[Congenital thoracic deformities and 3D custom-made implants. New classification based on a series of 789 treated cases]. / Malformations thoraciques congénitales et implants sur mesure 3D. Nouvelle classification basée sur une série de 789 cas traités.

The authors present a new study on 789 cases of congenital thoracic malformations including 638 pectus excavatum and 151 Poland syndromes, according to a new classification which completes Chin's one. All these malformations were treated with silicone elastomer implants. The contribution of computer-aided design and manufacturing (CAD/CAM) since 2008 is essential. The one-stage surgical protocol is precisely described. The results are impressive, permanent, for life, and complications are rare. The authors evoke a common vascular etiopathogenesis theory at the embryonic stage and question the heavy techniques of invasive remodeling that are most often unjustified.

Iatrogenic mediastinitis in bronchiolitis: Importance of avoiding pharyngeal aspiration.

An 11-month-old infant was hospitalized for his first episode of severe bronchiolitis, with pneumomediastinum on the chest x-ray performed in the emergency room before hospitalization. After a few days, the occurrence of a feverish torticollis motivated a CT scan, revealing mediastinitis. An iatrogenic perforation was objectified in the posterior wall of the esophagus, probably caused by nasopharyngeal aspiration. This exceptional case has never been reported before, except in premature infants. This encouraged us to report this case to change systematic aspiration practices and prefer nasal suctioning in healthy infants with bronchiolitis presenting to the emergency department.

Non-typhi Salmonella enterica urinary tract infections.

OBJECTIVE: Non-typhi Salmonella enterica urinary tract infections (UTIs) are not frequent and rarely reported in the literature. We aimed to characterize clinical presentations and risk factors for the infection. PATIENTS AND METHODS: We performed a retrospective study of non-typhi Salmonella enterica strains isolated from urine cytobacteriological examinations (UCBE) collected between January 1, 1996 and October 30, 2014 and analyzed by the microbiology laboratories of the university hospitals of the western part of Île-de-France and of Paris, France. RESULTS: Twenty UCBEs positive for non-typhi Salmonella enterica were analyzed. The sex ratio was 0.53 and the average age of patients was 57 years. Clinical presentations were acute pyelonephritis, acute cystitis, and prostatitis. Eight cases of bacteremia were identified. Diarrhea was observed in half of patients, either before the UTI or simultaneously. No patient required to be transferred to the intensive care unit. Immunodeficiency and/or diabetes were observed in eight patients. Three patients presented with a uropathy. Prescribed antibiotics were third generation cephalosporins and fluoroquinolones. The average treatment duration was 20 days. A spondylitis and a purulent pleurisy were observed and deemed related to the UTI. Patient outcome was always favorable following treatment prescription. CONCLUSION: Non-typhi Salmonella enterica UTIs are rare. They are mainly observed in elderly patients presenting with immunodeficiency or an underlying urological disorder.

[Febrile ulceronecrotic Mucha-Habermann disease]. / Maladie de Mucha-Habermann fébrile et ulcéronécrotique.

Pityriasis lichenoides et varioliformis acuta (PLEVA) is an inflammatory skin disease that is unknown to pediatricians. The ulceronecrotic febrile form is a rare and potentially lethal variant. We report the case of a 7-year-old boy with a papulovesicular eruption lasting for 4 weeks, secondarily associated with ulcers and necrotic crusts, fever, and systemic signs. After exploring infectious causes such as chickenpox, we discussed and confirmed PLEVA with histological analysis. Systemic steroids and methotrexate improved the symptoms. It is necessary to mention PLEVA in case of prolonged papulovesicular eruption. Prompt diagnosis allows appropriate treatment, although there is no consensus on therapeutic guidelines.

[Childhood eruptive nevi: a case report]. / Syndrome du nævus éruptif de l'enfant à propos d'un cas.

