Vnitr Lek 2008, 54(11):1106-1110

Persistent diarrhoea, hypotension, polyneuropathy

R. Sýkora1, J. Raděj1, I. Novák1, A. Kroužecký1, J. Mareš1, I. Irová2, Š. Hadravská2, J. Chvojka1, T. Karvunidis1, T. Maňáková2, M. Matějovič1,*
1 I. interní klinika Lékařské fakulty UK a FN Plzeň, přednosta doc. MUDr. Martin Matějovič, Ph.D.
2 Šiklův patologicko-anatomický ústav Lékařské fakulty UK a FN Plzeň, přednosta prof. MUDr. Michal Michal

We present a case report of a 59-year-old man with a history of arterial hypertension and excision of malignant melanoma. He was admitted to the hospital because of two months history of diarrhoea, weight loss and circulatory collapse. In addition, the patient suffered from marked vegetative instability with symptomatic hypotension, polyneuropathy and progression of renal insufficiency, without proteinuria. Complex examination did not reveal neoplasms, endocrine, autoimmune, infectious or neurodegenerative disorders. A serial biopsy of colon failed to provide a clue to the diagnosis. However, AA amyloidosis was found on the kidney biopsy. Neither chronic inflammation nor malignancy was revealed and, hence, no causal treatment could have been established. The patient died from multiple organ failure. The autopsy confirmed systemic AA amyloidosis. The triad consisting of diarrhoea, polyneuropathy and hypotension should rise the suspicion on amyloidosis.

Keywords: amyloidosis; biopsy; hypotension; polyneuropathy; diarrhoea; renal insufficiency

Received: May 12, 2008; Accepted: August 18, 2008; Published: November 1, 2008  Show citation

ACS AIP APA ASA Harvard Chicago Chicago Notes IEEE ISO690 MLA NLM Turabian Vancouver
Sýkora R, Raděj J, Novák I, Kroužecký A, Mareš J, Irová I, et al.. Persistent diarrhoea, hypotension, polyneuropathy. Vnitr Lek. 2008;54(11):1106-1110.
Download citation

References

  1. Westermark P, Benson MD, Buxbaum JN et al. Nomenclature Committee of the International Society of Amyloidosis. Amyloid: toward terminology clarification. Report from the Nomenclature Committee of the International Society of Amyloidosis. Amyloid 2005; 12: 1-4. Go to original source... Go to PubMed...
  2. Adam Z, Ščudla V. Klinické projevy a diagnostika AL-amyloidózy a některých dalších typů amyloidóz. Vnitř Lék 2001; 47: 36-45. Go to PubMed...
  3. Brychta T, Pařenica J, Zatočil T et al. Restriktivní kardiomyopatie jako projev primární amyloidózy. Vnitř Lék 2004; 50: 66-71. Go to PubMed...
  4. Kyle RA, Gertz MA. Primary systemic amyloidosis: clinical and laboratory features in 474 cases. Semin Hematol 1995; 32: 45-59.
  5. Maysouye I. Diagnostic screening of systemic amyloidosis by abdominal fat aspiration: an analysis of 100 cases. Am J Dermatopathol 1997; 19: 41-45. Go to original source... Go to PubMed...
  6. Li K, Kyle RA, Dyck PJ. Immunohistochemical characterization of amyloid proteins in sural nerves and clinical associations in amyloid neuropathy. Am J Pathol 1992; 141: 217-226.
  7. Hazenberg BP, Bijzet J, Limburg PC et al. Diagnostic performance of amyloid A protein quantification in fat tissue of patients with clinical AA amyloidosis. Amyloid 2007; 14: 133-140. Go to original source... Go to PubMed...
  8. Ryšavá R. Perspektivy: Současné terapeutické postupy u amyloidózy ledvin. Postgrad Nefrol 2006; 4: 50-51.
  9. Dember LM. Emerging treatment approaches for the systemic amyloidoses. Kidney Int 2005; 68: 1377-1390. Go to original source... Go to PubMed...
  10. Lachmann H, Obici L, Berber L et al. Results of multi-center, randomized, placebo-controlled trial for the treatment of amyloid A (AA) amyloidosis associated renal disease with NC-503 (eprodisate disodium). Nephrol Dial Transplant 2006; 21 (Suppl 4): iv294.
  11. Gillmore JD, Hawkins PN. Drug insight: emerging therapies for amyloidosis. Nature Clin Pract Nephrol 2006; 2: 263-270. Go to original source... Go to PubMed...
  12. Wechalekar AD, Hawkins PN, Gillmore JD. Perspectives in treatment of AL amyloidosis. Br J Haematol 2008; 140: 365-377. Go to original source... Go to PubMed...
  13. Palladini G, Russo P, Nuvolone M et al. Treatment with oral melphalan plus dexamethasone produces long-term remissions in AL amyloidosis. Blood 2007; 110: 787-788. Go to original source... Go to PubMed...
  14. Dispenzieri A, Gertz MA, Kyle RA et al. Serum cardiac troponins and N-terminal pro-brain natriuretic peptide: a staging system for primary systemic amyloidosis. J Clin Oncol 2004; 22: 3751-3757. Go to original source... Go to PubMed...
  15. Bohle A, Wehrmann R, Eissele R et al. The long-term prognosis of AA and AL renal amyloidosis and the pathogenesis of chronic renal failure in renal amyloidosis. Path Res Pract 1993; 189: 316-331. Go to original source... Go to PubMed...
  16. Hachulla E, Grateau G. Diagnostic tools for amyloidosis. Joint Bone Spine 2002; 69: 538-545. Go to original source... Go to PubMed...
  17. Kebbel A, Röcken C. Immunohistochemical classification of amyloid in surgical pathology revisited. Am J Surg Pathol 2006; 30: 673-683. Go to original source... Go to PubMed...
  18. Freeman R. Autonomic dysfunction. In: Samuels M, Feske S (eds). The Office Practice of Neurology. 2nd ed. Philadelphia: Churchill-Livingstone 2003; 14: 145. Go to original source...




Vnitřní lékařství

Madam, Sir,
please be aware that the website on which you intend to enter, not the general public because it contains technical information about medicines, including advertisements relating to medicinal products. This information and communication professionals are solely under §2 of the Act n.40/1995 Coll. Is active persons authorized to prescribe or supply (hereinafter expert).
Take note that if you are not an expert, you run the risk of danger to their health or the health of other persons, if you the obtained information improperly understood or interpreted, and especially advertising which may be part of this site, or whether you used it for self-diagnosis or medical treatment, whether in relation to each other in person or in relation to others.

I declare:

  1. that I have met the above instruction
  2. I'm an expert within the meaning of the Act n.40/1995 Coll. the regulation of advertising, as amended, and I am aware of the risks that would be a person other than the expert input to these sites exhibited


No

Yes

If your statement is not true, please be aware
that brings the risk of danger to their health or the health of others.