Browsing by Subject "Jaundice"

Now showing 1 - 4 of 4
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    A Unique Case of Caroli Disease in Kenya's Medical Landscape
    (International Scientific Information, 2024-01-12) Lengoiboni, Timothy Sanny; Kailani, Leway; Bikoro, Maureen K.; Mudeheri, Michael M.; Medicine, School of Medicine
    Background: If a young patient presents with fever, abdominal pain, jaundice and significant imaging abnormalities, especially dilation of the biliary system, it is usually due to obstruction from stones or strictures. However, on very rare occasions, it can be due to complications of congenital cystic dilatation of the biliary system, known as Caroli disease. We present such a patient and discuss the differential diagnosis and implications for long-term management. Case Report: A 14-year-old boy presented to the Emergency Department with a sudden onset of high-grade fever and abdominal pain for 2 weeks, accompanied by vomiting of blood. The patient had no relevant medical history. He was malnourished and had moderate pallor, jaundice, and right upper quadrant pain. Imaging revealed cystic dilatation of intrahepatic ducts and a central dot sign. There were no features suggesting advanced liver disease otherwise, and no tumors or cysts in the kidneys. A diagnosis of Caroli disease was made. The symptoms were ascribed to acute cholangitis and improved with antibiotics. He was discharged home 1 week later. No further blood loss was observed. Conclusions: This case study describes a patient with ascending cholangitis, a complication of Caroli disease. This diagnosis should be considered in the differential diagnosis when a child or young adult presents with features of cholangitis, abnormal biliary imaging, and/or upper gastrointestinal bleeding, or portal hypertension. No prior cases of this disease have been encountered, documented, or published in Kenya. This case can increase awareness among primary care clinicians, including pediatricians.
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    Biliary Obstruction After Transjugular Intrahepatic Portosystemic Shunt Placement
    (Wolters Kluwer, 2021-06-21) Patel, Feenalie; Bick, Benjamin; Pediatrics, School of Medicine
    A 19-year-old man with noncirrhotic portal hypertension status post transjugular intrahepatic portosystemic shunt, gastric esophageal varices status post coil embolization, and thrombophilia because of Factor V Leiden heterozygosity presented with jaundice and elevated liver enzymes. His cholangiogram during endoscopic retrograde cholangiopancreatography demonstrated biliary tract obstruction at the bifurcation of the right and left hepatic ducts. With the aid of digital single-operator cholangioscopy, the patient was found to have a perforation of the common hepatic duct from the shunt. This case presents a novel use for digital single-operator cholangioscopy in identifying this rare complication and appropriately differentiating biliary compression vs perforation from transjugular intrahepatic portosystemic shunt.
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    Febuxostat-induced acute liver injury
    (Wiley, 2016-03) Bohm, Matt; Vuppalanchi, Raj; Chalasani, Naga; Department of Medicine, IU School of Medicine
    Febuxostat is a unique xanthine oxidase inhibitor that was approved for the prevention of gout in the United States in 2009. Unlike allopurinol, febuxostat is not a nucleoside analogue and has a higher specificity for xanthine oxidase. Although this drug has not been on the market for a long time, febuxostat may be safer than allopurinol. Importantly, febuxostat appears to be safer than allopurinol, which has been linked to many instances of severe hypersensitivity reactions, including skin rash with eosinophilia and systemic manifestations (DRESS syndrome), Stevens Johnson syndrome, acute nephritis, acute icteric hepatitis and acute liver failure. While febuxostat is not infrequently associated with serum enzyme elevations during treatment, it has yet to be linked to cases of clinically apparent acute liver injury. We report a case of acute liver injury with jaundice due to febuxostat and which was identified in a prospective study of drug-induced liver injury in the United States.
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    Presentation and Outcomes of Infants With Idiopathic Cholestasis: A Multicenter Prospective Study
    (Wolters Kluwer, 2021-10-01) Hertel, Paula M.; Hawthorne, Kieran; Kim, Sehee; Finegold, Milton J.; Shneider, Benjamin L.; Squires, James E.; Gupta, Nitika A.; Bull, Laura N.; Murray, Karen F.; Kerkar, Nanda; Ng, Vicky L.; Molleston, Jean P.; Bezerra, Jorge A.; Loomes, Kathleen M.; Taylor, Sarah A.; Schwarz, Kathleen B.; Turmelle, Yumirle P.; Rosenthal, Philip; Magee, John C.; Sokol, Ronald J.; Pediatrics, School of Medicine
    Objectives: The aim of the study was to determine the frequency and natural history of infantile idiopathic cholestasis (IC) in a large, prospective, multicenter cohort of infants. Methods: We studied 94 cholestatic infants enrolled up to 6 months of age in the NIDDK ChiLDReN (Childhood Liver Disease Research Network) "PROBE" protocol with a final diagnosis of IC; they were followed up to 30 months of age. Results: Male sex (66/94; 70%), preterm birth (22/90 with data; 24% born at < 37 weeks' gestational age), and low birth weight (25/89; 28% born at <2500 g) were frequent, with no significant differences between outcomes. Clinical outcomes included death (n = 1), liver transplant (n = 1), biochemical resolution (total bilirubin [TB] ≤1 mg/dL and ALT < 35 U/L; n = 51), partial resolution (TB > 1 mg/dL and/or ALT > 35 U/L; n = 7), and exited healthy (resolved disease per study site report but without documented biochemical resolution; n = 34). Biochemical resolution occurred at median of 9 months of age. GGT was <100 U/L at baseline in 34 of 83 participants (41%). Conclusions: Frequency of IC and of death or liver transplant was less common in this cohort than in previously published cohorts, likely because of recent discovery and diagnosis of genetic etiologies of severe/persistent cholestasis that previously were labeled as idiopathic. Preterm birth and other factors associated with increased vulnerability in neonates are relatively frequent and may contribute to IC. Overall outcome in IC is excellent. Low/normal GGT was common, possibly indicating a role for variants in genes associated with low-GGT cholestasis-this warrants further study.