Prophylaxis of intravenous immunoglobulin and acyclovir in perinatal varicella

Maternal chickenpox around the time of delivery can cause severe and even fatal illness in the newborn but an effectively preventive method has not yet been established. We proposed that a combination of intravenous immunoglobulin (IVIG) and acyclovir (ACV) intravenously could effectively prevent perinatal varicella. A group of 24 newborn infants whose mother had developed a varicella rash within 14 days before and after delivery were studied. Some 15 infants whose mothers' rash appeared within 7 days before and 5 days after delivery were categorised as an at-risk group and received IVIG prophylaxis (500 mg/kg) administered soon after birth or post-natal contact either alone or with intravenous acyclovir (5 mg/kg every 8 h) for a total of 5 days starting from 7 days after the onset of maternal rash. Of four infants receiving IVIG alone, two developed clinical varicella. None of ten infants receiving both IVIG and ACV contracted varicella. One infant receiving ACV alone had no varicella vesicles either. Of nine infants in the not at-risk group four had undetectable varicella-zoster virus antibody on admission and developed clinical varicella subsequently. Conclusion The combination of intravenous immunoglobulin given soon after birth and prophylactic acyclovir intravenously administered 7 days after the onset of maternal rash can effectively prevent perinatal varicella. Received: 16 February 2000 / Accepted: 2 August 2000     

Teratomas of the tongue present at birth

A large teratoma of the tongue, present at birth, was successfully removed at 32 h of age. It contained immature neural tissue consisting of a loose neurofibrillary matrix with primitive neurons, rossettes, and papillary tissue. Serum alpha fetoprotein was present at 8 days of age, as reported in some patients with malignant teratomas. Our case, however, had no histologic evidence of malignancy, no serum alpha fetoprotein at 7 months of age, and no recurrence at 1 years. The four glossal tumors reported in the literature, including one with immature neural elements, were all present at birth. In 1 case, another teratoma of the tongue was found 5 months after removal of the congenital one. It may not have been a recurrence, as the mass was located in a different area of the tongue. It appears that congenital teratomas of the tongue do not behave in a malignant fashion, at least in the 5 known cases.     

Teratomas of the Tongue Present at Birth

A large teratoma of the tongue, present at birth, was successfully removed at 32 h of age. It contained immature neural tissue consisting of a loose neurofibrillary matrix with primitive neurons, rossettes, and papillary tissue. Serum alpha fetoprotein was present at 8 days of age, as reported in some patients with malignant teratomas. Our case, however, had no histologic evidence of malignancy, no serum alpha fetoprotein at 7 months of age, and no recurrence at 1 years. The four glossal tumors reported in the literature, including one with immature neural elements, were all present at birth. In 1 case, another teratoma of the tongue was found 5 months after removal of the congenital one. It may not have been a recurrence, as the mass was located in a different area of the tongue. It appears that congenital teratomas of the tongue do not behave in a malignant fashion, at least in the 5 known cases.     

Early nasal continuous positive airway pressure treatment reduces the need for intubation in very low birth weight infants

Nasal continuous positive airway pressure (CPAP) applied shortly after birth is said to be an effective treatment of respiratory distress in very low birth weight infants (VLBW). We tested the hypothesis that the use of early nasal CPAP (applied as soon as signs of respiratory distress occurred, usually within 15 min after birth) reduces the need for intubation, the duration of intermittent mandatory ventilation and the incidence of bronchopulmonary dysplasia. All liveborn VLBW infants (birth weight < 1500 g) admitted to our tertiary neonatal intensive care unit in 1990 (historical controls) and in 1993 (early nasal CPAP group) entered the study. The intubation rate was significantly lower after introduction of nasal CPAP (30% vs 53%, P = 0.016). Median duration of intubation was 4.5 days (interquartile range 3–7 days) before versus 6.0 days (2.8–9 days) after nasal CPAP was introduced (P = 0.73). The incidence of bronchopulmonary dysplasia was not reduced significantly (32% vs 30%, P = 0.94). Survival until discharge was 89.5% before versus 92.9% after introduction of nasal CPAP (P = 0.54). Conclusion Early nasal CPAP is an effective treatment of respiratory distress in VLBW infants, significantly reducing the need for intubation and intermittent mandatory ventilation, without worsening other stan dard measures of neonatal outcome. We found no significant decrease in the incidence of bronchopulmo nary dysplasia. Received: 5 February 1996 and in revised form: 12 September 1996 / Accepted: 23 October 1996     

