Pediatric Clinics of North America - CIPERJ

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512 TRACY & RICE German experience An alternative and more extensive approach to partial splenectomy in children who have HS has been advocated by Stoehr and colleagues [49,50] from Go¨ttingen, Germany. In response to the concerns of persistent hemolysis after partial splenectomy as noted by the French and the authors’ groups, Stoehr and colleagues have advocated a more radical spleen resection. This technique is termed, near-total splenectomy (NTS), and is designed to preserve a residual spleen volume of approximately 10 mL, the equivalent of an approximately 98% resection. In their most recent summary, 30 children who had HS received a NTS. At follow-up, the mean hemoglobin concentration increased by 2.9 to 5.0 g/dL, whereas the serum bilirubin level decreased significantly by 15.4 to 56.4 mol/L (approximately 1–3 mg/dL). Although a NTS seems to result in improved hemoglobin levels and decreased hemolysis compared with baseline, preserved splenic function is not demonstrated clearly in this patient cohort and is a point of concern for many clinicians. Therefore, the authors’ group continues to advocate a less radical splenic resection, because an acceptable hematologic result and preserved splenic function are essential to validate and support the use of a partial splenectomy. North American experience Several investigators in North America have reported the use of partial splenectomy in children who have not only HS but also a range of other congenital hemolytic anemias [14,51,53,54]. The authors’ initial experience was with 25 children from Duke University or the Children’s Hospital of Wisconsin [14]. In this initial report, the underlying diagnoses included HS (n ¼ 16), pyruvate kinase deficiency (n ¼ 2), congenital nonspherocytotic hemolytic anemia of unknown etiology (n ¼ 1), HbSC disease (n ¼ 2), HbSS disease (n ¼ 2), HbCC disease (n ¼ 1), and combined HbS/b-thalassemia (n ¼ 1). Since the authors’ original publications, combined experience at these two centers alone is more than 60 children [14,54]. Similar numbers of children have undergone partial splenectomy at other pediatric centers in North America. The results of these studies are summarized later. Splenic regrowth One concern often cited regarding partial splenectomy is regrowth of the splenic remnant and the potential for recurrent hematologic signs and symptoms. In the authors’ experience, there almost always is mild to moderate splenic regrowth after partial splenectomy, as measured by sonographic splenic volume. Regrowth initially is slow, as the splenic remnant usually grows only 15% to 30% above the baseline volume during the first 2 years of follow-up, thus remains significantly below the preoperative size (P!.05). Although the rate of splenic regrowth is variable among children, by 4 years
PARTIAL SPLENECTOMY FOR HEREDITARY SPHEROCYTOSIS 513 of follow-up, the splenic regrowth can be more pronounced, averaging 40% of the original splenic size. Regrowth of the spleen after partial splenectomy, however, is not associated with recurrent hemolysis, similar to the French experience. These findings suggest that although moderate regrowth may occur in some children, the clinical importance of these findings is unclear, because few children, even those who have a high rate of regrowth, have recurrent hematologic symptoms. Therefore, surveillance of splenic regrowth and correlation with clinical status are critical to understanding the longterm outcomes of partial splenectomy. Effect on anemia and hemolysis In the authors’ initial experience, children who had HS and underwent partial splenectomy achieved significantly increased hemoglobin concentrations, similar to the findings of the European investigators. On average, the mean hemoglobin increased by 2 to 3 gm/dL within 3 months of surgery. In some children, this increase persisted for up to 10 years (Fig. 2) [14,54]. Sustained control of hemolysis also was observed, as reticulocyte counts decreased from 12.7% 4.2% preoperatively to 4.9% 2.9% at 2.3 1.5 years of follow-up (mean SD, P!.05). Mean serum bilirubin levels decreased from a level of 2.6 1.1 mg/dL preoperatively to 1.3 0.7 mg/dL at similar follow-up (mean SD, P!.05). Partial splenectomy eliminates almost all symptoms of hypersplenism and control of splenic sequestration (Table 1). As suggested initially in the French experience, the authors also showed that partial splenectomy does not eliminate hemolysis completely. Two of 16 14 Hemoglobin (g/dl) 12 10 8 6 4 Preop 1 mon 3 mon 6 mon 12 mon 2 yr 3 yr 4 yr 5 yr Postoperative interval Fig. 2. Mean hemoglobin concentration after partial splenectomy for 12 recent children who had HS and underwent partial splenectomy at Duke University. Hemoglobin concentration increased compared with preoperative values throughout 4 to 6 years of follow- up (P!.05 by paired Student t test). Error bars show standard deviation.
