Recurrent Lower Respiratory Infections Research Articles

M199 X- LINKED HYPER-IGM SYNDROME WITH CD40LG MUTATION IN A FEMALE PATIENT AND HER MALE SIBLING

We studied a 14-year-old female and 18-year-old male siblings who initially presented to Oklahoma Allergy and Asthma Clinic at the ages of 5 and 9 respectively, following recurrent upper and lower respiratory tract infections. Prick intradermal allergy testing was mildly reactive with no response to allergen immunotherapy previously.

A screening tool to identify risk for bronchiectasis progression in children with cystic fibrosis.

BackgroundThe marked heterogeneity in cystic fibrosis (CF) disease complicates the selection of those most likely to benefit from existing or emergent treatments.ObjectiveWe aimed to predict the progression of bronchiectasis in preschool children with CF.MethodsUsing data collected up to 3 years of age, in the Australian Respiratory Early Surveillance Team for CF cohort study, clinical information, chest computed tomography (CT) scores, and biomarkers from bronchoalveolar lavage were assessed in a multivariable linear regression model as predictors for CT bronchiectasis at age 5–6.ResultsFollow‐up at 5–6 years was available in 171 children. Bronchiectasis prevalence at 5–6 was 134/171 (78%) and median bronchiectasis score was 3 (range 0–12). The internally validated multivariate model retained eight independent predictors accounting for 37% (adjusted R 2) of the variance in bronchiectasis score. The strongest predictors of future bronchiectasis were: pancreatic insufficiency, repeated intravenous treatment courses, recurrent lower respiratory infections in the first 3 years of life, and lower airway inflammation. Dichotomizing the resulting prediction score at a bronchiectasis score of above the median resulted in a diagnostic odds ratio of 13 (95% confidence interval [CI], 6.3–27) with positive and negative predictive values of 80% (95% CI, 72%–86%) and 77% (95% CI, 69%–83%), respectively.ConclusionEarly assessment of bronchiectasis risk in children with CF is feasible with reasonable precision at a group level, which can assist in high‐risk patient selection for interventional trials. The unexplained variability in disease progression at individual patient levels remains high, limiting the use of this model as a clinical prediction tool.

