dr. sakeena noureldine consultant in paediatrics anemias in children.pdf · or morphine derivatives...
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Dr. Sakeena NourEldine
Consultant in Paediatrics
Definition:
- Areduction of the red blood cell or hemoglobin
concentration below
the range of values occuring
The Anaemias
.The leuel of Hb differ according to
* Age
* Race . black have levels about o.s gm lower than white
and Aslan
* Ethnic group.
Physiological adjustment to anemia includes:
• Increased cardiac output
• Increased A.V oxygen differences
• shunt of blood flow toward vital organ and
tissues
• Increase conc. Of 2,3 –DPG within the RBCs• Increase conc. Of 2,3 –DPG within the RBCs
• shift to the right of the oxygen dissociation curve
- in moderate anemia few symptoms and findings
may be evident
- in severe anemia tachypnoea tachycardia and
congestive heart failure occur
COMMON CAUSES OF ANEMIACOMMON CAUSES OF ANEMIA
Hypochromic microcytic anemia :
Cause
1. iron deficiency anemia
2. Thalassemia
3. chronic inflammatory disease 3. chronic inflammatory disease
4. Copper defficiency
5. Sideroblastic anemia
6. Hereditary
.NORMOCHROMIC NORMOCYTIC
1.Chronic inflammatory disease.
2.Recent blood loss
3.Malignancy and marrow infiltration
4.Marrow aplasia or hypoplasia
5.Chronic renal failure5.Chronic renal failure
6.H I V
7.Hemophagocytic syndrome
Macrocytic Anemia:
Cause:
1. Vitamin B12 deficiency
2. Folate deficiency
3. Hypothyroidism
4. Marrow failure 4. Marrow failure
Hemolytic Anemia:
Cause:
1. Hemoglobinopathy eg(Hbs)
2. Enzymatic deficiency eg(G6PD deficiency)
3. Cell membrane defect eg(heriditary
spherocytosis)
4. Extrinsic factor eg(DIC,HUS)4. Extrinsic factor eg(DIC,HUS)
5. Immune hemolytic anemia eg(Autoimmune
deficiency,Drug induced )
IRON DIFICIENCY
ANEMIA
-It is the common hematologic diseae -It is the common hematologic diseae
of infancy and childhood
-30 percent of the globalpopulation
suffers from it mostof them in the
developing countries
Etiology
• inadequate dietary intake
• .blood loss
• Occlut bleeding in different sites
• -peptic ulcer • -peptic ulcer • -mechel diverticulum
• -polyps
• haemangioma
Pulmonary haemosiderosis
• Hook worm infestation
Clinical manifestations:
• Pallo,r irritability.
• If hemoglobin falls below 5gm
tachycardia and cardiac dilatation
occur as well as systolic murmur. occur as well as systolic murmur.
• Iron deficiency have effects on
neurologic and intellectual function. It
affects attention, alertness and
learning.
Laboratories findings:
• Serum ferritin and serum iron.
• Iron stores, bone marrow hemosiderin
• Iron binding capacity "transferrin" .
• Later the RBCs become smaller and the • Later the RBCs become smaller and the hemoglobin content deceases-- Microcytic, hypochromic.
• MCV and MC H decreases.
• Thrombocytosis.
• Hyper cellular marrow with Erythroid hyperplasia.
.
TREATMENT:
• Iron salts supplementation in ferrous forms ,eg
• fumarate sulphate or gluconate.
• Dose : 4-6 m g / kg / day
• Parenteral iron rarely used, eg in malabsorption
• Iron rich food
• Parents education
• Iron medication should be continued for 8 wks after
• blood values returned to normal.
• Blood transfusion is rarely indicated.
HEMOLYTIC ANEMIAS
• Definition:
• Hemolysis is the premature destruction of
red blood cells.
• Anemia results when the rate of • Anemia results when the rate of
destruction exceeds the capacity of the
marrow to produce RBCs
• The normal RBC survival time is 110-120
days
classification of hemolytic anemia
1) cellular :
resulting from intrinsic
abnormalities of the membrane ,
Enzymes or hemoglobin Enzymes or hemoglobin
2) Extracellular :
resulting from antibodies ,
mechanical factors or plasma factors .
