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1.
Children (Basel) ; 11(2)2024 Feb 03.
Artículo en Inglés | MEDLINE | ID: mdl-38397305

RESUMEN

The aim of this study was to assess the long-term results of liver transplantation (LT) in pediatric patients with unresectable hepatoblastoma (HB) or hepatocellular carcinoma (HCC) with special reference to the risk of tumor recurrence. We retrospectively analyzed data from 46 HB and 26 HCC patients who underwent LT between 1990 and 2022. In HCC patients, we compared outcomes depending on donor type. We evaluated the impact of a number of risk factors on recurrence-free survival after LT. Estimated patient survival after 5, 10, and 15 years was 82%, 73%, and 73% in the HB group and 79%, 75%, and 75% in the HCC group, respectively (p = 0.76). In the HCC group, living donor LT (LDLT) and deceased donor LT (DDLT) provided similar patient survival (p = 0.09). Estimated recurrence-free survival in patients who had three or fewer risk factors was significantly better than in patients with more than three risk factors (p = 0.0001). Adequate patient selection is necessary when considering LT for primary liver tumors in children. The presence of more than three risk factors is associated with a very high risk of recurrence and indicates poor prognosis, whereas extrahepatic disease may be considered a contraindication for transplantation.

2.
Children (Basel) ; 8(8)2021 Jul 29.
Artículo en Inglés | MEDLINE | ID: mdl-34438552

RESUMEN

(1) Background: Post-transplant lymphoproliferative disease (PTLD) is a significant complication of solid organ transplantation (SOT). However, there is lack of consensus in PTLD management. Our aim was to establish a present benchmark for comparison between international centers and between various organ transplant systems and modalities; (2) Methods: A cross-sectional questionnaire of relevant PTLD practices in pediatric transplantation was sent to multidisciplinary teams from 17 European center members of ERN TransplantChild to evaluate the centers' approach strategies for diagnosis and treatment and how current practices impact a cross-sectional series of PTLD cases; (3) Results: A total of 34 SOT programs from 13 European centers participated. The decision to start preemptive treatment and its guidance was based on both EBV viremia monitoring plus additional laboratory methods and clinical assessment (61%). Among treatment modalities the most common initial practice at diagnosis was to reduce the immunosuppression (61%). A total of 126 PTLD cases were reported during the period 2012-2016. According to their histopathological classification, monomorphic lesions were the most frequent (46%). Graft rejection after PTLD remission was 33%. Of the total cases diagnosed with PTLD, 88% survived; (4) Conclusions: There is still no consensus on prevention and treatment of PTLD, which implies the need to generate evidence. This might successively allow the development of clinical guidelines.

3.
Am J Case Rep ; 22: e932374, 2021 Jul 20.
Artículo en Inglés | MEDLINE | ID: mdl-34283821

RESUMEN

BACKGROUND Current treatment options for progressive intrahepatic familial cholestasis type 1 (PFIC-1) comprise ursodeoxycholic acid (UDCA), partial external biliary diversion (PEBD), and liver transplantation (LTx). The role and timing of LTx in PFIC-1 remains debated. We present 2 case reports of male siblings with PFIC-1 who benefited from different treatments. CASE REPORT Both siblings harbored a homozygous truncating mutation in ATP8B1 characteristic for PFIC-1 and both underwent PEBD after unsuccessful UDCA treatment at the age of 7 and 5 months, respectively. The older brother, after initial improvement of symptoms, developed severe pruritus, cholestasis, and diarrhea 9 months after PEBD and underwent LTx at the age of 16 months. Chronic diarrhea and abnormal transaminases activity appeared soon after transplantation. A liver biopsy was performed 3 months after LTx and showed severe macrovesicular steatosis (95%). Sixteen months after LTx, total biliary diversion was performed, with rapid relief from diarrhea and significant regression of graft steatosis by <30%. In his brother we observed persistent severe pruritus and cholestasis after PEBD, but we decided to postpone LTx due to lack of a living related donor and risk of graft steatosis. Eight months after PEBD, bilirubin and bile acids significantly decreased and pruritus disappeared completely. Currently, in 5-year follow-up, liver function is stable and he has no pruritus. CONCLUSIONS The good effect of PEBD may be delayed in PFIC-1, even in severe mutation; thus, the decision to perform LTx should be made cautiously. Total biliary diversion is an efficient procedure in case of persistent symptoms after LTx and can reverse graft steatosis in children with PFIC-1.


