Early Acoustic Warning for the Onset of Acute Chest Syndrome in Sickle Cell Patients

Acute chest syndrome (ACS) is a leading cause of death for those with sickle cell disease (SCD). ACS is defined by the development of a new pulmonary infiltrate on chest X-ray, with fever and respiratory symptoms. Efforts have been made to apply various technologies in the hospital setting to provide earlier detection of ACS than X-ray, but they are expensive, increase radiation exposure to the patient, and are not technologies that are easily transferrable for home use to help with early diagnosis. We present preliminary studies on patients suggesting that acoustical measurements recorded quantitatively with contact sensors (electronic stethoscopes) and analyzed using advanced computational analysis methods may provide an earlier diagnostic indicator of the onset of ACS than is possible with current clinical practice. Novel in silico models of respiratory acoustics utilizing image-based and algorithmically developed lungs with full conducting airway trees support and help explain measured acoustic trends and provide guidance on the next steps in developing and translating a diagnostic approach. More broadly, the experimental and computational techniques introduced herein, while focused on monitoring and predicting the onset of ACS, could catalyze further advances in mobile health (mhealth)-enabled, computer-based auscultative diagnoses for a wide range of cardiopulmonary pathologies.

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Arterial Hypoxemia Syndrome in Sickle Cell Disease.

Blood ◽

10.1182/blood.v106.11.3801.3801 ◽

2005 ◽

Vol 106 (11) ◽

pp. 3801-3801

Author(s):

Patricia Adams-Graves ◽

M. Muthiah ◽

G. Presbury ◽

G. Somes ◽

K. Lamar

Keyword(s):

Mechanical Ventilation ◽

Sickle Cell Disease ◽

Sickle Cell ◽

Normal Lung ◽

Acute Chest Syndrome ◽

Cell Disease ◽

X Ray ◽

Arterial Hypoxemia ◽

Arterial Blood ◽

Chest X Ray

Abstract Acute chest syndrome (ACS) is the most common cause of death during hospitalization of adults with sickle cell disease (SCD). ACS includes symptoms referable to the chest and a new infiltrate on chest X-ray. Adults over age 20 years have more symptoms of the disease and are at increased risk of early death compared to children. ACS may be the presenting diagnosis for a patient with SCD, but equally as often, develops while the patient has a painful vascular occlusive crisis. Notably, 35% of SCD patients have a normal lung exam upon presentation to the hospital. Previous research studies indicate that nearly three-fourths of SCD patients who die present during painful crises in an extremity, and about 50% conclusively by autopsy died of massive fat embolism syndrome (FES). Unfortunately, definitive diagnostic tests with rapid turn-around for FES and other acute vascular occlusive lung events do not exist. Earlier identification of the danger that this event may be evolving can be life saving. Clinicians who adhere to the strict definition of ACS may prematurely dismiss the likelihood of a subsequent fatal event. This alarming rate of adverse events may represent a “pre-chest syndrome” prodromal phase of ACS. Arterial hypoxemia syndrome (AHS) or pre-chest syndrome is defined as any sign or symptom referable to the chest, an oxygen saturation (Sp02) of <94% by direct pulse oximeter or a Pa02 <80% by arterial blood gas on room air plus a clear chest X-ray with or without fever. AHS may be a warning sign of an ultimately fatal event if earlier interventions are not done in a timely manner. A secondary data analysis was performed utilizing 500 health records of SCD patients from 1960 to 2004. Prior to 2003, we averaged 2 to 3 ICU admissions per month for ACS with about 20% requiring mechanical ventilation. This study sought to gain insight on 45 years of experience in the treatment of SCD, particularly “pre-chest syndrome.” The primary aims of the study were to devise treatment protocols to reduce ICU admissions and the need for mechanical ventilation in SCD patients presenting with AHS. Retrospective analysis suggests that earlier blood exchanges for patients with SCD may substantially reduce ICU admissions and the need for mechanical ventilation in patients presenting with AHS, compared with patients receiving standard supportive care. Examination of computerized hospital records of 437 sickle cell hospital admissions from January 2003 to March 2005 revealed 3 ICU and 2 step-down unit admissions. During this time period, there were 101 chest syndrome occurrences, of which 2 died. Both patients required mechanical ventilation and underwent red cell apheresis to reduce hemoglobin S to <30%. One patient was admitted due to major trauma from a motor vehicle accident. Death was due to multi-organ failure. The medical condition of the second patient improved. This patient was discharged home in stable condition but died, unexpectedly, 48 hours at home of a massive pulmonary embolus. A protocol has been developed to prospectively evaluate our aims.

