Acute Liver Failure With Transient Liver Steatosis Following Multiple Hits Postoperatively in a Patient With Limb-Girdle Muscular Dystrophy: A Case Report.

Transient liver steatosis is rarely described. A fast development of liver steatosis leading to acute liver failure (ALF) is, to our knowledge, rarely observed. It is so far observed and published in acute fatty liver of pregnancy, and in a few cases of ALF. However, it is observed in elective surgery for pancreaticoduodenectomy and some cases of cytostatic treatment, without subsequent development of ALF. Paracetamol toxicity within the maximum daily allowed dosage (and only a few days of paracetamol administration) prior to the development of ALF has been described in eight patients with neuromuscular disease (NMD). These patients carried genotypes consistent with altered drug metabolism, possibly changing the hepatic disposition of paracetamol. In this report, we describe a patient case of limb-girdle muscular dystrophy, a subgroup of the NMD, that developed acute liver steatosis within 30 h and subsequently ALF. A 36-year-old white woman was electively admitted for a surgical diversion with an end-colostomy due to chronic constipation. Postoperatively, she was exposed to different factors potentially affecting liver function (multiple hits against the liver), such as paracetamol administration in maximal daily allowed dose, a hypotensive event, and a redo surgery due to perforated colon on postoperative Day 21. Subsequently, she developed severe ALF three days later. The patient responded to standard medical treatment for ALF and was discharged 2 months after the initial hospital admission. Pharmacogenetic analyses indicated a change in paracetamol metabolism towards increased level of toxic metabolite. Six months after the admission, both CT and MR scans showed complete regression of the liver steatosis; this was in addition confirmed with normal liver elastography. To our knowledge, this is the first reported clinical observation of a transient acute liver steatosis with complete regression to normal liver function and morphology. Moreover, it is of great importance to early recognize the development of acute steatosis since it is one of multiple liver hits that predisposes these patients to develop ALF. The importance of pharmacogenetics for risk in paracetamol-induced liver toxicity should be further investigated.