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PEDIA 1 > HEMA DIS NEWBORN > Flashcards

HEMA DIS NEWBORN Flashcards

1
Q

is defined as a reduction of the hemoglobin concentration or red blood cell (RBC) volume below
the range of values occurring in healthy persons.

A

Anemia

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2
Q

Physiologic Anemia of Infancy

A
  • At birth, normal full-term infants have higher hemoglobin (Hb) levels and larger red blood cells (RBCs) than do older children and adults.
  • progressive decline in Hb level begins and then persists for 6-8wk.
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3
Q

What causes the progressive decline?

A
  • Onset of Respiration at birth
  • There is also a gradual, normal developmental switch from fetal to adult Hb synthesis after birth
  • downregulation of erythropoietin (EPO) production
  • Hb concentration continues to decline until tissue oxygen needs become greater than oxygen delivery
    > this point is reached between 8 and 12 wk of age, when the Hb concentration is about 11 g/dL
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4
Q

The supply of stored reticuloendothelial iron, derived from previously degraded RBCs,remains sufficient for this renewed Hb synthesis, even in the absence of dietary iron intake, until approximately

A

20 wk. of age

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5
Q

Blood transfusions (PRBC) in NEWBORN recommended

A

RBC volume of 10-15 mL/kg is recommended

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6
Q

Iron supplementation in newborn

A
  • at 1mo of age and continuing until about 1 yr.
  • Starting dose is 1-2 mg/kg/day of elemental iron.
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7
Q
  • most widespread and common nutritional disorder in the world
  • Most iron in neonates is in circulating hemoglobin
    > iron stores are usually sufficient for blood formation in the 1st 6-9 mo of life in term infants
    > Delayed (1-3 min) clamping of the umbilical cord can improve iron status and reduce the risk of iron
    deficiency
    > anemia caused solely by inadequate dietary iron usually occurs at 9-24 mo of age and is relatively
    uncommon thereafter.
A

lron-Deficiency Anemia

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8
Q
  • is the most recognized clinical sign
  • not usually visible until the hemoglobin falls to 7-8 g/dL
  • readily noted in the palms, nail beds, or conjunctivae
A

Pallor

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9
Q
  • Happens when the hemoglobin level falls to <5 g/dL
  • Ifthe hemoglobin continues to fall
A
  • anorexia, and lethargy develop, and systolic flow murmurs
  • tachycardia and high output cardiac failure
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10
Q

LAB Findings in Iron Def Anemia
- serum ferritin
- transferrin
- MCV
- MCH
- WBC
- Hb

A
  • reduced serum ferritin
  • Increased serum transferrin
  • decrease in mean corpuscular volume (MCV) and mean corpuscular hemoglobin (MCH)
  • White blood cell (WBC) count is normal, but thrombocytosis is often present
  • An increase in hemoglobin 21 g/dL after 1 mo of iron therapy is usually the most practical
    means to establish the diagnosis.
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11
Q

How much dosage is adequate in px with Iron Def Anemia

A

A daily total dose of 3-6 mg/kg of elemental iron in 1 or 2 doses is adequate

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12
Q
  • is defined as the premature destruction of red blood cells (RBCs)
  • Anemia results when the rate of destruction exceeds the capacity of the marrow to produce
    additional RBCs
  • Normal RBC survival time is 110-120 days
A

Hemolytic Anemias

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13
Q

Classification of hemolytic anemias:

A
  • Intrinsic
  • Extrinsic
  • Inherited
  • Acquired
  • Immune
  • Nonimmune mediated
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14
Q

Daignostic Clues Based in RBC Shape Suggestive of Hemolytic Anemia

  • Sickle cells >
  • Target Cells >
  • Spherocytes >
  • “Bite” Cells >
A
  • Sickle cells > Sickle Cell Disease
  • Target Cells > hemoglobinopathies
  • Spherocytes > hereditary spherocytosis
  • “Bite” Cells > G6PD deficiency
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15
Q
  • Common cause of inherited hemolytic anemia
  • Described in patients of all ethnic groups
  • An intrinsic defect of the erythrocyte membrane and an intact spleen that selectively retains,
    damages and removes the erythrocyte
  • Ankyrin or spectrin > most common molecular defect
  • Clinical manifestations
    > 75% transmitted as an autosomal dominant trait
    > Anemia
    > Hyperbilirubinemia
  • Severity varies:
    > Asymptomatic
    > Fatigue, pallor and intermittent jaundice
    > splenomegaly
A

Hereditary Spherocytosis

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16
Q
  • refers to a group of genetic disorders of globin-chain production in which there is an imbalance between the a-globin and B-globin chain production
  • unbalanced a- and B-globin chain production leading to ineffective erythropoiesis.
  • 2 types of Thalassemia:
    + B Thalassemia
    • result from a decrease in B-globin chains, which results in a relative excess of a-globin chains
      + a Thalassemia
    • there is an absence or reduction in a-globin production usually due to deletions of a-globin genes.
A

