Clinical events after surgical splenectomy in children with sickle cell anemia.

Division of Pediatric Hematology and Oncology, The Children's Mercy Hospitals and Clinics, 2401 Gillham Road, Kansas City, MO 64108, USA.
Pediatric Surgery International (Impact Factor: 1.06). 03/2010; 26(5):495-500. DOI: 10.1007/s00383-010-2587-4
Source: PubMed

ABSTRACT Despite advances in immune prophylaxis, sepsis remains the most feared complication following splenectomy for acute splenic sequestration crisis (ASSC) in children with sickle cell anemia (SCA). We seek to investigate the true prevalence of sepsis and other complications of splenectomy in this patient population.
We reviewed the records of children with SCA (HbSS) who underwent splenectomy for ASSC between 1993 and 2008 at a single institution.
Fifty-eight patients (33 males) at a median age of 2 years at splenectomy were included with an average post-splenectomy follow-up of 6.4 years (range 6 months-14 years). Thirty-seven patients (64%) underwent laparoscopic splenectomy, and acute chest syndrome (ACS) was the most common post-operative complication (6.9%). There was no difference in the incidence of sepsis pre- and post-splenectomy. The occurrence of vaso-occlusive pain crises (VOC) and ACS was significantly higher after splenectomy. In addition, 14 patients (24%) developed stroke (n = 5) or an abnormal transcranial Doppler (TCD) (n = 9) after splenectomy.
Our data suggest that splenectomy can be safely performed in children with SCA given a low risk of sepsis. However, the increased incidence of VOC, ACS, and stroke or abnormal TCDs after splenectomy remains a concern.

  • [Show abstract] [Hide abstract]
    ABSTRACT: Performance deterioration with time is one of the most important issues in a vacuum insulation panel (VIP), which is mostly due to the inner gas pressure rise. Outgassing from the interior of core materials is the major gas source when the core material is a polymer. Outgassing characteristics of a polycarbonate as the VIP’s core material are examined theoretically and experimentally. To measure the outgassing rate, specific outgassing tests are carried out using a pressure rise method. Diffusive outgassing mechanism is discussed based on the Fick’s law. As the result, the total amount of dissolved gas and the diffusion coefficients of various gases in the polycarbonate are obtained by using the measured outgassing rate. Temperature dependence of the diffusion coefficient of nitrogen is also examined. It is shown that the outgassing rate of polymer core materials can be significantly reduced to a negligible level by a baking pre-treatment in vacuum and/or by a metal coating on the polymer surface.
    Vacuum 02/2011; 85(8):839-846. DOI:10.1016/j.vacuum.2010.12.009 · 1.43 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Pyruvate kinase deficiency is a chronic illness with age specific consequences. Newborns suffer life-threatening hemolytic crisis and hyperbilirubinemia. Adults are at risk for infections because of asplenia, pregnancy-related morbidity, and may suffer organ damage because of systemic iron overload. We describe 27 Old Order Amish patients (ages 8 months-52 years) homozygous for c.1436G>A mutations in PKLR. Each subject had a predictable neonatal course requiring packed red blood cell transfusions (30 ± 5 mL/kg) to control hemolytic disease and intensive phototherapy to prevent kernicterus. Hemochromatosis affected 29% (n = 4) of adult patients, who had inappropriately normal serum hepcidin (34.5 ± 12.7 ng/mL) and GDF-15 (595 ± 335pg/mL) relative to hyperferritinemia (769 ± 595 mg/dL). A high prevalence of HFE gene mutations exists in this population and may contribute to iron-related morbidity. Based on our observations, we present a strategy for long-term management of pyruvate kinase deficiency.
    American Journal of Hematology 10/2011; 86(10):827-34. DOI:10.1002/ajh.22118 · 3.48 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: To compare the effectiveness of different types of splenectomy in children with congenital hemolytic anemias. We constructed key questions that addressed outcomes relevant to clinicians and families on effects of partial or total splenectomy, including hematologic effect, splenic function, and the risk of adverse events. We identified from Pubmed and Embase 703 studies that evaluated different types of splenectomy and accepted 93 studies that satisfied entry criteria. We graded the quality of each report and summarized the overall strength of research evidence for each key question. We did not identify any randomized clinical trials. All types of splenectomy have favorable clinical outcomes in most diseases. We did not identify any hematologic advantage of laparoscopy compared with laparotomy. Adverse events are uncommon in most studies and are minimized with use of laparoscopy. There is a need for randomized clinical trials and improved data collection of different types of splenectomy in congenital hemolytic anemias. Outcomes studied should address the concerns of families and clinicians to assess the risks and benefits of various treatments.
    The Journal of pediatrics 11/2011; 160(4):684-689.e13. DOI:10.1016/j.jpeds.2011.09.030 · 3.74 Impact Factor