Three-dimensional imaging of active acid sulfate soil using a DUALEM-21S and EM inversion software.

One of the major environmental issues in Finland is the presence of large tracts of acid sulfate soil (ASS) landscapes along the coast. Accurately identifying the distribution of ASS sediments, and in particular soil pH, is essential for developing targeted management strategies. One approach is the use of digital soil mapping (DSM) with various ancillary information. Although electromagnetic (EM) induction data has shown potential in mapping ASS, few studies have been conducted to map the spatial distribution of pH at different depths. In this study, a DUALEM-21S was used to collect apparent soil electrical conductivity (ECa) data across a 23-ha field near Vaasa, which lies along the western coast of Finland. A quasi-3D inversion algorithm was used to calculate the estimated true electrical conductivity (σ - mS m-1). A calibration relationship was developed between σ and incubation-pH measured at various depths from topsoil (0-0.2 m), subsurface (0.2-0.4 m) and subsoil (e.g. 0.4-0.6 and 1.8-2 m) using an artificial neural network (ANN) model. The performance of the ANN model was good given the large R2 values for calibration (0.72) and validation (0.65). It was concluded that the combination of ECa data and quasi-3D inversion algorithm (in EM4Soil) was able to map the spatial distribution of incubation-pH associated within an ASS landscape. The approach has the potential to be applied across the coastal areas of Finland and elsewhere to map incubation-pH and identify active-ASS areas and thereby improve the management of these areas.

[Monitoring network for vulnerable children in the Pays de la Loire ("Grandir ensemble" - Cohort LIFT): 10 years of activity 2003-2013]. / Réseau de suivi des enfants vulnérables dans les Pays de la Loire (Grandir Ensemble - Cohorte LIFT) : dix ans d'activité de 2003 à 2013.

INTRODUCTION: Vulnerable children are at-risk newborns including premature infants and some children with pathologies presented by fear anomalies and deficiencies, most particularly neurological. Monitoring is based on the detection of these abnormalities and their early management. The organization of this monitoring system is based on a network of doctors, mostly pediatricians, trained regularly. The objective of this review was to assess the resources, means, and results of 10 years of follow-up. METHODS: The Pays de la Loire network includes 24 maternity wards and 13 neonatal departments. Annual admissions are around 5000 newborns to approximately 45,000 annual births. Upon discharge of newborns, born prematurely at 34 weeks of gestation (WG) or less, or term infants with neurological problems, parents are asked to have their child monitored by a referring doctor. During the consultation, a reference document is filled out by the doctor and sent to the project manager for data collection and specific compensation for private practitioners. Standardized questionnaires were used such as the ASQ (Ages and Stage Questionnaire) completed by parents, the developmental quotient (DQ) with the Lézine Brunet-Revised test (BLR), the intelligence quotient (IQ) with the Wechsler Preschool and Primary Scale of Intelligence (WIPPSI III) completed by psychologists employed in the network, and a questionnaire completed by the teacher at 5 years of age. RESULTS: The network started on 1st March 2003, and 28th February 2013, after 10 years of inclusion, 10,800 children had been included. This population accounts for 2.4% of all annual births: 1.1% were included for prematurity less than 33 weeks and 0.25% were term-born infants. The characteristics of children are presented with gestational age, birth weight, and obstetric and neonatal pathologies. The percentage of these children followed was 80% at 2 years and 63% at 5 years. At 2 years, the results are presented according to gestational age with approximately 60% of children without disabilities at 25-26 WG, 73% at 27-28 WG, 77% at 29-30 WG, and 86% at 31-32 WG. Absorptions are diverse and vary according to the age of the child with physical therapy, psychomotor skill work, speech therapy, hearing and vision consultations, and psychology/psychiatry. Assessment tools were refined by specific analyses: the ASQ 24 months (completed by parents) was deemed valid and predictive with respect to IQ (abandoned in 2012), and the grid completed by the teacher was found to predict abnormalities in 5 years. CONCLUSION: The Pays de la Loire monitoring network has met its initial objective, namely to detect disabilities early and provide practical help to parents in a population of vulnerable children. Benefits for professionals and other children not followed in the network were observed, with an increase in pediatricians' skills. The benefits of the evaluation results are more difficult to assess with the care than neonatal care in obstetrics. The sustainability of such a network seems assured for healthcare professionals, provided that funding is maintained by the health authorities.

