Catastrophic antiphospholipid syndrome and pregnancy: an experience of 13 cases.

OBJECTIVE: Catastrophic antiphospholipid syndrome (CAPS) is a life-threatening disease caused by the onset of rapidly progressive and widespread small-vessel thromboses in the presence of aPLs. The aim of this study was to examine pregnancy-related CAPS. METHODS: Retrospective series of 13 patients with pregnancy-related CAPS with special focus on the follow-up. RESULTS; Eleven patients had known APS and had been treated with low-molecular-weight heparin (n = 10), aspirin (n = 8), oral anticoagulants (n = 1), HCQ (n = 3) and/or steroids (n = 1) during pregnancy. The most frequent manifestations of CAPS were cutaneous (n = 11), hepatic (n = 11), renal (n = 10), cardiac (n = 8) and neurological (n = 5). CAPS usually followed haemolysis, elevated liver enzymes and low platelet count (HELLP) syndrome (n = 12), which was associated with pre-eclampsia (n = 6) or with eclampsia (n = 3). No maternal death was observed. The perinatal mortality of 54% was related to prematurity with a mean gestational age of 26.6 weeks at onset of CAPS or HELLP syndrome. During a mean follow-up of 4.8 years (range 2-8 years), seven new pregnancies occurred in five patients and led to one miscarriage, four successful pregnancies and two HELLP syndrome with pre-eclampsia or eclampsia that occurred at 28 weeks gestation in both cases despite optimal treatment. No relapse of CAPS was observed. Two mothers suddenly died 2.5 and 6 years after CAPS. CONCLUSION: The occurrence of HELLP syndrome in a patient with APS should raise the suspicion of CAPS in the following days, and anticoagulation should be maintained post-partum or post-abortum. Subsequent pregnancies are at very high risk.

Alveolar concentration of nitric oxide predicts pulmonary function deterioration in scleroderma.

BACKGROUND: Respiratory failure is a life-threatening and unpredictable complication of systemic sclerosis (SSc). A study was undertaken to assess the value of alveolar nitric oxide (NO) in predicting the risk of lung function deterioration leading to respiratory failure or death in patients with SSc. METHODS: 105 patients with SSc were enrolled in this prospective cohort and were followed longitudinally over a 3-year period during which the risk of occurrence of deleterious events was analysed according to alveolar concentration (C(A)NO), conducting airway output (J'(aw)NO) and fractional concentration (F(E)NO(0.05)) of exhaled NO measured at inclusion. Comparison was made between each NO parameter to predict the occurrence of deleterious events, defined as a 10% decrease in total lung capacity or forced vital capacity from baseline, or death. RESULTS: The area under the receiver operating characteristic curve of C(A)NO to predict the occurrence of the combined events was 0.84 (95% CI 0.76 to 0.92; p<0.001), which was significantly higher than those of J'(aw)NO and F(E)NO(0.05) (p<0.001). A cut-off of C(A)NO of 5.3 ppb had a sensitivity of 88% and a specificity of 62% for the prediction of the occurrence of combined events during follow-up, and was validated in an independent cohort of patients with SSc. Combined events occurred more frequently in patients whose C(A)NO was >5.3 ppb. The adjusted HR for patients with C(A)NO >5.3 ppb was 6.06 (95% CI 2.36 to 15.53; p<0.001). C(A)NO accurately predicted the occurrence of combined events irrespective of forced vital capacity values or the presence of interstitial lung disease at baseline. CONCLUSIONS: Increased C(A)NO accurately identifies patients with SSc with a high risk of developing lung function deterioration and may help to initiate early appropriate treatment.

The 2003 heat wave in France: hydratation status changes in older inpatients.

