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Because Systemic Lupus Erythematosus (SLE) is a rare disease, and due to the significant prognostic impact of early management, a diagnosis confirmed by a physician with experience in SLE is recommended, for example from an expert center. Once the diagnosis is confirmed, existing manifestations should be identified in particular, renal involvement by an assessment of proteinuria, disease activity and severity should be determined, potential complications anticipated, associated diseases searched for, and the patient's socioprofessional and family context noted. Therapeutic management of SLE includes patient education on recognizing symptoms, understanding disease progression as well as when they should seek medical advice. Patients are informed about routine checkups, treatment side effects, and the need for regular vaccinations, especially if they are receiving immunosuppressive treatment. They are also advised on lifestyle factors such as the risks of smoking, sun exposure, and dietary adjustments, especially when they are receiving corticosteroids. The importance of contraception, particularly when teratogenic medications are being used, and regular cancer screening are emphasized. Support networks can help relieve a patient's isolation. The first-line medical treatment of SLE is hydroxychloroquine (HCQ), possibly combined with an immunosuppressant and/or low-dose corticosteroid therapy. The treatment of flares depends on their severity, and typically involves HCQ and NSAIDs, but may be escalated to corticosteroid therapy with immunosuppressants or biologic therapies in moderate to severe cases. Because there is no curative treatment, the goals of therapy are patient comfort, preventing progression and flares, and preserving overall long-term health and fertility. The frequency of follow-up visits depends on disease severity and any new symptoms. Regular specialized assessments are necessary, especially when treatment changes, but a frequency of every 3 to 6 months is recommended during periods of remission and monthly during active or severe disease, especially in children. These assessments include both clinical and laboratory tests to monitor complications and disease activity, with specific attention to proteinuria. [ABSTRACT FROM AUTHOR]

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Kawasaki disease (KD) is an acute vasculitis with a particular tropism for the coronary arteries. KD mainly affects male children between 6 months and 5 years of age. The diagnosis is clinical, based on the international American Heart Association criteria. It should be systematically considered in children with a fever, either of 5 days or more, or of 3 days if all other criteria are present. It is important to note that most children present with marked irritability and may have digestive signs. Although the biological inflammatory response is not specific, it is of great value for the diagnosis. Because of the difficulty of recognising incomplete or atypical forms of KD, and the need for urgent treatment, the child should be referred to a paediatric hospital as soon as the diagnosis is suspected. In the event of signs of heart failure (pallor, tachycardia, polypnea, sweating, hepatomegaly, unstable blood pressure), medical transfer to an intensive care unit (ICU) is essential. The standard treatment is an infusion of IVIG combined with aspirin (before 10 days of fever, and for a minimum of 6 weeks), which reduces the risk of coronary aneurysms. In case of coronary involvement, antiplatelet therapy can be maintained for life. In case of a giant aneurysm, anticoagulant treatment is added to the antiplatelet agent. The prognosis of KD is generally good and most children recover without sequelae. The prognosis in children with initial coronary involvement depends on the progression of the cardiac anomalies, which are monitored during careful specialised cardiological follow-up. [ABSTRACT FROM AUTHOR]

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This paper collects and describes several clinical experiences with extremely pre-term born babies. They were nursed using different kind of settings: institutional, are NICU (Neonatal Intensive Care Unit) with the subsequent clinical follow-up and with the help of private therapies. Through these lengthwise efforts, some of the pre-term newborn's features, such as the complexity in psychosomatic integration, were identified and better understood. The cases described show how the sensorial intrusion achieved from the intensive cares, may influence the integration process as the newborn's development further up, entangling the parental interactions. [ABSTRACT FROM AUTHOR]

