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Pulmonary arterial hypertension (PAH) is a progressive disease characterized by proliferative remodeling and obliterative narrowing of the pulmonary vasculature. While outcomes have improved with existing treatments targeting 3 main pathways, there remains a critical need for novel therapies that address different and novel mechanisms of PAH. Sotatercept, recently Food and Drug Administration (FDA) approved, is a groundbreaking fusion protein that binds to activin and growth differentiation factors, rebalancing antiproliferative and pro-proliferative signals to reverse remodeling in both the pulmonary vasculature and the right ventricle.

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Hydrocephalus commonly occurs after subarachnoid hemorrhage (SAH) and is associated with increased morbidity and disability in patients with SAH. Choroid plexus cerebrospinal fluid (CSF) hypersecretion, obliterative arachnoiditis occluding the arachnoid villi, lymphatic obstruction, subarachnoid fibrosis, and glymphatic system injury are considered the main pathological mechanisms of hydrocephalus after SAH. Although the mechanisms of hydrocephalus after SAH are increasingly being revealed, the clinical prognosis of SAH still has not improved significantly.

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Article Synopsis
  • - Biliary atresia (BA) is a severe liver condition in infants that causes inflammation and blockage of bile ducts, leading to cirrhosis within two years if untreated.
  • - Early diagnosis is crucial, and the best initial treatment is the Kasai procedure, which should be performed within the first two months of life to maximize the chances of success.
  • - The outcomes of the Kasai procedure vary, with potential long-term complications requiring liver transplant; pathologists need to differentiate between various liver damage patterns in successful versus failed surgeries for accurate diagnosis.
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Tubulointerstitial nephritis with IgM-positive plasma cells complicated by liver failure.

CEN Case Rep

October 2024

Department of Rheumatology, Endocrinology, and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Kita 14, Nishi 5, Kita-ku, Sapporo, 060-8648, Japan.

Tubulointerstitial nephritis (TIN) is characterized by inflammation of the renal interstitium with the infiltration of immune cells, mainly consisting of T cells. Recently, patients with TIN with the predominant infiltration of immunoglobulin M (IgM)-positive plasma cells were reported, coined IgMPC-TIN. Here we report the case of a 70-year-old woman diagnosed with Fanconi syndrome and renal tubular acidosis.

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Article Synopsis
  • TREM-1 is a protein important for the immune system, and scientists are studying how it affects a disease called obliterative bronchiolitis (OB).
  • In a mouse study, they found that blocking TREM-1 helps reduce the problems caused by OB, while boosting it makes things worse.
  • The research suggests that targeting TREM-1 could be a useful way to treat OB by changing how immune cells work.
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