxillofacial Surgery service of the Eduardo Agramonte Piña Provincial Pediatric Hospital in Camagüey, Cuba, takes the case of a 1 year and 10-month-old female patient who was hospitalized for an alarming inflam- mation in the facial and cervical region, preceded by a fever and difficulty to eat. The appropriate studies were indicated, which results, together with the clinical characteristics, al- lowed the diagnosis of Caffey's disease. Although it is a rare entity, it is important to study it to carry out a correct analysis of each case and differentiate it from other diseases that re- quire aggressive therapeutic behaviors (AU)

Long-term effects of hypercalcemia in kidney transplant recipients with persistent hyperparathyroidism.

BACKGROUND: Hypercalcemia is highly prevalent in kidney transplant recipients with hyperparathyroidism. However, its long-term impact on graft function is uncertain. METHODS: We conducted a prospective cohort study investigating adverse graft outcomes associated with persistent hypercalcemia (free calcium > 5.2 mg/dL in ≥ 80% of measures) and inappropriately elevated intact parathyroid hormone (> 30 pg/mL) in kidney transplant recipients. Asymptomatic mild hypercalcemia was monitored unless complications developed. RESULTS: We included 385 kidney transplant recipients. During a 4-year (range 1-9) median follow-up time, 62% of kidney transplant recipients presented persistent hypercalcemia. Compared to kidney transplant recipients without hypercalcemia, there were no significant differences in graft dysfunction (10% vs. 12%, p = 0.61), symptomatic urolithiasis (5% vs. 3%, p = 0.43), biopsy-proven calcium deposits (6% vs. 5%, p = 1.0), fractures (6% vs. 4%, p = 0.64), and a composite outcome of urolithiasis, calcium deposits, fractures, and parathyroidectomy indication (16% vs. 13%, p = 0.55). In a subset of 76 kidney transplant recipients, subjects with persistent hypercalcemia had higher urinary calcium (median 84 [43-170] vs. 38 [24-64] mg/day, p = 0.03) and intact fibroblast growth factor 23 (median 36 [24-54] vs. 27 [19-40] pg/mL, p = 0.04), and lower 25-hydroxyvitamin D levels (11.3 ± 1.2 vs. 16.3 ± 1.4 ng/mL, p < 0.001). In multivariate analysis, pretransplant intact parathyroid hormone < 300 pg/mL was associated with a reduced risk of post-transplant hypercalcemia (OR 0.51, 95% CI 0.32-0.80). CONCLUSIONS: Long-term persistent mild hypercalcemia (tertiary hyperparathyroidism) was frequent in kidney transplant recipients in our series. This condition presented with lower phosphate and 25-hydroxyvitamin D, and higher urinary calcium and intact fibroblast growth factor 23 levels compared to kidney transplant recipients without hypercalcemia, resembling a mild form of primary hyperparathyroidism. Despite these metabolic derangements, the risk of adverse graft outcomes was low.

Pathophysiology of bone disease in chronic kidney disease: from basics to renal osteodystrophy and osteoporosis.

Chronic kidney disease (CKD) is a highly prevalent disease that has become a public health problem. Progression of CKD is associated with serious complications, including the systemic CKD-mineral and bone disorder (CKD-MBD). Laboratory, bone and vascular abnormalities define this condition, and all have been independently related to cardiovascular disease and high mortality rates. The "old" cross-talk between kidney and bone (classically known as "renal osteodystrophies") has been recently expanded to the cardiovascular system, emphasizing the importance of the bone component of CKD-MBD. Moreover, a recently recognized higher susceptibility of patients with CKD to falls and bone fractures led to important paradigm changes in the new CKD-MBD guidelines. Evaluation of bone mineral density and the diagnosis of "osteoporosis" emerges in nephrology as a new possibility "if results will impact clinical decisions". Obviously, it is still reasonable to perform a bone biopsy if knowledge of the type of renal osteodystrophy will be clinically useful (low versus high turnover-bone disease). However, it is now considered that the inability to perform a bone biopsy may not justify withholding antiresorptive therapies to patients with high risk of fracture. This view adds to the effects of parathyroid hormone in CKD patients and the classical treatment of secondary hyperparathyroidism. The availability of new antiosteoporotic treatments bring the opportunity to come back to the basics, and the knowledge of new pathophysiological pathways [OPG/RANKL (LGR4); Wnt-ß-catenin pathway], also affected in CKD, offers great opportunities to further unravel the complex physiopathology of CKD-MBD and to improve outcomes.

