sickle cell disease and grand round notes

Question 1

what is a vaso-occlusive crisis

Answer 1

Sickled red blood cells clump together and occlude vessels, resulting in ischaemia of downstream tissues

The most common complaint is of pain, and recurrent episodes may cause irreversible damage

One of the most severe forms is the acute chest syndrome which occurs as a result of infarction in the lung parenchyma

Question 2

mx of vaso-occlusive crisis

Answer 2

Strong pain relief (IV opiates) is a priority
Oxygen as required and IV fluids are normally given in addition to this
Treat any suspected infections
Top-up transfusions may be required
Haematology input should be sought
Incentive spirometry and physiotherapy should additionally be used in cases of acute chest syndrome to minimise the risk of atelectasis

Question 3

what is a aplastic crisis

Answer 3

This is another possible complication of sickle cell disease and is caused by parvovirus B19. It will typically cause a sudden drop in haemoglobin and a low reticulocyte count due to an acute reduction in bone marrow function

Question 4

how is an acute chest crisis managed

Answer 4

High-flow oxygen, antibiotics and set up for an urgent exchange transfusion

Question 5

A small number of people with E. coli O157 infection go on to develop a serious condition called haemolytic uraemic syndrome (HUS). This can sometimes lead to kidney failure and death, although this is rare. The risk of HUS is highest in children aged under 5 years.

Answer 5

gi infection

low thyroxine seen in nephrotic syndrome

Question 6

paroxysmal AF do you need a DOAC

Question 7

how does kidney cancer caused raised Hb

Answer 7

The high EPO levels in the cyst fluid further confirmed EPO production in the renal cysts. EPO production by RCC cells and cyst epithelial cells in the left kidney may cause polycythemia- too many red blood cells so too much hb

Question 8

qt segment should be half of pg length

Answer 8

corrrect

Question 9

if someone has discitis think what other disease

Answer 9

infective endocarditis

need to take blood cultures and echo

MRI for discitis

Question 10

saddle anaethesia what conditon

Answer 10

can you feel when you wipe yourself

Question 11

how logn does inflammation take to settle in pneumona

not alwyas show on xray

Answer 11

6 weeks

cannot alwyas here either 50% of penumonia

Question 12

gingivitis linked to what conditions

Answer 12

Chronic gingiva inflammation has been thought to be associated with some systemic diseases such as respiratory disease, diabetes, coronary artery disease, stroke and rheumatoid arthritis
alzeihermers

Question 13

constipatin first line kids

Answer 13

macrogol

then senna
lactulose if cannot tolerate macrogol

Question 14

mx of a squint

Answer 14

Glasses – these can help if a squint is caused by a problem with your child’s eyesight, such as long-sightedness.
Eye exercises – exercises for the muscles that control eye movement may sometimes help the eyes work together better.

Question 15

best test to exclude sickle cell disease

Answer 15

Haemoglobin electrophoresis - measures different types in the blood

Question 16

high reticulocytes indicate what

Answer 16

haemolytic process

Question 17

folate if what vitmain

Answer 17

B9

Question 18

Vitamin B6 (pyridoxine) given to prevent

Answer 18

peripheral neuropathy
RIPE

Question 19

evidence of haemolysis

Answer 19

Investigations will show evidence of haemolysis (normocytic anaemia, high reticulocyte count, high LDH etc.

Question 20

heart, trachea, and aortic notch pulled to one side on a CXR what thinking - towards side

then goes from being grey to white what happened

Answer 20

collapse of lung more liekly over consolidation if whole lung

became effused