eMedicine Specialties > Neurology > Pediatric Neurology

Thrombotic Thrombocytopenic Purpura: Follow-up

Author: Robert Rust Jr, MD, Thomas E Worrell Jr Professor of Epileptology and Neurology, Co-Director of FE Dreifuss Child Neurology and Epilepsy Clinics, University of Virginia School; Clinical and Residency Training, Child Neurology, University of Virginia Hospital and Clinics
Contributor Information and Disclosures

Updated: Jun 26, 2006

Follow-up

Further Inpatient Care

  • In some instances, admission for rehabilitation may be necessary after the acute phase of illness is managed.

Further Outpatient Care

  • In some instances, outpatient rehabilitation is indicated.
  • Patients with persistent renal failure may need outpatient renal dialysis.
  • Other patients require follow-up for neurologic or cardiologic disease

Deterrence/Prevention

  • Preventing the first episode of TTP is difficult because the heritable predilection due to ADAMTS-13 deficiency is usually not recognized before the first event.
  • After this predilection is recognized, avoidance of provocative circumstances could be contemplated, though this, too, may prove difficult.
  • Ensuring that meat is well cooked may prevent the rare occurrence of TTP n adults due to the acquisition of enterohemorrhagic E coli infection.

Complications

  • Aside from untoward events due to TTP itself (stroke, cardiac conduction abnormality, etc), additional complications are the result of therapies, particularly plasma exchange and plasmapheresis.

Prognosis

  • The old observation that recurrence cannot be predicted on a clinical or laboratory basis (Meacham, 1952) remains true except that recurrence tends to occur in patients who have TTP due to heritable ADAMTS-13 deficiency.
    • Severe ADAMTS deficiency manifesting in infancy (Schulman-Upshaw syndrome) is especially prone to recurrence. Individuals with this presentation tend to have particularly poor outcome because the first presentation is often severe, and correct diagnosis and specific therapeutic intervention is often delayed.
    • With repeated episodes, individuals with Schulman-Upshaw syndrome are likely to develop brain lesions due to severe ischemia.
  • Two large studies of more than 130 episodes of TTP in adults receiving plasma exchange showed a 8-25% mortality rate during the acute phase of disease management. The risk of relapse was as high as 21%, and the risk of end-stage renal failure requiring dialysis was 16% (Hayward, 1994; Conlon, 1995).
    • The risk for unfavorable outcomes or death may not be predictable on the basis of clinical or laboratory variables at presentation.
    • As many as 58% of survivors continued to have health problems.
  • Studies of 169 adults treated with plasmapheresis for TTP showed a 7-9.6% mortality rate. The rate of unresponsiveness to therapy was 6.7%, and the risk for end-stage renal disease requiring dialysis was as high as 36% (Hollenbeck, 1998; Lara, 1999).
    • The estimated risk for end-stage renal disease was as high as 82% in the patients compared with similar populations of TTP not treated with plasmapheresis.
    • Lara et al (1999) found that 6.7% of their patients did not respond to plasmapheresis, and though follow-up was incomplete, at least 13% of patients had relapses at 3 weeks to 152 months after plasmapheresis.
    • Deaths during the acute phase of illness may result from cerebral hemorrhage, septic shock, or other complications of illness and treatment.
    • As with plasma exchange, clinical and laboratory features of illness were not predictive of poor outcome in the study by Hollenbeck et al (1998).
    • Lara et al (1999) found that the absence of fever during the acute initial episode of TTP was a risk factor for recurrence.
  • Devenoulas et al found that advanced age at the first episode of TTP indicated an increased risk for a poor outcome, as did severe renal impairment (defined as serum creatinine level > 2mg%).
  • In some instances, dialysis is discontinued after intervals as long as 6 years in acute TTP.
  • Most available outcome data are based on studies of heterogeneous populations of adults treated for TTP.
  • Epidemic occurrences of TTP may pose a high risk for poor outcome, as was seen in the 1996 outbreak of TTP after infection with enterohemorrhagic E coli O157:H7. Among 22 individuals who developed TTP, 16 were treated with plasma exchange. Five (31%) died, and 4 (24%) of the survivors required dialysis.
  • Available relapse data almost certainly underestimate the lifetime risk for relapse, abnormal renal function, and hypertension (George, 2000).
  • Some have suggested that, with proper support, the neurologic outcome of TTP among adults who survive the initial episode is reasonably good. However, newer data (Silverstein, 1968) have confounded older data by suggesting that less than one half of all patients with neurologic manifestations of TTP have a substantial improvement in neurologic function after adequate treatment.
  • Among the difficulties most often reported are chronic fatigue and difficulties with memory and concentration.
  • The myocardial outlook is guarded in patients who have clinical or ECG changes consistent with clinically significant myocardial disease.

