Rapid Progression To Respiratory Failure Research Articles

Human viral encephalitis associated with suid herpesvirus 1.

BackgroundSuid herpesvirus type 1 (SHV1) is a type of neurotropic virus able to infect various species. However, the clinical cases of human SHV1 encephalitis are still rarely reported, and the clinical characteristics, treatment, and prognosis of human SHV1 encephalitis are still unclear.MethodsIn this study, we reported 2 cases of human encephalitis associated with SHV1 infection and reviewed the other 18 cases from the literatures. A total of 20 cases with human SHV1 encephalitis were summarized and re-analyzed.ResultsNineteen of 20 patients had a history of swine-related occupational exposure before illness onset. All patients initially presented with influenza-like symptoms and then developed seizures, disturbed consciousness, and endophthalmitis. All patients with clinical outcome of modified Rankin Scale of 5 or 6 suffered from rapid progressive respiratory failure. The results of cerebrospinal fluid (CSF) indicated aseptic or viral infection. MRI findings of SHV1 encephalitis were prone to distribute in temporal-frontal and insular cortex, which was similar to the pattern of herpes simplex virus encephalitis, while some cases with involvements of gray matter nuclei had a high rate of mortality. Metagenomic next-generation sequencing (mNGS) revealed that all patients had unique SHV1 sequences with variable reads in the CSF.ConclusionsThe variant SHV1 can cause a new type of human viral encephalitis, characterized by acute, fulminating, and catastrophic central nervous system infection. Rapid progressive respiratory failure and extensive lesions of deep gray matter nuclei might be indicators to poor prognosis. No approved treatments for the encephalitis are available, but it is possible to diagnose encephalitis quickly by mNGS.Supplementary InformationThe online version contains supplementary material available at 10.1007/s10072-021-05633-0.

Radiologically Undetectable Latent Pulmonary Lymphocyte Infiltration Suggesting the Need for Early Glucocorticoid Therapy: An Autopsy Case of Rapidly Progressive COVID-19 Pneumonia with Lymphopenia.

We herein report a fatal case of coronavirus disease 2019 (COVID-19) pneumonia with rapid progression of respiratory failure and lymphopenia. Excessive recruitment and sequestration of lymphocytes in the lung were suggested as the pathophysiology underlying COVID-19-associated lymphopenia. Interestingly, the autopsy in this case revealed lymphocytic infiltration in the lungs even at sites that appeared normal on autopsy imaging. These findings suggest that in COVID-19 cases with risk factors of severe exacerbation, early glucocorticoid administration should be considered, especially if lymphopenia is present, even if the imaging findings show only mild abnormalities.

Hermansky-Pudlak syndrome-associated pneumothorax with rapid progression of respiratory failure: a case report

BackgroundHermansky-Pudlak syndrome (HPS) is an extremely rare disease with pulmonary fibrosis (PF), oculocutaneous albinism, induced platelet dysfunction, and granulomatous colitis. Although patients with HPS-associated PF (HPS-PF) often receive treatment with anti-fibrotic agents, including pirfenidone, many HPS-PF cases are progressive. The development of pneumothorax is known to be rare in HPS-PF. Pneumothorax development is generally important for prognosis in patients with interstitial pneumonia. However, there are few reports regarding the development of pneumothorax in patients with HPS-PF.Case presentationA 50-year-old Japanese man with chestnut hair, white skin, and light brown squint eyes visited our hospital for interstitial pneumonia examination. Chest high-resolution computed tomography (HRCT) demonstrated diffuse bilateral reticular opacities along the bronchovascular bundles and traction bronchiectasis predominantly in the upper lung fields. He was definitively diagnosed with HPS because genetic analysis showed that he had a homozygous mutation, c.398 + 5G > A, in the HPS-1 gene. After diagnosis with HPS-PF, he initiated home oxygen therapy due to gradually progressive hypoxemia.Three months after the HPS-PF diagnosis, the patient suddenly developed severe chest pain and dyspnea and was admitted to our hospital on emergency. He was diagnosed with pneumothorax by chest radiological findings. He immediately received chest drainage; however, his pneumothorax did not improve. Therefore, he underwent video-assisted surgery by thoracic surgeons. The leak point was not detected, but multiple bullae were found, mainly in the upper lung lobes. Thus, the surgeons did not perform bullectomy and only covered the apical areas. Fifteen days after the surgery, the patient developed high fever and dyspnea with a new diffuse reticular shadow found through HRCT. We first initiated the patient on broad-spectrum antibiotics; however, the symptoms and radiological findings worsened. Therefore, we started treatment with pirfenidone for inhibition of PF progression. The patient re-developed pneumothorax with severe respiratory failure. Although he re-underwent chest drainage, he died of progressive respiratory failure.ConclusionsWe herein report the case of a rare HPS patient who developed pneumothorax with progressive PF. Pneumothorax may cause rapid progressive respiratory failure and may be associated with PF progression in HPS-PF.

