Successful Treatment of Unremitting Gastro-Intestinal Haemorrhage & Rashes of Henoch-Schӧnlein Purpura with Colchicine & Cryoprecipitate: A Case Report
Journal of Pediatric Nephrology,
Vol. 9 No. 2 (2021),
https://doi.org/10.22037/jpn.v8i2.33428
Abstract
Severe gastrointestinal manifestations and rashes of Henoch Schonlein Purpura (HSP) may not always respond to prednisolone or other immunosuppressive agent. Though reduced factor XIII may play a role in gastrointestinal manifestations but its role in management of skin rashes is uncertain. Here, we reported a case treated with different immunosuppressive agents but didn`t respond. Thereafter, despite having the normal factor XIII assay, the unremitting GI symptoms and skin rashes, has improved after prescribing colchicines & infusion of Cryoprecipitate containing factor XIII.
Keywords:
- HSP, Gastrointestinal bleeding, Rash, Cryoprecipitate, Colchicine
How to Cite
1.
Huque SS, Amaan A, Mamun A-A, Jesmin T, Begum A, Roy RR, Uddin GM. Successful Treatment of Unremitting Gastro-Intestinal Haemorrhage & Rashes of Henoch-Schӧnlein Purpura with Colchicine & Cryoprecipitate: A Case Report. J Ped Nephrol [Internet]. 2021Mar.31 [cited 2022Jul.4];9(2). Available from: https://journals.sbmu.ac.ir/jpn/article/view/33428
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13. Alioglu B, Ozsoy MH, Tapci E, Karamercan S, Agras PI, Dallar Y. Successful use of recombinant factor VIIa in a child with Scho¨enlein-Henoch-purpura presenting with compartment syndrome and severe factor XIII deficiency. Blood Coagul Fibrinolysis 2013 Jan;24(1):102-5.
14. Lorenz R, Born P, Classen M. Substitution of factor XIII concentrate in treatment refractory ulcerative colitis. A prospective pilot study. Med Klin (Munich) 1994 Oct, 15; 89(10):534-7.
15. Henriksson P, Hedner U, Nilsson IM. Factor XIII (fibrin stabilizing factor) in Henoch-Scho¨nlein purpura. Acta Paediatr Scand 1977 May;66(3):273-7.
16. Fukui H, Kamitsuji H, Nagao T, Yamada K, Akatsuka J, Inagaki M, et al. Clinical evaluation of a pasteurized factor XIII concentrate administration in Henoch-Scho¨nlein purpura. Japanese Pediatric Group. Thromb Res 1989 Dec; 56(6): 667-75.
17. Hogendorf A, Mlynarski W. Factor XIII deficiency in Henoch- Scho¨nlein purpura e report of two case and literature review. Dev Period Med 2014 Jul-Sep;18(3):318-22.
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19. Matayoshi T, Omi T, Sakai N, Kawana S. Clinical significance of blood coagulation factor XIII activity in adult Henoch-Schӧnlein purpura. J Nippon Med Sch 2013;80(4):268-78
2. Prenzel F, Pfa¨ffle R, Thiele F, Schuster V. Decreased factor XIII activity during severe Henoch-Schoenlein purpuraedoes it play a role? Klin Padiatr 2006 May-Jun; 218(3): 174-6.
3. Calvo-Rı´o V, Loricera J, Mata C, Martı´n L, Ortiz-Sanjua´n F, Alvarez L, et al. Henoch Scho¨nlein purpura in northern Spain: clinical spectrum of the disease in 417 patients from a single center. Medicine (Baltimore) 2014 Mar;93(2):106-13.
4. Henriksson P, Hedner U, Nilsson IM: Factor XIII (fibrin stabilizing factor ) in Henoch-Schönlein purpura. Acta Paediatr Scand 1977; 66: 273―277.
5. Fukui H, Kamitsuji H, Nagao T, et al.: Clinical evaluation of a pasteurized factor XIII concentrate administration in Henoch-Schönlein purpura. Thrombs Res 1989; 56: 667―675.
6. Migita M, Hayakawa J, Shima H, et al.: A Case of Henoch-Schönlein Purpura with Rare Complications: Necrosis of the Small Intestine, Neurological Symptoms, and Pericardial Tamponade. J Nippon Med sch 2005; 72: 383―386.
7. Saulsbury FT. Henoch-Scho¨nlein purpura in children. Report of 100 patients and review of the literature. Medicine (Baltimore) 1999 Nov;78(6):395e409.
8. Anwar R, Miloszewski KJA. Factor XIII deficiency. Br J Hematol 1999 Dec; 107(3):468-84.
9. Hayat M, Ariens RA, Moayyedi P, Grant PJ, O’Mahony S. Coagulation factor XIII and markers of thrombin generation and fibrinolysis in patients with inflammatory bowel disease. Eur J Gastroenterol Hepatol 2002 Mar;14(3):249-56.
10. Ahmad F, Solymoss S, Poon MC, Berube C, Sullivan AK. Characterization of an acquired IgG inhibitor of coagulation factor XIII in a patient with systemic lupus erythematosus. Br J Haematol 1996 Jun;93(3):700-3.
11. Weinberg JB, Pippen AM, Greenberg CS. Extravascular fibrin formation and dissolution in synovial tissue of patients with osteoarthritis and rheumatoid arthritis. Arthritis Rheum 1991 Aug;34(8):996-1005.
12. Imai T, Okada H, Nanba M, Kawada K, Kusaka T, Itoh S. Henoch-Scho¨nlein purpura with intracerebral hemorrhage. Brain Dev 2002 Mar;24(2):115-7.
13. Alioglu B, Ozsoy MH, Tapci E, Karamercan S, Agras PI, Dallar Y. Successful use of recombinant factor VIIa in a child with Scho¨enlein-Henoch-purpura presenting with compartment syndrome and severe factor XIII deficiency. Blood Coagul Fibrinolysis 2013 Jan;24(1):102-5.
14. Lorenz R, Born P, Classen M. Substitution of factor XIII concentrate in treatment refractory ulcerative colitis. A prospective pilot study. Med Klin (Munich) 1994 Oct, 15; 89(10):534-7.
15. Henriksson P, Hedner U, Nilsson IM. Factor XIII (fibrin stabilizing factor) in Henoch-Scho¨nlein purpura. Acta Paediatr Scand 1977 May;66(3):273-7.
16. Fukui H, Kamitsuji H, Nagao T, Yamada K, Akatsuka J, Inagaki M, et al. Clinical evaluation of a pasteurized factor XIII concentrate administration in Henoch-Scho¨nlein purpura. Japanese Pediatric Group. Thromb Res 1989 Dec; 56(6): 667-75.
17. Hogendorf A, Mlynarski W. Factor XIII deficiency in Henoch- Scho¨nlein purpura e report of two case and literature review. Dev Period Med 2014 Jul-Sep;18(3):318-22.
18. Kawasaki K, Komura H, Nakahara Y, Shiraishi M, Higashida M, Ouchi K. Factor XIII in Henoch-Scho¨nlein purpura with isolated gastrointestinal symptoms. Pediatr Int 2006 Aug;48(4):413-5.
19. Matayoshi T, Omi T, Sakai N, Kawana S. Clinical significance of blood coagulation factor XIII activity in adult Henoch-Schӧnlein purpura. J Nippon Med Sch 2013;80(4):268-78
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