Recurrent Respiratory Symptoms Research Articles

État de santé, suivi et qualité de vie à moyen terme d’enfants opérés d’une atrésie de l’œsophage de type III

The aim of this study was to evaluate the medium-term outcome (health status, medical and surgical French National Health Authority-recommended follow-up, and quality of life) of children born with type III esophageal atresia (EA). Previous events (during the perinatal period, associated abnormalities, respiratory and digestive complications) of children treated for type III EA at the Marseille university hospitals between 1999 and 2009 were noted. Parents completed a standardized questionnaire concerning the health of their children during the previous year, and a quality-of-life questionnaire (PedsQL 4.0) was also completed by children aged more than 8 years. Among the 68 children treated, 44 responded to our solicitation (mean age, 7.6 years; range, 3-12.8 years). Previous important events were : pneumonia(s) (65%), asthma before the age of 3 years (66%), hospitalization for a respiratory event (45%), fundoplication (20%), and esophageal dilatation (45%). We noted current chronic cough (16%), asthma (30%), dysphagia (39%), and symptomatic gastroesophageal reflux (9%). National guidelines were not respected, except for the surgical indications in children aged less than 6 years. The quality-of-life scores (n=43 children) were similar to healthy controls but were negatively influenced by a gastrostomy procedure (P=0.020), pneumonia (P=0.013), and hospitalization due to a respiratory event (P=0.006) or a digestive event (P=0.010), and also by current asthma (P=0.004). In conclusion, despite recurrent respiratory or digestive symptoms and inadequate recommended follow-up, the quality of life of children treated for type III of EA is good.

Long-term respiratory consequences of premature birth at less than 32 weeks of gestation

Chronic respiratory morbidity is a common adverse outcome of very premature birth, particularly in infants who had developed bronchopulmonary dysplasia (BPD). Prematurely born infants who had BPD may require supplementary oxygen at home for many months and affected infants have increased healthcare utilisation until school age. Chest radiograph abnormalities are common; computed tomography of the chest gives predictive information in children with ongoing respiratory problems. Readmission to hospital is common, particularly for those who have BPD and suffer respiratory syncytial virus lower respiratory infections (RSV LRTIs). Recurrent respiratory symptoms requiring treatment are common and are associated with evidence of airways obstruction and gas trapping. Pulmonary function improves with increasing age, but children with BPD may have ongoing airflow limitation. Lung function abnormalities may be more severe in those who had RSV LRTIs, although this may partly be explained by worse premorbid lung function. Worryingly, lung function may deteriorate during the first year. Longitudinal studies are required to determine if there is catch up growth.

Common Variable Immunodeficiency and Type 1 Diabetes Mellitusin a 19-year-old Filipino Male with Alopecia Universalis

A 19-year-old Filipino male presents with recurrent respiratory tract symptoms every 4 to 5 months since childhood and sudden onset of diabetes type 1 with ketoacidosis. He had patchy hair loss which started at 7 years old, and was diagnosed with alopecia universalis. Thyroid antibodies and 21-hydroxylase antibody were normal. Common Variable Immunodeficiency with Polyglandular Autoimmune Syndrome IV was considered. The patient is on monthly intravenous immunoglobulin therapy and basal bolus insulin regimen.

Complete vascular ring leading to long-term tracheal compression in a young adult

Vascular ring is usually an isolated anomaly of embryonic development leading to compression of the encircled trachea and/or esophagus. Double aortic arch is the most commonly encountered form of vascular ring. Vascular rings may either be symptomatic shortly after birth or less commonly, they may persist with mild symptoms until they are diagnosed later in life. In this report, we present a case of double aortic arch forming a complete VR and tracheal compression in a young adult with long term respiratory symptoms. 14 years old female patient had been followed up with recurrent respiratory tract infections since her infancy period. The patient was evaluated by computed tomography and a complete vascular ring due to double aortic arch was demonstrated. The patient was operated; right arcus aorta was divided and tracheal compression was released. Postoperative period was uneventful and symptoms of tracheal compression were dramatically reduced in intensity. Vascular rings must be considered in patients with recurrent respiratory symptoms, since early diagnosis and treatment are important to prevent the long term sequelae of tracheobronchial compres- sion.