Global rheumatology in the time of COVID-19.

Global rheumatology in the time of COVID-19.

Miller Fisher syndrome associated with respiratory failure

Miller Fischer syndrome (MFS) is a rare clinical variant of Guillain-Barre syndrome. The diagnosis suspected primarily on clinical trial of areflexia, ophtalmoplegia and ataxia, is confirmed by the mandatory presence of antiganglioside antibodies (anti GQ1b). An acute onset is typical of MFS, beginning with neurological symptoms following a respiratory or digestive infectious illness. The therapeutic options are either the plasmapheresis or the administration of intravenous immunoglobulin (IVIG). Although rare, in certain cases the patients present with respiratory symptoms needing intensive care. We report the case report of a patient which presented with the classical triad of MFS but also with rapid progressive respiratory failure due to bilateral vocal cords palsy and general muscle weakness. He needed respiratory mechanical support. The patient received a treatment by IVIG without any improvement in symptomatology. Eventually he completely recovered and he was discharged from the hospital 3 month later.

A Japanese patient with a VCP mutation c.290G > A (p.G97E) presenting a rapid progressive respiratory failure

AbstractInclusion body myopathy with Paget's disease of bone and frontotemporal dementia (IBMPFD) is an autosomal dominant disorder caused by mutations in the valosin‐containing protein (VCP) gene. IBMPFD usually presents progressive limb muscle weakness resulting in wheelchaired after mean course of 9 years with myopathy, and Paget's osteolytic lesions and frontotemporal dementia (FTD) rarely present respiratory failure. Different from previous reports, we reported a Japanese IBMPFD patient with a VCP mutation c.290G > A (p.G97E), presenting a progressive respiratory failure accompanied by wheelchaired in only 6 years after myopathy onset, suggesting unique relationship between clinical severity and c.290G > A (p.G97E) mutation of VCP gene in Japanese.

