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Performance validation of the ALPPS risk model.
No full textBACKGROUND
Based on the International ALPPS registry, we have recently proposed two easily applicable risk models (pre-stage1 and 2) for predicting 90-day mortality in ALPPS but a validation of both models has not been performed yet.
METHODS
The validation cohort (VC) was composed of subsequent cases of the ALPPS registry and cases of centers outside the ALPPS registry.
RESULTS
The VC was composed of a total of 258 patients including 70 patients outside the ALPPS registry with 32 cases of early mortalities (12%). Development cohort (DC) and VC were comparable in terms of patient and surgery characteristics. The VC validated both models with an acceptable prediction for the pre-stage 1 (c-statistic 0.64, P = 0.009 vs. 0.77, P < 0.001) and a good prediction for the pre-stage 2 model (c-statistic 0.77, P < 0.001 vs. 0.85, P < 0.001) as compared to the DC. Overall model performance measured by Brier score was comparable between VC and DC for the pre-stage 1 (0.089 vs. 0.081) and pre-stage 2 model (0.079 vs. 0087).
CONCLUSION
The ALPPS risk score is a fully validated model to estimate the individual risk of patients undergoing ALPPS and to assist clinical decision making to avoid procedure-related early mortality after ALPPS
Parent experiences with paediatric allergy pathways in the West Midlands: A qualitative study.
No full textBACKGROUND
The prevalence, severity and complexity of allergic diseases have been increasing steadily in the United Kingdom over the last few decades. Primary care physicians are often not adequately trained in allergy management while specialist services for allergy are scarce and heterogeneous. Services, therefore, have been unable to meet the rising demand. This is particularly true for paediatric allergy services in the United Kingdom.
OBJECTIVE
To understand parent experiences with paediatric allergy pathways in the West Midlands (WM) region of the United Kingdom.
METHODS
Parents of children aged between 0 and 16 years from the WM region were recruited opportunistically until thematic saturation was achieved. Eighteen semi-structured interviews were carried out and transcribed verbatim. Data were analysed on NVivo software using the framework method. Themes were identified from the transcripts as well as from existing literature.
RESULTS
Parents highlighted numerous issues related to allergy services in the region including difficulties with being taken seriously by their physicians, problems with accessing health care and issues with information and the need for additional supportive care for allergies.
CONCLUSIONS AND CLINICAL RELEVANCE
Primary care for children with allergies in the WM is disparate. Parents experience difficulties in accessing primary and secondary care services and also obtaining timely and appropriate information regarding their child's allergies. Most parents were happy to be reviewed by either specialist nurses or by consultants in the hospital. Improving accessibility and availability of reliable information as well as provision of additional services (such as psychologists and dietetics) were highlighted by parents as being important to allergy services in the region. These findings can help inform future planning and commissioning of allergy services
The Risk of Inflammatory Bowel Disease in Subjects Presenting With Perianal Abscess: Findings From the THIN Database.
No full textBACKGROUND
Perianal abscess [PA] is associated with inflammatory bowel disease [IBD]. The incidence of IBD after a diagnosis of PA and potential predictors of a future diagnosis of IBD are unknown.
METHODS
The Health Improvement Network [THIN] is a primary care database representative of the UK population. Incident cases of PA were identified between 1995 and 2017. Subjects with PA were matched to controls within the same general practice. The primary outcome was a subsequent diagnosis of Crohn's Disease [CD] or ulcerative colitis [UC]. A Cox regression model was used to assess potential predictors of a new diagnosis of CD or UC following PA.
RESULTS
The risk of CD was higher in the PA cohort compared with controls; adjusted hazard ratio [HR] 7.51 (95% confidence interval [CI] 4.86-11.62), p < 0.0001. The risk of UC was also higher in the PA cohort compared with controls; adjusted HR 2.03 [1.38-2.99], p < 0.0001. Anaemia in men (HR 2.82 [1.34-5.92], p = 0.002), and use of antidiarrhoeal medications (HR 2.70 [1.71-4.25], p < 0.0001) were associated with an increased risk of CD following PA. Anaemia in men (HR 2.58 [1.09-6.07], p = 0.03), diarrhoea (HR 2.18 [1.23-3.85], p = 0.007), and use of anti-diarrhoeal medication (HR 2.27 [1.19-4.30], p = 0.012) were associated with an increased risk of UC following PA.
CONCLUSION
Subjects with PA are at an increased risk of subsequent diagnosis of CD and UC. Clinicians should strongly consider investigation for IBD in young patients presenting with diarrhoea and anaemia [in males] following PA. Future research should discern appropriate screening strategies for this high-risk cohort
Important lessons about testosterone therapy- weight loss vs. testosterone therapy for symptom resolution, classical vs. functional hypogonadism, and shortterm vs. lifelong testosterone therapy.