Asunto(s)
Colestasis Intrahepática , Colestasis , Trasplante de Hígado , Adenosina Trifosfatasas , Niño , Colestasis Intrahepática/genética , Colestasis Intrahepática/cirugía , Humanos , Lactante , Masculino , Mutación , Hermanos , Resultado del Tratamiento
4.
Ann Transplant ; 26: e926217, 2021 Feb 12.
Artículo en Inglés | MEDLINE | ID: mdl-33574216

RESUMEN

BACKGROUND In this report, we present technical problems and solutions used in the reconstruction of the inferior vena cava and graft venous outflow during living donor liver transplantation (LDLT) in children. MATERIAL AND METHODS In 65 grafts out of 379 liver transplantations from living donors, reconstruction of multiple hepatic venous branches and/or IVC was necessary. In 4 cases, cryopreserved deceased donor venous grafts were used for the reconstruction of the IVC and/or HV. RESULTS Follow-up ranged from 2 months to 17.8 years (median 7.2 years). In 4 children, liver re-transplantation was required for a reason not related to venous outflow (biliary complications in 3 patients, graft insufficiency caused by small-for-size syndrome). Two patients died: 1 due to tumor recurrence and 1 due to multi-organ failure. Fifty-nine patients are alive with good liver function. One patient (1.5%) after deceased donor venous graft reconstruction showed symptoms of venous outflow obstruction, which was successfully treated with endovascular balloon angioplasty and stent placement. The remaining 59 transplanted patients do not show any signs of venous outflow obstruction. CONCLUSIONS In most cases, the reconstruction of multiple hepatic veins of living donor allografts can successfully be done with local venoplasty, while using cold-stored vein grafts may be helpful in selected cases of LDLT.


Asunto(s)
Venas Hepáticas , Trasplante de Hígado , Donadores Vivos , Vena Cava Inferior , Niño , Venas Hepáticas/cirugía , Humanos , Estudios Retrospectivos , Vena Cava Inferior/cirugía
5.
Ann Transplant ; 24: 25-29, 2019 Jan 11.
Artículo en Inglés | MEDLINE | ID: mdl-30631030

RESUMEN

BACKGROUND Diffuse thrombosis of iliac veins and IVC has been considered a significant technical obstacle in pediatric kidney transplantation (KT). MATERIAL AND METHODS Between 1984 and 2018, 951 KTs were performed in our institution. In 4 children qualified for KT, diffuse thrombosis of iliac veins or IVC was found. The surgical techniques, complications, patient and graft survival, and long-term renal function were studied retrospectively. The patients' age at transplantation was 2.5-13 years and body mass was 11-39 kg. RESULTS All children were transplanted with venous anastomoses made to infrahepatic IVC (3 patients) or collateral circulation (1 patient). Early complications developed in 2 patients: significant bleeding from the graft area requiring revision on the second day after transplantation and chyle leak that resolved spontaneously. The follow-up period was 1-12.5 years. Three patients are alive with a follow-up at 7 months, 4.5, and 12 years with serum creatinine 0.7 mg%, 0.6 mg% and 1.4 mg%, respectively. One patient died 1 year after KT, with normal graft function. No late complications related to KT were observed in any patient. CONCLUSIONS Renal transplantation in pediatric patients with thrombotic vascular complications is associated with a number of technical difficulties and problems.


Asunto(s)
Trasplante de Riñón/métodos , Vena Cava Inferior/anomalías , Adolescente , Niño , Preescolar , Femenino , Supervivencia de Injerto , Humanos , Masculino , Estudios Retrospectivos , Vena Cava Inferior/cirugía
6.
PLoS One ; 13(2): e0193327, 2018.
Artículo en Inglés | MEDLINE | ID: mdl-29474400

