Osteoporosis transitoria de cadera familiar ligada al factor V de Leiden / Transient familial factor V Leiden-linked hip osteoporosis

INTRODUCCIÓN: La osteoporosis transitoria de cadera (OTC) es una enfermedad poco frecuente y de patogenia desconocida que ha sido considerada tradicionalmente una fase precoz y reversible de la necrosis avascular (NA). La trombofilia o la hipofibrinólisis familiar se considera un factor de riesgo para el desarrollo de NA y OTC. Dentro de los trastornos heredados de hipercoagulabilidad, el factor V de Leiden es uno de los más prevalentes. Casos clínicos: Estudio tipo serie de casos. Se describen el desarrollo y la evolución de 3 casos de OTC en 3 hermanos (2 varones y una mujer) con edades comprendidas entre los 40 y 43 años de forma consecutiva. El estudio clínico y de imagen con resonancia magnética nuclear (RM) confirmó el diagnóstico de OTC y descartó la presencia de NA. La mutación G1691A para el factor V de Leiden fue positiva en todos los casos. La evolución clínica y radiológica fue favorable, con curación sin secuelas y desaparición del edema óseo en la RM de control a los 6 meses en todos los casos. DISCUSIÓN: Los resultados del presente estudio apoyan la etiología isquémica y establecen a la OTC como una fase precoz y reversible de la NA de cadera. El factor V de Leiden promueve un estado de hipercoagulabilidad e hipofibrinólisis que favorece el desarrollo de la OTC por causa isquémica. CONCLUSIONES: El presente estudio desarrolla la primera descripción familiar de la OTC ligada al factor V de Leiden

INTRODUCTION: Transient osteoporosis of the hip (THO) is a rare disease of unknown pathogenesis that has traditionally been considered an early and reversible stage of avascular necrosis (AN). Thrombophilia or familial hypofibrinolysis is considered a risk factor for the development of AN and THO. Factor V Leiden is one of the most common hereditary hypercoagulability disorders. Clinical cases: Case series study. The development and course of 3 THO cases in 3 siblings (two males and one female) aged between 40 and 43 years are described consecutively. Clinical and nuclear magnetic resonance imaging (MRI) studies confirmed the diagnosis of THO and ruled out the presence of AN. The G1691A mutation of factor V Leiden was positive in all cases. The clinical and radiological outcome was favourable, with healing without sequelae and disappearance of bone oedema on control MRI at 6 months in all cases. DISCUSSION: The results of this study support the ischaemic aetiology and establish HTO as an early and reversable stage of hip AN. Factor V Leiden causes a state of hypercoagulability and hypofibrinolysis that encourages the development of THO due to ischaemic causes. CONCLUSIONS: This study outlines the first familiar description of factor V Leiden-linked THO

Transient Familial Factor V Leiden-Linked Hip Osteoporosis. / Osteoporosis transitoria de cadera familiar ligada al factor v de Leiden.

INTRODUCTION: Transient osteoporosis of the hip (THO) is a rare disease of unknown pathogenesis that has traditionally been considered an early and reversible stage of avascular necrosis (AN). Thrombophilia or familial hypofibrinolysis is considered a risk factor for the development of AN and THO. Factor V Leiden is one of the most common hereditary hypercoagulability disorders. CLINICAL CASES: Case series study. The development and course of 3THO cases in 3siblings (two males and one female) aged between 40 and 43 years are described consecutively. Clinical and nuclear magnetic resonance imaging (MRI) studies confirmed the diagnosis of THO and ruled out the presence of AN. The G1691A mutation of factor v Leiden was positive in all cases. The clinical and radiological outcome was favourable, with healing without sequelae and disappearance of bone oedema on control MRI at 6 months in all cases. DISCUSSION: The results of this study support the ischaemic aetiology and establish HTO as an early and reversable stage of hip AN. Factor V Leiden causes a state of hypercoagulability and hypofibrinolysis that encourages the development of THO due to ischaemic causes. CONCLUSIONS: This study outlines the first familiar description of factor v Leiden-linked THO.

ICU survival and need of renal replacement therapy with respect to AKI duration in critically ill patients.

