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PubMed2002

Successful treatment of a young infant who developed high-titer inhibitors against VWF-cleaving protease (ADAMTS-13): important discrimination from Upshaw-Schulman syndrome.

We report herein the case of a 9-month-old female infant with acquired thrombotic thrombocytopenic purpura (TTP), which was initially suspected to be either Upshaw-Schulman syndrome (USS or a congenital TTP) or hemolytic uremic syndrome (HUS) because of onset of clinical signs in infancy and accompanying diarrhea. She received combination therapy of plasma exchange, steroid pulse, and high-dose intravenous immunoglobulin infusion that was initiated before the definitive diagnosis, which resulted in excellent clinical improvement. The retrograde analysis of plasma ADAMTS-13 activity and its inhibitor showed a lack of this enzyme activity and the presence of a high-titer IgG inhibitor (200-320 Bethesda units/mL) to this enzyme activity. From our experience, it was suggested that we should recognize the possibility of the patient with acquired TTP in infancy and the importance of plasma exchange therapy for management of its clinical symptoms.

ADAM Proteins↗
PubMed2003

Effect of dexamethasone treatment on serum GH, IGF-I, and the binding proteins IGFBP-1 and -3 in ventilated very preterm infants.

Very preterm infants developing bronchopulmonary dysplasia frequently show a compromised growth in the neonatal period especially when steroids are given to facilitate weaning from the ventilator. The aim of this study was to evaluate the short-term effect of dexamethasone (DEXA) on the GH-IGF axis in ventilated very preterm infants developing bronchopulmonary dysplasia. We studied 10 very preterm artificially ventilated infants with bronchopulmonary dysplasia [median (range) gestational age 27.5 wk (25.9-32.0 wk), median (range) birth weight 970 g (610-2150 g)] immediately before and 2 d after the start of DEXA treatment. On both days of study, serum GH profiles were obtained, and serum IGF-I and IGF binding protein (IGFBP) -1 and -3 levels were measured. The ventilation score and the nutritional intake were calculated. Before the start of DEXA treatment, the median serum mean GH level was 12.0 microg/L (6-28.4 microg/L), whereas 2 d after the start of DEXA treatment the median serum mean GH level declined significantly to a value of 4.4 microg/L (1.7-11.9 microg/L). During DEXA treatment, mean, baseline, and maximal GH levels (Pulsar analysis) were significantly lower compared with pretreatment levels (p < 0.01, p < 0.01, and p < 0.05, respectively). Serum IGF-I and IGFBP-3 levels did not decline during DEXA. Serum IGFBP-1 levels were significantly lower compared with pretreatment levels (p < 0.01). Serum GH levels during DEXA treatment were correlated with neither the time interval between the administration of DEXA and the second GH profile nor the cumulative DEXA dose administered. Ventilation score and nutritional intake did not significantly correlate with serum GH, IGF-I, or IGFBP-1 or -3 levels, either before or after the start of DEXA. Two days of DEXA treatment in very preterm ventilated infants has a suppressive effect on serum GH levels, without an acute decline in serum IGF-I levels. A concomitant decrease in serum IGFBP-1 levels was found.

Bronchopulmonary Dysplasia↗
PubMed1991

Visual development of infants with severe ocular disorders.

Among 11 patients who presented as blind in early infancy, with Leber's congenital amaurosis (5 patients), optic nerve hypoplasia (4 patients), or macular colobomata (2 patients), 8 developed visually guided behavior and measurable grating acuity by age 5 to 46 months. All children with measurable grating acuity demonstrated visually guided mobility. Grating acuity was predictive of later visual performance in 10 of 11 patients by age 12 to 16 months. The best grating acuity attained by 7 months was 1.3 to 3.0 cycles/degrees (20/460 to 20/200) and 0.13 cycles/degrees (20/4700) by month 8. Two patients with Leber's congenital amaurosis and one with optic nerve hypoplasia remained blind. No clinical features existed to differentiate these three patients from the eight whose visual status improved. Posterior visual pathway maturation may underlie the improvement.

Child↗
PubMed1995

Inhaled nitric oxide in infants with developing or established chronic lung disease.

The effect on gas exchange of increasing concentrations of nitric oxide (0-60 parts per million) added to the inspired gases of nine ventilator-dependent infants (median postnatal age = 4 weeks; range 2-16 weeks) with chronic lung disease and pathological oxygenation index values was studied by means of arterial or transcutaneous PO2/PCO2. A significant improvement of oxygenation, indicated by a reduction of oxygenation index, was found (p < 0.014). The optimal nitric oxide concentration and the individual response varied between patients. PO2 returned to baseline values after the discontinuation of nitric oxide in all patients except one. No effect on PCO2 could be identified. Methaemoglobin values only increased marginally during the nitrous oxide exposition (pre-nitric oxide: 0.56% +/- 0.27; post-nitric oxide: 0.78 +/- 0.08; p = ns). Systemic blood pressure and heart rate were unaffected in all patients. Before inhaled nitric oxide can be considered for prolonged use in this patient category further studies regarding long-term efficacy and safety are needed.

