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M Rela

Publications and source records attributed to M Rela.

At least 37 records · Page 2Linked to original sources
PubMed2001

Proton nuclear magnetic resonance analysis of hepatic bile from donors and recipients in human liver transplantation.

The current shortage of donor organs in liver transplantation has led experienced transplant centers to use more "marginal" grafts. The development of a reliable technique of bile collection gives access to hepatic bile from donors and recipients for bile analysis to characterize the grafts. Proton nuclear magnetic resonance analysis has been applied to the study of bile for more than 30 years, showing encouraging results. This is the first study where proton nuclear magnetic resonance analysis has been applied to hepatic bile from selected liver grafts to evaluate its potential role in graft assessment. Hepatic bile was collected from eight liver donors (four with normal and four with steatotic grafts) during organ retrieval and four transplant recipients (two with good early graft function and two with primary dysfunction) immediately after graft reperfusion. A Varian Unity+ NMR spectrometer, operating at 11.7 Tesla (500 MHz for 1H), was used to obtain the proton nuclear magnetic resonance spectra. The results showed that the hepatic bile from steatotic grafts collected before transplantation had more intense phosphatidylcholine head group resonance than bile from normal grafts. It also showed slower clearance of University of Wisconsin solution in grafts with subsequent primary graft dysfunction, suggesting a slower recovery of bile secretion. These preliminary findings suggest that proton nuclear magnetic resonance analysis might help to differentiate the characteristics of bile acids and biliary lipids from normal and steatotic grafts. The monitoring of the resonance signal of University of Wisconsin solution washout, bile acid, and biliary lipid secretion may help to predict the development of primary graft dysfunction and avoid the need for retransplantation.

Adolescent↗
PubMed2001

Calcineurin-inhibitor related nephrotoxicity- reversibility in paediatric liver transplant recipients.

AIM: To study the efficacy of mycophenolate mofetil (MMF) as renal rescue in paediatric liver transplant recipients with calcineurin-inhibitor- (CI) related nephrotoxicity. METHODS: Pediatric liver transplant recipients with stable graft function and a glomerular filtration rate (GFR) <80 ml/min/1.73 m2 were enrolled. MMF was introduced at 20 mg/kg/day and increased to 40 mg/kg/day after 1 week. CI dose was then reduced 6 weeks to achieve blood levels 25% of baseline. GFR was reassessed after 6 and 12 months. RESULTS: Fourteen children with a median (range) interval from transplant of 57 (4-111) months were studied. Their median (range) GFR in ml/min/1.73 m2 increased from a baseline of 52 (31-71), to 69 (38-111) and 73 (35-98) at 6 and 12 months, respectively (P=0.00014). Side effects of MMF include leucopaenia in two and backache in one, two of whom discontinued MMF. Acute allograft rejection occurred in three children. All 14 are well with a median (range) follow-up of 24 (14-38) months from MMF introduction. CONCLUSION: MMF allows the recovery of renal function from CI related nephrotoxicity in more than 70% of paediatric liver transplant recipients with renal impairment.

Adolescent↗
PubMed2001

Clinical and biochemical outcome of hepatorenal transplantation for hereditary systemic amyloidosis associated with apolipoprotein AI Gly26Arg.

BACKGROUND: Treatment of systemic amyloidosis comprises measures to support failing organ function coupled with attempts to reduce the supply of the respective amyloid fibril precursor protein. Orthotopic hepatic transplantation is effective in familial amyloid polyneuropathy associated with variant transthyretin, because this protein is produced almost exclusively in the liver. Hepatic transplantation has not been performed in hereditary apolipoprotein AI (apoAI) amyloidosis, and the liver's contribution to plasma apoAI levels has not been determined in vivo. METHODS: A 57-year-old Irish man with hereditary systemic amyloidosis associated with apoAI Gly26Arg, which had led to end-stage renal failure and progressive liver dysfunction, underwent hepatorenal transplantation. His outcome was followed clinically and his amyloid deposits were monitored with serum amyloid P component scintigraphy. The proportion of variant apoAI in the plasma was estimated by quantitative isoelectric focusing before and after liver transplantation. RESULTS: Plasma levels of variant apoAI decreased by 50% after liver transplantation, and the patient was asymptomatic 2 years after surgery. Subclinical amyloid deposits that were present in his spleen and heart preoperatively have regressed and stabilized respectively. CONCLUSIONS: Orthotopic liver transplantation substantially reduces the supply of the amyloid fibril precursor protein in hereditary apoAI amyloidosis, and the excellent outcome in this patient probably reflects the balance between deposition and turnover of amyloid having been altered in favor of the latter. These findings support the use of liver transplantation in patients with hereditary apoAI amyloidosis who develop hepatic dysfunction.