A case of neonatal intrapericardial teratoma. Clinical and pathological findings

Aim: It is of general agreement that complete surgical removal after birth of intrapericardial fetal teratomas is needed, because of the risk of severe cardiovascular and respiratory distress, related to the mass size, location and secondary pericardial effusion. Histological examination generally shows mature aspect of cells and tissues. Methods: We present a case of grade II immature pericardial teratoma, diagnosed in utero and completely removed after birth. Results: Even surgical removal was complete, histological aspects raised the need of long follow‐up with serial alpha‐fetoprotein determinations. Conclusion: A neonatal grade II immature pericardial teratoma was completely removed after birth. The follow‐up of the patient, until 10 months of life, was good with no recurrence of the disease.     

Renal injury in perinatal hypoxia: ultrasonography and changes in renal function

 We studied 12 hypoxaemic neonates (5 mature newborns, birth weight 2850–4200 g, gestational age 37–41 weeks; and 7 premature newborns, birth weight 770–1850 g, gestational age 27–34 weeks;) with repeated urine and blood chemistry on the 1st and 3rd days of life. Nephrosonographical examinations on the 1st, 3rd and 5–7th days of life were also performed. As controls, 12 healthy infants were examined (gestational age 36–42 weeks; birth weight 2450–4200 g). Hypoxic neonates had higher serum creatinine and blood urea nitrogen levels. Tubular markers also demonstrated renal tubular damage. Neonates in both hypoxic groups were hyperuricaemic and hyperuricosuric, and had higher urinary protein concentrations. All these infants exhibited an increased echogenicity of the renal cortex, and 11/12 showed the same finding in the medullary area. These findings disappeared within 1 week in all infants. Among the 12 healthy control infants, no cortical hyperechogenicity was found and only three of these infants displayed transient medullary renal hyperechogenicity. Conclusion Since the hypoxaemic infants demonstrated greatly increased urinary concentrations of uric acid and protein, we suggest that a temporary precipitation of these two agents may be responsible for the ultrasonographic findings. Circulatory redistribution might play a role in the phenomenon of cortical hyperechogenicity. Received: 23 March 2000 / Accepted: 14 March 2001     

Postnatal body weight curves for infants below 1000 g birth weight receiving early enteral and parenteral nutrition

No body weight curves are available for preterm infants <1000 g birth weight receiving early enteral and parenteral nutrition. Postnatal weight changes of 136 infants with a birth weight <1000 g were analysed retrospectively. Body weight curves for the first 30 days of life were generated for five separate birth weight groups (430–599 g, 600–699 g, 700–799 g, 800–899 g, 900–999 g). All infants had received intravenous glucose and amino acids from day 1 and intravenous lipids from day 2. Enteral feeding was started on day 1. Thus caloric intake (±SD) was advanced to 384 ± 46 kJ/kg per day (92 ± 11 kcal/kg/day) in the 1st week of life. In 136 preterm infants mean postnatal weight loss was 10.1% ± 4.6% of birth weight, birth weight was regained at a mean postnatal age of 11 ± 3.7 days, but significantly earlier (7.8 ± 3.5 days) in the lowest compared to the highest weight group. Mean subsequent weight gain was 15.7 ± 7.2 g/kg per day. This was accomplished by exclusive enteral nutrition from day 20 (median). Conclusion Our body weight curves are more adequate to evaluate growth of preterm infants than older published reference values because they are based on infants treated according to current nutritional standards. Received: 6 July 1997 and in revised form 5 October 1997 / Accepted: 15 October 1997     

