Acute Sickle Hepatic Crisis after Liver Transplantation in a Patient with Hb SC Disease

Gillis, J. H.; Parsa, Laxmi; Sylvestre, Pamela B.; Dbouk, Nader

doi:10.1155/2015/761740

Cited by 5 publications

(4 citation statements)

References 6 publications

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“…In practice, LT can be indicated in two settings [4]: acute liver failure mimicking fulminant hepatitis and end-stage chronic liver disease. Data regarding liver transplantation (LT) in patients with SCD are limited to mostly case reports (Table 1) [5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24]. Because of the paucity of data available regarding the indications for LT and perioperative outcomes as well as the lack of long-term follow-up in most reported cases, the present study entailed a thorough analysis of a large single-center experience of LT in patients with SCD.…”

Section: Introductionmentioning

confidence: 99%

Liver transplantation in patients with sickle cell disease: possible but challenging—a cohort study

et al. 2020

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The liver is frequently affected in patients with sickle cell disease (SCD), but few reports have described liver transplantation (LT) in patients with SCD. We present a thorough analysis of the largest single-center series of LT in patients with SCD and the first systematic review. There were 21 patients with a median age of 37.6 years. LT was performed for acute liver failure related to the sickling process (57%) or electively for end-stage liver disease (43%). Prior to LT, 13 patients (62%) were in the intensive care unit and required mechanical ventilation (33%), vasopressor therapy (24%), renal replacement therapy (10%), or molecular adsorbent recirculating system therapy (19%). Post-LT morbidity and mortality were 95% and 33%, respectively. Patient survival at 1 and 5 years were 58.3% and 41.7%, respectively, in the urgent group and 88.9% and 77.8%, respectively, in the elective group. A total of 22 transplant patients with SCD are described in 20 articles in the literature. The 1-and 5-year patient survival rates for the 18 evaluable patients were 75% and 65%, respectively. LT improves survival in patients with SCD and acute liver failure or end-stage liver disease but is associated with high morbidity during the early postoperative course.

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Section: Introductionmentioning

confidence: 99%

Liver transplantation in patients with sickle cell disease: possible but challenging—a cohort study

et al. 2020

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“…36 For liver grafts, acute sickle hepatic crises and recurrent hepatopathy were reported posttransplantation. [37][38][39] These observations suggest that transplanted organs are at risk of injury from sickling and vaso-occlusion and highlight that patients undergoing SOT for complications of SCD should be considered for disease-modifying procedures such as HSCT.…”

Section: Sot In Scdmentioning

confidence: 96%

Toward dual hematopoietic stem-cell transplantation and solid-organ transplantation for sickle-cell disease

Hosoya

Levine

Abt³

et al. 2018

Blood Advances

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Sickle-cell disease (SCD) leads to recurrent vaso-occlusive crises, chronic end-organ damage, and resultant physical, psychological, and social disabilities. Although hematopoietic stem-cell transplantation (HSCT) is potentially curative for SCD, this procedure is associated with well-recognized morbidity and mortality and thus is ideally offered only to patients at high risk of significant complications. However, it is difficult to identify patients at high risk before significant complications have occurred, and once patients experience significant organ damage, they are considered poor candidates for HSCT. In turn, patients who have experienced long-term organ toxicity from SCD such as renal or liver failure may be candidates for solid-organ transplantation (SOT); however, the transplanted organs are at risk of damage by the original disease. Thus, dual HSCT and organ transplantation could simultaneously replace the failing organ and eliminate the underlying disease process. Advances in HSCT conditioning such as reduced-intensity regimens and alternative donor selection may expand both the feasibility of and potential donor pool for transplantation. This review summarizes the current state of HSCT and organ transplantation in SCD and discusses future directions and the clinical feasibility of dual HSCT/SOT.

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“…Outro aspecto particular é o acometimento da criança em um período crítico de crescimento e desenvolvimento, do que decorre o caráter mais urgente do transplante pediátrico, para que possíveis déficits possam ser evitados ou revertidos. Mais recentemente, o diagnóstico precoce de algumas doenças metabó-licas, com ou sem comprometimento neurológico, também são indicação de transplante 1 . Na população pediátrica, 80% das mortes causadas por falência hepática ocorrem antes dos 2 anos de vida.…”

Section: Desenvolvimentounclassified

Indicações e contra-indicações no transplante hepático pediátrico

Porta¹,

Tofoli²

2017

Int J Nutrol

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RESUMOAvanços nas técnicas cirúrgicas e nos imunossupressores propiciaram melhor prognósticos dos pacientes eleitos para transplante hepático pediátrico. Muitas dúvidas ainda surgem quanto as indicações de transplante. É importante ressaltar que na infância o tempo de desconpensação hepática pode ocorrer de forma mais rápida que no adulto. E os principais sintomas são : colestase progressiva, encefalopatia, ascite, sangramento por varizes de esôfago, síndrome hepatopulmonar, desnutrição, infecções de repetição, disfunção hepática, prurido incapacitante e retardo no crescimento e desenvolvimento.Palavras-chave: transplante hepático, crianças e indicações. ABSTRACTAdvances in cirurgical techniques and immunessupressores result in better prognosis of patients elected to pediatric liver transplant. Many questions still arise as the transplant indications . Notes that in childhood the liver failure can occur faster than in adults. The main symptons are: progressive cholestasis, encephalopathy , ascites, bleeding of esophageal varices , Hepatopulmonary syndrome, malnutrition , recurrent infections , liver dysfunction , disabling itching and delayed growth and development. Keywords: Liver transplantation , children and indications. INTRODUÇÃONas últimas décadas, o transplante hepático tornou-se um tratamento efetivo e amplamente aceito para a população pediátrica com doença hepática crônica avançada, doenças metabólicas com ou sem comprometimento hepático e hepatite fulminante. Nos últimos 15 anos, houveram grandes avanços em virtude do desenvolvimento de variações das técnicas cirúrgicas, aparecimento de novos imunossupressores e melhor controle das infecções pós cirurgia, além da maior disponibilidade de órgãos destinados às crianças. Estes fatores foram decisivos para aumentar a sobrevida pós-operatória e o acesso de maior número de candidatos ao transplante. O que resultou em um aumento em mais de 10 vezes o número de crianças e adolescentes pós transplante hepático comparado com aqueles pré transplante. DesenvolvimentoNas doenças hepáticas da infância, o intervalo entre diagnóstico, disfunção e descompensação hepáticas é, frequentemente, mais curto quando

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Acute Sickle Hepatic Crisis after Liver Transplantation in a Patient with Hb SC Disease

Cited by 5 publications

References 6 publications

Liver transplantation in patients with sickle cell disease: possible but challenging—a cohort study

Liver transplantation in patients with sickle cell disease: possible but challenging—a cohort study

Toward dual hematopoietic stem-cell transplantation and solid-organ transplantation for sickle-cell disease

Indicações e contra-indicações no transplante hepático pediátrico

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