Acquisition of motor abilities up to independent walking in very low birth weight preterm infants

Objective: To determine chronological and corrected ages at acquisition of motor abilities up to independent walking in very low birth weight preterms and to determine up to what point it is necessary to use corrected age.Methods: This was a longitudinal study of preterms with birth weight < 1,500 g and gestational age <= 34 weeks, free from neurosensory sequelae, selected at the high-risk infants follow-up clinic at the Hospital das Clinicas, Faculdade de Medicine de Botucatu, Universidade Estadual Paulista (UNESP) in Botucatu, Brazil, between 1998 to 2003, and assessed every 2 months until acquisition of independent walking.Results: Nine percent of the 155 preterms recruited were excluded from the study, leaving a total of 143 patients. The mean gestational age was 30 +/- 2 weeks, birth weight was 1,130 +/- 222 g, 59% were female and 44% were small for gestational age. Preterms achieved head control in their second month, could sit independent at 7 months and walked at 12.8 months' corrected age, corresponding to the 4th, 9th and 15th months of chronological age. There were significant differences between chronological age and corrected age for all motor abilities. Preterms who were small for their gestational age acquired motor abilities later, but still within expected limits.Conclusions: Very low birth weight preterms, free from neurosensory disorders, acquired their motor abilities within the ranges expected for their corrected ages. Corrected age should be used until independent walking is achieved.

Crescimento de prematuros de baixo peso ao nascer nos dois primeiros anos de vida: influência da Síndrome hipertensiva gestacional

Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)

Long-term oral sensitivity and feeding skills of low-risk pre-term infants

This study examined the oral sensitivity and feeding skills of low-risk pre-term infants at 11-17 months corrected age. Twenty pre-term infants (PT) born between 32 and 37 weeks at birth without any medical comorbidities were assessed. All of this PT group received supplemental nasogastric (NG) tube feeds during their birth-stay in hospital. A matched control group of 10 healthy full-term infants (FT) was also assessed. Oral sensitivity and feeding skills were assessed during a typical mealtime using the Royal Children's Hospital Oral Sensitivity Checklist (OSC) and the Pre-Speech Assessment Scale (PSAS). Results demonstrated that, at 11-17 months corrected age, the PT group displayed significantly more behaviours suggestive of altered oral sensitivity and facial defensiveness, and a trend of more delayed feeding development than the FT group. Further, results demonstrated that, relative to the FT group, pre-term infants who received greater than 3 weeks of NG feeding (PT>3NG) displayed significantly more facial defensive behaviour, and displayed significant delays across more aspects of their feeding development than pre-term infants who received less than 2 weeks of NG feeding (PT

Impact de trois interventions: méthode mère kangourou, massage en incubateur et massage en position kangourou sur la croissance et le développement des enfants prématurés nés à moins de 33 semaines d’âge gestationnel

