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Blood Flashcards

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1
Q

What is the physiological definition of anemia?

A

Hemoglobin level too low to meet cellular oxygen demand.

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2
Q

What is the practical definition of anemia?

A

Hemoglobin level at least 2 standard deviations (SD) below the mean for age, gender, and race.

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3
Q

What are three reductions that characterize anemia?

A
  • Red cell/mm3
  • Hematocrit
  • Blood hemoglobin concentration
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4
Q

What is the hemoglobin threshold for anemia in newborns?

A

<13.5 g/dL

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5
Q

What is the most common type of anemia in infants and children?

A

Iron deficiency anemia.

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6
Q

What are the main causes of impaired red cell function leading to anemia?

A
  • Deficiencies
  • Decreased dietary intake
  • Increased demand
  • Decreased absorption
  • Impairment of red cell production
  • Increased loss
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7
Q

What are the dietary factors that enhance iron absorption?

A
  • Meat
  • Fish
  • Poultry
  • Seafood
  • Gastric acid
  • Ascorbic acid
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8
Q

What are the dietary factors that inhibit iron absorption?

A
  • Phosphate
  • Calcium
  • Tea (tannic acid)
  • Coffee
  • Colas
  • High doses of minerals
  • Bran/fiber
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9
Q

What is the primary nutrient deficiency in iron deficiency anemia?

A

Iron.

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10
Q

What is the typical iron absorption requirement during the first 15 years of life?

A

0.8 mg of iron daily.

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11
Q

What is the most common nutritional disorder in the world?

A

Iron deficiency anemia.

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12
Q

What is the major clinical manifestation of iron deficiency anemia?

A

Pallor.

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13
Q

Fill in the blank: The term for the desire to ingest non-nutritive foods is _______.

A

Pica.

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14
Q

What is the typical hemoglobin level associated with severe iron deficiency anemia?

A

<5 g/dL

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15
Q

What is the best test to confirm storage iron in the laboratory diagnosis of iron deficiency anemia?

A

Serum ferritin.

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16
Q

What is a common laboratory finding in peripheral blood for iron deficiency anemia?

A

Hypochromic, microcytic red blood cells.

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17
Q

What is the Mentzer Index value indicating iron deficiency anemia?

A

> 13.

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18
Q

What is the recommended therapeutic dose of iron for mild iron deficiency anemia?

A

3 mg/kg/day.

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19
Q

What is the recommended iron supplementation for full-term infants after 4 months?

A

15 mg/day.

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20
Q

What condition is caused by a loss of function mutation in the TMPRSS6 gene?

A

Iron refractory iron deficiency anemia.

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21
Q

What are the causes of vitamin B12 deficiency?

A
  • Inadequate nutrients
  • Malabsorption
  • Failure to secrete intrinsic factor
  • Inherited defects
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22
Q

What is the significance of elevated methylmalonic acid in laboratory findings?

A

Indicates vitamin B12 deficiency.

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23
Q

What are common clinical features of cobalamin and folate deficiency?

A
  • Pallor
  • Lethargy
  • Fatigability
  • Anorexia
  • Sore red tongue
  • Glossitis
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24
Q

Fill in the blank: The recommended intake of folate for pregnant women is _______.

A

3000–4000 μg/day.

