Remodelling and inflammation in preschoolers with severe recurrent wheeze and asthma outcome at school age.

BACKGROUND: The influence of airway remodelling and inflammation in preschoolers with severe recurrent wheeze on asthma outcomes is poorly understood. OBJECTIVE: To assess their association with asthma symptoms and lung function at school age. METHODS: Preschoolers (38.4 months) initially investigated with bronchial biopsies were re-assessed for asthma symptoms and lung function at school age. RESULTS: Thirty-six of 49 preschoolers (73.5%) were assessed at 10.9 years. Twenty-six (72.2%) had persistent asthma. Submucosal eosinophil counts were higher in children with severe exacerbations at school age than in those without (16/0.1 mm2 [11.2-30.4] vs 8/0.1 mm2 [2.4-17.6], P = .02), and correlated with the number of severe exacerbations (P = .04, r = .35). Submucosal neutrophil counts correlated with FEV1/FVC (P < .01, r = .47) and FEF25-75% predicted (P = .02, r = .43). Airway smooth muscle (ASM) area correlated with FEV1/FVC (P < .01, r = .51). Vessel numbers negatively correlated with FEV1% predicted and FEV1/FVC (P = .03, r = -.42; P = .04, r = -.41; respectively) and FEF25-75% predicted (P = .02, r = -.46). CONCLUSION: Eosinophilic inflammation in preschoolers with severe recurrent wheeze might be predictive of future severe exacerbations, neutrophilia might be associated with better lung function. Changes in ASM and vascularity might affect lung function at school age.

[Non invasive ventilation and pediatric palliative care. A French survey]. / Ventilation non invasive et soins palliatifs en pédiatrie. État des lieux en France.

OBJECTIVES: The benefits of non-invasive ventilation (NIV) have been clearly demonstrated in pediatrics. In palliative care, NIV can improve the level of comfort and quality of life and can decrease dyspnea. The objective was to survey pediatricians' opinions and practices regarding NIV in palliative care in France. DESIGN: A mail survey was conducted among pediatric pneumologists, intensivists and palliative medicine consultants from February 2015 to March 2015. RESULTS: In case of acute respiratory failure, 84% of the responding practitioners found NIV appropriate in do-not-intubate (DNI) children, while only 35% of them found it appropriate in comfort-measures-only (CMO) children (P<0.0001). In case of progressive respiratory failure, 68% of the responders found NIV appropriate in DNI children, while only 30% in CMO children (P<0.05). The major criterion for initiating NIV in pediatric palliative care was the presence of dyspnea. In pediatric palliative care, the efficacy of NIV was evaluated primarily clinically in terms of the improvement of the child's comfort level, as well as the child's and family's satisfaction. Hypercapnia and desaturation were rarely measured to initiate NIV or to assess its efficacy. Sixty percent of the responding practitioners indicated that referral to NIV was anticipated with children and family before acute events or end-of-life occurred. CONCLUSION: French pediatricians habitually use NIV for management of acute or progressive respiratory symptoms in DNI children. In CMO children, a majority of responding practitioners find NIV inappropriate. In palliative care, the indications for and efficacy of NIV are evaluated based on clinical criteria and rarely on gasometric criteria.

[Yellow nail syndrome: two pediatric case reports]. / Syndrome des ongles jaunes : deux cas pédiatriques.

BACKGROUND: Yellow nail syndrome (YNS) is a combination of specific nail dystrophy (yellowish or green nails, transverse ridging, increased curvature with "a hump" and distal onycholysis), lymphoedema and respiratory tract involvement (mainly bronchial hyper-responsiveness, recurrent pneumonia, bronchiectasis and pleural effusion), with or without sinusitis. This rare condition is most often seen in middle age. We report two pediatric cases. PATIENTS: A 9-year-old boy had 20 yellow nails and lymphedema of the lower and upper left limbs. He was born at 32 weeks of gestation. At birth, he had fetal hydrops with bilateral chylothorax. Since then, he presented bronchopulmonary dysplasia. In his first year of life, he had recurrent nasopharyngitis and serosal otitis. Lymphoscintigraphy was not performed. A 14-year-old boy had 10 yellow toenails and 2 yellow fingernails. He was born at 36 weeks of gestation with fetal hydrops. He had recurrent serosal otitis and asthma attacks. Lymphoscintigraphy showed lymphatic hypoplasia, especially of the left limbs. DISCUSSION: These two children have the classic triad of YNS, including yellow nails, lymphedema and respiratory tract involvement. Thirteen cases have been reported in children from birth to 10 years of age, three of whom were siblings, thus suggesting genetic predisposition. In most cases lymphoscintigraphy showed hypoplasia and/or ectasia of lymphatic vessels. These lymphatic abnormalities may be responsible for nail changes as seen in Turner's syndrome. The prognosis of YNS in adults may be serious because of the pulmonary tract involvement. However, it is not known in children. CONCLUSION: Yellow dystrophic nails in children may be associated with lymphedema and respiratory tract involvement, as in adults, and this condition should therefore be investigated in cases of YNS.