Cette recherche vise à étudier l’impact d’interventions réalisées par les parents dans l’unité néonatale de soins intensifs. Plus spécifiquement, le premier objectif est de documenter les effets différentiels de la Méthode Mère Kangourou « MMK » accompagnée ou non du Massage en incubateur «MI » ou du Massage en Position Kangourou « MPK » et des Soins Traditionnels «ST » accompagnés ou non du massage dans l’incubateur sur la croissance physique mesurée par le poids, la taille et le périmètre crânien pendant une période de 5 et 15 jours dans l’unité néonatale et l’impact à 40 semaines d’âge gestationnel. Le second objectif est de comparer, chez des enfants qui bénéficient de la « MMK » la valeur ajoutée du « MPK » ou du «MI » sur le neuro-développement à 6 et 12 mois d’âge corrigé de l’enfant. Un échantillon total de 198 enfants et leur famille a été recruté de la façon suivante dans trois hôpitaux de Bogota. Dans chaque hôpital, 66 sujets ont été répartis aléatoirement à deux conditions. Ces hôpitaux ont été choisis afin de tester les effets de diverses conditions expérimentales et de diminuer les bais de sélection. Dans chaque hôpital, deux techniques ont été assignées aléatoirement. Il s’agit, dans le premier, de la « MMK & MPK » vs « MMK & MI ». Dans le second, « MMK sans massage » vs « MMK & MI ». Dans le troisième, « MI » a été comparé aux « ST » ce qui implique une absence de contact physique continu des bébés avec leurs parents. Les résultats rapportés dans le premier article sont à l’effet que, dans le premier hôpital, il y a un effet compensatoire de l’intervention « MMK & MPK » sur la perte physiologique du poids de l’enfant prématuré dans les 15 premiers jours de vie avec un impact sur le poids à 40 semaines d’âge gestationnel, sur la durée du portage kangourou et sur la durée d’hospitalisation totale. Aucun effet sur le périmètre crânien ou la taille n’est apparu. Dans le deuxième hôpital, aucune différence significative n’est rapportée pour le poids sauf quand l’intervention est commencée après le 10ième jours de vie alors que l’enfant « MPK» semble grossir mieux que le «MMK avec MI». Finalement, dans le troisième hôpital il n’y a aucun effet du massage sur les variables anthropométriques, le groupe avec MI grossissant moins vite avec un léger impact sur le poids à 40 semaines. Cela pourrait être dû à la perte de chaleur due à l’ouverture de l’incubateur quand l’enfant est très immature. Dans le second article, les 66 enfants de l’hôpital sont répartis aléatoirement dans le groupe « MMK & MPK» vs le groupe « MMK & MI», ont complété, à 6 et 12 mois d’âge corrigé, un test de neuro-développement, le Griffiths. Les résultats à 6 mois ne montrent aucune différence entre les 2 interventions, mais a 12 mois le IQ semble dépendant du nombre de jours d’hospitalisation de l’enfant, cette durée d’hospitalisation correspond au temps que met l’enfant à se stabiliser physiquement et correspond également au temps que mettent la mère et l’enfant à s’adapter à la méthode kangourou. Une fois, l’adaptation kangourou réussie, la dyade mère enfant sort avec l’enfant toujours en position kangourou. Le temps d’hospitalisation correspond au temps que met l’enfant à être éligible à l’apprentissage de la MMK par la mère. À 12 mois les deux groupes montrent des résultats équivalents, mais des différences positives sont apparues pour le groupe « MMK & MPK» dans les sous échelle Coordination Oculo Manuelle et Audition et Langage du test Griffiths. Dans l’ensemble, les résultats suggèrent que la pratique des deux interventions non traditionnelles peut contribuer à une meilleure croissance physique dans nos cohortes. Le gain de poids du bébé, notamment, est affecté par l’intervention MPK (Hôpital 1) ou sans l’ajout du Massage (Hôpital 2). Par ailleurs, le massage en incubateur n’a pas de différence significative en comparaison aux soins traditionnels, ces interventions ont toutefois un impact mineur (tendances) sur le neuro développement à 6 et 12 mois d’âge corrigé dans cette étude.

Esotropie précoce: résultats à long terme d'une série de 82 cas opérés par myopexie rétro-équatoriale et recul simultané des deux muscles droits médiaux avant l'âge de 24 mois [Early-onset esotropia operated before the age of 24 months: long-term results of Cüpper's retropexy combined with simultaneous recession of both medial recti]

BACKGROUND: When and how to operate an early-onset esotropia (onset before 6 months of age) is still controversial. We conducted a retrospective study of such patients operated before the age of 24 months. PATIENTS AND METHODS: 82 patients were operated by one surgeon (GK) and followed by the same team. At 5 years post-operation, evaluation criteria included the residual angle of deviation, visual acuity (Birkhäuser Nr 505, 5 m) and binocularity (Lang stereotest I, Bagolini glasses). RESULTS: At 5 years, the residual angle was excellent (0 degrees to + 5 degrees ) in 67 % good, (>+5 degrees to +10 degrees or 0 to -5 degrees ) in 23 %, and poor (>+10 degrees or <-5 degrees ) in 10 %. During the 5 years of follow-up the rate of reoperation was 9.7 %. Isoacuity was obtained in 62 %, slight amblyopia (2 lines of interocular difference) was present in 32 %, and average amblyopia (> 3 lines of interocular difference) was noted in 6 %. Simultaneous perception was present in 53 %, whereas one eye was suppressed or results were undetermined in 47 %. No patient demonstrated stereoscopy using the Lang's stereotest I. CONCLUSION: The results from our study demonstrate that early surgery of early-onset esotropia has a favourable outcome on both visual acuity and the residual angle of strabismus. Simultaneous perception was achieved in 53 %. These figures are comparable to the results of the ELISSS multicentric study.