Little is known about the impact of behavioral changes after the 2003 heat wave on hydration status of elderly citizens in France. We used an administrative data file provided information about 23,022 inpatients aged > or =70 years admitted between 2000 and 2006, including vital status at discharge and Charlson comorbidity index and matched it with the result of five blood tests (sodium, potassium, glucose, urea nitrogen, creatinine) within the first 24 h after admission and with daily temperatures before admission. We then measured the prevalence of plasma tonicity (PT) <275 mOsm/l or >300 mOsm/l, blood urea nitrogen/creatinine ratio (BUNC) >100 and inhospital mortality. In 2000-2002, 2003, 2004-2006, prevalence (%) was, respectively 7.5, 8.0, 9.5 (P < 0.0001) for PT < 275 mMol/l, 8.4, 10.4, 7.2 (P < 0.0001) for PT > 300 mOsm/l, and 35.4, 30.7, 26.7 (P < 0.0001) for BUNC > 100. Inhospital mortality rate was 10.8, 10.8 and 9.0%, respectively (P < 0.0001). After adjustment for covariates, OR (95% CI) in 2004-2006 with reference to 2000-2002 was 1.26 (1.13-1.39) for PT < 275 mMol/l, 0.85 (0.76-0.94) for PT > 300 mOsm/l, and 0.65 (0.61-0.69) for BUNC > 100. Inhospital mortality risk associated with hydration disorders did not vary significantly over periods for PT < 275 mMol/l (HR 1.06 to 1.40) and PT > 300 mOsm/l (HR 1.76 to 1.96) but was lower for BUNC > 100 in 2003 (HR 1.27) than in 2000-2002 (HR 1.64) or 2004-2006 (HR 1.77) (P = 0.04). So, since the 2003 heat wave, significant shifts in prevalence of intracellular hydration disorders indicate behavioral changes with positive impact on hydration status.

Cardiovascular risk factor levels and their relationships with overweight and fat distribution in children: the Fleurbaix Laventie Ville Santé II study.

This study aimed to document for the first time in a general population of French children the prevalence and levels of cardiovascular risk factors and to assess separately in boys and girls whether these risk factors were associated with fat mass distribution independently of subcutaneous overall adiposity. A cross-sectional analysis of baseline data from 452 children (235 boys and 217 girls) aged 8 to 17 years included in a 1999 population-based epidemiologic study (the Fleurbaix Laventie Ville Santé II study) was made. Overweight was defined according to the International Obesity Task Force references and the 90th percentiles of the French body mass index curves. The thresholds of parameters defining cardiovascular and metabolic risks were the 95th percentile of the Task Force Report on High Blood Pressure in Children and Adolescents for blood pressure and those of the American Academy of Pediatrics for lipids. Anthropometric and biological parameters were described by sex and according to overweight status. Partial correlations between cardiovascular risk factors and anthropometric measures of adiposity (body mass index, sum of 4 skinfold thicknesses, waist circumference, waist-to-height ratio) were calculated. Then, these correlations were additionally adjusted for the sum of 4 skinfold thicknesses. High plasma triglycerides, high insulin concentration, and low plasma high-density lipoprotein cholesterol (HDL-C) concentration were associated with all measures of adiposity (|r| > or = 0.20, P < .002). When obese children were excluded, overweight children already had high triglycerides and low HDL-C levels, respectively, 2 and 20 times more frequently than normal-weight children did. Among overweight children, 7.7% had at least 2 risk factors among high blood pressure, high plasma triglycerides or glucose, and low HDL-C concentration vs 0.25% among normal-weight children (P = .002). After adjusting for the sum of skinfolds, an independent association between the risk factors and waist circumference was found in girls. In conclusion, (a) modest excess weight is associated with increased levels of cardiovascular risk factors. (b) In girls, abdominal fat distribution is associated with cardiovascular risk factors, independently of overall adiposity. (c) International definition of abdominal obesity in children is required to standardize studies and to progress in the evaluation of childhood obesity and its consequences.

Serum adiponectin is related to plasma high-density lipoprotein cholesterol but not to plasma insulin-concentration in healthy children: the FLVS II study.

