Moral distress in the neonatal intensive care unit: an Italian study.

OBJECTIVE: The objective of this study was to evaluate the frequency, the intensity and the level of moral distress experienced by nurses working in neonatal intensive care units (NICUs). STUDY DESIGN: We conducted a cross-sectional questionnaire survey involving 472 nurses working in 15 level III NICUs. Frequency, intensity and level of moral distress was evaluated using a modified version of Moral Distress Scale Neonatal-Pediatric Version. Socio-demographic data were also collected. RESULT: Four hundred six nurses completed the study material indicating a low-to-moderate experience of moral distress. The situations receiving the highest scores for frequency, intensity and level of moral distress related to the initiation of extensive life-saving actions and participation to the care of ventilator-dependent child. No difference in the mean scores of moral distress was found according to the socio-demographic characteristics investigated. CONCLUSION: The present study provides further insight into the moral distress experienced by nurses working in Italian NICUs.

Boys who are born preterm show a relative lack of fat-free mass at 5 years of age compared to their peers.

AIM: Prematurity is associated with features of metabolic syndrome in young adulthood. We investigated the body composition and blood pressure of children born preterm. METHODS: A longitudinal, observational study was conducted with preterm infants who had a birth weight of <1500 g and a gestational age of <32 weeks. Growth and body composition were assessed by air displacement plethysmography at term equivalent age and at school age and were compared to those of 61 healthy, term breastfed subjects. RESULTS: A total of 63 preterm infants were enrolled. At term equivalent age, growth and fat-free mass were lower in preterm infants than in term newborns, but fat mass was higher. At 5 years of age, children born preterm were still lighter and shorter than children born at term. When the results were analysed by gender, the fat-free mass index was lower in boys born preterm than in their peers (12.1 ± 1.1 versus 13.0 ± 1.0 kg/h(2) p < 0.005), whereas no difference was detected among girls. Diastolic blood pressure was higher in children born preterm than in children born at term (61.14 ± 7.8 vs 56.69 ± 8.2 mmHg, p = 0.009). CONCLUSION: Boys born preterm showed a relative lack of fat-free mass at school age compared to their peers.

Consequences of prematurity on adult morbidities.

Early life events play a key role in the development of adult diseases. Survival is promoted by the developmental adaptation to environment although advantage in the short term implies a long-term cost with regard to the development of adult morbidities. This is particularly true for the infant born prematurely as preterm birth is accompanied by a disruption of the normal organogenesis of multiple organ systems. This review will examine the effect of prematurity on the development of cardiovascular, kidney, respiratory and metabolic diseases in later life in addition to the neurodevelopment disabilities associated with preterm birth.

[Prevalence and duration of breastfeeding during the first six months of life: factors affecting an early cessation]. / Prevalenza e durata dell'allattamento al seno nei primi sei mesi di vita e fattori che influenzano.

OBJECTIVES: To assess the mean duration, prevalence and reasons that lead to an early cessation of breastfeeding in a group of healthy term infants in the first six months of life. METHODS: Prospective, observational study. One-hundred Caucasian, non smoking mothers, that intended to breastfeed for at least 12 weeks, were enrolled. Information on anthropometric parameters, type of delivery, socio-demographic characteristics, mode of feeding and reasons for stopping breastfeeding have been obtained through three different questionnaires (submitted at enrollment, on the 7th day, at 1, 2, 3 and 6 months). RESULTS: Exclusive breastfeeding gradually decreased from the 7th day to the 6th month of life. Most of the mothers stopped breastfeeding during the first month and a half or after 3 months and a half. Two percent of the mothers stopped on the 7th day whereas at 6 months the percentage of cessation was 14%. The cumulative percentage of interruption at 6th month was 45%. Maternal factors, like sore nipples or delayed onset of lactation, were the most frequent reasons that led to an early cessation, while during the following months inadequate breast milk and latch-on problems were predominant. On the other hand, attending a pre-natal course or having a previous successful breastfeeding experience were significantly associated with a long-lasting breastfeeding. CONCLUSIONS: Promotion of breastfeeding during the prenatal course and a better support for lactation management during the first months seem to be the areas where more efforts are needed to implement breastfeeding rates.

[Growth and body composition in the premature infant]. / Crescita e composizione corporea nel prematuro.