Patient Education

  • Ensure that meat is well cooked.

Miscellaneous

Medicolegal Pitfalls

  • Medicolegal pitfalls are not unlike those encountered in any other area of medicine. The pitfalls chiefly consist of the failure to recognize and appropriately manage TTP.
  • The involvement of individuals skilled in the use of plasmapheresis and plasma infusion is important in the management of these disorders.
  • If immunosuppressive drugs are to be used in patients with relapse, the weight of evidence and the potential risks of such therapy should carefully be reviewed with the patient and his or her family.
  • Platelet transfusion poses a substantial risk and should be avoided in TTP, according to some authorities.
  • Antiplatelet agents should be used with caution, as noted in Medical Care.
  • Authorities have considered anticoagulation to be contraindicated in HUS.
 


More on Thrombotic Thrombocytopenic Purpura

Overview: Thrombotic Thrombocytopenic Purpura
Differential Diagnoses & Workup: Thrombotic Thrombocytopenic Purpura
Treatment & Medication: Thrombotic Thrombocytopenic Purpura
Follow-up: Thrombotic Thrombocytopenic Purpura
References
[ CLOSE WINDOW ]

References

  1. Adams RD, Cammermeyer J, Fitzgerald PJ. Neuropathological aspects of thrombotic acroagiothrombosis; clinico-anatomical study of generalized platelet thrombosis. J Neurol Neurosurg Psychiatry. 1948;11:27-43.

  2. Amorosi EL, Karpatkin S. Antiplatelet treatment of thrombotic thrombocytopenic purpura. Ann Intern Med. Jan 1977;86(1):102-6. [Medline].

  3. Baker KR, Moake JL. Thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome. Curr Opin Pediatr. Feb 2000;12(1):23-8. [Medline].

  4. Bell WR, Braine HG, Ness PM, Kickler TS. Improved survival in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Clinical experience in 108 patients. N Engl J Med. Aug 8 1991;325(6):398-403. [Medline].

  5. Berkowitz LR, Dalldorf FG, Blatt PM. Thrombotic thrombocytopenic purpura: a pathology review. JAMA. Apr 20 1979;241(16):1709-10. [Medline].

  6. Birgens H, Ernst P, Hansen MS. Thrombotic thrombocytopenic purpura: treatment with a combination of antiplatelet drugs. Acta Med Scand. 1979;205(5):437-9. [Medline].

  7. Blaker F, Altrogge H, Hellwege HH, et al. Treatment of severe haemolytic-uraemic syndrome by dialysis [in German]. Dtsch Med Wochenschr. Aug 4 1978;103(31):1229-32. [Medline].

  8. Bohle A, Helmchen U, Grund KE, et al. Malignant nephrosclerosis in patients with hemolytic uremic syndrome (primary malignant nephrosclerosis). Curr Top Pathol. 1977;65:81-113. [Medline].

  9. Bukowski RM, Hewlett JS, Harris JW, et al. Exchange transfusions in the treatment of thrombotic thrombocytopenic purpura. Semin Hematol. Jul 1976;13(3):219-32. [Medline].

  10. Bukowski RM. Thrombotic thrombocytopenic purpura: a review. Prog Hemost Thromb. 1982;6:287-337. [Medline].

  11. Byrnes JJ, Moake JL. Thrombotic thrombocytopenic purpura and the haemolytic-uraemic syndrome: evolving concepts of pathogenesis and therapy. Clin Haematol. May 1986;15(2):413-42. [Medline].

  12. Byrnes JJ, Khurana M. Treatment of thrombotic thrombocytopenic purpura with plasma. N Engl J Med. Dec 22 1977;297(25):1386-9. [Medline].

  13. Chin D, Chyczij H, Etches W, et al. Treatment of thrombotic thrombocytopenic purpura with intravenous gammaglobulin. Transfusion. Jan-Feb 1987;27(1):115-6. [Medline].

  14. Chow TW, Turner NA, Chintagumpala M, et al. Increased von Willebrand factor binding to platelets in single episode and recurrent types of thrombotic thrombocytopenic purpura. Am J Hematol. Apr 1998;57(4):293-302. [Medline].