Although low levels of plasma adiponectin were associated with an increase in cardiovascular risk in adults, few data investigated that relationship in children. The aim of this study was to investigate the relationship between plasma adiponectin and cardiovascular risk factors in healthy children. This cross-sectional population-based study was conducted in Fleurbaix and Laventie, 2 cities in the north of France. The main outcome measure was the correlations between plasma adiponectin and adiposity variables (the body mass index, the sum of 4 skinfolds, waist circumference [WC], and percent body fat [bioimpedance]), blood pressure, plasma glucose, triglycerides, high-density lipoprotein (HDL) cholesterol and insulin. In 398 children of both sexes, adiponectin was not significantly related to age and pubertal stage. In boys only, adiponectin correlated with WC (r = -0.19; P = .008) and body mass index (r = -0.15; P = .04) but not with other adiposity variables. After taking into account WC, adiponectin was positively correlated with HDL-cholesterol in boys (r = 0.14; P = .05) and girls (r = 0.25; P = .0004), but was not correlated with insulin and homeostasis model assessment index for insulin resistance in both sexes. These results suggest that, in apparently healthy children, adiponectin is related to the level of HDL-cholesterol independently of fat mass. The relationship between adiponectin and insulin resistance previously reported in obese or diabetic children was not apparent in these subjects and may therefore occur only at later age with fat accumulation.

[Evaluation of medical semiology teaching and teachers. Study of student satisfaction in the second cycle first year]. / Appréciation de l'enseignement et évaluation des enseignants de sémiologie médicale. Enquêtes de satisfaction auprès des étudiants de première année du deuxième cycle.

OBJECTIVE: To know the students' opinion concerning semiology teaching and teachers. METHODS: Annual enquiry between 1990-91 and 2000-01, before and after a reform increasing to 400 the hours dedicated to medical clerkships in the second and third years of the courses. RESULTS: Before the reform, at least 62% of the students were in favor of the general organization, while a growing percentage (reaching 79.6%; p < 0.0001) considered the number of hours dedicated to clinical clerkship as insufficient. Concerning the teachers, the assessment concerning the "assistant heads of departments" (AHD) showed great disparities. Out of 100 teachers-year, 4 obtained a score lesser than 10/20 and 59 greater than 15/20. After the reform, 19 AHD out of 33 obtained more than 15/20 and 5 less than 10/20. The reintroduction of lectures was not criticized. The professors who gave lectures were reproached by the students for delaying them or for not having given them at all. They were also reproached for their lack of relationship with the teaching objectives. The scores allocated to professors ranged from 4.5 to 15.9/20. CONCLUSION: Enhancing the value of teaching for an AHD's university career and reducing the administrative burden for all teachers would be useful to improve medical education, especially in semiology.

[Epidemiologic, clinical, biological and therapeutic aspects of Gaucher disease]. / Aspects épidémiologiques, cliniques, biologiques et thérapeutiques de la maladie de Gaucher.

GENERAL CHARACTERISTICS: Gaucher's disease is a genetic disease of autosomal recessive transmission due to a deficit in a lysosomal enzyme: beta-glucocerebrosidase. The disease is characterised by deposits of glucosylceramide in the cells of the liver, spleen and bone marrow. Acute or chronic neurological forms (type 2 and 3) account for only 5% of patients suffering from Gaucher's disease and are less frequent than the non-neurological forms (type 1). CLINICAL AND BIOCHEMICAL MANIFESTATIONS: Gaucher's disease is associated with spleno- or hepato-megalia, asthenia, bone complications (Erlenmeyer flask deformity, osteopenia and osteonecrosis), as well as with haematological (thrombopenia, anaemia) or biochemical abnormalities (increase in angiotensin-converting enzyme, ferritin, tartrate-resistant acid phosphatase and chitotriosidase). Central nervous system involvement is only found in the type 2 and 3. Diagnosis relies on measurement of beta-glucocerebrosidase activity in the circulating leukocytes. REGARDING TREATMENT: Treatment with enzyme replacement (imiglucerase: recombinant enzyme preparation) improves the haematological abnormalities, hepatosplenomegalia and quality of life in a matter of a few months. Regression of the bone disorders is usually observed only after 3-4 years of treatment. Recently, gene therapy trials have successfully been started.

Cutaneous lymphangitis carcinomatosa in a patient with lung adenocarcinoma: case report and literature review.

