Living with a child with familial Mediterranean fever: does it affect the quality of life of the parents?

OBJECTIVE: The aim of the present study was to assess the quality of life (QOL) and the psychological status of parents of children with familial Mediterranean fever (FMF). METHODS: The QOL, anxiety and depression of the parents of 35 children with FMF were evaluated and compared to the parents of 23 healthy children. RESULTS: Mothers of FMF children had lower QOL scores than mothers of healthy children: 5.5 +/- 1.1 versus 6.0 +/- 0.6 (p = 0.048). They also expressed higher levels of anxiety and depression. Within each group, mothers were more anxious and depressed than fathers. Parents with several FMF children were not significantly different from parents with only one FMF child. CONCLUSION: The QOL and psychological well being of parents with FMF children were found to be slightly impaired, especially that of the mothers.     

Aetiological role of bacteria associated with reactive arthritis in pauciarticular juvenile chronic arthritis.

BACKGROUND: The cause of juvenile chronic arthritis (JCA) is unknown. Pauciarticular JCA is the most common subtype and can be subdivided into early (type I) and late onset (type II) forms, the latter clinically resembling reactive arthritis. METHODS: The cellular immune responses to bacteria associated with reactive arthritis in blood and synovial fluid from 39 children with pauciarticular JCA, three children with classical reactive arthritis, and two children with psoriatic arthritis were examined. Specific titres of antibodies to bacteria in serum samples were measured in all patients. RESULTS: A bacteria specific synovial cellular immune response was found in two of three (67%) patients with reactive arthritis and 14 of 28 (50%) patients with pauciarticular JCA type II but only in one of 11 (9%) patients with pauciarticular JCA type I and none in patients with psoriatic arthritis. Six patients responded specifically to Chlamydia trachomatis and 11 to Yersinia enterocolitica. Antigen specific lymphocyte proliferation correlated poorly with the specific antibody response. CONCLUSIONS: These findings suggest that bacteria with associated reactive arthritis may have a causative role in pauciarticular JCA type II but not in JCA type I.     

Tumour necrosis factor alpha and its soluble receptors in juvenile chronic arthritis

OBJECTIVE: To identify possible imbalance of tumour necrosis factor alpha (TNFalpha) and its soluble receptors in the different subgroups of juvenile chronic arthritis (JCA). METHODS: Serum and synovial fluid samples from 45 children were examined, 25 pauciarticular JCA, 13 polyarticular JCA and seven spondyloarthropathy. TNFalpha, sTNFRI and sTNFRII levels were measured by EASIA and enzyme-linked immunosorbent assay (ELISA). Analysis of the results was carried out using non-parametric tests: Kruskal-Wallis one-way analysis of variance was used to compare the three clinical subgroups; the Mann-Whitney U-test was used to compare group medians. RESULTS: Thirty-three serum samples were assayed for TNFalpha. There was no significant difference between the three groups using the Kruskal-Wallis analysis of variance. Analysis of synovial fluid TNF levels showed significantly lower levels in the spondyloarthropathy group compared with the pauciarticular JCA (P = 0.01) and the polyarticular group (P = 0.002). Significantly higher levels of sTNFRI were observed in the synovial fluid of the polyarticular JCA group compared with the pauciarticular JCA group (P = 0.004) and similarly for sTNFRII (P = 0.03). Molar ratios were calculated for TNF vs sTNFRI. The sTNFRI/TNFalpha ratio was significantly higher in the spondyloarthropathy group compared with the pauci- (P 0.003) and the polyarticular JCA subgroups (P = 0.003). The combined soluble receptor levels expressed as molar ratio to TNF again showed a significantly higher ratio in the spondyloarthropathy group compared with the pauciarticular group (P = 0.01) and compared with the polyarticular group (P = 0.05). CONCLUSION: These results suggest that the increased joint destruction observed in polyarticular disease compared with the other two subtypes may be related to the lower sTNFR/TNFalpha ratios observed.     

Autoantibodies in chronic arthritis of childhood: relations with each other and with histocompatibility antigens.