.
SICKLE CELL ANEMIA• Hemoglobin S results from a change which
encodes valine instead of glutamine in the 6+h
position in the beta globin molecule
• Sickle cell anemia occurs when both beta globins
genes have the sickle cell mutation.
• Sickle cell disease accrues when one betaglobin • Sickle cell disease accrues when one betaglobin
gene have the sickle cell mutation and the other
carry gene mutation other than sickle cell
mutations.
• In sickle cell anemia Hbs is commonly as high as
90% of the total hemoglobin where as in sickle cell
disease Hbs is < 50%
• Inheritance is Autosomal recessive
Clinical Manifestations and treatment
• children with sickle cell anemia have abnormal immune function due to functional asplenia or autosplenectomy
• Bacterial sepsis is one of the greatest • Bacterial sepsis is one of the greatest causes of morbidity and mortality .
• Children with sickle cell anemia have deficient levels of serum opsonin of the alternate complement pathway against pneumococci. (encapculated)
• Children with sickle cell anemia should receive prophylactic oral penicillin V.
• Acute infection with parvovirus B19 is usually associated with red cell is usually associated with red cell oplasia – aplastic crises – fever, .pain , splenic sequestration and ACS
Dactylitis
• often refered to as hand – foot
syndrome
• It is the first manifestation of pain • It is the first manifestation of pain
in children with sickle cell anemia
• Occur in 50% of children by 2
years of age and is often
symmetrical
Acute splenic sequestration
• It is a life threatening complication .
• Approximately in 30 % of pts .
• The etiology is unknown
• Clinically it is associated with an engorged,
enlarged spleen , evidence of hyopvolemia
and reduction of hemoglobin
• Could be associated with URTI or viral
infection
• Treatment require early intervention and
maintenance of homodynamic stability by
either isotonic fluid or blood transfusion .
• Repeated episodes of splenic • Repeated episodes of splenic
sequestration are comon .
• Prophylactic splenectomy is the only
elective strategy for prevention of future
life threatening episodes
Vaso – occlusive episode
the pain is characterized as unremitting
discomfort that may occur any where but
most often in the chest , Abdomen and
extremities
The pathogenesis of pain /disruption of The pathogenesis of pain /disruption of
blood flow in the micro vessels by sickle
cells Resulting in tissue ischemia .
Precipitating factors include physical
stress , infection , dehydration , hypoxia ,
acidosis and exposure to cold.
Treatment or painful episodes requires
1) education of the parents and the patient about
symptoms and management strategy .
2) acetaminophen or anon steroidal agent early in the
course of pain .
3)hospital admission with iv administration of morphine
or morphine derivatives
4) Iv hydration is appropriate for children with
dehydration or is unable to drink as a \result of pain
5) Hydroxyurea a myelosuppresive agent which
reduces the frequency of painful episodes It acts
by raising the level or Hb F and the hemoglobin level
Priapism
• It is an involuntary penile erection lasting longer than 30 min and it is painful .
• It may persist for hours
• Treatment : • Treatment :
1. Acute treatment – supportive initially but if it persist for 4 hours urology consultation should be done for further treatment.
2. Preventive therapy-hydroxyurea has promising results .
Neurologic
• Presentation of stroke may occur as young as 1
year of age .
• stroke may run in particular families .
• other neurologic complications include
headaches, seizures , cerebral venous
thrombosis .thrombosis .
• Treatment of stroke includes :
• oxygen administration to maintain O2 sat more
than 96% .
• Blood transfusion as quickly as possible
exchange transfusion to reduce Hbs to < 50%
prevention of stroke by;
• Regular blood transfusion after the
stroke to decrease Hbs level .
• primary prevention can he • primary prevention can he
accomplished by transcranial
Doppler assessment or the blood
velocity
Acute chest syndrome
• characterized by fever, respiratory distress , chest pain and a new radio density on chest radiograph.
• Early detection of ACS will alter the • Early detection of ACS will alter the clinical management.
