A left ventricular assist system (LVAS, Toyobo CO., LTD., Japan) was used to provide life support for 190 days in a 44-year-old male patient with end-stage dilated cardiomyopathy. Before LVAS implantation, heart failure and cardiogenic shock with multi-organ dysfunction progressed despite the use of intra-aortic balloon pumping (18 days), mechanical ventilatory support (15 days), continuous veno-venous hemofiltration (two days) and intravenous administration of catecholamines. Before LVAS implantation, the patient had marked hepatic and renal dysfunction (total bilirubin 6.1, BUN 73 and serum creatinine 3.1 mg/dl). Those functions returned to normal (total bilirubin 1.2, BUN 11 and serum creatinine 0.6 mg/dl) one month after implantation. He was complicated by multiple cerebral embolisms occurring on the 9th, 57th and 175th postoperative days and died 190 days after surgery. Autopsy showed thrombi attaching to the diaphragm of the blood pump and multiple embolisms in the kidney and the spleen. By scanning electron microscopic examination, initial thrombi with attached erythrocytes were seen on the diaphragm surface without intimal lining formation. In conclusion, LVAS support can be useful for patients with end-stage heart disease complicated by multi-organ dysfunction. However, the LVAS may cause thromboembolic complications over the prolonged use.
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Pediatr Nephrol
January 2025
Pediatric Nephrology Services, Department of Pediatrics, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry, 605006, India.
Background: Information on the clinical characteristics and outcomes of children undergoing continuous kidney replacement therapy (CKRT) from lower-middle-income countries (LMIC) is limited.
Methods: Records of consecutive children 1 month to 18 years of age who underwent CKRT from Jan 2016 to Jan 2024 in a tertiary care pediatric intensive care unit (PICU) were retrospectively reviewed and analyzed for clinical and machine-related characteristics, and outcomes.
Results: Over the 8-year period, 102 patients (61.
Transplantation
January 2025
University of Zurich, Wyss Translational Center, Zurich, Switzerland.
Background: Early allograft dysfunction (EAD) affects outcomes in liver transplantation (LT). Existing risk models developed for deceased-donor LT depend on posttransplant factors and fall short in living-donor LT (LDLT), where pretransplant evaluations are crucial for preventing EAD and justifying the donor's risks.
Methods: This retrospective study analyzed data from 2944 adult patients who underwent LDLT at 17 centers between 2016 and 2020.
Mol Genet Metab
January 2025
Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX, USA. Electronic address:
Cerebrotendinous Xanthomatosis (CTX) is a treatable, inborn error of bile acids metabolism caused by pathogenic variants in CYP27A1. CTX is a multi-organ system disorder that progresses over decades. Clinical features include cerebellar dysfunction, pyramidal tract dysfunction, cognitive deficits and decline, peripheral neuropathy, chronic diarrhea, bilateral cataracts, and tendon xanthomas.
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January 2025
Department of Nephrology, State Key Laboratory of Kidney Diseases, Beijing Key Laboratory of Kidney Disease Research, National Clinical Research Center for Kidney Diseases, Nephrology Institute of the Chinese People's Liberation Army, Chinese PLA General Hospital, Beijing, China.
Spaceflight-induced multi-organ dysfunction affects the health of astronauts and the safety of in-orbit flight. However, the effect of microgravity on the kidney and the underlying mechanisms are unknown. In the current study, we used a hindlimb unweighting (HU) animal model to simulate microgravity and employed histological analysis, ischemia-reperfusion experiments, renal ultrasonography, bioinformatics analysis, isometric force measurement, and other molecular experimental settings to evaluate the effects of microgravity on the kidneys and the underlying mechanisms involved in this transition.
View Article and Find Full Text PDFFront Pharmacol
January 2025
Respiratory Department II, National Clinical Research Center for Respiratory Diseases, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.
Multisystemic smooth muscle dysfunction syndrome (MSMDS) is an autosomal dominant disorder caused by mutations in the gene, resulting in variable clinical manifestation and multi-organ dysfunction. Interstitial lung disease (ILD) is a rare phenotype of this condition. We describe a rare infant case of an 8-month-old boy who presented with progressively worsening dyspnea, along with intermittent episodes of respiratory distress and cyanosis since birth.
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