1/30. Hemophagocytic syndrome presenting as acute hepatic failure in two infants: clinical overlap with neonatal hemochromatosis.Two patients with hemophagocytic lymphohistiocytosis who presented with acute liver failure are reported. Both presented with fever, hepatosplenomegaly, markedly elevated liver function tests, abnormal coagulation profiles, and an increase in serum ferritin. Both infants were diagnosed with neonatal hemochromatosis based on a clinical picture of hepatic insufficiency with hyperferritinemia and were referred for liver transplantation. The first patient died of liver failure and septicemia before transplantation. review of autopsy material revealed a hepatitis-like pattern and extensive infiltration of liver and other organs including bone marrow by histiocytes, some of which were hemophagocytic. The second patient underwent liver transplantation but died 44 days thereafter from progressive hemophagocytic lymphohistiocytosis. Examination of the resected liver demonstrated a hepatitis-like pattern, proliferation of histiocytes, and hemophagocytosis, and the bone marrow revealed hemophagocytic histiocytosis. Hemophagocytosis recurred in the allograft. Hepatic manifestations are common in hemophagocytic lymphohistiocytosis and overt hepatic failure may occur, but initial presentation as fulminant hepatic failure is not well recognized. Elevated serum ferritin can make the distinction from neonatal hemochromatosis and other forms of neonatal liver failure difficult. Hemophagocytic lymphohistiocytosis should be considered in the differential diagnosis of neonatal liver disease, especially when it is accompanied by cytopenias.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
2/30. Hepatic amyloidosis with light chain deposition disease. A rare association.Monoclonal immunoglobulin deposition diseases are due to pathological protein deposition in various tissues and organs. Protein deposits may be found in a single tissue or systemically and the organs most frequently involved are kidney, heart, peripheral nerves and the liver. Depending on the pattern of the deposits and the type of immunoglobulin, these diseases are distinguished as primary amyloidosis, light chain deposition disease. Differential diagnosis is made in tissue specimens: microscopically by the identification of positive congo red staining of the deposits, by immunohistochemical demonstration of proteins reacting with light chain (lambda or kappa) antisera or by recognition of fibrillar structures on electron microscopy. We report an unusual case of light chain deposition disease associated with amyloidosis, where hepatomegaly was the presenting manifestation and liver failure the cause of death, without any kidney involvement.- - - - - - - - - - ranking = 2keywords = organ (Clic here for more details about this article) |
3/30. Severe perinatal liver disease associated with fetal thrombotic vasculopathy.Three neonates with fetal thrombotic vasculopathy in the placenta and severe neonatal liver disease are described. Symptoms included a bleeding disorder on the first day of life, followed by direct hyperbilirubinemia and elevated liver transaminases. All patients also had evidence of thrombosis outside the placenta, including cerebral infarct in two infants and thrombosis of the inferior vena cava in one infant. Liver disease was demonstrated to be thrombotic in one infant who died with budd-chiari syndrome. Two infants survived and had liver biopsy with cholestasis, bile duct proliferation, and portal fibrosis demonstrated at 4 weeks and 11 weeks of age, respectively. The etiology of thrombosis is unknown, though in one patient an excessively long and coiled umbilical cord may be implicated. The prenatal onset of thrombosis suggests an inherited or acquired thrombophilic state. In cases of enigmatic neonatal liver disease, an association with thrombosis should be considered and thrombi sought in placenta, umbilical cord, major blood vessels, and other organs. Evaluation for a hypercoagulable state is also suggested.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
4/30. Tolterodine-associated acute mixed liver injury.OBJECTIVE: To report a patient with an acute mixed liver injury associated with tolterodine therapy. CASE SUMMARY: An 81-year-old white woman with urge incontinence experienced malaise, fever, and gastrointestinal disturbances 18 days after starting tolterodine 2 mg twice daily. The patient's concurrent medications included flunitrazepam, diclofenac, and dorzolamide/timolol eye drops. Laboratory examination was consistent with the presentation of an acute mixed liver injury with increased transaminase enzymes, alkaline phosphatase, gamma-glutamyltransferase, and bilirubin. Additionally, she had mild leukocytosis with eosinophilia. After tolterodine was discontinued, the abnormal liver and hematologic parameters returned to normal within 4 weeks. DISCUSSION: Tolterodine, a muscarinic receptor antagonist, has predominantly anticholinergic effects. To our knowledge, this is the first case published describing tolterodine-associated acute mixed liver injury. However, some of the patient's additional symptoms can also be considered part of a drug-induced hypersensitivity syndrome. This is usually defined by the triad of fever, cutaneous reaction, and involvement of internal organs, mainly affecting the liver. The close temporal relationship between the start of tolterodine therapy and the first symptoms and the reversibility after dechallenge led us to conclude that the adverse reaction was possibly related to tolterodine exposure. CONCLUSIONS: Our case illustrates that tolterodine may rarely be associated with liver injury. This may have been an organ manifestation of tolterodine-induced hypersensitivity syndrome.- - - - - - - - - - ranking = 2keywords = organ (Clic here for more details about this article) |
5/30. Green skin discoloration associated with multiple organ failure.OBJECTIVE: To report intense green hyperpigmentation related to FD & C Blue No. 1. DESIGN: Retrospective case review. SETTING: Surgical intensive care unit. PATIENT: A 67-yr-old woman with unstable angina, electrocardiographic S-T segment elevation, and a left ventricle thrombus requiring emergent coronary revascularization surgery. INTERVENTIONS: Postoperative monitoring and treatment for multiple organ dysfunction that included small-bowel tube feeding and propofol sedation. MEASUREMENTS AND MAIN RESULTS: The patient developed an intense green skin color. CONCLUSION: patients with multiple organ failure may be at risk for unusual pigmentation effects from tube feeding dyes.- - - - - - - - - - ranking = 6keywords = organ (Clic here for more details about this article) |