Great interest has focused recently on the relationship between early nutrition, growth and subsequent health. Indeed, several studies have demonstrated that early life growth patterns exert programming effects on disease risk in later life, highlighting the key role played by early nutrition. Body composition, an index of quality of growth, is one of the factors apparently involved into this "programming" process. The American Academy of Pediatrics recommends to supply adequate amounts of nutrients so that growth and body composition of the preterm infant approximates that of the intrauterine fetus at the same gestational age. However, in clinical practice, the achievement of intrauterine growth rate and body composition is difficult Indeed, most preterm infants experience a significant postnatal growth retardation which can be accompanied by an increased and/or altered adiposity. The estimation of the dynamic features of body composition changes, in order to evaluate the quality, in addition to t he amount of weight gain plays a major role in the nutritional follow-up of preterm infants. Certainly, monitoring the somatic growth and the development of body composition in early infancy represents an extremely important clinical tool in the individualization of the nutritional management, the prevention and/or the recovery from the postnatal retardation and the identification of the subjects at high risk for developing the metabolic syndrome in young adulthood.

[Early nutrition: the role of genetics and epigenetics]. / Nutrizione nelle prime epoche della vita: ruolo della genetica e dell' epigenetica.

Many adult diseases seem to be associated with early nutrition and the subsequent growth pattern. Epidemiological studies hypotized that babies with intrauterine and/or neonatal growth retardation may be at greater risk of metabolic syndrome later in life. According to the Barker's "thrifty phenotype hypotesis" early malnutrition, whereas inducing physiological compensation by the promotion of early survival, appears to confer greater susceptibility to adults diseases. Epigenetics, that is the interindividual variation in DNA methylation patterns and chromatin remodelling, provide a potential explanation for how environmental factors can modify the risk for development of many common diseases. Beginning from animal models, many studies concerning early nutrition, epigenetic modifications and genes expression have been carried out. Maternal undernutrition during pregnancy, especially in the peri-implantation period, not only causes a prolonged growth retardation but also modifies the programming of biochemical mechanisms related to endocrine-metabolic control. Human studies have demonstrated the role played by IGF-1 as indicator of nutritional status and fetal/postnatal growth retardation. It has been reported that alterations in IGF axis, which predispose to adults diseases, may be due to an alterated epigenetic regulation that can modify IGF expression. Despite the critical inter-relation between early nutrition, growth, development, and subsequent health, there are few data on the influence of early nutrition on the modifications of the epigenetic gear. Furthermore it is hoped for a bigger attention to the early nutrition to prevent the development of diseases later in life.

Adiposity in small for gestational age preterm infants assessed at term equivalent age.

OBJECTIVE: Infants classified as small for gestational age are considered to have developed under adverse intrauterine conditions that lead to lack of fat mass accretion. The aim of this study was to test the null hypothesis that the fat mass in preterm small for gestational age infants assessed at term equivalent age was not different from that of full-term small for gestational age newborns. DESIGN: Observational study. SETTING: Northern Italy. PATIENTS: 67 small for gestational age preterm infants and 132 small for gestational age full-term newborns. MAIN OUTCOME MEASURES: Growth and body composition, assessed by means of a paediatric air displacement plethysmography system, were measured at term equivalent age in the preterm infants and on the third day of life in the full-term newborns. RESULTS: The mean (SD) gestational age of preterm infants was 30.6 (2.3) weeks and their mean (SD) birth weight was 1140 (237) g. At assessment weight was not different between the preterm and full-term infants, whereas the percentage of total body fat mass was higher in the preterm infants (14.3% (SD 4.7%) vs 5.8% (SD 3.5%), p<0.005). CONCLUSIONS: Preterm infants, born small for gestational age, appear to be at risk for increased adiposity, which is a risk factor for the development of the metabolic syndrome.

Influence of protein and energy intakes on body composition of formula-fed preterm infants after term.

The aim of the present study was to evaluate changes in body composition in 48 preterm infants in relation to protein and energy intakes from term up to 3 months of corrected age, using air displacement plethysmography. Protein intake (grams per kilogram per day) was negatively associated with percentage of fat mass at 1 month of corrected age. The high-protein-intake group showed greater gain in lean body mass gain than did the low-protein-intake group. This finding suggests that during the first month of corrected age, high protein intake results in a significantly different weight gain composition.

Quantitative ultrasound and dual-energy x ray absorptiometry in bone status assessment of ex-preterm infants.

Preterm infants are at increased risk of low bone mass. Quantitative ultrasound (QUS) is a non-invasive method for assessment of bone status. The application of QUS in a cohort of ex-preterm infants was investigated. This study suggests that QUS cannot be used as a surrogate for dual-energy x ray absorptiometry.