Unexplained Recurrent Upper and Lower Respiratory Tract Infections in Otherwise Young Healthy Male

SESSION TYPE: Airway Global Case Report Posters

Strategies for Molecular Classification of Asthma Using Bipartite Network Analysis of Cytokine Expression

Asthma is a chronic inflammatory disease of the airways that leads to various degrees of recurrent respiratory symptoms affecting patients globally. Specific subgroups of asthma patients have severe disease leading to increased healthcare costs and socioeconomic burden. Despite the overwhelming prevalence of the asthma, there are limitations in predicting response to therapy and identifying patients who are at increased risk of morbidity. This syndrome presents with common clinical signs and symptoms; however, awareness of subgroups of asthma patients with distinct characteristics has surfaced in recent years. Investigators attempt to describe the phenotypes of asthma to ultimately assist with diagnostic and therapeutic applications. Approaches to asthma phenotyping are multifold; however, it can be partitioned into 2 essential groups, clinical phenotyping and molecular phenotyping. Innovative techniques such as bipartite network analysis and visual analytics introduce a new dimension of data analysis to identify underlying mechanistic pathways.

Factors associated with elevated exhaled nitric oxide fraction in infants with recurrent respiratory symptoms

Exhaled nitric oxide fraction (F(eNO)) has been proposed as a noninvasive marker of eosinophilic bronchial inflammation in active asthma, and supposed to reflect responsiveness to corticosteroid therapy. There are several factors influencing F(eNO), and its role in early childhood respiratory disorders needs to be established. Between 2004 and 2008, 444 children aged <3 yrs with recurrent lower respiratory tract symptoms were referred to a tertiary centre for further investigation. 136 full-term, steroid-free, infection-free infants, median age of 16.4 months (range 4.0-26.7 months), successfully underwent measurement of F(eNO), lung function tests, and a dosimetric methacholine challenge test. The median level of F(eNO) was 19.3 ppb (interquartile range 12.3-26.9 ppb). Elevated F(eNO) (≥ 27 ppb, the highest quartile) was associated with maternal history of asthma (adjusted OR 3.2, 95% CI 1.3-8.1; p=0.012), and increased airway responsiveness (the provocative dose of methacholine causing a 40% fall in maximal expiratory flow at functional residual capacity ≤ 0.30 mg) (adjusted OR 4.1, 95% CI 1.4-12.7; p=0.012). Atopy, blood eosinophilia and lung function were not associated with elevated F(eNO). In conclusion, maternal history of asthma, and increased airway responsiveness are associated with elevated F(eNO) in infants with recurrent lower respiratory tract symptoms.

Long-term respiratory outcome of babies born prematurely

Chronic respiratory morbidity is a common adverse outcome of premature birth, particularly in infants who develop bronchopulmonary dysplasia (BPD). Prematurely born infants who had BPD may require supplementary oxygen at home for many months, but few remain oxygen dependent beyond 2 years of age. Readmission to hospital is common, particularly for those who had BPD, but only in the first 2 years after birth. The readmissions are usually for respiratory problems, particularly respiratory syncytial virus lower respiratory infections. Recurrent respiratory symptoms requiring treatment are common, particularly in those who had BPD, even at school age and in adolescence. Affected children have evidence of airways obstruction. Pulmonary function does improve with age, but children with BPD may have ongoing airflow limitation. Computed tomography of the chest gives helpful information at follow up of patients with ongoing respiratory problems who had BPD.

