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Cystic fibrosis presenting with hypoproteinemia, anemia, and failure to thrive in two neonates
S96
Abstracts
measured in the morning. Peak serum cortisol values were measured after 30 min low-dose ACTH stimulation test.
Materials and methods
The mean basal serum and peak serum cortisol values of ventilated preterm infants (n=21; 11.46±7.61 and 23.25±7.46 μg/dl) were significantly lower than that of preterm infants not ventilated (n=12; 18.21±8.68 and 31.21±9.38 μg/dl) in respire disease group and similar to controls (n=10; 10.91±5.50 and 26.27±7.12). Response to ACTH stimulation was not significantly different in the three groups.
Retrospective review of 40 PPHN infants from 2001 to 2007 admitted to our NICU. The diagnosis of PPHN was made by clinical manifestation and/or echocardiography. All of them received tolazoline and nonhyperventilation mechanical ventilation. Initial dosage of tolazoline was 1 mg/kg IV in 10 min, maintaining dosage was 1 mg/kg h. They were divided by two groups: effected group (group A) and invalid group (group B). Compare with OI of two groups before and after using tolazoline 6 h, 12 h, and 24 h. To investigate the therapeutic efficacy and complications of tolazoline.
Conclusions
Results
Adrenal function of preterm infants with and without respiratory disease, whether ventilated or not ventilated has a similar response to ACTH test. We did not find any relationship between adrenal function, and respiratory disease and/or mechanical ventilation.
The main gestational weeks were 38.97 ± 2.86";; main birth weight were 3038 ± 662 g, main age were 7.45 h (1.5–24 h). 77.5% (31/40) of infants was effective, 22.5% was ineffective. There were statistically significant difference (F 45.37, p < 0.01) compare with OI before and after using tolazoline 6 h, 12 h, and 24 h in A group. Although OI decreased after using tolazoline, there was no statistically significant difference (F 0.83, p > 0.05) in B group. The main tolazoline using times was 24.15 + 5.57 h and the main mechanical ventilation time was 5.18 ± 2.43d in A group.
Results
doi:10.1016/j.earlhumdev.2008.09.245
Abstract UENPS.230 Relationship between adrenal function and pulmonary disease in preterm infants Yunlin Shen⁎ Shanghai, China Background and aim To assess the adrenal function in preterm infants with various degrees of pulmonary diseases and respiratory support in order to elucidate relationship between adrenal function and pulmonary disease. Materials and methods 43 preterm infants (33 preterm with respire disease and 10 control) treated at Shanghai Children's Hospital. Serum cortisol values were measured in the morning. Peak serum cortisol values were measured after 30 min low-dose ACTH stimulation test.
Conclusions The effective rate of administration of tolazoline in PPHN was 77.5%. Tolazoline was a nonexpensive and easy to use drug. We recommended if the OI was not significantly decrease after 6 h administration of tolazoline, other therapeutic method should be considered.
doi:10.1016/j.earlhumdev.2008.09.247
Abstract UENPS.232 Cystic fibrosis presenting with hypoproteinemia, anemia, and failure to thrive in two neonates Cristina Maria Mihai⁎,a, Larisia Mihaia, Elena Leub, Adriana Luminita Balasaa, Viviana Cuzica, Corina Elena Frecusa, Ramona Mihaela Stoicescua “Ovidius” University, Faculty of Medicine, Constanta, Romania Constanta County Hospital, Constanta, Romania
Results Background and aim The mean basal serum and peak serum cortisol values of ventilated preterm infants (n=21; 11.46±7.61 and 23.25±7.46 μg/dl) were significantly lower than that of preterm infants not ventilated (n=12; 18.21±8.68 and 31.21±9.38 μg/dl) in respire disease group and similar to controls (n=10; 10.91±5.50 and 26.27±7.12). Response to ACTH stimulation was not significantly different in the three groups.
