فهرست مطالب

Neonatology - Volume:10 Issue:2, 2019
  • Volume:10 Issue:2, 2019
  • تاریخ انتشار: 1398/03/11
  • تعداد عناوین: 16
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  • Tisha Ann Skariah *, Leslie Edward Lewis Pages 1-8
    Background
    Early nasal continuous positive airway pressure (NCPAP) has emerged as a primary modality of respiratory support for preterm infants withrespiratory distress syndrome (RDS). However, 30%-40% of these newborns need subsequent mechanical ventilation. Nasal intermittent positive pressure ventilation (NIPPV) is a promising alternative to NCPAP, especially in post-extubation settings, apnea of prematurity, or NCPAP failure as the primary mode of respiratory support in RDS. Application of these two methods in neonates with RDS needs further studies. 
    Methods
    This open-label randomized clinical trial (RCT) was stratified by gestational age (i.e., 28-32 and 33-36 weeks). The sample included 78 infants divided into the two groups of 37 NIPPV and 41 CPAP.  We compared the effect of ventilator delivered asynchronous NIPPV with NCPAP in reducing the need for invasive ventilation within 48 h of non-invasive support in infants of 28-36 weeks with RDS [onset of distress within ≤ 6 h of life with a fraction of inspired oxygen (FiO2) ≥ 0.25 compatible with chest radiograph]. The FiO2 > 0.3 and/or Downes score ≥ 4 were the indications for surfactant therapy administered by endotracheal tube. The infants were extubated and returned to their initial assigned mode of support within 60 min.  The primary outcome was considered as failure of the allocated mode within 48 h.
    Results
    According to our findings, the two groups showed no significant difference in terms of failure rates with 5 (13.5%) and 6 (15%) failed NIPPV and NCPAP cases (P=0.8). There was a trend toward less surfactant therapy in NIPPV [12 (32.4%) vs. 22 (53.7%), P=0.06], and lower Downes score in the first 12 h. The hazard ratio (HR; adjusted for gestation, surfactant therapy, and birth weight) for failure in NIPPV was similar to that of NCPAP (HR=1.03) at 95% confidence interval. No difference in air leaks or abdominal distension was noted between the two groups.
    Conclusion
    Early NIPPV may not have a benefit, compared to NCPAP as a primary mode of respiratory support for infants with RDS.
    Keywords: asynchronous, nasal continuous positive airway pressure, Nasal intermittent positive pressure ventilation, Non-invasive Ventilation, respiratory distress syndrome
  • Majid Mohammadizadeh, Zohreh Badiei, Roya Choopani *, Marzie Khalili Pages 9-16
    Background
    Continuous positive airway pressure (CPAP) is used as respiratory support in preterm neonates; however, the best weaning method has not yet been determined. In this study, we compared sudden complete and gradual weaning from nasal CPAP (NCPAP) in preterm newborns.
    Methods
    This randomized controlled trial was conducted on 62 preterm neonates who were born with a gestational week of < 32 weeks and required NCPAP for at least 24 h. The neonates were stable on NCPAP at 0.21 FiO2 and 5 cm H2O positive end-expiratory pressure. They were randomized into two groups of gradual and sudden weaning using random numbers sheet. The primary outcome was successful weaning at the first attempt. The secondary outcomes included the number of NCPAP weaning attempts, the need for mechanical ventilation (nasal and endotracheal), duration of NCPAP, oxygenation, and length of hospital stay.
    Results
    According to the results, 80.6% of the patients in the sudden weaning group and 74.2% of the patients in the gradual weaning group were weaned successfully in the first attempt. However, there was no statistically significant difference between the two groups in this regard (P=0.54). Duration of NCPAP was significantly lower in the sudden weaning group, compared to that in the gradual weaning group (P<0.001). Numbers of NCPAP weaning attempts, the need for mechanical ventilation, duration of oxygenation, and hospital stay in the two groups were not significantly different.
