Biomedical subjects
Publications and source records attributed to A Fasth.
A fatal case of the pre-excitation syndrome in a 3 10/12 years old girl with no other heart disease is presented. Her death is thought to be the consequence of ventricular fibrillation. This observation is in contrast to the reported benign course in infants and children with the Wolff-Parkinson-White syndrome without other heart disease.
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Instability of the centromeric region of chromosome 1, and multibranched configurations formed by the short and long arms were seen in a brother and sister with facial dysmorphism, mental retardation and recurrent infections. No chromosomal abnormalities were seen in the parents, who were first cousins. The fragility of chromosome 1 was identified in amniotic fluid cells of the sister. A combined immunodeficiency characterized by a lack of immunoglobulin production, low numbers of T cells and a lack of cells with NK cell markers was diagnosed. This is the first report of familial occurrence of this unique chromosomal aberration. The cause may be an autosomal recessive gene defect.
OBJECTIVE: To investigate pain, coping and health status in children with chronic arthritis. Additional aims were to explore gender variations in pain, coping and health status, and the same factors in children under 16 years versus children 16 years or older. METHODS: For 125 children (43 boys) with a median age of 14.1 years (range 10.3-17.8) disability and discomfort were assessed (Childhood Health Assessment Questionnaire, CHAQ). Pain, coping, fatigue, sleeping and satisfaction with life were evaluated (Visual Analogue Scales). The Pain Intensity Scale was used to assess the variation in pain intensity during one week. RESULTS: The CHAQ disability index (DI) was 0.63. In the total sample, girls had larger numbers of affected joints in upper extremities (p = 0.028) and more days with morning pain (p = 0.025) than boys. Boys reported greater perceived ability to reduce pain than girls (p = 0.020). Compliance with treatment was more frequent in girls than in boys (p = 0.029). In children 16 years of age and older, boys reported greater perceived ability to control pain (p = 0.032) and to reduce pain than girls (p = 0.001) and fewer days with worst pain in the mornings (p = 0.013), as well as less pain variations over the period of a day (p = 0.014) and over a period of a week (p = 0.035). CONCLUSIONS: As a group, the children were clearly affected by their arthritis. Pain was related to the severity of disease. Some significant gender and age differences were highlighted, which should be considered in clinical pain management.
OBJECTIVE: The aim of this study was to describe a model for predicting well-being in children with juvenile chronic arthritis (JCA). METHODS: 125 children (43 boys) (median age 14.1 yrs; range 10.3-17.8) rated disability and discomfort (Childhood Health Assessment Questionnaire). Pain control, pain reduction and fatigue were evaluated (visual analogue scales). In addition, variation of pain intensity was rated by a pain intensity scale. Analysis by the stepwise regression technique was used to explain the variability in well-being. Eight independent variables were included as possible predictors in the model (p < 0.1). RESULTS: The analyses indicated that well-being in children with JCA is related to three clusters of variables; pain "as it normally is", number of pain-free days and attending physical education classes. The analysis explained a substantial portion of the total variance in the children's well-being (55.1%). CONCLUSION: Pain is a robust predictor of well-being in children with JCA. This supports the concept of the benefits of reducing chronic joint pain as a major goal in caring of these children.
In order to adequately care for patients with chronic disorders and to properly allocate resources, the epidemiology of the underlying disease must be know. Proper population based studies involve substantial planning and educational programs, however. To prepare for such a study of pediatric rheumatic disorders, we performed a referral-based pilot study. During an eleven-month period pediatricians all over Costa Rica were asked to refer to us all new cases of possible rheumatic disorders among children less than 16 years of age, using the EULAR criteria for juvenile chronic arthritis. The children were evaluated at the National Children's Hospital. An annual incidence of 5.9 cases of all types of pediatric rheumatic diseases per 100,000 was found. Incidences of 5.4 per 100,000 children below 16 years of age and 6.1 for children below 12 years of age were observed for juvenile chronic arthritis (JCA). 77% of the JCA cases were of pauciarticular onset, and 23% were of polyarticular onset. No cases of systemic JCA were diagnosed. The female to male ratio was 3:2. Antinuclear antibodies were positive in 13% of the JCA cases, and IgM rheumatoid factor was found in 15% of the children. Chronic iritis was noted in only 2 cases; both were girls and both were ANA positive. The incidence found was low compared to population-based studies, but in the same range as hospital-based investigations.
Few well-validated self-and/or parent-administered instruments are available for measuring functional status in children with rheumatic diseases. Parts of the Stanford Health Assessment Questionnaire (HAQ) have been adapted for use in children in the so-called Child HAQ. The aim of this study was to investigate the validity of this instrument in a Swedish setting. The Child HAQ was administered to 186 patients and 211 patients participating in a population-based follow-up study of juvenile chronic arthritis (JCA) in southwestern Sweden. The EULAR criteria were used for inclusion. Children who were 9 years of age or older self-reported. Reliability, evaluated by test-retest, inter-observer correlations and internal reliability, was excellent. Convergent validity was demonstrated by strong correlations of the disability index, pain, and morning stiffness with disease activity and the Steinbrocker functional classes. Discriminant validity was evidenced by the capacity of the instrument to evaluate patients as being active or in remission. Thus, the Child HAQ showed excellent measurement performance in a Swedish setting when using parents or children more than 9 years old as responders.