Enfermedad ósea de Paget: 1877-2023. Etiología y abordaje de una enfermedad en transición epidemiológica / Paget's disease of bone: 1877–2023. Etiology, and management of a disease on epidemiologic transition

La enfermedad ósea de Paget se caracteriza por la alteración, en una o varias localizaciones óseas, del equilibrio entre formación y resorción ósea. Este desequilibrio da lugar a un hueso ensanchado, desorganizado, en muchos casos con una densidad ósea aumentada, aunque más frágil. Existiría una predisposición genética para su desarrollo, que explicaría entre un 5 y un 40% de los casos, sobre la que actuarían distintos factores ambientales. La enfermedad ósea de Paget fue considerada clásicamente la segunda enfermedad metabólica ósea más frecuente. Sin embargo, durante las últimas décadas presenta un marcado descenso tanto de la incidencia como de la gravedad clínica, lo que ha llevado a especular sobre la disminución o desaparición de la influencia de algún factor ambiental. Este descenso en la incidencia no debe servir como excusa para el abandono de su estudio, sino ser la razón para tratar de entender mejor su patogenia (AU)

Paget's disease of bone is characterized by the alteration, in one or several bone locations, of the equilibrium between bone formation and bone resorption. This imbalance results in a disorganized, widened bone, in many cases with increased bone density, although more fragile. A genetic predisposition for Paget's disease of bone could explain between 5% and 40% of the cases. Different environmental factors should explain the rest of the cases. Paget's disease of bone was classically considered the second most common metabolic bone disease. However, in recent decades there has been a marked decrease in both incidence and clinical severity. These changes have led to believe that the influence of some environmental factor may have diminished or even disappeared. This decrease in incidence should not be an excuse for abandoning Paget's disease of bone research, but rather it should be the reason to remain searching to try to understand better its pathogenesis(AU)

[Paget's disease of bone: 1877-2023. Etiology, and management of a disease on epidemiologic transition]. / Enfermedad ósea de Paget: 1877-2023. Etiología y abordaje de una enfermedad en transición epidemiológica.

Paget's disease of bone is characterized by the alteration, in one or several bone locations, of the equilibrium between bone formation and bone resorption. This imbalance results in a disorganized, widened bone, in many cases with increased bone density, although more fragile. A genetic predisposition for Paget's disease of bone could explain between 5% and 40% of the cases. Different environmental factors should explain the rest of the cases. Paget's disease of bone was classically considered the second most common metabolic bone disease. However, in recent decades there has been a marked decrease in both incidence and clinical severity. These changes have led to believe that the influence of some environmental factor may have diminished or even disappeared. This decrease in incidence should not be an excuse for abandoning Paget's disease of bone research, but rather it should be the reason to remain searching to try to understand better its pathogenesis.

Oligometastases in oncological orthopedic surgery. Our experience. / Oligometástasis en la cirugía ortopédica oncológica. Nuestra experiencia.

OBJECTIVE: To analyze the incidence and survival of patients with oligometastases (solitary and normal) when they are treated in centers that are experts in multidisciplinary approach to patients with sarcoma. MATERIAL AND METHOD: Retrospective analysis of 414 patients with bone metastases secondary to carcinomas at Hospital Universitario La Paz and Hospital MD Anderson Cancer Center (Madrid) between May 2006 and May 2019. Metastases located in the pelvis and axial skeleton were excluded, analyzing a total of 28 patients who met the criterion for solitary metastases or oligometastases with normal criteria. The study survival estimate was carried out following the Kaplan-Meier statistical method. RESULTS: The survival of the patients following the oligometastases criteria (solitary and normal) was 53%. Breast cancer was the most prevalent and had a survival rate of more than 70%. The average age of the patients was 58 years old. DISCUSSION: Systemic treatments in cancer treatment have managed to improve disease-free survival curves and lead us to redirect on the paradigm for the treatment of oligometastases, stating that treatment should be carried out in the centers that are experts in the treatment of sarcomas. CONCLUSIONS: The choice of surgical treatment for patients with oligometastases in the strict sense (solitary) and normal should be evaluated by multidisciplinary teams according to the prognoses of the patient, anatomical location and histiotype of the neoplasm.

Adynamic bone disease: Revisited.

The bone and mineral disorders form an integral part of the management of a chronic kidney disease (CKD) patient. Amongst various types of bone pathologies in chronic kidney disease-mineral bone disorder (CKD-MBD), the prevalence of adynamic bone disease (ABD) is increasing. The present review discusses the updated pathophysiology, risk factors, and management of this disorder.

