Clinical validation of a prognostic tool in a population of outpatients treated for incurable cancer undergoing anticancer therapy: PRONOPALL study.

BACKGROUND: In 2008, a study of the characteristics of hospitalised patients led to the development of a prognostic tool that distinguished three populations with significantly different 2-month survival rates. The goal of our study aimed at validating prospectively this prognostic tool in outpatients treated for cancer in terminal stage, based on four factors: performance status (ECOG) (PS), number of metastatic sites, serum albumin and lactate dehydrogenase. PATIENTS AND METHODS: PRONOPALL is a multicentre study of current care. About 302 adult patients who met one or more of the following criteria: life expectancy under 6 months, performance status ≥ 2 and disease progression during the previous chemotherapy regimen were included across 16 institutions between October 2009 and October 2010. Afterwards, in order to validate the prognostic tool, the score was ciphered and correlated to patient survival. RESULTS: Totally 262 patients (87%) were evaluable (27 patients excluded and 13 unknown score). Median age was 66 years [37-88], and women accounted for 59%. ECOG PS 0-1 (46%), PS 2 (37%) and PS 3-4 (17%). The primary tumours were: breast (29%), colorectal (28%), lung (13%), pancreas (12%), ovary (11%) and other (8%). About 32% of patients presented one metastatic site, 35% had two and 31% had more than two. The median lactate dehydrogenase level was 398 IU/l [118-4314]; median serum albumin was 35 g/l [13-54]. According to the PRONOPALL prognostic tool, the 2-month survival rate was 92% and the median survival rate was 301 days [209-348] for the 130 patients in population C, 66% and 79 days [71-114] for the 111 patients in population B, and 24% and 35 days for [14-56] the 21 patients in population A. These three populations survival were statistically different (P <0.0001). CONCLUSION: PRONOPALL study confirms the three prognostic profiles defined by the combination of four factors. This PRONOPALL score is a useful decision-making tool in daily practice.

Scattering of slow metastable argon atoms by dielectric nanospheres.

The elastic scattering at low energy of metastable argon atoms with internal angular momentum J = 0 and 2 by dielectric nanospheres is investigated. The differential cross sections are calculated for both isotropic and anisotropic interactions. A polarization effect is clearly evidenced. The possible use of a metastable atom beam as a probe of an ensemble of nanospheres deposited on a passive substrate is examined.

Negative-index media for matter-wave optics.

We consider the extension of optical metamaterials to matter waves and then the down scaling of metaoptics to nanometric wavelengths. We show that the generic property of pulsed comoving magnetic fields allows us to fashion the wave-number dependence of the atomic phase shift. It can be used to produce a transient negative group velocity of an atomic wave packet, which results into a negative refraction of the matter wave. Application to slow metastable argon atoms Ar(3P2) shows that the device is able to operate either as an efficient beam splitter or an atomic metalens.

[Gastroesophageal reflux in infants: myths and realities]. / Reflux gastro-oesophagien du nourrisson : mythes et réalités.

Gastroesophageal reflux (GER) is a common problem in infants but the distinction between GER and GER disease remains difficult. Clinical manifestations such as vomiting, poor weight gain, respiratory disorders, and apneas do always not correlate with the demonstration of reflux episodes. Premature infants frequently suffer from reflux but correlations with apneas are also poor. Esophagitis is a complication suggested in infants experiencing pain but reflux by itself can induce pain as well. The "gold" diagnosis test is pH recording; however, overlap between normal and abnormal indices is obvious. Impedance measurement demonstrates more reflux episodes but non-acid reflux harm is not established. GER disease is probably self-limited in most infants, although it is impossible to predict whether some of them continue to have GER in adult life. The treatment raises doubts concerning indications and efficacy. Overprescription is frequent in infants with regurgitations. Nonpharmacological treatment - small-volume thickened milk and correct positioning - should be the first-line treatment. Prokinetic drugs have not proved their efficacy. Among anti-acid drugs, proton pump inhibitors are the best choice, but their indications are not very clearly established for infants. On the other hand, considerable variations of their metabolism due to the patients' age and genetic factors can explain variations in therapeutic effects.

Update on Anti-Saccharomyces cerevisiae antibodies, anti-nuclear associated anti-neutrophil antibodies and antibodies to exocrine pancreas detected by indirect immunofluorescence as biomarkers in chronic inflammatory bowel diseases: results of a multicenter study.

