History Of Recurrent Pneumonia Research Articles

Aerodigestive evaluation of pediatric patients with chronic aspiration

ObjectiveTo evaluate the effectiveness of coordinated endoscopy with otolaryngology, pulmonology, and gastroenterology in diagnosing and managing chronic aspiration in pediatric patients. MethodsWe reviewed our REDCap Pediatric Aerodigestive Database for patients with chronic aspiration who underwent coordinated endoscopy between January 2013 and July 2023. Patient demographics, comorbidities, operative findings, interventions, and outcomes were reviewed. ResultsForty-nine patients were identified with a diagnosis of aspiration. Their mean (SD) age was 28 (36) months (range 1.2–163 months) with more than half of the patients younger than 24 months. The most common findings noted on combined endoscopies were laryngeal cleft (n = 30), positive bacterial culture (n = 18), positive viral PCR (n = 17), and active reflux-induced esophagitis/gastritis (n = 9). Patients with a positive bacterial culture were associated with a history of recurrent pneumonia (p = 0.009). There were no other significant associations between endoscopy findings and patient demographics, co-morbidities, or symptoms. Twenty-five (51 %) had multiple abnormalities identified by at least 2 different specialists at the time of endoscopy and 6 patients (12 %) had abnormalities across all three specialists. ConclusionCoordinated endoscopy should be considered in pediatric patients presenting with aspiration on MBS or non-specific symptoms suggestive for chronic aspiration for comprehensive diagnosis and management.

Enhancing pathogen detection and characterization of respiratory microbiome signatures of infection through next-generation sequencing

Next-generation sequencing-based (NGS) methods for pathogen detection, which can capture the entire microbial composition in a clinical sample, are used strategically in parallel to culture-based methods. While NGS-based methods cannot confirm organism viability, culture-based methods can but are limited by the number of culture conditions routinely utilized in clinical microbiology laboratories. The relatively wide scope provided by sequencing compensates for the limited scope of culture-based methods but creates obstacles in distinguishing pathogens from an expected body-site-specific microbiome or benign transient colonizers. This study compares NGS-based methods for pathogen detection in respiratory samples to current diagnostics at Texas Children's Hospital in Houston, Texas. Current diagnostics include various culture-based assays, targeted PCR assays, and reference laboratory-generated sequencing. We hypothesize that employing sample metadata (source, type, etc.) and patient data (clinical history, chronic conditions, etc.) during data analysis will help distinguish pathogens from expected flora in respiratory samples. To develop these methods, 27 respiratory samples with suffcient volume for both routine diagnostic testing and this study were collected. Nucleic acid was extracted using the Qiagen DNeasy PowerSoil Pro Kit after collection, and targeted short-read sequencing (16S, 18S/ITS) was performed. These data were then analyzed using a custom bioinformatic pipeline built around Qiime2-2022. In brief, sequences are denoised and quality filtered using DADA2, an algorithm that uses a statistical model for correcting sequencing errors, after which similar amplicon sequence variants (ASVs) are clustered. Next, a feature table of ASVs that are 100% identical is generated, and taxonomy assigned using a naive Bayes taxonomy classifier against the Silva 13.2 database. Finally, unassigned and low abundance ASVs are removed. When species-level identification couldn't be determined through targeted short-read sequencing, untargeted long-read sequencing was used. In one case, we found that a bronchoalveolar lavage sample from the lung of a patient with a history of recurrent pneumonia contained a singular pathogenic bacterium, H. influenzae. This finding agreed with results obtained through reference laboratory-generated sequencing. In another case, we identified >14 unique bacteria at varying concentrations in a wash sample collected from the lung of an asymptomatic patient during a routine bronchoscopy. These findings, however, were determined to be expected respiratory flora. Our NGS-based methods’ enhanced pathogen detection compared to current diagnostics and discriminates between respiratory flora and active infections. Setting thresholds for pathogen detection, evaluating clinical relevance in the context of body site and presentation, and deploying additional molecular techniques when necessary are crucial steps to increase the accuracy of pathogen detection in the clinic. This study was supported by the Texas Children's Hospital Department of Pathology. This is the full abstract presented at the American Physiology Summit 2024 meeting and is only available in HTML format. There are no additional versions or additional content available for this abstract. Physiology was not involved in the peer review process.

