Hepatopulmonary syndrome from liver disease associated with autosomal recessive polycystic kidney disease.

Hepatopulmonary syndrome (HPS) is a life-threatening complication of chronic liver disease (CLD) that currently can be managed only by liver transplant. Though uncommon, some children with kidney disease have coexistent CLD and hence are at risk of developing HPS. Paediatric cases of HPS are rarely described in the nephrology literature.

Feasibility of home blood pressure screening in the paediatric outpatient clinic setting.

Objective: The aim of this study was to evaluate the acceptability and feasibility of home-based blood pressure (BP) screening in a group of paediatric patients with known elevated risk of developing hypertension.

Design: Cross-sectional study.

Setting: Specialist outpatient clinic and patient homes.

Validation of the Uscom BP+ automated oscillometric blood pressure monitor for professional office use in children and adolescents according to the AAMI/ESH/ISO Universal Standard (ISO 81060-2:2018).

To (1) assess the accuracy of the Uscom BP+ oscillometric upper-arm professional blood pressure (BP) monitor in children and adolescents as per the AAMI/ESH/ISO Universal Standard (ISO 81060-2:2018); (2) assess the suitability of a non-standard cuff; and (3) assess the impact of motion artefact on device accuracy and precision. Children and adolescents were recruited to fulfil the sex/cuff distribution criteria of the Universal Standard. Single arm sequential BP measurements were used, the test device measured on deflate with an altered child/adolescent algorithm, using three standard (Extra Small, Small Adult, Adult) and, as exploratory analysis, additionally in one non-standard cuff (Wide Range; WR) (arm circumferences: 12-17, 16-24, 22-32, 22-42 cm, respectively).

Discovery of Highly Selective Inhibitors of Microtubule-Associated Serine/Threonine Kinase-like (MASTL).

By virtue of its role in cellular proliferation, microtubule-associated serine/threonine kinase-like (MASTL) represents a novel target and a first-in-class (FIC) opportunity to provide a new impactful therapeutic agent to oncology patients. Herein, we describe a hit-to-lead optimization effort that resulted in the delivery of two highly selective MASTL inhibitors. Key strategies leveraged to enable this work included structure-based drug design (SBDD), analysis of lipophilic efficiency (LipE) and novel synthesis.