Adrenal function of preterm infants with and without respiratory disease, whether ventilated or not ventilated have a similar response to ACTH test. We did not find any relationship between adrenal function, and respiratory disease and/or mechanical ventilation.
Cystic fibrosis (CF) is a hereditary disease that has primary effects on the lungs, pancreas, intestine, liver, sweat glands, and male reproductive tract as well as important secondary effects on growth and nutrition. The clinical course is variable, but most patients succumb to lung disease in early adulthood. CF usually presents in infancy. Meconium ileus, a neonatal intestinal obstruction, occurs in approximately 17% of infants with CF. Beyond the perinatal period, CF presents as failure to thrive secondary to exocrine pancreatic insufficiency, chronic respiratory symptoms, or both. Nutritional deficits can be severe at presentation and may lead to edema and hypoproteinemia from protein–calorie malnutrition.
doi:10.1016/j.earlhumdev.2008.09.246
Materials and methods
Conclusions
The authors present two patients with hypoproteinemia in neonatal period of different clinical severities. Both of them had anemia and failure to thrive. Abstract UENPS.231 Tolazoline treating persistent pulmonary hypertension of the newborn Qunfang Rong⁎ Shanghai, China
Results The diagnosis of cystic fibrosis was confirmed by a positive sweat test. No one of the patients had prenatal diagnosis. Conclusions
Background and aim To evaluate the effectiveness of tolazoline treating persistent pulmonary hypertension of the newborn.
Despite its relative frequency among autosomal recessive diseases and the availability of the sweat test, cystic fibrosis (CF) has been difficult to diagnose in early childhood, and delays can lead to severe malnutrition, lung
Abstracts
disease, or even death. Whether newborn screening and early treatment may prevent the development of a nutritional deficiency is not known. doi:10.1016/j.earlhumdev.2008.09.248
S97
common respiratory complaints of the small graduates in the follow-up period. We present a former very low birth weight infant whose respiratory symptoms persisted for months with partial relief in response to symptomatic therapies against BPD and GER. Materials and methods
Abstract UENPS.233 Prenatal corticosteroids and respiratory distress: Is there benefit in preterm babies with 34 weeks of gestation? Sónia Antunes⁎, Dora Fontes, Susana Gomes, Ana Serrano Hospital Espirito Santo de Évora, E.P.E, Évora, Portugal Background and aim Previous studies have shown that prenatal corticosteroids (PC) significantly reduce the incidence of respiratory distress syndrome (RDS) in preterm babies born before 34 weeks of gestation. Their use at 34 weeks and after is not a common practice in all centres and there is controversy about their effective benefit. A recent study suggests that PC are effective in reducing RDS and transient tachypnoea of the newborn (TTN) in term babies delivered by elective caesarean section. The aim of this study is to assess if the use of PC in newborns with 34 weeks of gestation reduced the incidence and severity of respiratory distress (RD). Materials and methods Retrospective case–control study. Review of the clinical registers of all newborns with gestational age between 34 weeks and 0 days and 34 weeks and 6 days, born between January 2003 and December 2007. The newborns included were divided into 2 groups: in Group I (cases) were included the newborns of mothers to whom were administrated PC and in Group II (controls) were included those that had no PC. The newborns admitted to Neonatal Intensive Care Unit were considered as having RD in the first 24 h of life, with tachypnoea, chest retractions or nasal flaring and that needed oxygen therapy. We admitted as criteria of gravity of RD the need for ventilation, duration of oxygen therapy and length of hospital stay. The statistical analysis was performed with the Statistical Package Social Sciences (SPSS), version 16.0. The tests used were Fisher's Exact Test and t-student. It was considered a p value < 0.05 as statistically significant. Results Seventy seven children were included in the study, 31 in Group I and 46 in Group II. There was no statistically significant difference in birth weight and sex between the two groups. The number of caesareans was significantly higher in group I (71% vs. 48%, p = 0.037), but when considering the mode of delivery there was no significant difference in the incidence of RD. There was need for resuscitation only in Group II (17%, p = 0.012). There was no difference concerning the incidence of RD, RDS or TTN, need for ventilation, duration of oxygen therapy or days of hospital stay. Conclusions In our study the use of PC didn't prove to reduce the incidence or severity of RD in newborns with 34 weeks of gestation.
