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Large pulmonary arteriovenous malformation with hyperammonemia

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Abstract

A 45-year-old female presented with generalized fatigue, unaccompanied by other symptoms. Investigation revealed severe anemia due to gastric bleeding, and hereditary hemorrhagic telangiectasia accompanied by a large pulmonary arteriovenous malformation (PAVM). Additionally, the presence of hepatic arteriovenous and portovenous shunts indicated hepatic involvement. In addition to hypoxemia due to right-to-left shunting in PAVM, hyperammonemia with normal hepatic function was detected. The large PAVM was successfully managed with surgical resection. Hyperammonemia, persisting despite the hemostasis of gastric bleeding, improved postoperatively in the absence of treatment directed at hepatic involvement. We believe that resection of large PAVM contributed to the improvement of hyperammonemia.

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References

  1. Porterous ME, Burn J, Procter SJ. Hereditary haemorrhagic telangiectasia: A clinical analysis. J Med Genet 1992; 29: 527–30.

    Article  Google Scholar 

  2. Gossage JR, Kanj G. Pulmonary arteriovenous malformations: A state of the art review. Am J Respir Crit Care Med 1998; 158: 643–61.

    Article  CAS  PubMed  Google Scholar 

  3. White RI, Lynch-Nyhan A, Terry P, Buescher PC, Farmlett EJ, Charnas L, et al. Pulmonary arteriovenous malformations: Techniques and long-term outcome of embolotherapy. Radiology 1988; 169: 663–9.

    Article  PubMed  Google Scholar 

  4. Lee DW, White RI Jr, Egglin TK, Pollak JS, Fayad PB, Wirth JA, et al. Embolotherapy of large pulmonary arteriovenous malformations: Long-term results. Ann Thorac Surg 1997; 64: 930–40.

    Article  CAS  PubMed  Google Scholar 

  5. Watanabe N, Munakata Y, Ogiwara M, Miyatake M, Nakagawa F, Hirayama J. A case of pulmonary arteriovenous malformation in a patient with brain abscess successfully treated with video-assisted thoracoscopic resection. Chest 1995; 108: 1724–7.

    Article  CAS  PubMed  Google Scholar 

  6. Larson AM. Liver disease in hereditary hemorrhagic telangiectasia. J Clin Gastroenterol 2003; 36: 149–58.

    Article  CAS  PubMed  Google Scholar 

  7. Relman AS. Renal acidosis and renal excretion of acid in health and disease. Adv Intern Med 1964; 12: 295–347.

    CAS  PubMed  Google Scholar 

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Takuwa, T., Yoshioka, S., Miyata, Y. et al. Large pulmonary arteriovenous malformation with hyperammonemia. Jpn J Thorac Caridovasc Surg 52, 484–487 (2004). https://doi.org/10.1007/s11748-004-0146-4

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  • DOI: https://doi.org/10.1007/s11748-004-0146-4

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