Indian Journal of Neonatal Medicine & Research

Adams Oliver Syndrome- A Rare Entity

Jyoti Rani Thakur, Nancy Pherlin Kharkongor, Jagjit Singh Dalal

Adams-Oliver syndrome (AOS) is a rare congenital disorder with an incidence of 1 in 225,000 individuals. It is characterised by the presence of Aplasia Cutis Congenita (ACC) of the scalp and transverse limb defects. The authors herein describes a rare case of late preterm (36-weeks) male neonate born with cranial vault defect and transverse limb defect in the form of constricted short fingers of bilateral hand with Atrial Septal Defect (ASD) diagnosed on Echocardiography (ECHO) while screening. No other associated anomalies were found. Baby was diagnosed as AOS based on these features. Patient was clinically stable with no signs of complications due to skull defect. Multidisciplinary team was involved in management and closure of skull defect was planned if not closed spontaneously in follow-up and early physical therapy or prosthetic application for the limb defects was planned as the child grows. Since the ASD was small, parents were reassured about the spontaneous closure with regular two dimensional (2D) ECHO monitoring. On follow-up at nine months of age, scalp defect closed spontaneously and child is growing well. Clinicians should be aware and all the babies born with ACC and scalp defect should be evaluated for associated anomalies as it can be a part of AOS. The early evaluation and management can be planned accordingly for better outcome.

Neonatal Multisystem Inflammatory Syndrome Associated with Passive Transfer of Antibodies

Multisystem Inflammatory Syndrome in Children (MIS-C) is a postinfection immune-mediated condition, seen four to six weeks after Coronavirus Disease-2019 (COVID-19). Multisystem Inflammatory Syndrome in Neonates (MIS-N) is a similar hyperinflammatory syndrome seen among neonates. The exact mechanism of MIS-N is not clear. The transplacental transfer of antibodies from Severe Acute Respiratory Syndrome-Coronavirus-2 (SARS-CoV-2) infected mothers may lead to hyperinflammatory response among the neonates. In the current case report, the first 36-week-gestational-age male baby presented with respiratory symptoms and increased coronary Z scores which responded well to Intravenous Immunoglobulins (i.v. IG), steroids, and aspirin. The second 36-week-gestational-age male baby presented with unusual recurrent apnoeic attacks for that gestation (36 weeks) which responded dramatically to i.v IG and steroids. Immunoglobulins play a key role in the management of MIS-N.

Clinical Pattern and Course of Nephrotic Syndrome in Children: An Observational Study

Rahul Kadam, Vidya Singaravelu, Sushama S Bai, Madhusudhan

Introduction: Chronic renal diseases not only affects the personal life of an individual but also results in social and financial burden on their families too. Nephrotic syndrome is most common in the paediatric age group contributing to significant illness related morbidity and even mortality if not identified early and treated promptly. This common disease has shown varying clinical trends with studies attempting to document them. Aim: To study the clinical pattern and course of nephrotic syndrome in children. Materials and Methods: The study was a retrospective analysis of medical records of diagnosed nephrotic syndrome patients in the Paediatrics and Nephrology Departments of a Tertiary Care Medical college hospital. The patients were prospectively followed-up for a period of two years. A total of 90 patients were enrolled into the study and their case records were systematically reviewed to obtain the details regarding age, sex distribution, number of admissions, hospital stay during first admission, complications and interim problems, histopathology finding where indicated, diagnostic classifications based on traditional operational definitions, treatment received, duration of remission after first episode and its relation to future relapses and the final outcome were noted and then statistically analysed. Results: Of the 90 cases of nephrotic syndrome, 14 were lost during follow-up, so remaining 76 patients were included. There were 49 males (64.5%) and 27 females (35.5%). Steroid-Sensitive Nephrotic Syndrome (SSNS) was the most common type (59%). It was found that 67 (88.1%) patients had first episode within 1-5 years of age, 31(40.8%) had only 1st time admission, 35 (46%) had 2-4 times admissions, while the rest (13.2%) required more than 4 times admissions. Majority of the cases {48 (63.2%)} had hospital stay between 7-14 days. Infectious complications were the most common (35.3%) and 48 (63.1%) cases showed frequent relapse, while 16 (21%) never had a relapse. The final outcome showed 64 (84.2%) of the cases were in remission, 12 (15.8%) were still relapsing, and there was no mortality. Conclusion: Age of onset for nephrotic syndrome was 5 years in majority of cases. Males were more affected than females. Majority of the patients had hospital stay for 7-14 days. SSNS was the most common type of nephrotic syndrome and Urinary Tract Infection (UTI) was the most common infectious complication. As respiratory and UTIs account for majority of relapses, prevention of respiratory infections by observing respiratory etiquettes, early and aggressive treatment and respiratory protective vaccines will go a long way in prevention of relapses.

