Persistent diarrhoea, hypotension, polyneuropathy
Authors:
R. Sýkora 1; J. Raděj 1; I. Novák 1; A. Kroužecký 1; J. Mareš 1; I. Irová 2; Š. Hadravská 2; J. Chvojka 1; T. Karvunidis 1; T. Maňáková 2; M. Matějovič 1
Authors place of work:
I. interní klinika Lékařské fakulty UK a FN Plzeň, přednosta doc. MUDr. Martin Matějovič, Ph. D.
1; Šiklův patologicko‑anatomický ústav Lékařské fakulty UK a FN Plzeň, přednosta prof. MUDr. Michal Michal
2
Published in the journal:
Vnitř Lék 2008; 54(11): 1106-1110
Category:
Case Report
Summary
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.
Key words:
amyloidosis – biopsy – hypotension – polyneuropathy – diarrhoea – renal insufficiency
Zdroje
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.
2. Adam Z, Ščudla V. Klinické projevy a diagnostika AL-amyloidózy a některých dalších typů amyloidóz. Vnitř Lék 2001; 47: 36–45.
3. Brychta T, Pařenica J, Zatočil T et al. Restriktivní kardiomyopatie jako projev primární amyloidózy. Vnitř Lék 2004; 50: 66–71.
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.
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.
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.
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.
12. Wechalekar AD, Hawkins PN, Gillmore JD. Perspectives in treatment of AL amyloidosis. Br J Haematol 2008; 140: 365–377.
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.
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.
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.
16. Hachulla E, Grateau G. Diagnostic tools for amyloidosis. Joint Bone Spine 2002; 69: 538–545.
17. Kebbel A, Röcken C. Immunohistochemical classification of amyloid in surgical pathology revisited. Am J Surg Pathol 2006; 30: 673–683.
18. Freeman R. Autonomic dysfunction. In: Samuels M, Feske S (eds). The Office Practice of Neurology. 2nd ed. Philadelphia: Churchill-Livingstone 2003; 14: 145.
Štítky
Diabetology Endocrinology Internal medicineČlánok vyšiel v časopise
Internal Medicine
2008 Číslo 11
Najčítanejšie v tomto čísle
- Hypoglycaemic periodic paralysis in hyperthyroidism patients
-
Langerhans cell histiocytosis in adult patients – a disease with many faces
Experience of a centre and an overview of the disease symptoms - The safety of long-term administration of losartan in current clinical practice: a non-intervention NCT-CZ 14/04/LOZ study
- Place of inhibin B investigation in clinical andrological praxis