BACKGROUND: Studies have shown the presence of either antibodies to histone or anticardiolipin antibodies in some forms of childhood chronic arthritis. The relation between these autoantibodies has not been previously reported, however, and the immunogenetics of their association with childhood arthritis has not been studied. METHODS: The interrelation of fluorescent antinuclear antibodies, antibodies to histone, and anticardiolipin antibodies and their associations with histocompatibility antigens (HLA) were studied in 114 children with chronic arthritis (45 children with pauciarticular onset juvenile chronic arthritis (JCA), 22 with polyarticular onset JCA, 13 with systemic onset JCA, and 34 with juvenile psoriatic arthritis (JPsA). Antibodies to histone and anticardiolipin antibodies were determined in 108 children. HLA antigens (A, B, C, and DR) were studied in the 83 white children. RESULTS: Antibodies to histone occurred in 0% (systemic onset JCA) to 42% (uveitis negative, pauciarticular onset JCA), and anticardiolipin antibodies in 26% (JPsA) to 55% (polyarticular onset JCA) of patients. Only 12 patients (11%) had both antibodies to histone and anticardiolipin antibodies. Neither antibodies to histone nor anticardiolipin antibodies associated with the type of arthritis. Neither of these antibodies alone associated with uveitis. Antibodies to histone were associated with HLA-A2, probably reflecting the known association of HLA-A2 with pauciarticular onset JCA. There was no other HLA association. Fluorescent antinuclear antibodies occurred most often in patients with uveitis; however, the occurrence of fluorescent antinuclear antibodies in patients with pauciarticular onset JCA (the group most at risk for uveitis) was not significantly greater in children with uveitis than in those without uveitis (100 and 88% respectively). CONCLUSIONS: Although antibodies to histone and anticardiolipin antibodies often occur in serum samples from patients with JCA and JPsA, they rarely occur together. Their presence does not associate with uveitis. This study did not show any strong evidence that production of either antibodies to histone or anticardiolipin antibodies in patients with JCA or JPsA is under the control of the histocompatibility locus.     

Nutrient intake and nutritional status in children with juvenile chronic arthritis.

Nutrient intake and nutritional status were assessed in 15 children with juvenile chronic arthritis (JCA) and in 17 healthy controls. Anthropometric measurements were similar in children with pauciarticular JCA and in controls, whereas weight (p = 0.05) and upper arm muscle area (UAMA) (p less than 0.01) were reduced in children with polyarticular JCA. Compared with healthy controls the concentrations of hemoglobin, serum iron and serum zinc were reduced in the children with polyarticular JCA (p less than 0.01) and serum copper was increased (p less than 0.01). In the patients the concentrations of hemoglobin, serum iron and serum zinc correlated negatively with erythrocyte sedimentation rate (ESR), whereas serum copper correlated positively. Impaired nutritional status was found in the children with polyarticular JCA in spite of increased energy and protein intake. In this group of patients the dietary intake of calcium was also found to be reduced.     

CLINHAQ Scale – Validation of a Hebrew Version in Patients with Fibromyalgia

Assessment of health status in patients with rheumatic disease, including fibromyalgia (FM), using structured questionnaires has become an important approach to evaluate treatment and outcome. The objectives of this study were to validate a translated version of the Clinical Health Assessment Questionnaire (CLINHAQ) to be used by Hebrew-speaking populations, and specifically to evaluate its usefulness in fibromyalgia syndrome (FM). The CLINHAQ was translated into Hebrew and administered to 90 women with FM along with the Hebrew versions of the Fibromyalgia Impact Questionnaire (FIQ) and the Quality of Life (QOL) Scale. The CLINHAQ includes scales of functional disability, helplessness, anxiety and depression, as well as assessment of current health status and satisfaction with this. All subjects were asked about the presence and severity (assessed by visual analogue scale) of current FM symptoms (pain, fatigue, anxiety etc.); a count of 18 tender points was conducted by thumb palpation, and tenderness thresholds were measured by dolorimetry. Test–retest reliability was assessed by Pearson correlation coefficients, and internal consistency was evaluated with Cronbach’s α coefficient of reliability. Construct validity was tested by correlating the CLINHAQ items with measures of symptom severity, count of tender point, tenderness thresholds, physical functioning measured by FIQ, and with a score of QOL. Test–retest reliability coefficients ranged from 0.82 to 0.99, and Cronbach’s α coefficients from 0.725 to 0.929. Significant moderate to high correlations were obtained between most subscales of CLINHAQ and measures of physical functioning, quality of life and severity of FM symptoms. In conclusion, the CLINHAQ is a reliable and valid instrument for measuring health status and physical functioning in Israeli women with FM. Received: 22 June 1999 / Accepted: 24 September 1999     

