[Neonatal lupus erythematosus and neurologic involvement: an incidental association?]. / Lupus néonatal et atteinte neurologique: une association fortuite?

UNLABELLED: Neonatal lupus erythematosus is a rare disorder characterized by cutaneous lesions of the face and/or congenital heart block. The transplacental transfer of maternal anti-Ro/SSA, anti-La/SSB, or anti-U1RNP antibodies is responsible for the development of the disease. Few cases of neonatal lupus erythematosus with neurological involvement were reported in the medical literature. CASE REPORT: A 36-week GA female infant presented with neonatal lupus erythematosus comprising cutaneous, hematologic and hepatic disorders with a favorable outcome. However, cutaneous atrophy and hyperpigmentation persisted. Spastic paraparesis was diagnosed at the age of six months. CONCLUSION: The neurological lesions in neonatal lupus erythematosus could either be related to the presence of anti-Ro/SSA antibodies of maternal origin, or of anticardiolipin antibodies.

Trans fatty acids in adipose tissue of French women in relation to their dietary sources.

This study reports the fatty acid composition of subcutaneous adipose tissue in French women with special emphasis on the content of trans fatty acids originating from two main dietary sources, ruminant fats and partially hydrogenated vegetable oils (PHVO). Adipose tissue trans fatty acid levels from 71 women, recruited between 1997 and 1998, were determined using a combination of capillary gas chromatography and silver nitrate thin-layer chromatography. Results indicate that on average cis monounsaturates accounted for 47.9% of total fatty acids, saturates for 32.2%, and linoleic acid for 14.4%. Cis n-3 polyunsaturates represented only 0.7%. Total content of trans fatty acids was 2.32 +/- 0.50%, consisting of trans 18:1 (1.97 +/- 0.49%), trans 18:2 (0.28 +/- 0.08%), and trans 16:1 (0.06 +/- 0.03%). Trans 18:3 isomers were not detectable. The level of trans fatty acids found in adipose tissue of French women was lower than those reported for Canada, the United States, and Northern European countries but higher than that determined in Spain. Therefore, trans fatty acid consumption in France appears to be intermediate between that of the United States or North Europe and that of Spain. Based on the equation of Enig et al., we estimated the mean daily trans 18:1 acid intake of French women at 1.9 g per person. The major trans 18:1 isomer in adipose tissue was delta11 trans, as in ruminant fats. Estimates of relative contribution of trans fatty acid intake were 55% from ruminant fats and 45% from PHVO. This pattern contrasts sharply with those established for Canada and the United States where PHVO is reported to be the major dietary source of trans fatty acids.

[Maternal transport to the Bordeaux University Hospital: a retrospective study of 263 cases (1996-1998)]. / Le transfert des enfants in utero au Centre Hospitalier Universitaire de Bordeaux : analyse rétrospective de 263 cas (1996-1998).

OBJECTIVES: To describe a population of high risk pregnancies transported to a regional perinatal care centre (level III) and to analyze the advantages and limitations of the organization of this perinatal care network. METHODS: Retrospective study of 263 patients transported to the University Hospital of Bordeaux between September 1996 and September 1998. RESULTS: Maternal transport mainly came from the Gironde department (53%) and from level I care hospitals (66%). The rate of multiple pregnancies was 17%. Principal indications were preterm labor with or without rupture of fetal membranes (52%) and preeclampsia with or without intrauterine growth restriction (28%). The rate of returns to the referring care centre was 2%. The mean term at delivery was 32 WA. Forty-one percent of patients delivered within 24 hours of transport. The rate of caesarean sections was 55%. There were 303 live births. The mean duration of hospitalization of the neonates was 28 days. The rate of perinatal mortality was 6.2%. Among the 96 pregnancies beyond 32 WA, 17 (6.5%) could have been transported to some level IIb care hospitals, nearby the patient's residence, without changing maternal and neonatal prognosis. Neonatal mortality was not significantly different for maternel transport (18%) or neonatal transport (14%) in the group of premature infants born prior to 31 WA in our study. CONCLUSION: Our organization is currently trying to improve transport management (non-emergency situations, collaboration of level II care hospitals) and to increase the rate of returns to the referring care centres.

