Research protocol for the Paraesophageal hernia symptom tool, a prospective multi-center cohort study to identify the need and threshold for surgery and assess the symptom response to surgery.

Large hiatus hernias with a significant paraesophageal component (types II-IV) have a range of insidious symptoms. Management of symptomatic hernias includes conservative treatment or surgery. Currently, there is no paraesophageal hernia disease-specific symptom questionnaire. As a result, many clinicians rely on the health-related quality of life questionnaires designed for gastro-esophageal reflux disease (GORD) to assess patients with hiatal hernias pre- and postoperatively. In view of this, a paraesophageal hernia symptom tool (POST) was designed. This POST questionnaire now requires validation and assessment of clinical utility. Twenty-one international sites will recruit patients with paraesophageal hernias to complete a series of questionnaires over a five-year period. There will be two cohorts of patients-patients with paraesophageal hernias undergoing surgery and patients managed conservatively. Patients are required to complete a validated GORD-HRQL, POST questionnaire, and satisfaction questionnaire preoperatively. Surgical cohorts will also complete questionnaires postoperatively at 4-6 weeks, 6 months, 12 months, and then annually for a total of 5 years. Conservatively managed patients will repeat questionnaires at 1 year. The first set of results will be released after 1 year with complete data published after a 5-year follow-up. The main results of the study will be patient's acceptance of the POST tool, clinical utility of the tool, assessment of the threshold for surgery, and patient symptom response to surgery. The study will validate the POST questionnaire and identify the relevance of the questionnaire in routine management of paraesophageal hernias.

Longer-term outcomes of gastroesophageal reflux disease treated with magnetic sphincter augmentation.

Surgical intervention for gastroesophageal reflux disease (GERD) has historically been limited to fundoplication. Magnetic sphincter augmentation (MSA) is a less invasive alternative that was introduced 15 years ago, and it may have a superior side-effect profile. To date, however, there has been just a single published study reporting outcomes in a UK population. This study reports quality-of-life (QOL) outcomes and antacid use in patients undergoing MSA, with a particular focus on postoperative symptoms and those with severe reflux. A single-center cohort study was carried out to assess the QOL outcomes and report long-term safety outcomes in patients undergoing MSA. GERD-health-related quality of life (GERD-HRQL) and Reflux Symptom Index (RSI) scores were collected preoperatively, and immediately postoperatively, at 1-, 2-, 3-, and 5-year follow-up time points. All patients underwent preoperative esophagogastroduodenoscopy, impedance, and manometry. Two hundred and two patients underwent laparoscopic MSA over 9 years. The median preoperative GERD-HRQL score was 31, and the median RSI score was 17. There was a reduction in all scores from preoperative values to each time point, which was sustained at 5-year follow-up; 13% of patients had a preoperative DeMeester score of >50, and their median preoperative GERD-HRQL and RSI scores were 32 and 15.5, respectively. These were reduced to 0 at the most recent follow-up. There was a significant reduction in antacid use at all postoperative time points. Postoperative dilatation was necessary in 7.4% of patients, and the device was removed in 1.4%. Erosion occurred in no patients. MSA is safe and effective at reducing symptom burden and improving QOL scores in patients with both esophageal and laryngopharyngeal symptoms, including those with severe reflux.

Development of the ParaOesophageal hernia SympTom (POST) tool.

BACKGROUND: The aim of this study was to develop a symptom severity instrument (ParaOesophageal hernia SympTom (POST) tool) specific to para-oesophageal hernia (POH). METHODS: The POST tool was developed in four stages. The first was establishment of a Steering Committee. In the second stage, items were generated through a systematic review and online scoping survey of international experts. In the third stage, a three-round modified Delphi consensus process was conducted with a group of international experts who were asked to rate the importance of candidate items. An a priori threshold for inclusion was set at 80 per cent. The modified Delphi process culminated in a consensus meeting to develop the first iteration of the tool. In the final stage, two international patient workshops were held to assess the content validity and acceptability of the POST tool. RESULTS: The systematic review and scoping survey generated 64 symptoms, refined to 20 for inclusion in the modified Delphi consensus process. Twenty-six global experts participated in the Delphi consensus process. Five symptoms reached consensus across two rounds: difficulty getting solid foods down, chest pain after meals, difficulty getting liquids down, shortness of breath only after meals, and an early feeling of fullness after eating. The subsequent patient workshops deemed these five symptoms to be relevant and suggested that reflux should be included; these were taken forward to create the final POST tool. CONCLUSION: The POST tool is the first instrument designed to capture POH-specific symptoms. It will allow clinicians to standardize reporting of symptoms of POH and evaluate the response to surgical intervention.

Quality of life and symptom assessment in paraesophageal hernias: a systematic literature review of reporting standards.

