Acquired Cytomegalovirus Retinitis in Preterm Infant Hospitalized in the NICU: A Noteworthy Case Report.

BACKGROUND: Acquired human cytomegalovirus (CMV) is a noteworthy disease in infants. This case study will highlight the influence of early diagnosis of CMV retinitis (CMVR) on avoid visual impairment. CLINICAL FINDINGS: We describe a preterm female infant with a birth weight of 2060 gr that was admitted for tracheostomy placement due to hypoxic-ischemic encephalopathy. There were no signs of CMV infection or sepsis in laboratory results upon admission such as serology (IgG, IgM antibodies), Toxoplasma gondii , Rubella virus, Herpes simplex virus, CMVR and urine polymerase chain reaction (PCR). PRIMARY DIAGNOSIS: Incidentally, upon screening for retinopathy of prematurity, diffuse occlusive vasculitis was detected in the retinal image on the 112th day of life. INTERVENTION: Intravenous and intraocular ganciclovir were administered for 4 weeks. OUTCOMES: In the follow-up visit 6 weeks after discharge from the hospital, visual impairment was detected on both sides. PRACTICE RECOMMENDATIONS: This is a report of a case of acquired CMVR, a silent finding, as an uncommon complication in preterm neonates during the hospital stay. This diagnosis should be taken into consideration in preterm infants, since early diagnosis and treatment are crucial to avoid visual impairment.

Clinical Course and Outcome in Children with Congenital Hyperinsulinism.

BACKGROUND: Hyperinsulinism is the most common cause of persistent or recurrent neonatal hypoglycemia that may result in neurological deficits. The treatment goal in these patients is prevention of hypoglycemia to decrease mortality and morbidity. This study was done to determine the clinical course and outcome in children with congenital hyperinsulinism (CHI) referring to Mofid Children's Hospital from 2011 to 2017. METHODS: This study was done on 22 children with CHI referring to Mofid Children's Hospital from 2011 to 2017. The demographic, perinatal, clinical, laboratory, imaging, pharmacological, treatment and follow up data of these children were collected and analyzed. RESULTS: Among 22 children with CHI, the mortality rate was higher among those who received hydrocortisone versus those who did not receive hydrocortisone (46% versus 40%). CONCLUSION: According to the results of this study, hydrocortisone had a negative impact on the outcomes of these children, which is important in the management of hypoglycemia. The clinical course and outcome of children with CHI was better with medical compared to surgical treatment.

Administration of breast milk cell fractions to neonates with birthweight equal to or less than 1800 g: a randomized controlled trial.

BACKGROUND: Most premature and very low birthweight infants cannot tolerate breast milk feeding in the first few days of life and are deprived of its benefits. This study evaluates the clinical outcomes of administering breast milk cell fractions to neonates with a birthweight of ≤1800 g. METHODS: We conducted a randomized controlled trial on 156 infants in the neonatal intensive care unit of Mahdieh Maternity Hospital in Tehran, Iran, from May 2019 to April 2020. All neonates with a birthweight ≤1800 g were enrolled and divided into intervention and control groups using stratified block randomization. Neonates in the intervention group received the extracted breast milk cell fractions (BMCFs) of their own mother's milk after being centrifuged in the first 6 to 12 h after birth. The control group received routine care, and breastfeeding was started as soon as tolerated in both groups. Study outcomes were necrotizing enterocolitis (NEC), death, and in-hospital complications. RESULTS: We divided participants into two groups: 75 neonates in the intervention group and 81 neonates in the control group. The mean birthweight of neonates was 1390.1 ± 314.4 g, and 19 (12.2%) neonates deceased during their in-hospital stay. The incidence of NEC was similar in both groups. After adjustment for possible confounders in the multivariable model, receiving BMCFs were independently associated with lower in-hospital mortality (5 [26.3%] vs. 70 (51.1%]; odds ratio (OR): 0.24; 95% confidence interval [CI] 0.07, 0.86). Also, in a subgroup analysis of neonates with birthweight less than 1500 g, in-hospital mortality was significantly lower in the intervention group (4 [9.5%] vs. 13 [30.2%]; OR: 0.24; 95% CI 0.07, 0.82). There were no differences in major complications such as bronchopulmonary dysplasia and retinopathy of prematurity between the two groups. No adverse effects occurred. CONCLUSIONS: Our research demonstrated a significantly lower mortality rate in neonates (with a birthweight of ≤1800 g) who received breast milk cell fractions on the first day of life. Since this is a novel method with minimal intervention, we are looking forward to developing and evaluating this method in larger studies. TRIAL REGISTRATION: IIranian Registry of Clinical Trials. Registered 25 May 2019, IRCT20190228042868N1 .

