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HEREDITER SFEROSITOZ

Yıl 1993, Cilt: 46 Sayı: 1, 101 - 108, 31.03.1993

Duran Canatan

Öz

Klinigimizde 1980-1990 yillari arasinda izledigimiz 22 herediter
sferositozisli hasta degerlendirilmistir. Hastalarimizin 11’'i erkek, 11’i
kizdi, Fizik bulgu olarak hepsinde degisik agirlikta anemi, splenomegali, % 38 inde ikter vardi. Ayrica 3°tinde neonatal ikter, 3 inde aplastik kriz, ikisinde kolelithiazis ve birinde hemoglobiniiri vardi. Hastalarimizin 14’ne splenektomi yapuldi. Splenektomi yas ortalamési
8.36 + 4.13 tu. Splenektomi sonrasi izlenen hastalarimizda klinik ve
: Herediter sferositoz. sorun kalmamisti.

: Herediter, sferositoz, : Herediter

Anahtar Kelimeler

Herediter sferositoz : Herediter

Etik Beyan

-

Destekleyen Kurum

-

Proje Numarası

-

Teşekkür

-

Kaynakça

  • 1. Agre P et al; Inheritance pattern and clinical response to splenectomy as a reflection of erythrocyte spectrin deficiency in herediter spherocytosis. N. Engl. J. Med, 315 : 1579-83, 1986.
  • 2.a. Arcasoy A ve ark . Herediter sferositoz (Klinik, hematolojik ve genetik yonler ile) Ankara Tip Billteni, 1: 69-76, 1979.
  • 3. Balari SA Espinosa VJ Fuertes F: A new modification of the «Pink Test» for the diagnosis of hereditary spherocytosis. Acta Haemat. 82 ; 213-4, 1989.
  • 4.Becker PS Morrow JS Lux SE; Abnormal oxidant sensivity and beta-chain structure of spectrin in hereditary spherocytosis. Associated with defective spectrine-protein 4.1 pinding. J, Clin. Invest. 80 : 557-65, 1987.
  • 5.Becker PS Lux SE: Hereditary sherocytosis and related disorders. Clin. in Haemat. 14:1, 1985.
  • 6.Chasis JA Agra P Mohandas N : Decreased membran mechanical stability an in vivo. loss of surface area reflect spectrin deficiencies in hereditary spherocytosis. J. Clin. Invest. 82 : 617-24, 1088.
  • 7.Chilcote RB et al : Association of red cell spherocytosis with deletion of the short arm of chromosome 8, Blood 68:1: 156-9, 1987. 8.Croom RD et al: Hereditary spherocytosis Ann. Surg. 203 : 34-38, 1986.
  • 9.Eber SW Armbrust R Schréter W : Variable clinical severity of hereditary spherocytosis : Relation to erythrocytic spectrin concentration, osmotic fragility, and autohemolysis. J. Pediatrics ,117 (3) : 409-15, 1990,
  • 10.Gaczynska M et al: Abnormal degradation of erythrocyte membrrane proteins in hereditary spherocytosis. Clin. Chimica Acta. 168 : 7-11, 1987.
  • 11.Garbarz M et al . Hereditary pyropoikilocytosis and eliptocytosis in a white Frenc family with the Spectrin al/74 variant related to a CGT to CAT codn change (Arg to His) at position 22 of the spectrin al Domain. Blood 75 (8) : 1691 - 8, 1990.
  • 12.Ideguchi H et al: A genetic defect of erythrocyte band 4.2 protein associated with hereditary spherocytosis. Br. J. Haematol. 74 : 347-53, 1990,
  • 13.Judkieewicz L et al : Modifed end-point glycerol hemolysis assay as a screening test for herediteary spherocytosis that requieres no venipunctute. Am, J. Hematol, 26 « 89-91, 1987.
  • 14.Kitatani M et al ; Localization of spherocytosis gene to chromosome segment 8p 11.22 - 8p 21.1, hum. genet. 78 : 94-5, 1988.
  • 15Liu SC et al: Alteration of the erythrocyte membrane skeletal ultrastructure in hereditary spherocytosis, hereditary elliptocytosis and pyropoikilocytosis. Blood, 76 3 198-205, 1990.
  • 16.Lux SE : Hematology of infancy and childhood Ed : Nathon Oski 3rd edition W.B. Sounders Company. pp : 470-90, 1987.
  • 17.Manno C and Cohen AR ; Splenectomy in mild hereditary spherocytosis. Is it worth the risk? Am. J, Ped. Hemat. Oncol. 11 (3) : 300-303, 1989.
  • 18.Nunoue T et al : Infection with human parvovirus (B19), aplasia of the bone marrow and a rash in hereditary spherocytosis. J. Infect. 14 : 67-70, 1987.
  • 19.Pinto I et al : A modification of the «Pink test» may improve the diagnosis of hereditary spherocytosis. Acta Haemat. 2 : 53-54, 1989.
  • 20.Pothier B et al : Assingment of Sp 1/74 hereditary elliliptocytosis to thea or B chain of spectrin through in vitro dimer reconstitution. Blood, 75 (10), 2061-69, 1990.
  • 21Waugh RE and Agre P ; Reduction of erythrocyte membrane viscoelastic coefficient reflect spectrin deficiencies in hereditary spherocytosis. J. Clin, Invest. 81 + 133-141, 1088