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Infusion of Pegylated Bovine Carboxyhemoglobin (PEG-COHb) Is Associated with Rapid Reversal of Progressive Acute Chest Syndrome in a Jehovah's Witness Patient with Hemoglobin SC Sickle Cell Disease

Blood ◽

10.1182/blood.v126.23.4541.4541 ◽

2015 ◽

Vol 126 (23) ◽

pp. 4541-4541 ◽

Cited By ~ 1

Author(s):

Roberta Miyeko Kato ◽

Thomas Hofstra ◽

Herbert J. Meiselman ◽

Henry Jay Forman ◽

Abe Abuchowski ◽

...

Keyword(s):

Carbon Monoxide ◽

Sickle Cell Disease ◽

Sickle Cell ◽

Lower Lobe ◽

Acute Chest Syndrome ◽

Cell Disease ◽

X Ray ◽

Rapid Reversal ◽

Chest X Ray ◽

The Right

Abstract Acute chest syndrome (ACS) is a potentially fatal complication of sickle cell disease (SCD) and is characterized by opacification of the chest x-ray (CXR) and progressive pulmonary failure due, in part, to intra-pulmonary sickling. The ACS process can proceed very rapidly from a small area of lung involvement in one lobe to total opacification of the lung and pulmonary failure within 12 to 24 hours. In the early phases of this process, oxygenation and pulmonary function may be preserved. In the face of rapidly progressing CXR changes, the ACS process may be reversed if diagnosed early and managed by emergent transfusion to decrease the percent of sickle red blood cells (SRBC). A 10 years old African American child with hemoglobin SC type SCD was transferred to our institution with fever and right upper lobe consolidation. Her respiratory rate was 23 breaths/min, SpO2 was 95% breathing room air. Serial CXR showed opacification of the entire right lung and part of the left lower lobe over a 12-hour period (Panel A). Because of the rapid progression, transfusion was recommended. However, because of the family's Jehovah's Witness religious faith, transfusion was refused. PEG-COHb is in clinical development for the treatment of SCD and is designed to deliver preloaded carbon monoxide (CO), pick up O2, and deliver O2 to hypoxic tissue. PEG-COHb serves as a vasodilator and anti-inflammatory agent. It has been shown to have anti-sickling properties in vitro (ASH Abstract 1372, 2014). The agent was obtained from Prolong Pharmaceuticals via an emergency IND (16432) from the FDA. The agent was acceptable to the family and church elders. After written consent was obtained, 500 cc were infused according to dosing information obtained from Prolong Pharma. The CXR (Panel A) 3 hours before infusion shows opacification of the right lung and the left lower lobe. A CXR obtained one hour after infusion showed no worsening, and the CXR (Panel B) obtained 29 hours after Panel A shows significant improvement in the opacification of the lower lobes. The right upper lobe consolidation was likely bacterial pneumonia, and would not be expected to clear rapidly. The patient was mildly hypertensive for age (138/72 mmHg) prior to PEG-COHb infusion. Her blood pressure rose to 153/85 mmHg during infusion; the infusion was stopped and anti-hypertensives were administered. The infusion was restarted at a lower infusion rate and completed in 6 hours instead of the planned 4 with no untoward effects. She was discharged 4 days after the infusion. There were no other serious adverse events clearly related to the drug. There were significant laboratory abnormalities and transaminases that were most likely falsely elevated due to interference of the PEG-COHb with the laboratory methods. Continuous non-invasive monitoring of carboxyhemoglobin showed basal levels of 7% rose to 24% during infusions and returned to normal prior to discharge. Continual recording of SpO2, methemoglobin, heart-rate variability and blood rheological measures showed no significant abnormalities. The rapid reversal of radiographic features consistent with progressive "pure ACS" secondary to the right upper lobe infectious process suggests that PEG-COHb may be an effective treatment for sickle cell related ACS. SHAPE Figure 1. Panel A demonstrates the chest x-ray 3 hours prior to PEG-COHb with right upper lobe consolidation and evolving bilateral lower lobe consolidation and Panel B 29 hours following administration of PEG-COHb demonstrating improvement in the lower lobes. Figure 1. Panel A demonstrates the chest x-ray 3 hours prior to PEG-COHb with right upper lobe consolidation and evolving bilateral lower lobe consolidation and Panel B 29 hours following administration of PEG-COHb demonstrating improvement in the lower lobes. Disclosures Off Label Use: SANGUINATE (pegylated carboxyhemoglobin bovine) is 40 mg/mL of purified bovine hemoglobin that has been pegylated, saturated with carbon monoxide, and dissolved in a buffered saline solution.. Abuchowski:Prolong Pharmaceuticals: Employment. Parmar:Prolong Pharmaceuticals: Employment.