Amino Acid Substitution↗
PubMed2001

Sclerosing encapsulating peritonitis after orthotopic liver transplantation.

BACKGROUND: The etiology of abdominal cocoon (a rare cause of intestinal obstruction) is unknown. It has occurred in adolescent girls, cirrhotic patients after peritoneal-venous shunting, and patients undergoing peritoneal dialysis. We report our experience with patients after orthotopic liver transplantation (OLT). METHODS: Five patients (4 male, 1 female, aged 16 to 57 years) underwent OLT (3 whole liver, 2 right lobe grafts) and subsequently developed abdominal cocoon. RESULTS: All developed pyrexia by 66 +/- 21 hours posttransplant. Additional symptoms (epigastric discomfort and intermittent vomiting) occurred 12 +/- 10 days later. Bacterial peritonitis was confirmed by microbiology in 2 cases and diagnosed by exclusion in the others. C-reactive protein levels were persistently elevated in all patients (35 to 82 mg/L). While abdominal CT consistently demonstrated marked ascites with the small intestine confined to a particular area of the abdomen, intestinal contrast studies and ultrasound were not diagnostic. All patients underwent surgical removal of the cocoon membrane by 58 +/- 22 days after transplant. CONCLUSIONS: Sclerosing peritonitis may complicate liver transplantation and occurs because of low-grade intra-abdominal sepsis.

Adolescent↗
PubMed2001

Living related adult-to-adult liver transplantation: meeting the donor shortage.

Experience in the Cromwell Hospital London, Adult-to-Adult Living Donor Liver Transplantation programme is described with particular reference to the results obtained in the first five recipients. The first two of these received a left lobe graft and the remaining three a right lobe graft. Three sons/daughters, and two siblings were the donors. Four of the five recipients survived and did well. The one recipient who died was a complicated retransplant procedure. The donors showed rapid recovery of liver function with normal tests by the 10th day and with evidence of regeneration on follow-up CT volume evaluation. The value of the procedure for patients who have little chance of obtaining a cadaver organ is undoubted, but critical assessment of the recipient's clinical state is essential if success is to be obtained with a small graft and at all times the safety of the donor must remain of paramount concern, as reports to date indicate instances of donor death in adult programme.

Adolescent↗
PubMed2001

Use of cadaveric superior mesenteric artery as interpositional vascular graft in orthotopic liver transplantation.

BACKGROUND: Hepatic artery thrombosis remains the most common technical complication that causes graft failure following orthotopic liver transplantation. The development of split liver and living related liver transplantation has led to the use of shorter and smaller arteries for arterial reconstruction to the graft. The present aim was to assess the effectiveness of the superior mesenteric artery as an interpositional graft in arterial reconstruction during liver transplantation. METHODS: Cadaveric superior mesenteric artery was used to reconstruct small and short or multiple hepatic arteries in 35 liver transplants including 29 split, three living related, two whole liver transplants and one emergency revascularization post-transplantation. RESULTS AND CONCLUSION: A low incidence of hepatic artery thrombosis (one of 35 patients) was achieved utilizing cadaveric superior mesenteric artery as an interpositional vascular graft in liver transplantation.

Adolescent↗
PubMed2001

Graft dysfunction mimicking autoimmune hepatitis following liver transplantation in adults.