Left ventricle dimensions in preterm infants during the first month of life

This study was designed to evaluate left ventricle dimensions in preterm infants during the first month of life, in order to define reference values and their correlation with gestational age, birth weight, gender and baseline. Thirty-five infants, gestational age 25–29 (mean 27.9 ± 1.4) weeks, birth weight 750–1249 (mean 965 ± 206) g, were measured using echocardiography on days 3, 7, 14, 21 and 28 of life. The following dimensions were measured: end-systolic and end-diastolic interventricular septum thickness, end-systolic and end-diastolic left ventricle posterior wall thickness, end-diastolic and end-systolic left ventricle diameter. A progressive and significant increase of all the left ventricle measurements was observed during the first month of life. Left ventricle dimensions at the first scan (Day 3) correlated with birth weight but not with gestational age and gender. The degree of the increase observed during the first month of life was inversely related to the baseline, suggesting that the smaller the left ventricle is at birth, the higher is its postnatal increase toward dimensions similar to those of term infants. Our study gives reference data about left ventricle dimensions of preterm infants during the first month of life and is helpful when making a diagnosis of left ventricular hypertrophy in these subjects. Received: 23 March 2000 / Accepted: 11 October 2000     

Prevalence of congenital heart defects and persistent pulmonary hypertension of the neonate with Down syndrome

The aim of this study was to assess the prevalence of congenital heart defects (CHDs) and persistent pulmonary hypertension of the neonate (PPHN) in children with Down syndrome (DS) and to assess its impact on neonatal factors. It was a prospective study of a birth cohort of children with DS born between 2003 and 2006 registered by the Dutch Paediatric Surveillance Unit (DPSU). A CHD occurred in 43% of 482 children with trisomy 21. Atrioventricular septal defect was found in 54%, ventricular septal defect in 33.3% and patent ductus arteriosus in 5.8%. The incidence of PPHN in DS was 5.2%, which is significantly higher than the general population (p < 0.001). The reported mortality in newborns with DS was overall 3.3% and was still significant higher in children with a CHD versus no CHD (5.8% versus 1.5%) (p = 0.008). The presence of CHD in children with DS had no influence on their birth weight, mean gestational age and Apgar score. In neonates with DS, we found not only a 43% prevalence of CHD, but also a high incidence of PPHN at 5.2%. Early recognition of the cardiac condition of neonates with DS seems justified.     

Hepatitis B vaccination in preterm infants

Preterm infants, especially those with very low birth weight, are at risk of hepatitis B virus infection. They often require invasive diagnostic methods in their first weeks of life, intensive treatment and long-term hospitalisation. Therefore, hepatitis B vaccination is particularly justified in these patients. Our aim was to determine the reaction of preterm children to hepatitis B vaccination. The study comprised 64 preterm children whose birth weight ranged from 700 g to 2460 g (mean 1776.6 g ± 480.4 g) and whose gestational age was between 25 and 36 weeks. A 10 μg dose of the recombinant vaccine Engerix-B (SmithKline Beecham) was given at intervals of 0, 1, 2 and 12 months. In 49.2% of the children vaccination was administered on the 1st day of life, and in the remaining cases between the 2nd and 119th days post delivery. One month after vaccination completion the levels of anti-hepatitis B surface antigen (HBs) antibodies were evaluated. In 98.4% of the vaccinated preterm infants the level of antibodies was >10 mIU/ml. Mean level of anti-HBs antibodies in the group of children with birth weight ≤ 2000 g was 2431.4 mIU/ml, while in those with birth weight >2000 g it was 4803.9 mIU/ml. In children with a birth weight ≤ 1000 g, the mean level of anti-HBs antibodies was significantly lower than in those with birth weight >2000 g. The level of anti-HBs antibodies in children who started vaccination >1st day of life was significantly lower in preterm children with a birth weight ≤ 2000 g than in those with a birth weight >2000 g. Although vaccination was started on the 1st day of his life, one child with birth weight of 2300 g developed a hepatitis B virus infection. One child did not respond to vaccination (anti-HBs < 10 mIU/ml) and in three cases the response was very poor (11–100 mIU/ml). These patients were given a supplementary booster double dose of Engerix B (20 μg). After 1 month the level of anti-HBs antibodies was evaluated again and high values of 657 mIU/ml to 14520 mIU/ml were observed. In the group of children with a birth weight ≤ 1000 g the response to vaccination was weaker as compared to children with a birth weight >2000 g (P < 0.05). In systematic mass vaccination programmes, monitoring of antibody levels is not recommended unless the patient is at risk. However, in extremely preterm infants (< 1000 g at birth), especially after very serious infections, monitoring the level of anti-HBs antibodies after complete immunisation should be considered. In preterm infants who show very low post-vaccination levels of anti-HBs antibodies, stimulation with an additional double booster dose of vaccine gives positive results. Conclusion The majority of preterm infants (98.4%) responded well to hepatitis B vaccination given at intervals of 0, 1, 2 and 12 months and developed a protective level of antibodies. The level of anti-hepatitis B surface antigen antibodies in children with a birth weight >2000 g was higher than in those with a birth weight ≤ 1000 g. Received: 24 February 1998 / Accepted in revised form: 29 September 1998     