Copyright of Orthodontie Française is the property of John Libbey Eurotext Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Au cours de la maladie de Sjögren ou syndrome de Sjögren primitif (SSp), les patients peuvent présenter des atteintes hétérogènes. La triade symptomatique caractéristique incluant sécheresse, fatigue et douleur est fréquente. Trente à 40 % des patients peuvent développer des atteintes systémiques. Ainsi, le suivi des patients atteints de SSp est personnalisé et doit être adapté au phénotype clinique et biologique du patient. Les modalités et la fréquence du suivi dépendent notamment de la présence ou non de complications systémiques, ou de leur risque de survenue. Le SSp étant une pathologie rare, il est recommandé d'organiser cette prise en charge en collaboration avec un centre de référence. La prise en charge est multidisciplinaire, impliquant différents professionnels de santé en fonction des atteintes initiales et de l'évolution. Le suivi a pour objectif de préciser l'activité et la sévérité de la maladie, ainsi que de dépister l'apparition d'atteintes systémiques. Il évalue l'efficacité et la tolérance des traitements et recherche des comorbidités. De plus, les facteurs de risque de lymphome doivent être régulièrement réévalués. Un suivi régulier de l'atteinte ophtalmologique et de l'état buccodentaire est nécessaire. Les recommandations récemment établies par l'EULAR portent sur la prise en charge de la triade sécheresse, fatigue et douleur, et détaillent la prise en charge des atteintes systémiques. De plus, le Protocole National de Diagnostic et de Soins (PNDS), établi récemment, propose une stratégie claire pour le suivi des patients. Enfin, l'éducation thérapeutique constitue un pilier de la prise en charge, permettant aux patients d'être actifs dans la gestion de leur maladie. In primary Sjögren's syndrome (pSS), patients may present with heterogeneous symptoms. The triad of characteristic symptoms is frequent and includes dryness, fatigue, and pain. Also, 30 to 40% of patients may develop systemic damage. Thus, pSS patients' follow-up is individualized and must be adapted to the patient clinical and biological phenotype. The modalities and the frequency of the follow-up depend on the presence or not of systemic complications, or their risk factors. As pSS is a rare disease, it is recommended to organize its management in collaboration with reference centers. pSS management is multidisciplinary and involves different health professionals depending on the initial damage characteristics and the evolution. The aim of the follow-up is to determine the activity and severity of the disease, as well as to detect occurrence of systemic complications. It assesses the efficacy and tolerance of treatments and includes the search for comorbidities. In addition, risk factors of lymphoma must be regularly reassessed. Regular monitoring of ophthalmologic and oral status is required. Recent EULAR guidelines address the management of the triad of dryness, fatigue and pain, and detail the management of systemic involvement. In addition, the "Protocole National de Diagnostic et de Soins" has been recently established and presents a clear strategy for patient management and follow-up. Finally, therapeutic education is a pillar of pSS management, allowing patients to be active in their disease follow-up. [ABSTRACT FROM AUTHOR]

The purpose of this article is to describe the external irradiation process and updated recommendations of the French society for radiation oncology for patient follow-up. [ABSTRACT FROM AUTHOR]

There are two main arguments in favour of systematic reproductive monitoring in young women treated for cancer: 1) to assess follicular loss induced by chemotherapy or pelvic radiotherapy, as well as spontaneous fertility, in order to refine fertility preservation (FP) strategies prior to treatment, 2) to judge the interest of a second FP phase at a distance from the disease according to the degree of ovarian recovery. Longitudinal monitoring of anti-müllerian hormone, a realtime reflection of follicular loss and recovery during treatment, makes it possible to classify chemotherapy protocols according to their degree of ovarian toxicity, thus making it possible to develop decision trees for FP before and after the disease. [ABSTRACT FROM AUTHOR]

Bourneville's tuberous sclerosis (STB) is a rare autosomal dominant genetic disorder. It is characterized by the multisystem development of hamartomas. The association of brain, heart, and skin lesions is strongly suggestive of the diagnosis, especially in the neonatal period. The cognitive and behavioral prognosis is improved by controlling spasms. We report the case of a newborn admitted for the management of prematurity, the diagnosis of STB was made secondarily on the discovery of cutaneous and cerebral lesions, complicated by epilepsy. The evolution was favorable under treatment. [ABSTRACT FROM AUTHOR]