Influence of segmental chromosome abnormalities on survival in children over the age of 12 months with unresectable localised peripheral neuroblastic tumours without MYCN amplification.

BACKGROUND: The prognostic impact of segmental chromosome alterations (SCAs) in children older than 1 year, diagnosed with localised unresectable neuroblastoma (NB) without MYCN amplification enrolled in the European Unresectable Neuroblastoma (EUNB) protocol is still to be clarified, while, for other group of patients, the presence of SCAs is associated with poor prognosis. METHODS: To understand the role of SCAs we performed multilocus/pangenomic analysis of 98 tumour samples from patients enrolled in the EUNB protocol. RESULTS: Age at diagnosis was categorised into two groups using 18 months as the age cutoff. Significant difference in the presence of SCAs was seen in tumours of patients between 12 and 18 months and over 18 months of age at diagnosis, respectively (P=0.04). A significant correlation (P=0.03) was observed between number of SCAs per tumour and age. Event-free (EFS) and overall survival (OS) were calculated in both age groups, according to both the presence and number of SCAs. In older patients, a poorer survival was associated with the presence of SCAs (EFS=46% vs 75%, P=0.023; OS=66.8% vs 100%, P=0.003). Moreover, OS of older patients inversely correlated with number of SCAs (P=0.002). Finally, SCAs provided additional prognostic information beyond histoprognosis, as their presence was associated with poorer OS in patients over 18 months with unfavourable International Neuroblastoma Pathology Classification (INPC) histopathology (P=0.018). CONCLUSIONS: The presence of SCAs is a negative prognostic marker that impairs outcome of patients over the age of 18 months with localised unresectable NB without MYCN amplification, especially when more than one SCA is present. Moreover, in older patients with unfavourable INPC tumour histoprognosis, the presence of SCAs significantly affects OS.

Early dyadic patterns of mother-infant interactions and outcomes of prematurity at 18 months.

