Progressive cardiac failure following orthotopic liver transplantation for type IV glycogenosis

1992 ◽  
Vol 151 (3) ◽  
pp. 200-203 ◽  
Author(s):  
E. M. Sokal ◽  
F. Van Hoof ◽  
D. Alberti ◽  
J. de Ville de Goyet ◽  
T. de Barsy ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Orthotopic Liver Transplantation ◽  
Cardiac Failure ◽  
Type Iv ◽  
Type Iv Glycogenosis

scholarly journals High output cardiac failure in 3 patients with hereditary hemorrhagic telangiectasia and hepatic vascular malformations, evaluation of treatment

2020 ◽  
Vol 15 (1) ◽  
Author(s):  
Lilian B. Olsen ◽  
Anette D. Kjeldsen ◽  
Mikael K. Poulsen ◽  
Jens Kjeldsen ◽  
Annette D. Fialla
Keyword(s):  
Liver Transplantation ◽  
Orthotopic Liver Transplantation ◽  
Cardiac Failure ◽  
Hereditary Hemorrhagic Telangiectasia ◽  
Arteriovenous Malformations ◽  
Vascular Malformations ◽  
Symptom Relief ◽  
Case Series ◽  
Hospital Treatment ◽  
High Output

Abstract Background This report addresses how patients with hereditary hemorrhagic telangiectasia (HHT) and high output cardiac failure (HOCF) due to hepatic vascular malformations, should be evaluated and could be treated. HHT is a genetic disorder, leading to vascular abnormalities with potentially serious clinical implications. In the liver, arteriovenous malformations occur in more than 70% of patients, but only about 8% present clinical symptoms such as HOCF with pulmonary hypertension and less commonly portal hypertension, biliary ischemia and hepatic encephalopathy. Results Three female patients with HHT type 2 and HOCF caused by severe arteriovenous malformations in the liver are presented in this case series. The patients were seen at the HHT-Centre at Odense University Hospital. Treatment with either orthotopic liver transplantation (one patient) or bevacizumab (two patients) was initiated. All patients experienced marked symptom relief and objective improvement. New York Heart Association—class were improved, ascites, peripheral edema and hence diuretic treatment was markedly reduced or discontinued in all three patients. Bevacizumab also resulted in notable effects on epistaxis and anemia. Conclusion Our findings substantiate the importance of identification of symptomatic arteriovenous malformations in the liver in patients with HHT. Bevacizumab may possibly, as suggested in this case series and supported by previous case studies, postpone the time to orthotopic liver transplantation or even make it unnecessary. Bevacizumab represents a promising new treatment option, which should be investigated further in clinical trials.

scholarly journals An unusual case of cardiac failure following orthotopic liver transplantation

2018 ◽  
Vol 18 (4) ◽  
pp. 1026-1028
Author(s):  
Neal Bhutiani ◽  
Dylan Adamson ◽  
Mary Eng ◽  
Christopher M. Jones
Keyword(s):  
Liver Transplantation ◽  
Orthotopic Liver Transplantation ◽  
Cardiac Failure ◽  
Unusual Case

Cardiac Failure After Orthotopic Liver Transplantation

1994 ◽  
Vol 78 (1) ◽  
pp. 179???180 ◽  
Author(s):  
Joseph M. Levine ◽  
James D. Kindscher
Keyword(s):  
Liver Transplantation ◽  
Orthotopic Liver Transplantation ◽  
Cardiac Failure

Fine Structure of Type IV Glycogenosis

1981 ◽  
Vol 39 ◽  
pp. 464-465
Author(s):  
P.K. Simons
Keyword(s):  
Needle Biopsy ◽  
Tissue Concentration ◽  
Inborn Errors ◽  
Rare Type ◽  
Type Iv ◽  
Iron Copper ◽  
Proper Diagnosis ◽  
Type Iv Glycogenosis ◽  
Loss Of Appetite ◽  
Enzymatic Defect

Glycogenosis is defined as any condition in which the tissue concentration of glycogen is increased. There are currently ten recognized variants of glycogenosis that are heritable inborn errors of metabolism. The specific enzymatic defect in each of the variants is known or at least suspected. In all cases, the enzymatic defect prevents the proper metabolism or formation of the glycogen molecule. The clinical and histologic differences between the types of glycogenosis is important to a proper diagnosis after the presence of such a condition is realized. This study was initiated to examine the ultrastructure of the rare Type IV Glycogenosis (Amylopectinosis) of which there is very little morphologic characterization in the literature.Liver tissue was obtained by needle biopsy from a 12-month-old Oriental female who was originally admitted to the hospital after observation of poor development, loss of appetite, and hepatomegaly. The majority of the tissue was fixed for light microscopy in neutral buffered formalin and processed using routine and special staining procedures (reticulin, trichrome, iron, copper, PAS, PAS-diastase and PAS-pectinase.