We report the case of a man with a seven year history of lung adenocarcinoma who was diagnosed with cutaneous lymphangitis carcinomatosa. Skin examination revealed both an asymptomatic erysepelatoid rash localized on the posterior chest and an erythematous, eczematiform, itchy rash on the right anterior chest and on the left shoulder. Histopathologic examination of biopsies of these lesions revealed the same aspect with an infiltration of the dermal lymphatics by metastatic adenocarcinomatous cells. Cutaneous lymphangitis carcinomatosa is a rare condition accounting for less than 5% of skin metastases. A literature review identified eight other cases of cutaneous lymphangitis carcinomatosa in patients with lung cancer.

[Inflammatory aortitis in giant cell arteritis]. / Aortite inflammatoire au cours de la maladie de Horton.

A sub-clinical inflammatory aortitis is very frequent in patients with giant cell arteritis, and can be the only localization of the disease. In most patients, this aortitis is asymptomatic and is of no consequence on the patient's survival. The relative risk of developing an aortic dissection or aneurysm is 17.3. Evolution towards an aneurysm or an aortic dissection is unpredictable and rare; and seems independent of the disease activity and the associated vascular risk factors. Isolated aortitis treatment is not consensual, but often similar to the treatment of giant cell arteritis and adapted to clinical and biological markers of disease activity. Screening for sub-clinical aortitis with FDG-PET should not be prescribed in patients with typical presentation of giant cell arteritis. A systematic screening of aortic complications in giant cell arteritis patients could be done with a chest X-ray and an abdominal ultrasound possibly completed with an aortic CT-scan at time of diagnosis, in order to look for aneurysms with possible surgical indication.

Aldolase predicts subsequent myopathy occurrence in systemic sclerosis.

INTRODUCTION: Myopathy related to systemic sclerosis (Myo-SSc) is a disabling and unpredictable complication of SSc. We assessed the predictive value of serum aldolase, creatine kinase (CK), alanine transaminase (ALT), aspartate transaminase (AST) and C-reactive protein (CRP) to estimate the risk of developing Myo-SSc. METHODS: We enrolled 137 SSc patients without proximal muscle weakness in a prospective monocentric study to follow them longitudinally over a four-year period. The risk of occurrence of Myo-SSc was ascertained according to the European NeuroMuscular Centre criteria and was analyzed according to levels of plasma aldolase, CK, transaminase enzymes and CRP at inclusion. Performance of each parameter to predict Myo-SSc occurrence was assessed and compared with the others. RESULTS: The area under the receiver operating characteristic curves (ROC) of plasma aldolase for Myo-SSc occurrence prediction was 0.80 (95% CI: 0.67 to 0.94, P < 0.001), which was higher than that of plasma CK (0.75, P = 0.01), and that of ALT (0.63, P = 0.04). AST and CRP had no predictive value for Myo-SSc occurrence. The best cut-off of aldolase for prediction of Myo-SSc occurrence within three years after inclusion was 9 U/L and higher than the upper normality limit (7 U/L), unlike that of CK and ALT. Myo-SSc occurred more frequently in patients whose plasma aldolase was higher than 9 U/L. Adjusted Hazard Ratio for patients with aldolase > 9 U/L was 10.3 (95% CI: 2.3 to 45.5), P < 0.001. CONCLUSIONS: Increased plasma aldolase level accurately identified SSc patients with high risk to develop subsequent Myo-SSc. This could help initiate appropriate treatment when the disabling muscle damage is still in a reversible stage.

Liver stiffness measurement in patients with cirrhosis and hepatocellular carcinoma: a case-control study.

