Evaluation of cognitive impairment in a French sample of patients with restrictive anorexia nervosa: two distinct profiles emerged with differences in impaired functions and psychopathological symptoms.

PURPOSE: The cognitive profiles of patients suffering from anorexia nervosa (AN) are currently explored as potential facilitating and/or maintenance factors. Specific data in restrictive AN (AN-R) remain contradictory. This study focused on women with AN-R to evaluate their cognitive functions to develop a more specific cognitive remediation program. METHODS: Female patients older than 15 years who were suffering from AN-R were recruited in a specialized unit for eating disorder management. Female healthy control (HC) participants were recruited who were matched with AN patients on age. All participants completed a cognitive evaluation (premorbid intelligence quotient (IQ), planning, information processing speed, cognitive flexibility) and a clinical evaluation (impulsivity, anxiety, depression). RESULTS: A total of 122 participants were included. Patients suffering from AN-R had significant impairment in information processing speed and planning. Patients exhibited significantly better cognitive flexibility than did the HC group when adjustments were made for other cognitive functions and impulsivity. Two distinct subgroups of patients were identified. The first subgroup had more marked cognitive impairment and fewer psychopathological symptoms than did the second subgroup of patients and the HC group. CONCLUSION: Our results highlight cognitive impairment in patients with AN who had normal premorbid IQ. Two distinct profiles emerge. In clinical practice, these results open up perspectives for the development of more specific cognitive remediation programs (one specific program for cold cognitions and another specific program targeting emotions and hot cognitions). These results warrant confirmation by larger studies with a more specific evaluation of the impact of emotional status. Trial registration NTC02381639, Date of registration. March 6, 2015.

[Impact of partial nephrectomy in a hybrid operating room on the activity of kidney cancer surgery]. / Évaluation de l'utilisation de la salle hybride sur l'activité de chirurgie en cancérologie rénale.

INTRODUCTION: Partial nephrectomy (NP) after embolization of tumor vessels (NPESH) in a hybrid room combines embolization of tumor vessels and enucleation of the tumor under laparoscopy in the same operative time. The purpose of this study was to assess the impact of the use of NPESH in the management of patients treated with surgery for a localized kidney tumor. MATERIAL AND METHODS: Using the uroCCR database, we included all consecutive patients operated in a university hospital for localized kidney tumor. From 2011 to May 2015, patients were treated by Standard Partial Nephrectomy (NPS) Laparoscopic or Open and from May 2015 to May 2019 by NPESH. We evaluated characteristics of patients, tumors, perioperative data and complications. These data were compared by Student and Khi2 tests. RESULTS: 87 NPS were performed during Period 1 and 137 NPS were performed during period 2. The ASA score of patients undergoing NPESH was higher than NPS (P<0.0001). The tumor complexity and median tumor size were similar in the two groups (P=0.852 and P=0.48). The complication rate for NPS and NPESH was 55.2% and 33.6% (P=0.002). There were less severe complications in the NEPSH group (P=0.012). The median length of stay was 8 and 4 days for the NPS and NPESH groups (P<0.0001). Positive surgical margins were 2 (2.3%) and 6 (4.6%) for the NPS and NPESH group (P=0.713). DISCUSSION: NPESH is an efficient technique compared to NPS. It seems to be an interesting alternative to limit renal ischemia, complication rate and length of stay for the management of localized kidney tumors.

Screening for therapeutic trials and treatment indication in clinical practice: MACK-3, a new blood test for the diagnosis of fibrotic NASH.

