Literature DB >> 9537552

Successful surgical outcome in children with sickle hemoglobinopathies: the Duke University experience.

D M Adams1, R E Ware, W H Schultz, A K Ross, K T Oldham, T R Kinney.   

Abstract

BACKGROUND/
PURPOSE: Surgery in patients with sickle hemoglobinopathies can be problematic because of the potential for sickling events in the perioperative and postoperative period. The authors and others have previously reported successful surgical outcomes using an aggressive erythrocyte transfusion regimen, designed to alleviate anemia and to reduce the percentage of sickle hemoglobin to below 30%. Recently, a randomized trial compared this aggressive regimen with a more conservative transfusion regimen and found no differences in perioperative complications. The incidence of complications, however, was very high in each group (31% to 35%).
METHODS: The authors therefore analyzed retrospectively their surgical experience in children with sickle hemoglobinopathies over the past 10 years to determine the efficacy of an aggressive transfusion regimen and skilled perioperative care in their patient population.
RESULTS: A total of 130 surgical procedures were performed on 92 children including 54 cholecystectomies (42%), 23 splenectomies (18%), 12 ENT procedures (9%), 11 central line placements and removals (8%), 7 herniorrhaphies (5%), 7 appendectomies (5%), and 16 miscellaneous operations (13%). The mean age of the children was 10 years (range, 1 to 22 years), and the mean weight was 32.1 kg (range, 9.9 to 76.8 kg). The average hemoglobin (mean +/- 1 SD) at the time of surgery was 11.2+/-1.3 g/dL, and the average percent hemoglobin S was 21+/-11%.
CONCLUSIONS: Relatively few transfusions were required to achieve these endpoints, and the complications resulting from transfusions were minimal. Similarly, the number of perioperative and postoperative events was very low.

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Year:  1998        PMID: 9537552     DOI: 10.1016/s0022-3468(98)90083-5

Source DB:  PubMed          Journal:  J Pediatr Surg        ISSN: 0022-3468            Impact factor:   2.545


  3 in total

1.  Evaluation of partial and total splenectomy in children with sickle cell disease using an Internet-based registry.

Authors:  Sofia Mouttalib; Henry E Rice; Denise Snyder; Justin S Levens; Audra Reiter; Pauline Soler; Jennifer A Rothman; Courtney D Thornburg
Journal:  Pediatr Blood Cancer       Date:  2012-01-11       Impact factor: 3.167

2.  Should cholecystectomy be performed concomitantly with splenectomy in children with sickle-cell disease?

Authors:  Ahmed H Al-Salem
Journal:  Pediatr Surg Int       Date:  2003-01-16       Impact factor: 1.827

3.  Risks and Benefits of Prophylactic Transfusion before Cholecystectomy in Sickle Cell Disease.

Authors:  Elise Rambaud; Brigitte Ranque; Sofia Tsiakyroudi; Laure Joseph; Nathalie Bouly; Richard Douard; Anne François; Jacques Pouchot; Jean-Benoît Arlet
Journal:  J Clin Med       Date:  2022-07-09       Impact factor: 4.964

  3 in total

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