[Leukocyte adhesion deficiency about anew Tunisian case]

Leukocyte adhesion deficiency [LAD] is a rare primary immunedeficiency inherited as an autosomal recessive genetic disorder. LAD was suspected in a four days old girl. She was born from healthy first cousins. A family history of a boy who died from omphalitis and sepsis was reported. Our patient had the severe form, she had delayed umbilical cord separation and suffered recurrent infections. She had a deletion of the G at position 1497. The patient received a bone marrow transplantation from her mother HLA identical at age of 14 months. She is now 9 years old and in a good health

[Pulmonary alveolar proteinosis in two siblings. A case report]

Pulmonary alveolar proteinosis [PAP] is a rare disorder in children. This report describes two siblings in whom PAP developed during infancy [three years for the boy and four years two months for the girl]. The girl was admitted for chronic respiratory distress. Chest x-ray showed a reticulonodular pattern. Her brother was asymptomatic. The diagnosis of PAP was confirmed by open lung biopsy for the boy and broncho-alveolar lavage for the girl. Therapeutic broncho-alveolar lavages were performed [six for the girl and two for the boy], the girl lost dependence on oxygen therapy. 6 years later, the brother is still asymptomatic. The sister had two episodes of respiratory distress, after two and four years, that required therapeutic lavages. The last therapeutic broncho-alveolar lavage was performed for the first time by a Tunisian team