Recurrent Respiratory Failure Research Articles

Weaning failure due to isolated residual diaphragmatic paralysis after cervical spinal cord ischemia following aortic surgery- a case report

BackgroundBilateral diaphragmatic dysfunction can lead to dyspnea and recurrent respiratory failure. In rare cases, it may result from high cervical spinal cord ischemia (SCI) due to anterior spinal artery syndrome (ASAS). We present a case of a patient experiencing persistent isolated diaphragmatic paralysis after SCI at level C3/C4 following thoracic endovascular aortic repair (TEVAR) for Kommerell’s diverticulum. This is, to our knowledge, the first documented instance of a patient fully recovering from tetraplegia due to SCI while still exhibiting ongoing bilateral diaphragmatic paralysis.Case presentationThe patient, a 67-year-old male, presented to the Vascular Surgery Department for surgical treatment of symptomatic Kommerell’s diverticulum in an aberrant right subclavian artery. After successful surgery in two stages, the patient presented with respiratory insufficiency and flaccid tetraparesis consistent with anterior spinal artery syndrome with maintained sensibility of all extremities. A computerized tomography scan (CT) revealed a high-grade origin stenosis of the left vertebral artery, which was treated by angioplasty and balloon-expandable stenting. Consecutively, the tetraparesis immediately resolved, but weaning remained unsuccessful requiring tracheostomy. Abdominal ultrasound revealed a residual bilateral diaphragmatic paralysis. A repeated magnetic resonance imaging (MRI) 14 days after vertebral artery angioplasty confirmed SCI at level C3/C4. The patient was transferred to a pulmonary clinic with weaning center for further recovery.ConclusionsThis novel case highlights the need to consider diaphragmatic paralysis due to SCI as a cause of respiratory failure in patients following aortic surgery. Diaphragmatic paralysis may remain as an isolated residual in these patients.

The risk factors for silent aspiration: A retrospective case series and literature review.

Evidence shows that 20%-30% of patients who aspirate do so silently. Research to date has not demonstrated clear evidence to indicate which patients are at higher risk of silent aspiration. Our aim was to use univariate logistic regression analysis of retrospective case review to determine potential patterns of silent aspiration. We conducted a retrospective analysis of 455 fiberoptic endoscopic evaluation of swallowing (FEES) reports. The patients were divided into four groups: G1 - neurological diseases (n = 93), G2 - head and neck surgery (n = 200), G3 - gastroenterological diseases (n = 94) and G4 - other patients (n = 68). Data included the occurrence or absence of saliva penetration or aspiration, of silent fluid/solid food penetration or aspiration, type of penetration or aspiration, occurrence of cranial nerve paresis, radiotherapy and tracheostomy. Univariate logistic regression was used to evaluate independent risk factors of silent aspiration in the study population. Three models with different independent variables were considered. There is a statistically significant difference in the frequency of occurrence of silent penetration and aspiration within the groups (p < 0.001), with intraglutative being most frequent. Fluid and food penetration and aspiration correlated with saliva penetration and aspiration in all groups (p < 0.001). Cranial nerve paresis (IX and X), radiotherapy and tracheostomy correlate with saliva penetration and aspiration (p = 0.020 for cranial nerve paresis; p = 0.004 for radiotherapy; p < 0.001 for tracheostomy). One hundred and fifteen patients (45.81%) in the subgroup of patients with intraglutative aspiration had cranial nerve paresis (IX, X or IX-X). Patients who should be prioritised or considered to be at a higher need of instrumental swallowing evaluation are those with IX and X cranial nerve paresis, tracheostomy and those who have had radiotherapy, with saliva swallowing problems, especially after paraganglioma, thyroid and parathyroid glands and middle and posterior fossa tumour surgery. What is already known on the subject Clinical signs of penetration or aspiration include coughing, throat clearing and voice changes, while silent penetration or aspiration patients aspirate without demonstrating any clinical symptoms. The most common consequences of silent aspiration include aspiration pneumonia, recurrent lower respiratory tract infections and respiratory failure. Additionally, malnutrition and dehydration can be indicators of silent aspiration. Patients may unknowingly reduce their oral intake and lose weight. Retrospective studies have shown that 20%-30% of patients aspirate silently (e.g. patients after stroke, acquired brain injury, head and neck cancer treatment, prolonged intubation). Clinical examination of swallowing can miss up to 50% of cases of silent aspiration. What this paper adds to existing knowledge Currently, silent aspiration is often discussed in neurological literature, but its applications to head and neck surgery are limited. In this study, we identify head and neck surgery patients who should be prioritised or considered to be in higher need of instrumental swallowing evaluation due to a higher risk of silent aspiration. What are the potential or actual clinical implications of this work? Post-treatment structural changes can result in lower cranial nerve paresis (IX, X, XII) and face injury, in which vagus and glossopharyngeal nerves are injured. After tracheostomy and radiotherapy, patients with problems swallowing saliva need careful clinical examination, particularly cranial nerve examination.

Risk factors for recurrent respiratory tract infections and acute respiratory failure in children with spinal muscular atrophy.