Early repair of inguinal hernia in premature babies

Inguinal hernia (IH) is relatively common in premature newborn infants, and the timing of surgical correction is controversial. We studied 40 premature infants who developed an IH and who were initially treated in a neonatal intensive care unit. Birth weight (BW) ranged from 492 to 2,401 g; 21 infants had a BW less than 1,000 g. The weight of the infants at operation ranged from 1,000 to 4,400 g. Twenty-one patients underwent herniotomy within 2 weeks after the diagnosis (short waiting group), in which 1 case of incarceration occurred; 19 waited longer than 2 weeks between diagnosis and surgery (long waiting group). Two cases of strangulation occurred in this latter group, and in 1 of those testicular necrosis occurred. Operation time was analysed in boys with bilateral herniotomy (n = 25): the short waiting group (n = 12) showed a significantly reduced operation time compared to the long waiting group (n = 13). Patients weighing less than 1,000 g at birth (n = 21) had a longer average waiting period for surgery. In the group of male patients with bilateral herniotomy, average operation time was longer in the group weighing less than 1,000 g at birth (n = 13) than in the group over 1,000 g (n = 12). Body weight at surgery did not affect operation time. It is concluded that early hernia repair should be considered in premature infants to avoid operative difficulties and gonadal ischaemia caused by incarceration. Accepted: 3 July 1997     

Determinants of cognitive ability at 7 years: a longitudinal case–control study of children born small-for-gestational age at term

The aim of this study was to identify the determinants of children’s intelligence at 7 years, including pregnancy, postnatal, demographic factors, and small-for-gestational age (SGA) birth at term. Information was collected at birth (n = 871), 1 year (n = 744), 3.5 years (n = 550), and 7 years (n = 591). Approximately half of the children in this study were born SGA (birthweight ≤10th percentile). There was no significant difference found between IQ scores for SGA children compared to AGA children, after adjustment for potential confounders (p = 0.45). Paternal education, developmental delay, and birth order were associated with intelligence scores at 7 years in the multiple regression analysis. This study found no long-term effect of SGA birth at term on intelligence in middle childhood. Potentially modifiable postnatal and demographic factors were found to be strong predictors of intelligence at 7 years.     

Carotenoid supply in breast-fed and formula-fed neonates

Carotenoids have various biological functions including their role as antioxidants. For humans fruits and vegetables are the only source of carotenoids. In the first months breast milk and/or formula preparations are the only nutrition for infants. To study the influence of nutrition on the plasma carotenoid profile in newborns, breast milk, different formula preparations, and the plasma of breast-fed (BF) and formula-fed (FF) newborns were analyzed by high-performance liquid chromatography. The method used allowed β-carotene, α-carotene, lycopene, and β-cryptoxanthine to be detected and all four were found in breast milk. In colostrum carotenoids were up to five times higher than in mature breast milk (P < 0.05). In contrast, not all carotenoids could be found in formula preparations. β-Carotene was detected in four out of eight, and β-cryptoxanthine in three out of eight formula preparations. Lycopene and α-carotene were not detectable in any of the formula preparations. Four formula preparations did not contain any carotenoids. FF infants had different plasma carotenoid profiles compared to BF infants. β-Carotene was significantly lower in FF infants [14 (0–32) μg/l, median and interquartile ranges] than in infants after birth [24 (19–310) μg/l, P < 0.05], and BF infants [32 (22–63) μg/l, P < 0.05]. While newborns after birth had measurable plasma concentrations of lycopene (16 [14–18] μg/l) and of α-carotene [5 (0–8) μg/l), these carotenoids were no longer detectable in FF infants after day 14. Conclusion FF and BF infants show significant biochemical differences in plasma carotenoid concentrations. Received: 6 April 1999 / Accepted: 1 September 1999     

