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haematology Flashcards

1
Q

glanzmaen thrombostenia

A

bleeding disorder that is characterized by prolonged or spontaneous bleeding starting from birth. People with Glanzmann thrombasthenia tend to bruise easily, have frequent nosebleeds (epistaxis), and may bleed from the gums.

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2
Q

Immune thrombocytopenic purpura alternative name and what is it

A

Werholf

is a type of thrombocytopenic purpura defined as an isolated low platelet count with a normal bone marrow in the absence of other causes of low platelets so diagnosis requires exclusion. autoimmune + .

characteristic rash palpable

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3
Q

type of anemia in iron deficiency

diamond blackfan labs cause

A

microcytic

red blood cell aplasia - a plastic anemia congenital, all rbc lines will be gone so no rteiculocytes its a macrocytic anemia, dodgey thumbs
cause seems to be ribosomopathy
TX- CS , RBC transfusions

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4
Q

sick cell anemia is

AIHA tx and siagnosis

A

NORMOCYTIC

immunosuppresnat till yu get a negatuc coombs test

coombs test and spheroctosis !

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5
Q

anemia of chronic disease

A

microcytic (lecture)or normochronic(google)

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6
Q

haemolytic anemias are what type

pearson syndrome

A

normocyitc?

siderblastic anemia
mitcohondiral problem
macrocytic
liver prolems
lactic acidosis

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7
Q

which 2 conditions have anemia but increased RBC

A

SICKELE CELL AND THALLASEMIA

fanconi, diamond, pearson

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8
Q

constellation of thalassemia

fanconi anemia and siagnosis

A

low hb, low MCV! increased abc

congenital , short, onset around 4 years old
cafe au lait spots
malformations of urinary trcat

increased chromosome fragility !

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9
Q

how to prove thalssemia

tx glucos 6 phosphate

A

electrophoresis of the chains

rarely need transfuison just stop the ttrigeer supportive tx xygen etc

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10
Q

theory for hemmorrhaigc disease of newborn

A
  1. IV vit k 1mg/kg bw for 3 days

2. FFP 10-15ml preterm

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11
Q

TTP VS ITP

A

check tables

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12
Q

TTP

A

platelet count not a slow as iTP
but clots form and platelets low
you have haemolytic anemia (shsitocytes)

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13
Q

why would you do a urine electrophoresis

A

prove whether protein is tubular or glomerular

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14
Q

Type of child is more likely to present with jaundice in a uni tract infection

A

<3months

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15
Q

uti main symptoms

A

FEVER ESPCIALLI IN YOUNG KIDS
FAILURE TO THRIVE
JAUNDIC <3

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16
Q

<3

>3 YEARS

A

<3 urgent microscopy +culture

>3 leukocyte esterase +nitrate+dipstick

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17
Q

criteria for pyelonephritis

A

> 10*3

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18
Q

criteria for lower utilities

A

<10*3

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19
Q

atypical UTI

non immune causes of hemolutic anemia

A

poor urine flow , failure to respond to tx whiting 48 h, non e.coli organisms

membrane deficits : minkowski
enzyme : glucose 6 p
microangiopathic : dic, ttp, hus
macroangiopathic : prosthetic devcies

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20
Q

decreased reticulocyte count

A

bone marrow failure

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21
Q

normal reticulocyte count

NORMAL PLT COUNT

SEVERE THROMBOCYTPOENIA

A

0.5-2.5%

> 150

<20 -spontaneous bleeding

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22
Q

lead poisoning

A

microcytic anemia and basophils stippling
gum grey line
constipatin and vomititng

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23
Q

tx of anemia of chronic disease

A

need to treat the underlying disease if you just give iron - useless because its not the fact that you dont have enough its that YOU CANT USE THE IRON THERE

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24
Q

normal blood volume of term baby

where is iron absorbed

A

80 ml

duodenum

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25
Q

cafe au lait spots

A

falcon anemia
pancytopenia
typically presents around 8 years old

BM transplant + androgen therapy(raised ur rb + plt)

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26
Q

type of anemia of aplastic anemia

charcarcteristics of aplastic anmeia (lecture slide)

A

MACRO

necrotic lesions at entry sites - buccal mucosa and perianal lesiosn (neutophils) lack of lymph nodes and abscenes of HSM

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27
Q

SIGNS OF THALAMSEIA MAJOR

A

FRONTAL BOSSING
MAXIALLARY OVERGROWTH
EXTRAMEDULLARY HEMATOEPOESISASIS

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28
Q

TX thalsemmia

A

chelating : desferroxiamine
splenectomy
bm transpalnt

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29
Q

alpha thalseemia major

A

not compatible with life most fetuses are still born or die in utero- hydrops fetalis