RARE CAUSE OF PROXIMAL BRONCHIECTASIS: MOUNIER-KUHN SYNDROME

TOPIC: Imaging TYPE: Medical Student/Resident Case Reports INTRODUCTION: Mounier-Kuhn syndrome (MKS), also known as tracheobronchomegaly is a rare cause of proximal bronchiectasis which is characterized by deficient smooth muscle and elastic fibers and absence of myenteric plexus in the proximal airway wall. Less than 400 cases of MKS have been reported (1) (2). We present a patient with numerous hospitalizations due to recurrent dyspnea and pneumonia spanning twenty years who met chest CT criteria for MKS. CASE PRESENTATION: 44-year-old African American male, a former smoker, presented with worsening dyspnea and productive cough for 2 days. He has had similar symptoms for 20 years. Examination revealed clubbing of extremities and coarse auscultation crackles bilaterally. Chest x-ray revealed right upper lobe emphysematous changes and bilateral diffuse central opacities with cystic changes. CT chest revealed para-septal emphysema and extensive proximal cystic bronchiectasis bilaterally. Blood tests were negative for HIV, connective tissue disorders, autoimmune disorders and immunodeficiency. The absence of asthma, patient's ethnicity, having biological children and absence of other organ damage, made cystic fibrosis (CF) and allergic bronchopulmonary aspergillosis (ABPA) unlikely. The trachea, left and right mainstem bronchus measured at 30mm, 25mm, 27mm on chest CT with outpouching noted on proximal trachea, which led to the diagnosis of MKS. DISCUSSION: Proximal bronchiectasis is rare. Acquired causes are ABPA, CF, and congenital causes are MKS and William Campbell Syndrome. MKS is a rare congenital abnormality characterized by tracheobronchial dilation due to atrophy of elastic fibers and thinning of smooth muscle layer in the trachea and main bronchi (2) up to 1st to 4th bronchial generations. Clinical manifestations include that of chronic bronchitis and bronchiectasis with recurrent lower respiratory infection. Subtypes include 1) tracheobronchomegaly alone, 2) tracheobronchomegaly with diverticula in the proximal trachea as seen in our patient, 3) tracheobronchomegtly with diverticula extending to lower bronchi. The gold standard diagnostic test for MKS is CT chest. Diagnostic criteria are trachea > 30mm, right main bronchus > 20-24mm, and left main bronchus >18-23mm (3). Airway wall weakness can lead to extensive dynamic airway occlusion further accentuating symptoms. Treatments rely on daily frequent chest physiotherapy with postural drainage, antibiotics during infections, and immunization to help prevent infections and further airway damage. CONCLUSIONS: In patients with bronchiectasis, early diagnosis of tracheobronchomegaly using chest CT criteria will allow for implementation of treatment strategies that will hopefully prevent recurrent infection and further airway damage. REFERENCE #1: Mounier-Kuhn syndrome in a Namibian female patient. Mkandawire MJ, Muramira NM, Mraba N. s.l. : Pan Afr Med J, 2020. REFERENCE #2: Tracheobronchomegaly. The Mounier-Kuhn Syndrome. Katz I, Levine M, Herman P. s.l. : Am Journal Roentgenol Radium Ther Nucl Med. 88:1084-1094. REFERENCE #3: Mounier-Kuhn syndrome: report of 8 cases of tracheobronchomegaly with associated complications. Balakrishnan, Menon, et al. 1, s.l. : Southern Medical Journal, 2008, Vol. 101. pp.83-87. DISCLOSURES: No relevant relationships by Thomas George, source=Web Response No relevant relationships by Padmanabhan Krishnan, source=Web Response No relevant relationships by Nikisha Pandya, source=Web Response No relevant relationships by Gurjit Inder Sidhu, source=Web Response No relevant relationships by Sarita Singh, source=Admin input No relevant relationships by Fardad Zaghi, source=Web Response

Congenital cystic adenomatoid malformation associated with right aortic arch with mirror image branching and absence of left pulmonary artery in an adult

BackgroundCongenital cystic adenomatoid malformation is an uncommon developmental anomaly that is diagnosed prenatally or during early childhood and is rare to present in adulthood. Type 2 congenital cystic adenomatoid malformation is often associated with other anomalies such as congenital cardiac anomalies. We present a case of type 2 congenital cystic adenomatoid malformation in an adult male associated with absence of left pulmonary artery and right aortic arch with mirror image branching. To our knowledge, this is the first case report with the combination of all three anomalies.Case presentationA 33-year-old male presented to the Department of Pulmonary Medicine with high-grade fever, chills and rigor, breathlessness, and productive cough for a duration of one week. He had multiple similar self-limiting episodes in the past which did not require hospitalization. The plain radiograph showed right aortic arch and a small left hilum. CT thorax showed multiple cystic lesions of size 0.5–2.0 cm in the apico-posterior segment of the left upper lobe; anterior, posterior, and lateral segments of the left lower lobe; and the apical segment of the right upper lobe with a maximal cyst wall thickness of 2 mm. Volume of left lung was reduced with trans-thoracic herniation of the medial segment of the right middle lobe; bronchiectatic changes were seen in the apical, anterior, and posterior segments of the right upper lobe; and fibrotic strands were seen in the apical segment of the right lower lobe and the apico-posterior segment of the right upper lobe. Mediastinal window showed right-sided aortic arch with mirror image branching. The pulmonary trunk was seen to continue as the right pulmonary artery with absent left pulmonary artery.ConclusionsThough congenital cystic adenomatoid malformation is a disease of infancy and childhood, it should also be considered in adults presenting with recurrent lower respiratory tract infections and/or pneumothorax. Radiologists must be aware of the types of congenital cystic adenomatous malformation (CCAM) and their associations, as type 2 CCAM has a high association with congenital cardiac disease. Proximal interruption of pulmonary artery should be considered if the pulmonary trunk continues as either right or left pulmonary artery.

