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Biomedical subjects

A Menget

Publications and source records attributed to A Menget.

At least 19 recordsLinked to original sources

[Involvement of thyroid gland at non-Hodgkin lymphoma initial diagnosis: 2 pediatric cases].

Extranodal thyroid lymphomatous involvement is rare in childhood. We report here 2 children, 1 with vertical transmission-acquired human immunodeficiency virus (HIV), presenting with lymphomatous infiltration of the thyroid gland at diagnosis. One child had infra-clinical endocrine impairment and both responded well to chemotherapy. Although the cases are too scarce to be affirmative, thyroid gland involvement doesn't seem to alter the good prognosis of childhood Burkitt's lymphoma. The third child's cancer in frequency is Non-Hodgkin Lymphomas. Presenting as the initial AIDS event in 1 patient, this case report also highlights the need to systematically propose antiretroviral therapy in vertically HIV infected children.

Child, Preschool↗

Does smoking in pregnancy modify the impact of antenatal steroids on neonatal respiratory distress syndrome? Results of the Epipage study.

OBJECTIVES: To assess the relation between cigarette smoking during pregnancy and neonatal respiratory distress syndrome (RDS) in very preterm birth, and to analyse the differential effect of antenatal steroids on RDS among smokers and non-smokers. DESIGN: A population based cohort study (the French Epipage study). SETTING: Regionally defined births in France. METHODS: A total of 858 very preterm liveborn singletons (27-32 completed weeks of gestation) of the French Epipage study were included in this analysis. The odds ratio for RDS in relation to smoking in pregnancy was estimated using a logistic regression to control for gestational age. The odds ratio for RDS in relation to antenatal steroids was estimated taking into account an interaction between antenatal steroids and cigarette smoking, using multiple logistic regression to control for gestational age, birthweight ratio, main causes of preterm birth, mode of delivery, and sex. RESULTS: The odds ratio for RDS in relation to smoking in pregnancy adjusted for gestational age (aOR) was 0.59 (95% confidence interval (CI) 0.44 to 0.79). The aOR for RDS in relation to antenatal steroids was 0.31 (95% CI 0.19 to 0.49) in babies born to non-smokers and 0.63 (95% CI 0.38 to 1.05) in those born to smokers; the difference was significant (p = 0.04). CONCLUSIONS: Cigarette smoking during pregnancy is associated with a decrease in the risk of RDS in very preterm babies. Although antenatal steroids reduce the risk of RDS in babies born to both smokers and non-smokers, the reduction is smaller in those born to smokers.

Female↗

[Neonatal resuscitation in the delivery room: educational process and evaluation].

Based upon a three necessities basis: public health, biological and medico-legal, this article presents the state of the art about teaching neonatal resuscitation in the delivery room. The educational process is present worldwide; main experiences are described. Evaluation of these actions varies in the literature. We analyze the evaluation of the process of the trained professionals, their satisfaction, the changes in their practices, their theoretical and practical levels, and the impact on newborns' health. We propose a few measures to make official this kind of teaching in France, with a certificate for instructors and trained professionals.

Delivery Rooms↗

Acute vanishing bile duct syndrome after ibuprofen therapy in a child.

We report the case of a 10 year-old girl who had Stevens-Johnson syndrome and cholestasis after ibuprofen therapy. Liver histology was compatible with vanishing bile duct syndrome. She received ursodeoxycholic acid, and liver tests normalized within 7 months. This report confirms that ibuprofen may induce acute vanishing bile duct syndrome.

Acute Disease↗

[Obstetrical and neonatal prognosis of monoamniotic twin gestations].

Monoamniotic twin gestations result from a late division of the fertilized ovum with development of the two embryos within a common amnionic sac. We performed a retrospective study in our university hospital on nearly 19 years of clinical activities; we found 16 cases of monoamniotic twins. Obstetrical and neonatal outcomes were analyzed and compared to the data in the literature. The pronosis after 28 weeks of gestation does not appear to be influenced by this type of twin gestation and we demonstrate in this study that vaginal delivery is possible and not deleterious.

Adult↗

[An appreciation of maternal smoking with high-performance liquid chromatographic determination of maternal and neonatal urinary cotinine].

