Neonatal nephrotic syndrome: all is not gloomy.

Congenital nephrotic syndrome (CNS) is a rare clinical syndrome with a constellation of proteinuria, hypoalbuminaemia and oedema, presenting within 3 months of birth. We present a rare case of neonatal nephrotic syndrome with a probable sepsis induced aetiology. The neonate was referred at day of life 15 with Klebsiella pneumonia sepsis and anasarca. On investigation, the patient had nephrotic range proteinuria, hypoalbuminaemia, generalised anasarca and ascites. The neonate was started on broad-spectrum antibiotics and furosemide. Genetic and other secondary causes of CNS were ruled out. With supportive management and resolution of sepsis, the neonate improved. This case highlights the rare cause of sepsis-induced nephrotic syndrome (NS), which required only supportive treatment without the need for aggressive management of CNS.

Usefulness of skills in point of care ultrasound and simulation-based training as essential competencies in acute management of neonatal cardiac tamponade.

Peripherally inserted central catheter (PICC) insertion is a routine procedure in the neonatal intensive care unit required for prolonged intravenous fluid, nutrition and medication support. Neonatal cardiac tamponade is a serious and rare complication of PICC line insertion. Early detection by point of care ultrasound (POCUS) and management by pericardiocentesis improves the chances of survival. Regular simulation-based training sessions on a mannequin, along with knowledge of POCUS, can assist neonatologists and paediatricians for a quick and appropriate response in this emergency condition.

Unraveling Patterns of Congenital Structural Malformations in Infants: A Hospital-Based Descriptive Study.

Introduction Congenital malformation studies serve several purposes, including establishing baseline rates, monitoring changes over time, exploring the origins of these defects, and helping in planning health services. Increasing public awareness about pediatric surgical interventions is another goal of these studies. However, the impact of congenital malformations is often underestimated in developing countries due to insufficient healthcare data and diagnostic facilities, particularly in rural areas. Families affected by the birth of a child with congenital malformations face significant stress and hardship. Methods The main aims of this study were to evaluate the clinical pattern of congenital structural malformations in our region (Uttarakhand, India), identify possibly associated factors of congenital malformations, and find out the immediate outcome of congenital malformations in enrolled participants. Results Among a total of 150 cases, 73 (48.7%) cases were inborn, whereas 77 (51.3%) cases were outborn. Investigation of congenital malformation revealed cleft lip or palate in 37 (24.7%) cases, congenital heart disease (CHD) in 33 (22%) cases, meningomyelocele (MMC) in 18 (12.0%) cases, anorectal malformation (ARM) in 11 (7.3%) cases, hypospadias in 10 (6.7%) cases, congenital talipes equinovarus (CTEV) in nine (6.0%) cases, tracheoesophageal fistula (TEF) in nine (6.0%) cases, polydactyly in seven (4.7%) cases, pelviureteric junction obstruction (PUJO) in four (2.7%) cases, duodenal atresia in three (2.0%) cases, midgut volvulus in three (2.0%) cases, umbilical sinus in two (1.3%) cases, sacrococcygeal teratoma (SCT) in one (0.7%) case, phimosis in one (0.7%) case, microtia in one (0.7%) case, and micrognathia in one (0.7%) case. Mortality was observed in 11 (7.3%) cases, whereas 105 (70%) cases were successfully discharged. Among 11 mortality cases, the cause of death was CHD in seven (63.2%) cases, TEF+CHD in two (18.1%) cases, MMC in one (9%) case, and duodenal atresia in one (9%) case. Conclusion Contrary to the common belief that advanced maternal age of greater than 35 years is a major cause, 86.6% of the congenital structural anomalies in our hospital-based study in Uttarakhand occurred in babies of mothers belonging to the age group of 18-30 years. Also, consanguineous marriage was observed in only 3.3% of cases, indicating that it may not be a major contributing factor causing congenital structural malformations in our region. External congenital anomalies are most commonly observed (60.7%), with cleft lip and cleft palate being the most common. The most frequently observed internal congenital anomaly is CHD (22%) followed by gastrointestinal (GI) (18.6%) and urinary anomalies (10.1%). Death and referral are commonly seen in CHD.

