Erythropoietic (Congenital) Porphyria: A Rare Abnormality of the Normoblasts
نویسندگان
چکیده
منابع مشابه
Erythropoietic (congenital) porphyria: A rare abnormality of the normoblasts.
ECENT STUDIES” 2, 3 have emphasized that in human beings, photosensitivity occurs as a result of two fundamentally different disturbances of porphyrin metabolism. In porphyria cutanea tarda, in which the excessive porphyrin formation is believed to take place in the liver, symptoms are seldom manifest before adult life.’ Its porphyria erythropoietica (congeisital, photosensitive), where large a...
متن کاملCongenital Erythropoietic Porphyria . II .
Polycythemia and anemia were induced in a patient with congenital erythropoietic porphyria as a possible means of altering erythropoiesis and its attendant porphyrin production. Maintenance of hematocrits at 50 per cent and 60 per cent for periods of 2 weeks decreased both erythropoiesis and porphyrin excretion to about one-half of their initial levels. Conversely with a hematocrit of 25, eryth...
متن کاملCongenital Erythropoietic Porphyria
Polycythemia and anemia were induced in a patient with congenital erythropoietic porphyria as a possible means of altering erythropoiesis and its attendant porphyrin production. Maintenance of hematocrits at 50 per cent and 60 per cent for periods of 2 weeks decreased both erythropoiesis and porphyrin excretion to about one-half of their initial levels. Conversely with a hematocrit of 25, eryth...
متن کاملCongenital erythropoietic porphyria.
Congenital erythropoietic porphyria (CEP) is one of the rarest autosomal-recessive disorders of the porphyrin metabolism caused by the homozygous defect of uroporphyrinogen III cosynthase. High amounts of uroporphyrin I accumulate in all cells and tissues, reflected by an increased erythrocyte porphyrin concentration and excretion of high porphyrin amounts in urine and feces. Dermal deposits of...
متن کاملCongenital Erythropoietic Porphyria
Congenital erythropoietic porphyria (CEP) is a rare cutaneous pophyria which presents with photosensitivity, complicated by presence of hemolytic anemia. There is no permanent cure and the treatment is supportive. A 3 day-old male baby was admitted with neonatal jaundice for phototherapy. The baby developed bullous lesions over both the feet within minutes of starting phototherapy(Fig.1). These...
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ژورنال
عنوان ژورنال: Blood
سال: 1955
ISSN: 0006-4971,1528-0020
DOI: 10.1182/blood.v10.5.416.416