Idiopathic central precocious puberty in a Klinefelter patient: highlights on gonadotropin levels and pathophysiology.

Background: Idiopathic central precocious puberty (ICPP) is supposed to be non-existent in a context of testicular destruction that is typically present in Klinefelter syndrome (KS). Herein, we describe a rare case of ICPP in a Klinefelter patient (47,XXY) with 2 maternal X chromosomes. Moreover, we highlight the differences in gonadotropin levels in comparison to males with ICPP and a normal karyotype.

Urinary club cell protein 16 (CC16): Utility of its assay during acute bronchiolitis.

Acute bronchiolitis is responsible for high morbidity in infants. Club cell protein 16 kDa (CC16) is a major pneumoprotein secreted by club cells of the bronchial epithelium and eliminated by the renal pathway. CC16 seems to be a biomarker of epithelial damage in asthma.

Fascial Hydrodissection for Chronic Hamstring Injury.

Hamstring muscle injuries (HMI) are common among athletes. HMI can take many months to years to resolve. Often, athletes do not report complete resolution with typical conservative therapy.

Assessment of blood enterovirus PCR testing in paediatric populations with fever without source, sepsis-like disease, or suspected meningitis: a prospective, multicentre, observational cohort study.

Background: Enteroviruses are the most frequent cause of acute meningitis and are seen increasingly in sepsis-like disease and fever without source in the paediatric population. Detection of enterovirus in cerebrospinal fluid (CSF) specimens by PCR is the gold standard diagnostic test. Our aim was to assess a method of detecting enterovirus in blood specimens by PCR.

Laryngotracheoesophageal cleft, a rare differential diagnosis of esophageal atresia.

A laryngotracheoesophageal cleft, commonly called laryngeal cleft (LC), is a congenital malformation of the posterior part of the larynx creating an abnormal communication between the laryngotracheal axis and the pharyngoesophageal axis. The prenatal ultrasonographic features associating absent stomach, polyhydramnios and mediastinal "pouch sign" are usually considered pathognomonic for esophageal atresia. This observation demonstrates that they can also correspond to a severe form of laryngotracheoesophageal cleft extending to the carina.

Serum soluble receptor for advanced glycation end-products during acute bronchiolitis in infant: Prospective study in 93 cases.

Introduction: Acute bronchiolitis is a major cause of acute respiratory distress in infants. The soluble receptor for advanced glycation end-products (sRAGE) is a biomarker of pulmonary damage processes, with a diagnostic and a prognostic value in acute respiratory distress syndrome (ARDS). The RAGE pathway is also implicated in the pathogenesis of other respiratory diseases like asthma, but the value of sRAGE levels in acute bronchiolitis remains under-investigated.

Biomarkers for severe allergic asthma in children: could they be useful to guide disease control and use of omalizumab?

Although symptom controls in asthmatic children can be achieved through compliant use of conventional medication, some children have uncontrolled severe persistent asthma, especially if they are allergic. For these children, omalizumab (approved by the EMA and FDA in children aged > 6 years) could be a therapeutic option. However, response to omalizumab varies from one child to another.

RSV-hRV co-infection is a risk factor for recurrent bronchial obstruction and early sensitization 3 years after bronchiolitis.

To assess risk factors of recurrent bronchial obstruction and allergic sensitization 3 years after an episode of acute bronchiolitis, whether after ambulatory care treatment or hospitalization. A monocentric prospective longitudinal study including infants aged under 1 year with acute bronchiolitis was performed, with clinical (severity score), biological (serum Krebs von den Lungen 6 antigen), and viral (14 virus by naso-pharyngeal suction detection) assessments. Follow-up included a quaterly telephone interview, and a final clinical examination at 3 years.

International Adoption of Children Surviving the Haitian Earthquake.

Objective: To evaluate resilience and frequency of behavioral symptoms in Haitian children internationally adopted before and after the earthquake of January 12, 2010.

Methods: We conducted a retrospective quantitative study in 40 Haitian children. Families were also asked to participate in a qualitative study (individual interview at 18-24 months after the earthquake) and to complete State-Trait Anxiety Inventory (STAI) and STAI for children (STAI-C) questionnaires.

Respiratory Morbidity in Infants Born With a Congenital Lung Malformation.

Background And Objectives: The actual frequency of respiratory symptoms related to congenital pulmonary malformations (CPMs) remains undetermined. The goal of this study was to prospectively evaluate the respiratory symptoms occurring in infants with prenatally diagnosed CPMs, identify factors associated with the occurrence of these symptoms, and evaluate their resolution after surgery.

Methods: Infectious and noninfectious respiratory symptoms were prospectively collected in a French multicenter cohort of children with CPMs.

Functional Benefits of Bilateral Emphysema Reduction Surgery in an Adolescent.

Acquired emphysema is a rare pathology in pediatrics. We report the case of a patient born at term with a neonatal respiratory distress, which had required mechanical ventilation. She developed gradually chronic obstructive pulmonary disease with severe emphysematous lesions, respiratory failure and functional impairments.

Skin diseases in internationally adopted children.

Internationally adopted children often present diseases contracted in the country of origin. Skin diseases are common in new arrivals, and diagnosis may prove challenging for GPs or even dermatologists if they are inexperienced in the extensive geographic and ethnic diversity of international adoptees. To analyse the frequency and characteristics of skin diseases in international adoptees.

An age-wise comparison of human airway smooth muscle proliferative capacity.