BACKGROUND: Paraesophageal hernias (PEH) present with a range of symptoms affecting physical and mental health. This systematic review aims to assess the quality of reporting standards for patients with PEH, identify the most frequently used quality of life (QOL) and symptom severity assessment tools in PEH and to ascertain additional symptoms reported by these patients not captured by these tools. METHODS: A systematic literature review according to PRISMA protocols was carried out following a literature search of MEDLINE, Embase and Cochrane databases for studies published between January 1960 and May 2020. Published abstracts from conference proceedings were included. Data on QOL tools used and reported symptoms were extracted. RESULTS: This review included 220 studies reporting on 28 353 patients. A total of 46 different QOL and symptom severity tools were used across all studies, and 89 different symptoms were reported. The most frequently utilized QOL tool was the Gastro-Esophageal Reflux Disease-Health related quality of life questionnaire symptom severity instrument (47.7%), 57.2% of studies utilized more than 2 QOL tools and 'dysphagia' was the most frequently reported symptom, in 55.0% of studies. Notably, respiratory and cardiovascular symptoms, although less common than GI symptoms, were reported and included 'dyspnea' reported in 35 studies (15.9%). CONCLUSIONS: There lacks a QOL assessment tool that captures the range of symptoms associated with PEH. Reporting standards for this cohort must be improved to compare patient outcomes before and after surgery. Further investigations must seek to develop a PEH specific tool, that encompasses the relative importance of symptoms when considering surgical intervention and assessing symptomatic improvement following surgery.

Variations in arterial pedal circulation in idiopathic congenital talipes equinovarus: a systematic review.

Variations in pedal circulation in congenital talipes equinovarus (CTEV) are well documented. There is a reported risk of vascular injury to the posterior tibial artery (PTA) during operative procedures for CTEV, potentially leading to necrosis and amputation. The aim of this systematic review was to identify the most common anomalies in arterial pedal circulation in CTEV and to determine the relevance of these to clinical practice. The systematic review was registered on PROSPERO and was carried out according to Preferred Reporting Items for Systematic Reviews and Meta Analyses guidelines by two independent reviewers. Studies that examined pedal circulation in idiopathic CTEV were included. Articles that studied nonidiopathic CTEV and those not published in English were excluded. Data extracted included patient demographics, imaging modalities, and findings. A total of 14 articles satisfied the inclusion criteria, including 192 patients (279 clubfeet), aged 0-13.5 years, at various stages in their treatment. Imaging modalities included arteriography (n = 5), duplex ultrasound (n = 5), magnetic resonance angiography (n = 2), and direct visualization intraoperatively (n = 2). The dorsalis pedis was most frequently reported as absent (21.5%), and the anterior tibial artery (ATA) was most frequently reported as hypoplastic (18.3%). Where reported (n = 36 feet), 61% of patients were noted to have a dominant supply from the PTA. The most common variation in pedal circulation in CTEV is diminished supply from ATA and dorsalis pedis, although there are documented anomalies in all of the vessels supplying the foot. We therefore recommend routine Doppler ultrasound imaging prior to operative intervention in CTEV.

Effect of Pulsed Methylprednisolone on Pain, in Patients with HTLV-1-Associated Myelopathy.

HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) is an immune mediated myelopathy caused by the human T-lymphotropic virus type 1 (HTLV-1). The efficacy of treatments used for patients with HAM/TSP is uncertain. The aim of this study is to document the efficacy of pulsed methylprednisolone in patients with HAM/TSP. Data from an open cohort of 26 patients with HAM/TSP was retrospectively analysed. 1g IV methylprednisolone was infused on three consecutive days. The outcomes were pain, gait, urinary frequency and nocturia, a range of inflammatory markers and HTLV-1 proviral load. Treatment was well tolerated in all but one patient. Significant improvements in pain were: observed immediately, unrelated to duration of disease and maintained for three months. Improvement in gait was only seen on Day 3 of treatment. Baseline cytokine concentrations did not correlate to baseline pain or gait impairment but a decrease in tumour necrosis factor-alpha (TNF-α) concentration after pulsed methylprednisolone was associated with improvements in both. Until compared with placebo, treatment with pulsed methylprednisolone should be offered to patients with HAM/TSP for the treatment of pain present despite regular analgesia.

Lifelong antiretroviral therapy or HIV cure: The benefits for the individual patient.

There are an estimated 35 million people living with human immunodeficiency virus (HIV) globally, 19 million of whom are unaware of their HIV status and, in the absence of antiretroviral therapy (ART), will have a shortened life expectancy. Although ART remains the "gold standard" for treatment of HIV infection, the requirement for lifelong treatment poses multiple challenges for the patient. These include stigma, an untenable pill burden, side effects, and the threat of viral resistance in the case of non-compliance. This review evaluates the challenges of accessing, delivering, and sustaining ART for people living with HIV and will discuss the case for pursuing a goal of HIV cure, the potential benefits of such a cure for the individual patient, and the current potential candidates for such a cure.