"Eruptive nevi" is a phenomenon characterized by a rapid appearance of multiple melanocytic nevi. It is mainly developed in three groups of patients: those with systemic immunosuppression, bullous cutaneous disorders, and a melanocytic stimulation drug. We report on the case of an 11-year-old boy who was diagnosed with acute lymphoblastic leukemia. A few months after the beginning of the chemotherapy, he developed multiple pigmented lesions over the skin. Eruptive nevi syndrome has been described in the literature in 29 cases in the context of severe bullous disease and in immunosuppression. Nevi most often appear on the trunk and extremities, notably on the feet in the context of immunosuppression. They are localized in areas of bullous lesions in bullous diseases. Due to an increased melanocytic stimulation in eruptive nevi patients, long-term surveillance of individuals who have developed eruptive nevi is required, and increased sun prevention should be suggested.

[Two cases of severe pruritus revealing Hodgkin's disease in children]. / Prurit sévère révélant un lymphome de Hodgkin chez l'enfant : deux observations.

BACKGROUND: Pruritus in children is a frequent reason for consultation, most often related to a common dermatosis. Where dermatological investigation fails to reveal a dermatological cause, a general cause may be suspected. We report three cases of pruritus revealing Hodgkin's lymphoma in children. PATIENTS AND METHODS: Case 1: a 14-year-old girl presented pruritus with diffuse scratching lesions present for 6 months, associated with right cervical lymph nodes occurring after the onset of pruritus. Tomodensitometry revealed involvement of the supra- and sub-diaphragmatic lymph nodes as well as pulmonary involvement. Lymph node biopsy confirmed nodular sclerosing Hodgkin's lymphoma. Case 2: a 14-year-old boy was hospitalized for suspected psychogenic pruritus. He presented intense itching, predominantly in the lower extremities and at night, occurring over the previous 6 months as well as night sweats. Examination showed that the patient had lost 5kg in 1 month and had a low-grade fever of 38°C; he presented linear striated scratching lesions on both legs. Cervical and inguinal lymphadenopathy was seen. The chest scan also revealed supra-diaphragmatic adenomegalies. The biopsy confirmed Hodgkin's lymphoma. DISCUSSION: Systemic causes of pruritus in children are poorly described in the literature. In these two cases, pruritus allowed a diagnosis of Hodgkin's lymphoma to be made, emphasizing the important role of dermatologists in the early diagnosis of haematological malignancy.

[The emergency department: an appropriate place for identifying troubled teens]. / Les urgences, un lieu de repérage des adolescents en difficulté

OBJECTIVES: The main objective of this study was to estimate the prevalence of psychological distress among adolescents seen in emergency departments, and the secondary objective was to highlight their main reasons for consulting. METHODS: Cross-sectional study in three multicenter emergency departments receiving adolescents in Île-de-France conducted in 2010. All adolescents completed a questionnaire including the ADRS (Adolescent Depression Rating Scale, a screening questionnaire for depression) and a series of questions relating to somatization and risk behaviors. RESULTS: The study included 346 adolescents, 320 of which were fully analyzed. The ADRS score was considered normal (score < 3) for 70.6% of the sample (n = 226), 19.4% of adolescents (n = 62) had moderate depressive symptoms (3 ≤ score < 6), and 10.0% severe depressive symptoms (score ≥ 6) (n = 32). The majority of patients consulted for trauma and less than 10% for acute psychiatric problems; 17% of adolescents who came to the emergency department for a nonpsychiatric reason had an ADRS ≥ 3, i.e., with mental distress. CONCLUSION: The routine use of a self-administered questionnaire in the emergency services could identify adolescents with moderate to severe depressive symptoms.

[Apparent life-threatening events due to cow's milk allergy in infants]. / Malaises graves d'origine allergique chez le nourrisson.

The analysis of an apparent life-threatening event is mainly based on the clinical approach. When the event occurs after a meal, the diagnosis of acid gastroesophageal reflux complicated by esophagitis is suspected first. Cow's milk protein allergy is suspected when the event occurs during weaning from breastfeeding or when it is accompanied by urticaria or relapses when cow's milk is reintroduced. We report on two cases of an apparent life-threatening event associated with cow's milk protein allergy, whose symptoms and first diagnostic approach initially delayed diagnosis. Clinical manifestations of food allergy need to be known in order to avoid errors in diagnostic orientation and therapy, which may be responsible for more severe recurrences.