RESUMEN

OBJECTIVES: Living donor liver transplantation (LDLT) in patients with acute liver failure (ALF) has become an acceptable alternative to transplantation from deceased donors (DDLT). The aim of this study was to analyze outcomes of LDLT in pediatric patients with ALF based on our center's experience. MATERIAL AND METHODS: We enrolled 63 children (at our institution) with ALF who underwent liver transplantation between 1997 and 2016. Among them 24 (38%) underwent a LDLT and 39 (62%) received a DDLT. Retrospectively analyzed patient clinical data included: time lapse between qualification for transplantation and transplant surgery, graft characteristics, postoperative complications, long-term results post-transplantation, and living donor morbidity. Overall, we have made a comparison of clinical results between LDLT and DDLT groups. RESULTS: Follow-up periods ranged from 12 to 182 months (median 109 months) for LDLT patients and 12 to 183 months (median 72 months) for DDLT patients. The median waiting time for a transplant was shorter in LDLT group than in DDLT group. There was not a single case of primary non-function (PNF) in the LDLT group and 20 out of 24 patients (83.3%) had good early graft function; 3 patients (12.5%) in the LDLT group died within 2 months of transplantation but there was no late mortality. In comparison, 4 out of 39 patients (10.2%) had PNF in DDLT group while 20 patients (51.2%) had good early graft function; 8 patients (20.5%) died early within 2 months and 2 patients (5.1%) died late after transplantation. The LDLT group had a shorter cold ischemia time (CIT) of 4 hours in comparison to 9.2 hours in the DDLT group (p<0.0001). CONCLUSIONS: LDLT is a lifesaving procedure for pediatric patients with ALF. Our experience showed that it may be performed with very good results, and with very low morbidity and no mortality among living donors when performed by experienced teams following strict procedures.


Asunto(s)
Fallo Hepático Agudo/cirugía , Trasplante de Hígado , Donadores Vivos , Adolescente , Adulto , Niño , Preescolar , Femenino , Estudios de Seguimiento , Supervivencia de Injerto , Humanos , Hipotermia Inducida , Lactante , Estimación de Kaplan-Meier , Fallo Hepático Agudo/mortalidad , Masculino , Persona de Mediana Edad , Complicaciones Posoperatorias , Retratamiento , Estudios Retrospectivos , Tiempo de Tratamiento , Resultado del Tratamiento , Adulto Joven
7.
Ann Transplant ; 22: 555-562, 2017 Sep 15.
Artículo en Inglés | MEDLINE | ID: mdl-28912414

RESUMEN

BACKGROUND Liver transplantation (LT) is recommended for various metabolic diseases, including urea cycle disorders (UCDs). The aim of this study was to determine indications and outcomes of LT for UCDs in the tertiary reference Children's Hospital in Warsaw, Poland. MATERIAL AND METHODS Medical charts of children with UCD who underwent LT between 2008 and July 2016 were retrospectively reviewed. The following parameters were analyzed: symptoms at time of diagnosis, age at diagnosis, age at transplantation, graft characteristics and survival, postsurgical complications, and biochemical and laboratory results before and after transplantation. RESULTS Twelve patients with UCD who underwent LT at a mean age of 5 y (0.5-14 y) received a total of 14 liver grafts. Four children (33%) received a living donor graft, while 8 (68%) got a deceased donor liver graft. A total number of transplanted organs consisted of 9 (64%) whole-liver grafts and 5 (36%) reduced-size grafts. The 30-day post-transplant patient survival rate was 100% and graft survival rate was 93% (13/14). For those with a post-transplant follow-up of at least 1 year (n=10/12), the 1-year patient survival rate was 100% and the graft survival rate was 85.7% (12/14). Median peak of blood ammonia at presentation was 653 (159-2613) µg/dL (normal <80 µg/dl), and median peak of blood glutamine was 1273.2 µmol/l (964-3900 µmol/l). There was 1 episode of hyperammonemia following LT, but it was not due to UCD. Six (50%) patients were diagnosed with some degree of developmental delay/neurological impairment before transplantation, which remained stable or slightly improved after transplantation. Patients without developmental delay before transplantation maintained their cognitive abilities at follow-up. CONCLUSIONS LT leads to eradication of hyperammonemia, withdrawal of dietary restrictions with low-protein diet, and potentially improved neurocognitive development.


Asunto(s)
Supervivencia de Injerto/fisiología , Trasplante de Hígado/métodos , Trastornos Innatos del Ciclo de la Urea/cirugía , Adolescente , Factores de Edad , Niño , Preescolar , Femenino , Humanos , Lactante , Trasplante de Hígado/mortalidad , Masculino , Polonia , Estudios Retrospectivos , Tasa de Supervivencia , Resultado del Tratamiento
8.
Ann Transplant ; 22: 257-264, 2017 Apr 28.
Artículo en Inglés | MEDLINE | ID: mdl-28450694

