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J. Leighton. University of Portsmouth, Portsmouth, UK
Aims“Growing pains” (GP) has been associated with reduced tibial speed of sound (SOS), more tender points and reduced pain thresholds; however, its aetiology remains unknown. Femoral neck anteversion angle (FNA) has been implicated with several paediatric orthopaedic complaints. Normal FNA regression occurs from birth to mid‐adolescence from 36° down to 11–15° through progressive internal torsion of the femoral neck relative to the diaphysis. This blinded, cross‐sectional pilot study investigates relative difference in mean FNA between children (aged 2–10‐year old) with/without GP symptoms and for within‐group correlations between age and FNA.
MethodsHealthy subjects aged 2–10 years old, whose parents gave written consent and who met inclusion criteria, were consecutively recruited from two infants schools and a nursery; their parents completed a validated leg pains questionnaire used to systematically score and categorise subjects into control or test groups based on a widely accepted definition. Subjects were clinically evaluated for: FNA and hip internal/external rotation (IHR/EHR) by a chiropractor blinded to group designation. The study was terminated when 100 subjects had been examined, including at least 20 “with‐GP” designates. Examination data were concealed from the investigator during data analysis and scoring of questionnaire responses. Ethics committee approval was given.
ResultsOf 123 children with parental consent, 111 met criteria and all 111 questionnaires were returned. The study was terminated with 100 examined subjects; questionnaire scoring designated n=33 to test group, n=67 to controls. High‐side (control 54±9° (95% CI 51–56); test 62±12° (95% CI 58–67), p<0.0001) and absolute difference IHR (control 9±11° (95% CI 6–12); test 19±14° (95% CI 14–24), p=0.0002) were significantly different between groups. High‐side (control 30±8° (95% CI 28–32); test 37±8° (95% CI 34–39), p=0.0003) and absolute difference FNA (control 5±6° (95% CI 4–7); test 13±8° (95% CI 10–15), p<0.001) were significantly different between groups. Age correlated well with high‐side FNA for controls r=−0.5286 (95% CI −0.6823–0.3303), p<0.001 but not for GP subjects.
ConclusionsGP may be influenced by larger‐than‐normal unilateral IHR and FNA; and/or by larger‐than‐normal between‐limbs IHR‐ and FNA‐difference. Impaired/delayed FNA regression may be associated with GP.
K. Haslam1, presenting. , R. Wakefield1, L. McCann2, S. Wyatt1. 1Leeds Teaching Hospitals NHS Trust, Leeds, UK; 2Royal Liverpool Children's Hospital, Liverpool, UK
AimsAdult studies have demonstrated ultrasound (US) to be more sensitive at detecting synovitis than clinical examination. The detection of subclinical disease may have implications for deciding which patients receive more aggressive therapy from the outset. US has several advantages over other modalities such as MRI which include: low cost, ability to scan multiple joints in short periods of time and acceptability by children. We have previously demonstrated that in adults 1/3 of patients with oligo‐arthritis actually have a polyarticular disease demonstrated by US. This study aimed to determine whether children with clinically diagnosed oligo‐articular juvenile idiopathic arthritis (JIA) also had subclinical synovitis which could be detected by US.
MethodsSeventeen children with a median age of 10 years (range 3–13 years), with oligo‐articular disease of duration <12 months (median 5 months) as determined by a consultant paediatric rheumatologist, were recruited from a tertiary paediatric rheumatology clinic. All subjects were DMARD and oral corticosteroid naïve. All children were clinically assessed by a paediatric rheumatology SpR. A core set of the child's joints were examined for synovitis, including the knees, ankles, wrists, elbows, hands and feet. A rheumatologist trained in joint US and blinded to all clinical data, performed an US examination at the same clinic appointment.
ResultsA total of 43 joints out of 680 examined, were found either clinically (11), by US (15) or both (17) to be active. Subclinical synovitis was detected in 6 of the 17 children (35%), with the majority of joints involved being in the hands (33%) and feet (40%). One child was reclassified as having polyarticular disease. The 11 joints which had clinical synovitis but no evidence of US synovitis were also mostly in the hands.
ConclusionsThis pilot study has demonstrated that US is a potentially valuable tool, in both confirming the presence of synovitis and in diagnosing subclinical synovitis, particularly when considering the small joints of the hands and feet. Early detection of synovitis in the small joints can impact significantly in the active joint count and hence disease classification. A larger prospective study is required to determine the clinical significance of this finding.
