RESUMEN
Ten patients with familial Mediterranean fever (FMF) and histologically confirmed amyloidosis received cadaver kidney transplants for treatment of terminal renal disease. Colchicine, 1 mg daily, was included in the routine postoperative regimen from 1974 for amyloidotic patients. Graft and patient survival were compared with ten nonamyloidotic recipients of renal grafts matched for age, sex, type of allograft, and HLA compatibility. In the FMF group, five of ten grafts have survived from 20 to 64 months; in the control group, six of ten. While only recipients with functioning grafts survived in the FMF group, patient survival in the control group is eight of ten after one year. In all five FMF survivors, graft function is satisfactory, proteinuria is absent, and blood creatinine levels are normal. Amyloid involvement of an allograft was documented 16 months after transplantation in the only patient whose maintenance colchicine dosage had been reduced to 0.5 mg daily.
Asunto(s)
Amiloidosis/cirugía , Fiebre Mediterránea Familiar/complicaciones , Enfermedades Renales/cirugía , Trasplante de Riñón , Adulto , Amiloidosis/etiología , Cadáver , Colchicina/administración & dosificación , Femenino , Estudios de Seguimiento , Rechazo de Injerto , Supervivencia de Injerto , Antígenos HLA , Humanos , Israel , Enfermedades Renales/etiología , Masculino , Donantes de Tejidos , Trasplante HomólogoRESUMEN
BACKGROUND: The appropriate use of liver transplantation in children with type-1 primary hyperoxaluria (PH-1) is not well established. We reviewed our experience with 36 children with PH-1, including 12 who underwent liver transplantation. PATIENTS AND METHODS: From 1989-1998, 36 children from 10 families in northern Israel were diagnosed with PH-1. Eight children presented with renal failure; seven of these eight had the severe infantile form of the disease. One child was treated with kidney transplantation alone. Combined liver-kidney transplantation has been performed in nine children and preemptive liver transplantation in three children. A review of the patients' charts for the following parameters was performed: age, clinical signs, and renal sonographic findings at diagnosis, age at onset of dialysis, and current status. Type of transplant, pre- and posttransplant urine oxalate excretion, current renal function, survival, and complications were recorded in liver recipients. RESULTS: Of the 23 nontransplanted children, 9 died of complications related to severe systemic oxalosis and 14 are alive (mean follow-up, 7.4 years), including 2 who are candidates for transplantation. The child who underwent only kidney transplantation died of unrelated causes. Of the 12 liver recipients, 2 died within the first 3 months posttransplant and another child underwent retransplantation due to hepatic arterial thrombosis. At intervals after transplant ranging from 6-54 months, 10 recipients are alive (7 of the 9 recipients of combined liver-kidney transplants and all 3 recipients of preemptive liver transplants). Mean GFR in the 10 survivors is 77 ml/min/m2. In 9 of these 10, daily urinary oxalate excretion normalized. Renal function has improved (mean GFR 86 vs. 58 ml/min/m2) but renal oxalate deposits remain in the three recipients of isolated liver grafts. CONCLUSIONS: Our decade-long experience with children with PH-1 supports strategies for early diagnosis and timely liver transplantation. Preemptive isolated liver transplantation should be considered in children who develop the disease during infancy or in those with slowly progressive disease when significant symptoms develop. Combined liver-kidney transplantation is suggested for children with end-stage renal disease.
Asunto(s)
Hiperoxaluria Primaria/cirugía , Trasplante de Hígado , Adolescente , Adulto , Niño , Preescolar , Estudios de Cohortes , Femenino , Tasa de Filtración Glomerular , Supervivencia de Injerto , Humanos , Hiperoxaluria Primaria/complicaciones , Hiperoxaluria Primaria/mortalidad , Lactante , Recién Nacido , Fallo Renal Crónico/complicaciones , Fallo Renal Crónico/mortalidad , Fallo Renal Crónico/fisiopatología , Fallo Renal Crónico/cirugía , Trasplante de Riñón , Masculino , Análisis de SupervivenciaRESUMEN
BACKGROUND: There is at present very little information about hepatitis B virus (HBV) infection in children after liver transplantation. This is the first study to assess the safety and efficacy of lamivudine in this patient population. METHODS: We describe three children aged 5-14 years who underwent liver transplantation for fulminant hepatitis A, hyperoxaluria, and cystic fibrosis. Despite adequate immunoprophylaxis, two of the children who were serum hepatitis B surface antigen-positive before transplantation (HBV DNA-negative by hybridization) had a reactivation of the disease, and one had a de novo HBV infection, at 12-18 months after transplantation. Lamivudine 3 mg/kg was administered on a compassionate-use basis for 14-36 months. RESULTS: After 1 month of therapy, HBV DNA disappeared from the serum in all patients by hybridization and in two patients by polymerase chain reaction. In all three children, alanine transaminase levels normalized. One child developed lamivudine resistance after 22 months with no evidence of hepatic decompensation. Repeated liver histological studies revealed progression of hepatic fibrosis in one child. All children remained serum hepatitis B surface antigen- and hepatitis B e antigen-positive. No adverse effects of the drug were noted. CONCLUSION: Lamivudine is beneficial and well tolerated in children with HBV infection after liver transplantation.
