• Filter By:
• Specialty
• Source
• Condition
• Cancer
• Infectious Disease
• Drug
• Training
• Management
• Procedure
• Therapy
• Vaccine
• Nutrition
• Country

High molecular weight kininogen contributes to early mortality and kidney dysfunction in a mouse model of sickle cell disease.
CONCLUSIONS: We found elevated plasma levels of cleaved HK in sickle patients compared to healthy controls, suggesting ongoing HK activation in SCD. We used bone marrow transplantation to generate wild type and sickle cell mice on a HK-deficient background. We found that short-term HK deficiency attenuated thrombin generation and inflammation in sickle mice at steady state, which was independent of bradykinin signaling. Moreover, long-term HK deficiency attenuates kidney injury, reduces chronic inflammation, and ultimately improves of sickle mice. PMID: 32573897 [PubMed - as supplied by publisher]
Source: Thrombosis and Haemostasis - June 22, 2020 Category: Hematology Authors: Sparkenbaugh EM, Kasztan M, Henderson MW, Ellsworth P, Davis PR, Wilson KJ, Reeves B, Key NS, Strickland S, McCrae K, Pollock DM, Pawlinski R Tags: J Thromb Haemost Source Type: research

Description of the First Cases with ADAMTS13 Mutations in Hungary
CONCLUSIONS:Clinical and molecular characterization of the first 5 Hungarian cases with ADAMTS13 mutations was presented. Compound heterozygous, damaging ADAMTS13 mutations with deficient plasma ADAMTS13 activity were associated with serious clinical consequences. In case of thrombotic microangiopathy in young patients with stroke, or HELLP syndrome, complete workup for ADAMTS13 activity and inhibitors is necessary, and genetic analysis should be considered. Three new ADAMTS13 mutations (c.1016_1017delCA, c.3199T>A , c.2839C>T) were identified and shown to be associated with decreased plasma ADAMTS13 activity.Disclos...
Source: Blood - November 21, 2018 Category: Hematology Authors: Reti, M., Sinkovits, G., Cseprekal, O., Csuka, D., Szilagyi, A., Farkas, Z., Klucsik, Z., Szederjesi, A., Wagner, L., Reusz, G., Kremer Hovinga, J. A., Rigo, J., Masszi, T., Prohaszka, Z. Tags: 311. Disorders of Platelet Number or Function Source Type: research

Successful kidney transplantation in a patient with congenital thrombotic thrombocytopenic purpura (Upshaw-Schulman syndrome).
CONCLUSION: Our experience suggests that kidney transplantation may be an appropriate management for carefully selected patients with congenital TTP who develop end-stage renal disease. PMID: 28940540 [PubMed - as supplied by publisher]
Source: Transfusion - September 20, 2017 Category: Hematology Authors: Fattah H, Kumar D, George JN, Massey HD, King AL, Friedman KD, Gupta G Tags: Transfusion Source Type: research