The added value of the electrocardiogram in Noonan syndrome.

Noonan syndrome is a genetic disorder characteried by short stature, typical facial features, developmental delay, and CHD. In this single-centre retrospective study, we analysed typical Noonan syndrome-related electrocardiographic features in 95 patients with clinically and molecularly confirmed Noonan syndrome. Typical Noonan syndrome-related electrocardiographic features are left axis deviation, small left precordial R-waves, large right precordial S-waves, abnormal Q-wave, and abnormal wide QRS complex.

Lower incidence of necrotizing enterocolitis in infants fed a preterm formula with egg phospholipids.

Necrotizing enterocolitis (NEC) causes approximately 4000 deaths/y and significant morbidity among U.S.-born preterm infants alone.

Visual acuity and fatty acid status of term infants fed human milk and formulas with and without docosahexaenoate and arachidonate from egg yolk lecithin.

Preterm infants fed formulas with docosahexaenoic acid (DHA, 22:6n-3) during the interval equivalent to the last intrauterine trimester and beyond have higher circulating DHA and transiently higher visual acuity compared with infants fed formulas containing linolenic acid. In term infants several nonrandomized studies of infants receiving DHA from human milk suggest a relationship between DHA status and acuity, but the evidence for a cause-and-effect relationship is mixed. In the present study, term infants were randomly assigned to a standard term formula (n = 20) or the same formula with egg yolk lecithin to provide DHA (0.

Effect of long-chain n-3 fatty acid supplementation on visual acuity and growth of preterm infants with and without bronchopulmonary dysplasia.

Healthy preterm infants fed formula with long-chain n-3 fatty acids (n-3 LCFAs) from marine oil have better early visual acuity but lower plasma phosphatidylcholine (PC) arachidonic acid (AA) and growth than infants fed formula containing linolenic acid (LLA) as the sole n-3 fatty acid. This randomized, double-blind trial was designed to study the effects of a different source of n-3 LCFAs and a shorter feeding interval on visual acuity (by Teller Acuity Card) and growth of preterm infants (n = 59; 747-1275 g birth wt), some of whom required long periods of supplemental oxygen and developed bronchopulmonary dysplasia (BPD). Infants were studied at 0, 2, 4, 6, 9, and 12 mo past term.

A randomized trial of visual attention of preterm infants fed docosahexaenoic acid until nine months.

This randomized, double-blind trial tested the hypothesis that the addition of 0.2% docosahexaenoic acid (DHA, 22:6n-3) from marine oil to commercially-available preterm and term formulas with > or = 3% linolenic acid (18:3n-3) would enhance novelty preference and visual attention of preterm infants. Among preterm infants cared for in our center, study infants were a select group considered to be at lower risk for developmental delay.

A randomized trial of visual attention of preterm infants fed docosahexaenoic acid until two months.

This was a randomized, double-blind trial to determine if a nutrient-enriched (preterm) formula supplemented with 0.2% docosahexaenoic acid (DHA, 22:6n-3) from a low eicosapentaenoic acid (0.06%) source of marine oil would enhance visual novelty preference and attention of preterm infants.

Long-chain fatty acids and early visual and cognitive development of preterm infants.

Preterm infants fed formula supplemented with DHA were shown in two randomized, clinical trials to have improved visual acuity in the first half of infancy. In the second clinical trial, infants simultaneously supplemented with DHA and provided with a nutrient-enriched preterm formula had a higher Bayley MDI score at 12 months than controls fed preterm formula. These data are the first evidence that DHA alone can also improve performance on early tests of mental development.

Effect of vitamin A supplementation of intravenous lipids on early vitamin A intake and status of premature infants.

Preterm infants were randomly assigned to receive routine vitamin A supplementation (Regular A) or additional vitamin A in intravenous lipids (High A). Because infants with bronchopulmonary dysplasia (BPD) have poorer vitamin A status than infants who do not develop BPD, High A and Regular A infants were divided by BPD (no or yes) before determining the effects of treatment on intake and plasma concentration of retinol in the first month. Compared with infants without BPD, those with BPD received less retinol (RE.

Visual-acuity development in healthy preterm infants: effect of marine-oil supplementation.

Docosahexaenoic acid (DHA; 22:6n-3) is important for normal visual development. We hypothesized that preterm infants fed formulas with marine oil as a source of DHA would have better visual acuity than infants fed formulas without marine oil, as measured by the Teller Acuity Card procedure. Marine oil (P < 0.

Postnatal growth in infants born between 700 and 1,500 g.

Our purpose was to examine postnatal growth in very low birth weight (VLBW) infants (n = 54). A high frequency of intrauterine growth retardation was noted that was corrected by plotting birth weight against crown-heel length and not gestational age. No differences in postnatal growth were noted between infants whose size was appropriate for gestational age (n = 37) and those small for gestational age (n = 17).

Arachidonic acid status correlates with first year growth in preterm infants.