Diagnostic Strategy for Suspected Unilateral Absence of the Pulmonary Artery.

To analyze the diagnostic strategy applied to seven patients with UAPA who were examined and subsequently treated at the National Lung Hospital, Hanoi, Vietnam. All seven patients, including three pediatric cases (1, 2, and 14 years old) and four adult cases (21, 26, 44, and 53 years old), had a history of recurrent pneumonia, and the clinical symptoms on admission included cough, progressive dyspnea, chest pain, and fatigue. The patients were initially examined clinically, followed by hematological testing, blood biochemistry testing, and chest X-ray radiology. The results suggested UAPA, so echocardiography and contrast-enhanced chest computed tomography (CT) were performed as soon as practical. The echocardiographic and CT imaging findings confirmed the suspected diagnosis of UAPA in all seven patients, which was accompanied by congenital heart disease in three patients. Three of the seven patients had mild and medium pulmonary hypertension. All seven patients were treated with drugs, which led to improvement in symptoms. Frontal chest X-ray provided the initial signs suggesting a diagnosis of UAPA. Subsequent echocardiography and contrast-enhanced chest CT were effective diagnostic tools for fast and accurate confirmation of UAPA.</p>.

Diffuse large B-cell lymphoma in 1-year male with founder Slavic RAG1 mutation: case report

Background and AimsLymphomas in RAG1 deficiency are extremely rare, especially in early childhood. Only a few cases of lymphoma in RAG1 deficiency have been previously described in the literature. MethodsSanger sequencing of Slavic founders mutation (RAG1-c.256_257del, p.Lys86ValfsTer33, IL7Rα - c.132C>G, p.Ser44Arg, NBN1 -c.657_661del5, p.Lys219AsnfsTer16 genes) in DNA of died children with malignancy in early age in Belarus. ResultsWe report a case of 14 month-old boy affected by diffuse large B-cell lymphoma diagnosed with RAG1 deficiency. A male patient of Belarus origin was born full-term from non-consanguineous parents. He had a history of recurrent pneumonia since 3 months of age. The patient was hospitalized in 14 month-old with subfebrile fever, wet cough, lymphadenopathy and cyanosis of the nasolabial triangle. Chest x-ray showed bilateral polysegmental pneumonia. Microbiological analysis of sputum found Candida. Ultrasound examination showed hepato-(+3 cm)-spleno- (+3 cm)-megaly and lymphadenopathy of multiple groups of lymph nodes (cervical, inguinal and axillary). Lymph-node, liver, and intestines biopsies were suggestive of EBV-positive Diffuse Large B-cell lymphoma, NOS. He had progressive leukopenia (from 3100 cells/µl to 200 cells/µl). Lymphopenia (400 cells/µl), decreased T-cells (300 cells/µl) and elevated activated T-cells (41%) were detected. B-cell percentage was in normal ranges (33%), but absolute number was slightly decreased (160 cells/µl). TRECs – 0 per 1 million leukocytes, KRECs – 0 per 1 million leukocytes. According to the clinical and immunological phenotype, combined immunodeficiency was suspected.The patient died (age 15 m) one month after the start of the chemotherapy cycle due to sepsis and multiple organ failure without genetic diagnosis. His material was studied post-mortem due to the lymphoma at early age and place of birth (Brest, Western Belarus). Targeted Sanger sequencing revealed a homozygous deletion in RAG1 gene (NM_000448.3) c.256_257del (p.Lys86ValfsTer33). ConclusionsWe described a rare case of EBV-positive diffuse large B-cell lymphoma NOS in a patient with homozygous founder Slavic mutation in the RAG1 gene.

