Professional Documents
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HEMOPHILIA
Text A
Hemophilia results from mutations at the factor VIII or IX loci on the X
chromosome and each occurs in mild, moderate, and severe forms.
Text B
Hemophilia A
Primary sites of factor VIII (FVIII) production are thought to be the vascular
endothelium in the liver and the reticuloendothelial system.
Human synovial cells synthesize high levels of tissue factor pathway inhibitor,
resulting in a higher degree of factor Xa (FXa) inhibition, which predisposes
hemophilic joints to bleed.
This effect may also account for the dramatic response of activated factor VII
(FVIIa) infusions in patients with acute hemarthroses and FVIII inhibitors.
Bleeding into a joint may lead to synovial inflammation, which predisposes the
joint to further bleeds; a joint that has had repeated bleeds (by one definition, at
least 4 bleeds within a 6-month period) is termed a target joint.
Hemorrhage sites include joints (eg, knee, elbow), muscles, central nervous system
(CNS), GI system, genitourinary (GU) system, pulmonary system, and
cardiovascular system.
The intrinsic system is initiated when factor XII is activated by contact with
damaged endothelium.
FVIII and FIX circulate in an inactive form; when activated, these 2 factors
cooperate to cleave and activate factor X, a key enzyme that controls the
conversion of fibrinogen to fibrin.
Therefore, the lack of either of these factors may significantly impair clot
formation and, as a consequence, result in clinical bleeding.
Hemophilia is slowly progressing among pediatric patients in all parts of the globe.
Hemophilia A is the most common X-linked genetic disease and the second most
common factor deficiency after von Willebrand disease (vWD).
The worldwide incidence of hemophilia A is approximately 1 case per 5000 males,
with approximately one-third of affected individuals not having a family history of
the disorder.
In the United States, the prevalence of hemophilia A is 20.6 cases per 100,000
males; in 2016, the number of people in the United States with hemophilia was
estimated to be about 20,000.
The prevalence of hemophilia B is 5.3 cases per 100,000 male individuals, with
44% of those having severe disease.
Ryan White was an American hemophiliac who was infected with HIV from a
contaminated blood treatment (factor VIII).
Prehospital care. Rapid transport to definitive care is the mainstay of prehospital
care; prehospital care providers should apply aggressive hemostatic techniques,
assist patients capable of self-administered factor therapy, and gather focused
historical data if the patient is unable to communicate.
Emergency department care. Use aggressive hemostatic techniques; correct
coagulopathy immediately; include a diagnostic workup for hemorrhage, but never
delay indicated coagulation correction pending diagnostic testing; acute joint
bleeding and expanding, large hematomas require adequate factor replacement for
a prolonged period until the bleed begins to resolve, as evidenced by clinical
and/or objective methods; life-threatening bleeding episodes are generally initially
treated with FVIII levels of approximately 100%, until the clinicalsituation
warrants a gradual reduction in dosage.
Factor VIII and FIX concentrates. Various FVIII and FIX concentrates are
available to treat hemophilia A and B; besides improved hemostasis, continuous
infusion decreases the amount of factor used, which can result in significant
savings; obtain factor level assays daily before each infusion to establish a stable
pattern of replacement regarding the dose and frequency of administration.
Desmopressin.Desmopressin vasopressin analog, or 1-deamino-8-D-arginine
vasopressin (DDAVP), is considered the treatment of choice for mild and moderate
hemophilia A; DDAVP stimulates a transient increase in plasma FVIII levels;
DDAVP may result in sufficient hemostasis to stop a bleeding episode or to
prepare patients for dental and minor surgical procedures.
Management of bleeding Immobilization of the affected limb and the application
of ice packs are helpful in diminishing swelling and pain; early infusion upon the
recognition of initial symptoms of a joint bleed may often eliminate the need for a
second infusion by preventing the inflammatory reaction in the joint; prompt and
adequate replacement therapy is the key to preventing long-term complications.
PART A
TIME: 15 minutes
Look at the four texts , A – D, in the separate Text Booklet
For each question, 1-20, look through the texts, A-d, to find the relevant
information.
Write your answers on the spaces provided in this Question Paper.
