# Diagnostic Accuracy of Ultrasound for Assessment of Arthropathic hemophilia

- Link: https://sifsof.com/clinical-apps/diagnostic-accuracy-of-ultrasound-for-assessment-of-arthropathic-hemophilia/
- Published: 2021-10-28T10:45:22+00:00
- Author: Sales

The hemophilia is a combination of the greek word “blood” and “love” a way of saying
that people with hemophilia “love to bleed” or rather or rather that it’s hard to
stop bleeding. This is because the process called hemostasis, literally meaning 
to stop the flow of blood is impaired. Normally after a cut or damage to the endothelium
or lining of blood vessels walls there’s an immediate vasoconstriction or narrowing
of the blood vessels which limits the amount of blood flow. After that some platelets
adhere to the damaged vessel wall, and become activated and then recruit additional
platelets to form a plug. The formation of this platelet plug is called primary 
hemostasis. After that the coagulation cascade is activated. First Off the blood
has a set of clotting factors. Most of which are proteins synthesized by the liver
and usually these are inactive and just floating around in the blood. The coagulation
cascade starts when one of these proteins gets proteolytically cleaved. This active
protein then proteolytically cleaves and activates the next clotting factor and 
so on. This cascade has a great degree of amplification and takes only a few minutes
from injury to clot formation. The final step is activation of the protein fibrinogen(
factor1) to fibrin. Which deposits and polymerizes to form a mesh around the platelets.
So these steps leading up to fibrin reinforcement of the platelet plug make up the
process called secondary hemostasis and result in hard clot at the site of the injury.

In most cases of hemophilia there is a decrease in the amount or function of one
or more of the clotting factors which makes secondary hemostasis less effective 
and allows more blessing to happen. Now that coagulation cascade can start in either
two ways. First way is called the extrinsic pathway, which starts when tissue factor
gets exposed by the injury of the endothelium. Tissue factor turns inactive factor
7 into active factor 7A (A for active), and then tissue facto goes on to bind with
the newly formed factor 7A to form a complex that turns factor 10 into active factor
10A. Factor 10A with factor 5A as a cofactor turns factor 2 which is also (which
is also called prothrombin) into factor 2A which is also called thrombin. Thrombin
then turns factor 1 or fibrinogen which is soluble into 1A or fibrin which is insoluble
and precipitates out of the blood at the site of injury. Thrombin also turns factor
13 into factor 13A which crosslinks the fibrin to form a stable clot. The second
way is called the intrinsic pathway and starts with platelets near the blood vessel
injury activates factor 12 into factor 12A which then activates factor 11 into factor
11A which then activates factor 9 into factor 9A. Factor 9A along with factor 8A
work together to activate factor 10 to factor 10A and from that point it follows
the same fate as before. So the extrinsic and intrinsic pathways basically converge
on a single final path called the common pathway. This is a somewhat simplified 
version of the coagulation cascade but it has all the key parts needed to understand
hemophilia. An insufficient or decreased activity of any coagulation factor can 
cause hemophilia except factor 12 deficiency which is asymptomatic.

Hemophilia usually refers to inherited deficiencies, either quantitative or qualitative.
By far the most common of these are factor 8 which gives rise to factor 8A and is
stabilized by another factor called von wilebrand  factor. This deficiency is called
hemophilia A or classic hemophilia. Another common deficiency is deficiency in factor
9 called hemophilia B which used to be called Christmas disease named after the 
first person who had it not the holiday. 

Hemophilia patients require lifelong clotting factor replacement therapy to mitigate
spontaneous joint bleeding and other life threatening bleeding. However, clotting
factor replacement therapy is costly and imposes a high financial burden on individuals,
healthcare systems and society in general. Joint bleeding represents the most commonly
reported type of hemorrhage in patients affected by hemophilia. Although the widespread
use of prophylaxis has been able to significantly reduce the onset of arthropathy,
it has been shown that a non-negligible percentage of patients develop degenerative
changes in their joints despite this type of treatment. Thus, periodic monitoring
of the joint status in hemophilia patients has been recommended to identify early
arthropathic changes and prevent the development or progression of hemophilic arthropathy.
Ultrasound (US) has proven able to detect and quantify the most relevant biomarkers
of disease activity (i.e., joint effusion and synovial hypertrophy) and degenerative
damages (i.e., osteo-chondral changes) by means of scoring scales of increasing 
disease severity. Therefore, timely objective detection of acute or persistent joint
bleeding in hemophilia patients has become increasingly important.

