COPPER A N D CERULOPLASMIN CONTENTS I N T H E BLOOD
SERUM OF PERIPHERAL A N D PRE-HEPATIC V E I N S
H . M . C A N E L A S
I . R O I T M A N
M . S C A F F S . R A I A
F . B . D E JORGE
Several questions about the pathogenesis of hepatolenticular degeneration ( H L D ) remain unsolved, despite the extraordinary results achieved by the numerous researches carried out, mainly with radioactive c o p p e r9
.1 1 .1 2
. F o r instance, the low copper content of the stools, formerly ascribed to an increased intestinal absorption16
, is now regarded as a consequence of a de-fective biliary excretion5
.10 .
However, although some facts 5 >6
-8
seem to question the significance of the impaired synthesis of ceruloplasmin, the investigations with radiocopper12 and other arguments12
.13 .15
apparently support it.
In an a t t e m p t to contribute for the unraveling of the problem, the con-centrations both of copper and ceruloplasmin have been determined in samples of peripheral and pre-hepatic venous blood f r o m 11 patients submitted to portal decompression.
M A T E R I A L A N D M E T H O D S
D u r i n g the p e r f o r m a n c e of either p o r t o - c a v a l or spleno-renal shunt in 1 1 patients with Manson's hepato-splenic schistosomiasis, blood samples from t h e superior mesenteric and basilic veins were c o l l e c t e d .
Ceruloplasmin a n d copper contents were determined according to H o u c h i nT and a modified Gubler m e t h o d3
, respectively. D u e care w a s taken in order to prevent copper c o n t a m i n a t i o n of the m a t e r i a l s .
The results obtained in the s a m p l e s of peripheral blood, compared to n o r m a l v a l u e s2
,4
, showed increased copper contents in 6 cases, w h i l e all ceruloplasmin levels fitted in the n o r m a l r a n g e . I n these cases, the increase of the direct reacting copper m a y be due to t h e liver d a m a g e caused by schistosomiasis.
For c o m p a r a t i v e purposes, the s a m e study w a s done in a patient with H L D submitted to p o r t o - c a v a l shunt owing to a severe bleeding of esophageal varices.
T h e results w e r e submitted to statistical t r e a t m e n t , by m e a n s of the analysis of the individual differences in t h e copper and ceruloplasmin contents of both blood samples
F r o m the D e p a r t m e n t s of N e u r o p s y c h i a t r y (Division of N e u r o l o g y ) and Surgery ( 3r d
R E S U L T S
T h e results obtained in the 11 s c h i s t o s o m a patients are assembled in table 1. In the patient w i t h W i l s o n ' s disease no ceruloplasmin could be demonstrated in a n y of the samples, by the m e t h o d e m p l o y e d . On t h e other hand, determination of the total copper content showed 3 0 / ¿ g / 1 0 0 in the basilic vein blood, 3 0 in v e n a cava, 2 2 in the hepatic vein and 1 0 in the portal v e i n .
D I S C U S S I O N
T r i p1 4
, using vascular catheterization, has determined the blood copper and ceruloplasmin contents in the brachial artery and in the hepatic and subclavian veins of 37 patients. Of the 37 patients, 25 had liver cirrhosis or other hepatic pathology, and 12 had heart diseases. Total blood copper and ceruloplasmin contents in the brachial artery were lower than in the hepatic vein. F r o m his investigations, the author drew the conclusion that the ceruloplasmin in synthesized in the liver as fraction I ( C - C ) , which is carried to the tissues by the blood stream, part of it being inactivated or destroyed, part transformed in fraction I I ( C - D ) , and part staying in the blood as fraction I.
Our results, arising from an essentially different approach than Trip's, suggest that the copper content in the blood from the small bowel is not higher than that of the periperal blood. T h e same result was obtained regarding ceruloplasmin *, probably because the mesenteric blood contains an appreciable amount of elements coming from the general circulation. T h e -refore, the mesenteric blood differs in this regard from lymph, which contains no c e r u l o p l a s m i n1 4
.
In the patient with H L D the copper content in the portal vein was markedly lower than in vena cava and the basilic vein (10 /¿g/100 m l instead of 3 0 ) . This seems to indicate that, in Wilson's disease, even a decrease in the intestinal absorption of copper can be observed.
S U M M A R Y
Copper and ceruloplasmin contents were determined in samples of peri-pheral and pre-hepatic venous blood of 11 patients with Manson's schistoso-miasis and one patient with hepatolenticular degeneration, all of çhich sub-mitted either to porto-caval or spleno-renal shunt. Individual difference were not significant in any of the non-Wilsonian patients. T h e results are discussed in regard to the current knowledge on the pathogenesis of Wilson's disease.
R E S U M O
Concentrações de cobre e ceruloplasmina no soro sanguíneo
de veias periférica e pré-hepática
Foram determinadas as concentrações de cobre e ceruloplasmina em amostras de sangue venoso periférico e pré-hepático de 11 pacientes esquis-tossomóticos e de 1 paciente com degeneração hepatolenticular, submetidos a anastomose portocava ou esplenorrenal. N o s 11 pacientes não wilsonianos, as diferenças individuais não se revelaram estatisticamente significantes. Os resultados são comentados em relação aos conhecimentos atuais sobre a pa-togenia da moléstia de Wilson.
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