Severe hyperprostaglandin E syndrome with hyperthyroidism--studies of pathogenetic mechanisms.
Hyperprostaglandin E syndrome is a rare disease usually presenting with renal symptoms such as polyuria, polyhydramnios, hypercalciuria, hypokalaemia, and recurrent episodes of extreme fever, diarrhoea, and convulsions. We report a severe variant of this syndrome with obvious pain and prostaglandin E2 (PGE2)-stimulated hyperthyroidism, an association not previously described. Urinary excretion of PGE2 and its metabolite 7 alpha-hydroxy-5,11-diketotetranorprosta-1,16-dioic acid were markedly increased above normal levels (to 53.3 and 1895 ng/h per 1.73 m2, respectively). We studied oxidative capacity of peroxisomes and mitochondria, the sites where PGE2 oxidation takes place. A generalized mitochondrial disease could be ruled out and no deficiency was found in liver peroxisomal oxidases. The basic pathology of hyperprostaglandin E syndrome remains unsolved.
Children's Hospital, University of Helsinki, Finland.
V
V
Fellman
H
H
Pihko
A
A
Majander
H W
HW
Seyberth
D016861:Cyclooxygenase Inhibitors / Q000627:therapeutic use; D015232:Dinoprostone / Q000378:metabolism* / Q000652:urine; D005260:Female; D006801:Humans; D006980:Hyperthyroidism / Q000188:drug therapy / Q000209:etiology* / Q000378:metabolism; D007213:Indomethacin / Q000627:therapeutic use; D007223:Infant; D007231:Infant, Newborn; D008830:Microbodies / Q000378:metabolism; D008931:Mitochondria, Muscle / Q000378:metabolism; D010084:Oxidation-Reduction; D013577:Syndrome
D016861:Cyclooxygenase Inhibitors; D015232:Dinoprostone; D007213:Indomethacin