Evidence-based management of epistaxis in hereditary haemorrhagic telangiectasia

2015 ◽  
Vol 129 (5) ◽  
pp. 410-415 ◽  
Author(s):  
I Syed ◽  
V S Sunkaraneni
Keyword(s):  
Assessment Tool ◽  
Case Reports ◽  
Treatment Algorithm ◽  
Case Series ◽  
Team Approach ◽  
Hereditary Haemorrhagic Telangiectasia ◽  
Questionnaire Studies ◽  
Randomised Controlled ◽  
Specific Management

AbstractBackground:There are currently no guidelines in the UK for the specific management of hereditary haemorrhagic telangiectasia related epistaxis. The authors aimed to review the literature and provide an algorithm for the management of hereditary haemorrhagic telangiectasia related epistaxis.Method:The Medline and Embase databases were interrogated on 15 November 2013 using the search items ‘hereditary haemorrhagic telangiectasia’ (title), ‘epistaxis’ (title) and ‘treatment’ (title and abstract), and limiting the search to articles published in English.Results:A total of 46 publications were identified, comprising 1 systematic review, 2 randomised, controlled trials, 27 case series, 9 case reports, 4 questionnaire studies and 3in vitrostudies.Conclusion:There is a lack of high-level evidence for the use of many of the available treatments for the specific management of epistaxis in hereditary haemorrhagic telangiectasia. Current management should be based on a multidisciplinary team approach involving both a hereditary haemorrhagic telangiectasia physician and an ENT surgeon, especially when systemic therapy is being considered. The suggested treatment algorithm considers that the severity of epistaxis merits intervention at different levels of the treatment ladder. The patient should be assessed using a reproducible validated assessment tool, for example an epistaxis severity score, to guide treatment. More research is required, particularly in the investigation of topical agents targeting the development and fragility of telangiectasiae in hereditary haemorrhagic telangiectasia.

scholarly journals Immunoglobulin G4-related hypertrophic pachymeningitis

2019 ◽  
Vol 6 (4) ◽  
pp. e568 ◽  
Author(s):  
Michaël Levraut ◽  
Mikaël Cohen ◽  
Saskia Bresch ◽  
Caroline Giordana ◽  
Fanny Burel-Vandenbos ◽  
...  
Keyword(s):  
Steroid Therapy ◽  
Case Reports ◽  
Treatment Algorithm ◽  
Elevated Serum ◽  
Case Series ◽  
Hypertrophic Pachymeningitis ◽  
Immunoglobulin G4 ◽  
Systemic Involvement ◽  
Meningeal Involvement ◽  
Serum Igg4

ObjectiveMeningeal involvement in Immunoglobulin G (IgG)-4-related disease is rare and only described in case reports and series. Because a review into the disease is lacking, we present 2 cases followed by a literature review of IgG4-related hypertrophic pachymeningitis (IgG4-HP).MethodsTwo IgG4-HP cases were reported, one involving the spinal cord and responding to surgical management and a second involving the brain and responding to Rituximab therapy. We then review clinical cases and case-series of histologically proven IgG4-HP that were published in the PubMed-NCBI database.ResultsForty-two case reports and 5 case-series were studied (60 patients, 20 women). The median age was 53. Eighteen patients had systemic involvement and 24 had single-organ IgG4-HP. Fifty-five percent of patients had an elevated serum IgG4. Treatment was surgical in 20/53 cases. Steroid therapy and immunosuppressors were effective in 85% and more than 90% of the cases, respectively. The rate of disease relapse was 42.1% after steroid therapy was discontinued.Discussion/conclusionIgG4-HP is characterized by the lack of extra-neurologic organ-involvement and systemic signs. Histopathologic studies should be performed as it is crucial for diagnosis because serum markers are rarely informative. 18F-FDG positon tomography can be useful to characterize systemic forms. There is no specific CSF marker for IgG4-HP and the diagnostic value of CSF IgG4 levels needs to be studied with larger samples. We provide a treatment algorithm for IgG4-HP. Such treatment strategies rely on early surgery, steroids, and early immunosuppressive therapy to prevent neurologic complications.

scholarly journals Voriconazole versus Itraconazole for the Initial and Step-Down Treatment of Histoplasmosis: A Retrospective Cohort