Usefulness of an assessment score to predict early stopping of exclusive breast-feeding.

Exclusive breast-feeding is recommended during the first 6 months of life. Use of simple indicators to predict early stopping of exclusive breast-feeding may be desirable in clinical practice. A breast-feeding assessment evaluated at discharge and including maternal age, previous breast-feeding experience, latching difficulty, and breast-feeding interval could be useful.

The effects of an early developmental mother-child intervention program on neurodevelopment outcome in very low birth weight infants: a pilot study.

BACKGROUND: Several studies report increased minor neurodevelopment dysfunctions in children born very low birth weight (VLBW). Usefulness of preventive early intervention programs to improve neurodevelopment outcome of VLBW infants is still under investigation. AIMS: To evaluate the effects of an early post-discharge developmental mother-child intervention program on neurodevelopment outcome at 36 months in VLBW infants. STUDY DESIGN: Prospective study. SUBJECTS: 36 VLBW infants ([mean (S.D.)] birthweight=864 g (204 g); gestational age=27.9 weeks (2.4 weeks)), consecutively born January-August 2001, randomized in intervention and control groups. OUTCOME MEASURES: Neurodevelopment assessment at 36 months of chronological age with use of the Griffiths Mental Development Scale and related subscales. RESULTS: At 36 months of chronological age, as compared to controls, children in intervention group exhibited higher scores in personal-social subscales ([mean (S.D.)]=101.4 (9.3) vs. 92.9 (12.1), P=0.02), eye-hand coordination (92.7 (4.5) vs. 87.1 (9.9), P=0.041), practical reasoning (98.6 (8.2) vs. 89.4 (10.1), P=0.01). Development Scale were 97.6 (5.5) and 92.4 (9.9), respectively, in intervention and control groups (P=0.074). CONCLUSIONS: Early post-discharge developmental mother-child intervention program may have a positive effect on later neurodevelopment outcome of VLBW children.

Usefulness of an early neurofunctional assessment in predicting neurodevelopmental outcome in very low birthweight infants.

OBJECTIVE: To evaluate whether early neurofunctional assessment may be useful in predicting neurodevelopmental outcome in children of very low birth weight (VLBW). DESIGN: Observational longitudinal study. SETTINGS: Northern Italy. PATIENTS: A total of 250 VLBW children (129 boys, 121 girls) born consecutively 1996-1999. MAIN OUTCOME MEASURES: Neurodevelopment at 36 months of chronological age, classified in accordance with the classification of Tardieu and the International classification of functioning. RESULTS: Of the infants exhibiting normal neurodevelopment (n = 183) or major dysfunction (n = 17) at 3 months of corrected age, 72% and 94% respectively did not change their score during the study. Minor dysfunctions at 3 months of corrected age were transient in 17 (34%) children. After adjustment for neonatal variables, neurodevelopment at 3 months of corrected age remained predictive of dysfunction at 36 months (odds ratio = 4.33, 95% confidence interval 2.05 to 9.12). If the results for the normal and minor dysfunction groups were pooled, the predictive qualities of the 3 month neurofunctional assessment were: sensitivity 0.5, specificity 0.99, positive predictive value 0.94, negative predictive value 0.93. CONCLUSION: Early neurofunctional evaluation may be useful in predicting later neurodevelopmental outcome in VLBW children.

[Disability in childhood. Care and professional training regarding particularly the problems currently seen in long-term control of newborns at risk]. / La disabilita' in eta' pediatrica. Assistenza e formazione professionale con particolare riguardo ai problemi attualmente osservati nel controllo a distanza del neonato a rischio.

Children with disability are a heterogeneous population distributed between complex conditions including physical disorders, psychomotor and neurodevelopment disorders, social role and school activities limitations. Prenatal, perinatal and neonatal diseases together with disabling chronic conditions may cause childhood disability. Data from literature show a prevalence of childhood disability ranging from 5 to 20% with a wide range of severity level. The social impact of childhood disability is extremely relevant. Children with disabilities frequently require health care services and special education. VLBW (very low birth weight infants, birth weight <1500 gr) have major disabilities (10-15%) while minor disabilities can be found in 20-25% of these subjects. The first step stays in medical prevention but also an oriented training toward these problems for doctors both in pre and post-graduate formation can play a significant role. Support of society in order to understand the complexity and heterogeneity of childhood disability, through social care services, is mandatory. These measures, apparently costly, if correctly done, will be helpful for both the individual subject and the whole society, through a reduction of the disability problems and the final economy cost impact.