Oligometástasis en la cirugía ortopédica oncológica. Nuestra experiencia / Oligometastases in oncological orthopedic surgery. Our experience

Objetivo: Analizar la incidencia, factores pronósticos, indicación de tratamiento quirúrgico y supervivencia de los pacientes con criterios de oligometástasis óseas con los criterios de solitaria y normal ósea tratados en centros expertos en la resección tumoral de lesiones óseas. Material y método: Análisis retrospectivo de 414 pacientes afectos de metástasis óseas secundarias a carcinomas del Hospital Universitario La Paz y Hospital MD Anderson Cancer Center (Madrid), entre mayo de 2006 y mayo de 2019. Fueron excluidos las metástasis localizadas en pelvis y esqueleto axial, analizándose un total de 28 pacientes que cumplían con el criterio de metástasis solitaria u oligometástasis con criterio normal. La estimación de la supervivencia del estudio se llevó a cabo siguiendo el método estadístico de Kaplan-Meier. Resultados: La supervivencia de los pacientes siguiendo los criterios de oligometástasis (solitaria y normal) fue del 53%. El cáncer de mama fue el más prevalente y presentó una supervivencia superior al 70%. La edad promedio de los pacientes fue de 58 años. Discusión: Los tratamientos sistémicos en el tratamiento del cáncer han conseguido mejorar las curvas de supervivencia libre de enfermedad y nos lleva a reflexionar sobre el paradigma del tratamiento de las oligometástasis, planteando que el tratamiento debería realizarse en centros expertos en la resección tumoral de lesiones óseas. Conclusiones: La elección del tratamiento quirúrgico de los pacientes afectos de oligometástasis en sentido estricto (solitaria) y normal deben ser evaluada por equipos multidisciplinarios, según el pronóstico del paciente, localización anatómica e histiotipo de la neoplasia. Nivel de evidencia 3.(AU)

Objective: To analyze the incidence and survival of patients with oligometastases (solitary and normal) when they are treated in centers that are experts in multidisciplinary approach to patients with sarcoma. Material and method: Retrospective analysis of 414 patients with bone metastases secondary to carcinomas at Hospital Universitario La Paz and Hospital MD Anderson Cancer Center (Madrid) between May 2006 and May 2019. Metastases located in the pelvis and axial skeleton were excluded, analyzing a total of 28 patients who met the criterion for solitary metastases or oligometastases with normal criteria. The study survival estimate was carried out following the Kaplan–Meier statistical method. Results: The survival of the patients following the oligometastases criteria (solitary and normal) was 53%. Breast cancer was the most prevalent and had a survival rate of more than 70%. The average age of the patients was 58 years old. Discussion: Systemic treatments in cancer treatment have managed to improve disease-free survival curves and lead us to redirect on the paradigm for the treatment of oligometastases, stating that treatment should be carried out in the centers that are experts in the treatment of sarcomas. Conclusions: The choice of surgical treatment for patients with oligometastases in the strict sense (solitary) and normal should be evaluated by multidisciplinary teams according to the prognoses of the patient, anatomical location and histiotype of the neoplasm. Level of evidence 3.(AU)

[Translated article] Oligometastases in oncological orthopaedic surgery. Our experience / Oligometástasis en la cirugía ortopédica oncológica. Nuestra experiencia

Objetivo: Analizar la incidencia, factores pronósticos, indicación de tratamiento quirúrgico y supervivencia de los pacientes con criterios de oligometástasis óseas con los criterios de solitaria y normal ósea tratados en centros expertos en la resección tumoral de lesiones óseas. Material y método: Análisis retrospectivo de 414 pacientes afectos de metástasis óseas secundarias a carcinomas del Hospital Universitario La Paz y Hospital MD Anderson Cancer Center (Madrid), entre mayo de 2006 y mayo de 2019. Fueron excluidos las metástasis localizadas en pelvis y esqueleto axial, analizándose un total de 28 pacientes que cumplían con el criterio de metástasis solitaria u oligometástasis con criterio normal. La estimación de la supervivencia del estudio se llevó a cabo siguiendo el método estadístico de Kaplan-Meier. Resultados: La supervivencia de los pacientes siguiendo los criterios de oligometástasis (solitaria y normal) fue del 53%. El cáncer de mama fue el más prevalente y presentó una supervivencia superior al 70%. La edad promedio de los pacientes fue de 58 años. Discusión: Los tratamientos sistémicos en el tratamiento del cáncer han conseguido mejorar las curvas de supervivencia libre de enfermedad y nos lleva a reflexionar sobre el paradigma del tratamiento de las oligometástasis, planteando que el tratamiento debería realizarse en centros expertos en la resección tumoral de lesiones óseas. Conclusiones: La elección del tratamiento quirúrgico de los pacientes afectos de oligometástasis en sentido estricto (solitaria) y normal deben ser evaluada por equipos multidisciplinarios, según el pronóstico del paciente, localización anatómica e histiotipo de la neoplasia. Nivel de evidencia 3.(AU)