LATE-ONSET COMBINED IMMUNE DEFICIENCY IN A PATIENT WITH ADENOSINE DEAMINASE GENE MUTATION

<h3>Introduction</h3> We report on a 25-year-old man with a history of recurrent pneumonia and large joint infections who was found to have T cell dysfunction with B cell lymphopenia and a heterozygous adenosine deaminase(ADA) gene mutation. <h3>Case Description</h3> The patient has had three large joint infections and three-to-four episodes of pneumonia in his life. He is without other prior medical condition or family history of immune deficiency. He presented to the ER with tachycardia and left knee swelling. He was found to have pseudomonas bacteremia of unknown source for which he was treated. Due to his tachycardia, he had a chest CT to evaluate for pulmonary embolism which revealed found incidental bronchiectasis. Evaluation for immune deficiency was initiated and he was found to have IgA <10 mg/dL, IgG <75, and IgM <20, CD4 count 202 cells/uL, CD8 596, low response to polysaccharide and protein vaccines. HIV testing was negative. We then pursued genetic testing. Patient was found to carry a single pathogenic variant in the ADA gene. Loss of function variants in this gene are associated with severe combined immunodeficiency, but a second variant was not identified. Our patient is doing well on IVIG without further infections. <h3>Discussion</h3> To our knowledge, our patient is the first reported to be a carrier for a pathogenic ADA gene mutation and presenting with a clinical phenotype of B cell lymphopenia and T cell dysfunction. A more complete understanding of the underlying genetic causes for late-onset combined immune deficiency is needed as well as any correlation with ADA deficiency.

Plasma Metagenomic Sequencing Expedites Diagnosis of Disseminated BCG in an Infant With IKBKB Mutation.

Infants with inborn errors of immunity (IEI), born in countries where Bacillus-Calmette-Guerin (BCG) vaccination is recommended at birth, are at risk of developing infectious complications following vaccination. A prompt diagnosis of disseminated BCG infection in these infants is essential, as many will require stem cell transplantation (SCT) for the immunologic cure. In patients with IEI, the mortality risk from disseminated mycobacterial infection is high, both before and following SCT. A 7-month-old Qatari infant with an IEI, homozygous IKBKB gene mutation, was evaluated at our institution for SCT. He had a history of recurrent pneumonias, but pretransplant evaluation revealed negative cultures from bronchoalveolar fluid, blood and urine. At 8 months of age, the infant developed skin nodules of unclear etiology, prompting additional evaluation. Given his profound immunosuppression and receipt of broad-spectrum antimicrobials, plasma metagenomic next-generation sequencing (mNGS) was obtained and identified Mycobacterium tuberculosis complex within 72 hours. A skin biopsy was performed, and antimycobacterial therapy was initiated. Mycobacterium bovis-BCG was confirmed from cultures 3 weeks later. Treatment was complicated by elevated serum liver transaminases and aminoglycoside-associated high-frequency hearing loss. The infant completed 14 months of treatment from engraftment. Evaluation for active BCG infection after SCT was negative. In an infant with a unique IEI, plasma mNGS provided the first diagnosis of disseminated BCG infection. We believe that early initiation of antimycobacterial treatment improved the infant's clinical outcome. Plasma mNGS testing should be considered as a noninvasive screen for infectious pathogens in children with IEIs before SCT.

Primary Antibody Deficiencies With Pulmonary Complications: A Single-Center Experience.

BackgroundPrimary immunodeficiencies are a heterogeneous group of genetic diseases caused by one or more abnormalities in the immune system. Although pulmonary complications are common in patients with primary immunodeficiency diseases, these complications contribute significantly to morbidity and mortality.AimThe aim of our study was to evaluate the distribution of the features of pulmonary radiological involvement and demographic findings in this patient group.Materials and methodsThe files of patients who were treated and followed up with the diagnosis of primary immunodeficiency between 2014 and 2021 were analyzed retrospectively. Demographic data, symptoms, additional diseases, and computed tomography findings of the patients were recorded.ResultsThe mean age of 32 cases was 37.34±13.54 (20-69) and the age of diagnosis was 28.90±15.75 (1-62). Twenty of the cases were male and 10 were female. The most common symptom was diarrhea with 53.1% and cough with 34.4%. The most common radiological finding is bronchiectasis in 75% of cases. Twenty-one (65.6%) of the cases had recurrent pneumonia before diagnosis and no pneumonia was observed after intravenous ımmunoglobulin replacement therapy. Three of the cases (9.4%) died during the follow-up.ConclusionsPrimary immunodeficiency should be considered in patients with bronchiectasis and a history of recurrent pneumonia, and further investigations should be performed. Early diagnosis of patients with primary immunodeficiency is very important for the early detection and treatment of malignancy and the interstitial lung disease that may develop.

