Magnetic resonance imaging for abnormally invasive placenta: the added value of intravenous gadolinium injection.

OBJECTIVES: To assess the added value of intravenous gadolinium injection to magnetic resonance imaging (MRI) -based diagnosis of abnormally invasive placenta (AIP) and to examine this in relation to the radiologist's experience. DESIGN: Retrospective study. SETTING: Between March 2009 and October 2012, 31 pregnant women who had previous caesarean delivery together with a placenta praevia and suspected placenta accreta on ultrasound in the third trimester of pregnancy. POPULATION: All were offered MRI examination, and made aware of the limited (but so far reassuring) data regarding fetal safety of gadolinium. Twenty pregnant women agreed to undergo prenatal MRI (1.5 T), with and without gadolinium injection. METHODS: Two sets of MRI examinations without and with gadolinium were reviewed independently 2 months apart by two senior and two junior radiologists; all were blinded to the outcome (known in all cases). Histopathological findings and clinical signs of AIP were considered as the defining criteria of diagnosis. MAIN OUTCOME MEASURE: accuracy of MRI with and without gadolinium was assessed. RESULTS: Eight of the 20 women had confirmed abnormal placental invasion. The overall performance of both sets of readers in detecting AIP increased with gadolinium-sensitivity and specificity of 75.0% (42.0-100%) and 47.9% (19.9-75.9%) increasing to 87.5% (57.1-100%) and 60.4% (33.9-86.9%), respectively (P = 0.04). The added value of gadolinium remained irrespective of radiologist's experience, although senior radiologists performed better overall (sensitivity and specificity of 87.5% and 62.5% versus 62.5% and 33.3%, respectively, increasing with injection to 93.8% and 70.8% versus 81.3% and 50%, respectively; P < 10-4 ). CONCLUSION: There was an association between gadolinium use and improvement in MRI-based diagnostic accuracy for the diagnosis of AIP, for both junior and senior radiologists. TWEETABLE ABSTRACT: Gadolinium injection improves MRI performance of radiologists for the diagnosis of placenta accreta.

In Utero Diagnosis of Niemann-Pick Type C in the Absence of Family History.

Niemann-Pick type C (NPC) disease is a recessive disorder that results in unesterified cholesterol accumulating in the lysosomal and late endosomal system. It is caused by mutations in NPC1 or NPC2 genes and leads to systemic and neurodegenerative symptoms. Few cases of prenatal presentation of NPC have been reported and only two cases in the absence of previous family history, indicating the diagnosis is particularly difficult in such a situation. We report a prenatal diagnosis of NPC in a couple without family history. An ultrasound screening at 22 weeks of gestation (WG) detected fetal ascites and hepatomegaly, which were still present at 25, 27, and 29 WG, and a splenomegaly progressively appeared. No placentomegaly or other signs of hydrops fetalis were observed. The diagnostic of NPC was prenatally confirmed by a filipin test and NPC1 sequencing and multiplex ligation-dependent probe amplification assay which revealed a maternal missense mutation (c.2608T>C; p.Ser870Pro) and a paternal deletion of exons 5 to 25. This additional prenatal case of NPC suggests that even in the absence of family history, fetal ascites associated with splenomegaly but no hydrops should nonetheless arouse suspicion concerning this disease as a possible diagnosis.

Genetic associations with viral respiratory illnesses and asthma control in children.

BACKGROUND: Viral respiratory infections can cause acute wheezing illnesses in children and exacerbations of asthma. OBJECTIVE: We sought to identify variation in genes with known antiviral and pro-inflammatory functions to identify specific associations with more severe viral respiratory illnesses and the risk of virus-induced exacerbations during the peak fall season. METHODS: The associations between genetic variation at 326 SNPs in 63 candidate genes and 10 phenotypes related to viral respiratory infection and asthma control were examined in 226 children enrolled in the RhinoGen study. Replication of asthma control phenotypes was performed in 2128 children in the Copenhagen Prospective Study on Asthma in Childhood (COPSAC). Significant associations in RhinoGen were further validated using virus-induced wheezing illness and asthma phenotypes in an independent sample of 122 children enrolled in the Childhood Origins of Asthma (COAST) birth cohort study. RESULTS: A significant excess of P values smaller than 0.05 was observed in the analysis of the 10 RhinoGen phenotypes. Polymorphisms in 12 genes were significantly associated with variation in the four phenotypes showing a significant enrichment of small P values. Six of those genes (STAT4, JAK2, MX1, VDR, DDX58, and EIF2AK2) also showed significant associations with asthma exacerbations in the COPSAC study or with asthma or virus-induced wheezing phenotypes in the COAST study. CONCLUSIONS: We identified genetic factors contributing to individual differences in childhood viral respiratory illnesses and virus-induced exacerbations of asthma. Defining mechanisms of these associations may provide insight into the pathogenesis of viral respiratory infections and virus-induced exacerbations of asthma.

