Dipresentasikan oleh : Roro Rukmi Windi Perdani Etiology and Complication of Liver Cirrhosis in Children : Report of a Single Center from Southern Iran Sayed Mohsen Dehghani, Mohammad Hadi Imanieh, Mahmood haghihat, Abdorrasoul Malekpour, Zeinab Falizkar Middle East Journal of Digestive Disease.2013;5:41-6 Pembimbing : Dr. I. Hartantyo, SpAK Dr. Ninung Rose DK, M.Si.Med., SpAK
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Dipresentasikan oleh :Roro Rukmi Windi Perdani
Etiology and Complication of Liver Cirrhosis in Children :Report of a Single Center from Southern Iran
Sayed Mohsen Dehghani, Mohammad Hadi Imanieh, Mahmood haghihat, Abdorrasoul Malekpour, Zeinab Falizkar
Middle East Journal of Digestive Disease.2013;5:41-6
Pembimbing :Dr. I. Hartantyo, SpAK
Dr. Ninung Rose DK, M.Si.Med., SpAK
Introduction
Cirrhosis in children
is a diffuse process characterized by progressive hepatic fibrosis, distortion of the hepatic architecture and formation of regenerative nodules
uncommon in the pediatric age group
Chronic cholestasis, inborn errors of metabolism and chronic hepatitis are the main causes
83 children: biliary atresia
(27.7%), cryptogenic cir-rhosis (24.1%)
cryptogenic cirrhosis and
autoimmune hepatitis
Introduction ...(2)
jaundice
ascites
GI variceal bleedin
g
hepatic encephalopat
hy
edemspontaneo
us bacterial
peritonitis
edem
Hepatopulmonary and
hepatorenal syndrome
Complication of cirrhosis
hepatis
Material and Method
All cirrhotic children aged less than 18
years who re-ferred to an outpatient
pediatric gas-troenterology clinic affiliated
with Shiraz University of Medical Sci-ences be-
tween March 2009 and Sep-tember 2010
● Clinical examination● Data form contain : de-mographic, clinical and paraclinical data
• “Model for End-stage Liver Disease (MELD) : > 12 years
Jaundice was diagnosed clinically as yellowish discoloration of sclera and skin
Ascites was diagnosed according to physical findings such as abdominal flank bulge with fluid, fluid wave, shifting dullness, a ballotable liver and spleen, umbilical and inguinal hernia and ultrasonographic evidence of ascites
Hepatic encephalopathy was diagnosed clinically as irritability, lethargy, acute change in mental status, neurodevelopmental delay, school problems, sleep reversal, ataxia, and tremors
Esophageal varices were diagnosed endoscopically
Abdominal paracentesis with polymorphonuclear cells more than 250/mm3 was considered to be diagnostic of spontaneous bacterial peritonitis
Hepatopulmonary syndrome was diagnosed as arterial oxygen pressure of <70 mmHg in room air with alveolar/arterial gradient of >20 mmHg
Complications of cirrhosis
Material and Method...(4)
Analisis
ANOVA , student’s t-test SPSS
Result
106 subjects ▪ a mean age of 8.24 ± 6.12 years (range: 5 months to 18 years)
▪ 60 cases were boys (56.6%) and 46 were girls (43.4%)
▪ mean PELD/MELD scores were 14.2 ± 11.9 (range: 6-48) ▪ mean Child-Pugh score was 8.1 ± 2.2 (range: 5-13)
▪ 27% : grade A▪44% : grade B ▪ 29% : grade C
Most common complication of cirrhosis hepatis▪ jaundice (n=72; 67.9%)▪ ascites (n=47 ; 44.3%)▪ Non-bleeding esophageal varices (n= 32 children; 30.2%) ▪ gastrointestinal variceal bleeding (n= 17 ; 16.1%)▪ hepatic encephalopathy (n=13; 12.7%)▪ edema (n=8; 7.5%)▪ spontaneous bacterial peritonitis (n=5; 4.7%) ▪ hepatopulmonary syndrome (n=5; 4.7%)▪Hepatorenal syndrome was not present in any children with liver cirrhosis▪ no complication (n=19 ;17.9%)
Most common causes cof irrhosis hepatis▪ Wilson disease (n=22; 20.7%)▪ biliary atresia (n=19; 17.9%)▪ cryptogenic cirrhosis (n=14; 13.2%)▪ Other causes were autoimmune hepatitis (n=12; 11.3%)▪ idiopathic neonatal hepatitis (n=10; 9.4%)▪ Hepatorenal tyrosinemia (n=9; 8.5%)▪ glycogen storage disease (n=6; 5.7%)▪ progressive familial intrahepatic cholestasis (n=4; 3.8%)▪ paucity of bile ducts (n=3; 2.8%)▪ cardiac cirrhosis, choledocal cyst and primary sclerosing cholangitis (2 cases for each one; 1.9%)▪ Caroli’s disease in one child (0.9%)▪ infection (n=0; 0%)
