Hereditary spherocytosis. Recent experience and current concepts of pathophysiology.

Abstract:

:Hereditary spherocytosis is a clinically heterogeneous, genetically determined red blood cell membrane disorder resulting in hemolytic anemia. A deficiency of spectrin, the largest and most abundant structural protein of the erythrocyte membrane skeleton, results in the formation of spherocytes which lack the strength, durability, and flexibility to withstand the stresses of the circulation. Clinical manifestations of the disease are primarily dependent on the severity of hemolysis, which additionally results in an increased incidence of pigment gallstones. The likelihood of cholelithiasis is directly related to patient age and is uncommon before 10 years of age. Splenectomy is indicated in virtually every patient. When the disease is diagnosed in early childhood, the risk of overwhelming postsplenectomy sepsis makes it advisable to delay splenectomy until after 6 years of age if possible. At the time of splenectomy, it is important to identify and remove any accessory spleens. If gallstones are present, cholecystectomy should be performed. Although spherocytosis persists following splenectomy, hemolysis is alleviated and clinical cure of the anemia is achieved for most patients. Patients with recessively inherited spherocytosis are exceptions. Although they are significantly benefited by splenectomy, their anemia is not completely corrected. Splenectomy reduces hemolysis in all patients and thereby decreases the risk for development of pigment gallstones. Excision of an enlarged spleen removes the danger of traumatic rupture.

journal_name

Ann Surg

journal_title

Annals of surgery

authors

Croom RD 3rd,McMillan CW,Orringer EP,Sheldon GF

doi

10.1097/00000658-198601000-00007

subject

Has Abstract

pub_date

1986-01-01 00:00:00

pages

34-9

issue

1

eissn

0003-4932

issn

1528-1140

journal_volume

203

pub_type

杂志文章
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    authors: Eilber FC,Rosen G,Nelson SD,Selch M,Dorey F,Eckardt J,Eilber FR

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    更新日期:2012-03-01 00:00:00

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    更新日期:2011-03-01 00:00:00

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    doi:10.1097/00000658-197506000-00022

    authors: Bismuth H,Kuntziger H,Corlette MB

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  • Recurrent carotid stenosis. A five-year series of 65 reoperations.

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    authors: Thiede A,Engemann R,Korner HH,Deltz E,Muller-Ruchholtz W

    更新日期:1981-03-01 00:00:00

  • Management and outcome of abdominal shotgun wounds. Trauma score and the role of exploratory laparotomy.

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    journal_title:Annals of surgery

    pub_type: 临床试验,杂志文章

    doi:10.1097/00000658-199503000-00009

    authors: Cairns BA,Oller DW,Meyer AA,Napolitano LM,Rutledge R,Baker CC

    更新日期:1995-03-01 00:00:00

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    更新日期:2011-04-01 00:00:00

  • Cost-effectiveness of Enhanced Recovery Versus Conventional Perioperative Management for Colorectal Surgery.

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    pub_type: 临床试验,杂志文章,多中心研究

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    更新日期:2015-12-01 00:00:00

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    pub_type: 杂志文章

    doi:10.1097/SLA.0b013e3181a38ef5

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    doi:10.1097/00000658-198102000-00010

    authors: Kent RB 3rd,van Heerden JA,Weiland LH

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    pub_type: 杂志文章

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    更新日期:2006-04-01 00:00:00

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    更新日期:2013-04-01 00:00:00

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    doi:10.1097/00000658-197406000-00001

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  • Perineal transanal approach: a new standard for laparoscopic sphincter-saving resection in low rectal cancer, a randomized trial.

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    pub_type: 杂志文章

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    doi:

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