The baby was born at 31 weeks of gestation and stayed in NICU for 38 days. On the 2nd month of life she was brought to our outpatient clinic with the complaints of cough, wheezing and cyanosis and given symptomatic therapy with the diagnosis of viral pneumonia. After discharge, symptoms persisted with the predominance of wheezing, coughing, head tilting and vomiting after feeding. Unilateral hyperlucency and hyperinflation on the chest radiograms were prominent. Bronchoscopy revealed external compression of left main bronchus with a pulsatile structure. Right aortic arch and aberrant left subclavian artery were found on thorax CT angiography. Results On the postnatal 9th month she was operated and a right aortic arch, left subclavian artery and a left ligamentum arteriosum passing along the superior surface of the left main-stem bronchus, causing compression of an underlying malacic trachea segment were detected. After surgery, though respiratory symptoms improved, difficulty in feeding and swallowing solid foods remained. Conclusions Inspiratory stridor, dyspnea, cough, wheezing, recurrent respiratory tract infections and dysphagia are common symptoms during early childhood, but their persistence should alert the general pediatrician for the differential diagnosis of disease states, one of which is vascular ring. The diagnosis of vascular ring is often overlooked due to the relatively rare incidence, the nonspecific nature and high prevalence of these symptoms in the pediatric population especially in former prematures. It is important for the general pediatrician to be familiar with the condition and the appropriate diagnostic workup. doi:10.1016/j.earlhumdev.2008.09.250
Abstract UENPS.235 Bile acids cause lung injury through phospholipase A2 activity enhancement, revertible by exogenous surfactant administration An in vitro study in an extracellular model of aspiration Daniele De Luca⁎a, Angelo Minuccib, Enrico Zeccac, Marco Piastraa, Pietrini Domenicoa, Giorgio Contia, Ettore Domenico Capoluongod a Pediatric Intensive Care Unit, Department of Anaesthesiology and Intensive Care, Catholic University of the Sacred Heart, University Hospital “A. Gemelli”, Roma, Italy b Laboratory of Clinical Molecular Biology, Institute of Biochemistry and Clinical Biochemistry, University Hospital “A. Gemelli”, Catholic University of the Sacred Heart, Roma, Italy c Division of Neonatology, Department of Pediatrics, University Hospital “A. Gemelli”, Catholic University of the Sacred Heart, Roma, Italy d Laboratory of Clinical Molecular Biology, Institute of Biochemistry and Clinical Biochemistry, University Hospital “A. Gemelli”, Roma, Italy
doi:10.1016/j.earlhumdev.2008.09.249 Background and aim
Abstract UENPS.234 Vascular ring: A rare cause of persistent respiratory symptoms in a very low birth weight infant Akin Ilke Mungan, Begum Atasay, Emel Okulu, Saadet Arsan⁎, Tomris Turmen Ankara University, Ankara, Turkey
Secretory phospholipase A2 (sPLA2) has a key role in acute respiratory distress syndrome causing surfactant degradation and increasing inflammation. Bile acids (BA) are supposed to be toxic for the lung and implicated in different lung injury forms, such as meconium aspiration, neonatal bile acid pneumonia and aspiration due to gastroesophageal reflux. We sought to study the interaction sPLA2-BA in an extracellular environment. Materials and methods
Background and aim Bronchopulmonary dysplasia (BPD) and persisting respiratory symptoms flared with gastroesophageal reflux (GER) and viral infections became fairly
We collected bronchoalveolar lavage fluid (BALF) from 23 neonates/ infants aged <6 months with no lung disease and ventilated because of general anaesthesia requirement.