AIM: Anti-Saccharomyces cerevisiae antibodies (ASCA), anti-nuclear associated anti-neutrophil antibodies (NANA) and antibodies to exocrine pancreas (PAB), are serological tools for discriminating Crohn's disease (CrD) and ulcerative colitis (UC). Like CrD, coeliac disease (CoD) is an inflammatory bowel disease (IBD) associated with (auto) antibodies. Performing a multicenter study we primarily aimed to determine the performance of ASCA, NANA and PAB tests for IBD diagnosis in children and adults, and secondarily to evaluate the prevalence of these markers in CoD. METHODS: Sera of 109 patients with CrD, 78 with UC, 45 with CoD and 50 healthy blood donors were retrospectively included. ASCA, NANA and PAB were detected by indirect immunofluorescence (IIF). RESULTS: ASCA+/NANA- profile displayed a positive predictive value of 94.2% for CrD. Detection of ASCA was correlated with a more severe clinical profile of CrD and treatment of the disease did not influence their serum levels. ASCA positivity was found in 37.9% of active CoD. PAB were found in 36.7% CrD and 13.3% CoD patients and were not correlated with clinical features of CrD, except with an early onset of the disease. Fifteen CrD patients were ASCA negative and PAB positive. CONCLUSION: ASCA and PAB detected by IIF are specific markers for CrD although their presence does not rule out a possible active CoD. The combination of ASCA, NANA and PAB tests improves the sensitivity of immunological markers for CrD. Repeating ASCA, NANA, and PAB testing during the course of CrD has no clinical value.

[Apnea of prematurity: risk factors and ambulatory treatment with caffeine citrate]. / Apnées tardives du prématuré: facteurs de risque, traitement ambulatoire par citrate de caféine.

INTRODUCTION: Apnea of prematurity develop during the first days of life and usually resolve by the time the infant reaches 36-37 weeks postmenstrual age. In a few cases, they persist beyond term, especially in infants delivered at the youngest gestational ages (24-28 GA), and require specific care. In our unit, those preterm babies are discharged home with caffeine citrate treatment. Discontinuing the treatment is performed in hospital when they achieve a postmenstrual age of at least 42 weeks. OBJECTIVE: To identify predictive factors of persistent apnea in preterm babies. MATERIAL AND METHODS: Retrospective study comparing a population of 41 preterm infants discharged with treatment to 123 preterm babies discharged without treatment to identify predictors of persistent apnea. RESULTS: Factors significantly associated were: birth weight<1500 g, initial hypotension, gastroesophageal reflux, need for continuous positive airway pressure and multiparity. At home, no infant died and no adverse effect was reported by parents. CONCLUSION: Persistent apnea can be responsible for prolonged hospitalization. Risk factors can be identified in some children. Discharging with treatment can be an alternative to their hospitalization.

[Caffeine citrate utilization for treatment of apnea in French neonatal units]. / Utilisation du citrate de caféine pour le traitement des apnées idiopathiques du prématuré dans les équipes Françaises de médecine néonatale.

UNLABELLED: Caffeine citrate is commonly used for prophylaxis and treatment of apnea in preterm babies. OBJECTIVE: To evaluate the use of caffeine citrate in french neonatal units. MATERIALS AND METHODS: Postal survey in 100 neonatal units. RESULTS: Answers were obtained from 81 units. Sixty-three units use systematic prophylactic treatment and the threshold of gestationnal age (weeks gestation) for this systematic treatment is 32 weeks. Caffeine citrate is administered as a loading dose of 20 mg/kg followed by a maintenance dose of 5 mg/kg in 95% of the units. Discontinuing the treatment occurs between 33 and 35 weeks in 37% of the units and between 35 and 37 weeks in 53%. Two third of neonatologits describe recurrent apnea beyond 37 weeks, with the need to continue treatment. Fourteen units sometimes discharge babies at home with ambulatory caffeine citrate treatment and discontinue treatment by 42 to 46 weeks'gestation. A mean duration of 5 days without apnea is required before discharge. CONCLUSION: French teams respect "recommendations" concerning doses and duration without apnea before discharge. Indication of treatment, threshold for systematic treatment, duration of treatment and ambulatory treatment differ among teams.

[Analgesic effects of Emla cream and saccharose solution for subcutaneous injections in preterm newborns: a prospective study of 265 injections]. / Effet analgésique de la crème Emla, du saccharose et de leur association pour les injections sous-cutanées chez le nouveau-né prématuré: étude prospective de 265 injections.