No full textIn this commentary, we highlight important findings from a notable RCT by Ng Tang Fui et al. 2016 which investigated the effects of testosterone treatment in dieting obese men. First, a myopic focus on weight loss can detract from important improvements in body composition. Second, while weight loss in obese men may increase testosterone levels, this increase is commonly not enough to result in an improvement in symptoms associated with testosterone deficiency. Third, the RCT by Ng Tang Fui et al. adds evidence to the growing number of clinical trials showing that testosterone therapy should not be restricted to men with classical hypogonadism. Finally, the beneficial effects of testosterone therapy are not maintained after cessation of treatment. Currently, the British Society for Sexual Medicine guidelines are the only clinical guidelines which acknowledge that weight loss per se does not automatically translate to resolution of hypogonadal symptoms, that testosterone therapy can greatly benefit men with testosterone deficiency who do not have classical hypogonadism, and that cessation of testosterone therapy causes reappearance of symptoms and reversal of benefits. Lifelong testosterone therapy is therefore recommended for persistent health benefits in most men with testosterone deficiency. Physicians and patients need to be informed of this
The Preventative Effect of Hydrocolloid Dressings on Nasal Bridge Pressure Ulceration in Acute Non-Invasive Ventilation.
No full textBackground
Non-invasive ventilation (NIV) is a valuable treatment in the management of acute hypercapnic respiratory failure. NIV is not without risks. One such adverse effect is the development of pressure ulcers over the nasal bridge which have an incidence of up to 20% of patients requiring NIV in this setting. The role of medical devices in the development of hospital acquired pressure ulcers has been increasingly recognised with 10-35% of all hospital acquired ulcers attributed to medical devices. Guidelines on acute NIV use suggest good skin care strategies. However, data on the magnitude of the problem of nasal bridge pressure ulceration and the effect of proactive preventative steps remains scant.
Method
A quality improvement project was designed to reduce the incidence of nasal bridge pressure ulcers during acute NIV. Hydrocolloid dressings were placed over the nasal bridge in all patients requiring NIV between 30 October 2015 and the 29 October 2016. Tissue viability was assessed daily with new pressure ulceration defined as grade 2 or above. Rates of nasal bridge pressure ulcers were compared to all patients requiring NIV in the 12-month period prior to intervention.
Results
In Group 1, there were 161 admissions and 9 grade 2 pressure ulcers from 666 NIV bed-days. In Group 2 there were 134 admissions and 0 pressure ulcers from 718 NIV bed-days. There was a statistically significant reduction in grade 2 pressure ulceration rates (p= 0.0013) in Group 2 compared to Group 1.
Conclusion
Application of an early prophylactic pressure-relieving hydrocolloid nasal dressing reduces the risk of developing grade 2 pressure ulcers in patients in patients requiring acute NIV
Instrument-based tests for measuring anterior chamber cells in uveitis: a systematic review protocol.
No full textBACKGROUND
Uveitis describes a group of inflammatory conditions affecting the eye. The ability to monitor inflammatory changes in anterior uveitis is crucial in clinical practice for making treatment decisions and in clinical trials for testing therapeutic agents. The current standard for quantifying anterior segment inflammation is clinical slit-lamp examination findings classified using the Standardisation of Uveitis Nomenclature (SUN) grading system. Such clinical grading systems rely on a subjective estimation using the slit lamp and are often non-linear and non-continuous scales, with large increases in cell count between each grade. Novel instrument-based technologies have emerged over the last few decades, which can provide objective and quantifiable measurements. This review will evaluate the reliability of such technologies and their level of agreement with anterior chamber (AC) cell count using clinical slit-lamp examination.
METHODS
Standard systematic review methodology will be used to identify, select and extract data from studies that report the use of any instrument-based technology in the assessment of AC cells. Searches will be conducted through bibliographic databases (MEDLINE, EMBASE and Cochrane Library), clinical trial registries and the grey literature. No restrictions will be placed on language or year of publication. The outcomes of interest are the correlation of index test measurements of AC cells with clinical grading systems using slit-lamp examination and the reliability of each index test identified. Quality assessment will be undertaken using QUADAS2. Degree of correlation between the index and reference test measures will be pooled and meta-analysed if appropriate.
DISCUSSION
A number of instrument-based tools are available for measuring AC cells. This review will evaluate the technologies available and measure the level of correlation of these alternative methods with clinical grading systems as well as their performance in reliability and repeatability. The findings of this review will identify those objective, instrument-based technologies which show good utility for measuring AC cells in a quantifiable way and which warrant further exploration for their sensitivity and reliability over the current standard.
SYSTEMATIC REVIEW REGISTRATION
PROSPERO CRD42017084156 (Liu X, Moore DJ, Denniston AK). Instrument-based tests for measuring anterior chamber (AC) cells in uveitis: a systematic review. 2017). Study screening stage is complete. Data extraction stage has not yet commenced
Characterization and treatment of congenital thrombotic thrombocytopenic purpura.