OBJECTIVE: A wide range in values of liver stiffness measurement (LSM) is observed among cirrhotic patients. These variations reflect the extent of fibrosis and might influence the risk of hepatocellular carcinoma (HCC) occurrence. METHODS: We compared LSM in 66 Child-Pugh A patients with HCC and alcoholic (n=23) or HCV-related cirrhosis (n=43) referred for radiofrequency ablation and in 199 Child-Pugh A with alcoholic (n=69) or HCV-related cirrhosis (n=130) without HCC. RESULTS: Patients with HCC had higher LSM than patients without HCC [35.3 kPa (22.8-52.6) vs. 19.0 kPa (12.4-29.2), P<0.0001]. In multivariate analysis, HCC was associated with higher LSM [odds ratio=1.051 (1.030-1.072) (by 1 kPa increase), P<0.0001] and with age [odds ratio=1.075 (1.043-1.107) (by 1 year increase), P<0.0001]. In patients without HCC, LSM was not correlated with age but with decreased prothrombin activity, serum albumin, platelet count, and increased serum bilirubin level. Alcoholic patients had higher LSM compared with HCV-infected patients [22.1 kPa (14.0-36.5) vs. 15.9 kPa (10.8-21.9), P<0.0001] and LSM in the latter varied according to antiviral treatment response. CONCLUSION: In patients with Child-Pugh A cirrhosis, a wide range of LSM is observed according to the cause underlying liver disease and the presence of HCC is associated with higher values in these patients.

Relationships between fibrosis amounts assessed by morphometry and liver stiffness measurements in chronic hepatitis or steatohepatitis.

OBJECTIVES: To examine the relationships between fibrosis amounts evaluated by morphometric analysis and liver stiffness measurement (LSM) as well as the influence of steatosis, of activity grade and of the type of chronic liver injury. METHODS: One hundred and fifty-two consecutive patients were selected from a prospective cohort of patients with concurrent liver biopsy and LSM on the basis of a more than 25 mm long liver biopsy. Morphometric quantification of collagen deposition was expressed as fibrosis area fraction (FAF). Steatosis, activity grade, and predominant type of liver injury (chronic hepatitis or steatohepatitis) were assessed. RESULTS: In the whole population (chronic viral hepatitis n = 96, alcoholic or nonalcoholic steatohepatitis n = 56), FAF was significantly correlated to LSM (rho = 0.6, P<0.0001). Steatosis independently influenced LSM in patients without cirrhosis, but not activity grade. In the absence of cirrhosis, LSM and FAF were correlated in patients with chronic hepatitis (rho = 0.49, P<0.0001) but not in those with steatohepatitis (rho = 0.22, P = 0.16). In cirrhotic patients, LSM was correlated with fibrosis amount. (rho = 0.43, P = 0.006). The area under the receiver operating characteristics curve of LSM was 0.75 for separating no or minor fibrosis from significant fibrosis and 0.9 for the diagnosis of cirrhosis. CONCLUSION: LSM was significantly correlated with collagen deposition. However, the relationships between LSM and fibrosis amount seems to differ according to steatosis and to the pattern of liver fibrosis below the cirrhotic stage. Its performances are fair for segregating patients with no or minor fibrosis from those with significant fibrosis and high for the diagnosis of cirrhosis.

Factors associated with underlying malignancy in a retrospective cohort of 121 patients with dermatomyositis.

Demographic, clinical, and laboratory features that predict underlying malignancy in patients with dermatomyositis (DM) are poorly known. We conducted a retrospective study in all adult patients with a definite (n = 75) or probable (n = 32) diagnosis of DM according to Bohan and Peter criteria or with amyopathic DM (n = 14) who were referred to 2 departments during a 13-year period. The diagnosis of malignancy-associated DM was retained if DM occurred in a context of recently diagnosed malignancy or if a malignancy was diagnosed during the 5 years following the diagnosis of DM. The Kaplan-Meier method was used to assess the cumulative incidence rates of underlying malignancy during the first 5 years of DM. Factors associated with malignancy in patients with DM were identified by Cox proportional hazards models. During the study period, 121 patients fulfilled the inclusion criteria (median age, 52 yr; range, 19-77 yr; women: 70%). For 29 of them, the diagnosis of malignancy-associated DM was retained. The cumulative incidence rate of malignancy was 21 +/- 4% and 28 +/- 5%, 1 year and 5 years after the diagnosis of DM, respectively. The median duration of follow-up of the 92 patients with no malignancy diagnosed was 36 months (range, 1-140 mo). In multivariate analysis, independent factors associated with an underlying malignancy in patients with DM were an age at diagnosis >52 years (hazard ratio [HR], 7.24; 95% confidence interval [CI], 2.35-22.31), a rapid onset of skin and/or muscular symptoms (HR, 3.11; 95% CI, 1.07-9.02), the presence of skin necrosis (HR, 3.84; 95% CI, 1.00-14.85) or periungual erythema (HR, 3.93; 95% CI, 1.16-13.24), and a low baseline level of complement factor C4 (HR, 2.74; 95% CI, 1.11-6.75). Lastly, low baseline lymphocyte count (<1500/mm(3)) was a protective factor of malignancy (HR, 0.33; 95% CI, 0.14-0.80). Taken together, these data may help physicians focus on a group of patients who might benefit from extensive evaluation for malignancy.