Objective: To analyze the incidence and survival of patients with oligometastases (solitary and normal) when they are treated in centers that are experts in multidisciplinary approach to patients with sarcoma. Material and method: Retrospective analysis of 414 patients with bone metastases secondary to carcinomas at Hospital Universitario La Paz and Hospital MD Anderson Cancer Center (Madrid) between May 2006 and May 2019. Metastases located in the pelvis and axial skeleton were excluded, analyzing a total of 28 patients who met the criterion for solitary metastases or oligometastases with normal criteria. The study survival estimate was carried out following the Kaplan–Meier statistical method. Results: The survival of the patients following the oligometastases criteria (solitary and normal) was 53%. Breast cancer was the most prevalent and had a survival rate of more than 70%. The average age of the patients was 58 years old. Discussion: Systemic treatments in cancer treatment have managed to improve disease-free survival curves and lead us to redirect on the paradigm for the treatment of oligometastases, stating that treatment should be carried out in the centers that are experts in the treatment of sarcomas. Conclusions: The choice of surgical treatment for patients with oligometastases in the strict sense (solitary) and normal should be evaluated by multidisciplinary teams according to the prognoses of the patient, anatomical location and histiotype of the neoplasm. Level of evidence 3.(AU)

[Translated article] Oligometastases in oncological orthopaedic surgery. Our experience.

OBJECTIVE: To analyse the incidence and survival of patients with oligometastases (solitary and normal) when they are treated in centres that are experts in multidisciplinary approach to patients with sarcoma. MATERIAL AND METHOD: Retrospective analysis of 414 patients with bone metastases secondary to carcinomas at Hospital Universitario La Paz and Hospital MD Anderson Cancer Centre (Madrid) between May 2006 and May 2019. Metastases located in the pelvis and axial skeleton were excluded, analyzing a total of 28 patients who met the criterion for solitary metastases or oligometastases with normal criteria. The study survival estimate was carried out following the Kaplan-Meier statistical method. RESULTS: The survival of the patients following the oligometastases criteria (solitary and normal) was 53%. Breast cancer was the most prevalent and had a survival rate of more than 70%. The average age of the patients was 58 years old. DISCUSSION: Systemic treatments in cancer treatment have managed to improve disease-free survival curves and lead us to redirect on the paradigm for the treatment of oligometastases, stating that treatment should be carried out in the centres that are experts in the treatment of sarcomas. CONCLUSIONS: The choice of surgical treatment for patients with oligometastases in the strict sense (solitary) and normal should be evaluated by multidisciplinary teams according to the prognoses of the patient, anatomical location and histotype of the neoplasm.

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

Adynamic bone disease: Revisited / Enfermedad ósea adinámica: Una revisión

The bone and mineral disorders form an integral part of the management of a chronic kidney disease (CKD) patient. Amongst various types of bone pathologies in chronic kidney disease-mineral bone disorder (CKD-MBD), the prevalence of adynamic bone disease (ABD) is increasing. The present review discusses the updated pathophysiology, risk factors, and management of this disorder. (AU)

Los trastornos óseos y minerales son una parte fundamental del tratamiento del paciente con enfermedad renal crónica (ERC). Entre los distintos tipos de patologías óseas en la enfermedad renal crónica-trastorno mineral óseo (ERC-TMO), la prevalencia de la enfermedad ósea adinámica (EOA) está aumentando. En esta revisión se analizan los datos actuales sobre la fisiopatología, los factores de riesgo y el tratamiento de este trastorno. (AU)

Adynamic bone disease: Revisited.

The bone and mineral disorders form an integral part of the management of a chronic kidney disease (CKD) patient. Amongst various types of bone pathologies in chronic kidney disease-mineral bone disorder (CKD-MBD), the prevalence of adynamic bone disease (ABD) is increasing. The present review discusses the updated pathophysiology, risk factors, and management of this disorder.

Renal tubular acidosis (RTA) and kidney stones: Diagnosis and management. / Acidosis tubular renal (ATR) y litiasis renal: Diagnóstico y manejo.

