Camurati-Engelmann disease--a case report and literature review.
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Biomedical subjects
Publications and source records attributed to E M Baildam.
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Juvenile dermatomyositis (JDM) is a rare disease of childhood with significant morbidity. Although the mortality and morbidity has improved over recent decades, it is still a chronic disease for a significant proportion of children with JDM. There is a paucity of controlled trials in both adult and juvenile dermatomyositis. In this article, the different therapeutic agents used in clinical practice are outlined. Furthermore, our approach to the treatment of JDM with methotrexate and aggressively tapered steroids in an algorithmic fashion, is discussed.
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Methotrexate (MTX) has transformed the outlook for children with juvenile idiopathic arthritis (JIA). Most of the evidence from uncontrolled clinical trials suggests that MTX is an effective agent for treating active JIA. Data from controlled clinical trials suggests that MTX has statistically significant effects on patient centred disability measures in JIA patients with active arthritis. Although we would like a much larger study directed evidence base for our use of the drug, the studies that have been done are sound and have been followed by a change in clinical expectations and advice that speak of qualitative evidence from clinical practice, confirming the scientifically acquired data. Randomised controlled multicentre trials using sufficient numbers of patients, including functional assessment and quality of life measures, are needed to confirm the long term efficacy and safety of MTX in JIA.
OBJECTIVE: To assess the nutritional status of children with juvenile idiopathic arthritis (JIA) using anthropometric measurements and bioelectrical impedance. METHODS: Twenty-two consecutive JIA patients (seven pauciarticular, 15 polyarticular) attending the rheumatology clinic at Booth Hall Children's Hospital were compared with 22 age- and sex-matched controls attending the accident and emergency department of the same hospital. There were no patients with systemic-onset JIA in the cohort. Height, weight, head circumference and skinfold thickness at four sites (biceps, triceps, subscapular and suprailiac) were measured. Regression equations were used to calculate body fat as a percentage of weight, and arm muscle circumference. In addition, bioelectrical impedance measurements were made using a Holtain body composition analyser. These measurements were then used to calculate the total body water, which could be used as an indirect estimate of the lean body mass. RESULTS: Of the JIA patients, 22.7% were below the third centile for height, 18.1% had a weight less than the third centile. Mid-arm circumference was below the fifth centile in 36.4% of the patients. Patients with polyarticular disease showed significantly more signs of malnutrition than patients with pauciarticular disease. In the polyarticular group, comparison with controls revealed significant P values for reduction in height (0.047), weight (0.045), mid-arm circumference (0.002), arm muscle circumference (0.012), percentage body fat (0.008) and total body water (0.031). CONCLUSIONS: In view of the findings of lower total body water, indicating lower lean mass, in more nutritionally deprived JIA patients (as deduced by the other physical parameters measured), we conclude that bioelectrical impedance is a useful adjunct to anthropometric measures in assessing nutritional status in JIA.
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The time spent by 158 infants in contact with their carers at 6, 13, 26 and 52 weeks was reviewed prospectively. Periods of contact in the categories of (1) physical care, (2) holding the crying or sleeping infant, and (3) playing and interacting with the infant were recorded using 24-h log diaries completed by the mother. The mean total carer contact time over a 24-h day did not change significantly in the first year, varying between 6.5 and 73 h. Between 6 and 52 weeks, time spent by the mother in physical care declined significantly from 207 to 143 min and in holding the crying or sleeping infant from 61 to 17 min (P < 0.05 and 0.0001 respectively). There were no significant changes in the amount of time spent in playing and interacting with the infant over the first year by the mother and father, the time being on average 52.7 and 25.0 min respectively. Play and interaction with a non-parental carer increased significantly from 14 to 69 min (P < 0.0001). Relationships between infant size and holding became weaker as the infant became older. Infant gender, socioeconomic status and duration of breast-feeding did not influence infant contact time.
Twenty-nine children with juvenile chronic arthritis, aged from 7 to 16 yr were studied using the Juvenile Arthritis Functional Assessment Report for Children and Parents questionnaires (JAFAR-C and JAFAR-P). The results showed that JAFAR-C and JAFAR-P were highly correlated (P < 0.001) with each other and also with a predicted JAFAR score (P < 0.05). The JAFAR score correlated with the arthritic joint count at the time of the study (P < 0.01), Steinbrocker classification (P < 0.001), pain score (P < 0.01) and stiffness score (P < 0.005). Evidence of psychological dysfunction was found in eight of the 29 children (27%). Six children (21%) scored 13 or more on the Rutter A(2) for parents and one patient scored more than nine on the Rutter B(2) for teachers. Only one child had a score on the Birleson self-rating scale that was high enough to suggest a likely diagnosis of depression. The median Lipsitt self-concept scale score was 86, that expected for a healthy population. There was no correlation between the psychological scores and any of the other functional measurements, although the numbers affected were small. There was no significant difference between type of arthritis at onset with regard to any of the scores obtained.
In a prospective study of fetal and postnatal growth and development in a group of babies whose mothers were residents of an inner-city health district in the north of England, the total amount of crying of 157 infants was recorded at four periods during the first year of life by means of a 24-hour log. The mean number of crying episodes reduced from 4.4 at six weeks to 1.5 at one year. Early crying predicted later crying. It was not possible to predict which babies would cry a lot except that breast-fed infants tended to cry less. Mothers' perceptions of whether their babies cried a lot correlated with their perception of sleep difficulties. Rapid response to crying was associated with significantly less crying overall.
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