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25
What is the treatment for folic acid deficiency?
Folic acid: 2-5 mg/day x 2-3 weeks.
26
What is the treatment for vitamin B12 deficiency with neurological manifestations?
1 mg IM x 2 weeks.
27
What type of anemia is characterized by congenital red cell aplasia?
Congenital hypoplastic anemia.
28
What is the term for the condition characterized by an increased destruction of red blood cells?
Hemolytic anemia.
29
What condition is associated with hereditary spherocytosis?
Membrane defect leading to hemolytic anemia.
30
What is the daily folate requirement for adults?
3.1 μg/kg/day
31
What is the daily cobalamin requirement for adults?
1 μg/kg/day
32
What is the folate requirement for pregnant or lactating women?
3000–4000 μg/day
33
What is the primary etiology of congenital hypoplastic anemia?
Autosomal dominant (50%)
34
What mutation is associated with Diamond-Blackfan anemia?
Mutation on gene RPS19
35
What are two common clinical manifestations in infants with congenital hypoplastic anemia?
* Pallor * Rare hydrops
36
What percentage of patients with congenital hypoplastic anemia have congenital anomalies?
40-50%
37
What is the hallmark laboratory finding in Diamond-Blackfan anemia?
Elevated erythrocyte adenosine deaminase enzyme
38
What are the key diagnostic criteria for Diamond-Blackfan anemia?
* Normochromic macrocytic anemia * Reticulocytopenia * Normocellular marrow with selective deficiency of red cell precursors
39
What is the definitive therapy for Diamond-Blackfan anemia?
Bone marrow transplant (HSCT)
40
What is the median survival for patients with Diamond-Blackfan anemia?
40 years
41
What is the most common age group affected by transient erythroblastopenia?
6-30 months
42
What is a common clinical feature of transient erythroblastopenia?
Moderate to severe normocytic anemia
43
What is the prognosis for transient erythroblastopenia?
Spontaneous recovery within weeks to months
44
What laboratory evidence indicates extravascular hemolysis?
* Increased B1 (indirect bilirubin) * Increased LDH * Increased fecal and urinary urobilinogen
45
What are the two main classifications of hemolytic anemia?
* Cellular defects (intrinsic) * Extracellular defects (extrinsic)
46
What is the most common defect in hereditary spherocytosis?
Defect in RBC skeletal proteins
47
What is the prevalence of hereditary spherocytosis?
1:5000
48
What laboratory test is used to diagnose hereditary spherocytosis?
Peripheral blood smearo Hallmark: plenty spherocytes Reticulocytosis Bonemarrow: erythroid hyperplasia Osmotic fragility test
49
What is the hallmark laboratory finding in G6PD deficiency?
Increased reticulocyte count
50
What are the clinical symptoms of class I G6PD deficiency?
Chronic hemolytic anemia
51
What common food triggers hemolysis in G6PD deficiency?
Fava beans
52
What is the treatment for paroxysmal nocturnal hemoglobinuria?
Splenectomy
53
What is the main clinical manifestation of paroxysmal nocturnal hemoglobinuria?
Intravascular hemolysis
54
What is the primary laboratory test for diagnosing paroxysmal nocturnal hemoglobinuria?
Flow cytometry
55
What is a major group of disorders related to hemoglobin abnormalities?
Hemoglobinopathies
56
What is the predominant hemoglobin in adults?
HbA (α2β2)
57
What is the basic pathology of sickle cell disease?
Substitution of valine instead of glutamic acid in #6 position of β chain
58
What are the three categories of sickle cell anemia?
* Vaso-occlusive sickle cell crisis * Acute sequestration * Aplastic crisis
59
What is the hallmark of vaso-occlusive sickle cell crisis?
Painful crisis
60
What is the hallmark of Sickle Cell Disease (SCD)?
Painful crisis
61
What is acute sequestration?
Acute splenic sequestration, leading to splenomegaly
62
What is an aplastic crisis?
Temporary cessation of bone marrow activity due to suppression by infection
63
What are the most frequent sites of occlusion in vaso-occlusive sickle cell crisis?
* Lumbosacral * Spine * Ribs * Knees * Clavicle * Shoulder * Iliac crest * Femur
64
What is priapism?
Trapped blood in the cavernous blood vessels of the penis, causing rigidity and erection
65
What is acute chest syndrome?
New pulmonary infiltrate on chest X-ray involving at least 1 lung segment with symptoms
66
What are the symptoms required to diagnose acute chest syndrome?
* Temp. >38.5°C * Chest pain * Respiratory symptoms * Hypoxemia
67
What causes acute splenic sequestration?
Destruction of RBCs leading to splenomegaly
68
What is the main cause of aplastic crisis?
Human parvovirus B19
69
What is the most common cause of death in children with SCD?
Infection
70
What are common chronic organ damages due to SCD?
* Cardiovascular * Renal * Hepatobiliary * Skin * Ear * Skeletal * Lungs * CNS
71
What are the laboratory diagnosis findings for SCD?
* Target cells * Sickle cells * Poikilocytes * Nucleated RBC * Howell Jolly bodies
72
What is the treatment for painful crises in SCD?
NSAIDs
73
What is the only curative hope for SCD?
Bone marrow transplant (BMT)
74
What is the survival rate probability at 20 years for SCD patients?
95%
75
Fill in the blank: The most common hematologic disease of infancy and childhood is _______.
Iron Deficiency Anemia
76
True or False: Folate supplementation reduces the incidence of neural tube defects by 50-70%.
TRUE
77
What condition is caused by the substitution of valine instead of glutamic acid in the β chain?
Sickle Cell Disease
78
What is the treatment of choice for hereditary spherocytosis?
Splenectomy
79
What leads to development of megaloblastic anemia in a strict vegetable diet?
Folic acid deficiency
80
Which food has the highest iron content?
Beef
81
True or False: The world's leading cause of iron deficiency anemia is blood loss.
TRUE

Pediatric blood disorders (1 decks)

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