I. Yunas1, J. Bower1, A. Jordan1, M. Wood1, A. Tabor1, P. Campbell‐Stokes1, J. McDonagh2. 1Birmingham Children's Hospital, Birmingham, UK; 2University of Birmingham, Birmingham, UK
IntroductionSkills‐training in self management of both chronic illness and general health is an integral component of transitional care. How best to address these needs within an adolescent rheumatology service however has yet to be identified. In order to prioritise development of new educational interventions within the adolescent rheumatology service, the specific needs reported by the young people themselves were explored.
AimTo identify current need for intervention as identified by young people on transition checklists in routine use for early (11–13 years), mid (14–16 years) and late (16+ years) adolescents.
MethodsRetrospective review of transition checklists of last 72 adolescent rheumatology outpatients.
ResultsTransition checklists were reviewed of 26 male and 46 female patients, the majority of whom had juvenile idiopathic arthritis (87.5%).
ConclusionsThe routine use of developmentally appropriate checklists during transition assists in identifying a range of both disease‐specific and generic unmet needs among adolescents with rheumatic disease. This information is important for planning future educational interventions and resources.
D. James1, I. Yunas1, A. Jordan1, A. Tabor1, P. Campbell‐Stokes1, M. Wood1, J. McDonagh2. 1Birmingham Children's Hospital NHS Trust, Birmingham, UK; 2University of Birmingham, Birmingham, UK
BackgroundYoung people are the “new users” of health services, previously accessed by their parents on their behalf. One means of engaging young people in self‐management is to send them copies of the clinic letters. Although this proposal was made for adults in the NHS plan, there was no specific advice regarding adolescents.
AimsTo audit current practice of sending letters to adolescents in a paediatric rheumatology department.
MethodsA two‐phase study using structured proforma: (1) a retrospective case note review (n=100) and (2) a postal questionnaire was sent to consecutive adolescents (n=70) 2 weeks after a copy of the clinic letter was posted to them. If their parent was a co‐addressee, a questionnaire was also sent to the parent (n=40). Results of (1): 73% of young people received a letter from a doctor following an outpatient consultation (as a direct copy (57%), as primary addressee (11%) or as co‐addressee with their parent (5%). The majority of discharge summaries and letters to other professionals were never copied to the patient. The average ratio of letters received by young people compared to those received by professionals is 0.77. The majority of letters to professionals used a structured format whilst all letters written directly to the adolescent did not. Letters written specifically to adolescents were also less likely to include important information such as medication information (5% and 99% respectively) or diagnosis (11% and 98% respectively) compared to those written to professionals. Results of (2): 24 (34%) adolescents and 20 (50% parents) returned completed questionnaires. All adolescent respondents found the letter understandable, 23 found it useful, 23 filed it with other health information, 21 were satisfied with the content and 21 wanted to receive such letters in the future. Of 20 letters sent jointly to the young person and parent, 5 had not been read by the young person in question. 7 parents stated a preference for a letter to be just written only to them and not the young person.
ConclusionMost adolescents currently receive letters following clinic visits. The practice was well received by the young people. There is room for improvement with respect to content and format in those letters written specifically for the adolescents. Further consideration of parental involvement in the process for adolescents is required, particularly with reference to their rights to confidentiality.
M. Wood, K. Johnson, C. Ryder. Birmingham Children's Hospital, Birmingham, UK
IntroductionSacroiliitis in JIA can be resistant to conventional therapies. Intra‐articular injection with triamcinolone hexacetonide is widely regarded as the preferred treatment for juvenile idiopathic arthritis (JIA) involving most joints outside the spine. There are obvious advantages of reducing the unwanted systemic effects of corticosteroids in children and also longer benefit is achieved than after systemic corticosteroids. Experience from management of adults with sacroiliitis suggests intra‐articular injection is safe and effective. There are no reports in the English literature of sacroiliac joint injection for the management of JIA.
MethodsWe describe the procedure of sacroiliac joint injection with triamcinolone hexacetonide using CT guidance in children under a general anaesthetic.
ResultsDemographic details, baseline data and follow‐up data are provided for 9 injections in 4 different patients with JIA. All had sacroiliitis demonstrated before injection on MRI scan. Symptomatically 4 injections resulted in benefit maintained at latest follow‐up, 3 produced benefit for around 3 weeks and 2 injections lead to no significant benefit. Repeat MRI was performed in 2 patients 6 weeks after the injections: 2 joints showed resolution of sacroiliitis, but 2 joints showed continuing sacroiliitis. No adverse events were observed during follow‐up from 3 to 24 months.