In children, a type of graft dysfunction associated with autoimmune features has been described. We have identified 7 adult liver-transplant (LT) recipients from a series of over 1,000 consecutive transplant recipients who presented between 0.3 years and 7.2 years following transplantation with characteristic symptoms, autoantibody profiles, and histologic findings of autoimmune disease. The indications for transplantation were Ecstasy overdose, alcohol-related cirrhosis, primary sclerosing cholangitis (PSC) (2), primary biliary cirrhosis (PBC), hepatitis C cirrhosis, and cryptogenic cirrhosis. Two patterns of de novo autoantibody development were noted; anti-liver-kidney-microsome (LKM) antibody development at high titer in association with an aspartate transaminase (AST) > 500 and antinuclear (ANA) and antismooth muscle (AMA) antibody development at titers >1/80 with lower AST levels. All cases had elevated IgG. Liver biopsies showed changes of an autoimmune-type hepatitis with portal and periportal hepatitis in association with a marked infiltrate of plasma cells, lymphocytes, and bridging collapse. Two patients lost their grafts because of the disease. Patients were treated with reintroduction of steroids and azathioprine in cases in which it had been withdrawn. Major histocompatibility class I and II mismatching did not incur risk. Eight of 12 liver allografts were acquired from either DRB*0301- or DRB*0401-positive donors, and 4 recipients were DRB*0301-positive. This series illustrates that both symptoms and histologic findings of graft dysfunction compatible with autoimmune hepatitis (AIH) exist in adult LT recipients. Graft loss may be a consequence. This entity may represent a specific type of rejection that should currently be classified as "graft dysfunction mimicking autoimmune hepatitis."

Adult↗
PubMed2001

Successful outcome of orthotopic liver transplantation in patients with preexisting malignant states.

Preexisting malignancy is considered a relative contraindication to orthotopic liver transplantation (OLT) because of the risk of tumor recurrence. The purpose of this study is to assess the outcome of OLT in patients with a preexistent malignant state. Of 1,097 OLTs performed between 1989 and 1999 at King's College Hospital (London, UK), 18 patients had a pretransplantation malignant state, including 6 cases of myeloproliferative disorder (MPD) presenting as Budd-Chiari syndrome. Those patients with solid-organ malignancies had their tumor detected at an early stage and underwent curative treatment before or during OLT. Patients were followed up for a median of 71 months (range, 1 to 119 months) post-OLT, and the rates of rejection and malignancy were compared with those of transplant recipients without preexisting malignancy during the same period. One patient had a recurrence of his primary malignancy (non-Hodgkin's lymphoma) after 27 months, whereas another patient developed a de novo posttransplant lymphoproliferative disorder after 57 months. One patient with MPD developed acute leukemia 72 months after OLT. In comparison, of 1,079 OLTs performed in patients without preexisting malignancy during the same period, there were 34 cases of de novo malignancies. The rate of rejection in patients with and without preexisting malignancy was similar. Successful medium-term outcome after OLT can be achieved in carefully selected patients with preexisting malignancy providing the malignancy is amenable to curative treatment before or at OLT. Primary MPDs responsible for Budd-Chiari syndrome should not be considered a contraindication to OLT.

Adult↗
PubMed2001

Living related liver transplantation in biliary atresia with absent inferior vena cava.

The success of the triangulation technique for hepatic venous anastomosis in left lateral segment liver transplantation has led to standardization of this procedure. We report a case of syndromic biliary atresia with absent inferior vena cava in which we constructed a neo cava to implant a living related left lateral segment graft by using the triangulation technique.

Biliary Atresia↗
PubMed2001

Survival and outcome after hepatic artery thrombosis complicating pediatric liver transplantation.