OBJECTIVE: To compare the analgesic effects of non nutritive pacifier sucking, oral administration of a 30% saccharose solution, local application of Emla and their association for subcutaneous injection of erythropoietin (EPO) in preterm infants. METHODS: Our study was a randomised, prospective study conducted over 5 months. Neonates with a gestational age below 33 weeks of gestation and older than 8 days of life were included if they were treated with EPO (three subcutaneous injections per week during 6 weeks). For each consecutive EPO injection, patients were randomised between four groups of intervention: non nutritive pacifier sucking (T), oral administration of 0.2-0.5 ml of a 30% saccharose solution with non nutritive pacifier sucking (S), local application of Emla with non nutritive pacifier sucking (E), and oral administration of 0.2-0.5 ml of a 30% saccharose solution with local application of Emla and with non nutritive pacifier sucking (S + E). Each child was its own control. Pain was assessed with the Newborn Acute Pain scale (DAN) and with the Neonatal Facial Coding System (NFCS). RESULTS: Thirty-three neonates were included, representing 265 injections. Distribution was: 41 in group T, 71 in group E, 86 in group S and 67 in group E + S. Mean DAN and NFCS scores were statistically different between groups T, E and S. Analgesic effect of saccharose (-1.05) was greater than Emla (-0.56). Used together, effects were adding up without potentialisation. CONCLUSION: This study shows that the association of non nutritive pacifier sucking with oral administration of saccharose and local application of Emla has a better analgesic effect than each of these three interventions alone for subcutaneous injection of EPO.

[Congenital toxoplasmosis: a new case of infection during pregnancy in an previously immunized and immunocompetent woman]. / Toxoplasmose congénitale: un nouveau cas d'infection pendant la grossesse chez une femme antérieurement immunisée et immunocompétente.

UNLABELLED: Congenital toxoplasmosis is a potentially serious infection which usually affects infants born to non immune women. CASE REPORT: Our case report focuses on a baby born to a normally immunocompetent woman previously immunized against toxoplasmosis. To our knowledge only three similar cases have been published until now. CONCLUSION: We conclude that in front of a patient neonatal congenital infection picture, toxoplasmosis cannot be excluded on the ground of maternal immunity status and must be quickly investigated, given the emergency of appropriate treatment.

[Postnatal growth delay in 27 to 33 week premature infants: frequency and risk factors. Retrospective study of 161 cases]. / Retard de croissance postnatal du grand prématuré (27-33 SA): fréquence et facteurs de risque. Etude rétrospective à propos de 161 cas.

UNLABELLED: The optimization of the nutrition of very low birth weight premature neonates has become a major concern given the improvement in survival for these children. The goal of the recommended nutritional intakes is to reach a quantitative and qualitative growth similar to the in utero growth. The objectives of this study were to analyze the anthropometric data at birth and near term in a cohort of premature neonates with birth weight appropriate for gestational age and to try to determine risk factors of postnatal hypotrophy. POPULATION AND METHODS: We conducted a retrospective study over three years (1998-2001) in the neonatology unit of the Armand Trousseau Children's Hospital, Paris, France. The inclusion criteria was a gestational age under 33 weeks with birth weight appropriate for gestational age. Data were collected at admission, during hospitalisation and at discharge and a standardised form was filled for each child. We defined postnatal hypotrophy (PNH) as an hypotrophy at discharge (weight < 10(th) centile according to the Audipog reference curve) in neonates with birth weight appropriate for gestational age. RESULTS: One hundred and sixty one neonates were included. Eighty two had PNH. In univariate analysis, factors significantly associated with PNH were: birth weight, gestational age, length of hospitalisation, the occurrence of nosocomial infection, of enteropathy, preeclampsia, neonatal asphyxia and antenatal corticoid treatment. In multivariate analysis, risk factors of PNH were: low birth weight, low gestational age and the occurrence of nosocomial infection. CONCLUSION: Our study shows that half of the appropriate for gestational age premature neonates were hypotrophic near term. The causes may be various: nutrition is not optimal and intercurrent factors may play a major role such as nosocomial infection.