We compared the proliferation of neonatal and adult airway smooth muscle cells (ASMC) with no/moderate lung disease, in glucose- (energy production by glycolysis) or glucose-free medium (ATP production from mitochondrial oxidative phosphorylations only), in response to 10% fetal calf serum (FCS) and PDGF-AA. In the presence of glucose, cell counts were significantly greater in neonatal vs. adult ASMC.

Metabolic disturbances of the vitamin A pathway in human diaphragmatic hernia.

Congenital diaphragmatic hernia (CDH) is a common life-threatening congenital anomaly resulting in high rates of perinatal death and neonatal respiratory distress. Some of the nonisolated forms are related to single-gene mutations or genomic rearrangements, but the genetics of the isolated forms (60% of cases) still remains a challenging issue. Retinoid signaling (RA) is critical for both diaphragm and lung development, and it has been hypothesized that subtle disruptions of this pathway could contribute to isolated CDH etiology.

Neonatal outcomes of prenatally diagnosed congenital pulmonary malformations.

Background And Objective: Congenital pulmonary malformations (CPM) are mostly recognized on prenatal ultrasound scans. In a minority of cases, they may impair breathing at birth. The factors predictive of neonatal respiratory distress are not well defined, but an understanding of these factors is essential for decisions concerning the need for the delivery to take place in a tertiary care center.

Impact of a short early therapeutic education program on the quality of life of asthmatic children and their families.

Objectives: Few studies have been made on the impact of therapeutic education (TE) on the quality of life (QOL) of asthmatic primary-school aged children. We attempted to assess the beneficial effects on the QOL of children and their parents of a short TE program initiated immediately after the first consultation with a pediatric pulmonologist.

Methods: The QOL of 31 families of asthmatic children (aged 5-11) was measured before and 3 months after a short and early programme of TE by the French version of the Pictured Child's Quality of Life Self Questionnaire (AUQUEI, AUtoquestionnaire QUalité de vie Enfant Imagé) for the children, and by the Paediatric Asthma Caregiver's Quality of Life Questionnaire (PACQLQ) for the parents.

Predicting the severity of acute bronchiolitis in infants: should we use a clinical score or a biomarker?

Krebs von den Lungen 6 antigen (KL-6) has been shown to be a useful biomarker of the severity of Respiratory syncytial virus bronchiolitis. To assess the correlation between the clinical severity of acute bronchiolitis, serum KL-6, and the causative viruses, 222 infants with acute bronchiolitis presenting at the Pediatric Emergency Department of Estaing University Hospital, Clermont-Ferrand, France, were prospectively enrolled from October 2011 to May 2012. Disease severity was assessed with a score calculated from oxygen saturation, respiratory rate, and respiratory effort.

Improvement of the management of infants, children and adults with a molecular diagnosis of Enterovirus meningitis during two observational study periods.

Enteroviruses (EVs) are a major cause of aseptic meningitis, and RNA detection using molecular assay is the gold standard diagnostic test. The aim of this study was to assess the impact of an EV positive diagnosis on the clinical management of patients admitted for meningitis over the course of two observational study periods (2005 and 2008-09) in the same clinical departments. We further investigated in multivariate analysis various factors possibly associated with hospital length of stay (LOS) in all age groups (infants, children, and adults).

Flexible bronchoscopic cannulation of an isolated H-type tracheoesophageal fistula in a newborn.

Congenital isolated H-type tracheoesophageal fistula (H-TEF) is a rare malformation of the airways. Surgery should not be delayed once the diagnosis is established. Identification of the fistula during surgery is a prerequisite for a successful outcome.

Serum S100B determination in the management of pediatric mild traumatic brain injury.

Background: The place of serum S100B measurement in mild traumatic brain injury (mTBI) management is still controversial. Our prospective study aimed to evaluate its utility in the largest child cohort described to date.

Methods: Children younger than 16 years presenting at a pediatric emergency department within 3 h after TBI were enrolled prospectively for blood sampling to determine serum S100B concentrations.

Health care support issues for internationally adopted children: a qualitative approach to the needs and expectations of families.

Background: Families of internationally adopted children may face specific problems with which general practitioners (GPs) may not be familiar. The aim of the study was to explore problems faced by families before, during and after the arrival of their internationally adopted child and to assess the usefulness of a specific medical structure for internationally adopted children, which could be a resource for the GP.

Methodology/principal Findings: We conducted a qualitative study using individual semistructured guided conversations and interviewed 21 families that had adopted a total of 26 children internationally in the Puy de Dome department, France, in 2003.

Prospective genotyping of human rhinoviruses in children and adults during the winter of 2009-2010.

Background: About 100 serotypes of human rhinovirus (HRV), classified into two species, have been identified by 1990. Uncultivable HRV variants have recently been identified and designated a new species. Recent improved diagnosis has led to a re-appraisal of the clinical impact of HRV infections in lower respiratory diseases.

A simple tool to identify infants at high risk of mild to severe childhood asthma: the persistent asthma predictive score.

Background: Recurrent wheezing in infants is a recognized risk factor for the development of childhood asthma. We sought to develop an easy-to-use persistent asthma predictive score (PAPS) in a population of young recurrent wheezers.

Methods: We retrospectively studied clinical and biological data of infants under 2 years of age presenting recurrent wheezing and evaluated current asthma at 6 years of age using the International Study of Asthma and Allergies in Childhood (ISAAC) questionnaire.