    Conclusion
    There was no difference between sudden complete and gradual weaning from NCPAP in terms of treatment success and complications. The selection of the appropriate technique may depend on available equipment and treatment costs.
    Keywords: Continuous positive airway pressure (CPAP), Gradual weaning, Preterm, Sudden weaning
  • Varun R Nayak *, Tisha Ann Skariah, Leslie Lewis Pages 17-21
    Background
    Carbon dioxide (CO2) is a by-product of cellular metabolism, which could be considered as a reflection of metabolism, circulation, and ventilation. Arterial blood gas analysis (ABG) is the gold standard of monitoring for CO2. However, is an expensive method leading to blood loss and iatrogenic anemia. In addition, each sample is only a snapshot view of the sampling moment. End-tidal CO2 (ETCO2) measurement gives a non-invasive and continuous monitoring of exhaled CO2. Therefore, this study aimed to validate the non-invasive CO2 measurement performed by nasal cannula and evaluate the correlation with partial pressure of arterial CO2 (PaCO2 ) in neonates.
    Methods
    This single-center observational study was conducted in the Neonatal Intensive Care Unit (NICU), Kasturba Hospital, Manipal, India. PaCO2 was reported on routine ABG within a 15-minute interval of ABG sampling. Moreover, partial pressure of end-tidal carbon dioxide (PetCO2) was noted at continuous 30-sec intervals (i.e., 30, 60, 90, ..., 180) up to 3 minutes. The values of PaCO2 and PetCO2 were found to be correlated with Pearson correlation and were shown by scattered plot. Regression analysis was used to get the prediction equation and the variance.
    Results
    A total of 70 samples were taken in the initial phase to study the correlation between PaCO2 and measured PetCO2. Pearson correlation showed a moderate positive correlation (r= 0.589) between PetCO2 and measured PaCO2. Regression analysis demonstrated a variance of 33.8% between the measured PetCO2 and PaCO2, which was statistically significant (P<0.001). A prediction equation was obtained for PaCO2. In the final phase, 20 samples were recruited to standardize and validate the prediction equation. The PaCO2 was calculated using the predicted equation and a new prediction equation was obtained.
    Conclusion
    According to the findings of this study, there is a good correlation (r=0.681) between the non-invasively measured PetCO2 and PaCO2.
    Keywords: correlation, ETCO2, Nasal cannula, Neonates, Non-invasive, PaCO2
  • Naiire Salmani *, Zahra Dehghan, Zahra Mandegari, Azita Aryaee, Shima Dehghan Pages 22-29
    Background
    The sudden exposure of premature newborns to a cold and dry environment can lead to hypothermia. This study aimed at comparing the effectiveness of two polyethylene covers on the body temperature and oxygen saturation of premature neonates.
    Methods
    This experimental study was conducted on 72 premature newborns in the neonatal intensive care unit (NICU) of Shahid Sadoughi Hospital in Yazd, Iran. The study subjects were randomly assigned into three groups, including the neonates with polyethylene head cap, polyethylene body bag, and routine care. The data were collected using a thermometer and a pulse oximeter. The data analysis was performed through descriptive statistics, Chi-square test, analysis of variance (ANOVA), repeated measures ANOVA, and Tukey test using SPSS (version 18) software.
    Results
    There was a significant difference in the mean body temperature of control and intervention groups immediately after their NICU admission and 1 h later. Other significant differences were found in the mean of oxygen saturation belonging to the control group and the hat group upon admission and 1 h later. Also, the mean scores of oxygen saturation belonging to the control group were compared with those of the bag group, and the differences were significantly different 1 h after the admission.
    Conclusion
    In the light of these findings, the application of both polyethylene head cap and body bag can maintain the body temperature and oxygen saturation in the premature neonates transferring to the NICU. In particular, the head cap seems to better improve than oxygen saturation.
