ABDOMINAL ULTRASONOGRAPHY IN HEPATOLENTICULAR
DEGENERATION
A STUDY OF 33 PATIENTS
EDUARDO LUIZ RACHID CANÇADO MANOEL DE SOUZA ROCHA EGBERTO REIS BARBOSA MILBERTO SCAFF GIOVANNI GUIDO CERRI ALVARO MAGALHÃES HORACIO M. CANELAS
H e p a t o l e n t i c u l a r d e g e n e r a t i o n ( H L D ) a l w a y s e v o l v e s w i t h l i v e r i n v o l v e m e n t in a n y of t h e p o l a r c l i n i c a l p i c t u r e1 0
. T h e r e f o r e , u l t r a s o n o g r a p h y m a y yield n e w i n f o r m a t i o n o n t h e d e g r e e of t h a t d a m a g e a n d a l s o a f f o r d t h e d e t e c t i o n of c o m p l i c a t i o n s s o m e t i m e s u n n o t i c e d t h r o u g h t h e a n a m n e s i s a n d t h e p h y s i c a l e x a m i n a t i o n , s u c h a s a s c i t i s , s p l e n o m e g a l y , a n d c h o l e l i t h i a s i s , c o m p l i c a t i o n s t h a t m a y o c c u r d u r i n g t h e e v o l u t i o n of w h a t e v e r c h r o n i c h e p a t o p a t h y . T h e p r e s e n t s t u d y w a s d e s i g n e d t o e v a l u a t e b y u l t r a s o n o g r a p h y t h e s t r u c t u r a l c h a n g e s of t h e l i v e r , s p l e e n , g a l l b l a d d e r a n d b i l e d u c t s of p a t i e n t s w i t h H L D , c o m p a r i n g t h e f i n d i n g s w i t h t h e c l i n i c a l e v o l u t i o n .
MATERIAL. A N D METHODS
Thirty-three patients with H L D were studied. The, diagnosis was based on the clinical manifestations and laboratory findings. The results were analyzed according to the clinical evolution after D-penicillamine therapy had been started. Twenty patients were males; ages varied from 12 to 49 years. In 4 instances ultrasonography was performed just when the diagnosis was made and before the onset of treatment; in all other patients abdominal ultrasonography w a s performed until 19 years after the diagnosis and until 30 years after the beginning of the symptoms.
The ultrasonographic examinations were made by means of longitudinal and transversal sections of the liver, gall bladder and spleen, using a real and static time equipment with a 3.5 MHz transductor (Toshiba Sac 12).
RESULTS
Table 1 summarizes the hepatic and splenic changes. The changes of hepatic echotexture showed an evolutional development, the mildest being the posterior
Table 1 — Hepatic and splen'.c changes.
COMMENTS
T h e same u l t r a s o n o g r a p h i c a b n o r m a l i t y may r e p r e s e n t different morphologic changes, a s well a s a morphologic c h a n g e m a y be expressed by several w a y s in the u l t r a s o n o g r a p h y . Therefore, increased echogenicity, the attenuation of the posterior acoustic beam and the loss of details of vascular s t r u c t u r e s inside the hepatic p a r e n c h y m a point indistinctly to the presence of steatosis or fibrosis 1,3,4,9,11,12. A m o n g abnormalities found in this study, c h a n g e s of the hepatic echotexture w e r e the most frequent. T h e y indicate the presence of steatosis or fibrosis, but both m a y coexist in HLD. Only a patient showed h e p a t o m e g a l y while 10 ( 3 0 . 3 % ) showed decrease of the liver size, especially of the right lobe. One could a t t e m p t to explain the small incidence of h e p a -tomegaly emphasizing t h a t the u l t r a s o n o g r a p h y w a s made after the onset of D-penicillamine treatment, when the inflammatory and steatotic processes are in regression and the fibrosis must be established. However, the only patient with h e p a t o m e g a l y w a s in t r e a t m e n t for 5 years, with stable clinical picture. In the 4 patients w h e r e u l t r a s o n o g r a p h y w a s performed before the onset of t h e r a p y the liver size w a s reduced in two and w a s normal in the remainder. P e r h a p s the absence of h e p a t o m e g a l y even in t h a t s t a g e of the disease could be due to delay in the d i a g n o s i s of HLD, usually four y e a r s after the s y m p t o m s s t a r t e d . On the c o n t r a r y , in studies with prevalence of alcoholic liver disease — in spite t h a t the time of evolution h a s not been considered — h e p a t o m e g a l y w a s very frequent 3,12. C o n s e q u e n t l y other factors besides the time of diagnosis could be responsible for the r a r i t y of h e p a t o m e g a l y in HLD and the significant number of p a t i e n t s ( 3 0 . 3 % ) with decrease of the liver size. A m o n g these factors is the possibility t h a t copper h a s a g r e a t e r fibrogenic activity t h a n other hepatotoxic a g e n t s .