Sjögren-Larsson syndrome: early diagnosis, dietary management and biochemical studies in two cases.

BACKGROUND: Sjögren-Larsson syndrome (SLS) is a rare autosomal recessive disorder with worldwide distribution. It consists of ichthyosis, spastic diplegia and mental retardation caused by an enzymatic defect in fatty alcohol oxidation. OBJECTIVE: To study the effects of dietary management on clinical outcome and plasma/red blood cell fatty alcohol and plasmalogen concentrations. METHODS: To reduce fatty alcohol production, we reduced total fat intake to 30% of total intake of calories. To correct d 6 desaturase deficiency, we supplemented the diet with both n-3 and n-6 fatty acids to obtain a linoleic/linolenic acid ratio of 6 with low erucic acid rapeseed oil, plus high unsaturated fatty acids. We used gas liquid chromatography to assay blood cell membranes and plasma fatty alcohols/plasmalogens. RESULTS: Two SLS infants with proven fatty alcohol/NAD+ oxidoreductase deficiency were studied. Good clinical results were obtained in one of the patients when dietary intervention was started in early infancy and correlated well with plasma fatty alcohol decrease. However, no clinical improvement was seen in the other patient who started later with low compliance. Acitretin therapy was necessary to control skin symptoms in this second patient. CONCLUSION: Dietary intervention using the combined approach described here may improve fatty alcohol metabolism in SLS. However, only very early intervention seems clinically beneficial.

Localised proton magnetic resonance spectroscopy of the brain after perinatal hypoxia: a preliminary report.

OBJECTIVES: Perinatal hypoxic ischaemic injury is a significant cause of neurodevelopmental impairment. The aim of this study was to evaluate localised proton magnetic resonance spectroscopy (1H-MRS) after birth asphyxia. MATERIALS AND METHODS: Thirty newborn infants suspected of having perinatal asphyxia (Apgar score < 3) were studied. The mean gestational age was 37 weeks, mean age at the MR examination was 18 days and mean weight was 2.9 kg. A 1.5-T unit was used for imaging and spectroscopy. None of the babies had mechanically assisted ventilation. No sedation was used. Axial T1-weighted and T2-weighted images were obtained. 1H-MRS was recorded in a single voxel, localised in white matter, using a STEAM sequence. RESULTS: Image quality was good in 25 of 30 babies. 1H-MRS was performed in 19 of 30 subjects, with adequate quality in 16. Choline, creatine/phosphocreatine and N-acetylaspartate peaks and peak-area ratios were analysed. Lactate was detected in four infants. The N-acetylaspartate/choline ratio was lower in infants with an impaired neurological outcome, but the difference was not statistically significant. CONCLUSIONS: This study suggests that 1H-MRS may be useful for assessing cerebral metabolism in the neonate. A raised lactate level and decreased N-acetylaspartate/choline ratio may be predictive of a poor outcome. However, in our experience this method is limited by the difficulty in performing the examination during the first hours after birth in critically ill babies, the problems related to use of a monovoxel sequence, the dispersion of the ratios and the lack of determination of the absolute concentration of the metabolites.

Longitudinal multicentric study of plasma and red blood cell fatty acids and lipids in preterm newborns fed human milk.

The present multicentric study (three centers) deals with the values of plasma and red blood cell fatty acids obtained in a group of 18 preterm newborns after 2 days (D2), 15 days (D15) and 5 weeks (37th week postconception: 37th wk) of human milk feeding. Analytical methods were randomized between the three centers and quality control was evaluated by repeated analysis of reference samples. 20:4 n-6 varied from 10.71 +/- 1.58% to 9.51 +/- 1.65 and 10.10 +/- 1.42% in plasma phospholipids and from 16.59 +/- 3.30% to 14.68 +/- 3.14 and 18.24 +/- 4.09% in red blood cell phosphatidylethanolamine (RBC-PE) at D2, D15 and 37th wk, respectively, contrasting with the important rise of the precursor (18:2 n-6) in all the fractions studied. In RBC-PE, 22:6 n-3 significantly declined from 3.52 +/- 1. 03% at D2 to 2.56 +/- 0.83% at D15 (p < 0.02) and recovered its initial level at 37th wk (4.08 +/- 1.94%). The recovery of long chain polyunsaturated fatty acid homeostasis at that time was also confirmed by the decline of 16:1 n-7 in cholesterol esters (10.69 +/- 3.92 to 4.32 +/- 2.38%).