Abstracts
measured in the morning. Peak serum cortisol values were measured after 30 min low-dose ACTH stimulation test.
Materials and methods
The mean basal serum and peak serum cortisol values of ventilated preterm infants (n=21; 11.46±7.61 and 23.25±7.46 μg/dl) were significantly lower than that of preterm infants not ventilated (n=12; 18.21±8.68 and 31.21±9.38 μg/dl) in respire disease group and similar to controls (n=10; 10.91±5.50 and 26.27±7.12). Response to ACTH stimulation was not significantly different in the three groups.
Retrospective review of 40 PPHN infants from 2001 to 2007 admitted to our NICU. The diagnosis of PPHN was made by clinical manifestation and/or echocardiography. All of them received tolazoline and nonhyperventilation mechanical ventilation. Initial dosage of tolazoline was 1 mg/kg IV in 10 min, maintaining dosage was 1 mg/kg h. They were divided by two groups: effected group (group A) and invalid group (group B). Compare with OI of two groups before and after using tolazoline 6 h, 12 h, and 24 h. To investigate the therapeutic efficacy and complications of tolazoline.
Conclusions
Results
Adrenal function of preterm infants with and without respiratory disease, whether ventilated or not ventilated has a similar response to ACTH test. We did not find any relationship between adrenal function, and respiratory disease and/or mechanical ventilation.
The main gestational weeks were 38.97 ± 2.86";; main birth weight were 3038 ± 662 g, main age were 7.45 h (1.5–24 h). 77.5% (31/40) of infants was effective, 22.5% was ineffective. There were statistically significant difference (F 45.37, p < 0.01) compare with OI before and after using tolazoline 6 h, 12 h, and 24 h in A group. Although OI decreased after using tolazoline, there was no statistically significant difference (F 0.83, p > 0.05) in B group. The main tolazoline using times was 24.15 + 5.57 h and the main mechanical ventilation time was 5.18 ± 2.43d in A group.
Results
doi:10.1016/j.earlhumdev.2008.09.245
Abstract UENPS.230 Relationship between adrenal function and pulmonary disease in preterm infants Yunlin Shen⁎ Shanghai, China Background and aim To assess the adrenal function in preterm infants with various degrees of pulmonary diseases and respiratory support in order to elucidate relationship between adrenal function and pulmonary disease. Materials and methods 43 preterm infants (33 preterm with respire disease and 10 control) treated at Shanghai Children's Hospital. Serum cortisol values were measured in the morning. Peak serum cortisol values were measured after 30 min low-dose ACTH stimulation test.
Conclusions The effective rate of administration of tolazoline in PPHN was 77.5%. Tolazoline was a nonexpensive and easy to use drug. We recommended if the OI was not significantly decrease after 6 h administration of tolazoline, other therapeutic method should be considered.
doi:10.1016/j.earlhumdev.2008.09.247
Abstract UENPS.232 Cystic fibrosis presenting with hypoproteinemia, anemia, and failure to thrive in two neonates Cristina Maria Mihai⁎,a, Larisia Mihaia, Elena Leub, Adriana Luminita Balasaa, Viviana Cuzica, Corina Elena Frecusa, Ramona Mihaela Stoicescua “Ovidius” University, Faculty of Medicine, Constanta, Romania Constanta County Hospital, Constanta, Romania
Results Background and aim The mean basal serum and peak serum cortisol values of ventilated preterm infants (n=21; 11.46±7.61 and 23.25±7.46 μg/dl) were significantly lower than that of preterm infants not ventilated (n=12; 18.21±8.68 and 31.21±9.38 μg/dl) in respire disease group and similar to controls (n=10; 10.91±5.50 and 26.27±7.12). Response to ACTH stimulation was not significantly different in the three groups.