Diaphragmatic eventration plication repair with minimal access techniques: a case series

Diaphragmatic eventration is a rare pathological condition occurring in <0.05% individuals causing the diaphragm to permanently ascend into the chest due to partial or complete replacement of diaphragmatic musculature by fibroblastic tissue. Congenital diaphragmatic eventration rarely presents among adults with most patients remaining asymptomatic only to be diagnosed incidentally after chest radiography. Symptomatic patients can present with dyspnoea, orthopnoea, recurrent lower respiratory tract infections or gastrointestinal symptoms. Management varies according to symptoms with asymptomatic cases requiring no intervention and plication being the standard, well-described technique for symptomatic cases. Here we described two cases with eventration approached using different minimal access techniques.

Anaesthetic Management of an Infant with Laryngeomalacia and CHD Scheduled for Rigid Tracheobronchoscopy

Introduction: Laryngomalacia is defined as collapse of supraglottic structures during inspiration. It is the most common laryngeal disease of infancy and cause of stridor in newborns. Case Report: A 17 days old child (2.75kgs) with congential laryngomalacia was scheduled for rigid tracheobronchoscopy. Child had a history of recurrent lower respiratory tract infection which resolved with intravenous antibiotics and steroids. On auscultation he had no adventitious sounds when quiet but added sounds were heard when he cried. Echocardiography showed CHD with situs solitus levocardia with 4mm ASD and 2mm PDA with dilated RA/RV. The child was administered atropine, midazolam, atracurium and ketamine. Maintainence with sevoflurane . During rigid bronchoscopy we provided adequate analgesia and sedation without clinically significant hemodynamic or respiratory adverse effects. Conclusion: In summary we noted that ketamine provided a reliable and effective method of sedating infants undergoing a rigid bronchoscopic examination in spontaneous ventilating conditions and noted no clinically significant haemodynamic or respiratory problems. When these patients do present, their care is likely to be complex and challenging. As such , a multidisciplinary approach should be adopted throughout their period of care.

Clinical, Microbiological and Radiological Characterises of Lower Respiratory tract Infection in Children with Established Congenital Heart Diseases.

Introduction: Acute Lower Respiratory Tract Infection (ALRTI) is the most common cause of deathin children under five. Various demographic, socio-economic and environmental factors have beenassociated with ALRTI, with conflicting results. Children with congenital heart diseases are morevulnerable to lower respiratory tract infections following the fact that they had an anatomical defectthat causes hemodynamic disturbance of lung circulation and mucosal oedema finally leads todecreased lung compliance and recurrent lower respiratory tract infections. Methods: Children agedbetween1 month and 18 years with clinically established congenital heart disease confirmed byechocardiography and presenting with signs and symptoms suggestive of lower respiratory tractinfection were enrolled in the study. A detailed history was taken and routine blood investigationswere done in all cases. Chest X-Ray was done to confirm the clinical diagnosis. Results: Mean ageof the study subjects was 2 years. VSD was seen in 64.9% of cases while 10.5% of cases had VSDand ASD. The most common presenting symptoms were: cough (98.2%), reduced feeding (97.4%),fever (89.5%), running nose (67.5%) and wheeze (49.1%). Leucocytosis and raised ESR were seenin 66.7% and 64.9% cases respectively. In Throat swab Commensals were seen in 36% of caseswhile gram-positive cocci were seen in 3.5% cases respectively. In blood culture Streptococcuspneumoniae being the commonest organism isolated. Bilateral and unilateral opacities on chest x-ray were seen in 16.7% and 12.3% cases respectively. Conclusion: Bronchopneumonia was thecommonest LRTI in children with the predominance of VSD.