Clinical Profile and Epidemiology of Neonates Presenting with Acute Gastroenteritis with Special Emphasis on Acute Kidney Injuryby

Raman Sharma, Vipul Taneja, Kajal Khajuria, Rasmeen Kaur, AK Bhardwaj

Introduction: Acute Gastroenteritis (AGE) is one of the most common causes of hospitalisation in children as well as neonates. According to World Health Organisation (WHO), 80% of deaths due to diarrhoea occur in first two years of life. Acute Kidney Injury (AKI) is one of the most common complications associated with gastroenteritis and dehydration. Neonates constitute major bulk of infant mortality and morbidity. The goal of this study was to identify risk factors, feeding pattern and electrolyte abnormalities in neonates presenting with AGE. Aim: To study the clinical profile and epidemiology of neonates presenting with AGE with special emphasis on AKI. Materials and Methods: A prospective observational study was conducted in the Neonatal Intensive Care Unit (NICU) of Department of Paediatrics between December 2019 to May 2021 at MMIMS Research and Hospital, Mullana, Ambala, Haryana, India. Ethical clearance was obtained from the ethical committee prior to the study. A total of 510 neonates were admitted during this period in NICU. Out of them 151 neonates of AGE who met the inclusion criteria were enrolled in the study. Clinical Data and lab investigations i.e., serum electrolytes and Renal Function Test (RFT) were collected and entered in pretested proforma meeting the objectives of the study. Kidney Disease Improving Global Outcome (KDIGO) was used to diagnose AKI. Results: In this study, out of the 151 patients of AGE, 56 (37.09%) patients developed AKI. Hyperkalaemia in 85 (56.29%) patients was the most common electrolyte abnormality followed by hypernatremia 35 (23.18%). Hyponatraemia was present in only 15 (9.9%) patients. Acidosis was present in 41 (27.15%) patients. Exclusive formula feeding was the single most modifiable risk factor for AKI. Conclusion: It was found that feeding pattern is the most important and modifiable risk factor associated with increased incidence of AGE and AKI in neonates. Exclusive formula fed babies are more prone to AGE due to improper composition of feeds as compared to mixed feeding or exclusive breast feeding. Thus, exclusive breast feeding must be promoted. Hyperkalaemia remained the most common electrolyte abnormality in these patients.

Comparison of Umbilical Cord blood Bilirubin (UCB) and Bilirubin Albumin Ratio (BAR) in Predicting Neonatal Hyperbilirubinemia: A Prospective Observational Study

T Rehna, KP Shiyas

Introduction: Hyperbilirubinemia is a commonly encountered medical condition in neonates. It becomes problematic when the levels of bilirubin raises to abnormally high values leading on to neurological problems. Bilirubin Albumin Ratio (BAR) can be used as a prediction tool for subsequent hyperbilirubinemia in neonates thereby helping in early institution of therapy. Aim: To compare Umbilical Cord blood Bilirubin (UCB) and the BAR in predicting neonatal hyperbilirubinemia. Materials and Methods: This prospective observational study was conducted in the neonates born at Al-Azhar Medical College, Thodupuzha, Kerala, India, from April 2020 to February 2021. After obtaining clearance from Institute Research Committee and Institute Ethical Committee, 1025 healthy term babies were included in the study. After an informed consent from either of the parent, cord blood was sent for bilirubin, albumin and the blood group estimation. Babies were examined daily for any development of jaundice for five days or till discharge. Venous blood was sent for bilirubin estimation if clinical icterus was noted by Kramer’s rule any time after birth or at 72 hours. If hyperbilirubinemia was detected, treatment was instituted. Two cut-offs for UCB-2 and 2.5 mg/dL and two cut-offs for BAR 0.59 and 0.69 were correlated with the neonatal hyperbilirubinemia using Pearson correlation and Chi-square test. The p-value <0.05 was taken as statistically significant. The cut-off values for cord bilirubin and BAR that could predict hyperbilirubinemia was also obtained from Receiver Operating Curve (ROC). Results: Of the 1025 babies studied, hyperbilirubinemia was detected in 246 babies (24%). Babies with higher UCB and BAR had statistically significant risk of neonatal hyperbilirubinemia. UCB >2 mg/dL and 2.5 mg/dL and BAR 0.59 and 0.69 were found to strongly correlate with the risk of hyperbilirubinemia. Higher the UCB and BAR, higher the risk. On ROC analysis, cut-off points for UCB and BAR were 2 mg/dL and BAR >0.59 respectively. A highly significant correlation was found between UCB and hyperbilirubinemia as well as between BAR and hyperbilirubinemia with a p-value <0.001. Among UCB and BAR, UCB is found to have better sensitivity and specificity than BAR with cut-off 2 mg/dL with better sensitivity of 75.2% and cut-off 2.5 mg/dL with a better specificity of 89.6%. Conclusion: UCB and BAR are strong predictors of neonatal hyperbilirubinemia with UCB a better predictor than BAR.

