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Konjenital Eritropoietik Porfirya

Year 1981, Volume: 34 Issue: 3, 565 - 572, 30.09.1981

Abstract

Eritropoietik porfirya (KEP) veya Günther hastalığı nadir görü!en konjenital bir hastalıktır. Işığa karşı duyarlılığın çok belirgin oluşu ve aşırı porfirinüri bu hastalığın hepatik porfiryalar ile karıştırılmasına neden olmaktadır (4), Bu derle mede hastalığa özgü öze:likler üzerine eğilinerek diğer porfiryalardan nasıl ayır dedilebileceği anlatılacaktır

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References

  • Aldrİch RA ve ark. : Photosenesİtİve or congenital porphyria wİth hemolytic anemia, Blood 6 t 685, 1951
  • Garrod AE : Congenital porphyria : a postscripL Q J Med 5 : 473, 1936
  • 3 - Gray CH : ısotope studies in porphyria. Br Med 8 : 229, 1952
  • 4 - Günther H : Handbuch der Krankheiten deş Blutes und der bluthildengen Organe, kitabından zikren. Editör : Schittenhelm, Springer-Werlag, Berlin ,1955
  • 5 - Heilmeyr LE ve ark. : Porphyria eritropoietica congenita Günther, Dtsch MedWochenschr 88 : 2449, 1963
  • 6 - Heilmeyer L, Clotten R : Die congenitale erythropoİetİsche Coproporphyrie, Dtsch Med Wochenschr 89 2 649, 1964
  • 7 - Hofstad F, Eriksen L t Congenital erythropoİetİc porphyrıa Wİth a hitherto undescribed porphyrin pattern, Acta Pediatr Scand 62 : 380, 1973 Kramer S ve ark. : The anemia of erythropoietic porphyria With the filşt descripüon of the disease in an elderly patient, Br J Haematol II : 666, 1965 Alptuna -
  • 9 - Levin EY Comperative aspects of porphyria in man and animals, Ann N Y Acad Sci 241 : 347, 1974 10 Levin EY : Erythropoietic poryhyria ya cevap klsrmnda, Ann N Y Acad Sci 244 494, 1975
  • 11 - Levin EY, Flyger V : Erythropoietic porphyria of the fox squirrel Sciurus niger J ClinInvest 52 : 96, 1973
  • 12 - Miyagi K ve ark, : The activities of uroporphyrinogen synthetase and cosynthetase in congenital erythropoietic porphyria (CEP). Am J Haematol 1 : 3, 1976
  • 13 - Nicholson DC ve ark : Isotopic studies of the erythropoietic and hepatic components of congenital porphyria and «erythropoietic* protoporphyria Clin Sci 44 : 135, 1Ð73
  • 14 Pain R Ve ark. : Erythropoietic uroporphyria of Günther first presenting at 58 years with positive family studies. Br Med J 3 : 621, 1975
  • 15 - Schmid R, Seålwartz S, Sundberg D : Erythropoietic (congenital porphyris) : A rare abnormality of normoblasts. mood 10 : 4416, 1955
  • 16 - Varadi S : Haematological aspects in a case of erythropoietic porphyria. Br J Haemat01 4 : 270, 1958
  • 17 - Watson CJ ve ark, : A suggested control gene mechanism for the excessive production of types I and 111 porphyrins in congenital erythropoietic porphyria. Proc Natl Acad Sci USA 52 : 478, 1964
  • 18 - Weyer VA, Schmid R : The porphyrias, Metabolic bases of genetik disease adli kitataptan zikren Chap : 50. MC Graw Hill. London, 1978

Congenital Erythropoietic Porphyria

Year 1981, Volume: 34 Issue: 3, 565 - 572, 30.09.1981

Abstract

Erythropoietic porphyria (EPP) or Günther disease is a rare congenital disease. The very pronounced sensitivity to light and the extreme porphyrinuria cause this disease to be confused with hepatic porphyrias (4). In this review, the specific features of the disease will be discussed and how it can be distinguished from other porphyrias will be explained.