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Pediatr Clin N Am 55 (2008) xiii-xi
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Pediatr Clin N Am 55 (2008) 287-304
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DECISION ANALYSIS IN PEDIATRIC HEMA
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Nietert et al [30] Treatment of sic
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VENOUS THROMBOEMBOLISM IN CHILDREN
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PEDIATRIC ARTERIAL ISCHEMIC STROKE
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CARE OF PATIENTS WITH VASCULAR ANOM
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358 RODRIGUEZ & HOOTS Fig. 1. X-lin
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Table 1 Clotting factor concentrate
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Table 1 (continued ) Factor VIII (o
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364 RODRIGUEZ & HOOTS was administe
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366 RODRIGUEZ & HOOTS Antifibrinoly
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368 RODRIGUEZ & HOOTS infections an
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370 RODRIGUEZ & HOOTS Fig. 3. Schem
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372 RODRIGUEZ & HOOTS protein is no
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374 RODRIGUEZ & HOOTS [2] Berntorp
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376 RODRIGUEZ & HOOTS [42] Carcao M
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378 ROBERTSON et al von Willebrand
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380 ROBERTSON et al Fig. 2. A propo
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382 ROBERTSON et al a heterogenous
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384 ROBERTSON et al of considering
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386 ROBERTSON et al Type 2M Type 2M
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388 ROBERTSON et al Desmopressin is
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390 ROBERTSON et al References [1]
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392 ROBERTSON et al [46] Nesbitt IM
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394 BLANCHETTE & BOLTON-MAGGS A key
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396 BLANCHETTE & BOLTON-MAGGS recep
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398 BLANCHETTE & BOLTON-MAGGS and p
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400 BLANCHETTE & BOLTON-MAGGS Fig.
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402 BLANCHETTE & BOLTON-MAGGS in di
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404 BLANCHETTE & BOLTON-MAGGS the s
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406 BLANCHETTE & BOLTON-MAGGS findi
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408 BLANCHETTE & BOLTON-MAGGS as a
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410 BLANCHETTE & BOLTON-MAGGS splen
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412 BLANCHETTE & BOLTON-MAGGS with
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414 BLANCHETTE & BOLTON-MAGGS react
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416 BLANCHETTE & BOLTON-MAGGS [16]
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422 FASANO & LUBAN of storage and t
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424 FASANO & LUBAN Leukocyte reduct
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426 FASANO & LUBAN RBCs is decrease
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Table 1 Blood component characteris
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430 FASANO & LUBAN Alloimmunization
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432 FASANO & LUBAN of individual un
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434 FASANO & LUBAN patients have an
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436 FASANO & LUBAN adults; therefor
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438 FASANO & LUBAN [21]. Meta-analy
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440 FASANO & LUBAN Pretransfusion m
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442 FASANO & LUBAN [55,56]. This is
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444 FASANO & LUBAN [20] Roseff SD,
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THALASSEMIA UPDATE 449 hypochromia
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THALASSEMIA UPDATE 451 Fig. 1. Chan
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THALASSEMIA UPDATE 453 delivery of
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THALASSEMIA UPDATE 455 thromboses i
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THALASSEMIA UPDATE 457 On the basis
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THALASSEMIA UPDATE 459 [31] Kan YW,
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