    Keywords: Newborn, Oxygen saturation, Premature, Temperature, Transfer
  • Hamid Torabian, Saeed Alinejad, Akram Bayati, Fatemeh Rafiei, Sharareh Khosravi * Pages 30-36
    Background
    Positioning of premature newborns significantly affects their health status. However, the most suitable position remains controversial. The current study aimed to compare the effect of supine and prone positions on oxygen saturation and vital signs in premature newborns.
    Methods
    In this crossover clinical trial, a total of 22 newborns admitted to the Neonatal Intensive Care Unit (NICU) of Amir Kabir Hospital in Arak, Iran, were selected through purposive sampling technique, and then randomly assigned into groups 1 and 2. Newborns in group 1 were first placed in a prone position (i.e., the first period for 3 h), and then in a supine position (i.e., the second period for an additional 3 h). The reverse procedure was applied to the intervention group 2. Heart rate, respiratory rate, and oxygen saturation were measured and recorded every 15 min.
    Results
    The mean oxygen saturation in the prone position (96.164±0.148) was higher than in the supine position (90.479±0.513; P=0.0001). The mean heart rate in the prone position (138.24±1.87 beats/min) was lower than that in the supine position (147.48±1.597 beats/min; P=0.0001). The mean respiratory rate in the prone position (40.430±0.504 breaths/min) was lower than that in the supine position (46.773±0.685 breaths/min; P=0.0001).
    Conclusion
    The current study demonstrated that the prone position put the newborn admitted to NICU in a more stable condition. However, the selection of the best position must be made based on the newborn’s health status and situation.
    Keywords: Oxygen saturation, Position, Premature Newborn, Vital signs
  • Morteza Habibi *, Abolfazl Mahyar, Sanam Nikdehghan Pages 37-41
    Background
    The most common type of apnea among premature newborns is idiopathic apnea of prematurity. Recurrent apnea can lead to hypoxemia and central nervous system damage. Although caffeine and theophylline are the most commonly prescribed drugs in this field, there is disagreement about which medicine is preferred. The purpose of this study was to compare the therapeutic effect and side effects of caffeine and aminophylline in the treatment of idiopathic apnea of prematurity.
    Methods
    This randomized clinical trial study was conducted on 67 premature neonates with the definite diagnosis of idiopathic apnea of prematurity. In this regard, subjects were divided into two groups, namely aminophylline recipient group (n=31) and caffeine recipient group (n=36). The two groups were compared regarding the frequency of recurrent apnea and side effects of drugs.
    Results
    Regarding gender distribution, 15 (48.4%) patients in the aminophylline recipient group, and subjects in the 20 (55.6%) caffeine recipient group were male. After the treatment, apnea recurred only in one case (0.1%) of aminophylline recipient group. Moreover, 7 patients (58.3%) in the aminophylline recipient group and 5 cases (41%) in the caffeine recipient group suffered from gastrointestinal side effects (P=0.3).
    Conclusion
    The obtained results of the present study revealed that there was no significant difference between the two groups.
    Keywords: Aminophylline, Apnea, Caffeine, Prematurity
  • Gokul Krishna *, Tisha Ann Skariah, Leslie Lewis Edward Pages 42-46
    Background
    This study was targeted toward comparing volume-guaranteed (VG) ventilation with conventional pressure-controlled (PC) ventilation in preterm infants with respiratory distress syndrome (RDS) in terms of the facilitation of weaning and extubation and occurrence of complications, such as pneumothorax, intraventricular hemorrhage (IVH), retinopathy of prematurity (ROP), necrotizing enterocolitis (NEC).
    Methods
    This single-center randomized controlled trial was conducted on neonates who were mechanically ventilated in the Neonatal Intensive Care Unit of Kasturba Hospital Manipal Udupi, Karnataka, India. Infants with the gestational age (GA) of 27-34 weeks with RDS requiring mechanical ventilation in the first week of life were randomized to receive either SIMV-PC or SIMV-VG ventilation. Infants were stratified into two GA groups of 27-30 and 31-34 weeks. Sealed opaque envelope was used to randomize the infants into two treatment modalities. Sample size was calculated as 120 and 60 in each treatment group.
