The mystery of persistent pulmonary hypertension: an idiopathic infantile arterial calcification

The mystery of persistent pulmonary hypertension: an idiopathic infantile arterial calcification

BACKGROUND Idiopathic infantile arterial calcification (IIAC) is a rare autosomal recessive disorder, characterized by wide spread calcifications in arterial walls, leading to vaso-occlusive ischaemia of multiple organs. Mortality is high, and there is no definitive treatment. CASE PRESENTATION A male neonate, 36+5 weeks gestation, 2.81 kg, was admitted to NICU for respiratory distress. At on...

Idiopathic infantile arterial calcification: case report.

Idiopathic infantile arterial calcificationI (IIAC) is a rare disorder characterized by calcium deposition in the internal elastica lamina of medium and large arteries and it has been defined in term of molecular genetics. It is usually fatal, approximately 85% of all patients die within the first months of life owing to ischemia of vital organs. Death from myocardial infarction usually occurs ...

idiopathic pulmonary arterial hypertension

cytometry analysis of BAL cells. Table 1 integrates information given in our published paper, clearly demonstrating that the number of BAL neutrophils was fair in our case series. This unfortunately prevented a definitive evaluation of whether polymorph nucleates represent a source of IL-17. Nonetheless, as shown in figure 1, in selected cases with a significant number of BAL neutrophils (two s...

Idiopathic infantile arterial calcification with leukomalacia SWISS SOCIETY OF NEONATOLOGY

idiopathic pulmonary arterial hypertension sildenafil

cytometry analysis of BAL cells. Table 1 integrates information given in our published paper, clearly demonstrating that the number of BAL neutrophils was fair in our case series. This unfortunately prevented a definitive evaluation of whether polymorph nucleates represent a source of IL-17. Nonetheless, as shown in figure 1, in selected cases with a significant number of BAL neutrophils (two s...