Factors influencing the quality of life of Moroccan patients with juvenile idiopathic arthritis.

The aim of our study is to investigate the factors influencing the quality of life, assessed by the Pediatric Quality of Life Inventory 4.0 (PedsQL4) Generic Score Scales, in Moroccan patients with juvenile idiopathic arthritis. This is a cross-sectional study conducted between January and June 2012, covering children with juvenile idiopathic arthritis (JIA) seen at the consultations of El Ayachi Hospital and Children's Hospital of the University Hospital of Rabat. Quality of life is assessed by the PedsQL4 which is a questionnaire composed of 23 items, completed by the child and the parent; the response to each item ranges from 0 to 100, so that higher scores indicate a better quality of life. The functional impact is assessed by the Childhood Health Assessment Questionnaire (CHAQ), and the disease activity by the number of tender and swollen joints, visual analogue scale (VAS) activity, erythrocyte sedimentation rate (ESR), and C-reactive protein. Forty-seven patients are included; the average age of the patients is 11 ± 3.35 years, and 40.4 % are females, with a median disease duration of 4 (2; 6) years. The oligoarticular form presents 26.7 %, the systemic form 24.4 %, and the enthesic form 22.2 %. The median of PedsQL4 is 80.43 (63.19; 92.93), and the median of the CHAQ is 0 (0; 1). Our study shows that some clinical and biological characteristics have significant effects on PedsQL by both parent and child reports. This study suggests that the achievement of the quality of life of our patients with JIA depends on the disease activity measured by swollen joints, the number of awakenings, parent VAS, physician VAS, patient VAS, and the ESR.

Sleep and its relationship to pain, dysfunction, and disease activity in juvenile idiopathic arthritis.

The objective of this study was to determine the sleep abnormalities that may exist in Moroccan children with juvenile idiopathic arthritis (JIA) and their relationship to pain, dysfunction, and disease activity. Case control study including 47 patients diagnosed with JIA, according to the criteria of the International League of Associations for Rheumatology (ILAR), and 47 healthy children, age and sex matched. Sleep was assessed by Children's Sleep Habits Questionnaire (CSHQ). All parents have filled the 45 items of the CSHQ and grouped into eight subscales: bedtime resistance, sleep onset delay, sleep duration, sleep anxiety, sleep-disordered breathing, night awakenings, parasomnias, and morning awakening/daytime sleepiness. The disease activity was assessed by the number of painful joints, swelling joints, erythrocyte sedimentation rate, c-protein reactive, and Juvenile Arthritis Disease Activity Score (JADAS). Functional assessment was based on the value of Childhood Health Assessment Questionnaire. Pain was assessed by visual analog scale pain. Forty-seven patients were included, with 28 males (59.6 %). Children with JIA had a total score of CSHQ significantly higher than the control cases (p < 0.0001); significant differences were also found in the subscale sleep onset delay, sleep anxiety, sleep-disordered breathing, night awakenings, and parasomnias with a p value of <0.0001, 0.034, <0.0001, 0.001, and 0.00, respectively. Significant association was found between the CSHQ total score and visual analog scale (VAS) physician activity (p = 0.016) and JADAS (p = 0.05). There was a correlation between the sleep-disordered breathing and JADAS (p = 0.04). Sleep onset delay was associated with VAS patient pain (p = 0.05), as nocturnal awakenings and VAS patient pain (p = 0.016). Finally, parasomnias and physician's VAS activity (p = 0.015) and VAS patient pain (p = 0.03) were also correlated. This study suggests that sleep abnormalities are common in children with JIA. Strategies to improve sleep should be studied as a possible tool of improving the quality of life of children with rheumatic disease.

Sexual maturation in Moroccan patients with juvenile idiopathic arthritis.

Abnormal puberty is often reported in children suffering from many chronic diseases. Juvenile idiopathic arthritis (JIA) is the most common joint disorder in developing children. The aim of this study was to assess sexual maturation of Moroccan children with JIA and to compare the development of secondary sexual characteristics in children with JIA to children in the general population. Forty children with JIA and 74 healthy controls were included in a cross-sectional study. The diagnosis of JIA was made according to the criteria of the International League of Association of Rheumatology. Every child was examined for the development of genitalia as per criteria given by Tanner. The children with JIA were also divided into 3 groups: pre-puberty (stage 1), puberty (stages 2-3) and post-puberty (stage 4-5), and the association between puberty and cumulative dose of steroids, disease duration, disease activity, height, weight and age was investigated. Forty children with JIA were included (22 male, 18 female); the mean of age of the patients was 11 ± 4.23 years. Puberty in the patients (mean of tanner 2.43 ± 1.36) was lower than controls (2.55 ± 1.36). The prevalence of the children in prepuberty was of 15 (37.5 %) and 8 (20 %) in postpuberty. The prevalence of the children having a delayed puberty was of 6 (15 %) versus 1(1.4 %) in healthy controls (p = 0.005). There was an association between dose of corticosteroids, age at the administration of corticosteroids and the delayed puberty in boys (p = 0.009). In addition, there was no significant association in both sex between this poor puberty and duration of JIA (p = 0.45 in boys and p = 1.99 in girls) and its activity calculated by the DAS28 (p = 0.73 in boys and p = 1). Our study suggests that the puberty is retarded in Moroccan patients with JIA comparing to healthy children and that the dose of corticosteroid and the age at its administration may contribute to delayed puberty in boys.

