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1.
J. pediatr. (Rio J.) ; 90(2): 169-175, Mar-Apr/2014. tab, graf
Article in English | LILACS | ID: lil-709811

ABSTRACT

OBJECTIVE: to evaluate the frequency and factors associated with vascular complications after pediatric liver transplantation. METHOD: risk factors were evaluated in 99 patients under 18 years of age with chronic liver disease who underwent deceased donor liver transplantation (DDLT) between March of 1995 and November of 2009 at the Hospital de Clínicas de Porto Alegre, Brazil. The variables analyzed included donor and recipient age, gender, and weight; indication for transplant; PELD/MELD scores; technical aspects; postoperative vascular complications; and survival. RESULTS: vascular complications occurred in 19 patients (19%). Arterial events were most common, occurred earlier in the postoperative period, and were associated with high graft loss and mortality rates. In the multivariate analysis, the following factors were identified: portal vein diameter < 3 mm, donor-to-recipient body weight ratio (DRWR), prolonged ischemic time, and use of arterial grafts. CONCLUSION: the choice of treatment depends on the timing of diagnosis; however, in this study, surgical revision or correction produced worse outcomes than percutaneous angioplasty. The reduction of risk factors and early detection of vascular complications are key elements to a successful transplantation. .


OBJETIVO: avaliar a frequência e os fatores associados a complicações vasculares após transplante hepático pediátrico. MÉTODO: os fatores de risco foram avaliados em 99 pacientes com mais de 18 anos de idade comdoença hepática crônica submetidos a transplante hepático cadavérico (THC) entre marc¸o de1995 e novembro de 2009 no Hospital de Clínicas de Porto Alegre, Brasil. As variáveis analisadasincluíram: idade, sexo e peso dos doadores e receptores; indicac¸ão de transplante; escores PELD/MELD; aspectos técnicos; complicações vasculares pós-operatórias; e sobrevida. RESULTADOS: ocorreram complicações vasculares em 19 pacientes (19%). Os eventos arteriais foram mais comuns, tendo ocorrido precocemente no pós-operatório, e foram associados a altas taxas de perda do enxerto e mortalidade. Em uma análise multivariada, foram identificadosos seguintes fatores: diâmetro da veia porta < 3 mm, proporc¸ão de peso do doador/receptor (DRWR), tempo de isquemia prolongado e uso de enxertos arteriais. CONCLUSÃO: A escolha do tratamento depende do momento do diagnóstico; contudo, nessa série, a cirurgia de revisão, ou correc¸ão cirúrgica, produziu resultados piores que a angioplastia per-cutânea. A redução dos fatores de risco e a detecção precoce de complicações vasculares sãofundamentais para um transplante bem-sucedido. .


Subject(s)
Adolescent , Child , Child, Preschool , Female , Humans , Infant , Infant, Newborn , Male , Liver Transplantation/adverse effects , Postoperative Complications , Vascular Diseases/etiology , Brazil/epidemiology , Follow-Up Studies , Graft Survival , Liver Transplantation/mortality , Multivariate Analysis , Portal Vein/physiology , Retrospective Studies , Risk Factors , Survival Analysis , Vascular Diseases/epidemiology , Vascular Diseases/therapy
2.
Genet. mol. biol ; 31(2): 423-426, 2008. tab
Article in English | LILACS | ID: lil-484977

ABSTRACT

Serpin peptidase inhibitor, clade A (alpha-1 antiproteinase, antitrypsin), member 1 (SERPINA1) deficiency is one of the main genetic causes related to liver disease in children. In SERPINA1 deficiency the most frequent SERPINA1 alleles found are the PI*S and PI*Z alleles. We used the polymerase chain reaction and the amplification created restriction site (ACRS) technique to investigate the prevalence of the PI*S and PI*Z alleles in a group of Brazilian children (n = 200) with liver disease and established the general frequency of the PI*S allele in our population. We found a significant association of the PI*Z allele and liver disease, but no such relationship was found for the PI*S allele. Our results show that SERPINA1 deficiency due to the PI*Z allele, even when heterozygous, is a frequent cause of liver disease in our group of Brazilian children but that the PI*S allele does not confer an increased risk of hepatic disorders in our group of Brazilian children.

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