RESUMEN

BACKGROUND The diagnosis of post-liver transplant biliary strictures (BS) requires a high degree of clinical suspicion because of the diversity of symptoms and usually mild clinical presentation. If quickly treated, successful outcome is achieved most cases. The aim of our study was to analyze the value of diagnostic methods in BS after pediatric LTx. MATERIAL AND METHODS We retrospectively reviewed clinical data of children with BS after liver transplantation, with the main focus on diagnostic methods, including imaging studies and histology. All patients underwent endoscopic, transhepatic, or surgical treatment of the stricture. RESULTS Sixty-seven patients after LTx performed at the median age of 9.1 years (0.4-18) developed BS, mostly within the first 12 months (40%). Laboratory findings at diagnosis were: bilirubin 4.65 (5.8±SD), GGTP 434 (382.9±SD), and ALT 126.5 (116.8±SD); 16 patients presented with bilirubin level <1 mg% and 4 with GGTP below 100 IU. Ultrasound scan (USS) visualized dilatation of the bile ducts in 53 (79%) patients. Overall sensitivity of hepatobiliary scintigraphy (HBS) was 93%, with dilatation of bile ducts in 69% and impaired excretion in 68% of patients. MRCP showed 100% accuracy in detecting biliary dilatation and a stricture was visualized in 39%. Liver histology was consistent with biliary obstruction in 66%. Treatment of BS was successful in the majority of cases, with 89% graft survival. CONCLUSIONS Non-invasive investigations are highly sensitive in post-transplant BS and should play the key role in diagnostic algorithms.


Asunto(s)
Enfermedades de las Vías Biliares/diagnóstico , Pancreatocolangiografía por Resonancia Magnética , Trasplante de Hígado/efectos adversos , Ultrasonografía Doppler en Color , Adolescente , Enfermedades de las Vías Biliares/etiología , Niño , Preescolar , Constricción Patológica/diagnóstico , Constricción Patológica/etiología , Femenino , Humanos , Lactante , Masculino , Complicaciones Posoperatorias/diagnóstico , Estudios Retrospectivos , Adulto Joven
9.
Clin Res Hepatol Gastroenterol ; 40(4): e33-7, 2016 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-26850362

RESUMEN

Neuroendocrine tumors (NET) are extremely rare in children (0.75 cases per 100,000 children and adolescents a year) and the majority of these tumors are benign or present low grade of malignancy. According to the American registry Surveillance, Epidemiology, and End Results (SEER) Program of the National Cancer Institute, less than 2% of all neuroendocrine tumors in children occur in the pancreas, making it a rare site for these tumors. The majority of them are found in children over 10years of age, especially those with malignant potential. Treatment of NET consists of different methods: surgery, somatostatin analogues and chemotherapy. Radical surgical resection remains the standard of treatment; however, it is not always feasible because of distant metastases. The authors present a case report of pancreatic NET with multiple metastases to the liver. The patient was treated with pancreatic resection and liver transplantation for liver metastases. Prior to liver transplantation, the patient was treated with somatostatin analogues, sunitinib and chemotherapy. Management of liver metastases with liver transplantation is discussed.


Asunto(s)
Neoplasias Hepáticas/secundario , Neoplasias Hepáticas/cirugía , Trasplante de Hígado , Tumores Neuroendocrinos/cirugía , Neoplasias Pancreáticas/patología , Adolescente , Femenino , Humanos , Metástasis Linfática , Tumores Neuroendocrinos/patología , Tumores Neuroendocrinos/secundario , Neoplasias Pancreáticas/cirugía
10.
Ann Transplant ; 19: 283-7, 2014 Jun 10.
Artículo en Inglés | MEDLINE | ID: mdl-24916138

RESUMEN

BACKGROUND: Cavo-portal transposition (CPT) at liver transplantation (LTx) allows portal revascularization of the liver in recipients in whom portal system thrombosis does not allow performance of porto-portal anastomosis. The aim was to present the cases of 2 children who underwent LTx and CPT in our institution. CASE REPORT: 1. A 10-year-old boy, after Kasai procedure and living donor LTx, was qualified for retransplantation 9 years after first LTx complicated with late portal vein thrombosis, portal hypertension, hypersplenism, and multiple GI bleeding episodes, after splenectomy and meso-caval shunt preventing GI bleeding. At retransplant surgery, CPT was done. Actual follow-up was 40 months. Doppler ultrasound and angio CT show normal flow within the graft's portal vein. Biochemical parameters were within normal range. There was no bleeding from the gastrointestinal tract. 2. A 14-month-old child after Kasai procedure was qualified for living donor liver transplantation. During surgery, thrombosis of the recipient portal system was found, which was not diagnosed before. The CPT was done. There were no complications during the postoperative course. The actual follow-up was 32 months, and the patient is doing well, with normal liver and renal function, without hypersplenism or ascites. There was no gastrointestinal bleeding. Doppler ultrasound showed normal intrahepatic portal and arterial flow in the transplanted liver. CONCLUSIONS: Cavo-portal transposition is an important option in portal vein revascularization in liver transplant recipients without access to the portal system. Long-term observation of these 2 cases did not show any late problems (e.g., bleeding from the gastrointestinal tract, renal function, hyperammonemia, ascites) related to cavo-portal transposition.