[Aquagenic palmoplantar keratoderma in children with cystic fibrosis]. / Kératodermie palmoplantaire aquagénique chez l'enfant mucoviscidosique.

INTRODUCTION: Aquagenic palmoplantar keratoderma (APK) is a cutaneous phenomenon marked by the formation of edematous, translucent papules and plaques on the palms after water immersion. It can be observed in healthy subjects, but while this dermatosis is little known by practitioners treating these patients, most cases of APK have been described in patients with cystic fibrosis (CF). The primary objective of this study was to evaluate the frequency of APK in a population of children with CF. In addition, the relationship between APK and sex, genotype, pancreatic and pulmonary function, body mass index, and sweat chloride levels was analyzed. METHODS: This study was conducted in 60 children, 27 girls and 33 boys, aged 4 months to 18 years, followed at the CF care center at Angers (France) University Hospital, in whom CF had been confirmed by a positive sweat chloride level greater than 60 mmol. APK was determined by questioning searching for modifications of the palms noticed by the patient or his/her family after immersion in water and a clinical examination searching for the same signs before and after immersion of the right hand in a bucket of lukewarm water for 3 minutes (bucket sign). RESULTS: Forty-seven out of 60 children (78%) had a positive bucket sign. Thirty-eight upon these 47 children had already noticed modifications of the skin on their palms, appearing quickly during the bath and 6 had an edema and an increase in skin folds on the palms of the hands even before immersion of their hand in water. No genotype-phenotype correlations were detected in patients with APK, nor were there associations of APK with other phenotypic features of CF. CONCLUSION: APK is very frequent in patients with CF. It is most probably a consequence of the dysfunction of the CFTR protein. It should be systematically sought in all patients with CF. Its discovery in another context should suggest the diagnosis of CF or a carriage to the heterozygous state of a mutation involved in the disease.

[Transition from pediatric to adult care: experience of a cystic fibrosis care center]. / Passage de l'enfant à l'adulte: l'exemple d'un centre de ressources et de compétences pour la mucoviscidose.

BACKGROUND AND OBJECTIVE: Even though patients with cystic fibrosis (CF) are continuously improving their life expectancy, guidelines for transition to adult services have not been clearly determined yet. The aim of this study was to analyze the management of this transition in the CF care center of Angers, France. PATIENTS AND METHOD: From their medical files, we analyzed the transfer of 22 patients with CF from pediatric to adult care. The perceptions of patients and caregivers regarding this transition were evaluated using anonymous questionnaires. RESULTS: The initial objective was to transfer patients around 18 years of age, offering them 3 or 4 joint consultations with a pediatrician and adult lung specialist. The median age of transfer was 22 years and the median duration of the transition period was 9 months. Half of the patients had only 1 joint transfer consultation during transition. The patient reaching the age of 18 and maturity were the most common criteria mentioned for transfer. All highlighted problems leaving the pediatric team they had grown attached to and its familiar environment. Caregivers described the transfer as a success, whereas half of the patients were dissatisfied with it, with both patients and caregivers indicating that the transition was too short without enough joint consultations. CONCLUSIONS: This study shows that this period is a major life event for the patient with CF. The transition process must be organized with the patient and independent behaviors should be encouraged. Adult and pediatric teams need to cooperate. Based on this experience and former medical data, we suggest a transition program for patients with CF.

[Food avoidance emotional disorder in 3 to 10-year-old children: a clinical reality]. / Evitement alimentaire et trouble affectif chez l'enfant.

