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Biomedical subjects

D Rieu

Publications and source records attributed to D Rieu.

At least 37 records · Page 2Linked to original sources

[Protein and energy needs of the infant with severe malnutrition. Application in a hospital environment for the treatment of malnutrition caused by deficient intake].

Severe malnutrition is defined by a weight for height below 70% of international standards or by presence of oedema in a clinically undernourished child. Severe malnutrition associated with oedema is called kwashiorkor. The origin of oedemas of kwashiorkor is still debated, but its relation with protein deficiency is strongly questioned. The same dietary management is now recommended for malnutrition with or without oedema. Present recommendations are based, as for well nourished children, on the separate estimation of nutritional requirements for maintenance and growth. Total requirements vary between 0.7 g/kg/day in the first few days of treatment to 5 g/kg/day or more when weight gain is maximum. As a result of high energy requirement during catch-up growth, protein requirements never exceed 10 to 12% of total energy needs.

Child Nutrition Disorders↗

Circulating oxyntomodulin-like immunoreactivity in healthy children and children with celiac disease.

BACKGROUND: The aim of the study was to evaluate the new hormonal entity oxyntomodulin-like immunoreactivity in malabsorption states, and to assess its potential in celiac disease management. METHODS: We measured basal and postprandial oxyntomodulin-like immunoreactivity values in 35 children divided into 3 groups: group 1 was composed of 13 children with celiac disease, either under a gluten-free diet (8 patients) or normal diet (5 patients); group 2 was composed of 8 children hospitalized for gastroenteritis or chronic diarrhea, without biological evidence of malabsorption nor abnormal jejunal mucosa; group 3 was composed of 22 control subjects. RESULTS: Fasting and meal-stimulated levels in the control group were 71+/-10 and 130+/-26 pmol/l, respectively. Mean concentrations were elevated in patients with celiac disease (basal = 349+/-254 pmol/l, postprandial = 446+/-332 pmol/l) and in the group 2 (basal = 139+/-58 pmol/l, postprandial = 218+/-85 pmol/l), but the difference with control subjects did not reach statistical significance. In children with celiac disease, basal and stimulated values correlated with the degree of malabsorption as assessed by hemoglobin (p = 0.006 and p = 0.01, respectively) and serum folate concentrations (p = 0.03 and p = 0.02, respectively). CONCLUSIONS: Oxyntomodulin-like immunoreactivity is noticeably higher in healthy children than previously measured in healthy adult subjects. This hormonal parameter is not an adequate diagnostic tool in celiac disease. Nevertheless, in the context of celiac disease, its elevation reflects the degree of malabsorption and may provide a quantitative approach of the extent of mucosal damage.

Biomarkers↗

[Iron and pregnancy].

Infants, young children, and childbearing aged women are particularly exposed to iron deficiency. Pregnancy further increases iron requirements. Nevertheless the consequences of anemia and/or iron deficiency on pregnancy outcome, development of the foetus and postnatal iron status of the infant, remain to be determined. There is a 3-fold increase of premature deliveries in iron deficient anemic pregnant women whose anemia is discovered in early pregnancy: however this increased risk of premature delivery is not observed when iron deficiency anemia is discovered in late pregnancy. Iron supplementation during pregnancy improves the maternal hematological parameters but it is still unclear whether it also improves the maternal health and the pre and postnatal development of the child. Based on our actual knowledge, iron supplementation during pregnancy is to be recommended in risk groups only (ie mainly adolescents, low income women, women with multiple pregnancies), using ferrous iron at a dosage of 30 mg per day.

Anemia, Iron-Deficiency↗

Bioelectrical impedance analysis in small- and appropriate-for-gestational-age newborn infants.

