Pediatric

Question 1

Kerh Sign

Answer 1

Referred left shoulder pain

Question 2

Vitamine B1

Answer 2

Thiamine

Question 3

Vit B2

Answer 3

Riboflavine

Question 4

Vit B3

Answer 4

Niacin

Question 5

B6

Answer 5

Pyridoxine

Question 6

TB meningitis
Rare but serious complication
Of Primary TB infection due to hematogenous spread of mycobacteria to subarachnoid space
Cerebrospinal fluid:

Answer 6

Lymphocytic pleocytosis
High protein
Low glucose

Question 7

TB meningitis
Rare but serious complication
Of Primary TB infection due to hematogenous spread of mycobacteria to subarachnoid space
Cerebrospinal fluid:

Answer 7

Lymphocytic pleocytosis
High protein
Low glucose

Question 8

WAGR SYNDROME

Answer 8

11p deletion syndrome
Wilms tumor
Aniridia
Genitourinary abnormalities
Range of developmental delays

Question 9

Hemophilia coagulation test presents with

Answer 9

Prolonged PTT
Normal PT
Normal platelet count

Question 10

Acrocyanosis

Answer 10

A benign form of peripheral cyanosis

Question 11

Wilson Disease

Answer 11

AR mutation ATP7B
Hepatic Cooper accumulation
Leak from damaged hepatocytes
Deposits in tissues (basal ganglia cornea)

Question 12

Wilson D

Clinical findings

Answer 12

Hepatic (acute liver failure, chronic hepatitis, cirrhosis)
Neurologic (Parkinsonism, gait disturbance, disarthria)
Psychiatric ( depression, personality changes, psychosis)

Question 13

Wilson D
Dx

Answer 13

⬇️ Ceruloplamin
⬆️ Urinary Cooper excretion
⬆️ Cooper content on liver Bx
Kayser - fleischer rings on still lamp examination

Question 14

Wilson D
Tx

Answer 14

Chelators
D - Penicillamine; trientine

Zinc ( interfering with cooper absorption

Question 15

Tracheoesophagel Fistula (TEF)
With Esophageal Atresia (EA)
Dx with

Answer 15

Resistance of nasogastric tube advancement beyond the proximal esophagus.

Question 16

TEF & EA
Typically occurs with VACTERL Assoc

Answer 16

(Vertebral, Anal, Cardiac, TEF, Renal and or Limb anomalies
✨all patients who have TEF with EA should undergo screening echocardiography & renal ultrasonography.

Question 17

Chickenpox

Answer 17

Varicela

Question 18

Phenotypes
👉🏽DiGeorge syndrome
👉🏽 Velocardiofacial Syndrome

Answer 18

Loss of genes that control embryonic tissue template transformation (pharyngeal apparatus, Secondary heart field) and migration of neural crest cells

Question 19

Disgorge Syndrome
Clinical features

Answer 19

CATCH
Cardiac outflow tract anomalies (tetralogy of fallot, Persistent Truncus Arteriosus)

Anomalous face ( prominent nasal bridge, low set of ears, micro/retrognatia)

Tonic hypoplasia/aplasia 👉🏽 Decrease Tcell immunity

Cleft palate

Hypoparathyroidism ( hypoplastic/aplastic parathyroids) Hypocalcemia

Question 20

Howell-Jolly bodies will be an expected finding on peripheral smear
Small purple dots with in the red blood cell

Answer 20

Sickel cell
Due to functional asplenia
Howell - Jolly bodies are nuclear remnants of red blood cells that are usually removed by a functional spleen.

Question 21

Helmet cells or schistocytosis
Fragmented RBC

Answer 21

Suggest traumatic microangi
opathathic
Hemolytic conditions such us
DIC
Uremic syndrome
TTP
🤭not typically seen in SCD as this hemolysis is intrinsic, not traumatic

Question 22

Heinz cells,
Bite cells

Answer 22

Heinz bodies
Which appear as small inclusions within in erythrocyte, are aggregates of denatured hemoglobin and are commonly seen in patients with hemolysis due to
G6PD & Thalassemia
When phagocytes extract this rigid precipitate they form characteristic bite cells.

Question 23

Basophilic stippling

Answer 23

Ribosomal precipitate that appear as blue granules of various sizes dispersed throughout the cytoplasm of red blood cells
Seen
Thalassemia
Lead or heavy metal poisoning

Question 24

TACO

Answer 24

Transfusion Associated circulatory overload
Reaction when large volume of blood is transfused leading to fluid overload
Patient with chronic or renal disorders,children age <3 and pt >60 with chronic anemia are at increased risk
👉🏽toddlers with severe chronic iron deficiency anemia due to excessive milk consumption >24 oz/day and poor intake of iron reach food are susceptible to TACO

Question 25

Most common childhood acute cancer Peak age 2-5

Answer 25

Acute lymphoblastic Leukemia

Question 26

The most congenital CAH is

Answer 26

21 hydroxylase deficiency

Question 27

21 hydroxylase deficiency results in

Answer 27

Decreased cortisol and aldosterone Increased 17 hydroxyprogestererone (17-OHP) the normal substrate for 21 hydroxylase (17-OHP) is then converted in testosterone in peripheral tissues. This excess androgen production leads to virilization (clitoromegaly/ underdevelope phallus, nobpalpable gonads) in genotypically female (46XX)

Question 28

Tetralogy of fallot

Answer 28

Ventricular Septal defect Overriding aorta over the right and left ventricle RVOT Right Ventricular Outflow Tract Obstruction Right Ventricular Hypertrophy

Question 29

LAD Leukocytes adhesion deficiency

Answer 29

Defect in CD18 containing integrins Impaired leukocyte adhesion & endothelial transmigration

Question 30

LEUKOCYTE ADHESION DEFICIENCY LAD

Answer 30

Clx Skin, mucosal infections (Periodontitis, cellulitis) without pus formation. Impaired wound healing Delayed umbilical cord separation Lab Leukocytosis & Neutrofilia

Question 31

Bruton Agammaglobulinemia

Answer 31

BTK gene mutation resulting in in defective Bruton tyrosine kinase IMPAIRED B cell maturation & Immunoglobulin production.

Question 32

X- LINKED Agammaglubulinemia Clx

Answer 32

- Recurrent sinupulmonary & GI infections at age > 3-6 m - Chronic pain enteroviral infection - Small or absent lymphoid tissue (tonsils, adenoids)