Supporting Institution

-

Project Number

-

Thanks

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References

  • Aldrİch RA ve ark. : Photosenesİtİve or congenital porphyria wİth hemolytic anemia, Blood 6 t 685, 1951
  • Garrod AE : Congenital porphyria : a postscripL Q J Med 5 : 473, 1936
  • 3 - Gray CH : ısotope studies in porphyria. Br Med 8 : 229, 1952
  • 4 - Günther H : Handbuch der Krankheiten deş Blutes und der bluthildengen Organe, kitabından zikren. Editör : Schittenhelm, Springer-Werlag, Berlin ,1955
  • 5 - Heilmeyr LE ve ark. : Porphyria eritropoietica congenita Günther, Dtsch MedWochenschr 88 : 2449, 1963
  • 6 - Heilmeyer L, Clotten R : Die congenitale erythropoİetİsche Coproporphyrie, Dtsch Med Wochenschr 89 2 649, 1964
  • 7 - Hofstad F, Eriksen L t Congenital erythropoİetİc porphyrıa Wİth a hitherto undescribed porphyrin pattern, Acta Pediatr Scand 62 : 380, 1973 Kramer S ve ark. : The anemia of erythropoietic porphyria With the filşt descripüon of the disease in an elderly patient, Br J Haematol II : 666, 1965 Alptuna -
  • 9 - Levin EY Comperative aspects of porphyria in man and animals, Ann N Y Acad Sci 241 : 347, 1974 10 Levin EY : Erythropoietic poryhyria ya cevap klsrmnda, Ann N Y Acad Sci 244 494, 1975
  • 11 - Levin EY, Flyger V : Erythropoietic porphyria of the fox squirrel Sciurus niger J ClinInvest 52 : 96, 1973
  • 12 - Miyagi K ve ark, : The activities of uroporphyrinogen synthetase and cosynthetase in congenital erythropoietic porphyria (CEP). Am J Haematol 1 : 3, 1976
  • 13 - Nicholson DC ve ark : Isotopic studies of the erythropoietic and hepatic components of congenital porphyria and «erythropoietic* protoporphyria Clin Sci 44 : 135, 1Ð73
  • 14 Pain R Ve ark. : Erythropoietic uroporphyria of Günther first presenting at 58 years with positive family studies. Br Med J 3 : 621, 1975
  • 15 - Schmid R, Seålwartz S, Sundberg D : Erythropoietic (congenital porphyris) : A rare abnormality of normoblasts. mood 10 : 4416, 1955
  • 16 - Varadi S : Haematological aspects in a case of erythropoietic porphyria. Br J Haemat01 4 : 270, 1958
  • 17 - Watson CJ ve ark, : A suggested control gene mechanism for the excessive production of types I and 111 porphyrins in congenital erythropoietic porphyria. Proc Natl Acad Sci USA 52 : 478, 1964
  • 18 - Weyer VA, Schmid R : The porphyrias, Metabolic bases of genetik disease adli kitataptan zikren Chap : 50. MC Graw Hill. London, 1978
There are 16 citations in total.

Details

Primary Language English
Subjects Brain and Nerve Surgery (Neurosurgery), Pediatric Gastroenterology
Journal Section Research Article
Authors

Mehmet Selçuk

Project Number -
Publication Date September 30, 1981
Published in Issue Year 1981 Volume: 34 Issue: 3

Cite

APA Selçuk, M. (1981). Congenital Erythropoietic Porphyria. Ankara Üniversitesi Tıp Fakültesi Mecmuası, 34(3), 565-572.
AMA Selçuk M. Congenital Erythropoietic Porphyria. Ankara Üniversitesi Tıp Fakültesi Mecmuası. September 1981;34(3):565-572.
Chicago Selçuk, Mehmet. “Congenital Erythropoietic Porphyria”. Ankara Üniversitesi Tıp Fakültesi Mecmuası 34, no. 3 (September 1981): 565-72.
EndNote Selçuk M (September 1, 1981) Congenital Erythropoietic Porphyria. Ankara Üniversitesi Tıp Fakültesi Mecmuası 34 3 565–572.
IEEE M. Selçuk, “Congenital Erythropoietic Porphyria”, Ankara Üniversitesi Tıp Fakültesi Mecmuası, vol. 34, no. 3, pp. 565–572, 1981.
ISNAD Selçuk, Mehmet. “Congenital Erythropoietic Porphyria”. Ankara Üniversitesi Tıp Fakültesi Mecmuası 34/3 (September1981), 565-572.
JAMA Selçuk M. Congenital Erythropoietic Porphyria. Ankara Üniversitesi Tıp Fakültesi Mecmuası. 1981;34:565–572.
MLA Selçuk, Mehmet. “Congenital Erythropoietic Porphyria”. Ankara Üniversitesi Tıp Fakültesi Mecmuası, vol. 34, no. 3, 1981, pp. 565-72.
Vancouver Selçuk M. Congenital Erythropoietic Porphyria. Ankara Üniversitesi Tıp Fakültesi Mecmuası. 1981;34(3):565-72.