Our work aimed to investigate the illnesses unrelated to systemic sclerosis (IUSS), diagnosed among patients with systemic sclerosis (SSc) throughout their follow-up in a referral tertiary care center. All the patients with SSc followed in the Internal Medicine Department of the University Hospital between October, 2014 and December, 2015, were included. We specifically reviewed the medical records of the patients who exhibited IUSS, defined as an illness that could not be considered as a typical clinical manifestation or as a usual complication of the disease. Two hundred patients were included, and 38 IUSS were diagnosed among 31 SSc patients, over a 4 years median follow-up period. These diagnoses included vascular diseases (26%), heart diseases (21%), neoplasia (8%), infectious diseases (6%), autoimmune diseases (5%), endocrinopathies (5%), and others (24%). The median follow-up time before IUSS diagnosis was two years. Seventeen (45%) of these diagnoses were considered in patients showing suggestive clinical signs. A specific therapy was delivered in 25 cases (66%). Group comparisons revealed that dyslipidemia was more frequent in patients with IUSS (OR = 2.6 [1.1–1.5]; p = 0.014), while no differences were found for the other characteristics. Especially, no association between auto-antibodies specificity and the occurrence of IUSS was found. This study focused on IUSS in SSc patients and highlights the need for a polyvalent clinical approach all along the follow up of SSc patients. [ABSTRACT FROM AUTHOR]

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Copyright of Bulletin de la Société de Pathologie Exotique is the property of John Libbey Eurotext Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Summary In primary male to female (MTF) sex reassignment surgery (SRS), the most frequent postoperative functional complications using the penoscrotal skin technique remain neovaginal stenosis, urinary meatal stenosis and secondary revision surgery. We aimed to retrospectively analyze postoperative functional and anatomical complications, as well as secondary procedures required after MTF SRS by penile skin inversion. All patients operated on for MTF SRS, using the inverted technique, from June 2006 to July 2016, were retrospectively reviewed. The minimum follow-up was one year (five-years maximum follow-up). Soft postoperative dilationprotocol was prescribed until complete healing of the vagina. We did not prescribe long-term hard dilation systematically. Possible short-depth neovaginas were primarily treated with further temporary dilation using a hard bougie. Among the 189 included patients, we reported a 2.6% of rectovaginal wall perforations. In 37% of patients we had repeated compressive dressings and 15% of them required blood transfusions. Eighteen percent of patients presented with hematoma and 27% with early infectious complications. Delayed short-depth neovagina occurred in 21% of patients, requiring additional hard dilatation, with a 95.5% success rate. Total secondary vaginoplasty rate was 6.3% (4.7% skin graft and 3.7% bowel plasty). Secondary functional meatoplasty occurred in 1% of cases. Other secondary cosmetic surgery rates ranged between 3 to 20%. A low rate of secondary functional meatoplasty was showed after MTF SRS by penile skin inversion. Hard dilation was prescribed in case of healed short-depth vagina, with good efficiency in most of cases. Secondary vaginoplasty was required in cases of neovagina stenosis or persisting short-depth neovagina after failure of hard dilation protocol. [ABSTRACT FROM AUTHOR]