Assessment of and intervention for sleep-disordered breathing and malnutrition are related to the prevention of recurrent respiratory tract infections (RRTIs) and acute respiratory failure (ARF) in children with spinal muscular atrophy (SMA). However, specific standards for sleep-disordered breathing and malnutrition in the prevention of RRTIs and ARF have not been clarified. The study aimed to identify the risk factors and predictive indices for RRTIs and/or ARF in children with SMA. In this retrospective study, the differences in clinical characteristics between patients with and without RRTIs and ARF were compared, and binary logistic regression analysis was carried out. The optimal cutoff points for positive predictors were obtained. SMA type 1 (odds ratio (OR) = 5.21, 95% confidence interval (CI) 1.50-18.17, p = 0.010) and the apnea-hypopnea index (AHI) (OR = 1.12, 95% CI 1.01-1.24, p = 0.026) were risk factors, while the body mass index z score (BMIz) (OR = 0.65, 95% CI 0.46-0.91, p = 0.013) and mean pulse oxygen saturation (MSpO2 ) (OR = 0.72, 95% CI 0.52-1.00, p = 0.049) were protective factors. A standard consisting of (i) MSpO2 < 96% and (ii) AHI > 10 events/h and/or BMIz < -1 predicted the occurrence of RRTIs and/or ARF in the next year with a sensitivity of 0.513 and a specificity of 0.957. SMA type 1, BMIz, AHI and MSpO2 should be used to estimate the risk of RRTI and/or ARF in children with SMA. MSpO2 < 96% combined with AHI > 10 events/h or BMIz < -1 should be used as the intervention standard.

Progressive Pulmonary Venous Stenosis and Recurrent Hypoxemic Respiratory Failure in a Lung Transplant Recipient

<h3>Introduction</h3> Pulmonary venous stenosis (PVS) is a rare complication of lung transplant (LT) that typically presents soon after surgery with pulmonary congestion at the site of reduced venous drainage. We describe a case of a 34-year-old LT recipient with an unremarkable initial postoperative course who later developed recurrent hypoxemic respiratory failure due to progressive PVS. <h3>Case Report</h3> The patient underwent an uneventful bilateral LT; he had no evidence of primary graft dysfunction, was extubated the day after LT, and was discharged 10 days later. A month after LT, a chest CT showed moderate narrowing of the right superior pulmonary vein (RSPV); the remaining PVs appeared unremarkable. A subsequent chest CT showed mild right interlobular septal thickening and an incidental left bronchial anastomotic dehiscence. He recovered from dehiscence repair but continued to present with intermittent respiratory failure and bilateral, lower lobe predominant, ground-glass opacities and consolidations. As the location of the consolidations did not correlate with the location of PV narrowing, pulmonary edema from reduced venous drainage was not suspected. However, CT5 months after LT showed increased narrowing of the RSPV as well as narrowing of the right inferior pulmonary vein (RIPV) and left superior and inferior pulmonary veins (LSPV, LIPV); follow-up CT showed complete absence of opacification of the right venous system. In collaboration with interventional cardiology, PV stenting was selected over re-transplant. A 3-D model of the patient's proximal pulmonary vasculature was printed to guide intervention. Angiography at 16 months after transplant revealed occlusion of the RSPV and RIPV, severe stenosis of the LIPV, and moderate-severe stenosis of the LSPV (6-7mm). A transseptal approach was used to perform balloon angioplasty with stenting (Palmaz biliary stents) of the LSPV first and the RIPV 3 months later. The patient was treated with dual anti-platelet therapy, reported significant symptomatic improvement, remains off of supplemental oxygen, and still has stable allograft function 21 months after PV stenting. <h3>Summary</h3> PVS is a well-described postoperative complication of LT; however, progressive stenosis of multiple PVs is unusual. PV stenting can improve venous drainage and relieve pulmonary congestion in patients with progressive PVS.

Alpha Thalassemia X-Linked Intellectual Disability Syndrome in an Infant with Developmental Delay and DYS Recurrent Respiratory Failure Confirmed by Whole-Exome Sequencing

Alpha Thalassemia X-Linked Intellectual Disability Syndrome in an Infant with Developmental Delay and DYS Recurrent Respiratory Failure Confirmed by Whole-Exome Sequencing

Treatment option for diaphragm dysfunction after cardiac surgery: a review and a clinical case

INTRODUCTION. Dysfunction of diaphragm postoperatively is associated with worsened quality of life and increased health care costs due to prolonged respiratory support and morbidity. After cardiac operations, dysfunction of diaphragm can take place in up to 10 % of patients but often remains underdiagnosed. At the same time, histologically revealed changes, such as muscle atrophy in the diaphragm, start within 12 hours of mechanical ventilation. The therapy of diaphragm weakness is not well established and has a limited efficacy. Current analytic review describes therapeutic option for treatment ventilator-induced diaphragm dysfunction with emphasis on electric neuromodulation of phrenic nerve. OBJECTIVE. To optimize respiratory function by bilateral electrostimulation of phrenic nerves on the neck level in patient with diaphragm dysfunction after cardiac operation. MATERIALS AND METHODS. 82-year-old female patient after elective aortic valve replacement was diagnosed with dysfunction of diaphragm and respiratory failure. These changes caused the weaning failure from respirator and led to prolonged ventilator support. On the 20, 22, 24 and 26 days of postoperative period we performed invasive bilateral phrenic nerve stimulation on the neck level for 10 minutes with ultrasound navigation. RESULTS. After electrical modulation of phrenic nerves, diaphragm excursion, detected by ultrasound, was improved. After four procedures, duration of spontaneous breathing trial increased from 1 to 12 hours. On postoperative day 30, the patient was successfully weaned from respiratory support and decannulated without recurrent respiratory failure. There were no any complications during procedure, it was well-tolerated and associated with emotional improvement. CONCLUSIONS. Phrenic nerve stimulation on the neck level can be a valuable option in selected patients with respiratory failure and difficulties with ventilator weaning.

