Psychometric evaluation of the Moroccan version of the Bath Ankylosing Spondylitis Functional Index (BASFI) and Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) for use in patients with ankylosing spondylitis

The objectives of this study are to translate, adapt in the Moroccan cultural context, and validate in patients with ankylosing spondylitis (AS) the Bath Ankylosing Spondylitis Functional Index (BASFI) and Bath Ankylosing Spondylitis Disease Activity Index (BASDAI). The cross-cultural adaptation of the BASFI and BASDAI was obtained in accordance with the guidelines for translation of the health status measures. Eighty-five patients with AS were included in the study. The test–retest reliability and the internal consistency were analyzed, and both questionnaires were assessed for external construct validity. Structural validity was analyzed with correlation matrix. Twenty-four-hour test–retest reliability was good: BASFI intraclass correlation coefficient (ICC) = 0.96 (confidence interval (CI) at 95%, 0.93–0.97), BASDAI ICC = 0.93 (CI at 95%, 0.90–0.95). Cronbach’s alpha was 0.90 for the BASFI and 0.86 for BASDAI. The construct validity of the instruments was evaluated. The BASFI showed a strong validity when correlating its results with Schober’s test (r = −0.56), occipital wall distance (r = 0.46), chest expansion (r = −0.46), BASDAI (r = 0.54), Bath Ankylosing Spondylitis Metrology Index (r = 0.70), Bath Ankylosing Spondylitis Global Score (BAS-G; r = 0.58), Bath Ankylosing Spondylitis Radiology Index (r = 0.61), and the radiological changes in sacroiliac joints (r = 0.54). A good correlation was observed between the BASDAI and the spinal pain (r = 0.53), the number of nocturnal awakenings (r = 0.57), the morning stiffness (r = 0.65), the enthesic index (r = 0.47), the BAS-G (r = 0.53), the BASFI (r = 0.54), and the erythrocyte sedimentation rate (r = 0.41; for all p < 0.001). The correlation matrix showed an intermediate correlation between items. The Moroccan version of the BASFI and the BASDAI showed adequate reliability and validity. These instruments can be used in the clinical evaluation of Moroccan and Arabic-speaking patients with AS.     

Measuring outcomes of nursing care, improving the health of Canadians: NNQR (C), C-HOBIC and NQuiRE

The purpose of this paper is to inform the nursing community of the extraordinary progress that the Canadian National Nursing Quality Report (NNQR(C)), the Canadian Health Outcomes for Better Information and Care (C-HOBIC) and the Nursing Quality Indicators for Reporting and Evaluation (NQuiRE) have made to date, and to share our commitment to continue working together to build a strong nursing profession that, armed with evidence, will contribute to healthier Canadians.     

Health related quality of life survey about children and adolescents with juvenile idiopathic arthritis

This study aimed to investigate the proxy-reported Health related quality of life (HRQOL) and its determinants in patients with juvenile idiopathic arthritis (JIA). It was hypothesized that HRQOL would decrease with worsening disease and disability. Data were available in cross-sectional study on children and adolescents with JIA according to the ILAR criteria. Patient demographics, type of JIA, clinical determinants and laboratory parameters relating to JIA were obtained for each patient. Functional disability was assessed using the parent’s or children’s version of the child health assessment questionnaire (CHAQ). The HRQOL was evaluated using the juvenile arthritis quality of life questionnaire (JAQQ). These questionnaires were previously translated and validated in Moroccan children. A total of 80 participants were enrolled with mean age of 11 [6–17 years], and female predominance (59%). Many patients (42.5%) had oligoarticular subtype; 31.3% polyarticular subtypes and 26.2% systemic form. The mean global score of JAQQ was 2.6 ± 1.3 (1–6). Patients with persistant oligoarticular had better gross motor function (P < 0.0001), better fine motor function (P < 0.0001), less psychosocial impact (P = 0.001), and less symptoms (P = 0.001) in comparison with polyarticular and systemic subtypes. The HRQOL assessed by the JAQQ was worse in adolescent patients in comparison with children except for symptoms (P = 0.15). The gender (P = 0.95), age at onset of JIA (P = 0.81), and evolution duration (P = 0.34) were not correlated with global score of JAQQ. The diagnosis delay was significantly associated with decrease of HRQOL (P = 0.001). The decrease of HRQOL was correlated with disease activity [pain (VAS), painful and swollen joints, erythrocyte sedimentation rate (for P < 0.0001)], with disability index (CHAQ) (P = 0.001) and presence of hip involvement (P = 0.01). This study suggests that JIA can have a significant adverse effect on the HRQOL of moroccan patients, particularly adolescents with polyarticular and systemic subtypes. Disease duration, disability score (CHAQ) and pain were the strongest determinants of poorer HRQOL.     