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Résumé Objectif de l’étude Apprécier les résultats de traitement des patients souffrant d’une forme sévère d’asthme parmi ceux traités dans deux centres de pneumophtisiologie impliqués dans un projet de prise en charge des asthmatiques au Bénin. Patients et méthode Étude de cohorte rétrospective ayant inclus tous les patients enrôlés de 01/2013 à 11/2015. Les caractéristiques du patient, la sévérité de l’asthme, le respect des contrôles planifiés, les résultats de l’évaluation annuelle étaient colligés, enregistrées puis analysées dans EpiData et SPSS 21. Résultats Sur 491 asthmatiques ayant au moins une crise hebdomadaire, 194 (40 %) avaient une forme sévère, sex-ratio = 0,81, âge médian = 47 ans (écart interquartile = 32–60 ans). Parmi eux, la proportion venue aux contrôles avait progressivement diminué : 98 % (15 e jour), 74 % (1 mois), 63 % (3 mois), 54 % (6 mois), 51 % (9 mois) et 41 % (1 an). À l’évaluation annuelle, 115 (59 %) étaient absents. L’état était stationnaire chez 52 (27 %) vus. Seul un âge supérieur à 41 ans (risque relatif = 2,14 ; IC 95 % = 1,11–4,13 ; p = 0,02) était associé à une absence d’amélioration de l’état. Conclusion Les formes d’asthme diagnostiquées sévères dans le projet sont nombreuses. Ces patients honorent de moins en moins leur rendez-vous de contrôle et sont peu améliorés à l’évaluation annuelle. L’application des recommandations de GINA et le subventionnement d’autres molécules devraient aider à améliorer leur état. Objective To assess treatment outcomes of patients with severe diagnosed asthma among those treated in the two major pulmonology-tuberculosis centers involved in an asthma management programme in Benin. Patients and methods This was retrospective cohort study that included all patients enrolled between January 2013 and November 2015. Data on patient's characteristics, asthma severity, compliance with planned control visits, and annual evaluation results were collected, entered and analyzed using the EpiData and SPSS 21 software packages. Results Of the 491 asthmatic patients presenting at least one weekly attack, 194 (40%) had a severe form, with a male-female ratio of 0.81 and a median age of 47 years (interquartile range = 32–60 years). The proportion of those adhering to control visits gradually fell: 98% (2 weeks), 74% (1 month), 63% (3 months), 54% (6 months), 51% (9 months) and 41% (1 year). The annual assessment of 52 (27%) patients showed no improvement. In addition, of the 115 (59%) not attending this appointment, 82 (42%) did not improve, based on their last assessment in the programme. The only factor associated with lack of improvement was being aged over 41 years (adjusted relative risk = 2.14; 95% CI = 1.11–4.13; P = 0.02). Conclusion Many patients in the project were diagnosed with severe asthma. Their attendance of follow-up gradually decreased during and their condition showed little improvement at their annual evaluation visits. Application of the GINA recommendations and subsidizing other drugs would doubtless help to improve their condition. [ABSTRACT FROM AUTHOR]

Objective: To compare the performance of two novel recombinant antigens (EgP29, 2B2t) with imaging in a well-defined cohort of surgically treated cystic echinococcosis (CE) patients to determine whether serology reflects surgical cure as defined by imaging.Methods: From a cohort of 223 CE-confirmed patients of a national clinical center for echinococcosis, 36 surgically treated patients were eligible for analysis. Sera were tested by enzyme-linked immunosorbent assay (ELISA) for specific IgG and IgG4 antibodies against the EgP29 and 2B2t antigens. We used a hierarchical linear regression model to examine the course of antibody levels over time for each patient. A meta-analysis of the patient-specific estimates of the time to negativity was performed using the metan command in Stata.Results: The range of positive serological results at the beginning of post-surgical monitoring was 34-60%: 2B2t 51%, 2B2t-IgG4 34%, EgP29 60% and EgP29-IgG4 40%. The pooled estimates of time to seronegativity were as follows: 2B2t-ELISA 3.92 (3.24, 4.61) years; 2B2t-IgG4-ELISA 4.60 (3.91, 5.29) years; EgP29-ELISA 3.94 (3.50, 4.39) years; EgP29-IgG4-ELISA 2.55 (1.93, 3.18) years.Conclusion: After surgical treatment, antibodies to the recombinant antigens 2B2t and EgP29 become negative in the majority of CE-confirmed, surgically cured patients. The major drawback is the fact that only around half of the CE-confirmed, surgically treated patients were at all responsive to the test antigens, so they are of limited benefit for documenting primary cure. Equally, these antigens do not appear to be sensitive to recurrences. [ABSTRACT FROM AUTHOR]

The purpose of this study is to analyze the effectiveness of surgery and follow-up of children operated on for burn sequelae. For many years, we have organized two missions per year to Benin and Togo, one for surgery and one for follow-up. We analyzed the files of children born in Africa and victims of burns from the years 2002 to 2011. Children were referred through a non-governmental organization (NGO) and assessed in Africa by local paediatricians before and after surgery. Treatment consisted in operating on burn sequelae such as contractures, hypertrophic scars and hard cords. Impaired mobility was our only indication for the operation. We kept a database on all patients. Sixty files were reviewed, of which fifty were deemed suitable for analysis. The most common methods of surgery were skin grafting and Z-plasty. There were no complications, such as infection or graft/flap necrosis after immediate surgery. Long-term follow-up revealed a recurrence of hypertrophic scarring (47%), retractions (24%) and hard cords (2%) due to a lack of occupational therapy and physiotherapy treatment. Partnership with an NGO and a local team allows us to treat children with burn injury sequelae in Western Africa. A continued and often long-lasting follow-up by occupational therapists and physiotherapists is highly mandatory in order to guarantee good long-term results. In 2010, we initiated local rehabilitation therapy. [ABSTRACT FROM AUTHOR]