ConclusionsWe suggest that this small retrospective case series suggests intra‐articular corticosteroid injections for sacroiliitis in juvenile idiopathic arthritis is safe. The degree and length of benefit was variable, so with the current data we suggest this is a procedure to consider when sacroiliitis persists despite standard therapies.
A. McMahon, M. Friswell. Newcastle upon Tyne Hospitals NHS Trust, Newcastle upon Tyne, UK
AimWe report on a male patient with chronic infantile neurological cutaneous and articular (CINCA) syndrome without genetic mutation in CIAS1 gene.
CaseThe patient was diagnosed and commenced on treatment at 4 months of age. Over a 3‐month period the patient had an initial response to pulsed IV methylprednisolone with resolution of pyrexia and rash and a reduction in inflammatory markers. However, despite daily oral prednisolone in combination with weekly IV methotrexate there was a relapse of symptoms of rash and pyrexia and a corresponding increase in inflammatory markers and serum amyloid A (SAA), (CRP>100, SAA>200). Daily administration of the recombinant human interleukin‐1 receptor antagonist Anakinra was commenced at 8 months of age at an initial dose of 2 mg/kg s/c. On this dose regime the rash disappeared, but the pyrexia only partially settled and inflammatory markers only partially decreased. Within 1 month pyrexia and rash returned with rising CRP and SAA.
His dose was therefore recalculated, extrapolating from the conventional adult dose. Current adult dose used is 100 mg daily s/c, which equates to about 60 mg/m2. When this was calculated against the patient's body surface area this equated to a rise from 12 to 16 mg daily. Within 2 weeks at this higher dose his rash and temperatures resolved completely, and within 2 months SAA and CRP had normalised. His methotrexate and prednisolone were stopped. However when the given dose fell below 50 mg/m2 during subsequent reviews, his symptoms returned and inflammatory markers rose again.
DiscussionDosages of many drugs are better expressed based on body‐surface area rather than weight, and this is especially true in infancy. This patient demonstrates the use of dose/surface area with excellent benefits in this patient. Patients with CINCA who appear to be non‐ or partial responders to conventional dosages may benefit from recalculating the dose according to surface area rather than weight.
A. McMahon, B. Davies, R. Wyllie, M. Friswell. Newcastle upon Tyne Hospitals NHS Trust, Newcastle upon Tyne, UK
AimThe aim was to review the results of the current Methotrexate (MTX) monitoring programme in a tertiary paediatric rheumatology centre.
MethodA retrospective audit of all patients with juvenile idiopathic arthritis (JIA) commenced on MTX since June 2002 at a tertiary centre. The results at 2 weeks and time to first abnormal result were established.
ResultsThere were 84 patients in total. All blood results documented at 2 weeks after commencing MTX were normal. 6/84 (7.1%) patients developed a neutropenia, median 1.5 (range 0.5–1.8×109/l) within median 5.5 months (range 3–22). 8/84 (9.5%) patients developed an abnormal alanine transferase (ALT), median result 97.5 (range 82–364 U/L) with a median time 11 months (range 3–30). There was no significant discrepancy between JIA subtypes.
DiscussionCurrently the timing of blood tests with patients taking MTX varies between individual centres. At this tertiary centre current MTX monitoring consists of the first blood test taking place 2 weeks after MTX is commenced. Thereafter, blood tests are checked monthly for 6 months then if results are normal they are changed to 2‐monthly. Monitoring of blood tests is principally performed for two main reasons; full blood count (FBC) because MTX can cause haemopoietic suppression and liver function tests (LFTS) because liver cirrhosis has been reported. BSPAR guidelines state “FBC and LFTS should be checked regularly.” The frequency of measurement is noted to vary between different centres from every 2 weeks for 4–8 weeks or until the dose is stable and then monthly to 6‐weekly. This study in this centre provides further information with a median time to abnormal results of between 5.5–11 months. It suggests that the blood test after 2 weeks of starting MTX is not necessary. Indeed the results of this survey suggest that it may be possible to start monitoring later than the current BSPAR guidelines suggest.