OBJECTIVE: To determine the reliability of bioelectrical impedance analysis, and to compare and contrast the anthropometric and BIA status of newborns. DESIGN: BIA and anthropometric parameters were compared in the few days after birth and at about 3 weeks of age. SETTING: At the maternity hospital or in a paediatric care unit. SUBJECTS: Small- or appropriate-for-gestational-age newborns, with birth weight below or above the 10th percentile of the reference value, respectively. Measurements were performed on 36 and 47 newborns at birth, and for a subgroup (21 and 11) again about 3 weeks later, respectively. RESULTS: At birth, length2/resistance was 4.3 +/- 0.6 and 6.1 +/- 1.2 cm2/omega (P = 10(-7)), and at 3 weeks of age length2/resistance was 5.0 +/- 0.6 and 5.7 +/- 0.8 cm2/omega (P = 0.11), for small- and appropriate-for-gestational-age newborns, respectively. Percentage reliability was 2.2% and 2.6% for intra- and inter-observer measurements of resistance. Importance of a correct placement of the sensor electrode was demonstrated. CONCLUSIONS: Ease of measurement and reliability of BIA in neonates were shown. Evolution of BIA values is in agreement with the known increase in total body water linked to regrowth of cell mass in small-for-gestational-age infants. Additional study is required before BIA should be used in usual clinical setting in newborns due to the lack of prediction equation.

Age Factors↗

[Antigliadin, antireticulin, antiendomysium antibodies: value in the diagnosis and follow-up of celiac disease in children].

In recent years, the clinical spectrum of coeliac disease has changed and forms with mild aspecific symptoms are today frequent. Therefore many infants are submitted to jejunal biopsy in order to exclude coeliac disease or to allow an early diagnosis. This has led to a search for a simple and reliable diagnostic test of coeliac disease in order to limit the use of jejunal biopsy. Recent data suggest that the study of serum antigliadin, antireticulin and antiendomysium antibodies may possibly play the role. In this paper the working group on coeliac disease of the Groupe Francophone de Gastroentérologie et Nutrition Pédiatriques expresses its view on the place of the dosages of these antibodies in the diagnosis and follow up procedures of coeliac disease in infants and children. At the present time, although it allows a simplification of the procedures, these dosages are presently not sufficiently reliable to serve as a substitute of jejunal biopsy.

Autoantibodies↗

Evidence that liver microsomes of human neonates desaturate essential fatty acids.

delta 6- and delta 5-Desaturation of essential fatty acids of n-6 and n-3 series are required for the biosynthesis of polyunsaturated fatty acids (PUFAs), which are precursors of eicosanoids and constituents of membrane phospholipids. This pathway could be of special importance during the perinatal period, when PUFAs accretion in the central nervous system is very active. However, experimental evidence of delta 6- and delta 5-desaturase activities in man is very scarce, and no data are available for newborns. We report the delta 6- and delta 5-desaturase activities detected in human liver microsomes from three neonates who died from associated malformations. Radiochemical assays of delta 6- and delta 5-desaturase activities performed with reverse phase HPLC analysis of the products in the n-6 series ranged from 4.8-13.6 to 3.2-16.4 pmol substrate converted.min-1.mg-1 microsomal proteins, respectively. In the n-3 series delta 6-desaturase activity ranged from 5.3 to 12.8 pmol.min-1.mg-1. The relationships between enzyme activities and substrate concentrations suggest excess substrate inhibition for n-6 and not for n-3 fatty acids. These results demonstrate significant delta 6- and delta 5-desaturase activities in human liver of neonates, but this activity was lower than previously reported in adult humans and in mammals, especially rodents.

Delta-5 Fatty Acid Desaturase↗

Nervonic acid in red blood cell sphingomyelin in premature infants: an index of myelin maturation?

The present study addresses the question whether nervonic acid (24:1n-9) accumulation in sphingomyelin (SM) of red blood cells (RBC) could yield information on cerebrum maturation in premature infants. The study included 28 premature eutrophic infants of 31.5 wk gestational age. Eleven were fed with human milk, nine with a regular formula and eight with an alpha-linolenate-enriched formula. The fatty acid composition of the SM fraction was determined by gas-liquid chromatography on a 50-m fused silica capillary column. At 32 wk gestational age, the main fatty acids in SM were 16:0, 18:0, 20:0, 22:0, 24:0 and 24:1n-9. After five weeks of feeding, at week 37 of postconceptional age, the most striking variation was a rise in 24:1n-9, from 9.9 +/- 0.7 to 12.8 +/- 0.9 (P < 0.02), regardless of regimen in all three feeding groups. The rise in 24:1n-9 after birth in premature eutrophic infants is the beginning of a trend toward the higher levels in 24:1n-9 observed in mature newborns and older infants. The 24:1n-9 level in SM of RBC from premature infants may reflect 24:1n-9 levels in SM of brain and could thus reflect brain maturity.

