[A 14-day-old boy with jaundice and apnoea]. / 14 dager gammel gutt med gulsott og apnéanfall.

We describe an infant who was readmitted from home at 14 days of age with jaundice and a history of apnoea and episodes of retrocollis/opisthotonos. He had been only mildly jaundiced on discharge from the maternity clinic at 2 days of age. The total serum bilirubin (TSB) on admission was 542 µmol/L, and the infant was treated intensively with triple phototherapy and exchange transfusion. In contrast to what is recommended in Norwegian national guidelines for management of neonatal jaundice, the parents had apparently neither received oral nor written information about jaundice and its follow-up at the time of discharge from maternity. They therefore contacted their child healthcare centre when they had questions about jaundice, though the national guidelines specifically state that follow-up for neonatal jaundice during the first 2 weeks of life is the responsibility of the birth hospital. Inappropriate advice resulted in delayed referral, and the child has been diagnosed with chronic kernicterus, probably the first such case in Norway since national guidelines were formalised in 2006. Genetic work-up disclosed compound heterozygosity for Crigler-Najjar syndrome type I, to the best of our knowledge the first instance of this disorder ever to have been diagnosed in Norway. The incidence of kernicterus is Norway is much lower than in other industrialised countries. This is most likely due to national guidelines for management of neonatal jaundice, which place the responsibility for management and follow-up of jaundice with the birth hospital during the crucial first 2 weeks of life. This case report reminds us that tragedies may occur when guidelines are disregarded.

First year growth among very low birth weight infants.

AIM: The aim of this study was to describe first-year growth among very low birth weight infants and the effect of growth restriction at hospital discharge on first year growth. METHOD: Anthropometric measures and background information for 118 very low birth weight infants were collected from medical records. Z-scores were calculated based on recent Norwegian growth references. RESULTS: Significant catch-up growth for weight and length was observed during the first year with mean z-score change (SD) of 0.40 (1.05) and 1.01 (1.25) respectively. However, the very low birth weight infants remained lighter and shorter than full-term peers until 12 months corrected age with mean z-score of -0.93 (1.09) and -0.48 (1.06) respectively. Head circumference followed a normal growth pattern after 2 months. Infants discharged from hospital as growth restricted had increased catch-up in weight and length, but remained smaller than infants not subjected to early growth restriction and full-term peers. Multiple regression showed that weight below the 10th percentile at discharge is important for weight and length during the first year of life. CONCLUSION: Very low birth weight infants showed catch-up growth during the first year, but their weight and length remained less than full-term peers. Growth deficiencies were more pronounced among infants subjected to early growth restriction, despite increased catch-up growth.

The impact of shunt size on lung function in infants with univentricular heart physiology.

OBJECTIVES: To assess the relationship that shunt size, blood gases, and radiologic findings has on respiratory function in infants with univentricular heart physiology. SETTING: Cardiac catheter laboratory at Rikshospitalet University Hospital, Norway. PATIENTS: Fifteen infants with univentricular heart physiology admitted for cardiac catheterization. MEASUREMENTS: Lung function was measured by a fixed-orifice differential pressure flow sensor and mainstream volumetric capnography in 15 infants with univentricular heart arrangements during routine invasive assessment before the bidirectional cavopulmonary connection. Blood gases were measured from the indwelling catheters. Chest radiographs were assessed for heart size and pulmonary vasculature. Shunt size was assessed angiographically. MAIN RESULTS: Respiratory compliance was reduced in patients with a large surgical systemic-pulmonary arterial shunt (r = -0.67, r = 0.45, p = 0.03). Respiratory resistance was higher with increased heart size (r = 0.72, r = 0.52, p = 0.004). There was no association between arterial and end-tidal CO2 values. The arterial to end-tidal CO2 difference had an inverse relationship with the pulmonary to systemic shunt ratio (r = -0.38, r = 0.14, p = 0.015). CONCLUSION: A large surgical shunt size is related to stiffer lungs and a large heart is associated with a higher respiratory resistance. During mechanical ventilation of patients with univentricular heart physiology the end-tidal CO2 may be an unreliable substitute for arterial CO2 before the bidirectional cavopulmonary connection. We found a relationship between a decreased pulmonary to systemic shunt ratio and an increased arterial to end-tidal CO2 difference. This may indicate that a reason for the unreliability of end-tidal CO2 is an impaired gas exchange partially due to pulmonary hypoperfusion.