2020 ◽  
Author(s):  
Michael Joshua Hendrix ◽  
Lindsey Larson ◽  
Adriana M Rauseo ◽  
Sasinuch Rutjanawech ◽  
Alexander D Franklin ◽  
...  
Keyword(s):  
Retrospective Cohort ◽  
Proportional Hazards ◽  
Histoplasma Capsulatum ◽  
Case Reports ◽  
Case Series ◽  
Clinical Information ◽  
Cox Proportional Hazards ◽  
Heaviside Function ◽  
Step Down

Abstract Background Itraconazole is the preferred azole for histoplasmosis in the current Infectious Diseases Society of America guidelines. Voriconazole is increasingly used as treatment for histoplasmosis; it has in-vitro activity against Histoplasma capsulatum and has shown success in case reports and small case series but may have a lower barrier to resistance. No comparative studies have been published. Methods We constructed a single-center retrospective cohort of adult patients diagnosed with histoplasmosis from 2002 to 2017. Individual charts were reviewed to gather clinical information including demographics, clinical features, immune status, treatments, and mortality. Patients were categorized based on initial choice of azole, either as initial treatment or as step-down therapy from amphotericin B. Initial therapies with other azoles were excluded. Mortality was compared using a multivariable Cox proportional hazards with Heaviside function at 42 days. Results We identified 261 cases of histoplasmosis from 2002 to 2017. After excluding patients not treated with itraconazole or voriconazole, 194 patients remained. 175 (90%) patients received itraconazole and 19 (10%) received voriconazole. There were no significant demographic differences between patient populations receiving either azole as their initial azole treatment. Death at 180 days occurred in 41 patients (23.4%) in the itraconazole group and 6 patients (31.6%) in the voriconazole group. Patients on voriconazole had a statistically significant increase in mortality during the first 42 days after initiation of treatment when compared to patients receiving itraconazole (HR 4.30 [95% CI 1.3-13.9, p 0.015]) when controlled for other risk factors. Conclusion Voriconazole in histoplasmosis was associated with increased mortality in the first 42 days compared to itraconazole.

scholarly journals P416 Results of the first paediatric randomised controlled trial of faecal microbiota transplant for ulcerative colitis

2020 ◽  
Vol 14 (Supplement_1) ◽  
pp. S379-S380
Author(s):  
N Pai ◽  
J Popov ◽  
L Hill ◽  
E Hartung ◽  
K Grzywacz ◽  
...  
Keyword(s):  
Ulcerative Colitis ◽  
Clinical Response ◽  
Healthy Donor ◽  
Activity Index ◽  
Case Reports ◽  
Case Series ◽  
Faecal Microbiota ◽  
Open Label ◽  
Randomised Controlled

Abstract Background The role of faecal microbiota transplant (FMT) for the treatment of ulcerative colitis (UC) has been reported across 4 randomised-controlled trials (RCT) in adults. Promising data have emerged from small, open-label paediatric case series and case reports but a proper blinded, placebo-controlled RCT has not been described in children. We report results from the first multicentre RCT of FMT in paediatric UC patients, conducted over 36 months in Ontario and Quebec, Canada. Methods We enrolled 25 children, ages 4–17 years old with active UC across two tertiary IBD clinics. Patients had active inflammation and remained on stable doses of medication at entry. Blinded participants received enemas containing healthy donor stool (active) or normal saline (placebo), 2×/week for 6 weeks. Faecal calprotectin (fCal), C-reactive protein (CRP), and paediatric ulcerative colitis activity index (PUCAI) scores were compared between groups during intervention, and at four follow-up time points over 30 weeks. Donor and recipient stools were measured for 16s rRNA and metagenomics analyses. Results In intention-to-treat (ITT) analysis, FMT (n = 13) at 6 weeks was more likely to improve clinical response (OR 9.3, 95% CI [0.7, 122.6]), CRP (OR 4.7, 95% CI [0.8, 28.4]), and fCal (OR 13.3, 95% CI [1.1, 166.4]) from baseline compared with placebo (n = 12). FMT at 30 weeks was also more likely than placebo to improve clinical response, CRP, and fCal (Table 1). In ITT analysis of the open-label arm (n = 7), FMT at 6 weeks and 30 weeks decreased CRP (−42.9%, −28.6%), fCal (−28.6%, −42.9%), and PUCAI score (−14.3%, −42.9%) from baseline. Conclusion Serial FMT enemas containing healthy donor microbiota led to greater improvements in serum and stool inflammatory markers, and rates of clinical response, in paediatric patients with active UC compared with placebo. These improvements largely persisted beyond 6 months after final FMT treatment. This study offers the strongest preliminary evidence, from a blinded, placebo-controlled multicentre RCT for the role of FMT in the management of paediatric UC.

