Fingertip nail bed injuries in children: Comparison of suture repair versus glue (2-octylcyanoacrylate) with 1-year follow-up.

The main objective of this study was to compare the medium-term results of nail bed repair in children using glue (2-octylcyanoacrylate) versus absorbable sutures. The secondary objective was to compare the results of treatment in the emergency room versus the operating room. This retrospective review of 74 fingertip nail bed lacerations (68 children) evaluated the appearance and pain at the last follow-up visit (minimum of 1 year), and the operating time. Mean age was 3.3 years at time of injury (range 10 months-13 years), with a mean follow-up of 2.6 (1-7) years. Thirty-six nail beds were repaired with glue; 38 were sutured. The clinical outcomes in the two groups were similar. The rate of nail dystrophy was 14% (5% major) regardless of the technique. Nail bed repair time was significantly shorter in the glue group (10.2 vs. 20.3min, p<0.001). Forty-five repairs were performed in the operating room and 29 in the emergency room. The complication rate (early infections) was significantly higher in patients treated in the emergency room. Tissue adhesive (2-octylcyanoacrylate glue) is a reliable option for repairing nail bed lacerations, both in terms of outcomes and speed of repair. Treatment in the operating room is preferable.

Dermatological manifestations in Noonan syndrome: a prospective multicentric study of 129 patients positive for mutation.

BACKGROUND: Data on dermatological manifestations of Noonan syndrome (NS) remain heterogeneous and are based on limited dermatological expertise. OBJECTIVES: To describe the dermatological manifestations of NS, compare them with the literature findings, and test for dermatological phenotype-genotype correlations with or without the presence of PTPN11 mutations. METHODS: We performed a large 4-year, prospective, multicentric, collaborative dermatological and genetic study. RESULTS: Overall, 129 patients with NS were enrolled, including 65 patients with PTPN11-NS, 34 patients with PTPN11-NS with multiple lentigines (NSML), and 30 patients with NS who had a mutation other than PTPN11. Easy bruising was the most frequent dermatological finding in PTPN11-NS, present in 53·8% of patients. Multiple lentigines and café-au-lait macules (n ≥ 3) were present in 94% and 80% of cases of NSML linked to specific mutations of PTPN11, respectively. Atypical forms of NSML could be associated with NS with RAF1 or NRAS mutations. In univariate analysis, patients without a PTPN11 mutation showed (i) a significantly higher frequency of keratinization disorders (P = 0·001), including keratosis pilaris (P = 0·005), ulerythema ophryogenes (P = 0·0001) and palmar and/or plantar hyperkeratosis (P = 0·06, trend association), and (ii) a significantly higher frequency of scarce scalp hair (P = 0·035) and scarce or absent eyelashes (P = 0·06, trend association) than those with PTPN11 mutations. CONCLUSIONS: The cutaneous phenotype of NS with a PTPN11 mutation is generally mild and nonspecific, whereas the absence of a PTPN11 mutation is associated with a high frequency of keratinization disorders and hair abnormalities.

MACVIA-LR (Fighting Chronic Diseases for Active and Healthy Ageing in Languedoc-Roussillon): A Success Story of the European Innovation Partnership on Active and Healthy Ageing.

The Région Languedoc Roussillon is the umbrella organisation for an interconnected and integrated project on active and healthy ageing (AHA). It covers the 3 pillars of the European Innovation Partnership on Active and Healthy Ageing (EIP on AHA): (A) Prevention and health promotion, (B) Care and cure, (C) and (D) Active and independent living of elderly people. All sub-activities (poly-pharmacy, falls prevention initiative, prevention of frailty, chronic respiratory diseases, chronic diseases with multimorbidities, chronic infectious diseases, active and independent living and disability) have been included in MACVIA-LR which has a strong political commitment and involves all stakeholders (public, private, patients, policy makers) including CARSAT-LR and the Eurobiomed cluster. It is a Reference Site of the EIP on AHA. The framework of MACVIA-LR has the vision that the prevention and management of chronic diseases is essential for the promotion of AHA and for the reduction of handicap. The main objectives of MACVIA-LR are: (i) to develop innovative solutions for a network of Living labs in order to reduce avoidable hospitalisations and loss of autonomy while improving quality of life, (ii) to disseminate the innovation. The three years of MACVIA-LR activities are reported in this paper.