Congenital H-type tracheo-oesophageal fistula: An institutional review of a 10-year period.

BackgroundCongenital H-type tracheo-oesophageal fistula (H-TOF ) accounts for 4%–5% of all congenital tracheo-oesophageal malformations. We present our experience in managing 18 cases with congenital H-TOF at a tertiary institute over a 10-year period.MethodsRecords of all patients with congenital H-TOF managed from January 2009 to December 2018 in the Department of Paediatric Surgery at a tertiary institute were retrospectively analysed based on the age at presentation, gender, antenatal ultrasonography findings; birth history; details of previous hospitalisations, previous treatment details, presenting symptoms and associated anomalies; time to diagnosis; radiological investigations performed, bronchoscopy findings, intraoperative details, complications and postoperative follow-up.ResultsTotally 18 patients with congenital H-TOF were managed over a 10-year period. There were 12 females and six males. Six patients had associated anomalies. There was wide variation in age at the start of symptoms (3 days–4 years) and presentation/referral to us (15 days–12 years). Four patients were diagnosed to have H-TOF at first admission. The most common presenting symptom was recurrent pneumonias (n=18). Bronchoscopy was done in all patients, and fistula was diagnosed and cannulated before surgery. The fistula was present at C8–T1 in 14 patients. The median age at surgery was 12 months. In 17 patients, the fistula was repaired by the cervical approach. There were two deaths, and 16 patients are doing well on median follow-up of 8 years.ConclusionCongenital H-TOF should be considered in differential diagnosis while managing patients with recurrent lower respiratory tract infection and ‘coughing and choking episodes’; early diagnosis and management of the associated H-TOF is important for improved survival and outcome.

A rare case of obstructive supracardiac TAPVR (Total Anomalous Pulmonary Venous Return) with aberrant right subclavian artery.

Congenital aortic arch malformations manifest a broad-spectrum of differences and abnormalities that come from disturbed embryogenesis of branchial arches. Current case was a 10 months old baby girl with length of 69 cm (less than –3 SD) and weight of 5.5 kg (less than –3 SD). The patient had history of recurrent lower respiratory tract infections since the time of birth and failure to gain adequate weight since the time of birth. The patient has been having multiple check-ups with registered medical practitioners in the nearby locality and multiple courses of antibiotics with only partial resolution of symptoms. The 2-D echocardiogram showed her to be a case of supracardiac type of “Total Anomalous Pulmonary Venous Return (TAPVR)”. All pulmonary veins making a confluence and draining into the right atrium. Significant turbulence observed at the level of superior vena cava to right atrium junction. A level of obstruction was recorded at the junction of the confluence of pulmonary veins and the vertical vein. There was aberrant right subclavian artery from the aortic arch as its third branch with no obstruction or aneurysm formation, having retrotracheal and esophageal course.

Immunodeficiency Caused Byheterozygous RAG1 Gene Mutation in a Boy Manifested As Severe Granulomatous Diseases and Eosinophilia: A Case Report

