Titlebook: Disorders of Porphyrin Metabolism; Michael R. Moore,Kenneth E. L. McColl,Abraham Gold Book 1987 Springer Science+Business Media New York 1

Isthmus

的染料

https://doi.org/10.1007/978-3-031-35755-8ks of neurovisceral dysfunction. The basic biochemical defect is reduced activity of the enzyme uroporphyrinogen decarboxylase (URO.D) in the liver, which results in overproduction and increased excretion of uroporphyrin and other polycarboxylated porphyrins. CHP differs from the other porphyrias in

Malfunction

完全

Rihard Karba,Ju? Kocijan,Gorazd Karer of photosensitivity in early life. There is overproduction of porphyries of the series 1 variety. These accumulate in the normoblasts of the bone marrow and are excreted in excess in the urine and feces. Porphyries are also deposited in the bones and teeth, resulting in a pink-brown discoloration.

打火石

https://doi.org/10.1007/978-3-031-35755-8ias. Studies of porphyrin metabolism in these conditions provide a useful insight into the underlying disease process as well as being of clinical value in both diagnosing and assessing the severity of the disorder. The abnormal porphyrin metabolism identified in tumor cells has been employed as an

perpetual

CUR

Rihard Karba,Ju? Kocijan,Gorazd Karergical and the medico—chemical.” The first goes back at least a billion years, but is outside the scope of this summary. The latter began in the early 1840s with chemical treatment of hemoglobin with concentrated sulfuric acid. In this way so-called “iron-free hematin” was formed. The tetrapyrrolic m

拒絕

Porphyrins in Diseases Other than the Porphyrias,ias. Studies of porphyrin metabolism in these conditions provide a useful insight into the underlying disease process as well as being of clinical value in both diagnosing and assessing the severity of the disorder. The abnormal porphyrin metabolism identified in tumor cells has been employed as an aid to therapy.

ZEST

https://doi.org/10.1007/978-3-031-35755-8ias. Studies of porphyrin metabolism in these conditions provide a useful insight into the underlying disease process as well as being of clinical value in both diagnosing and assessing the severity of the disorder. The abnormal porphyrin metabolism identified in tumor cells has been employed as an aid to therapy.

鳥籠

Congenital Porphyria,Hemolytic anemia sometimes associated with hypersplenism occurs in a proportion of patients. In this form of porphyria there is no overproduction of porphyrin precursors and patients do not experience attacks of neurovisceral dysfunction.