Multimodal Approach to Acquired Bronchobiliary Fistula Secondary to Hepaticojejunostomy Stricture Following Yttruim-90 Therapy

Introduction: Bronchobiliary fistula (BBF) is an abnormal connection between the bronchial system and biliary tree. Bilioptysis (bilious-tinged sputum) is pathognomic of BBF. Mortality rate of BBF is as high as 12.7%. Method: A 56-year-old male who presented with stage IV pancreatic neuroendocrine tumor, underwent pancreaticoduodenectomy with partial hepatectomy, followed by Yttrium-90, and microwave ablation. Surveillance imaging, 2.5 years later, noted increase in hepatic lesions. Additional Yttrium-90 was poorly tolerated and patient was referred for peptide receptor radionuclide therapy. Ten months later patient presented with abdominal pain, cough with yellow-tinged sputum and history of recurrent pneumonia. Imaging demonstrated intrahepatic biliary dilation, peri-hepatic abscess and right-sided pleural effusion. Results: Patient underwent percutaneous drainage of abscess and chest tube placement. Persistent cough and sputum production prompted evaluation with HIDA (hepatobiliary iminodiacetic acid) and bronchoscopy, confirming BBF. Cholescintigraphy with single-photon emission computed tomography (NM-SPECT) localized the fistula tract to the right upper lobe of liver (Figure-1). Initially attempted biliary decompression with percutaneous transhepatic cholangiogram was unsuccessful due to failed cannulation of the hepaticojejunostomy, indicating complete stenosis at the anastomosis. Finally, the patient underwent resection of the hepaticojejunostomy with mobilization of the pancreaticobiliary limb and Kasai-type porto-enteric anastomosis between the pancreaticobiliary limb and hepatic duct. Complete resolution of symptoms and the bronchopulmonary fistula on follow up imaging, was noted. Conclusion: BBFs are rare, misdiagnosed, and difficult to treat. Although treatment with minimally invasive approach has favorable results, history of previous biliary reconstruction, may warrant multimodal treatments including percutaneous and complex surgical intervention.