  15. Cimolai N, Basalyga S, Mah DG, et al. A continuing assessment of risk factors for the development of Escherichia coli 0157:H7-associated hemolytic uremic syndrome. Clin Nephrol. Aug 1994;42(2):85-9. [Medline].

  16. Drews RE, Weinberger SE. Thrombocytopenic disorders in critically ill patients. Am J Respir Crit Care Med. Aug 2000;162(2 Pt 1):347-51. [Medline].

  17. Elliott MA, Nichols WL. Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome. Mayo Clin Proc. Nov 2001;76(11):1154-62. [Medline].

  18. Fakhouri F, Vincent F, Legendre C. Pathological and therapeutic distinctions in HUS/TTP. Lancet. Feb 5 2000;355(9202):497; discussion 497-8. [Medline].

  19. Fiorani L, Vianelli N, Gugliotta L, et al. Brain MRI and SPET in thrombotic thrombocytopenic purpura. Ital J Neurol Sci. Apr 1995;16(3):149-51. [Medline].

  20. Fujikawa K, Suzuki H, McMullen B, Chung D. Purification of human von Willebrand factor-cleaving protease and its identification as a new member of the metalloproteinase family. Blood. Sep 15 2001;98(6):1662-6. [Medline].

  21. Furlan AJ, Robles R. von Willebrand. n Engl J Med. 1998.

  22. Furlan M, Lammle B. Haemolytic-uraemic syndrome and thrombotic thrombocytopenic purpura-- new insights into underlying biochemical mechanisms. Nephrol Dial Transplant. Aug 2000;15(8):1112-4. [Medline].

  23. Furlan M, Robles R, Solenthaler M, Lammle B. Acquired deficiency of von Willebrand factor-cleaving protease in a patient with thrombotic thrombocytopenic purpura. Blood. Apr 15 1998;91(8):2839-46. [Medline].

  24. Furlan M, Robles R, Solenthaler M. Deficient activity of von Willebrand factor-cleaving protease in chronic relapsing thrombotic thrombocytopenic purpura. Blood. May 1 1997;89(9):3097-103. [Medline].

  25. Furlan M, Robles R, Galbusera M, et al. von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome. N Engl J Med. Nov 26 1998;339(22):1578-84. [Medline].

  26. Galbusera M, Noris M, Rossi C, et al. Increased fragmentation of von Willebrand factor, due to abnormal cleavage of the subunit, parallels disease activity in recurrent hemolytic uremic syndrome and thrombotic thrombocytopenic purpura and discloses predisposition in families. The Italian. Blood. Jul 15 1999;94(2):610-20. [Medline].

  27. Galbusera M, Ruggenenti P, Noris M, et al. alpha 1-Antitrypsin therapy in a case of thrombotic thrombocytopenic purpura. Lancet. Jan 28 1995;345(8944):224-5. [Medline].

  28. George JN. Idiopathic thrombocytopenic purpura in adults: current issues for pathogenesis, diagnosis and management. Hematol J. 2004;5 Suppl 3:S12-4. [Medline].

  29. George JN, Vesely SK. How can we provide the best care for our patients with immune thrombocytopenic pupura?. Mayo Clin Proc. Apr 2004;79(4):456-7. [Medline].

  30. George JN, Vesely SK, Terrell DR, George JN. The Oklahoma Thrombotic Thrombocytopenic Purpura-Hemolytic Uremic Syndrome (TTP-HUS) Registry: a community perspective of patients with clinically diagnosed TTP-HUS. Semin Hematol. Jan 2004;41(1):60-7. [Medline].

  31. George JN. How I treat patients with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Blood. Aug 15 2000;96(4):1223-9. [Medline].

  32. George JN, Sadler JE, Lammle B. Platelets: thrombotic thrombocytopenic purpura. Hematology (Am Soc Hematol Educ Program). 2002;315-34. [Medline].

  33. Habib R, Mathieu H, Royer P. Arteriole-capillary thrombotic disease of the kidney in children. [in French]. Rev Fr Etud Clin Biol. Oct 1958;3(8):891-4. [Medline].

  34. Hosler GA, Cusumano AM, Hutchins GM. Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome are distinct pathologic entities. A review of 56 autopsy cases. Arch Pathol Lab Med. Jul 2003;127(7):834-9. [Medline].

