Hereditory Sph Jotes

Question 0

Why MCHC increased

Answer 0

MCHC ^ coz cells are smaller

Question 1

Age of splenectomy for HS

Answer 1

After 5-6yrs

Question 2

Complications

Answer 2

Aplastic crisis
Megaloblastic crisis
Hemolytic crisis
Cholecystitis
Cholelithiasis
Severe neonatal hemolysis

Question 3

Why more jaundice in HS compared to SS and Thal

Answer 3

HbF has less affinity for 2,3-BPG, free 2,3-BPG affects the already defective RBC membrane leading to more hemolysis(read more)

Question 4

Risk of kernicterus at what bilirubin level

Answer 4

> 20

Question 5

Level of bil at which phototherapy should be started

Answer 5

> 10mg(read more)

Question 6

Phototherapy in conjugated hyperbil.

Answer 6

Don’t give phototherapy to conjugated hyperbil. It will lead to bronze baby(read more)

Question 7

Color of phototherapy light and wavelength

Answer 7

Blue light = 450-470

Question 8

Signs of kernicterus

Answer 8

Hearing problem, distonic posture

Question 9

Calculate expected weight of a child equal to or less than 7

Answer 9

Age/2 +8

Question 10

When to start measuring the height of a child rather than the length

Answer 10

2years of age
75 - 1yr
85 - 2yrs

Question 11

When an infant with Fe def has stunted growth(failure to thrive) what would you think of

Answer 11

Lactose intolerance

Question 12

Causes of right upper quadrat pain

Answer 12

Hepatitis
Gallstone 
Lower lobe pneumonia
Tender hepatomegaly in congestive heart failure
Pancreatitis

Question 13

Signs and symp of bacterial infection

Answer 13

High fever and rigor

Question 14

Direct and indirect hyperbilirubinemia in a patient with HS means what?

Answer 14

Indicates liver involvement

Question 15

Causes of spherocytes

Answer 15

ABO incompatibility
Wilson disease
Autoimmune hemolytic anemia

Question 16

Why Hb level normal in HS

Answer 16

Coz though membrane is abnormal, Hb synthesis is normal

Question 17

Infant suspected to have HS, grandmother had cholecystectomy at age of 60, significant or not?

Answer 17

Not significant, only if less than 30yr will be significant

Question 18

Types of gallstones and their causes

Answer 18

Cholesterol stones -

Pigment stone - hemolytic anemia due to hyperbilirubinemia

Question 19

Causes of sudden onset of pallor

Answer 19

Autoimmune hemolytic anemia (reddish urine) eg drug induced,
Other hemolytic anemia
Paroxysmal nocturnal Hburia

Question 20

Percentage of spherocytes in HS and autoimmune hem. anemia

Answer 20

HS = >30%
AIH = <30%

Question 21

Why aplastic crisis during infection

Answer 21

Because cell production is decreased (why?)

Question 22

What is the normal neonatal wt loss

Answer 22

10% in the 1st week

Question 23

What is the name and color of stool at birth

Answer 23

It’s called meconium and it’s black/black green, it changes to yellow after first week of birth

Question 24

How to examine for jaundice

Answer 24

Apply pressure on boney prominences, observe the blanched area to see if it’s white, then its negative, if yellow = jaundiced
Face - zygomatic bone(cheek)
Trunk - ribs and sternum
Lower limb - tibia

Question 25

How to clinically judge the degree of jaundice

Answer 25

Face 5%
Trunk 10%
Lower limb 15%
Sole 20%

Question 26

Level of bilirubin before it appears clinically,( on skin )

Answer 26

5% on face

Question 27

Difference between physiological and pathological jaundice

Answer 27

Physiological; very slow rise, level less than….., does not start at birth but ….. after birth

Question 28

Vitals in new born

Answer 28

BP - half of that of adult = 60/40
HR - double of that of adult = 140
RR - double of that of adult = 40
Hb - 30% more than adult’s =

Question 29

How cephalohematoma cause rise in bilirubin?

Answer 29

The extravasated blood that forms the hematoma will be hemolysed leading to release of bilirubin

Question 30

Why palpable spleen in young children not considered splenomegaly

Answer 30

Bcoz spleen is normally palpable in children upto 2yrs

Question 31

Tests for HS

Answer 31

First do bilirubin level (direct(>2mg/dL) & indirect(>20%)), then CBC and Peripheral smear, comb’s test (direct/indirect)(read more),

Question 32

Life span of spherocytes

Answer 32

30 days

Question 33

Fetal RBC life span

Answer 33

60 - 90 days

Question 34

Counseling of hereditary spherocytosis

Answer 34

Autosomal dominant
Lifelong folic acid
Vaccination against capsulated microorganism
Splenectomy