No full textCongenital Thrombotic Thrombocytopenic Purpura (cTTP) is an ultra rare thrombomicroangiopathy caused by an inherited deficiency of ADAMTS13. There is limited data on the genotype-phenotype correlation and no consensus on treatment. We reviewed the largest cohort of cTTP cases, diagnosed in the UK, over the past 15 years. 73 cases of cTTP were diagnosed, confirmed by genetic analysis. 93% were alive at the time of review. 36% had homozygous mutations and 64% compound heterozygous mutations. Two presentation peaks were seen, childhood (median diagnosis age 3.5 years) and adulthood, typically related to pregnancy (median diagnosis age 31 years). Genetic mutations differed by age of onset with pre-spacer mutations more likely to be associated with childhood-onset (p=0.0011). 69% of adult presentations were associated with pregnancy. Fresh Frozen Plasma (FFP) and intermediate purity factor VIII concentrate were used as treatment. 88% of patients with normal blood counts but headaches, lethargy or abdominal pain reported symptom resolution with prophylactic therapy although the most common currently used regimen of three weekly FFP proved insufficient for 70% of patients and weekly or fortnightly infusions were required. Stroke incidence was significantly reduced in patients receiving prophylactic therapy (2% versus 17%, p=0.04). Long term, there is a risk of end organ damage, seen in 75% of patients with a late diagnosis of cTTP. In conclusion, pre-spacer mutations are associated with earlier development of cTTP symptoms. Prophylactic ADAMTS13 replacement decreases the risk of end organ damage such as ischemic stroke and resolved previously unrecognized symptoms in patients with non-overt disease
British Society for Immunology/United Kingdom Primary Immunodeficiency Network consensus statement on managing non-infectious complications of Common Variable Immunodeficiency Disorders.
No full textCommon variable immunodeficiency (CVID) represents a heterogeneous group of rare disorders. There is considerable morbidity and mortality as a result of non-infectious complications and this presents clinicians with management challenges. Clinical guidelines to support the management of CVID are urgently required. The UK Primary Immunodeficiency Network and the British Society for Immunology funded a joint project, to address this. A modified Delphi Survey was conducted for the assessment, diagnosis and treatment of the non-infectious blood, respiratory, gut and liver complications of CVID. A steering group of ten consultant immunologists and one nurse specialist developed and reviewed the survey statements and agreed the final recommendations. In total 22 recommendations and three areas for research were developed. This article is protected by copyright. All rights reserved
Intravenous pulse methylprednisolone for induction of remission in severe ANCA associated Vasculitis: a multi-center retrospective cohort study.
No full textBACKGROUND
Intravenous pulse methylprednisolone (MP) is commonly included in the management of severe ANCA associated vasculitis (AAV) despite limited evidence of benefit. We aimed to evaluate outcomes in patients who had, or had not received MP, along with standard therapy for remission induction in severe AAV.
METHODS
We retrospectively studied 114 consecutive patients from five centres in Europe and the United States with a new diagnosis of severe AAV (creatinine > 500 μmol/L or dialysis dependency) and that received standard therapy (plasma exchange, cyclophosphamide and high-dose oral corticosteroids) for remission induction with or without pulse MP between 2000 and 2013. We evaluated survival, renal recovery, relapses, and adverse events over the first 12 months.
RESULTS
Fifty-two patients received pulse MP in addition to standard therapy compared to 62 patients that did not. There was no difference in survival, renal recovery or relapses. Treatment with MP associated with higher risk of infection during the first 3 months (hazard ratio (HR) 2.7, 95%CI [1.4-5.3], p = 0.004) and higher incidence of diabetes (HR 6.33 [1.94-20.63], p = 0.002), after adjustment for confounding factors.
CONCLUSIONS
The results of this study suggest that addition of pulse intravenous MP to standard therapy for remission induction in severe AAV may not confer clinical benefit and may be associated with more episodes of infection and higher incidence of diabetes
Echocardiography and monitoring patients receiving dopamine agonist therapy for hyperprolactinaemia: A joint position statement of the British Society of Echocardiography, the British Heart Valve Society and the Society for Endocrinology.
No full textThis is a joint position statement of the British Society of Echocardiography, the British Heart Valve Society and the Society for Endocrinology on the role of echocardiography in monitoring patients receiving dopamine agonist (DA) therapy for hyperprolactinaemia. Evidence that DA pharmacotherapy causes abnormal valve morphology and dysfunction at doses used in the management of hyperprolactinaemia is extremely limited. Evidence of clinically significant valve pathology is absent, except for isolated case reports around which questions remain. Attributing change in degree of valvar regurgitation, especially in mild and moderate tricuspid regurgitation, to adverse effects of DA in hyperprolactinaemia should be avoided if there are no associated pathological changes in leaflet thickness, restriction or retraction. Note must be taken that even where morphological change in leaflet structure and function may be suspected, grading is semi-quantitative on echocardiography and may vary between different machines, ultrasound settings and operators. Decisions regarding discontinuation of medication should only be made after review of serial imaging by an echocardiographer experienced in analysing drug-induced valvulopathy or carcinoid heart disease. A standard transthoracic echocardiogram should be performed before a patient starts DA therapy for hyperprolactinaemia. Repeat transthoracic echocardiography should then be performed at 5 years after starting cabergoline in patients taking a total weekly dose less than or equal to 2 mg. If there has been no change on the 5-year scan, repeat echocardiography could continue at 5-yearly intervals. If a patient is taking more than a total weekly dose of 2 mg, then annual echocardiography is recommended