Comparison of Three Methods of Umbilical Cord Management in Late Preterm and Term Newborns on Hemoglobin and Ferritin Levels at Six Weeks of Age: A Randomized Controlled Trial.

BACKGROUND: Umbilical cord milking (UCM) and delayed cord clamping (DCC) are strategies that improve the hemodynamic condition of the newborn and also increase the storage of iron. This study aimed to compare the effects of DCC with or without milking in late preterm and term neonates at different time intervals after birth (60, 120, and 180 seconds) on hematological and hemodynamic parameters in neonates at six weeks of age. MATERIALS AND METHODS: In this double-arm, parallel-group, triple-blind, and active-controlled trial, all 150 eligible neonates were randomized with allocation concealment into three groups: Group A (DCC with UCM at 60 seconds), Group B (DCC with UCM at 120 seconds), and Group C (only DCC for 180 seconds). Hemodynamic parameters were recorded and compared during the first 48 hours, and hematological parameters were compared at six weeks of age. RESULTS: At six weeks, a significant difference in hemoglobin levels was noted between Groups A, B, and C (p<0.001). The difference in serum ferritin values at six weeks was also statistically significant in comparisons across all three groups (p=0.003). Regarding secondary outcomes examined, hemodynamic parameters and the incidence of neonatal hyperbilirubinemia were found to be comparable at 48 hours after birth. CONCLUSION: DCC followed by UCM at 120 seconds and DCC till 180 seconds proves superior to DCC with UCM at 60 seconds in preserving elevated hemoglobin levels and iron stores in neonates at six weeks of age. DCC for 180 seconds yielded comparable results, followed by UCM at 120 seconds. All three methods are considered safe and effective without compromising the neonate's hemodynamics.

Extracerebral choroid plexus papilloma in a newborn: a rare presentation.

Choroid plexus papillomas (CPPs) are extremely rare lesions that originate in the central nervous system. Still rarely, these can occur in heterotopic locations. We report a case of aneonate who presented at fourth week of life with a small swelling in the left side of the oropharynx. There were no other symptoms. MRI scan revealed a cystic lesion with the possibility of lymphatic malformation or teratoma. Swelling which was gradually increasing in size was electively excised. Histopathological examination revealed it to be an extracerebral CPP. Postoperative period was uneventful, and baby had no further complications. CPP is a rare lesion and, almost always, cannot be anticipated before surgery. Imaging only helps in surgical planning. Complete excision is curative.

Novel variant CPLANE 1: c.5051C>A (p.Ser1684Ter) in an Indian neonate with Joubert syndrome.

Joubert syndrome (JS) is a rare ciliopathy that presents with the triad of hypotonia, developmental delay and molar tooth sign (MTS) in brain MRI. Next-generation sequencing has identified about 35 genes which are known to cause JS of which CPLANE 1 mutation is found in 8%-10% of cases. We report a case of JS in an Indian neonate who presented with hypotonia, dysmorphic facies, polydactyly, syndactyly and occipital encephalocele. MRI of the brain revealed MTS, and compound heterozygous mutations in CPLANE 1 gene were detected by clinical exome sequencing, one of them a novel variant CPLANE 1: c.5051C>A (p.Ser1684Ter) in exon 26, which was inherited from the parents.

Double BCG vaccination in a neonate: implications, management and prevention.

Tuberculosis is a common cause of morbidity and mortality especially in low-income and middle-income countries like India. BCG vaccination is recommended for all neonates after birth in areas with a high tuberculosis disease burden. Here, we describe a case where a neonate received two doses of the BCG (Chennai strain) vaccine within a span of 4 days after birth due to a vaccination error. Parents were informed about the event. The infant was managed conservatively and followed up till 12 months of life for any possible complication. There were no serious adverse effects apart from the localised reaction and a double scar on the left arm. Measures to avoid any such error in the future and the need for reporting medication error has been highlighted. Parental concerns are frequent in such scenarios and should be actively addressed.

The dilemma of feeding during the treatment of patent ductus arteriosus with oral ibuprofen in preterm infants ≤30 weeks of gestation-a randomized controlled trial.