Asunto(s)
Hepatitis B Crónica/tratamiento farmacológico , Lamivudine/uso terapéutico , Trasplante de Hígado/fisiología , 2-Aminopurina/análogos & derivados , 2-Aminopurina/uso terapéutico , Adolescente , Alanina Transaminasa/sangre , Antivirales/uso terapéutico , Niño , Preescolar , Fibrosis Quística/cirugía , ADN Viral/sangre , Famciclovir , Femenino , Hepatitis A/cirugía , Antígenos de Superficie de la Hepatitis B/sangre , Antígenos e de la Hepatitis B/sangre , Virus de la Hepatitis B/aislamiento & purificación , Humanos , Hiperoxaluria/complicaciones , Fallo Hepático/etiología , Fallo Hepático/cirugía , Donadores Vivos , Masculino , Complicaciones Posoperatorias , Estudios Retrospectivos , Factores de TiempoRESUMEN
BACKGROUND: Veno-occlusive disease (VOD) after liver transplantation is associated with acute rejection and poor outcome. The use of antithrombotic and thrombolytic agents is limited by their toxicity. Defibrotide is a polydeoxyribonucleotide with thrombolytic and antithrombotic properties and no systemic anticoagulant effect. METHODS: Defibrotide, 35-40 mg/kg/day, was administered intravenously for 21 days on a compassionate-use basis to two patients aged 66 and 49 years. VOD had developed 6 weeks and 4 months after orthotopic liver transplantation for hepatitis C and hepatitis B infection, respectively. VOD was diagnosed clinically by findings of weight gain (8.5% and 16%), ascites, jaundice (serum bilirubin 5.4 mg/dl and 21.7 mg/dl), and severe coagulopathy (in one patient), and histologically by the presence of hemorrhagic centrilobular necrosis and fibrous stenosis of the hepatic venules. One of the patients had received azathioprine as part of the immunosuppressive regimen. There was no evidence of acute cellular rejection histologically. RESULTS: After 3 weeks of defibrotide administration, the first patient showed complete clinical resolution of the VOD, and serum bilirubin level normalized. He is alive 6 months after transplantation. The second patient, treated at a later stage of disease, showed marked improvement in the coagulopathic state, but there was no resolution of the VOD. He died 2 months later of multiorgan failure due to Escherichia coli sepsis. Neither patient had side effects from the drug. CONCLUSIONS: Defibrotide is a promising drug for the treatment of VOD after liver transplantation and needs to be evaluated in large, prospective studies.
Asunto(s)
Enfermedad Veno-Oclusiva Hepática/tratamiento farmacológico , Enfermedad Veno-Oclusiva Hepática/etiología , Trasplante de Hígado/efectos adversos , Inhibidores de Agregación Plaquetaria/uso terapéutico , Anciano , Resultado Fatal , Enfermedad Veno-Oclusiva Hepática/patología , Humanos , Hígado/patología , Masculino , Persona de Mediana Edad , Polidesoxirribonucleótidos/uso terapéutico , Resultado del TratamientoRESUMEN
A chronic renal failure patient requiring an access vein for hemodialysis underwent an a-v shunting procedure in the left leg. Post operative proliferating edema of the limb warranted further investigation and iliac vein obstruction syndrome was discovered which existed occulty until venous insufficiency was precipitated by the construction of the a-v shunt. Ilio-caval by-pass graft was capable of correcting the high venous pressure and ensuring the patency of the a-v shunt. This occult syndrome appears to be present in a high precentage of autopsy findings and occurs predominantly on the left side in young females. It is proposed that this high risk groups undergo radionuclide venography before any vascular procedure is undertaken in order to ensure a patent iliac vein.