• Other pulmonary complications include pneumonia and bronchiolitis
Treatment of acute chest synome includes:
–Oxygen administration.
–Simple or exchange transfusion.
–Admission to the ICU.
–Antibiotics – Macrolide and a third
generation cephalosporine.generation cephalosporine.
Sickle cell nephropathy include gross
• hematuria.
• Papillary necrosis.
• Nephrotic syndrome.
• Pyelonephritis.
Other complications
• Sickle cell retinopathy.
• Delayed onset of puberly. • Delayed onset of puberly.
• Avascular necrosis of the
femoral head, and humerus.
• Leg ulcer.
Diagnosis of sickle cell disease
• Complete blood count.
• Hemoglobin analysis –
Electrophoresis.
• Newborn screening programs. • Newborn screening programs.
• Other sickle cell syndrome:
• Hb Sc
• Hbs beta thalassemia.
• Hb So.
Sickle cell trait
• The amount of Hbs in individuals
with sickle cell trait is less than
50% and Hb A is more than 50%.
• Life span is normal and serious • Life span is normal and serious
complications are very rare.
• Complications include splenic
infarcts at high alttitude .
• Hematuria, hyposthenuria
Thalassemia syndromes
• These are genetic disorders in globins chain
production.
• Beta thalassemia is either:
– beta0 thanlsemia due to complete absence of beta globins
gene production.
– beta+ thalassemia due to partial reduction. – beta+ thalassemia due to partial reduction.
• Alpha thalassemia is also due to cither absent or
partially reduced alpha – glbobin gene production.
• In thalassaeima the primary pathology is the quantity
of globins gene production where as in sickle cell
disease the pathology is the quality of globins
production.
Homozygous beta-thalassemia:
• Called also thalasssemia major or colley
anemia.
• Usually become symptomatic in the 2nd
half of the first year.
• The classic findings includes maxillary • The classic findings includes maxillary
hyperplasia, flat hasal bridge and frontal
bossing – thalassemic facies .
• Pathologic bone fiactuces.
• Marked hpatosplenomegaly and cachexia.
• Secondary hypersplenism.
• Features of ineffective erythropoiesis. Eg
expanded medullary spaces.
• Pallor, hemosiderosis – jaundice.
• Symptoms due to excessive iron stores: • Symptoms due to excessive iron stores:
– Endocroine dysfunction includes hypothyroidism,
gonadal failure, hypoparathyroidism and DM.
– Cardiac dysfunction include congestive heart failure
– cardiac arrhythmia.
Laboratory findings
• CBC showed severe anemia Hb 5gm
reticulocyte count 8%.
• Chematy: showed increased
unconjugated bilirubin unconjugated bilirubin
• Hemoglobin electropholesis.
• Treatment:
• Confirm the diagnosis.
• councelling.
• Regular blood treastnsion morthly to
keep the hemoglobin > 9.5 < 10.5
• Iron chelating agent to avoid
transfusional hemosiderosis. E.g
deferoxamine subcutaneous infusion.
• Bone marrow thransplantation can • Bone marrow thransplantation can
give cure if an HLA matched sibling is
available.
Alpha Thalassemia• Infants are identified in the newborn period by the
increased production of barts hemoglobin (gama
4) during fetal life and its presence at birth.
• Occur most frequently in southeast Asia.
• The hemoglobin analysis is normal except during
the newborn period when Hb bart's is found < 8% the newborn period when Hb bart's is found < 8%
• The deletion of 1 alpha globin gene is not
identifiable hematological.
• The deletion of 2 globin genes lesnlts in alpha
thalassemia trait with considered as having iron
diffiency anemia – 10 w Mcv and Mc
• The deletion of 3 alpha globin genes give adiagnosis of Hb H disease
• The deletion of all alpha globins genes causes profound anemia during fetal life resulting in hydrops fetalis
• Children with alpha th major are transfusion dependant and bone marrow transfusion dependant and bone marrow transplant is the only cure.
• Treatment includes.
–Folic acid supplementation
–Possible splenectomy
–Chromic transfusion therapy
–Bone marrow transplantation
•THANK YOU
•AND HAVE ANICE
•DAY
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