Tyrosinemia type I treated by NTBC: How does AFP predict liver cancer?

Koelink, Cecile; Hasselt, Peter van; Ploeg, A. van der; Heuvel‐Eibrink, Marry M. van den; Wijburg, Frits A.; Bijleveld, Cma; Spronsen, F. J. van

doi:10.1016/j.ymgme.2006.07.009

Cited by 58 publications

(51 citation statements)

References 18 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…5,6 Signs suggestive of HCC in the individual HT1 patient are a slow decrease to normal in AFP values; AFP not reaching normal values; an increase in AFP levels; and/or a new lesion found on imaging. 3,[5][6][7] In other diseases with a high risk of developing HCC (eg, hepatitis B and C), up to 44% of patients diagnosed with HCC show no clear increase in AFP levels. 8,9 Until now, all reported HT1 patients with proven HCC had exhibited a well-defined rise in AFP levels and a clear lesion at imaging.…”

Section: Discussionmentioning

confidence: 99%

“…6 She had hypotonia, an enlarged abdomen due to hepatosplenomegaly, and rickets. Laboratory investigations showed a blood tyrosine level of 335 mmol/L, an AFP level of 528 568 mg/L, and a urinary succinylacetone concentration of 148 mg/mmol of creatinine.…”

Section: Case Reportmentioning

confidence: 99%

“…At that time, AFP concentrations had stabilized around 24 mg/L (Fig 1), wheras a further decrease was expected. 6 More specific characterization of the lesion was performed by using multiphase contrast-enhanced CT scanning. The scans revealed 2 lesions (15 3 15 mm each), with no arterial enhancement and with hypodensity in the venous phase.…”

Section: Case Reportmentioning

confidence: 99%

“…[1][2][3][4] However, NTBC-treated patients with HTI are at increased risk of developing HCC. 5,6 Patients with a late initiation of NTBC due to delayed diagnosis or unavailability of NTBC, a slow decrease of a 1 -fetoprotein (AFP), or an AFP level that remains just above the normal range of 0 to 10 mg/L have an increased risk of developing HCC. 3,[5][6][7] In HTI, early detection of HCC is based on routine follow-up of AFP levels and liver imaging.…”

mentioning

confidence: 99%

See 3 more Smart Citations

Hepatocellular Carcinoma in Tyrosinemia Type 1 Without Clear Increase of AFP

Ginkel¹,

Gouw²,

Jagt

et al. 2015

PEDIATRICS

View full text Add to dashboard Cite

Section: Discussionmentioning

confidence: 99%

Section: Case Reportmentioning

confidence: 99%

Section: Case Reportmentioning

confidence: 99%

mentioning

confidence: 99%

See 2 more Smart Citations

Hepatocellular Carcinoma in Tyrosinemia Type 1 Without Clear Increase of AFP

Ginkel¹,

Gouw²,

Jagt

et al. 2015

PEDIATRICS

View full text Add to dashboard Cite

“…9 Increases in the levels of AFP, or slow decreases in their levels, or non-normalizing concentrations of AFP after NTBC treatment are important predictors of HCC development. 10 In a study on 12 patients with HT-1, lectin-reactive AFP is an additional marker that may help distinguish HCC from benign liver disease. 11 There is no consensus between the transplant centers on the precise time a child with HT-1 should receive a liver transplant.…”

Section: Discussionmentioning

confidence: 99%

Untitled

Bahador

Dehghani²,

Geramizadeh³

et al. 2015

Exp Clin Transplant

View full text Add to dashboard Cite

Objectives: This study sought to determine the prevalence of hepatocellular carcinoma and other premalignant lesions in children with hereditary tyrosinemia type 1 who had undergone an orthotopic liver transplant at the Shiraz Transplant Center, in Shiraz, Iran. Materials and Methods: Between September 2006, and June 2011, thirty-six patients with hereditary tyrosinemia type 1 received a liver transplant from a deceased (whole or split) or a living-related donor. Clinical records and pathologic specimens, before and after surgery, for each case were reviewed. In addition, ultrasound, abdominal computed tomographic imaging scan findings, and levels of alpha-fetoprotein were recorded. Results: Twenty-two patients with hepatic nodules larger than 10 mm underwent a Tru-Cut needle biopsy before their liver transplant. In 2 patients, a diagnosis of hepatocellular carcinoma was made by pathologic examination; in the other 20, cirrhosis was confirmed with no evidence of malignancy. After pathologic examination of the explanted livers, the largest nodules in the 36 patients were 35 mm. Five cases had at least 1 nodule of hepatocellular carcinoma. Three of the other patients had small cell dysplasia in some of nodules. All 5 cases with hepatocellular carcinoma were patients older than 2 years of age (19 patients were older than 2 years of age). All patients with hepatocellular carcinoma received pretransplant nitisinone treatment. All patients with hepatocellular carcinoma after their liver transplant are alive at the time of this writing. Conclusions: The prevalence of cell dysplasia and hepatocellular carcinoma in children with hereditary tyrosinemia type 1 in our study is not as high as that reported previously, so it appears that patients older than 2 years of age require a liver transplant.

show abstract