Ramipril treatment in a patient with glycogen storage disease I non-A.

We studied the effect of ramipril on proteinuria and mild hypertension in a 21-year-old patient affected by glycogen storage disease type I non-A. After few months of therapy we obtained a decrease in total urine protein excretion that later re-increased in spite of the high dose of ACE inhibitor. Even if ACE inhibitors are the only effective therapy for GSD I nephropathy, further studies are requested.

Sonographic findings in type I glycogen storage disease.

PURPOSE: The aim of this study was to document the sonographic appearance and dimensions of the liver and spleen in patients affected by type I glycogen storage disease and to correlate those findings with laboratory data to evaluate the potential role of sonography in diagnosing that disease. METHODS: Fourteen patients (age range, 3-26 years; 10 patients younger than 18 years) with type I glycogen storage disease proved by liver biopsy were studied prospectively with gray-scale sonography, color Doppler sonography, and spectral analysis. The liver, kidneys, spleen, portal system, hepatic veins, and hepatic arteries were evaluated. Laboratory data were correlated with sonographic findings. RESULTS: In 13 (93%), of 14 patients, the liver was enlarged, and in 11 patients (79%), hepatic echogenicity was increased. In 9 patients (64%), both kidneys were enlarged, and in 6 cases (43%), the spleen was enlarged. In all patients, flow in the portal, splenic, and superior mesenteric veins was hepatopetal, and flow in the hepatic veins was triphasic. In 5 patients (36%), both triglyceride and total cholesterol levels were higher than normal. No focal hepatic lesions were identified. Analysis found no significant association between sonographic findings and laboratory data. CONCLUSIONS: The most frequent sonographic findings in patients with type I glycogen storage disease were hepatomegaly, increased hepatic echogenicity, and enlarged kidneys. Sonography may help in the diagnosis of type I glycogen storage disease, but a liver biopsy is required for a definitive diagnosis.

Growth pattern of breastfed and nonbreastfed infants with atopic dermatitis in the first year of life.

OBJECTIVE: The growth of infants with atopic dermatitis (AD) has been poorly investigated based on the early type of feeding. The aim of this study was to assess the growth pattern of AD infants during the first 12 months of life in comparison to healthy infants, according to the early type of feeding (breastfed or nonbreastfed). METHODS: Fifty-five term AD infants (36 breastfed and 19 nonbreastfed) and 114 term healthy infants (58 breastfed and 56 nonbreastfed) were evaluated by standardized growth indices (z scores; National Center for Health Statistics-World Health Organization data) through the first 12 months of life. RESULTS: No difference was found between AD and healthy groups at birth. In AD infants, weight (WA) and length (LA) z scores decreased with age and were significantly lower, compared with healthy infants from the second month of age onward. The difference of mean z scores between AD and healthy infants at 12 months of age was -.69 (95% confidence interval [CI]: -1.00 to -.38) for WA and -.67 (95% CI: -.98 to -.36) for LA. The growth pattern of AD infants was not influenced by the early type of feeding, whereas in the 6- to 12-month period, the delay in growth was more pronounced in patients with more severe dermatitis. CONCLUSIONS: In the first year of life, AD infants show a progressive impairment in growth irrespective of the early type of feeding. The severity of disease may be an independent factor negatively influencing growth.

Growth patterns of breast fed and formula fed infants in the first 12 months of life: an Italian study.

AIM: To compare the growth patterns of breast fed and formula fed Italian infants in the first 12 months of life using World Health Organisation (WHO) reference data. METHODS: The growth patterns of 73 breast fed infants (36 male, 37 female) and 65 formula fed infants (35 male, 30 female) were compared. Solid foods were introduced with the same weaning schedules from the 5th month in both groups. The weight for age (WA), length for age (LA), and weight for length (WL) z scores (National Center for Health Statistics-WHO data) were calculated at birth, 1, 2, 3, 4, 6, 9, and 12 months. RESULTS: Breast fed infants had the highest z scores (WA, WL) at birth. Breast fed groups had significantly higher growth indices at 1 month (WA, LA), 2 months (WA) and 3 months (WA, LA) of age. Compared to breast fed groups, formula fed infants showed significantly higher WA z score changes in the 1-2, 2-3, 3-4, and 4-6 month intervals. LA z score changes were higher for breast fed infants at 0-1 month and for the formula fed infants at 4-6 months. In the 6-12 month interval growth indices progressively increased for the formula fed infants and declined for infants breast fed for longer (12 months). The 0-12 month changes in WA, LA, and WL z scores were positive for formula fed infants and negative for the 12 month breast fed group. Nevertheless, the 12 month breast fed group showed an absolute WA z score just below 0 (mean (SEM) -0.04 (0.26)) at 12 months. CONCLUSION: The growth pattern of breast fed and formula fed Italian infants differs in the first 12 months of life. This questions the validity of current reference values for monitoring the growth of breast fed infants. Growth indices in breast fed groups, high at birth and closer than expected to the reference at 12 months, may reflect differences in genetic factors, intrauterine conditions, or both.