Is PM2.5 associated with emergency department visits for mechanical ventilation in acute exacerbation of chronic obstructive pulmonary disease?

Patients with chronic obstructive pulmonary disease (COPD) can have recurrent exacerbations and acute respiratory failure (ARF) triggered by particulate matter with a diameter of ≤2.5μm (PM2.5). To prevent ventilator shortages, this study investigated the short-term association between PM2.5 concentration and emergency department visits (EDVs) among patients with acute exacerbation of COPD (AECOPD) requiring mechanical ventilation (MV). We conducted a time-series study to predict the PM2.5 concentration and number of ventilators needed. Daily counts of EDVs among AECOPD patients requiring ventilation from 2015 to 2019 were obtained from a hospital. Generalized linear models extending Poisson regression were used to explore the association of AECOPD with PM2.5 after controlling for the time trend, seasonal variations, and meteorological variables. Eight hundred seventy-five AECOPD patients receiving MV were recorded, of whom 734 received noninvasive ventilation and 141 received invasive ventilatory support. EDVs for AECOPD patients with ARF significantly increased by 3.5% (95% confidence interval [CI]: 2.51%-4.42%) per 10μgm-3 increase in PM2.5 concentration. Among seasons, PM2.5 concentration had the strongest effect on AECOPD patients with ARF in spring (<24.5°C), with a 1.64% (95% CI: -0.56% to 3.83%) increase in admissions per 10μgm-3 increase in same-day PM2.5 concentration. The interquartile range increase of 20μgm-3 between winter and spring was associated with an average EDV increase of 48.66%. This is the first study to predict the number of ventilators required by calculating quantitative estimates of the short-term effects of PM2.5 on EDVs for AECOPD patients with ARF. Adverse effects of PM2.5 on AECOPD patients requiring MV are evident, especially in the spring. Establishing protective standards and reducing the PM2.5 concentration to below various thresholds are urgently needed.

IS ACUTE FIBRINOUS AND ORGANIZING PNEUMONIA AN EMERGING CAUSE OF DIFFUSE ALVEOLAR HEMORRHAGE?

TOPIC: Diffuse Lung Disease TYPE: Medical Student/Resident Case Reports INTRODUCTION: Acute fibrinous and organizing pneumonia (AFOP) is a rare and poorly understood form of interstitial lung disease that can present either acutely or insidiously. Among the acute manifestations of AFOP, diffuse alveolar hemorrhage (DAH) has only been described once before in the literature. CASE PRESENTATION: A 25 year old male with a history of end stage renal disease secondary to bilateral posterior urethral valves (on hemodialysis) and cutaneous neutrophilic dermatosis (Sweet syndrome) presented with the abrupt onset of severe dyspnea. Given concern for flash pulmonary edema on initial chest radiograph (Figure 1), he was placed on a nitroglycerine drip and underwent emergent hemodialysis with ultrafiltration. He subsequently developed rapidly progressive acute hypoxic respiratory failure with worsening pulmonary infiltrates (Figure 2) requiring emergent intubation and mechanical ventilation. Copious bloody secretions were noted during intubation, and the patient required multiple blood transfusions following admission. Despite mechanical ventilation with paralysis and proning, the patient developed severe adult respiratory distress syndrome with a P:F ratio nadir of 74. Chest CT revealed diffuse severe consolidative lung injury of unclear etiology (Figure 3). Bronchoscopy with serial aliquots on bronchoalveolar lavage confirmed the presence of diffuse alveolar hemorrhage. The patient’s condition improved rapidly with initiation of systemic glucocorticoids, and he was subsequently extubated on hospital day 5. Shortly after discharge home, the patient was readmitted with recurrent severe acute hypoxic respiratory failure in the setting of tapering his glucocorticoids, but he was successfully extubated within 24 hours following reinitiation of high-dose systemic glucocorticoids. Bronchoscopically-guided cryobiopsy during this admission revealed a diagnosis of AFOP, and the patient was ultimately discharged home with plans for a prolonged course of steroids and possible transition to steroid-sparing agents. DISCUSSION: Further investigations are needed to determine whether AFOP represents a distinct clinical entity, or rather the sequelae of concomitant inflammatory or autoimmune processes such as those present in this patient. Regardless of the etiology, data from case reports suggest that AFOP can be highly responsive to systemic glucocorticoids and other forms of immunosuppression. Delay in diagnosis, however, remains a significant barrier to the treatment of AFOP and portends poor prognosis in the acute form of this illness. CONCLUSIONS: The addition of this case to the literature suggests that DAH may represent an emerging manifestation of AFOP. Prompt recognition of this rare and heterogeneous disease process is imperative, as increasing evidence suggests favorable outcomes with early initiation of systemic glucocorticoids. REFERENCE #1: Crowley N, Sessler C, Miller K, Robila V, Debesa, O. Relapsing acute fibrinous and organizing pneumonia complicated by diffuse alveolar hemorrhage. Chest. 2018;154(4)(suppl):409A-410A. doi: 10.1016/j.chest.2018.08.375. REFERENCE #2: Lee JH, Yum HK, Jamous F, Santos C, Campisi A, Surani S, Lococo F, Goo JM, Yoon SH. Diagnostic procedures and clinico-radiological findings of acute fibrinous and organizing pneumonia: a systematic review and pooled analysis. Eur Radiol. 2021 Mar 31. doi: 10.1007/s00330-021-07868-z. Epub ahead of print. REFERENCE #3: Santos C, Oliveira RC, Serra P, Baptista JP, Sousa E, Casanova P, Pimentel J, Carvalho L. Pathophysiology of acute fibrinous and organizing pneumonia - Clinical and morphological spectra. Pathophysiology. 2019 Sep-Dec;26(3-4):213-217. doi: 10.1016/j.pathophys.2019.04.001. DISCLOSURES: No relevant relationships by Andrew Davis, source=Web Response No relevant relationships by Nathan Sandbo, source=Web Response

