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Purpose: Preterm infants are at increased risk of adverse neurodevelopmental outcome (NDO). Cranial ultrasound has limited predictability. The purpose of the study is to evaluate whether magnetic resonance imaging (MRI) done at term equivalent age (TEA) predicts NDO at 18-22 months of corrected gestational age (CGA).Materials and methods: This cohort study of preterm infants born at ≤32 weeks of gestation and/or birth weight <1500 grams between April 2011 and August 2012 was conducted in a tertiary care institute in India. MRI done at TEA was reported using objective scoring. NDO at 18-22-month CGA was assessed using Bayley Scale of Infant Development (BSID) version III. Composite score (CS) < 85 in motor, language, or cognition domain was taken as adverse NDO. Association between individual MRI subscores and NDO was evaluated using multiple linear regressions by backward elimination method. Validity of MRI abnormality in predicting adverse NDO was assessed.Results: Out of 94 infants who had MRI at TEA, 56 (60%) underwent BSID III. Mean gestational age was 29.8 ± 2.1 weeks. Median CS of all domains was lower with higher total MRI score. Predictive accuracy for various subscores ranged from 55 to 73%. By multiple regression analysis, signal abnormality was associated with motor delay (β -8.4; p .02) and cystic white matter (WM) changes with motor delay (β -7.3; p .003) and cognitive delay (β -6.1; p .005).Conclusions: Although specificity and negative predictive value were moderate to high across all subscores in MRI to predict the NDO, the accuracy has been only low to moderate, which limits its use as sole predictor. [ABSTRACT FROM AUTHOR]

Introduction: Maple syrup urine disease (MSUD) is caused by the deficiency of branched‐chain keto acid dehydrogenase (BCKAD) and, it is well described that BCKAD contributed by an allograft following liver transplantation (LT) phenotypically normalizes this inborn error of metabolism (IEM). There is, however, a paucity of data especially with regards to the neurodevelopmental aspects and catch‐up growth profiles after LT in a resource‐challenged setting. We present our series of children under 6 years of age who underwent LT for MSUD particularly focusing on their amino acid homeostasis, neurodevelopmental and somatic growth profiles. Methods: Of 580 consecutive pediatric LT (PLT) performed between January 2011 and December 2022, all children who underwent LT for MSUD were included for analysis. Data accrued included peri‐LT details, pre‐ and post‐LT metabolic profile, neurodevelopmental assessment, somatic growth evaluation, and long‐term outcomes. Results: Six children underwent LT for MSUD with a median age and weight at LT of 20.5 (IQR: 8–60) months and 10.1 (IQR: 6.7–15.8) kg, respectively. One explanted liver was used as a domino graft for Arginase deficiency. Median follow‐up period was 52.5 (IQR: 27–94) months. None had vascular or biliary complications. Following LT, all children were started on an unrestricted protein diet and had normalization of BCAA levels. Post‐LT height and weight improved by 1 SD but did not achieve the normal profile. None of the children had neuro‐deterioration and have achieved new milestones. Conclusion: This is the first‐report presenting the growth aspects, amino acid and neurodevelopmental profiles of children who underwent LT for MSUD within the socio‐economic‐cultural idiosyncrasies and constraints prevalent in our part of the world. [ABSTRACT FROM AUTHOR]

BACKGROUND AND OBJECTIVES: Hypoglycemia occurs in 5% to 15% of neonates in the first few days. A significant proportion requires admission for intravenous fluids. Dextrose gel may reduce admissions and mother-infant separation. We aimed to study the utility of dextrose gel in reducing the need for intravenous fluids. METHODS: This stratified randomized control trial included at-risk infants with asymptomatic hypoglycemia. Study populations were stratified into 3 categories: small for gestational age (SGA) and intrauterine growth-restriction (IUGR), infants of diabetic mothers (IDM) and large for gestational age (LGA), and late preterm (LPT) neonates. Intervention group received dextrose gel followed by breastfeeding, and the control group (CG) received only breastfeeding. RESULTS: Among 629 at-risk infants, 291 (46%) developed asymptomatic hypoglycemia; 147 (50.4%) in the dextrose gel group (DGG) and 144 (49.6%) in CG. There were 97, 98, and 96 infants in SGA/IUGR, IDM/LGA, and LPT categories, respectively. Treatment failure in the DGG was 17 (11.5%) compared to 58 (40.2%) in CG, with a risk ratio of 0.28 (95% confidence interval [CI]: 0.17-0.46; P < .001). Treatment failure was significantly less in DGG in all 3 categories: SGA/IUGR, IDM/LGA, and LPT with a risk ratio of 0.29 (95% CI:0.13-0.67), 0.31 (95% CI:0.14-0.66) and 0.24 (95% CI:0.09-0.66), respectively. CONCLUSIONS: Dextrose gel reduces the need for intravenous fluids in at-risk neonates with asymptomatic hypoglycemia in the first 48 hours of life. [ABSTRACT FROM AUTHOR]