[Prevalence of chronic hyperventilation syndrome in children and teenagers]. / Prévalence du syndrome d'hyperventilation chronique chez les enfants et les adolescents.

OBJECTIVE: The aim of this study was to investigate the prevalence of hyperventilation syndrome in the general population of children and teenagers from the Île-de-France region (France). METHODS: Three hundred children and teenagers (170 girls and 130 boys, aged 1 to17 years) were included in the study. To evaluate the probability of hyperventilation syndrome, we asked the children and teenagers to complete the Hyperventilation Syndrome Ambroise-Paré Enfant (SHAPE) questionnaire. The frequency of occurrence of the signs was evaluated by the child himself or herself with or without parental help. Children and teenagers with a score of 25 or over were considered to have hyperventilation syndrome. RESULTS: Sixty-three out of 300 questionnaires with a score of 25 or over revealed the presence of hyperventilation syndrome: 21% of the population evaluated. Among those surveyed, 42 were girls and 21 boys: 24.7 and 16.2%, respectively. The 280 questionnaires filled out among the non-asthmatics showed that 52 were positive (18.6%), while the positivity rate in the asthma group amounted to 55%. CONCLUSION: Although the diagnostic criteria for hyperventilation syndrome remains contested, this study shows that the disorder is real and frequent.

Counselling on sun protection, a survey of French paediatricians.

BACKGROUND: High sun exposure during childhood is an important risk factor for skin cancers at adulthood. Paediatricians are first in line to provide sun protection (SP) information. OBJECTIVE: To describe paediatricians' attitudes towards SP counselling and compare French and American paediatricians' behaviours. METHODS: Methodology used by the American Academy of Pediatrics in 2001. Validation of the French version of the questionnaire. Survey of all 1108 paediatrician members of the Association Française de Pédiatrie Ambulatoire (AFPA) RESULTS More than 90% of paediatricians agreed that skin cancers are a Public Health concern, that preventing episodic high sun exposures during childhood would reduce the risk of adult melanoma, and that it is a paediatrician's role to educate parents/patients on SP. Three quarters reported counselling all of their patients. The most important SP recommendation was to avoid sun during peak hours, followed by sunscreen use. Only 48.3% of paediatricians rated SP as very important to their patients' health, at the 8th rank among selected preventive care topics. The most frequently identified barrier to SP counselling was cost of sunscreens followed by lack of sufficient time. CONCLUSIONS: The majority of paediatricians believe that prevention of skin cancers is a worthy issue and it is their role to educate patients, but less than half of them consider SP as an important topic among selected preventive care issues. Although paediatricians seem to know SP measures well, their (counselling?) seems to respond to patients' preoccupations more than to expert recommendations. French and American behaviours show some major differences.

[Recent progress in Marfan syndrome]. / Nouveautés dans le syndrome de Marfan.

For 20 years, the clinical, genetic and therapeutic knowledge of Marfan syndrome made great progress. The new classification, allowing the diagnosis and published in 2010, is easier to use. The diagnosis remains sometimes difficult particularly during childhood, because of the great variability of expression of the disease, from neonatal Marfan syndrome, to a weak clinical expression and especially because of the evolution of the signs with the age, which no classification takes into account. The search for amutation in the FBN1 gene is long and expensive and must be reserved only for patients having a very strong diagnostic suspicion. The therapeutics is preventive and based on patient education, limitation of the sport, beta blockade therapy, regular echocardiography as well as aorta replacement. New treatments are at present on approval but did not make the proof of their efficiency. The global care of the patient, by taking into account difficulties about genetic origin of the disease, aesthetic and psychological consequences, allows an improvement of the quality of life.

[Benign afebrile seizures in rotavirus gastroenteritis]. / Convulsions non fébriles bénignes associées à une gastro-entérite aiguë à rotavirus chez l'enfant.

