Do not miss any of the new cases! Subscribe above to receive our newsletter! ↑↑

Do not miss the opportunity to download our app on the Google Play Store. Click here to enjoy it.

Previous

Case 312

Next


Click on the images above to zoom in

17 year-old male patient comes from Carmo da Mata (Minas Gerais) to the EC unit after hospitalisation due to hematemesis, melena, altered hepatic function and low level of platelets. No other comorbidities. Denies alcohol intake, smoking or use of recreational drugs. Denies any contact with fresh water. Physical examination reveals hepatomegaly and pain during right hypochondrium palpation. Requestes Upper Gastrointestinal Endoscopy (Image 1) and laboratorial review (Image 2) to diagnostic elucidation.

Considering the clinical data, the results and that viral serology were negative to Hepatitis B and C, which one is the most likely diagnosis?

a) Wilson's disease

25%

b) Primary sclerosing cholangitis

25%

c) Type 1 autoimmune hepatitis

25%

d) Type 2 autoimmune hepatitis

25%
   

Image analysis

Image 1 - Analysis: Upper Gastrointestinal Endoscopy, picture of the oesophagus showing small and medium-size esophageal varices (red arrows) due to portal hypertension.

Highlights

- Autoimmune Hepatitis (AIH) is a chronic affection leading to progressive destruction of liver parenchyma. It must be suspected in young patients with chronic hepatopathy and negative viral serology;

- Type 1 AIH is positive to ASMA and AAN, while type 2 is positive to Anti-LKM1;

- Diagnostic criteria include: positive autoantibodies, elevated transaminases, hypergammaglobulinemia and high levels of IgG;

- 1st line treatment includes prednisone with or without azathioprine, showing good prognosis;

- Treatment is suspended after 18 months of clinical and histological remission, although most cases presents recidives.

References

- Couto C, Bittencourt P. Controvérsias no diagnóstico e tratamento da hepatite auto-imune. In: Savassi-Rocha P, Coelho L, Sanchez M, Rausch M, ed. by. Tópicos em Gastroenterologia 14 - Controvérsias. 1st ed. Rio de Janeiro: Guanabara Koogan; 2015.

- Santos BC, Campos MLP, Couto CA. Hepatopatias Crônicas Não-Virais. In: Santos BC, Faria IM, Couto CA. Manual Diagnóstico em Gastroenterologia para o Estudante de Medicina. 1st ed. Belo Horizonte: GASTROliga; 2018.

- Blumberg, S., Burakoff, Robertgreenberger, Norton, J. Current diagnosis & treatment. Gastroenterology, hepatology, & endoscopy. 3rd ed. New York: McGraw-Hill, 2016.

- Santos R, Alissa F, Reyes J, Teot L, Ameen N. Fulminant hepatic failure: Wilson's disease or autoimmune hepatitis? Implications for transplantation. Pediatric Transplantation. 2005;9(1):112-116.

- Santos B, Guedes L, Faria L, Couto C. Wilson’s disease presentation resembling autoimmune hepatitis: case report. In press.

Authors

Bruno Campos Santos, 4th year medical student at Universidade Federal de Minas Gerais.

Email: bruno_campos[at]outlook.com

Fernando Henrique Melo Amorim Pinto, 4th year medical student at Universidade Federal de Minas Gerais.

Email: 12fernandoamorim[at]gmail.com

Mateus Jorge Nardelli, 3rd year medical student at Universidade Federal de Minas Gerais.

Email: mateusnardelli[at]gmail.com

Supervisor

Claudia Alves Couto, MD, Ph.D., Associated Professor and Doctor of the Department of Medical Clinic at the Medical School of Universidade Federal de Minas Gerais.

Email: clalcouto[at]gmail.com

Reviewers

Lucas Bruno Rezende, Luana Almeida, Daniela Tereza Gonçalves Manso, Izabella Costa, Luanna Monteiro, MD, Viviane Santuari Parisotto Marino, MD, Ph.D.

Translated by

Bruno Campos Santos, 4th year medical student at Universidade Federal de Minas Gerais.

E-mail: bruno_campos[at]outlook.com

Test question

(Medical Residence 2009 - UFF) Young patient, with history of jaundice and recurring pruritus, shows elevated transaminases over 5 times the normal range, gamma-GT and alkaline phosphatase over 1,5 times normal range, HAV serology negative, negative HBsAg, positive Anti-HBs, positive HBc, positive anti-HCV, hypergammaglobulinemia and positive Anti-LKM1. The most likely diagnosis is:

a) Acute hepatitis C

25%

b) Chronic hepatitis B

25%

c) Autoimmune hepatits

25%

d) Primary sclerosing cholangitis

25%

e) Primary billiar cholangitis

25%
   

Commentics

Sorry, there is a database connection problem.

Please check back again shortly.

Bookmark and Share




Unfortunately there is no english translation available yet for this case.

Please refer to the Portuguese version instead or come back later.





Follow us:      Twitter  |    Facebook  |    Get the news  |    E-mail