alpha(1)-Antitrypsin (alpha 1PLT) deficiency is characterized by a hig
h risk of developing pulmonary emphysema anti liver disease. Arteriopa
thy may be associated with alpha 1AT deficiency. We present a patient
with only one kidney and alpha 1AT deficiency, but only mild pulmonary
symptoms and no signs of liver disease, and atraumatic loss of the re
maining kidney. Histological examination of the renal artery showed de
sposits of a mucoid ground substance in the arterial media, as has bee
n demonstrated in other patients with alpha 1AT deficiency. After dial
ysis treatment for 15 months, the patient underwent an uneventful kidn
ey transplantation. The case draws attention to the awareness of compl
ications of alpha 1AT deficiency even in cases with only mild pulmonar
y manifestations of the disorder. To our knowledge, atraumatic loss of
a kidney in a patient with alpha 1AT deficiency has not previously be
en reported.