Clinical utility of esomeprazole for treatment of gastroesophageal reflux disease in pediatric and adolescent patients

Gastroesophageal reflux is a common condition in the pediatric population, with an increasing incidence in the last few years. It can be defined as an effortless retrograde movement of gastric contents into the esophagus related to complex multifactorial pathogenesis, involving anatomical, hormonal, environmental, and genetic factors. In some cases, it may be associated with esophageal or extraesophageal symptoms (heartburn and regurgitation), and is defined as gastroesophageal reflux disease (GERD). The therapeutic approach to gastroesophageal reflux in infants and children is often conservative, including changes in lifestyle (eg, posture and thickening of meals). If these children remain symptomatic after lifestyle changes (nutrition, feeding, and positional modification), or present with clinical red flags (poor weight gain, recurrent respiratory symptoms, or hematemesis) and complications of GERD (esophagitis, bleeding, stricture, Barrett’s esophagus, or adenocarcinoma) it may be necessary to set up a proper diagnostic protocol. Proton pump inhibitors have been recommended as the most effective acid suppression therapy for adults and pediatric patients. Esomeprazole, the S-isomer of omeprazole, is the only single-isomer proton pump inhibitor available. The paper assesses the safety and tolerability of esomeprazole in pediatric and adolescent patients.

548 Immunoglobulin a Deficiency, HPV and Oral Cancer

BackgroundSelective IgA deficiency is the most common primary immunodeficiency. Serum IgA level lower than 7 mg/dL is considered selective IgA deficiency. Most people with selective IgA deficiency are asymptomatic, with incidental findings. Others may present recurrent respiratory infections, allergic symptoms, other infections and autoimmune diseases. It represents a genetically heterogeneous group of abnormalities. We report 2 cases of IgA-deficiency, HPV, and cancer, which required oral mucosa and tongue surgery.MethodsCase I: Female patient, 30 years old. Medical history: vaginal HPV and Herpes. No promiscuous conduct. Complaint: recurrent infections. Physical exam: oral white lesions are observed. Laboratory findings: serum immunoglobulin A: lower than 7 mg%, secretory immunoglobulin A: lower than 1 mg%. Both exams were repeated and determinations showed low values. Cytology - Glucose - serum protein electrophoresis - Ig G - Ig M - CD3 - CD4 - CD8 - CD19 - CD56 all determinations showed normal values. HIV I /II: negative. Biopsy of oral mucosa with the following report: severe dysplasia and intraepithelial carcinoma. Signs of HPV. Surgery was performed on oral mucosa with the following pathology report: moderately differentiated squamous cell carcinoma. Microscopic, morphological changes related to cytopathogenic viral effects. The patient presented good evolution. Case II: Female patient 40 years of age. Medical history: HPV and genital herpes. No promiscuous conduct. Complaint: leukoplakia in tongue edges. Physical examination: oral white lesions. Laboratory serum immunoglobulin A: value obtained: lower than 7 mg%. Cytology - Glucose - serum protein electrophoresis - Ig G - Ig M - CD3 - CD4 - CD8 - CD19 - CD56 with normal values. HIV I/II: negative. Surgery was performed in tongue and regional lymph node. Tongue Pathology: moderately differentiated squamous cell carcinoma with negative edges. HPV (+) PCR.ConclusionWe report on the possible association between selective IgA deficiency - HPV - Cancer which has not been previously reported in time. We suggest further screening of these possible associations and detailed monitoring of these patients.

Pulmonary Sequestration and Diaphragmatic Eventration in a 6-month-old Infant

A congenital pulmonary malformation, such as pulmonary sequestration or congenital cystic adenomatoid malformation, should be suspected in infants with recurrent lower respiratory symptoms or unifocal infiltrations. The possibility of congenital pulmonary malformation associated with additional abnormalities, such as diaphragmatic hernia, is relatively high and can lead to misdiagnosis. We report a case of a 6-month-old girl who presented with relapsing respiratory infection and hematemesis. Computerized axial tomography scan and barium swallowing study were performed, revealing a suspected intralobar pulmonary sequestration associated with sliding gastric hernia. Since the patient's condition was complicated by sliding hernia, pneumonia and pleural effusion, a surgical procedure instead of cardiac catheterization with coil embolization was performed. During surgery, the absence of a sliding gastric hernia but a diaphragmatic eventration was noted. Only a partial portion of the left-side diaphragm was relaxing, making the barium swallowing study difficult to interpret. This case serves as a reminder that if pulmonary sequestration is suspected, a full work-up with a complete set of imaging studies should be ordered for the possible detection of associated gastrointestinal, respiratory and thoracic abnormalities.

