Radiation Belt Storm Probes Ion Composition Experiment (RBSPICE) Revisited: In-Flight Calibrations, Lessons Learned and Scientific Advances.

The Radiation Belt Storm Probes Ion Composition Experiment (RBSPICE) on both the Van Allen Probes spacecraft is a time-of-flight versus total energy instrument that provided ion composition data over the ring current energy (∼7 keV to ∼1 MeV), and electrons over the energy range ∼25 keV to ∼1 MeV throughout the duration of the mission (2012 - 2019). In this paper we present instrument calibrations, implemented after the Van Allen Probes mission was launched. In particular, we discuss updated rate dependent corrections, possible contamination by "accidentals" rates, and caveats concerning the use of certain products.

Chylous Ascites Developing Into Bilateral Chylothorax in High-Grade Lymphoma: A Case Report.

Chylothorax and chylous ascites occur when lymphatic fluid accumulates in the pleural space or peritoneum, respectively. They are classified as either traumatic or non-traumatic, and lymphomas are the most common non-traumatic cause. Lymphomas can obstruct the lymphatic architecture causing lipid-rich chyle to leak out below the level of the obstructing mass.

Double faecal immunochemical testing in patients with symptoms suspicious of colorectal cancer.

Background: Faecal immunochemical test (FIT)-directed pathways based on a single test have been implemented for symptomatic patients. However, with a single test, the sensitivity is 87 per cent at 10 µg haemoglobin (Hb) per g faeces. This aims of this study were to define the diagnostic performance of a single FIT, compared with double FIT in symptomatic populations.

Homocystinuria diagnosis and management: it is not all classical.

Homocystinuria (HCU) refers to a group of inherited disorders of homocysteine metabolism associated with high blood homocysteine concentration, thromboembolic tendency and neurocognitive symptoms. The most common causes of a high blood homocysteine relate to underlying vitamin B or folate deficiency which must be excluded first. Thereafter, an inherited metabolic condition can be considered.

A case series of cerebral venous thrombosis as the first manifestation of homocystinuria.

Background: Cerebral venous thrombosis (CVT) is an important cause of stroke particularly in younger patients and potentially fatal if diagnosis is delayed. The presentation of symptoms is highly variable and consequently the diagnosis and underlying cause is often delayed or overlooked. Homocystinuria, a rare autosomal recessive disorder is an identified risk factor for CVT.

Patient partners' perspectives of meaningful engagement in synthesis reviews: A patient-oriented rapid review.

Background: A growing literature describes promising practices for patient-oriented research (POR) generally; however, those for systematic reviews are largely derived through the lens of a researcher. This rapid review sought to understand meaningful engagement in synthesis reviews from the patient partner (PP) perspective.

Design: The review team comprised PPs, librarians, SCPOR staff and academic faculty.

Mirror Instabilities in the Inner Magnetosphere and Their Potential for Localized ULF Wave Generation.

Results from the NASA Van Allen Probes mission indicate extensive observations of mirror/drift-mirror (M/D-M hereafter) unstable plasma regions in the night-side inner magnetosphere. Said plasmas lie on the threshold between the kinetic and frozen-in plasma regimes and have favorable conditions for the formation of M/D-M modes and subsequent ultralow frequency (ULF) wave signatures in the surrounding plasma. We present the results of a climatological analysis of plasma- (anisotropy measure) and total plasma- (ratio of particle to magnetic field pressure) in regard to the satisfaction of instability conditions on said M/D-M modes under bi-Maxwellian distribution assumption, and ascertain the most likely region for such plasmas to occur.

Metformin as a radiosensitiser for pelvic malignancy: A systematic review of the literature.

Background: The treatment of pelvic malignancies has continued to improve over recent years, with neoadjuvant radiotherapy often considered the gold standard to downstage disease. Radiosensitisers are routinely employed in an attempt to improve response of cancers to radiotherapy. Previous preclinical evidence has suggested a role for metformin, a commonly used drug for type 2 diabetes.

Inter-laboratory analytical improvement of succinylacetone and nitisinone quantification from dried blood spot samples.

Background: Nitisinone is used to treat hereditary tyrosinemia type 1 (HT-1) by preventing accumulation of toxic metabolites, including succinylacetone (SA). Accurate quantification of SA during newborn screening is essential, as is quantification of both SA and nitisinone for disease monitoring and optimization of treatment. Analysis of dried blood spots (DBS) rather than plasma samples is a convenient method, but interlaboratory differences and comparability of DBS to serum/plasma may be issues to consider.

Limits to sustained energy intake. XXX. Constraint or restraint? Manipulations of food supply show peak food intake in lactation is constrained.

Lactating mice increase food intake 4- to 5-fold, reaching an asymptote in late lactation. A key question is whether this asymptote reflects a physiological constraint, or a maternal investment strategy (a 'restraint'). We exposed lactating mice to periods of food restriction, hypothesizing that if the limit reflected restraint, they would compensate by breaching the asymptote when refeeding.

[3-hydroxy-3-methylglutaryl-CoA lyase deficiency: a case report and literature review].

Introduction: 3-hydroxy-3-methylglutaryl-CoA (HMG-CoA) lyase deficiency is an autosomal recessive disorder that usually presents in the neonatal period with vomiting, metabolic acidosis, hypoglycemia and absent ketonuria. Few cases are reported in the literature, and optimal dietary management and long term outcome are not fully understood.

Case Report: We report a 2 year old girl with HMG-CoA-lyase deficiency who had limited fasting tolerance on a low protein diet, with several recurrent hospital admissions with severe hypoketotic hypoglycaemia and metabolic acidosis.

The safety of Lipistart, a medium-chain triglyceride based formula, in the dietary treatment of long-chain fatty acid disorders: a phase I study.

Background: Children with long-chain fatty acid β-oxidation disorders (LCFAOD) presenting with clinical symptoms are treated with a specialist infant formula, with medium chain triglyceride (MCT) mainly replacing long chain triglyceride (LCT). It is essential that the safety and efficacy of any new specialist formula designed for LCFAOD be tested in infants and children.

Methods: In an open-label, 21-day, phase I trial, we studied the safety of a new MCT-based formula (feed 1) in six well-controlled children (three male), aged 7-13 years (median 9 years) with LCFAOD (very long chain acyl CoA dehydrogenase deficiency [VLCADD], n=2; long chain 3-hydroxyacyl CoA dehydrogenase deficiency [LCHADD], n=2; carnitine acyl carnitine translocase deficiency [CACTD], n=2).