A multi-centre UK-based survey on angioedema secondary to acquired C1 inhibitor deficiency.

Background: Acquired angioedema due to C1-inhibitor deficiency (AAE-C1-INH) is very rare compared to its prototype, hereditary angioedema. An updated characterisation of the AAE-C1-INH cohort in UK is required to inform management.

Objectives: To describe the disease burden of AAE-C1-INH, long-term prophylaxis (LTP) and the clinical, immunochemical and treatment profiles of AAE-associated diseases in UK.

Outcomes of X-Linked Agammaglobulinaemia Patients.

Background: X-linked agammaglobulinaemia (XLA), caused by mutations in BTK, is characterised by low or absent peripheral CD19 + B lymphocytes and agammaglobulinaemia. The mainstay of treatment consists of immunoglobulin replacement therapy (IgRT). As this cannot fully compensate for the immune defects in XLA, patients may therefore continue to be at risk of complications.

Efficacy and safety of leadless pacemaker: A systematic review, pooled analysis and meta-analysis.

Background: Leadless pacemakers have been designed as an alternative to transvenous systems which avoid some of the complications associated with transvenous devices. We aim to perform a systematic review of the literature to report the safety and efficacy findings of leadless pacemakers.

Methods: We searched MEDLINE and EMBASE to identify studies reporting the safety, efficacy and outcomes of patients implanted with a leadless pacemaker.

Predicting successful sperm retrieval in transfeminine adolescents after testicular biopsy.

Purpose: Increasing numbers of transgender adolescents are receiving gender-affirming treatments (GAT). Given GAT can impair reproductive function, clinical guidelines advise prior counselling regarding fertility preservation (FP). For transgender adults assigned male at birth, FP is usually achieved via a masturbatory sample and sperm cryopreservation.

Assessment of diastolic blood pressure with the auscultatory method in children and adolescents under exercise conditions.

Controversy surrounds whether to define resting diastolic blood pressure (DBP) as the onset of the fourth or fifth Korotkoff phase (K4, sound muffling, or K5, sound disappearance) in children and adolescents. Although undetectable in some children (due to sounds continuing to zero cuff pressure), K5 is currently recommended for consistency with adult practice and because K4 can be difficult to discern or undetectable. However, to our knowledge, no studies have specifically assessed the reliability of measuring DBP with K4 and K5 in children and adolescents under exercise conditions.

Acquired C1-inhibitor deficiency presenting with nephrotic syndrome.

Acquired C1-inhibitor (C1-INH) deficiency is a rare and potentially life-threatening disorder, which presents with recurrent attacks of non-pitting oedema to the face, airway, limbs or gastrointestinal tract. It is often associated with underlying B-cell lymphoproliferative disorders. We describe a case of a 73-year-old man with acquired C1-INH deficiency who presented with nephrotic syndrome due to glomerular IgM deposition, secondary to an underlying secretory lymphoplasmacytic lymphoma.

The United Kingdom Primary Immune Deficiency (UKPID) registry 2012 to 2017.

This is the second report of the United Kingdom Primary Immunodeficiency (UKPID) registry. The registry will be a decade old in 2018 and, as of August 2017, had recruited 4758 patients encompassing 97% of immunology centres within the United Kingdom. This represents a doubling of recruitment into the registry since we reported on 2229 patients included in our first report of 2013.

A short report on current fertility preservation strategies for boys.

Background: Advances in cancer treatment have led to improved long-term survival after childhood cancer, but often at a price of impaired future fertility. Fertility preservation (FP) in male children and early adolescents poses unique challenges as efficacy is unproven.

Objectives: To describe characteristics of testicular tissue cryopreservation (TTCP) specimens taken from paediatric and adolescent patients, stratified by age, and prior chemotherapy, if any, and to demonstrate evidence for germ cells.

Lost Dollars Threaten Research in Public Academic Health Centers.

The decrease of federal and state support threatens long-term sustainability of research in publicly supported academic health centers. In weathering these financial threats, research at the University of California, San Francisco (UCSF), has undergone 3 substantial changes: institutional salary support goes preferentially to senior faculty, whereas the young increasingly depend on grants; private and government support for research grows apace in clinical departments but declines in basic science departments; and research is judged more on its quantity (numbers of investigators and federal and private dollars) than on its goals, achievements, or scientific quality. We propose specific measures to alleviate these problems.

