Hereditary corpoporphyria presenting with deep jaundice and photosensitivity
(EN)
Avgerinos A.,
Kapetanos D.,
Kapetis E.,
Ilias A.,
Kokozidis G.,
Kitis G.,
Xiarchos P.,
SUMMARY
Hereditary coproporphyria is a rare hepatic porphyria with
symptoms similar to acute intermittent porphyria. Photosensitivity
is not described in the latter. We report the case
of a young female with deep jaundice, photosensitivity, anemia,
renal failure and no abdominal pain. Hereditary coproporphyria
was diagnosed and the patient was discharged
after 111 days in full recovery. She was readmitted after 45
days with abdominal pain and no jaundice or photosensitivity.
Severe neuropathy developed which caused tetraparesis
and deterioration of the respiratory muscle function.
Haem arginate was administered with gradual improvement
of neuropathy.
Key words: Hereditary corpoporphyria, jaundice, photosensitivity,
neuropathy, haem arginate
(EN)
