scholarly journals EFFECT OF AGE, AND SPLEEN AND TREATMENT STATUS ON MALE REPRODUCTIVE HORMONES AND SOME PHYSIOLOGICAL PARAMETER LEVELS IN PATIENTS WITH BETA-THALASSEMIA MAJOR

2019 ◽  
pp. 213-216
Author(s):  
JAMELA JOUDA ◽  
RAJWAN ABDULJABBAR GHAZZAY ◽  
WAFAA F AL-MOSAWY
Keyword(s):  
Thalassemia Major ◽  
Reproductive Hormones ◽  
Beta Thalassemia ◽  
Physiological Parameter ◽  
Abo Blood Groups ◽  
Sexual Hormones ◽  
Treatment Status ◽  
High Level ◽  
The Relationship ◽  
Effect Of Age

Objective: This research was conducted to study the relationship between spleen status and chelation treatment status with the male reproductive hormones and some physiological parameter levels. Methods: A total of 35 males with thalassemia major (TM) patients are recruited. Patients are grouped according to (1) their age into two groups; ˂18 years and ≥18 years, (2) their spleen status into two groups; splenectomize and non-splenectomize, and (3) their chelation treatment status into two groups; regulation and irregulation. Body mass index (BMI) was calculated and blood was collected from all patients just before blood transfusion session. Hb, ABO blood groups, ferritin, and hormone (luteinizing hormone [LH], follicle-stimulating hormone [FSH], testosterone, prolactin, and cortisone) levels were determined. Results: The highest frequency of thalassemia was in Group O and the lowest was in AB. BMI was higher in TM patients aged ≥18 years, TM patients with splenectomize, and TM patient who take the treatment irregularly. TM patients aged ≥18 years have high level of LH, FSH, and testosterone. TM patients without splenectomize have higher FSH, prolactin, and testosterone. TM patients take the treatment regularly have significantly higher testosterone levels and non-significantly lower FSH level. Conclusion: The levels of cortisol and prolactin hormones are not disturbed in TM patients and not associated with the rate of transfusion, but the level of Hb and ferritin leads to underweight in BMI and may lead to endocrine dysfunction, especially sexual hormones (FSH, LH, and testosterone). These BMI and sex hormones are related to age, spleen, and treatment status.

scholarly journals Relationship of Reproductive Hormones and Fertility in Patients with Beta-Thalassemia Major

2021 ◽  
Vol 15 (2) ◽  
Author(s):  
A. D. Mays ◽  
M. M. Abdulrasool ◽  
Mustafa Mudhafar ◽  
H. A. Alsailawi ◽  
Abbas M. Bashi ◽  
...  
Keyword(s):  
Thalassemia Major ◽  
Reproductive Hormones ◽  
Beta Thalassemia ◽  
Beta Thalassemia Major ◽  
Relationship Of

scholarly journals ASSESSMENT OF SERUM SCLEROSTIN LEVEL AS A BIOMARKER ASSOCIATED WITH BONE DISORDERS IN Β-THALASSEMIA PATIENTS IN AL- NAJAF CITY, IRAQ

2022 ◽  
Author(s):  
Intisar Razzaq SHARBA ◽  
◽  
Arshad Noori AL-DUJAILI
Keyword(s):  
Serum Iron ◽  
Hematological Parameters ◽  
Thalassemia Major ◽  
Normal Weight ◽  
Beta Thalassemia ◽  
Healthy Controls ◽  
Age Group ◽  
The Relationship ◽  
Sclerostin Level ◽  
Weight Groups

Aim of the study: To assess serum sclerostin in female patients with beta-thalassemia and compare with the healthy controls and to predict its complication associated with the bone pathophysiology, for designed improvement the lifestyle goodliness for these patients. Material and methods: Sixty-nine female beta-thalassemia (βT) patients (54 βT major and 15 βT Intermedia), aged 8-40 years who dependent on transfused blood, and 20 healthy controls were evaluated serum sclerostin, and was examined the relationship with hematological parameters RBC, Hb, PCV, WBC, PLT, BMI, splenic status, iron, and ferritin levels. The information of beta-thalassemia patients was collected and recorded by the questioner. Results: A significantly increased serum sclerostin level (mean 26.80±0.91) pg/ml was shown in βT patients compared with the healthy controls (10.03±0.68, p < 0.001) pg/ml. Furthermore, a significant decrease (p<0.05) of the sclerostin level was observed in β-thalassemia major compared to intermedia β-thalassemia patients. Serum sclerostin level revealed a significant increase in progress age; it is highest in the age group (30-40) year as compared with age group (8-18) and (19-29) year respectively. Sclerostin showed no associations with the RBC, Hb, PCV, and significantly positively correlated (p<0.05) with serum iron, ferritin levels, WBC, and PLT count. Significantly higher sclerostin levels in splenectomized and underweight groups were observed compared to unsplenectomized and normal-weight groups (p<0.05) of βT patients. Conclusion: Sclerostin plays an important role in beta-thalassemia patients and can serve as a biomarker associated with the bone pathophysiology and indicator to prevent the continuation of such serious diseases caused by iron overload in these patients.

