Publications by authors named "J Pfeiffenberger"
Article Synopsis
- Glycogen storage disorder type IIIa is a rare genetic condition affecting the liver and muscles, where liver transplantation can help with metabolic control but does not address muscle issues.
- A case study describes a 31-year-old man who had a liver transplant for end-stage liver disease due to GSD type IIIa, but afterward, he experienced worsening muscle pain and elevated enzyme levels linked to muscle involvement rather than the liver.
- The increase in muscle symptoms post-transplant may be due to improved liver function which led to excess glucose in the muscle cells, and while a high-protein ketogenic diet was attempted, it did not significantly help the patient's muscle pain.
Background: The combination of gemcitabine and cisplatin (gem/cis) with the anti-PD-L1-antibody durvalumab was recently approved as first line therapy for biliary tract cancer (BTC) based on the results of the TOPAZ-1 trial.
Objective: We aim to analyse the feasibility and efficacy of the triple combination therapy in patients with BTC in a real-world setting and in correspondence with the genetic alterations of the cancer.
Methods: In this single-centre retrospective analysis, all patients with BTC and treated with durvalumab plus gem/cis from April 2022 to September 2023 were included.
Background & Aims: Prevention of neurological worsening (NW) under therapy is an unmet need in the management of Wilson disease (WD). In this study, we aimed to characterize the occurrence, associated outcomes and potential reversibility of NW in WD.
Methods: From a total cohort of 457 patients with WD, 128 patients with WD and neurological features at any time point (all Caucasian, 63 females, median age at diagnosis 22 years) were identified by chart review at University Hospital Heidelberg and grouped according to initial presentation.
Background: Rare liver lesions and diseases have seldomly aroused major interest of researchers. For most guidelines, presumably similar clinical conditions are pooled without detailed investigations of singularities that they present.
Main Text: A multidisciplinary project aiming to establish evidence-based therapies for rare liver diseases has been founded.
Spleen transient elastography predicts actuarial survival after liver transplantation.
Transl Gastroenterol Hepatol
July 2022
Background: Splenic transient elastography (TE) correlates with increased portal pressure. Little data are available in the post liver transplantation (LTx) setting.
Methods: Three months after LTx, we performed splenic TE in 125 LTx recipients.