BACKGROUND: The composite histological endpoint comprising nonalcoholic steatohepatitis (NASH) and NAFLD activity score ≥4 and advanced fibrosis (F ≥ 2) ("fibrotic NASH") is becoming an important diagnostic target in NAFLD: it is currently used to select patients for inclusion in phase III therapeutic trials and will ultimately be used to indicate treatment in clinical practice once the new drugs are approved. AIM: To develop a new blood test specifically dedicated for this new diagnostic target of interest. METHODS: Eight Hundred and forty-six biopsy-proven NAFLD patients from three centres (Angers, Nice, Antwerp) were randomised into derivation and validation sets. RESULTS: The blood fibrosis tests BARD, NFS and FIB4 had poor accuracy for fibrotic NASH with respective AUROC: 0.566 ± 0.023, 0.654 ± 0.023, 0.732 ± 0.021. In the derivation set, fibrotic NASH was independently predicted by AST, HOMA and CK18; all three were combined in the new blood test MACK-3 (hoMa, Ast, CK18) for which 90% sensitivity and 95% specificity cut-offs were calculated. In the validation set, MACK-3 had a significantly higher AUROC (0.847 ± 0.030, P ≤ 0.002) than blood fibrosis tests. Using liver biopsy in the grey zone between the two cut-offs (36.0% of the patients), MACK-3 provided excellent accuracy for the diagnosis of fibrotic NASH with 93.3% well-classified patients, sensitivity: 90.0%, specificity: 94.2%, positive predictive value: 81.8% and negative predictive value: 97.0%. CONCLUSION: The new blood test MACK-3 accurately diagnoses fibrotic NASH. This new test will facilitate patient screening and inclusion in NAFLD therapeutic trials and will enable the identification of patients who will benefit from the treatments once approved.

Cult membership: What factors contribute to joining or leaving?

We assumed that, as in the case of addiction disorders, former cult members exhibit vulnerability and protective factors for cult commitment and membership. Thus, the aim of our study was to identify vulnerability factors that are involved in the commitment and in the retention in the group, as well as protective factors that are involved in the departure. We interviewed 31 former cult members, using semi-structured interviews to evaluate their clinical profile, characteristics of the cultic group and their experience in the group. Cult membership and addictive disorders share some characteristics: persistence despite damage, initial psychological relief, occupation of an exclusive place in the thoughts of members, high psychiatric comorbidity prevalence, high accessibility, leading to social precariousness and the importance of familial support when leaving. Three main axes of improvement were highlighted: regulations concerning cults in order to limit their social presence, which appears to be a vulnerability factor for commitment; social and therapeutic follow-up when a member leaves a group so that social precariousness does not become an obstacle to departure; and familial support to maintain a link with the member, as the intervention of a person from outside of the group is an important protective factor for leaving.

Disordered eating in French high-level athletes: association with type of sport, doping behavior, and psychological features.

PURPOSE: Over the last few years, disordered eating in athletes has received increasing attention. According to several studies, athletes could be more vulnerable to disordered eating and some characteristics specific to the athletic community could be in favour of an increased risk of poor body image and disturbed eating habits in athletes. However, the literature is sparse and some methodological issues in studies have been pointed out. In this context, we aimed at determining the prevalence of disordered eating in French high-level athletes using clinical interviews of three different clinicians and identifying what are the factors associated with disordered eating in athletes. METHODS: In France, all athletes registered on the French high-level list have to undergo a yearly evaluation. Data collected during the somatic assessment, the dietary consultation, and the psychological of the yearly evaluation were used. Multivariate analysis was performed for identification of factors associated with disordered eating. RESULTS: Out of the 340 athletes included, 32.9% have been detected with a disordered eating. They were difficult to detect by clinicians, as usual criteria did not seem to be reliable for athletes. Competing in sports emphasizing leanness or low body weight was associated with disordered eating; however, gender was not. CONCLUSION: These results highlight the need for the development of specific screening tools for high-level athletes. Furthermore, the identification of factors associated with disordered eating could improve early detection and prevention program effectiveness.

Circulating Cd34+ cell count differentiates primary myelofibrosis from other Philadelphia-negative myeloproliferative neoplasms: a pragmatic study.

A high number of circulating CD34+ cells has been advocated to distinguish primary myelofibrosis from other Philadelphia-negative myeloproliferative neoplasms. We re-evaluated the diagnostic interest of measuring circulating CD34+ cells in 26 healthy volunteers and 256 consecutive patients at diagnosis for whom a myeloproliferative neoplasm was suspected. The ROC curve analysis showed that a number of CD34+ <10/µl excludes the diagnosis of primary myelofibrosis with a sensitivity of 97 % and a specificity of 90 % (area under the curve: 0.93 [0.89-0.98]; p < 0.001). Patients with PMF harboring a CALR mutation had more circulating CD34+ cells than patients with either a JAK 2 or MPL mutation (p = 0.02 and p < 0.01, respectively). These results suggest that this fast, simple, non-invasive, and standardized test is of particular interest to exclude the diagnosis of primary myelofibrosis.

