Plasma Corticotropin Levels in Patients with
Familial Amyloidotic Polyneuropathy During
Liver Transplantation
JULIAN DIAZ,
1,2
FRANCISCO ACOSTA,
1
TEODOMIRO FUENTE,
1
JERONIMO MORENO,
1
LUIS F. CARBONELL,
2
and PASCUAL PARRILLA
1
1
Liver Transplant Unit, University Hospital “Virgen de la Arrixaca,” Murcia, Spain, and
2
Department of
Physiology, University of Murcia School of Medicine, Murcia, Spain
Introduction
F
amilial amyloidotic polyneuropathy type I (FAP)
is an autosomal dominant inherited disorder and
hereditary amyloidosis. The disease is characterized
by a progressive polyneuropathy affecting both the
peripheral and the autonomic nervous systems. Ner-
vous system affection is typical of FAP and amyloid
deposits can also infiltrate and destroy other organs,
such as kidneys, intestines, eyes, and adrenal
glands. Progressive peripheral and autonomic neu-
ropathy are associated with neural and visceral
deposition of amyloid (1,2). Currently, liver trans-
plantation (LT) is an efficient treatment for this
disease. FAP has previously been incurable, but LT
halts the progression of the disease (3).
The role of the hypothalamic-pituitary-adrenal
axis in stress is well established. -Endorphin and
corticotropin (ACTH) are released into the circula-
tion from the pituitary gland in response to various
stressful stimuli, including pain, surgery, acute
hemorragic, hypotension, hypoxia, and acidosis (4).
Increased concentrations of plasma -endorphin and
ACTH have been observed in non-premedicated pa-
tients before and during surgery (4,5). These studies
demonstrated a direct correlation between stress
and -endorphin and ACTH plasma concentrations
(4,6). Because of the importance of ACTH measure-
ments as a marker of response to various stressful
stimuli, we undertook the current study to describe
the response of plasma ACTH levels in patients with
FAP during LT.
Materials and methods
After Hospital Ethics Committee approval and pa-
tient consent, we studied 28 LT patients divided into
two groups: group A (n = 12), patients diagnosed
with FAP; and group B (n = 16), patients diagnosed
with alcoholic cirrhosis. The diagnosis of FAP was
always based on the following:
1. compatible neurological symptoms and electro-
myographic signs;
2. family history;
3. location of amyloid in abdominal fat and sural
nerve; and
4. finding of the biochemical marker in plasma with
the enzyme-linked immunosorbent assay method.
The marker was TTR-Met-30 in 10 patients and
TTR-Ala-71 in 2 patients. The classification accord-
ing to Sales-Luis et al. (5), which includes neurolog-
ical and electromyographic parameters to evaluate
the severity of polyneuropathy, are expressed in
Table 1.
The anesthetic technique was identical in the 2
groups. Anesthesia was induced with sodium thio-
pental, and succinylcholine; and maintained with an
oxygen/air mixture (FiO
2
= 0.5) and continous infu-
sion of fentanyl, pancuronium bromide, and midazo-
lam; there was no premedication.
Arterial blood samples were collected immediately
before (A
1
) and after anesthesia induction (A
2
); at
the beginning (A
3
) and the end of the preanhepatic
phase (A
4
); at the beginning (B
1
) and the end of the
anhepatic phase (B
2
); at 5 and 60 min after the
beginning of the reperfusion phase (C
1
and C
2
,
respectively); and at the end of the procedure (C
3
).
Blood was collected into 5 mL evacuated tubes
containing ethylenediaminetetraacetic acid solution
as anticoagulant. After centrifugation (2500 g) for
10 min in a centrifuge cooled to 4° C, the superna-
tant plasma was removed carefully within 30 min
Correspondence: Julian Diaz, M.D., C/ Jose Maria
Mortes Lerma, 32 Dpl, Pta 14, 46014-Valencia, Spain.
Manuscript received March 3, 1998; accepted July 31,
1998.
Clinical Biochemistry, Vol. 31, No. 8, 689 – 691, 1998
Copyright © 1998 The Canadian Society of Clinical Chemists
Printed in the USA. All rights reserved
0009-9120/98 $19.00 + .00
PII S0009-9120(98)00068-X
CLINICAL BIOCHEMISTRY, VOLUME 31, NOVEMBER 1998 689