This is the first case report of neonatal sepsis with Streptococcus pseudopneumoniae. This preterm baby with Intrauterine Growth Restriction (IUGR) developed brain abscess on day 6 of life and the organism both in blood and cerebrospinal fluid (CSF) could be identified by genomic studies and not by blood culture. Streptococcus pseudopneumoniae is usually a respiratory pathogen in chronic obstructive pulmonary disease (COPD) patients whereas in the present scenario it caused cerebral abscess in a neonate which makes this an unusual presentation. [ABSTRACT FROM AUTHOR]

Background: Gastrointestinal (GI) malformations have varied short-term and long-term outcomes reported across various neonatal units in India. Methods: This descriptive study was done to study the clinical profile, outcomes and predictors of mortality in neonates operated for congenital GI malformations in a tertiary neonatal care unit in South India between years 2011 and 2020. Details were collected by retrospective review of the case sheets. Results: Total of 68 neonates were included with esophageal atresia (EA) in 10, infantile hypertrophic pyloric stenosis (IHPS) in 9, duodenal atresia (DA) in 10, ileal atresia in 8, jejunal atresia in 5, anorectal malformations (ARM) in 11, meconium ileus/peritonitis in 9, malrotation in 2, and Hirschsprung's disease (HD) in 4. Antenatal diagnosis was highest in DA (80%). Associated anomalies were maximum in EA (50%), the most common being vertebral, anal atresia, cardiac defects, tracheoesophageal fistula, renal and radial abnormalities, and limb abnormalities association (VACTERL). Overall mortality was 15%. IHPS, DA, Malrotation, HD and ARM had 100 % survival while ileal atresia had the least survival (38%). Gestational age <32 weeks (odds ratio [OR] 12.77 [1.96, 82.89]) and outborn babies (OR 5.55 [1.01, 30.33]) were significant predictors of mortality in babies operated for small intestinal anomalies. None of the surviving infants were moderately or severely underweight at follow-up. Conclusion: Overall survival of surgically correctable GI anomalies is good. Among the predictors for mortality, modifiable factors such as in-utero referral of antenatally diagnosed congenital anomalies need attention. One-fifth had associated anomalies highlighting the need to actively look for the same. Although these neonates are vulnerable for growth failure, they had optimal growth on follow-up possibly due to standardized total parenteral nutritional policy during neonatal intensive care unit stay. [ABSTRACT FROM AUTHOR]

Varicella zoster virus (VZV) is a neurotropic alphaherpesvirus that causes neurological manifestations either as a complication of primary infection or reactivation. VZV induced neurological diseases have a good prognosis when confirmed early and treated with anti-viral therapy. Myelitis, encephalitis, ventriculitis or meningitis can occur without a telltale rash in immunocompetent and immunocompromised individuals making the diagnosis difficult. We analyzed CSF and serum samples from 30 unvaccinated study participants (17 male and 13 female) to determine the presence of VZV DNA by PCR in CSF and to estimate serum and CSF anti-VZV IgG and albumin levels in participants with neurological manifestations with/without rash. Anti-VZV IgG was detected in CSF (n = 22, [73%]) and serum (n = 29, [97%]) of pediatric and adult participants. Anti-VZV IgG were detected in CSF of participants with varied clinical presentation altered sensorium (n = 8, [36%]), meningitis (n = 4, [18%]), acute febrile illness (n = 3, [14%], encephalopathy/meningoencephalitis (n = 2, [9%]), irritability (n = 2, [9%]) and each patient from cerebrovascular stroke, demyelinating disorder and febrile seizure (n = 1, [4.5%]). VZV DNA was detected from one participant and CSF serum albumin levels were elevated in 53% of study participants. VZV DNA is present up to 1–2 weeks post onset of disease, after which anti-VZV antibody may be the only indicator of disease and therefore both VZV DNA and anti-VZV IgG need to be tested for in CSF. As VZV DNA and VZV IgG antibody are both good indicators of VZV reactivation, routine testing would result in reduced morbidity and mortality by early detection of disease and antiviral treatment. [ABSTRACT FROM AUTHOR]

