A neonate with a spongy failing heart - What could it be?

A neonate born of third-degree consanguineous marriage presented on day 12 of life with congestive cardiac failure. A male sibling died at 3 months of age, cause of which was not known. He was treated with decongestive measures and multiple inotropes. 2D Echocardiogram revealed severe Left ventricular dysfunction with prominent trabeculations and deep recesses in the left ventricle suggestive of Left ventricular non-compaction. He was also found to have horse-shoe kidney. Considering the presence of cardiac left ventricular non compaction, horse-shoe kidney and family history of neonatal death and pregnancy loss clinical exome sequencing was done. It detected a homozygous missense variant in exon 6 of the AGK gene suggestive of Senger's syndrome. Baby was on regular follow-up and was thriving well on diuretics, sacubitril-valsartan and weekly levosimendan infusions. At 8 months of age, cardiac transplantation was successfully done and baby has been doing well post-transplantation. LVNC in children is rare with an estimated incidence of 0.11 per 100,000, the highest incidence being during infancy. Senger's syndrome is autosomal recessive in inheritance. Senger's syndrome associated with Left ventricular non compaction has been reported only once in literature so far. Renal manifestations in the form of horse shoe kidney like in our index baby has not been reported previously with Senger's syndrome.

Prognostic Value of Amplitude-Integrated Electroencephalography in Term Neonates With Encephalopathy.

OBJECTIVE: To evaluate the prognostic value of amplitude-integrated EEG in term neonates with encephalopathy. METHODS: In this prospective observational study we enrolled 58 term neonates with encephalopathy from March, 2019 to March, 2020. Level of alertness was ascertained as per Volpe's classification and tone as per Amiel-Tison scale of tone assessment. Abnormal aEEG was defined as background activity other than continuous normal voltage, or immature or absent sleep-wake cycle, or presence of electrical seizure. Primary outcome was abnormal neurological examination at discharge and/or death prior to discharge. RESULTS: Out of 58 neonates, aEEG was abnormal for 50 (86.2%). There was a statistically significant association between abnormal aEEG findings and primary outcome (P=0.04). The aEEG score cut-off of >2 had satisfactory sensitivity (88.8%) and specificity (79.5%) to predict primary outcome. CONCLUSIONS: Abnormal aEEG had good sensitivity but low specificity to predict the primary outcome in term neonates with encephalopathy.

Goat lung surfactant for treatment of respiratory distress syndrome among preterm neonates: a multi-site randomized non-inferiority trial.

OBJECTIVE: To investigate the safety and efficacy of goat lung surfactant extract (GLSE) compared with bovine surfactant extract (beractant; Survanta®, AbbVie, USA) for the treatment of neonatal respiratory distress syndrome (RDS). STUDY DESIGN: We conducted a double-blind, non-inferiority, randomized trial in seven Indian centers between June 22, 2016 and January 11, 2018. Preterm neonates of 26 to 32 weeks gestation with clinical diagnosis of RDS were randomized to receive either GLSE or beractant. Repeat dose, if required, was open-label beractant in both the groups. The primary outcome was a composite of death or bronchopulmonary dysplasia (BPD) at 36 weeks postmenstrual age (PMA). Interim analyses were done by an independent data and safety monitoring board (DSMB). RESULT: After the first interim analyses on 5% enrolment, the "need for repeat dose(s) of surfactant" was added as an additional primary outcome and enrolment restricted to intramural births at five of the seven participating centers. Following second interim analysis after 98 (10% of 900 planned) neonates were enroled, DSMB recommended closure of study in view of inferior efficacy of GLSE in comparison to beractant. There was no significant difference in the primary outcome of death or BPD between GLSE group (n = 52) and beractant group (n = 46) (50.0 vs. 39.1%; OR 1.5; 95% CI 0.7-3.5; p = 0.28). The need for repeat dose of surfactant was significantly higher in GLSE group (65.4 vs. 17.4%; OR 9.0; 95% CI 3.5-23.3; p < 0.001). CONCLUSIONS: Goat lung surfactant was less efficacious than beractant (Survanta®) for treatment of RDS in preterm infants. Reasons to ascertain inferior efficacy of goat lung surfactant requires investigation and possible mitigating strategies in order to develop a low-cost and effective surfactant.

Performance on Paladai Feeding of Preterm Infants with Bronchopulmonary Dysplasia.

