A Case of Primary Biliary Cholangitis With Late Diagnosis Leading to Failure To Thrive

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Journal of General Internal Medicine



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LEARNING OBJECTIVE #1: Assess the severity of illness and arrive at goals of care by balancing patient’s wishes and objective evidence-based evaluation. LEARNING OBJECTIVE #2: Recognize unspecific abnormal labs and symptoms and ensure continuity of care for early intervention. CASE: Patient was a 77 year old female with recently diagnosed nonalcoholic cirrhosis due to primary biliary cholangitis who presented with encephalopathy. Patient’s prior workup from two months prior showed positive IgG, and anti-mitochondrial antibody as well as cirrhosis on imaging. She was scheduled to be evaluated for transplant at an out-ofstate tertiary medical center. However, her functional status declined rapidly over the course of two weeks, leading to readmission. Alkaline phosphatase and total bilirubin were markedly elevated. Thrombocytopenia, hypoalbuminemia and coagulopathy were also noted. While ambulatory at baseline, patient became bedbound from debility and encephalopathy. Endoscopic ultrasound showed cirrhosis with mild ascites, and dilated CBD at 9mm without filling defects. Portal hypertension without esophageal varices was noted on endoscopy. Despite attempts to medically optimize patient for transferring to a tertiary medical center for further hepatology workup, patient remained debilitated with minimal improvement in her symptoms. On hospital day 10, she displayed signs of acute hypoxic respiratory failure with increasing oxygen requirement. She tested positive for COVID-19, although she tested negative on pre-admission screening with PCR assay. Patient was not a candidate for Remdesivir due to comorbidities and was treated with Dexamethasone. Given her poor prognosis, patient was categorized to DNR/DNI per family’s wishes. Patient continued to decline and passed away on hospital day 15. IMPACT/DISCUSSION: On further chart review, laboratory data from 2 years ago showed elevated alkaline phosphatase... (See full abstract in article).


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