  35. Hovinga JA, Studt JD, Alberio L, Lammle B. von Willebrand factor-cleaving protease (ADAMTS-13) activity determination in the diagnosis of thrombotic microangiopathies: the Swiss experience. Semin Hematol. Jan 2004;41(1):75-82. [Medline].

  36. Jubinsky PT, Moraille R, Tsai HM. Thrombotic thrombocytopenic purpura in a newborn. J Perinatol. Jan 2003;23(1):85-7. [Medline].

  37. Kakishita E. Pathophysiology and treatment of thrombotic thrombocytopenic purpura/hemolytic uremic syndrome (TTP/HUS). Int J Hematol. Jun 2000;71(4):320-7. [Medline].

  38. Kaplan BS, Thomson PD. Hyperuricemia in the hemolytic-uremic syndrome. Am J Dis Child. Aug 1976;130(8):854-6. [Medline].

  39. Karlsberg RP, Lacher JW, Bartecchi CE. Adult hemolytic-uremic syndrome. Familial variant. Arch Intern Med. Sep 1977;137(9):1155-7. [Medline].

  40. Kennedy SS, Zacharski LR, Beck JR. Thrombotic thrombocytopenic purpura: analysis of 48 unselected cases. Semin Thromb Hemost. 1980;6(4):341-9. [Medline].

  41. Kojouri K, Vesely SK, Terrell DR, George JN. Splenectomy for adult patients with idiopathic thrombocytopenic purpura: a systematic review to assess long-term platelet count responses, prediction of response, and surgical complications. Blood. Nov 1 2004;104(9):2623-34.

  42. Kokame K, Matsumoto M, Soejima K, et al. Mutations and common polymorphisms in ADAMTS13 gene responsible for von Willebrand factor-cleaving protease activity. Proc Natl Acad Sci U S A. Sep 3 2002;99(18):11902-7. [Medline].

  43. Kovacs MJ, Roddy J, Gregoire S, et al. Thrombotic thrombocytopenic purpura following hemorrhagic colitis due to Escherichia coli 0157:H7. Am J Med. Feb 1990;88(2):177-9. [Medline].

  44. Lawlor ER, Webb DW, Hill A, Wadsworth LD. Thrombotic thrombocytopenic purpura: a treatable cause of childhood encephalopathy. J Pediatr. Feb 1997;130(2):313-6. [Medline].

  45. Levy GG, Nichols WC, Lian EC, et al. Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura. Nature. Oct 4 2001;413(6855):488-94. [Medline].

  46. MacWhinney JB, Packer JT, Miller G, Greendyke RM. Thrombotic thrombocytopenic purpura in childhood. Blood. 1962;18:181-99.

  47. Malbrain ML, Lambrecht GL, Brans B, et al. Acute renal failure in non-fulminant hepatitis A [letter]. Clin Nephrol. Mar 1994;41(3):180-1. [Medline].

  48. Martin DL, MacDonald KL, White KE, et al. The epidemiology and clinical aspects of the hemolytic uremic syndrome in Minnesota [see comments]. N Engl J Med. Oct 25 1990;323(17):1161-7. [Medline].

  49. Meacham GC, Orbiston JL, et al. Thrombotic thrombocytopenic purpura: A disseminated disease of arterioles. Blood. 1952;6:706.

  50. Misiani R, Appiani AC, Edefonti A, et al. Haemolytic uraemic syndrome: therapeutic effect of plasma infusion. Br Med J (Clin Res Ed). Nov 6 1982;285(6351):1304-6. [Medline].

  51. Moake JL. von Willebrand factor in the pathophysiology of thrombotic thrombocytopenic purpura. Clin Lab Sci. Nov-Dec 1998;11(6):362-4. [Medline].

  52. Moake JL. TTP--desperation, empiricism, progress [editorial; comment]. N Engl J Med. Aug 8 1991;325(6):426-8. [Medline].

  53. Moake JL. Thrombotic thrombocytopenic purpura. Thromb Haemost. Jul 1995;74(1):240-5. [Medline].

  54. Moake JL. The role of von Willebrand factor (vWF) in thrombotic thrombocytopenic purpura (TTP) and the hemolytic-uremic syndrome (HUS). Prog Clin Biol Res. 1990;337:135-40. [Medline].

  55. Moake JL, McPherson PD. von Willebrand factor in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome. Transfus Med Rev. Apr 1990;4(2):163-8. [Medline].