[Diagnostic value of selective anorexia in pathological weight loss]. / Évaluation de l'apport diagnostique des anorexies sélectives au cours des amaigrissements pathologiques.

PURPOSE: The diagnostic value of selective anorexia is debated. Some authors have suggested an association between meat aversion and cancer, but most do not use it as a diagnostic tool. We aimed to characterize anorexia of different diseases to search for an association between selective aversions and diagnostic groups. METHODS: All the patients admitted to three departments of a teaching hospital were included consecutively for 22months if they had more than 10 % weight loss in less than one year. Patients were excluded if history taking was not reliable, or if they suffered from anorexia nervosa. We compiled diagnoses at discharge and validated them six months later. We used logistic regression to identify independent factors associated with selective anorexia. RESULTS: Inclusion criteria were met in 106patients (female 44 %, median age 65years). Most frequent diagnoses were: cancer (36 %), infection (35 %), digestive diseases (19 %), non organic diseases (21 %). Recent selective anorexia was found in 46 % of the cases. It was significantly associated with female gender (P=0.002), marginally with young age (P=0.069) and long duration of weight loss (P=0.079). Opioid use at admission was negatively associated with selective anorexia (P=0.001). No specific diagnostic category was found to be associated. CONCLUSION: Selective anorexia does not appear to be a useful symptom to investigate pathological weight loss. It behaves more like a non-specific reactivation by current disease of earlier latent personal food aversions.

[Mitochondrial neurogastrointestinal encephalopathy disease]. / L'encéphalomyopathie neuro-gastro-intestinale mitochondriale : un tableau d'anorexie mentale atypique.

Mitochondrial neurogastrointestinal encephalopathy disease (MNGIE) is a rare autosomal-recessive syndrome, resulting from mutations in the TYMP gene, located at 22q13. The mutation induces a thymidine phosphorylase (TP) deficit, which leads to a nucleotide pool imbalance and to instability of the mitochondrial DNA. The clinical picture regroups gastrointestinal dysmotility, cachexia, ptosis, ophthalmoplegia, peripheral neuropathy, and asymptomatic leukoencephalopathy. The prognosis is unfavorable. We present the case of a 14-year-old Caucasian female whose symptoms started in early childhood. The diagnosis was suspected after magnetic resonance imaging (MRI), performed given the atypical features of mental anorexia, which revealed white matter abnormalities. She presented chronic vomiting, postprandial abdominal pain, and problems gaining weight accompanied by cachexia. This diagnosis led to establishing proper care, in particular an enteral and parenteral nutrition program. There is no known specific effective treatment, but numerous studies are in progress. In this article, after reviewing the existing studies, we discuss the main diagnostic and therapeutic aspects of the disease. We argue for the necessity of performing a cerebral MRI given the atypical features of a patient with suspected mental anorexia (or when the clinical pattern of a patient with mental anorexia seems atypical), so that MNGIE can be ruled out.

Nodular recurrence and hypothyroidism following partial thyroidectomy for benign nodular thyroid disease in children and adolescents.

