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D Luton

Publications and source records attributed to D Luton.

At least 19 recordsLinked to original sources
PubMed2006

[Specific follow-up for pregnant patients with a thyroid dysfunction].

Association between thyroidian disease and pregnancy is a frequent event. Thyroidian hormones are mandatory for foetal development especially at the level of brain structures. Any shortage of thyroidian hormone can severely and irreversibly alter neurological development. On the other hand it is also clear that an excess of thyroidian hormone can jeopardize the embryo then the foetus. In case of maternal hyperthyroidism, strict guidelines relying mainly on foetal thyroid monitoring echographic scanning will allow in most cases the delivery of a healthy euthyroid newborn. Hypothyroidia, providing an adequate substitution, has no significant impact on pregnancy. Biological monitoring is the key of monitoring. Iodine deficiency is a matter of concern when considering neurodevelopmental outcome, however it is still an unsolved issue in France. A multidisciplinary team will sometimes be necessary for taking care of pregnant patients with active Graves' disease.

Adult↗
PubMed2006

Prenatal Marfan syndrome: report of one case and review of the literature.

OBJECTIVES: Our objective was to describe the features of prenatal Marfan syndrome. METHODS: Doppler fetal echocardiograms were performed. The morphology and rhythm of the fetal heart were examined sequentially. RESULTS: The case was referred because of cardiomegaly and dilated great vessels. Sequential Doppler echocardiographic evaluation led to the diagnosis of prenatal Marfan syndrome. The main features are cardiomegaly, dysplastic atrioventricular valves with tricuspid regurgitation and dilated great vessels, which can be aneurysmal at their origin. The fetus died in utero at 39 weeks of gestation because of cardiac failure. Pathological study confirmed the Marfan habitus and complications. Molecular genetic study showed a de novo point mutation in exon 26 of the FBN1 gene. CONCLUSION: We report a case of prenatal Marfan syndrome diagnosed by sequential evaluation of the cardiac signs, which are essential for prenatal diagnosis. The prognosis seems as poor as the neonatal one. The prenatal diagnosis is essential for adequate counselling.

Adult↗
PubMed2006

Gastroschisis and omphalocele: retrospective study of initial postoperative management in the ICU.

AIM OF THE STUDY: The purpose of this study is to describe the management of infants with gastroschisis (G) and omphalocele (O) during the first 7 days after surgery. METHODS: A retrospective review of all cases of O or G managed at the ICU of the Robert Debré Teaching Hospital between January 1993 and July 2000 was carried out. PATIENTS: 29 infants with G, 15 with O (12 unruptured O [UO] and 3 ruptured O [RO]). RESULTS: Ventilatory support consisted of conventional mechanical ventilation (46 %) and/or in high-frequency oscillatory ventilation (61 %). After day 4, ventilatory requirements evaluated by mean airway pressure (MAP) differed significantly between G (n = 10/29) and O (n = 7/15; group vs. day of life, p = 0.04). The average of MAP measured on days 5, 6, and 7 was significantly higher in O than in G (14.7 +/- 3.0 versus 10.9 +/- 2.8, p < 0.01, respectively). Volume expansion was required at least once in 90 % of patients. Mean fluid requirements were significantly lower in UO than in G and in RO (41 +/- 31 ml/kg, 91 +/- 73 ml/kg, and 137 +/- 25 ml/kg, respectively; p = 0.02 for each comparison). Patients with G were significantly more likely to receive norepinephrine (59 % vs. 20 %, p = 0.027) than patients with O. Twenty-six infants with G (90 %) and 11 with O (73 %) were discharged alive from ICU. CONCLUSIONS: Haemodynamic instability can be expected in patients with G or RO, and ventilatory requirements were higher in infants with O than in infants with G during the first week after surgery.

Female↗
PubMed2006

Prenatal isolated mild ventriculomegaly: outcome in 167 cases.

