Childhood onset SLE- case report

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that can affect any organ and may result in significant morbidity and mortality. Childhood-onset c-SLE is a rare disease with an incidence of 0.3-0.9/100,000 children-years and a prevalence of 3.3-8.8/100,000 children.1 Asians, African American, Hispanic and Native Americans have higher frequency of the disease.2,3 Similar to adult SLE, c-SLE affects mainly females with around 80% of the affected children. According to the literature, the median age of onset is 11-12 years and it is rare in children under 5 years of age. The manifestations of c-SLE are variable and most of the times are atypical compared to adult SLE which make the diagnosis of cSLE a challenge to the treating physician.4 This report shows a series of presentation in pediatric patient who finally was diagnosed with SLE.

Prevalence of hearing impairment and outcome of universal neonatal hearing screening program in a tertiary care hospital – in UAE

Background and objective: Hearing loss is one of the most common congenital anomalies, occurring in 1 to 3 per 1000 newborn infants in the well-baby nursery population, and 2 to 4 per 100 infants in the Neonatal intensive care unit graduates. The aim of the study is to identify babies with potential hearing loss and direct them to early intervention and rehabilitation. The program also initiated to create awareness about the need for detecting childhood deafness among parents and general population. An estimate of coverage, prevalence and proportion of babies defaulting newborn hearing screening program and diagnostic evaluation monitored closely to find the effectiveness of the program. Study design: This is a retrospective study and was conducted at Latifa women and children’s hospital (LWCH), a tertiary care referral hospital at Dubai, United Arab Emirates, over a period of two years from January 2018 to December 2019. Neonates admitted in Neonatal intensive care unit (NICU) and Post Natal wards (PNW) were screened. We followed three tier approach of hearing screening. All eligible neonates were subjected to Transient Evoked Oto-acoustic emission (TEOAE) prior to discharge. Automated auditory brainstem response (AABR) hearing screening was conducted in neonates who did not pass TEOAE test. Infant who failed second screening (AABR) were subjected to comprehensive hearing evaluation prior to three months of age. Results: Out of the total 7923 neonates, 7895(99.65%) were screened and 118 (1.49%) newborns referred for comprehensive audiological evaluation. Among them, 27(0.34%) were identified with various degrees of hearing loss. The prevalence of hearing loss was found to be 0.16% and 0.92% among neonates from PNW and NICU respectively. Conclusion: Implementation of Universal newborn hearing screening program is the need of the hour, as early detection of Hearing loss will aid early rehabilitation and better outcomes.

Persistent air leak (bronchopleural fistula) in neonates

Background: Pneumothorax is a known complication in neonates on ventilation but persistent air leak is infrequently seen in neonates and is troublesome to manage. Persistent air leak on chest tube insertion is suggestive of bronchopleural fistula, often resulting in significant mortality and morbidity. Various modalities of management like multiple chest tube insertions (thoracostomies), selective bronchial occlusion, pleurodesis using talc, tetracycline etc and urgent surgery to repair the leak have been mentioned. Islolated case reports for management of bronchopleural fistula in neonates are abound in literature but consensus and attention should be directed to improve awareness and access to clinical guidelines in management of bronchopleural fistula in neonates. Methods: Our aim was to evaluate the management and outcome of neonates with persistent air leak (bronchopleural fistula). Result: Eighteen neonates with bronchopleural fistula (BPF) were managed from 2012-2018. All neonates were managed by chest tube insertion, and slow suction (10-15cm of H2O). In those patients having persistent pneumothorax despite functioning tube with persistent air leak, second chest tube was inserted in 2nd intercostal space. Five of these patients even had cardiac arrest due to tension pneumothorax but were revived. One neonate required pleurodesis, two expired and remaining improved on multiple chest tube insertion and were discharged. None required selective bronchial intubation or surgery. Conclusion: Tension pneumothorax with persistent air leak on chest tube suggestive of Broncho-pleural fistula is a difficult and a rare problem in neonates. If not timely taken care of it can lead to cardiac arrest but despite cardiac arrest aggressive resuscitation and judicious use of multiple chest tube drainage and slow suction can help these little patients improve.

Neonatal Tuberous Sclerosis: is skin really the window?

