Prognostic implication of hepatopulmonary syndrome in pediatric patients

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[ C02/25

I c0 27

I

ERYTHROCYTE CREATINE AS A NON-ISOTOPE HYPERSPLENISM IN CIRRHOSIS

INDICATOR OF

T. Saibara, T. O k u m i y a , Y. Jiao, T. Sugiura, M. Ono, Y. M a t s u u r a , S. lwasaki, T. M a e d a . S. Onishi 1st D e p t o f M e d a n d D e p t Clin L a b M e d , K o c h i M e d i c a l School, N a n k o k u 783-8505, J a p a n .

Anemia is a common feature in liver cirrhosis and hypersplenism has been suggested to be responsible. Up to now, kinetic analysis of red blood cell was performed by administrating chromiuna labeled autologous cell and there is

no substitutional non-isotope analytical methods. Recently we have established an EIA of erythrocyte creatine, which is thought to be a sensitive parameter of erythrocyte age, and proved that erythrocyte creatine is significantly increased in hemolytic anemia and mydodysplastic syndrome with severecopper deficiency (Lancet 353:842-843, 1999). In this study, we applied this method to assess hypersplenism in liver cirrhosis. We measured erythrocyte creatine in fifty postnecrotic cirrhotic patients (20 women and 30 men; aged 3884 years). Erythrocyte creatine showed significant correlations with hemoglobin (r =-0.48; p 40sqcm) (1.83+PO.86mmol/g hemoglobin, n--37) than in those without splenomegaly (SI~40sqcm) (1.27 +1-0.32 mmol/g hemoglobin, n=13) (p<0,002). These data indicated that erythrocyte creatine is strongly associated with hypersplenism. These data suggested that erythrocyte creatine could be a nonidotope indicator of hypersplenism, and of erythropoietic status in liver cirrhosis.

I

FREQUENT REBLEEDING, ENCEPHALOPATHY AND MORTALITY FOLLOWING TRANSJUGULAR INTRAHEPATIC PORTOS Y S T E M I C S H U N T ( T I P S ) I N C H I L D C L A S S C C I R R H O T I C PATIENTS W.-Y. C h a n ~ I, Y.-H. C h u n g I, S.H. Y a n g ~, B.-C. S o n g l, H.C. Lee 1, H . K . Yoon 2, K.B. S u n g 2, Y.S. Lee 1, D.J. Suh l, ~ D e p a r t m e n t o f I n t e r n a l Medicine, U n i v e r s i t y o f U l s a n , College o f M e d i cine, A s a n M e d i c a l Center, Seoul, K o r e a . Z D e p a r t m e n t o f D i a g n o s t i c Radiology, U n i v e r s i t y o f U l s a n , College o f Medicine, A s a n M e d i c a l Center, Seoul, K o r e a . Although TIPS has been reported to be effective in the treatment of varioeal hemorrhage in patients with liver cin'hosis (LC), shunt failure and hepatic encephalolmthy (HE) as well as mbleeding following the procedure frequently limit its therapeutic efficacy and eventually the survival periods of patients. In this retrospective study we were to evaluate the effectiveness of TIPS in the management of recurrent or refractory variocd bleeding in terms of rehieodlngand the survival of patients, and also to assess the clinical and h~nodynamic predictors of mortality, shunt failure, rebleeding and HE following TIPS. Thirty-live cinlietic patients (Child A: 9, B: 16, C: I0) were subjected to emergency (n = 19) or elective (11= 16) TIPS for the control of variceal bloc(ling. Mean follow-up period was 16 months. TIPS was sucoessfolly o~'npletod in 33 out of 35 patients (94%), and the variceal bl¢odings were controlled suo~asfidly in 29 out of 33 patients (88%). Portasyaternic pressme gradients were decreased ~om 26.4 :t: 11.9 to 14.3 + 10.6 nmaHg following TIPS. Rehi¢¢ding occurred in 18 patients (62%); in 21% of patiants at 30 days, 42% at I year, and 51% at 2 year after TIPS. Rdol¢odings were more fi'¢quent among paticats in Child-Pugh class C (p = 0.014) and thusc Ireatod with crae~cncy procedure (p=0.022). Hepatic enc¢ldmlopathywas present in 39% of patients (13 of 33), mostly within l month aflor TIPS (27%, 9 of 33). Si~ificant and independent predictors of HE were the reduction in portusystamic pressure gradients > 12 mm Hg (p = 0.041) and ~ Child-Pugh score (p ffi0.017). Shunt dysfunction ocosrred in 8 patients (24%); the actual probability of dysfonction was 18% at 1 year and 21% at 2 y~r. None of the variables mmlyzed predicted shtmt dysfunction. Cumulative survival fetes were 77% at I month, 61% at I year, and 53% at 2 year after TIPS. indapendent prognostic voriabtes predicting early death were the ChildPugh score (p < 0,001), the prasenc¢ of rebl¢oding within 1 month following TIPS (p = 0.011) and the emergen~ procodore (p = 0.018). In conclusion, it is suggested that LC patients in Child-Pugh class C may not be good candidates for TIPS in the treatment of variaud bleeding because of frequ~t rebleeding and encel2udopathy following the procedure. Tberefore, in patients with high Child-Pugh score, liver mmsplantation should be conside~'~ earlier after the control of active vark:enl bleeding.

