Haemoglobin disorders among Southeast-Asian refugees in France

Haemoglobin disorders were studied among Southeast-Asian refugees (Vietnamese, Cambodians and Laotians). Phenotypic haemoglobin investigations and genotypical studies concerning the alpha loci were carried out. Most of the observed cases of microcytosis were related to a thalassaemic defect. High prevalence of Hb E and alpha-thalassaemia were found.     

Le mégauretère primitif de type obstructif : stratégies thérapeutiques à propos de 30 cas

ObjectivesTo present the natural history of primary obstructed megaureters, and to reassess the therapeutic approach towards patients based on both the natural history and the etiologies of the disease.Subjects and methodsWe conducted a retrospective review of 30 children between newborn and 15 years of age who were followed and treated for primary obstructed megaureters between January 1996 and February 2008 in the department of urology at the Point G hospital. They were divided into 2 groups based on etiology: group A included 16 children with an antenatal etiology and group B included 14 children with a post-natal etiology. The patients were evaluated by ultrasound of the urinary system, voiding cystourethrography, scintigraphy, and magnetic resonance imaging.ResultsOf the 30 children included in the study 25 were males. The mean age in group A was 32 weeks and 3 days of gestation, and 4 years in Group B. The megaureter was located on the left side in 48% of cases, on the right side in 30%, and was bilateral in 22%. The megaureter was obstructed and nonrefluxing in 87% of cases, obstructed and refluxing in 13%. The follow-up period was 4.2 ±1.4 years in group A and 3.6 ±1.2 years in group B. Half of the patients had a spontaneous regression of their retrovesical ureteral dilation within the first 2 years of life. A surgical procedure was required for 63% of the patients.ConclusionThis study showed the importance of early diagnosis, and clinical and radiologic surveillance as a first step in the care of megaureters. A conservative approach can result in the spontaneous resolution of the megaureter.     

Self-disclosure of a HIV-positive serostatus: factors favoring disclosure and consequences for persons living with HIV/AIDS in Burkina Faso

Disclosure of HIV-serostatus remains a way to avoid sexual transmission of HIV because it allows partners to take the necessary protective measures, e.g. use of condoms. Disclosure is nevertheless difficult due to the discrimination associated with HIV. The objective of this study was to analyze factors leading to self-disclosure of HIV-positive status within a sample of persons of both sexes attending different healthcare services in Burkina Faso.MethodologyCross-sectional study conducted by interviewing 740 patients in 26 healthcare services. Univariate (Chi² test) and multivariate (logistic regression) analyses were performed. The significance level was 5%. Qualitative data on factors associated with self-disclosure of HIV-positive status were analyzed.ResultsThe majority of the patients (81.4%) informed at least one person who was very often a close relative (descendant, ascendant and sibling) or the partner. At multivariate analysis, HIV-serostatus was associated with using antiretroviral treatment, (OR = 0.40, 95% CI: 0.3–0.7, P < 0.001), known HIV-serostatus for at least one year (OR = 0.6; 95% CI: 0.4–0.9), living in couple (2.3; 95% CI: 1.4–3.8).ConclusionIn a context limiting HIV testing due to the fear of social stigma, these results appear to be in favor of the Voluntary Counseling Testing model with a focus on the couple and/or families.     

Standard values and relationship-specific validity of the Bielefeld Relationship Expectations Questionnaire (BFPE)

Background  

The Bielefeld Partnership Expectations Questionnaire (BFPE) is a tool to assess attachment in the romantic relationships of adults. The attachment styles are operationalized as configuration patterns of scale scores. While convergent validity has already been investigated, discriminant validity is still lacking confirmation.     

Expression and prognostic significance of Bcl-2 family proteins in myelodysplastic syndromes

Excessive apoptosis is implicated in the pathogenesis of myelodysplastic syndromes (MDS). We assessed by flow cytometry the expression of several members of the Bcl-2 family in bone marrow mononuclear cells (BMMNC) of 168 MDS samples at diagnosis. The proteins studied were Bcl-2, Bcl-xL (anti-apoptotic), Bax, Bad, Bak, and Bcl-xS (pro-apoptotic). The percentage of BMMNC expressing Bcl-2 and Bcl-xL was higher in refractory anemia with excess of blasts (RAEB), RAEB in transformation (RAEB-T), and chronic myelomonocytic leukemia (CMML) than in refractory anemia (RA) and RA with ringed sideroblasts (RAS). Conversely pro-apoptotic proteins Bad, Bak, and Bcl-xS were detected in a higher percentage of cells in RA and RAS. RA and RAS were associated with an increased Bcl-xS/Bcl-xL ratio. The expression of anti-apoptotic proteins was also correlated with that of CD34 and P170 and with the percentage of blast cells. Two-color analyses demonstrated that CD34 and Bcl-2 were usually expressed in the same cells. No significant correlation was found with cytogenetic abnormalities. Higher expression of pro-apoptotic Bcl-2-family proteins (Bak, Bad, Bcl-xS) and higher Bcl-xS/Bcl-xL ratio were associated with longer survival and decreased risk of leukemic transformation in univariate analysis, whereas expression of anti-apoptotic proteins was associated with decreased survival. Consequently Bcl-2 proteins expression was well correlated with the International Prognostic Scoring System (IPSS). Our data confirm that the control of apoptosis is deregulated in MDS cells. Moreover, the study of markers such as CD34 (or Bcl-2), Bcl-xL, and Bcl-xS provides additional prognostic information.     