Asunto(s)
Arteria Hepática/cirugía , Venas Hepáticas/cirugía , Trasplante de Hígado/métodos , Vena Porta/cirugía , Vena Cava Inferior/cirugía , Trombosis de la Vena/cirugía , Niño , Femenino , Rechazo de Injerto/cirugía , Humanos , Hipertensión Portal/cirugía , Lactante , Circulación Hepática , Trasplante de Hígado/efectos adversos , Masculino , Reoperación
11.
J Pediatr Surg ; 48(11): 2202-6, 2013 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-24210186

RESUMEN

BACKGROUND: Undifferentiated embryonal sarcoma of the liver (UESL) represents less than 5% of all malignant hepatic tumors in childhood. It is considered an aggressive neoplasm with an unfavorable prognosis. The aim of this paper is to present a single center experience in the treatment of children with UESL. MATERIALS AND METHODS: Ten children with UESL were treated between 1981 and 2012. Age at diagnosis ranged from 4 months to 17 years (median age, 6 years and 9 months). Surgery after neoadjuvant chemotherapy (CHT) was performed in 7 patients, and in 3 patients primary surgery was done. Adjuvant chemotherapy was administered in all 10 patients (CYVADIC, CAV, CAV/ETIF/IF+ADM, CDDP/PLADO). Right hemihepatectomy was performed in 1 patient, extended right hemihepatectomy in 6, and partial resection of the right lobe (segments V-VI, segment V) in 2 patients. One patient with unresectable tumor affecting both lobes was listed for liver transplantation (LTx). RESULTS: Follow-up from diagnosis ranged from 50 to 222 months (mean 138 months). Among 9 patients treated with partial liver resection, distant metastases/local recurrence was not observed in any, and disease-free survival in this group is 100% (9 patients alive). The patient that underwent liver transplantation died of multiorgan failure 4 months postoperatively. However, this patient was misdiagnosed as having hepatoblastoma (HBL) and received PLADO chemotherapy. The overall survival rate is 90%. CONCLUSION: Excellent results with long-term survival can be achieved in children with UESL with conventional therapy, including a combination of neoadjuvant and adjuvant chemotherapy and surgery, even in large extensively growing tumors.


Asunto(s)
Hepatectomía , Neoplasias Hepáticas/cirugía , Neoplasias de Células Germinales y Embrionarias/cirugía , Sarcoma/cirugía , Adolescente , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Carcinoma Hepatocelular/diagnóstico , Niño , Preescolar , Terapia Combinada , Errores Diagnósticos , Supervivencia sin Enfermedad , Femenino , Hepatectomía/métodos , Humanos , Lactante , Neoplasias Hepáticas/diagnóstico , Neoplasias Hepáticas/tratamiento farmacológico , Neoplasias Hepáticas/mortalidad , Trasplante de Hígado , Masculino , Neoplasias de Células Germinales y Embrionarias/diagnóstico , Neoplasias de Células Germinales y Embrionarias/tratamiento farmacológico , Neoplasias de Células Germinales y Embrionarias/mortalidad , Inducción de Remisión , Estudios Retrospectivos , Sarcoma/diagnóstico , Sarcoma/tratamiento farmacológico , Sarcoma/mortalidad , Resultado del Tratamiento
12.
J Pediatr Surg ; 47(7): 1331-9, 2012 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-22813792