OBJECTIVE: To evaluate the feeding performance of infants with bronchopulmonary dysplasia (BPD) on paladai. METHODS: This cross-sectional study was performed in a level III neonatal unit in North India from March through August 2012. Nineteen infants (27-32 wk of gestation) were enrolled; 9 in BPD group (oxygen requirement for at least 28 d) and 10 in 'No BPD' group. Paladai feeding (PF) sessions were video recorded for 3 d serially, at first successful (FSF) at postnatal age of ≥28 d and follow up feeding (FUF) at 40 ± 2 wk. Successful feeding was defined as ≥80% intake of volume prescribed. One hundred and four videos were analysed (58 in BPD group and 46 in 'No BPD' group). The outcome variables were: (1) postmenstrual age (PMA) at FSF (2) feeding performance, as assessed by proficiency (mL/min, volume of feed intake during only active feeding), efficiency (mL/min, volume of feed intake during total duration of feeding) and overall feed transfer (OT, % of prescribed feed volume taken), and (3) change in heart rate (∆HR) and oxygen saturation (∆SpO2) on PF. RESULTS: PMA (Weeks, 34.2 ± 2.0 vs. 33.6 ± 1.2, p = 0.13), performance on FSF [Median (range), Proficiency: ml/min, 4.2 (1.1, 21.7) vs. 3.4 (1.1, 12.4), efficiency: ml/min, 2.7 (0.4, 6.2) vs. 2.5 (0.9, 10.9)] and OT (%, mean ± SD: 84.9 ± 22.5 vs. 89.1 ± 9.6), and on FUF were comparable between the groups. Changes in SpO2 and HR were not significantly different. CONCLUSIONS: Infants with BPD perform comparably well on PF. PF can safely be attempted in them to facilitate transition to oral feeding.

Assisted Physical Exercise for Improving Bone Strength in Preterm Infants Less than 35 Weeks Gestation: A Randomized Controlled Trial.

OBJECTIVE: To compare the efficacy of daily assisted physical exercise (starting from one week of postnatal age) on bone strength at 40 weeks of post menstrual age to no intervention in infants born between 27 and 34 weeks of gestation. DESIGN: Open-label randomized controlled trial. SETTING: Tertiary-care teaching hospital in northern India from 16 May, 2013 to 21 November, 2013. PARTICIPANTS: 50 preterm neonates randomized to Exercise group (n=26) or Control group (n=24). INTERVENTION: Neonates in Exercise group underwent one session of physical exercise daily from one week of age, which included range-of-motion exercises with gentle compression, flexion and extension of all the extremities with movements at each joint done five times, for a total of 10-15 min. Infants in Control group underwent routine care and were not subjected to any massage or exercise. MAIN OUTCOME MEASURES: Primary: Bone speed of sound of left tibia measured by quantitative ultrasound at 40 weeks post menstrual age. Secondary: Anthropometry (weight length and head circumference) and biochemical parameters (calcium, phosphorus, alkaline phosphatase) at 40 weeks post menstrual age. RESULTS: The tibial bone speed of sound was comparable between the two groups [2858 (142) m/s vs. 2791 (122) m/s; mean difference 67.6 m/s; 95% CI - 11 to 146 m/s; P=0.38]. There was no difference in anthropometry or biochemical parameters. CONCLUSIONS: Daily assisted physical exercise does not affect the bone strength, anthropometry or biochemical parameters in preterm (27 to 34 weeks) infants.

Trial of daily vitamin D supplementation in preterm infants.

OBJECTIVE: To compare the effect of 800 vs 400 IU of daily oral vitamin D3 on the prevalence of vitamin D deficiency (VDD) at 40 weeks' postmenstrual age (PMA) in preterm infants of 28 to 34 weeks' gestation. METHODS: In this randomized double-blind trial, we allocated eligible infants to receive either 800 or 400 IU of vitamin D3 per day (n = 48 in both groups). Primary outcome was VDD (serum 25-hydroxyvitamin D levels <20 ng/mL) at 40 weeks' PMA. Secondary outcomes were VDD, bone mineral content, and bone mineral density at 3 months' corrected age (CA). RESULTS: Prevalence of VDD in the 800-IU group was significantly lower than in the 400-IU group at 40 weeks (38.1% vs. 66.7%; relative risk: 0.57; 95% confidence interval: 0.37-0.88) and at 3 months' CA (12.5% vs. 35%; relative risk: 0.36; 95% confidence interval: 0.14-0.90). One infant (2.4%) in the 800-IU group had vitamin D excess (100-150 ng/mL). Bone mineral content (mean ± SD: 79.6 ± 16.8 vs. 84.7 ± 20.7 g; P = .27) and bone mineral density (0.152 ± 0.019 vs. 0.158 ± 0.021 g/cm2; P = .26) were not different between the 2 groups. CONCLUSIONS: Daily supplementation with 800 IU of vitamin D reduces the prevalence of VDD at 40 weeks' PMA and at 3 months' CA in preterm infants without showing any improvement in bone mineralization. However, there is a possibility that this dose may occasionally result in vitamin D excess.