Predictors of Success Rate in Different Initial Respiratory Supports in Very Low Birthweight Infants with Respiratory Distress.

BACKGROUND: Ideal respiratory support for very low birth weight infants (VLBW) can be selected based on demographic and clinical status at birth. METHODS: In this prospective cohort study, we included 163 VLBW neonates treated with either invasive or non-invasive respiratory support in their first 72 hours of life in the neonatal intensive care unit of Mahdiyeh hospital, Tehran, Iran. We used descriptive statistics to describe the data, and multiple logistic regression to determine the factors associated with the success rate of different strategies and the choice of strategy for primary respiratory support. All analyses were done using SPSS version 20 and STATA version 12 at a significance level of 0.05. RESULTS: The success rates of initial respiratory supports with nasal continuous positive airway pressure (NCPAP), noninvasive positive pressure ventilation (NIPPV), and INSURE (intubation surfactant extubation) were 63.20%, 42.10% and 61.90%, respectively. The results of multiple logistic regression analysis showed patent arterial duct (PDA) (yes vs. no: OR = 0.42) had a significant effect on initial respiratory support success (P<0.05). Also, gestational age (>28 vs. ≤28 weeks: OR = 0.26) and 5-min APGAR (≤6 vs. >6: OR = 9.69) had a significant effect on the choice of initial respiratory support in VLBW infants (P<0.05). CONCLUSION: The neonatal clinical condition may be a predictor of success for initial respiratory support at birth. Since the arterial duct may be open during the first hours of life, more study is needed to verify if early closure of the arterial duct may help increase the success rate of non-invasive respiratory support.

Bilirubin Induced Encephalopathy.

Hyperbilirubinemia is one of the most common neonatal disorders. Delayed diagnosis and treatment of the pathologic and progressive indirect hyperbilirubinemia lead to neurological deficits, defined as bilirubin induced encephalopathy (BIE) (2). The incidence of this disorder in underdeveloped countries is much more than developed areas. All neonates with the risk factors for increased the blood level of indirect bilirubin are at risk for BIE, especially preterm neonates which are prone to low bilirubin kernicterus . BIE can be transient and acute (with early, intermediate and advanced phases)or be permanent, chronic and lifelong ( with tetrad of symptoms including visual (upward gaze palsy), auditory (sensory neural hearing loss), dental dysplasia abnormalities, and extrapyramidal disturbances (choreoathetosis cerebral palsy).Beside the abnormal neurologic manifestations of the jaundiced neonates ,brain MRI is the best imaging modality for the confirmation of the diagnosis. Although early treatment of extreme hyperbilirubinemia by phototherapy and exchange transfusion can prevent the BIE, unfortunately the chronic bilirubin encephalopathy does not have definitive treatment.

Neonatal neuroblastoma presented with respiratory distress, a case report.

BACKGROUND: The most common neonatal tumor is neuroblastoma and adrenal gland is the most common site of involvement. 11-26% of this tumor is seen in the thorax of neonates. Due to a similar appearance of the mediastinal tumor with pneumonia, a high index of suspicion is necessary for early diagnosis of this disorder. CASE REPORT: A 17-day -old female and term neonate delivered by caesarian section was reported in the study. The mother had a normal pregnancy without any reported complications. The newborn admitted to a neonatal intensive care unit (NICU) in her hometown, because of respiratory distress, stridor, and tachypnea which was started from 7 days of life and due to lack of recovery and persistent respiratory symptoms, the patient referred to our hospital. In chest X-ray, opacity in the right upper lobe of the lung was seen. In barium study, a mass like lesion in the size of 35.34 mm adjacent to upper intra-thoracic esophagus with mild mass effect was observed and in CTS a posterior mediastinal mass with severe compression on the trachea and extension to the spinal canal was reported. Due to severe obstruction on the airway, the baby underwent emergency surgery and a mediastinal mass with adhesion and involvement of the ribs and spinal cord was resected. The final diagnosis of poorly differentiated neuroblastoma was confirmed by biopsy. CONCLUSION: In every neonate with persistent respiratory distress, stridor and abnormal chest X-ray, diagnosis of neonatal neuroblastoma should be considered.