Atom symmetry break and metastable level coupling in rare gas atom-surface van der Waals interaction.

van der Waals interactions between an atom and a planar surface exhibit a quadrupolar component in D(2)(z)-D(2)/3 (D, atomic dipole; z, normal to surface). This coupling is responsible for an atom symmetry break, mixing levels of the same parity such as metastable 3P0, 3P2 levels of rare gas atoms. The strongly exoenergic 3P0-3P2 transition in Ar and Kr is observed by a time-of-flight technique, using as a surface the edge of a copper slit. The results confirm the predicted strong peaking of the angular distribution of inelastically scattered atoms and give a good estimate of the transition probability.

[Incidence of symptomatic celiac disease in French children]. / Incidence de la maladie coeliaque symptomatique de l'enfant en France.

OBJECTIVE: The study was carried out by the GFHGNP to determine the annual incidence of symptomatic celiac disease in children. PATIENTS AND METHODS: The diagnostic criteria were: symptomatic patients diagnosed under 15 years of age during 1996, villous atrophy and positivity of antigliadin and/or other antibodies. Cases were collected from referral centers, general hospital pediatric departments and private pediatricians with endoscopic practice. RESULTS: The study involved roughly half of the French pediatric population in 41 out of the 95 French districts. In all, 124 patients were collected: 76 girls and 48 boys. By geographical areas, in 30 districts where collection of data was complete which counted 186,285 births, the yearly incidence varied from 1/1731 births to 1/3110. (0.57@1000 to 0.32@1000). On the whole there were 77 cases i.e. an annual incidence of 1/2419 or 0.41@1000 (confidence interval 95%: 0.32 to 0.50@1000). Lower incidences were observed in the district of Paris: 1/4865 (0.21@1000) and Lyon: 1/3310 (0.27@1000). Those lower incidences could be explained by the difficulties of collecting the data in the biggest urban areas. The first signs occurred before one year of age in 73% of the cases, during the second year of life in 20.5% and after 3 in only 6.5%. The diagnosis was made before 2 years of age in 77% of the cases and after 3 in only 13%. In order of frequency symptoms were: failure to thrive (80%), diarrhea (59%), anorexia (59%), abdominal distension (57%), weight under 2 standard deviations (43%), short stature (43%). CONCLUSION: Compared with previous studies in two French districts between 1975 and 1990, the annual incidence of symptomatic celiac disease in children appears to be on the rise. The usual clinical signs continue to be observed.

[Early revealing of Williams-Beuren syndrome by digestive disorders]. / Révélation précoce par des troubles digestifs d'un syndrome de Williams-Beuren.

UNLABELLED: Williams-Beuren syndrome is a rare syndrome for which diagnosis is usually made during early childhood. It includes mental retardation, friendly outgoing personality, typical facies, supravalvular aortic stenosis and hypercalcemia. CASE REPORT: We report the case of a newborn whose gastroesophageal reflux led to the diagnosis of Williams-Beuren syndrome. Hypercalcemia is known to precipitate digestive symptoms but was not present in this case. CONCLUSION: Announcing such a diagnosis in the neonatal period is difficult and may destabilize the family, but at least allows early care of the cardiovascular pathologies that may lead to death.

Neurophysiological brainstem investigations in isolated Pierre Robin sequence.

Polysomnography, electromyography (EMG) of the face, tongue, and soft palate, blink reflexes (BRs), EMG during bottle-feeding, and brainstem auditory evoked responses (BAERs) were performed in 25 newborn babies with isolated Pierre Robin sequence (PRS) to aid in evaluation and management. Obstructive apneas were found in 23/24 patients (the 25th having undergone tracheotomy). Number and duration of central respiratory pauses were always normal, as well as electroencephalographic and clinical organization of sleep stages. EMG recruitment pattern in facial and lingual muscles, and BRs were normal in all cases. EMG recruitment pattern in muscles of the soft palate was normal in 14/25 patients, showed a reduced average amplitude with short-duration and low amplitude motor unit potentials in 10/25, and showed signs of denervation in 1/25. EMG during bottle-feeding showed sucking-swallowing disorders in 20/25 patients. BAERs showed a bilateral conductive impairment with increased latencies and thresholds in 5/19 patients, but with normal and symmetric I-III and I-V interpeak latencies in 19/19. These neurophysiological findings suggest that in isolated PRS a dysfunction of the lingual and pharyngeal motor organization exists without any structural impairment in brainstem nuclei and pathways.

Chronic intestinal pseudoobstruction syndrome: clinical analysis, outcome, and prognosis in 105 children. French-Speaking Group of Pediatric Gastroenterology.