E. Smith1, J. M. Gardner‐Medwin2. 1FY2 Paediatrics, Dumfries and Galloway Royal Infirmary, Dumfries; 2Department of Child Health, University of Glasgow, Glasgow, UK
IntroductionComplications of accelerated atherosclerosis are a major cause of long‐term morbidity and mortality in adults with systemic lupus erythematous (SLE). Although children with SLE (JSLE) rarely display these complications the atherosclerotic process is thought to begin in childhood, and be related to traditional risk factors for cardiovascular (CV) disease and SLE associated vascular, immune, inflammatory and medication effects.
AimsTo evaluate CV disease risk factors identified during routine clinical care in JSLE.
MethodsMedical records of 8 children (7 female) mean 12.3 years (range 8.16–14.6) at JSLE onset, with a mean follow‐up of 4.48 years (range 2.91–6.83) were assessed for traditional CV risk factors, including body mass index (BMI), blood pressure (BP), serum lipid profiles, smoking and diabetes mellitus. BP and BMI values were adjusted for age, sex and height providing a Z score. BILAG index defined active disease (score A and B) or remission (D and E) respectively, together with laboratory activity markers (dsDNA/complement/ESR).
ResultsAll patients had raised BP during follow‐up. The time interval from JSLE diagnosis to elevation of SBP‐Z score ranged between 2 to 34 months (mean 8.2). The mean SBP‐Z scores were 1.22 (range −0.1 to 2.41) and 1.41 (range −0.5 to 3.76) during times of active disease and remission, and 0.93 (range −0.5 to 2.56) and 1.05 (range −0.9 to 2.89) during treatment with high or low dose corticosteroid, respectively. 7/8 patients had a high BMI‐Z score during follow‐up. The time interval between JSLE diagnosis and BMI first becoming elevated ranged from 4 to 43 months (mean 14.8). The mean BMI‐Z score was 1.03 (range −1.27 to 3.19) and 1.84 (range −1.56 to 3.63) during periods of active disease and remission, and 1.53 (range −0.6 to 3.36) and 1.15 (range −1.27 to 3.19) during treatment with high or low dose corticosteroids, respectively. 1/6 patients had an elevated total cholesterol level (>5.2 mmol/l), and 2 further patients had borderline levels. 2/8 had a random blood glucose/HbA1c measured (both normal). No smoking history documentation was found.
ConclusionThe early and persistent nature of the CV risk factors identified in JSLE highlights that these factors require monitoring from the initial stages of the disease. BMI/BP‐Z scores did not vary significantly with disease activity and corticosteroid intake, suggesting that BMI and BP need to be targeted in addition to standard JSLE disease control. Improved clinical awareness early in the disease course and recording of these simple measures would immediately improve management. A formal study of traditional/non‐traditional CV risk factors in JSLE, is required for future CV events to be prevented.
S. Blelock, D. Gibson, S. Clarke, C. McAllister, M. Rooney. Queen's University Belfast, Belfast, UK
IntroductionThere are extensive studies on the histology of the synovial membrane in adult arthritis, but to date there are no prospective studies in early untreated juvenile idiopathic arthritis (JIA).
AimTo characterise the synovial membrane of early inflammatory disease in untreated JIA.
MethodsSynovial biopsies (n=5) were obtained from 30 JIA patients defined according to ILAR criteria with ultrasound guidance and the joint subsequently injected as per clinical indication. x Rays, MRI scans, clinical and biochemical measurements are obtained at regular intervals over the following 2 years. We report the histological, clinical and disease course findings of the oligo and poly populations. Three biopsies were analysed per patient, with 10 high power fields (HPF) studied per biopsy (×200 magnification). Macrophages, lymphocytes, growth factors, RANK, OPG and vessel density and distribution were subsequently assessed by immunohistochemistry.
ResultsWe found significantly more pathology in the poly compared with oligo patients. Moreover, polys had more vessels. The degree of cellular infiltration was modest, with diffuse infiltrates being the commonest feature observed in all patients. Marked macrophage populations were present in both the lining layer and the sublining layer. B cell density was also modest mean 1.0 (0.5–1.9). Diffuse, occasional B cells were observed in 76.5% of cases. 23.5% cases revealed low/moderate staining, with 75% of these patients exhibiting focal aggregates at vessels. The immuno‐pattern of the total T‐cell population revealed CD3+ cells (mean 1.7 (0.8–2.7)) were predominately CD4+ (mean 1.7 (0.7–2.6)) and distributed mainly within the sublining layer.
ConclusionA positive correlation between vessel score and the number of swollen joints in both disease subgroups suggests that angiogenesis and disease progression may be coupled.