P-PN003. Refractory chronic inflammatory demyelinating polyneuropathy treated with subcutaneous bortezumib

Introduction. A 73-year-old man with underlying diabetes presented in May 2017 with progressive bilateral upper and lower limb weakness and numbness for 3 months. Examination revealed power of 2-3 on lower limbs and 3 on upper limbs. Reflexes were absent with gloves and stocking sensory loss. NCS showed diffused sensorimotor demyelinating polyneuropathy. Monoclonal IgG kappa was detected. Urine free light chain showed raised kappa. However, BMAT showed no evidence of myeloma. Abdominal fat pad biopsy showed no evidence of amyloidosis. Anti-ganglioside and paraneoplastic antibodies were not detected. Sural nerve biopsy showed axonal loss. CT NTAP was unremarkable. He responded initially with IVIG therapy but In July 2017, he had a relapse with worsening lower limb weakness. He was given IVIG 1 g/kg maintenance together with dexamethasone 4 weekly pulse therapy with some improvement. In Oct 2017, he presented again with worsening weakness. Besides, there was dysphagia and dysphonia. He developed type 2 respiratory failure requiring NIV and ICU admission, requiring 2 weekly plasmapheresis to rescue him from recurrent respiratory failure. IV Rituximab was tried but with little success even after 1 month of completion. Subcutaneous Bortezumib was given for 2 courses; Feb 2018 and Aug 2018. Results. After first course of Bortezumib, he improved significantly; with no more dysphagia and respiratory failure. He was subsequently discharged in Apr 2018, since then on monthly maintenance plasmapharesis and later 3 monthly. Upon latest review in Nov 2019, he was able to ambulate independently; muscle power improved with upper limbs proximal 5, distal 3; lower limbs proximal 5, distal 4. NCS showed improvement of motor CMAP especially in upper limbs. Conclusion. Bortezumib can be considered in refractory CIDP despite limited experience in the literature. However it should be reserved after conventional therapies have failed due to its neurotoxic side effects.

The usefulness of the transesophageal Doppler in the anesthetic management of Morgagni hernia repair - A case report

Morgagni hernia in adults is a rare condition and is associated with complications such as recurrent chest infections, respiratory failure and cardiac tamponade. Its correction represents an anesthetic challenge. Intraoperative esophageal Doppler provides important information in real time about the patient’s cardiac function and vascular filling, aiding in decision making by the anesthesiologist. We report a case of a 70–year–old woman, ASA Physical Status–III, and presented with chest pain, dyspnea and hypoxemia. Her chest x-ray revealed opacification of 2/3 of the left hemithorax and 1/2 of the right. Computed tomography confirmed a bilateral Morgagni hernia. The patient underwent immediate corrective surgery. Intraoperative cardiovascular function was monitored with esophageal Doppler. Compression and decompression of thoracic structures produces enormous hemodynamic and respiratory impact. After herniated contents removal it showed an increase in systolic volume and cardiac index and a correction of flow time. Transient arterial hypotension was verified, requiring fluid therapy and vasopressor support. Diaphragmatic defect was corrected and general hemodynamic stabilization was achieved. Patient was discharged asymptomatic. The esophageal Doppler was important in this case because it allowed us to measure fundamental hemodynamic variables in real time, such as cardiac index or systolic volume, and to subsequently act accordingly.&#x0D; Key words: Esophageal; Doppler; Morgagni hernia; Anesthesia&#x0D; Citation: Babo NM, Monteiro MT, Santos AB, Carreira CR. The usefulness of the transesophageal Doppler in the anesthetic management of Morgagni hernia repair - A case report. Anaesth pain intensive care 2021;25(2):206-211. DOI: 10.35975/apic.v25i2.1467 &#x0D; Received: 19 November 2020, Reviewed: 22 December 2020, Accepted: 12 January 2021

Clinical and Immunological Features of 96 Moroccan Children with SCID Phenotype: Two Decades' Experience.