Sodium Glycerophosphate Use in Parenteral Nutrition Improves Mineral Metabolism in Extremely Low Birth Weight Infants.

OBJECTIVE: To evaluate the clinical effect of sodium glycerophosphate (NaGP) in parenteral nutrition solutions on mineral metabolism in extremely low birth weight (ELBW) infants. STUDY DESIGN: NaGP was introduced for use in place of potassium phosphate (K3PO4) in January 2018; this retrospective cohort study included 95 ELBW infants treated with K3PO4 between January 2015 and December 2017 and 77 infants treated with NaGP between August 2018 and January 2021. Mineral intake over the first 14 days; changes in serum calcium, phosphorus, sodium, and alkaline phosphatase (ALP) levels over the first 1-3 months; and the rates of electrolyte imbalance and clinical morbidity were compared. High-risk infants who had nil per os (NPO) status for >14 days and prolonged parenteral nutrition exposure were further analyzed as a subgroup. RESULTS: The use of NaGP instead of K3PO4 significantly increased Ca and P intake, but intakes remained below the recommended range (Ca, 64-140 mg/kg/day; P, 50-108 mg/kg/day). Compared with levels in the K3PO4 group, the NaGP group had significantly higher serum Ca and P levels after day 14 and lower ALP levels after day 56. In the subgroup analysis, the NaGP group had significantly lower incidences of hypophosphatemia, hyponatremia, bronchopulmonary dysplasia, and ALP >500 IU/L. CONCLUSIONS: Although the administration of NaGP instead of K3PO4 in parenteral nutrition regimens still did not provide adequate Ca and P intake for ELBW infants, higher intake significantly improved serum Ca and P levels, especially in ELBW infants with prolonged parenteral nutrition exposure.

A Patient with Bone Fragility, Multiple Fractures, Osteosarcoma, and the Variant c.143A>G in the IFITM5 Gene: A Case Report.

Osteogenesis imperfecta (OI) is a group of genetic skeletal disorders, with a prevalence of 1 in 15,000-20,000 births. OI type V has been described in approximately 150 cases and all patients carry the variant (c.-14C> T) in the IFITM5 gene. However, two other variants, p.S40L and p.N48S have been reported in this gene, leading to clinical phenotypes different from OI type V. Here we described a patient with multiple bone fractures, scoliosis, skull alteration (plagiocephaly), bone deformation, bone rickets, and intramedullary epithelioid osteosarcoma that bears the recently reported heterozygous variant c.143A>G (p.N48S) in the IFITM5 gene. This case supports the pathogenicity of this new variant in the IFITM5 gene and adds information regarding its clinical phenotype.

Aerobic training improves bone fragility by reducing the inflammatory microenvironment in bone tissue in type 2 diabetes.

Aerobic training (AT) is indicated in type 2 diabetes mellitus (T2DM) to control hyperglycaemia and inflammation. AT improves bone microarchitecture and resistance to fracture. The intensity of AT and the mechanisms that lead to the improvement in bone quality are still unknown. Using a mouse model of T2DM, we evaluated the effects of two intensities of forced AT. We divided mice into: sedentary (SED), T2DM-SED, low runners (LOW), T2DM-LOW, high runners (HIGH) and T2DM-HIGH. The AT for low was 8 m/minute (m/min); 5° slope or high 18 m/min; 15° slope for 2 months. We measured metabolic parameters, the serum cytokines concentration, lipocalin-2 (LCN-2) and adiponectin; and the tibial concentrations of LCN-2, tumour necrosis factor alpha (TNF-α) and protein carbonylation (CO). We evaluated femur morphometry and biomechanical properties. We performed multiple correlation analysis. The T2DM-LOW versus T2DM-SED group, shown an increase of interleukin (IL)-10 (417 ± 90 vs 102 ± 25 pg/mL) and improved trabecular bone (BV/TV: 31.8 ± 2.3 vs 19.25 ± 1.4%; Tb.Sp.: 1.62 ± 0.02 vs 2.0 ± 0.07 mm), by a decrease bone CO (3.4 ± 0.1 vs 6.0 ± 0.5 nmol/mg), bone TNF-α (84 ± 4 vs 239 ± 13 pg/mL) and LCN-2 (2887 ± 23 vs 3418 ± 105 pg/mL). The T2DM-HIGH versus T2DM-SED group showed a greater hypoglycaemic effect (228 ± 10 vs 408 ± 5 mg/dL), with improved cortical bone density (0.26 ± 0.012 vs 0.21 ± 0.007 mm) and fracture resistance (102 ± 8 vs 78 ± 5 MPa), by a reduction of bone TNF-α (77 ± 34 vs 239 ± 13 pg/mL); LCN-2 (2768 ± 20 vs 3418 ± 105 pg/mL) and CO (4.8 ± 0.5 vs 6.0 ± 0.5 nmol/mg). In conclusion, AT improves bone morphometry and biomechanical properties by reducing the bone inflammatory microenvironment.

