Stem Cell Transplantation and Vinblastine Monotherapy for Relapsed Pediatric Anaplastic Large Cell Lymphoma: Results of the International, Prospective ALCL-Relapse Trial.

Purpose: To analyze the efficacy of a risk-stratified treatment of children with relapsed anaplastic large cell lymphoma (ALCL). The ALCL-Relapse trial (ClinicalTrials.gov identifier: NCT00317408) stratified patients according to the time of relapse and CD3 expression to prospectively test reinduction approaches combined with consolidation by allogeneic or autologous hematopoietic stem cell transplantation (SCT) and vinblastine monotherapy.

A review of the tumour spectrum of germline succinate dehydrogenase gene mutations: Beyond phaeochromocytoma and paraganglioma.

The citric acid cycle, also known as the Krebs cycle, plays an integral role in cellular metabolism and aerobic respiration. Mutations in genes encoding the citric acid cycle enzymes succinate dehydrogenase, fumarate hydratase and malate dehydrogenase all predispose to hereditary tumour syndromes. The succinate dehydrogenase enzyme complex (SDH) couples the oxidation of succinate to fumarate in the citric acid cycle and the reduction of ubiquinone to ubiquinol in the electron transport chain.

Primary pneumococcal peritonitis can be the first presentation of a familial complement factor I deficiency.

Primary pneumococcal peritonitis is a rare infection that has been described in women but has not been previously linked with immunodeficiency. The complement system plays a central role in immune defence against Streptococcus pneumoniae and, in order to evade complement attack, pneumococci have evolved a large number of mechanisms that limit complement-mediated opsonization and subsequent phagocytosis. We investigated an apparently immunocompetent woman with primary pneumococcal peritonitis and identified a family with deficiency for complement factor I.

British Gynaecological Cancer Society (BGCS) vulval cancer guidelines: Recommendations for practice.

The purpose of this guideline is to collate evidence and propose evidence-based guidelines for the diagnosis and management of adult patients with vulva carcinoma treated in the UK. Malignant melanoma may present via similar routes and will be discussed. The reader is referred to the Ano-uro-genital Mucosal Melanoma Full Guideline [1] for more detailed recommendations.

ENDOCRINOLOGY IN THE TIME OF COVID-19: Clinical management of neuroendocrine neoplasms (NENs).

In viral pandemics, most specifically Covid-19, many patients with neuroendocrine neoplasms (NENs), including phaeochromocytomas, paragangliomas and medullary thyroid carcinoma, may develop Covid-19 in a mild or severe form, or be concerned about the influence of viral infection relative to their anti-tumoral therapy. In general, newly presenting patients should be assessed, and patients recently receiving chemotherapy, targeted therapy or radionuclide therapy, or showing tumour growth, should be closely followed. For previously diagnosed patients, who have indolent disease, some delay in routine follow-up or treatment may not be problematic.

Cancer Surveillance Guideline for individuals with PTEN hamartoma tumour syndrome.

PTEN hamartoma tumour syndrome is a diverse multi-system disorder predisposing to the development of hamartomatous growths, increasing risk of breast, thyroid, renal cancer, and possibly increasing risk of endometrial cancer, colorectal cancer and melanoma. There is no international consensus on cancer surveillance in PHTS and all current guidelines are based on expert opinion. A comprehensive literature review was undertaken and guidelines were developed by clinicians with expertise from clinical genetics, gynaecology, endocrinology, dermatology, radiology, gastroenterology and general surgery, together with affected individuals and their representatives.

Experiences of oldest-old caregivers whose partner is approaching end-of-life: A mixed-method systematic review and narrative synthesis.

Population ageing has rapidly increased the number of people requiring end-of-life care across the globe. Governments have responded by promoting end-of-life in the community. Partly as a consequence, older spouses are frequently providing for their partner's end-of-life care at home, despite potentially facing their own health issues.

Informed consent procedures in patients with an acute inability to provide informed consent: Policy and practice in the CENTER-TBI study.

