A couple of the first cousins born with hypotonia and maternal polyhydramnios.

Congenital myotonic dystrophy should be considered in hypotonic infants with polyhydramniotic mothers with a positive history of myotonia. Abstract: Congenital myotonic dystrophy (CDM) is a predominantly maternally inherited disease and results from increased numbers of cytosine, thymine, and guanine (CTG) repeats in the unstable DNA regions and presents as hypotonia in the neonatal period and myotonia in adulthood. This report aims to present two cases of CDM. A first-cousin couple was born and hospitalized due to hypotonia at birth and a maternal history of polyhydramnios during this pregnancy. The first-born baby girl was admitted to the NICU with tachypnea and hypotonia, clubfoot, and frog-like posture. The pregnancy was complicated by polyhydramnios. Interestingly, her first cousin was born the next day with a similar picture and history. Myotonia was detected in their mothers. The concurrent presence of hypotonia and polyhydramnios as well as maternal myotonia in a first cousin should be considered CDM until proven otherwise and this was confirmed by the EMG- NCV test.

The effect of oral use of concentrated pomegranate juice by mothers on hyperbilirubinemia in neonates under phototherapy: A randomized clinical trial.

Background: Hyperbilirubinemia is a common problem in neonates that causes hospitalization. The aim of this study was to investigate the effects of concentrated pomegranate juice (CPJ) consumption by breastfeeding mothers on neonatal hyperbilirubinemia. Materials and Methods: In this open-labeled, add-on, randomized clinical trial, 86 breastfeeding mothers and their neonates were allocated into two groups. In the control group, neonates received phototherapy. Besides neonates' phototherapy in the intervention group, their mothers received CPJ (1 tablespoon [15 g] three times a day) up to discharge. The bilirubin level was the primary outcome. The duration of phototherapy, the duration of hospital stay, and the need for exchange transfusions were secondary outcomes. Results: CPJ reduced the bilirubin level of hospitalized neonates within 48 h after consumption (P = 0.048, standard mean difference = 0.648). It also resulted in reduced duration of hospital stay and faster discharge of the neonates. Furthermore, in 48 h after discharge, bilirubin was significantly lower in the CPJ group (P = 0.003, partial eta squared = 0.123). Conclusion: Compared to the control group, consumption of CPJ by lactating mothers whose infants underwent phototherapy resulted in lower bilirubin levels, shorter hospital stay, and faster discharge.

Comparison of heated humidified high flow nasal cannula and nasal continuous positive airway pressure after surfactant administration in preterm neonates with respiratory distress syndrome.

BACKGROUND AND OBJECTIVE: The aim of this study was to evaluate the effect of humidified high-flow nasal cannula (HHHFNC) therapy, and compare it with the effect of nasal Continuous Positive Airway Pressure (NCPAP) in neonates with respiratory distress syndrome (RDS). METHOD: In this clinical study, consecutively admitted 27-32 weeks preterm infants with RDS who received surfactant through a brief intubation (INSURE method) were randomly assigned immediately after extubation to HHHFNC or NCPAP. Primary outcomes were oxygen saturation values and oxygen need at 6, 12 and 24 h after surfactant administration as well as duration of oxygen and respiratory support, need for intubation and mechanical ventilation and incidence of apnea. Secondary outcomes were duration of hospitalization and incidence of complications such as pneumothorax. RESULTS: Sixty-four infants met the inclusion criteria and were enrolled in the study, 32 per arm. Two cases in HHFNC group dropped due to congenital pneumonia/sepsis. No differences were seen between groups in primary and secondary outcomes except for arterial oxygen saturation values (SaO2) 24 h after surfactant administration that were significantly higher in the NCPAP group [95.97% ± 1.96% vs. 95.00% ± 1.80% (P = .04)] with similar oxygen needs. The treatment failure was observed in four (11.8%) infants of the NCPAP group compared to five (16.7%) cases of the HHHFNC group (P = .57). CONCLUSION: Based on the results of the present study, the HHHFNC can be as effective as NCPAP to treat the neonates with RDS after surfactant administration.

Cord Blood Alkaline Phosphatase as an Indicator of Neonatal Jaundice.

