Pay attention to back pain reported by children. Half will have a specific or serious cause, the presenting symptoms of serious conditions may be misleadingly mild, and the spectrum of causes and mode of presentation differ from adults. Warning features include onset aged < 4 yr, symptoms persisting beyond 4 weeks, interference with function, systemic features, worsening pain, neurological features and recent onset of scoliosis. Scintigraphy is often useful where clinical features and plain radiographs fail to identify the diagnosis. Sports activities may cause stress reactions in the immature spine, particularly at the junction between spinal segments of differing mobility, the vascularity of the disc and vertebra predisposes to infection, spinal tumours presenting as pain tend to be primary and benign, congenital spinal anomalies causing pain tend to present in childhood, spondylitis presents differently from adults, and conversion hysteria, typically presenting with gross, bizarre and disabling symptoms, is not uncommon in adolescent girls.
OBJECTIVES: To determine whether baseline demographic, clinical, articular and laboratory variables predict methotrexate (MTX) poor response in polyarticular-course juvenile idiopathic arthritis. METHODS: Patients newly treated for 6 months with MTX enrolled in the Paediatric Rheumatology International Trials Organization (PRINTO) MTX trial. Bivariate and logistic regression analyses were used to identify baseline predictors of poor response according to the American College of Rheumatology pediatric (ACR-ped) 30 and 70 criteria. RESULTS: In all, 405/563 (71.9%) of patients were women; median age at onset and disease duration were 4.3 and 1.4 years, respectively, with anti-nuclear antibody (ANA) detected in 259/537 (48.2%) patients. With multivariate logistic regression analysis, the most important determinants of ACR-ped 70 non-responders were: disease duration > 1.3 years (OR 1.93), ANA negativity (OR 1.77), Childhood Health Assessment Questionnaire (CHAQ) disability index > 1.125 (OR 1.65) and the presence of right and left wrist activity (OR 1.55). Predictors of ACR-ped 30 non-responders were: ANA negativity (OR 1.92), CHAQ disability index > 1.14 (OR 2.18) and a parent's evaluation of child's overall well-being < or = 4.69 (OR 2.2). CONCLUSION: The subgroup of patients with longer disease duration, ANA negativity, higher disability and presence of wrist activity were significantly associated with a poorer response to a 6-month MTX course. Results In all, 405/563 (71.9%) of patients were women; median age at onset and disease duration were 4.3 and 1.4 years, respectively, with anti-nuclear antibody (ANA) detected in 259/537 (48.2%) patients.
Fibrodysplasia Ossificans Progressiva (FOP) is a rare, autosomal dominant condition, classically characterised by heterotopic ossification beginning in childhood and congenital great toe malformations; occurring in response to a c.617 G>A ACVR1 mutation in the functionally important glycine/serine-rich domain of exon 6. Here we describe a novel c.587 T>C mutation in the glycine/serine-rich domain of ACVR1, associated with delayed onset of heterotopic ossification and an exceptionally mild clinical course. Absence of great toe malformations, the presence of early ossification of the cervical spine facets joints, plus mild bilateral camptodactyly of the 5th fingers, together with a novel ACVR1 mutation, are consistent with the 'FOP-variant' syndrome. The c.587 T>C mutation replaces a conserved leucine with proline at residue 196. Modelling of the mutant protein reveals a steric clash with the kinase domain that will weaken interactions with FKBP12 and induce exposure of the glycine/serine-rich repeat. The mutant receptor is predicted to be hypersensitive to ligand stimulation rather than being constitutively active, consistent with the mild clinical phenotype. This case extends our understanding of the 'FOP-variant' syndrome.
The sequence of events from asymptomatic hyperuricemia to acute gout, intercritical gout, and finally chronic tophaceous gout is well established. One-fifth of patients who suffer acute gout eventually develop tophaceous deposits if not treated with hypouricemic drugs (1). The patients described here paradoxically developed tophi without any history of acute gouty arthritis. One patient had coexistent rheumatoid arthritis and the other four showed various degrees of renal impairment, a situation inviting speculation on the mechanism of inhibitory factors involved in acute gout. lone approximately 7 mg daily, and in 1978 penicillamine was started. After taking penicillamine 6 months he developed proteinuria, and a chalky deposit was noted on the pinna of one ear (Figure 1). Salient investigations included a normal pyelogram and urine microscopy, creatinine clearance of 126 ml/minute, and proteinuria of 2.1 gm/24 hours.Renal biopsy showed no abnormality by light microscopy in the cortex. Immunofluorescent staining showed diffuse and discrete granular deposits of IgG around the periphery of the glomerular capillary loops. By electron microscopy, numerous small electrondense deposits were identified on the epithelial cell aspect of the glomerular basement membrane. There was fusion of the foot processes of the epithelial cells but the mesangial regions were within normal limits. These changes are compatible with penicillamine-induced glomerulonephritis. In addition, a typical gouty tophus was identified in the medulla (Figure 2). Dissolution of urate from the tissues had been minimized since urate is relatively insoluble in the alcoholic acidic fixative used (Duboscq-Brazil). Polarized light microscopy showed the presence of crystals that stained positively by the Gomori methenamine technique, identifying them as salts of either calcium or urate (2). However preincubation of the sections in a saturated solution of lithium carbonate dissolved out the crystals, specifically identifying them as urate which, unlike calcium salts. is soluble in such a solution. A positive urate control specimen and a duplicate specimen preincubated in lithium carbonate confirmed these findings.Polarized light microscopy of the deposit in the ear showed variable sized needle-shaped crystals that gave a negative sign of birefringence typical of mono-
The use of NSAIDs for arthritis differs in children from adults in their indications, uses and pharmacokinetics, and fewer are available. Children with arthritis are assessed differently, as they complain less of pain. Salicylates, indomethacin and ibuprofen are used for the fever of systemic JCA. For control of joint symptoms, diclofenac, ibuprofen, tolmetin and naproxen are equal in their efficacy and tolerance:salicylates and indomethacin are no more effective but more toxic. Children tolerate NSAIDs well. Gastrointestinal symptoms appear to be less common than in adults, but the evidence regarding endoscopic changes in conflicting. Renal toxicity is rare. Tolmetin can cause pseudoproteinuria and naproxen pseudoporphyria. The liver in systemic JCA is vulnerable to drug toxicity. A therapeutic trial of an NSAID should continue for 8 weeks. Interactions with methotrexate and carbonic anhydrase inhibitors for glaucoma complicating iridocyclitis may occur.
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