ABSTRACT
We present a case about a 25-year-old male patient suffering from a rare genetic disorder called Mizuho haemoglobin. He was admitted to the Intensive Care Unit with acute liver and renal failure. During admission he also developed a cardiac tamponade twice. Finally he received a liver transplantation. Hereafter the patient stabilised and his liver and renal functions improved. His symptoms could not be explained solely by his known disease. After searching the literature, similarities between his symptoms and a rare complication of sickle cell disease were found. Molecular diagnostics showed that the patient also suffered from Gilbert's syndrome. Due to his chronic haemolysis, symptoms of this other disease were masked. This stresses the importance of always looking for other causes if symptoms or changes cannot be explained by a known rare disorder.
Subject(s)
Cholestasis, Intrahepatic/diagnosis , Gilbert Disease/diagnosis , Hemoglobins, Abnormal/genetics , Renal Insufficiency/diagnosis , Adult , Bilirubin/blood , Cardiac Tamponade , Cholangiopancreatography, Endoscopic Retrograde , Echocardiography , Gilbert Disease/genetics , Gilbert Disease/surgery , Hemolysis , Humans , Liver Transplantation , Male , Polymorphism, Single Nucleotide , Renal Insufficiency/genetics , Renal Insufficiency/surgeryABSTRACT
Hypoxic hepatitis secondary to heart failure is a known and treatable cause of liver failure. The diagnosis may be difficult, especially when symptoms of heart failure are absent. We present two patients who were transferred to our hospital with the diagnosis of acute liver failure to be screened for a liver transplantation. Both patients had increased serum levels ofaminotransferases, lactic acidosis, coagulation disorders, and non-specific clinical symptoms. Echocardiography revealed right ventricular dysfunction. Treatment with inotropes resulted in a fast normalization of liver enzymes, acidosis and coagulation, confirming the diagnosis hypoxic hepatitis. In conclusion, when the cause of acute liver dysfunction is unclear, hypoxic hepatitis due to heart failure should be considered and echocardiography should be performed, even when symptoms are non-specific for heart failure.
Subject(s)
Heart Failure/complications , Hepatitis/etiology , Hypoxia/complications , Liver Failure/etiology , Acute Disease , Diagnosis, Differential , Female , Heart Failure/diagnostic imaging , Hepatitis/diagnosis , Humans , L-Lactate Dehydrogenase/blood , Liver/enzymology , Liver Failure/diagnosis , Middle Aged , Transaminases/blood , Ultrasonography , Ventricular Dysfunction, Left/complications , Ventricular Dysfunction, Left/diagnostic imagingABSTRACT
UNLABELLED: OBJECTIVE. To describe the experience with combined liver and kidney transplantation at the University Medical Centre Groningen, The Netherlands. DESIGN. Retrospective. METHOD: Data were analysed from all patients who underwent combined liver and kidney transplantation in the University Medical Centre Groningen, in the period November 1994-December 2005. RESULTS: During the study period 582 orthotopic liver transplantations and 1026 isolated kidney transplantations were performed. 16 patients underwent combined liver and kidney transplantation: 4 were children (aged 17 months-16 years) and 12 were adults (aged 19-59 years). For all patients, both organs were obtained from the same post-mortem donor. Indications for combined liver and kidney transplantation were primary hyperoxaluria type I (n=6), polycystic liver and kidney disease (n=3) and unrelated liver and kidney failure (n=7). The 1- and 5-year survival rate was 88% (14/16), which was not significantly different from the results after isolated liver transplantation. Two patients died 11 days and 74 months after combined transplantation, due to complications from unsuccessful retransplantation of the liver for hepatic artery thrombosis and secondary biliary cirrhosis, respectively. A third patient died 51 days after combined transplantation due to sepsis. CONCLUSION: Combined liver and kidney transplantation was a life-saving intervention in this selected group of patients with combined liver and kidney failure. Patient survival was comparable to that of patients undergoing isolated liver transplantation.
Subject(s)
Kidney Failure, Chronic/surgery , Kidney Transplantation , Liver Failure/surgery , Liver Transplantation , Adolescent , Adult , Child , Child, Preschool , Female , Graft Survival , Humans , Hyperoxaluria, Primary/complications , Infant , Kidney Transplantation/methods , Kidney Transplantation/mortality , Liver Transplantation/methods , Liver Transplantation/mortality , Male , Middle Aged , Netherlands/epidemiology , Postoperative Complications/mortality , Retrospective Studies , Survival Analysis , Treatment OutcomeABSTRACT
The identification problem of the dose-limiting tissue component was investigated in the CNS of rats. Moderate single doses of radiation, ranging from 20 to 25 Gy were applied to the brain of adult female rats. The sequence of events was analyzed by scoring a series of morphological changes in one of the white matter structures that appears to represent a sensitive location, that is the fimbria hippocampi. The previously defined "Tissue Injury Unit", characterized by a dilation of the blood vessel lumen, a thickening of the blood vessel wall, an enlargement of endothelial cell nuclei, and a hypertrophy of the adjacent astrocytes which represents a combined score of four different, but related histological changes, proved to be slightly more sensitive and responsive than the earliest recognizable changes in the neurological structures, that is demyelination. In addition, the incidence of demyelination could be expressed as a function of the intensity of the "Tissue Injury Unit". These findings can be interpreted as an additional indication that blood vessel changes and the hypertrophy of the perivascular astrocytes precede degenerative changes in the white matter of the CNS after moderate doses of X rays.
