Busy can be happy: 'Cake Thursday' as a team bonding exercise.

Implementation of 'Cake Thursday' as a team bonding and morale-boosting exercise.

GATA1-mutant clones are frequent and often unsuspected in babies with Down syndrome: identification of a population at risk of leukemia.

Transient abnormal myelopoiesis (TAM), a preleukemic disorder unique to neonates with Down syndrome (DS), may transform to childhood acute myeloid leukemia (ML-DS). Acquired GATA1 mutations are present in both TAM and ML-DS. Current definitions of TAM specify neither the percentage of blasts nor the role of GATA1 mutation analysis. To define TAM, we prospectively analyzed clinical findings, blood counts and smears, and GATA1 mutation status in 200 DS neonates. All DS neonates had multiple blood count and smear abnormalities. Surprisingly, 195 of 200 (97.5%) had circulating blasts. GATA1 mutations were detected by Sanger sequencing/denaturing high performance liquid chromatography (Ss/DHPLC) in 17 of 200 (8.5%), all with blasts >10%. Furthermore low-abundance GATA1 mutant clones were detected by targeted next-generation resequencing (NGS) in 18 of 88 (20.4%; sensitivity ∼0.3%) DS neonates without Ss/DHPLC-detectable GATA1 mutations. No clinical or hematologic features distinguished these 18 neonates. We suggest the term "silent TAM" for neonates with DS with GATA1 mutations detectable only by NGS. To identify all babies at risk of ML-DS, we suggest GATA1 mutation and blood count and smear analyses should be performed in DS neonates. Ss/DPHLC can be used for initial screening, but where GATA1 mutations are undetectable by Ss/DHPLC, NGS-based methods can identify neonates with small GATA1 mutant clones.

Outcomes of inhaled nitric oxide in preterm neonates - a five-year experience in a tertiary neonatal centre.

AIM: To review the outcome of preterm neonates treated with iNO. METHODS: The neonatal electronic database was reviewed between April 1, 2009 and March 31, 2014. RESULTS: In the five-year period, 142 babies were treated with iNO. Forty-two preterm neonates <34(+0)  weeks were included in this review. During this period, the use of iNO in preterm neonates <26(+0)  weeks rose from 1.4% to 4.8% and the mortality was significantly higher in this group (69%). CONCLUSION: There is an increasing trend in using iNO as a rescue treatment in preterm neonates. The outcomes were very poor in the preterm neonates <26(+0)  weeks. A cautious approach is warranted in the absence of robust evidence to support the use of iNO, in preterm neonates <26(+0)  weeks.

Successful emergency cardiac pacing and permanent pacemaker insertion in a preterm 29-week gestation hydropic baby with congenital complete heart block.

A preterm 29-week gestation baby was delivered because of gross foetal hydrops secondary to congenital complete heart block. Despite a poor prognosis, she survived stabilisation and received emergency epicardial pacing followed by permanent pacemaker insertion on day 13, weighing 1.2 kg.

Assessment of myocardial function in preterm infants with patent ductus arteriosus using tissue Doppler imaging.

OBJECTIVE: To assess myocardial function in preterm infants with different degrees of ductal patency in the first week of life using tissue Doppler imaging. STUDY DESIGN: Infants <30 weeks of gestation underwent echocardiography on day 3. A total of 72 infants were recruited into the study and categorised into three groups (i) haemodyamically significant ductus arteriosus, (ii) patent ductus arteriosus and (iii) no patent ductus arteriosus. Those with haemodynamically significant ductus arteriosus were treated with indometacin and echocardiography was repeated after 48-72 hours following treatment. Peak systolic and diastolic myocardial velocities were obtained using tissue Doppler imaging, and myocardial performance index was calculated. RESULTS: Initial myocardial velocities were significantly lower and myocardial performance index significantly higher in the haemodynamically significant ductus arteriosus group compared with other groups. For the haemodynamically significant ductus arteriosus group, post-treatment myocardial velocities were higher and myocardial performance index lower than pre-treatment. CONCLUSION: Preterm infants with haemodynamically significant ductus arteriosus had lower myocardial velocities and higher myocardial performance index, suggesting relative systolic and diastolic myocardial dysfunction. Babies whose patent ductus arteriosus remained open despite indometacin had lower pre-treatment myocardial velocities and higher myocardial performance index than those babies whose patent ductus arteriosus closed, suggesting worse myocardial function in this group. Measurement of myocardial function using tissue Doppler imaging in preterm infants is feasible and may prove to be helpful in the management of babies with patent ductus arteriosus.

Management of asymptomatic cardiac murmurs in term neonates.

Approximately 2 % of newborn infants are noted to have cardiac murmur on routine postnatal examination. Our aim was to look at current evidence and practice in the management of asymptomatic cardiac murmur in term neonates. We performed a systematic literature review and a telephone survey of all neonatal units in the United Kingdom (UK). The systematic review of the literature did not support the routine practice of four-limb blood pressure (BP), chest X-ray (CXR), and electrocardiogram (ECG) in the assessment of asymptomatic cardiac murmur in term neonates. The survey had participation from 132 (68 %) of 193 neonatal units in the UK. In an asymptomatic term neonate with cardiac murmur, 124 (94 %) units perform pulse oximetry, 100 units (76 %) measure four-limb BP, 36 units (27 %) perform a CXR, and 52 units (39 %) perform an ECG. Eight-six units (65 %) have availability of in-house echocardiography services provided mainly by paediatricians with cardiology interest in special care units and neonatologists in neonatal intensive care units. Currently there is wide variation in practice in the management of asymptomatic cardiac murmur in term neonates. There is no evidence to support the routine use of four-limb BP, CXR, and ECG in the assessment of asymptomatic cardiac murmur in term neonates. Based on the evidence available, both structured clinical examination (including determining presence and quality of bilateral femoral pulses) and universal use of pulse oximetry are most important in identifying CHD in asymptomatic term neonates with cardiac murmur before discharge home.

