Solved by verified expert:Hello, In this paper I need from 3 to 4 paragraphs that review (NOT analyze) the study. The review of the main study shouldn’t take more that 1/4 of the whole paper. The rest of the paper has to be your own opinion about the topic and the findings with supporting your opinion with reliable resources. example: why do you think this topic is important? what do you think about the study and the findings? What other new research find?I am attaching the main journal, and the Journal Article Grading Rubric (you should follow it):Use APA format and follow the grading rubric for more details about the assignment.
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Journal Article Grading Rubric
2
1
0
Length 3-4 paragraphs.
Somewhat too long or
Much too long or short.
Reference, and article
short. Reference or
No reference or article
Basic
or URL given.
article/URL given. Small given. Terminology,
Technical terminology
Mechanics
number of errors in
formalism frequently
and formalism are
terminology or formalism. misused.
used correctly.
Chosen point
identified and clearly
No point identified, or
explained. Factually Chosen point identified, but
explanation very unclear.
correct as report of
explanation not fully clear.
Major errors in report of
chosen aspect of
Minor errors in report of
authors’ meaning. Relies
authors’ meaning. Chosen
Understanding article. Explains
on quotations or
chosen point; goes
point paraphrased in
superficial paraphrase;
of article
beyond merely
student’s own words, but
little evidence of
paraphrasing or
very close to original.
understanding. Major
quoting. Class
Minor misunderstandings
problems from not
knowledge used
related to class knowledge.
applying class knowledge.
correctly where
appropriate.
Goes beyond
Shows understanding of
summary; includes
relevant issues, but
Critical discussion
critique, connects to
contributes no substantial missing, or shows serious
other data or ideas.
Active and
original points. Focus is
misunderstanding of
Tight focus on main
critical
somewhat loose. Some
article. No clear focus.
point. Report is well
organization,
but
Structure of discussion has
thinking;
organized; paragraphs
argumentation and overall discussion relationships between ideas no clear organization.
not always clear. Crucial
Examples used, but not
is focused, coherent.
examples, data not always connected to discussion.
Examples, data used
given.
appropriately.
Clear articulate writing
Edits needed. Proof reading
used. One or two
Turns in something. Not
will help you. Read aloud
minor edits needed to
college level work at all.
to yourself and or ask
Writing
be a perfect paper!
Get help at the writing
others to read it out loud to
Keep up the great
center.
you.
work
Articulates your
thoughts on the article
Brief mention of thoughts, Does not write any of your
in a clear manner.
but did not elaborate. No own thoughts or ideas
Your Thoughts Discusses what you mention of learning from about what is discussed in
learned from reading
reading the article.
the article.
the article or ideas you
might use in the future.
Your
Score
The Journal of Emergency Medicine, Vol. 51, No. 4, pp. 370–381, 2016
! 2016 Elsevier Inc. All rights reserved.
0736-4679/$ – see front matter
http://dx.doi.org/10.1016/j.jemermed.2016.05.042
Clinical
Review
EMERGENCY MEDICINE MANAGEMENT OF SICKLE CELL DISEASE
COMPLICATIONS: AN EVIDENCE-BASED UPDATE
Erica Simon, DO, MHA,* Brit Long, MD,* and Alex Koyfman, MD†
*Department of Emergency Medicine, San Antonio Military Medical Center, Fort Sam Houston, Texas and †Department of Emergency
Medicine, The University of Texas Southwestern Medical Center, Dallas, Texas
Reprint Address: Brit Long, MD, 506 Dakota Street, Apartment 1, San Antonio, TX 78203
prevalent in persons of African, Mediterranean, Indian,
and Middle Eastern descent (1–3). The sickle cell
mutation is inherited in an autosomal recessive fashion;
homozygotes exhibit sickle cell disease (SCD or HbSS)
and heterozygotes exhibit sickle cell trait (SCT).
Assuming that they have not inherited a second
abnormal hemoglobin (Hb) chain, individuals with SCT
are commonly asymptomatic and possess a normal
lifespan, while those with SCD are predisposed to
severe infections, complications associated with
repetitive capillary obstruction, painful vaso-occlusive
crises, and multi-system organ damage (1,2).
Complications of SCD occur secondary to the sickle
cell mutation: a sixth codon substitution of the B-globin
chain, replacing hydrophobic valine with hydrophilic
glutamic acid, thereby causing sickling of the Hb
molecule under de-oxygenated conditions. The congregation of these sickled cells results in microvascular
sludging and vascular obstruction, leading to the acute
manifestations (1,2).
As SCD is a component of American newborn
screening, the discovery of undiagnosed SCD in the
emergency department (ED) is relatively uncommon.
More frequently, patients with known SCD present to
the ED for evaluation secondary to sequelae of the disease
after the fourth month of life (decline in fetal hemoglobin
concentration) (3).