Long-term psychological distress, and styles of coping, in parents of children and adolescents who underwent invasive treatment for congenital cardiac disease

OBJECTIVE: To assess the level of psychological distress and styles of coping in both mothers and fathers of children who underwent invasive treatment for congenital cardiac disease at least 7 years and 6 months ago. METHODS: The General Health Questionnaire and the Utrecht Coping List were completed by parents of children with 4 different cardiac diagnoses. RESULTS: Overall, in comparison with reference groups, parents of children treated for congenital heart disease showed lower levels of distress, manifested as lower levels of somatic symptoms, anxiety and sleeplessness and serious depression. Mothers of children with congenital heart disease reported significantly more somatic symptoms than fathers.Further, compared to reference groups more favourable outcomes on coping were found; parents in our sample showed a weaker tendency to use styles of coping such as reassuring thoughts and less often expressed negative emotions (anger, annoyance). Mothers in our sample appeared to seek social support more often compared to fathers. CONCLUSION: Overall, lower levels of psychological distress and few differences in styles of coping compared to reference groups were found in parents of children treated for congenital heart disease. We need to remain alert however, for individual parents at risk of adjusting poorly.     

Self-Reported Symptoms among Danish Women following Cosmetic Breast Implant Surgery

The aim of this study was to examine self-reported symptomatology and to identify distinctive characteristics among women with silicone breast implants (SBI). Using the Danish hospital and population registers we identified three groups of women with a hospital diagnosis of muscular rheumatism (a non-specific soft-tissue diagnostic code) who had previously undergone SBI surgery (n= 28), breast reduction surgery (n= 29) or no breast surgery (n= 27); and three groups of women without a diagnosis of muscular rheumatism who had undergone SBI surgery (n= 21), breast reduction surgery (n= 27) or no breast surgery (n= 56). All study subjects completed a self-administered questionnaire focusing on sociodemographic factors, lifestyle habits, somatic symptoms and psychological symptoms. Women with SBI and women with breast reduction with no previous diagnosis of muscular rheumatism had similar patterns of reporting for most symptoms and characteristics. They reported significantly more somatic symptoms and psychological distress, including somatisation, obsessive–compulsiveness and depression, than women with no breast surgery. No significant differences in self-reported symptomatology and characteristics were observed among the three groups of women with a previous diagnosis of muscular rheumatism. Overall, women with prior muscular rheumatism reported more symptoms than those without. We concluded that self-reported somatic symptoms among women with SBI were similar to those of controls. Women with cosmetic breast surgery appear to have distinctive psychological characteristics. Our study emphasises the importance of taking the psychological profile and previous history of rheumatic diseases into account when examining women with SBI. Received: 28 February 2001 / Accepted: 2 July 2001     

Increased prevalence of spondylarthropathies in parents of children with pauciarticular juvenile chronic arthritis,type 1

Summary Using a standard questionnaire, parents of 70 children with type 1 pauciarticular juvenile chronic arthritis were asked about inflammatory backpain. When the answer was positive, the parents were seen by a rheumatologist who used the preliminary criteria of the European Spondylarthropathy Study Group to decide whether the parent had spondylarthropathy or not. A diagnosis of spondylarthropathy was established in 6 out of 138 parents (4%). This is considerably higher than the expected prevalence of spondylarthropathy, which should not exceed 0.25%. This finding sheds a new light on pauciarticular JCA, type 1, since no adult counterpart has as yet been described.     