Inverted papilloma of the bladder is a rare and benign urothelial tumor, which usually affects adult patients with a clear male predominance. Macroscopic haematuria is the most frequent revealing symptom. Radiologic and cystoscopic signs are not always specific. Definitive diagnosis is based on anatomo-pathological examination. Treatment is based on endoscopic resection with coagulation of the tumor bed. Long term monitoring is necessary due to its common association with urothelial carcinoma. [ABSTRACT FROM AUTHOR]

The Cancer Survivorship Care Plans (SCP) aim to organize the post-cancer care and to give a new prominence to out-of-hospital health professionals. The structured follow-up (FU) based on a determined schedule is able to help professionals to comply with good practices and women to be regularly followed. Nowadays, the implementation of the SCP has been of limited scale and generally SCP are conceived without the inputs of non-hospital physicians. No assessment has been yet performed. Based on our more than 10 years experience of breast cancer patients FU in the network Gynecomed, we consider that the SCP improve the monitoring after cancer if a real cooperation has been set up between hospital and out-of-hospital physicians. This implies a co-construction FU process, a shared protocol, a mutual training process, a recall procedure, an information system between in and out hospital professionals allowing to discuss individual cases. [ABSTRACT FROM AUTHOR]

Résumé La radiothérapie stéréotaxique est une technique moderne de radiothérapie permettant de délivrer à visée ablative, de fortes doses d’irradiation dans de petits volumes. Cette modalité est en cours de développement pour le traitement des tumeurs hépatiques, qu’il s’agisse de carcinome hépatocellulaire, de cholangiocarcinome ou de métastases hépatiques. Elle constitue une véritable alternative à la résection chirurgicale ou à la transplantation hépatique, lorsque celles-ci sont contre-indiquées ou indisponibles. Il apparaît nécessaire de standardiser et de simplifier les méthodes actuelles d’évaluation et de suivi, afin de bénéficier d’une meilleure connaissance de l’efficacité, mais aussi de la tolérance de cette nouvelle modalité de traitement. Plusieurs sociétés savantes ont récemment proposé des recommandations quant à la réalisation de la radiothérapie stéréotaxique pour les tumeurs hépatiques. Nous avons réalisé une revue de la littérature afin de proposer des recommandations pour le suivi après réalisation d’une radiothérapie stéréotaxique de tumeurs de siège hépatique. Stereotactic body radiation therapy is a modern approach for delivering ablative high doses of irradiation in small volumes. This technique is being developed for the treatment of liver tumors, such as hepatocellular carcinoma, cholangiocarcinoma, or liver metastases. It represents a real alternative to surgical resection or liver transplantation when patients are inoperable or when tumours are unresectable. It is mandatory to standardize and simplify the current methods of evaluation and monitoring in order to benefit from a better understanding of the effectiveness and of the tolerance of this new treatment modality. Several scientific societies have recently proposed recommendations for the implementation of stereotactic radiotherapy for liver tumors. We conducted a literature review to provide recommendations for follow-up after completion of a stereotactic body radiation therapy for liver tumours. [ABSTRACT FROM AUTHOR]

Résumé La prise en charge des cancers des voies aérodigestives supérieures nécessite souvent un traitement multimodal lourd au sein duquel s’intègre la radiothérapie. La toxicité reste importante malgré les améliorations apportées par la radiothérapie avec modulation d’intensité. Après traitement, les patients sont confrontés à des problématiques différentes selon le délai post-thérapeutique. Ainsi, le suivi à court terme permet d’évaluer la cicatrisation des effets de la toxicité aiguë et la réponse au traitement. Le suivi à plus long terme permet de dépister les seconds cancers et récidives à distance ainsi que d’évaluer et de prendre en charge les séquelles tardives. Les soins de support médicaux et psychosociaux restent indispensables, même plusieurs années après rémission complète. L’objectif de ce travail était de préciser les modalités du suivi à court et long termes des patients ayant bénéficié d’une radiothérapie pour un cancer des voies aérodigestives supérieures. Head and neck cancer management often involves heavy multimodal treatments including radiotherapy. Despite the improvement of intensity-modulated radiation therapy, acute and late toxicities remain important. After such treatment, patients have to face different potential problems, depending on the post-therapeutic delay. In this way, short-term follow-up permits to appreciate the healing of acute toxicities and response to treatment. Long-term follow-up aims to recognize second primitive tumours and distant failure, and to detect and manage late toxicities. Medical and psychosocial supportive cares are essential, even after several years of complete remission. The objective of this article is to review the modalities of short-term and long-term follow-up of patients who receive a radiotherapy for head and neck cancer. [ABSTRACT FROM AUTHOR]