Transhiatal esophagectomy with gastric transposition for esophageal replacement in post-corrosive stricture in children

Despite potential advantages of gastric transposition there are some concerns about this surgical technique of esophageal substitution in children. In the current study the morbidity and functional outcome are surveyed in a series of patients undergoing gastric transposition due to extensive post-corrosive esophageal strictures. Retrospective analysis of children proposed for esophageal replacement from September 2003 through April 2007 after endoscopic dilations failure. Demographic and pathological features, intra and postoperative complications and clinical outcome were assessed. Continuous variables are expressed as median [range]. There were six children (age: 4.3 [2.5;14.4] years) with esophageal structures secondary to corrosive alkali ingestion; five had been submitted to dilation (n = 8 [7;27]) and one had undergone gastrostomy. Esophagectomy without thoracotomy plus gastric transposition were uneventfully performed in all cases. There was no mortality. On postoperative course there was one hypertensive pneumothorax and one pneumonia. With a follow-up of 50 [38;80] months, two children experienced mild dysphagia that was promptly solved by one or two sessions of dilations of esophagogastric anastomosis; both weight and height were between 5th and 75th centiles; none presented gastrointestinal or recurrent respiratory symptoms, anemia or substitute dilation. CONCLUSION. Transhiatal esophagectomy with gastric transposition in posterior mediastinum presented low morbidity and excellent functional outcome with no impairment of growth or respiratory function, at least on short/medium term.

Skin Prick Testing to Aeroallergen Extracts: What Is the Optimal Panel in Children and Adolescents in Turkey?

Background/Objective: The skin prick test (SPT) is the standard tool for the diagnosis of IgE-mediated allergic diseases. The number and spectrum of allergen extracts to be applied for the diagnosis of sensitized patients in order to achieve the most cost-effective and informative results are critical from both economic and scientific viewpoints. Methods: In order to determine the minimum test battery panel necessary to cover at least 95% of the cases of SPT sensitization in children and adolescents (2–18 years) with suspected respiratory allergies, we retrospectively analyzed the SPT results of the largest referral center of the country. Results: Over a 2.5-year period, a total of 2,457 children (male/female ratio 1.51) with a median (range) age of 6.8 years (2.0–18.0) were subjected to SPT. Eight hundred and sixty-two (35.1%) children were found to be sensitized to at least one of the 30 aeroallergen extracts tested. The most common sensitizations were to grasses (Festuca pratensis, Phleum pratense, Dactylis glomerata, and Lolium perenne), house dust mites (Dermatophagoides pteronyssinus and Dermatophagoides farinae), cat, weeds (Chenopodium, Plantago, and Artemisia), and moulds (Alternaria and Cladosporium). The sensitization rates increased in conjunction with an increase in age. Testing with 12, 8, and 7 allergens was sufficient to identify over 95% of the sensitized preschool children, school children, and adolescents, respectively. Conclusion: An SPT panel covering 12 allergen extracts was sufficient to detect most of the sensitized children and adolescents with recurrent respiratory symptoms. As the patients grow older, a smaller test panel is required compared to the panels used at younger ages.

Exercise Induced Changes of Gastroesophageal Acidity in Children

Background: Gastroesophageal reflux (GER) is often related to recurrent respiratory symptoms. Whether GER accounty for exercise-induced bronchoconstriction remains underdebate.

Respiratory protective equipment, mask use, and respiratory outcomes among World Trade Center rescue and recovery workers

Serious respiratory illnesses have been reported among rescue/recovery workers (RRW) following the World Trade Center (WTC) attacks. We studied RRW enrolled in the WTC Health Registry to assess the effects of different respiratory protection equipment (RPE) types on respiratory outcomes, such as recurrent respiratory symptoms and diseases possibly associated with 9/11 exposures. We performed descriptive and multivariate analyses adjusting for demographics and exposure variables. A total of 9,296 RRW met inclusion criteria. The strongest predictors of using adequate RPE were being affiliated with construction, utilities or environmental remediation organizations and having received RPE training. Workers who used respirators were less likely to report adverse respiratory outcomes compared to those who reported no/lower levels of respiratory protection. Level of respiratory protection was associated with the odds of reporting respiratory symptoms and diseases. Training, selection, fit testing, and consistent use of RPE should be emphasized among emergency responders.

