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. 1999 May;52(5):390–392. doi: 10.1136/jcp.52.5.390

Systemic vasculitis and aneurysm formation in the Wiskott-Aldrich syndrome.

W G McCluggage 1, D J Armstrong 1, R J Maxwell 1, P K Ellis 1, D R McCluskey 1
PMCID: PMC1023080  PMID: 10560364

Abstract

A 24 year old male who suffered from the Wiskott-Aldrich syndrome developed intra-abdominal bleeding on two occasions. Radiological investigations showed aneurysmal dilatation of branches of the hepatic and superior mesenteric arteries. The second abdominal bleed necessitated laparotomy and the bleeding was localised to the kidneys. Right nephrectomy was performed and histological examination showed a necrotising vasculitis, mainly involving medium and small sized renal blood vessels. Steroids, immunosuppressive treatment, and control of blood pressure resulted in resolution of the vasculitic process and prevented further haemorrhage. Vasculitis and aneurysm formation are rarely described complications of Wiskott-Aldrich syndrome and may account for the life threatening haemorrhage which occurs in this condition.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. ALDRICH R. A., STEINBERG A. G., CAMPBELL D. C. Pedigree demonstrating a sex-linked recessive condition characterized by draining ears, eczematoid dermatitis and bloody diarrhea. Pediatrics. 1954 Feb;13(2):133–139. [PubMed] [Google Scholar]
  2. DeSanto N. G., Sessa A., Capodicasa G., Meroni M., Capasso G., Esposito L., Ferrara M., Torri Tarelli L., Annunziata S., Giordano C. IgA glomerulonephritis in Wiskott-Aldrich syndrome. Child Nephrol Urol. 1988;9(1-2):118–120. [PubMed] [Google Scholar]
  3. Filipovich A. H., Krivit W., Kersey J. H., Burke B. A. Fatal arteritis as a complication of Wiskott-Aldrich syndrome. J Pediatr. 1979 Nov;95(5 Pt 1):742–744. doi: 10.1016/s0022-3476(79)80726-x. [DOI] [PubMed] [Google Scholar]
  4. Gutenberger J., Trygstad C. W., Stiehm E. R., Opitz J. M., Thatcher L. G., Bloodworth J. M., Jr Familial thrombocytopenia, elevated serum IgA levels and renal disease. A report of a kindred. Am J Med. 1970 Dec;49(6):729–741. doi: 10.1016/s0002-9343(70)80055-9. [DOI] [PubMed] [Google Scholar]
  5. Hitzig W. H., Truniger B. Wiskott-Aldrich syndrome--a truly interdisciplinary problem. Nephrol Dial Transplant. 1996 Oct;11(10):2093–2095. doi: 10.1093/oxfordjournals.ndt.a027108. [DOI] [PubMed] [Google Scholar]
  6. Ilowite N. T., Fligner C. L., Ochs H. D., Brichacek B., Harada S., Haas J. E., Purtilo D. T., Wedgwood R. J. Pulmonary angiitis with atypical lymphoreticular infiltrates in Wiskott-Aldrich syndrome: possible relationship of lymphomatoid granulomatosis and EBV infection. Clin Immunol Immunopathol. 1986 Dec;41(3):479–484. doi: 10.1016/0090-1229(86)90018-8. [DOI] [PubMed] [Google Scholar]
  7. Lau Y. L., Wong S. N., Lawton W. M. Takayasu's arteritis associated with Wiskott-Aldrich syndrome. J Paediatr Child Health. 1992 Oct;28(5):407–409. doi: 10.1111/j.1440-1754.1992.tb02703.x. [DOI] [PubMed] [Google Scholar]
  8. Nozicka Z., Parízková E., Bednárová J., Lichý J. Izolovaná arteritida bazálních mozkových tepen u dítete s primární imunodeficiencí podmínenou Wiskottovým-Aldrichovým syndromem. Cesk Patol. 1987 Dec;23(4):215–221. [PubMed] [Google Scholar]
  9. Perry G. S., 3rd, Spector B. D., Schuman L. M., Mandel J. S., Anderson V. E., McHugh R. B., Hanson M. R., Fahlstrom S. M., Krivit W., Kersey J. H. The Wiskott-Aldrich syndrome in the United States and Canada (1892-1979). J Pediatr. 1980 Jul;97(1):72–78. doi: 10.1016/s0022-3476(80)80133-8. [DOI] [PubMed] [Google Scholar]
  10. Radl J., Dooren L. H., Morell A., Skvaril F., Vossen J. M., Uittenbogaart C. H. Immunoglobulins and transient paraproteins in sera of patients with the Wiskott-Aldrich syndrome: a follow-up study. Clin Exp Immunol. 1976 Aug;25(2):256–263. [PMC free article] [PubMed] [Google Scholar]
  11. Spitler L. E., Wray B. B., Mogerman S., Miller J. J., 3rd, O'Reilly R. J., Lagios M. Nephropathy in the Wiskott-Aldrich syndrome. Pediatrics. 1980 Sep;66(3):391–398. [PubMed] [Google Scholar]
  12. Standen G. R. Wiskott-Aldrich syndrome: a multidisciplinary disease. J Clin Pathol. 1991 Dec;44(12):979–982. doi: 10.1136/jcp.44.12.979. [DOI] [PMC free article] [PubMed] [Google Scholar]
  13. Symons M., Derry J. M., Karlak B., Jiang S., Lemahieu V., Mccormick F., Francke U., Abo A. Wiskott-Aldrich syndrome protein, a novel effector for the GTPase CDC42Hs, is implicated in actin polymerization. Cell. 1996 Mar 8;84(5):723–734. doi: 10.1016/s0092-8674(00)81050-8. [DOI] [PubMed] [Google Scholar]
  14. Watson R. D., Gershwin M. E., Smithwick E., Castles J. J., Ruebner B. Cutaneous T cell lymphoma and leukocytoclastic vasculitis in a long-term survivor of Wiskott-Aldrich syndrome. Ann Allergy. 1985 Nov;55(5):654-7, 703-5. [PubMed] [Google Scholar]

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