Severe combined immunodeficiency (SCID) is an inherited diseases caused by the defective development of T- and/or B-lymphocytes refractory to common therapy. We report a 13-year old boy with combined immunodeficiency caused by a heterozygous mutation at cDNA position 1435 (c.1435C>T) in RAG1 gene. His father and grand-father carried the same heterozygous RAG1 gene mutation, and experienced anaphylactic rhinitis. His mother had mutations in 6 immunodeficiency-related heterozygous genes but no RAG1 mutation, and experienced thrombocytopenia. Both his parents had normal NK and T cell numbers, and serum immunoglobulin (Ig) levels. After birth, this patient had severe allergies to many food and drugs, and suffered from recurrent upper and lower respiratory tract infections every year. After admission, the patient complained of a generalized body rash with itches lasting about several years. We found severe granuloma lesions in the face, mouth and nose, lymphadenectasis and splenomegaly by physical examination (Figure 1a). Laboratory examinations demonstrated that he had a high eosinophilic granulocyte count as 2.19×109/L, but decreased NK cell numbers and CD4+/CD8+ T cell ratios, and IgG, IgM and IgA serum levels. This patient has been previously treated with immune-modulatory drugs including anti-histamine drugs, prednisone, γ-globulin, and chemotherapy including Cyclophosphamide, but lacking clinical improvement. In our center, after Methotrexate (1g/m2 q.w.)+L-asparaginase (10000 unit q.d.×10 days) + Dexamethasone (10mg/m2q.d.×5 days) chemotherapy, and immune enhancement therapy with γ-globulin(400mg/kg×5 days per month), the patient got his symptoms ameliorated (Figure 1b). Haploidentical hematopoietic stem cell transplantation (HCT) from his HLA 7/10 matched mother was subsequently applied, and the patient remained remission without HCT-related complications for at least 8 months after transplantation (Figure 1c). Our results thus showed the immune disorder in a boy caused by a novel heterozygous RAG1 gene c.1435C>T mutation which has been efficiently treated by our specific chemotherapy combined with HCT. [Display omitted] DisclosuresNo relevant conflicts of interest to declare.

Novel STAT-3 gain-of-function variant with hypogammaglobulinemia and recurrent infection phenotype.

Signal transducer and activator of transcription 3 (STAT‐3) gain‐of‐function (GOF) syndrome is an early‐onset monogenic inborn error of immunity characterized by multi‐organ autoimmune disorders, growth failure and lymphoproliferation. We describe that STAT‐3 GOF syndrome may be presented with hypogammaglobulinemia and recurrent severe upper and lower respiratory tract infections. In addition, the patient had lymphoproliferation, short stature and interstitial lung disease. Chest computerized tomography examinations showed mild bronchiectasis with areas of non‐fibrosing alveolar‐interstitial disease and maldevelopment of bilateral first ribs. Using Sanger sequencing, we revealed a novel c.508G>C, p.D170H STAT‐3 variant affecting the coiled coil domain of STAT‐3. Functional studies confirmed that p.D170H was a GOF variant, as shown by increased phosphorylated STAT‐3 (pSTAT‐3) and STAT‐3 transcriptional activity. Our observation suggests that STAT‐3 GOF syndrome can manifest in early childhood with hypogammaglobulinemia and recurrent severe respiratory tract infections. We suggest that patients with lymphoproliferation, hypogammaglobulinemia and severe recurrent infections should be screened for STAT‐3 variants, even if autoimmune manifestations are missing.

Adult Primary Antibody Deficiencies and the Lung.

Primary antibody deficiency diseases result from a genetic defect that causes misfunction of 1 or more of the immune system elements. Due to the increased awareness among physicians and the success of new treatment modalities, the number of pediatric patients reaching adult age and the number of patients diagnosed in adult age is increasing. Adult patients comprise more than half of the total cases. Primary antibody deficiencies are the most common immunodeficiency type in adults, and these may cause recurrent upper and lower respiratory tract infections and result in the development of bronchiectasis. Among non-infectious pulmonary complications, any type of interstitial lung disease may be seen; however, a special type seen in patients with common variable immunodeficiency, namely granulomatous lymphocytic interstitial lung disease, is the one most commonly investigated. Underlying or accompanying immunodeficiency may be present in patients with asthma and chronic obstructive pulmonary disease, especially if the disease requires frequent hospitalizations and/or is severe. Early diagnosis and appropriate management of primary antibody deficiency diseases in patients with respiratory symptoms are crucial to decrease complications and increase survival.

Pediatric flexible bronchoscopy: A single-center report.