OBJECTIVE: To define the contribution of prenatal investigation and evaluate the prognosis of isolated mild ventriculomegaly (IMV). DESIGN: Retrospective study. SETTING: University hospital between January 1992 and December 2002. POPULATION: One hundred and sixty-seven cases of prenatal unilateral or bilateral IMV without any associated anomaly at the time of initial diagnosis. METHODS: Complementary investigations were performed: amniocentesis with karyotyping, screening for viruses and acetylcholinesterase electrophoresis, magnetic resonance imaging (MRI), and ultrasonography every 3-4 weeks. MAIN OUTCOME MEASURES: Results of prenatal investigations, pregnancy outcome, and postnatal psychomotor development. RESULTS: IMV was diagnosed around 26.5 weeks. Amniocentesis revealed four chromosomal anomalies and two cytomegalovirus infections. MRI diagnosed brain-associated anomalies in 15 cases and ultrasonographic monitoring highlighted malformations not initially diagnosed in 28 cases. Termination of pregnancy (TOP) was considered in 21 pregnancies (12.6%). Indications were aneuploidy, fetal infectious disease or associated malformations. In women for whom a TOP was considered, consanguinity, fetus of female sex and frontal horn enlargement were statistically more frequent, ventriculomegaly was more often bilateral and asymmetrical, atrial width, and the rate of progressive ventricular enlargement were significantly higher. One hundred and one children with prenatal IMV were assessed between 19 and 127 months (mean age 54.68 +/- 2.87 months). Twelve children had neurological disease or psychomotor delay and 89 children had a normal psychomotor development. Poor neurological outcome was more often associated with atrial width greater than or equal to 12 mm, asymmetrical bilateral enlargement, and progression of the ventriculomegaly. CONCLUSION: The detection of IMV raises the question of the child's psychomotor development and justifies meticulous prenatal investigation. In addition to associated anomalies, three criteria are often associated with an unfavourable outcome: atrial width greater than 12 mm, progression of the enlargement, and asymmetrical and bilateral ventriculomegaly.

Adult↗
PubMed2005

Prenatal diagnosis of a cystic type IV sacrococcygeal teratoma mimicking a cloacal anomaly: contribution of MR.

OBJECTIVE: To report a case of pelvic midline cystic mass in a female fetus without visibility of the rectum and which is not a cloacal anomaly. METHODS: Ultrasound (US) and magnetic resonance imaging (MRI) were performed respectively at 27 and 27.5 weeks' gestation and the findings of these examinations were compared with post-mortem examination after termination of pregnancy (TOP). RESULTS: US showed a pelvic midline cystic mass in a female fetus with mild enlargement of the left ureter and renal pelvis. MRI did not show the T1 hypersignal of meconium in the rectum. The analysis of MR findings were suggestive of the diagnosis of a sacrococcygeal teratoma. Pregnancy was terminated because of the possibility of severe sphincterial disorders and the fetopathological examination confirmed this diagnosis. CONCLUSION: This observation illustrates the diagnostic problems generated by the detection of a pelvic midline cystic mass in a female fetus. The absence of visibility of the T1 hypersignal of the meconium in the rectum is traditionally supposed to be highly suggestive of a cloaca, but may also be explained by the emptiness of the rectum, compressed by the mass.

Adult↗
PubMed2005

Pial arteriovenous fistulas: dilemmas in prenatal diagnosis, counseling and postnatal treatment. Report of three cases.

We report three cases of the antenatal appearance of a pial arteriovenous fistula (AVF). In Case 1, the diagnosis of pial AVF was made by ultrasound examination at 32 + 3 weeks of gestation and confirmed by magnetic resonance imaging (MRI) at 34 + 3 weeks of gestation. After birth, the neonate had cardiac insufficiency and an embolization was performed 8 days postpartum which was followed by extended cerebral ischemia and death. In Cases 2 and 3 pial fistulae were diagnosed at birth. In Case 2, the patient was referred following the diagnosis of an aneurysmal malformation of the vein of Galen at 38 weeks of gestation. Owing to immediate delivery following her transfer, no ultrasound examination was performed and the diagnosis of pial AVF was established on the first postnatal day. An embolization was performed and the child is doing well. Case 3 presented with cardiomegaly at 30 weeks of gestation and cerebral ischemic lesions after birth. These cases highlight the difficulties encountered in the prenatal diagnosis of pial AVFs. The diagnosis should be considered in cases of unexplained cardiomegaly and it is also important to make sure that AVFs draining into the vein of Galen are not misdiagnosed as aneurysmal malformations of this vein. The complications of pial AVFs are cardiac failure and cerebral ischemia. Embolization is the treatment of choice; however it is a risky procedure in neonates.