Magnetic resonance imaging (MRI) is considered the “gold standard” to detect various
[abnormalities in hemophilic arthropathy](https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5826858/#R6).
However, in the past few years, musculoskeletal ultrasound (MSKUS) has emerged as
a point-of-care (POC) imaging tool to assess the [extent of arthropathic changes](https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5826858/#R9),
thus opening new avenues for the management of hemophilic arthropathy and also rapid
joint bleed detection. Recent advances in technology, accessibility, and training
have made POC MSKUS an attractive alternative to MRI in instances where imaging 
is desired. MSKUS is faster, more economical, and without the need of sedation for
claustrophobic subjects or children. In addition, MSKUS does not require intravenous
contrast to distinguish synovial proliferation from fluid and can also be used to
[assess synovial vascularity](https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5826858/#R14).

MSKUS appears very adept in detecting joint effusions based on the ability of dynamic
maneuvers during scanning. For hemophilia, this feature appears particularly valuable
for the detection and management of hemarthrosis, where precise diagnosis of presence
or absence of (bloody) effusions can complement patient or physician perception,
thereby optimizing targeted treatment options. It allows visualization of shifting
fluid in communicating spaces as well as sonopalpation.

 Sonopalpation assesses compressibility and displacement of echogenic intra-articular
material. Effusions can be separated into simple versus complex. Complex fluid accumulations
are characterized by mixed echogenicity and displaceable speckles, indicating the
presence of particulate matter such as proteins or blood products, while simple 
effusions appear anechoic with clear and serous fluid upon aspiration. Thus, MSKUS
not only documents the presence of an effusion, but also distinguishes between bloody
versus non-bloody effusions based on echogenicity (echogenic versus anechoic) and
presence of displaceable echogenic reflectors. 

In the context of hemophilia, complex effusions with echogenic reflectors can be
assumed to represent blood products based on previous documentation of the great
accuracy of this approach as documented by[ joint aspiration](https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5826858/#R4).
MSKUS algorithms to detect hemarthrosis are therefore well defined, and can be performed
quickly as part of the daily clinic routine, thereby fulfilling POC criteria. Moreover,
MSKUS enables guided aspiration and fluid analysis as clinically indicated.

In this context, it is noteworthy that radiological MRI criteria to assess blood
contents in the joint are less well defined, and mainly derived from[ previous neurological studies](https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5826858/#R21).
A preliminary study 30 years ago suggested that MRI may not have the same usefulness
for distinguishing between bloody and non-bloody effusions in joints. However, formal
studies employing modern imaging technology are lacking, and clinical imaging interpretation
algorithms more commonly use inference rather than evidence. Moreover, in daily 
clinical practice, joint effusions on MRI automatically may be deemed as bloody 
if arising in the context of hemophilia.

MSKUS has proven to be extremely sensitive in detecting low concentrations of intra-
articular blood and in discriminating between bloody and non-bloody fluid, whereas
conventional MRI is not. These observations demonstrate the advantages of MSKUS 
over MRI in detecting intra-articular blood, and show that MSKUS is ideal for rapid
bleed detection in the clinic.

For this type of diagnoses we highly recommend the Color 5-10 MHz Wireless Linear
Ultrasound Scanner 128 Elements [SIFULTRAS-5.38](https://sifsof.com/product/color-5-10-mhz-wireless-linear-ultrasound-scanner-128-elements-sifultras-5-38/).
This ultrasound Resolution visualizes delicate tissue structure in shallower regions.
It’s image clarity reduces the noise in blood vessels at a frequency range of 5-
10 MHz frequency and 40-120mm depth. , the SIFULTRAS-5.38 does not only serve hemarthrosis
but orthopedy in general. The Color Linear ultrasound scanner provides qualitative
and quantitative for musculoskeletal diagnosis. For example: Tendon tears, or tendinitis
of the rotator cuff in the shoulder, Achilles tendon in the ankle and other tendons
throughout the body, muscle tears, masses or fluid collections., ligament sprains
or tears.

Using the SIFULTRAS-5.38 the physician can detect;  inflammation or fluid (effusions)
within the bursae and joints,  early changes of rheumatoid arthritis, nerve entrapments
such as carpal tunnel syndrome,  benign and malignant soft tissue tumors, ganglion
cysts, hernias., foreign bodies in the soft tissues (such as splinters or glass),
dislocations of the hip in infants,  fluid in a painful hip joint in children, neck
muscle abnormalities in infants with torticollis (neck twisting), soft tissue masses(
lumps/bumps) in children.

This procedure is performed by a qualified orthopedist trained in ultrasound imaging.*

 * ![Color Doppler Mini Linear WiFi Ultrasound Scanner SIFULTRAS-3.51 main](https://
   sifsof.com/wp-content/uploads/2018/11/SIFULTRAS-3.51-wireless-mini-linear-color-
   doppler-ultrasound-scanner-300x300.jpg)
 * Color Doppler Mini Linear WiFi Ultrasound Scanner SIFULTRAS-3.51