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Sickle cell patient with an acute chest syndrome and a negative chest X-ray: Potential role of the ventilation and perfusion (V/Q) lung scan

American Journal of Hematology ◽

10.1002/ajh.10411 ◽

2003 ◽

Vol 74 (3) ◽

pp. 214-215 ◽

Cited By ~ 6

Author(s):

Lawrence Feldman ◽

Robert Gross ◽

Jack Garon ◽

Anitha Nallari ◽

Navleen Kaur ◽

...

Keyword(s):

Sickle Cell ◽

Potential Role ◽

Acute Chest Syndrome ◽

X Ray ◽

Lung Scan ◽

Chest X Ray ◽

Ventilation And Perfusion

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Point‐of‐care lung ultrasound is more reliable than chest X‐ray for ruling out acute chest syndrome in sickle cell pediatric patients: A prospective study

Pediatric Blood & Cancer ◽

10.1002/pbc.29283 ◽

2021 ◽

Author(s):

Marcela Preto‐Zamperlini ◽

Eliana P.C. Giorno ◽

Danielle S.N. Bou Ghosn ◽

Fernanda V.M. Sá ◽

Adriana S. Suzuki ◽

...

Keyword(s):

Prospective Study ◽

Sickle Cell ◽

Pediatric Patients ◽

Lung Ultrasound ◽

Point Of Care ◽

Acute Chest Syndrome ◽

X Ray ◽

Chest X Ray ◽

A Prospective Study

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Distributed X-ray photon correlation spectroscopy data reduction using Hadoop MapReduce

Journal of Synchrotron Radiation ◽

10.1107/s160057751800601x ◽

2018 ◽

Vol 25 (4) ◽

pp. 1135-1143 ◽

Cited By ~ 6

Author(s):

Faisal Khan ◽

Suresh Narayanan ◽

Roger Sersted ◽

Nicholas Schwarz ◽

Alec Sandy

Keyword(s):

Real Time ◽

Photon Correlation Spectroscopy ◽

Correlation Spectroscopy ◽

Frame Rate ◽

Computational Techniques ◽

Complex Materials ◽

Photon Correlation ◽

X Ray ◽

Wide Range ◽

Recent Developments

Multi-speckle X-ray photon correlation spectroscopy (XPCS) is a powerful technique for characterizing the dynamic nature of complex materials over a range of time scales. XPCS has been successfully applied to study a wide range of systems. Recent developments in higher-frame-rate detectors, while aiding in the study of faster dynamical processes, creates large amounts of data that require parallel computational techniques to process in near real-time. Here, an implementation of the multi-tau and two-time autocorrelation algorithms using the Hadoop MapReduce framework for distributed computing is presented. The system scales well with regard to the increase in the data size, and has been serving the users of beamline 8-ID-I at the Advanced Photon Source for near real-time autocorrelations for the past five years.

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Microfluidics in Sickle Cell Disease Research: State of the Art and a Perspective Beyond the Flow Problem

Frontiers in Molecular Biosciences ◽

10.3389/fmolb.2020.558982 ◽

2021 ◽

Vol 7 ◽

Author(s):

Anupam Aich ◽

Yann Lamarre ◽

Daniel Pereira Sacomani ◽

Simone Kashima ◽

Dimas Tadeu Covas ◽

...

