scholarly journals Synergistic Integration of Laboratory and Numerical Approaches in Studies of the Biomechanics of Diseased Red Blood Cells

Biosensors ◽  
2018 ◽  
Vol 8 (3) ◽  
pp. 76 ◽  
Author(s):  
He Li ◽  
Dimitrios Papageorgiou ◽  
Hung-Yu Chang ◽  
Lu Lu ◽  
Jun Yang ◽  
...  
Keyword(s):  
Hereditary Spherocytosis ◽  
Cell Disease ◽  
Cell Deformability ◽  
Hematological Disorders ◽  
Pathological Conditions ◽  
Cell Stability ◽  
Rbc Models ◽  
Extracellular Environment ◽  
Recent Laboratory ◽  
Numerical Approaches

In red blood cell (RBC) disorders, such as sickle cell disease, hereditary spherocytosis, and diabetes, alterations to the size and shape of RBCs due to either mutations of RBC proteins or changes to the extracellular environment, lead to compromised cell deformability, impaired cell stability, and increased propensity to aggregate. Numerous laboratory approaches have been implemented to elucidate the pathogenesis of RBC disorders. Concurrently, computational RBC models have been developed to simulate the dynamics of RBCs under physiological and pathological conditions. In this work, we review recent laboratory and computational studies of disordered RBCs. Distinguished from previous reviews, we emphasize how experimental techniques and computational modeling can be synergically integrated to improve the understanding of the pathophysiology of hematological disorders.

scholarly journals Laparoscopic splenectomy: how minimal can we make it?

2022 ◽  
Author(s):  
Adetokunbo Fadipe ◽  
David Wilkinson ◽  
Robert Peters ◽  
Catherine Doherty ◽  
Nick Lansdale
Keyword(s):  
Length Of Stay ◽  
Laparoscopic Splenectomy ◽  
Operative Time ◽  
Hereditary Spherocytosis ◽  
Surgical Trauma ◽  
Cell Disease ◽  
Median Length ◽  
Post Operative Pain ◽  
Age At Surgery ◽  
Splenic Size

Abstract Aims Laparoscopic splenectomy (LS) is routinely performed in children, however, a large spleen in a small child can pose significant operative challenges. We instigated a highly standardised surgical and anaesthetic approach to LS to minimise surgical trauma and enhance recovery. The aim of this study was to assess the outcomes of this programme. Methods Prospective study of all LS’s performed 2018–2021. Surgical approach was via one 10 mm and three 5 mm ports. Early hilar control was accomplished with Hem-o-loks. Splenic retrieval via the 10 mm incision used finger morcellation within an Espiner EcoSac. Anaesthesia utilised a standardised regime of agents and bupivacaine was infiltrated to the splenic bed and wound sites. Post-operative opiates were minimised. Data are presented as median [IQR]. Results Twenty consecutive children were included. Indications for LS were hereditary spherocytosis (n = 12), sickle cell disease (n = 6), beta-thalassaemia (n = 1) and splenic haemangiomatosis (n = 1). Age at surgery was 101 months [75–117] and weight 30 kg [21–37]. Splenic size was 13.4 cm [12–14.4]. Operative time was 178 min [156–185]. There were no open conversions and no significant intra or post-operative bleeding. One patient developed pancreatitis. Median post-operative pain score was 1 [1–3]. Median length of stay was 2 days [2–3]. Conclusion LS is feasible, safe and efficient in smaller children with large spleens. This standardised programme of anaesthesia and surgery based around a core team reliably results in few complications, good analgesia and short length of stay.

Long‐term hematologic and clinical outcomes of splenectomy in children with hereditary spherocytosis and sickle cell disease

2020 ◽  
Vol 67 (8) ◽  
Author(s):  
Bria J. Hall ◽  
Audra J. Reiter ◽  
Brian R. Englum ◽  
Jennifer A. Rothman ◽  
Henry E. Rice ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Clinical Outcomes ◽  
Sickle Cell ◽  
Hereditary Spherocytosis ◽  
Cell Disease

scholarly journals Management of a case of supra-acute immune thrombocytopenic purpura in pregnancy finalized with splenectomy

2015 ◽  
Vol 10 (1) ◽  
pp. 73-78
Author(s):  
Adriana NICA ◽  
◽  
Ana Maria VLĂDĂREANU ◽  
Minodora ONISÂI ◽  
Ion DUMITRU ◽  
...  
Keyword(s):  
Hereditary Spherocytosis ◽  
Red Cell ◽  
Therapeutic Procedure ◽  
Thrombocytopenic Purpura ◽  
Hematological Disorders ◽  
Wide Range ◽  
Haemolytic Anemia ◽  
Immunosuppressant Treatment ◽  
In Pregnancy