Renal tubular acidosis (RTA) is a set of raredis orders in which the renal tubule is unable to excreteacid normally and there by maintain normal acid-basebalance, resulting in a complete or incomplete metabolicacidosis. In distal RTA (dRTA, also known as classicalor type 1 RTA), there is a defect in excreting H+ ionsalong the distal nephron (distal tubule and collectingduct), leading to an alkaline urinary pH with calcium phosphate precipitation and stones. Causes of dRTAinclude genetic mutations, autoimmune disease, and some drugs.Clinical manifestations of the genetic forms of dRTA typically occur during childhood and may vary from mildclinical symptoms, such as a mild metabolic acidosis, hypokalaemia,and incidental detection of kidney stones, to more serious manifestations such as failure to thrive,severe metabolic acidosis, rickets and nephrocalcinosis.Progressive hearing loss may develop in patients withrecessive dRTA, which, depending the causative genemutation, can be present at birth or develop later in adolescence or early adulthood. Diagnosis of dRTA can be challenging, since it requires a high index of suspicion and/or measurement of urinary pH after an acid load, usually in the form of oral ammonium chloride; this should normally acidify the urine to pH below 5.3. In dRTA, urinary citrate levels a real so low and patients are at increased risk of for mingkidney stones from a combination of alkaline urine and low citrate. Ideally, affected patients need regular outpatient follow-up by a urologist and nephrologist. Thus, any patient found to have a calcium phosphate kidney stone, low urinary citrate, and raised urinary pH, especially with an early morning pH >5.5, should be evaluated for underlying dRTA. Patients with complete dRTA will have a low (<20 mmol/L) plasma or serum bicarbonate concentration, whereas in those with incomplete dRTA, bicarbonate levels are usually normal. Oral alkali as potassiumcitrate is still the mainstay of treatment in dRTA.

La acidosis tubular renal (ATR) es un conjunto de enfermedades raras en las que el túbulo renal es incapaz de excretar ácido de forma normal y por ello de mantener un balance ácido-base normal, resultando en una acidosis metabólica completa o incompleta. En la ATR distal (ATRd, también conocida como ATR tipo 1 o clásica), hay un defecto en la excreción de iones H+a lo largo de la parte distal de la nefrona (túbulo distal  y tubo colector) que conduce a un pH urinario alcalino con precipitación de fosfato cálcico y litiasis. Las causas de la ATRd incluyen mutaciones genéticas, enfermedad autoinmune y algunos fármacos. Las manifestaciones clínicas de la forma genética de la ATRd ocurren típicamente durante la infancia y pueden variar desde síntomas leves, como acidosis metabólica leve, hipokaliemia y detección accidental de litiasis renal, hasta manifestaciones más graves tales como falta de crecimiento, acidosis metabólica severa, raquitismo y nefrocalcinosis. En pacientes con ATRd recesiva puede desarrollarse una pérdida progresiva de audición que, dependiendo de la causa de la mutación genética, puede estar presente en el momento del nacimientoo desarrollarse más tarde en la adolescencia o edad adulta temprana.El diagnóstico de la ATRd puede ser un reto ya que requiere un alto grado de sospecha y/o la medición del pH urinario tras una carga ácida, normalmente en forma de cloruro amónico oral; esto debería normalmente acidificar la orina a un pH inferior a 5,3. En la ATRd, los niveles de citrato urinario también son bajos y los pacientes tienen un mayor riesgo de formar litiasis renal por una combinación de orina alcalina e hipocitraturia. Lo ideal es que los pacientes afectados sean seguidos de forma regular por un urólogo y un nefrólogo. Así,cualquier paciente con litiasis de fosfato cálcico, hipocitraturia y pH urinario elevado, especialmente con un pH urinario matutino >5,5, debería ser estudiado para descartar una ATRd oculta. Los pacientes con ATRd completa tendrán una concentración plasmática o sérica de bicarbonato baja (<20 mmol/L), mientras que en aquellos con una ATRd incompleta, los niveles de bicarbonato son generalmente normales. Los alcalinizantes orales como el citrato potásico son aún el principal pilar del tratamiento en la ATRd.

Distar Renal Tubular Acidosis (dRTA): Epidemiological, diagnostics, clinical follow-up and therapeutical issues. Nephrologists cohort survey outcome.