Answer all the questions within the 15-minute time limit.
HEMOPHILIA
Questions 8-14
Answer each of the questions, 8-4, with a word or short phrase from one of the
texts. Each answer may include words, number of the both. Your answers should
be correctly spelled.
Questions 15-20
Complete each of the sentences, 15- 20, with a word or short phrase from one of
the texts. Each answer may include words, number or both. Your answers should
be correctly spelled
1. Elements of malpractice:
1. Elements of malpractice
C. can be used easily at home and disrupt the eating and speaking of the patient
2. Nasal Cannula
A variety of devices are available for delivering oxygen to the patient. Each has a
specific function and oxygen concentration. Device selection is based on the
patient’s condition and oxygen needs. A nasal cannula, also called nasal prongs, is
the most commonly used oxygen delivery device. The cannula is a disposable
plastic device with two protruding prongs for insertion into the nostrils. The
cannula connects to an oxygen source with a flow meter and, many times, a
humidifier. It is commonly used because the cannula does not impede eating or
speaking and is used easily in the home. Disadvantages of this system are that it
can be dislodged easily and can cause dryness of the nasal mucosa. A nasal
cannula is used to deliver from 1 L/minute to 6 L/minute of oxygen. Table 14-1
compares amounts of delivered oxygen for these flow rates.
3. An expressed consent to be taken during certain situations except;
3. Expressed Consent
An express consent is one the terms of which are stated in distinct and explicit
language. It may be oral or written. For the majority of relatively minor
examinations or therapeutic procedures, oral consent is employed but this should
preferably be obtained in the presence of a disinterested party. Oral consent, where
properly witnessed, is as valid as written consent, but the latter has the advantage
of easy proof and permanent form. It should be obtained when the treatment is
likely to be more than mildly painful, when it carries appreciable risk, or when it
will result in diminishing of a bodily function. Consent may be confirmed and
validated adequately by means of a suitable contemporaneous notation by the
treating physician in the patient’s record. Expressed consent in written form should
be obtained for surgical operations and invasive investigative procedures. It is
prudent to obtain written consent, also where never analgesic, narcotic or
anesthetic agents will significantly affect the patient’s level of consciousness
during the treatment.
4. Irrational use of medicines is a major global problem because?
A. the waist restraints and the jacket restraints are the same
C. the potential risk of using waist restraints are more than its benefits
Waist restraints are a form of restraint that is applied to the patient’s torso. It is
applied over the patient’s clothes, gown, or pajamas. When using a waist restraint,
patients can move their extremities but cannot get out of the chair or bed.
Restraints should be used only after less-restrictive methods have failed. Ensure
compliance with ordering, assessment, and maintenance procedures. Historically,
vest or jacket restraints were used to prevent similar patient movement, but their
use has significantly decreased due to concerns for the potential risk for
asphyxiation with the device. Research suggests that waist restraints pose the same
potential risk for asphyxial death as vest restraints (Capezuti, et al., 2008).
Healthcare providers need to be aware of this potential outcome and weigh it
against possible benefit from use of the device.
6. A study of serotonin on people with anorexia nervosa found that;
A. people with anorexia are more likely to starve for long time.
C. people with anorexia have how low count of serotonin metabolites in CSF.
6. SEROTONIN
Addison's disease symptoms usually develop slowly, often over several months,
and may include: muscle weakness and fatigue, weight loss and decreased appetite,
darkening of skin (hyperpigmentation), low blood pressure (even fainting), salt
cravings, low blood sugar (hypoglycemia), nausea, diarrhea or vomiting, muscle or
joint pains etc. Sometimes, however, the signs and symptoms of Addison's disease
may appear suddenly. In acute adrenal failure (addisonian crisis), the signs and
symptoms may also include: pain in your lower back, abdomen or legs, severe
vomiting and diarrhea, leading to dehydration, low blood pressure and loss of
consciousness.
Your adrenal glands are composed of two sections: the interior (medulla) produces
adrenaline-like hormones; the outer layer (cortex) produces a group of hormones
called corticosteroids, which include glucocorticoids, mineralocorticoids and male
sex hormones (androgens).
