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Pathological Case of the Month FREE

Lewis Barness, MD; Enid Gilbert-Barness, MD
[+] Author Affiliations

Accepted for publication October 18, 1991.

Reprint requests to Departments of Pathology and Pediatrics, University of Wisconsin, Center for Health Sciences, E5/326, 600 Highland Ave, Madison, WI 53792 (Dr Gilbert-Barness).


Am J Dis Child. 1992;146(1):111-112. doi:10.1001/archpedi.1992.02160130113031.
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A 16-year-old girl was admitted to the hospital with jaundice of 2 weeks' duration. She appeared well until age 14 years, when she developed a tremor, the cause of which was not determined. Menarche occurred at age 15 years.

On admission, she was markedly jaundiced and extremely lethargic. She was intermittently incoherent and had a persistent tremor. The liver edge was palpable 4 cm below the right costal margin, and the liver span was 12 cm. Ascites was present.

Laboratory values were as follows: hemoglobin, 60 g/L; serum albumin, 10 g/L; serum ammonia, 235 μmol/L; serum alanine aminotransferase, 800 U/L; aspartate aminotransferase, 600 U/L; γ-glutamyl transferase, 800 U/L; and prothrombin time, 34 seconds. Results of tests to detect hepatitis A, B, and C were negative.

A diagnosis of fulminant hepatitis with cirrhosis was made. After additional tests, a liver transplant was performed. The gross appearance of

REFERENCES

Scheinberg IH, Sternlieb I. Wilson's Disease . Philadelphia, Pa: WB Saunders Co; 1984;.
Archer GJ, Monie RDH.  Wilson's disease and chronic active hepatitis . Lancet . 1977;;1:486-487.
Scott J, Gollan JL, Samourian S, Sherlock S.  Wilson's disease presenting as chronic active hepatitis . Gastroenterology . 1978;;74:645-651.
Stromeyer FW, Ishak KG.  Histology of the liver in Wilson's disease: a study of 34 cases . Am J Clin Pathol. 1980;;73:12-24.
Ishak KG.  Pathology of inherited metabolic disorders . In: Balistreri WF, Stocker JT, eds. Pediatric Hepatology . Washington, DC: Hemisphere Publishing Corp; 1990;:77-158.
Goldfischer S, Sternlieb I.  Changes in the distribution of hepatic copper in relation to the progression of Wilson's disease (hepatolenticular degeneration) . Am J Pathol. 1968;; 53:883-901.
Irons RD, Schenk EA, Lee JCK.  Cytochemical methods for copper . Arch Pathol Lab Med. 1977;;101:298-301.
Lindquist RR.  Studies on the pathogenesis of hepatolenticular degeneration, II: cytochemical methods for the localization of copper . Arch Pathol. 1969;;87:370-379.
Jain S, Scheuer PJ, Archer BB, et al.  Histological demonstration of copper and copper-associated protein in chronic liver diseases . J Clin Pathol. 1978;;31:784-790.
Salaspuro M, Sipponen P.  Demonstration of an intracellular copper-binding protein by orcein staining in longstanding cholestatic liver diseases . Gut . 1976;;17:787-790.
Phillips MJ, Poucell S, Patterson J, Valencia P. The Liver: An Atlas and Text of Ultrastructural Pathology . NEW York, NY: Raven Press; 1987;:328-333.
Sternlieb I.  Evolution of hepatic lesion in Wilson's disease (hepatolenticular degeneration) . Prog Liver Dis. 1972;;4:511-525.
Nartey NO, Frei JV, Cherian MG.  Hepatic copper and metallothionein distribution in Wilson's disease (hepatolenticular degeneration) . Lab Invest . 1987;;57:397-501.

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Country-Specific Mortality and Growth Failure in Infancy and Yound Children and Association With Material Stature

Use interactive graphics and maps to view and sort country-specific infant and early dhildhood mortality and growth failure data and their association with maternal

References

Scheinberg IH, Sternlieb I. Wilson's Disease . Philadelphia, Pa: WB Saunders Co; 1984;.
Archer GJ, Monie RDH.  Wilson's disease and chronic active hepatitis . Lancet . 1977;;1:486-487.
Scott J, Gollan JL, Samourian S, Sherlock S.  Wilson's disease presenting as chronic active hepatitis . Gastroenterology . 1978;;74:645-651.
Stromeyer FW, Ishak KG.  Histology of the liver in Wilson's disease: a study of 34 cases . Am J Clin Pathol. 1980;;73:12-24.
Ishak KG.  Pathology of inherited metabolic disorders . In: Balistreri WF, Stocker JT, eds. Pediatric Hepatology . Washington, DC: Hemisphere Publishing Corp; 1990;:77-158.
Goldfischer S, Sternlieb I.  Changes in the distribution of hepatic copper in relation to the progression of Wilson's disease (hepatolenticular degeneration) . Am J Pathol. 1968;; 53:883-901.
Irons RD, Schenk EA, Lee JCK.  Cytochemical methods for copper . Arch Pathol Lab Med. 1977;;101:298-301.
Lindquist RR.  Studies on the pathogenesis of hepatolenticular degeneration, II: cytochemical methods for the localization of copper . Arch Pathol. 1969;;87:370-379.
Jain S, Scheuer PJ, Archer BB, et al.  Histological demonstration of copper and copper-associated protein in chronic liver diseases . J Clin Pathol. 1978;;31:784-790.
Salaspuro M, Sipponen P.  Demonstration of an intracellular copper-binding protein by orcein staining in longstanding cholestatic liver diseases . Gut . 1976;;17:787-790.
Phillips MJ, Poucell S, Patterson J, Valencia P. The Liver: An Atlas and Text of Ultrastructural Pathology . NEW York, NY: Raven Press; 1987;:328-333.
Sternlieb I.  Evolution of hepatic lesion in Wilson's disease (hepatolenticular degeneration) . Prog Liver Dis. 1972;;4:511-525.
Nartey NO, Frei JV, Cherian MG.  Hepatic copper and metallothionein distribution in Wilson's disease (hepatolenticular degeneration) . Lab Invest . 1987;;57:397-501.

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