Adult↗
PubMed2005

[Ductus venosus agenesis].

OBJECTIVE: Congenital absence of the ductus venosus is a rare anomaly in the fetus. The aim of our study was to evaluate the clinical and ultrasonographic features and outcome of the fetuses with ductus venosus agenesis. STUDY DESIGN: We describe 12 cases in the period between 1992 and 2004. The umbilical vein drained either into the right atrium directly (2 cases) or by the coronary sinus (1 case), or in the inferior vena cava (5 cases), or in the azygos vein (1 case), or in the portal vein (3 cases). Our data where analyzed with the cases published in the literature. Two groups of anastomoses where defined on the basis of the hemodynamic consequences: the group of extrahepatic anastomoses (53 cases) and the group of intrahepatic anastomoses (22 cases). RESULTS: In the group of extra hepatic anastomoses, cardiomegaly was the most common antenatal finding (39%), while in the intra hepatic group hydrops fetalis occurred most frequently (23%). Malformation rate was high in both groups (56% and 45%) and chromosomal anomalies where present in 9% of cases. CONCLUSION: Careful assessment of the umbilical venous return and the ductus venosus should be a part of examination of every fetus with cardiomegaly, polyhydramnios, ascites or hydrops. In case of absence of the ductus venosus a referral scan, a fetal echocardiography and a karyotype should be performed.

Autopsy↗
PubMed2004

Pulmonary sequestration children: is the thoracoscopic approach a good option?

BACKGROUND: The thoracoscopic approach for pulmonary sequestration in children seems to be feasible and reproducible. To date, there have been no studies comparing the thoracoscopic approach with the conventional technique in this indication. Therefore, the aim of this study was to compare these two approachs. METHODS: We performed a retrospective study in patients treated surgically for a prenatal diagnosis of pulmonary sequestration between 2000 and 2003. Three or five ports were used, and single-lung ventilation was required. The clinical, operative, and postoperative data were compared with those for patients operated on during the same period via thoracotomy. For statistical purposes, the children who required conversion to the open proecedure were grouped with those who underwent thoracotomy. The following data were compared: age, and weight, operating time, duration of postoperative drainage via chest tube, duration of postoperative narcotic use, pain scores on the 1(st) and 2(nd) days after the procedure, and length of hospital stay. The Mann-Whitney test was used for statistical analysis. RESULTS: Five infants were operated on via a thoracoscopic approach. Two required conversion to an open procedure. There were four intralobar sequestrations (ILS) and one extralobar sequestration (ELS). The mean age at surgery was 6.2 months (range, 4-9). The mean operating time, excluding the patients who were converted to open, was 155 min (range, 120-190). In the thoracotomy group, there were one ELS and one ILS. Two groups were defined: the exclusively laparoscopy group (EL) (n = 3) and the thoracotomy and conversion group (TC) (n = 4). The duration of postoperative drainage, duration of postoperative narcotic use, and hospital stay seemed to be shorter in the group EL. These times, respectively, were 3.4 vs 2.75 days, 4.1 vs 3.3 days, and 7.5 vs 4.3 days = (p = 0.03). There were two complications in the TC group (one wound abcess and one pleural leak that required a chest tube). CONCLUSION: The thoracoscopic approach for pulmonary sequestration is feasible. It seems to reduce the duration of postoperative drainage, postoperative narcotic use, hospital and stay. These results need to be confirmed by a prospective and more detailed study.

Bronchopulmonary Sequestration↗
PubMed2004

Impact of trisomy 21 on human trophoblast behaviour and hormonal function.

