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James A.T.,Rotunda Hospital | Bee C.,Rotunda Hospital | Corcoran J.D.,Rotunda Hospital | Mcnamara P.J.,Physiology and Experimental Medicine | And 3 more authors.
Journal of Perinatology | Year: 2015

Objective:Milrinone has been proposed as an effective treatment for pulmonary hypertension (PH) and right ventricular (RV) dysfunction. We aimed to determine the effect of milrinone therapy on clinical and echocardiography parameters of PH in preterm infants with elevated pulmonary pressures.Study Design:A retrospective case review was conducted on infants <32 weeks gestation who received milrinone for the treatment of PH and reduced RV function. Echocardiographic data were collected before and after treatment with milrinone, and serial clinical parameters were recorded over a 72hperiod.Result:Seven infants met the inclusion criteria with a median gestation and birth weight of 27.3 weeks and 1140 g, respectively. Four infants had a diagnosis of pulmonary hypoplasia with PH, and three infants were recipients in twin-to-twin transfusion syndrome who also developed PH. Nitric oxide was used in six infants before commencement of milrinone. Milrinone was commenced at a dose of 0.33 μg kg -1 min -1 to 0.5 μg kg -1 min -1 and continued for a median duration of 70 h. Use of milrinone was associated with a fall in oxygenation index and inhaled nitric oxide dose. Following an initial fall in blood pressure over the first 6 h, there was an increase in blood pressure over the subsequent 72 h. Echocardiographic data demonstrated an increase in indicators of myocardial performance and PH. One infant died before discharge.Conclusion:This case series suggests that milrinone may be a useful therapy for premature infants with echocardiography findings of PH and/or RH dysfunction. This data support the need for a randomised control trial to confirm its efficacy. © 2015 Nature America, Inc. Source

Rowland M.,Dublin Academic Medical Center | Bourke B.,Our Ladys Childrens Hospital Crumlin | Bourke B.,University College Dublin
Current Opinion in Pulmonary Medicine | Year: 2011

Purpose of Review: This review looks at what is currently known about liver disease in cystic fibrosis (CF) in light of the literature over the past year, and what the ongoing challenges are from a clinical and research perspective for the optimal management of cystic fibrosis liver disease (CFLD). Recent Findings: Patients with CF who develop clinically significant liver disease have a worse overall phenotype, and whereas there is no definite evidence that they have a shorter life expectancy, longer follow-up is required to determine if liver disease is a risk factor for mortality in CF.The development of the ferret and pig animal models of CF with multiorgan involvement is an important breakthrough which will enhance our understanding of the pathogenesis of CFLD, and with which it is hoped novel therapeutic targets for the treatment of CFLD will be identified. Summary: Whereas there is still no effective treatment for liver disease in CF, recent developments of animal models of CFLD will enhance our capacity to develop new therapeutic targets and reduce the impact of liver disease on mortality in CF. © 2011 Wolters Kluwer Health | Lippincott Williams & Wilkins. Source

El-Khuffash A.,Rotunda Hospital | El-Khuffash A.,Royal College of Surgeons in Ireland | James A.T.,Rotunda Hospital | Cleary A.,Rotunda Hospital | And 5 more authors.
Archives of Disease in Childhood: Fetal and Neonatal Edition | Year: 2015

Objective To investigate the effect of late treatment with intravenous paracetamol on patent ductus arteriosus (PDA) closure prior to possible PDA ligation. Methods A retrospective review of infants with a haemodynamically significant PDA, considered for PDA ligation and treated with intravenous paracetamol prior to possible ligation. Results Thirty six infants with a median gestation of 26.1 weeks received paracetamol at a median age of 27 days. Paracetamol was associated with immediate closure in nine (25%) infants. There was no response to paracetamol treatment in four (11%) infants who subsequently underwent a PDA ligation. In 23 (64%) infants, the PDA constricted and all but one of this group demonstrated complete PDA closure prior to discharge. Conclusions There may be a role for intravenous paracetamol in late closure of infants with a significant PDA to avoid ligation. The use of paracetamol for late treatment of PDA should be systematically evaluated. Source

James A.T.,The Rotunda Hospital | Corcoran J.D.,The Rotunda Hospital | Jain A.,Mount Sinai Hospital | Jain A.,University of Toronto | And 5 more authors.
Early Human Development | Year: 2014

Background: The transitional circulation and its effect on myocardial performance are poorly understood in preterm infants. Aims: We assessed myocardial performance in infants less than 29. weeks gestation in the first 48. h of life using a comprehensive echocardiographic assessment. Design: Infants < 29. weeks gestation were prospectively enrolled. Small for gestation, infants on inotropes and/or inhaled nitric oxide and septic infants were excluded. Conventional echocardiography, left ventricular (LV), septal and right ventricular (RV) tissue Doppler imaging (TDI) and tissue Doppler-derived strain and strain rate (SR), tricuspid annular plane systolic excursion (TAPSE) and global RV fractional area change (FAC) were assessed at a median of 10 and 45. h post-delivery. Results: Fifty-four infants with a median [IQR] gestation and birth weight of 26.5 weeks [25.8-28.0 weeks] and 915 g [758-1142 g] were included. There was no change in shortening or ejection fraction across the two time points. Systolic and diastolic TDI of the LV, septum and RV increased across the two time points (all p values ≤ 0.01). There was an increase in septal peak systolic and early diastolic SR (p = 0.002). Septal systolic strain and late diastolic SR did not change. With the exception of RV strain and early diastolic SR, all RV functional parameters including SR, late diastolic SR, TAPSE, and FAC increased across the two time points (all p values. <. 0.01). Conclusion: Describing the normal hemodynamic adaptations in stable preterm infants during the transitional period provides the necessary information for the assessment of those parameters in various disease states. © 2014 Elsevier Ltd. Source

Irvine A.D.,Our Ladys Childrens Hospital Crumlin | Irvine A.D.,National Childrens Research Center | Irvine A.D.,Trinity College Dublin
Journal of Investigative Dermatology | Year: 2014

In this issue of the Journal, Stout and colleagues report a novel and creative approach to replacement of genetically determined absence or deficiency of epidermal proteins. While these early data are certainly interesting, further validation work is required to determine the utility of this approach in genodermatoses. © 2014 The Society for Investigative Dermatology. Source

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