Pediatric Liver Transplantation in Sickle Cell Anemia

Alder, Laura; Vasquez, Robert; Reichman, Trevor; Serrano, Maraistella

doi:10.1177/0009922816648943

Cited by 6 publications

(6 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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“…These cases have a high complication rate, require aggressive postoperative therapy (transfusion/chelation or hydroxyurea/erythropoietin when possible), and outcomes are greatly influenced by comorbidities 21. For example, in a pediatric SCD and biliary atresia patient previously written about, the post-transplant course was complicated by intrahepatic congestion in part caused by ischemia of the bile ducts as a result of sickling; she was treated with aggressive transfusions to keep hemoglobin S levels low 22. It may be extrapolated that also in cases of liver transplant for SCD-caused hepatopathies, that the SCD itself makes the transplant less likely to succeed.…”

Section: Discussionmentioning

confidence: 99%

Autoimmune Liver Disease in Patients With Sickle Cell Disease

Waisbourd‐Zinman

Frenklak

Hakakian

et al. 2020

Journal of Pediatric Hematology/Oncology

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Objective: Hepatic and biliary tract diseases are common in sickle cell disease (SCD) patients, likely due to sickling, hemosiderosis, viral hepatitis, or cholelithiasis. Literature is lacking on associations between SCD, autoimmune hepatitis (AIH), and/or sclerosing cholangitis (SC)-together, autoimmune liver disease (AILD). We aimed to better understand the relationship of these diseases in pediatric patients. Materials and Methods: A retrospective analysis of patients with SCD and AILD at the Children’s Hospital of Philadelphia (January 2008 to August 2015). Results: Seven patients, ages 8 to 23 years (3 males), were identified. Three had AIH, 2 SC, and 2 AIH/SC overlap, known as autoimmune SC. All patients with AIH treated with azathioprine significantly improved their liver enzymes. One patient with SC and inflammatory bowel disease underwent successful bone marrow transplant. Two SC patients died from SCD complications. Conclusions: In this cohort, there seems to be an association between SCD and AILD; SC in this population was severe. Physicians should be aware of this and evaluate patients with SCD and elevated liver enzymes for AILD.

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“…22 Notably, liver disease is likely multifactorial from viral hepatitis, iron overload, and ischemic injury. 23 Manci et al 24 reported that 74.7% of the patients had evidence 1 (50) 89 (78-96) 8 (62) 93 (89-96) 1 y 92 (87)(88)(89)(90)(91)(92)(93)(94)(95)(96) 4 (66) 89 (78)(79)(80)(81)(82)(83)(84)(85)(86)(87)(88)(89)(90)(91)(92)(93)(94)(95)(96) 1 (50) 76 (62-88) 8 (62) 87 (83-91) 2 y 87 (80)(81)(82)(83)(84)(85)(86)(87)(88)(89)…”

Section: Sot In Scdmentioning

confidence: 99%

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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Pediatric Liver Transplantation in Sickle Cell Anemia

Cited by 6 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

Autoimmune Liver Disease in Patients With Sickle Cell Disease

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

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