Severe combined immunodeficiency (SCID) is a heterogeneous group of primary immunodeficiency diseases (PIDs) characterized by a lack of autologous T lymphocytes. This severe PID is rare, but has a higher prevalence in populations with high rates of consanguinity. The epidemiological, clinical, and immunological features of SCIDs in Moroccan patients have never been reported. The aim of this study was to provide a clinical and immunological description of SCID in Morocco and to assess changes in the care of SCID patients over time. This cross-sectional retrospective study included 96 Moroccan patients referred to the national PID reference center at Casablanca Children's Hospital for SCID over two decades, from 1998 to 2019. The case definition for this study was age < 2years, with a clinical phenotype suggestive of SCID, and lymphopenia, with very low numbers of autologous T cells, according to the IUIS Inborn Errors of Immunity classification. Our sample included 50 male patients, and 66% of the patients were born to consanguineous parents. The median age at onset and diagnosis were 3.3 and 6.5months, respectively. The clinical manifestations commonly observed in these patients were recurrent respiratory tract infection (82%), chronic diarrhea (69%), oral candidiasis (61%), and failure to thrive (65%). The distribution of SCID phenotypes was as follows: T-B-NK+ in 44.5%, T-B-NK- in 32%, T-B+NK- in 18.5%, and T-B+NK+ in 5%. An Omenn syndrome phenotype was observed in 15 patients. SCID was fatal in 84% in the patients in our cohort, due to the difficulties involved in obtaining urgent access to hematopoietic stem cell transplantation, which, nevertheless, saved 16% of the patients. The autosomal recessive forms of the clinical and immunological phenotypes of SCID, including the T-B-NK+ phenotype in particular, were more frequent than those in Western countries. A marked improvement in the early detection of SCID cases over the last decade was noted. Despite recent progress in SCID diagnosis, additional efforts are required, for genetic confirmation and particularly for HSCT.

Costs Associated With Lobectomy for Lung Cancer: An Analysis Merging STS and Medicare Data

Costs related to care of patients who undergo lobectomy for lung cancer may vary depending on patient, disease, and treating facility characteristics. We aimed to identify underlying case mix factors that contribute to variability of 90-day costs of lobectomy for early-stage lung cancer. The Society of Thoracic Surgeons General Thoracic Surgery Database was queried for lobectomy for clinical stage I lung cancer (2008-2013). Demographics, clinical outcomes, and 90-day episode-of-care costs across all care settings were analyzed for patients successfully linked to Medicare data. Hospital costs were estimated from charges using cost-to-charge ratios. Comprehensive regression models were created to identify impact of preoperative patient factors and hospital characteristics on costs, and to delineate additive costs due to perioperative outcomes and complications. The mean 90-day cost for lobectomy was $45,080 ± $38,239. Variables associated with significant additive costs were age greater than or equal to 75 years, American Society of Anesthesiologists classification III or IV, forced expiratory volume in 1 second less than 80% predicted, body mass index less than 18.5 or greater than 35, current or past smoker, cerebrovascular disease, chronic kidney disease, impaired functional status, open thoracotomy, prolonged operative time, government hospitals, metropolitan setting, and geographic location. Patients with 1 or more postoperative complication resulted in an overall mean added cost of $27,259. Added costs increased with the number of complications; isolated recurrent laryngeal nerve paresis ($3,911) and respiratory failure ($35,011) were associated with the least and most additive cost, respectively. Lobectomy is associated with substantial variability of episode-of-care costs. Variability is driven by patient demographic and clinical factors, hospital characteristics, and the occurrence and severity of complications.