Diets deficient in the omega-6 fatty acid linoleic acid reduce arachidonic acid (Ach) concentrations and retard growth of developing animals and humans. Nevertheless, plasma phosphatidylcholine Ach concentrations declined from 84 +/- 23 mg/liter at birth to a nadir of 38 +/- 11 mg/liter at 4 mo of age in preterm infants fed commercial formulas with linoleic acid, and weight normalized to that of term infants fell progressively beginning at 2 mo of age. The nadir of plasma phosphatidylcholine Ach (31 +/- 7 mg/liter) and growth were further reduced by formula containing marine oil compared with the commercial formulas.

First year growth of preterm infants fed standard compared to marine oil n-3 supplemented formula.

Very low birth weight (VLBW) infants (748-1390 g, n = 65) were randomly assigned to receive control or marine oil-supplemented formula when they achieved intakes > 454 kJ (110 kcal)/kg/d of a formula designed for VLBW infants. Study formulas with or without marine oil were provided until 79 wk of postconceptional age (PCA), first in a formula designed for preterm infants followed by a formula designed for term infants. Infants were studied at regular intervals through 92 wk PCA.

Effects of type of dietary protein on acid-base status, protein nutritional status, plasma levels of amino acids, and nutrient balance in the very low birth weight infant.

Study Objective: To determine the effect of the type of dietary protein (3.3 gm/kg per day) on acid-base status, protein nutritional status, plasma amino acid concentrations, and nutrient (nitrogen, fat, mineral, trace element) balance.

Subjects: Preterm infants (birth weight less than or equal to 1250 gm, gestational age less than or equal to 32 weeks) with no evidence of systemic disease, who had achieved a minimal enteral intake of 110 kcal/kg per day by 21 days of age.

Effect of vegetable and marine oils in preterm infant formulas on blood arachidonic and docosahexaenoic acids.

Adding docosahexaenoic acid (DHA) (22:6n-3) to formulas is more effective than increasing formula alpha-linolenic acid (18:3n-3) in maintaining blood phospholipid DHA levels similar to those in breast-fed infants. However, in long-term trials supplementary DHA given as marine oil reduces blood phospholipid arachidonic acid (AA) in preterm infants. This effect is not seen in short-term trials unless the total n-3 intake from marine oil exceeds 0.

Long-term feeding of formulas high in linolenic acid and marine oil to very low birth weight infants: phospholipid fatty acids.

Red blood cell (RBC) phospholipids of infants fed human milk compared with formula have more arachidonic acid (AA) and docosahexanoic acid (DHA). The addition of low levels of marine oil to infant formula with 0.6 to 2.

Vitamin A status of preterm infants during infancy.

Plasma retinol and retinol-binding protein (RBP) were measured in 67 enterally fed preterm infants (750-1398 g) at 33 +/- 2 wk postconceptional age (PCA), and at regular intervals during infancy. Retinol and RBP declined by 35 +/- 2 wk PCA and remained low at 38 wk after discharge, with the infants fed a term-infant formula. At 38 +/- 2 wk PCA, 48% (32 of 67) of these infants had plasma retinol concentrations less than 0.

Vitamin D and mineral metabolism in the very low birth weight infant receiving 400 IU of vitamin D.

Study Objective: To examine (1) the effect of vitamin D intake (380 to 480 IU daily) on plasma 25-hydroxyvitamin D (25-OHD) and 1,25-dihydroxyvitamin D (1,25-(OH)2D) concentrations and (2) the relationship of 1,25-(OH)2D to calcium and phosphorus absorption and retention in the very low birth weight infant receiving a preterm infant formula.

Subjects: Eleven "well" infants with a birth weight and gestational age (mean +/- SD) of 1078 +/- 128 gm and 29 +/- 1.9 weeks, respectively, were studied for a 3-week period.

Psychometric characteristics of the Multidimensional Scale of Perceived Social Support.

The initial study describing the development of the Multidimensional Scale of Perceived Social Support (MSPSS) indicated that it was a psychometrically sound instrument (Zimet, Dahlem, Zimet, & Farley, 1988). The current study attempted to extend the initial findings by demonstrating the internal reliability, factorial validity, and subscale validity of the MSPSS using three different subject groups: (a) 265 pregnant women, (b) 74 adolescents living in Europe with their families, and (c) 55 pediatric residents. The MSPSS was found to have good internal reliability across subject groups.

Cardiac dysrhythmia with the use of clonidine in explosive disorder.

Clonidine has been used in the management of hypertension in adults with few cases of cardiac dysrhythmia reported. These appear to occur most frequently in association with preexisting cardiac disease or toxic concentrations of the drug. We observed a case of clonidine-induced bradycardia and irregular firing of the sinoatrial node in a child who does not have cardiac disease given low doses of the drug in the treatment of intermittent explosive disorder.

Preferential effects of caffeine on limbic and cortical dopamine systems.

In this study, we investigated the effects of acute caffeine administration on the activity of midbrain dopamine neurons. Caffeine significantly depressed the firing rates of dopamine neurons in the ventral tegmental area (A10 group), but had no significant effect on the firing rates of dopamine neurons in the substantia nigra zona compacta (A9 group). The action of caffeine in A10 was completely blocked by pretreatment with the adenosine agonist L-phenyl-isopropyl-adenosine (L-PIA), confirming numerous lines of evidence that caffeine and other xanthines act as competitive antagonists at adenosine receptors.