Our aim was to collect a large number of cases to characterize clinical presentation, outcome, and prognosis of chronic intestinal pseusoobstruction in children. We conducted a retrospective multicenter study that included children treated for chronic intestinal pseusoobstruction defined as recurrent episodes of intestinal obstruction with no mechanical obstruction, excluding Hirschsprung's disease. In all, 105 children, 57 boys and 48 girls, were studied, including five familial forms. Prenatal diagnosis was made in 18 patients. Eighty patients were less than 12 months old at onset; the disease began at birth for 37 patients. The most frequent signs were abdominal distension, vomiting, and constipation. Megacystis was noted in myopathies (7 cases), neuropathies (10 cases) and unclassified forms (13 cases). For all but three cases (two patients with CMV infection, one with Munchhausen-by-proxy syndrome), the associated diseases and disorders could not account for chronic intestinal pseusoobstruction as a secondary disorder. At least one full-thickness biopsy from the digestive tract was studied for 99 patients. The diagnosis recorded was visceral neuropathy in 58 cases, visceral myopathy in 17 cases, and uncertain or normal biopsy results in 24 cases. Seventy-eight children were fed intravenously, and only 18 were able to be fed orally throughout their illness. Seventy-one patients underwent surgery during their illness, and 217 surgical procedures, a mean of 3 per patient, were performed. Ostomy was the most performed procedure. Follow-up continued in 89 patients for 3 months to 16 years (mean 85 months). Forty-two patients were still fed by parenteral (39 patients) or enteral nutrition (3 patients) at the time of the study. Eleven patients died between the age of 1 month and 14 years 7 months.

[Myocardiopathy and isolated glucocorticoid deficit with ACTH resistance: a fortuitous association?]. / Myocardiopathie et déficit isolé en glucocorticoïdes par résistance à l'ACTH: une association fortuite?

UNLABELLED: Hereditary syndrome of unresponsiveness to ACTH is a rare autosomal recessive disorder characterized by an isolated glucocorticoid deficiency which is exceptionally associated to regressive cardiomyopathy. CASE REPORT: A male newborn had iterative episodes of hypoglycemia since the first hours of life. Acute bronchiolitis at the age of 14 days was associated with transitory dilated cardiomyopathy. Hypoglycemia was due to glucocorticoid deficiency secondary to ACTH insensitivity. Molecular biology showed a composite heterozygotism for the ACTH receptor gene. CONCLUSION: Any congenital glucocorticoid deficiency should lead to search for cardiomyopathy.

[Allergy to cow's milk protein hydrolysates: apropos of 8 cases]. / L'allergie aux hydrolysats de protéines due lait de vache. A propos de huit cas.

BACKGROUND: The use of extensively hydrolyzed protein formulas is the best alternative for children with cow's milk allergy, though cases of allergies to hydrolyzed proteins have been reported. The aim of this study was to clarify from our experience the diagnostic, evolutive and therapeutic aspects of allergies to extensively hydrolyzed protein formulas. PATIENTS AND METHODS: We report eight cases of allergy to extensively hydrolyzed protein formulas seen between 1985 and 1998. The diagnostic criteria for allergy were either the appearance of immediate anaphylactic reactions after the ingestion of protein hydrolysate or a positive challenge test with the protein hydrolysate. RESULTS: Four children developed immediate anaphylactic symptoms after ingesting protein hydrolysate, and four children demonstrated subacute or chronic gastrointestinal symptoms. All children who developed acute anaphylactic symptoms had positive skin tests and specific IgF, antibodies (RAST) to cow's milk and/or hydrolyzed proteins. Conversely, in the four children with chronic gastrointestinal symptoms, skin tests and specific IgE antibodies were negative in three cases, but intestinal histology was abnormal in all of them when they were fed with a protein hydrolysate; this became normal after excluding the hydrolysate (data available in only two cases). Three children tolerated another protein hydrolysate form (whey vs. casein), four children had a favourable outcome when fed with human milk, and an amino-acid-based formula was successfully used in the most recent case. Nonhydrolyzed cow's milk proteins were tolerated after the age of 18 months in six children. Other atopic symptoms were observed in six children. CONCLUSION: Allergy to cow's milk protein hydrolysate is rare. The diagnosis is usually easy in children who develop acute anaphylactic symptoms, though intestinal histology is generally necessary for the diagnosis of allergy with chronic gastrointestinal symptoms. Treatment is based on the use of either another protein hydrolysate form (whey vs. casein) or an amino-acid-based formula.