    Results
    A total of 115 neonates were enrolled. The mean GA and birth weight of the treatment groups were 31±2.3 weeks and 1230±374 g, respectively, and 70% of them received antenatal steroids. As the primary outcome variable, the total duration of ventilation was 8 h (range: 3-17) (median and IQR) in the SIMV-PC group and 6 h (range: 3-13) in the SIMV-VG group (P=0.366). Stratified analysis of neonates with the GA of > 31-34 weeks showed a significant difference between the VG and PC ventilation groups regarding the duration of ventilation.
    Conclusion
    There was a decrease in the duration of ventilation in VG ventilation, compared to that in PC ventilation at a higher GA. The leak was the major issue with VG ventilation in the lower GA group.
    Keywords: RDS, SIMV-PC, SIMV-VG
  • Shahin Mafinezhad *, Hasan Boskabadi, Ghasem Bayani, Hojatollah Ehteshammanesh Pages 47-49
    Background
    A fault in chromosome distribution during cell division leads to aneuploidy, which can be associated with thrombocytopenia. Various hematological abnormalities have been reported among neonates with Down syndrome (DS). Neutrophilia, thrombocytopenia and polycythemia were the most common hematological abnormalities observed among neonates with Down syndrome. In particular, thrombocytopenia below 150×109/L was found approximately in two-third of DS and 6% of counts below 50,000 was detected during the first week of life.The exact mechanism remains unknown, but is thought to be due to decreased platelet production from chronic fetal hypoxia. fetal hypoxia also leads to intrauterine growth retardation and suboptimal response of thrombopoitin system (TPO) to thrombocytopenia in DS during the neonatal period. Few cases of alloimmune thrombocytopenia with DS due to anti-HPA antibody were reported.
    Methods
    Data from multihospital healthcare systems shows large two case series of infants with cytogenetically confirmed DS and a reference group infants without birth defects all born during the period 2009-2015. During this period, 145,522 live births were recorded at 18 hospitals. Down syndrome was recognized in 226 newborn (1 in 644). Data were analyzed using multivariate logistic regression analysis expressed as adjusted odds ratio (aORs) with 95% confidence intervals (95% CIs).
    Results & Conclusion
    Infants with DS had a significantly higher risk for thrombocytopenia (aOR = 32.4). Platelet counts in DS averaged 104600 per microliter. The mean platelet volume did not correlate with the platelet count, but tended to run slightly large (9.2 +/- 1.3 fl). Persistence of thrombocytopenia beyond 8 to 12 weeks after birth should warrant a hematology consult. Thrombocytopenia can be seen associated with some types of congenital heart defects. Karyotype testing should be done in all obviously dysmorphic infants with thrombocytopenia. It seems reasonable to recommend that one or more CBCs be obtained on all neonates with Down syndrome.
    Keywords: Down syndrome, Thrombocytopenia, neonate, CBC
  • Elham Mobasheri, Marzieh Savarrakhsh, Seyyed Mohsen Hosseininejad, Ehsan Alaee * Pages 50-54
    Background
    Fetal asphyxia and the consequent neurological damages could be avoided if the risk factors are controlled at earlier stages. Umbilical cord arterial blood gas (UCABG) seems to be related to the Apgar scores, as well as the neonatal morbidity and mortality rates. Therefore, we designed this study to assess the correlation between the Apgar score and UCABG in Northeast of Iran.
    Methods
    This case-control study evaluated 438 term newborns and 62 preterm infants born during 2012-2013. Peripartum asphyxia, admission in the neonatal intensive care unit, and death were recorded as outcome measures. All the data were analyzed using the t-test and logistic regression analysis.