Should genetic analysis in newborn screening and a heterozygote test for hyperphenylalaninaemia be recommended? An Italian study.

OBJECTIVE: To determine whether the introduction of genetic analysis for phenylalanine hydroxylase (PAH) deficiency into regional screening programmes can be supported by the benefit-cost ratio. METHOD: Tests for the genetic PAH locus were carried out in 151 patients with hyperphenylalaninaemia originally from all of the Italian regions. PAH mutations were identified by extraction of genomic DNA from leucocytes (whole blood in EDTA), PAH exon amplification was determined by polymerase chain reaction, restriction enzyme analysis was carried out for some recognised mutations, and DNA sequence analysis for the other mutations. RESULTS: It was found that the eight most common mutations in the population accounted for 49% of the mutant alleles, which is well below the required standard for effective population screening (90%). CONCLUSIONS: Genetic screening for PAH deficiency in Italy does not increase the sensitivity of the methodology and the benefit-cost ratio, and thus provides no advantage, particularly as the correlation between genotype and the metabolic phenotype needed to optimise dietary intervention is still being studied.

Growth in the first two years of uninfected children born to HIV-1 seropositive mothers.

OBJECTIVE: To assess the growth curves of uninfected infants born to type 1 human immunodeficiency virus (HIV-1) seropositive mothers by means of standardised anthropometric indices. METHODS: The z scores (National Center for Health Statistics-World Health Organization data) of weight for age, length for age, and weight for length of 92 uninfected full term infants born to HIV positive mothers were compared with those of 65 bottle fed full term infants born to healthy mothers at 0, 1, 2, 3, 4, 6, 9, 12, 18, and (in a subgroup) 24 months of age. Confounders were also recorded. RESULTS: The study population had a lower length for age z score at birth (95% confidence intervals (CI): 0.02, -0.58) and higher weight for length z scores at 1 (95% CI: 0.21, 0.63), 2 (95% CI: 0.25, 0.66), and 3 (95% CI: 0.0, 0.48) months compared with the reference group. After a temporary recovery, the length for age z score difference increased progressively from the 4th month onwards and was significant at 18 (95% CI: -0.31, -1.05) and 24 (95% CI: -0.02, -0.91) months. The difference between the length for age z scores at birth was associated with maternal covariates, but the between group difference at 18 months was apparent even after adjustment for covariates. CONCLUSION: Uninfected infants born to HIV positive mothers have a rapid weight gain immediately after birth. A decrease in length progression during the second year might be a result of the social risk connected with the family environment and an unfavourable programming related to the maternal HIV status.

Anthropometric indicators of human immunodeficiency virus infection in infants with early and late symptoms in the first months of life.

UNLABELLED: Standardised growth indices (Z-scores of weight-for-age, WA, length-for-age, LA, weight-for-length, WL, according to the reference data of the World Health Organization) have been compared for the first 4 months of life among 119 infants born to mothers affected by the type-1 human immunodeficiency virus (HIV). Infants were subdivided according to their HIV serostatus and the clinical expression of the disease. Uninfected status (n = 92), late (>6 months, n = 18) and early (<3 months, n = 9) onset of symptoms among the HIV infected defined three groups. Infants with early symptoms showed the lowest median WA and LA Z-scores at all times and the LA difference with their uninfected counterparts was already significant at birth. Infants with late symptoms showed early differences in WL and then in WA also compared with the uninfected ones. A < -0.40 LA Z-score at birth gave a 5.9 relative risk (RR) to be an infant with early symptoms (95% CI = 1.2-27.4) while a negative WL Z-score at 2 months of age gave a 4.2 RR for the HIV seropositivity (95% CI = 2.1-8.3). CONCLUSION: Linear growth is the first parameter to be negatively affected among human immunodeficiency virus seropositive infants with early symptoms. In infants with late symptoms the lack of rapid WA and WL increase found among uninfected patients may be viewed as an early anthropometric indicator of HIV status.