sickle cell disease

Question 1

what would you find in the blood film of sickle cell disease ?

Answer 1

sickle cells

Question 2

what would you find in the blood film in HbSc

Answer 2

target cells

Question 3

which test will reveal the genotype of the sickle cell ?

Answer 3

Hb electrophoresis or HPLC

Question 4

what are the clinical syndromes associated with sickle cell disease ?

Answer 4

vaso-occlusive crisis 
aplastic crisis
sequestrian crisis
hemolytic crisis 
infections

Question 5

what are aplastic crisis commonly associated with ?

Answer 5

parvovirus B19

Question 6

what is the most common acute presentation of sickle cell anemia ?

Answer 6

acute painful episodes

Question 7

what are the precipitating factors of sickle pain ?

Answer 7

infection 
dehydration 
cold damp conditions 
unaccustomed exercise 
stress

Question 8

what are the presentations of vaso-occlusive crises ?

Answer 8

dactylitis 
girdle syndrome
chest syndrome 
avascular necrosis of the hip 
priapism

Question 9

what is girdle syndrome ?

Answer 9

pain in the lower abdomen, localized ileus due to sickling in the bowel

Question 10

what is the drug management of acute sickle pain ?

Answer 10

opioid analgesics such as paracetamol
diamorphine
morphine
oxycodone

Question 11

what is acute chest syndrome ?

Answer 11

acute illness that may progress to ARDS and is characterized by :
fever 
respiratory symptoms 
a new pulmonary infiltrate  
( hypoxia not included )

Question 12

what are the causes of ACS ?

Answer 12

pulmonary infection
fat embolism
pulmonary infarction
hypoventilation

Question 13

what are the clinical features of ACS?

Answer 13

onset usually within 24-72 hours of severe crisis 
fever 
shortness of breath 
bilateral crackles 
pulmonary infiltrates ( alveolar consolidation of the lower lobe )

Question 14

what are the complications of ACS?

Answer 14

neurological complications

multi organ failure

Question 15

what is the treatment of ACS ?

Answer 15

emergency blood transfusion 
IV fluids 
IV antibiotics 
effective pain relief. 
respiratory support

Question 16

what is priapism ?

Answer 16

painful resistant erection due to vaso-occlusive obstruction of the venous drainage of the penis

Question 17

when does sequestrian crisis happen ?

Answer 17

young children under the age of 5

old people with HbSc

Question 18

what is a sequestrian crisis ?

Answer 18

pooling of large volumes of blood in the spleen or the liver

Question 19

what are the ned results of sequestrian crisis ?

Answer 19

hypotension and hypovolemic shock

Question 20

what are infections in sickle cell disease a result of ?

Answer 20

hyposplenism

Question 21

how do we manage acute complications ?

Answer 21

hawi 3al 3ayann
oxygen fluids analgesia
transfusion if very anemic
DVT prophylactic

Question 22

why may a new born with sickle cell appear normal or well ?

Answer 22

dur to high levles of HbF

Question 23

what is the pattern of inheritance associated with sickle cell disease ?

Answer 23

autosomal reecessive

Question 24

what is the difference between HbSS and HbSC ?

Answer 24

HbSS - is the most severe from of the disease
HbSCC - less severe but retinopathy and avascular necrosis are common associated problems

Question 25

what disease are patients with sickle cell resistant to ?

Answer 25

malaria

Question 26

what is present in blood film of HbSC ?

Answer 26

target cells

Question 27

what are thee investigations needed to make a diagnosis of sickle cell anemia ?

Answer 27

gel electrophoresis
high performance liquid chromatography
sickle cell solubility

Question 28

what is the prognosis of aplastic crisis ?

Answer 28

resolves spontaneously

Question 28

what type of infections are associated with hyposplenism ?

Answer 28

encapsulated organsims