Iron Overload in the Liver of 2 Children: Nonalcoholic Steatohepatitis and Juvenile Hemochromatosis

Background: Iron overload disorders are hereditary hemochromatosis and secondary etiologies other than hereditary hemochromatosis. We describe 2 boys presenting with iron overload. Juvenile hemochromatosis and nonalcoholic steatohepatitis (NASH) related iron overload are the genetic and secondary causes, respectively. Observations: Both patients benefited from phlebotomy even if they had different etiologies. Conclusions: In childhood, the diagnosis of iron overload syndromes is crucial because they do not confront us with obvious symptoms and findings. Early initiation of a phlebotomy program can prevent mortality. NASH might lead to iron overload and iron overload might aggravate the clinical course of NASH.

[1]  A. Pietrangelo.  Genetics, Genetic Testing, and Management of Hemochromatosis: 15 Years Since Hepcidin. , 2015, Gastroenterology.

[2]  M. Tosun,et al.  Serum hepcidin levels and iron metabolism in obese children with and without fatty liver: case–control study , 2014, European Journal of Pediatrics.

[3]  M. Jaganjac,et al.  Progressive pigmented purpuric dermatitis and alopecia areata as unusual skin manifestations in recognizing hereditary hemochromatosis. , 2012, Acta dermatovenerologica Croatica : ADC.

[4]  A. Pietrangelo,et al.  Iron and steatohepatitis , 2012, Journal of gastroenterology and hepatology.

[5]  J. Krieger,et al.  Molecular Diagnostic and Pathogenesis of Hereditary Hemochromatosis , 2012, International journal of molecular sciences.

[6]  M. Hulihan,et al.  Uric Acid as a Potential Cue to Screen for Iron Overload , 2011, The Journal of the American Board of Family Medicine.

[7]  B. Bacon,et al.  Diagnosis and Management of Hemochromatosis: 2011 Practice Guideline by the American Association for the Study of Liver Diseases , 2011, Hepatology.

[8]  C. Camaschella,et al.  Inherited disorders of iron metabolism , 2011, Current opinion in pediatrics.

[9]  L. N. Valenti,et al.  Venesection for non-alcoholic fatty liver disease unresponsive to lifestyle counselling--a propensity score-adjusted observational study. , 2011, QJM : monthly journal of the Association of Physicians.

[10]  Youming Li,et al.  High Serum Uric Acid Increases the Risk for Nonalcoholic Fatty Liver Disease: A Prospective Observational Study , 2010, PloS one.

[11]  Y. Le Marchand-Brustel,et al.  Increased adipose tissue expression of hepcidin in severe obesity is independent from diabetes and NASH. , 2006, Gastroenterology.

[12]  W. Burke,et al.  HFE gene and hereditary hemochromatosis: a HuGE review. Human Genome Epidemiology. , 2001, American journal of epidemiology.

[13]  B. Neuschwander‐Tetri,et al.  Nonalcoholic steatohepatitis: an expanded clinical entity. , 1994, Gastroenterology.

[14]  J. Hoofnagle,et al.  Measurements of iron status in patients with chronic hepatitis. , 1992, Gastroenterology.

[15]  Brian K. Crownover,et al.  Hereditary hemochromatosis. , 2013, American family physician.

[16]  A. Shorbagi,et al.  What is the future of research for hereditary hemochromatosis in Turkey. , 2013, The Turkish journal of gastroenterology : the official journal of Turkish Society of Gastroenterology.

[17]  Jia Ji-don.  EASL clinical practice guidelines for HFE hemochromatosis. , 2010, Journal of hepatology.