scholarly journals Hereditary haemorrhagic telangiectasia and pregnancy: a review of the literature

2020 ◽  
Vol 15 (1) ◽  
Author(s):  
Olivier Dupuis ◽  
Laura Delagrange ◽  
Sophie Dupuis-Girod
Keyword(s):  
Heart Failure ◽  
Literature Review ◽  
Arteriovenous Malformations ◽  
Case Reports ◽  
Case Series ◽  
Main Body ◽  
Hereditary Haemorrhagic Telangiectasia ◽  
Review Of The Literature ◽  
Management Decisions ◽  
Recurrent Epistaxis

Abstract Background Hereditary haemorrhagic telangiectasia (HHT) is a dominantly inherited genetic vascular disorder that has prevalence of 1:5000 to 1:8000, and which is characterised by recurrent epistaxis, cutaneous telangiectasia, and arteriovenous malformations (AVMs) that affect many organs including the lungs, gastrointestinal tract, liver, and central nervous system. The aim here was to carry out a review of the literature on HHT complications during pregnancy in order to guide management decisions. Main body A literature review was carried out to analyse all publications on complications that occurred during pregnancy in women with HHT. The PubMed/Medline and Scopus databases were searched. The complications observed in HHT women during pregnancy were then described. The authors identified 5 case series and 31 case reports that describe the evolution of 1577 pregnancies in 630 women with HHT. The overall maternal death rate described in the case series was estimated at 1.0% of pregnancies in the case series and 2 maternal deaths occurred in 31 pregnancy case reports. Severe maternal complications occurred in 2.7 to 6.8% of pregnancies in the case series. Severe complications occurred mostly in the second and third trimester in non-diagnosed and non-screened HHT patients. Severe complications were related to visceral involvement. The most frequent complications were related to pulmonary arteriovenous malformations (PAVMs) (haemothorax (n = 10), haemoptysis (n = 4), and severe hypoxaemia (n = 3)). Neurological complications were related to PAVMs in one case (right to left shunt) and to cerebral arteriovenous malformations (CAVM) and intracranial haemorrhage in 2 cases. Complications were related to hepatic arteriovenous malformations (HAVMs) in 8 cases (acutely decompensated heart failure due to hepatic involvement (n = 1), dyspnoea related to heart failure (n = 5), and hepatobiliary necrosis (n = 2)). Conclusion Based on the literature review, most pregnancies in HHT women occur normally. However, these pregnancies should be considered high-risk, given the potential life-threatening events related to AVM rupture. Furthermore, there is currently no international consensus regarding the medical follow-up of pregnancy in women with HHT and the aim here was to carry out a review of the literature in order to guide screening and management decisions for this rare disease.

scholarly journals Emergency Management of Paraphimosis

2003 ◽  
Vol 10 (4) ◽  
pp. 253-257 ◽  
Author(s):  
Ch Chung
Keyword(s):  
Case Reports ◽  
Data Extraction ◽  
Treatment Options ◽  
Case Series ◽  
Treatment Modalities ◽  
Invasive Treatment ◽  
Manual Search ◽  
Study Selection ◽  
Library Network ◽  
Randomised Controlled

Objective To review the treatment modalities available for paraphimosis, with special emphasis on those applicable to the emergency department. Data source Relevant medical literature was searched through MEDLINE, EMBASE, CINAHL, and Cochrane Database. Manual search was performed in books on Urology, General Surgery and Emergency Medicine available in the Hospital Library. Further information was obtained through the Internet at < www.infoseek.com >. References cited in articles were also retrieved. Study selection Key words for the literature, Internet and textbook search were ‘paraphimosis’ and ‘treatment’. All available years of study were reviewed. Data extraction Relevant full text articles were obtained through the hospital library network. Original articles, review papers, medical practice, case reports, and relevant book chapters were reviewed. Data synthesis There were no prospective, randomised, controlled studies available. The majority were case series and expert experience or opinions only. Currently, a multitude of non-invasive and invasive treatment options are available, including manual reduction, help of non-crushing tissue forceps, puncture technique and dorsal slit. Conclusion All treatment methods are within the capability of the emergency physician. Hospitalization should rarely be required, unless there are serious complications.

