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MRCPCH GUIDE Brittle asthma Type 1: wide variability in peak expiratory flow ra

MRCPCH GUIDE Brittle asthma Type 1: wide variability in peak expiratory flow rate despite intensive therapy (i.e. > 40% diurnal variation for > 50% of the time over > 150 days) Type 2: sudden severe attacks despite apparently well-controlled asthma Asthma - Management What assessment is recommended during an exacerbation of asthma?  Ask about possible trigger factors, such as a recent upper respiratory tract infection.  Ask about the type and duration of symptoms, what treatment has been started (if any), and whether treatment has improved symptoms.  Assess the severity of the exacerbation: o Look for signs of exhaustion (inability to complete sentences) and cyanosis (bluish lips or extremities). o Examine the person's chest and record the respiratory rate, pulse, and blood pressure. o Record the peak expiratory flow rate (if the person is old enough to comply) and use the best of three recordings to grade the severity of the attack on the basis of the person's best or predicted value: o Moderate: more than 50–75%. o Acute severe: 33–50%. o Life-threatening: < 33%. o Measure a person's oxygen saturation in room air using pulse oximetry (if available).  Ask about depression, alcohol misuse, poor compliance with medication, social isolation and any previous exacerbations. Together with the severity of the exacerbation, this will help to determine the risk of death and the need for hospital admission. Clarification / Additional information  Symptoms are a more sensitive measure than peak expiratory flow rate (PEFR) of the onset of an exacerbation, as the increase in symptoms usually precedes the deterioration in PEFR. Symptoms vary among individuals and age ranges. No symptom or sign alone (or together) is specific, and their absence does not exclude a severe exacerbation.  Signs to look for and record include: o Pulse rate (increasing suggests worsening asthma, whilst a decrease indicates a life-threatening situation). o Respiratory rate and use of accessory muscles. o Degree of wheeze (less apparent with increasing obstruction). o Degree of agitation and consciousness.  Peak expiratory flow rate is a more reliable indicator of severity than symptoms. Use a predicted PEFR value only if the person's recent (within 2 years) best PEFR is unknown. Ideally, use the person's own peak flow meter or a similar type.  Pulse oximetry may not be available in primary care, especially for young children.  When deciding to admit someone to hospital, assess the severity of this current exacerbation and also review the person's history. If they have any associated medical, behavioural, or psychosocial factors that are of concern, lower the threshold for admission.  Use the criteria in Table 1 to grade and record the severity of an asthma exacerbation. Table 1. Levels of severity of acute asthma exacerbation. Level of severity Criteria Near-fatal Respiratory acidosis (increased arterial carbon dioxide) and/or requiring mechanical ventilation with increased inflation pressures Life-threatening Any one of the following in someone with severe asthma: Peak expiratory flow rate < 33% of best or predicted Oxygen saturation < 92% Bradycardia Dysrhythmia Hypotension Silent chest Cyanosis Feeble respiratory effort Exhaustion Confusion Coma Acute severe Any one of: Peak expiratory flow rate 33–50% of best or predicted Respiration rate: 2–5 years old: 40 breaths/min 5–12 years old: 30 breaths/min > 12 years old: 25 breaths/min Pulse: 2–5 years old: 140 beats/min 5–12 years old: 125 beats/min > 12 years old: 110 beats/min Inability to complete sentences in one breath Use of accessory neck muscles (in children) Moderate asthma exacerbation Increasing symptoms Peak expiratory flow rate > 50–70% of best or predicted No features of acute severe asthma Brittle asthma Type 1: wide variability in peak expiratory flow rate despite intensive therapy (i.e. > 40% diurnal variation for > 50% of the time over > 150 days) Type 2: sudden severe attacks despite apparently well-controlled asthma Caution: people with severe or life-threatening attacks sometimes do not appear to be distressed and may not have all the features listed. Agitation and behavioural changes in a child may be a sign of hypoxia. Consider the occurrence of any feature as an alert for the presence of severe or life-threatening asthma The following cardiac conditions are associated with a risk of developing IE: acquired valvular heart disease with stenosis or regurgitation, valve replacement, structural congenital heart disease (including surgically corrected or palliated structural conditions) and hypertrophic cardiomyopathy. The following cardiac conditions are not associated with a risk of IE: • isolated atrial septal