Incontinentia Pigmenti Misdiagnosed as Neonatal Herpes Simplex Virus Infection.

Incontinentia pigmenti (IP) is an X-linked dominant neurocutaneous syndrome with ophthalmologic, neurologic, cutaneous, and dental manifestations and in most cases antenatally lethal in boys. Occasionally, typical IP may occur in boys due to Klinefelter syndrome or a genomic mosaicism. Skin lesions are observed in 4 stages: blistering, verrucous linear plaques, swirling macular hyperpigmentation, followed by linear hypopigmentation that develop during adolescence and early adulthood. Neonatal herpes simplex virus (HSV) infection can be manifested in 3 forms: localized, disseminated, and central nervous system (CNS) involvement. Timely diagnosis and treatment of neonatal HSV infection is critical. In this case report, we present a 12-day female newborn with a history of maternal genital HSV in second trimester and vesicular lesions on the upper and lower limbs that was appeared at first hours of life. She was admitted in the maternity hospital that was born and was treated by antibiotic and acyclovir for 11 days. Then, she readmitted for her distributed vesicular lesions. The results of blood and CSF for HSV PCR were negative. Eventually the diagnosis for incontinentia pigmenti was made by consultation with a dermatologist, and skin biopsy confirmed the diagnosis.

Causes and Factors Associated with Neonatal Seizure and its Short-term Outcome: A Retrospective Prognostic Cohort Study.

OBJECTIVE: Neonatal seizures are common, difficult to diagnose and treat, and associated with a great mortality rate and long-term risk of neurodevelopmental impairments. We aimed to determine the etiology, clinical presentation, and neurodevelopmental outcome of neonatal seizures. MATERIALS AND METHODS: In this cross-sectional study, 88 neonates, aged < 28 days, admitted to Mofid Children's Hospital, Tehran, Iran, from September 2011 to 2013 with the initial diagnosis of seizure were enrolled by convenient sampling method. Data, including age, gestational age, birth weight, Apgar of the fifth minute, neonatal intensive care unit (NICU) admission, family history, type, cause, and age of seizure, EEG and paraclinic findings, anticonvulsant used for treatment, neurodevelopmental status, and the final outcome, were collected from medical records and the missed cases were completed by phone calls. The frequency and association of variables were analyzed using SPSS software. RESULTS: Among neonates with seizures, 67% were male, 79.5% were born term, and 72.7% had normal birth weight. The most common type of seizure was multifocal clonic seizures (45.5%). The main diagnosis in neonates with seizures was hypoxic-ischemic encephalopathy (HIE) (23.9%) and hypoglycemia (10.22%). The mortality rate was 11.36% during a mean follow-up period of 21.4±6.4 months. Neurodevelopmental assessments showed that 64% were normal, 27% had global delay, and 9% had motor delay. Positive family history of epilepsy (P=0.006), low Apgar score (P=0.002) and epilepsy (P<0.001) were significantly associated with adverse neurodevelopmental outcome. CONCLUSION: Since HIE and hypoglycemia were the most common cause of neonatal seizures, efforts should be made to improve care during delivery and early breastfeeding.

Utilization of SNAP II and SNAPPE II Scores for Predicting the Mortality Rate Among a Cohort of Iranian Newborns.