Emergency physicians are adept at managing
multiple disease processes; however, given the range of
, Abstract—Background: Sickle cell disease (SCD) affects
approximately 100,000 individuals in the United States. Due
to alterations in the structural conformation of hemoglobin
molecules under deoxygenated conditions, patients with SCD
are predisposed to numerous sequelae, many of which require
acute intervention. Objective: Our aim was to provide emergency physicians with an evidence-based update regarding
the diagnosis and management of SCD complications.
Discussion: SCD patients experience significant morbidity
and mortality secondary to cerebrovascular accident, acute
chest syndrome, acute vaso-occlusive pain crises, SCD-related
multi-organ failure, cholecystitis, acute intrahepatic cholestasis, acute sickle hepatic crisis, acute hepatic sequestration,
priapism, and renal disease. Emergency physicians must recognize acute manifestations of SCD in order to deliver timely
management and determine patient disposition. Conclusions:
A comprehensive review of the emergency department
management of acute SCD complications is provided. Comprehensive understanding of these aspects of SCD can assist physicians in expediting patient evaluation and treatment, thus
decreasing the morbidity and mortality associated with this
hemoglobinopathy. ! 2016 Elsevier Inc. All rights reserved.
, Keywords—sickle cell disease; acute chest; acute pain
crisis; cerebrovascular accident; transfusion
INTRODUCTION
Sickle cell disease (SCD) affects nearly 100,000
individuals in the United States, and approximately
2 million Americans carry the sickle cell trait. SCD is
RECEIVED: 21 January 2016; FINAL SUBMISSION RECEIVED: 12 May 2016;
ACCEPTED: 17 May 2016
370
Emergency Medicine Management of Sickle Cell Disease
pathophysiologic manifestations of SCD, encounters
with these patients often prove challenging. This review
seeks to provide emergency physicians with an improved
understanding of SCD complications and an evidencebased approach to their management.
MANAGING ACUTE COMPLICATIONS OF SCD:
VASO-OCCLUSIVE CRISES AND SEQUELAE OF
HEMOGLOBINOPATHY
Vaso-Occlusive Crises
Cerebrovascular accident: Ischemic stroke and intracranial hemorrhage. Cerebrovascular accident (CVA),
including ischemic stroke and subsequent intracranial
hemorrhage due to hemorrhagic conversion of the
ischemic stroke, is a major complication of SCD. Patients
presenting to the ED for assessment will display
symptoms that vary according to the anatomic location
of the infarct or hemorrhage. Small infarcts in the adult
and pediatric populations are relatively common and
involve the basal ganglia and deep white matter within
the anterior circulation (4). Risk factors for CVA in
patients with SCD include low Hb, history of acute chest
syndrome (ACS), and history of hypertension (4). The
pathophysiology regarding anemia and a history of
ACS as CVA risk factors is poorly understood. SCD
experts hypothesize severe anemia as precipitating
increased cerebral blood flow and increased cerebral
flow velocity, thereby predisposing SCD patients (the
majority experiencing chronic anemia) to cerebrovascular damage. Scientists also postulate the temporal
association between ACS and CVAs as resulting from
repetitive episodes of hypoxia in the setting of ACS.
This hypoxia likely causes additional damage to cerebral
vessels, previously injured by microvascular insults (5).
In the assessment of adult and pediatric patients
presenting with symptoms concerning for acute intracranial pathology, neuroimaging is key. Initial evaluation of
the adult patient commonly includes non-contrast head
computed tomography (CT), subsequently followed by
CT angiography or magnetic resonance angiography
(MRA) during the inpatient course.
Goals for the acute treatment of ischemic stroke in the
adult SCD patient include limiting injury due to the CVA
and establishing secondary prevention through the
optimization of cerebral perfusion (maintenance of
euglycemia and normothermia and avoidance of hypoxia)
(6). Caution is advised when considering the administration of thrombolytics to adult SCD patients experiencing
an acute ischemic CVA. Increased rates of intracranial
hemorrhage have been reported in this patient population
(6). Similar to adult patients without a medical history of
SCD, antiplatelet and statin therapy should be considered
after an ischemic CVA (6). In addition to the strategies
371
mentioned for secondary CVA prevention, experts
also recommend regular transfusions to maintain Hb
S < 30%; however, data supporting this intervention
was collected in young adults with SCD having
experienced their first CVA during childhood (hence its
employment in the pediatric population, as discussed
later) (6).
In contrast to adults, magnetic resonance imaging
(MRI) with diffusion-weighted imaging and MRA of
the head and neck should be performed in pediatric
patients with suspected acute ischemic stroke, as a
non-contrast head CT will miss early signs of ischemic
infarct (5). All pediatric patients diagnosed with an
ischemic stroke thought secondary to SCD should receive
intravenous (IV) fluids and undergo exchange transfusion
to achieve an Hb S level of < 30% (5). This procedure
should be performed in consultation with a hematologist.