OBJECTIVES: Diagnostic and therapeutic processing of a thyroid nodule in children and adolescents may require lobectomy-isthmusectomy (LI) or nodule-resection (NR). Very few data in the literature report the long-term evolution of the remaining thyroid lobe in a defined pediatric population. In this study, we aimed to answer the following questions: Does a nodule recurrence occur in the remainder lobe? Is a post-operative thyroxine treatment necessary? MATERIAL AND METHODS: This retrospective study describes 28 patients under 18 who underwent LI (22 cases) or NR (6 cases) from January 2004 to March 2012. Ten of them were lost to follow up, 18 could be assessed (4 NR (22%) and 14 LI (78%) - mean follow-up 45±31 months). All patients benefited of post-operative thyroid ultrasonography, and regular endocrinologic follow-up. The following data were analysed: emergence of new thyroid nodules, evolution of pre-existing nodules, occurrence of post-operative hypothyroidism and requirement for completion thyroidectomy. RESULTS: The mean age at the time of surgery was 14.3±1.9 years. Two patients (11%) had pre-existing nodules in the remaining thyroid gland, none of which showed an increase in size after surgery. De novo nodules developed in five patients (27.8%). Three patients who underwent LI (21.4%) needed thyroxine treatment for post-operative hypothyroidism. One patient (5.5%) needed completion thyroidectomy. CONCLUSIONS: In this children and adolescents population, after performing LI or NR, remaining thyroid tissue stays free of nodules in 72.2% of the cases. A post-operative thyroxin treatment is necessary in 21.4% of cases after LI.

Thyroid surgery in children and adolescents: a series of 65 cases.

OBJECTIVES: To describe the specificities and complications of thyroid surgery in children and adolescents. MATERIAL AND METHODS: This retrospective study was based on 64 patients under the age of 18 who underwent thyroid surgery between January 2004 and March 2012, with two operations in one case. The following data were analysed: anatomical variants of the recurrent laryngeal nerve, postoperative recurrent laryngeal nerve paralysis rate, postoperative hypoparathyroidism rate, and histological results. RESULTS: Two cases of right non-recurrent inferior laryngeal nerve were observed (2.2% of the 93 recurrent laryngeal nerves dissected). One case of persistent left recurrent laryngeal nerve paralysis was observed (1.1%) despite intraoperative recurrent laryngeal nerve monitoring. Eight cases of immediate postoperative hypocalcaemia were observed (23.5% of the 34 total thyroidectomies) and permanent hypocalcaemia was observed in 5 cases (14.7%) with a significantly lower immediate postoperative serum calcium than in the case of transient hypocalcaemia (P=0.035). Among the 11 patients operated for familial medullary thyroid carcinoma (MTC), 36.3% presented one or more sites of C-cell carcinoma. Among the 32 patients operated for thyroid nodule, 6.3% presented papillary adenocarcinoma. Histological results were benign in all other cases. CONCLUSIONS: Thyroid surgery in children and adolescents is part of global multidisciplinary management of thyroid disorders in children. Recurrent laryngeal nerve paralysis is a rare complication, but may occur despite the use of intraoperative recurrent laryngeal nerve monitoring. Permanent hypoparathyroidism is the most common complication and is correlated with immediate postoperative serum calcium. Systematic prophylactic total thyroidectomy in patients with a RET proto-oncogene mutation allowed early diagnosis of MTC in one-third of cases. In view of the low rate of malignant nodules in our series, the malignant thyroid nodule rates reported in children in the literature may be overestimated.

[Guidelines concerning urinary incontinence in elderly: construction and validation of GRAPPPA algorithm]. / Recommandations concernant l'incontinence urinaire de la personne âgée : construction et validation de l'algorithme décisionnel GRAPPPA.

OBJECTIVES: Provide guidelines presented as an algorithm for practical evaluation and first line therapy of urinary incontinence in elderly. PATIENTS AND METHODS: Guidelines using formalized consensus guidelines method. These guidelines have been validated by a group of 40 experts quoting proposals, subsequently reviewed by an independent group of multidisciplinary experts (urologist, general practitioner, neurologist, gynecologist, geriatrist, specialist in physical medicine and rehabilitation). RESULTS: By means of 3 rounds of interrogation of the expert panel, GRAPPPA algorithm was constructed. This algorithm take in account both evaluation and first line therapeutic options in the different type of incontinences observed in this population (urge, stress and mixed incontinence). Initial evaluation consists to track down urinary retention (and subsequently fecal stool impaction, use of anticholinergic or morphinic drugs), urinary tract infection and cognitive impairment. Haematuria, bladder-pelvic pain, history of radiotherapy or recent pelvic surgery, lead to refer the patient to a specialized unit. First line therapy is in all the cases pelvic floor training, use of local oestrogenotherapy and dietetic measures. In urge incontinence, anticholinergic drugs may be used. CONCLUSIONS: Implementation of this algorithm may promote best practice in management of urinary incontinence in elderly.

