ABSTRACT
The objective of this study was to establish the potential utility of N-terminal pro-brain natriuretic peptide (NT-proBNP) in the management of patent ductus arteriosus (PDA). This was a monocentric prospective blind study that was conducted in a referral neonatal intensive care unit. The patients were very low-birth-weight/gestational-age neonates. Babies with cardiac congenital anomaly other than PDA, life-threatening congenital malformation, severe asphyxia at birth, persistent pulmonary hypertension, and death within the first week of life were excluded. Plasma NT-proBNP concentrations were determined on days 2, 4, and 7 of life. Echocardiography was performed on days 4 and 7. Results were blinded to clinicians. Only echographic results were available upon request. Thirty-one infants were included. NT-proBNP levels were significantly correlated to ductal size and to left atrial-to-aortic diameter ratio. The median NT-proBNP on both days 2 and 4 was significantly higher in neonates with later treated or persistent PDA. A level above 10.000 pg/mL at 48 h of age yielded a 100% positive and a 87% negative predictive value to exclude spontaneous ductal closure. However, no NT-proBNP threshold could predict which PDA would be judged necessary to treat. It was concluded that early low NT-proBNP values can be used as a reliable independent marker to predict spontaneous ductal closure in preterm neonates. Yet, high NT-proBNP levels should not be used to guide the decision to treat PDA, the risk being of treating many bystanding PDAs.
Subject(s)
Ductus Arteriosus, Patent/blood , Early Diagnosis , Natriuretic Peptide, Brain/blood , Peptide Fragments/blood , Premature Birth/blood , Body Weight , Ductus Arteriosus, Patent/diagnostic imaging , Ductus Arteriosus, Patent/physiopathology , Echocardiography , Follow-Up Studies , Humans , Infant, Newborn , Predictive Value of Tests , Prognosis , Prospective Studies , Protein Precursors , ROC Curve , Remission, Spontaneous , Severity of Illness Index , Time FactorsABSTRACT
UNLABELLED: A term infant born to consanguineous parents presented at birth with hypoglycaemia, thrombocytopenia, coagulopathy and hyperbilirubinaemia associated with polycythaemia due to delayed cord clamping. Despite phototherapy and correction of polycythaemia by partial exchange transfusion, coagulopathy, hypoglycaemia and conjugated hyperbilirubinaemia persisted, suggesting hepatic failure. Metabolic work-up led to the diagnosis of tyrosinaemia type 1 on day 4. Two--(2-nitro-4-trifluoromethylbenzoyl)--1,3 cyclohexanedione (NTBC) treatment, started on day 5, resulted in progressive clinical improvement and unambiguous biochemical response. Severe skin purpuric lesions occurred in areas exposed to phototherapy. These resolved slowly after its discontinuation. Urine analysis sampled just before and 6 days after starting NTBC treatment showed high levels of type 1 coproporphyrin isomers. Such findings do not seem directly related to tyrosinaemia type 1 where succinylacetone inhibits delta-aminolevulinic acid (delta-ALA) dehydratase and where the accumulation of delta-ALA results in neurotoxicity without photosensitivity. CONCLUSION: We describe a cutaneous form of porphyria in a neonate presenting with severe liver failure due to tyrosinaemia type 1. This porphyria is tentatively attributed to a secondary accumulation of coproporphyrins due to cholestasis, as reported in the bronze baby syndrome and recently described in neonates with purpuric phototherapy-induced eruption, rather than to a primary defect of porphyrin metabolism. The hypothesis of a direct effect of tyrosinaemia type 1 on porphyrin excretion is also discussed.
