scholarly journals Bleeding patterns in patients before and after diagnosis of von Willebrand disease: Analysis of a US medical claims database

Haemophilia ◽  
2021 ◽  
Author(s):  
Jonathan C. Roberts ◽  
Lynn M. Malec ◽  
Imrran Halari ◽  
Sarah A. Hale ◽  
Abiola Oladapo ◽  
...  

Author(s):  
Keisuke Maeda ◽  
Kenta Murotani ◽  
Satoru Kamoshita ◽  
Yuri Horikoshi ◽  
Akiyoshi Kuroda

Abstract Background This study examined the association between parenteral energy/amino acid doses and in-hospital mortality among inpatients on long-term nil per os (NPO) status, using a medical claims database in Japan. Methods Hospitalized patients with aspiration pneumonia, aged ≥65 years and on >7-days NPO status, were identified in a medical claims database between January 2013 and December 2018. Using multivariate logistic regression and regression analyses, we examined the association between mean parenteral energy/amino acid doses and in-hospital mortality, and secondarily the association between prognosis (in-hospital mortality, inability to receive full oral intake, re-admission, hospital stay length) among four groups classified by mean amino acid dose (No dose: 0 g/kg/day; Very low dose: >0, ≤0.3 g/kg/day; Low dose: >0.3, ≤0.6 g/kg/day; Moderate dose: >0.6 g/kg/day). Results The analysis population included 20,457 inpatients (≥80 years: 78.3%). In total, 5,920 mortalities were recorded. Increased amino acid doses were significantly associated with reduced in-hospital mortality (p <0.001). With a No dose reference level, the odds ratios (95% confidence interval) of in-hospital mortality adjusted for potential confounders, were 0.78 (0.72–0.85), 0.74 (0.67–0.82), and 0.69 (0.59–0.81) for Very low, Low, and Moderate amino acid doses, respectively. Additionally, patients prescribed amino acid dose levels >0.6 g/kg/day had shorter hospitalization periods than those prescribed none. Conclusions Increased amino acid doses were associated with reduced in-hospital mortality. Sufficient amino acid administration is recommended for patients with aspiration pneumonia requiring NPO status.



1989 ◽  
Vol 86 (10) ◽  
pp. 3723-3727 ◽  
Author(s):  
D. Ginsburg ◽  
B. A. Konkle ◽  
J. C. Gill ◽  
R. R. Montgomery ◽  
P. L. Bockenstedt ◽  
...  




2008 ◽  
Vol 100 (09) ◽  
pp. 462-468 ◽  
Author(s):  
Huub H. D. M. van Vliet ◽  
Mies C. Kappers-Klunne ◽  
Jan J. Michiels ◽  
Frank W. G. Leebeek

SummaryDose-response relationship was studied between PFA-100 closure times (PFA CTs) and factor (F)VIII-von Willebrand factor (VWF) parameters in patients with von Willebrand disease (VWD) type 1 and type 2 before and after treatment with DDAVP (n=84) or FVIII/VWF concentrate (n=38). DDAVP treatment of patients with VWD type 1 normalised the PFA CTs by increasing VWF levels to normal. Of the 14 patients with VWD type 2, PFA CTs did not normalize in eight. Haemate-P substitution in patients with VWD type 1 induced a less favourable response as compared to DDAVP, because PFA CTs did not correct in all patients. Of 12 patients with VWD type 2 treated with Haemate-P, six showed a correction of PFA CTs (<250 sec), which correlated with the normalisation of the VWF CB/ Ag ratio. In-vitro studies were performed by using whole blood of patients with VWD and adding various amounts of FVIII/VWF concentrate. Addition of Haemate-P induced an increase of the VWF CB/Ag ratio from 0.30 to 0.70 in blood of patients with VWD type 2 with correction of the PFA CTs. Immunate did not result in an increase of VWF CB/Ag ratio in blood of VWD type 2 patients, and the PFA CTs remained prolonged. We conclude that PFA-100 might be an adequate instrument not only for diagnosis but also for monitoring of DDAVP responses and FVIII/ VWF substitution of patients with VWD type 1 and 2,but this is dependent upon the type of VWD and the concentrate used.





Blood ◽  
1999 ◽  
Vol 94 (5) ◽  
pp. 1637-1647 ◽  
Author(s):  
P.L. Turecek ◽  
L. Pichler ◽  
W. Auer ◽  
G. Eder ◽  
K. Varadi ◽  
...  

Abstract Although proteolytic processing of pro-von Willebrand factor (pro-vWF) resulting in free propeptide and mature vWF is known to be initiated intracellularly, vWF released from endothelial cells may contain a high proportion of incompletely processed pro-vWF. Because pro-vWF is only rarely detectable in normal human plasma, we investigated whether extracellular processing of pro-vWF is possible. A recombinant preparation (rpvWF) containing both pro-vWF and mature vWF subunits was infused into 2 pigs and 1 dog with severe von Willebrand disease, 2 mice with a targeted disruption of the vWF gene, and 2 healthy baboons. Total vWF antigen (vWF:Ag), free propeptide, and pro-vWF were measured using enzyme-linked immunosorbent assay techniques in blood samples drawn before and after infusion. vWF:Ag increased promptly. No pro-vWF could be detected when the first postinfusion sample was drawn after 30 minutes (pigs) or 60 minutes (mice), but pro-vWF was detectable for short periods when postinfusion samples were drawn after 15 minutes (dog) or 5 minutes (baboons). In contrast, free propeptide was increased at the first timepoint measured, suggesting that it was generated from the pro-vWF in the rpvWF preparation. vWF multimers were analyzed in the rpvWF preparation and in plasma samples drawn before and after infusion of rpvWF using ultra-high resolution 3% agarose gels to allow separation of homo- and hetero-forms of the vWF polymers. Within 30 minutes after infusion in the pigs, 1 hour in the dog and the mice, and within 2 hours in the baboons, the multimer pattern had changed to that typically seen in mature vWF. These data indicate that propeptide cleavage from unprocessed vWF can occur extracellularly in the circulation. The enzyme or enzymes responsible for this cleavage in plasma remain to be identified.



Blood ◽  
1978 ◽  
Vol 51 (4) ◽  
pp. 751-761 ◽  
Author(s):  
Y Sultan ◽  
C Jeanneau ◽  
J Lamaziere ◽  
P Maisonneuve ◽  
JP Caen

Four unrelated patients with a severe form of von Willebrand disease showed no detectable factor VIII-related antigen (VIIIR:AG) in either their plasma or their platelets. They received cryoprecipitate infusions, three patients in a single injection each and one every day for 9 days before and after surgery. Platelet VIIIR:Ag was studied at different times during and after transfusion using electroimmunoassay of platelet extracts and electron microscopy of the platelets incubated with anti-VIIIR:Ag antibodies coupled to peroxidase. No VIIIR-Ag was detected in or around the patients' platelets, although this antigen was detected in the circulating blood. These results that there was no VIIIR:Ag uptake from the plasma by the platelets and that platelet VIIIR:Ag came from megakaryocytes.



2019 ◽  
Vol 36 (1) ◽  
pp. 51-61 ◽  
Author(s):  
Janet H. Ford ◽  
Krista Schroeder ◽  
Dawn C. Buse ◽  
Shivang Joshi ◽  
Steven Gelwicks ◽  
...  


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