Accidental staggered paracetamol overdose: An interesting case report.

Paracetamol is one of the most commonly used drugs both over the counter and on prescription. Liquid paracetamol is available over the counter all over the world. Most commonly available concentrations are 120 mg/5 ml and 250 mg/5 ml. Many parents and healthcare professionals assume that doses available in different countries are similar. However, 500 mg/5 ml bottle is available in some countries including the United Kingdom. This leaves a potential for accidental overdose with therapeutic intent. We have reviewed the experience of diagnosing and managing an interesting case of paracetamol over dosage caused by several ingestions over 24 hours period (staggered paracetamol over dosage). It highlights the importance of communication between health professionals and parents while managing common medical problems.

Juvenile idiopathic arthritis (JIA): a screening study to measure class II skeletal pattern, TMJ PDS and use of systemic corticosteroids.

OBJECTIVE: To screen patients with oligoarticular and polyarticular forms of Juvenile Idiopathic Arthritis (JIA) to determine (i) the severity of their class II skeletal pattern; (ii) temporomandibular joint signs and symptoms and (iii) use of systemic corticosteroids. DESIGN: Cross-sectional screening. SUBJECTS AND SETTING: Sixty-eight children with JIA aged between 9 and 16 years old who were screened at four regional treatment centres in the UK. METHOD: Patients were screened clinically and radiographically for the presence of class II skeletal pattern and temporomandibular (TMJ) pain dysfunction syndrome. In addition, the JIA sub-type and history of disease activity and medication were recorded. MAIN OUTCOME MEASURES: Class II skeletal pattern, TMJ signs and symptoms, use of systemic corticosteroids. RESULTS: The mean ANB values were 4.2 degrees (SD = 2.9 degrees) in the oligoarticular group and 5.1 degrees (SD = 3.8 degrees) in the polyarticular group. Just under one-third of children had a moderate or severe class II skeletal pattern and a further quarter of children had a mild class II skeletal pattern. Clinical signs and symptoms of temporomandibular joint pain dysfunction syndrome were low (<20%), except for crepitus and click which affected between 24 and 40% of JIA children. Radiographically, 57% of oligoarticular and 77% of polyarticular cases exhibited condylar erosion. Use of systemic corticosteroids varied between centres, but overall, was prescribed more in polyarticular cases (P = 0.001). CONCLUSIONS: Just under one-third of oligoarticular and polyarticular JIA patients exhibited a moderate or severe class II skeletal pattern. It is, therefore, likely that any future clinical trial to investigate the effect of functional appliance treatment in JIA patients, will need multicentre co-operation to fulfil potential sample size requirements. Clinical signs and symptoms of temporomandibular joint pain dysfunction syndrome were low except for crepitus and click. However, radiographic evidence of condylar erosion was high particularly in the polyarticular group. Use of systemic corticosteroids was prescribed more in polyarticular cases and this is likely to reflect the severity of the disease.

Biologic therapy in primary systemic vasculitis of the young.

OBJECTIVES: To describe the biologic treatment regimens and report the efficacy and safety of biologic therapies in a multicentre series of children with primary systemic vasculitis (PSV). METHODS: This was a retrospective descriptive case series of children with PSV treated with biologic therapy between February 2002 and November 2007. Primary retrospective outcome assessment measures were: daily corticosteroid dose; Birmingham Vasculitis Activity Score (BVAS); and adverse events (including infection rate). RESULTS: Twenty-five patients median age 8.8 (range 2.4-16) years; 11 male with active PSV (n = 6 with anti-neutrophil cytoplasmic antibody associated vasculitides, n = 11 with polyarteritis nodosa, n = 7 with unclassified vasculitis and n = 1 with Behçet's disease) were treated with biologic agents including infliximab (n = 7), rituximab (n = 6), etanercept (n = 4), adalimumab (n = 1) or multiple biologics sequentially (n = 7). Overall, there was a significant reduction in BVAS from a median of 8.5 (range 5-32) at start of therapy to 4 (range 0-19) at median 32 months follow-up (P = 0.003) accompanied by significant reduction in median daily prednisolone requirement from 1 (range 0.2-2) to 0.25 (range 0-1) mg/kg/day, P = 0.000. For those receiving multiple biologic agents sequentially, a similar clinical improvement was observed with corticosteroid sparing. Infections occurred in 24%, the most severe in those receiving infliximab. CONCLUSION: Our data provide retrospective evidence of efficacy of these agents, and highlight the associated infectious complications. Further multicentre standardization of treatment protocols and data collection to inform clinical trials of biologic therapy in systemic vasculitis of the young is required.

Pfeiffer-type cardiocranial syndrome: a patient with features of this condition and with an unbalanced subtelomeric rearrangement involving chromosomes 1p and 17q.

Pfeiffer-type cardiocranial syndrome (MIM 218450) was first delineated in 1987; several further patients have been reported confirming this as a distinct nosological entity. The aetiology of this condition remains unknown although an autosomal recessive pattern of inheritance has been suggested following the description of sib pairs. A patient is described with features of this condition including sagittal suture synostosis, growth retardation, learning difficulties, hypertelorism, low-set ears, micrognathia, congenital heart defects and genital anomalies. Telomere studies on blood and skin samples identified a de novo unbalanced rearrangement resulting in partial monosomy for 1p36.1 to pter and partial trisomy for 17q25.1 to qter. This case provides the first insight into the possible aetiology of this condition.

Prospective validation of the Glasgow Meningococcal Septicaemia Prognostic Score. Comparison with other scoring methods.

UNLABELLED: A prospective observational study was done to derive performance characteristics for the Glasgow Meningococcal Septicaemia Prognostic Score (GMSPS) and compare it with nine other severity scores (Stokland, Stiehm and Damrosch, Ansari, Niklasson, Leclerc, Kahn and Blum, Lewis, Istanbul and Bjark) and laboratory markers of disease severity. In the paediatric departments of six hospitals in Merseyside, UK, 278 children with confirmed or probable meningococcal disease were admitted between November 1988 and August 1990 ( n=152) and between September 1992 and April 1994 ( n=126); 26 of whom died. GMSPS was recorded on admission and again if there was clinical deterioration. Laboratory markers of disease severity (including endotoxin and cytokine levels) were measured on admission. The nine other scores were recorded on the first cohort. "Maximum" GMSPS (before referral to the paediatric intensive care unit) was achieved within 12 h of arrival in 97% of children. A GMSPS > or =8 had sensitivity 100%, specificity 75% and positive predictive value for death of 29%, GMSPS > or =10 had 100%, 88% and 46% respectively. All 26 who died scored >10, before referral to the paediatric intensive care unit. GMSPSs calculated by other medical staff had similar characteristics to those calculated by research fellows. All scores correlated significantly with white cell count, coagulopathy, endotoxin and cytokine levels. However, the predominantly clinical scores were the most robust. GMSPS had amongst the best performance characteristics of all scores and was more sensitive than laboratory markers. CONCLUSION: the Glasgow Meningococcal Septicaemia Prognostic Score is an easily performed, repeatable, clinical score that can rapidly identify children with fulminant meningococcal disease. When performed prospectively, a score > or =8 had a positive predictive value for death of 29%. This score can identify those children who should be offered intensive care and can select those who may benefit from novel therapies.