BACKGROUND: Transient and persistent acute kidney injury (AKI) could share similar physiopathological mechanisms. The objective of our study was to assess prognostic impact of AKI duration on ICU mortality. DESIGN: Retrospective analysis of a prospective database via cause-specific model, with 28-day ICU mortality as primary end point, considering discharge alive as a competing event and taking into account time-dependent nature of renal recovery. Renal recovery was defined as a decrease of at least one KDIGO class compared to the previous day. SETTING: 23 French ICUs. PATIENTS: Patients of a French multicentric observational cohort were included if they suffered from AKI at ICU admission between 1996 and 2015. INTERVENTION: None. RESULTS: A total of 5242 patients were included. Initial severity according to KDIGO creatinine definition was AKI stage 1 for 2458 patients (46.89%), AKI stage 2 for 1181 (22.53%) and AKI stage 3 for 1603 (30.58%). Crude 28-day ICU mortality according to AKI severity was 22.74% (n = 559), 27.69% (n = 327) and 26.26% (n = 421), respectively. Renal recovery was experienced by 3085 patients (58.85%), and its rate was significantly different between AKI severity stages (P < 0.01). Twenty-eight-day ICU mortality was independently lower in patients experiencing renal recovery [CSHR 0.54 (95% CI 0.46-0.63), P < 0.01]. Lastly, RRT requirement was strongly associated with persistent AKI whichever threshold was chosen between day 2 and 7 to delineate transient from persistent AKI. CONCLUSIONS: Short-term renal recovery, according to several definitions, was independently associated with higher mortality and RRT requirement. Moreover, distinction between transient and persistent AKI is consequently a clinically relevant surrogate outcome variable for diagnostic testing in critically ill patients.

[Analysis of the 2015 British guidelines on the prevention and management of tumor lysis syndrome]. / Analyse des recommandations britanniques 2015 sur la prévention et la prise en charge du syndrome de lyse tumorale.

Whether it appears spontaneously or is induced by therapy, the tumor lysis syndrome is responsible for a massive release of ions and puric bases degradation of products in the circulation exceeding the renal excretion capacity. Some, such as uric acid, xanthine, and calcium phosphate, can precipitate in the renal tubules or parenchyma. It must be known to any practitioner supporting patients with hematologic malignancies, mainly high-grade but also some solid tumors. The 2015 publication of the British recommendations pertaining to patients suffering from hematological diseases should be broadcast. The main goal of treatment is to prevent the occurrence of renal dysfunction associated with heavy morbidity and mortality, either for his own conduct or consequences on obtaining a good tumor response. Some items proposed for the care, whether curative or preventive, should be discussed or detailed, which is the subject of this paper.

Novedades en cirugía de rodilla / What’s new in knee surgery

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Intolerancia a tornillo interferencial tibial. Una rara complicación en la Cirugía de reconstrucción del ligamento cruzado anterior / Intolerance to tibial fixation devices for ACL repair. A rare complication

Introducción: Se presenta una serie de casos de pacientes que han desarrollado bultoma, secreción y dolor pretibial tras la reconstrucción del LCA fijado con tornillos reabsorbibles (HA-PLLA [Hidroxiapatita- ácido L-poliláctico]). Método: Se revisan de manera retrospectiva todas las reconstrucciones de LCA realizadas entre 2008 y 2014 para identificar las complicaciones relacionadas con el sistema de fijación bioabsorbible. Durante ese periodo de 6 años se realizaron 620 reconstrucciones de LCA usando el tornillo bioabsorbible (HA-PLLA) para la fijación tibial del injerto tetrafascicular de isquiotibiales. Resultados: Nueve pacientes (1,45%) con un rango de edad de 29,8 (19-44 años), presentaron el periodo postoperatorio dolor, tumoración y secreción pretibial en la zona de inserción del tornillo. Todos presentaron marcadores inflamatorios normales. Todos los pacientes requirieron desbridamiento quirúrgico, y retirada de los restos del tornillo y de los tejidos reactivos. Se evidenció infección en 4 cultivos intraoperatorios. La anatomía patológica reveló restos detríticos birrefringentes en el citoplasma de los macrófagos. La retirada de los restos del tornillo y el desbridamiento y curetaje del túnel, supuso la recuperación completa de todos los pacientes de nuestra serie. Conclusiones: La tumoración pre-tibial como respuesta biológica adversa, debe considerarse como una posible complicación en la reconstrucción del LCA. Se aprecia una incidencia de 1,45% de reacción pretibial tras el uso de tornillos reabsorbibles (HA-PLLA) en tibia proximal en la reconstrucción de LCA