OBJECTIVES: To evaluate the effect of minimal enteral feeding (MEN) versus withholding feeding on time to reach full feeds during treatment of hs-PDA with oral ibuprofen in infants ≤30 weeks. STUDY DESIGN: We performed a single-center, randomized control trial of 126 premature infants born ≤30 weeks gestation, <7 days of age with hs-PDA comparing continuation of MEN (n = 64) vs no feeding (n = 62) during treatment. The primary outcome was time to reach a feed volume of 150 ml/kg/day. Secondary outcomes included were episodes of feed intolerance, GI bleed, NEC and other comorbidities. RESULTS: There was no difference in the time to reach full feeds - median age of 16 days in both groups (p = 0.573). Incidence of feed intolerance, NEC and other secondary outcomes were also similar in both groups. CONCLUSIONS: Continuing MEN during treatment of hs-PDA with oral ibuprofen does not decrease time to reach full enteral feeds in very preterm infants.

Pain and Physiological Stress During Minimally Invasive Surfactant Therapy (MIST) in Very Preterm Infants.

OBJECTIVE: To evaluate the pain or physiological stress caused during minimally invasive surfactant therapy (MIST) to very preterm neonates. METHODS: In this prospective observational study conducted in a tertiary NICU, very preterm neonates were assessed for pain using Premature Infant Pain Profile-Revised (PIPP-R) score before, during and after MIST. Changes in the heart rate and oxygen saturation were also recorded during the procedure. RESULTS: 23 neonates who received MIST were assessed for pain using PIPP-R. Mean (SD) PIPP-R score during MIST was 3.87(1.3), before; 12.83 (1.9), during; and 6.26 (1.0), after the procedure, respectively (all P<0.001). Heart rate and oxygen saturation were also significantly reduced during MIST (P<0.001). CONCLUSION: The high PIPP-R scores during surfactant administration suggest that MIST can cause moderate to severe pain/discomfort and significant physiological stress in very preterm infants.

Comparison of Two Pumping Strategies to Improve Exclusive Breastfeeding at Discharge in Mothers of VLBW Infants with Low Milk Output - A Pilot Randomized Controlled Trial.

OBJECTIVES: To compare the effect of two strategies of breast pumping -power pumping (PP) vs. routine pumping (RP) over one week in mothers of very low birth weight (VLBW) infants with low milk output to improve breastfeeding rates at discharge. METHODS: Mothers with low milk output, defined as inability to express sufficient breastmilk to meet the feeding requirements of their infant on or after post-natal day 14, were randomized to receive power pumping vs. routine pumping - once daily for 7 d coupled with routine lactation support and hand expression 3 hourly in both groups. The primary outcome was exclusive breastfeeding at discharge. RESULTS: There was no difference in the two pumping strategies with respect to exclusive breastfeeding rates [61.1% in PP vs. 50% in RP group; (p = 0.477, RR 1.2; 95% CI 0.76 to 2.17)]. Median milk volume pumped in the individual power pumping session on 7th day of intervention was significantly higher than that in the individual routine pumping session on the 7th day (50 mL vs. 27 mL, p = 0.014). The cumulative median milk volume expressed per individual pumping session over the 7 sessions of power pumping was also higher than that with routine pumping (305 mL vs. 213 mL, p = 0.054). CONCLUSIONS: In this pilot trial, expressed milk volume was significantly higher after each individual power pumping session compared to routine pumping. However, the exclusive breastfeeding rates at discharge were similar in the two groups.

Improving Early Colostrum Feeding in a Tertiary Neonatal Intensive Care Unit: A Quality Improvement Initiative.

