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Update on Hepatorenal Syndrome: From Pathophysiology to Treatment.

Annu Rev Med

January 2025

Division of Gastrointestinal and Liver Diseases, Department of Medicine, Keck School of Medicine, University of Southern California, Los Angeles, California, USA; email:

Hepatorenal syndrome-acute kidney injury (HRS-AKI) occurs in the setting of advanced chronic liver disease, portal hypertension, and ascites. HRS-AKI is found in ∼20% of patients presenting to the hospital with AKI, but it may coexist with other causes of AKI and/or with preexisting chronic kidney disease, thereby making the diagnosis challenging. Novel biomarkers such as urinary neutrophil gelatinase-associated lipocalin may be useful.

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Extra-adrenal pheochromocytomas are rare neuroendocrine tumors originating outside the adrenal glands and can pose significant diagnostic challenges due to their variable presentations. This report highlights a case of an extra-adrenal pheochromocytoma masquerading as a pancreatic head malignancy. We underscore the importance of considering extra-adrenal pheochromocytoma in the differential diagnosis of pancreatic masses, particularly when biochemical or clinical features suggest catecholamine excess.

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Quantifying urinary catecholamines and metanephrines is essential for the clinical screening and diagnosis of neuroendocrine tumours. HPLC with electrochemical detection (HPLC-ECD) is commonly used for this type of analysis but requires extensive sample cleanup. Simple and rapid dilute-and-shoot LC-multiple-reaction monitoring (MRM)-MS assays have been developed for quantitating these analytes in urine but have not yet been validated according to the Clinical and Laboratory Standards Institute (CLSI) guidelines.

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Pheochromocytoma is a catecholamine-secreting tumor that arises from the medullary chromaffin cells but can rarely be extra-adrenal in origin. We present a case of a 16-year-old female patient with uncontrolled hypertension, despite being on lisinopril and metoprolol, and associated left-sided chest pain, recurrent headaches, and an unintentional weight loss of 10 pounds in one month. Laboratory work-up showed a markedly elevated plasma metanephrine level of 4463.

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Pheochromocytoma is a rare tumor originating from the adrenal gland, characterized by the secretion of catecholamines. Due to the risk of hypertensive crises associated with catecholamine release, surgical procedures in pheochromocytoma patients are risky. In this case report, laparoscopic appendectomy for acute appendicitis in a patient who has pheochromocytoma will be presented.

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