HEREDITER SFEROSITOZ

Yıl 1993, Cilt: 46 Sayı: 1, 101 - 108, 31.03.1993

Duran Canatan

Öz

Klinigimizde 1980-1990 yillari arasinda izledigimiz 22 herediter
sferositozisli hasta degerlendirilmistir. Hastalarimizin 11’'i erkek, 11’i
kizdi, Fizik bulgu olarak hepsinde degisik agirlikta anemi, splenomegali, % 38 inde ikter vardi. Ayrica 3°tinde neonatal ikter, 3 inde aplastik kriz, ikisinde kolelithiazis ve birinde hemoglobiniiri vardi. Hastalarimizin 14’ne splenektomi yapuldi. Splenektomi yas ortalamési
8.36 + 4.13 tu. Splenektomi sonrasi izlenen hastalarimizda klinik ve
hematolojik sorun kalmamisti.

Anahtar Kelimeler

Herediter sferositoz : Herediter

Etik Beyan

-

Destekleyen Kurum

-

Proje Numarası

-

Teşekkür

-

Kaynakça

  • 1. Agre P et al; Inheritance pattern and clinical response to splenectomy as a reflection of erythrocyte spectrin deficiency in herediter spherocytosis. N. Engl. J. Med, 315 : 1579-83, 1986.
  • 2.a. Arcasoy A ve ark . Herediter sferositoz (Klinik, hematolojik ve genetik yonler ile) Ankara Tip Billteni, 1: 69-76, 1979.
  • 3. Balari SA Espinosa VJ Fuertes F: A new modification of the «Pink Test» for the diagnosis of hereditary spherocytosis. Acta Haemat. 82 ; 213-4, 1989.
  • 4.Becker PS Morrow JS Lux SE; Abnormal oxidant sensivity and beta-chain structure of spectrin in hereditary spherocytosis. Associated with defective spectrine-protein 4.1 pinding. J, Clin. Invest. 80 : 557-65, 1987.
  • 5.Becker PS Lux SE: Hereditary sherocytosis and related disorders. Clin. in Haemat. 14:1, 1985.
  • 6.Chasis JA Agra P Mohandas N : Decreased membran mechanical stability an in vivo. loss of surface area reflect spectrin deficiencies in hereditary spherocytosis. J. Clin. Invest. 82 : 617-24, 1088.
  • 7.Chilcote RB et al : Association of red cell spherocytosis with deletion of the short arm of chromosome 8, Blood 68:1: 156-9, 1987. 8.Croom RD et al: Hereditary spherocytosis Ann. Surg. 203 : 34-38, 1986.
  • 9.Eber SW Armbrust R Schréter W : Variable clinical severity of hereditary spherocytosis : Relation to erythrocytic spectrin concentration, osmotic fragility, and autohemolysis. J. Pediatrics ,117 (3) : 409-15, 1990,
  • 10.Gaczynska M et al: Abnormal degradation of erythrocyte membrrane proteins in hereditary spherocytosis. Clin. Chimica Acta. 168 : 7-11, 1987.
  • 11.Garbarz M et al . Hereditary pyropoikilocytosis and eliptocytosis in a white Frenc family with the Spectrin al/74 variant related to a CGT to CAT codn change (Arg to His) at position 22 of the spectrin al Domain. Blood 75 (8) : 1691 - 8, 1990.
  • 12.Ideguchi H et al: A genetic defect of erythrocyte band 4.2 protein associated with hereditary spherocytosis. Br. J. Haematol. 74 : 347-53, 1990,
