- The primary research objective is to estimate the prognosis of children affected by ICL. - A secondary research objective is to explore the heredity of ICL between first degree family members.
ID
Source
Brief title
Condition
- White blood cell disorders
- Immune system disorders congenital
Synonym
Research involving
Sponsors and support
Intervention
Outcome measures
Primary outcome
- CD4 cell counts
- Medical history defined by the questionnaire and medical files provided by
the general practitioner.
Secondary outcome
- Number of first degree family members affected by ICL.
Background summary
Idiopathic CD4 lymphocytopenia (ICL) is rare immune deficiency. The clinical
presentation of ICL can vary between life threatening opportunistic infections
to asymptomatic. To date, the aetiology, incidence and prognosis of ICL
patients remain largely unknown. Cases of ICL have been described in both
adults and children. Past studies to unravel aetiology, incidence and prognosis
have mainly been focussed on adult ICL patients, showing high morbidity and
mortality in affected patients. Several cases of hereditary ICL have been
identified, suggesting a genetic cause for ICL. This aim of this study is to
estimate the prognosis of children affected by ICL as well as exploring the
heredity of ICL between first degree family members.
Study objective
- The primary research objective is to estimate the prognosis of children
affected by ICL.
- A secondary research objective is to explore the heredity of ICL between
first degree family members.
Study design
Observational
Study burden and risks
HIV test associated risks:
The chance of a positive HIV test is low considering the population has no risk
factors. Also, early ICL diagnosis with specialized treatment in the WKZ may
improve prognosis.
ICL diagnosis associated risks:
The diagnosis of ICL may have psychological consequeses. To monitor these
consequences the researcher will ask the first three ICL patients and parents
to fill in a 'vierdimensionale klachtenlijst'. The results of these forms will
be report back to the METC.
POB 85090 Kamer E4.133.1
Utrecht 3508 AB
NL
POB 85090 Kamer E4.133.1
Utrecht 3508 AB
NL
Listed location countries
Age
Inclusion criteria
- Age between 0 to 16
- Lymphopenic two or more occasions, separated by at least a month (identified by pre-clinical screening, see research protocol)
- Good understanding of the Dutch language
Exclusion criteria
- Immunosuppressive co-morbidities explaining lymphopenia
- Immunosuppressive medication explaining lymphopenia
- Unavailability for follow-up research (immigration, mortality)
Design
Recruitment
Followed up by the following (possibly more current) registration
No registrations found.
Other (possibly less up-to-date) registrations in this register
No registrations found.
In other registers
| Register | ID |
|---|---|
| CCMO | NL42932.041.13 |