Body composition using bio-impedance analysis in pediatric patients with inflammatory bowel disease. Corcordance with dual energy X-ray absorptiometry and comparison with healthy controls

Introduction: Growth is a central process in paediatrics. Weight and height evaluation are therefore routine exams for every child but in some situation, particularly inflammatory bowel disease (IBD), a wider evaluation of nutritional status needs to be performed. Objectives: To assess the accuracy of bio-impedance analysis (BIA) compared to the gold standard dual energy X-ray absorptiometry (DEXA) in estimating percentage body fat (fat mass; FM) and lean body mass (fat free mass; FFM) in children with inflammatory bowel disease (IBD). To compare FM and FFM levels between patients with IBD and healthy controls. Methods: Twenty-nine healthy controls (12 females; mean age: 12.7 ± 1.9 years) and 21 patients (11 females; 14.3 ± 1.3 years) were recruited from August 2011 to October 2012 at our institution. BIA was performed in all children and DEXA in patients only. Concordance between BIA and DEXA was assessed using Lin's concordance correlation and the Bland-Altman method. Between-group comparisons were made using analysis of variance adjusting for age. Results: BIA-derived FM% showed a good concordance with DEXA-derived values, while BIA-derived FFM% tended to be slightly higher than DEXA-derived values (table). No differences were found between patients and controls regarding body mass index (mean ± SD: 19.3 ± 3.3 vs. 20.1 ± 2.8 kg/m2, respectively; age-adjusted P = 0.08) and FM% (boys: 25.3 ± 10.2 vs. 22.6 ± 7.1%, for patients and controls, respectively; P = 0.20; girls: 28.2 ± 5.7 vs. 26.4 ± 7.7%; P = 0.91). Also, no differences were found regarding FFM% in boys (74.9 ± 10.2 vs. 77.4 ± 7.1%; P = 0.22) and girls (71.8 ± 5.6 vs. 73.5 ± 7.7%; P = 0.85). Conclusion: BIA adequately assesses body composition (FM%) in children with IBD and could advantageously replace DEXA, which is more expensive and less available. No differences in body composition were found between children with IBD and healthy controls.

Influence des habitudes alimentaires sur la morbidité chez 645 patients obèses d'une consultation spécialisée. [Effect of eating habits on morbidity in 645 patients at a specialized clinic].

Among 645 obese patients examined at an out-patient clinic for obese patients by physical examination and a computerized questionnaire, two subgroups of patients could be identified according to their nutritional preferences: 177 patients preferred carbohydrates exclusively (group A) and 73 patients fat exclusively (group B). No definite preferences were formulated by the other patients. Among patients under 25 years, only 3 belonged to group B and 49 to group A, while in older patients no significant differences were found. Among patients with BMI less than 30, there were significantly fewer patients from group B than from group A (p = 0.006), while in patients with BMI greater than 30 no significant difference was observed. There were significantly more men in group B than in group A. 57% of the patients of group B complained of physical symptoms related to their obesity, compared to 37% in group A (p = 0.006). 26% of group B suffered from joints and muscles compared to 13% of group A (p = 0.003). Hyperglycemia (greater than 5,6 mmol/l) was found in 21% of group A and in 40% of group B (p less than 0.005). Hypercholesterolemia (greater than 6.5 mmol/l) was found in 20% of group A and in 32% of group B (p less than 0.05). In conclusion, obese patients who prefer fat have more general symptoms related to obesity, more abnormal physical signs, and more frequently have hyperglycemia and hypercholesterolemia than patients who prefer carbohydrates.

International periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis syndrome cohort: description of distinct phenotypes in 301 patients.