BACKGROUND: The present study was conducted to determine the utility of Score for Neonatal Acute Physiology II (SNAP II) and Score for Neonatal Acute Physiology with Perinatal Extension II (SNAPPE II) scoring systems as predictors of neonatal mortality rate, and to compare the predictive value of these two methods. METHODS: In this prospective study data were gathered from infants admitted to the neonatal intensive care unit (NICU) of Imam Hossein Medical Center, Tehran, Iran, from March 2015 to December 2015. In addition to demographic data, Apgar score at 5 minutes after birth, initial and final diagnosis, SNAP II, and SNAPPE II were recorded within 24 hours after admission to the NICU. RESULTS: One hundred ninety-one newborn infants entered into the study. Birth weight (2555 ± 722 g in survival group versus 1588 ± 860 g in expired group, P<0.001), and Apgar score more than 7 at 5 minutes after birth (99.4% in survival group versus 57.1% in expired group, P<0.001) were significantly related to the mortality rate. By analyzing the data using logistic regression, it was found that SNAP II (area under the curve [AUC] = 0.992; 95% CI: 0.98-1) and SNAPPE II (AUC = 0.994; 95% CI: 0.984-1)had better value for predicting the patients' survival compared to Apgar score at 5 minutes after birth (AUC = 0.711; 95% CI: 0.568-0.855). There was no statistically significant difference in predictive value of SNAP II and SNAPPE II methods (P>0.99). CONCLUSION: According to our findings SNAP II and SNAPPE II are useful tools in predicting the mortality rate among Iranian neonates admitted to NICU. Although there was no significant difference between SNAP II and SNAPPE II, both methods had a much better predictive value compared to Apgar score at 5 minutes after birth.

Frequency and Causes of Hypotonia in Neonatal Period with the Gestational Age of More Than 36 Weeks in NICU of Mofid Children Hospital, Tehran, Iran During 2012-2014.

OBJECTIVE: Hypotonia is a serious neurologic problem in neonatal period. Although hypotonia is a nonspecific clinical finding but it is the most common motor disorder in the newborn. The objective of this study was to determine the frequency of neonatal hypotonia then to ascertain of the most common causes. MATERIALS & METHODS: This cross -sectional prospective study was carried out on the 3281 term infants hospitalized in conventional and NICU of Mofid Children Hospital, Tehran, Iran during 2012-2014. Diagnosis was made by history, physical & neurological examination and accessible diagnostic tests. RESULTS: Fifty nine hypotonic neonates were identified, forty seven (79.66%) had central hypotonia (Hypoxic ischemic encephalopathy (n= 2), other causes of encephalopathy (n=2), intracranial hemorrhage (n=4), CNS abnormalities (n= 7), chromosomal disorders (n=4), syndromic-nonsyndromic (n=8), and metabolic diseases (n=8). Peripheral hypotonic recognized in 6 infants (10.17%); spinal muscular atrophy (n= 1), and myopathy (n= 5). Six cases (10.17%) remained unclassified. Twelve infants had transient hypotonia. In final study, 18 of 59 infants (30%) died, nearly 90% before one year of age. Twenty-eight (47%) infants found developmental disorders and only 13 (22%) infants achieved normal development in their follow up. CONCLUSION: Neonatal hypotonia is a common event in neonatal period. A majority of diagnosis is obtained by history and physical examination. Neuroimaging, genetic and metabolic tests were also important in diagnosis. Genetic, syndromic-nonsyndromic, and metabolic disorders were the most causes of neonatal hypotonia.

Congenital Pleomorphic Adenoma in a SubmandibularGland of a Newborn- A Case Report.

INTRODUCTION: Pleomorphic adenoma is a rare benign salivary gland neoplasm in children, which can be treated by simple excision. This tumor is rarely included in the differential diagnosis of solid submandibular masses in children. In the neonates, congenital pleomorphic adenoma usually presents in the nasopharynx. Surgical excision is the treatment of choice and recurrence is not expected. We report what appears to be the first case of congenital pleomorphic adenoma in the submandibular region in a one-day-old newborn. CASE REPORT: The case of a one-day-old term baby is presented with a 5x2 cm left submandibualr mass with extension to the oral cavity. The mass was hard and non-mobile. During Ultrasonography and Contrast-enhanced Computed Tomography (CT) scan, the mass was solid with a heterogeneous internal structure. The tumor was completely excised and proved to be a pleomorphic adenoma during histopathological examination. CONCLUSION: Congenital pleomorphic adenoma rarely occurs in the nasopharynx and is treated by surgical excision. Our case is unique because the congenital pleomorphic adenoma is located in the submandibular gland of a newborn.

