Davinder Singh-Grewal

Circumcision for the prevention of urinary tract infection in boys: a systematic review of randomised trials and observational studies

Objective: To undertake a meta-analysis of published data on the effect of circumcision on the risk of urinary tract infection (UTI) in boys. Data sources: Randomised controlled trials and observational studies comparing the frequency of UTI in circumcised and uncircumcised boys were identified from the Cochrane controlled trials register, MEDLINE, EMBASE, reference lists of retrieved articles, and contact with known investigators. Methods: Two of the authors independently assessed study quality using the guidelines provided by the MOOSE statement for quality of observational studies. A random effects model was used to estimate a summary odds ratio (OR) with 95% confidence intervals (CI). Results: Data on 402 908 children were identified from 12 studies (one randomised controlled trial, four cohort studies, and seven case–control studies). Circumcision was associated with a significantly reduced risk of UTI (OR = 0.13; 95% CI, 0.08 to 0.20; p<0.001) with the same odds ratio (0.13) for all three types of study design. Conclusions: Circumcision reduces the risk of UTI. Given a risk in normal boys of about 1%, the number-needed-to-treat to prevent one UTI is 111. In boys with recurrent UTI or high grade vesicoureteric reflux, the risk of UTI recurrence is 10% and 30% and the numbers-needed-to-treat are 11 and 4, respectively. Haemorrhage and infection are the commonest complications of circumcision, occurring at rate of about 2%. Assuming equal utility of benefits and harms, net clinical benefit is likely only in boys at high risk of UTI.

Feasibility and effectiveness of an aerobic exercise program in children with fibromyalgia: results of a randomized controlled pilot trial.

OBJECTIVE To determine the feasibility of conducting a randomized controlled trial of a 12-week exercise intervention in children with fibromyalgia (FM) and to explore the effectiveness of aerobic exercise on physical fitness, function, pain, FM symptoms, and quality of life (QOL). METHODS FM patients ages 8-18 years were randomized to a 12-week exercise intervention of either aerobics or qigong. Both groups participated in 3 weekly training sessions. Program adherence and safety were monitored at each session. Data were collected at 3 testing sessions, 2 prior to and 1 after the intervention, and included FM symptoms, function, pain, QOL, and fitness measures. RESULTS Thirty patients participated in the trial. Twenty-four patients completed the program; 4 patients dropped out prior to training and 2 dropped out of the aerobics program. Better adherence was reported in the aerobics group than in the qigong group (67% versus 61%). Significant improvements in physical function, functional capacity, QOL, and fatigue were observed in the aerobics group. Anaerobic function, tender point count, pain, and symptom severity improved similarly in both groups. CONCLUSION It is feasible to conduct an exercise intervention trial in children with FM. Children with FM tolerate moderate-intensity exercise without exacerbation of their disease. Significant improvements in physical function, FM symptoms, QOL, and pain were demonstrated in both exercise groups; the aerobics group performed better in several measures compared with the qigong group. Future studies may need larger sample sizes to confirm clinical improvement and to detect differences in fitness in childhood FM.

Predicting the Course of Juvenile Dermatomyositis : Significance of Early Clinical and Laboratory Features

OBJECTIVE Juvenile dermatomyositis (DM) is a rare chronic inflammatory disease of childhood. The clinical course of juvenile DM appears to be variable, and little is known about predictors of the disease course. The aims of this study were to describe the clinical course of juvenile DM and to determine whether early clinical and laboratory features can be used to predict the time to remission and/or the disease course. METHODS Clinical and laboratory data from a cohort of 84 patients with juvenile DM were prospectively entered into a database (1990-2005). Remission was defined as a clinical state of no active skin rash, weakness, or elevated muscle enzyme levels for 6 months off medication. The disease course was defined as monophasic, polyphasic, or chronic. Data were reviewed at the time of diagnosis and at 3 months and 6 months after the diagnosis to determine predictors of the time to remission and/or the disease course. RESULTS The median time to remission was 4.67 years. Sixty percent of patients had a chronic course, 37% a monophasic course, and 3% a polyphasic course. The presence of rash (most strongly indicated by Gottrons papules) at 3 months was the earliest predictor of a longer time to remission (relative risk [RR] 0.55 [95% confidence interval (95% CI) 0.37-0.81], P = 0.002). At 6 months, the presence of nailfold abnormalities and rash also predicted a longer time to remission (RR 0.35 [95% CI 0.14-0.74], P = 0.003). We were unable to determine a prediction model of disease course. CONCLUSION The majority of patients in our cohort had a chronic disease course. The persistence of Gottrons papules and nailfold abnormalities early in the disease course was associated with a longer time to remission.

Familial Aicardi–Goutières syndrome due to SAMHD1 mutations is associated with chronic arthropathy and contractures†‡§

We report on two siblings doubly heterozygous for null mutations in the recently identified AGS5 gene SAMHD1. The older female child showed mild intellectual disability with microcephaly. Her brother demonstrated a significant spastic paraparesis with normal intellect and head size. Both children had an unclassified chronic inflammatory skin condition with chilblains, and recurrent mouth ulcers. One child had a chronic progressive deforming arthropathy of the small and large joints, with secondary contractures. This family illustrate the remarkable phenotypic diversity accruing from mutations in genes associated with Aicardi–Goutières syndrome (AGS). The association of arthropathy with SAMHD1 mutations highlights a phenotypic overlap of AGS with familial autoinflammatory disorders such as chronic infantile neurological cutaneous and articular syndrome (CINCA). This family therefore illustrate the need to consider mutation analysis of SAMHD1 in non‐specific inflammatory phenotypes of childhood. We propose that arthropathy with progressive contractures should now be considered part of the spectrum of Aicardi–Goutières syndrome because of SAMHD1 mutations.

