The mystery of persistent pulmonary hypertension: an idiopathic infantile arterial calcification.
BMC Pediatr
; 13: 107, 2013 Jul 16.
Article
em En
| MEDLINE
| ID: mdl-23855924
ABSTRACT
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 one hour of age, he was noted to be pale, hypoperfused, with weak pulses, a hyperdynamic precordium and a grade IV/VI pansystolic murmur. The rest of his examination was normal. A chest X-ray showed massive cardiomegaly and pulmonary oedema. An echocardiogram (ECHO) indicated moderate persistent pulmonary hypertension (PPHN) of unclear etiology. A diagnosis of Idiopathic infantile arterial calcification was made and a trial of Editronate therapy was given without success.CONCLUSION:
IIAC is a rare disorder, it should be considered whenever a neonate presents with unexplainable cardiac failure, PPHN, echogenic vessels on X-ray/ultrasound and, or concentric hypertrophic ventricles on ECHO. Serial antenatal ultrasound findings of echogenic cardiac foci should raise the suspicion of IIAC. Further studies to determine the long term effects of Editronate on vascular calcifications, disease outcome, and other treatment options are needed.
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Calcificação Vascular
/
Hipertensão Pulmonar
Tipo de estudo:
Diagnostic_studies
Limite:
Humans
/
Male
/
Newborn
Idioma:
En
Ano de publicação:
2013
Tipo de documento:
Article