Demographic characteristics of SMA type 1 patients at a tertiary center in Turkey

The aim of this study was to demonstrate demographics of 39 consecutive Spinal Muscular Atrophy (SMA) type 1 patients diagnosed genetically in a tertiary center between June 2006 and June 2009. There was history of consanguineous marriage in 27 (69%) patients. The average patient lifespan was 251 days (30–726 days). The average patient age at diagnosis was 129 days (33–297 days). A statistically significant correlation was found between the age at diagnosis and the lifespan (p = 0.00). No significant correlation was found between the time spent in intensive care and the lifespan (p = 0.43). Routine physical therapy was found to have no significant impact on the lifespan average (p = 0.17). The cause of death in all of our patients was respiratory issues. Genetic counseling was given to 35 families. A second child with SMA was born in three out of the 14 families who declined prenatal diagnosis. Conclusion: A national program is needed in Turkey for SMA prevention and creation of expert teams for the management of these patients.     

Gastric teratoma disclosed by neonatal digestive hemorrhage

A gastric teratoma diagnosed after a gastro-intestinal tract bleeding in a neonate is reported. The endogastric tumor was shown by gastric endoscopy. The tumor was pediculated and a simple tumorectomy was performed, without trouble later. Fifty-three other cases have been found in the literature. Most of them presented with abdominal distension and a palpable mass; diagnosis was always made after surgery and the diagnosis of mature gastric teratoma was confirmed by histological examination. These rare tumors are always of benign nature, but are often revealed by complications. Their frequency is less than 1% of infants teratoma and 85% are found in the first year of life; they are more frequent in males.     

Head and neck teratomas in children

A retrospective review of seven patients with teratomas of the head and neck treated at out center over the past 5 years, which represented 2% of all teratomas (sacrococcygeal, ovarian, and retroperitoneal) seen over the past 20 years, was performed. After investigation to exclude associated anomalies, all but one of the children underwent surgery for removal of the tumour. All excised tumours were subjected to histopathological examination to confirm their teratomatous nature. The patients were followed up at regular intervals for up to 4 years. The patients' ages ranged from newborn to 2.5 years. There were three cervical, two oral, and two skull teratomas. The youngest patient had a cervical teratoma with respiratory compromise, requiring tracheal intubation. All but one patient (skull teratoma) had excision of the tumours with a satisfactory outcome. Histopathological examination of the excised tumours showed mature tissue from the three germinal layers in all specimens. Specific components included glandular epithelium, keratinising epithelium, and muscle fibres. Follow-up did not show any recurrence in the operated children. The three with cervical teratomas had normal levels of thyroid hormone post-operatively. Head and neck teratomas in children are mostly benign lesions amenable to curative excision. In cervical teratomas airway management takes priority. Accepted: 8 November 1999     

Italian multicentre study on retinopathy of prematurity

The aim of this prospective multicentre study was to evaluate the influence of a number of perinatal factors on the development of ROP in high risk preterm infants with gestational age ≤30 weeks. All infants consecutively born in, or transferred to, one of the 14 participating centres from 1 January 1992 through 31 December 1993, who had a gestational age of 30 weeks or less and no congenital anomalies and survived to the age of 6 months, were included in the study. Of the 380 infants with mean ± SD gestational age of 28.4 ± 1.6 weeks (range 23–30 weeks) and birth weight of 1157 ± 335 g (range 485–2480 g) that were eligible for the study, 82 (21.5%) developed ROP stage 1 or 2 and 57 (15%) ROP stage 3 or 3+. Step-wise logistic regression analysis showed that the following factors had a significant predictive value for the development of ROP stage 3 or 3+: gestational age (Odds Ratio (OR)=0.6144 for each increment of 1 week of gestational age), birth weight (OR=0.843 for each increment of 100 g of birth weight), prenatal steroids (OR 4.044 for lacking or incomplete prophylaxis), RDS (OR 2.294), oxygen dependency at 60 days (OR 2.085), necrotising enterocolitis (OR 2.597). Conclusion This study confirms the role of prematurity, low birth weight and RDS in the pathogenesis of ROP, and emphasises the importance of prenatal steroid prophylaxis of RDS in very preterm infants. Furthermore, our data suggest that infants with oxygen dependency at 60 days or necrotising enterocolitis are at very high risk of developing ROP. Received: 29 September 1996 and in revised form: 28 January 1997 / Accepted: 1 April 1997     