BACKGROUND/PURPOSE: Hepatic artery thrombosis (HAT) represents a significant cause of graft loss and mortality after pediatric orthotopic liver transplantation (OLT). The incidence and etiology of this complication have been investigated in detail but relatively little is known about outcome. METHODS: A review was conducted of all children with confirmed HAT complicating OLT during a 10-year period (1990 through 1999) in a single center. HAT was established by angiography or at operation in all cases. RESULTS: From a consecutive series of 400 pediatric OLTs, there were 31 (7.8%) instances of HAT in 29 children of median age 3.8 years (range, 8 days to 16 years). Twenty-four (83%) are alive after a median follow-up of 3.6 years. Fourteen cases occurred after transplantation of whole grafts and 17 after reduced or split livers. Of the 18 episodes resulting in retransplantation, there were 5 deaths and 2 second episodes of HAT; surviving children are alive with good graft function. Of the 13 episodes managed without retransplantation, 4 patients underwent attempted early revascularisation of the graft, which was successful in 2, and the remainder initially were treated conservatively. All 13 children are alive after a median follow-up of 4.1 years (range, 0.6 to 5.8), but 5 required radiologic or surgical intervention for biliary or septic complications; biochemical liver function is normal in 8, mildly abnormal in 3, and poor in 2. Retransplantation was less likely in those who had received reduced or split grafts (7 of 17) compared with those who had received whole grafts (11 of 14), but this difference just failed to reach statistical significance (chi(2) = 3.01, 0.1 > P > .05). CONCLUSIONS: Using a selective policy of retransplantation, revascularisation, and conservative treatment, 83% of children survived HAT complicating OLT. Approximately 40% of children with HAT survived without retransplantation. J Pediatr Surg 36:888-891.

Child↗
PubMed2001

Glycopeptide-resistant Enterococcus faecium infections in paediatric liver transplant recipients: safety and clinical efficacy of quinupristin/dalfopristin.

We describe our experience of quinupristin/dalfopristin for glycopeptide-resistant Enterococcus faecium (GREF) infections in 19 paediatric liver transplant recipients. The median patient age was 2 years and all were receiving immunosuppressive regimens. Quinupristin/dalfopristin was well tolerated and complete resolution of infection was seen in 74% of patients. Side-effects included reversible elevation of serum alkaline phosphatase, skin rash, itching, diarrhoea and vomiting, but therapy was not withdrawn from any patient. Quinupristin/dalfopristin appears safe and efficacious in critically ill immunocompromised children with renal or hepatic impairment.

Adolescent↗
PubMed2001

Liver and intestinal lactate metabolism in patients with acute hepatic failure undergoing liver transplantation.

OBJECTIVE: To determine the relative contribution of the gastrointestinal tract and the liver in lactate metabolism in patients with acute liver failure (ALF) and the effect of liver transplantation on this. We hypothesized that the liver and gut are net producers of lactate in ALF and that this is reversed after liver transplantation. SETTING: A university-affiliated specialist liver transplant operating theater. SUBJECTS: Eleven patients with ALF undergoing liver transplantation. MEASUREMENTS AND INTERVENTIONS: After ethical approval, 11 patients with ALF listed for orthotopic hepatic transplantation were studied. Whole blood was analyzed for lactate concentration from radial artery (RA) catheter, portal vein (PV), and hepatic vein (HV) during the dissection phase and was repeated postreperfusion of the liver graft. Gradients across the gut and the liver were calculated to see if there was net production or consumption. RESULTS: HV lactate was significantly higher than arterial (p =.028) in patients with ALF before liver transplantation, suggesting splanchnic production of lactate. Total splanchnic lactate gradient (HV-RA) is positive in ALF. Both the gut (PV-RA) and the liver (HV-PV) were net producers of lactate. After liver transplantation, hepatic venous lactate falls below arterial levels but not significantly. The gradient across the gut (PV-RA) remained positive, but the transhepatic gradient (HV-PV) became significantly negative, showing consumption by the graft (p =.021). The magnitude of lactate consumption after transplantation correlated positively with portal venous lactate concentration (p =.029) and inversely with graft cold ischemic time (p =.007). CONCLUSION: The liver is a net producer of lactate in patients with ALF and an elevated whole blood lactate. After liver transplantation, the graft becomes a consumer of lactate as shown by the negative lactate gradient. The degree of consumption is dependent on portal venous lactate concentration and cold ischemic time.

Female↗
PubMed2000

Recurrence of Langerhans cell histiocytosis in the graft after pediatric liver transplantation.

