Combined Liver and Kidney Transplant in Acute Intermittent Porphyria: A Case Report.

BACKGROUND Acute intermittent porphyria is an inherited disease caused by a defect in heme biosynthesis, with accumulation of neurotoxic metabolites leading to acute neurovisceral symptoms. Some patients develop long-term neurological and renal damage after the acute episodes, many of them requiring hemodialysis. Since heme production in the human body occurs predominantly in the bone marrow and liver, liver transplantation has been shown to significantly reduce the production of neurotoxic metabolites, effectively controlling the disease. Patients with severe acute intermittent porphyria who have chronic kidney failure may benefit from combined kidney and liver transplant. Only 2 uses of this approach have been previously reported in the literature. CASE REPORT We report here the case of a 19-year-old male patient who received a combined liver and kidney transplant for the treatment of acute intermittent porphyria. He presented the first symptoms of the disease 4 years before the procedure, with abdominal pain and significant neurological impairment, with weakness requiring prolonged mechanical ventilation. He also had chronic kidney failure secondary to the porphyria. A combined liver and kidney transplant was performed, with no intraoperative complications. The explanted liver showed light siderosis, as well as portal and perisinusoidal fibrosis at microscopy. At 3.5 years of follow-up, he remains clinically well, with normal hepatic and renal function, had had no further acute porphyria episodes, and shows progressive neurological recovery. CONCLUSIONS This case demonstrates that combined liver and kidney transplant can be a curative treatment for patients with severe acute intermittent porphyria associated with end-stage renal failure. The patient shows satisfactory long-term function of both grafts, with no clinical or biochemical signs of porphyria recurrence.

Tacrolimus-Associated Psychotic Disorder: A Report of 2 Cases.

Psychotic disorders are a group of psychiatric disorders characterized by the presence of delusions, hallucinations, bizarre behavior, and disorganized speech. There are several possible causes for the occurrence of psychotic disorders in patients who underwent solid organ transplant, including pre-existing mental illness, electrolyte disturbances, infections of the central nervous system, and adverse reaction to drugs. Calcineurin inhibitors are a class of immunosuppressive drugs, such as tacrolimus and cyclosporine, that are currently considered the mainstay in the immunosuppressive drug regimen of patients who underwent solid organ transplant. Neurotoxicity is one of the adverse reactions associated with the use of calcineurin inhibitors, ranging from upper limb tremors to psychotic disorders and seizures. We report the cases of 2 liver transplant recipients who developed severe psychotic disorder 1 month after the procedure. After an extensive investigation for other possible triggers of psychiatric disease, the use of tacrolimus was considered to be the most likely cause for the acute psychotic disorder. In less than 24 hours after suspension of that drug, all symptoms disappeared in both patients, making a causal relationship with tacrolimus even more likely. The patients were then given cyclosporine, another drug from the same class, allowing for adequate immunosuppression and preserved graft function, with no further psychiatric symptoms. This report confirms that a 24-hour trial of tacrolimus suspension can be safe and effective in the diagnosis of drug-related psychotic disorders in patients who underwent liver transplant. This article is compliant with the Helsinki Congress and the Istanbul Declaration.

Visceral Leishmaniasis in a Liver Transplant Patient: A Case Report.

Leishmaniasis is an infection caused by protozoa of the genus Leishmania, transmitted by sandflies and endemic to more than 88 countries. Visceral leishmaniasis in immunosuppressed patients is a growing concern. We report the case of a 61-year-old male patient with a previous history of alcoholic cirrhosis and portal vein thrombosis who underwent liver transplantation for the treatment of hepatocellular carcinoma. Thirty-six days after the procedure, the patient showed an increase in liver enzymes and was diagnosed with moderate acute rejection of the graft. He was treated with high-dose intravenous corticosteroids, and while showing improvement in biochemical markers, he became febrile 12 days after corticosteroid treatment. He presented daily episodes of fever, even after the use of several antimicrobial, antiviral, and antifungal agents, and a number of negative cultures from different sites were obtained. A bone marrow biopsy was then performed, showing a large number of amastigote forms of Leishmania spp. Treatment with liposomal amphotericin B was initiated; however, the patient progressed to refractory septic shock and death. This case highlights several aspects of visceral leishmaniasis in liver transplant recipients, such as the association of malnutrition to Leishmania infection and the challenges of diagnosing leishmaniasis in cirrhotic patients in which splenomegaly and pancytopenia, the hallmarks of leishmaniasis, may also be attributed to portal hypertension and end-stage liver disease. A high index of suspicion is necessary for the correct diagnosis and treatment of leishmaniasis in this group of patients. This study is compliant with the Helsinki Congress and the Istanbul Declaration.