Echocardiographic Diagnosis and Hemodynamic Evaluation of Patent Ductus Arteriosus in Extremely Low Gestational Age Newborn (ELGAN) Infants.

Persistent Patent ductus arteriosus (PDA) is a common finding in extremely low gestational age newborn infants and its prevalence is inversely proportional to the gestational age. The presence of a persistent PDA is associated with increased mortality and several significant morbidities including intraventricular hemorrhage, pulmonary hemorrhage, necrotizing enterocolitis, and chronic lung disease or bronchopulmonary dysplasia. However, treating PDA has not been demonstrated to have beneficial impact on the long term outcomes. Currently there is no consensus on whether to treat the PDA or not, and if treat, when to treat and how to treat. The echocardiography is the investigation of choice to diagnose PDA, estimating the magnitude of shunt volume and assessing its hemodynamic significance, and to exclude/diagnose any associated congenital heart defect before any intervention. Various echocardiographic parameters and staging/scoring systems have been described to help the clincians making the clinical decisions and some of theses scoring systems are quite complex to apply in a busy day to day clinical practice. This concised review paper is focused to help the clinicians in making a clinical decision based upon clincial and echocardiography parameters. Hence, only the parameters which are commonly used and helpful in making the clinical decisions in day to day clincial practice have been described in this paper

A Successful Whole Body Therapeutic Hypothermia for Hypoxic Ischemic Encephalopathy During an ECMO Run in a Newborn

Data regarding the safety of using therapeutic hypothermia (TH) with extracorporeal membrane oxygenation (ECMO) in neonates with both hypoxic ischemic encephalopathy (HIE), and respiratory failure are lacking. TH is not associated with an increased incidence of hemostatic complications, but hypothermia may impair coagulation. Herein, we report a case of a newborn who had meconium aspiration syndrome and HIE and underwent both TH and ECMO. He did not have any bleeding or circuit complications, and mortality as short-term outcome along with well-neurodevelopmental outcome

Comparative effectiveness of drugs used to constrict the patent ductus arteriosus: a secondary analysis of the PDA-TOLERATE trial (NCT01958320).

ObjectiveTo evaluate the effectiveness of drugs used to constrict patent ductus arteriosus (PDA) in newborns < 28 weeks.MethodsWe performed a secondary analysis of the multi-center PDA-TOLERATE trial (NCT01958320). Infants with moderate-to-large PDAs were randomized 1:1 at 8.1 ± 2.1 days to either Drug treatment (n = 104) or Conservative management (n = 98). Drug treatments were assigned by center rather than within center (acetaminophen: 5 centers, 27 infants; ibuprofen: 7 centers, 38 infants; indomethacin: 7 centers, 39 infants).ResultsIndomethacin produced the greatest constriction (compared with spontaneous constriction during Conservative management): RR (95% CI) = 3.21 (2.05-5.01)), followed by ibuprofen = 2.03 (1.05-3.91), and acetaminophen = 1.33 (0.55-3.24). The initial rate of acetaminophen-induced constriction was 27%. Infants with persistent moderate-to-large PDA after acetaminophen were treated with indomethacin. The final rate of constriction after acetaminophen ± indomethacin was 60% (similar to the rate in infants receiving indomethacin-alone (62%)).ConclusionIndomethacin was more effective than acetaminophen in producing ductus constriction

Congenital analbuminemia caused by a novel aberrant splicing in the albumin gene

Introduction:Congenital analbuminemia is a rare autosomal recessive disorder manifested by the presence of a very low amount of circulating serum albumin. It is an allelic heterogeneous defect, caused by variety of mutations within the albumin gene in homozygous or compound heterozygous state. Herein we report the clinical and molecular characterization of a new case of congenital analbuminemia diagnosed in a female new-born of consanguineous (first degree cousins) parents from Ankara, Turkey, who presented with a low albumin concentration (< 8 g/L) and severe clinical symptoms. Materials and methods: The albumin gene of the index case was screened by single-strand conformation polymorphism, heteroduplex analysis, and direct DNA sequencing. The effect of the splicing mutation was evaluated by examining the cDNA obtained by reverse transcriptase - polymerase chain reaction (RT-PCR) from the albumin mRNA extracted from proband’s leukocytes. Results: DNA sequencing revealed that the proband is homozygous, and both parents are heterozygous, for a novel G>A transition at position c.1652+1, the first base of intron 12, which inactivates the strongly conserved GT dinucleotide at the 5’ splice site consensus sequence of this intron. The splicing defect results in the complete skipping of the preceding exon (exon 12) and in a frame-shift within exon 13 with a premature stop codon after the translation of three mutant amino acid residues. Conclusions: Our results confirm the clinical diagnosis of congenital analbuminemia in the proband and the inheritance of the trait and contribute to shed light on the molecular genetics of analbuminemia

Efficacy and Safety of Nebulized Recombinant Human DNase as Rescue Treatment for Persistent Atelectasis in Newborns: Case-series

Aim: To evaluate the efficacy and safety of using recombinant human DNase (rhDNase) in diminishing persistent atelectasis unresponsive to conventional treatment and mucus plugging in newborns with insufficient ability to clear thick and purulent airway secretions. Methods: Twelve newborns (10 preterms), who did not respond to conventional methods, received rhDNase nebulized therapy at a dose of 1.25 mg over a 15-minute period, twice a day (2 hours between the doses) for up to 3 days. The application of the drug was continued for up to 3 days or until the improvement of atelectasis. After a-three-day therapy, if atelectasis did not improve, a single dose (1.25 mg) of the same drug in liquid form was administered endotracheally. Clinical (respiration rate, requirement for oxygen concentration) and radiological response (chest x-ray scoring), duration of the treatment, recurrence of atelectasis and requirement for additional therapy were evaluated. Results: Ten out of 12 patients showed rapid clinical and radiological improvement after nebulized treatment. Two patients who did not respond to the three-day regimen received a single dose of the drug endotracheally and both recovered completely. Six patients did not require completion of three day regimen for radiological recovery. Chest x-ray scores and respiratory parameters showed significant improvement after the treatment. The respective median (range) values before and after treatment were 4 (1-5) and 0 (0-4) points for chest x-ray scores, 66 (60-78) and 49 (44-64) breaths/min for respiratory rates, and 45% (35-64) and 30% (21-40) for oxygen requirement. Comparison of pCO2 before (median, 56 mm Hg; range, 46-64) and after treatment (median, 41 mm Hg; range 38-58) in 7 patients showed significant improvement. Conclusion: In a large series of newborns to receive rhDNase and we demonstrated the usefulness of rhDNase as a mucolytic agent in treating newborns with persistent atelectasis who do not respond to other treatments

Nicolau syndrome induced by intramuscular vitamin K in a premature newborn

Nicolau syndrome (NS), also known as livedo-like dermatitis or embolia cutis medicamentosa, is a very rare complication of intramuscular and intraarticular injection of various drugs