We report a case of a neonate who was diagnosed to have intracardiac mass as a fetus and presented with white linear papules that was diagnosed to be Linear Verrucous Epidermal Nevus. Apart from the intracardiac mass, most likely rhabdomyoma, MRI Brain also showed presence of tubers and the Next Generation Sequence Analysis confirmed the diagnosis of Neonatal Tuberous Sclerosis. The neonate remained asymptomatic and was discharged home and remains under close follow up without any symptoms. It is thus suggested that Linear Verrucous Epidermal Nevus, a cutaneous manifestation not described with Tuberous Sclerosis could be considered under the ever-expanding cutaneous signs of Tuberous Sclerosis and should alert the physician toward its possibility.

Outcomes of intrauterine interventions for the treatment in fetal urinary tract obstructions

Introduction: The leading cause of chronic renal failure in newborns, which occurs soon after birth is obstructive uropathies. Objectives: To prove the effectiveness of nephroamniotic shunting based on the evaluation of perinatal outcomes of this procedure, as well as a comparative analysis of the use of the stent manufactured by "Cook" (Ireland), 3.0 Fr/100 mm and the stent “SDE–MED”, 3.0 Fr/50 mm with the original shape of pigtails. Methods: After checking the safety of the stent “SDE-MED”, 3.0 Fr/50 mm developed by FGBU Mother and Child Care Research Institute with the original shape of pigtails in an animal experiment, the stent was used in clinical practice for intrauterine shunting in cases of unilateral or bilateral hydronephrosis of grade III to IV and posterior urethral valve disorder. In the final part of the study, a comparison of the outcomes of nephroamniotic shunting using two different stents was made. Results: The “SDE–MED” 3.0 Fr/50 mm stent provided more effective fixation in the fetal kidney cavity system in comparison to the stent manufactured by “Cook” (Ireland) 3.0 Fr/100 mm. Conclusion: Intrauterine shunting surgery of the upper urinary tract is a pathogenetically justified method of treating urinary tract obstructions and can be used to correct all types of fetal renal obstructions.

Clinical profile of fungal sepsis in new born: a tertiary centre experience from Bangladesh

Background: Candida infections are frequent and major causes of septicemia in neonatal intensive care units and are associated with high morbidity and mortality. Low birth weight preterm infants are especially vulnerable to these devastating infections. Material and methods: A prospective observational study was done from May 2013 to June 2014 in SCABU (Special Care Baby Unit) & ICU (Intensive Care Unit) of Dhaka Shishu (Children) Hospital, Dhaka. All neonates admitted with suspected clinical sepsis were analyzed in the study. Among which 30 culture positive candida cases were identified and included in this study. Outcome measures assessed was the incidence of candidemia in our NICU with clinical profiles and associated risk factors. Results: Out of 30 newborns 18 (60%) were preterms, 20% of study population were having a birth weight of <1 kg, 23.3% with a birth weight of 1-1.5 kg and 23.3% with a birth weight of 1.5-2.5 kg. 73.4% of the study population were hospitalized for >1 week. In the study group 56.7% had feed intolerance, 53.3% needed ventilator support, 56.7% had temperature instability, 73.3% had thrombocytopenia 63.3% had apnea and 73.3% had jaundice. The overall survival was 63.3%. Conclusions Low birth weight, prematurity, use of broad spectrum antibiotics, mechanical ventilation and prolonged hospital stay were important risk factors associated with neonatal candidiasis in this study. Thrombocytopenia, feed intolerance, increased requirement for ventilator support, temperature instability, jaundice and apnea were significant clinical parameters noted in babies with culture proven neonatal candidiasis. The overall survival was 63.3% in the study group.

Foreign body aspiration in an 11-month-old child; the importance of anamnesis and respiratory examination

Foreign body aspiration (FBA) is common in children. Especially in children, the majority of accidental deaths occur due to FBA. Morbidity and mortality rates increase, especially in children between the ages of one and four, and as a result of delay in diagnosis. The most common symptoms in patients with FBA are cough, dyspnea, hemoptysis, stridor and wheezing. In this case report, a patient who presented with sudden respiratory distress in the Pediatric Emergency Department was presented and the importance of anamnesis and respiratory examination in the diagnosis of FBA was emphasized.