  56. Moake JL. Thrombotic microangiopathies. N Engl J Med. Aug 22 2002;347(8):589-600. [Medline].

  57. Moschowitz E. Hyaline thrombosis of the terminal arterioles and capillaries: A hitherto undescribed disease. Proc N Y Pathol Soc. 1924;24:21-4.

  58. Nair JM, Bellevue R, Bertoni M, Dosik H. Thrombotic thrombocytopenic purpura in patients with the acquired immunodeficiency syndrome (AIDS)-related complex. A report of two cases. Ann Intern Med. Aug 1 1988;109(3):209-12. [Medline].

  59. Noris M, Ruggenenti P, Perna A, et al. Hypocomplementemia discloses genetic predisposition to hemolytic uremic syndrome and thrombotic thrombocytopenic purpura: role of factor H abnormalities. Italian Registry of Familial and Recurrent Hemolytic Uremic Syndrome/Thrombotic Thrombocytopenic P. J Am Soc Nephrol. Feb 1999;10(2):281-93. [Medline].

  60. Raife TJ. Pathogenesis of thrombotic thrombocytopenic purpura. Curr Hematol Rep. Mar 2003;2(2):133-8. [Medline].

  61. Raniele DP, Opsahl JA, Kjellstrand CM. Should intravenous immunoglobulin G be first-line treatment for acute thrombotic thrombocytopenic purpura? Case report and review of the literature. Am J Kidney Dis. Aug 1991;18(2):264-8. [Medline].

  62. Remis RS, MacDonald KL, Riley LW, et al. Sporadic cases of hemorrhagic colitis associated with Escherichia coli 0157:H7. Ann Intern Med. Nov 1984;101(5):624-6. [Medline].

  63. Remuzzi G, Ruggenenti P. The hemolytic uremic syndrome. Kidney Int. Jul 1995;48(1):2-19. [Medline].

  64. Richards A, Goodship JA, Goodship TH. The genetics and pathogenesis of haemolytic uraemic syndrome and thrombotic thrombocytopenic purpura. Curr Opin Nephrol Hypertens. Jul 2002;11(4):431-5. [Medline].

  65. Ridolfi RL, Bell WR. Thrombotic thrombocytopenic purpura. Report of 25 cases and review of the literature. Medicine (Baltimore). Nov 1981;60(6):413-28. [Medline].

  66. Rizzoni G, Claris-Appiani A, Edefonti A, et al. Plasma infusion for hemolytic-uremic syndrome in children: results of a multicenter controlled trial. J Pediatr. Feb 1988;112(2):284-90. [Medline].

  67. Robertson RJ, Gilcher R, Metz KF, et al. Hemoglobin concentration and aerobic work capacity in women following induced erythrocythemia. J Appl Physiol. Aug 1984;57(2):568-75. [Medline].

  68. Ruggenenti P, Remuzzi G. Treatment of adult hemolytic-uremic syndrome. Adv Nephrol Necker Hosp. 2000;30:83-94. [Medline].

  69. Ruggenenti P, Remuzzi G. The pathophysiology and management of thrombotic thrombocytopenic purpura. Eur J Haematol. Apr 1996;56(4):191-207. [Medline].

  70. Schwartz SG, McPherson AR, Mieler WF, et al. Bilateral combined occlusion of the central retinal artery and vein secondary to thrombotic thrombocytopenic purpura. Arch Ophthalmol. Sep 2000;118(9):1304-5. [Medline].

  71. Sheth KJ, Swick HM, Haworth N. Neurological involvement in hemolytic-uremic syndrome. Ann Neurol. Jan 1986;19(1):90-3. [Medline].

  72. Silverstein A. Thrombotic thrombocytopenic purpura. The initial neurologic manifestations. Arch Neurol. Apr 1968;18(4):358-62. [Medline].

  73. Studt JD, Hovinga JA, Antoine G, et al. Fatal congenital thrombotic thrombocytopenic purpura with apparent ADAMTS13 inhibitor: in vitro inhibition of ADAMTS13 activity by hemoglobin. Blood. Jan 15 2005;105(2):542-4. [Medline].

  74. Studt JD, Hovinga JA, Radonic R, et al. Familial acquired thrombotic thrombocytopenic purpura: ADAMTS13 inhibitory autoantibodies in identical twins. Blood. Jun 1 2004;103(11):4195-7. [Medline].

  75. Tarr PI, Neill MA, Allen J, et al. The increasing incidence of the hemolytic-uremic syndrome in King County, Washington: lack of evidence for ascertainment bias. Am J Epidemiol. Mar 1989;129(3):582-6. [Medline].