Post-treatment MRI aspects of photodynamic therapy for prostate cancer.

OBJECTIVES: Photodynamic therapy is a new focal therapy for prostate cancer. METHODS: In this technique, a photosensitising agent is introduced intravenously, then activated by local laser illumination to induce tumour necrosis. Treatment efficacy is assessed by magnetic resonance imaging (MRI). RESULTS AND CONCLUSIONS: We illustrate specific post-treatment MRI aspects at early and late follow-up with pathological correlations. TEACHING POINTS: • Dynamic phototherapy is a new and promising focal therapy for prostate cancer. • One-week MRI shows increased volume of the treated lobe and large, homogeneous necrosis area. • Six-month MRI shows significant changes of the prostate shape and signal. • Six-month MRI becomes "base line" appearance for further follow-up or monitoring.

Combination of blood tests for significant fibrosis and cirrhosis improves the assessment of liver-prognosis in chronic hepatitis C.

BACKGROUND: Recent longitudinal studies have emphasised the prognostic value of noninvasive tests of liver fibrosis and cross-sectional studies have shown their combination significantly improves diagnostic accuracy. AIM: To compare the prognostic accuracy of six blood fibrosis tests and liver biopsy, and evaluate if test combination improves the liver-prognosis assessment in chronic hepatitis C (CHC). METHODS: A total of 373 patients with compensated CHC, liver biopsy (Metavir F) and blood tests targeting fibrosis (APRI, FIB4, Fibrotest, Hepascore, FibroMeter) or cirrhosis (CirrhoMeter) were included. Significant liver-related events (SLRE) and liver-related deaths were recorded during follow-up (started the day of biopsy). RESULTS: During the median follow-up of 9.5 years (3508 person-years), 47 patients had a SLRE and 23 patients died from liver-related causes. For the prediction of first SLRE, most blood tests allowed higher prognostication than Metavir F [Harrell C-index: 0.811 (95% CI: 0.751-0.868)] with a significant increase for FIB4: 0.879 [0.832-0.919] (P = 0.002), FibroMeter: 0.870 [0.812-0.922] (P = 0.005) and APRI: 0.861 [0.813-0.902] (P = 0.039). Multivariate analysis identified FibroMeter, CirrhoMeter and sustained viral response as independent predictors of first SLRE. CirrhoMeter was the only independent predictor of liver-related death. The combination of FibroMeter and CirrhoMeter classifications into a new FM/CM classification improved the liver-prognosis assessment compared to Metavir F staging or single tests by identifying five subgroups of patients with significantly different prognoses. CONCLUSIONS: Some blood fibrosis tests are more accurate than liver biopsy for determining liver prognosis in CHC. A new combination of two complementary blood tests, one targeted for fibrosis and the other for cirrhosis, optimises assessment of liver-prognosis.

Anomalías cromosómicas subteloméricas en pacientes con retraso mental criptogénico / Subtelomeric rearrangements in cryptogenic mental retardation

Introducción: El retraso mental afecta al 3% de la población. En el 50% no es posible determinar la etiología. Las alteraciones cromosómicas submicroscópicas subteloméricas, no detectables con técnicas citogenéticas convencionales, pueden explicar algunos casos de retraso mental criptogénicos. Pacientes y métodos: Cohorte de 200 pacientes, con edades comprendidas entre los 2,5 y los 15 años, y retraso psicomotor (< 6 años) o retraso mental (> 6 años) criptogénicos. Variables: grado de retraso, dismorfias (faciales, manuales, macrosomía/microsomía), crecimiento intrauterino retardado, epilepsia. Identificación de reordenamientos cromosómicos subteloméricos mediante MLPA (multiplex ligation dependent probe amplification), que detecta pérdidas o ganancias de material genético. Confirmación de los hallazgos patológicos mediante FISH (fluorescent in situ hybridization) y/o array de CGH (comparative genomic hybridization). Resultados: Se detectaron anomalías subteloméricas en 9 pacientes, lo que representa el 4,5% de los casos. El estudio de progenitores demostró en un caso una traslocación en equilibrio. El resto eran alteraciones «de novo». Existía asociación significativa con la presencia de más de un rasgo fenotípico dismórfico o el antecedente de crecimiento intrauterino retardado, pero no con el grado de retraso ni con la presencia de epilepsia. Conclusiones: Las alteraciones cromósomicas submicroscópicas subteloméricas explican el 4,5% de los retrasos mentales de causa desconocida en nuestra serie. En nuestra población se asocian a la presencia de más de un rasgo fenotípico anormal o al antecedente de crecimiento intrauterino retardado (AU)