BACKGROUND: Management of hyperbilirubinemia remains a challenge for neonatal medicine because of the risk of neurological complications related to the toxicity of severe hyperbilirubinemia. OBJECTIVES: The purpose of this study was to examine the validity of cord blood alkaline phosphatase level for predicting neonatal hyperbilirubinemia. PATIENTS AND METHODS: Between October and December 2013 a total of 102 healthy term infants born to healthy mothers were studied. Cord blood samples were collected for measurement of alkaline Phosphatase levels immediately after birth. Neonates were followed-up for the emergence of jaundice. Newborns with clinical jaundice were recalled and serum bilirubin levels measured. Appropriate treatment based on serum bilirubin level was performed. Alkaline phosphatase levels between the non-jaundiced and jaundiced treated neonates were compared. RESULTS: The incidence of severe jaundice that required treatment among followed-up neonates was 9.8%. The mean alkaline phosphatase level was 309.09 ± 82.51 IU/L in the non-jaundiced group and 367.80 ± 73.82 IU/L in the severely jaundiced group (P = 0.040). The cutoff value of 314 IU/L was associated with sensitivity 80% and specificity 63% for predicting neonatal hyperbilirubinemia requiring treatment. CONCLUSIONS: The cord blood alkaline phosphatase level can be used as a predictor of severe neonatal jaundice.

Short Term Outcome of Neonates Born With Abnormal Umbilical Cord Arterial Blood Gases.

BACKGROUND: Umbilical arterial blood gas (UABG) analysis is more objective than other methods for predicting neonatal outcome. Acidemic neonates may be at risk for unfavorable outcome after birth, but all neonates with abnormal arterial blood gas (ABG) analysis do not always have poor outcome. OBJECTIVES: This study was carried out to determine the short term outcome of the neonates born with an abnormal ABG. PATIENTS AND METHODS: In a cohort prospective study 120 high risk mother-neonate pairs were enrolled and UABG was taken immediately after birth. All neonates with an umbilical cord pH less than 7.2 were considered as case group and more than 7.2 as controls. Outcomes like need to resuscitation, admission to newborn services and/or NICU), seizure occurrence, hypoxic ischemic encephalopathy (HIE), delayed initiation of oral feeding and length of hospital stay were recorded and compared between the two groups. P value less than 0.05 was considered as being significant. RESULTS: Comparison of short term outcomes between normal and abnormal ABG groups were as the fallowing: need for advanced resuscitation 4 vs. 0 (P = 0.001), NICU admission 16 vs. 4 (P = 0.001), convulsion 2 vs. 0 (P = 0.496), HIE 17 vs. 4 (P = 0.002), delay to start oral feeding 16 vs. 4 (P = 0.001), mean hospital stay 4 vs. 3 days (P = 0.001). None of the neonates died in study groups. CONCLUSIONS: An umbilical cord PH less than 7.2 immediately after birth can be used as a prognostic factor for unfavorable short term outcome in newborns.

Correlation between hyperglycemia and retinopathy of prematurity.

BACKGROUND: Several risk factors are attributed to retinopathy of prematurity (ROP). This study was done to determine any association between hyperglycemia and ROP in premature infants. METHODS: In a retrospective case-control analysis, all infants with a gestational age (GA) < 34 weeks and a birthweight (BW) < 2000 g admitted and treated in the Neonatal Intensive Care Unit at Amirkola Children's Hospital, Iran, during March 2007-September 2010 were included. Hyperglycemia was defined as a plasma glucose level of >150 mg/dL during the hospital stay. The duration of being hyperglycemic was also recorded. All of these neonates were examined for ROP by a retinologist unaware of group assignment. The difference in the ROP incidence and also the severity of ROP was compared between the hyperglycemic and non-hyperglycemic infants. Matching was done for GA, BW, and also Clinical Risk Index for Babies score. The data were analyzed by t-test, χ(2) -test and logistic regression test and a P < 0.05 was considered significant. RESULTS: In total, 155 neonates were examined. Seventy (45.2%) of them developed ROP but 85 (54.8%) did not show any evidence of ROP. The frequency of hyperglycemia in patients with ROP was 33 (47.2%), but in those without ROP, hyperglycemia occurred in five (5.9%) (P = 0.0001). The severity of ROP showed no significant differences between the two groups (P = 0.35). The logistic regression for GA and BW showed a significant correlation between hyperglycemia and ROP (P = 0.0001). CONCLUSIONS: Hyperglycemia is an important risk factor for ROP that can be prevented along with other risk factors by accurate supervision.