Aortoarteritis and cardiomyopathy in a child with Blau Syndrome

Description MG, now a 10 year old female, was seen with a history of recurrent fevers since 1 month of life with bilateral knee effusions noted at 1.5 years of age, in the year 2002. This was initially treated as ANA negative oligoarticular JIA, requiring intra-articular steroid injections twice. She has never had a history of skin manifestations or uveitis. Over time, the knees showed increasing bogginess without restriction of activity. Six years after being lost to follow up, in mid 2008, at the age of 8 years, she presented with features of cardiac failure, arrhythmias (ventricular premature beats) and arthritis of the elbow joint. Investigations showed normal blood counts, normal eye examination and raised ESR. The 2D Echo showed dilated cardiomyopathy with a 20% ejection fraction with an abnormal myocardial echogenicity. Recurrent fevers since early childhood with arthritis and cardiomyopathy lead to a suspicion of an autoinflammatory syndrome. Further investigations showed a normal serum ACE and the DNA analysis showed a punctual mutation at G464W in exon 4 of the CARD15 gene. Liver and cardiac biopsies were deferred due to poor cardiac function. An identical genetic mutation was found in the mother who had a history of recurrent fevers, abdominal pain and respiratory symptoms that had never been investigated. Since then, MG has been on tapering oral steroid (started at 1mg/kg/ day), injectable Methotrexate (1517.5 mg/m) and anti-failure line of therapy. Initially, her symptoms improved with only a marginal improvement in the cardiac function. In early 2009, mild hypertension was documented and in September 2009, carotid pulsations were found reduced. By mid 2010, she had severe hypertension (230/120 mmHg), uncontrolled on multiple antihypertensives, with renal and carotid bruits. Digital Subtraction Angiography showed generalized narrowing of the aorta, carotids, left subclavian artery and bilateral renal arteries, suggestive of an aortoarteritis. The cerebral circulation was maintained normally with diffuse collaterals. The ultrasound abdomen showed a contracted left kidney with the right kidney perfusion maintained with an accessory artery with multiple renal hypo-echogenecities. The renal function and calcium excretion were normal with negative ANCAs. Three years into follow-up after diagnosis of aortoarteritis, on continuing therapy, she remains asymptomatic with an improvement in her cardiac function to an ejection fraction of 55%. Intervention such as stenting of the stenosed major vessels has currently been deferred due to the widespread nature of the aortoarteritis being aided with diffuse collateral circulation maintaining normal function.

Gastroesophageal pH monitoring, exercise and atopic inflammation in children with recurrent respiratory symptoms

A double-blind, randomized, parallel-group pilot study compared the relative efficacy of hydrofluoroalkane-134a beclomethasone dipropionate (HFA-BDP [QVAR]; mass median aerodynamic diameter, 0.8-1.2 m) versus cholorofluorocarbon-11/12 BDP (CFC-BDP [Beclovent]; mass median aerodynamic diameter, 3.5-4.0 m) in 31 steroid naive patients with mild to moderate asthma (PC20, 4 mg/mL). Functional high-resolution computed tomography was used to assess the relative efficacy of HFA-BDP and CFC-BDP on regional air trapping, as an indirect measure of small airways function and on regional hyperreactivity. Pretreatment functional computed tomography was performed at residual volume before and after methacholine challenge. After 4 weeks of treatment, functional imaging was repeated before and after the same concentration of methacholine that was administered before the treatment (n = 19 patients). Quantitative assessment of changes in distribution of lung attenuation was performed. After 4 weeks of treatment, the HFA-BDP group showed significantly more improvement in air trapping overall (a shift in the lung attenuation curve at residual volume toward more attenuation) on the posttreatment computed tomography scan (P < .05; Fisher’s Exact Test). After an equal constrictor stimulus (methacholine concentration), subjects treated with HFA-BDP (n = 10 patients) showed less increase in air trapping overall than subjects treated with CFC-BDP (n = 9 patients) on the posttreatment scans compared with the pretreatment scans (P < .001; Fisher’s Exact Test). No significant difference was demonstrated between the 2 treatment groups with respect to improvement in symptoms, spirometry, or methacholine responsiveness assessed by FEV1, except for a greater reduction in breathlessness in the HFA-BDP group (P < .05). We conclude that HFA-BDP may have greater efficacy in the peripheral airways and that this effect is better assessed with functional imaging computed tomography techniques than with conventional physiologic tests. (J Allergy Clin Immunol 1999;104:S258-67.)