A PUZZLING CASE OF RECURRENT PNEUMONIA: PLEUROPARENCHYMAL FIBROELASTOSIS

TOPIC: Lung Pathology TYPE: Medical Student/Resident Case Reports INTRODUCTION: Pleuroparenchymal fibroelastosis (PPFE) is a fibroelastic process mainly affecting the visceral pleura and subpleural spaces with a propensity towards the upper lobes. It has unique clinical, pathological and radiological features1. CASE PRESENTATION: An 80 year old female with history of recurrent pneumonias and remote granulomatous disease presented with progressive dyspnea and fatigue. She denied other symptoms including chronic cough or sputum production or fever or chills. Chest X-ray showed extensive parenchymal and pleural fibrotic changes of the upper lobes. Pulmonary function tests suggested a restrictive pattern with reduced diffusion capacity (DLCO). Echocardiogram showed no significant abnormalities. Serial CT scans of the chest done over two years showed worsening pleuroparenchymal fibrotic changes of the upper lobes in a pattern very consistent with PPFE, with no evidence of honeycombing. This was likely idiopathic but potentially related to prior pneumonias and felt less likely to be related to her remote minimal parakeet exposure associated hypersensitivity pneumonitis. Her ANA titers were mildly elevated at 1:160 but further workup for auto-antibodies were negative including RF and CCP antibodies. ESR and CRP were minimally elevated. A bronchoscopy was offered to evaluate for infectious or other etiologies but she opted to wait. Her CT findings were however very consistent with PPFE and a provisional diagnosis of the same was made. DISCUSSION: PPFE is a rare but under-diagnosed type of interstitial lung disease. It is mostly idiopathic in nature but sometimes occurs as a late complication after lung transplantation. Although similar findings may be sometimes described in patients with autoimmune or connective tissue disease, it usually presents as a late complication and in certain patients with hypersensitivity pneumonitis, the idiopathic ones are best classified as PPFE1. PPFE is mostly seen in patients aged 40 to 70 years and presents as progressive dyspnea with or without cough. PPFE has to be differentiated from other upper lobe diseases like hypersensitivity pneumonia, sarcoidosis, idiopathic interstitial pneumonitis, non-tuberculous mycobacterial infection, pneumoconiosis, malignancy and apical pleural cap. High resolution CT scan remains the best diagnostic modality to show diffuse fibroelastic process predominantly affecting pleura and upper lobe parenchyma1. Pulmonary function test shows a restrictive pattern with reduced DLCO. No specific laboratory tests are diagnostic2. Surgical biopsies although not required for diagnosis can demonstrate intra-alveolar fibrosis and elastosis2. CONCLUSIONS: The prognosis of PPFE remains poor as no treatment is proven to be effective except therapeutic lung transplantation1. Further studies to understand pathogenesis, diagnostic markers and treatment modalities for this rare progressive disease are much warranted. REFERENCE #1: Chua F, Desai SR, Nicholson AG, et al. Pleuroparenchymal fibroelastosis. A review of clinical, radiological and pathological characteristics. Ann Am Thorac Soc 2019;16:1351-9. REFERENCE #2: Reddy TL, Tominaga M, Hansell DM, von der Thusen J, Rassl D, Parfrey H, Guy S, Twentyman O, Rice A, Maher TM, Renzoni EA, Wells AU, Nicholson AG. Pleuroparenchymal fibroelastosis: a spectrum of histopathological and imaging phenotypes. Eur Respir J. 2012 Aug;40(2):377-85. DISCLOSURES: No relevant relationships by Ansaam Daoud, source=Web Response No relevant relationships by Palak Rath, source=Web Response No relevant relationships by Lokesh Venkateshaiah, source=Web Response

Petrifying: ears as hard as stone in adrenal insufficiency

A 75-year-old man was admitted to our Department of Endocrinology with a 2 month history of recurrent pneumonia, somnolence, and progressive cachexia requiring enteral nutrition via a percutaneous endoscopic gastrostomy tube. His wife reported that the man had had recurrent episodes of weakness during stressful situations over the past 20 years. Clinical examination of the patient revealed somnolence, hypotension (blood pressure <100/60 mm Hg), and cachexia (weight 50 kg, height 174 cm, BMI 16·5 kg/m2).

PULMONARY HYPOPLASIA SECONDARY TO PULMONARY ARTERY STENOSIS: SURGICAL FIX FOR RECURRENT PNEUMONIA?