OBJECTIVE: With the increased survival of very preterm infants, there is a growing concern for their developmental and socioemotional outcomes. The quality of the early mother-infant relationship has been noted as 1 of the factors that may exacerbate or soften the potentially adverse impact of preterm birth, particularly concerning the infant's later competencies and development. The first purpose of the study was to identify at 6 months of corrected age whether there were specific dyadic mother-infant patterns of interaction in preterm as compared with term mother-infant dyads. The second purpose was to examine the potential impact of these dyadic patterns on the infant's behavioral and developmental outcomes at 18 months of corrected age. METHODS: During a 12-month period (January-December 1998), all preterm infants who were <34 weeks of gestational age and hospitalized at the NICU of the Lausanne University Hospital were considered for inclusion in this longitudinal prospective follow-up study. Control healthy term infants were recruited during the same period from the maternity ward of our hospital. Mother-infant dyads with preterm infants (n = 47) and term infants (n = 25) were assessed at 6 months of corrected age during a mother-infant play interaction and coded according to the Care Index. This instrument evaluates the mother's interactional behavior according to 3 scales (sensitivity, control, and unresponsiveness) and the child's interactional behavior according to 4 scales (cooperation, compliance, difficult, and passivity). At 18 months, behavioral outcomes of the children were assessed on the basis of a semistructured interview of the mother, the Symptom Check List. The Symptom Check List explores 4 groups of behavioral symptoms: sleeping problems, eating problems, psychosomatic symptoms, and behavioral and emotional disorders. At the same age, developmental outcomes were evaluated using the Griffiths Developmental Scales. Five areas were evaluated: locomotor, personal-social, hearing and speech, eye-hand coordination, and performance. RESULTS: Among the possible dyadic patterns of interaction, 2 patterns emerge recurrently in mother-infant preterm dyads: a "cooperative pattern" with a sensitive mother and a cooperative-responsive infant (28%) and a "controlling pattern" with a controlling mother and a compulsive-compliant infant (28%). The remaining 44% form a heterogeneous group that gathers all of the other preterm dyads and is composed of 1 sensitive mother-passive infant; 10 controlling mothers with a cooperative, difficult, or passive infant; and 10 unresponsive mothers with a cooperative, difficult, or passive infant. Among the term control subjects, 68% of the dyads are categorized as cooperative pattern dyads, 12% as controlling pattern dyads, and the 20% remaining as heterogeneous dyads. At 18 months, preterm infants of cooperative pattern dyads have similar outcomes as the term control infants. Preterm infants of controlling pattern dyads have significantly fewer positive outcomes as compared with preterm infants of cooperative pattern dyads, as well as compared with term control infants. They display significantly more behavioral symptoms than term infants, including more eating problems than term infants as well as infants from cooperative preterm dyads. Infants of the controlling preterm dyads do not differ significantly for the total development quotient but have worse personal-social development than term infants and worse hearing-speech development than infants from cooperative preterm dyads. The preterm infants of the heterogeneous group have outcomes that can be considered as intermediate with no significant differences compared with preterm infants from the cooperative pattern or the controlling pattern dyads. CONCLUSION: Among mother-preterm infant dyads, we identified 2 specific patterns of interaction that could play either a protective (cooperative pattern) or a risk-precipitating (controlling pattern) role on developmental and behavioral outcome, independent of perinatal risk factors and of the family's socioeconomic background. The controlling pattern is much more prevalent among preterm than term dyads and is related to a less favorable infant outcome. However, the cooperative pattern still represents almost 30% of the preterm dyads, with infants' outcome comparable to the ones of term infants. These results point out the impact of the quality of mother-infant relationship on the infant's outcome. The most important clinical implication should be to support a healthy parent-infant relationship already in the NICU but also in the first months of the infant's life. Early individualized family-based interventions during neonatal hospitalization and transition to home have been shown to reduce maternal stress and depression and increase maternal self-esteem and to improve positive early parent-preterm infant interactions.

Intensive care for extreme prematurity--moving beyond gestational age.

BACKGROUND: Decisions regarding whether to administer intensive care to extremely premature infants are often based on gestational age alone. However, other factors also affect the prognosis for these patients. METHODS: We prospectively studied a cohort of 4446 infants born at 22 to 25 weeks' gestation (determined on the basis of the best obstetrical estimate) in the Neonatal Research Network of the National Institute of Child Health and Human Development to relate risk factors assessable at or before birth to the likelihood of survival, survival without profound neurodevelopmental impairment, and survival without neurodevelopmental impairment at a corrected age of 18 to 22 months. RESULTS: Among study infants, 3702 (83%) received intensive care in the form of mechanical ventilation. Among the 4192 study infants (94%) for whom outcomes were determined at 18 to 22 months, 49% died, 61% died or had profound impairment, and 73% died or had impairment. In multivariable analyses of infants who received intensive care, exposure to antenatal corticosteroids, female sex, singleton birth, and higher birth weight (per each 100-g increment) were each associated with reductions in the risk of death and the risk of death or profound or any neurodevelopmental impairment; these reductions were similar to those associated with a 1-week increase in gestational age. At the same estimated likelihood of a favorable outcome, girls were less likely than boys to receive intensive care. The outcomes for infants who underwent ventilation were better predicted with the use of the above factors than with use of gestational age alone. CONCLUSIONS: The likelihood of a favorable outcome with intensive care can be better estimated by consideration of four factors in addition to gestational age: sex, exposure or nonexposure to antenatal corticosteroids, whether single or multiple birth, and birth weight. (ClinicalTrials.gov numbers, NCT00063063 [ClinicalTrials.gov] and NCT00009633 [ClinicalTrials.gov].).