RESUMEN

BACKGROUND/PURPOSE: The aim of the study was to analyze changing management and survival of children with hepatoblastoma (HBL) treated in one center. MATERIALS AND METHODS: Over the last 20 years, 51 children with HBL were treated. Surgery was performed in 48 children (94.1%), conventional liver resection in 38 (of those, 2 received a rescue liver transplantation [LTx] for relapse), and total hepatectomy and primary LTx in 10 patients. The remaining 3 patients received only palliative treatment. Patient data were analyzed for survival with respect to PRETreatment EXTent of disease (PRETEXT), metastases, histopathology, conventional resection, and LTx. RESULTS: Survival of children with HBL treated with liver resection is 71% and 80% for primary LTx. Favorable prognostic factors for patient survival was tumor histology as epithelial-fetal subtype and mixed epithelial and mesenchymal type, without teratoid features, and good response to chemotherapy (necrosis, fibrosis). Unfavorable prognostic factors were small cells undifferentiated, transitional liver cell tumor, α-fetoprotein level above 1,000,000 IU/mL and below 100 IU/mL at diagnosis, lung metastases, and local recurrence after initial resection. Survival was related to PRETEXT stage. However, among patients with PRETEXT III and IV, LTx resulted in better survival. CONCLUSION: Liver transplantation is a good option for children with advanced HBL. Early referral of children with potentially unresectable tumors to centers where combined treatment (chemotherapy, surgery including LTx) is available is crucial.


Asunto(s)
Hepatectomía/tendencias , Hepatoblastoma/cirugía , Neoplasias Hepáticas/cirugía , Trasplante de Hígado/tendencias , Adolescente , Antineoplásicos/uso terapéutico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Quimioterapia Adyuvante , Niño , Preescolar , Cisplatino/uso terapéutico , Doxorrubicina/uso terapéutico , Femenino , Estudios de Seguimiento , Hepatectomía/métodos , Hepatoblastoma/tratamiento farmacológico , Hepatoblastoma/mortalidad , Humanos , Lactante , Neoplasias Hepáticas/tratamiento farmacológico , Neoplasias Hepáticas/mortalidad , Trasplante de Hígado/métodos , Masculino , Terapia Neoadyuvante , Cuidados Paliativos , Análisis de Supervivencia , Resultado del Tratamiento
13.
Ann Transplant ; 17(1): 128-31, 2012.
Artículo en Inglés | MEDLINE | ID: mdl-22466919

RESUMEN

BACKGROUND: Amoxicillin/clavulanic acid-induced liver injury is a well recognized complication. Presentation and outcome may vary, which is related to individual liver maturity, genetic predisposition, enzyme heterogeneity, intensity of treatment, and drug interactions. In most cases withdrawing the drug is sufficient treatment; however, cases of progressive liver damage leading to liver transplantation have been reported. CASE REPORT: We present the case of an 8-year-old patient after liver transplantation who developed drug induced liver injury (DILI) after amoxicillin/clavulanic acid treatment for upper respiratory tract infection. Jaundice appeared 2 days after cessation of treatment. Clinical presentation and liver biopsy were consistent with DILI. Because of rapidly increasing bilirubin levels, we used 3 boluses of methylprednisolone and ursodeoxycholic acid. The treatment reversed progression of the cholestasis and full recovery was achieved in 3 months. CONCLUSIONS: In most cases of DILI, withdrawing the toxic drug is sufficient treatment, but we must be aware of a possible fatal outcome in case of progressive cholestasis. Corticosteroids may have beneficial effects in these patients.


Asunto(s)
Combinación Amoxicilina-Clavulanato de Potasio/efectos adversos , Antibacterianos/efectos adversos , Enfermedad Hepática Inducida por Sustancias y Drogas/tratamiento farmacológico , Enfermedad Hepática Inducida por Sustancias y Drogas/etiología , Colestasis Intrahepática/inducido químicamente , Colestasis Intrahepática/tratamiento farmacológico , Trasplante de Hígado/efectos adversos , Corticoesteroides/administración & dosificación , Enfermedad Hepática Inducida por Sustancias y Drogas/patología , Niño , Colagogos y Coleréticos/administración & dosificación , Colestasis Intrahepática/patología , Humanos , Masculino , Metilprednisolona/administración & dosificación , Infecciones del Sistema Respiratorio/tratamiento farmacológico , Ácido Ursodesoxicólico/administración & dosificación
14.
Ann Transplant ; 16(1): 14-7, 2011.
Artículo en Inglés | MEDLINE | ID: mdl-21436769