[Ocular syphilis. A report of two patients]. / Syphilis oculaire : à propos de deux cas.

INTRODUCTION: Whereas syphilis is a classical cause of uveitis, it is still often under-recognized. Treatment recommendations are not based on specific clinical studies. CASE REPORTS: We report two patients with syphilitic uveitis diagnosed and treated at the University Hospital of Angers. Ocular involvement was inaugural in both patients. Each had a specific treatment but none had a complete recovery of visual function. CONCLUSION: Syphilis must be discussed in all patients diagnosed with uveitis or papillitis. Although not evidence-based, prompt therapy may lead to functional recovery.

Systemic and immune manifestations in myelodysplasia: a multicenter retrospective study.

OBJECTIVE: The presence of systemic and/or immune manifestations in myelodysplasia has been currently reported. The influence of these manifestations on the natural outcome of myelodysplastic syndrome has to be considered. We present a multicenter retrospective study (2002-2009) of patients with myelodysplastic syndrome disclosing systemic and/or immune manifestations. METHODS: Forty-six patients with myelodysplasia presenting with systemic and/or immune manifestations were compared in terms of survival with 189 patients with myelodysplasia lacking these features. RESULTS: The clinical picture in these cases consisted of fever (13%), arthralgia or arthritis (13%), and cutaneous manifestations (67%). Four cases of systemic vasculitis have been reported in our series, and they have a worse prognosis. Immune anomalies were recorded in 29% of the cases, and the presence of cryoglobulins was also associated with a worse prognosis. CONCLUSION: A difference in survival between patients with myelodysplastic syndrome with systemic manifestations and patients lacking these manifestations has been observed in the presence of systemic vasculitis and/or cryoglobulins.

Anti-Ku antibodies: Clinical, genetic and diagnostic insights.

Anti-Ku antibodies are reported in various connective tissue diseases and the Ku complex can be responsible for a very strong autoimmune answer in autoimmune disease. Nowadays, anti-Ku antibodies are detected by ELISA, counterimmunoelectrophoresis (CIE), immunoblot (IB) and new highly performant techniques. Although the prevalence of anti-Ku antibodies is not homogenous, depending on several features such as disease type, genetic and geographical clustering, and also method of detection, they could be found in 55% overlap PM/systemic sclerosis patients. Moreover, anti-Ku antibodies are not associated with a particular clinical outcome, and especially with cancer related to myositis.

[Parent satisfaction with the Loire Infant Follow-up Team (LIFT) premature and at-risk infant network in the Pays-de-la-Loire area (France)]. / Satisfaction des parents dans le réseau de suivi des prématurés et des enfants à risques « Grandir ensemble ¼ des Pays-de-la-Loire.

BACKGROUND: The Loire Infant Follow-up Team (LIFT) is a network for caring for premature infants whose gestational age is 34 WA or less and at-risk neonates in the Pays-de-la-Loire area in France. The network aims to screen for clinical anomalies early and to propose adapted care. Trained physicians follow the included children in a standardized manner at 3, 6, 9, 12, and 18 months and 2 years, with a specific examination by psychologists at 2 years. The aim of the study was to assess the satisfaction of the parents of the children followed. METHODS: To evaluate parent satisfaction, a questionnaire from the Consumer Satisfaction Survey (CSS) in its French version was sent to parents whose infants were 2 years old, stratifying on the presence of an anomaly. The questioner had 39 items, with 8 specific items on the network and 31 from the CSS. The questionnaire was mailed twice in September 2006. RESULTS: Out of 300 questionnaires mailed, 269 were returned (rate 89.7 %). The questionnaire was assessed using principal component analysis with 2 dimensions for the 30 items common to all children, one of which covered empathy with physicians and the other with the consulting psychologists at 2 years. The validity was good (Cronbach coefficient, 0.91). The answers to overall questions such as "We are satisfied with the care in the network" scored 16.1±0.7/20, with 90 % "totally agree" or "moderately agree" responses. The "The care is perfect" scored 14.6±0.7/20 with 78 % agreeing with the statement. The total score for 30 general questions was 14.6±3.1 (median, 14.9). The total score was lower for infants with anomalies: 13.7±3.3 versus 14.9±2.9 (P<0.01). The answers with a low score (<10) were given by 22 parents (8.2 %). There was no significant relation between the total score or the satisfaction score and neonatal events. CONCLUSION: A postal survey is helpful to know the views of parents on the follow-up of their infants. This good level of satisfaction seems to stem from the parents feeling they belong to the network, the quality of the relationships with personnel, and the doctors' empathy, as well as the number of contacts between parents and the network coordinator.