SESSION TITLE: Global Case Report Posters SESSION TYPE: Global Case Reports PRESENTED ON: October 18-21, 2020 INTRODUCTION: Introduction Congenital bronchopulmonary vascular malformations include a wide spectrum of disorders that compromise one or more of the three main systems of the lung (lung parenchyma, arteries and veins). The stage of gestation where disruption of the pulmonary vessels occurs determines the severity of lung affectation and alveolarization, ranging for segmentary affectation or the complete pulmonary hypoplasia. We present a case of primary pulmonary hypoplasia secondary to pulmonary artery stenosis. CASE PRESENTATION: A 11-year-old boy with history of recurrent pneumonia is evaluated in clinic. He was first admitted to the intensive care unit at age 40 months for pneumonia. Since then, he has had multiple admissions for recurrent pulmonary infections and wheezing treated with beta agonists. His chest x-ray revealed a marked decrease in volume in the right lung, with ipsilateral mediastinal shift. Computerized tomography angiography showed generalized bronchiectasis, main bronchus and pulmonary artery hypoplasia in the right side, with a significant reduction in lung volume. The left lung was hyperinflated and had compensatory emphysema. Scintigraphy revealed a total absence of perfusion in the right lung. Right heart catheterism was consistent with pulmonary artery stenosis. Patient underwent right pneumonectomy. He developed pneumonia and hydrothorax in the postoperative period, and was treated conservatively with antibiotics and incentive spirometer use. Repeat imaging showed a decrease in pleural effusion size. He had clinical improvement and he was discharged home with ambulatory follow up. DISCUSSION: Pulmonary hypoplasia is frequently associated with other malformation, including abnormalities of the thoracic cavity, masses and amniotic fluid disorders. When severe, it is generally diagnosed prenatally or shortly after birth. It can also be associated with congenital heart defects and in rare cases, no cause can be found. The severity of symptoms and their time of occurrence is defined by the degree of hypoplasia present in the patient. There have been reports of diagnosis in adulthood, presenting with recurrent respiratory infections. Clinical exam can show a shorter hemitorax of the affected side, intercostal spaces or diminished breath sounds. Imaging studies like CT can aid diagnosis, showing deviation of the mediastinum to the affected side, thinner vasculature and enlarg. CONCLUSIONS: Pulmonary hypoplasia secondary to pulmonary artery stenosis might present as recurrent respiratory infections and hospitalization. In cases with severe hypoplasia where lung function is severely compromised, pneumonectomy can be considered. Reference #1: Katayama Y, Kusagawa H, Komada T, Shomura S, Tenpaku H. Bronchopulmonary foregut malformation. Gen Thorac Cardiovasc Surg. 2011;59(11):767-770. doi:10.1007/s11748-010-0763-z Reference #2: Abrams, M., Ackerman, V. & Engle, W. Primary Unilateral Pulmonary Hypoplasia: Neonate through Early Childhood — Case Report, Radiographic Diagnosis and Review of the Literature. J Perinatol 24, 667–670 (2004). https://doi.org/10.1038/sj.jp.7211156 Reference #3: Papadopoulos D, Misthos P, Chorti M, et al. Unilateral pulmonary hypoplasia in an adult patient. Monaldi Arch Chest Dis. 2018;88(1):829. Published 2018 Jan 29. doi:10.4081/monaldi.2018.829 DISCLOSURES: No relevant relationships by Amarilys Alarcon-Calderon, source=Web Response No relevant relationships by ISMAR LOPEZ MURALLES, source=Web Response No relevant relationships by LUIS PEREZ MANCILLA, source=Web Response No relevant relationships by Shubert Iván Quiñónez Najera, source=Web Response

A rare anaesthetic challenge in a one-lung ventilated paediatric patient with right upper lobe tracheal bronchus.

A tracheal bronchus is a rare congenital anomaly, suggesting abnormal bronchial development. The prevalence of tracheal bronchus in children who undergo bronchoscopy is estimated to be between 0.2% and 3%. When associated with recurrent infection, lobes of the lung must be removed to avoid further lung injury. In such cases, perioperative one-lung ventilation and airway management remain a huge challenge for anaesthesiologists. The case of this rare airway anatomic abnormality in a paediatric patient with two bronchial openings into the right upper lobe, and with a history of recurrent pneumonia, is reported. In addition to a normal opening, a distinct opening in the upper lobe of the right lung was observed, that originated directly from the trachea, superior to the carina. The entire right lung was deflated by left-lung ventilation using a single lumen tracheal tube, and the patient underwent right upper lobe resection. No anaesthesia complications were observed during recovery. In this case, timely identification of the tracheal bronchus and successful collapse of the right lung were key points in the anaesthesia management of this patient.