Background: We report a series of cases that presented as pre-tibial cyst, swelling and pain following anterior cruciate ligament (ACL) reconstruction using bioabsorble fixation devices (HA-PLLA). Methods: All ACL reconstructions were done between 2008 and 2014 reviewed retrospectively to identify complications related to bioabsorbable fixation devices. During this period of 6 years, 620 ACL reconstructions were performed using the bioabsorbable screw (HA-PLLA) for tibial fixation of the quadrupled hamstring autograft. Results: Nine patients (1,45%) with mean age of 29,8 (19-44) years, presented in the post-operative period, pre-tibial pain, cyst and swelling in tibial screw site. All of them had normal inflammatory markers. All of these patients underwent surgical debridement, which revealed remnants of screw and reactive material. There was evidence of infection in 4 intra-operative specimen cultures. Histopathology revealed detritic birefringent remains in macrophages cytoplasm. Removal of screw debris and curettage of the tunnel resulted in complete recovery of all patients in our series. Conclusions: Pre-tibial cyst as an adverse biological response should be considered as a possible complication in ACL reconstruction. We report a 1,45% of pre-tibial reaction in patients undergoing ACL reconstruction with bio-absorbable (HAPLLA) interference screw fixation for the proximal tibia

Thoracic involvement in systemic primary vasculitis: radiological patterns and follow-up.

Systemic primary vasculitides are rare idiopathic diseases causing an inflammatory injury to the vessel walls. A pulmonary involvement is frequent, and chest-CT is the imaging technique of reference in its assessment. An extremely wide variety of parenchymal, vascular and airways abnormalities, has been described and diagnosis can be challenging: knowledge of clinical data and a close cooperation with the referring physician is often crucial. The aim of this work is to describe the most common typical and atypical CT features of pulmonary vasculitis and their possible changes over time and therapy, focusing on the differential diagnosis with other inflammatory/infectious or neoplastic diseases.

Pacientes con fractura de pelvis inestables hemodinámicamente in extremis ¿packing pélvico o arteriografía? / Patients with hemodynamic unstable pelvic fractures in extremis: Pelvic packing or angiography?

El manejo multidisciplinar de los pacientes con traumatismo pélvico ha mejorado su pronóstico, sin embargo la mortalidad sigue siendo muy elevada. La estrategia de un tratamiento adecuado es aún controvertida, sobre todo respecto al control de la hemorragia en pacientes cuya situación clínica es extrema mediante el uso de arteriografía o packing pélvico. Planteamos mediante una herramienta de medicina basada en evidencia (CAT) el beneficio de la realización del packing pélvico en relación a una pregunta clínica específica desde una situación concreta, ¿qué es mejor para el manejo del sangrado, packing pélvico extraperitoneal o arteriografía, en pacientes con fractura de pelvis inestables hemodinámicamente in extremis? De este estudio podemos concluir que la arteriografía puede mejorar el control de la hemorragia en los pacientes con sangrado arterial y hemodinámicamente estables, pero el packing tiene prioridad en los pacientes con fracturas de pelvis e inestabilidad hemodinámica (AU)

The multidisciplinary management of patients with pelvic trauma has improved prognosis, but mortality is still very high. The appropriate treatment strategy remains controversial, especially regarding the control of bleeding in patients whose clinical situation is extreme by using angiography or pelvic packing. We propose using a tool of evidence-based medicine (CAT) the benefit of the completion of pelvic packing in relation to a specific clinical question from a specific situation. What is best for the management of bleeding, extraperitoneal pelvic packing or angiography, in patients with hemodynamically unstable pelvic fracture in extremis? From this study we can conclude that angiography may improve control of bleeding in patients with arterial bleeding and hemodynamically stable but the packing has priority in patients with pelvic fractures and hemodynamic instability (AU)

[Patients with hemodynamic unstable pelvic fractures in extremis: pelvic packing or angiography?]. / Pacientes con fractura de pelvis inestables hemodinámicamente in extremis ¿packing pélvico o arteriografía?