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Flow Problem ◽

Red Cells ◽

Acute Chest Syndrome ◽

Cell Disease ◽

Intercellular Interaction ◽

Blood Disorder ◽

Resource Setting ◽

Wide Range

Sickle cell disease (SCD) is the monogenic hemoglobinopathy where mutated sickle hemoglobin molecules polymerize to form long fibers under deoxygenated state and deform red blood cells (RBCs) into predominantly sickle form. Sickled RBCs stick to the vascular bed and obstruct blood flow in extreme conditions, leading to acute painful vaso-occlusion crises (VOCs) – the leading cause of mortality in SCD. Being a blood disorder of deformed RBCs, SCD manifests a wide-range of organ-specific clinical complications of life (in addition to chronic pain) such as stroke, acute chest syndrome (ACS) and pulmonary hypertension in the lung, nephropathy, auto-splenectomy, and splenomegaly, hand-foot syndrome, leg ulcer, stress erythropoiesis, osteonecrosis and osteoporosis. The physiological inception for VOC was initially thought to be only a fluid flow problem in microvascular space originated from increased viscosity due to aggregates of sickled RBCs; however, over the last three decades, multiple molecular and cellular mechanisms have been identified that aid the VOC in vivo. Activation of adhesion molecules in vascular endothelium and on RBC membranes, activated neutrophils and platelets, increased viscosity of the blood, and fluid physics driving sickled and deformed RBCs to the vascular wall (known as margination of flow) – all of these come together to orchestrate VOC. Microfluidic technology in sickle research was primarily adopted to benefit from mimicking the microvascular network to observe RBC flow under low oxygen conditions as models of VOC. However, over the last decade, microfluidics has evolved as a valuable tool to extract biophysical characteristics of sickle red cells, measure deformability of sickle red cells under simulated oxygen gradient and shear, drug testing, in vitro models of intercellular interaction on endothelialized or adhesion molecule-functionalized channels to understand adhesion in sickle microenvironment, characterizing biomechanics and microrheology, biomarker identification, and last but not least, for developing point-of-care diagnostic technologies for low resource setting. Several of these platforms have already demonstrated true potential to be translated from bench to bedside. Emerging microfluidics-based technologies for studying heterotypic cell–cell interactions, organ-on-chip application and drug dosage screening can be employed to sickle research field due to their wide-ranging advantages.

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Evidence-Based Mini-Review: Are Systemic Corticosteroids an Effective Treatment for Acute Pain in Sickle Cell Disease?

Hematology ◽

10.1182/asheducation-2010.1.416 ◽

2010 ◽

Vol 2010 (1) ◽

pp. 416-417 ◽

Cited By ~ 5

Author(s):

L. Vandy Black ◽

Wally R. Smith

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Adjunctive Treatment ◽

Cell Disease ◽

X Ray ◽

Systemic Corticosteroids ◽

Potential Efficacy ◽

History Of ◽

Chest X Ray ◽

Pain Crisis

Abstract An 18-year-old African-American male with sickle cell disease (SCD) is admitted to the hospital with a vaso-occlusive pain crisis affecting his chest and right upper extremity. He has a history of asthma but does not have a fever or respiratory symptoms, and a chest X-ray is negative for an infiltrate. He is treated with intravenous fluids and morphine. You are asked about the potential efficacy of systemic corticosteroids as an adjunctive treatment for pain control.

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How I treat acute chest syndrome in children with sickle cell disease

Blood ◽

10.1182/blood-2010-11-261834 ◽

2011 ◽

Vol 117 (20) ◽

pp. 5297-5305 ◽

Cited By ~ 50

Author(s):