A DISTINCT KIND OF MORNING SICKNESS: WOUND BOTULISM IN A PREGNANT FEMALE

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: Wound botulism is caused by contamination with Clostridium Botulinum spores. When the spores germinate, they produce a potent neurotoxin causing the characteristic descending flaccid paralysis. Botulism in pregnancy may be amplified due to natural physiological changes. To the best of our knowledge, there have been only two other cases reported of pregnant women with wound botulism. CASE PRESENTATION: This is a 38-year-old female with hypertension, asthma, and 10-week intrauterine pregnancy who presented with two weeks of diplopia, dysphasia, dysarthria, anorexia, and ptosis followed by symmetric and progressive weakness in her legs. Physical exam revealed diminished patellar reflexes, weakness of bilateral lower extremities, and swollen and bruised forearms, without ultrasound evidence of fluid collection. Due to decreased vital capacity, she was intubated for airway protection. Initial serological and spinal fluid studies were normal. Brain and spine imaging were unremarkable. Electromyography (EMG) revealed gross reduction in motor response amplitudes suggestive of an axon loss variant of Guillan Barre syndrome. IVIG therapy was initiated. Due to progressive weakness and persistent ventilator support, the patient underwent tracheostomy. Further history provided by significant other confirmed IV drug use 2 weeks prior broadening the differential diagnosis. Acetylcholine receptor binding antibody was negative, yet botulism neurotoxin was positive and the heptavalent antitoxin was administered. Repeat EMG demonstrated progressive low amplitude compound muscle action potentials with minimal increment and early recruitment on rapid repetitive stimulation suggested the presence of a presynaptic neuromuscular junction defect consistent with botulism. The patient’s clinical status dramatically improved. Although her fetus was unharmed, she decided to terminate her pregnancy due to psychosocial issues. DISCUSSION: Unlike foodborne botulism, wound contamination requires spore germination before the toxin can be released resulting in longer incubation periods. The neurotoxin inhibits the release of acetylcholine from the motor neurons to the muscles. The blockade is most pronounced in the cranial nerves and muscles of respiration, sparing sensory innervation. Treatment must be initiated with a presumptive clinical diagnosis, as laboratory confirmation is timely. Prognosis is favorable with proper respiratory support and early recognition. There is minimal literature describing maternal and fetal outcomes, however congenital botulism or neonatal loss is deemed unlikely. CONCLUSIONS: Early recognition and neutralization of the neurotoxin is paramount to improve outcomes. Pregnant women physiologically have increased oxygen consumption causing rapid progression of respiratory failure. There are no known adverse maternal or fetal outcomes from treatment or infection of botulinum toxin. Reference #1: Burningham, Mark D, et al. “Wound Botulism.” Anals of Emergency Medicine 1994 Dec;24(6):1184-1187. Reference #2: Thwaites, C.L. "Botulism and tetanus.” Medicine 2017 Dec;45(12):739-742. Reference #3: Rimawi, Bassam H. "Botulism During Pregnancy and the Postpartum Period: A Systematic Review.” Clinical Infectious Disease 2018 Jan;66(Suppl 1):S30-S37. DISCLOSURES: No relevant relationships by Aarti Mittal, source=Web Response No relevant relationships by Erin Wiltchik, source=Web Response

The role of microRNAs in chronic pseudomonas lung infection in Cystic fibrosis.

Cystic Fibrosis (CF) is the most common life limiting genetic disorder, characterized by chronic respiratory failure secondary to inflammation and chronic bacterial lung infection. Pseudomonas aeruginosa lung infection is associated with more severe lung disease and rapid progression of respiratory failure when compared to Staphylococcus aureus infection. We hypothesized that a specific signature of epigenetic factors targeting specific gene transcripts contributes to the increased morbidity seen in CF patients with chronic Pseudomonas infection. We collected exhaled breath condensate (EBC) from 27 subjects and evaluated miRNA signatures in these samples using commercial PCR array. We identified predicted mRNA targets and associated signaling pathways using Ingenuity Pathway Analysis. We found 11 differentially expressed miRNAs in EBC of patients infected with Pseudomonas aeruginosa compared to EBC from CF patients who were not chronically infected with Pseudomonas aeruginosa (p < 0.05). Six of these miRNAs (hsa-miRNA-1247, hsa-miRNA-1276, hsa-miRNA-449c, hsa-miRNA-3170, hsa-miRNA-432-5p and hsa-miR-548) were significantly different in the CF Pseudomonas positive group when compared to both the CF Pseudomonas negative group and healthy control group. Ingenuity pathway analysis (IPA) revealed organismal injury and abnormalities, reproductive system disease and cancer as the top diseases and bio functions associated with these miRNAs. IPA also detected RELA, JUN, TNF, IL-10, CTNNB1, IL-13, SERPINB8, CALM1, STARD3NL, SFI1, CD55, RPS6KA4, TTC36 and HIST1H3D as the top target genes for these miRNAs. Our study identified 6 miRNAs as epigenetic factors specifically associated with chronic Pseudomonas infection in patients with CF.

Efficacy of concurrent treatments in idiopathic pulmonary fibrosis patients with a rapid progression of respiratory failure: an analysis of a national administrative database in Japan.