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Abstract: Aim: The purpose of the study was to look for the prevalence, significance and management of preexcitation syndrome (PS) or symptoms reappearance after accessory pathway (AP) ablation. AP ablation actually is the first treatment of PS. Methods: Successful AP ablation was performed in 261 patients; reappearance of symptoms or PS on ECG occurred in 47 patients (18%) from 20minutes to several years. Their data were compared with remaining patients. Results: Recurrences were more frequent in patients with spontaneous malignant form (34 vs. 21%), in congenital heart disease (4.2 vs. 0%) (P <0.002), in case of complication (11 vs. 2%) (P <0.007) and of a longer duration of applications (304±209 vs. 188±182sec) (P <0.019). Forty percent of patients had the same symptoms and electrophysiological data as before ablation. Twenty-four percent had an improvement of symptoms and/or electrophysiological data. However, 3 initially asymptomatic patients became symptomatic after ablation. Twenty-six percent had another AP or another rhythm disorder. We recommend transesophageal electrophysiological study for the control because only 40% of patients required second ablation. Conclusions: Reappearance of symptoms or a PS on ECG after AP ablation was not rare (18%) and was inconsistently associated with the reappearance of all initial AP electrophysiological properties. Only 40% of patients required a second AP ablation. Another arrhythmia was possible. Non-invasive second evaluation should be preferred. However, asymptomatic patients before ablation could become symptomatic. [Copyright &y& Elsevier]

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At present, monitoring tumor response to therapy is a crucial issue in oncology. In daily clinical practice, tumormarkers tests and anatomical imaging modalities are the most used techniques, but they have significant limits. Positron emission tomography (PET) is a molecular and functionnal imaging technique and, is by this fact, considered as a method of choice for assessing tumor response with cytotoxic chemotherapies and even more with targeted therapies. Some limits of 18F-fluoro-deoxy-glucose (18F-FDG) PET are overcome by the current development of new radioactive tracers. For those reasons, PET appears to be the most encouraging technique to assess tumor response for the next decade. [ABSTRACT FROM AUTHOR]

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Abstract: Maple syrup disease (MSD) is a rare autosomal recessive disorder caused by a decreased activity of branched-chain-ketoacid dehydrogenase, the second enzyme in the pathway of degradation of branched-chain aminoacids (leucine, isoleucine and valine). The three main clinical forms described are : classical form, at the neonatal period, marked by a severe neurological toxicity, subacute form appearing later and thiamine sensitive form. In every case, diagnosis is based on an increase of leucine, isoleucine and valine in blood and urines, with an associated presence of alloisoleucine. Diagnosis is confirmed by molecular analysis of corresponding genes. In several situations of protein catabolism, episodes of intoxication can occur. Emergency treatment for neonates and acutely sick children or adults uses hypoenergetic artificial nutrition (parenteral or enteral nutrition) without aminoacids and consequently involving only glucido-lipidic nutriments. Forty-eight hours later, proteins are progressively reintroduced. Natremia must be controlled, and a supplementation with isoleucine et valine is mandatory. Hemodialysis or hemofiltration can be necessary. Chronic treatment needs a strictly limited diet concerning branched-chain aminoacids. Natural proteins are limited as well as fruits and vegetables intakes. Lipids and sweetened food are permitted ad libitum. Food is supplemented by hyprotidic produces available in some hospital pharmacies. Oncoming of deficiencies is prevented by suplementations in minerals, vitamins, trace-elements, n–3 fatty acids and non-essential aminoacids. Follow-up is performed for children by a pediatrist and by an informed practitioner for adults. Psychomotricity and growth of children must be as normal as possible. All along the life, a metabolic equilibrium is needed and nutritional assessments are recommended at least once a year. Blood leucine level is regularly measured. A diagnosis before birth is possible when MSD has been identified in a member of the family. Pregnancy must be carefully prepared and followed. [Copyright &y& Elsevier]