A CASE OF ACUTE SILICOSIS WITH SEVERE PULMONARY HYPERTENSION

SESSION TITLE: Medical Student/Resident Occupational and Environmental Lung Disease SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Silicosis occurs from inhalation of silica dust particles. Acute and accelerated silicosis share nodular formations as seen in chronic silicosis but have granular lipo-proteinaceous collections in the alveolar spaces [1]. These collections impair gas exchange resulting in cyanosis, and ultimately respiratory failure usually exacerbated by respiratory infections. We report a case of a young patient developing acute silicosis from working in a gold mine. CASE PRESENTATION: A 30-year-old African man presented with a 2-week history of worsening shortness of breath associated with productive cough and pleuritic chest pain. The patient was previously diagnosed with progressive massive fibrosis (PMF) and silicosis via lung biopsy at an outside hospital, attributed to a 3-year long silica exposure from gold mining in West Africa. Our patient was initiated on intravenous glucocorticoids, broad spectrum antibiotics, and noninvasive positive pressure ventilation for acute hypoxic and hypercarbic respiratory failure. Chest radiograph revealed extensive bilateral airspace opacities (figure 1). CT-chest showed extensive bilateral perihilar parenchymal opacities with traction bronchiectasis, consistent with PMF (figure 2)(figure 3). Transthoracic echocardiogram showed septal wall abnormalities and a right ventricular systolic pressure of 173 mmHg measured via tricuspid regurgitant jet. The patient’s condition stabilized during hospitalization and the patient was discharged home on prednisone and oxygen supplementation via nasal cannula. Three weeks after discharge, the patient presented again with recurrent acute respiratory failure with a positive viral PCR for RSV requiring intubation and mechanical ventilation. Shortly after intubation, the patient went into cardiac arrest and expired. DISCUSSION: Generally, silicosis presents as a chronic condition but rarely, acute silicosis, most often related to massive exposure, can present within a few years . Lung transplantation is the only definitive treatment of advanced silicosis with studies showing successful lung transplantation with the 6-month, 1-year and 3-year survival rates of 86, 86, and 76 percent respectively [2]. One case report examined initiation of intravenous glucocorticoid therapy followed by oral prednisone which showed transient improvement in chest radiography and pulmonary function tests [3]. Unfortunately, treatment with glucocorticoid therapy was unable to alter the outcome of our patient. Lung transplantation was in the process for the patient but was unable to be obtained. CONCLUSIONS: This case presents a rare case of acute silicosis/PMF with severe pulmonary hypertension, who developed respiratory failure after RSV infection leading to death. Reference #1: Ozkan M, Ayan A, Arik D, Balkan A, Ongürü O, Gümüş S. FDG PET findings in a case with acute pulmonary silicosis. Ann Nucl Med 2009;23:883–886. Reference #2: Singer, J. P., et al. “Survival Following Lung Transplantation for Silicosis and Other Occupational Lung Diseases.” Occup Med (Lond) 62.2 (2012): 134-7. Print. Reference #3: Goodman, George B., et al. “Acute Silicosis Responding to Corticosteroid Therapy.” Chest 101.2 (1992): 366-70. Print. DISCLOSURES: No relevant relationships by Khaled Abu-Ihweij, source=Web Response No relevant relationships by Sung Choi, source=Web Response no disclosure on file for Keith Guevarra; No relevant relationships by John Wisener, source=Web Response

Cause of death in spontaneous intracerebral hemorrhage survivors: Multistate longitudinal study.

To determine the leading causes of death in intracerebral hemorrhage (ICH) survivors, we used administrative data from 3 large US states to identify adult survivors of a first-time spontaneous ICH and track all hospital readmissions resulting in death. We performed a longitudinal analysis of prospectively collected claims data from hospitalizations in California (2005-2011), New York (2005-2014), and Florida (2005-2014). Adult residents admitted with a nontraumatic ICH who survived to discharge were included. Patients were followed for a primary outcome of any readmission resulting in death. The cause of death was defined as the primary diagnosis assigned at discharge. Multivariable Cox proportional hazards and multinomial logistic regression were used to determine factors associated with the risk for and cause of death. Of 72,432 ICH survivors (mean age 68 years [SD 16], 48% female), 12,753 (18%) died during a median follow-up period of 4.0 years (interquartile range 2.3-6.3). The leading causes of death were infection (34%), recurrent intracranial hemorrhage (14%), cardiac disease (8%), respiratory failure (8%), and ischemic stroke (5%). Death in patients with atrial fibrillation (AF) was more likely to be caused by ischemic stroke (odds ratio [OR] 2.4, 95% confidence interval [CI] 1.9-2.9, p < 0.001) and less likely to be caused by recurrent intracranial hemorrhage (OR 0.7, 95% CI 0.6-0.8, p < 0.001) compared to patients without AF. Infection is the leading cause of death in all ICH survivors. Survivors with AF were at increased risk for death from ischemic stroke. These findings will help prioritize interventions aimed to improve long-term survival and recovery in ICH survivors.

Clinical Profile and Outcome of Pediatric Mitochondrial Myopathy in China.

Introduction: Mitochondrial myopathy in children has notable clinical and genetic heterogeneity, but detailed data is lacking.Patients and Methods: In this study, we retrospectively reviewed the clinical presentation, laboratory investigation, genetic and histopathological characteristics, and follow-ups of 21 pediatric mitochondrial myopathy cases from China.Results: Twenty-four patients suspected with mitochondrial myopathy were enrolled initially and 21 were genetically identified. Fourteen patients were found to harbor mitochondrial DNA point mutations (14/21, 66.7%), including m.3243A>G (9/15, 60%), m.3303C>T (2/15, 13.3%), m.3302A>G (1/15, 6.7%), m.3250T>C (1/15, 6.7%), m.3251A>G (1/15, 6.7%), of whom 12 patients presented with progressive proximal mitochondrial myopathy (12/14, 85.7%). Three patients revealed large-scale deletion in blood or muscle tissue (3/21, 14.3%), presenting with Kearns-Sayer syndrome (1/3, 33.3%) or chronic progressive external ophthalmoplegia (2/3, 66.7%). Four patients were found to harbor pathogenic nuclear gene variants (4/21, 19.0%), including five variants in TK2 gene and two variants in SURF1 gene. During the follow-ups up to 7 years, 10 patients developed cardiomyopathy (10/21, 47.6%), 13 patients occurred at least once hypercapnic respiratory failure (13/21, 61.9%), six experienced recurrent respiratory failure and intubation (6/21, 28.6%), eight patients failed to survive (8/21, 38.1%). With nocturnal non-invasive ventilation of BiPAP, three patients recovered from respiratory failure, and led a relative stable and functional life (3/21, 14.3%).Conclusion: Mitochondrial myopathy in children has great clinical, pathological, and genetical heterogeneity. Progressive proximal myopathy is most prevalent. Mitochondrial DNA point mutations are most common. And respiratory failure is a critical risk factor of poor prognosis.