    Results
    Mild to moderate acidemia (pH=7-7.2) was reported in 9.6% and severe acidemia (PH<7) was observed in 1% of the participants. Multivariate analysis showed that aspiration of amniotic fluid, umbilical cord pH ≤ 7.2, umbilical cord base excess, and being preterm could be considered as the predictors of lower first-minute Apgar score.
    Conclusion
    Umbilical cord blood gas measurement is a useful tool to detect the neonates who are at higher risk of low Apgar.
    Keywords: Apgar score, Neonates, Umbilical cord blood gas
  • Mahbobeh Sajadi, Mehran Akbari *, Saeed Alinejad, Sharareh Khosravi Pages 55-60
    Background
    Nutritional problems in premature newborns are of great importance for their development. In this regard, the neonatal positioning during and after feeding can have a significant effect on their nutritional tolerance. Therefore, the present study was conducted to compare the effect of two positions (i.e., prone and right lateral) on gastric residual volume in preterm newborns after gavage feeding.
    Methods
    This crossover clinical trial was performed on 52 eligible premature newborns who were selected using purposive sampling. They were randomly assigned into two groups, namely A and B. After gavage feeding, the newborns in group A were placed in the prone position and their gastric residual volume was measured 3 times. Subsequently, after gavage feeding, they were put in the right lateral position and subjected to the same measurement. On the other hand, the newborns in group B were first put in the right lateral and then a prone position. The other procedures and measurements were performed the same as those in group A. The data were analyzed using repeated measure ANOVA.
    Results
    The results indicated a significant difference regarding the mean value of gastric residual volume (P=0.001). Moreover, the mean gastric residual volume in the prone position (2.152±0.299) was significantly (P=0.001) lower than that in the lateral position (2.618±0.403).
    Conclusion
    According to the results, The prone position can reduce the mean gastric residual. Therefore, it can be recommended as a suitable position after gavage feeding in premature newborns.
    Keywords: Gastric, Newborn, Premature, Prone position, Residual volume, Right lateral position
  • Mojgan Mirghafourvand *, Robabe Seyedi, Abdollah Jannat Dost, Sakineh Mohammad Alizadeh Charandabi, Mohammad Asghari Jafarabadi Pages 61-67
    Background
    Neonatal jaundice is the most common cause of newborns' hospitalization. This study aimed to examine various maternal, childbirth, and neonatal factors affecting the neonatal skin bilirubin level and severe jaundice.
    Methods
    This cross-sectional study was conducted on 1066 healthy neonates with a gestational age of 35 weeks or over and birth weight of ≥2000 g at teaching hospitals of Alzahra and Taleghani in Tabriz, Iran, during 2016-2017. The participants were selected using convenience sampling. Data were collected using a three-part researcher-made questionnaire assessing maternal, childbirth, and neonatal characteristics based on the medical histories of mothers and newborns as well as interviews with mothers. Neonatal jaundice was measured utilizing the Kj-8000 device on days 3-6 after birth by measuring the skin bilirubin level. Moreover, the need to phototherapy (severe jaundice) was determined based on the American Academy of Pediatrics guideline. The generalized linear model and multivariate logistic regression were employed for data analysis.
    Results
    Out of the followed-up neonates, 94 (9.96%) cases developed severe jaundice and 850 (90.04%) newborns did not show any symptoms of this complication. The results of the adjusted generalized linear model showed that infant’s bilirubin level correlated significantly with a history of jaundice in previous children, infant’s age at first meconium excretion, frequency of feeding, gestational age, mother’s blood type, and a number of maternal ultrasound during pregnancy. Moreover, the results of adjusted logistic regression revealed an association between severe jaundice and variables, such as the place of residence, history of jaundice in previous children, feeding the infant with water or sugar water during breastfeeding intervals, frequency of feeding the infant, gestational age, mother’s blood type, time of discharge from the hospital, and number of pregnancies.
    Conclusion
    Some factors related to neonatal jaundice were identified to raise the awareness of healthcare personnel for the early identification and treatment of neonatal jaundice in order to prevent its complications.