Morbidity and Mortality Profile among Low Birth Weight Neonates- A Cross-sectional Study from Jalandhar, India

Introduction: World Health Organisation (WHO) has defined Low Birth Weight (LBW) as weight at birth of fewer than 2,500 grams in the first hour of delivery. This practical cut-off for international comparison is based on epidemiological observations that infants weighing less than 2,500 gm are approximately 20 times more likely to die than babies with a normal birth weight. LBW is closely associated with neonatal and infant mortality and morbidity and stifled growth and cognitive development of children. Aim: To know the aetiological profile and outcome of admitted Low birth weight babies in Neonatal Intensive Care Unit (NICU). Materials and Methods: The present study was hospital based retrospective study conducted on 610 neonates admitted in NICU at a teaching hospital, Punjab Institute of Medical Sciences, Jalandhar, from January 2019 to December 2019. Neonate with birth weight less than 2500 gm and less than 28 days old were included in the study. The total subjects were divided into three groups- Group 1: Extremely Low Birth Weight (ELBW) <1000 gm; Group 2: Very Low Birth Weight (VLBW): 1000-<1500 gm; Group 3: Low Birth Weight (LBW): 1500-<2500 gm. All the babies were investigated and managed as per standard hospital protocol. Data recorded were: demographic profile, aetiology, morbidity and mortality among LBW babies. Data was analysed using Statistical Package for Social Sciences(SPSS) version 21.0 using standard statistical tests. Results: Total number of LBW babies registered in the study was 610. Among them, weight of 72 (11.8%) of neonates were <1000 g (Group 1), 208 (34.1%) were weighed 1000-<1500 g (Group 2) and 330 (54.1%) were weighed between 1500-<2500 g (Group 3). The mortality rate was 3.77%. The major causes of admission observed in neonates were sepsis (45.2%), hyperbilirubinemia (29.5%), apnoea (24.2%), hypoglycaemia (21.9%) and respiratory distress (17.2%). Highest mortality was observed amongst cases with Extremely Low Birth Weight (ELBW) (p<0.01). Two neonates out of total subjects 19 (3.1%) diagnosed with necrotising enterocolitis (NEC) succumbed to death, both belonging to group 1. Conclusion: The leading cause of morbidity and mortality in LBW babies were sepsis and Necrotising Enterocolitis (NEC) so we need to address this problem more effectively.

Neonatal Outcome of Antenatally Diagnosed Hydronephrosis at a Tertiary Care Centre, Telangana, India: A Prospective Study