Novel destabilizing Dynactin variant (DCTN1 p.Tyr78His) in patient with Perry syndrome

IntroductionPerry syndrome, also recognized as Perry disease, is a rare autosomal dominant disorder characterized by midlife-onset atypical parkinsonism, apathy or depression, respiratory failure and weight loss caused by a mutation in the Dynactin (DCTN1) gene. Case descriptionA fifty-six years-old adopted male presented with atypical parkinsonism with bradykinesia and postural instability, apathy, weight loss, and recurrent respiratory failure due to central hypoventilation requiring tracheostomy. Methods and resultsClinical workup revealed a novel DCTN1 p.Tyr78His variant. Using bioinformatic protein structure modeling, we compare our patient's variant to known DCTN1 mutations and predict protein stability of each variant at the CAP-Gly domain of p150Glued. All eight variants causing Perry syndrome, as well as Tyr78His, are located at site expected to interact with MAPRE1 tail and are predicted to be destabilizing. Variants causing atypical parkinsonism with incomplete Perry syndrome phenotype (K56R and K68E) are not significantly destabilizing in silico. ConclusionWe propose p.Tyr78His as the ninth pathogenic DCTN1 variant causing Perry syndrome. Bioinformatic protein modeling may provide additional window to understand and interpret DCTN1 variants, as we observed non-destabilizing variants to have different phenotype than destabilizing variants.

Does the Hyper IgM Phenotype Affect Prognosis in Ataxia Telangiectasia?

Objective: To evaluate the characteristics of the patients who were followed-up with the diagnosis of ataxia telangiectasia (AT) and to assess the relationship between the hyper IgM (HIGM) phenotype and their prognosis. Materials and Methods: From 2007 to 2019, the study included 68 patients aged 3-35 years who were followed-up with the diagnosis of AT. We retrospectively evaluated the clinical and immunological characteristics and follow-up results. Results: There were 36 girls and 32 boys with a median follow-up of 10 years (1-12 years). The most common complaints upon admission were unsteady walk in 87%, infection in 6%, presence of a family history in 6%, and intracranial mass in 1%. The marriage was consanguineous in 85% of the parents. Ataxia was seen in 100% of the patients, telangiectasia in 97%, and immune deficiency in 88%. Bronchiectasis was observed in 23.5% of the patients, chronic diarrhea in 19%, lymphoproliferation in 15%, malignancy in 10%, autoimmunity in 10%, liver failure in 6%, and granulomatous skin lesions in 6%. Thirteen patients (19%) died during follow-up. The HIGM phenotype was identified in 31% of the patients. Recurrent upper and lower respiratory tract infections (p=0.004 and p<0.0001, respectively), liver failure (p=0.005), and autoimmune diseases (p=0.023) were significantly higher in the HIGM (+) group than the HIGM (-) group. Life expectancy was shorter in the HIGM (+) group with 14 ± 0.73 years (CI 95% 12.55-15.44) compared to the HIGM (-) group with 18 ± 1.64 years (CI 95% 14.77-21.22) (p=0.054). Conclusion: During the early childhood period and before the characteristic findings of AT develop, the patients might present at a hospital with infections, autoimmunity, lymphoproliferation, or malignancy. Physical examination, high alpha-fetoprotein (AFP) levels and immunological testing provide important data for the correct diagnosis. The HIGM phenotype aggravates the clinical course of the disease resulting in fatalities at an earlier age and at a higher rate.

Tiroides endotorácico. Indicaciones del abordaje endotorácico

Introducción y objetivo: La incidencia de bocio endotorácico entre los pacientes sometidos a tiroidectomía oscila entre el 6% y el 30%. Aunque el abordaje cervical es suficiente en la mayoría de los casos, el abordaje endotorácico puede ser necesario en el 1-5.5% de los pacientes. Por lo que es recomendable anticiparlo. El objetivo del presente artículo es describir los factores predictivos de necesidad de este tipo de abordaje. Síntesis: Los principales factores de riesgo para la realización de esternotomía en los casos de bocio subesternal son la extensión por debajo del cayado aórtico y la localización retrotraqueal o retrovascular. El abordaje torácico será igualmente necesario en bocios ectópicos mediastínicos sin conexión con el tiroides cervical y se recomendará la valoración por un cirujano torácico en casos de bocio olvidado, así como en pacientes con antecedentes de radioterapia o cirugía cervical y sospecha de malignidad con afectación extra-tiroidea. La elección del abordaje dependerá de la localización, del tamaño de la masa y su relación con los órganos vecinos; los abordajes mínimamente invasivos pueden ser alternativas seguras a la esternotomía o la toracotomía. Las complicaciones postoperatorias más frecuentes de la resección de tiroides endotorácico son típicas de la cirugía tiroidea: parálisis recurrencial temporal o permanente, hipoparatiroidismo, insuficiencia respiratoria y sangrado postoperatorio. Conclusiones: Hasta en un 5% de las tiroidectomías, puede ser necesario un abordaje torácico, por lo que es conveniente anticiparlo. El abordaje torácico será necesario en casos de bocio con extensión por debajo del cayado, localización retrotraqueal o retrovascular y en bocios mediastínicos sin conexión con el tiroides cervical.