Administration, Inhalation↗
PubMed1998

Low-level prenatal lead exposure and neurobehavioral development of children in the first year of life: a prospective study in Shanghai.

We used a prospective study design to assess the effects of prenatal low-level lead exposure on the development of urban, inner-city children in Shanghai. Umbilical cord blood samples wee consecutively collected from 605 live newborns. Two hundred and fifty-seven samples were excluded from the study due to clotting. Lead levels were determined on 348 cord blood samples. The geometric mean was 9.2 micrograms/dl. Based on their cord blood lead levels, infants were classified into two exposure groups: 104 in a relatively low lead group (lead levels < or = 30 percentile), and 104 in a relatively high lead group (lead levels > or = 70 percentile). Seventy-five subjects failed to complete the study, and 133 babies were included in the final cohort: 69 babies in the high lead group and 64 in the low lead group. At 3, 6, and 12 months, the Bayley Scales of Infant Development were administered and capillary blood lead levels were measured. Detailed information was obtained on a wide range of variables relevant to infant development. At all three ages, the Mental Development index (MDI) scores, adjusted for confounders, were inversely related to the infants' cord blood lead levels. The difference of the mean adjusted MDI scores between low and high lead groups was 3.4 at 3 months, 6.3 at 6 months, and 5.2 at 12 months of age. These differences were statistically significant at all time points. No significant association between cord blood lead levels and the Psychomotor Development Index (PDI) scores was detected at all three visits after adjustment for confounders. Postnatal lead levels were unrelated to concurrent developmental status. We conclude that prenatal low-level lead exposure, which is relatively common in Shanghai, is associated with an adverse developmental impact on children through the first year of life.

Child Development↗
PubMed2006

Theoretical interfaces in the acute paediatric context: a psychotherapeutic understanding of the application of infant-directed singing.

Psychotherapy literature provides a theoretical understanding of parent-infant attachment. This article will reflect upon the specific need to give thoughtful consideration to those infants admitted to the acute-care setting, such as neonatal and paediatric intensive care units, and the potential for this environment to affect infant development and the parent-infant relationship. Infant-directed singing, as described in this article, is an improvised form of vocal interaction that is specifically informed by an understanding of the musical parameters of pitch, rhythm, phrasing, timbre, register, dynamic, tempo and silence. This article will detail a theoretical understanding of using infant-directed singing to foster parent-infant interaction within the acute care environment. In particular, the potentially sensitive, reciprocal and engaging nature of infant-directed singing, coupled with its ability to promote and support maternal demonstrations of empathy, will be discussed with a view to the psychological and physical development of the hospitalised infant.

Child Development↗
PubMed2003

Apolipoprotein E genotype and neurodevelopmental sequelae of infant cardiac surgery.

BACKGROUND: There has been increasing recognition of adverse neurodevelopmental sequelae in some children after repair of congenital heart defects. Even among children with the same cardiac defect, significant interindividual variation exists in developmental outcome. Polymorphisms of apolipoprotein E have been identified as a risk factor for worse neurologic recovery after central nervous system injury. METHODS: A single-institution prospective study of patients <or=6 months of age undergoing cardiopulmonary bypass for repair of congenital heart defects was undertaken to evaluate the association between apolipoprotein E genotype and postoperative neurodevelopmental dysfunction. Developmental outcomes were evaluated at 1 year of age by using the Bayley Scales of Infant Development. RESULTS: One-year evaluation was performed in 244 patients. After adjustment for preoperative and postoperative covariates-including gestational age, age at operation, sex, race, socioeconomic status, cardiac defect, and use of deep hypothermic circulatory arrest-the apolipoprotein E epsilon2 allele was associated with a worse neurologic outcome as assessed by the Psychomotor Developmental Index of the Bayley Scales of Infant Development (P =.036). Patients with the apolipoprotein E epsilon2 allele had approximately a 7-point decrease in the Psychomotor Developmental Index. CONCLUSIONS: Apolipoprotein E epsilon2 allele carriers had significantly lower Psychomotor Development Index scores at 1 year of age after infant cardiac surgery. The effect was independent of ethnicity, socioeconomic status, cardiac defect, and use of deep hypothermic circulatory arrest. An effect of the apolipoprotein E epsilon4 allele was not detected. Genetic polymorphisms that decrease neuroresiliency and impair neuronal repair after central nervous system injury are important risk factors for neurodevelopmental dysfunction after infant cardiac surgery.

Alleles↗
PubMed1977

Vestibular stimulation influence on motor development in infants.

Preambulatory, normal human infants were exposed to sessions of mild semicircular canal stimulation on 2 days per week for 4 weeks. The gross motor ability of each child was assessed before and after the 4-week period. The vestibular stimulation effected a significant improvement in gross motor skills.

Child Development↗
PubMed2005

Epidemiology of necrotizing enterocolitis--Part II: Risks and susceptibility of premature infants during the surfactant era: a regional study.