Donor milk banking and breastfeeding in Norway.

Milk banks in Norway have a long tradition of using raw milk. This is a practice the authors hope to continue as they see it as the best choice until a child's own mothers' milk production is sufficient. Not only will the premature babies benefit from having milk from the bank, but if a mother, for any reason, can not supply her baby while it's in the hospital her baby should be offered milk from a bank. In Norway, with a high breastfeeding rate this can be done at many hospitals.

Differing lung function development in infants with univentricular hearts compared with healthy infants.

AIM: To compare the difference in lung function development of healthy controls and patients with univentricular hearts from birth prior to surgery and during the first year of life when cardiac shunt procedures and the cavopulmonary connection are required. METHODS: Tidal flow-volume measurements and single-occlusion tests were performed from birth serially up to 18 months of age on 28 unsedated spontaneously breathing infants with univentricular hearts and 58 healthy control infants. RESULTS: Infants with univentricular heart physiology had low tidal volumes, low compliance of the respiratory system and high respiratory rate at birth, which over time normalized, whereas the peak expiratory flow increased during the study period. The lung function measured at birth was predictive of later lung function measurements. CONCLUSION: The pattern of lung function development is different in the patients with univentricular hearts compared to healthy controls. Lung function measured at birth is predictive of later lung function.

Neonatal Morbidity and 1-Year Survival of Extremely Preterm Infants.

OBJECTIVE: To determine 1-year survival and major neonatal morbidities (intracranial hemorrhage grade >2, cystic periventricular leukomalacia, retinopathy of prematurity grade >2, necrotizing enterocolitis, severe bronchopulmonary dysplasia) among extremely preterm infants in Norway in 2013-2014, and to compare the results to the first Norwegian Extreme Prematurity Study 1999-2000 and similar contemporary European population-based studies. METHODS: Population-based study of all infants born at 22 through 26 weeks' gestation in Norway in 2013-2014. Prospectively collected data were obtained by linking data in the Norwegian Neonatal Network to the Medical Birth Registry of Norway. RESULTS: Of 420 infants (incidence 3.5 per 1000 births), 145 were stillborn (34.5%), 275 were live-born (82.3% of the 334 fetuses alive at admission for obstetrical care), and 251 (91.3% of live-born infants) were admitted to a neonatal unit. The survival among live-born infants was 18% at 22 weeks, 29% at 23 weeks, 56% at 24 weeks, 84% at 25 weeks and 90% at 26 weeks (for each week increment in gestational age: odds ratio 3.3; 95% confidence interval, 2.4-4.4). Among infants surviving to 1 year of age, major neonatal morbidity was diagnosed in 55%. Decreasing gestational age was moderately associated with rates of major morbidity (odds ratio 1.6; 95% confidence interval, 1.2-2.2). CONCLUSIONS: Compared to the previous 1999-2000 cohort, the rate of stillbirth before admission to an obstetrical unit increased, whereas the survival rate among live born infants was similar in our 2013-2014 cohort. Neonatal morbidity rates remain high among extremely preterm infants.

Improved Visual Perception in Very Low Birth Weight Infants on Enhanced Nutrient Supply.