Rapid measurement of the yield stress of anaerobically-digested solid waste using slump tests.

The anaerobic digestion of solid waste is usually performed using dry or semi-dry technology. Incoming waste and fermenting digestate are pasty media and thus, at the industrial scale, their suitability for pumping and mixing is a prerequisite at the industrial scale. However, their rheology has been poorly characterised in the literature because there is no suitable experimental system for analysing heterogeneous media composed of coarse particles. We have developed a practical rheometrical test, a "slump test", for the analysis of actual digested solid waste. It makes it possible to estimate yield stress from the final slump height. From the slump behavior, we conclude that digestates behave as visco-elastic materials. The yield stress of different digested waste was measured between 200 and 800Pa. We show that the media containing smaller particles or with higher moisture content are characterised by smaller yield stresses. This study thus demonstrates the impact of the origin of the digestate on the yield stress.

[MRI aspects of anoxic-ischemic encephalopathy of the term and premature newborn]. / Aspects IRM de l'encéphalopathie anoxo-ischémique du nouveau-né à terme et du prémature.

These past few years have seen an increasing role of MRI in the investigation of neonatal cerebral anoxic-ischemic pathology. This is due not only to greater precision in diagnosing lesion extension, but also to earlier detection of lesions with the diffusion of weighted imagery. The aim of this iconographic review is to illustrate the main MRI aspects of anoxic-ischemic encephalopathy in a pedagogical way. After a brief physiopathology reminder, the different cerebral lesions are studied in a first chapter on the premature newborn pathology and a second chapter on full-term newborn pathology.

Towards new indicators for the prediction of solid waste anaerobic digestion properties.

The biochemical composition can be seen as a good indicator of both the biodegradability and the methane potential of a given waste. The work presented here is an attempt to elaborate a typology of wastes and to compare it to the anaerobic degradation characteristics. The first data indicate that there is a link between the ligno-cellulosic content of the waste and the biodegradability. When dealing with application to anaerobic digestion processes, having a tool to predict the ability of the waste to be degraded could be of the greatest interest for preventing failures, estimating biogas production, methane content, or for the management of co-digestion processes.

Prenatal diagnosis of absence of the septum pellucidum associated with septo-optic dysplasia.

Septo-optic dysplasia (SOD; De Morsier syndrome) is a rare congenital disorder characterized by the absence of the septum pellucidum (SP), hypoplasia of the optic chiasma and nerves, and various types of hypothalamic-pituitary dysfunction. We report on two fetuses with absence of the SP diagnosed by ultrasound examination at 29 and 30 gestational weeks. In the first case the diagnosis of SOD was suspected in utero and confirmed postnatally; to the best of our knowledge this is the first report of the prenatal diagnosis of SOD. In the second case absence of the SP appeared to be isolated and no visual or endocrine impairment were detected after birth.

Growth and adult height in GH-treated children with nonacquired GH deficiency and idiopathic short stature: the influence of pituitary magnetic resonance imaging findings.