BACKGROUND AND OBJECTIVES: dRTA is a genetic or acquired rare disease, characterized by an unability to excrete hydrogens (H+) into urine, hypobicarbonatemia, hyperchloremia, and frequently hypercalciuria and hypokalaemia. Genetic forms are usually diagnosed during the first months of life and its treatment is based on providing alkali supplements in order to prevent long term clinical consequences, particularly chronic kidney disease (described in some cohorts up to 82% of dRTA patients) and the associated bone disease. A 10 queries multi choice closed response survey was designed to know more about epidemiological, diagnostics, clinical management and therapeutical issues of this disease among Spanish nephrologists. METHODS AND MATERIALS: This survey was delivered to the attendees to a scientific meeting on dRTA at the Spanish Nephrology Society congress in 2019. Surveys were collected at the end of this dRTA event. Results were analyzed by using a parametric statistical test, obtaining the percentage of each response for the 10 questions. RESULTS: Among the survey responders, 44.4% and 37.7% did not visit any dRTA patient during the 1st and 3rd last year respectively. When having a suspicious diagnose, confirming genetic diagnostic test is only performed on the 13.3% of cases and pedigree studies only on 11.1%. Only a 26.6% confirms that metabolic control is excellent, good or very good. 69% of the responders believe that treatment compliance is not bad, bad or very bad. CONCLUSIONS: This survey enhances the fact that dRTA is not a well known entity, satisfaction with metabolic control is poor and compliance is low. All these factors can lead to a higher severity of renal and bone diseases associated to dRTA.

Distar Renal Tubular Acidosis (dRTA): Epidemiological, diagnostics, clinical follow-up and therapeutical issues. Nephrologists cohort survey outcome. / Acidosis tubular renal distal (ATRd): aspectos epidemiológicos, diagnósticos, de seguimiento clínico y terapéuticos. Resultados de una encuesta a un colectivo de nefrólogos.

BACKGROUND AND OBJECTIVES: dRTA is a genetic or acquired rare disease, characterized by an unability to excrete hydrogens (H+) into urine, hypobicarbonatemia, hyperchloremia, and frequently hypercalciuria and hypokalaemia. Genetic forms are usually diagnosed during the first months of life and its treatment is based on providing alkali supplements in order to prevent long term clinical consequences, particularly chronic kidney disease (described in some cohorts up to 82% of dRTA patients) and the associated bone disease. A 10 queries multi choice closed response survey was designed to know more about epidemiological, diagnostics, clinical management and therapeutical issues of this disease among Spanish nephrologists. MATERIALS AND METHODS: This survey was delivered to the attendees to a scientific meeting on dRTA at the Spanish Nephrology Society congress in 2019. Surveys were collected at the end of this dRTA event. Results were analyzed by using a parametric statistical test, obtaining the percentage of each response for the 10 questions. RESULTS: Among the survey responders, 44.4% and 37.7% did not visit any dRTA patient during the 1st and 3rd last year respectively. When having a suspicious diagnose, confirming genetic diagnostic test is only performed on the 13.3% of cases and pedigree studies only on 11.1%. Only a 26.6% confirms that metabolic control is excellent, good or very good, and 69% of the responders believe that treatment compliance is not bad, bad or very bad. CONCLUSIONS: This survey enhances the fact that dRTA is not a well known entity, satisfaction with metabolic control is poor and compliance is low. All these factors can lead to a higher severity of renal and bone diseases associated to dRTA.

No dejemos morir al hueso: interiorizándonos con los procesos óseos avasculares / Do not let the Bone Die: Interiorizing with the avascular Bone Processes

Resumen El objetivo de este artículo es realizar una revisión de las localizaciones, causas y hallazgos radiológicos específicos de los procesos avasculares óseos. Se define como isquemia ósea a las alteraciones ocasionadas por déficit de irrigación, llevando a la destrucción del hueso. Existen diferencias entre necrosis isquémica e infarto óseo, en base al sitio óseo de afectación. La etiología traumática es la más frecuente y suele ser unilateral. Los sitios de presentación más frecuentes son: cabeza femoral y humeral, rodilla y semilunar. La resonancia magnética (RM) es considerada la modalidad de imagen más sensible y específica, tanto para el diagnóstico temprano como estadificación y control. El conocimiento de las causas, características radiológicas y sus diferentes fases evitan el diagnóstico erróneo de otras etiologías, como las primarias o infecciosas, facilitando un correcto algoritmo terapéutico.