"A case of vanishing bone disease complicated by chylothorax- diagnosis and treatment".

A 16-year old girl with Gorham-Stout disease is presented. She had progressive replacement of the bones of her left arm and shoulder girdle by fibroadipose tissue and numerous proliferated, non-neoplastic, lymphatic channels. The clinico-pathologic features of this condition are discussed, as are its possible complications and available therapeutic modalities.

Cambios en el metabolismo óseo y mineral en pacientes con Enfermedad Renal Crónica sometidos a trasplante renal en el Hospital General Plaza de la Salud durante el período comprendido entre enero 2010­agosto 2018 / Changes in bone and mineral metabolism in patients with chronic kidney disease undergoing kidney transplantation at Plaza de la Salud General Hospital during the period between January 2010­August 2018

Introducción: las alteraciones del metabolismo óseo-mineral, son una causa importante de morbilidad en los pacientes con trasplante renal, por lo que el manejo de las complicaciones del paciente trasplantado, a largo plazo, deben de ser seguidas. El estudio intenta demostrar cambios en el metabolismo óseo y mineral en pacientes con enfermedad renal crónica sometidos a trasplante renal en el Hospital General Plaza de la Salud durante el período comprendido entre enero 2010 ­ agosto 2018, Santo Domingo, República Dominicana. Método: estudio observacional, descriptivo, retrospectivo y transversal de 131 trasplantes realizados en el Hospital General Plaza de la Salud, evaluando cambios de calcio (Ca), fósforo (P) y hormona paratiroidea (PTH) antes y tres meses post-trasplante. Resultados: la edad media de los pacientes incluidos fue 43.1 ±13.1 años, 72.51 % pertenecía al sexo masculino, con un tiempo medio en hemodiálisis en meses de 27.0 ± 33.6, 60 % de los trasplantes realizados fueron de donante vivo y un 63 % de los pacientes tenía HTA como comorbilidad. El nivel medio de PTH disminuyó en los primeros 3 meses posteriores al trasplante comparado con el pre-trasplante (779.6 ± 1004.0 vs. 167.9 ± 138.2 pg/ml). El fosfato disminuyó significativamente (4.9 ± 1.6 vs. 3.5 ± 0.8) y el calcio aumentó (9.0 ± 1.2 mg/dl vs. a 9.7± 0.8 mg/dl). Discusión: los cambios generales en los niveles séricos de Ca, P, PTH, BUN y creatinina desde el momento del TR a los 3 meses post TR, fueron todos significativos

Introduction: Alterations of bone-mineral metabolism are an important cause of morbidity in patients with kidney transplantation, so the management of long-term transplant patient complications should be followed. The study tries to demonstrate changes in bone and mineral metabolism in patients with chronic renal disease undergoing kidney transplant in the Hospital General Plaza de la Salud during the period January 2010 to August 2018, Santo Domingo, Dominican Republic. Method: Observational, Descriptive, Retrospective and Cross-sectional Study of 131 transplants performed at Hospital General Plaza de la Salud, evaluating changes of calcium (Ca), phosphorus (P) and parathyroid hormone (PTH) before and 3 months post-transplant. Results: The mean age of the patients included was 43.1 ± 13.1 years, 72.51% belonged to the male sex, with a mean time on hemodialysis in months of 27.0 ± 33.6, 60% of the transplants performed were from live donors and 63% from the patients had hypertension as comorbidity. The mean PTH level decreased in the first 3 months after transplantation compared to the pre-transplant (779.6 ± 1004.0 vs 167.9 ± 138.2 pg/ml). Phosphate decreased significantly (4.9 ± 1.6 vs 3.5 ± 0.8) and calcium increased (9.0 ± 1.2 mg / dl vs. 9.7 ± 0.8 mg / dl). Discussion: The general changes in serum levels of Ca, P, PTH, BUN and Creatinine from the time of TR to 3 months post TR were all significant