IntroductionPediatric flexible laryngotracheal bronchoscopy (FB) is an integral part of diagnostics and treatment at tertiary pediatric respiratory centers.AimFBs performed between 2013 and 2018 at our Pediatric Allergy and Respiratory Medicine Unit of the Department of Women's and Children's Health at Padua University were examined in terms of the indications, findings, and adverse events.Materials and MethodsThe electronic medical records of pediatric patients who underwent FB at least once between 1 January 2013 and 31 December 2018 were considered. Patients' clinical data, indications for FB, anatomical findings, information derived from bronchoalveolar lavage (BAL) and bronchial brushing, and possible adverse events were analyzed.ResultsThere were 447 pediatric FBs performed in 428 patients (aged from 1 month to 18 years) for diagnostic purposes (92.4%), to clear secretions (3.6%), or to monitor a known condition (4.0%). The main indications were recurrent lower respiratory tract infections (LRTI, 32.2%) and chronic wet cough (9.4%). Lower airway malacia was the most common abnormal finding in these two groups (36.1% and 28.6%, respectively). BAL bacterial culture was positive in 55 children (39.6%) with recurrent LRTI and in 25 (59.5%) with chronic wet cough, being Haemophilus influenzae, Streptococcus pneumoniae, and Moraxella catarrhalis the microorganisms most commonly isolated. FB proved a safe procedure and was well tolerated.ConclusionsPediatric FB is an essential tool at our tertiary pediatric respiratory center. It helps establish the anatomical conditions underlying several chronic respiratory conditions and any correlated microbiological findings, with a significant impact on further patient management.

NUTRITIONAL ASSESSMENT OF CHILDREN WITH CONGENITAL HEART DISEASE IN A TERTIARY CARE CENTRE, AURANGABAD, MAHARASHTRA: A CROSS SECTIONAL STUDY

Background: Children with congenital heart disease (CHD) have increased prevalence of malnutrition for various reasons. This study aimed to identify prevalence and predictors of malnutrition in children with symptomatic CHD. Methods: This study included 110 children with symptomatic CHD (78 acyanotic and 32 cyanotic CHDs). Clinical Evaluation and Laboratory Assessment of Nutritional Status were documented. Anthropometric measurements were recorded and Z scores for weight for age (WAZ), weight for height (WHZ), and height for age (HAZ) have been calculated. Malnutrition was dened as weight, height, and weight/height Z score ≤–2. Results: The overall prevalence of malnutrition was 87.0 % in patients with CHD. Severe malnutrition was diagnosed in 49 % of cases. In both group of CHD patients, stunting and wasting was high. Malnutrition correlated with pulmonary hypertension, recurrent respiratory tract infections and feeding difculties but not statistically signicant levels. Conclusion: Malnutrition is a very common problem in children with symptomatic CHD and presence of feeding difculties, pulmonary hypertension, recurrent lower respiratory tract infections are additional risks for malnutrition.

Role of bronchoscopy and imaging in long-standing foreign body bronchus presenting as recurrent or non-resolving lower respiratory tract infection.

A long-standing retained foreign body in the bronchus is unusual. In majority of cases, an adequate history is not obtained, the clinical picture is usually clouded by superadded pathological changes. We report three cases of long-standing foreign body in the airway who presented with recurrent lower respiratory tract infection. Examination of respiratory system revealed no significant abnormality. Chest radiograph was normal. CT scan of the chest was useful to indicate endobronchial opacity in the airway suggestive of a foreign body. The patients underwent rigid bronchoscopy under general anesthesia for successful removal of the foreign body. So the patients with non-resolving or recurrent lower respiratory symptoms in spite of medical treatment and without any obstructive findings must undergo diagnostic bronchoscopy evaluation and imaging.

Morbidity after thoracoscopic resection of congenital pulmonary airway malformations (CPAM): single center experience over a decade.