Adrenal function of preterm infants with and without respiratory disease, whether ventilated or not ventilated have a similar response to ACTH test. We did not find any relationship between adrenal function, and respiratory disease and/or mechanical ventilation.
Cystic fibrosis (CF) is a hereditary disease that has primary effects on the lungs, pancreas, intestine, liver, sweat glands, and male reproductive tract as well as important secondary effects on growth and nutrition. The clinical course is variable, but most patients succumb to lung disease in early adulthood. CF usually presents in infancy. Meconium ileus, a neonatal intestinal obstruction, occurs in approximately 17% of infants with CF. Beyond the perinatal period, CF presents as failure to thrive secondary to exocrine pancreatic insufficiency, chronic respiratory symptoms, or both. Nutritional deficits can be severe at presentation and may lead to edema and hypoproteinemia from protein–calorie malnutrition.
doi:10.1016/j.earlhumdev.2008.09.246
Materials and methods
Conclusions
The authors present two patients with hypoproteinemia in neonatal period of different clinical severities. Both of them had anemia and failure to thrive. Abstract UENPS.231 Tolazoline treating persistent pulmonary hypertension of the newborn Qunfang Rong⁎ Shanghai, China
Results The diagnosis of cystic fibrosis was confirmed by a positive sweat test. No one of the patients had prenatal diagnosis. Conclusions
Background and aim To evaluate the effectiveness of tolazoline treating persistent pulmonary hypertension of the newborn.
Despite its relative frequency among autosomal recessive diseases and the availability of the sweat test, cystic fibrosis (CF) has been difficult to diagnose in early childhood, and delays can lead to severe malnutrition, lung
Abstracts
disease, or even death. Whether newborn screening and early treatment may prevent the development of a nutritional deficiency is not known. doi:10.1016/j.earlhumdev.2008.09.248
S97
common respiratory complaints of the small graduates in the follow-up period. We present a former very low birth weight infant whose respiratory symptoms persisted for months with partial relief in response to symptomatic therapies against BPD and GER. Materials and methods
Abstract UENPS.233 Prenatal corticosteroids and respiratory distress: Is there benefit in preterm babies with 34 weeks of gestation? Sónia Antunes⁎, Dora Fontes, Susana Gomes, Ana Serrano Hospital Espirito Santo de Évora, E.P.E, Évora, Portugal Background and aim Previous studies have shown that prenatal corticosteroids (PC) significantly reduce the incidence of respiratory distress syndrome (RDS) in preterm babies born before 34 weeks of gestation. Their use at 34 weeks and after is not a common practice in all centres and there is controversy about their effective benefit. A recent study suggests that PC are effective in reducing RDS and transient tachypnoea of the newborn (TTN) in term babies delivered by elective caesarean section. The aim of this study is to assess if the use of PC in newborns with 34 weeks of gestation reduced the incidence and severity of respiratory distress (RD). Materials and methods Retrospective case–control study. Review of the clinical registers of all newborns with gestational age between 34 weeks and 0 days and 34 weeks and 6 days, born between January 2003 and December 2007. The newborns included were divided into 2 groups: in Group I (cases) were included the newborns of mothers to whom were administrated PC and in Group II (controls) were included those that had no PC. The newborns admitted to Neonatal Intensive Care Unit were considered as having RD in the first 24 h of life, with tachypnoea, chest retractions or nasal flaring and that needed oxygen therapy. We admitted as criteria of gravity of RD the need for ventilation, duration of oxygen therapy and length of hospital stay. The statistical analysis was performed with the Statistical Package Social Sciences (SPSS), version 16.0. The tests used were Fisher's Exact Test and t-student. It was considered a p value < 0.05 as statistically significant. Results Seventy seven children were included in the study, 31 in Group I and 46 in Group II. There was no statistically significant difference in birth weight and sex between the two groups. The number of caesareans was significantly higher in group I (71% vs. 48%, p = 0.037), but when considering the mode of delivery there was no significant difference in the incidence of RD. There was need for resuscitation only in Group II (17%, p = 0.012). There was no difference concerning the incidence of RD, RDS or TTN, need for ventilation, duration of oxygen therapy or days of hospital stay. Conclusions In our study the use of PC didn't prove to reduce the incidence or severity of RD in newborns with 34 weeks of gestation.