Result...(2)
Result...(3)
Discussion...(3)
The most common causes of liver cirrhosis in this study were Wilson disease (20.7%), biliary atresia (17.9%), cryp-togenic cirrhosis (13.2%), and autoimmune hepatitis (11.3%)
might be because it has become an active pediatric liver transplant center in the Middle East region with more than 70 pediatric liver transplants her parts of the country
In a recent study from Iran the most common hepatic diseases among inpatients were hepatitis B virus (HBV) and cryptogenic or non-alcoholic fatty liver disease induced cirrho-sisIn another study from Southern Iran HBV infection was the ma-jor cause for cirrhosis and ascites was the most common complication in adult patients
Other study :Tunisian study (36 girls and 35 boys):
most frequent clinical signs : jaundice and hep-atomegaly
Other etiology : hepatorenal tyrosinemia, Wilson disease (17%) and post-hepatitis cirrhosis (17%)
No definite etiology : 27% of cases
because of the lack of availability of some spe-cific tests
In Iran : main indications for liver transplantation in chil-dren : Wilson disease (20.3%), cryptogenic cirrhosis (16.7%), autoimmune cirrhosis (14.5%)
agreed with the results of this study.
Bernard: chronic cholestasis starting in early infancy, chronic hepatitis, and metabolic disorders : main causes of cirrhosis in children
recommend : precise identification of the cause for effective
therapy close follow-up is necessary for prolonging life and
to prevent diagnose and manage complication on time if liver
Chronic liver diseases : post-hep-atitic cirrhosis (13%), Wilson dis-ease (21%), autoimmune (4%), and non-Wilsonian metabolic dis-eases (16%)
Northern India
Argentina
Brazil
Oman
Other studyMost common cause of chirrhosis hepatis metabolic disorders,
cholestatic syndrom, autoim-
mune hepatitis
most common indications for liver transplantation : biliary atresia (30%), fulminant he-patic failure ( 27%) and au-toimmune cirrhosis (16%)
Most common causes of cirrhosis hepatis :
biliary atresia (50%), autoimmune disorders (20.5%) and cryptogenic (17.6%)
most common causes of liver cirrhosis :
progressive familial intrahep-atic cholestasis (30), fibrocys-tic diseases of the liver and kidneys (21%)
Discussion ...
Japan : (33379 patients with liver cirrhosis at 58 hospitals) :most common causes : hepatitis B virus (13.9%), hepatitis C virus (60.9%), alcohol (13.6%), primary biliary cirrhosis (2.4%) and autoimmune hepatitis (1.9%)
In this center : (Saberifiroozi et al.): 480 adult patients (mean age: 39 ± 13years; 68.1% men) on the liver transplantation, most common causes : cryptogenic (143; 29.9%) and hepatitis B virus (127; 26.5%)
These studies : different causes of liver cirrhosis in children and adults
as the most common cause of liver cirrhosis in adults : viral hepatitis.
in the current study : no cases of viral hepatitis
Cirrhosis hepatis
GI bleeding
Jaundice
Hepatic encephalopathy
Ascites
Bacterial infections
Complication
Conclusion
This study concluded that metabolic disorders (Wilson disease, hepa-torenal tyrosinemia), cholestatic syndromes (biliary atresia, idio-pathic neonatal hepatitis, progressive familial intrahepatic cholestasis), and autoimmune hepatitis : most common causes of cirrhosis in children
Early diagnosis and determining the common causes of cirrhosis effective treatment and decreasing the rate of complications and mortality
EVIDENCE BASED MEDICINE
RESEARCH QUESTION
to report the causes of liver cirrhosis and evaluate its complications in children
POPULATION : seluruh penderita sirosis hepatiis pada anak berusia kurang dari 18 tahun yang dirujuk ke klinik rawat jalan gastroenterologi yang berafiliasi dengan Fakultas Kedokteran Universitas Shiraz pada Maret 2009 hingga September 2010