Video-assisted thoracoscopic (VATS) resection of CPAM in children is an established, albeit controversial strategy for its management. We report a 10-year single center experience. All children underwent VATS (2008-2017) and their current status was reviewed. Patients were grouped: 'symptomatic-P' (if parents reported recurrent lower respiratory tract infections etc.) or 'symptomatic-S' (neonates presenting with respiratory distress/difficulty) or 'asymptomatic'. 73 children, aged 10m (4d-14yrs) underwent VATS; a neonate as an emergency ('symptomatic-S') and all others electively. The lesion was unilateral in all but one case. Histologically none were malignant. Of the elective 72 cases, 7 (10%) required conversion to open thoracotomy. Twenty (27.7%) were 'symptomatic-P' and the duration of surgery when compared to 'asymptomatic' children was longer 269 (range 129-689) versus 178 (range 69-575) minutes (P = 0.01). Post operatively, 8 children (11%) had a grade III/IV (Clavien-Dindo) complication; persistent air leak/pneumothorax (n = 5), chylothorax (n = 1), pleural effusion (n = 1) and seizure/middle cerebral artery thrombosis (n = 1). There was no mortality. Twenty-four children (33.3%) were reported 'symptomatic-P' post-surgery after a median follow up of 2.18years. The surgical intervention had no impact on 'symptomatic-P' status (P = 0.46). The risks of surgery may outweigh benefit in asymptomatic children. CLINICALTRIALS. NCT04449614.

Patterns of Recurrence and Antimicrobial Management in Respiratory Infections: A Real-World Retrospective Study

Objectives: Respiratory tract infections (RTIs) significantly contribute to morbidity and mortality. Management of RTIs is complex with several etiological agents, development of resistance to antimicrobials, and multiple risk factors which may result in recurrence of infections. Therefore, choice of antibiotic therapy needs to be evidence-based for appropriate treatment and prevention of recurrence. This study aimed to explore the pattern of recurrence in RTIs and the type of antimicrobials used in real-world clinical settings. Methods: This study was a retrospective, cross-sectional, observational, multicenter study. Electronic medical records of patients with upper RTIs (URTIs) and lower RTIs (LRTIs) visiting three centers from 2014 to 2020 were considered for the analysis. Results: A total of 236 patients had an overall 531 episodes of RTIs with a mean age of 41.75 years. There were 391 LRTIs and 140 URTIs. Majority of the patients were adults (64.83%), and males were predominant (65.68%). In LRTI episodes, the most frequently prescribed antimicrobial monotherapy/fixed dose combination was macrolide (clarithromycin) (12.53%), followed by cefpodoxime-clavulanic acid (9.97%). Majority of the URTIs were managed with clarithromycin (49.28%) and azithromycin (16.43%). Recurrence of LRTI was observed in 23 patients with gram stain positive cocci. Additionally, out of 27 patients with culture-positive pseudomonas infections, 10 had recurrent LRTI episodes. Macrolides and cephalosporins were mostly prescribed in these episodes. Conclusion: This study highlights that one of the major causes of recurrence could be incomplete eradication of pathogens. In the real-world scenario, recurrent URTI and LRTI episodes can be managed by macrolides and cephalosporins.

Implementation of a screening tool for primary ciliary dyskinesia (PCD) in a pediatric otolaryngology clinic

