The use of long-arm (indirect) supervision for nursing, midwifery and allied health professionals in health and social care settings: A systematic literature review.

Background: Internationally, critical shortages of healthcare workers have been reported. Indirect supervision is one educational approach which has the potential to expand placement opportunities for the future healthcare workforce. However, its use across healthcare professions is yet to be systematically reviewed.

Measuring symptom burden in patients with cancer during a pandemic: the MD Anderson symptom inventory for COVID-19 (MDASI-COVID).

Background: Symptom expression in SARS-CoV-2 infection (COVID-19) may affect patients already symptomatic with cancer. Patient-reported outcomes (PROs) can describe symptom burden during the acute and postacute stages of COVID-19 and support risk stratification for levels of care. At the start of the COVID-19 pandemic, our purpose was to rapidly develop, launch through an electronic patient portal, and provide initial validation for a PRO measure of COVID-19 symptom burden in patients with cancer.

The Unique Symptom Burden of Patients Receiving CAR T-Cell Therapy.

Objectives: There is little research on the patient experience of symptom burden from CAR T-cell therapy, and no validated measure specific to the symptoms of CAR T-cell therapy currently exists. The purpose of this study was to identify symptoms experienced and to determine the content domain for a patient-reported outcome (PRO) measuring symptom burden for patients who had received standard of care CAR T-cell therapy for advanced B-cell lymphoid malignancies.

Data Sources: Semi-structured qualitative interviews were conducted with a sample of 21 patients who had received CAR T-cell therapy.

Healing of vitamin D deficiency rickets complicating hypophosphatasia suggests a role beyond circulating mineral sufficiency for vitamin D in musculoskeletal health.

Hypophosphatasia (HPP) is the metabolic bone disease caused by loss-of-function mutation(s) of the ALPL gene that encodes the cell-surface tissue-nonspecific isoenzyme of alkaline phosphatase (TNSALP). In HPP, extracellular accumulation of inorganic pyrophosphate (PPi), a TNSALP natural substrate and inhibitor of biomineralization, often leads to rickets or osteomalacia despite normal or sometimes elevated circulating levels of calcium (Ca) and inorganic phosphate (Pi). We report an infant girl with vitamin D deficiency rickets subsequently healed by cholecalciferol administration alone before receiving TNSALP-replacement therapy for accompanying HPP.

Development and validation of a modified performance-oriented mobility assessment tool for assessing mobility in children with hypophosphatasia.

Purpose: To modify the Performance-Oriented Mobility Assessment-Gait (POMA-G) subtest and validate this modified POMA-G (mPOMA-G) in children with hypophosphatasia (HPP), a rare metabolic disorder that can manifest with musculoskeletal symptoms that impair mobility and ambulation.

Methods: Based on feedback from an expert panel, the POMA-G was modified by removing gait initiation/path assessments and expanding the rating scale for step length/continuity to capture aspects of observational gait analysis relevant to children with HPP. Three trained physical therapists used the mPOMA-G for video-based assessments of gait in 14 children with childhood HPP who participated in a clinical study of asfotase alfa or in a natural history study.

Physical therapy management of infants and children with hypophosphatasia.

Hypophosphatasia (HPP) is a rare inborn error of metabolism resulting in undermineralization of bone and subsequent skeletal abnormalities. The natural history of HPP is characterized by rickets and osteomalacia, increased propensity for bone fracture, early loss of teeth in childhood, and muscle weakness. There is a wide heterogeneity in disease presentation, and the functional impact of the disease can vary from perinatal death to gait abnormalities.

Hypophosphatasia: validation and expansion of the clinical nosology for children from 25 years experience with 173 pediatric patients.

Hypophosphatasia (HPP) is caused by loss-of-function mutation(s) within the gene TNSALP that encodes the "tissue-nonspecific" isoenzyme of alkaline phosphatase (TNSALP). In HPP, inorganic pyrophosphate, an inhibitor of mineralization and substrate for TNSALP, accumulates extracellularly often leading to rickets or osteomalacia and tooth loss, and sometimes to craniosynostosis and calcium crystal arthropathies. HPP's remarkably broad-ranging expressivity spans stillbirth from profound skeletal hypomineralization to adult-onset dental problems or arthropathies without bone disease, which is largely explained by autosomal recessive versus autosomal dominant transmission from among several hundred, usually missense, TNSALP mutations.

Characterization of the SCP/TAPS gene family in Drosophila melanogaster.

The SCP/Tpx-1/Ag5/PR-1/Sc7 (SCP/TAPS) gene family encodes proteins found in many eukaryotes. SCP/TAPS proteins are defined by the presence of an SCP/TAPS domain, and many participate in important physiological processes. Five SCP/TAPS genes were previously identified in Drosophila melanogaster and are expressed in the digestive tract or in the testes.