Background and Objective: Colostrum feeding is known for its immune benefits for reduction in nosocomial sepsis, necrotizing enterocolitis, and ventilator-associated pneumonias. Colostrum feeding also helps in improving breastfeeding rates and early discharge of vulnerable neonatal intensive care unit (NICU) babies. The objective of this study was to improve early colostrum feeding/oropharyngeal colostrum administration in a busy tertiary NICU in India. Methods: Multiple plan-do-study-act (PDSA) cycles were conducted from January 2020 to September 2020 to improve early colostrum feeding rates in NICU babies to >60%. We tested change ideas such as training of health care personnel, counseling of mothers and families about importance of colostrum expression and feeding, bedside collection of colostrum, safe transportation of colostrum to the NICU, and electronic data handling. Sustainability of the interventions was studied from October 2020 to March 2021 and data were analyzed. Results: Early colostrum feeding rates improved from a baseline of 4.36-68.21% after six PDSA cycles through 9 months. After counseling of mothers and families of NICU babies, rates of breastfeeding and colostrum feeding improved to 98.8% and 97.11%, respectively. The early breast stimulation and colostrum expression rates also improved to 87.28% and 68.2%, respectively. Early colostrum feeding rate was 87.5% after 6 months through the sustainability phase. Conclusions: Quality improvement interventions significantly improved the rate of early colostrum feeding in sick babies admitted to a busy NICU, and the improvement was sustained for 6 months.

Ultrasound-Guided Umbilical Venous Catheter Insertion to Reduce Rate of Catheter Tip Malposition in Neonates: A Randomized, Controlled Trial.

OBJECTIVE: To investigate whether ultrasound-guided umbilical venous catheter (UVC) insertion (US group) reduced the rate of malpositioning of the catheter tip compared to the standard method of insertion (SD group). METHODS: In this open-label, randomized, controlled trial, neonates admitted to NICU within the first week of life were randomly assigned to the US group (n = 26) or SD group (n = 27). Neonates with major congenital anomalies of the thorax and abdomen were excluded. The primary outcome was the rate of malpositioning of the catheter tip. RESULTS: The rate of malpositioning of the catheter tip was observed in a significantly lower number of neonates in the US group as compared to the SD group (11/26, 42.3% vs. 20/27, 74%; RR = 0.57, 95% CI: 0.34 to 0.94, p = 0.019). As more of the UVCs were positioned optimally in the first attempt in the US group than SD group, the need for repeated attempts at catheter repositioning was reduced, resulting in reduced procedure time (minutes) [mean (SD), 23.96 (6.42) vs. 30 (1.83); mean difference 6.04 (95% CI: 3.46 to 8.62), p = 0.005]. This also led to a reduction in the additional X-ray exposure in the US group (n = 11) compared to the SD group (n = 20) [95% CI: 3.12 to 44.26; p = 0.020]. CONCLUSION: Ultrasound-guided UVC insertion significantly reduced the rate of catheter tip malposition. It also reduced the number of attempts at catheter manipulation, procedure time, and X-ray exposures. With adequate training, it could be incorporated into routine bedside practice during UVC insertion for optimum placement. TRIAL REGISTRATION: Clinical Trial Registry of India ( www.ctri.nic.in ) CTRI/2021/03/031894.

Bleeding duodenal ulcer with ovarian torsion-a rare presentation in neonates.

Massive upper gastrointestinal bleed is an emergency in newborns. Common causes are coagulopathy and thrombocytopenia. Stress-induced duodenal ulcer has also been reported as an unusual cause for massive upper gastrointestinal bleed. Managing such cases requires correct diagnosis and prompt treatment to prevent catastrophic complications. We report a case of bleeding duodenal ulcer probably secondary to ovarian torsion.

Hypothermia for moderate or severe neonatal encephalopathy in low-income and middle-income countries (HELIX): a randomised controlled trial in India, Sri Lanka, and Bangladesh.