Acquired deforming hypertonia and contractures in elderly subjects: definition and prevalence in geriatric institutions (ADH survey).

OBJECTIVE: By proposing a new terminology (acquired deforming hypertonia or ADH) and a new definition for contractures, the main objective of this study was to establish their prevalence among institutionalized elderly patients. Secondary objectives were to analyze the negative impact of ADH and collect the opinions of clinicians on the possible treatments available. METHODOLOGY: A multicenter cross-sectional study was conducted among residents of 39 geriatric institutions (29 EHPAD and 10 USLD). All subjects presenting at least one ADH were surveyed over a one-week period. RESULTS: Among 3145 observed patients (mean age 88.9±9.7 years) 22% (n=692) presented at least one ADH. In average, each patient presented 4.4±3.2 ADHs. Negative consequences on self-care, nursing and difficulties in getting dressed were most frequently observed. Only 25.4% of clinicians considered the ADH to be potentially reversible. Physical therapy was the therapeutic option most frequently chosen over medication and surgery. CONCLUSION: This study confirms the important prevalence of ADH among elderly institutionalized patients. Consequences on the level of dependence were significant. It seems relevant to elaborate hierarchical therapeutic strategies in order to counter these disorders and the fatalism that ensues.

[Chronic obstructive pulmonary disease (COPD) in the elderly]. / La bronchopneumopathie chronique obstructive (BPCO) du sujet âgé en huit questions/réponses.

Chronic obstructive pulmonary disease (COPD) is a chronic disease characterized by incompletely reversible airflow limitation, measured by a decrease of FEV(1)/FVC ratio. International consensus does not agree on a single threshold for this ratio, which can define airflow obstruction. Although the prevalence of COPD in the elderly population varies according to the definition used, it definitely increases with age and could reach 15% in those over 65 years of age. Therefore, ageing of the population should result in increased prevalence and socioeconomical costs of COPD during coming years. In France, diagnosis of COPD in the elderly is difficult, late and insufficient. Management, which has the same goals as in younger populations, has to be global and coordinated. Some points should be considered with particular attention considering the cumulative risks related on the one hand to COPD and on the other to ageing: pharmacological side-effects, decreased physical and social autonomy, nutritional impairment, comorbidities. Given the lack of specific data in elderly populations, pharmacological indications are generally considered to be the same as in younger populations, but some additional precautionary measures are necessary. Pulmonary rehabilitation seems to be beneficial at any age. Palliative care comes up against important difficulties: an indefinite beginning of the palliative stage in COPD; insufficient palliative care resources; insufficient communication; insufficient utilization of palliative care resources. Global COPD management in elderly requires coordination, best reached in health care network organizations involving medical and/or social professionals.

[Recommendations for the treatment of prostate cancer in the elderly man: A study by the oncology committee of the French association of urology]. / Recommandations pour la prise en charge du cancer de la prostate chez l'homme âgé : un travail du comité de cancérologie de l'association française d'urologie.

The increase in life expectancy combined with the increase in the global incidence of cancers will probably results in an increase in the number of cancers observed in the elderly. The increase in the incidence of prostate cancers in geriatric patients (45% of prostate cancers are diagnosed after 75 years old) is in sharp contrast with the lack of strong scientific data on the topic. By the meantime, oncogeriatrics has been developing for some years now under the guidance of the International Society of Oncogeriatrics. Such an approach aims at palliating the low quality of care of cancers in geriatric patients. The reasons for the low quality of care come from the characteristics of these patients and from the training of the care providers. The authors recall the principles of oncogeriatric evaluation and the classification of patients as it is actually proposed. They describe the main treatments and their results in the geriatric population and they describe the decision process concerning the choice of the treatment. They also suggest some guidelines on the diagnosis of prostate cancer, evaluation of the patients and the treatments of the disease in the elderly. Prostate cancer is almost the perfect model for oncogeriatrics. Urologists should remain the corner stone of its management, whatever the age of their patient.