Incidence, risk factors, and severity of herpesvirus infections in a cohort of 121 patients with primary dermatomyositis and dermatomyositis associated with a malignant neoplasm.

OBJECTIVE: Opportunistic infections have been reported in 15% to 21% of patients with inflammatory myositis. However, to our knowledge, no data are available regarding the incidence, risk factors, and severity of herpesvirus infections. DESIGN: Retrospective inception cohort study. SETTING: Two departments in tertiary teaching hospitals. Patients All patients diagnosed as having dermatomyositis (DM) according to the criteria of Bohan and Peter seen during a 13-year period. MAIN OUTCOME MEASURES: Cumulative incidence rates of herpesvirus infections using the Kaplan-Meier method and risk factors for herpesvirus infections during the first year of DM using Cox proportional hazards models. RESULTS: A total of 121 patients met the inclusion criteria (mean [SD] age, 52 [15] years; 85 were women [70%]). Seventy-six percent had primary dermatomyositis, and 24% had dermatomyositis associated with a malignant neoplasm. The mean (SD) duration of follow-up was 42 (33) months. During follow-up, 20 patients developed a total of 22 herpesvirus infections (16 developed herpes zoster infections). The incidence rates for herpesvirus and for herpes zoster infections were 49 and 33 episodes per 1000 patient-years, respectively. In multivariate analysis, a positive association was noted between the risk of herpesvirus infection and use of systemic corticosteroid therapy (hazard ratio [HR], 3.71 [95% confidence interval {CI}, 1.02-13.41]; P = .04), lymphocyte count lower than 6000/microL (HR, 3.55 [95% CI, 1.00-12.65]; P = .05), and creatine phosphokinase level higher than 300 U/L (HR, 4.81 [95% CI, 1.28-18.06]; P = .02). Dermatomyositis associated with a malignant neoplasm tended to be negatively associated with the risk of herpesvirus infection (HR, 0.16 [95% CI, 0.02-1.29]; P = .08). CONCLUSIONS: The risk of serious herpesvirus infections in patients with DM is high. Educational strategies and studies evaluating the risk-to-benefit and the cost-to-benefit balances of a prophylaxis with valacyclovir hydrochloride in selected patients with DM are warranted.

Digital photography as an operational tool for assessing corticosteroid-induced lipodystrophy.

BACKGROUND: Corticosteroid-induced lipodystrophy (CIL) is exclusively diagnosed in a subjective manner. OBJECTIVE: To evaluate the reliability of digital photographs in the diagnosis of CIL. METHODS: All consecutive patients starting long-term, high dosage corticosteroid therapy were photographed at baseline and after 3 months of therapy. At the end of the study, 3 physicians with expertise in corticosteroids classified patients as lipodystrophic yes/no/unclassifiable. Photographs analyses performed by 9 medical readers and evaluation of CIL using visual analog scale (VAS) performed during the M3 visit were compared to this classification. RESULTS: Eighty-eight patients were monitored. Fifty of them were classified by the 3 experts as lipodystrophic and 30 as not lipodystrophic (8 were unclassifiable). Their intra- and inter-observer agreements were moderate or fair (kappa coefficientor=0.75) when M3 photographs were analysed beside baseline ones. By comparison with expert consensus, only 3 out of 4 patients were correctly classified using VAS. The AUROC curve and inter-observer agreement significantly improved with experience for the 9 non-experts. CONCLUSION: The use of digital photographs do better than VAS to evaluate CIL. The accuracy of diagnosis improves with experience. Morphological changes are more important than morphological phenotype.