Some of the hormones the cortex produces are essential for life (glucocorticoids
and mineralocorticoids). Glucocorticoids: These hormones, which include cortisol,
influence your body's ability to convert food fuels into energy, play a role in your
immune system's inflammatory response and help your body respond to stress.
Mineralocorticoids: These hormones, which include aldosterone, maintain your
body's balance of sodium and potassium to keep your blood pressure normal.
Androgens, male sex hormones, are produced in small amounts by the adrenal
glands in both men and women. They cause sexual development in men and
influence muscle mass, libido and a sense of well-being in men and women.
12. According to the information given in the passage, the statement that
“Androgen influences muscle mass and physical and mental nature of men and
women” is
A. True
B. False
C. Can’t say
D. Not given in the passage(s)
13. Doctors refer to the damage to the cortex of the kidney as
A. Primary adrenal insufficiency
B. Secondary adrenal insufficiency
C. Other fatal infections
D. Not given
Carpal tunnel syndrome has been around for a long time; meat packers began
complaining of pain and loss of hand function in the 1860s. Back then, these
complaints were largely attributed to poor circulation. The nature of work has
changed over the years; today, more jobs are highly specialized and require use of
only a small number of muscles repeatedly. With the growing numbers of people
using computers and keyboards, plus the focus on better health-care for workers,
carpal tunnel syndrome is of real concern to both employers and health-care
professionals.
Recent studies have shown that carpal tunnel syndrome, like all other cumulative
trauma disorders, is on the rise while other workplace injuries have leveled off.
Many companies are turning to physical therapists for help with designing and
implementing health promotion and injury prevention programs to protect their
employees from CTS.
People with CTS usually experience feelings of numbness, weakness, tingling, and
burning in their fingers and hands. If not treated, the symptoms may escalate into
acute, persistent pain. CTS can become so crippling that people can no longer do
their work or even perform simple tasks at home. At its most extreme, carpal
tunnel syndrome forces people to undergo surgery and miss many days of work, or
prevents them from working at all because their hand functions are permanently
impaired.
Carpal tunnel syndrome occurs in men and women of all ages, and is often found
in workers whose tasks require repeating the same motion in the fingers and hand
for long periods of time. CTS has surfaced among meat packers, assembly line
workers, jackhammer operators, and employees who spend hours working at a
computer or typewriter. Carpal tunnel syndrome shows up in athletes as well as
homemakers.
The U.S. Department of Labor has cited carpal tunnel syndrome, as well as other
cumulative trauma disorders, as the cause of 48 percent of all industrial workplace
illnesses. The disease affects more than five million Americans.
Physical therapists also work with employers and their engineering departments to
design and modify the work environment, helping to remove the causal factors of
CTS. If anyone has symptoms of carpal tunnel syndrome then consulting a
physical therapist or other qualified health care practitioner for an evaluation and
individualized treatment is always recommended
Text 2: Questions 15 to 22
15. According to the passage, CTS
A. is a cumulative trauma disorder
B. is caused due to weakness in musculoskeletal system
C. occurs due to weakness in ligaments between joints
D. all of the above
HEMOPHILIA
1 D
2 B
3 C
4 D
5 A
6 B
7 B
8 PEDIATRIC PATIENTS
9 FACTOR VIII/ F VIII
10 THE INTRINSIC SYSTEM
11 FEMELES / WOMEN
12 RENAL
13 DESMOPRESSIN / DESMOPRESSIN VASOPRESIN AMLOG / DDAVP
14 VON WILLEBRAND DISEASE (VWD)
15 CHRISTMAS DISEASE
16 TARGET JOINT
17 INHIBITORS HEMOPHILIA B
18 25,000-30,000 MALE
19 X-LINKED
20 HUMAN SYNOVIAL CELLS
READING SUB- TEST- ANSWER KEY
Elements of malpractice:
PART B: QUESTIONS 1-6
1. C
2. B
3. A
4. B
5. C
6. B
7. C
8. D
9. D
10. A
11. B
12. B
13. A
14. D
15. A
16. D
17. B
18. C
19. A
20. B
21. B
22. A