The multidisciplinary management of patients with pelvic trauma has improved prognosis, but mortality is still very high. The appropriate treatment strategy remains controversial, especially regarding the control of bleeding in patients whose clinical situation is extreme by using angiography or pelvic packing. We propose using a tool of evidence-based medicine (CAT) the benefit of the completion of pelvic packing in relation to a specific clinical question from a specific situation. What is best for the management of bleeding, extraperitoneal pelvic packing or angiography, in patients with hemodynamically unstable pelvic fracture in extremis? From this study we can conclude that angiography may improve control of bleeding in patients with arterial bleeding and hemodynamically stable but the packing has priority in patients with pelvic fractures and hemodynamic instability.

[Focusing on peritoneal dialysis adequacy]. / Adéquation en dialyse péritonéale : mise au point.

The optimal method to assess the adequacy of peritoneal dialysis therapies is controversial. Today, the adequacy must not be considered as a number or a concept assessed only by two parameters (total KT/V urea and total solute clearance) but defined by many more items. In the absence of data, based on theoretical considerations, the reanalysis of the CANUSA study showed that renal kidney function, rather than peritoneal clearance, was associated with improved survival. Residual renal function is considered as a major predictor factor of cardiovascular mortality. Results of this reanalysis were supported by the adequacy data in ADEMEX, EAPOS and ANZDATA studies. Therefore, clinical assessment plays a major role in PD adequacy. The management of fluid balance, the regular monitoring of malnutrition, the control of mineral metabolism and particularly the glucose load, considered as the "corner-stone" of the system, are the main points to be considered in the adequacy of PD patients. The essential goal is to minimize glucose load by glucose-sparing strategies in order to reduce the neoangiogenesis of the peritoneal membrane.

Secuela de fractura de pelvis. A propósito de un caso y revisión de la literatura / Pelvis fracture sequelae. A case report and literature review

Objetivos: Presentación y análisis crítico de paciente con secuela post-fractura de pelvis que permita exponer la complejidad de su manejo y tratamiento. Caso clínico: Mujer de 29 años con dolor invalidante en región sacroilíaca a la sedestación y la marcha. Exploración: Deformidad externa pélvica con repercusión estética, dismetría de miembro inferior izquierdo de 4 cm y dolor sacroilíaco a la apertura pélvica o al apoyo monopodal izquierdo. Limitación funcional a la marcha con cojera y problemas de sedestación (sitting imbalance). Estudio radiográfico convencional y TAC con signos de asimetría pélvica, elevación de hemipelvis izquierda y pseudoartrosis sacra y de ramas ilioisquiopubianas izquierdas post-fractura. Diagnóstico: Secuela compleja de Fractura de Pelvis con deformidad, tipo III de Mears y Velyvis. Tratamiento: Secuenciado en 2 fases. (1) Liberación Anterior: Desarticulación sinfisaria, esqueletización de ramas y osteotomía sacra anterior. Liberación Posterior: Liberación ligamentos suelo pélvico y osteotomía sacra posterior. Tracción progresiva de la extremidad izquierda 2 semanas. (2) Osteosíntesis Anterior: Fijación sacroilíaca anterior con placa de reconstrucción, doble placa anterior sinfisaria y aporte de autoinjerto. Osteosíntesis Posterior: Fijación posterior mediante barra transilíaca. Evolución: Infección herida quirúrica posterior que se resolvió con curas quirúrgicas y antibioterapia. Se consiguió disminución del nivel de dolor, resolución del trastorno de sedestación y mejora de la cojera. SF-36 con PCS 60 y MCS de 50, Majeed-Score excelente. Discusión: La pseudoartrosis y la deformidad son entidades infrecuentes generadas por tratamientos conservadores o inadecuados en lesiones con gran inestabilidad pélvica. El tratamiento puede ser realizado de forma directa o en varias fases. Los pacientes deben ser informados de que el tratamiento implica riesgos más elevados que respecto a la cirugía aguda convencional. Suele obtener mejoras en resultados de consolidación y funcionalidad. El paciente aquí referido ha sido tratado de forma análoga a lo establecido por la comunidad científica, a pesar de ello, no existe evidencia clínica respecto a protocolos de manejo en dicha enfermedad. La realización de estudios multicéntricos ayudaría a la comprensión de los patrones lesionales y al desarrollo de herramientas estandarizadas de tratamiento. Conclusión: La secuela de la fractura pélvica es una entidad compleja de dificultosa resolución y difícil manejo incluso para expertos en dicha materia. Es preciso un abordaje multidisciplinar de dichos paciente en centros de tercer nivel para la obtención de los mejores resultados en esta patología (AU)

