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.