Brucella Prosthetic Valve Endocarditis: A Systematic Review

ObjectiveBrucella prosthetic valve endocarditis is a rare but a life-threatening complication of brucellosis. It remains a diagnostic challenge. Optimal treatment of Brucella prosthetic valve endocarditis is debated. Available data is limited to case reports or small case series. The purpose of this study was to systematically review all published cases of Brucella prosthetic valve endocarditis in the literature.MethodA systematic review of PubMed database, Google, Google Scholar, and Scopus (From January 1974 to the present) for studies providing epidemiological, clinical and microbiological data as well as data on treatment and outcomes of Brucella prosthetic valve endocarditis was performed.ResultsA total of 51 reported cases were reviewed. Brucella melitensis (45%) and Brucella abortus (11.7%) were the most frequently isolated species. Most common type of prosthesis valve was mechanical prothesis (84.3%) and ten patients had double valve prosthesis (19.6%). Fever and dyspnea were present in 100% and 37.2% of the cases, respectively. The diagnosis was set with echocardiographic finding in 30 cases (93.7%), which revealed vegetation in 27 cases (84.3%). Most used antibiotics were rifampicin, doxycycline and aminoglycoside or cotrimoxazole. No deaths were noted in patients treated by combined medical and surgical treatment, but mortality was noted in 27.7% of the cases treated by antibiotics alone (p = 0.006).ConclusionThis systematic review highlights diagnostic challenges and demonstrates that surgery improved outcome by reducing mortality in patients treated with the combined surgical and medical treatment option. Brucellosis should be considered in the differential diagnosis of prosthetic valve endocarditis in patients residing in or traveling to areas of endemicity.     

Hepatocellular carcinoma in Gaucher disease: an international case series

Gaucher disease (GD) is associated with an increased risk for malignancies. Next to hematological malignancies, the development of solid tumors in several organs has been described. The liver is one of the major storage sites involved in GD pathogenesis, and is also affected by liver-specific complications. In this case series, we describe 16 GD type 1 (GD1) patients from eight different referral centers around the world who developed hepatocellular carcinoma (HCC). Potential factors contributing to the increased HCC risk in GD patients are studied. Eleven patients had undergone a splenectomy in the past. Liver cirrhosis, one of the main risk factors for the development of HCC, was present in nine out of 14 patients for whom data was available. Three out of seven examined patients showed a transferrin saturation?>?45%. In these three patients the presence of iron overload after histopathological examination of the liver was shown. Chronic hepatitis C infection was present in three of 14 examined cases. We summarized all findings and made a comparison to the literature. We recommend that GD patients, especially those with prior splenectomy or iron overload, be evaluated for signs of liver fibrosis and if found to be monitored for HCC development.     

3-Dimensional Echocardiography: Latest Developments and Future Directions

The ongoing refinements in 3-dimensional (3D) echocardiography technology continue to expand the scope of this imaging modality in clinical cardiology by offering new features that stem from the ability to image the heart in its complete dimensionality. Over the years, countless publications have described these benefits and tested new frontiers where 3D echocardiographic imaging seemed to offer promising ways to improve patients’ care. These include improved techniques for chamber quantification and novel ways to visualize cardiac valves, including 3D printing, virtual reality, and holography. The aims of this review article are to focus on the most important developments in the field in the recent years, discuss the current utility of 3D echocardiography, and highlight several interesting future directions.     

The management of familial hypercholesterolaemia in Morocco

Familial hypercholesterolaemia (FH) is an autosomal dominant disorder characterized by high levels of low-density lipoprotein cholesterol (LDL-C) as a result of mutations that impair their removal from plasma.The clinical consequence is a high risk of premature cardiovascular disease. Because of the extreme risk of mortality and morbidity, diagnosis, recruitment and management of FH patients must be one of the priorities of public health. In Morocco, specialized consultation for dyslipidaemia and strategy for management of this cardiovascular major risk factor does not exist, making FH identification and management difficult. In this review, we present the first FH state of the art in our country through a sample of 66 subjects. By this analysis, we have tried to elucidate some points that impede the identification and recruitment of heterozygous FH and the management of both heterozygous and homozygous FH in Morocco. Also, we have attempted to propose some strategies for an adequate management of FH in our country, taking into account the local specifications.     

Dynamic history of low-grade gliomas before and after temozolomide treatment

OBJECTIVE: To evaluate the natural progression and the impact of temozolomide in low-grade gliomas and to correlate these changes with the profile of genetic alterations. METHODS: The mean tumor diameter (MTD) of low-grade gliomas was evaluated on serial magnetic resonance images before (n = 39), during, and after (n = 107) treatment with neoadjuvant temozolomide. MTD growth curves were correlated with chromosomes 1p-19q loss and p53 overexpression in the tumors. RESULTS: Before temozolomide onset, MTD increased linearly over time, indicating a continuous growth that was significantly slower in 1p-19q deleted tumors (3.4 vs 5.9mm/year; p = 0.0016) and in tumors that did not overexpress p53 (4.2 vs 6.3mm/year; p = 0.05). During temozolomide treatment, almost all patients (92%) experienced initial decrease of MTD. Subsequently, some tumors started to resume growth despite continuous administration of temozolomide, with a lower rate of relapse in 1p-19q deleted tumors (16.6 vs 58%; p = 0.0004) and in tumors that did not overexpress p53 (26 vs 68%; p = 0.003). When temozolomide was discontinued in the absence of tumor progression, a majority of tumors resumed their progressive growth within a year. INTERPRETATION: Untreated low-grade gliomas grow continuously at a rate that is influenced by the genetic alterations of the tumors. Temozolomide reverses this pattern at the onset, but this effect is often brief in patients whose tumors overexpress p53 and do not harbor the 1p-19q codeletion, suggesting acquired chemoresistance. A majority of tumors will resume their growth when treatment is discontinued, raising the issue of the optimal duration of treatment in continuously responding patients.