Biliary cast syndrome after orthotopic liver transplantation: risk factors and outcome

2013 ◽  
Vol 51 (01) ◽  
Author(s):  
T Voigtländer ◽  
AA Negm ◽  
CP Strassburg ◽  
F Lehner ◽  
MP Manns ◽  
...  
Keyword(s):  
Risk Factors ◽  
Liver Transplantation ◽  
Orthotopic Liver Transplantation ◽  
Cast Syndrome

Prevalence and predictors of renal impairment after orthotopic liver transplantation

2013 ◽  
Vol 51 (01) ◽  
Author(s):  
MF Sprinzl ◽  
H Tönissen ◽  
A Weinmann ◽  
N Lohse ◽  
S Koch ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Renal Impairment ◽  
Orthotopic Liver Transplantation

Evolution of Urokinase-Type Plasminogen Activator (u-PA) and Tissue-Type Plasminogen Activator (t-PA) in Orthotopic Liver Transplantation (OLT)

1993 ◽  
Vol 69 (01) ◽  
pp. 056-059 ◽  
Author(s):  
G Himmelreich ◽  
G Dooijewaard ◽  
P Breinl ◽  
W O Bechstein ◽  
P Neuhaus ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Plasminogen Activator ◽  
Orthotopic Liver Transplantation ◽  
Tissue Type ◽  
Bleeding Complications ◽  
Activity Levels ◽  
Tissue Type Plasminogen Activator ◽  
Type Plasminogen Activator ◽  
Urokinase Type Plasminogen Activator ◽  
Anhepatic Phase

SummaryIn orthotopic liver transplantation (OLT) hyperfibrinolysis seems to be of causative importance for intra- and postoperative bleeding. Although recently hyperfibrinolysis has been successfully reduced by intraoperative aprotinin treatment, small increases of fibrinolysis still remain during OLT. Originally, tissue-type plasminogen activator (t-PA) was considered to be responsible for the increases, but the efficacy of aprotinin which inhibits besides plasmin also kallikrein and urokinase-type plasminogen activator (u-PA) suggested also a role for the intrinsic and contact system-dependent plasminogen activators. We investigated the role of u-PA. From 29 patients undergoing OLT with intraoperative aprotinin infusion arterial blood samples were taken at 7 different time points. The preoperative median values for u-PA antigen (u-PA Ag) and plasmin-activatable single-chain u-PA (scu-PA) levels, which were more than 2-fold above normal (both: p <0.01), decreased slightly during the preanhepatic phase and remained unchanged during the anhepatic phase. With reperfusion of the graft liver the two levels decreased significantly (p = 0.0003 and p = 0.006, respectively) to almost normal values, probably due to clearance by the graft liver. Active two-chain u-PA (tcu-PA) was preoperatively 2-fold above the detection limit, remained stable during the preanhepatic phase and increased 2-fold in the anhepatic phase (p = 0.0018). As expected tcu-PA also relapsed upon reperfusion, but to the preoperatively enhanced level, possibly caused by sustained activation of scu-PA by cathepsin B. t-PA activity levels were at the upper end of the normal range preoperatively, slightly increased during preanhepatic and anhepatic phases and decreased significantly with reperfusion. The increases in tcu-PA and t-PA activities during the anhepatic phase coincided with greatly increased fibrinolysis as demonstrated by thrombelastography, indicating that both u-PA and t-PA are involved in the development of fibrinolysis during OLT.One patient was excluded from statistical evaluations because preoperative u-PA Ag, scu-PA, tcu-PA and t-PA activity levels were much higher than in the other 28 patients. In the investigated group this patient was the only one with diffuse peritonitis intraoperatively and severe bleeding complications postoperatively which made retransplantation mandatory.

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