Levels of Emotional Distress Among Parents Living with AIDS and their Adolescent Children

This study examined the level and the relationship between emotional distress reported by parents living with AIDS (PLAs) and the emotional distress of their adolescent children. Levels of depression and anxiety were assessed among 268 PLAs and 403 of their adolescent children. Compared to normative clinical samples, emotional distress was significantly higher among PLAs; fathers' reports were in the clinical range. In contrast to their parents, adolescents' reports of emotional distress were similar to those of normative same-age peers. The levels of depression and anxiety reported by female adolescents were significantly higher than those of the male adolescents. The level of adolescents' depression and anxiety was significantly related to their parents' depression. In conclusion, a parental diagnosis of AIDS does not appear to be associated with high rates of emotional distress among their adolescent children.     

LACK OF TEMPORAL ASSOCIATION OF IRIDOCYCLITIS WITH IgG REACTIVITIES TO CORE HISTONES AND NUCLEOSOME SUBPARTICLES IN PAUCIARTICULAR JUVENILE CHRONIC ARTHRITIS

This study aimed to measure IgG reactivities to DNA-free nucleosomesubparticles (H2AH2B, H3H4) and nucleosome subparticles (H2AH2B–DNA,H3H4–DNA) and to evaluate the temporal relation of thesereactivities, as well as those to single core histones, withiridocyclitis (IC) in patients with antinuclear antibody positive(ANA +) pauciarticular juvenile chronic arthritis (JCA). Reactivitiesto nuclear substrates were determined by enzymatic immunoassaysin 120 sera from 45 children with ANA + pauciarticular JCA.Significantly elevated IgG levels to H3 and H4, to DNA-freenucleosome subparticles, and to the nucleosome subparticle H3H4–DNAwere present in patients with ANA + pauciarticular JCA; no evidenceof recognition of conformational epitopes was found. In bothhorizontal and follow-up studies, no relation between the reactivitiesstudied and the development, presence, or history of IC wasfound. Our results show the absence of a relation of antibodiesto histone molecules or to nucleosome subparticles with IC inpatients with ANA+ pauciarticular JCA. KEY WORDS: Juvenile chronic arthritis, Antinuclear antibodies, Iridocyclitis ^*Present address: Laboratorio Biotecnologie e Tecnologie Biomediche,IRCCS Policlinico San Matteo, 27100 Pavia, Italy.     

A subgroup-specific evaluation of the efficacy of intraarticular triamcinolone hexacetonide in juvenile chronic arthritis

OBJECTIVE: To determine the subgroup-specific differences of intraarticular triamcinolone hexacetonide (TH) in the treatment of joint inflammation in patients with juvenile chronic arthritis (JCA). METHODS: A retrospective review of 194 children of all subgroups of JCA, treated by a single or repeated TH injection between 1989 to 1994. Efficacy and duration of benefit were evaluated after a mean duration of 3, 15, 30, and 64 weeks. RESULTS: In all, 1439 TH injections were given to 194 patients; 368 of these were reinjections. The median duration of improvement of all injections was 74 weeks. Responses were significantly different among subgroups (p = 0.0001): there were 121 weeks of efficacy in early-onset pauciarticular JCA type I (223 injections), 47 weeks in late-onset pauciarticular JCA type II (190 injections), 105 weeks in rheumatoid factor negative polyarticular JCA (445 injections), 63 weeks in rheumatoid factor positive polyarticular JCA (127 injections), and 36 weeks in systemic JCA (413 injections). Forty-one injections were done in other rheumatic diseases. In relation to this result there were also differences with regard to joint groups, antinuclear antibody (ANA) and HLA-B27 status, and sex. Side effects were rare: infections of skin or joints were not noted; skin and lipoatrophy were seen after 15 injections, necrosis of the hip in one case, luxation of 2 shoulders of one patient, and periarticular calcification in 3 patients. CONCLUSION: Intraarticular TH is an effective therapy for inflammatory joint disease in all subgroups of JCA. The risk of major complications is low. The median duration of improvement depends on the subgroup of the disease.     