Sneha Latha Gopu, Ayesha Begum

Introduction: Antenatal Hydronephrosis (ANH) is one of the most commonly detected congenital anomaly by antenatal sonogram. It requires periodic follow-up and in selected cases, early interventions are required in postnatal period. Aim: To study the relationship between Anteroposterior Diameter (APD) of foetal renal pelvis and postnatal outcome of hydronephrosis in neonates and to study the relation between APD of foetal renal pelvis and structural abnormalities of kidney. Materials and Methods: This prospective study included neonates whose antenatal scan showed hydronephrosis. The study was conducted during the period of November 2018 to November 2020, in Niloufer Hospital (Osmania Medical College), Hyderabad, Telangana, India. A total of 50 neonates were included. For all neonates postnatal monitoring with ultrasonography was done to detect structural anomalies and resolution of hydronephrosis. Micturating cystourethrogram was done in selected cases. The cases which needed surgery were treated accordingly. The Statistical Package for the Social Sciences (SPSS) version 24.0 was used for data analysis. Kruskal-Wallis Chi-square test was used to test the significance of difference between quantitative variables. The p-value less than 0.05 was taken as statistically significant. Results: Among 50 neonates enrolled in the study, 27 (54%) had transient hydronephrosis and 23 (46%) had urogenital structural abnormalities. Twenty-two cases (44%) required micturating cystourethrogram. Antenatal APD of 9.5 mm predicted the development of significant postnatal uropathy with a sensitivity of 74% and specificity of 89%. Antenatal APD of 9.5 mm had 100% sensitivity and 67% specificity in predicting the need for surgical intervention. Only 3 (6%) cases needed surgical intervention in neonatal period and all cases were kept under follow-up. Conclusion: All cases of ANH need postnatal evaluation and pertinent follow-up to detect significant uropathy for their proper management to prevent renal damage. Antenatal renal pelvic diameter has significant role in predicting the significant uropathy and surgical intervention.

Hepaticojejunostomy vs. Hepaticoduodenostomy after Excision of Choledochal Cyst in Paediatric Population- A Retrospective Study

Gagan Deep, Suhitha Gajanthody, Ashraf Ahmad, B Harishchandra

Introduction: Choledochal cyst is a rare congenital anomaly of the bile duct with female predominance. Surgical excision of the choledochal cyst with hepaticoduodenostomy or hepaticojejunostomy is the standard of care in the last few decades. Many surgeons favour hepaticojejunostomy. Aim: To evaluate the outcomes of open hepaticoduodenostomy vs. hepaticojejunostomy procedure in excision of choledochal cyst in paediatric population. Materials and Methods: This was a retrospective cohort study conducted from January 2015 to December 2019 on data of 16 paediatric patients who underwent choledochal cyst excision with biliary reconstruction in a tertiary health institute. The data of these patients were collected from Medical Records Department (MRD) on type of surgery performed, mean operating time, postoperative hospital stay, the onset of oral feeds and complications associated with the surgery and were analysed and evaluated in July 2021. All statistical analyses were performed with Statistical Package for Social Sciences (SPSS) version 23.0. A p-value of <0.05 was considered as statistically significant. Results: A total of 16 patients (5 males and 11 females) were included, out of them 9 (56%) had undergone hepaticoduodenostomy and 7 (44%) had undergone hepaticojejunostomy. The most common type of choledochal cyst was Type 1 (n=7, 43.7%). Operative time was longer for the hepaticojejunostomy than the hepaticoduodenostomy (212.86±31.33 vs 88.89±15.16 min; p-value 0.001). Patients who underwent hepaticoduodenostomy early feeds were started as compared to the hepaticojejunostomy (2.22 vs 5.29 days; p-value 0.001). Hospital stay was longer with the hepaticojejunostomy than the hepaticoduodenostomy (14.71±7.76 days vs 8.11±4.48 days; p-value 0.05). There were three complications (biliary enteric fistula that opened into the anterior abdominal wall at the previous surgical site incision, jejunal stump formed the fistulous tract with high output bile leak and surgical site infection was noted) in total noted in both groups. Conclusion: The present study concluded that hepaticoduodenostomy had a better outcome in factors such as operating time, the onset of feeds and hospital stay than with the hepaticojejunostomy.