scholarly journals Advances in stem cell transplantation and gene therapy in the β-hemoglobinopathies

Hematology ◽  
2012 ◽  
Vol 2012 (1) ◽  
pp. 276-283 ◽  
Author(s):  
Emmanuel Payen ◽  
Philippe Leboulch
Keyword(s):  
Gene Therapy ◽  
Stem Cell ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Hematopoietic Stem ◽  
Transfer Vectors ◽  
Lentiviral Gene Therapy ◽  
High Level ◽  
Cell Gene ◽  
Level Production

Abstract High-level production of β-globin, γ-globin, or therapeutic mutant globins in the RBC lineage by hematopoietic stem cell gene therapy ameliorates or cures the hemoglobinopathies sickle cell disease and beta thalassemia, which are major causes of morbidity and mortality worldwide. Considerable efforts have been made in the last 2 decades in devising suitable gene-transfer vectors and protocols to achieve this goal. Five years ago, the first βE/β0-thalassemia major (transfusion-dependent) patient was treated by globin lentiviral gene therapy without injection of backup cells. This patient has become completely transfusion independent for the past 4 years and has global amelioration of the thalassemic phenotype. Partial clonal dominance for an intragenic site (HMGA2) of chromosomal integration of the vector was observed in this patient without a loss of hematopoietic homeostasis. Other patients are now receiving transplantations while researchers are carefully weighing the benefit/risk ratio and continuing the development of further modified vectors and protocols to improve outcomes further with respect to safety and efficacy.

scholarly journals Determining the Effect of Age on the Reproductive and Stress Physiology of Octopus Bimaculoides Using Dermal Hormones

2021 ◽  
Author(s):  
Stephanie Chancellor ◽  
Bret Grasse ◽  
Taylor Sakmar ◽  
David Scheel ◽  
Joel S. Brown ◽  
...  
Keyword(s):  
Stress Test ◽  
Fold Increase ◽  
Reproductive Hormones ◽  
Stress Physiology ◽  
Reproductive State ◽  
Hormone Production ◽  
Non Invasive ◽  
Reproductive Females ◽  
The Relationship ◽  
Effect Of Age

Abstract Our goal was to validate the use of dermal swabs to evaluate both reproductive and stress physiology in the California two-spot octopus, Octopus bimaculoides. Our objectives were to: 1. use a biological stressor to validate glucocorticoid analysis; 2. compare the concentration of reproductive hormones (estrogen and progesterone in females; testosterone in males) of reproductive (N = 4) and senescent (N = 8) individuals to determine the effect of age on hormonal patterns; and 3. determine the relationship between glucocorticoid and reproductive hormone production. For the stress test, individuals were first swabbed and then chased around the aquarium with a net for five minutes. Afterwards, individuals were swabbed for 2 hours at 15-minute intervals to compare to a pre-stress test swab. Reproductive individuals responded to the stressor with a 2-fold peak of cortisol at 15 and 90 minutes. Six of eight senescent individuals did not produce a 2-fold increase in cortisol. Reproductive females had significantly higher sex hormone concentrations (progesterone, estrogen, and testosterone) compared to senescent females. Reproductive males also had significantly higher levels of testosterone compared to their senescent counterparts. After the stressor, only reproductive males produced a 2-fold increase in testosterone concentrations, while sex hormones in females showed no change. Dermal swabs provide an effective and non-invasive means for evaluating octopus hormones, which can provide indicators of both reproductive state and perceptions of acute stressors.

scholarly journals ASSESSMENT OF SERUM SCLEROSTIN LEVEL AS A BIOMARKER ASSOCIATED WITH BONE DISORDERS IN Β-THALASSEMIA PATIENTS IN AL- NAJAF CITY, IRAQ

2021 ◽  
Vol 29 (31) ◽  
pp. 10-20
Author(s):  
SHARBA Intisar Razzaq ◽  
AL-DUJAILI Arshad Noori
Keyword(s):  
Hematological Parameters ◽  
Thalassemia Major ◽  
Normal Weight ◽  
The Body ◽  
Beta Thalassemia ◽  
Healthy Controls ◽  
Age Group ◽  
Blood Disorder ◽  
The Relationship ◽  
Sclerostin Level

