Altered elemental profiles in neuronal ceroid lipofuscinosis.
The elemental profiles of thrombocytes and mononuclear cells were investigated in five patients with Infantile and eight with Juvenile Neuronal Ceroid Lipofuscinosis. The patients with the infantile form had suffered from the disease for a year and those with the juvenile form for some six years. The thrombocytes exhibited increased concentrations of calcium and magnesium, but the same concentrations of iron and zinc as found in healthy subjects. The mononuclear cells exhibited an increased concentration of iron and a reduced concentration of zinc. The elevated concentrations of magnesium, calcium and iron in the thrombocytes and mononuclear cells may represent the end products of ceroid pigmentation. Five patients with Juvenile and one with Infantile Neuronal Ceroid Lipofuscinosis were treated with antioxidants along with vitamins E, B2 and B6, but this treatment did not affect significantly the concentration of iron in the mononuclear cells. However, selenium was detected in some mononuclear cells in all the patients so treated. This was unexpected since iron (III), being antagonistic to selenium in the form of selenite--which was the antioxidant given--forms a stable complex which cannot be broken down biologically.
Johansson
E
E
Department of Radiation Sciences, Division of Physical Biology, Uppsala, Sweden.
Lindh
U
U
Westermarck
T
T
Heiskala
H
H
Santavuori
P
P
D000293:Adolescent; D000975:Antioxidants / Q000627:therapeutic use; D001792:Blood Platelets / Q000378:metabolism; D002118:Calcium / Q000097:blood*; D002648:Child; D003300:Copper / Q000097:blood; D005260:Female; D006801:Humans; D007501:Iron / Q000097:blood*; D007963:Leukocytes, Mononuclear / Q000378:metabolism; D008274:Magnesium / Q000097:blood*; D008297:Male; D009472:Neuronal Ceroid-Lipofuscinoses / Q000097:blood* / Q000188:drug therapy; D011736:Pyridoxine / Q000627:therapeutic use; D012256:Riboflavin / Q000627:therapeutic use; D012643:Selenium / Q000097:blood; D014810:Vitamin E / Q000627:therapeutic use; D015032:Zinc / Q000097:blood*
D000975:Antioxidants; D014810:Vitamin E; D003300:Copper; D007501:Iron; D012643:Selenium; D008274:Magnesium; D015032:Zinc; D011736:Pyridoxine; D002118:Calcium; D012256:Riboflavin