Outcome of ventilated infants born at term without major congenital abnormalities

The longer-term outcome of term-born infants without congenital anomalies requiring ventilation in the first 24 h after birth has rarely been reported. Our aims were to determine the mortality and long-term morbidity of such infants and identify risk factors for adverse outcome. The outcomes of 43 of 45 infants born at term consecutively requiring mechanical ventilation were reviewed. The infants had: meconium aspiration syndrome (n = 11), hypoxic ischaemic encephalopathy (HIE) (n = 11), respiratory depression (n = 12), sepsis (n = 5), persistent pulmonary hypertension of the newborn (n = 3) and middle cerebral artery infarction (n = 1). Eleven infants developed seizures (26%), 13 (30%) had abnormal electroencephalograms and 11 (26%) had abnormal MRI scans; 26% had an adverse outcome: six died, and five had severe neurodisability at 2 years. The infants with congenital toxoplasmosis and a middle cerebral artery infarction were excluded from the prediction analysis. In the remaining 41 patients, requirement for anticonvulsants (relative risk, RR = 4.44, 95% CI = 1.48 to 12.70; p = 0.014) and prolonged ventilation (longer than 3 days) (RR 4.83, 95% CI 1.51 to 15.64) predicted adverse outcome. Infants with HIE had an increased risk of adverse outcome (relative risk 5.45, 95% CI 1.01 to 33.85), but an adverse outcome occurred in infants with other diagnoses. Conclusion: Mortality and neurodisability at follow-up were common in infants born at term without major congenital anomalies who required mechanical ventilation in the first 24 h after birth, particularly in those who developed seizures requiring treatment and prolonged ventilation.     

Intraosseus teratoma of the iliac bone

We present a 4-year-old child with a large iliac bone mass incidentally discovered in a plain abdominal radiograph. The pathological examination revealed a benign mature teratoma. To the best of our knowledge, the occurrence of intraosseous mature teratoma has not been previously reported. The child had had an immature teratoma of the neck discovered in a fetal ultrasound, and resected on day 6 of life. The neck teratoma recurred twice, at 16 months and at 3.5 years of age. In these two recurrences the lesion appeared progressively more mature. At the time of discovery of the iliac bone teratoma there was no evidence of residual neck disease. The radiological and pathological characteristics, differential diagnosis, and clinical course are discussed. Received: 2 November 1999/Accepted: 9 November 1999     

Total serum bilirubin levels during the first 2 days of life and subsequent neonatal morbidity in very low birth weight infants: a retrospective review

To determine the relationship between total serum bilirubin (TSB) during the first 2 days of life and subsequent neonatal morbidity in very low birth weight (VLBW, less than 1500 g) infants. We performed a prospective study of 582 VLBW infants born between July 1, 2005 and December 31, 2009. TSB was measured in umbilical cord blood (UCB), at 24 and 48 h after birth. Demographic and clinical characteristics of infants in hospital were recorded. The interaction between TSB variables during the first 48 h of life and subsequent neonatal morbidity were assessed in logistic regression analyses adjusted for multiple risk factors. It was found that TSB in UCB was in a negative correlation with occurrence of respiratory distress syndrome (RDS) [OR 0.626, 95% confidence interval (95% CI): 0.446–0.879, p = 0.007], and there was also a negative correlation between TSB in UCB and occurrence of intraventricular hemorrhage (IVH) [OR 0.695, 95% CI 0.826–0.981, p = 0.020]. However, TSB in UCB positively correlated with hyperbilirubinemia [OR 2.471, 95% CI 1.326–3.551, p = 0.012], and TSB at 24 h after birth was also in a positive correlation with early onset sepsis (EOS) [OR 1.299, 95% CI 1.067–1.582, p = 0.011]. VLBW infants with low TSB levels in UCB were more likely to develop RDS and IVH, and those with low TSB levels in UCB were less likely to develop hyperbilirubinemia. Infants with high TSB levels at 24 h after birth were more likely to develop EOS. The protective effect of raised TSB in UCB with respect to RDS and IVH warrants further investigation.