BackgroundPrimary ciliary dyskinesia (PCD) is a rare genetic disease arising from motile ciliary dysfunction and associated with recurrent and chronic upper and lower respiratory tract infections. Pediatric otolaryngologists may see these patients prior to the development of lung disease. Features of PCD may overlap with other suppurative respiratory diseases, creating diagnostic challenges. A simple screening tool would be beneficial to identify potential patients who have chronic upper respiratory tract disease requiring further specialist evaluation. ObjectiveTo test a simple screening tool consisting of four questions to detect PCD in children with chronic otitis media and chronic rhinosinusitis seen in a tertiary otolaryngology clinic. MethodsA prospective, single site, observational study in a tertiary care pediatric otolaryngology clinic. Children aged 3–17 years diagnosed with chronic otitis media or rhinosinusitis with onset at less than 2 years of age were recruited. All study subjects had at least one of four key clinical features for PCD as determined by answers to screening questions, while control subjects had none. All participants completed a medical history questionnaire and nasal nitric oxide measurements. Those with reduced nasal nitric oxide levels were referred to our PCD center for further evaluation. ResultsA total of 153 patients were screened and 62 subjects were enrolled. Of those, 35 were enrolled as study subjects and 27 as matched controls. Study subjects had mean age of 7.5 years (3.2–16.5) with pre-screening diagnosis of chronic otitis media (n = 29) or chronic rhinosinusitis (n = 6). Control subjects (n = 27) had mean age 7.2 years (3.0–16.3) with pre-screening diagnosis of chronic otitis media (n = 25), and chronic rhinosinusitis (n = 2). There were no differences in subject demographics or mean nasal nitric oxide values between the two groups (179.8 vs 210.8 nl/min). Ten individuals had low nasal nitric oxide values, 7 of which were normal on repeat testing. Three subjects failed to return for follow up evaluations. Four referrals were made for further evaluation on the basis of clinical symptoms and nasal nitric oxide results. While no new cases of PCD were detected, a subject and his sibling with recurrent sinopulmonary infections were referred for immunologic evaluation. ConclusionThe use of standardized screening questions can be used in an otolaryngology clinic to identify patients who require further evaluation for PCD or primary immunodeficiency.

Tekrarlayan Akciğer Enfeksiyonu ve Dirençli Demir Eksikliği Anemisi Ayırıcı Tanısında Nadir Bir Antite: İdiyopatik Pulmoner Hemosiderozis

İdiyopatik pulmoner hemosiderozis (İPH), demir eksikliği anemisi (DEA), tekrarlayan alveoler hemoraji atakları ve hemoptizi ile seyreden ve etyolojisi tam bilinmeyen nadir bir hastalıktır. Olguların büyük çoğunluğu çocukluk döneminde görülür. Klinik olarak hemoptizi, alveoler hemoraji atakları sırasında gelişen solunum sıkıntısı, akciğer grafisinde parankimal infiltratlar ve sekonder demir eksikliği anemisi gibi bulgularla prezente olur. Bu nedenle de tekrarlayan solunum sıkıntısı ve demir eksikliği anemisine neden olan diğer hastalıklarla ayırıcı tanısı yapılmalıdır. Biz burada tekrarlayan alt solunum yolu infeksiyonu ve kistik fibrozis ön tanısıyla izlenen ve İPH tanısı alan iki yaşındaki bir olguyu sunuyoruz. Olgunun tanısı bronkoalveoler lavaj sıvısında hemosiderin yüklü makrofajlar görülerek doğrulanmış ve steroid tedavisi ile remisyon sağlanmıştır.

Antibiotic prophylaxis for the prevention of respiratory tract infections in antibody deficient patients: A retrospective cohort study

Abstract Background Antibiotic prophylaxis is commonly used in the management of antibody deficient patients, but very few studies have attempted to define its role in this particular patient population. Objectives The aim of this study was to clarify the role of antibiotic prophylaxis in the prevention of lower respiratory tract infections in these patients. Methods In a tertiary Immunology referral centre in England we retrospectively analysed the Immunology database to identify adult antibody deficient patients who had received antibiotic prophylaxis to prevent lower respiratory tract infections over the past 15 years. Results One hundred and five patients were identified and antibiotic prophylaxis was found to be beneficial in this cohort. There was a significant reduction in the number of positive sputum cultures from 1.61 to 1.05 per patient year. Infection-related hospital admissions also dropped significantly, from 0.53 to 0.19 per patient year. Similar results were seen in the subgroup of patients that were not receiving immunoglobulin replacement. Although our study showed emergence of secondary antimicrobial resistance (14%) and a 7.5% rate of colonisation with multi-drug resistant organisms, these factors did not have any significant impact on outcomes. Conclusions The above findings suggest that antibiotic prophylaxis should be considered in antibody deficient patients who are troubled by recurrent lower respiratory tract infections. Randomized controlled trials are required to confirm these findings.