Clinical presentation and outcomes of neonates born with neural tube defects- an experience from a level III B NICU in Western India.

PURPOSE: Neural tube defects (NTDs) are one of the most common congenital anomalies and a cause of chronic disability. The study was done to study outcomes of neural tube defects admitted at a tertiary level neonatal intensive care unit (NICU) from 2018 to 2022, a period of 4 years that also coincided with the COVID pandemic. The secondary outcome was to study the clinical presentation, associated anomalies and epidemiological features. METHODS: It was a retrospective observational study; data of infants was obtained from medical records and analysis was done. RESULTS: Thirty-four neonates were enrolled, of which there were 16 (47%) males and 18 (53%) females. History of pre-pregnancy maternal folate intake was present in 4 (11.7%) cases. 33 (97%) babies were diagnosed with meningomyelocele (MMC) and one each had anencephaly, iniencephaly and encephalocele, of which one had frontal and two had occipital encephalocele. The median age of surgery was 16 days of life with primary repair being the most common procedure followed by MMC repair with VP shunt. Twenty babies (58.8%) were discharged successfully, while 9 (26.5%) expired and 5 (14.7%) were discharged against medical advice; which can be attributed to the financial problems of the patients in a developing country. The overall deaths in our series were four (26.5%) which is slightly higher than other studies which may be due to the fact that this study was conducted during the COVID era with lesser rates of folate supplementation, reduced access to prenatal diagnosis coupled with poor follow-up and compliance of patients post-surgical repair. CONCLUSION: This study emphasizes the importance of periconceptional folic acid supplementation, prenatal diagnosis, early surgery and meticulous follow-up as being pivotal to improving outcomes in children with NTDs.

Neurological Manifestations of Perinatal Dengue.

Dengue viruses (DENVs) are single-stranded RNA viruses belonging to the family Flaviviridae. There are four distinct antigenically related serotypes, DENVs types 1, 2, 3, and 4. These are all mosquito-borne human pathogens. Congenital dengue disease occurs when there is mother-to-fetus transmission of the virus and should be suspected in endemic regions in neonates presenting with fever, maculopapular rash, and thrombocytopenia. Although most of the infected infants remain asymptomatic, some can develop clinical manifestations such as sepsis-like illness, gastric bleeding, circulatory failure, and death. Neurological manifestations include intracerebral hemorrhages, neurological malformations, and acute focal/disseminated encephalitis/encephalomyelitis. Dengue NS1Ag, a highly conserved glycoprotein, can help the detection of cases in the viremic stage. We do not have proven specific therapies yet; management is largely supportive and is focused on close monitoring and maintaining adequate intravascular volume.

Respiratory Syncytial Virus Infections in Neonates: A Persisting Problem.

Respiratory syncytial virus (RSV) is the most common cause of lower respiratory tract infections in young infants. It is an enveloped, single-stranded, nonsegmented, negative-strand RNA virus, a member of the family Pneumoviridae. Globally, RSV is responsible for 2.3% of deaths among neonates 0-27 days of age. Respiratory syncytial virus infection is most common in children aged below 24 months. Neonates present with cough and fever. Respiratory syncytial virus-associated wheezing is seen in 20% infants during the first year of life of which 2-3% require hospitalization. Reverse transcriptase polymerase chain reaction (RT-PCR) gives fast results and has higher sensitivity compared with culture and rapid antigen tests and are not affected by passively administered antibody to RSV. Therapy for RSV infection of the LRT is mainly supportive, and preventive measures like good hygiene and isolation are the mainstay of management. Standard precautions, hand hygiene, breastfeeding and contact isolation should be followed for RSV-infected newborns. Recent AAP guidelines do not recommend pavilizumab prophylaxis for preterm infants born at 29-35 weeks without chronic lung disease, hemodynamically significant congenital heart disease and coexisting conditions. RSV can lead to long-term sequelae such as wheezing and asthma, associated with increased healthcare costs and reduced quality of life.

CMV-induced Hearing Loss.

Congenital cytomegalovirus (cCMV) infection is the most common fetal viral infection and contributes to about 25% of childhood hearing loss by the age of 4 years. It is the leading nongenetic cause of sensorineural hearing loss (SNHL). Infants born to seroimmune mothers are not completely protected from SNHL, although the severity of their hearing loss may be milder than that seen in those whose mothers had a primary infection. Both direct cytopathic effects and localized inflammatory responses contribute to the pathogenesis of cytomegalovirus (CMV)-induced hearing loss. Hearing loss may be delayed onset, progressive or fluctuating in nature, and therefore, a significant proportion will be missed by universal newborn hearing screening (NHS) and warrants close monitoring of hearing function at least until 5-6 years of age. A multidisciplinary approach is required for the management of hearing loss. These children may need assistive hearing devices or cochlear implantation depending on the severity of their hearing loss. In addition, early intervention services such as speech or occupational therapy could help better communication, language, and social skill outcomes. Preventive measures to decrease intrauterine CMV transmission that have been evaluated include personal protective measures, passive immunoprophylaxis and valacyclovir treatment during pregnancy in mothers with primary CMV infection. Several vaccine candidates are currently in testing and one candidate vaccine in phase 3 trials. Until a CMV vaccine becomes available, behavioral and educational interventions may be the most effective strategy to prevent maternal CMV infection.