Introduction: Mental retardation affects 3% of the population, the origin of which cannot be established in 50% of cases. Subtelomeric rearrangements, not detected by routine cytogenetic studies, might explain some cases of unknown cause. Patients and methods: A study was conducted on 200 subjects with unexplained mental retardations using multiplex ligation dependent probe amplification (MLPA). Abnormal findings were confirmed by fluorescent in situ hybridization (FISH) and/or comparative genomic hybridization technology (CGH-array). Results: A subtelomeric aberration was identified in 9 patients. Eight were «de novo»; one was inherited from a phenotypically normal parent. There was a statistically significant association with the presence of more than one dysmorphic feature or with intrauterine growth retardation, but not with the severity of retardation or epilepsy. Conclusions: Subtelomeric rearrangements explained 4.5% of cases of mental retardation in our series. The presence of more than one dysmorphic feature or intrauterine uterine growth retardation increases the probability of this type of chromosomal aberration (AU)

[Pulmonary Langerhans histiocytosis and Hodgkin's lymphoma]. / Histiocytose langerhansienne pulmonaire et lymphome de Hodgkin.

Pulmonary Langerhans histiocytosis (PLH) is a rare disease due to the accumulation of Langerhans cells at the level of the bronchioles. These dendritic immunocytes form granulomata and destroy the wall of the airway. We report a case of PLH developing at the same time as Hodgkin's lymphoma in a young woman who smoked tobacco and cannabis. We observed a complete remission of the PLH lesions parallel to the remission of the Hodgkin's lymphoma after chemotherapy, in the absence of any change in the consumption of tobacco and cannabis. This observation leads us to discuss the potential relationships between PLH on one hand, and smoking, the lymphoma and its treatment on the other.

[Subtelomeric rearrangements in cryptogenic mental retardation]. / Anomalías cromosómicas subteloméricas en pacientes con retraso mental criptogénico.

INTRODUCTION: Mental retardation affects 3% of the population, the origin of which cannot be established in 50% of cases. Subtelomeric rearrangements, not detected by routine cytogenetic studies, might explain some cases of unknown cause. PATIENTS AND METHODS: A study was conducted on 200 subjects with unexplained mental retardations using multiplex ligation dependent probe amplification (MLPA). Abnormal findings were confirmed by fluorescent in situ hybridization (FISH) and/or comparative genomic hybridization technology (CGH-array). RESULTS: A subtelomeric aberration was identified in 9 patients. Eight were «de novo¼; one was inherited from a phenotypically normal parent. There was a statistically significant association with the presence of more than one dysmorphic feature or with intrauterine growth retardation, but not with the severity of retardation or epilepsy. CONCLUSIONS: Subtelomeric rearrangements explained 4.5% of cases of mental retardation in our series. The presence of more than one dysmorphic feature or intrauterine uterine growth retardation increases the probability of this type of chromosomal aberration.

[Tuberculosis with a hemophagocytic syndrome]. / Tuberculose compliquée d'un syndrome hémophagocytaire ou syndrome d'activation macrophagique.

INTRODUCTION: The hemophagocytic syndrome is rare and sometimes associated with tuberculosis. OBSERVATION: We report the case of a 30-year-old migrant from Congo presenting with a recurrent right pleuropneumonia, cachexia and night sweat. Blood tests revealed bicytopenia with a normal myelogram. Thorax CT-scan showed large mediastinal lymph nodes and pleuritis. Mediastinal lymph node biopsy concluded to granulomatosis lymphadenopathy with necrosis and bone marrow biopsy suggested hemophagocytic syndrome. The outcome was favorable with antibiotics and corticosteroids.

