Liver dysfunction usually accompanies metabolic decompensation in fatty acid oxidation disorders, including carnitine palmitoyltransferase (CPT) Ia deficiency. Typically, the liver is enlarged with raised plasma transaminase activities and steatosis on histological examination. In contrast, cholestatic jaundice is rare, having only been reported in long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency. We report a 3-year-old boy with CPT Ia deficiency who developed hepatomegaly and cholestatic jaundice following a viral illness. No cause for the jaundice could be found, apart from the fatty acid oxidation disorder. Liver histology showed diffuse, predominately macrovesicular steatosis, hepatocellular and canalicular cholestasis but no bile duct paucity or evidence of large duct obstruction. The liver dysfunction resolved in 4-7 weeks.
Download full-text PDF |
Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3575043 | PMC |
| http://dx.doi.org/10.1007/8904_2012_135 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Serum Vitamin D Status in Infants with Cholestatic Jaundice.
Mymensingh Med J
January 2025
Dr Subir Ananda Biswas, Resident, Department of Paediatric Gastroenterology & Nutrition, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh; E-mail:
Cholestatic jaundice is a potentially serious condition that requires early diagnosis for proper management. Fat-soluble vitamin (FSV) deficiency develops as a consequence of cholestasis. Vitamin D deficiency is common and remains a challenge in patients with cholestasis.
View Article and Find Full Text PDFComputed Tomographic Findings in Lemmel Syndrome: A Rare Cause of Obstructive Jaundice.
Cureus
November 2024
Radiology, Government Medical College & Hospital, Jammu, Jammu, IND.
Introduction: Obstructive jaundice resulting from a duodenal diverticulum is known as Lemmel syndrome. Lemmel syndrome should be included in the differential diagnosis in patients presenting with obstructive jaundice in the absence of choledocholithiasis, mass, or a stricture.
Aims And Objectives: To describe the computed tomography (CT) findings in patients with Lemmel syndrome.
Advances in minimally invasive treatment of malignant obstructive jaundice.
World J Gastrointest Surg
December 2024
Department of Outpatient, Puer People's Hospital, Puer 665000, Yunnan Province, China.
Malignant obstructive jaundice (MOJ) encompasses a range of diseases stemming from malignant tumors such as cholangiocarcinoma, pancreatic cancer, and primary liver cancer, among others, which cause obstruction in both intra- and extra-hepatic bile ducts. This obstruction may lead to elevated bilirubin levels, hepatic function impairment, and a low rate of successful surgical resection in clinical settings. There are various minimally invasive treatment options for MOJ, including endoscopic biliary drainage, ultrasound-guided procedures, and percutaneous biliary tract puncture drainage.
View Article and Find Full Text PDFRobotic-assisted Kasai portoenterostomy for child biliary atresia.
World J Gastrointest Surg
December 2024
Department of Pediatric, The Seventh Medical Center of PLA General Hospital, Beijing 100700, China.
Background: The Kasai procedure (KPE) is an important treatment for biliary atresia (BA), the most common cause of neonatal obstructive jaundice.
Aim: To investigate the efficacy of robotic-assisted Kasai portoenterostomy (RAKPE) in patients with BA.
Methods: Clinical data of 10 patients with BA who underwent RAKPE at the Seventh Medical Center of the People's Liberation Army General Hospital between December 2018 and December 2021 were retrospectively analyzed.
Demographic Profiles, Etiological Spectrum, and Anatomical Locations of the Post-Hepatic Obstructive Jaundice in Adult Population in Hadhramout Region in Yemen.
Diseases
December 2024
Department of Diagnostic Radiology, College of Applied Medical Sciences, Taibah University, Al-Madinah Al-Munawwarah 41477, Saudi Arabia.
Background: Obstructive jaundice is a common health challenge in daily clinical practice caused by a heterogeneous group of benign and malignant conditions in or around extrahepatic bile ducts. This study aimed to investigate the causes of obstructive jaundice, analyze the age and sex distribution, and report the locations of obstruction.
Methods: This was a retrospective study of electronic records of patients diagnosed with obstructive jaundice in the Hadhramout region in Yemen.
Want AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!