Abstract: The following article presents the main points of the follow-up plan of Alzheimer''s disease (AD) and related syndromes patients. The general objective of this follow-up plan is to improve the quality of live of these subjects and their family. The key points are assessments of cognitive decline, functional decline and complications such as behavioural and psychological symptoms of dementia (BPSD), malnutrition and gait and balance disorders. In clinical practice, different tools are available, but frequency of evaluation is not consensual. However, the aim of this follow-up is to detect, prevent and treat complications and to improve the use of residual functional abilities in basic activities of daily living. The physician also needs to detect and prevent caregiver''s exhaustion and to consider the ethical issues raised by the disease. The care plan is based on non pharmacological and pharmacological measures. The non pharmacological approach must be implemented first. The place of antidementia drugs is considered. Lastly, this follow-up plan aims to limit iterative admissions to emergency room and to increase the access to geriatric units. Communication and collaboration between specialist, family practitioner and caregivers are needed in order to reach the objective of quality of life improvement in AD patients. [Copyright &y& Elsevier]

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Copyright of Bulletin de la Société de Pathologie Exotique is the property of John Libbey Eurotext Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Objectives In human African trypanosomiasis (HAT, sleeping sickness), staging of disease and treatment follow-up relies on white cell count in the cerebrospinal fluid (CSF). As B lymphocytes (CD19 positive cells) are not found in the CSF of healthy individuals but occur in neurological disorders such as multiple sclerosis, B lymphocyte count may be useful for field diagnosis/staging and therapeutic follow-up in HAT. Methods Seventy-one HAT patients were diagnosed and 50 were followed-up 6–24 months after treatment. White cell counts were used for conventional staging (stage 1, ≤5 cells/μl CSF, n = 42; stage 2, ≥20 cells/μl, n = 16) and intermediate stage (6–19 cells/μl, n = 13). Slides containing 1 μl of CSF mixed with Dynabeads® CD19 pan B were examined microscopically to detect B cell rosettes (bound to at least four beads). Results Stage 1 patients exhibited zero ( n = 37) or one CSF rosette/μl ( n = 5), contrary to most stage 2 patients (14/16: ≥2 rosettes/μl). Intermediate stage patients expressed 0 ( n = 9), 1 ( n = 3) or 2 ( n = 1) rosettes/μl of CSF. During follow-up, rosette counts correlated with white cell count staging but were much easier to read. Conclusion B cell rosettes being easily detected in the CSF in field conditions may be proposed to replace white cell count for defining HAT stages 1 and 2 and limit uncertainty in treatment decision in patients with intermediate stage. [ABSTRACT FROM AUTHOR]

Abstract: Conversely to diagnostic criteria, no consensus follow-up criteria are available in primary Sjögren''s syndrome. Recent improvement in the evaluation of disease activity thanks to the consensus EULAR activity score (the European Sjögren''s syndrome Disease Activity index), the knowledge on natural history due to the setting-up of prospective patient cohorts, and the evaluation of new activity biomarkers, could allow the establishment of follow-up criteria. Thus, it could be possible to individualize the therapeutic strategy and identify patients with primary Sjögren''s syndrome who would benefit from an immunosuppressive treatment. [Copyright &y& Elsevier]

