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Case 269

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Female lactant, one month and 23 days of life, went to PA/HC-UFMG with a history of progressing jaundice since she was born, light ‘’clay-colored”stools, dark urine and insuficient weigh gain in the last 20 days. She was a term newborn, suitable for the gestacional age, had higid parents and they were non consanguineous. She was emaciated, icteric. She had a liver 6 cm far awar from the right costal ridge and her spleen was 4 cm far away from the left costal ridge. Direct bilirubin: 13.72 mg/dL, indirect bilirubin: 3.39 mg/dL; AST (GOT): 423 IU/L; ALT (GPT): 184 IU/L; ALP: 474 IU/L; GGT 634 IU/L.

According to the case and to the ultrasonography images, the most probable etiology of this neonatal cholestasis is:

a) Alagille syndrome

25%

b) Choledocal cyst

25%

c) Biliary atresia

25%

d) Idiopathic neonatal hepatitis

25%
   

Image Analysis

Image 1: Abdominal ultrasound showing a very small gallbladder even after fasting. Measures of the gallbladder in its longer axis are 10x1.4 mm.

 

Image 2: Hyperechogenic thickening of 3.5 mm in front of bifurcation of the portal vein. It shows the thickening following longitudinal axis of portal vein.

 

Image 3: Hyperechogenic thickening of 3.5 mm in front of bifurcation of the portal vein. It shows the transversal cut of the portal vein with the thickening in its anterior portion: this is the triangular cord sign.

Highlights

- Light "clay-colored" stools, dark urine and jaundice are clinic manifestations of cholestasis and they point to the necessity of urgent doctor’s assessment as well to the need of dosing serum bilirubin;

- Elevated direct (conjugated) bilirubin is always a pathologic signal. The patient needs to be referred to a tertiary reference center urgently;

- Abdominal ultrasound and histopathology study of the liver are the more accurate diagnostic methods to distinguish between intra and extra hepatic cholestasis;

- Late referencing of biliary atresia suspected is one of the main factors to Kasai’s surgery be less successful in its bile drainage;

- It is important to warning parents, caregivers and primary care physicians about the need to recognizing clay-colored stools which is one of the first signs of biliary atresia.

References

- Roquete MLV et al. Colestaseneonatal.Jornal de Pediatria - Vol. 76, Supl.2, pp. 187-197, 2000.

- Zhou L, ShanQ,Tian W, Wang Z,Liang J, XieX.Ultrasound for theDiagnosisofBilaryAtresia: a meta-analysis. AJR 2016; 206: W1-W10.

- Ferreira AR, Fangundes EDT, Roquete MLV, Ottoni CMC. Colestase do recém-nascido e do lactente. Leão E. et al. PediatriaAmbulatorial,  5°edição,capítulo 42: pp 605-615.

- Protocolo clínico setorial HC-UFMG n°35.[tema:Abordagem da criança colestática e o diagnóstico de atresia de vias biliares]. AtualizaçãoJunho/2016. Versão n/2.

Authors

Heloisa Di Matteo Joaquim, 5th year medical student at Universidade Federal de Minas Gerais
Email: helodimatteo[at]gmail.com

 

Joice Carneiro Dias Prodígios, 5th year medical student at Universidade Federal de Minas Gerais

Email: joicecdp[at]hotmail.com

Supervisors

Professor Elisa Seiler Poelman, Pediatrician and Professor of the Department of Pediatrics of the Federal University of  Minas Gerais. 

Reviewers 

Fernando Carvalho Bottega, Bruno Santos, Bruno Campos, André Naback and Professor Jesiana Ferreira Pedrosa

Translated by

Joice Carneiro Dias Prodígios, 5th year medical student at Universidade Federal de Minas Gerais

Email: joicecdp[at]hotmail.com

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