[Type B Niemann Pick disease: clinical description of three patients in a same family]. / Maladie de Niemann-Pick de type B: description clinique de trois cas familiaux.

INTRODUCTION: The Niemann Pick disease type B is a rare deficiency in sphingomyelinase activity, autosomal recessively inherited. CASE REPORTS: We report three patients (two men, one woman) of the same family, who showed pulmonary and hepatosplenic lesions, usually present in the disease but also adrenal gland lesions confirmed by tomodensitometry. CONCLUSION: The current treatment of Niemann Pick disease is purely symptomatic awaiting the use of enzymatic replacement therapy which has been successfully experimented in animal model.

[Tuberculosis with a hemophagocytic syndrome]. / Tuberculose compliquée d'un syndrome hémophagocytaire ou syndrome d'activation macrophagique.

INTRODUCTION: The hemophagocytic syndrome is rare and sometimes associated with tuberculosis. OBSERVATION: We report the case of a 30-year-old migrant from Congo presenting with a recurrent right pleuropneumonia, cachexia and night sweat. Blood tests revealed bicytopenia with a normal myelogram. Thorax CT-scan showed large mediastinal lymph nodes and pleuritis. Mediastinal lymph node biopsy concluded to granulomatosis lymphadenopathy with necrosis and bone marrow biopsy suggested hemophagocytic syndrome. The outcome was favorable with antibiotics and corticosteroids.

[Scarlet fever with multisystem organ failure and hypertrophic gastritis]. / Scarlatine compliquée d'une défaillance multiviscérale avec gastrite hypertrophique.

Scarlet fever is a rare disease in adult patients. We report a patient in whom scarlet fever was associated with hypertrophic gastritis and multiple organ failure. A 62-year-old woman presented with septic shock and multiple organ failure. Bacteriological survey was negative. Abdominal tomodensitometry showed an hypertrophic gastritis. Histological analysis demonstrated a non specific gastritis without any tumoral sign. Cefotaxime and amoxicillin led to improvement and hypertrophic gastritis progressively resolved. A sandpaper rash over the body with finger desquamation, elevation of antistreptolysin O and a recent contact with an infected grandson led to the diagnosis of scarlet fever. Due to antibiotic prescription, scarlet fever is now uncommon. Although classical, ENT or gastroenteritis presentations may be puzzling for the diagnosis of scarlet fever. As 150 years ago, diagnosis of scarlet fever is still a clinical challenge.

[Follow-up network for newborns at risk for handicap in a French region]. / Réseau de suivi des nouveau-nés à risque de développer un handicap. L'expérience du réseau de suivi régional << grandir ensemble en Pays de la Loire >>.