Prevention of progression to dementia in the elderly: rationale and proposal for a health-promoting memory consultation (an IANA Task Force).

Alzheimer's disease (AD) is the most frequent form of dementia and according to the most recent estimation it affects nearly 27 million people in the world. The onset of the disease is generally insidious. It is becoming increasingly evident that the underlying pathophysiological mechanisms are active long before the appearance of the clinical symptoms of the disease. In the current context, it is important to develop strategies to delay the onset of cognitive decline. Delaying the onset by 5 years would reduce the prevalence by half at term, and a delay of 10 years would reduce it by three-quarters. The effectiveness of currently suggested preventive approaches remains to be confirmed, but certain strategies could be applied straight away to at-risk subjects. We propose that a health-promoting memory consultation should be set up for elderly persons who have attended a specialized memory consultation and in whom the diagnosis of dementia and of AD in particular, has not been established by standardized tools. Through this consultation, they would be offered full multidimensional investigation of all aspects of their health status, follow-up could be organized, general practitioners in private practice could be made more conscious of this population and the elderly could be made more aware of the risk factors to which they are exposed. The development of an information policy for the elderly would meet a present need. In our reflection, we must take into account the question of how to give this preventive consultation its due place in the healthcare pathway of the elderly person in order to ensure coordinated follow-up with all the other health professionals involved. The principle of the health-promoting memory consultation is undergoing validation in a large French multicentre preventive trial in 1200 frail elderly persons aged 70 years followed for three years, the Multidomain Alzheimer Preventive Trial (MAPT).

[Clinical expression of precocious pubertal development in girls]. / Expression clinique des précocités pubertaires chez la fille.

The paediatric endocrinologist is frequently asked whether pubertal development in a girl is normal, early or too early (precocious). This review will cover all clinical expression of premature development of puberty: central precocious puberty (neurogenic, secondary, and idiopathic) where treatment with GnRHa is considered, early puberty, partial puberty or pubertal variants and peripheral or pseudo precocious puberty related to an antonomous hypersecretion of estrogens by the ovaries. A special attention should be paid also to the role of environmental disruptors in the development of peripheral precocious puberty. GnRHa treatment should be considered only when evidence of central activation of the gonadotropic axis is proved by the LHRH-test.

A nationwide study of granulosa cell tumors in pre- and postpubertal girls: missed diagnosis of endocrine manifestations worsens prognosis.

BACKGROUND: There are very few data on the natural history of ovarian granulosa cell tumors (OGCT) in children. The aim of this study was to determine whether early recognition and diagnosis of the initial endocrine signs could improve the outcome of these tumors. METHODS: In a nationwide study from 1990 to 2004, we analyzed the clinical, biological and pathologic data from 40 pre- and postpubertal girls presenting an OGCT. RESULTS: 1. Among the prepubertal girls (n = 29), 17 OGCTs were diagnosed on the basis of precocious pseudopuberty. None of the 17 girls had a peritoneal spread of the tumor (100% FIGO stage Ia). Diagnosis based on a tumoral or acute abdomen (12 cases) was associated with frequent intraperitoneal ruptures of the tumor (50%) and a risk of relapse (2 cases). Of the eight girls who had had a misdiagnosed precocious pseudopuberty, five had a pre- or perioperative tumoral rupture. 2. Among the postpubertal girls (n = 11), endocrine manifestations such as secondary amenorrhea or virilization had been underevaluated in three of them and the diagnosis was established from a tumoral abdomen. This clinical presentation was associated with frequent ruptures of the mass in the peritoneum (80%) and a higher risk of recurrence (30%). 3. A delayed diagnosis of OGCT despite previous endocrine signs (11 cases; 8 pre- and 3 postpubertal) was associated with a high risk of pre- or peri-operative peritoneal tumor spreading (70% FIGO stage Ic or IIc, p <0.05). The mean delay for diagnosis ranged from 3 to 11 months. CONCLUSION: This study highlights the critical role of early diagnosis of OGCT in pre- and postpubertal girls, particularly at the first seemingly banal signs of endocrine disorder. Peritoneal spread of the tumor may thereby be prevented, which improves the prognosis.