Abstract The aim of this article is to review the locations, causes and specific radiological findings of avascular bone processes. Bone ischemia is defined as the alterations caused by irrigation deficit, leading into the bone destruction. However, there are differences between ischemic necrosis and bone infarction, based on the bone site of involvement. The most frequent etiology is traumatic and is usually unilateral. The most frequent sites of presentation are: femoral and humeral head, knee, and lunate. Magnetic resonance imaging (MRI) is considered the most sensitive and specific imaging modality for early diagnosis, staging and control. The knowledge of the causes, radiological findings and their different phases avoid the erroneous diagnosis of other lesions, such as primary or infectious, facilitating a correct therapeutic algorithm.

Hearing in Paget's disease of bone. / La audición en la enfermedad ósea de Paget.

INTRODUCTION AND OBJECTIVES: Paget's disease of bone (PDB) may lead to hearing loss. The present study was conducted with the aim of measuring, characterizing and determining the risk factors for hearing loss in a group of subjects with PDB. METHODS: An observational, transversal, case-control study was conducted, a cohort of 76 subjects diagnosed with PDB in the case group and a control group of 134 subjects were included. Clinical, demographic and audiometric data were analysed. RESULTS: The comparative analysis between the subjects in the PDB group and the control group found that the case group showed higher hearing thresholds (39,51dB) compared with the control group (37.28dB) (P=.069) and presented a greater rate of conductive hearing loss (22.76%) than the control group (12.05%) (P=.0062). The study of risk factors for hearing loss found that skull involvement in bone scintigraphy, age and high blood pressure were risk factors for higher impairment in PDB. CONCLUSIONS: The subjects with PDB showed more profound and a higher proportion of conductive hearing loss than the control group. The patients with PDB and skull involvement presented a more severe hearing loss compared with the subjects without skull involvement. Skull involvement and age were found to be risk factors for hearing loss.

Manejo con instrumentación 360 o de enfermedad ósea metastásica que condiciona inestabilidad en columna cervical. Reporte de un caso clínico / 360 degrees instrumentation for cervical instability due to metastatic bone disease. Case report

Resumen: Introducción: La columna vertebral es el sitio más frecuente de localización para las metástasis óseas; siendo los tumores de mama, próstata y pulmón los que mayor afectación presentan. La columna torácica es afectada en un 70% de los casos, seguida de la región lumbar y cervical. Material y métodos: se presenta el caso de un paciente con diagnóstico de adenocarcinoma mamario derecho, con enfermedad ósea metastásica en región cervical a nivel de C2, C3, inestabilidad del segmento cervical por fractura por compresión del cuerpo C3 de mayor al 80%, sin invasión a canal medular, inicia con parestesias de extremidades torácicas. Se realiza instrumentación 360º en dos tiempos. Posterior al procedimiento la paciente evolucionó sin dolor cervical y tolerando la vía oral. Discusión: la enfermedad ósea metastásica genera lesiones importantes en la columna vertebral condicionando inestabilidad; la instrumentación mejora el estado funcional y el pronóstico.

Abstract: Introduction: The spine is the most common site for bone metastases; being the breast, prostate and lung cancer which have most affected. The thoracic spine is involved in 70% of cases, followed by the lumbar and cervical region. Material and methods: This is a 59 years old female diagnosed with breast adenocarcinoma and metastatic bone disease in cervical spine C2, C3 level and instability of that segment because of a compression fracture of C3 greater than 80% without invasion of the spinal canal, she begins with paresthesias of upper limbs. A 360º instrumentation was performed in two stages. After the surgical procedure the patient were without neck pain and a good neurological status. Discussion: Metastatic bone disease causes significant damage to the spine sometimes create instability proper instrumentation is needed to improve the functional status and prognosis of these lesions.

Metabolismo mineral en niños en diálisis peritoneal crónica / Mineral metabolism in patients on chronic peritoneal dialysis

