splenectomy ORPHA:822 Hereditary spherocytosis D58.0
Conditions
Interventions
Group 1: 1. identification of the operated spherocytosis patients in the patient data management system (PDMS).
2. creation of an individual questionnaire as well as selection of the quality of life q
Sponsors
Universitätsklinikum Freiburg, Kinder und Jugendklinik
Eligibility
Sex/Gender
All
Inclusion criteria
Inclusion criteria: Patients treated for spherocytosis and operated on the spleen in the Department of Paediatric Surgery and the Clinic for Paediatric Haematology & Oncology of any current age in the last 20 years (since 2000).
Exclusion criteria
Exclusion criteria: Absence of informed consent
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Subtotal splenectomy leads to a complete reduction in the number of haemolytic crises and days missed from school and work. | — |
Secondary
| Measure | Time frame |
|---|---|
| 2a The disease-related quality of life of spherocytosis patients after subtotal splenectomy compares well with the published quality of life of patients with other haemolytic anaemias. 2b No severe infections and no thromboses occur after subtotal splenectomy. 2c No relevant hypertrophy of the residual spleen occurs postoperatively. 2d Measurable haemolytic activity is still detectable after subtotal splenectomy at 3, 6, 12 and 24 months in the form of increased reticulocyte levels, increased LDH and decreased haptoglobin. 2e No other operative or postoperative complications occur. 2f No follow-up surgery including re-splenectomy is required. 2g Antibiotic prophylaxis is taken regularly and booster vaccinations are given regularly. | — |
Countries
Germany
Contacts
Public ContactAlexander Puzik
Universitätsklinikum Freiburg, Kinder- und Jugendklinik
Outcome results
None listed