Purpose: Enrolling traumatic brain injury (TBI) patients with an inability to provide informed consent in research is challenging. Alternatives to patient consent are not sufficiently embedded in European and national legislation, which allows procedural variation and bias. We aimed to quantify variations in informed consent policy and practice.

The impact of frailty in major trauma in older patients.

As our population ages and increasing numbers of older patients experience major trauma it is important to understand factors that influence outcomes in this patient cohort. The aim of this study is to assess the impact of frailty in older patients who experience major trauma (Injury Severity Score (ISS) greater than 15). A retrospective cohort review using the national trauma registry data (Trauma Audit and Research Network) and an institutional database was carried out on all patients aged 60 years or older with an ISS> 15 who were treated at the regional Major Trauma Centre from 2014 to 2017 following major trauma.

Genetic testing for hereditary hyperparathyroidism and familial hypocalciuric hypercalcaemia in a large UK cohort.

Background: Primary hyperparathyroidism (PHPTH) is a common endocrine disorder and an estimated 10% of cases are hereditary, related to syndromes including; multiple endocrine neoplasia (MEN) type 1, MEN type 4, MEN2A and hereditary hyperparathyroidism-jaw tumour syndrome. Establishing the underlying genetic cause for PHPTH allows for personalized and cost-effective management. Familial hypocalicuric hypercalcaemia (FHH) is a benign disorder of hypercalcaemia associated with an inappropriately low urinary calcium excretion, which is quantified by the calcium creatinine clearance ratio (CCCR).

Predictors of physical activity in older adults early in an emergency hospital admission: a prospective cohort study.

Background: Reduced mobility may be responsible for functional decline and acute sarcopenia in older hospitalised patients. The drivers of reduced in-hospital mobility are poorly understood, especially during the early phase of acute hospitalisation. We investigated predictors of in-hospital activity during a 24-h period in the first 48 h of hospital admission in older adults.

Editorial Commentary: Pain After Hip Arthroscopy-Are We Truly Addressing the Problem?

Pain after hip arthroscopy is a significant and challenging issue as is evidenced by the number of publications on this subject. Various analgesic strategies to circumvent this issue have been tried, with variable results. The central problem is that pain experienced by patients after hip arthroscopy is multifactorial in origin.

Cerebrospinal fluid dynamics in non-acute post-traumatic ventriculomegaly.

Background: Post-traumatic hydrocephalus (PTH) is potentially under-diagnosed and under-treated, generating the need for a more efficient diagnostic tool. We aim to report CSF dynamics of patients with post-traumatic ventriculomegaly.

Materials And Methods: We retrospectively analysed post-traumatic brain injury (TBI) patients with ventriculomegaly who had undergone a CSF infusion test.

Normosmic idiopathic hypogonadotropic hypogonadism due to a novel GNRH1 variant in two siblings.

Summary: Hypogonadotropic hypogonadism is characterised by insufficient secretion of pituitary gonadotropins resulting in delayed puberty, anovulation and azoospermia. When hypogonadotropic hypogonadism occurs in the absence of structural or functional lesions of the hypothalamic or pituitary gland, the hypogonadism is defined as idiopathic hypogonadotropic hypogonadism (IHH). This is a rare genetic disorder caused by a defect in the secretion of gonadotropin releasing hormone (GNRH) by the hypothalamus or a defect in the action of GNRH on the pituitary gland.

Eccentric Taylor spatial frame placement for the correction of femoral fracture deformity: a novel technique.

The treatment of post-traumatic femoral deformity with circular frames can be difficult both for the patient and for the surgeon. Patients frequently suffer from residual knee stiffness, and the cumbersome location of the frame can even result in psychiatric problems such as depression and anxiety during their treatment. Here we describe a novel technique of constructing the Taylor spatial frame (TSF) eccentrically from the femur to allow the same level of comfort as a monolateral external fixator, while applying trigonometric principles to successfully retain the utility of the TSF prescription software.

