Publications by authors named "Raj A Shah"

Vanishing bile duct syndrome (VBDS) refers to a form of cholestatic liver disease with many etiologies. Vanishing bile duct syndrome is characterized by biliary ductopenia and chronic cholestasis. This is a challenging condition for clinicians because of its rarity and unclear pathophysiology.

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Hepcidin, a peptide hormone produced by hepatocytes, is the central regulator of systemic iron homeostasis through its interaction with ferroportin, the major cellular iron export protein. Hepcidin binding to ferroportin results in reduced iron export from macrophages and intestinal absorptive cells, leading to decreased serum iron levels. Hepcidin expression is influenced by several factors that include serum and liver iron stores, erythropoiesis, hypoxia, inflammation, and infection.

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Introduction: The discovery of the farnesoid X receptor and its role in bile and lipid homeostasis has led to the investigation of FXR agonists as a therapy in cholestatic disorders and in NAFLD. With increasing prevalence of obesity and metabolic syndrome, NAFLD has grown to become the most prevalent chronic liver disease, present in a quarter of the worldwide population. FXR agonists represent a promising avenue for this burgeoning disease with few options for pharmacologic therapy.

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Introduction: NAFLD has grown to become the most prevalent liver disease in the world, with a quarter of the general population estimated to have the disease. NASH, characterized as NAFLD with inflammation, is associated with worsening fibrosis along with increased incidence of HCC. Despite high prevalence of this disease, no pharmacologic treatments approved by regulatory agencies are available.

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Up to 40% of patients with primary biliary cholangitis have an incomplete response to first-line treatment with ursodeoxycholic acid. Obeticholic acid was approved by the US Food and Drug Administration in 2016 as a second-line treatment for patients with primary biliary cholangitis who are unresponsive to ursodeoxycholic acid; however, approximately 50% of patients might need additional treatments to reach therapeutic goals. A considerable need exists for effective treatment options to prevent progression to liver transplantation or death in these patients.

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Pruritus is a frustrating and sometimes debilitating symptom that commonly accompanies primary biliary cholangitis (PBC). The mechanism by which this symptom manifests remains elusive but extensive research has now shown that the itch is not just "weak pain" as had been the commonly held belief for decades. As this research now shines a light on the many diverse paths by which pruritus might be experienced, the necessity for a comprehensive approach to the symptom becomes clear.

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Background: Routine lung cancer surveillance has resulted in early detection of pulmonary nodules and masses. Combined endobronchial ultrasound (EBUS) and trans-esophageal endoscopic ultrasound (EUS) are approved methods for sampling lymph nodes or masses. Furthermore, EUS allows for adrenal sampling as part of staging, and can assist with fiducial placement for stereotactic body radiation therapy (SBRT).

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Coccidioidomycosis causes significant morbidity in endemic areas. In the absence of sensitive diagnostic serologic testing, clinicians have increasingly relied on lung and lymph node biopsies for diagnosis. Recently, endobronchial ultrasound guided transbronchial needle aspiration (EBUS-TBNA) has been shown to be an excellent sampling method for the diagnosis and staging of lung cancers, especially when combined with endoscopic ultrasound guided fine needle aspiration (EUS-FNA).

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Article Synopsis
  • Researchers have identified a mutation in the CACNA1C gene linked to Timothy Syndrome, a rare condition causing heart issues and physical abnormalities.
  • The study reveals that the calcium channel CaV1.2 is crucial for jaw development, as it is expressed in early structures that form the jaw.
  • Findings indicate that alterations in calcium signals through CaV1.2 lead to changes in jaw growth, providing new insights into the role of calcium channels in the development of nonexcitable cells.
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