We describe a case series of five infants (age range: 1-90 days; 4 females and 1 male) who presented to Al Jalila Children's intensive care units (ICU) with complex multisystem disorders. Patients were Emirati, Kenyan, Jordanian, Filipino, or Pakistani. Trio rapid whole genome sequencing (rWGS) was performed on all five patients and their parents within the hospital's genomics facility. Results were returned within ~37 h from blood sample draws and were diagnostic in 3 out of 5 patients. Positive findings were a homozygous pathogenic variant in POMT1 gene causing muscular dystrophydystroglycanopathy, a mosaic tetrasomy of the short arm of chromosome 12 (12p13.33p11.1) causing Pallister-Killian syndrome, and compound heterozygous pathogenic variants in the LIPA gene causing lysosomal acid lipase deficiency and Wolman disease. The rWGS analysis provided fast and precise diagnostic findings in those 3 patients and also aided in devising better management plans for them in the intensive care setting. For example, the 3-month-old infant with pathogenic variants in the LIPA gene is now a candidate for an FDA-approved, potentially lifesaving enzyme replacement therapy (sebelipase alfa). Our case series emphasize the feasibility and utility of rWGS in pediatric intensive care setting, in a diverse population that has long been underserved in genomic services. Significant investments in local healthcare infrastructure are needed, globally, for more equitable access of genomic medicine among vulnerable patients.
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http://dx.doi.org/10.1186/s13073-022-01061-7 | DOI Listing |
J Cardiovasc Surg (Torino)
February 2025
Department of Vascular Surgery, ASST Settelaghi Universitary Teaching Hospital, University of Insubria, Varese, Italy.
Optimizing the longevity of vascular access in hemodialysis patients remains a critical aspect of patient care, given the significant role of arteriovenous fistulas (AVFs) and arteriovenous grafts (AVGs) in enabling effective dialysis. Vascular access complications, such as stenosis, thrombosis, and cannulation-related damage, continue to challenge both the functionality and the sustainability of these access points. Recent advancements underscore the importance of a robust follow-up strategy, integrating clinical evaluations with diagnostic tools like color Doppler ultrasound (CDU) and emerging interventional approaches such as drug-coated balloon (DCB) angioplasty.
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March 2025
Department of Medicine (Pulmonary & Critical Care), Medical College of Wisconsin, Milwaukee, Wisconsin, USA.
Physiol Rep
February 2025
Berlin Institute of Health at Charité, Universitätsmedizin Berlin, Center of Functional Genomics, Berlin, Germany.
The zona glomerulosa (ZG) synthesizes the mineralocorticoid aldosterone. The primary role of aldosterone is the maintenance of volume and electrolyte homeostasis. Aldosterone synthesis is primarily regulated via tightly controlled oscillations in intracellular calcium levels in response to stimulation.
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January 2025
UT Health San Antonio, San Antonio, TX, USA.
Background: Provision of palliative care in acute care settings is significantly lacking despite evidence that early integration leads to better patient/family-related outcomes and improved healthcare cost and efficiency.
Objective: This study investigated influencing factors that affect the nature and frequency of palliative care practices of acute care nurses.
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Brain Behav Immun Health
February 2025
Lovisenberg Diaconal University College, Oslo, Norway.
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