OBJECTIVES: The aims of this study were to describe the clinical features of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) and identify distinct phenotypes in a large cohort of patients from different countries. METHODS: We established a web-based multicentre cohort through an international collaboration within the periodic fevers working party of the Pediatric Rheumatology European Society (PReS). The inclusion criterion was a diagnosis of PFAPA given by an experienced paediatric rheumatologist participating in an international working group on periodic fever syndromes. RESULTS: Of the 301 patients included from the 15 centres, 271 had pharyngitis, 236 cervical adenitis, 171 oral aphthosis and 132 with all three clinical features. A total of 228 patients presented with additional symptoms (131 gastrointestinal symptoms, 86 arthralgias and/or myalgias, 36 skin rashes, 8 neurological symptoms). Thirty-one patients had disease onset after 5 years and they reported more additional symptoms. A positive family history for recurrent fever or recurrent tonsillitis was found in 81 patients (26.9%). Genetic testing for monogenic periodic fever syndromes was performed on 111 patients, who reported fewer occurrences of oral aphthosis or additional symptoms. Twenty-four patients reported symptoms (oral aphthosis and malaise) outside the flares. The CRP was >50 mg/l in the majority (131/190) of the patients tested during the fever. CONCLUSION: We describe the largest cohort of PFAPA patients presented so far. We confirm that PFAPA may present with varied clinical manifestations and we show the limitations of the commonly used diagnostic criteria. Based on detailed analysis of this cohort, a consensus definition of PFAPA with better-defined criteria should be proposed.

Osteoporotic fracture risk in elderly women: estimation with quantitative heel US and clinical risk factors

PURPOSE: To derive a prediction rule by using prospectively obtained clinical and bone ultrasonographic (US) data to identify elderly women at risk for osteoporotic fractures. MATERIALS AND METHODS: The study was approved by the Swiss Ethics Committee. A prediction rule was computed by using data from a 3-year prospective multicenter study to assess the predictive value of heel-bone quantitative US in 6174 Swiss women aged 70-85 years. A quantitative US device to calculate the stiffness index at the heel was used. Baseline characteristics, known risk factors for osteoporosis and fall, and the quantitative US stiffness index were used to elaborate a predictive rule for osteoporotic fracture. Predictive values were determined by using a univariate Cox model and were adjusted with multivariate analysis. RESULTS: There were five risk factors for the incidence of osteoporotic fracture: older age (>75 years) (P < .001), low heel quantitative US stiffness index (<78%) (P < .001), history of fracture (P = .001), recent fall (P = .001), and a failed chair test (P = .029). The score points assigned to these risk factors were as follows: age, 2 (3 if age > 80 years); low quantitative US stiffness index, 5 (7.5 if stiffness index < 60%); history of fracture, 1; recent fall, 1.5; and failed chair test, 1. The cutoff value to obtain a high sensitivity (90%) was 4.5. With this cutoff, 1464 women were at lower risk (score, <4.5) and 4710 were at higher risk (score, >or=4.5) for fracture. Among the higher-risk women, 6.1% had an osteoporotic fracture, versus 1.8% of women at lower risk. Among the women who had a hip fracture, 90% were in the higher-risk group. CONCLUSION: A prediction rule obtained by using quantitative US stiffness index and four clinical risk factors helped discriminate, with high sensitivity, women at higher versus those at lower risk for osteoporotic fracture.

Early antifungal intervention strategies in ICU patients.

PURPOSE OF REVIEW: Despite progress in the understanding of the pathophysiology of invasive candidiasis, and the development of new classes of well tolerated antifungals, invasive candidiasis remains a disease difficult to diagnose, and associated with significant morbidity and mortality. Early antifungal treatment may be useful in selected groups of patients who remain difficult to identify prospectively. The purpose of this review is to summarize the recent development of risk-identification strategies targeting early identification of ICU patients susceptible to benefit from preemptive or empirical antifungal treatment. RECENT FINDINGS: Combinations of different risk factors are useful in identifying high-risk patients. Among the many risk factors predisposing to invasive candidiasis, colonization has been identified as one of the most important. In contrast to prospective surveillance of the dynamics of colonization (colonization index), integration of clinical colonization status in risk scores models significantly improve their accuracy in identifying patients at risk of invasive candidiasis. SUMMARY: To date, despite limited prospective validation, clinical models targeted at early identification of patients at risk to develop invasive candidiasis represent a major advance in the management of patients at risk of invasive candidiasis. Moreover, large clinical studies using such risk scores or predictive rules are underway.