    Keywords: Jaundice, Risk Factor, Transcutaneous bilirubin
  • Batul Bahrami, Maryam Marofi *, Ziba Farajzadegan, Behzad Barekatain Pages 68-75
    Background
    Feeding difficulties are common and important in premature infants. In order to identify neonatal feeding difficulties, clinicians and nurses require assessment tools to conduct an objective evaluation of infant oral feeding (breast/bottle-feeding). Early identification of infants with feeding difficulty is critical to implement appropriate therapies and optimize the infant’s nutrition and oral feeding skill development. The aim of this study was to validate the Early Feeding Skills (EFS) Instrument for the Evaluation of Oral Feeding in Premature Infants.
    Methods
    In this descriptive cross-sectional study, the researcher initially got permission from the designer of the tool through email. Then, the main version of this tool was translated into Persian using the standard backward/forward method. Subsequently, the formal and content validity of the questionnaire was confirmed. After visiting a neonatal specialist, the assessment of preterm infants' oral feeding skills was performed using the EFS Instrument by observing the infants (n=180) during feeding. The exploratory factor analysis and Cronbach’s alpha were utilized to determine the construct validity and assess the reliability of the instrument, respectively.
    Results
    All items of the EFS Instrument had formal validity. Two items were removed from the instrument, based on content validity. On the basis of factor analysis, the items with a minimum factor load of 0.4 were evaluated and according to the factor load of all items (above 0.4), EFS Instrument had the appropriate construct validity. The Cronbach's coefficient of the instrument was obtained 0.88.
    Conclusion
    The EFS Instrument obtained the required reliability and validity for the evaluation of preterm infants oral feeding.
    Keywords: Infant, oral feeding, Premature, Validity
  • Ashraf Abou, Taleb *, Hisham A. Alghany Algahlan Pages 76-83
    Background
    Periventricular hemorrhage (PVH) is common in early preterm neonates but much less common in term and late preterm neonates. The aim of this study was to identify the risk factors and outcomes of PVH in term and late preterm neonates in a tertiary care center in Upper Egypt.
    Methods
    A total of 33 term and late preterm (≥ 34 weeks of gestation) neonates (18 females and 15 males) admitted at the neonatal intensive care unit (NICU) of Sohag University Hospital and diagnosed with PVH from January 2016 to October 2018 were included in this prospective study. The neonates with major congenital malformation of the nervous system were excluded. The risk factors for PVH were ascertained, and the diagnosis was confirmed by transcranial ultrasound, computed tomography or magnetic resonance imaging. The outcomes were assessed at discharge time and the age of 6 months.
    Results
    ThePVH was diagnosed in 5.9% of the neonates. The median of age was 12 days (age range: 2-28 days) at presentation, and convulsions were the most frequent presentations followed by poor suckling and pallor. The risk factors for PVH included prolonged prothrombin time, thrombocytopenia, non-administration of vitamin K, and prolonged labor in 54.5%, 30 %, 30 %, and 27.3% of the cases, respectively. Moreover, 26 (78.7 %) cases were discharged with good conditions, and 7 (21.2 %) subjects died in the NICU. The investigation of the patients’ follow-up at the age of 6 months revealed neurological impairments in 36 % of the survivors. Grade IV and III PVH had mortality rates of 66.67% and 18.75%, respectively (P=0.009). Neurodevelopmental impairments were more frequent in Grade III and IV (P=0.002).
    Conclusion
    The incidence of PVH was 5.9% among term and late preterm neonates admitted to our NICU. Coagulation disorders and prolonged labor were the leading risk factors. Most patients had favorable outcomes, and one-third of the cases had neurodevelopmental impairments. In addition, mortality was reported in one-fifth of the neonates. Severe grades of PVH had the worst outcomes.
    Keywords: Late preterm, Outcome, Periventricular hemorrhage, Risk factors, Term neonates
  • Reza Saeidi, Mahboobe Gholami * Pages 84-88
    Background
    Many factors affect exclusive breastfeeding, include method of delivery, parity, maternal age and education, smoking, ethnicity, pregnancy complications, infant health, and environmental factors such as hospital practices, and social support.