Non-bilious vomiting in preterm neonates discharged from neonatal intensive care units is a common complaint and is often associated with benign conditions such as gastro-oesophageal reflux. A neonate of 27 weeks gestation who presented later with vomiting owing to gastric outlet obstruction is described. He was discharged at 11 weeks of age and required re-admission 1 week later. He had persistent non-bilious vomiting from 7 weeks of age, failure to thrive and metabolic alkalosis. Clinical examination demonstrated visible gastric peristalsis, and hypertrophic pyloric stenosis was suspected. Ultrasound of the gastric pylorus and upper gastro-intestinal contrast studies were negative. Exploratory laparotomy after failure of conservative management revealed a thickened mucosal fold in the gastric pylorus, which was excised. Histopathology demonstrated inclusion bodies which are pathognomonic of cytomegalovirus infection. He was treated with valganciclovir for 6 weeks and was asymptomatic and thriving well at follow-up. Gastric outlet obstruction can be one of the manifestations of CMV infection of the gastro-intestinal tract. Diagnosis can be confirmed only by histopathology. Abbreviations: BPD: bronchopulmonary dysplasia; CMV: cytomegalovirus; H&E: haematoxylin and eosin; IHC: immunohistochemistry; IHPS: infantile hypertrophic pyloric stenosis; NEC: necrotising enterocolitis; PCR: polymerase-chain reaction; VGP: visible gastric peristalsis. [ABSTRACT FROM AUTHOR]

Objectives: Early establishment of human milk (HM) feeding significantly decreases neonatal morbidity. There are barriers in establishing and maintaining lactation in mothers of hospitalized extreme preterm and very low birth weight (VLBW) infants, particularly during the first 2 weeks of life. Infant feeding policy modification was done to improve lactation as a quality initiative project and the effect of this modification was studied. Material and Methods: This study was done at SRIHER by analyzing retrospective data from the period before infant feeding policy modification (April 1, 2017-June 30, 2017) and prospective data from post-policy modification (July 1, 2017-December 31, 2017). All extreme preterm babies and VLBW babies both inborn and outborn were included in the study. The policy was approved by the institutional quality control cell. Fishbone analysis and "why" question pattern were implemented to identify pitfalls in establishing and maintaining lactation in mothers of extreme preterm and VLBW mothers. Prior antenatal counseling (for inborn deliveries), immediate postnatal assessment, constant surveillance, internal audits, and regular interprofessional team meets were done to carry out the implementation of the quality control program. Results: Daily milk output improved after intervention from baseline value in the pre-policy modification group to nearly 3 times after policy modification. The total duration of the requirement of parenteral nutrition decreased from 11 days to 7 days and time to reach birth weight decreased from 17 days to 11 days after quality improvement initiative. Conclusion: A simple quality improvement initiative was able to achieve increased lactation in mothers of extreme preterm and VLBW neonates. [ABSTRACT FROM AUTHOR]

Meconium peritonitis as a cause of non-immune hydrops in neonates is rarely reported. Here we report such a rare occurrence. In our case, a routine antenatal scan at 25 weeks revealed isolated ascites. By 31 weeks of gestation, all features of hydrops were observed in scans. However, antenatal workup for immune and non-immune hydrops was negative. Subsequently, a preterm hydropic female baby was delivered at 32 weeks. She required intubation and ventilator support. An X-ray revealed calcification in the abdomen suggestive of meconium peritonitis. Ultrasound showed gross ascites, a giant cyst compressing the inferior vena cava, and minimal bilateral pleural effusion. Emergency laparotomy revealed meconium pellets and perforation of the ileum. Double-barrel ileostomy was performed, and the edema resolved and activity improved. The baby was discharged after 3 weeks. Ileostomy closure was done at follow-up. The baby is growing well. [ABSTRACT FROM AUTHOR]