Although trisomy 21 (T21) is the most frequent genetic abnormality and some maternal serum markers for this fetoplacental aneuploidy are of placental origin, little is known of its impact on placental development. We therefore studied the influence of T21 on trophoblast behaviour. Using cultured cells from 46 human T21 pregnancies, we confirmed the defective morphological and functional differentiation of the villous cytotrophoblast in this setting; indeed, villous cytotrophoblast cells aggregate normally but fuse inefficiently to form the syncytiotrophoblast. This is in part related to the abnormal oxidative status of the T21 cytotrophoblast, characterized by a gene dosage-related increase in SOD-1 (copper-zinc superoxide dismutase) expression and activity. This was associated with a significant (P < 0.01) increase in catalase activity but no significant change in glutathione peroxidase activity. On the basis of these in vitro findings and studies of large panels of maternal serum, we propose a pathophysiological explanation for trisomy 21 maternal serum markers of placental origin.

Adult↗
PubMed2004

Trophoblast production of a weakly bioactive human chorionic gonadotropin in trisomy 21-affected pregnancy.

Total human chorionic gonadotropin (hCG) is high in maternal serum at 14-18 wk of trisomy 21 (T21)-affected pregnancy, despite low placental hCG synthesis. We sought an explanation for this paradox. We first observed that, in T21-affected pregnancies, maternal serum hCG levels peaked at around 10 wk and then followed the same pattern throughout pregnancy as in controls, albeit at a higher (2.2-fold) level. After delivery, hCG clearance was not significantly different from that in controls. We isolated cytotrophoblasts from 29 T21-affected placentas (12-25 wk) and 13 gestational age-matched control placentas and cultured them for 3 d. In this large series, we confirmed that, in the culture medium of trophoblasts isolated from T21 placentas, hCG secretion was significantly lower (P < 0.003) than in controls, in contrast to the high hCG in maternal serum of the same patients. In T21 cultured trophoblasts, transcripts of sialyltransferase-1 and fucosyltransferase-1 were abnormally high. In corresponding culture medium, hCG was abnormally glycosylated; highly acidic [isoelectric points (pHi) = 4.5] as shown by isoelectric focusing, immunoblotting, and lectin binding; and weakly bioactive (46% of control) as determined using the Leydig cell model. In conclusion, T21 trophoblast cells produced hCG that was weakly bioactive and abnormally glycosylated but whose maternal clearance was unaltered.

Antigens, CD↗
PubMed2003

Anti-Müllerian hormone levels in serum from human foetuses and children: pattern and clinical interest.

Serum anti-Müllerian hormone (AMH) determination has been used to investigate gonadal development and abnormal sexual differentiation, but until recently, it was based on assays developed by specialized laboratories. A short time ago, a sensitive assay kit was developed commercially (Immunotech-Beckman Coulter) for clinical use. With this method, we established usual levels of serum AMH in fetuses, newborns, and pre-pubertal children, and evaluated the clinical value of this assay. AMH measurement required only 25 microl of sample and could be performed within 3 h. In females, AMH emerged after birth at low levels (median: 4 ng/ml). In males, AMH levels remained stable during fetal life (median: 44.4 ng/ml), peaked in the first months of life to reach a median of 124.7 ng/ml, then fell with wide individual variations. Cord blood AMH levels at birth may be useful to investigate ambiguous genitalia suspected prenatally. In children with isolated microphallus or hypospadias, decreased AMH values are in favor of testis dysfunction. When testes cannot be palpated, a single determination of serum AMH levels can distinguish between anorchia and cryptorchidism.

Age Factors↗
PubMed2003

A comparison of the neonatal morbidity of second twins to that of a low-risk population.

OBJECTIVE: To assess the neonatal morbidity of second twins. STUDY DESIGN: Cohort study in a department of perinatalogy. The neonatal morbidity of second twins was compared to that of a low-risk population: singletons in the cephalic presentation delivered vaginally. RESULTS: Five hundred fifty-nine second twins and 18,061 vaginally delivered singletons in the cephalic presentation were studied. Of 452 (81%) second twins delivered vaginally, 310 (69%) were extracted using obstetrical maneuvers: internal version and breech extraction, breech extraction alone, or assisted breech delivery if the breech was already engaged. Before 33 weeks of gestation, there was no significant difference between the neonatal morbidity of the vaginally delivered second twins and the vaginally delivered singletons in the cephalic presentation. After 33 weeks of gestation, only the 1-min Apgar score <7 and the rate of intubation at birth were significantly higher in the second twins. Whatever the gestational age, there was no significant difference between the neonatal morbidity of the vaginally delivered second twins and that of the second twins born by cesarean section before labor. At comparable gestational ages, there was no significant difference between the death rate of the vaginally delivered second twins and that in the reference population. CONCLUSION: The neonatal morbidity of second twins was comparable to that of a low-risk population. Immediate management of the vaginally delivered second twins was, however, more intensive than that of vaginally delivered singletons in the cephalic presentation. It, therefore, requires appropriate equipment in a suitable obstetric-pediatric setting.

