Effect of FS (α2γβs) hybrid hemoglobin on Hb S nucleation and aggregation

Author(s):  
Keiko Nibu ◽  
Kazuhiko Adachi
Keyword(s):  
Hb S ◽  
2021 ◽  
Vol 14 (1) ◽  
pp. e238317
Author(s):  
Nibash Budhathoki ◽  
Sunita Timilsina ◽  
Bebu Ram ◽  
Douglas Marks

Prevalence of haemoglobin sickle-β+ thalassaemia (Hb S/β+thal) is variable with geography ranging from 0.2% to 10% among sickle cell patients. Clinical presentation of Hb S/β+thal patients depends on HbA level, with milder disease often going undiagnosed. However, rarely these patients can present with a fulminant vaso-occlusive crisis (VOC). Given VOC can present with non-specific symptoms, the diagnosis and treatment is often delayed. Here, we present a patient who initially developed altered mental status, pancytopenia and multiorgan failure due a critical VOC resulting in bone marrow necrosis and fat embolism. Subsequent workup confirmed that our patient had Sickle-β+ thalassaemia, which had gone undiagnosed, despite subclinical evidence of haemolysis on routine lab work for years. Following diagnosis and initiation of RBC exchange, he improved significantly and was discharged home. High index of suspicion and bone marrow biopsy is vital for early diagnosis and management of this rare condition.


1980 ◽  
Vol 14 (3) ◽  
pp. 266-267 ◽  
Author(s):  
A E Felice ◽  
S M Mayson ◽  
B B Webber ◽  
A Miller ◽  
M E Gravely ◽  
...  
Keyword(s):  

Hemoglobin ◽  
2002 ◽  
Vol 26 (1) ◽  
pp. 13-20 ◽  
Author(s):  
Claude Préhu ◽  
Jean Riou ◽  
Isabelle Sartelet ◽  
Danielle Promé ◽  
Catherine Claparols ◽  
...  
Keyword(s):  
Hb S ◽  

1988 ◽  
Vol 70 (1) ◽  
pp. 85-89 ◽  
Author(s):  
J. M. Gonzalez-Redondo ◽  
F. Kutlar ◽  
A. Kutlar ◽  
T. A. Stoming ◽  
J. MA. Pablos ◽  
...  
Keyword(s):  
Hb S ◽  

2010 ◽  
Vol 32 (1) ◽  
pp. 80-82
Author(s):  
Natália Ferreira ◽  
Paula J. A. Zamaro ◽  
Claudia R. Bonini-Domingos
Keyword(s):  

2020 ◽  
Vol 8 (10) ◽  
pp. 332-334
Author(s):  
Hira Chaudhary ◽  
Peter Gillette ◽  
Michael Ashamalla ◽  
Angeleque Hartt ◽  
Moro Salifu ◽  
...  

2021 ◽  
Author(s):  
S Mazzotti ◽  
John Townend

We use a Bayesian analysis to determine the state of stress from focal mechanisms in ten seismic zones in central and eastern North America and compare it with regional stress inferred from borehole measurements. Comparisons of the seismologically determined azimuth of the maximum horizontal compressive stress (S HS ) with that determined from boreholes (S HB ) exhibit a bimodal pattern: In four zones, the S HS and regional S HB orientations are closely parallel, whereas in the Charlevoix, Lower St. Lawrence, and Central Virginia zones, the S HS azimuth shows a statistically significant 30°-50° clockwise rotation relative to the regional S HB azimuth. This pattern is exemplified by the northwest and southeast seismicity clusters in Charlevoix, which yield S HS orientations strictly parallel and strongly oblique, respectively, to the regional S HB trend. Similar ~30° clockwise rotations are found for the North Appalachian zone and for the 2003 Bardwell earthquake sequence north of the New Madrid zone. The S HB /S HS rotations occur over 20-100 km in each seismic zone, but they are observed in zones separated by distances of up to 1500 km. A possible mechanism for the stress rotations may be the interaction between a long-wavelength stress perturbation source, such as postglacial rebound, and local stress concentrators, such as low-friction faults. The latter would allow low-magnitude (<10 MPa) postglacial rebound stresses to locally perturb the preexisting stress field in some seismic zones, whereas postglacial rebound stresses have little effect on the intraplate state of stress in general. © 2010 Geological Society of America.


2015 ◽  
Vol 3 (3) ◽  
Author(s):  
Thayna Nogueira Dos Santos ◽  
Maritza Cavalcante Barbosa ◽  
Talyta Ellen De Jesus Dos Santos ◽  
Davi Damasceno e Silva Diniz ◽  
Vanessa Páscoa Lemos ◽  
...  
Keyword(s):  

As hemoglobinas variantes, ou anormais, são decorrentes de alterações estruturais na hemoglobina que promovem a formação de moléculas com características bioquímicas diferentes das hemoglobinas normais. O objetivo do estudo foi investigar a frequência de hemoglobinas variantes na população do Estado do Ceará. Foram analisadas 298 amostras de sangue venoso de indivíduos adultos, de ambos os sexos, no período de julho de 2009 a julho de 2011. Para cada amostra foram realizados os exames de hemograma e eletroforese de hemoglobina em pH alcalino. Para confirmação dos resultados realizou-se a cromatografia líquida de alta eficiência. A prevalência das hemoglobinas variantes na população estudada foi de 2,01%, sendo 5 (1,68%) amostras Hb AS e 1 (0,33%) Hb AD - Los Angeles. Os dados obtidos confirmam que a Hb S é a hemoglobina variante de maior frequência no Ceará, sendo relevante a realização mais abrangente de triagem para hemoglobinas variantes com a finalidade de identificá-las precocemente, com o objetivo de diminuir o número de nascimentos de indivíduos com a forma grave.


1994 ◽  
Vol 47 (1) ◽  
pp. 33-35
Author(s):  
John S. Waye ◽  
Margaret Patterson ◽  
Barry Eng ◽  
David H. K. Chui ◽  
Graham D. Sher ◽  
...  
Keyword(s):  

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