Gériatrie [Review in geriatric medicine]

Several studies published in 2008 underline the potential danger of polymedication in older patients and propose indicators to identify those at higher risk for adverse events. A study from Oregon highlighted the difficulties to diagnose depression in patients, especially older ones, who made a request for assisted suicide. The HYVET study demonstrated that treatment of hypertension is beneficial even in some very old persons. A meta-analysis confirmed the benefits from community-based geriatric interventional programs, in particular when targeting older individuals recently discharged from the hospital. Finally, mixed results were observed in the field of dementia.

Preoperative percutaneous portal vein embolization: evaluation of adverse events in 188 patients.

PURPOSE: To retrospectively assess the frequency of adverse events related to percutaneous preoperative portal vein embolization (PPVE). MATERIALS AND METHODS: Institutional review board did not require its approval or patient informed consent for this study. The adverse events that occurred during PPVE or until planned hepatic surgery was performed or cancelled were retrospectively obtained from clinical, imaging, and laboratory data files in 188 patients (109 male and 79 female patients; mean age, 60 years; range, 16-78 years). Liver resection was planned for metastases (n = 137), hepatocarcinoma (n = 31), cholangiocarcinoma (n = 15), fibrolamellar hepatoma (n = 1), and benign disease (n = 4). PPVE was performed with a single-lumen 5-F catheter and a contralateral approach with n-butyl cyanoacrylate mixed with iodized oil as the main embolic agent. The rate of complications in patients with cirrhosis was compared with that in patients without cirrhosis by using the chi(2) test. RESULTS: Adverse events occurred in 24 (12.8%) of 188 patients, including 12 complications and 12 incidental imaging findings. Complications included thrombosis of the portal vein feeding the future remnant liver (n = 1); migration of emboli in the portal vein feeding the future remnant liver, which necessitated angioplasty (n = 2); hemoperitoneum (n = 1); rupture of a metastasis in the gallbladder (n = 1); transitory hemobilia (n = 1); and transient liver failure (n = 6). Incidental findings were migration of small emboli in nontargeted portal branches (n = 10) and subcapsular hematoma (n = 2). Among the 187 patients in whom PPVE was technically successful, there was a significant difference (P < .001) between the occurrence of liver failure after PPVE in patients with cirrhosis (five of 30) and those without (one of 157). Sixteen liver resections were cancelled due to cancer progression (n = 12), insufficient hypertrophy of the nonembolized liver (n = 3), and complete portal thrombosis (n = 1). CONCLUSION: PPVE is a safe adjuvant technique for hypertrophy of the initially insufficient liver reserve. Post-PPVE transient liver failure is more common in patients with cirrhosis than in those without cirrhosis.

Prevalence of articular chondrocalcinosis in elderly subjects in a rural area of Catalonia

The occurrence of rheumatic disorders did not differ significantly between subjects with articular chondrocalcinosis and those without. CONCLUSIONS--Articular chondrocalcinosis is an age related disorder, which could partly explain the discrepancies in its prevalence reported in previous studies. In most subjects with articular chondrocalcinosis recruited from an unselected population the clinical manifestations are probably mild or even absent.

Stages of change, barriers, benefits, and preferences for exercise in RA patients: a cross-sectional study.

OBJECTIVES: To determine the distribution of exercise stages of change in a rheumatoid arthritis (RA) cohort, and to examine patients' perceptions of exercise benefits, barriers, and their preferences for exercise. METHODS: One hundred and twenty RA patients who attended the Rheumatology Unit of a University Hospital were asked to participate in the study. Those who agreed were administered a questionnaire to determine their exercise stage of change, their perceived benefits and barriers to exercise, and their preferences for various features of exercise. RESULTS: Eighty-nine (74%) patients were finally included in the analyses. Their mean age was 58.4 years, mean RA duration 10.1 years, and mean disease activity score 2.8. The distribution of exercise stages of change was as follows: precontemplation (n = 30, 34%), contemplation (n = 11, 13%), preparation (n = 5, 6%), action (n = 2, 2%), and maintenance (n = 39, 45%). Compared to patients in the maintenance stage of change, precontemplators exhibited different demographic and functional characteristics and reported less exercise benefits and more barriers to exercise. Most participants preferred exercising alone (40%), at home (29%), at a moderate intensity (64%), with advice provided by a rheumatologist (34%) or a specialist in exercise and RA (34%). Walking was by far the preferred type of exercise, in both the summer (86%) and the winter (51%). CONCLUSIONS: Our cohort of patients with RA was essentially distributed across the precontemplation and maintenance exercise stages of change. These subgroups of patients exhibit psychological and functional differences that make their needs different in terms of exercise counselling.