Two girls were diagnosed with Langerhans cell histiocytosis (LCH) at the age of 16 and 7 months and developed end stage chronic liver disease related to LCH-induced sclerosing cholangitis at 28 and 8 months, respectively. They received liver transplants at 34 and 14 months of age. Five months post-orthotopic liver transplantation (OLT) one of the patients developed posttransplant lymphoproliferative disease, successfully treated with a combination of surgery and reduction of immunosuppression. Fourteen months post-OLT she developed diabetes insipidus, bilateral ear discharge, and new osteolytic lesions. After transplantation both girls had mild skin reactivations of LCH, requiring minimal steroid increments. At 60 and 5 months post-OLT intrahepatic LCH recurrence was diagnosed on the basis of abnormal biliary enzymes and presence of Langerhans cells in the grafts. Initial cholangiography in both patients was unremarkable. LCH activity was controlled by maintenance chemotherapy with vinblastine, etoposide, and prednisolone. Ten months after reappearance of LCH in the liver graft a follow-up cholangiography in one of the girls demonstrated a low grade cholangiopathy. Residual elevation of liver enzymes probably represents an ongoing pathogenic process.

Cholangiography↗
PubMed2000

Isolated liver transplant and sequential small bowel transplantation for intestinal failure and related liver disease in children.

Liver dysfunction is a well-recognized complication of intestinal failure in children. Advances in total parenteral nutrition (TPN) have allowed these children to survive while their intestinal tract gradually adapts. Unfortunately TPN may lead to cholestatic liver disease particularly in the young children. Progression of liver disease is associated with a poor prognosis and is an indication for small bowel transplantation. We report our experience of orthotopic liver transplantation in four children with short gut and sequential liver and small bowel transplantation in one child. All children had TPN-related liver failure. Causes of intestinal failure included necrotising enterocolitis (n=2), gastroschisis (n=1), intestinal atresia (n=1), and megacystic, microcolon syndrome (n=1). At the time of liver transplantation the children's mean age was 10.9 months (2.5-24) and weight 6.7 kg (4.8-10.1). The mean serum bilirubin was 522 micromol/liter (299-823), aspartate transaminase 423 IU/liter (49-1024) and international normalized ratio 2.8 (2-3.9). There were two deaths both from respiratory failure secondary to adenovirus pneumonia including the child who received a sequential small bowel transplant. Three children with isolated liver grafts are alive and off TPN at 20 months (mean) follow up (range 6-35). Isolated orthotopic liver transplantation has a role in selected children with intestinal failure, particularly those with short but normally functioning gut and progressing with satisfactory intestinal adaptation but developing liver disease. Those children with TPN-related liver disease and unadapted gut or irreversible intestinal disease need combined liver and small bowel transplantation. Sequential small bowel transplantation is feasible after orthotopic liver transplantation and may provide an option for the child with terminal liver and small bowel failure.

Adolescent↗
PubMed2000

Size reduction of small bowels from adult cadaveric donors to alleviate the scarcity of pediatric size-matched organs: an anatomical and feasibility study.

BACKGROUND: Small bowel transplantation in children weighing less than 10 kg is hindered by the lack of size-matched donors. The ability to create reduced size small bowel grafts from adult cadaveric donors suitable for use in young children has been studied. METHODS: Volumetric assessment of computed tomography scans were used to evaluate abdominal cavity and small bowel volumes in children. Small bowels were retrieved from adult cadaveric donors and reduced in size. RESULTS: Computed tomography studies of the abdominal cavity showed that the mean volume available for a small bowel graft was 260 ml in children less than 5 kg (n = 5) and 460 ml in children weighing 5-10 kg (n = 5). Fifteen small bowels were successfully reduced to provide an ileal graft of one meter while keeping the whole length of the superior mesenteric artery and vein after their dissection in the proximal part of the mesentery. The mean volume of the grafts created was 270 ml in seven thin patients (body mass index [BMI] <25), 390 ml in five preobese patients (25< BMI<30), and 490 ml in three obese patients (BMI>30). Mesenteric transillumination in thin donors allowed safe dissection and complete hemostasis. No diameter reduction was required. Technical modifications permitted the creation of two grafts, one ileal and the other jejunal from a single donor. Volumetric and surgical data show that implantation of up to two meters of ileum from a thin adult weighing up to 80 kg is feasible in children weighing less than 10 kg. CONCLUSION: Size reduction of adult cadaveric small bowels can provide suitable grafts for children of less than 10 kg and could expand the potential pool of donors for these patients.

Adult↗