Sjögren-Larsson syndrome: an appraised of the litterature and presentation of two cases

A Síndrome de Sjögren-Larsson é uma doença autossômica recessiva rara com distribuiçäo universal. Consiste em ictiose, displegia espástica e retardo mental causado por um defeito enzimático na oxidaçäo do álcool-graxo. Nós relatamos dois casos e fazemos uma revisäo da literatura respectiva. As duas crianças tinham atividade deficiente da NAD oxidorredutase. Foram estudados os lípides de membrana das células plasmáticas e eritrócitos. Bons resultados foram obtidos em um dos pacientes quando submetido a dieta na infância precoce o que se correlacionou com diminuiçäo do álcool-graxo no plasma. Entretanto näo obtivemos melhora clínica no outro paciente cujo tratamento teve início tardio.Terapia com etretinato foi necessária para controlar os sintomas cutâneos neste segundo paciente.

Effects of preterm infant formula supplementation with alpha-linolenic acid with a linoleate/alpha-linolenate ratio of 6: a multicentric study.

OBJECTIVE: To investigate the effects of a milk formula supplemented with a alpha-linolenic acid (ALA) (18:2 n-6/18:3 n-3 ratio near 6/1) on plasma and red blood cell (RBC) fatty acids (FAs) in premature infants and compare with a non supplemented formula (18:2 n-6/18:3 n-3 = 22/1). DESIGN AND SUBJECTS: Infants of mothers who elected not to breast-feed were randomly assigned to either a high alpha linolenic formula (HLF: n = 31) group or a low alpha-linolenic formula (LLF: n = 32) control group. Infants fed human milk (HM: n = 25) were enrolled concurrently as a reference group. Anthropometric and biological measurements were made after two days (D2) and 15 d (D15) of enteral feeding and at the 37th week (W37) of postconceptual age. In HLF, the 18:3 n-3 content was 1.95% of total FAs (0.77% of total energy) and the 18:2 n-6/18:3 n-3 ratio was near 6/1. In LLF, the 18:3 n-3 content was 0.55% of total FAs (0.22% of total energy) and the 18:2 n-6/18:3 n-3 ratio was 22/1. RESULTS: ALA supplementation had minimal effect on the n-6 series, did not alter the anthropometric data and confirmed the conversion of ALA into docosahexaenoic acid (DHA). Throughout the study, it maintained, the RBC membrane DHA values within the confidence interval of those obtained in the HM group. Such was not the case with LLF CONCLUSION: alpha-linolenic acid supplementation (from Rapeseed oil and in a 18:2 n-6/18:3 n-3 ratio = 6) in premature infant formula can contribute efficiently to the maintenance of the n-3 status in the premature newborns.

Relationships between red blood cell vitamin E and polyunsaturated fatty acid in the premature infant.

Low-birth-weight infants were fed breast milk (BM, n = 25) for 1 of 2 formulas containing 0.5 (PG, n = 32) or 1.7% fatty acids as alpha-linolenic acid (ALA)(ALA-PG, n = 31). Polyunsaturated fatty acids (PUFA) and alpha-tocopherol (alpha T) of RBC membrane phospholipids were measured at days 2 (D2) and 15 (D15) of enteral feeding, and at term. PUFA concentrations did not differ significantly between the 3 groups from D2 to term. The membrane integrity assessed by the alpha T/phospholipids ratio neither changed with time nor differed between formula groups. A metabolic relationship between alpha T and long-chain PUFA in RBC membranes was suggested by multiple regression analysis: alpha T showed a significant positive correlation with docosahexaenoic acid at D15 and term and with arachidonic acid at D15; the correlation with arachidonic acid tended toward significantly (p = 0.06) at term.

[Prenatal diagnosis of a hydrothorax secondary to an extralobar pulmonary sequestration]. / Diagnostic prénatal d'un hydrothorax secondaire à une séquestration pulmonaire extralobaire.