388 Detection of a novel CFTR mutation c.122C>G (p.Pro41Arg) in a young cystic fibrosis (CF) patient with recurrent respiratory symptoms

Background: At present more than 1800 mutations have been described in the CFTR-gene. Several rare mutations are believed to cause mild lung disease and late onset of symptoms. We present a 5 y old Belgian boy heterozygous for an atypical CFTR variant and a novel variant which was never described before. Case report: A 5 y old boy with recurrent bronchitis and chronic sinusitis was referred to our clinic with repeated abnormal sweat chloride (Cl) (right 70mEq/L; left 53mEq/L) and elevated sweat sodium values (right 86mEq/L; left 62mEq/L), performed by Gibson and Cooke gauze method. Chest CT scan showed bronchial wall thickening and mucous plugging, without bronchiectasis. He is pancreatic sufficient, without history of pancreatitis. By screening the coding region of CFTR, using sequencing and MLPA (Multiplex Ligation-dependent Probe Amplification), he was shown to be heterozygous for c.224G>A (p.Arg75Gln − legacy name: R75Q) and c.122C>G (p.Pro41Arg), a novel variant which was never described before. Analysis of the parents is ongoing to prove that both variants are on different alleles. Discussion: R75Q is described as a variant overrepresented in patients with atypical CF, sarcoidosis, COPD and chronic pancreatitis and rather influences bicarbonate conductance than Cl. Our young patient has already respiratory symptoms and abnormal sweat chloride values. To our knowledge CFTR p.Pro41Arg has never been described before and its clinical relevance is not clear. As this boy has signs and symptoms of CF disease in combination with an atypical CFTR mutation, this novel mutation might be involved. However, mutations outside the coding region cannot be excluded.

Does RSV Infection Alter the Course of Chronic Lung Disease in Prematurely Born Infants?

Very premature birth frequently results in chronic respiratory morbidity, particularly if complicated by the development of chronic lung disease, bronchopulmonary dysplasia (BPD). Affected infants may remain oxygen dependent for many months and require hospital readmission in the first two years after birth. Troublesome, recurrent respiratory symptoms requiring treatment are common. The worst affected may remain symptomatic with evidence of airways obstruction even as adults. Infant who are born prematurely and develop BPD are at increased risk of severe respiratory syncytial virus (RSV) infection. RSV infection in prematurely born infants is associated with excess chronic respiratory morbidity, as evidenced by greater frequency of respiratory symptoms, lung function abnormalities and healthcare utilisation. There is some evidence to suggest that prematurely born infants, as those born at term, may be predisposed to symptomatic RSV lower respiratory tract infections by pre-morbid abnormal lung function. Longitudinal studies assessing lung function before and after RSV infection are required to determine whether RSV infection does alter the course of chronic lung disease in prematurely born infants.

Congenital tracheobiliary fistula: a case report with review of literature

Congenital tracheo-or-bronchobiliary fistula is a rare anomaly. Here, we present a malnourished child with persistent and recurrent respiratory symptoms. Flexible bronchoscopy and imaging studies were carried out to ascertain the diagnosis, anatomy and associated anomalies prior to surgery. Patient underwent successful corrective surgery with complete resolution of symptoms.