BACKGROUND: Optimal nutrient supply to very low birth weight (VLBW: BW <1,500 g) infants is important for growth and neurodevelopment. Growth restriction is common among these infants and may be associated with neurocognitive impairments. OBJECTIVES: To compare an enhanced nutrient supply to a routine supply given to VLBW infants and to evaluate the effects on visual perception of global form and motion measured by visual event-related potentials (VERP). METHODS: A total of 50 VLBW infants were randomized to an intervention group that received an increased supply of energy, protein, fat, essential fatty acids, and vitamin A or a control group that received standard nutritional care. At 5 months' corrected age the infants were examined using VERP to investigate the responses to global form and motion. VERP were analysed at the first (f1) and third (f3) harmonics of the stimulus frequency. RESULTS: Data from 31 subjects were eligible for analysis. The motion VERP responses for the f1 and f3 components were stronger in the area near the posterior midline region in the intervention group compared to the controls in the group analyses (p = 0.02 and p = 0.001, respectively). CONCLUSION: The results showed a more consistent response to global motion among infants receiving enhanced nutrition. The intervention may have improved visual perception of global motion.

[Nutrition for preterm infants]. / Ernaering av premature barn.

BACKGROUND: Each year about 630 infants are born with very low birth weight (below 1500 g) in Norway. In spite of an increased survival rate over the past 30 years, many challenges remain in the treatment of premature infants; their nutritional need is an important aspect. MATERIAL AND METHODS: This review is based on searches in the Medline database. RESULTS AND INTERPRETATION: Human milk is the first choice for premature babies in Norway. The beneficial effects of human milk for premature babies are well documented, but unfortified human milk does not meet the nutritional needs of very low birth weight infants. Infants fed human milk grow slower than babies fed preterm formula. Fortification is necessary. There are many unsolved problems concerning nutrition for premature babies. What is the best rate of advancement in parenteral and enteral nutrition? How to improve the energy and protein fortification of human milk? Does human milk meet the need for long-chain polyunsaturated fatty acids or is a supplement indicated? What are the optimal doses of vitamin and mineral supplements? What is the recommended nutrition after discharge? More clinical trials are needed to establish evidence-based practice.

Improved cognitive development among preterm infants attributable to early supplementation of human milk with docosahexaenoic acid and arachidonic acid.

OBJECTIVE: The objective of our study was to evaluate the effect of supplementation with docosahexaenoic acid and arachidonic acid for human milk-fed preterm infants. The primary end point was cognitive development at 6 months of age. METHODS: The study was a randomized, double-blind, placebo-controlled study among 141 infants with birth weights of <1500 g. The intervention with 32 mg of docosahexaenoic acid and 31 mg of arachidonic acid per 100 mL of human milk started 1 week after birth and lasted until discharge from the hospital (on average, 9 weeks). Cognitive development was evaluated at 6 months of age by using the Ages and Stages Questionnaire and event-related potentials, a measure of brain correlates related to recognition memory. RESULTS: There was no difference in adverse events or growth between the 2 groups. At the 6-month follow-up evaluation, the intervention group performed better on the problem-solving subscore, compared with the control group (53.4 vs 49.5 points). There was also a nonsignificant higher total score (221 vs 215 points). The event-related potential data revealed that infants in the intervention group had significantly lower responses after the standard image, compared with the control group (8.6 vs 13.2). There was no difference in responses to novel images. CONCLUSIONS: Supplementation with docosahexaenoic acid and arachidonic acid for very preterm infants fed human milk in the early neonatal period was associated with better recognition memory and higher problem-solving scores at 6 months.

Preoperative lung function in newborn infants with univentricular hearts compared with healthy controls.