If an exchange transfusion cannot be arranged, a simple
transfusion should be performed (5). A maximum Hb of
13 g/dL status post transfusion is the recommended
target, as pediatric children with SCD may be at risk
for recurrent ischemia secondary to increased blood
viscosity (5). Currently, thrombolysis is not recommended in pediatric SCD patients presenting with ischemic
CVAs (5). One key point that the emergency physician
must consider when evaluating the pediatric SCD patient
is that hemorrhagic transformation occurs in 30% of
children with arterial ischemic and is frequently
asymptomatic (7).
To date, there are no published studies regarding the
management of hemorrhagic CVA in adult or pediatric
SCD patients (6). Previously recognized efficacious
treatments for acute intracranial hemorrhage in the
general adult and pediatric population include reversal
of anticoagulation, treatment in an intensive care unit
(ICU), treatment of seizures with antiepileptic agents,
and appropriate management of blood pressure (BP) (6).
While BP management in acute CVA is well addressed
in adult emergency medicine literature, the management
of pediatric hypertension in the setting of CVA is not as
well studied. Hypertension in children, defined as
BP > 95th percentile for age, within the first 72 h after
ischemic stroke is associated with an increased risk of
death (8). In the pediatric population, a BP goal of the
50–95th percentile for age and height, with permissive
hypertension up to 20% > 95th percentile, should be
targeted (6). Pediatric experts recommend use of
labetolol or an angiotensin-converting enzyme inhibitor
to lower BP by 25%, though renal function should be
considered (6,9).
Of note, seizures are common after pediatric neurologic injury (10). Patients with persistent lethargy or
altered mental status should be evaluated with electroencephalography for subclinical seizure activity (7).
372
E. Simon et al.
Table 1. Differentiating Acute Chest Syndrome and Acute Pain Crisis
Acute Chest Syndrome
Clinical presentation
Laboratory studies
Chest x-ray study
Treatment
Feared complication
Pulmonary Acute Pain Crisis
Chest pain, fever, shortness of breath, hypoxia
Leukocytosis
New infiltrate (pediatric: middle or upper lobe; adult: lower lobe)
Antibiotics: community-acquired pneumonia vs. health
care–associated pneumonia (history-dependent);
ICU admission
Acute respiratory distress syndrome
Chest pain, fever, shortness of breath
Leukocytosis
No acute cardiopulmonary findings
Pain controlled without hypoxia: home
Unable to attain pain relief: admission
Atelectasis and subsequent pneumonia
due to splinting and low tidal volumes
ICU = intensive care unit.
In the setting of SCD, an investigation of alternative
causes of CVA cannot be overlooked. Etiologies include
infection, cardiac embolism, and cavernous venous sinus
thrombosis (7).
Imaging, to include MRI and MRA of the head and
neck, may be essential in narrowing the differential
diagnosis (7).
ACS.ACS, the most common reason for ICU admission in
the SCD patient population, is a leading cause of
morbidity and mortality (case fatality rate of 10%) (11).
The classic triad of ACS includes fever, hypoxia, and a
new pulmonary infiltrate on chest x-ray study. The
presence of any one of these signs or symptoms should
raise clinical suspicion in the setting of SCD. When
evaluating a patient for ACS, a chest x-ray study should
be obtained to identify the presence of a new infiltrate,
a complete blood count (CBC) should be sent to assess
anemia, and continuous oxygenation monitoring should
be performed to detect hypoxia.
While the pathogenesis of ACS has yet to be determined, infection secondary to Mycoplasma pneumoniae
frequently represents the underlying etiology in the
pediatric population (12). In adult sickle cell patients,
Chlamydophila pneumonia is the most commonly
encountered pathogen (13). Additional non-infectious
etiologies of ACS include fat emboli (released as a result
of bony infarct from vaso-occlusion) and pulmonary
emboli (disseminated post microvascular pulmonary
infarction) (13).
Differentiating ACS and pulmonary acute pain crisis
(APC) (which will be discussed) is difficult, as these
SCD complications often present with fever, shortness
of breath, chest pain, and leukocytosis (1,2,14). Any
respiratory symptoms associated with chest pain and
hypoxia should raise suspicion for ACS. A new
infiltrate on chest x-ray study is diagnostic of ACS, as
opposed to APC. Unfortunately, the chest x-ray study
may be normal early in ACS (2,14). When evaluating
chest x-ray studies, note that children are more likely to
display upper or middle lobe disease, as opposed to
adults, who frequently display lower lung disease with
an infiltrate and associated pleural effusion (14). See
Table 1 for a review of ACS vs. pulmonary symptoms
of APC.