BACKGROUND: Although therapeutic hypothermia reduces death or disability after neonatal encephalopathy in high-income countries, its safety and efficacy in low-income and middle-income countries is unclear. We aimed to examine whether therapeutic hypothermia alongside optimal supportive intensive care reduces death or moderate or severe disability after neonatal encephalopathy in south Asia. METHODS: We did a multicountry open-label, randomised controlled trial in seven tertiary neonatal intensive care units in India, Sri Lanka, and Bangladesh. We enrolled infants born at or after 36 weeks of gestation with moderate or severe neonatal encephalopathy and a need for continued resuscitation at 5 min of age or an Apgar score of less than 6 at 5 min of age (for babies born in a hospital), or both, or an absence of crying by 5 min of age (for babies born at home). Using a web-based randomisation system, we allocated infants into a group receiving whole body hypothermia (33·5°C) for 72 h using a servo-controlled cooling device, or to usual care (control group), within 6 h of birth. All recruiting sites had facilities for invasive ventilation, cardiovascular support, and access to 3 Tesla MRI scanners and spectroscopy. Masking of the intervention was not possible, but those involved in the magnetic resonance biomarker analysis and neurodevelopmental outcome assessments were masked to the allocation. The primary outcome was a combined endpoint of death or moderate or severe disability at 18-22 months, assessed by the Bayley Scales of Infant and Toddler Development (third edition) and a detailed neurological examination. Analysis was by intention to treat. This trial is registered with ClinicalTrials.gov, NCT02387385. FINDINGS: We screened 2296 infants between Aug 15, 2015, and Feb 15, 2019, of whom 576 infants were eligible for inclusion. After exclusions, we recruited 408 eligible infants and we assigned 202 to the hypothermia group and 206 to the control group. Primary outcome data were available for 195 (97%) of the 202 infants in the hypothermia group and 199 (97%) of the 206 control group infants. 98 (50%) infants in the hypothermia group and 94 (47%) infants in the control group died or had a moderate or severe disability (risk ratio 1·06; 95% CI 0·87-1·30; p=0·55). 84 infants (42%) in the hypothermia group and 63 (31%; p=0·022) infants in the control group died, of whom 72 (36%) and 49 (24%; p=0·0087) died during neonatal hospitalisation. Five serious adverse events were reported: three in the hypothermia group (one hospital readmission relating to pneumonia, one septic arthritis, and one suspected venous thrombosis), and two in the control group (one related to desaturations during MRI and other because of endotracheal tube displacement during transport for MRI). No adverse events were considered causally related to the study intervention. INTERPRETATION: Therapeutic hypothermia did not reduce the combined outcome of death or disability at 18 months after neonatal encephalopathy in low-income and middle-income countries, but significantly increased death alone. Therapeutic hypothermia should not be offered as treatment for neonatal encephalopathy in low-income and middle-income countries, even when tertiary neonatal intensive care facilities are available. FUNDING: National Institute for Health Research, Garfield Weston Foundation, and Bill & Melinda Gates Foundation. TRANSLATIONS: For the Hindi, Malayalam, Telugu, Kannada, Singhalese, Tamil, Marathi and Bangla translations of the abstract see Supplementary Materials section.

Hypomelanosis of Ito with an unusual pulmonary abnormality in an infant.

Hypomelanosis of Ito (HI) is a neurocutaneous syndrome characterized by hypopigmented cutaneous lesions and extracutaneous manifestations frequently affecting the nervous system and the musculoskeletal system. Dysmorphic features, dental, ophthalmic, gastrointestinal, cardiac, and renal abnormalities are described in a minority of patients. The authors describe a 4-month-old infant having HI with unusual pulmonary hypoplasia that has not been reported so far.

Burkholderia cepacia sepsis among neonates.

Burkholderia cepacia is a rare cause of sepsis in newborns and its transmission involves human contact with heavily contaminated medical devices and disinfectants. The authors aimed to determine epidemiology, clinical features, antibiotic sensitivity pattern, complications and outcome of blood culture proven B. cepacia infections in 12 neonates. All neonates were outborn, 5 preterm and 7 term. B. cepacia was isolated from blood in all and concurrently from CSF in three neonates. Lethargy and respiratory distress (41.7 %) were major presenting features. Five newborns (41.7 %) required mechanical ventilation for 3-7 d. Highest bacterial susceptibility was observed for meropenem (100 %), followed by cefoperazone-sulbactam, piperacillin-tazobactam, sulfamethoxazole-trimethoprim (all 83 %), ceftazidime (75 %) and ciprofloxacin (42 %). Piperacillin-tazobactam, ciprofloxacin and cotrimoxazole either singly or in combination led to complete recovery of 11 (91.7 %) newborns; one developed hydrocephalus. Eight of nine infants who completed 6 mo follow up were normal. Prompt recognition and appropriate antibiotic therapy for B. cepacia infection results in complete recovery in majority.