BackgroundSome IPF patients show a rapid progression of respiratory failure. Most patients are treated with high-dose corticosteroids. However, no large clinical studies have investigated the prognosis or efficacy of combined treatments including high-dose corticosteroids in IPF patients with a rapid progression of respiratory failure.MethodsWe enrolled IPF patients who received mechanical ventilation and high-dose corticosteroids between April 2010 and March 2013. Records were extracted from a Japanese nationwide inpatient database. We conducted a retrospective epidemiologic and prognostic analysis.ResultsTwo hundred nine patients receiving an average of 12.8 days of ventilatory support were enrolled. There were 138 (66 %) fatal cases; the median survival was 21 days. The short-term (within 30 days) and long-term (within 90 days) survival rates were 44.6 and 24.6 %, respectively. The average monthly admission rate among the IPF patients with the rapid progression of respiratory failure in the winter was significantly higher than that in spring (p = 0.018). Survival did not differ to a statistically significant extent in the different geographic areas of Japan. Survivors were significantly younger (p = 0.002) with higher rates of mild dyspnea on admission (p = 0.012), they more frequently underwent bronchoscopy (p < 0.001), and received anticoagulants (p = 0.027), co-trimoxazole (p < 0.001) and macrolide (p = 0.02) more frequently than non-survivors. A multivariate logistic analysis demonstrated that two factors were significantly associated with a poor prognosis: >80 years of age (OR = 2.94, 95 % Cl 1.044–8.303; p = 0.041) and the intravenous administration of high-dose cyclophosphamide (OR = 3.17, 95 % Cl 1.101–9.148; p = 0.033). Undergoing bronchoscopy during intubation (OR = 0.25, 95 % Cl 0.079–0.798; p = 0.019) and the administration of co-trimoxazole (OR = 0.28, 95 % Cl 0.132–0.607; p = 0.001) and macrolides (OR = 0.37, 95 % Cl 0.155–0.867; p = 0.033) were significantly associated with a good prognosis. The dosage of co-trimoxazole significantly correlated with survival.ConclusionsCo-trimoxazole and macrolides may be a good addition to high-dose corticosteroids in the treatment of IPF patients with a rapid progression of respiratory failure.

Neurogenic Pulmonary Edema and Acute Respiratory Distress Syndrome in a Healthy Child With Febrile Status Epilepticus

Neurogenic pulmonary edema is a clinical syndrome that manifests as an acute onset of pulmonary edema in the setting of a central nervous system injury, without cardiac dysfunction. Neurogenic pulmonary edema is rare in children, and the mechanism is still not completely understood. The clinical pathology overlaps with acute lung injury and acute respiratory distress syndrome. The authors report a case of a 14-month-old previously healthy child who presented with febrile status epilepticus, fulminant neurogenic pulmonary edema, and acute respiratory distress syndrome. Neurogenic pulmonary edema should be considered in the differential diagnosis for the rapid progression of respiratory failure following an acute neurological injury such as status epilepticus in a child. Prompt respiratory support and treatment of the acute neurological insult can prevent further cerebral hypoxemic injury.

Methicillin-resistant Staphylococcus lugdunensis carrying SCCmec type V misidentified as MRSA

Staphylococcus lugdunensis is a rare cause of severe infections and clinical manifestations are similar to those related to S. aureus infection. We describe a hospital-acquired bacteremia due to methicillin-resistant Staphylococcus lugdunensis, misidentified as methicillin-resistant S. aureus. The oxacillin MIC was 16 µg/mL and the mecA gene and SCCmec type V were determined by PCR. Although treatment had been appropriated, the patient died after rapid progressive respiratory failure and another nosocomial sepsis. It is important not only to identify S. lugdunensis in view of its clinical course, but also to determine its susceptibility to oxacillin by detecting the mecA gene or its product.