Treatment of liver fibrosis: clinical aspects.

The main objective of antifibrotic treatment is to avoid the complications of chronic liver disease where its cause cannot be treated. Three main therapeutic endpoints can be targeted: cause; comorbidity; and fibrosis. Antifibrotic treatment is any intervention independent of cause that is intended to modify the course and/or level of fibrosis through direct action on the mechanisms of fibrosis. Several modalities are here considered: reduction of fibrosis course; reversion of fibrosis; and reversion of cirrhosis. Semiquantitative histological staging and morphometry are complementary techniques for monitoring fibrosis. The degree of fibrosis should preferentially be estimated by fibrosis progression based on measurements taken at baseline and during treatment, rather than by raw static measurements. Surrogate markers are the only tools for assessing drug efficacy in clinical practice, and are especially useful for checking compliance and identifying poor or non-responders. We propose to define non-response as no decrease in fibrosis progression. The renin-angiotensin system is a good candidate target for antifibrotic treatment, and angiotensin-II type-1 receptor blockers, such as sartans, are probably effective. Clinical trials are currently ongoing using marketed drugs, while new multitargeted drugs are likely to emerge from basic research.

FibroMeters: a family of blood tests for liver fibrosis.

FibroMeters are blood tests for liver fibrosis with several specificities: two main diagnostic targets (fibrosis stage and area of fibrosis); adaptation to specific causes; and results confirmed by an expert system. Thus, FibroMeters comprise six different tests: one for staging and one for quantitation of liver fibrosis in each of the three main causes of chronic liver disease-chronic viral hepatitis, alcoholic liver disease (ALD) and non-alcoholic fatty liver disease (NAFLD). FibroMeters display a high overall diagnostic accuracy and are the only tests to correctly classify 100% of HCV patients without fibrosis or with cirrhosis. They have 90% predictive values in a higher proportion of patients than with other usual blood tests. A 90% correct classification is available in 100% of HCV patients with the following reliable diagnostic intervals: F0/1, F1/2, F2+/-1, F3+/-1. In real-life conditions, the reproducibility of FibroMeters is higher than that of liver biopsy or ultrasonographic elastometry. FibroMeters are robust tests with the most stable diagnostic performance across different centers. Optional tests are also available, such as a specific one for cirrhosis, which has a diagnostic accuracy of 93.0% (AUROC: 0.92) and a 100% positive predictive value for diagnosis of HCV cirrhosis. Determination by FibroMeters of the area of fibrosis - the only direct, non-invasive, quantitative measurement of liver fibrosis - are especially useful for following-up cirrhosis as it correlates well with clinical events. FibroMeters are also very accurate in HVB or HIV-HCV co-infected patients. The tests specific for ALD and NAFLD also have a high diagnostic accuracy (AUROCs: 0.96 and 0.94, respectively, for significant fibrosis).

Evaluation and improvement of a reliable diagnosis of cirrhosis by blood tests.

OBJECTIVE: To evaluate the rates of reliable diagnosis of cirrhosis by two usual blood tests. METHODS: Reliable diagnosis was mainly evaluated by comparing rates of positive (PPV) and negative (NPV) predictive values with FibroTest and FibroMeters, as either standard test or specifically designed for cirrhosis, in 1056 patients with chronic hepatitis C. RESULTS: Using the diagnostic limits provided by fibrosis stage scales, the PPV for cirrhosis was: standard FibroMeters: 68.5% versus FibroTest: 37.1%. Using 95% PPV, the cirrhosis detection rate was: specific FibroMeter: 26.1% versus FibroTest: 2.0% (P<10(-3)). The cirrhosis detection rate increased from 26 to 65% by performing liver biopsy in 8% of patients with indeterminate results on specific FibroMeter between 95% NPV and PPV. On the other hand, specific FibroMeter provided three intervals of 95% reliable diagnosis with no biopsy: less than or equal to 95% NPV: no cirrhosis (threshold: diagnosis); significant fibrosis; and greater than or equal to 95% PPV: cirrhosis. CONCLUSION: The detection rate and PPV for cirrhosis using fibrosis scales were fair for standard FibroMeter and poor for FibroTest. Around one-fourth of cases of cirrhosis are detected by the 95% PPV of specific FibroMeter, and around two-thirds by performing an additional liver biopsy in only 8% of patients. Finally, specific FibroMeter can avoid liver biopsy by classifying patients into three categories: no cirrhosis; significant fibrosis; and cirrhosis.