We analyzed the final height of 146 short children with either nonacquired GH deficiency or idiopathic short stature. Our purpose was 1) to assess growth according to the pituitary magnetic resonance imaging findings in the 63 GH-treated children with GH deficiency and 2) to compare the growth of the GH-deficient patients with normal magnetic resonance imaging (n = 48) to that of 32 treated and 51 untreated children with idiopathic short stature (GH peak to provocative tests >10 microg/liter). The mean GH dose was 0.44 IU/kg.wk (0.15 mg/kg.wk), given for a mean duration of 4.6 yr. Among the GH-deficient children, 15 had hypothalamic-pituitary abnormalities (stalk agenesis), all with total GH deficiency (GH peak <5 microg/liter). They were significantly shorter and younger at the time of diagnosis than those with normal magnetic resonance imaging, had better catch-up growth (+2.7 +/- 0.9 vs. +1.3 +/- 0.8 SD score; P < 0.01), and reached greater final height (-1.1 +/- 1.0 vs. -1.7 +/- 1.0 SD score; P < 0.05). Among patients with normal magnetic resonance imaging, there was no difference in catch-up growth and final height between partial and total GH deficiencies. GH-deficient subjects with normal magnetic resonance imaging and treated and untreated patients with idiopathic short stature had comparable auxological characteristics, age at evaluation, and target height. Although they had different catch-up growth (+1.3 +/- 0.8, +0.9 +/- 0.6, and +0.7 +/- 0.9 SD score, respectively; P < 0.01, by ANOVA), these patients reached a similar final height (-1.7 +/- 1.0, -2.1 +/- 0.8, and -2.1 +/- 1.0 SD score, respectively; P = 0.13). Pituitary magnetic resonance imaging findings show the heterogeneity within the group of nonacquired GH deficiency and help to predict the response to GH treatment in these patients. The similarities in growth between the GH-deficient children with normal magnetic resonance imaging and those with idiopathic short stature suggest that the short stature in the former subjects is at least partly due to factors other than GH deficiency.

Nf1;Trp53 mutant mice develop glioblastoma with evidence of strain-specific effects.

Astrocytomas are the leading cause of brain cancer in humans. Because these tumours are highly infiltrative, current treatments that rely on targeting the tumour mass are often ineffective. A mouse model for astrocytoma would be a powerful tool for dissecting tumour progression and testing therapeutics. Mouse models of astrocytoma have been designed to express oncogenic proteins in astrocytes, but have had limited success due to low tumour penetrance or limited tumour progression. We present here a mouse model of astrocytomas involving mutation of two tumour-suppressor genes, Nf1 and Trp53. Humans with mutations in NF1 develop neurofibromatosis type I (NF1) and have increased risk of optic gliomas, astrocytomas and glioblastomas. The TP53 tumour suppressor is often mutated in a subset of astrocytomas that develop at a young age and progress slowly to glioblastoma (termed secondary glioblastomas, in contrast to primary glioblastomas that develop rapidly de novo). This mouse model shows a range of astrocytoma stages, from low-grade astrocytoma to glioblastoma multiforme, and may accurately model human secondary glioblastoma involving TP53 loss. This is the first reported mouse model of astrocytoma initiated by loss of tumour suppressors, rather than overexpression of transgenic oncogenes.

[Preoperative evaluation of choledochal cyst with MR cholangiopancreatography]. / Bilan préthérapeutique d'une dilatation congénitale de la voie biliaire principale par cholangio-pancréatographie-IRM.

UNLABELLED: The choledochal cyst is a rare congenital disorder usually diagnosed in childhood. It requires a complete surgical resection to prevent complications, particularly the risk of malignant changes. At present, the preoperative examination requires a direct opacification of the biliary tree, but this is an invasive technique with a high risk of infection, especially in pediatric patients. CASE REPORT: A choledochal cyst was diagnosed in a five-year-old girl with recurrent abdominal pain. Diagnosis was made by ultrasound and preoperative evaluation by magnetic resonance-cholangiopancreatography using single-shot fast-spin echo sequences. A complete correlation was observed between surgical, preoperative cholangiography and MRCP data. CONCLUSION: Recent improvement in MRCP techniques provide a complete anatomic analysis of choledochal cysts, enabling one to diagnose an anomalous junction of the pancreaticobiliary duct, even the presence of stones within the biliary tree. This short and noninvasive examination should in the future replace direct opacification of the biliary tree for the preoperative assessment of choledochal cysts.

Ultrasonographic diagnosis of hepatic fibrosis or cirrhosis.

BACKGROUND/AIMS: Evaluation of the degree of hepatic fibrosis is especially important in patients with chronic liver disease. Our aim was to study the diagnostic accuracy of abdominal ultrasonography for cirrhosis or fibrosis. METHODS: Twenty-three clinical (n=12) and Doppler ultrasonic (n=11) variables were recorded in 243 patients with chronic (alcoholic and viral) liver disease under conditions close to those of clinical practice. Fibrosis was classified into six grades by two pathologists. Diagnostic accuracy was evaluated by discriminant analysis, first globally using all variables, then by stepwise analysis. RESULTS: A) Diagnosis of cirrhosis: 1) whole group (n=243): diagnostic accuracy was globally 84%, and 84% with two variables: spleen length, portal velocity; 2) compensated chronic liver disease (n=191): diagnostic accuracy was globally 85%, and 82% with two variables: liver surface, liver length (right kidney); 3) alcoholic compensated chronic liver disease (n=109): diagnostic accuracy was globally 86%, and 88% with two variables: spleen length, liver length (middle clavicle); 4) viral compensated chronic liver disease (n= 83): diagnostic accuracy was globally 86% and 86% with one variable: liver surface. By subtracting the proportion of patients who could not be investigated due to anatomical limitations, the highest calculated univariate diagnostic accuracy decreased by 7%. B) Diagnosis of fibrosis: diagnostic accuracy was globally 84% for extensive fibrosis. CONCLUSIONS: Cirrhosis can be correctly diagnosed in 82-88% of patients with chronic liver disease using a few ultrasonographic signs. However, the diagnostic accuracy of ultrasound is decreased by the anatomical limitations of this technique.