Introduction: Children with chronic kidney disease (CKD) and receiving peritoneal dialysis (PD) have disorders of mineral metabolism that impact their growth, survival and cardiovascular functions. New molecular markers offer a better understanding of the pathophysiology of this disease. Objective: To characterize some components of mineral metabolism, with emphasis on FGF23/Klotho and cardiovascular functions (CV) of these patients. Patients and Method: Prospective observational cohort study. Exclusion criteria: serum 25 (OH) vitamin D < 20 ng/ml, peritonitis within the last two months and active nephrotic syndrome. Calcemia, phosphemia, parathyroid hormone (PTH), 25 (OH) vitD3, 1.25 (OH) vitD3, FGF23 and Klotho in plasma were measured. FGF23 and Klotho were quantified in healthy children as a control group. Echocardiography was performed calculating the left ventricular mass index (LVMI). Descriptive statistics analysis, Pearson correlation coefficient for association among variables and multivariate analysis were conducted. Results: 33 patients, 16 males, aged between 1.2 and 13.4 years were included. Age of onset for PD: 7.3 +/- 5.0 years, time receiving PD: 13.5 +/- 14.5 months. The plasma concentration of 25 (OH) vitD3 was 34.2 +/- 6.3 pg/ml. Calcemia and phosphemia values were 9.8 ± 0.71 and 5.4 +/- 1.0 mg/dl respectively. PTH was 333 +/- 287 pg/ml. FGF23 in plasma was 225.7 +/- 354.3 pg/ml and Klotho 131.6 +/- 72 pg/ml, and in the controls ( n = 16 ), it was 11.9 +/- 7.2 pg/ml and 320 +/- 119 pg/ml, respectively. The residual and total dose of dialysis (KtV) was 1.6 +/- 1.3 and 2.9 +/- 1.6, respectively. FGF23 levels significantly correlated with calcium (p < 0.001, r = 0.85), and inversely with residual KtV, showing no relationship with phosphemia. Klotho level correlated negatively with residual KtV and also, it showed a negative association with chronological age and age at onset of PD. LVMI > 38 g/m² was confirmed in 20/28 patients...

Introducción: Los niños portadores de Enfermedad renal crónica (ERC) en diálisis peritoneal (DP) presentan alteraciones del metabolismo mineral que afectan su crecimiento, estado cardiovascular y sobrevida. Nuevos marcadores moleculares representan una mejor comprensión de la fisiopatología de esta enfermedad. Objetivo: Caracterizar componentes del metabolismo mineral, con énfasis en FGF23/Klotho, y estado cardiovascular (CV) en este grupo de pacientes. Pacientes y Método: Estudio prospectivo observacional. Criterios de exclusión: niveles de 25 (OH) vitamina D < 20 ng/ml, peritonitis hasta 2 meses previos y síndrome nefrótico activo. Se midió calcemia, fosfemia, paratohormona (PTH), 25 (OH) vitD3, 1,25 (OH) vitD3, FGF23 y Klotho en plasma. Se cuantificó FGF23 y Klotho en niños sanos como grupo control. Se efectuó ecocardiografía, calculándose el índice de masa ventricular izquierda (IMVI). Se realizó análisis estadístico descriptivo, coeficiente de correlación de Pearson para asociación entre variables y análisis multivariado. Resultados: Se incluyeron 33 pacientes, 16 varones, edad 1,2 a 13,4 años. Edad de inicio de DP: 7,3 +/- 5,0 años, tiempo en DP: 13,5 +/- 14,5 meses. El nivel plasmático de 25 (OH) vitD3 fue 34,2 +/- 6,3 pg/ml. Los valores de calcemia y fosfemia fueron 9,8 +/- 0,71 y 5,4 +/- 1,0 mg/dl respectivamente. La PTH fue de 333 +/- 287 pg/ml. El FGF23 en plasma fue de 225,7 +/- 354,3 pg/ml y Klotho 131,6 +/- 72 pg/ml, y en los controles (n = 16) fue de 11,9 +/- 7,2 pg/ ml y 320 +/- 119 pg/ml, respectivamente. La dosis de diálisis (KtV) residual y total fue de 1,6 +/- 1,3 y 2,9 +/- 1.6, respectivamente. El nivel de FGF23 se correlacionó significativamente con la calcemia (p < 0,001, r = 0,85), e inversamente con el KtV residual, sin mostrar relación con la fosfemia. El nivel de Klotho...

The importance of bone biomarkers in the diagnosis of renal osteodystrophy / Importancia de los biomarcadores óseos en el diagnóstico de la osteodistrofia renal