BACKGROUND AND OBJECTIVES Postnatal growth failure happens in about half of the very low birth weight infants and this can have long-term consequences. Human milk-based multi-nutrient fortifiers (HMBF) are thought to be better tolerated than bovine milk-based multi-nutrient fortifiers (BMBF), thus facilitating early progression to full feeds and improved growth in preterm neonates. This study was done to find the advantage of HMBF over BMBF on postnatal growth and other clinical outcomes. METHODS This is a retrospective cohort study where babies <1500 g birth weight or gestational age <32 weeks were included to compare the velocity of weight gain (g/kg/day), duration of hospital stay and clinical outcomes between fortification using HMBF and BMBF till 34 weeks postmenstrual age. RESULTS Eligible neonates included in the study were 322, out of whom 123 (37%) received HMBF and 209 (63%) received BMBF. During the stay, 18 babies were changed from BMBF to HMBF and vice versa in 24 babies due to logistic reasons and parents' preferences. The mean birth weight of the babies was 1124 ± 237 g. Weight gain was higher in the exclusive HMBF group [mean difference 0.77 (0.14, 1.39) g/kg/day; p -value = 0.018]. Feed intolerance [odds ratio (OR) 0.45 (0.22, 0.95), p -value 0.037] was also significantly less in this group. However, other morbidities did not differ significantly between the groups. CONCLUSION Higher weight gain and lower feed intolerance in the HMBF group underscores the possible advantage of using HMBF over BMBF. Larger prospective studies might bring out its effect on the duration of hospital stay and other morbidities. [ABSTRACT FROM AUTHOR]

We present the diagnostic dilemma of a neonate with two umbilical soft tissue masses. Case report: The baby had an umbilical mass herniating through the umbilical cord, and another mass hanging from the umbilical mass by a string of tissue. Both masses were amorphous solid soft tissues and the hanging mass had hair on the surface. Clinical diagnosis was umbilical cord teratoma. However, histopathological examination of the masses showed that tissues representing various organs were arranged in cephalocaudal order as in a fetus, revealing that it was a parasitic twin. The hanging mass was probably the cephalic part and the umbilical mass was malformed torso and limbs. Conclusion: This parasitic omphalopagus heteropagus parasitic twin presented as two amorphous masses without externally identifiable anatomic structure, The parasitic twin of omphalopagus heteropagus may have unusual presentations. Histopathological examination was essential to diagnose whether it is a twin or a tumor. [ABSTRACT FROM AUTHOR]

Objective: To assess the utility of autologous umbilical cord blood (UCB) for red cell concentrate (RCC) transfusion in preterm infants. Methods: We recruited preterm infants born at â¤30 weeks' gestation or have an estimated fetal weight <1,200 g. We intended to perform delayed cord clamping (DCC) and to collect UCB following DCC. The quality parameters used included blood culture performed once, and biochemical and haematological parameters assessed weekly. Results: Of the 46 recruited neonates, DCC could be performed for 1 minute in 11 (23.9%) and for 30-59 seconds in 10 (21.7%) infants. The success rate of UCB collection was significantly lower in infants who underwent DCC for 1 minute (27%) compared to those who underwent DCC for 30-59 seconds (70%) or immediate cord clamping (72%) (p value 0.031). Twenty-five UCBs were stored after eliminating three that had positive culture. UCB had satisfactory quality for transfusion from day 3 (when blood culture report was available) to 14 (after which pH decreased to <6.5). Thirteen infants required 27 RCC transfusions. Autologous UCB could be used for only five (18.5%) transfusions. Conclusion: The success rate of UCB collection after DCC for 1 minute is low. Autologous UCB meets less than one-fifth of transfusion requirements. Hence, autologous UCB transfusion is not a workable option in preterm infants. [ABSTRACT FROM AUTHOR]

To study the utility of clinical exome sequencing (CES) using next generation sequencing (NGS) in evaluating neonates with suspected genetic conditions. This is an observational study conducted in a tertiary care neonatal unit. We included neonates with suspected genetic conditions, for whom CES were done either by direct sampling or from stored DNA. Data was collected from the Sri Ramachandra centre of excellence in perinatal health (SCOPE) case records of 2016–2019. Yield of CES, percentage of pathogenic, non-pathogenic and variant of uncertain significance (VUS) and associated disorders were studied. CES was done in 36 neonates. Variants were detected in 78% (28/36). However, significant variants with clinical correlation were present in 20 (56%) babies. Test was carried out from the stored sample in 10 (28%) babies. Mean turn-around time was 39 ± 7 days. Specialist was involved in 1 and treatment changes were done in 5 neonates. Five out of 8 VUS were clinically correlating. Inborn errors of metabolism were the commonest (60%). Two VUS were ascertained as likely pathogenic after parental segregation analysis. CES has a definite role in evaluation of suspected genetic conditions for diagnosis and prognostication. It also helps scientific society to build in additional evidence so that the "VUS" could be asserted as "likely pathogenic". Our experience reiterates the importance of storing and archiving DNA of the affected child. [ABSTRACT FROM AUTHOR]