OBJECTIVE: To study the correlation of urinary cotinine levels in mothers and newborns with the number of cigarettes smoked at the end of pregnancy. Population and methods. We recorded the smoking habits of 123 mothers attending a university maternity clinic and measured urinary cotitine levels in mothers and their newborns. All mothers were Europeans and gave birth to a normal full-term (37 weeks gestation) infant. Cotinine levels were measured with high-performance liquid chromatography from urine samples taken during the 6-hour period prior to or after delivery for the mothers and 24-h after birth for the newborns. RESULTS: The average cotinine level for non-smoking mothers, for those who smoked one to nine cigarettes a day and heavy smokers (ten or more cigarettes per day) were 0.21, 2.17 and 4.28 mol/l respectively (p<0.001). The average levels in their newborns were 0.04, 0.39 and 1.36 mol/l respectively (p<0.001). Thirteen percent of the mothers who claimed they did not smoke had cotinine levels higher than the significance cut-off (0.3 mol/l). There was a significant correlation 1) between the number of cigarettes the mothers stated they smoked at the end of pregnancy and their urinary cotinine concentrations (cotinine level=0.213 + 0.349 cigarettes, r=0.78, p<0.001); 2) between the number of cigarettes smoked and newborn's urinary cotinine concentration (cotinine level=0.002 + 0.104 cigarettes/day, r=0.81, p<0.001); and 3) between the mother's and the newborn's urinary cotinine concentrations (newborn cotinine=0.027 + 0.219 maternal cotinine, r=0.77, p<0.001). CONCLUSION: The number of cigarettes smoked at the end of pregnancy accounts for roughly 50% of the variance in the mother's urinary cotinine level and that in her newborn at birth. The urinary cotinine concentration in newborns is 3 to 5 times lower than that of their mothers. A woman smoking 3 cigarettes per day has a urinary cotinine concentration of 1 mol/l. The urinary cotinine level in newborns is 1 mol/l for mothers smoking 10 cigarettes per day.

Chromatography, High Pressure Liquid↗

[For what reasons is the neurologic status of very premature infants altered between 1 and 2 years in a follow-up study? The contribution of a Frache-Comté region study].

BACKGROUND: The purpose of this population-based study was firstly to compare the neuro-developmental outcome at one and two years of very preterm infants, and secondly, to identify the risk factors for a misdiagnosis of cerebral impairment at the age of one year. POPULATION AND METHODS: The preterm cohort included 203 infants born between 25 and 32 weeks of gestational age in the region of Franche-Comté (France) during a two-year period. The control group included 196 full-term infants born in the same maternity wards. Neuro-developmental assessments were performed by pediatricians or physicians, both at one and two years of age, on 94% (161/171) surviving preterms and 89% (173/195) full-terms. RESULTS: There is a fair correlation between the two neurological evaluations of the control group (170/173, 98% have the same classification at the age of one and two). There is a weak correlation (kappa = 0.37) between the two neurological evaluations of the preterm group. Sixteen preterms (10%) had been classified more abnormal at one year than they were at two years. The presence of a broncho-pulmonary dysplasia, linked to male sex and extreme prematurity, was statistically linked to this first kind of misclassification. Seventeen preterms (10%) had been considered more normal at one year than they were at two years. The presence of a diplegia, family precariousness and the examination at one year of age by a general practitioner were statistically linked to this second kind of misclassification. CONCLUSION: This prospective population-based study identifies structural situations (bronchopulmonary dysplasia linked to extreme prematurity) and environmental situations (family precariousness, examiner's qualifications) linked to a misclassification of the neurological status of one-year-old former preterm infants.

Age Factors↗

[Neurodevelopmental outcome of premature infants born at less than 33 weeks of gestational age and not cerebral palsy at the age of 5 years].

