Near-total splenectomy for hereditary spherocytosis: clinical prospects in relation to disease severity.
We prospectively studied the efficacy of near total splenectomy (NTS) for managing hereditary spherocytosis (HS) based on haemoglobin (Hb), total bilirubin and splenic remnant regrowth in 30 children receiving NTS for HS between November 1996 and December 2004 (mean followup 3.6 years). Patients were classified into three severity groups. At followup, mean Hb had increased by 2.9-5.0 g/dl (P = 0.110) and bilirubin dropped by 15.4-56.4 mumol/l. Remnant regrowth was moderate, within the weight-specific norm. Amongst spleen-preserving techniques, NTS shows markedly lower rates of recurrent haemolysis, remnant regrowth and secondary operations, thus potentially benefiting all clinical forms of HS.
Department of General Surgery, Georg August University, Goettingen, Germany. gstoehr@chirurgie-goettingen.de
Gerhard A
GA
Stoehr
Julian N
JN
Sobh
Jan
J
Luecken
Kathrin
K
Heidemann
Uwe
U
Mittler
Reinhard
R
Hilgers
Stefan W
SW
Eber
D000293:Adolescent; D000328:Adult; D001663:Bilirubin / Q000097:blood; D002648:Child; D002675:Child, Preschool; D005260:Female; D006454:Hemoglobins / Q000032:analysis; D006461:Hemolysis / Q000502:physiology; D006801:Humans; D008297:Male; D011183:Postoperative Complications; D011184:Postoperative Period; D011446:Prospective Studies; D012307:Risk Factors; D018805:Sepsis / Q000209:etiology; D012720:Severity of Illness Index; D013103:Spherocytosis, Hereditary / Q000473:pathology / Q000503:physiopathology / Q000601:surgery*; D013154:Spleen / Q000473:pathology; D013156:Splenectomy / Q000379:methods*; D016896:Treatment Outcome
D006454:Hemoglobins; D001663:Bilirubin