[Medical practice analysis in internal medicine: a national descriptive study]. / Analyse des pratiques des spécialistes de médecine interne: une enquête transversale descriptive nationale.

PURPOSE: This descriptive and epidemiological study was conducted in Mars 2002 in Internal Medicine in order to (1) participate in elaborating a White Book about the speciality, (2) analyse the post-university formation needs of the specialists in Internal Medicine. METHODS: A questionnaire was sent to all specialists in Internal Medicine listed on the ADELI file (n = 2155). For the first three patients seen in consultation and during hospital stay, questioned specialists had to mention the age, sex, origin, motive of the visit, nature of symptoms, complexity of the problem and the nature of the required abilities. They also had to precise the main diagnosis of all patients seen in the same day. RESULTS: Three hundred and sixty answers have been received. Three hundred and thirty two were exploitable. Five thousand six hundred and eleven main diagnosis were listed. Fifteen percent of the questioned specialists did practise in other specialities than Internal Medicine. Orphaned diseases were the most common pathologies carried out in consultation (17%). Patients seen during their hospital stay suffered more frequently from infectious, haematological and malignant diseases. In 55% of the cases, patients were seen in second or third line after a visit to a general practitioner or another specialist. The abilities of the Internal Medicine specialist alone were sufficient in 70% of the cases to solve the problem. Complexity of the problem was evaluated by the specialists themselves at about 45/100 on an analogical scale. CONCLUSIONS: This study inform the medical community about the type of patients treated by the specialists in Internal Medicine, precise the exact nature of their professional exercise and their real need in medical post-university formation.

[Polycystic ovary syndrome in pubertal period: clinical, biological, metabolic and genetic polymorphism]. / Le syndrome des ovaires polykystiques en période péri-pubertaire : polymorphisme clinique, biologique, métabolique et génétique.

Polycystic ovary syndrome (PCOS) is a common cause of hyperandrogenism in adolescent girls. In its complete post menarchal expression, the syndrome is characterized by the association of typical clinical, biological, and ultrasonographic findings. Many factors have contributed to our knowledge of different clinical forms of PCOS in adolescent girls. They are helpful for clarifying misleading situations in a period of life when diagnosis of PCOS implies a treatment for many years and may interfere with gynecological outcome. During the last 3 years, we had the opportunity to manage in our unit 45 adolescent girls with ovarian hyperandrogenism: 32 of them had PCOS and the other 13 functional ovarian hyperandrogenism defined by clinical and biological hyperandrogenism without ultrasonographic abnormality. In this review, we report, from our personal experience as well as from recent literature data, the different clinical expressions of PCOS in the pubertal period: the classical post menarchal form, the exceptional pre menarchal form, the post precocious pubarche and the post precocious puberty forms, the familial expression as well as the dominant metabolic expression.

Improved muscle strength and power in elderly exercising regularly.

Since the number of elderly people with a sedentary lifestyle is considerable and still growing, regular exercise could be an extra stimulant to compensate for the decrease in functional capacities. The potential positive effects of physical and sporting activities (PSA) on muscular function in relation to the period of practice were studied. Muscular strength and power were evaluated by dynamometric knee and ankle tests on 40 healthy subjects aged over 60 years old, split into four groups according to the period of practice. Higher muscular performance was obtained in subjects who had always practised PSA, whereas subjects who had always been inactive displayed the worst muscular performances. Subjects who had begun PSA practice lately had good muscular strength and power, close to those of the AA group, whereas the subjects who had stopped the practice at an early age did not perform as well. Life-long PSA attenuates the age-related loss of muscle function, and initiation at old age improves it. This, and the absence of any beneficial effects of PSA stopped when young, indicates that the actual activity level is an important determinant of skeletal muscle function at old age. Practicing PSA increases muscular strength and power and thus improves daily living activities in elderly people.