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Liver transplantation
Published in Mark Davenport, James D. Geiger, Nigel J. Hall, Steven S. Rothenberg, Operative Pediatric Surgery, 2020
Caroline Lemoine, Riccardo A. Superina
A broad range of investigations may be indicated but the preoperative workup should be customized based on the underlying cause of liver failure. Malnutrition is common because of malabsorption, anorexia, and catabolism. Nutritional status is a major determinant of outcome. Aggressive nutritional support, including nasogastric or even parenteral nutrition, may be needed. Bleeding esophageal varices are treated, preferably by variceal band ligation. Viral screening should include cytomegalovirus (CMV) and Epstein–Barr virus (EBV) serology, both of which are important potential post-transplant pathogens. Patients should be immunized against Pneumococcus and hepatitis A and B in addition to routine vaccines. Live vaccines, such as varicella, are best given at least 30 days before transplant, as they are generally contraindicated in the immunosuppressed patient.
Lysosomal acid lipase deficiency: Wolman disease/cholesteryl ester storage disease
Published in William L. Nyhan, Georg F. Hoffmann, Aida I. Al-Aqeel, Bruce A. Barshop, Atlas of Inherited Metabolic Diseases, 2020
Deficiency of the same lysosomal acid lipase that is defective in Wolman disease is found in cholesteryl ester storage disease [26]. Patients with this disorder have a much more indolent disorder which may present with otherwise asymptomatic hepatomegaly or hepatosplenomegaly in childhood or adulthood [27–33]. Massive splenomegaly and a splenic abscess were reported in one patient [32]. Recurrent abdominal pain has occurred in some patients, and some have had recurrent epistaxis or intestinal bleeding. There may be evidence of cirrhosis on biopsy. Esophageal varices have occasionally been observed [31, 34, 35]. Acute or chronic hepatic failure has been reported in a few patients [35, 36]. Some are icteric. Clotting factors, including prothrombin and factor V, may be reduced. Some patients have hyperlipemia and elevation of the plasma concentration of cholesterol. Pulmonary hypertension has been reported as a complication, leading to death at 18 years [36]. Hyperlipoproteinemia type IIb is commonly encountered, and some patients have xanthelasma. There may be impressive premature atherosclerosis.
Obstruction of the Respiratory Orifices, Larynx, Trachea and Bronchia
Published in Burkhard Madea, Asphyxiation, Suffocation,and Neck Pressure Deaths, 2020
In cirrhosis of the liver, particular attention should be paid to any ruptured oesophageal varices. On occasion, a gastric ulcer may be the source of the haemorrhage. If there is reason to suspect that lung tissue is the source of the haemorrhage, additional histological and serological examinations will be necessary where no relevant syndrome was diagnosed antemortem. Any skull fractures, injuries to the soft tissues in the head and neck region and lung injuries that could be the origin of blood aspiration must be established.
Comparison of transjugular intrahepatic portosystemic with endoscopic treatment plus anticoagulation for esophageal variceal bleeding and portal vein thrombosis in liver cirrhosis
Published in Scandinavian Journal of Gastroenterology, 2022
Wenyue Wu, Hejiao Zhang, Zhuang Zeng, Xi Wang, Derun Kong
ET was performed under local anesthesia by an experienced endoscopist (Derun Kong) following routine procedures [19]. EVL was performed using multipleband devices (Wilson-Cook Medical, Winston-Salem, NC, USA). Briefly, during EVL, 4–6 elastic bands were placed to ligate the varices. During EIS, a mixture of lauromacrogol (Tianyu, Xi'an, Shanxi, China) and methylene blue (Jumpcan, Jinan, Shandong, China) was intravariceally injected into the target varices at a ratio of 100:1. The total amount did not exceed 35 ml, with 0.5-10 ml per injection point. Patients underwent ET every 4 weeks until the complete eradication of esophageal varices. Subsequently, subcutaneous injection of LMWH was administered for 7-14 days, followed by a choice between continuous LMWH and oral warfarin for at least 6 months or until the complete recanalization of PVT.
Cirrhosis and partial portal thrombosis leading to severe variceal bleeding, an unusual presentation of sarcoidosis
Published in Acta Clinica Belgica, 2022
Marco Moretti, Pierre Lefesvre, Joop Jonckheer
Physical examination revealed an apyretic patient (36.6°C tympanic), with an arterial blood pressure of 117/71 mmHg and a regular heartbeat of 119 bpm. The patient was not icteric. Abdominal examination revealed hepato-splenomegaly with a painless abdomen. Blood analysis showed an increased urea of 123 mg/dL (normal range: 21–43 mg/dL) and creatinine of 1.11 mg/dL (normal range: 0.51–0.95 mg/dL) with an estimated glomerular filtration rate of 53 ml/min (normal values <60 ml/min), a hyperkaliemia of 5.4 mmol/L (normal range: 3.4–4.5 mmol/L), and a hyperlactatemia of 6.5 mmol/L (normal range: 0.7–2.1 mmol/L). Increased liver tests were observed (aspartate and alanine transferase within the normal range, alkaline phosphatase of 993 U/L (normal range: 35–104 U/L), gamma glutamyl-transferase of 421 U/L (normal values <40 U/L), and total bilirubin of 1.24 mg/dL (normal range: 0–1.2 mg/dL) with direct bilirubin of 0.8 mg/dl (normal range: 0–0.3 mg/dL)). Profound microcytic anemia with hemoglobin of 6.4 g/dL (normal range: 11.7–15.1 g/dL) was found. Gastroscopy detected esophageal varices grade II with active bleeding. No clear varices were found within the stomach, but blood clots were visualized on the fundus. Elastic banding was performed. The patient was initially treated with somatostatin, ceftriaxone (2 g qd for 7 days), and a transfusion of two blood units. The patient was admitted to the intensive care unit (ICU) for hemodynamic monitoring.
Nonalcoholic steatohepatitis (NASH) cirrhosis: a snapshot of therapeutic agents in clinical development and the optimal design for clinical trials
Published in Expert Opinion on Investigational Drugs, 2022
Pankaj Aggarwal, Mazen Noureddin, Stephen Harrison, Sophie Jeannin, Naim Alkhouri
In addition to histologic endpoints, another reasonable efficacy endpoint for clinical trials may be endoscopic endpoints. Preventing the development of esophageal varices has been established as a reasonable clinical endpoint in other liver-related conditions [64,65]. Reasonably, the time-to-development or progression of esophageal varices in patients with NASH cirrhosis could serve as a tangible and consequential endpoint; however, this presents certain challenges including the subjectivity of endoscopic assessment of small varices. An alternative could be the time-to-development of clinically significant portal hypertension (CSPH). Although traditionally defined as HVPG of 10 mmHg or higher as obtained via transjugular liver biopsy, another method to assess for CSPH is by using liver stiffness from VCTE and platelet count according to the Baveno criteria 66. Additionally, machine learning algorithms have demonstrated that CSPH can be reliably predicted from histology in patients with NASH cirrhosis, which could subsequently be trended as a secondary efficacy endpoint in a clinical trial [67].