6/30. Early experiences with a porcine hepatocyte-based bioartificial liver in acute hepatic failure patients.Orthotopic liver transplantation (OLT) is the only effective therapeutic modality in severe acute hepatic failure (AHF). The scarcity of organs for transplantation leads to an urgent necessity for temporary liver support treatments in AHF patients. A hepatocyte-based bioartificial liver (BAL) is under investigation with the main purpose to serve as bridging treatment until a liver becomes available for OLT, or to promote spontaneous liver regeneration. We developed a novel radial-flow bioreactor (RFB) for three-dimensional, high-density hepatocyte culture and an integrated pumping apparatus in which, after plasmapheresis, the patient's plasma is recirculated through the hepatocyte-filled RFB. Two hundred thirty grams of freshly isolated porcine hepatocytes were loaded into the RFB for clinical liver support treatment. The BAL system was used 8 times in supporting 7 AHF patients in grade III-IV coma, all waiting for an urgent OLT Three patients with no history of previous liver diseases were affected by fulminant hepatic failure (FHF) due to hepatitis b virus, 3 by primary non-function (PNF) of the transplanted liver, and one by AHF due to previous abdominal trauma and liver surgery. Six out of 7 patients underwent OLT following BAL treatment(s), which lasted 6-24 hours. All patients tolerated the procedures well, as shown by an improvement in the level of encephalopathy, a decrease in serum ammonia, transaminases and an amelioration of the prothrombin time, with full neurological recovery after OLT Our initial clinical experience confirms the safety of this BAL configuration and suggests its clinical efficacy as a temporary liver support system in AHF patients.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
7/30. mars procedure as a bridge to combined liver-kidney transplantation in severe chromium-copper acute intoxication: a paediatric case report.We describe a case of multi-organ failure (liver-kidney insufficiency and brain oedema) caused by accidental, acute intoxication with a chromium and copper-containing substance, as an example of the introduction of the new extracorporeal procedure mars (molecular adsorbents recirculating system) in a girl 3.5 years old.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
8/30. liver failure in adult Still's disease during corticosteroid treatment.adult Still's disease is a well-characterized rheumatic disorder of unknown origin, which may affect multiple organs and may have a fatal course. However, liver failure has rarely been described in adult Still's disease. We present the case of a 25-year-old woman who was admitted with acute liver failure 2 years after the start of symptoms (arthritis, fever, sore throat) of a yet undefined rheumatic disease. She had been treated with prednisolone for 2 months before admission. The diagnosis of adult Still's disease was made in accordance with well-established criteria. Other causes of liver failure were excluded. Withdrawal of prednisolone did not affect the course of liver disease. ursodeoxycholic acid therapy was started when the patient slowly began to recover. To the best of our knowledge, this is the first case of adult Still's disease reported in which hepatic failure developed when other symptoms were well controlled by corticosteroid treatment.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
9/30. Re-use of a liver allograft; an exceptional opportunity to enlarge the organ donor pool.Liver allograft re-use is an exceptional way of enlarging the donor pool. We describe here a case of a re-used liver allograft, originating from an insulin-intoxicated donor and transplanted at first into a recipient presenting with hyperacute liver failure due to paracetamol intoxication. Because the original recipient developed an irreversible cerebral oedema, the allograft was re-implanted electively 55.5 h later into a patient with post-viral C cirrhosis and solitary hepatocarcinoma. Both donor and recipient operations were technically successful; liver function after the second use of the graft was normal.- - - - - - - - - - ranking = 4keywords = organ (Clic here for more details about this article) |
10/30. Case report of the first severe acute respiratory syndrome patient in china: successful application of extracorporeal liver support mars therapy in multiorgan failure possibly induced by severe acute respiratory syndrome.A previously healthy patient was transferred to our infectious department with a 9-day-history of continued fever. The patient was placed on assisted respiration support in addition to anti-viral medication. The diagnosis of SARS (severe acute respiratory syndrome) was made in view of the severe hypoxemia and the characteristic symptoms exhibited by the patient. Despite the best intensive therapy, he clinically deteriorated into multiorgan dysfunction syndrome (MODS) including additional dysfunction of kidney, liver, and heart. We initiated mars therapy (extracorporeal liver support utilizing albumin dialysis) with intention to positively influence the organ functions in his MODS on the basis of recently published studies which suggested a positive impact of mars in multiorgan failure secondary to respiratory illnesses and the possible influence on inflammatory mediators and cytokines. The application of 4 intermittent mars treatments (8 h each, mean blood flow rate 180 ml/min) on 4 consecutive days resulted in an immediate improvement of clinical conditions within the treatment days. The further improvement of organ functions allowed withdrawing the patient from ventilatory support 13 days after start of mars, and 44 days after admission he was discharged home with completely resolved organ functions and laboratory abnormalities. SARS is a severe form of the epidemic outbreak of atypical pneumonia which remains poorly defined regarding etiology and special therapy recommendations. However, the development and aggravation of this ARDS-like severe acute respiratory syndrome is pathologically associated with the systemic inflammatory response syndrome (SIRS) which may then mediate or cause MODS. To our knowledge, this is the first report of an application of mars therapy in MODS which was probably induced by SARS in a patient in china which improved the clinical condition of the patient in multi-organ failure secondary to respiratory failure indicating that mars might be an additional therapeutic option in multiorgan failure induced by SARS.- - - - - - - - - - ranking = 11keywords = organ (Clic here for more details about this article) |
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