Pre-Discharge Screening Trans-Cutaneous Bilirubinometry in Healthy Newborns in Mahdieh Hospital, Tehran.

BACKGROUND: Incidence of jaundice is high in newborn infants. Since well appearing newborns are rapidly and routinely discharged from hospital, performing an inexpensive noninvasive pre-discharge screening test for evaluation of jaundice seems to be necessary. OBJECTIVES: This study was conducted to compare the accuracy of cutaneous v/s serum bilirubin measurements in this regard. PATIENTS AND METHODS: This was a prospective cross sectional study conducted in Mahdieh hospital, Tehran. 613 neonates weighing ≥ 1,800 g with gestational age of ≥ 35 weeks were enrolled. A pre discharge transcutaneous bilirubin test (TcB) was performed in all. Serum samples were taken from neonates with TcB ≥ 5 mg/dL in first and > 8 mg/dL in second 24 hours. Decision for treatment or recheck of bilirubin level after discharge was made based on serum bilirubin results. RESULTS: Based on the study protocol, among 613 studied neonates, 491 (80%) revealed high TcB, of them 240 (49%) cases showed TBC ≥ 5 mg/dL in first and 251 (51 %) in second pre-discharge 24 hours. TcB ranged 3.3 - 17.1, mean TcB in first 24 hours was 6.9 ± 1 .7 (mode 6) and in second 24 hours 9.1 ± 2.1 (mode 10). Of 491 neonates with high TcB, capillary serum sample was taken as the second step and 398 neonates revealed high total serum bilirubin (TsB) with the same protocol for TcB. 108 (27.1%) neonates showed TsB ≥ 5 mg/dL in first and 290 (72.9%) in second 24 hours. According to the study results TcB has a 81% positive predictive value (PPV) in diagnosis of hyperbilirubinemia. Correlation coefficient of TcB and TsB in highest rate is equal to 72% (P value < 0.001). CONCLUSIONS: TcB is an inexpensive, noninvasive and precise pre-discharge screening test for evaluation of hyperbilirubinemia, with a high PPV. It is highly recommended to be performed routinely due to high incidence of hyperbilirubinemia in neonates.

Outcome of very low birth weight infants over 3 years report from an Iranian center.

OBJECTIVE: Very low birth weight (VLBW) infants are at high risk for morbidity and mortality. This article determines the frequency of disease, rate od survival, complications and risk factors for morbidity and mortality in VLBW neonates admitted to a level III neonatal intensive care unit (NICU) at Mahdieh Hospital in Tehran. METHODS: This cross-sectional retrospective study was performed from April 2007 to March 2010 on all hospitalized VLBW neonates. Relevant pre- and peri-natal data up to the time of discharge from the hospital or death, including complications during the course of hospitalization, were collected from the case notes, documented on a pre-designed questionnaire and analyzed. FINDINGS: Out of 13197 neonates, 564 (4.3%) were VLBW with 51.4% males. Mean gestational age was 29.6±2.5 weeks; mean birth weight 1179±257 grams. Mean birth weight, gestational age and Apgar scores were significantly higher in babies who survived than in those who died, (1275±189 vs. 944±253 grams; 30.5±2.2 vs. 27.5±2 weeks and 6.9±1.7 vs. 5±2.1 respectively, P<0.001 in all instances). Overall survival was 70.9%; in extremely low birth weight (ELBW) newborns this figure was 33.3% rising to 84.1% in infants weighing between 1001-1500 grams. Respiratory failure resulting from RDS in ELBW babies was the major factor leading to death. Need for mechanical ventilation, pulmonary hemorrhage and gastro-intestinal bleeding were also significant predictive factors for mortality. CONCLUSION: Birth weight and mechanical ventilation are the major factors predicting VLBW survival.