[Is ultrasound screening for ovarian cancer possible in 1998?]. / Le dépistage échographique du cancer de l'ovaire est-il possible en 1998?

Ovarian cancer is the most common cause of gynecologic cancer death, as most patients present with advanced disease, in which the prognosis is poor. Five year-survival is only 35% for all stages, while it exceeds 90% in stage I. Consequently, there has been heightened interest in the development of screening modalities that can detect ovarian cancer at an early stage to reduce the mortality of this disease. Unfortunately, transvaginal sonography and color Doppler imaging still have a high false positive rate and low specificity increasing the number of surgical procedures, even among women with a strong family history of ovarian cancer. Psychological impact and economical cost has also to be discussed when considering such programs.

Intravenous access in newborn infants: impact of extended umbilical venous catheter use on requirement for peripheral venous lines.

Central venous lines are used to care for critically ill neonates in cases of limited peripheral venous access. This prospective, randomized study evaluated the risks and benefits of the use of single- and double-lumen umbilical venous catheters for up to 14 days. Patients were randomized to one of three treatment arms: (1) single-lumen umbilical catheter, (2) double-lumen umbilical catheter, or (3) no umbilical catheter; peripheral intravenous lines only. Infants in the groups treated with an umbilical venous catheter had significantly fewer venipunctures and peripheral intravenous lines placed during their first 2 weeks of life than those in the peripheral line only group. Less time and money were spent obtaining peripheral line placement in the umbilical venous catheter groups. The incidence rates of sepsis and complications were not higher in treated patients than in control patients. The double-lumen catheter further reduced peripheral venipunctures and lines. We conclude that an umbilical venous catheter used during the first 2 weeks of life is a relatively safe, less stressful, cost-effective means of providing intravenous therapy to neonates.

[Tracheal compression by mediastinal mass in children. Value of fiberoptic tracheobronchoscopy for anesthetic management]. / Compression trachéale par une masse médiastinale chez l'enfant. Intérêt de la fibroscopie trachéobronchique pour la prise en charge anesthésique.

Due to the action of the drugs and the position of the patient, general anaesthesia in a child with a mediastinal tumor can complete the obstruction of the trachea and/or the main bronchi and possibly result in irreversible respiratory failure. The risk is directly linked to the degree of lumen amputation of the trachea, usually evaluated by CT-scan: there is a risk of accident if the degree of lumen amputation is greater than 30%. The case reports underline the value of the flexible fibreoptic bronchoscopy under local anaesthesia. This examination, as well as the CT-scan, allows to evaluate the degree of tracheal compression. Moreover, it carries a lower risk than the CT-scan which requires, in a small child, either general anaesthesia or heavy premedication which could lead to asphyxia. Total obstruction most often occurs with anterior mediastinal tumors. However, even posterior or lateral tumors can extend to an anterior position, especially in the young child. If the obstruction of the lumen is greater than 30% and if general anaesthesia is required, the fibreoptic bronchoscopy allows endotracheal intubation under local anaesthesia before general anaesthesia. We report 5 cases which illustrate the risk of tracheal compression during general anaesthesia in patients with a mediastinal tumor and substantiate the advantages of a flexible fibreoptic bronchoscope in the management of these patients before and during general anaesthesia.

Intracranial arteriovenous malformation in a neonate: aneurysm of the Great Vein of Galen.

Intracranial AVM is a complex medical emergency in newborn infants. The aim of therapy is to decrease blood flow through the AVM and control cardiac failure. Embolization and surgery offer amelioration of symptoms but may not ensure a favorable prognosis because of damage caused by the AVM itself and/or complications of treatment.