Improvement of voluntary quadriceps muscle activation after total knee arthroplasty

OBJECTIVE: To evaluate the maximal voluntary contraction (MVC) force and the voluntary activation of the quadriceps femoris muscle in patients with knee osteoarthritis (OA) before and after total knee arthroplasty (TKA). DESIGN: A prospective intervention study. SETTING: University hospital clinic in Germany. PATIENTS: Fifty patients (32 women, 18 men; mean age +/- standard deviation, 65.8+/-5.6 y) with knee OA and 23 healthy age- and gender-matched control subjects. INTERVENTION: Unilateral TKA without patella resurfacing. MAIN OUTCOME MEASURES: Voluntary activation, MVC, and true maximal contraction forces of the bilateral quadriceps femoris muscles, using the twitch interpolation technique before and 33+/-8 months after TKA. Assessment of postoperative knee pain by the Lewis score. RESULTS: Voluntary activation increased bilaterally after surgery (P<.01 operated side, P=.02 nonoperated side) but remained lower than the voluntary activation of the controls. MVC (P<.001) and true maximal contraction forces (P=.01) increased significantly on the operated side. MVC remained unchanged (P=.45), and true maximal contraction forces decreased significantly (P=.04) on the nonoperated side. CONCLUSION: Patients with knee OA have significant bilateral voluntary activation deficits that are, at least in part, reversible within 3 years after TKA. Rehabilitation programs immediately after TKA should focus on reduction of voluntary activation deficits. After voluntary activation improves, physical therapy should target the augmentation of quadriceps femoris muscle strength.     

Long-term ophthalmic health care in Usher Syndrome type I from an ICF perspective

Purpose. The aim was to explore ophthalmic health care in female patients with Usher Syndrome type I (USH I) over 20 years and to evaluate the relationship between the ophthalmic health care and the health state of the patients from a health perspective.

Methods. A retrospective study of records from ophthalmology departments (OD) and low vision clinics (LVC) from 1985 to 2004. Assessment of the reports was performed based on the International Classification of Functioning, Disability and Health (ICF). Findings were analysed by manifest content analysis with ICF as a framework and using four themes: health care system, procedure examinations, patient's functioning and disability and procedure actions.

Results. The records of nine female patients (aged 25–39 years, 1985) with USH I were selected from the national database of USH. A great number of notes were collected (OD 344 and LVC 566). Procedure examinations were exclusively oriented towards body structure and function. All patients showed aggravated visual impairment over and above the hearing and vestibular impairment. Procedure actions were oriented towards environmental factors. No correlation was found between procedures performed and patient's experience of disability.

Conclusions. The high degree of resource allocation was not correlated to the patients' impairment. The study indicates that the ophthalmic health care was characterised by inefficiency. This conclusion is very serious because patients very likely face severe disability and emotional difficulties. ICF is ought to be incorporated in ophthalmic health care strategy to improve the health care.     

A randomized controlled trial of R‐salbutamol for topical treatment of discoid lupus erythematosus

Background In a recent open pilot trial, R‐salbutamol sulphate, a well‐known molecule with anti‐inflammatory effects, was tested successfully on patients with therapy‐resistant discoid lupus erythematosus (DLE). Objectives To compare the efficacy and safety of R‐salbutamol cream 0·5% vs. placebo on DLE lesions in a multicentre, double‐blinded, randomized, placebo‐controlled phase II trial. Methods Thirty‐seven patients with at least one newly developed DLE lesion were randomized – 19 to the R‐salbutamol cream 0·5% and 18 to placebo – and treated twice daily for 8 weeks. Efficacy was evaluated through scores of erythema, scaling/hypertrophy and induration as well as pain and itching; general improvement scored by the investigator and global improvement scored by patients’ assessment were also evaluated. Results The mean area under the curve of improvement for scaling/hypertrophy, pain, itching and global patient assessment was significantly better for the actively treated patients as compared with placebo (scaling/hypertrophy, P = 0·0262; pain, P = 0·0238; itching, P = 0·0135; global patient assessment, P = 0·045). Moreover, the percentage of patients without induration was significantly higher in the active group compared with the placebo group (P = 0·013), and a statistically significantly greater decrease in the size of the lesional area was also seen in the overall analysis of the R‐salbutamol‐treated patients (P = 0·0197). No serious adverse events were reported. Conclusions Application of R‐salbutamol cream 0·5% was safe and well tolerated. Statistically significant effects were seen on scaling/hypertrophy, induration, pain and itching as well as patient global assessment, suggesting that R‐salbutamol could be a promising new topical therapy alternative for DLE.     

Chromosome anomalies in brain tumors]

The authors review the literature concerning chromosomal abnormalities in childhood brain tumors: medulloblastomas, ependymomas, and gliomas of low and high grade. Though these technics require further development, they highlight the role of cytogenetic modification in oncogenesis of brain tumors.