Doença metabólica óssea em prematuros de muito baixo peso internados em UTI neonatal: ocorrência e fatores associados / Metabolic bone disease in extremely low birth weight premature infants admitted to a neonatal ICU: prevalence and associated factors

Introdução: Recém-nascidos (RN) prematuros de muito baixo peso (MBP) apresentam um risco aumentado de desenvolver doença metabólica óssea (DMO). A realização de suplementação mineral e de triagem para DMO em UTI Neonatal auxilia na prevenção, no diagnóstico e no tratamento desta condição clínica. Objetivos: Avaliar a ocorrência de DMO, a realização de suplementação mineral e de investigação para DMO em prematuros de MBP internados em UTI Neonatal de um Serviço Hospitalar Materno-Infantil de alto risco, vinculado ao SUS. Métodos: Estudo transversal, a partir de dados secundários coletados de prontuários eletrônicos. Foram avaliados os recém-nascidos prematuros de muito baixo peso internados em UTI neonatal. Estudaram-se perfil do recém-nascido, realização da triagem para DMO, ocorrência de DMO, regime alimentar, intercorrências clínicas, uso de suplementação de vitaminas e minerais, idade, peso, sexo, via de parto e Apgar. Resultados: Foram incluídos 112 participantes. A triagem para DMO foi feita em 56 pacientes (50%), com dosagem sérica de fosfatase alcalina, cálcio, fósforo e magnésio séricos. A ocorrência de DMO foi de 8,9% (5 casos). Todos os participantes com DMO apresentaram doença respiratória, quadro infeccioso e estavam recebendo nutrição parenteral. Conclusão: A ocorrência de DMO em recém-nascidos de muito baixo peso internados em UTI neonatal foi de 8,9%, inferior à descrita na literatura. Identificamos como fatores associados à DMO a ocorrência concomitante de doença respiratória, a sepse e o uso de nutrição parenteral.

Introduction: Extremely low birth weight (ELBW) premature infants have an increased risk of developing metabolic bone disease (MBD). The use of mineral supplementation and MBD screening in the neonatal ICU helps prevent, diagnose, and treat this clinical condition. Objectives: To evaluate the occurrence of MBD, as well as the presence of mineral supplementation and MBD screening in premature infants with MBD admitted to the neonatal ICU of a high-risk maternal-child hospital associated with the Brazilian Unified Health System. Methods: This was a cross-sectional study based on secondary data from electronic medical records. We evaluated ELBW premature infants admitted to a neonatal ICU. Infant profile, MBD screening, MBD occurrence, diet, clinical complications, vitamin and mineral supplementation use, age, weight, sex, delivery method, and Apgar score were assessed. Results: We included 112 participants in the study. MBD screening was conducted in 56 patients (50%) with serum levels of alkaline phosphatase, calcium, phosphorus, and magnesium. The rate of MBD occurrence was 8.9% (5 cases). All participants with MBD had respiratory disease, infection, and were receiving parenteral nutrition. Conclusions: The occurrence rate of MBD in ELBW infants admitted to a neonatal ICU was 8,9%, lower than that found in the literature. Factors associated with MBD were the concomitant occurrence of respiratory disease, sepsis, and parenteral nutrition.

Age and Spinal Disease Correlate to Albumin and Vitamin D Status.

STUDY DESIGN: Cross-sectional study. OBJECTIVES: Thus, this study aimed to assess the epidemiological profile of a patient sample that underwent spinal surgery regarding their nutritional and vitamin D status. METHODS: Serum albumin and vitamin D (25-hydroxyvitamin D) levels were measured in patients with different spinal surgical approaches and various pathologies at a single institution. 112 patients were retrospectively identified for inclusion and stratified by age into 4 age groups and by pathology. The nutritional status of the patients was classified in vitamin D inadequacy (< 30ng/mL), vitamin D deficiency (<20ng/mL), and hypoalbuminemia (<3.5g/dL). Data was analyzed comparing vitamin D, and albumin means considering gender, age group, and pathologies. RESULTS: Twenty-eight (25.2%) patients had hypoalbuminemia. There was no difference between gender (p = 0.988); there was a significant decrease in albumin concentration increasing the age (p < 0.001). The prevalence of hypoalbuminemia was significantly higher in patients with trauma, tumor and infection than in those patients with degenerative and deformity diseases (p = 0.003). The prevalence of vitamin D inadequacy was 33.7%, and that of deficiency was 62.2%, while severe deficiency (< 10 ng/mL) in 16.3%. The vitamin D concentration was significantly different among the pathologies (P = 0.047), the lower concentration occurring in patients with tumor. CONCLUSION: Older patients, as well as patients with tumor and infectious pathologies, seem to have a higher prevalence of hypoalbuminemia, inferring malnutrition. There was a low epidemic level of vitamin D concentration, almost all patients presenting some degree of hypovitaminosis D, independent of age, gender and nutritional status.