Fibrinogen angers with a new deletion (gamma GVYYQ 346-350) causes hypofibrinogenemia with hepatic storage.

INTRODUCTION: This study reports a family with chronically abnormal blood liver function tests (LFT) and congenital hypofibrinogenemia. The proposita had cirrhosis initially related to alcohol abuse and chronic viral hepatitis C (HCV), but abnormal LFT persisted even when alcohol intake was stopped and despite HCV treatment was efficient based on serum RNA negative testing. RESULTS: Needle biopsy specimens of the proposita and her brother showed eosinophilic intra-cytoplasmic inclusions that reacted strongly with fibrinogen antisera on direct immunofluorescence. Electron microscopic examination showed that the rough endoplasmic reticulum was filled with inclusions that consisted of densely packed, curved tubular structures arranged in a fingerprint-like pattern. Coagulation studies revealed low functional and antigenic fibrinogen concentrations suggestive of hypofibrinogenemia. Amplification and DNA sequencing showed a heterozygous deletion of the a7690 to g7704 nucleotides of the gamma chain gene in the 3'end of exon 8 (g 7690_7704del14; Genbank access M10014); this deletion encompassed the splicing site at position 7703 and predicts in a new putative consensus splicing sequence (AATGgtatgtt). RNA was extracted from a liver specimen from the proposita's brother. The cDNA obtained by reverse transcription polymerase chain reaction confirmed the usage of a newly generated donor site at position 7688 of the genomic sequence resulting in an in-frame heterozygous 5 amino acid deletion (GVYYQ 346-350; p.G372_Q376del) and that this mutation is responsible for a new splicing site at position 7688 of the genomic sequence. CONCLUSION: we suggest that the molecular defect in fibrinogen Angers results in an impaired assembly and causes defective secretion and hepatic storage of fibrinogen.

[Intracranial ependymomas in adult patients. Diagnosis and histological prognostic factors]. / Ependymomes intracrâniens de l'adulte. Diagnostic histologique et facteurs histopronostiques.

BACKGROUND: Intracranial ependymomas are rare in adults and histopathological prognostic factors are poorly determined. PURPOSE: A retrospective multicentric study was conducted in France in order to assess the prognostic value of histology. MATERIAL: Between 1990 and 2004, 216 adult patients with newly diagnosed ependymomas were treated in 19 French centers. Eligibility required institutional histopathological confirmation of an ependymoma and available clinical history and MRI features (see comparison paper). METHODS: Histological preparations and one paraffin embedded block from each patient were sent to Pr D. Figarella-Branger in Marseille. Central review by four neuropathologists (D. Figarella-Branger, A. Maues de Paula, C. Fernandez and A. Jouvet) was performed. Specimens for which all pathologists agreed with the histological diagnosis of ependymomas were included, whereas cases for which all disagree were excluded and reclassified. In the event of doubt and/or discrepancies between pathologists immunostaining was performed in order to reach a consensus diagnosis. Diagnostic of ependymomas was confirmed in 121 cases (56%). In theses cases, ependymomas were classified according to the WHO system (subtype and grade). The potential prognostic value (overall survival OS and disease free survival DFS) of the following histological parameters was examined: perivascular pseudorosettes, ependymal rosettes, hyalinized vessels, mitotic index, microvascular proliferation, necrosis, area of increased cellularity, nuclear atypia, brain invasion and Mib-1 labelling index. RESULTS: Among the 121 ependymomas, 88 were grade II (47 classic, 17 cellular, 2 papillar, 6 clear cells and 16 tanicytic) and 33 grade III. WHO grading, occurrence of microvascular proliferation, necrosis, nuclear atypia and high proliferative index were correlated with both OS and DFS. Moreover, quantification of certain parameters enabled a reproducible grading system correlated with both OS and DFS.

