jayashree purkayastha

Data on congenital heart diseases in neonates, especially critical congenital heart disease, varies worldwide. Developing countries have higher mortality than developed countries. To study the clinical profile of congenital heart diseases in neonates. Prospective observational study. Neonates admitted to a tertiary care hospital with congenital heart disease. Clinical details, investigations, and management of neonates admitted to a tertiary care hospital were studied prospectively. Among the study neonates, 60% had acyanotic heart disease, the most common being ASD, while 40% had cyanotic heart disease, the most common being TGA—outborn cases comprised 60% of the total neonates. The most common presenting features were fast breathing and cyanosis; the most common clinical sign was murmur(59%). Among the study neonates, 15 cases had low calcium levels. Definitive surgery was done in 21 neonates, of which 7 succumbed. The staged repair was done in 9 neonates, of which 2 succumbed. Of 12 TGA cases,10 underwent definitive repair, and 7 did well at follow-up. Among the 10 TOF babies, 6 underwent staged repair. Out of the 7 cases of CoA, 6 underwent definitive surgery, and 1 expired. Among Critical CHD, mortality was 23.3%. The most common cyanotic heart disease was ASD, and the most common cyanotic heart disease was TGA. Surgery was done in 30 cases, out of which 9 succumbed, and mortality among surgical cases was 30%. At follow up TGA  cases with definitive surgeries were doing well. Mortality was lower compared to other studies in India. Hypocalcemia was observed in 16.6% of the CHD cases, which needs to be studied in the future.

Rubinstein-Taybi syndrome is a rare disorder characterized by broad thumbs and great toes, short stature, dysmorphic facial features, eye abnormalities, cryptorchidism, and moderate to severe intellectual disability. Renal, cardiac, and dental anomalies as well as obesity may be associated with the syndrome. The patients may also have behavioral problems. They have a weak laryngeal wall that can easily collapse resulting in swallowing and breathing problems.

We report a case of an infant with Rubinstein-Taybi syndrome. The present case had typical facial features, broad thumbs and great toes, right corneal opacity, and bilateral vocal cord abductor paralysis. To the best of our knowledge, no study has been conducted on bilateral vocal cord abductor paralysis in Rubinstein Taybi syndrome. In addition, the infant recovered with symptomatic treatment without tracheostomy.

Rubinstein-Taybi syndrome is associated with various congenital anomalies, however further studies are required to investigate the rare association between Rubinstein-Taybi syndrome and bilateral vocal cord abductor paralysis in future cases.

The quest persists for an ideal newer antiepileptic drug (AED) with better efficacy and tolerability. Levetiracetam (LEV) is one of these AEDs with a novel mechanism of action, good pharmacokinetic profile, acceptable tolerability, and side-effect profile. The present study assessed the safety and efficacy of intravenous levetiracetam as a first-line AED in neonatal seizures. This prospective observational study was conducted on all term neonates with seizures admitted to the Neonatal Intensive Care Unit (NICU) of a tertiary care center. Neonates with hypoglycemia, hypocalcemia, hypomagnesemia, inborn errors of metabolism, or those who received other AEDs prior to admission were excluded from the study. 20mg/kg Intravenous LEV was administered as first-line AED and graded up to 40mg/kg if seizures were not controlled in 2 h; thereafter, second-line AED was added. Only 36.2% (21/58) of the cases responded to LEV as first-line AED. Hypoxic Ischaemic Encephalopathy(HIE) was the most common etiology of seizures (55.2%). Subtle seizures were most responsive to LEV (60%), while multifocal clonic seizures (22.3%) responded the least. No adverse effect of LEV was observed during the study period. Only 36.2% of the cases responded to LEV as first-line AED, and subtle seizures were the most responsive seizures. Therefore, the efficacy of LEV as first-line AED in neonatal seizures is yet to be proven by a larger study. There were no adverse effects of LEV during the study period indicating the relative safety of this drug.