Prevalence of permanent and transient congenital hypothyroidism in Babol City -Iran.

AIM: To determine the incidence of transient and permanent hypothyroidism in Babol, Iran. METHODS: The cross sectional study on 10,573 newborns who participated in a thyroid screening program in Babol between 2006 and 2008 was performed. It recalled patients who had been diagnosed in the initial screening program and treated for at least 3 years, discontinued their treatment for 4 weeks and then measured their plasma thyroid hormone concentrations. If their thyroid stimulating hormone (TSH) concentration was greater than 10 m/l they were considered to have permanent CH. T3 uptake, T4 and TSH were measured in venous blood samples. Other information collected and recorded included age, presence of diabetes, birth weight and gestational age at birth. RESULTS: The incidence of transient and permanent hypothyroidism in the studied infants was 5.7 and 20.8 per 10,000, respectively. The incidence of transient hypothyroidism in male and female infants was 16.9 and 24.6 per 10,000, respectively, and of permanent hypothyroidism in male and female infants 7.5 and 3.8 per 10,000, respectively. CONCLUSION: A noteworthy incidence of permanent hypothyroidism in infants found in this study confirms that screening programs need to be continued in our region and that interventions to address the causes of hypothyroidism are necessary.

Outcomes of very-low-birthweight infants after discharge with a discharge weight of 1500 grams.

BACKGROUND: Discharge of very-low-birthweight (VLBW) neonates from hospital is an important issue and has a standard criterion worldwide. According to this criterion, VLBW infants will be discharged from hospital when weighing 1800-2100 g but in the newborn services at Amirkola Children's Hospital (ACH), VLBW neonates are discharged when reaching 1500 g. The aim of this study was to determine the safety of this policy. METHODS: In this analytic-prospective study, 100 premature neonates with discharging weight (DW) of 1500 g and a control group of 150 neonates with DW of 1600-2500 g were included. Both groups showed similar socioeconomic, perinatal and postnatal conditions. They were admitted and treated in the newborn services at ACH. The outcome variables, including death after discharge, readmission and need for emergency visit for an acute problem up to 3 months after discharge, were studied. The information was analyzed by SPSS-16 software and a P-value < 0.05 was considered significant. RESULTS: Need for emergency visit after discharge reached a significant difference between the two groups (31 in the case group vs 21 in the control group, P= 0.000), but there were no significant differences in the readmission rate and death after discharge (two deaths and 15 readmissions in the case group vs two deaths and 21 readmissions in the control group, P= 0.855) CONCLUSION: In order to decrease the need for emergency visits, we suggest a program of early hospital discharge with home nursing visits and neonatologist support.

Effects of phototherapy on cytokines' levels and white blood cells in term neonate with hyperbilirubinemia.

OBJECTIVE: Phototherapy is the most common treatment used for severe jaundice. There is increasing evidence that phototherapy can directly affect the expression and function of cell surface receptors including adhesion molecules, cytokines, and growth factor receptors. The aim of this study is to investigate the effect of phototherapy use on the levels of interleukin (IL)-1α, IL-6, and tumor necrosis factor (TNF)-α as cytokine expressions from keratinocytes, and also white blood cell counts in the treatment of neonate with hyperbilirubinemia. MATERIALS AND METHODS: We studied 32 term newborns with hyperbilirubinemia. Blood samples were obtained before and 72 h after phototherapy. Serum levels of IL-1α, IL-6, TNF-α, and WBC count were measured in the samples using appropriate methods. RESULTS: Serum TNF-α at 72 h of exposure to phototherapy increased, while the levels of IL-1α and IL-6 at the same time were decreased. These changes were not statistically significant. WBC counts rose significantly with phototherapy. CONCLUSION: Phototherapy in term neonate does not affect cytokines' levels, but can raise peripheral WBC count.

Early surgical intervention for diaphragmatic paralysis in a neonate; report of a case and literature review.