Antiseptic Use in Orthopaedic Wounds

2020 ◽  
pp. 46-61
Author(s):  
Fahima A. Begum ◽  
Tiffanie-Marie Borg ◽  
Hamed Mazoochy ◽  
Nima Heidari
Keyword(s):  
Strong Evidence ◽  
Case Reports ◽  
Single Agent ◽  
Case Series ◽  
Human Studies ◽  
In Vivo Studies ◽  
Foot And Ankle ◽  
Skin Preparation ◽  
Systemic Complications

Objectives: The aim of this study is to review the available literature addressing the safety and efficacy of antiseptics in surgical wounds. The different antiseptic solutions, irrigation volumes, time scales and delivery methods have been compared so that evidence-based recommendations on antiseptic use in orthopaedic, foot and ankle surgical procedures can be proposed. Methods: A literature search was performed using the online databases Medline and EMBase to identify in-vitro and in-vivo studies pertaining to antiseptic use in an orthopaedic context. Terms including antiseptic, irrigation fluid, bacitracin, hydrogen peroxide, povidone-iodine and chlorhexidine were searched. Literature published in English from inception to July 2020 in which the full text was accessible was considered for inclusion. Cellular and animal studies were included on the basis that authors analysed antiseptic efficacy and/or toxic effect of antiseptic on cells present in orthopaedic wounds. Clinical studies that met the criteria for inclusion in this review assessed antiseptic use in a surgical context, with a focus on foot and ankle procedures.         These included case reports, case series, case control, prospective and retrospective studies as well as randomised controlled trials. Studies were categorised as in-vitro, animal and human studies. Twenty-three, eleven and forty-four studies were identified as in-vitro, animal and       human studies respectively. These have been summarised and presented herein in a narrative format. Results: There is strong evidence that skin preparation with antiseptics before orthopaedic procedures reduces the risk of post-operative infection. Conclusion: Routine prophylactic intra-operative antiseptic use should be performed with caution as they increase the risk of local and systemic complications. However, there is strong evidence supporting the use of antiseptics pre-operatively when preparing the skin. Determining the best antiseptic preparation remains a matter of debate since a single agent or solution is not effective against all organisms. Further research is therefore needed to assess the efficacy of antiseptics in prevention and treatment of infections.

Subcutaneous levetiracetam for the management of seizures at the end of life

2017 ◽  
Vol 8 (2) ◽  
pp. 129-135 ◽  
Author(s):  
Anna Elizabeth Sutherland ◽  
John Curtin ◽  
Victoria Bradley ◽  
Olivia Bush ◽  
Maggie Presswood ◽  
...  
Keyword(s):  
Palliative Care ◽  
Literature Review ◽  
End Of Life ◽  
Terminal Care ◽  
Seizure Control ◽  
Case Reports ◽  
Subcutaneous Administration ◽  
Case Series ◽  
Comprehensive Literature Review ◽  
Randomised Controlled

ObjectivesTo report the results of a combined case series analysis of subcutaneous levetiracetam (Keppra) for the management of seizures in palliative care patients.MethodsA comprehensive literature review on the use of subcutaneous levetiracetam was performed, and these data were combined with a prospective observational audit of its use in terminal care undertaken in a regional palliative care network.Results7 papers were identified from the literature review-four case reports and three observational case series-reporting on a total of 53 cases where subcutaneous levetiracetam was administered.We report 20 further cases of subcutaneous levetiracetam administration from a prospective observational audit. Doses ranged from 250mg to 4000 mg daily. Oral to subcutaneous conversion ratios where stated were 1:1. Levetiracetam was reported as the sole administered antiepileptic drug (AED) in eight cases, and no seizures were reported until death in five cases. Five were switched back to enteral levetiracetam. In seven cases, levetiracetam was combined with AEDs to provide seizure control at the end of life. There was one report of a sterile abscess after 25 days of continuous subcutaneous administration.ConclusionsCombined analysis of 73 reported cases of subcutaneous levetiracetam suggests this treatment may have a role in the management of seizures at the end of life. However, randomised controlled trials are urgently needed to establish the efficacy and tolerability of subcutaneous levetiracetam administration. If proven to be safe and effective, subcutaneous levetiracetam offers the potential to prevent and treat seizures without causing unnecessary sedation at the end of life.