OBJECTIVES: We observed a reduced incidence of necrotizing enterocolitis (NEC) despite increased survival of extremely premature infants over the last two decades. A different susceptibility pattern to NEC according to gestation has been proposed. We aim to examine the influence of perinatal risk factors for NEC in infants less than 32 weeks gestation, in our region, in the post-surfactant era. METHODS: NEC incidence, perinatal risk factors and neonatal outcomes were examined in a cohort of 4649 infants of 24-31 weeks gestation from the Neonatal Intensive Care Unit Study (NICUS) database, admitted during 1994-99. RESULTS: A total of 178 (3.8%) infants developed NEC. More 24-27 weeks infants than 28-31 weeks infants had NEC (6.6% vs 2.6%, P < 0.001). Although low gestation infants had higher mortality and surgery need, post-operative survival (60%) was comparable to the higher gestation infants. NEC in the higher gestation group tended to be associated with perinatal risk factors, while risk factors were equally common in low gestation infants who developed NEC or not. Overall, gestation (P < 0.001), small for gestational age (P= 0.008), hyaline membrane disease (P < 0.001), placental abruption (P = 0.002) and earlier year of birth (P = 0.03) were independently associated with increased odds of NEC. Maternal hypertensive disease of pregnancy was associated with reduced NEC risk (P = 0.02). CONCLUSIONS: The incidence of NEC continues to decrease in the surfactant era and the pattern of susceptibility to NEC is based on gestational age grouping and risk factors. The reduced risk associated with hypertensive disease of pregnancy is unexplained.

Enterocolitis, Necrotizing↗
PubMed2005

Species comparative studies and cognitive development.

The comparative study of infant development and animal cognition brings to cognitive science the promise of insights into the nature and origins of cognitive skills. In this article, I review a recent wave of comparative studies conducted with similar methodologies and similar theoretical frameworks on how two core components of human cognition--object permanence and gaze following--develop in different species. These comparative findings call for an integration of current competing accounts of developmental change. They further suggest that evolution has produced developmental devices capable at the same time of preserving core adaptive components, and opening themselves up to further adaptive change, not only in interaction with the external environment, but also in interaction with other co-developing cognitive systems.

Animals↗
PubMed1984

Myelin development in infant brain.

Myelin was isolated from subcortical areas of ten human brains, with ages ranging from 24 days to 350 days-of-age; samples were subsequently analyzed for lipid composition. Eight infants were victims of Sudden Infant Death Syndrome, and two infants were accident cases. Gray and white matter samples from each brain were also dissected and analyzed. Galactolipids were only 12% of the total lipids in white matter from brains of infants that were 24 days-of-age, a time when myelination was just starting in the subcortical areas. At 175 and 350 days of age, myelination was well underway and galactolipids measured 22% of the total lipids. Total phospholipids decreased (65% to 54%) in white matter during development, with the decrease mostly in phosphatidylcholine (23% to 15%). Even though there was little white matter present at early ages, myelin could be isolated. Surprisingly, the lipid composition of myelin from the 24-day-infant brain was similar to that from adult brain. Galactolipids were 22% of the total lipids, cholesterol, 23%, and phospholipids, 52%. These results suggest that only subtle remodeling of myelin occurs in humans once myelination commences. All four major gangliosides were present in myelin during this first year of development. Interestingly, the yield of myelin from the 350-day-old infant subcortical white matter was similar to that from an adult. Thus major tracts in this area may have acquired most of the myelin by one year-after-birth. Since the control samples blend quite well into the developmental pattern obtained, it is believed there are no abnormalities in myelin lipids from SIDS infants.

Brain↗
PubMed1998

Evidence of altered central nervous system development in infants with iron deficiency anemia at 6 mo: delayed maturation of auditory brainstem responses.

Iron deficiency anemia has long been thought to have effects on the central nervous system (CNS). Finding direct evidence of this in human infants, however, has been challenging. Auditory brainstem responses (ABRs) provide a noninvasive means of examining an aspect of the CNS that is rapidly maturing during the age period when iron deficiency is most common. ABRs represent the progressive activation of the auditory pathway from the acoustic nerve (wave I) to the lateral lemniscus (wave V). The central conduction time (CCT, or wave I-V interpeak latency) is considered an index of CNS development because myelination of nerve fibers and maturation of synaptic relays lead to an exponential reduction in the CCT from birth to 24 mo. In 55 otherwise healthy, 6-mo-old Chilean infants (29 with iron deficiency anemia and 26 nonanemic control infants), the CCT was longer in those who had been anemic at 6 mo, with differences becoming more pronounced at 12- and 18-mo follow-ups despite effective iron therapy. The pattern of results--differences in latencies but not amplitudes, more effects on the late ABR components (waves III and V), and longer CCTs (as an overall measure of nerve conduction velocity)--suggested altered myelination as a promising explanation, consistent with recent laboratory work documenting iron's essential role in myelin formation and maintenance. This study shows that iron deficiency anemia in 6-mo-old infants is associated with adverse effects on at least one aspect of CNS development and suggests the fruitfulness of studying other processes that are rapidly myelinating during the first 2 y of life.

Anemia, Iron-Deficiency↗