I C02/28

I

PROGNOSTIC IMPLICATION OF DROME IN PEDIATRIC PATIENTS

HEPATOPULMONARY

SYN-

0 . D . Siio~lu, B. Elkabes, G. Saner, S. S6k0cti D e p a r t m e n t o f Pediatric G a s t r o e n t e r o l o g y a n d H e p a t o l o g y , I.U. I s t a n b u l F a c u l t y o f Medicine, Istanbul, Turkey. Hepatopulmonery syndrome is a serlous complication of intrahepotic or extrshepotiG portal hypertension. It is ao=epted se an Indt~tlon for liver transplantation in olrrholic patients. Although a few cases of spontaneous regression have been reported in adult patients, data about natural history of hepatopulmonary syndrome with varying degrees of hypoxemla Is scares both in adults and podiatric patients. 9 pediatric patients with hepatopulmonery syndrome (HPS) diagnosed in a three yearn period at our pedistric gastroenterology and hepotolegy department were reviewed In order to evaluate the natural hiatory of HPS and the impact of degree of hypoxemla on prognosis, Dlegncais of HPS was based on increased siveoloarterlsi gradient and demon~xalton of dght to leR shunt by contrset enhanced echocardiogram and/or lung porfcalon acan in the sbsenoe of intrinsic pulmonary and cardiac disease. Mean follow up podod was 15.4 + tt.1 months (range 1.-36 months). Characteriatk== of patient= are seen in the Table. Ntlent no 1 2

Dilir, enll

~

Follow up

¢oume

K~"

A-e

imdieet*

IXXlod-

16/F 11/M

55 ,~

S8 S8

36 monthl 8 mor~s

Stld~ Died

Sex PVT Budd

Chlsrd 3

II

S~"

54

66

4~s

WD

16/M

73

36

CC

9iF

II=t'l

14/F

EHBA AIH CC

1/M 10/F 11/F

50 54 64 45 67

51 55 40 84 52

12 rnomhs 12 months e manths ISmonths

Died ReDressed Stable Stable Stable

1 month 12 m~ths

Died Stable

GSDW

*At the time of dlagnmd~ while Ix~thing room mir rand in ~tilnti lanl~m; --Aftlr the beginning of ~.ymptems of HPS; PV'r: ~mmbenis of the ~ vein; GSD IV: glycogen mmge diesels type IV; WD: Wltsen disease; CC: o r ~ n i ¢ eirrhoUt; IPH: idiopeti¢ podal hE~tor.don; EHBA:extmhepati¢blhry strolls; AIH: mJ~immum,h e ~ . In our smell sample of podiatric petinnts we obNrved that 3 (30%) of the children with HPS died In the first 6 months alter the beginning of the symptoms while 5 (50 %) of them remained stable with mlnmsl limitation of dally activities during a 12-36 months poriod. Hypoxemis and Intrapulmonary shunts had regressed in a boy after successful treatment of WIlacn disease.

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