The occurrence of hepatoma in the chronic form of hereditary tyrosinemia

Weinberg, Arthur G.; Mize, Charles E.; Worthen, Howard G.

doi:10.1016/s0022-3476(76)80259-4

Cited by 251 publications

(110 citation statements)

References 23 publications

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“…HT is a rare inborn metabolic disease characterized by hepatocellular and renal tubular dysfunction. Affected children usually die before the end of their second year, with hepatocarcinoma as the cause of death in most instances (Weinberg et al, 1976). Notably, these individuals are unable to utilize methionine.…”

Section: -Mc Depletion During Tumour Development 467mentioning

confidence: 99%

5-Methylcytosine depletion during tumour development: An extension of the miscoding concept

Nyce¹,

Weinhouse²,

Magee³

1983

Br J Cancer

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Perhaps the most ubiquitous aspect of tumours and the neoplastic cells of which they are composed is their loss of the ability to control cellular functions in a normal way. During the development of the malignant state, the neoplastic cell becomes increasingly refractory to both the internal and external stimulae which integrate its normal counterparts into functional tissues and organ systems. This lack of integration is associated with alterations in the expression of a host of gene products, including, for example, the ectopic biosynthesis of hormones (

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Section: -Mc Depletion During Tumour Development 467mentioning

confidence: 99%

5-Methylcytosine depletion during tumour development: An extension of the miscoding concept

Nyce¹,

Weinhouse²,

Magee³

1983

Br J Cancer

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“…18 Also, Weinberg and associates reported 37% of HCC in patients who survived beyond age of 2 years old. 19 The yield from Tru-Cut biopsies for preoperative diagnosis of cell dysplasia and HCC was 25% (2 of 8 patients). Dubois and associates studied the imaging (ultrasonography or CT) of 30 patients with HT-1, and were unable to discern the cancerous nodules.…”

Section: Discussionmentioning

confidence: 97%

Untitled

Bahador

Dehghani²,

Geramizadeh³

et al. 2015

Exp Clin Transplant

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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.

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“…The early occurrence of HCC related to HBV in children also may suggest that other factors are involved in the carcinogenesis by HBV. However the early occurrence of HOC has been also documented in children with other cirrhotic or metabolic diseases, usually developing at the age of 1 to 4 year (Okuyama 1965 ;Ishak and Glunz 1967 ;Weiberg et al 1976). The explanation for the early occurrence of HCC related to HBV in childhood is difficult, but growing individuals might have a diathesis of developing HCC in liver injury caused by various origins, or superimposed infections or coexsist metabolic disarrangement might be involved in the occurrence of HCC in childhood.…”

Section: Discussionmentioning

confidence: 99%