Untitled

Bahador, Abbas; Dehghani, Seyed Mohsen; Geramizadeh, Bita; Nikeghbalian, Saman; Bahador, Mohsen; Malekhosseini, Seyed Ali; Kazemi, Kourosh; Salahi, H

doi:10.6002/ect.2013.0158

Cited by 6 publications

(6 citation statements)

References 21 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…There was 86% tumor-free survival at a median of 6.6 years[ 47 ]. Contrastingly, report from Iran has shown that HCC was present in 5 (23%) of the 22 explants, the diagnosis was confirmed before LT in 2 children[ 48 ].…”

Section: Pathogenic Risk Factorsmentioning

confidence: 95%

Pediatric hepatocellular carcinoma

Khanna

Verma

2018

WJG

110

View full text Add to dashboard Cite

Pediatric hepatocellular carcinoma (HCC) is the second common malignant liver tumor in children after hepatoblastoma. It differs from the adult HCC in the etiological predisposition, biological behavior and lower frequency of cirrhosis. Perinatally acquired hepatitis-B virus, hepatorenal tyrosinemia, progressive familial intrahepatic cholestasis, glycogen storage disease, Alagille’s syndrome and congenital portosystemic shunts are important predisposing factors. Majority of children (87%) are older than 5 years of age. Following mass immunization against hepatitis-B, there has been a drastic fall in the incidence of new cases of pediatric HCC in the Asia-Pacific region. Management is targeted on complete surgical removal either by resection or liver transplantation. There is a trend towards improving survival of children transplanted for HCC beyond Milan criteria. Chemotherapeutic regimens do not offer good results but may be helpful for down-staging of advanced HCC. Surveillance of children with chronic liver diseases with ultrasound and alpha-fetoprotein may be helpful in timely detection, intervention and overall improvement in outcome of HCC.

show abstract

Section: Pathogenic Risk Factorsmentioning

confidence: 95%

Pediatric hepatocellular carcinoma

Khanna

Verma

2018

WJG

110

View full text Add to dashboard Cite

show abstract

“…Hypoglycemia secondary to hyperinsulinism and hypertrophic cardiomyopathy is a less frequent feature [ 9 , 10 ]. The condition, if not treated, progresses into life-threatening liver failure with an increased risk of hepatocellular carcinoma [ 11 – 13 ].…”

Section: Introductionmentioning

confidence: 99%

Hereditary Tyrosinemia Type 1 in Jordan: A Retrospective Study

Megdadi

Almigdad

Alakil

et al. 2021

International Journal of Pediatrics

View full text Add to dashboard Cite

Background. Hereditary tyrosinemia type 1 (HT1) is a recessively inherited inborn error of metabolism affecting the final step of tyrosine catabolism. The accumulation of tyrosine toxic metabolites leads to progressive hepatic, renal, and neurological manifestations. Treatment of HT1 consists of tyrosine-restricted diets and nitisinone. The untreated disease progresses into life-threatening liver failure with an increased risk of hepatocellular carcinoma. Methods. From April 2010 to March 2021, eighteen patients were diagnosed with HT1 in the metabolic department at Queen Rania Al Abdullah Hospital for Children in Jordan. Patients were reviewed retrospectively regarding their clinical features, laboratory data, and sociodemographic history. Results. The mean age of nine boys and nine girls was 6.03 years ( SD ± 3.85 ). The mean age for symptom onset was 5.61 months ( SD ± 6.02 ). However, the diagnosis was belated from the onset by 10.50 months (±10.42). Nitisinone treatment was delayed from diagnosis around 12.28 months ( SD ± 25.36 ). Most of the patients (66.7%) had acute onset of the disease. Two children (11.1%) died due to hepatic complications. Positive family history was identified in 61.1% of patients, and a similar percentage were born to parents with consanguineous marriage. The most common presentation was abdominal pain, vomiting, and fever. Hepatomegaly and abdominal distention were the most common findings. Six patients’ (42.9%) first presentation was rickets. Conclusion. HT1 diagnosis is usually delayed because it is not part of newborn screening and nonfamiliarity with the clinical features of the disease. Therefore, nationwide newborn screening should be expanded to include HT1.

show abstract

“…Most of HCCs in the pediatric age group has been reported in adolescents after 10 years of age, and occurrence of this tumor in young children is almost always associated with an underlying metabolic or viral liver disease (7,8). There have not been any studies regarding HCC in the pediatric age group from Iran.…”

Section: Discussionmentioning

confidence: 96%

“…The most common underlying cause in our center has been tyrosinemia. Tyrosinemia is an important cause of pediatric HCC, especially in the untreated patients (8). However, we had 1 case of PFIC (Progressive familial intrahepatic cholestasis).…”

Section: Discussionmentioning

confidence: 99%

Pediatric Hepatocellular Carcinoma, a Single Center Study from the South of Iran: Case Series

et al. 2017

View full text Add to dashboard Cite

Background: Hepatocellular carcinoma is a rare tumor in children, thus, there are not many studies regarding clinicopathologic findings of this tumor and no report regarding this tumor in this age group from Iran. Objectives: In this study, we tried to find out the clinicopathologic findings including outcome of hepatocellular carcinoma in the patients under the age of 18. Methods: During the last 10 years (2005 -2016), we collected all hepatocellular carcinoma cases in the affiliated hospitals of Shiraz University of Medical Sciences (as the largest referral center in the South of Iran and the only center of pediatric liver transplantation in the whole country) from the archives of Pathology department. Next all clinical data were subsequently extracted from the patients' hospital charts. Results: We collected 30 cases of hepatocellular carcinoma with the age range of 9 months to 18 years with no significant sex preference. All of the patients, except 3, showed abnormal levels of AFP. The most common underlying cause of liver tumorigenesis was type I tyrosinemia. Conclusions: Hepatocellular carcinoma in children is a rare tumor and is mostly secondary to an underlying liver disease, which is tyrosinemia in our center. Hepatitis B related hepatocellular carcinoma is rare in our country, which seems to be related to successful early neonatal vaccination against this viral disease. This tumor has a poor outcome in children and without liver transplantation, has a very short survival rate.

show abstract

Untitled

Cited by 6 publications

References 21 publications

Pediatric hepatocellular carcinoma

Pediatric hepatocellular carcinoma

Hereditary Tyrosinemia Type 1 in Jordan: A Retrospective Study

Pediatric Hepatocellular Carcinoma, a Single Center Study from the South of Iran: Case Series

Contact Info

Product

Resources

About