Long-read single molecule real-time (SMRT) sequencing of GBA1 locus in Gaucher disease national cohort from Argentina reveals high frequency of complex allele underlying severe skeletal phenotypes: Collaborative study from the Argentine Group for Diagnosis and Treatment of Gaucher Disease.

Gaucher disease is reckoned for extreme phenotypic diversity that does not show consistent genotype/phenotype correlations. In Argentina, a national collaborative group, Grupo Argentino de Diagnóstico y Tratamiento de la Enfermedad de Gaucher, GADTEG, have delineated uniformly severe type 1 Gaucher disease manifestations presenting in childhood with large burden of irreversible skeletal disease. Here using Long-Read Single Molecule Real-Time (SMRT) Sequencing of GBA1 locus, we show that RecNciI allele is highly prevalent and associates with severe skeletal manifestations in childhood.

Comparison between different prognostic models to be used for metastatic bone disease on appendicular skeleton in a Chilean population.

PURPOSE: Several preoperation prognosis models used on the treatment of metastatic bone disease on appendicular skeleton have been devised. The purpose of this study was to compare the performance of different survival prognostic models on patients with metastatic bone disease in long bones in a Chilean population. METHODS: This is a multicentric retrospective study. We retrospectively reviewed the medical records of 136 patients who were confirmed with metastatic bone disease of the appendicular skeleton and who were treated surgically from 2016 to 2019. The minimum follow-up time was 12 months. All patients were assessed using four appendicular metastatic bone disease scoring systems. A preoperative predicted survival time for all 136 patients was retrospectively calculated making use of the revised Katagiri, PathFx, Optimodel and IOR score model. RESULTS: The PathFx model demonstrated an accuracy at predicting 3 (area under the curve [AUC] = 0.61) and 6-month (AUC = 0.65) survival time after surgical management. IOR score model demonstrated an accuracy at predicting 12-month survival time (AUC = 0.64). The survival rate reached the 44% in a year. The median survival time to death or last follow-up time was 14.9 months (SD ± 15). CONCLUSION: PathFx score model demonstrated the highest accuracy at predicting a survival time of 3 and 6 months. IOR score model was the most accurate measure at predicting a survival time of 12-months. To our knowledge, this is the first study reporting a comparative analysis of metastatic bone disease with predicting models in a country located in Latin America. PathFx's and IOR score models are the ones to be used in the Chilean population as the predictive models in metastatic bone disease of the appendicular skeleton.

Cambios Clínicos e Imagenológicos en Terapias no Invasivas en Articulaciones Temporomandibulares con Alteraciones Óseas Degenerativas / Clinical and Imaging Changes with Non-Invasive Therapies in Temporomandibular Joints with Degenerative Joint Disease

El objetivo del presente estudio fue describir los cambios clínicos e imagenológicos de las terapias no invasivas aplicadas a pacientes con alteraciones óseas degenerativas de las articulaciones temporomandibulares (ATM). Metodología: Se evaluaron 25 pacientes con alteraciones óseas degenerativas de las ATM, sin tratamiento previo de trastornos temporomandibulares (TTM) al momento del diagnóstico. Se realizó tratamiento no invasivo y un año después fueron evaluados según criterios clínicos e imagenológicos DC/TMD y Ahmad. Los resultados fueron presentados por medio de estadística descriptiva, odds ratio con sus respectivos intervalos de confianza, comparaciones de medianas y correlaciones. Se estudiaron 50 ATM, 72 % mujeres (32,2 años promedio). Se observó mejora significativa en los parámetros: dolor (p=0,0001), sinovitis (p=0,001) e incremento de la esclerosis del trabeculado óseo (p=0,051) a un año post-tratamiento. Después de un año del establecimiento de terapias no invasivas en pacientes con alteraciones óseas degenerativas de las ATM, se observaron cambios positivos tanto clínicos como imagenológicos, reduciéndose significativamente la sintomatología dolorosa, limitándose la progresión del daño óseo degenerativo, y observándose recuperación de los casos de sinovitis.