A case of antenatal hydrothorax was observed. The aetiology could not be determined before birth. At birth an extra-lobe lung sequestation was treated surgically after 3 weeks in the intensive care unit. The surgical cure was successful with no sequellae. This favourable outcome is unusual in this type of pathology and can be explained by the fact that the sequestration was isolated in a fetus with only minimal anasarca. Indications for prenatal pleural drainage are discussed.

[Biological effects on premature neonates of a milk formula enriched with alpha-linolenic acid: a multicenter study]. / Effets biologiques, chez le prématuré, d'une supplémentation des formules lactées en acide alpha-linolénique: une étude multicentrique.

Nervous tissues and the retina are rich in docosahexaenoic acid [DHA, C22: 6(n-3)], an essential fatty acid which is the product of the elongation and desaturation of alpha-linolenic acid [alpha-LnA, C18:3(n-3)]. Lower plasma and membrane DHA levels as well as impaired nervous function (psychomotor development, visual disturbances) have been observed in premature babies receiving standard milk products compared to breast-fed babies and have been related to the lack of long-chain (> C20) fatty acids in these products. In the present study, we have investigated whether it is possible to increase the plasma and erythrocyte DHA level in premature infants by giving them a milk formula enriched in alpha-LnA (1.95% vs 0.55%). Results showed that, after 15 days of feeding, alpha-LnA supplementation significantly increased plasma DHA availability [2.20 vs 1.54% (p < 0.0001) in the plasma phospholipids] and increased DHA level in erythrocyte membranes [3.02 vs 2.54% (n.s.) in the red blood cell phosphatidylethanolamines]. At the LA/alpha-LnA ratio used (6/1), there was only a limited impact on the n-6 pathway which was reflected in a slight decrease in the arachidonic acid (AA) of the plasma phospholipids [6.88 vs 7.53% (p < 0.05) after 15 days]. Furthermore, the AA content of the red blood cell phosphatidylethanolamines was perfectly preserved even after a mean period of 4-5 weeks of feeding [(15.70 vs 14.86% (n.s)]. The observed enhancement in fatty acids of the n-3 series calls for a prospective study investigating whether these improvements are associated with changes in the kinetics of psychomotor development and in the visual function of enriched-formula-fed versus breast-fed babies.

Relative contribution of physical activity to neonatal oxygen consumption.

To explore the relevance of distinguishing between resting and global energy expenditure in newborn infants, oxygen consumption (VO2) was measured during extremes of physical activity in 17 parenterally fed newborn infants with a large range of body weights (1.0-3.4 kg) and gestational ages (28-41 weeks). Under constant nutrient intakes, each infant served as his/her own control when comparing VO2 during resting conditions and spontaneous intense physical activity, called exercise. VO2 was significantly correlated with body weight at rest (r = 0.96). But during intense activity, the better predictor of exercise-induced VO2 was body weight in the smaller infants (< 2.0 kg) and gestational age in the larger infants (> 35 weeks). The difference in VO2 between both levels of activity represented the oxygen cost of exercise, which decreased (P < 0.01) with body weight. For clinical purposes, the physical activity of low-birth-weight infants does not contribute substantially to their energy balance.

Respiratory gas exchange in response to fat-free parenteral nutrition: a comparison after thoracic or abdominal surgery in newborn infants.

Thoracic surgery is known to cause a postoperative respiratory failure because of the mechanical problems following chest wall disruption and/or diaphragmatic dysfunction. This study was to verify whether the fat-free intravenous nutritional support of neonates who underwent thoracic surgery could lead to a CO2 production exceeding the patients' respiratory reserves. Respiratory gas exchange and alveolar ventilation were obtained by indirect calorimetry and continuous recordings of transcutaneous PO2 and PCO2. These noninvasive measurements were compared at the same age of 7 +/- 1 days between a group of 7 newborn infants (mean +/- SEM: 3.09 +/- 0.14 kg, 39 +/- 1 weeks) after thoracic surgery versus a group of 8 newborn infants (2.88 +/- 0.17 kg, 37 +/- 1 weeks) after abdominal surgery. The intravenous macronutrient support was the same between both groups: 14 g/kg/d of glucose, 2 g/kg/d of amino acids, 250 kJ/kg/d of energy. One week after surgery, the global metabolic rate (195 kJ/kg/d) was not increased, and comparable between both groups. We documented that early after thoracic surgery, the ventilatory compensation required to handle the CO2 production (6.7 +/- 0.2 mL/kg/min) associated with a positive energy balance (45 +/- 8 kJ/kg/d) was effective.