We underline the clinical importance of a specific eating disorder in 3 to 10 years old children, when the majority of the works about the prepubertal eating disorders focus either on the period just preceding adolescence (often between 10 and 13 years), or on the second half of the first year of the baby. Within the eating disorders described in the literature, we compare the clinical presentation of most of these 3 to 10 years old children with the food avoidance emotional disorder described during adolescence. These problems of eating behaviour (various selective eating with or without provoked vomiting) are ignored for a long time in these young children because of quite a satisfactory growth, but these children are often seen in emergency rooms because of a brutally complete eating refusal. Therapeutic consultations allow these children to express their fears about diseases, poisoning and death, for themselves or for their close relations, in particular the mother, without endangering their body. The early recognition and care of these difficulties of conciliation between the body and the thoughts impose a narrow collaboration between paediatric and psychiatric staffs.

[Antineutrophil cytoplasmic antibodies in cystic fibrosis. Frequency and significance]. / Anticorps anticytoplasme des polynucléaires neutrophiles au cours de la mucoviscidose. Fréquence et signification.

UNLABELLED: Some immune system abnormalities have been reported in cystic fibrosis, particularly the presence of antineutrophil cytoplasmic antibodies (ANCAs). The purpose of this study was to determine the frequency of these antibodies in a population of patients with cystic fibrosis and to assess their relationship with the disease progression. POPULATION AND METHODS: This retrospective study looked for the presence of these antibodies in sera from 64 patients (30 boys and 34 girls) aged one to 29 years. All patients were followed up within the Cystic Fibrosis Center of the University Hospital of Angers. The serum samples were collected in 2001 during the yearly check-up to evaluate disease status. RESULTS: Seven of the 64 patients presented the antibodies. Univariate analysis showed that these patients were significantly older and more often chronically infected with Pseudomonas aeruginosa than the other patients. They also scored significantly more poorly on pulmonary radiography and showed significantly higher IgG and CRP. Multivariate analysis revealed that the two following independent factors were associated with the presence of ANCAs: chronic P. aeruginosa infection and high IgG level. CONCLUSION: This work confirms the abnormally high frequency of antineutrophil cytoplasmic antibodies in patients with cystic fibrosis. These antibodies were observed in the sickest patients with severe infection status. They reflect the impact of inflammatory processes in the pathogenesis of the disease. Detection of these antibodies might be an indication for intensified treatment of bronchial infections.

[Effect of oral biphosphonates in patients with cystic fibrosis and low bone mineral density]. / Efficacité des biphosphonates oraux sur la déminéralisation osseuse des patients atteints de mucoviscidose.

UNLABELLED: Disturbances in bone mineralization are frequent in cystic fibrosis but few studies have focused on the use of biphosphonates in this indication, and none on the use of oral etidronate. We report our experience using this latter treatment. METHODS: The study was retrospective and included five children and three adults with cystic fibrosis (six males and two females) aged seven to 30 years with Z-scores for lumbar bone density lower than -2 SD after one year of calcium (1 g/day) and vitamin D (900 UI/day and 300,000 UI/6 months) supplementation. All were treated during one year with etidronate: four courses of 15 days (one course per trimester) with doses ranging from 4 to 8 mg/kg per day. Calcium and vitamin D supplementation was continued between the etidronate treatment course. Total body and lumbar bone mineral density (BMD) were measured three times: at the beginning and the end of the year of calcium and vitamin D supplementation and at the end of the year of supplementation plus the four courses of etidronate treatment. RESULTS: The increase in BMD in absolute value (g/cm2) and in Z-score was significantly higher (P <0.05) after the year of combined supplementation and etidronate treatment (total body g/cm2: 3+/-1%, Z-score: 2+/-1% and lumbar spine g/cm2: 6+/-5%, Z-score: 3+/-4%) than after supplementation alone (total body g/cm2: -1+/-3%, Z-score: -4+/-3% and lumbar spine g/cm2: -1+/-3%, Z-score: -4+/-4%). Supplementation alone improved the total BMD in only one patient and the lumbar BMD in three, whereas after etidronate treatment the total and lumbar BMD were improved in the eight patients. None of the patients presented with side effects that could be attributed to the treatment. CONCLUSION: Oral etidronate treatment is well-tolerated and capable of improving bone mineralization in patients with cystic fibrosis. Further work will be necessary to determine the optimal dosage and the optimal frequency for the treatment series.