AIM: To appreciate the impact of prematurity, fetal hypotrophy and familial environment on the neurodevelopmental performances of very premature infants without cerebral palsy at the age of five years. POPULATION AND METHODS: We followed a regional cohort of 171 very premature infants (< or = 32 weeks of gestation) until they were five years of age. Cognitive functions were tested with the WPPSI test and the development quotient was assessed by the ability to draw a "bonhomme". Twenty-two premature infants suffered from cerebral palsy diagnosed before the age of two years. Another infant had a moderate diplegia at the five-year examination. We had no information for 16 prematures (9.3% of survivors). Twenty-eight premature infants were considered as having no severe disability on phone or mailed contact, and another child had a severe isolated mental retardation. We examined 104/148 infants, and 96/148 survivors without cerebral palsy passed the tests. The cognitive functions of these premature infants are compared to the performances of a control group made up of 108 children born at term > or = 37 weeks, matched for birthplace and single or twin characteristics of the pregnancy. RESULTS: The values of the different quotients are significantly decreased in the preterm group. The global IQ and the performance IQ are 0.8 SD, verbal IQ is 0.5 SD and the development quotient is 0.4 SD below the values observed in the control group. A performance IQ less than -2 SD for the mean of the control group is observed three times more than in the controls (13.5% vs 3.7%, P < 0.01). Multiple linear regression shows that prematurity explains, independent of hypotrophy and socioeconomic environment, 8% of the variation of the performance IQ (P < 0.01), 2% of the variation of the verbal IQ and 2% of the development quotient (P < 0.05). CONCLUSION: The five-year neurologic outcome of the children born prematurely in this regional study is similar to the results observed in regional studies conducted in Europe: 13.4% of the survivors have cerebral palsy, and the cognitive functions of the children with no cerebral palsy are significantly lower than the term control group. Other risk factors such as hypotrophy, which modulates the developmental quotient, and the socioeconomic status, which modulates the verbal IQ, are underlined.

Cerebral Palsy↗

[Smith-Lemli-Opitz syndrome].

BACKGROUND: Smith-Lemli-Opitz syndrome (SLOS) is an autosomic recessive metabolic affection. Children affected by SLOS exhibit a defect in cholesterol biosynthesis associated with a high concentration of cholesterol precursor 7 dehydrocholesterol (7 DHC) and its isomers, which is due to an enzymatic block at the level of delta-7-DHC reductase. SLOS has been subdivided into two types on the basis of clinical severity: type I is the classic and type II is the severe one. CASE REPORT: A full term female was born from a pregnancy complicated by oligoamniosis and intra-uterine growth retardation. The neurologic status was immediately impaired with severe hypotonia, absence of reflexes, and abnormal crying. She exhibited multiple congenital anomalies with a facial dysmorphia, anomalies of members, unicornus uterus and a pyloric stenosis. Plasmatic concentration exhibited a normal cholesterolemia contrasting with an elevated level of 7 and 8 DHC. Major alimentary tract defect led to enteral and parenteral nutrition. The severe neurological defect led to death on the 16th day of life. CONCLUSION: Despite normal blood cholesterol levels that can be attributed to enteral and parenteral nutrition, the severity of clinical findings and the lethal course permit to classify this case as type II.

Adult↗

Some risk factors for cerebral palsy in very premature infants: importance of premature rupture of membranes and monochorionic twin placentation.

OBJECTIVE: To delineate the perinatal risk factors of neurodevelopmental disabilities in very preterm birth applying logistic regression analysis. DESIGN: This prospective, geographically defined collaborative study was carried out in the Franche-Comté region of France. SUBJECTS: From October 1, 1990 to September 30, 1992, perinatal and follow-up data were collected on 203 consecutive live-born singleton or twin non-malformed infants with strictly ascertained gestational ages of less than 33 weeks. MAIN OUTCOME MEASURE: The rate of cerebral palsy and/or severe mental retardation as diagnosed by a family physician or pediatrician with a screening-skill test performed at 2 years of age. RESULTS: 167/171 (98%) survivors were evaluated. Twenty-two of the 167 examined infants (13%) showed signs of cerebral palsy, and 10 of these had severe cerebral palsy or mental retardation. Risk factors for disabilities were selected by a multivariate approach: premature rupture of membranes >/=48 h (OR 4.3, 95% CI 1.6-11.8); monochorionic twin placentation (OR 6.0, 95% CI 1.7-21.3), and respiratory distress syndrome (OR 2.8, 95% CI 1.1-7.1). CONCLUSION: This geographically defined prospective study gives epidemiological data and highly suggests that there is a link between prenatal events (premature rupture of membranes, monochorionic twin placentation), postnatal events (respiratory distress syndrome), and neurological disabilities in former preterm infants.

Birth Weight↗

Apparent segregation of null alleles ascribed to deletions of the ornithine transcarbamylase gene in congenital hyperammonaemia.

Ornithine transcarbamylase (OTC) deficiency is an X-linked disorder of the urea cycle mapped to chromosome Xp21.1. Here, we show that apparent segregation of null alleles at the OTC locus and flanking polymorphic loci mimicked false maternity or false paternity in three affected families. Based on these observations, we suggest giving consideration to gene deletion when dealing with segregation of null alleles in OTC deficiency.

