Working…
ClinicalTrials.gov
ClinicalTrials.gov Menu

Sexual Maturation in β-Thalassemia Major Patients in Assiut University Hospital

The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Read our disclaimer for details.
 
ClinicalTrials.gov Identifier: NCT03847558
Recruitment Status : Unknown
Verified February 2019 by ayman ekram, Assiut University.
Recruitment status was:  Not yet recruiting
First Posted : February 20, 2019
Last Update Posted : February 20, 2019
Sponsor:
Information provided by (Responsible Party):
ayman ekram, Assiut University

Tracking Information
First Submitted Date December 4, 2018
First Posted Date February 20, 2019
Last Update Posted Date February 20, 2019
Estimated Study Start Date April 15, 2019
Estimated Primary Completion Date April 15, 2020   (Final data collection date for primary outcome measure)
Current Primary Outcome Measures
 (submitted: February 19, 2019)
  • sexual maturation in patients receiving regular chelation will be compared to patients not receiving chelation helps us to improve quality of life for these patients and have normal sexual life [ Time Frame: 1 year ]
    The present study will be carried out on previously diagnosed transfusion dependent thalassemia patients under chelation therapy in Assiut University children hospital.
    1. patients will be selected and examined clinically to asses thier sexual maturation by measuring presence of secondary sexual characters.
    2. hormonal assesment for patients including FSH,LH,Serum Testosterone.
  • calculate body mass index (kg/m^2) [ Time Frame: 1 year ]
    assesment of hieght in meters,weight in kilograms.
Original Primary Outcome Measures Same as current
Change History No Changes Posted
Current Secondary Outcome Measures Not Provided
Original Secondary Outcome Measures Not Provided
Current Other Pre-specified Outcome Measures Not Provided
Original Other Pre-specified Outcome Measures Not Provided
 
Descriptive Information
Brief Title Sexual Maturation in β-Thalassemia Major Patients in Assiut University Hospital
Official Title Assessment of Sexual Maturation in β-Thalassemia Major Patients Receiving Iron Chelation Therapy in Assiut University Hospital
Brief Summary cross sectional study to asses sexual maturation in β-Thalassemia Major patients receiving Iron Chelation Therapy in assuit University Hospital and maintenance of 2ry sexual characters and reproduction
Detailed Description

Thalassemia refers to a group of inherited diseases characterized by decreased or absent synthesis of normal globin chains . The direct consequence is an imbalance of the alpha and beta globin chain synthesis that results in anemia from ineffective erythropoiesis and hemolysis.

The term thalassemia major refers to the severe form that is often associated with life-long transfusion dependent anemia.

Hypogonadism is the most frequently reported endocrine complication, affecting 70-80% of thalassemia major patients. Hypogonadism is likely to be caused by iron deposits in the gonads, pituitary gland or both.

Hypogonadotropic hypogonadism resulting from iron deposition in the pituitary gonadotrope is more commonly found than gonadal iron deposition in ovaries or testes occurs.

Iron deposition in the anterior pituitary gland can be demonstrated beginning in the first decade of life, but clinical manifestations are usually not evident until the onset of puberty. At the earlier stage, only a diminished gonadotropin reserve with intact gonadotropin pulse was observed .

The direct effect of iron, in particular that of NTBI(non-transferrin-bound iron) on the ovaries and testes is currently unknown. The ovarian reserve is preserved in the majority of female thalassemia patients, even in women with amenorrhea. In males, histological examination of testicular tissues from autopsies demonstrated testicular interstitial fibrosis with small, heavily pigmented, undifferentiated seminiferous tubules and an absence of Leydig cells .

There are three main clinical presentations of the HPG(hypothalamic pituitary gonadal axis)axis derangement in thalassemia major, including delayed puberty, arrested puberty and hypogonadism. Delayed puberty is defined as the absence of any pubertal signs by 14 years in boys and 13 years in girls . Arrested puberty is defined as the absence of further pubertal progression for more than 1 year after puberty has started.

Chelation therapy with desferoxamine before the age of puberty has helped patients to attain normal sexual maturation in some studies but not in others. In a study of 40 patients with transfusion-dependent thalassemia major, 90% of 19 patients who began treatment with desferoxamine before the age of 10 had normal sexual development compared with only 38% of those treated after the age of 10. In contrast, another study reported no difference in the frequency of pubertal maturation when iron chelation therapy was started at the age of 10 or earlier. Serum ferritin levels were still higher than normal in previous studies.

After a period of 5-7 years, 50% of hypogonadal males achieved normal testosterone levels and 32% of amenorrheic women became pregnant, either spontaneously or using in vitro fertilization . With modern medications, iron-induced hypogonadism may be reversible with intensive iron chelation regimens.

Study Type Observational
Study Design Observational Model: Case-Control
Time Perspective: Cross-Sectional
Target Follow-Up Duration Not Provided
Biospecimen Not Provided
Sampling Method Non-Probability Sample
Study Population β-thalassemic Patients aged 10 years and older patients receiving regular blood transfusions in Assiut Univ Hospitals and have chelation therapy.
Condition β-Thalassemia Major Patients Receiving Chelation Therapy
Intervention Diagnostic Test: FSH((Follicle Stimulating Hormone) and LH (Luteinizing Hormone) hormone for females and Testosterone hormone for males
assess of sexual maturation by clinical examination and hormonal studies (FSH.LH,Testosterone)
Study Groups/Cohorts sexual maturation in patient receiving iron chelation
assess of sexual maturation by clinical examination and hormonal studies (FSH.LH,Testosterone)
Intervention: Diagnostic Test: FSH((Follicle Stimulating Hormone) and LH (Luteinizing Hormone) hormone for females and Testosterone hormone for males
Publications * Not Provided

*   Includes publications given by the data provider as well as publications identified by ClinicalTrials.gov Identifier (NCT Number) in Medline.
 
Recruitment Information
Recruitment Status Unknown status
Estimated Enrollment
 (submitted: February 19, 2019)
100
Original Estimated Enrollment Same as current
Estimated Study Completion Date April 15, 2020
Estimated Primary Completion Date April 15, 2020   (Final data collection date for primary outcome measure)
Eligibility Criteria

Inclusion Criteria:

  • β-thalassemic Patients aged 10 years and older
  • β-thalassemic patients receiving regular blood transfusions
  • All patients are on chelation therapy

Exclusion Criteria:

  • β-thalassemic patients with major system complications as cardiac ,renal or hepatic.
  • Patients not receiving chelation therapy
  • Patients with other hemolytic anemia
Sex/Gender
Sexes Eligible for Study: All
Ages 10 Years to 16 Years   (Child)
Accepts Healthy Volunteers Yes
Contacts Contact information is only displayed when the study is recruiting subjects
Listed Location Countries Not Provided
Removed Location Countries  
 
Administrative Information
NCT Number NCT03847558
Other Study ID Numbers Assiut Univ Children Hospitals
Has Data Monitoring Committee Yes
U.S. FDA-regulated Product
Studies a U.S. FDA-regulated Drug Product: No
Studies a U.S. FDA-regulated Device Product: No
IPD Sharing Statement
Plan to Share IPD: Undecided
Plan Description: i hope i can publish my study to help other researchers to get accurate assesment and improve reproduction of these patients
Responsible Party ayman ekram, Assiut University
Study Sponsor Assiut University
Collaborators Not Provided
Investigators Not Provided
PRS Account Assiut University
Verification Date February 2019