RESUMEN

BACKGROUND: Children with biliary atresia and polysplenia syndrome (BA-PS) have always been considered as high risk liver transplant recipients due to technical problems during transplant surgery. We report single-center experience with liver transplantation in children with this syndrome. MATERIAL/METHODS: Between 2000 and 2010, 401 liver transplantations were performed in 358 children, including 6 patients with BA-PS, who underwent living (5 patients) or deceased (1 patient) donor liver transplantation. Patients demonstrated various malformations: absence of retrohepatic vena cava (3), intestinal malrotation (3), preduodenal portal vein (1), hepatic artery anomalies (3), cardiac anomalies (2), and situs inversus (1). Transplantations were performed at the patient age of 8 months to 11 years. RESULTS: There were no serious technical problems during the operations, and we did not have to use vascular conduits for graft revascularization in any case. All patents were alive at follow-up between 14 and 123 months after transplantation (mean 75 months). We observed, however, increased incidence of PV thrombosis and biliary complications in these patients, which did not influence patient and graft survival. In 1 child with graft failure due to chronic rejection after discontinuation of immunosuppression due to PTLD, retransplantation was performed. CONCLUSIONS: Results of liver transplantation in children with BA-PS are as good as for other indications and non-syndromic BA in an experienced pediatric liver transplant center. Although there were no serious technical problems during deceased or living related donor transplantation in these children, close observation for possible vascular complications should be the routine in the postoperative period.


Asunto(s)
Atresia Biliar/cirugía , Trasplante de Hígado , Bazo/anomalías , Bazo/cirugía , Anomalías Múltiples/cirugía , Niño , Femenino , Humanos , Lactante , Hígado/anomalías , Hígado/cirugía , Trasplante de Hígado/efectos adversos , Masculino , Vena Porta , Reoperación , Situs Inversus/cirugía , Síndrome , Trombosis de la Vena/etiología
15.
Pediatr Transplant ; 13(7): 906-12, 2009 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-18992048

RESUMEN

The aim of this study was to present acute hemodynamic failure as a rare indication for liver transplantation in neonates and infants with liver hemangiomatosis. We report four patients aged one to six months with giant liver hemangiomas, with huge arterio-venous shunting within these malformations. In three, many skin hemangiomas were found. All children developed right ventricular failure. In two, a trial of pharmacological reduction was attempted with corticosteroids and cyclophosphamide. In one patient, the arterio-venous fistulas were embolized without any improvement in hemodynamic status. Two children underwent rescue hepatic artery surgical ligation, which did not prevent heart and then multiorgan failure including liver failure. After unsuccessful conventional therapy, all infants were considered for urgent liver transplantation; in three cases, it was performed with a living-related donor, and in one case with a deceased donor. All patients are alive and well with the follow-up between nine and 37 months after transplantation. Liver transplantation should be considered as a rescue treatment in children with hepatic vascular malformations leading to hemodynamic insufficiency when conventional therapy is unsuccessful and multiorgan failure develops.


Asunto(s)
Hemangioma/cirugía , Hepatopatías/terapia , Trasplante de Hígado/métodos , Malformaciones Vasculares/cirugía , Fístula Arteriovenosa/patología , Femenino , Ventrículos Cardíacos/fisiopatología , Hemangioma/terapia , Hemodinámica , Humanos , Lactante , Recién Nacido , Hepatopatías/cirugía , Donadores Vivos , Masculino , Tomografía Computarizada por Rayos X/métodos , Resultado del Tratamiento , Malformaciones Vasculares/terapia
16.
Ann Transplant ; 13(2): 37-41, 2008.
Artículo en Inglés | MEDLINE | ID: mdl-18566558

RESUMEN

BACKGROUND: There is a group of children with primary hepatic tumors which can not be resected by conventional partial liver resection. Total hepatectomy followed by liver transplantation may be the only solution in such cases. Authors reviewed own experience with the liver transplantation for unresectable tumors in children and assessed the possible indications and role of transplantation in these patients. MATERIAL AND METHODS: Liver transplantation was performed in 17 children with unresectable hepatic tumors out of total number of 350 children transplanted. Hepatocarcinoma was present in 8 children, hepatoblastoma in 6 and benign giant hemangioma in 3. There was no other option for the treatment which would lead to the oncological cure of children with malignant tumors. All patients with giant hemangiomas were infants transplanted urgently due to circulatory and then multiorgan failure. RESULTS: Survival within whole group is 75.5% (13 of 17 pts), 3 children died of malignant tumor recurrence, one of other causes. All 3 children with benign tumors are alive and well. Actual follow-up is from 3 months to 7 years. CONCLUSIONS: Liver transplantation should be considered as option in the treatment of all children with unresectable hepatic tumors. With the careful and individual patient selection significant chances for survival can be achieved in this group of patients which would otherwise not survive with the conventional treatment.


