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INTRODUCTION: Transjugular intrahepatic portosystemic shunt (TIPS) placement has now been well established as an effective and considered relatively safe treatment for the management of sequelae of portal hypertension. Hepatic infarction is an uncommon, potentially fatal complication after a TIPS procedure but rarely reported in cirrhosis patients with a low MELD (Model for End Stage Liver Disease). Here, we present a case of large hepatic infarct developing within a day of successful placement of TIPS in a patient with low MELD, which resolved with supportive treatment. CASE DESCRIPTION/METHODS: We present a 55 y/o Caucasian lady with Laennec cirrhosis with MELD 15 and normal bilirubin, who underwent elective TIPS placement for refractory ascites at our tertiary care center. Patient tolerated the procedure well with normal monitored vitals post procedure. However, she started complaining of right upper quadrant abdominal pain on next day with elevated transaminases and INR (40- fold and 2- fold increase from her baseline, respectively) with a stable bilirubin, CBC and creatinine. CT triple phase of the liver revealed a large hypodensity involving segments 6, 7 of the liver, suggestive of massive hepatic infarction and a patent TIPS. Patient not being a suitable candidate for liver transplant (with a history of recently diagnosed bladder carcinoma), was managed with supportive measures- Fluid, albumin, pain control and antibiotics. Liver enzymes continued to improve with return to baseline in 2 weeks. Subsequent clinic follow up showed complete control of ascites. DISCUSSION: Potential sequelae of TIPS are reported more frequently in cirrhotic with a MELD score greater than 18. Hepatic infarction is an uncommon development after a TIPS procedure. However, physicians must be aware of this virtually serious, post TIPS complication in cirrhotics even with a low MELD, which may progress to acute liver failure, with a need for liver transplant. Though our patient responded to supportive care, we think, an ideal patient planned for elective TIPS should be aware of this rare phenomenon. Prompt diagnosis is imperative to decrease the mortality from hepatic infarction.

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INTRODUCTION: Cystic fibrosis (CF) poses considerable morbidity and mortality. Largely identified in children with “typical” pulmonary problems, some present past adolescence and instead display “atypical” complaints. CF liver disease (CFLD) has a significant disease burden as a leading cause of death in CF. Symptom variation makes diagnosing CF in adults difficult. Our case report describes a diagnosis of atypical CF and CFLD in adulthood after nearly two decades of inconclusive findings. The case highlights hepatic sequelae of CF, disease diversity, and the impact of delayed diagnosis. CASE DESCRIPTION/METHODS: We present a 32-year-old African American female first noted to have elevated liver function tests (LFTs) on routine labs at age 16, yet was asymptomatic. Despite numerous clinical encounters over 17 years - including chemistry panels, imaging, and biopsies - the etiology remained unclear. In adulthood, she began experiencing chronic abdominal pain, and was later found to have critically low magnesium levels resulting in a seizure. The differential was expanded, and sweat chloride testing was positive, though genetic testing was negative. Given clinical history of high LFTs with abdominal pain and hypomagnesemia, appearance of symptoms past age 18, and unfixed results, she qualified for the diagnosis of atypical CF with CFLD. Her delayed diagnosis was further masked by unconventional misnomers not typically seen in CF. The patient was enlisted by our CF team for treatment and surveillance, and her LFTs improved. DISCUSSION: Our patient's clinical course made the diagnosis of CF less obvious, including: age, race, lack of family history, absence of pulmonary complications, presence of fertility, and vagueness of complaints. While sweat chloride testing was positive, genetic testing was negative. Retrospectively, each result lends itself to the diagnosis of atypical CF, which may show negative genetics despite sweat testing. The presence of a positive sweat test and clinical history is consistent with non-classic CF, and the chronically elevated LFTs and abdominal complaints were indicative of CFLD. Formal declaration of CFLD is important, as it leads to biliary cirrhosis and portal HTN, imposing high mortality. No gold standard modality exists for CFLD, further making diagnosis difficult in lieu of typical CF features. Our case emphasizes the importance of identifying both atypical CF and CFDL, how limited findings confound diagnosis, and the role of a multi-disciplinary team approach for CF patients.