ACS can rapidly progress to acute respiratory distress
syndrome due to pulmonary sequestration or infarct.
Given this fact, patients with signs or symptoms
consistent with ACS should be managed in an intensive
care setting. Long-term complications of ACS include
pulmonary fibrosis, pulmonary hypertension, and cor
pulmonale. Acute right ventricular failure is a
complication of ACS and if suspected, ultrasound (US)
should be utilized to assess right ventricular contractility
and size (14).
Evidence-based guidelines and expert panels are
shown in Table 2 for the management and treatment of
ACS.
APC. Vaso-occlusive pain crises may manifest in a
number of locations, including the pulmonary system,
central nervous system, skeletal system (arthralgias/
dactylitis), and gastrointestinal system (abdominal
pain). In the setting of these crises, patients commonly
present with fever and leukocytosis (12). Although fever
and leukocytosis are not specific indicators of infection, it
is wise to evaluate for an infectious etiology in the sickle
cell patient population, as these individuals are highly
susceptible to pathogens (addressed within at a later
juncture) (11).
SCD patients are prone to several complications that
must be considered during the evaluation of patients
presenting with pain crisis. These complications range
from the sequelae of hemoglobinopathy to renal
pathology (both later addressed) to the infectious
etiologies mentioned. Inquiries regarding prior pain
crises, differences between current and previous
episodes, the presence of fever, transfusion history,
medications, baseline Hb level, and a thorough physical
examination can assist in determining diagnoses. Any
atypical pain pattern not consistent with previous
episodes requires further evaluation.
In addition to an assessment for conditions requiring
acute interventions, it is important to note that the
Emergency Medicine Management of Sickle Cell Disease
373
Table 2. Management and Treatment of Acute Pain Crisis (3,15)
Level of
Recommendation
Recommendation
ACS patients should be hospitalized for pain control and SpO2 monitoring
ACS patients should receive antibiotics (parenteral cephalosporin or oral macrolide therapy)
ACS patients should receive supplemental O2 to maintain SpO2 > 95%
Patients with ACS should receive a blood transfusion to improve O2 carrying capacity
if Hb is > 1 g/dL below baseline (if baseline is > 9 g/dL, may not be required;
consult hematology)
ACS with rapid progression (SpO2 < 90% despite O2 therapy, respiratory distress, progressive
pulmonary infiltrates, decline in Hb despite simple transfusion) requires urgent exchange
transfusion
Consult hematology
Quality of
Evidence
Consensus
Strong
Strong
Weak
Panel expertise
Low quality
Low quality
Low quality
Strong
Low quality
ACS = acute chest syndrome; APC = acute pain crisis; Hb = hemoglobin.
management of pain crises includes the provision of early
analgesia—an area in which emergency physicians
commonly under-prescribe (2,3). Evidence-based
guidelines regarding management and treatment of pain
crises are demonstrated in Table 3.
Opioid analgesics are the current mainstay of APC
therapy. Morphine, fentanyl, and hydromorphone are
commonly utilized in the ED treatment of acute pain
crisis (15–23). Caution is recommended in the
utilization of meperidine (normeperidine, the active
metabolite of meperidine, undergoes renal excretion
and is associated with an increased incidence of
seizures in the setting of renal dysfunction; a finding
common in occlusive crisis) (3,12,15).
Varying algorithms for the management of APC
are detailed by multiple guidelines and organizations
(16–22). Provided as an example, the National Heart,
Lung, and Blood Institute (NHLBI) algorithm is
depicted in the Figure 1. This example was chosen by
the authors given its value in demonstrating an
all-encompassing approach to patient analgesia: the
inclusion of patient perception of pain, a mention of
detailed recommendations regarding initial opioid
dosing, the provision of direction regarding adjuncts to
pain control, and an emphasis on repeated patient
assessment in determining disposition.
As all of the APC guidelines note, further studies are
required to evaluate the adequacy of varying opioid
analgesics regimens in controlling APC pain, to
determine the efficacy of delivery routes and dosing
intervals, and to develop consensus statements regarding
the provision of patient analgesia in APCs (16–22). If
possible, organizations should work toward the
development of APC patient-management protocols, as
case studies have demonstrated decreased time to the
delivery of patient analgesia, improvement in overall
patient pain control, decreased frequency of ED visits,
fewer total hospital days, and increased utilization
of primary provider services status post algorithm
employment (23–25).
As depicted in Figure 1, pain-control adjuvants
detailed by the NHLBI include sedatives, anxiolytics,
and antihistamines. While employed to augment the
analgesic effect of opioids by managing associated
symptoms, such as anxiety, and to prevent mast cell
degranulation induced by opioid administration,
controlled studies of these treatments in SCD are lacking,
and per the NHLBI, guidelines for their use are derived
from employment in other pain ...
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