The Effect of α-Thalassemia on Cord Blood Red Cell Indices and Interaction with Sickle Cell Gene

2000 ◽  
Vol 20 (5-6) ◽  
pp. 367-370 ◽  
Author(s):  
Mohammad I. Quadri ◽  
Sherief I.A.M. Islam ◽  
Zaki Nasserullah
Keyword(s):  
Cord Blood ◽  
Sickle Cell ◽  
Red Cell ◽  
Red Cell Indices ◽  
Cell Gene

Arginine Supplementation of Sickle Transgenic Mice Leads to a Rapid Decrease in MCHC and Percent Dense Cells and a Slow Increase When Supplementation Is Discontinued.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 3579-3579
Author(s):  
Mary E. Fabry ◽  
Zipora Etzion ◽  
Robert M. Bookchin ◽  
Sandra M. Suzuka ◽  
Ronald L. Nagel
Keyword(s):  
Nitric Oxide ◽  
Transgenic Mice ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
High Density ◽  
Red Cells ◽  
Red Cell ◽  
Red Cell Indices ◽  
Plasma Arginine ◽  
Arginine Supplementation

Abstract Nitric oxide (NO) is produced from arginine by nitric oxide synthase (NOS) and is essential to the maintenance of vascular tone. Plasma arginine levels are reduced in sickle cell anemia patients, and we have previously reported that S+S− Antilles mice have lower plasma arginine than control mice (C57BL). Long term arginine supplementation (5% arginine in chow) restored plasma arginine levels in S+S− Antilles mice to that found in C57BL mice, and, in S+S− Antilles mice, MCHC and the percent high density red cells were reduced. Our observation that Ca++-activated K+ channel [K(Ca) channel or Gardos channel] activity is reduced in supplemented vs non-supplemented S+S− Antilles mice can account for reduced red cell density and dense cell formation (Blood99: 1103, 2002). The time course of arginine induced changes in red cell indices in sickle transgenic mice has not yet been studied. Using the Advia 120 Hematology System, we found that, within 7 days after the onset of arginine supplementation, MCHC* (CHCM in the Advia system) fell from 33.4±0.5 g/dL to 31.0±0.2 (mean ±SD, P<0.000002, N=6) and the percent high density red cells (MCHC>37 g/dL) decreased from 10.5±4.3 % to 4.5±1.6 (P<0.01, N=6). MCV rose from 43.6±0.8 fL to 46.4±0.5 (P<0.00003, N=6). Reticulocyte counts and mean corpuscular hemoglobin did not vary significantly. Although plasma arginine levels increase within a few hours of oral administration and persist for only a few hours, no change in red cell indices was observed 7 days after discontinuing arginine supplementation, and the indices slowly returned to baseline levels after 40 days. In summary, MCHC* and the percent high density RBCs decrease significantly within 7 days of the onset of arginine supplementation. When arginine supplementation is withdrawn, the return to baseline values is much slower and required more than 40 days to complete. These observations have important implications for arginine supplementation in sickle cell anemia patients. In particular, the persistence of decreased MCHC* and reduced percent high density RBCs for an extended period of time after supplementation was withdrawn parallels our previously reported observation of persistent improvement of perfusion in sickle transgenic mice after supplementation was withdrawn and suggests that daily administration of arginine may not be necessary once an induction period has passed.

The effect of Hb F and α-thalassemia on the red cell indices in sickle cell anemia

1986 ◽  
Vol 21 (4) ◽  
pp. 383-395 ◽  
Author(s):  
Paul F. Milner ◽  
George J. Garbutt ◽  
L. V. Nolan-Davis ◽  
Felix Jonah ◽  
Lois B. Wilson ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Red Cell ◽  
Red Cell Indices

Predictive Value of Red Cell Indices and High Performance Liquid Chromatography in the Diagnosis of Haemoglobinopathies: Interim Results of the National Neonatal Cord Blood Screening Programme.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 3352-3352
Author(s):  
Anil Pathare ◽  
Salam AlKindi ◽  
Ali AlMadhani ◽  
Shoaib AlZadjali ◽  
Hamood AlHaddabi ◽  
...  
Keyword(s):  
Cord Blood ◽  
Clinical Care ◽  
Globin Gene ◽  
Mononuclear Leukocytes ◽  
Red Cell ◽  
Mean Cell Volume ◽  
Distribution Width ◽  
Red Cell Indices ◽  
Blood Screening ◽  
Rbc Count

