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Case 367

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36-year-old male patient started cramp-type pain in lower abdomen and in both legs two days ago. Known sickle cell anaemia patient with multiple red cells transfusion in the past, also had a recent diagnosis of fibrous chronic liver disease. Denies alcohol consumption nor familial liver diseases. Diagnosis of sickle cell pain crisis is made and patient is hospitalized for laboratorial review and imaging study to better elucidate the liver disease aetiology.

Considering the clinical data, the laboratorial panel and the images, the most likely aetiology is:

a) Wilson disease

25%

b) Secondary hemochromatosis.

25%

c) Non-alcoholic fatty liver disease

25%

d) Autoimmune hepatitis

25%
   

Image analysis

Image 1 - Analysis: Magnetic resonance imaging (MRI) of the upper abdomen, axial view at L2 level, T2-weighted. Reduced liver (in red), with irregular contours and significative hyposignal suggestive of iron overload. There are no signs of dilated intra- or extra-hepatic bile ducts. Spleen is not seen (post-splenectomy status). This exam was used for the quantitative analysis of the iron overload.

 

 

Image 2 - Analysis: Hepatic iron concentration evaluation at the upper abdomen MRI using the MRQuantIF software (Pr Yves Gandon, Université de Rennes). Three points of interest are picked at the right lobe and two points at the paravertebral muscles to analyse signal intensity and its decrease as the echo time goes on – liver intensity is compared to the muscular because the latter does not suffer from iron overload. Intensity averages and liver and muscular signals are used to calculate the signal intensity ratio (SIR), from which is obtained the estimated hepatic iron concentration (EHIC). In the image, the Y axis represents the signal intensity in the MRI and, the X axis, the echo time. The yellow line demonstrates the decrease in hepatic signal intensity. The pink line represents the decrease in muscular tissue. The values analysis is compatible with iron overload, with iron concentration estimated to be of 476 µmol/g (RV < 36 µmol/g).

Highlights

-   Fibrous chronic liver disease requires aetiology investigation due to the possibility of a curative therapy;

-   High ferritin levels and increased transferrin saturation index are suggestive of iron overload;

-   Iron overload in the liver is seen as hyperattenuation at computed tomography and hyposignal at magnetic resonance imaging;

-    It is possible to measure iron overload by MRI;

-   Iron overload treatment is based on serial phlebotomy (non-anaemic patient) or iron chelator (anaemic patient).

References

- Leonis MA, Balistreri WF. Outros transtornos metabólicos hereditários do fígado. In: Feldman M, Friedman LS, Brandt LJ. Tratado gastrointestinal e doenças do fígado. 9 ed. Rio de Janeiro. Elsevier. 2014. p. 1283-1305.

- Bourbon Filho LA, Ferrão TO, França AVC, Rocha RD, Dantas LD, Carvalho LFP, Moreira MDAB, Cipolotti R. Sobrecarga de ferro transfusional em portadores de anemia falciforme: comparação entre ressonância magnética e ferritina sérica. Radiol Bras. 2011;44(3):151-155.

- Pinho IF, Nobeschi L, Goto RE, Munhoz BNS, Melo HJF. A importância da ressonância magnética no diagnóstico da hemocromatose hereditária. Atas de Ciência da Saúde. 2014;(2):01-13.

- Schrier SL, Bacon BR, Kwiatkowski J. Approach to the patient with suspected iron overload. In: UpToDate. Available at: https://www.uptodate.com/contents/approach-to-the-patient-with-suspected-iron-overload?search=iron%20overload&source=search_result&selectedTitle=1~150&usage_type=default&display_rank=1.

Author

Mateus Jorge Nardelli, 5th year medical student at Universidade Federal de Minas Gerais.

Email: mateus.nardelli[at]gmail.com

Supervisor

Luciana Costa Faria, MD, Ph.D., Associated Professor and Doctor of the Department of Internal Medicine at the Medical School of Universidade Federal de Minas Gerais.

Email: lucostafaria[at]hotmail.com

 

Júlio Guerra Domingues, MD, Auxiliary Professor and Doctor of the Department of Anatomy and Imaging at the Medical School of Universidade Federal de Minas Gerais.

Email: jgdjulio[at]gmail.com

Reviewers

Gustavo Monteiro, Fernando Amorim, Bruno Campos, Luiz Gustavo, Rafael Valério, José Nelson Mendes Vieira,  MD, Ph.D., Viviane Santuari Parisotto Marino, MD, Ph.D.

Translated by

Bruno Campos Santos, 6th year medical student at Universidade Federal de Minas Gerais.

E-mail: bruno_campos[at]outlook.com

Test question

(2014 – UFG – Emergency Medicine - Adapted)

Analyse the following clinical cases:

                     1. 15-year-old female with mild elevation of transaminases and ANA positivity.

                     2. 56-year-old male, diabetic, dark skin, family history of cirrhosis.

                     3. 35-year-old male, ulcerative rectocolitis and GGT and PA alterations, also mild transaminases alteration.

                     4. 32-year-old male with dysarthria, spasticity, low platelets and Kayser-Fleischer rings.

 The diagnosis of these clinical cases are, respectively:

a) Wilson disease, hemochromatosis, primary biliary cirrhosis and sclerosing cholangitis.

25%

b) Autoimmune hepatitis, hemochromatosis, sclerosing cholangitis and Wilson disease.

25%

c) Viral hepatitis, Wilson disease, primary biliary cirrhosis and alcoholic hepatitis.

25%

d) Primary biliary cholangitis, non-alcoholic fatty liver disease, alcoholic hepatopathy and Wilson disease.

25%

e)

25%
   

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