Heated humidified high-flow nasal cannula (HHHFNC) is gaining popularity as an alternative to nasal continuous positive airway pressure (nCPAP) therapy in the management of preterm neonates with respiratory distress due to ease of administration and patient comfort. However, limited evidence is available addressing its risks and benefits. To study the efficacy and safety of HHHFNC in comparison to nCPAP for the facilitation of extubation in preterm neonates (born at 27-34 weeks of gestation) with respiratory distress.
A prospective observational study was conducted, where 64 neonates were assigned either to nCPAP (n=34) or HHHFNC (n=30) groups post-extubation. The primary outcome was treatment failure (defined by pre-specified criteria) requiring a higher modality of respiratory support within 72 hours after extubation.
Treatment failure was seen in 36.7% of neonates assigned to the HHHFNC group compared to 14.7% in the nCPAP group (P=0.043). The incidence and severity of nasal trauma were higher in the nCPAP group compared to the HHHFNC group (nCPAP: 58.6% vs. HHHFNC: 15.7%; P=0.001). No significant difference was observed between the two groups in terms of other outcomes such as days on primary non-invasive ventilation (NIV), days of total NIV, duration of hospitalization, days to reach full enteral feeding, weight gain at discharge, incidence and severity of nasal trauma, incidence of pneumothorax, necrotizing enterocolitis, intraventricular hemorrhage, retinopathy of prematurity, sepsis, and death.
Though a gentler modality with less incidence of nasal trauma, HHHFNC does not appear to be as effective as nCPAP in the management of preterms with respiratory distress

The reported prevalence and pattern of thrombocytopenia in neonatal sepsis vary widely.
We aimed to determine the prevalence and severity of thrombocytopenia in blood culture proven neonatal sepsis.
The study was conducted in a University hospital by recruiting neonates with sepsis in whom blood culture had grown microorganisms. The initial platelet count refers to the one obtained at the same time as the positive blood culture. Platelet counts were monitored 12 - 24 hourly. Thrombocytopenia was considered mild if between 50,000/mm3 and 150,000/mm3, moderate if between 20,000/mm3 and 50,000/mm3, and severe if A total of 143 episodes of blood culture proven sepsis in 131 neonates were studied. Gram positive bacteria identified in 33.6%, gram negative bacteria in 53.8%, and fungi in 12.6%. Klebsiella predominated among Gram negative bacteria (39%) and Candida species (94.4%) among fungi. Initial thrombocytopenia was observed in 84 (58.7%) episodes; it was mild, moderate, and severe in 39.3%, 25%, and 35.7% respectively. Initial thrombocytopenia among Gram positive, Gram negative, and fungal sepsis were 41.7%, 70.1%, and 55.6%, respectively. Severe thrombocytopenia in the respective groups was 20%, 44.4% and 20%. Overall (80%) and severe thrombocytopenia (45.8%) was highest in Klebsiella sepsis. Thrombocytopenia was moderate in 60% of Candida sepsis. An additional 23.7% had thrombocytopenia subsequently. In 51.2%, thrombocytopenia persisted beyond 3 days.
Thrombocytopenia was observed in 58.7% of culture proven neonatal sepsis. Initial thrombocytopenia was common among Gram negative sepsis and mostly of a moderate degree in Candida sepsis.

Preterm birth, if not actively managed with developmental supportive care, can significantly contribute to neurodevelopmental compromise. Use of appropriate positioning aids helps with mimicking the intrauterine environment and facilitating neuromuscular development. Herein, we aimed to determine the effectiveness of snuggle up positioning aids on clinical outcomes of preterm ( This randomized controlled trial was performed at a tertiary neonatal intensive care unit (NICU) to investigate the effect of snuggle up position using positioning aids (vs. standard care) on heart rate, respiratory rate, oxygen saturation, duration of ventilation, weight gain, and duration of NICU stay.
There was a significant difference in respiratory rate, oxygen saturation, stability of the cardiorespiratory system in preterm infants (SCRIP) score, and weight gain between the intervention and control groups (P0.05).
Use of snuggle up position with positioning aids increased stability of physiological parameters and weight gain and reduced duration of NICU stay. Thus, the use of positioning aids for preterm infants is recommended to facilitate their growth and clinical outcomes.