Primitive Immunodeficiency and Pregnancy

Study Overview
=================
Brief Summary
-----------------
The management of patients with primary immune deficiency is increasingly codified, however contraception and pregnancy have not yet extensively studied or codified, and the medical monitoring and the prevention of infectious complications thus remains at the discretion of the practitioner. The aim the research is to study the obstetric features and outcome of patients with primary immune defects.

Detailed Description
-----------------
Primary immune deficiencies constitute a large group of immune system disorders of genetic origin which can associate, to varying degrees, an increased susceptibility to infections and immunopathological manifestations: allergy, inflammation, autoimmunity, lymphoproliferation, tumors malignant. Although their prevalence remains underestimated, there has been an increase in the number of cases diagnosed in the past 10 years. The national average prevalence is 8.6 patients per 100,000 inhabitants and the diagnostic incidence is 400 new cases per year in France. Major improvement in the management of primary immunodeficiencies have drastically changed patients outcome. Most patients now reach adulthood and the possibility of carrying out a pregnancy project, that has already reported for patients suffering from hypogammaglobulinemia or variable common immune deficiency, is now increasingly reported for other types of inherited immunodeficiencies. Whereas the management of patients with hereditary immunodeficiencies is increasingly codified, contraception and pregnancy have not yet been the subject of recommendations: medical monitoring and the prevention of infectious complications thus remain at the discretion of the practitioner. The aim the research is to study the obstetric experiences of patients with a primary immune deficiency, paying particular attention to infectious complications.

Official Title
-----------------
Primitive Immunodeficiency and Pregnancy

Conditions
-----------------
Primary Immune Deficiency

Intervention / Treatment
-----------------
* Other: Questionnaire


Participation Criteria
=================
Eligibility Criteria
-----------------
Inclusion Criteria: Adult woman with Hereditary Immune Deficits and entered in the reference Center for Hereditary Immune Deficits (CEREDIH, hospital Necker-Enfants Malades, Paris) register Patient having reported at least one pregnancy or attempted pregnancy Patient not opposing to participation in research Exclusion Criteria: - Refusal to participate of the study

Ages Eligible for Study
-----------------
Minimum Age: 18 Years

Sexes Eligible for Study
-----------------
Female

Accepts Healthy Volunteers
-----------------
No

Study Plan
=================
How is the study designed?
-----------------

Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Patients<br>Patients entered in the register of the reference Center for Hereditary Immune Deficits (CEREDIH), hospital Necker-Enfants Malades, Paris, and having reported at least one pregnancy or attempted pregnancy | Other: Questionnaire<br>* Questionnaire intended for patients on the course of pregnancy and postpartum, the neonatal period and contraceptive modalities.<br>|

What is the study measuring?
-----------------
Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Infectious events | Number of infectious events at any time during pregnancy and post-partum | 18 months | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Infectious gynecological complications | Number of infectious gynecological complications during contraception | Up to 50 years | 
| Onset of pregnancy | Number of pregnancies during reproductive age period | Up to 50 years | 
| Pregnancy outcomes | Rate of early or late miscarriage, completed pregnancy, live birth and childbearing | Up to 9 months | 
| Treatments | Number of treatments aiming at preventing infections | 9 months | 
| Screening of infections | Presence/absence of screening for infections | 9 months | 
| Occurrence of obstetric complications | Rate of obstetric complications | 9 months | 
| Contraception effectiveness | Number of unwanted pregnancies | Up to 50 years | 
| Contraception complications | Number of non-infectious complications of contraception | Up to 50 years | 
| Deaths and complications in the neonatal period | Number of complications during neonatal period, including death | 3 months | 

 Terms related to the study
=================
Keywords Provided by Centre Hospitalier Valida
-----------------
Obstetric experiences, Primary immune deficiency, Infectious complications, Pregnancy, Postpartum, Contraception