Haemato Flashcards

1
Q

What is the most common nutritional deficiency in children?

A

Iron deficiency anemia (IDA)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What is the highest burden age group for IDA?

A

Children less than 5 years old

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What are the ferritin and hemoglobin cutoffs for IDA in children?

A

Ferritin <15 mcg/L, Hb <11 g/dL (6 months-<5 y/o), <11.5 g/dL (5-<12 y/o), <12 g/dL (12-15 y/o)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What is the main regulatory hormone for iron homeostasis?

A

Hepcidin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What are common causes of IDA?

A

Chronic blood loss, increased iron demand, malabsorption, inadequate dietary intake

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What are the clinical features of severe IDA?

A

Koilonychia, angular stomatitis, fatigue, pallor, cardiomegaly, tachypnea, pica

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What are complications of IDA?

A

Impaired neurodevelopment, growth issues, weakened immunity

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What are perinatal risk factors for IDA?

A

Maternal iron deficiency, prematurity, perinatal hemorrhagic events

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What dietary factors contribute to IDA in infancy?

A

Lack of iron supplements, cow’s milk intake, low-iron formula, malabsorptive diseases

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What is the first-line investigation for IDA?

A

Complete blood count (CBC)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What are the characteristic findings on peripheral blood smear in IDA?

A

Microcytic hypochromic RBCs, increased central pallor, pencil cells, target cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

How is IDA managed in infants?

A

Exclusive breastfeeding, iron supplements, iron-rich complementary foods, vitamin C intake

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What is the pathophysiology of Henoch-Schönlein Purpura (HSP)?

A

IgA-mediated immune complex vasculitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What are the classical triad symptoms of HSP?

A

Purpuric rash, arthralgia, abdominal pain

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What are complications of HSP?

A

Intussusception, nephritis, CNS involvement, orchitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What is the management for HSP?

A

Supportive care, prednisolone for severe cases

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What is the most common bleeding disorder in children?

A

Idiopathic Thrombocytopenic Purpura (ITP)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What is the usual age of presentation for ITP?

A

1-7 years old

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What is the hallmark finding in ITP pathophysiology?

A

Autoantibodies against platelet glycoproteins IIb/IIIa

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What platelet count is associated with spontaneous bleeding in ITP?

A

<20,000 cells/mm³

21
Q

What is the typical clinical presentation of ITP?

A

Petechiae, purpura, epistaxis, oral mucosal bleeding

22
Q

What is the first-line treatment for ITP?

A

Observation if platelets >20,000/mm³; corticosteroids, IVIG for severe cases

23
Q

What is the definitive treatment for chronic ITP?

A

Splenectomy

24
Q

What is the genetic inheritance pattern of thalassemia?

A

Autosomal recessive

25
Which hemoglobin components are affected in thalassemia?
Alpha (HBA) and beta (HBB) globin chains
26
What are characteristic skeletal findings in beta-thalassemia major?
Frontal bossing, maxillary overgrowth (chipmunk facies)
27
What are complications of iron overload in beta-thalassemia?
Cardiac failure, liver cirrhosis, endocrinopathies, renal insufficiency
28
What is the management of beta-thalassemia major?
Regular transfusions, iron chelation therapy, folic acid, splenectomy if needed
29
What is the cause of alpha-thalassemia?
Gene deletions in the alpha-globin locus
30
What is the fatal form of alpha-thalassemia?
Hb Barts (hydrops fetalis)
31
What is the hallmark pathophysiology of thalassemia?
Imbalanced globin chain synthesis leading to ineffective erythropoiesis and hemolysis
32
What are the characteristic RBC findings in thalassemia?
Hypochromic microcytic RBCs, target cells, anisopoikilocytosis
33
How is thalassemia diagnosed?
Hemoglobin electrophoresis, genetic testing
34
What are the main types of hemophilia?
Hemophilia A (Factor VIII deficiency) and Hemophilia B (Factor IX deficiency)
35
What is the inheritance pattern of hemophilia?
X-linked recessive
36
What is a characteristic bleeding feature in hemophilia?
easy Bruising Epistaxis Gum bleeding Hemarthrosis (bleeding into joints)
37
What is the screening test for hemophilia?
Prolonged APTT with normal PT and platelet count
38
What is the definitive test for hemophilia?
Factor VIII or IX activity assay
39
What are the main complications of hemophilia?
Joint destruction, viral infections, inhibitor development
40
What is the primary treatment for hemophilia A?
Factor VIII replacement therapy
41
What is the primary treatment for hemophilia B?
Factor IX replacement therapy
42
What medications should be avoided in hemophilia?
Aspirin, NSAIDs
43
What are alternative pain management options in hemophilia?
Paracetamol, opioids if necessary
44
What prophylactic measures should be taken for hemophilia patients?
Regular factor replacement, vaccination for Hep B, medical alert identification
45
What is the classification of hemophilia severity?
<1% factor level = severe, 1-5% = moderate, 5-25% = mild
46
What viral infections are hemophilia patients at risk for?
Hepatitis B, Hepatitis C, HIV
47
How is hemophilia inherited in families with no prior history?
Spontaneous mutations
48
Why should IM injections be avoided in hemophilia patients?
Risk of deep muscle bleeding
49
What is the role of desmopressin (DDAVP) in hemophilia?
Increases Factor VIII levels in mild hemophilia A