Asunto(s)
Derivación Arteriovenosa Quirúrgica/métodos , Vena Ilíaca/cirugía , Complicaciones Posoperatorias/cirugía , Diálisis Renal , Adulto , Angiografía , Prótesis Vascular , Constricción Patológica , Edema/cirugía , Femenino , Arteria Femoral/cirugía , Humanos , Vena Ilíaca/diagnóstico por imagen , Isquemia/cirugía , Pierna/irrigación sanguínea , Vena Cava Inferior/cirugíaRESUMEN
Liver transplantation is the treatment of choice for end-stage liver disease. During the past 8 years we performed 102 liver transplants in 84 adults and 16 children. In the adults, 9 were combined transplants: 1 a liver-pancreas transplant for type I diabetes, and 8 liver-kidney transplants. In the children, transplants included 5 whole-livers, 5 left-lateral liver segments from living-related donors, 4 reduced-grafts of right or left lobes, and 2 split left-lateral segments. At a mean follow-up of 31 months (range 1-96) 70 were alive, 3 had died during surgery and 15 during the first postoperative months. Mortality was due to primary graft non-function (7), sepsis (10), intracranial hemorrhage (1), tumors (4), recurrent hepatitis B (2), biliary strictures (2) and chronic rejection (1). The 1- and 4-year survival rates were 79.5% and 69.6%, respectively. After transplantation, 10 developed biliary stricture (5 corrected by balloon dilatation) and 8 anastomotic stricture (7 corrected by surgery), and there were 2 multiple intrahepatic strictures. There was hepatic artery thrombosis in 5, including 4 children. In 3, grafts were salvaged by thrombectomy and 2 others underwent re-transplantation. In those who survived transplantation by more than 1-month, recurrent hepatitis B was seen in 6 of 17 (35%) and recurrent hepatitis C in 12 of 19 (63%). Thus, results of our first 100 liver transplants are similar to those reported by larger centers, showing that in an appropriate setting good results can be achieved by small transplant programs.
Asunto(s)
Trasplante de Hígado/estadística & datos numéricos , Adolescente , Adulto , Niño , Femenino , Estudios de Seguimiento , Humanos , Israel , Trasplante de Hígado/mortalidad , Trasplante de Hígado/fisiología , Masculino , Complicaciones Posoperatorias/clasificación , Complicaciones Posoperatorias/epidemiología , Estudios Retrospectivos , Tasa de Supervivencia , Factores de TiempoRESUMEN
With limited organ resources and an increasing number of candidates for liver transplantation, the world-wide trend is towards using liver allografts from donors older than 60 years. This strategy, however, may be hazardous because of the known correlation between advanced donor age and graft dysfunction. Since January 1996, each of 5 patients received a liver allograft from a donor older than 60 years. Preservation time in these cases was shortened as much as possible and liver allografts were used only if there were no other potential risk factors for primary nonfunction. Mean cold ischemic time was significantly shorter in this donor group (7.8 hrs) than for livers from 28 younger donors (10.2 hour; p < 0.01). 3 of the 5 grafts from older donors had normal function immediately. The other 2 initially had biochemical features of preservation injury, but graft function returned to normal within the first week after transplantation. All 5 patients currently have normal graft function, with follow-up ranging from 3-8 months. There was no difference between the 5 recipients of grafts from older donors and 28 adult recipients of grafts from younger donors in extent of preservation injury and in immediate graft function. We conclude that in countries with limited organ resources, such as Israel, liver allografts from older donors can be used within defined limits and minimal preservation time.
Asunto(s)
Trasplante de Hígado , Adulto , Factores de Edad , Estudios de Seguimiento , Humanos , Trasplante de Hígado/fisiología , Persona de Mediana Edad , Preservación de Órganos , Donantes de Tejidos , Trasplante Homólogo , Resultado del TratamientoRESUMEN
Our experience with living-related liver transplantation is described. In 2 boys and 1 girl, aged 4-4.5 years with acute, fulminating hepatitis A, the presence of very severe jaundice (bilirubin levels > 18 mg%) associated with severe coagulopathy (INR > 10) and encephalopathy indicated the need for urgent liver transplantation. In all 3 cases the left lateral hepatic segment of a matched blood type parent was transplanted. None of the donors suffered a serious complication postoperatively and all returned to full activity in 6-16 weeks. The post-transplantation course was uneventful in 1 child, but in the other 2 there was hepatic arterial thrombosis in 1 at 1 day and in the other at 8 days post-transplantation. Early detection of arterial thrombosis by Doppler sonography permitted salvage of the 2 hepatic grafts after thrombectomy and re-anastomosis. In 1 of these 2 children an anastomotic biliary stricture was found 2 months after transplantation. It was corrected at surgery and a percutaneous stent was inserted. All 3 children are alive with normal graft function at 2, 7 and 8 months post-transplantation, respectively. This initial experience indicates that living-related liver transplantation is feasible in Israel. The technique might help to solve our severe organ shortage for children awaiting liver transplantation.