Hereditory Sph Jotes Flashcards
Why MCHC increased
MCHC ^ coz cells are smaller
Age of splenectomy for HS
After 5-6yrs
Complications
Aplastic crisis Megaloblastic crisis Hemolytic crisis Cholecystitis Cholelithiasis Severe neonatal hemolysis
Why more jaundice in HS compared to SS and Thal
HbF has less affinity for 2,3-BPG, free 2,3-BPG affects the already defective RBC membrane leading to more hemolysis(read more)
Risk of kernicterus at what bilirubin level
> 20
Level of bil at which phototherapy should be started
> 10mg(read more)
Phototherapy in conjugated hyperbil.
Don’t give phototherapy to conjugated hyperbil. It will lead to bronze baby(read more)
Color of phototherapy light and wavelength
Blue light = 450-470
Signs of kernicterus
Hearing problem, distonic posture
Calculate expected weight of a child equal to or less than 7
Age/2 +8
When to start measuring the height of a child rather than the length
2years of age
75 - 1yr
85 - 2yrs
When an infant with Fe def has stunted growth(failure to thrive) what would you think of
Lactose intolerance
Causes of right upper quadrat pain
Hepatitis Gallstone Lower lobe pneumonia Tender hepatomegaly in congestive heart failure Pancreatitis
Signs and symp of bacterial infection
High fever and rigor
Direct and indirect hyperbilirubinemia in a patient with HS means what?
Indicates liver involvement
Causes of spherocytes
ABO incompatibility
Wilson disease
Autoimmune hemolytic anemia
Why Hb level normal in HS
Coz though membrane is abnormal, Hb synthesis is normal
Infant suspected to have HS, grandmother had cholecystectomy at age of 60, significant or not?
Not significant, only if less than 30yr will be significant
Types of gallstones and their causes
Cholesterol stones -
Pigment stone - hemolytic anemia due to hyperbilirubinemia
Causes of sudden onset of pallor
Autoimmune hemolytic anemia (reddish urine) eg drug induced,
Other hemolytic anemia
Paroxysmal nocturnal Hburia
Percentage of spherocytes in HS and autoimmune hem. anemia
HS = >30% AIH = <30%
Why aplastic crisis during infection
Because cell production is decreased (why?)
What is the normal neonatal wt loss
10% in the 1st week
What is the name and color of stool at birth
It’s called meconium and it’s black/black green, it changes to yellow after first week of birth