Biomarkers↗

Multiple congenital anomalies associated with an oto-palato-digital syndrome type II.

We report the case of a male fetus with an oto-palato-digital (OPD) type II syndrome and multiple congenital anomalies (MCA) including omphalocoele, hypospadias, thoracic dysplasia, skeletal abnormalities, pulmonary hypoplasia and an absent right adrenal gland. These MCA are sometimes reported in Melnick-Needles syndrome, which leads us to discuss the possibility that the spectrum of malformations in these two syndromes might be due to two allelic forms of the same X-linked gene.

Abnormalities, Multiple↗

Interstitial deletion of the distal long arm of chromosome 4.

We report the first case of an interstitial deletion of the distal long arm of chromosome 4 (q31.22----q34.2). The major clinical features are described and compared to those of other published reports of del 4q, mainly those sharing a common deleted segment with the present case (both interstitial and terminal). This comparison suggests that the characteristic phenotype attributed to terminal deletions of 4q31----qter probably mainly results from loss of the segment q31----q33-34.

Abnormalities, Multiple↗

Brainstem conduction time abnormalities in small for gestational age infants.

Brainstem auditory evoked responses (BAER) were recorded in 89 neonates born between 32 and 40 weeks, in order to analyze the consequences of intrauterine growth retardation on brainstem conduction time. The I-V interval was measured in 28 appropriate for gestational age (AGA) infants (control group), in 24 small for gestational age (SGA) infants with maternal hypertensive disease (MHD) and in 37 SGA infants without MHD. At any gestational age, SGA infants with MHD always presented an acceleration of their brainstem conduction time as compared to the other SGA infants. For the SGA full-term twins without MHD, the brainstem conduction time was delayed. These results reflect the specific consequences on brainstem development of the various factors responsible for intrauterine growth retardation (IURG).

Brain Stem↗

Multiple congenital anomalies due to partial 2p13----2pter duplication resulting from an unbalanced X;2 translocation.

It has been suggested that partial distal 2p2----2pter duplication causes a relatively well defined clinical syndrome, mostly as regards craniofacial dysmorphism, musculoskeletal and genitalia anomalies. Duplications covering a larger portion of 2p i.e. 2p12 or 2p13----2pter are however less documented. The authors report a new case of partial 2p13----2pter duplication which supplies further evidence for short life expectancy due to the large number of malformations in these partial duplications.

Abnormalities, Multiple↗

Apert syndrome with partial preaxial polydactyly.

Acrocephalosyndactyly type I or Apert syndrome is characterized by craniosynostosis, particular dysmorphic features and abnormalities of the hands and feet. Rarely, polydactyly of the toes has been reported, and in this event the diagnosis of Carpenter syndrome must be discussed. A case of atypical Acrocephalosyndactyly type I syndrome with partial preaxial polydactyly is reported. Despite this preaxial polydactyly a diagnosis of Apert syndrome consecutive to a new mutation was made, and the possibility of recurrence considered to be highly improbable.

Acrocephalosyndactylia↗

[Use of flow cytometry in the study of a non-specific immunodeficiency in children. A case].

Using flow cytometry, we explored a case of nonspecific immunodeficiency in a seven month old girl with repeated infections. This method showed evidence of granulocyte phagocytosis and oxidative metabolism abnormalities suggesting the diagnosis of a variant form of chronic granulomatous disease (CGD). Findings also showed that flow cytometry can be useful to study phagocytic cells during the neonatal period as it allows rapid multiparametric analysis with a very small amount of blood.

Complement C3b Inactivator Proteins↗

Epidemiology of diaphragmatic hernia in Languedoc-Roussillon.

The frequency of diaphragmatic hernia (DH) varies, according to the studies, between 1/2000 and 1/7000. In the Languedoc-Roussillon (South of France), due to the presence of a Regional Foeto-Pathology Department and Medico Surgical Paediatric Department, it was possible to itemize all of the DH over a 24 month period (June 1989----May 1991). 20 children presenting DH (10 foetuses and 10 liveborns) were examined for a total population of 49.350 foetuses and liveborns (frequency of DH: 0.40/1000). 10 DH were associated with extra-pulmonary malformations (50%). 4 chromosome abnormalities were found (20%). Prenatal chromosome analysis in cases of ultrasound malformation detection has increased the number of karyotype abnormalities diagnosed.

Chromosome Aberrations↗