A novel WT1 heterozygous nonsense mutation (p.K248X) causing a mild and slightly progressive nephropathy in a 46,XY patient with Denys-Drash syndrome

WT1 mutations have been described in a variety of syndromes, including Denys-Drash syndrome (DDS), which is characterized by predisposition to Wilms` tumor, genital abnormalities and development of early nephropathy. The most frequent WT1 defects in DDS are missense mutations located in exons 8-9. Our aim is to report a novel WT1 mutation in a 46,XY patient with a DDS variant, who presented a mild nephropathy with a late onset diagnosed during adolescence. He had ambiguous genitalia at birth. At 4 months of age he underwent nephrectomy (Wilms` tumor) followed by chemotherapy. Ambiguous genitalia were corrected and bilateral gonadectomy was performed. Sequencing of WT1 identified a novel heterozygous mutation (c.742A > T) in exon 4 that generates a premature stop codon (p.K248X). Interestingly, this patient has an unusual DDS nephropathy progression, which reinforces that patients carrying WT1 mutations should have the renal function carefully monitored due to the possibility of late-onset nephropathy.

Conductive hearing loss in children with bronchopulmonary dysplasia: A longitudinal follow-up study in children aged between 6 and 24 months

Aims: To determine the occurrence of isolated and recurrent episodes of conductive hearing loss (CHL) during the first two years of life in very low birth weight (VLBW) infants with and without bronchopulmonary dysplasia (BPD). Study design, subjects and outcome measures: In a longitudinal clinical study. 187 children were evaluated at 6, 9, 12,15 18 and 24 months of age by visual reinforcement audiometry, tympanometry and auditory brain response system. Results: Of the children with BPD, 54.5% presented with episodes of CHL, as opposed to 34.7% of the children without BPD. This difference was found to be statistically significant. The recurrent or persistent episodes were more frequent among children with BPD (25.7%) than among those without BPD (8.3%). The independent variables that contributed to this finding were small for gestational age and a 5 min Apgar score. Conclusions: Recurrent CHL episodes are more frequent among VLBW infants with BPD than among VLBW infants without BPD. (C) 2010 Elsevier Ireland Ltd. All rights reserved.

Maternal Anxiety and Depression and Development of Prematurely Born Infants in the First Year of Life

The Purpose of this study was: (a) to assess and to compare anxiety and depression symptoms in mothers of preterm neonates during hospitalization in the Neonatal Intenive Care Unit, after discharge, and at the end of the infants` first year of life and (b) to assess the child`s development at 12 months of chronological corrected age (CCA). Thirty-six mothers, with no psychiatric antecedents assessed with the SCID-NP were evaluated by STAI and BDI The infants were assessed with Bayley-II Scales. There was a significant decrease in clinical symptoms of state-anxiety in mothers (p =.008). comparing the period during hospitalization and after discharge of the infants. Clinical symptoms of anxiety and depression were observed in 20% of the mothers at the end of the infants` first year of age. The majority of the infants exhibited normal development on Bayley-II at 12 months CCA: however. 25% of the infants displayed cognitive problems and 40% motor problems. The mothers` anxiety and depression symptoms decreased it the end of the first year of life of the pre-term infants and the children showed predominately normal development Lit this phase.

Physical therapy assessment tools to evaluate disease progression and phenotype variability in Golden Retriever muscular dystrophy

Dogs suffering from Golden Retriever muscular dystrophy (GRMD) present symptoms that are similar to human patients with Duchenne muscular dystrophy (DMD). Phenotypic variability is common in both cases and correlates with disease progression and response to therapy. Physical therapy assessment tools were used to study disease progression and assess phenotypic variability in dogs with GRMD. At 5 (TO), 9 (T1), 13 (T2) and 17 (T3) months of age, the physical features, joint ranges of motion (ROM), limb and thorax circumferences, weight and creatine kinase (CK) levels were assessed in 11 dogs with GRMD. Alterations of physical features were higher at 13 months, and different disease progression rates were observed. Passive ROM decreased until 1 year old, which was followed by a decline of elbow and tarsal ROM. Limb and thorax circumferences, which were corrected for body weight, decreased significantly between TO and T3. These measurements can be used to evaluate disease progression in dogs with GRMD and to help discover new therapies for DMD patients. (C) 2011 Elsevier Ltd. All rights reserved.