BACKGROUND: Diaphragmatic paralysis in newborns is related to brachial plexus palsy. It can cause respiratory failure necessitating prolonged mechanical ventilation and subsequent extubation failure. CASE PRESENTATION: We present a two-hour-old male newborn with a birth weight of 4500 grams who had a right-sided brachial plexus palsy and right diaphragmatic paralysis due to shoulder dystocia. He developed respiratory distress due to isolated paralysis of the right hemi diaphragm. The clinical course was progressive, his condition worsening despite oxygen application. Physical examination, chest X-rays and M-mode ultrasonography of the diaphragm confirmed the diagnosis diaphragmatic paralysis. Surgical plication of diaphragm was done earlier than the usual time because of recurrent extubation failure. Diaphragmatic plication led to rapid improvement of pulmonary function and allowed discontinuation of mechanical ventilation in less than 3 days. CONCLUSION: Early diaphragmatic plication enhances weaning process and may prevent or minimize the morbidity associated with long-term mechanical ventilation in a neonate with diaphragmatic paralysis.

Cord blood -fetoprotein as a predictive index for indirect hyperbilirubinemia in term neonates.

BACKGROUND: Prediction of severe neonatal hyperbilirubinemia is very important for early treatment and prophylaxis of neurologic sequels. The aim of this study was to evaluate the predictive role of umbilical cord -fetoprotein (UCAFP) as a marker of an increased risk for neonatal hyperbilirubinemia in full term babies. METHODS: Umbilical cord blood was collected from 400 term singleton full term well newborn babies who met our inclusion criteria and stored in -20°C. Those who developed jaundice and admitted for phototherapy (34 newborns: 22 males and 12 females), considered as case group and 31 non-jaundiced infants (13 males and 18 females) gestational age-weight-matched considered as the control group. The serum level of UCAFP was checked in these 65 newborns and was compared between these two groups. RESULTS: Mean UCAFP in case group was 523.429±174.158 and in control group was 664.548±154.894 µg/L. In the non-jaundiced group, mean UCAFP values was higher than neonate with hyperbilirubinemia (664.548 vs. 523.429µg/L). The mean UCAFP in males was 519.023 µg/L and in females was 531.508 µg/L (p=0.066). Sixty (92.3%) babies delivered by cesarean section (CS) and 5 (7.7%) by normal vaginal delivery (p=0.566). CONCLUSION: According to our study, there was no significant positive association between UCAFP and subsequent neonatal indirect hyperbilirubinemia or serum bilirubin level.

Correlation between Umbilical Cord pH and Apgar Score in High-Risk Pregnancy.

OBJECTIVE: The Apgar score as a proven useful tool for rapid assessment of the neonate is often poorly correlated with other indicators of intrapartum neonatal well-being. This study was carried out to determine the correlation between umbilical cord pH and Apgar score in high-risk pregnancies. METHODS: This is a prospective cross-sectional, analytic study performed on 96 mother-fetal pairs during 2004-2005 at Shahid Yahyanejad Hospital, which is affiliated to Babol University of Medical Sciences. Apgar score at 1 and 5 minutes after birth was taken and an umbilical cord blood gas analysis was done immediately after birth in both groups. Mothers came with a labor pain and were divided into high-risk and low risk if they have had any perinatal risk factors. Other data like gestational age, birth weight, need for resuscitation and admission to the newborn ward or Neonatal Intensive Care Unit was gathered by a questionnaire for comparison between the two groups. P-value less than 0.05 was considered being significant. FINDINGS: The gestational age and birth weight were the same in high-risk and low risk mothers. Mean umbilical artery blood pH in high-risk mothers was significantly lower than in low risk mothers (P=0.004). Mean Apgar scores at 1 and 5 minutes were significantly lower in high-risk mothers than in low risk mothers (P<0.05). According to the Kendal correlation coefficient there was no significant correlation between Apgar score at 1 and 5 minutes and umbilical cord pH in low risk group (r=0.212, P=0.1). But in high-risk group there was significant correlation between Apgar score at 1(st) and 5(th) minute and the umbilical cord pH (r=0.01, P=0.036 and r=0.176, P=0.146, respectively). CONCLUSION: Combination of Apgar score and umbilical cord pH measurement in high-risk pregnant mother could better detect jeopardized baby.

Efficacy of clofibrate on severe neonatal jaundice associated with glucose-6-phosphate dehydrogenase deficiency (a randomized clinical trial).