defect • repaired ventricular septal defect • repaired patent ductus arteriosus • closure devices that are judged to be endothelialised Case–control study Comparative observational study in which the investigator selects individuals who have experienced an event (for example, developed a disease) and others who have not (controls), and then collects data to determine previous exposure to a possible cause. Cohort study (also known as follow-up, incidence, longitudinal, or prospective study): An observational study in which a defined group of people (the cohort) is followed over time. Outcomes are compared in subsets of the cohort who were exposed or not exposed (or exposed at different levels) to an intervention or other factor of interest. Confidence interval The range within which the ‘true‘ values (for example, size of effect of an intervention) are expected to lie with a given degree of certainty (for example, 95% or 99%). (Note: confidence intervals represent the probability of random errors, but not systematic errors or bias). Odds ratio A measure of treatment effectiveness. The odds of an event happening in the intervention group, divided by the odds of it happening in the control group. The ‘odds’ is the ratio of non-events to events. Quality-adjusted life year (QALY) A statistical measure, representing 1 year of life, with full quality of life. Randomised controlled trial A form of clinical trial to assess the effectiveness of medicines or procedures. Considered reliable because it tends not to be biased. Relative risk Also known as risk ratio; the ratio of risk in the intervention group to the risk in the control group. The risk (proportion, probability or rate) is the ratio of people with an event in a group to the total in the group. A relative risk (RR) of 1 indicates no difference between comparison groups. For undesirable outcomes, an RR that is less than 1 indicates that the intervention was effective in reducing the risk of that outcome. Sensitivity (of a test) The proportion of people classified as positive by the gold standard who are correctly identified by the study test. Systematic review Research that summarises the evidence on a clearly formulated question according to a predefined protocol using systematic and explicit methods to identify, select and appraise relevant studies, and to extract, collate and report their findings. It may or may not use statistical meta-analysis. An article in a leading medical journal reads: ‘an insertion of 5 nucleotides in the gene was identified as the cause of hypertrophic cardiomyopathy in this family’. Which of the following type of mutation is the author referring to? Because the group of nucleotides inserted is not a multiple of three, the mutation changes the frame in which translation occurs and hence the name ‘frame-shift mutation’. A Missense mutation is an alteration in a nucleotide sequence that converts a codon for one amino acid into a codon for a second amino acid. An insertion mutation arises by the insertion of one or more nucleotides into a DNA sequence. A nonsense mutation is an alteration in nucleotide sequences that changes a triplet coding for an amino acid into a termination codon. A point mutation results from a single nucleotide change in a DNA molecule A mid to late systolic impulse in the precordial motion (triple ripple) is seen in patients with HOCM. The second impulse is due to contraction against a narrowed LVOT and the third due to a late systolic bulge in the ventricle near the end of systole. A hyperdynamic impulse is seen in mild to moderate AR. Conditions A, B, D and E can produce the murmur but not this characteristic finding. Syncope is common in adolescence, and is usually benign. Causes of syncope can be classified broadly into: • Neurally-mediated syncope (also called vasovagal syncope), is the commonest cause in adolescence • Cardiac (e.g. arrhythmias) • Non-cardiac (including epilepsy and psychogenic causes) The history is extremely important in the evaluation of syncope, and several warning signs can be identified that could indicate a potentially life-threatening cause. These include: syncope on exercise (associated with hypertrophic cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, catecholaminergic polymorphic VT, and long QT syndrome type 1); syncope with loud noise, fright or emotion (associated with long QT syndrome type 2); syncope when lying down (associated with long QT syndrome type 3 and Brugada syndrome); family history of sudden death (associated with all of the above syndromes); and an odd history. The 12-lead ECG is the most important investigation for recurrent or unexplained syncope. Pre-excitation, long QT interval, heart block, and ventricular hypertrophy with repolarisation abnormalities can all be diagnosed from uploads/Finance/ mrcpch-guide.pdf