The impact of peer victimization and psychological symptoms on quality of life in children and adolescents with systemic lupus erythematosus

There is no documentation about the association between peer victimization, psychological status, and quality of life (QOL) in children and adolescents with systemic lupus erythematosus (SLE). The aim of this study was to evaluate the association between peer victimization, psychological symptoms, and QOL in a cohort of children and adolescents with SLE. Forty-one patients (aged 9–18 years) participated in this study. The control group (n = 49) was composed of healthy children and adolescents from local community. Questionnaires were used to evaluate the peer victimization, psychological status, and QOL of children and adolescents with and without SLE. No significant difference was found between the study and control groups for peer victimization, depression, state and trait anxiety, and QOL scores. The peer victimization, depression, anxiety, and self-esteem scores were negatively correlated with psychosocial and total subscale scores of QOL in the study group. According to regression analyses, trait anxiety had a negative predictive effect on the physical health domain scores of QOL, whereas trait anxiety and peer victimization had a negative effect on the psychosocial domain and total scores of QOL in the SLE patients. This study suggests that trait anxiety and peer victimization are risk factors for poor QOL in adolescents with SLE.     

Lymphokines and soluble interleukin-2 receptors in juvenile chronic arthritis

Summary Serum levels of interleukin (IL)-2, interferon gamma (IFNg) and soluble IL-2 receptors (sIL-2R) were determined in sera from 34 patients with poly-or pauciarticular juvenile chronic arthritis (JCA) by use of enzyme-linked immunosorbent assays (ELISAs). Levels of sIL-2R were elevated in the group of patients compared with those of healthy children and correlated significantly with several parameters of clinical activity, including the functional capacity, joint score, visual-analogue score and erythrocyte sedimentation rate (ESR). Serum IL-2 levels were also elevated in the JCA patients, correlating with the patients functional capacity. Serum levels of IFNg were below the detection limit of the assay. Our data supported the notion that T-cell activation plays a role in the immunopathologic processes leading to clinical JCA.     

Juvenile Chronic Arthritis in Adult Life: A Study of Long-term Outcome in Patients with Juvenile Chronic Arthritis or Adult Rheumatoid Arthritis

We compared the prognostic factors and outcome of 30 patients with juvenile chronic arthritis (JCA) extending into adult life with those of 30 patients with adult rheumatoid arthritis (RA) at a university adult rheumatology clinic; pairs were matched for sex and duration of disease (mean 8 years). One-third of JCA patients had seronegative polyarticular disease and another third had oligoarticular disease. In a third of the JCA patients, the clinical presentation changed during the follow-up. Over half of the RA patients had seropositive polyarticular and a one-third had seronegative polyarticular disease. Fewer seropositive patients were recorded in the JCA group than in the RA group both at the beginning (16.7% versus 56.7%; p = 0.003) and at the end of the follow-up (14.3% versus 59.3%; p = 0.001). JCA patients developed less radiographic changes than RA patients (46.7% versus 76.7%; p = 0.034); oligoarthritis in the JCA group had the best prognosis whereas seropositive polyarthritis in the RA group had the worst prognosis. Significantly more patients with JCA than RA (60% versus 23%; p = 0.009) were in remission at the end of the follow-up. In conclusion, when studied in adult life, the long-term prognosis is better in patients with JCA than in those with RA. Received: 23 March 1998 / Accepted: 3 November 1998     

Decreased fibrinolytic activity in juvenile chronic arthritis.

The basal fibrinolytic activity in 17 children with active juvenile chronic arthritis (JCA) was investigated. It was found that patients with JCA, and particularly those with the systemic form, show decreased plasma fibrinolytic activity and a marked increase in plasminogen activator inhibitor. Additionally, it was found that patients with systemic JCA, but not those with the polyarticular or pauciarticular form, have increased circulating levels of tissue-type plasminogen activator, and endothelial cell protein, suggesting possible endothelial cell participation in systemic JCA.     