Abstract Objective: The aim of the study was to ascertain the feasibility of cord blood screening to determine the prevalence of haemoglobinopathies by a cost-effective method. Background: High rate of consanguinity and intercousin marraiges are prevalent in the Sultanate of Oman, leading to an increase in haemoglobinopathies, which is of growing importance as knowledge of a population structure can be a unique aid in planning genetic services. Methods: 1864 consecutive cord blood samples were screened by HPLC using Biorad Variant II program between April 2005 & March 2006. Complete blood counts [CBC] were also obtained on Cell Dyn 4000 automated blood cell counter. All samples were then processed to isolate and store mononuclear leukocytes for subsequent molecular diagnostics. Results: We observed a 46.83% incidence of α-thalassaemia, based on significant amounts of Hb Barts on HPLC and low mean cell volume [MCV] & mean cell haemoglobin [MCH] on the CBC. Table : Neonatal Cord Blood Screening - HPLC and CBC data with Red Cell Indices Normal ATT HbS HbD HbE HbC ?BTT Values in parenthesis represent SD Mean HbF % 77.4[7.3] 76.4[8.7] 89.4[9.4] 78.1[1.2] 81.1[4.9] 80.2[7.8] 92.1[2.0] Mean HbA % 22.5[7.3] 23.6[8.2] 14.1[6.0] 13.7[9.9] 12.7[3.1] 13.1[4.5] 8.1[2.1] Mean Haemoglobin g/dl 15.4[1.8] 14.4[1.4] 14.7[1.6] 14.9[0.7] 15.6[1.1] 15.2[0.8] 13.9[2.5] Mean RBC Count × 1012/L 4.5[0.6] 5.0[0.6] 4.9[0.6] 4.5[0.4] 4.8[0.4] 4.6[0.2] 4.0[0.6] Mean MCV fl 105.2[5.4] 89.9[4.1] 95.1[9.1] 100.8[7.0] 98.1[4.7] 99.1[5.6] 104.4[9.5] Mean MCH pg 34.4[2.1] 28.5[1.8] 30.7[3.4] 33.3[2.7] 32.6[3.3] 32.9[1.2] 34.8[1.0] Mean MCHC g/dL 32.7[1.1] 31.7[1.3] 32.2[1.0] 33.1[0.9] 33.6[2.1] 33.2[1.5] 33.3[1.0] Mean RDW % 16.1[1.1] 17.4[2.1] 17.1[2.4] 16.1[1.5] 17.7[2.4] 16.5[1.9] 16.1[2.5] Furthermore, the overall incidence of other haemoglobinopathies was 9.39%, with 6.01% incidence of sickle haemoglobin. On HPLC, D-window, E-window and C-window were present in 1.02%, 0.32% and 0.11% of the samples respectively. Figure: Distribution of abnormal Haemoglobins in Newborns Figure:. Distribution of abnormal Haemoglobins in Newborns Since HPLC cannot diagnose β-thalassemia major at birth, in samples with HbA below 10%, the beta globin gene was directly sequenced including the promoter, all exons and introns in the abnormal samples. [n=36] Additionally, direct sequencing of abnormal samples with HbS,[n=112] HbD,[n=19], HbE[n=6] and HbC[n=2] were also performed to validate the HPLC results. Conclusions: It is emphasized that neonatal cord blood screening is an important the first step in the national strategy towards total management of haemoglobinopathies including early diagnosis, comprehensive clinical care and counseling of the affected families. Between group differences were significant for RBC count, MCV, MCH, MCHC and the red cell distribution width (RDW), which along with Hb Barts, and HPLC results can successfully predict the correct underlying diagnosis.

scholarly journals Determination of Red Cell Indices Cut-off for Identifying Iron Deficiency Anaemia among Pre-school Children with Sickle Cell Anaemia