Townes-Brocks syndrome in an infant with translocation t (5;16).

We report the occurrence of Townes-Brocks syndrome (TBS) in an infant with a two break reciprocal translocation between chromosome 5 and chromosome 16. The occurrence of both abnormalities in the same subject could be due to chance. However, it is of interest to note that a familial case of TBS associated with an inv(16) with the same breakpoint at 16q12.1 has been reported. We suggest the possible disruption of the TBS gene at this breakpoint.

[Maternal-fetal transmission of genital mycoplasma infection]. / Infection materno-foetale à mycoplasmes génitaux.

The authors report on a case of a preterm infant who suffered from pneumonia due to genital mycoplasma infection. Hepatic dysfunction was associated. Tetracyclines were successful.

Energy expenditure and severity of respiratory disease in very low birth weight infants receiving long-term ventilatory support.

We attempted to determine whether the hypermetabolism of infants with bronchopulmonary dysplasia was detectable during assisted ventilation. Respiratory gas exchange variables were measured with a metabolic gas monitor in 10 infants under similar nutritional conditions. Oxygen consumption increased linearly with the need for ventilatory support (R2 = 0.75), as documented by the ventilatory index.

Isolation of genital mycoplasmas from blood of febrile obstetrical-gynecologic patients and neonates.

In a prospective study, 1156 blood specimens collected from hospitalized febrile obstetrical-gynecologic patients and neonates with suspected sepsis, were inoculated into a conventional biphasic culture medium, Castaneda S and cultures incubated aerobically. 15-24 h later the broth cultures were subcultured to specific media for detection of mycoplasmas. Genital mycoplasmas were isolated in 15 samples (taken from 8 women) and in 2 from 1 neonate. Mycoplasmas and members of the family Enterobacteriaceae were the most frequent significant bacteria isolated from adult specimens. Mycoplasma isolations were associated with either postpartum or postabortum febrile infections in women. Four of the neonates, whose mothers were infected, showed respiratory distress at birth; 1 of them had mycoplasmas in the blood. All febrile states in obstetrical or gynecological patients, and in neonates, should routinely lead to blood cultures for detection of mycoplasmas and ureaplasmas.

Gastric emptying in infants with or without gastro-oesophageal reflux according to the type of milk.

The present study was designed to determine the effects of milk composition and the influence of gastro-oesophageal reflux (GOR) on gastric emptying. Cineoesogastrocintigraphy (COGS) was performed in 201 infants between 0-1 year of age in order to detect GOR, and provided a means of estimation of the gastric emptying (GE). Ninety infants appeared free from GOR and constituted the control group; 111 had GOR. There infants were fed human milk or various standard formulae. In addition, 20 infants fed a whey-hydrolysate formula were tested. An appropriate volume of milk was marked with sulfur-colloid Tc (200 microcuries). Measurements of gastric radioactivity were made 30 min and 120 min after ingestion. For the whole population, the infants with GOR had slightly more rapid GE after 30 min (P less than 0.05), but, for the same type of milk, there was no significant difference between GOR and controls. GE did not differ with age or sex, but differed mainly according to the type of milk. In the control group, gastric residual content (GRC) at 120 min was 18 +/- 11 per cent with human milk (n = 7), 16 +/- 21 per cent with whey-hydrolysate formula (n = 8), 25 +/- 17 per cent with acidified formula (n = 13), 26 +/- 19 per cent with whey-predominant formula (n = 22), 39 +/- 17 per cent with casein-predominant formulae (n = 20), 47 +/- 19 per cent with follow-up formulae (n = 16) and 55 +/- 19 per cent with cow's milk (n = 12).(ABSTRACT TRUNCATED AT 250 WORDS)