OBJECTIVE: To evaluate the association of serum biochemical markers in patients with chronic kidney disease (CKD) in Jamaica for early detection of renal osteodystrophy (ROD). METHODS: The study contained two groups: CKD group (221) which consisted of adult patients, from dialysis units and renal clinics, with stage III to V CKD. The control group (23 7) had adult individuals, from the medical outpatient clinics, with mild and controlled chronic diseases and absence ofrenal failure. The patients in the study were between 18-80 years of age and gave informed consent to participate in the study. The differences in distribution of demographic, clinical and pathologic variables between the two groups were evaluated. Pearson's chi-squared test and Spearman' rho correlation coefficient test was used, with p < 0.01 considered statistically significant. Data analysis was conducted using the statistical package for the social sciences (SPSS) version 17.0. RESULTS: Among the 221 CKD patients in the study, 174 (78.7%) had ROD based on serum intact parathyroid hormone (iPTH) levels. The majority of patients in the control group did not have bone disease ie 95-96%. The majority of CKD patients (70.0%) had high-turnover (HTO) bone disease compared to 29.3% of patients with low-turnover (LTO) bone disease. Dialysis patients who had HTO bone disease compared with those with LTO had significantly higher levels of iPTH and total serum alkaline phosphatase (ALP). A similar relationship was observed among CKD patients not on dialysis. There was a significant individual variation in bone turnover biochemical markers. A total of237patients were recruited in the control group. Based on the levels of iPTH and tALP, six ofthem were found to have bone disease. The majority ofthese patients with bone disease were diabetic (83.3%) while the other patient had cancer (16.7%). The six patients in the control group with bone disease were within the age cohort of64-80 years, most ofwhom were 78 years old. CONCLUSION: A combination of serum biochemical markers might predict underlying renal osteodystrophy better that would individual biochemical markers. A predictive model using bone histology and biochemical markers can be developed in the future.

OBJETIVO: Evaluar la asociación de marcadores bioquímicos séricos en pacientes con la enfermedad renal crónica (ERC) en Jamaica, para la detección precoz de la osteodistrofia renal (ODR). MÉTODOS: El estudio comprendió dos grupos: un grupo ERC (221) formados por pacientes adultos, provenientes de las unidades de diálisis y las clínicas renales, y en las fases III a V de la ERC. El grupo control (237) estaba constituido por individuos adultos, provenientes de las clínicas ambulatorias médicas, con enfermedades crónicas moderadas y controladas, y sin insuficiencia renal. Los pacientes del estudio tenían edad que fluctuaban de 18 a 80 años de edad y dieron consentimiento informado para participar en el estudio. Se evaluaron las diferencias en la distribución de las variables demográficas, clínicas y patológicas entre los dos grupos. SE usaron la prueba de chi-cuadrado de Pearson y la prueba de coeficiente de correlación rho de Spearman, considerándose p < 0.01 estadísticamente significativa. El análisis de los datos se llevó a cabo usando paquete que usó el paquete estadístico para las ciencias sociales (SPSS) versión 17.0. RESULTADOS: De los 221 pacientes de ERC en el estudio, 174 (78.7%) tenían ODR, basado en los niveles de hormona paratiroidea intacta (PTHi) sérica. La mayor parte de los pacientes en el grupo de control, ie. 95-96%, no tenían enfermedades óseas. La mayoría de los pacientes con ERC (70.0%) presentaban la enfermedad de alto recambio óseo (ARO) en comparación con 29.3% de pacientes con la enfermedad de bajo recambio óseo (BRO). Los pacientes de diálisis con alto recambio óseo - comparados con los de bajo recambio óseo - tuvieron niveles significativamente más altos PTHi y fosfatasa alcalina sérica total (FASt). Una relación similar se observó entre pacientes de ERC sin tratamiento de diálisis. Hubo una variación individual significativa en los marcadores bioquímicos de recambio óseo. Un total de 237 pacientes fueron reclutados para el grupo de control. Sobre la base de los niveles de PTHi y FASt, se hallaron seis con la enfermedad ósea. La mayoría de estos pacientes con enfermedad ósea eran diabéticos (83.3%) mientras que el otro paciente tenía cáncer (16.7%). Los seis pacientes en el grupo de control con enfermedad ósea estaban dentro de la cohorte de 64-80 años de edad, en la que la mayoría tenía 78 años. CONCLUSIÓN: Una combinación de marcadores bioquímicos séricos podrían predecir una osteodistrofia renal subyacente mejor que los marcadores bioquímicos individuales. En el futuro puede desarrollarse un modelo de predicción que use marcadores tanto bioquímicos como histológicos del tejido óseo.

Osteólisis multicéntrica idiopática de tipo III: presentación de un caso clínico y caracterización del síndrome / Idiopathic multicentric osteolysis type III: case report and syndrome characterization

Presentamos el primer reporte de caso en Colombia de un paciente con osteólisis multicéntrica idiopática con compromiso de carpo y tarso y nefropatía. Se exponen, de manera clara y bien documentada, el cuadro clínico y paraclínico actual y la evolución del paciente, junto a una breve y completa revisión de esta enfermedad, rara, progresiva, degenerativa e incapacitante.

We report the first case in Colombia of a patient with idiopathic multicentric osteolysis with carpal-tarsal compromise and nephropathy, The current clinical and paraclinical status of the patient is clearly exposed and well documented, along with a brief and comprehensive review of this rare progressive, degenerative and disabling disease.