Thalassemia Syndromes

17
Q
  • If not treated, children with homozygous BO-thalassemia usually become symptomatic from progressive anemia, with profound weakness and cardiac decompensation during the 2nd 6 mo of life.
  • classic presentation of children with severe disease:
    > Maxilla hyperplasia
    > Flat nasal bridge
    > Frontal bossing
    > Pathologic bone fractures
    > Marked hepatosplenomegaly
  • LAB FINDINGS
    > Identified on newborn screening (expanded)
    > Progressive anemia after the newborn period, often < 6mg/dL
    > Hemoglobin electrophoresis > not definitive
    > DNA diagnosis of the B thalassemia nutation is recommended
  • Management
    > Transfusion therapy
    + Generally given at intervals of 3-4 weeks
    + Goal to maintain Hb level of 9.5-10 g/dL
    + Monitor for iron overload
A

Homozygous B Thalassemia
(Thalassemia Major, Cooley Anemia)

18
Q
  • identified in the newborn period by the increased production of Bart hemoglobin (y4 ) during fetal
    life and its presence at birth
  • occur most frequently in Southeast Asia
  • The different phenotypes in a-thalassemia largely result from whether 1 (a+ -thalassemia) or both (
    a0 -thalassemia) a-globin genes are deleted in each of the 2 loci.
A

a-Thalassemia Syndromes

19
Q
  • Is not identifiable hematologically
  • No alterations are noted in the MCV and MCH
A

a-Thalassemia Syndromes
- The deletion of 1 a-globin gene (silent trait)

20
Q
  • a-globin alleles can be lost in a trans (-a/-a) or cis (a,a/-SEA) configuration
  • cis deletions are most common seen in persons from or descended from Asia or the Mediterranean region
  • Manifest as a microcytic anemia than can be mistaken for iron deficiency anemia
A

a-Thalassemia Syndromes
- The deletion of 2 a-globin genes results in a-thalassemia trait

21
Q
  • The deletion of 4 a-globin genes causes profound anemia during fetal life
  • The deletion of 3 a-globin genes leads to diagnosis of
A
  • hydrops fetalis
  • HbH disease
22
Q
  • X-linked inherited disorder
  • DEFECT: glucose 6 phosphate dehydrogenase
    > Helps protect RBCs from oxidative stress
  • triggered by infection, drugs, or ingestion of fava beans
  • hemolysis ensues in about 24-48 hr after a patient has ingested a substance with oxidant properties
  • Laboratory Findings
    > Heinz bodies, precipitated hemoglobin
    > A peripheral smear will show “bite” cells
A

Glucose-6-Phosphate Dehydrogenase Deficiency

23
Q
  • is defined as a central hemoglobin or hematocrit (Hct) exceeding 2 standard deviations (SD) above
    the normal value for gestational and postnatal age.
  • full-term infant is therefore considered when the hemoglobin concentration is >22 g/dL or Het is >65%.
  • Etiologies of neonatal polycythemia are numerous but can be grouped into two broad categories:
    > based on passive RBC transfusion into the fetus
    > increased intrauterine erythropoiesis
A

Neonatal Polycythemia

24
Q
  • result from hyper viscosity (sluggish blood flow causing decreased tissue perfusion)
  • irritability, lethargy, tachypnea, respiratory distress, cyanosis, feeding disturbances,
    hyperbilirubinemia, hypoglycemia, and thrombocytopenia
  • Severe complications include:
    > seizures, stroke, b
    > pulmonary hypertension,
    > Necrotizing enterocolitis (NEC),
    > renal vein thrombosis, and renal failure
A

Neonatal Polycythemia

25
Q
  • is the process of blood clotting in areas of blood vessel injury
  • If clotting is impaired, hemorrhage occurs
A

Hemostasis

26
Q

The main components of the hemostatic process are:

A
  • the vessel wall
  • plateletss
  • coagulation proteins
  • anticoagulant proteins
  • and fibrinolytic system.
27
Q

In acquired hemostatic disorders, there are frequently multiple problems with homeostasis that
perturb and dysregulate hemostasis.

A
  • A primary illness (sepsis)
  • its secondary effects (shock and acidosis)
  • activate coagulation and fibrinolysis and impair the host’s ability to restore normal hemostatic
    function.
28
Q
  • When sepsis triggers
  • platelets, procoagulant clotting factors, and anticoagulant proteins are consumed, leaving the
    hemostatic system unbalanced and prone to bleeding or clotting
A

Disseminated Intravascular Coagulation

PEDIA 1 (12 decks)

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