Benign afebrile seizures associated with mild gastroenteritis and normal serum electrolytes are often described in Asian infants under the name of convulsions with mild gastroenteritis (CwG). Herein, we report 3 cases of CwG that occurred in Caucasian children. Clinical features and outcomes are described and the cases reported in the literature are reviewed. The CwG syndrome is now recognized as a distinct entity characterized by the following criteria: (1) it occurs in previously healthy 6-month to 3-year-old children who present with afebrile convulsions, (2) it is associated with mild gastroenteritis, (3) seizures tend to occur in a clustered manner, (4) interictal EEG shows no paroxysmal discharge, and (5) laboratory examinations are normal, including normal cerebrospinal fluid, serum electrolytes and glycemia. The CwG syndrome is a self-limited disease with a short course and a good prognosis. Investigations must be targeted and anticonvulsivant therapy is not required. As rotavirus is the most common agent among the diarrheal viruses detected to be the cause of CwG, one could hypothesize that this virus may play a role in the CwG pathogenesis.

High frequency, diversity and severity of skin diseases in a paediatric emergency department.

BACKGROUND: Skin disorders are a major concern in the Paediatric Emergency Department (PED). We provide an accurate evaluation of the incidence, characteristics and severity of skin disorders seen in our PED over a 1-year period. METHODS: A total of 20,652 children's medical notes were reviewed in a single centre, retrospective study in the PED of a University Hospital over a 1-year period. The dermatological disorders were analysed on the basis of different criteria including their incidence, patient age, sex ratio, diagnosis, seasonal variations and hospitalization rates. RESULTS: A total of 1897 (9.2%, F/M: 1.2; mean age: 4.1 ± 3.6 years) children presented with 1999 skin diseases and 69 different diagnoses. This frequency increased in the summer months (more than 14% of all patients). A total of 46.5% of diseases were infectious in nature (27.6% viral and 14.4% bacterial), inflammatory diseases accounted for 26.2% (urticaria and angio-oedema 15.9%, atopic dermatitis 3.5%, Henoch-Schönlein purpura: 2.1%), non-specific focal disease (balanitis, vulvitis, etc.) and insect bites, burns, transient diseases of the newborn and drug reactions for 9.2%, 7.8%, 6.4%, 3.7% and 1.2% respectively. More than 90% of children presented at the hospital for an acute condition and 155 (8.2% of children with skin disorders; F/M: 0.9; age: 4.0 ± 4.0 years) were hospitalized. More than 90% of hospitalizations were for infectious and inflammatory diseases. CONCLUSION: Our data reveal the extremely high frequency, diversity and potential severity of paediatric emergency skin disorders. Specific educational measures and closer co-operation between Dermatologists and Paediatricians are essential if the skin care dispensed to children and teenagers is to be improved.

[A new case of food protein-induced enterocolitis syndrome]. / Syndrome d'entérocolite induit par les protéines alimentaires, à propos d'une observation.

We report a case of food protein-induced enterocolitis syndrome (FPIES) with milk whose signs of milk intolerance began in the 1st days of life, consisting in minor and nonspecific symptoms. The 3 foods in question were cow's milk, soja, and wheat. The diagnosis of FPIES was suspected at the age of 9 months, after 3 hospitalizations for vomiting, sometimes associated with lethargy and hypotension, which occurred around 2h after cow's milk ingestion. Symptoms were not associated with positive specific IgE and cutaneous tests. Signs then occurred with soja and wheat. Because of the late diagnosis, 3 anaphylactic shock episodes occurred. FPIES is an uncommon cell-mediated food allergy reaction. This syndrome is characterized by gastrointestinal symptoms, especially severe vomiting, sometimes associated with anaphylactic shock. Usually signs occur 2h after ingestion. These reactions begin early, in the 1st months of life, and regress by the age of 3 years in 38-100% of cases depending on the responsible food. They are usually induced by cow's milk and soy proteins. Diagnosis is difficult and delayed because of nonspecific symptoms. Oral food challenge is the only examination that confirms the diagnosis. Treatment involves the exclusion of the specific food involved. Severe reactions require treatment of shock and adjunction of corticosteroids.