SEVERE TRACHEOBRONCHOMALACIA IN AN END-STAGE RENAL DISEASE PATIENT REFERRED FOR KIDNEY TRANSPLANT EVALUATION

SESSION TITLE: Medical Student/Resident Obstructive Lung Disease Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Tracheobronchomalacia (TBM) is characterized by loss of the integrity of the central airway wall cartilaginous structures resulting in hyper-dynamic airway collapse. In adults, TBM is either idiopathic or associated with other conditions. We present a case of severe TBM in a patient with end stage renal disease undergoing evaluation for kidney transplant. CASE PRESENTATION: A 45-year-old male with history of type one diabetes mellitus, end stage renal disease on hemodialysis and chronic obstructive pulmonary disease (COPD) diagnosed without Pulmonary function testing (PFT). He was referred for pulmonary evaluation prior to kidney transplant. He had history of recurrent pneumonia, coughing with choking sensations and high-pitched wheezing that resolved after expectoration of scant thick phlegm. He denied tobacco use, chemical exposures, or asthma. PFT revealed severe obstruction with FEV1/FVC ratio of 43.2 and FEV1 1.71 Liter. The loops showed small volumes and flattening of the expiratory loops (Figure 1). Computerized tomography (CT) scan of the chest showed crescentic deformity of the trachea and bilateral mainstem bronchi (Figure 2) consistent with TBM. He was started on airway clearance therapy with flutter valves and albuterol nebulization. Non-invasive-positive pressure ventilation (NIPPV) was denied due to unconventional indication. Shortly after outpatient evaluation, he was re-admitted for severe pneumonia requiring invasive mechanical ventilation. He failed extubation. Tracheal stent placement was unsuccessful due to intraprocedural episodes of desaturation and stent dislodgement. Percutaneous tracheostomy was performed and he was successfully weaned off mechanical ventilation. He uses cuffless tracheostomy tube and has not had pneumonia since. Unfortunately, he is not a transplant candidate due to his pulmonary disease. DISCUSSION: TBM is commonly mistaken as COPD or asthma due to the similarities in symptoms and obstructive pattern on spirometry. The gold standard for confirmation is flexible bronchoscopy and visualization of more than 50% dynamic airway collapse. 51-75% is considered mild obstruction, 76 - 90% moderate and greater than 90% is severe. Dynamic CT can be used quantify the severity of the TBM with a strong correlation. The initial treatment involves the use of bronchodilators and airway hygiene. If these fails, then NIPPV is used. The next intervention would be stenting or tracheostomy which splints the airways. Tracheobronchoplasty is a surgical option when stenting have shown significant symptoms improvement. CONCLUSIONS: This case highlights the importance of having a wide differential diagnosis when evaluating patients. A close look at the PFT flow-volume loops led us towards the diagnosis. Reference #1: Beckerman, Z. at al. Tracheobronchomalacia: Does One Size Fit All? Semin Thorac Cardiovasc Surg, 2019. 31(3): p. 486-487. Reference #2: Biswas, A., et al., Tracheobronchomalacia. Dis Mon, 2017. 63(10): p. 287-302. Reference #3: Uyar, M., et al., Tracheobronchomalacia as a Rare Cause of Chronic Dyspnea in Adults. Med Princ Pract, 2017. 26(2): p. 179-181. DISCLOSURES: No relevant relationships by RAMYA GORTHI, source=Web Response No relevant relationships by Stephanie Link, source=Web Response No relevant relationships by John Mwangi, source=Web Response

Recurrent Pneumonias and Bronchiectasis - Is it an Immunodeficiency Disorder? - A Case Report

Common Variable Immunodeficiency (CVID) is a form of primary immunodeficiency disorder characterised by hypogammaglobulinemia and recurrent sino-pulmonary infections. Its diagnosis is based on the presence of low serum IgG (&lt; 2 SD below normal for age) with or without low IgA/ IgM levels and presentation beyond two years of age. These children also have disorders of autoimmunity with majority of them presenting as autoimmune cytopenias, predominantly thrombocytopenia and some having anaemia and neutropenias. Here we report a nine years old boy with past history of recurrent pneumonia, presenting this episode with fungal pneumonia, thrombocytopenia and anaemia eventually diagnosed as CVID.

Cryodebulking of endobronchial hamartoma via fibreoptic bronchoscopy and literature review

Endobronchial hamartoma is a rare tumour. We report a 65-year-old woman with a history of recurrent pneumonia. Bronchoscopy revealed a 1 cm endobronchial mass obstructing the left upper lobe bronchus. Histopathological examination was consistent with a pulmonary hamartoma. This lesion was successfully debulked endoscopically with the use of a flexible cryoprobe without any complications. This case highlights both the importance of investigating recurrent pneumonia and the usefulness of endoscopic recanalisation in an obstructed segmental bronchus.