Conductive hearing loss in preterm infants with bronchopulmonary dysplasia

Objective: To determine the risk of conductive hearing loss in preterm infants with bronchopulmonary dysplasia (BPD) and preterm controls. Methodology: The study population consisted of 78 infants with BPD of 26-33 weeks gestation and 78 controls of similar gestational age matched for broad-based birthweight categories. An auditory brainstem response (ABR) audiology was performed shortly before hospital discharge. Visual reinforcement orientation audiometry (VROA) and impedance audiometry were performed at 8-12 months corrected for prematurity. Infants with persistent audiological abnormalities were referred for evaluation to paediatric ENT surgeons. Results: Infants with BPD had a significantly higher rate of ABR abnormalities (BPD: 22%, controls: 9%; P = 0.028). On VROA and impedance audiometry, the infants with BPD also had a higher rate of persistent abnormalities. Following ENT assessment, 22.1% of infants with BPD and 7.7% of controls had persistent conductive dysfunction requiring myringotomy and grommet tube insertion (P = 0.03). Most of these infants had normal ABR audiometry at hospital discharge. Conclusions: Preterm infants with BPD are at high risk of persistent conductive hearing loss late in the first year of life compared to controls. An ABR audiology conducted at the time of hospital discharge does not predict accurately later conductive hearing problems. Infants with BPD should have routine audiological evaluation toward the end of the first year of life.

Lack of association between iron status at birth and growth of preterm infants

OBJECTIVE: To assess the association between iron status at birth and growth of preterm infants. METHODS: Ninety-five premature babies (26 to 36 weeks of gestational age) born from July 2000 to May 2001 in a public hospital in Rio de Janeiro, Southeastern Brazil, were followed up for six months, corrected by gestational age. Iron measurements at birth were available for 82 mothers and 78 children: hemoglobin, hematocrit, mean corpuscular volume and plasma iron. All children received free doses of iron supplement (2 mg/kg/day) during the follow-up period and up to two years of age. Multivariate linear regression analyses with repeated measurements were performed to assess factors associated to linear growth. RESULTS: Growth was more pronounced up to 40 weeks of gestational age, increasing about 1.0 cm/week and then slowing down to 0.75 cm/week. The multivariate analysis showed growth was positively associated with birth weight (0.4 cm/100 g; p<0.001) and negatively associated with gestational age at birth (-0.5 cm/week; p<0.001). There was no association between cord iron and mother iron measurements and growth (p>0.60 for all measures). Only two children had anemia at birth, whereas 43.9% of mothers were anemic (hemoglobin <11 g/dl). Also, there was no correlation between anemia indicators of mothers and children at birth (r<0.15; p>0.20). CONCLUSIONS: Maternal anemia was not associated with anemia in preterm infants and iron status of mothers and children at birth was not associated with short-term growth of preterm infants.

Growth patterns in early childhood and the onset of menarche before age twelve

OBJECTIVE: To examine the relationship between growth patterns in early childhood and the onset of menarche before age 12. METHODS: The study included 2,083 women from a birth cohort study conducted in the city of Pelotas, Southern Brazil, starting in 1982. Anthropometric, behavioral, and pregnancy-related variables were collected through home interviews. Statistical analyses were performed using Pearson's chi-square and chi-square test for linear trends. A multivariable analysis was carried out using Poisson regression based on a hierarchical model. RESULTS: Mean age of menarche was 12.4 years old and the prevalence of menarche before age 12 was 24.3%. Higher weight-for-age, height-for-age, and weight-for-height z-scores at 19.4 and 43.1 months of age were associated with linear tendencies of increased prevalence and relative risks of the onset of menarche before age 12. Girls who experienced rapid growth in weight-for-age z-score from birth to 19.4 months of age and in weight-for-age or height-for-age z-scores from 19.4 to 43.1 months of age also showed higher risk of menarche before age 12. Higher risk was seen when rapid growth in weight-for-age z-score was seen during these age intervals and the highest risk was found among those in the first tertile of Williams' curve at birth. Rapid growth in weight-for-height z-score was not associated with menarche before age 12. CONCLUSIONS: Menarche is affected by nutritional status and growth patterns during early childhood. Preventing overweight and obesity during early childhood and keeping a "normal" growth pattern seem crucial for the prevention of health conditions during adulthood.