AIM: To measure the difference in lung function between newborns with univentricular hearts and healthy controls and study associations between lung function and pulmonary blood flow and pulmonary vasculature markings. METHOD: Tidal flow-volume measurements and single occlusion tests were performed before surgery on 25 unsedated spontaneously breathing newborns with univentricular hearts recruited over a 3-year period. Seventy-five healthy control infants were measured. Pulmonary blood flow was graded according to the haemodynamic effect of the echocardiographically defined anatomy of the heart defect. Pulmonary vasculature was graded according to radiological markings. RESULTS: The infants with univentricular hearts had a lower mean tidal volume of 4.7 ml (CI 2.3, 7.2, p < 0.001) (n = 24) and a lower mean compliance of the respiratory system of 12.7 ml/kPa (CI 4.6, 20.8, p = 0.004) (n = 14). Pulmonary blood flow grading was associated with respiratory rate (r = 0.53, p < 0.001), tidal volume (r =-0.48, p < 0.001), compliance (r =-0.55, p < 0.001) and resistance (r =-0.31, p = 0.043). Pulmonary vasculature grading was associated with compliance (r =-0.68, p = 0.006), resistance (r =-0.69, p = 0.007) and the time constant (r =-0.62, p = 0.042). CONCLUSION: Newborns with univentricular hearts have reduced tidal volumes and reduced compliance of the respiratory system. The lung function abnormalities are associated with the degree of pulmonary blood flow and pulmonary vasculature markings.

A screening and intervention program aimed to reduce mortality and serious morbidity associated with severe neonatal alloimmune thrombocytopenia.

The study's objective was to identify HPA 1a-negative women and to offer them an intervention program aimed to reduce morbidity and mortality of neonatal alloimmune thrombocytopenia (NAIT). HPA 1 typing was performed in 100 448 pregnant women. The HPA 1a-negative women were screened for anti-HPA 1a. In immunized women, delivery was performed by Cesarean section 2 to 4 weeks prior to term, with platelets from HPA 1a-negative donors reserved for immediate transfusion if petechiae were present and/or if platelet count was less than 35 x 10(9)/L. Of the women screened, 2.1% were HPA 1a negative, and anti-HPA 1a was detected in 10.6% of these. One hundred seventy pregnancies were managed according to the intervention program, resulting in 161 HPA 1a-positive children. Of these, 55 had severe thrombocytopenia (< 50 x 10(9)/L), including 2 with intracranial hemorrhage (ICH). One woman with a twin pregnancy missed the follow-up and had one stillborn and one severely thrombocytopenic live child. In 15 previous prospective studies (136 814 women) there were 51 cases of severe NAIT (3 intrauterine deaths and 7 with ICH). Acknowledging the limitation of comparing with historic controls, implementation of our screening and intervention program seemed to reduce the number of cases of severe NAIT-related complications from 10 of 51 to 3 of 57.

Reduced pulmonary function in children with the Fontan circulation affects their exercise capacity.

Most children with functionally univentricular hearts nowadays are treated surgically by creating a total cavopulmonary connection. In the resulting Fontan circulation, the venous return and the pulmonary arterial bed are coupled in series, bypassing the heart. This gives the potential for interaction between the abnormal circulation and function of the lungs. In this study, we investigated the pattern of impairment of pulmonary function, and its relation to decreased exercise capacity. We performed spirometry in 33 (85 percent) of 39 eligible Norwegian children, aged from 8 to 16, with a total cavopulmonary connection, along with whole body plethysmography, the carbon monoxide single breath test, and a peak treadmill exercise test. The single breath test showed a mean corrected diffusing capacity of 66.5 percent of predicted, giving a z score of minus 2.88. The mean residual volume measured by whole body plethysmography was 146.8 percent, equivalent to a z score of 2.46, whereas the mean residual volume measured by the single breath test was 102.4 percent of predicted, this being the same as a z score of 0.43. The mean peak treadmill exercise test was 70.0 percent of predicted, equivalent with a z score of minus 3.07. Mean forced vital capacity was 85.7 percent of predicted, the equivalent z score being minus 0.92. Lung function correlated with the peak treadmill exercise test. We have shown, therefore, that children with the Fontan circulation have reduced diffusing capacity, possibly caused by the abnormal circulation through the lungs. The difference between residual volume measured by plethysmography and the single breath test implies trapping of air. The correlation of parameters for lung function with peak consumption of oxygen during exercise indicates that the abnormalities of pulmonary function may affect physical capacity.