Closed vitrification of human oocytes and blastocysts: outcomes from a series of clinical cases.

Purpose: High survival rates and clinical outcomes similar to those from fresh oocytes and blastocysts have been observed with open oocyte vitrification systems. It has been suggested that the extremely fast cooling rates that are only achieved with open systems are necessary for human oocyte and blastocyst vitrification. However, there is a potential risk of introducing contamination with open systems.

Respiratory Health and Related Quality of Life in Patients with Congenital Agammaglobulinemia in the Northern Region of the UK.

Introduction: Patients with congenital agammaglobulinemia, characterized by a defect in B lymphocyte differentiation causing B alymphocytosis, require life-long IgG replacement. There is scant literature regarding the effectiveness of IgG treatment at preventing mucosal (particularly sinopulmonary tract) infection and whether current management adequately restores "normal" health and quality of life (QoL). We aimed to document infective episodes pre- and post-commencing IgG replacement, determine any change in lung function and structure and assess respiratory status and QoL in a cohort of patients treated in Newcastle.

A fair deal for PhD students and postdocs.

The relentless expansion that threatens the sustainability of biomedical research in the US takes a heavy toll on young researchers.

A recipe for mediocrity and disaster, in five axioms.

Biomedical research in the US will become unsustainable unless scientists and research institutions start to question certain assumptions they have long taken for granted.

The writing on the wall.

The biomedical research enterprise in the US has become unsustainable and urgent action is needed to address a variety of problems, including a lack of innovation, an over-reliance on soft money for faculty salaries, the use of graduate students as a source of cheap labour, and a 'holding tank' full of talented postdocs with limited career opportunities.

Outcomes from the first assisted reproduction program for HIV-serodiscordant couples in Australia.

Objective: To describe the clinical outcomes for all HIV-serodiscordant couples attending an assisted reproduction program.

Design, Setting And Participants: Retrospective review of demographic, clinical and outcome data for all HIV-serodiscordant couples who attended an assisted reproduction program at a tertiary hospital in Melbourne, between its commencement in 2003 and June 2010.

Main Outcome Measures: Pregnancies, miscarriages, births, HIV transmission to the HIV-negative partner, semen quality and detection of HIV (HIV RNA and HIV DNA) in semen.

Anglicising psychoanalysis innocently.

Finding Freud not properly on his feet, Rycroft turned him upright. Not by producing yet another school of psychoanalysis, but by pseudo-innocently amending its German polysyllabic script radically into English, and so revealing too how humanity is distinctly and to the very depths a symbolising animal. The present study describes how he contrived both to achieve all this, along with eminence in English society, and perversely to remain unconsciously and steadfastly little known or audible in the world of psychoanalysis.

Comparison of outcomes of conventional intracytoplasmic sperm injection and intracytoplasmic sperm injection using sperm bound to the zona pellucida of immature oocytes.

This study was to compare the outcomes of conventional (scientist-selected sperm) intracytoplasmic sperm injection (ICSI) and a modified ICSI using zona pellucida (ZP)-bound sperm. Although with 39 couples in each group there was no statistical significance in fertilization, embryo development, implantation, and fetal heart clinical pregnancy rates, the higher implantation and clinical pregnancy rates with ZP-bound sperm encourages further evaluation with larger numbers of subjects using sibling oocytes to determine if the ZP-bound sperm are biologically and functionally superior.

Factors affecting low birthweight after assisted reproduction technology: difference between transfer of fresh and cryopreserved embryos suggests an adverse effect of oocyte collection.

Background: Data show that differences exist in the birthweight of singletons after frozen embryo transfer (FET) compared with fresh transfer or gamete intra-Fallopian transfer (GIFT). Factors associated with low birthweight (LBW) after assisted reproduction technology (ART) were studied.

Methods: Birthweight, distribution of birthweight, z-score, LBW (<2500 g), gestation and percentage preterm (<37 weeks) for singleton births >19 weeks gestation, conceived by ART or non-ART treatments (ovulation induction and artificial insemination) between 1978 and 2005 were analysed for one large Australian clinic.