The uveitis and its relationship with disease activity and quality of life in Moroccan children with juvenile idiopathic arthritis.

The aim of our study is to investigate ocular involvement in juvenile idiopathic arthritis (JIA) and its relationship with disease activity and quality of life in Moroccan patients who suffer from JIA. This is a cross-sectional study conducted between January and June 2012 which includes patients with juvenile idiopathic arthritis (n = 30). All patients have undergone clinical and paraclinical assessment of JIA and a complete eye examination. Functional impairment is assessed by the Childhood Health Assessment Questionnaire while visual function is studied by the Effect of Youngsters' Eyesight in Quality of Life instrument (EYE-Q). Quality of life is assessed using the Pediatric Quality of Life Inventory Version 4.0 (PedsQL 4.0). Four patients (13.33 %) have uveitis with a confidence interval between 3.4 and 30.7. Involvement is bilateral in three children (75 %). One patient (25 %) has elevated intraocular pressure with loss of the right eye due to glaucoma. There is a strong but not significant relationship between uveitis and the number of awakenings (r = 0.71, p = 0.69) and morning stiffness (r = 3.05, p = 0, 21). This relationship is moderate with erythrocyte sedimentation rate (r = 0.48, p = 0.78) and C-reactive protein (r = 0.25, p = 0.88). A strong but not significant association is found between the overall quality of life assessed by the PedsQL 4.0 and visual function assessed by EYE-Q in the uveitis group (r = -0.64, p = 0.55). This study suggests that uveitis associated with JIA can present serious complications and could have a direct relationship with the activity of the JIA as well as with the quality of life of the patient.

[Fibroblastic rheumatism: a case report]. / Rhumatisme fibroblastique: à propos d'un cas.

UNLABELLED: Fibroblastic rheumatism is a rare entity. Nineteen cases were reported in the literature, and among them, only one in a child. CASE REPORT: Cam. (born August 19, 1988) had an onset of disease in October 1996 with nodules on the MCP and PIP, elbows and tibia, with partial improvement after three months. In April 1997, she suffered from arthralgia and stiffness of both wrists, and then of the big toes. X-rays showed destructive and erosive lesions on both wrists and on the PIP of the second and third fingers and the big toes. Laboratory investigations disclosed normal values for ESR and CRP and negative results for ANA and RF. The diagnosis of fibroblastic rheumatism was based on the typical histologic pattern of a nodule. The treatment associated colchicin and rehabilitation. In August 1998, the wrists' stiffness began to improve, though the big toes remained totally stiff. The radiologic erosive lesions did not show progress. COMMENTS: The diagnosis of fibroblastic rheumatism is based on the histologic pattern of the nodules. The erosive evolution of the arthropathies is infrequent (8/15 cases in adults). Juvenile onset is very rare; only one case has been reported, in a 10-year-old boy. The mechanism of the disease remains unknown. As it is very rare, the therapeutic strategies are not well established. CONCLUSION: This disease should be considered among the causes of juvenile arthritis with erosion.

[Anomalous origin of the left coronary artery from the pulmonary artery: report of 4 cases]. / Anomalie de naissance de la coronaire gauche à partir de l'artère pulmonaire: à propos de quatre observations.

Ahomalous origin of the left coronary artery from the pulmonary artery is a rare but severe congenital cardiopathy that often causes myocardial infraction within the first months of birth, often with congestive heart failure. We report four cases, three boys and one girl. The appearance of the first symptoms is early, usually between the second and the fifth month. The chest X-ray showed cardiomegaly. Echocardiography showed dilated and hypokinesis cardiomyopathy. In all patients the diagnosis was suspected because the standard electrocardiogram showed a constant deep Q wave in leads I and aVL. Angiography with coronarography was realized in only two cases confirming the diagnosis. The left coronary was directly reimplanted into the aorta in one child at 12 months, he has improved throughout six years. Two infants died after four and six years; one child had a spontaneous progressive recovered normal ventricular function because he had an important collateral circulation from right coronary artery. In conclusion, this rare ischemic myocardiopathy is serious because it's life threatening. The indirect signs given by the electrocardiogram and the echocardiography are of a big diagnosis aid. The prognosis depends on the precocity of the chirurgical reimplantation.