Splenectomy is a therapeutic procedure for a wide range of conditions, mainly hematological disorders. From the hematological disorders we can mention the red cell disorders: haemolytic anemia, hereditary spherocytosis and thalassemia, that have indication for splenectomy in case of failure to the drug treatment. A wide variety of thrombocytopenic disorders are improved by splenectomy, and the most common indicatios is for Idiopatic Thrombocitopenic Purpura (ITP). However, splenectomy imparts a high risk of fulminant infections that can be avoided with proper prophylaxis. Although in ITP splenectomy in not curative, it does not remove the autoimmune component, it will lead to relief of symptoms, improved platelet count with the cessation of the immunosuppressant treatment and better quality of life.

scholarly journals The incidence of painful crisis in homozygous sickle cell disease: correlation with red cell deformability

Blood ◽  
1988 ◽  
Vol 72 (6) ◽  
pp. 2056-2059
Author(s):  
WM Lande ◽  
DL Andrews ◽  
MR Clark ◽  
NV Braham ◽  
DM Black ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Hemoglobin Concentration ◽  
Red Cells ◽  
Strong Positive Correlation ◽  
Red Cell ◽  
Cell Disease ◽  
Cell Deformability ◽  
Red Cell Deformability ◽  
Cellular Dehydration ◽  
Painful Crisis

To determine whether the vasoocclusive severity of homozygous sickle cell (SS) disease is influenced by cellular dehydration, we correlated the incidence of painful crisis with steady-state measurements of red cell hydration. Sixteen children with SS disease were followed for 3.3 to 8 years (mean, 6.8 years), and a single crisis rate was calculated for each patient. At the time of well visits, cellular hydration was assessed by measuring cell deformability, the percentage of red cells with a density greater than or equal to 1.1056 g/mL, and the percentage of irreversibly sickled cells (ISC). The incidence of painful crisis showed a strong positive correlation with Omax, a deformability measurement reflecting cellular hydration (r = .84, P less than .002), and with hemoglobin concentration (r = .59, P = .04). That is, higher crisis rates were observed in patients with less dehydrated, more deformable red cells and also in patients with higher hemoglobin concentrations. Furthermore, cell deformability and hemoglobin concentration were independent predictors of the incidence of painful crisis, which is consistent with separate effects of these two red cells parameters on vasoocclusive severity.

scholarly journals Human red cell membrane adenylate cyclase in normal subjects and patients with hereditary spherocytosis, sickle cell disease and unidentified hemolytic anemias

Blood ◽  
1980 ◽  
Vol 56 (6) ◽  
pp. 963-968 ◽  
Author(s):  
JP Piau ◽  
J Delaunay ◽  
S Fischer ◽  
M Tortolero ◽  
G Schapira
Keyword(s):  
Sickle Cell Disease ◽  
Adenylate Cyclase ◽  
Red Blood Cells ◽  
Sickle Cell ◽  
Blood Cells ◽  
Hereditary Spherocytosis ◽  
Reticulocyte Count ◽  
Cyclase Activity ◽  
Adenylate Cyclase Activity ◽  
Cell Disease

We have investigated adenylate cyclase in ghosts from normal and pathologic human red blood cells. Basic parameters such as specific activity, apparent Michaelis constant (KMapp), and response to effectors: sodium fluoride (NaF), 5′-guanylyl imidodiphosphate (Gpp (NH)p), isoproterenol, and PGE1 were investigated. Basal and NaF- stimulated activities were measured in ghosts from patients with hereditary spherocytosis, sickle cell disease, and various unidentified hemolytic anemias. Both activities were increased in any of these pathologic conditions as compared with those of normal red blood cells. Normal values were found in patients with hereditary spherocytosis after splenectomy and in patients with heterozygous sickle cell disease. There was a good correlation between the reticulocyte count and adenylate cyclase activity in hereditary spherocytosis and in sickle cell disease with reticulocyte count lower than 10%. The enzyme activity of the first group was about three times that of the second group. There was no correlation at all in sickle cell disease with higher reticulocytosis and in the group of unidentified hemolytic anemias. These results suggest that increased adenylate cyclase activities are not specific of any of these diseases. In the patients with hereditary spherocytosis, the adenylate cyclase activity seems to be essentially related to younger mean age of red blood cell population while in the patients with sickle cell disease and in others with unidentified hemolytic anemias some additional factors might interfere directly with the enzyme and alter its activity.

scholarly journals Prospective Newborn Screening for Sickle Cell Disease and Other Inherited Blood Disorders in Central Malawi

2021 ◽  
Vol 66 ◽  
Author(s):  
Gerald Tegha ◽  
Hillary M. Topazian ◽  
Portia Kamthunzi ◽  
Thad Howard ◽  
Zondwayo Tembo ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Newborn Screening ◽  
Sickle Cell ◽  
Early Identification ◽  
G6pd Deficiency ◽  
The United States ◽  
Sub Saharan Africa ◽  
Cell Disease ◽  
Hematological Disorders ◽  
Alpha Thalassemia