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30
Q

when should you suspect thalssemia

A

when mcv is way lower than hb

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31
Q

thalaseemia labs

A

MCV- very low
HB low
high levels of fatal hb >90%
HBA - absent

32
Q

what would electrophoresis show in thalassemia

A

increase hb f or increased a2

33
Q

specific cells thalseemia

A

target cells

34
Q

Guthrie test

A

Best for sickle cell anaemia heel prick test newborn

35
Q

tx sickle cell

A

increase fetal hb
hydrocarbamide
BM TRANSPLANT IF FAILS

36
Q

Minkowski chauffeur disease inheritance

triad

A

ad

hemolytic crisis
splenomegaly
spherocytes

37
Q

diagnosis of Minkowski

A

Enzyme is typically low but during the crisis it actually will be high so you do repeat after

38
Q

Minkowski chauffer symptoms

A

gallstones
family history
splenomegaly
abnormal rbc morphology(spherocytes))

39
Q

Heinz body

A

glucose 6 phospahte

40
Q

treatment for Minkowski

A

folate supplements
rbc transfusions

splectomy- but try to avoid till after 7

41
Q

Minkowski chauffer type of anemia

A

normocytic

42
Q

osmotic fragility test done for

glucos 6 ph symptoms

A

thlassmia and hedrideaty spehrocytosi

fever, jaundice, adominal pain, DARK COCO COLA URIEN

43
Q

tx for minnowki

A 
folate supplements !!!!!!!
splenectomy > 7 to avoid sepsis 
before splenectomy give vaccines 
blood transfusion in crisis 
cholecystectomy
44
Q

blood smear minnows

diagnose for G6P

A

spheorcytes

enzyme assay but only after reticulocytes have normlaised!!!

lack central pallor and smaller than rbc

45
Q

labs minnkowski

drinking goats milk you are deficent in

A

increased br indirect
LDH
increased reticulocytes
decreased haptoglobin (haemolytic)

folate!!!

46
Q

signs of hemolysis

A

ldh
decreased haptoglobin
br

47
Q

exotic face is seen in

coca cola urine implies

A

thalassemia

intravscular hemolysis! like g6pd

48
Q

specific values for minkwoski

A

normocytic (size)

but mch is increased (hb)

49
Q

dx of glanzeman disease

A

normal with ristocetin but abnormal with adrenalin and adp

50
Q

dx of vwf

A

abnormal with ristocetin

aptt increased

51
Q

main type of bleeding in VWF

A

MUCOSAL IS LEADING

52
Q

dead definition of hypertension

A

> 95% on at least 3 different occasions

53
Q

classification of blood pressure

A

Normal: <90th BP percentile
x Prehypertensive: 90th -95th
x Stage 1 HTN: 95th – 99th (+5mmHg)
x Stage 2 HTN >99th (+5mmHg)

54
Q

What type of hypertension are babies more likely to have

A

secondary

55
Q

HTN In teens

A

most likely essential hypertension

56
Q

causes of hypertension in newborns

A

umbilical artery catheterisation + renal artery thrombosis

57
Q

Metanephrines

A

used to check for pheocytochroma

58
Q

1st line treatment for hypertension in kinds

A

calcium channel blocker

59
Q

signs of hypertensions

A

headache seizures nose bleeds stroke, mi

60
Q

Hypertensive criss vs hypertensive urgency

A

crisis: acute increase in both systolic BP and DBP with end organ damage.g stroke, encephalopathy
urgency: without end organ damage

61
Q

tx for HTN crisis

A

ICU, parenteral drugs CA channel blocker nicarpidine , labetelol, esmolol, sodium itroprusside . once stabilised then be on Aral antihypertensives over 1-2 days

62
Q

tx urgency hTN

A

ORAL 1-2 DAYS

63
Q

what’s the most important thing to rememebr about a child with UTI

A

if the child has a fever but there are no other signs of infection! UTI !!!!

64
Q

markers for increased consumption

A

thrombin time (DIC has thrombin excess!)
d dimers

65
Q

which lab parameter does vit k mainly affect

A

PT time ( extrinsic) as majority factors are there

66
Q

pathophys of minkowski

A

change of shape, loose concavity and become spherocytes and more prone to stasis and so get stuck in the spleen.EXTRAVASCULAR hemolysis

67
Q

what is the mutation in SS

A

glutamate replaced by valine

68
Q

HEMOPHILIA A

factors dependant on vitamin K

A

factor 8

2 7 9 10 +protein C +S

69
Q

diagnosis of hemophilia

A
    • fH
  2. apTT prolonged but normal pT
70
Q

when would parents typically bring child in

A

when learning to walk
keep bleeding after teeth eruption or circumcision

71
Q

dx of hemophilia

A

MONOARTHRITIS - you cant tell the difference the only clue perhaps would be brusing in hemophilia

72
Q

complications for hemophilia

A

immobility due to stiff muscles which healby fibrosis - hematomas

nerve damage

vasculitis?

joint defromities

73
Q

what drugs are CI in hemophilia

A

aspirin and nsaids (upper gi bleeding risk)

74
Q

when can we use desmopressin

A

hemophilia A its uneffective for hemophilia B

also for VWF

75
Q

what does VWF do

A

has 2 functions

1) allows the platelets to adhere together
2) binds to factor 8 to keep it activated

76
Q

type of bleeding in VWD and tX

A

mucousal bleeding

desmopressin + factor 8 transfusion

peds (26 decks)

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