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The Dallas Hereditary Spherocytosis Cohort Study

This study has been terminated.
(Insufficient staff to continue pursuing project)
Information provided by (Responsible Party):
University of Texas Southwestern Medical Center Identifier:
First received: June 4, 2010
Last updated: May 13, 2013
Last verified: May 2013

The purpose of this study is to

  1. better characterize the short term and long term natural history of hereditary spherocytosis (HS) including diagnosis, complications, and indications for and response to splenectomy
  2. evaluate and describe the health-related quality of life in children with HS.

Hereditary Spherocytosis

Study Type: Observational
Study Design: Observational Model: Cohort
Time Perspective: Prospective
Official Title: The Dallas Hereditary Spherocytosis Cohort Study

Resource links provided by NLM:

Further study details as provided by University of Texas Southwestern Medical Center:

Primary Outcome Measures:
  • Health related quality of life [ Time Frame: Approximately at 5 years ]
    PedsQL measurement

Secondary Outcome Measures:
  • Primary indications for splenectomy [ Time Frame: Approximately at 5 years ]
    Primary indication for splenectomy determined at time of splenectomy, if performed.

  • Complications of HS [ Time Frame: Approximately at 5 years and at 10 years ]
  • Complications of splenectomy [ Time Frame: Approximately at 5 years ]
  • Diagnosis of HS [ Time Frame: At enrollment ]

Biospecimen Retention:   Samples Without DNA

Enrollment: 55
Study Start Date: May 2010
Study Completion Date: May 2013
Primary Completion Date: May 2013 (Final data collection date for primary outcome measure)
Detailed Description:

Patients with a new or established diagnosis of HS seen at Children's Medical Center will be asked to enroll in the study. Previous and current medical records will be reviewed to systematically catalogue their history of HS, including diagnosis, complications, hospitalizations, medications and laboratory data. Health-related quality of life questionnaires will be given to the patients and their parents at enrollment and periodically during the follow-up. Those who agree will have up to three small samples of blood collected and frozen for future laboratory studies of complications associated with HS and/or splenectomy.

We anticipate enrolling approximately 200 children and young adults with HS in this study and following them until adulthood (age 18-21 years).


Ages Eligible for Study:   up to 21 Years   (Child, Adult)
Sexes Eligible for Study:   All
Accepts Healthy Volunteers:   No
Sampling Method:   Non-Probability Sample
Study Population
Center for Cancer and Blood Disorders outpatient clinics

Inclusion Criteria:

  • Diagnosis of HS with or without prior splenectomy
  • Age 0 - 21 years
  • Spanish-speaking subjects are eligible to participate

Exclusion Criteria:

  • Unable to provide contact information for follow-up
  Contacts and Locations
Choosing to participate in a study is an important personal decision. Talk with your doctor and family members or friends about deciding to join a study. To learn more about this study, you or your doctor may contact the study research staff using the Contacts provided below. For general information, see Learn About Clinical Studies.

No Contacts or Locations Provided
  More Information

Responsible Party: University of Texas Southwestern Medical Center Identifier: NCT01141621     History of Changes
Other Study ID Numbers: IRB # 022010-024
Study First Received: June 4, 2010
Last Updated: May 13, 2013

Keywords provided by University of Texas Southwestern Medical Center:
congenital hemolytic anemia
quality of life

Additional relevant MeSH terms:
Spherocytosis, Hereditary
Anemia, Hemolytic, Congenital
Anemia, Hemolytic
Hematologic Diseases
Genetic Diseases, Inborn processed this record on September 19, 2017