Objectives: Critical assessment of a case report related to complications in pelvis fracture. The aim is to determine the complexity in management and treatment of pelvic sequelae. Case Report: 29 years old woman with disabling pain in sacroiliac joint when sitting and walking. External pelvic deformity with aesthetic evidence, left lower limb with 4 cm dysmetria and sacroiliac pain pelvic when examination maneuvers opening pelvis or standing on one foot. Functional limitation with sitting and gait problems. Conventional X-rays and CT-scan reveal pelvic asymmetry, upper left hemipelvis and nonunions in sacrum and left ilioischiopubic ramus fractures. Diagnosis: Pelvic fracture complication Velyvis and Mears type III. A two phases sequenced treatment was performed: (1) Anterior Release: Symphyseal disjoint, branches skeletonization and anterior sacral osteotomy. Posterior Release: Posterior sacroiliac complex and pelvic floor ligaments release with posterior sacral osteotomy. Progressive left extremity traction 2 weeks. (2) Anterior Osteosynthesis: Anterior sacroiliac fixation with reconstruction plate, double symphyseal plate and patient bonegrafting. Posterior Osteosynthesis: Transiliac bar. Outcome with surgical wound infection solved with antibiotics and surgical treatment. A pain decrease, sitting disorder resolution and improvement of lameness was achieved. SF-36 with 60 PCS and 50 MCS. Excelent Majeed-Score. Discussion: Pelvic malalignement and nonunions are rare entities, usually associated with initial conservative or inadequate treatment in pelvic instable fractures. Treatment may be accomplished directly or in stages. Patients should be informed that treatment involves higher risks than over conventional acute surgery. It often obtains improved consolidation and functionality results. The patient here referred has been treated similarly to that established by the scientific community, nevertheless, there is no clinical evidence regarding management protocols in the disease. Multicenter studies would help the understanding about patterns and development of standardized treatment algorithms. Conclusion: Pelvic fracture complications are complex entities. It involves a hard resolution and difficult management even for expert surgeons. A multidisciplinary approach of these patients in tertiary centers is needed to obtain the best results in this pathology (AU)

Study of prone positioning to reduce ventilator-associated pneumonia in hypoxaemic patients.

The aim of the present study was to examine whether prone positioning (PP) affects ventilator associated-pneumonia (VAP) and mortality in patients with acute lung injury/adult respiratory distress syndrome. 2,409 prospectively included patients were admitted over 9 yrs (2000-2008) to 12 French intensive care units (ICUs) (OUTCOMEREA). The patients required invasive mechanical ventilation (MV) and had arterial oxygen tension/inspiratory oxygen fraction ratios <300 during the first 48 h. Controls were matched to PP patients on the PP propensity score (+/-10%), MV duration longer than that in PP patients before the first turn prone, and centre. VAP incidence was similar in the PP and control groups (24 versus 13 episodes.1,000 patient-days MV(-1) respectively, p = 0.14). After adjustment, PP did not decrease VAP occurrence (HR 1.64 (95% CI 0.70-3.84); p = 0.25) but significantly delayed hospital mortality (HR 0.56 (95% CI 0.39-0.79); p = 0.001), without decreasing 28-day mortality (37% in both groups). Post hoc analyses indicated that PP did not protect against VAP but, when used for >1 day, might decrease mortality and benefit the sickest patients (Simplified Acute Physiology Score >50). In ICU patients with hypoxaemic acute respiratory failure, PP had no effect on the risk of VAP. PP delayed mortality without decreasing 28-day mortality. PP >1 day might decrease mortality, particularly in the sickest patients.

[Surgery to save body-packers]. / La chirurgie au secours des body-packers.