Coronary artery disease screening in diabetic patients: how good is guideline adherence?

INTRODUCTION: Diabetic patients are at high risk for coronary artery disease (CAD), which is the leading cause of death in this population. The Swiss Society of Endocrinology-Diabetology (SSED) recommends CAD screening for diabetic patients with > or = 2 additional cardiovascular risk factors (CVRF), by stress echocardiography (SE) or myocardial perfusion imaging (MPI). The aim of this study was to assess the application of these guidelines and the treatment of CVRF in the diabetes outpatient clinics of the five Swiss University Hospitals. METHODS: The study was initiated in Lausanne and the study questionnaires were circulated to the endocrinologists of the five Swiss University Hospitals. Practitioners were asked to include consecutive patients attending the diabetes outpatient clinics over one month. Prevalence of CAD, screening methods for CAD, prevalence of CVRF, biological analyses over the last 6 months and medical therapy were recorded. RESULTS: A total of 302 subjects were included. The mean age was 53 +/- 14 years, 68% had type 2 diabetes, 27% type 1 and 5% other types. Among T2DM with > or = 2 CVRF, 45% were screened for CAD according to SSED guidelines. In T2DM 25% had blood pressure < or = 130/80 mm Hg, 15% a lipid profile within target, 23% HbA1c < or = 7.0%. Overall, 2% achieved all 3 targets. CONCLUSIONS: Only 45% of T2DM with > or = 2 CVRF were screened for CAD according to SSED guidelines and 2% of T2DM had proper control over all CVRF. Efforts are still necessary to improve CAD prevention and screening of diabetic patients in Swiss University Hospitals.

Efavirenz Dose Adjustment in HIV Patients with Impaired CYP2B6 Function

Efavirenz dosage adjustment in several patients with high EFV levels and presenting CNS toxicity have been successfully achieved in Switerland over the past seven years but many others have not beneficiated from dosage reduction owing to the lack of prospective studies evaluating the safety and clinical benefit of reduced dosage regimens.

Novel Cone Transducin Alpha Subunit Mutation In Tunisian Patients And Genotype-phenotype Correlation In Complete Achromatopsia

Purpose: Complete achromatopsia is a rare autosomal recessive disease due to CNGA3, CNGB3, GNAT2 and PDE6C mutations. We studied a large consanguineous Tunisian family including twelve individuals.Methods: Ophthalmic evaluation included a full clinical examination, color vision testing, optical coherence tomography and electroretinography. Linkage analysis using microsatellite markers flanking CNGA3, CNGB3, GNAT2 and PDE6C genes was performed. Mutations were screened by direct sequencing.Results: In all affected subjects, acuity ranged from 20/50 to 20/200. Fundus examination was normal except for two patients who had respectively 4 mm and 5 mm diameters of peripheral congenital hypertrophy. Likewise retinal layers exploration by OCT revealed no change in the thickness of the central retina. Color Vision with 100 Hue Farnsworth test described a profound color impairment along all three axes of color vision. The haplotype analysis of GNAT2 markers revealed that all affected offspring were homozygous by descent for the four polymorphic markers. The maximum lod score value, 4.33, confirmed the evidence for linkage to the GNAT2 gene.A homozygous novel nonsense mutation R313X was identified segregating with an identical GNAT2 haplotype in all affected subjects. This mutation could interrupt interaction with photoactivated rhodopsin, resulting in a failure of visual transduction. In fact, ERG showed a clearly abolished photopic b-wave and flicker responses with no residual cone function justifying the severe GNAT2 achromatopsia phenotype.Conclusions: This is the first report of the clinical and genetic investigation of complete achromatopsia in North Africa and of the largest family with recessive achromatopsia involving GNAT2, thus providing a unique opportunity for genotype phenotype correlation for this extremely rare condition.