Abstract: Aims: Authors report their personal experience on the surgical treatment of repeated anterior shoulder dislocations, using an anterior bone block in accordance with the procedure suggested by Didier Patte. Results: From the year 1988 and during 10years, 35patients suffering from repeated anterior shoulder dislocations were surgically treated by this procedure. It was appropriate to retrospectively review the clinical and functional outcomes after Patte repair for recurrent anterior glenohumeral instability. The mean follow-up was approximately 10years, with a mean percentage of 75% for the observance. There was no recurrence after surgery. Moreover, 88% of patients had good outcomes, according to the scale suggested by Butel et al., with total resumption of work and physical activities. The occurrence of chronic complication such as osteoathrosis was scarce (only one case for the series), while 20% of subjects showed apprehension during some specific shoulder movements. Conclusion: In order to expect best functional outcomes, the choice of the surgical procedure should take into account the extent of initial injury. Considering these ground requirements, the Patte procedure seems to provide very satisfactory long-term functional outcomes. [Copyright &y& Elsevier]

Copyright of Ginecología y Obstetricia de México is the property of Federacion Mexicana de Ginecologia y Obstetricia and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Résumé: Objectif: Élaborer des recommandations relatives au suivi et au pronostic d’un rhumatisme psoriasique de forme périphérique pour la pratique quotidienne. Méthode: La procédure était la suivante: [•] le choix de 5 questions, bases des recommandations, par un comité scientifique selon la méthode Delphi; [•] la recherche bibliographique dans PUBMED, COCHRANE et les communications des congrès de la SFR, EULAR et ACR; [•] l’élaboration et la validation des recommandations par un groupe de 68 experts en précisant leur force, selon le niveau de preuve, et le degré d’accord des experts pour chaque recommandation. Résultats: Les questions sélectionnées étaient: 1) Comment évaluer en pratique courante l’activité articulaire et cutanée d’un rhumatisme psoriasique ? 2) Quels sont les éléments prédictifs de l’évolution (structurale, fonctionnelle et mortalité) d’un rhumatisme psoriasique ? 3) Quels sont les paramètres cliniques et biologiques à recueillir pour le suivi d’un rhumatisme psoriasique ? À quelle fréquence ? 4) Quels sont les examens d’imagerie à réaliser pour le suivi d’un rhumatisme psoriasique ? À quelle fréquence ? 5) Comment apprécier la réponse thérapeutique en pratique courante dans le rhumatisme psoriasique ? La recherche bibliographique a permis de sélectionner 1 181 articles. Après lecture des titres et des résumés, les résultats de 123 études ont été présentés aux experts comme base de la discussion. Sept recommandations de force B à D ont ainsi pu être élaborées et validées par les experts. Conclusion: Sept recommandations ont été élaborées concernant le suivi de patients atteints de rhumatisme psoriasique. Elles devraient permettre une homogénéisation ainsi qu’une optimisation des pratiques cliniques en France, conduisant à une meilleure prise en charge des patients. [Copyright &y& Elsevier]

Copyright of Pelvi-Perineologie is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Abstract: Implantable cardioverters-defibrillators (ICD) have emerged as a major treatment for life threatening ventricular arrhythmias. This technique is available in France in all the university hospitals and, with the favor of the new regulation, in some qualified private centers. However, ICD implantation and follow-up in a non-university hospital is infrequent. This study reports long-term results following ICD implantation in 152 patients (age 61 ± 13 years). The vast majority (49%) of the patients were implanted for post-infarction ventricular tachycardia and 17% in primary prevention for left ventricular dysfonction. An appropriate therapy was delivered in 83 patients including 68 (81%) treated by antitachycardia pacing without the need for a cardioversion shock. An inappropriate shock was observed in 13 patients (9%). Because of iterative shocks, catheter radiofrequency ablation was proposed among 9 patients, with a success in 8. In conclusion, the technique of the implantable defibrillator can be performed in a non-university hospital with acceptable results. The increase in the ICD number in France implies that there is a need for collaboration between non-university and university hospitals in managing routine and emergency follow-up. [Copyright &y& Elsevier]

Abstract: The purpose of this review was to analyze the literature concerning community-acquired pneumonia (CAP) and acute exacerbations of chronic obstructive pulmonary disease (AECOPD) published in the last five years. CAP and AECOPD are frequent diseases that are to this day still associated with high morbidity and mortality rates. Patient management, especially the choice of the management environment, outpatient or intensive care, depends on the knowledge and recognition of predictors of complications, mortality risk factors, and severity signs to the extent that predictive algorithms have been established. However, research and publications concerning discharge criteria and follow-up of CAP and AECOPD patients is more sparse. [Copyright &y& Elsevier]