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Budd–Chiari syndrome has different presentations and disease severity during adolescence

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Abstract

There are limited data on clinical profile of adolescent patients with Budd–Chiari syndrome (BCS). We studied clinical, radiological, thrombophilia profile and treatment outcomes in adolescent patients with BCS.

Methods

Forty-three consecutive patients of BCS with onset of symptoms during adolescence (10–19 years) were enrolled in the study. 129 randomly selected adult patients with BCS and 36 children with BCS formed the two control groups. The clinical history, physical examination, laboratory tests, thrombophilic disorders, radiological features and treatment outcomes of adolescents were compared to adults and children.

Results

In adolescents, ascites (25/43 vs. 110/129, p = 0.0004) and thrombophilic disorders (16/43 vs. 93/129 p < 0.0001) were less frequent than adults. More adolescents (14/43) presented with hepatomegaly alone without ascites than adults (9/129, p < 0.001) or children (1/36, p = 0.005). Adolescents had lower Clichy scores [3.75 (1.2)] than adults [4.72 (1.3), p < 0.0001) or children [4.43 (1.7), p = 0.041]. JAK-2 V617F mutation was the most common thrombophilic disorder in adolescents (5/43) and more common than children (0/36, p = 0.043). Response to therapy was better in adolescents (74.4%) than children (52.8%, p = 0.038), but similar to adults (63.56%, p = 0.13).

Conclusion

During adolescence, patients with BCS present less commonly with ascites and may present with hepatomegaly alone. JAK-2 V617F mutation is the most common thrombophilic disorder during adolescence; though thrombophilic disorders are less common in adolescents than adults. Response to therapy is similar to adults, but better than children.

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Abbreviations

BCS:

Budd–Chiari syndrome

MRV:

Magnetic resonance venography

HV:

Hepatic vein

IVC:

Inferior vena cava

TIPS:

Trans-jugular intrahepatic porto-systemic shunt

VEGF:

Vascular endothelial growth factor

WBC:

White blood cell

AST:

Aspartate transaminase

ALT:

Alanine transaminase

ALP:

Alkaline phosphatase

INR:

International normalized ratio

AT:

Anti-thrombin

APLA:

Anti-phospholipid antibody

JAK-2:

Janus kinase-2 gene

PNH:

Paroxysmal nocturnal hemoglobinuria

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Funding

There is no financial support for this study.

Author information

Authors and Affiliations

  1. Department of Gastroenterology, KEM Hospital and Seth GS Medical College, Multistory Building, 11th floor, Parel, Mumbai, Maharashtra, 400012, India

    Akash Shukla, Pratin Bhatt, Deepak Kumar Gupta, Tejas Modi, Jatin Patel, Amit Gupte, Megha Meshram & Shobna Bhatia

Authors
  1. Akash Shukla
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  2. Pratin Bhatt
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  3. Deepak Kumar Gupta
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  4. Tejas Modi
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  5. Jatin Patel
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  6. Amit Gupte
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  7. Megha Meshram
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  8. Shobna Bhatia
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Contributions

Study concept and design: Dr. AS, Dr. PB, Dr. DKG, Dr. TM, Dr. JP, Dr. AG, Dr. SB. Acquisition of data; analysis and interpretation of data: Dr. AS, Dr. Pratin Bhatt, Dr. DKG, Dr. TM, Dr. AG, MM, Dr. SB. Drafting of the manuscript; critical revision of the manuscript: Dr. AS, Dr. Pratin Bhatt, Dr. TM, Dr. JP, DrAmit Gupte, Dr. SB. Statistical analysis: Dr. AS, Dr. PB, Dr. DKG, Dr. TM, Dr. JP, Dr. AG, Dr. SB. Administrative, technical, or material support; study supervision: Dr. AS, Dr. DKG, Dr. SB.

Corresponding author

Correspondence to Akash Shukla.

Ethics declarations

Conflict of interest

Akash Shukla, Pratin Bhatt, Deepak Kumar Gupta, Tejas Modi, Jatin Patel, Amit Gupte, Megha Meshram and Shobna Bhatia declare that they have no conflict of interest.

Ethical approval

The study was performed in a manner conforming to the Helsinki Declaration of 1975, as revised in 2000 and 2008 concerning human and animal rights, and the authors followed the policy concerning informed consent as per guidelines.

Writing assistance

There is no writing assistance for this study.

Implications and contributions statement

Hepatomegaly may be an only presenting feature of BCS in adolescence. Radiological interventions are effective in adolescent patients not responding to medical management with similar response rate when compared with adult patients with BCS.

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Shukla, A., Bhatt, P., Gupta, D.K. et al. Budd–Chiari syndrome has different presentations and disease severity during adolescence. Hepatol Int 12, 560–566 (2018). https://doi.org/10.1007/s12072-018-9880-z

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  • DOI: https://doi.org/10.1007/s12072-018-9880-z

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