Incidence, risks, and outcome of idiopathic pneumonia syndrome early after allogeneic hematopoietic stem cell transplantation

We retrospectively analyzed 23 cases with early-onset idiopathic pneumonia syndrome (IPS) of 192 patients undergoing allogeneic hematopoietic stem cell transplantation (HSCT) from April 1997 to October 2007. Risk factors for IPS development were evaluated using Cox proportional hazards model, including age, gender, underlying disease, disease status at transplant, transplant type, conditioning regimens, donor type, acute graft-vs.-host disease (GVHD), severity of acute GVHD (aGVHD), human leukocyte antigen (HLA) disparity, and organ involvement of aGVHD. Factors that were significant at the 0.1 level on univariate analysis were evaluated by multivariate analysis. Twenty-three of 192 patients developed IPS (12.0%). Median time to IPS onset after allogeneic HSCT was 76 d (range 32-120 d); median time to death after the diagnosis of IPS was 9 d (range 3-92 d); 20 patients with IPS died because of the rapid progression of respiratory failure (87.0%). Nineteen patients with IPS developed aGVHD (82.6%), with grade III-IV aGVHD in 11 patients (47.8%) and aGVHD of gut in 16 patients (69.6%). The following six factors were associated with an increased risk of IPS by univariate analysis: not in remission, unrelated donor, HLA disparity, occurrence of aGVHD, grade III-IV aGVHD and aGVHD of gut. These risk factors were entered into a multivariate analysis model. Only unrelated donor, grade III-IV aGVHD and aGVHD of gut are identified as being significantly associated with the occurrence of IPS, and among them, aGVHD of gut was associated with the largest risk of IPS, suggesting that the lung may be a target organ of aGVHD.

Hantavirus Cardiopulmonary Syndrome

Hantavirus cardiopulmonary syndrome (HCPS) is caused by infection with multiple types of hantaviruses throughout the Americas. All strains induce a pulmonary capillary leak syndrome with cardiogenic shock in severe cases, and almost all strains have an overall mortality rate of 35%. Some strains in South America are commonly associated with either mild disease without pulmonary edema or severe disease with pulmonary hemorrhage. Early recognition during the thrombocytopenic prodrome phase and transport to intensive care improve survival, due to very rapid progression of respiratory failure and shock in some patients. Limited fluid replacement, early inotropic therapy, and mechanical ventilation also improve survival. Extracorporeal membrane oxygenation (ECMO) has been life saving for patients with refractory shock. Ribavirin does not appear to reduce mortality, but hyperimmune serum offers a promising future therapy because survival is correlated with higher neutralizing antibody titers at admission.

Bronchiolitis obliterans organizing pneumonia: experience at three hospitals in Riyadh.

BACKGROUNDBecause reports of bronchiolitis obliterans organizing pneumonia (BOOP) are lacking from the Middle East, we conducted a retrospective review of of all histopathologically proven cases of BOOP over a 10-year period at three tertiary care hospitals in Riyadh and describe the clinical features and outcome.METHODSCharts at the three hospitals were searched using a specific code for BOOP or cryptogenic organizing pneumonia (COP). Lung specimens had to show histological proof of BOOP with a compatible clinical picture. Chest radiographs and high-resolution CT scans were reviewed.RESULTSTwenty cases of biopsy-proven BOOP had well-documented clinical and radiographic data. There were 11 males and 9 females (mean age, 58 years; range, 42–78). The clinical presentation of BOOP was acute or subacute pneumonia-like illness with cough (85%), fever (70%) dyspnea, (85%) and crackles (80%). The most frequent radiological pattern was a bilateral alveolar infiltrate. The most common abnormality on pulmonary function testing (n=14) was a restrictive pattern (11 patients). Most patients (70%) had no underlying cause (idiopathic BOOP). Other associations included thyroid cancer, rheumatoid arthritis, syphilis and Wegner's granulomatosis. Ten patients (50%) had a complete response to steroids, 6 (30%) had a partial response and 3 (15.8%) with secondary BOOP had rapid progressive respiratory failure and died.CONCLUSIONThe clinical presentation of BOOP in our patients is similar to other reported series. A favorable outcome occurs in the majority of cases. However, BOOP may occasionally be associated with a poor prognosis, particularly when associated with an underlying disease.