2020 ◽  
pp. 357-364
Author(s):  
OS Akodu ◽  
FA Adekanmbi ◽  
TA Ogunlesi
Keyword(s):  
Iron Deficiency ◽  
Sickle Cell ◽  
School Children ◽  
Iron Deficiency Anaemia ◽  
Sickle Cell Anaemia ◽  
Red Cell ◽  
Predictive Values ◽  
Deficiency Anaemia ◽  
Red Cell Indices ◽  
Discriminatory Performance

Nigerian pre-school children have a high risk of developing iron deficiency and there is no consistent evidence that patients with sickle cell anaemia are protected from iron deficiency anaemia. The objective is to explore red cell indices cut-off values useful as surrogate for detecting iron deficiency in children with sickle cell anaemia. Ninety-seven children with sickle cell anaemia were recruited from Children Outpatient. Reference intervals were developed using the 2.5th – 97.5th, 3.0rd – 97.0th, 5 – 95th, and 10th – 90th percentile intervals for MCV and MCH. The discriminatory performance of the proposed red cell indices criterion was assessed by use of sensitivity, specificity, accuracy, likelihood ratio and predictive values. The 2.5th, 3rd, 5th, 10th, 90th, 95th, 97th, and 97.5th percentile values were: MCV (62.7, 63.6, 66.5, 69.6, 86.3, 87.7, 89.5, and 90.1fl), and MCH (19.0, 19.5, 20.8, 21.4, 28.2, 29.1, 29.5 and 29.7pg). The various calculated cut-off points for the MCV and MCH had lower sensitivity but a higher specificity for detecting iron deficiency than the standard reference values for the general population. The calculated cut-off point for the study subjects below the 10th percentiles had the best discriminatory performance. The cut-off for iron deficiency was 69.6fl for MCV and 21.4pg for MCH either use singly or in combination. In conclusion, standard reference cut-offs of MCV and MCH based on results from western individuals without sickle cell anaemia of the same age are not in agreement with the estimated values for children with sickle cell anaemia in Nigeria.

The orientation of sickle hemoglobin fibers within fascicles

1988 ◽  
Vol 46 ◽  
pp. 400-401
Author(s):  
Christopher A. Miller ◽  
Bridget Carragher ◽  
William A. McDade ◽  
Robert Josephs
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Relative Orientation ◽  
Unit Cell ◽  
Red Cell ◽  
High Ph ◽  
Sickle Hemoglobin ◽  
Polar Crystal ◽  
Helical Configuration

Highly ordered bundles of deoxyhemoglobin S (HbS) fibers, termed fascicles, are intermediates in the high pH crystallization pathway of HbS. These fibers consist of 7 Wishner-Love double strands in a helical configuration. Since each double strand has a polarity, the odd number of double strands in the fiber imparts a net polarity to the structure. HbS crystals have a unit cell containing two double strands, one of each polarity, resulting in a net polarity of zero. Therefore a rearrangement of the double strands must occur to form a non-polar crystal from the polar fibers. To determine the role of fascicles as an intermediate in the crystallization pathway it is important to understand the relative orientation of fibers within fascicles. Furthermore, an understanding of fascicle structure may have implications for the design of potential sickling inhibitors, since it is bundles of fibers which cause the red cell distortion responsible for the vaso-occlusive complications characteristic of sickle cell anemia.

Sibling Donor Cord Blood Banking for Children with Sickle Cell Disease

2001 ◽  
Vol 20 (2) ◽  
pp. 167-174 ◽  
Author(s):  
William Reed ◽  
Mark Walters ◽  
Elizabeth Trachtenberg ◽  
Renee Smith ◽  
Bertram Lubin
Keyword(s):  
Sickle Cell Disease ◽  
Cord Blood ◽  
Sickle Cell ◽  
Cell Disease ◽  
Blood Banking ◽  
Sibling Donor ◽  
Cord Blood Banking

Overview of the World Congress on cord blood and innovative approaches to the treatment of sickle-cell anemia in Monaco on 24-27th october 2013

2014 ◽  
Vol 2 (1) ◽  
pp. 95-98
Author(s):  
V. Kyryk
Keyword(s):  
Cord Blood ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
World Congress ◽  
The World ◽  
Innovative Approaches

Overview of the World Congress on cord blood and innovative approaches to the treatment of sickle-cell anemia in Monaco on 24-27th october 2013

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