1/14. Curative hepatorenal transplantation in systemic amyloidosis caused by the Glu526Val fibrinogen alpha-chain variant in an English family.A 53-year-old English woman who had been thought to have systemic monoclonal immunoglobulin light chain (AL) amyloidosis was investigated further because of her unusually long 17-year history and a suggestion of renal disease in the family. She was found to have the Glu526Val fibrinogen alpha-chain variant that causes autosomal dominant hereditary systemic amyloidosis. This has not previously been described in a British family. The mutant gene was associated with the same haplotype as in all other reported cases, suggesting a common founder. The patient had already received a renal transplant, but the graft failed within 6 years due to amyloid deposition. Progressive hepatic amyloidosis eventually caused liver failure, although the function of other organs was well preserved. She therefore received hepatic and renal transplants to replace the failed organs and the hepatic source of the amyloidogenic variant fibrinogen. Three years later she is completely well and has no amyloid deposits identifiable by serum amyloid P component scintigraphy. This is the first detailed report of hepatic transplantation for liver failure caused by amyloidosis of any type. The substantial follow-up suggests that fibrinogen alpha-chain amyloidosis is one of the inherited metabolic diseases that can be cured by liver transplantation. The mutation underlying Glu526Val fibrinogen alpha-chain amyloidosis is incompletely penetrant and has a variable phenotype that can clinically mimic AL amyloidosis. Hereditary fibrinogen amyloidosis may be more prevalent than previously suspected and, since AL amyloid is sometimes a diagnosis of exclusion, genotyping for other amyloidogenic proteins is mandatory in all cases in which the amyloid fibrils cannot be positively identified as AL.- - - - - - - - - - ranking = 1keywords = hepatorenal (Clic here for more details about this article) |
2/14. Continuous arteriovenous hemofiltration in the patient with hepatorenal syndrome. A case study.CAVH has gained increasing medical acceptance, as favorable outcomes in the critically ill have proven its worth. Unfortunately, CAVH is often used as a "last ditch" effort for the patient with multisystem organ failure and septicemia. Performing the procedure in such a setting sometimes seems little more than an exercise in futility. However, as more patients such as the patient described in this case study are shown to benefit from CAVH, the nursing role needs to be examined both in performing the procedure and in caring for the critically ill patients. The nursing literature has kept abreast of the procedural aspects of CAVH. Now, critical care nurses must move on to the patient-oriented approach, share case studies, and work together to formulate nursing diagnoses, develop specialized care plans, and identify areas for nursing research.- - - - - - - - - - ranking = 6.0780394677952keywords = hepatorenal syndrome, hepatorenal (Clic here for more details about this article) |
3/14. maternal mortality due to the hepatorenal syndrome of pre-eclampsia. A case report.A maternal death in the 23rd wk of pregnancy is described. The patient was gravida 8, with 6 previous abortions and one growth-retarded term infant. The clinical symptoms and biochemical findings strongly suggest that the mode of death was septic shock, with the hepatorenal syndrome due to pre-eclampsia as the underlying cause.- - - - - - - - - - ranking = 7.597549334744keywords = hepatorenal syndrome, hepatorenal (Clic here for more details about this article) |
4/14. Pseudo-zellweger syndrome: deficiencies in several peroxisomal oxidative activities.We describe an infant girl with a clinical, chemical, and pathologic syndrome remarkably similar to Zellweger cerebrohepatorenal syndrome but whose liver parenchymal cells contained abundant peroxisomes. Peroxisomal L-alpha hydroxy acid oxidase, catalase, and the plasmalogen synthesizing enzyme dihydroxy acetone phosphate-acyl transferase activities were normal; other peroxisomal enzymatic activities, including fatty acyl-coa oxidase and D-amino acid oxidase, were reduced by 80% to 85%. Oxidation of bile acids and pipecolic acid was also deficient. autopsy revealed the presence of neuronal heterotopia, renal cortical cysts, adrenal atrophy, and accumulation of very long chain fatty acids. The clinical and pathologic features of this case of "pseudo-zellweger syndrome" reflect a deficiency in multiple peroxisomal activities rather than a defect in peroxisomal biogenesis. The deficient enzymatic activities require flavin adenine dinucleotide, and the underlying defect may be in the utilization of this cofactor.- - - - - - - - - - ranking = 1.5195098669488keywords = hepatorenal syndrome, hepatorenal (Clic here for more details about this article) |
5/14. Multiple peroxisomal enzymatic deficiency disorders. A comparative biochemical and morphologic study of Zellweger cerebrohepatorenal syndrome and neonatal adrenoleukodystrophy.Biologic, morphologic, and biochemical investigations performed in 2 patients demonstrate multiple peroxisomal deficiencies in the cerebrohepatorenal syndrome of Zellweger (CHRS) and neonatal adrenoleukodystrophy (NALD). Very long chain fatty acids, abnormal bile acids, including bile acid precursors (di- and trihydroxycoprostanoic acids), and C29-dicarboxylic acid accumulated in plasma in both patients. Generalized hyperaminoaciduria was also present. peroxisomes could not be detected in CHRS liver and kidney; however, in the NALD patient, small and sparse cytoplasmic bodies resembling altered peroxisomes were found in hepatocytes. Hepatocellular and Kupffer cell lysosomes were engorged with ferritin and contained clefts and trilaminar structures believed to represent very long chain fatty acids. Enzymatic deficiencies reflected the peroxisomal defects. Hepatic glycolate oxidase and palmitoyl-CoA oxidase activities were deficient. No particle-bound catalase was found in cultured fibroblasts, and ether glycerolipid (plasmalogen) biosynthesis was markedly reduced. Administration of phenobarbital and clofibrate, an agent that induces peroxisomal proliferation and enzymatic activities, to the NALD patient did not bring about any changes in plasma metabolites, liver peroxisome population, or oxidizing activities.- - - - - - - - - - ranking = 7.597549334744keywords = hepatorenal syndrome, hepatorenal (Clic here for more details about this article) |