Bronchiolitis obliterans-organizing pneumonia: an Italian experience

The purpose of this study was to describe the clinical features at onset and outcome and the diagnostic approach in subjects with bronchiolitis obliterans-organizing pneumonia (BOOP). Over a 7-year period we observed 78 cases of biopsy-proven bronchiolitis obliterans-organizing pneumonia, in which well documented clinical and radiographic data were available. The final diagnosis of BOOP was validated when patients presented: (i) negative microbiological analysis on BAL fluid; (ii) a well documented improvement either spontaneous, or after steroid treatment or (iii) cases with progressive respiratory failure and increasing radiographic shadows, an open lung biopsy or autopsy that excluded other entities. There were 42 males and 36 females; the mean age was 61±12 years (range 12–85 years). Forty-two (54%) patients were current smokers, 25 (32%) had never smoked and 11 (14%) were ex-smokers. The clinical pattern at presentation of BOOP was more frequently similar to classical acute or sub-acute infectious pneumonia. Fever (63%), dyspnoea (58%) and dry cough (53%) were the typical symptoms on admission. A flu-like syndrome preceeding BOOP was observed in 21 cases (27%). Inspiratory crackles (78%) were the most typical finding at physical examination. However, 13% of the patients were asymptomatic and an abnormal chest X-ray film was the reason for seeking medical attention. Radiographically the most frequent pattern of BOOP was a unilateral consolidation (44%) with lower field predominance. A migratory behaviour was present in 22% of the cases. High-resolution computed tomographic (HR-CT) scan when performed, was more sensitive in detecting ground glass infiltrates, sub-pleural or peri-bronchovascular distribution or the presence of nodules or cavitation.Most patients (68%) were classified as having idiopathic BOOP. However, the same clinical-roentgenological pattern was observed in patients after radiotherapy for ductal breast carcinoma (6%), in collagen-vascular diseases (6%), related to drugs (9%), to infections serologically documented (4%), and to graft vs. host disease (4%).Four patients (all of whom had idiopathic BOOP) presented a rapid progressive respiratory failure needing mechanical ventilation. In another two cases respiratory failure appeared after a long period during which patients experienced exertional dyspnoea and low grade fever.BAL profile was characterized by lymphocytosis with a reduction of the CD4/CD8 ratio, associated with a slight increase of neutrophils and eosinophils and scattered mast cells. However in two cases we had an increased CD4/CD8 ratio and in one case the presence of a significant 12% of polyclonal B cells. In a few cases atypical (cytokeratin-positive cells) epithelial cells were detected: these cells were constantly present in the BAL fluid of patients with rapidly progressive respiratory failure.From the diagnostic point of view this series documents that transbronchial lung biopsy (coupled with BAL) can be the first diagnostic step. However, therapy can be started on the basis of BAL data (when a characteristic morphological and phenotypical profile is evident) in cases in which the clinical presentation is suggestive and a biopsy cannot be made. Most patients showed a rapid and good response to steroid therapy. However, three patients died (4%) in spite of steroid therapy (two cases) and steroid and cyclophosphamide therapy (one case).In conclusion, although clinical findings, chest X-ray film and CT Scan findings usually suggest the diagnosis a definite confirmation requires transbronchial lung biopsy and BAL and, less frequently, open lung biopsy.

Facioscapulohumeral dystrophy associated with sensorineural hearing loss, tortuosity of retinal arterioles, and an early onset and rapid progression of respiratory failure

Two sibling cases of facioscapulohumeral dystrophy (FSHD) are described. One was characterized by sensorineural hearing loss, marked tortuosity of retinal arterioles, an early onset and progression of severe restrictive-type pulmonary dysfunction, and cor pulmonale. The other had a mild course of FSHD without involvement of any other organ than muscles at the time of diagnosis. Recently, a new nosological entity of FSHD, with sensorineural hearing loss and tortuosity of retinal arterioles, was advocated. Our cases, especially the first case, seem to belong to this newly recognized entity of FSHD. Moreover, it is noteworthy that our first case exhibited rapid aggravation of severe restrictive-type respiratory failure and cor pulmonale, leading to death, which was never seen in any other reported cases.