Congenital and Perinatal Varicella Infections.

Varicella-zoster virus (VZV) is a human pathogen of the α-herpesvirus family. Some fetuses infected in utero around 8-20 weeks of pregnancy show signs of congenital varicella syndrome (CVS). Infants born to mothers who develop varicella within 5 days before and 2 days after delivery can experience severe disease with increased mortality. The best diagnostic modality is polymerase chain reaction (PCR), which can be done using vesicular swabs or scrapings, scabs from crusted lesions, tissue from biopsy samples, and cerebrospinal fluid. The prevention and management of varicella infections include vaccination, anti-VZV immunoglobulin, and specific antiviral drugs. In this article, we have reviewed the characteristics of VZV, clinical manifestations, management of perinatal infections, and short- and long-term prognosis.

Enteroviral Infections in Infants.

Enteroviruses (EVs) are major pathogens in young infants. These viruses were traditionally classified into the following four subgenera: polio, coxsackie A and B, and echoviruses. Now that poliomyelitis seems to be controlled in most parts of the world, coxsackie and echoviruses are gaining more attention because (i) the structural and pathophysiological similarities and (ii) the consequent possibilities in translational medicine. Enteroviruses are transmitted mainly by oral and fecal-oral routes; the clinical manifestations include a viral prodrome including fever, feeding intolerance, and lethargy, which may be followed by exanthema; aseptic meningitis and encephalitis; pleurodynia; myopericarditis; and multi-system organ failure. Laboratory diagnosis is largely based on reverse transcriptase-polymerase chain reaction, cell culture, and serology. Prevention and treatment can be achieved using vaccination, and administration of immunoglobulins and antiviral drugs. In this article, we have reviewed the properties of these viruses, their clinical manifestations, and currently available methods of detection, treatment, and prognosis.

Post-INSURE Administration of Heated Humidified High-Flow Therapy Versus Nasal Continuous Positive Airway Pressure in Preterm Infants More Than 28 Weeks Gestation with Respiratory Distress Syndrome: A Randomized Non-Inferiority Trial.

In preterm infants with respiratory distress syndrome (RDS), non-invasive ventilation (NIV) is usually provided using nasal continuous positive airway pressure (NCPAP) or non-invasive mechanical ventilation after surfactant administration by INtubation-SURfactant-Extubation (INSURE) method. Heated humidified high-flow nasal cannula (HHHFNC) is a mode of NIV with advantages of ease of application, less grades of nasal injury and easy handling. This study was done to compare the effectiveness of HHHFNC therapy administration as post-INSURE respiratory support in preterm infants as compared to NCPAP. The primary outcome was to compare the rate of treatment failure within 7 days of randomization to HHHFNC or NCPAP as a post-INSURE ventilatory modality. It was a pilot trial wherein all preterm infants more than 1 kg and more than 28 weeks gestational age with RDS who required surfactant were randomized to receive NCPAP or HHHFNC. Infants with an urgent need for intubation and mechanical ventilation were considered to have treatment failure. Thirty babies were enrolled-15 in each group. Baseline demographic characteristics were comparable. There was no significant difference in the primary outcome of early failure rate, i.e. mechanical ventilation rate within 7 days of starting treatment. There were no significant differences in other outcomes except nasal injury which was significantly lesser in the HHHFNC group. In conclusion, HHHFNC appears to be non-inferior to NCPAP when used in preterm infants more than 28 weeks gestation with RDS as a post-INSURE ventilatory modality.

Infection associated relapses in children with nephrotic syndrome: A short-term outcome study.

Children with nephrotic syndrome (NS) encounter multiple episodes of relapses associated/triggered by an episode of infection. The primary objective of this study was to find the proportion of infection associated relapses that resolve on the treatment of acute infection over an observation period of two weeks in children with NS. This prospective observational study enrolled 45 children with steroid-sensitive NS presenting with an infection associated relapse during the study period (February 2015 to February 2016). Baseline information and examination findings of all children were recorded. Biochemical and other investigations were performed according to the site of infection for all patients and were treated appropriately. None of the patients received daily 2 mg/kg of prednisolone during the observation period. All children were followed for two weeks for resolution of relapse and subsequently every month for another three months. The 45 patients (median age 66 months) enrolled in the study had 64 episodes of infections, of which upper respiratory tract infections (45%) were the commonest, followed by peritonitis (18.5%) and diarrhea in 12%. Twenty-seven (60%) patients achieved remission on symptomatic treatment of infection with/without the use of stress doses of prednisolone. Most (77.8%) patients who achieved remission without the use of daily 2 mg/kg of prednisolone did so within the 1st week and a majority of patients were still in remission at three months follow-up. We conclude that most infection associated relapses can be managed with treatment of underlying infection alone and use of stress doses of steroids for inducing remission without increasing the prednisolone doses to 2 mg/kg/d and thus reducing the cumulative steroid doses.