RECURRENT ARDS IN THE SETTING OF POLYSUBSTANCE ABUSE

SESSION TITLE: Wednesday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/23/2019 09:45 AM - 10:45 AM INTRODUCTION: Drug abuse remains a significant social problem in many countries. Adverse pulmonary complications of substance abuse are well documented, including diffuse alveolar haemorrhage, aspiration pneumonitis, and opportunistic infections related to HIV and hepatitis. Acute lung injury and acute respiratory distress syndrome are well known complications of narcotic overdose, but ARDS from drug use without overdose is a much rarer entity. [1] CASE PRESENTATION: We report a case of a 39 year old female, current 10 pack-year smoker, with a history of polysubstance abuse including heroin, cocaine and alcohol, who was admitted 5 times in 8 months with recurrent episodes of dyspnea, fever, myalgias and fatigue. Each time she presented with hypoxic respiratory failure and bilateral infiltrates on chest imaging, and required high flow oxygen and BIPAP. She was treated with IV antibiotics, steroids and diuresis with good response. An extensive workup was performed including negative cultures, negative connective tissue disease serologies, and negative HIV testing. Echocardiogram showed a normal ejection fraction and negative bubble study. CT scan during the first hospitalization showed diffuse bilateral ground-glass opacities with bilateral lower lobe predominance and subpleural sparing without bronchiectasis or subpleural honeycombing. Outpatient PFTs showed only a moderately decreased diffusing capacity; methacholine challenge test showed mild bronchial hyperresponsiveness. Patient underwent bronchoscopy during one hospitalization that showed grossly normal airways, neutrophil predominant BAL and no eosinophilia; endobronchial biopsy showed atypical bronchial epithelium favoring reactive process. Surgical lung biopsy revealed rare intracapillary granulomas with exogenous polarizable material – microcrystalline cellulose consistent with IV drug injection. DISCUSSION: Pulmonary foreign body granulomatosis is described in patients who inject oral drugs intravenously. [2-3]. This would not account for the fulminant respiratory failure with ARDS in this patient. A case series of 6 patients using narcotics, gabapentin, and other psychoactive drugs, with presentation similar to our patient, with recurrent ARDS and diffuse alveolar damage on lung biopsy, has been reported [4]. DAD was not identified on this patient’s surgical lung biopsy, perhaps because the patient was biopsied in between recurrences. Management of this condition is unclear with some reports indicating a potential benefit from systemic glucocorticoids [5]. Some patients with recurrent ARDS progress to worsening fibrosis and may eventually need lung transplant. Our patient has been managed with recurrent prolonged prednisone taper, but further treatment is complicated by her social issues and medication noncompliance. CONCLUSIONS: DAD is pathologic manifestation of severe acute lung injury, recurrence can be attributed to polysubstance abuse. Reference #1: [1] Benson MK, Bentley AM. Lung disease induced by drug addiction. Thorax. 1995; 50(11): 1125–1127. Reference #2: [2]Bishay A, Amchentsev A, Saleh A, Patel N, Travis W, Raoof S. A hitherto unreported pulmonary complication in an IV heroin user. Chest 2008;133(2)549-51. Reference #3: [3] Radow SK, Nachamkin I, Morrow C, et al. Foreign body granulomatosis. Clinical and immunologic findings. Am Rev Respir Dis 1983;127:575. [4] Savici D, Katzenstein A-LA. Diffuse alveolar damage and recurrent respiratory failure: report of 6 cases. Human Pathology 2001;32(12):1398-402. [5] Chau CH, Yew WW, Lee J. Inhaled budesonide in the treatment of talc-induced pulmonary granulomatosis. Respiration. 2003;70(4):439. DISCLOSURES: No relevant relationships by Moayad Al Sona, source=Web Response No relevant relationships by Sandrine Hanna, source=Web Response No relevant relationships by Margaret Zambon, source=Web Response

Fulminant Presentation of Systemic Lupus Erythematous in A Four Year Old Girl

Systemic Lupus Erythematous (SLE) is a chronic systemic disease which can involve multiple organs such as skin, kidney, and brain. Lung is another organ that can be affected. Pulmonary complications including pleuritis, pneumonitis, infectious pneumonia, pulmonary hemorrhage, pulmonary hypertension and pneumothorax have been reported in patients with SLE. Pulmonary involvement is relatively frequent in adult patients. It has infrequently been reported in children with SLE. However, pulmonary manifestations may be an initial and/or life-threatening complication of SLE. We report here a 4 year old Saudi girl who presented with prolonged fever and respiratory manifestations due to acute lupus pneumonitis associated with pulmonary Tuberculosis (TB) then developed recurrent pulmonary hemorrhage and acute respiratory failure.