Asunto(s)
Carcinoma Hepatocelular/cirugía , Hemangioma/cirugía , Hepatectomía , Hepatoblastoma/cirugía , Neoplasias Hepáticas/cirugía , Trasplante de Hígado , Adolescente , Carcinoma Hepatocelular/diagnóstico , Carcinoma Hepatocelular/mortalidad , Niño , Preescolar , Estudios de Cohortes , Hemangioma/diagnóstico , Hemangioma/mortalidad , Hepatoblastoma/diagnóstico , Hepatoblastoma/mortalidad , Humanos , Lactante , Estudios Retrospectivos , Tasa de Supervivencia
17.
Pediatr Transplant ; 11(8): 868-75, 2007 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-17976121

RESUMEN

Aspergillus infection in immunocompromised patients is associated with high morbidity and mortality. We retrospectively reviewed cases of Aspergillosis (A), in a series of 277 children who received LTx between 1990 and 2006. All children were given antifungal prophylaxis after transplantation. Aspergillosis was identified in 10 cases (3.6%) and diagnosis was confirmed when clinical symptoms were associated with identification of Aspergillus sp. or detection of galactomannan antigen. Incidence of Aspergillosis considerably decreased from 6.9% to 0.6% when liposomal amphotericin B was introduced as prophylaxis in high-risk patients. Mean time since LTx to Aspergillosis was 14.5 days. Histologically, Aspergillosis was diagnosed in two cases. Galactomannan antigen was present in two recipients. Aspergillus infection occurs usually within first 30 days after transplantation as a result of a combination of several risk factors. Following risk factors were observed: multiple antibiotic therapy, prolonged intensive care unit stay, poor graft function, retransplantation, relaparotomies, co-infection. Amphotericin B was administered in all cases. Two patients (20%) died because of Aspergillosis Liposomal Amphotericin B prophylaxis in high-risk children decreases the incidence of Aspergillus infection. High index of suspicion and early diagnosis followed by intensive treatment with amphotericin B facilitates achieving mortality rate lower than presented in other reports.


Asunto(s)
Aspergilosis/epidemiología , Rechazo de Injerto/complicaciones , Trasplante de Hígado/efectos adversos , Hígado/microbiología , Adolescente , Adulto , Antígenos Fúngicos/análisis , Aspergilosis/diagnóstico , Aspergilosis/etiología , Aspergillus/inmunología , Aspergillus/aislamiento & purificación , Niño , Preescolar , Femenino , Estudios de Seguimiento , Rechazo de Injerto/diagnóstico , Rechazo de Injerto/prevención & control , Humanos , Inmunosupresores/efectos adversos , Inmunosupresores/uso terapéutico , Incidencia , Lactante , Hígado/patología , Masculino , Polonia/epidemiología , Estudios Retrospectivos , Factores de Riesgo
18.
Ann Transplant ; 10(2): 53-8, 2005.
Artículo en Inglés | MEDLINE | ID: mdl-16218034

RESUMEN

OBJECTIVES: Study was performed to evaluate prospectively long-term renal function in pediatric liver transplant recipients. METHODS: In 50 children aged 1-18 years, renal function tests (sCr, GFR, ERPF, Cr clearance, renal Doppler ultrasound, ABPM) have been performed before and several times during 36 months follow-up after liver transplantation. RESULTS: Signifficant reduction of renal filtration function was found in studied children (increase of sCr, decrease of GFR, ERPF, Cr clearance) within 12 months after transplantation, which did not progress further in most children after this time. None of patients progressed to stage 4 or 5 renal failure according to CKD. Schwartz formula was not found reliable in assessing renal function in children after liver transplantation. There was no difference in renal function according to cyclosporine or tacrolimus treatment, however arterial hypertension was more common in cyclosporine treated group. CONCLUSIONS: Although renal damage may be a problem for an individual chuild after liver transplantation, majority of children have stable renal function 36 months after Tx. DTPA GFR should be performed yearly in all children until adulthood not to overlook progression of renal injury.


Asunto(s)
Riñón/fisiopatología , Trasplante de Hígado , Adolescente , Presión Sanguínea , Niño , Preescolar , Femenino , Tasa de Filtración Glomerular , Hemodinámica , Humanos , Inmunosupresores/uso terapéutico , Lactante , Fallo Renal Crónico/prevención & control , Pruebas de Función Renal , Estudios Longitudinales , Masculino , Estudios Prospectivos , Circulación Renal , Medición de Riesgo
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