U l t r a s o n o g r a p h y is not infallible in the exclusion or in the diagnosis of an existing liver disease, be it acute or chronic 1,2,3,4,9,11,12. it is usually admitted t h a t an a b n o r m a l u l t r a s o n o g r a p h y in a case with a clinical suspicion of chronic liver disease is a finding which t e n d s s t r o n g l y to confirm the illness. On the other hand, the absence of u l t r a s o n o g r a p h i c c h a n g e s in the liver does not exclude a h e p a t o p a t h y , not even cirrhosis 9. T h i s can be illustrated by the finding of 3 absolutely normal u l t r a s o n o g r a p h i e s in our series, including a patient w h o s e liver biopsy showed cirrhosis when the d i a g n o s i s of HLD w a s made, two y e a r s before.
T h e incidence of cholelithiasis in cirrhotic p a t i e n t s is high, p r o b a b l y in view of the increase in the c o n c e n t r a t i o n of n o n - c o n j u g a t e d bilirubin in bile, either c a u s e d by an i n c r e a s e in its p r o d u c t i o n by hemolysis, or by d e c r e a s e of its c o n j u g a t i o n in the h e p a t o c y t e . W h e n the c o n c e n t r a t i o n of indirect bilirubin rises it w o u l d occur an imbalance a m o n g the solid c o n s t i t u e n t s of bile, inducing the formation of a calcium bilirubinate calculus, a type of lithiasis related to t h a t s i t u a t i o n and to chronic hemolysis 7. In the p r e s e n t s t u d y an increase of the p r e v a l e n c e of cholelithiasis w a s found in females, with an a v e r a g e of 46.2% a n d a t r e n d to rise with age. In m a l e s the a v e r a g e incidence is the s a m e a s in the g e n e r a l p o p u l a t i o n6
-8
; it must be e m p h a s i z e d , however, t h a t the sole t w o male p a t i e n t s with cholelithiasis had 12 a n d 28 y e a r s of age. In o t h e r s t u d i e s on cholelithiasis in cirrhotic p a t i e n t s the incidence varied from the a b s e n c e of an i n c r e a s e6
-8
to r a t e s a r o u n d 3 2 % ! . 5 .7
; the increase,
however, w a s indistinct a m o n g both s e x e s1
^ a n d in the several a g e r a n g e s1
.
In conclusion, a b d o m i n a l u l t r a s o n o g r a p h y is an i m p o r t a n t p r o p e d e u t i c r e s o u r c e for the e v a l u a t i o n of p a t i e n t s w i t h HLD, i n d e p e n d e n t l y of its clinical form, and is also v a l u a b l e in d e t e c t i n g possible a b d o m i n a l c o m p l i c a t i o n s of the disease.