[Rapid malnutrition in patient with anorexia nervosa: experience of a general pediatric department]. / Dénutrition rapide au cours de l'anorexie mentale : expérience d'un service de pédiatrie générale.

OBJECTIVE: Rapid undernutrition in patients with anorexia nervosa can compromise vital functions, notably due to cardiac complications. The aim of this study was to analyze the clinical parameters of anorexic patients, hospitalized for substantial weight loss, in a general pediatric inpatient unit, in order to determine which parameters should be tested by the medical doctor. POPULATION AND METHODS: We performed a retrospective study on 20 consecutive patients (18 girls), median age of 13.75 (+/-2.3) years, admitted for the first time in our pediatric inpatient unit for anorexia nervosa. RESULTS: Symptoms evolved for a median duration of 11.5 (+/-10.2) months before admittance and was shorter for the youngest patients (r = 0.42, p = 0.067). The mean BMI was 13.3 (+/-0.6) kg/m(2) (-3.0+/-1.2 Z-score) and was inversely correlated with serum creatinine levels (74+/-15 micromol/l) (r = 0.44, p < 0.05). The mean BMI variation between the beginning of the disease and hospitalization (Delta BMI) was-3.5 Z-score and was correlated to low systolic blood pressure (r = 0.45, p< or =0.05) and the presence of a pericardial effusion at admittance (r = 0.45, p < 0.05). Complete blood count, electrolyte balance and the serum phosphorus levels were normal except in 1 case. Mean serum glucose was 3.5+/-1.2 mmol/l. At admittance, an electrocardiogram, performed for 16 patients, showed sinusal bradycardia without conduction impairment. Enteral nutrition was necessary for 14 patients (70%) for a mean duration of 18.1 days (range, 6-56 days). The mean weight gain was 3.1+/-2 kg and was inversely correlated to the BMI at admittance (r = 0.49, p < 0.05). CONCLUSION: Medical supervision of undernutrition tolerance during anorexia nervosa is above all clinical, as hematological and biological parameters remain normal for a long time. The cardiac complications found in our study appeared to be more related to the rapid rate of weight loss than to the amount of weight loss itself.

[Diagnostic value of the SHAPE questionnaire in recognition of the hyperventilation syndrome in children: a pilot study]. / Valeur diagnostique du questionnaire SHAPE pour le syndrome d'hyperventilation de l'enfant : étude pilote.

OBJECTIVE: Determination of a diagnostic scoring method for hyperventilation syndrome (HVS) in children and proposal of a simplified questionnaire. METHOD: We used the main clinical signs of HVS in children and adolescents identified in a previous study and classified them according to their odds ratios (OR). The intensity of each sign, measured using a visual analogic scale in the previous study, led us to analyze several scoring methods, with a breakdown between major and minor signs according to their OR. Building receiver operating characteristics (ROC) curves allowed us to choose the best diagnostic combination. RESULTS: A sample of 85 children and adolescents aged from 7 to 20 years (mean age, 11.9 years) was tested. This sample comprised 45 children with isolated HVS or HVS associated with asthma and 40 controls, with asthma but without HVS or attending our emergency unit after trauma. Seven respiratory signs and 10 nonrespiratory signs were selected. For each scoring method, a significant difference was observed between HVS and non-HVS patients. The most suitable area under the curve (0.934) and the best combination between specificity and sensitivity (Sp=0.90 and Se=0.82) were obtained when the scoring method was 0,3,6 for major signs, and 0,1,2 for minor signs, according to perceived intensity of each sign ("never or almost never", "sometimes" or "often or very often"). CONCLUSION: Since no gold standard is available to confirm the diagnosis of HVS in children, we propose using a simplified questionnaire composed of 17 items to compute a diagnostic score. The questionnaire will be validated shortly in a new prospective study.