    Recently, maternal obesity has been suggested as a risk factor for poor breast-feeding practices whose role has not yet been completely determined. This study was conducted to evaluate the effect of maternal obesity on exclusive breastfeeding.
    Methods
    This Cross sectional study was carried out among women receiving prenatal care by midwives at the health center of Neyshabur University of Medical Sciences during 2014-2016. Data regarding maternal and neonatal variables were gathered. To analyze the data, Chi-squared test was run to determine the association between categorical variables.
    Results
    Planned feeding method, educational level, employment status, and gestational age were the four strongest predictors for poor breast-feeding practices at discharge. Method of delivery, gestational diabetes, and maternal hypertension and maternal obesity were significantly associated with exclusive breastfeeding at discharge (p<0.05).
    There was not any significant relationship between neonatal weight and gestational age with exclusive breastfeeding at discharge (p<0.05), but there was a significant association between gestational age and exclusive breastfeeding at discharge (p=0.042).
    Conclusion
    This study indicated that maternal obesity is an important predictor of exclusive breastfeeding at discharge.
    Keywords: Exclusive breast-feeding, Maternal obesity, Newborn
  • Bansal Sharad *, Jangir Narendra, Sharma Rambabu Pages 89-91
    Background
    Meckel-Gruber Syndrome (MGS) is a rare autosomal recessive congenital syndrome with triad of encephalocele, polydactyly, and polycystic kidneys. The worldwide incidence of the MGS is 1 in 1.3-1 in 140,000 live births. The highest incidence of 1 per 1,300 live births (carrier rate of 1 in 18) was reported in Gujarati Indians. MGS is caused by mutation in the meckelin transmembrane receptor (MKS3) located in the interior of the cells in the ciliary transition zone. Therefore, MGS as a fatal congenital syndrome belongs to a group of diseases known as ciliopathies. Most of the fetuses affected by this syndrome die before birth or soon after birth due to oligohydramnios, respiratory failure, and renal failure. There are few case reports of this syndrome associated with cleft lip and palate, inguinal hernia, congenital heart disease, micrognathia, microcephaly, and other abnormalities.
    Case report
    We report a case of unusual and interesting occurrenceof ambiguous genitalia in the MGS syndrome.
    Conclusion
    The MGS is a rare fatal syndrome and can be diagnosed prenatally. In the current case, we observed that ambiguous genitalia should be taken into consideration, in addition to the cardinal features. Parents should be counselled about the outcomes of a child, as well as the chance of recurrence (25%) in the subsequent pregnancies.
    Keywords: Ambiguous genitalia, Encephalocele, Polycystic kidneys
  • Gholamreza Faal *, Elham Nokandi, Elham Noferesti, Fatemeh Noferesti Pages 92-95
    Background
    This is a case report regarding a 2051-gram female newborn affected by right-sided congenital diaphragmatic hernia (CDH) presenting with encephalocele in the occipital region.
    Case report
    The newborn was delivered by a 38-year-old mother from Darmian city, a rural district located in South Khorasan province, Iran.
    Conclusion
    The CDH is an abnormality that rarely occurs with an approximate ratio of one in 3000 live births. It has been reported that 85% of the infants affected by this condition are left-sided and the classic posterolateral or Bochdalek hernia is the most common form. An incidence of 40%–50% has been reported regarding the other malformations associated with CDH, the most common of which are those involving the central nervous system. Some studies reported other rare associated abnormalities, including hepatopulmonary fusion, hypoplastic left heart syndrome, left heart hypoplasia, duodenal atresia, malrotation, and anorectal malformation. This case report aimed to mention encephalocele as another complication, which has not been reported for the CDH.
    Keywords: Congenital, Diaphragmatic, Encephalocele, Hernia, Newborn