Children's experiences of living with juvenile idiopathic arthritis: A thematic synthesis of qualitative studies

To describe the experiences and perspectives of children and adolescents living with juvenile idiopathic arthritis (JIA).

Diagnosis, Monitoring, and Treatment of Systemic Lupus Erythematosus: A Systematic Review of Clinical Practice Guidelines

Management of systemic lupus erythematosus (SLE) is complex and variability in practices exists. Guidelines have been developed to help improve the management of SLE patients, but there has been no formal evaluation of these guidelines. This study aims to compare the scope, quality, and consistency of clinical practice guidelines on the diagnosis, monitoring, and treatment of patients with SLE.

Kawasaki Disease: A Clinician’s Update

Aims. Kawasaki disease is an acute systemic vasculitis and is the most common cause of acquired heart disease in children in the developed world. This review aims to synthesise recent insights into the disease and provide an update for clinicians on diagnostic and treatment practices. Methods. We conducted a review of the literature exploring epidemiology, aetiology, diagnosis, and management of Kawasaki disease. We searched MEDLINE, Medline In-Process, Embase, Google Scholar, and reference lists of relevant articles. Conclusions. Kawasaki disease is a febrile vasculitis which progresses to coronary artery abnormalities in 25% of untreated patients. The disease is believed to result from a genetically susceptible individuals exposure to an environmental trigger. Incidence is rising worldwide, and varies widely across countries and within different ethnic groups. Diagnosis is based on the presence of fever in addition to four out of five other clinical criteria, but it is complicated by the quarter of the Kawasaki disease patients with “incomplete” presentation. Treatment with intravenous immunoglobulin within ten days of fever onset improves clinical outcomes and reduces the incidence of coronary artery dilation to less than 5%. Given its severe morbidity and potential mortality, Kawasaki disease should be considered as a potential diagnosis in cases of prolonged paediatric fever.

Antibody binding to neuronal surface in movement disorders associated with lupus and antiphospholipid antibodies

Aim  Systemic lupus erythematosus is a multi‐organ autoimmune disorder associated with autoantibodies of complex diversity. Antiphospholipid antibodies (aPL), which are commonly associated with lupus, create a pro‐thrombotic tendency, but are also associated with non‐thrombotic neurological features. Movement disorders are rare neuropsychiatric complications of lupus and antiphospholipid syndrome, and autoimmune and thromboembolic disease mechanisms have been proposed.

Children's experiences of cystic fibrosis: a systematic review of qualitative studies.

BACKGROUND AND OBJECTIVE: Cystic fibrosis (CF) is a common life-shortening genetic disease and is associated with poor psychosocial and quality of life outcomes. The objective of this study was to describe the experiences and perspectives of children and adolescents with CF to direct care toward areas that patients regard as important. METHODS: MEDLINE, Embase, PsycINFO, and Cumulative Index to Nursing and Allied Health Literature were searched from inception to April 2013. We used thematic synthesis to analyze the findings. RESULTS: Forty-three articles involving 729 participants aged from 4 to 21 years across 10 countries were included. We identified 6 themes: gaining resilience (accelerated maturity and taking responsibility, acceptance of prognosis, regaining control, redefining normality, social support), lifestyle restriction (limited independence, social isolation, falling behind, physical incapacity), resentment of chronic treatment (disempowerment in health management, unrelenting and exhausting therapy, inescapable illness), temporal limitations (taking risks, setting achievable goals, valuing time), emotional vulnerability (being a burden, heightened self-consciousness, financial strain, losing ground, overwhelmed by transition), and transplant expectations and uncertainty (confirmation of disease severity, consequential timeliness, hope and optimism). CONCLUSIONS: Adolescents and children with CF report a sense of vulnerability, loss of independence and opportunities, isolation, and disempowerment. This reinforces the importance of the current model of multidisciplinary patient-centered care that promotes shared decision-making, control and self-efficacy in treatment management, educational and vocational opportunities, and physical and social functioning, which can lead to optimal treatment, health, and quality of life outcomes.

A prospective study of the immediate and delayed adverse events following intravenous immunoglobulin infusions

Aim: To document the incidence of immediate and delayed adverse events (AE) following intravenous immunoglobulin (IVIG) infusion in children. Methods: Immediate and delayed adverse events were prospectively recorded for 345 infusions in 58 children receiving IVIG for immunodeficiency (n = 33) or immunomodulation (n = 25). For each infusion adverse events were documented during the infusion and by follow up interview 4–7 days later. Results: Immediate adverse events occurred in 10.3% and delayed adverse events in 41.4% of children treated during the study period. Three and a half per cent of the infusions were associated with immediate AE and 20.9% with delayed adverse events. Headache was the most common delayed AE, occurring in 24.1% of patients and 12.8% of infusions. Conclusions: Delayed adverse events to IVIG infusions are common in children. They occur more frequently than immediate adverse events and are the cause of significant morbidity. Recognition of the high frequency of delayed adverse events is important in the care of children receiving IVIG therapy.