Cocaine body packing may expose to the risk of intestinal obstruction or acute cocaine poisoning. During the last five years (1998-2002), 17 symptomatic body-packers were admitted to our intensive care unit, for a closed monitoring. Clinical evolution of three patients required a surgical intervention. The first patient had a laparotomy, in the presence of an occlusive syndrome, to recover the packets of cocaine. The second patient presented peritonitis, in relation to an ileal perforation, treated with an intestinal segment resection followed by a double ileostomy. The third patient presented severe ventricular dysrhythmia, due to cocaine toxicity, after a bullet disruption in the stomach. Adrenaline and labetalol-combined therapy as well as gastric incision to recover the bullets were needed to allow cardiovascular stabilization. In these three cases, the surgical treatment allowed patient survival.

A continuous series of 96 laparoscopic inguinal hernia repairs in children by a new technique.

BACKGROUND: The aim of this study was evaluate a novel technique of laparoscopic hernia repair in children. METHODS: Eighty two consecutive patients aged >or=2 years old were operated on for hernia repair using a laparoscopic technique that reproduced every step of the conventional open procedure. Technical details and clinical results are reported. RESULTS: Ninety six sacs were divided in 82 patients (15 girls and 67 boys). Fourteen bilateral hernias that had been diagnosed before operation in nine cases and during laparoscopy in five cases were repaired. Nine controplateral dimples were left and not repaired. Mean operative time was 23 min in girls and 28 min in boys for unilateral hernias, and 30 min in girls and 40 min in boys for bilateral hernias. Sixty-seven of the children were followed up 6 months later. None of them suffered recurrences. No unsightly scars were observed at the port sites. Discomfort did not extend beyond 48 h after the operation, enabling a rapid return to normal activities. CONCLUSION: Dividing the sac and suturing the peritoneum is feasible and efficient by laparoscopy. Compared with the other techniques that have previously been reported, either without any dissection of the sac or any ligature, our technique seems to be advantageous. It is not time consuming and does not require any special laparoscopic skill.

[Chloroquine cardiomyopathy revealed by complete atrio-ventricular block. A case report]. / Cardiomyopathie à la chloroquine révélée par un bloc auriculo-ventriculaire complet. A propos d'une observation.

We describe a rare case of chloroquine cardiomyopathy occurring during long term (7 years) treatment for rheumatoid polyarthritis in a 42 year old woman. There was an isolated acute severe conduction defect, which is particularly rare. Histological study with the electron microscope allowed confirmation of this diagnosis. We report here the secondary cardiological effects of this frequently used synthetic antimalarial.

Trends in opiate and opioid poisonings in addicts in north-east Paris and suburbs, 1995-99.

AIMS: (1). To assess the trends in the number, mortality and the nature of severe opiate/opioid poisonings from 1995 to 1999 in north-east Paris and adjacent suburbs and (2). to examine the effects of the introduction of high-dose buprenorphine on these parameters. DESIGN: Retrospective, 5-year study with review of pre-hospital, hospital and post-mortem data. SETTING AND PARTICIPANTS: Eighty patients from the toxicological intensive care unit (TICU) in north-east Paris, 421 patients from the pre-hospital emergency medical service in a north-east suburb of Paris (SAMU 93) and 40 deaths from the coroner's office in Paris. MEASUREMENTS AND RESULTS: We found that the number of pre-hospital opiate/opioid poisonings and deaths decreased over 5 years. During the same time frame, opiate/opioid poisoning admissions to our TICU remained steady, but the number of deaths declined. From 1995 to 1999, the detection of buprenorphine among opiate/opioid-poisoned TICU patients increased from two to eight occurrences per year while detection of opiates diminished from 17 to 10 occurrences per year. Increased buprenorphine detection correlated directly with increasing sales over this time period. In spite of the increased use of buprenorphine, the mortality associated with opiate/opioid poisonings has diminished in the pre-hospital environment from 9% in 1995 to 0% in 1999, and in the TICU from 12% in 1995 to 0% in 1997 and thereafter. We found a high frequency of multiple opiate/opioid use in severe poisonings, as well as the frequent association of other psychoactive drugs including ethanol. CONCLUSIONS: The number and the mortality of opiate/opioid poisonings appear to be stable or decreasing in our region. The association of multiple opiates/ opioids appears nearly as common as the association with other psychoactive drugs. The introduction of high-dose buprenorphine coincides with a decrease in opiate/opioid poisoning mortality. Further study will be necessary to clarify this observation.