[Tumor like presentation of primitive amyloidosis: amyloidoma]. / Forme pseudotumorale d'une amylose primitive: l'amyloïdome.

INTRODUCTION: AL-amyloidosis is a rare disease due to monoclonal immunoglobulin deposits, secondary to lymphoproliferative disorder or primitive. The deposits of amyloidosis have usually a systemic repartition. We report a tumor like presentation of amyloidosis, so-called amyloidoma. EXEGESIS: A 72-year old woman lost 10 kg within 6 months, associated with epigastric and mediastinal bulks. The biopsy of the abdominal mass showed AL-amyloidosis with kappa light chains. Since no secondary etiology could be found, the final diagnosis of primary AL-amyloidosis in a tumour like presentation, or amyloidoma, was performed. Investigations showed cardiac involvement with MRI findings, as well as kidney and bone marrow involvement. Oral melphalan as monotherapy was administered. The prognosis and the treatment of this unusual disease are discussed. CONCLUSION: Amyloidoma is a rare presentation of amyloidosis which should be evocated in front of a soft tissue mass with no clear etiology.

Lesión medular por violencia de género / Spinal cord injury due to gender violence

Objetivo. Analizar las características fundamentales de la lesión medular en una serie de mujeres de edad superior a 18 años en situación de violencia de género. Material y métodos. Período de estudio: 1992-2004. Variables: edad, nivel de lesión, clasificación ASIA, mecanismo lesional, nacionalidad, nivel cultural y aspectos legales. Resultados. Número de mujeres totales: 689 y 7 lo fueron como consecuencia de violencia de género. Edad media: 32 años (rango: 23-40). Nivel lesional: cervical (4), dorsal (2), lumbar (1). Clasificación ASIA: completa (6), incompleta (1). Causas: agresión por arma blanca (3), precipitación (3), accidente de tráfico intencionado por parte del agresor (1). Dos mujeres estaban embarazadas en el momento de la agresión. Nacionalidad: española (3) y extranjera (4). Pico de incidencia máximo (año 2002) con 4 casos. En todos los casos el nivel cultural era bajo. Se apreciaba gran reticencia para reconocer el problema de violencia de género como causa de la agresión. Conclusiones. La incidencia de la agresión en el contexto de violencia género como causa de la lesión medular en nuestra población femenina adulta fue del 1 %. Esta causa se reconoce de forma más explícita, y por lo tanto con mayor frecuencia, en los años recientes. Consideramos que nuestra serie muestra indicios de que es probable que un porcentaje (de cuantía desconocida) de situaciones como caídas casuales o precipitaciones, puedan en realidad traducir un trasfondo de violencia de género. Creemos que el abordaje de estos casos implica a un equipo multidisciplinar, ya que en su génesis intervienen factores socioculturales y psicológicos

Objective. The authors review the main clinical and social features of a series of women aged above 18 years who suffered spinal cord injury in setting of gender violence. Material and methods. Study period: 1992-2004. Variables: age, lesion level, ASIA classification, injury mechanism, sociocultural level, legal aspects. Results. The total number of women admitted to our hospital was 689, 7 of whom had been injured as a result of domestic violence. Mean age: 32 years (range: 23-40). Level of spinal cord injury: cervical (4), dorsal (2), lumbar (1). ASIA classification: complete (6), incomplete (1). The direct causes were: aggression by knife (3), precipitation (3), and provoked car accident (1). Two women were pregnant at the time of the aggression. Nationality: Spanish (3), foreigner (4). Maximum peak incidence was in 2002 (4 cases). In all cases the social level was low. It was observed that all women, in spite of evidence, did not initially admit a problem of domestic violence by a partner as the real cause of their lesion. Conclusions. The incidence of gender violence as a cause of spinal cord injury in our female population was 1 %. It seems that this cause is recognized more frequently in recent years. It is probable that an undetermined number of accidental falls or precipitations may actually be generated in a background of gender violence. We think that a multidisciplinary approach is warranted as social and psychological factors play an important role in these patients