  76. Torok TJ, Holman RC, Chorba TL. Increasing mortality from thrombotic thrombocytopenic purpura in the United States--analysis of national mortality data, 1968-1991. Am J Hematol. Oct 1995;50(2):84-90. [Medline].

  77. Torok TJ, Holman RC, Chorba TL. Increasing mortality from thrombotic thrombocytopenic purpura in the United States--analysis of national mortality data, 1968-1991. Am J Hematol. Oct 1995;50(2):84-90. [Medline].

  78. Tsai HM. Von Willebrand factor, ADAMTS13, and thrombotic thrombocytopenic purpura. J Mol Med. Oct 2002;80(10):639-47. [Medline].

  79. Tsai HM. Shear stress and von Willebrand factor in health and disease. Semin Thromb Hemost. Oct 2003;29(5):479-88. [Medline].

  80. Tsai HM, Lian EC. Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med. Nov 26 1998;339(22):1585-94. [Medline].

  81. Tsai HM. Advances in the pathogenesis, diagnosis, and treatment of thrombotic thrombocytopenic purpura. J Am Soc Nephrol. Apr 2003;14(4):1072-81. [Medline].

  82. Tsai HM, Shulman K. Rituximab induces remission of cerebral ischemia caused by thrombotic thrombocytopenic purpura. Eur J Haematol. Mar 2003;70(3):183-5. [Medline].

  83. Tsai HM, Sussman II, Ginsburg D, et al. Proteolytic cleavage of recombinant type 2A von Willebrand factor mutants R834W and R834Q: inhibition by doxycycline and by monoclonal antibody VP-1. Blood. Mar 15 1997;89(6):1954-62. [Medline].

  84. Vesely SK, Perdue JJ, Rizvi MA, et al. Management of adult patients with persistent idiopathic thrombocytopenic purpura following splenectomy: a systematic review. Ann Intern Med. Jan 20 2004;140(2):112-20. [Medline].

  85. Vesely SK, George JN, Lammle B, et al. ADAMTS13 activity in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: relation to presenting features and clinical outcomes in a prospective cohort of 142 patients. Blood. Jul 1 2003;102(1):60-8. [Medline].

  86. Zheng X, Chung D, Takayama TK, et al. Structure of von Willebrand factor-cleaving protease (ADAMTS13), a metalloprotease involved in thrombotic thrombocytopenic purpura. J Biol Chem. Nov 2 2001;276(44):41059-63. [Medline].

  87. Zhou W, Inada M, Lee TP, et al. ADAMTS13 is expressed in hepatic stellate cells. Lab Invest. Jun 2005;85(6):780-8. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

TTP, thrombocytic acroangiothrombosis, Schulman-Upshaw syndrome, Upshaw-Schulman syndrome, constitutional TTP, severe ADAMTS13 deficiency, thrombotic microangiopathy, TMA, hemolytic uremic syndrome, HUS, TTP-HUS, TTP/HUS, ADAMTS13, ADAMTS-13, Shiga toxin, Stx

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Robert Rust Jr, MD, Thomas E Worrell Jr Professor of Epileptology and Neurology, Co-Director of FE Dreifuss Child Neurology and Epilepsy Clinics, University of Virginia School; Clinical and Residency Training, Child Neurology, University of Virginia Hospital and Clinics
Robert Rust Jr, MD is a member of the following medical societies: American Academy of Neurology, American Epilepsy Society, American Headache Society, American Neurological Association, Child Neurology Society, International Child Neurology Association, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Medical Editor

David A Griesemer, MD, Professor, Departments of Neurology and Pediatrics, Medical University of South Carolina
David A Griesemer, MD is a member of the following medical societies: American Academy of Neurology, American Epilepsy Society, and Child Neurology Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Kenneth J Mack, MD, PhD, Senior Associate Consultant, Department of Child and Adolescent Neurology, Mayo Clinic
Kenneth J Mack, MD, PhD is a member of the following medical societies: American Academy of Neurology, Child Neurology Society, Phi Beta Kappa, and Society for Neuroscience
Disclosure: Nothing to disclose.

CME Editor

Selim R Benbadis, MD, Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital
Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association
Disclosure: Nothing to disclose.

Chief Editor

Nicholas Y Lorenzo, MD, Chief Editor, eMedicine Neurology; Consulting Staff, Neurology Specialists and Consultants
Nicholas Y Lorenzo, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Neurology
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.