Postnatal Maturation of Amplitude Integrated Electroencephalogram in Preterm SGA and Preterm AGA Neonates: A Prospective Observational Study

Prabhudev Basavaraj Hasbi, Gopi Kishan Sharma, Amrit Lal Bairwa

Introduction: Maturation of the brain is affected by various biological and environmental factors encountered by the infant during the intensive care period in Neonatal Intensive Care Unit (NICU) due to the medical treatments, procedures, and the noisy environment that disrupts the normal brain development process. Severe neurological sequelae of preterm infants are common because of the immature central nervous system. Cerebral Function Monitor (CFM) or Amplitude integrated Electroencephalogram (a-EEG) is a device for monitoring the background neurological activity. Aim: To assess postnatal maturation of a-EEG in clinically stable and neurologically normal preterm Small for Gestational Age (SGA) and preterm Appropriate for Gestational Age (AGA) neonates from 30 weeks 0/7 days to 34 weeks 6/7 days of gestation admitted in a tertiary care NICU at J. K. Lon Mother and Child Hospital, attached to Government Medical College, Kota. Materials and Methods: This prospective observational study was conducted over a one year duration, from January 2020 to December 2020 on 60 preterm neonates that were admitted in NICU of a tertiary care hospital. The serial a-EEG recording was done on haemodynamically stable, included preterm neonates after taking consent, on 3rd, 7th, and 14th postnatal day of life during the course of admission. The postnatal maturation of amplitude integrated EEG of Preterm Small for Gestational Age (PSGA) neonates was compared with their Preterm Appropriate for Gestational Age (PAGA) neonates based on a validated a-EEG scoring. The analysis was done by using Statistical Package for Social Sciences (SPSS) version 21.0. Student t-test was applied. Results: The total a-EEG scores for 3rd, 7th, and 14th day of SGA group neonates were 7.55±1.45, 7.25±1.02 and 10.22±1.05 and were delayed from the AGA group of neonates with 7.86±1.55, 8.68±1.00 and 10.62±1.01, with mean difference (95% CI), 0.30 (-0.49 to 1.13), 1.43 (0.88 to 1.97) and 0.39 (-0.15 to 0.95) respectively. Only the total a-EEG scores for day 7 were significantly delayed in SGA group. Conclusion: All the maturation a-EEG scores of clinically stable and neurologically normal PSGA neonates was found to be significantly delayed at any point of life on postnatal day 7^th of life.

Effects of Maternal Vitamin D Deficiency on the Newborn: A Cohort Studybirth

Ramesh Sonowal, Monigopa Das, Bipul Prasad Deka, Pritam Das

Introduction: Vitamin D deficiency occurs during pregnancy all over the world because of lifestyle modifications, less sun exposure, sunscreen application and reduced amount of vitamin D in diet. At present, there are very limited number of studies regarding the prevalence of vitamin D deficiency during pregnancy and its effects in the newborns in the North Eastern region of India. Aim: To estimate serum vitamin D levels in pregnant women and newborn and correlation between maternal and newborn hypovitaminosis D. The study also assess the association of maternal vitamin D levels with the outcomes of early neonatal period. Materials and Methods: A prospective cohort study was conducted in Assam Medical College and Hospital (AMCH), Dibrugarh, Assam, India, in the Obstetrics and Gynaecology Department over a period of one year (1st July 2019 to 30th June 2020). Seventy pregnant women attending antenatal ward, labour room of AMCH were recruited. The 25-hydroxy vitamin D level was assessed in the maternal and cord blood. Delivery details and foetal parameters were recorded. Primary outcome was to assess the correlation between maternal and cord blood vitamin D level and secondary outcome was to assess the association of maternal vitamin D level with demographic characteristics, economic and obstetrical characteristics, newborn characteristics and perinatal outcome. Analysis of Variance (ANOVA), chi-square test and Fischer’s-exact test, Pearson’s correlation coefficient (r) were used for statistical analysis. Results: Out of 70 participants, majority of the pregnant women (61.43%) were vitamin D deficient, 22.86% were vitamin D insufficient and 15.71% had sufficient vitamin D levels. Maternal vitamin D level was significantly associated with maternal age (p=0.0043) and socio-economic status (p=0.0417). There was a significant positive association between the vitamin D level of the mother with the birth weight (p<0.001), birth length (p=0.0001), head circumference (p=0.0003) and chest circumference (p=0.001) of the newborns and also a positive correlation was found between the vitamin D level of the mother and the cord blood (r=0.984). There was a significant association between the vitamin D level of the mother and perinatal outcomes such as Neonatal Intensive Care Unit (NICU) admission (p=0.0169) and Respiratory Distress Syndrome (RDS) (p=0.0029). Conclusion: The study shows that there is a high prevalence of vitamin D deficiency during pregnancy in North Eastern part of India and it has a significant role in foetal development and perinatal outcomes.