Left Ventricular Dysfunction Persists in the First Week after Re-Warming following Therapeutic Hypothermia for Hypoxic-Ischaemic Encephalopathy.

OBJECTIVES: The aim of this study was to assess serial myocardial function in newborn infants receiving therapeutic hypothermia (TH) as treatment for moderate to severe hypoxic-ischaemic encephalopathy (HIE). METHODS: Serial echocardiography was performed in 20 term infants receiving TH on days 1-3 and again after re-warming. Left ventricular (LV) fractional shortening, LV cardiac output, and tissue Doppler imaging-derived myocardial velocities and myocardial performance index were measured. Similar assessments were obtained from 20 well term infants within 48 h of birth. RESULTS: LV fractional shortening (LVFS) was similar between cases and controls during all measurements (25.3% vs. 27.4%). The mean LV cardiac output on day 1 was significantly lower in cases (109 mL/kg/min) than in controls (162 mL/kg/min) but increased after re-warming (145 mL/kg/min). All myocardial velocities were significantly lower in cases on day 1, increased during TH, but LV indices remained consistently lower compared to controls even after re-warming. LV myocardial performance index was higher in cases compared to controls on day 1, improved during TH but remained abnormal after re-warming. The right ventricular myocardial performance index was similar between cases and controls. CONCLUSION: Among infants affected by moderate to severe HIE, LV function appears to be more affected than right ventricular function with LV dysfunction persisting after completion of TH. LVFS was not useful to determine dysfunction in this cohort.

Assessment of myocardial function in preterm infants with chronic lung disease using tissue Doppler imaging.

OBJECTIVES: To assess myocardial function and presence of pulmonary hypertension (PH) using both tissue Doppler imaging (TDI) and conventional echocardiography in preterm infants of <32 weeks gestation with chronic lung disease (CLD). DESIGN: Prospective observational study. SETTING: Tertiary neonatal intensive care unit. PATIENTS: Three groups of preterm infants were recruited. Group 1-CLD receiving positive pressure airway support including high-flow humidified nasal cannula oxygen (n=25), group 2-CLD receiving low-flow nasal oxygen (n=25) and group 3-no CLD (n=22). METHODS: Echocardiography was performed around 36 weeks corrected gestational age. Myocardial function and PH were assessed using both conventional (left ventricular fractional shortening (LVFS) and left ventricular output (LVO), tricuspid regurgitation and ventricular septal flattening) and TDI techniques (myocardial velocities, myocardial performance index (MPI) and right ventricular isovolumetric relaxation time (RV-IVRT)). RESULTS: The MPI of right ventricle (RV) and left ventricle (LV) was significantly higher in CLD infants: mean RV MPI group 1-0.79, group 2-0.65 and group3-0.52. LV MPI: group 1-0.77, group 2-0.70 and group 3-0.45. There was a trend towards higher MPIs in group 1 compared with group 2. LVFS and LVO were similar across all three groups. RV-IVRT was also significantly higher in infants with CLD infants (group 1-64 milliseconds, group 2-62 milliseconds and group 3-52 milliseconds). PH was not detected by conventional echocardiography. CONCLUSIONS: Infants with CLD have evidence of relative biventricular dysfunction and higher pulmonary arterial blood pressure as demonstrated by TDI, which were not detected by conventional echocardiography.

Congenital lung lesions: Postnatal management and outcome.

Antenatal diagnosis of lung lesion has become more accurate resulting in dilemma and controversies of its antenatal and postnatal management. Majority of antenatally diagnosed congenital lung lesions are asymptomatic in the neonatal age group. Large lung lesions cause respiratory compromise and inevitably require urgent investigations and surgery. The congenital lung lesion presenting with hydrops requires careful postnatal management of lung hypoplasia and persistent pulmonary hypertension. Preoperative stabilization with gentle ventilation with permissive hypercapnia and delayed surgery similar to congenital diaphragmatic hernia management has been shown to result in good outcome. The diagnostic investigations and surgical management of the asymptomatic lung lesions remain controversial. Postnatal management and outcome of congenital cystic lung lesions are discussed.

The impact of routine predischarge pulse oximetry screening in a regional neonatal unit.

OBJECTIVES: (i) To evaluate the impact of routine early pulse oximetry screening on the rate of unexpected neonatal unit (NNU) admissions and the need for echocardiography. (ii) To review the outcomes of babies admitted as a result of a positive pulse oximetry screening test. DESIGN: Retrospective review over a 40-month period. SETTING: Level 3 NNU. PATIENTS: All babies admitted as a result of positive pulse oximetry screening. MAIN OUTCOME MEASURES: Indication for admission, clinical diagnosis and management were collated. Results 3552 babies were admitted during the study period. Of these, 1651 were unexpected admissions and 208/1651 (12.6%) were as a result of positive pulse oximetry screening. 165/208 babies (79%) had a significant clinical condition which required further intervention including 17 with congenital heart defect (CHD) (nine critical), 55 with pneumonia, 30 with sepsis and 12 with pulmonary hypertension. No baby died or collapsed on the postnatal ward during the study period. 61/208 babies (29%) had echocardiography and CHD was detected in 28%. Conclusions Routine use of pulse oximetry screening identifies babies with CHD and other illnesses, which, if not identified early could potentially lead to postnatal collapse. It does not appear to overload clinical services, resulting in appropriate admission in the majority and a modest increase in the number of echocardiograms performed.