Liver Transplantation because of Acute Liver Failure due to Heme Arginate Overdose in a Patient with Acute Intermittent Porphyria

In acute attacks of acute intermittent porphyria, the mainstay of treatment is glucose and heme arginate administration. We present the case of a 58-year-old patient with acute liver failure requiring urgent liver transplantation after erroneous 6-fold overdose of heme arginate during an acute attack. As recommended in the product information, albumin and charcoal were administered and hemodiafiltration was started, which could not prevent acute liver failure, requiring super-urgent liver transplantation after 6 days. The explanted liver showed no preexisting liver cirrhosis, but signs of subacute liver injury and starting regeneration. The patient recovered within a short time. A literature review revealed four poorly documented cases of potential hepatic and/or renal toxicity of hematin or heme arginate. This is the first published case report of acute liver failure requiring super-urgent liver transplantation after accidental heme arginate overdose. The literature and recommendations in case of heme arginate overdose are summarized. Knowledge of a potentially fatal course is important for the management of future cases. If acute liver failure in case of heme arginate overdose is progressive, super-urgent liver transplantation has to be evaluated

Role of the reverse-Trendelenberg patient position in maintaining low-CVP anaesthesia during liver resections.

BACKGROUND AND AIMS: The Trendelenberg position is recommended during liver resection, to decrease the risk of venous air embolism. However, this position raises the central venous pressure and may increase blood loss. We propose that the reverse-Trendelenberg position can be safely and effectively used to maintain a low central venous pressure during liver surgery. MATERIALS AND METHODS: Fifty consecutive patients underwent elective liver resection at a single centre during a 17-month period. Patients were positioned with a head-up tilt during division of the liver parenchyma. RESULTS: Patients had a mean central venous pressure of 9.2 mmHg when supine, despite fluid restriction. The central venous pressure fell consistently and rapidly when they were tilted head-up, to a mean of 1.7 mmHg. The resections were completed with a median operative blood loss of 600 mL. No patient developed a clinically apparent venous air embolism. Postoperative renal dysfunction that could be attributed to low central venous pressure anaesthesia occurred in only one case. CONCLUSION: The reverse-Trendelenberg position effectively lowers the CVP during liver surgery. It is easy to monitor, titrate and reverse, and avoids the need for complex pharmacological interventions. We recommend this position to liver surgeons and anaesthetists who have found it difficult to maintain a low CVP with the supine or Trendelenberg positions

Audit of the use of regular Haem Arginate infusions in patients with Acute Porphyria to prevent recurrent symptoms

The National Acute Porphyria Service (NAPS) provides acute care support and clinical advice for patients in England with active acute porphyria requiring haem arginate treatment and patients with recurrent acute attacks. This audit examined the benefits and complications of regular haem arginate treatment started with prophylactic intent to reduce the frequency of recurrent acute attacks in a group of patients managed through NAPS. We included 22 patients (21 female and 1 male) and returned information on diagnosis, indications for prophylactic infusions, frequency and dose, analgesia, activity and employment and complications including thromboembolic disease and iron overload. The median age at presentation with porphyria was 21 years (range 9–44), with acute abdominal pain as the predominant symptom. Patients had a median of 12 (1–400) attacks before starting prophylaxis and had received a median of 52 (0–1,350) doses of haem arginate. The median age at starting prophylaxis was 28 years (13–58) with a median delay of 4 years (0.5–37) between presentation and prophylaxis. The frequency of prophylactic haem arginate varied from 1 to 8 per month, and 67% patients were documented as having a reduction in pain frequency on prophylaxis. Only one patient developed clinically significant iron overload and required iron chelation, but the number of venous access devices required varied from 1 to 15, with each device lasting a median of 1.2 years before requiring replacement. Six patients stopped haem arginate and in three this was because their symptoms had improved. Prophylactic haem arginate appears to be beneficial in patients with recurrent acute porphyria symptoms, but maintaining central venous access may prove challenging