The syndrome of Barth is a grave genetic disease bound in him X which gets essentially boys. She shows itself by one or some of the following symptoms, in different degrees:
The neutropénie :
It is a weakness of the immune system, more particularly a decrease of the number of neutrophiles white blood cells that are essential to fight the bacterial infections.Because of this neutropénie, the boys affected by the syndrome of Barth run a higher risk of catching grave infections such as bacterial pneumonias and cutaneous infections.
It is a weakness of the cardiac muscle which, bound to a weakened immune system, constitutes most great danger for the boys who suffer from the syndrome of Barth.
Muscular weakness and General Fatigue
All themuscles, including the heart, present a cellular deficiency which limits their capacity to produce some energy. This lack of energy causes, in his turn, an extreme fatigue during the activities of the simplest in the most complicated as, for example, to walk, to write or to grow, but who ask for the strength, for the vigour and for the stamina.
Defect of Development and Delay of Growth
Most ofthe boys suffering from the syndrome of Barth are also below the average in weight and in size, some even in a considerable way. Most of the time, we suppose that the low growth of the boys is due to a nourishing deficiency or that it would be one of side effects of a chronic disease, a situation described by the expression " defect of development ". A recent study led thanks to the patients'international list belonging to the Foundation of the Syndrome of Barth ( the BSF) revealed that the young boys undergo a growth accelerated during the last years by the adolescence, what allows them then to affect a normal size.
It is about a higher rate of an organic acid measured in the urine. However, there were cases where the rate of acid in the urine of certainindividuals suffering from the syndrome was completely normal.
Deficiency it cardiolipine
The metabolism does not produce sufficiencies of tetralinoleoyl-cardiolipin , a lipid which is essential in the structure and in the energy of mitochondria.
The transmission of the syndrome of Barth
It is a disease bound to the X chromosome, what what means that it is passed on from mother to son. Asurrogate mother of the syndrome of Barth presents herself anybody signs or symptom of the disease. The probability that a boy arisen from a conductive mother has the syndrome of Barth is 50 %. Also, the probability than a girl arisen from a conductive mother is it itself is 50 %. All the girls arisen from a father affected by the syndrome of Barth will be conductive whereas none of his sons will begot.
RonaldMcDonald House Charities
Create in 1994 under the aegis of the foundation of France by McDonald's restaurant, the Ronald McDonald foundation acts for the welfare of children and their family. His priority action is the realization of parent's house Ronald McDonald. These houses allow the children hospitalized to be surrounded with their close relations. The children so keep anemotional balance determining for the quality of their cure.
Today 285 houses in the world among which 9 in France, which welcome more than 10 000 families.
For 10 € a day, families have a bed-room, a common kitchen and places of relaxation. Every country where OF MCDONALD supports the development of Parents' Houses, decides on its own structure. In France, it is a corporate foundation in which thefranchisees are the founders. The whole community of the franchisees finances and manages the Foundation.
Houses are managed themselves locally by autonomous associations in which the franchisees are actively involved.
The Office of the United Nations high commissioner for refugees was created on December 14th, 1950 by the United Nations General Assembly. The agency has for mandate...