SUMMARY
T h e u l t r a s o n o g r a p h i c s t u d y of 33 p a t i e n t e s with h e p a t o l e n t i c u l a r d e g e n e -r a t i o n s h o w e d t h e following main c h a n g e s : d i s o -r d e -r s of the hepatic e c h o t e x t u -r e (29 c a s e s ) , c h a n g e s of the splenic d i m e n s i o n s ( 2 1 ) , liver s h r i n k a g e ( 1 0 ) , cholelithiasis ( 8 ) , h e p a t o m e g a l y a n d ascitis ( 1 ) . T h e d i s o r d e r s of liver echo¬ t e x t u r e exhibited different p a t t e r n s , from slight to severe c h a n g e s of the hepatic echogenicity, a s s o c i a t e d with a n a t o m i c d i s t o r t i o n s of the liver, such a s a l t e r a t i o n s of outline a n d d e c r e a s e of dimensions. Liver s h r i n k a g e w a s a l w a y s a c c o m p a n i e d by s p l e n o m e g a l y . T h e s c a r c i t y of h e p a t o m e g a l y m a y be explained by t h e fact t h a t the u l t r a s o n o g r a p h y w a s p e r f o r m e d after the o n s e t of t r e a t m e n t with penicillamine, or by othe r f a c t o r s still u n k n o w n , c o p p e r p e r h a p s p o s s e s s i n g a s t r o n g e r fibrogenetic action t h a n othe r h e p a t o t o x i c a g e n t s . Cholelithiasis w a s very frequent in females (6 of 13 p a t i e n t s ) , its incidence t e n d i n g to i n c r e a s e with age. R e g a r d i n g m a l e s t h e r e w a s no i n c r e a s e w h e n the incidence w a s c o m p a r e d to the g e n e r a l p o p u l a t i o n . T h e t w o sole m a l e s w e r e y o u n g , an u n u s u a l finding in n o r m a l men.
RESUMO
Uitra-sonografia abdominal na degeneração hepatolenticular: estudo de 33 casos.
O e s t u d o u l t r a s o n o g r á f i c o de 3 3 p a c i e n t e s com d e g e n e r a ç ã o h e p a t o l e n t i cular revelou a s s e g u i n t e s a l t e r a ç õ e s p r i n c p a i s : d e s o r d e n s d a e c o t e x t u r a h e p á -tica (29 c a s o s ) , a l t e r a ç õ e s d a s d i m e n s õ e s e s p l é n i c a s ( 2 1 ) , c o n t r a ç ã o do fígado
c o m o m o d i f i c a ç õ e s d o p e r f i l e r e d u ç ã o d a s d i m e n s õ e s . A c o n t r a ç ã o h e p á t i c a s e m p r e s e a c o m p a n h o u d e e s p l e n o m e g a l i a . A r a r i d a d e d a h e p a t o m e g a l i a p o d e s e r e x p l i c a d a p e l o f a t o d e q u e a u l t r a - s o n o g r a f i a foi r e a l i z a d a a p ó s o i n í c i o d o t r a t a m e n t o c o m p e n i c i l a m i n a , o u p o r o u t r o s f a t o r e s a i n d a d e s c o n h e c i d o s , c o m o a p o s s i b i l i d a d e d e q u e o c o b r e p o s s u a u m a a ç ã o f i b r o g ê n i c a m a i o r d o q u e a d e o u t r o s a g e n t e s h e p a t o t ó x i c o s . A c o l e l i t í a s e foi m u i t o f r e q ü e n t e n o s e x o t e m i n i n o ( 6 d e 13 p a c i e n t e s ) e s u a i n c i d ê n c i a t e n d e u a c r e s c e r c o m a i d a d e . Q u a n t o a o s d o e n t e s d o s e x o m a s c u l i n o , n ã o s e n o t o u a u m e n t o d a i n c i d ê n c i a e m r e l a ç ã o à f r e q ü ê n c i a n a p o p u l a ç ã o g e r a l . O s d o i s ú n i c o s p a c i e n t e s d o s e x o m a s c u l i n o e r a m j o v e n s , f a t o r a r a m e n t e o b s e r v a d o n o s h o m e n s n o r m a i s .
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