Scott T. Miller

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Supplemental Oxygen ◽

Fat Embolism ◽

Pulmonary Infiltrate ◽

Clinical Findings ◽

Acute Chest Syndrome ◽

Cell Disease ◽

Mild Anemia ◽

Optimal Dosing

AbstractAcute chest syndrome describes new respiratory symptoms and findings, often severe and progressive, in a child with sickle cell disease and a new pulmonary infiltrate. It may be community-acquired or arise in children hospitalized for pain or other complications. Recognized etiologies include infection, most commonly with atypical bacteria, and pulmonary fat embolism (PFE); the cause is often obscure and may be multifactorial. Initiation of therapy should be based on clinical findings. Management includes macrolide antibiotics, supplemental oxygen, modest hydration and often simple transfusion. Partial exchange transfusion should be reserved for children with only mild anemia (Hb > 9 g/dL) but deteriorating respiratory status. Therapy with corticosteroids may be of value; safety, efficacy and optimal dosing strategy need prospective appraisal in a clinical trial. On recovery, treatment with hydroxyurea should be discussed to reduce the likelihood of recurrent episodes.

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One Shot Cluster Based Approach for the Detection of COVID-19 from Chest X-Ray Images

10.20944/preprints202007.0656.v1 ◽

2020 ◽

Author(s):

V.N. Manjunath Aradhya ◽

Mufti Mahmud ◽

Basant Agarwal ◽

D.S. Guru ◽

M. Shamim Kaiser

Keyword(s):

Virus Disease ◽

X Rays ◽

Probabilistic Neural Networks ◽

X Ray ◽

The Core ◽

Wide Range ◽

Test Instance ◽

Chest X Ray ◽

Detection And Diagnosis ◽

The Way

Corona virus disease (COVID-19) has infected over more than 10 million people around the globe and killed at least 500K worldwide by the end of June 2020. As this disease continues to evolve and scientists and researchers around the world now trying to find out the way to combat this disease in most effective way. Chest X-rays are widely available modality for immediate care in diagnosing COVID-19. Precise detection and diagnosis of COVID-19 from these chest X-rays would be practical for the current situation. This paper proposes one shot cluster based approach for the accurate detection of COVID-19 chest x-rays. The main objective of one shot learning (OSL) is to mimic the way humans learn in order to make classification or prediction on a wide range of similar but novel problems. The core constraint of this type of task is that the algorithm should decide on the class of a test instance after seeing just one test example. For this purpose we have experimented with widely known Generalized Regression and Probabilistic Neural Networks. Experiments conducted with publicly available chest x-ray images demonstrate that the method can detect COVID-19 accurately with high precision. The obtained results have outperformed many of the convolutional neural network based existing methods proposed in the literature.

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Employing Texture Features of Chest X-Ray Images and Machine Learning in COVID-19 Detection and Classification

MENDEL ◽

10.13164/mendel.2021.1.009 ◽

2021 ◽

Vol 27 (1) ◽

pp. 9-17

Author(s):

Hiam Alquran ◽

Mohammad Alsleti ◽

Roaa Alsharif ◽

Isam Abu Qasmieh ◽

Ali Mohammad Alqudah ◽

...

Keyword(s):

Machine Learning ◽

Texture Features ◽

Automated System ◽

Rt Pcr ◽

False Negatives ◽

X Ray ◽

Organ Systems ◽

Wide Range ◽

Chest X Ray

The novel coronavirus (nCoV-19) was first detected in December 2019. It had spread worldwide and was declared coronavirus disease (COVID-19) pandemic by March 2020. Patients presented with a wide range of symptoms affecting multiple organ systems predominantly the lungs. Severe cases required intensive care unit (ICU) admissions while there were asymptomatic cases as well. Although early detection of the COVID-19 virus by Real-time reverse transcription-polymerase chain reaction (RT-PCR) is effective, it is not efficient; as there can be false negatives, it is time consuming and expensive. To increase the accuracy of in-vivo detection, radiological image-based methods like a simple chest X-ray (CXR) can be utilized. This reduces the false negatives as compared to solely using the RT-PCR technique. This paper employs various image processing techniques besides extracted texture features from the radiological images and feeds them to different artificial intelligence (AI) scenarios to distinguish between normal, pneumonia, and COVID-19 cases. The best scenario is then adopted to build an automated system that can segment the chest region from the acquired image, enhance the segmented region then extract the texture features, and finally, classify it into one of the three classes. The best overall accuracy achieved is 93.1% by exploiting Ensemble classifier. Utilizing radiological data to conform to a machine learning format reduces the detection time and increase the chances of survival.

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