Birth Order↗
PubMed2003

Isolated pericardial effusion in the human fetus: a report of three cases.

OBJECTIVE: Our objective was to determine the possible underlying etiologies and outcome in isolated fetal pericardial effusion. METHODS: Doppler fetal echocardiography allowed the diagnosis of pericardial effusion in three patients and revealed the etiology in two. RESULTS: We present the findings in three cases of isolated pericardial effusion. In the first, the pericardial effusion was a manifestation of trisomy 21 associated with a myeloproliferative disorder. In the second, the pericardial fluid collection was the first sign of an autosomal recessive disease, idiopathic infantile arterial calcification. The third case was remarkable because of the spontaneous resolution of a large pericardial fluid collection. CONCLUSION: Isolated fetal pericardial effusion covers a wide spectrum of etiologies from severe genetic and chromosomal diseases to transient forms.

Adult↗
PubMed2003

Prenatal management of gastroschisis: the place of the amnioexchange procedure.

Gastroschisis is a malformation of the anterior abdominal wall that consists of a right paraumbilical defect with bowel loops bathed in the amniotic fluid. The survival rate is now greater than 90% and the prognosis relies mainly on morbidity attributable to bowel dysfunction. Recent research has examined gastrointestinal waste present in amniotic fluid that induces bowel toxicity and an inflammatory process. The amnioexchange procedure (changing the amniotic fluid regularly) involves a new therapeutic approach: reducing bowel injuries in the fetuses. This article shows that there is an inflammatory reaction in human gastroschisis and in the authors' model, and that the clinical and biological data plead for the practice of amnioexchange in human beings. A randomized, controlled study is now needed.

Amniotic Fluid↗
PubMed2003

Expression of pregnancy-associated plasma protein-A (PAPP-A) during human villous trophoblast differentiation in vitro.

Pregnancy-associated placental protein-A (PAPP-A), first isolated from maternal serum, has been identified as a metalloprotease cleaving insulin-like growth factor binding protein-4 (IGFBP-4). The source of PAPP-A during pregnancy is unclear. We therefore investigated PAPP-A expression during in vitro human villous cytotrophoblast cell (CT) differentiation into syncytiotrophoblast (ST). CT were isolated from normal first trimester, second trimester and term placentae (n=10) and cultured to form ST. PAPP-A mRNA was quantified by real-time PCR, and PAPP-A protein expression was studied by immunocytochemistry and TRACE technology with specific monoclonal antibodies. PAPP-A mRNA expression in total placental extracts increased during the course of pregnancy. PAPP-A protein was detected in the cytoplasm of both CT and ST. ST formation in vitro was associated with a 19-fold increase in PAPP-A mRNA expression and an 8-fold increase in PAPP-A secretion into the culture medium. No significant difference in PAPP-A production was observed between cultured cells isolated from early and term placentae. In conclusion, PAPP-A production in vitro, is associated to the differentiation of villous cytotrophoblast cells into syncytiotrophoblast, independently of the age of gestation.

Adult↗
PubMed2003

[Antenatal diagnosis of congenital left ventricular aneurysm].

Congenital aneurysm of the left ventricle is a rare condition of unknown origin, the main differential diagnosis of which is the diverticulum. The natural history of this pathology is well known in adults and adolescents, contrary to those forms diagnosed by foetal echocardiography. Based on a case of congenital left ventricular aneurysm diagnosed ante-natally and a review of the literature, the authors propose echocardiographic prognostic factors useful for prenatal management. Thus, early antenatal diagnosis, size and progression of the aneurysm, signs of antenatal cardiac failure, are poor prognostic factors and should be discussed during parent counselling.

Adult↗