The objective of this study was to describe clinical and imaging changes of non-invasive therapies applied to patients with degenerative bone disorders of the temporomandibular joints (TMJ). To carry out this study, 25 patients with degenerative bone disorders of TMJ without previous treatment at the time of diagnosis, were evaluated. Non-invasive treatment was performed and one year later they were evaluated according to clinical and imaging criteria DC/TMD and Ahmad. Results were presented by descriptive statistics, odds ratio, confidence interval, comparisons of means, and correlations. 50 TMJs, 72 % women, (32.2 years mean of age) were studied. Significant improvement was observed in the parameters: pain (p=0.0001), synovitis (p=0.001), and increased sclerosis of the bone trabeculae (p=0,051) at one-year post-treatment. After one year of the establishment of non-invasive therapies in patients with degenerative joint disease of TMJ, it was observed positive changes, both clinical and imaging, reducing painful symptomatology, limiting effect on the progression of degenerative bone damage, and recovery of synovitis cases.

Biphasic α2ß1 Integrin Expression in Breast Cancer Metastasis to Bone.

Integrins participate in the pathogenesis and progression of tumors at many stages during the metastatic cascade. However, current evidence for the role of integrins in breast cancer progression is contradictory and seems to be dependent on tumor stage, differentiation status, and microenvironmental influences. While some studies suggest that loss of α2ß1 enhances cancer metastasis, other studies suggest that this integrin is pro-tumorigenic. However, few studies have looked at α2ß1 in the context of bone metastasis. In this study, we aimed to understand the role of α2ß1 integrin in breast cancer metastasis to bone. To address this, we utilized in vivo models of breast cancer metastasis to bone using MDA-MB-231 cells transfected with an α2 expression plasmid (MDA-OEα2). MDA cells overexpressing the α2 integrin subunit had increased primary tumor growth and dissemination to bone but had no change in tumor establishment and bone destruction. Further in vitro analysis revealed that tumors in the bone have decreased α2ß1 expression and increased osteolytic signaling compared to primary tumors. Taken together, these data suggest an inverse correlation between α2ß1 expression and bone-metastatic potential. Inhibiting α2ß1 expression may be beneficial to limit the expansion of primary tumors but could be harmful once tumors have established in bone.

Bone Marrow Adiposity in Premenopausal Women With Type 2 Diabetes With Observations on Peri-Trabecular Adipocytes.

CONTEXT: No study has yet evaluated the relationships among bone marrow adiposity (BMA), bone histomorphometry (BH), and glycemic control in premenopausal women with type 2 diabetes (T2DM). OBJECTIVE: We aimed to assess the effect of glycemic control on BMA, correlate the parameters of BH with BMA, and correlate BMA with the use of hypoglycemic agents and with bone mineral density (BMD). METHODS: This was a cross-sectional study that evaluated 26 premenopausal women with T2DM who were divided into groups with HbA1c < 7% (good control [GC], n = 10) and HbA1c > 7% (poor control [PC], n = 16). BMA parameters (adipocyte number [Ad.N], total adipocyte perimeter [Ad.Pm], total adipocyte area [Ad.Ar], percentage adipocyte volume per marrow volume [Ad.V/Ma.V]) and peri-trabecular adipocyte number divided by bone surface (Ad.N/BS) were evaluated. BH static (bone volume fraction [BV/TV], osteoid thickness [O.Th], osteoid surface/bone surface [OS/BS]) and dynamic parameters and serum insulin-like growth factor-1 were measured. BMA data were compared between the GC and PC groups. Correlations were performed. RESULTS: Ad.N, Ad.Pm, and Ad.Ar were higher in PC (all, P = 0.04). HbA1c correlated positively with Ad.N/BS (P < 0.01) and Ad.N/BS correlated negatively with O.Th (P < 0.01) and OS/BS (P = 0.02). Positive and negative correlations were observed between insulin and metformin use, respectively, with all adipocyte parameters except Ad.N/BS (P < 0.05). Structural parameters were negatively correlated with the BMA. BMD of the femoral neck (r = -549, P < 0.01) and total femur (r = -0.502, P < 0.01) were negatively correlated with Ad.V/Ma.V. CONCLUSION: Poor glycemic control is associated with hyperplasia and hypertrophy of BMAs and with lower BV/TV. Ad.N/BS, a new BMA parameter, is correlated with HbA1c and negatively with O.Th. The use of insulin seems to stimulate the expansion of BMA while that of metformin has the opposite effect. These findings suggest that the increase in BMA may play a role in the T2DM bone disease; on the other hand, good glycemic control might help prevent it.