Logo American Journal of Case Reports

Call: 1.631.629.4328
Mon-Fri 10 am - 2 pm EST

Contact Us

Logo American Journal of Case Reports Logo American Journal of Case Reports Logo American Journal of Case Reports

: Case report  Saudi Arabia

[In Press] Management of Patients with Alagille Syndrome Undergoing Living Donor Liver Transplantation: A Report of 2 Cases

Rare disease

Odai Jamaan Alqahtani1ABCDEFG, Riaz Nazeer Ahmad1ADFG, Abdullah Bakr Abolkhair1ADEFG, Aljazi Dhari Alrashid1ABCDEFG

Am J Case Rep In Press; DOI:   :: ID: 936513

Available online: , In Press, Corrected Proof

Publication in the "In-Press" formula aims at speeding up the public availability of the pending manuscript while waiting for the final publication. The assigned DOI number is active and citable. The availability of the article in the Medline, PubMed and PMC databases as well as Web of Science will be obtained after the final publication according to the journal schedule

Abstract

BACKGROUND
Alagille syndrome (AGS) is a rare genetic disease characterized by 5 typical features: peculiar facial anomaly, posterior embryotoxon, chronic cholestasis, butterfly-like vertebral-arch defects, and cardiovascular malformations. AGS in a liver transplant setting is particularly rare in Saudi Arabia. This case report presents successful anesthetic management in AGS patients during liver transplantation surgery.
CASE REPORT
We present here 2 patients with AGS who underwent liver transplantation surgery. Case 1 describes a 3-year-old boy who was diagnosed with AGS at the age of 2 weeks, manifesting as a prominent forehead, deep-set eyes, pointed chin, and butterfly-shaped vertebrae, along with coarctation of the aorta, peripheral branch pulmonary artery stenosis, direct hyperbilirubinemia, cholestasis, and G6PD deficiency. Case 2 describes a 12-year-old girl, known to have AGS, who presented with decompensated liver disease, portal hypertension, splenomegaly, hypersplenism, and portal vein thrombosis, as well as the other dysmorphic features of AGS, such as a prominent forehead, deep-set eyes, pointed chin, and butterfly-shaped vertebrae. The anesthetic management of the 2 patients started from the perioperative period with careful assessment and optimization of the cardiovascular system, intraoperative maintenance of stable hemodynamics, the use of inhaled nitric oxide when clinically indicated (used in case 1), and the use of goal-directed management of fluid as well as blood and blood products. These all played a major role in the successful management of anesthesia for these patients.
CONCLUSIONS
The important features of successful anesthetic management included thorough preoperative cardiovascular system evaluation and intra-operative maintenance of normal temperature, ionized calcium, pH, hemoglobin, and stable hemodynamics.

Keywords: Alagille Syndrome; Anesthesia; Liver Transplantation

SARS-CoV-2/COVID-19

29 June 2022 : Case report  USA

A Clinical Case of COVID-19 Vaccine-Associated Guillain-Barré Syndrome

Am J Case Rep In Press; DOI: 10.12659/AJCR.936896  

16 June 2022 : Case report  Ecuador

Multidrug-Resistant Klebsiella pneumoniae in a Patient with SARS-Cov-2 Pneumonia in an Intensive Care Unit ...

Am J Case Rep In Press; DOI: 10.12659/AJCR.936498  

13 June 2022 : Case report  Japan

Stenotrophomonas maltophilia Infection Associated with COVID-19: A Case Series and Literature Review

Am J Case Rep In Press; DOI: 10.12659/AJCR.936889  

13 June 2022 : Case report  Saudi Arabia

Atlantoaxial Subluxation Secondary to SARS-CoV-2 Infection: A Rare Orthopedic Complication from COVID-19

Am J Case Rep In Press; DOI: 10.12659/AJCR.936128  

In Press

01 Jul 2022 : Case report  Poland

A 75-Year-Old Female Smoker with Advanced Small-Cell Lung Cancer and Eastern Cooperative Oncology Group Per...

Am J Case Rep In Press; DOI: 10.12659/AJCR.936536  

30 Jun 2022 : Case report  Philippines

Erasmus Syndrome: A Case Report and Literature Review

Am J Case Rep In Press; DOI: 10.12659/AJCR.937061  

30 Jun 2022 : Case report  USA

A Clinical Case of COVID-19 Vaccine-Associated Guillain-Barré Syndrome

Am J Case Rep In Press; DOI: 10.12659/AJCR.936896  

30 Jun 2022 : Case report  China (mainland)

Septic Shock Induced by Acute Pyelonephritis Resulting from Kidney Stones Treated by Double-J Ureteral Sten...

Am J Case Rep In Press; DOI: 10.12659/AJCR.936967  

Most Viewed Current Articles

23 Feb 2022 : Case report  USA

Penile Necrosis Associated with Local Intravenous Injection of Cocaine

DOI :10.12659/AJCR.935250

Am J Case Rep 2022; 23:e935250

17 Feb 2022 : Case report  Oman

Myocarditis, Pulmonary Hemorrhage, and Extensive Myositis with Rhabdomyolysis 12 Days After First Dose of P...

DOI :10.12659/AJCR.934399

Am J Case Rep 2022; 23:e934399

06 Dec 2021 : Case report  Brazil

Lipedema Can Be Treated Non-Surgically: A Report of 5 Cases

DOI :10.12659/AJCR.934406

Am J Case Rep 2021; 22:e934406

09 Feb 2022 : Case report  Saudi Arabia

Extensive Cerebral Venous Sinus Thrombosis (CVST) After the First Dose of Pfizer-BioNTech BNT162b2 mRNA COV...

DOI :10.12659/AJCR.934744

Am J Case Rep 2022; 23:e934744

Your Privacy

We use cookies to ensure the functionality of our website, to personalize content and advertising, to provide social media features, and to analyze our traffic. If you allow us to do so, we also inform our social media, advertising and analysis partners about your use of our website, You can decise for yourself which categories you you want to deny or allow. Please note that based on your settings not all functionalities of the site are available. View our privacy policy.

American Journal of Case Reports eISSN: 1941-5923
American Journal of Case Reports eISSN: 1941-5923