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37 Cards in this Set
- Front
- Back
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What is sickle cell disease?
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Autosomal recessive disorder, an abnormal hemoglobin leads to chronic hemolytic anemia with numerous clinical consequences. Single DNA base change substitution of valine for glutamine.
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What contributes to the formation of sickled red blood cells?
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Hemoglobin S is unstable and polymerizes in the setting of various stressors, including hypoxemia, dehydration and acidosis.
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What are the Symptoms and Signs of sickle cell disease?
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Chronic hemolytic anemia produces jaundice, pigment gallstone, splenomegaly, and poorly healing ulcers.
Life-threatening severe anemia can occur during hemolytic or aplastic crises, generally associated with viral or other infection or by folate deficiency. |
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What organ systems are affected with repeated episodes of vascular occlusion?
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heart, lungs, kidney, bones and liver. Ischemic necrosis of bone occurs, leading to osteomyelitis due to staphylococci or salmonellae.
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What will peripheral blood smear show in sickle cell anemia?
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Sickled red blood cells and target cells. In addition, the red blood cell in the lower central area of the field contains a hard peripheral inclusion called a Howell-Jolly body.
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What is Howell-Jolly body?
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These area nuclear remnants and is the consequence of hyposplenism.
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How do you dx sickle cell anemia?
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Diagnosis of sickle cell anemia is confirmed by hemoglobin electrophoresis.
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What are the treatments when an acute painful episodes occurs?
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Precipitating factors should be identified and infections treated if present. Hydration, and provide oxygen when needed.
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What is the treatment for an Acute vaso-occlusive crises?
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exchange transfusion.
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What is the indication for exchange transfusion?
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Intractable pain crises, priapism, and stroke.
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What is the role of Cytotoxic agents?
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By stimulating erythropoiesis as to increase the hemoglobin F levels. Hydroxyurea (500–750 mg/d) reduce frequency of painful crisis.
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What is special about hemoglobin F?
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hemoglobin F cannot participate in polymer formation, thus its presence markedly retards sickling.
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is SSD compensated?
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chronic well compensated hemolytic anemia with appropriate reticulocytosis
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when is acute drop inhb in SSD
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secondary to hyperhemolytic crisis, splenic sequestration or aplastic crisis
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what is aplastic crisis
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transient arrest of erytropoesis that result in severe drop of Hb and virtual absence of reticulocytes in peripheral blood( reticulocytosis < 1%)
maybe cause by Parvovirus 19 tx is usualy blood transfusion |
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what is difference in aplastic crisis in SSD and aplastic anemia?`
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in anemia will be pancytemia
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what is splenic sequestration
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this occurs in patients who doesn't have yet autosplenoectomy
caused by vaso occlusion and pooling of RBCwithng the spleen may lead to severe hypotensive shock characterized by dramatic drop of Hband persistent reticulocytosis classic physicial s finding rapidly enlarged spleen mortality 10 -15 % and 5 0% chance of recurrence splenectomy is recommended after 1 episode |
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acute chest syndrome
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presence of fever
chest pain and infiltrate in chest xray etiology is related to infection and pulmonary infarction |
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vasooclusive crisis clinical
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acute onset f pain and it caused by vasoocclusion secodnary to sickling or RBC
may be precipatating by weather dehydration or infection severe anemcia and low reticulocytes are not seen in vasoocclusive crisis |
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hyperhemolytic crisis
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rare complication of SSD
sudden severe anemia, appropriate reticulocytosis etiology is uknown |
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what is the most likely ds
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look for AA with sudden onset of severe pain in chest , back, tight
maybe fever if P is old enough, it is rare that he didnot have same before( history!) |
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common clinical manifestation of SSD
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chronic-- gallstones
increased infection osteomyelitis( salm) retinopathy increased heart with hyperdynamic features and systolic murmur lower extr - ulcers avascular necrosis dactilitus in chidren papillary necrosis - dues to chronic kidney injury stroke |
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acute painful vasooclusive crisis caused by
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hypoxia
dehydration infection cold temperature |
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what will be on per smear
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remnats- of nuclear aterial inside the red cells- Howell jolly bodies
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if fever - management
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dont wait for blood test- start AB cefriaxone
levofloxacin moxifloaxacin |
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what vit should be added
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folate on chronuc basis
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vaccination
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pneimoc
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what will increased hem F
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hydroxyurea
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when exange tranfusuin is nedded
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acute chest syndrome
priapism stroke visual disturb |
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what is best initial test in aplastic crisis?
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reticul
P with chronic SSD have high reticul. but in aplastic crisis , they will drop |
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what is MCC of aplastic srisis
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parvovitrus
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what is SStrait
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p is heterozigote for the sickel gene (AS)
only one manifestation- isostenuria- cant concentrate urine clinical asymptomatic normal CBC and smear hematuria may be sometime tx? no tx |
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epidemio;ogy of SS trait
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1 of 12 AA carry the trait
also in Italian Greeks and Saudi Arabian |
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what is hand foot syndrome
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dactilitis painful swelling od dorsal of hands and feet in infants and early childhood( 4- 6 month usually)
avascular necrosis in metacarpal and metatarsal bones |
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avascular necrosis of joints
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most common in h ips( decreased blood supply)- femoral head
shoulder_(humeral head) |
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priapism
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30min -3 hours lasts
usually subside after urine passed, light exercise or cold shower |
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management of painful crisis
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hydration
morphine keep him warm oxygen hydroxy urea bllod tranmfusion |