The baby was born at 31 weeks of gestation and stayed in NICU for 38 days. On the 2nd month of life she was brought to our outpatient clinic with the complaints of cough, wheezing and cyanosis and given symptomatic therapy with the diagnosis of viral pneumonia. After discharge, symptoms persisted with the predominance of wheezing, coughing, head tilting and vomiting after feeding. Unilateral hyperlucency and hyperinflation on the chest radiograms were prominent. Bronchoscopy revealed external compression of left main bronchus with a pulsatile structure. Right aortic arch and aberrant left subclavian artery were found on thorax CT angiography. Results On the postnatal 9th month she was operated and a right aortic arch, left subclavian artery and a left ligamentum arteriosum passing along the superior surface of the left main-stem bronchus, causing compression of an underlying malacic trachea segment were detected. After surgery, though respiratory symptoms improved, difficulty in feeding and swallowing solid foods remained. Conclusions Inspiratory stridor, dyspnea, cough, wheezing, recurrent respiratory tract infections and dysphagia are common symptoms during early childhood, but their persistence should alert the general pediatrician for the differential diagnosis of disease states, one of which is vascular ring. The diagnosis of vascular ring is often overlooked due to the relatively rare incidence, the nonspecific nature and high prevalence of these symptoms in the pediatric population especially in former prematures. It is important for the general pediatrician to be familiar with the condition and the appropriate diagnostic workup. doi:10.1016/j.earlhumdev.2008.09.250
Abstract UENPS.235 Bile acids cause lung injury through phospholipase A2 activity enhancement, revertible by exogenous surfactant administration An in vitro study in an extracellular model of aspiration Daniele De Luca⁎a, Angelo Minuccib, Enrico Zeccac, Marco Piastraa, Pietrini Domenicoa, Giorgio Contia, Ettore Domenico Capoluongod a Pediatric Intensive Care Unit, Department of Anaesthesiology and Intensive Care, Catholic University of the Sacred Heart, University Hospital “A. Gemelli”, Roma, Italy b Laboratory of Clinical Molecular Biology, Institute of Biochemistry and Clinical Biochemistry, University Hospital “A. Gemelli”, Catholic University of the Sacred Heart, Roma, Italy c Division of Neonatology, Department of Pediatrics, University Hospital “A. Gemelli”, Catholic University of the Sacred Heart, Roma, Italy d Laboratory of Clinical Molecular Biology, Institute of Biochemistry and Clinical Biochemistry, University Hospital “A. Gemelli”, Roma, Italy
doi:10.1016/j.earlhumdev.2008.09.249 Background and aim
Abstract UENPS.234 Vascular ring: A rare cause of persistent respiratory symptoms in a very low birth weight infant Akin Ilke Mungan, Begum Atasay, Emel Okulu, Saadet Arsan⁎, Tomris Turmen Ankara University, Ankara, Turkey
Secretory phospholipase A2 (sPLA2) has a key role in acute respiratory distress syndrome causing surfactant degradation and increasing inflammation. Bile acids (BA) are supposed to be toxic for the lung and implicated in different lung injury forms, such as meconium aspiration, neonatal bile acid pneumonia and aspiration due to gastroesophageal reflux. We sought to study the interaction sPLA2-BA in an extracellular environment. Materials and methods
Background and aim Bronchopulmonary dysplasia (BPD) and persisting respiratory symptoms flared with gastroesophageal reflux (GER) and viral infections became fairly
We collected bronchoalveolar lavage fluid (BALF) from 23 neonates/ infants aged <6 months with no lung disease and ventilated because of general anaesthesia requirement.