Alleles↗

[Neurologic development in premature infants under 33 weeks of gestational age: determination of risk of neurological abnormalities in a prospective regional survey with a control group].

AIM: The purpose of this population-based study was to compare the incidence of neurodevelopmental disability and its risk factors between preterm and full-term infants matched as control group. POPULATION AND METHODS: The preterm cohort included 203 infants born between 25 and 33 weeks of gestational age in the region of Franche-Comté (France) during a two-year-period. The control group included 196 full-term infants born in the same maternities. Survival up to the date of follow-up was 171/203 (84%) for preterms and 195/196 (99.5%) for full-term infants (uncorrected age, mean 12 months). Neurodevelopmental assessments were performed by pediatricians or physicians on 164/171 surviving preterms (96% follow-up) and 179/195 full-terms (92%). RESULTS: Thirty-two (19.5%) preterm infants had disability, ten of these (6%) showing severe disability. Five (2.8%) full-term infants had disability, one of these (.5%) having severe disability. Risk factors predicting a disability included in a multivariate approach: prematurity (odds-ratio [OR]: 7.8), maternal age > 37 (OR: 3.0), lack of profession for both parents (OR: 3.7), male gender (OR: 2.9). The pediatrician observed a disability more frequently than the physician (OR: 2.46). Likewise, risk factors predicting a severe disability included: prematurity (OR: 10.8), lack of profession for both parents (OR: 5.8) and monochorial twin-placentation (OR: 4.5). CONCLUSIONS: Prematurity is not the only risk factor to be taken into account for neurodevelopmental evaluation of premature infants, but its influence still remains widely predominant.

Child Development↗

[Effectiveness of and tolerance to human recombinant erythropoietin in the treatment of kidney failure anemia in children undergoing continuous peritoneal dialysis. Multicenter study].

Eight young children with renal failure, undergoing continuous peritoneal dialysis (CDP) and presenting an anemia (hemoglobin level [Hb] 57 to 89 g/l) were treated by subcutaneous recombinant human erythropoietin (rHu EPO) twice weekly. The initial dose of 75 U/kg was adjusted to induce progressive increase of Hb with a target level of 100-120 g/l. Treatment duration was 24 weeks in five of these children and 10 to 13 weeks in the three others. In seven cases out of eight, anemia was corrected. The target Hb level was reached in 3 to 21 weeks with rHu EPO doses of 150 to 300 U/kg/w (mean: 200 U/kg/w) for four children without recent transfusion; then the median maintenance dose was 135 U/kg/w (range: 50-300 U/kg/w). In only one patient, Hb never reached a level higher than 77 g/l despite weekly dose of 350 U/kg, a reticulocytosis of 5.6%, rHu EPO treatment lasting up to 24 weeks and the absence of iron deficiency. In any case, no transfusion was necessary after the first day of rHu EPO treatment. In three patients, the increase of a preexisting hypertension required the adaptation of antihypertensive treatments. One patient presented a marked thrombocytosis. In conclusion, twice-a-week subcutaneous injections of 75 to 150 U/kg of rHu EPO appear to be well tolerated and effective in the treatment of anemia of CPD children.

Anemia↗

[Choanal atresia and CHARGE syndrome].

The acronym CHARGE names a syndrome described by Pagon et Graham in 1981, which associates mainly ocular anomalies, i.e. coloboma, and uni or bilateral choanal atresia. We report three observations of children with choanal atresia confirmed by CT scan. One child exhibited all the signs of the CHARGE syndrome including cerebral involvement at autopsy.

Abnormalities, Multiple↗

[Bourneville tuberous sclerosis manifested by prenatal finding of intracardiac tumors].

Neonatal cardiac rhabdomyoma is the most frequent cardiac tumour in the newborn, and a classical way to diagnose tuberous sclerosis (Bourneville's disease). The authors report 4 cases, including 2 antenatal diagnosis: 2 of them had arrhythmia, one with asystolic cardiac failure and the other with cyanosis due to a right-left shunt; the tumour was asymptomatic in the 2 others. The 4 babies had clinical and radiological neurologic signs of tuberous sclerosis, initially or during the course of the disease. The authors discuss the polymorphism of the clinical presentation of tuberous sclerosis in the newborn. Although tuberous sclerosis is transmitted through a dominant gene, the expressivity and the penetrance are very variable. The authors discuss the examination and investigation of at risk family members. The genetic counsel is difficult but very important because of the poor neurologic prognosis of the disease.

Female↗