ADAMTS-13 METALOPROTEINASE ACTIVITY AND COMPLEMENT INHIBITORY FACTOR H LEVEL IN A GROUP OF PATIENTS WITH THROMBOTIC MICROANGIOPATHY (ORAL PRESENTATIONS: O206)

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ARDALAN M.R.; MOHAJEL SHOJA MOHAMMAD ALI; TAGHIPOUR R.

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Journal: Iranian Journal of Kidney Diseases Year:2009 | Volume:3 | Issue:SUPPLEMENT 1 (12TH INTERNATIONAL CONGRESS OF NEPHROLOGY, DIALYSIS, AND TRANSPLANTATION) Page(s): 7-8

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Title

ADAMTS-13 METALOPROTEINASE ACTIVITY AND COMPLEMENT INHIBITORY FACTOR H LEVEL IN A GROUP OF PATIENTS WITH THROMBOTIC MICROANGIOPATHY (ORAL PRESENTATIONS: O206)

Author(s)

ARDALAN M.R. | MOHAJEL SHOJA MOHAMMAD ALI | TAGHIPOUR R.

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Abstract

Introduction. Thrombotic microangiopathy (TMA) is a unifying term for both hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenia purpura (TTP). TMA is characterized by thrombocytopenia, hemolytic anemia, and microvascular thrombi in different organ systems. Known risk factors for development of TMA including bacterial toxins, viral infection, autoimmune disease, and pregnancy catastrophes. Inherited deficiency of complement inhibitory factor H (FH) and metalloproteinase ADAMTS-13 (vWF-cleaving protease) deficiencies or inhibitors are another important risk factor for development of TMA and may coexist with above risk factors. Methods. In this single center prospective study, all patients with clinical diagnosis of TMA were entered. Patients’ clinical presentation and routine laboratory measurements were re-ordered and serum samples were taken for measurement of complement inhibitory factor H, ADAMTS-13 activity, and presence or absence of ADAMTS-13 inhibitor. Serum complement C3, C4, CH50 level, serologic test for hepatitis B, hepatitis C, HIV, systemic lupus erythematous, and rheumatoid arthritis were performed. History of recent diarrhea and obstetric complications was recorded. We followed recurrence of TMA and renal outcome of these patients for two years.Results. Between 2003 and 2005, we diagnosed 11 consecutive TMA patients (f/m 10/1, age; 22 to 70 years). Majority of our patients were young women. Routine laboratory measurements were as the following: hemoglobin: 8.8 ± 0.9 mg/dl, platelet count: 40800 ± 14793/ml, LDH: 1793 ± 585 IU/L, C3: 195 ± 60mg/ dl, C4: 51 ± 11mg/dl, complement factor H: 772 ± 211 mg/dl, and ADAMTS-13 activity percentage: 32.2% ± 13.7% (range, 50% to 150% activity). One patient had ADAMTS-13 inhibitory factor. Acute dialysis was started in four patients (37%). One patient had a positive history of diarrhea. Two patients (2/11) had systemic lupus erythematous. HBS-Ag and HCV antibody positive results were detected in two patients. Obstetric catastrophes, including IUFD and sever preeclampsia were detected in two patients. One patient with ADAMTS-13 inhibitory factor who presented with unconsciousness and convulsion died in his first week of admission. Recurrent TMA occurred in a patient with low C3 complement level. Chronic Renal dysfunction was developed in two patients. Conclusion. In our study, majority of the patients with TMA were young women. Patients with ADAMTS-13 inhibitory factors and complement factors deficiency had a poor prognosis outcome.

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APA: Copy

ARDALAN, M.R., MOHAJEL SHOJA, MOHAMMAD ALI, & TAGHIPOUR, R.. (2009). ADAMTS-13 METALOPROTEINASE ACTIVITY AND COMPLEMENT INHIBITORY FACTOR H LEVEL IN A GROUP OF PATIENTS WITH THROMBOTIC MICROANGIOPATHY (ORAL PRESENTATIONS: O206). IRANIAN JOURNAL OF KIDNEY DISEASES (IJKD), 3(SUPPLEMENT 1 (12TH INTERNATIONAL CONGRESS OF NEPHROLOGY, DIALYSIS, AND TRANSPLANTATION)), 7-8. SID. https://sid.ir/paper/308964/en

Vancouver: Copy

ARDALAN M.R., MOHAJEL SHOJA MOHAMMAD ALI, TAGHIPOUR R.. ADAMTS-13 METALOPROTEINASE ACTIVITY AND COMPLEMENT INHIBITORY FACTOR H LEVEL IN A GROUP OF PATIENTS WITH THROMBOTIC MICROANGIOPATHY (ORAL PRESENTATIONS: O206). IRANIAN JOURNAL OF KIDNEY DISEASES (IJKD)[Internet]. 2009;3(SUPPLEMENT 1 (12TH INTERNATIONAL CONGRESS OF NEPHROLOGY, DIALYSIS, AND TRANSPLANTATION)):7-8. Available from: https://sid.ir/paper/308964/en

IEEE: Copy

M.R. ARDALAN, MOHAMMAD ALI MOHAJEL SHOJA, and R. TAGHIPOUR, “ADAMTS-13 METALOPROTEINASE ACTIVITY AND COMPLEMENT INHIBITORY FACTOR H LEVEL IN A GROUP OF PATIENTS WITH THROMBOTIC MICROANGIOPATHY (ORAL PRESENTATIONS: O206),” IRANIAN JOURNAL OF KIDNEY DISEASES (IJKD), vol. 3, no. SUPPLEMENT 1 (12TH INTERNATIONAL CONGRESS OF NEPHROLOGY, DIALYSIS, AND TRANSPLANTATION), pp. 7–8, 2009, [Online]. Available: https://sid.ir/paper/308964/en

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