Background: β-thalassemia is a blood disorder in which the body does not make hemoglobin normally. Aim: To assess serum sclerostin in female patients with beta-thalassemia and compare with the healthy controls and to predict its complication associated with the bone pathophysiology, for designed improvement the lifestyle goodliness for these patients. Material and methods: Sixty-nine female beta-thalassemia (βT) patients (54 βT major and 15 βT Intermedia), aged 8-40 years who dependent on transfused blood, and 20 healthy controls were evaluated serum sclerostin, and was examined the relationship with hematological parameters RBC, Hb, PCV, WBC, PLT, BMI, splenic status, iron, and ferritin levels. The information of beta-thalassemia patients was collected and records by the questioner. Results: A significantly increased serum sclerostin level (mean 26.80±0.91) pg/ml was showed in βT patients compared with the healthy controls (10.03±0.68, p  smaller than  0.001) pg/ml. Furthermore, a significant decrease (p smaller than 0.05) of the sclerostin level was observed in β-thalassemia major compared to intermedia β-thalassemia patients. Serum sclerostin level revealed a significant increase in progress age; it is highest in the age group (30-40) year as compared with age group (8-18) and (19-29) year respectively. Sclerostin showed no associations with the RBC, Hb, PCV, and significantly positively correlated (p smaller than 0.05) with serum iron, ferritin levels, WBC, and PLT count. Significantly higher sclerostin levels in splenectomized and underweight groups were observed compared to unsplenectomized and normal-weight groups (p smaller than 0.05) of βT patients. Conclusions: Sclerostin plays an important role in beta-thalassemia patients and can serve as a biomarker associated with the bone pathophysiology and indicator to prevent the continuation of such serious diseases caused by iron overload in these patients.

The relationship between coping strategies and resilience among adolescents with beta‐thalassemia major

2021 ◽  
Author(s):  
Ali Mohammad Parviniannasab ◽  
Mahnaz Rakhshan ◽  
Marzieh Momennasab ◽  
Mitra Soltanian ◽  
Mostafa Bijani
Keyword(s):  
Coping Strategies ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Beta Thalassemia Major ◽  
The Relationship

scholarly journals Depression Leads to Physical Inactivity in Patients with Beta-Thalassemia Major

2021 ◽  
Vol 9 (2) ◽  
Author(s):  
Faza Nurul Wardhani ◽  
Susanti Dharmmika ◽  
Hilmi Sulaiman Rathomi
Keyword(s):  
Physical Activity ◽  
Beck Depression Inventory ◽  
Random Sampling ◽  
Simple Random Sampling ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Chi Square ◽  
Global Physical Activity Questionnaire ◽  
The Relationship ◽  
Activity Questionnaire

Beta-thalassemia major (BTM) is difficult to treat chronic disease, causing physical and psychological burdens for the patient. Several studies have confirmed a decrease in physical activity and depression in thalassemia patients, but limited studies examine the relationship between these two conditions. This study aims to analyze the relationship between depression and physical activity in BTM patients in Bandung city. It was analytical observational research with a cross-sectional design. Data were collected during September–December 2018 by interviewing 65 patients selected by simple random sampling from 300 thalassemia patients registered at the Association of Parents with Thalassemia Indonesia/Perhimpunan Orangtua Penderita Thalassemia Indonesia (POPTI) Bandung city. The instruments used were the Global Physical Activity Questionnaire (GPAQ) to measure physical activity and the Beck Depression Inventory (BDI) to assess depressive symptoms. Data were analyzed by chi-square test using SPSS for Windows ver. 23.0. The results showed that most BTM patients in Bandung city were depressed (52%) and had low physical activity levels (65%). Furthermore, there was a statistically significant relationship between depression and physical activity in thalassemia patients in Bandung city (p=0.04, p<0.05). Therefore, it can be concluded that BTM patients in Bandung city with depression have lower physical activity. DEPRESI BERDAMPAK PADA AKTIVITAS FISIK YANG RENDAH PADA PASIEN TALASEMIA BETA MAYORTalasemia beta mayor merupakan penyakit kronis yang sulit disembuhkan sehingga menimbulkan beban fisik dan psikologis bagi pasien. Beberapa penelitian telah mengonfirmasi penurunan aktivitas fisik dan depresi pada pasien talasemia, namun studi yang mengkaji hubungan antara kedua kondisi ini masih terbatas jumlahnya. Penelitian ini bertujuan menganalisis hubungan antara kondisi depresi dan tingkat aktivitas fisik pada penderita talasemia beta mayor di Kota Bandung. Desain penelitian bersifat observasional analitik dengan rancangan potong lintang. Pengambilan data dilakukan selama September–Desember 2018 dengan mewawancarai 65 pasien yang dipilih secara simple random sampling dari 300 pasien talasemia yang terdaftar di Perhimpunan Orangtua Penderita Thalassemia Indonesia (POPTI) Kota Bandung. Instrumen yang digunakan adalah Global Physical Activity Questionnaire (GPAQ) untuk mengukur aktivitas fisik dan Beck Depression Inventory (BDI) untuk menilai gejala depresi. Data dianalisis dengan uji chi-square menggunakan SPSS for Windows ver. 23.0. Hasil penelitian menunjukkan mayoritas penderita talasemia beta mayor di Kota Bandung mengalami depresi (52%) dan memiliki tingkat aktivitas fisik rendah (65%). Selanjutnya, terdapat hubungan bermakna secara statistik antara depresi dan aktivitas fisik pada penderita talasemia di Kota Bandung (p=0,04; p<0,05). Oleh karena itu, dapat disimpulkan bahwa penderita talasemia beta mayor di Kota Bandung yang mengalami depresi memiliki aktivitas fisik yang lebih rendah.

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