Mothers’ emotional states and attitudes regarding their children with asthma

Objectives: The aim of this study was to investigate the associations of asthma with the psychological profile (depression and anxiety) of children with asthma and their mothers as well as the attitudes of these mothers toward their children and their family relationships. Methods: Sixty-four children with asthma, 60 healthy children and their mothers were included in the study. The Children’s Depression Inventory (CDI) and Childhood Anxiety Sensitivity Index (CASI) were applied to the children. All mothers completed the Beck Depression Inventory, the State-Trait Anxiety Inventory, the Parental Attitude Research Instrument and the Family Assessment Device. Results: CASI scores were significantly higher in children with asthma (p?<?0.001) than in healthy children. The increasing duration and severity of asthma were associated with increasing anxiety levels in children with asthma. Depression and anxiety scores were significantly higher in the mothers of children with asthma than in the comparison group. The mothers of children with asthma did not have supportive and friendly relationships with their children. In addition, these mothers had significantly higher Attitude of Hostility and Rejection, Attitude of Over-parenting and Authoritarian Attitude scores than those of the comparison group. Increasing duration and severity of asthma influenced family functions and the attitude of the mothers of children with asthma. There was a correlation between an increasing number of emergency department visits and increasing depression in the mothers of children with asthma. Conclusions: This cross-sectional study revealed that the disease may negatively affect the lives of children with asthma and their mothers and their family relationships.     

Symptoms of attention deficit hyperactivity disorder and quality of life of mothers of school-aged children: The roles of child,mother, and family variables

This study examined the impact of inattention and hyperactivity symptoms in children and mothers, opposition problems in children, maternal depression, and perceived family support on the quality of life (QOL) of mothers. Mothers of children in one elementary school were contacted. The relationship between sociodemographic variables, the levels of inattention and hyperactivity symptoms in children and mothers, oppositional symptoms in children, maternal depression, perceived family support, and maternal QOL were examined. Three hundred and eighty-two participants were included in this study. Consistent factors related to the mother's QOL in the four domains were maternal depression, perceived family support, and housing status after controlling for several family, maternal, and child variables. Maternal QOL was more related to her own and family factors including maternal inattention, hyperactivity and depression symptoms, perceived family support, and housing status, instead of parent-rated inattention and hyperactivity symptoms of the child. Screening for maternal inattention, hyperactivity and depression symptoms, and mental health services for these mothers are warranted based on these findings.     

Incidence and prevalence of juvenile chronic arthritis: a population survey.

In a population based epidemiological survey of juvenile chronic arthritis (JCA), performed in Western Sweden in 1983, an incidence of 12/100,000 was found. The estimated prevalence was 56/100,000. Subgroup distribution showed a preponderance of mono- and pauciarticular forms. The peak age of onset was between 0 and 4 years of age. Girls predominated over boys in a ratio of 3:2. Overall, 30% were antinuclear antibody (ANA) positive, 9% rheumatoid factor (RF) positive, and eye involvement occurred in 10% of the children. The results suggest differences in population based studies of JCA compared with previously reported hospital based series.     

Differences in mothers' and fathers' psychological distress after pediatric SCT: a longitudinal study

The purpose of this study was to examine longitudinally psychological distress and its correlates in mothers and fathers of children who undergo SCT, up to 2 years post SCT. A total of 111 parents of patients diagnosed mainly with leukemia completed standardized measures of depression and anxiety symptoms as indicators of psychological distress, 85 at 1 year pre-SCT and 81 at 2 years post SCT. Parents' age and gender, child's age, diagnosis, radiation history, behavior and physical health were examined as potential related factors. Linear mixed models for repeated measures with appropriate covariance structure were used in the analysis. Depression and anxiety scores significantly decreased by 2 years for mothers and fathers. Mothers reported significantly more depression symptoms than did fathers, but reported comparable symptoms of anxiety. Pre-SCT depression and anxiety scores, mother's age (younger), child's behavior problems, radiation history and diagnosis of neuroblastoma predicted maternal distress 2 years post SCT; pre-SCT depression and anxiety scores, father's age (older) and child's diagnosis predicted father's distress. This study highlights differences and similarities in mothers' and fathers' psychological distress and identifies related risk factors. The results can guide interventions for mothers and fathers whose children undergo SCT based on their pre-SCT psychosocial risk.