Propranolol for treating emotional, behavioural, autonomic dysregulation in children and adolescents with autism spectrum disorders

2018 ◽  
Vol 32 (6) ◽  
pp. 641-653 ◽  
Author(s):  
Ilyas Sagar-Ouriaghli ◽  
Kate Lievesley ◽  
Paramala J Santosh
Keyword(s):  
Clinical Trials ◽  
Cognitive Performance ◽  
Single Case ◽  
Case Reports ◽  
Case Series ◽  
Semantic Networks ◽  
Neural Correlates ◽  
Autism Spectrum ◽  
Autonomic Dysregulation ◽  
Randomised Controlled

Objectives: To date, there is no single medication prescribed to alleviate all the core symptoms of Autism Spectrum Disorder (ASD; National Institute of Health and Care Excellence, 2016). Both serotonin reuptake inhibitors and drugs for psychosis possess therapeutic drawbacks when managing anxiety and aggression in ASD. This review sought to appraise the use of propranolol as a pharmacological alternative when managing emotional, behavioural and autonomic dysregulation (EBAD) and other symptoms. Materials and methods: Sixteen reports examined the administration of propranolol in the context of ASD. Results: Sixteen reports broadly covered cognitive domains, neural correlates, and behavioural domains. From the eight single-dose clinical trials, propranolol led to significant improvements in cognitive performance – verbal problem solving, social skills, mouth fixation, and conversation reciprocity; and changes in neural correlates – improvement in semantic networks and functional connectivity. The remaining eight case series and single case reports showed improvements in EBAD, anxiety, aggressive, self-injurious and hypersexual behaviours. Additionally, propranolol significantly improved similar behavioural domains (aggression and self-injury) for those with acquired brain injury. Conclusion: This review indicates that propranolol holds promise for EBAD and cognitive performance in ASD. Given the lack of good quality clinical trials, randomised controlled trials are warranted to explore the efficacy of propranolol in managing EBAD in ASD.

scholarly journals Profile of Scientific Production on Ankyloglossia

2020 ◽  
pp. 1-13
Author(s):  
Roberta Lopes de Castro Martinelli ◽  
Reinaldo Jordão Gusmão ◽  
María Paz Moya Daza ◽  
Irene Queiroz Marchesan ◽  
Giédre Berretin-Felix
Keyword(s):  
Clinical Trials ◽  
Randomized Clinical Trials ◽  
Case Reports ◽  
Case Series ◽  
Scientific Production ◽  
Publication Type ◽  
Level Of Evidence ◽  
First Results ◽  
Bibliometric Review

This work aims to describe the profile of scientific production referring to ankyloglossia. For this an investigation was carried out by searching for scientific articles indexed in the electronic databases LILACS and PUBMED. For the bibliometric review, the data referring to the year of publication, type of study and level of evidence were examined and tabulated. The data were discussed on the quantitative and representative values optics. The first results allowed to analyzic 651 published studies were analyzed. Most of the research on tongue tie found correspond to descriptive studies and case series (49.31%), followed by case reports, in vitro research, in animals and literature review (24.27%), cohort and cases and controls (11.98%), specialist opinion (11.68%), randomized clinical trials (1.54%) and systematic reviews (1.22%). Few studies addressed complications during or after lingual frenulum release surgery. In conclusion Scientific production on ankyloglossia has shown an increasing increase in the last 28 years, with studies with evidence levels 1, 2 and 3 being published, whose main focus was the performance of surgery to release the lingual frenulum.

Surgical Management of Juvenile Idiopathic Arthritis Related Dentofacial Deformities

FACE ◽  
2022 ◽  
pp. 273250162110696
Author(s):  
Walter H. Wilson ◽  
Peter D. Waite ◽  
Zeyad Alrajhi ◽  
Kathlyn Powell ◽  
Randy Q. Cron ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Treatment Algorithm ◽  
Case Series ◽  
Joint Disease ◽  
Team Approach ◽  
Dentofacial Deformities ◽  
Temporomandibular Joints ◽  
Appropriate Treatment ◽  
Dentofacial Deformity ◽  
Growth Deformity

Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatologic disease of childhood and frequently affects the temporomandibular joints (TMJ). JIA TMJ management requires a team approach. Initially, TMJ JIA involvement is managed with systemic therapy or intra-articular medications to treat symptoms and limit the growth deformity, however may later require surgical intervention. This case series describes 4 patients with different presentations and treatments of juvenile idiopathic arthritis affecting the temporomandibular joints to illustrate a proposed treatment algorithm. This algorithm is not designed to be an absolute treatment regimen but a framework to help clarify the presenting problems and interventions that may be considered to treat JIA associated temporomandibular dysfunction and dentofacial deformity. This case series represents the variety of JIA temporomandibular joint disease and offers a graduated appropriate treatment algorithm.

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