Sclerosi Laterale Amiotrofica (SLA): valutazione della disfagia e gestione degli aspetti nutrizionali

Background This research examines a number of patients with Amyotrophic Lateral Sclerosis (ALS), by evaluating the impact of specific dietary protocols in the malnutrition management. The study aims at verifying how the correct nutritional aspects management- through diet protocols and dysphagia assessment scales - can improve the nutrition and nutritional status of patients affected by this pathology, reducing the related complications and improving their life quality. Materials and Methods 11 patients diagnosed with ALS were examined and subjected to two different eating attitude screening tests (Eating Attitude Test 10; Northwestern Dysphagia Patient Check Sheet), which were repeated six months apart. Results The carried out evaluations showed an improvement (36% of the cases) in the history of recurrent pneumonia and in the frequency of feverish peaks. As regards the behavioural variables, improvements (36% of cases) in the awareness of dysphagia and in the management of salivary secretions have been observed. In 91% of cases there was a marked improvement in the delay of the pharyngeal reflex of swallowing. Discussion The results of the research, according to the scientific evidence of the sector, point out the importance of taking care of the nutritional status of patients in relation to their life expectancy and quality and highlight the value of constant monitoring of swallowing abilities to prevent associated complications (e.g. malnutrition and / or dehydration). Conclusion A correct mutlidisciplinary management, which includes a constant assessment of swallowing and the use of specific dietary protocols, improves the nutrition of patients with ALS, reducing the risk of complications development (e.g. malnutrition, aspiration during the meal) and improving the life quality of the patient. Key – words Dysphagia, Speech Therapy, Malnutrition, ALS.

Annals for Educators - 2 June 2020

Annals for Educators - 2 June 2020

Oral management of a patient with down syndrome and agammaglobulinemia: a case report

BackgroundDown syndrome is characterized by a variety of dysmorphic features and congenital malformations, such as congenital heart disease, gastrointestinal disease, and other conditions like leukemia and autoimmune disorders. Patients with Down syndrome are highly prone to respiratory tract infections, which might be fatal to them. However, there are only few available data on patients diagnosed with Down syndrome and agammaglobulinemia. In this report, we describe a case of successful prevention of post-dental treatment complications (e.g., pneumonia and other bacterial infections) in a patient with Down syndrome and agammaglobulinemia.Case presentationA 43-year-old man with Down syndrome, untreated agammaglobulinemia, and a history of recurrent pneumonia, was referred to our clinic for tooth mobility. To reduce the risk of post-operative infections, gammaglobulin treatment and prophylactic administration of antibiotics was scheduled before the dental procedure. Furthermore, the dental treatment, which included a filling and extractions, was conducted under general anesthesia and with the supervision of a hematologist. The dental procedures were successfully performed without any post-operative infection, and the patient is undergoing follow-up care.ConclusionsThe purpose of this case report was to recommend a close liaison between physicians and dentists who may encounter a similar case, and to emphasize the importance of improving oral health of immunodeficient patients to prevent infections caused by oral microbial flora.

Lifelong Idiopathic Unilateral Diaphragmatic Paralysis With Recurrent Pneumonia: A Case Report

A 50-year-old woman was admitted to the emergency center with dyspnea, cough, and fever symptoms. She had a medical history of diabetes mellitus type II, rheumatoid arthritis, as well as several admission records due to aspiration pneumonia. The primary diagnosis was diabetic ketoacidosis and pneumonia. Normal breath sounds were reduced on the lower posterior right side of the thorax. A standard chest x-ray and lung Computed Tomography (CT) scan revealed collapse consolidation in the Right Lower Lobes (RLL) and Right Middle Lobes (RML). We here presented a case of unilateral diaphragmatic paralysis with a history of recurrent pneumonia.

A 58-year-old woman with recurrent pneumonias and a pulmonary abscess

A 58-year-old female with a history of recurrent pneumonia was evaluated for fevers, right lower back pain, and hematuria. A noncontrast abdominal computed tomography (CT) scan showed air and fluid-filled area in the right lower lobe for which a contrast-enhanced CT chest was performed. The CT of the chest revealed the cystic mass was supplied by an anomalous artery from the descending aorta. The patient was then diagnosed with a superinfected bronchopulmonary sequestration which was treated with surgical resection.