Glucose-6-phosphate dehydrogenase (G6PD) deficiency may cause severe hyperbilirubinemia with bilirubin encephalopathy unless intervention is initiated. The aim of this study was to assess the efficacy of clofibrate in full term G6PD deficient neonates with jaundice. A randomized clinical trial study was performed in two groups of full-term G6PD deficient jaundiced neonates (clofibrate treated group, n = 21; control group, n = 19). Infants in the clofibrate group received a single oral dose of 100 mg/kg clofibrate, whereas control group received nothing. Both groups were treated with phototherapy. Serum total and direct bilirubin levels were measured at the onset of treatments, 16, 24 and 48 hours later. On enrollment, the mean total serum bilirubin (TSB) level in the clofibrate treated group was 18.40 +/- 2.41 and in the control group was 17.49 +/- 1.03 (p = 0.401). At 16, 24 and 48 hours of treatment, the mean TSB in the clofibrate group were 15.2 +/- 1.9, 12.6 +/- 2.4, and 10.1 +/- 2.4 and in the control group were 16.5 +/- 1.2, 13.3 +/- 2.2 and 11.4 +/- 2.4, respectively (p = 0.047). At 48 hours, 7 (33%) cases in the clofibrate group and one (5%) case in the control group were discharged with a TSB < 10 mg/dl (p = 0.031). No side effects were observed on serial examinations during hospitalization, or on the 1st and 7th days after discharge. The results show that clofibrate induces a faster decline in serum total bilirubin level, a shorter duration of phototherapy, and hospitalization with no side effects in full-term G6PD deficient neonates with jaundice.

Effect of clofibrate in jaundiced full-term infants:a randomized clinical trial.

BACKGROUND: Hyperbilirubinemia is a common problem in newborn infants. It can progress to kernicterus in severe forms, unless an intervention is initiated. The objective of this study was to determine the therapeutic effect of clofibrate in full-term neonates with nonhemolytic jaundice. METHODS: A randomized clinical trial was performed on two groups of full-term jaundiced neonates: the clofibrate-treated group (n = 30) and the control group (n = 30). Infants in the clofibrate group received a single oral dose of 100 mg/kg clofibrate while the neonates in the control group received distilled water (same color and volume); both groups received phototherapy. Serum total and direct bilirubin levels were measured at the beginning, 16, 24, 48, and 74 hours, after the start of the trial. RESULTS: The mean+/-SD total serum bilirubin level of the control and clofibrate groups at enrollment was 17.5+/-2.3 and 18.2+/-1.9 mg/dL, respectively (P = 0.199). The mean+/-SD total serum bilirubin in the control and clofibrate groups after 48 hours was 11.4+/-2.4 and 10.1+/-2.4 mg/dL, respectively (P = 0.047). After 72 hours of intervention, 25 (83%) neonates of the clofibrate group and 16 (53%) of the control group were discharged with a total serum bilirubin of <10 mg/dL (P = 0.026). No side-effect was observed on serial examination during hospitalization, and on the first and seventh day after discharge. CONCLUSION: Clofibrate results in a faster decline in TSB, shorter duration of hospitalization and had no side effects in jaundiced full-term neonates.

The effect of topical application of human milk, ethyl alcohol 96%, and silver sulfadiazine on umbilical cord separation time in newborn infants.

BACKGROUND: Several agents have been used for neonatal umbilical cord care, but we did not find any study evaluating the effect of human milk on umbilical cord separation time. The purpose of this study was to compare the effect of topical application of human milk, ethyl alcohol 96%, and silver sulfadiazine on umbilical cord separation time in newborn infants. This study was undertaken place at a primary-level newborn nursery at a university teaching hospital and a private hospital. Of 373 singleton near- to full-term newborns enrolled in the study, 312 completed the study. METHODS: Newborns from birth were randomized to either: 1) mother's milk group, 2) alcohol group, 3) silver sulfadiazine group, and 4) control (no treatment) group. Mother's milk for group 1, ethyl alcohol for group 2, and silver sulfadiazine ointment for group 3 were applied to the umbilical stump three hours after birth and continued every eight hours until two days after umbilical cord separation. The time to umbilical cord separation and any discomfort such as infection, hemorrhage, and granuloma formation were reported by mothers. Nothing was applied to the umbilical stump of the control group and they received dry cord care only. RESULTS: It was observed a significant difference in the mean cord separation time among the four group. No significant complications were observed in any group. CONCLUSION: Breast milk could be substituted for other topical agents for umbilical cord care, but a multicenter study is required in order to advise it for routine umbilical cord care.