Shunt infusion studies: impact on patient outcome, including health economics.

Objectives: The diagnosis of shunt malfunction is often not straightforward. We have explored, in symptomatic shunted patients with hydrocephalus or pseudotumour cerebri syndrome (PTCS), the accuracy of CSF infusion tests in differentiating a functioning shunt from one with possible problems, and the health economic consequences.

Methods: Participants: hydrocephalus/PTCS patients with infusion tests performed from January 2013 until December 2015.

Development of a UK liver transplantation selection and allocation scheme.

Purpose Of Review: Equitable allocation of organs for liver transplantation remains a major area of ongoing study. In United Kingdom, it was agreed that the success of any national donor offering scheme would be judged from the point of registration on a national list for a transplant, and would therefore include outcome while waiting for the procedure.

Recent Findings: Standard minimal criteria for acceptance onto a transplant list have been developed for chronic liver disease, hepatocellular carcinoma and for a number of variant syndromes where current scores do not adequately reflect the risk of death without a transplant or symptom burden.

Robotic-assisted versus open pancreaticoduodenectomy for patients with benign and malignant periampullary disease: a systematic review and meta-analysis of short-term outcomes.

Background: Although several non-randomized studies comparing robotic pancreaticoduodenectomy (RPD) and open pancreaticoduodenectomy (OPD) recently demonstrated that the two operative techniques could be equivalent in terms of safety outcomes and short-term oncologic efficacy, no definitive answer has arrived yet to the question as to whether robotic assistance can contribute to reducing the high rate of postoperative morbidity.

Methods: Systematic literature search was performed using MEDLINE, the Cochrane Central Register of Controlled Trials, and EMBASE databases. Prospective and retrospective studies comparing RPD and OPD as surgical treatment for periampullary benign and malignant lesions were included in the systematic review and meta-analysis with no limits of language or year of publication.

Introducing PHIL (precipitating hydrophobic injectable liquid) - a new embolic agent for the body interventional radiologist.

A number of embolic agents are currently available each with their own properties. Precipitating hydrophobic injectable liquid (PHIL) is a new dimethyl sulfoxide (DMSO) compatible embolic agent with a number of specific properties which make it of interest to interventional radiologists. We review the use of PHIL in a non-neurointerventional setting, describing its use in a range of procedures such as trauma embolization, pseudoaneurysm embolization, and tumor embolization.

Advance care planning in patients referred to hospital for acute medical care: Results of a national day of care survey.

Background: Advance care planning (ACP) is a voluntary process of discussion about future care between an individual and their care provider. ACP is a key focus of national policy as a means to improve patient centered care at the end-of-life. Despite a wide held belief that ACP is beneficial, uptake is sporadic with considerable variation depending on age, ethnicity, location and disease group.

International consensus definition of low anterior resection syndrome.

Background: Low anterior resection syndrome (LARS) is pragmatically defined as disordered bowel function after rectal resection leading to a detriment in quality of life. This broad characterization does not allow for precise estimates of prevalence. The LARS score was designed as a simple tool for clinical evaluation of LARS.

International consensus definition of low anterior resection syndrome.

Aim: Low anterior resection syndrome (LARS) is pragmatically defined as disordered bowel function after rectal resection leading to a detriment in quality of life. This broad characterization does not allow for precise estimates of prevalence. The LARS score was designed as a simple tool for clinical evaluation of LARS.

Identification of Subtypes of Barrett's Esophagus and Esophageal Adenocarcinoma Based on DNA Methylation Profiles and Integration of Transcriptome and Genome Data.

Background & Aims: Esophageal adenocarcinomas (EACs) are heterogeneous and often preceded by Barrett's esophagus (BE). Many genomic changes have been associated with development of BE and EAC, but little is known about epigenetic alterations. We performed epigenetic analyses of BE and EAC tissues and combined these data with transcriptome and genomic data to identify mechanisms that control gene expression and genome integrity.