BACKGROUND AND OBJECTIVES: - Follow-up networks for newborns with a handicap risk need to be put into place downstream of the perinatal health networks. Such a network was implemented in the Pays de la Loire region in 2003. Our objective is to evaluate the feasibility and the capacity to detect infants with an incapacitating condition at nine months corrected age and the patents'satisfaction with such a network. MATERIAL AND METHODS: - A common tool based on the Amiel Tison assessment was set up. Infants included since 1(st) March 2003 and who were two years old corrected age on 1(st) September 2006 were taken into consideration. A satisfaction survey was conducted with the parents of infants showing normal development at two years old corrected age or with an abnormal neuromotor examination. RESULTS: - Amongst the 1339 infants included, 1185 (88.4 %) were seen at the age of two years: 7.3 % showed pathological development, 4.5 % infants showed suspect neuromotor examination. Cares were proposed for 11% of the infants followed, permitting cares from nine months old for 65 % of the infants considered as having a pathological development at the age of two years. DISCUSSION/CONCLUSION: - This follow-up network has fulfilled its initial goal: 2.2 % of newborn babies in the region were included, a low rate of lost for follow-up was observed. Moreover, this network has a relative good capacity for detection and early initiation of care. The application of the parents is important and their level of satisfaction appeared to be high. This experiment shows that regional follow-up networks can become reality, but their efficiency still needs to be improved.

[Follow-up network for newborns with a handicap risk: experience of the Pays-de-la-Loire network]. / Réseau de suivi des nouveau-nés à risque de développer un handicap: l'exemple du réseau Grandir ensemble en Pays-de-la-Loire

Newborns are given attentive perinatal care but the organization of further follow-up can be haphazard. The main consequences are the difficulties parents have in finding appropriate medical assistance for caring for high-risk infants and the absence of appropriate surveillance or efficient care. An inpatient-outpatient healthcare network enables early care of these infants and can reduce the consequences of neurosensorial sequelae. The overall impact of the regional perinatal care can also be evaluated. Such a network has been implemented in the Pays de Loire region in France since early 2003. In six months, among 1000 initially included infants, 500 were followed by pediatricians working in an outpatient (40%) or inpatient (60%) setting. This organization enables correction of over-centralization of neonatal care and the absence of coordination for follow up.

[The role of cytomegalovirus in infantile deafness and deafness of unknown origin]. / Le Rôle du cytomégalovirus dans la surdité du nourrisson et sa place dans les surdités d'étiologie méconnue.

It is widely accepted that the cause of congenital deafness is genetic in one third of cases roughly, is due to acquired affections during pregnancy or delivery in another third and remains unknown in the last third. It is possible that the cytomegalovirus (CMV) plays an important role in the latter group. The CMV is thought to be involved in 10 to 30% of cases of auditory sequelae from fetal infection, either severe neonatal CMV-induced disease, which is rare, or the frequent subclinical infections affecting an average of 1% of newborn infants. The only certain way to determine the importance of the role of CMV in deafness of unknown etiology is large-scale neonatal biologic screening followed by long-term audiologic surveillance: currently available documented data suggest that this role is very important.

[Branchio-oto-renal malformation syndrome (author's transl)]. / Le syndrome malformatif branchio-oto-rénal. A propos de 5 observations.

After a brief review of the dysgenetic abnormalities which may affect the ear, branchial arches and pouches and the kidneys, the authors summarise those syndromes associating abnormalities of the ear and kidney on the one hand, and secondly abnormalities of the ear and facial and cervical fistulae. However, they are specifically interested in the branchio-oto-renal syndrome, reporting 5 cases. The syndrome combines deafness (middle ear and inner ear), pre-auricular and cervical fistulae and renal abnormalities. They attempt to summarise the syndrome by combining these 5 cases with the 15 published previously (4 initial cases of Melnick in 1974, 4 cases of Fitch in 1976, 7 of Fraser in 1978). The genetically transmissible nature of the syndrome would appear to be beyond doubt. The branchio-oto-renal syndrome was described for the first time in 1974 by Melnick, Bixter and Silk, who reported its existence in a father and three of his children, with an association of a malformation of the middle ear (block of the stapes), of the inner ear (cochlear hypoplasia), bilateral pre-auricular fistulae, bilateral cervical fistulae and renal hypoplasia with or without displasia. In 1974, Fitch and Srolowitz reported 4 new cases, and Fraser, Ling, Ologe and Nogrady a further 7 in 1978. The 5 cases published here bring the total known number to 20.