Low glycosylated ferritin, a good marker for the diagnosis of hemophagocytic syndrome.

OBJECTIVE: A very low percentage of glycosylated ferritin (<20%) has only been reported in association with adult-onset Still's disease (AOSD), a disease classically associated with hemophagocytic syndrome. We undertook this study to determine whether hemophagocytic syndrome outside the context of AOSD is also associated with a very low percentage of glycosylated ferritin. METHODS: From October 2006 to September 2007, the serum level of glycosylated ferritin was determined in all consecutive patients seen in 3 departments and for whom the diagnosis of hemophagocytic syndrome was suspected. The level of glycosylated ferritin in these patients was compared with that in age- and sex-matched controls with a marked inflammatory syndrome not associated with hemophagocytic syndrome. We assessed the value of glycosylated ferritin as a marker for the diagnosis of hemophagocytic syndrome. RESULTS: Forty-two patients were included in the study (14 with confirmed hemophagocytic syndrome, 7 with suspected but unconfirmed hemophagocytic syndrome, and 21 controls). The median level (interquartile range [IQR]) of total serum ferritin was significantly higher in patients with confirmed hemophagocytic syndrome (3,344 microg/liter [2,074-7,334]) than in patients with suspected but unconfirmed hemophagocytic syndrome (555 microg/liter [464-1,420]) (P = 0.02) or in controls (451 microg/liter [126-929]) (P < 0.001). The median (IQR) percentage of glycosylated ferritin was significantly lower in patients with confirmed hemophagocytic syndrome (10% [3-14]) than in patients with suspected but unconfirmed hemophagocytic syndrome (40% [36-47]) (P < 0.001) or in controls (36% [26-49]) (P < 0.001). The diagnostic performance of glycosylated ferritin tended to be higher than that of total serum ferritin for the diagnosis of hemophagocytic syndrome (area under the receiver operating characteristic curve [95% confidence interval] 0.97 [0.92-1.00] versus 0.79 [0.59-1.00]; P = 0.10). CONCLUSION: These results suggest that glycosylated ferritin may be a helpful marker for the diagnosis of hemophagocytic syndrome.

Assessment of liver fibrosis using transient elastography in patients with alcoholic liver disease.

BACKGROUND/AIMS: The aim of this study was to assess the accuracy of liver stiffness measurement (LSM) for the diagnosis of extensive fibrosis and cirrhosis in patients with alcoholic liver disease (ALD). METHODS: One hundred and seventy-four patients with ALD were enrolled in four liver units and underwent concomitant liver biopsy and LSM. Fibrosis was assessed using the Brunt et al. and the Chevallier et al. scoring systems. Steatosis and histological alcoholic hepatitis (HAH) were quoted in classes. RESULTS: Twenty-seven patients had inadequate biopsy or LSM. Distribution in 147 patients according to the Brunt score (median LSM) was: F1: n=13 (5.7kPa); F2: n=24 (8.3kPa); F3: n=31 (17.5kPa) and F4: n=79 (40.9kPa) (P<0.0001). LSM was correlated with the amount of fibrosis according to the Chevallier score (r=0.70, P<0.0001). LSM was correlated to fibrosis stage (tau beta, 0.53; P<0.0001) and HAH (tau beta, 0.30; P<0.0001). In multivariate analysis, fibrosis was the only parameter correlated with LSM. The areas under the ROC curve were 0.94 and 0.87 for the diagnosis of extensive fibrosis (Brunt et al. score > or =3) and cirrhosis, respectively (threshold-values: 12.9 and 22.6kPa). CONCLUSIONS: LSM accurately assesses extensive fibrosis and cirrhosis in alcoholic patients.