Objectives: Newborn screening in the United States and Europe allows early identification of congenital disorders but does not yet exist in most low-resource settings, especially in sub-Saharan Africa. Newborn screening can identify multiple inherited hematological disorders, but feasibility and effectiveness for Africa are not fully determined.Methods: Surplus dried blood spot collected in Central Malawi through the HIV Early Infant Diagnosis surveillance program were repurposed and tested by isoelectric focusing for sickle cell disease and trait. Additional genetic testing identified G6PD deficiency and alpha thalassemia.Results: Testing of 10,529 cards revealed an overall sickle cell trait prevalence of 7.0% (range 3.9–9.7% by district); 10 of 14 infants identified with sickle cell disease (prevalence 0.1%) were located and received care at a specialized clinic. Subsequent testing of 1,329 randomly selected cards identified alpha thalassemia trait in 45.7% of samples, and G6PD deficiency in 20.4% of males and 3.4% of females, with 29.0% of females as heterozygous carriers.Conclusion: Inherited hematological disorders are common in Central Malawi; early identification through newborn screening can improve clinical outcomes and should be supported throughout Africa.

Prädilektion des Malleolus medialis beim mit hämolytischer Anämie assoziierten Ulcus cruris

VASA ◽  
2002 ◽  
Vol 31 (3) ◽  
pp. 191-193 ◽  
Author(s):  
Sawhney ◽  
Weedon ◽  
Gillette ◽  
Solomon ◽  
Braverman
Keyword(s):  
Sickle Cell ◽  
Hereditary Spherocytosis ◽  
Ulcus Cruris ◽  
Venous Stasis ◽  
Leg Ulcers ◽  
Cell Disease ◽  
Common Site ◽  
The Difference ◽  
Ischemic Ulcers ◽  
Chronic Hemolytic Anemia

Backround: Ischemic ulcers are usually found above the lateral, and venous stasis ulcers at the medial malleoli. Leg ulcers occur in at least 25% of sickle cell disease (SSD) patients in clinic populations, usually in the malleolar region. The function of the large leg veins in most SSD patients is unimpaired. Patients and methods: We determined leg ulcer location in 41 sickle cell anemia (SS), and 4 sickle-b0 thalassemic patients with longstanding chronic and/or recurrent leg ulceration, and reviewed published reports of leg ulcers in hereditary spherocytosis and thalassemias. Results: Of the 57 legs of the 45 SSD patients with only 1 ulcer, 42 (74%) were medial and 15 lateral. The difference was significant (p < 0.001). Of patients with only a single ulcer, 22 were medial and 4 lateral. Of 15 reported patients with leg ulcers related to spherocytosis or thalassemia, 20/24 (83%) ulcers were medial. Conclusions: The medial malleoli are the most common site of leg ulceration in SSD and in other chronic hemolytic anemias. This suggests that stasis may play a role in the leg ulceration associated with chronic hemolytic anemia.

scholarly journals Human red cells protein kinase in normal subjects and patients with hereditary spherocytosis, sickle cell disease, and autoimmune hemolytic anemia

Blood ◽  
1976 ◽  
Vol 48 (6) ◽  
pp. 887-898 ◽  
Author(s):  
E Beutler ◽  
E Guinto ◽  
C Johnson
Keyword(s):  
Protein Kinase ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Kinase Activity ◽  
Hereditary Spherocytosis ◽  
Cell Protein ◽  
Protein Kinase Activity ◽  
Red Cell ◽  
Cell Disease ◽  
Membrane Phosphorylation

Abstract A somewhat simplified modification of a previously described method for the measurement of red cell membrane phosphorylation by ATP has been devised. Phosphorylation of membranes was linear with time for only 5- 10 min, and linearity with membrane concentration was observed only when assays were limited to short incubation times. Protein kinase activity of hereditary spherocytosis (HS) membranes was found to be normal. However, the average phosphorylation after 60 min incubation was less in HS membranes than in normal membranes. Findings similar to those in HS membranes were observed in sickle cell disease. The Km of red cell protein kinase for ATP is approximately 10(-5) M. Membrane phosphate binding sites are not saturated in either HS or normal membranes after 1 hr incubation with ATP. Approximately 27% of phosphorylating activity is lost after 1 hr incubation at 37 degrees C. GTP is a very inefficient phosphate donor. Under the conditions of measurement employed, the enzyme is slightly stimulated by 1 muM cAMP, but is not stimulated by 1 muM cGMP. Dephosphorylation of red cell membranes after labeling occurs at a similar rate in HS as in normal membranes. Although a mild abnormally in membrane phosphorylation is observed in HS, this could not be demonstrated to be due to a decrease in protein kinase activity or in alterations of its kinetic properties. The abnormally seen is not specific for HS.

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