6/14. Analysis of urinary sediment by transmission electron microscopy. An innovative approach to diagnosis and prognosis in renal disease.Urinary sediment TEM is capable of unequivocally demonstrating renal tubule cells and distinguishing them from urinary tract epithelial cells. The renal tubule cells and the accompaniments including myeloid bodies, inflammatory cells, or fibrin permit, in a particular clinical setting, synthesis of a meaningful renal diagnosis. Sequential TEM sediment studies can clarify ambiguities in diagnosis. Precisely, when much difficulty is experienced in distinguishing ATN from aminoglycoside nephrotoxicity in a patient with sepsis who has received aminoglycoside, urinary sediment TEM can facilitate the differential diagnosis with confidence. In another clinical setting, such as hypersensitivity acute interstitial nephritis, TEM urinary sediment has an irrevocable place by exhibiting the characteristic eosinophil granules that will confirm the above diagnosis, or deny it when they are absent. The morphologic features in the renal tubule cells in the sediment reflect similar changes in the tubular cells in renal tissue. Therefore, the severity of tubular changes are commensurate with the clinical outcome in terms of renal function recovery, need of dialysis, and mortality. The degree of correlation is significant. Thus, slight or no TEM sediment tubular changes signifies a good prospect for renal function recovery and low or no mortality. Conversely, severe tubular changes in the TEM sediment denote persistent renal failure accompanied by high mortality. Furthermore, the most severe tubular changes, found in hepatorenal syndrome, are consistent with its dismal prognosis.- - - - - - - - - - ranking = 1.5195098669488keywords = hepatorenal syndrome, hepatorenal (Clic here for more details about this article) |
7/14. The reversal of the hepatorenal syndrome in four pediatric patients following successful orthotopic liver transplantation.Four pediatric patients are presented in whom profound renal failure (hepatorenal syndrome) developed in association with severe end-stage liver disease. All four patients had successful orthotopic liver transplantation. Special emphasis is given to the preoperative and postoperative renal function in the patients, and the criteria used to establish the diagnosis of the hepatorenal syndrome are discussed. In the initial work on liver transplantation and reversal of the hepatorenal syndrome, two of the three patients recovered renal function but died in the perioperative period. The four patients presented in this report have not only had reversal of the hepatorenal syndrome after successful orthotopic liver transplantation but have also survived long term. The four patients have been followed up for periods ranging from 18 months to 4.5 years. Three of the four patients have maintained near normal renal function, whereas the fourth patient (who had a left nephrectomy for obstruction and sepsis) has had a significant decline in renal function.- - - - - - - - - - ranking = 12.15607893559keywords = hepatorenal syndrome, hepatorenal (Clic here for more details about this article) |
8/14. hepatorenal syndrome complicating chronic lymphocytic leukemia.hepatorenal syndrome and portal hypertension developed in a 59-year-old man with chronic lymphocytic leukemia. At autopsy, he had portal hypertension from lymphocytic infiltration of the liver with compression of the portal veins, and no other pathologic process in the liver. Histologic examination of the kidney did not reveal a lesion that could account for the observed renal insufficiency. This case report documents the association of a lymphoproliferative disorder with portal hypertension and hepatorenal syndrome.- - - - - - - - - - ranking = 1.5195098669488keywords = hepatorenal syndrome, hepatorenal (Clic here for more details about this article) |
9/14. The renal lesion in syndromes of multiple congenital malformations. Cerebrohepatorenal syndrome; Jeune asphyxiating thoracic dystrophy; tuberous sclerosis; Meckel syndrome.Renal malformations in cerebrohepatorenal and the Jeune syndromes are variable, encompassing both focal cystic change and severe cystic dysplasia. Morphologic differences might reflect either genetic heterogeneity or injury to the kidney by an underlying metabolic defect at differing times in renal development. The renal lesion in the Meckel syndrome is cystic dysplasia with markedly defective nephronic differentiation. A histologically distinctive cystic tubular lesion is identified in tuberous sclerosis. These lesions must be differentiated from infantile and adult polycystic disease.- - - - - - - - - - ranking = 6.3280394677952keywords = hepatorenal syndrome, hepatorenal (Clic here for more details about this article) |
10/14. Giant cell transformation cerebrohepatorenal syndrome.An infant with cerebrohepatorenal syndrome of Zellweger had extensive hepatic giant cell transformation at 6 1/2 weeks of age. At 16 weeks the liver showed early cirrhosis and rare giant cells. Changes previously described have ranged from no abnormality in the neonate to cirrhosis at 20 weeks of age and indicate progression of liver disease in affected patients.- - - - - - - - - - ranking = 7.597549334744keywords = hepatorenal syndrome, hepatorenal (Clic here for more details about this article) |
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