  • 13.Judkieewicz L et al : Modifed end-point glycerol hemolysis assay as a screening test for herediteary spherocytosis that requieres no venipunctute. Am, J. Hematol, 26 « 89-91, 1987.
  • 14.Kitatani M et al ; Localization of spherocytosis gene to chromosome segment 8p 11.22 - 8p 21.1, hum. genet. 78 : 94-5, 1988.
  • 15Liu SC et al: Alteration of the erythrocyte membrane skeletal ultrastructure in hereditary spherocytosis, hereditary elliptocytosis and pyropoikilocytosis. Blood, 76 3 198-205, 1990.
  • 16.Lux SE : Hematology of infancy and childhood Ed : Nathon Oski 3rd edition W.B. Sounders Company. pp : 470-90, 1987.
  • 17.Manno C and Cohen AR ; Splenectomy in mild hereditary spherocytosis. Is it worth the risk? Am. J, Ped. Hemat. Oncol. 11 (3) : 300-303, 1989.
  • 18.Nunoue T et al : Infection with human parvovirus (B19), aplasia of the bone marrow and a rash in hereditary spherocytosis. J. Infect. 14 : 67-70, 1987.
  • 19.Pinto I et al : A modification of the «Pink test» may improve the diagnosis of hereditary spherocytosis. Acta Haemat. 2 : 53-54, 1989.
  • 20.Pothier B et al : Assingment of Sp 1/74 hereditary elliliptocytosis to thea or B chain of spectrin through in vitro dimer reconstitution. Blood, 75 (10), 2061-69, 1990.
  • 21Waugh RE and Agre P ; Reduction of erythrocyte membrane viscoelastic coefficient reflect spectrin deficiencies in hereditary spherocytosis. J. Clin, Invest. 81 + 133-141, 1088
Toplam 20 adet kaynakça vardır.

Ayrıntılar

Birincil Dil İngilizce
Konular Hematoloji
Bölüm Makaleler
Yazarlar

Duran Canatan

Proje Numarası -
Yayımlanma Tarihi 31 Mart 1993
Yayımlandığı Sayı Yıl 1993 Cilt: 46 Sayı: 1

Kaynak Göster

APA Canatan, D. (1993). HEREDITER SFEROSITOZ. Ankara Üniversitesi Tıp Fakültesi Mecmuası, 46(1), 101-108.
AMA Canatan D. HEREDITER SFEROSITOZ. Ankara Üniversitesi Tıp Fakültesi Mecmuası. Mart 1993;46(1):101-108.
Chicago Canatan, Duran. “HEREDITER SFEROSITOZ”. Ankara Üniversitesi Tıp Fakültesi Mecmuası 46, sy. 1 (Mart 1993): 101-8.
EndNote Canatan D (01 Mart 1993) HEREDITER SFEROSITOZ. Ankara Üniversitesi Tıp Fakültesi Mecmuası 46 1 101–108.
IEEE D. Canatan, “HEREDITER SFEROSITOZ”, Ankara Üniversitesi Tıp Fakültesi Mecmuası, c. 46, sy. 1, ss. 101–108, 1993.
ISNAD Canatan, Duran. “HEREDITER SFEROSITOZ”. Ankara Üniversitesi Tıp Fakültesi Mecmuası 46/1 (Mart 1993), 101-108.
JAMA Canatan D. HEREDITER SFEROSITOZ. Ankara Üniversitesi Tıp Fakültesi Mecmuası. 1993;46:101–108.
MLA Canatan, Duran. “HEREDITER SFEROSITOZ”. Ankara Üniversitesi Tıp Fakültesi Mecmuası, c. 46, sy. 1, 1993, ss. 101-8.
Vancouver Canatan D. HEREDITER SFEROSITOZ. Ankara Üniversitesi Tıp Fakültesi Mecmuası. 1993;46(1):101-8.
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